I

DIFFERENTIAL
DIAGNOSIS
OF MUSCULOSKELETAL
DISORDERS

DIFFERENTIAL.DIAGNOSTIC
GAMUTOF BONE DELAYED
BONEAGE
DISORDERS A. CONSTITUTIONAL
Conditionsto be considered= "dissectbone diseasewith 1. Fami l i al
a DIATTOM" 2. IU GR
Dysplasia+ Dystrophy B. METABOLIC
Infection 1. Hypopituitarism
Anomaliesof development 2. Hypothyroidism
Tumor+ tumorlikeconditions 3. Hypogonadism (Turnersyndrome)
Trauma 4. Cushingdisease,steroidtherapy
Osteochondritis+ ischemicnecrosis 5. Diabetesmellitus
Metabolicdisease 6. Rickets
DYSPLASIA = disturbanceof bone growth 7. Malnutrition
DYSTROPHY = disturbanceof nutrition 8. lrradiationof brain (for cerebraltumor/ ALL)
C. SYSTEMICDISEASE
1. Congenitalheartdisease
L IM P I NG CHI LD 2. Renaldisease
1-4 Years 3. Gl disease:celiacdisease,Crohndisease,
A. CONGENITAL ulcerativecolitis
1. Developmental dysplasiaof hip 4. A nemi a
B. TRAUMATIC 5. Bone marrowtransplantation(<5 years of age)
1. Toddler'sfracture D . S Y N D R OME S
2. Nonaccidentaltrauma 1. Tri somi es
3. Otherfractures 2. Noonandisease
4. Foreignbody 3. Corneliade Langesyndrome
C. INFLAMMATORY 4. Cleidocranialdysplasia
1. Dis k it is 5. Lesch-Nyhan disease
2. Septicarthritis 6. Metatrophicdwarfism
3. Osteomyelitis
4. Transientsynovitisof hip BONESCLEROSIS
Diffuse Osteosclerosis
mnemonic.' "5 M'S To PROoF"
4-10 Years Metastases
A. TRAUMATIC Myelofibrosis
B. INFLAMMATORY Mastocytosis
1. Septicarthritis Melorheostosis
2. Osteomyelitis Metabolic: hypervitaminosis
D, fluorosis,
3. Transientsynovitisof hip phosphoruspoisoning
hypothyroidism,
4. Diskitis Sicklecelldisease
5. Juvenilerheumatoidarthritis Tuberoussclerosis
C, VASCULAR Pyknodysostosis,Pagetdisease
1. Legg-Perthes disease Renalosteodystrophy
Osteopetrosis
Fluorosis
10-15Years
A. TRAUMATIC Constitutional
SclerosingBoneDisease
1. Stressfracture 1. Engelmann-Camurati disease
2. Osteochondritis dissecans 2. lnfantilecorticalhyperostosis
3. Osgood-Schlatter disease 3. Melorheostosis
C. INFLAMMATORY 4. Osteopathiastriata
1. Juvenilerheumatoidarthritis 5. Osteopetrosis
2. Ankylosingspondylitis 6, Osteopoikilosis
3. Septicarthritis 7. Pachydermoperiostosis
4. Osteomyelitis 8, Pyknodysostosis
D. HORMONAL 9. Van Buchemdisease
1. Epiphyseolysis of femoralhead 10. Williamssyndrome
2 RadiologyReviewManual

SclerosingBoneDysplasia (c) malignanttumor: lytic metastasisafter radiation,

A. Dysplasiasof ENDOCHONDRAL OSSIFICATION chemo-,hormonetherapy
(PRTMARY SPONGTOSA) D. INFECTION/ INFLAMMATION
= failurein resorption+ remodelingof primary (low-gradechronicinfection/ healinginfection)
immaturespongiosaby osteoclasts 1. Osteoidosteoma
r/ accumulationof calcifiedcartilagematrixpacking 2. Chronic/ healedosteomyelitis:bacterial,
the medullarycavity fungal
tuberculous,
Targetsites: tubular+ flat bones,vertebrae,skull 3. Sclerosingosteomyelitisof Garr6
base, ethmoids,ends of clavicle 4. Granuloma
1. Osteopetrosis 5. Brodieabscess
2. Pyknodysostosis E . B E N IGNTU MOR
B. Dysplasiasof ENDOCHONDRAL OSSIFICATION 1. Osteoma
(SEcoNDARYSPONGTOSA) 2. Osteoblastoma
= €rrors in resorption+ remodelingof secondary 3. Ossifyingfibroma
spongiosa 4. Healedfibrouscorticaldefect
r/ focal densities/ striations 5. Enchondroma/ osteochondroma
1. Enostosis F. MALIGNANTTUMOR
2. Osteopoikilosis 1. Osteoblasticmetastasis(prostate,breast)
3. Osteopathiastriata 2. Lymphoma
C. Dysplasias of INTRAMEMBRANOUS OSSIFICATION 3. Sarcoma: osteo-,chondro-,Ewingsarcoma
= disequilibriumbetweenperiostealbone formation G. OTHERS
+ endostealbone resorption 1. Scleroticphase of Pagetdisease
Targetsites: cortexof tubular+ flat bones,calvaria, 2. Fibrousdysplasia
bones of upperface, tympanicparts
of temporalbone,vomer,medial Cortical Sclerotic Lesion in Child
pterygoid 1. Osteoidosteoma
1. Progressivediaphysealdysplasia 2. Stressfracture
2. Hereditarymultiplediaphysealsclerosis(Ribbing 3. Chronicosteomyelitis
disease) 4. Healedfibrouscorticaldefect
3. Hyperostosiscorticalisgeneralisata
Van Buchemdisease Multiple Osteosclerotic Lesions
Sclerosteosis(Truswell-Hansen disease) A. FAMILIAL
Worth disease 1. Osteopoikilosis
Nakamuradisease 2. Enchondromatosis = Ollierdisease
4. Diaphysealdysplasiawith anemia 3. Melorheostosis
5. Oculodento-osseous dysplasia 4. Multipleosteomas:associated withGardner
6. Trichodento-osseous dysplasia syndrome
7. Kenny-Caffeysyndrome 5. Osteopetrosis
D. MIXEDSCLEROSINGDYSPLASIAS 6. Pyknodysostosis
(a) predominantlyendochondraldisturbance 7. Osteopathiastriata
1. Dysosteosclerosis 8. Chondrodystrophia calcificanscongenita
2. Metaphysealdysplasia(Pyledisease) = cor'tgehital
stippledepiphyses
3. Craniometaphyseal dysplasia 9. Multipleepiphysealdysplasia = Fairbank
disease
4. Frontometaphyseal dysplasia B. SYSTEMIC DISEASE
(b) predominantlyintramembranous defects 1. Mastocytosis= ufiicariapigmentosa
1. Melorheostosis 2. Tuberous sclerosis
2. Craniodiaphyseal dysplasia
3. Lenz-Majewskihyperostoticdwarfism
4. Progressivediaphysealdysplasia Bone-within-bone Appearance
= endosteal newboneformation
SolitaryOsteoscleroticLesion 1. Normal
A. DEVELOPMENTAL (a)thoracic+ lumbarvertebrae (ininfants)
1. B oneis l a n d (b)growthrecovery lines(afterinfancy)
B. VASCULAR corticalhyperostosis
2. Infantile (Caffey)
1. Old bone infarct 3. Sicklecelldisease/ thalassemia
2. Aseptic/ ischemic/ avascularnecrosis 4. Congenital syphilis
C. HE A LI NGB O N EL E SION 5. Osteopetrosis i oxalosis
(a) trauma: callusformationin stressfracture 6. Radiation
(b) benigntumor: fibrouscorticaldefect/ 7. Acromegaly
nonossifyingfibroma,browntumor;bone cyst 8. Pagetdisease
 DifferentialDiagnosisof Musculoskeletal
Disorders

mnemonic; "BLT PLT RSD RSD" Etiology:

Bismuthingestion A . C ON GE N ITA DL IS OR D E R S
Lead ingestion 1. Osteogenesis imperfecta
Thoriumingestion 0 The only osteoporosis with bendingl
Petrosis(osteopetrosis) 2. Homocystinuria
Leukemia B. IDIOPATHIC(bonelossbeginsearlier+ proceeds
Tuberculosis more rapidlyin women)
Rickets 1. Juvenileosteoporosis: <20 years
Scurvy 2. Adultosteoporosis: 20-40 years
D toxicity(vitaminD) 3. Postmenopausal osteoporosis: >50years
RSD (reflexsympatheticdystrophy) (40-50%lswertrabecular bonemineraldensity
in elderly
thanin youngwomen)
Dense Metaphyseal Bands 4. Senileosteoporosis: >60 years
mnemonic; "DENSELINES" progressivelydecreasingbone densityat a rate
D-vitaminintoxication of 8/" in femalesi3% in males
Elementalarsenicand heavymetals(lead,bismuth, C . N U TR ITION AD L IS TU R B A N C E S
phosphorus) scurvy;proteindeficiency(malnutrition, nephrosis,
Normalvariant chronicliverdisease,afcoholism,anorexianervosa,
Systemicillness kwashiorkor,starvation),calciumdeficiency
Estrogento motherduringpregnancy D . E N D OC R IN OP A TH Y
Leukemia Cushingdisease,hypogonadism (Turner
Infection(TORCH),ldiopathichypercalcemia syndrome,eunuchoidism), hyperthyroidism,
Neverforgethealedrickets hyperparathyroidism, acromegaly, Addison
Earlyhypothyroidism (cretinism) disease,diabetesmellitus,pregnancy,
Scurvy,congenitalSyphilis,Sicklecell disease paraneoplastic phenomenonin livertumors
also: methotrexatetherapy E. RENALOSTEODYSTROPHY
decrease/ same / increasein spinaltrabecular
bone;rapidloss in appendicular skeleton
OSTEOPENIA F. IMMOBILIZATION = disuseosteoporosis
= decreasein bonequantitymaintaining normalquality G. COLLAGENDISEASE,RHEUMATOID ARTHRITIS
./ increasedradiolucencyof bone: H . B ON EMA R R OWR E P LA C E ME N T
r/ verticalstriationsin vertebralbodies infiltration
by lymphomai leukemia(ALL),multiple
r/ accentuationof tensile+ compressivetrabeculaeof myeloma,diffusemetastases,marrowhyperplasia
proximalfemur secondaryto hemolyticanemia
r/ reinforcementlines (= bone bars)crossingmarrow I. D R U GTH E R A P Y
cavityabout knee heparin(15,000-30,000 U for >6 months),
r/ corticalresorptionof 2nd metacarpal: methotrexate,corticosteroids, excessivealcohol
r/ measuringoutercorticaldiameter(W) and width of consumption, smoking,Dilantin
medullarycavity(m) at midportionof bone and J. R A D IA TION TH E R A P Y
reportingcombinedcorticalthickness(CCT) K. LOCALIZEDOSTEOPOROSIS
{ subperiosteal tunneling Sudeckdystrophy,transientosteoporosisof hip,
regionalmigratoryosteoporosisof lower
Categories: extremities
A. DI F F US EO ST E O P EN IA . serumcalcium,phosphorus, alkalinephosphatase
1. Osteoporosis= decreasedosteoidproduction frequentlynormal
2. Osteomalacia = undermineralization
of osteoid . hydroxyproline may be elevatedduringacute stage
3. Hyperparathyroidism Technique:
4. Multiplemyeloma/ diffusemetastases (1) Srrrrole-PHororu AesonploMErRy
5. Drugs measuresprimarilycorticalboneof appendicular
6. Mastocytosis bones,single-energyl-125radioisotope source
7. Osteogenesisimperfecta Site: distalradius(= wristbonedensity),os calcis
B. RE G I O NA LOS T EOP EN IA Dose: 2-3 mrem
Precision: 1-3%
Osteoporosis (2) Dunl-PHoroN AssonprroMErRy
= reducedbone mass of normalcompositionsecondary radioactiveenergysourcewith two photonpeaks;
to (a) osteoclasticresorption(85%) (trabecular, shouldbe reservedfor patients<65 years of age
endosteal,intracortical,
subperiosteal) becauseof interferencefrom osteophytosis+
(b) osteocyticresorption(15%) vascular calcifications
lncidence: 7"h of all women aged 95-40 years; Site: vertebrae,femoralneck
1 in 3 women > age 65 years D ose:5-10 mrem: Precision:2-4/"
 Radiology Review Manual

(3) SrrucleX-nnvAasonprtoMErRY Location: axialskeleton(lowerdorsal+ lumbarspine),

= €u€€lprojectionaltechniquefor quantitativebone proximalhumerus,neckof femur,wrist,ribs
densitymeasurement 0 Radiographsare insensitivepriorto bone loss of
Site: distalradius,calcaneus 25-30%
Dose: low 0 Bone scansdo NOT show a diffuseincreasein
Precision: 0.F2% activity
(4) Dunr Eruency X-nnvAesonprtoMErny (DEXA) r/ decreasednumber+ thicknessof trabeculae
0 Mostwidelyused& mostprecisetechnique! { corticalthinning(endosteal+ intracorticalresorption)
= quantitativedigitalradiography r/ juxtaarticularosteopeniawith trabecularbone
= beamswith two distinctenergylevelsallow predominance
of trabecularfrom corticalbone
identification r/ delayedfracturehealingwith poor callusformation
. has replaceddual-photonabsorptiometryand is (DDx:abundantcallusformationin osteogenesis
producedby x-ray tube with higherradiationflux imperfecta+ Cushingsyndrome)
than radioisotopesource Cx: (1) Fracturesat sitesrichin labiletrabecularbone
Site: lumbarspine,femoralneck,wholebody, (eg, vertebrae,wrist) in postmenopausal
forearm osteoPorosis
Dos e:<3 mre m; Precision:1-2o/" (2) Fracturesat sites containingcortical
Data collected: + trabecularbone (eg, hip) in senile
BMD (bone marrowdensity)value (g/cm2; osteooorosis
%BMDcomparedto youngadults Rx: calcitonin, sodiumfluoride,diphosphonates,
%BMD comparedto age-matchedadults parathyroid hormonesupplements, estrogen
T-score(SD of young-adultmean) replacement
Z-score(SD of age-matchedmean)
(5) QunurrrATrvE Cotupureo TotuocnnpHv Osteoporosisof Spine
= determinestrue volumetricdensity(mg/cm3) r/ diminishedradiographic density
. high-turnover cancellousbone is importantfor r/ verticalstriations(= markedthinningof transverse
vertebralstrengthand has high responsiveness trabeculaewith relativeaccentuationof vertical
. trabecularbone + low-turnovercompactbone trabeculaealong linesof stress)
can be measuredseparately { accentuationof endplates
{ comparedto externalbone mineralreference { "pictureframing"(= ?ccentuationof corticaloutline
phantomscannedsimultaneously with patientto with preservationof externaldimensionssecondary
calibrateCT attenuationmeasurements to endosteal+ intracorticalresorption)
r/ 1O-mm-thicksectionwith gantryangle { compressiondeformitieswith protrusionof
correctionthroughcenterof vertebralbody intervertebral disks:
Site: vertebraeL1-L3, other sites ./ biconcavevertebrae
Use: assessmentof vertebralfracturerisk; r/ Schmorlnodes
measurement of age-relatedbone loss; r/ wedging
follow-upof osteoporosis+ metabolicbone { decreasedheightof vertebrae
disease { absenceof osteoPhYtes
(a) singleenergy: 300-500 mrem;
6-25% precision Osteomalacia
(b) dual energy: 750-800 mrem; = occurnulationof excessiveamountsof uncalcified
5-10% precision osteoidwith bone softening+ insufficient
0 Mostsensitivetechnique! mineralization of osteoiddue to
(6) PenreHennl Qunurrnlvr CT (a) high remodelingrate: excessiveosteoidformation
= €X?ct3-dimensionallocalizationof target + normal/ littlemineralization
volumeswith multisection data acquisition (b) low remodelingrate: normalosteoidproduction
capabilitycoveringa largevolumeof bone + diminishedmineralization
Site: distalradius Etiology:
(1) dietarydeficiencyof vitaminD. + lackof solar
irradiation
(2) deficiencyof metabolismof vitaminD:
DEXAInterpretation - chronicrenaltubulardisease
- chronicadministration of phenobarbital
Diagnosis T-score Management Follow-up (alternateliverPathwaY)
- diphenylhydantoin (interferes
with vitaminD
Normal >-1 prevention 3 years
Osteopenia <-1 and >-2.5 prevention 2 years actionon bowel)
or theraPy (3) decreasedabsorptionof vitaminD:
- malabsorptionsyndromes(mostcommon)
Osteoporosis<-2.5 therapy 1 year
- partialgastrectomy(self-restrictionof fattyfoods)
 Differential Diagnosis of Musculoskeletal Disorders 5

(4) decreaseddepositionof calciumin bone FrayedMetaphyses

- diphosphonates (fortreatmentof pagetdisease) mnemonic-' "CHARMS"
Histo: excessof osteoidseams + decreased Congenitalinfections(rubella,syphilis)
appositionalrate Hypophosphatasia
. bone pain/ tenderness;muscularweakness
Achondroplasia
. serumcalciumslightlylow / normal
Rickets
. decreasedserum phosphorus
Metaphysealdysostosis
. elevatedserumalkalinephosphatase
Scurvy

r/ uniformosteopenia BONETUMOR
.,1fuzzy indistincttrabeculardetailof endostealsurface
Roleof Radiologist
{ coarsenedfrayedtrabeculaedecreasedin number+
1. l s therea l esi on?
size
2. ls it a bonetumor?
r/ tnin corticesof long bone
3. ls the tumorbenignor malignant?
r/ bone deformityfrom softening:
4. ls a biopsynecessary?
r/ hourglassthorax
5. ls the histologicdiagnosisconsistentwith the
r/ bowingof long bones
radiographic image?
{ acetabularprotrusion
! buckled/ compressedpelvis Assessment of Agg ressiveness
./ biconcavevertebralbodies A. BENIGN
^/ increasedincidenceof insufficiencyfractures 1. Diagnosis certain:no furtherwork-upnecessary
r/ pseudofractures = Looserzones 2. Asymptomatic lesionwith highlyprobable
! mottledskull benigndiagnosismay be followedclinically
3. Symptomatic lesionwith highlyprobable
benigndiagnosismay be treatedwithout
Localized/ RegionalOsteopenia furtherwork-up
1. Disuseosteoporosis
/ atrophy B . C ON FU S IN GLE S ION
Etiology: localimmobilizationsecondaryto not clearlycategorizedas benignor malignant;
(a) fracture(morepronounceddistalto fracturesite) needsstagingwork-up
(b) neuralparalysis C. MALIGNANT:needsstagingwork-up
(c) muscularparalysis Staging work-up:
2. Reflexsympatheticdystrophy= Sudeckdystrophy Bone scan: identifiespolyostoticlesions(eg,
3. Regionalmigratoryosteoporosis,transientregional multiplemyeloma,metastaticdisease,
osteoporosis of hip primaryosteosarcomawith bone-
4. Rheumatologic disorders formingmetastases,histiocytosis,
5. Infection:osteomyelitis,
tuberculosis Pagetdisease)
6. Osteolytictumor ChestCT: identifiesmetastaticdeposits+
7. Lyticphase of Pagetdisease changesfurtherwork-upand therapy
8. Earlyphase of bone infarctand hemorrhage Local staging with MB imaging:
9. Burns+ frostbite (1) Marginsrencapsulated / infiltrating
(2) Compartment:intra-/ extracompartmental
(3) Intraosseousextent+ skip lesions
BoneMarrowEdema (4) Soft-tissue extent(DDx:hematoma,edema)
= hypointensity
onTlWl+ hyperintensity
onT2Wl (5) Jointinvolvement
1. Transientosteoporosis of hip (6) Neurovascularinvolvement
2. Osteonecrosis= early stage of AVN Localassessrnent with CT imaging:
3. Trauma r/ matrix/ rim calcifications
(a) "bonebruise"
(b) radiographically
occultfracturein elderlywomen TumorlikeConditions
4. Infection= osleomyelitis 1. Solitarybonecyst
5. lnfiltrativeneoplasm 2. Juxtaarticular("synovial")cyst
3. Aneurysmalbonecyst
TransverseLucentMetaphysealLines 4. Nonossifyingfibroma;corticaldefect;cortical
mnemonic.' "LlNlNG" desmoid
Leukemia 5 . Eosinophilicgranuloma
lllness,systemic(rickets,scurvy) 6 . Reparativegiantcell granuloma
Normalvariant 7. Fibrousdysplasia(monostotic;polyostotic)
Infection,transplacental
(congenital
syphilis) 8 . Myositisossificans
Neuroblastomametastases 9. "Brownturnor"of hyperparathyroidism
Growthlines 1 0 . Massiveosteolysis
6 Radiology Review Manual

Pseudomalignant Appearance TumorPositionin LongitudinalPlane

1. Osteomyelitis A . E P IP H Y S E A LE
L S ION
2. Aggressiveosteoporosis 1. Chondroblastoma (priorto closureof growthplate)
2. Intraosseous ganglion,subchondral cyst
3. Giantcell tumor(originating in metaphysis)
Patternof Bone Destruction 4. Clearcell chondrosarcoma
A. GEOGRAPHIC BONE DESTRUCTION 5. FibrousdysPlasia
Cause: (a) slow-growing usuallybenigntumor 6. Abscess
"CAGGIE"
(b) rarelymalignant:plasmacell myeloma, mnemonic.'
metastasis Chondroblastoma
(c) infection:granulomatousosteomyelitis Aneurysmalbone cyst
r/ well-definedsmoothi irregularmargin Giantcelltumor
{ short zone of transition Geode
Infection
B. MOTH-EATENBONE DESTRUCTION Eosinophilic granuloma
Cause: (a) rapidlygrowingmalignantbone tumor [after40 years of age throw out "CEA"and
(b) osteomyelitis insertmetastases/ mYeloma]
^/ less well defined/ demarcatedlesionalmargin B. METAPHYSEAL LESION
r/ longerzone of transition 1, Nonossifying fibroma(closeto growthplate)
mnemonic.' "H LEMMON" 2. Chondromyxoidfibroma(abuttinggrowthplate)
HistiocytosisX 3. Solitarybone cyst
Lymphoma 4. Osteochondroma
Ewingsarcoma 5. Brodieabscess
Metastasis 6. Osteogenicsarcoma,chondrosarcoma
Multiplemyeloma C . D IA P H Y S E ALE
L S ION
Osteomyelitis 1. Roundcelltumor(eg, Ewingsarcoma)
Neuroblastoma 2. Nonossifying fibroma
3. Solitarybone cYst
B O N ED E ST R U C T IO N
C. P E RM E AT IV E 4. AneurysmalbonecYst
Cause: aggressivebone tumor with rapidgrowth 5. Enchondroma
potential(eg, Ewingsarcoma) 6. Osteoblastoma
{ poorlydemarcatedlesionimperceptibly merging 7. Fibrousdysplasia
with uninvolvedbone mnemonic.' "FEMALE"
r/ tongzone of transition Fibrousdysplasia
E osi nophi l granul
ic oma
Size, Shape, and Margin of Bone Lesion Metastasis
0 Primarymalignanttumorsare largerthan benign Adamantinoma
tumors Leukemia,LymPhoma
r/ elongatedlesion(= greatestdiameterof >1.5times Ewingsarcoma
the leastdiameter): Ewingsarcoma,histiocytic
lymphoma,chondrosarcoma, angiosarcoma TumorsLocalizingto HematopoieticMarrow
ri scleroticmargin(= r€?ctionof host tissueto tumor) 1. Metastases
2. Plasmacell myeloma
3. Ewingsarcoma
TumorPositionin TransversePlane 4. lymphoma
Histiocytic
A. CENTRALMEDULLARYLESION
1. E nc ho n d ro ma Diffuse BoneMarrow Abnormalities in
2. Solitarybone cyst Childhood
B . E CCE NTR IC L E SION
ME D U L L AR Y A. REPLACEDBY TUMORCELLS
1. G iantc e l ltu m o r (a) metastaticdisease
2. Osteogenicsarcoma,chondrosarcoma, 1. Neuroblastoma (in Youngchild)
fibrosarcoma 2. Lymphoma(in olderchild)
3. Chondromyxoidfibroma 3. Rhabdomyosarcoma (in olderchild)
C. CORTICALLESION (b) primaryneoplasm
1. Nonossifying fibroma 1. Leukemi a
2. Osteoidosteoma B. REPLACEDBY RED CELLS
D. PERIOSTEAL / JUXTACORTICAL LESION = Red cell hyperplasia = reconversion
1. Juxtacortical chondroma/ osteosarcoma (a) severeanemia: sicklecell disease,
2. Osteochondroma thalassemia,hereditaryspherocytosis
3. Parostealosteogenicsarcoma (b) chronicsevereblood loss
 DifferentialDiagnosisof Musculoskeletal
Disorders

(c) marrowreplacement by neoplasia SARCOMASBYAGE:

(d)treatmentwithgranulocyte-macrophage
colony mnemonic; "EveryOtherRunnerFeelsGrampyPain
stimulating
factor On Moving"
C. REPLACED BY FAT Ewingsarcoma 0 -10 years
1. Myeloiddepletion= aplastic
anemia Osteogenicsarcoma 10-30years
D. REPLACED BYFIBROUS TISSUE Reticulumcell sarcoma 20-40 years
1. Myelofibrosis Fibrosarcoma 2040 years
Chondrosarcoma 40-50 years
Parostealsarcoma 40-50 years
Age Incidenceof Malignant Bone Tumors Osteosarcoma 60-70 years
0 80%of bonetumorsarecorrectly
determined
on the Metastases 60-70 years
basisof agealone!
Age [years] Tumor
0.1 Neuroblastoma R OU N DC E LLTU MOR S :
0.1-10 Ewingtumorin tubularbones(diaphysis) { arise in midshaft
10 -30 Osteosarcoma(metaphysis);Ewingtumor r/ osteolyticlesion
in flat bones ! reactivenew bone formation
30 -40 Reticulumcell sarcoma(similarhistology r/ no tumor new bone
to Ewingtumor);fibrosarcoma;malignant mnemonic; "LEMON"
giantcelltumor(similarhistologyto Leukemia,Lymphoma
fibrosarcoma) ; parostealsarcoma; Ewingsarcoma,Eosinophilicgranuloma
lymphoma Multiplemyeloma
>40 Metastaticcarcinoma;multiplemyeloma; Osteomyelitis
chondrosarcoma Neuroblastoma

Osteosarcoma Metastases
Chordoma

Myelomq ignant fibrous histiocytoma

Chondrosarcoma Extraosseoussarcoma
Lymphoma

Adamantinoma

Osteosarcoma

Ewing sarcoma
Neuroblastoma

Age
10 20 30

Fibrous cortical defect, Eosinophilic granuloma

ABcrc\Cortical desmoid, Nonossifying fibroma, Desmoplastic fibroma, Chondroblastoma

Osteoblastoma Osteoid osteoma, Chondromyxoid fibroma, Enchondroma

Giant cell tumor

Average Age for Occurrence of Benign and Malignant Bone Tlrmors

8 Radiology Review Manual

MALIGNANCYWITH SOFT-TISSUEINVOLVEMENT Tumors of Fatty Tissue Origin

mnemonic.' "My MotherEats ChocolateFudgeOften" A . B E N IGN
Metastasis 1. Intraosseous liPoma
Myeloma 2. Parosteallipoma
Ewingsarcoma B. MALIGNANT
Chondrosarcoma 1. Intraosseous liPosarcoma
Fibrosarcoma 0 Lipomasfollowthe signalintensityof subcutaneous
Osteosarcoma fat in all sequences!

TumorMatrixof BoneTumors Tumors of VascularOrigin

Cartilage-forming Bone Tumors <1o/oof all bonetumors
{ centrallylocatedringlike/ flocculent/ flecklike A. BENIGN
radiodensities 1. H emangi oma
A. BENIGN 2. Gl omustumor
1. E nc h o n d ro m a 3. Lymphangioma
2. Parostealchondroma 4. Cysticangiomatosis
3. Chondroblastoma 5. Hemangiopericytoma
4. Chondromyxoid fibroma B . MA LIGN A N T
5. Osteochondroma 1. MalignanthemangioPericYtoma
B. MALIGNANT = hemangioendothelioma
2. AngiosarcolT't€l
1. Chondrosarcoma Metastaticsites: lung,brain,lymphnodes,
2. Chondroblastic osteosarcoma other bones

Bone-torming Tumors Tumors of Neural Origin

r/ inhomogeneous / homogeneous radiodense A. BENIGN
collectionsof variablesize + extent 1. Solitaryneurofibroma
A . B E NI G N 2. N euri l emoma
1. O s t e o ma B. MALIGNANT
2. Osteoidosteoma 1. Neurogenicsarcoma= malignantschwannoma
3. Osteoblastoma
4. Ossifyingfibroma
B. MALIGNANT INTRAOSSEOUS LESION
1. Osteogenicsarcoma
Bubbly Bone Lesion
Fibrous Connective Tissue Tumors mnemonic; "FOGMACHINES"
A . B E NI G NF IBR O U SBON EL ES IO N S Fibrousdysplasia,Fibrouscorticaldefect
(a) cortical Osteoblastoma
1. Benigncorticaldefect Giantcelltumor
2. Avulsioncorticalirregularity Myeloma(plasmacytoma), Metastasesfrom kidney,
(b) medullary thyroid,breast
1. H e rn i a ti opni t AneurysmalbonecYst/ Angioma
2. Nonossifying fibroma Chondromyxoid f ibroma,Chondroblastoma
3. Ossifyingfibroma HistiocytosisX, Hyperparathyroid browntumor,
4. Congenitalgeneralized fibromatosis H emophi l i a
(c) corticomedullary lnfection(Brodieabscess,Echinococcus,
1. Nonossifying fibroma coccidioidomycosis)
2. Ossifyingfibroma Nonossifying fibroma
3. Fibrousdysplasia Enchondroma, Epithelialinclusioncyst
4. Cherubism SimpleunilocularbonecYst
5. Desmoplasticfibroma
6. Fibromyxoma InfectiousBubbly Lesion
B. MALIGNANT 1. Brodieabscess(StaPh.aureus)
1. Fibrosarcoma 2. Coccidioidomycosis
3. Echinococcus
Tumorsof HistiocyticOrigin 4. Atypicalmycobacterium
A. LOCALLYAGGRESSIVE 5. Cystictuberculosis
1 . G i a n tc e l l t u m o r
2. Benignfibroushistiocytoma Blowout Lesion
B. MALIGNANT A. METASTASES
1. Malignantfibroushistiocytoma Carcinomaof thyroid,kidney,breast
 Disorders g
DifferentialDiagnosisof Musculoskeletal

B. PRIMARY BONETUMOR Mixed Sclerotic and Lytic Lesion

1. Fibrosarcoma A. WITHSEQUESTRUM: osteomyelitis
2. Multiple (sometimes)
myeloma B. WITHOUT SEQUESTRUM:
3. Aneurysmal
bonecyst 1. Osteomyelitis
4. Hemophilic
pseudotumor 2. Tuberculosis
3. Ewingsarcoma
NonexpansileUnilocularWell-demarcatedBone 4. Metastasis
5. Osteosarcoma
Defect
1. Fibrouscorticaldefect
Trabeculated Bone Lesion
2. Nonossifying fibroma
1. Giantcelltumor:delicate thintrabeculae
3. Simpleunicameral bonecyst 2. Chondromyxoid fibroma:coarsethicktrabeculae
4. Giantcelltumor
3. Nonossifyingfibroma:lobulated
5. Browntumorof HPT
4. Aneurysmal bonecyst: delicate, horizontally
6. Eosinophilicgranuloma
orientedtrabeculae
7. Enchondroma
5. Hemangioma: striatedradiating
trabeculae
8. Epidermoid inclusion
cyst
9. Posttraumatic / degenerativecyst
10. Pseudotumor of hemophilia
11. Intraosseous ganglion Lytic Bone Lesion Surrounded by Marked
12. Histiocytoma Sclerosis
13. Arthritic
lesion mnemonic.'"BOOST"
14. Endosteal pigmented villonodular
synovitis Brodieabscess
15. Fibrousdysplasia Osteoblastoma
16. Infectiouslesion Osteoidosteoma
Stressfracture
Tuberculosis
Nonexpansile Multilocular Well-demarcated
Bone Defect
1. Aneurysmal Multiple Lytic Lesions
bonecyst
2. Giantcelltumor mnemonic.'"FEEMHI"
3. Fibrousdysplasia Fibrousdysplasia
4. Simplebonecyst Enchondromas
Eosinophilic granuloma
Expansile Unilocular Well-demarcatedOsteolysis Metastases, Multiplemyeloma
Hyperparathyroidism (browntumors),Hemangiomas
1. Simpleunicameralbonecyst Infection
2. Enchondroma
3. Aneurysmal bonecyst
4. Juxtacortical Multiple Lytic Lesions in Child
chondroma
5. Nonossifyingfibroma 1. Histiocytosis
X
6. Eosinophilicgranuloma 2. Metastaticneuroblastoma/ leukemia
7. Browntumorof HPT 3. Fibrousdysplasia
4. Enchondromatosis
Poorly DemarcatedOsteolytic Lesion without 5. Rare:cysticangiomatosis,multifocal
osteomyelitis
Periosteaf Reaction
A. NONEXPANSILE Lytic Bone Lesion in Patient <30 Years of Age
1. Metastasesfromanyprimaryneoplasm mnemonic; "CAINES"
2. Multiple
myeloma Chondroblastoma
3. Hemangioma Aneurysmal bonecyst
B. EXPANSILE Infection
1. Chondrosarcoma Nonossifyingfibroma
2. Giantcelltumor granuloma
Eosinophilic
3. Metastasisfromkidney/ thyroid Solitarybonecyst

Poorly DemarcatedOsteolytic Lesion with Lytic Bone Lesion on Both Sides of Joint
Periosteal Reaction mnemonic.' "SAC"
1. Osteomyelitis Synovioma
2. Ewingsarcoma Angioma
3. Osteosarcoma lesion
Chondroid
 MultipleBoneLesions& Soft-tissueTumor (b) with predominant axialinvolvement:
1. Neurofibromatosis& fibroxanthomas vertebralsegmentationdefects(Klippel-Feil),
2. Mafuccisyndrome= enchofldromatosis& Sprengelanomaly,spondylocostal dysostosis,
hemangioma oculovertebralsyndrome
3. Mazabraudsyndrome= fibrousdysplasia& myxoma (c) with predominant involvement of extremities:
4. Metastases acheiria(= absenceof hands),apodia(= absence
(a) Multiplemyeloma of feet),polydactyly,syndactyly,camptodactyly,
(b) Malignantmelanoma Rubinstein-Taybisyndrome,pancytopenia-
(c) Lymphoma dysmeliasyndrome(Fanconi),Blackfan-Diamond
anemiawith thumbanomaly,thrombocytopenia-
OsteoblasticBoneLesion radialaplasiasyndrome,cardiomelicsyndromes
A. BEN]GN (Holt-Oram),focalfemoraldeficiency,multiple
1. B oneis l a n d synostoses
2. Osteoma (3) TDToPATHIC OSTEOLYSIS
= disorderswith multifocalresorptionof bone
3. Osteoidosteoma
B. MALIGNANT (4) CHROMOSOMALABERRATION
1. Osteosarcoma (5) P R TMA R ME
Y TA B OLIC D IS OR D E R
2. Parostealsarcoma (a) calcium/ phosphorus:hypophosphatasia
(b) complexcarbohydrates:mucopolysaccharidosis
WidespreadOsteoscleroticLesions
Terminology:
1. Metastases:prostate,breast,lung,bladder,
Micromelia = shorteninginvolvesentirelimb
pancreas,stomach,colon,carcinoid,brain
(eg,humerus,radius+ ulna,hand)
2. Pagetdisease
Rhizomelia = shorteninginvolvesproximalsegment
3. Sarcoma
(eg,humerus)
4. Myelofibrosis
Mesomelia = shorteninginvolvesintermediate
5. Mastocytosis
segment(eg, radius+ ulna)
Acromelia = shorteninginvolvesdistalsegment
DWARFISM (eg,hand)
Classification:
(1) OSTEOCHON DRODYSPLASIA Micromelic Dwarfism
= abnormalitiesof cartilage/ bone growthand = disproportionateshorteningof entireleg
development A. Mildmicromelicdwarfism
(a) identifiableat birth: 1. Jeunesyndrome
- usuallylethal: achondrogenesis, 2. Ellis-vanCreveldsyndrome
fibrochondrogenesis, thanatophoricdysplasia, = chondroectodermal dysplasia
shortrib syndrome 3. Diastrophicdwarfism
- usuallynonlethal:chondrodysplasia punctata, B. Mild bowed micromelicdwarfism
camptomelic dysplasia,achondroplasia, 1. Camptomelic dysplasia
diastrophicdysplasia,chondroectodermal 2. Osteogenesisimperfecta,type lll
dysplasia,Jeunesyndrome, C. Severemicromelicdwarfism
spondyloepiphyseal dysplasiacongenita, 1. Thanatophoric dysplasia
mesomelicdysplasia,cleidocranial dysplasia, 2. Osteogenesisimperfecta,type ll
oto-palato-digital syndrome 3. HomozygousachondroPlasia
(b) identifiablein laterlife: hypochondroplasia, 4. Hypophosphatasia
dyschondrosteosis, spondylometaphyseal 5. Short-ribpolydactylysyndrome
dysplasia,acromicricdysplasia 6. Fibrochondrogenesis
(c) abnormalbonedensity:osteopetrosis,
pyknodysostosis, Melnick-Needles syndrome AcromelicDwarfism
(2) DYSOSTOSIS = distalshortening(hands,feet)
= malformation of individualbonessingly/ in 1. Asphyxiatingthoracicdysplasia
combination
(a) with cranial+ facialinvolvement: Rhizomelic Dwarfism
craniosynostosis, craniofacialdysostosis = shorteningof proximalsegments(humerus,femur)
(Crouzon),acrocephalosyndactyly, mnemonic.' "MA CAT"
acrocephalopolysyndactyly, branchialarch Metatrophicdwarfism
syndromes(Treacher-Collins, Franceschetti, Achondrogenesis(mostsevereshortening)
acrofacialdysostosis,oculo-auriculo-vertebral Chondrodysplasia punctata(autosomalrecessive)
dysostosis,hemifacialmicrosomia,oculo- Thanatophoricdysplasia
mandibulo-facial syndrome Achondroplasia,heterozygous
 DifferentialDiagnosisof Musculoskeletal
Disorders l1

Osteochond
rodysplasia LargeHeadin Fetus
A. Failureof 1. Achondroplasia
(a) articularcartilage:spondyloepiphyseal dysplasia 2. Thanatophoric
dysplasia
(b) ossificationcenter: multipleepiphysealdysplasia
(c) proliferatingcartilage: achondroplasia NarrowChestin Fetus
(d) spongiosaformation: hypophosphatasia polydactyly
1. Short-rib syndrome
(e) spongiosaabsorption:osteopetrosis 2. Asphyxiating
thoracic
dysplasia
(f) periostealbone: osteogenesisimperfecta 3. Chondroectodermaldysplasia
(g) endostealbone: idiopathicosteoporosis 4. Camptomelicdysplasia
B. Excessof 5. Thanatophoricdwarfism
(a) articularcartilage:dysplasiaepiphysealis 6. Homozygousachondroplasia
hemimelica 7. Achondrogenesis
(b) hypertrophiccartilage:enchondromatosis 8. Hypophosphatasia
(c) spongiosa: multipleexostosis
(d) periostealbone:progressivediaphysealdysplasia Platyspondyly
(e) endostealbone: hyperphosphatemia
1. Thanatophoricdysplasia
2. Osteogenesisimperfectatype ll
3. Achondroplasia
Lethal Bone Dysplasia 4. Morquiosyndrome
in orderof frequency
1. Thanatophoric dysplasia BowedLong Bonesin Fetus
2. Osteogenesis imperfecta typell 1. Campomelicsyndrome
3. Achondrogenesis type| + ll 2. Osteogenesisimperfecta
4. Jeunesyndrome (maybe nonlethal) Thanatophoricdysplasia
3.
5. Hypophosphatasia, congenital lethalform 4. Hypophosphatasia
6. Chondroectodermal dysplasia (usuallynonlethal)
7. Chondrodysplasia punctata, rhizomelictype Bone Fracturesin Fetus
8. Camptomelicdysplasia
1. Osteogenesisimperfecta
9. Short-rib polydactyly syndrome 2. Hypophosphatasia
10. Homozygous achondroplasia
3. Achondrogenesis
0 Lethalshort-limbed dysplasias typically
aremanifest
on sonograms before24 weeksMA!
LIMBREDUCTION
ANOMALIES
Nonlethal Dwarfism Amelia = absenceof limb
1. Achondroplasia
(heterozygous) Hemimelia = absenceof distalparts
2. Asphyxiating Phocomelia = proximalreductionwith distalparts
thoracicdysplasia
3. Chondroectodermaldysplasia attachedto trunk
4. Chondrodysplasia
punctata
5. Spondyloepiphyseal
dysplasia(congenital) Aplasia/ Hypoplasiaof Radius
6. Diastrophic
dwarfism mnemonic; "The Furry Cat Hit My Dog"
7. Metatrophic
dwarfism Thrombocytopenia-absent radiussyndrome
8. Hypochondroplasia Fanconianemia
Gorneliade Langesyndrome
Holt-Oramsyndrome
Late-onset Dwarfism Myositisossificansprogressiva(thumbonly)
1. Spondyloepiphyseal
dysplasia
tarda Diastrophicdwarfism("hitchhiker's
thumb")
2. Multiple
epiphyseal
dysplasia
3. Pseudoachondroplasia PubicBoneMaldevelopment
4. Metaphysealchondrodysplasia mnemonic.' "CHlEF"
5. Dyschondrosteosis Gleidocranialdysostosis
6. Cleidocranial
dysostosis Hypospadia,epispadia
7. Progressive
diaphyseal
dysplasia ldiopathic
Exstrophyof bladder
F for syringomyelia
Hypomineralizationin Fetus
A. DIFFUSE
1. Osteogenesis
imperfecta BONEOVERGROWTH
2. Hypophosphatasia BoneOverdevelopment
B. SPINE 1. Marfansyndrome
1. Achondrogenesis 2. Klippel-Trenaunay
syndrome
 "hair-on-end":Ewing
macrodactyly
3. Nerveterritory-oriented (d) perpendicular spicules=
(a) Macrodystrophialipomatosa sarcoma
(b) Fibrolipomatoushamartomawith macrodactyly (e) amorphous: malignancy(depositsmay represent
extensionof tumor / periostealresponse);
Erlenmeyer Flask Deformity osteosarcoma
= eXpohsion of distalend of longbones,usuallyfemur (f) Codmantriangle:hemorrhage; malignancy
1. Gaucherdisease,Niemann-Pick disease (osteosarcoma, Ewingsarcoma);acute
2. Anemia: thalassemia, sicklecell osteomyelitis; f racture
3. Osteopetrosis
4. Heavy metalpoisoning
5. Metaphysealdysplasia= Pyle disease SymmetricPeriostealReactionin Adulthood
6. Rickets (lower
stasis
1. Venous extremity)
7. Fibrousdysplasia 2. Hypertrophicosteoarthropathy
8. Down syndrome 3. Pachydermoperiostosis
9. Achondroplasia 4. Thyroidacropachy
10. Rheumatoidarthritis 5. Fluorosis
11. Hypophosphatasia 6. Rheumatoidarthritis
7. Psoriaticarthritis
mnemonic; "TOP DOG" 8. Reitersyndrome
Thalassemia 9. ldiopathic-degenerative
Osteopetrosis
Pyle disease
Diaphysealaclasis Periosteal Reaction in Childhood
Ollierdisease (a) benign
Gaucherdisease 1. Physiologic(up to 35%): symmetricinvolvement
of diaphysesduringfirst 1-6 monthsof life
2. Nonaccidentaltrauma= batteredchild syndrome
REACTION
PERIOSTEAL 3 lnfantilecorticalhyperostosis:<6 monthsof age
1. T r aum an
hemo p h i l i a 4. Hypervitaminosis A
2. Infection 5. ScurvY
3. Inflammatory:arthritis 6. OsteogenesisimPerfecta
4. Neoplasm 7. CongenitalsyPhilis
5. Congenital:physiologic in newborn (b) malignant
6. thyroid
Metabolic: hypertrophicosteoarthropathy, osteosarcoma
1. Multicentric
acropachy,hyPervitaminosisA 2. Metastasesfrom neuroblastoma+ retinoblastoma
7. Vascular: venousstasis 3. Acute leukemia

Solid PeriostealReaction "PERIOSTEALSOCKS"

mnemonic:
= reactionto periostealirritant Physiologic,Prostaglandin
{ even + uniformthickness>1 mm Eosinophilic granuloma
r/ persistent+ unchangedfor weeks Rickets
Pafterns: InfantilecorticalhYPerostosis
granuloma,osteoidosteoma
(a) thin: eosinophilic . Osteomyelitis
(b) denseundulating:vasculardisease Scurvy
(c) thin undulating:pulmonaryosteoarthropathy Trauma
(d) denseelliptical:osteoidosteoma;long-standing Ewingsarcoma
malignantdisease(withdestruction) A-hypervitaminosis
(e) cloaking: storagedisease;chronicinfection Leukemia+ neuroblastoma
Syphilis
Osteosarcoma
InterruptedPeriostealReaction Childabuse
= pleomorphic,rapidlyprogressingprocessundergoing Kinky hair syndrome
constantchange Sicklecelldisease
(a) buttressing= periostealbone formationmergeswith
underlyingcortex: eosinophilicgranuloma
(b) laminated= "onionskin": acuteosteomyelitis; PeriostealReactionin Infant
malignanttumor (osteosarcoma,Ewingsarcoma) before6 monthsof age
(c) radiatingspicules= "sunburst":osteosarcoma; 1. Infantilecorticalhyperostosis
Ewing sarcoma;chondrosarcoma; f ibrosarcoma; 2. Physiologic
leukemia;metastasis;acuteosteomyelitis 3. Extracorporeal membraneoxygenation
 DifferentialDiagnosisof Musculoskeletal
Disorders 13

after 6 monthsof age Epiphyseal/ ApophysealLesion

1. Hypervitaminosis A 1. Chondroblastoma
2. Scurvy 2. Brodieabscess
3. Rickets 3. Fungal/ tuberculousinfection
anytimeduringinfancy 4. Langerhanscell histiocytosis
1. Nonaccidental trauma 5. Osteoidosteoma
2. Syphilis 6. Chondromyxoidfibroma
3. Metastaticneuroblastoma / leukemia 7. Enchondroma
4. Prostaglandin therapy: within40 days 8. Bonecyst
5. Sicklecell dactylitis 9. Foreign-bodygranuloma
DDx: motion artifact
Subarticular Lesion
Enthesopathy 1. G i a n tc e l l t u m o r
Enthesis= osseousattachmentof tendoncomposedof 2. Solitarysubchondralcyst
4 zones,ie, tendonitself+ unmineralized 3. ltraosseous ganglion
fibrocartilage+ mineralizedfibrocartilage+ bone 4. Brodieabscess
Cause: 5. Clearcell chondrosarcoma
1. Degenerative disorder
2. Seronegativearthropathies:ankylosing StippledEpiphyses
spondylitis,Reiterdisease,psoriaticarthritis 1. Normalvariant
3. Diffuseidiopathicskeletalhyperostosis 2. Avascularnecrosis
4. Acromegaly 3. Hypothyroidism
5. Rheumatoidarthritis(occasionally) 4. Chondrodysplasia punctata
Location: at site of tendon+ ligamentattachment 5. Multipleepiphysealdysplasia
r/ bone proliferation(enthesopnyte) 6. Spondyloepiphyseal dysplasia
{ calcificationof tendon+ ligament 7. Hypoparathyroidism
r/ erosion 8. Down syndrome
9. Trisomy18
10. Fetalwarfarinsyndrome
BONETRAUMA 11. Homocystinuria (distalradial+ ulnarepiphyses=
ChildhoodFractures pathognomonic)
1. Greenstickfracture 12. Zellwegercerebrohepatorenal syndrome
2. Bowingfracture
3. Traumaticepiphyseolysis Physeal/ MetaphysealWidening& lrregularity
4. Batteredchild syndrome 1. Rickets
5. Epiphysealplate injury 2. Hypophosphatasia
3. Metaphysealchondroplasia
Pseudarthrosis
in Long Bones
1. Nonunionof fracture EpiphysealOvergrowth
2. Fibrousdysplasia 1. Juvenilerheumatoidarthritis
3. Neurofibromatosis 2. H emophi l i a
4. Osteogenesisimperfecta 3. HealedLegg-Perthesdisease
5. Congenital:clavicularpseudarthrosis 4. Tuberculousarthritis
5. Pyogenicarthritis(chronic)
ExuberantCallusFormation 6. Fungalarthritis
1. Steroidtherapy/ Cushingsyndrome 7. Epiphysealdysplasiahemimelica
2. Neuropathicarthropathy 8. Fibrousdysplasiaof epiphysis
3. Osteogenesisimperfecta 9. Winchestersyndrome
4. Congenitalinsensitivity
to pain
5. Paralysis Epiphyseolysis
6. Renalosteodystrophy = SLIPPEDEPIPHYSIS(zoneof maturinghypertrophic
7. Multiplemyeloma cartilageaffected,not zone of proliferation)
8. Batteredchild syndrome 1. ldiopathici juvenileepiphyseolysis
Age: 12-15 years (? puberty-related hormonal
dysregulation)
EPIPHYSIS . adiposogenital type; tall stature
PrematureEpiphysealOssification 2. Renalosteodystrophy
@ Proximalfemoraland humeralepiphyses 3. Hyperparathyroidism in chronicrenaldisease
1. Jeuneasphyxiating thoracicdysplasia 4. Hypothyroidism
2. Ellis-vanCreveldchondroectodermaldysplasia 5. Fladiotherapy
 Radiology Review Manual

JOINTS F. ENTEROPATHIC ARTHROPATHY

Approach to Arthritis (a) INFLAMMATORY BOWELDISEASE
mnemonic; "ABCDE'S" 1. Ulcerative colitis(in 10-20%)
A lignm ent 2. Crohndisease(in 5%): peripheralarthritis
Bone mineralization increaseswith colonicdisease
Cartilageloss 3. Whippledisease(in 60-90% transient
intermittent polyarthritis:
sacroiliitis,
Distribution
Erosion spondylitis)
Soft tissues 0 Resectionof diseasedbowel is associatedwith
regressionof arthriticsymptomatology!
(b) TN FE C TIOUBSOW E LD IS E A S E
Signs of Arthritis
Infectiousagents: Salmonella, Shigella,Yersinia
Prevalenceof arthritis: 15'/. of populationin USA
(c) after intestinalbypasssurgery
Conventional x-ray:
r/ narrowingof radiologicjoint space SPONDYLARTHRITIS + positiveHLA-B27
(a) uniform= inflammatory arthritis HISTOCOMPATIBI LITYCOMPLEX
(b) nonuniform= degenerative arthritis 1. A nkyl osi ng spondyl i ti s . . . . . 95/ o
r/ evidenceof diseaseon both sidesof joint: 2. Reiterdisease .80%
r/ osteopenia 3. Arthropathy of inflammatory boweldisease....75%
{ subchondral sclerosis 4. P sori atispondyl
c i ti s . . . . . . . . 70%
{ erosion 5 . N o r m apl o p u l a t i o n ...........10%
{ subchondralcyst formation
{ m alali g n m e n t Monoarthritis
i/ loint effusion DestructiveMonoarthritis
r/ loint bodies 0 Any destructivemonoarthritisshouldbe regarded
NUC: as infectionuntilprovedotherwise!
^/ increasein regionalbloodflow (activedisease)
A. Septicarthritis
r/ distributionof disease B. Monoarticularpresentationof a systemicarthritis
MR: 1. Rheumatoid arthritis
r/ irregularity+ narrowingof articularcartilage 2. Gout
./ OO-OfpAenhancementof synovium(activedisease)
3. Amyloidosis
4. Seronegativearthritis
Classificationof Arthritides C. Jointtumor
A . S E P T I CAR T H R IT IS 1. PVNS
1. T uber cu l o u s 2. Synovialchondromatosis
2. Pyogenic 3. A rti cul arhemangi oma
3. Lyme arthritis
4. Fungalarthritis:Candida,Coccidioides immitis, Non septic Monoarth ritis
Blastomycesdermatitidis,Histoplasma 1. Gout
capsulatum,Sporothrixschenckii,Cryptococcus 2. Milwaukeeshoulder
neoformans, Aspergillus f umigatus 3. Rapidlydestructivearticulardisease
N.B.: Tuberculous + fungalarthritisshows 4. Amyloidarthropathy
prominentosteoporosis+ slowerrate of Hemophilicarthropathy
5.
destruction + lessjoint narrowingthan a 6. Primarysynovialosteochondromatosis
pyogenicinfection(Phemistertriad)
7. Pigmentedvillonodular synovitis
B. COLLAGEN/ COLLAGEN-LIKE DISEASE NeuropathicarthroPathY
8.
1. Rheumatoid arthritis 9. Foreign-bodysynovitis
2. Ankylosingspondylitis
3. Psoriaticarthritis ArthritiswithoutDemineralization
4. Rheumaticfever
1. Gout
5. Sarcoidosis
2. Neuropathicarthropathy
C. B I O CHE M IC AAR L T H R IT IS
3. Psoriasis
1. Gout
4. Reiterdisease
2. Chondrocalcinosis
5. Pigmentedvillonodularsynovitis
3. Ochronosis
4. Hemophilicarthritis mnemonic.' "PONGS"
D. DEGENERATIVE JOINT DISEASE= Osteoarthritis Psoriaticarthritis
E. TRAUMATIC Osteoarthritis
1. Secondaryosteoarthritis Neuropathic joint
2. Neurotrophicarthritis Gout
3. Pigmentedvillonodularsynovitis Sarcoidosis
 Differential Diagnosis of Musculoskeletal Disorders 15

Arthritiswith Demineralization SubchondralCyst

mnemonic; "HORSE" = SYNOVIAL CYST= SUBARTICULAR PSEUDOCYST
Hem ophilia = NECROTIC PSEUDOCYST = GEODES
Osteomyelitis Etiology: bone necrosisallowspressure-induced
Rheumatoidarthritis,Reiterdisease intrusionof synovialfluidintosubchondral
Scleroderma bone;in conditions withsynovialinflammation
Erythematosrrs,
systemiclupus Cause: 1. Osteoarthritis
2. Rheumatoid arthritis
DeformingNonerosiveArthropathy 3. Osteonecrosis
1. Collagen-vasculardisease,especiallySLE 4. CPPD
2. Rheumatoidarthritis(rare) { size of cyst usually2-35 mm
3. Rheumaticfever (Jaccoudarthritis)(rare) ^/ may be large+ expansile(especially in CPPD)
DDx: (1) Giantcelltumor
(2) Pigmentedvillonodular synovitis
Arthritiswith Periostitis (3) Metastasis
1. Juvenilerheumatoidarthritis (4) Intraosseous ganglion
2. Psoriaticarthritis (5) Hemophilia
3. Reitersyndrome
4. lnfectiousarthritis LooseIntraarticular
Bodies
1. Osteochondrosisdissecans
2. Synovialosteochondromatosis
PrematureOsteoarthritis 3. Chipfracturefromtrauma
mnemonic; "COMECHAT" 4. Severedegenerativejoint disease
Calciumpyrophosphatedihydratearthropathy 5. Neuropathicarthropathy
Ochronosis
Marfansyndrome Intraarticular
Processwith CorticalErosion
Epiphysealdysplasia 1. Pigmentedvillonodular
synovitis
Charcotjoint = neuroarthropathy 2. Synovialosteochondromatosis
Hemophilicarthropathy 3. Rheumatoidarthritis
Acromegaly 4. Gout
Trauma 5. Synovialhemangioma
6. Lipomaarborescens
Synovial Disease with Decreased Signal Intensity
= hemosiderindeposition Erosionsof DIP Joints
1. Pigmentedvillonodular
synovitis 1. Inflammatory osteoarthritis
2. Rheumatoidarthritis 2. Psoriaticarthritis
3. Hem ophili a 3. Gout
4. Multicentricreticulohistiocytosis
5. Hyperparathyroidism
Chondrocalcinosis 6. Frostbite
mnemonic; "WHIPA DOG" 7. Septicarthritis
Wilsondisease
Hemochromatosis, Hemophilia, 1o
Hypothyroidism,
Hyperparathyro idism (15/"), Hypophosphatasia, ArticularDisordersof Handand Wrist
FamilialHypomagnesemia 1. Osteoarthritis = degenerative jointdisease
ldiopathic(aging) = abnormalstresswith minor+ majortraumatic
Pseudogout(CPPD) episodes
Afthritis(rheumatoid,postinfectious,
traumatic, Targetareas DlP, PlP, 1st CMC,trapezioscaphoid;
degenerative),Amyloidosis, Acromegaly bilateralsymmetric/ asymmetric
Diabetesmellitus r/ ioint space narrowing
Ochronosis r/ subchondral eburnation
Gout r/ marginalosteophytes+ smallossicles
r/ radialsubluxationof 1st metacarpalbase
mnemonic.' "3 C's" 0 Radiocarpaljoint normalunlesshistoryof trauma
Crystals CPPD, 2. Erosiveosteoarthritis = inflammatoryosteoarthritis
sodiumurate (gout) Age: predominantly middle-aged /
Cations calcium(any cause of postmenopausal women
hypercalcemia), copper,iron . acute inflammatoryepisodes
Cartilagedegeneration osteoarthritis,
acromegaly, Targetareas: DlP, PlP, 1stCMC,trapezioscaphoid;
ochronosis bilateralsymmetric/ asymmetric
16 RadiologyReviewManual

r/ centralerosionscombinedwith osteophytes 5 . Goutyarthritis

= subchondral"gullwing"erosions
r/ loint space narrowing+ sclerosis
r/ rare ankylosis
3 . Psoriaticarthritis
= rheumatoidvariant/ seronegative
spondyloarthropathy; peripheralmanifestationin
monarthritis/ asymmetricoligoarthritis / symmetric
polyarthritis
Targetareas: all hand + wristjoints (commonly
distal);bi- / unilateralasymmetric
polyarticularchanges
{ "mouseears"marginalerosions
r/ intraarticularosseousexcrescences
^/ new boneformation+ fusion
r/ osteoporosismay be absent
4. Rheumatoidarthritis
= synoVialproliferativegranulationtissue= pannus
Targetareas: PIP (earlyin 3rd),MCP (earliest
changesin 2nd + 3rd),all wristjoints
(earlyin RC, IRU),ulnarstyloid;both
handsin relativesymmetricfashion
r/ fusiformsoft-tissueswelling
r/ regionalperiarticularosteoporosis
r/ diffuseloss of joint space
ri marginal+ centralpoorlydefinederosions
^/ loint deformities
. monosodiumuratecrystalsin synovialfluid
. asymptomaticperiodsfrom monthsto years
Targetareas: commonlyCCMC + all handjoints
{ developmentof chronictophaceousgout
= lobulatedsoft-tissuemasses
^/ well-definedeccentricerosionswith overhanging
edge (oftenperiarticular) + scleroticmargins
r/ preservationof joint spaces
r/ absenceof osteoporosis
r/ most extensivechangesin common
carpometacarpal compartment:
{ scallopederosionsof basesof ulnar
metacarpals
6 . Calciumpyrophosphatedihydratecrystaldeposition
disease= CPPD
Targetareas: MCP (2nd,3rd), radiocarpal;bilateral
symmetrici asymmetricchanges
{ chondrocalcinosis + periarticular
calcifications:
{ calcificationof triangularfibrocartilage
{ "degenerativechanges"in unusuallocations:
{ narrowing+ obliterationof space betweendistal
radiusand scaphoidt fragmentationof surfaces
r/ scapholunateseparation
{ destructionof trapezioscaphoid space
{ no erosions
r/ + largeosteophytes= hemochromatosis

,,,':i,,'r],i:i
i tJ illu \
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oif]t.\.-
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.i ''J'u"',"illl
f\ €i |
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\<el
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i:?t\ \3\ \=ft\
t t i

Rheumatoid Degenerative Inflammatory

Arthritis Joint Disease Osteoarthritis

,,il0rli^0ii]1:i^tFtfin,
i:l:I)]"oi
,['! .,.J
Ii:
*'l:u:i;rl
'Li 'l)=j $*,
CCMC = common
Articulationsof Hand and Wrist
MC = midcarpal
"-e\ r(\ i*8\
carpometacarpal (trapezioscaphoidregion CPPD Crystal Psoriatic Gouty
CMC = first carpometcarpal separatedby vertical line) DepositionDisease Arthritis Arthritis
DIP = distal interphalangeal MCP = metacarpophalangeal
IRU = inferior radioulnar PIP = proximal interphalangeal Distribution Pattern of Arthritic Lesions
RC = radiocarpal (adapted from Donald Resnick, M.D.)
 DifferentialDiagnosisof Musculoskeletal
Disorders 1 7

7. SLE B . BILATERALASYMMETR]C
= myositis,symmetricpolyarthritis, deforming 1. Psoriaticarthritis
nonerosivearthropathy,osteonecrosis r/ largeextensiveerosion
Targetareas: PlP, MCP r/ subchondralsclerosis+ occasionalankylosis
r/ reversibledeformities 2. Reitersyndrome
8. ScleroderrT't?= progressivesystemicsclerosis(pSS) 3. Juvenilerheumatoidarthritis
Targetareas: DlP, PlP, 1st CMC c. U N ILA TE R A L
! tuft resorption 1. Infecti on
r/ soft-tissuecalcifications 2. Osteoarthritis from abnormalmechanicalstress
r/ no erosions
r/ irregularnarrowingof joint spacewith
ArthritisInvolvingDistallnterphalangeal
Joints subchondralsclerosis
mnemonicr"POEM" r/ osteophytesat anterosuperiorl-inferioraspect
Psoriaticarthritis of joint (may resembleankylosis)
Osteoarthritis DDx: psoriaticarthritis,Reitersyndrome,trauma,
Erosiveosteoarthritis gout,pigmentedvillonodular synovitis,osteitis
Multicentricreticulohistiocytosis condensansilii

Ankylosis of Interphalangeal Joints Sacroiliac Joint Widening

mnemonic.' "S - Lesions" mnemonic; "CRAPTRAP"
1. Psoriaticarthritis Colitis
2. Ankylosingspondylitis Rheumatoidarthritis
3. Erosiveosteoarthritis Abscess(infection)
4. $till disease Parathyroiddisease
Trauma
Reitersyndrome
Sacroiliitis Ankylosing spondylitis
Joint anatomy: Psoriasis
ligamentous portion(superior213to 112of joint):
formedby interosseous sacroiliacligament
synovialcomponent(inferior1/3 to 112of joint): SacroiliacJoint Fusion
the sacralsurfaceis linedby 3-5 mm-thickhyaline mnemonic.' "CARPl"
cartilage;the iliacsurfaceof jointis linedby 1- Goliticspondylitis
mm-thickfibrocartilage Ankylosingspondylitis
2-5 mm normaljointwidth Reitersyndrome
Positioning; obliqueview + modifiedFergusonview = Psoriaticarthritis
AP projectionwith 23. cephaladangulation Infection(TB)

r/ findingspredominate on the iliacside(thinnercartilage)

A. BILATERALSYMMETRIC
1. Ankylosingspondylitis
! smallregularerosion= lossof definitionof DifferentialDiagnosisof SacroiliacJoint Disease
whitecorticallineon iliacside (initially) Osteoarthritis Ankylosing Osteitis
r/ subchondralsclerosis+ subsequentankylosis spondylitis condensans
r/ ossificationof interosseousligaments itii
2. Enteropathicarthropathy
^/ same signsas in ankylosingspondylitis Age older younger younger
3. Rheumatoidarthritis(in latestages) Sex M, F M>F F>M
! loint space narrowingwithoutreparation Distribution bi- / unilateral bilateral bilateral
ri osteoporosis symmetric symmetric
r/ ankylosismay occur
4. Depositionarthropathy:gout,CPPD,ochronosis, Sclerosis iliac iliac iliac
acromegaly mild,focal + extensive triangular
{ slow loss of cartilage Erosions absent common absent
! subchondralreparativebone + osteophytes Intraarticular
5. Osteitiscondensansilii ankylosis rare common absent
DDx: Hyperparathyroidism (subchondralbone
resorptionon iliacside resemblingerosion+ Ligamentous
wideningof joint) ossification lesscommon common absent
 Radiology Review Manual

Widened Symphysis Pubis 2. Forked/ bifidrib (0.6%)= duplication of anterior

mnemonic.' "EPOCH" portion
Exstrophyof the bladder Location: 4th rib (mostoften)
Prunebellysyndrome May be associatedwith: Gorlin basal cell nevus
Osteogenesisimperfecta syndrome
Cleidocranialdysostosis 0 A singlebifidrib is mostcommonlya normal
Hypothyroidism incidentalfinding!
3. R i bfusi on(0.3% )
Arthritis of Interphalangeal Joint of Great Toe May be associatedwith: vertebralsegmentation
1. Psoriaticarthritis anomalies
2. Reiterdisease Location: 1st + 2nd rib / severaladjacentribs
3. Gout Site: posterior/ anteriorportion
4. Degenerativejoint disease 4. Bonebridging= focaljoiningby bone outgrowth
Cause: congenital/ Posttraumatic
Location: anywherealongone pair of ribs/
RIBS severaladjacentribs
^/ completebridging/ pseudarthrosis
ThoracicDeformity / hypoplasticrib (0.2%)
5. Rudimentary
Funnel Chest= PectusExcavatum Location: 1st rib (usually)
= posteriordepressionof sternumcompressingheart
r/ transverseprocessangulatedsuperiorly
againstspine DDx: cervicalrib
0 Mostfrequentlyan isolatedanomaly! 6. Pseudarthrosis of 1st rib (0.1%)
May be associated with: r/ radiolucent linethroughmidportion with dense
prematurity, homocystinuria, Marfansyndrome, scleroticborders
Noonansyndrome,fetalalcoholsyndrome 7. Intrathoracic / pelvicrib (rare)
^/ depressedpositionof sternum(LATERAL)
8. Abnormalnumberof ribs
./ indistinctrightheartbordermimickingrightmiddle (a) supernumerary: trisomy21, VATERsyndrome
lobe process(FRONTAL) (b) 11 pairs: normalindividuals (5-8%);trisomy21
./ decreasedheartdensity(FRONTAL) (33%);cleidocran ial dysplasia;campomelic
r/ leftwarddisplacementof heartmimicking dysplasia
cardiomegaly (FRONTAL)
r/ horizontalcourseof posteriorportionof ribs
r/ accentuateddownwardcourseof anteriorportions
Short Ribs
1. Thanatophoricdysplasia
of ribs (FRONTAL)
2. Jeuneasphyxiating thoracicdysplasia
3. Ellis-vanCreveldchondroectodermaldysplasia
Barrel Chest 4. syndromes(Saldino-Noonan,
Shortrib-polydactyly
= largesagittaldiameterof thorax Majewski,Verma-Naumoff)
Cause: COPD,emphysema 5. Achondroplasia
r/ lateralsegmentsof ribs elongated+ straight 6. Achondrogenesis
pointingvertical(FRONTAL) 7. Mesomelicdwarfism
r/ squareshape on cross section(CT) 8. Spondyloepiphyseal dysplasia
9. Enchondromatosis

CongenitalRib Anomalies Rib Lesions

Prevalence: 1.4o/o A . B E N I G NR I BT U M O R
1. Cervicalrib (0.2-1-8%): McF 1. Fibrousdysplasia(mostcommonbenignlesion)
. usuallyasymptomatic ri predominantlyposteriorlocation
. thoracicoutletsyndrome(dueto elevationof floor 2. Osteochondroma / exostosis:at costochondral/
of scalenetrianglewith decreaseof costovertebral j unction
costoclavicularspace): Associatedwith: spontaneoushemothorax
0 10-20% of symptomaticpatientshave a 3. Langerhanscell histiocytosis (eosinophilic
responsiblecervicalrib granuloma)
0 5-10% of completecervicalribscausesymptoms 4. Benigncorticaldefect
May be associatedwith: Klippel-Feilanomaly 6. Hemangiomaof bone
{ uni-i bilateral 7. Enchondroma:at costochondralI
^/ may fuse with first ribs anteriorly costovertebral j unction
r/ adjacenttransverseprocessangulatedinferiorly 8. Giantcelltumor
Cx: aneurysmaldilatationof subclaviana. 9. Aneurysmalbonecyst
DDx: elongatedtransverseprocessof 7th cervical 10. Osteoblastoma
vertebra;hypoplastic 1stthoracicrib 11. Osteoi dosteoma
 DifferentialDiagnosisof Musculoskeletal
Disorders t 9

12. Chondroblastoma B . V E N OU S
13. Enostosis= bone island(0.4o/") Cause: enlargementof intercostalveins
14. Pagetdisease (a) AV malformationof chestwall
15. Browntumorof HPT (b) Superiorvena cava obstruction
16. Xanthogranuloma C . N E U R OGE N IC
B . P RI M A RYM AL IGN AN TR IBT U M O R 1. Intercostalneuroma
1. Chondrosarcoma (calcifiedmatrix):most common 2. Neurofibromatosistype 1
2. Plasmacytoma / quadriplegia
3. Poliomyelitis / paraplegia
3. Lymphoma D . OS S E OU S
4. Osteosarcoma(rare) 1. Hyperparathyroidism
5. Fibrosarcoma 2. Thalassemia
6. Primitiveneuroectodermaltumor (= Askintumor) 3. Melnick-Needles syndrome
C. SECONDARYMALIGNANTRIB TUMOR
- in adult: 1. Metastasis(mostcommon Unrurenal Rra Norcxlr.tcor.rlrurtnronManclu
malignantlesion) 1. PostoperativeBlalock-Taussig shunt
2 . Multiple
myeloma (subclavianto pulmonaryartery)
3 , Desmoid tumor 2. Coarctationbetweenoriginof innominatea. + L
- i nc h i l d : 1 . Ewingsarcoma (mostcommon subclaviana.
malignanttumoraffecting
ribsof 3. Coarctationproximalto aberrantsubclaviana.
children+ adolescents)
2. Metastaticneuroblastoma
D. T RA UM A T ICR IB D IS O R D E R Rib Notching on SuperiorMargin
1. Healingfracture 1. Rheumatoid arthritis
2. Radiationosteitis 2. Scleroderma
DDx: pulmonarynodule 3. Systemiclupuserythematosus
E . A G G RE S S IVEGR AN U L OMA T O UIN
S F E CTION S 4. Hyperparathyroidism
= osteomyelitis 5. Restrictive
lungdisease
6. Martansyndrome
ExpansileRib Lesion
mnemonic.' "O FEELTHE CLAMP" Dysplastic Twisted Ribbon Ribs
Osteochondroma(25% of all benignrib tumors) 1. Osteogenesisimperfecta
Fibrousdysplasia 2. Neurofibromatosis
granuloma
Eosinophilic
Enchondroma(7o/"of all benignrib tumors) Bulbous Enlargement of Costochondral
Lymphoma/ Leukemia
Junction
Tuberculosis
1. Rachitic
rosary
Hematopoiesis
2. Scurvy
Ewingsarcoma
3. Achondroplasia
Ghondromyxoid fibroma 4. Hypophosphatasia
Lymphangiomatosis
5. Metaphyseal
chondrodysplasia
Aneurysmalbone cyst
6. Acromegaly
Metastases
Plasmacytoma
Wide Ribs
(anemias)
1. Marrowhyperplasia
AbnormalRib Shape 2. Fibrous
dysplasia
Rib Notching on lnferior Margin 3. Pagetdisease
= minimalconcavescalloping/ deep ridgesalong the 4. Achondroplasia
neurovasculargroovewith reactivesclerosis 5. Mucopolysaccharidoses
0 Minorundulations in the inferiorribsare normal!
0 The medialthird of posteriorribs near transverse Slender Ribs
processof vertebraemay be notchednormally! 1. Trisomy18syndrome
A. ARTERIAL 2. Neurofibromatosis
Cause: intercostalaa. functionas collateralsto
descendingaorta/ lung
(a) Aorta: coarctation(usuallyaffectsribs 4-8; Dense Ribs
rare beforeage I years),thrombosis 1. Tuberoussclerosis
(b) Subclavianartery: Blalock-Taussig shunt 2. Osteopetrosis
(c) Pulmonaryartery: pulmonarystenosis, 3. Mastocytosis
tetralogyof Fallot,absentpulmonaryartery 4. Fluorosis
 5. Fibrousdysplasia MetacarpalSign
6. Chronicinfection = relativeshorteningof 4th + 5th metacarpals
7. Trauma { tangentialline along headsof Sth + 4th metacarpals
8. rib resection
Subperiosteal intersects3rd metacarpal
1. ldiopathic
Hyperlucent Ribs 2. Pseudo-and pseudopseudohypoparathyroidism
Congenitally Lucent Ribs 3. Basalcell nevussyndrome
1. Osteogenesisimperfecta 4. Multipleepiphysealdysplasia
2. Achondrogenesis 5. Beckwith-Wiedemann syndrome
3. Hypophosphatasia 6. Sicklecell anemia
4. Campomelic dysplasia 7. Juvenilechronicarthritis
8. Gonadaldysgenesis:Turnersyndrome,Klinefelter
Acquired Lucent Ribs syndrome
1. Cushing
disease 9. Ectodermaldysplasia= Corneliade Langesyndrome
2. Acromegaly 10. Hereditarymultipleexostoses
3. Scurvy 11. Peripheral dysostosis
12. Melorheostosis

CLAVICLE mnemonic.' "PingPong ls ToughTo Teach"

Pseudohypoparathyroidism
Absence of Outer End of Clavicle
Pseudopseudohypoparathyroidism
1. Rheumatoid arthritis
ldiopathic
2. Hyperparathyroidism
Trauma
3. Posttraumaticosteolysis
Turnersyndrome
/ multiple
4. Metastasis myeloma
Trisomy13-18
dysplasia
5. Cleidocranial
6. Gorlinbasalcellnevussyndrome
LucentLesionin Finger
Penciled Distal End of Clavicle
A. BENIGNTUMOR
1. E nchondroma
mnemonic; "SHIRTPocket"
2. Epidermoidinclusioncyst
Scleroderma
3. Giantcelltumor
Hyperparathyroidism
4. Reparativegranuloma
Infection
5. Sarcoidosis
Rheumatoid arthritis
6 Glomustumor(rare)
Trauma
others: aneurysmalbonecyst,browntumor,
Progeria hemophilicpseudotumor,solitarybone
cyst, osteoblastoma
Destruction of Medial End of Clavicle B. MALIGNANTTUMOR
mnemonic; "MILERS" 1. Osteosarcoma
Metastases 2. Fibrosarcoma
lnfection 3. Metastasisfrom lung,breast,malignant
Lymphoma melanoma
granuloma
Eosinophilic
Rheumatoid arthritis mnemonic; "GAMESPAGES"
Sarcoma Gl omustumor
Arthritis(gout,rheumatoid)
Metastasis(lung,breast)
WRIST & HAND Enchondroma
Carpal Angle Simplecyst (inclusion)
= ?ngl€of 130'formedby tangents
to proximal
rowof Pancreatitis
carpalbones Aneurysmalbone cyst
A. DECREASED CARPAL ANGLE(<124) Giantcelltumor
1. Turnersyndrome Epidermoid
2. Hurlersyndrome Sarcoid
3. Morquiosyndrome
4. Madelung deformity Dactylitis
B. INCREASED CARPAL ANGLE(>139') = exp?r'rsion
of bone with cysticchanges
1. Downsyndrome 1. Tuberculousdactylitis(= sp;n" ventosa)
2. Arthrogryposis 2. Pyogenic/ fungalinfection
3. Bonedysplasiawithepiphysealinvolvement 3. Syphiliticdactylitis
 DifferentialDiagnosisof Musculoskeletal
Disorders 21

4. Sarcoidosis processinvolving
r/ tyticdestructive distal+ middle
5. Hemoglobinopathies phalanges
6. Hyperparathyroidism { NO periosteal reaction
7. Leukemia { epiphyses resistosteolysis
untillate

Resorption of Terminal Tufts Acroosteosclerosis

A. TRAUMA = focalopaqueareas+ endosteal
thickening
1. Amputation 1. Incidental
in middle-aged
women
2. Burns,electric
injury 2. Rheumatoid arthritis
3. Frostbite 3. Sarcoidosis
poisoning
4. Vinylchloride 4. Scleroderma
B. NEUROPATHIC 5. Systemiclupuserythematosus
1. Congenital to pain
indifference 6. Hodgkindisease
2. Syringomyelia 7. Hematologic disorders
3. Myelomeningocele
4. Diabetesmellitus Fingertip Calcifications
5. Leprosy 1. Scleroderma / CRESTsyndrome
C. COLLAGEN-VASCULAR DISEASE 2. Raynauddisease
1. Scleroderma 3. Systemiclupuserythematosus
2. Dermatomyositis 4. Dermatomyositis
3. Raynaud disease 5. Calcinosis
circumscripta
universalis
D. METABOLIC 6. Hyperparathyroidism
1. Hyperparathyroidism
E. INHERITED
1. Familial
acroosteolysis Brachydactyly
2. Pyknodysostosis = shortening/ broadening + phalanges
of metacarpals
3. Progeria= Wernersyndrome 1. ldiopathic
4. Pachydermoperiostosis 2. Trauma
F. OTHERS 3. Osteomyelitis
1. Sarcoidosis 4. Arthritis
2. Psoriatic
arthropathy 5. Turnersyndrome
3. Epidermolysisbullosa 6. Osteochondrodysplasia
7. Pseudohypoparathyroidism,
Acroosteolysis Pseudopseudohypoparathyroid
ism
1. Acroosteolysis:
(a)acquired,
(b)familial 8. Mucopolysaccharidoses
2. Massiveosteolysis 9. Corneliade Langesyndrome
3. Essentialosteolysis 10. Basalcellnevussyndrome
4. Ainhumdisease 11. Hereditary multiple
exostoses

Acq ui red Acroosteolys i s Clinodactyly

mnemonic;"PETER's = curvature plane
of fingerin mediolateral
DIAPER SPLASH"
Psoriasis,Porphyria 1. Normalvariant
Ehlers-Danlos syndrome 2. Downsyndrome
Thrombangitis obliterans 3. Multiple
dysplasia
Ergottherapy 4. Trauma,arthritis,
contractures
Raynaud disease
Diabetes, Dermatomyositis, Dilantin
therapy Polydactyly
Injury(thermal+ electrical
burns,frostbite) Frequently with:
associated
Arteriosclerosis
obliterans 1. Carpentersyndrome
PVC(polyvinylchloride) worker 2. Ellis-van
Creveld
syndrome
Epidermolysisbullosa 3, Meckel-Grubersyndrome
Rheumatoid arthritis,
Reitersyndrome 4. Polysyndactyly
syndrome
Scleroderma, Sarcoidosis 5. Shortrib-polydactyly
syndrome
Progeria,Pyknodysostosis 6. Trisomy 13
Leprosy,Lesch-Nyhan syndrome
Absenceof pain Syndactyly
Syringomyelia = osseous+ cutaneous
fusionof digits
Hyperparathyroidism 1. Apertsyndrome
alsoin: yaws;Kaposisarcoma; 2. Carpentersyndrome
pachyde rmoperiostosis 3. Downsyndrome
22 RadiologyReviewManual

4. Neurofibromatosis 5 . Asepticloosening
5. Polandsyndrome lncidence: 50"/"of prosthesesafter 10 years
6. Others Cause:
(a) mechanicalwear + tear
disease(= inflammatory-immune
(b) small-particle
HIP reactionto methylmethacrylate / metallic
Snapping Hip Syndrome fragmentsactivatesphagocyteswith secretion
A. INTRAARTICULAR of cytokines+ proteolyticenzymesleadingto
1. Osteocartilaginousbodies osteolysis)
B. EXTRAARTICULAR = tendonslippage Rx: 30% requiresingle-stagerevisionarthroplasty
1. fascialata lgluteusmaximusovergreater 6 . Infection(= septicloosening)
trochanter lncidence: 1-9%
2. iliopsoastendonover iliopectinealeminence Organisms: Staphylococcus epidermidis(31%),
3. longheadof bicepsfemorisover ischial Staphylococcus aureus (20/"),
tuberosity Streptococcus viridans(11%),
4. iliofemoralligamentover anteriorportionof hip Escherichia coli (11%),Enterococcus
capsule faecalis(8h), group B streptococcus
(5%)
Timeof onset: 33% within3 months,33% within 1
Increasein TeardropWidth year,33o/">1 year aftersurgery
^/ increasein distancebetweenteardrop+ femoralhead Rx: excisionalarthroplasty+ protractedcourseof
Cause: hip joint effusion antimicrobialtherapy+ revisionarthroplasty
r/ increasein mediolateralsize of teardrop
Cause: hip dysplasia,chronichipjointeffusion P l ai nfi l m:
duringskeletalmaturation { migrationof prostheticcomponentscomparedto
previousfilm:
{ subsidenceof prosthesis(up to 5 mm is
ProtrusioAcetabuli normalfor noncemented femoralcomponent
= acetdbularfloorbulgingintopelvis in first few months)
r/ acetabularlineprojectingmediallyto ilioischial lineby r/ cement/ prosthesisfracture
>3 mm (in males)/ >6 mm (in females) { motionof componentson stressviews/
r/ crossingof medial+ lateralcomponentsof pelvic fluoroscopy
"teardrop"(U-shapedradiodensearea medialto hip r/ wideningof prosthesis-cement interface
joint with (a) lateralaspect= acetabulararticular ^/ lucencyat cement-boneinterface>2 mm
surface(b) medialaspect= anteroinferiormarginof ^/ progressive wideningof cement-bonelucency
quadrilateralsurfaceof ilium) after 12 postoperativemonths
A . UNI LA T E R AL r/ focal lytic area (due to particulatedebriswith
1. Tuberculous arthritis foreignbodygranulomai abscess)
2. Trauma { extensiveperiostitis(in infection,but rare)
3. Fibrousdysplasia
B. BILATERAL NUC (83%sensitive,88% specific):
1. Rheumatoidarthritis ./ increaseduptakeof bone agent,gallium-67,
2. Pagetdisease indium-'l11-labeledleukocytes, complementary
3. Osteomalacia technetium-labeled sulfurcolloid+ combinations
mnemonic,' "PROT" BoneScintigraphy:
Pagetdisease ri normal= strongevidenceagainsta prosthetic
Rheumatoidarthritis abnormalitY (= high NPV)
Osteomalacia(HPT) r/ diffuseintenseuptakearoundfemoral
Trauma component(= generalizedosteolysisassociated
with asepticlooseningor infection)
r/ focal uptakeat distaltip of femoralcomponentin
Pain with Hip Prosthesis >1 year old prosthesis= asepticloosening
Approximately 120,000hip arthroplasties per year in Sequentialbone-gallium scintigraphy:
USA r/ congruentspatialdistributionof both tracerswith
1. Heterotopic ossification galliumintensitylessthan bonetracer= no
2. Trochantericbursitis infection
3. Prostheticfracture/ periprosthetic
fracture/ cement { spatiallyincongruent / intensityof gallium
fracture exceedsthat of bone agent = infection
4. Dislocation(dueto capsularlaxity/ incorrect { spatiallycongruent+ similarintensityof both
componentplacement) tracers= inconclusive
 Differential Diagnosis of Musculoskeletal Disorders 23

Combined labeledleukocyte-marrowscintigraphy: 4. Lateralacetabularinclination = horizontalversion

Accuracy: >90/"
Concept: Tc-99msulfurcolloidmaps aberrantly
located normal bone marrowas a
point of referencefor leukocytetracer
r/ spatiallycongruentdistributionof both
radiotracefs= fto infection
r/ tabeledleukocyteactivitywithoutcorresponding
sulfurcolloidactivity= infection
Arthrography:
r/ irregularityof joint pseudocapsule
^/ tiltingof nonbursalspaces/ sinustracts/
abscesscavities
Aspirationof fluid underfluoroscopy(12-93%
sensitive,83-92% specificfor infection):
r/ injectionof contrastmaterialto confirm
intraarticular
location
= ongleof cup in referenceto line R (40' + 10o
desirable)
Lessangulation: stablehip, limitedabduction
Greaterangulation: riskof hip dislocation
5. Varus/ neutral/valgusstem position
Varusposition: tip of stem restsagainstlateral
endosteum,increasedrisk for
loosening
Valgusposition: tip of stem restsagainstmedial
endosteum,not a significant
problem
6. Acetabularanteversion (15' + 10' desirable)
= lateralradiographof groin
Retroversion: riskof hip dislocation
7. Femoralneck anteversion
works synergisticallywith acetabularanteversion
true angleassessedby CT

Evaluationof TotalHip Arthroplasty

M E A S URE ME N T S Radiographic findings
Referenceline: transischialtuberosityline (R) A . N OR MA L
1. Leg length= verticalpositionof acetabular r/ irregularcement-boneinterface
component = norfi'r€ll
interdigitationof
= corTlp€lfing
levelof greater/ lessertuberosity(T) polymethylmethacrylate (PMMA) with
with respectto line R adjacentbone remodelingprovidinga
High placement: shorterleg, less effective mechanicalinterlock
musclescrossingthe hipjoint 0 P MMAi s not a gl ue!
Low placement: longerleg,musclesstretchedto r/ tfrinlucentlinealongcement-bone interface
pointof spasmwith risk of = 0.1-1.5-mmthi nconnecti ve ti ssuemem br ane
dislocation ("demarcation") along cement-boneinterface
2. Verticalcenterof rotation accompanied by thin lineof bonesclerosis
= distancefrom centerof femoralhead (C) to line R B . A B N OR MA L
3. Horizontalcenterof rotation r/ wide lucentzone at cement-boneinterface
= distancefrom centerof femoralhead (C) to = )2-mm lucentlinealongbone-cement
teardrop/ othermediallandmark interfacedue to granulomatous membrane
Lateralposition: iliopsoastendoncrossesmedial Cause: componentloosening+ reactionto
to femoralheadcenterof rotation particulatedebris(eg, PMMA,
increasingrisk of dislocation colyethylene)

Initial Evaluation of Total Hip Arthroplasty

24 Radiology Review Manual

r/ lucentzone at metal-cementinterfacealong Associated with injury of:

proximallateralaspectof femoralstem (1) medialcollateralligament(at femoral
= suboptimalmetal-cementcontactat time of attachmentsite
surgery/ loosening (2) anteriorcruciateligament
r/ well-definedarea of bone destruction (3) medialmeniscus
(= histiocyticresponse,aggressive (4) combination of all three= O'Donoghuetriad
granulomatous disease) { anterolateral aspect of lateralfemoralcondyle
Cause: granulomatousreactionas response ^/ inferomedialaspectof patella
to particulate
debris/ infection/ tumor Predisposed: teenaged/ young adult athleteswith
{ asymmetricpositioning of femoralheadwithin shallowtrochleargroove
acetabularcomponent Cause: lateral patellar dislocation = twisting
Cause: acetabularwear / dislocationof motionwith knee in flexion+ quadriceps
femoralhead / acetabulardisruption/ contraction
linerdisplacement / deformity Associated with injury of:
{ cementfracture (1) medi alreti nacul um
Cause: loosening (2) medialpatellofemoral ligament(nearfemoral
attachmentsite) most importantstabilizing
structure)
KNEE (3) medialpatellotibial ligament

BoneContusionPattern UniqueTibialLesions
r/ edema of midportionof lateralfemoralcondyle 1. Fibrousdysplasia
Cause: pivot shift injury = volgusload + external 2. Ossifyingfibroma
rotationof tibia/ externalrotationof femur 3. Adamantinoma
appliedto variousstatesof flexion
combined(noncontact injury) TibiotalarSlanting
= downwardslantingof medialtibialplafond
Predisposed: skier,footballplayer
Associated with injury of: 1. Hemophilia
(1) anteriorcruciatelig. (midsubstance > femoral 2. Stilldisease
attachment> tibialattachmentsite) 3. Sicklecell disease
(2) posteriorjoint capsule+ arcuateligament 4. Epiphyseal dysplasia
(3) posteriorhornof lateral/medialmeniscus 5. Trauma
(4) medialcollateralligament
r/ + edemaof posteriorpatellarsurface FOOT
Cause: dashboard injury = force upon anterior AbnormalFoot Positions
proximaltibiawith knee in flexedposition A. FOREFOOT
Associated with: 1, Varus= adduction
(1) ruptureof posteriorcruciatelig. (midsubstance > = ?XiSof 1st metatarsaldeviatedmediallyrelative
femoralattachment> tibialattachmentsite) to axis of talus
(2) tear of posteriorjoint capsule 2. Valgus= abduction
(3) fracture/ osteochondralinjuryof patella = ?Xisof 1st metatarsaldeviatedlaterallyrelative
(4) injuryof hip to axis of talus
rl "kissing"bone contusionpattern= anterioraspectof 3. Inversion= supination
tibialplateau+ anterioraspectof femoralcondyle = inwardturningof sole of foot
Cause: hyperextension injury = directforce upon 4. Eversion= pronation
anteriortibiawhilefoot is planted/ indirect = outwardturningof sole of foot
force of forcefulkickingmotion B . H IN D FOOT
Associated with: talipes(talus,pes) = any deformityof the ankle and
(1) injuryto posterior/ anteriorcruciatelig. hindfoot
(2) meniscalinjury 1. Equinus
(3) dislocationof knee = hindfootabnormalitywith reversalof calcaneal
(4) poplitealneurovascular injury pitchso that the heelcannottouchthe ground
(5) completedisruptionof posterolateral complex 2. Calcanealfoot
./ edema in lateralaspectof femoralcondyle = v€fY high calcanealpitchso that forefoot
(secondaryto directblow) cannottouchthe ground
{ smallarea of edemain medialfemoralcondyle(due 3. Pes planus= flatfoot
to avulsivestressto medialcollateralligament) = low calcanealpitch+ (usually)heelvalgus+
Predisposed: footballplayer forefooteversion
Cause: clip injury = pure valgusstresswith knee 4. Pes cavus
in mildflexion = high calcanealpitch (fixedhigh arch)
 DifferentialDiagnosisof Musculoskeletal
Disorders 25

Clubfoot= TalipesEquinovarus SOFTTISSUES

Commonseverecongenitaldeformitycharacterizedby HistologicClassificationof Soft-tissueLesions
. equinusof heel (reversedcalcanealpitch) A. FATTY
. heel varus (talocalcanealangleof almostzero on AP 1. Lipoma
view with both bonesparallelto each other) 2. Angiolipoma
. metatarsusadductus(axisof 1st metatarsaldeviated 3. Liposarcoma
mediallyrelativeto axis of talus) B. FIB R OU S
1. Arthrogryposis multiplexcongenita 1. Fi broma
2. Chondrodysplasia punctata 2. Nodularfasciitis
3. Neurofibromatosis 3. Aggressivefibromatosis/ desmoid
4. Spinabifida 4. Fibrosarcoma
5. Myelomeningocele C. MUSCLE
1. Rhabdomyoma
Rocker-bottomFoot = VerticalTalus 2. Leiomyoma
r/ verticallyorientedtaluswith increasedtalocalcaneal 3. Rhabdomyosarcoma
angle on lateralview 4. Leiomyosarcoma
{ dorsalnaviculardislocation joint
at talonavicular D. VASCULAR
r/ heetequinus 1. H emangi oma
{ rigiddeformity 2. Hemangiopericytoma
Associatedwith: Arthrogryposismultiplexcongenita; 3. Hemangiosarcoma
spinabifida;trisomy13-18 E. LY MP H
1. Lymphangi oma
TalarBeak= HypertrophiedTalarRidge 2. Lymphangiosarcoma
1. Talocalcanealtype of tarsalcoalition 3. Lymphadenopathy in lymphoma/ metastasis
2. Diffuseidiopathicskeletalhyperostosis(DISH) F. SYNOVIAL
3. Acromegaly 1. Nodularsynovitis
4. Rheumatoidarthritis 2. Pigmentedvillonodular synovitis
3. Synovialsarcoma
G. NEURAL
Heel Pad Thickening 1. Neurofibroma
= heelpad thickening>25 mm (normal<21 mm) 2. N euri l emoma
mnemonic.' "MAD COP" 3. Ganglioneuroma
Myxedema 4. Malignantneuroblastoma
Acromegaly 5. Neurofibrosarcoma
Dilantintherapy H. C A R TILA GE A N D B ON E
Callus 1. Myositisossificans
Obesity 2. Extraskeletal osteoma
Peripheraledema 3. Extraskeletal chondroma
4. Extraskeletal chondrosarcoma
Soft-tissueMassesof Foot + Ankle 5. Extraskeletalosteosarcoma
A. NONTUMORAL
(a) synovialproliferations
1. Pigmentedvillonodular synovitis(PVNS) Fat-containingSoft-tissueMasses
2. Giantcelltumor(GCT)of tendonsheath A . B E N IGNLIP OMA TOU TU S MOR S
(b) posttraumatic 1. Li poma
1. Plantarfasciitis 2. Intra-/ intermuscular lipoma
(c) inflammatory 3. Synoviallipoma
(d) uncertainorigin 4. Lipomaarborescens= diffusesynoviallipoma
1. Ganglioncyst 5. Neuralfibrolipoma= fibrolipomatous tumorof
2. Epidermoidcyst nerve
3. Mortonneuroma 6. Macrodystrophia lipomatosa
4. Floridreactiveperiostitis B. LIPOMAVARIANTS
6. Rheumatoidnodules 1. Lipoblastoma (onlyin infancy+ earlychildhood)
B . B E NI G NT U M O R S 2. Lipomatosis = diffuseovergrowthof mature
1. Plantarfibromatosis adiposetissueinfiltratingthroughthe soft tissues
2. Deepfibromatosis of affectedextremity/ trunk
3. Infantiledigitalfibromatosis 3. Hibernom? = ra.rabenigntumor of brownfat;
4. Hem an g i o m a oftenin peri-/ interscapularregion,axilla,thigh,
5. Nervesheathtumor chestwall
6. Lipoma,angiolipoma r/ markedhypervascularity
 C. M A LI G NA N TL IPOMA T O U T SU M O R Cause:
1. Liposarcoma (a) Skeletaldeossification
D. OTHERFAT-CONTAINING TUMORS 1 . 1 "H P T
1. Hem an g i o ma 2. EctopicHPT production(lung/ kidneytumor)
2. Elastofibroma 3. Renalosteodystrophy + 2' HPT
E . LE S I O NSM IM IC K IN G
F A T -C ON T AIN INTU
G MOR S 4. Hypoparathyroidism
1. Myxoidtumors: intramuscular myxoma, (b) Massivebone destruction
extraskeletalmyxoidchondrosarcoma, myxoid 1. Widespreadbone metastases
malignantfibroushistiocytoma 2. P l asmacel lmyel oma
2. Neuraltumors: neurofibroma, neurilemoma, 3. Leukemia
malignantschwannoma (c) Increasedintestinalabsorption
\l 73% have tissueattenuationless than muscle 1. Hypervitaminosis D
3. Hemorrhage 2. Milk-alkali
syndrome
3. Excessingestion/ lV administration
of
calciumsalts
4. Prolongedimmobilization
ExtraskeletalOsseous+ CartilaginousTumors 5. Sarcoidosis
A. OSSEOUSSOFT-TISSUE TUMORS (d) ldiopathichypercalcemia
r/ cloudlike"cumulus"type of calcification
1. Myositisossificans
2. Fibrodysplasia ossificansprogressiva Dystrophi c Calcif ication
3. Soft-tissueosteoma = in presenceof normalserumCa + P levels
4. Extraskeletal osteosarcoma secondaryto localelectrolyteI enzymealterations
5. Myositisossificansvariants in areasof tissueinjury
(a) Panniculitisossificans
(b) Fasciitisossificans Cause:
(c) Fibroosseous pseudotumor of digits (a) Metabolicdisorderwithouthypercalcemia
B . CA RT I LA GIN O USOFS T -T IS SUTEU M O R S 1. Renalosteodystrophy with 2' HPT
./ arcs and rings,spiculesand flocculesof 2. Hypoparathyroidism
calcification 3. Pseudohypoparathyroidism
1. Synovialosteochondromatosis 4. Pseudopseudohypoparathyroidism
2. Soft-tissue chondroma 5. Gout
3. Extraskeletal chondrosarcoma 6. Pseudogout= chondrocalcinosis
7. Ochronosis= alkaptonuria
DDx: L Diabetesmellitus
(1) Synovialsarcoma (b) Connectivetissuedisorder
(2) Benignmesenchymoma 1. S cl eroderma
= lipomawith chondroid/ osseousmetaplasia 2. Dermatomyositis
(3) Malignantmesenchymoma 3. Systemiclupuserythematosus
= 2 or moreunrelatedsarcomatous components (c) Trauma
(4) Calcified/ ossifiedtophusof gout 1. Neuropathic calcifications
(5) Ossifiedsoft-tissuemassesof melorheostosis 2. Frostbite
(6) Pilomatricoma= calcifying epitheliomaof Malherbe 3. Myositisossificansprogressiva
. lesionarisesfrom hair matrixcellswith slow 4. Calcifictendinitisi bursitis
growthconfinedto the subcutaneous tissueof (d) Infestation
the face,neck,upperextremities 1. Cysticercosis
r/ centralsandlikecalcifications(84%) 2. Dracunculosis (guineaworm)
{ peripheralossification(20%) 3. Loiasis
(7) Tumoralcalcinosis 4. Bancroftfilariasis
5. Hydatiddisease
6. Leprosy
Soft-tissueCalcification (e) Vasculardisease
MetastaticCalcification 1. Atherosclerosis
= depositof calciumsaltsin previouslynormaltissue 2. Mediasclerosis(Monckeberg)
(1) as a resultof elevationof Ca x P productabove 3. Venouscalcifications
60-70 4. Tissue infarction(eg, myocardialinfarction)
(2) with normalCa x P productafter renaltransplant (f) Miscellaneous
Location: lung (alveolarsepta,bronchialwall,vessel 1. Ehlers-Danlos syndrome
wall),kidney,gastricmucosa,heart, 2. Pseudoxanthoma elasticum
peripheralvessels 3. Wernersyndrome= progeria
t-
I
DifferentialDiagnosisof Musculoskeletal
Disorders 27

4. Calcinosis(circumscripta,
universalis, . Laboratoryfindings:
tumoralcalcinosis) (a) relativelyspecific: ANA in peripheralrim /
5. Necrotictumor nucleolarpattern,anti-DNA,elevatedmuscle
enzyme
General ized Cal ci nosr's (b) relativelynonspecific:ANA in homogeneous
(a)Collagen vascular disorders pattern,anti-single-stranded DNA,positive
1. Scleroderma rheumatoidfactor
2. Dermatomyositis Types and most distinctive features:
(b)ldiopathictumoral calcinosis 1. Rheumatoid arthritis
(c) ldiopathic
calcinosisuniversalis positiverheumatoid factor,prominentmorning
stiffness,symmetricerosivearthritis
2. Systemiclupuserythematosus
InterstitialCalcinosis malarrash,photosensitivity, serositis,renal
Calci nosis Ci rcu mscripta disorderswith hemolyticanemia,leukopenia,
1. Acrosclerosis: granulardeposits aroundjointsof lymphopenia, thrombocytopenia, positiveANA
fingers+ toes,fingertips 3. Sjdgrensyndrome
2. Sclerodermar acrosclerosis + absorptionof ends dry eyes + mouth,abnormalSchirmertest
of distalphalanges 4. Scleroderma
3. Dermatomyositis: extensive subcutaneous deposits Raynaudphenomenon, skinthickeningof distal
4. Varicosities:particularly
in calf extremities proceedingto includeproximal
5. 1' Hyperparathyroidism: infrequentlyperiarticular extremities + chest+ abdomen,positiveANA in a
calcinosis nucleolarpattern
6. Renalosteodystrophy with2" hyperparathyroidism: 5. Polymyositis, dermatomyositis
extensive vasculardepositsevenin young heliotroperashover eyes,proximalmuscle
individuals weakness,elevatedmuscleenzymes,
7. Hypoparathyroidism: occasionallyaroundjoints; inflammation at musclebiopsy
symmetrical in basalganglia
8. VitaminD intoxication:periarticularin rheumatoid Mixed ConnectiveTissue Disease
(puttylike);
arthritis calcium depositin tophi = disorderthat sharesdistinctivefeaturesof 22
differentconnectivetissuediseasesin same patient
Calcinosis U niversalis (eg,overlapping featuresof SLE, PSS,
Progressive diseaseof unknown origin polymyositis)
Age: children + youngadults . pulmonaryhypertension (dueto interstitial
{ plaquetike calciumdepositsin skin+ subcutaneous pulmonaryfibrosis/ intimalproliferation
of
tissues;sometimes in tendons+ muscles pulmonaryarterioles)
r/ trtOtrueboneformation

Muscle
Soft-tissue Ossification MR signalintensityof normalmuscle:
= formationof trabecular
bone ./ higfrerthan water + lowerthan fat on Tl Wl
1. Myositisossificansprogressiva/ circumscripta { much lowerthan water + fat on T2Wl
2. Paraosteoarthropathy
3. Soft-tissueosteosarcoma
4. Parostealosteosarcoma Intramuscular Mass
5. Posttraumatic = periosteoma
periostitis A. NEOPLASM
6. Surgicalscar B . IN FE C TION / IN FLA MMA TION
7. Severely burnedpatient 1. Intramuscular abscess
2. Focalmyositis= benigninflammatory
Connective Tissue Disease pseudotumor
= cTD= ICOLLAGEN VASCULAR DTSEASE] 3. Necrotizing fasciitis
= groupof disordersthatsharea numberof clinical 4. Sarcoidosis
+ laboratory
features r/ noduleswith centralstar-shapedarea of
. Features: fibrosissurroundedby granuloma
(a)relatively myositis,
specific:arthritis, Raynaud C . MY ON E C R OS IS
phenomenon withdigitalulceration,
tetheredskin 1. S i ckl ecel lcri si s
in extremities
+ trunk,malarrashsparing 2. Poorlycontrolleddiabetes
nasolabialfolds,morningstiffness 3. Compartmentsyndrome
(b)relatively
nonspecific:polyarthralgias
(most 4. Crushinjury
commoninitialsymptom), myalgias,mottling
ol 5. Severeischemia
extremities,
muscleweakness + tenderness 6. Intraarterialchemotherapy
 Radiology Review Manual

7. Rhabdomyolysis = seVef€muscleinjurywith Cause: trauma,burns,heavyexercise,

loss of integrityof musclecell membranes
Cause: trauma,severeexercise,ischemia,
burn,toxin,lV heparintherapy,
autoimmuneinflammation
Cx: renaldamagefrom myoglobulinemia,
tetany,compartmentsyndrome
D. TRAUMA
1. Intramuscular hematoma(eg,severemuscle
strain,laceration,contusion,spontaneous)
2. Myositisossificanstraumatica
Muscle Edema
on STIR images
{ musclehyperintensity
A. INFLAMMATION
1. Dermatomyositis
2. Polymyositis
4. Radiationtherapy: straightsharp margins,
involvesmuscle+ subcutaneousfat
5. Earlystageof myositisossificans
B. CELLULARINFILTRATE
1. Ly m p h o ma
C. I NF E CT ION
1. Bacteriali infectiousmyositis
(a) directextensiontrom adjacentinfection(eg,
osteomyelitis,subcutaneousabscess)
(b) hematogenous
2. Inclusionbody myositis(probablydue to
paramyxovirusinfection)resembling
polymyositis
D. RHABDOMYOLYSIS
1. Sport/ electricinjury
2. Diabeticmuscularinfarction
3. Focalnodularmyositis
4. Metabolicmyopathy: eg, phosphofructokinase
deficiency,hypokalemia,alcoholoverdose
5. Viral myositis
E. TRAUMA
1. Subacute muscle denervation
Time of onset: 2-4 weeks afterdenervation
Mechanism: spinalcord injury,poliomyelitis,
peripheralnerve injury/
compression(ganglioncyst,
bone spur),Gravesdisease,
neuritis
2 . Musclecontusion(fromdirectblow)
3 . Musclestrain (= injuryat musculotendinous
junctionfrom overlyforcefulmusclecontraction)
Predilectionfor: hamstring,gastrocnemius m.,
bicepsbrachiim.)
extrinsicpressure,intramuscular
hemorrhage
. severepain
. dysfunctionof sensory+ motornerves
passingthroughaffectedcompartment
6. Sicklecellcrisis
Fatty Infiltration of Muscle
1. Chronicstageof muscledenervation(eg,
poliomyelitis,stroke,peripheralnerve injury)
2. Chronicdisuse(eg,chronictendontear,severe
osteoarthritis)
3. Late stage of severemuscleinjury
4. Long-termhigh-dosecorticosteroidmedication
affectingtruncalmuscles
FIXATION DEVICES
lnternalFixationDevices
A. Screws
1. Corticalscrew= shallowfinelythreadedover
entirelength,blunttip
Use: fixationof plates
2. Cancellousscrew= wide threaddiameterwith
varyinglengthof smoothshank betweenhead +
threads
Use: compressionacrossfracturesite
3. Malleolarscrew= partiallythreaded
4. lnterferencescrew = short,fullythreaded,
cancellousthreadpattern,self-tappingtip,
recessedhead
Use: withintunnelholdingbonegraftof ACL
and PCL reconstruction
5. Cannulatedscrew = hollowscrewinsertedover
gui depi n
Use: fractureof femoralneck
6. Herbertscrew = c?flr'ruhtedscrewthreadedon
both ends with differentpitches,no screw head
Use: scaphoidfracture
Cannulated Screw
ffi
Washer

wwwww
w
4. Delayed-onsetmusclesoreness
= oveIUS€injurybecomingsymptomatichours/
days after overuseepisode)
5. Compartment syndrome
= increasedpressurewithin indistensiblespace '
\ /
K '

of confiningfascialeadingto venous Cortical CancXlous Malleolar Herbert Interference

occlusion,muscle+ nerve ischemia,arterial
occlusion,tissue necrosis Screws
 DifferentialDiagnosisof Musculoskeletal
Disorders 29

WW
Dynamic Malleable L-Plate
Compression Reconstruction
Plate Plate
Cloverleaf
Plate
T:-Plate

Plates

7. Dynamichip screw= sct€wfree to slidewithin Pins + Figqte-of-Eight Rush Pin

barrelof side plate allowingimpactionof fracture Banil Wiring
duringhealingwithoutperforationof subarticular
cortex
Use: subcapital,intertrochanteric,
subtrochantericf racture
B. Washer
1. Flatwasher= increasesurfacearea over which
force is distributed
2. Serratedwasher= spikededges used for affixing
avulsedligaments sliding lag screw
C. Plates
- compressionplate
Use: compressionof stablefractures
- neutralization plate = protectsfracturefrom
bending,rotation+ axial-loadingforces
- buttressplate = supportof unstablefracturesin
compression/ axial loading
1. Straightplate
(a) straightplatewith round holes
(b) dynamiccompressionplate = ovol holes
(c) tubularplate= thin pliableplatewith
concaveinnersurface
(d) reconstruction plate = thin pliableplateto
allow bending,twisting,contouring
2. Specialplates
T-shaped,L-shaped,Y-shaped,cloverleaf,
spoon,cobra,condylarbladeplate,dynamic JewettNail DynamicCompressionScrew
compressionscrewsystem
D. Staples
Fixation= bone = epiphyseal= fracturestapleswith
smooth/ barbedsurface
- Coventry= slspted osteotomystaple
- stone = table staple
E. Wires
1. K wire = unthreadedsegmentsof extrudedwire
of variablethickness
Use: temporaryfixation
2. Cerclagewiring= wire placedaroundbone
Use: fixationof comminutedpatellarfracture,
holdingbone graftsin position
3. Tensionbandwiring= figure-of-eight wire placed Fixation Staple Thble Staple Coventry Staple
on tensionside of bone
Use: olecranon/ patellarfractures Staples
ExternalFixationDevices 4. Kuntschernail = cloverleafin cross sectionwith
= srTrooth/ threadedpins / wires attachedto an external roundedtip
frame
(a) unilateralpin = ehtersbone onlyfrom one side
1. Steinmannpin = large-caliber wire withpointedtip
2. Rushpin = smoothintramedullary pin
3. Schanzscrew= pin threadedat one end to
engagecortex,smoothat other end to connectto
externalfixationdevice
4. Knowlespin (for femoralneck fracture)
(b) transfixingpin - passesthroughextremitysupported
by externalfixationdeviceon both ends

lntramedullaryFixationDevices
Use: diaphyseallong bone fractures
(a) nail = driveninto bone withoutreaming
(b) rod = solid/ hollowdevicewith bluntedtip driven into
reamedchannel(reamingdisruptsbloodsupplyand
may decreasethe rate of fracturehealing)
(c) interlockingnail = ?cc€ssorypins / screws/
deployablefins placedto preventrotation
1 . Rush pin = beveledend + hookedend
2 . Endernail = oval in cross section Charnley-Mueller Thompson Austin-Moore
3 . Sampsonrod = slightlycurvedrigidrod with fluted
surface Hip Prostheses
 Anatomy and Metabolismof Bone 31

ANATOMYANDMETABOLISM
OF BONE

BONE M I NE RA LS Biochemistry:
G alc ium inactiveform of vitaminD" presentthroughdiet/
A. 99% in bone exposureto sunlight;vitafrinDois convertedinto25-
B. serumcalcium OH-vitaminDoby liverand then'converted into 1,25-
(a) protein-boundfraction(albumin) OH vitaminDl1= hormone)by kidney
(b) ionic (pH-dependent)3/" as calciumcitrate/ Stimulusfor cbnversion: (1) hypophosphatemia
phosphatein serum (2) PTH elevation
Absorption: facilitatedby vitamin D Action:
Excretion: relatedto dietary intake;>500 mgl24 (a) INTESTINE:(1) increasedabsorptionof calcium
hours= hypercalciuria from bowel
(2) increasedabsorptionof
phosphatefrom distalsmall
Phosphorus bowel
Absorption: requiressodium;decreasedby aluminum (b) B ON E : (1) propermineralization of osteoid
hydroxidegel in gut (2) mobilizationof calcium+
Excretion: increasedby estrogen,parathormone phosphate(potentiates
decreasedby vitaminD, growthhormone, parathormone action)
glucocorticoids (c) K ID N E Y : (1) increasedabsorptionof calcium
from renaltubule
HORMONES (2) increasedabsorptionof
phosphatefrom renaltubule
Parathormone
Majorstimulus: low levelsof serumcalciumions
(actionrequiresvitaminD presence) Calcitonin
Targetorgans: secretedby parafollicular cells of thyroid
(a) BONE: increasein osteocytic+ osteoclastic Major stimulus: increasein serum calcium
activitymobilizescalcium+ phosphate Target organs:
= bone resorption (a) B ON E : (1) i nhi bi tsparathormone-i nduced
(b) KIDNEY: (1) increasein tubularreabsorption of osteoclasis by reducingnumberof
calcium osteoclasts
(2) decreasein tubularreabsorptionof (2) enhancesdepositionof calcium
phosphate(+ aminoacids) phosphate;responsible for sclerosis
= phosphatediuresis in renalosteodystrophy
(c) GUT: increasedabsorptionof calcium+ (b) KIDNEY:inhibitsphosphatereabsorption in renal
phosphorus tubul e
(c) GUT: increasesexcretionof sodium+ water
Major function: intogut
. increaseof serumcalciumlevels Major function: decreasesserum calcium+ phosphate
. increasein serumalkalinephosphatase
(50%)
PHYSIS
VitaminD Metabolism Fourdistinctzonesof cartilagein longitudinal
layers
requiredfor (1) Germi nalzone= smal lcel l s
(1) adequatecalciumabsorptionfrom gut adjacentto epiphysealossificationcenter
(2) synthesisof calciurn-binding
proteinin intestinal (2) Proliferatingzefia = flattenedcells
mucosa arrangedin columns
(3) parathormoneeffects(stimulationof osteoclastic+ (3) HypertrophicZofle= swollenvacuolatedcells
osteocyticresorptionof bone) (4) Zone of provisionalcalcification

Parathormone
Function
PTH Action Net Effect
Principal: (1) phosphatediuresis (1) S erum: i ncreasei n C a
(2) resorptionof Ca + P from bone decreasein P
Secondary: (3) resorptionof Ca from gut (2) Urine: increasein Ca
(4) reabsorption of Ca from renaltubule increasein P
32 Radiology Review Manual

NORMALSHOULDER JOINTANATOMY opening to subcoracoid recess

GlenoidLabrum biceps tendon
= 4-mm-widefibrocartilaginous ring with considerable
variationin shape attachedto glenoidrim
superior
{ triangular/ roundedshape on cross-sectional image - glenohumeral
r/ blendssuperiorlywith bicepstendon ligament

BicepsTendon lANrI
= longheadof bicepsmuscle
{ attachedto anterosuperior aspectof glenoidrim with subscapularis
fibersto tendon
(a) anterosuperiorlabrum(labral-bicipital
complex)
(b) posterosuperior labrum(labral-bicipital
complex)
(c) supraglenoidtubercle middle
(d) base of coracoidprocess glenohumeral
lieament
{ exitsjoint throughintertubercular
groove axillary pouch
{ securedto intertuberculargrooveby transverselig. of inferior
anterior band of inferior
glenohumeral
glenohumeral ligament
Ligaments
Glenohumeral lisament
= thickenedbandsof jointcapsulefunctioning as
shoulderstabilizers Normal Anatomy of Right Shoulder En Face
(arrow in sublabral foramen)
Superior Glenohumeral Ligament
= most consistentlyidentified
{ arisesfrom anterosuperiorlabrum/ tendon Sublabral Foramen
attachmentof long head of biceps/ middle = sublabralholebetweenlabrum+ glenoid
glenohumeral ligament lncidence: 11"/"of individuals
{ coursesin planeperpendicular to middlegleno- Location: 2 o'clockpositionanteriorto biceps
humeralligament+ paralleltocoracoidprocess tendonattachment
{ best visualizedon transverseCT / MR r/ may coexistwith sublabralrecess
DDx: labraltear
Middle Glenohumeral Ligament
= varies most in size + attachment;may be absent Buford Complex
{ coursesobliquelyfrom superomedialto inferolateral = cordlikethickeningof middleglenohumeral ligament
r/ attachesmediallyon glenoidneck/ superiorportion glenoid
directlyattachingto anterosuperior
of anteriorglenoidrim + absenceof anterosuperior labrum
^/ ligamentstretchedthroughexternalarm rotated lncidence: 1.5'h of individuals
{ may be thick+ cordlike Location: 2 o'clockpositionanteriorto biceps
{ best visualizedon sagittal/ transverseCT / MR tendonattachment
{ may coexistwith sublabralrecess
lnferior GlenohumeralLigament DDx: displacedlabralfragment
= important stabilizer
of anteriorshoulderjoint
1. Anteriorband
r/ insertsalonginferior213of anteriorglenoid
labrum
r/ usuallyquiteprominent, in 25'/"verythin supraspinatus
2. Posterior band(usually thinner)

Normal Anatomic Variants of Shoulder

Superior Sublabral Recess
= variationsin depthof sulcusbetween glenoidrim infraspinatus
+ labrum
Location: 12 o'clockpositionat thesiteof biceps
tendonattachment
typel = nosulcus
typell = smallsulcus teres minor
typelll = deepprobe-patent sulcus
{ maybe continuous withsublabralforamen
r/ bestvisualizedon coronalCT / MR Rotator Cuff
(dorsal aspect)
DDx: typell SLAPlesion
 Anatomyand Metabolismof Bone 33

ROTATORCUFF MUSCLES radioscaphocapitate

mnemonic.'"SlTS"
Supraspinatus radioluno-
triquetral
lnfraspinatus - long
Teresminor radio-
Subscapularis lunate
coracoacromial arch
luno-
triquetral
acromion coracoacromiallie.
short
scapho- radiolunate
lunate radioscapho-
lunate
l
I
I
Carpal Bones and Ligaments
(volar aspect)

CARPAL BONES
mnemonic.'"SomeLoversTry Positions
ThatTheyGan't
Handle"
proximalrow distalrow
Scaphoid Trapezium
Lunate Trapezoid
Triquetrum Capitate
Coracoacromial Arch
(lateral aspect) Pisiform Hamate

0 Rememberthat trapeziumcomes beforetrapezoidin

the dictionaryas well!

OCCURRENCE OF BONECENTERS
AT ELBOW
mnemonic.'"CRITOE"
Gapitellum 1 year (3-6 months)
Radialhead 4 years (3-6 years) -
Internalhumeralepicondyle 7 years (5-7 years,
lastto fuse) t"r
'l"I)l*-"
Trochlea 10 years (9-10years)
Olecranon 10 years (6-10years) trapeziumi
Externalhumeralepicondyle 11 years (9-13years) scaPhoid hamate
I
capltate
mnemonic.' "Nelson'sX: 1,7, 10, 11 years"
Carpal T[nnel

Lister extensor extensor extensof

extensor carpl tubercle pollicis digitorum indicis
radialis brevis longus
2 3 4 5
extensor disiti
extensor carpi mmlml
radialis lonsus
extensor
extensor carpi ulnaris
pollicis brevis

abductor
@ pollicis longus

Occurrence of Bone Centers Wrist CrossSectionof Distal RadioulnarJoint

(numbers in years) with the 6 ExtensorCompartments
 MuscleAttachmentsof Shoulder
Name of Muscle Origin lnsertion
Deltoid lateralthird of clavicle deltoidtuberosityof humerus
lateralborderof acromion deltoidtuberosityof humerus
lower part of spinousprocessof scapula deltoidtuberosityof humerus
Subscapularis medial213of costalsurfaceof scapula superioraspectof lessertubercleof humerus
Pectoralismajor
- clavicularportion medialhalf of clavicle crest of greatertubercleof humerus
- sternocostalportion manubrium+ corpusof sternum crest of greatertubercleof humerus
- abdominalportion anteriorsheathof rectusabdominis crest of greatertubercleof humerus
Pectoralisminor 2nd l3rd-Sth ribs superomedialaspectof coracoidprocess
Bicepsbrachii
- longhead supraglenoidtubercleof scapula tuberosityof radius
- short head tip of coracoidprocess tuberosityof radius
Coracobrachialis tip of coracoidprocess medialsurfaceof middtethird of humerus
Supraspinatus supraspinatusfossa of scapula greatertubercleof humerus,highestfacet
Infraspinatus infraspinatusfossa of scapula greatertubercleof humerus,middlefacet
Teresminor upper 213ol lateralborderof scapula greatertubercleof humerus,lowerfacet
Teres major dorsumof inferiorangle of scapula inferiorcrest of lessertubercleof humerus

tensor
fasciae
latae

t
I adductor
I
t longus +
brevis
pectineus

gracilis

Hip Flexors

Muscle Attachments of Thigh

Name of Muscle Origin lnsertion
Gracilis inferiorpubicramus pesansennus
Semimembranosus ischialtuberosity medialtibialcondyle
Semitendinosus ischialtuberosity pes anserinus
Bicepsfemoris
- longhead ischialtuberosity fibularhead
- shorthead laterallineaaspera fibularhead
Adductor
- longus superiorpubicramus mediallineaaspera
- magnus inferiorpubic ramus mediallineaaspera
Sartorius anteriorsuperioriliacspine pes anserinus
Quadriceps
- rectus anteriorinferioriliacspine patellartendon
- vastuslateralis greatertrochanter patellartendon
- vastusmedialis medialintertrochanteric line patellartendon
lliopsoas
- iliacus i l i um lessertrochanter
- psoas lumbarspine lessertrochanter
Tensorfasciaelatae anteriorsuperioriliacspine anterolateraltibia
 Anatomyand Metabolism
of Bone 35

int. abdominal obl. m. rectus abdominis m.

ext. abdominal obl. m. psoasm. femoral n.
transverse r 17 rliohypogastric n.
abdominal m.
'l
I t
l , /
genitofemoral n. iliacus m.
\.1 obturator n.
t
I
\t
-t
t - / -
t
/
60o lateral
femoral
cutaneusn.
gluteus
medius m. lumbosacral
plexus
psoasm. L4 root
gluteus maximus m. erector sprnaem.
quadratuslumborum m. erector spinae m.

Cross Section through L4-5 CrossSectionthroughLs-Sl

ext. abdominal obl. m. rectus abdominis m. tensor fasciae

int. abdominal obl. m. femoral n.
latae m.
femoral n.
gluteus obturator n.
mlnlmus m.
obturator
\-;.=i- internus m.
l-/',
obturator n.
lumbosacral sciatic n.
gluteus
trunk
medius m. 1 l
pudendal n.
S1
nerve root piriform m.

S2
nerve root
iliopsoasm.

CrossSectionthroughS1-2 CrossSectionthrough 54

iliopsoasm.
femoral n. sartorius m.
sartonus m.
rectus femoris m.
tensor fasciae
Iatae m. tensor fasciae
pectineusm.
obturator n. latae m.
gluteus
mlnlmus m. obturator obturator
internus m. mtemus m.
,.).-+
gluteus levator
medius m. sclatlc n. ani m.

gluteus maximus m. sciatic n.

gemellus m.

Cross Section through Actabular Roof Cross Section through Greater Trochanter
36 Radiology Review Manual

rectus femoris m. sartonus m. rectus femoris m.

tensor fasciae sanonus m.
latae m.
pectineusm.

adductor pectmeusm.
iliopsoasm. magnusm.

obturator obturator
quadratus
extemus m. intemus m.
femoris m.

Cross Section through Level of Obturator Foramen Cross Section through Level of Minor Thochanter

vastus femoral n. branches

medialis m.
deep
rectus femoris m. deep
femoral a. + v.
vastus rectus femoris m. femoral a. + v.
superficial
intermedius m. femoral a. + v. superficial
vastus
tensor fasciae intermedius m. femoral a. + v.
pectineusm.
latae m.
adductor sartorius m.
longus m.
adductor
gracilis m. longus m.

adductor gracilis m.
brevis m. adductor
Vastus brevis m.
adductor
vastus magnus m. lateralis m.
lateralis m. adductor
semimembranosusm. magnus m.
(shorthead) (long head)
gluteusmaximus m. sciatic n. semitendinosusm. biceps femoris m.
biceps femoris m. semitendinosusm.

Cross Section through Level of Proximal Thigh Cross Section through Level of Mid Thigh

iliopubic line
of anterior column

obturator
fat pad teardrop
= medial
anterior
acetabular
acetabularrim
wall

posterior
acetabularrim
iliopsoas
fat pad ilioischial line
of posterior column

Fat Padsof the Rigth Hip in PerfectAP Position Bony Landmarks of the Rigth Hip
in Perfect AP Position
 Anatomyand Metabolism
of Bone 37

LOWEREXTREMITY Insertion: most distal+ anterioraspectof innerface

PesAnserinus of medialfemoralcondyle
= [pes, Latin= foot; anser, Latin= goose] r/ ttrict<dark band slightlyposteriorlyconvex(arclire
= tendinousconfigurationof 3 flexors+ medialrotators courseon sagittalMR with knee in extension)
^/ medialto ACL (coronalMR)
of kneejoint attachinginferomedially
to tibialtuberosity
mnemonic.' "Say GracSe before eating goose"
1. Sartoriustendon(anterior)
2. Gracilistendon(middle) CollateralLigamentsof KneeJoint
3. Semitendinosus tendon(posterior) Medial (Tibial) CollateralLigament
Origin: justdistalto adductor
tubercle
offemur
lliotibialTract Insertion: anteromedialface of tibia distalto levelof
= stror'tgstabilizingband of deep fasciacomposedof tibialtubercleabout5 cm belowjoint line
(a) deep portion:
the fusionof aponeuroticcoveringsof:
1. Tensorfascialata meniscofemoral ligament
2. Gluteusmaximusm. meniscotibial ligaments
(b) superficialportion
3. Gluteusmediusm.
Insertion: verticalband from femoralepicondyleto pes
(a) supracondylartubercleof lateralfemoralcondyle anserinus
(b) lateraltubercleof tibia = Gerdytubercle(mainsite) posteriorobliqueligament= posterior
(c) patella+ patellarligament obliquebandfrom femoralepicondyleto
semimembranosus tendon
r/ deep and superficialdark bands separatedby a thin
Hamstrings
bursa + fatty tissue (on coronalMR)
(a) medialhamstring
1. S em im em b ra n o s u
ms.
2. Semitendinosus m.
Function: flexion+ medialrotationof knee joint
Lateral(Fibular)CollateralLigament
(b) lateralhamstring Origin: lateralaspectof lateralfemoralcondyle
= long + short head of bicepsfemorism. Insertion: styloidprocessof fibularhead
r/ bicipitaltendon + iliotibialband join lateralcollateral
Function: flexion+ lateralrotationof kneejoint
ligament

CruciateLigaments
0 Both cruciateligamentsare intracapsularbut
extrasynovial!
Anterior CruciateLigament (ACL)
Function: limitsanteriortibialtranslation Tibialis anterior
Origin: innerface of lateralfemoralcondyle extensor
Insertion: noncartilaginous regionof anterioraspect Hallucislongus
of intercondylar eminenceof tibia Tibialis
Anatomy: severaldistinctbundlesof fibers extensor posterior
(1) posteriorbulk= spiralingtogetherat Digitorum flexor
longus
femoralorigin Digitorum
(2) anteromedial bundledivergingat tibial longus
insertion flexor
r/ tnin solidtaut dark band (sagittalMR with knee in Hallucis
peroneuslongus longus
extension)almostparallelto intercondylarroof
(= Blumensaatline) peroneusbrevis 'Achilles tendon
r/ tnin hypointenseband parallelto inneraspectof
lateralfemoralcondyle+ fanlikeconfiguration CrossSectionthrough Distal Right Leg
towardtibialspine (coronalMR)
r/ tninovoidhypointense bandproximally, elliptical
, configuration distallywith higherintensity(axialMR)
r/ greatersignalintensitythan posteriorcruciate
ligament(due to anatomy)

PasteriorCruciateLigament (PCL) mnemonic for posterior tendons: "Tom, Dick and Hafry"
Function:limits
posteriortibial
translation Tibialisposterior
Origin: in a depressionposteriorto intercondylar Digitorumlongus(flexor)
regionof tibia belowjoint surface Hallucislongus(flexor)
38 Radiology Review Manual

transversefascia flexor digitorum transversefascia

of midfoot longus m. adductor offorefoot
quadratus hallucis m.
plantae m.

medial
lateral septum septum

lateral
septum
abductor
flexor hallucis hallucis m.
brevis m.
flexor digiti lumbrical mm. +
abductor.digiti plantar .
minimi m. tendon of flexor
mlnlml m. flexor digitorum flexor hallucis aponeurosls
digitorum longus
brevis m. lonqus m. et brevis m.
plantar aponeurosis

Plantar Compartmentsof the Midfoot Plantar Compartments of the Forefoot

Medial compartment = borderedby medial septum(extendingfrom plantaraponeurosisto navicularbone,medial cuneifom bone,and
lateml borderof plantarsurfaceof 1stmetatarsalbone);
containsabductorhallucism. + flexor hallucisbrevism. + flexor hallucislongustendon
Lateral compartment = bordered by lateral septum (extending from plantar aponeurosisto medial surface of 5th metatarsal bone);
contains abductor m. + short flexor m. + opponens m. of 5th toe
Central compartment = bord.eredby medial + lateral septa; communicates directly with posterior compartment of calf;
subdivided by horizontal septa: adductor hallucis m. separatedfrom quadratusplantae m.
contains flexor digitorum brevis m. + flexor digitorum longus tendon + quadratusplantae m. + lumbricales mm.'
+ adductor hallucis m.
Deep subcompafiment = bordered by transversefascia of forefoot; separatedfrom quadratusplantae m.; contains adductor hallucis m.

Accessory Ossicles of the Foot

1 Os talotibiale 10 Os tibialeexternum 19 Os cuneometatarsale I Plantare
2 Os supratalare 11 Trigonum 20 C uboi dessecundar ium
3 Os supranaviculare 12 Os accessorium supracalcaneum 21 Os trochlearecalcanei
4 Os infranaviculare 13 Os subcalcis 22 Sesamoidtalus- int. malleolus
5 Os intercuneiforme 14 Os peroneum 23 Os subtibiale
6 Os cuneometatarsale ll dorsale 15 Os vesal i anum 24 Os sustentaculi
7 Os intermetatarsale 16 Talusaccessorius 25 Os retinaculi
8 Secondarycuboid 17 Os cuneonaviculare mediale 26 Os subfibulare
9 Calcaneussecundarius 18 Sesamumtibialeanterius 27 Talussecundarius
 Anatomyand Metabolismof Bone 39

Calcaneal Pitch
= Calcaneal Inclination Angle
= determines longitudinal arch of foot;
angle betweenline drawn along the inferior
border of calcaneusconnecting the anterior
and posterior prominences + line
representing the horizontal surface

5-10"
I frs-3so
lf
Y
Talocalcaneal Angle
on AP View
= KITE ANGLE = the midtalar and
Boehler Angle midcalcaneal lines parallel the lst + 4th
= angle between first line drawn from metatarsals;angle is greater in infants
posterosuperiorprominence of calcaneus
Intermetatarsal Angle
anteriorly to sustentaculumtali + second = amount that lst + 2nd metatarsals
line drawn from anterosuperior
diverge from each other
prominence posteriorly to sustentaculum
tali; measuresintegrity of calcaneus

n
---9flfi$n N ( J

::+h'#ff
\ )(lrr
s-10"
Talocalcaneal Angle on LAT
View
= anglebetweenlines drawn through mid-
transverseplanesoftalus + calcaneus;the
midtalar line parallelsthe longitudinal axis
II
Heel Valgus
cannot be measured directly on
radiographs but inferred from the
talocalcaneal angle and estimated on
\tnt
Angle of Metatarsal Heads
= obtuse angle formed by lines tangential
of the first metatarsal coronal CT sections to metatarsalheads
 DISORDERS
BONEANDSOFT.TISSUE

AC HO NDRO G E N ES IS Cx: apnea+ suddendeath (due to compression

= autosomalrecessivelethalchondrodystrophy of spinalcord + lowerbrain stem)
characterizedby extrememicromelia,shorttrunk, large @ Chest
cranium r/ anteroposterior narrowingof chest
TRIAD: (1) severeshort-limbdwarfism ! short anteriorlyflaredconcaveribs
(2) lack of vertebralcalcification { squaringof inferiorscapularmargin
(3) large head with normal/ decreasedcalvarial @ S pi ne
ossification ^/ hypoplasticbullet-/ wedge-shapedvertebra:
Birlhprevalence: 2.3:100,000 { roundedanteriorbeakingof vertebrain upper
Path: disorganization of cartilage lumbarspine(DDx:Hurlerdisease)
A. TYPE | = Parenti-Fraccaro disease { decreasedvertebralheight
= defectiveenchondral+ membranousossification r/ scallopedposteriorlyconcavevertebralmargin
r/ completelack of ossificationof calvarium+ spine { scoliosis:
{ thoracolumbar angularkyphosis(gibbus)
, + pelvis r/ exaggeratedsacrallordosis
r/ absentsacrum+ pubicbone
r/ extremelyshort long boneswithoutbowing, { stenosisof lumbarspine:
especiallyfemur,radius,ulna { narrowingof interpedicular spacedue to laminar
r/ tnin ribs with multiplefractures(frequent) thickening
B. TYPE ll = Langer-Saldino disease { ventrodorsalnarrowingof spinedue to short
= defectiveenchondralossificationonly pedicles
^/ good ossificationof skullvault rl butging/ herniationof intervertebral disks
r/ nonossification of lower lumbarvertebrae+ sacrum r/ wide intervertebral foramina
r/ short + stubbyhorizontalribs withoutfractures @ Pelvis
. protuberantabdomen
. oftensubcutaneousedema . prominentbuttocks
r/ irregularflaredmetaphyses(esp.humerus) ' rollinggait from backwardtilt of pelvisand hip
r/ shorttrunk with narrowchest + protrudingabdomen joints
r/ redundantsoft tissues { squareflattenediliac bones= tombstone
{ polyhydramnios (common) configuration
{ increasein HC:ACratio { "champagneglass"-shaped pelvicinlet
Prognosis; lethal often in utero / within few hours or days ./ lack of flaringof iliacwings
after birth (respiratoryfailure) ./ horizontalacetabula(= flat acetabularangle)
DDx: often confusedwith thanatophoricdwarfism { smallsacrosciaticnotch
@ Extremities
ACHONDROPLASIA . shortstubbylimbs+ fingers
HeterozygousAchondroplasia . tridenthand = separationof 2nd + 3rd digit and
0 Prototypeof rhizomelicdwarfism! inabilityto approximate3rd + 4th finger
= autosomaldominant/ sporadic(80%)diseasewith . limitedrangeof motionof elbow
quantitativelydefectiveendochondralbone formation; ./ predominantly rhizomelicmicromeliaof longbones
relatedto advancedpaternalage; epiphyseal (femur,humerus):
maturation+ ossificationunaffected ./ "trumpet"appearanceof long bones
lncidence; 1:26,000-66,000births,most commonof = shorteningwith disproportionate metaphyseal
lethalbone dysplasias;M < F flaring(actuallynormalwidth of metaphysis)
. normalintelligence + motorfunction { short femoralnecks
. neurologicdefects r/ timObowing
. classicallycircusdwarfs r/ "ball-in-socket" epiphysis= broadV-shapeddistal
@ Skull femoral metaphysis in which epiphysisis
. flat nasalbridge(hypoplasticbase of skull) incorporated
. macrocephaly+ brachycephalywith enlarged r/ nign positionof fibularhead (= disproportionately
bulgingforehead(nonprogressive hydrocephalus) longfibula)
. relativeprognathism { shortulnawiththickproximal+ slenderdistalend
./ largecalvariumwith frontalbossing r/ brachydactyly(shorttubularbonesof hand + feet),
{ depressionof nasion especiallyshort proximal+ middlephalanges
r/ broad mandible
{ constricted basicranium + smallforamenmagnum: OB-US (diagnosable>21-27th week GA):
r/ communicatinghydrocephaluscausedby r/ shorteningof proximallongbones: femurlength
obstructionof basalcisterns+ aqueduct <99th percentilebetween21 and 27 weeks MA
 Boneand Soft-IissueDisorders 41

r/ increasedBPD,HC, HC:ACratio @ Skull

./ decreasedFL:BPDratio ./ wormianbones
r/ normalmineralization, no fractures r/ craniosynostosis
{ normalthorax+ normalcardiothoracicratio ! basilarimpression
r/ three-pronged(= trident)hand = 2nd + 3rd + 4th r/ protuberantocciput
fingerof similarlyshortlengthwithoutcompletely r/ resorptionof alveolarprocesses+ loss of teeth
approximatingeach other (= PATHOGNOMONIC) @ Spine
Cx: (1) Hydrocephalus+ syringomyelia(small d spinalosteoporosis+ fracture
foramenmagnum) r/ kyphoscoliosis + progressivedecreasein height
(2) Recurrentear infection(poorlydeveloped
facialbones) ACROMEGALY
(3) Neurologiccomplications(compressionof Etiology: excess growth hormonedue to eosinophilic
spinalcord,lowerbrainstem,caudaequina, adenoma/ hyperplasiain anteriorpituitary
nerveroots): apnea and suddendeath . gigantismin children:advancedbone age + excessive
(4) Crowdeddentition+ malocclusion height(DDx: Soto syndrome of cerebralgigantism
Prognosis; long life = largeskull,mentalretardation,cerebralatrophy,
DDx: variousmucopolysaccharidoses advancedbone age)
r/ osseousenlargement(phalangealtufts,vertebrae)
HomozygousAchondroplasia r/ flaredends of long bone
= hereditaryautosomaldominantdiseasewith severe r/ cysticchangesin carpals,femoraltrochanters
featuresof achondroplasia(disproportionate
limb ri osteoporosis
shortening,more markedproximallythan distally) @ Hand
Risk: marriageof two achondroplaststo each other . spadelikehand
! wideningof terminaltufts
^/ largecraniumwith short base + smallface
@ Skull
{ flattenednose bridge r/ prognathism(= elongationof mandible)in few cases
r/ short ribs with flaredends r/ sellarenlargement + erosion
r/ hypoplasticvertebralbodies r/ enlargementof paranasalsinuses: largefrontal
r/ decreasedinterpedicular distance
r/ shortsquaredinnominatebones , sinuses(75%)
ri calvarialhyperostosis(especiallyinnertable)
r/ flattenedacetabularroof ./ enlargedoccipitalprotuberance
{ small sciaticnotch @ Vertebrae
r/ short limb boneswith flaredmetaphyses r/ posteriorscallopingin 30% (secondaryto pressure
r/ short,broad,widelyspacedtubularbones of hand of enlargedsoft tissue)
Prognosis; oftenstillborn;lethalin neonatalperiod { anteriornew bone
(fromrespiratoryfailure) ri loss of disk space (weakeningof cartilage)
DDx: thanatophoricdysplasia @ Soft tissue
r/ neel pad >25 mm
@ Joints
ACROCEPHALOSYN
DACTYLY r/ prematureosteoarthritis(commonlyknees)
= syfldromecharacterizedby
(1) increasedheightof skullvaultdue to generalized ACTINOMYCOSIS
craniosynostosis (= ocrocephaly,oxycephaly) = chronicsuppurativeinfectioncharacterizedby formation
(2) syndactylyof fingers/ toes of multipleabscesses,drainingsinuses,abundant
granulation tissue
TypeI Apertsyndrome= ?crocephalosyndactyly Organism; Actinomycesisraelii,Gram-positive
Typell Vogt cephalosyndactyly anaerobicpleomorphicsmall bacteriumwith
Typelll Acrocephalosyndactyly
with asymmetryof skull proteolyticactivity,superficiallyresembling
+ mild syndactyly the morphologyof a hyphalfungus(Gomori
TypelV Wardenburgtype methenamine silverstain-positivefilaments);
TypeV Pfeiffertype closelyrelatedto mycobacteria
Histo: mycelialform in tissue;rod-shapedbacterialform
ACROOSTEOLYSIS, FAMILIAL normallyinhabitingoropharynx(dentalcaries,
dominantinheritance gingivalmargins,tonsillarcrypts)+ Gl tract
Age: onsetin 2nd decade; M:F = S:1 Predisposed: individualswith very poor dentalhygiene,
. sensorychangesin hands+ feet
immunosuppressedpatients
. destructionof nails Location: mandibulofacial > intestinal> lung
. joint hypermobility Types:
' swellingof plantarof foot with deep wide ulcer+ ejection (1) Mandibulo-/ cervicofacialactinomycosis(55%)
of bone fragments Cause: poor oral hygiene
 . drainingcutaneoussinuses
. "sulfurgranules"in sputum/ exudate= coloniesof
organismsarrangedin circularfashion= mYGelial
clumpswith thin hyphae1-2 mm in diameter
{ osteomyelitisof mandible(mostfrequentbone
involved)with destructionof mandiblearound
tooth socket
r/ no new-boneformation
{ spreadto soft tissuesat angle of jaw + into neck
(2) Abdominopelvic / ileocecalactinomycosis(20%)
Cause: rupture/ surgeryof appendix;IUD use
Location; initiallylocalizedto cecum/ appendix
. fever,leukocytosis, mildanemia
. weightloss,nausea,vomiting,pain
. c hr onics i n u si n g ro i n
ri totOthickening+ ulcerations (resembling Crohn
disease)
r/ ruptureof abdominalviscus(usuallyappendix)
^/ fistulaformation
r/ abscessin liver (15%),retroperitoneum, psoas
"sulfurgranules"
muscle(containingyellow
= 1-2-mm colonyof gram-positivebacilli)
(3) Pleuropulmonary actinomycosis (15%)
Cause: aspirationof infectedmaterialfrom
oropharynx/ hematogenoussPread
Predisposed: alcoholics
Histo: massesof PMN leukocytescontaining
roundactinomycotic / sulfurgranules
surroundedby a rim of granulation
@ Lung
. drainingchestwall sinuses(spreadthrough
fascialplanes)
r/ enhancingextensivetransfissuralchronic
segmentalconsolidation(acuteairspace
pneumoniarare);usuallyunilateral+ upper
lobe predominance
{ cavitarylesion(abscess)with ringlike
enhancement
r/ fibroticpleuritis
r/ chronicpleuraleffusioni empyema
CT:
{ centralareas of low attenuationwithin
consolidation
{ adjacentpleuralthickening
DDx: carcinoma,tuberculosis, bacterial/ fungal
pneumonia
@ Vertebra+ ribs
r/ destructionof vertebrawith preservationof disk
+ smallparavertebralabscesswithout
calcification(DDx to tuberculosis:disk
destroyed,large abscesswith calcium)
r/ thickeningof cervicalvertebraearoundmargins
r/ destruction/ thickeningof ribs
(a) Mixedorgans(10%)
@ Tubularbonesof hands
r/ destructivelesionof mottledpermeatingtype
r/ cartilagedestruction+ subarticularerosive
defectsin joints(simulating TB)
@ Skin,brain,pericardium
Rx: surgicaldr6bridement + penicillin
ADAMANTINOMA
= (MALTGNANT) ANGIOBLASTOMA
= locallyaggressive/ malignantlesion
Histo: pseudoepithelial cell masseswith peripheral
columnarcells in a palisadepailernwith varying
amountsof fibrousstroma;areasof squamous/
tubular/ alveolar/ vesseltransformation;
prominentvascularity; resemblesameloblastoma
of the jaw
Age: 25-50 years,commonestin 3rd-4th decade
. frequentlyhistoryof trauma
. l ocal sw el l i ng
+ P ai n
Location: middle113of tibia(90%),fibula,ulna,carpals,
metacarpals, humerus,shaftof femur
r/ eccentricroundosteolyticlesionwith scleroticmargin,
may have additionalfoci in continuitywith majorlesion
(cHARACTERISTIC)
^i may show mottleddensity
rl bone expansionfrequent
{ oftenmultiple
Prognosis; tendencyto recurafter localexcision;after
severalrecurrencespulmonarymetastases
may develoP
DDx: fibrousdysplasia(possiblyrelated)
A IN H U M D IS E A S E
= DACTYLOLYSIS SPONTANEA
[ainhum= fissure,saw,sword]
tissue Etiology: unknown
Histo: hyperkeratotic epidermiswith fibroticthickeningof
collagenbundlesbelow;chroniclymphocytic
inflammatory reactionmay be present;arterialwalls
may be thickenedwith narrowedvessellumina
Incidence: up Io 2"1"
Age: usuallyin malesin 4th + 5th decades;Blacks
(WestAfrica)+ their Americandescendants;M > F
' deep soft-tissuegrooveformingon medialaspectof
plantarsurfaceof proximalphalanxwith edemadistatly
. painfululcerationmaYdeveloP
Location: mostlysth / 4th toe (rarelyfinger);near
interphalangealjoint; mostlybilateral
./ sharplydemarcatedprogressivebone resorptionof
distal/ middlephalanxwithtaperingof proximalphalanx
to completeautoamputation (afteran averageof 5 years)
i osteoporosis
Rx: earlysurgicalresectionof groovewith Z-plasty
DDx: (1) Neuropathic disorders(diabetes,leprosy,
sYPhilis)
(2) Trauma (burns,frostbite)
(3) Acroosteolysis from inflammatoryarthritis,
infection,polyvinylchlorideexposure
(4) Congenitallyconstrictingbands in amnioticband
syndrome
AMYLOIDOSIS
= extracellulardepositionof a chemicallydiversegroup of
proteinpolysaccharides in body tissues
(Br-microglobulin);tendsto form aroundcapillaries
+ endothelial cellsof largerbloodvesselscausing
vascularobliteration
ultimately with infarction

Fr-microglobulin = low-molecular-weight serumprotein
not filteredby standarddialysis
membranes
Path: stainswith Congo red
At risk: patientson long-termhemodialysis
. bone pain (eg,shoulderpain)
. periarticularrubberysoft-tissueswelling+ stiffness
(shoulders,hips,fingers)
. carpal-tunnelsyndrome(commonlybilateral)
. BenceJones protein(withoutmyeloma)
1. Synovial-articular pattern= amVloid arthropathy
Location: cervicalspine,hip, shoulder,elbow
r/ juxtaarticularsoft-tissueswelling(amyioiddeposited
in synovium,jointcapsule,tendons,ligaments)
+ extrinsicosseouserosion
r/ mild periarticularosteoporosis
r/ subchondralcysts + well,definedscleroticmargin
^/ loint space preserveduntil late in courseof disease
r/ subluxationof proximal humerus+ femoralneck
MR:
{ extensivedepositionof abnormalsoft tissueof low
to intermediate signalintensityon TlWl + T2Wl
coveringsynovialmembrane,filfingsubchondral
defects,extendinginto periarticulartissue
./ loint effusion
DDx: inflammatoryarthritis
2. Diffusemarrowdeposition
j generalizedosteoporosis
ri coarsetrabecularpattern(DDx: sarcoidosis)
r/ pathologiccollapseof vertebralbody may occur
3. Localizeddestructivelesion(rarestform)
Location: appendicular> axialskeleton
./ focal medullarylytic lesionwith endostealscalloping
(+ secondaryinvasion+ erosionof articularbone)
= amyloidoma
Cx: pathologicfracture
ANEURYSMAL
BONECYST
= eXponSile lesionof bone containingthin-walledblood-
filledcysticcavities;name derivedfrom roentgen
appearance
Etiology:
(a) primaryABC (65-99%)
localcirculatorydisturbanceas a resultof trauma
(b) secondaryABC (1-95%)
arisingin preexisting bonetumorcausingvenous
obstruction / arteriovenous
fistula:giantcelltumor
(39%),osteoblastoma, chondroblastoma, angioma,
telangiectaticosteosarcoma,solitarybone cyst,
fibrousdysplasia,xanthoma,chondromyxoid
fibroma,nonossifyingfibroma,metastaticcarcinoma
Histo: "intraosseousarteriovenousmalformation,, with
honeycombedspacesfilledwith blood + linedby
granulationtissue/ osteoid;areas of free
hemorrhage; sometimesmultinucleated giant
cells;solidcomponentpredominatesin S-7%
Boneand Soft-TissueDisorders 43
Types:
1. IN TR A OS S E OUASB C
= primarycystic/ telangiectatic tumor of giant cell
family,originating in bone marrowcavity,slow
expansionof cortex;rarelyrelatedto historyof
trauma
2. EXTRAOSSEOUSABC
= posttraumatichemorrhagiccyst; originatingon
surfaceof bones,erosionthroughcortexinto
marrow
Age: peak age 16 years (range 10-30 years);
i n 7 5 h < 2 0 y e a r s ;F > M
. pain of relativelyacute onsetwith rapid increaseof
severityover 6-12 weeks
. t historyof trauma
. neurologicsigns(radiculopathy to quadriplegia)if in
spine
Location: (a) spine (12-30%)with slightpreditectionfor
posteriorelements;thoracic> lumbar>
cervicalspine (22%);involvementof
vertebralbody (40-90%);may involvetwo
contiguousvertebrae (25%)
(b) long bones: eccentricin metaphysisof
femur,tibia,humerus,fibula;pelvis
{ purelylyticeccentricradiolucency
r/ aggressiveexpansileballooninglesionof "soap-bubble"
pattern+ thin internaltrabeculations
r/ rapid progressionwithin6 weeks to 3 months
r/ scleroticinner portion
{ almostinvisiblethin cortex(CT shows integrity)
r/ tumor respectsepiphysealplate
{ no periostealreaction(exceptwhen fractured)
CT:
r/ "blood-filledsponge"= fluid-fluidi hematocritlevels
due to bloodsedimentation (in 10-35%)
MR:
r/ multiplecysts of differentsignalintensityrepresenting
, differentstagesof bloodby-products
! low-signalintensityrim = intactthickenedperiosteal
membrane
NUC:
r/ "doughnutsign" = peripheralincreaseduptake(64%)
A ngi o:
r/ hypervascularity in lesionperiphery(in 7S%)
Prognosis: 20-30/" recurrence rate
Rx: preoperativeembolotherapy; completeresection;
radiationtherapy(subsequentsarcomapossible)
Cx: (1) pathologicfracture(frequent)
(2) extraduralblockwith paraplegia
DDx: (1) Giantcelltumor(particularly in spine)
(2) Hemorrhagiccyst (end of bone / epiphysis,not
expansile)
(3) Enchondroma
(4) Metastasis(renalcell + thyroidcarcinoma)
(5) Plasmacytoma
(6) Chondro-and fibrosarcoma
(7) Fibrousdysplasia
(8) Hemophilicpseudotumor
(9) Hydatidcyst
 Radiology Review Manual

ANGIOMATOSIS AN KYLOSING SPONDYLITIS

= diffuseinfiltrationof bone / soft tissueby = autoimmunediseaseof unknownetiologyprimarily
hemangiomatous / lymphangiomatous lesions affectingaxialskeleton
Age: first 3 decadesof life A ge: 15-35years;M:F = 3:1-10:1;
May be associated with: Caucasians:Blacks = 3:1
chylothorax, chyloperitoneum, lymphedema, Associatedwith: (1) ulcerativecolitis,regionalenteritis
hepatosplenomegaly, cystichygroma (2) iritisin 25%
(3) aorticinsufficiency+ atrioventricular
A. OSSEOUSANGIOMATOSIS(30-40%) conductiondefect
. indolentcourse . HLA-B27 positivein 96%
Location: femur > ribs > spine > pelvis> humerus> . insidiousonset of low back pain + stiffness
scapula> other long bones > clavicle Path: involvessynovial+ cartilaginousjoints and sites of
{ osteolysiswith honeycomb/ latticework("hole- ligamentousattachment
appearance
within-hole") Location:
{ may occur on both sides of ioint (a) axialskeleton:sacroiliacioints,thoracolumbar
DDx: solitaryosseoushemangioma + lumbosacraljunctions
B. CYSTICANGIOMATOSIS 0 HALLMARKis sacroiliacjointinvolvement!
= extensiveinvolvementof bone (b) peripheralskeleton (10-20%): sternaljoint,
Histo: endothelium-lined cystsin bone symphysispubis,hip,glenohumeral joint
Age: peak 10-15 years;rangeof 3 monthsto (c) tendinousinsertionsin pelvis+ proximalfemur
55 Years Temporalcourse: initialabnormalities of sacroiliacjoints
Location: longbones,skull,flat bones + thoracolumbar junctionwith gradual
r/ multipleosteolyticmetaphyseallesionsof 1-2 mm to involvement of remainderof sPine
severalcm with fine scleroticmargins+ relative @ S kul l
sparingof medullarycavity { temporomandibular jointspacenarrowing,erosions,
rl may show overgrowthof long bone osteophytosis
r/ endostealthickening @ Hand (30%)
r/ sometimesassociatedwith soft{issue mass Targetarea: MCP, PlP, DIP
+ phleboliths { exuberantosseousproliferation
{ chylouspleuraleffusionsuggestsfatal prognosis { osteoporosis,joint space narrowing,osseous
DDx: (otherpolyostoticdiseasessuch as) erosions(deformities lessstrikingthan in rheumatoid
histiocytosisX, fibrousdysplasia,metastases, arthritis)
Gaucherdisease,congenitalfibromatosis, @ Sacroiliac joint/ symphysispubis
Maffucisyndrome,neurofibromatosis, { initiattysclerosisof joint marginsprimarilyon iliac
enchondromatosis side (bilateral+ symmetriclate in disease,may be
(60-70%)
ANGIoMATOSIS
c. soFT-TrssuE unilateral+ asymmetricearly in disease)
= VISCERALANGIOMATOSIS { later irregularities + wideningof joint (cartilage
. poor prognosis destruction)
D. ANGIOMATOUS SYNDROMES r/ bony fusion
1. Maffucisyndrome @ Pelvis
"whiskering":ischialtuberosity,iliaccrest,
2. Osler-Weber-Rendusyndrome { periostitic
disease
3. Klippel-Trenaunay-Weber ischiopubicrami,greaterfemoraltrochanter,external
syndrome
4. Kasabach-Merritt occipitalprotuberance,calcaneus
5. Gorhamdisease @ Spine
{ squaring= straightened/ convexanteriorvertebral
margins= erosiveosteitisof anteriorcorners
ANGIOSARCOMA { "shinycorner"= Ieactivesclerosisof cornersof
= aggressivevascularmalignancywith frequentlocal vertebralbodies
recurrence+ distantmetastasis rl disXitis= erosiveabnormalitiesof diskovertebral
Histo: vascularchannelssurroundedby junction
hemangiomatous / lymphomatouscellular ./ .diskalballooning"= biconvexshape of intervertebral
elementswith highdegreeof anaplasia disk relatedto osteoporoticdeformity+ diskal
A ge: M : F = 2: 1 calcification
Associatedwith: Stewart-Treves syndrome { marginalsyndesmophyteformation= thin vertical
= angiosarcomawith chroniclymphedemadeveloping radiodensespiculesbridgingthe vertebralbodies
in postmastectomy patients = ossification of outerfibersof annulusfibrosus
Location: skin (33%);soft tissue (24%\; (NOT anteriorlongitudinalligament)
bone (6/"):tibia(23%),femur(18%), ./ "bamboospine"on AP view = undulatingcontour
humerus(13%),pelvis(7%) due to syndesmoPhYtosis
DDx: hemangioendothelioma, hemangiopericytoma Cx: proneto fractureresultingin pseudarthrosis
 Boneand Soft-TissueDisorders 45

{ asymmetricerosionsof laminar+ spinousprocesses ! thickeningof posteriortibial+ fibularcortices

of lumbarspine ! minorradioulnarbowing
r/ ossificationof supraspinous+ interspinousligaments: r/ kyphoscoliosis
r/ "daggerSign"= singleradiodenselineon AP view { extensivedural calcification
r/ "trolley-track" sign on AP view = centralline of DDx: Lueticsabershin (bowingat lowerend of tibia
ossificationwith two laterallinesof ossification + anteriorcorticalthickening)
(apophysealjoint capsules)
r/ apophyseal+ costovertebraljoint ankylosis(on APERTSYNDROME
obliqueviews) = ACROCEPHALOSYNDACWLY
type I
! dorsalarachnoiddiverticulain lumbarspinewith Frequency; 5.5:1,000,000neonates
erosionof posteriorelements(Cx: cauda equina Etiology: autosomaldominantwith incomplete
syndrome) penetrance;sporadic(in majority)
r/ atlantoaxialsubluxation Associated with CNS anomalies:
@ Chest megalocephaly, gyral abnormalities,hypoplasticwhite
lncidence: 1o/ool patientswith ankylosingspondylitis matter,heterotopicgray matter,frontalencephalocele,
Histo: interstitial+ pleuralfibrosiswith foci of dense corpuscallosalagenesis,KleeblattschAdel, cleft palate,
collagendeposition,NO granulomas ventriculomegaly(? relatedto skull base hypoplasia,
. bone manifestations obvious+ severe rarelyprogressive)
Location: apices/ upper lungfields . lQ variesdependingon CNS anomalies(in 50% normal)
r/ sternomanubrial joint irregularities
+ sclerosis . otitismedia(highprevalence)
./ uni- / bilateralcoarseupp-erlobe pulmonaryfibrosis . bifiduvula
with upwardretractionof hila (DDx: tuberculosis) . conductivehearingloss (commondue to external
! reticulonodular progressivelyconfluentopacitiesin + middleear malformations)
lung apices
{ apicalbullae+ cavitation(mimicking TB) @ Skull
HRCT: . downturnedmouth
r/ peripheralinterstitiallung disease r/ brachycephalic skull (due to coronal
r/ bronchiectasis craniosynostosis) + flat occiput
./ paraseptalemphysema r/ widenedmetopic+ sagittalsuturesextendingfrom
r/ apicalfibrosis glabellato posteriorfontanel(closingbetween2 to
Cx: superinfection, especiallywith aspergillosis 4 years)
(mycetomaformation)/ atypicalmycobacteria r/ hypoplastic/ retrudedmidface:
DDx: othercausesof pulmonaryapicalfibrosis r/ hypertelorism
(primaryinfectionby fungi/ mycobacteria; r/ shalloworbitswith proptosis
cancer) r/ underdeveloped paranasalsinuses
@ Cardiovascular ! underdeveloped maxillawith prognathism
1. Aortitis (5%) of ascendingaorta + aortic valve ^/ frignpointedarch of palate
insufficiency r/ prominentverticalcrest in middleof forehead
(increasedintracranialpressure)
Prognosis: 20/o progressto significantdisability; r/ V-shapedanteriorfossa due to elevationof lateral
occasionallydeathfrom cervicalspine marginsof lessersphenoid
fracture/ aortitis { sellamay be enlarged
DDx: (1) Reitersyndrome(unilateral asymmetricSljoint r/ stylohyoidligamentcalcification(38-88%)
involvement,paravertebralossifications) r/ cervicalspinefusion(in up to71"/"),commonlyof
(2) Psoriaticarthritis(unilateral
asymmetricSljoint Sth and 6th vertebrae
involvement,paravertebralossifications) r/ choanalstenosis
(3) Inflammatoryboweldisease
(4) Sternocostoclavicular hyperostosis(pustulosis @ Hand & feet
palmariset plantaris) { severesymmetricsyndactyly= fusionof distal
portionsof phalanges,metacarpals/ carpals(most
oftenof 2nd, 3rd + 4th digit)
ANTERIOR
TIBIALBOWING r/ absenceof middlephalanges
= WEISMANN-NETTER SYNDROME { missing/ supernumerarycarpal/ tarsalbones
= coflg€nitalpainlessnonprogressive bilateralanteriorleg r/ pseudarthroses
bowing
Age: beginningin earlychildhood @ GU (10%)
. may be accompaniedby mentalretardation,goiter, . cryptorchidism
anemia ./ hydronephrosis
{ anteriorbowingof tibia+ fibula,bilaterally,
symmetrically r/ polycystickidneys(rare)
at middiaphysis ri bicornuateuterus(rare)
 Radiology Review Manual

FISTULAOF BONE
ARTERIOVENOUS . respiratorydistressdue to reducedthoracicmobility
Etiology: (a) acquired(usuallygunshotwound) (abdominalbreathing)+ frequentpulmonaryinfections
(b) congenital . progressiverenalfailure+ hypertension
Location: lowerextremitymost frequent
r/ soft-tissuemass @ Chest
r/ presenceof largevessels { markedlynarrow+ elongatedbell-shapedchest:
r/ phleboliths(DDx:long-standingvaricosity) r/ chestdiametersignificantlydecreasedcompared
{ acceleratedbone growth with that of the abdomen
{ corticalosteolyticdefect (= pathwayfor large vessels r/ normalsize of heartleavinglittleroom for lungs
intomedulla) { horizontalclaviclesat levelof 6th cervicalvertebra
r/ increasedbone density { short horizontalribs + irregularbulbous
costochondral junction
ARTHROGRYPOSIS @ Pelvis
= ARTHROGRYPOSIS MULTIPLEX CONGENITA r/ tridentpelvis(retardationof ossificationof triradiate
= hol'lpfogressive congenitalsyndromecomplex cartilage)
characterizedby poorlydeveloped+ contracted { smalliliacwingsflared+ shortenedin cephalocaudal
muscles,deformedjointswith thickenedperiarticular diameter("wineglass" Pelvis)
capsuleand intactsensorysystem { shortischial+ pubicbones
Pathophysiology: r/ reducedacetabularangle + acetabularspurs
congenital/ acquireddefectof motor unit (anteriorhorn ri prematureossificationof capitalfemoralepiphysis
cells,nerveroots,peripheralnerves,motorend plates, @ Extremities
muscle)earlyin fetal lifewith immobilizationof jointsat ri rhizomelicbrachymelia(humerus,femur)= long
in
variousstages theirdevelopment bonesshorter+ widerthan normal
Cause: ? neurotropicagents,toxic chemicals,hard { metaphysealirregularitY
drugs,hyperthermia, neuromuscular blocking { postaxialhexadactyly(occasionally)
agents,mytoticabnormalities,mechanical { shorteningof distalphalanges+ cone-shaped
immobilization epiphysesin hands+ feet
lncidence: 0.03% of newborninfants;5% risk of ./ proximalhumeral+ femoralepiphysesossifiedat
recurrencein sibling birth (frequently)
Path: diminutionin size of musclefibers+ fat depositsin @ Kidneys
fibroustissue ri medullarycysticrenaldisease= enlargedkidneys
with linearstreakingon nephrogram (in adulthood)
Associated with:
(1) neurogenicdisorders(90%)
(2) myopathicdisorders OB .U S :
(3) skeletaldysplasias r/ proportionateshorteningof long bones
(4) intrauterinelimitationof movement(myomata, r/ smallthoraxwith decreasedcircumference
amnioticband,twin,oligohydramnios) { increasedcardiothoracicratio
(5) connectivetissuedisorders r/ occasionallypolydactyly
Distribution: all extremities(46%),lowerextremities only r/ polyhydramnios
(43%),upperextremities only (11%);
peripheral joints>> proximaljoints; Prognosis; neonataldeath in 80% (respiratoryfailure
symmetrical + infections)
. clubfoot DDx: Ellis-vanCreveldsyndrome
. congenitaldislocation of hip
. claw hand
. diminishedmusclemass AVASCULAR NECROSIS
. skin webs = AVN= OSTEONECROSIS = ASEPTIC NECROSIS
r/ flexion+ extensioncontractures = corseguenceof interruptedblood supplyto bone with
{ osteopepis+ pathologicfractures death of cellularelements
r/ congenitaldislocationof hip
{ carpalcoalition Histo:
r/ verticaltalus (a) cellularischemialeadingto deathof hematopoietic
{ calcanealvalgusdeformity cells (in 6-12 hours),osteocytes(in 12-48 hours)
and lipocytes(in 2-5 days)
(b) necroticdebrisin intertrabecular spaces
ASPHYXIATING THORACIC DYSPLASIA + proliferation by mesenchymal
and infiltration cells
= J E UNEDI S E A SE + capillaries
= autosomalrecessivedysplasia (c) mesenchymalcells differentiateto osteoblastson the
lncidence; 100 cases surfaceof dead trabeculaesynthesizingnew bone
Associatedwith: renalanomalies(hydroureter),
PDA layers+ resultingin trabecularthickening
 Bone and Soft-Ti Disorders 47

Pathogenesis: Location: femoralhead (mostcomm ), humeralhead,

(1) obstructionof extra-and intraosseousvesselsby femoralcondyles
arterialembolism,venousthrombosis, traumatic
disruption,
externalcompression(increasedmarrow
space pressure) AvascularNecrosisof Hip
(2) cumulativestressfrom cytotoxicfactors 0 Involvement of one hip increases to contralateral
Cause: hi pto 70/" 1
A. Traumaticinterruptionof arteries Age: 20-50 years
@ femoralhead: . hip / groin/ thigh/ kneepain
1. Femoralneck fracture(60-75%) . limitedrangeof motion
2. Dislocation of hipjoint (25%) Plainfilm (positiveonly several s aftersymptoms):
3. Slippedcapitalfemoralepiphysis(15-40%) r/ subtle relativesclerosisof head secondary
@ carpalscaphoid: to resorptionof surrounding v larized bone
4-6 monthsafterfracture(in 10-15o/o), in (earliestsign)
30-40% of nonunionof scaphoidfracture { radiolucentcrescentparallelto icularsurfacein
Site: proximalfragment(mostcommon) weight-bearingportionsecon to subchondral
@ humeralhead (infrequent) structuralcollapseof necrotic
B. Embolization of arteries Site: anterosuperior portionof head (best
1. Hemoglobinopathy: sickle-cell
disease seen on froglegview)
2. Nitrogenbubbles:Caissondisease r/ preservationof joint space (DDx arthritis)
C. Vasculitis r/ flatteningof articularsurface
1. Collagen-vascular disease:SLE ./ increaseddensityof femoralh (compressionof
2. Radiationexposure bonytrabeculaefollowingmi ure of
D. Abnormalaccumulation of cells nonviablebone,calcificationof riticmarrow.
1. Lipid-containinghistiocytes:Gaucherdisease creepingsubstitution = depositio of new bone)
2. Fat cells: steroidtherapy
E. ldiopathic CI assification ( Steinberg):
1. Spontaneousosteonecrosisof knee Stage0 = normal
2. Legg-Calv6-Perthes disease Stage | - normallbarely trabecular
3. Freibergdisease mottling;abnormal ne scani MRt
StagellA = focalsclerosis+ ia
mnemonic; "PLASTICRAGS" StagellB = distinctsclerosis+ eoporosts
Pancreatitis,Pregnancy + earlycrescentsi
Legg-Perthesdisease,Lupus erythematosus S tagel l l A = subchondralu ning ("cresgenl
Alcoholism,Atherosclerosis sign")+ cyst n
Steroids StagelllB = mildalterationin head
Trauma(femoralneck fracture,hip dislocation) contourI
ldiopathic(Legg-Perthesdisease),Infection + normal j oi nt
Caissondisease,Collagendisease(SLE) Stage lV = markedcollapseof moral head
Rheumatoidarthritis,Radiationtreatment + significant
Amyloid StageV = + acetabular
Gaucherdisease degenerative
Sicklecell disease
NUC (80-85% sensitivity) :
mnemonic: "GlVE INFARCTS" 0 Bonemarrowimaging(with loid)more
Gaucherdisease sensitivethan bone imaging(wi diphosphonates)
ldiopathic(Legg-Calv6-Perthes, Kohler,Chandler) 0 Moresensitivethan plainfilmsin y AVN
Vasculitis(SLE,polyarteritisnodosa,rheumatoid (evidenceof ischemiaseen as as 1 year
arthritis) earlier)
Environmental (frostbite,
thermalinjury) 0 Lesssensitivethan MR
lrradiation Technique; imagingimprovedwi doublecounts,
Neoplasia(-associatedcoagulopathy) pi nhol ecol l i mati on
Fat (prolongedcorticosteroiduse increasesmarrow) { early: cold = photopenicdefect(i blood
Alcoholism supply)
Renalfailure+ dialysis ^/ late: "doughnutsign" = cold spot by
Caissondisease increaseduptakesecondaryto
Trauma(femoralneck fracture,hip dislocation) (a) capillaryrevascularization + synthesis
Sicklecell disease (b) degenerativeosteoarthritis
CT (utilizedfor stagingof knowndi
NO predisposing
factorsin 25/"1 r/ stagingupgradesin 30% with plainfilms
 Radiology Review Manual

MR (90-100% sensitive,85% specificfor symptomatic DDx: bone marrowedema (ill-delimited marrow

disease): changes,no reactiveinterface);epiphyseal
Prevalenceof clinicallyoccultdisease: 6% fracture(speckled/ linearhypointenseareas,
0 MR imagingchangesreflectthe death of marrowfat focal depressionof epiphysealcontour);
cells (not death of osteocyteswith empty lacunae)! spondyloarthroPathY
0 Sagittalimagesparticularlyuseful!
BlountDisease
CI assification (MitcheII) : = TIBIAVARA
T1 T2 Analogous to = oVsscularnecrosisof medialtibialcondyle
Age: >6 years
A high intermediate fat . limping,lateralbowingof leg
B high high subacuteblood
{ medialtibialcondyleenlarged+ deformed
c low high fluid/ edema
(DDx:Turnersyndrome)
D low low fibrosis
{ irregularityof metaphysis(medially+ posteriorly
prolongedwith beak)
EARLYAVN:
{ decreasedGd-enhancement on short-inversion- Disease
Galv6-Kiimmel-Verneuil
recovery(STIR)images(veryearly) = VERTEBRAL OSTEOCHONDROSIS = VERTEBRA PLANA
r/ low-signalintensityband with sharp inner = aV€lscular necrosisof vertebralbody
interface+ blurredoutermarginon TlWl within Age: 2-15 years
1248 hours (= Iil€seflchymal+ fibrousrepair { uniformcollapseof vertebralbody into flat thin disk
tissue,amorphouscellulardebris,thickened ./ increaseddensityof vertebra
trabecularbone)seen as r/ neuralarchesNOT affected
(a) band extendingto subchondralbone plate ^/ dist<sare normalwith normalintervertebral disk space
(b) completering (lessfrequent) vacuumcleftsign (PATHOGNOMONIC)
r/ intravertebral
r/ "double-line sign"on T2Wl (in 80%) [MORE DDx: eosinophilicgranuloma,metastaticdisease
SPECIFICI= juxtaposition of innerhyperintense
band (granulationtissue)+ outer hypointense
band (chemicalshift artifact/ fibrosis+ sclerosis) FreibergDisease
ADVANCEDAVN: (1868-1940),orthopedicsurgeonin
[AlbertHenryFreiberg
{ "pseudohomogeneous edema pattern" Ohiol
Cincinnati,
= inhomogeneouslargeareasof mostly = osteochondrosis of head of 2nd (Srd l4th) metatarsal
decreasedsignalintensityon TlWl A ge: 10-18 years; M:F = 1:3
{ nypo-to hyperintenselesionon T2WI . metatarsalgia,swelling,tenderness
r/ contrast-enhancement of interface+ surrounding
marrow+ withinlesion Earlv:
SUBCHONDRALFRACTURE: ^/ flattening,increaseddensity,cysticlesionsof
r/ predilectionfor anterosuperiorportionof femoral metatarsalhead
head (sagittalimages!) ri wideningof metatarsophalangeal joint
i/ cleft of low-signalintensityrunningparallelto the Late:
subchondralbone platewithinareasof fatlike { osteochondralfragment
signalintensityon Tl Wl r/ sclerosis+ flatteningof metatarsalhead
^/ hyperintenseband (= fracturecleftfilledwith r/ increasedcorticalthickening
articularfluid/ edema)withinthe intermediate-or
necroticmarrowon T2Wl
low-signal-intensity
r/ lack of enhancementwithin+ aroundfracture KienbdckDisease
cleft = LUNATOMALACIA
EPIPHYSEALCOLLAPSE: Kienbdck
[Robert (1871-1953), in Vienna,
radiologist Austria]
r/ focal depressionof subchondralbone = €lV€rscular
necrosisof lunatebone
Predisposed: individualsengagedin manuallabor
Cx: throughcollapseof femoral
earlyosteoarthritis with repeated/ singleepisodeof
head + joint incongruityin 3-5 years if left trauma
untreated Age: 20-40 years
Associatedwith: ulna minusvariant(shortulna)in 75%
Rx: (1) coredecompression (forgrade0-ll): most . progressivepain + soft-tissueswellingof wrist
successfulwith <25o/"involvementof femoral
head Location: uni-> bilateral(usuallyrighthand)
(2) osteotomy(for grade 0-ll) r/ initiatlynormalradiograph
(3) arthroplasty/ arthrodesisi total hip r/ osteonecroticfractureof carpallunate
replacement (forgrade>lll) ./ increaseddensity+ alteredshape + collapseof lunate
I

Bone and Soft-Ti, Disorders 49

Cx: scapholunateseparation,ulnardeviationof r/ slightwideningof jointspace to thickeningof

triquetrum,degenerative jointdiseasein cartilage,failureof epiphyseal , presence
radiocarpal/ midcarpalcompartments of jointfluid,jointlaxity(60%)
Rx: ulnarlengthening / radialshortening,lunate { ipsilateral bonedemineralizatin (6%)
replacement { afierationof pericapsularsoft- outlinedue
to atrophyof ipsilateralperia softtissues
(73%)
K6hlerDisease { rarefactionof lateral+ medial lareas
= €lV?scUlar necrosisof tarsalscaohoid of neck
Age: 3-10years; boys { nfVf n destruction
of articularlcortex
as in
r/ irregularoutline bacterialarthritis
r/ fragmentation Latesigns:
./ disklikecompressionin AP direction r/ delayedosseousmaturation a milddegree
r/ increaseddensity rl "radiolucentcrescentline"of bchondralfracture
r/ loint space maintained = sm?llarchlikesubcortical (32%)
^/ decreased/ increaseduptakeon radionuclidestudy r/ subcorticalfractureon anterior rticularsurface
(bestseen on froglegview)
Legg-Calv6-Perthes
Disease { femoralhead fragmentation
= COXAPLANA femoralneck cysts (from i lary
= idiopathicavascularnecrosisof femoralhead in hemorrhagein responseto strrss fractures)
children;one of the mostcommonsitesof AVN: r/ loosebodies(onlyfoundin ma )
in 5-10% bilateral Reqenerativesions:
Age: (a) 2-12 (peak,4-8) years: M:F = 5:1 r/ coxa plana = flattenedcollecti of sclerotic
(b) adulthood:Chandler disease fragments(over 18 months)
Cause: traumain 30% (subcapitalfracture, ./ coxa magna= rerT'rod€ling of fe head to
epiphyseolysis, esp. posteriordislocation), becomewider + flatterin configuration
closedreductionof congenitalhip dislocation, to matchwidenedmetaphysis epiphysealplate
prolongedintervalbetweeninjuryand reduction CT:
Pathophysiology: r/ lossof "asterisk"
sign(= starlike rn of crossing
insufficientfemoralhead bloodsupply(epiphyseal trabeculaein centerof femoral withdistortion
plate acts as a barrierin ages 4-10; ligamentumteres of asteriskand extensionto su of femoralhead
vesselsbecomenonfunctional; bloodsupplyis from MR:
medialcircumflexartery+ lateralepiphysealartery { normalsignalintensityin marrow femoral
only) epiphysisreplacedby low signali sityon TlWl
Stages: + highsignalintensityon T2Wl = isk"sign
| - histologic+ clinicaldiagnosiswithout r/ "double-line" sign (80%)= nonsignalrim
radiographic findings producingline betweennecrotic+ viablebone
ll = sclerosis+ cysticchangeswith preservationof edged by a hyperintenserim of g ulation tissue
contour+ surfaceof femoralhead fluidwithinfractureplane
lll = loss of structuralintegrityof femoralhead hipjoint incongruity:lateralfemor head
lV = in additionloss of structuralintegrityof uncovering, labralinversion, head deformity
acetabulum Cx: severedegenerativejoint in early
adulthood
. 1 week-6 months(mean2.7 months)durationof Rx: bed rest,abductionbracing(to ce stresson
symptomspriorto initialpresentation:limp,pain infarctedhead)
NUC (mayassistin earlydiagnosis):
rl decreaseduptake(early)in femoralhead PannerDisease
= interruption
of bloodsupply = osteonecrosis
of capitellum
{ increaseduptake(late)in femoralhead =
(a) revascularization
+ bone repair PreiserDisease
(b) degenerativeosteoarthritis = nontraumatic
osteonecrosis
of
r/ increasedacetabularactivitywith associated
degenerativejoint disease SpontaneousOsteonecrosisof Kn
= SONK
X-RAY: Cause: ? meniscaltear (781"), withresultant
Earl)Lsigns: microfractures, vascularin flcY'
./ femoralepiphysissmallerthan on contralateral degenerativejoint disease,
, side (96%) chondromalacia, gout, arthritis,
r/ sclerosisof femoralhead epiphysis joint bodies,intraarticular injection
(sequestration+ compression)(82%) (45-85%)
50 Radiology Review Manual

Age: 7th decade(range13-83 years) CHILDSYNDROME

BATTERED
. acute onset of pain = CAFFEY-KEMPE SYNDROME = CHILDABUSE= PARENT -
INFANT TRAUMATIC STRESS SYNDROME = NON-
Location: weight-bearingmedialcondylemore toward ACCIDENTAL TRAUMA
epicondylus(95%),lateralcondyle(5%), 0 Mostcommoncauseof seriousintracranial injuriesin
may involvetibialplateau children<1 year of age;3rd mostcommoncauseof
r/ radiographs usuallynormal(within3 monthsafter deathin childrenaftersuddeninfantdeathsyndrome
onset) + true accidents
i/ positivebone scan within5 weeks (mostsensitive)
./ flatteningof weight-bearingsegmentof medial Prevalence: 1.7 millioncases reported+ 833,000
femoralepicondyle substantiated in UnitedStatesin 1990(45%
r/ radiolucent focusin subchondralbone + peripheral neglectedchildren,25o/"physicallyabused,
zone of osteosclerosis 16% sexuallyabusedchildren);resultingin
r/ horizontalsubchondralfracture(within6-9 months) 2,500-5,000deathslyear;5-10% of children
+ osteochondralfragment seen in emergencyrooms
./ periostealreactionalong medialside of femoralshaft Age: usually<2 years
(30-50%)
Cx: osteoarthritis . skin burns,bruising,lacerations,
hematomas
(SNAT= sUSpectednonaccidental trauma)

BASALCELLNEVUSSYNDROME @ Skeletaltrauma(50-80%)
= NEVOID BASALCELLCARCINOMA SYNDROME = GORLIN Site: multipleribs,costochondral/ costovertebral
SYNDROME separation,acromion,skull,anterior-superior
= syndromeof autosomaldominantinheritance wedgingof vertebra,tibia,metacarpus
characterizedby Unusualsites: transversefractureof sternum,Iateral
(1) multiplecutaneousbasalcellcarcinomasduring end of clavicles,scapula,vertebral
childhood compression,vertebralf racture
(2) odontogenickeratocystsof mandible dislocation, disk spacenarrowing,
(3) ectopiccalcifications spinousprocesses
(4) skeletalanomalies Otherclues: bilateralacutefractures,fracturesof
. multiplenevoidbasalcell carcinomas(nose,mouth, lowerextremities in childrennot yet
chest,back)at mean age of 19 years;after puberty walking
aggressive,may metastasize { muftipleasymmetricfracturesin differentstagesof
' pitlikedefectsin palms + soles healing(repeatedinjury= HALLMARK)
. mentalretardation ^/ exuberantcallusformationat fracturesites
r/ avulsionfractureof ligamentous insertion;frequently
Association: high incidenceof medulloblastomain seen withoutperiostealreaction
children;ovarianfibroma(in 17%);cardiac @ Epiphysis
f ib ro ma(i n 1 4 % ) r/ separationof distalepiphysis
@ Metaphysis
r/ mandibularhypoplasia: r/ markedirregularity+ fragmentationof
r/ multiplemandibular+ maxillarycysts(dentigerous metaphyses(DDx: osteochondritis stage of
cysts + ectopicdentition) congenitalsyphilis;infractionsof scurvy)
r/ anomaliesof upper5 ribs: { "corner"fracture(11o/") = "bucket-handle" fracture
r/ forked= bifid rib (mostcommonly4th rib) = avulsionof an arcuatemetaphysealfragment
^/ agenesis/ supernumerary ribs overlyingthe lucentepiphysealcartilage
r/ fusionof adjacentribs Cause: suddentwistingmotionof extremity
r/ dysplasticdistortedribs (periosteumeasilypulledaway from
./ OitiOspinousprocesses,spinabifida diaphysisbut tightlyattachedto
r/ scoliosis(cervical+ upperthoracic) metaphysis)
r/ hemivertebrae+ blockvertebrae Location: knee,elbow,distaltibia,fibula,
{ Sprengeldeformity(scapulaelevated,hypoplastic, radi us,ul na
bowed) @ Diaphysis
r/ deficiencyof lateralclavicle r/ isotatedspiralfracture(15%)of diaphvsis
r/ brachydactyly secondaryto externalrotatoryforce appliedto
r/ extensivecalcificationof falx + tentorium femur/ humerus
r/ ectopiccalcifications of subcutaneoustissue,ovaries, r/ extensiveperiostealreactionfrom large
sacrotuberousligaments,mesentery subperiostealhematomaapparentafter
d bony bridgingof sella turcica 7-14 daysfollowinginjury(DDx: scurvy,copper
r/ macrocephaly deficiency)
 Boneand Soft-Tissue
Disorders 51

! corticalhyperostosisextendingto epiphyseal Cx: pathologic/ avulsionfracturefollowingminor

plate (DDx: not in infantilecorticalhyperostosis) trauma(infrequent)
@ Headtrauma(13-25%) Prognosis; (1) Spontaneoushealingresultingin
Most commoncause of death + physicaldisability sclerosis/ disappearance
(1) lmpactinjurywith translational force: skullfracture (2) Ballooningof endostealsurfaceof
(flexiblecalvaria+ meningesdecreaselikelihoodof cortex= fibrouscorticaldefect
skullfractures),subduralhematoma,brain (3) Medullaryextensionresultingin
contusion,cerebralhemorrhage, infarction, nonossifying fibroma
generalized edema
(2) Whiplashinjurywith rotationalforce: shearing BONEINFABCT
injuries+ associatedsubarachnoid hemorrhage Etiology:
. bulgingfontanels,convulsions
A. Occlusionof vessel:
. ocularlesions,retinaldetachment
(a) thrombus: thromboembolicdisease,sicklecell
Skullfilm (associated fracturein 1%): anemia(SS + SC hemoglobin), polycythemia
r/ linearfracture> comminutedfracture> diastases rubravera
(conspicuously absent) (b) fat: pancreatitis(intramedullary fat necrosisfrom
CT: circulatinglipase),alcoholism
subduralhemorrhage(mostcommon): (c) gas: Caissondisease,astronauts
interhemisphericlocationmostcommon B. Vesselwall disease:
{ subarachnoid hemorrhage 1. Arteritis:SLE, rheumatoidarthritis,polyarteritis
^/ epiduralhemorrhage(uncommon) nodosa,sarcoidosis
^/ cerebraledema (focal,multifocal,diffuse) 2. Arteriosclerosis
{ acutecerebralcontusionas ovoidcollectionof C. Vascularcompression by depositionof:
intraparenchymal bloodwith surroundingedema (a) fat: corticosteroidtherapy(eg, renaltransplant,
M R : more sensitivein identifyinghematomasof Cushingdisease)
differingages (b) blood: trauma(fractures+ dislocations)
! white mattershearinginjuriesas areas of (c) inflammatory cells: osteomyelitis, infection,
prolongedT1 +T2 at corticomedullary junction, histiocytosisX
centrumsemiovale,corpuscallosum (d) edema: radiationtherapy,hypothyroidism,
@ Viscera(3%) frostbite
0 Secondleadingcause of death in child abuse (e) substances:Gaucherdisease (vascular
Cause: crushingblow to abdomen(punch,kick) compression by lipid-filled
histiocytes),gout
Age: often >2 years D. Others: idiopathic,hypopituitarism,
r/ smallbowel/ gastricrupture pheochromocytoma (microscopic thrombotic
r/ hematomaof duodenum/ jejunum disease),osteochondroses
{ contusion/ lacerationof lung,pancreas,liver,
spleen,kidneys Medullarylnfarction
r/ traumaticpancreaticpseudocyst 0 Nutrientarteryis the sole bloodsupplyfor diaphysis!
Cx: (1) Brainatrophy(up to 100%) Location: distalfemur,proximaltibia,iliacwings,ribs,
(2) Infarction(50%) humeri
(3) Subduralhygroma (a) Acute phase:
(4) Encephalomalacia r/ tttOradiographicchangeswithoutcortical
(5) Porencephaly involvement
DDx: normalperiostitisof infancy,long-termventilator ^/ area of rarefaction
therapyin prematurity,osteogenesisimperfecta, ./ bone marrowscan: diminisheduptakein
congenitalinsensitivity
to pain,infantilecortical medullaryRESfor longperiodof time
hyperostosis,Menkeskinky hair syndrome, r/ bonescan: photon-deficient lesionwithin
Schmid-typechondrometaphyseal dysplasia, 24-48 hours;increaseduptakeafter collateral
scurvy,congenitalsyphiliticmetaphysitis circulationestablished
(b) Healingphase(completehealing/ fibrosis/
calcification):
BENIGNCORTICAL
DEFECT ./ demarcation by zone of serpiginous / linear
= developmentalintracorticalbone defect calcification+ ossificationparallelto cortex
Age: usually1st-2nd decade;uncommonin boys <2 ./ dense bone indicatingrevascularization
years of age; uncommonin girls <4 years of age
' asymptomatic
CofiicalInfarction
Site: metaphysisof long bone 0 Requirescompromiseof
r/ well-definedintracorticalround/ oval lucency (a) nutrientarteryand (b) periostealvessels!
r/ usually<2 cm long Age: particularly in childhoodwhereperiosteumis
r/ scleroticmargins easilyelevatedby edema
 RadiologyReviewManual
{ avascularnecrosis= osteonecrosis
r/ osteochondrosis dissecans
Cx: (1) Growthdisturbances
r/ cupped/ triangular/ coned epiphyses
r/ "H-shaped"vertebralbodies
(2) Fibrosarcoma(mostcommon),malignant
fibroushistiocytoma,benigncysts
(3) Osteoarthritis
BONEISLAND
= ENOSTOSIS = ENDOSTEOMA = COMPACT ISLAND
= FOCALSCLEROSIS = SCLEROTIC BONEISLAND
= CALCIFIED MEDULLARY DEFECT
= focal lesionof denselysclerotic(compact)bone nesting
withinspongiosa
Age: any age (mostly20-80 years of age); grows more
rapidlyin children
Histo: nest of lamellarcompactedbone with haversian
systemembeddedwithinmedullarycanal
Pathogenesis:
? misplacedcorticalhamartoma,? developmentalerror
of endochondralossificationas a coalescenceof mature
bpnetrabeculaewith failureto undergoremodeling; not
inherited
. asymptomatic
Location: ilium+ proximalfemur(8V92%), ribs,spine
(1-14%),humerus,phalanges(notin skull)
./ roundI oval/ oblongsolitaryosteoblasticlesionwith
abrupttransitionto surroundingnormaltrabecularbone
{ long axis of bone islandparallelslong axis of bone
{ usually2-10 mm in size;lesion>2 cm in longestaxis
= giant bone island
{ "brushborder"= "thorflyradiations"= sharplydemarcated
marginswith featheryperipheralradiations(HALLMARK)
blendingwithtrabeculaeof surrounding spongiosa
ri may show activityon bone scan, esp. if large (33%)
r/ may demonstrateslow growth/ decreasein size (32%)
rl ruOinvolvementof cortex/ radiolucencies / periosteal
reaction
Prognosis.' may increaseto 8-12 cm over years (40%);
may decrease/ disappear
DDx: (1) Osteoblasticmetastasis(aggressive,break
throughcortex,periostealreaction)
(2) Low-gradeosteosarcoma(corticalthickening,
extensionbeyondmedullarycavity)
(3) Osteoidosteoma(painrelievedby aspirin,nidus)
(4) Benignosteoblastoma
(5) lnvolutednonossifyingfibromareplacedby
dense bone scar
(6) Eccentricfocus of monostoticfibrousdysplasia
(7) Osteoma(surfacelesion)
BRUCELLOSIS
= multisystemiczoonosisof worldwidedistribution;
endemicin SaudiArabia,ArabianPeninsula,South
America,Spain,ltaly (secondaryto ingestionof raw milk
i milk products)
Organism; small Gram-negativenonmotile,nonsporing,
aflagellate,nonencapsulated coccobacilli:
Brucellaabortus,B. suis,B. canis,B. melitensis
Histo: small intracellularpathogensshed in excretaof
infectedanimals(urine,stool,milk,productsof
conception) causesmallnoncaseating granuloma
within RES
Location: commonestsite of involvementis reticulo-
endothelialsystem;musculoskeletal system
. 1-3 weeks betweeninitialinfection+ symptoms
0 Radiologic evidenceof diseasein 69% of
symptomaticsites!
@ Brucellarspondylitis(53%)
Age: 40 years is averageage at onset
. pain,localizedtenderness,radiculopathy, myelopathy
Location: lumbar(71"/")> thoracolumbar(10%)>
umbosacraf(8%) > cervical(7%) ,
thoracic(4%)
(a) focal form
^/ bone destructionat diskovertebral junction
(anterioraspectof superiorendplate)
r/ associatedwith bone sclerosis+ anterior
osteophyteformation+ smallamountof gas
(b) diffuseform: entirevertebralendplateI whole
vertebralbody affectedwith spread to adjacent
disks + vertebralbodies
r/ bone destructionassociatedwith sclerosis
{ smallamountof diskgas (25-30%)
r/ obliterationof paraspinalmuscle-fatplanes
{ no / minimalepiduralextension
DDx: TB (paraspinalabscess,gibbus)
@ Extraspinal disease
(a) Brucellarsynovitis(81%)
Location: knee> sacroiliacjoint> shouldert hip
> sternoclavicular joint > ankle > elbow
Site: organismlocalizedin synovialmembrane
. serosanguinous sterilejointeffusion
(b) Brucellardestructivearthritis(9%)
^/ indistinguishable from tuberculousI pyogenic
arthritis
(c) Brucellarosteomyelitis(2%)
. pain,tenderness,swelling
(d) Brucellarmyositis(2%)
Dx: serologictests (enzyme-linked immunosorbent
assay,counterimmunoelectrophoresis, rose bengal
' plate test
Rx: combinationof aminoglycosides + tetracyclines
DDx: fibrousdysplasia,benigntumor,osteoidosteoma
CAISSON DISEASE
= DECOMPRESSION SICKNESS = THEBENDS
Etiology: duringtoo rapiddecompression= reductionof
surroundingpressure(ascentfrom dive, exit
from caisson/ hyperbaricchamber,ascentto
altitude)nitrogenbubblesform (nitrogenmore
solublein fat of panniculusadiposus,spinal
cord, brain,bonescontainingfatty marrow)
. "thebends"= localpainin knee,elbow,shoulder,hip
. neurologicsymptoms(paresthesia,majorcerebral/
spinalinvolvement)
. "chokes"= substernaldiscomfort+ coughing
(embolization of pulmonaryvessels)
 Boneand Soft-TissueDisorders 53

mostlyin longtubularbonesof lowerextremity @ Knee

(distalendof shaft+ epiphyseal
portion); r/ medialfemorotibial+ patellofemoral compartments
symmetricallesions commonlyinvolvedsimultaneously (as in
{ early: area of rarefaction but with greaterosseousdestruction
osteoarthritis)
healingphase: irregularnew-boneformationwith + fragmentation
greaterdensity { disproportionate narrowingof patellofemoral joint
{ peripheralzone of calcification/ ossification @ Spine
{ ischemicnecrosisof articularsurfacewith secondary { chondrocalcinosis / calcificationsof outerfibersot
osteoarthritis annulusfibrosusresemblingsyndesmophytes
r/ verticalradiodenseline in symphysispubis
CALCIUMPYROPHOSPHATEDIHYDRATE
CRYSTALDEPOSITION
DISEASE CAMPOMELIC DYSPLASIA
= CPPD = PSEUDOGOUT= FAMILIALCHONDROCALCINOSIS = sPorodic/ autosomalrecessivedwarfism
0 Most commoncrystallinearthropathy lncidence; 0.05:10,000births
Types: 1. Osteoarthritic form (35-60%) Associated with:
2. Pseudogout= acute synovitis(10-20%) 1. Hydrocephalus (23%)
3. Rheumatoidform (2-6%) 2. Congenitalheartdisease(30%):VSD,ASD,
4. Pseudoneuropathic arthropathy(2%) tetralogy,AS
5. Asymptomaticwith tophaceouspseudogout 3. Hydronephrosis (30%)
(common) . pretibialdimple
Associatedwith: hyperparathyroidism, hypothyroidism, r/ macrocephaly,cleft palate,micrognathia(90-99%)
hemochromatosis, hypomagnesemia @ Chest & spine
Prevalence; widespreadin older population;M:F = S:2 r/ hypoplasticscapulae(92%)
. calciumpyrophosphatecrystalsin synovialfluid + within { narrowbell-shapedchest
leukocytes(characteristic weaklypositivebirefringent { hypoplasticvertebralbodies+ nonmineralized
diffractionpattern) pedicles(especiallylowercervicalspine)
. acute/ subacute/ chronicjoint inflammation @ Pelvis
Location: (a) knee (especiallymeniscus+ cartilageof r/ verticallynarrowediliac bones
patellofemoral joint) I verticalinclination of ischii
(b) wrist (triangularfibrocartilagein distal { wide symphysis
radioulnar joint bilaterally) ./ narrowiliacboneswith smallwings
(c) pelvis(sacroiliacjoint,symphysis) { shallowacetabulum
(d) spine (annulusfibrosisof lumbar @ Extremities(lowerextremitymore severelyaffected)
intervertebraldisk;NEVERin nucleus r/ dislocationof hips + knees
pulposusas in ochronosis) { anteriorbowing(= camPo)of long bones: markedin
(e) shoulder(glenoid),hip (labrum),elbow, tibia + moderatein femur
ankle,acromioclavicular joint r/ hypoplasticfibula
r/ polyarticularchondrocalcinosis (in fibro-and hyaline { smallsecondaryossificationcenterof knee
cartilage) r1 small primaryossificationcenterof talus
rl largesubchondralcyst (HALLMARK) r/ clubfoot
r/ numerousintraarticular bodies (fragmentation of OB -U S :
subchondral bone) r/ bowingof tibia + femur
./ involvementof tendons,bursae,pinnaeof the ear ./ decreasedthoraciccircumference
N.B.: pyrophosphatearthropathyresembles i/ hypoplasticscapulae
osteoarthritis:joint space narrowing+ extensive r/ + cleft palate
subchondralsclerosis Prognosis; death usually<5 monthsof age (withinfirst
year in 97%) due to respiratoryinsufficiency
@ Hand
Site: radiocarpalcompartment;trapezioscaphoid joint CARPALTUNNELSYNDROME
+ 1 CMC; 2,3 MCP joints;bilateralsymmetric = entrapment
syndrome
caused pressure
bychronic on
{ resemblingdegenerative jointdisease(withoutDIP the mediannervewithinthe carpaltunnel
and PIP involvement) Cause: repetitivewrist/ fingerflexion;carpaltunnel
r/ extensivenarrowingl obliterationof joint space crowdingby cyst / mass / flexortendontendinitis
betweendistalradius+ scaphoid: or tenosynovitis/ anomalousoriginof lumbrical
./ incorporationof scaphoidinto articularsurfaceof muscles
radius Pathogenesis: probablyischemiawith venous
r/ prominentcysts congestion(stage1), nerveedemafrom
{ calcificationof triangularfibrocartilage anoxicdamageto capillaryendothelium
{ scapholunateseparation (stage2), impairmentof venous+ arterial
! destructionof trapezioscaphoid space bloodsupply(stage3)
54 Radiology Review Manual

. nocturnalhand discomfort 0 213in lowerextremity,50% about knee

. weakness,clumsiness,fingerparesthesias 0 may occur in apophyses(minor+ greater
MR: trochanter,patella,greatertuberosityof humerus)
^/ "pseudoneuroma" of mediannerve = swellingof (b) flat bones:neartriradiatecartilageof innominate
mediannerveproximalto carpaltunnel bone,rib (3%)
^/ swellingof nervewithincarpaltunnel (c) shorttubularbonesof hand + feet
increasedsignalintensityof nerveon T2Wl Site: eccentricmedullary,subarticularlocationwith open
,Vvolar bowingof flexorretinaculum growthplate(98%beginwithinepiphysis);tumor
^/ swellingof tendonsheath(due to tenosynovitis) growthmay continueto involvemetaphysis(50%)
mass(es)withincarpaltunnel + rarelydiaphysis
^/ markedenhancement(nerveedema = breakdownof { oval / roundeccentricallyplacedlytic lesionof epiphysis
blood-nervebarrier) ri t-+ cm in diameteroccupying< one-halfof epiphysis
no enhancement(ischemia)provokedby wrist held in r/ well-definedscleroticmargin,lobulatedin 50%
an extended/ flexedposition { stippled/ irregularcalcificationsin 25-30-50%
(cartilaginous clumpsbettervisualizedby CT)
CARPENTER
SYNDROME r/ intactscallopedcorticalborder
= ACROCEPHALOPOLYSYNDACTYLY type2 r/ tnicf periostealreactionin metaphysis(50%)/ joint
autosomalrecessive involvement
. retardation { periostitisof adjacentmetaphysis/ diaphysis(30-50%)
. hypogonadism { open growthplate in majorityof patients
r/ patentductusarteriosus MR:
{ acro(oxy)cephaly 0 MR tends to overestimateextent+ aggressiveness
r/ preaxialpolysyndactyly
+ soft-tissuesyndactyly due to large area of reactiveedema!
^/ intermediateto low signalintensityon T2Wl relativeto
fat
CEREBROCOSTOMANDIBULAR
SYNDROME { extensiveintramedullary signalabnormalities
= fofe bonedisorderof uncertaintransmission consistentwith bone marrowedema
. respiratorydistress(due to flailchest + airway r/ peripheralrim of very low signalintensity
abnormalities) { hypointense changeson TlWl + hyperintense on
r/ 1t pairsof ribs: TzWl in adjacentsoft tissues(muscleedema)in 50%
r/ abnormalcostovertebralarticulations r/ + jointeffusion
r/ posteriorossificationgaps resemblingfractures
r/ microcephaly Prognosis: almostalwaysbenign;may becomelocally
r/ micrognathia aggressive;rarelymetastasizes
r/ congenitalheartdisease Dx: surgicalbiopsy
DDx: multiplefractures Rx: curettage+ bone chip grafting(recurrencein 25%)

DDx: (1) lschemicnecrosisof femoralhead (maybe

indistinguishable,moreirregularconfiguration)
CHONDROBLASTOMA
= CODMAN TUMOR= BENIGN (2) Giantcelltumor(usuallylarger+ lesswell
CHONDROBLASTOMA
= CARTILAGE-CONTAINING GIANTCELLTUMOR demarcated,not calcified,older age group with
lncidence: 1"/"of primarybone neoplasms(700 cases closedgrowthplate)
in world literature) (3) Chondromyxoid fibroma
Age: peak in 2nd decade (rangeof 8-59 years); (4) Enchondroma
(5) Osteomyelitis(lesswell-defined, variable
10-26 years (90%l; M:F = 2:1; occursbefore
cessationof enchondralbone growth margins)
(6) Aneurysmalbone cyst
Path: derivedfrom primitivecartilagecells
giant (7) lntraosseous ganglion
Histo: polyhedralchondroblasts+ multinucleated
(8) Langerhans (lesswell-defined,
cell histiocytosis
cells+ nodulesof pinkamorphousmaterial
(= chondroid)= gplphysealchondromatous giant variablemargins)
cell tumor (resembfeschondromyxoidfibroma); (9) Primarybonesarcoma
"chickenwire" calcification= pericellulardeposition
of calcificationis virtuallyPATHOGNOMONIC CHONDRODYSPLASIA PUNCTATA
. symptomaticfor monthsto years priorto treatment = CONGENITAL STIPPLED EPIPHYSES= DYSPLASIA
. mildjointpain,tenderness,swelling(jointeffusion) EPIPHYSEALIS PUNCTATA = CHONDRODYSTROPHIA
. limitationof motion CALCIFICANS CONGENITA
Location: = proportionali mesomelicdwarfism
(a) long bones (80%): proximalfemur + greater Etiology: peroxisomaldisordercharacterizedby
trochanter(23%),distalfemur (20%),proximaltibia fibroblastplasmalogendeficiency
(17"/"),proximalhumerus(17%) lncidence: 1:110,000births
 Boneand Soft-Tissue
Disorders 55

A. AUTOSOMALRECESSIVECHONDRODYSPLASIA @ Skull
PUNCTATA= RHIZOMELIC TYPE ^/ wormianbones
Associatedwith: CHD (common) r/ cleft lip
. flat face @ Chest
. congenitalcataracts r/ elongatednarrowthorax in AP + transverse
. ichthyoticskin thickening dimensionsexaggeratingthe heartsize
. mentalretardation ri cardiomegaly (frequently ASD / singleatrium)
. cleft palate r/ short horizontalribs + anteriorosseousexpansion
rl multiplesmallpunctatecalcifications of varyingsize r/ elevatedclavicles
in epiphyses(knee,hip,shoulder,wrist),in base of @ Pelvis
skull, in posteriorelementsof vertebrae,in r/ smallflattenedilium
respiratorycartilageand soft tissues(neck,rib ends) r/ tridentshape of acetabulumwith indentationin roof
beforeappearanceof ossificationcenters + bony spur (almostpathognomonic)
r/ prominentsymmetrical shorteningof femur r/ acetabular+ tibialexostoses
+ humerus(rarelyall limbssymmetrically affected) @ Extremities
.i congenitaldislocationof hip r/ varietyof micromelia (= 15;6loening
+ shorteningof all
r/ flexioncontracturesof extremities l ongbones):
r/ clubfeet ./ acromelia= hypoplasia/ absenceof terminal
r/ metaphyseal splayingof proximaltubularbones(in phalanges
particularaboutknee) r/ mesomelia= shorteningof forearms+ lowerlegs
r/ thickeningof diaphyses (radius+ tibia> humerus+ femur)
r/ prominentvertebral+ paravertebralcalcifications r/ cone-shapedepiphyses
^/ coronalcleftsin vertebralbodies r/ prematureossificationof proximalhumeral+ femoral
Prognosis: death usually<1 year of age epiphyses
DDx: Zellwegersyndrome @ Upperextremity
B . CO NRA DI - H U N E R MA NDNIS EA SE r/ "drumstick"forearm= Swellingof proximalend of
= NO NRHI ZOME L IC T Y PE ulna+ distalend of radius
more commonmildernonlethalvariety; r/ anteriordislocationof radialhead (due to
autosomaldominant shorteningof ulna)
. normalintelligence ri carpal/ tarsalfusion= frequentfusionof two 1
r/ more widespreadbut milderinvolvementas above more carpal(hamate+ capitate)+ tarsalbones
Prognosis; survivaloften into adulthood (aftercompleteossification)
Cx: respiratoryfailure(severeunderdevelopment of ^l supernumerary carpalbones
ribs),trachealstenosis,spinalcord compression r/ postaxialpolydactyly common(usuallyfinger,
DDx: (1) Cretinism(mayshow epiphysealfragmentation, rarelytoe) t syndactylyof hands+ feet
much largercalcifications withinepiphysis) @ Lowerextremity
(2) Warfarinembryopathy ^/ genuval gum:
(3) Zellwegersyndrome rl slantingof proximaltibialmetahysis(= delayed
development of tibialplateau)
^i excessiveshorteningof fibuta
CHONDROECTODERMAL
DYSPLASIA ri wideningof proximaltibialshaft
= ELLIS-VAN CREVELD SYNDROME = MESODERMAL r/ medialtibialdiaphysealexostosis
DYSPLASIA
= autosomalrecessiveacromesomelicdwarfism OB -U S :
lncidence: 120 cases;in inbredAmish communities ri proportionalshorteningof long bones
Associatedwith: congenitalheartdiseasein 50% r/ smallthoraxwith decreasedcircumference
(s i n g l ea tri u m,AS D ,VS D ) ^/ increasedcardiothoracicratio
. ectodermaldysplasia: ./ nso
absent/ hypoplastic brittlespoon-shaped nails r/ polydactyly
irregular+ pointeddysplasticteeth,partialanodontia, Prognosis; deathwithinfirst monthof life in 33-50%
teeth may be presentat birth (due to respiratory/ cardiaccomplications)
scant/ fine hair DDx: asphyxiatingthoracicdysplasia(difficultdistinction);
. obliterationof maxillarymucobuccalspace(thickfrenula rhizomelicachondroplasia
betweenalveolarmucosa+ upperlip)
. strabismus
. genitalmalformations: epispadia,hypospadia, CHONDROMALACIA
PATELLAE
hypoplasticexternalgenitalia,undescendedtesticles = pathologicsofteningof pateltarcartilage
r/ hepatosplenomegaly Cause: trauma,trackingabnormalityof patella
r/ acceleratedskeletalmaturation . anteriorknee pain
ri normalspine . asymptomatic (incidental
arthroscopic diagnosis)
56 Radiology Review Manual

Classificationof ChondromalaciaPatellae
Grade Arthroscopic pathology TlWl of MRI
1 softening+ swellingof articularcartilage focal hypointenseareas not extendingto cartilagesurface/
subcondralbone
2 blisteringof articularcartilageproducing focal hypointenseareas extendingto cartilagesurfacewith
deformityof surface preservationof sharpcartilagemargins
3 surfaceirregularity+ cartilagefibrillationwith focal hypointenseareas extendingto articularsurfacebut not to
minimalextensionto subchondral bone osseoussurface;lossof sharpdark marginbetweenarticular
("brush-bordersign") cartilageof patella+ trochlea
4 ulcerationwith exposureof subchondralbone focal hypointenseareas extendingfrom subchondralbone to
cartilagesurface;cartilagethinnedto subchondralbone

FIBROMA
CHONDROMYXOID CHONDROSARCOMA
Rarebenigncartilaginous tumor;initiallyarisingin cortex A. PRIMARYCHONDROSARCOMA
lncidence: <1ohof all bone tumors no preexisting bone lesion
Histo: chondroid+ fibrous+ myxoidtissue (relatedto B . S E C ON D A R Y C H ON D R OS A R C OMA
chondroblastoma); may be mistakenfor as a complicationof a preexistingskeletalabnormality
chondrosarcoma such as
Age: peak 2nd-3rd decade (rangeof 5-79 years); 1. Osteochondroma
M : F= 1 : 1 2. E nchondroma
. slowlyprogressivelocalpain, swelling,restrictionof 3. Parostealchondroma
motion Spread: via marrowcavity/ periosteum
Location:(a) longbones(60%): aboutknee (50%), Metastases(uncommon)to: lung,epiduralspace
proximaltibia(82%of tibiallesions),distal
femur(71%of femorallesions),fibula CT:
"rings and arcs"
(b) short tubularbonesof hand + feet (20%) chondroidmatrix mineralizationof
(c) flat bones: pelvis,ribs(classicbut (CHARACTE RlSTIC)in 70%
unc o mmo n ) nonmineralized portionof tumorhypodenseto muscle
Site: eccentric,metaphyseal(47-53%),metadiaphyseal (highwater contentof hyalinecartilage)
(20-43/.), metaepiphysea| (26/"), diaphyseal extensioninto soft-tissues
(1-1 0%), epiphyseal(3%) MR:
{ expansileovoidlesionwith radiolucent center+ oval low to intermediate signalintensityon TlWl
, shape at each end of lesion highsignalintensityon T2Wl + hypointense areas
r/ longaxis parallelto longaxis of hostbone (1-10 cm in (dueto mineralization / fibroussepta)
length and 4-7 cm in width) enhancement of fibrousseptations
r/ geographicbone destruction(100%)
r/ well-definedscleroticmargin(86%)
r/ expandedshell= bulged+ thinnedoverlyingcortex(68%) GentralChondrosarcoma
{ partialcorticalerosion (68%) = INTRAMEDULLARY CHONDROSARCOMA = ENDOSTEAL
r/ scallopedmargin(58%) CHONDROSARCOMA
! septations(57%) may mimictrabeculations lncidence: 3rd most common primary bone tumor
r/ stippledcalcifications withintumor in advancedlesions (1st multiplemyeloma,2nd osteosarcoma);
(7%) 8-17% of biopsiedprimarybonetumors
r/ tttOperiostealreaction(unlessfractured) Path: lobularmorphologywith variableamountsof
calcium;presenceof fibrousbandsat tumor-
Prognosis: 25"h recurrencerate followingcurettage marrowinterfacesuggestsmalignancy(DDxfrom
Cx: malignantdegeneration distinctlyunusual atypicalenchondroma)
DDx: (1) Aneurysmalbone cyst Histo: arisesfrom chondroblasts(tumorosteoidis
(2) Simplebone cyst neverformed)
(3) Nonossifyingfibroma Age: median45 years; 50/" >40 years; 10% in
(4) Fibrousdysplasia chi l dren(rapi dl yfatal );M:F = 2:1
(5) Enchondroma . hyperglycemiaas paraneoplastic syndrome(85%)
(6) Chondroblastoma Location: neckof femur,pubicrami,proximal
granuloma
(7) Eosinophilic humerus,ribs (19%),skull(sphenoidbone,
(8) Fibrouscorticaldefect cerebellopontine angle,mandible),sternum,
(9) Giantcelltumor spine(3-12%)
 Disorders 57
Boneand Soft-Tissue

Site: centralwithinmedullarycanal+ meta-/ diaphysis r/ focal regionsof radiolucencyin interiorof lesion

{ expansileosteolyticlesion1 to severalcm in size
r/ shorttransitionzone + scleroticmargin(welldefined
from host bone)
{ t small irregularpunctate/ snowflaketype of
calcification;
single/ multiple
{ late: loss of definition+ breakthroughcortex
{ endostealcorticalthickening,sometimesat a distance
from the tumor (due to invasionof haversiansystem)
{ presenceof largesoft-tissuemass
DDx: benignenchondroma,osteochondroma,
osteosarcoma,f ibrosarcoma
PeripheralChondrosarcoma
= EXOSTOTIC CHONDROSARCOMA
= malignantdegeneration of hereditarymultiple
osteochondromatosis and rarelyof a solitary
exostosis(beginningin cartilaginous cap of exostosis)
Frequency: 8o/"of all chondrosarcomas
Averageage: 50-55 years for solitaryexostosis;
25-30 years for hereditarymultiple
i s ; = 1 .5:1
o s te o c h o n d ro ma to sM:F
Histo: low histologicgrade in 67-85/"
. growthafterskeletalmaturity
. graduallyincreasingpain,oftenworse at night
. localswellingi palpablemass (45%)
Location: pelvis,hip,scapula,sternum,ribs,ends of
humerus/ femur,skull,facialbones
{ growthof a previouslyunchangedosteochondromain
, a skeletallymaturepatient
! unusuallylargesoft-tissuemass 1=hyalinecartilage
cap) containingflocculent/ streakychondroid
calcifications(CHARACTE RISTIC):
{ cartilagecap 1.5-12 cm (average,5.5-6 cm) thick
0 >1.5cm is suspectof malignanttransformation
./ irregular/ indistinctlesionsurface:
r/ dense radiopaquecenterwith streaksradiatingto
peripherywith loss of smoothmargin
r/ erosion/ destructionof adjacentbone
Metastases: in 3-7oh,most commonlyto lung
Rx: wide resection
Prognosis; 70-90%long-termsurvival
DDx: (1) Osteochondroma(denselycalcifiedwith
multiplepunctatecalcifications)
(2) Parostealosteosarcoma(morehomogeneous
densityof calcifiedosteoid)
ClearCellChondrosarcoma
0 Usuallymistakenfor chondroblastoma becauseof low
grademalignancy(maybe related)!
Histo: small lobulesof tissuecomposedof cellswith
centrallyfilledvesicularnucleisurroundedby
largeclear cytoplasm
Age: 19-68 years,predominantlyafter epiphyseal
fusion
Location: proximalfemur,proximalhumerus,proximal
ulna,laminavertebrae(5%);pubicramus
Site: epiphysis
r/ singlelobulatedoval / roundsharplymarginated
lesionof 1-2 cm in size
./ surroundingincreasedbonedensity
r/ aggressiverapidgrowthover 3 cm
r/ may containcalcifications
r/ bone often enlarged
r/ indistinguishable from conventional
chondrosarcoma /
chondroblastoma (slowgrowthover years)
ExtraskeletalChondrosarcoma
lncidence: 2'/. of all soft-tissuesarcomas
Myxoid Extraskeletal Chondrosarcoma
(mostcommon)
Mean age: 50 years (range 4-92 years); M > F

Enchondroma
versusChondrosarcoma
in Appendicular
Skeleton
Enchondroma lntramedullary
Chondrosarcoma
Meanage and sex 40 years;M:F = 2:3 50 years;M:F = 11:9
Palpablemass 28% 82%
Pain 40% (tractureassociated) 95% (longerduration+ increasingseverity)
Lesionlocation hands,feet axialskeleton(spine,pelvis)
Site diaphysis metaphysis,epiphysis
Lesionsize <5 cm >5-6 cm
Endostealscalloping
relativeto corticalthickness 90/" <213of corticalthickness 90/" >213of corticalthickness,
refativeto lesionlength 66% along <213of lesion 79o/"along>213of lesion
Corticalremodeling(radiography) 15% 47%
Corticalthickening(radiography) 17% 47%
Perisotealreaction(radiography) 3% 47%
Pathologicfracture(radiography) 5% 27%
Matrixmineralization(CT) 100%(moreextensive) 94% (lessextensive)
Corticaldestruction(CT) 8% 88/o
Soft-tissueextension(MR) 3% 76%
Small hyperintensefoci (T1Wl) 65% 35%
 Histo: surroundedby fibrouscapsule+ dividedinto @ Chest
multiplelobulesby fibroussepta;delicate r/ hypoplasia/ absence(10%)of clavicles(defective
strandsof small elongatedchondroblastsare developmentusuallyof lateralportion,R > L (DDx:
suspendedin an abundantmyxoidmatrix;foci congenitalpseudarthrosis of clavicle)
of maturehyalinecartilageare rare ./ thorax may be narrowed+ bell-shaped
. slowlygrowingsoft-tissuemass r/ supernumerary ribs
. pain+ tenderness(33%) r/ incompletely ossifiedsternum
0 Metastaticin 40-45/o at time of presentation! r/ hemivertebrae, spondylosis(frequent)
Location: extremities(thighmostcommon) @ Pelvis
Site: deep soft tissues;subcutis(25%) { delayedossification of bonesformingsymphysis
r/ lobulatedsoft-tissuemass WITHOUTcalcification/ pubis(DDx: bladderexstrophy)
ossification ri hypoplasticiliacbones
r/ usuallybetween4 and 7 cm in diameter @ Extremities
MR: r/ radiusshort/ absent
r/ approximately equalto muscleon Tl Wl + equal { elongatedsecondmetacarpals
to fat on T2Wl ./ pseudoepiphyses of metacarpalbases
{ may mimica cyst/ myxoma r/ short hypoplasticdistalphalangesof hand
Prognosis: 45/" 1O-yearsurvivalrate;5-15 years { pointedterminaltufts
survivalafterdevelopmentof metastases { conedepiphyses
{ coxa v?fzl= deformed/ absentfemoralnecks
ExtraskeletalMesenchymalChondrosarcoma r/ accessoryepiphysesin hands + feet (common)
0 50% of all mesenchymal chondrosarcomas arisein OB .U S :
soft tissues { cephalopelvicdisproportion(largefetal head + narrow
Histo: proliferationof small primitivemesenchymal birthcanal of affectedmaternalpelvis)necessitates
cellswith scatteredislandsof cartilage; cesareansection
hemangiopericytoma-like vascularpattern
Bimodal age distribution: M = F
(a) tumorsof head+ neck in 3rd decade(common): coccrDrorDoMYcosrs
meninges,periorbital region Histo: chronicgranulomatous processin bones,joints,
(b) tumorsof thigh+ trunkin Sthdecade periarticular structures
. frequentlymetastasized to lungs+ lymphnodes Location: (a) bones: most frequentlyin metaphysesof
{ matrix mineralization(50-100%) characterizedas longbones+ medialend of clavicle,spine,
rings + arcs / flocculent+ stippledcalcificationi ribs,pelvisi bony prominences of patella,
densemineralization tibialtuberosity,calcaneus,olecranon,
MR: acromion
r/ approximately equalto muscleon Tl Wl + equal (b) weight-bearing joints(33%): ankle,knee,
to fat on T2Wl ytfi:i"lTeu
r/ signalvoids from calcifications " = immune-comptex-
matism
^/ homogeneous enhancement mediatedarthritis
Prognosis: 25o/"10-yearsurvival rate (c) tenosynovitis
of hand,bursitis
r/ focal areasof destruction,formationof cavities(early)
= bubblybone lesion
CLEI DOCRANIAL DYSOSTOSIS ./ bone sclerosissurrounding osteolysis(later,rare)
= CLEIDOCRANIAL DYSPLASIA = MUTATIONAL d proliferationof overlyingperiosteum
DYSOSTOSIS { destructionof vertebrawith preservationof disk space
= delayedossification of midlinestructures(particularly
of r/ psoasabscessindistinguishable fromtuberculosis,may
membranousbone) calcify
Autosomaldominantdisease r/ loints rarelyinfected(usuallymonoarticularfrom direct
@ S k ull extensionof osteomyelitic focus): synovialeffusion,
. large head osteopenia,joint space narrowing,bone destruction,
^/ diminished/ absentossificationof skull(in early ankylosis
infancy) r/ soft-tissueabscessescommon
r/ wormianbones DDx: tuberculosis
r/ widenedfontanels+ sutureswith delayedclosure
{ persistentmetopicsuture
r/ brachycephaly + prominentbossing CONGENITAL INSENSITIVITY TO PAIN
./ largemandible WITH ANHYDROSIS
^/ frighnarrowpalate(t cleft) = ra(aautosomalrecessivedisorderpresumablyon the
^/ hypoplasticparanasalsinuses basisof abnormalneuralcrestdevelopment
r/ delayed/ defectivedentition Age: presentingat birth
 Boneand Soft-TissueDisorders 59

Incidence; 15 reportedcases Histo: shallowdefectfilledwith proliferatingfibroblasts,

Path: absenceof dorsal+ sympatheticganglia, multiplesmallfragmentsof resorbingbone
deficiencyof neuralfibers<6 pm in diameter (microavulsions) at tendinousinsertions
+ disproportionate numberof fibersof 6-10 pm in . no localizingsigns/ symptoms
diameter Location: posteromedial aspectof medialfemoral
. historyof painlessinjuries+ burns(DDx:familial epicondylealongmedialridgeof lineaaspera
dysautonomia, congenitalsensoryneuropathy, at attachmentof adductormagnus
hereditarysensoryradicularneuropathy,acquired aponeurosis; 1/3 bilateral
sensoryneuropathy, syringomyelia) r/ area of corticalthickening
. abnormalpain + temperatureperception 4 t-Z cm irregular,shallow,concavesaucerlikecrater
. burns,bruises,infectionsare common with sharpmargin
. bitinginjuriesof fingers,lips,tongue r/ lamellatedperiostealreaction
. absenceof sweating r/ localizedcorticalhyperostosisproximally(healingphase)
. mentalretardation 0 May be confusedwith a malignanttumor
CRITERIA: (1) defectmustbe presentat birth (eg,osteosarcoma) / osteomyelitis!
(2) generalinsensitivityto pain
(3) generalmental/ physicalretardation CRI.DU.CHAT
SYNDROME
{ epiphysealseparationin infancy(epiphyseal injuries = deletionof shortarm of Sthchromosome(5 p)
resultin growthproblems) . generalizeddwarfismdue to markedgrowthretardation
r/ metaphysealfracturesin early childhood . failureto thrive
r/ diaphysealfracturesin late childhood . peculiarhigh-pitched cat cry (hypoplastic
larynx)
r/ Charcotjoints= neurotrophic joints(usuallyweight- . antimongoloid palpebralfissures
bearingjoints)with effusions+ synovialthickening . strabismus
r/ ligamentouslaxity . profoundmentalretardation
./ bizarredeformities+ gross displacement+ considerable . foufld facies
hemorrhage(unnoticedfractures+ dislocations) . low-setears
r/ osteomyelitis+ septicarthritismay occur + progress Associatedwith: congenitalheartdisease(obtainCXR!)
extensively r/ agenesisof corpuscallosum
DDx: (1) sensoryneuropathies (eg,diabetesmellitus) r/ microcephaly
(2) hysteria r/ hypertelorism
(3) syphilis r/ smaltmandible
(4) mentaldeficiency, r/ faultylong-bonedevelopment
(5) syringomyelia i/ short 3rd, 4th, 5th metacarpals
(6) organicbraindisease ^/ tong2nd, 3rd, 4th, sth proximalphalanges
r/ horseshoekidney
CORNELIA
DE LANGESYNDROME Dx: madeclinically
= AMSTERDAM DWARFISM
. mentalretardation(lQ <50) CROUZON
SYNDROME
. genitalia
hirsutism;hypoplastic = CRANIOFACIALSYNOSTOSIS/ DYSOSTOSIS
. feeblegrowlingcry = Apert syndromewithoutsyndactyly
. highforehead;shortneck = skull + cranialbase deformitiescharacterizedby
. archedpalate craniosynostosis, maxillaryhypoplasia, shalloworbits,
. bushyeyebrowsmeetingin midline+ longcurved ocularproptosis,bifid uvula,cleft palate
eyelashes Prevalence: 1:25,000
. small nose with depressedbridge;upwardtiltednostrils; Etiology: autosomaldominantinheritance(in 67%)
excessivedistancebetweennose + upper lip Associatedintracranialanamalies:
{ small+ brachycephalic skull anomalousvenousdrainage,hydrocephalus (often
^/ hypoplasiaof long bones (upperextremitymore progressive),ChiariI malformation(71%)
involved) . parrot-beaknose
r/ forearm bones may be absent . strabismus
{ shortradius+ elbowdislocation . deafness
r/ thumbsplacedproximally(hypoplastic 1st metacarpal) . mentalretardation
{ shortphalanges+ clinodactyly of Sthfinger . dentalabnormalities
. bifiduvula
DESMOID
CORTICAL . acanthosisnigrans(= hyperpigmented hyperkeratotic
= AVULSIVECORTICALIRREGULARITY = PERIOSTEAL/ lesionson neck+ nearjointflexures)
SUBPERIOSTEAL DESMOID= SUBPERIOSTEAL / CORTICAL
ABRASION= SUBPERIOSTEAL CORTICALDEFECT rl prematurecraniosynostosis:acro(oxy)cephaly/
= tdra fibrous lesion of the periosteum brachycephalyI scaphocephalyI trigonocephalyI
Age: peak 14-16 years (range of 3-17 years); M:F = 3:1 "cloverleaf"
skull
 { hypertelorism+ exophthalmos(due to shalloworbits)
r/ hypoplasticmaxilla(relativeprominenceof mandible)
{ cleft palate
r/ calcificationof stylohyoidligament(in 50% of patients
>4 years of age)
\i C2 to C5 spine abnormalities(in up to 4O%)
! elbowmalformation (18%)
{ minorhanddeformities(10%)
r/ visceralanomalies(7%)
r/ musculoskeletal deformities (7%)
O B . US :
r/ cloverleafappearance(coronalview) + bilateral
frontalindentations(axialview) of skull
^/ increasedinterorbitaldistance+ ocularproptosis
{ mildventriculomegaly
CRUCIATELIGAMENTINJURY
A. COMPLETE
TEAR
r/ failureto identifyligament
{ amorphousareasof high signalintensityon TlWl
+ T2Wl with inabilityto defineligamentousfibers
{ focaldiscretecompletedisruptionof all visiblefibers
B. PARTIAL/ INTRASUBSTANCE TEAR
r/ abnormalsignalintensitywithinsubstanceof
ligamentwith some intact+ some discontinuous
fibers
AnteriorCruciateLigamentInjury(ACL)
0 lf the ACL appearsintactin one of the sagittaloblique
seguencesdiscordantfindingsin othersequences
can be disregarded!
Site: intrasubstance tear near insertionof femoral
, condyle(frequently); bone avulsion(rarely)
{ hyperintensesignal(= focalfluid collection/ soft-
tissueedema)replacingthe tendonsubstancein
acute tear
{ mass (hematoma+ torn fibers)in intercondylarnotch
near femoralattachment
r/ concavityof anteriormarginof ligament
lndirect findings:
0 The indirectsigns of ACL injuryhave a low
sensitivitybut high specificity!
r/ bone bruisein lateralcompartment(posterolateral
tibia + mid lateralfemur)in >50%
r/ deepeningof lateralfemoralsulcus>1.5 mm
{ posteriordisplacementof posteriorhorn of lateral
meniscus>3.5 mm behindtibialplateau
{ anteriortranslationof tibia (= ?nteriordrawersign)
^/ pCL bowing= €lflgl€betweenproximal+ distal
lim bsof P C L < 1 0 5 o
False-positive Dx:
(1) slicethickness/ interslicegap too great
(2) adjacentfluid/ synovialproliferation
(3) cruciateganglion/ synovialcyst
Associated injuries:
meniscaltear (lateral> medial)in 65%
Chronic ACL Tear
{ often completeabsenceof ligament
r/ bridgingfibrousscar within intercondylarnotch
(simulatingan intactligament)
PartialAcL Tear(15%)
0 Extremelydifficultto diagnose!40-50% of partial
tearsare missedon MR!
. positiveLachmantest (in 1244%)
^/ Un primarysigns positivefor injury(in 33-43%)
PosteriorCruciateLigamentInjury(PCL)
Prevalence: 2-23% of all knee injuries
r/ midsubstanceof PCL most frequentlyinvolved(best
seen on sagittalimages)
{ boneavulsionfrom posteriortibialinsertion(<10%),
bestseen on lateralplainfilm
Mechanism:
(1) Directblowto proximalanteriortibiawith knee
flexed (dashboardaccident)
r/ midsubstancePCL tear
r/ inluryto posteriorjoint capsule
./ bone contusionat anteriortibialplateau
+ femoralcondylesfartherposteriorly
(2) Hyperextension of knee
{ avulsionof tibialattachmentof PCL (with
preservationof PCL substance)
./ t RCL rupture
./ bone contusionin anteriortibialplateau
+ anterioraspectof femoralcondyles
(3) Severeab- / adduction+ rotationalforces
{ + injuryto collateralligaments
Associatedwith: coexistentligamentousinjury in 70o/"
anteriorcruciateligament 27-38%
medialcollateralligament 20-23'/"
lateralcollateralligament 6-7%
medial meniscaltear 32-35%
fateralmeniscaltear 28-30%
bone marrow injury 35-36%
effusion 6+65%
0 In 30% of casesinjuryof PCL is isolated!
. posteriortibiallaxity
. difficultto evaluatearthroscopically
unlessACL torn
DEEPFIBROMATOSES
AggressiveInfantileFibromatosis
= childhoodequivalentof deep fibromatosis
Age: first 2 years of life; rarely>5 years of age; M > F
Histo: may mimic infantilefibrosarcoma
. firm nodularsoft-tissuemass withinskeletalmuscle/
fascia/ periosteum
Location: head,neck (tongue,mandible,mastoid),
shoulder,thigh,foot
DesmoidTumor
Extraabdominal
= AGGRESSIVE FIBROMATOSIS = DEEPFIBROMATOSIS
= MUSCULOAPONEUROTIC FIBROMATOSIS
= corT]rTlor't
benignaggressivelygrowingsoft-tissue
tumorarisingfrom connectivetissueof muscle,
fascia,aponeurosisoutsideabdominalcavity
Peak age: 25-35 years

Histo: parallelhalo arraysof uniform-appearing
fibroblastssurroundedby highlyvariable
amountsof collagenfiberswith infiltrative
growth pattern
. painlesssoft-tissuemass
Location: extremities(70%);shoulder(20/"), chest
wall + back (15%),thigh (12%),mesentery
(10%),neck (10%),knee (T%);solitary
(majority)/ synchronousmulticentricityin
same extremity(10-1 5%)
0 Most commonbenignsoft-tissuetumor of the foot
Site: fasciain / aroundmuscle
{ mostly<10 cm in diameter
@ Bone
r/ Erlenmeyer flaskdeformityin multicentric
f ibromatosis (infrequent)
@ Abdominalwall
Prevalence: in 87"/"in femalesof childbearingage
Predilection: for female patientstaking birth control
pills/ duringor afterpregnancy
Location: aponeurosis of rectusabdominis,
internalobliquemuscle
MR:
{ poorlydefined(withinvasionof fat / muscle)/
lobulatedwell-definedlesion
1 nypo-/ isointense to muscleon Tl Wl
! hyperintense (hypercellular)/ hyperintense
with
areasof low intensity(intermixedwith fibrous
components) / hypointense (hypocellular)
on T2Wl
Cx: compresses/ engulfsadjacentstructures
Prognosis: 75/" recurrencewithin2 years aftersurgical
excision(up to 87"h localrecurrencein
<30 years of age; 20/" recurrencerate in
>20 years of age)
AbdominalDesmoidTumor
= DESMOID TUMOR
= uftcorTlrnon benigntumorof the subgroupof
fibromatoses consistingof fibroustissuewith insidious
growthldesmos, Greek = band / tendon]
Location: mesentery(mostcommonmesenteric
primary),musculoaponeurosis of rectus
internalobliquemuscle;occasionally
externalobliquemuscle
Age: peak age in 3rd decade,70/" between20 and
40 y ear so f a g e ; M:F = 1 :3
Path: poorlycircumscribedcoarselytrabeculated
tumorresemblingscar tissue,confinedto
musculature + overlyingaponeurosis
Histo: elongatedspindle-shaped cellsof uniform
appearance,septatedby dense bands of
collagen,infiltrationof adjacenttissue
(DDx: low-gradefibrosarcoma,reactivefibrosis)
Associatedwith: Gardnersyndrome,multiple
pregnancies, priortrauma
. firm slowlygrowingdeep-seatedmass
Size: 5-20 cm in diameter
MR:
r/ hypointense to muscleon TlWl + variableintensity
on T2Wl
Boneand Soft-Tissue
Disorders 61
CT:
,Vill-defined/ well-circumscribed
mass
,Vusuallyhigherattenuationthan muscle
{ t enhancement
^/ retraction,angulation,distortionof small llarge
bowelwith mesentericinfiltration
US:
r/ sharplydefined+ smoothlymarginatedmass of low
/ medium/ highechogenicity
Cx: compressioni displacementof bowelI ureler,
intestinal perforation
Prognosis; locallyaggressivegrowth; 2545%
recurrencerate
Rx: localresection+ radiotherapy,antiestrogen
therapy
DDx: (1) Malignanttumor: metastasis, fibrosarcoma,
rhabdomyosarcoma, synoviosarcoma,
liposarcoma,fibroushistiocytoma, lymphoma,
(2) Benigntumor: neurofibroma, neuroma,
leiomyoma
(3) Acute hematoma
InfantileMyofibromatosis
= GENERALIZED HAMARTOMATOSIS = CONGENITAL
MULTIPLEFIBROMATOSIS = MULTIPLEVASCULAR
LEIOMYOMAS = DESMOFIBROMATOSIS
= tale disorder characterized by proliferation of
fibroblasts
Cause: unknown
Frequency; most commonfibromatosisin childhood
Age: at birth(in 60%),<2 years(in 89%); M:F = 1.7:1
Path: well-marginated softtissue lesion0.5-3 cm in
diameterwith scarlikeconsistency+ infiltrationof
surrounding tissues
Histo: spindle-shaped cells in shortbundlesand
fasciclesin peripheryof lesionwith featuresof
both smoothmuscle+ fibroblasts;
hemangiopericytoma-like patternin centerwith
necrosis,hyalinization, calcification
(1) Solitarylesion(50-75%)
Location: dermis,subcutis,muscle(86%);head,
neck,trunk,bone (9/"), Gl tract(4%)
Prognosis; spontaneousregressionin 100%;
recurrenceaftersurgicalexcisionin
7-10%
(2) Multicentricdisease(25-50%)
Location: skin (98%),subcutis(98%),muscle
(98%),bone (57%),viscera(25-37%):
lung (28%),heart(16%),Gl tract(14h),
pancreas(9%), liver(8%)
Prognosis: relatedto extent + locationof visceral
lesionswith cardiopulmonary + Gl
involvementas harbingersof poor
prognosis(deathin 75-80%);
spontaneousregression(33%)
. firm nodulesin skin,subcutis,muscle
. + overlyingscarringof skin with ulceration
 @ Skeleton . gradualonset of muscleweakness
Location: any bone may be involved;commonlyin . elevatedmuscleenzymes(creatininekinase,aldolase)
femur,tibia,rib,pelvis,vertebralbodies, . myositis-specific autoantibodies: anti-Jo-1
calvarium;oftensymmetric (a) anti-aminoacyl-tRNA synthetase
Site: metaphysisof long bones . arthritis,Raynaudphenomenon, fever,fatigue
{ eccentriclobulatedlyticfoci with smoothmargins . interstitial lungdisease
0 . 5- 1 . 0 c m i n s i z e Prognosis; requiresprolongedtreatment
r/ well-definedwith narrowzone of transition (b) anti-Mi-2antibodies:
r/ initiattyno sclerosis;scleroticmarginwith healing . V-shapedchest rash (= shawl rash)
./ osseousfoci may increasein size and number . cuticularovergrowth
{ healingleaveslittleresidualabnormality Prognosis: good responseto medication
r/ unusualosseousfindings: (c) anti-signalrecognitionparticleantibodies
{ periostealreaction,pathologicfracture . abruptonset myositis+ heart involvement
{ vertebraplana,kyphoscoliosis with posterior @ Skeletalmusculature
scallopingof vertebralbodies Location: thigh(vastuslateralis+ intermedius m. with
NUC (bonescan): relativesparingof rectus+ bicepsfemoris
{ increased/ littleradiotraceruptake m.) > pelvicgirdle> upperextremity>
DDx: (1) Langerhanscell histiocytosis (skinlesions) neck flexors> pharyngealmuscles
(2) Neurofibromatosis (multiplemasses) r/ bilateralsymmetricedemain pelvic+ thighmuscles
(3) Osseoushemangiomas / r/ tany infiltration+ muscleatrophy(over monthsto
ymphangiomatosis / lipomatosis years)
(4) Metastaticneuroblastoma { sheetlikeconfluentcalcificationsin soft tissuesof
(5) Multiplenonossifying fibromas extremities(quadriceps, deltoid,calf muscles),
(6) Enchondromatosis elbows,knees,hands,abdominalwall,chestwall,
,t, (unusual
osteomyelitis axi l l a,i ngui nalregi on)i n75/"
lt,.ilfi,Tflenous @ Skeleton
(8) Fibrousdysplasia { pointing+ resorptionof terminaltufts
@ Soft tissue { rheumatoid-like arthritis(rare)
r/ solid mass with centralnecrosis r/ "floppy-thumb" sign
r/ central/ peripheralsolitary/ multiplecalcifications Cx: flexioncontractures;soft-tissueulceration
{ prominentvascularity of skin lesionsresembles @ Chest
hemangiomas . respiratorymuscleweakness
CT : r/ disseminated pulmonaryinfiltrates (reminiscentof
{ attenuationincreasedcomparedto muscle, scleroderma)
before+ aftercontrastenhancement ^/ diaphragmatic elevationwith reducedlungvolumes
MR: + basilaratelectasis
^/ nypo-to hyperintensemass on TlWl + T2Wl r/ interstitialfibrosis(5-30%), most severelyat lung
DDx: (1) Neurofibromatosis bases:
(2) Infantilefibrosarcoma,leiomyosarcoma ^/ fine reticularpatternprogressingto coarse
(3) Angiomatosis reticulonodular pattern+ honeycombing
@ Lung { bronchiolitis obliteransorganizingpneumonia
r/ interstitialfibrosis,reticulonodular infiltrates r/ diffusealveolardamage
r/ discretemass H R C T:
! generalizedbronchopneumonia { predominantly linearabnormalities + ground-glass
@ Gl tract attenuation
{ diffusenarrowing/ multiplesmallfillingdefects { air-spaceconsolidation in middle+ lowerlung
zoneswith peribronchial + subpleuraldistribution
Cx: aspirationpneumonia(mostcommonfinding
DERMATOMYOSITIS due to pharyngealmuscleweakness)
= autoimmuneinflammatorymyopathywith diffuse @ Myocardium
nonsuppurative inflammationof striatedmuscle+ skin r/ changessimilarto skeletalmuscle
Cause: cell-mediated(type lV) autoimmuneattackon @ Gl tract
striatedmuscle . progressiveweaknessof proximalstriatedmuscle:
Pathophysiology: damaged chondroitinsulfate no . dysphagia
longerinhibitscalcification { atony + dilatationof esophagus
Path: atrophyof musclebundlesfollowedby edema and r/ atonyof small intestines+ colon
coagulationnecrosis,fibrosis,calcification
Histo: mucoiddegenerationwith roundcell infiltrates ACUTEFORM= childhood-onset form
concentratedaroundbloodvessels . fever,jointpain,lymphadenopathy,
splenomegaly,
Age: bimodal:5-15 and 50-60 years; M:F = 1:2 subcutaneousedema

/ more severedermatomyositis
Prognosis; deathwithina few months
CHRONICFORM= adult-onset form
= insidiousonsetwith periodsof spontaneous remission
and relapse
. low-gradefever,muscularaches+ pains,edema
. muscleweakness(dueto activeinflammation,
necrosis,muscleatrophywith fatty replacement,
steroid-inducedmyopathy)
0 first symptomin 50%
. skin erythema: heliotroperash (= dusky erythemaof
eyelids)with periorbitaledema,Gottronsign (= scaly
erythematouspapulesof knuckles,majorjointsand
upperbody)
0 firstsymptomin 25/.
Cx: increasedprevalenceof malignantneoplasmsof
breast,prostate,lung,ovary,Gl tract,kidney
Dx: musclebiopsy(normalin up to 1S%)
Polymyositis
= involves
skeletal
muscleonly
Age: 4thdecade
DESMOPLASTICFIBROMA
= INTRAOSSEOUS DESMOID TUMOR
= r?r€locallyaggressivebenignneoplasmof bonewith
borderlinemalignancyresemblingsofttissuedesmoids/
musculoaponeurotic f ibromatosis
lncidence: 107 cases in world literature
Histo: intracellularcollagenousmaterialin fibroblasts
with smallnuclei
Age: meanof 21 years(range15 monthsto 75 years);
in 90% < 30 y e a rs ;M:F = 1 :1
. slowlyprogressivepain + localtenderness
. palpablemass
Location: mandible(26%),ilium(14/"),>50o/oin long
bones(femur114/.1,humerus111%1, radius
[9%], tibia[7%1,clavicle),scapula, vertebra,
calcaneus
Site: centralmeta-/ diaphyseal(if growthplateopen);
may extendintoepiphysiswith subarticular location
(if growthplate closed)
^/ geographic(96%)/ moth-eaten(4%) bone destruction
withoutmatrixmineralization
^/ narrow(96%)/ poorlydefined(4h) zoneof transition
r/ no marginalsclerosis(94%)
r/ residualcolumnsof bonewith "pseudotrabeculae"
, o LA S S T C( e1% )
ri bone expansion(89%);may grow to massivesize
(simulating aneurysmalbonecyst/ metastaticrenalcell
carcinoma)
i/ breachof cortex+ soft-tissuemass (29%)
Cx: pathologictracture(9/")
Prognosis: 52o/"rate of local recurrence
Rx: wide excision
DDx: (1) Giantcelltumor(roundratherthan oval,may
extendintoepiphysis+ subchondralbone plate)
Boneand Soft-TissueDisorders 63
(2) Fibrousdysplasia(occupieslongerbone,
containsmineralizedmatrix,oftenwith sclerotic
rim)
(3) Aneurysmalbone cyst (eccentricblowout
appearanceratherthan fusiform)
(,4) Chondromyxoidfibroma(eccentricwith
delicatemarginalsclerosis+ scallopedborder)
D E V E LOP ME N TA L D Y S P LA S IA OF H IP (D DH)
= CONGENITAL DYSPLASIA OF HIP
= deformityof acetabulumdue to disruptedrelationship
betweenfemoralhead and acetabulum
0 Acetabulardysplasia(withoutfemoralsubluxationi
dislocation) can be determinedonly by imaging!
Etiology:
A. Late intrauterineevent (98%)
(a) mechanical:
- oligohydramnios (restricted spacein utero)
- firstborn(tightmaternalmusculature)
0 in 60% of patientswith DDH
- breechposition(hip hyperflexion resultsin
shorteni ngof i l i opsoas muscl e;L:R = 4: 1)
0 in 30-50"/. o'tpatientswith DDH
0 only 24/" of deliveriesare breech
(b) physiologic(femalesare moresensitiveto):
- maternalestrogen(not inactivatedby
immaturefetalliver)blockscross-linkage of
collagenfibrils
pregnancyhormonerelaxin
B. Teratologic(2%)due to a neuromuscular disorder
(myelodysplasia, arthrogryposis) occurringduring
12th-18th week GA
C. Postnatalonset(<1%)
lncidence; 0.15/" of neonates(Australia1%,
Netherland s 3.7"/",Poland3.9o/o, lsrael
5.9/", Austria6.6o/o, Norway16.9%)
Age: most dislocationsprobablyoccur after birth;
M:F = 1:4-1:8;C aucasi ans > B l acks
lncreased risk:
(1) infantsborn in frankbreechposition(25%;riskof
breech:vertex = 6-8:1)
(2) congenitaltorticollis(10-20%)
(3) skull-molding deformities; scoliosis;generalized joint
laxity(Larsensyndrome,Ehlers-Danlos syndrome,
Downsyndrome[5%]);neuromuscular disorders(eg,
myelodysplasia, spinabifida,sacralagenesis,
arthrogryposismultiplex)
are (4) familyhistoryof DDH (6-20%): 6% risk for
subsequentsiblingof normalparents,36% riskfor
subsequentsiblingof one affectedparent;12h risk
for patient'sown children
(5) foot deformities [metatarsusadductus,clubfo otl(z%)
(6) neonatalhyperextension of hips: swaddlingof
infantsin hip extension/ strappingto cradleboard
Anatomy: acetabulumhas a small bony component+ a
largecartilaginous componentat birth;
acetabulumhighlysusceptible for modeling
withinfirst6 weeksof age + minimally
susceptible>16 weeksof age
Classification:
1 . N o r m a hl i p
2. Lax = subluxablehip
0 subluxability up to 6 mm is normalin newborns
(stillunderinfluenceof maternalhormones);
decreasingto 3 mm by 2nd day of life
3. ConcentricDISLOCATABLE UNSTABLEHIP
= joint laxityallowingnondisplacedfemoralhead to
becomesubluxedI dislocatedunderstress
lncidence: 0.25-0.85ohof all newborn infants
(213are firstborns)
. Barlowpositive
i/ slightincreasein femoralanteversion
r/ mild marginalabnormalitiesin acetabularcartilage
{ early labraleversion
Prognosis: 60ohwill become stable after 1 week;
88% will becomestableby age of
2 months
4. DecenteredSUBLUXEDHIP
= femoralheadshallowin location
{ loss of femoralhead sphericity
^/ increasedfemoralanteversion
r1 early labralinversion
{ shallowacetabulum
5. EccentricDISLOCATEDHIP
= femoralhead franklydisplacedout of acetabulum
(a) reducible= Ortolanipositive
(b) irreducible= Ortolaninegative
r/ accentuatedflatteningof femoralhead
r/ shallowacetabulum
r/ limbusformation(= inwardgrowth+ hypertrophy
of labrum)
. "hipclick"= usuallyresultof jointcapsuleand tendon
"hip clunk")
stretching+ snapping(oftenconfusedwith
. positiveexaminationresult(up to 3 monthsof age):
. positiveOrtolani reduction test = reductionof
proximalfemur into the acetabulumby progressive
abductionof flexedhips and knees+ associatedwith
audible"clunk"
. positiveBarlow dislocation test = displacementof
proximalfemur by progressiveadductionwith
downwardpressure(pistonmaneuver)on flexed hips
and kneesassociatedwith audible"clunk"
. warningsignson physicalexamination:
. limitedhip abductionon affectedside
. shorteningof thigh on affectedside:
. asymmetricthigh / buttockcreases
. Allis sign = Galeazzisign = affectedknee is lower
with kneesbent in supineposition
. Trendelenburg test = visibledrooping+ shortening
on dislocatedside with child standingon both feet,
then one foot
= 11:1:4
Location: left:right:bilateral
Radiologic lines:
1.Lineof Hilg e n re i n e r
= line connectingsuperolateralmarginsof triradiate
cartilage
Perkin's line
center-edge
acetabularangle
Shenton's line
RadiographicLines of Hip Joint Position
2. Acetabularangle/ index
= slope of acetabularroof = ?rl9lethat lies between
Hilgenreiner's lineand a linedrawnfrom most
superolateralossifiededge of acetabulumto
superolateralmarginof triradiatecartilage
3. P erki n' sl i ne
= verticallineto Hilgenreiner's linethroughthe lateral
rim of acetabulum
4. Shenton'scurvedline
= dfc formedby inferiorsurfaceof superiorpubic
ramus(= top of obturatorforamen)+ medialsurface
of proximalfemoralmetaphysisto levelof lesser
trochanter
ri disruptionof line (DDx: coxavalga)
5. Center-edge angle
= oflgle subtendedby one line drawnfrom the
acetabularedge to centerof femoralhead + second
lineperpendicular to lineconnectingcentersof
femoralheads
./ <25'suggestsfemoralhead instability
AP pelvicradiograph:>6-8 weeks of age (von Rosen
view = legs abducted45" + thighs internallyrotated)
0 Not reliablefirst 3 monthsof life!
r/ proximal+ lateralmigrationof femoralneck:
r/ eccentricpositionof proximalfemoralepiphysis
(positionestimatedby a circledrawn with a
diameterequivalentto width of femoralneck)
^/ interrupteddiscontinuous arc of Shenton'sline
./ tinedrawn along axis of femoralshaftwill not pass
throughupperedge of acetabulumbut intersectthe
anterior-superioriliacspine(duringBarlow
maneuver)
{ apex of metaphysislateralto edge of acetabulum
ri femoralshaft above horizontalline drawn through
the Y-synchondroses
 Boneand Soft-Tissue
Disorders 65

r/ unilateralshorteningof verticaldistancefrom tr posterior+ superiordislocationof head against

femoralossificnucleus/ femoralmetaphysisto
Hilgenr ein e r'l isn e
r/ femoralossificnucleus/ medialbeak of femoral
metaphysisoutsideinnerlowerquadrantof
coordinatesestablished by Hilgenreiner's+ Perkin's
lines
r/ acetabulardysplasia= shallowincompletely
developedacetabulum:
I acetabularangle >30ostronglysuggestsdysplasia
^/ developmentof false acetabulum
r/ delayedossificationof femoralepiphysis(usually
evidentbetween2nd and 8th monthof life)
US (practicalonly up to 4-6 monthsof age):
0 Too sensitiveduringfirst 2 weeks of lifel
(1) staticevaluation(popularized in Europeby Graf)
(2) dynamicevaluation(popularizedin USA by Harcke)
@ Relationship of femoralhead& acetabulum
r/ femoralhead positionat rest in neutralposition:
'V frip instabilityunder motion
+ stressmaneuvers:
r/ dislocated(= eccentric)hip can be reduced
(Ortolanipositive):
ri hypoechoicfemoral head not centeredover
triradiatecartilagebetweenpubis+ ischium(on
transverseview)
r/ increasedamountof soft-tissueechoes
("pulvinar")betweenfemoralhead and
acetabulum
r/ cartilaginousacetabularlabruminterposed
betweenhead and acetabulum(inverted
labrum)
i l i um
{ equatorsign = <5O/oof femoralhead lies medial
to linedrawnalongiliacbone (on coronalview):
>58% coverageis normal;58% to 33% coverage
is indeterminate; <33% coverageis abnormal
@ Femoralhead
r/ disparityin size of directlyvisualizedunossified
femoralhead
r/ disparityin presence+ size of ossificnucleus
@ Acetabulum
r/ delayedossificationof acetabularcorner
tr wavy contourof bony acetabulumwith onlyslight
curvature
r/ abnormallyacutealphaangle(= anglebetween
straightlateraledgeof ilium+ bonyacetabular
margin)
{ o >60oin an infantis normal
r/ u 55-60" can be normal<4 weeks of age
{ cr<55'occurs in an immatureacetabulum
0 4o-6ointerobservervariation!
Prognosis: alpha-angle<50'at birth/ 50"-59" after
3 monthsindicatessignificantriskfor
dislocationwithouttreatment;follow-upat
4-weekintervalsare recommended
CT:
sectorangle = ?rtgl€betweenline drawn from centerof
femoralhead to acetabularrim + horizontalaxis of
pelvis(= reflectionof acetabularsupport)
! anterioracetabularsectorangle <50'
{ posterioracetabularsectorangle <90"

SonographicHip Types
Type Description a Angle B Angle
1 maturehip >60o
1A narrowcartilaginousroof <55o
1B wide cartilaginousroof >55o ct >60"
2 deficientbony acetabulum
2A physiologic <3 months 50 - 59.
28 delayedossification bony
acetabulum
>3 months
2C concentricbut unstable; \
critical range 43 - 49. 70 -77" \ fibrocartilaginous
acetabulum
2D decentered= subluxed >77"
3 eccentric= dislocated <43o
4 severedysplasiawith
invertedlabrum

(1) crangle = ?flgl€betweenstraightlateraledgeof ilium

+ bony acetabularmargin(on coronalview);
determinessonographichip type F.77'
(2) B angle = drtglebetweenstraightlateraledge of ilium
+ f ibrocartilaginous
acetabulum;
Coronal View of Left Hip
determinesnuancesof sonographic hip type
 500 *'::fllTJllJ'*
multiplejointflexioncontractures (notablyof major
joints)
dislocation of one / more largejoints(hip,elbow),
lateraldislocationof patella
horizontal axis of pelvis coxavara (common)
mediallybowed metatarsals
clubfoot= seV€fetalipesequinovarus
ulnardeviationof hands
oval + hypoplastic1st metacarpalbone + abducted
proximallypositionedthumb= "hitchhiker's thumb"
(cHARACTERTSTTC)
Acetabular Sector Angles (in normalright hip) r/ bizarrecarpalboneswith supernumerarycenters
r/ widelyspacedfingers
Cx: (1) Avascularnecrosisof femoralhead OB -U S :
(2) Intraarticular
obstacleto reduction ri proportionately shortenedlong bones
(a) pulvinar= fibrofattytissueat apex of { nitcfrniner thumb
acetabulum r/ clubfeet
(b) hypertrophyof ligamentumteres ^/ loint contractures
(c) labralhypertrophy/ inversion ^/ abnormalspinalcurvature
(3) Extraarticularobstacleto reduction Prognosis; death in infancy(due to abnormalsoftening
iliopsoastendonimpingement on anteriorjoint of trachealcartilage)
capsulewith infoldingof jointcapsule
Prognosis: 78/" of hips becomespontaneouslynormalby SKELETALHYPEROSTOSIS
IDIOPATHIC
DIFFUSE
4th week + 90% by 9th week = DISH= FORESTIER DISEASE = ANKYLOSING
Rx: (1) Flexion-abduction-external rotationbrace (Pavlik HYPEROSTOSIS
harness)/ splint/ spicacast = corT'rrnol]ossifyingdiathesischaracterizedby bone
(2) Femoralvarus osteotomy proliferation at sitesof tendinous+ ligamentous
(3) Pelvic(Salter)/ acetabularrotation attachment(enthesis)
(4) lncreasein acetabulardepth (Pemberton) Etiology:
(5) Medialization of femoralhead (Chiari) (1) may be causedby alteredvitaminA metabolism
(elevatedplasmalevelsof unboundretinol)
(2) long-termingestionof retinoidderivativesfor
DIASTROPHIC DYSPLASIA treatmentof acne (eg, Accutane@);
= DIASTROPHIC DWARFISM = EPIPHYSEAL DYSOSTOSIS ? hypertrophicvariantof spondylosisdeformans
= autosomalrecessivesevererhizomelicdwarfism A ge: > 50 years;M:F = 3:1
secondaryto generalizeddisorderof cartilagefollowed . pain,tendernessin extraspinal locations
by fibrousscars + ossifications . restrictedmotionof vertebralcolumn
. diastrophic= "twisted"habitus . hyperglycemia
. "cauliflowerear" = €€rrdeformityfrom inflammationof . positiveHLA-B,27in 34o/o
pinna r/ increasedincidenceof hyperostosisfrontalisinterna
. laryngomalacia @ S pi ne
. lax + rigidjointswith contractures Location: middle+ lowerthoracic> lowercervical>
. normalintellectual development enti rel umbarspi ne
./ flowingossificationof at least4 contiguousvertebral
@ Axial skeleton bodies:
r/ cleft palate (25%) { osteophyteslocatedanteriorly+ laterallyon right
{ cervicalspinabifidaocculta side (not on left becauseof aorta)
r/ hypoplasiaof odontoid r/ osteophyteslargestat levelof intervertebral disk
{ severeprogressivekyphoscoliosis of lumbarspine r/ radiolucencybeneathdepositedbone
(not presentat birth) r/ Cist<spaceswell preserved,no apophyseal
^/ narrowedinterpedicular spacein lumbarspine ankylosis,no sacroiliitis
{ short + broadbony pelvis @ Pelvis
r/ posteriortilt of sacrum ^/ briOgeacrosssuperioraspectof symphysispubis
@ Extremities r/ ossificationof iliolumbar+ sacrotuberous+ sacroiliac
r/ severemicromelia(predominantly rhizomelic ligaments(highprobability for presenceof spinal
= humerus+ femurshorterthan distallongbones D IS H ,D D x:fl uorosi s)
r/ widenedmetaphysis { "whiskering" at iliaccrest,ischialtuberosity,
r/ flattenedepiphysis(retardationof epiphyseal trochanters
ossification)with invaginationof ossificationcenters ri broadosteophytesat lateralacetabularedge, inferior
intodistalendsof femora portionsof sacroiliacjoints
 Boneand Soft-TissueDisorders 67

@ Extremities { contusion/ microfracture / osteochondralinjuryof

r/ Oigheel spurs (on plantar+ posteriorsurfaceof nonarticularsurfaceof lateralfemoralcondyle
calcaneus) + medialarticularsurfaceof patella
{ spur of olecranonprocessof ulna r/ hemarthrosis
{ spur on anteriorsurfaceof patella MR:
r/ ossificationof coracoclavicularligament,patellar r/ bone contusionof anterolateralaspectof lateral
ligament,tibialtuberosity,interosseous membranes femoralcondyle+ inferomedialaspectof patella
Cx: postoperativeheterotopicbone formation(hip) { sprain/ disruption/ avulsionof medialretinaculum
DDx: (1) Fluorosis(increasedskeletaldensity) + medialpatelfofemoral ligament+ medial
(2) Acromegaly(posteriorscalloping, skullfeatures) patellotibial
ligament
(3) Hypoparathyroidism i elevationof vastusmedialisobliquusmuscle
(4) X-linkedhypophosphatemic vitaminD-resistant Rx: (1) Temporaryimmobilization + rehabilitation:
rickets successful i n75/"
(5) Ankylosingspondylitis(squaringof vertebral (2) Surgery: fixationof osteochondralfragments,
bodies,coarsersyndesmophytes, sacroiliitis, medialcapsulerepair,lateralretinacular
apophysealalteration) release,vastus medialiset lateralis
(6) Intervertebral
osteochondrosis (vacuum rearrangement, medialretinaculum reefing
phenomenon, vertebralbody marginalsclerosis,
decreasedintervertebraldisk height) ShoulderDislocation
SternoclavicularDislocation p%)
DISLOCATION Acromioclavicular Dislocation (t2%)
Hip Dislocation grade 1 = soft-tissue
swelling+ no jointwidening
lncidence: 5h of all dislocations grade2 = subluxation with elevationof clavicleof
A. posTERroR Htp DTSLOCATTON (80_85%) <5 mm (weight-bearing !)
Mechanism: classicaldashboardinjury (= flexed grade 3 = dislocationwith wide AC joint + increased
knee strikesdashboard) coracoclavicular distance
Associatedwith: fracturesof posteriorrim of
acetabulum, femoralhead GlenohumeralDislocation(gS%)
B . A NT E RT O R
H tp D T S L O C AT T ON
(s _ 1 0 % ) 0 Glenohumeraljoint dislocations
make up >50%of
1. anteriorobturatordislocation all dislocations!
2. superoanterior lpubic hip dislocation
Associatedwith: fracturesof acetabularrim,
Arurenron/ SueconacotoSnouloeR DrslocATtoN
greatertrochanter,femoralneck,
(e6%)
femoralhead (characteristic
Types: subcoracoid, subglenoid, subclavicular,
depressionon posterosuperior
intrathoracic
and lateralportion)
C. CENTRALACETABULARFRACTURE- Mechanism: externalrotation+ abduction:
DISLOCATION 40% recurrent
Mechanism;forceappliedto lateralside of trochanter Age: in youngerindividuals
May be associated with:
r/ fractureof greatertuberosity(15%)
PatellarDislocation
= TRANSIENT r/ Bankartlesion= anteriortear of glenoidlabrum
LATERAL PATELLAR DISLOCATION
lncidence: 2-3% of all knee injuries (originallyonly referringto injuryof anterior
Mechanism; duringattemptto slow forwardmotion band of glenohumeral ligament)
[ArthurSydneyBlundell Bankart (1879-1951), British
whilepivotingmediallyon a plantedfoot;
orthopedicsurgeonl
internalrotationof femuron fixedtibia
whileknee is flexed+ quadriceps
hemorrhage /disruption
contractionproducesa net lateralforce of medial retinaculum \ lateralpatellar tilt
At risk: shallowtrochleargroove
Associatedwith: medialmeniscallear lmajor
l i g a m e n to uisn j u ryi n 3 1 % effusion /
hemarthrosis
Age: young physicallyactivepeople
. hemarthrosis (mostcommoncauseof hemarthrosis in
youngconscripts)
swelling+ tendernessof medialretinaculum
0 >50% not clinicallydiagnosedinitially!
increasedsignalintensity/ thickening/ disruptionof
medialpatellarretinaculum
^/ lateralpatellartilt MR Imaging Signs of Pateltar Dislocation
 Radiology Review Manual

{ fractureof anteriorrim of glenoid

^/ Hill-Sachsdefect (50%)= depression/
impactedfractureof posterolateralsurfaceof
humeralhead at / above levelof coracoid
process(dueto impactionagainstanterior

ffi ffi
edge of glenoidrim in subglenoidtype)
ArthurHill(1901-1973)
[Harold andMaurice David
Sachs(1909-1987), in SanFrancisco,
radiologists
Californial
Cx: recurrentdislocations

Posrenpn SnouuoenDlslocarton (24%) Normal Lunate Perilunate Midcarpal

Cause: (a) traumatic: convulsivedisorders/
electricshocktherapy
(b) nontraumatic:voluntary,involuntary,
congenital,developmental
Types: subacromial, subglenoid, subspinous
0 In >50% unrecognized initially+ subsequently
misdiagnosed as frozenshoulder!
0 Averageintervalbetweeninjuryand diagnosisis
1 year!
{ rim sign (66%)= distancebetweenmedialborder
of humeralhead+ anteriorglenoidrim <6 mm
May be associated with:
{ troughsign (75%) = "reverseHill-Sachs"
= colTtPf€ssionfractureof anteromedial humeral
DOWNSYNDROME
head (tangential Grasheyview of glenoid!)
r/ fractureof posteriorglenoidrim
{ avulsionfractureof lessertuberosity
lHrenpn Sxout-oEnDtslocerrcn (1-2%)
= LUXATIO ERECTA
= extremityheld over head in fixed positionwith
elbowflexed
Mechanism: severe hyperabductionof arm
resultingin impingement of humeral
head againstacromion
r/ humeralarticularsurfacefaces inferiorly
Cx: rotatorcuff tear; fractureof acromion
+ inferiorglenoidfossa+ greatertuberosity;
neurovascular injury
SupentoRSnouloeR Dtslocnlon (<1%)
= humeralhead drivenupwardthroughrotatorcuff
May be associatedwith: fractureof humerus,
clavicle,acromion
DDx: droopingshoulder(transientphenomenon
afterfractureof surgicalneck of humerus
due to hemarthrosis / muscleimbalance)
Wrist Dislocation
Mechanism; hand
fallonoutstretched
lncidence: 10/" of all carpalinjuries
Lunate Dislocation
PeriIu nateDisl ocation
2-3 timesmorecommonthan lunatedislocation
accompanied by fracturein75/" (= transscaphoid
perilunatedislocation)
{ most commonlydorsaldislocation
Dislocation Dislocation Dislocation
Rotary Subluxation of ScaPhoid
= tearingof interosseousligamentsof lunate,
scaphoid,capitate
Mechanism: acutedorsiflexionof wrist;may be
associatedwith rheumatoidarthritis
{ g"p >4 mm betweenscaphoid+ lunate(PA view)
^i foreshorteningof scaphoid
{ ringsignof distalpoleof scaphoid
Midcarpal Dislocation
= MONGOLTSM = TRTSOMY 21
5% translocation
Chromosomes:95% nondisjunction,
lncidence: 1:870liveborninfants,most common
karyotype/ chromosomalabnormalityin USA
. mentalretardation
. hypotoniain infancy
. characteristic facies
. Simiancrease
@ Skull
r/ hypotelorism
{ persistentmetopicsuture(40-79%)after age 10
! hypoplasiaof sinuses+ facialbones
{ microcrania(brachycephaly)
ri delayedclosureof sutures+ fontanels
r/ dentalabnormalities(underdeveloped tooth No. 2 )
^/ ttaubridgednose
@ Axial skeleton
r/ atlantoaxialsubluxation(25%)
r/ anteriorscallopingof vertebralbodies
{ "squaredvertebralbodies"= centrahigh and narrow
= positivelaterallumbarindex (ratioof horizontalto
verticaldiametersof L2)
@ Chest
{ congenitalheartdisease(40%): endocardial
cushiondefect,VSD, tetralogyof Fallot
./ hypersegmentation of manubrium
= 2-3 ossificationcenters (90%)
r/ gracileribs; 11 pairsof ribs (25%)
@ Pelvis(frontalview)
r/ flaringof iliacwings (= rotationof iliacwingstoward
"MickeyMouse
coronalplaneat sacroiliacjoints)=
ears"/ "elephant ears":
{ decreasediliacangle + index (in 70-80%)
./ flatteningof acetabularroof (smallacetabularangle)

r/ elongated+ taperedischia
@ Extremities
i metaphysealflaring
r/ clinodactyly(50%);widenedspace betweenfirsttwo
, digitsof hands+ feet
r/ hypoplasticand triangularmiddle+ distalphalanges
of Sthfinger= ?cforTlicria
(DDx: normalindividuals,
cretins,achondroplastic dwarfs)
{ pseudoepiphyses of 1st + 2nd metacarpals
@ Gastrointestinal
{ umbilicalhernia
r/ "doublebubble"sign (8-1O/o)= duodenalatresia/
stenosis/ annularpancreas
r/ tracheoesophageal fistula
r/ anorectalanomalies
./ Hirschsprungdisease
OB - US :
. triple-marker screening test:
. low (0.7 MoM) maternalalpha-fetoprotein (20-30%)
. increased(2.04MoM)hCG (DDx:decreasedin
trisomy18)
. decreased(0.79MoM) unconjugatedestriol(ue3)
. dimericinhibinA (= new additionalanalyte)
0 Detects60% + 75/" of all Down syndrome
pregnanciesin women <35 years + >35 years
0 Most accuratebetween16 and 18 weeks!
. advancedmaternalage
0 ln 1:385livebirthsfor women>35 yearsof age
0 HOWEVER: 80% of fetuseswith Down syndrome
are born to mothers<35 years of age
{ majorstructuralmalformations:
r/ VSO / compteteAV canat(S0%)
r/ cystichygroma,resolvedby 20th week MA
r/ omphalocele
r/ doublebubbleof duodenalatresia(8-10%),not
apparentbefore 22 weeks GA
r/ hydrothorax
{ mild cerebralventriculardilatation
^/ agenesisof corpuscallosum
, r/ imperforateanus
r/ occipital-nuchal skinthickening>5 mm during1+19
weeks/ >6 mm during 19-24 weeks
{ mild fetal pyelectasis(17-25%)
{ hyperechoicbowelat <20 weeks GA (1So/o, in 0.6/" ot
normals)
r/ intracardiacechogenicfocus,usuallyin left ventricle
= thickeningof papillarymuscle(18/", in 5% of
normals)
{ ratioof measuredto-expected femur length<0.91
[expectedfemurlength:-9.3105 + 0.9028x BpD]
(sensitivity40y", specificitygS/", false-positiverate of
2-7o/o,03% PPV for low-riskpopulation11:7001, 1"h
, PPV for high-riskpopulation[1:250])
r/ elevatedBPD / femur ratio (secondaryto shortfemur)
{ ratioof measured-to-expected humeruslength<0.g0
[expectedhumeruslength:-7.9404 + 0.8492x BpD]
(1-2% PPV for low-riskpopulation; 3% ppV for high-
risk population)
! sandal-gapdeformity= ssprrationof greattoe (45%)
Boneand Soft-TissueDisorders 69
ri hypoplasiaof middlephalanxof Sthdigitresultingin
clinodactyly(= inwardcurve)in 60%
r/ flaredilium= iliacwings rotatedtowardcoronalplane:
r/ meaniliacangleat superiormost levelof 95.6t
11.7" (comparedto 76.4+ 16.8.for euploidfetuses)
r/ brachycephaly
r/ smallcerebellum
^/ luon (in3o%)
{ polyhydramnios
Cx: leukemia(increasedfrequency3-20 x)
DYSCHONDROSTEOSIS
= LERI-LAYANI-WEILL SYNDROME
= ffiesorneliclong-boneshortening(forearm+ leg);
autosomaldominant
M : F= 1 : 4
. limitedmotionof elbow+ wrist
./ bilateralMadelungdeformity:
r/ radialshorteningin relationto ulna
^/ bowingof radiuslaterally+ dorsally
r/ dorsalsubluxationof distalend of ulna
r/ carpalwedgingbetweenradius+ ulna (due to
triangularshapeof distalradialepiphysis+
underdevelopment of ulna)
DDx: pseudo-Madelung deformity(fromtrauma/
infection)
DYSPLASIA EPIPHYSEALIS HEMIMELICA
= TREVOR DISEASE = TARSOEPIPHYSEAL ACLASIS
= ufrcorTrrTronskeletaldevelopmentaldisorder
representingan epiphysealosteochondroma
lncidence; 1:1,000,000
Age: 24years: M;F = 3:1
Cause: failureof normalprogressionof cellularcartilage
breakdown(= aclasis);spontaneousoccurrence
Path: lobulatedmass protrudingfrom epiphysiswith a
cartilaginous cap
Histo: normalbone + hyalinecartilagewith abundant
enchondralossification (= abnormalcellular
activityat cartilaginousossificationcenter)
Forms:
(1) Localizedform = rTtor'tostoticinvolvement:usually
hindfootand ankle
(2) Cfassicform (>66Yo)= morethan one area of
involvementin a singleextremitywith characteristic
hemimelicdistribution:talus,distalfemur,distaltibia
(3) Generalizedlsevere form = diseaseinvolvingthe
whole lowerextremity
r/ pelvicinvolvement:femoralhead,symphysis
pubis,triradiatecartilage
{ hypertrophyof ipsilateraliliac bone
. antalgic(= pain-avoiding)
gait;palpablemass
. varus/ valgusdeformity;limb lengthdiscrepancy
. limitedjointmobilityand function
Location:lowerextremity(tarsus,knee,ankle);rare in
upperextremity(humerus,ulna,scapula)
Site: restrictedto medialOR lateralside of limb
(= hemimelic), = 2:1
ie, medial:lateral
@ lnfant& toddler
{ prematureappearanceof an eccentric,lobulated,
overgrown,asymmetricossificationcenter
r/ stippledcalcificationof anomalouscartilage
@ Childhood
./ disorganizedepiphysealcalcificationaccompanied
by irregularossification
{ osteochondroma-like growthfrom one side of
epiphysis
r/ prematureclosureof physisresultsin limb deformity
and limb lengthdiscrepancy
r/ irregulararticularsurfacecombinedwith angular
deformity
r/ undertubulation of bone as a consequenceof
secondaryinvolvementof metaphysis
Cx: prematuresecondaryosteoarthritis
DDx: osteochondroma
OF BONE
ECHINOCOCCUS
Occursoccasionally in the USA;usuallyin foreign-born
individuals; bone involvement in 1%
Histo: no connectivetissuebarrier;daughtercysts
extenddirectlyinto bone
@ Pelvis,sacrum,rarelylongtubularbones
r/ round/ irregularregionsof rarefaction
r/ multiloculated lesion(bunchof grapes)
./ no sharpdemarcation(DDx:chondroma,giantcell
tumor)with secondaryinfection:
r/ thickeningof trabeculaewith generalizedperifocal
condensation
r/ corticalbreakthroughwith soft-tissuemass
@ Vertebra
r/ sclerosiswithoutpathologicfracture
^/ intervertebraldisks not affected
{ vertebrallaminaoften involved
r/ frequentlyinvolvementof adjacentribs
SYNDROME
EHLERS-DANLOS
= group of autosomaldominantdiseasesof connective
tissuecharacterizedby abnormalcollagensynthesis
Types: 10 types have been describedthat differ
clinically,biochemically,and genetically
Age: presentat birth; predominantly in males
. hyperelasticity of skin
. fragilebrittleskinwith gapingwoundsand poor healing
. molluscoidpseudotumorsover pressurepoints
. hyperextensibility of joints
. joint contractureswith advancedage
. bleedingtendency(fragilityof bloodvessels)
. blue sclera,microcornea,myopia,keratoconus,ectopia
lentis
@ Soft tissues
{ muftipleovoidcalcifications (2-10 mm) in subcutis/
in fatty cysts ("spheroids"),
most frequentlyin
periarticularareas of legs
r/ ectopicbone formation
@ Skeleton
i/ hemarthrosis(particularlyin knee)
{ malalignment of jointson
/ dislocation
/ subluxation
stress radiographs
{ recurrentdislocations (hip,patella,shoulder,radius,
clavicle)
r/ precociousosteoarthrosis(predominantly in knees)
^/ ulnarsynostosis
r/ kyphoscoliosis
{ spondylolisthesis
! spinabifidaocculta
@ Chest
r/ diaphragmatic hernia
r/ panacinaremphysema+ bullaformation
{ tracheobronchomegaly + bronchiectasis
@ Arteries
r/ aneurysmof greatvessels,aorticdissection,
tortuosityof arch,ectasiaof pulmonaryarteries
O AORTOGRAPHY CONTRAINDICATED!
(Cx followingarteriography:aorticrupture,
hematomas)
@ Gl tract
r/ ectasiaof gastrointestinaltract
ELASTOFIBROMA
= benigntumorlikelesionformingas a reactionto
mechanicalfriction
lncidence: in 24/" of women + 11o/o of men >55 years
(autopsystudY)
A ge: el derl y;M:F = 1:2
Histo: enlargedirregularserratedelastic
hypereosinophilic f ibers, collagen,scattered
fibroblasts,occasionallobulesof adiposetissue
. asymptomatic
. may remainclinicallyinapparent
Location: betweeninferiormarginof scapula+ posterior
chestwall; bilateralin 25/"
{ inhomogeneouspoorlydefinedlesionof soft-tissue
attenuation similarto muscle
r/ well-defined intermediate-signal intensitylesionwith
interlacedareas of fat-intensitysignalon Tl Wl + T2Wl
ENCHONDROMA
= benigncartilaginous growthin medullarycavity;bones
preformedin cartilageare affected(NOT skull)
lncidence: 3-17"/" of biopsiedprimarybone tumors
0 Secondmost commoncartilage-containing
tumor!
Etiotogy: continuedgrowthof residualbenignrestsof
cartilagedisplacedfrom the growthplate
Age: 10-30 years; M:F = 1:1
Histo: lobulesof pure hyalinecartilage
. usuallyasymptomatic, painlessswelling
Location: (usuallysolitary; multiple= erlchondromatosis)
(a) in 40% smalltubularbonesof wrists+
hand (mostfrequenttumorhere),distal+
mid aspectsof metacarpals,proximal/
middlephalanges
(b) proximalfemur,proximalhumerus,tibia,
radius,ulna,foot,rib (3%)
Site: centralwithinmedullarycanal + metaphyseal;
epiphysisonly affectedafterclosureof growthplate
r/ oval / roundarea of geographicdestructionwith
lobulatedcontour+ fine marginalline
 Boneand Soft-TissueDisorders 71

r/ corticalendostealscalloping r/ associatedwith dwarfingof the involvedbone due to

r/ ground-glassappearance
r/ dystrophiccalcifications withinsmallcartilagenodules/
fragmentsof lamellarbone: pinhead,stippled,
flocculent,"ringsand arcs" pattern
r/ bulbousexpansionof bone with thinningof cortexin
smalltubularbonesof phalanx,rib,fibula
./ Madelungdeformity= bowingdeformities of limb,
discrepantlength
{ NO corticalbreakthrough/ periostealreaction
MR:
^/ low-to intermediate-signal intensityon Tl Wl + high-
signalintensityon T2Wl
r/ low-signalintensitymatrixcalcifications
r/ normalfat marrowinterspersedbetweencartilage
nodules
i/ peripheralenhancementpattern
Cx: (1) Pathologicfracture
(2) Malignantdegeneration in long-bone
enchondromas in 1S-20%(graduallyincreasing
pain in an adult patient)
DDx: (1) Epidermoidinclusioncyst (phalangeal tuft,
historyof trauma,more lucent)
(2) Unicameralbonecyst (rarein hands,more
radiolucent)
(3) Giantcelltumorof tendonsheath(commonly
erodesbone,soft-tissuemass outsidebone)
(4) Fibrousdysplasia(rarein hand, mostly
polyostotic)
(5) Bone infarct
(6) Chondrosarcoma (exceedingly rare in
phalanges,metacarpals,metatarsals)
ENCHONDROMATOSIS
= OLLIER DISEASE= DYSCHONDROPLASIA = Mg111P1'
ENCHONDROMATOSIS
= nonhereditaryfailureof cartilageossification
Cause: derangement of cartilaginousgrowthresultingin
migrationof cartilaginousrestsfrom epiphyseal
plate into metaphysiswhere they proliferate
Age: early childhoodpresentation
Association: juvenilegranulosacell tumor of ovary
. growthdisparitywith leg I arm shortening
. hand + feet deformity
Location: predominantlyunilateral monomelic
distribution(a) locatized(b) regional
(c) generalized
r/ well-demarcated roundedradiolucencies / columnar
streaksof decreaseddensityfrom epiphysealplate into
, diaphysisof long bones= cartilaginous rests
! expansileremodelingof affectedbone:
r/ clublikedeformity/ expansionof metaphysealregion
! preOominant thinningof cortex+ endostealscalloping
./ bony spurs pointingioward the joint (DDx: exostosis
pointsaway from it)
r/ cartilaginousareasshow punctatecalcifications with
age:
r/ matrixmineralization with Typ|CAL arc-and-ring
appearanceof chondroidlesions
impairmentof epiphysealfusion
r/ bowingdeformitiesof limb bones
r/ discrepancyin length= Madelungdeformity(radius,
ul na)
r/ small bones of feet + hands: aggressivedeforming
tumorsthat may breakthroughcortexsecondaryto
tendencyto continueto proliferate
r/ fanlikeradiationof cartilagefrom centerto crest of ilium
Cx: sarcomatoustransformation(in 25-30%):
osteosarcoma(youngadults);chondro-/
fibrosarcoma(in older patients)
Maffuci Syndrome
= nonhereditarymesodermaldysplasiacharacterizedby
enchondromatosis + multiplesoft-tissue
cavernous
hemangiomas + lesscommonlylymphangiomas
Age: 25/" during 1st year of lite; 45h prior to 6 years;
78/"betore puberty;M > F
Association: juvenilegranulosacell tumor of ovary
. multiplebluesubcutaneous nodulesparticularlyon
digits+ extremities(cavernoushemangiomas)
. normalintelligence
Location: unilateralinvolvement(50%)/ marked
asymmetry;distinctpredilection for tubular
bonesof hands+ feet
r/ phlebolithsmay be present
{ strikingtendencyfor enchondromatato be very large
projectinginto soft tissues
{ growthdisturbanceof long bones (common)
Cx: (a) malignanttransformationof
(1) Enchondroma to chondrosarcoma /
fibrosarcoma(15-20%)
(2) Cavernoushemangiomato hemangio-
sarcoma/ hemangioendothelioma /
lymphangiosarcoma (in 3-5%)
(b) increasedprevalenceof ovariancarcinoma,
pancreaticcarcinoma,CNS glioma,
gastrointestinal adenocarcinoma
Prevalenceof malignancy: Zg-100%
DDx: Ollierdisease(withouthemangiomas)
ENGELMANN.CAMURATI
DISEASE
= PROGRESSIVE DIAPHYSEAL DYSPLASIA = CAMURATI-
ENGELMANN DISEASE
Cause: autosomaldominant;disturbancein
intramembranous boneformation+ modeling
(as occursin cortexof long bones,calvaria,
mandible,facialbones,midsegmentof clavicle)
Age: 5-25 years (primarilyin childhood);M > F
' neuromusculardystrophy= delayedwalking(18-24
months)with wide-basedwaddlinggait; often
misdiagnosedas musculardystrophy/ poliomyelitis
. weakness+ easy fatigabilityin legs
. bonepain+ tenderness usuallyin midshaftof longbones
. underdevelopment of musclessecondaryto malnutrition
. NORMALlaboratoryvalues
Location: usuallysymmetrical;NO involvement of
hands,feet,ribs,scapulae
@ Skull(initiallyaffected)
r/ amorphousincreasein densityat base of skull
r/ encroachment of frontal+ sphenoidsinus;sparingof
maxillarysinus
@ Longbones(bilateralsymmetrical distribution)
Site: tibia> femur> fibula> humerus> ulna> radius
r/ fusiformenlargementof diaphyseswith cortical
thickening(endosteal+ periostealaccretionof
mottlednew bone)and progressiveobliterationof
medullarycavity
r/ progressionof lesionsalong long axis of bone
towardeitherend
r/ abruptdemarcationof lesions(metaphyses+
epiphysesspared)
r/ relativeelongationof extremities
DDx: (1) Chronicosteomyelitis (singlebone)
(2) Hyperphosphatasemia (highalkaline
phosphataselevels)
(3) Pagetdisease(age,new-boneformation,
increasedalkalinephosphatase)
(4) Infantilecorticalhyperostosis(fever;mandible,
rib, clavicles;regresses,<1 year of age)
(5) Fibrousdysplasia(predominantly unilateral,
subperiostealnew bone)
(6) Osteopetrosis(very littlebony enlargement)
(7) VitaminA poisoning
CYST
EPIDERMOID
= INFUNDIBULAR CYST
= proliferationof surfaceepidermalcellswithinthe dermis
Histo: productionof keratinwithinclosedspace linedby
surfaceepidermis
Associatedwith: nevoidbasalcell syndrome(Gorlin
syndrome)patientshave a high
prevalenceof epidermoid/ dermoid
cysts
r/ isointense/ slightlyhyperintenserelativeto muscleon
TlWI
r/ hyperintensewith focal areas of decreasedsignalon
T2WI
EpidermoidInclusionCyst
= INTRAOSSEOUS KERATIN CYST= IMPLANTATION CYST
Age: 2nd-4th decade; M > F
Histo: stratifiedsquamousepithelium,keratin,
cholesterolcrystals(softwhite cheesycontents)
. historyof trauma(implantation of epitheliumunder
skin with secondarybone erosion)
. asymptomatic
Location: superficiallysituatedbonessuch as
calvarium(typicallyin frontal/ parietal
bone),phalanx(usuallyterminaltuft of
middlefinger),L > R hand,occasionally
foot
r/ well-definedroundosteolysiswith scleroticmargin
{ cortexfrequentlyexpanded+ thinned
{ NO calcifications / periostealreaction/ soft-tissue
swelling
r/ pathologicfractureoftenwithoutperiostealreaction
DDx: (a) in finger: glomustumor,enchondroma(rare
in terminalPhalanx)
(b) in skull: infection,metastasis(poorlydefined),
eosinophilic granuloma(beveledmargin)
EPIPHYSEOLYSIS OF FEMORAL HEAD
= SLIPPED CAPITAL FEMORAL EPIPHYSIS
= atraumaticfracturethroughhypertrophiczone of
physealplate
Frequency: 2:100,000PeoPle
Etiotogy: growth spurt, renal osteodystrophy,rickets,
childhoodirradiation, growthhormonetherapy,
trauma (Salter-Harris type I epiphysealinjury)
Pathogenesis; widening of physeal plateduringgrowth
spurt + changein orientationof physis
from horizontalto obliqueincreasesshear
forces
Age: overweight8-17 year old boys (meanage for boys
13,for gi rl s11 years);M:F = 3:1; bl ack> whit e
Associated with:
(a) malnutrition, endocrineabnormality, developmental
dysplasiaof hip (duringadolescence)
(b) delayedskeletalmaturation(afteradolescence)
. hip pain (50%)/ knee pain (25'h) for 2-3 weeks
Location: usuallyunilateral;bilateralin 20-37"/"(at initial
presentation in 9-18%)
{ wideningof epiphysealgrowthplate(preslipphase):
{ irregularity + blurringof physealphysis
r/ demineralization of neckmetaphysis
{ posteromedial displacement of head (acuteslip):
{ decrease in neck-shaft angle with alignmentchange
in the growthplateto a more verticalorientation
r/ lineof Klein(= linedrawnalongsuperioredgeof
femoralneck)failsto intersectthe femoralhead
r/ epiphysisappearssmallerdue to posteriorslippage:
early slips are best seen on cross-tableLAT view
CAVE: positioning intoa froglegview may cause
furtherdisplacement
{ sclerosis+ irregularity of widenedphysis(chronicslip):
r/ metaphysealblanchsign = area of increasedopacity
in proximalpart of metaphysis(healingresponse)
Grading (based on femoral head position):
mild = displacedby <1/3of metaphysealdiameter
moderate= displacedby 113-2/3of diameter
severe = displacedby>213of metaphysealdiameter
in
Line of Klein
in Normal Hip

Cx: (1) Chondrolysis= ocute cartilagenecrosis(7-10%)
= rapid loss of >50y" of thicknessof cartilage
r/ joint space <3 mm
(2) Avascularnecrosisof femoralhead (10-15%):
risk increaseswith advanceddegreeof slip,
delayedsurgeryfor acuteslip, anteriorpin
placement,largenumberof fixationpins,
subcapitalosteotomy
(3) Pistol-grip deformity= Spes6ening + shortening
of femoralneck in varus deformity
(4) Degenerativeosteoarthritis(90%)
(5) Limb-lengthdiscrepancydue to premature
physealclosure
Rx: (1) limitationof activity,(2) prophylactic
pinning
(3) osteotomy
Attemptedreductionsincreaserisk of AVNI
ESSENTIAL OSTEOLYSIS
= progressiveslow bone-resorptive disease
Histo: proliferation+ hyperplasiaof smoothmusclecells
of synovialarterioles
{ progressiveosteolysisof carpal+ tarsalbones
^/ thinnedpointedproximalends of metacarpals+
metatarsals
r/ elbowsshow same type of destruction
r/ bathyrocephalic depressionof base of skull
DDx: (1) Massiveosteolysis= Gorhamdisease(local
destruction of contiguousbones,usuallynot
affectinghands/ feet)
(9) Mutilatingformsof rheumatoidafihritis
(2) Tabes dorsalis
(3) Leprosy
(4) Syringomyelia
(5) Scleroderma
(6) Raynauddisease
(7) Regionalposttraumaticosteolysis
(8) Ulcero-mutilatingacropathy
(9) Mutilatingforms of rheumatoidarthritis
(10) Acrodyniamutilante(nonhereditary)
EWINGSARCOMA
= EWING TUMOR
lncidence: 4-10% of all bone tumors (lesscommonthan
osteo-/ chondrosarcoma); mostcommon
malignantbonetumorin children;4th most
commonbonetumoroverall
0 Clinically,radiologically,
and histologically
very similarto
PNETI
Histo:
smallroundcells,uniformlysized+ solidlypacked(DDx:
lymphoma,osteosarcoma, myeloma,neuroblastoma,
carcinoma,eosinophilic granuloma)invadingmedullary
cavityand enteringsubperiosteumvia Haversiancanals
producingperiostitis,soft-tissuemass,osteolysis;
glycogengranulespresent(DDxto reticulumcell
sarcoma);absenceof alkalinephosphatase(DDx to
osteosarcoma); MlC2 cell surfaceimmunoreactivity (in
1 00%)
Boneand Soft-Tissue
Disorders 73
Age: peak 15 years (range5 monthsto 54 years);in
95% 4-25 years;in30/o <10 years;in 39% 11-1S
years;in 31"/">15 years;in50Y"<20 years;
M:F = 1:2; Caucasians in 96%
. severelocalizedpain
. soft-tissuemass
. fever,leukocytosis,anemia(in early metastases)
simulatinginfection
Location:
femur(25% ),pel vi s-i l i um (14% ),ti bi a(11Y o),
hum er us
(10% ),fi bul a(8% ),ri bs(6-10% )
(a) longbonesin 60%:
metadiaphysis(44%),middiaphysis(39%),
metaphysis(15%), metaepiphyseal (6%),epiphyseal
(2%);usuallyno involvement of epiphysisas tumor
originatesin medullarycavitywith invasionof
Haversiansystem
(b) flat bonesin 40h: pelvis,scapula,skull,vertebrae
(in 3-107o;sacrum> lumbar> thoracic> cervical
spi ne);ri bs(i n 7" /o>age 10; i n 30% < age 1 0)
0 >20 years of age predominantlyin flat bones
0 <20 years of age predominantlyin cylindrical
bones (tumorderivedfrom red marrow)
,
! 8-10 cm long lytic lesionin shaft of long bone (62%
lylic,23/" mixeddensity,15% dense)
{ mottled"moth-eaten"destructivepermeativelesion
(72%)(latefinding)
{ penetrationinto soft tissue(55%)with preservationof
tissueplanes(DDx: osteomyelitiswith diffusesoft-
, tissueswelling)
r/ earlyfusiformlamellated"onionskin"periostealreaction
(53%)/ spiculated= "sunburst"/ "hair-on-end"(23h),
Codmantriangle
{ corticalthickening(16%)
{ corticaldestruction(18%)
r/ t corticalsequestration
tr reactivescleroticnew bone (30%)
./ boneexpansion(12%)
@ Ewingsarcomaof rib:
r/ primarilylytic/ sclerotic/ mixtureof lysis + sclerosis
r/ disproportionately large inhomogeneoussofttissue
MASS
r/ largeintrathoracic + minimalextrathoracic component
r/ may spreadinto spinalcanalvia intervertebral
foramen
Metastasesto: lung,bones,regionallymphnodesin
11-30% at time of diagnosis,
in 40-45'/. within 2years of diagnosis
Cx: pathologicfracture(5-14%)
Prognosis: 60-75o/"S-yearsurvival
DDx: (1) Multiplemyeloma(olderage group)
(2) Osteomyelitis(durationof pain <2 weeks)
(3) Eosinophilicgranuloma(solidperiosteal reaction)
(4) Osteosarcoma(ossificationin soft tissue,near
age 20, no lamellarperiostealreaction)
(5) Reticulumcell sarcoma(clinically healthy,
between30 and 50 years,no glycogen)
(6) Neuroblastoma(< age 5 )
(7) Anaplasticmetastaticcarcinoma(>30 years of
age)
 Radiology Review Manual

(8) Osteosarcoma { severeosteoporosis+ fracturesat multiplesites (esp.of

(9) Hodgkindisease spine)
{ protrusioacetabuli
EXTRAMEDU LLARY HEMATOPOIESIS
= colT'tP€I'tsatoryresponseto deficientbone marrowblood
cell production FA N C ON I A N E MIA
Etiology: prolongederythrocytedeficiencydue to = autosomalrecessivediseasewith severehypoplastic
(1) destruction of RBC: anemia+ skin pigmentation + skeletaland urogenital
congenitalhemolyticanemia( thalassemia, anomalies
sicklecell anemia), . skin pigmentation (melanindeposits)in 74% (trunk,
hereditaryspherocytosis,
acquiredhemolyticanemia,idiopathicsevere axi l l a,groi n,neck)
fetalis . anemiaonset between17 months and22 years of age
anemia,erythroblastosis
(2) inabilityof normalblood-forming organsto produce . bleedingtendency(pancytopenia)
. hypogonadism(40%)
erythrocytes:
irondeficiencyanemia,perniciousanemia, . microphthalmia(20%)
myelofibrosis, myelosclerosis, polycythemia vera, { anomaliesof radialcomponentof upperextremity
carcinomatous / lymphomatous replacement of (strongly suggestive) :
bone marrow(chronicmyelogenous leukemia, { absent/ hypoplastic / supernumerary thumb
Hodgkindisease) r/ hypoplastic/ absentradius
0 NO hematologic diseasein 25z r/ absenti hypoplasticnavicularI grealermultangular
. absenceof pain,bone erosion,calcification bone
. chronicanemia r/ slighti moderatedwarfism
Sites: in areas of fetal erythropoiesis { minimalmicrocephaly
@ spleen r/ renalanomalies(30%):renalaplasia,ectopia,
r/ splenomegaly horseshoekidney
r/ focal isodensemasseson enhancedCT Prognosis; fatal within5 years afteronset of anemia;
patient'sfamilyshowshighincidenceof
@ liver,lymphnodes
@ thorax: mediastinum, heart,thymus,lung leukemia
{ uni-/ bilateralsmoothlobulatedparaspinalmasses
betweenT8 and T12
r/ anteriorrib ends expandedby masses FA R B E R D IS E A S E
@ S pine = DISSEMINATED LIPOGRANULOMATOSIS
0 Mostcommonlyafflictedin thalassemia Histo: foam cell granulomas; lipidstorageof neuronal
. back pain,symptomsof spinalcord compression tissue(accumulation of ceramide+ gangliosides)
. hoarseweak cry
rl coarsenedtrabeculation
r/ extramedullaryhematopoiesisin epiduralspace . swellingof extremities; generalized jointswelling
. subcutaneous granulomas
+ periarticular
@ adrenalglands
. intermittent fever,dYsPnea
@ renalpelvis
. lymphadenopathy
@ gastrointestinal lymphatics
@ dura mater (falxcerebriand over brainconvexity) r/ capsulardistensionof multiplejoints(hand,elbow,
@ cartilage,broad ligaments knee)
@ thrombi,adiposetissue
ri juxtaarticular bone erosionsfrom soft-tissuegranulomas
r/ lack of calcification/ bone erosion { subluxation / dislocation
r/ disuse/ steroiddeossification
Prognosis; death from respiratoryfailurewithin2 years
FAMILIAL IDIOPATHIC ACROOSTEOLYSIS
= HAJDU-CHENEY SYNDROME
= fore bizarreentityof unknownetiology FIB R OC H ON D R OGE N E S IS
Location: may be unilateral = autosomalrecessivelethalshort-limbskeletaldysplasia
. fingernailsremainintact lncidence: 5 cases
. sensorychanges+ plantarulcersrare { severemicromelia+ broaddumbbell-shaped
r/ pseudoclubbing of fingers+ toes with osteolysisof metaphyses
terminal+ moreproximalphalanges ^/ ttat + cleftedpear-shapedvertebralbodies
r/ genuvarum/ valgum r/ short + cuppedribs
r/ hypoplasiaof proximalend of radius r/ frontalbossing
{ subluxationof radialhead ./ low-setabnormallyformedears
r/ scaphocephaly, basilarimpression Prognosis; stillbirthi death shortlyafter birth
r/ wide sutures,persistentmetopicsuture,Wormian DDx: (1) Thanatophoric dysPlasia
bones,poorlydevelopedsinuses (2) Metatropicdysplasia
r/ kyphoscoliosis (3) Spondyloepiphysealdysplasia
 Boneand Soft-TissueDisorders 75

FIBRODYSPLASIA
OSSIFICANS
PROGRESSIVA MR:
= MYOSITIS OSSTFTCANS pROGRESSTVA (misnomersince r/ smallhypointense
noduleon all pulsesequences
primarily
connectivetissuesareaffected)
= rdfe slowlyprogressivesporadic/ autosomaldominant
FIBROSARCOMA
diseasewith variablepenetrancecharacterizedby
remissions+ exacerbationsof fibroblasticproliferation, lncidence: 4./" of all primary bone neoplasm
subsequentcalcification+ ossificationof subcutaneous Etiology:
fat, skeletalmuscle,tendons,aponeuroses,ligaments A. PRTMARY FTBROSARCOMA (70%)
Histo: edemawith proliferatingfibroblastsin a loose B. SECONDARYFTBROSARCOMA (30%)
1. followingradiotherapy of giantcell tumor/
myxoidmatrix;subsequentcollagendeposition
plus calcification+ ossificationof collagenized lymphoma/ breastcancer
fibroustissuein the centerof nodules 2. underlyingbenignlesion: pagetdisease
Age: presentingby age 2 years (50%) (common);giant cell tumor,bone infarct,
. initiallysubcutaneous painfulmasseson neck, osteomyelitis, desmoplastic fibroma,
shoulders,upperextremities enchondroma, fibrousdysplasia(rare)
. progressiveinvolvementof remainingmusculatureof 3. dedifferentiation of low-gradechondrosarcoma
Histo: spectrumof well to poorlydifferentiated fibrous
back,chest,abdomen,lower extremities
. lesionsmay ulcerateand bleed tissueproliferation; will not produceosteoid/
. musclesof back + proximalextremitiesbecomerigid chondroid/ osseousmatrix
Age: predominantlyin 3rd-Sthdecade(rangeof g-gg
followedby thoracickyphosis
. inanitionsecondaryto jaw trismus(masseter,temporal y e a r s ) ;M : F= 1 : 1
Metastasesto: lung, lymphnodes
muscle) . localizedpainfulmass
. "wry neck"= torticollis(due to restrictionof
Location: tubularbonesin young,flat bonesin older
sternocleidomastoid muscle)
. respiratoryfailure(thoracicmusclesaffected) patients;femur(40/"),tibia(16%)(aboutknee
. conductivehearingloss (fusionof middleear ossicles) in 30-50%),jaw, pelvis(g%);rare in small
bonesof hand+ feet or spinalcolumn
Site: eccentricat diaphyseal-metaphyseal junctioninto
A. ECTOPICOSSIFICATION
metaphysis;intramedullary / periosteal
r/ rounded/ linearcalcification in neck/ shoulders,
paravertebralregion,hips,proximalextremity,trunk, A. CENTRALFIBROSARCOMA
= intramedullary
palmar+ plantarfasciaformingossifiedbars + bony
r/ well-defined lucentbone lesion
bridges
r/ ttrinexpandedcortex
r/ ossificationof voluntarymuscles,completeby 20-25
years (sparingof sphincters+ head) ri aggressiveosteolysiswith geographicI raggedI
permeativebone destruction+ wide zone of transition
B. SKELETALANOMALIES
{ occasionallylargeosteolyticlesionwith cortical
may appearbeforeectopicossification
. clinodactyly destruction,periostealreaction+ soft-tissueinvasion
r/ microdactylyof big toes (90%)and thumbs (S0%) r/ sequestrationof bone may be present
= usuallyonly one largephalanxpresent/ synostosis (DDx: eosinophilic granuloma,bacterialgranuloma)
{ sparseperiostealproliferation luncommon;
of metacarpal+ proximalphalanx(firstsign)
^/ intramedullary discontinuous spread
r/ phalangealshorteningof hand + foot (miJdfe
phalanxof Sthdigit) { no calcification
^/ shortened1st metatarsal+ halluxvalgus(TS%) DDx: malignantfibroushistiocytoma,myeloma,
telangiectaticosteosarcoma,lymphoma,
r/ shortenedmetacarpals+ metatarsals
desmoplasticfibroma,osteolyticmetastasis
{ shallowacetabulum
./ shortwidenedfemoralneck B. PERIOSTEALFIBROSARCOMA
= r€lrstumor arisingfrom periostealconnectivetissue
r/ thickeningof medialcortexof tibia
Location: long bonesof lowerextremity,jaw
! progressivefusionof posteriorarchesof cervical
{ contourirregularityof corticalborder
spine
^/ narrowedAP diameterof cervical+ lumbarvertebral r/ periostealreactionwith perpendicularbone
formationmay be present
bodies
r/ rarelyextensioninto medullarycavity
{ + bony ankylosis
Cx: pathologicfracture(uncommon)
CAVE: surgeryis hazardouscausingaccelerated
Prognosis: 20/" 1O-yearsurvival
ossificationat the surgicalsite
DDx: (1) Osteolyticosteosarcoma(2nd-3rd decade)
(2) Chondrosarcoma (usuallycontains
FIBROMA OF SOFT TISSUE
characteristiccalcifications)
Histo: hypocellularhighlycollagenictumor (3) Aneurysmalbone cyst (eccentricblown-out
Age: 3rd and 4th decades; M > F
appearancewith rapidprogression)
Location: tendonsheathof distalupperextremity
(4) Malignantgiantcelltumor(beginsin metaphysis
r/ slowlygrowinglesion1-5 cm in size
extendingtowardjoint)
FIBROUS DEFECT
CORTICAL
Incidence: 30/. of children;M:F = 2:1
Age: peak age of 7-8 years (rangeof 2-10 years);
mostlybeforeepiphysealclosure
Histo: fibroustissuefrom periosteuminvadingunderlying
cortex
. asymptomatic
Location: metaphysealcortexof long bone; posterior
medialaspectof distalfemur,proximaltibia,
proximalfemur,proximalhumerus,ribs,ilium,
fibula
r/ roundwhen small,averagediameterof 1-2 cm
^/ oval,extendingparallelto long axis of host bone
r/ corticalthinning+ expansionmay occur
r/ smooth,well-defined/ scallopedmargins
{ largerlesionsare multilocular
r/ involutionover 24 years
Prognosis:
(a) potentialto grow and encroachon the medullary
cavityleadingto nonossifyingfibroma
(b) bone islandsin the adultmay be residueof
incompletelyinvolutedcorticaldefect
FIBROUS DYSPLASIA
= LICHTENSTEIN-JAFFE DISEASE
= benignfibroosseousdevelopmentalanomalyof the
mesenchymalprecursorof bone, manifestedas a defect
in osteoblasticdifferentiation and maturation
Cause: probablegene mutationduringembryogenesis
Age: 1st-2nd decade(highestincidencebetween3 and
15 years),75/"betore age 30; progressesuntil
growthceases;M:F = 1:1
Histo: medullarycavityreplacedby immaturematrixof
collagenwith smallirregularly shapedtrabeculae
of immature"woven"bone + inadequate
mineralization; neverreplacedby maturelamellar
bone
Types:
A. MONOSTOTICFORM (70-80%)
. usuallyasymptomaticuntil2nd-3rd decade
Location: ribs (28%),proximalfemur (23'/"),
craniofacialbones (10-25%)
B. POLYOSTOTICFORM (20-30%)
Age: mean age of 8 years
. 213symptomaticby age 10
. leg pain, limp,pathologicfracture(75%)
. abnormalvaginalbleeding(25%)
Location: unilateral+ asymmetric; femur(91%),
tibia(81%), pelvis(78%),foot (73/"),
ribs,skull+ facialbones(50%),upper
extremities,lumbarspine(14"/"),clavicle
(10%),cervicalspine(7%)
Site: metadiaPhysis
r/ teg lengthdiscrepancy(70%)
r/ "shepherd'scrook"deformity(35%)
r/ facialasymmetry
./ tioiatbowing
{ rib deformity
C. CRANIOFACIAL FORM= LEONTIASISOSSEA
lncidence; in 10-25% of monostoticform I in 50'/"
of polyostoticform I isolated
. cranialasymmetry
. facialdeformity
. exophthalmos
. visualimpairment
Location: sphenoid,frontal,maxillary,ethmoid
bones> occiPital, temPoralbones
i/ unilateralovergrowthof facialbones + calvarium
(NO extracranial lesions)
r/ outwardexpansionof outertable maintaining
convexity(DDx: Pagetdiseasewith destructionof
inner+ outertable)
r/ prominenceof externaloccipitalprotuberance
Cx: neurologicdeficitsecondaryto narrowed
cranialforamina(eg,blindness)
D. CHERUBISM(specialvariant)
= FAMILIALFIBROUSDYSPLASIA
= autosomaldominantdisorderof variablepenetrance
Age: childhood;moreseverein males
. bilateraljaw fullness+ slightupwardturningof eyes
./ bilateralexpansilemultiloculated cysticmasses
with symmetricinvolvementof mandible+ maxilla
Cx: problemswith dentitionafter perforationof
cortex
Prognosisi regressionafter adolescence
May be associated with:
(a) endocrinedisorders:
- precociouspubertyin girls
- hyperthyroidism
- hyperparathyroidism: renalstones,calcinosis
- acromegaly
- diabetesmellitus
- Cushingsyndrome:osteoporosis, acne
- growth retardation
(b) soft-tissuemyxoma(rare)= Mazabraudsyndrome:
lesionsin vicinityof
typicallymultipleintramuscular
most severelyaffectedbone
V A R IA N T: McOune-A l bri ghtsyndrome( 10%)
(1) polyostotic
unilateralfibrousdysplasia
(2) "coastof Maine"caf6-au-laitspots (35%)
(3) endocrinedysfunction:peripheralsexualprecocity
(menarchein infancy120%l),hyperthyroidism
.swelling+ tenderness
.limp,pain (t pathologicfracture)
.increasedalkalinephosPhatase
.advancedskeletal+ somaticmaturation(early)
.coast of Mainecaf6-au-laitspots = yellowishto brownish
patchesof cutaneouspigmentationwith irregular/
serratedborder,predominantly on backof trunk(30-
5O/o),buttocks,neck,shoulders;oftenipsilateralto
"coastof California"spotsof
bone lesions(DDx:
neurofibromatosis)
Commonlocation:
rib cage (30%),craniofacialbones[calvarium,mandible]
(25%),femoralneck + tibia (25%),pelvis
 Boneand Soft-TissueDisorders 77

Site: metaphysisis primarysite with extensioninto r/ pseudarthrosisin infancy= osteofibrousdysplasia

diaphysis(rarelyentirelength) (DDx: neurofibromatosis)
{ normalbone architecturealtered+ remodeled r/ prematureonset of arthritis
{ lesionsin medullarycavity:radiolucent / "ground-glass" Cx: (1) Transformationinto osteo-/ chondro-/
appearance/ increaseddensity fibrosarcomaor malignantfibroushistiocytoma
{ trabeculatedappearancedue to reinforced (0.5-1o/o,moreoften in polyostoticform)
subperiostealbone ridgesin wall of lesion . increasingpain
{ expansionof bones(ribs,skull,longbones) r/ enlargingsofttissue mass
{ well-definedscleroticmarginof reactivebone = rind r/ previouslymineralized lesionturnslytic
^/ endostealscallopingwith thinned/ lost cortex(ribs,long (2) Pathologicfractures:transformationof woven
bones)and interveningnormalcortexis HALLMARK intolamellarbone may be seen,subperiosteal
{ lesionmay undergocalcification+ enchondralbone healingwithoutendostealhealing
formation= fibrocartilaginous dysplasia DDx:
{ increasedactivityon bone scan duringearly perfusion+ (1) HPT (chemicalchanges,generalized deossification,
on delayedimages subperiostealresorption)
MR: (2) Neurofibromatosis (rarelyosseouslesions,cystic
r/ homogeneous / mildlyheterogeneous marrowlesions intraosseousneurofibromarare,caf6-au-laitspots
hyperintenseto fat (60%)/ of intermediate/ of low smooth,familialdisease)
signalintensityon T2Wl (3) Pagetdisease(mosaicpatternhistologically,
r/ marrowlesionshypointenseto muscleon Tl Wl radiographically identicalto monostoticcranial
@ S k ull lesion)
. skulldeformitywith cranialnervecompromise (4) Osteofibrousdysplasia(almostexclusivelyin tibia
. proptosis of infants,monostotic,lesionbeginsin cortex)
Location: frontalbone > sphenoidbone;hemicranial (5) Nonossifying fibroma
involvement (DDx:Pagetdiseaseis (6) Simplebone cyst
bilateral) (7) Giantcelltumor(no scleroticmargin)
{ scleroticskull base, may narrowneuralforamina (8) Enchondromatosis
(visual+ hearingloss) (9) Eosinophilic granuloma
r/ wideneddiploicspacewith displacementof outer (10)Osteoblastoma
table,innertablespared(DDx: Pagetdisease,inner ( 1 1 )H e m a n g i o m a
table involved) (12)Meni ngi oma
{ obliteration of sphenoid+ frontalsinusesdue to
encroachmentby fibrousdysplasticbone
r/ inferolateral displacementof orbit FIBROUS HISTIOCYTOMA
{ sclerosisof orbitalplate + smallorbit + hypoplasiaof Benignfibroushistiocytoma
frontalsinuses(DDx: Pagetdisease,meningioma lncidence: 0.1% of all bone tumors
en plaque) Histo: interlacingbundlesof fibroustissuein storiform
r/ occipitalthickening pattern(whorled/ woven) interspersedwith
r/ cysticcalvariallesions,commonlycrossingsutures mono-/ multinucleated cellsresembling
r/ mandibularcysticlesion(verycommon) histiocytes,
benigngiantcells,and lipid-laden
= osteocementoma, ossifyingfibroma macrophages;resemblesnonossifyingfibroma/
@ Pelvis+ ribs fibroxanthoma
r/ cysticlesions(extremelycommon) Age: 23-60 years
r/ fusiformenlargementof ribs + loss of normal . localizedintermittently
painfulsofttissue swelling
trabecularpattern+ thin preservedcortex(in up to Location: long bone, pelvis,vertebra(rare)
30%) Site: typicallyin epiphysis/ epiphysealequivalent
0 Fibrousdysplasiais the most commoncause of a r/ well-definedradiolucentlesionwith septa/ soap-
benignexpansilelesionof a rib! bubbleappearance/ no definablematrix
0 A rib is the most commonsite of monostotic { may have reactivescleroticrim
fibrousdysplasia! ! narrowtransitionzone (= nonaggressive lesion)
r/ protrusioacetabuli { no periostealreaction
@ Extremities Rx: curettage
. short statureas adult/ dwarfism DDx: nonossifyingfibroma(childhood/ adolescence,
r/ prematurefusionof ossificationcenters asymptomatic,eccentricmetaphyseallocation)
r/ epiphysisrarelyaffectedbeforeclosureof growth
plate AtypicalBenignFibrousHistiocytoma
r/ bowingdeformities + discrepantlimb length(tibia, Histo: "atypicalaggressive"features= mitoticfigures
, femur) due to stressof normalweightbearing present
r/ "shepherd'scrook"deformityof femoralneck r/ tyticdefect with irregularedges
= COX? Vdf?
Prognosis: maymetastasize
MalignantFibrousHistiocytoma Osseous MFH
= MFH= MALIGNANT FTBROUS XANTHOMA = XANTHO- Prevalence: 5/." of all primarymalignantbone tumors
SARCOMA = MALIGNANT HISTIOCYTOMA = FIBRO- . painful,tender,rapidlyenlargingmass
SARCOMA VARIANT . pathologicfracture(20%)
neoplasmof a mixtureof fibroblasts
Histo: spindle-cell Associated with:
+ giantcellsresemblinghistiocytes with nuclear prior radiationtherapy,bone infarcts,Paget
atypiaand pleomorphism in pinwheel disease,fibrousdysplasia,osteonecrosis,
arrangement; closelyresembleshigh-grade fibroxanthoma (= nonossifyingfibroma),
fibrosarcoma(= fibroblasticcells arrangedin enchondroma, chronicosteomyelitis
uniformpatternseparatedby collagenfibers) 0 20% of all osseousMFH arise in areas of
subtype(50-60%)
(a) pleomorphic-storiform abnormalbone!
(b) myxoidsubtype(25%) Location: femur (45/"), tibia (20%),50/" about
(c) giantcellsubtype(5-10%) knee;humerus(10% );i l i um(1 0%) ;spine;
(d) inflammatorysubtype(5-10%) sternum;clavicle;rarelysmallbonesof
(e) angiomatoidsubtype(<5%) hand + feet
Site: centralmetaphysisof long bones (90%);
Age: 10-90 (average50) years;peak prevalencein eccentricin diaphysisof longbones(10%)
Sth decade;more frequentin Caucasians; r/ radiolucent defectwith ill-definedmargins(2.5-10
M : F= 3 : 2 cm in diameter)
Location: potentialto arisein any organ(ubiquitous r/ extensivemineralization / small areasof focal
mesenchymal tissue);softtissues>> bone metaplasticcalcification
r/ permeation+ corticaldestruction
Soff-fiss ue MFH r/ expansionin smallerbones(ribs,sternum,fibula,
lncidence: 20-30/" of all soft-tissuesarcomas;most clavicle)
commonprimarymalignantsoft-tissue ./ occasionallylamellatedperiostealreaction
tumorof lateadultlife (especiallyin presenceof pathologicfracture)
0 Any deep-seatedinvasiveintramuscular mass in a r/ soft-tissueextension
patient>50 yearsof age is most likelyMFH! Cx: pathologicfracture(30-50%)
Location: extremities(75/"), flowerextremity DDx: (1) Metastasis
(50%),upperextremity(25%)1, (2) Fibrosarcoma(oftenwith sequestrum)
retroperitoneum (15/"), head + neck (5%) (3) Reticulumcell sarcoma
Site: withinlargemusclegroups (4) Osteosarcoma
. large painlesssoft-tissuemass with progressive (5) Giantcelltumor
enlargement overseveralmonths (6) Plasmacytoma
{ mass usually5-10 cm in sizewith increaseover
months/ years
{ poorlydefinedcurvilinear / punctateperipheral Pulmonary MFH (extremely rare)
calcifications/ ossifications(in 5-20%) { solitarypulmonarynodulewithoutcalcification
r/ corticalerosionof adjacentbone (HIGHLY r/ diffuseinfiltrate
S UG G E ST IV E F E AT U R E ) NUC:
CT: ./ increaseduptakeof Tc-99mMDP (mechanism
r/ well-definedsoft-tissuemass with central not understood)
hypodensearea = rnyxoidMFH (DDx: ri increaseduptakeof Ga-67citrate
hemorrhage, necrosis,leiomyosarcoma with US:
necrosis,myxoidlipo-/ chondrosarcoma) r/ well-definedmass with hyperechoic+ hypoechoic
enhancement of solidcomponents (necrotic)areas
MR: CT:
inhomogeneous poorlydefinedlesioniso-/ ./ mass of muscledensitywith hypodenseareas
hyperintenseto muscleon Tl Wl + hyperintense (necrosis)
on T2Wl ^/ invasionof abdominalmusculature, but not IVC/
Prognosis: renalveins(DDxto renalcell carcinoma)
larger+ more deeplylocated tumors have a worse A ngi o:
prognosis; 2-year s urvivalrate of 60'/"; S-year r/ hypervascularity+ earlyvenousreturn
survivalrate of 50%; local recurrencerate of 44o/";
metastaticrate of 42h (lung,lymph nodes,liver, FOCAL FIBROCARTILAGINOUS DYSPLASIA OF
bone) TIB IA
DDx: (1) Liposarcoma(youngerpatient,presenceof Associatedwith: tibia vara
fatin >40/", calcificationsrare) Age: 9-28 months
(2) Rhabdomyosarcoma Histo: densehypocellular fibroustissueresembling
(3) Synovialsarcoma(corticalerosion) tendonwith lacunaformation
 Boneand Soft-TissueDisorders 79

. slightshorteningof affectedleg LENGTH

Location: insertionof pes anserinus(= tendinous = longitudinal changeof fragments
insertionof gracilis,sartorius,semitendinosus distraction= increasefrom originalanatomiclength
muscles)distalto proximaltibialphysis; shortening= decreasefrom originalanatomiclength
unilateralinvolvement - impacted = fragmentsdriveninto each other
r/ unilateraltibia vara - overriding = alsoincludeslatitudinal changes
r/ well-defined ellipticobliquelyorientedlucentdefectin - overlapping= bayonetapposition
medialtibial metadiaphyseal cortex D IS P LA C E ME N T
r/ sclerosisalong lateralborderof lesion = latitudinal changeof anatomicaxis:
r/ absenceof bone marginsuperomedially - undisplaced
Prognosis.' resolutionin 1-4 years - anterior,posterior,mediallulnar,lateral/ radial
DDx: (1) UnilateralBlountdisease(typicallybilateralin A N GU LA TION / TILT
infants,varusangulationof uppertibia, = longaxes of fragmentsintersectat the fractureapex:
decreasedheightof medialtibialmetaphysis, - medi al /l ateralventral
, /dorsal
irregularphysis) - varus = dt']gulardeviationof distalfragment
(2) Chondromyxoid fibroma,eosinophilic toward midlineon frontalprojection
granuloma,osteoidosteoma,osteoma,fibroma, - valgus = angulardeviationof distalfragment
chondroma(not associatedwith tibiavara, soft- away from midlineon frontalprojection
tissuemass) eg, "ventralangulationof fractureapex"
eg, "in anatomicI nearanatomicalignment"
FRACTURE ROTATION
= soft-tissue injuryin whichthereis a breakin the 0 Difficultto detectradiographically!
continuityof boneor cartilage r/ differencesin diametersof apposingfragments
General description: r/ mismatchof fractureline geometry
( 1)O P E N/ [ CL OS ED ] - internal/externalrotation
open Fx = ColT'lrTlU1'lication
between
fracturedbone + skin NUC:
(2) ICOMPLETE] / TNCOMPLETE Typicaltime course:
completeFx = all corticalsurfacesdisrupted 1. Acute phase (3-4 weeks)
incompleteFx = partialseparationof bone abnormalin 80% <24 hours,in g5% <72 hours
greenstickFx = breakof one corticalmarginonly 0 Elderlypatientsshow delayedappearanceof
with intactperiosteumdue to positivescan
tensionon soft growingbone r/ broad area of increasedtraceruptake(wider
buckle/ torus Fx = bucklingof cortexdue to than fractureline)
compression 2. Subacutephase (2-3 months)= time of most
bowingFx = plasticdeformityof thin long bone intensetraceraccumulation
(clavicle,ulna,fibula) ./ more focal increasedtraceruptake
lead-pipeFx = combination of greenstick+ torus correspondingto fractureline
Fx 3. Chronicphase(1-2 years)
( 3) S T M P LE
i C O MMT N U T E D r/ slowdeclinein traceraccumulation
simpleFx = rlorlcOlTtminuted r/ in OSZ normalafter 1 year;>95/o normalafter3
comminutedFx = >2 fragments years
segmentalFx = isolatedsegmentof shaft Return to normal:
butterflyfragment= V-shapedfragmentnot 0 Non-weight-bearing bone returnsto normalmore
completelycircumscribedby quicklythan weight-bearingbone
cortex 0 Rib fracturesreturnto normalmost rapidly
(4) DIRECTIONOF FRACTUREL|NE in retationto tong 0 Complicatedfractureswith orthopedicfixation
axis of bone: devicestake longestto returnto normal
transverse,oblique,oblique-transverse, spiral 1. Simplefractures:907onormalby2 years
2. Open reduction/ fixation: <50"/"normalby 3 years
Special terminology: 3. Delayedunion: slowerthan normalfor type of
avulsionFx = fragmentpulledoff by tendon/ fracture
ligamentfrom parentbone 4. Nonunion:persistentintenseuptakein 80%
transchondral Fx = cartilaginoussurfaceinvolved 5. Complicated union(truepseudarthrosis, soft-
chondralFx = cartilagealoneinvolved tissueinterposition,impairedbloodsupply,
osteochondralFx = cartilage+ subjacentbone involved presenceof infection)
^/ intenseuptakeat fractureends
Description of anatomic positional changes: ^/ decreaseduptakeat fracturesite
= chang€in positionof distalfracturefragmentin 6. Vertebralcompressionfractures: 60% normalby
relationto proximalfracturefragment 1 year; 90%by 2 years; 97%by 3 years
 Radiology Review Manual

Types:
Typesof Fractures (a) occultfracture:
Bone Quality Load Site: sacrum> supraacetabulum, ilium
Type
r/ scleroticband,corticaldisruption,fracture
Traumatic normal singlelarge line
Fatigue(stress) normal repetitive 0 Often obscuredby overlyingbowelgas +
Insufficiency(stress)abnormal(metabolic)minimal osteopenia!
Pathologic abnormal(tumor) minimal (b) aggressivefracture:
Site: parasymphysis, Pubicrami
^/ exuberantcallusformation,osteolysis+
PathologicFracture fragments(withprolongedor delayed
= fractureat site of preexistingosseousabnormality healing/ chronicnonunion)
Cause: tumor,osteoporosis,infection,metabolic CAVE: fracturemay be misdiagnosedas
disorder also
neoplasm; interpretation
difficult
histologically
StressFracture NUC:
= fracturesproducedas a resultof repetitiveprolonged ri butterfly/ H-shaped("Hondasign")/
muscularactionon bone that has not accommodated asymmetricincompleteH-shapedpatternof
itselfto such action sacral uptake
{ pelvicoutletview for parasymphyseal fx
Insufficiency StressFracture CT and MR (most accuratemodalities):
= horffi?lphysiologicstressappliedto bone with r/ scleroticband, linearfractureline,cortical
abnormalelasticresistance/ deficient disruption,fragmentation,displacement
mineralization r/ bone marrowedema
Cause: 0 Excludesbone destruction+ softtissue
1. Osteoporosis masses!
2. Rheumatoidarthritis Prognosis; healingin 12-30 months
3. Pagetdisease
4. Fibrousdysplasia Feuonll lusurncleNcYFRAcTURE
5. Osteogenesisimperfecta Site: subcapital
6. Osteopetrosis r/ subtlefemoralneck angulation
7. Osteomalacia/ rickets ./ trabecularangulation
8. Hyperparathyroidism { subcapitalimpactionline
9. Renalosteodystrophy
10. Radiationtherapy Fatigue Stress Fracture
11. Prolongedcorticosteroid treatment = norrn?lbone subjectedto repetitivestresses(none
12. Tumortreatmentwith ifosfamide,methotrexate of whichis singularlycapableof producinga
Location:lowerextremity(calcaneus, tibia,fibula), fracture)leadingto mechanicalfailureovertime
thoracicvertebra,sacrum,ilium,pubicbone
Plainfilm / CT (1-2 weeksafteronsetof fracture):
r/ corticallucency(due to disruption)
r/ periostealnew bone formation
r/ medullarysclerosis(endostealcallusformation)
MR:
r/ marrowedema(hypointense on Tl Wl)
{ corticalfractureline
NUC ( bonesc a n ):
r/ increasedabnormaluptake
impaction line
Pelvrc InsurrtcrerucvSrness Fnncrune
. severepain in lowerback + sacroiliacjoints;
radiatesto buttocks,hips,groin,legs;worsens
withweightbearing
. walkingabilityimpaired
lncidence: 1.8-5/" of women >55 years
Predisposed: postmenopausalwomen
Location: sacralala, parasymphyseal regionof
os pubis,pubic rami,supraacetabular
region,iliacblades,superomedial
portionof ilium Fatigue (Stress) and Insufficiency Fracture of Hip

Riskfactors: new / different/ rigorousrepetitive
activity;femalesex; increasedage;
Caucasianrace;low bone mineral
density;low calciumintake;fluoride
treatmentfor osteoporosis;condition
resultingin alteredgait
. activity-relatedpain abatingwith rest
. constantpain with continuedactivity
1. Glay shoveler'sfracture: spinousprocessof
lowercervical/ upperthoracicspine
2. Clavicle: postoperative (radicalneckdissection)
3. Goracoid process of scapula: trap shooting
4. Ribs: carryingheavypack,golf, coughing
5. Distal shaft of humerus: throwingball
6. Coronoid process of ulna: pitchingball,
throwingjavelin,pitchforkwork,propelling
wheelchairs
7. Hook of hamate: swinginggolf club/ tennis
racquet/ baseballbat
8. Spondylolysis= pars interarticularis of lumbar
vertebrae: ballet,liftingheavyobjects,scrubbing
floors
9. Femoral neck: ballet,long-distance running
Site: medialfemoralneck
r/ subtlelucency/ sclerosis(= acute fracture)
r/ lucentlinesurroundedby sclerosis(= su5r.r,.
fracture)
10. Femoralshaft: ballet,marching,long-distance
running,gymnastics
11. Obturator ring of pelvis: stooping,bowling,
gymnastics
Site: superior/ inferiorpubicramus
12. Sacrum (<2%): long-distance runner,military
recruits
Site: unilateral(? due to leg lengthdiscrepancy)
13. P at ella:h u rd l i n g
14. Tibial shaft: ballet,jogging
e n n i n gj,u mp i n g ,
15. F ibula: l o n g -d i s ta n cru
parachuting
16. Calcaneus: jumping,parachuting, prolonged
standing,recentimmobilization
17. Navicular: stompingon ground,marching,
prolongedstanding,ballet
18. M et at ar s a(c
l ommonly2nM d T ): ma rc h i ng,
stompingon ground,prolongedstanding,ballet,
postoperativebunionectomy
19. Sesamoidsof metatarsal: prolongedstanding
X-RAY (15% sensitivein earlyfractures,increasingto
50% on follow-up):
cancellous(trabeculaQ bone (notoriously
difficult
to detect)
r/ subtleblurringof trabecularmargins
^/ faint scleroticradiopaquearea of
peritrabecular callus(50%changein bone
densityneeded)
r/ scleroticband (due to trabecularcompression
+ callusformation)usuallyperpendicular to
cortex
Boneand Soft-Tissue
Disorders 81
compact(cortical)bone
tr "graycortexsign" = subtle ill definitionof
cortex
./ intracorticalradiolucentstriations(early)
r/ solidthicklamellarperiostealnew bone
formation
r/ endostealthickening(later)
r/ follow-upradiographyafter 2-3 weeksof
conservativetherapy
NUC ("goldstandard"= almost100%sensitive):
{ abnormaluptakewithin6-72 hoursof injury(prior
to radiographicabnormality)
r/ "stressreaction"= focus of subtlyincreased
uptake
./ focalfusiformarea of intensecorticaluptake
{ abnormaluptakepersistsfor months
MR (verysensitivemodality;fat saturationtechnique
most sensitiveto detectincreasein water contentof
medullaryedemaI hemorrhage):
./ diminishedmarrowsignalintensityon TlWl of
fractureline
^/ increasedmarrowsignalintensityon T2Wl
(edemamay obscurethe fractureline),resolves
within6 monthsin g0%
r/ low-intensityband contiguouswith cortexon
T2Wl = fractureline of more advancedlesion
CT (leastsensitivemodality):
helpfulin: longitudinal stressfractureof tibia;in
confusingpediatricstressfracture(to
detectendostealbone formation)
DDx:
(1) Shinsplints(activitynot increasedin
angiographic / blood-poolphase)
r/ long linearuptakeon posteromedial (soleus
muscle)/ anterolateral(tibialisanterior
muscle)tibialcortexon delayedimages(from
stressto periosteumat muscleinsertionsite)
(2) Osteoidosteoma(eccentric, nidus,solid
periostealreaction,nightpain)
(3) Chronicsclerosingosteomyelitis (dense,
sclerotic,involvingentirecircumference, little
changeon serialradiographs)
(4) Osteomalacia(bowedlong bones,looserzones,
gross f ractures,demineralization)
(5) Osteogenicsarcoma(metaphyseal,aggressive
periostealreaction)
(6) Ewingtumor (lyticdestructiveappearancewith
soft-tissue component,littlechangeon serial
radiographs)
EpiphysealPlateInjury
Prevalence;6-18-30% of bone injuriesin children<16
years of age
Peak age: 12 years
Location: distalradius(28'/"),phalangesof hand
(26%),distaltibia(10%),distalphalanges
of foot (7%),distalhumerus(7"/"),distal
ulna (4/"), proximalradius(4'/"),
metacarpals (4%),distalfibula(3%)
 Radiology Review Manual

Normal Type I Type2

6$Type3 Type4

Salter-Harris Classification of Epiphyseal Plate Injuries

l (
/ \
l d
\=^/
Type6 Type 7 Type8

Rang and Ogden'sAdditions to Salter-Harris

Mechanism: 80% shearingforce; 20o/ocompression Salter Type 2 (73-75%)

to trauma: ligament> bone > physis
Resistance = shearingforce splitsgrowthplate
(hypertrophiczone mostvulnerable) Lineof fracture: throughphysis+ extendingthrough
marginof metaphysisseparatinga
Salter-Harrisclassification (consideringprobabilityof fragment
triangularmetaphyseal
growthdisturbance) (= "cornersign")
[RobertBruceSalter(1924-) andW. RobertHarris(1922-), Location: distalradius(33-50%),distaltibia+
orthopedicsurgeonsin Toronto,
Canada] fibula,phalanges
0 Prognosisis worsein lowerextremities(ankle+ Prognosis.'good, may resultin minimalshortening
knee) irrespectiveof Salter-Harristype!
Salter Type 3 (6.5-8%)
mnemonic; "SALTR" = intraarticularfracture,often occurringafter partial
Slip of physis = type 1 closureof physis
Above physis = type 2 Lineof fracture: vertically/ obliquelythrough
Lower than physis = type 3 epiphysis+ extendinghorizontally
Throughphysis = type 4 to peripheryof physis
Rammedphysis = type 5 Location: distaltibia,distalphalanx,rarelydistal
femur
SalterType 1 (6-8.5%) r/ epiphysissplitvertically
= slip of epiphysisdue to shearingforceseparating Prognosis: tair (imprecisereductionleadsto
epiphysisfrom physis alterationin linearityof articularplane)
Lineof cleavage: confinedto physis
Location: mostcommonlyin phalanges,distal Salter Type 4 (10-12%)
radius(includes:apophysealavulsion, Location: lateralcondyleof humerus,distaltibia
slippedcapitalfemoralepiphysis) r/ fractureinvolvesmetaphysis+ physis+ epiphysis
r/ displacementof epiphysealossificationcenter Prognosis; guarded(may resultin deformity+
Prognosis; favorableirrespectiveof location angulation)
 Boneand Soft-Tissue
Disorders 83

Salter Type 5 (<1%) r/ intraarticular

fracture-dislocation
of base of 1st
= cfUShinjurywith injuryto vascularsupply metacarpal
Location: distalfemur,proximaltibia,distaltibia { smallfragmentof 1st metacarpalcontinuesto
Often associatedwith: fractureof adjacentshaft articulatewith trapezium
{ no immediateradiographic finding r/ lateralretractionof 1st metacarpalshaft by
r/ shorteningof bone + cone epiphysis/ angular abductorpollicislongus
deformityon follow-up Rx: anatomicreductionimportant,difficultto keep
Prognosis.'poor (impairmentof growthin 100%) in anatomicalignment
Cx: pseudarthrosis
Triplane Fracture (6%)
Location: distaltibia,lateralcondyleof distal Boxer's Fracture
h u me ru s Mechanism;directblow with clenchedfist
{ verticalfractureof epiphysis+ horizontalcleavage r/ transversefractureof distalmetacarpal(usually5th)
planewithinphysis+ obliquefractureof adjacent
metaphysis Gamekeeper'sThumb
= S K IE R 'TH
S U MB
MR: originallydescribedas chroniclesionin hunters
r/ focal dark linear area(= line of cleavage)within stranglingrabbits
brightphysison gradientecho images(GRE) lncidence: 6/" of all skiinginjuries;50% of skiing
Cx: (1) progressive angulardeformityfrom segmental injuriesto the hand
arrestof germinalzone growthwith formation Mechanism;violentabductionof thumb with injuryto
of a bone bridgeacrossphysis= "bonebar" ulnarcollateralligament(UCL)in 1st
(2) limb lengthdiscrepancyfrom total cessation MCP (faultyhandlingof ski pole)
of growth ri disruptionof ulnarcollateralligamentof 1st MCP
(3) articularincongruity from disruptionof joint,usuallyoccurringdistallynear insertionon
articularsurface proximalphalanx
(4) Bone infarctionin metaphysis/ epiphysis r/ radialstressexaminationnecessaryto document
ligamentous disruption
Apophyseallnjury r/ displacement of UCL superficialto aponeurosis of
Mechanism: avulsiveforce adductorpollicis(= Stenerlesion)[tornend of UCL
0 Physisundersecondaryossificationcenteris weakesl may be markedby avulsedbone fragmentl
part!
At risk: hurdlers,sprinters,cheerleaders(repetitive
to and fro adduction/ abduction+ flexion/ Navicular Fracture
= SCAPHOID FRACTURE
extension)
. pain,pointtenderness, swelling 0 Most frequent(90%)of all carpalbonesfracturesl
Mechanism: fall on dorsiflexedoutstretchedhand
. pain+ tendernessat anatomicsnuffbox
Location Muscle Origin / lnsertion
Radiographicmisses: 25-33-65%
Anteriorsuperioriliacspine sartoriusmuscle+ tensor N .B .: l f i ni ti alradi ographnegati ve,
reexam ine
in2+
fasciaefemorism. 6 weeks aftertreatmentwith short-armspica cast!
Anteriorinferioriliacspine rectusfemorismuscle MR: highsensitivity
Lessertrochanter psoas muscle Bonescan: up to 100%sensitive,93% PPV after
lschialtuberosity hamstringmuscle 2-3 days
Greatertrochanter glutealmuscle Prognosis; dependenton followingfactors
lliaccrest abdominalmuscle ./ fracturedisplacement= >1 mm offset/ angulation
Symphysispubis adductormuscle / rotationof fragments(lessfavorable)
r/ location(bloodsupplyderivedfrom distalpart):
r/ irregularityat site of avulsion distal1i3 (10/o)= usuallyfragmentsreunite
^/ displacedpiecesof bone of variablesize: middle113(70%)= failureto reunitein 30%
r/ crescenticossificopacityif viewedon tangent proximal113(20%)= failureto reunitein 90%
r/ very subtledisk-shapedopacityif seen en face r/ orientationof fracture
r/ abnormalfoci of heterotopicossification(later) transverse/ horizontaloblique= relatively
stable
verticaloblique(lesscommon)= unstable
HandFracture 0 Good prognosiswith distalfracture+ no
BennettFracture displacement + no ligamentous injury!
[EdwardHallaranBennett(1837-1907), surgeonin Dublin, 0 Less favorableprognosiswith displaced/
lrelandl comminutedfracture+ proximalpolefracture!
Mechanism.' forced abduction of thumb Cx: avascularnecrosisof proximalfragment
 Rolando Fracture Significant postreduction deformity :
[SilvioRolando(?-1931?), surgeonin Genoa,ltaly] 1. Residualpositiveulnarvariance>5 mm
{ comminuted Y- / T-shaped fracture-
intraarticular indicatesunsatisfactory outcomein 40/"
throughbaseof thumbmetacarpal
dislocation 2. Dorsalangulationof palmartilt >15odecreases
Prognosis; worsethanBennett'sfracture(difficultto grip strength+ endurancein >50%
reduce)
Essex-LoprestiFracture
Forearm Fracture [PeterGordonEssex-Lopresti (1918-1951), at
surgeon
Barton Fracture Birmingham Accident Center,Englandl
pohn RheaBarton(1794-1871), orthopedicsurgeon at ./ comminuteddisplacedradialhead fracture
Pennsylvania Philadelphial
Hospital, r/ dislocationof distalradioulnarjoint
Mechanism: tall on outstretchedhand
obliquefractureof ventral/ dorsallip
r/ intraarticular GaleazziFracture
of distalradius [Ricardo Galeazzi(1866-1952), orthopedic surgeonin ltaly]
{ carpusdislocateswith distalfragmentup and back = PIEDMONT FRACTURE
on radius Mechanism: lall on outstretchedhand with elbow
flexed
Chauffeur Fracture { radialshaft fracture(mostcommonly)at junctionof
= HUTCHINSON FRACTURE = BACKFIRE FRACTURE = distalto middlethird with dorsalangulation
LORRYDRIVER FRACTURE r/ subluxation / dislocation of distalradioulnarjoint
[JonathanHutchinson(1828-1913), Britishsurgeon] { ulnarplusvariance(= radialshortening) of >10 mm
= n?rTl€derivedfrom directtraumato radialside of impliescompletedisruptionof interosseous
wrist sustainedfrom recoilof crank used in era of membrane= completeinstability of radioulnarjoint
hand crankingto start automobiles Cx: (1) Highincidenceof nonunion,delayedunion,
Mechanism;acutedorsiflexion+ abductionof hand malunion(unstablefracture)
^/ triangularfractureof radialstyloidprocess (2) Limitationof pronation/ supination

CollesFracture Monteggia-typeFracture
[Abraham Colles(1773-1843), surgeonin Dublin,lreland] = fractureof ulnarshaft + dislocationof radialhead
= POUTEAU FRACTURE (termusedin France) Bado Classification:
[ClaudePouteau (1725-1775),surgeonin Lyon,France] [JoseLuisBado(1903-1977), orthopedicsurgeon from
0 Most commonfractureof forearml Uruguayl
Mechanism: tall on outstretchedhand Type | = classic Monteggia fracture
r/ nonarticularradialfracturein distal2 cm [Giovanni Monteggia
Battista (1762-1815), ltalian
./ dorsaldisplacementof distalfragment+ volar surgeonl
angulationof fractureapex Mechanism: direct blow to the forearm
{ + ulnarstyloidfracture { anteriorlyangulatedproximalulnarfracture
{ "silver-fork"
deformity r/ anteriordislocationof radialhead
Cx: posttraumaticarthritis r/ may have associatedwrist injury
Rx: anatomicreductionimportant Cx: nonunion,limitationof motionat elbow,
nerveabnormalities
Type ll = IeveISeMonteggiafracture
r/ radiathead displacedposteriorly/
posterolaterally
{ dorsallyangulatedproximalulnarfracture
Type l l l
r/ anterior/ anterolateraldislocationof radial
head
{ ulnar metaphysealfracture
Colles Fracture Type lV
{ anteriordisplacementof radialhead

I UJ
r/ fractureof proximalthird of radius+ ulna at the
\1I same level

s\l@ Smith Fracture

Barton Fracture
YlrT
ChauffeurFracture
= REVERSECOLLESFRACTURE= REVERSEBARTON
FRACTURE= GOYRANDFRACTURE(termused in
France)
[RobertWilliamSmith(1807-1873),succeedingCollesas
professorof surgeryat TrinityCollegein Dublin,lrelandl
 Boneand Soft-Tissue
Disorders 85

BadoTypeI
q BadoTypeIII

Monteggia-typeFractures

BadoTypeII BadoTypeIV

Essex-LoprestiFracture GaleazziFracture Medial epicondylar fracture (5%)

Mechanism: hyperextensionwith valgus stress
{ avulsionof medialepicondyle(by flexor muscles
of forearm)
Mechanism; hyperflexionwith fall on back of hand r/ may becometrappedin joint space (after
r/ nonarticulardistalradialfracture reductionof concomitantelbowdislocation)
r/ ventraldisplacementof fragment @ Radius(10%)
! radialdeviationof hand Mechanism; hyperextension with valgusstress
{ "gardenspade"deformity r/ Salter-Harristype ll / lV fracture
Cx: alteredfunctionof carpus r/ transversemetaphyseal/ radialneckfracture
Mechanism: hyperextensionwith varus stress
r/ dislocationas part of Monteggiafracture(from
ElbowFracture ruptureof annularligament)
commonamongchildren2-14 years of age
@ Soft-tissue
r/ displacementof anterior+ posteriorfat pads
(= elbowjoint effusionwith supracondylar/lateral
condylar/ proximalulnarfractures)
r/ supinatorfat pad (= fractureof proximalradius)
r/ focal edema medially(= medialepicondylefx) / posterior
laterally(= lateralcondylefx) fat pad
supinator
@ Humerus (80%) fat pad
Supracondylar fracture (55%)
Mechanism; hyperextensionwith verticalstress
r/ transversefractureline
r/ distalfragmentposteriorlydisplaced/ tilted
r/ anteriorhumeralline intersectinganteriorto "teardrop"
configuration
|
posteriorthird of capitellum(on lateralx-ray) i formed bv coronoid
Lateral condylar fracture (20%) r and olecranon fossa
Mechanism; hyperextension with varus stress
r/ fractureline betweenlateralcondyle+ trochlea/ Anterior Humeral Line
throughcapitellum and Elbow Fat Pads
 @ U l n a( 1 0 % ) Classification(Judet and Letournel):
r/ longitudinal linearfracturethroughproximalshaft A. Elementaryfractures
Mechanism; hyperextension with verticalstress Posteriorwall 27% Anteriorcolumn 5%
r/ transversefracturethrougholecranon Transverse 9% Posteriorcolumn 4o/"
Mechanism; hyperextension with valgus/ varus Anteriorwall 2%
stress;blow to posteriorelbow in B. Associatedfractures
flexedposition Transverse+ posteriorwall 27"/.
r/ coronoidprocessavulsion Bothcolumns 19%
Mechanism; hyperextension-rotation associated T-shaped 6%
with forcefulcontractionof brachialm. Anteriorwall + posteriorhemitransverse 5%
Posteriorcolumn+ posteriorwall 3%

Rib Fracture PosrenronwALL(lle / nlu) rnncrvae (27%)

Associatedwith: pneumothorax,hemothorax,lung Mechanism: indirectforce transmittedthrough
contusion/ laceration lengthof femurwith flexedhipjoint
@ 1 s tr i b (kneestrikesdashboard)
0 Indicatessubstantialtrauma (due to protected Associatedwith: posteriordislocationof femur
location)
Cause: acutetrauma/ fatiguefracture(from TRarusvensE FRAcrune(9%)
carryinga heavyback pack) N.B.: mostdifficultto diagnose+ comprehend
Associatedwith: aortic/ greatvesselinjury; ./ transectsboththe iliopubic+ ilioischial
columns
thoracicvertebralfracture; with fractureline in an anteroposterior
direction
scapularfracture
@ Lower ribs ArurenroncoLUMNrnacrune (5%)
Associatedwith: injuryto upperabdominalorgans Mechanism; blow to greatertrochanterwith hip
/ diaphragm externallyrotated
Associatedwith: posteriorcolumn/ transverse
fracture
Flail Chest r/ fracturebeginsbetweenanterioriliacspines
= fractureol >4 contiguousribs + traversesthe acetabularfossa + ends in the
. paradoxicmotionof chestwall with respiration ischiopubicramus
. respiratoryfailure
PosrenroncoLUMNrnncrunr (4%)
Cough Fracture Mechanism: indirectforcetransmittedthrough
Location: 4-gth rib in anterioraxillaryline lengthof femurwith hip abducted
Associatedwith: posteriordislocationof femur
+ sciaticnerve injury
PelvicFracture r/ fracturebeginsat greatersciaticnotch
Unstablepelvicfractures + traversesthe posterioraspectof acetabular
(a) anteriorcompression fossa+ ends in the ischiopubicramus
1. Bilateralverticalpubicramifractures
2. Symphysis+ sacroiliac jointdiastasis AHreRroRwALLrnacrune (2%)
(b) lateralcompression Mechanism: force transmittedthroughgreater
1. Malgaigne(ipsilateralanterior+ posteriorfx) trochanter
2. Bucket-handle(contralateral anterior Associatedwith: posteriordislocationof femur
+ posteriorfx) + sciaticnerveinjury
(c) verticalshear { fracturebeginson anteriorrim of acetabulum
1. Superiordisplacement of pelvis + emergeson lateralaspectof superiorpubic
ramus

Acetabular Fracture Malgaigne Fracture

Anatomy & Function:
most importantportionof acetabulumis roof/ dome;
weight-bearing surfacefor entirelowerlimbis
derived+ supportedby 2 columnswhichare
orientedin an inverted"Y" and join abovethe
acetabularroof at an angleof 60o:
(a) anterioriliopubiccolumnof acetabulum
(b) posteriorilioischialcolumnof acetabulum
[Joseph Francois Malgaigne (1806-1865), Frenchsurgical
published
historian, firstcomprehensive bookon fractures]
= fracture-dislocation of one side of the pelviswith
anterior+ posteriordisruptionof pelvicring
Mechanism:directtrauma
. shorteningof involvedextremity
r/ verticalfracturesthroughone side of pelvicring
(1) superiorto acetabulum(ilium)
 Boneand Soft-TissueDisorders 87

TransverseFracture Anterior Column Fracture Posterior Column Fracture Anterior Wall Fracture

Fracture of Both Columns T-shaped Fracture Anterior Wall + Posterior Posterior Wall + Posterior
HemitransverseFracture Column Fracture
Fracturesof the Acetabulum

e-z F
-------= 1
-9:[
!-g

Malgaigne Fracture DuverneyFracture

 (2) inferiorto acetabulum(pubicrami) 0 Lateralplateaufractures(type l-lll) are most
(3) + sacroiliacdislocation/ fracture common!
r/ lateralunstablefragmentcontainsacetabulum 0 Fracturesof medialplateauare associatedwith
greaterviolenceand higherpercentageof
Bucket Handle Fracture associatedinjuries!
r/ doubleverticalfracturethroughsuperiorand inferior
pubicrami+ sacroiliacjointdislocation
on Foot Fracture
contralateralside Ankle Fracture
lncidence; ankle injuriesaccountfor 10% of all
DuverneyFracture emergencyroom visits;85% of all ankle
[Joseph GuichardDuverney (1648-1730),
French
surgeon] sprainsinvolvelateralligaments
r/ isolatedfractureof iliacwing Ligamentous connections at ankle:
(a) bindingtibia+ fibula
KneeFracture 1. anteriorinferiortibiofibularligament
SegondFracture (= tibiofibular
syndesmosis)
[PaulFerdinand Segond (1851-1912), in chiefat
surgeon 2. posteriorinferiortibiofibularligament
SalpOtridre in Paris,Francel 3. transversetibiofibular ligament
Mechanism;externalrotation+ varus stresscausing 4. interosseous membrane
excessivetensionon the lateral (b) lateralmalleolus
capsularligament 85%of all anklesprainsinvolvetheseligaments:
Associatedwith: lesionof anteriorcruciateligament 1. anteriortalofibularligament
(75-100V"),meniscaltear (67%) 2. posteriortalofibularligament
. anterolateralinstabilityof the knee 3. calcaneofibular ligament
r/ smallcorticalavulsionfractureof proximallateral (c) medialmalleolus= deltoidligamentwith
tibial rim just distalto lateralplateau 1. navicularportion
2. sustentaculum portion
Tibial Plateau Fracture (Schatzkerclassification) 3. talarportion
Mechanism.'valgusforce ("bumper/ fenderfracture"
from lateralforce of automobileagainst Larenal Malleoun Fnacrunes
a pedestrian's fixedknee)/ compression Weber Type A
force often in extension [Bernhard GeorgWeber(1929-), orthopedic
surgeon
TypeI = wedge-shapedpure cleavagefracture 6% in St.Gall,Switzerlandl
Typell = combinedcleavage+ median 25% = SUPINATION-ADDUCTION INJURY = INVERSION-
compressionfracture A D D U C TION
IN JU R Y
Typelll = pure compressionfracture 36%
TypelV = medialplateaufracturewith a split/ 10%
depressedcomminution

ffi
TypeV = bicondylarfracture,oftenwith 3%
invertedY appearance
Type Vl = transverse/ obliquefracturewith 20"/"
separationof metaphysisfrom
diaphysis

TypeI TypeII Type III

(o)
Pellegrini-Stieda Disease
I 5
\ ( \
\ /l/
Segond Fracture
Type IV
ilry
TypeV

Tibial Plateau Fractures

TypeVI
 Boneand Soft-TissueDisorders 89

Supination- Supination- Pronation-

Adduction Abduction External Rotation LeFort Ankle Fracture Tillaux Fracture

Mechanism: r/ tear of tibiofibularligament/ avulsionof

(1) avulsiveforcesaffectlateralanklestructures anteriortubercle(Tillaux-Chaput) i avulsionof
(2) impactiveforcessecondaryto talar shift posteriortubercle(Volkmann)
stressmedialstructures r/ tear of interosseousmembrane= lateral
r/ spraini ruptureof lateralcollateralligament instability
0 Anteriortibiofibular ligamentrupturesalone (a) Tillaux Fracture
in 66/" [PaulJulesTillaux (1834-1904),French surgeonand
0 Injuryof all 3 lateralligamentsin 20"/" anatomistl
Prognosis.'chroniclateralankle instabilityin { avulsioninjuryof anteriortibialtubercleat
10-2oo/o attachmentof distalanteriortibiofibular
{ transverseavulsionof malleolussparing ligament
tibiofibularligaments (b) Maisonneuve Fracture
r/ + obliquefractureof medialmalleolus [Jacques GillesMaisonneuve (1809-1897),
student
r/ t posteriortibiallip fracture of Dupuytrenl
r/ tear of distaltibiofibularsyndesmosis
Weber Type B + interosseous membrane
= SUPINATION-ABDUCTION INJURY = EVERSION. { spiralfractureof upperthird of fibula
EXTERNAL ROTATION r/ associatedfractureof medialmalleolus/
Mechanism: ruptureof deep deltoidligament
(1) avulsiveforceson medialstructures
(2) impactingforceson lateralstructures(talar Chopart Fracture
impact) Chopart(1743-1795),
[Francois surgeonin Paris,France]
r/ oblique/ spiralfractureof lateralmalleolus ! fracture-dislocation throughmidtarsal/ Chopart
startingat levelof joint space extending (calcaneocuboid joint
+ talonavicular)
proximally ! commonlyassociatedwith fracturesof the bones
r/ lateralsubluxationof talus abuttingthe joint
r/ partialdisruptionof tibiofibularligament
I * tpt"in / rupture/ avulsionof deltoidligament
r/ + transversefractureof medialmalleolus
(a) Dupuytren Fracture
[Guillaume Dupuytren (1777-1835),Frenchsurgeon]
r/ fractureof distalfibulaabove a disrupted
tibiofibular
ligament+ disruptionof deltoid
ligament
(b) Le Fort Fracture of Ankle
[LeonClement Le Fort(1829-1893), Frenchsurgeon]
r/ verticalfractureof anteriormedialportionof
distalfibula
r/ avulsionof anteriortibiofibular ligament

Weber Type C
= PRONATION-EXTERNAL ROTATION = EVERSION
+ EXTERNAL ROTATION
r/ fibularfracturehigherthan anklejoint
(Maisonneuve fractureif aroundknee)
./ + deltoidligamenttear
r/ + medialmalleolarfracture Maisonneuve Fracture
 CalcanealFracture
lncidence;most commonlyfracturedtarsalbone;
60% of all tarsalfractures;2o/oof all fracturesin the
body;commonlybilateral
Mechanism: fall from heights
May be associatedwith: lumbarvertebralfracture
A ge: 95% i n adul ts,5/" i n chi l dren
- adulthood: intraarticular (75'/.),
extraarticular(25%)
- childhood: extraarticular(63-92%)
Classification:
(a) extraarticularfractureof calcanealtuberosity:
Lisfranc Shepherd Fracture beaktype,vertical,horizontal, medialavulsion
Fracture-Dislocation (b) intraarticular
fracture
- subtalarjointinvolvement:undisplaced,
displaced,comminuted
- calcaneocuboid joint involvement
Jones Fracture ^/ apex of lateraltalar processdoes not pointto
[RobertJones(1857-1933), Britishorthopedic
surgeon and "crucialangle"of Gissane
pioneerin radiologyl
r/ Boehlerangle decreasedbelow 28o4}o
r/ transversefractureat base of Sth metatarsaldistal
to metatarsaltuberosity>1.5cm from proximaltip

PeroneusBrevis Avulsion Fracture FROSTBITE

= METATARSAL 5 TUBEROSITY FRACTURE
Mechanism: plantarflexion+ inversion(stepping
off a curb)
./ transverseavulsionfractureof base of 5th
metatarsalproximalto metatarsaltuberosity
(insertionof peroneusbrevistendon)
DDx: Jones fracture(slightlydifferentlocation)
Lisfranc Fracture
[Jacques LisfrancDeSaintMartin(1790-1847), surgeon in
Napoleon's army]
Mechanism: metatarsalheadsfixed and hindfoot
forced plantanirrard and into rotation
r/ fracture-dislocation/ fracture-subluxation of
tarsometatarsal joints (typically2 through5)
r/ lateraldisplacementof metatarsals
Shepherd Fracture
[FrancisJ. Shepherd(1851-1929), demonstrator in anatomy
at McGillUniversityin Montreal,Canadal
r/ fracture of lateral tubercle of posterior process of
talus
DDx: os trigonum
Peroneus Brevis JonesFracture
Avulsion Fracture
Cause: (1) cellularinjury+ necrosisfrom freezing
process
(2) cessationof circulation secondaryto cellular
aggregates+ thrombiformingas a resultof
exposureto low temperaturesbelow
-13" C (usuallycold air)
. firm whitenumbareasin cutis(separation of epidermal-
dermalinterface)
Location: feet,hands(thumbcommonlyspareddue to
protectionby clenchedfist)
Earlychanges:
{ soft-tissueswelling+ loss of tissueat tips of digits
CHILD
{ fragmentation / prematurefusioni destruction of distal
phalangealepiphyses
{ secondaryinfection,articularcartilageinjury,joint
spacenarrowing,sclerosis,osteophytosis of DIP
{ shortening+ deviationi deformityof fingers
A D U LT
{ osteoporosis(4-10 weeks after injury)
r/ periostitis
./ acromutilation(secondaryto osteomyelitis+ surgical
removal)+ tuftal resorption(resultof soft-tissueloss)
r/ smallroundpunched-out areasnearedge of joint
^/ interphalangeal jointabnormalities(simulating
osteoarthritis)
{ calcification/ ossificationof pinna
A ngi o:
^/ vasospasm,stenosis,occlusion
{ proliferationof arterial+ venouscollaterals(in
recoveryphase)
Bonescintigraphy:
{ persistentabsenceof uptake(= lack of vascular
perfusion)indicatesnonviabletissue
Rx: selectiveangiographywith intraarterialreserpine

GANGLION
Ganglionclst = mucin-containing cyst arisingfrom tendon
sheath/ joint capsuleI bursaI
subchondral bone linedby flatspindle-
shapedcells
Synovialcyst = cystcontinuouswithjointcapsulelined
by synovialcells (termis used by some
synonymously with ganglion)
Soft-tissueGanglion
= cystictumorlikelesionusuallyattachedto a tendon
sheath
Origin: synovialherniation/ coalescenceof smaller
cystsformedby myxomatousdegeneration
of periarticularconnectivetissue
. asymptomatic/ pain
. uni-/ multilocular swelling
GARDNER
Location: hand,wrist,foot (overdorsum)
Site: arisefrom tendon,muscle,semilunarcartilage
{ soft-tissuemass with surfacebone resorption
r/ periostealnew-boneformation
! arthrographymay demonstratecommunicationwith
joint/ tendonsheath
./ internalseptations,lobulatedconfiguration
r/ low to intermediatesignalintensityon Tl Wl
r/ frignsignalintensityon T2Wl
Prognosisi mayresolvespontaneously
Rx: steroidinjectionmay improvesymptomatology
lntraosseousGanglion
= benignsubchondralradiolucent lesionWITHOUT
degenerativearthritis
GAUCHER
. mild localizedpain (4% of patientswith unexplained
wrist pain)
Age: middleage
Origin: (1) mucoiddegenerationof intraosseous
connectivetissueperhapsdue to traumai
ischemia
(2) penetrationof juxtaosseoussoft-tissue
ganglion(= sYnovialherniation) into
underlyingbone (occasionally)
Path: uni- I multilocularcyst surroundedby fibrous
lining,containinggelatinousmaterial
Location: epiphysisof long bone (medialmalleolus,
femoralhead,proximaltibia,carpalbones)/
subarticularflat bone (acetabulum)
{ well-demarcated solitary0.6-6 cm lytic lesion
r/ scleroticmargin
r/ ruOcommunicationwith joint
^/ increasedradiotraceruptakeon bone scintigraphy(in
10%)
DDx: posttraumatic / degenerativecyst
PeriostealGanglion
= cysticstructure
withviscid
/ mucinous
contents
lncidence; 11 cases in literature
Age: 39-50 years; M > F
. swelling,mild tenderness
Location: long tubularbones of lowerextremity
{ corticalerosion/ scalloping/ reactivebone formation
Boneand Soft-IissueDisorders 91
./ NO intraosseouscomponent(endostealsurfaceintact)
CT:
r/ well-definedsoft-tissuemass adjacentto bone
cortexwith fluid contents
MR:
r/ homogeneous isointensesignalto muscleon Tl Wl
^/ homogeneous hyperintense signaltofat on T2Wl
r/ tttOinternalseptations(DDx to soft-tissueganglion)
DDx: periostealchondromawithoutmatrixcalcification,
corticaldesmoid,subperiosteal aneurysmalbone
cyst, acutesubperiostealhematoma(historyof
trauma/ blooddyscrasia),subperiostealabscess
(involvementof adjacentbone marrow)
Rx: surgicalexcision(localrecurrencepossibte)
SYNDROME
= autosomaldominantsyndromecharacterized by
(1) osteomas(2) soft-tissuetumors(3) colonicpolyps
Locationof osteomas: paranasalsinuses;outer table of
skull(frequent);mandible(at angle)
r/ endostealcorticalthickening/ osteomasin any bone
r/ may have solid periostealcorticalthickening
r/ osteomas/ exostosesmay protrudefrom periosteal
surface
{ wavy corticalthickeningof superioraspectof ribs
^/ polyps: colon,stomach,duodenum,ampullaof Vater,
small intestine
Cx: highincidenceof carcinomaof duodenumi ampulla
of Vater
DISEASE
= tala autosomalrecessive/ dominant(in a few) lipid
storagedisorder;commonamongAshkenaziJews;
M=F
Etiology: deficiencyof lysosomalhydrolaseacid
B-glycosidase (= glucocerebrosidase) leadsto
accumulation of glucosylceramide
(glucocerebroside) withinmacrophages of RES
(liver,spleen,bone marrow,lung,lymphnodes)
Histo: bone-marrowaspirateshows Gauchercells
(kerasin-ladenhistiocytes) of 20-100 pm in
diameterwith a foamy appearance
Types:
(1) Adult/ chronicnonneuropathic form = type 1
(mostcommonform in USA)
Age of onset: 3rd-4th decade
Prognosis; longesttime of survival;pulmonary
involvementI hepaticfailuremay lead to
early death
(2) Rapidlyfatal infantile/ acuteneuropathic form = type 2
Age of onset: 1-12 months
. earlyonsetof significanthepatosplenomegaly
. severeprogressiveneurologicsymptoms:
seizures,mentalretardation,strabismus,spasticity
r/ skeletalmanifestations are rare
Prognosis; fatal duringfirst 2 years of life
(3) Juvenile/ subacuteneuropathicform = type 3
(raresttype)
Age ofonset: 2-6years
92 Radiology Review Manual
' hepatosplenomegaly
. mild neurologicinvolvement
r/ delayedonset of skeletalmanifestations
Prognosis; survivalinto adolescence
. hepatosplenomegaly, impairmentof liverfunction,ascites
. elevatedserum acid phosphatase
. pancytopenia,anemia,leukopenia,thrombocytopenia
(hypersplenism)
. hemochromatosis (yellowishbrownpigmentationof
conjunctiva+ skin)
. dull bone pain; bone involvement in 75%
Location:predominantly longtubularbones(distalfemur),
axialskeleton,hip,shoulder,pelvis; bilateral
{ generalizedosteopenia(decreasein trabecularbone
density):
r/ strikingcorticalthinning+ bonewidening
{ endostealscalloping(due to marrowpacking)
r/ numeroussharplycircumscribedlytic lesionsresembling
metastases/ multiplemyeloma(marrowreplacement)
ri periostealreaction= cloaking
ri Erlenmeyerflask deformityof distalfemur + proximal
tibia(2'to marrowinfiltration) MOSTCHARACTERISTIC
r/ weakeningof subchondralbone:
r/ osteonecrosis(common),frequentlyof femoralhead
, { degenerativearthritis
r/ bone infarctionin long-bonemetaphyses(common):
{ focal/ serpentineareas of sclerosis
{ bone-within-bone appearance
r/ H-shaped/ "step-off"/ biconcave"fish-mouth"vertebrae
(DDx: sicklecelldisease)
@ Liver
{ hepatomegaly
r/ nonspecificfatty + cirrhoticchanges
@ Spleen
tr splenomegaly+ lymphadenopathy
{ multiplenodularlesions(= clustersof RES cells
ladenwith glucosylceramide) : r/ thinningof overlyingcortex
r/ hypodensewithoutenhancementon CT
r/ hypoechoic/ hyperechoicon US
r/ isointenseon Tl Wl + hyperintenseon T2Wl
r/ splenicinfarctionleadingto fibrosis
@ Lung
{ diffusereticulonodular infiltratesat lung bases
(= infiltrationwith Gauchercells)
Cx: 0 >90% have orthopediccomplicationsat some
t im e
(1) pathologicfractures+ compressionfracturesof
vertebrae
(2) avascularnecrosisof femoralhead, humeral
head,wrist,ankle(common)
(3) osteomyelitis(increasedincidence)
(4) myelosclerosis in long-standingdisease
(5) repeatedpulmonaryinfections
Prognosis; highlyvariableclinicalcourse;strong
relationshipbetweensplenicvolumeand
diseaseseverity
DDx: metastaticdisease,multiplemyeloma,leukemia,
sickle-celldisease,fibrousdysplasia
GIANTCELLREPARATIVE
GRANULOMA
= GIANTCELLREACTION = GIANTCELLGRANULOMA
Cause: ? reactiveinflammatoryprocessto trauma/
infection(not a true neoplasm)
Histo: numerousgiant cells in exuberantfibrousmatrix
arrangedin clustersaroundfoci of hemorrhage
+ commonlyexhibitingosteoidformation(unusual
in giantcelltumor);indistinguishable from brown
tumorof HPT; cysticdegeneration+ ABC
componentsdistinctlyuncommon
Peak age: 2nd + 3rd decade(rangingfrom childhoodto
76 years);74/" <30 yearsof age; M:F = 1:1
May be associatedwith: enchondromatosis,Goltz
syndrome,fibrousdysplasia,
Pagetdisease
Location:
@ Gnathic(1-7% of all benignoraltumors):gingiva
+ alveolarmucosaof mandible,maxilla
(a) centraltype = in bone
(b) peripheraltype = in gingivalsoft tissue
M : F= 1 : 2
. nonspecific pain+ swelling(increasing during
pregnancy)
{ expansileremodelingof bonewith multilocular
appearance
./ thinnedusuallyintactcortex
DDx: indistinguishable from odontogeniccyst,ABC,
ameloblastoma, odontogenicmyxoma,
odontogenic fibroma
@ Smallbonesof hand+ feet (lesscommon):
phalangesof hand > metcarpals> metatarsals>
carpalbones > tarsalbones > phalangesof foot
M : F= 1 : 1
. nonspecificpain + swellingfor monthsto years
Site: metaphysis+ extensioninto diaphysis;
extensionintoepiphysisis UNCOMMON
r/ expansilelyticdefectof 2-2.5 cm in diameterwith
internaltrabeculations
d matrixmineralization may be seen (DDx to GCT)
r/ periostealreactionis unusual(as in GCT)
r/ extensionbeyondcortexis unusual
@ Otherlocations(rare):
ethmoidsinus,sphenoidsinus,temporalbone,skull,
spine,clavicle,tibia,humerus,ribs,femur
Cx: pathologicfracture
Prognosisi may recur;no malignanttransformation
Rx: curettage(22-50% recurrencerate) / local excision
DDx: (1) Enchondroma (samelocation,matrixcalcification)
(2) Aneurysmalbonecyst (rarein smallbonesof
hand + feet, typicallypriorto epiphysealclosure)
(3) Giantcell tumor (moreaggressiveappearance)
(4) lnfection(clinical)
(5) Browntumor of HPT (periostealbone
resorption,abnormalCa + P levels)
GIANTCELLTUMOR
= OSTEOCLASTOMA= OSTEOBLASTOCLASTOMA
= TUMOR
OF MYELOPLAXUS= MYELOIDSARCOMA

= nonmineralized lyticeccentricmetaepiphyseal lesion
involvinga long bonewith extensionto subarticular
bone in the skeletallymaturepatient
Origin: probablyarisefrom zone of intenseosteoclastic
activity(of endochondralossification)in
skeletallyimmaturepatients
lncidence: 4-9.5/" of all primarybone tumors; 18-29%
of benignskeletaltumors;unusuallyhigh
prevalencein China+ southernIndia
Path: friablevascularstromaof numerousthin-walled
capillarieswith necrosis+ hemorrhage+ cyst
formation(DDx:aneurysmalbonecystwithoutsolid
areas)
Histo: large numberof multinucleated osteoclasticgiant
cells in a diffusedistributionin a backgroundof
mononuclear cellsintermixedthroughouta spindle
cell stroma(DDx:giantcellscharacteristic of all
reactivebone diseaseas in pigmentedvillonodular
synovitis,benignchondroblastoma, nonosteogenic
fibroma,chondromyxoid fibroma,fibrousdysplasia)
Age: peaks in 3rd decade;1-3% < age 14;80o/obetween
20 and 50 years;9-13% > age 50; M:F = 1:1.1to
1 : 1 . 5( i ns p i n e1 : 2 . 5 )
May be associatedwith: Paget disease (in 50-60%
locatedin skull + facialbones)
Staging:
Stage 1 indolentradiographic+ histologicappearance
( 10-1 5 % )
Stage2 more aggressiveradiographicappearance
with expansileremodeling(70-80T.)
Stage3 extensioninto adjacentsoft tissueswith
histologically benignappearance(10-1 5%)
. pain at affectedsite (mostcommon- in 10% pathologic
fracture)
. localswelling+ tenderness
. weakness+ sensorydeficits(if in spine)
Location:
@ long bones (75-85-90%)
- lowerextremity(50-65% about knee):
distalend of femur (23-30%) > proximalend of
tibia (20-25%) > proximalfemur (4%) > distal
tibia (2-5%) > proximalfibula (94%) > foot
(1-2%)
RARE in patella(the largestsesamoidbone)
+ greatertrochanter(epiphysealequivalent)
- upperextremity(awayfrom elbow):
distalend of radius(10-12/") , proximalend of
humerus(4-8%) > hand and wrist (1-S%)
@ flat bones (15%)
pelvis: sacrumnearSIJ (4%),iliacbone (3%)
spine(3-6%): thoracic> cervical> lumbarspine
(tumorfrequency2nd only to chordoma)
rib (anterior/ posteriorend)
skull(sphenoidbone)
Site: eccentric(42-93%\ in metaphysisof long bones
adjacentto ossifiedepiphysealline;extensionto
within 1 cm of subarticularbone (84-99%) after
fusionof epiphysealplate (MOSTTYPTCAL) with
possibletransarticularspread
Boneand Soft-Tissue
Disorders 93
0 The open epiphysealplate acts as a barrierto
tumorgrowthl
r/ well-circumscribed expansilesolitarylytic bone lesion
with a narrowzone of transition:
r/ wide zone of transition(10-20%)
r/ largelesionsare more centrallylocated
{ "soapbubble"appearance(47-60%) = expansile
remodelingwith multiloculated appearance:
{ NO internalmineralization of tumormatrix
./ prominenttrabeculation(33-57%):
(a) reactivewith appositionalbone growth
(b) pseudotrabeculation of osseousridgesin
endostealscalloping
^/ no sclerosis(80-85%) lperiostealreaction(10-30%)
, due to aggressiverapidgrowthin absenceof fracture
r/ corticalpenetration(33-50%):
r/ corticalthinning
r/ soft-tissueinvasion(25%)
r/ complete/ incompletepathologicfracture(11-37%)
r/ destructionof vertebralbody with secondaryinvasionof
posteriorelements(DDx: ABC, osteoblastoma)
r/ frequentlyvertebralcollapse
r/ involvesadjacentvertebraldisks + vertebrae,crosses
joint
, sacroiliac
r/ may crossjoint space in long bones (exceedinglyrare)
NUC:
{ diffuselyincreaseduptake* "doughnut"sign (57%)ot
, centralphotopeniaon delayedbone scintigraphy
r/ increaseduptakeacrossan articulation+ in adjacent
joints (62%)due to increasedbloodflow + disuse
osteoporosisand NOT tumor extension
A ngi o:
r/ hypervascular(60-65%)/ hypovascular(20%)I
avascular(10%) lesion
CT:
r/ tumor of soft-tissueattenuationsimilarto musclewith
foci of low attenuation(hemorrhage/ necrosis):
r/ tttOmatrixmineralization
{ well-definedmargins+ thin rim of sclerosis(in up to
,20%)
r/ soft-tissueextension(33-44%) usuallyat
metaphysealend of tumor
{ aneurysmalbone cyst components(in 1a%\ of low
densitywith fluidlevels
{ joint involvement is unusualexceptfor sacroiliac joint
(38%)with sacrallesion
^/ significantenhancement
MR:
relativelywell-defined lesionof heterogeneous signal
intensitywith low to intermediateintensityon TlWl
+ T2Wl (63-96%)due to increasedcellularity+ high
collagencontent+ hemosiderin
0 HELPFULfeatureto distinguish from other
subarticularlesions(solitarysubchondralcyst,
intraosseous ganglion,Brodieabscess,clearcell
chondrosarcoma with hyperintensematrixon T2Wl)
focal aneurysmalbone cyst components(in 14%)in
tumor centerwith markedhyperintensity on T2Wl
0 Directbiopsyto peripheralsolid-tissuecomponent
to preventmisdiagnosisl
94 Radiology Review Manual
margin(= osSeoussclerosis/
{ low-signal-intensity
pseudocapsule
enhancement
r/ significant of solid-tissuecomponent
Cx: in 5-10% malignanttransformationwithinfirst
5 years(M:F= 3:1); metastases to lung
Prognosis; locallyaggressive;80-90% recurrencerate
withinfirst 3 years after initialtreatment
Rx: currettage+ bone grafting(4040% recurrence);
currettagewith fillingof void with high-speedburr
+ polymethylmeth-acrylate (2-25% recurrence);
wide resection(7'/" recurrence)and reconstruction
with allografts/ metalprosthesis;radiationtherapy
for inoperableGCT (3943% recurrence)
DDx: (1) Aneurysmalbone cyst (containsonly cystic
regions;in posteriorelementsof spine)
(2) Browntumor of HPT (lab values)
(3) Osteoblastoma
(4) Cartilagetumor: chondroblastoma,
enchondroma(notepiphyseal), chondromyxoid
fibroma,chondrosarcoma
(5) Nonossifying fibroma
(6) Bone abscess
(7) Hemangioma
(8) Fibrousdysplasia
(9) Giantcell reparativegranuloma
MultifocalGiantCellTumor
= additionalGCTs (up to a maximumof 20) developing
synchronously/ metachronously for up to 20 years
withoutincreasedrisk of pulmonarymetastases
lncidence: <1o/"of all GCT cases
Age: 25 years (range,1142 years); McF
May be associated with:
Pagetdisease,usuallypolyostotic(GCT developsat a
mean age of 61 years + after an averagetime lapse
of 12 years)with involvement of skull+ facialbones
Location: increasedprevalenceof hands + feet
MalignantGiantCellTumor
= group of giant cell-containinglesionscapableof
malignantbehavior+ pulmonarymetastases
Prevalence: 5-10% of all GCTs
Age: olderthan patientswith benignGCTs
Types:
(1) Benignmetastasizing GCT
Prevalence: 1-5%
{ pulmonarymetastasesmay remainstable/
regressspontaneously
{ pulmonarynodulesmay show peripheral
ossification
Prognosis; death in 13%
(2) Primarymalignanttransformationof GCT
= malignanttumor of bone composedof a
sarcomatousgrowthjuxtaposedto zones of
typicalbenignGCT withouta historyof radiation
therapy/ repeatedcurrettage/ resection
Prognosis; median survivaltime of 4 years
(3) SecondarymalignantGCT (86%)
= ssfcorT'l?tousgrowth that occurs at a site of
previouslydocumentedGCT usuallyafter
radiationtherapy(80%)/ repeatedresections
Prognosis; mediansurvivaltimeof 1 year
(4) Osteoclastic(giantcell) sarcoma
= highlymalignanttumorcomposedof anaplastic
osteoclast-likegiantcellswithouttumorosteoid/
bone / cartilage
TUMOR
GLOMUS
= hamartomacomposedof cellsderivedfrom
neuromyoarterial apparatus(regulatingbloodflow in
skin)
Glomus body = €I'tc€lPSUlated oval organof 300 pm
length;locatedin reticulardermis(= deepestlayerof
skin);concentratedin tips of digits(93-501lcm2);
composedof an afferentarteriole,an anastomotic
vessel(= Sucquet-Hoyer canallinedby endothelium
+ surroundedby smoothmusclefibers),a primary
collectingvein,the intraglomerular reticulum+ capsule
Histo: (a) vascular(b) myxoid(c) solidform
Prevalence: 1-5% of soft-tissuetumors of hand
Age: mostlyin 4-5th decade
. joint tenderness+ pain (on averageof 4-7 years
durationpriorto diagnosis)
. Love test = elicitingpain by applyingprecisepressure
with a penciltip
' Hildrethsign = disappearanceof pain after applicationof
a tourniquetproximallyon arm (PATHOGNOMONIC)
GLOMU STU MOR
@ S U B U N GU A L
r/ increaseddistancebetweendorsumof phalanx
+ undersideof nail (25%)
r/ extrinsicbone erosion(14-25-65%),oftenwith
scleroticborder
{ smallhypoechoictumorby US (>3 mm detectable)
r/ homogeneously high-signal-intensity lesionon T2Wl
(detectableit >2 mm in diameter)
@ GLOMUSTUMOROF BONEoccasionally withinbone
r/ resemblesenchondroma
DDx: (1) Mucoidcyst (painless,in proximalnailfold,
communicating with DIP joint,associatedwith
osteoarthritis)
(2) Angioma(moresuperficiallylocated)
GOUT
= characterizedby derangementof purinemetabolism
manifestedby:
(1) hyperuri cemi a
(2) depositionof positivelybirefringentmonosodium
urate monohydratecrystalsin synovialfluid
leukocytes
(3) gross depositsof sodiumurate in periarticularsoft
tissues(synovialmembranes,articularcartilage,
ligaments,bursae)
(4) recurrentepisodesof arthritis
Age: >40 years; males (in women gout may occur after
menopause)

Cause:
A. PrimaryGout (90%)
Incidence: 0.3o/";M:F = 20:1;
5% in postmenopausal women
Disturbance:
. overproduction of uricaciddue to inbornerrorof
metabolism
. inheriteddefectin renafurateexcretion
(a) ldiopathic(99%)
. normalurinaryexcretion(80-90%)
. increasedurinaryexcretion(10-20%)
(b) Specificenzyme/ metabolicdefect(1%)
(1) increasedactivityof PP-ribose-psynthetase
(2) p artialdeficiencyof hypoxanthine-guanine
phosphoribosyltransferase
B. SecondaryGout (10%)
0 Rarelycausefor radiographically apparentdisease
(a) increasedturnoverof nucleicacids:
(1) Myeloproliferativedisorders+ sequelaeof
theirtreatment:polycythemia vera,leukemia,
lymphoma,multiplemyeloma
(2) Blooddyscrasias: chronichemolysis
(b) increasein purinesynthesisde novodue to
enzymedefects:
(1)GlycogenstoragediseaseType | (vonGierke
= glucose-6-phosphatase deficiency)
(2) Lesch-Nyhan syndrome(choreoathetosis,
spasticity,mentalretardation,self-mutilation
of lips+ fingertips)
due to absenceof hypo-
xanthine-guani ne phosphoribosyltransferase
(c) acquireddefectin renalexcretionof urates(due
to reductionin renalfunction):
(1) Chronicrenalfailure
(2) Drugs,toxins:lead poisoning
(3) Endocrinologic: myxedema,hypo-/
hyperparathyroidism
(4) Vascular: myocardialinfarction,hypertension
Histo: tophus(PATHOGNOMONTC LESTON) composed
of crystalline
/ amorphousuratessurroundedby
highlyvascularized inflammatory tissuerichin
histiocytes,lymphocytes,f ibroblasts,foreign-body
giantcells(similarto a foreign-bodygranuloma)
Clinical stages in chronologic order:
(1) Asymptomatic hyperuricemia
(2) Acute gouty arthritis
0 Gout accountstor 5h of all cases of arthritis
Precipitatedby: trauma,surgery,alcohol,dietary
indiscretion,
systemicinfection
. monoarticular (90%)
. polyarticular(10"/"): anyjoint may be affected
Prognosis; usuallyself-limited(pain resolvingwithin
a few hours/ days)without.treatment
(3) Chronic tophaceousgout
= multiplelargeuratedepositsin intraarticular,
extraarticular,intraosseouslocation
Prevalence: <50% of patientsexperiencingacute
attacks; M:F = 20:1
Boneand Soft-TissueDisorders 95
Histo: cartilagedegeneration+ destruction,synovial
proliferation+ pannus,destructionof sub-
articularbone+ proliferationof marginalbone
Distribution: symmetricpolyarticular disease
(resembling rheumatoidarthritis),
asymmetricpolyarticulardisease,
monoarticular disease
. rnore severe prolongedattacks
. may ulcerateexpressingwhitishchalkymaterial
Cx: tendon rupture,nervecompressioni paralysis
(4) Gouty nephropathy / nephrolithiasis
(a) Acute urate nephropathy
(b) Uricacid urolithiasis
0 May precedearthritisin up to 20.h of cases!
. renalhypertension
. isosthenuria (inability
to concentrateurine)
. proteinuria
. pyelonephritis
Cx: increasedincidenceof calciumoxalatestones
(uratecrystalsserve as a nidus)
Location:
(a) joints: hands+ feet (1st MTPjoint mostcommonly
affected= podagra)> ankles> heels> wrists
(carpometacarpal compartmentespecially
commonand severe)> fingers> elbows;knees;
shoulder;sacroiliacjoint (157", unilateral);
0 involvement of hip + spineis rare
(b) bones,tendon,bursa,bones
(c) externalear; pressurepointsover elbow,
forearms,knees,feet
Radiologic featuresusuallynot seen until6-12 years
after initialattack
Radiologicfeaturespresentin 45h of inflictedpatients
@ Soft tissues
r/ eccentricjuxtaarticularlobulatedsoft-tissuemasses
(hand,foot,ankle,elbow,knee)
Site: tendencyfor extensortendons,eg,
quadriceps,triceps,Achillestendon
{ calcificdepositsin peripheryof goutytophi in 50%
(sodiumuratecrystalsare not radiopaque,tophi
radiographically
visibleonly aftercalciumdeposition
of an underlyingabnormality of calciummetabolism)
{ bilateraleffusionof bursaeolecrani
(PATHOGNOMONIC), prepatellarbursa
{ auralcalcification
@ Joint
./ lointeffusion(earliestsign)
r/ periarticularswelling(in acutemonoarticular gout)
{ preservation of jointspaceuntillatein disease
(TMP OR TA NCTLU E ):
, r/ cartilagedestruction(latein courseof disease)
{ absenceof periarticular demineralization (dueto
short durationof attacks;importantDDx for
rheumatoidarthritis)
{ eccentricerosionswiththin scleroticmargins:
r/ scallopederosionof bases of ulnar metacarpals
 { chondrocalcinosis (5%): SARCOMA
GRANULOCYTIC
Location: menisci(fibrocartilageonly) = CHLOROMA = MYELOBLASTOMA
0 Patientswith gout have a predisposition for = extramedullary solidtumorconsistingof primitive
calciumpyrophosphatedihydratedeposition precursorsof the granulocyticseriesof WBCs
disease(CPPD) (myeloblasts,promyelocytes,myelocytes)
Cx: secondaryosteoarthritis Clinical setting:
r/ round/ oval well-marginated subarticularcysts (1) patientwith acutemyelogenous leukemia(in 3-8%)
(pseudotumor)up to 3 cm (containingtophus/ urate (2) harbingerof AML in nonleukemic patient(usually
crystal-richfluid) developingwithin1 year)
DDx: rheumatoidarthritis(marginalerosionswithout (3) indicatorof impendingblastcrisisin CML (in 1%)/
scleroticrim, periarticular
demineralization) leukemictransformationin myelodysplastic
@ Bone syndromes(polycythemiarubravera, myelofibrosis
r/ "punched-out"lyticbone lesion+ sclerosisof margin with myeloidmetaplasia, hypereosinophilic
= "mouse/ rat bite"from erosionof long-standing syndrome)
soft-tissuetophus (4) isolatedevent
{ "overhangingmargin"(40%)= elevatedosseous . 60/" are of greencolor(chloroma)due to highlevelsof
spiculeseparatingtophaceousnodulefrom adjacent myeloperoxidase (30% are whitelgray / brown
erosion(in intra-and extraarticularlocations) dependingon preponderanceof cell type + oxidative
(HALLMARK) state of myeloperoxidase)
r/ proliferativebone changes: Location: orbit,softtissue,skin,paranasalsinus,lymph
{ club-shapedmetatarsals,metacarpals,phalanges node,Periosteum, organs,bowel;
{ enlargement of ulnarstyloidprocess oftenmultiPle
r/ diaphysealthickening Site: propensityfor bone marrow(arisesfrom bone
r/ ischemicnecrosisof femoral/ humeralheads marrowtraversinghaversiancanal + reachingthe
^/ intraosseouscalcification : periosteum), perineural+ epiduraltissue
r/ punctate/ circularcalcificationsof subchondral/ { osteolysiswith ill-definedmargins
subligamentous regions(DDx:enchondroma) ^i homogeneous enhancement on CT / MR
./ bone infarctiondue to depositsat vascular (DDx to hematoma/ abscess)
basementmembrane(DDx:bone island) MR:
@ Kidney r/ isointense to brain/ bone marrow/ muscleon Tl Wl
{ renalstones(in up to 20"/"): + T2Wl
- pure uric acid stones $a%): radiolucenton Prognosis; resolutionunderchemotherapy+ radiation
radiographs,hyperdenseon CT therapy; recurrencerale of 23o/"
- uric acid + calciumoxalate(4%) DDx: osteomyelitis,histiocytosisX, neuroblastoma,
- pure calciumoxalate/ calciumphosphate(12%) lymphoma,multiPlemYeloma
MR:
r/ tophus(mostfrequently)isointenseto muscleon TlWl INJURY
GUNSHOT
./ low or intermediatesignalintensityon T2Wl Firearms: handgun,rifle (greatenergy),shotgun
^/ homogeneous intenseenhancement Projectiles:
(a) bul l et:
Rx: colchicine,allopurinol(effectivetreatmentusually jacketedbulletwith mantleof copper
does not improveroentgenograms) semijacketedbullet = exPosedlead at tip
DDx: nonjacketedbullet
(1) CPPD (pseudogoutsymptomatology, polyarticular (b) pelletsof steel/ lead:
chondrocalcinosis involvinghyalineand birdshot= srfldllpellets
fibrocartilage+ degenerativearthropathywith joint buckshot=largepellets
space narrowing)
(2) Psoriasis(progressivejoint space destruction,
paravertebralossification,sacroiliacjoint
involvement)
(3) Rheumatoidarthritis(nonproliferative
erosions,fusiformsoft-tissue
marginalbone
swelling,symmetric
distribution,earlyjoint-spacenarrowing,osteopenia)
(4) Joint infection(rapiddestructionof joint space,loss
of articularcortexover a continuoussegment)
bullet

charge
pellets
plastic
casmg
wadding
ffi
WW
1

primer
(5) Amyloidosis(bilateralsymmetricinvolvement, 2 5
periarticularosteopenia)
(6) Xanthomatosis(laboratorywork-up) Projectiles
(7) Osteoarthritis(symmetricdistribution,elderly I = fully jacketed rifle bullet, 2 = soft-point bullet,
women) 3 = hollow-point bullet
 Boneand Soft-Tissue
Disorders 97

Assessment: Histo: frequentlycontainingvariableamountsof

(1) Type of projectile
{ fullyjacketedbulletsshow no trailof lead
fragments
{ semi-/ nonjacketedbulletsdistributelead
fragmentsalong bullettrack
r/ hollow-pointbulletstransforminto mushroom
shape
r/ "leadsnowstorm"of high-velocitysoft-pointrifle
bullets:
r/ conicaldistributionwith apex pointingtoward
entry site
{ steel pelletsremainround,lead pelletsbecome
deformed+ fragmented
(2) Path of projectile
! bullettips pointsto entrywound(aftertumbling
t hr ough1 8 0 " ):
, ! impactdeformationof bulletmodifiestumbling
./ bultet+ bone fragmentsdepositedalongtrack
./ bone fracturebeveledtowardthe directionof
travel
(3) Extentof injury
Cx: pelletembolization, magnetizationin MRI
H EMANGIOENDOTHELIAL SARCOMA
= HEMANGIOENDOTHELIOMA = HEMANGIOEPITHELIOMA
= neopl€lsmof vascularendothelialcells of intermediate
aggressiveness with eitherbenignor malignantbehavior
Histo: irregularanastomosingvascularchannelslinedby
one / severallayersof atypicalanaplastic
endothelialcells
Age: 4th-5thdecade;M:F = 2:1
. historyof traumai irradiation
Soft-tissueHemangioendothelioma
(common)
Location: deep tissuesof extremities
Site: in 50% closelyrelatedto a vessel(oftena vein)
OsseousHemangioendothelioma
(rare)
Age: 2nd-3rd decadeof life; M > F
Location: calvarium,spine,femur,tibia,humerus,
pelvis;multicentriclesionsin 30% oftenwith
regionaldistribution(lessaggressive)
-bones
{ eccentriclesionin metaphysisof tong
r/ osteolyticaggressivelydeitructive area with indistinct
, margins(highgrade)
r/ well-demarcated marginswith scatteredbony
trabeculae(low grade)
r/ osteoblasticarea in vertebrae,contiguousthrough
severalvertebrae
Metastasesto: lung (early)
Prognosis: 26/" S-yearsurvivalrate
DDx: aneurysmalbone cyst, poorlydifferentiated
f ibrosarcoma,highlyvascularmetastasis,
alveolarrhabdomyosarcoma
HEM A NG I O M A
0 Most commonbenignsoft-tissuetumor of vascular
or igin!
nonvascular elements:fat, smoothmuscle,
fibroustissue,bone,hemosiderin, thrombus
0 Fat overgrowthmay be so extensivethat some
lesionmay be misdiagnosed as a lipoma!
A. CAPILLARYHEMANGIOMA(mostcommon)
= small-cdlibervesselslinedby flattenedepithelium
Site: skin,subcutaneous tissue;vertebralbody
Age: first few years of life
(a) JuvenilecapillaryhemangiorT't? = strawberrynevus
Prevalence: 1:200births;in20./. multiple
Prognosis: involutesin75-90% by age 7 years
(b) Verrucouscapillaryhemangioma
(c) Senilecapillaryhemangioma
r/ enlargedarteries+ arteriovenousshunting
{ poolingof contrastmaterial
B . C A V E R N OU SH E MA N GIOMA
= dilatedblood-filledspaceslinedby flattened
endothelium
Site: deepersofttissues,frequentlyintramuscular;
calvarium
Age: childhood
r/ phleboliths= dystrophiccalcification
in organizing
thrombus(in nearly50%)
r/ largecysticspaces
r/ enlargedarteries+ arteriovenousshunting
{ poolingof contrastmaterial
Prognosis; NO involution
C . A R TE R IOV E N OUH SE MA N GIOMA
= persistenceof fetal capillarybed with abnormal
communications of an increasednumberof normal/
abnormalarteriesand veins
Etiology: (?)congenitalarteriovenousmalformation
Age: young patients
Site: soft tissues
(a) superficiallesionwithoutarteriovenous shunting
(b) deep lesionwith arteriovenousshunting
. limbenlargement, bruit
. distendedveins,overlyingskinwarmth
. Branhamsign = reflexbradycardiaafter
compression
r/ largetortuousserpentinefeedingvessels
r/ fast bloodflow + dense staining
{ earlydrainingveins
D. VENOUSHEMANGIOMA
= thick-walledvesselscontainingmuscle
Site: deep softtissuesof retroperitoneum,
mesentery,musclesof lowerextremities
Age: adulthood
r/ t pnteootiths
ri serpentinevesselswith slow bloodflow
{ vesselsorientedalong long axis of extremity(in
78%) + neurovascularbundle(in 64%)
{ multifocalinvolvement(in g7V.)
r/ muscleatrophywith increasedsubcutaneousfat
! may be normalon arterialangiography
98 RadiologyReviewManual
OsseousHemangioma
lncidence: 10%
Histo: mostlycavernous;capillarytype is rare
Age: 4th-sth decade;M:F = 2:1
. usuallyasymptomatic
@ Vertebra(28/" of all skeletalhemangiomas)
lncidence; in 5-1 1'h of all autopsies;multiplein 1/3
Histo: capillaryhemangiomainterspersedin fatty
matrix
0 The largerthe degreeof fat overgrowth,the less
likelythe lesionwill be symptomatic!
Age: >40 years;female
Location: in lowerthoracic/ upperlumbarspine
"honeycomb"vertebra
{ "accordion"I"corduroy"/
= codfseverticaltrabeculaewith osseous
reinforcementadjacentto bone resorptioncaused
by vascularchannels(alsoin multiplemyeloma,
lymphoma,metastasis)
^/ butgeof posteriorcortex
{ extraosseousextensionbeyondbony lesioninto
spinalcanal (withcord compression)/ neural
foramina
! paravertebralsoft-tissueextension
r/ lesionenhancement(dueto hypervascularity)
CT:
r/ polka-dotappearaflc€= small punctateareasof
sclerosis(= thickenedverticaltrabeculae)
MR:
r/ mottledpatternof low-to-highintensityon TlWl
+ very-highintensityon T2Wl dependingon
degreeof adiposetissue(CHARACTERISTIC)
Cx: vertebralcollapse(unusual),spinalcord
compression
@ Calvarium(20% of all hemangiomas)
Location: frontal/parietalregion
Site: diploe
{ <4 cm roundosteolyticlesionwith sunburst/
weblike/ spoke-wheelappearanceof trabecular
thickening
r/ expansionof outertable to a greaterextentthan
innertableproducingpalpablelump
@ Flat bones & long bones (rare)
ribs,clavicle,mandible,zygoma,nasalbones,
metaphyseal ends of longbones(tibia,femur,
humerus)
{ radiatingtrabecularthickening
r/ OuOOly bone lysiscreatinghoneycomb/ latticelike/
"hole-within-hole"appearance
MR:
{ serpentinevascularchannelswith low signal
intensityon Tl Wl + highsignalintensityon
T2Wl (= slow btoodflow)/ low signalintensity
on all sequences(= high bloodflow)
NUC (bone/ RBO-labeled scintigraphy):
{ photopenia/ moderateincreasedactivity
ioma
Soft-tissueHemang
lncidence: 7o/"of all benigntumors;most frequent
tumor of infancy+ childhood
Age: primarilyin children;M < F
May be associated with:
Maffucisyndrome(= multiplecavernous
hemangiomas + enchondromas)
. intermittent changein size
' Painful
. bluishdiscoloration of overlyingskin (rare)
. may dramatically increasein sizeduringpregnancy
Location: usuallyintramuscular; synovia(<1"/"of all
hemangiomas);common in headand neck
{ nonspecific soft-tissue mass
r/ may extendintobonecreatingsubtleroundedi linear
areas of hyperlucency(rare)
{ t longitudinal/axialbone overgrowth(secondary to
chronichyperemia)
{ may containphleboliths (30%of lesions,SPECIFIC)
r/ nonspecific curvilinear / amorphouscalcifications
{ may containsuch largeamountsof fat as to be
indistinguishable from liPoma
CT:
r/ poorlydefinedmass with attenuationsimilarto
muscle
r/ areasof decreasedattenuationapproximating
subcutaneousfat (= fat overgrowth)most prominent
in peripheryof lesion
MR:
r/ poorlymarginatedmass hypo-/ isointenseto
muscl eon Tl W l
r/ areaswith increasedsignalintensityon TlWl in
peripheryof lesionextendinginto septations(= fs1;
r/ well-marginated markedlyhyperintense masson
T2Wl (increasedfree water contentin stagnant
blood)with striated/ septatedconfiguration
r/ tubularstructureswith bloodflow characteristics
(ftowvoid / inflowenhancement;markedcontrast
enhancement)
r/ phleboliths as low-intensityareasinsidelesion
r/ nign-signal-intensity areason TlWl + T2Wl
(= hemorrhage)
US:
{ complexmass
r/ low-resistance arterialsignal(occasionally)
Synovial Hemangioma
. repetitivebleedingintojoint
Location: knee (60%),elbow (30%)
DDx: hemophilicarthropathy(polyarticular)
HEMANGIOPERICYTOMA
= borderlinetumorwith benign/ locallyaggressive/
malignantbehavior(counterpart of glomustumor)
Age: 4th-sth decade; M:F = 1:1
Path: largevesselspredominantlyin tumor periphery
Histo: cells packedaroundvascularchannelscontaining
cystic+ necroticareas;arisingfrom cells of
Zimmermanthat are locatedaroundvessels
@ Soft tissue
= deep-seated well-circumscribed lesionarisingin
muscle
Location: lowerextremityin 35% (thigh),pelviccavity,
retroPeritoneum
 Disorders gg
Boneand Soft-Tissue

. painlessslowlygrowingmass up to 20 cm Cx: hepatoma(in 30%)

@ Bone (rare) Prognosis; deathfrom CHF (30%),deathfrom hepatic
Location: lowerextremity,vertebrae,pelvis,skull failure(25%)
(durasimilarto meningioma) DDx: (1) Pseudogout(no arthropathy)
r/ osteolyticlesionsin metaphysisof long lflat bone (2) Psoriaticarthritis(skin+ nail changes)
r/ subperiosteal largeblowoutlesion(similarto (3) Osteoarthritis(predominantly distaljoints in
aneurysmalbone cyst) hands)
Angio: (4) Rheumatoidarthritis
ri displacementof main artery (5) Gout (mayalso havechondrocalcinosis)
! pedicleof tumor feederarteries
! spider-shaped arrangementof vesselsencirclingtumor H E MOP H ILIA
{ smallcorkscrewarteries = X-linkeddeficiencyi functionalabnormality of
{ densetumorstain coagulationfactorVlll (= hemophiliaA) in >80%I factor
DDx: hemangioendothelioma, angiosarcoma lX (= 6srophilia B = Christmasdisease)
lncidence.' 1:10,000males

HEMOCHROMATOSIS Hemophilic Arthropathy (mostcommon)

1 . P RI M A RYHE M OC H R O MA T O S IS Cause: repeatedbleedingintosynovialjoint
= autosomalrecessivei indeterminateinheritance Path: pannusformationerodescartilagewith loss of
(abnormaliron-loadinggene) in thalassemia, subchondralbone pfateand formationof
sideroblasticanemia subarticularcysts
2. SECONDARYHEMOCHROMATOSIS Histo: synovialhyperplasia,chronicinflammatory
= eXcessive ironabsorptionin anemias,myelofibrosis, changes,fibrosis,siderosisof synovial
portacavalshunt,exogenousadministration of iron, membrane
porphyriacutaneatarda,beer brewedin iron vessels Age: 1st and 2nd decade
+ depositionof excessiveiron in liver,pancreas, . tense red warm joint with decreasedrangeof motion
spleen,Gl tract,kidney,gonads,heart,endocrine (musclespasm)
glands(pituitary,hypothalamus) . fever,elevatedWBC (DDx:septicarthritis)
Age: >40 years;M:F = 10:1(femalesprotectedby
menstruation) Location:knee > ankle> elbow > shoulder;commonly
. cirrhosis
bilateralalthoughbleedingepisodestend to
. "bronzeddiabetes"
recurwithinsamejoint
. congestiveheartfailure
i/ loint effusion(= hemarthrosis)
. skin pigmentation
{ enlargementof epiphysis(secondaryto synovial
. hypogonadism
inflammation+ hyperemia)
. arthriticsymptoms(30%) r/ juxtaarticularosteoporosis(secondaryto synovial
. increasein serumiron
inflammation + hyperemia)
@ Skeleton r/ loint space narrowing(particularlypatella)secondary
Site: mostcommonlyin hands(metacarpal heads, to cartilaginousdenudation
particularly2nd + 3rd MCp joints),carpal ri erosionof articularsurfacewith multiplesubchondral
proximalinterphalangeal joints,knees,hips cysts
ri generalizedosteoporosis
{ smallsubchondralcystlikerarefactionswith fine rim
of sclerosis(metacarpalheads)
r/ arthropathyin 50% with iron depositionin synovium bulbous
ri uniformjoint space narrowing widened condyles
r/ enlargementof metacarpalheads intercondylar
ri eventuallyosteophyteformation notch
r/ chondrocalcinosis in >G07o,kneesmostcommonly
affected
(a) calciumpyrophosphate deposition(inhibition of
pyrophosphatase enzymewithincartilagewhich
hydrolyzespyrophosphateto soluble
orthophosphate)
(b) calcificationof triangularcartilageof wrist,
menisci,annulusfibrosus,ligamentumflavum,
symphysispubis,Achillestendon,plantarfascia
@ B r ainM RI : slanting of squared
r/ markedloss in signalintensityof anteriorlobe of tibial plateau patella
pituitarygland(irondeposition) HemophiliacArthritis
 { sclerosis+ osteophytosis(secondaryto { wide irregularepiphyseallines(resembling ricketsin
superimposeddegenerativejoint disease) childhood),persistentmetaphysealdefects(40% of
@ Knee adults)
r/ "squared"patella r/ bowingof long bones + fractureswith irregularcallus
r/ wideningof intercondylarnotch r/ widenedmedullarycanalwithcorticalthinning(cortex
./ flatteningof condylarsurface modeledfrom trabecularbone)
r/ medial"slanting"of tibiotalarjoint { skull greatlythickenedwith wide tables,cottonwool
MR: appearance
r/ low signalintensityof hypertrophiedsynovial { vertebraplana
membraneon all pulsesequences(dueto magnetic OB -U S :
susceptibilityeffectof hemosiderin) ./ diagnosissuspectedin utero in 20/o
r/ varyingintensityof subarticulardefects(depending Cx: pathologicfractures;vertebraplana universalis
on substrate:fluid/ soft tissueI hemosiderin) DDx: (1) OsteogenesisimPerfecta
(2) Polyostoticfibrousdysplasia
Hemophilic Pseudotumor (1-2o/.\ (3) Pagetdisease(> age 20, not generalized)
= posthemorrhagic cysticswellingwithinmuscle+ bone (a) Pyle disease(sparesmidshaft)
characterizedby pressurenecrosis+ destruction (5) van Buchemsyndrome(onlydiaphyses> age
(a)juvenileform = usuallymultipleintramedullary 20, no long-bonebowing)
expansilelesionswithoutsoft-tissuemass in small (6) Engelmannsyndrome(lowerlimbs)
bones of hand / feet (beforeepiphysealclosure)
(b) adultform = usuallysingleintramedullary expansile HEREDITARY SCLEROSIS
DIAPHYSEAL
MULTIPLE
lesionwith largesoft-tissuemassin ilium/ femur = R IB B IN D
GIS E A S E
(c) soft-tissueinvolvementof retroperitoneum (psoas = autosomalrecessivedisordersimilarto Engelmann-
muscle),bowelwall, renalcollectingsystem Camuratidisease
r/ mixedcysticexpansilelesion Age: after puberty
r/ bone erosion+ pathologicfracture . mild neuromuscular symptoms
CT: Location: longbonesonlY
{ sometimesencapsulatedmass containingareas of ! unilateralasymmetric/ asynchronousbilateralsclerosis
low attenuation+ calcifications DDx: Engelmann-Camurati disease(beginsin childhood,
MR: severeneuromuscularsymptoms,symmetric
{ hemorrhageof varyingage bilateralsclerosisof long bones,skull involved)
Cx: joint contracture(afterrepeatedbleedinginto
muscle) SPHEROCYTOSIS
HEREDITARY
N.B.: Needleaspiration/ biopsy/ excisionmay cause = autosomaldominantcongenitalhemolyticanemia
fistulae/ infection/ uncontrolledbleeding! Age: anemiabeginsin early infancyto late adulthood
Rx: palliativeradiationtherapy(destroysvessels . rarelysevereanemia
proneto bleed)+ transfusionof procoagulation . jaundice
factor concentrate . spherocytesin periPheralsmear
{ bonechangesrare (dueto mildanemia);longbones
rarely affected
HYPERPHOSPHATASIA
HEREDITARY { wideningof diploewithdisplacement + thinningof outer
= "JUVENILE PAGETDISEASE" table
= fore autosomalrecessivediseasewith sustained { hair-on-endappearance
elevationof serumalkalinephosphatase, especiallyin Rx: splenectomycorrectsanemiaeven though
individualsof PuertoRicandescent spherocytemiapersists
Histo: rapidturnoverof lamellarbone withoutformation r/ improvementin skeletalalterationsfollowing
of corticalbone; immaturewoven bone is rapidly splenectomy
laid down, but simultaneousrapiddestruction
preventsnormalmaturation
Age: 1st-3rd yeari usuallystillborn HERNIATION PIT
. rapidenlargement of calvarium+ long bones = SYNOVIAL HERNIATION PIT= CONVERSION DEFECT
. dwarfism = ingrowthof fibrous+ cartilaginouselementsfrom
. cranialnervedeficit(blind,deaf) adjacentjoint throughperforationin cortex
. hypertension Histo: fibroalveolartissue
. frequentrespiratoryinfections Age: usuallyin olderindividuals
. pseudoxanthoma elasticum . may be symptomatic
. elevatedalkalinephosphatase . no clinicalsignificance
{ deossification= decreaseddensityof long boneswith Location: anteriorsuperolateralaspectof proximal
coarsetrabecularpattern femoralneck; uni- or bilateral
r/ metaphysealgrowthdeficiency Site: subcortical
 Boneand Soft-Tissue
Disorders l0l

MartanSyndrome Homocystinuria
Inheritance autosomal autosomal
dominant recessive
Biochemicaldefect not known cystathionine
synthetase
Osteoporosis no yes
Spine scoliosis biconcave
vertebrae
Lens dislocation upward downward
Herniation Pit on AP and LAT radiographs Arachnodactyly 100% gg%

r/ well-circumscribed roundlucency r/ enlargedparanasalsinuses

{ usually<1 cm in diameter;mayenlargeover time r/ osteoporosisof vertebrae(biconcave/ flattened/
r/ reactivethin scleroticborder widenedvertebrae)
r/ hyperintensearea on T2Wl (= fluid signalintensity) r/ scoliosis
r/ bone scan may be positive r/ pectusexcavatum/ carinatum(75%)
1 osteoporosisof long bones (75%)with bowing+ fracture
HOLT.ORAM
SYNDROME ! children:metaphyseal cupping(50%);enlargement of
Autosomaldominant;M < F ossificationcentersin 50% (knee,carpalbones);
Associatedwith CHD: secundumtype ASD (most epiphysealcalcifications(esp. in wrist,resembling
common),VSD, persistentleft phenylketonuria); delayedossification
SVC, tetralogy,coarctation ! Harrislines= multiplegrowthlines
. intermittentcardiacarrhythmia { genu valgum,coxa valga,coxa magna,pes cavus
. bradycardia(50-60/min) r/ prematurevascularcalcifications
Location: upperextremityonly involved;symmetryof Prognosis; death from occlusivevasculardisease/
lesionsis the rule; left side may be more minorvasculartrauma
severelyaffected
r/ aplasia/ hypoplasiaof radialstructures:thumb, HYPERPARATHYROIDISM
1st metacarpal,carpalbones,radius = Uflcor'ttfolled
productionof parathyroidhormone
r/ "fingerized"
hypoplastic thumb/ triphalangeal thumb Age: 3rd-5thdecade;M:F = 1:3
{ slenderelongatedhypoplasticcarpals+ metacarpals Histo: decreasedbone mass secondaryto increased
r/ hypoplasticradius;absentradialstyloid numberof osteoclasts,increasedosteoidvolume
r/ shallowglenoidfossa (voluntarydislocationof shoulder (defectin mineralization),
slightlyincreased
, common) numberof osteoblasts
{ hypoplasticclavicula . increasein parathyroid hormone(100%)
r/ nigh archedpalate . increasein serum alkalinephosphatase(50%)
r/ cervicalscoliosis . elevationof serum calcium(due to acceleratedbone
r/ pectus excavatum turnoverand increasedcalciumabsorption)
+ decreasein serum phosphate(30%)
HOMOCYSTINURIA . hypotonicityof muscles,weakness,constipation,
Autosomalrecessivedisorder difficultyin swallowing,duodenal/ gastricpepticulcer
Etiology: cystathionineB synthetasedeficiencyresults disease(secondaryto hypercalcemia)
in defectivemethioninemetabolismwith . polyuria,polydipsia(hypercalciuria+ hyperphosphaturia)
accumulationof homocystine+ homocysteine . renalcolic+ renalinsufficiency (nephrocalculosis
in bloodand urine;causesdefectin collagen/ + nephrocalcinosis)
elastinstructure . rheumaticbone pain + tenderness(particularlyat site of
. thromboembolic phenomenadue to stickinessof browntumor),pathologicfracturesecondaryto brown
platelets tumor
. ligamentouslaxity
. downward+ inwarddislocationof lens (DDx: upward A. BONE RESORPTION
+ outwarddislocationin Marfansyndrome) (a) subperiosteal(mostconstant+ specificfinding;
. mild / moderatemental retardation virtuallyPATHOGNOMONICof
. crowdingof maxillaryteeth and protrusionof incisors hyperparathyroid ism):
. malarflush r/ lacelikeirregularityof corticalmargin;may
r/ arachnodactylyin 1/3 (DDx: Marfansyndrome) progressto scalloping/ spiculation
r/ microcephaly (pseudoperiostitis)
 Radiology Review Manual

Site: phalangealtufts (earliestinvolvement),radial Location: jaw, pelvis,rib, metaphysesof longbones

aspectof middlephalanxof 2nd + 3rd finger (femur),facialbones,axialskeleton
beginningin proximalmetaphyseal region Site: often eccentric/ cortical;frequentlysolitary
(earlyinvolvement), bandlikezone of { expansilelyticwell-marginated cystlikelesion
resorptionin middle/ base of terminaltuft, (DDx:giant cell tumor)withoutadjacentreactive
distalend of clavicles,medialtibiaplateau, boneformation
medialhumerusneck,medialfemoralneck, r/ endostealscalloping
distalulna,superior+ inferiormarginsof ribs { destructionof midportionsof distalphalangeswith
in midclavicular line,laminadura of skulland telescoping
teeth
(b) subchondral: D. OSTEOSCLEROSIS
^/ pseudowidening of joint space Morefrequentin 2' HPT
r/ collapseof corticalbone + overlyingcartilagewith Cause: ? PTH-stimulated osteoblasticactivity,
development of erosion,cyst,jointnarrowing ? role of calcitonin(poorlyunderstood)
(similarto rheumatoidarthritis) Site: strongpredilection for axialskeleton,pelvis,
Site: DIPjoint (mostcommonly4th + 5th digit), ribs,clavicles,metaphysis+ epiphysisof
MCPjoint,PIPjoint,distalclavicle, appendicular skeleton
acromioclavicular joint(clavicular side), { "ruggerjerseyspine"
"pseudowidening" of sacroiliacjoint (iliac
side),sternoclavicular joint,symphysispubis, E. SOFT-TISSUE CALCIFICATION
"scalloping"of posteriorsurfaceof patella, Morefrequentin 2" HPT; metastaticcalcificationwhen
Schmorlnodes;typicallypolyarticular Ca x P product>70 mg/dl
(c) cortical(due to osteoclasticactivitywithinhaversian (a) cornea,viscera(lung,stomach,kidney)
canal): (b) periarticularin hip,knee,shoulder,wrist
r/ intracortical tunneling (c) arterialtunica media (resemblingdiabetesmellitus)
r/ scallopingalong innercorticalsurface(endosteal (d) chondrocalcinosis (15-l 8/o) = calcification
of
resorption) hyaline/ fibrouscartilagein menisci,wrist,
(d) trabecular: shoulder,hip,elbow
r/ spottydeossification with indistinct+ coarse
trabecularpattern F. EROSIVEARTHROPATHY
ri granularsalt and pepperskull . asymptomatic
^/ loss of distinctionbetweeninnerand outertable r/ simulatesrheumatoidarthritiswith preservedjoint
^/ ground-glassappearance spaces
(e) subligamentous: G. PERIOSTEALNEW-BONEFORMATION
r/ bone resorptionwith smoothscalloped/ irregular Cause: PTH-stimulationofosteoblasts
ill-defined margins Site: pubicramusalongiliopectineal line (most
Site: inferiorsurfaceof calcaneus(longplantar frequent),humerus,femur,tibia,radius,ulna,
tendons+ aponeurosis),inferioraspectof metacarpals,metatarsals,phalanges
distalclavicle(coracoclavicular ligament), ./ linearnew bone parallelingcorticalsurface;may be
greatertrochanter(hip abductors),lesser laminated;often separatedfrom cortexby
trochanter(iliopsoas), anteriorinferioriliac radiolucentzone
spine(rectusfemoris),humeraltuberosity r/ increasein corticalthickness(if periostealreaction
(rotatorcuff),ischialtuberosity(hamstrings), becomesincorporatedinto adjacentbone)
proximalextensorsurfaceof ulna
(anconeus),posteriorolecranon(triceps) Sequelae:
1. Renalstones/ nephrocalcinosis (70%)
B . B O NES O FT EN IN G 2. Increasedosteoblasticactivity(25%)
{ basilarimpressionof skull . increasedalkalinePhosPhatase
r/ wedgedvertebrae,kyphoscoliosis, biconcave (a) osteitisfibrosacYstica
vertebraldeformities r/ subperiostealbone resorption+ cortical
r/ bowingof long bones tunnel i ng
r/ slippedcapitalfemoralepiphysis { browntumors(PrimarYHPT)
c. BROWNTUMOR (b) bone softening
= OSTEOCLASTOMA r/ fractures
Cause: PTH-stimulated osteoclasticactivity(more 3. Pepticulcerdisease(increasedgastricsecretion
o f 2 " H P T)
f r e q u e nitn 1 " H P T ;i n 1 .5 o /o from gastrinoma)
Path: localizedreplacementof bone by vascularized 4. Calcificpancreatitis
fibroustissue (osteitisfibrosacystica) 5. Soft-tissuecalcifications(2" HPT)
containinggiantcells;lesionsmay become 6. Marginaljointerosions+ subarticular collapse(DlP'
cysticfollowingnecrosis+ liquefaction P l P ,M C P )
 Boneand Soft-Tissue
Disorders l0g

PrimaryHyperparathyroidism
= pHPT = 1oHPT = hypercalcemia due to uncontrolled Skeletal Findings 1" HPT 2. HpT
secretionof parathormoneby one / more Osteopenia, diffuse present present
hyperfunction ing parathyroid glandsfeaturing
Osteosclerosis,
( 1) br ownt u m o r
regional/diffuse rare common
(2) chondrocalcinosis (20-90%)
0 requiressurgicalRx Boneresorption common common
lncidence: 25:100,000per year;incidenceof bone Browntumor common lesscommon
lesionsin HpT is 25-40o/o Soft-tissue
calcification notinfrequent common
Etiology:
(1) Parathyroid adenoma(87.h):single(gO%); Chondrocalcinosis notinfrequent rare
multiple(7%)
(2) Parathyroid hyperptasia (10%): chiefceil (5%); (b) calciumdeprivation,maternalhypoparathyroidism,
clearcell (5%) pregnancy,hypovitaminosis D
(3) Parathyroidcarcinoma(3%) (c) rise in serum phosphateleadingto decreasein
Histo: increasednumberof osteoclasts,increased calciumby feedbackmechanism
osteoidvolume(defectin mineralization), . low to normalcalciumlevels
slightlyincreasedosteoblasts= decreasedbone o Qsz+POo,-solubility productoftenexceeded
MASS NUC:
Age: 3rd-Sthdecade; M:F = 1:3 { "superscan" in 2" HPT:
Associated with: r/ absentkidneysign
(a) Wermersyndrome= MEA | (+ pituitaryadenoma r/ increasedbone-to-softtissueuptakeratio
+ pancreaticisletcelltumor) ./ increaseduptakein calvarium,mandible,
(b) Sipplesyndrome= MEA ll (+ medullarythyroid acromioclavicular region,sternum,vertebrae,
carcinoma + pheochromocytoma) distalthirdof longbones,ribs
X-RAY (skeletalinvolvementin 10-20%): ./ diffuseTc-99m MDp uptakein tungs(60%)
r/ thin corticeswith lacy corticalpattern(subperiosteal
bone resorption) TertiaryHyperparathyroidism
r/ browntumor(particularly in jaw + long bones) = IHPT = 3" HPT = development of autonomouspTH
r/ osteitiscysticafibrosa(= intertrabecular fibrous adenomain patientswith chronicallyoverstimulated
connectivetissue) hyperplasticparathyroidglands (renalinsufficiency
NUC: + prolongedrenaldialysis)
r/ normalbone scan in 80% 0 requiressurgicalRx
r/ foci of abnormaluptake: calvarium(especially Clue: (a) intractablehypercalcemia
periphery),mandible,sternum,acromioclavicular (b) inabilityto controlosteomalaciaby vitaminD
. joint,lateralhumeralepicondyles, hands administration
r/ increaseduptakein browntumors
r/ extraskeletaluptake: cornea,cartilage,joint EctopicParathormone
Production
capsules,tendons,periarticular areas,lungs, = pseudohyperparathyroidism
as paraneoplastic
stomach syndromein bronchogenic
carcinoma+ renalcell
r/ normalrenalexcretion[exceptin stone disease/ carcinoma
calciumnephropathy (10%)l
Rx: pathologicglandsidentifiedby experienced
surgeonsin 90-95% on initialneckexploration HYPERTROPHIC
OSTEOARTH
ROPATHY
(ectopic+ supernumerary glandsoftenoverlooked = HYPERTROPHIC PULMONARY OSTEOARTHROPATHY
at operation;recurrenthypercalcemia in 3-10%) Etiology: (1) Releaseof vasodilatorswhich are not
Surgicalriskfor repeatsurgery metabolizedby lung
6.6% recurrentlaryngealnerve injury (2) IncreasedflowthroughAV shunts
20.0/o permanenthypoparathyroidism (3) Reflexperipheralvasodilation(vagal
<1.0o/o perioperativemortality impulses)
(4) Hormones:estrogen,growthhormone,
SecondaryHyperparathyroidism prostaglandin
= sHPT = 2o HPT = diffuse/ adenomatous hyperplasia A. THORACICCAUSES
of all four parathyroidglandsas a compensatory (a) malignanttumor (0.7-1 2o/"): bronchogenic
mechanismin any state of hypocalcemiafeaturing carcinoma(88%),mesothelioma, lymphoma,
(1) soft-tissuecalcifications(2) osteosclerosis pulmonarymetastasisfrom osteogenicsarcoma,
0 requiresmedicalRx melanoma,renalcell carcinoma,breastcancer
Etiology: (b) benigntumor: benignpleuralfibroma,tumorof ribs,
(a) renalosteodystrophy(renalinsufficiency thymoma,esophagealleiomyoma,pulmonary
+ osteomalacia/ rickets) hemangioma, pulmonarycongenitalcyst
 (c) chronicinfection/ inflammation:pulmonary
abscess,bronchiectasis, blastomycosis, TB (very
rare);cysticfibrosis,interstitialfibrosis
(d) congenitalheartdiseasewith R-to-Lshunt
B. EXTRATHORACIC CAUSES
(a) Gl tract: ulcerativecolitis,amebic+ bacillary
dysentery,intestinalTB, Whippledisease,Crohn
disease,gastriculcer,bowellymphoma,gastric
carcinoma
(b) liverdisease:biliary+ alcoholiccirrhosis,
posthepaticcirrhosis,chronicactivehepatitis,bile
duct carcinoma,benignbileduct stricture,
amyloidosis,liverabscess
(c) undifferentiatednasopharyngeal carcinoma,
pancreaticcarcinoma,chronicmyelogenous
leukemia
. burningpain,painfulswellingof limbs,and stiffnessof
joints: ankles(88%),wrists(83%),knees(75%),elbows
(17"/"),shoulders(10%),fingers(7%)
. peripheralneurovascular disorders:localcyanosis,
areasof increasedsweating,paresthesia,chronic
erythema,flushing+ blanchingof skin
. hippocraticfingers+ toes (clubbing)
. hypertrophyof extremities(softtissueswelling)
Location: tibia+ fibula(75'/"),radius+ ulna (80%),
proximalphalanges(60%),femur(50%),
metacarpus+ metatarsus(40%),humerus
+ distalphalanges(25%),pelvis(5%);
unilateral(rare)
Site: in diametaphyseal regions
{ corticalthickening
{ lamellarperiostealproliferationof new bone,at first
smooththen undulating+ rough
Site: most conspicuouson concavityof long bones
(dorsal+ medialaspects)
{ soft-tissueswelling("clubbing")of distalphalanges
r/ regressionof periostealreactionafterthoracotomy
Bone scan (revealschangesearlywith greatersensitivity
+ clarity):
r/ symmetricdiffuselyincreaseduptake along cortical
marginsof diaphysis+ metaphysisof tubularbones of
the extremitieswith irregularities
{ increasedperiarticularuptake(= sYnovitis)
r/ scapularinvolvementin 2/3
{ mandible+ maxillaabnormalin 40%
{ symmetricalsolid periostealnew-boneformationalong
shaftsof long+ shortbones(ulna,clavicle)
{ prematureepiphysealclosure+ thinningof epiphyseal
plates
r/ acceleratedgrowth
r/ tendinous,ligamentous,pericapsularcalcifications
./ changesusuallydisappearaftercessationof vitaminA
ingestion
DDx: infantilecorticalhyperostosis(mandibleinvolved)
HYPERVITAMINOSIS D
= eXoessiveingestionof vitaminD (largedoses act like
parathormone)
' loss of appetite,diarrhea,drowsiness,headaches
. polyuria,polydipsia, renaldamage
. convulsions
. excessivephosphaturia(parathormonedecreases
tubularabsorption)
hypercalcemia + hypercalciuria;anemia
{ deossification
{ wideningof provisionalzone of calcification
./ cortical+ trabecularthickening
./ alternatingbands of increased+ decreaseddensitynear
/ in epiphysis(zoneof provisional calcification)
vertebraoutlinedby dense band of bone + adjacent
radiolucent linewithin
r/ dense calvarium
r/ metastaticcalcinosisin
(a) arterialwalls (betweenage 20 and 30)
(b) kidneys= nePhrocalcinosis
(c) periarticulartissue(puttylike)
(d) prematurecalcificationof falx cerebri(most
consistentsign!)
HYPOPARATHYROIDISM
. tetany= hypocalcemic neuromuscularexcitability
(numbness,cramps,carpopedalspasm,laryngeal
stridor,generalizedconvulsions)
. hypocalcemia+ hyperphosphatemia
. normal/ low serum alkalinephosphatase
r/ prematureclosureof ePiPhYses
rl hypoplasiaof tooth enamel+ dentine;bluntingof roots
r/ generalizedincreasein bonedensityin 9oh:
r/ localizedthickeningof skull
HypoPT Pseudo Pseudopseudo
HypoPT HypoPT

SerumCa \P J e >
HYPERVITAMINOSIS A SerumP t t €
Age: usuallyinfants+ children AlkaPhos J or <-+ Jor<+ €
Cause: overdosingvitaminA, 13-cis-retinoic acid
(treatmentfor neuroblastoma)
. anorexia,irritability Responseto
. lossof hair,dry skin,pruritus,fissuresof lips PTH-lnjection Norm/ HypoPT PseudoHypoPT
. jaundice,enlargement of liver
UrineAMP T e)
r/ separationof cranialsuturessecondaryto U ri neP t
/l.
e
hydrocephalus (coronal> lambdoid)in children PlasmaAMP I e
<10 years of age, may appearwithina few days
 Boneand Soft-Tissue
Disorders l0S

r/ sacroiliacsclerosis r/ poorlymineralized spine

r/ bandlikedensityin metaphysisof long bones (25/o\, r/ short poorlyossifiedribs
iliaccrest,vertebralbodies r/ polyhydramnios
r/ thickenedlaminadura (innertable)+ wideneddiploe Prognosis; death within 6 months
^/ deformedhips with thickening+ sclerosisof femoral B. GROUp ll = juvenilesevereform
head + acetabulum onset of symptomswithinweeks to months
@ Soft tissue . moderate/ severedwarfism
r/ intracranialcalcificationsin basalganglia,choroid . delayedweightbearing
plexus,occasionally in cerebellum ! resemblesrickets
r/ calcificationof spinaland other ligaments r/ separatedcranialsutures;craniostenosisin 2nd year
r/ subcutaneouscalcifications Prognosis: 50/"mortality
r/ ossificationof muscleinsertions C . GR OU Pl l l = adul tmi l dform
r/ ectopicbone formation recognizedlater in childhood/ adolescence/ adulthood
. dwarfism
ldiopathicHypoparathyroidism { clubfoot,genu valgum
= f?ro conditionof unknowncause r/ demineralization of ossificationcenters(at birth/
. roundface, shortdwarflike,obese 3-4 monthsof age):
. mentalretardation r/ widenedmetaphyses
' cataracts r/ wormianbones
. dry scaly skin, atrophyof nails Prognosis; excellent;after 1 year no further
. dentalhypoplasia(delayedtooth eruption,impaction progresston
of teeth,supernumerary teeth) D. GROUPlV = latentform
heterozygousstate
SecondaryHypoparathyroid
ism . normal/ borderlinelevelsof alkalinephosphatase
= accidentalremoval/ damageto parathyroidglands in . patientsare smallfor age
thyroidsurgery lradical neck dissection(5%); . disturbance of primarydentition
l-131therapy(rare);externalbeam radiation; r/ bone fragility+ healedfractures
hemorrhage; infection; thyroidcarcinoma; r/ enlargedchondralends of ribs
hemochromatosis (irondeposition) r/ metaphysealnotchingof long bones
! Erlenmeyer flaskdeformityof femur
HYPOPHOSPHATASIA
= autosomalrecessivecongenitaldiseasewith low activity
of serum-,bone-,liver-alkaline
phosphatase resultingin HYPOTHYROIDISM
poor mineralization
(deficientgenerationof bone A . C hi l dhood = C R E TIN IS M
crystals) Frequency; 1:4,000live birthshave congenital
lncidence; 1:100,000 hypothyroidism
Histo: indistinguishable
from rickets Cause: sporadichypoplasia/ ectopiaof thyroid
. phosphoethanolamine in urineas precursorof alkaline ! delayedskeletalmaturation(appearance+ growthof
phosphatase ossificationcenters,epiphysealclosure)
. normalserumcalcium+ phosphorus r/ fragmentedstippledepiphyses
r/ wide sutures/ fontanelswith delayedclosure
A. GROUP | = fleoflatal= congenitallethalform r/ delayeddentition
r/ markeddemineralization of calvarium(*caput { delayed/ decreasedpneumatization of sinuses
membranaceum" = soft skull) + mastoids
r/ lact<of calcificationof metaphysealend of long r/ hypertelorism
bones r/ densevertebralmargins
r/ streakyirregularspotty marginsof calcification r/ demineralization
I cuppingof metaphysis r/ hypoplasticphalangesof Sth finger
! angulatedshaftfractureswith abundantcallus MR:
formation { reducedmyelination of brain(usuallybeginning
r/ short poorlyossifiedribs duringmidgestation)
r/ poorlyossifiedvertebrae(especiallyneuralarches) OB -U S :
r/ small pelvicbones ./ tetal goiter(especiallyin hyperthyroidmothers
O B - US : treatedwith methimazole/ propylthiouracif I l-191)
^/ nighincidenceof intrauterine fetaldemise B. Adulthood
r/ increasedechogenicityof falx (enhancedsound { catuarialthickening/ sclerosis
transmissionsecondaryto poorlymineralized { wedgingof dorsolumbarvertebralbodies
calvarium) ! coxa vara with flattenedfemoralhead
{ poorlymineralizedshort bowedtubularbones r/ prematureatherosclerosis
+ multiplefractures 0 No skeletalchangeswith adult onsetl
 Radiology Review Manual

INFANTILECORTICALHYPEROSTOSIS ./ osteoporosisin long bones (mostprominentin hands)

= CAFFEY DISEASE ^/ absenceof facialbone involvement
= uftcorTtrTlonself-limitingproliferativebone diseaseof
infancy; remission + exacerbations are common JACCOUDARTHROPATHY
Cause: ? infectious; ? autosomaldominantwith variable After subsidenceof frequentsevereattacksof rheumatic
expression+ incompletepenetrance/ sporadic fever
occurrence(rare) Path: periarticularfascial+ tendonfibrosiswithout
Age: <6 months,reportedin utero; M:F = 1:1 synovitis
Histo: inflammation of periostealmembrane,proliferation . rheumaticvalvedisease
of osteoblasts+ connectivetissuecells,deposition Location: primarilyinvolvement of hands;occasionally
of immaturebony trabeculae in great toe
. sudden,hard,extremelytendersoft-tissue swellings r/ muscularatrophy
over bone r/ periarticular swellingof smalljointsof hands+ feet
. irritability,
fever { ulnardeviation+ flexionof MCP jointsmost markedin
. r elevatedESR, increasedalkalinephosphatase 4th + 5th finger
. leukocytosis,anemia ^/ trtOjoint narrowing/ erosion
Location: mandible(80%)> clavicle> ulna + others
(exceptphalanges+ vertebrae+ roundbones SYNDROME
KLINEFELTER
of wristsand ankles) 47,XXY(rarelyXXYY) chromosomalabnormality
Site: hyperostosisaffectsdiaphysisof tubularbones Incidence: 1:750livebirths(probablycommonest
asymmetrically, ePiPhYses sPared chromosomalaberration)
r/ massiveperiostealnew-boneformation+ perifocalsoft- ' testicularatrophy(hyalinization of seminiferous tubules)
tissueswelling = Srn?ll/ absenttestes,sterility(azoospermia)
r/ "double-exposed" ribs . eunuchoidconstitution:gynecomastia; paucityof hair
r/ narrowingof medullaryspace (= proliferationof on face + chest;femalepubic escutcheon
endosteum) . mild mentalretardation
{ bone expansionwith remodelingof old cortex . high levelof urinarygonadotropins + low levelof
Prognosis; usuallycompleterecoveryby 30 months 17-ketosteroidsafter PubertY
Rx: mildanalgesics, steroids 0 NO distinctiveradiological findings!
{ may have delayedbone maturation
ChronicInfantileHyPerostosis ri failureof frontalsinusto develop
. disease
maypersist foryears
or recurintermittently r/ smallbridgedsellaturcica
. delayedmusculardevelopment, cripplingdeformities ! + scoliosis,kyphosis
{ bowingdeformities, osseousbridging,diaphyseal r/ + coxa valga
expansion ./ t metacarpalsign (short4th metacarpal)
DDx: (1) Hypervitaminosis A (rarely<1 year of age) { accessoryepiphysesof 2nd metacarpalbilaterally
(2) Periostitisof PrematuritY
(3) Healingrickets 47,XXX= SuperfemaleSYndrome
. usuallyover 6 feet tall;subnormalintelligence;
(4) Scurvy(uncommon<4 monthsof age)
(5) Syphilis(focaldestruction) frequentlyantisocialbehavior
(6) Childabuse
(7) Prostaglandin administration(usuallyfollowing
4-6 weeks of theraPy) KLIPPEL.TRENAUNAY SYNDROME
(8) OsteomYelitis = sPor?dic(nonhereditary) rare mesodermalabnormality
(9) Leukemia that usuallyaffectsa singlelower limb characterizedby
(10)Neuroblastoma a triad of:
(11) Kinkyhairsyndrome (1) port-winenevus= unilaterallargeflat infiltrative
(12) Hereditaryhyperphosphatasia cutaneouscapillaryhemangiomaoftenin
dermatomaldistribution on affectedlimb;may fade in
IRO N DE F I CI E N C YAN EMIA 2nd-3rd decade
(2) gigantisrTt= overgrowthof distaldigitsi entire
Age: infants affected
extremity(especiallyduringadolescentgrowthspurt)
Cause:
involvingsoft-tissue+ bone
(1) inadequateironstoresat birth
(3) varicoseveins on lateralaspectof affectedlimb;
(2) deficientiron in diet
absorptionof iron to hemangioma
usuallyipsilateral
(3) impairedgastrointestinal
(4) excessiveiron demandsfrom blood loss Pathogenesis:
(5) polycythemiavera (6) cyanoticCHD superficiallateralvenouschannelof largecaliber
r/ wideningof diploe+ thinningof tableswith sparingof thoughtto representthe fetal laterallimb bud vein that
has failedto regress;tissueovergrowthis secondaryto
occiput(no red marrow)
impairedvenousreturn
r/ hair-on-endappearanceof skull

A ge: us uallyin c h i l d re n ;M:F = 1 :1
Associated with:
- polydactyly,syndactyly,clinodactyly,oligodactyly,
ectrodactyly,congenitaldislocationof hip
- hemangiomas of colon/ bladder(3-10%)
- spinalhemangiomas + AVMs
- hemangiomas in liver/ spleen
- lymphangiomas of limb
Location: lowerextremity(10-15 x morecommonthan
upperextremity);bilateralin <5%; extension
into trunk may occur
. extremitypain
. spontaneouscutaneoushemorrhage
. chronicvenousinsufficiency
. cutaneouslymphaticvesicles,lymphorrhea
r/ elongationof bones:
r/ leg lengthdiscrepancy
./ increasedmetatarsal/ metacarpal+ phalangealsize
r/ corticalthickening
r/ soft-tissuehypertrophy(at birth/ later in life)
tr punctatecalcifications(phleboliths)in pelvis(bowelwall,
, urinarybladder)
r/ pulmonaryvein varicosities
r/ cysticlung lesions
Venogram:
r/ extensivedilationof superficialveins
r/ enlarged perforatingveins
r/ aplasia/ hypoplasiaof lowerextremityveins
(8-a0%): ? selectiveflow of contrastmaterialup the
lateralvenouschannelmay fail to opacifythe deep
venoussystem
^/ incompetentvalvelesscollateralvenouschannels
(? persistentlaterallimb bud vein = Klippel-Trenaunay
vein) arisesnear the ankle+ extendsa variable
distanceup the extremity+ drainsinto deep femoral
veini iliacveins(in >66%)
ColorDopplerUS:
r/ normaldeep veins
Lymphangiography:
r/ hypoplasiaof lymphaticsystem
Cx: thrombophlebitis, deep venousthrombosis,
pulmonaryembolism,lymphangitis
Rx: (1) conservative:applicationof graded
compressivestockings,pneumaticcompression
devices,percutaneoussclerosisof localized
venous malformations / superficialvaricosities
(2) surgical: epiphysiodesis,excisionof soft-tissue
hypertrophy,vein stripping
DDx: (1) Parke-Weber syndrome
= coflgenitalpersistenceof multiplemicroscopic
AV fistulas+ spectrumof Klippel-Trenaunay-
Webersyndrome(pulsatility, thrill,bruit)
(2) Neurofibromatosis (caf6-au-laitspots,axillary
freckling,cutaneousneurofibromas,
macrodactylysecondaryto plexiform
neurofibromas, wavy corticalreaction,early
fusionof gr:owthplate,limb hypertrophynot as
extensive/ bilateral)
Boneand Soft-Tissue
Disorders 107
(3) Beckwith-Wiedemann syndrome(aniridia,
macroglossia, cryptorchidism,
Wilmstumor,
broad metaphyses,thickenedlong-bonecortex,
advancedbone age, periostealnew-bone
formation,hemihypertrophy)
(4) Macrodystrophia lipomatosis(hyperlucencyof
fat, distalphalangesmost commonlyaffected,
overgrowthceaseswith puberty,usuallylimited
to digits)
(5) Maffucisyndrome(cavernoushemangiomas,
softtissuehypertrophy, phleboliths,
multiple
enchondromas)
LABRALTEARSOF SHOULDER
AnteriorLabralTear
Location: anteroinferior labrum> entireanterior
labrum> isolatedtear of anterosuperior
labrum
Subtypes of anteroinferiorlabral tears:
(1) Bankartlesion
(2) Anteriorlabroligamentous periostealsleeve
avulsion
(3) Pertheslesion
r/ absence/ detachmentof labrum
r/ frayedlabrumwith irregularmargin
DDx: (1) Middle+ inferiorglenohumeral ligaments
closelyapposedto anteriorlabrum
(2) Recessbetweenanteriorlabrum+ glenoidrim
(3) Recessbetweenmiddle+ inferiorligaments
SLAPLesion
= anterior-to-posterior lesionof the superiorlabrum
centeredat bicepstendonattachment
Mechanism: sportsactivitywith overheadarm motion,
fall on an outstretchedhand
. pain,clickingsensation
Type I = smalltear lirregularityconfinedto superior
labrum;commonin elderlyas a degenerative
tear
Type ll = detachmentof superiorlabral-bicipital
complexfrom glenoidrim
DDx: superiorsublabralrecess(less
distancebetweenlabrum+ glenoid,no
irregularappearance,no lateral
extensionof defect)
Type lll = detachmentof superiorportionof labrumfrom
glenoid+ bicepstendon(similarto bucket-
handletear of knee meniscus)
Type lV = type lll + tear extendinginto bicepstendon
LANGERHANS CELL HISTIOCYTOSIS
= LCH= HISTIOCYTOSIS X (formername)
= poorlyunderstoodgroup of disorderscharacterizedby
proliferation of Langerhanscells(normallyresponsible
for first-lineimmunologic defensein the skin)
Cause: uncertain(? primaryproliferativedisorder
possiblydue to defectin immunoregulation;
neoplasm)
Path: influxof eosinophilic leukocytessimulating
inflammation ; reticulumcellsaccumulate
cholesterol+ lipids(= foam cells);sheetsor
nodulesof histiocytesmay fuse to form giant cells,
cytoplasmcontains(? viral) Langerhansbodies
Histo: Langerhanscells are similarto mononuclear
macrophages+ dendriticcells as the two major
typesof nonlymphoid mononuclear cellsinvolved
in immune+ nonimmuneinflammatory response;
derivedfrom promonocytes(= bone marrowstem
cell)
Age: any age, mostlypresentingat 1-4 years;[vl;f = 1:1
Location: bone + bone marrow,lymphnodes,thymus,
ear, liverand spleen,gallbladder, Gl tract,
endocrinesystem
DDx: osteomyelitis,Ewingsarcoma,leukemia,
lymphoma,metastaticneuroblastoma
Clinical manifestations:
A. LocalizedLCH (7O%)
= eosinophilic granuloma
B. DisseminatedLCH (30%)
1. Chronicdisseminated LCH (20/")
= Hand-Schtiller-Christiandisease
2. Fulminantdisseminated LCH (10%)
= Letterer-Siwe disease
a (7o%)
EosinophilicGranulom
= ffiostbenignvarietyof LCH localizedto bone
Age: 5-10 years (highestfrequency);range
2-30 years;<20 Years(in75%);M:F = 3:2
Path: bone lesionsarisewithin medullarycanal (RES)
Histo: considerablenumberof eosinophilsin addition
to the dominantLangerhanscell constituent
. eosinophilia in blood+ CSF
Location: bone (in children)or lung (in adults)
Sites: monostoticinvolvementin 5G-75%;
(a) flat bones: calvarium> mandible> ribs >
pelvis> vertebrae(rarelyposteriorelements)
(b) long bones: diaphyseal(58%)+ metaphyseal
(28%)+ metadiaphyseal(12%)+ epiphyseal
(2%)in humerus,femur,tibia
./ bone lesions1-15 cm in diameter:
. often accompaniedby tendersoft-tissuemass
{ geographic/ permeative/ moth-eatenconfiguration
{ well-/ poorlydefinedborders
DDx: neuroblastomametastases, leukemia,
lymphoma
@ Skull (50%)
Site: diploicspace of parietalbone (most
commonlyinvolved)+ temporalbone (petrous
ridge,mastoid)
{ round/ ovoidpunched-out lesion:
DDx: venous lake,arachnoidgranulation,parietal
foramen,epidermoidcyst,hemangioma
^/ bevelededge / "hole-within-hole"appearance
(due to asymmetricdestructionof inner + outer
tables)
{ sharplymarginatedwithoutscleroticrim
(DDx:epidermoidwith bone sclerosis)
{ scleroticmarginduringhealingphase (50%)
r/ "buttonsequestrulfi"= felrlfiantsof bone as a
centralbonedensitywithina lyticlesion
{ soft-tissuemass overlyingthe lytic processin
calvarium(oftenPalPable)
r/ isodensehomogeneously enhancingmass in
hypothalamus/ PituitarYgland
@ Orbit
{ benignfocal masst infiltrationof orbitalbones
@ Mastoidprocess
. intractableotitismediawith chronicallydraining
ear (in temporalbone involvement)
ri destructivelesionnear mastoidantrum
DDx: mastoiditis,cholesteatoma,metastasis
Cx: extensionto middleear may destroyossicles
leadingto deafness
@ Jaw
. gingival+ contiguoussoft-tissue swelling
^/ "floating"teeth = destructionof alveolarbone
r/ mandibularfracture
@ Axial skeleton(25%) "coin
{ "vertebraplana" = on edge"= Calv6disease
(6"/")= collapseof vertebra(mostcommonly
thoracic):
0 Mostcommoncauseof vertebraplanain children
{ preserveddisk space
r/ rare involvementof posteriorelements
r/ no kyphosis
r/ tyticlesionin supraacetabular region
@ Proximal long bones (15%)
. painfulbone lesion+ swelling
Site: mostlydiaphyseal; epiphyseallesionsare
uncommon
^/ expansilelyticlesionwith ill-defined/ sclerotic
edges
./ endostealscalloping,wideningof medullarycavity
r/ corticalthinning,intracortical tunneling
r/ erosionof cortex+ soft-tissuemass
r/ laminatedperiostealreaction(frequent),may show
interruptions
! may appearraPidlywithin3 weeks
./ lesionsrespectjoint space + growthplate
@ Lung involvement(20%)
lncidence; 0.05-0.5:100,000 annually
Age: peak between20 and 40 Years
0 Strongassociationbetweensmoking+ primary
pulmonaryLangerhans cell histiocytosis!
r/ g-t0 mm nodules
r/ reticulonodular patternwith predilectionfor apices
{ may developinto honeYcomblung
^/ recurrentpneumothoraces (25%)
r/ rib lesionswith fractures(common)
^/ pleuraleffusion,hilaradenopathy(unusual)
NUC:
{ negativebone scans in 35% (radiographsmore
sensitive)
{ bone lesionsgenerallynot Ga-67 avid
./ Ca-OZmay be helpfulfor detectingnonosseous
lesions
Prognosis; excellentwith spontaneousresolutionof
bone lesionsin 6-18 months
 Boneand Soft-Tissue
Disorders 109

Hand-Schtiller-Christian
Diseaseeo%) . skin rash:scaly erythematousseborrhea-likebrownto
= chronicdisseminatedform of LCH characterizedby red papules
CLA S S I CT R IA D(i n 1 0 -1 5 % )o f Location: especiallypronouncedbehindears,in
(1) exophthalmos(masseffecton orbitalbone) axillary,inguinal,and perinealareas
(2) diabetesinsipidus(basilarskulldisease/ direct Sites: liver,spleen,bone marrow,lymphnodes,skin
infiltrationof posteriorpituitarygland) r/ hepatosplenomegaly + lymphadenopathy (mostoften
(3) destructivebone lesions(oftenof calvaria) cervical)
Path: proliferationof histiocytes,may simulateEwing r/ obstructivejaundice
sarcoma @ Boneinvolvement (50%):
Age at onset: <5 years (rangefrom birthto 40 years); r/ widespreadmultiplelytic lesions;"raindrop"
M : F= 1 ; 1 patternin calvarium
. diabetesinsipidus(30-50%) oftenwith large lytic Prognosis; rapidlyprogressivewith 70/" mortalityrate
lesionin sphenoidbone/ panhypopituitarism
. otitismediawith mastoid+ innerear invasion
. exophthalmos(33%),sometimeswith orbitalwall LAURENCE.MOON.BIEDL
SYNDROME
destruction . retardation
. generalizedeczematoidskin lesions(30%) . obesity
. ulcersof mucousmembranes(gingiva,palate) . hypogonadism
Sites: bone,liver,spleen,lymphnodes,skin r/ craniosynostosis
@ Bone ! polysyndactyly
r/ osteolyticskull lesionswith overlyingsoft-tissue
nodules
r/ "geographic skull"= oVoid/ serpiginous LEAD POISONING
destructionof largearea = PLUMBISM
r/ "floatingteeth"with mandibularinvolvement Path: lead concentratesin metaphysesof growing
r/ destructionof petrous ridge + mastoids+ sella bones (distalfemur > both ends of tibia > distal
turcica radius)leadingto failureof removalof calcified
@ Orbit cartilaginoustrabeculaein provisionalzone
r/ diffuseorbitaldiseasewith multipleosteolyticbone . loss of appetite,vomiting,constipation,abdominal
lesions cramps
@ Liver . peripheralneuritis(adults),meningoencephalitis
d hepatosplenomegaly (rare) (children)
ri scatteredechogenic/ hypoattenuating liver . anemia
granuloma . lead line at gums (adults)
r/ lymphadenopathy (may be massive) r/ bandsof increaseddensityat metaphysesof tubular
r/ gallbladderwall thickening(frominfiltration) bones(onlyin growingbone)
@ Lung r/ lead linesmay persist
r/ cyst + bleb formationwith spontaneous { clubbingif poisoningsevere(anemia)
pneumothorax(25%) { bone-in-boneappearance
r/ ill-defineddiffusenodularinfiltrationoften DDx: (1) Healedrickets
progressingto fibrosis+ honeycomblung (2) Normalincreaseddensityin infants<3 years of
@ Thymus age
{ enlargedthymus+ punctatecalcifications
Prognosis.'spontaneousremissions+ ex€lc€rbations:
fatalin 15% LEPROSY
= HANSEN DISEASE
Organism; Mycobacteriumleprae
Letterer-Siwe
Disease(1o%) Types:
= dcutedisseminated, fulminantform of LCH (1) lepromatous:in cutis,mucousmembranes,viscera
characterizedby wasting,pancytopenia(frombone (2) neural: enlargedinduratednodularnervetrunks;
marrowdysfunction), generalizedlymphadenopathy, muscularatrophy,neurotrophic
anesthesia, changes
hepatosplenomegaly (3) mixedform
lncidence; 1: 2,000,000
Age: severalweeks after birthto 2 years
@ Osseouschanges(in 15-540/"of patients)
Path: generalizedinvolvement of reticulumcells;may { specificsigns:
be confusedwith leukemia Location: centerof distalend of phalanges/
. hemorrhage,purpura(secondaryto coagulopathy)
. severeprogressiveanemia/ pancytopenia eccentric
. intermittentfever r/ ill-definedareas of decalcification,
reticulated
. failureto grow/ malabsorption trabecularpattern,small roundedosteolytic
+ hypoalbuminemia lesions,corticalerosions
 r/ loint spacespreserved
r/ healingphase: completeresolution / bonedefect
with scleroticrim + endostealthickening
./ nasalspineabsorption+ destructionof maxilla,
nasalbone,alveolarridge
^/ enlargednutrientforaminain clawlikehand
ri erosivechangesof ungualtufts
r/ nonspecific signs:
r/ soft-tissueswelling;calcificationof nerves
{ contractures/ deep ulcerations
r/ neurotrophic joints(distalphalangesin hands,
MTP in feet,Charcotjointsin tarsus)
OF BONE
LEUKEMIA
A . CHI LDHO O D
mostcommonmalignancyof childhood:
1i3 of all pediatricmalignancies
Histo: acutelymphocytic leukemia(in75%)
Peak age: 4 years
. migratoryparaarticulararthralgias(25-50%)due to
adjacentmetaphyseallesions(may be confusedwith
acute rheumaticfever/ rheumatoidarthritis)
. low-gradefever,bruising,fatigue,bone pain
. elevatederythrocytesedimentationrate,anemia
. hepatosplenomegaly, occasionallylymphadenopathy
0 Peripheralbloodsmearsmay be negativein
aleukemicform!
Skeletalmanifestations in 50-90%:
Location: proximal+ distalmetaphysesof long
bones,flat bones,spine
(a) Diffuseosteopenia(mostcommonpattern)
^/ diftusedemineralization of spine+ long bones
(= leukemicinfiltration of bone marrow
+ catabolicprotein/ mineralmetabolism)
^/ coarsetrabeculationof spongiosa(due to
destructionof finer trabeculae)
r/ multiplebiconcave/ partiallycollapsed
vertebrae(14%)
(b) "Leukemiclines"(40-53% in acute lymphoblastic
leuk e mi a ):
r/ transverseradiolucentmetaphysealbands,
uniform+ regularacrossthe width of
metaphysis(= lstlk.mic infiltrationof bone
marrow/ osteoporosisat sites of rapidgrowth)
Location: largejoints(proximaltibia,distal
femur,proximalhumerus,distal
radius+ ulna)
r/ horizontal/curvilinear bandsin vertebral
bodies+ edges of iliaccrest
^/ dense metaphyseallinesaftertreatment
(c) Focaldestructionof flat / tubularbones:
! multiplesmallclearlydefinedovoid/ spheroid
osteolyticlesions(destructionof spongiosa,
latercortex)in 30-60%
{ moth-eatenappearance, suturalwidening,
prominentconvolutional markingsof skull
0 Lyticlesionsdistalto knee/ elbowin children
are suggestiveof leukemia(ratherthan
metastases)!
(d) lsolatedperiostitisof long bones (infrequent):
{ smooth/ lamellated/ sunburstpatternof
periostealreaction(corticalpenetrationby
sheetsof leukemiccellsintosubperiosteum) in
12-25%
(e) Metaphysealosteosclerosis+ focal osteoblastic
lesion(veryrare)
^/ osteoscleroticlesions(latein diseasedue to
reactiveosteoblasticproliferation)
r/ mixed lesions(lytic+ bone-forming)in 18%
Dx: sternalmarrow/ peripheralblood smear
Cx: of leukemiccellsin marrow
proliferation
leadsto extraskeletalhematopoiesis
DDx: metastaticneuroblastoma, Langerhanscell
histiocytosis
B . A D U LTH OOD
0 Deathusuallyoccursbeforeskeletalabnormalities
manifest
{ osteoporosis
{ solitaryradiolucentfoci (vertebralcollapse)
{ permeatingradiolucentmottling(proximalhumerus)
LIPOBLASTOMA
= postnatalproliferationof mesenchymalcellswith a
spectrumof differentiationrangingfrom prelipoblasts
(spindlecells)to matureadipocytes
Path: immatureadiposetissueseparatedby septa into
multiplelobules
Histo: uni- and multivacuolated lipoblastsinterspersed
betweenspindlei stellatemesenchymal cells;
suspendedin myxoidstroma
Age: <3 yearsof age; M:F = 2:1
Location: subcutaneous tissueof extremities, neck,
trunk,perineum,retroPeritoneum
./ tatty tumorwith enhancingsoft-tissuecomponent
DDx: liposarcoma(extremelyrare in children)
LIPOMAOF BONE
= INTRAOSSEOUS LIPOMA
lncidence: <1:1,000primarybonetumors
Age: any (4th-6thdecade);M:F = 1:1
May be associatedwith: hyperlipoproteinemia
. asymptomatici localizedbone pain
Location: calcaneus,extremities(proximalfemur> tibia,
i l i um,skul l ,mand ible,
fi bul a,humerus),
maxilla,ribs,vertebrae,sacrum,coccyx,
radius
Site: metaphysis
r/ expansilenonaggressive radiolucent lesion
r/ loculated/ septatedappearance(trabeculae)
r/ tfrinwell-definedscleroticborder
^/ + thinnedcortex(NO corticaldestruction)
./ tlO periostealreaction
! may containclump of calcificationcentrally
(= dystrophiccalcificationfrom fat necrosis)
O VIRTUALLYDIAGNOSTIC:
@ Calcaneus
^/ in triangularregionbetweenmajortrabecular
groups (LAT projection)
r/ calcifiedi ossifiednidus
 Boneand Soft-TissueDisorders 1 1 1

@ Proximalfemur LipomaArborescens
r/ on / above intertrochanteric
line = DIFFUSE SYNOVIAL LIPOMA
{ markedossificationof marginsof lesion = lipoma-likelesioncomposedof hypertrophicsynovial
0 Radiographic appearancesimilarto unicameralbone villi distendedwith fat, probablyreactiveprocessto
cyst (infarctedlipom€r= uflicameralbone cyst ?) chronicsynovitis
DDx: fibrousdysplasia,simplebone cyst, posttraumatic Location: knee;monoarticular
cyst,giantcelltumor,desmoplastic fibroma, Frequently associated with:
chondromyxoidfibroma,osteoblastoma degenerativejoint disease,chronicrheumatoid
arthritis,priortrauma
LIPOMA OF SOFT TISSUE NeuralFibrolipoma
Mostcommonmesenchymal tumorcomposedof mature = FIBROLIPOMATOUS HAMARTOMA OF NERVE
adiposetissue = tdre tumorlikeconditioncharacterizedbysausage-
Histo: maturefat cells (adipocytes)that are uniformin shaped/ fusiformenlargementof a nerveby fibrofatty
size + shape,occasionallyhave fibrousconnective tissue
tissueas septations;fat unavailablefor systemic Age: early adulthoodbeforeage 30 years / at birth
metabolism Histo: infiltration of epineurium+ perineurium by
. stablesize after initialperiodof discerniblegrowth
fibrofattytissuewith separationof nervebundles
Age: 5th-6th decade; M > F . soft slowlyenlargingmass
Location: . pain,tenderness, decreasedsensation,paresthesia
(a) superficial= subcutaneous lipoma(morecommon)in Location: volar aspectof hand,wrist,forearm
posteriortrunk,neck,proximalextremities Site: mediann. (mostfrequently), ulnarn., radialn.,
(b) deep lipomain retroperitoneum, chestwall,deep soft brachialplexus;
tissueof hands + feet; multiplein S-7"h (up to May be associated with:
severalhundredtumors) macrodactyly(in 213)= macrodystrophia lipomatosa
^/ mass of fat opacityi densityi intensityidenticalto ! may not be visibleradiographically
subcutaneousfat MR:
{ corticalthickening(withadjacentparosteallipoma) r/ longitudinally oriented,cylindrical ,linearI
CT: serpiginous structuresof signalvoid about3 mm in
well-defined+ homogeneoustumor with low diameter(= r'leruefascicleswith epi- and perineural
attenuationcoefficient(-65 to -120 HU) fibrosis)separatedby areas of fat signalintensity
no enhancementfollowinglV contrastmaterial (= rnaturefat infiltratingthe interfascicular
MR: connectivetissue)
well-defined + homogeneous, oftenwith septations US:
signalintensitycharacteristics similarto { "cablelikeappearance"= alternatinghyper-and
subcutaneous fat: hyperintense on TlWl hypoechoicbandson US
+ moderatelyintenseon T2Wl DDx: cyst,ganglion,lipoma,traumaticneuroma,
differentiationfrom other lesionsby fat suppression plexiformneurofibroma, vascularmalformation
technique
LIPOSARCOMA
Angiolipoma Malignanttumorof mesenchymal originwith bulkof tumor
= lesioncomposedof fat separatedby small branching tissuedifferentiating intoadiposetissue
vessels lncidence: 12-18/. of all malignantsoft-tissuetumors;
Age: 2nd + 3rd decade; 5/" familialincidence 2nd mostcommonsoft-tissue sarcomain
. tender
adults(aftermalignantfibroushistiocytoma)
Location: upperextremity,trunk Age: 5th-6th decade
r/ signalcharacteristics
of fat + mixedwith varying Histo:
numbersof large/ smallvessels rarelyarisingfrom lipoma
r/ mostlyencapsulatedlesion,may infiltrate (a) myxoidtype (mostcommon,in 40-50%): varying
degreesof mucin+ fibroustissue+ relativelylittle
BenignMesenchymoma lipid (<10o/")= intermediatedifferentiation
= long-standing
lipomawith chondroid+ osseous r/ radiodensitybetweenwater + muscle
metaplasia (b) lipogenictype: malignantlipoblasts with large
amountsof lipid+ scantymyxoidmatrix
lnfiltratingLipoma = well-differentiated
= INTRAMUSCULAR LIPOMA r/ radiodensityof fat with thick + poorlydefined
= relativelycommonbenignlipomatoustumorextending streaksof high attenuation
betweenmusclefibersthat becomevariablyatrophic (c) pleomorphic type (leastcommon): markedcellular
Peak age: 5th-6th decade; M > F pleomorphism, paucityof lipid+ mucin
Location: thigh (50%),shoulder,upper arm = highlyundifferentiated
 Radiology Review Manual

r/ radiodensity of muscle(no distinguishing imaging Age: found at birth (50-65%);withinfirst 2 years of life
featuresfrom other soft-tissuesarcomas) (e0%)
. usuallypainlessmass (maybe painfulin 10-15%) Location: soft tissue;bone (rare)
Location: lowerextremity(45%),abdominalcavity { multilocularcysticlesionwith fibrousseptations
+ retroperitoneum (14%),trunk (14%),upper .i occasionally serpentinevascularchannels
extremity(7.6%),head& neck (6.5%), { opacification duringlymphangiography / directpuncture
m is c e l l a n e o u(1
s 3 .5% ) r/ clear/ milkyfluidon aspiration
Spread: hematogenousto lung,visceralorgans; DDx: hemangioma(bloodon aspiration)
myxoidliposarcomashows tendencyfor
serosal+ pleuralsurfaces,subcutaneous
tissue,bone OF BONE
LYMPHOMA
r/ nonspecificsoft-tissuemass (frequentlyfat is = RETICULUM CELLSARCOMA = HISTIOCYTIC LYMPHOMA
radiologicallynot detectable) = PRIMARY LYMPHOMA OF BONE
^/ inhomogeneousmass with soft-tissue+ fatty 0 the generalized form of reticulumcellsarcomais
components lymphoma
r/ enhancementafter lV contrastmaterial Prevalence: 2-6% of all primarymalignantbone tumors
(contradistinction to lipoma) i n chi l dren
^/ concomitantmass in retroperitoneum / thigh (in up to lncidence of bone marrow involvement:
10/" of myxoidliposarcomas)as multicentriclesion/ 5--15%i n H odgki ndi sease;
metastasis 2540% in non-Hodgkin lYmPhoma
r/ mass of near water density/ hypoechoic/ hypointense 0 Bone marrowinvolvementindicatesprogressionof
on Tl Wl + hyperintense on T2Wl in myxoidliposarcoma disease
(highcontentof myxoidcells) 0 Bonemarrowimaging-guidance for biopsy!
NUC: 40% sensitivity; 88% specificity
LYMEARTHRITIS MR: 65% sensitivity; 90% specificity
Agent: spirocheteBorreliaburgdorferi;transmittedby Histo: sheetsof reticulumcells,largerthan those in
tick lxodesdammini Ewingsarcoma(DDx:myeloma,inflammation,
Histo: inflammatorysynovialfluid,hypertrophicsynovia osteosarcoma,eosinophilicgranuloma)
withvascularproliferation
+ cellularinfiltration Age: any age; peak age in 3rd-5th decade;50%
. historyof erythemachronicummigrans <40 years;35%<30 Years;M:F = 2:1
. endemicareas: Lyme,Connecticut, firstrecognized . strikingcontrastbetweensize of lesion+ patient'swell-
location;now alsothroughoutUnitedStates,Europe, being
Australia Location: lowerfemur,uppertibia (40% about knee),
. recurrentattacksof arthralgiaswithindays to 2 years humerus,pelvis,scapula,ribs,vertebra
aftertick bite (80%) Site: dia- lmetaphysis
Location: mono-/ oligoarthritis of largejoints(especially { cancellousboneerosion(earliestsign)
knee) r/ mottledpermeativepatternof separatecoalescent
r/ erosionof cartilage/ bone (4%) areas
Rx: antibiotics ^/ late corticaldestruction
DDx: (1) Rheumaticfever (2) Rheumatoidarthritis rl lamellated/ sunburstperiostealresponse(lessthan in
(3) Gonococcalarthritis(4) Reitersyndrome Ewingsarcoma)
r/ tytic/ reactivenew-boneformation
{ associatedsoft-tissuemass without calcification
LYMPHANGIOMA i synovitisof kneejointcommon
= s€QUestered noncommunicating lymphoidtissuelined Cx: pathologicfracture(mostcommonamong
by lymphaticendothelium malignantbonetumors)
Cause: congenitalobstructionof lymphaticdrainage Prognosis: 50o/"5-year survival
Subtypes: DDx: (1) Osteosarcoma(less medullaryextension,
(1) Capillarylymphangioma (rare) youngerPatients)
Location: subcutaneoustissue (2) Ewingtumor(systemicsymptoms,debility,
(2) Cavernouslymphangioma youngerPatients)
Location: aboutthe mouth+ tongue (3) Metastaticmalignancy(multiplebonesinvolved,
(3) Cysticlymphangioma(mostcommon) more destructive)
= cYstichygroma
Associatedwith: hydropsfetalis,Turnersyndrome LIPOMATOSA
MACRODYSTROPHIA
Location: head,neck(75h), axilla(20/"), = taranonhereditary congenitalform of localized
extensioninto mediastinum(3-10%) gigantism= r€Uf€llfibrolipomawith macrodactyly
. soft fluctuantmass Path: strikingincreasein adiposetissuein a fine fibrous
0 Lymphangiomasare frequentlya mixtureof networkinvolvingperiosteum,bone marrow,nerve
subtypes! sheath,muscle,subcutaneous tissue
 Boneand Soft-TissueDisorders 113

May be associatedwith: syn-, clino-, polydactyly @ SPine

. painless . ratioof measurementbetweensymphysisand
Location: 2nd or 3rd digitof hand/ foot;unilateral; floor+ crownand floor>0.45
one / few adjacentdigitsmay be involvedin { pectuscarinatum/ excavatum(common)
the distributionof the median/ plantarnerves { scoliosis/ kyphoscoliosis (45-60%)
^/ tong+ broadsplayedphalangeswith endosteal ^/ increasedincidenceof Scheuermann disease
+ periostealbone deposition and spondylosis
^/ overgrowthof soft tissue,greatestat volar + distal { dural ectasia
aspects r/ increasedinterpedicular distance
r/ slantingof articularsurfaces r/ posteriorscalloping
r/ lucentareas of fat (DIAGNOSTIC) ^/ presacral+ lateralsacralmeningoceles
Prognosis.' acceleratedmaturationpossible;growth r/ expansionof sacralspinalcanal
stops at puberty ./ enlargementof sacralforamina
DDx: fibrolipomatous hamartomaassociatedwith r/ wingedscapulae
macrodystrophia lipomatosa(indisti
nguishable), @ Joints
Klippel-Trenaunay-Weber syndrome, . ligamentous laxity+ hypermobility + instability
lymphangiomatosis, hemangiomatosis, r/ prematureosteoarthritis
neurofibromatosis, chronicvascularstimulation. r/ patellaalta
Proteussyndrome r/ genu recurvatum
! recurrentdislocations of patella,hip,clavicle,
mandible
MARFAN SYNDROME r/ slippedcapitalfemoralepiphysis
= ARACHNODACTYLY ./ progressiveprotrusioacetabuli(50%),bilateral>
= autosomaldominantfamilialdisorderof connective unilateral.
F>M
tissuewith high penetrancebut extremelyvariable
expression,new mutationsin 15./" B. OCULARMANIFESTATIONS
Etiology: fibrillingene defecton chromosome15 . bilateralectopialentis,usuallyupward+ outward
resultingin abnormalcross-linking
of collagen (secondaryto poor zonularattachments)
fibers . glaucoma,macrophthalmia
Prevalence;5:100,000;M:F = 1:1 ' hypoplasiaof iris+ ciliarybody
. contractedpupils(absenceof dilatormuscle)
A. MUSCULOSKELETAL MANIFESTATIONS . myopia,retinaldetachment
. tall thin staturewith long limbs,arm span greater . strabismus, ptosis
than height . bluesclera
. muscularhypoplasia+ hypotonicity . megalocornea= flat enlargedthickenedcornea
. scarcityof subcutaneousfat (emaciatedlook)
r/ generalizedosteopenia c. cARDtovAScuLAR MANTFESTATTONS (60-98%)
affectingmitralvalve,ascendingaorta,pulmonary
@ Skull artery,splenic+ mesentericarteries(occasionally)
. elongatedface 0 Causeof deathin 93%!
r/ dolichocephaly . chestpain, palpitations,shortnessof breath,fatigue
r/ prominentjaw . mid-to-late systolicmurmur+ one / moreclicks
^/ nigh archedpalate Associatedwith congenitalheart defect (33%):
@ Hand incompletecoarctation, ASD
. Steinbergsign = protrusionof thumbbeyondthe
@ Aorta (causeof death in 55%)
confinesof the clenchedfist (foundin 1.1%of Llisto: myxomatousdegenerationof aorticannulus
normalpopulation) r/ "tulipbulb aorta"= syrT'rmetrical
dilatationof aortic
. metacarpalindex(averagingthe 4 ratiosof length sinusesof Valsalvaslightlyextendinginto
of 2nd through5th metacarpalsdividedby their ascendingaorta (58%)
respectivemiddiaphyseal width)>8.8 (male)or r/ annuloaorticectasia= combinationof aorticroot
9.4 (female) dilatation+ aorticregurgitation
{ arachnodactyly = elongationof phalanges { fusiformaneurysmof ascendingaorta, rarely
+ metacarpals beyondinnominateartery(dueto cysticmedial
r/ flexiondeformityof 5th finger necrosis)
@ Foot { aorticwall calcificationrare
./ pes planus Cx: (1) Aorticregurgitation (in 81% if root
r/ clubfoot diameter>5 cm; in 100%if rootdiameter
^/ halluxvalgus >6 cm (2) Aorticdissection(3) Aorticrupture
^/ hammertoes (secondaryto progressiveaorticroot
{ disproportionate elongationof 1st digitof foot dilatation)
 @ Mitralvalve MELORHEOSTOSIS
Histo: myxomatousdegenerationof valve Nonhereditary diseaseof unknownetiology;often
leadsto redundancy+ laxness incidentalfinding
. midto-latesystolicmurmur+ one i moreclicks Age: slowchroniccoursein adults;rapidprogression in
r/ "floppyvalve syndrome"(95%) = redundant children
chordaetendineaewith mitralvalveprolapse Associatedwith: osteopoikilosis, osteopathiastriata,
+ regurgitation tumors/ malformations of blood
Cx: (1) Mitralregurgitation vessels(hemangioma, vascularnevi,
(2) Ruptureof chordaetendineae(rare) gl omustumor,A V M,aneurysm ,
@ Coarctation (mostlynot severe) lymphedema, lymphangiectasia)
@ Pulmonaryarteryaneurysm+ dilatationof . severepain+ limitedjoint motion(bonemay encroach
pulmonaryarterialroot (43%) on nerves,bloodvessels,or joints)
@ Cor pulmonale(secondary to chestdeformity) . thickening+ fibrosisof overlyingskin (resembling
scleroderma)
D . P ULM O NA R Y MA N IF E ST A T IO N S . muscleatrophy(frequent)
r/ cysticlung disease
r/ recurrentspontaneouspneumothoraces Location: diaphysis,usuallymonomelicwith at leasttwo
bonesinvolvedin dermatomaldistribution
E . A B DO M I NA LM AN IF ES T AT ION (followsspinalsensorynervesclerotomes);
r/ recurrentbiliaryobstruction entirecortex/ timitedto one side of cortex;
morecommonin lowerlimb;skull,spine,ribs
DDx: (1) Homocystinuria (osteoporosis) rarelyinvolved
(2) Ehlers-Danlos syndrome "candlewax dripping"= continuous/ interrupted streaks
(3) Congenitalcontracturalarachnodactyly(ear / blotchesof sclerosisalongtubularbone beginningat
NO ocular/ cardiacabnormalities)
deformities, proximalend extendingdistallywith slow progression
(4) Type lll MEN (medullarythyroidcarcinoma, ./ may crossjointwith jointfusion
mucosalneuromas,pheochromocytoma, smallopacitiesin scapula+ hemipelvis(similarto
marfanoidhabitus) osteopoikilosis)
discrepantlimb length
flexioncontracturesof hip + knee
MASSIVE OSTEOLYSIS genuvalgum/ varus
= GORHAM DISEASE = "VANISHING BONE"SYNDROME .l dislocatedpatella
= PHANTOM BONE= HEMANGIOMA OF BONE ossifiedsoft-tissuemasses(27%)
= LYMPHANGIOMATOSIS OF BONE
= infrequentdisorderof unknownetiologywith DDx: (1) Osteopoikilosis (generalized)
unpredictablecourse+ progression (2) Fibrousdysplasia(normalbonestructurenot
lncidence: >100 casesdescribed lost,not as dense)
Histo: massiveproliferationof hemangiomatous / (3) Engelmanndisease
lymphangiomatous tissuewith largesinusoid (4) Hyperostosis tuberous
of neurofibromatosis,
spaces+ fibrosis sclerosis,hemangiomas
Age: children+ adults<40 years (5) OsteoarthroPathY
Associatedwith: soft-tissuehemangiomaswithout
calcifications
. frequentlyhistoryof severetrauma(50%) ME N IS C A L TE A R
. little/ no pain Type of tear:
A . LON GITU D IN ATE L AR
Location: any bone;mostcommonlymajorlongbones 1. Horizontal cleavagetear
( hume ru ss, h o u l d e r,ma n d i b l e ),
i n n o mi nate Cause: usuallydegenerative
bone,spine,thorax,shorttubularbonesof Associatedwith: meniscalcYst
hand+ feet (unusual) Site: primarilyinvolvingthe centralhorizontal
planeof meniscusbeginningat inner
./ progressiverelentlessdestructionof bone margin
r/ lack of reaction(no periostealreaction,no repair) 2. Buckethandletear
{ advancingedgeof destructionnot sharplydelineated Cause: traumatic
{ taperingmarginsof bone ends at sitesof osteolysiswith Site: usuallyin medialrarelyin lateralmeniscus
conelikespiculeof bone (earlychanges) r/ longitudinalverticaltear with unstable
^/ no respectfor joints displacedinnerfragment
r/ may destroyall bones in a particulararea 3. Peripheraltear
DDx: Langerhanscell histiocytosis, fibrousdysplasia, Cause: traumatic
browntumorof hyperparathyroidism r/ verticaltear in peripheralthirdof meniscus
 Boneand Soft-Tissue
Disorders 115

B . O B LI Q UETE AR entire 227o entire 87o

radial tear
Site: commonin midportionof medialmeniscus
^/ Oottrhorizontaland verticalcomponents
./ commonlyextendingto inferiorsurfaceof
meniscus
anterior 67o
1. Parrotbeaktear
Cause: usuallydegenerative body l47o
Site: in body of lateralmeniscusnearthe
junctionof body + posteriorhorn posterior 537o
r/ frayingof free edge lSVo
2. Flaptear = oblique+ incomplete tear
Cause: traumatic,at times degenerative posteior 60Vo posterior &body l9Vo
C. TRANSVERSETEAR = RADIALTEAR
Site: posterior+ midportionof lateralmeniscus Medial Meniscus Tears Lateral MeniscusTears
r/ peripheraldisplacement of meniscus
r/ "absent"I graymeniscusposteriorly
Cx: lack of resistanceto hoop stresses
D. M E NI S CO C AP SU L AR S EP AR AT ION
= tearingof peripheralattachmentsof meniscus
r/ linearregionsof fluidseparatingmeniscusfrom oblique tear longitudinal longitudinal
capsule horizontal tear vertical tear
r/ uncoveringof a portionof tibialplateauowingto
inwardmovementof separatedmeniscus
L-
-F
MR Classification
type 0 type I type II
Grade Type MR Finding PPV for Tear

F>
0 0 nor m a me
l niscus 1%
1 I globular/ punctateintrameniscalsignal 2% -F
2 ll linearsignalnot extendingto surface S% type III type V
type IV
lll shorttaperedapex of meniscus 23%
lV truncatedi bluntedapex of meniscus 71%
3 V signalextendingto only one surface AS%
3 Vl signalextendingto both surfaces gS%
3 Vll comminutedreticulated signalpattern 82% type VI type VII

0 Diagnosisof tear hingeson surfaceinvolvement! Types of Meniscal Injury

0 Intrameniscal signalmay be a sign of persistent
vascularityin children+ youngadults(typeVll)!
0 Truncationartifact+ magicangle artifactmay cause
increasedintrameniscal signal!
0 Grade3 signalidentified only on a singleimageis MR sensitivity, specificity, and accuracy:
unlikelyto be confirmedas a tear at surgery! Tear of Sensitivity Specificity Accuracy
Siteof injury:
(a) medialmeniscusin 45o/o:no isolatedtears of body / Medialmeniscus 95% 88% 59-92%
anteriorhorn Lateralmeniscus 81% 96% 87-92%
(b) lateralmeniscusin 22o/":posteriorhorn involvedin Anteriorcruciatelig. 91-96%
80% of all lateralmeniscaltears Posteriorcruciatelig. up to 99%
(c) both menisciinvolvedin 33%
Associatedwith: ligamentousinjury 0 MR has a high negativepredictivevalue!
. asymptomaticin up to 20/" of older individuals 0 60-97% accuracyfor arthrography
r/ signalextendingto articularsurface(typeV + Vl) 0 84-99/" accuracyfor arthroscopy(poor at posterior
r/ notchsign = linearsignalintensitybecomingwideras it hornof medialmeniscus)
extendstoward meniscalsurfaceindicatestype V
, finding(taperingtowardsurface= type ll finding) lnterpretative
errors(12%for experiencedradiologist)
:
r/ meniscalcyst = impliespresenceof meniscaltear Lateralmeniscus: 5.0% FN (middle+ posteriorhorn)
DDx: synovialcyst,tendonsheathfluid,fluidwithin 1.5%FP (posteriorhorn)
normalsynovialrecess,fluidcollectionremote Medialmeniscus: 2.5o/"FN (posteriorhorn)
from meniscus 2.5% FP (posteriorhorn)
 PITFALLS: E. Wernertype: autosomaldominant
A. Normalvariantssimulatingtears: F. Lamy-Bienenfeldtype autosomaldominant
1. Superiorrecesson posteriorhornof medial . ligamentous
laxity
meniscus r/ shorteningof radius+ ulna+ tibia
2. Poplitealhiatus { absentfibula
r/ hiatusof poplitealtendonseparateslateral r/ normalfemur+ humerus
meniscusfromjointcapsule
0 Seen above posterioraspect of lateral { shorteningof all long bones at birth,most markedin
meniscuson most superficialsagittalslice! tibia+ radius
0 Tendonmovesbehind+ inferiorto meniscus { modelingdeformitywithwideningof diaphysis
on adjacentdeepersections! { mild to moderatebowing
3. Transverseligament { hypoplasiaof fibulawith absentlateralmalleolus
Course: connectsanteriorhornsof both menisci { short+ thickulnawith hypoplastic distalend
r/ overridessuperioraspectof meniscibefore rl Madelungdeformityof wrist
completelyfusingto menisci { hypoplasiaof a vertebralbody may be present
0 Trace the cross sectionof the transverse
ligamentthroughthe infrapatellarfat pad on
morecentralimages! CHONDRODYSPLASIA
METAPHYSEAL
4. Meniscofemoral ligaments = S€Vereshort-limbeddwarfism
Origin: superior+ medialaspectof posterior r/ metaphysealflaring(Erlenmeyerflask deformity)
horn of lateralmeniscus extendingintodiaphysis
Attachment: medialfemoralcondyle A. Schmid type (mostcommon)
{ demonstratedin 1/3 of cases on SAG images autosomaldominant
(a) Wrisbergligament . waddlinggate
{ posteriorto posteriorcruciateligament Distribution: moremarkedin lowerlimbs;mild
(b) Humphryligament involvementof hands + wrists
{ anteriorto posteriorcruciateligament { shortenedbowed long bones
0 Findingusuallylimitedto singlemostmedial r/ widenedepiphysealgrowthplates
im ag e ! r/ irregularwidenedcuppedmetaphyses
5. Soft tissuebetweencapsule+ medialmeniscus r/ coxa vara
B. Healedmeniscus r/ genuvarum
r/ persistentgrade 3 signal at least up to 6 months DDx: vitamin D-refractory rickets
{ S/P meniscectomy(false-positive type lV finding) B. McKusick type
C. Degenerativechanges autosomalrecessive(eg, in Amish)
r/ grade 1 signal= globularincreasein intensity . sparsebrittlehair,deficientpigmentation
r/ grade2 signal= linearsignalnot extendingto . florrr'rdl
intelligence
articularsurface { shorteningof longboneswith normalwidth
D. Diskoidmeniscus { cupped + widened metaphyseswith lucent defects
= abnormallyshapedenlargeddiskus-likemeniscus r/ short middlephalanges+ narrowdistalphalanges
Prevalence; in 1.5-15.5% becomingtriangularand bullet-shaped (more
Age: children,adolescents frequentin handsthan feet)
Side: lateral>> medialmeniscus r/ widenedcostochondraljunctions + cysticlucencies
{ centrallydisplacedfragmentwith meniscus C. Jansen type (lesscommon)
apparentlyof normalsize (coronalimages) sporadicoccurrencewith wide spectrum
. intelligencenormal/ retarded
. serumcalciumlevelsoften elevated
MESOMELIC DWARFISM Distribution: symmetricalinvolvement of all long
= heritablebone dysplasiawith shorteningof intermediate + shorttubularbones
segments(radius+ ulnaor tibia+ fibula) r/ widenedepiphysealplates
A. Langer type autosomalrecessive { expandedirregular+ fragmentedmetaphyses
. mentalimpairment (unossifiedcartilageextendinginto diaphyses)
r/ mesomelicshorteningof limbs DDx: rickets
r/ hypoplasiaof ulna + fibula D. Pyle disease = Metaphysealdysplasia
r/ hypoplasiaof mandiblewith short condyles . oftentall
B. Nievergelttype autosomaldominant . often asymptomatic
r/ severemesomelicshorteningof lower limbs Distribution: majorlongbones,tubularbonesof
rl markedthickeningof tibia + fibulain centralportion hands,medialend of clavicle,sternal
r/ clubfoot(frequent) end of ribs,innominatebone
c. Reinhardttype: autosomal dominant r/ splayingof proximal+ distalends of long boneswith
D . Robinowtype: autosomal dominant thinnedcortex
 Boneand Soft-Tissue
Disorders 117

r/ relativeconstrictionof centralportionof shafts Neuroblastoma: extensivedestruction,resembles

r/ craniofacialhyperostosis leukemia(metaphysealband of
{ genu valgum rarefaction),mottledskull destruction
+ increasedintracranialpressure,
METASTASES
TO BONE perpendicular spiculesof bone
0 15-100timesmorecommon
thanprimary
skeletal
neoplasms! Mode of spread: through bloodstream/ lymphatics/
Frequency: directextension
lf primary known lf primary unknown
Location: predilectionfor marrow-containing skeleton
Breast 35% Prostate 25% (skull,spine,ribs,pelvis,humeri,femora)
Prostate 30% Lymphoma 15% r/ single/ multiplelesionsof variablesize
Lung 10% Breast 10% { usuallynonexpansile
Kidney 5% Lung 10% r/ joint spaces + intervertebral
spacespreserved(cartilage
Uterus 2% Thyroid 2% resistantto invasion)
Stomach 2% Colon 1%
Others 13o/"
OsteolyticBoneMetastases
METASTASESOF PRIMARYBONETUMORS Most commoncause: neuroblastoma(in childhood);
1. Osteosarcoma: 2o/owith distant metastases, lungcancer(in adultmale);
adjuvanttherapyhas changedthe naturalhistoryof breastcancer(in adultfemale),
the diseasein that bone metastasesoccur in 10% of thyroidcancer;kidney;colon
osteosarcomaswithoutmetastasesto the lung ^/ may begin in spongybone (associatedwith soft
2. Ewingsarcoma: 13% with distantmetastases tissuemass in ribs)
r/ vertebralpediclesoften involved(not in multiple
SOLITARYBONELESION myeloma)
0 Of all causes only 7Y" are due to metastasis
0 In patientswith knownmalignancysolitarybone OsteoblasticBoneMetastases
lesionsare due to metastasis(55%),due to trauma = evidenceof slow-growingneoplasm
(25%),due to infection(10%) Primary: prostate,breast,lymphoma,malignant
Location: axialskeleton(64-68%),ribs (45%), carcinoid,meddlloblastoma, mucinsus
extremities(24/"),skull(12%) adenocarcinonta of Gl tract,TCC of bladder,
pancreas,neuroblastoma
mnemonic.' "SeveralKinds Of HorriblyNastyTumors Most common cause: prostatecancer (in adult male);
Leap PromptlyTo Bone" breastcancer(in adult female)
Sarcoma,Squamouscell carcinoma mnemonic.' "5 Bees Lick Pollen"
Kidneytumor Brain(medulloblastoma)
Ovariancancer Bronchus
Hodgkindisease Breast
Neuroblastoma Bowel (especiallycarcinoid)
Testicularcancer Bladder
Lung cancer Lymphoma
Prostatecancer Prostate
Thyroidcancer r/ frequentin vertebrae+ pelvis
Breastcancer ri may be indistinguishablefrom Pagetdisease

Breastcancer: extensiveosteolyticlesions;involvement MixedBone Metastases

of entireskeleton;pathologicfractures breast,prostate,lymphoma
common
Thyroid/ kidney: oftensolitary;rapidprogressionwith
Expansile/ BubblyBone Metastases
boneexpansion(bubbly);frequently
kidney,thyroid
associatedwith soft-tissuemass
(distinctive)
Rectum/ colon: may resembleosteosarcomawith PermeativeBone Metastases
sunburstpattern+ osteoblasticreaction Burkitt
lymphoma,mycosis
fungoides
Hodgkintumor: upperlumbar+ lowerthoracicspine,
pelvis,ribs; osteolytic/ occasionally Bone Metastaseswith "Sunburst"Periosteal
osteoblasticlesions Reaction(infrequent)
Ewingtumor: extensiveosteolytic/ osteoblastic prostaticcarcinoma,retinoblastoma,
neuroblastoma
reaction (skull),Gl tract
 Radiology Review Manual

BoneMetastaseswith Soft-tissueMass ^/ increasedactivityin:

thyroid,kidney (a) enlargement of bone lesions/ appearanceof
new lesionsindicateprogression of the disease
Calcifying Bone Metastases (b) "healingflare"phenomenon(in 20-61%)
mnemonic; "BOTTOM" = transientincreasein lesionactivitysecondary
Breast to healingunderantineoplastic treatment
Osteosarcoma concomitantwith increasedsclerosis,detected
Testicular at 3.2 + 1.4 monthsafterinitiationof hormonal/
Thyroid chemotherapy,of no additionalfavorable
Ovary prognosticvalue
Mucinousadenocarcinomaof Gl tract (c) avascularnecrosisparticularly in hips,knees,
shoulderscausedby steroidtherapy
Skeletal Metastases in Children (d) osteoradionecrosis / radiation-i
nduced
1. Neuroblastoma(mostoften) osteosarcoma
2. Retinoblastoma r/ decreasedactivityin:
3. Embryonalrhabdomyosarcoma (a) predominatelyosteolyticdestruction
4. Hepatoma (b) metastasesunderradiotherapy;as early as
5. Ewingtumor 2-4 monthswith minimumof 2000 rads

Skeletal Metastases in Adult ROLEOF BONESCAN ]N BREASTCANCER

mnemonic.' "CommonBone LesionsCan Kill The Routinepreoperative bonescan notjustified:
Patient" Stage | : unsuspectedmetastasesin2o/o, mostly
Colon si ngl el esi on
Breast Stage ll : unsuspectedmetastasesin 6%
Lung Stage lll : unsuspected metastasesinl4/"
Carcinoid
Kidney Follow-upbonescan:
Thyroid At 12 monthsno new cases;al28 monthsin 5/.
Prostate new metastases;at 30 monthsin 29% new
metastases
Conversionfrom normal: Stage | : in 7%
Role of Bone Scintigraphy in Bone Metastases Stage ll : in 25/o
Pathophysiology: accumulationof tracer at sites of Stage lll : in 58%
reactivebone formation 0 With axillarylymphnode involvement conversion
False-negativescan: very aggressivemetastases rate2.5 x that of thosewithout!
False-positive
scan: degeneration, healingfractures, 0 Serialfollow-upexaminations are importantto
metabolicdisorders assesstherapeuticefficacy+ prognosis!

Baselinebone scan: ROLEOF BONESCAN ]N PROSTATECANCER

(a) high sensitivityfor many metastatictumorsto bone Stage B : 5% with skeletalmetastases
(particularlycarcinomaof breast,lung,prostate); Stage C : 10% with skeletalmetastases
5'h of metastaseshave normalscan: 540% Stage D : 20'/.with skeletalmetastases
occurin appendicular skeleton Test sensitivitiesfor detectionof osseousmetastases:
(b) substantially lesssensitivethan radiographs in (a) sci nti graphy
1.0
marrowlesions(multiplemyeloma,
infiltrative (b) radiographic survey0.68
neuroblastoma, histiocytosis) (c) alkalinephosphatase 0.5
(c) screeningof asymptomaticpatients (d) acid phosphatase 0.5
- usefulin: prostatecancer,breastcancer DDx: pulmonarymetastasis(SPECThelpfulin
- not usefulin: non-small-cell bronchogenic distinguishing nonosseouslungfrom overlyingrib
carcinoma,gynecologicmalignancy, headand uptake)
neckcancer
r/ multipleasymmetricareasof increaseduptake Role of Magnetic Resonance lmaging in Bone
{ axial> appendicular skeleton(dependenton Metastases
distributionof bone marrow);vertebrae, ribs,pelvis idealfor bone marrowimagingdue to high contrast
involvedin 80% betweenbone marrowfat + water-containing
./ superscanin diffusebony metastases metastaticdeposits
(1) Focall yti cl esi on:
Follow-upbonescan: r/ hypointense on T2Wl
on TlWl + hyperintense
= suggestiveof relativelygood
{ stableSc€lr'r (2) Focalscleroticlesion:
prognosis r/ hypointenseon Tl Wl + T2Wl
 Boneand Soft-Tissue
Disorders 119

(3) Diffuseinhomogeneous lesions: Histo: densecollagenous + fibroustissue

^/ inhomogeneously hypointenseon Tl Wl . burning/ electricforefootpain duringwalking
+ hyperintense
on T2Wl Location: typically3rd intermetatarsal space
(4) Diffusehomogeneous lesions: MR:
r/ homogeneously hypointense on Tl Wl { smalllesionof intermediate signalintensityon TlWl
+ hyperintense
on T2Wl + low signalintensityon T2Wl
r/ t fluid in intermetatarsal
bursa
METATROPHIC DYSPLASIA Rx: conservativetreatment;surgicalexcision
= HYPERPLASTIC ACHONDROPLASIA = METATROPHIC
DWARFISM M UCOPOLYSACCHARI DOSES
metatrophic = "changeable" (changein proportionsof = lysosomalstoragedisorderfrom deficiencyof specific
trunkto limbsovertime secondaryto developing lysosomalenzymesinvolvedin degradation of
kyphoscoliosis in childhood) mucopolysaccharides
' longitudinaldoubleskin fold overlyingcoccyx
r/ longbonesshortwith dumbbell-like / trumpet-shaped Type | =Hurler TypeV =Scheie
configuration (exaggerated metaphyseal flaring) Type ll = Hunter Type Vl = fi4spsteaux-Lamy
./ "hourglass" phalanges(shortwith widenedends) Type lll = Sanfilippo Type Vll = 51y
r/ wide separationof majorjoint spaces(thickarticular Type lV = Morquio
cartilage)
r/ detayedossificationof flat irregularepiphyses 0 All autosomalrecessiveexceptfor Hunter(X-linked)!
@ Chest Associatedwith: valvular heart disease
ri cylindricalnarrowedelongatedthorax . cornealclouding
ri short + wide ribs . retardati on (promi nent i n typesl , l l , l l l , V l l )
{ pectuscarinatum . skeletalinvolvement dominatesin types lV and Vl
@ Vertebrae { scaphocephaly, macrocephaly; thickcalvarium;
d odontoidhypoplasiawith atlantoaxialinstability hypertelorism
ri progressivekyphoscoliosis r/ platyspondylywith kyphosis+ dwarfism
ri platyspondyly+ very wide intervertebralspaces ri irregularityat anterioraspectof vertebralbodies
ri wedge-/ keel-shapedvertebralbodies r/ atlantoaxialsubluxation+ instability(laxityof transverse
@ Pelvis ligament/ hypoplasiaor absenceof odontoid)
ri coccygealappendagesimilarto a tail (rarebut r/ timOcontractures
cHARACTERtSTtC) ri broad hands + brachydactyly
ri shortsquarediliacbones+ irregularacetabula r/ hepatosplenomegaly
r/ narrowedgreatersciaticnotch @ Brain
Prognosis.' compatiblewith life,increaseddisabilityfrom r/ brain atrophy
kyphoscoliosis ri varyingdegreeof hydrocephalus
DDx: achondroplasia, mucopolysaccharidoses r/ multiplewhitematterchangeswithincerebral
hemispheres(diffusehypodenseareas,prolongation
METHOTREXATE
OSTEOPATHY ofTl + T2)
= Syhdromethat consistsof Cx: cordcompression at atlantoaxialjoint (typeslV + Vl)
( 1) bonepain Dx: combination of clinicalfeatures,radiographic
(2) osteopenia abnormalities correlatedwith genetic+ biochemical
(3) pathologicfractures studies
Methotrexate PrenatalDx: occasionallysuccessfulanalysisof
= dihydrofolate reductaseinhibitormostoftenused in fibroblasts culturedfrom amnioticfluid
childrenfor treatmentof ALL / osteosarcoma / brain DDx: Gaucherdisease.Niemann-Pick disease
t um or
0 Radiographic findingssimilarto scurvy: HurlerSyndrome
r/ osteopenia = GA R GOY LIS=M P FA U N D LE R -H U RDLE
ISREASE
./ dense provisionalzones of calcification = MP Sl -H
i/ pathologicfractures(mostoften metaphyseal) = autosomalrecessivedisease
{ sharplyoutlinedepiphyses Cause: homozygousfor MPS lll genewith excess
./ NO massivesubperiostealhemorrhage chondroitinsulfateB due to deficientX-L
r/ impairedhealingof fractures iduronidase(= Hurlercorrectivefactor)
lncidence; 1:10,000births
MORTONNEUROMA Age: usuallyappears >1styear
= INTERDIGITAL NEUROMA . dwarfism
M : F= 1 : 4 . progressivementaldeteriorationafter 1-3 years
Path: perineuralfibrosisinvolving+ entrappinga plantar . largehead;sunkenbridgeof nose;hypertelorism
digitalnerve . earlycornealcloudingprogressing to blindness
 Mucopolysaccharidoses

Type Eponym lnheritance Enzyme Deficiency Urinary Glycosaminoglycan Neurologic Signs

l-H Hurler autosomal aloha-L-iduronidase dermatansulfate matked

re@ssve
ll Hunter Xlinked iduronatesulfatase dsrmatan/ heparansulfate mildto moderate
rs@ssive
lll Sanfilippo autosomal heparansultate mentaldeterioration
recessive
A heDaran sulfatssulfatase
B N-acetyl-alpha-D-glucosaminidase
C alpha-glucosamine-N-acetyl
transferase
D N-acetylglucosamine-6-sullate
sulfatase
lV Morquio autosomal N-acetylgalactosamins-6-sulfatekeratansulfate none
A-D recessive sulfatase
beta-galactosidase
l-S(V)Scheie autosomal alpha-L-iduronidase hepalansul{ate none
recessrve
Vl Maroteaux- autosomal arylsulfatase B dermatansulfate none
Lamy recessive
Vll Sly autosomal beta-glucuronidase dermatansulfate variable
recessrve heparansulfate

."gargoyle"featureS= €Vertedlips + protrudingtongue

@ S pi ne
.teeth widelyseparated+ poorlyformed ./ thoracolumbar kyphosiswith lumbargibbus
.progressivenarrowingof nasopharyngeal airway ! ovalcentrawith normal/ increasedheight
.protuberantabdomen(secondaryto dorsolumbar + anteriorbeak atT12lL1lL2
kyphosis+ hepatosplenomegaly) .i tongslenderpedicles
. urinaryexcretionof chondroitinsulfateB (dermatan ^/ proximallylongslenderribsat levelof neck+ wide
sulfate)+ heparansulfate distally= spatulaterib configuration
. Reillybodies(metachromic granules)in whiteblood @ Pelvis
cellsor bone marrowcells r/ widelyflarediliacwingswith inferiortapering
@ Skull(earliestchanges>6 monthsof age) r/ constrictionof iliacbones
r/ frontalbossing ./ coxa valga
r/ calvarialthickening Prognosis; death by age 10-15 years
r/ prematurefusionof sagittal+ lambdoidsutures
^/ deepeningof optic chiasm
{ enlargedJ-shapedsella(undermining of anterior MorquioSyndrome
ctinoidprocess) = KERATOSULFATURIA = MPSlV
r/ smallfacialbones = autosomalrecessive;excesskeratosulfate
r/ wide mandibularangle+ underdevelopment of lncidence: 1:40,000births
condyles Etiology: N-acetylgalactosamine-6-sulfatase deficiency
r/ communicating hydrocephalus resultingin defectivedegradationof keratin
@ Extremities sulfate(mainlyin cartilage,nucleuspulposus,
r/ tnicf periostealcloakingof long-bonediaphyses cornea)
(earlychanges) Age: normalat birth;skeletalchangesmanifestwithin
{ swelling/ enlargement of diaphyses+ cortical first 18 months
thinning(dueto dilatationof medullarycanal) . €XC€ssive urinaryexcretionof keratansulfate
+ taperingof eitherend: distalhumerus,radius, . horrTr?lintelligence
ulna, proximalends of metacarpals . weakness+ hypotonia
{ deossification with heterogenousbone density . dwarfismwith shorttrunk(<4 feettall)
+ coursetrabeculation(dueto depositionof . head thrustforward+ sunkenbetweenhigh shoulders
accumulatedprecursormetabolitesin bone marrow) . frorrn?lintelligence
./ flexiondeformitiesof knees + hips . cornealopacitiesevidentaroundage 10
./ tridenthands;clawing(occasionally) . progressivedeafness
./ delayedmaturationof irregularcarpalbones . short nose,wide mouth,spacingbetweenteeth
 Boneand Soft-Tissue
Disorders 121

. semicrouchingstance+ knockkneesfrom flexion DDx: (1) Hurlersyndrome(normal/ increasedvertebral

deformities of knees+ hips height;vertebralbeak inferior)
@ S k ull (2) Spondyloepiphyseal dysplasia(autosomal
r/ mild dolichocephaly dominant,presentat birth,absenttlaredilia/
r/ hypertelorism deficientacetabularossification,
small
{ poor mastoidair cell development acetabularangle,deficientossificationof
! short nose + depressionof bridgeof nose pubic bones,varus deformityof femoralneck,
r/ prominentmaxilla minimalinvolvement of hand+ foot,myopia)
@ Chest
{ increasedAP diameter+ markedpectuscarinatum MULTIPLE EPIPHYSEAL DYSPLASIA
ri slightlordosiswith wide shortribs = FAIRBANK DISEASE
r/ bulbouscostochondral junctions = ? tarda form of chondrodystrophia calcificanscongenita
r/ failureof fusionof sternalsegments r/ mild limbshortening
@ S pine r/ irregularmottledcalcificationsof epiphyses(in
^/ hypoplasia/ absenceof odontoidprocessof C2 childhood+ adolescence)
^/ Ct -CZ instabilitywith anteriorsubluxation r/ epiphysealirregularities+ prematuredegenerative joint
r/ tnicf C2-bodywith narrowingof vertebralcanal disease,especiallyof hips (in adulthood)
r/ atlascloseto occiput/ posteriorarch of C1 within i/ short phalanges
foramenmagnum DDx: Legg-Perthesdisease,hypothyroidism
r/ platyspondyly= universalvertebraplanaesp.
affectinglumbarspine(DDx:normalheightin
Hurlersyndrome) MULTIPLE MYELOMA
i/ ovoidvertebralbodieswith centralanteriorbeak / 0 Mostcommonprimarymalignantneoplasmin adults!
tongueat lowerthoracic/ upperlumbarvertebrae Histo: normali pleomorphic plasmacells(not
{ mildgibbusat thoracolumbar transition= low pathognomonic), may be mistakenfor
dorsalkyphosis lymphocytes (lymphosarcoma, reticulumcell
r/ exaggeratedlumbarlordosis sarcoma,Ewingtumor,neuroblastoma)
{ widenedintervertebral disk spaces (a) diffuseinfiltration:myelomacellsintimatelyadmixed
@ Pelvis with hematopoieticcells
{ "goblet-shaped" / "wineglass"pelvis= constricted (b) tumornodules:displacement of hematopoietic cells
iliacbodies+ elongatedpelvicinlet+ flarediliac by massesentirelycomposedof myelomacells
wings Age: usuallysth-Bth decade;98/" >40 years;
r/ obliquehypoplasticacetabularroofs rare<30 yearsof age; M:F = 2:1
@ F em ur (a) DISSEMINATED FORM: >40 yearsof age
r/ initialtywell-formed femoralheadepiphysis,then (98% );M:F = 3:2
involution+ fragmentationby age 3-6 years (b) SOLITARYFORM: mean age 50 years
r/ lateralsubluxation of femoralheads;laterhip . bone pain (68%)
dislocation . normochromicnormocyticanemia(62%)
{ wide femoralneck + coxa valga deformity . RBC rouleauformation
@ Tibia . renal insufficiency(55%)
r/ delayedossificationof lateralproximaltibial . hypercalcemia(30-50%)
epiphysis . proteinuria (88%)
r/ slopingof superiormarginof tibiaplateaulaterally . BenceJonesproteinuria (50%)
+ severegenuvalgum . increasedglobulinproduction(monoclonal gammopathy)
@ Hand & foot Location:
r/ short bonesof forearmwith wideningof proximal A . D IS S E MIN A TEFOR
D M:
ends scattered;axialskeletonpredominantsite;vertebrae
r/ delayedappearance+ irregularityof carpalcenters (50%)> ribs> skull> pelvis> longbones
r/ smallirregularcarpalbones (distributioncorrelateswith normalsitesof red
r/ proximallypointedshort metacarpals2-5 marrow)
r/ enlargedjoints;hand + foot deformities(flatfeet) B. SOLITARYPLASMACYTOMA OF BONE:
tr ulnardeviationof hand vertebrae> pelvis> skull> sternum> ribs
C. SPINALPLASMACELL MYELOMA
Cx: cervicalmyelopathy(traumaticquadriplegia/ leg r/ sparingof posteriorelements(no red marrow)
painsi subtleneurologicabnormality)most (DDx: metastaticdisease)
commoncause of death secondaryto C2 ri paraspinalsoft-tissuemass with extradural
abnormality;frequentrespiratoryinfections(from extension
respiratoryparalysis) { scallopingof anteriormarginof vertebralbodies
Rx: earlyfusionof C1-C2 (osseouspressurefrom adjacentenlargedlymph
Prognosisj may live to adulthood nodes)
{ generalized osteopeniaonly (15%)with accentuation of Myelomatosis
trabecularpattern,especiallyin spine (early) { generalizeddeossification
withoutdiscretetumors
r/ punchedout appearanceof widespreadosteolytic r/ vertebralflattening
lesions(skull,longbones)with endostealscallopingand
of uniformsize MUSCULOTENDINOUS INJURY
i/ diffuseosteolysis(pelvis,sacrum) MuscleContusion
r/ expansileosteolyticlesions(ballooning) in ribs,pelvis, Cause: directtrauma,usuallyby bluntobject
longbones Site: deep withinmusclebelly
r/ soft-tissuemass adjacentto bone destruction . injuryat pointof impact
(= extrapleural+ paraspinalmass adjacentto ribs/ r/ NO architecturalchanges
vertebralcolumn) r/ featheryappearanceof diffusemuscleedema
{ periostealnew-boneformationexceedinglyrare r/ increasedmusclegirth
r/ involvementof mandible(rarelyaffectedby metastatic hematoma(withseveretrauma
r/ deep intramuscular
disease) resultingin disruptionof musclefibers)
r/ sclerosismay occur after chemotherapy,radiotherapy,
f luorideadministration Myotendinous
Strain
r/ scleroticform of multiplemyeloma(1-3%)
Cause: singletraumaticeventfrom excessive
(a) solitaryscleroticlesion: frequentlyin spine stretching
(b) diffusesclerosis Susceptibility factors:
Associatedwith POEMS syndrome: (1) musclecomposedof (fastcontracting)
Polyneuropathy type ll fibers
Organomegaly (2) fusiformshapeof muscle
Endocrineabnormalities (3) extensionacrosstwo joints
Myeloma,sclerotic (4) superficial locationof muscle
Skinchanges (5) eccentricmuscleaction
MR (recognition dependenton knowledgeof normalrange
of bone marrowappearancefor age): Site: myotendinous junction(= weakestpointof
r/ hypointensefocal areas on Tl Wl (25%) muscul otendi nousuni t)
r/ hyperintensefocal areas on T2Wl (53%) Classification:
{ absenceof fatty infiltration(nonspecific) 1" degree= stretchinjury(somefiberdisruptions)
. no loss of musclefunction
Y B O N ESC AN SVS . R A D IOGR A P H S
S E NS I T I V I T OF Path: interstitialedema + hemorrhageat
Radiographs: in 90% of patientsand 80% of sites myotendinous junctionwith extensioninto
Bonescan : in75/" of patientsand24-54% of sites adjacentmusclefibers
Galliumscan : in 55% of patientsand 40/" of sites r/ featheryappearanceof muscle
0 30% of lesionsonly detectedon radiographs 2" degree= partialtear withoutretraction
0 10% of lesionsonly detectedon bone scans . mild lossof musclefunction
r/ hematomaat myotendinous junction
Cx: (1) Renalinvolvement frequent ri perifascial
fluidcollection
(2) Predilectionfor recurrentpneumonias 3'degree = compl€terupture
(leukopenia) ' completelossof musclefunction
(3) Secondaryamyloidosis in 6-15% r/ retractedmuscletendon
(4) Pathologicfracturesoccur often r/ hematomaat myotendinous junction
Prognosis: 2O/" S-yearsurvival;death from renal
insufficiency,bacterialinfection, AcuteAvulsionInjury
thromboembolism Cause: forcefulunbalancedofteneccentricmuscle
DDx: contraction
- with osteopenia: (1) Postmenopausal osteoporosis Path: periostealstrippingwith hematomaat tendon
(2) Hyperparathyroidism attachmentsite
- with lyticlesion: (1) Metastaticdisease
Site: at tendoninsertion
(2) Amyloidosis . lossof function,severetenderness
(3) Myeloidmetaplasia r/ waviness+ retractionof the torn end of tendonwith
- with scleroticlesion: (1) Osteopoikilosis
fragmentof bone/ cartilage
(2) Lymphoma
(3) Osteoblasticmetastasis
MY E LOFIB R OS IS
(4) Mastocytosis = MYELOSCLEROSIS = AGNOGENIC MYELOID METAPLASIA
(5) Myelosclerosis = MYELO-PROLIFERATIVE SYNDROME
(6) Fluorosis = PSEUDOLEUKEMIA
(7) Lymphoma = hematologicdisorderof unknownetiologywith gradual
(8) Renalosteodystrophy replacementof bone marrowelementsby fibrotictissue
 Boneand Soft-Tissue
Disorders 123

Characterizedby 2. Chronicgranulomatous leukemia= chronic

(1) extramedullary hematopoiesis myelogenous leukemia
(2) progressive splenomegaly 3. Essentialidiopathic thrombocytopenia
(3) anemia 4. Agnogenicmyeloidmetaplasia(= primary
(4) variablechangesin numberof granulocytes myelofibrosis+ extramedullary hematopoiesisin liver
+ platelets;oftenpredatedby polycythemiavera + spleen)
Cause: Pathophysiology:
(a) primary:rare in children - self-perpetuating intra-and extramedullary
(b) secondary: radiationtherapy,chemotherapy hematopoietic cell proliferation
withoutstimulus
Age: usually>50 years - trilinearpanmyelosis(RBCs,WBCs,platelets)
Path: fibrous/ bony replacementof bone marrow; - myelofibrosis with progressionto myelosclerosis
extramedullary hematopoiesis - myeloidmetaplasia= extramedullary hematopoiesis
Associatedwith: metastaticcarcinoma,chemical (normocyticanemia, leukoerythroblastic anemia,
poisoning,chronicinfection(TB),acute low plateletcount,normal/ reduced
reticulocytosis,
myelogenous leukemia,polycythemia WBC count)
vera,McCune-Albright syndrome,
histiocytosis
. dyspnea,weakness,fatigue,weightloss,hemorrhage MYOSITIS OSSIFICANS
. normochromic normocyticanemia;polycythemia may = PSEUDOMALIGNANT OSSEOUS TUMOROF SOFTTISSUE
precedemyelosclerosis in 59% = EXTRAOSSEOUS LOCALIZED NONNEOPLASTIC BONE
. dry marrowaspirate ANDCARTILAGE FORMATION = MYOSITIS OSSIFICANS
CIRCUMSCRIPTA = HETEROTOPIC OSSIFICATION
Location: red marrow-containing bones in 40./. = benignsolitaryself-limiting ossifyingsoft-tissuemass
(thoraciccage, pelvis,femora,humeralshafts,
typicallyoccurringwithinskeletalmuscle
lumbarspine,skull,peripheralbones)
./ splenomegaly 0 Myositisis a misnomerfor lackof inflammation!
Cause: directtrauma(75%),paralysis,burn,tetanus,
ri widespreaddiffuseincreasein density(groundglass)
intramuscular hematoma,spontaneous
r/ '1ail-bar"ribs
Age: adolescents,young athleticadults; M > F
! sandwich/ ruggerjerseyspine
Path: lesionrimmedby compressedfibrousconnective
r/ generalizedincreasein bonedensityin skull+
tissue+ surroundedby atrophicskeletalmuscle
obliterationof diploicspace;scatteredsmall rounded
(myositis= misnomersinceno primary
radiolucent lesions;or combination of both
inflammation of musclepresent)
MR:
r/ hypointensemarrowon Tl Wl + fat-suppression Histo:
(a) early: focalhemorrhage+ degeneration + necrosis
NUC:
of damagedmuscle;histiocytic invasion;central
r/ diffuseincreaseduptakeof bone tracer in affected
nonossifiedcore of proliferatingbenignfibroblasts
skeleton,possibly"superscan"
+ myofibroblasts; mesenchymal cellsenclosedin
r/ increaseduptakeat ends of long bones
groundsubstanceassumecharacteristics of
DDx: (1) With splenomegaly:chronicleukemia,
osteoblastswith subsequentmineralizalion
lymphoma,mastocytosis
+ peripheralboneformation
(2) Withoutsplenomegaly:osteoblastic
(b) intermediate age (3-6 weeks): "zonephenomenon"
metastases,f Iuorine poisoning, osteopetrosis,
with centralarea of cellularvariationand atypical
chronicrenaldisease
mitoticfigures(impossibleto differentiatefrom soft-
tissuesarcoma);middlezone of immatureosteoid;
MYELOID
DEPLETION outer zoneof well-formedmaturetrabeculateddense
= APLASTIC ANEMIA bone
Cause: idiopathic;? sequelaeof viral infection, . pain,tenderness, mass
soft-tissue
medication,
toxin,chemo-/ radiationtherapy Location: largemusclesof extremities(80%)
Path: normalmarrowreplacedby fat cells (a) withinmuscle: anterolateral aspectof thigh+ arm;
MR:
temporalmuscle;smallmusclesof hands;gluteal
r/ nignsignalintensityon Tl Wl muscle; "rider's bone" (adductorlongus);
ri low signalintensityon fat-suppressed
T2Wl "fencer's bone" (brachialis); "dancer's bone"
0 Best seen in areaswith high percentageof (soleus);breast,elbow,knee
hematopoieticmarrow:proximalfemoralmetaphyses, (b) periostealat tendoninsertion:Pellegrini-Stieda
spine disease (in / near medial(tibial)collateralligament
of knee)as a resultof Stiedafracture(= ?vulsion
MYELOPROLIFERATIVE DISORDERS injuryfrom medialfemoralcondyleat originof tibial
= autonomousclonaldisorderinitiatedby an acquired collateralligament)
pluripotentialhematopoieticstem cell Pellegrini
(1877-1958),
surgeonin Florence,ltaly]
[Augusto
Types: [Alfred Stieda(1869-1945),surgeon in Kdnigsberg,
1. Polycythemia vera Germanyl
^/ faint calcifications developin 2-6 weeks afteronset of (6) Parostealsarcoma(usuallymetaphysealwith thick
symptoms denselymineralizedattachmentto bone)
{ well-definedpartiallyossifiedsoft-tissuemass apparent (7) Posttraumaticperiostitis(ossificationof
by 6-8 weeks,becomingsmaller+ matureby subperiostealhematomawith broad-based
5-6 months attachmentto bone)
r/ radiolucentzone separatinglesionfrom bone (8) Acute osteomyelitis(substantialsoft-tissueedema
(DDx:periostealsarcomaon stalk) + early periostealreaction)
{ + periostealreaction (9) Tumoralcalcinosis(periarticular calcificmassesof
MR: lobularpatternwith interspersedlucentsoft-tissue
r/ initialtyheterogenous muscleedema septa)
! progression to masslikeregionof high-signal intensity (10) Osteochondroma (stalkcontiguouswith normal
on T2Wl (duringfirst days to weeks after injury) adjacentcortex+ medullaryspace)
CT:
{ well-definedmineralization at peripheryof lesionafter MyositisOssificansVariants
4-G weeks + less distinctlucentcenter Panniculitis Ossificans
(DDx: sarcomawith ill-definedperiphery+ calcified Location: subcutisof mostlyupperextremities
ossificcenter) r/ lessprominentzoningphenomenon
^/ diffuseossificationin maturelesion
MR: FasciitisOssificans
Earlyphase: Location: fascia
{ mass with poorlydefinedmargins
r/ inhomogeneously hyperintenseto fat on T2Wl FibroosseousPseudotumorof Digits
{ isointenseto muscleon Tl Wl = FLORID REACTIVE PERIOSTITIS
'v contrastenhancement
= flor'il"r€oplastic
solitaryself-limitingprocessof
Intermediateohase: unknownpathogenesis,probablyrelatedto trauma
r/ isointense / slightlyhyperintense core on T1Wl, Age: mean age of 32 years (range4 -64 years);
increa6ingin intensityon T2Wl M : F= 1 : 2
{ rim of curvilinearareasof decreasedsignal . fusiformsoft-tissueswelling/ mass
intensitysurrounding the lesion(= peripheral Location: predominantly tubularbonesof hand
mineralization / ossification) + foot: fingers(2nd> 3rd > Sth)
{ increasedperitumoralsignalintensityon T2Wl Site: proximal> distal> middlephalanx
(= edema of diffusemyositis) r/ radiopaquesoft-tissuemass with radiolucentband
! focal signalabnormalitywithinbone marrow between mass + cortex
(= ffiofrowedema) r/ visiblecalcifications(50%)
Maturephase: r/ focal periostealthickening(50%)
r/ well-defined inhomogeneous masswith signal r/ corticalerosion(occasionally)
, intensity approximating fat Rx: localexcision
r/ decreasedsignalintensitysurroundinglesion DDx: parosteal/ periostealosteogenicsarcoma,
+ within (denseossification+ fibrosis,hemosiderin peripheralchondrosarcoma, periosteal
from previoushemorrhage) chondroma,soft-tissuechondroma
NUC:
r/ intensetraceraccumulationon bone scan (directly
relatedto depositionof calciumin damagedmuscle) NAIL.PATELLA SYNDROME
{ in phase of matureossificationactivitybecomes = FONGDISEASE = ILIACHORNS = FAMILIAL
/ HEREDITARY
reduced+ surgerymay be performedwith littlerisk of OSTEO-ONYCHODYSPLASIA = OSTEO-ONYCHODYS-
recurrence OSTOSIS = HOODSYNDROME = ELBOW-PATELLA
Angio: SYNDROME
.l diffusetumor blush + fine neovascularity in early = tdte autosomaldominantdisordercharacterizedby
activephase symmetricalmeso-and ectodermalanomalies
! avascularmass in maturehealingphase Etiology: ? enzymaticdefect in collagen metabolism
Age: evidentin 2nd + 3rd decades
. aplasia/ hypoplasiaof thumb+ indexfingernails
Prognosis; ? resorptionin 1 year
. bilateralspooning/ splitting/ ridgingof fingernails
DDx:
. abnormalgait
0 In early stagesdifficultto differentiatehistologically
. abnormalpigmentation of iris
+ radiologically
from soft-tissuesarcomas!
. renaldysfunction(secondaryto abnormalglomerular
(1) Osteosarcoma
(2) Synovialsarcoma basementmembrane):proteinuria, hematuria,failure
(3) Fibrosarcoma later in life
(4) Chondrosarcoma ./ bilateralposterioriliac horns in 80% (occasionally
(5) Rhabdomyosarcoma cappedby an epiphysis)DIAGNOSTIC
 Boneand Soft-Tissue
Disorders 125

r/ flared iliaccrest with protuberantanterioriliacspines NEUROPATHIC

OSTEOARTH
ROPATHY
./ genu valgumdue to asymmetricaldevelopmentof = NEUROTROPHIC JOINT= CHARCOT JOINT
femoralcondyles = traumaticarthritisdue associatedwith loss of sensation
./ prominenttibialtubercles + proprioceptionof affectedlimb
r/ fragmentation/ hypoplasia/ absenceof patella;
frequentlywith recurrentlateraldislocations Pathogenesis;(1) decreasedpain sensationproduces
r/ radialheadi capitellumhypoplasiawith subluxation/ repetitivetrauma
dislocationof radialhead dorsallyand increased (2) sympatheticdysfunctionresultsin local
carryingangleof elbow(DDx:congenitaldislocation of hyperemia+ bone resorption
, radialhead)
r/ clinodactylyof Sthfinger Cause:
r/ short Sth metacarpal A. Congenital
r/ flexioncontracturesof hip, knee,elbow,fingers,foot 1. Myelomeningocele
r/ deltoid,triceps,quadricepshypoplasia 2. Congenitalindifference to pain = asymbolia
r/ mandibularcysts (occasionally) 3. Familialdysautonomia (Riley-Daysyndrome)
r/ scoliosis 4. Hereditarysensoryand motor neuropathy
^/ renalosteodystrophy (Charcot-Marie-Tooth disease)
DDx: (1) Seckelsyndrome= bird-headeddwarfism B. Acquired
(2) Poplitealpterygiumsyndrome(absenceof (a) centralneuropathy
patella,toenaiI dysplasia) 1. Injuryto brain/ spinalcord
2. Syringomyelia (in 1/3at patients):shoulder,
elbow
NECROTIZING
FASCIITIS 3 . Neurosyphilis = tabesdorsalis(in 15-20% of
lncidence; 500 cases in literature patients):hip,knee,ankle,tarsals
Age: 58 + 14 years;M>F 4 . Spinalcord tumors/ infection
Cause: deep internalinfectionI malignancy(perforated 5 . Extrinsiccompressionof spinalcord
duodenalulcer/ retroperitoneal appendix, 6 . Multiplesclerosis
retroperitoneal / perirectalinfection,infiltrating 7 . Alcoholism
rectal/ sigmoidcarcinoma (b) peripheralneuropathy
Predisposed: patientswith diabetes,cancer,alcohol/ 1. Diabetesmellitus(mostcommoncause,
drug abuse,poor nutrition althoughincidencelow): ankle,foot,hand
Organism; Staphylococcus, E.coli, Bacteroides, 2. Peripheralnerve injury
Streptococcus,Peptostreptococcus, 3. Peripheralnervetumor
Klebsiella, Proteus,C. perfringens (5-1S%) 4. Leprosy(Hansendisease)
(multipleorganismsin 75%) 5. Poliomyelitis
Histo: necroticsuperficialfascia,leukocyticinfiltrationof (c) others
deep fasciallayers;fibrinoidthrombosisof 1. Scleroderma, Raynauddisease,Ehlers-
arterioles+ venuleswithvesselwall necrosis: Danlossyndrome
microbialinfiltrationof destroyedfascia 2. Rheumatoidarthritis,psoriasis
. indolent(1-21 days delay beforediagnosis) 3. Amyloidinfiltration of nerves,adrenal
. nonspecificsymptoms: severepain,fever,leukocytosis, hypercorticism
shock,alteredmentalstatus 4. U remi a
. crepitus(50%),overlyingskin may be completelyintact 5. Perniciousanemia
C. latrogenic
Location: lowerextremity,arm, neck,back, male 1. Prolongeduse of pain-relieving drugs
, perineum/ scrotum(= Fourniergangrene) 2. lntraarticular
/ systemicsteroidinjections
r/ asymmetricfascialthickeningwith fat stranding(80%)
from fluid mnemonic.' "D56"
{ gas in soft-tissuesdissectingalongfascialplanesfrom Diabetes
gas-forming organisms(in 55%) Syphilis Spinabifida
r/ associateddeep abscess (gS%) Steroids Syringomyelia
r/ + secondarymuscleinvolvement Spinalcord injury Scleroderma
Prognosis; poor with delay in diagnosis
Rx: extensivesurgicafd6bridement
DDx: (1) Myonecrosis (infectionoriginatingin muscle) Pathophysiology:
(2) Fasciitis-panniculitis
syndromes(chronic Loss of proprioception with sensorydeficitsarisingin
swellingof skin + underlyingsofttissues the spinalcord / peripheralnerves
+ fascialplanesin arm + calf) (1) Neurotraumatic theory
(3) Softtissueedemaof CHF / cirrhosis = repetitivetraumawith absenceof normal
(symmetricaldiffusefat stranding) protectivesensory feedback
 (2) Neurovascular theory r/amputatedappearanceof proximalhumerus
= absenceof neuralstimulileadsto a lossof r/dislocation
sympathetictone resultingin vasodilatationand r/largejointeffusion
hyperemia,which promotesbone resorption r/fragmentedosseousdebrisin jointcapsule
+ weakeningof subchondralbone + subacromial-subdeltoid bursa
Pathology: DDx: chondrosarcoma
(a) atrophicpattern(mostcommon): @ Hands+ feet
osseousresorptionin which osteoclasts Cause: leprosy(dueto trauma+ secondarybacterial
+ macrophagesremovebone + cartilagedebris infection)
makingbone susceptibleto fractures { claw hand / claw toes
Associatedwith: syringomyelia,peripheralnerve r/ lict<edcandy stick phalanx= taperedphalanx(due to
lesion concentricbone atrophywith decreasein bone
Location: non-weight-bearing joints of upper length+ width)
extremitY DDx: diabetesmellitus,frostbite,perniciousanemia,
DDx: surgicalamputation,septicarthritis scleroderma, syringomyelia,tabesdorsalis,
(b) hypertrophicpattern: familialsensoryneuropathy
joint destruction+ fragmentation,osseoussclerosis, @ Spine (involvedin 6-21"/.):
osteophyteformation(earlyattainingenormoussize) Cause: traumaticspinalcord injury,inadequately
DDx: severe osteoarthritis treatedsyphilis,amyloidosis, congenital
(c) mixed pattern insensitivity to pain,diabetesmellitus
(d) commonto both: Site: thoracolumbarjunction,lumbarspine
jointdisorganization, largepersistentbloodyjoint r/ oist<space narrowing
effusion { osteolysis/ sclerosisof vertebrae+ facetjoints
{ subluxation
. no historyof trauma r/ abruptcurvature
. swollen+ warmjointwith normalWBC count+ ESR r/ scoliosis
(infectionmay coexist) { paraspinoussoft-tissuecalcification
. usuallypainlessjoint;pain at presentation (in 1/3)with r/ large hypertrophicbeakingendplateosteophytes
decreasedresponseto deep pain + proprioception r/ extensiveosseousfragmentationwith extension
. jointchangesfrequentlyprecedeneurologicdeficit beyondconfinesof vertebralbody margininto
. synovialfluid: frequentlyxanthochromic / bloody,lipid paraspinousmusculature + intospinalcanal
crystals(frombone marrow) DDx: vertebralosteomyelitis,metastasis,
r/ persistentjoint effusion(firstsign) granulomatousinfection
{ narrowingof joint space @ Foot + ankle
{ speckledcalcificationin soft tissue (= calcificationof Cause: long-termpoorlycontrolleddiabetesmellitus,
synovialmembrane) syP hi l i s
^/ fragmentationof eburnatedsubchondralbone . soft-tissueswelling,warmth,erythema
{ NO juxtaarticularosteoporosis(unlessinfected) Site: oftenbeginsin midfoot
r/ "bag-of-bones" appearancein late stage (= marked ./ vascularcalcifications
deformitiesaroundjoint) { subluxation(startingat 2nd tarsometatarsal joint)
mnemonic.' "6 Ds" r/ avulasionfractureof posterior tubercleof calcaneus
{ Densesubchondral bone (= sclerosis) r/ subchondralfractureof head of 2nd metatarsal
{ Degeneration(= attemptedrepairby osteophytes) ^/ talonaviculardisplacementwith midfootarthropathy
r/ Destructionof articularcortex(withsharp margins
resemblingthose of surgicalamputation)
^/ Deformity("pencilpoint"deformityof metatarsal TENOSYNOVITIS
NODULAR
heads) = GIANTCELLTUMOROFTENDON SHEATH
.l Debris(loosebodies) = benignproliferativelesionof synovialoriginviewedas
./ Dislocation(nontraumatic) form of PVNS
an extraarticular
r/ subluxationof joints (laxityof periarticularsoft tissues) Age: youngadult
r/ progressiverapidbone resorption Path: very cellulartumorwith a capsulethat separates
./ lointdistension(by fluid,hypertrophic synovitis, the tumorinto lobules
osteophytes,subluxation) Histo: multinucleated giant cells,macrophages,
r/ fracture: healingwith exuberantbizarrecallusformation fibroblasts,xanthomacells,varyingamountsof
MR: hemosiderin
{ decreasedsignalintensityin bone marrowon Tl Wl
+ T2Wl (due to osteosclerotic changes) Location: hand,occasionally ankle/ foot
@ S houlder 0 One of the most commontumorsof the
Cause: syringomyelia,cord traumawith paraplegia hand!
. shouldermass (dueto fluiddistension) Site: tendonsheath,jointcapsule,bursa,ligament
 Boneand Soft-Tissue
Disorders 127

r/ lobulatedlesionwithwell-defined nodulesup to 4 cm in Prognosis; spontaneoushealingin most cases

size Cx: (1) Pathologicfracture(not uncommon)
r/ locatedalongtendonsheath(CHARACTERTSTTC) (2) Hypophosphatemic vitaminD--resistant rickets
./ boneerosion(iS%) + osteomalacia(tumormay secretesubstance
MR: that increasesrenaltubularresorptionof
./ low signalintensityon Tl Wl + T2Wl (hemosiderin phosphorus)
deposition) DDx: (1) Adamantinoma (midshaftof tibia)
^/ homogeneous contrastenhancement (2) Chondromyxoid fibroma(bulgingof cortexmore
DDx: (1) Floridproliferativesynovitis(diffuseinfiltrative striking,hyperintense on T2Wll)
subtypeof pVNS)
form of GCT = extraarticular (3) Fibrousdysplasia(internalseptationsrare)
(2) Pigmentedvillonodular synovitis (4) Aneurysmalbone cyst (heterogeneously
(3) Desmoidtumor hyperintense with fluid-fluidlevels)
(4) Malignantfibroushistiocytoma (5) lntraosseous ganglion(hyperintense on T2Wl)

Jaff6-Campanacci Syndrome
NO NO S S I F Y I NG F IB R OMA = flor'rosSifyingfibromawith extraskeletalmanifestations
= FIBROXANTHOMA = NONOSTEOGENIC FIBROMA i n chi l dren
= XANTHOMA = XANTHOGRANULOMA OF BONE . mentalretardation
= FIBROUS METAPHYSEAL-DIAPHYSEAL DEFECT . hypogonadism
= FIBROUS MEDULLARY DEFECT . oculardefect
lncidence: up to 40/" of all children>2 years of age . cardiovascular congenitaldefect
Etiology: lesionresultingfrom proliferativeactivityof a . caf6-au-laitspots
fibrouscorticaldefectthat has expandedinto
medullarycavity
Histo: interlacingwhorledbundlesof spindle-shaped NOONAN SYNDROME
fibroblasts+ scatteredmultinucleated giantcells = PSEUDO_TURNER = MALETURNER SYNDROME
+ foamyxanthomatous cells,variabledegreeof = phenotypesimilarto Turnersyndromebut with normal
hemosiderin; usuallycellularwith only small karyotype(occursin both males+ females)
amountsof collagen Strikingfamilialincidence
Age: 8-20 years;75/"in 2nd decadeof life . shortI may have normalheight
. usuallyasymptomatic;pain if large . webbedneck
Location: shaft of long bone; mostlyin bonesof lower . agonadism/ normalgonads
extremity,especiallyaboutknee (distalfemur ' delayedpuberty
+ pr o x i m atil b i a );d i s tatil b i a ;fi b u l a . mentalretardation
Site: eccentricmetaphyseal,severalcm shaftwardfrom r/ osteoporosis
epiphysis,mostlyintramedullary, rarelypurely r/ retardedbone age
diaphyseal r/ cubitusvalgus
Multiplefibroxanthomas (in 8-1 0%): @ S kul l
Associatedwith: neurofibromatosis, fibrousdysplasia, r/ mandibularhypoplasia with dentalrnalocclusion
Jaff6-Campanacci syndrome ri hypertelorism
r/ biparietalforamina
r/ multilocularovoidbubblyosteolyticarea r/ dolichocephaly, microcephalyI cranialenlargement
r/ alignmentalonglongaxis of bone,about2 cm in length { webbedneck
ri densescleroticbordertowardmedulla;V- or U-shaped @ Chest
at one end { sternaldeformity: pectusexcavatum/ carinatum
^/ endostealscalloping+ thinning+ eysplying bulge ri right-sided congenitalheartdisease(valvarpulmonic
r/ migratestowardcenterof diaphysis stenosis,ASD, eccentrichypertropfryof left ventricle,
./ resolveswith age P D A ,V S D )
N UC: { m inim a l /m i l du p ta k eo n b o n es c a n r/ coronalcleftsof spine
MR: ^i may have pulmonarylymphangiectasis
r/ AOZ hypointenseon Tl Wl + T2Wl (extensive @ Gastrointestinal tract
hypocellular
fibroustissue,hemosiderin pigment) r/ intestinallymphangiectasia
^l ZOt" hypointense on TlWl + hyperintense on T2Wl ri eventrationof diaphragm
(massiveaggregationof foamy histiocytes) r/ renalmalrotation, renalduplication,hydronephrosis,
r/ peripheralhypointenserim + internalseptation large redundantextrarenalpelvis
, (marginalreactivesclerosis+ trabeculation) DDx: Turnersyndrome(mentalretardationrare, renal
r/ intensecontrastenhancement(in B0%)/ marginal anomaliesfrequent)
septalenhancement(in 20%)on Tl Wl
CAVE: lesions>33 mm long involving>50%of the OCHRONOSIS
transversebone diameterneed observation = ALKAPTONURIA
 = inheritedabsenceof homogentisicacid oxidasewith . localpain+ tendernesson pressure
excessivehomogentisicacid production+ depositionin . swellingof overlyingsofttissue
connective tissueincludingcartilage, synovium,and bone r/ soft-tissue swellingin frontof tuberosity(= s6st..1
Histo: black-pigmented cartilagesubjectto deterioration skin + subcutaneous tissue)
resultingin calcification+ denudationof .1 thickeningof distalportionof patellartendon
cartilaginous tissue ./ indistinctmarginof patellartendon
M : F= 2 : 1 r/ increasedradiodensityof infrapatellar fat pad
. blackpigmentin softtissues(in 2nd decade):yellowish { avulsionwith separationof smallossiclesfrom the
skin;gray pigmentation of sclera;bluishtingeof ears developingossificationcenterof tibialtuberosity
+ nose cartilage { single/ multipleossifications in avulsedfragment
. alkaptonuriawith blackstainingof diapers { comparisonwith otherside ( irregulardevelopment
. heartfailure,renalfailure(pigmentdeposition) normal)
@ S pine MR:
Age: middleage ^/ increasedsignalintensityat tibialinsertionsite of
Site: lumbarregionwith progressiveascension patellartendonon Tl Wl + T2Wl
r/ laminatedcalcificationof multipleintervertebral disks ^/ distensionof deep infrapatellar bursa
r/ severenarrowingof intervertebral disk space ./ bone marrowsignalchangesin tibialtuberosity+ tibial
r/ multiple"vacuum"phenomena(common) apophysis(rare)
r/ osteoporosisof adjoiningvefiebrae Cx: nonunionof bonefragment,patellarsubluxation,
./ massiveosteophytosis+ ankylosisof spine (in older chondromalacia, avulsionof patellartendon,genu
patient) recurvatum
{ spottycalcificationsin tissueanteriorto vertebral Rx: immobilization / steroidinjection
bodies DDx: (1) Normalossificationpatternof tibialtuberosity
@ Joints betweenages 8-14 (no sYmPtoms)
r/ hypertrophicchangesin humeralhead (2) Osteitis:tuberculous / syphilitic
{ severeprematureprogressiveosteoarthritic changes (3) Soft-tissuesarcomawith calcifications
in shoulder,knee,hip,spineof youngpatients
./ intraarticularosseousbodies
./ smallcalcificationsin paraarticularsoft tissues OS S IFY IN G FIB R OMA
+ tendoninsertions Closelyrelatedto fibrousdysplasia+ adamantinoma
Age: 2nd-4th decade; M < F
Histo: maturingcellularfibrousspindlecellswith
SYNDROME
ORODIGITOFACIAL
= OROFACIODIGITAL osteoblasticactivityproducingmany calcific
SYNDROME
= group of heterogeneousdefects,probablyrepresenting cartilaginous+ bonedensities
varyingexpressivity,involvingface, oral cavity,and limbs Location:frequentlyin face
Etiology: autosomaltrisomyof chromosomeNo. 1 with @ Mandible,maxilla
. painlessexpansionof tooth-bearingportionof jaw
47 chromosomes; X-linkeddominant
^/ t-S cm well-circumscribed round/ ovaltumor
Sex; nuclearchromatinpatternfemale (lethalin male)
./ moderateexpansionof intactcortex
Associatedwith: renal polycysticdisease
. mentalretardation r/ homogeneous tumormatrix
. hypertelorism ./ distodgmentof teeth
. cleftlip + tongue,lingualhamartoma @ Tibia
. bifidnasaltip r/ eccentricground-glass lesion(resemblingfibrous
r/ cleft in palate+ jaw bone dysplasia)
r/ hypoplasiaof mandible(micrognathia) + occiputof skull Cx: frequentrecurrences
r/ hypodontia
r/ clinodactyly,syndactyly,brachydactyly(metacarpals
may be elongated),polysyndactyly, duplicationof hallux OSTEITIS CONDENSANS ILII
lncidence: 2'/. of population
Cause: chronicstresssecondaryto instabilityof pubic
DISEASE
OSGOOD.SCHLATTER symphysis
Age: youngmultiparous women
[RobertB. Osgood(1873-1956), Bostonorthopedic surgeon]
. associatedwith low back pain when instabilityof pubic
(1864-1934),surgeonin Zurich,Switzerland]
[CarlSchlatter
= traumaticallyinduceddisruptionof the attachmentof the symphysispresent
patellarligamentto the tibialtuberosity(chronicfatigue r/ triangularareaof sclerosisalong inferioranterioraspect
injury,NOT osteonecrosis); bilateralin25/" of ileumadjacentto Sl joint (jointspaceuninvolved)
Age: 10-15 years; M > F { similartriangleof reparativeboneon sacralside
Cause: trauma (commonin sportsthat involvejumping, { usuallybilateral+ symmetric;occasionally unilateral
kicking,squatting)= ? cartilaginous avulsion r/ sclerosisdissolvesin 3-20 yearsfollowingstabilization
fracture,? tendinitis of pubic symphysis
 Boneand Soft-TissueDisorders 129

DDx: (1) Ankylosingspondylitis(affectsilium+ sacrum, @ Hip

jointspacenarrowing,involvement of other r/ femoral+ acetabularosteophytes,sclerosis,
bones) subchondralcyst formation
(2) Rheumatoidarthritis(asymmetric, joint r/ thickening/ buttressingof medialfemoralcortex/
destruction) calcar
(3) Pagetdisease(thickenedtrabecularpattern) r/ migrationof femoralhead
! superolateralsubluxationof femoralhead
OSTEOARTHRITIS r/ medialI axialsubluxation+ protrusioacetabuli(in
= DEGENERATIVE JOINTDISEASE ,20%)
= predominantly noninflammatory degeneration of r/ primaryhereditaryprotrusio= Otto pelvis(M . F)
cartilagein synovialjoints @ Knee
Cause: (1) abnormalforcesactingon a normaljoint(eg, Location: medial> lateralfemorotibial>
slippedcapitalfemoralepiphysis) patellofemoral compartment
(2) normalforcesactingon abnormaljointdue to r/ varus deformity(MttF)
(a) cartilageabnormality @ S pi ne
(b) subchondral bone abnormality { sclerosis+ narrowingof intervertebral apophyseal
Path: decreasedchondroitinsulfatewith age creates joints
unsupportedcollagenfibrilsfollowedby irreversible { osteophytosisusuallyassociatedwith diskogenic
hyalinecartilagedegeneration (= inabilityfor disease
regeneration) @ Sacroiliac joint
Stages: 0 Mostcommondisorderof sacroiliac joints
I cartilageswelling+ softeningfrom damageto Location:bi- / unilateral(contralateralSIJ with bad hip)
collagenmatrixresultingin decreasedproteoglycan ./ diffusejoint space loss
contentand aggregatesize + increasein water ! vacuumphenomenon
content ! well-defined lineof sclerosis,especiallyon iliacside
ll increasedcartilagethicknessfrom proliferationof of articulation
chondrocyteswith increasein anabolic+ catabolic { prominentbridgingosteophyteat superior+ inferior
activity limitsof joint
lll cartilagelosswithcontinueddamagedue to DDx: osteoblasticmetastasis
decreasein cellularproliferationof chondrocytes
resultingin fibrillation
+ erosion+ crackingof ErosiveOsteoarthritis
articularcartilage = inflammatoryform of osteoarthrosis
^/ loint space narrowing(stagelll) = insssurateindicatorof Predisposed: postmenopausalfemales
cartilageintegrity Site: distribution identicalto noninflammatory
r/ subchondralsclerosis/ eburnationin areas of stress DIP > PIP > MCPjointsof hands;
osteoarthritis:
r/ subchondralcyst formation(geodes) radialaspectof wrist;bilateral+ symmetric
./ increasedjointfluid ^/ "bird-wing"/ "sea-gull"
jointconfiguration
= central
r/ synovialinflammation(in severeosteoarthritis) erosions+ osteophytosis
r/ osteophytosisat articularmargin/ nonstressedarea r/ may lead to bony ankylosis
MR: DDx: Rheumatoidarthritis,Wilsondisease,chronic
r/ increasedsignalintensityof abnormalcartilageon liverdisease,hemochromatosis
T2Wl (= increasedamountof free water)
r/ morphologicdefectson surfaceof cartilage(best EarlyOsteoarthritis
seen on fat-suppressed spoiledgradient-echoMR) mnemonic;"EarlyOsteoArthritis"
Epiphyseal
dysplasia,
multiple
@ Hand + foot Ochronosis
Targetarea: 1st MCP; trapezioscaphoid; DIP > Acromegaly
P I P ;1 s tM T P
^/ lossof jointspace,subchondral eburnation,
marginal MilwaukeeShoulder
osteophytes, smallossiclesin DIP + PIP: = ?SSoci€ltionof
{ Bouchardnodes= osteophytosisat PIP joint (1) completerotatorcuff tear
r/ Heberdennodes= osteophytosisat Dlploint: (2) osteoarthritic
changes
M : F= 1: 1 0 (3) noninflammatory jointeffusioncontainingcalcium
r/ radialsubluxationof 1st metacarpalbase hydroxyapatite and calciumpyrophosphate
r/ loint space narrowing+ eburnationof dihydrate(CPPD)crystals
trapezioscaphoid area (4) hyperplasiaof synovium
@ Shoulder (5) destructionof cartilage+ subchondralbone
r/ elevationof humeralhead + lack of significant (6) multipleosteochondralloose bodies
glenohumeral joint involvement (DDxto rheumatoid Age: olderwomen
arthritis) . frequenthistoryof trauma
 Radiology Review Manual

. rapidlyprogressive arthritisof shoulder Location:(rarelymultifocal)

Radiograph: (a) spine (33-37%): 62-94% in posteriorelements,
1
joint space narrowing secondaryextensioninto vertebralbody (28-42%);
r/ subchondralsclerosis+ cyst formation cervicalspine(31%),thoracicspine(34%),lumbar
r/ destructionof subchondralbone spine(31%),sacrum(3%)
r/ soft-tissueswelling (b) longbones(26-32%): femur(50%),tibia(19%),
./ capsularcalcifications humerus(19%),radius(8%),fibula(%); unusualin
r/ intraarticular
loosebodies neckof femur
MR: (c) small bones of hand + feet (15-26%): dorsaltalus
r/ largeeffusion neck(62/"), calcaneus(4%),scaphoid(8%),
{ completerotatorcuff tear metacarpals(8%), metatarsals(8%)
{ narrowingof glenohumeral joint (d) calvarium+ mandible(= cem€ntoblastoma)
Site: diaphyseal(58%),metaphyseal (42%);eccentric
RapidlyDestructiveArticularDisease (42%),centric(12/"), may be
(46/"), intracortical
= UfTUSU€llform of osteoarthritis
typicallyinvolvingthe periosteal
hip (almostalwaysunilateral)
Age: elderlywomen { similarto osteoidosteoma:
Associatedwith: conventionalosteoarthritisin hands, ri radiolucent nidus>2 cm (rangeof 2-12 cm) in size
wrists,knees,oppositehip { well demarcated(83%)
. hip pain 'V + stippled/ ringlikesmallflecksof matrixcalcification
{ progressiveloss of joint space r/ reactivesclerosis(22-91%)I no sclerosis(9-56%)
r/ lossof subchondral bone in femoralhead+ acetabulum ri progressiveexpansilelesionthat may rapidlyincrease
resultingin "hatchet"deformityof femoralhead in size (25%):
r/ superolateralsubluxationof femoralhead / intrusion r/ corticalexpansion(75-94%)/ destruction(20-22%)
deformitywithinilium r/ tumor matrixradiolucent(25-64%)/ ossified
r/ no / small osteophytes (36-72%)
Prognosis; rapiddestructionof hip within 14 monthsafter { sharplydefinedsoft-tissue component
onsetof symptoms ^/ tnin shellof periostealnew bone (58-77%)I no
Rx: totaljointreplacement periostealreaction
DDx: osteonecrosis, septicarthritis,neuroarthropathy, r/ scoliosis(35%)
crystal-induced arthropathy r/ osteoporosisdue to disuse+ hyperemiain talar location
r/ rapidcalcificationafter radiotherapy
OSTEOBLASTOMA CT:
= GIANTOSTEOID OSTEOMA = OSTEOGENIC FIBROMA OF r/ multifocalmatrixmineralization, sclerosis
BONE= OSSIFYING FIBROMA r/ expansilebone remodeling, thin osseousshell
= tdte benigntumorwith unlimitedgrowthpotential NUC:
+ capabilityof malignanttransformation ^/ intensefocalaccumulation of bone agent(100%)
lncidence: <1ohof all primarybone tumors;3% of all A ngi o:
benignbonetumors r/ tumor blush in capillaryphase (50%)
Age: mean age of 16-19 years;6-30 years (90%);2nd MR:
decade(55%);3rddecadeQA%); M:F = 2:1 ^/ low to intermediate signalintensityon Tl Wl
Path: lesion>1.5cm; smallerlesionsare classifiedas r/ mixedintermediate to high intensityon T2Wl
osteoidosteoma ^/ surrounding edema
Histo: numerousmultinucleated giant cells (osteoclasts), Prognosis: 10/" recurrenceafter excision;incomplete
irregularlyarrangedosteoid+ bone;very vascular curettagecan effectcure due to cartilage
connectivetissuestromawith interconnecting production+ trappingof host lamellarbone
trabecularbone;trabeculaebroader+ longerthan DDx:
in osteoidosteoma (1) Osteo-/ chondrosarcoma (periosteal
new bone)
. asymptomatic in <2/" (2) Osteoidosteoma(densecalcification+ halo of
. dull localizedpain of insidiousonset(84%),worseat bone sclerosis,stablelesionsize <2 cm due to
nightin 7- 13 % limitedgrowthpotential)
. responseto salicylatesin 7% (3) Cartilaginous tumors(lumpymatrixcalcification
. localizedswelling,tenderness,decreasedrangeof (4) Giantcell tumor(no calcification, epiphyseal
motion(29%) involvement)
. painfulscoliosisin 50% (withspinal/ rib location) (5) Aneurysmalbone cyst
secondaryto musclespasm,may be convextoward (6) Osteomyelitis
sideof tumor (7) Hemangioma
. paresthesias, mildmuscleweakness,paraparesis, (8) Lipoma
paraplegia(due to cord compression) (9) Epidermoid
. occasionalsystemictoxicity(highWBC, fever) (10) Fi brousdyspl asi a
 Boneand Soft-TissueDisorders 1 3 1

(11) Metastasis Rx: surgicalexcision(2o/"recunencerale, 13/"

(12) Ewingsarcoma complicationrate [neuropraxia,arteriallaceration,
compartmentsyndrome,f racture])
Cx:
OSTEOCHONDROMA (1) Osseousand cosmeticdeformity(mostfrequent)
= OSTEOCARTILAGINOUS EXOSTOSIS . mechanicallimitationof joint movement
= developmentalhyperplastic/ dysplasticbone . snappingtendoni ligament
disturbance;growthends when nearestepiphysealplate . hematuria(irritating pubicosteochondroma)
fuses { saucerization/ scallopingof cortexof adjacent
0 Mostcommonbenigngrowthof the skeleton! bonedue to extrinsicpressureerosion(of paired
0 Mostcommonbenigncartilage-containing tumor! tubularbones)
Etiology: separationof a fragmentof physealcartilage r/ prematureosteoarthritis
herniatingthroughperiostealbone cuff that { pleuraleffusion/ spontaneous hemothorax(dueto
surroundsthe growthplate (encocheof irritatingrib lesion)
RanvieQ;the fragmentcontinuesto grow and (2) Fracturethroughstalkof osteochondroma
undergoesenchondralossification (3) Vascularcompromise
(a) microtrauma / Salter-Harrisinjurywith in vivo venous/ arterialstenosis
transplantation of physealtissue arterialocclusion/ venousthrombosis
(b) radiationtherapy(in 6-24%) with latencyperiodof pseudoaneurysm formation:
3-17 years in patientsbetween8 monthsand 11 Cause: repetitivetraumato vesselwall
years of age receiving1,500-5,500cGy (frequently Age: near end of normalskeletalgrowth
for treatmentof neuroblastoma / Wilmstumor) Location: popliteala., brachiala., superficial
0 Mostcommonbenignradiation-induced tumor femorala., posteriortibiala.
Path: continuityof lesionwith marrow+ cortexof host (4) Neurologiccompromise
bone (HALLMARK) peripheralnervecompressionwith entrapment
Histo: hyalinecartilagecap containinga basalsurface neuropathy:foot dropwith peronealnerve
with enchondralossification (thincortex+ involvement(mostfrequent)
trabecularbone + marrowspace) centralnervecompression:cranialnervedeficit,
{ continuityof bone cortexwith host bone cortex radiculopathy, caudaequinasyndrome,cord
r/ continuityof medullarymarrowspacewith host bone compression with myelomalacia
r/ hyalinecartilaginouscap: r/ oftenvery narrowstalk of attachment
, r/ arcs / ringsi flocculentcalcifications on radiographs { difficultimagingdiagnosisowingto complex
{ growthpointingaway from nearestjoint + towardcenter anatomyof skullbase (21%TP)
of shaft: { spinalcanalosteochondroma (15% FN)
{ at rightangleon diaphysealsideof stalk (5) Reactivebursaformation(in 1.5%)
{ slopeon epiphysealside . enlargingmassoverlyingan osteochondroma
CT: simulatingmalignanttransformation
{ optimaldepictionof cortical+ marrowcontinuitywith Location: scapula(>50%),lessertrochanter,
hostbone (PATHOGNOMONIC) shoul der
r/ nonmineralized cartilagecap hypodenseto muscle(in r/ ttuiO-tittedmass+ chondralfillingdefects:
75-80%): { mineralization of intrabursalchondralbodies
r/ O-A mm thick in skeletallymaturepatients may mimica thick cartilagecap with growth
r/ up to 30 mm thickin skeletallyimmaturepatients Cx: inflammation, infection,hemorrhageinto
MR: bursa,secondarysynovialchondromatosis
r/ cortical+ medullarycontinuity(MR best modality) (6) Malignanttransformation intosecondarylperipheral
r/ hyalinecartilagecap very hyperintenseon T2Wl + of chondrosarcoma / osteosarcoma
intermediate intensityon Tl Wl: Frequency: 1"/" in solitary osteochondroma; <5o/"in
r/ hypointensemineralizedareas of cartilage hereditarymultipleosteochondromatosis
r/ hypointense periphery= perichondrium Location: iliacbonecommonestsite
{ slightseptal+ peripheralenhancement 0 Any cartilagecap >1.5cm thickis suspectof
US; malignanttransformation !
^/ hypoechoicnonmineralized cartilaginous cap easily mnemonic; "GLAD PAST"
distinguished from muscleand fat Growthafter physealclosure
r/ posterioracousticshadowingfor mineralizedportion Lucency(new radiolucency)
NUC: Additionalscintigraphicactivity
r/ activelesion(predominantly in young patient) Destruction(cortical)
r/ quiescentlesionin olderpatient Pain after puberty
And
Prognosis.'exostosisbeginsin childhood;stops growing Soft-tissuemass
when nearestepiphysealcenterfuses Thickenedcartilaginous cap >1.5cm
OSTEOCHON DROMAVARIANTS @ Lowerextremity
1. Dysplasiaepiphysealis hemimelica ri coxa valga (25%)
2. Subungualexostosis r/ genu valgus (2040%)
3. Turretexostosis r/ valgusdeformityof ankle = tibiotalartilt (4$-54%)
4. Tractionexostosis(at tendinousattachments) { undertubulation with widenedmetadiaphyseal
5. Bizarreparostealosteochondromatous proliferation j uncti on:
= Nora lesion r/ Erlenmeyerflask deformityof distalfemur
6. Floridreactiveperiostitis C T:
{ wavy pelvissign = small sessilelesioncreate
Sol itary Osteochondroma undulatingcorticalcontour
Frequency: 1-2o/o;M:F = 1.6:1-3.4:1
20-50% of benignbone tumors; OSTEOCHON DROSIS DISSECANS
10-15% of all bonetumors = OSTEOCHONDRITIS DISSECANS
Age: 1st-3rddecade; M:F = 1.5:1 = OSTEOCHONDRAL FRACTURE
. incidentalnontenderpainlessmass nearjoints = fragmentation+ possibleseparationof a portionof the
. symptomatic(in 75o/"beforethe age of 20 years) articularsurface
Site: metaphysisof long bones;rarelydiaphysis Etiology:
Location:in any bone that developsby enchondromal (1) subchondral fatiguefractureas a resultof shearing,
femur(30%),tibia(15-20/"),
calcification; rotatory/ tangentiallyalignedimpactionforces
about knee (40%),humerus(10-20%),hands (2) ? autosomaldominanttrait associatedwith short
and feet (10%),pelvis(5%),scapula(4/"), rib stature,endocrinedysfunction, Scheuermann
(3%),spine (2h, cervical[esp. Czl, thoracic disease,Osgood-Schlatter disease,tibia vara, carpal
[T8tT4] rlumbar) tunnelsyndrome
Type: (a) pedunculatedosteochondroma = nsllow Stolk A ge: adol escence; M> F
(b) sessileosteochondroma= broadbase . asymptomatic/ vague comPlaints
. clicking,locking,limitationof motion
. swelling,pain aggravatedby movement
HereditaryMultipleExostoses
= DTAPHYSEAL ACLASTS (ACLASIA) = MULTIPLE Location: (a) knee: medial(in 10% lateral)femoral
oSTEoCHoNDROMAS = FAM|LIAL condylecloseto fossa intercondylaris;
OSTEOCHONDROMATOSIS bilateralin 20-30h
= rTrostcommonof osteochondrodysplasias (b) humeralhead
characterizedby formationof multipleexostoses (c) capitellumof elbow
Prevalence: 1:50,000-1:100,000; (d) talus
1 :1 ,0 0 0o n Gu a m/ M a ri a n al s l ands { purelycartilaginous fragmentunrecognized on plainfilm
Genetics: autosomaldominant(incompletepenetrance r/ fractureline parallelsjoint surface
in females);3 distinctlocion chromosomes { mouse= osteochondrotic fragment
8 ,1 1 , 1 9 Location: posteriorregionof kneejoint,olecranon
0 213of affectedindividualshave a positive fossa,axillary/ subscapularrecessof
familyhistory gl enohumeral j oi nt
Age: forms shortlyafter birth;virtuallyall patients { mousebed = sclerosedpit in articularsurface
d y l 2 y e a rs o f a g e ; M:F = 1.5:1.0
dis c o v e re b { soft-tissueswelling,ioint effusion
. short stature(40%)due to developmentof exostoses DDx: spontaneousosteonecrosis,neuroarthropathy,
at the expenseof longitudinalbone growth degenerativejoint disease,synovial
Location:multiple+ usuallybilateral;knee(70-98 ), osteochondromatosis
humerus(50-98%),scapula+ rib (40%),
elbow (35-40%),hip (30-907o),wrist
(30-60%),ankle (25-54/"), hand (20-30%), OSTEOFIBROUS DYSPLASIA
foot (10-25/"), pelvis(5-15%), vertebra(7"h) = entitypreviouslymistakenfor fibrousdysplasia
Site: metaphysesof long bones near epiphysealplate Age: newbornup to 5 years
(distanceto epiphysealline increaseswith growth) Histo: fibroustissuesurrounding trabeculaein a whorled
r/ disproportionate shorteningof an extremity(50%) storiformpattern
@ Upperextremity Location: normallyconfinedto tibia(middiaphysis in
{ pseudo-Madelung deformity: 50o/"),lesion beginsin anteriorcortex;
r/ ulnarshortening+ longerbowed radius ipsilateralfibulaaffectedin 20/"
r/ ulnartilt of distalradialarticularsurface { enlargement of tibiawith anteriorbowing
r/ ulnardeviationof hand { cortexthin/ invisible
r/ dislocationof radialhead { periostealexpansion
{ radioulnarsynostosis { scleroticmargin(DDx: nonosteogenic fibroma,
r/ shorteningof 4th + 5th metacarpals chondromyxoidfibroma)
! supernumeraryfingers/ toes { spontaneousregressionin 1/3
 Boneand Soft-Tissue
Disorders 133

Cx: pathologicfracturein 25/", fractureswill healwith I infantsof normalweight+ length

immobilization;infrequently complicatedby { osteoporosis
pseudarthrosis r/ fracturesin neonate(occurring
duringdelivery)
DDx: fibrousdysplasia,Pagetdisease OB-US:
r/ markedbowingof longbones
{ N OI U G R
OSTEOGEN
ESISIMPERFECTA
= PSATHYROSIS = FRAGILITAS OSSIUM= LOBSTEIN Osteogenesis lmperfecta Type ll
DISEASE = CONGENITAL LETHAL OI
= heterogeneousgroup of a generalizedconnectivetissue = perinatallethalform
disorderleadingto micromelicdwarfismcharacterized Transmission: sporadicnew dominantmutations/
by bone fragility,blue sclerae,and dentinogenesis autosomalrecessive
imperfecta lncidence; 1:54,000births;most frequentvariety
lncidence; overallin 1:28,500(1:20,000-1:60,000) live . bluesclerae
b i r t h s ;M : F= 1 : 1 . ligamentouslaxity+ looseskin
Histo: immaturecollagenmatrix r/ shortenedbroadcrumpledlong bones
Clinicaltypes: r/ boneangulations, bowing,demineralization
1. OSTEOGENESIS IMPERFECTA CONGENITA r/ focalizedbone thickeningfrom callusformation
= diseasemanifestat birth (occurringin utero); r/ tnin beadedribs+ fracturesresultingin bell-shaped/
autosomaldominant;corresponds to type ll; lethal narrowchest
variety ^/ tnin poorlyossifiedskull
2. OSTEOGENESIS IMPERFECTA TARDA { wormianbones (presentin most cases)
= usuallynot manifestat birth;recessive/ sporadic r/ spinalosteopenia
correspondsto type | + lV; nonlethalvariety r/ platyspondyly
. soft skull (caputmembranaceum) OB -U S :
. hyperlaxityof joints 0 A normalsonogramafter17 weeksMA excludes
. blue sclerae
. poor dentition , the diagnosis!
r/ increasedthrough-transmission of skull (extremely
. otosclerosis poor mineralization):
. thin looseskin { unusuallygood visualizationof brainsurface
r/ diffusedemineralization, deficienttrabecularstructure, { unusuallygood visualization of orbits
corticalthinning r/ increasedvisualizationof intracranialarterial
r/ defectivecorticalbone: increasein diameterof proximal pulsations
ends of humeri+ femora;slenderfragilebone;multiple { abnormalcompressibility of skullvaultwith
cystlikeareas transducer
! multiplefractures+ pseudarthrosis with bowing r/ decreasedvisualizationof skeleton
(vertebralbodies,long bones) r/ multiplefetal fractures+ deformitiesof long bones
r/ normal/ exuberantcallusformation + ribs:
r/ rib thinning/ notching r/ wrinkledappearanceof bone (= rTlorethan one
! thin calvarium fracturein singlebone)
r/ sinus + mastoidcell enlargement r/ beadedribs (callusformationaroundfractures)
{ thickenedundermineralized oticcapsule(= sles6lerosis) r/ abnormallyshortlimbs
r/ wormianbones persistinginto adulthood { smallthorax(collapseof thoraciccage)
r/ basilarimpression(= platybasia) ./ decreasedfetal movement
r/ biconcavevertebralbodies+ Schmorlnodes,increased r/ infantssmallfor gestationalage (frequent)
heightof intervertebral disk space r/ polyhydramnios + nonimmunehydrops
{ bowingdeformitiesafter child beginsto walk Prognosis; stillborn/ death shortlyafter birthdue to
Cx: (1) lmpairedhearing/ deafnessfrom otosclerosis pulmonaryhypoplasia
(20-60%) DDx: congenitalhypophosphatasia; achondrogenesis
(2) Deathfrom intracranialhemorrhage(abnormal type l; campomelicdysplasia
plateletfunction)
Dx: chorionicvilloussampling OsteogenesislmperfectaType lll
= SEVERE PROGRESSIVELY DEFORMING OI
OsteogenesislmperfectaType I Transmission: autosomalrecessive;progressively
Transmission: autosomaldominant;compatiblewith deformingdisordercompatiblewith life
life . bluishscleraeduringinfancythat turn palewith time
Age at presentation: 2-6 years . joint hyperlaxity(50%)
. blue sclerae r/ decreasedossificationof skull
. preseniledeafness r/ normalvertebrae+ pelvis
. normal/abnormaldentinogenesis { shortened+ bowed long bones
 ./ progressive of limbs+ spineinto
deformities
adulthood
^/ t rit fractures
r/ multiplefracturespresentat birth in 213of cases
r/ fracturesheal well
O B - US :
ri short + bowed long bones
r/ fractures
r/ humerusalmostnormalin shape
r/ normalthoraciccircumference
Prognosisj progressivelimb + spine deformities
duringchildhood/ adolescence
OsteogenesislmperfectaType lV
Transmission: autosomaldominant;mildestform with
best prognosis
. normalscleralcolor
. littletendencyto develophearingloss
r/ tubularbones of normallength;mildfemoralbowing
may occur
r/ osteoporosis
O B - US :
r/ bowingof long bones
OSTEOIDOSTEOMA
= benignskeletalneoplasmcomposedof osteoid+ woven
bone lessthan 1.5 cm in diameterper definition
lncidence: 12/" of benignskeletalneoplasms
Etiology: ? inflammatoryresponse
Histo: small nidusof osteoid-ladeninterconnected
trabeculaewith a backgroundof highly
vascularized fibrousconnectivetissuesurrounded
by zone of reactivebone sclerosis;osteoblastic
rimming;indistinguishable from osteoblastoma
Age: 10-20 years(51%);2nd+ 3rd decade(73%);
5-25 years (90%);rangeof 19 months-56years;
uncommon<5 and >40 yearsof age; M:F = 2:1;
uncommonin Blacks
. tenderto touch + pressure
. localpain (95-987o),weeks to years in duration,worse
at night,decreasedby activity
. salicylates give reliefin 20-30 minutesin75-90%
. prostaglandin E2 elevated100-1000 x normalwithin
nidus (probablecause of pain and vasodilatation)
Location:
(a) meta-/ diaphysisof long bones (73%):upperend of
femur(43%),hands(8/"), feet (4%);frequentin
proximaltibia+ femoralneck,fibula,humerus;no
bone exempt
(b) spine (10-14'h): predominantly in posteriorelements
(50%in pedicle+ lamina+ spinousprocess;20/"in
articularprocess)of lumbar(59%),cervical(27"/"),
thoracic(12/"),sacral(2%)segments
. painfulscoliosis,tocalI radicularpain
. gait disturbance,muscleatrophyut
(c) skull,rib, ischium,mandible,patella
Classification:
Cortical osteoid osteoma (mostcommon)
= niduswithincortex
r/ solid/ laminatedperiostealreaction
{ fusiformscleroticcorticalthickeningin shaft of long
bone
r/ radiolucentarea withincenterof osteosclerosis
Cancellous Osteoid Osteoma (intermediate frequency)
= intramedullary
0 Intraarticularlesiondifficultto identifywith delayin
diagnosisof 4 months-Syears!
Site: juxta-i intraarticularat femoralneck,vertebral
posteriorelements,smallbonesof hands
+ feet
/ scleroticcortexdistantto nidus
r/ tittteosteosclerosis
(functionaldifferenceof intraarticularperiosteum)
^/ loint spacewidened(effusion,synovitis)
Subperiosteal Osteoid Osteoma (rare)
= foufld soft-tissuemass adjacentto bone
Site: juxta-/ intraarticular
at medialaspectof
femoralneck,hands,feet (neckof talus)
r/ juxtacorticalmass excavatingthe cortex(bony
pressureatrophy)with almostno reactivesclerosis
r/ roundI ovalradiolucentnidus (75%)of <1.5 cm in size
^/ variablesurroundingsclerosis+ centralcalcification
rl painfulscoliosisconcavetowardlesion/ kyphoscoliosis
/ hyperlordosis i torticolliswith spinallocation(dueto
spasm)
r/ may show extensivesynovitis+ effusion+ premature
lossof cartilagewith intraarticular site (lymphofollicular
synovitis)
{ osteoarthritis(50%)with intraarticular site 1.5-22 years
afteronset of symptomatology
{ regionalosteoporosis(probablydue to disuse)
0 Radiographically difficultareas: vertebralcolumn,
femoralneck,small bonesof hand + feet
NUC:
^/ intenselyincreasedradiotraceruptake(increased
bloodflow + new-boneformation)
./ doubledensitysign = small area of focal activity
(nidus)superimposed on largerarea of increased
traceruptake
CT (for detection+ preciselocalizationof nidus):
{ smallwell-defined round loval nidussurroundedby
variableamountof sclerosis
^/ nidus enhanceson dynamicscan
r/ niduswith variableamountof mineralization (50%):
punctate/ amorphous/ ringlike/ dense
MR (diminished conspicuity of lesioncomparedwith CT):
i/ nidusisointense to muscleon Tl Wl
{ signalintensityincreasesto betweenthat of muscle
+ tat lremainslow on T2Wl
rl perinidalinflammationof bone marrow(63%)
r/ perinidalsoft-tissueinflammation/ edema (47%)
{ synovitis+ joint effusionwith intraarticular site
A ngi o:
./ frightyvascularized niduswith intensecircumscribed
blushappearingin early arterialphase + persisting
late into venousphase
Prognosis.' no growthprogression,infrequentregression
 Boneand Soft-Tissue
Disorders 135

Rx: (1) completesurgicalexcisionof nidus(reactive Location:

bone regressessubsequently) @ Lowerextremity(75%)
(2) percutaneousCT-guidedremoval over pressurepointsin diabeticfoot
(3) percutaneousablationwith radio-frequency @ Vertebrae(53%)
electrode/ laser/ alcohol lumbar(75%)> thoracic> cervical(= infectious
DDx: spondylitis)
(1) Corticalosteoidosteoma: Brodieabscess, @ Radialstyloid(24%)
sclerosingosteomyelitis, syphilis,bone island,stress @ Sacroiliacjoint (18%)
fracture,osteosarcoma,Ewingsarcoma,osteoblastic
metastasis, lymphoma,subperiosteal aneurysmal . leukocytosis+ fever (66%)
bone cyst, osteoblastoma(progressivegrowth)
(2) Intraarticular
osteoidosteoma: inflammatory/ septic A. ACUTENEONATALOSTEOMYELITIS
/ tuberculous/ rheumatoidarthritis,nonspecific Age: onset <30 days of age
synovitis/ Legg-Calve-Perthes disease . little/ no systemicdisturbance
r/ multicentricinvolvement morecommon;oftenjoint
involvement
OSTEOMA r/ bone scan falselynegative/ equivocalinT0/.
= benigntumorof membranousbone (hamartoma)
Age: adult life B. ACUTEOSTEOMYELITIS IN INFANCY
Associatedwith: Gardnersyndrome(multipleosteomas
Age: <18 monthsof age
+ cofonicpolyposis) Pathomechanism: spread to epiphysisbecause
Location: inner/ outertableof calvarium(usuallyfrom
transphysealvesselscross
externaltable),paranasalsinuses(frontal/ growthplate into epiphysis
ethmoidsinuses),mandible,nasalbones r/ strikingsoft-tissuecomponent
r/ well-circumscribedroundextremelydensestructureress { subperiostealabscesswith extensiveperiosteal
lesionusually<2 cm in size new bone
Cx: frequentjoint involvement
FibrousOsteoma Prognosis; rapidhealing
Probablya form of fibrousdysplasia
Age: childhood C . A C U TEOS TE OMY E LITIS IN C H ILD H OOD
./ lessdensethan osteomai radiolucent Age: 2-16 years of age
r/ expandingexternaltable withoutaffectinginternal Pathomechanism:
table transphysealvesselsclosed;metaphysealvessels
DDx: endostoma,bone island,bone infarct(locatedin adjacentto growth plate loop back toward
m edulla) metaphysislocatingthe primaryfocus of infection
intometaphysis; abscessformationin medulla
OSTEOMYELITIS with corticalspread
AcuteOsteomyelitis Location: femur,tibia
Age: most commonlyaffectschildren r/ sequestrationfrequent
Organisms: r/ periostealelevation(withdisruptionof periosteal
(a) newborns: S. aureus,group B streptococcus,
, bloodsupply)
Escherichia coli r/ smallsinglei multipleosteolyticareasin metaphysis
(b) children:S. aureus(bloodculturesin 50% r/ extensiveperiostealreactionparallelto shaft laiter
positive) 3-6 weeks);may be "lamellarnodular"
(c) adults: S. aureus(60%),entericspecies(29/"), (DDx: osteoblastoma, eosinophilicgranuloma)
Streptococcus(8%) r/ shorteningof bone with destructionof epiphyseal
(d) drug addicts: Pseudomonas (86%),Klebsiella, cartilage
Enterobacteriae; (57 days averagedelay in r/ growthstimulationby hyperemia+ premature
diagnosis) maturationof adjacentepiphysis
(e) sicklecelldisease:S. aureus,Salmonella r/ midshaftosteomyelitisless frequentsite
Cause: r/ serpiginoustractwith small scleroticrim
(1) genitourinarytract infection(72%) (PATHOGNOMONTC)
(2) lunginfection(14o/")
(3) dermal infection(14%\ directcontamination from D. ACUTEOSTEOMYELITIS IN ADULTHOOD
a soft-tissuelesionin diabeticpatient Associatedwith: soft-tissueabscess,
pathological
Pathogenesis: fracture
(a) hematogenousspread Risk factors: lV druguse,previous
trauma,
(b) directimplantation from a traumatic/ iatrogenic immunosuppressed
state,diabetes
source delicateperiostealnew bone
(c) extensionfrom adjacentsoft-tissueinfection joint involvement
common
Conventional radiographs (insensitive):
./ initiatradiographs oftennormal(notoriously poor in
early phaseof infectionfor as long as 10-14 days)
r/ localizedsoft-tissueswellingadjacentto
metaphysiswith obliterationof usualfat planes
(after3-10 days)
r/ permeativemetaphysealosteolysis(lags7-14 days
behindpathologicchanges)
{ endostealerosion
r/ intracorticalfissuring
{ involucrurTr = cloak of laminated/ spiculated
periostealreaction(developsafter 20 days)
r/ sequestrutn= detachednecroticcorticalbone
(developsafter 30 days)
r/ cloacaformation= space in which dead bone
resides
CT:
r/ marrowdensityof >+20 HU differenceto healthy
side indicatesmarrowinfection
MR:
{ bone marrowhypointenseon Tl Wl + hyperintense
on T2Wl (= water-richinflammatorytissue)
DDx: neuropathicosteoarthropathy,aseptic
arthritis,acute fracture,recentsurgery
{ focal/ linearcorticalinvolvement hyperintense on
T2WI
r/ hyperintensehalo surroundingcortexon T2Wl
= subperiostealinfection
r/ hyperintense lineon T2Wl extendingfrom boneto
skin surface+ enhancement of borders(= sinustract)
Abscesscharacteristics :
{ hyperintense enhancingrim (= hyperemiczone)
arounda centralfocusof low intensity(= necrotic
/ devitalizedtissue)on contrast-enhanced Tl WI
r/ hyperintense fluidcollectionsurroundedby
hypointensepseudocapsuleon T2Wl + contrast
enhancement of granulationtissue
{ hyperintenseadjacentsoft tissueson T2Wl
./ fat-suppressed contrast-enhanced imaging
(88/. sensitive+ 93/" specificcomparedwith
79/" + 53/"tor nonenhanced MR imaging)
NUC (accuracyapprox.90%):
(1) Ga-67 scans: 100%sensitivity;increaseduptake
1 day earlierthan for Tc-99mMDP
0 Galliumhelpfulfor chronicosteomyetitis!
(2) StaticTc-99mdiphosphonate:83olosensitivity
5-60% false-negativerate in neonates+ children
becauseof (a) maskingeffectof epiphysealplates,
(b) earlydiminishedbloodflow with infection,
(c) spectrumof uptakepatternfrom hot to cold
(3) Three-phase. skeletalscintigraphy:
927osensitivity,87% specificity
Phase 1: Radionuclideangiography= perfusion
phase of regionalbloodflow
Phase2: "bloodpool" images
Phase3: "boneuptake"
Limitations;diagnosticdifficultiesin children,in
posttraumatic/ postoperativestate,
diabeticneuropathy(poorblood
supply),neoplasia,septicarthritis,
Pagetdisease,healedosteomyelitis,
noninfectiousinflammatoryprocess
DDx: cellulitis(decreasein activityover time)
(4) WBC-scan:
(a) In-111-labeled leukocytes:best agentfor
acute infections
(b) Tc-99m labeledleukocytes:preferredover
In-111-leukocyteimagingespeciallyin
extremities
0 WBC scans have largelyreplacedgallium
imagingfor acute osteomyelitisdue to improved
photonflux + improveddosimetry(higherdose
allowedrelativeto In-111) allowingfaster
imaging+ greaterresolution
r/ "cold"area in earlyosteomyelitissubsequently
"hot"if localizedto longbones/ pelvis
becoming
(not seen in vertebralbodies)
./ localincreasein radiopharmaceutical uptake
(positivewithin2+72 hours)
Cx: (1) Soft-tissueabscess
(2) Fistulaformation
(3) Pathologicfracture
(4) Extensionintojoint
(5) Growthdisturbancedue to epiphyseal
involvement
(6) Neoplasm
(7) Amyloidosis
(8) Severedeformitywith delayedtreatment
GhronicOsteomyelitis
r/ tnicf irregularscleroticbonewith radiolucencies,
elevatedperiosteum, chronicdrainingsinus
SclerosingOsteomyelitisof Garrd
= STERILE OSTEOMYELITIS
= low-gradenonnecrotic nonpurulentinfection
Locati on: mandi bl e(mostcommonl y)
^/ focal bulge of thickenedcortex(sclerosing
periostealreaction)
DDx: osteoidosteoma,stressfracture
Chronic RecurrentMultifocal Osteomyelitis
= benignself-limited diseaseof unknownetiology
Age: children+ adolescents; M:F = 1:2
Histo: nonspecificsubacute/ chronicosteomyelitis
. pain,soft-tissue swelling,limitedmotion
Location: tibia> femur> clavicle> fibula
Site: metaphysesof long bones;often symmetric
r/ small areasof bone lysis,oftenconfluent
Brodie Abscess
= subacutepyogenicosteomyelitis indolent
(smoldering
infection)
Organism: S. aureus(mostcommon)
Histo: granulationtissue+ eburnation

Age: morecommonin children;M > F
Location: predilection for ends of tubularbones
(proximal/ distaltibialmetaphysismost
common);carpal+ tarsalbones
Site: metaphysis,rarelytraversingthe open growth
plate;epiphysis(children+ infants)
rl centralarea of lucencysurroundedby dense rim of
reactivesclerosis
rl lucenttortuouschannelextendingtowardgrowthplate
(PATHOGNOMONTC)
{ periostealnew-boneformation
^/ t adlacentsoft-tissueswelling
r/ may persistfor many months
MR:
r/ "doubleline"effect= high signalintensityof
granulation tissuesurroundedby low signal
, intensityof bone sclerosison T2Wl
r/ well-defined low-to intermediate-signal lesion
outlinedby low-signalrim on TlWl
DDx: Osteoidosteoma
EpidermoidCarcinoma
Etiology: complicationof chronicosteomyelitis
( 0. 2-1 .7 % )
Histo: squamouscell carcinoma(90%);occasionally:
basal cell carcinoma,adenocarcinoma, fibro-
sarcoma,angiosarcoma, reticulumcellsarcoma,
spindlecell sarcoma,rhabdomyosarcoma,
parostealosteosarcoma,plasmacytoma
Age: 30-80 (mean55) years; M >> F
Latentperiod: 20-30 (range ot 1.5-72) years
. historyof childhoodosteomyelitis
. exacerbationof symptomswith increasingpain,
enlargingmass
. changein character/ amountof sinusdrainage
Location: at site of chronically/ intermittently
draining
sinus;tibia(50%),femur(21%)
r/ tyticlesionsuperimposed on changesof chronic
osteomyelitis
r/ soft-tissuemass
{ pathologicfracture
Prognosis:
(1) Earlymetastasesin 14-20-40/o (within18 months)
(2) No recurrencein 80%
OSTEOPATHIA
STRIATA
= VOORHOEVE DISEASE
= autosomaldominant/ sporadicinheriteddisorder
. usuallyasymptomatic(similarto osteopoikilosis)
Location: all longbonesaffected;theonly bonesclerosis
primarilyinvolvingmetaphysis(withextension
into epi- and diaphysis)
r/ longitudinalstriationsof denie bone in metaphysis
r/ radiatingdensitiesof "sunburst"appearance'from
acetabulumintoileum
OSTEOPETROSIS
= ALBERS-SCHONBERG DISEASE = MARBLE
BONE
= tarehereditary
DISEASE disorder
Boneand Soft-Tissue
Disorders 137
Path: defectiveosteoclastfunctionwith failureof proper
reabsorption+ remodelingof primaryspongiosa;
bone sclerotic+ thick but structurallyweak + brittle
A. INFANTILEAUTOSOMALRECESSIVETYPE
= cofigeflitalmore severeform
Cause: defecton chromosome11q13
. failureto thrive
. prematuresenileappearanceof facies
. severedentalcaries
' pancytopenia(= ?nemia,leukocytopenia,
thrombocytopenia) due to severemarrowdepression
. cranialnervecompression(opticatrophy,deafness)
. hepatosplenomegaly (extramedullary hematopoiesis)
. lymphadenopathy
. subarachnoid hemorrhage(dueto
thrombocytopenia)
May be associated with:
renaltubularacidosis+ cerebralcalcification
./ dense skeleton
r/ splayedmetaphyses+ costochondral junctions
r/ fracturesfrom minortrauma (due to brittlebones)
Prognosis; stillbirth,survivalbeyondmiddlelife
uncommon(deathdue to recurrent
infection,massivehemorrhage, terminal
leukemia)
DDx: chronicrenalfailure,oxalosis,pyknodysostosis,
physiologicsclerosis
B. BENIGNADULTAUTOSOMALDOMINANTTYPE
Cause: defecton chromosome1p21
. 50o/oasymptomatic
. recurrentfractures,mildanemia
. occasionallycranialnervepalsy
r/ Erlenmeyer flaskdeformity= clublikelongbonesdue
to lack of tubulization+ flaringof ends
Phenotypel:
^/ diffuseosteosclerosis= generalizeddense
amorphousstructurelessboneswith obliterationof
normaltrabecularpattern;mandibleleast
commonly involved
,
! corticalthickeningwith medullaryencroachment
Phenotypell:
r/ bone-within-bone appearance(= endobones)
r/ "sandwich"vertebrae/ rugger-jerseyspine
r/ alternatingsclerotic+ radiolucenttransverse
metaphyseallines (phalanges,ilium)as indicators
of fluctuatingcourseof disease
r/ longitudinalmetaphysealstriations
{ obliterationof mastoidcells,paranasalsinuses,
basal foraminaby osteosclerosis
{ sclerosispredominantly involvingbase of skull;
calvariaoften spared
Prognosis.' normal life expectancy
Cx: (1) Usuallytransversefractures(commonbecause
of brittlebones)with abundantcallus+ normal
healing
(2) Crowdingof marrow(myelophthisic anemia
+ extramedullary hematopoiesis)
(3) Frequentlyterminatesin acute leukemia
138 Radiology Review Manual

Rx: bonemarrowtransplant Histo: variableamountsof neoplasticosteoid+ bone

DDx: (1)Heavymetalpoisoning + cartilage;frequentlyassociatedwith
(limited
(2)Melorheostosis to oneextremity) fibrosarcoma,malignantfibroushistiocytoma,
(3)Hypervitaminosis
D malignantperipheralnervesheathtumor
(4)Pyknodysostosis Mean age: 50 years; 94h >30 years of age; M > F
(5)Fibrous
dysplasiaof skullllace Location: lowerextremity(thighin 42-47"/"),upper
extremity (12-23/"), retroperitoneu
m
(8-170/"),buttock,back,orbit,submental,
OSTEOPOIKILOSIS axilla,abdomen,neck,kidneY,breast
= OSTEOPATHIA CONDENSANS DISSEMINATA . slowlygrowing soft{issuemass
= autosomal-dominant disorder;M > F . painful+ tender (25-50%)
Associatedwith: dermatofibrosis lenticularisdisseminata . historyof trauma(12-31"/"): in preexistingmyositis
. asymptomatic ossificans/ site of intramuscular injection
Histo: compactbone islands { oftendeep-seated+ fixed soft-tissuetumor (average
Location: in most metaphyses+ epiphyses(rarely diameterof 9 cm)
extendinginto midshaft);concentratedat ./ focal/ massivearea of mineralization (>50%)
glenoid+ acetabulum, wrist,ankle,pelvis; r/ increasedradionuclideuptakeon bone scan
rare in skull,ribs,vertebralcentra,mandible Prognosis:
r/ multipleovoid/ lenticularbone islands(2-10 mm) in (1) multiplelocal recurrences(in 80-90%) after
cancellousbone intervalof 2 monthsto 10 years
./ tongaxis of lesionsparallelto long axis of bone (2) metastasesafter intervalof 1 monthto 4 years:
r/ scintigraphicactivityrare l ungs(81-100% ),l ymphnodes(25/" ),b one,
Prognosis; not progressive,no changeaftercessationof subcutis,liver
growth (3) deathwithin2-3 years (>50%)with tumor size as
DDx: (1) Epiphysealdysplasia(metaphyses normal) majorpredictor
(2) Melorheostosis(diaphysealinvolvement)
Osteosarcoma
High-gradeIntramedullary
= CENTRAL OSTEOSARCOMA = CONVENTIONAL
OSTEOSARCOMA OSTEOSARCOMA
Mostcommonmalignantprimarybonetumorin young Histo: arisingfrom undifferentiated mesenchymal
adults+ children;2nd mostcommonprimarymalignant tissue;formingfibrous/ cartilaginous / osseous
bonetumoraftermultiplemyeloma matrix(mostlymixed)that producesosteoidi
'15%of
Prevalence: 4-5:1,000,000; all primarybone immaturebone
tumorsconfirmedat biopsy (a) osteoblastic(50-80%)
Types & Frequency: (b) chondroblastic(5-25%)
A. Conventionalosteosarcoma: ibrohistiocytic(7-25%)
(c) f ibroblastic-f
high-gradeintramedullary 75% Age: bimodaldistribution10- 25 years and
telangiectatic 4.5-11"/" >60 years;21"/"<10 years;68% <15 years;70/.
low-gradeintraosseous 4-5% between10 and 30 Years;M:F = 3:2 to 2:1;
s m allc e l l 14/" >35 years: relatedto preexistingcondition
osteosarcomatosis 34% . pai nfulsw el l i ng(1-2 months' durati on)
gnathic 6-9% . fever (frequent)
B. Surface/ juxtacorticalosteosarcoma: 4-10o/o . slightelevationof alkalinephosphatase
intracortical rare . diabetesmellitus(paraneoplastic syndrome)in 25%
parosteal 65% Location: longbones(70-80%),femur(40-45%),tibia
periosteal 25% (16-20%) ; 50-55% about knee; proximal
high-gradesurface 10% humerus(10-15% );faci albones( 8%) ;
C. Extraskeletal 4o/o cylindricalbone<30 years; flat bone (ilium)
D. Secondaryosteosarcoma 5-7.4 >50 years
Prognosis; dependenton age, sex, tumor size,site, Site: originin metaphysis(90-95%) ldiaphysis
best predictoris degreeof
classification; (2-11% )/epi physi (< s 1% );grow ththroughopen
tissuenecrosisin postresectionspecimen physiswith extensioninto epiphysis(75-88%)
followingchemotherapy(917osurvivalwith Doublingtime: 20-30 day
tumor necrosis>90"/o,14% survivalwith ri usuallylarge bone lesionof >5-6 cm when first
<90/" tumor necrosis) detected
{ cloudlikedensity(90%)/ almostnormaldensityi
Extraskeletal Osteosarcoma osteolytic(fibroblastic type)
= locatedwithinsoft tissuewithoutattachmentto bone / ./ aggressiveperiostealreaction:sunburst/ hair-on-end
periosteum i onion-peel= laminated/ Codmantriangle
lncidence: 1"/oof soft-tissuesarcomas r/ moth-eatenbone destruction+ corticaldisruption
 Boneand Soft-TissueDisorders | 39

r/ soft-tissuemass with tumor new bone (osseous/ DDx: Osteoidosteoma,

sclerosing
osteomyelitis,
cartilaginoustype) Charcotjoint
r/ transphysealspreadbeforeplateclosure(75-88%);
, physisdoes NOT act as a barrierto tumorspread High-grade Surface Osteosarcoma
{ spontaneouspneumothorax(due to subpleural Location:femur,humerus, fibula
metastases) Site: diaphysis
NUC (bonescintigraphy): { similarto periosteal
osteosarcoma
r/ intenselyincreasedactivityon bloodflow, blood { ofteninvolveentirecircumference of bone
pool, delayedimages(hypervascularity, new-bone r/ frequentinvasionof medullarycanal
formation) Prognosis; identical to conventional
intramedullary
r/ soft-tissueextensiondemonstrated,especiallywith osteosarcoma
S P E CT
r/ bone scan establisheslocalextent(extentof lntracortical Osteosarcoma
involvementeasilyoverestimateddue to intensityof Rarestformof osteosarcoma
uptake),skip lesions,metastasesto bone + soft Histo: sclerosingvariantof osteosarcoma whichmay
tissues containsmallfociof chondro-or fibrosarcoma
CT: Location:femur,tibia
r/ soft-tissueattenuation(nonmineralized portion) { tumor<4 cm in diameter
replacingfatty bone marrow geographic
r/ intracortical bonelysis
r/ low attenuation(higherwatercontentof
! tumormarginmaybe welldefinedwiththickening of
chondroblastic component/ hemorrhageI necrosis) surrounding
, cortex
! very high attenuation(mineralizedmatiix; { metastases in 29%
MR (preferredmodality):
ri tumor of intermediatesignalintensityon Tl Wl
Low-grade Intraosseous Osteosarcoma
+ highsignalintensityon T2Wl = WELL.DIFFERENTIATED / SCLEROSTNG
r/ clearlydefinesmarrowextent(beston TlWl),
OSTEOSARCOMA
vascularinvolvement,soft-tissuecomponent(best Path: penetrationamongbony trabeculae;fibrous
on T2Wl) stromasometimeslackingnuclearatypia
Evaluate for: + pleomorphism; highlyvariableamountof tumor
(1) extentof marrow+ soft-tissueinvolvement
osteoidproduction;may be misinterpreted as
(2) invasionof epiphysis fibrousdysplasia
(3) joint (19-24%) + neurovascularinvolvement Age: mostfrequently3rd decade; M:F = 1:1
(4) viabletumor+ mineralized matrixfor biopsy . protractedclinicalcoursewith nonspecificsymptoms
Metastases (in 2% at presentation): Location: aboutthe knee;femur involvedin 50%
(a) hematogenous lung metastases(15%): calcifying; Site: metaphysis;oftenwith extensioninto epiphysis
spontaneouspneumothoraxsecondaryto { may have well-definedmargins+ scleroticrim
subpleuralcavitatingnodulesrupturingintopleural ^/ diffusesclerosis
space r/ expansileremodelingof bone
(b) lymphnodes,liver,brain(maybe calcified)
{ subtlesignsof aggressiveness: bone lysis,focally
(c) skeletalmetastasesuncommon(unlikeEwing
indistinctmargin,corticaldestruction,soft-tissue
sarcoma);skip lesions= discontinuous tumorfoci mass,periostealreaction
in marrowcavityin 1-25% N.B.: the relativelybenignappearancehas resulted
in misdiagnosisas a benignentity!
Cx: (1) pathologicfracture(15-20%) Cx: transformationinto high-gradeosteosarcoma
(2) radiation-i
nducedosteosarcoma Prognosis; similarto parostealosteosarcoma;80-90%
(30 years delay)
S-yearsurvivalrate; localrecurrencein
Rx: chemotherapyfollowedby wide surgical 10% (dueto inadequateresection)
resection DDx: fibrousdysplasia,nonossifying fibroma,
Prognosis; 60-80% S-yearsurvival
chondrosarcoma, chondromyxoid fibroma
(1) amputation:20% S-yearsurvival;15% develop
skeletalmetastases;75/"dead within<2 years Osteosarcomaof Jaw
(2) multidrugchemotherapy:55/" 4-yearsurvival = GNATHIC OSTEOSARCOMA
more proximallesionscarry highermortality Averageage: 34 years (10-15 years olderthan in
(Q/" 2-yearsurvivalfor axial primary) conventionalosteosarcoma)
Predictorsof poor outcome: Histo: chondroblasticpredominance(-50%),
metastasisat presentation,soft-tissuemass >20 osteoblasticpredominance (-25%); better
cm, pathologicfracture,skip lesionsin marrow differentiated(grade2 or 3) than conventional
Predictorsof poor responseto chemotherapy: osteosarcoma(grade3 or 4)
no change/ increasein size of soft-tissuemass, . simulatingperiodontaldisease: rapidlyenlarging
increasein bone destruction mass,lump,swelling
 . paresthesia(if inferioralveolarnerve involved)
. painfull looseteeth,bleedinggum
Location: body of mandible(lytic),alveolarridgeof
maxilla(sclerotic),maxillaryantrum
r/ osteolytic/ osteoblastic/ mixedpattern
{ osteoid matrix (60-80%)
{ aggressiveperiostealreactionfor mandibularlesion
{ soft-tissuemass (100%)
{ opacificationof maxillarysinus (frequentin maxillary
lesions)
Prognosis: 40/" S-yearsurvivalrate (lower probability
of metastases,lower grade)
DDx: metastaticdisease(lung,breast,kidney),multiple
myeloma,directinvasionby contiguoustumor
from oral cavity,Ewingsarcoma,primary
lymphomaof bone, chondrosarcoma,
fibrosarcoma, acuteosteomyelitis, ameloblastoma,
Langerhanscell histiocytosis, giant cell reparative
granuloma,"browntumor"of HPT
Osteosarcomatosis
= MULTIFOCAL OSTEOSARCOMA = MULTIPLE
SCLEROTIC OSTEOSARCOMA
Frequency: 2.74.2/" of osteosarcomas
Etiology: (a) multicentrictype of osteosarcoma
(b) multiplemetastaticbone lesions
Classification(Amstutz):
Type I multiplesynchronousbone lesions
occurringwithin5 monthsof diagnosis
+ patient<18 yearsof age
Type ll multiplesynchronous bone lesions
occurringwithin5 monthsof presentation
+ patient>18 years of age
Type llla early metachronousmetastatic
osteosarcomaoccurring5 to 24 months
afterdiagnosis
Type lllb late metachronousmetastatic
osteosarcomaoccurring>24 monthsafter
diagnosis
Age: Amstutztype | = 4-18 (mean 11) years
Amstutztype ll = 19-63 (mean30) years
Site: metaphysisof long bones;may extendinto
epiphysealplate/ begin in epiphysis
! multicentric simultaneously appearinglesionswith a
dominanttumor (97%)
radiologically
{ smallerlesionsare denselyopaque(osteoblastic)
r/ lesionsbilateral+ symmetrical
{ early: bone islands
^/ late: entiremetaphysisfills with scleroticlesions
breakingthroughcortex
{ lesionsare of same size
{ lung metastases(62%)
Prognosis; uniformlypoor with mean survivalof
12 (range,6-37) months
DDx: heavy metalpoisoning,sclerosingosteitis,
progressivediaphysealdysplasia,
melorheostosis, bone infarction,
osteopoikilosis,
osteopetrosis
ParostealOsteosarcoma
Frequency: 4ohof all osteosarcomas;65% of all
j uxtacorticalosteosarcomas
Origin: outer layerof periosteum;slowlygrowing
lesionwithfulminatingcourseif tumor
reachesmedullarYcanal
Histo: low-gradelesionwith higher-graderegions
(22-64%), invasionof medullarycanal (8-59%);
fibrousstroma+ extensiveosteoidwith small
foci of cartilage
Age: peak age 38 years (rangeof 12-58 years);
50% > age 30 (for centralosteosarcoma75/" <
age 30); 1Y ' P= 2:3
Location: posterioraspectof distalfemur (50-65%),
eitherend of tibia,proximalhumerus,fibula,
rare in otherlong bones
Site: metaphysis(80-90%)
. palpablemass
r/ large lobulated
"cauliflower-like"
homogeneousossific
mass extendingaway from cortex
{ "stringsign"= initiallyfine radiolucent lineseparating
tumor mass from cortex(30-40%)
{ tumor stalk (= attachmentto cortex)grows with tumor
obliteratingthe radiolucentcleavageplane
{ corticalthickeningwithoutaggressiveperiosteal
reaction
{ tumorperipherylessdensethan center
(DDx:myositisossificanswith peripherymore dense
than center+ withoutattachmentto cortex)
r/ large soft-tissuecomponentwith osseous
+ cartilaginous elements
Prognosis; 80-90% 5- and 1O-yearsurvival rates
(bestprognosisof all osteosarcomas)
DDx: osteochondroma, myositisossificans,
juxtacorticalhematoma,extraosseous
osteosarcoma
PeriostealOsteosarcoma
Origin: deep layerof periosteum
Histo: intermediate-grade lesion;highlychondroblastic
lesionwith smallerareas of osteoidformation
Age: peak 10-20 years (rangeof 13-70 years)
Location: femurand tibia(85-95%),ulnaand
humerus(5-10%)
Site: diaphysisi metadiaphysis of longbone;limitedto
peripheryof cortexwith normalendostealmargin
+ medullarycanal (resemblesparostealsarcoma)
{ tumor 7-12 cm in length,24 cm in width,involving
50% of osseouscircumference
{ tumor base closelyattachedto cortexover entire
extentof tumor
{ tumor lies in apparentdepressionon bone surface
causingscallopedsurfaceof thickeneddiaphyseal
cortex
r/ short spiculesof new bone perpendicularto shaft
extendinginto broad-basedellipticalsoft-tissuemass
r/ solid (corticalthickening)/ aggressiveperiosteal
reaction(Codmantriangle)at upperand lower
marginsof lesion
i/ tttOcorticaldestruction/ medullarycavityinvasion
 Boneand Soft-Tissue
Disorders 1 4 1

r/ chondroblasticareasof low attenuationon CT, r/ ttuio-fluidtevets(90%)

hypointense
on T1Wl,very hyperintense on T2Wl
Prognosis; 80-90% cure rate (betterprognosisthan
centralosteosarcomawith S0% S-year
survivalbut worse than parosteal
osteosarcoma)
DDx: juxtacorticalchondrosarcoma
OXALOSIS
SecondaryOsteosarcoma
Cause: malignanttransformation withinbenignprocess
(1) Pagetdisease(67-90%)
0 0.2-75% of patientswith Pagetdiseasedevelop
osteosarcomadependenton extentof disease
(2) Sequelaeof irradiation(6-22%) 240 years ago
(malignantfibroushistiocytoma mostcommon.
fibrosarcoma3rd most common)
0 0.02-4/o of patientswith radiationtherapy
developosteosarcomarelatedto exposuredose
( us uall y> 1 ,0 0 0c G y )
(3) Osteonecrosis, fibrousdysplasia,metallicimplants,
osteogenesisimperfecta,chronic osteomyelitis,
retinoblastoma(familialbilateraltype)
Path: high-gradeanaplastictissuewith little/ no
mineralization
Age: middle-aged/ late adulthood
r/ aggressivebone destructionin area of preexisting
conditionassociatedwith largesoft-tissuemass PACH YD E RMOPE RIOSTOSIS
Prognosis: <5/" S-yearsurvival rate
Small-cellOsteosarcoma
Age: similarto conventional osteosarcoma; M:F = 1:1
Histo: smallroundbluecells(similarto Ewing
sarcoma)lackingcellularuniformityand
consistentlyproducingfine reticularosteoid
Location: distalfemur
Site: metaphysiswith frequentextensioninto
epiphysis;diaphysis(in 15%)
r/ predominantlypermeativelyticmedullarylesion
! corticalbreakthrough
./ aggressiveperiostealreaction
r/ associatedsoft-tissuemass
Prognosis; extremelypoor
TelangiectaticOsteosarcoma
= MALIGNANT BONEANEURYSM
Frequency: +11% of all osteosarcomas
Age: 3-67 (mean20) years; M:F = 3:2
Path: malignantosteoid-forming sarcomaof bone with
largeblood{illedvascularchannels PAGETDISEASE
Histo: hemorrhagic+ cystic + necroticspaces
occupying>90/" of the lesionbeforetherapy;
blood-filledcavernousvesselslinedwith
osteoclasticgiant cells
Location: about knee (62%);distalfemur (49%),
proximaltibia(14/"), proximalhumerus
(16%)
Site: metaphysis(90%);extensionintoepiphysis(87%)
r/ geographicbonedestruction with a wide zone of
transition
r/ markedaneurysmalexpansionof bone (19%)
r/ nodularcalcificfoci of osteoid(01-81%)
r/ "doughnutsign" = peripherallyincreaseduptakewith
centralphotopeniaon bone scan
DDx: aneurysmalbone cyst (no enhancingrim of
viabletumoralonglesionperiphery)
Rare inbornerror of metabolism
Etiology: excessiveamountsof oxalicacid combinewith
calciumand depositthroughoutbody (kidneys,
soft tissue,bone)
. hyperoxaluria= urinaryexcretionof oxalicacid
>50 mg/ day
. progressiverenalfailure
r/ osteoporosis= cysticrarefaction+ scleroticmarginsin
tubularboneson metaphysealside, may extend
throughoutdiaphysis
r/ erosionson concaveside of metaphysisnear epiphysis
(DDx: hyperparathyroidism)
r/ bone-within-bone appearanceof spine
r/ nephrocalcinosis (2" HPT: subperiostealresorption,
ruggerjersey spine,scleroticmetaphysealbands)
Cx: pathologicfractures
= oSTEoDERMOPATHtA HypERTROPHtCANS (TOURAtNE-
SoLENTE-GOLE) = pRtMARY HypERTROPHtC
OSTEOARTHROPATHY
Autosomaldominant
Age: 3-38 years with progressioninto late 20s / 30s;
M>>F
. largeskin folds of face + scalp
Location: epiphyses+ diametaphysealregionof tubular
bones;distalthird of bonesof legs + forearms
(early);distalphalangesrarelyinvolved
{ enlargement of paranasalsinuses
r/ irregularperiostealproliferationof phalanges+ distal
longbones(hand+ feet)beginningin epiphysealregion
, tendon/ ligamentinsertions
at
ri thick cortex,BUT NO narrowingof medulla
r/ clubbing
r/ may have acroosteolysis
Prognosisj progressionceases after several years
DDx: pulmonaryosteoarthropathy, thyroidacropachy
= OSTEITIS DEFORMANS
= multifocalchronicskeletaldiseasecharacterizedby
disorderedand exaggeratedbone remodeling
Etiology: ? chronicparamyxoviralinfection
Prevalence: 3/" of individuals>40 years; 1C /"of
persons>80 years;higherprevalencein
northernlatitudes;2nd mostcommon
disease(afterosteoporosis)affectingolder
individuals
Age: Caucasian>55 years(in 3%); >85 years(in 10%);
unusual<40 years;M:F = 2:1
Histo: increasedresorption+ increasedbone formation; ^/ Oiptoicwidening
newlyformedbone is abnormallysoftwith r/ osteoporosiscircumscripta= well-definedlysis,most
disorganizedtrabecularpattern("mosaicpattern") commonlyin calvariumanteriorly, occasionallyin
causingdeformity long bones (destructiveactivestage)
(a) ACTIVEPHASE= OSTEOLYTICPHASE { "cottonwool" appearance= mixed lytic + blastic
= intenseosteoclasticactivity= aggressivebone patternof thickenedcalvarium(latestage)
resorptionwith lyticlesions { basilarimpressionwith encroachment on foramen
Path: replacementof hematopoieticbone marrowby magnum
fibrousconnectivetissuewith numerouslarge ri deossification + sclerosisin maxilla
vascularchannels r/ sclerosisof base of skull
r/ osteoporosiscircumscriptaof skull @ Longbones(almostinvariableat end of bone;rarelyin
r/ flame-shapedradiolucency diaphysis)
( b) M I DDLE/ MIX EDPH AS E(c o mmo n ) ./ "candleflame"/ "bladeof grass"lysis= advancingtip
= decreasedosteoclasticactivity+ increased of V-shapedlyticdefect in diaphysisof long bone
osteoblasticactivity originatingin subarticularsite (CHARACTERISTIC)
{ coexistenceof lytic+ scleroticphases ./ lateralcurvatureof femur,anteriorcurvatureof tibia
(c) INACTIVE/ LATE PHASE= QUIESCENTPHASE (commonlyresultingin fracture)
= diminishedosteoblastic activitywith decreasedbone @ Ribs(14%)
turnover @ Small/ flat bones
Path: loss of excessivevascularity r/ OuOOty destruction+ periostealsuccessivelayering
r/ osteosclerosis+ corticalaccretion(eg, ivory @ Pelvis
vertebralbody) ^/ thickenedtrabeculaein sacrum,ilium;rarefaction in
centralportionof ilium
. asymptomatic(115- 314) r/ thickeningof iliopectineal line
. fatigue { acetabularprotrusion(DDx: metastaticdiseasenot
. enlargedhat size deforming)+ secondarydegenerativejoint disease
. peripheralnervecompression @ Spine(uppercervical,low dorsal,midlumbar)
. neurologicdisordersfrom compression of brainstem ./ tytlc/ coarsetrabeculationsat peripheryof bone
(basilar invagination) ^/ "picture-frame vertebra"= bone-within-bone
. hearingloss,blindness,facialpalsy(narrowingof neural appearance = enlargedsquarevertebralbody with
foramina)- rare reinforcedperipheraltrabeculae+ radiolucent inner
. pain from (a) primarydiseaseprocess- rare aspect,typicallyin lumbarsPine
(b) pathologicfracture ./ "ivoryvertebra"= blasticvertebrawith increased
(c) malignanttransformation density
(d) degenerative jointdisease/ rheumatic r/ ossificationof spinalligaments,paravertebralsoft
disorderaggravatedby skeletaldeformity tissue,disk spaces
. localhyperthermia of overlyingskin
. high-outputcongestiveheartfailurefrom markedly Bonescan (94%sensitive):
increasedperfusion(rare) r/ usuallymarkedlyincreaseduptake(symptomatic
. increasedalkalinephosphatase(increasedbone lesionsstrikinglypositivedue to increasedbloodflow
formation) + osteoblasticactivity)
. hydroxyproline increased(increasedbone resorption) r/ normalscan in some scleroticburned-outlesions
. normalserumcalcium+ phosphorus ./ marginaluptakein lyticlesions
./ enlargement+ deformityof bones
Sites: usuallypolyostotic+ asymmetric;pelvis(75%) >
lumbarspine> thoracicspine> proximalfemur> Bonemarrowscan:
calvarium> scapula > distalfemur> proximaltibia r/ sulfurcolloidbone marrowuptakeis decreased
> proximalhumerus (marrowreplacement tissue)
by cellularfibrovascular
Sensitivity: scintigraphy+radiography(60%)
scintigraphyonly (27-94%) MR:
radiographyonly (13-7 4%) lndications; imagingof complications(spinalstenosis,
basilarimpression, tumorstaging)
osseousexpansion
r/ ^/ areas of decreasedsignalintensitywithin marrowon
trabecularcoarsening
r/ TlWl + increasedintensityon T2Wl (= fibrovascular
{corticalthickening tissueresemblinggranulation tissue)
r/
cystlikeareas (fat-filledmarrowcavity/ bloodjilled r/ hypointensearea I areaof signalvoid on Tl Wl
sinusoids/ liquefactive degeneration+ necrosisof + T2Wl (corticalthickening,coarsetrabeculation)
proliferatingfibroustissue) ri reductionin size + signalintensityof medullarycavity
@ Skull (involvementin 29-65%) (replacementof high-signal-intensity fatty marrowby
r/ inner+ outertableinvolved increasedmedullarybone formation)

r/ focal areas of highersignalintensitythan fatty marrow
(= cystlikefaffilled marrowspaces)
r/ wideningof bone
Cx: (1) Associatedneoplasia(0.7-1-20%)
(a) sarcomatoustransformationinto
osteosarcoma (22-90%),f ibrosarcoma/
malignantfibroushistiocytom a (29-51"h),
chondrosarcoma (1-1 5%)
r/ osteolysisin pelvis,femur,humerus
Prognosis: <10/" S-yearsurvival
(b) multicentric giantceiltumor(S-10%)
r/ tyticexpansilelesionin skull,facialbones
(c) lymphoma,plasmacell myeloma
(2) Insufficiency fracture
(a) "bananafracture"= tiny horizontalcortical
infractionson convexsurfacesof lower
extremitylong bones (lateralbowingof
femur,anteriorbowingof tibia);
(b) compressionfracturesof vertebrae(soft
bonedespiteincreaseddensity)
(3) Neurologicentrapment
(a) basilarimpressionwith obstructive
hydrocephalus + brainstemcompression
+ syringomyelia
(b) spinalstenosiswith extraduralspinalblock
(osseousexpansion/ osteosarcoma/
vertebralretropulsionowingto compression
fracture)
(4) Early-onsetosteoarthritis
Pathogenesis: alteredbiomechanicsacross
affected articulations
Rx: calcitonin, biphosphonates,mithramycin
Detection of recurrence:
(a) in 1/3 detectedby bone scan
(b) in 1/3 detectedby biomarkers(alkaline
phosphatase, urinehydroxyproline)
(c) in 113by scan + biomarkerssimultaneously
r/ diffuse(mostcommon)/ focalincreasein traceruptake
r/ extensionof uptakebeyondboundariesof initial
lesion
DDx: osteoscleroticmetastasis,osteolyticmetastasis,
Hodgkindisease,vertebralhemangioma
PARAOSTEOARTHROPATHY
= HETEROTOPIC BONEFORMATION = ECTOPIC
osslFlcATloN= MYOStTtS OSSTFTCANS
Commoncomplication followingsurgicalmanipulation,
totalhip replacemenl(62%)and chronicimmobilization
(spinalcord injury/ neuromuscular disorders)
Mechanism; pluripotentmesenchymalcell lays down
matrixfor formationof heterotopicbone
similarto endostealbone
Causes: para-/ quadriplegia(40-50%),
myelomeningocele, poliomyelitis,
severehead
injury,cerebrovascular disease,CNS infections
(tetanus,rabies),surgery(commonlyfollowing
total hip replacement)
Boneand Soft-TissueDisorders 143
Evolution: calcificationsseen 4-10 weeks following
insult;progression for 6-14 months;
trabeculations by 2-3 months;stablelamellar
boneankylosisin 5o/"by12-18 months
r/ largestquantityof calcificationsaroundjoints,especialfy
hip,alongfascialplanes
ri disuseosteoporosisof lowerextremities
! renalcalculi(elevationof serumcalciumlevels)
Radiographic grading system (Brooker):
0 no soft-tissueossification
I separatesmallfoci of ossification
ll >1 cm gap betweenopposingbone surfacesof
heterotopicossifications
lll <1 cm gap betweenopposingbonesurfaces
lV bridgingossification
Bonescan:
r/ traceraccumulationin ectopicbone
^/ assessmentof maturityfor optimaltime of surgical
resection(indicatedby same amountof uptakeas
normalbone)
Cx: ankylosisin 5'/"
Rx: 1000-2000rad within4 days followingsurgical
removal
PHENYLKETONURIA
Highincidenceof x-raychangesin phenylalanine-
restrictedinfants:
r/ metaphysealcuppingof long bones (30-50%),
especiallywrist
! calcificspiculesextendingverticallyfrom metaphysis
into epiphysealcartilage(DDxto rickets)
r/ scleroticmetaphysealmargins
r/ osteoporosis
ri delayedskeletalmaturation
DDx: homocystinuria
P H OS P H OR U S P OIS ON IN G
Etiology: (1) ingestionof metallicphosphorus(yellow
phosphorus)
(2) treatmentof rachitisor TB with phosphorized
cod liveroil
Location: longtubularbones,ilium
r/ multipletransverselines(intermittent treatmentwith
, phosphorus)
! linesdisappearafter some years
PIERREROBINSYNDROME
May be associatedwith: CHD, defects of eye and ear,
hydrocephalus,microcephaly
. glossoptosis
r/ micrognathia = hypoplastic
recedingmandible
r/ arched+ cleft palate
ri rib pseudarthrosis
Cx: airwayobstruction(relativelylargetongue),
aspiration
 VILLONODULAR
PIGMENTED SYNOVITIS GiantCellTumor
Tenosynovial
= PVNS= benigntumorof histiocytic origin/ inflammatory = localizedform of PVNSonly involvingthe tendon
processwith extensiveintraarticular highlyvascular sheath
synovialproliferation
Cause: frequentlyhistoryof antecedenttrauma P OLA N D S Y N D R OME
Path: many villousI frondlikesynovialproliferations May be associatedwith: aplasia of mamilla/ breast
Histo: (1) hyperplasiaof undifferentiated connective Autosomalrecessive
tissuewith multinucleated largecellsingesting r/ unilateralabsenceof the sternocostalhead of the
hemosiderin / lipoid(foam/ giantcells) pectoralismajormuscle
(2) villonodular appearanceof synovialmembrane { ipsilateralsyndactyly+ brachydactyly
+ fibrosis r/ rib anomalies
(3) pressureerosion/ invasionof adjoiningbone
Age: mainly3rd-4th decade(range12-68 years); POLIOMYELITIS
50'/"<40 years; M:F=1:1 r/ osteoporosis
. hemorrhagic "chocolate" / serosanguinous i r/ soft-tissuecalcification/ ossification
xanthochromic jointeffusionwithouttrauma r/ intervertebraldisk calcification
. insidiousonsetof swelling,painof longduration r/ rib erosioncommonlyon superiormarginof 3rd + 4th rib
. joint stiffnesswith decreasedrangeof motion,joint (secondaryto pressurefrom scapula)
locking r/ "bamboo"spine (resemblingankylosingspondylitis)
r/ sacroiliacjoint narrowing
Location: knee (80%)> hip > ankle> shoulder> elbow
> tarsal+ carpaljoints;predominantly POPLITEAL CYST
monoarticular (DDx: degenerative arthritis) = BAKERCYST
r/ soft-tissue swellingaroundjoint (effusion+ synovial = synovialcyst in the posterioraspectof knee joint
proliferation): communicating with posteriorjointcapsule
rl lointeffusionin knee,but not relevantin otherjoints Prevalence: 19% in generalorthopedicpatients,6l%in
./ dense soft tissues(hemosiderindeposits) patientswith rheumatoidarthritis
r/ subchondralpressureerosion(56%)at marginsof joint Pathophysiology:
on both sidesof joint formedby escapeof synovialeffusioninto one of the
r/ multiplesites of irregularcystlikeradiolucentdefects bursae;fluidtrappedby one-wayvalvularmechanism
due to invasionof bone (a) Bunsentypevalve = exP"ndingcyst compressesthe
r/ trlOcalcifications, osteoporosis,joint space narrowing communicating channel
(untillate) (b) balltype valve= ballcomposedof fibrin+ cellular
CT: debrisplugsthe communication channel
r/ smallradiographically invisibleerosions Etiology: (1) Arthritis(rheumatoidarthritismost common)
r/ juxtaarticularsoft-tissuemass with high attenuation (2) Internalderangement(meniscal/ anterior
MR: cruciateligamenttears)
r/ massesof synovialtissuein a joint with effusion (3) Pigmentedvillonodular synovitis
r/ scaltoping/ truncationof prefemoratfat pad . pseudothrombophlebitis syndrome(= pain + swellingin
r/ predominantly low signalintensityon all sequences calf)
(dueto presenceof iron)is CHARACTERISTIC . cellulitis(afterleakage/ rupture)
r/ often heterogeneouslow + high signalintensityon Location: (a) gastrocnemio-semimembranous bursa
T 2W I : = posteriorto gastrocnemiusmuscleat
r/ low-signalintensityon T2Wl in peripheryof lesions levelof medialcondyle
(magneticsusceptibilityeffectof hemosiderin) (b) supralateralbursa= betweenlateralhead
DDx: hemosiderindepositsin otherdiseases of gastrocnemius muscle+ distalend of
(eg, rheumatoidarthritis) bicepsmusclesuperiorto lateralcondyle
i/ rrign-signal intensityareas (due to fat, effusion, (uncommon)
edema,inflammation) (c) poplitealbursa= beneathlateralmeniscus
Rx: synovectomy(50% recurrencerate),arthrodesis, + anteriorto poplitealmuscle(uncommon)
arthroplasty,radiation r/ communication with bursa(documented on arthrogram)
DDx: (1) Degenerative/ traumaticarthritis { hypointense collectionon Tl Wl + hyperintenseon T2Wl
(2) Synovialsarcoma(solitarycalcifiedmass Types:
outsidejoint) 1. Intactcyst
(3) Sclerosinghemangioma ri smoothcontour
(4) Benignxanthoma 2. Dissectedcyst
(5) Xanthogranuloma r/ smoothcontourextendingalongfascialplanes
(usuallybetweengastrocnemius + soleus)
IntraarticularLocalizedNodularSynovitis 3. Rupturedcyst
= sYnovialliningwithouthemosiderin r/ leakageinto calf tissues
 Boneand Soft-TissueDisorders 145

DDx of other synovial cysts about the knee: r/ neurotrophic jointlesions(feet)

(1) Meniscalcyst (at lateral/ medialside of jointline; d widespreadosteomyelitis+ septicarthritis(hands,
associatedwith horizontalcleavagetears) feet,limbs)
(2) Tibiofibular
cyst (at proximaltibiofibular
joint,which @ Soft tissue
communicates with kneejointin 10%) { soft-tissueatrophyof extremities
(3) Cruciatecyst (surroundinganterior/ posterior { soft-tissuecalcificationsaroundbony prominences
cruciateligamentsfollowingligamentous injury) (ankle,wrist,elbow,knee)
r/ peripheralvascularcalcifications= premature
PROGERIA atherosclerosis
= HUTCHINSON-GILFORD SYNDROME Prognosis; most patientsdie in their 30s / 40s from
= autosomalrecessiveinheritance;
mostcommonlyin (myocardial
complicationsof arteriosclerosis
populations
with consanguineousmarriages(Japanese, infarction,stroke)or neoplasm(sarcoma,
Jewish) meningioma, thyroidcarcinoma)
Age: shortlyafteradolescence;M:F = 1:1 DDx: Cockaynesyndrome(mentalretardation,retinal
atrophy,deafness,familyhistory)
Characteristichabitus+ stature:
. symmetricretardationof growth
. absentadolescentgrowthspurt PSEUDOACHONDROPLASIA
. dwarf with short stature+ lightbody weight . normalface + head
. spindlyextremitieswith stockytrunk d timOshortening
. beak-shapednose + shalloworbits r/ irregularepiphyses
Prematuresenescence: { scoliosis
. birdlikeappearance r/ coxa vara
. grayingof hair + prematurebaldness { markedshorteningof bones in hands+ feet
. hyperpigmentation
. voice alteration
. diffusearteriosclerosis PSEUDOFRACTURES
. bilateralcataracts = LOOSER LINES= LOOSER ZONES= OSTEOID SEAMS
. osteoporosis = MILKMAN SYNDROME
Scleroderma-like skin changes: = insufficiencystressfractures+ nonunion(incomplete
. atrophicskin + muscles healingdue to mineraldeficiency)
. circumscribedhyperkeratosis Path: area of unmineralized woven bone occurringat
' telangiectasia sites of mechanicalstress/ nutrientvesselentry
. tight skin Associated with:
. cutaneousulcerations (1) Osteomalacia / rickets
. localizedsoft-tissuecalcifications (2) Pagetdisease("bananafracture")
Endocrineabnormalities : (3) Osteogenesisimperfectatarda
. diabetes (4) Fibrousdysplasia
. hypogonadism (5) Organicrenaldiseasein 1"/"
(6) Renaltubulardysfunction
r/ generafizedosteoporosis (7) Congenitalhypophosphatasia
@ Skull (8) Congenitalhyperphosphatasia ("juvenilePaget
./ tnin cranialvault disease")
r/ delayedsuturalclosure+ wormianbones (9) VitaminD malabsorption / deficiency
./ hypoplastic facialbones(maxilla+ mandible) (10) Neurofibromatosis
@ Chest mnemonic.' "POOF"
r/ narrowthorax+ slenderribs Pagetdisease
r/ progressiveresorptionwith fibrousreplacementof Osteomalacia
, outerportionsof thinnedclavicles(HALLMARK) Osteogenesisimperfecta
r/ coronaryartery+ heartvalve calcifications with Fibrousdysplasia
cardiacenlargement Commonlocations:
@ Extremities & joints scapulae(axillarymargin,lateral+ superiormargin),
i/ short+ slenderlongbones medialfemoralneck+ shaft,pubic+ ischialrami,ribs,
r/ coxa valga lessertrochanter,ischialtuberosity,proximal1/3 of ulna,
r/ valgusof humeralhead distal1/3 of radius,phalanges,metatarsals,
r/ acroosteolysisof terminalphalanges(occasionally) metacarpals,clavicle
r/ flexion+ extensiondeformities of toes (halluxvalgus, r/ typicallybilateral+ symmetricat rightanglesto bone
pes planus) margin
r/ excessivedegenerativejoint diseaseof major r/ paralleledby marginalsclerosisin laterstages
+ per ipher a l j o i n ts ! healingfracturewith littleor no callusresponse
 @ Eyes
PHypoPT PPHypoPT . diminishedvisualacuitydue to alterationof
r/ calcificationof basal ganglia 44% 8% chorioretinalstructure
40% . angioidstreaks= reddishbrownserratedlines
ri soft-tissuecalcifications 55%
r/ metacarpalshortening extendingfrom opticdisk in a spoke-wheel fashion
75'/" 90%
(4+5alwaysinvolved) @ Arteries
. claudication + decreasedpulses
r/ metatarsalshortening 70/" 99%
Histo: tissuedegenerationof internalelasticlamina
(3 + 4 involved)
+ medi al thi ckeni ng
^/ lobulatedappearanceof arteries(similarto
fibromuscu lar hyperplasia)
^/ Z-3-mm stripeof lucencyat right angleto cortex rl aneurysmformation
(= osteoidseamsformedwithinstress-induced r/ vesselcalcificationat early age
(PATHOGNOMONIC)
infractions + nonunion Cx: Gl tract hemorrhage
(= incompletehealingdue to mineraldeficiency)

DISM
PSEUDOHYPOPARATHYROI PSORIATIC ARTHRITIS
= PHypoPT= corg€nitalX-linkeddominantabnormality Uncommondiseaseinvolvingsynovium+ ligamentous
with renal + skeletalresistanceto PTH due to (1) end- attachments with propensityfor sacroiliitis/ spondylitis
organresistance, (2) presenceof antienzymes, classifiedas seronegativespondyloarthropathy 6/c
(3) defectivehormone lncidence: 20o/"of patientswith psoriasis(peripheral
May be associatedwith: hyperparathyroidismdue to in 29/", peripheral
arthritisin 5o/",sacroiliitis
hypocalcemia;F > M in 10%)
arthritis+ sacroiliitis
. short obese stature.roundface
Path: synovialinflammation(lessprominentthan in
. mentalretardation rheumatoidarthritis)with earlyfibrosisof
. corneal+ lenticularopacity proliferative synovium;bony proliferation at joint
. abnormaldentition(hypoplasia, delayederuption, margins/ tendoninsertions/ subperiosteum
excessivecaries) Types:
. hypocalcemia+ hyperphosphatemia (resistantto PTH (1) true psoriaticarthritis(31%)
injection) (2) psoriaticarthritisresemblingrheumatoidarthritis
. normallevelsof PTH (38%)
r/ brachydactylyin bones in which epiphysisappears (3) concomitantrheumatoid+ psoriaticarthritis(31%)
latest(metacarpal,metatarsalbones l, lV, V) (75%) . skin rashprecedes/ developssimultaneously with onset
r/ acceleratedepiphysealmaturationresultingin dwarfism of arthritisin 85%
+ coxa vara lvalga 0 Arthritisantedatesdermatological changesby an
ri multiplediaphysealexostoses(occasionally) intervalof up to 20 yearsl
r/ calcificationof basalganglia+ dentate nucleus ' pitting,discoloration, hyperkeratosis, subungual
r/ calcification/ ossificationof skin + subcutaneoustissue separation,ridgingof nails(in 80%)
' positiveHLA-827 in 80%
. negativerheumatoidfactor
PSEUDOPSEU DOHYPOPARATHYROI DISM
= PPHypoPT= differentexpressionof samefamiliai Location: widelyvariabledistribution+ asymmetrywith
disturbancewith identicalclinical+ radiographic
features involvement of lower+ upperextremities;
as pseudohypoparathyroidism but normocalcemic distinctivepattern:terminalinterphalangeal
Cause: end-organresistanceto PTH joints,ray distribution,unilateralpolyarticular
. short stature,roundfacies asymmetricaldistribution
. NO bloodchemicalchanges(normalcalcium ^i lttOi minimaljuxtaarticularosteoporosis (earlystage);
+ phosphorus) frequentosteoporosis(laterstages)
. normalresponseto injectionof PTH ^/ marginalerosions
r/ brachydactyly ri periostealreactionfrequent
osseousexcrescences
r/ intraarticular
@ Hand + foot
ELASTICU
PSEUDOXANTHOMA M Targetarea: DlP, PlP, MCP
= recessivehereditarysystemicdisordercharacterizedby ri "sausagedigit"= soft-tissue swellingof entiredigit
degenerationof elastictissue r/ destructionof distalinterphalangealjoints (erosive
@ Skin polyarthritis)
+ osseousresorption
. redundantskin folds,particularly
in flexorregions ./ bonyankylosis(10%)
. yellowishxanthomatous papules r/ "pencil-in-cup"deformity= erosionswith ill-defined
r/ large amorphouscalcificdepositsin soft tissueabout margins+ adjacentproliferationof periostealnew
the joints bone (CHARACTERISTIC)
 Boneand Soft-Tissue
Disorders 147

r/ ivoryphalanx= sclerosisof terminalphalanx(ZB%) RADIATION

r/ destructionof interphalangeal joint of 1st toe with
exuberantperiostealreaction+ bony proliferationat
distalphalangealbase (PATHOGNOMONTC)
r/ poorlydefineddiffusenew bone formationat
attachmentof Achillestendon+ plantaraponeurosis
r/ erosionsat superior/ posteriormarginof calcaneus
(20%)
r/ acroosteolysis(occasionally)
@ Axial skeleton
r/ "floating"osteophyte= largebulkyverticallyoriented
paravertebralsoft-tissueossification(AP view):
r/ ilt-definedexcrescencesweepingacrossthe
diskovertebral junctionfrom midportionof one
vertebrato the next
Location: lowercervical,thoracic,upperlumbar
spine;asymmetric/ unilateral
{ squaringof vertebraein lumbarregion
d sacroiliitis(40%)= (mostcommonly)bilateral
+ symmetricsacroiliacjointwidening,increased
density,fusion
i/ apophysealjoint narrowing+ sclerosis
r/ atlantoaxial subluxation + odontoidabnormalities
DDx: (1) Reitersyndrome(affectsonly lowerextremity)
(2) Ankylosingspondylitis
(3) Rheumatoidarthritis(bilaterallysymmetricwell-
definederosions,juxtaarticularosteoporosis)
PYKNODYSOSTOSIS
= MAROTEAUX-LAMY DISEASE
= autosomalrecessiveinheriteddisease;probablyvariant
of cleidocranialdysostosis
Cause: mutationin cathepsin-Kgene
Age: children;M:F = 2'1
. dwarfism
. mentalretardation(10%)
. widenedhands+ feet
. dystrophicnails
. yellowishdiscoloration of teeth
. characteristic facies(beakednose, recedingjaw)
r/ brachycephaly+ platybasia
{ wide cranialsutures,wormianbones
./ trrict<
skull base
^/ hypoplasiaof mandible+ obtusemandibularangle
r/ hypoplasia+ nonpneumatization of paranasalsinuses
./ nonsegmentation of C112and L5/S1
i/ generalizedincreaseddensityof long boneswith
thickenedcortices(resemblingosteopetrosisbut with
preservationof medullarycanal)
r/ claviculardysplasia
ri hypoplastictaperedterminaltufts (=s6yoosteolysis)
r/ multiplespontaneousfractures
DDx: (1) Osteopetrosis (no dwarfism,no mandibular/
skullabnormality, no phalangealhypoplasra,no
transversemetaphysealbands,anemia,
Erlenmeyer f laskdeformity;"bone-within-bone"
appearance)
(2) Cleidocranial dysostosis(no densebones/
terminalphalangealhypoplasia,short stature)
INJURYTO BONE
Pathogenesis: vascularcompromisewith obliterative
endarteritis+ periarteritisfollowedby
damageto osteoblasts+ osteoclastswith
decreasedmatrixproduction(growing
bone + periostealnew bone most
sensitive)
Dose effects:
dependon age of patient,absorbeddose, size of
radiationfield,beam energy,fractionation
>300 rad: microscopic changes
>400 rad: growthretardation
<600-12O0rad: histological recoveryretained
> 1200rad; pronouncedcellulardamageto
chondrocytes;bone marrowatrophy
+ cartilagedegenerationafter
>6 months;vascularfibrosis
A . B ON EGR OW THD IS TU R B A N C E
@ Appendicular skeleton
ri lointspacewidening(dueto cartilage
hypertrophy)after8-10 months
i/ growthplatewideningin 1-2 months,often
returningto normalby 6 months
./ permanentalterationin bone lengthI size
(dueto prematurefusionof physis)
{ metaphysealbowing
r/ scleroticmetaphysealbands
d metaphysealirregularity+ frayingresembling
rickets
r/ longitudinalstriations
r/ overtubulation(= abnormalnarrowingof the
diaphysealshaft)
Cx: slippageof femoral/ humeralepiphysis
+ ischemicnecrosis(afterdoses of >25 Gy)
@ Axialskeleton(doseof <15 Gy)
{ "bone-within-bone" appearanceafter g-12 months
d irreversiblescalloping+ irregularityof vertebral
endplatewith decreasedheightof vertebra
(= failureof verticalgrowth)
ri scoliosisconcaveto the side of irradiation(due to
asymmetricvertebralgrowth+ muscularfibrosis)
r/ hypoplasiaof ilium+ ribs
^/ acetabulardysplasia,coxa vara lvalga
B. RADIATIONOSTEITIS= OSTEORADIONECROSIS
= R A D IA TION N E C R OS IS
= bone mottlingdue to osteopenia+ coarse
trabeculation+ focallyincreasedbone density(due
to attemptsof osseousrepairwith depositionof new
boneon ischemictrabeculae)
Dose: >6,000cGy in adults;>2,000cGy in children
Timeof onset: 1-3 years followingradiationtherapy
Location: mandible,ribs,clavicle,humerus,spine,
pelvis,femur
r/ focal lyticarea with abnormalbone matrix:
ri radiolucencyconfinedto radiationfield with narrow
zone of transition
r/ periostitis
^/ increasedfragilitywith sclerosis(= pathologic
insufficiency fx)
r/ + corticalthinningfrom chronicinfection
 MR: r/ generalizedosteopenia= ground-glassappearancewith
r/ increasedintensityof spinalbone marrowon Tl Wl endosteal+ intracorticalexcavation:
+ T2Wl correspondingto radiationport (fatty { subperiostealbone resorption
infiltration) r/ lysisof juxtaarticular+ subchondralbone
NUC: ./ preservationof joint space (DDx: rheumatoid/ septic
./ bone scan with decreaseduptakein radiationfield arthritis)
Cx: increasedsusceptibility of irradiatedbone to NUC (3-phasebone scan):
infection ri increasedflow + increasedblood pool + increasein
DDx: recurrentmalignancy,radiation-induced periarticularuptakeon delayedimagesin affected
sarcoma(soft-tissuemass),infection part (60%)
C. B E NI G NNE O PL A SM r/ diminisnedflow / delayeduptake(15-20%)
Most likelyin patients<2 years of age at treatment; Rx: sympatheticblock,a- / B-adrenergic blockingagents,
with doses of 1600-6425rads nonsteroidal antiinflammatorydrugs,radiation
Latentperiod: 1.5-5-14 years therapy,hypnosis,acupuncture,acupressure,
1. Osteochondroma = exostosis(exclusively in transcutaneousnervestimulation,physiotherapy,
childrenunder2years of age duringtreatment) calcitonin, earlymobilization
corticosteroids,
2. Osteoblastoma
D. MALIGNANTNEOPLASM REITER
SYNDROME
= RADIATION-INDUCED SARCOMA = triadof (1) arthritis(2) uveitis(3) urethritis;
98% male
Latencyperiod: 3-55 (averageof 11-14) years Types:
Minimum dose: 1,660-3,000 rad (1) endemic(venereal)
Criteria:(a) malignancyoccurringwithin irradiatedfield (2) epidemic(postdysenteric)
(b) latency period of >5 years . historyof sexualexposure/ diarrhea3-11 days before
(c) histologicproof of sarcoma onset of urethritis
(d) microscopicevidenceof alteredhistology . mucocutaneous lesions(keratosisblennorrhagia,
of the originallesion balanitiscircinatasicca)
Histo: 1. Osteosarcoma (90%)= 4-11% of all . uveitis,conjunctivitis
osteogenicsarcomas . posi ti veH LA -827i n76/"
2. Fibrosarcoma> chondrosarcoma> Location: asymmetricmono-/ pauciarticular
malignantf ibroushistiocytoma r/ polyarthritis
. pain,soft-tissuemass, rapidprogressionof lesion { articularsoft-tissueswelling+ joint space narrowingin
50% (particularlyknees,ankles,feet)
{ widening+ inflammation of Achilles+ patellatendons
DYSTROPHY
REFLEXSYMPATHETIC ./ "fluffy"periostealreaction(DISTINCTIVE)at metatarsal
= CAUSALGIA = SHOULDER-HAND SYNDROME necks,proximalphalanges,calcanealspur,tibia+ fibula
= POSTTRAUMATIC OSTEOPOROSIS = SUDECK at ankleand knee
DYSTROPHY r/ juxtaarticularosteoporosis(rarein acute stage)
= serious+ potentially disablingconditionwith poorly
understoodorigin+ cause C H R ON ICC H A N GE S
Etiology: . recurrentjoint attacksin a few cases
(1) Traumdin >50% (fracture,frostbite;may be trivial) r/ calcanealspur at insertionof plantarfascia+ Achilles
0 Affects0.01%of all traumapatients tendon
(2) ldiopathicin 27"/"(immobilization,infection) r/ periarticulardeossification
(3) Myocardialischemiain 6% r/ marginalerosions,loss of joint space
(4) CNS disordersin 6h ^/ bilateralsacroiliacchangesindistinguishable from
0 Affects 12-21% of patientswith hemiplegia ankylosing/ psoriaticspondylitis(in 10-40%)
(5) Diskogenicdiseasein 5% r/ paravertebralossification= isolated"floating
. burningpain,tenderness,allodynia,hyperpathia osteophyte" usuallyin thoracolumbar area
. soft-tissueswelling+ pittingedemaout of proportionto
degreeof injury Cx: aorticincompetence;
gastriculcer+ hemorrhage;
. dystrophicskin + nail changes heartblock;amyloidosis
. sudomotorchanges: hyperhidrosis+ hypertrichosis
. vasomotorinstability(Raynaudphenomenon,local RELAPSING POLYCHONDRITIS
vasoconstriction / -dilatation) = raf€ disordercharacterizedby generalizedrecurring
. end-stage(after6-12 months): contractures,atrophyof inflammation + destruction of cartilagein joints,ears,
skin + soft tissues nose, larynx,airways
Location: hands and feet distalto injury Etiology: acquiredmetabolicdisorder(? abnormalacid
{ periarticularsofttissue swelling mucopolysaccharide metabolism)/
{ patchyosteopenia(50%)as early as 2-3 weeks after hypersensitivity/ autoimmuneprocess
onset of symptoms(DDx: disuseosteopenia) (antibodiesdirectedagainsttype ll collagen)
 Disarders 149
Boneand Soft-Tissue

Histo: loss of cytoplasmin chondrocytes;plasmacell Pathogenesis:

+ lymphocyteinfiltration (1) Renalinsufficiency causesa decreasein vitaminD
Age: 40-60 years (no age predilection) conversionintothe active1,25(OH)rD. (doneby
. nasalchondritis= saddle-nosedeformity 25-OH-D-1-ahydroxylase,which is exclusiveto
. bilateralauricularchondritis= swollen+ tenderears, renaltissuemitochondria); vitaminD deficiency
cauliflower ears slows intestinalcalciumabsorption;vitaminD
. hearingloss (obstructionof externalauditorymeatus/ resistancepredominates and calcium levels stay
audiovestibu lar damage) low (Ca x P productremainsalmostnormal
. ocularinflammation secondaryto hyperphosphatemia); low calcium
. cough,hoarseness,dyspnea(collapseof trachea) levelslead to OSTEOMALACIA;additionalfactors
. nonerosiveseronegativeinflammatorypolyarthritis responsiblefor osteomalaciaare inhibitorsto
= arthralgia calcificationproducedin the uremicstate,aluminum
@ Head toxicity,dysfunctionof hepaticenzymesystem
r/ calcificationof pinna of ear (2) Renalinsufficiency with diminishedfiltrationresults
@ Chest in phosphateretention;maintenanceof Ca x P
r/ manubriosternal / costochondralarthropathy(30%) productlowersserumcalciumdirectly,which in turn
@ Respiratorytract (in up to 70%) increasesPTH production(2'HPT); 2'HPT
{ ectasia+ collapsibility(cartilaginous destruction)of predominafes associatedwith mild vitamin D
tracheaand mainstembronchiwith focalthickening resistanceand leadsto an increasein Ca x P
(mucosaledema)+ luminalnarrowing(fibrosis) productwith SOFT-TISSUE CALCIFICATION in
r/ bronchiectasis kidney,lung,joints,bursae,bloodvessels,heartas
{ generalized+ localizedemphysema well as OSTEITISFIBROSA
@ Cardiovascular(in 15-46%) (3) Mixtureof (a) and (b): increasedserum phosphate
d aorticaneurysm(4-10%), mostlyin ascendingaofia, inhibitsvitaminD activationvia feedbackregulation
may be multiple/ dissecting . phosphateretention
r/ aortic/ mitralvalve insufficiency(8%) . hypocalcemia
{ systemicvasculitis(13%)
@ Bone A. OSTEOPENIA(in 0-25-83%)
r/ periarticularosteoporosis = diminutionin numberof trabeculae+ thickeningof
{ erosivechangesin carpalbones resembling stressedtrabeculae= increasedtrabecularpattern
rheumatoidarthritis Cause: combinedeffectof
r/ soft-tissueswellingaroundjoints + styloidprocessof (1) Osteomalacia (reducedbone mineralization due
ulna to acquiredinsensitivityto vitaminD / antivitamin
{ erosiveirregularities in sacroiliacjoints D factor)
r/ Oist<space erosion+ increaseddensityof articular (2) Osteitisfibrosacystica(boneresorption)
plates (3) Osteoporosis(decreasein bone quantity)
Rx: corticosteroids, immunosuppression Contributingfactors:
Prognosis: 74/o S-yearsurvivalrale;55"h 1O-year chronicmetabolicacidosis,poor nutritionalstatus,
survivalrate; mediansurvivaltime of pre- and posttransplantationazolemia,use of
ications
compr ntfor
accou steroids,hyperparathyroidism, low vitaminD levels
]lJ"i.l?;3rftr Cx: fracturepredisposition(lessenedstructural
strength)with minortrauma/ spontaneously;
RENALOSTEODYSTROPHY fractureprevalenceincreaseswith durationof
= colstellationof musculoskeletal abnormalitiesthat occur hemodialysis+ remainsunchangedafter renal
with chronicrenalfailureas a combinationof transplantation
(a) osteomalacia(adults)/ rickets(children) Site: vertebralbody (3-25%), pubic ramus,rib
(b) 2" HPT with osteitiscysticafibrosa+ soft-tissue (tzs%)
calcifications ^/ Milkmanfracturei Looserzones(in 1%)
(c) osteosclerosis r/ metaphysealfractures
(d) soft-tissue+ vascularcalcifications Prognosis.' osteopeniamay remain unchanged/
Classification: worsenafter renaltransplantation
+ during
(1) Glomerularform = ?cQuiredrenaldisease:chronic hemodialysis
glomerulonephritis(common)
(2) Tubularform = cortgerlitalrenalosteodystrophy: B. RICKETS(children)
1. VitaminD-resistantrickets= hypophosphatemic Cause: in CRF normalvesselsfail to developin an
rickets orderlyway along cartilagecolumnsin zone
2. Fanconisyndrome= impairedresorptionof of provisionalcalcification;
this resultsin
glucose,phosphate,amino acids,bicarbonate, disorganizedproliferationof the zone of
uric acid,sodium,water maturing+ hypertrophying cartilageand
3. Renaltubularacidosis disturbedendochondralcalcification
 Radiology Review Manual

Location: most apparentin areas of rapidgrowth . inflammatoryresponsein surrounding

such as kneejoints tenosynovial tissue
^i diffusebone demineralization r/ discretecloudlikedense areas
r/ wideningof growthplate r/ ttuid-ttuidlevelin tumoralcalcinosis
r/ irregularzone of provisionalcalcification Prognosis; often regresseswith treatment
r/ metaphysealcupping+ fraying - visceral(79%): heart,lung,stomach,kidney
r/ bowingof long bones,scoliosis ./ ttuttyamorphous"tumoral"calcification
^/ diffuseconcaveimpressionat multiplevertebralend
plates,basilarinvagination Rx: 1. Decreaseof phosphorusabsorptionin bowel (in
r/ slippedepiphysis(10%): capitalfemoral,proximal hyperphosphatemia)
humerus,distalfemur,distalradius,headsof 2. VitaminD" administration (if vitaminD
metacarpals+ metatarsals resistance"predominates)
r/ generaldelay in bone age 3. Parathyroidectomy for 3' HPT (= autonomous
H P T)
C. SECONDARYHPT (in 6-66%)
Cause: inabilityof kidneysto adequatelyexcrete Dialysis-related Disorders
phosphateleadsto hyperplasiaof parathyroid 1. Osteomyelitis
chief cells (2' HPT);excessPTH affectsthe 2. Osteonecrosis
developmentof osteoclasts,osteoblasts, 3. Crystaldeposition
osteocytes 4. Destructivespondyloarthropathy
. hyperphosphatemia 5. Amyloidosis
. hypocalcemia 6. Dialysiscysts
. increasedPTH levels
rl subperiosteal,cortical,subchondral,trabecular, Congenital Renal Osteodystrophy
endosteal,subligamentousbone resorption Vitamin D-Resistant Rickets
r/ osteoclastoma= browntumor = osteitisfibrosa = PHOSPHATE DIABETES = PRIMARY
cysticain 1.5-1.7% (dueto PTH-stimulated HYPOPHOSPHATEMIA = FAMILIAL
osteoclasticactivity;morecommonin 1" HPT) HYPOPHOSPHATEM IC RICKETS
r/ periostealnew-boneformation(8-25%) = t?te X-linkeddominantdisorderof renaltubular
{ chondrocalcinosis (morecommonin 1" HPT) reabsorptioncharacterizedby
(a) impairedresorptionof phosphatein proximal
D. OSTEOSCLEROSTS (9-34%) renaltubule(dueto defectin renalbrush-border
0 One of the mostcommonradiologicmanifestations; membrane)
mostcommonlywith chronicglomerulonephritis; may (b) inappropriatelylow synthesisof
be the sole manifestationof renalosteodystrophy 1,2S-dihydroxyvitamin in renal
D. [1,25(OH)rD.l
r/ diffusechalkydensity: thoracolumbarspine in 60% tubulesresultingin decreasedintestinal
(ruggerjerseyspine);also in pelvis,ribs,longbones, resorptionof calcium+ phosphate
facialbones,baseof skull(children) Age: <1 year
. hypophosphatemia + hyperphosphaturia
Prognosisi may increase/ regressafter renal
. elevatedserum alkalinephosphatase
transplantation
. normalplasma+ urinecalcium
. normal/ low serum1,2S(OH),D,
E. SOFT.TISSUECALCIFICATIONS
(a) metastatic r/ classicrachiticchanges
secondaryto hyperphosphatemia (solubilityproduct r/ skeletaldeformity,particularlybowed legs
for calcium+ phosphate[Caz.x POo-tlexceeds ./ retardedbone age; dwarfism if untreated
60-75 mgidLin extracellular fluid),hypercalcemia, r/ osteosclerosis / bonethickening(from
alkalosiswith precipitationof calciumsalts overabundanceof incompletelycalcifiedmatrix)
(b) dystrophic Rx: phosphateinfusion+ largedoses of vitaminD
secondaryto localtissueinjury DDx: vitamin-D-deficient and -dependent rickets
Location: (absenceof muscleweakness+ seizures
- arterial(27-83%): + tetany)
in medial+ intimalelastictissue
Location: dorsalpedisa., forearm,hand,wrist, Fanconi Syndrome
leg Triad of
r/ pipestemappearancewithoutprominent (1) Hyperphosphaturia
luminalinvolvement (2) Aminoaciduria
- periarticular(0-52%): (3) Renalglucosuria(normalbloodglucose)
multifocal,frequentlysymmetric,may extendinto Etiology: renal tubulardefect
adjacentjoint r/ rickets,osteomalacia,osteitisfibrosa,
. chalkyfluid/ pastelikematerial osteosclerosis

Prognosis; functionalrenal impairmentlikelywhen
bonechangesoccur
Rx: largedosesof vitaminD + alkalinization
LATESIGNS:
Renal Tubular Acidosis
. systemicacidosis,bone lesions
r/ rickets,osteomatacia,pseudofractures,
nephrocalcinosis, osteitisfibrosa(rare)
(a) LightwoodsyndrorT'le = salt-losingnephritis
(transientself-limited
form) @ Hand & wrist
. NO nephrocalcinosis
(b) Butler-Albrightsyndrome(severeform)
. nephrocalcinosis
RHEUMATOID
ARTHRITIS
= generalizedconnectivetissuedisease
= type lll hypersensitivity
= delayedhypersensitivity
= immunecomplexdisease(= formationof antigen-
antibodycomplexeswith complementfixation)
Prevalence: 1'/. of population
Cause: geneticpredisposition; ? reactionto antigen
from Epstein-Barr virus/ certainstrainsof E. coli @ Cervicalspine
Age: highestincidence40-50 years; M:F = 1:3 if
<40 years; M:F = 1:1 if >40 years
Pathosen*"'iij;y",',",,'#il:ii:l*?ffJ3l,i5,jilreads
to impairednutritionwith chondronecrosis,
jointnarrowing,subluxation, and ankylosis
Diagnosticcriteria of American RheumatismAssociation
(at least4 criteriashouldbe present):
(1) morningstiffnessfor >1 hour
(2) swellingof >3 joints,particularlyof wrist,
metatarsophalangeal or proximalinterphalangeal
jointsfor >6 weeks
(3) symmetricswelling.
(4) typicalradiographicchanges
(5) rheumatoidnodules
(6) positiverheumatoidfactor
. morningstiffness @ Ribs
. fatigue,weightloss,anemia
. carpaltunnelsyndrome
. rheumatoidfactor(positivein 85-94%) = lgM-antibody @ Shoulder
= agglutinationof sensitizedsheep RBCsclosely
correlatingwith diseaseseverity;
falsepositive:normal(5/"), asbestosworkerswith
fibrosingalveolitis(25%),viralI bacterial/ parasitic
infection,otherinflammatory diseases
. antinuclear antibodies(positivein many)
. LE cells (positivein some)
. positivelatexflocculationtest
. hormonalinfluence:
(a) decreasein activityduringpregnancy
(b) men with RA have low testosteronelevels
Location: symmetricinvolvementof diarthrodialjoints
E A RLYS I G NS : @ Hip
r/ fusiformperiarticularsoft-tissueswelling(resultof
effusion)
Boneand Soft-TissueDisorders 1 5 1
.i regionalosteoporosis(disuse+ localhyperthermia)
r/ widenedjoint space
r/ giant synovialcyst
./ diffuseloss of interosseousspace
r/ markeddestruction+ fracturesof joint space
ri extensivedestructionof bone ends
^i bonyfusion
Target areas:
all five MCP, PlP, interphalangealjoint of thumb,all
wristcompartments (especially radiocarpal,inferior
radioulnar, pisiform-triquetral joints);earliest
c h a n g e s e e ni n 2 + 3 M C P , 3P I P
r/ marginal+ centralbone erosions(lesscommonin
largejoints);site of first erosionis classicallybase of
proximalphalanxof 4th finger
^/ changesin the ulnarstyloid+ distalradioulnar joint
(earlysign)
r/ flexion+ extensioncontractureswith ulnar
subluxation + dislocation
./ erosionsof odontoidprocess(1) betweenanterior
arch of atlas + dens, (2) betweentransverseligament
of atlas + dens, (3) at tip of odontoidprocess
{ anterioratlantoaxial subluxation(in >6%): >2.5mm
in adults,>4.5 mm in childrenduringneckflexion
r/ "cranialsettling"= odontoidprocessprojectsinto
skull base due to significantdiseaseof atlanto-
occipitaland atlantoaxialjoints
r/ lateralhead tilt = lateralsubluxation= asymmetry
betweenodontoidprocess+ lateralmassesof atlas
r/ "stepladderappearance"of cervicalspine due to
subaxialsubluxations + absenceof osteophytosis:
r/ destruction+ narrowingof disk spaces
r/ irregularvertebralbody outlines
r/ erosion+ destruction of zygapophysealjoints
r/ resorptionof spinousprocesses
r/ osteoporosis
r/ erosionof superiormarginsof posteriorportionsof
ribs 3-5
r/ symmetriclossof glenohumeraljoint space:
ri marginalerosionsat superolateralaspectof
humeralhead
^/ osteoporosis
r/ elevationof humeralheads = narrowingof
acromiohumeral distance(2'to tear latrophyof
rotatorcuff)
r/ widenedacromioclavicular joint:
^/ erosionsat acromial+ clavicularend
ri taperedmarginsof distalclavicle
r/ scallopederosionon undersurfaceof distalclavicle
oppositethe coracoidprocess(attachmentof
coracoclavicular ligament)
ri often appearsnormalduringearly diseaseprocess
./ pannusformation(MR imaging)
 r/ symmetricloss of joint spacewith axial migrationof 3. Aortitis(5%) of ascendingaorta+ aorticvalve
femoralhead insufficiency
r/ marginal+ centralerosions,cysts,localizedsclerosis (f) Rheumatoidvasculitis
r/ decompression of jointeffusioninto iliopsoasbursa = leukocytoclastic lesionof smallvenulesmimicking
throughweak anteriorcapsuledisplacingmuscle periarteritisnodosa
+ vasculature . polyneuropathy, gangrene,
cutaneousulceration,
r/ ruptureof glutealtendon polymyopathy,myocardial/ visceralinfarction
{ protrusioacetabuli(fromosteoporosis) (g) Neurologicsequelae
@ Knee 1. Distalneuropathy(relatedto vasculitis)
Location:medial+ lateralfemorotibialcompartments; 2. Nerveentrapment(atlantoaxial subluxation,
bilateralsymmetric carpaltunnelsyndrome,Bakercyst)
r/ diffuseloss of joint space (h) Lymphadenopathy (up to 25%)
{ osteoporosis r/ splenomegaly (1-5%)
r/ superficial+ deep marginal+ centralerosions
r/ subchondral sclerosis(especially in tibia)
{ synovialherniation+ cysts (eg, poplitealcyst) CysticRheumatoid Arthritis
r/ varus/ valgusangulation(dueto crumblingof = intraosseous
cysticlesions feature
asdominant
osteoporoticbone of tibia + ligamentous increased
Pathogenesis; pressure
insynovial
space
abnormalities) from joint effusiondecompresses
@ Foot throughmicrofractures of weakened
Target areas: marginalcortexinto subarticularbone
medialaspectof MT heads(2,3,4),medial+ lateral 0 Increasein size + extentof cysts correlateswith
aspectof MTS(earliestsign);interphalangealjoints increasedlevelof activity+ absenceof synovialcysts
of foot (esp.greattoe); bilateral+ symmetric Age: as above;M:F = 1:1
r/ calcanealplantarspur . seronegativein 50%
r/ juxtaarticularsubcorticallytic lesionswith well-defined
DDx: SLE, psoriaticarthritis,seronegative scleroticmargins
spondylarthropathies r/ relativelack of cartilageloss,osteoporosis,joint
disruption
EXTRA-ARTICU LAR MANIFESTATTONS (76/.) DDx: gout (presenceof uratecrystals),pigmented
(a) Felty syndrome (.1%) villonodular synovitis(monoarticular)
= rheumatoidarthritis(presentfor >10 years)
+ splenomegaly+ neutropenia JuvenileRheumatoidArthritis
Age: 40-70 years; F > M; rare in Blacks = rheumatoidarthritisin patients<16 years of age;
. rapidweightloss M< F
. therapyrefractoryleg ulcers Classification:
. brown pigmentationover exposedsurfacesof (1) Juvenile-onset adulttype (10%)
extremities . lgM RA factorpositive;age 8-9; poor prognosis
(b) Sjogrensyndrome(15%) ^/ erosivechanges;perfuseperiostealreaction;
= keratoconjunctivitis + xerostomia+ rheumatoid hip diseasewith protrusio
arthritis (2) Polyarthritisof the ankylosingspondylitictype
(c) Pulmonarymanifestations . iridocyclitis;boysage 9-11 years
{ pleuraleffusion,mostlyunilateral,withoutchange r/ peripheralarthritis;fusionof greatertrochanter;
for months,usuallynot associatedwith completefusionof both hips; heel spur
parenchymaldisease (3) Still disease
r/ interstitialfibrosiswith lower lobe predominance (a) systemic
Prevalence: 2-9"/" of rheumatoidpatients (b) polyarticular
{ rheumatoidnodules(30%): well-circumscribed, (c) pauciarticular + iridocyclitis(30%)
peripheral,with frequentcavitation . fever,rash,lymphadenopathy,
r/ Caplansyndrome(= hyperimmunereactivityto hepatosplenomegaly; pericarditis,dwarfism
silicainhalationwith rapidlydevelopingmultiple . fatal kidneydiseasein ZQ'h
pulmonarynodules) Age: 2-4 and 8-1 1 years of age; M < F
ri pulmonaryhypertensionsecondaryto arteritis Location: involvementof carpometacarpal joints
(d) Subcutaneous nodules ("squashed carpi"in adulthood),hind
(in 5-35% with activearthritis)over extensor foot, hip (40-50%)
surfacesof forearm+ other pressurepoints(eg, { periostealreactionof phalanges;broadeningof
olecranon)withoutcalcifications (DDx to gout) bones;acceleratedbone maturation+ early
(e) Cardiovascularinvolvement fusion(stuntingof groMh)
1. Pericarditis (20-50%) . morningstiffness,arthralgia
2. Myocarditis(arrhythmia,heartblock) . subcutaneousnodules(10%)
 Boneand Soft-TissueDisorders 153

. skin rash (50%) ./ increasein distancebetweenend of shaft and

. fever,lymphadenopathy epiphysealcenter
Location: earlyinvolvement of largejoints(hips, ./ cupping+ frayingof metaphysiswith threadlikeshadows
knees,ankles,wrists,elbows);laterof into epiphysealcartilage(weight-bearing bones)
hands + feet r/ corticalspurs projectingat right anglesto metaphysis
{ radiologicsignssimilarto rheumatoidarthritis(except r/ coarsetrabeculation(NO ground-glasspatternas in
for involvementof largejointsfirst,late onset of bony scurvy)
changes,more ankylosis,wide metaphyses) r/ periostealreactionmay be present
r/ periarticularsoft-tissueswelling ^/ deformitiescommon(bowingof soft diaphysis,molding
r/ thinningof jointcartilage of epiphysis,fractures)
r/ largecystlikelesionsremovedfrom articularsurface r/ bowingof long bones
(invasionof boneby inflammatory pannus);rarein d frontalbossing
children
{ articularerosionsat ligamentous + tendinousinsertion mnemonic; "RICKETS"
sites Reactionof periosteummay occur
{ ioint destructionmay resembleneuropathicjoints Indistinctcortex
ri juxtaafticularosteoporosis Coarsetrabeculation
r/ "balloonepiphyses"+ "gracilebones"(epiphyseal Knees + wrists+ anklesmainlyaffected
overgrowth+ earlyfusionwith bone shortening Epiphysealplateswidened+ irregular
secondaryto hyperemia) Tremendousmetaphysis(fraying,splaying,cupping)
@ Hand i foot Spur (metaphyseal)
r/ "rectangular" phalanges(periostitis+ cortical
thickening) Causesof Rickets
{ ankylosisin carpaljoints I. ABNORMALITYIN VITAMIND METABOLISM
@ Axial skeleton Associatedwith reactivehyperparathyroidism
Location: predominantlyuppercervicalspine A . V ITA MIND D E FIC IE N C Y
ri ankylosisof cervicalspine (apophysealjoints), 1. Dietarylackof vitaminD
sacroiliacjoints = famineosteomalacia
r/ decreasedsize of vertebralbodies+ atrophic 2. Lackof sunshineexposure
intervertebraldisks 3. Malabsorption of vitaminD
{ subfuxation (66%)
of atlantoaxialjoint = gastroenterogenous ricketsdue to
r/ thoracicspinalcompressionfractures (1) Pancreatitis+ biliarytract disease
@ Chest (2) Steatorrhea,celiacdisease,
{ ribbonribs postgastrectomy
r/ pleural+ pericardialeffusions (3) Inflammatorybowel disease
pulmonarylesions(simulating
! interstitial B . D E FE C TIV EC ON V E R S ION OF V ITA M I ND TO
scleroderma,dermatomyositis) 25-OH -C H OLE C A LC IFE RIN OLLIV E R
r/ solitarypulmonarynodules,may cavitate 1. Liverdisease
Prosnosist -, coverv (30%); secondarv 2. Anticonvulsant drug therapy(= inductionof
:;" iJi.,:re hepaticenzymesthat acceleratedegradation
of biologicallyactivevitaminD metabolites)
RICKETS C. DEFECTIVECONVERSIONOF 25-OH-D3TO
= osteomalaciaduringenchondralbone growth 1,25-OH -DIN 3 K ID N E Y
Age: 4-18 months 1. Chronicrenalfailure= rerralosteodystrophy
Histo: zone of preparatorycalcificationdoes not form, 2. VitaminD{ependent rickets= autosomal
heap up of maturingcartilagecells; failureof recessiveenzymedefectof 1-OHase
osteoidmineralizationalso in shaftsso that II. ABNOHMALITYIN PHOSPHATEMETABOLISM
osteoidproductionelevatesperiosteum not associatedwith hyperparathyroidismsecondary
. irritability,bone pain,tenderness to normalserumcalcium
. craniotabes A . P H OS P H A TED E FIC IE N C Y
. rachiticrosary 1. Intestinalmalabsorption of phosphates
. bowed legs 2. Ingestionof aluminumsalts[Al(OH)r]forming
. delayeddentition insolublecomplexeswith phosphate
. swellingof wrists+ ankles 3. Low phosphatefeedingin prematurely born
infants
Location: metaphysesof long bones subjectedto stress 4. Severemalabsorptionstate
are particularlyinvolved(wrists,ankles,knees) 5. Parenteralhyperalimentation
{ poorlymineralizedepiphysealcenterswith delayed B . D IS OR D E R SOF R E N A LTU B U LA R
appearance REABSORPTION OF PHOSPHATE
^/ irregularwidenedepiphysealplates(increasedosteoid) 1. Renaltubularacidosis(renallossof alkali)
 Radiology Review Manual

2. deToni-Debr6-Fanconi syndrome= Age: lifelongprocess;1st stage <25 years;2nd stage

hypophosphatemia, glucosuria,aminoaciduria 25-40 years; complete rotatorcuff tear >40 years
3. VitaminD-resistantrickets Pathophysiology:
4. Cystinosis movementof humerusimpingesrotatorcuff tendons
5. Tyrosinosis againstcoracoacromial arch resultingin microtrauma,
6. Lowe syndrome whichcausesinflammation of subacromial bursa
7. lfosfamidenephrotoxicity(for the treatmentof (= fibrousthickeningof subacromialbursa)/ rotator
rhabdomyosarcoma, Wilmstumor) cuff (criticalzone of rotatorcuff = supraspinatus
C. HYPOPHOSPHATEMIA WITH tendon2 cm from its attachmentto humerus)
NO NE N D O C R INTEU MOR S 0 lmpingementpathophysiology may be secondaryto
= Oncogenicrickets= elaborationof humeral primaryinstability!
substancewhichinhibitstubularreabsorption lmpingement anatomy:
of phosphates(paraneoplastic phenomenon) narrowingof subacromialspace secondaryto
1. Nonossifying fibroma (1) acquireddegenerative subacromial osteophyteI
2. Sclerosinghemangioma enthesophytefrom
3. Hemangiopericytoma (a) bony outgrowthalong coracoacromialligament
4. Ossifyingmesenchymal tumor (b) acromioclavicular joint osteoarthritis
D. HYPOPHOSPHATASIA (2) congenitalsubacromialhookof anterioracromion
III. CALCIUM DEFICIENCY (= subacromial spur)
1. Dietaryrickets= milk-freediet (extremelyrare) 0 lmpingement syndromemay existwithout
2. Malabsorption impingement anatomyl
3. Consumption of substancesformingchelates . painfularc of motion
with calcium ^/ subacromialenthesophyte
rl afterationin acromialshape + orientation
Classification of Rickets ri thickeningof coracoacromialligament
l. PrimaryvitaminD-deficiencyrickets Cx: (1) partial/ completetear (may be precipitatedby
ll. Gastrointestinal malabsorption acutetraumaticevent on preexisting
A. Partialgastrectomy degenerative changes)
B. Smallintestinaldisease;gluten-sensitive (2) cutftendinitis/ degenerativetendinosis
enteropathy/ regionalenteritis Dx: Lidocaineimpingement test (= subacromial
C. Hepatobiliarydisease:chronicbiliary lidocaineinjectionrelievespain)
obstruction / biliarycirrhosis Rx: acromioplasty(= removalof a portionof the
D. Pancreatic disease:chronicpancreatitis acromion),removalof subacromial osteophytes,
lll. Primaryhypophosphatemia; vitaminD-deficiency removal/ lysis/ d6bridement of coracoacromial
rickets ligament,resectionof distalclavicle,removalof
lV. Renaldisease acromioclavicular joint osteophytes
A. Chronicrenalfailure
B. Renaltubulardisorders:renaltubularacidosis
C. Multiplerenaldefects Glenohumeral Instability
V. Hypophosphatasia+pseudohypophosphatasia Glenohumeral stabilityis dependenton a functional
Vl. Fibrogenesisimperfectaosseum anatomicunit (= anteriorcapsularmechanism)formed
Vll. Axial osteomalacia by: glenoidlabrum,jointcapsule,superior+ middle
V lll. M is c ellan e o u s + anteroinferior+ posteroinferior
glenohumeral
Hypoparathyroidism, hyperparathyroidism, ligaments,coracohumeral ligament,subscapularis
thyrotoxicosis, osteoporosis,Pagetdisease, tendon,rotatorcuff
fluorideingestion,ureterosigmoidostomy, Age: <35 years
neurofibromatosis,osteopetrosis, Frequency; acute, recurrent,fixed
macroglobulinemia, malignancy Cause: traumatic,microtraumatic, atraumatic
Direction: anterior> multidirectional > inferior>
ROTATORCUFFLESIONS posterior
SubacromialPainSyndrome Type of lesions:
(1) lmpingement syndrome labralabnormalities (compression,avulsion,
(2) Rotatorcuff tendinitis shearing),capsular/ ligamentous tear/ avulsion
(3) Degeneration withoutimpingement Associated lesions:
(4) Shoulderinstability
with secondaryimpingement Hill-Sachs fracture,troughlinefracture,glenoid
(5) lnstability
withoutimpingement fracture,labralcyst
0 Normalcleftsmay existwithinlabrum!
lmpingementSyndrome Falsepositivefor labralseparation:
= lateralshoulderpain with abduction;commoncause (1) Articularcartilagedeepto labrum
of rotatorcuff tears; NOT radiographicdiagnosis (2) Glenohumeral ligamentspassingadjacentto labrum

RotatorCuff Tear
Etiology: (1) Attritionalchange+ tendondegeneration
due to aging,repeatedmicrotrauma as a
resultof impingement betweenhumeral
head + coracoacromialarch, overuseof
shoulderfrom professionali athletic
activities
(2) Acutetrauma(rare)
Age: most commonly>50 years
Location: "criticalzone"of supraspinatustendon 1 cm
medialto tendonattachment(areaof
relative hypovascularity)
Classification:
EXTENTOF TEAR
(a) incompleterupturs= parti?l tear involveseither
bursalor synovialsurfaceor remains
intratendinous
(b) completerupture= full-thickness tear bridging
subacromial bursaand glenohumeral joint
- pure transversetear
- pure vertical/ longitudinaltear
- tear with retractionof tendonedges
- globaltear = massive lear lavulsionof cuff
involvingmorethan one of the tendons
TOPOGRAPHYOF TEAR
(a) extentin frontalplane: nondisplaced, minimally
displaced,dramaticallydisplaced
(b) extentin anteriordirection:supraspinatus
tendon + coracohumeralligament
+ subscapularistendon
(c) extentin posteriordirection:supraspinatus
tendon + infraspinatus+ teres minortendon
Arthrography(71-100%sensitive,71-100% specificfor
combinedfull + partialthicknesstears):
{ opacificationof subacromial-subdeltoid bursa
MR (41-100%sensitiveand 79-100%specificfor
combinedfull + partialthicknesstears):
{ discontinuityof cuff with retractionof
musculotendinous junction
r/ focal / generalizedintense/ markedlyincreased
signalintensityon T2Wl 1=ffuidwithincuff defect)
in <50%
^/ ttuiOwithinsubacromial-subdeltoid bursa(MOST
S E NS T T TV E)
r/ low i moderatesignalintensityon T2Wl (= severoly
degeneratedtendon,intactbursal/ synovial
surface,granulation / scartissuefillingthe regionof
torn tendinousfibers)
r/ cuff defect with contour irregularity
r/ abruptchangein the signalcharacterat boundary
of the lesion
^/ supraspinatus muscleatrophy(MOSTSPECIFIC)
Pitfalls:
r/ hyperintensefocus in distalsupraspinatustendon
{ gray signalisointenseto muscleon all pulse
sequences
(a) partialvolumeaveragingwith superior
+ lateralinfraspinatus tendon
Boneand Soft-Tissue
Disorders 155
(b) vascular"watershed"area
(c) magicangle effect= orientationof collagen
fibersat 55' relativeto main magneticfield
{ hyperintensefocuswithin rotatorcuff on T2Wl
(a) partialvolumeaveragingwith fluid in biceps
tendonsheathi subscapularis bursa
(b) partialvolumeaveragingwith fat of peribursal
fat
(c) motionartifacts: respiration,vascular
pulsation,patientmovement
./ tatty atrophyof muscle
(a) impingement of axillary/ suprascapular nn.
= quadrilateralspace syndrome
US (scansin hyperextendedposition,75-100o/o
sensitive,43-97% specific,65-95% negativepredictive
value, 55-75% positivepredictivevalue):
r/ nonvisualization of rotatorcuff (largetear),most
reliablesign:
r/ dettoidmuscledirectlyon top of humeralhead
r/ defectfilledwith hypoechoicthickenedbursa
+ fat (withhypervascularity on color Doppler)
betweendeltoidand humeralhead
r/ focal nonvisualization of rotatorcuff, reliablesign:
r/ "nakedtuberositysign" = retractedtendon leaves
a bare area of bone
r/ toldingof bursal+ peribursalfat tissueinto focal
defect
r/ discontinuity of rotatorcufffilledwithjointfluidi
hypoechoicreactivetissue
! abrupt+ sharplydemarcatedfocalthinning
r/ small comma-shapedarea of hyperechogenicity
(smalltear filledwith granulation tissue/
hypertrophiedsynovium)
Falsenegative: longitudinaltear, partialtear
Falsepositive: intraarticular bicepstendon,
soft-tissuecalcification,small scar /
fibroustissue
Bursitis
Subacromial-Subdeltoid
commonfindingin rotatorcuff tears
r/ peribursalfat totally/ partiallyobliterated+ replaced
by low-signal-intensity tissueon all pulsesequences
ri tluiOaccumulation withinbursa
Tendinopathy
Supraspinatus / Tendinosis
Cause: impingement,acute/ chronicstress
Histo: mucinous+ myxoiddegeneration
r/ increasein signalintensityin tendonon proton-
densityimageswithoutdisruptionof tendon
{ tendinousenlargement+ inhomogeneous signal
pattern
RUBELLA
= GERMAN MEASLES
lncidence.' endemic rate of 0.1"/"
Age: infants(in uterotransmission)
. neonataldwarfism(intrauterinegrowthretardation)
. failureto thrive
. retinopathy,cataracts,deafness
. mentaldeficiencywith encephalitis+ microcephaly
., thrombocytopenic purpura,petechiae,anemia @ Cardiovascularabnormalities
{ "celery-stalk" sign (50%)= metaphysealirregular { atrialseptaldefect
margins+ coarsenedtrabeculaeextendinglongitudinally { patentductusarteriosus
from epiphysis;distalend of femur> proximalend of { coarctationof aorta
tibia,humerus r/ valvularaorticstenosis
r/ no periostealreaction { pulmonicstenosis
r/ hepatosplenomegaly + adenopathy OB .U S :
ri pneumonitis r/ decreasedhead circumference
@ Cardiovascular: { smallfor gestationalage
{ congenitalheartdisease(PDA) Cx in infancy: obstipation,feedingproblems,recurrent
{ peripheralpulmonaryarterystenosis upper respiratoryinfection
{ necrosisof myocardium
@ CNS SAPHOSYNDROME
r/ punctate/ nodularcatcifications = Synovitis, pustulosis,
Acne,Palmoplantar Hyperostosis,
r/ porencephallccysts Osteitis
r/ occasionallymicrocephaly = PUSTULOTIC ARTHROSTEITIS
Prognosis; osseousmanifestationsdisappearin = STERNOCLAVICULAR HYPEROSTOSIS
1-3 months = ?ssoci€rtion betweenrheumatologicand cutaneous
DDx: (1) CMV lesions(= seronegativespondyloarthropathy)
(2) Congenitalsyphilis(diaphysitis
+ epiphysitis) 0 Delayof severalyears can separateosseousfrom
(3) Toxoplasmosis cutaneouslesions!
Etiology: ? variant of psoriasis
Age: youngto middle-aged adults;M:F = 1:1
RUBINSTEIN-TAYBI
SYNDROME . palmoplantarpustulosis(52%)= chroniceruptionof
= BROAD THUMBSYNDROME yellowishintradermal sterilepustuleson palms+ soles
= fole sporadicsyndromewithoutknownchromosomal/ . severeacne (15%)= acne fulminans,acne conglobata
bioc hem ic alm a rk e rsM:F
; = 1 :1 . pain,soft-tissue swelling,limitationof motionat skeletal
. small stature site of involvement
. mental,motor,languageretardation @ Sternoclavicular joint (70-90%)
@ Characteristicfacies Site: insertionof costoclavicular ligament,clavicles,
. beaked/ straightnose + low nasalseptum manubri um sterni
. antimongoloidslant of palpebralfissures { osteolysisat beginningof disease
. epicanthicfolds { hyperostosis+ osteosclerosis
. broadfleshynasalbridge { arthritis+ ankylosisof sternoclavicular joint
. high-archedpalate @ Axial skeleton (33%)
. dentalabnormalities { osteosclerosisof one / more vertebralbodies
@ Ophthalmologic findings .l Oist<space narrowing+ endplateerosion
. strabismus,ptosis, refractiveerrors { paravertebralossifications(mimickingmarginal/
@ Cutaneousfindings nonmarginal syndesmophytes / massivebridging)
. keloids,hirsutism,simiancrease r/ unilateralsacroiliitis+ associatedosteosclerosisof
. flat capillaryhemangiomaon forehead/ neck adjacentiliac bone
@ Musculoskeletal findings @ Appendicularskeleton(30%)
! short broad "spatulate"terminalphalangesof thumb Locati on: di stal femur, proxi mal ti bi a,
fi bu la,
and great toe + angulationdeformity(MOST humerus,radi us,ul na
CONSISTENT+ CHARACTERISTIC FINDING) Site: metaphysis
{ radialangulationof distalphalanx(50%)causedby r/ osteosclerosis i osteolysis+ periostealnew bone
trapezoid/ delta shape of proximalphalanx formationwith aggressiveappearance
./ tufted"mushroom-shaped" fingers+ webbing @ Joints
rl thin tubularbones of hand + feet Location: knee,hip,ankle,DIP of hand
r/ club feet r/ synovialinflammationwith juxtaarticular
r/ skeletalmaturationretardation osteoporosis(early)
r/ dysplasticribs r/ loint narrowing,marginalerosion,hyperostosis,
ri spina bifidaocculta enthesopathy(later)
{ scoliosis Prognosis: chroniccoursewith unpredictable
r/ tlat acetabularangle + flaringof ilia exacerbations+ remissions
@ Genitourinarytract anomalies Rx: nonsteroidal antiinflammatorydrugs,
^l bilateralrenalduplication corticosteroids, analgesics,cyclosporine
r/ renal agenesis DDx: infectiousosteomyelitis/ spondylitis,osteosarcoma,
{ bifid ureter Ewingsarcoma,metastasis,Pagetdisease,aseptic
^/ incomplete/ delayeddescentof testes necrosisof clavicle
 Boneand Soft-TissueDisorders 157

SARCOIDOSIS @ Teeth
Osseousinvolvementin 6-15-20% { cyst formation+ hemorrhagein enamel
. unimpairedjoint function,jointsare rarelyinvolved DDx: TORCH infections,leukemia,neuroblastoma
Location: small bonesof hands+ feet (middle+ distal
phalanges)
r/ reticulated"lacelike"trabecularpatternin metaphyseal Adult Scurvy
ends of middle+ distalphalanges,metacarpals, lncidence: rare
metatarsals r/ hemarthroses+ bleedingat synchondroses
r/ well-definedcystlikelesionsof varyingsize
^/ neuropathy-like destructionof terminalphalanges SEPTIC ARTHRITIS
(DDx: scleroderma) N.B.: MEDICALEMERGENCY= immediatetreatment
r/ phalangealendostealsclerosis+ periostealnew bone necessaryto preventpermanentjoint damage!
(infrequent) Organism:
r/ vertebralinvolvementunusual: destructivelesionswith most often due to Staphylococcus aureus;gonorrhea
scleroticmargin (multifocalsepticarthritisin young adults;
r/ diffusesclerosisof multiplevertebralbodies indistinguishable from tuberculousarthritis,but more
{ paravertebralsoft-tissuemass (DDx:indistinguishable rapid);brucellararthritis(indistinguishable from
from tuberculosis) tuberculosis, slow infection); Salmonella(commonly
r/ osteolyticchangesin skull associatedwith sicklecell disease/ Gaucherdisease)
(a) neonates,infants: group D streptococcus
(b) <4 years of age: Hemophilusinfluenzae,Strepto-
SCURVY coccuspyogenes,S. aureus,
= BARLOWDISEASE = HYPOVITAMNOSIS C (c) >4 years of age: S. aureus
= vitaminC (= ascorbicacid) deficiencywith defective (d) t10 yearsof age: S. aureus,Neisseriagonorrheae
osteogenesisfrom abnormalosteoblastfunction (e) adults: S. aureus
Pathophysiology:
InfantileScurvy (a) lyticenzymesin purulentarticularfluid destroy
Age: 6-9 months(maternalvitaminC protectsfor first articular+ epiphysealcartilages
6 months) (b) pus increasesintraarticular pressureand
Predisposed feedingwith pasteurized/ boiledmilk compromises bloodflowto epiphysisresultingin
Pathogenesis: abnormalcollagenformation osteonecrosis
. irritability Mode of infection:
. tenderness+ weaknessof lower limbs (1) hematogenous spread(mostcommon;lV drug
. scorbuticrosaryof ribs abuser,immunocompromised patient):
. bleedingof gums (teething) (a) hip, knee in children
. legs drawn up + widelyspread= pseudoparalysis (b) the five "S" joints in adults:
Location: distalfemur(esp.medialside),proximal Shoulder(AC joint)
and distaltibia+ fibula,distalradius+ ulna, Sternoclavicular joint
proximalhumerus,sternalend of ribs Spine
r/ "ground-glass" osteoporosis(CHARACTERISTIC) Sl joint
r/ corticalthinning Symphysispubis
r/ soft-tissueedema (rare) (2) contiguousspreadfrom focusof osteomyelitis
(a) metaphysealfocus: hip, elbow,shoulder,ankle
@ Metaphysis
i/ white line of Frdnkel= metaphysealzone of have intraarticularmetaphyses(children)
preparatorycalcification(DDx:lead / phosphorus (b) epiphysealfocus: growthplate perforatedby
poisoning,bismuthtreatment,healingrickets) vascularchannelsin children<1 year of age
r/ Trummerfeldzone = radiolucentzone on shaft (3) directinoculationof joint from penetratingwound:
side of Frdnkel'swhite line (siteof subepiphyseal smalljointsof hand + feet;unusualorganismslike
infraction) Pseudomonas + Klebsiella
./ Pelkanspurs = metaphysealspurs projectingat Age: most prevalentin the young
rightanglesto shaft axis Location: lowerextremity(75%)with hip + knee in g0%
r/ Parkecornersign = subepiphysealinfractioni . pain,limp,pseudoparalysis
. warmth,swelling
comminution resultingin mushrooming / cupping
. septicclinicalpicture
of epiphysis(DDx:syphilis,rickets)
. bacteremia,leukocytosis
@ Epiphysis
r/ Wimbergerring = scleroticring aroundlow-density
epiphysis(due to osteopeniaof epiphysis) A C U TES IGN S :
^/ initialradiographsfrequentlynormal
@ Diaphysis
{ subperiostealhematomawith calcificationot r/ softtissue swelling (firstsign secondaryto local
elevatedperiosteum(suresign of healing) hyperemia+ edema)
 RadiologyReviewManual

r/ jointdistension(effusion) + subluxation of hip and uptakeon delayedimages

linearlongitudinal
humerusin children(early) MR:
./ loint space narrowing= rapiddevelopmentof { marrowedema/ hemorrhage
destructionof articularcartilage(not in tuberculous { periostealfluid
arthritis)
{ rapid periarticularosteoporosis SHORT-RIBPOLYDACTYLY
SYNDROME
US: = group of autosomalrecessivedisorderscharacterized
r/ detection+ follow-upof hip effusion by short limb dysplasia,constrictedthorax,postaxial
NUC: polydactyly(on ulnar/ fibularside)
r/ increasedactivityin joint + adjacentosteomyelitis TY P E I -S A LD IN O-N OON A N S Y N D R OME
{ occasionallydecreasedactivitysimulating ryPE II = MAJEWSKI TYPE
avascularnecrosis(2" to joint effusion,vascular TYPEIII = NAUMOFF TYPE
spasm,vascularcompression) TY P EB E E ME R
MR:
r/ hypointensesignalin bone marrowon TlWl { severemicromelia
r/ enhancementof abnormalbone marrowon fat- { pointedfemursat bothends (typel);widened
suppressedTl Wl , metaphyses(type lll)
r/ hyperintensesignalin bone marrowon fat- { narrowthorax
suppressedT2Wl { extremelyshort horizontallyorientedribs
{ distortedunderossifiedvertebralbodies+ incomplete
SUBACUTESIGNSafter8-10 days: coronalclefts
r/ small marginal+ centralerosionsin articularcortex/ { polydactyly
loss of entirecorticaloutline(marginalerosionsin r/ cleft lip / palate
tuberculosis) Prognosis.' uniformlylethal
{ reactivebone sclerosisin underlyingbone
r/ subchondralbone destruction(by synovial SICKLECELLDISEASE
proliferation) Abnormalhemoglobins:
{ defectivereparation/ ankylosis(if entirecartilageis HbS = DNA mutationsubstitutingglutamicacid in
destroyed) position6 on B-chainwith valine
r/ localbone atrophy(immobility) HbC = DNA mutationsubstituting glutamicacid in
{ metaphyseatbone destruction(if osteomyelitisis position6 on B-chainwith lysine
sourceof septicjoint) (a) homozygous= HbSS= sicklecell anemia
(b) heterozygous = HbSA= sicklingtraitbut no anemia
Dx: (1) promptarthrocentesis: (c) heterozygousvariants:
. turbid/ franklypurulentsynovialfluid - HbSC (lesssevereform)
. fluid WBC >20,000/mm3 with predominanceof - HbS B-thalassemia anemia(seenoccasionally)
PMNs lncidence; 8-1 3'h of American Blacks carry sickling
. positiveresultof Gram stain factor(HbS);1:40with sicklecelltraitwill
(2) blood culture manifestsicklecell anemia(HbSS);1:120
Cx: (1) bone growthdisturbance(lengthening, with sicklecelltraitwill manifestHbSC
shortening, angulation) disease
(2) chronicdegenerativearthritis Pathogenesis:
(3) ankylosis alteredshape + plasticityof RBCs under lowered
(4) osteonecrosis= oV€lscuhrnecrosis oxygentensionlead to increasedbloodviscosity,stasis,
"logjam" occlusionof smallbloodvessels,infarction,
SHIN SPLINTS necrosis,superinfection; damageof intimaoccursmost
= SHINSORENESS = MEDIAL TIBIALSTRESS SYNDROME frequentlyin vesselswith high flow rates (terminalICA);
= SOLEUS SYNDROME sicklingoccursin areasof
= Dor-rspecific
term describingexertionallower leg pain (a) slowflow (spleen,liver,renalmedulla)
lncidence: 75o/"of exertionalleg pain (b) rapid metabolism(brain,muscle,fetal placenta)
Cause: ? atypicalstress fracture,traction periostitis, . chronichemolyticanemia(increasedsequestrationof
compartmentsyndrome sickledRBCsin spleen),jaundice
. diffusetendernessalong posteromedialtibia in its . chronicleg ulcers,priapism
middleto distalaspect . abdominalcrisis
Location: posterior/ posteromedialtibialcortex . rheumatism-like jointpain
Plainradiographs: . skeletalpain (osteomyelitis, bone marrow
cellulitis,
r/ normal/ longitudinalperiostealnew bone infarction)
Bonescintigraphy: . splenomegaly(in children+ infants),laterorgan atrophy
r/ normalradionuclideangiogram+ blood-poolphase Cx: high incidenceof infections(lung,bone,brain)
(DDx to stressfracture) Prognosis; death <40 years
 Boneand Soft-Tissue
Disorders 159

(1) DE O S S |F |CA T IOND U ET O M AR R OWH y p E RpLA S tA . stroke(5-17%): ischemicinfarction(70%),ischemia

r/ porousdecreasein bone densityof skull (25%) of deep white matter(25%),hemorrhage(20%),
r/ wideningof diploewith decreasein widthof outer embolicinfarction
table (22%) Angio(in 87% abnormal):
r/ verticalhair-on-end striations(5%) r/ arterialstenosis/ occlusionof supraclinoidportion
r/ osteoporosiswith thinningof trabeculae of ICA + proximalsegmentsof ACA and MCA
^/ biconcave"fish"vertebrae(bonesoftening)in 70./" .i moyamoyasyndrome(35%)
r/ wideningof medullaryspace+ thinningof cortices ^/ distatbranchocclusion(secondaryto thrombosis/
./ coarseningof trabecularpatternin long + flat bones embolism)
r/ rib notching r/ aneurysm(rare)
r/ pathologicfractures C T:
(2) T HRO M B O STASN D IN F A R C T T O N r/ cerebralinfarction(meanage of 7.7 years)
Location: in diaphysisof smalltubularbones(children); r/ subarachnoidhemorrhage(meanage of 27 years)
in metaphysis+ subchondrium of long
bones(adults) @ Chest
r/ osteolysis(in ACUTE infarction) { cardiomegaly
+ CHF
r/ dystrophicmedullarycalcification
r/ periostealreaction(bone-within-bone appearance) @ Gallbladder
r/ juxtacorticalsclerosis r/ cholelithiasis
r/ Lincolnlog = psynoldsign = H-vertebfde= steplike
endplatedepression @ Kidney
r/ articulardisintegration . hematuria
r/ collapseof femoralhead (DDx: Pertheswith . hyposthenuria
involvement of metaphysis) . nephroticsyndrome
MR: . renaltubularacidosis(distal)
r/ diffuselydecreasedsignalof marrowon short . hyperuricemia
+ long TR/TE images(= hematopoieticmarrow . progressiverenal insufficiency
replacingfatty marrow) r/ normalurogram(70%)
r/ focal areasof decreasedsignalintensityon short r/ papillarynecrosis(20%)
TR/TE + increasedintensityon long TR/TE r/ focal renal scarring(20%)
(= dcute marrowinfarction) r/ smooth large kidney(4%)
./ focal areas of decreasedsignalintensityon short MR:
TRiTE + long TR/TE images(= old infarction/ ./ decreasedcorticalsignalon T2-weightedimages
fibrosis) (renalcorticaliron deposition)
(3) SECONDARYOSTEOMYELITTS
Organism: Salmonellain unusualfrequency,also @ Spleen
Staphylococcus { splenomegaly < age 10 (in patientswith
r/ periostitis(DDx:indistinguishable from bone heterozygoussicklecell disease)
infarction) Cx: splenicrupture
r/ dactylitis= hand-footsyndrome { splenicinfarction
(4) GROWTHEFFECTS(secondary to diminishedblood r/ hemosiderosis
supply)
Location: particularly in metacarpal / phalanx FunctionalAsplenia
^/ boneshortening= preffi?tureepiphysealfusion = ?natomically presentnonfunctional
spleen
./ epiphysealdeformitywith cuppedmetaphysis . Howell-Jollybodies,siderocytes,anisocytosis,
{ cup / peg-in-hole defectof distalfemur irreversibly
sickledcells
ri diminutionin vertebralheight(shortening of stature r/ normal-sized / enlargedspleenon CT
+ kyphoscoliosis) ^/ absenceof traceruptakeon sulfurcolloidscan

Autosplenectomy
@ Brain = autoinfarction
of spleenin homozygoussicklecell
Pathophysiology: disease(functionlost by age 5)
chronicanemiaproducescerebralhyperemia, Histo: extensiveperivascularfibrosiswith
hypervolemia, impairedautoregulation depositionof hemosiderin + calcium
(a) cerebralbloodflow cannotbe increasedleading ri small(as smallas 5-10 mm) denselycalcified
to infarctionin time of crisis spleen
(b) increasedcerebralbloodflow producesepithelial
hyperplasia of largeintracranial
vessels(terminal Acute Splenic Sequestration Crisis
ICA/ proximalMCA) resultingin thrombus = suddentrappingof largeamountof bloodin
formation spleen
160 Radiology Review Manual
Cause: obstructionof small intrasplenicveins/
sinusoids;unknowntriggerevent
Age: (a) homozygous:infancy/ childhood
(b) heterozygous:any age
. LUQ pain
. suddenmassivesplenicenlargement
. rapiddrop in hemoglobin,hematocrit,platelets
(spleentraps largevolumesof blood)
. rise in reticulocytes
r/ enlargedspleen
r/ multiplelesionsat peripheryof spleen:
hypoechoicby US, of low attenuationby CT
{ hyperdenseareas (due to acute hemorrhage)
{ hyperintense areason TlWl + T2Wl (dueto
subacutehemorrhage)
r/ main splenicvesselspatentby DopplerUS
Prognosis; in 50% death <2 years of age (due to
hypovolemicshock)
Bone marrowscintigraphy:
r/ usuallysymmetricmarkedexpansionof
hematopoieticmarrowbeyondage 20 involvingentire
femur,calvarium,small bones of hand + feet
(normallyonly in axialskeleton+ proximalfemurand
humerus)
r/ bone marrowdefectsindicativeof acute/ old
infarction
Tc-99mdiphosphonatescan:
r/ increasedoverallskeletaluptake(highbone-to-soft
, tissueratio)
r/ prominentactivitiesat knees,ankles,proximal
humerus(delayedepiphysealclosure/ increased
bloodflow to bone marrow)
^/ bone marrowexpansion(calvarialthickeningwith
relativedecreasein activityalong falx insertion)
{ decreased/ normaluptakeon bone scan within
24 hours in acute infarction/ posthealingphase
followinginfarction(cystformation)
{ increaseduptake on bone scan after 2-10 days
persistentfor severalweeks in healinginfarction
^/ increaseduptakeon bone scan within 24-48 hours in
osteomyelitis
r/ increasedblood-poolactivity+ normaldelayedimage
on bonescan in cellulitis
r/ renalenlargementwith markedretentionof tracer in
renalparenchyma(medullaryischemia+ failureof
countercurrentsystem)in 50%
r/ persistentsplenicuptake( secondaryto
degeneration,atrophy,fibrosis,calcifications)
Sickle Cell Trait
Hb SA carrier;milddiseasewith few episodesof crisis
+ infection;sicklingprovokedonly underextreme
stress(unpressurized aircraft,anoxiawith CHD,
prolongedanesthesia,marathonrunning)
lncidence; in 8-10/" of AmericanBlacks
. may have normalbloodcount
. recurrentgross hematuria
{ splenicinfarction
SC Disease
Hb SC carrier
lncidence: 3/o of AmericanBlacks
. retinalhemorrhages
. hematuriadue to multipleinfarctions
{ asepticnecrosisof hip
Sickle-ThalDisease
ResemblingclinicallyHb SS patients
. anemia(no normaladulthemoglobin)
rl persistentsplenomegaly
SINDING.LARSEN.JOHANSSON DISEASE
= osteochondrosis of inferiorpole of patella,often bilateral
(NOT osteonecrosis/ epiphysitisi osteochondritis)
Cause: tractionwith contusion+ subsequenttendinitis/
traumaticavulsionof bone;repeated
subluxation + dislocation of patella
Age: adolescents(often10-14 years)
Predisposed: cerebrospasticchildren
. tenderness+ soft-tissueswellingover lowerpole of
patella
rl peripatellarsoft-tissueswelling
r/ calcification/ ossificationof patellartendon
r/ small bone fragmentsat lowerpole of patella(LAT view)
MR:
ri hypointense area on Tl Wl + hyperintense on T2Wl in
inferiorpoleof patella+ surrounding softtissues
SMALLPOX
5'/. of infants
Location: elbowbilateral;metaphysisof longbones
i/ rapidbone destructionspreadingalongshaft
r/ periostealreaction
r/ endosteal+ corticalsclerosisfrequent
r/ prematureepiphysealfusionwith severedeformity
r/ ankylosisis frequent
SOFT.TISSUE CHONDROMA
= EXTRASKELETAL CHONDROMA = CHONDROMA OF SOFT
PARTS
lncidence: 1.5/" of all benignsoft-tissuetumors
Age: 30-60 years(range1-85 years); M:F = 1.2:1
Histo: adult-typehyalinecartilagewith areasof
calcification+ ossification;myxoidchange;regions
of increasedcellularity+ cytologicatypia
. slow-growingsoft-tissuemass
. occasionally pain + tenderness
Location: hand (54-64%) + foot (20-28%)
^/ lobulatedwell-definedextraskeletalmass <2 cm in size
r/ may containcalcifications(33-70%)with ringlike
appearance/ ossifications
{ scaltopingof adjacentbone with scleroticreaction
MR:
^/ trighsignalintensityon T2Wl
r/ intermediate signalintensityon Tl Wl
Rx: localexcision
Prognosis: 15-25'/.recurrence rate
 Boneand Soft-TissueDisorders 1 6 1

DDx: (1) Extraskeletalmyxoidchondrosarcoma SOLITARYPLASMACYTOMA

OF BONE
(deep-seatedin large musclesof upper+ lower = fepfes€ntsearly stage of multiplemyeloma,precedes
extremities,pelvic+ shouldergirdles) multiplemyelomaby 1-20 years
(2) Periostealchondroma Age: sth-7th decade
. negativemarrowaspiration;no lgG spikein serum/
SOFT.TISSUE
OSTEOMA uri ne
= OSTEOMA OF SOFTPARTS(extremety rare) A. SOLITARYMYELOMAOF BONE
Histo: maturelamellarbone with well-definedhaversian Site: thoracic/ lumbarspine(mostcommon)> pelvis
system;bone marrow,myxoid,vascular,fibrous > ribs> sternum,skull,femora,humeri
connectivetissuebetweenbone trabeculae; r/ solitary"bubbly"osteolyticgrosslyexpansilelesion
collagenous capsuleblendingintobenignhyaline r/ poorlydefinedmargins,Swiss-cheesepattern
cartilage r/ frequentlypathologicfracture(collapseof vertebra)
Location: head (usuallyposteriorpart of tongue),thigh DDx: giantcelltumor,aneurysmalbonecyst,
r/ ossifiedmass osteoblastoma, solitarymetastasisfrom renal
NUC: cell/ thyroidcarcinoma
^/ intensetraceraccumulation,greaterthan adjacent B. EXTRAMEDULLARY PLASMACYTOMA
bone Location: majorityin head + neck;80% in nasal
cavity,paranasalsinuses,upperairways
of trachea,lung parenchyma
SOLITARY
BONECYST
= UNICAMERAL / SIMPLE BONECYST SPON DYLOEPI PHYSEAL DYSPLASIA
lncidence: up to 5/" ol primarybone lesions Spondyloepiphyseal
DysplasiaCongenita
Etiology: ? trauma(synovialentrapmentat capsular Autosomal
dominant (most)
/ sporadic
reflection),? vascularanomaly(blockageof . disproportionate dwarfismwith spine + hips more
interstitialdrainage) involvedthan extremities
Histo: cyst filledwith clear yellowishfluid often under . waddlinggait + muscularweakness
pressure,wall linedwith fibroustissue+ . flat facies
hemosiderin, giantcellsmay be present . short neck
Age: 3-19 years (80%);occursduringactivephaseof . deafness
bonegr ow th ;M :F = 3 :1 r/ cleft palate
. asymptomatic,unlessfractured
@ Axial skeleton
Location: proximalfemur+ proximalhumerus r/ ovoidvertebralbodies+ severeplatyspondyly
(60-75/.), fibula,at base of calcanealneck (incomplete fusionof ossification
centers
(4/", >12 yearsof age),talus;rare in ribs, + flatteningof vertebralbodies)
ilium,smallbonesof hand+ feet (rare),NOT r/ hypoplasiaof odontoidprocess
in spine/ calvarium;solitarylesion (Cx: cervicalmyelopathy)
Site: intramedullary centricmetaphyseal,adjacentto { progressive kyphoscoliosis (shorttrunk)involving
epiphysealcartilage(duringactivephase)/ thoracic+ lumbarspine
migratingintodiaphysiswith growth(duringlatent r/ narrowingof disk spaces(resultingin short trunk)
phase),does not cross epiphysealplate .i broad iliacbases + deficientossificationof pubis
'V Z-g cm oval radiolucencywith long
axis parallelto long r/ ttat acetabularroof
axis of host bone @ Chest
fine scleroticboundary r/ bell-shapedthorax
{ scalloping+ erosionof internalaspectof underlying r/ pectuscarinatum
cortex
@ Extremities
{ photopenicarea on bone scan (if not fractured) { normal/ slightlyshortenedlimbs
"fallenfragment"
sign if fractured(20%)= centrally ^/ severecoxa vara + genu valgum
dislodgedfragmentfalls into a dependentposition r/ multipleaccessoryepiphysesin hands+ feet
Prognosis; mostlyspontaneousregression r/ talipesequinovarus
Cx: pathologicfracture(65%) Cx: (1) Retinaldetachment, myopia(50%)
DDx: (1) Enchondroma (calcificstipplings) (2) Secondaryarthritisin weight-bearing joints
(2) Fibrousdysplasia(moreirregularlucency)
(3) Eosinophilic granuloma Spondyloepiphyseal
DysplasiaTarda
(4) Chondroblastoma (epiphyseal) = sex-linkedrecessiveform with mildermanifestation
(5) Chondromyxoid fibroma(moreeccentric + laterclinicalonset
+ expansile) Age: apparentby 10 years;exclusiveto males
(6) Giantcelltumor r/ hyperostoticnew bone along posterior213of vertebral
(7) Aneurysmalbone cyst (eccentric) end plate(PATHOGNOMONIC)
(8) Hemorrhagiccyst { platyspondylywith depressionof anterior1/3 of
(9) Browntumor vertebralbody
 r/ narrowingwith calcificationof disk spaces Age: children+ adolescents(withinfirst 2 decadesof
+ spondyliticbridging l i f e ) ;M : F = 2 : 1
r/ shorttrunk . slow-growingasymptomaticsoft-tissuemass
r/ dysplasticjoints (eg, flattenedfemoralheads) Location:deep palmarfasciaof hand + wrist (67%);
{ prematureosteoarthritis soles of feet
DDx: Ochronosis r/ nonspecificsoft-tissuemass overlyinginflamedbursa
(oftenmistakenfor calcifiedbursitis)
{ stippledcalcifications(frequent)
SP RE NG E L DE F OR M IT Y { interosseoussoft-tissuemass of forearm+ wrist
= failureof descentof scapulasecondaryto fibrous/ r/ erosion/ scallopingof bone may occur
osseousomovertebralconnection Prognosis.' recurrencerate of >50% after resection
Associatedwith: Klippel-Feilsyndrome,renalanomalies Dx: biopsy(to differentiatefrom synovialsarcoma)
. webbed neck DDx: synovialsarcoma(commonlycalcifies,bone
. shoulderimmobility erosion),chondroma,fibrosarcoma,
{ elevation+ medialrotationof scapula osteosarcoma,myositisossificans

PalmarFibromatosis
SUBUNGUAL EXOSTOSIS = DUPUYTREN DISEASE
= DUPUYTREN EXOSTOSIS Prevalence: 1-2%
Cause: repetitivetrauma (14-25%) A ge: i n24h of peopl e> 65 years; M:F = 4:1
. subcutaneous noduleson palmarsurfaceof distal
Age: 2nd - 3rd decade(range,7-58 years)
Histo: proliferatingfibroblastsdevelopinginto creaseof hand progressingto cords and bands
fibrocartilage+ bone . flexioncontractures of digits(2'to fibrousattachment
Location: toes (86-90%, great toe in 77-80%), thumb to flexortendons)
+ indexfinger(10-147",dominanthandin 75%) Location: 4th + Sth (mostcommonly)> 2nd + 3rd
Site: dorsal/ dorsomedialaspectof distalphalanx digit;bilateralin 42-60oh
. mass under/ adjacentto nail bed t rapidgrowth r/ predominantly hypointense on T2Wl + Iowto
. may be painfulwithoverlyingskin ulceration intermediate intensityon TlWl (in hypocellular lesions)
r/ ossificmass distalto physealscar: ^/ intermediatesignalintensityon T2Wl (proliferative
^/ ttO continuityto cortex/ medullaof host bone hypercellular lesions)
r/ broad / narrow base
^/ indistinct/ well-demarcated cartilagecap largerthan
base PlantarFibromatosis
Rx: completesurgicalexcision = PLANTAR FASCIITIS = LEDDERHOSE DISEASE
Prognosis: 11-53/" recurrencerate = corTrllroh
form of superficialfibromatosis
DDx: osteochondroma(exostosiscontinuouswith cortex Cause: trauma
and medullaof host bone) Age: 30-50 years
Path: abnormalfibroustissuereplacingthe plantar
aponeurosis and infiltrating
subcutaneous tissue
SUPERFICIAL FIB ROMATOSES + skin
InfantileDigitalFibromatosis Histo: nonencapsulated proliferationof fibroblasts
= REYETUMOR= INFANTILEDIGITALFIBROMA separatedby variableamountsof collagen
= INFANTILEDIGITALMYOFIBROBLASTOMA At risk: runners,obese patients
= single/ multiplenodulardermalprotrusionof fibrous Associatedwith (in 50%): Dupuytrencontracture
tissueon extensorsurfaceof digits (10-65%), Peyroniedisease
Age: 1st year of life (>80%);30% congenital . heelpain (oneof the mostcommoncauses)
Histo: intracytoplasmic perinuclearinclusionbodies . one / multiplefirm fixedsubcutaneous nodules
Location: fingers(60%) , toes (40%) Location: proximal/centralportionof plantar
site: lateralaspectof distal/ middlephalanx aponeurosis; bilateralin 20-50%
^/ nonspecificsofttissue mass involvinga digit Site: medialaspectof aponeurosis
r/ infrequentlybone involvement { calcanealspur
Prognosis; spontaneousregression(in 8%); MR:
60% recurrencerate after excision { singleor multiplenodules/ poorlydefinedinfiltrative
mass iso-/ hypointensecomparedto plantar
JuvenileAponeuroticFibroma muscl eson Tl W l + T2W l
= CALCIFIED APONEUROTIC FIBROMA r/ markedcontrastenhancementin 50%
= fdre locallyaggressivebenignfibroustumor r/ t subcutaneus edema
Histo: cellulardense fibroustissuewith focalchondral
elementsinfiltratingadjacentstructures SYNOVIAL OSTEOCHON DROMATOSIS
(= cartilaginous
tumor) = SYNOVIAL = JOINTCHONDROMA
CHONDROMATOSIS
 Boneand Soft-Tissue
Disorders 163

PrimarySynovialOsteochondromatosis (2) Malignantdedifferentiation

to chondrosarcoma
= benignself-limiting monoarticulardisorder Rx: removalof loosebodies(recurrenceis common)
characterizedby proliferation+ metaplastic DDx: (1) Synovialsarcoma,chondrosarcoma
transformationof the synoviumwith formationof (2) Osteochondralfracture(historyof trauma),
multipleintrasynovial cartilaginous
/ osteochondritis
dissecans,osteonecrosis
osteocartilaginous nodules (3) Secondaryosteochondromatosis
Cause: hyperplasticsynoviumwith cartilage (4) Pigmentedvillonodularsynovitis,synovial
metaplasia(foci<2-3 cm); loosebody may hemangioma,lipomaarborescens
remainfree floating/ conglomerate with other
loosebodiesinto largemass / reattachto SecondarySynovialOsteochondromatosis
synoviumwith eitherreabsorptionor = joint surfacedisintegration
continuedgrowth Cause: trauma,osteonecrosis,rheumatoidarthritis,
Histo: foci of hyalinecartilagewith mineralized neuropathicarthropathy,tuberculousarthritis,
chondroidmatrixbeneathsynovialsurface degenerativejoint disease
,
+ withinsubsynovial connectivetissue; r/ intraarticular
bodiestendto be larger,less numerous,
hypercellularity + nuclearatypiamay be morevariedin size comparedto primarysynovial
confusedwith malignancy osteochondromatosis
Composition of cartilaginousbodies: r/ prominentosteoarthritis
cartilagealone / cartilage+ bone I malure
bone + fatty marrow
Age: presentsin 3rd-5th decade; M:F = 2-4:1 SYNOVIOMA
. slow-growingsoft-tissuemass in joint = SYNOVIAL SARCOMA
. progressivejoint pain for severalyears with limitation = slow-growing expansilemalignanttumororiginatingin
of motion/ locking the synoviallining/ bursa/ tendonsheath;uncommonly
. + hemorrhagic jointeffusion intraarticular
Location: knee (mostcommonwith >50%, in 10/" lncidence: 10/" of soft-tissuesarcomas
bilateral)elbow> hip > shoulder> ankle> Histo: fibrosarcomatous + synovialcomponent
wrist;usuallymonoarticular, occasionally Age: 3rd-5th decade; M:F = 2:3
bilateral . painfulsoft-tissuemass
Sites: joint/ tendonsheath/ ganglion/ bursa Location: knee (mostcommon),hip,ankle,elbow,wrist,
r/ multiplecalcified/ ossifiedloosebodiesin a single hands,feet;usuallysolitary
joint ./ largespheroidwell-definedsoft-tissuemass
(bonyshellof remodeledlamellarbone is rare) ^/ lesionabout 1 cm removedfrom joint cartilage
r/ size of nodulesvariesbetweena few mm to several ^/ amorphouscalcifications (1/3),oftenat periphery
cm { involvementof adjacentbone (11-20%\
r/ varyingdegreesof bone mineralization (25-30.h of r/ periostealreaction
chondromasshow no radiopacity) r/ bone remodeling(pressurefrom tumor)
^/ pressureerosionof adjacentbone in jointswith tight r/ invasionof cortexwith wide zone of transition
capsule(eg, hip) r/ juxtaarticularosteoporosis
r/ wideningof jointspace(fromaccumulation of loose MR:
bodies) ./ low signalintensityon TlWl
^/ lointeffusionuncommon ! inhomogeneously increasedsignalintensityon T2Wl
r/ tttOosteoporosis { mu[ilocularappearancewith internalseptation
CT : ^/ tluid-ttuid
levels(previoushemorrhage)
r/ multiplecalcified/ ossifiedintraarticular
bodies Rx: localexcision/ amputation+ radiation/
r/ intraarticularsoft-tissuemass of near-water chemotherapy
attenuation containingmultiplesmallcalcifications
MR: S Y P H ILIS OF B ON E
r/ lobulatedintraarticular mass isointenseto muscle CongenitalSyphilis
on Tl Wl + hyperintense to muscleon T2Wl 0 Transplacental transmission
cannotoccur<16 weeks
containingmultiplefoci of low signalintensity gestationalage
DDx: largeeffusion,softtissue tumor . positiverapidplasmareagin(measuresquantityof
r/ peripheralcontrastenhancement of chondral antibodiesto assessnew infection/ efficacyof Rx)
lesions . positivemicrohemagglutination
r/ centralarea of high-signalintensityon Tl Wl for test for Treponema
pallidum(remainsreactivefor life)
intraarticularbodieswith fatty marrow
Cx: (1) Long-standingdiseasemay lead to { pneumoniaalba
degenerativearthritis(fromchronic r/ hepatomegaly
mechanicalirritation+ destructionof articular Location: symmetricalbilateralosteomyelitis
involving
cartilageby loosebodies) multiplebones(HALLMARK)
 A. Earlyphase
0 Skeletalradiographyabnormalin 19% of infected
newbornswithoutovertdisease!
1. Metaphysitis
r/ lucentmetaphysealband adjacentto thin /
widenedzone of provisionalcalcification
(disturbance in enchondralbone growth)
./ frayededge of metaphyseal-physeal junction
(osteochondritis)= erosions+ lyticdefects
2. Diaphysealperiostitis = "lueticdiaphysitis"
r/ solid/ lamellatedperiostealnew-bonegrowth
= bone-within-bone appearance
3. SpontaneousepiphysealfracturescausingParrot
pseudopalsy(DDx:batteredchild syndrome)
4. Bone destruction
r/ marginaldestructionof spongiosa+ cortex
alongside of shaftwith wideningof medullary
canal(in shorttubularbones)
r/ patchyrarefactionin diaphysis
5. Wimbergersign
r/ symmetricalfocal bone destructionof medial
portionof proximaltibial metaphysis(ALMOST
PATHOGNOMONTC)
B. Late phase
. Hutchinson triad= dentalabnormality,interstitial
keratitis.8th nerve deafness
r/ frontalbossingof Parrot= diffusethickeningof
outertable
{ saddlenose + high palate(syphilitic chondritis
+ rhinitis)
r/ short maxilla(maxillaryosteitis)
r/ thickeningat sternalend of clavicle
{ "saber-shin"deformity= snlgpiorlyconvexbowing
in upper213of tibiawith bonethickening
AcquiredSyphilis
= TERTIARYSYPHILISresembleschronicosteomyelitis
r/ dense bone sclerosisof long bones
r/ irregularperiostealproliferation+ endosteal
thickeningwith narrowmedulla
r/ extensivecalvarialbone proliferationwith mottled
pattern(anteriorhalf + lateralskull)in outertable
(DDx: fibrousdysplasia,Pagetdisease)
^/ itt-detinedtyticdestructionin skull,spine,long bones
(gummaformation)
^/ enlargementof clavicle(cortical+ endostealnew
bone)
r/ Charcotarthropathyof lowerextremities+ spine
TARSAL COALITION
= abnormalfibrous/ cartilaginous / osseousfusionof two
or more tarsalossificationbones
0 Clinicallymost importantcongenitalproblemof calcaneus
Prevalence: 1-2% of population
Cause: abnormalsegmentationof primitive
mesenchymewith lack of joint formation
Age: fibrouscoalitionat birth,ossificationduring2nd
decadeof lifewith onsetof symptoms;M:F = 1:1
. asymptomatic;oftenfirst notedafter antecedenttrauma
/ weightgain / increasein athleticactivity
. peronealspastic/ rigidpes planus(=flatfoot)in
adjustmentfor calcaneusvalgus (= heel valgus)
. hindfoot/ tarsalpain or stiffness
{ ootn feet affected in 2o-50%
{ osseousbars betweenbones of hindfoot/ bones in
close proximitywith irregularsurfaces
MR (of jointspace):
r/ bone marrowcontiguity(osseouscoalition)
./ ttuiO-/ cartilage-intensity (cartilaginouscoalition)
./ intermediate-to low-signalintensity(fibrouscoalition)
r/ reactiveperiarticularbone changes
^/ bone marrowedemaalongfusedjoint (STIRimages)
Types:
(1) Calcaneonavicular coalition(45%)
Age: 8-12 years (due to earlierossification)
. rigidflat foot+ pain in 2nd decadeof life
Radiographs:
r/ narrowedcalcaneonavicular joint with indistinct
articularmargins(bonesthat usuallydo not
articulate)
{ widening/ flatteningof anteromedial calcaneus
r/ "anteater'snose"= elongationof anteriordorsal
calcaneuson lateralradiograPh
^/ hyPoPlastic talar head
CT (axialscan):
{ broadeningof medialaspectof anterodorsal
calcaneusin appositionto navicular
{ narrowingof space betweenthe 2 bones
+ minimalmarginalreactivesclerosis
Dx: mostlydiagnosedon 45" internalobliquefilms
(2) Talocalcanealcoalition(45%)
Age: 12-16 years
. painfulperonealspasticflat foot, relievedby rest
Site: middlefacet at levelof sustentaculumtali
(mostfrequentlY)
Secondaryradiographicsigns (due to alterationin
hindfootbiomechanics) :
./ prominenttalar beak (66%)arisingfrom dorsal
aspectof head i neck of talus (due to impaired
subtalarjoint motion)
{ roundingof the lateraltalar process
{ narrowingof posteriorsubtalarjoint
r/ lack of depictionof middlefacets
r/ asymmetricanteriortalocalcanealjoint
r/ "ball-in-socket" ankle mortisein severecases
r/ "C sign"= C-shapedoutlineof the medialtalar
dome + posteroinferior sustentaculumon lateral
radiograph(frombone bridgebetweentalar
dome + sustentaculum)
CT (coronalscan):
{ bony bar bridgingthe middlefacetof subtalar
j oi nt
./ narrowedmiddlefacet with reactivecystic
+ hypertrophicchanges
./ downwardor horizontalslopeof sustentaculum,
insteadof uPward
Dx: requirescross-sectional imagingfor diagnosis
(3) Talonavicular coalition
(4) Calcaneocuboid coalition
(5) Cubonavicularcoalition
 Boneand Soft-TissueDisorders 165

Rx: orthotics,casting,NSAID,steroidinjections, ! thinningof outertable

physicaltherapy,resection,arthrodesis r/ frontalbossing
DDx: acquiredintertarsalankylosis(infection,trauma, r/ severehair-on-endappearance(frontalbone,
arthritis,surgery) NOT inferiorto internaloccipitalprotuberance)
{ marrowexpansionin paranasalsinuses:
THALASSEMIA
SYNDROMES r/ impairedpneumatization of maxillaryantra
= inheriteddisordersof hemoglobinsynthesistypically + mastoidsinuses
seen in individualsof Mediterraneandescent { narrowingof nasalcavity
PhysioIogic hemogIobins : r/ rodentfacies= ventraldisplacementof incisors
(a) in adulthood: (marrowovergrowthin maxillarybone)with
Hb A (98% = 2 s.- and 2 B-chains); dentalmalocclusion
Hb 42 (2/o = 2 u- and 2 6-chains) r/ lateraldisplacementof orbits
(b) in fetal life, rapidlydecreasingup to 3 monthsof @ Peripheralskeleton:
newbornperiod: . earliestchangesin smallbonesof hands+ feet
Hb F (= 2 cr-and 2 Y-chains) (>6 monthsof age)
A. ALPHA-THALASSEMIA r/ diffuseosteopenia:
= decreasedsynthesisof a-chainsleadingto excessof r/ atrophy+ coarseningof trabeculae(from
B-chains+ y-chains(Hb H = 4 F-chains; marrowhyperplasia)
HbBart=4T-chains) r/ prominenceof nutrientforamina
. diseasebeginsin intrauterinelife as no fetal ri widenedmedullaryspaceswith thinningof cortices:
hemoglobinis produced r/ Erlenmeyer flaskdeformity= bulgingof normally
. homozygosityis lethal(lackof oxygentransport) concaveoutlineof metaphyses
B. BETA-THALASSEMIA { prematurefusionof epiphyses(10%),usuallyat
= decreasedsynthesisof B-chainsleadingto excessof proximalhumerus+ distalfemur
g-chains+ y-chains(= fetalhemoglobin) ! arthropathy(secondaryto hemochromatosis
. diseasemanifestin early infancy + CPPD + acute gouty arthritis)
(a) homozygousdefect= thalassemiamajor= Cooley ^/ regressionof peripheralskeletalchanges(as red
anem ia marrowbecomesyellow)
(b) heterozygous defect= thalassemiaminor @ Chest:
{ cardiacenlargement+ congestiveheartfailure
ThalassemiaMajor (secondary to anemia)
= COOLEY ANEMIA = MEDITERRANEAN ANEMIA r/ paravertebralmasses(= extramedullary
= HEREDITARY LEPTOCYTOSIS = P.THALASSEMIA hematopoiesis)
= ffiostsevereform with trait inheritedfrom both parents @ Ribs
(= homozygousform) r/ costalosteomas= bulbouswideningof posterior
lncidence: 1o/otor AmericanBlacks;7 .4% for Greek aspectof ribs with thinnedcortices
population; 10/" for certainltalian r/ undertubulated broad ribs
populations r/ heterogenousrib ossification:
Age: developsafter newbornperiodwithinfirst2 years r/ localizedlucencies
of life r/ corticalerosion
. retardedgrowth r/ rib-within-rib
appearance
. elevatedserumbilirubin @ Abdomen:
. hyperpigmentation of skin r/ hepatosplenomegaly
. hyperuricemia r/ gallstones
. secondarysexualcharacteristics retarded,normal
menstruationrare (primarygonadotropininsufficiency Cx: (1) Pathologicfractures
from iron overloadin pituitarygland) (2) lron overload+ hemosiderosisfrom frequent
. hypochromicmicrocyticanemia(Hb 2-3 g/dl), bloodtransfusiontherapy(absentpuberty,
nucleatedRBC,targetcells,reticulocytosis, decrease diabetesmellitus,adrenalinsufficiency,
in RBC survival,leukocytosis myocardialinsufficiency)
. susceptibleto infection(leukopeniasecondaryto
splenomegaly) Prognosis; usuallydeathwithin 1st decade
. bleedingdiathesis(secondaryto thrombocytopenia) Rx: systematictransfusionhas lessenedthe severity
of skeletalabnormalities
@ S k ull: DDx: chronicanemia,storagediseases,fibrous
. mongoloidfacies dysplasia
r/ marrowexpansionof diploe:
r/ wideningof diploicspacewith coarsened Thalassemia lntermed ia
trabeculationsand displacement(frommarrow = subgroupof homozygousform
hyperplasia= extramedullaryhematopoiesis) . milderclinicalpresentation
 . not requiringhypertransfusion
to maintainan r/ short-limbeddwarfismwith extremelyshort + bowed
"telephonereceiver"-like
femurs
adequatehematocrit
Prognosis; longer life expectancy { extremelysmall hypoplasticthoraxwith short ribs
dimension
+ narrowedin anteroposterior
^ i protuberant
Minor
Thalassemia abdomen
= beta-thalassemiatrait inheritedfrom one parent macrocraniawith frontalbossing+ hydrocephalus
(= heterozygous) (increasedHC:AC ratio)
. usuallyasymptomaticexceptfor periodsof stress { "cloverleafskull"(in 14%)(DDx:encephalocele)
(pregnancy,infection)
,Vdiffuseplatyspondyly
. microcytichypochromic anemia(Hb 9-11 g/dL) { redundantsoft tissues
. occasionally
jaundice+ splenomegaly
Prognosis; often stillborn;uniformlyfatal withina few
hoursi days afterbirth (respiratoryfailure)
THANATOPHORIC DYSPLASIA DDx: (1) Ellis-vanCreveldsyndrome(extradigit,
= Sporddiclethalskeletaldysplasiacharacterizedby acromesomelic shortlimbs)
severerhizomelia(micromelicdwarfism)transmittedby (2) Asphyxiatingthoracicdysplasia(lessmarked
a dominantgene mutation bone shortening,vertebraespared)
lncidence; 6.9:100,000 births;1:6,400-16,700births; (3) Short-ribpolydactylysyndrome
0 Most commonlethalbone dysplasiaafter (4) Homozygousachondroplasia
osteogenesisimperfectatype ll (5) Achondrogenesis
. severerespiratorydistress(earlyin life)
. hypotonicinfants RADIUS
THROMBOCYTOPENIA.ABSENT
. protuberantabdomen SYNDROME
. extendedarms + abductedexternallyrotatedthighs = TA R S Y N D R OME
= ta:teautosomalrecessivedisorder
@ Head Age: presentationat birth
r/ large head with short base of skull + prominent May be associatedwith: CHD (33%): ASD, tetralogy
frontalbone . plateletcount <1OO,O0O/mm3 ldecreasedproductionby
r/ occasionallytrilobedcloverleafskull bone marrow)
= "Kleeblattschddel" { usuallybilateralradialaplasia/ hypoplasia
@ Chestradiograph(PATHOGNOMONIC) { uni-/ bilaterallyhypoplastic/ absentulna/ humerus
{ narrow chest r/ defectsof hands,feet, legs
r/ short horizontalribs: Prognosis; death in 50% in early infancy(hemorrhage)
r/ not extendingbeyondanterioraxillaryline
^/ cuppedanteriorends ACROPACHY
THYROID
{ short curved"telephonehandle"humeri Onset: after 18 monthsfollowingthyroidectomyfor
r/ H- I U-shapedvertebraplana hyperthyroidism (doesnot occurwith antithyroid
r/ smallscapula+ normalclavicles medication)
@ Spine lncidence; 0.5-1% of patientswith thyrotoxicosis
r/ normallengthof trunk . clubbing,soft-tissue swelling
r/ reductionof interpediculate space of last few lumbar . eu- / hypo-i hyperthyroidstate
vertebrae Location: diaphysesof phalanges+ metacarpalsof
r/ extremegeneralizedplatyspondyly= s€ValeH- / hand;lesscommonlyfeet,lowerlegs,forearms
U-shapedvertebraplana ./ tfrict<spiculatedlacy "feathery"periostealreaction
r/ excessiveintervertebralspace height DDx: (1) Pulmonaryosteoarthropathy (painful)
@ Pelvis(hypoplastic iliacbones) (2) Pachydermoperiostosis
^/ itiacwings small+ square(verticalshorteningbut (3) Fluorosis(ligamentous calcifications)
wide horizontally)
r/ flat acetabulum TRANSIENT REGIONAL OSTEOPOROSIS
r/ narrow sacrosciaticnotch = TR A N S IE N TB ON EMA R R OWE D E MA
r/ short pubicbones Cause: unknown;? overactivityof sympatheticnervous
@ Extremities system+ localhyperemiasimilarto reflex
r/ severemicromelia+ bowingof extremities sympatheticdystrophysyndrome,trauma,
r/ metaphysealflaring= "telephonehandle" synovitis,transientischemia
appearanceof long bones
r/ thornlikeprojectionsin metaphysealarea RegionalMigratoryOsteoporosis
r/ polydactyly = rapidonset of self-limitingepisodesof severe
localizedosteoporosisand pain but repetitive
OB-US(findingsmay be seenvery earlyin pregnancy): occurrenceof same symptomsin other regionsof the
r/ polyhydramnios
(71%) same or oppositelower extremitY
 Boneand Soft-Tissue
Disorders 167

. rapidonset of localpain DDx: (1) AVN (cystic+ scleroticchanges,early

. diffuseerythema,swelling,increasedheat
. significant disabilitydue to severepain on weight
bearing
Age: middle-aged males
Location: usuallylowerextremity(ie,ankle,knee,hip,
foot)
r/ rapidlocalizedosteoporosiswithin4-8 weeks after
onset migratingfrom one joint to another;may affect
trabecular/ corticalbone
r/ linear/ wavy periostealreaction
r/ preservationof subchondralcorticalbone
{ no jointspacenarrowing,boneerosion
MR:
r/ affectedarea has low signalintensityon T1Wl, high
signalintensityon T2Wl (= bone marrowedema)
NUC:
^/ increasedactivity
Prognosis: persistsfor 6-9 months in one area;cycle
of symptomsmay last for severalyears
Rx: variableresponseto analgesics/ corticosteroids
Partial Transient Osteoporosis
= vari?ntof regionalmigratoryosteoporosiswith more
focal patternof osteoporosis,which may eventually
becomemoregeneralized
(a) Zonalform = portionof bone involved,ie, one
femoralcondyle/ one quadrantof femoralhead
(b) Radialform = only one i two rays of hand/ foot
involved
TransientOsteoporosisof Hip
= self-limiting diseaseof unknownetiology
Age: typicallyin middle-aged malesi in 3rd trimester
of pregnancyin femalesinvolvinglefthip; M > F
. spontaneous onsetof hip and groinpain,usually
progressiveover severalweeks
. painfulswellingof jointfollowedby progressive
demineralization
. rapiddevelopment of disability,limp,decreasedrange
of motion
Site: hip mostcommonlyaffected;generallyonly one
jointat a time
r/ progressivemarkedosteoporosisof femoralhead,
neck,acetabulum(3-8 weeks after onset of illness)
r/ virtuallyPATHOGNOMONIC strikinglossof
subchondralcortexof femoralhead + neck region
r/ lttOjoint space narrowing/ subchondralboneiollapse
NUC:
r/ markedlyincreaseduptakeon bone scan without
cold spotsi inhomogeneities (positivebefore
radiograph)
MR:
./ diffusebone marrowedema involvingfemoralhead
+ neck+ sometimesintertrochanteric region
r/ smalljointeffusion
Cx: pathologicfracturecommon
Prognosis; spontaneousrecoverywithin2-6 months;
recurrencein anotherjointwithin2 years
possible
subchondralundermining)
(2) Septici tuberculousarthritisfiointaspiration!)
(3) Monoarticularrheumatoidarthritis
(4) Metastasis
(5) Reflexsympatheticdystrophy
(6) Disuseatrophy
(7) Synovialchondromatosis
(8) Villonodular
synovitis
TRANSIENT SYNOVITIS OF HIP
= OBSERVATION HIP= TRANSITORY SYNOVITIS = TOXIC
SYNOVITIS = COXITIS FUGAX
= noospecificinflammatoryreaction
0 Mostcommonnontraumatic causeof acutelimp in a
chi l d
Etiology: unknown;no organismon joint aspiration
Age: 5-10 (average6) years; M:F = 2:1
. historyof recentviral illness(65%)
. developinglimpover 1-2 days
. pai ni n hi p,thi gh,knee
. mild fever (25/"), mildlyelevatedESR (50%)
r/ radiographsusuallynormal
r/ jointeffusion:
r/ displacementof femurfrom acetabulum
ri displacementof psoas line
ri lateraldisplacementof glutealline (leastsensitive
+ leastreliable)
r/ regionalosteoporosis(? hyperemia,disuse)
NUC:
r/ normal/ slightincreasein activity(excluding
osteomyelitis+ avascularnecrosis)
Prognosis; completerecoverywithin a few weeks
Dx: per exclusion
Rx: non-weight-bearingtreatment
DDx: trauma,Legg-Perthesdisease,acute rheumatoid
arthritis,acute rheumaticfever,septicarthritis,
tuberculosis, malignancy
TR E A C H E R -C OLLIN S S Y N D R OME
= MANDIBULOFACIAL DYSOSTOSIS
= autosomaldominantdisease(withnew mutationsin
60%) characterizedby bilateralmalformationsof eyes,
malarbones,mandible,and ears resultingin birdlike
face
lncidence: 1:50,000births
Cause: defectin growthof 1st + 3rd branchialarches
beforethe 7th to 8th week of gestation
0 NO limbanomalies(importantDDx!)
. extensionof scalp hair growthonto cheek
. microstomia
r/ craniosynostosis
r/ narrowingof retropharyngeal space (apnea,speech
difficulties)
@ Eyes
. antimongoloid eye slant(droopinglaterallower
eyelidsdue to hypoplasiaof lateralcanthaltendonof
orbicularmuscle)
. sparsei absenteye lashes/ colobomain lower lids
 { egg-shapedorbits= droopingof outer inferiororbital r/ arrhinencephaly
r im r/ holoprosencephaly
r/ hypoplasiaof lateralwall of orbits+ shallow/ @ Chest
incompleteorbitalfloor { tnin malformedribs
@ Nose r/ diaphragmatichernia(frequent)
. broad/ protrudednose r/ congenitalheartdisease
r/ choanalshortening Prognosis; deathwithin6 monthsof age
@ Malarbone
r/ sunkencheekdue to markedhypoplasia / agenesis
of zygomaticarches(= malar hypoplasia) TRISOMYE SYNDROME
@ Maxilla = Trisomy
16-18groupsyndrome
r/ hypoplasiaof maxilla+ maxillarysinus Etiology: additionalchromosomeat 18 or E group
./ narrow/ overprojectedmaxilla location
r/ high-arched/ narrowpalate Sex: usuallyfemale
@ Mandible 0 Markedphenotypicvariability!
. retrudedchin, retrognathism . hypertonicspasticinfants
. dentalmalocclusion . mental+ psychomotorretardation
^/ pronouncedmicrognathia = mandibUlar
hypoplasia . typicalfacies: micrognathia,high narrowpalatewith
with broadconcavecurve on lower borderof body small buccalcavity,low-setdeformedears
@ Ear . flexedulnar-deviated fingers+ short adductedthumb
. dysplasticlow-setauricles . 2ndfingeroverlapping of 3rd (CHARACTERISTIC)
. preauricular skintags / fistulas Associatedwith: congenitalheartdiseasein 100%
. conductivehearingloss (common) (PDA,VSD);hernias;renalanomalies;
r/ microtiawith small middleear cavity eventrationof diaphragm
^/ deformed/ fusedi absentauditoryossicles { stippledepiphyses
r/ atresia/ stenosisof externalauditorycanal @ S kul l
O B - US : r/ tnin calvarium
r/ polyhydramnios(fromswallowingdifficulty) { persistentmetopicsuture
Prognosis: early respiratoryproblems(tongue relatively ./ dolichocephaly with prominentocciput
too largefor hypoplastic mandible) { micrognathia due to hypoplasticmandible(most
Rx: surgicalcorrection constantfeature)+ maxilla
DDx: (1) Goldenhar-Gorlin syndrome(unilateralmicrotia @ Chest
+ midfaceanomalies,hemivertebrae, block { increasein AP diameterof thorax
vertebrae,vertebralhypoplasia, microphthalmia, { "shielddeformity"due to hypoplasticshort sternum
colobomaof upperlid) { hypoplastic clavicles(DDx:cleidocranialdysostosis)
(2) Acrofacialdysplasia(limb malformations) r/ t t rin pairswith slenderhypoplastic+ taperedribs
(3) Crouzondisease(maxillaryhypoplasiawith r/ diaphragmaticeventration(common)
protrusionof mandible,hypertelorism, @ Pelvis
exophthalmos,craniosynostosis) { smallpelviswith forwardrotationof iliacwings
r/ increasedobliquityof acetabulum
TRISOMY D SYNDROME r/ acuteiliacangle(DIAGNOSTIC)
= Trisomy
13-15groupsyndrome @ Hand & foot
Etiology: additionalchromosomein D group;high r/ adductedthumb = short 1st metacarpal+ phalanges
maternalage (D TA GN OS TTC )
. severementalretardation { overlapof 2nd on 3rd finger (DIAGNOSTIC)
. hypertonicinfant r/ flexedulnar-deviated fingers
. cleft lip + palate ! short1st toe
Associatedwith: capillaryhemangiomaof face + upper { varus deformitiesof forefoot+ dorsiflexionof toes
trunk { rockerbottomfoot / extremepes planus(frequent)
. hypotelorism OB .U S :
. coloboma,cataract,microphthalmia ./ hydrocephalus
. malformedear with hypoplasticexternalauditorycanal rl cystichygroma
., hyperconvexnails r/ diaphragmatic hernia
r/ postaxialpolydactyly r/ clubfoot
@ S k ull r/ overlappingindexfinger
r/ deficientossificationof skull r/ choroidplexuscyst (30%)
{ cleft/ absentmidlinestructuresof facialbones
r/ poorlyformedorbits Prognosis: child rarelysurvivesbeyond6 monthsof age
r/ slantingof frontalbones DDx: osteogenesisimperfecta,trisomy13 syndrome,
r/ microcephaly Cockaynesyndrome,Werdnig-Hoffmann disease
 Boneand Soft-Tissue
Disorders 169

TUBERCULOSIS
OF BONE DDx: pyogenicarthritis(centralerosionof articular
lncidence: 1-3-5% of tuberculouspatients,30% in cartilage,earlyjoint space narrowing,bony
patientswith extrapulmonary tuberculosis ankylosis)
Age: any, rare in 1styear of life,M:F = 1:1
. negativeskin test excludesdiagnosis
. historyof activepulmonarydisease(in 50%) TuberculousOsteomyel
itis
Location: vertebralcolumn,hip,knee,wrist,elbow lncidence: 16'/" of skeletaltuberculosis
Associatedwith: concurrentactive intrathoracic Age: children<5 years(0.5-14/"), rarein adults
tuberculosis in <50% . painlessswellingof hand/ foot
Pathogenesis: Location: femur,tibia,smallbonesof hand+ foot
1. Hematogenousspreadfrom (mostcommon);any bone may be involved
(a) primaryinfectionof lung (particularly
in children) Site: (a) metaphysis(TYPICALLY)with transphyseal
(b) quiescentprimarypulmonarysite / extraosseous spread(in child)(DDx; pyogenicinfections
focus usuallydo not extendacrossphysis)
2. Reactivation:especiallyin hip (b) epiphysiswith spreadto joint / spreadfrom
adjacentaffectedjoint
(c) diaphysis(<1%)
TuberculousArthritis r/ initiallyroundI ovalpoorlydefinedlyticlesionwith
= joint involvementusuallysecondaryto adjacent minimal/ no surrounding sclerosis
osteomyelitis / hematogenous dissemination { varyingamountsof eburnation+ periostitis
lncidence: 84/" of skeletaltuberculosis r/ advancedepiphysealmaturity/ overgrowth(due to
Pathophysiology: synovitiswith pannus formation hyperemia) t limbshorteningfrom prematurephyseal
leadsto chondronecrosis fusion
Age: middle-agedi elderly ^/ cystic tuberculosis = well-marginated round/ oval
. chronicpain,weakness,musclewasting radiolucent lesionswithvariableamountof sclerosis
. soft-tissue swelling,drainingsinus (a) in children(frequent):in peripheralskeleton,
. jointfluid: highWBC count,low glucoselevel,poor + symmetricdistribution, no sclerosis
mucinclotformation(similarto rheumatoidarthritis) (b) in adults(rare): in skull/ shoulder/ pelvis/ spine,
Loion:
cat gl'3:# with sclerosis
!IB;li'# l#l:i,i ru[ l"Jll,1l]l,, (DDx: eosinophilic granuloma,sarcoidosis, cystic
articulationof hand + foot angiomatosis, plasmacell myeloma,
0 Monoarticular involvement is typical! chordoma,fungalinfections,metastases)
r/ Phemister triad: { tuberculous dactylitis= digitwith exuberantlamellated
1. Gradualnarrowingof jointspacedue to slow / solid periostealnew-boneformationand fusiform
cartilagedestruction(DDx: cartilagedestructionin soft-tissue swelling(children>> adults):
pyogenicarthritisis much quicker) r/ spina ventosa ("wind-filled sail")= ballooning
2. Peripherally located(= marginal)bone erosions dactylitisformingan enlargingcystlikecavitywith
3. Juxtaarticularosteoporosis erosionof endostealcortex (end-stagedisease)
(DDx: fungaldisease,rheumatoidarthritis) DDx: (1) pyogenicosteomyelitis (no transphyseal
Ear,lyradiographs: spread)
r/ joint effusion(hip in OYo,knee in 60%, ankle in (2) syphiliticdactylitis(bilateralsymmetric
80%) involvement,less soft-tissueswellingand
r/ extensiveperiarticularosteopenia(deossification) sequestration)
adjacentto primarilyweight-bearing joints (3) Sarcoidosis,hemoglobinopathies,
{ soft tissuesnormal hyperparathyroidism, leukemia
Late radiographs:
! smallcystlikeerosionsalongjoint marginsin
non-weight-bearing lineopposingone another TuberculousSpondylitis
, (DDx:pyogenicarthritiserodesarticularcartilage) = POTTDISEASE
r/ no jointspacenarrowingfor months(CLASSIC!) Pott(1714-1788),
[Percival surgeonin London, England
ri articularcorticalbone destructionearlierin joints associatedcancerof thescrotum
withcoaltarin chimney
with littleunopposedsurfaces(hip,shoulder) sweepsl
r/ "kissingsequestra"= wedg€-shapedareasof = destructionof vertebralbody + intervertebral
disk by
necrosison both sides of the joint due to infection tuberculousmycobacterium
of subchondralbone lncidence.' <1% of patientswith tuberculosis;
r/ increaseddensitywith extensivesoft-tissue 25-50-60"h ot all skeletaltuberculosis
calcificationsin healingphase Age: children/ adults; M > F
Cx: fibrousankylosis,leg shortening . insidiousonset of back pain, stiffness
Dx: synovialbiopsy(in 90% positive),cultureof . localtenderness
synovialfluid (in 80% positive) . NO pulmonarylesionsin 50%
Location: upperlumbar+ lowerthoracicspine(L1 TUMORALCALCINOSIS
mostcommon);TYPICALLY morethan one = LIPOCALCINOGRANULOMATOSIS
vertebra affected = tdta diseasewith progressivelarge nodular
Site: vertebralbody (82%)with predilectionfor anterior juxtaarticularcalcifiedsoft-tissuemassesin patients
part adjacentto superior/ inferiorendplate>> with normalserumcalcium+ phosphorusand no
posteriorelements(18%) evidenceof renal,metabolic,or collagen-vascular
disease
Spread: Etiology: autosomaldominant(1/3)with variableclinical
(a) hematogenousspreadvia paravertebralvenous expressivity;unknownbiochemicaldefectof
plexusof Batson: separatefoci in 14% phosphorusmetabolismresponsible for
(b) contiguousintodisk by penetrating subchondral abnormalphosphatereabsorption
endplate+ cartilaginous endplate + 1,25-dihydroxy-vitamin D formation
(c) subligamentousspreadbeneathanterior/ Path: multilocular cysticlesionswith creamywhitefluid
posteriorlongitudinalligamentsto adjacent (hydroxyapatite) + many giant cells
vertebralbodies (granulomatous foreignbody reaction)surrounded
by fibrouscapsule
./ collapseof intervertebraldisk space Age: onset mostlywithin 1sII 2nd decade (rangeof
N.B.: vertebraldisk spacemaintainedlongerthan in 1-79 years);M:F = 1:1; predominantly in Blacks
pyogenicarthritis(diskitselfpreservedbut . progressive painful/ painlesssofttissuemasswith
fragmented) overlyingskin ulceration+ sinustractdrainingchalky
r/ demineralization (= resorptionof dense margin)of mi l kl i kefl ui d
vertebralendplates: . swelling
r/ "gougedefect"= mildcontourirregularity of anterior . limitationof motion
and lateralaspectof vertebralbody (= erosionfrom . hyperphosphatemia + hypervitaminosis D
subligamentous extensionof tuberculousabscess) . normalserumcalcium,alkalinephosphatase, renal
r/ reactivesclerosis/ periostealreactionTYPICALLY function,parathyroid hormone
absent @ Soft tissue
r/ collapseof vertebralbody: Location: paraarticular in hips> elbows> shoulders
r/ vertebraplana in children > feet,ribs,ischialspines;single/ multiple
r/ angularkyphoticdeformity(= gibbus)due to joints;ALMOSTNEVERknees;usually
preferentialanteriorinvolvementin adults along extensorsurfaceof joints (? initially
r/ vertebrawithina vertebra(= growth recoverylines) a calcificbursitis)
^/ ivoryvertebra(= reossification as healingresponseto ./ denseloculatedmultiglobular homogeneously
osteonecrosis) calcifiedsofttissue mass of 1-20 cm in size
r/ paraspinalinfection: { radiolucentsepta (= cor'thective tissue)
r/ largecold fusiformabscessin paravertebralgutters ^/ t ttuiO-tluid levelswith milk-of-calcium consistency
/ psoas,commonlybilateralt anterolateral r/ underlyingbonesNORMAL
scallopingof vertebralbodies ./ increasedtraceruptakeof soft-tissuemasseson
r/ amorphous/ teardrop-shaped calcificationin bone scan
paraspinalarea betweenL1 + L5 @ Bone
(DDx: nontuberculous abscessrarelycalcifies) r/ diaphysealperiostealreaction(diaphysitis)
r/ abscessmay extendinto groin/ thigh r/ patcnyareasof calcificationin medullarycavity
(calcificmyelitis)
Cx: angularkyphosis1=gibbusdeformity), scoliosis, @ Teeth
paralysis(spinalcord
ankylosis,osteonecrosis, ./ bulbousrootenlargement
compression from abscess,granulationtissue, r/ pulpstones= intrapulpcalcifications
bone fragments,arachnoiditis) @ Pseudoxanthoma elasticum-like features
r/ calcinosiscutis = skin calcifications
Prognosis: 26-30/" mortality rate ./ vascularcalcifications
{ angioidstreaksof retina
DDx: (1) Pyogenicspondylitis(rapiddestruction, Prognosis; tendencyfor recurrenceafter incomplete
multipleabscesscavities,no thickening/ excision
calcificationof abscessrim, littlenew-bone Rx: phosphatedepletion
formation,posteriorelementsnot involved) DDx: Chronicrenalfailureon hemodialysis, CPPD;
(2) Brucellosis (gaswithindisk,minimal paraosteoarth ropathy,hyperparathyroidism
paraspinalmass,no kyphosis,predilection for
lowerlumbarspine)
(3) Sarcoidosis TURNER
SYNDROME
(4) Neoplasia(multiplenoncontiguous lesions,no = due to nondisjunction
of sex chromosomesas
disk destruction,littlesoft-tissueinvolvement) (1) completemonosomy(45,XO)
 Boneand Soft-Tissue
Disorders 1 7 1

(2) partialmonosomy(structurallyalteredsecondX @ Knee

chromosome) r/ tiOiav€lf€l= enlargedmedialfemoralcondyle
(3) mosaicism(XO + anothersex karyotype) + depressionof medialtibial plateau
lncidence; 1:3,000-5,000livebirths (DDx: Blountdisease)
Associatedwith: coarctation,aortic stenosis, r/ small exostosis-likeprojectionfrom medialborderof
horseshoekidney(mostcommon) proximaltibial metaphysis
. sexualinfantilism (spontaneous pubertyin 5-15%): @ Foot
. primaryamenorrhea r/ deossificationof tarsalbones
. absentsecondarysex characteristics r/ shorteningof 1st,4th,and 5th metatarsals
. short stature;absenceof prepubertalgrowthspurt 1 pes cavus
. webbedneck;low irregularnuchalhair line
. shield-shapedchest + widelyspacednipples US:
. mentaldeficiency(occasionally) r/ prepubertaluterus
. high palate;thyromegaly { nonvisualized/ streakyovaries(in complete
. multiplepigmentednevi;keloidformation monosomy);normalovaries(in mosaickaryotype)
. idiopathichypertension;elevatedurinarygonadotropins OB -U S :
@ General r/ large nuchalcystichygroma
r/ normalskeletalmaturationwith growtharrestat ^/ lymphangiectasia with generalizedhydrops
skeletalage of 15 years ! symmetricaledema of dorsumof feet
r/ delayedfusionof epiphyses> age 20 years ! CHD (20%): coarctationof aorta (70%),leftheart
r/ osteoporosisduring/ after 2nd dLcade(gonadal lesions
hormonedeficiency) r/ horseshoekidney
r/ coarctationof aorta (10%);aorticstenosis
{ renalectopia/ horseshoekidney Bonnevie-Ullrich
Syndrome
r/ lymphedema = infantileform of Turnersyndrome
@ S k ull (1) congenitalwebbedneck
r/ basilarimpression;basal angle>140o (2) widelyseparatednipples
r/ parietalthinning (3) lymphedemaof hands+ feet
r/ smallbridgedsella
r/ hypertelorism
@ Axial skeleton TURRETEXOSTOSIS
^/ hypoplasiaof odontoidprocess+ C1
Cause: traumawith formationof subperiosteal
r/ osteochondrosis of vertebralplates hematoma
r/ squaredlumbarvertebrae;kyphoscoliosis . immobile,occasionally painfullumpon dorsumof finger
r/ deossificationof vertebrae . reducedabilityto flex finger(= ossifiedhematoma
r/ smalliliacwings;latefusionof iliaccrests
diminishesexcursionof extensortendon)
r/ androidpelvicinletwith narrowedpubicarch + small
Location:dorsumof proximal/middlephalanxof hand
sacrosciaticnotches { smoothdome-shapedextracorticalmass
@ Chest
./ thinningof lateralaspectsof clavicles
r/ thinned+ narrowedribs with pseudonotching
VANBUCHEMDISEASE
@ Hand+ arm = GENERALIZED CORTICAL HYPEROSTOSIS
r/ positivemetacarpalsign = relativeshorteningof 3rd = autosomalrecessivedisease;may be relatedto
+ 4th metacarpal
r/ positivecarpalsign = narrowingof scaphoid-lunate- hyperphosphatasemia
Cause: defecton chromosome17
triquetrumangle<117o . paralysisof facial nerve
r/ phalangealpreponderance= lengthof proximal . auditory+ oculardisturbances(in late teens secondary
+ distalphalanxexceedslengthof 4th metacarpalby
to foraminalencroachment)
>3 mm . increasedalkalinephosphatase
r/ shorteningof 2nd + Sthmiddlephalanx(alsoin
Location: skull,mandible,clavicles,ribs,long-bone
Down syndrome)
r/ "drumstick"distalphalanges= slendershaft + large diaphyses
{ symmetrical generalizedsclerosis+ thickeningof
distal head
r/ "insetting"of epiphysesinto bases of adjacent endostealcortex
{ obliterationof diploe
, metaphyses(phalanges+ metacarpals) { spinousprocessesthickened+ sclerotic
r/ Madelungdeformity= shorteningof ulna/ absence
DDx: (1) Osteopetrosis(sclerosisof all bones,not
of ulnarstyloidprocess
confinedto diaphyses)
! cubitusvalgus= bifateralradialtilt of articularsurface
(2) Generalizedhyperostosiswith pachydermia
of trochlea
(involvesentirelongbones,considerable pain,
r/ deossificationof carpalbones
skin changes)
 (3) Hyperphosphatasia (infancy,widenedbones but Age of onset: 7-50 years;hepaticmanifestations
decreasedcorticaldensity) predominate in children;neuropsychiatric
(4) Engelmanndisease(rarelygeneralized, manifestationsPredominatein
involveslowerlimbs) adolescents+ adults
(5) Pyle disease(does not involvemiddiaphyses)
(6) Polyostoticfibrousdysplasia(rarely Histo: macrovesicular fat depositionin hepatocytes,
symmetricallygeneralized,paranasalsinuses glycogendegenerationof hepatocytenuclei,
abnormal,skullinvolvement) Kupffercell hypertroPhy
(7) Sclerosteosis= Truswell-Hansen disease
(syndactylyof 2nd + 3rd fingers,nail dysplasia) Stage 1 asymptomaticcopperaccumulationin
hepatocyticcytosol
Stage 2 redistribution of copperinto hepaticlysosomes
WILLIAMS SYNDROME + circulationfrom saturatedhepatocyticcytosol
= IDIOPATHIC HYPERCALCEMIA OF INFANCY (a) gradualredistribution is asymptomatic
. peculiarelfinlikefacies,dysplasticdentition (b) rapid redistribution causesfulminanthepatic
. neonatalhypercalcemia(not in all patients) failure/ acuteintravascular hemolysis
. mental+ physicalretardation Stage3 cirrhosis,neurologic, ophthalmologic, renal
@ Skeletalmanifestations dysfunctionmay be reversiblewith therapy
r/ osteosclerosis(secondaryto trabecularthickening) . tremor,rigidity,dysarthria,dysphagia(excessivecopper
r/ dense broadzone of provisionalcalcification depositionin lenticularregionof brain)
r/ radiolucentmetaphysealbands . intellectual impairment, emotionaldisturbance
{ dense vertebralend plates+ acetabularroofs . Kayser-Fleischer ring (= greenpigmentation
r/ bone islandsin spongiosa surroundinglimbuscorneae)is DIAGNOSTIC
rl metastaticcalcification . jaundice/ portalhypertension (livercirrhosis)
r/ craniostenosis . elevatedcopperconcentrationin serumceruloplasmin
@ Cardiovascularmanifestations (B E S TS C R E E N IN G TE S T)
r/ supravalvularaorticstenosis(33%),aortic . decreasedincorporationof orallyadministered
hypoplasia radiolabeledcopperinto newlysynthesized
,Vvalvular+ peripheral
pulmonary
arterystenosis ceruloplasmin
^/ ASD,VSD Skeletalmanifestations (in 2i3):
of majorvessels(innominate,
{ stenoses carotids, { generalized deossification may producepathologic
renalarteries) fractures
@ Ot and GU tract: @ Joints: shoulder(frequent), knee,hip,wrist,
r/ colonicdiverticula 2nd-4th MCP joints
./ bladderdiverticula . articularsymptomsin75/": pain,stiffness,gellingof
joints
Prognosis; spontaneousresolutionafter 1 year in most { subarticularcysts
Rx: withholdvitaminD + calcium r/ prematureosteoarthritis(narrowingof joint space
D
DDx: Hypervitaminosis + osteophyteformation)
{ osteochondritis dissecans
r/ chondrocalcinosis
WILSON DISEASE r/ prematureosteoarthrosisof spine,prominent
= HEPATOLENTICULAR DEGENERATION Schmorlnodes,wedgingof vertebrae,irregularities
= autosomalrecessivediseasewith excessivecopper of vertebralplates
retention(= coPPertoxicosis) @ Liver(in children)
Prevalence;1:33,000-200,000;1:90 personsis a r/ normalhepaticattenuation(fattyinfiltration+ copper
heterozygouscarrier depositioncanceleach other out)
r/ normalT1 relaxationtime (in spiteof paramagnetic
Cause: alterationof chromosome13 resultingin inability effectsof copper)
of liverto excretecopperinto bile; @ Brain(adolescents + adults)
hypothetically due to either Location: basalganglia,rarelythalamus
(a) lysosomaldefect in hepatocytes,or { cerebralwhite matteratrophy
(b) deficiencyof biliarycopper-bindingproteins, { hypodensities, prolongation of F1 +T2
or Cx: rickets+ osteomalacia(secondaryto renaltubular
(c) persistenceof fetal mode of copper dysfunction) in minorityof patients
metabolism, or Rx: life-longpharmacologictherapywith chelation
(d) hepaticsynthesisof high-affinitycopper- agents(penicillamine i trientine/ zinc);liver
bindingproteins) transPlantation
 DIFFERENTIAL
DIAGNOSISOF SKULL AND SPINEDISORDERS

LOWBACKPAIN CaudaEquinaSyndrome
Low BackPainin Childhood = constellation
ofsigns+ symptoms
resulting
from
1. Spondylosis,spondylolisthesis compressivelesionin lowerlumbarspinalcanal
2. Osteomyelitis,diskitis Cause:
3. Leukemia (1) displaceddiskfragment
4. Histiocytosis
X (2) intra-/ extramedullarytumor
5. Osteoidosteoma (3) osseous: Pagetdisease,osteomyelitis,
osteoarthrosisof facetjoints,complicationof
LumbosacralPostsurgicalSyndrome ankylosingspondylitis
= FAILED BACKSURGERY SYNDROME . diminishedsensationin lowerlumbar+ sacral
= signsof dysfunctionand disability+ pain and dermatomes
paresthesiafollowingsurgery . wasting+ weaknessof muscles
0 Interpretation in immediatepostoperativeperiod . decreasedankle reflexes
difficult,stabilizationof findingsoccursin 2-6 months . impotence
Frequency: failureof improvementin 5-15% . disturbedsphincterfunction+ overflowincontinence
A. OSSEOUSCAUSES . decreasedsphinctertone
1. Spondylolisthesis
2. Centralstenosis SKULL
3. Foraminalstenosis SuturalAbnormalities
4. Pseudarthrosis Wide Sutures
B. SOFT-TISSUE CAUSES = >10 mm at birth,>3 mm at 2 years,>2 mm at 3
1. Perioperative intraspinal hemorrhage years of age; (suturesare splittableup to age 12-15;
(onset<1 week)
completeclosureby age 30)
2. Residualdisk herniation
A. NORMALVARIANT
(onset<1 week)
in neonate+ prematurity;growthspurt occursat
3. Recurrentdisk herniation 2-3 years and 5-7 years
(onset1 week- 1 month)
B . C ON GE N ITA U L N D E R OS S IFIC A TION
^/ no enhancementon earlyTl Wl (appears
osteogenesisimperfecta,hypophosphatasia,
enhanced>30 minutespost injection) rickets,hypothyroidism, pyknodysostosis,
4. S pinal/m e n i n g e a ln/ e u ra il n fl a m m a ti o/ n
cleidocranial dysplasia
infection
C. METABOL]CDISEASE
(onset1 week- 1 month)
hypoparathyroidism; lead intoxication;hypo-/
5. lntraspinal scar formation(onset>1 month) hypervitaminosis A
(a) Epiduralfibrosis(scarring)
D . R A IS E DIN TR A C R A N IAPLR E S S U R E
r/ enhancingepiduralplique / mass Cause: (1) intracerebraltumor (2) subdural
./ heterogeneousenhancementon early
hematoma(3) hydrocephalus
TlWl (maximumat about5 minutespost Age: seen only if <10 yearsof age
injection) Location: coronal> sagittal> lambdoid>
(b) Fibrosing arachnoiditis = adhesivearachnoiditis
squamosalsuture
r/ thickenedirregularclumpednerve roots E . IN FILTR A TION OF S U TU R E S
r/ adhesionof rootsto wallof thecalsac Cause: metastasesto meningesfrom
r/ abnormalenhancement of thickened (1) neuroblastoma
meninges+ mattednerve roots (2) leukemia
C . S URG I CA LE R R OR S (3) lymphoma
1. Wronglevel/ sideof surgery
r/ poorlydefinedmargins
2. Directnerveinjury
F. RECOVERY
D. Remotephenomenaunrelatedto spine
from (1) deprivational dwarfism
(2) chronicillness
mnemonic.'"ABCDEF"
(3) prematurity
Arachnoiditis (4) hypothyroidism
Bleeding
Contamination (infection) Craniosynosfosrs
Disk (residual/ recurrentlnew level) = CRANIOSTENOSIS = pf€rTl?tUreclosureof sutures
Error (wrongdisk excised) (normallyat about30 years of age)
Fibrosis(scar) Age: oftenpresentat birth; M:F = 4:1
174 RadiologyReview Manual

Etiology: 2. Cerebralatrophyfollowingshuntingof
A. Primarycraniosynostosis hydrocephalus
B. Secondarycraniosynostosis 3. Engelmanndisease: mainlyskullbase
(a) hematologic:sicklecell anemia,thalassemia 4. Hyperparathyroidism
(b) metabolic:rickets,hypercalcemia, 5. Acromegaly
hyperthyroidism, hypervitaminosis D 6. Osteopetrosis
(c) bone dysplasia: hypophosphatasia, B. FOCAL
achondroplasia, metaphysealdysplasia, 1. Meningioma
mongolism,Hurlerdisease,skull 2. FibrousdysPlasia
hyperostosis,Rubinstein-Taybisyndrome 3. Pagetdisease
(d) syndromes: Crouzon,Apert,Carpenter, 4. Dyke-Davidoff-Mason sYndrome
Treacher-Collins,cloverleafskull, 5. Hyperostosisfrontalisinterna
craniotelencephalicdysPlasia, = dense hyperostosisof innertable of frontal
arrhinencephaly bone; M < F
(e) microcephaly:brainatrophy/ dysgenesis
mnemonic; "HIPFAM"
(f) after shuntingprocedures
Hyperostosisfrontalisinterna
Types:
ldiopathic
sagittalsuturemost commonlyaffectedfollowedby
Pagetdisease
coronalsuture
Fibrousdysplasia
1. Scaphocephaly= Dolichocephaly(55%)
thalassemia,
Anemia(sicklecell,irondeficiency,
prematureclosureof sagittalsuture (longskull)
spherocytosis)
2. Brachycephaly= Turricephaly(10%)
prematureclosureof coronal/ lambdoidsutures Metastases
(shorttall skull)
3. Plagiocephaly (7%) Hair-on-end Skull
unilateralearlyfusionof coronal+ lambdoidal mnemonic.' "Hl NEST"
suture(lopsidedskull) Hereditary spherocYtosis
4. Trigonocephaly: prematureclosureof metopic lron deficiencyanemia
suture(forwardpointingskull) Neuroblastoma
5. Oxycephaly: prematureclosureof coronal, Enzymedeficiency(glucose-6-phosphate
sagittal,lambdoidsutures dehydrogenasedeficiencycauseshemolytic
6. Cloverleaf skull = Kleeblattsch6del: anemia)
intrauterineprematureclosureof sagittal, Sicklecell disease
coronal,lambdoidsutures; Thalassemiamajor
May be associatedwith: thanatophoricdysplasia
r/ sharplydefinedthickenedscleroticsuturemargins LeontiasisOssea
r/ delayedgrowthof BPD in early pregnancy = ovefgfowthof facialbonescausingleonine(lionlike)
facies
1. Fibrousdysplasia
WormianBones 2. Pagetdisease
= intrasuturalossiclesin lambdoid,posteriorsagittal, 3. CraniometaphYseal dYsPlasia
temporosquamosal sutures;normalup to 6 monthsof 4. Hyperphosphatasia
age (mostfrequently)
mnemonic: "PORKCHOPS.I" A bnormal l y Thi n S kul l
Pyknodysostosis A . GE N E R A LIZE D
Osteogenesisimperfecta 1. ObstructivehYdrocePhalus
Ricketsin healingphase 2. Cleidocranial dYsostosis
Kinkyhair syndrome 3. Progeria
Cleidocranialdysostosis 4. Rickets
Hypothyroidism / Hypophosphatasia 5. OsteogenesisimPerfecta
Otopalatodigitalsyndrome 6. Craniolacunia
Primaryacroosteolysis(Hajdu-Cheney) / B. FOCAL
Pachydermoperiostosis / Progeria 1. Neurofibromatosis
Syndromeof Down 2. Chronicsubduralhematoma
ldiopathic 3. Arachnoidcyst

Increased Skull Thickness Inadeq uate Calvarial Calcification

A . G E NE RA L IZ E D 1. Achondroplasia
sickle
1. Chronicsevereanemia(eg,thalassemia, 2. OsteogenesisimPerfecta
cell disease) 3. HypophosPhatasia
 Differential Diagnosis of Skull and Spine Disorders 175

Osteolytic Lesion of Skull Multiple Lytic Lesionsin Skull

A. NORMALVARIANT mnemonic.' "BAMMAH"
1. E m is s a ryv e i n Browntumor
connectingvenoussystemsinside+ outsideskull AVM
^/ bony channel<2 mm in width Myeloma
2. Venouslake Metastases
= outpouching of diploicvein Amyloidosis
r/ extremelyvariablein size,shape,and number Histiocytosis
r/ irregularwell-demarcated contour
3. Pacchionian granulations Lytic Area in Bone Flap
r/ usuallymultiplelesionswith irregularcontourin mnemonic.' "RATl"
parasagittallocation(within3 cm of superior Radiationnecrosis
sagittalsinus)primarilyinvolvingthe innertable Avascularnecrosis
Associatedwith: impressionsby arachnoid Tumor
granulations Infection
4. Parietalforamina
nonossification of embryonalrests in parietal Button Sequestrum
fissure;bilateralat superiorposterioranglesof
mnemonic; "TOREME"
parietalbone;hereditarytransmission
Tuberculosis
Osteomyelitis
B. TRAUMA
Radiation
1. S ur gic abl u rrh o l e
granuloma
Eosinophilic
2. Leptomeningeal cyst Metastasis
Epidermoid
C. I NF E CT I O N
1. Osteomyelitis
Absent GreaterSphenoid Wing
2. Hydatiddisease
3. Syphilis
mnemonic.'"M FORMARINE"
4. Tuberculosis Meningioma
Fibrousdysplasia
D. CO NG E NI T A L Opticglioma
1. Epidermoid
Relapsinghematoma
/ dermoid
2. Neurofibromatosis
Metastasis
(asteriondefect)
3. Meningoencephalocele
Aneurysm
4. Fibrousdysplasia
Retinoblastoma
5. Osteoporosiscircumscriptaof paget disease
ldiopathic
Neurofibromatosis
E . B E NI G NT UM O R
granuloma
Eosinophilic
1. Hem ang i o ma
2. Browntumor
3. E os inop h i l g
i cra n u l o ma Absence ol Innominate Line
= OBLIQUE CAROTID LINE
F . M A LI G NA N T U M O R
= verticallineprojectingintoorbit(on PA skullfilm)
1. Solitary/ multiplemetastases
producedby orbitalprocessof sphenoid
2. Multiplemyeloma
A . C ON GE N ITA L
3. Leuk em i a
1. Fibrousdysplasia
4. Neuroblastoma
2. Neurofibromatosis
B . IN FE C TION
Solitary Lytic Lesionin Skull C . TU MOR
mnemonic; "HELP MFT HOLE"
Hem angio ma
Epidermoid/ dermoid
WidenedSuperiorOrbitalFissure
mnemonic.' "A FAN"
Leptomeningealcyst
Aneurysm(internalcarotidartery)
Postop,Pagetdisease
Fistula(cavernoussinus)
Metastasis,Myeloma
Adenoma(pituitary)
Fibrousdysplasia
Neurofibroma
Tuberculosis
Hyperparathyroidism
Osteomyelitis Tumorsof CentralSkull Base
Lambdoiddefect(neurofibromatosis) A. DEVELOPMENTAL
Eosinophilic
granuloma 1. E ncephal ocel e
176 RadiologyReview Manual

B . I NF E CT I O Ni IN F L AMMA T IO N D . OTH E R S
1. Extensionfrom paranasalsinus/ mastoid 1. Seckelsyndrome(bird-headed dwarfism)
infection 2. MultiplepterygiumsYndrome
2. Complication of trauma 3. Pena-Shokeir syndrome
3. Fungaldisease: mucormycosis in diabetics, 4. Beckwith-Wiedemann sYndrome
aspergillosis in immunosuppressed patients 5. Arthrogryposis
4. Sinus+ nasopharyngeal sarcoidosis 6. Skeletaldysplasias
5. Radiationnecrosis 7. Trisomy13, 18,9 (abnormalkaryotypein25/")
C. BENIGN
1. J uv eni l ea n g i o fi b ro m a
2, M ening i o m a Joint
Destructionof Temporomandibular
3. Chor do m a mnemonic.' "HlRT"
4. Pituitarytumor Hyperparathyroidism
5. Pagetdisease Infection
6. Fibrousdysplasia Rheumatoidarthritis
D. MALIGNANT Trauma
1. Metastasis:prostate,lung,breast
2. Chondrosarcoma
3. Nasopharyngeal carcinoma Radiolucent Lesion of Mandible
4. Rhabdomyosarcoma A . S H A R P LYMA R GIN A TE D LE S ION
5. Perineuraltumorspread: head + neckneoplasm (a) aroundapex of tooth
cY st
1. R adi cul ar
GraniofacialSyndromes 2. Cementoma
= developmental of theface+ skull
malformations (b) arounduneruptedtooth
1. Dentigerous cYst
withCNSmalformations
associated
1. Midfacial
clefts 2. Ameloblastoma
(c) unrelatedto tooth
2. Goldenhaarsyndrome
3. Apertsyndrome 1. S i mpl ebonecY st
4. Crouzonsyndrome 2. Fongdisease
5. TreacherCollinssyndrome 3. Basalcell nevussYndrome
B . P OOR LYMA R GIN A TE D LE S ION S
r/ "floatingteeth": suggestiveof primary/ secondary
MAXILLAAND MANDIBLE malignancy
Maxillary Hypoplasia { resorptionof toothroot: hallmarkof benign
1. Downsyndrome process
2. Drugs(alcohol, valproate)
dilantin, (a) infection
3. ApertlCrouzonsyndrome 1. Osteomyelitis:actinomYcosis
4. Achondroplasia (b) radiotherapy
5. Cleftlip/palate 1. Osteoradionecrosis
(c) malignantneoPlasm
1. Osteosarcoma (1/3 lytic,1/3 sclerotic,llS
MandibularHypoplasia= Micrognathia mixed)
A. WITHABNORMAL EARS 2. Localinvasionfrom gingival/ buccal
syndrome
1. Treacher-Collins neoplasms(morecommon)
2. Goldenhar syndrome (hemifacialmicrosomia) 3. Metastasisfrom breast,lung,kidneyin 1% (in
spectrum(x-raysof
= facio-auriculo-vertebral 70oh adenocarcinoma)
(d) other
vertebrae!)
(lUGR,protruding 1. Eosinophilic granuloma:"floatingtooth"
3. Langer-Giedionsyndrome
ears) 2. FibrousdYsPlasia
B. ABNORMALITIES OF EARS+ OTHERORGANS 3. Osteocementoma
1. Millersyndrome(severe postaxialhand 4. Ossifyingfibroma(verycommon)
anomalies)
2. Velo-cardio{acial
syndrome (hand+ cardiac
lesions) Cystic Lesionof Jaw
syndrome
3. Otopalatodigital - typell (hand A. ODONTOGENIC MINERALIZATION
WITHOUT
abnormalities) = mostlybenignlesiondevelopingduringI after
4. Sticklersyndrome(earanomalies notsevere) formationof teeth
(largefleshyears) . asymPtomatic lPain+ swelling
5. Pierre-Robinsyndrome
. paresthesia,tooth displacement/ mobility
C. NOEARANOMALIES
1. Pyknodysostosis { radiolucent
 Differential Diagnosis of Skull and Spine Disorders 177

1. Ameloblastoma= adamantinomaof jaw { root of tooth often outsidelesion

= benignlocallyaggressiveepithelialneoplasm r/ well-definedround/ ovoidcorticatedlucent
Prevalence: 10Y"of odontogenictumors lesion+ mandibularexpansion
Origin: enamel-type epithelialtissueelements Cx: may degenerateinto muralameloblastoma
aroundtooth;30-50% arisefrom (rare)
epitheliumof dentigerous cyst (= mural DDx: unilocularodontogenickeratocyst
ameloblastoma) 4. Radicular cyst = periapical cyst
Age: 3rd-5thdecade;M:F = 1:1 Prevalence: most common cyst of the jaw
. slow-growingpainlessmass Cause: periapicalinflammatorylesion
Location: ramus+ posteriorbody of mandible secondaryto pulpalnecrosisin deep
(75%),maxilla(25%) cariouslesion/ deep fillingi trauma
Site: in regionof bicuspids+ molars (angleof Age: 30-50 years
mandiblecommonly affected) Site: intimatelyassociatedwith apex of nonvital
{ well-defined well-corticatedunilocularlucent tooth
lesion(DDx:odontogenic keratocyst, ri round/ pear-shapedunilocularperiapical
dentigerouscyst) l ucentl esi on,usual l y< 1 cm i n di ame t er
r/ multilocular lesionwith internalseptations ^/ borderedby thin rim of corticalbone
, (honeycomb / soap bubbleappearance) r/ t displacementof adjacentteeth
ri typicallyexpansilewith scallopedmargin ^/ t mitOroot resorption
r/ may perforatethe lingualcortex+ infiltrate DDx: periapicalgranuloma
adjacentsoft tissues
{ often associatedwith the crown of an impacted B . OD ON TOGE N IC W ITHMIN E R A LIZA TION
/ uneruptedtooth = elaborateenamel,dentin,cementum
r/ resorptionof the root of a tooth r/ varyingdegreesof opacity
Prognosis; frequentlylocal recurrenceeven 1. Odontoma
more aggressiveafter excision = odontogenichamartomatous malformation
2. Odontogenic keratocyst Prevalence; most commonodontogenicmass
Origin: dentallamina+ other sourcesof (67%)
odontogenic epithelium Age: 2nd decade
Prevalence: 5-15% of alljaw cysts r/ t-g cm in diameter
Age: 2nd-4th decade r/ may be surroundedby lucentfollicle
Path: daughtercysts + nests of cystic epithelia Types:
in vicinity(highrateof recurrence) (a) compoundodontoma(morecommon)
Histo: parakeratinizedliningepithelium r/ multipleteeth i tooth-likestructures
+ "cheesy"materialin lumenof lesion (b) complexodontoma
Location:body+ ramusof mandible(mostoften); = multiplemassesof dentaltissue
may be anywherein mandible/ maxilla r/ well-defined lesionwith amorphous
r/ unilocularlucentlesionwith smoothcorticated calcifications
border Cx: impaction,malpositioning, resorptionof
r/ often associatedwith impactedtooth adjacentteeth
r/ t undulatingborders/ multilocular appearance DDx: focal cemento-osseous dysplasia,
ri + corticalthinning,toothdisplacement, root ameloblastic f ibro-odontoma. adenomatoid
resorption odontogenic tumor
DDx: indistinguishable from dentigerous cyst/ 2. Odontogenic myxoma
ameloblastoma Prevalence: 3-6% of odontogenictumors
3. Dentigerous cyst = follicular cyst Origin: mesenchymalodontogenictissue
Prevalence: most common type of Age: 10-30 years;M < F
noninflammatoryodontogenic cyst . usuallypainless
Path: epithelial-lined cyst from odontogenic Location: maxilla> mandible
epitheliumdevelopingaroundunerupted r/ well-demarcated / ill-definedlyticlesionof
tooth varyingsize
Histo: formswithinliningof dentalfollicle { oftenmultilocular with honeycomblike internal
Age: adolescent/ young adult structure
. typicallypainfree r/ foci of irregularcalcifications(frequent)
Location: mandible,maxilla(may expandinto Cx: can be locaflyaggressivecausing
maxillarysinus) considerabledestructionof adjacentbone
Site: aroundthe crown of an uneruptedtooth + softtissue infiltration
(usually3rd molar) DDx: malignancy,traumaticbone cyst, central
! cysticexpansilepericoronal lesioncontaining giantcell granuloma,calcifyingepithelial
impactedtooth odontogenic tumor
178 Radiology Review Manual

C. NO NO DO N T O GE N IC ^/ + thinningof mandibularcortex+ osseous

expansion
1. Ossifying fibroma (conventional slow-growing DDx: vascularlesion,centralgiantcell
ossifyingfibroma,juvenileactiveaggressive granuloma,ossifyingfibroma
ossifyingfibroma) 6 . Lingual salivary gland inclusion defect
= €r'rc?psUlated circumscribed benignneoplasm = well-defined depressionin lingualsurfaceof
Histo: highlycellularfibrousconnectivetissue mandible(= Stafnecyst)
containingvaryingamountsof osteoid, Path: aberrantlobe of submandibulargland/ fat
b o n e ,c e me n tu m,
c e m e n tu m l i ke . asymptomatic
calcifiedtissue Location: usuallynear mandibularangle
. asymptomatic Site: just aboveinferiorborderof mandible,
. facialasymmetrydue to bone expansion anteriorto angleof jaw, inferiorto
. toothdisolacement mandibularcanal,posteriorto 3rd molar
^/ initiattylucent+ lateroftenopaquelesion r/ oval / rectangularwell-definedarea of lucency
(dependingon degreeof calcification) ./ bordersurroundedby an opaqueline
r/ surroundedby thin line of lucency(= fibrous .i may extendto buccalcortex
capsule)+ in turn surroundedby thin sclerotic DDx: arteriovenousmalformation
rim of reactivebone 7 . Centralgiant cell granuloma (common)
^/ intensefocal uptakeon bone scan Age: <30 years (75%)
DDx: odontoma,sequestrum, fibrousdysplasia, . painlessswelling,tendernesson palpation
vascularlesion Location: mandible:maxilla = 2:1
2. Focal cemento-osseous dysplasia Site: anteriorto 1st molar(= deciduousteeth);
= rofl I'teoplastic benignfibro-osseous lesion propensityfor crossingmidline(especially
Age: adultlife in maxilla)
. asymptomatic r/ unilocularareaof lucency(early)
Location: mandible>> maxilla r/ multilocular with wispyinternalsepta(later)
r/ one / more,closelyapposed/ confluent,round ./ expansionof bone + displacementof teeth
/ ovoidlucentlesionwith varyingamountsof r/ usuallywell-defined border
opacity DDx: browntumorof HPT (histologically similar)
^/ initiattycysticlucency+ later progressively 8. Brown tumor of hyperparathyroidism
moreopaqueinternally = osteoclastoma = centralgiantcell lesionin
rl no extensioninto adjacentbone patientswith long-standing HPT
r/ no corticalexpansion . hypercalcemia, hypophosphatemia
DDx: periapicalperiodontitis, ossifyingfibroma . elevatedlevelsof parathyroidhormone
3 . Periapicalcemento-osseous dysplasia r/ variablydefinedmargin+ corticalexpansion
(= c€ffientoma)= fibro-osteoma ^/ generalized demineralization of medullary
Age: 30-40 years of age; most commonin bones of jaw
women ^/ loss of laminadura aroundrootsof teeth
. asymptomatic 9. Arteriovenous malformation
Location:in anteriorportionof mandible 0 Tooth extractioncan resultin lethal
Site: at apex of vital tooth exsanguination!
r/ often multicentric . occasionallypulsatilesoft-tissueswelling
r/ mixedlucent+ scleroticlesionwith little Location: ramus+ posteriorbody of mandible
expansion,calcifieswithtime r/ cystlikedue to bone resorption+ calcifications
DDx: ossifyingfibroma,fibrousdysplasia,Paget ^/ + multilocular+ boneexpansion
disease { + erosivemargins
4 . Florid osseous dysplasia r/ angiogramconfirmsdiagnosis
Age: adult life DDx: traumaticbone cyst, centralgiant cell
. asymptomatic granuloma,ossifyingfibroma
r/ diffusemultiquadrant distribution of mixed 10. Mucoepidermoid carcinoma
lucent-opaque osseouschanges
5 . Traumatic bone cyst
= flot a true cyst for lack of epitheliallining Tooth Mass
Cause: ? responseto trauma A. CYSTICLESION
Age: 2nd decade 1. Radicularcyst = periapicalcyst
. asymptomatic 2. Ameloblastoma = adamantinoma of jaw
Location: mandible 3. Giantcell reparativegranuloma
r/ unilocularsharplymarginatedlucentdefect 4. Primordialcyst
r/ scallopedsuperiormarginwith fingerlike arisingfrom follicleof tooth that neverdeveloped
projectionsextendingbetweenrootsof teeth 5. Traumaticbone cyst
 Differential Diagnosis of Skull and Spine Disorders 179

6. Dentigerouscyst = follicularcyst ' limitationin rangeof motionof CVJ

7. Odontogenickeratocyst r/ abnormalcraniometry
B . S CLE RO TICL E SION r/ C-spine+ foramenmagnumbulgeintocranial
1. Cem en to m a cavity
2. True cementoma= benigncementoblastoma r/ elevationof posteriorarch of C1
3. Gigantiform cementoma
4. Hypercementosis
= bulbousenlargement of a root Basilar Impression
(a) idiopathic = acquiredform of basilarinvagination with bulgingof
(b) associatedwith Pagetdisease C-spineand foramenmagnumintocranialcavity
5. Benignfibro-osseous lesions r/ tip of odontoidprocessprojects>5 mm above
(a) ossifyingfibroma:youngadults; Chamberlainline(= linebetweenhardpalate
mandible> maxilla + opisthion)
(b) monostoticfibrousdysplasia:M < F, younger Cause: Pagetdisease,osteomalacia,rickets,
patients fibrousdysplasia,hyperparathyroidism,
(c) condensingosteitis= focal chronicsclerosing Hurlersyndrome,osteogenesis
osteitis imperfecta,skull base infection
r/ near apex of nonvitaltooth mnemonic.' "PF ROACH"
6. Pagetdisease Pagetdisease
involvement of jaw in 20h; maxilla> mandible Fibrousdysplasia
Location: bilateral,symmetricinvolvement Rickets
r/ widenedalveolarridges Osteogenesisimperfecta,Osteomalacia
r/ ttat palate Achondroplasia
r/ looseningof teeth Cleidocranial
dysplasia
r/ hypercementosis Hyperparathyroidism, Hurlersyndrome
{ may causedestruction of laminadura
7. Torus mandibularis = exostosis
Site: midlineof hardpalate;lingualsurfaceof Platybasia
mandiblein regionof bicuspids = anthropometricterm referringto flatteningof skull base
May be associatedwith: basilar invagination
CRANIOVERTEBRAL JUNCTION . cord symptoms
CraniovertebralJunctionAnomaly = clivus-canalangle becomesacute
r/ craniovertebral
Basilar lnvagination (< 150" )
= primarydevelopmental anomalywith abnormally ^/ Welcherbasalangle= sphenoidangle>140o
highpositionof vertebralcolumnprolapsinginto r/ bowstringdeformityof cervicomeduLlary junction
skull base
Associatedwith: Chiarimalformation,
syringohydromyeIia in 25-35/" ATLAS AND AXIS
Cause: AtlasAnomalies
1. Condylustertius= ossicleat distalend of clivus A . P OS TE R IOR A R C HA N OMA LIE S
r/ pseudojointwith odontoidprocessI anterior 1. Posterioratlasarch rachischisis(4%)
archof C1 Location: midline(97"/"),lateralthroughsulcus
2. Condylarhypoplasia of vertebralartery(3%)
r/ lateralmassesof atlas may be fused to r/ absenceof arch-canalline (LAT view)
condyles r/ superimposedon odontoidprocess/ axis body
r/ violationof Chamberlain line simulatinga fracture(open-mouth odontoid
r/ wideningof atlantooccipitaljoint
axis angle view)
r/ tip of odontoid>10 mm abovebimastoidline 2. Total aplasiaof posterioratlas arch
3. Basiocciputhypoplasia 3. Keller-typeaplasiawith persistenceof posterior
r/ shorteningof clivus tubercle
r/ violationof Chamberlain line 4. Aplasiawith uni-/ bilateralremnant+ midline
{ clivus-canalangletypicallydecreased rachischisis
4. Atlantooccipitalassimilation 5. Partial/ total hemiaplasiaof posteriorarch
= cornplete/ partialfailureof segmentation B . A N TE R IORA R C HA N OMA LIE S
betweenskull + 1st cervicalvertebra 1. lsolatedanteriorarch rachischisis(0.1%)
r/ violationof Chamberlainline 2. Splitatlas= anterior+ posteriorarch rachischisis
r/ clivus-canalangle decreased { plumproundedanteriorarchoverlapping the
May be associatedwith: fusion of C2 + C3 odontoidprocessmakingidentification of
Cx: atlantoaxialsubluxation(50%); predentalspace impossible(LAT view)
suddendeath ri duplicatedanteriormargins(LAT view)
180 Radiology Review Manual

.(p"
secondarycenters
(b) Arthritis
due to laxityof transverseligamentor erosionof
dens
1. Rheumatoidarthritis
2. Psoriaticarthritis
3. Reitersyndrome
4. AnkylosingsPondYlitis
5. S LE
Axis Atlas rare: in gout + CPPD
(c) Inflammatoryprocess
Primary and Secondary Ossification Centers Pharyngealinfectionin childhood,retropharyngeal
abscess,coryza,otitismedia,mastoiditis,cervical
adenitis,parotitis,alveolarabscess
./ dislocation8-10 days afteronset of symptoms
Axis Anomalies (d) Trauma (very rare withoutodontoidfracture)
1. Persistent = Bergman
terminale
ossiculum ossicle (e) Marfandisease
r/ unfusedodontoidprocess>12 years of age
DDx: type 1 odontoidfracture mnemonic.' "JAP LARD"
2. Odontoidaplasia(extremelyrare) Juvenilerheumatoidarthritis
3. Os odontoideum AnkylosingspondYlitis
= independentos cephaladto axis body in location Psoriaticarthritis
of odontoidprocess Lupus erythematosus
r/ absenceof odontoidprocess Accident(trauma)
{ anteriorarch of atlas hypertrophic+ situatedtoo Retropharyngeal abscess,Rheumatoidarthritis
far posteriorin relationto axis body Down syndrome
Cx: . atlantoaxialinstability
DDx: type 2 odontoidfracture(uncorticated PSEUDOSUBLUXATION
margin) = ligamentouslaxityin infantsallowsfor movementof
the vertebralbodieson each other,esp. C2 on C3

Odontoid Erosion SPINAL DYSRAPHISM

mnemonic.' "P LARD" = abnormal/incompletefusionof midlineembryologic
Psoriasis mesenchymal, bony structures
neurologic,
Lupus erythematosus Externalsigns(in 50%):
. subcutaneous lipoma . spasticgait disturbance
Ankylosingspondylitis
. hypertrichosis . foot deformities
Rheumatoidarthritis
. pigmentednevi . absenttendonreflexes
Down syndrome
. skindimple . sinus tract
. bladder+ boweldYsfunction
. pathologicplantarresponse
AtlantoaxialSubluxation
= displacementof atlaswith respectto axis
(1) Posterioratlantoaxialsubluxation(rare) Spina Bifida
(2) Anterioratlantoaxialsubluxation(common) = incomplete of thespine
closureof bonyelements
= distancebetweendens + anteriorarch of C1 (lamina+ spinousprocesses)posteriorly
(measurementalong midplaneof atlason lateral
view): Spina Bifida Occulta
(a) predentalspace: >2.5 mm; = OCCULT SPINAL DYSRAPHISM
>4.5 mm (in children) = cl€ft/ tetheredcord WITH skin cover
(b) retrodental
space: <18 mm Frequency: 15ohof spinaldysraphism
. rarelyleadsto neurologicdeficitin itself
Causes of subluxation: Associated with:
(a) Congenital (a) vertebraldefect (85 - 90%)
of atlas
1. Occipitalization (b) lumbosacral dermallesion(80%): hairytuft
0.75%of population; fusionof basion+ anterior dimple,sinustract,nevus,
(= hypertrichosis),
arch of atlas hyperpigmentation, hemangioma, subcutaneous
2. Congenitalinsufficiencyof transverseligament MASS
3. Os odontoideum i aplasiaof dens 1. DiastematomYelia
4. Down syndrome(20%\ 2. Lipomeningocele
5. Morquiosyndrome 3. Tetheredcord sYndrome
6. Bonedysplasia 4. Fi l umtermi nal el i P oma
 DifferentialDiagnosisof Skulland SpineDisorders 1 8 1

5. Intraspinaldermoid 1. Asomia = ?geh€sisof vertebralbody

6. Epidermoidcyst ! completeabsenceof vertebralbody
7. Myelocystocele ./ hypoplasticposteriorelementsmay be present
8. Split notochordsyndrome 2. Hemivertebra
9. Meningocele (a) Unilateralwedge vertebra
10. Dorsaldermalsinus r/ right/ left hemivertebra
11. Tightfilumterminalesyndrome { scoliosisat birth
(b) Dorsalhemivertebra
Spina Bifida Aperta r/ rapidlyprogressivekyphoscoliosis
= SPINABIFIDA CYSTICA (c) Ventralhemivertebra(extremelyrare)
= posteriorprotrusionof all / parts of the contentsof 3. Goronal cleft
the spinalcanalthrougha bony spinaldefect = failureof fusionof anterior+ posteriorossification
Frequency: 85% of spinal dysraphism centers
0 Most severeform of midlinefusiondefect May be associatedwith: prematuremale infant,
. neuralplacodeWITHOUTskincover
Chondrodystrophia
. associatedwith neurologicdeficitin >90%
calcificanscongenita
1. S im plem e n i n g o c e l e Location: usuallyin lowerthoracic+ lumbarspine
= herniationof CSF-filledsac withoutneural r/ verticalradiolucentband just behindmidportionof
elements vertebralbody;disappearsmostlyby 6 monthsof
2. Myelocele life
= midlineplaqueof neuraltissuelyingexposedat 4. Butterfly vertebra
the skin surface = failureof fusionof lateralhalvessecondaryto
3. Myelomeningocele persistenceof notochordaltissue
= d rny€loceleelevatedaboveskin surfaceby May be associatedwith: anteriorspina bifida
expansionof subarachnoidspace ventralto + anteriormeningocele
neuralplaque r/ widened vertebralbody with butterflyconfiguration
4. Myeloschisis (AP view)
= surfacepresentationof neuralelements r/ adaptationof vertebralendplatesof adjacent
completelyuncoveredby meninges vertebralbodies
5. Block vertebra
Caudal SpinalAnomalies = coflgeflitalvertebralfusion
= malformationof distalspine and cord in associated Location: lumbar/ cervical
with hindgut,renal,and genitourinaryanomalies ./ neigfrtof fused vertebralbodiesequalsthe sum of
1. Terminalmyelocystocele heightsof involvedbodies+ intervertebral disk
2. Lateralmeningocele r/ "waist"at level of intervertebraldisk space
3. Caudalregression 6. Hypoplasticvertebra
7. Klippel-Feil syndrome
SegmentationAnomaliesof VertebralBodies
duringI - 12thweek of gestationtwo ossification
centersform for the ventral+ dorsalhalf of vertebral VERTEBRAL BODY
body Destructionof VertebralBody
A. NEOPLASM
1. Metastasis
2. Primaryneoplasm:lymphoma,multiple
persistent neurocentral myeloma,chordoma
synchondrosis
B . IN FE C TION
1. Pyogenicvertebralosteomyelitis
z/ pedicular cleft =
2. Tuberculous spondylitis
retrosomatic cleft
3. Brucellosis
4. Fungaldisease
5. Echinococcosis
6. Sarcoidosis

SmallVertebralBody
1. Radiationtherapy
retroisthmic cleft duringearlychildhoodin excessof 1,000rads
2. Juvenilerheumatoidarthritis
spinous cleft
Location: cervicalspine
r/ atlantoaxialsubluxationmay be present
Clefts in Neural Arch r/ vertebralfusionmay occur
182 Radiology Review Manual

3. E os inophi l g
i cra n u l o ma VertebralBorderAbnormality
Location: lumbar/ lowerthoracicspine Straighteningof Anterior Border
r/ compressiondeformity/ vertebraplana 1. Ankylosing
spondylitis
4. Gaucherdisease 2. Pagetdisease
= depositsof glucocerebrosides within RES 3. Psoriaticarthritis
{ compression deformity 4. Reiterdisease
5. Platyspondylygeneralisata 5. Rheumatoidarthritis
= flattenedvertebralbodiesassociatedwith many 6. Normalvariant
hereditarysystemicdisorders (achondroplasia,
spondyloepiphyseal dysplasiatarda, Anterior Scalloping of Vertebrae
mucopolysaccharidosis, osteopetrosis, 1. Aorticaneurysm
neurofibromatosis, osteogenesisimperfecta, 2. Lymphadenopathy
thanatophoricdwarfism) 3. Tuberculosis
r/ disf spacesof normalheight 4. Multiplemyeloma(paravertebral
softtissue mass)

VertebraPlana
mnemonic; " FETISH" Posterior Scalloping of Vertebrae
Fracture(trauma,osteogenesisimperfecta) in conditionsassociatedwith dural ectasia
Eosinophilicgranuloma IN TR A S P IN APLR E S S U RE
A . IN C R E A S E D
Tumor (metastasis,myeloma,leukemia) 1. Communicatinghydrocephalus
Infection 2. Ependymoma
Steroids(avascularnecrosis) B. MESENCHYMAL TISSUELAXITY(duralectasia)
Hemangioma 1. Neurofibromatosis
2. Marfansyndrome
3. Ehlers-Danlos syndrome
Signs of Acute VertebralCollapseon MRI 4. Posteriormeningocele
1. O S T E O P O R O S IS C . B ON ES OFTE N IN G
{ retropulsionof posteriorbone fragment 1. Mucopolysaccharidoses: Hurler,Morquio,
2. MALIGNANCY Sanfilippo
r/ epiduralsoft-tissuemass 2. Achondroplasia
r/ no residualnormalmarrowsignalintensity 3. Acromegaly(lumbarvertebrae)
^/ abnormalenhancement 4. Ankylosingspondylitis(lax dura actingon
osteoporoticvertebrae)
EnlargedVertebralBody
1. Pagetdisease mnemonic: "DAMN MALE SHAME"
r/ "pictureframing";bonesclerosis Dermoid
2. Gigantism Ankylosingspondylitis
r/ increasein heightof body + disk Meni ngi oma
3. Myositisossificansprogressiva Neurofibromatosis
{ bodiesgreaterin heightthanwidth
r/ osteoporosis Marfansyndrome
of ligamentumnuchae
r/ ossification Acromegaly
Lipoma
EnlargedVertebralForamen Ependymoma
1. Neurofibroma
2. Congenitalabsence/ hypoplasiaof pedicle Syringohydromyelia
3. Duralectasia(Marfansyndrome,Ehlers-Danlos Hydrocephalus
syndrome) Achondroplasia
4. Intraspinal
neoplasm Mucopolysaccharidoses
5. Metastaticdestructionof pedicle syndrome
Ehlers-Danlos

CervicalSpineFusion
mnemonic.' "SPAR BlT" Bony Outgrowthsfrom Vertebra
Senilehypertrophicankylosis(DISH) A . C H ILD H OOD
Psoriasis,Progressivemyositisossificans 1. Hurlersyndrome= gargoylism
Ankylosingspondylitis r/ roundedappearanceof vertebralbodies
Reiterdisease,Rheumatoidarthritis(juvenile) { mild kyphoticcurve with smallervertebralbody
Blockvertebra (Klippel-Feil) at apex of kyphosisdisplayingtonguelikebeak
Infection(TB) at anteriorhalf(usuallyat T12 I L1)
Trauma { "step-off"deformitiesalong anteriormargins
 Differential Diagnosis of Skull and Spine Disorders 183

2 . Huntersyndrome
lessseverechangesthan in Hurlersyndrome
3 . Morquiodisease
r/ flattened+ widenedvertebralbodies
{ anterior"tonguelike" elongationof central
www Normal Wedge Vertebra Fish Vertebra

www
portionof vertebralbodies
4 . Hypothyroidism = cretinism
r/ smallflat vertebralbodies
r/ anterior"tonguelike" deformity(in childrenonly)
r/ wideneddisk spaces+ irregularendplates
B. ADULTS Pancake Vertebra H Vertebra Schmorl Node
1. Spondylosis deformans
r/ osteophytosisalong anterior+ lateralaspects Vertebral Endplate Abnormalities
of endplateswith horizontal+ verticalcourse
as a resultof shearingof the outerannular 2. Osteophyte
fibers(Sharpeyfibersconnectingthe annulus = ossification of anteriorlongitudinal
ligament
fibrosusto adjacentvertebralbody) ^/ initiattytriangularoutgrowthseveralmillimeters
2. Diffuseidiopathicskeletalhyperostosis (DISH) from edge of vertebralbody
r/ flowingcalcifications+ ossificationsalong Associatedwith: osteoarthritis
anterolateralaspectol >4 contiguousthoracic 3. Flowinganteriorossification
vertebralbodies+ osteophytosis = ossification of disk,anteriorlongitudinal
3. Ankylosingspondylitis ligament,paravertebralsoft tissues
./ bilateralsymmetricsyndesmophytes Associatedwith: diffuseidiopathicskeletal
(ossificationof annulusfibrosus) hyperostosis
r/ "bamboospine" 4. Paravertebralossification
r/ "diskalballooning"= biconvexintervertebral ^i initiattyirregular/ poorlydefinedparavertebral
disks secondaryto osteoporoticdeformityof ossification eventuallymergingwith vertebral
endplates body
r/ straighteningof anteriormarginsof vertebral Associatedwith: psoriaticarthritis,Reiter
bodies(erosion) syndrome
r/ ossificationof paraspinalligaments
4. Fluorosis VertebralEndplateAbnormality
r/ vertebralosteophytosis+ hyperostosis 1. Cupid'sbow vertebra
r/ scleroticvertebralbodies+ kyphoscoliosis Cause: ? (normalvariant)
r/ calcificationof paraspinalligaments Location: 3-5th lumbarvertebra
5. Acromegaly r/ two parasagittalposteriorconcavitieson inferior
^/ increasein anteroposterior diameterof aspectof vertebralbody (viewedon AP)
vertebrae+ concavityon posteriorportion 2. Osteoporosis (senile/ steroid-induced)
r/ enlargement of intervertebral
disk (a) "fish/ fish-mouthvertebrae"
6. Hypoparathyroidism Cause: osteomalacia,Pagetdisease,
7. Neuropathicarthropathy hyperparathyroidism, Gaucherdisease
8. Sternoclavicular hyperostosis r/ biconcavevertebrae
r/ bone sclerosisalongendplates
Spine Ossification (b) wedge-shapedvertebrae
1. Syndesmophyte = ossification
of annulusfibrosus ri anteriorborderheightreducedby >4 mm
! thin slenderverticaloutgrowthextendingfrom comparedto posteriorborderheight
marginof one vertebralbody to next (c) "pancake"vertebrae
Associatedwith: ankylosingspondylitis, r/ overallflatteningof vertebra
ochronosis 3 . "H-vertebrae"
Cause: sicklecell + otheranemias,Gaucher
disease

WWW
= coffiPressionof centralportionsfrom subchondral
infarcts
4 . Schmorl/ cartilaginous node
= intraosseous herniationof nucleuspulposusat
centerof weakenedendplate
Ankylosing Psoriasis Spondylosis C ause: S cheuermanndi sease,traum a,
Spondylitis Reiter Syndrome Deformans hyperparathyroidism, osteochondrosis
5 . Butterflyvertebra
Syndesmophytes Cause: congenitaldefect
184 Radiology Review Manual

6. Limbusvertebrae Sclerotic Pedicle

= intraosseous herniationof disk materialat junction 1. Osteoidosteoma
of vertebralbony rim of centra+ endplate 2. Unilateralspondylolysis
(anterosuperior corner) congenitally
3. Contralateral absentpedicle
7. " Ring"epip h y s i s
8. "Rugger-jerseyspine"
Cause: hyperparathyroidism, myelofibrosis TUMORS OF VERTEBRA
r/ horizontalsclerosissubjacentto vertebral ExpansileLesionof Vertebrae
endplateswith intervening normalosseousdensity A. INVOLVEMENT OFMULTIPLE VERTEBRAE
(resemblingthe stripeson rugbyjerseys) multiplemyeloma/ plasmacytoma,
Metastases,
9. "Sandwich"/ "hamburger"vertebrae lymphoma,hemangioma,Pagetdisease,
Cause: osteopetrosis,myelofibrosis granuloma
eosinophilic
angiosarcoma,
r/ scleroticendplatesalternatewith radiolucent
midportionsof vertebralbodies B . IN V OLV E ME NOFT TW O i MOR EC ON TI G UO US
V E R TE B R A E
Ring Epiphysis Osteochondroma, chordoma,aneurysmalbonecyst,
= rrorffi?laspectof developingvertebra(between6 myeloma
and l2y ea rs o fa g e )
r/ small steplikerecessat cornerof anterioredge of C . B E N IGNLE S ION
vertebralbody 1. Osteochondroma (1-5% with solitary
1. Severeosteoporosis osteochondromas, 7-9% with hereditarymultiple
2. Healingrickets exostoses)commonlycervical,esp.C2;
3. Scurvy commonlyrisingfrom posteriorelements
2. Osteoblastoma(30-40% in spine)
M:F = 2:1;equaldi stri buti on
i n spi ne;p ost er ior
Bullet-shapedVertebralBody elements(lamina,pedicle),may involvebody if
mnemonic.' "HAM" large;expansilelesionwith sclerotic/ shell-like
Hypothyroidism rim,foci of calcifiedtumormatrixin 50%
Achondroplasia 3. Giantcelltumor(5-7% in spine)
Morquiosyndrome commonlysacrum,expansilelyticlesionof
vertebralbody with well-definedborders;
secondaryinvasionof posteriorelements;
Vertebra
Bone-within-bone malignantdegeneration in 5-20/0 afterradiation
- "ghostvertebra"followingstressfuleventduring therapy
vertebralgrowthphase in childhood 4. Osteoidosteoma(10-25% in spine)
1. Stresslineof unknowncause commonlylowerthoracic/ upperlumbarspine,
2. Leuk em ia posteriorelements(pedicle,lamina,spinous
3. Heavymetalpoisoning process),painfulscoliosiswith concavitytoward
4. Thorotrastinjection,TB lesion
5. Rickets 5. Aneurysmalbonecyst (12-30/" in spine)
6. Scurvy thoracic> lumbar> cervicalspine,posterior
7. Hypothyroidism elementswith frequentextensioninto vertebral
8. Hypoparathyroidism bodies,well-defined margins,may arisefrom
primarybone lesion(giantcelltumor,fibrous
dysplasia)in 507o,may involvetwo contiguous
lvory Vertebra vertebrae
mnemonic; "LOST FROM CHOMP" 6. Hemangioma(30% in spine)
Lymphoma 10% incidencein generalpopulation; commonly
Osteopetrosis lowerthoracic/ upper lumbarspine,vertebral
"corduroy"appearance
Sicklecell disease body, "accordion"/
Trauma,Tuberculousspondylitis 7 . Hydatidcyst (1% in spine)
Fluorosis slow-growing destructivelesion,well-defined
Renalosteodystrophy scleroticborders,endemic areas
Osteoblasticmetastasis 8. Pagetdisease
Myelosclerosis vertebralbodyt posteriorelements,enlargement
Chronicsclerosingosteomyelitis,
Chordoma of bone,"pictureframing";bonesclerosis
Hem angio ma 9. Eosinophilic granuloma(6% in spine)
Osteosarcoma most oftencervical/lumbarspine,vertebral
Myeloma body,"vertebraplana";multipleinvolvement
Pagetdisease common
 Differential Diagnosis of Skull and Spine Disorders 185

10.F ibr ousd y s p l a s i a(1 % i n s p i n e ) Primary Vertebral Tumors in Children

vertebralbody, nonhomogeneous trabecular in orderof frequency:
"ground-glass"appearance
1. Osteoidosteoma
11.E nos t o s i (1
s -14 /" i n s p i n e ) 2. Benignosteoblastoma
Location: T1-T7 > L2-L3 3. Aneurysmalbone cyst
4. Ewingsarcoma
D. M A LI G NAN T
1. Chordoma(15o/"in spine)
mostcommonnonlymphoproliferative primary PrimaryTumorof PosteriorElements
malignanttumorof the spinein adults; mnemonic.' "A HOG"
particularlyC2, withinvertebralbody;violates Aneurysmalbone cyst
disk space Hydatidcyst,Hemangioma
2. Metastases(especiallyfrom lung, breast) Osteoblastoma,Osteoidosteoma
Age: >50 years of age; Giantcelltumor
Clue: pediclesoften destroyed
3. Multiplemyeloma/ plasmacytoma
Clue: vertebralpediclesusuallyspared
4. Angiosarcoma SACRUM
10% involvespine,mostcommonlylumbar DestructiveSacralLesion
5. Chondrosarcoma (3-12% in spine) mnemonic.' "SPACEMON"
2nd mostcommonnonlymphoproliferative Sarcoma
primarymalignanttumorof the spinein adults Plasmacytoma
Site: vertebralbody (15%),posteriorelements Aneurysmalbone cyst
(40/"), both (45%) Chordoma
{ involvementof adjacentvertebraby extension Ependymoma
throughdisk (35%) Metastasis
6. Ewingsarcomaand PNET Osteomyelitis
most common nonlymphoproliferative primary Neuroblastoma
malignanttumorof the spinein children;
metastasesmore commonthan primary
Site: vertebralbody with extensionto posterior SacralTumor
elements Sacral Bone Tumor
ri diffusesclerosis+ osteonecrosis(Og%) A. BENIGN
7. Osteosarcoma (0.6-3.2%in spine) 1. Giantcelltumor(2nd mostcommonprimary)
Average age: 4th decade 2. Aneurysmalbone cyst (rare)
Location: lumbosacral segments 3. Cavernoushemangioma(veryrare)
Site: vertebralbody,posteriorelements(10-17%) 4. Osteoidosteomai osteoblastoma(very rare)
r/ may presentas "ivoryvertebra"
8. Lymphoma B. MALIGNANT
1. Metastases(mostcommonsacralneoplasm):
hematogenous:lung,breast,kidney,
Blowout Lesion of Posterior Elements prostate
mnemonic.' "GO APE" contiguous:rectum,uterus,bladder
G iantc elltu mo r 2 . Plasmacytoma, multiplemyeloma
Osteoblastoma 3 . Lymphoma,leukemia
Aneurysmalbone cyst 4 . Chordoma(mostcommonprimary)
Plasmacytoma 0 2-4% of malignantosseousneoplasmsl
Eosinophilic granuloma 5 . Sacrococcygealteratoma
6 . Ewingsarcoma(rare)

BoneTumorsFavoringVertebralBodies
mnemonic.' "CALL HOME" Sacral Canal Tumor (less common)
Chordoma A. BENIGN
Aneurysmalbonecyst 1. Neurofibroma:multiplesuggestiveof NF
Leukemia 2. Schwannoma(rare)
Lymphoma 3. Meningioma(veryrare)
Hem angiom a B . MA LIGN A N T
Osteoidosteoma,Osteoblastoma 1. E pendymoma
Myeloma,Metastasis 2. Drop metastases
E os inophiligcra n u l o m a 3. Carcinoidtumor
186 RadiologyReviewManual

INTERVERTEBRAL DISK Schmorl= Cartilaginous

Node
Loss of Disk Space = superior/ inferiorintravertebralherniationof disk
1. Degenerativedisk disease materialthroughweakenedarea of vertebralendplate
2. Neuropathicosteoarthropathy Pathogenesis: disruptionof cartilaginousplate of
3. Dialysisspondyloarthropathy with amyloidosis vertebralbody left duringregressionof
4. Ochronosis chordadorsalis,ossitication gaps,
5. Ankylosingspondylitiswith pseudarthrosis previousvascularchannels
6. Sarcoidosis Cause:
(a) osseous: osteoporosis,osteomalacia,Paget
disease,hyperparathyroidism, infection,neoplasm
SpinalVacuumPhenomena (b) cartilaginous:intervertebral
osteochondrosis, disk
(a)nucleuspulposus Osteochondrosis juvenilekyphosis
infection,
(b)annulusfibrosus Spondylosis deformans r/ concavedefectsat upper and lowervertebral
(c) disk withinvertebralbody Cartilaginousnode endplateswith sharpmargins
(d) diskwithinspinalcanal Intraspinal
disk herniation MR:
(e) apophysealjoint Osteoarthritis r/ nodeof similarsignalintensityas disk
(f) vertebralbody lschemic necrosis ^/ low signalintensityof rim
r/ associatedwith narroweddisk space
VacuumPhenomenonin Intervertebral Disk DDx: mnemonic.' "SHOOT"
Space Scheuermann disease
= liberationof nitrogengas from surrounding Hyperparathyroidism
tissues
intocleftswith an abnormalnucleusor annulus Osteoporosis
attachment Osteomalacia
Prevalence: in up to 20'/" of plain radiographs/ in Trauma
up to SOh of spinalCT in patients>
age 40
Cause: S P IN A L C OR D
1. Primary/ secondarydegeneration of nucleus 0 Mostspinalcord neoplasmsare malignantl
pulposus 0 90-95% are classifiedas gliomas
2. Intraosseous of disk (= Schmorlnode)
herniation
3. Spondylosis deformans(gasin annulusfibrosus)
4. Adjacentvertebralmetastaticdiseasewith Intramedullary
Lesion
vertebralcollapse Prevalence: 4-10% of all CNS tumors;20/" of all
5. Infection(extremelyrare) tumorsin adults(35%in
intraspinal
children)

IntervertebralDisk Calcification A . TU MOR

mnemonic.' " A DISK SO WHITE" r/ expansionof cord
Amyloidosis,Acromegaly d heterogenoussignalon T2Wl
Degenerative r/ cysts + necrosis
Infection ./ variableenhancement(vast majoritywith some
Spinalfusion enhancement)
CPPD (a) primary:
Spondylitisankylosing 1. Ependymoma(60%of all spinalcordtumors)
Ochronosis 0 The mostcommonglialtumorin adults
Wilsondisease 2. Astrocytoma(25%)
Hemochromatosis,Homocystinuria, 0 The mostcommonintramedullary tumorin
Hyperparathyroidism children
ldiopathicskeletalhyperostosis 3. Hemangioblastoma (5%)
Traumatic 4. Oligodendroglioma (3%)
Etceteras:Gout and othercausesof 5. Epidermoid, dermoid,teratoma(1-2%)
chondrocalcinosis (1% )
6. Gangl i ogl i oma
7. Li poma(1% )
IntervertebralDisk Ossification Location: cervicalregion: astrocytoma
Associatedwith: fusion of vertebralbodies thoracicregion:teratoma-
1. Ankylosingspondylitis dermoid,astrocytoma
2. Ochronosis lumbarregion: ependymoma,
3. Sequelaeof trauma dermoid
4. Sequelaeof disk-spaceinfection (b) metastatic:eg, malignantmelanoma,breast,
5. Degenerativedisease l ung
 Differential Diagnosis of Skull and Spine Disorders 187

B. CYSTICLESION lntramedullaryNonneoplasticMass
r/ ttuiOisointenseto CSF 1. E pi dermoi d
r/ smoothwell-defined internalmargins 2. Congenitallipoma
r/ thinnedadjacentparenchyma 3. Posttraumaticpseudocyst
r/ cord atrophy 4. Wegenergranuloma
r/ no contrastenhancement 5. Cavernousmalformation
(a) peritumoralcyst = syringomyelia 6. Abscess
r/ polari satellitecysts = fostral/ caudalcysts
representingreactivedilatationof centralcanal IntramedullaryNonneoplasticLesion
0 A higherlocationwithinspinalcanalraisesthe Prevalence: 4%
likelihoodof syrinxdevelopment r/ no cord expansion
Prevalence: in 60% of all intramedullary tumors 1. Demyelinating disease
1. S y r in g o my e l i a 2. Sarcoidosis
2. Hydromyelia 3. Amyloidangiopathy
3. Reactivecyst 4. Pseudotumor
(b) tumoralcyst 5. Duralarteriovenousfistula
r/ shows peripheralenhancement 6. Cord infarction
1. Ganglioglioma (in 46%) 7. Chronicarachnoiditis
2. Astrocytoma(in 20%) 8. Cysticmyelomalacia
3. Ependymoma(in 3%)
4. Hemangioblastoma (24%) IntraduralExtramedullary
Mass
1. Nervesheathtumor(35%)
C. VASCULAR 2. Meningioma(25%)
1. Cord concussion= rev€fsiblelocaledema 3. Lipoma
2. Hemorrhagic contusion 4. Dermoid
3. Cord transection commonlyconus/ caudaequina;associatedwith
4. AVM spinaldysraphism(1/3)
5. Ependymoma
D. CHRO NI CI N F E C T ION commonlyfilumterminale;NO spinaldysraphism
1. Sarcoid 6. "Drop metastases"from CNS tumors
2. Transversemyelitis 7. Metastasesfrom outsideCNS
3. Multiplesclerosis 8. Arachnoidcyst
9. Neurentericcyst
mnemonic.' "l'M ASHAMED"
10. Hemangioblastoma
Inflammation (multiplesclerosis,sarcoidosis,
myelitis) 11. P aragangl i oma
Medulloblastoma
Astrocytoma mnemonic.' "MAMA N"
Syringomyelia i hydromyelia Metastasis
Hematoma,Hemangioblastoma Arachnoiditis
Arteriovenousmalformation Meningioma
Metastasis AVM, Arachnoidcyst
Ependymoma Neurofibroma
Dermoid
EpiduralExtramedullary
Lesion
IntrameduIIary Neop I astic Lesion Prevalence: 30% of all spinaltumors
A. GLIALNEOPLASM A . TU MOR
1. Ependymoma(60%) (a) benign
2. Astrocytoma(33%) 1. D ermoi d,epi dermoi d
(1 % )
3. G ang l i o g l i o ma 2. Lipoma: overseveralsegments
B . NO NG LI A LN EOP L AS M 3. Fibroma
(a) highlyvascularlesions: 4. Neurinoma(withintraduralcomponent)
1. He ma n g i o b l a s to m a 5. Meningioma(withintraduralcomponent)
2. Paraganglioma 6. Ganglioneuroblastoma, ganglioneuroma
(b) rare lesions: (b) malignant
3. Metastasis 1. H odgki ndi sease
4. Lymphoma 2. Lymphoma:mostcommonlyin dorsalspace
5. Primitiveneuroectodermal tumor 3. Metastasis:breast,lung- mostcommonly
C. EXTRAMEDULLARY NEOPLASM from involvedvertebraewithoutextension
1. Intramedullary meningioma throughdura
2. Intramedullary schwannoma 4. Paravertebralneuroblastoma
188 RadiologyReview Manual

B. DI S KDI S E A SE ExtraarachnoidMyelography
1. B ulgin gd i s k A . S U B D U R A LIN JE C TION
2. Herniatednucleuspulposus { spinalcord, nerve roots,bloodvesselsnot outlined
3. Sequesterednucleuspulposus { irregularfillingdefects
c. BONE: spinalstenosis,spondylosis r/ slow flow of contrastmaterial
D . INFECTION:epiduralabscess r/ CSf pulsationsdiminished
E . BLOOD: hematoma r/ contrastmaterialpools at injectionsite within
F . OTHERS: synovialcyst, arachnoidcyst, extradural anterior/ posteriorcompartments
lipomatosis,extrameduIlary hematopoiesis B . E P ID U R A LIN JE C TION
ri contrastextravasationalong nerveroots
mnemonic.' "MANDELIN" r/ contrastmateriallies near peripheryof spinal
Metastasis(dropmetsfrom CNS tumor),Meningioma canal
Arachnoiditis,Arachnoidcyst ./ intraspinal structuresare not well outlined
Neurofibroma
Dermoid/ epidermoid
Ependymoma TUMORS
NEUROGENIC
MUSCULOSKELETAL
Lipoma A . B E N I G NN E U R O G E N ITCU M O R
Infection(TB, cysticercosis) 1. Traumati cneuroma
Normalbut tortuousroots 2. Mortonneuroma
3. Neuralfibrolipoma
4. Nervesheathganglion
Cord Lesions 5. Benignperipheralnervesheathtumors(PNST)
A. INFLAMMATION B . MA LIGN A N T N E U R OGE N IC N E OP LA S M
1. Multiplesclerosis = malignantperipheralnervesheathtumor(PNST)
2. Acute disseminatedencephalomyelitis
3. Acutetransversemyelitis BenignTumorof NerveSheath
r/ involveshalf the cross-sectional
area of cord = BENIGN PERIPHERAL NERVESHEATH TUMOR(PNST)
4. Lym,edisease = N E U R IN OMA
5. Devicsyndrome containscellularelementscloselyrelatedto Schwann
B . I NF E CT I O N cell
1. Cytomegalovirus Schwanncell = cellthat surroundscranial,spinal,and
2. Progressivemultifocalleukoencephalopathy peripheralnervesproducingmyelinsheatharound
3. HIV axonsthus providingmechanicalprotection, serving
C. VASCULAR as a tract for nerveregeneration
1. Anteriorspinalarteryinfarct 0 NOTE that myelinsheathswithinbrainsubstanceare
{ affectscentralgray matterfirst madeby oligodendrocytes!
{ extendsto anteriortwo-thirdsof cord P l ai nfi l m:
2. Venousinfarcti ischemia r/ fusiformmassdelineatedby surrounding fat
{ startscentrallyprogressingcentripetally r/ soft-tissueand osseousovergrowth
D. NEOPLASM r/ bone involvement + mineralization(osteoid/
chondroid/ amorphous)only in largerlesions
A ngi o:
Cord Atrophy r/ displacementof majorvascularstructures
1. Multiplesclerosis { corkscrew-type vesselsat upper/ lower pole of
2. Amyotrophiclateralsclerosis tumor (= hypertrophyof nutrientnervevasculature)
3. Cervicalspondylosis MR , C T:
4. Sequelaeof trauma { fusiformmass in a typicalnervedistribution(94%):
5. lschemia { entering+ exitingnerve (intradural/ extradural)
6. Radiationtherapy r/ dumbbellshapewith extensionintoenlarged
7. AVM of cord neuralforamen(intra-and extradural)
ri low attenuation(as low as 5-25 HU) due to
(a) high lipidcontentof myelinfrom Schwann
DelayedUptakeof Water-Soluble
Gontrastin cells
Cord Lesion (b) entrappedfat
1, Syringohydromyelia (c) endoneuralmyxoidtissuewith high water
2. Cystictumor of cord content(AntoniB areas)
3. Osteomalacia { isointense to muscleon TlWl + hyperintense to
exceedinglyrare: 4. Demyelinatingdisease fat on T2Wl
5. Infection { well-definedhyperdense/ hypointensemargins
6. Infarction r/ hypointenseon T2Wl in diffuseneurofibromas:
 DifferentialDiagnosisof Skulland SpineDisorders 1 8 9

r/ "targetsign": 0 Usuallysporadictumor,but 5-20% of patients

r/ hypo-to isointensecenter+ hyperintense with solitaryintracranialschwannomashave
peripheryon T2Wl (almostPATHOGNOMONTC) neurofibromatosis type 2!
r/ hyperdensecenter+ hypodenseperiphery . painless,fairlymobilemass
r/ "fascicular sign"= multiplesmallringlike . * neurologicsymptoms
structureswith peripheralhighersignalintensity r/ solitaryfusiformwell-encapsulated <5 cm lesion
on T2Wl { slow growth
r/ "split-fatsign" = rim of fat surroundingmass MR:
suggestsa tumororiginin the intermuscular { well-delineated massof intermediate signal
space intensityon Tl Wl
r/ markeduniformenhancement(mosthelpfulfor r/ heterogeneous massof highsignalintensityon
intradurallesions) T2WI
r/ muscleatrophywith striatedincreasedfat content ^/ frequentlylow-signal-intensityrim (= capsule)
(in 23%) r/ heterogenous enhancementin 33%
Ga-67scintigraphy: r/ peritumoral edemain 33%
r/ significantuptakein malignantPNST DDx: may appearsimilarto meningioma
Rx: excision(affectednerve usuallyseparablefrom
neoplasmafterincisionof epineurium)
Schwannoma= Neurilemmoma
= usuallysolitarywell-encapsulated benignslowly
growingneoplasmarisingfrom Schwanncells Neurofibroma
displacingnervefiberseccentrically = usuallymultipleoften infiltrativetumorsof nerve
0 Nerve root NOT incorporated sheathseparatingnervefibers resultingin fusiform
Prevalence: 5-10% of all benignsofttissue tumors enlargementof nerve
Age: 20-30 years Prevalence: 5'/. of all benign soft-tissuetumors
Path: fusiformmass with entering+ exitingnerve Histo: swirlsof neuronalelementscontaining
surroundedby a true capsuleof epineurium;in Schwanncells,nervefibers,fibroblasts,
large nervesmass is eccentricto involved collagen
nervewith nervefiberssplayedaboutthe Associatedwith: neurofibromatosistype 1
neoplasm Age: 20-30 years; M:F = 1:1
Histo: S-100 proteinpositive 0 Malignanttransformationexceedinglyrarel
(a) cellularcomponent(Antoni type A tissue): 0 The spinalneurofibroma is rarelysporadicand
more organizedarea composedof densely usuallya sign of type 1 neurofibromatosis!
packedcellularspindlecellsarrangedin short 0 Only 10T"of patientswith neurofibromashave von
bundles/ interlacing fascicles Recklinghausen disease!
Location: posteriormediastinum,
retroperitoneum, 25'/. of extremity Location: any level,but particularlycervical
lesions (a) peripheralnerves
r/ hypointenseon T2Wl ^/ nonencapsulatedwell-circumscribedf usiform
(b) myxoidcomponent(Antoni type B tissue): mass of peripheralnerves
less organizedlooselyarrangedarea of (b) intraduralextramedullarymass
hypocellular myxoidtissuewith highwater { well-defined masswith dumbbellconfiguration
content (= extraduralcomponentextendsthrough
r/ hyperintenseon T2Wl neuralforamen)
(c) ancientschwannoma:degenerative changesof { wideningof intervertebral foramen+ erosion
cystformation,calcification, hemorrhage, of pedicles
fibrosis r/ scallopingof vertebralbodies
Location: spinal+ sympatheticnerve roots r/ hypodense(CHARACTERISTIC) approaching
(a) extracranial:neck,flexorsurfacesof upper characteristics
of water / isodenseto skeletal
+ lowerextremities, posteriormediastinum, muscle
retroperitoneum r/ usuallyNO contrastenhancement
Site: spinaland sympatheticnerveroots,ulnar MR:
n ., p e ro n e anl .' ./ homogeneousmass isointenseto cord / muscle
0 Usuallysolitary,but in 57" associatedwith on Tl W l
neurofibromatosis type 1 (= >2 schwannomas r/ hyperintensetumor on T2Wl comparedwith
/ one plexiformneurofibroma) fat
, surrounding
(b) intracranial:mostlyfrom sensorynerves, r/ "targetsign" = low signal-intensitycenteron T2Wl
vestibulocochlear (Vlll)cranialnerve(most (dueto collagen+ condensedSchwanncells)in
common)> trigeminal(V) cranialnerve(2nd 70% of extracranialneurofibromas
most common)> Vll r/ + muscularatrophy
190 Radiology Review Manual
DDx: conjoinednerve root sleeve
Rx: surgicalresectionwith sacrificeof nerve (tumor
not separablefrom normalnerve)
Locnuzeo Neunonenonra
Prevalence: 90% of all neurofibromas
Path: fusiformtumor,often remainingwithin
epineuriumas a true capsule
Histo: interlacingfasciclesof wavy elongated
cellscontainingabundantamountsof
collagen
Location: affectingprimarilysuperficial
cutaneousnerves,occasionallydeep-
seatedlargernerves
{ mostlysolitaryslow-growinglesion<5 cm in size
Drrruse NeunoneRoMA
Age: children+ youngadults
Path: poorlydefinedlesionwithinsubcutaneous
fat, infiltratingalongconnectivetissuesepta,
inseparablefrom normalnervetissue
Histo: very uniformprominentfibrillarycollagen
Location: most frequentlyin subcutaneous
tissuesof head + neck
. plaquelikeelevationof skinwiththickeningof
entiresubcutis
r/ isolatedlesionin 90% unassociatedwith NF1
{ alwaysindistinctinfiltrativemarginsdue to
subcutaneousspreadalong connectivetissue
sePta
Plexiform Neurotibroma
= involvementof long segmentof nerve+ branches
extendinginto adjacentmuscle,fat, subcutaneous
tissue
= PATHOGNOMONICof neurofibromatosis type 1
{ serpentine"bag of worms"appearance= tortuous
tangles/ fusiformenlargementof peripheralnerves
{ reticulatedlinearbranchingpatternwithin
subcutaneous tissue
MalignantTumorof NerveSheath
= P E RI P HE R A MA
L L IG N AN T N E R V ESH EA TH
T UM O R( P N S T )= N E U R O GE N IC
S PIN D L EC E LL
SARCOMA
Prevalence; 5-1 0/" of all soft-tissuesarcomas
Age: 20-50 years; M:F = 8:1
Associatedwith: neurofibromatosistype 1 (in
25-70/"), radiationtherapy(in 11%
of all malignantPNSTsaftera latent
periodof 10-20 years)
Histo: tumor cells arrangedin fasciclesresembling
fibrosarcoma;additionalheterotopicfoci with
maturecartilageand bone,
rhabdomyosarcoma elements,glandularand
epithelialcomponents(in 10-15%)
. pain, motorweakness,sensorydeficits
Location: majornervetrunks(sciaticn., brachial
plexus,sacralplexus)
Metastases: lung,bone, pleura,retroperitoneum
(60%);regionallymphnodes(9%)
{ fusiformmass with entering+ exitingnerve
{ frequentlyindistinctmargins
{ suddenincreasein size of a previouslystable
neurofibroma
{ frequentlyareasof hemorrhage+ necrosis
Rx: resection+ adjuvantchemo-and radiation
therapywith local recurrencein 40/"
Prognosis: 44/" S-yearsurvival rate
SPINAL FIXATION DEVICES
Function: (1) to restoreanatomicalignmentin fractures
(fracturereduction)
(2) to stabilizedegenerativedisease
(3) to correctcongenitaldeformities(scoliosis)
(4) to replacediseased/ abnormalvertebrae
(infection,tumor)
PosteriorFixationDevices
usingpaired/ unpairedrods attachedwith
1. S ubl ami narw i ri ng
= passinga wire aroundlamina+ rod
2. Interspinous wiring
= passinga wirethrougha holein the spinous
process;a Drummondbuttonpreventsthe wire
from pullingthroughthe bone
3. Subparswiring
= passinga wire aroundthe pars interarticularis
4. Laminar/ sublaminarhooks
used on rods for compression/ distractionforcesto
be appliedto pedicles/ laminae
(a) upgoinghookcurvesunderlamina
(b) downgoinghookcurvesover lamina
5. Pedicle/ transpedicalscrews
6. Rods
(a) Luque rod = straighti L-shapedsmoothrod
6-8 mm in diameter
(b) O-ringfixator,rhomboid-shaped bar, Luque
rectangle,segmentalrectangle= preshapedloop
to form a flat rectangle
(c) Harringtondistractionrod
(d) Harringtoncompressionrod
(e) Knodtrod = threadeddistractionrod with a
centralfixed nut (turnbuckle)and opposing
threadpattern
(f) Cotrel-Dubousset rods = ? Pdirof rods with a
serratedsurfaceconnectedby a cross-linkwith
>4 laminarhooks/ Pediclescrews
7. Plates
(a) Roy-Camille plates
= simplestraightplateswith round holes
(b) Luqueplates
= long oval holeswith clips encirclingthe plate
(c) Steffeeplates= straightplateswith long slots
 DifferentialDiagnosisof Skulland SpineDisorders l gl

F*
tl^*
1**
rte*
Cotrel-Dubousset Rods and Pedicle Screws

Dunn Rod Harrington Rod 8. Translaminar screw

Dwyer Cable = c?nc€llousscrewsfor singlelevelfusion
9. Percutaneous pinning
= (hollow)interferencescrewsplacedacrossdisk
: : level
l - - - l A
I t \-/l
/ l AnteriorFixationDevices
|t_ 1. Dwyerdevice
: :
: : = scfewsthreadedinto vertebralbody over staples
embeddedinto vertebralbody connectedby

ii braidedtitaniumwire; placedon convexside of

spine
2. Zielke device
- = modifiedDwyersystemreplacingcablewith solid
II ^.
I
1
\ l rod
t t l l 3. Kanedadevice
|
I _ - J
| V
lt
= = = 2 curvedvertebralplateswith staplesattachedto
vertebralbodieswith screws,platesconnectedby
Knodt Rod Luque Rod Steffee Plate 2 threadedrods attachedto screw heads
4. Dunndevice
Spinal Fixation Devices (similarto Kanedadevice,discontinued)
192 RadiologyReview Manual
ANATOMYOF SKULL AND SPINE
OF BASEOF SKULL
FORAMINA
on inneraspectof middlecranialfossa3 foraminaare
orientedalongan obliqueline in the greatersphenoidal
wing from anteromedialbehindthe superiororbitalfissure
to posterolateral
mnemonic.' "rotos"
foramenrotundum
foramenovale
foramenspinosum
ForamenRotundum
= coflolwithingreatersphenoidwing connectingmiddle
cranialfossa + pterygopalatine fossa
Location: inferiorand lateralto superiororbitalfissure
Course: extendsobliquelyforward+ slightly
inferiorlyin a sagittaldirectionparallelto
superiororbitalfissure
Contents:
(a) nerves: V, (maxillarynerve)
(b) vessels: (1) arteryof foramenrotundum
(2) emissaryw.
r/ best visualizedby coronalCT
ForamenOvale
= c€rrdlconnectingmiddlecranialfossa + infratemporal
fossa
Location: medialaspectof sphenoidbody, situated
posterolateralto foramenrotundum
(endocranialaspect)+ at base of lateral
pterygoidplate (exocranialaspect)
Contents:
(a) nerves: (1) V, (mandibular nerve)
(2) lesserpetrosalnerve (occasionally)
(b) vessels: (1) accessorymeningealartery
(2) emissaryvv.
ForamenSpinosum
Location: on greatersphenoidwing posterolateralto
foramenovale (endocranialaspect)
+ lateralto eustachiantube (exocranial
aspect)
Contents:
(a) nerves: (1) recurrentmeningealbranchof
mandibularnerve
(2) lessersuperficialpetrosalnerve
(b) vessels: (1) middlemeningeala.
(2) middlemeningealv.
ForamenLacerum
carotidarteryrests
cover (occasionally),
Fibrocartilage
on endocranialaspectof fibrocartilage
Location: at base of medialpterygoidplate
Contents: (inconstant)
(a) nerve: nerveof pterygoidcanal (actuallypierces
cartilage)
(b) vessel: meningealbranchof ascending
pharyngeala.
ForamenMagnum
Contents:
(a) nerves: (1) medullaoblongata
(2) cranialnerveXl (spinalaccessoryn.)
(b) vessels: (1) vertebrala.
(2) anteriorspinala.
(3) posteriorspinala.
PterygoidGanal
= VIDIAN CANAL
= withinsphenoidbody connectingpterygopalatine
fossa
anteriorlyto foramenlacerumposteriorly
Location: at base of pterygoidplate belowforamen
rotundum
Contents:
(a) nerves: vidian nerve= rl€fveof pterygoidcanal
= continuationof greatersuperficialpetrosalnerve
(fromcranialnerveVll) afterits unionwith deep
petrosalnerve
(b) vessel: vidianartery= art€rYof pterygoidcanal
= branchof terminalportionof internalmaxillarya.
arisesin pterygopalatinefossa + passesthrough
foramenlacerumposteriorto Vidiann.
HypoglossalCanal
= ANTERIOR CONDYLAR CANAL
Location: in posteriorcranialfossa anteriorlyabove
condytestartingabove anterolateralpart of
foramenmagnum,continuingin an
anterolateraldirection+ exitingmedialto
jugularforamen
Contents:
(a) nerves: cranialnerveXll (hypoglossal nerve)
(b) vessels:(1) pharYngealarterY
(2) branchesof meningealarterY
JugularForamen
Location: at the posteriorend of petro-occipital suture
directlyposteriorto carotidorifice
(a) anteriorpart:
(1) inferiorpetrosalsinus
(2) meningealbranchesof pharyngealartery+
occipitalartery
(b) intermediatepart:
(1) cranialnervelX (glossopharyngeal nerve)
(2) cranialnerveX (vagusnerve)
(3) cranialnerveXl (spinalaccessorynerve)
(c) posteriorpart: internaljugularvein
 Anatomyof Skulland Spine 193

CRA NI O V E RT E B R A L J U N C T IO N
Craniometry:
- LATERALVIEW
1. Chamberlainline = linebetweenposteriorpole
of hardpalate+ opisthion(= posteriormarginof
foramenmagnum)
^/ tip of odontoidprocessusuallylies below/
tangentto Chamberlainline
! tip of odontoidprocessmay lie up to 1 t 6.6
mm abovethe Chamberlain line
2. McGregor line = line betweenposteriorpole of r - r r atlanto-occipital joint axis angle (124' - 127')
hard palate+ most caudalportionof occipital
. . ' . bimastoidline
squamosalsurface
0 Substituteto Chamberlainlineif opisthionnot
Open-Mouth Odontoid View
visible
^/ tip of odontoid<5 mm abovethis line

- VIEW(= "open-mouth"
ANTEROPOSTERIOR /
odontoid
view)

7. Atlantooccipital joint axisangle

= formedby linesdrawnparallelto bothatlanto-
occipitaljoints
{ linesintersectat centerof odontoidprocess
./ averageangleof 125' (rangeof 124'to 127")
posterior pole 8. Digastricline= linebetween incisurae
of hard palate mastoideae (originof digastric
muscles)
^/ tipof odontoid
belowthisline
9. Bimastoidline= lineconnecting thetipsof both
mastoidprocesses
anterior arch C 1 WpostenorarchCl r/ tipof odontoid
<10mmabovethisline
@
odontoidprocess

r - r r r. Chamberlainline
basion-posterior
r. i r r r..... ff'glgher baSal angle (<140") / axial line interval
,.,,,.,,,rrr'r',',,,Craniovertebral angle (150"-l
80")

Skull Base Lines on Lateral View

3 . Wackenheim clivus baseline

= BASILARLINE= linealongclivus
^/ usuallyfallstangentto posierioraspectof tip
of odontoidprocess
4 . Craniovertebralangle = clivus-canalangle posterior axial line
= €rhgleformedby line along posteriorsurfaceof
axis body and odontoidprocess+ basilarline
r/ rangesfrom 150" in flexionto 180" in extension
! ventralspinalcord compressionmay occur at
< 1500
5 . Welcher basal angle
= formedby nasion-tuberculum line and
tuberculum-basion line thickness of prevertebral soft tissues
r/ angleaverages132' (shouldbe <140.)
6 . McRae line = linebetweenanteriorlip (= basion) Normal Relationship of Craniocervical Junction
to posteriorlip (= opisthion)of foramenmagnum (range of the two extreme normal positions of the basion
r/ tip of odontoidbelowthis line is drawn in dashes)
| 94 Radiology Review Manual

Normal dimensions for adults: SPACE

[posterioraxial line = verticalline drawn along posterior
aspectof the subdentalbody of
czl
B as ion- dens in te rv a(il n 9 5 % )
Basion-posterior axialline interval(in 98%)
pos t er iorto dens
anteriorto dens
Prevertebral softtissuesatC2
A nt er iorat lan to -d e ni sn te rv a...............
l <2 mm
Lateralatlanto-densinterval(sideto-side)... <3 mm
Atlanto-occipital articulation .............
A t lant oax ialarti c u l a ti o n .........
ME NI NG E S O F S PIN AL C OR D
A. P E RI O S T EU M
= continuationof outer layer of cerebraldura mater
B . E P I DURA LS PA C E
= spoce betweendura mater + bone containingrich
plexusof epiduralveins,lymphaticchannels,
connectivetissue,fat
(a) cervical+ thoracicspine: spaciousposteriorly,
potentialspace anteriorly
r/ normalthicknessof epiduralfat 3-6 mm at T7
(b) lowerlumbar+ sacralspine: may occupymore
than half of cross-sectionalarea
C. DURA
= continuation of meningeal/ innerlayerof cerebral
dura mater;ends at 2nd sacralvertebra+ forms
coccygealligamentaroundfilumterminale;sends
tubularextensionsaroundspinalnerves;is
continuouswith epineuriumof peripheralnerves
Attachment: al circumferenceof foramenmagnum,
bodiesof 2nd + 3rd cervicalvertebrae,
posteriorlongitudinalligament(by
connectivetissue strands)
D . S U B A R A C H N OID
= sP?cebetweenarachnoidand pia matercontaining
CSF, reachingas far lateralas spinalganglia
dentateligamentpartiallydividesCSF space into an
anterior+ posteriorcompartmentextendingfrom
.....< 1 2 mm foramenmagnumto 1st lumbarvertebra,is
continuouswith pia materof cord medially+ dura
.....< 1 2 mm materlaterally(betweenexitingnerves)
<4 mm dorsalsubarachnoid septumconnectsthe arachnoidto
..,...<6 mm the pia mater(cribriform septum)
E . P IA MA TE R
= firm vascularmembraneintimatelyadherentto spinal
<2 mm cord,blendswith dura materin intervertebral
........< 3 mm foraminaaroundspinalganglia,formsfilum
terminale,fuseswith periosteumof 1stcoccygeal
segment
SPINE
THORACIC
12 load-bearingvertebrae
posteriorarch (= pedicles,laminae,facets,transverse
processes)handlestensionalforces
vertebralbodies:
(a) heightof vertebraeanteriorly2-3 mm less than
posteriorly= mild kyphoticcurvature
(b) AP diameter:gradualincreasefrom T1 toT12
(c) transversediameter:gradualincreasefrom T3 to
T12
SPINE(T11-12)
THORACOLUMBAR
- anteriorcolumn= anteriorlongitudinalligament,anterior
annulusfibrosus,anteriorvertebralbody
- middlecolumn= posteriorlongitudinal ligament,
posteriorannulusfibrosus,posteriorvertebralbody
margin
0 Integrityof the middlecolumnis synonymouswith
stability!
- posteriorcolumn= posteriorelements+ ligaments

anterior spinal a. ventral root

pia mater
dorsal root

dentate ligament

subarachnoid space
dorsal
subarachnoid subdural space
septum
dura mater epidural space

K/
posterior spinal aa.,

Meninges of Spinal Cord

 Anatomy of Skull and Spine 195

uncrnateprocess costal process

anterior tubercle
costotransversebar

posterior tubercle

facet of superior
articular process
lamina (arch)
spinousprocess

Typical Cervical Vertebra

(cranialaspect)

inferior superror
articulating superror
process articulating lateral recess
process articulating
root of 5th process of
lumbar nerve L5

A
B
inferior
C articulating
lamina process of
L4

Lateral Scout View of L5 Cross Sectionthrough @

basivertebral v.

lumbar v. ventral rami

dorsal intervertebral
of 5th
ganglia of disk L5lS1
lumbar nerve
5th lumbar
nerve
anterior internal
vertebral
(epidural) vv.
interarticularis
lig. flavum thecal sac
spinous process L5

Cross Sectionthrough @ Cross Sectionthrough @

Cross Sectionsthrough Sth Lumbar Vertebra
196 Radiology Review Manual

VERTEBRA
TRANSITIONAL OF CONUSMEDULLARIS
NORMALPOSITION
= vertebraretainingpartialfeaturesof segmentsbelow 0 Vertebralbodiesgrow more quicklythan spinalcord
and above;totalnumberof vertebraeusuallyunchanged duringfetalperiodof <19 weeksMA!
Prevalence: 20/" 0 No significantdifferenceregardlessof agel
. incidentalfinding
Location: aspectof conus:
lnferior-most
often at sacrococcygeal+ lumbosacraljunction L1-L2level: normal(rangeT12to L3)
{ "sacralizedL5" = L5 incorporatedinto sacrum L2-L3 or higher: in 97.8%
r/ "lumbarized 51" = 51 incorporatedinto lumbarspine L3 level: indeterminate(in 1.8%)
Cx: confusionover labeling/ assignmentof vertebral L3-L4 / lower: abnormal
levelsduringtreatmentplanning by 3 month: aboveinferiorendplateof L2 (in
e8%)

postenor N.B.: lf conusis at / belowL3 level,a searchshouldbe

anterior
longitudinal
longitudinal made for tetheringmass, bony spur,thick filum!
ligament ligament

Sharpey's fibers

Anatomy of Discovertebral Junction

anterior longitudinal ligament attachesto anterior surface of
vertebral body; it is less adherentto intervertebral disk;
posterior longitudinal ligament is applied to back of intervertebral
disk and vertebral bodies

sup. band of cruciatelig.

ant. atlanto-
occipital membrane tectorial membrane

apical ligament
post. atlanto-occipital
membrane
ant. arch of atlas synovial bursa

synovial cavities

tranverse lig. of atlas lisamentum flavum

inf. band of cruciate lie.

post. longitudinal lig.

ant. longitudinal lig.

Joints and Ligaments of Occipito-Atlanto-Axial Region

 Skull and Spine Disorders l 97

SKULLANDSPINEDISORDERS

ARACHNOIDITIS 3. Duralarteriovenousfistula
Etiology:
trauma,
backsurgery,
meningitis,
subarachnoid = singleshunt betweenmeningealarteries+
hemorrhage,pantopaguemyelography intradural
vein
(inflammatory effectpotentiatedby blood), 4. Metamericangiomatosis
idiopathic
Associatedwith: syrinx ATLANTOAXIAL ROTARY FIXATION
Myelo: . historyof insignificantcervicalspine trauma/ upper
{ bluntingof nerve root sleeves respiratorytract infection
! blockednerve rootswithoutcord displacement(2/3) . limitedpainfulneckmotion
r/ streaking+ clumpingof contrast . head held in "cock-robin"position+ inabilityto turn head
CT: { atlanto-odontoid asymmetry(open mouthodontoid
./ fusioni clumpingof nerve roots view):
r/ intraduralpseudomass r/ decreasein atlanto-odontoid space + wideningof
{ intraduralcysts lateralmass on side ipsilateralto rotation
rl "emptythecalsoc" = featurelessempty-lookingsac r/ increasein atlanto-odontoid space + narrowingof
with individualnerve rootsadherentto wall (finat lateralmass on side contralateralto rotation
stage) r/ atlantoaxialasymmetryremainsconstantwith head
turned into neutralposition
ARACHNOID
CYSTOF SPINE Types:
Location: dorsalto cord in thoracicregion | <3 mm anteriordisplacementof atlason axis
Site: ll 3-5 mm anteriordisplacement
(a) extraduralcyst secondaryto congenital/ acquired lll >5 mm anteriordisplacement
dural defect lV posteriordisplacementof atlas on axis
(b) intraduralsecondaryto congenitaldeficiencywithin DDx: torticollis(atlantoaxialsymmetryrevertsto normal
arachnoid(= true arachnoidcyst)/ adhesionfrom with head turnedinto neutralposition)
, prior infectionor trauma(= €lr€lchnoid
loculation)
r/ oval sharplydemarcatedextramedullarymass BRACHIAL
PLEXUSINJURY
r/ immediate/ delayedcontrastfillingdependingupon size 1. Erb-Duchenne:adductioninjuryaffectingC5/6
of openingbetweencyst + subarachnoidspace (downwarddisplacementof shoulder)
r/ localdisplacement+ compressionof spinalcord 2. Klumpke:abductioninjuryalC7, C8, T1 (arm
^/ highersignalintensitythan CSF (fromrelativelack of stretchedover head)
CSF pulsations) r/ pouchlikeroot sleeveat site of avulsion
r/ asymmetricalnerve roots
ARACHNOID
DIVERTICULUM rl contrastextravasationcollectingin axilla
= wideningof root sheathwith arachnoidspace occupying r/ metrizamidein neuralforamina-(CTmyelography)
>50/" of total transversediameterof root + sheath
together CAUDALREGRESSION
SYNDROME
Cause: ? congenital/ traumatic,arachnoiditis,infection = SACRAL AGENES]S
Pathogenesis; hydrostaticpressureof CSF = midlineclosuredefectof neuraltubewith a spectrumof
rl scallopingof posteriormarginsof vertebralbodies anomalies
{ myelographiccontrastmaterialfillsdiverticula Etiology: disturbanceof caudalmesoderm<4th week of
gestationfrom toxici infectious/ ischemicinsult
Prevalence: 1:7,500births; 0.005-0.01./"of population;
ARTERIOVENOUS
MALFORMATION
OF SPINAL 0.1-0.2%in childrenof diabeticmothers
CORD Predisposed: infantsof diabetic mothers (1C22% ot
Classification: childrenwith sacralagenesishave
1. True intramedullary AVM motherswith diabetesmellitus!)
= nidusof abnormalintermediaryarteriovenous 0 NOT associatedwith VATERsyndromel
structurewith multipleshunts A. Musculoskeletal anomalies
Age: 2nd-3rd decade @ Lowerextremity
Cx: subarachnoidhemorrhage,paraplegia . symptomsfrom minor muscleweaknessto
Prognosis.' poor (especiallyin midthoracic completesensorimotorparalysisof both lower
location) extremities
2. lntraduralarteriovenousfistula r/ rripdislocation
= singleshunt betweenone / severalmedullary r/ foot deformities
arteries+ singleperimedullaryvein r/ trypoplasiaof extremities
198 Radiology Review Manual

@ Lumbosacral spine= SACRAL AGENESIS Prevalence: 1:2,000,000;1-2-4o/oof all primary

Spectrum:
Type 1 = unilateralpartialagenesislocalizedto
sacrum/ coccyx
Type 2 = bilateralpartialsymmetricdefectsof
sacrum+ iliosacralarticulation
Type 3 = total sacralagenesis+ iliolumbar
articulation
Type 4 = total sacralagenesis+ ilioilialfusion
posteriorly
! fusionof caudal-most2 or 3 vertebrae
{ spinabifida(lipomyelomeningocele oftennot in
combination with hydrocephalus)
{ narrowingof spinalcanal rostralto last intact
vertebra
B. Spinalcord anomalies
! characteristic club- / wedge-shapedconfigurationof
conusmedullaris(hypoplasia of distalspinalcord)
^/ + tetheredspinalcord
r/ + dural sac stenosiswith hightermination
r/ t spinalcord lipoma,teratoma,cauda equinacyst
{ + syrinx
C. Genitourinary anomalies
. neurogenicbladder(it >2 segmentsare missing)
. malformedexternalgenitalia
. lack of bowelcontrol
^/ + bilateralrenalaplasiawith pulmonaryhypoplasia
+ Potterfacies
{ anal atresia
O B - US :
. normal/ imperforateanus
{ normal/ mildlydilatedurinarysystem
r/ normalI increasedamnioticfluid
r/ 2 umbilicalarteries
^/ e nypoplasticnonfusedlowerextremities
{ sacralagenesis,absentvertebraefrom lowerthoracic
/ upperlumbarspinecaudally
Sirenomelia
= recentlyconsidereda distinctseparateentityfrom
caudalregressionsyndrome
0 NOT associatedwith maternaldiabetesmellitusl
Potterfacies
: absenceof anus
o
absentgenitalia
{ bilateralrenalagenesis/ dysgenesis(lethal)
markedoligohydramnios
{ singleaberrantumbilicalartery
{ single/ fused lowerextremity
^/ sacralagenesis,absentpelvis,lumbosacral"tail",
lumbarrachischisis
Prognosis; incompatiblewith life
CHORDOMA
0 Chordomais the mostcommonprimarymalignanttumor
of the spinein adultsexcludinglymphoproliferative
neoplasmsl
malignantneoplasmsof bone;1'/" of all
intracranialtumors
Etiology: originatesfrom embryonicremnantsof
notochord/ ectopiccordalfoci (notochord
appearsbetween4th and 7th week of
embryonicdevelopment,extendsfrom Rathke
pouchto coccyxand forms nucleuspulposus)
Age: 30-70 years (peakage in 6th decade);
M:F = 2:1;hi ghl ymal i gnanti n chi l dren
Path: lobulatedtumor containedwithinpseudocapsule
Histo:
(1) typicalchordoma:cords+ clustersof large
bubblelikevacuolated(physaliferous) cells
containing intracytoplasm ic mucousdroplets;
abundantextracellularmucusdeposition+ areasof
hemorrhage
(2) chondroidchordoma: cartilageinsteadof mucinous
extracellularmatrix
Location: (a) 50% in sacrum(b) 35% in clivus
(c) 15% in vertebrae(d) othersites (5%) in
mandible,maxilla,scapula
r/ enhancementaftercontrastadministration
CT:
r/ low-attenuation withinsoft-tissuemass (due to
myxoid{ype tissue)
r/ nigherattenuationfibrouspseudocapsule
MR (modalityof choice):
^/ low to intermediate intensityon T1Wl,occasionally
hyperintense (dueto highproteincontent):
r/ heterogeneousinternaltexturedue to calcification,
necrosis,gelatinousmucoidcollections
r/ very highsignalintensityon T2Wl (similarto nucleus
pulposuswith high water content)
A ngi o:
./ prominentvascularstain
NUC:
{ cold lesionon bonescan
{ no uptakeon galliumscan
Metastases(in 5-43%) to: liver,lung, regionallymph
nodes,peritoneum, skin
(late),heart
Prognosis: almost 100% recurrencerate despite radical
surgery
Sacrococcygeal Chordom a (50-70%)
0 Mostcommonprimarymalignantsacraltumor;
24% of all malignantosseousneoplasms!
Peak age: 40-60 years; M:F = 2:1
. low back pain (70%)
. constipation/ fecal incontinence
. rectalbleeding(42%)
. sciatica
. frequency,urgency,strainingon micturition
. sacralmass (17%)
Location: esp. in 4th + 5th sacralsegment
! presacralmass with averagesize of 10 cm extending
superiorly+ inferiorly;rarelyposteriorlocation
r/ displacementof rectum+ bladder
{ solidtumor with cysticareas (in 50%)

r/ osteolyticmidlinemass in sacrum+ coccyx
r/ amorphousperipheralcalcifications(15-gg%)
r/ secondarybonesclerosisin tumorperiphery(50%)
r/ honeycombpatternwith trabeculations1fO-f bZ1
r/ may cross sacroiliacjoint
Prognosis; 8-10 years averagesurvival;66% S-year
survivalrate (adulthood)
DDx: Giantcell tumor,plasmacytoma, lymphoma,
metastaticadenocarcinoma,aneurysmalbone
cyst, atypicalhemangioma,chondrosarcoma,
osteomyelitis, ependymoma
Spheno-occipital Chordoma (15-35%)
Age: youngerpatient(peakage of 2O4O years);
M : F- 1 : 1
. orbitofrontalheadache
. visualdisturbances,ptosis
. 6th nerve palsy/ paraplegia
Location: clivus,spheno-occipital synchondrosis
r/ bonedestruction(in 90%): ciivus> sella> petrous
bone > orbit> floorof middlecranialfossa> jugular
fossa > atlas > foramenmagnum
{ reactivebone sclerosislrare)
r/ calcifications / bone fragments(20-70%)
r/ soft-tissueextensioninto nasopharynx(common),into
sphenoid+ ethmoidsinuses(occasionally), may
reachnasalcavity+ maxillaryantrum
r/ variabledegreeof enhancement
MR:
r/ large intraosseousmass extendinginto prepontine
cistern,sphenoidsinus,middlecranialfossa,
nasopharynx
r/ posteriordisplacementof brainstem
{ usuallyisointense to brain/ occasionally
inhomogeneously hyperintense on T1Wl
r/ hyperintenseon T2Wl
Prognosis; 4-5 years average survival
DDx: meningioma, metastasis, plasmacytoma,giant
celltumor,sphenoidsinuscyst,nasopharyngeal
carcinoma,chondrosarcoma
Vertebraf/ SpinalGhordoma(1s-20%)
more aggressivethan sacralI cranialchordomas
Age: youngerpatient;M:F = 2:1
. low back pain + radiculopathy
Location: cervical(8% - particularlyC2),thoracic
, spine(4%),lumbarspine(3%)
r/ solitarymidlinespinalmass
r/ tumor calcificationin 30%
r/ sclerosis/ "ivory vertebra"in 4J-62/"
^/ totatdestructionof vertebra,initiallyunaccompanied
, by collapse
r/ variableextensionintospinalcanal
r/ violatesdisk space to involveadjacentbodies(10-
14%) simulatinginfection
r/ anteriorsoft-tissuemass
Cx: completespinalblock
Prognosis: f5 years averagesurvival
DDx: metastasis,primarybone tumor,primarysoft-
tissuetumor,neuroma,meningioma
Skulland SpineDisorders 1 9 9
CSFFISTULA
Cause:
(1) Traumato skullbase (mostcommonly)
0 2% of all head injuriesdevelopCSF fistula
(2) Tumor: especiallythosearisingfrom pituitarygland
(3) Congenitalanomalies:encephalocele
. traumaticleak: usuallyunilateral; onsetwithin48 hours
aftertrauma,usuallyscanty;resolvein 1 week
. nontraumaticleak: profuseflow; may persistfor years
. anosmia(in78% of traumacases)
Location: fracturesthroughfrontoethmoidal complex
+ middlecranialfossa(mostcommonly)
r/ high-resolution thin-sectionCT in coronalplaneiollowed
by rescanningafter low-doseintrathecalcontrast
materialinstilledinto lumbarsubarachnoid space
Cx: infection(in 25-50% of untreatedcases)
DEGENERATIVE
DISKDISEASE
0 Therapeutic decision-making shouldbe basedon
clinicalassessmentalone!
0 There are no prognosticindicatorson imagesin patients
with acutelumbarradiculopathy!
35Y"of individuals withoutbacktroublehave abnormal
findings(HNP,disk bulging,facetdegeneration, spinal
stenosis)
0 lmagingis onlyjustifiedin patientsfor whom surgeryis
considered!
Pathophysiology:
loss of disk heightleadsto stresson facetjoints
+ uncovertebraljoints (= uncinateprocess),
exaggerated joint motionwith misalignment
(= rostrocaudalsubluxation)of facetjoints,spine
instabilitywith arthritis,capsularhypertrophy,
hypertrophyof posteriorligaments,facet fracture
P l ai nfi l m:
^/ intervertebral osteochondrosis = diseaseof
nucleuspulposus(desiccation= loss of disk water):
{ narrowingof disk space
r/ vacuumdisk phenomenon= radiolucent interspace
accumulationof nitrogengas at sites of negative
pressure
r/ Oist< calcification
./ bone sclerosisof adjacentvertebralbodies
r/ spondylosis deformans = degenerationof the outer
fibersof the annulusfibrosus:
r/ endplateosteophytosisgrowinginitiallyhorizontally
+ then verticallyseveralmillimeters from disko-
vertebraljunction (2. to displacement of nucleus
pulposusin anterior+ anterolateraldirection
producingtractionon osseousattachmentof
annulusfibrosus[= fibersof Sharpey])
r/ enlargementof uncinateprocesses
r/ osteoarthritis = degenerativediseaseof synovium-
linedapophyseal/ costovertebral joints:
r/ degenerativespondylolisthesis
! cartilaginous node = intraosseous disk herniation
Myelography:
r/ delineationof thecalsac, spinalcord, exitingnerve
roots
200 Radiology Review Manual

CT (accuracy>90%): 3. Diskherniation
r/ facetjointdisease(marginalsclerosis,joint narrowing, = disk protrusionthat exceeds>3 mm beyond
cyst formation,bony overgrowth) vertebralbody margin
{ uncovertebraljoint diseaseof cervicalspine 4. Diskextrusion
(osteophytesprojectinto lateralspinalcanal = prominentfocalextensionof disk materialthrough
+ neuroforamen) the annuluswith only an isthmusof connectionto
MR: parentdisk + intact/ rupturedposteriorlongitudinal
r/ scallopingof cord (T2W FSE/ GRE images): ligament
r/ anteriorencroachmentby disk / spondylosis 5. Freefragment
{ posteriorencroachment by ligamentumflavum = frank separationof disk materialfrom parentdisk
hypertrophy 6. Freefragmentmigration
^/ loss of disk signal(due to desiccation) = s€Porsteddisk materialtravelsabove/ below
^/ Oist< collapse disk space
intervertebral
r/ endplate + marrow changes (Modic& DeRoos):
(a) Type | (4%)hypointense on TlWl BulgingDisk
+ hyperintense on T2Wl (= bone marrow = broad-baseddisk extensionoutwardin all directions
edema+ vascularcongestion),contrast- with weakenedbut intactannulusfibrosus+ posterior
enhancement of marrow longitudinalligament
(b) Type ll (16%)hyperintense on TlWl + iso-to Age: commonfindingin individuals >40 yearsof age
hypointenseon T2Wl (= localfatty Location: lumbar,cervicalsPine
replacementof marrow) r/ roundedsymmetricdefectlocalizedto disk space
(c) Type lll hypointense signalon TlWl + T2Wl level
(= advancedsclerosisafter a few years) { concaveanteriormarginof thecalsac
r/ juxtaarticularsynovial cyst in posterolateralspinal r/ encroachment on inferiorportionof neuroforamen
canal(mostfrequentlyat L4-5): MR:
r/ smoothwell-definedextraduralmass adjacentto r/ nucleuspulposushypointense on Tl Wl
facetjoint + hyperintenseon T2Wl (waterloss through
r/ variablesignalpattern(dueto serous,mucinous, degeneration)
gelatinousfluidcomponents,air, hemorrhage)
r/ hypointenseperimeter(= fibrouscapsulewith Herniationof NucleusPulPosus
calcium+ hemosiderin)with contrastenhancement = HNP = focalprotrusionof disk material>3 mm
NUC: beyondmarginsof adjacentvertebralendplates
SPECTimagingof vertebraecan aid in localizing secondaryto ruptureof annulusfibrosusconfined
increaseduptaketo vertebralbodies,posterior withinposteriorlongitudinalligament
elements,etc. 0 21% of asymptomatic populationhas disk herniation!
r/ eccentricallyplacedincreaseduptakeon eitherside . localsomaticspinalpain = sharp/ aching,deep,
of an intervertebralspace (osteophytes,diskogenic localized
sclerosis) . centrifugalradiatingpain= sharp,well-circumscribed,
Sequelae:(1) disk bulging "electric,"
confinedto dermatome
superficial,
(2) disk herniation . centrifugalreferredpain = dull,ill-defined,
deep or
(3) spinalstenosis achingor boring,confinedto somatome
superficial,
(4) facetjoint disease (= dermatome+ myotome+ sclerotome)

DDx: ldiopathic segmental sclerosis of vertebral body

(middle-aged / youngpatient,hemispherical
sclerosisin anteroinferioraspectof lower lumbar
vertebraewith smallosteolyticfocus,only slight
narrowingof intervertebral disk; unknowncause)

TERMINOLOGY:
1. Dis kbulge
= coficefltricsmoothcircumferential expansionof
softeneddisk materialbeyondthe confinesof
endplatesafter lengtheningof annularfibers
2. Diskprotrusion
= focal eccentricprotrusionof disk material
maintaining broadbasewith parentdisk due to Lateral Disk Herniation
focallyweakened/ rupturedannulusbut intact Nerve compression usually occurs posterolaterally (here atL4-5);
posteriorlongitudinalligament;<3 mm beyond therefore an atypical lateral compression (here of L4 root) directs
vertebralmargin surgery to the wrong more cephalad level (L3-4 disk)
 Skulland SpineDisorders 201

S it e: (4) Conjoinednerve root (2 nerve rootsarising

(a) posterolateral(49%)= wedkestpoint along from thecalsac simultaneously representing
posterolateralmarginof disk at lateralrecessof mass in ventrolateralaspectof spinalcanal;
spinalcanal(posteriorlongitudinal ligamenttightly normalvariantin 1-3/" of population)
adherentto posteriormarginsof disk) (5) Tarlovcyst (dilatednerve root sleeve)
(b) posterocentral(8%)
(c) bilateral(on both sides of posteriorligament)
(d) lateral/ foraminal(<10%) CervicalDiskHerniation
(e) intraosseous / vertical= Schmorlnode (14%) Peak age: 3rd-4th decade
(f) extraforaminal = anterior(commonlyoverlooked) . neckstiffness,musclesplinting
(2e%) . dermatomicsensoryloss
Myelography: . weakness+ muscleatrophy
r/ sharplyangularindentationon lateralaspectof . reflexloss
thecalsac with extensionabove or below levelof S i tes: C 6-7(69% );C S -G(19% );C 7-T1(10% ) ;
, disk space (ipsilateralobliqueprojectionbest view) c4-5 (2%)
r/ asymmetryof posteriordisk margin Sequelae: (1) compressionof exitingnerve rootswith
{ doublecontoursecondaryto superimposed normal pain radiatingto shoulder,arm, hand
+ abnormalside (horizontal beam lateralview) (2) cord compression(spinalstenosis
r/ narrowingof intervertebral disk space (most + massivedisk rupture)
, commonlya sign of disk degeneration)
! deviationof nerve root/ root sleeve ThoracicDiskHerniation
r/ enlargementof nerve root secondaryto edema Prevalence: 1"/"of all disk herniations
("trumpetsign") S i tes:T11112
r/ amputated/ truncatednerve root (nonfillingof root { calcificationof disk fragments+ parentdisk (frequent)
sleeve)
MR: LumbarDisk Herniation
r/ herniateddisk materialof low signalintensity . sciatica=
displacesthe posteriorlongitudinal ligamentand (1) stiffnessin back
, epidural fat of relativehigh signal intensityon Tl Wl (2) pain radiatingdown to thighI calf ttool
./ "squeezedtoothpaste"effect= hourglass- (3) paresthesia/ weakness/ reflexchanges
appearanceof herniateddisk at posteriordisk . painexaggeratedby coughing,sneezing,physical
, marginon sagittalimage activity+ worse while sitting/ straighteningof leg
! asymmetryof posteriordisk marginon axialimage Sites: L4l5 (35%) > L5/S1 (27%) > L3l4 (19%) > L2t3
(14%)> L1t2(5%)
Cx: spinalstenosis
Prognosis:
conservativetherapyreducessize of herniationby D E R MOID OF S P IN E
0-50% in 1|Y" of patients, = Ufli-/ multilocular
cystictumorlinedby squamous
50-75% in 36/" of patients, epitheliumcontainingskinappendages(hairfollicles,
75-100% in 46% of patients sweat glands,sebaceousglands)
(secondaryto growthof granulationtissue) Cause:
(a) congenitaldermalrest / focal expansionof dermal
si nus
FreeFragmentHerniation (b) acquiredfrom implantation of viabledermaltissue
= DISKSEQUESTRATION (by spinalneedlewithouttrocar)
= compf€teseparationof disk materialwithrupture Prevalence: 1"/"of spinafcord tumors
throughposteriorlongitudinal ligamentintoepidural Age at presentation: <20 years; M:F = 1:1
space May be associatedwith: dermal sinus (in 20%)
0 Missedfree fragmentsare a commoncause of failed . slowlyprogressivemyelopathy
backsurgery! . acuteonsetof chemicalmeningitis(secondaryto
r/ migrationsuperiorly/ inferiorlyaway from disk space ruptureof inflammatorycholesterolcrystalsfrom cyst
with compressionof nerve root above/ below levelof intoCSF)
disk herniation Location: lumbosacraf(60%),cauda equina(20%)
r/ Oist<materialnoted>9 mm away from intervertebral Site: extramedullary (60%),intramedullary (40%)
disk space r/ almostalwayscompletespinalblockon myelograpny
{ soft-tissuedensitywith highervaluethan thecalsac I intensityof fat
DDx: (1) Postoperativescarring(retractionof thecal r/ occasionally hypointense on TlWl + hypodenseon CT
sac to side of surgery) (secretionsfrom sweatglandswithintumor)
(2) Epiduralabscess ^/ ruOcontrastenhancement
(3) Epiduraltumor ^/ Cf myelographyfacilitatesdetection
202 HadiologyReviewManual
DIASTEMATOMYELIA
= SPLITCORD= MYELOSCHISIS
= sagittaldivisionof spinalcord intotwo hemicords,each
of whichcontainsa centralcanal,one dorsalhorn+ one
ventralhorn
Etiology: congenitalmalformationas a resultof adhesions
betweenectodermand endoderm;M:F = 1:3
Path:
(a) 2 hemicordseach coveredby layerof pia within
singlesubarachnoid space+ duralsac (60%);not
accompaniedby bony spur / fibrousband
(b) 2 hemicordseachwith its own pial,subarachnoidal +
duralsheath(40%);accompanied by fibrousband (in
/ bony spurs(in75%)
25/"), cartilaginous
Associatedwith: myelomeningocele
. hypertrichosis,
nevus,lipoma,dimple,hemangioma
overlyingthe spine (26-81%)
. clubfoot(50%)
. musclewasting,ankleweaknessin one leg
Location: lowerthoracic/ upperlumbar> upperthoracic
> cervicalspine
r/ sagittalcleft in spinalcord resultingin 2 asymmetric
hemicords
{ tne 2 hemicordsusuallyreunitecaudalto cleft
^/ occasionally2 coni medullaris
r/ eccentriccentralcanal withinboth hemicords
./ bony spurthroughcenterof spinalcanalarisingfrom
posterioraspectof centra (<50%)
./ thickenedfilumterminale>2 mm (>50%)
^/ tetheredcord (>50%)
r/ low conus medullarisbelow L2 level (>75%)
r/ defectin thecalsac on myelogram
@ Vertebrae
{ congenitalscoliosis(50-75%)
0 5% of patientswith congenitalscoliosishave
diastematomyelia
{ spinabifidaover muttiplelevels
r/ anteroposterior narrowingof vertebralbodies
r/ wideningof interpediculate distance
./ narroweddisk space with hemivertebra,butterfly
vertebra,blockvertebra
^l fusion+ thickeningof adjacentlaminae(90%)
(a) fusionto ipsilaterallaminaat adjacentlevels
(b) diagonalfusionto contralateraladjacentlamina
= intersegmental laminarfusion
Cx: progressivespinalcord dysfunction
DISKITIS
0 Mostcommonpediatricspineproblem!
Etiology:
(1) Bloodbornebacterialinvasionof vertebraeinfecting
vesselsthroughendplate
disk via communicating
0 Vertebralosteomyetitis+ diskitismay be the same
entity!
(2) lnvasiveprocedure:surgery,diskography,
myelography, chemonucleolysis
Agents:
(a) pyogenic: Staphylococcus aureus(by far most
common),Gram-negative rods (in lV drug abusers/
immunocomprom isedPatients)
(b) nonpyogenic:tuberculosis, coccidioidomycosis
0 TB has a propensityto extendbeneathlongitudinal
ligamentswith involvement of multiplevertebral
levels
Pathogenesis: infectionstarts in disk (stillvascularized
in children)/ in anteriorinferiorcornerof
vertebralbody (in adults)with spread
acrossdisk to adjacentvertebralendplate
Age peak: 6 monthsto 4 years and 10-14 years;
averageage of 6 years at presentation
. over 2-4 weeks graduallyprogressingirritability,
malaise,low-gradefever
. back lrelerredhip pain,limP
. refusalto bear weight
. elevatedsedimentationrate,WBC count often normal
Location: L314,L415, unusualaboveT9; usuallyone
disk space (occasionally 2) involved
Plainfilm (positive24weeks after onset of symptoms):
^/ decreasein disk spaceheight(earliestsign)
= intraosseous herniationof nucleuspulposusinto
vertebralbody throughweakenedendplate
{ indistinctnessof adjacentendplateswith destruction
endplatesclerosis(duringhealingphasebeginning
anywherefrom 8 weeksto 8 monthsafteronset)
bone fusion (after6 monthsto 2 years)
CT:
paravertebralinflammatorymass
^/ epiduralsoft-tissueextensionwith deformityof thecal
sac
MR (preferredmodality;93% sensitive,97% specitic,95o/o
accurate):
0 Very sensitivemodalityearly on in diseaseprocess
(especially enhancedTlWl + fat suppression)
^/ decreasedmarrowintensityon TlWl in two
contiguousvertebrae
r/ in early stage preserveddisk heightwith variable
intensityon T2Wl (oftenincreased)
./ in laterstages loss of disk heightwith increased
intensityon T2Wl
NUC (41%sensitive,g3/"specific,63%accurateon Tc-
99m MDP + Tc-99mWBC scans):
r/ positivebeforeradiographs
r/ increaseduptakein vertebralendplateadjacentto
di sk
^/ bone scan usuallypositivein adjacentvertebrae(until
age 20) secondaryto vascularsupplyvia endplates;
may be negativeafter age 20
Cx: epiduralI paravertebralabscess, kyphosis
Rx: immobilizationin body cast tor -4 weeks
DDx: neoplasticdisease(no breachof endplate,disk
space often intact)
Postoperative Diskitis
Frequency: 0.75-2.8/"
Organism; Staphylococcusaureus;many times no
organismrecovered

. severerecurrentback pain 7-28 days aftersurgery
accompaniedby decreasedback motion,muscle
spasm,positivestraightleg raisingtest
. fever (33%)
. wound infection(8%)
. persistentlyelevated/ increasingESR
MR:
r/ decreasedsignalintensitywithindisk + adjacent
vertebralbody marrowon TlWl
./ increasedsignalintensityin disk + adjacentmarrow
on T2Wl oftenwith obliterationof intranuclearcleft
{ contrast-enhancement of vertebralbone marrow+
disk space
DDx: degenerativedisk diseasetype I (no gadolinium-
enhancement of disk)
DISLOCATION OF SPINE
Atlanto-occipitalDislocation
= ATLANTO-OCCIPITAL DISTRACTION INJURY
= disruptionof tectorialmembrane+ pairedalar
ligamentsresultingin grosslyunstableinjury
Cause: rapiddecelerationwith eitherhyperextension
or hyperflexion
Age: childhood(dueto largersize of head relativeto
body, increasedlaxityof ligaments,horizontally
joint, hypoplastic
orientedoccipito-atlanto-axial
occipitalcondyles)
May be associatedwith: occipitalcondyle fracture
. neurologicsymptoms: rangefrom respiratoryarrest
withquadriplegia to normalneurologicexam
. discomfort,stiffness
Lateralradiograph:
r/ >t 0 mm soft-tissueswellinganteriorto C2
+ pathologicconvexityof soft tissues(80%)
r/ basion-densinterval(BD)>12 mm withouttraction
, placedon head/ neck
r/ basion-posterior axiallineinterval>12 mm anterior/
>4 mm posteriorto axialline
^/ gClOn ratio >1 = r?tioof distancebetweenbasion
+ posteriorarch of C1 dividedby distancebetween
opisthion+ anteriorarch of C1
Atlanto-occipital Landmarks
on Lateral Radiograph
CT:
r/ bloodin regionof tectorialmembrane+ alar
ligaments
r/ condylarfracture+ fractureextension
through
hypoglossalcanal(forcranialnerveXll)
Skulland SpineDisorders 203
{ widening/ incongruityof articulationbetween
occipitalcondyles+ lateralmassesof C1
MR:
{ ttuiOin articularcapsules,nuchalligament,
interspinous ligament
Cx: (1) lnjuryto caudalcranialnerves+ upper3
cervicalnerves
(2) Epiduralhematomawith brainstem
compression+ upperspinalcord injury
(3) Vasospasm/ dissectionof internalcarotidand
vertebralarteries
AtlantoaxialDistraction
= injuryto transverseatlantalligament,alar ligament,
tectorialmembranebetweenC1 and C2, disruptionof
articularcapsules
May be associatedwith: type 1 dens fracture
r/ prevertebralsoft-tissueswelling
r/ subluxationwith enlargementof predentalspace to
>5 mm in children<9 yearsof age
>3 mm in adults
r/ wideningof C1-C2facets
MR:
{ prevertebral,interspinous,nuchalligamentedema
r/ facetwidening/ fluid
r/ increasedsignalintensityof spinalcord
^/ + epiduralhematoma
DORSALDERMALSINUS
= epithelium-lined duraltube extendingfrom skin surface
to intracanalicularspace+ frequentlycommunicating
with CNS i its coverings
Cause: focal point of incompleteseparationof
cutaneousectodermfrom neuralectoderm
duringneurulation
Age: encounteredin early childhood-3rddecade;
M : F= 1 : 1
. smallmidlinedimple/ pinpointostium
. hyperpigmented patch lhairy nevus/ capillaryangioma
Location: lumbosacral(60%),occipital(25%),thoracic
(10%),cervical(2/"), sacrococcygeal (1%),
ventral(8%)
Course: in a craniaddirectionfrom skin leveltowardcord
(due to ascensionof cord relativeto spinalcanal
duringembryogenesis)
0 50% of dorsaldermalsinusesend in dermoid/
epidermoidcysts!
0 20-30% of dermoidcystsi dermoidtumorsare
associatedwith dermalsinustracts!
CT myelography(best modalityto defineintraspinal
anatomy):
r/ groovein uppersurfaceof spinousprocess+ lamina
of vertebra
r/ hypoplasticspinousprocess
{ singlebifidspinousprocess
r/ focal multilevelspina bifida
{ laminardefect
r/ dorsaltentingof dura + arachnoid
204 Radiology Review Manual
r/ sinusmay terminatein conusmedullaris/ filum
terminale/ nerve root/ fibrousnoduleon dorsal
aspectof cord / dermoid/ epidermoid
r/ nerve roots bounddown to capsuleof dermoid/
epidermoidcyst
r/ displacement/ compressionof cord by extramedullary
dermoids/ epidermoids
{ expansionof cord by intramedullary dermoids/
epidermoids
r/ clumpingof nerve rootsfrom adhesivearachnoiditis
Cx: (1) Meningitis(bacterialI chemical)
(2) Subcutaneous / epidural/ subdural/
subarachnoid/ subpialabscess(bacterial
ascent)
0 Dermalsinusaccountsfor up to 3% of spinal
cord abscesses!
(3) Compressionof neuralstructures
DDx: pilonidalsinus/ simplesacraldimple(no extension
to neuralstructures)
EP I DE RM O I D O F SP IN E
= cystictumor linedby a membranecomposedof
epidermalelementsof skin
Cause:
(a) congenitaldermalrest / focal expansionof dermal
s inus
(b) acquiredfrom implantationof viableepidermaltissue
(by spinalneedlewithouttrocar)
Prevalence: 1"h of spinalcord tumors
Age at presentation: 3rd-Sth decade; M > F
May be associatedwith: dermal sinus
. slowlyprogressivemyelopathy
. acute onset of chemicalmeningitis(secondaryto
ruptureof inflammatorycholesterolcrystalsfrom cyst
intoCSF)
Type II
Le Fort Fractures
Location: upperthoracic(17"/"),lower thoracic(26/"),
lumbosacral(22'/"),cauda equina (35%)
Site: extramedullary(60%),intramedullary(40%)
{ almostalwayscompletespinalblockon myelography
r/ displacementof spinalcord / nerve roots
{ smalltumorsisointense to CSF
^/ ttlOcontrastenhancement
./ Cf myelographyfacilitatesdetection
EPIDURAL OF SPINE
ABSCESS
Cause: diskitis,osteomyelitis,idiopathic
. back pain,radicularPain
. fever, leukocytosis
MR:
r/ thickeningof epiduraltissues(earlystage):
{ isointenseon TlWl
{ moderatelyhYPerintense on T2Wl
r/ liquefiedabscesscavityoval-shapedon axial images:
r/ hypointense on TlWl + hyperintenseon T2Wl
{ cellulitissurroundingabscess:
^/ best seen on with contrastenhancement(inflamed
hypervasculartissue)+ fat suppression
E P ID U R A L H E MA TOMA OF S P IN E
Etiology: (1) vertebralfracture/ dislocation(2) traumatic
lumbarpuncture(3) hypertension(4) AVM (5)
vertebralhemangioma(6) bleedingdiathesisi
/ hemophilia(7) idiopathic
anticoagulation
(45%)
Peak age: 40-50 Years
. acute radicularPain
. paraplegia
Location: thoracicspine(mostcommon)
{ compressionof posterioraspectof cord
r/ nigh attenuationlesionon CT
 Skulland SpineDisorders 205

{ iso-/ slightlyhypointenselesionon Tl Wl with marked SphenoidBone Fracture

increasein intensityon T2Wl Prevalence: involvedin 1S/" of skull-basefractures
. CSF rhinorrhea / otorrhea
FRACTURES OF SKULL . hemotympanum
1. Linearfracture(mostcommontype) . battlesign = mastoidregionecchymosis
r/ deeplyblacksharplydefinedline . raccoonelres= periorbitalecchymosis
DDx: (1) vasculargroove,esp. temporalartery(gray . 7th / 8th nerve palsy
line,slightlyscleroticmargin,branchinglike . musculardysfunction:problemswith ocularmotility,
a tree, typicallocation(temporalartery mastication, speech,swallowing, eustachiantube
projectsbehinddorsumsellae) function
(2) suture r/ air-fluidlevel in sinuses+ mastoid
2. Depressedfracture { axialthin-slicehigh-resolution CT for bestdelineation
. often palpable
of fractures
^/ bone-on-bone density r/ water-solubleintrathecalcontrastmaterialfor CSF
Rx: surgeryindicatedif depression>3-5 mm (due to fistula
arachnoidtear lbrain injury)
N.B.: CT / MR mandatoryto assessextentof TemporalBone Fracture
underlyingbraininjury Longitudinal Fracture of TemporalBone (TS%)
3. Skull-basefracture= basilar skull fracture = fractureparalleltothe axis of petrouspyramid
' rhinorrhea(CSF)
arisingin squamosaof temporalbone through
. otorrhea(CSF/ hemotympanum)
tegmentympani,EAC (externalauditorycanal),
., f?Ccoofletes = periorbitalecchymosis
middleear,terminatingin foramenlacerum
r/ pneumocephalus . bleedingfrom EAC (disruption of tympanic
r/ air in sulci membrane)
r/ air-fluidlevelin sinuses . otorrhea(CSF leak with rupturedtympanic
Cx: infection,acute/ delayedcranialnervedeficit, membrane;rare)
vascularlaceration/ dissectionI occlusion/ . conductivehearingloss (dislocationof auditory
infarction ossicles- mostcommonlyincusas the least
4. Healingskull fracture anchoredossicle)
@ infants: in 3-6 monthswithouta trace . NO neurosensoryhearingloss
@ children(5-12 years): in 12 months . facialnervepalsy (10-20%)due to edema/ fracture
@ adults: in 2-3 years of facialcanalneargeniculateganglion;frequent
r/ persistentlucencymimickingvasculargroove spontaneousrecovery
Cx: leptomeningeal cyst (= growingfracture) r/ pneumocephalus
ri herniationof temporallobe
LeFortFracture r/ incudostapedial joint dislocation(weakestjoint):
[ReneLe Fort(1869-1951), Frenchsurgeon] r/ "ice cream"(malleus)has fallenoff the ,bone"
0 All LeFortfracturesinvolvethe pterygoidprocess! (incus)on directcoronalCT scan
A. LeFort| = transverse(horizontal)maxillaryfracture r/ fractureof "molartooth"on directsagittalCT
causedby blow to premaxilla scan
Fractureline: (a) alveolarridge r/ mastoidair cells opaque/ with air-fluidlevel
(b) lateralapertureof nose Plainfilm views: Stenvers/ Owensprojection
r/detach m.,,!?jll'5j',:i ij"'
;,*::I3illTJ, TransverseFracture of TemporalBone (25%)
r/ teeth
containedin detachedfragment = fracture
perpendicular
to axisof petrous
pyramid
B. LeFortll =,,pyramidalfracture" originatingin occipitalbone extendinganteriorly
0 May be unilateral acrossthe base of skull + acrossthe petrous
Fractureline: archthrough pyramid
(a) posterioralveolarridge . irreversibleneurosensoryhearingloss (fractureline
(b) medialorbitalrim acrossapex of IAC / labyrinthinecapsulewith injury
to both parts of cranialnerveVlll)
r/separat,
". "l',],.,,X'J|ilo
lXii:::"' . persistentvertigo
r/ floor of orbit + hard palate+ nasalcavityinvolved . facial(cranialnerveVll) nervepalsy in 50% (injury
C. LeFortlll - "craniofacial disjunction" in IAC);less frequentspontaneousrecovery
Fractureline: horizontalcoursethrough becauseof disruptionof nervefibers
(a) nasofrontalsuture . rhinorrhea(CSF leak with intacttympanic
(b) maxillo-frontal
suture membrane)
(c) orbitalwall . bleedinginto middleear
Plainfilm views: posteroanterior
(transorbital)
r/separat,""
.l?.iil3?[ffitr1"."
orskurl + Towne projection
206 Radiology Review Manual

Anterior Arch Fracture PosteriorArch Fracture Lateral MassFracture

@P Jefferson Fracture

Atlas Fractures

/ \
t l
I --.i.
I \) r\,
| \\r---2
L€1'--=.---
Hangman's Fracture

Axis Fractures

'4-- A #\5
l l

/--r\ /--=-\ ,-_-\

Tlpe III
r3:r"ti.,
Dens

A
[-=-\
Os Odontoideum Ossiculum Terminale Aplasia of Dens

DensVariants

Fracture . diplopiaon upwardgaze (entrapmentof inferiorrectus

Zygomaticomaxillary
= "TRIPOD" FRACTURE = MALAR/ ZYGOMATIC COMPLEX + inferiorobliquemuscles)
FRACTURE . enophthalmos
Cause: directblow to malareminence . facialanesthesia
. loss of sensibilityof face beloworbit Associatedwith: fractureof the thin lamina papyracea
. deficientmastication (= medialorbitalwall) in 20-50%
. doublevision/ ophthalmoPlegia { soft{issue mass extendinginto maxillarysinus
. facialdeformity (= herniationof orbitalfat)
Fractureline: r/ completeopacificationof maxillarysinus (edema
(a) lateralwall of maxillarysinus + hemorrhage)
(b) orbitalrim closeto infraorbitalforamen .'/ depressionof orbitalfloor (= orbitalprocessof maxilla)
suture/
(c) floor of orbit (d) zygomatico-frontal { posttraumaticatrophyof orbitalfat leadsto
zygomaticarch enophthalmos
{ opacificationof adjacentethmoidair cells
BlowoutFracture r/ disruptionof lacrimalduct
= isolatedfractureof orbitalfloor
Cause: suddendirectblow to globe (ballor fist) with FRACTURES OF CERVICAL SPINE
increasein intraorbitalpressuretransmittedto Factors associatedwith higher risk of fracture:
the weak orbitalfloor (1) Gl asgowC omaS core< 14
 Skulland SpineDisorders 207

(2) Neck tenderness 5. Bilateral facet lock = interlockingof articular

(3) Lossof consciousness surfaces(unstable)
(4) Neurologicdeficit r/ anterolisthesis of affectedvertebraby 112
(5) Drugingestion vertebralbody width
(6) Specificmechanismof injury: motorvehicle r/ mildfocalkyphosis
accident,fall from a height>3 m { softtissue swelling
lndications for screening CT of cervical spine: r/ no rotation
high-riskadult patients(= >So/"pretestprobabilityof 6. Anteriordisk space narrowing
injury)definedby: 7. Spinousprocessfracture= clay shoveler'sfracture
(1) High-speed(>35 mph) motorvehicleaccident = suddenloadon flexedspinewith avulsion
(2) Crash resultingin death at scene of accident fractureof C6 / C7 tT1 (stabte)
(3) Fallfrom height>3 m (10 feet) 8. Flexioninstability= isolatedruptureof posterior
(4) Significantclosedhead injury(intracranial ligaments
hemorrhageseen on CT) 0 Dx may be missedwithoutdelayedflexionviews
(5) Neurologicsigns/ symptomsreferredto C-spine r/ no fracture
(6) Pelvic/ multipleextremityfractures r/ interspinous widening
r/ loss of facet paralleliim
Frequency: 1-3% of all trauma cases; r/ wideningof posteriorportionof disk
c2, c6 > c5, C7 > C3, C4 > C1 r/ anterolisthesis >3 mm
0 Cervicalspinetraumaaccountstor 213ot ^/ focal kyphosis
all spinalcord injuries!
. neurologic/ spinalcord damage(39-50%) B. HYPEREXTENSTON tNJURy (20-38/")
Location: 0 Highriskfor neurologicdeficit!
(a) uppercervicalspine= C112(19-25%): 0 Radiographs may be completelynormal!
ailas (4%),odontoid(6%) 1. Anteriorlywideneddisk space
(b) lowercervicalspine= C3-7 (75-Bt%) 2. Prevertebral swelling
(c) cervicothoracic junction(9-1g%) 3. Extension teardrop fracture
(d) multiplenoncontiguousspine fractures(15-20%) Location: C2, C3
Site: vertebralarch (50%),vertebralbody (gO%), 4. Neuralarch fractureof C1 (stable= anteriorring
intervertebraldisk (25%),posteriorligaments(16%), + transverseligamentintact)
dens (14%),lockedfacets(12/"), anteriorligament 5. Subluxation(anterior/ posterior)
(2%) 6. Hangman's fracture (unstable)
Associated with injury to: = TRAUMATIC SPONDYLOLISTHESIS
head (70/"),thoracicspine(1Eyo),lumbar spine(10%), ^/ bilateralpars fractureof C2
thorax(35%),pelvis(15%),upperextremity(1O/"),
lowerextremity(30%)
N.B.: 5-l8%of patientswith fracturesmay have normal
radiographs!
0 Most missedfracturesinvolveC1 (g%),CZ (94%),
C4 (12/"), CG-7(14%),occipitalcondyles!
0 C711 space not visualizedin at least 26/" of all
traumapatients
Cx: neurologicdeterioration with delayin diagnosis
TeardropFracture Flexion Instability Unilateral Facet Lock
A. HYPERFLEXTON TNJURY(46_79%)
1. Odontoidfracture
2. Simplewedge fracture(stable)
3. Flexion teardrop fracture = avulsionof
anteroinferior cornerby anteriorligament(unstable)
0 Most severe+ unstableinjuryof C-spine
Location: C5, CG,C7
r/ triangularfragmentin soft tissuesanteriorto
vertebralbody
r/ retrolisthesis
{ wideningof facets
r/ narrowingof spinalcanal
r/ mild kyphosis
Associatedwith: ligamentoustears,spinalcord
compresston Bilateral Facet Lockk Hyperextension Dislocation
4. Anteriorsubluxation Spine Injury
208 Radiology Review Manual
./ prevertebralsoft-tissueswelling>5 mm at
marginof C2
anterior-inferior
r/ anteriorsubluxationof C2 on C3:
r/ disruptionof C1-C2 spinolaminar line
r/ disruptionof C2-C3 posteriorvertebralbody
line
cornerof C2 (ruptureof
r/ avulsionof anteroinferior
anteriorlongitudinalligament) Atlas Fracture
c. FLEXION-ROTATION INJURY(12%)
1. Unilateralfacet lock (obliqueviews!,stable)
{ anterolisthesis <l14verilebralbody width
r/ "bow-tie"sign = the 4 rotatedfacetson LAT view
^/ decreasein spinolaminar space
r/ rotationof spinousprocess(on AP view) Axis Fracture
! "nakedfacet"(on CT)
D. V E RT I CA LCO MP R E SS IO N (4 % )
1. Jefferson fracture
[SirGeofrey Jefferson (1886-1961), neurosurgeon in
Manchester, England]
r/ comminutedburstfractureof ringof C1 (unstable)
with uni-/ bilateralipsilateralanterior+ posterior
fractures
r/ lateraldisplacementof lateralmasses(self-
decompressing) on AP view
(DDx: Pseudo-Jefferson fracture= lateraloffsetof
lateralmassesof atlaswithoutfracturein fusion FRACTURES
anomaliesof anterior/ posteriorarchesof C1, in
childrenas lateralmassesof atlas ossifyearlier
than C2)
2. Burst fracture = intervertebral disk driveninto
vertebralbody below (stable)
./ loss of posteriorvertebralbody heightwith
severalfragments:
r/ sagittalfracturecomponentextendingto
inferiorendplate
r/ retropulsedfragmentfrom posteriorsuperior
marginin spinalcanal
^/ interpedicularwidening
{ posteriorelementfracture
Associatedwith: wideningof apophysealjoints,
fractureof posteriorvertebral
arches
E. LATERALFLEXION/ SHEARING(4-6%)
1. Uncinatefracture
2. lsolatedpillarfracture
3. Transverseprocessfracture
4. LateralvertebralcomPression
SignificantSignsof CervicalVertebralTrauma
(a) most reliable+ specific:
r/ wideningof interspinousspace (43%)
{ wideningof facetjoint (39%)
r/ displacementof prevertebralfat stripe(18%)
(b) reliablebut nonspecific:
r/ wide retropharyngeal space >7 mm (31%)
(DDx: mediastinal hemorrhageof othercause,
cryingin children,S/P difficultintubation)
(c) nonspecific:
ri loss of lordosis(63%)
r/ anterolisthesis/ retrolisthesis(36%)
r/ kyphoticangulation(21%)
./ trachealdeviation(13%)
ri Oist<space: narrow(24%),wide (8%)
Prevalence: 4ohof cervicalspine injuries
Site: posteriorarch,anteriorarch,massalateralis,
Jeffersonfracture
Associatedwith: fracturesof C7 (25/"), C2 pedicle
fractures(58%)
(15/.), extraspinal
Prevalence: 6/. of cervicalspine injuries
Associatedwith: fracturesof C1 in 8"/"
Type | = avulsionof tip of odontoid(5-8%)
{ difficultto detect
Type ll = fracturethroughbase of dens (54-67%)
Cx: nonunion
0 AxialCT alonemisses>50%!
Type lll = subdentalfracture(30-33%)
Prognosis; good
DDx: os odontoideum, ossiculumterminale,hypoplasia
of dens,aPlasiaof dens
SPINE
OFTHORACOLUMBAR
O 40%of all vertebralfracturesthat cause neurologic
deficit;mostlycomplex(body+ posteriorelements
involved)
Location: 213atthoracolumbar junction
r/ diastasisof apophysealjoints
ligament
r/ disruptionof interspinal
{ retropulsion of bodyfragmentsintospinalcanal
r/ "burst"fragmentsat superiorsurfaceof body
Fractureof UpperThoracicSpine(T1to T10)
Frequency; in 3h of all blunt chesttrauma
Types:
1. compression / axialloadingfracture(most
common)
ri wedgingof vertebralbodY
ri retropulsion of bonefragments
r/ posttraumaticdisk herniation
2. burstfracture
i associatedfractureof posteriorneuralarch
^/ comminutedretropulsedbone fragments
3. sagittalslice fracture
r/ vertebraabovetelescopesinto vertebrabelow,
disPlacingit laterallY
4. anterior/ posteriordislocation
ligament
r/ torn anterior/ posteriorlongitudinal
r/ facet dislocation
0 Relativelystablefracturesdue to rib cage + strong
costovertebralligaments+ more horizontalorientation
of facetjoints!
0 Only51% detectedon initialCXR!
Often associatedwith: fractureof sternum
r/ wideningof paraspinallines

r/ mediastinal widening
{ loss of heightof vertebralbody
r/ obscurationof pedicle
r/ tett apical cap
ri deviationof nasogastrictube
Signsof spinalinstability:
= inabilityto maintainnormalassociations between
vertebralsegmentswhileunderphysiologic load
r/ displacedvertebra
r/ wideningof interspinous / interlaminardistance
r/ facet dislocation
r/ disruptionof posteriorvertebralbody line
Fractureof Thoracolumbar
Junction(T11to L2)
= €ue€lof transitionbetweena stiff + mobilesegmentof
the spine
. neurologicdeficit(in up to 40%)
Classificationbased on injury to the middte column:
(1) Hyperflexion injury(mostcommon)
= coffipressionof anteriorcolumn+ distractionof
posteriorspinalelements
(a) hyperflexion-compression fracture
ri loss of heightof vertebralbody anteriorly
+ laterally
r/ focal kyphosis/ scoliosis
r/ fractureof anterosuperior endplate
(b) flexion-rotation injury(unusual)
0 Very unstable!
. catastrophic neurologicsequelae:
paraplegia
r/ subluxation / dislocation
r/ wideningof interspinous distance
{ fracturesof lamina,transverseprocess,
facets,adjacentribs
(c) shearingf racture-dislocation
= damageof all 3 columnssecondaryto
horizontallyimpactingforce
(d) flexion-distraction injury: Chancefracture
2. fyperextensioninjury(extremelyuncommon)
r/ wideneddisk space anteriorly
{ posteriorsubluxation
r/ vertebralanteriorsuperiorcorneravulsion
{ posteriorarch fracture
3. Axial compressionfracture
0 Unstable!
r/ burstfracturewith herniationof intervertebral
disk throughendplates+ comminution of
vertebralbody
r/ markedanteriorvertebralbody wedging
r/ retropulsedbone fragment
r/ increasein interpediculate distance
r/ + verticalfracturethroughvertebralbody,
pedicle,lamina
ChanceFracture
= SEATBELTFRACTURE
[GeorgeQuentinChance,Britishradiologistin Manchester,
Englandl
Skulland SpineDisorders 209
Mechanism: shearingflexion-distraction injury(lap-
type seatbeltinjuryin back-seat
passengers)
. neurologicdeficitinfrequent(20%)
Location: L2 or L3
r/ horizontalsplittingof spinousprocess,pedicles,
laminae+ superiorportionof vertebralbody
r/ disruptionof ligaments
{ distractionof intervertebral disk + facetjoints
0 Fractureoftenunstable!
Often associated with:
(1) otherbonei nj ury
rib fracturesalongthe courseof diagonalstrap;
sternalfractures;clavicularfractures
(2) soft-tissueinjury
transversetear of rectusabdominismuscle;
anteriorperitonealtear;diaphragmatic rupture
(3) vascularinjury
mesentericvasculartear;transectionof common
carotidartery;injuryto internalcarotidartery,
subclavianartery,superiorvena cava; thoracic
aortictear; abdominalaortictransection
(4) visceralinjury
perforation of jejunum+ ileum> largeintestine>
duodenum(freeintraperitoneal fluidin 1OO/",
mesentericinfiltrationin 88%,thickenedbowel
wall in 75/", extraluminalair in 56%);laceration /
ruptureof liver,spleen,kidneys,pancreas,
distendedurinarybladder;uterineinjury
ChanceEquivalent
= purelyligamentous disruptionleadingto lumbar
subluxation/ dislocation
{ mildwideningof posterioraspectof affecteddisk
space
r/ widenedfacetjoints
r/ splayingof spinousprocesses- "emptyholesign"
on AP view
HoldsworthFracture
[SirFrankWildHoldsworth (1904-1969), pioneering
British
orthopedist
in rehabilitation
of spinatinjuriesl
Location: thoracolumbarjunction
{ unstablespinalcolumnfracture-dislocation with
fracturethroughvertebralbody + articularprocesses
{ ruptureof posteriorspinalligaments
SeatbeltInjury
= injurycausedby three-pointrestrainttype (combined
lap and shoulderbeltdevice)
. bruisein subcutaneous tissue+ fat of anteriorchest
wall
. skin abrasionsare associatedwith significantinternal
injuries(in 30%)
@ Skeleton
sternum,ribs (alongdiagonalcourseof shoulder
harness),clavicle,transverseprocessesof C7 or T1
@ Cardiovascular
aortictransection,cardiaccontusion,ventricular
rupture,subclavianartery,SVC
210 RadiologyReview Manual

@ Airways MR:
rupture
tracheal/ laryngealtear,diaphragmatic r/ usuallyhomogeneous extensiveill-definedcord
tumorwith wideningof sPinalcord:
Transverse Process Fracture of Lumbar Spine r/ iso-/ hypointenseto cord on TlWl
Cause: directtrauma,violentlateralflexion-extension r/ hyperintenseon T2Wl
forces,avulsionof psoasmuscle,Malgaigne rl poorlydefinedmargins
fracture ri eccentriclocationwithinspinal cord (57'/")
Frequency: 7"/" r/ ditatedveins on surfaceof cord
ln 21-51lo associatediniury: r/ patchyirregularGd enhancement on MR
genitourinary injury,hepatic+ spleniclaceration { eccentricirregulartumor cysts + polar cysts +
Location:L3 > L2 > L1 > L4 > L5; L:R = 2:1; syrinx(common):
multiple:single = 20:1
= 2:1; unilateral:bilateral ./ water-solublemyelographiccontrastenterscystic
r/ vertical:horizontalpa%:6%) fractures space on delayedCT images
{ associatedlumbarburst/ compressionfracture ri leptomeningeal spread(in 60% of glioblastoma
0 Detectionby conventional radiography in 40% only! multiforme)
Prognosis; minor and stableinjury;10% mortatity Rx: tumor debutking+ radiationtherapy
Prognosis: 95/" S-yearsurvivalin low-gradetumors;
SacralFracture highermortalityratethan for ependymoma
Zone 1 = fracturelateralto sacralforamina DDx: ependymoma(capsign,centrallocation,well
. significantneurologicdeficit(uncommon) defined,hemorrhagecommon,focalintense
= enhancement, predilectionfor conus)
Zone 2 fracturethrough21 foramina
. unilaterallumbar/ sacralradiculopathy
(rare)
Zone 3 = fracturethroughcentralcanal
. significantbilateralneurologicdamage of SpinalCord
Ependymoma
(frequent):bowel/ bladderincontinence 0 Mostcommonintramedullary spinalneoplasmin adults!
Cx: chronicdisability(in up to 50%) Frequency: 40-60/" of primary spinal cord tumors; 90%
of primarytumorsin the filumterminale
Mean age: 39 Years;M:F = 57:43
GLI O M A O F S P IN AL C O R D Origin: ependymalcells liningthe centralcanal (62-
Astrocytomaof SpinalCord 76%)
0 Mostcommonintramedullary spinalneoplasmin Path: symmetriccord expansionwith displacementof
c hildr en! neuraltissue
Frequency: 30/" of spinalcord tumors;2nd in Histo: perivascularpseudorosettes; cystic
prevalenceto ependymomain adults degeneration (50%);hemorrhageat superior
Mean age: 29 years; M:F = 58:42 + inferiortumormargins
Path: ill-defined fusiformcord enlargement without Subtypes: cellular(mostcommon),papillary,
cleavageplane/ caPsule clearcell,tanycytic,myxopapillary
Histo: hypercellularitywith infiltrativegrowthalong (alongfilumterminale),melanotic
the scaffoldof normalastrocYtes, Location: cervical cord alone (44%)/ with extension
oligodendrocytes and axons; into thoraciccord (23%);thoraciccord
Grade I pilocyticastrocytoma(75o/o), usually alone(26%);conusmedullaris(7%);
mostcommonin cerebellum extendsover severalvertebralsegments
Gradell fibrillarytype (on average3.6 segmentsinvolved)
Grade lll anaplasticastrocytomawith ectopic: sacrococcygealregion,broad
necrosis(up to 25%) ligamentof ovary (associated
GradelV glioblastoma multiformewith with spinabifidaocculta[33%])
endothelialproliferation(0.2-1.5%) . long antecedenthistory(meandurationof 37 months):
Location: thoraciccord (67"/"),cervicalcord (49%), ' back/ neck pain (67"/")= comPression/ interruption
conusmedullaris(3%);on averageover 7 of centralspinothalamictractsfirst
vertebralsegmentsinvolved;holocord . sensorydeficits(52/"), motorweakness(46%)
presentation (in up to 60% in children); . bowel/ bladderdysfunction(15%)
often extendinginto lower brainstem Metastasesto: lung, retroperitoneum, lymph nodes
. pain + sensorydeficit(54%)
. motordysfunction(41%) { well-demarcated / diffuselyinfiltratingcord tumor
. gait abnormalities(27%) r/ associatedwith at leastone cyst (in78-84%):
. torticollis(27%) r/ polar cysts (62/")
Radiographs: ^/ tumoral cysts (4-50%)
! scoliosis(24%) r/ syringohydromyelia (9-50%)
r/ widenedinterpedicular distance Radiographs:
^/ bone erosion r/ scoliosis(16%)
 Skull and Spine Disorders Zl l

r/ wideningof spinalcanal(11%): Prevalence:13Y" of all spinalependymomas; most

r/ scallopingof vertebralbody commonneoplasmof conusmedullaris
r/ pedicleerosion,taminarthinning (83%)
Myelography: Mean age: 35 years;M>F
r/ enlargedcord with complete/ partialblockto flow of Origin: ependymalglia of filumterminale
contrastmaterial Path: heterogenous tumorwith generousmucin
CT: production
r/ iso- / slightlyhyperattenuating
cord mass . lowerback / leg / sacralpain
I intenseenhancement . weaknessi sphincterdysfunction
MR: Location: conusmedullaris, filumterminale;
./ iso-/ hypointense(rarelyhyperintensefrom occasionallymultiple(1443%)
hemorrhage)mass relativeto spinalcord on Tl Wl r/ isointenseon TlWi + hyperintense on T2Wl
r/ hyper-/ isointenseon T2Wl ./ occasionallyhyperintense on Tl Wl + T2Wl due to
r/ "capsign"= extremelyhypointense rim at the tumor mucincontenti hemorrhage
, poles on T2Wl (in 20-53%) due to hemorrhage r/ almostalwayscontrastenhancing
r/ cord edema (60%) r/ occasionallylarge lyticarea of bone destruction
r/ mostlyintensehomogeneous enhancement on Gd-
enhancedMR (84%) Subependymoma
of SpinalCord
r/ well-definedmarginson contrast-enhanced images = variantof CNS ependymoma
(8e%) Origin: tanycytesltanyos, Greek = stretchlthat
Prognosis: 82/" S-yearsurvival rate bridgepial + ependymallayers
DDx: astrocytoma(pediatrictumor,eccentriclocatron, Mean age: 42years; M:F = 74:26
ill defined,hemorrhageuncommon,patchy Histo: sparselydispersedependymalcells among
irregularenhancement) predominantfibrillarastrocytes
. 52 monthsmeandurationof symptoms:
. pain,sensory+ motordysfunction
Ganglioglioma of Spinal Cord . atrophyof one / both distalupperextremities(93%)
= GANGLIOGLIONEUROMA = GANGLIONIC NEUROMA Location: ventricularsystemof brain,some in cervical
= NEUROASTROCYTOMA = T6UPOGANGLIOMA cord
= GANGLIONIC GLIOMA = = IlEgPOGLIOMA r/ fusiformdilatationof spinalcord:
= NEUROMA GANGLIOCELLULARE r/ enhancinglesionwith well-definedborders(SO%)
Prevalence: 0.4-6.2% of all CNS tumors;1.1"hof all r/ nonenhancing lesionwith diffusesymmetriccord
spinalneoplasms enlargement
Mean age: 19 years;children> adults;M:F = 1:1 { eccentricallylocatedmass
Histo: mixtureof irregularlyorientedneoplastic ./ + edema
matureneuronalelements(neurons/ ganglion
cells)+ glialelements(neoplastic astrocytes),
arrangedin clusters= gradeI or ll lesions H E MA N GIOB LA S TOMA OF S P IN E
Location: cervicalcord (48/r), thoraciccord(22/"), = ANGIOBLASTOMA = ANGIORETICULOMA
conus,holocord;usuallysupratentorial Prevalence: 1-7.2% of all spinalcord tumors;mostly
(temporallobe) sporadic
., durationof symptomsbetween1 monthand 5 years
Associatedwith: von Hippel-Lindau disease(in 1/3)
! scoliosis(44%),spinalremodeling(93%) Recommendation: screeningMR imagingof brain +
r/ eccentric
spinein patientswithvon Hippel-
r/ smalltumoralcysts (in 46%) Lindausyndrome
r/ calcifications(rarecomparedwith intracranialtumor)
A ge: mi ddl eage; M:F = 1:1
MB: Path: highlyvasculardiscretenodularmassesabutting
r/ mixedtumorsignalintensities on Tl Wl (in g4%) leptomeninges with prominentdilated+ tortuous
^/ tumorhomogeneously hyperintense on T2Wl vesselson posteriorcord surface
./ surrounding edema(lesscommon) Histo: large pale stromalcells packedbetweenblood
r/ patchytumor enhancement(65%),no vesselsof varyingsizes
enhancement(15%) Location: intramedullary(75%),radicular(20./.),
r/ enhancementof pial surface(5g%) intradural extramedullary (S%); thoracic cord
Cx: malignanttransformation (10%) (50%),cervicalcord (40%);solitaryin >907o,
Prognosis.' slow growth; 89% S-yearand gg% 10-year multiplelesionsindicatevon Hippel-Lindau
survivalrate ; 27"/" recunencerate syndrome
. meandurationof symptomsis 38 months:
. sensorychanges(39%): impairedproprioception
MyxopapillaryEpendymomaof SpinatCord . motordysfunction(31"/"),pain (31%)
= specialvariantof ependymomaof lowerspinalcord ,
ri increasedinterpediculate distance(masseffect)
212 RadiologyReviewManual

Angio: ./ gas collectionincreasingwith extension+ traction,

r/ frignryvascularmass with dense prolongedblush
./ largedrainingveinsform sinuousmassalong
posterioraspectof cord
MR:
LEPTOMENINGEAL
r/ iso- (50%)/ hyperintense(25%)diffusecord
expansionon TlWl
{ hyperintenselesionwith intermixedfocal tlow voids
on T2Wl:
r/ curvilinearareaof signalvoid
! cystformation/ syringohydromyelia (in up to 100%):
{ intratumoralcystic component(50-60%)
{ occasionallycystic mass with enhancingmural
nodule(CLASSICfor cerebellarhemangioblastoma)
{ denselystainingtumornodule
rl + surrounding edemaand cap sign
r/ well-demarcated Gd-enhancingmass
Cx: intramedullary hemorrhage, hematomyelia,
subarachnoid hemorrhage(rare)
DDx: arteriovenousfistula(not well circumscribed,
heterogenoussignalintensitY)
decreasingwith flexion
CYST
= ,,Growing" fracture= loculationof csF into/ throughskull
Prevalence: 1"h of all pediatricskullfractures
Pathogenesis;skull fracturewith duraltear leads to
arachnoidherniationinto dural defect;
CSF pulsationsproducefracturediastasis
+ erosionof bone margins(apparent2-3
monthsafter injurY)
{ skull defectwith indistinctscallopedmargins
r/ CSf-Oensitycyst adjacentto / in skull,may contain
cerebraltissue
MR:
{ cyst isointensewith CSF + communicating with
subarachnoidsPace
r/ area of encephalomalacia underlyingfracture
(frequent)
r/ intracranialtissueextendingbetweenedges of bone

KLIPPEL.FEIL SYNDROME LIPOMA OF SPINE

= BREVICOLLIS = partiallyencapsulatedmass of fat + connectivetissuein
= syflostosisof two / more cervicalsegments continuitywith leptomeninges / spinalcord
May be associated with: . skin-coatedsubcutaneousback mass,occasionally
platybasia,syringomyelia,encephalocele, facial associatedwith hemangiomatous / hairy lesion
+ cranialasymmetry,Sprengeldeformity(2540Y"), . selsory deficiency,paresis,neurogenicbladder
syndactyly,clubbedfoot, hypoplasticlumbarvertebrae; Types:
renalanomaliesin 50% (agenesis,dysgenesis, (a) lipomyelomeningocele (84%)
malrotation,duplication,renal ectopia);congenitalheart (b) fibrolipoma of filumterminale(12%)
disease in 5o/o(atrialseptal defect, coarctation) (c) intraduralliPoma(4%)
. c linic alt r iad
of Location: lumbosacralregion
(1) shortneck 0 Intradurallipomas+ lipomyelomeningoceles represent
(2) restr,ictionof cervicalmotion 35/" of skin-coveredlumbosacralmasses + 20-50/" ot
(3) low posteriorhairline occultspinaldysraPhism !
. deafness(30%)
. webbed neck lntraduralLipoma
Location: cervicalsPine = subpialjuxtamedullary masstotallyenclosedin intact
./ fusionof vertebralbodiesand posteriorelements dural sac
r/ t hemivertebrae Prevalence: <1"/"of primaryintraspinaltumors
{ may have cervicothoracic/ cervical/ atlanto-occipital Age peaks; first 5 years of life (24'/"\,2nd + 3rd
fusion decade(55%),Sthdecade(16%)
r/ torticollis . slow ascendingmono-/ paraparesis,spasticity,
r/ bcoliosis cutaneoussensoryloss,defectivedeep sensation
{ rib fusion (withcervical+ thoracicintradurallipoma)
./ Sprengeldeformity(2540%\ . flaccidparalysisof legs,sphincterdysfunction(with
r/ ear anomalies:absentauditorycanal, microtia, lumbosacral intraduralliPoma)
deformedossicles,underdevelopment of bony labyrinth . overlyingskin most often normal
. elevationof proteinin CSF (30%)
Location: cervical(12%)/cervicothoracic(24/") I
TUUUEILDISEASE thoracic(30%);dorsalaspectof cord (75V"),
vacuumphenomenon
= intravertebral lateral/ anterolateral(25%)
Cause: 1. Osteonecrosis { spinalcord open in midlinedorsally
2. Weeksto monthsfollowingacutefracture r/ lipomain openingbetweenlipsof placode
Pathophysiology: likely to representgaseous release r/ exophyticcomponentat upper/ lower pole of lipoma
into bony cleftswithina nonhealed { syringohydromyelia (2%)
fractureunderneathendPlate r/ focalenlargement of spinalcanal+ adjacentneural
Age: >50 years foramina
Location: most commonlyat thoracolumbarjunction ./ narrowlocalizedspina bifida
 Skulland SpineDisorders 213

Lipomyelomeningocele Prognosis; spontaneousregressionwithinfirst 6 months

= lipomatightlyattachedto exposeddorsalsurfaceof of life
neuralplacodeblendingwith subcutaneousfat DDx: (1) Convolutional = "digital"markings
impressions
Prevalence: 20% of skin-coveredlumbosacralmasses; (visibleat 2 years,maximallyapparentat 4
up to 50% of occultspinaldysraphism years, disappearby I years of age)
Age: typically<6 monthsof age; M < F (2) "Hammeredsilver"appearanceof increased
. semifluctuantlumbosacralmass with overlyingskin intracranialpressure
intact
. sensoryloss in sacraldermatomes,motor loss, LYMPHOMA OF SPINAL CORD
bladderdysfunction Prevalence: 3.3% of CNS lymphoma,1% of all
. foot deformities,leg pain lymphomas
Location: lumbosacral; longitudinalextensionover Mean age: 47 years; M<F
entirelengthof spinalcanal (in 7%) Histo: monotonouscollectionof lymphocytespacked
r/ lipomadorsallycontinuouswith subcutaneousfat tightlyinto perivascularspace;predominantlyB-
{ lipomamay entercentralcanal and extendrostrally cell lymphocytepopulation
(= "intradural intramedullarylipoma") . weakness,numbness,progressivedifficultyin
r/ lipomamay extendupwardwithinspinalcanal ambulation
externalto dura (= "€pidurallipoma") Location: cervical> thoracic> lumbarcord
^/ deformedundulatingspinalcord with dorsalcleft { mostlysolitary,rarelymulticentric
r/ tethered cord I isointenserelativeto cord on TlWl
r/ ventral + dorsal nerve roots leave neural placode r/ nign signalintensityon T2Wl (in contrastto
, ventrally characteristiclow signalintensityof intracraniallesions)
! dilatedsubarachnoidspace ./ extensivecord edema
US: V hetero-/ homogeneousenhancement
r/ echogenicintraspinalmass adjacentto deformed
spinalcord + continuouswith slightfyhypoechoic MENINGEAL CYST
subcutaneousfat = abnormaldilatationof meningeswithinsacral canalI
@ Vertebralchanges foramina
r/ largespinalcanal Prevalence: 5%
r/ erosionof vertebralbody + pedicles { remodelingerosionof sacralcanal/ foramen(due to
r/ posteriorscallopingof vertebralbodies(50%)
r/ focal spina bifida , CSF pulsations)
! thinnedcorticalmargins
{ segmentalanomalies/ butterflyvertebra(up to
43%) PerineuralSacralCyst / TarlovCyst
r/ confluentsacralforamina/ partialsacralagenesis = dilatednerve-root
sleeveas normalvariant
(up to s0%)
Location: posteriorrootlets(S2+ 53 mostcommon)
. neurologicsymptoms if large
Fibrolipomaof FilumTerminale r/ cystcommunicates freelywithsubarachnoid
space
Prevalence: 6% of autopsies
. asymptomatic
Sacral Meningeal/ Arachnoid Cyst (tesscommon)
Location: intradural
filum,extraduralfilum, = OCCULTINTRASACRAL
MENINGOCELE
involvementof both portions . usuallyasymptomatic
Prognosis; potentialfor developmentof symptomsof r/ cyst does not communicatewith subarachnoidspace
tetheredcord

IUcxeTScHADEL ME N IN GIOMA OF S P IN E
= CBANIOLACUNIA = LACUNAR SKULL Prevalence: 25-45% of all spine tumors; 2-3/" ot
= rTr€seflchymal dysplasiaof calvarialossification pediatricspinaltumors;12o/oof all
(developmental disturbance) meningiomas
Age: presentat birth Age: >40 years + female(80%)
Associatedwith: (1) meningocelei myelomeningocele Location: thoracicregion(82%);cervicalspine on
(2) encephalocele (3) spinabifida anteriorcord surfacenear foramenmagnum
(4) cleft palate(5) Arnold-Chiarill (2nd mostcommonlocation);90% on lateral
malformation aspect
. normalintracranialpressure Site: intraduralextramedullary (50%);entirelyepidural;
Location: particularlyupperparietalarea intradural+ epidural
r/ honeycombedappearanceabout 2 cm in diameter ', spinalcord / nerveroot compression
, (thinningof diploicspace) r/ bone erosionin <10o/"
r/ prematureclosureof sutures(turricephaly / { scallopingof posterioraspectof vertebralbody
scaphocephaly) { wideningof interpedicular distance
214 RadiologyReview Manual

r/ enlargementof intervertebralforamen ./ nignsignalintensityon T2Wl (reflecting

edema/
{ may calcify(notas readilyas intracranial meningioma) tumorinfiltration)
CT: ^/ intensehomogeneous enhancement
r/ solidsmoothlymarginatedmass isodenseto skeletal largeamountof surrounding
r/ disproportionately
muscle edema
r/ markedenhancement Prognosis: 66h die within 6 months
MR: Rx: radiationtherapy,corticosteroids
r/ isointenseto gray matteron Tl Wl + T2Wl
{ rapid+ dense enhancementafter Gd-DTPA IntraduralTumorSeeding
DDx: nervesheathtumor ^/ focalnodularmassesvaryingsubstantially in size
froma few mm to > 10 mm
TO VERTEBRA
METASTASES { enlargedcord (fromdiffusecoatingof outerwall of
Source: lesion
spinalcord)simulatingan intramedullary
(a) Metastatictumors: lung, breast,prostate,kidney, r/ thickeningof meninges
lymphoma,malignantmelanoma ./ thickened+ nodularmattednerve roots
(b) Primarytumor: multiplemyeloma { nodular+ irregularlynarrowedthecalsac
Pathogenesis;hematogenousspreadto vertebralbodies ^/ CO-ofPA enhancement
(boneswith greatestvascularity)
MR: Metastasesfrom OutsideCwS
r/ patchymultifocalrelativelywell-definedlesions (a) with subarachnoidhemorrhage:malignant
./ diminishedsignalon TlWl on backgroundof high- melanoma,choriocarcinoma, hypernephroma,
signalappearanceof marrowfat bronchogeniccarcinoma
r/ increasedsignalon T2Wl (exceptfor blastic (b) others:breast,lymPhoma
metastaseswith diminishedT1 +T2 signals) { predominantly dorsallocation
! contrastenhancementon Tl Wl (majority)
{ pathologiccompressionfracture: Drop Metastases
ri fractureonly after all vertebralbody fat replaced = CSFSEEDING OF INTRACRANIAL NEOPLASMS
r/ hyperintense on diffusion-weighted images(DDx: Age: occursmore frequentlyin pediatricage group
hypointensebenign f racture) than in adults
DDx: (1) Infection(centeredarounddisk space) Location: lumbosacral + dorsalthoracicspine(due
(2) Primaryvertebraltumor (rare in older patients, to CSF flow / gravitation)
almostalwaysbenignin patients<21 yearsof Site: on spinalarachnoid/ pia mater
age) CNS tumors causing drop metasfases;
1. Primitiveneuroectodermal tumor
2. Medulloblastoma: uP to 33%
METASTASES TO SPINALCORD 3. Anaplasticglioma
Intramedullary Metastasis 4. Ependymoma:afterlocalrecurrence, more
Prevalence: 0.9-2.1% (autoptic) commonin infra-than supratentorial
Origin: lung (40-857o),breast(117o),melanoma ependymomas
(5%),renalcell (4/"), colorectal(3/"), 5. Germi noma
lymphoma(3%),cerebellarmedulloblastoma; 6. Pineoblastoma, PineocYtoma
5/" of unknownorigin Lesscommon: choroidplexuscarcinoma,
Spread: teratoma,angioblastic
(a) common: hematogenous (viaarterialsupply)/ meni ngi oma
directextensionfrom leptomeninges mnemonic.'"MEGOTP"
(b) rare: disseminationalongcentralcanal/ Medulloblastoma
extensionalong Batsonvenousplexusfrom Ependymoma
retroperitoneal primarytumor/ extensionalong Glioblastoma multiforme
perineurallymphaticducts Oligodendroglioma
. s y m pt om atifo c r < 1 m o n th(i n 7 5 % ): Teratoma
. motorweakness,bowel/ btadderdysfunction(60%) Pineoblastoma, PNET
. pain (70%),paresthesia(50%)
Location: cervical(45/"),thoracic(35%),lumbarcord
(8%) MYELOCYSTOCELE
Myelography(up to 40/" undetected) = SYRINGOCELE
MR: = hydromyelicspinalcord + arachnoidherniatedthrough
{ mild cord expansionover severalsegments posteriorspina bifida;leastcommonform of spinal
(averagelengthof 2-3 vertebralsegments) dysraphism
{ centralareaof low signalintensity(mimicking May be associatedwith: Gl tract anomalies,GU tract
s y r inx )on T l Wl anomalies

. cysticskin-coveredmass over spine
. cloacalexstrophy(frequent)
Location: lowerspine> cervical> thoracicspine
r/ directcontinuityof meningocele with subarachnoid
space
r/ cyst communicating with widenedcentralcanalof spinal
, cord posteriorly
typically + inferiorlyto meningocele
{ lordosis,scoliosis,partialsacralagenesis(common)
MYELOMENINGOCELE
= s?c coveredby leptomeninges containingCSF
+ variableamountof neuraltissue;herniatedthrougha
defectin the posterior/ anteriorelementsof spine
Prevalence; 1:1,000-2,000births(in Great Britain1:200
births);twiceas commonin infantsof
mothers>35 years of age; Caucasians>
Blacks> Orientals;mostcommoncongenital
anomalyof CNS
Etiology: localizeddefectof closureof caudal neuropore
(usuallyclosedby 28 days);persistence of
neuralplacodecausesderangementin the
developmentof mesenchymal + ectodermal
structures
. positivefamilyhistoryin 10%
. neuralplacode= reddishneuraltissuein the middleof
back made up of open spinalcord
. normalskin/ cutaneousabnormality:pigmentednevus,
abnormaldistribution of hair,skindimple,angioma,
lipoma
. MS-AFP(> 2.5 S.D. over mean)permitsdetectionin
80% (positivepredictivevalue of 2-S%) if defectnot
coveredby full skinthickness
Recurrencerate: 3-7% chanceof NTD with previously
affectedsibling/ in fetus of affected
parent
Associated with:
(1) Hydrocephalus (70-90%): requiring
ventriculoperitoneal shuntin 90%
0 25% of patientswith hydrocephalushave spina
bifida!
(2) Chiarill malformation (99%)
(3) Congenitali acquiredkyphoscotiosis (90%)
(4) Vertebralanomalies(vertebralbodyfusion,
hemivertebrae, cleft vertebrae,butterflyvertebrae)
(5) Diastematomyelia Q}aG%| spinalcord split
above (31%),below (ZS%),at the same level(22%)
as the myelomeningocele
(6) Duplication of centralcanal(5%)cephalicto + at
levelof placode
(7) Hemimyelocele(10%)= two hemicordsin
separatedural tubes separatedby fibrous/ bony
spur: one hemicordwith myelomeningocele on one
side of midline,one hemicordnormal/ with smaller
myelomeningocele at a lowerlevel
. impairedneurological functionon side of
hemimyelocele
(8) Hydromyelia(29-77%) cranialto placodeas a
resultof disturbedCSF circulation
Skulland SpineDisorders 215
(9) C hromosomal anomal i es(10-17% ): tri som y18,
trisomy13, triploidy,unbalancedtranslocation
Q ln 20/" no detectableassociatedanomalies!
(10)Tetheringof spinalcord (70-90%)
(11) Arachnoidcyst (2%) dueto developmental
deficiencyduringformationof arachnoid/ dura
materwith a subdurallocation
Distribution: thoracic(2/"),thoracolumbar
(J2%),lumbar
(22%), Iumbosacral (44%)
Location:
(a) dorsal / posterior meningocele:
lumbosacral(70% below L2): may be associated
with tetheredcord, partialsacralagenesis
suboccipital
(b) anterior sacral meningocele= prolapsethrough
anteriorsacralbony defect; occasionallyassociated
with neurofibromatosis type 1, Marfansyndrome,
partialsacralagenesis,imperforate anus,anal
stenosis,tetheredspinalcord,GU tract/ colonic
anomalieslM:F = 1'.4
(c) lateralthoracic meningocelethroughenlarged
intervertebral
forameninto extrapleuralaspectof
thorax;right> leftside,in 10"hbilateral;often
associatedwith neurofibromatosis (85%)+ sharply
angledscoliosisconvexto meningocele
r/ expandedspinalcanal
./ erosionof posteriorsurfaceof vertebralbody
./ thinningof neuralarch
r/ enlargedneuralforamen
(d) laterallumbar meningocelethroughenlarged
neuralforaminaintosubcutaneous tissue/
retroperitoneum; often associatedwith Marfan/
Ehlers-Danlos syndrome/ neurofibromatosis
r/ expandedspinalcanal
r/ erosionof posteriorsurfaceof vertebralbody
ri thinningof neuralarch
! enlargedneuralforamen
(e) traumatic meningocele= dvulsionof spinalnerve
rootssecondaryto tear in meningealrootsheath;in
C-spineafterbrachialplexusinjury(mostcommonly)
r/ smallirregulararachnoiddiverticulum with
extensionoutsidethe spinalcanal
(f) cranialmeningocele = encephalocele
OB-US:
detectionrate of 85-90%; sensitivitydependenton GA
(fetalspine may be adequatelyvisualizedafter 1G-20
weeks GA); false-negativerateof 24h
r/ spinallevelestimatedby countingup from lastsacral
ossification center= 54 in 2nd trimester+ 55 in 3rd
trimester(79% accuracyfor + spinallevel)
r/ may have clubfoot/ rocker-bottomfoot
r/ polyhydramnios
@ S pi ne:
^/ lossof dorsalepidermalintegrity
r/ soft-tissuemass protrudingposieriorly
+ visualization
of sac
216 RadiologyReview Manual
r/ wideningof lumbarspinewith fusiform
enlargement of spinalcanal:
{ splaying(= divergentposition)of ossification
centersof laminaewith cup- / wedge-shaped
pattern(in transverseplane = IT'lost important
sectionfor diagnosis)
{ absenceof posteriorline = posteriorvertebral
elements(in sagittalplane)
{ grossirregularity in parallelism
of lines
representing laminaeof vertebrae(in coronal
plane)
^/ anomaliesof segmentation/ hemivertebrae(33%)
with short-radius kyphoscoliosis
^/ tetheredcord (with lumbar/ lumbosacral
myelomeningocele)
@ Head:
r/ "lemonsign"= concave/ linearfrontalcontour
abnormalitylocatedat coronalsutureassociated
with nonskin-covered myelomeningocele (PPVof
81-84/", in 0.7-1.3/" of normalfetuses)
Prevalence: in 98% of fetuses<24 weeks;in 13%
of fetuses>24 weeks;disappearsin
3rd trimester
{ "bananasign"
Prevalence: in 96% of fetuses<24 weeks; in 91%
of fetuses>24 weeks
lemon sign
bananasign
dangling choroid
r/ "nonvisualization" of cerebellum
{ effacedcisternamagna(100%sensitivity)
0 A normalcisternamagnais 3-10 mm deep and
usuallyvisualizedin 97"/oat 15-25 weeks GA
r/ gpO <Sthpercentileduring2nd trimester(65-79%
sensitivity)
r/ HC <5th percentile(35% sensitivity)
r/ ventriculomegaly (40-90%)with choroidplexus
incompletely fillingthe ventricles(54-63%
= "dangling"choroidon dependentside
sensitivity)
Prevalence: in 44o/"of myelomeningoceles <24
weeksGA; in 94/" of myelomeningo-
celesduring3rd trimester
P lainf ilm s :
r/ bony defect in neuralarch
r/ deformity+ failureof fusionof lamina
{ absentspinousprocess
r/ widenedinteroedicular distance
r/ widenedspinalcanal
Rx: (1) Possiblyelectivecesareansectionat 36-38
weeks GA (may decreaserisk of contaminating/
rupturingthe meningomyelocele sac)
(2) Repairwithin48 hours
Postoperative comp Iications :
(1) Postoperative tetheringof spinalcord by placode/
scar
(2) Constricting duralring
(3) Cordcompressionby lipoma/ dermoid/ epidermoid
cyst
(4) lschemiafrom vascularcompromise
(5) Syringohydromyelia
Prognosis:
(1) Mortality15% by age 10 Years
l Q < 80 (27% );l Q > 100 (27%) ;lear ning
(2) l ntel l i gence:
disability(50%)
(3) Urinaryincontinence:85/" achievesocial
continence(scheduledintermittent catheterization)
(4) Motorfunction:somedeficit(100%);improvement
after repair (37%)
(5) Hindbraindysfunctionassociatedwith Chiari ll
malformation(32%)
(6) Ventriculitis:7"/"in initialrepairwithin48 hours,
morecommonin delayedrepair>48 hours
CYST
NEURENTERIC
= incompleteseparationof foregutand notochordwith
persistenceof canal of Kovalevskibetweenyolk sac
+ notochord;cyst connectedto meningesthrough
midlinedefect
Incidence: rarestof bronchopulmonaryforegut
malformations (pulmonarysequestration,
bronchogeniccYst,entericcYst)
Associatedwith: neurofibromatosis; meningocele;
spinalmalformation(stalkconnects
cyst and neuralcanal;usuallyno stalk
betweencyst and esoPhagus)
Location: anteriorto spinalcanalon mesentericside of
gut
r/ posteriormediastinalmass
r/ air-fluidlevel(if communicating with Gl tractthrough
diaphragmaticdefect)
r/ spinaldysraphismat the same level:
! midlinecleft in centra (accommodatesstalk)
r/ anterior/ posteriorspinabifida
r/ vertebralbody anomalies:absentvertebra,butterfly
vertebra,hemivertebra,scoliosis
r/ diastematomyelia
./ thoracicmyelomeningocele
OS S IFY IN G FIB R OMA
Peak incidence: first 2 decades of life
Histo: areasof osseoustissueintermixedwith a highly
cellularfibroustissue
Sites: maxilla> frontal> ethmoidbone > mandible(rarely
seen elsewhere)
rl areas of increased+ decreasedattenuation
r/ intactinner+ outertable
r/ slow-growing expansilelesion
r/ usuallyunilateral+ monostotic
DDx: may be impossibleto differentiatefrom fibrous
dysplasia
 Skulland SpineDisorders 217

OSTEOMYELITIS
OF VERTEBRA Location: spinalcord,intradural-extramedullary
Prevalence: 2-10/" of all casesof osteomyelitis compartment,extraduralcompartment
Causes: Age: morecommonin adultsthan children;M:F = 6:4
(1) directpenetratingtrauma(mostcommon);following Histo: smallroundbluecellswith hyperchromatic
surgicalremovalof nucleuspulposus nuclei+ scantycytoplasm,frequentmitoses
(2) hematogenous:associatedwith urinarytract . weakness,paresthesia,gait disturbance,pain
infections
/ followingGU surgeryi instrumentation; Spread: throughoutCSF spaceintocranium,lung,bone,
diabetesmellitus;drug abuse lymphnode
Pathophysiology: infectionbegins in low-flowend- r/ T1 and T2 prolongation
vasculararcadesadjacentto Prognosis; in >50% deathwithin2 years
subchondralplate
Organism; Staphylococcusaureus, Salmonella
Peak age: sth-7th decade SACROCOCCYG
EALTERATOMA
. pain in back,neck,chest,abdomen,flank,hip Prevalence:1:40,000livebirths; type | + ll (80%);most
. neurologicdeficit commoncongenitalsolidtumorin the
' fever(mostcommonpresentingsymptom),leukocytosis n e w b o r n :M : F = 1 : 4
. increasederythrocytesedimentationrate Pathogenesis:
. positiveblood/ urineculture (1)growthof residualprimitivepluripotential cells
r/ Oist< space narrowing(earliestradiographicsign) derivedfrom the primitivestreak+ knot (Hensen
r/ demineralization of adjacentvertebrll endplates node)of very earlyembryonicdevelopment
r/ Oulgingof paraspinallines (2) attemptat twinning
r/ tracer uptake in adjacentportionsof two vertebralbodies . increasedprevalenceof twinsin family
^/ decreasedmarrowsignalon Tl Wl Histo:
r/ iso-/ hyperintensemarrowsignalon T2Wl (1) Matureteratoma(55-75%)with elementsfrom glia,
Cx: secondaryinfectionof intervertebral disk is frequent bowel,pancreas,bronchialmucosa,skin
Rx: >4 weeks courseof lV antibiotics appendages,striated+ smoothmuscle,bowel loops,
DDx: diskitis bonecomponents(metacarpal bones+ digits),well-
formedteeth,choroidplexusstructures(production
of CSF)
PARAGANGLIOMA 0 MATURETERATOMA= benigntumorcomposed
Mean age: 46 years; M > F of tissuesforeignto the anatomicsite in which
Path: soft encapsulated(75%)slightlyhemorrhagicmass they arise,usuallycontainingtissuesfrom at least
suppliedby numerousfeedingarteries 2 germ cell layers
Histo: chief cells + sustentacularcells surroundedby (2) lmmatureteratoma(11-28%): admixedwith
fibrovascular stroma;nestsof chiefcellsin primitiveneuroepithelial / renaltissue
classic "Zellballen"configuration 0 IMMATURETERATOMA= benignteratomawith
. mean durationof symptomsfor 4 years:
embryonicelements
. lowerback pain,sciatica
(3) Malignantgerm cell tumor
Location: caudaequina, filumterminale (a) mixedmalignantteratoma(7-17%): elementsof
Site: intraduralextramedullary compartment endodermalsinustumor(= yolk sac tumor)
CT : + eitherform of teratoma
./ bone erosionof spine (b) pure endodermalsinustumor(rare)
MR: (c) seminoma(dysgerminoma), embryonal
^/ S.g (range,1.5-10.0)cm averagetumorsize carcinoma,choriocarcinoma (extremelyrare)
{ well-circumscribed mass isointenseto cord on Tl Wl Metastasesto: lung,bone,lymphnodes(inguinal,
^/ iso-to hyperintense on T2Wl: retroperitoneal), liver,brain
r/ cap sign = low-signal-intensity rim on T2Wl from
hemorrhage Age: 50-70% duringfirstfew days of life; 80% by 6
r/ + "salt-and-pepper" appearance monthsof age; <10"/">2 years of age; rare in
^/ intenseenhancement ; : F= 1 : 4
a d u l t h o o dM
r/ serpentineflowvoidsalongsurface+ withintumor
nodule Classification (Altman):
{ + syringohydromyelia Type I predominantly externallesioncoveredby skin
Angio: with only minimalpresacralcomponent(47%)
^/ intenseearly blushpersistingwell into late arterial+ Type ll predominantly externaltumorwith significant
earlyvenousphase presacralcomponent(35%)
Type lll predominantly sacralcomponent+ external
PRI M I T I V E NE UR O E C T OD ER M AL T U MOR O F extension(8%)
SPI NA L CO RD Type lV presacraltumor with no externalcomponent
Prevalence: 20 cases reportedin literature (10%)
218 RadiologyReview Manual

Associatedwith: other congenitalanomalies(in 18%): Prognosis; prevalenceof malignantgerm celltumors

(1) musculoskeletal (5-16%): spinaldysraphism,sacral increaseswith patient'sage
agenesis,dislocationof hip 0 predominantly fattytissuetumorsare usuallybenign
(2) renalanomalies:hydronephrosis, renalcystic 0 hemorrhagelnecrosisis suggestiveof malignancy
dysplasia,Pottersyndrome 0 cysticlesionsare lesslikelymalignant
anus,gastroschisis,
(3) Gl tract: imperforate 0 sacraldestructionindicatesmalignancy
constipation 0 patients>2 monthsof age havea malignanttumor
(4) fetalhydrops(dueto high-output cardiacfailure) with a 50-90% probability
(5) placentomegaly(due to fetal hydrops) Cx: (1) dystociain 6-13%
(6) curvilinearsacrococcygeal defect (rareautosomal (2) massiveintratumoral hemorrhage
dominantinheritance with equalsex incidence,low (3) fetal death in utero/ stillbirth
malignantpotential,absenceof calcifications) + Rx: 1. Completetumorresection+ coccygectomy
anorectalstenosis/ atresia,vesicoureteralreflux + reconstruction of pelvicfloor: up to 37"/o
recurrencerate, esp. withoutcoccygectomy
AFP elevatedwith mixed malignantteratoma 2. Multiagentchemotherapy (in malignancy) with
+ endodermalsinustumor(CAVE:fetal+ newborn longterm survivalrate of 50%
serumcontainsAFP,whichdoes not reachadultlevels DDx: (1) Myelomeningocele (superiorto sacrococcygeal
untilabout 8 monthsof age) region,not septated,axial bone changes)
prematurelabor(dueto polyhydramnios + largemass) (2) Rectalduplication, anteriormeningocele (purely
uteruslargefor dates cYstic)
radicularpain,constipation,
urinaryfrequency/ (3) Hemangioma, lymphangioma, lipomeningocele,
incontinence lipoma,epidermalcyst,chordoma,sarcoma,
ependymoma,neuroblastoma
Plainf ilm :
r/ amorphous,punctate,spiculatedcalcifications,
possiblyresemblingbone (36-50%);suggestiveof DISEASE
SCHEUERMANN
benigntumor = SPINAL OSTEOCHONDROSIS = KYPHOSIS DORSALIS
r/ soft-tissuemass in pelvisprotrudinganteriorly = V E R TE B R A
JU V E N ILIS ELP IP H Y S ITIS
+ inferiorly = disorderconsistingof vertebralwedging+ endplate
BE: irregularity+ narrowingof intervertebral disk space
r/ anterosuperiordisplacementof rectum Prevalence: in 31% of male + 21"/"of femalepatientswith
r/ luminalconstriction back pain
IVP: Age: onset at puberty
r/ displacement of bladderanterosuperiorly Location: lowerthoracic/ upperlumbarvertebrae;in
r/ developmentof bladderneck obstruction mild cases limitedto 3-4 vertebralbodies
Myelography: ! anteriorwedgingof vertebralbody of >5"
r/ intraspinalcomponentmay be present r/ increasedanteroposterior diameterof vertebralbody
Angio: r/ slightnarrowingof disk space
{ neovascularity(arterialsupplyby middle+ lateral r/ kyphosisof >40olloss of lordosis;scoliosis
sacral+ glutealbranchesof internaliliac artery, ./ Schmorlnodes (intravertebral herniationof nucleus
branchesof profundafemorisartery) pulposusinto vertebralbody) = depressionin contourof
r/ enlargementof feedingvessels endplatein posteriorhalf of vertebralbody; found in up
r/ arterialencasement to 30% of adolescents + youngadults
{ arteriovenous shunting ^/ flattenedarea in superiorsurfaceof epiphysealring
{ earlyvenousfillingwith serpiginous dilatedtumor anteriorly= dvulsionfractureof ringapophysisdue to
veins migrationof nucleuspulposusthroughweak point
betweenring apophysis+ vertebralendplate(fusionof
US / CT : ring apophysisusuallyoccursat about18 yearsof age)
r/ solid (25%)/ mixed (60%)/ cystic (15%)sacralmass ./ detachedepiphysealring anteriorly
^/ t-SO cm (averagesize of 8 cm) in diameter DDx: (1) Developmental notchingof anteriorvertebrae
r/ polyhydramnios(2i3) (NO wedgingor Schmorlnodes)
r/ oligohydramnios, fetalhydronephrosis, fetalhydrops (2) Osteochondrodystrophy (earlierin life,
with ascites,pleuraleffusions,skin edema, extremitiesshow same changes)
placentomegaly are poor prognosticfactors
MR: S P IN A L S TE N OS IS
r/ lobulated+ sharplydemarcatedtumor extremely = eflcfo€tchment on centralspinalcanal, lateralrecess,or
heterogeneous on TlWl as a resultof highsignal neuroforamenby bone / soft tissue
from fat, intermediatesignalfrom soft tissue,low Cause:
signalfrom calcium A. Congenitally shortpedicles
r/ best modalityto detectspinalcanal invasion (a) idiopathic
 Skulland SpineDisorders 219

(b) developmental:Downsyndrome, ./ unusualsmallquantityof contrastmaterialto fill thecal

achondroplasia, hypochondroplasia,Morquio sac
disease r/ anteroposterior + interpediculate
diameterspinal
B. Acquired: canalconstricted
1. Hypertrophy of ligamentum flavum= bucklingof ./ hourglassconfigurationof thecalsac (SAG view)
ligamentsecondaryto jointslippagein facetjoint r/ triangular/ trefoilshape of thecalsac (AXIALview)
osteoarthritis(mostcommon) r/ redundantserpiginousnerve rootsabove + below
2. Facetjoint hypertrophy stenosis
3. Degenerated bulgingi herniateddisk r/ thickenedarticularprocess,pedicles,laminae,
4. Spondylosis, spondylolisthesis ligaments
5. Surgicalfusion ^/ butgingdisks
6. Fracture
7. Ossification of posteriorlongitudinal
ligament SPLITNOTOCHORD
SYNDROME
8. Pagetdisease = spectrumof anomalieswith persistentconnection
9. Epidurallipomatosis betweengut + dorsalectoderm
Age: middle-aged for congenitalcause/ elderlyduring Etiology: failureof completeseparationof ectoderm
6th-8th decadefor acquiredcause; M > F from endodermwith subsequentsplittingof
Location: generallyinvolveslumbarspinalcanal; notochordand mesodermaroundthe
cervicalspinalcanal may be similarlyaffected adhesionabout3rd week of gestation
./ obliterationof epiduralfat r/ fistula/ isolateddiverticula / duplication/ cyst/ fibrous
{ interpediculardistance<25 mm cord / sinus alongthe tract
0 Measurementsare not a valid indicatorof disease! Types:
1. Dorsal enteric fistula
= fistulabetweenintestinal cavity+ dorsalmidline
GervicalSpinatStenosis skin traversingprevertebralsoft tissue,vertebral
Location: multiplelevelsin mid-and lowercervical body,spinalcanal,posteriorelementsof spine
spine . bowelostiumi exposedpad of mucousmembrane
! sagittaldiameterof cervicalspinalcanal<13 mm in dorsalmidlinein newborn
{ hourglassnarrowingof thecalsac with scallopingof . openingpassesmeconium+ feces
the dorsal+ ventralmarginsof the cord r/ dorsalbowelherniaintoa skin-i membrane-
{ greater degree of stenosis in hyperextendedposition covereddorsalsac after passingthrougha
(dueto bucklingof ligamentaflava): combinedanterior+ posteriorspinabifida
{ may appearas spinalblock in hyperextendedneck 2. Dorsal enteric sinus
on AP views = blindremnantof posteriorpartof tractwith midline
openingto dorsalexternalskin surface
LumbarSpinalStenosis 3. Dorsal enteric enterogenous cyst
Cause: = prevertebrallpostvertebral/ intraspinalenteric-
1. Achondroplasia: linedcyst derivedfrom intermediatepart of tract
r/ narrowedinterpediculatedistanceprogressive Intraspinalentericcyst
towardlumbarspine Age at presentation: 2040 years
2. Pagetdisease: bony overgrowth . intermittentlocal/ radicularpain worsenedby
3. Spondylolisthesis elevationof intraspinalpressure
4. Operativeposteriorspinalfusion Location: intraspinalin lowercervical/ upper
5. Herniateddisk thoracicregion
6. Metastasisto vertebrae r/ enlargedspinalcanal at site of cyst
7. Developmental / congenital r/ hemivertebrae, segmentationdefect,partial
Age: presentationbetween30 and 50 years of age fusion,scoliosisin regionof cyst
. often asymptomaticuntil middleage (until 4. Dorsal enteric diverticulum
developmentof secondarydegenerativechanges) = tubular/ sphericaldiverticulum arisingfrom dorsal
. low back pain mesentericborderof bowelas a persistentportion
. "neurogenic = bilaterallower
/ spinalclaudication" of tract betweengut + vertebralcolumn
extremitypain,numbness,weaknessworseduring 5. Dorsal enteric cyst
walkingi standing+ relievedin supinepositionand = involutionof portionof diverticulum neargut
flexion . fi'r?SS in abdomen/ mediastinum(due to bowel
. caudaequinasyndrome:paraparesis, incontinence, rotation)
sensoryfindingsin saddlelikepattern,areflexia
r/ sagittaldiameterof spinalcanal<16 mm (normal SPONDYLOLISTHESIS
rangein adults: 15-23 mm) = forwarddisplacementof one vertebraover another
{ duralsac area <100 mm2 Prevalence: 4/" of generalpopulation
r/ diminisnedamountof CSF + crowdingof nerveroots Location: L5/S1or L4lL5
220 Radiology Review Manual

Grades l-lV (Meyerdingmethod): each grade equals 1/4

anteriorsubluxationof superioron inferiorvertebralbody

lsthmicSpondylolisthesis= op€h-archtype
Cause: usuallybilateralspondylolysis
= s€pdf€ltionof anteriorpart (vertebralbody, pedicles,
transverseprocesses,superiorarticularfacet)slipping
forwardfrom posteriorpart (inferiorfacet,laminae,
dorsalspinousprocess)
Age: often <45 years
. symptomaticif intervertebral disk + posterosuperior
aspectof vertebralbody encroacheson superior
portionof neuroforamen
r/ elongationof spinalcanal in anteroposteriordiameter
r/ bitobedconfigurationof neuroforamen
r/ ratioof maximumanteroposterior diameterof spinal
canalat any leveldividedby diameterat L1 >1.25
Spondylolisthesis= closed-arch
Degenerative
type
= PSEUDOSPONDYLOLISTHESIS
Cause: degenerative/ inflammatoryjoint disease(eg,
rheumatoidarthritis)
Pathophysiology: excess motion of facet joints allowing
forward/ posteriormovement
Age: usually>60 years; M < F (at L4-5)
. commonlysymptomatic due to spinalstenosis
+ narrowingof neuroforamen
{ narrowingof spinalcanal
{ hypertrophyof facet joints
r/ ratioof maximumanteroposterior diameterof spinal
canalat any leveldividedby diameterat L1 <1.25
SPONDYLOLYSIS
= pars interarticularis defectbetweensuperior+ inferior
articulatingprocessesas the weakestportionof spinal
unit
Prevalence: 3-7"/" of population;in 30-70% other family
membersafflicted
= 3:1
Age: earlychildhood;M:F = 3:1; Whites:Blacks
Cause:
(a) pseudarthrosis followingstress (fatigue)fractureof
pars (in most)from repetitiveminortrauma;common
in gymnastics(30%),diving,contactsports(football,
soccer,hockey,lacrosse)
(b) hereditaryhypoplasiaof pars leadsto insufficiency
fracture;eg, pars defectin 34o/"of Eskimos
(c) secondaryspondylolysis:neoplasm,osteomyelitis,
Pagetdisease,osteomalacia,osteogenesis
imperfecta
(d) congenitalmalformation:frequentlyassociatedwith
spinabifidaoccultaof S1, dorsallywedge-shaped
body of 15, hypoplasiaof L5; HOWEVER:no pars
defectshave been identifiedin fetal cadavers
. symptomaticin 50% (if associatedwith degenerative
disk disease/ spondylolisthesis)
Location: L5 (67-95%);La(15-30%);L3 (1-2%);
in75 /o b i l a te ra l
Oblique Radiograph of L5 CT Scan through Mid-
Vertebral Body
Spondylolysis
P l ai nfi l m:
{ radiolucent band+ scleroticmarginresembling the
of the "Scottiedog" (on obliqueview)
collar
^/ may be associatedwith spondylolisthesis
{ subluxationof involvedvertebra(if pars defect
bilateral)
r/ Wilkinsonsyndrom€= Ie?ctivesclerosis+ bony
hypertrophyof contralateralpedicle+ lamina
(producedby stresschangesrelatedto weakeningof
neuralarch in unilateralpars defect)
0 Planari SPECTbonescintigraphy may be useful!
C T:
{ pars defectlocated10-15 mm abovedisk space
^/ innercontourof spinalcanalinterrupted
Spondylolysisof CervicalSpine
= progressivedegenerationof intervertebral disks
teadingto proliferativechangesof bone + meninges;
morecommonthan disk herniationas a causefor
cervicalradiculopathy
Prevalence: 5-1 0/" at age 20-30; >50/" at age 45;
>90"/"bY age 60
. spasticgait disorder
. neckpain
Location: C4-5, C5-6, C6-7 (greaternormalcervical
motionat these levels)
Sequelae: (a) directcompressionof spinalcord
(b) neuralforaminalstenosis
(c) ischemiadue to vascularcompromise
(d) repeatedtraumafrom normalflexion/
extension
DDx of myelopathy:
rheumatoidarthritis,congenitalanomaliesof
craniocervicaljunction,intraduralextramedullary
tumor,spine metastases,cervicalspinalcord tumor,
arteriovenous malformation,amyotrophic lateral
sclerosis,multiplesclerosis,neurosyphilis
SYRINGOHYDROMYELIA
= SYRINGOMYELIA = SYRINX (usedin a general
manner
reflecting in classification)
difficulty
= longitudinally orientedCSF-filledcavities+ gliosiswithin
spinalcord frequentlyinvolvingboth parenchyma
+ centralcanal
Age: primarilychildhoodI earlyadult life
Cause: Chiari I malformation(41"/"), lrauma (28h),
neoplasm(15/"), idiopathic(15%)
 Skulland SpineDisorders 221

. loss of sensationto pain + temperature(interruptionof Syringomyelia

spinothalamictracts) = ACQUIRED / SECONDARY SYRINGOHYDROMYELIA
. trophicchanges[skinlesions;Charcotjointsin 2S/" = ar'rycavitywithinsubstanceof spinalcord that may
(shoulder,elbow,wrist)] communicate with the centralcanal,usuallyextending
. muscleweakness(anteriorhorncell involvement) over severalvertebralsegments
. spasticity,hyperreflexia(uppermotor neuron Histo: not linedby ependymaltissue
involvement) Pathophysiology: interruptedflow of CSF throughthe
. abnormalplantarreflexes(pyramidaltract involvement) perivascularspacesof cord between
Location: predominantlylowerend of cervicalcord; subarachnoidspace + centralcanal
extensioninto brainstem1=syringobulbia) Cause:
1. Posttraumaticsyringomyelia
CT: Prevalence:in3.2/" after spinalcord injury
r/ distinctarea of decreasedattenuationwithinspinal Location: 68% in thoraciccord
, cord (100%) r/ O.S-+Ocm (average6 cm) in length
r/ swollen/ normal-sizedlatrophiccord ! syrinxmay be septated(parallelareas of
r/ no contrastenhancement cavitation)on transverseTlWl
r/ flattenedvertebralborder(rare)with increased ^/ loss of sharp cord-CSFinterface(obliterationof
transversediameterof cord arachnoidspace by adhesions)
r/ changein shape+ size of cordwith changein position ^l in q+y" associatedwith arachnoidloculations
(rare) (extramedullary arachnoidcysts)at upper
r/ fitlingof syringohydromyelia with intrathecalcontrast aspectof syrinx
(a) earlyfillingvia directcommunication with 2. Postinflammatory svringomyelia
subarachnoidspace subarachnoid hemorrhage, arachnoidadhesions,
(b) late fillingafter4-8 hours (80-90%) secondaryto S/P surgery,infection(tuberculosis, syphilis)
permeationof contrastmaterial 3. Tumor-associated syringomyelia
Myelography: spinalcord tumors,herniateddisk;secondaryto
r/ enlargedcord (DDx:intramedullary tumor) circulatorydisturbance+ thoracicspinalcord
r/ "collapsingcord sign"= collapsingof cord with gas atrophy
myelographyas fluid contentmovescaudadin the 4. Vascularinsufficiency
erect position(rare)
MR: ReactiveCyst
r/ cysticarea of low signalintensityon T1Wl, increased = POSTTRAUMATIC SPINALCORDCYST
, intensityon T2Wl = CSFJilledcyst adjacentto levelof trauma;usually
r/ presenceof CSF flow-void(= low signalon T2Wl) single(75%)
withincavityfrom pulsations . late deteriorationin patientswith spinalcord injury
r/ beadedcavityfrom multipleincompleteseptations (not relatedto severityof originalinjury)
r/ cord enlargement Rx: shuntingleadsto clinicalimprovement

Hydromyelia TERATOMA
OF SPINE
= PRIMARY / CONGENITAL SYRINGOHYDROMYELIA = neoplasmcontainingtissuebelongingto all 3 germinal
= dilatationof persistentcentralcanal of spinalcord (in layersat siteswhere these tissuesdo not normally
70-80% obliterated),which communicateswith 4tn occur
ventricle(= cornmunicating syringomyelia) Prevalence; 0. 15% (excludingsacrococcygealteratoma)
Histo: lined by ependymaltissue A ge: al l ages; M:F = 1:1
Path: solid,thin-/ thick-walled partially/ whollycystic
Associated with: with cleari milkyI darkcyst fluid,uni- /
(1) Chiarimalformation in 20-70% multilocular,presenceof bone/ cartilage
r/ metamerichaustrationswithinsyrinxon sagittal Location: intra-/ extramedullary
TlWI r/ completeblock at myelography
(2) Spinaldysraphism r/ syringomyeliaabove levelof tumor
3) Myelocele { spinalcanalmay be focallywidened
4) Dandy-Walkersyndrome
5) Diastematomyelia TERMINAL MYELOCYSTOCELE
6) Scoliosisin 48-87% = cornbir'r€rtion
of posteriorspina bifida+ meningocele
7) Klippel-Feilsyndrome + tetheredcord + hydromyelia+ cysticdilatationof the
8) Spinalsegmentationdefects distalcentralcanal
9) Tetheredcord (in up to 25%) Cause: disturbedCSF circulationresultingin dilatation
DDx: transientdilatationof the centralcanal (transient of ventriculus
terminalis+ disruptionof dorsal
findingin newbornsduringthe firstweeksin life) mesenchyme
222 RadiologyReviewManual
Associatedwith: anorectal+ genitourinary+ vertebral
anomalies(analatresia,cloacal
exstrophy,scoliosis,sacralagenesis)
. skin-coveredmass in lumbosacralregion
{ spinalcord surroundeddorsally+ ventrallyby dilated
subarachnoidspace of the meningocele
r/ nerve root exit ventrally
./ OitiOspinalcord
r/ hydromyelia
TETHERED
CORD
= TIGHTFILUMTERMINALE SYNDROME = LOWCONUS
M E DULLA RIS
= abnormallyshort+ thickenedfilumterminalewith
TRAUMATIC
positionof conus medullarisbelow L2l3 (normallocation
of tip of conusmedullaris:L 415at 16 weeksof
gestation,L2l3 at birth,L112>3 monthsof age)
0 RULEOF THREES: aboveL3 by age 3 months!
Etiology: incompleteinvolutionof distalspinalcord with
failureof ascentof conus
Pathophysiology: stretchingof cord leads to vascular
insufficiencyat levelof conus
Age at presentation: 5-15 years (in years of groMh
s p u rt);M:F = 2 :3
Associatedwith: filar lipoma in 29-78/", filar cyst,
diastematomyelia, imperforateanus
. dorsalnevus,dermalsinustract, hair patch (50%)
. bowel+ bladderdysfunctionin childhood
. spasticgait with musclestiffness
. lowerextremityweakness+ muscleatrophy
. asymmetrichyporeflexia+ fasciculations
. orthopedicanomalies:scoliosis,pes cavus,tight
Achillestendon
. hypalgesia,dysesthesia
. paraplegia,paraparesis
. radiculopathy(adults)
. hyperactivedeep tendon reflexes
. extensorplantarresponses
. anal/ perinealpain (in adults)
. back pain (particularly with exertion)
@ T ightf ilum
r/ diameterof filumterminale>2 mm (normalrangeof
0.5 to 2 mm) at L5-S1 level (55%)
{ smallfibrolipoma withinthickenedfilum(23%)
{ smallfilar cyst (3%)
{ spinalcord endingin a smalllipoma(13%)
@ Tetheredcord (100%)
VENTRICULUS
{ conus medullarisbelow levelof L3 at birth and below
L2 by age 12 (86%)
{ abnormaldorsalfixationof cord adjacentto vertebral
arches(in prone position)
{ reduced/ absentpulsatilemovementof the cord
+ nerveroots (on M-modescanning)
r/ widenedtriangularthecal sac tentedposteriorly
(thecalsac pulledposteriorlyby filum)
{ abnormallateralcourseof nerveroots(t15" angle
relativeto spinalcord)
@ Vertebrae
r/ lumbarspinabifidaoccultawith interpedicular
widening
{ scoliosis(20%)
MR:
r/ prolongedT1 relaxationin centerof spinalcord on
Tl Wl in 25h (? myelomalacia/ mildhydromyelia)
Rx: decompressivelaminectomy/ partialremovalof
lipoma+ freeingof cord
Dx: tip of conus medullarisbelow L2-3
NEUROMA
proliferationof the proximalend of a
= flor'll'teoplastic
severed/ partiallytransectedinjurednerve
Histo: nonencapsulatedtangledmultidirectional
regeneratingaxonalmasses+ Schwanncells
+ endo-and perineuralcellsin dense
collagenousmatrixwith surrounding fibroblasts
Types:
(a) spindleneuroma= internalfocalfusiformswelling
Cause: chronicfrictioni irritationof nondisrupted
injuredbut intactnervetrunk
(b) lateral/ terminalneuroma
Cause: severetraumawith partialavulsion/
disruption/ total transectionof nerve
Timeof onset: 1-12 monthsafter injury
' Pain
. Tinelsign = palpation/ tappingon lesionreproduces
Pain
Location: lowerextremity(afteramputation),head and
neck (aftertooth extraction),radialnerve,
brachialplexus
{ fusiformmass/ focalenlargement with enteringand
exitingnerve (spindletype)
./ bulbousmass in continuitywith normalnerveproximally
(lateral/ terminaltype)
MR:
r/ isointense to muscleon TlWl
r/ heterogeneous intermediate to highsignalintensityon
T2WI
! "fascicularsign" = heterogeneousringlikepatternon
T2WI
Rx: acupuncture,cortisoneinjection,transcutaneous/
directnervestimulation,physicaltherapy,surgical
resection
TERMINALIS
= srTl€tllependyma-lined oval cyst at the transitionfrom the
tip of the conusmedullaristo the originof the filum
terminale
Origin: result of canalizationand regressive
of the caudalend of the
differentiation
developingspinalcord duringembryogenesis
Size: 8-10 mm long,24 mm in diameter
0 Regressesduringthe firstweeks after birth
 DifferentialDiagnosisof BrainDisorders 223

DIFFERENTIALDIAGNOSISOF BRAIN DISORDERS

BIRTHTRAUMA 5. Increasedvenouspressure
1. Gaputsuccedaneum 6. ElevatedCSF protein
= localizededemain presentingportionof scalp, 7. Pseudotumor cerebri
frequentlyassociatedwith microscopichemorrhage . papilledema
+ subcutaneous hyperemia r/ enlargementof periopticnervesubarachnoidspace
Cause: commonaftervaginaldelivery
., soft superficialpittingedema PROLACTIN
ELEVATION
r/ crossessuturelines Normallevel: up to 25 ng/ml
2. Subgaleal hemorrhage Cause:
= hemorrhagebetweengalea aponeurotica(= central 1. Interferencewith hypothalamic-pituitary
axis:
fasciaformedby occipitofrontal + temporoparietal (a) hypothalamic tumor
muscles)and periosteumof outertable (b) parasellartumor
. may becomesymptomaticsecondaryto significant (c) pituitaryadenoma
bloodloss in children (d) sarcoidosis
. firm fluctuantmass increasingin size after birth (e) histiocytosis
. may dissectintosubcutaneous tissueof neck (f) traumaticinfundibular transection
. usuallyresolvesover 2-3 weeks 2. Pharmacologic agents
0 Occasionally due to spontaneous decompression of alpha-methyldopa, reserpine,phenothiazine,
intracraniaf(epidural)hematoma butyrophenone, tricyclicantidepressants,
oral
3. Gephalohematoma contraceptives
= hematomabeneathouter layerof periosteum 3. Hypothyroidism (TRHalsostimulatesprolactin)
Cause: incorrect applicationof obstetricforceps/ 4. Renalfailure
skullfractureduringbirth 5. Cirrhosis
lncidence: 1-2/" of all deliverres 6. Stress/ recentsurgery
Location: mostcommonlyparietal 7. Breastexamination
. firm tense mass 8. Pregnancy
. usuallyincreasein size after birth L Lactation
. resolutionin few weeks to months
^/ crescent-shaped lesionadjacentto outer table of
s k ull STROKE
i/ will not crosscranialsutureline = genericterm designatinga heterogeneous groupof
r/ may calcify/ ossifycausingthickeningof diploe cerebrovascu lar disorders
4. Skullfracture lncidence:
lncidence: 1"h of all deliveries 3rd leadingcause of death in UnitedStates(afterheart
./ Cf shows associatedintracranialhemorrhage disease+ cancer);2nd leadingcauseof deathdue to
5. Subduralhemorrhage cardiovascular diseasein U.S.;2nd leadingcauseof
(a) convexityhematoma(b) interhemispheric death in patients>75 years of age; 450,000new cases
hematoma(c) posteriorfossa hematoma per year;160 new strokesper 100,000populationper
6. Benign subdural effusion year; leadingcause of death in Orient
= benignconditionthat resolvesspontaneously Age: >55 years(12%occurin youngadults);M:F = 2:1
. clear/ xanthochromicfluidwith elevatedproteinlevel Riskractors: okins'
r/ extracerebralfluid collectionaccompaniedby i iJfjiY;
I'l;j:;:n J1'#' li"
ventriculardilatation(= coffimunicating rypercholesterolem ia, myocardial
hydrocephalus causedby impairedCSF absorption infarction,
atrialfibrillation,
congestive
of thesesubduralfluidcollections) heartfailure,alcoholicexcess,substance
abuse,oral contraceptives, pregnancy,
GlasgowComaScale high anxiety+ stress
. eye openrng: (1 -5 ) Etiology:
. motorresponse: (1-5) A. NONVASCULAR (5%): eg, tumor,hypoxia
. verbalresponse: (1-5) B. VASCULAR(e5%)
Total: 3-15 1. Braininfarction= ischemicstroke(80%)
(a) Occlusiveatheromatousdiseaseof
INCREASED INTRACRANIAL PRESSURE (35%)/ intracranial
extracranial (10%)arteries
1. Intracranial
mass = largevesseldiseasebetweenaorta
2. Hydrocephalus + penetratingarterioles
3. Malignanthypertension - criticalstenosis,thrombosis,
4. Diffusecerebraledema - plaquehemorrhage / ulceration
/ embolism
224 Radiology Review Manual
(b) Smallvesseldiseaseof penetratingarteries
(25%)= lacunarinfarct
(c) Cardiogenicemboli(6-15-23%)
- lschemicheartdiseasewith mural
ItT:if *yocardiat (3%rist</
infarction
year)
cardiacarrhythmia
- Valvularheartdisease
postinflammatory (rheumatic)valvulitis
infectiveendocarditis(20% rislVyear)
nonbacterialthromboticendocarditis
(30% rislVyear)
mitralvalve prolapse(low risk)
mitralstenosis(20% risk/year)
- *.fJ:i'ffi1'Jill?i,1
I#l;'8fl',',
fl),"",,
- Left atrial myxoma (27-55o/"risVyear)
(d) Nonatheromatous disease(5%)
- elongation, coil,kinks(up to 20%)
- fibromuscular dysplasia(typicallyspares
origin+ proximalsegmentof ICA)
- aneurysm(rare)may occur in cervical/
petrousportion/ intracranially
- dissection:traumatic/ spontaneous(2%);
up to 15'/" of strokesin young adults
- cerebralarteritis(Takayasu,collagen
disease,lymphoidgranulomatosis,
temporal arteritis,Behgetdisease,chronic
meningitis, syphilis)
- postendarterectomythrombosis/
embolism/ restenosis
(e) Overactivecoagulation(5%)
2. Hemorrhagicstroke(20%)
(a) Primaryintracerebralhemorrhage(15%)
- Hypertensivehemorrhage(40-60%)
- Amyloidangiopathy(15-25%)
TRANSIENT
- Vascularmalformation (10-15%)
- Drugs: eg, anticoagulants (1-2%)
- Bleedingdiathesis(<1"/"):eg, hemophilia
(b) Vasospasmdue to nontraumaticSAH (4%)
- Rupturedaneurysm(75-80%)
- Vascularmalformation (10-15%)
- 'If\epsneurysmal" SAH (5-15%)
(c) Veno-occlusive disease(1%):sinusthrombosis
May be preceded by TIA
0 10-14Y" of all strokesare precededby TIA!
0 60% of all strokesascribedto carotiddiseaseare
precededby TIA!
Prognosis:
(1) death duringhospitalization(25%): alterationin
consciousness, gaze preference,
densehemiplegia
have a 40/" mortality rate
(2) survivalwith varyingdegreesof neurologicdeficit
(75%)
(3) good functionalrecovery(40o/")
0 Hypodensityinvolving>50% of MCA territoryhas
a fatal outcomein 85%!
0 Clinicaldiagnosisinaccuratein 13%!
Role of imaging:
1. Confirmclinicaldiagnosis
2. ldentifyprimaryintracerebralhemorrhage
3. Detectstructurallesionsmimickingstroke: tumor,
subduralhematoma
vascularmalformation,
4. Detectearly complicationsof stroke:
hemorrhagic
cerebralherniation, transformation
Indications for cerebrovasculartesting:
1. TIA = transientischemicattack
2. Progressionof carotiddiseaseto 95-98% stenosis
3. Cardiogenic cerebralemboli
Temporal cl assification:
1. TIA = transientischemicattack
2. RIND= reVefsible
ischemicneurologicdeficit
= fullyreversibleprolongedischemiceventresulting
in minorneurologicdysfunctionlor >24 hoursand
<8 weeks
lncidence; 16 per 100,000populationper year
3. Progressing stroke = stepwise/ gradually
progressing accumulative neurologicdeficitevolving
over hours/ days
4. Slow stroke = rdta clinicalsyndromepresentingas
developingneuronalfatiguewithweaknessin lower/
proximalupperextremityafter exercise;occursin
patientswith occludedinternalcarotidartery
5. Completed stroke = seVefe+ persistentstable
neurologicdeficit= cerebralinfarction(deathof
neuronaltissue)as end stageof prolongedischemia
>21 days
. levelof consciousnesscorrelateswell with size of
infarction
Prognosis; 6-1 1"h recurrentstroke rate
ATTACK
ISCHEMIC
= briefepisodeof transientfocalneurological deficitowing
to ischemiaol <24 hoursdurationwith returnto pre-
attack status
lncidence: 31 per 100,000populationper year;
increasingwith age up to 300; 105,000new
cases per year in UnitedStates; M > F
Cause: (1) embolic: usuallyfrom ulcerativeplaqueat
carotidbifurcation
(2) hemodynamic:fall in perfusionpressure
distalto a high-gradestenosis/ occlusion
Riskfactors:
(1) Hypertension (linearincreasein probabilityof stroke
with increasein diastolicbloodpressure)
(2) Cardiacdisorders(priormyocardialinfarction, angina
pectoris,valvularheartdisease,dysrhythmia,
congestiveheartfailure)
(3) Diabetesmellitus
(4) Cigarettesmoking(weak)
Prognosis: 5.3/" stroke rate per year for 5 years after first
TIA; per year 12/"increaseof stroke/
myocardialinfarction/ death;completestroke
in 33% within5 years;completestrokein 5o/o
in 1 month
 DifferentialDiagnosisof BrainDisorders 225

A. CAROTTDTtA (2/3) DEMENTIA

. carotidattacks<6 hours in 90%
1. A l zhei mer
di sease
. transientweakness/ sensorydysfunction
2. Pickdisease
CLASSICALLY in 3. Normalpressurehydrocephalus
(a) hand ltace with embolicevent 4. Subduralhematoma
(b) proximalarm + lowerextremitywith 5. Brainmass
hemodynamicevent (watershedarea)
- motordysfunctiofl= we?kfless,paralysis, TRIGEMINAL
NEUROPATHY
clumsinessof one / both limbson same side . facialpain, numbness,weaknessof masticatory
- sensoryalteratiofi= r'rUrTlbfless,
loss of sensation, muscles,trismus
paresthesiaof one / both limbson same side . diminished/ absentcornealreflex
- speech/ languagedisturbance= difficultyin . abnormaljawreflex
speaking(dys-/ aphasia)/ writing,in . decreasedpain / touchi temperaturesensation
comprehension of language/ readingi performing . atrophyof masticatorymuscles
calculations . tic douloureux = paroxysmalfacialpain (usually
- visualdisturbance= loss of visionin one eye,
confinedto V, and Vj mainlycausedby neurovascufar
homonymoushemianopia, amaurosisfugax compression ltortuout elongated superiorcerebelIar
. paresis(mono-,hemiparesis) in 61% artery/ anteriorinferiorcerebellararteryI
. paresthesia(mono-,hemiparesthesia) in 57"/" vertebrobas ilar dolichoectasia/ venous compression)
. amaurosisfugax (= transientpremonitoryattack
A . B R A IN S TE M LE S ION
of impairedvisiondue to retinalischemia)in 12/" 1. Vascular:infarct,AVM
causedby transienthypotension or emboliof 2. Neoplastic:glioma,metastasis
platelets/ cholesterolcrystals,which may be 3. Inflammatory:multiplesclerosis(1-8%),herpes
revealedby funduscopy rhombencephalitis
. facialparesthesiain 30%
4. Other: syringobulbia
B . C IS TE R N A LCAUSES
B . V E RT E B RO BA ST L A R (1 /3 )
T tA 1. Vascular:aneurysm,AVM, vascularcompression
. vertebrobasilar events<2 hours in g0% 2. Neoplastic:acousticschwannoma,meningioma,
- motordysfunctiofl= os with carotidTIA but
trigeminalschwannoma, epidermoidcyst,lipoma,
sometimeschangingfrom sideto side including metastasis
quadriplegia, diplopia,dysarthria,dysphagia 3. Inflammatory;neuritis
- sensoryalteratioh= ?S with carotidTIA usually
C . ME C K E LC A V E+ C A V E R N OU S S IN U S
involvingone / bothsidesof face/ mouth/ tongue 1. Vascular:carotidaneurysm
- visualloss= as with carotidTIA includinguni-/
2. Neoplastic:meningioma, trigeminalschwannoma,
bilateralhomonymoushemianopia epidermoidcyst,lipoma,pituitaryadenoma,base of
- disequilibrium of gait/ posturaldisturbance,
ataxia, skullneoplasm,metastasis, perineuraltumor
imbalance/ unsteadiness spread
- drop attack= suddenfall to the groundwithoutloss
3. Inflammatory:Tolosa-Huntsyndrome
of consciousness D. EXTRACRANIAL
. binocularvisualdisturbancein S7o/"
1. Neoplastic:neurogenictumor,squamouscell
. vertigoin 50%
carcinoma,adenocarcinoma, lymphoma,adenoid
. paresthesiain 40%
cysticcarcinoma,mucoepidermoid carcinoma,
. diplopiain 38%
melanoma,metastasis, perineuraltumorspread
. ataxiain 33%
2. Inflammatory:sinusitis
. paresisin 33%
3. Other: masticatorspace abscess,trauma
. headachesin 25%
. s eiz ur esin 1 .5 % CLASSIFICATION OF CNS ANOMALIES
A. DORSALINDUCTIONANOMALY
Accelerating / crescendo TIA = defectsof neuraltube closure
= fepeotedperiodiceventsof neurologicdysfunction 1. A nencephal y
with completerecoveryto normalin interphase 2. Cephalocele: at 4 weeks
3. Chiarimalformation: at 4 weeks
Rx: 1. Carotidendarterectomy (1% mortality,S/" 4. Spinaldysraphism
stroke) 5. Hydromyelia
2. Anticoagulation B. VENTRALINDUCTIONANOMALY
3. Antiplatelet
agent: aspirin,ticlopidine(Ticlid@) = defectsin formationof brainvesicles+ face
- in patientswith recentlysymptomaticTIA /
1. Holoprosencephaly: 5 - 6 weeks
minorstroke+ >70o/o carotidarterystenosis: 2. Septo-optic dysplasia: 6 - 7 weeks
prophylacticcarotidendarterectomy + 3. Dandy-Walker malformation: 7 - 10 weeks
chroniclow-doseaspirintherapy 4. Agenesisof septumpellucidum
226 Radiology Review Manual

C. NE URO NA LP R OL IF E R A T IO&N H IST OGE N E S IS Etiology: genetic;viral infection;nutritionaldisorders(eg,

1. Neurofibromatosis: 5weeks-6months anorexianervosa,Cushingsyndrome) ;
2. Tuberoussclerosis: 5weeks-6months immunesystemdisorders(eg,AIDS);
3. Primaryhydranencephaly: >3 months exposureto toxins(eg,CO);exposureto drugs
4. Neoplasia (eg,alcohol,methotrexate + radiation)
5. Vascularmalformation (veinof Galen,AVM,
hemangioma) Leukodystrophy
D. NEURONALMIGRATIONANOMALY = degenerativediffusesclerosiswith symmetrical
due to infection.ischemia.metabolicdisorders bilateralwhite matterlesions
1. Schizencephaly: 2 months
2. Agyria+ pachygyria: 3 months Leukoencephalopathy
3. Gray matterheterotopia: 5 months = diseaseof white matter
4. Dysgenesisof corpuscallosum: 2 - 5 months
5. Lissencephaly A . MY E LIN OC LA S TIC / D E MY E LIN A TIND
GISEASE
6. Polymicrogyria = diseasethat destroysnormallyformedmyelin
7. Unilateralmegalencephaly 0 Usuallyaffectsolderchildren/ adults
E . DE S T RUCTIVE L E SION S (a) infectious
1. Hydranencephaly 1. Progressive multifocalleukoencephalopathy
2. Porencephaly 2. Subacutesclerosingpanencephalitis (SSPE)
3. Hypoxia:periventricularleukomalacia, germinal 3. Acutedisseminated encephalomyelitis(ADE)
matrixhemorrhage (b) noninfectious
4. Toxicosis 1. R adi ati on
5. Infections(TORCH) 2. Anoxia
(a) Toxoplasmosis 3. HypertensiveencePhaloPathy
(b) Other: syphilis,hepatitis,zoster 4. Disseminated necrotizingleukoencephalopathy
(c) Rubella (from methotrexatetheraPY)
{ punctate/ nodularcalcifications (c) others
{ porencephaliccysts 1. Multiplesclerosis(mostfrequentprimary
{ occasionallymicrocephaly demyelinating disease)
(d) Cytomegalovirus inclusiondisease 2. Alzheimerdisease(mostcommonof diffuse
r/ typicallypunctate/ stippled/ curvilinear gray matterdegenerativediseases)
periventricular calcifications 3. Parkinsondisease(mostcommonsubcortical
{ often hydrocephalus degenerativedisease)
(e) Herpessimplex 4 . Creutzfeldt-Jakob disease
5 . Menkesdisease(sex-linked recessivedisorder
of coppermetabolism)
Absenceof SeptumPellucidum 6 . Globoidcell leukodystrophy
1. Holoprosencephaly 7 . Spongiformdegeneration
2. Callosalagenesis 8 . Cockaynesyndrome
3. Septo-opticdysplasia 9 . Spongiformleukoencephalopathy
4. Schizencephaly 1 0 . Myelinoclasticdiffusesclerosis(Schilder
5. Severechronichydrocephalus disease)
6. Destructiveporencephaly
B . DYSMYELINATI
NG DISEASE
Phakomatoses = metabolicdisorder(= eflzYmedeficiency)resultingin
phako(Greek)= lens/ lentil-shaped
object deficient/ absentmyelinsheaths
= NEUROCUTANEOUS SYNDROMES 0 Usuallypresentsin first2 years/ 1st decadeof life!
= NEUROECTODERMAL DYSPLASIAS 0 Associatedwith white matteratrophy
= development of benigntumors/ malformations (a) macrencephalic:
in organsof ectodermal
especially origin 1. Alexanderdisease(frontalareas affectedfirst)
1. Neurofibromatosis 2. Canavandisease(whitematterdiffusely
2. Tuberous sclerosis affected)
3. vonHippel-Lindaudisease (b) hyperdense thalami,caudatenuclei,coronaradiata
4. Sturge-Weber-Dimitrisyndrome 1. Krabbedisease
5. Ataxia-telangiectasia (c) familyhistory(X-linkedrecessive)
1. X-linkedadrenoleukodystrophy
DEGENERATIVEDISEASESOF CEREBRAL 2. Pelizaeus-Merzbacher disease
HEMISPHERES (d) others
= progressive /
by destruction
fataldiseasecharacterized 1. Metachromatic leukodystrophy(mostcommon
of grayandwhitematter
alteration hereditaryleukodystroPhy)
 DifferentialDiagnosisof BrainDisorders 227

2. Binswangerdisease(SAE)
3. Multi-infarct
dementia(MlD) Intra-versus ExtraaxialMass
4. Pickdisease
lntraaxial Extraaxial
5. Huntingtondisease
6. Wilsondisease Relationship no attachment contiguous
7. Reyesyndrome to dura untiladvanced
8. Mineralizingmicroangiopathy
9. Diffusesclerosis Localbony changes uncommon common

BRAINATROPHY Cortexdisplaced towardbone away from bone,

CerebralAtrophy bucklingof gray
= irreversibleloss of brainsubstance+ subsequent + white matter,
enlargementof intra-and extracerebralCSF- displacementof
containingspaces(hydrocephalus ex vacuo vessels
= ventriculomegaly)
Subarachnoidcisterneffaced widened,CSF
A. DI F F US EB R A INAT R O P H Y cleft
Cause:
(a) Trauma,radiationtherapy Feedingarteries pial dural
(b) Drugs (dilantin,steroids,methotrexate,
marijuana,harddrugs,chemotherapy),
alcoholism, hypoxia Hippocampal
Atrophy
(c) Demyelinating disease(multiplesclerosis, 1. AlzheimerDisease
encephalitis) 2. Mesialtemporalsclerosis
(d) Degenerativedisease . complexpartialseizures
eg, Alzheimerdisease,Pickdisease,Jakob- 3. Normalin octogenarians
Creutzfeldtdisease
(e) Cerebrovascular disease+ multipleinfarcts
(f) Advancingage, anorexia,renalfailure EXTRAAXIAL LESIONS
{ enlargedventricles+ sulci Extraaxial Tumor
B. FOCALBRAINATROPHY mnemonic.' "MABEL"
Cause: vascular/ chemical/ metabolic/ traumatic/ Meni ngi oma
idiopathic(Dyke-Davidoff-Mason syndrome) Arachnoidcyst
C. REVERSIBLEPROCESSSIMULATINGATROPHY Bony lesion
(in youngerpeople) Epidermoid
Cause: anorexianervosa,alcoholism,catabolic Leukemic/ lymphomatous
infiltration
steroidtreatment,pediatricmalignancy
{ prominentsulci Leptomeningeal Disease
r/ ipsilateraldilatationof basalcisterns+ ventricles A . IN FLA MMA TION
r/ ex vacuo dilatationof ventricles 1. Langerhanscell histiocytosis
r/ thinningof gyri 2. Sarcoidosis
3. Wegenergranulomatosis
CerebellarAtrophy 4. Chemicalmeningitis:ruptureof epidermoid
A. WITH CEREBRALATROPHY B . IN FE C TION
= gen€rolizedsenilebrainatrophy 1. Bacterialmeningitis
B. W I T HO UTCER EB R A LA T R OP H Y 2. Tuberculousmeningitis
1. Olivopontocerebellar degeneration / Marieataxia 3. Fungalmeningitis
/ Friedreichataxia 4. Neurosyphilis
. oflSetof ataxiain young adulthood C . TU MOR
2. Ataxia-telangiectasia (a) Primarymeningealtumor
3. Ethanoltoxicity:predominantly affectingmidline 1. Meningioma
(vermis) 2. Glioma: primaryleptomeningeal
4. Phenytointoxicity: predominanflyaffecting gfiobfastomatosis/ gliosarcomatosis
cerebellarhemispheres 3. Melanoma/ melanocytoma
5. ldiopathicdegeneration secondaryto carcinoma 4. Sarcoma
(= paraneoplastic), usuallyoat cell carcinomaof 5. Lymphoma
lung (b) CSF-spreadfrom primaryCNS tumor
6. Radiotherapy 1. Medul l obl astoma
7. Focalcerebellaratrophy: 2. Germi noma
(a) infarction(b) traumaticinjury 3. Pineoblastoma
228 Radiology Review Manual

(c) Metastasis (b) Vasogenicedema(mostcommonform)

1. Breastcarcinoma increasein pinocytoticactivitywith passageof
2. Lymphoma/ leukemia proteinacrossvesselwall into intercellularspace
3. Lungcarcinoma (lackof contrastenhancementmeansbreakdown
4. Malignantmelanoma of blood-brainbarrieris not the cause);associated
5. Gastrointestinal
carcinoma with primarybrain neoplasm,metastases,
6. Genitourinarycarcinoma hemorrhage, infarction,inflammation
D. T RA UM A Types:
1. Old subarachnoid hemorrhage 1. Hydrostatic edema
2. Surgicalscarringfrom craniotomy rapid increase/ decreasein intracranialpressure
3. Lumbarpuncture 2. Interstitial
edema
increasein periventricular interstitialspaces
FluidCollectionin Childhood
Pericerebral secondaryto transependymal flow of CSF with
A . E NLA RG EDS U B AR AC H N O ID SP AC E elevatedintraventricular pressure
(a) due to macrocephaly 3. Hypoosmoticedema
(b) due to brain atrophy producedby overhydrationfrom lV fluid/
{ superficialcorticalveins cross subarachnoidspace inappropriatesecretionof antidiuretichormone
to reachsuperiorsagittalsinus 4. Congestivebrainswelling
^/ wide sulci,normalconfiguration of gyri rapidaccumulationof extravascularwater as a
^/ normal/ prominentsize of ventricles resultof head trauma;may becomeirreversible
B . S UB DUR A LF L U IDC O L L E C T ION (braindeath)if intracranialpressureequals
( 1) S ubdu rahl y g ro m a systolicblood pressure
(2) Subduralempyema/ abscess(dueto meningitis)
(3) Subduralhematoma r/ decreaseddistinctionbetweengray + white matter
. { superficialcorticalveins are preventedfrom r/ compressedslitlikelateralventricles
crossingtge subarachnoidspace by the presence { compressionof cerebralsulci + perimesencephalic
of arachnoid/ neomembrane cisterns
{ wide interhemispheric fissure CT:
{ areasof hypodensity
SubduralFluidCollection 0 Edemais alwaysgreatestin white matter!
A. Hyperd€flse= acute subduralhematoma ./ mass effect: flatteningof gyri, displacement
B. lsodens€= sub?cutesubduralhematoma + deformationof ventricles,midlineshift
C. Hypodense r/ returnto normal from nonhemorrhagic edema/
1. Chronicsubduralhematoma brain atrophyfrom white mattershearinginjury
2. Subduralhygroma MR:
3. Effusionfrom meningo-encephalitis { decreasedintensityon TlWl, increasedintensityon
T2WI
HY P O DE NS E B R A IN L E SION S { enhancement with gadolinium
DiffuselySwollenHemispheres US:
./ generalizedlfocal increaseof parenchymal
A . M E T A B O L IC
1. Metabolicencephalopathy: eg, uremia,Reye echogenicitywith featurelessappearance
syndrome,ketoacidosis r/ decreasedresistiveindices
2. Anoxia: cardiopulmonary arrest,near-drowning,
smokeinhalation, ARDS
B. NEUROVASCULAR BrainHerniation
1. Hypertensive encephalopathy 1. S ubfal ci ne
2. Superiorsagittalsinusthrombosis contralateralshift of midlinestructuresunderfalx
3. Head trauma cerebri
4. Pseudotumor cerebri 2 . Transtentorial
C. INFLAMMATION (a) upward(superiorvermian):displacement of
eg, herpesencephalitis,CMV,toxoplasmosis cerebellumthroughtentorialincisura
(b) downward
BrainEdema anterior:uncalherniation(mostcommon)
= increasein brainvolumedue to increasedtissue- causedby lesionsin anteriorhalf of brain
water content(80% for gray matter+ 68% for white posterior: herniationof parahippocampal
matteris normal) gyrus
Etiology: total: herniationof entirehippocampus
(a) Cytotoxicedema 3 . Retroalar
reversibleincreasein intracellularwater content herniationof frontallobe posteriorlyacrossedge of
secondaryto ischemiaI anoxia(axonalpallor) sphenoidridge
 DifferentialDiagnosisof BrainDisorders 229

4. Transforaminal SuprasellarLow-densityLesionwith
herniationof inferiormesialportionsof cerebellum Hydrocephalus
downwardthroughforamenmagnum(= inferior A. CYST
tonsillar) 1. Arachnoidcyst
2. Ependymalcyst of 3rd ventricle
3. Parasiticcyst of 3rd ventricle(cysticercosis)
Cholesterol-containing
CNSLesions 4. Dilated3rd ventricle(in aqueductalstenosis)
1. Epidermoidinclusioncyst B. CYSTICMASS
2. Cholesterolgranuloma 1. E pi dermoi d
3. Acquiredepidermoidof middleear 2. Hypothalamicpilocyticastrocytoma
4. Congenitalcholesteatomaof middleear 3. Cysticcraniopharyngioma
5. Craniopharyngioma N.B.: Cysticlesionmay be inapparent within
surroundingCSF; metrizamide cisternographyis
helpfulin detection+ to excludeaqueduct
Cystwith MuralNodule stenosis
1. Pilocyticastrocytoma(childhood)
2. G anglioglio ma MultipleTiny CNSCysts
3. Pleomorphicxanthoastrocytoma A . D IFFU S ED E GE N E R A TIVD
EIS E A S E
4. Glioblastoma multiforme B. DIFFUSEINFLAMMATORY PROCESS
5. Hemangioblastoma (posterior
fossa,spinalcord) C. LOW-GRADECYSTICNEOPLASM
1. Gangl i ogl i oma
2. Pyelocyticastrocytoma
MidlineCyst 3. Pleomorphicxanthoastrocytoma
1. Cavum septi pellucidi = "5thventricle"
= thintriangularmembraneconsistingof two glial Mesencephalic
Low-densityLesion
layerscoveredlaterallywith ependymaseparating 1. Normal: decussation of superiorcerebellar
the frontalhornsof lateralventricles pedunclesat levelof inferiorcolliculi
lncidence; in 80% of term infants;in 15% of adults 2. Syringobulbia
Location: posteriorto genu of corpuscallosum, foundin conjunction with syringomyelia,
Arnold-
inferiorto bodyof corpuscallosum, Chiarimalformation, trauma
pirrar
orrornix ./ CSf densitycentrally
r/extends
,"T1"#:XTifi:f,;""rior r/ intrathecalcontrastenterscentralcavity
ri may dilate + cause obstructivehydrocephalus 3. Brainsteminfarction
(rare) { abnormalcontrastenhancementafter 1 week
2. Cavum vergae = "6th ventricle" r/ well-definedlow-attenuationregionwithout
= cavityposteriorto columnsof fornix;contracts enhancementafter 2-4 weeks
after about 6th gestationalmonth 4. Centralpontinemyelinolysis
lncidence; in 30% of term infants;in 1Sy"of adults 5. Brainstemglioma
Location: posteriorto fornix,anteriorto spfenium r/ masswith indistinctmargins+ vagueenhancement
of corpuscallosum,inferiorto bodyof 6. Metastasis
corpuscallosum,superiorto transverse r/ well-definedcontrastenhancement
fornix 7. Granulomain TB / sarcoidosis(rare)
r/ posteriormidlinecontinuationof cavum septi
pellucidibeyondforamenof Monro Intracran
ial Pneumocephal
us
3. Gavum veli interpositi Cause:
= extensionof quadrigeminalplatecisternabove A. TRAUMA Qa%\:
3rd ventricleto foramenof Monro,laterally (a) blunttrauma
boundedby columnsof fornix+ thalamus in 3'/. of all skullfractures;in 8% of fractures
4. Colloidcyst: anterior+ superiorto cavum septi involvingparanasalsinuses(frontal> ethmoid>
pellucidi sphenoid> mastoid)or base of skull
5. Arachnoidcyst: in regionof quadrigeminal plate (b) penetratinginjury
cistern B . N E OP LA S M| N V A D IN GS TN U S(1s% ):
r/ curvilinearmargins 1. Osteomaof frontal/ ethmoidsinus
2. Pituitaryadenoma
3. Mucocele,epidermoid
PosteriorFossaCystic Malformation 4. Malignancyof paranasalsinuses
1. Dandy-Walkermalformation c. TN FE C T| ON W | THGA S -FOR MINOR G GA NTSM ( 9%) :
2. Dandy-Walkervariant in mastoiditis, sinusitis
3. Megacisterna
magna D . S U R GE R Y(4% ):
4. Arachnoidpouch hypophysectomy, paranasalsinus surgery
230 Radiology Review Manual

Mechanism (dural laceration): F. A R TE R IOV E N OU S

(1) ball-valvemechanismduringstraining,coughing, Atherosclerosis,aneurysm,AVM, occultvascular
sneezing malformation, hemangioma, subdural+ epidural
(2) vacuumphenomenonsecondaryto lossof CSF hematomas,intracerebralhemorrhage
G. LEFTOVERLs
Timeof onset: on initialpresentation(25/"), usuallyseen Lipoma,lipoidproteinosis,
lissencephaly
within4-5 days,delayup to 6 months(33%)
Mortality: 15%
Cx: (1) CSF rhinorrhea(50%) Physiologic Intracranial Calcification
/ epidural/brainabscess(25%)
(2) Meningitis 1. Pinealcalcification
(3) Extracranial
pneumocephalus = air collectionin Age: no calcification<5 years of age, in 8-10%
subaponeuroticspace at 8-14 years of age, in 40/" by 20 years of
age,2l3of adultPoPulation
{ amorphous/ ringlikecalcification <3 mm from
HYPERDENSE INTRACRANIAL LESIONS mi dl i neusual l y< 10 mm i n di amete r
ial Calcitications
lntracran { approximately30 mm above highestposterior
mnemonic; "PINEEAL" elevationof pyramids
Physiologic CAVE: pinealcalcification >14 mm suggests
lnfection pinealneoplasm(teratoma/ pinealoma)
Neoplasm 2. H abenul a
Endocrine lncidence: approximatelyin 1/3 of population
Embryologic Age: >10 yearsof age
Arteriovenous ! posteriorlyopen C-shapedcalcification4-6 mm
Leftover Ls anteriorto pinealgland
3. Choroidplexus
A. PHYSIOLOGIC INTRACRANIAL CALCIFICATIONS may calcifyin all ventricles:mostcommonlyin
B . I NF E CTIO N glomuswithinatriumof lateralventricles,near
TORCH(toxoplasmosis, others[syphilis,hepatitis, foramenof Monro,tela choroideaof 3rd ventricle,
zoster],CMV, rubella,herpes),healedabscess, roof of 4th ventricle,along foraminaof Luschka
hydatidcyst,granuloma(tuberculoma, actinomycosis, Age: >3 years of age
coccidioidomycosis, cryptococcosis, mucormycosis), r/ eO-gOmm behind+ slightlybelowpinealon
trichinosis,paragonimiasis
cysticercosis, lateralprojection,symmetricalon AP projection
mnemonic: DDx: neurofibromatosis
CMV calcificationsare circumventricular 4. Dura,falx cerebri,falx cerebelli,tentorium
Toxoplasmacalcificationsare inlraparenchymal lncidence: 10/" of population
C. NE O P LA SM Age: >3 years of age
Craniopharyngioma (40-80%),oligodendroglioma DDx: basalcell nevussyndrome(Gorlin
(50-70%),chordoma(2540/"), choroidplexus syndrome),pseudoxanthoma elasticum,
papiIloma (10/"), meningioma (20/"), pituitary congenitalmyotonicdystroPhy
adenoma(3-5%),pinealoma(10-2O/"),dermoid 5. Petroclinoidligament(= reflectionof tentorium)
(20/"),lipomaof corpuscallosum,ependymoma betweentip of dorsumsellaeand apex of petrous
(50%),astrocytom a (15/"), after radiotherapy, bone
metastases(1-2/",lung > breast> Gl tract) Age: >5 years of age
N.B.: astrocytomascalcifyless frequentlybut are 6. Interclinoid ligament
the mostcommontumor = interclinoid bridging
mnemonic: 'nCa?* COME" 7. Arteriosclerosis:particularlyintracavernous
Craniopharyngioma segmentof lCA, basilara., vertebrala.
Astrocytoma,Aneurysm, 8. Basalganglia
Choroidplexuspapilloma
Oligodendroglioma
Meningioma, Medulloblastoma
Ependymoma lncreasedDensityof Falx
D. ENDOCRINE 1. Subarachnoid hemorrhage
Hyperparathyroidism, hypervitaminosis D, 2. Interhemisphericsubduralhematoma
hypoparathyroidism, pseudohypoparathyroidism, 3. Diffusecerebraledema (= increaseddensityrelative
CO poisoning,lead poisoning to low-densitybrain)
E. EMBRYOLOGIC 4. Duralcalcifications(hypercalcemia
from chronic
Neurocutaneoussyndromes (tuberoussclerosis, renalfailure,basalcell nevussyndrome,
Sturge-Weber,neurofibromatosis), Fahrdisease, hyperparathyroidism)
Cockaynesyndrome,basalcell nevussyndrome 5. Normalfalx (can be normalin pediatricpopulation)
 DifferentialDiagnosisaf Brain Disorders Zgl

Intraparenchymal
Hemorrhage OlrcoorruoRocLtoMA
mnemonic; "ITHACANS" PnRnclrovn
Infarction(hemorrhagic) 1. Ependymoma
Trauma 2. Choroidplexuspapilloma
Hypertensivehemorrhage Gnruclrocr-lovR
Arteriovenous malformation MEout-LoeLASToMA
Goagulopathy (b) Pinealtumor
Aneurysm,Amyloidangiopathy 1. Germinoma
Neoplasm:metastasis/ primaryneoplasm 2. Teratoma
Sinusthrombosis 3. Pineocytoma
4. Pineoblastoma
DenseCerebralMass (c) Pituitarytumor
Substrate; calcification/ hemorrhage/ dense protein 1. Pituitaryadenoma
A. VESSEL 2. Pituitarycarcinoma
1. A neur y s m (d) Meningioma
2. Arteriovenous malformation (e) Nervesheathtumor
3. Hematoma(acute/ subacute) 1. Schwannoma
B . T UM O R 2. Neurofibroma
1. Ly m pho ma (f) Miscellaneous
2. Medulloblastoma 1. Sarcoma
3. M ening i o m a 2. Lipoma
4. Metastasis 3. Hemangioblastoma
(a) from mucinous-producing adenocarcinoma B. TUMORSOF EMBRYONALREMNANTS
(b) hemorrhagic metastases:melanoma, (a) Craniopharyngioma
choriocarcinoma,hypernephroma, (b) Colloidcyst
bronchogenic carcinoma,breastcarcinoma (c) Teratoidtumor
(rarety) 1. E pi dermoi d
2. Dermoid
Dense Lesionnear Foramenof Monro 3. Teratoma
A . I NT RA VE N T R IC U L AR L ES IO N
1. Colloidcyst
2. M enin g i o ma Incidenceof BrainTumors
3. Choroidplexustumor/ granuloma = 9o/"of all primaryneoplasms(Sthmost common
4. AVM of septal,thalamostriate,internalcerebral primaryneoplasm);S-t 0 casesper 100,000
veins populationper year;accountfor 1.2/" of autopsied
B . P E RI V E N T R IC U L AR MA SS deaths
1. P r im a ryC N S l y m p h o m a
2. Tuberoussclerosis
(a) subependymal tuber Incidenceof BrainTumors
(b) giant cell astrocytoma
All Age Groups Pediatric Age Group
3. Metastasisfrom mucin-producing
adenocarcinoma / hemorrhagicmetastasis Glioma 34% Astrocytoma 50%
(melanoma,choriocarcinoma, hypernephroma, Meni ngi oma 17% Medulloblastoma 15%
bronchogenic carcinoma,breastcarcinoma) Metastasis 12% Ependymoma 10%
4. Glioblastoma of septumpellucidum Pituitaryadenoma 6% Craniopharyngioma 6%
C. M A S S E SPR O J EC T IN G SU PE R IOR L Y
FR OM N euri noma 4/" Choroidplexus papilloma2/"
SKULLBASE Sarcoma 3%
1. Pituitaryadenoma Granuloma 3%
2. Craniopharyngioma Craniopharyngioma 2h
3. Aneurysm Hemangioblastoma2o/"
4. Dolichoectatic basilarartery

CNSTumorsPresentingat Birth
BRAIN MASSES
1. Hypothalamic astrocytoma
Classification
of PrimaryCNSTumors 2. Choroidplexuspapilloma/ carcinoma
A . T UM O RSO F B R A INAN D ME N IN G E S 3. Teratoma
(a) Gliomas 4. Primitiveneuroectodermal
tumor
Asrnocwonan (50%) 5. Medulloblastoma
1. Astrocytoma(astrocytomagrades l-ll) 6. Ependymoma
2. Glioblastoma(astrocytoma gradeslll-lv) 7. Craniopharyngioma
232 RadiologyReviewManual

CNSTumorsin PediatricAge Group 6. Lipoma(6%)

lncidence: 7. Teratoma(3.5%)
2.4:100,000(<15yearsof age);2nd mostcommon 8. Pituitaryadenoma(3.5%)
pediatrictumor(afterleukemia);15%of all pediatric 9. Meningioma(2/")
neoplasms;15-20/" of all primarybraintumors; 10. Choroidplexuspapilloma(2%)
M>F
. increasedintracranialpressure Supratentoriallntraventricular Tumors
. increasingheadsize (a) Lateralventricle(3/4)
A. SUPRATENTORIAL (50%) 1. Choroidplexustumor(44%)
Age: first 2-3 years of life in tuberoussclerosis
2. Giantcell astrocytoma
Coveringof brain duralsarcoma,schwannoma, (1s%)
m e n i n g i o m (3
a %) 3. Hemangiomain Sturge-Weber syndrome
Cerebral (12%)
hemisphere astrocytoma(37"/.), (b) Thirdventricle(1i4)
oligodendroglioma 1. Astrocytoma(13%)
Corpuscallosum astrocytoma 2. Choroidplexustumor(6%)
3rd ventricle colloidcyst,ependymoma 3. Meni ngi oma (6% )
Lateralventricle ependymoma(5%),choroid
plexuspapilloma(12%) CLASSIFICATION BY HISTOLOGY
Optic chiasm craniopharyngioma (12/"), 1. Astrocytictumors(33.5%)
opticnerveglioma(13%), 2. "Primitive"neuroectodermal tumor= PNET (21%)
teratoma,pituitaryadenoma highlymalignantneoplasmsoriginating from
Hypothalamus glioma(8%),hamartoma germinalmatrix+ containingglial+ neural
Pinealregion germinoma,pinealoma, elements
teratoma(8%) - Medulloblastoma (16%)
B. TNFRATENTORIAL (50%) - Ependymoblasto ma (2.5%)
Age: 4-11 years - PNETof cerebralhemisphere(2.5%)
Cerebellum : astrocytoma(31-33%), PNET/ 3 . Mixedgliomas(16%)
meduIloblastoma(26-31%) 4. Malformative tumors(11.5%)
Brainstem : glioma(16-21%) - Craniopharyngioma (5.5%)
4thventricle : ependymoma(6-14%), choroid - Lipoma(4.5%)
plexuspapilloma - Dermoidcyst (1%)
- Epidermalcyst (0.5%)
mnemonic.'"BE MACHO" 5. Choroidplexustumors(4%)
Brainstemglioma 6. Ependymaltumors(%)
Ependymoma 7. Tumorsof meningealtissues(3.5%)
Medulloblastoma - Meni ngi oma(3% )
AVM - Meningealsarcoma(0.5%)
Cysticastrocytoma 8. Germcelltumors(2.5%)
Hemangioblastoma - Germi noma(1.5% )
Other - Teratomatous tumor(1%)
9. Neuronaltumors
- Gangliocytoma (1.5%)
PNET EpendymomaAstrocytoma 10. Tumorsof neuroendocri ne ori gi n
- Pituitaryadenoma(1%)
CT hyper iso hypo
T2Wl intermed. intermed. increased tumors(0.5%)
1 1 .Oligodendroglial
Enhancement moderate minimal nodule 12. Tumorsof bloodvessel
- H emangi oma (1% )
Calcification 10-15% 40-50% <10o/o
Cyst formation rare common typical
rare tare acousttc
CSF seeding 1540%
neuroma
Foraminal no yes no
spread
pituitary
adenoma
SupratentorialMidline Tumors
1. glioma(39%)
Optic+ hypothalamic
2. Craniopharyngioma(20%)
3. Astrocytoma(9%)
4. Pineoblastoma(9%)
5. Germinoma(6%) Intracranial Tirmors in Adult Population
 DifferentialDiagnosisof BrainDisorders 233

SuperficialGliomas JugularForamenMass
= peripherallylocatedcorticalneoplasmsservingas a A. NONNEOPLASTIC ENTITIES
seizurefocus 1. Asymmetrically enlargedjugularforamen
1. G angliogli o ma j ugul arbul b
2. H i gh-ri di ng
2. Desmoplastic infantileganglioglioma 3. Dehiscentjugular bulb
3. Gangliocytoma . pulsatiletinnitus
4. Dysplasticcerebellargangliocytoma . vasculartympanicmembrane
5. Pleomorphicxanthoastrocytoma r/ middleear soft-tissuemass contiguouswith
6. Dysembryoplastic neuroepithelialtumor jugularforamen(= jugularbulbbulginginto
middleear cavity)
MultifocalCNSTumors ^/ absenceof bony plate separatingjugularbulb
A. METASTASESFROM PRIMARYCNS TUMOR from posteroinferior middleear cavity
(a) via commissuralpathways:corpuscallosum, DDx: Jugularmegabulb(risesabovefloorof
internalcapsule,massaintermedia EAC but with preservationof bony plate)
(b) via CSF: ventricles/ subarachnoid cisterns 4. Jugularvein thrombosis
(c) satellitemetastases B . N E OP LA S M
B . M ULT I CE N T R IC
C N S T U MOR 1. P aragangl i oma = gl omustumor
(a) true multicentricgliomas(4%) 2. Nervesheathtumor = fleutorTl?
(b) concurrenttumorsof differenthistology 3. Meni ngi oma
(coincidental) 4. Vascularmetastasis(renali thyroidcancer)
C. M ULT I CE N T R ICME N IN G IOMA (g S % )w i thout C. PRIMARYBONE LESION
neurofibromatosis 1. Mul ti pl emyel oma
D. M ULT I CE N T R ICP R IMA R YC N S L YMP H OMA 2. Lymphoma
E . PHAKOMATOSES 3. Langerhanscell histiocytosis
1. Generalized neurofibromatosis:
meningiomatosis, bilateralacousticneuromas,
bilateralopticnervegliomas,cerebralgliomas, DumbbellMassSpanningPetrousApex
choroidplexuspapillomas,multiplespine 1. Largetrigeminalschwannoma
tumors,AVMs 2. Meni ngi oma
2. Tuberoussclerosis: 3. Epidermoidcyst
subependymal tubers,intraventricular gliomas
(giantcell astrocytoma),ependymomas
3. von Hippel-Lindau disease: PosteriorFossaTumorIn Adult
retinalangiomatosis, hemangioblastomas,
congenitalcysts of pancreas+ liver,benign Extraaxial lntraaxial
renaltumors,cardiacrhabdomyomas
1. Acousticneuroma 1. (lung,breast)
Metastasis
2. Meningioma 2. Hemangioblastoma
CNSTumorsMetastasizing
OutsideCNS 3. Chordoma 3. Lymphoma
mnemonic.' "MEGO" 4. Choroidplexuspapilloma 4. Lipoma
Medulloblastoma 5. Epidermoid 5. Glioma
Ependymoma
Glioblastomamultiforme
Oligodendroglioma Cerebellopontine
AngleTumor
= extraaxialtumorarisingin CSF-filledspaceboundby
CalcifiedIntracranial
Mass pons + cerebellarhemisphere+ petrousbone
mnemonic: "Caz*COME" lncidence; 5-1 0/" of all intracranialtumors
Craniopharyngioma . cranialneuropathy:highfrequencyhearingloss (n.
Astrocytoma,Aneurysm Vlll), tinnitus+ facialmotordysfunction(n. V ll), facial
Choroidplexuspapilloma sensorydysfunction(n. V), taste disturbance(chorda
Oligodendroglioma tympani)
M eningiom a . signsof posteriorfossa mass effect: headache,
Ependymoma nausea,vomiting,disequilibrium, ataxia
. hemifacialspasm,trigeminalneuralgia(tic
AvascularMassof Brain douloureux)
mnemonic.' "TEACH" ./ may widen CSF space (cistern)in 25h
Tumor: astrocytoma,
metastasis,
oligodendroglioma r/ bone erosion/ hyperostosis
Edema { sharpmargination with brain
Abscess Types:
Cyst,Contusion 1. Acousticneuroma= schwannoma(80-90%):
Hematoma,Herpes from intracanalicular portionof 8th cranialnerve
234 Radiology Review Manual

2. M ening i o m (1a 0 -1 8 % ) CysticMassin CerebellarHemisphere

2nd most commonextraaxialmass in posterior 1. H emangi obl astoma
fossa;<5/" of all intracranial larger
meningiomas; 2. Cerebellarastrocytoma
+ more hemispheric in shape+ more 3. Metastasis
homogeneously enhancingthanacousticneuroma 4. Lateralmedulloblastoma(= "cerebellarsarcoma")
3. Epidermoidinclusioncyst (5-9%) 5. Choroidplexuspapillomawith lateralextension
4. Arachnoidcyst (<1%)
5. Aneurysmof basilar/ vertebrali posteriorinferior
cerebellarartery: LesionExpandingCavernousSinus
congenitalberryaneurysm/ saccularaneurysm/ A . TU MOR
atherosclerotic dolichoectasia schw annoma
1. Tri gemi nal
6 . Choroidplexuspapilloma 2. Pituitaryadenoma
7 . Ependymoma 3. Parasellarmeningioma
8. Trigeminalneuroma 4. Parasellarmetastasis
from gasserianganglionwithinMeckelcave in 5. Invasionby tumorof skullbase
the most anteromedialportionof petrouspyramid B. VESSEL
/ trigeminalnerve root 1. lnternalcarotidarteryaneurysm
9. G lom u sj u g u l a retu m o r 2. Carotid-cavernous fistula
withinadventitiaof bulb of jugularvein at base of 3. Cavernoussinusthrombosis
petrousbone with invasionof posteriorfossa C. TOLOSA.HUNTSYNDROME
10. Chor do m a = granulomatous invasionof cavernoussinus
11. Exophyticbrainstemglioma
Histo: usuallydiffusefibrillaryastrocytoma
12. Metastasis(0.2-2%) CorpusCallosumLesion
13. Lipoma(<1%) A . TU MOR
1. GBM
mnemonic.' "EverGrave GerebelloPontineAngle
2. Lymphoma
Masses"
3. Metastasis
Epidermoid
B. TRAUMA
Glomusjugularetumor
1. S heari ngi nj ury
Chondroma,Ghordoma,Cholesteatoma
Pituitarytumor,Pontineglioma(exophytic) c. W H ITEMA TTE RD IS E A S E
1. Mul ti pl escl erosi s
Acoustic+ trigeminalneuroma,Aneurysmof basilar
2. Progressivemultifocalleukoencephalopathy
/ vertebralartery,Arachnoidcyst
Meningioma,Metastasis 3. AdrenoleukodystroPhy
4. Marchiafava-Bignami disease
c. IN FE C TION
Meningioma Schwannoma 1. Toxoplasmosis
Anglewith dura obtuse acute
Duraltail frequent rare Ring-enhancing Lesion Crossing Corpus
Calcification 20/" rare Callosum
Cystic/necrotic rare 10% mnemonic.' "GAL"
fAC involvement rare 80% Glioblastomamultiforme(butterflyglioma)
NECT hyperdense isodense Astrocytoma
Enhancement uniform 32"/"nonuniform Lymphoma

Low-attenuationExtraaxial Lesion E N H A N C IN G B R A IN LE S ION S

1. Acoustic (occasionally
schwannoma low-density GyralEnhancement
mass) A . M E N I N G E ATLU M O R
2. Epidermoidtumor (a) Meningealcarcinomatosis from systemictumor:
3. Arachnoidcyst eg, breastcarcinoma,smallcellcarcinomaof
l ung,mal i gnantmel anoma, l ymphom a/ leukem ia
(b) SeedingprimaryCNS tumor:
Epidermoid Arachnoid
1. Medul l obl astoma
CT density + hyperdense to CSF CSF-like 2. Pineoblastoma
Margins scalloped smooth 3. EPendYmoma
Vessels encased disPlaced B. MENINGITIS
Protondensity deviatesfrom CSF CSF-like pyogenic,tuberculous, fungal,cysticercosis,
Diffusion restricted CSF-like sarcoidosis
 DifferentialDiagnosisof BrainDisorders 2gs

C. S E Q UE LAEO F SU BA R A C H N OID
H EMOR R H A GE Small SphericalRing-enhancing Lesionat
(from f ibroblasticproliferation)
CorticomedullaryMargin + SubstantialAmount
D. SUBACUTEBRAININFARCT
of VasogenicEdema
mnemonic; "CAL MICE" 1. Metastasis
Cerebritis 2. Abscessof brain
Arteriovenousmalformation (a) bacterial,fungal,granulomatous
Lymphoma (b) parasitic:cysticercosis,paragonimiasis,
Meningitis echinococcus
Infarct 3. Subacuteinfarction
Carcinomatosis 4. Resolvinghematoma
Encephalitis
Dense& EnhancingLesions
SolitaryRing-enhancing
Lesionof Brain 1. Aneurysm
Cause: 2. Meni ngi oma
A. NEOPLASM 3. C N S l ymphoma
1. Primaryneoplasm: high-gradeglioma, 4. Medulloblastoma
meningioma, lymphoma,leukemia,pituitary 5. Metastasis
macroadenoma,acousticneuroma,
craniopharyngioma MultifocalEnhancingLesions
2. Metastaticcarcinoma+ sarcoma 1. Multipleinfarctions
B. ABSCESS 2. Arteriovenous malformations
1. Abscess:bacterial,fungal,parasitic 3. Multifocalprimary/ secondaryneoplasms
2. Empyemaof epidural/ subdural/ 4. Multifocalinfectiousprocesses
intraventricularspaces 5. Demyelinating diseases:eg, multiplesclerosis
C. HE M O R R H A G IC -ISC H E MLICE SION
1. Resolvinginfarction Innumerable
SmallEnhancingCerebralNodules
2. Aginghematoma A. METASTASES
3. Operativebed followingresection B . P R IMA R YC N S LY MP H OMA
4. Thrombosedaneurysm C . D IS S E M]N A TEIN
D FE C TION
D. DEMYELINATING DISORDER 1. Cysticercosis
1. Radiationnecrosis 2. Histoplasmosis
2. Tumefactive demyelinating lesion(,,singular 3. Tuberculosis
sclerosis") D. INFLAMMATION
3. Necrotizingleukoencephalopathy after 1. Sarcoidosis
methotrexate 2. Multiplesclerosis
Pathogenesis: E. SUBACUTEMULTIFOCALINFARCTION
(1) hypervascularmarginof lesion= granulation from hypoperfusion,multipleemboli,cerebral
tissue/ peripheralvascularchannels/ vasculitis(SLE),meningitis,corticalvein thrombosis
hypervasculartumor capsule
(2) breakdownof blood-brainbarrier= leakageof EnhancingLesionin InternalAuditoryCanal
contrastout of abnormallypermeablevesselsinto A. NEOPLASTIC
extracellularfluidspace 1. Acousticschwannoma
(3) hypodensecentef = €rV€lscular / hypovascular 2. Ossifyinghemangioma
(requirestime to fill)/ cysticdegeneration B. NONNEOPLASTIC
lncidence of ring blush: 1. Sarcoidosis
abscess(in73%);glioblastoma (in 48%);metastasis 2. Meningitis
(in 33%);grade ll astrocytoma(in 26%)INOT in grade 3. Postmeningitic
/ postcraniotomy fibrosis
I astrocytomal 4. Vascularloopof anteriorinferiorcerebellara.

mnemonic; "MAGICALDR"
Metastasis VASCULAR DISEASE OF BRAIN
Abscess/ cerebritis Classification
of VascularCNSAnomalies
Glioblastoma multiforme,Glioma A. VASCULARMALFORMATION
lnfarct(resolving),lmpact (a) arterial= arteriovenousmalformation(AVM)
Contusion 1. Facial/ brainarteriovenousmalformation
AIDS toxoplasmosis 2. Vein of Galenmalformation
Lymphoma(oftenAl DS-related) (b) capillary= telangiectasia
Demyelinatingdisease 1. Facialport wine stain
Radiationnecrosis,Resolvinghematoma . commonlyasymptomatic
236 Radiology Heview Manual

(c) venous = V€IloUSmalformation 3. Watershedinfarct

= tangleof abnormalvaricesof a "caput- involvingdeep white matterbetweentwo adjacenl
medusae"/ "spoked-wheel" configuration vascularbeds in global hypoperfusionsecondary
draininginto a dilatedcorticalvein to poor cardiacoutput/ cervicalcarotidartery
. soft + compressible withoutthrills/ pulsations occlusion
. distensionwith Valsalvamaneuver 0 6% of cerebralinfarctshave hemorrhage(red
. commonlyasymptomatic infarct)
Location: white matterwith normalintervening , stroke(3rd most commoncause of death in
brainparenchyma .USA,5/" of strokesyndromesare causedby
Cx (uncommon): hemorrhage,ischemia underlyingtumor)
1. V en o u sa n g i o ma . TIA = transitoryischemicattack: clearswithin
2. Sinuspericranii 24 hours
(d) lymphatic . RIND= reVefsible ischemicneurologicdeficit:
1. Cystichygroma stillevident>24 hourswith eventualtotal recovery
(e) combinations . amaurosisfugax= transientmonocularblindness
1. Sturge-Weberdisease . weakness/ numbnessin an extremity
2. Rendu-Osler-Weber disease . aphasia
B. VASCULARTUMOR . dizziness,diplopia,dysarthria(vertebrobasilar
1. Hem an g i o m a ischemia)
(a) capillaryhemangioma:seen in children, 4. Hypertension
involutionby 7 years of age in 95% (a) HypertensiveencePhaloPathY
(b) cavernoushemangioma:seen in adults,no { diffusewhite matterhypodensity(edema
involution secondaryto arterialsPasm)
r/ thrombosedblood + hemosiderin (b) Hypertensivehemorrhage
r/ normalangiogram Location:basalganglia(putamen,external
2. Hemangiopericytoma capsule),thalamus,pons,cerebellum
3. Hemangioendothelioma (c) Lacunarinfarction
4. Angiosarcoma (d) Subcorticalarteriosclerotic encephalopathy
5. Amyloidosis
Occult/ crypticvascularmalformation involvement of small-+ medium-sized arteriesof
1. Cavernoushemangioma meninges+ cortex
2. Capillarytelangiectasia . normotensivepatient>65 years of age
{ muftiplesimultaneous / recurrentcortical
OcclusiveVascularDisease hemorrhages
(a) Embolicstate: 6. Vasculitis
r/ singlevascularterritory (a) Bacterialmeningitis, TB, syphilis,fungus,virus,
(b) Hypoperfusivestate: rickettsia
r/ multiplevascularterritories (b) Collagen-vascular disease:Wegener
Cause: granulomatosis, polyarteritisnodosa,SLE,
1. Vasospasmfrom subarachnoid hemorrhage scleroderma,dermatomYositis
2. Embolicinfarction(50%) (c) Granulomatousangitis: giant cell arteritis,
(a) thrombus(atrialfibrillation,
valvulardisease, sarcoidosis,Takayasudisease,temporal
Atheromatousplaquesof extracerebral arteritis
arteries,fibromusculardysplasia,intracranial (d) lnflammatoryarteritis: rheumatoidarteritis,
aneurysm,surgery,paradoxicemboli,sickle hypersensitivity arteritis,Behgetdisease,
cell disease,atherosclerosis,thrombotic lymphomatoid granulomatosis
thrombocytopenic purpura) (e) Drug-induced:lV amphetamine, ergot
. fluctuatingblood pressures preparations,oral contraceptives
. hypercoagulability (f) Radiationarteritis= mineralizing
{ cerebralpetechialhemorrhagewithincortical microangiopathy
/ basalgray matterduring2nd week (from (g) Moyamoyadisease
fragmentsof embolus)in up to 40/"; initial 7. AnoxicencephalopathY
ischemiais followedby reperfusion cardiorespiratory arrest,near-drowning, drug
(= HALLMARKof embolicinfarction) overdose,CO poisoning
{ "supernormal artery"on NECT= high-density 8. Venousthrombosis
materiallodgedin cerebralvesselnear major
bifurcations Multiple lnfarctions
{ atheromatousnarrowingof vessels 0 Typicalin extracranialocclusivedisease,cardiac
(b) fat outputproblems,smallvesseldisease;in 6% from
(c) nitrogen a showerof emboli
 Differential Diagnosis of Brain Disorders 237

Location: usuallybilateral+ supratentorial(3/4); lateralsylvian frontal,frontotemporal,

supra-and infratentorial(1/4) parietotemporal
centralsylvian deep posteriorfrontal,
Displacementof Vessels basalganglia
A . A RT E RI A LS H IF T B . C E R E B R A LV E IN S
(a) Pericallosalarteries = indicatethe midlineof the posteriorpart of the
1. Roundshift= frontallesionanteriorto coronal forebrainshowingthe exact locationof the roof
suture of the 3rd ventricle
2. Squareshift= lesionbehindforamenof
Monroin lowerhalfof hemisphere B R A IN V E N TR IC LE S
3. Distalshift= posteriorto coronalsuturein Ventriculomegaly
upperhalfof hemisphere A. MACROCEPHALY
4. Proximalshift = basifrontallesion/ anterior . increasedintraventricularpressure
middlecranialfossaincludinganterior (a) Obstructionto CSF flow
temporallobe 1. Communicating hydrocephalus
(b) Sylviantriangle 2. Noncommunicating hydrocephalus
= branchesof MCA withinsylvianfissureon (b) Overproductionof CSF = hor'tobstructive
outer surfaceof insulaform a loop upon hydrocephalus
reachingthe uppermarginof the insula;serves (c) Neoplasm
as angiographic landmarkfor localizing
supratentorialmasses B . MIC R OC E P H A LY
. hofffialintraventricular
pressure
Locationof lesion: (a) Primaryfailureof braingrowth
anteriorsylvian frontalregion - dysgenesis
- suprasylvian posteriorfrontal+ parietal 1. Holoprosencephaly
retrosylvian occipital,parieto-occipital 2. Aneuploidysyndromes(trisomies)
infrasylvian temporallobe + 3. Migrational(<6 layers)
extracerebralregion - environment:alcohol,drugs,toxins
intrasylvian usuallydue to meningioma - infection:TORCH

Round Shift Distal Shift Square Shift Proximal Shift

anterior

*''4 \ ."'-i

round shift

1 = anterior sylvian territory

proximal 2 = suprasylvian territory
most anterior extent 3 = retrosylvian territory
of middle cereberal a. 4 = infrasylvian territory
5 = intrasylvian territory

Sylvian Triangle and Intracranial Shifts

238 Radiology Heview Manual

(b) Lossof brainmantle PERTVENTRICULAR REGION

- Infection:TORCH HypodensitY
Periventricular
- Vascularaccident: 1. E ncephal omal aci a
1. Hydranencephaly r/ slightlydenserthan CSF
2. Schizencephaly 2. Porencephaly
3. Porencephaly = cavitycommunicatingwith ventricle/ cisternfrom
Hemorrhage: intracerebralhemorrhage
1. Porencephaly Associatedwith: dilated ventricle,sulci, and fissures
2. Leukomalacia r/ CSf density
C. NORMOCEPHALY 3. Resolvinghematoma
. historyof previouslydemonstratedhematoma
{ may show ringenhancement + compression of
Colpocephaly adjacentstructures
= dilatationof trigones+ occipitalhorns+ posterior 4. Cystictumor
temporalhornsof lateralventricles ^/ mass effect+ contrastenhancement
1. Agenesisof corpuscallosum
2. Arnold-Chiari malformation EnhancingVentricularMargins
3. Holoprosencephaly (a) Subependymalspreadof metastatictumor
1. Bronchogenic carcinoma(especially
smallcell
carcinoma)
Tumor
Intraventricular 2. Mel anoma
Prevalence: 10/" of all intracranialneoplasms 3. Breastcarcinoma
1. E pendy mo ma 20% (b) Subependymal seedingof CNS primary
2. Astrocytoma 18% 1. Glioma
3. Colloidcyst 12% 2. Ependymoma
4. M eningi o ma 11% (c) Ependymalseedingof CNS primary
5. Choroidplexuspapilloma 7% 1. Medul l obl astoma
6. Epidermoid / dermoid 6% 2. Germi noma
7. Craniopharyngioma 6% (d) PrimaryCNS lymphoma/ systemiclymphoma
8. Medulloblastoma 5% (e) Inflammatory ventriculitis
9. Cysticercosis 5/"
10. Arachnoidcyst 4o/" Periventricular in Childhood
Calcifications
11. S ubepen d y mo ma 2/o 1. Tuberous
sclerosis
12. AVM 2o/o 2. Congenitalinfection:CMV,toxoplasmosis
13. Teratoma 1"/"
14. Metastasis
15. Intraventricular
neurocytoma Periventricular Lesions
T2Wl-hyperintense
16. Oligodendroglioma A. YOUNGPATIENTS
1. Multiplesclerosis
2. Migraine:in 41ohwith classicmigraine , in 57"/"
Tumor in 4th Ventricle with complicatedmigraine;presumedto
1. plexuspapilloma
Choroid representvasculitis-induced small infarcts
2. Ependymoma / glioma 3. Vasculiticdisorder:SLE, Behgetdisease,sickle
3. Hemangioblastoma cell disease
4. Vermianmetastasis r/ triad of deep white matterlesions+ cortical
5. AVM infarcts+ hemorrhage
6. tumor(rare)
Epidermoid 4. Acutedisseminated encephalomyelitis (ADE)
7. Inflammatory
mass = postviralleukoencePhaloPathY
8. Cyst 5. Virchow-Robinspace
= SrTl€lllinvaginationsof subarachnoidspace
followingpia materalongperforatingnutrient
Tumor in 3rd Ventricle end vesselsinto brain substance
1. Colloid
cyst Location: inferiorthirdof putamen;usually
2. Glioma bilateral
3. Aneurysm r/ t-Z-mm roundlesionsisointense to CSF (well
4. Craniopharyngioma seen on coronal sections through centrum
5. Ependymoma semiovale+ on low-axialsectionsat levelof
6. Meningioma anteriorcommissure)
7. Choroidplexuspapilloma 6. Leukodystrophy:in children
8. Intraventricular
neurocytoma ./ symmetricdiffuseconfluentinvolvement
 Differential Diagnosis of Brain Disorders 2gg

7. Ependymitis granularis BASALGANGLIA

= syffirnetricallyfocal areasof hyperintensity on
T2Wl anterior+ lateralto frontalhorns in
normalindividuals
Histo: patchyloss of ependymawith paucity
of hydrophobic myelin,whichallows
migrationof fluidout of the ventricle
into interstitium
B . E LDE RLY
1. Etat cribl6 (sievetike) i gtiosis
= deep whitematterischemia
= extensivenumberof perivascularfluidspaces
predominantlyat arteriolarlevelas part of
subacutearteriosclerotic encephalopathy
Cause: chronicischemiadue to
arteriosclerosis of long penetrating
arteriesarisingfrom circleof Willis
(lenticulostriate + thalamo-perforators)
= SrT]?llvesseldisease
Predisposed: cigarettesmoker, hypertensive
patient
Histo: lipohyalindepositswithinvesselwalls
followedby partialdemyelination,
gliosis,interstitialedema
lncidence; in 10% withoutrisk factors,in g4/o
with risk factorsand symptoms
Age: >60 years (in 30-60%)
Location: periventricular white matter> optic
radiation> basalganglia> centrum
semiovale> brainstem(usually
sparescorpuscallosum+ subcortical
U-fibers)
r/ multiplefocal lesions<2 mm
2. Lacunarinfarction
C. PAT]ENTSWITHAIDS
1. HI V enc e p h a l i ti s :
"patchy" ,,dirtywhite
r/ well-defined / ill-defined
matter"
r/ centralatrophy
2. Toxoplasmosis
3. Lymphoma
4. Progressivemultifocalleukoencephalopathy
D. PATIENTSWITHTRAUMA
1. Diffuseaxonal/ shearinginjury
2. Diffusewhite matterinjury
= radiation-induced demyelination of
periventricular white matter
Cause: whole-brainirradiation
. subclinical
3. Diffusenecrotizingleukoencephalopathy
Cause: intrathecalmethotrexate+ whole brain
irradiation
. rapidlydeteriorating
clinicalcourse
r/ confluentpatternwith scallopedmarginswithin
periventricular white matterextendingout to
subcorticalU-fibers
E. PATIENTSWITH HYDROCEPHALUS
1. Transependymal CSF flow
r/ smoothhalo of even thickness
BilateralBasalGangliaLesionsin Childhood
0 Basalgangliaare susceptible to damageduring
childhoodbecauseof highenergyrequirements (ATp)
mandatinga richbloodsupply+ highconcentration of
tracemetals(iron,copper,manganese)
. increasedirritability,lethargy,dystonia
. seizure,behavioralchanges
r/ bilateralnecrosisof basalganglia
ACUTECAUSES
A. Compromiseof vascularsupply
1. Hemolytic-uremic syndrome
causingmicrothrombosis of basalganglia,
thalami,hippocampi, cortex
2. Encephalitis (usuallyviralagents)
B. Compromiseof nutrientsupply
1. Hypoxia:respiratory arrest,neardrowning,
strangling,barbiturateintoxication
2. Hypoglycemia
r/ hemorrhagerarelyseen
3. Osmoticmyelinolysis
{ associatedcentralpontinelocationcommon
C. Acutepoisoning
1. Carbonmonoxide
r/ preferentially
affectsglobuspallidus
rarein children:
2. Hydrogensulfide
3. Cyanidepoisoning
4. Methanolpoisoning
C H R ON ICC A U S E S
A. Inbornerrorsof metabolism
1. Lei ghdi sease
= subacutenecrotizing encephalomyelopathy
= autosomalrecessivedisordercharacterized
by deficienciesin pyruvatecarboxylase,
pyruvatedehydrogenase complex,
cytochromec oxidaseresultingin anaerobic
ATP production
. lacticacidosis(elevatedratio of lactateto
pyruvatein CSF + serum)
(pML) r/ propensityto involveputamen
2. Wilsondisease
= hepatolenticulardegeneration
= increaseddepositionof copperin brain
+ liver
. decreasedlevelsof serum copper
+ ceruloplasmin
. increasedurinarycopperexcretion
r/ celldamageof lenticularnucleus(= lenslike
configurationof putamen+ globuspallidus)
3. Mitochondrial encephalomyelopathies
= subsetof lacticacidemiaswith structurally
abnormalmitochondria
. "raggedred" fibers in musclebiopsy
4. Maplesyrupurinedisease
= inabilityto catabolizebranched-chain amino
acids(leucine,isoleucine,valine)
. urinesmellsof maplesyrup
240 RadiologyReviewManual

5. M et h y l ma l o n i c a c i d e m i a E . IN FLA MMA TION / IN FE C TION

= group of geneticallydistinctautosomal 1. Toxoplasmosis, congenitalrubella,CMV
recessivedisordersof organicacid 2. Measles,chickenPox
metabolismaffectingconversionof 3. Pertussis,CoxsackieB virus
methylmalonyl-CoA to succinyl-CoA 4. Cysticercosis
. accumulation of methylmalonic acid in blood 5. SystemiclupuserYthematosus
+ u ri n e 6. AIDS
B. Degenerativedisease F. TR A U MA
1. Hunti n g todni s e a s e 1. Childhoodleukemiafollowingmethotrexate
2. Dystrophiccalcifications theraPy
C. Dysmyelinating disease 2. S/P radiationtheraPY
basalgangliaare a mixtureof gray + whitematter 3. Birthanoxia,hYPoxia
1. Canavandisease 4. Cardiovascular event
2. MetachromaticleukodystroPhY G. TOXIC
D. Others 1. CarbonmonoxidePoisoning
1. Neurofibromatosis tyPe 1 2. Lead intoxication
3. Nephroticsyndrome
"BlRTH"
mnemonic.'
Lesionin BasalGanglia
Low-attenuation Birthanoxia
1. Poisoning:carbonmonoxide,barbiturate ldiopathic(mostcommon),Infarct
hydrogensulfidepoisoning,cyanide
intoxication, Radiationtherapy
poisoning,methanolintoxication Toxoplasmosis / CMV
2. Hypoxia Hypoparathyroidism / PseudoHPT
3. Hypoglycemia
4. Hypotension(lacunarinfarcts) LinearEchogenicFociin Thalamus+ Basal
5. Wilsondisease Ganglia
A . IN U TE R OIN FE C TION
= mineralizingvasculopathy= destructionof wall
BasalGangliaGalcification arteries+ replacement
of lenticulostriate by deposits
Prevalencein children: 1.1-1.6% of amorphousgranularbasophilicmaterial
1. TORCHagents: Toxoplasma, others(syphilis'
A . P HY S I O L OGIC W IT HA G IN G hepatitis,zoster),rubellavirus,cytomegalovirus,
B . E NDO CR IN E herpesvirus
1. Hypoparathyroidism, pseudo-,pseudopseudo- 2. S yphi l i s
(60%) 3. HumanimmunodeficiencY virus
2. Hyperparathyroidism B. CHROMOSOMAL ABNORMALITY
3. Hypothyroidism 1. D ow nsyndrome
C. METABOLIC 2. Tri somy13
1. Leighd i s e a s e C. OTHERS(anoxicinjurY?)
2. Mitochondrialcytopathy 1. Perinatalasphyxia,respiratory distress
(a) Kearns-Sayre syndrome= ophthalmoplegia, syndrome,cyanoticcongenitalheartdisease,
retinalpigmentarydegeneration, complete necrotizing enterocolitis
heartblock,short stature,mentaldeterioration 2. FetalalcoholsYndrome
(b) MELAS = Mitochondrialmyopathy, 3. N oni mmune hydroP s
Encephalopathy, Lacticacidosis,And Stroke
(c) MERRF= MyoclonicEpilepsywith Ragged Eye-of-the-Tiger Sign
Red Fibers = rT'ldrkedly
hypointense globuspalliduson T2Wl
3. Fahrdisease= familialcerebrovascular surrounding a center
higher-intensity
ferrocalcinosis Cause: excessironaccumulation + centralgliosis
D. CO NG E N IT A/LD EV EL OP M EN T AL Associated with: Hallervorden-Spatz syndrome
1. Familialidiopathicsymmetricbasalganglia (Hallervorden-Spatz disease,
calcification dementia,tetraparesis,
2 . Hastings-James syndrome tangles,retinitis
neurofibrillary
3 . Cockaynesyndrome pigmentosa,acanthocYtosis,
4 . Lipoidproteinosis = hyalinosiscutis pallidaldegeneration,X-linked
5 . Neurofibromatosis disorderswith mental
6. Tuberoussclerosis retardation+ Dandy-Walker
7 . Oculocraniosomatic disease malformation,disorderswith
8 . Methemoglobinopathy Lewy bodies); extrapyramidal
L Down syndrome parkinsoniandisorders
 DifferentialDiagnosisof BrainDisorders 241

SELLA Pituitary Gland Enlargement

Destructionof Sella 1. Neoplasm:eg, pituitaryglandadenoma
1. Pituitaryadenoma 2. Hypertrophy:primaryprecociouspuberty,primary
2. Suprasellar tumor hypothyroidism
3. Carcinomaof sphenoid+ posteriorethmoidsinus 3. Lymphocytichypophysitis
r/ opacificationof sinus + destructionof walls 4. lnfection
r/ associatedwith nasopharyngealmass (common) 5. SevereduralAV fistula
4. Nasopharyngeal carcinoma
(a) squamouscell carcinoma lntrasellarMass
(b) lymphoepithelioma = Schmincketumor= t-toft- 1. Pituitaryadenoma/ carcinoma(mostcommon
keratinizingform of squamouscell carcinoma cause)
r/ sclerosisof adjacentbone 2. Craniopharyngioma (2nd mostcommoncause)
5. Metastasisto sphenoid 3. Meningioma:from surfaceof diaphragm/
from breast,kidney,thyroid,colon,prostate,lung, tuberculumsellae
esophagus 4 . Chordoma
6. Primarytumorof sphenoidbone (rare) 5 . Metastasis:lung,breast,prostate,kidney,Gl tract,
osteogenicsarcoma,giantcelltumor,plasmacytoma spreadfrom nasopharynx
7. Chordoma 6. lntracavernousICA aneurysm: bilateralin 25/"
8. Mucoceleof sphenoidsinus(uncommon) 7 . Pituitaryabscess: rapidlyexpandingmass
9. Enlarged3rd ventricle associatedwith meningitis
aqueductalstenosisfrom infratentorial mass, 8 . Emptysella
maldevelopment 9 . Rathkecleft cyst: commonlyat junctionof anterior
+ posteriorpituitarygland
1 0 . Granularcell tumor= myeloblastoma:benign
J-shaped Sella neoplasmof posteriorpituitarygland
mnemonic.' "CONMAN" 1 1 .Granuloma:sarcoidosis, giantcell granuloma,TB,
Chronichydrocephalus syphilis,eosinophilicgranuloma
OpticAlioma,Osteogenesisimperfecta 1 2 . Lymphoidadenohypophysitis
Neurofibromatosis 1 3 . Pituitaryhyperplasia, eg, in Nelsonsyndrome
Mucopolysaccharidosis
Achondroplasia Hypointense Lesion of Sella
Normalvariant 1. E mptysel l a
2. Pituitarystone(= pituilith)
= segUeldeof autonecrosisof pituitaryadenoma
EnlargedSella 3. Intrasellaraneurysm
A. PRIMARY TUMOR 4. Persistent trigeminalartery
1. Pituitaryadenoma 5. Calcifiedmeningioma
2. Craniopharyngioma 6. Pituitaryhemochromatosis (anteriorpituitarylobe
3. Meningioma: hyperostosis onl y)
4. Opticglioma: J-shapedsella
B . P I T UI T A R Y
H Y PE R P L AS IA
1. Hypothyroidism Parasellar Mass
2. Hypogonadism 1. Meni ngi oma:tentori um cerebel l i
3. Nelsonsyndrome(occurringinTo/"of patients (l l l , l V , V 1, V 2,V l )
2. N euri noma
subsequentto adrenalectomy) 3. Metastasis:lung,breast,kidney,Gl tract,spread
C. CS F S P A C E from nasopharynx
1. EnlargedSrdventricle 4. Epidermoid
2. Hydrocephalus 5. Aneurysm
3. Emptysella 6. Carotid-cavernous fistula
D. V E S S E L
1. Arterialaneurysm mnemonic; "SATCHMO"
2. Ectaticinternalcarotidartery Setlaneoplasmwith superiorextension,Sarcoidosis
Aneurysm,ectaticcarotid,carotid-cavernoussinus
mnemonic; "CHAMPS" fistula,Arachnoidcyst
Craniopharyngioma Teratoma:dysgerminoma (usually),dermoid,
Hydrocephalus (emptysella) epidermoid
AVM, Aneurysm Craniopharyngioma, Chordoma
M eningioma Hypothalamic glioma,Histiocytoma,
Hamartoma
Pituitaryadenoma Metastaticdisease,Meningioma,Mucocele
Sarcoidosis,TB Opticnerveglioma,neuroma
242 RadiologyReview Manual

SuprasellarMass B . GR A N U LA R
1. Meningioma 1, C rani opharyngi oma
2. Craniopharyngioma: in 80% suprasellar 2. Meni ngi oma
3. Chiasmal+ opticnerveglioma 3, Granul oma
in 38% of neurofibromatosis; adolescentgirls; 4. Dermoidcyst/ teratoma
DDx: chiasmalneuritis 5. Optic/ hypothalamicglioma(rare)
4. Hypothalamic glioma
5. Hamartomaof tubercinereum EnhancingSupra-and lntrasellarMass
6. lnfundibular tumor 1. Pituitaryadenoma
metastasis(esp.breast);glioma;lymphoma/ 2. Meni ngi oma
leukemia;histiocytosis X; sarcoidosis,
tuberculosis 3. Germi noma
r/ diameterof infundibulum >4.5 mm immediately 4. Hypothalamic glioma
abovelevelof dorsum;cone-shaped(on coronal 5. Craniopharyngioma
scan)
7. G er m inom a
= malignanttumor similarto seminoma(= "ectopic PerisellarVascularLesion
pinealoma") 1. IC Aaneurysm
^/ frequentlycalcified(teratoma) Giantaneurysmsare >2.5cm in diameter
ri CSf spread(germinoma+ teratocarcinoma) { destruction of bonysella/ superiororbitalfissure
r/ enhancementon CECT (common) { calcifiedwall i thrombus
L Epidermoid/ dermoid .i CfCf enhancement, nonuniform with thrombosis
{ cysticlesioncontainingcalcifications + fat 2. Ectaticcarotid arlerY
r/ minimal/ no contrastenhancement { curvilinearcatci{ications
9 . Arachnoidcyst r/ encroachment uponsellaturcica
' hydrocephalus (common),visualimpairment 3. Carotid-cavernous sinusfistula
. endocrinedysfunction
Age: mostcommonin infancy
1 0 . Enlarged3rd ventricleextendinginto pituitaryfossa P IN E A L GLA N D
1 1 .Suprasellaraneurysm of PinealGlandTumors
Classification
r/ rim calcification+ eccentricposition lncidenceof pineal mass:
<1o/o tumors,4o/oof all childhood
of all intracranial
intracranial masses,9/" of all intracranialmassesin
Suprasellar Masswith Low Attenuation Asia
1. Cr aniop h a ry n g i o ma A . P R IMA R YTU MOR
2. Dermoid/ epidermoid (a) Germ cell origin(2/3)
3. Arachnoidcyst - formingembryonictissue
4. Lipoma 1. Germinoma(40-50%)
5. Simplepituitarycyst 2. Embryonalcell carcinoma
6. Gliomaof hypothalamus 3. Teratoma(15%): benignmatureteratoma,
benignimmatureteratoma,malignant
teratoma
SuprasellarMasswith MixedAttenuation - formingextraembrYonic tissue
A. INCHILDREN 4. Choriocarcinoma (<5%)
1. Hypothalamic-chiasmatic glioma 5. Endodermalsinustumor= yolk Sactumor
2. Craniopharyngioma (b) Pinealparenchymal cell origin(<15%)
3. Hamartomaof tubercinereum 1. P i neocytoma
4. Histiocytosis 2. Pineoblastoma
B . I N A DUL T S (c) Othercell origin
1. Suprasellarextensionof pituitaryadenoma 1. Retinoblastoma (trilateral = left
retinoblastoma
2. Craniopharyngioma eye + righteYe+ Pinealgland
3. Epidermoidcyst 2. Astrocytoma
4. Thrombosedaneurysm 3. E pendymoma
5. Low-gradehypothalamic / opticglioma 4. Meningioma
6. lnflammatory lesion: sarcoidosis,TB, sphenoid 5. Hemangiopericytoma
mucocele 6. Pineal+ tectalglioma
7. Cavernoushemangioma
Suprasellar Mass with Calcification 8. Meni ngi oma
A . CURV I L IN EA R (d) Cysts
1. Giantcarotidaneurysm 1. Pinealcyst
2. Craniopharyngioma 2. Malignantteratoma
 DifferentialDiagnosisof BrainDisorders 243

3. AVM, vein of Galenaneurysm Serummarkers:

4. Arachnoidcyst choriocarcinoma - p-HcG
5. Inclusioncyst (dermoid/ epidermoid) embryonalcell carcinoma- cr-FPand B-HCG
B. SECONDARYTUMOR endodermalsinustumor - cr-FP
Metastasis' rg, lungcarcinoma teratoma - B-HCGandu-FP
germinoma - placental
alkaline
phosphatase
DDxconsiderations:
- female: likelyNOT germ celltumor
- hypodensematrix: likelyNOT pinealcelltumor IntenselyEnhancingMassin PinealRegion
- distinct
tumormargins:probablypineocytoma/ 1. Germinoma
teratoma/ germinoma 2. Pineocytoma / -blastoma
- calcification: likelyNOT teratocarcinoma, 3. Pinealteratocarcinoma
metastasis,germinoma 4. Gliomaof brainstem/ thalamus
- CSFseeding: NOT teratoma 5. Subsplenialmeningioma
- intense
enhancement:
likelyNOT teratoma 6. Vein of Galenaneurysm
244 Radiology Review Manual

ANATOMYOF BRAIN

EMBRYOLOGY
Neurulation
neuralPlate = CNS originatesas a plate of thickened
ectodermon the dorsalaspectof the
embryo
neuralcrest = elevationof the lateralmarginsof the
neuralplate;{ormsthe peripheral
nervoussystem
neuraltube = invaginationbetweenthe two neural
crests;its wall formsthe brain+ spinal
cord;its lumenformsthe ventricles
+ spinalcanal
4.6 weeksMA: formationof neuraltube
5.6 weeks MA: rostralneuroporecloses
5.9 weeksMA: caudalneuroporecloses
6.0 weeks MA: 3 primarybrainvesiclesdevelop
(prosencephalon, mesencephalon,
rhombencephalon) development of
cervicalflexure
7.0 weeksMA: 2 additionalprimarybrainvesiclesform SagittalSectionthroughBrainat 10-11WeeksGA
out of rhombencephalon (pontine
flexuredividesinto myelencephalon,
metencephalon)
15 weeksMA: dorsalportionof alar platesbulginginto
4th ventriclehavefusedin midlineto CLASSIFICATION OF BRAIN ANATOMY
form cerebellarvermis A. PROSENCEPHALON = forebrain
^/ cerebrum,lateralventricles, choroid,thalami,
cerebellumsonographically visibleat 12 weeksMA
BrainGrowth 1. Telencephalon= cerebrum
= increasein thicknessof brain mantlewith relative = celebralhemispheres, putamen,caudatenucleus
constantventricularwidth 2. Diencephalon
0 Most rapid braingroMh from 12lo 24 weeks MA! = thalamus,hypothalamus, epithalamus(= pineal
gland+ habenula),globusPallidus
NeuronalMigration = mi dbrai n
B . ME S E N C E P H A LON
7th week subependymal neuronalproliferation = shortsegmentof brainstemabove pons;traverses
= germinalmatrix the hiatusin tentoriumcerebelli;containscerebral
8th week radialmigrationto cortexalong radial peduncles,tectum,colliculi(corporaquadrigemina)
glialfibers C. RHOMBENCEPHALON = hindbrain
r/ posteriorcystic space of 4th ventriclesonogra-
phicallydetectable between8 and 10 weeks MA
Myelination 1. Metencephalon= cerebellarhemispheres, vermis
Progression: caudal to cranial;posteriorto anterior 2. Myelencephalon= medullaoblongata,pons
M R: T lW l if < 7 mo n th so f a g e ; D. BRAINSTEM= rT'teseflcephalon + myelencephalon
T2Wl if >7 monthsof age contains
Milestones: (a) cranialnervenuclei
term birth: brainstem,cerebellum,posteriorlimbof (b) sensoryand motortracts betweenthalamus,
internalcapsule cerebralcortex,and sPinalcord
2 months: anteriorlimbof internalcapsule (c) reticularformationcontrollingrespiration,blood
3 months: spleniumof corpuscallosum pressure,gastrointestinal function,centersfor
6 months: genu of corpuscallosum arousaland wakefulness
Occipitalwhite matter:
! centralat 5 months(T1Wl),14 months(T2Wl)
{ peripheralat 7 months(T1Wl),15 months(T2Wl) SCALP
Frontalwhitematter: 0 The outer3 layersare oftentorn off as a unitin
r/ centralat 6 months(Tl Wl), 16 months(T2Wl) accidents;woundsdo not gape if epicraniusnot involved
r/ peripheralat 11 months(T1Wl),18 months(T2Wl) A . S K IN
 Anatomyof Brain 245

calvaria 0.7 LVWFIW=

dura mater = pachymeninx ratioof Tateral
ventricular
0.6 width to hemispheric width
barrier cells l
arachnoid
subarachnoid space I B o.s
pia mater
F) o.+
0.3

0.2
Meninges of Brain 16 20 24 28 32 36 40
Gestational
Age[weeks]
Lateral Ventricular Width to Hemispheric Width
during Gestation
B . S UB CUT I S
= fibro-adiposetissuecloselyadherentto skin and
underlyingepicranius
C. E P I CRA NI U S+ GA L EAA PON EU R OT IC A
= occipitofrontal+ temporoparietalmusclesforming
centrallythe epicranialaponeurosis
D. SUBGALEALSPACE
= subaponeuroticareolartissuebetweenperiosteumof CEREBROSPINAL
FLUID
outertableand galea Totalvolume:
E. PERICRANIUM = periosteumof outertable 50 mL i n new born,150mL i n adul t
F . S UB P E RI O S T EASLPA C E Composition:
= cfedt€dwhen periosteumof outertable becomes inorganicsaltslikethosein plasma,tracesof protein
detachedfrom calvaria(= cephalohematoma) + glucose
Production:
0.3 - 0.4 mUmin resultingin 500 mUday;secretedinto
MENINGES
OF BRAIN ventriclesby choroidplexuses(80-90%), 10-20%
A. CALVARIA= upperpart of craniumenclosingthe brain formedby parenchymaof the cerebrum+ spinalcord
(a) outertable of resilientcompactbone Circulation:
(b) diploe= trabecular bonecontaining red bonemarrow from ventriclesthroughforaminaof Magendie+ Luschka
(c) innertableof thin and brittlecompactbone of 4th ventricleintocisternamagna+ basilarcisterns;
B. E P I DURA LS P AC E 80/" of CSF flows initiallyinto suprasellarcistern
= cfe?tedwhen outer layerof dura (periosteumof + cisternof laminaterminalis, the ambient/ superior
innertable)becomesdetachedfrom calvaria cerebellarcisterns,eventuallyascendingover
C. P A CHY M E N IN GE=SD U R AMA T ER superolateralaspectsof each hemisphere;20o/oinitially
(a) outerdurallayer entersspinalsubarachnoid space+ eventually
= highlyvascularized periosteumof innertable recirculatesinto cerebralsubarachnoidspace
(b) spacefor venoussinuses Absorption:
(c) innerdurallayer into venoussystemby
= meningeallayerderivedfrom meninx (a) arachnoidvilliof superiorsagittalsinus(villibehave
D. S UB DURA LS PA C E as one-wayvalveswith an openingpressure
= cleft formedin pathologicstateswithininner layer of between 20-50 mm of CSF)
dur a (b) cranial+ spinalnerveswith eventualabsorptionby
E . LE P T O M E N IN GE S lymphatics(50%)
1. A r ac hnoi d (c) prelymphatic channelsof capillaries withinbrain
= closelyappliedto innersurfaceof dura parenchyma
2. Subarachnoidspace (d) vertebralvenousplexuses,intervertebral veins,
Histo: fine connectivetissue+ cellularsepta link posteriorintercostal+ upper lumbarveins into
pia and arachnoid azygos+ hemiazygosveins
containsCSF that drainsthroughthe valvesof
arachnoidgranulations intovenoussinuses
forms basal cisterns GerebralAqueduct
3. Pia mater pulsatileflow (dueto brainmotionduringcardiaccycle)
F. S UB P I A LS P A C E + net outflowinto 4th ventricle;diameterof 2.64.2 mm;
= perivascular(Virchow-Robin) space peak outflowvelocityof 6-51 mm/sec;inflowvelocityof
G . E P E NDY M A 3-28 mm/sec
246 RadiologyReviewManua[

septum pellucidum
genu of corpus callosum
frontal horn

internal capsule

external capsule

extreme capsule claustrum

thalamus 3rd ventricle

pulvinar tail of caudate nucleus

pineal gland temporal hom

vein of Galen

Axial Section through Level of Third Ventricle

anterior cornu of head of bodv of fornix caudatenucleus

lateral ventricle caudatenucleus
3rd thalamus
anterior limb of ventricle
column internal
of fornix capsule

.i.ir,,, putamen clausffum

,il' insula ...-..--
,.j;,:
:=-=--
pummen
globus
pallidus inferior globus
horn pallidus

claustrum crus tail of

cerebri caudate
amygdaloid nucleus
anterior body
commlssure hippocampus nucleus subthalamicus
substantianigra red nucleus
optic tract infundibular recess anterior perforated
of 3rd ventricle substance
interpeduncular fossa

Coronal Sectionthrough Anterior Commisssure Coronal Section through Ventral Part of Pons

BASALNUCLEI S i ze:
= BASALGANGLIA (earlierincorrectdesignation) adult size is achievedat puberty
A. Amygdaloidbody Heightin adultfemales= 7 (range4-10) mm
B. Claustrum Heightin adultmales = 5 (range3- 7) mm
C. Corpusstriatum
(1) Caudate Shape:
(2) Lentiformnucleus ./ ttat / downwardlyconvexsuperiorborder
(a) pallidu6= globuspallidus r/ upwardlyconvexduringpuberty,pregnancy,in
(b) putamen hypothyroidism (due to hyperplasia)

PITUITARY
GLAND
= HYPOPHYSISCEREBRIwithinhypophyseal fossaof A. ANTERIORLOBE
= largeranteriorportionof adenohypophysis
sphenoid,coveredsuperiorlyby sellardiaphragm
(= dura mater)which has an aperturefor the comprising80% of pituitarygland volume
infundibulumcentrally Origin: ectodermalderivativeof stomadeurn
 Anatomyof Brain 247

centrally peripherally MRI:

r/ hyperintense on TlWl + isointenseon T2Wl in
abducens(VI) comparisonwith anteriorlobe(? due to relaxing
occulomotor (III)
agentof phospholipid granules/
/ neurosecretory
trochlear (IV) vasopressin)
r/ isointensein 10/" of normalindividuals
ophthalmic(v1)

maxillary(V2)

CavernousSinus
(coronalview)

Function:
(a) chromophilcells
1. ac id o p h icl e l l s= u c e l l s
growthhormone= soffi?totropin(STH),
\::
prolactin= lactogenichormone(LTH) $

2. basophilcells= B cells
adrenocorticotropi n = adrenocorticotropic
hormone(ACTH),thyrotropin= thyroid-
stimulatinghormone(TSH),follicle-stim ulating
hormone(FSH),interstitial-cel l-stimulating
hormone(ICSH),luteinizing hormone(LH),
melanocyte-stimulating hormone(MSH)
(b) chromophobe I
cells= 50% of epithelialcell 't
population, of unknownsignificance ,1,
MRI: a;l
::r.
r:irrI
r/ largerhomogeneous componentisointense to
whitematteron TlWl + T2Wl
r/ prominentcontrastenhancement(duringfirst
, 3 minutes)due to lackof blood-brain barrier
! hyperintensein the newbornfadingto normaladult
signalby 2nd monthof life

B . P A RSI NT E R M ED IA
= posteriorportionof adenohypophysis; separated
from anteriorlobe by hypophysealcleft in fetal life
Origin: Rathkecleft/ pouchwithinintermediatelobe
of pituitarygland
Function: terminationpoint of short hypothalamic
axons elaboratingtropic hormones
(= releasingfactors+ prolactininhibiting
factor),which are carriedto anteriorlobe
via the portalsystem t.-
r/ not visiblewith imagingtechniques
Cranial Nuclei of Brainstem and Reticular Formation
c. POSTERIORLOBE A = sleep,wakefulness,consciousness
= majorportionof neurohypophysis B = visual spatial orientation, higher autonomic
Origin: diencephalicoutgrowth(terminationpoint of coordination of food intake
axons from supraoptic+ paraventricular C = pneumotaxic center, coordination of breathing and
nucleiof hypothalamus) circulation
Function: storagesite for vasopressin(= spll6iuretic D = swallowing
hormoneIADHI)+ oxytocintransported E = blood pressure,cardiac activity, vascular tone
from paraventricular
+ supraopticnucleiof F = expiration
hypothalamusalong neurosecretory G = areapostrema = trigger zone for vomiting
hypothalamohypophyseal tract H = inspiration
248 Radiology Review Manual

mes'encephalic gasserian ganglion

nilcleu$
ophthalmic n.

rnain,sensory
hilbleds

ibular n.
infraorbital n.

spinal nucleus

inferior alveolar n.
r \ 1 \
t\ \'. 1\
mylohyoid n.

Trigeminal Nerve

D . P I T UI T A RYS T AL K= IN F U N D IB U L U M Location: attachedto upperaspectof posteriorborder

arisesfrom anterioraspectof floor of 3rd ventricle of 3rd ventricle,lieswithinCSF of
(infundibular recess) quadrigeminal cistern,anteriorto pinealgland
Histo: formedfrom axonsof cells lyingin supraoptic is cisternof velum interpositum(= cisternof
+ paraventricular nucleiof hypothalamus transversefissure)
ri loins posteriorlobe at junctionof anterior+ posterior S i z e : 8 m m l o n g , 4m m w i d e
lobes
r/ up to 3 mm thicksuperiorly,up to 2 mm thick
inferiorly
{ usualtyin midline,may be slightlytiltedto one side
MRI: NERVE(V)
TRTGEMINAL
ri prominentcontrastenhancement Nuclei:
(1) mesencephalic nucleus:proprioception extendsto
PINE A L G LA ND levelof inferiorcolliculus
Development: (2) mainsensorynucleus:tactilesensation
from area of ependymalthickeningat the mostcaudal (3) motornucleus:motorinnervation
portionof roof of 3rd ventriclethat evaginatesinto a (4) spinalnucleus:pain+ temperaturesensation
pinecone-shaped massduring7th week of gestation; extendsto level ol2nd cervicalvertebra
initiallycontainsependymaliningin centralcavitythat Location: in tegmentumof lateralpons,along
connectswith 3rd ventricle anterolateralaspectof 4th ventricle
Function: Course:
1. Regulationof long-termbiologicrhythm(eg,onsetof - throughprepontinecistern
puberty) - exitsthroughporustrigeminus(= openingin dura)
2. Regulationof short-termbiologicrhythm(eg,diurnal - entersMeckelcave with dura mater+ leptomeninges
/ circadian)due to photoperiodiccluesvia accessory formingtrigeminalcistern(= CSF-filledsubarachnoid
optic pathway space)at the most anteromedialportionof the
Histo: petrouspyramid
(a) pinealocyteswith dendriticprocesses(= neuronal - formsgasserianganglion(= trigeminalganglion),
cells)make up 95% of population which containscell bodiesof sensoryfibersexcept
(b) neuroglialsupportingcellsmake up 5%.of population those for propriocePtion
 Anatomyof Brain 249

Trifurcationinto3 principalbranches:
(1) ophthalmic nerve (V,) intracanalicular
Course: in lateralwall of cavernoussinus
labvrinthine cistemal
Exit: superiororbitalfissure
Supply: sensoryinnervation of scalp,forehead,
nose,globe
. mediatesafferentaspectof cornealreflex
(2) maxillary nerve (V2)
Course: betweenlateralduralwall of cavernous
s i n u s+ s k u l lb a s e
Exit: throughforamenrotunduminto
pterygopalatine fossa
Supply: sensoryinnervation of middlethirdof face,
upperteeth
Maintrunk: infraorbital nerve SegmentalAnatomy of Facial Nerve Intracranially
(3) mandibularnerve (Vr) (viewed from anteriorly)
Course: NOT throughcavernoussinus
Exit: throughforamenovaleinto masticator
space
Supply: (a) sensoryinnervation of lowerthirdof
face,tongue,floorof mouth,jaw
(b) motorinnervationof musclesof Nuclei:
mastication(masseter,temporalis, (1) Motornucleus:ventrolateral deep in reticular
medial+ lateralpterygoid),mylohyoid formationof the caudalpart of the pons
m., anteriorbellyof digastricm., tensor Intrapontine course:
tympanim., tensorveli palatinim. - dorsomediallytowards4th ventricle
- curvinganterolaterally aroundupperpoleof
FACTALNERVE(Vil) abducensnucleus(= geniculum)
Function: - descendinganterolaterally throughreticular
1. Lacrimation (viagreatersuperficial petrosalnerve) formation
2. Stapediusreflex: sounddamping Innervation to: stapediusm., stylohyoidm.,
3. Taste of anterior213of tongue (via chordatympani posteriorbellyof digastricm.,
nerveto lingualnerve) occipitalism., buccinator,muscles
4. Facialexpression(platysma) of facialexpression,platysma
5. Secretionof lacrimal+ submandibular + sublingual (2) Nucleussolitarius(sensorynucleus):
glands(vianervusintermedius) - nervus intermediust sensationfrom anterior
213of tongue,skin on + adjacentto ear
(3) Superiorsalivatorynucleus(parasympathetic
common vestibularn. secretomotorinnervation)
- greaterpetrosaln.: secretionof lacrimalglands,

r^'(€3^
cochlear\ @l
branchl V
nasalcavity,paranasalsinuses
- chordatympani:submandibular
sublingualglands
gland,

l:i"', i ,,' Course & Segments:

(a) intracranialsegment
from brainstemto porusacusticusinternus:
inferior - pontinesegment:motorrootfibersof facialn.
vestibular
branch hookaroundthe abducensnucleusformingthe
facialcolliculus(= elevationin the floorof the
4th ventricle);the nervecontinueslaterallyfrom
common the corticospinaltract
vestibularn. - cisternalsegment:facialn. emergesfrom lateral
aspectof pontomedullary junction+ courses
vestibular
anterolaterally in cerebellopontine anglecistern
ganglion
of Scarpa vestibulocochlearn.
to internalauditorycanal(lAC)
(b) intracanalicular segment
= rTlotorroot of facialn. within internalauditorycanal
Internal Auditory Canal in anterosuperior grooveof vestibulocochlear n.
Posterior wall of IAC is removed; cross sections through IAC with nervusintermedius betweenthem
are displayed above; A = anterior, P = posterior
mnemonic.' "seven up"
250 Radiology Review Manual

caudatenucleus choroid plexus choroidal fissure

transverse
fissureof
temporal Bichat
horn
perimesencephalic
cistern
dentate
gyrus hippocampal
fissure

Irr,rEpIALI
parahippocampal
collateral sulcus gyrus

Coronal Section through the Right Mesial Temporal Lobe

(CA1 through CA4 = hippocampus)

(c) labyrinthine segment (3) Chorda tympani (sensory+ parasympathetic fibers)

short segmentof facial n. travelswithinits own bony
canal(= fallopian canal) curvinganteriorlyovertop
of cochlea;terminatesin anteromedialgenu
(geniculateganglion)
(d) tympanicsegment
from anteriorto posteriorgenu just
= segffi€r'rt
underneathlateralsemicircular canal
- horizontalsegment:facialn. makesa 130'turn
posteriorlyand horizontallyalongmedialwall of
mesotympanumlateralto vestibulebetween
lateralsemicircularcanal (above)and oval
window (below)
- pyramidalsegment:facialn. turnsinferiorlyat
second genu in pyramidaleminence;givesoff
the nervefor the stapediusmuscle
(e) mastoidsegment
facialn. descendsfrom posteriorgenuthrough
anteriormastoid(= medialwall of aditusad antrum)
and givesoff chordatympanijustpriorto exitfrom
skull base throughstylomastoidforamen
(f) parotid/ extracranialsegment
facialn. travelsforwardbetweensuperficial+ deep
lobesof parotidgland lateralto styloidprocess
+ externalcarotida. + retromandibular v.
Branches:
(1) Greater superticial petrosal nerve
(parasympathetic + motorfibers)arisesfrom
geniculateganglion,runs anteromedially, and exits
at the facialhiatuson the anteriorsurfaceof the
temporalbone + passesunder Meckelcave near
foramenlacerum
- forms vidian nerve after receivingsympathetic
fibersfrom deep petrosalnerve,which surrounds
the internalcarotidartery
(2) Stapedial nerve (motorfibers)arisesfrom proximal
descendingfacialn.
leavesfacial n. about 6 mm above stylomastoid
foramen
- ascendsfonruard in a bony canal (= posterior
canaliculus)
- perforatesposteriorwall of tympaniccavity
- crossesmedialto handleof the malleolus
underneathmucosaof tympaniccavity
- reentersbone at medialend of petrotympanic
fissure(= posteriorcanaliculus)
- joinsthe lingualnerve(= branchof V.) containing
sensoryfibersfrom anterior213of tongue
+ secretomotorfibersfor submandibularand
sublingualglands
PERIHIPPOCAMPAL FISSURES
1. Transversefissureof Bichat
= lateralextensionof perimesencephalic cistern
separatingthalamussuperiorlyfrom
parahippocampal gyrusinferiorly
2. Choroidalfissure
= superiorlateralextensionof transversefissure
extendingsuperiorto hippocampus
3. Hippocampal fissure
= inferiorlateralextensionof transversefissure
extendingbetweenhippocampusand
parahippocampal gyrus
4. Temporalhorn of lateralventricle
= lateralmarginof hippocampus;separatedfrom
transversefissureby fimbria+ choroidplexus
0 Does not communicatewith transversefissure
C E R E B R A L V E S S E LS
CommonCarotidArtery
. 70/" of bloodflow is deliveredto ICA
 Anatomyof Brain 251

anterior ophthalmic a.
hypophyseal a.
communlcatlng a.
A2

middle cerebral a. anterior cerebral a.

internal carotid a.
posterior
communicatins a.
anterior choroidal a.
posterior cerebral a.

basilar a. superior cerebellar a.

internal auditorv a.
anterior inferior
cerebellar a.

vertebral aa.

Circle of Willis
r/ shareswaveformcharacteristics of both internal . hypersensitive carotidsinus
+ externalcarotidarteries = slighttouch/ head movementinitiates
r/ velocityincreasestowardthe aorta (9 cm/secfor each (a) vasodilatationwith drop in blood pressure
cm of distancefrom the carotidbifurcation) (b) vagal stimulationwith sinoatrialI
atrioventricular
cardiacblock
Carotid Bifurcation r/ stagnanteddy that rotatesat outervesselmargin
= physiologicstenosisdue to inertialforcesof blood
flow divertingmain-flowstreamfrom midvesselto a Gervical Segment
path alongvesselmarginat flow divider ascendsposteriorand medialto ECA; enterscarotid
Location: lateralto upperborderof thyroidcartilage; canalof petrousbone;NO branches
at levelof C3-4 intervertebral
disk
Branches: ECA arisesanterior+ medialto ICA (95%) B . P E TR OU SS E GME N T
ascendsbriefly,in carotidcanal bends
ExternalGarotidArtery Branches anteromedially in a horizontalcourse(anteriorto
mnemonic.' "All Summer Long EmilyOgled Peter's tympaniccavity+ cochlea);exits near petrousapex
Sportylsuzu" throughposteriorportionof foramenlacerum;
Ascendingpharyngealartery ascendsto juxtasellarlocationwhere it piercesdural
Superiorthyroidartery layerof cavernoussinus
Lingualartery Branches:
Externalmaxillary = facialartery 1. Caroticotympanic a.: to tympaniccavity,
Occipitalartery anastomoseswith anteriortympanicbranchof
Posteriorauricularartery maxillarya. + stylomastoida.
Superficialtemporalartery 2. Pterygoid(vidian)a.: throughpterygoid
Internalmaxillaryartery canal;anastomoseswith recurrentbranchof
greaterpalatinea.
InternalCarotidArtery
A . CE RV I CA LS EGME N T C . C A V E R N OU SS E GME N T
ascendsposteriorand medialto ECA; enterscarotid ascendsto posteriorclinoidprocess,then turns
canalof petrousbone;NO branches anteriorly+ superomedially throughcavernoussinus;
Carotid bulb = carotidsinus: exitsmedialto anteriorclinoidprocesspiercingdura
= dilatedproximalpartof ICAwith thinnermedia Branches:
+ thickeradventitiacontainingmany receptor 1. Meningohypophysealtrunk
endingsof glossopharyngeal nerve (a) tentorialbranch
Function: baroreceptorresponsiveto changesin (b) dorsalmeningealbranch
arterialblood pressure (c) inferiorhypophysealbranch
252 RadiologyReviewManual

2. Anterior meningeala.: suppliesdura of AnteriorCerebralArtery (ACA)

anteriorfossa;anastomoseswith meningeal A. HORIZONTALPORTION= A 1 SEGMENT
branchof posteriorethmoidala. = SeOITI€I]I betweenoriginand anterior
3. Cavernousramisupplytrigeminalganglion, communicating a. (aCom)
wallsof cavernous+ inferiorpetrosalsinuses (a) Inferiorbranches
supplysuperiorsurfaceof optic nerve+ chiasm
D. S UP RA CL IN O IDSE G ME N T (b) superiorbranches
ascendsposterior+ lateralbetweenoculomotor penetratebrainto supplyanteriorhypothalamus,
+ optic nerve septumpellucidum, anteriorcommissure, fornix
Branches: columns,anteriorinferiorportionof corpus
mnemonic.' "OPA" striatum(largeststriatalartery= medial
Ophthalmica. lenticulostriate artery= Iecurfefltartery of
Posteriorcommunicating a. Heubner for anteroinferiorportionof head of
Anteriorchoroidala. caudate,putamen,anteriorlimbof internal
1. Ophthalmica. exitsfrom ICA medialto capsule)
anteriorclinoidprocess,travels throughoptic B . IN TE R H E MIS P H E RP ICOR TION= A 2 S EG M ENT
canal inferolateraltooptic nerve = SegrTl€I'tt
after originof anteriorcommunicatinga.
(a) recurrentmeningealbranch: dura of (aOom);ascendsin cisternof laminaterminalis
anteriormiddlecranialfossa Branches:
(b) posteriorethmoidala.: suppliesdura of 1. Medialorbitofrontala.: alonggyrusrectus
planumsphenoidale 2. Frontopolar a.
(c) anteriorethmoidala. 3. Gallosomarginala.: withincingulusgyrus
2. Superior hypophyseala.: opticchiasm, 4. Pericallosala.: over corpuscallosumwithin
anteriorlobe of pituitary callosalcistern
3. Posteriorcommunicatinga. (pOom) (a) Superiorinternalparietala.: anterior
4. Anterior choroidal a. portionof precuneus+ convexityof superior
5. Middle + anterior cerebral arteries (MCA, parietallobule
ACA) (b) Inferiorinternalparietala.
(c) Posteriorpericallosal a.
CarotidSiphon
flow direction: C4-C1 Ii ;,1 ::fl,:'L?'
i ?:'J3li
li"""lfi135:'
(a) C4 segment = beforeoriginof ophthalmica. frontalaa.
(b) C3 seglrellt = genu of ICA Paracentrala.: suppliesprecentral
(c) C2 segment= supraclinoid segmentafteroriginof + postcentralgyri
ophthalmica.
(d) C1 segment= terminalsegmentof ICA between Supply: anterior213of medialcerebralsurface
pOom+ ACA + 1 cm of superomedialbrain over convexity

anterior falx a.

supratrochlear a. anterior ethmoidal a.

posterior choroidal a.
anterior choroidal a.

dorsal nasal a.
optic canal
choroidal crescent
ophthalmic a.
central retinal a.

lacrimal a. muscular branches

Ophthalmic Artery
 Anatomyof Brain 253

MiddleCerebralArtery 4. Posteriormedialchoroidala.: circlesmidbrain

= largestbranchof ICA arisinglateralto opticchiasm; parallelto PCA;enterslateralaspectof
passeshorizontalin lateraldirectionjust ventralto quadrigeminal cistern;
anteriorperforatedsubstanceto enter sylvianfissure passeslaterallyand above pinealgland and
whereit dividesinto2 I 3 I 4branches entersroof of 3rd ventricle;supplies
Branches: 1. Anterior temporal a. quadrigeminal plate+ pinealgland
2. Ascending frontal a. (candelabra)/ 5. Posteriorlateralchoroidala.: courseslaterally
prefrontala. and enterschoroidalfissure;anteriorbranchto
3. Precentrala. = pre-Rolandica. temporalhorn + posteriorbranchto choroidplexus
4. Centrala. = Rolandica. of trigoneand lateralventricle+ lateralgeniculate
5. Anterior parietal s. = post-Rolandica. bodY
6. Posterior parietal a. 6. Corticalbranches:(a) Anteriorinferiortemporala.
7. Angular a. (b) Posteriorinferiortemporala.
8. Middle temporal a. (c) Parieto-occipitala.
9. Posterior temporal a. (d) Calcarinea.
10. Temporo-occipital a. (e) Posteriorpericallosal
a.
Supply: lateralcerebrum,insula,anterior+ lateral Supply: medial+ posteriortemporallobe, medial
temporallobe parietallobe,occipitallobe

PosteriorGerebralArtery ArterialAnastomosesof the Brain

originatesfrom bifurcationof basilararterywithininter- ANASTOMOSES VIA THE ARTERIESAT THE BASE
peduncularcistern(in 15% as a directcontinuation of OF TH E B R A IN
posteriorcommunicatingartery);lies above oculomotor A. Circleof Willis
nerveand circlesmidbrainabovethe tentoriumcerebelli 1. Right ICA <+ rightACA <+ aOom<-+left ACA
Branches: <-+left ICA
1. Mesencephalic perforating branches:tectum 2. ICA e pOom<-+basilara.
+ cerebralpeduncles 3. ICA <+ anteriorchoroidala. <+ posterior
2. Posteriorthalamoperforating aa.: midlineof choroidala. <+ PCA <+ basilara.
thalamus+ hypothalamus B. Developmental anomaly
3. Thalamogeniculate aa.: geniculatebodies threetransientembryonalcarotid-basilar
+ pulvinar anastomosesappearingconsecutivelyin fetal life:

Frontal Supraorbital a.

Zygomatico-orbital Superior palpebral t. -

- Superficial temporal a.
Transverse facial Inferior palpebral a.

Middle temporal

- Dorsal nasal a. Opthalmic a.

-,-Angular
Extemal maxillary a. --/
\Lateral nasal

Infraorbital

ECA Orbital (lacrimal branch) \ Lacrimal a.

'of
middle meninseal a.
-Internal maxillary a. Sphenopalatine Ethmoidal
ICA
Artery of pterygoid canal Artery to pterygoid canal

' Cavemous branch of

\ middle meningeal a. Cavemous a.
\' --
Anterior tympanic -..-..-.--.-..--..-.-..-..-...--
caroticotympanic a'
- posterior auricular a. -stvromastoro

Occipital a. Muscular branches Vertebral a.

Anastomoses between ICA and ECA and Vertebral Artery

254 Radiology Review Manual

1. Primitive hypoglossal aflery 3. Venousangle= ?cuteangleat junctionof

= arterialconnectionbetweenthe intrapetrosal
portionof ICA and proximalportionof basilar
arterY
2. Primitive acoustic (otic) artery
= arterialconnectionbetweencervicalportion
of ICA + vertebralarteryin regionof 12th
nerve
3. Persistent primitive trigeminal artery
lncidence: 1-2 / 1,000angiograms
r/ shortwide connectionbetweenthe
cavernousportionof ICA and upperthird of
basilarartery(beneathposterior
communicating artery)
r/ enlargementof ipsilateralICA
{ ectopicvesselcrossingthe pontinecisternto
anastomosewith basilarartery
thalamostriatewith internalcerebralv. = posterior
aspectof foramenof Monro
4. Internalcerebralw. = demarcatecaudadborderof
spleniumof corpuscallosumsuperiorly+ pineal
glandinferiorly
5. Copularpoint= junctionof inferior+ superior
tributariesdrainingcerebellartonsilsin
retrotonsillar
regionof copularpyramidsof vermis
CEREBELLAR VESSELS
VertebralArtery
originatesfrom subclaviana. proximalto thyrocervical
trunk; left vertebrala. usuallygreaterthan rightcerebral
a.; left vertebrala. may originatedirectlyfrom aorta (5%)

ANASTOMOSES VIA SURFACEVESSELS A . P R E V E R TE B R ASLE GME N T

A. Leptomeningeal anastomosesof the cerebrum: ascendsposterosuperiorly betweenlonguscolli
ACA ++ MCA e PCA + anteriorscalenemuscle; enterstransverse
B. Leptomeningeal anastomosesof the cerebellum: foraminaat C6
. Superiorcerebellara. <->AICA <+ PICA Branches: muscularbranches
RE T EM I RA B IL E B . C E R V IC A LS E GME N T
ECA <+ middlemeningeala. / superficial temporala. ascendsthroughtransverseforaminain close
<+ leptomeningeal aa. <+ ACA / MCA proximityto uncinateprocesses
Branches: 1. Anterior meningeal a.
CerebralVeins C. ATLANTICSEGMENT
Important markers
vascular : exitstransverseforamenof atlas;passesposteriorly
1. Pontomesencephalic v. = anteriorborderof in a grooveon superiorsurfaceof posteriorarch of
brainstem atlas;piercesatlanto-occipital membrane+ dura
2. PrecentralcerebellarV. = positionof tectum materto enter cranialcavity
point= midpointof Twining'sline
0 Colliculocentral Branches: 1. Posterior meningeal branch to
at knee of precentralcerebellarvein posteriorfalx + tentorium

septal vv. vein of Galen

superior vermian

basalvein of
Rosenthal

- _ _ - ^ : r - ^ - - -

colliculo
central
point
inferior vermian

lateral mesencephalic v. copular point

Cerebral Veins
 Anatomyof Brain 255

D. I NT RA CR A N IASE
L G ME N T Supply:
ascendsanteriorly+ laterallyaroundmedullato partof pons,middlecerebellarpeduncle,
lateroinferior
reachmidlineat pontomedullary junction; floccularregion,anteriorpetrosalsurfaceof cerebellar
anastomoseswith contralateralside to form basilar hemisphere
arteryat clivus
Branches: 0 Quitevariablecourse+ vascularsupplywith
1. Anterior + posterior spinal a. reciprocalrelationbetweenvascularterritoriesof
2. Posteriorinferior cerebellara. (plCA) A IC A+ P IC A ,
3. Anterior inferior cerebellar a. (AICA)
4. Internal auditory a.
5. Superior cerebellara.
6. Posterior cerebral a. (PCA) PosteriorlnferiorCerebellarArtery
7. Medullary+ pontineperforatingbranches = PICA = last and largestbranchof vertebralartery
0 May terminatein commonAICA-PICAtrunk Supply:
inferoposterior
surfaceof cerebellarhemisphere
AnteriorInferiorCerebellar
Artery adjacentto occipitalbone, ipsilateralpart of inferior
= AICA = first branchof basilarartery vermis,inferiorportionof deepwhitematteronly
superior postenor
cerebellar artery cerebral arterv
basilar artery
posteflor
penetratmg
cerebral artery
arteries superior
cerebellar artery
anterior inferior
cerebellar artery

basilar arterv
I posterior inferior
1 cerebellar artery
1
a (:,
a
- posterior inferior
cerebellar arterv vertebral artery
vertebral artery

Blood Supply of the Cerebellum

supratonsillar
t
: segment basilar artery
bifurcation
posterior retrotonsillar.'
medullary segment j
segment

inferior
anteri,or"... vermian
medullary branch
segment
tuberculum
lateral medullarv tonsillo-hemispheric
segment caudal loop branch anterior margin of
foramen magnum

Posterior fnferior Cerebellar Artery

I, 2 = lines to establish orthotopic choroid point (see text)
256 Radiology Review Manual

Parts: Orthotopicchoroid point establishedby:

1. Premedullar segment= c?udalloop around
medulla,may descendbelowlevelof foramen
m agnum
2. Retromedullar segment= ascendingportionup to
the levelof 4th ventricleand tonsils
3. Supratonsillar segment= the mostcranialpointis
the choroidal point
Pl segment = horizontalsegmentbetweenoriginof
PICA+ pOom
P2 segment = segmentdownstreamfrom pCom
SuperiorCerebellar
take-off
Variations: commonlyasymmetric;hypoplastic/ absent
in 20o/"[vascularsupplythen providedby
anteriorinferiorcerebellarartery(AICA)l
1. perpendicular linefrom choroidpointonto
Twining'sline= TTT-line(Twining'sTuberculum-
Torcularline)bisectsTTT-line(lengthof anterior
portion 52-60%)
2. perpendicular linefrom choroidpointcuts CT-line
(Clivus-Torcular line)<1 mm anterior/ <3 mm
posteriorto junctionof anteriorand middlethirds
of CT-line
Artery
= SCA = last but one branchof basilarartery
Supply:
superioraspectof cerebellarhemisphere(tentorial
superiorvermis,largestpartof
surface),ipsilateral
deepwhitematterincludingdentatenucleus,pons

Tl *H**
N g;""""*u- E sfil""iif;l:i EAwHii**;:;' lm 3if*f,Y:,1iff**

Vascular Territories of Cerebellum

tpoRSAtl
postenor posterior choroidal anterior spinal artery +
communicating paramedian
artery vertebral paramedian
artery inrcrpeduncular branches of anterior inferior branches
basilar artery cerebellar artery
branches circumferential
branches of
basilar artery

long
circumferential.
branches of posterior inferior
basilar artery cerebellar arterv
Mesencephalon Pons Medulla oblongata

ItENTRAI

Vascular Territories of Brainstem

 BrainDisorders 257

BRAINDISORDERS

ABSCESSOF BRAIN MR: (mostsensitivemodality)

PyogenicAbscess { centrallyincreased/ variableintensitywith
= focal area of necrosisbeginningin area of cerebritis hypointense rim on T2Wl
with formationof surroundingmembrane r/ outsideborderof increasedsignalintensityon
Cause: T2Wl (edema)
1. Extensionfrom paranasalsinusinfection(41%)I Cx: (1) Developmentof daughterabscessestoward
mastoiditis / otitismedia(5%) lfacialsoft-tissue white matter
infection/ dentalabscess (2) Ruptureintoventricularsystem/
2. Generalizedsepticemia(82%): subarachnoid space (thinnerabscess
(a) lung (mostcommon):bronchiectasis, capsuleformationon medialwall of abscess
empyema,lung abscess,bronchopleural relatedto fewer bloodvessels)producing
fistula,pneumonia + meningitis
ventriculitis
(b) heart(lesscommon): CHD with R-L shunt(in Dx helpfulfeatures:
children>60%),AVM, bacterialendocarditis multiplelesionsat gray-whitematterborder
(c) osteomyelitis clinicalhistoryof alteredimmunestatus
3. Penetrating traumaor surgery Rto-L shunt:eg, pulmonaryAV fistula
4. Cryptogenic(25%) foreigntravel
high-riskbehavior:eg, lV drug abuse
Predisposed: diabetesmellitus,patientson steroids/ DDx: primary/ metastaticneoplasm,subacute
immunosuppressive drugs,congenital/ infarction,resolvinghematoma
acquiredimmunologic deficiency
Organism; anaerobicstreptococcus(mostcommon), GranulomatousAbscess
bacteroides,staphylococcus; in 20./. 1. Tubercul oma
multipleorganisms;in 2|oh sterilecontents 2. Sarcoidabscess
Pathophysiology: 3. Fungalabscess:coccidioidomycosis, mucormycosis
Stage l: vascularcongestion,petechialhemorrhage, (in diabetics),aspergillosis,
cryptococcus
edema Predisposed: immunocompromised host
Stagell: cerebralsoftening+ necrosis r/ enhancement of leptomeningealsurface
"''
stase r/ nodular/ ring-enhancing parenchymal lesion
l"::;;,,:ffT:ll3X?il'i31,;i':1''o' Cx: Communicating hydrocephalus (secondaryto
granulation tissue,a middlecollagenous thick exudateblockingbasal cisterns)
layerand an outerastrogliallayer;edema
outsideabscesscapsule
. headache,drowsiness,confusion,seizure ACRANIA
. focal neurologicdeficit = EXENCEPHALY
. fever, leukocytosis(resolveswith encapsulation) = developmental anomalycharacterized by partial/
Location: typicallyat corticomedullary junction;frontal completeabsenceof membranousneurocranium +
+ temporallobes;supratentorial : completebut abnormaldevelopment of braintissue
infratentorial= 2:1 lncidence: 25 cases reported
Cause: impairedmigrationof mesenchymeto its normal
NCCT: locationunderthe calvarialectodermresultingin
./ zone of low densitywith mass effect(92%) failurefor development of dura mater+ skull
r/ slightlyincreasedrim density(4%),development of + musculature
collagenlayertakes 10-14 days Time: developsafter closureof anteriorneuroporeduring
r/ gas withinlesion(4%) is diagnosticof gas-forming 4th week
organism May be associated with:
CE CT : cleft lip, bilateralabsenceof orbitalfloors,metatarsus
r/ ringenhancement(90%)with peripheralzone of varus,talipes,cervicothoracic spina bifida
edema . + elevationof maternalserumAFP
r/ homogeneous enhancementin lesions<0.5cm r/ absenceof calvarium
r/ edema + contrastenhancementsuppressedby r/ normalossification of chondrocranium (face,skullbase)
steroids r/ hemispheres surroundedby thin membrane
r/ smoothregular1-3-mmthickwall with relative Prognosis; uniformlylethal;progressionto anencephaly
thinningof medialwall (secondary to poorerblood (braindestructionsecondaryto exposureto
supplyof white matter) amnioticfluid+ mechanicaltrauma)
r/ muftiloculation
+ subjacentdaughterabscessin DDx: encephalocele, anencephaly, osteogenesis
white matter imperfecta,hypophosphatasia
258 Radiology Review Manual
ADRENOLEUKODYSTROPHY
AGENESIS
= BRONZED SCLEROSING ENCEPHALOMYELITIS
= inheritedmetabolicdisordercharacterizedby
progressivedemyelinationof cerebralwhite matter
+ adrenalinsufficiency
Etiology: defectiveperoxisomalfatty acid oxidationdue
to impairedfunctionof lignoceryl-coenzyme A lncidence: 0.7-5.3/"
ligasewith accumulationof saturatedvery long
chain fatty acids (cholesterolesters)in white
matter + adrenal cortex + testes
Dx: assay of plasma,red cells,culturedskin fibroblasts
for the presenceof increasedamountsof very long
chain fatty acids
Mode of inheritance:
(a) X-linkedrecessivein boys (common)
(b) autosomalrecessivein neonates(uncommon)
Histo: PAS cytoplasmicinclusionsin brain,adrenals,
othertissues
Age: 3-10 years (X-linkedrecessive)
. deteriorating vision(27"/"),lossof hearing(50%)
. ataxia
. opticdisk pallor
. adrenalgland insufficiency(abnormalincreased
pigmentation,elevatedACTH levels)
. alteredbehavior,attentiondisorder,mental
deterioration,death
Location: diseaseprocessusuallystartsin central
occipitalwhite matter,advancesanteriorly
throughinternal+ externalcapsules
+ centrumsemiovale,centripetalprogression
to involvesubcorticalwhite matter,
interhemispheric spreadvia corpuscallosum
particularlysplenium,involvementof optic
radiation+ auditorysystem+ pyramidaltract
CT:
{ largesymmetriclow-density lesionsin
occipitoparietotemporal white matter(80%)advancing
towardfrontallobes+ cerebellum
r/ tnin curvilinear / serratedenhancingrims nearedges
of lesion
r/ initialfrontallobe involvement(12%)
{ calcificationswithinhypodenseareas (7%)
{ cerebralatrophyin late stage (progressiveloss of
corticalneurons)
MR:
r/ hypointensityon TlWl in affectedareas (hypointense
atrophicspleniumof corpuscallosum)
./ hyperintensebilateralconfluentareas on T2Wl
Prognosis; usuallyfatal withinseveralyears after onset
of symptoms
AdrenomyeIoneuropathy
= clinicallymilderformwith laterage of onset
. symptomsof spinalcord demyelination+ peripheral
neuropathy
OF CORPUSCALLOSUM
= COMPLETE DYSGENESIS OF CORPUS CALLOSUM
= failureof formationof corpuscallosumoriginating from
the laminaterminalisal7-13 weeksfrom where a
phalanxof callosaltissueextendsbackwardarching
overthe diencephalon; usuallydevelopedby 20 weeks
Cause: congenital,acquired(infarctionof ACA)
Histo: axonsfrom cerebralhemispheresthat would
normallycrosscontinuealongmedialwallsof
lateralventriclesas longitudinal callosalbundlesof
Probstthat terminaterandomlyin occipital
+ temporallobes
Associated with:
(a) CNS anomalies(85%):
1. Dandy-Walker cyst (11%)
2. lnterhemispheric arachnoidcyst may be
continuouswith 3rd and lateralventricles
3. Hydrocephalus (30%)
4. Midlineintracerebral lipomaof corpuscallosum
oftensurroundedwith ringof calcium(10%)
5. Arnold-Chiari ll malformation (7%)
6. Mi dl i neencephal ocel e
7. Porencephaly
8. Holoprosencephaly
9. Hypertelorism mediancleftsyndrome
10. Polymicrogyria, gray-matterheterotopia
(b) Cardiovascular, gastrointestinal,genitourinary
anomalies(62%)
(c) Abnormalkaryotype(trisomy13, 15, 18)
. normalbrainfunctionin isolatedagenesis
. intellectual impairment; seizures
{ absenceof septumpellucidum+ corpuscallosum
+ cavumseptipellucidi
{ longitudinalbundlesof Probstcreatecrescenticlateral
ventricles:
r/ colpocephaly(= dilatationof trigones+ occipitalhorns
+ posteriortemporalhornsin the absenceof splenium
r/ "bat-wing"appearanceof lateralventricles(= wide
separationof lateralventricleswith straightparallel
parasagittalorientationwith absentcallosalbody)
r/ laterallyconvexfrontalhorns in case of absentgenu
of corpuscallosum
./ "high-riding thirdventricle"= uPWarddisplacement of
widened3rd ventricleoftento levelof bodiesof lateral
ventricle
r/ anteriorinterhemispheric fissureadjoinselevated
3rd ventricle+ communication (PATHOGNOMONIC)
./ "interhemispheric cyst"= interhemispheric CSF
collectionas an upwardextensionof 3rd ventricle
{ enlargedforaminaof Monro
{ "sunburstgyralpattern"= dysgenesisof cingulategyrus
with characteristicradialorientationof cerebralsulci
from the roof of the 3rd ventricle(on sagittalimages)
ri failureof normalconvergenceof calcarine+ parieto-
occipitalsulci
{ persistenteversionof cingulategyrus (rotatedinferiorly
+ laterally)with absenceon midsagittalimages
^/ incompleteformationof Ammon'shorn in the
hippocampus
 BrainDisorders 259

OB-US (>22 weeksGA): (2) PrimaryCNS lymphoma(20-30"/.1

r/ absenceof septumpellucidum Prevalence: in75/" at autopsy
r/ "teardrop"ventriculomegaly = disproportionate 0 Initialmanifestation in 0.6% of AIDS patients
enlargementof occipitalhorns= colpocephaly 0 2% of AIDS patientsdevelopprimaryCNS
! dilated+ elevated3rd ventricle lymphomaat some pointduringtheirillness
r/ radial arraypatternof medial cerebralsulci (3) ProgressivemultifocalIeukoencephalopathy (10-20%)
An gio: (4) Fungal,granulomatous, viral,bacterialinfection
r/ wanderingstraightposteriorcourseof pericallosal (a) Cryptococcosis
, arteries(lateralview) Location: extensionalongVirchow-Robin
r/ wide separationof pericallosalarteriessecondaryto spaces
, intervening3rd ventricfe(anteriorview) r/ hydrocephalus+ cortical/ centralatrophy (with
r/ separationof internalcerebralveins inadequateimmuneresponse)
r/ loss of U-shapein vein of Galen r/ enhancinggranulomatous meningitis(with
sufficientimmuneresponse)
DDx: (1) Prominentcavumseptipellucidi+ cavumvergae r/ bilateralnonenhancing hyperintense
(shouldnot be mistakenfor 3rd ventricle) abnormalities in lenticulostriate
region
(2) Arachnoidcyst in midline(suprasellar,
collicular (= gelatinouspseudocyst) on T2Wl
plate)raisingand deformingthe 3rd ventricle (b) Otheropportunistic CNS infections:tuberculosis,
and causinghydrocephalus neurosyphilis
0 With multipleCNS lesionstoxoplasmicencephalitis is
the more likelydiagnosis!
PartialAgenesisof CorpusCallosum 0 With a singleCNS lesionthe probability of lymphoma
= milderform of callosaldysgenesis(bestseen on MR) is at leastequalto toxoplasmosis!
dependingon time of arrestedgrowth (anteroposterior Rx: azidothymidine (AZT)
development of genu + body + splenium,however,
rostrumforminglast)
(a) genu only ALEXANDER
DISEASE
(b) genu + part of the body = FIBRINOID LEUKODYSTROPHY
(c) genu + entirebody Age: as early as firstfew weeks of life
(d) genu + body + splenium(withoutrostrum) . macrocephaly
. failureto attaindevelopmental milestones
. progressivespasticquadriparesis
AIDS . intellectualfailure
= DNA retrovirusinfectionattackingmonocytes Location: frontalwhite mattergraduallyextending
+ macrophages,which leadsto deficientcell-mediated posteriorlyinto parietalregion+ internal
im m unit y capsule
lncidence: 1"/oof populationin UnitedStatesis HIV- CT:
seropositive;187,000new casesin 1991 { low-densitywhite matterlesion
Histo: formationof microglialnodulesinsteadof r/ contrastenhancementnear tip of frontalhorn
granulomasin 75-80% of autopsiedbrains MR:
. neurologicsymptomsas initialcomplaintin 107o, { prolongedT1 + T2 relaxationtimes
ultimatelyafflictup to 40-60h: headache,memory Prognosis; death in infancyI earlychildhood
loss,confusion,dementia,focaldeficitfrom mass lesion
0 Any male with neurologicsymptomsbetweenage 20
and 50 has AIDS untilprovenotherwise A LZH E IME R D IS E A S E
0 Unusualpresentations are cluesto HIV infection: = diffusegray matterdiseasewith large loss of cellsfrom
pan-sinusitis,mastoiditis,parotidcysts,cervical cerebralcortex+ other areas
adenopathy,hypointensespine 0 Mostcommonof dementingdisordersin elderly!
lncidence: 10/" of people >65 years of age; S}.h of
D IF F US ECHA NG E S: people>85 years of age
(1) HIV / CMV encephalopathy (mostcommon . slowlyprogressing memoryloss,dementia(large
complication) overlapwith other dementiasof elderly)
both virusesoccur alwaysin combination { "crackedwalnut" appearanc€= syffirr'tetricallyenlarged
. dementiain up to 60% duringcourseof disease sulciin high-convexityarea
. cognitivedysfunctionin up to 90% r/ focal atrophicchangein medialtemporallobe
r/ patchywhite matterlesions(= subacute (82% sensitive,75% specific,80% accurate):
leukoencephalitis)
in 31./" { volumeloss of hippocampus+ parahippocampal
gyrus
FOCALCHANGES: r/ of perihippocampal
, enlargement fissures
(1) Toxoplasmosis(50-70%) r/ smoothperiventricular halo of hyperintensity
(50%)
260 Radiology Review Manual

AMYOTROPHIC LATERAL SCLEROSIS (d) traumatic

= rTroStcommonform of motor neurondisease(without (e) neoplastic
autonomic/ sensory/ cognitiveinvolvement) (f) fibromuscular
disease
Cause: free radicaldamageto neurons/ autoimmune (g) collagenvasculardisease
process/ heavy metaltoxicity
Age: middle- late adulthood; M > F Risk factors:
Path: atrophy of precentralgyrus (1) familyhistoryfor aneurysmsin 1st-/ 2nd-degree
Histo: loss of pyramidal+ Betz cells in motorcortex;loss relatives
of anteriorhorncellsin spinalcord;swellingof (2) femalegender
proximalaxonsof neuronalcells (3) age >50 years
. progressiveneurodegenerative disorder (4) cigarettesmoking
. upper neuronalsymptoms: hyperreflexia, spasticity / pregnancy
(5) oral contraceptives
. lower neuronalsymptoms: fasciculation,atrophy (6) Marfansyndrome,pseudoxanthoma elasticum,
MR: Ehlers-Danlos syndrome,neurofibromatosis type 1
r/ hyperintensecorticospinaltracts (coronaradiata, (7) polycystickidneydisease
corpuscallosum,posteriorlimbof internalcapsule, (8) asymmetryof circleof Willis
ventralaspectof brain stem, anterolateralcolumnof (9) cerebralarteriovenousmalformation
spinalcord)on T2Wl Pathogenesis;arterialwall deficientin tunica media +
./ low signalintensityin motorcortexon T2Wl (due to externalelasticlamina(naturaloccurrence
irondeposition) with advancingage)
DDx: Friedreichataxia,vitaminB,, deficiency(abnormal
signallimitedto internalcapsule) Locationof aneurysm:
A. by autopsy:
A NE NCE P HA LY (a) circleof Willis(85%):
= lethalanomalywith failureof closureof the rostralend aCom (25/"), pOom(18/"), MCA bifurcation
of the neuraltube by 5.6 weeks MA (25%),distalACA (5%), ICA at bifurcation(4%),
0 Associatedwith highestAF-AFPand MS-AFPvalues; ophthalmica. (4/"), anteriorchoroidala. @%)
>90/" will be detectedwith MS-AFP>2.5 MoM (b) posteriorfossa (15%)
lncidence; 1:1,000births(3.5:1,000in SouthWales); basilarbifurcation(7%),basilartrunk (3%),
M:F = 1:4;mostcommoncongenitaldefect vertebral-P|CA (3%),PCA (2%)
of CNS; 50% of all neuraltube defects B. by angiography(= sYmPtomatic aneurysms):
Recurrence rate: 3-4% pOom (38%)> aOom (36%)> MCA bifurcation
Etiology: multifactorial (genetic+environmental) (21%) > ICA bifurcation> tip of basilarartery(2.8%)
Path: absenceof cerebralhemispheres+ cranialvault; C . by ri skof bl eedi ng:1-2/"per year
partial/ completeabsenceof diencephalic aCom (70%bleed),pCom (2ndhighestrisk)
+ mesencephalicstructures;hypophysis 0 Aneurysmsat bifurcations/ branchingpointsare
+ rhombencephalic structuresusuallypreserved at greatestrisk for ruPture!
Risk factors: familyhistoryof neuraltube defect;twin
pregnancy MU LTIP LEA N E U R Y S MS
Associated anomalies: Cause: congenitalin 20-307", mycoticin 22/"
spinaldysraphism(17-50%),cleft lip / palate(2/"), mnemonic; "FECALP"
clubfoot(2/"), umbilicalhernia,amnioticband syndrome Fibromuscular dysplasia
r/ absenceof bony calvariumcephaladto orbits Ehlers-Danlos syndrome
r/ + cranialsoft-tissuemass (= angiomatousstroma) Coarctation
r/ oulgingfroglikeeyes Arteriovenousmalformation
r/ short neck Lupus erythematosus
{ polyhydramnios(40-50%) atler26 weeks GA (due to Polycystickidneydisease(adult)
failureof normalfetalswallowing) / oligohydramnios 0 35/o of patientswith one MCA aneurysmhave one on
Dx: in 100%>14 weeksGA the contralateral side (= mirrorimageaneurysms)!
Prognosis.' uniformlyfatal withinhoursto days of life; in 0 Simultaneous aneurysm+ AVM in 4-15h
53% prematurebirth;in 68% stillbirth
DDx: acrania,encephalocele, amnioticbandsyndrome CECT: detectionrate of aneurysmsat pOom (40/"),
aCom / MCA, basilararterY(80%)
OF CNS
ANEURYSM Angio(all4 cerebralvessels):
Etiology: ri contrastoutpouching
(a) congenital(97%)- "berryaneurysm"in 2o/oof ri <2 mm infundibuli typicallyoccurat pCom/ anterior
population(in 20% multiple);associatedwith aortic choroidala. origin
coarctation+ adult polycystickidneydisease { mass effectin thrombosedaneurysm
(b) infectious( 3%) = mYcoticaneurysm 0 2nd arteriogramwithin 1-2 weeks detectsaneurysm
(c) arteriosclerotic:fusiformshape in 10-2C,/"followingnegative1st angiogram!
 BrainDisorders 261

Prognosis: GiantAneurysm
(1) Deathin 10% within24 hoursfrom concomitant = ?heulysmlargerthan 2.5 cm in diameter,usually
intracerebral
hemorrhage, extensivebrainherniation, presentingwith intracranialmass effect
massiveinfarcts+ hemorrhagewithinbrainstem; lncidence: 25/" of all aneurysms
45Tomortalitywithin30 days (25%priorto admission) Age: no age predilection;M:F = 2:1
(2) Completerecoveryin 58%of survivors Location: (arisefrom arteriesat the base of the brain)
(3) Cerebralischemia+ infarction (a) middlefossa: cavernoussegmentof ICA (49%),
(4) Rebleedingrate: 12-20% within2 weeks, t j-22% supraclinoid segmentof lCA, terminalbifurcation
within30 days,up to 50% within6 months of lCA, middlecerebralartery
(increasedmortality);thereafter1-24% risk/year (b) posteriorfossa: at tip of basilarartery,AICA,
Surgicalmortalityrate: 50o/otor ruptured,1-jo/" lor vertebralartery
unrupturedaneurysms S kul lfi l m:
Cx: subduralhematoma ^/ predominantlyperipheralcurvilinearcalcification
(22%)
./ bone erosion(44%)
RupturedBerryAneurysm r/ pressurechangeson sellaturcica(18%)
lncidence; 28,000cases/yeaf= 10 cases/10,000 C E C T:
people/year r/ "targetsign" = centrallyopacifiedvesseltumen
Age: 50-60 years of age; M:F = 1:2 + ringof thrombus+ enhancedfibrousouterwall
Rupturesize: 5-15 mm r/ simplering-blush(75%)of fibrousouter wall with
. "worstheadacheof one's life" total thrombosis
. heck stiffness,nausea,vomiting r/ littte/ no surroundingedema
. suddenlossof consciousness (in up to 4S%) MR:
. historyof warningleak/ sentinelhemorrhagehoursto r/ mixedsignalintensity(combination of subacute
days earlier + chronichemorrhage, calcification)
Cx: subarachnoid hemorrhagein <30%
Clues for which aneurysm is bleeding:
(a) the largestaneurysm(87%)
(b) anteriorcommunicatingartery(70%) MycoticAneurysm
(c) contralateralside of all visualizedaneurysms = 3/" of all intracranial
aneurysms,multiplein20./.
(60%),nonvisualization due to spasm Source: subacutebacterialendocarditis(65%),acute
mnemonic.' "BlSH" bacterialendocarditis (9%),meningitis(g%),
Biggest septicthrombophlebitis (9%),myxoma
lrregularcontour Location: peripheralto first bifurcationof majorvessel
Spasm (adjacent) (6a%);often locatednear surfaceof brain
Hematomalocation especiallyover convexities
(a) suprasellarcistern= circleof Willis
Locationof blood suggestingaccuratelyin 70./.the site (b) inferolateral
sylvianfissure= middle
of the rupturedaneurysm: cerebralarterytrifurcation
(a) accordingto locationof subarachnoid (c) genu of corpuscallosum= originof
hemorrhage: callosomarginalartery
1. Anteriorchiasmaticcistern aCom (d) bottomof 3rd ventricle= pericallosala.
2. Septumpellucidum aCom NCCT:
3. Interhemisphericfissure aOom r/ aneurysmrarelyvisualized;indirectevidencefrom
4. Intraventricular aCom,lCA, MCA focal hematomasecondaryto rupture
5. Sylvianfissure MCA, lCA, pOom r/ zone of increaseddensity/ calcification
6. Anteriorpericallosalcistern ACA, aOom r/ increaseddensityin subarachnoid, intraventricular,
7. Prepontinecistern basilara. intracerebralspaces(extravasatedblood)
8. Foramenmagnum PICA { focal/ diffuselucencyof brain(edema/ infarction/
9. Symmetricdistribution in vasospasm)
subarachnoidspace ACA + basilara. CECT:
(b) accordingto locationof cerebralhematoma: r/ intensehomogeneous enhancement withinround/
1. Inferomedialfrontallobe : aOom oval mass contiguousto vessels
2. Temporallobe : MCA r/ incompleteopacificationwith muralthrombus
3. Corpuscallosum : pericallosal Cx: developrecurrentbleedingmore frequentlythan
artery congenitalaneurysms
(c) intraventricular
hemorrhage
from aneurysmsat aCom, MCA, pericallosalartery
(CAVE: blood may have enteredin retrograde SupraclinoidCarotidAneurysm
mannerfrom subarachnoid location) = 38/o of intracranialaneurysms
262 Radiology Review Manual
Site: (a) at originof pCom (65%)
ARACHNOID
(b) at bifurcationof internalcarotidartery(23%)
(c) at originof ophthalmicartery(12%) medialto
anteriorclinoidprocess; most likelyto
becomegiantaneurysm
Presentation: bitemporalhemianopia(extrinsic
compression on chiasm)
r/ catcificationis rare (frequentin atherosclerotic
cavernoussinusaneurysm)
CavernousSinusAneurysm
Age: 20-70 years,peak 5th-6th decade;F >> M
Cause: sinusthrombophlebitis
. progressive visualimpairment
. cavernoussinussyndrome:trigeminalnervepain,
oculomotornerveparalysis
Site: extraduralportionof cavernoussinus ICA
{ undercuttingof anteriorclinoidprocess
{ erosionof lateralhalf of sella
{ erosionof posteriorclinoidprocess
^/ invasionof middlecranialfossa
./ enlargementof superiororbitalfissure
r/ erosionof tip of petrouspyramid
r/ rimlikecalcification(33%)
./ displacement of thin bony marginswithoutsclerosis
Rx: ofteninoperable; t parent
balloonembolization
aftery occlusion
STENOSIS
AQUEDUCTAL
= focal reductionin size of aqueductat levelof superior
colliculi/ intercollicularsulcus(normalrangeof 0.2-1.8
mm2)
Embryology:
aqueductdevelopsaboutthe 6th week of gestation
+ decreasesin size until birthdue to growthpressure
from adjacentmesencephalicstructures
lncidence: 0.5-1 :1,000births;most frequentcause of
congenitalhydrocephalus(20-43%);
recurrencerate in siblingsof 1-4.5/o;
M : F= 2 : 1
Etiology:
(a) postinflammatory (50%): secondaryto perinatal
infection(toxoplasmosis, CMV, syphilis,mumps,
influenzavirus)or intracranial hemorrhage
= destructionof ependymalliningof aqueductwith
adjacentmarkedfibrillarygliosis
(b) developmental:aqueductalforking(= marked
branchingof aqueductinto channels)/ narrowing/
transverseseptum(X-linkedrecessiveinheritancein
25o/ool males)
(c) neoplastic(extremelyrare): pinealoma,
meningioma,tectal astrocytoma(may be missedon
routineCT scans,easilydifferentiatedby MR)
May be associatedwith: other congenitalanomalies
(16%): thumbdeformities
r/ enlargementof lateral+ 3rd ventricleswith normal-sized
4th ventricle(4th ventriclemay be normalwith
communicatinghydrocephalus)
Prognosis; 11-30%mortality
CYST
= CSF-containingintraarachnoid cyst withoutventricular
communication / brainmaldevelopment
Incidence: 1'/" of all intracranialmasses
Origin:
(1) congenital:arisingfrom clefts/ duplication/
"splitting"of arachnoidmembranewith expansionby
CSF due to secretoryactivityof arachnoidcells
= true arachnoid cyst
(2) acquired: followingsurgery/ trauma/ subarachnoid
hemorrhage/ infectionin neonatalperiod/
associatedwith extraaxialneoplasm= loculationof
CSF surroundedby arachnoidal scarringwith
expansionby osmoticfiltration/ ball-valve
mechanism= leptomeningealGlst = secondary
arachnoid cyst = acquired arachnoid cyst
Histo:cystfilledwith clearfluid,thinwall composedof
cleavedarachnoidmembranelinedby ependymal/
meningothelial cells
Age: presentationat any time duringlife
. often asymptomatic
. symptomaticdue to mass effect,hydrocephalus,
seizures,headaches,hemiparesis, intracranial
hypertension, craniomegaly, developmental delay,
visualloss,precociouspuberty,bobble-head doll
syndrome
Location:(arisein CSF cisternsbetweenbrain + dura)
(a) floor of middlefossa near tip of temporallobe
(sylvianfissure)in 50%
(b) suprasellarlchiasmaticcistern(may produce
endocrinopathy) in 10%
(c) posteriorfossa (1/3): cerebellopontine angle(11'/"),
quadrigeminal platecistern(10%),in relationship to
vermis(9%),prepontine/ interpeduncular cistern(3%)
(d) interhemispheric fissure,cerebralconvexity,anterior
midline
infratentorial
r/ forwardbowingof anteriorwall of cranialfossa
+ elevationof sphenoidridge
r/ extraaxialunilocularthin-walledCSF-densitycyst with
well-defined smoothangularmargins
r/ compressionof subarachnoidspace + subjacentbrain
(minimalmass effect)
r/ may erode innertable of calvarium
r/ ttO enhancement(intrathecalcontrastpenetratesinto
cyst on delayedscans)
r/ trtOcalcifications
MR (bestmodality):
r/ well-circumscribedlesionwith same uniformsignal
intensityas CSF + mass effect
Cx: (1) hydrocephalus(30-60%)
(2) concurrentsubdurali intracystichemorrhage
Prognosis; favorableif removedbefore onset of
braindamage
irreversible
Rx: fenestration/ cyst-peritoneal shunting
CT-DDx:
epidermoidcyst, dermoid,subduralhygroma,infarction,
porencephaly

US-DDx:
choroidplexuscyst, porencephaliccyst (communicates
with ventricle),cystictumor(solidcomponents),midline
cyst associatedwith agenesisof corpuscallosum,
dorsalcyst associatedwith holoprosencephaly, Dandy-
Walkercyst (extensionof 4th ventricle,developmental
delay),vein of Galenaneurysm
ARTERIOVENOUS
FISTULA
= abnormalcommunicationbetweenartery+ vein
resultingin tremendousamountof flow due to high
pressuregradient;leadingto enlargement+ elongationof
drainingveins
Cause:
(1) Vessellaceration(delaybetweentrauma+ clinical
manifestationdue to delayedlysisof hematoma
surroundingarteriallaceration)
(2) Angiodysplasia: fibromuscular disease,
neurofibromatosis, Ehlers-Danlossyndrome
(3) Congenitalfistula
. pulsatilemass + thrill/ bruit
. * fleUrologicsymptoms/ deficit(due to arterialsteal)
Location:
(a) carotid-cavernous sinusfistula(mostcommon)
(b) vertebralarteryfistula
(c) externalcarotidfistula(rare)
ARTERIOVENOUS
MALFORMATION
= corrgehitalabnormalityconsistingof a nidusof abnormal
dilatedtortuousarteries+ veins with racemosetangleof
closelypackedpathologicvesselsresultingin shunting
of bloodfrom arterialto venousside without
intermediarycapillarybed
Prevalence: most commonvascularlesion
Histo: affectedarterieshave thin walls (no elastica,small
amountof muscularis); interveningglioticbrain
parenchymabetweenvessels
Age: 80% by end of 4th decade;20./"<20 years of age
. headaches,seizures(nonfocalin 40/"), mental
deterioration
. progressivehemispheric neurologicdeficit(50%)
. ictusfrom acute intracranialhemorrhage(50%)
Location:
(a) supratentorial(90%): parietal> frontal> temporal
lobe > paraventricular> intraventricular
region>
occipitallobe
(b) infratentorial(10%)
Vascularsupply:
(a) pial branchesof ICA in73/" of supratentorial
location,in 50% of posteriorfossa location
(b) dural branchesof ECA in 27% with infratentorial
lesions
r/ trtOmass effect
Sk ullf ilm :
r/ speckled/ ringlikecalcifications(15-30%)
{ thinning/ thickeningof skullat contactareawith AVM
r/ prominentvasculargrooveson innertableof skull
(dilatedfeedingarteries+ drainingveins) in 27./.
Brain Disorders 263
NCCT:
r/ irregularlesionwith largefeedingarteries+ draining
veins
r/ mixeddensity(60%): dense largevessels
+ hemorrhage+ calcifications
ri isodenselesion(15%): may be recognizableby mass
effect
r/ low density(15%): brainatrophydue to ischemia
r/ not visualized(10%)
C E C T:
{ serpiginous denseenhancementin 80% (tortuous
dilatedvessels)
r/ wo enhancementin thrombosedAVM
^/ No avascularspaceswithinAVM
r/ lack of mass effect/ edema (unlessthrombosed/
, bleeding)
{ rapidshunting
r/ thickenedarachnoidcovering
r/ adjacentatrophicbrain
MR:
./ flow void (imagingwith GRASS gradientecho + long
TR sequences)
Angio:
r/ grosslydilatedefferent+ afferentvesselswith a
racemosetangle ("bagof worms")
r/ arteriovenous shuntingintoat leastone earlydraining
vein
{ negativeangiogram(compressionby hematoma/
thrombosis)
Cx: (1) Hemorrhage(common):bleedingon venous
side due to increasedpressure/ ruptured
aneurysm(5%)
(2) Infarction
Prognosis: 10o/"mortality;30% morbidity;2-3% yearly
chanceof bleedingincreasingto 6% in year
following1st bleed+ 25o/"in year following
2nd bleed
Wyburn-MasonSyndrome
= telangiectasias
of skin + retinalcirsoidaneurysm
+ AVM involvingentireoptictract (opticnerve,
thalamus,geniculatebodies,calcarinecortex);
May be associatedwith: AVMs of posteriorfossa,
neck,mandible/ maxilla
presentingin childhood
ASTROCYTOMA
lncidence: 70-75o/"of all primary intracranialtumors;
mostcommonbraintumorin children
(40-50% of all primarypediatricintracranial
neoplasms)
Location:
cerebralhemisphere(lobar),thalamus,pons,midbrain,
may spreadacrosscorpuscallosum(incidenceof
occurrenceproportionalto amountof white matter);no
particularlobardistribution ;
(a) in adults: centralwhite matterof cerebrum(15-30%
of all gliomas)
264 Radiology Review Manual

(b) in children:cerebellum(40%)+ brainstem(20/.), Prognosis: 3-10 years postoperativesurvival;

supratentorial(30%)
= Low-gradeAstrocytoma
Well-differentiated
lncidence: 9/" of all primaryintracranialtumors;
1 0 -1 5 %o f g l i o m a s
Age: 20- 40 years; M > F
Path: benignnonmetastasizing; poorlydefinedborders
with infiltrationof white matter+ basalganglia
+ cortex;NO significanttumor vascularity/
necrosis/ hemorrhage;blood-brainbarriermay
remainintact
Histo: homogeneousrelativelyuniformappearance
with proliferationof well-differentiated
fibrillary/ protoplasmic astrocytes;mildnuclear
pleomorphism + mildhypercellularity;
rare
Location: posteriorfossain children,supratentorial
adults(typicallylobar);distribution
proportionalto amountof white matter
./ may developa cyst with high-proteincontent(rare)
CT:
r/ usuallyhypodenselesionwith minimalmass effect
+ minimal/NO peritumoral edema
{ well-defined tumormargins
r/ centralcalcifications(15-20%)
{ minimal/ no contrastenhancement(normal
capillaryendothelialcells)
MR:
d well-definedhypointenselesionwith littlemass
effect/ vasogenicedema/ heterogeneityon TlWl
r/ hyperintenseon T2Wl
./ tittte/ no enhancement on Gd-DTPA
r/ cyst with contenthyperintenseto CSF (protein
content)
{ hyperintense areawithintumormass
(paramagneticeffectof methemoglobin)
r/ inhomogeneous gadolinium-DTPA enhancement of
tumornodule
A ngio:
r/ majorityavascular
occasionallyconvertinginto more
malignantform severalyears after
Presentation
AnaplasticAstrocytoma
lncidence: 11'/" ol all primary intracranialneoplasms;
25h of gliomas
Path: frequentlyvasogenicedema;NO necrosis/
hemorrhage
Histo: less well differentiated with greaterdegreeof
hypercellularity + pleomorphism, multipolar
fibrillary/ protoplasmicastrocytes;mitoses
multipolar + vascularendothelialproliferation common
Location: typicallyfrontal+ temporallobes
mitoses Distribution:proportionalto amountof white matter
MR:
in { moderatemass effect
r/ well-defined slightlyheterogeneous hypointense
lesionon TlWl with prevalentvasogenicedema
{ hyperintenseon TzWl
./ + enhancement on Gd-DTPA
Prognosis: 2 years postoperativesurvival
PilocyticAstrocytoma
= JUVENILE PILOCYTIC ASTROCYTOMA
= ffiostbenignhistologicsubtypeof astrocytomawithout
progression to high-gradeglioma
0 Most commoninfratentorialneoplasmin pediatricage
group
Histo: alternatingpatternof compactbipolarpilocytic
(hairlike)astrocytesarrangedmostlyaround
vessels+ looselyaggregatedprotoplasmic
astrocytesundergoingmicrocysticdegeneration
Age: predominantly in children+ youngadults;peak
age betweenbirthand 9 yearsof age; M:F = 1:1
Associatedwith: neurofibromatosistype 1
Location: cerebellum,hypothalamus (around3rd
ventricle),optic nerve/ chiasm
r/ micro-/ macrocystsin cerebellarlocation(in 60-80%)
! muraltumornodulelocatedin wall of cerebellarcyst

WHOClassificationof Astrocytomas
GradeI Circumscribed tumor,specificuniquehistologicfeaturesfor
astrocytoma generallybenignwelFcircumscribed
eachtumor,pilocytlc astrocytoma(mostcommon),subependymal glant cell
astrocytoma;@; lowrateof recurrence

Gradsll Astrocytoma ditfuselyinfiltrating; minimalpleomorphism

wellditferentiated; ot nuclearatypja;
no vascularDroliferation/ necrosis

Gradelll Anaplastic
astrocytoma mitoticactivity;vascular
increasedcellularity;
plegmgnhisrcltt-ouCleal3lypja;
prolifsration
+ nscrosisabsent

GradalV multilorme
Glioblastoma andnecrosis;increasedcellularity;
markedvascularoroliferation anaplasia
+ pleomorphism;variablemitoticactivity;celltypemaybe poorlydifferentiatod,
fusiform,roundor multinucleated
 Brain Disorders 265

I multilobulated / dumbbellappearancealongoptic . progressiveneurologicdeterioration

, pathway . oculomotorabnormalities, dysarthricspeech,
r/ rarelycalcifies choreoathetosis, myoclonicjerks
r/ increasedheterogeneous signalintensityon earlyGd- . mucocutaneoustelangiectasias:bulbarconjunctiva,
DTPA-enhanced Tl Wl; homogeneous enhancement ears, face, neck,palate,dorsumof hands,antecubital
on delayedimages + poplitealfossa
Prognosis; relativelybenignclinicalcourse,almost . recurrentbacterial+ viralsinopulmonary infections
neverrecursaftersurgicalexcision; r/ cerebellarcorticalatrophy:diminishedberebellarsize,
>904 postsurgicalS-yearsurvival;NO dilatationof 4th ventricle,increasedcerebellarsulcal
malignanttransformationto anaplasticform prominence
DDx: metastasis,hemangioblastoma, atypical r/ cerebralhemorrhage(ruptureof telangiectaticvessels)
medulloblastoma r/ cerebralinfarct(embolishuntedthroughvascular
malformations in lung)
PleomorphicXanthoastrocytoma Cx:
= Sup€rficially locatedsupratentorial tumor that involves (1) Bronchiectasis + pulmonaryfailure(mostcommon
leptomeninges causeof death)
Prevalence: 1./"of all brain neoplasms (2) Malignancies (10-15%): lymphoma,leukemia,
Age: average age of 2Gyears (range, S-82 years) epithelialmalignancies
Path: circumscribedtumor attachedto meningeswith
infiltration
intosurrounding brain
Histo: pleomorphicspindledtumor cells (reactiveto BINSWANGER
DISEASE
glialfibrillaryacidicprotein)with = ENCEPHALOPATHIA SUBCORTICALIS PROGRESSIVA
intracytoplasmic lipid (xanthomatous) deposits = LEUKOARIAOSIS = SUBCORTICAL ARTERIOSCLEROTIC
in a denseintercellular reticulinnetwork;giant ENCEPHALOPATHY (SAE)
cells;eosinophilic granularbodies; Cause: arteriosclerosis affectingthe poorlycollateralized
WHO gradell tumor distalpenetratingarteries(perforatingmedullary
. longhistoryof seizures(71%)
arteries,thalamoperforators, lenticulostriates,
Location:supratentorial(98%): temporal(49%)I pontineperforators);positivecorrelationwith
parietal(17%) / frontal (10%) I occipital(7"/") hypertension + aging
lobe;thalamus;cerebellum; spinalcord Path: ischemicdemyelination/ infarction
0 lts peripherallocationis the singlemost Age: >60 years
consistentimagingfeature . psychiatric changes,intellectual impairment, slowly
ri cystic(48%)supratentoriat mass with mural nodule progressive dementia,transientneurologicdeficits,
r/ intenseenhancement of solidportions seizures,spasticity,syncope
r/ CHnnnCTERISTICinvolvement of leptomeninges Location: periventricularwhite matter,centrum
(71%) semiovale,basalganglia;subcortical white
r/ peritumoral vasogenicedema/ calcification / skull matter"U" fibers+ corpuscallosumare spared
erosionare uncommon r/ multifocalhypodenselesions(periventricular, centrum
CT: semiovale) with sparingof U fibers
,
r/ frypo-/ isoattenuating mass ri lacunarinfarctsin basalganglia
MR: r/ sulcalenlargement+ dilatedlateralventricles(brain
1 hypo-to isointenserelativeto gray matteron TlWl atrophy)
! hyper-to isointenseon T2Wl MR:
Rx: surgicalresection(unresponsive to r/ focal areasof increasedsignalintensityon T2Wl
chemotherapy+ radiationtherapy) (= "unidentifiedbrightobjects")
Prognosis: 81"/"S-yearsurvival rate;7Ooh1O-year DDx: leukodystrophy, progressivemultifocal
survivalrate; high rate of recurrence; leukoencephalopathy, multiplesclerosis
malignanttransformation in 20%
DDx: meningioma, glioblastoma multiforme, CANAVAN DISEASE
oligodendroglioma, metastaticdisease,infection = SPONGIFORM LEUKODYSTROPHY
= r?re form of leukodystrophyas an autosomalrecessive
ATAXIA.TE
LANGIECTASIA disorder,mostcommonin AshkenaziJews
= autosomalrecessivedisordercharacterizedby lncidence; <100 reportedcases
telangiectasiasof skin + eye, cerebellarataxia,sinus Cause: deficiencyof aspartoacyclase leadingto
+ pulmonaryinfections, immunodeficiencies, propensity accumulationof N-acetylasparticacid in brain,
to developmalignancies plasma,urine,CSF
lncidence; 1:40,000livebirths Histo: spongydegenerationof white matterwith
Path: neuronaldegradation+ atrophyof cerebellar astrocyticswelling+ mitochondrial
elongation
cortex(? from vascularanomalies) Age: 3-6 months
. cerebellarataxiaat beginningof walkingage . markedhypotonia
266 Radiology Review Manual

. progressive megalencephaly MR:

. seizures ri well-defined area of mixedsignalintensitycentrally
. failureto attainmotor milestones (= "mulberry"-shaped lesion)with a mixtureof
. spasticity r/ increasedsignalintensity(= extracellular
. intellectual failure methemoglobin / slow bloodflow/ thrombosis)
. opticatrophywith blindness ./ decreasedintensity(= deoxyhemoglobin /
. swallowingimpairment methemoglobin
intracellular / hemosiderin /
^/ diffusesymmetricwhite matterabnormality calcification)
^/ may involvebasal ganglia ^/ surroundedby hypointense rim (= hemosiderin)on
r/ corticalatrophy T2WI
CT: A ngi o:
r/ low-densitywhite matter ^/ negative= "cfyPtic/ occultvascularmalformation"
MR: Cx: hemorrhageof varyingages
r/ whitematterhypointense on TlWl + hyperintense on DDx: (1) Hemorrhagic neoplasm(edema,masseffect)
T2WI (2) SmallAVM (thrombosed / smallfeedingvessels,
Prognosis; death in 2nd-Sth year of life associatedhemorrhage)
Dx: (1) elevationof N-acetylaspartic acid in urine (3) Capillaryangioma(no difference)
(2) deficiencyof aspartoacyclasein culturedskin
fibroblasts CENTRAL PONTINE MYELINOLYSIS
= OSMOTIC MYELINOLYSIS = OSMOTIC DEMYELINATION
CAPI LLARY TELANG I ECTASIA SYNDROME
= CAPILLARY ANGIOMA Etiology:
= abnormaldilatedcapillariesseparatedby normalneural unknown;osmoticinsult+ metaboliccompromise:
"cryptic" comatosepatientreceivingrapidcorrectioni
tissue;commonly
May be associated with: overcorrectionof severehyponatremia>12 mmol/L/day
hereditary Rendu-Osler-Weber syndrome,ataxia- (followingprolongedlV fluidadministration; 60-70% in
telangiectasiasyndrome,irradiation(latencyperiodof chronicalcoholics)
5 monthsto 22 years) Pathophysiology:
Age: typicallyin elderly rapidcorrectionof sodiumreleasesmyelinotoxic
. usuallyasymptomatic (incidental
findingat necropsy) compoundsby gray mattercomponentsresultingin
Location: mostlyin pons/ midbrain;usuallymultiple/ destruction of myelinsheaths(osmoticmyelinolysis)
may be solitary with preservationof neurons+ axons
' spasticquadriparesis + pseudobulbar palsy
r/ poorlydefinedarea of dilatedvessels(resembling
petechiae) . acute mentalstatuschange
r/ best delineatedwith MR (due to hemorrhage) . progression to pseudocoma(locked-insyndrome)in
Cx: punctatehemorrhage(uncommon), gliosis 3-5 days
+ calcifications(rare) Location: (a) isolatedpons lesion(mostcommonly)
Prognosis; bleedingin pons usuallyfatal (b) combinedtype: central+ extrapontine
DDx: cavernousangioma(identicalon images) areas: basalganglia,cerebellarwhite
matter,thalamus,caudatenucleus,
subcorticalcerebralwhite matter,corona
C A V E RNO US H E M AN G IOMA O F B R A IN radiata,lateralgeniculatebodY
= CAVERNOUSANGIOMA= CAVERNOMA CT:
Path: well-circumscribed noduleof honeycomblike large ./ diminishedattenuation in centralregionof pons
sinusoidalvascularspacesseparatedby fibrous MR (positive1-2 weeks post-onsetof symptoms):
collagenousbandswithoutintervening neural r/ singlecentralsymmetricmidlinepons lesion:
^/ trident-shaped/ round (coronalscan) + bat-wing
tissue;slow bloodflow in vascularchannels
Age: 3rd-6th decade;M > F configuration(sagittalscan)
. seizures(commonlypresentingsymptom) { hypointense on TlWl + hyperintense on T2Wl
Location: cerebrum(mainlysubcortical) > pons > r/ bilateralsymmetricwell-demarcatedlesionsin basal
cerebellum; solitary> multiPle ganglia+ otherextrapontine sites
r/ lttOobviousmass effect/ edema Prognosis; 5-10% survivalrate beyond6 months
NCCT: DDx: hypoxia,Leighdisease,Wilsondisease
{ extensivecalcifications= hemangiomacalcificans
(20%) CEPHALOCELE
r/ smallroundhyperdenseregion(CLUE) = rn€sod€rmaldefectin skull + dura with extracranial
r/ minimalsurrounding edema extensionof intracranialstructureswith persistent
CECT: connectionto subarachnoidspace
r / m inim al/in te n s ee n h a n c e m e n t CRANIALMENINGOCELE= herniationof meninges
r/ low-attenuation areasdue to thrombosedportions + C S F onl Y
 BrainDisorders 267

ENCEPHALOCELE = herniationof meninges r/ lemonsign = inwarddepressionof frontalbones (33%)

+ CSF + neuraltissue r/ cyst-within-a-cyst = herniationof
(ventriculocele
Prevalence: 4th ventricleinto cephalocele)
1-4 per 10,000livebirths;54-20% of all craniospinal r/ acute angle betweenmass + skin line of neck and
malformations; predominant neuralaxisanomalyin occiput
fetusesspontaneouslyaborted<20 weeks GA; DDx: cystic hygroma
3h of fetalanomaliesdetectedwith MS-AFPscreening;
6/" of all detectedneuraltube defectsin fetuses
Cause: Sincipital Encephalocele (1s-1s%)
failureof surfaceectodermto separatefrom Most commonvarietyin SoutheastAsia!
neuroectodermearlyin embryonicdevelopment Location: midfaceaboutdorsumof nose, orbits,and
@ Skullbase forehead
(1) faultyclosureof neuraltube (withoutmesenchyme Cause: failureof anteriorneuroporelocatednear optic
membranouscranialbone cannotdevelop) recessto close normallyat 4th week GA
(2) failureof basilarossificationcentersto unite Types:
@ Calvarium 1. Frontonasal(a0-60%)
(1) defectiveinductionof bone = herniationof dura materthroughforamencecum
(2) pressureerosionof bone by intracranialmass / + fonticulusfrontalis
cyst 2. Nasoethmoidal (30%)
ln 60% associated with: = persistentherniationof duraldiverticulum
1. Spinabifida(7-30%) throughforamencecum into prenasalspace
2. Corpuscallosumdys-/ agenesis 3. Combination of both (10%)
3. Chiarimalformation Associatedwith: midlinecraniofacialdysraphism
4. Dandy-Walkermalformation (dysgenesis of corpuscallosum,
5. Cerebellarhypoplasia interhemispheric lipoma,anomalies
6. Amnioticbandsyndrome:multipleirregular of neuralmigration,facialcleft,
asymmetricoff-midline encephaloceles schizencephaly)
7. Migrational abnormalities . obviousmass,broad nasal root, hypertelorism
8. Chromosomalanomaliesin 44./"(trisomy18) . nasalstuffiness,rhinorrhea
. MS-AFPelevatedin 3h (skin-coveredin 60%) . changein size duringcrying/ Valsalvamaneuver
. CSF rhinorrhea . positiveFUrstenberg test = changein size during
. m eningit is jugular
, compression
Prognosis; dependenton associatedmalformations r/ soft-tissuemass extendingto glabella/ nasalcavity
+ size and contentof lesion;21"/"liveborn; r/ pedunculatedintranasalmass extendingfrom
50% survivalin liveborns,74/o retarded superomedialnasalcavitydownward
0 Outcomepoorerthe largerthe brainvolume r/ enlargedforamencecum
Riskof recurrence: 3/" (25% with Meckel syndrome) OB:
DDx: teratoma,cystichygroma,iniencephaly,scalp ! widenedinterorbitaldistance
edema,hemangioma,branchialcleft cyst, CT:
cloverleafskull 'V OitiO/ absentcristagalli
r/ absentcribriformplate/ frontalbone
Nomenclature: MR:
basedon originof their roof + floor,eg r/ isointenserelativeto gray matter
frontonasal: frontalbone = roof, nasal bone = floor r/ may be hyperintenseon T2Wl (due to gliosis)
N.B.: biopsyis CONTRAINDICATED (dueto potential
for CSF leaks,seizures,meningitis)
Occipital Encephalocefe (7s%) Bisk of recurrence: 6"/" of congenitalCNS
Mostcommonencephalocele in WesternHemisphere abnormalities for youngersiblings
Associated with: Rx: completesurgicalresectionwith repairof dura
(1) Meckel-Gruber syndrome mater(NO neurologicdeficitdue to abnormal
= occipitalencephalocele+ microcephaly+ cystic functionof herniatedbrain)
dysplastickidneys+ polydactyly DDx:
(2) Dandy-Walkermalforrnation (1) Dacryocystocele / nasolacrimalmucocele
(3) Chiarimalformation (2) Nasalglioma(no subarachnoid connectionon
. externaloccipitalmass cisternography)
Location: supra-and infratentorial structuresinvolved
with equalfrequency Sphenoidal Encephalocele (10%)
{ skulldefect (visualizedin g0y.) = BASALENCEPHALOCELE
{ flatteningof basiocciput Age: presentat end of firstdecadeof life
I ventriculomegaly . clinicallyoccult
268 Radiology Review Manual

. mass in nasalcavity,nasopharynx,mouth,posterior r/ cystwall slightlyhyperdense+ nonenhancing

portionof orbit (= comPressed cerebellartissue)
. mouth breathingdue to nasopharyngeal obstruction ^/ uni-/ multilocular cyst (= necrosis)with irregular
. nasopharyngealmass increasingwith Valsalva enhancement of solidtumorportions
. diminishedvisualacuitywith hypoplasiaof optic discs r/ roundI ovallobulatedfairlywell-definediso-/
. hypothalamic-pituitary dysfunction hypodensesolidtumorwith hetero-/ homogeneous
Associatedwith: agenesisof corpuscallosum(80%) enhancement
Types: MB:
(a) sphenopharyngeal = throughsphenoidbody r/ hypointense on TlWI + hyperintense on T2Wl
(b) spheno-orbital= throughsuperiororbitalfissure { enhancement of solidtumorportion
(c) sphenoethmoidal = throughsphenoid+ ethmoid Angio: { avascular
(d) transethmoidal= throughcribriformplate Prognosis:
(e) sphenomaxillary = throughmaxillarysinus malignanttransformationexceedinglyrare
- 40% 2l-year survivalrate for solidcerebellar
ParietalEncephalocele(a-12%) astrocytoma
- 90% 2l-year survivalrate for cysticjuvenilepilocytic
Associatedwith: dysgenesisof corpuscallosum,large
interhemisphericcyst astrocytoma
r/ note in sphenoidbone (seenon submentovertex film) DDx of solid astrocytoma:
"splitcranium" (1) Medulloblastoma (hyperdensemass, noncalcified)
r/ craniumbifidum= cranioschisis=
(= skull defect)= slTloothopeningwith well-defined (2) Ependymoma(fourthventricle,50% calcify)
scleroticrim of corticalbone DDx of cystic astrocytoma:
r/ hydrocephalusin 15-80% (fromassociated (1) Hemangioblastoma (lesion<5 cm)
aqueductalstenosis,Arnold-Chiari malformation, (2) Arachnoidcyst
Dandy-Walkercyst) (3) Trapped4th ventricle
{ nonenhancing expansilehomogeneous paracranial (4) Megacisterna magna
MASS (5) Dandy-Walkercyst
{ mantleof cerebraltissueoftendifficultto image in
encephalocele(exceptwith MR) CEREBRITIS
r/ intracranialcommunicationoften not visualized = focal area of inflammationwithinbrainsubstance
{ metrizamide/ radionuclideventriculography diagnostic CT:
r/ microcephaly (2o/") r/ area of decreaseddensity+ mass effect
{ polyhydramnios { no contrastenhancement(initially)i centralor patchy
DDx: (1) sonog-aphicrefractionartifactat skull edge enhancement(lateQ
(2) cloverleaf skull (temporalbone may be MR:
partiallyabsent) r/ focal area of increasedintensityon T2Wl
Cx: brain abscess

ASTROCYTOMA
CEREBELLAR
2nd most frequenttumor of posteriorfossa in children CHIARI MALFORMATION
lncidence: 10-20% of pediatricbrain tumors Ghiari I Malformation (adulthood)
Histo: mostly grade I = "cerebellar tonsillarectopia"= herniationof cerebellar
Age: children> adults;no specificage peak; M:F = 1:1 tonsilsbelowa lineconnectingbasionwith opisthion
Path: (= foramenmagnum)
(1) cysticlesionwith tumor nodule ("muralnodule")in 0 Frequentlyisolatedhindbrainabnormality of little
cyst wall (50%);(midlineastrocytomascysticin 50%, consequence withoutsupratentorialanomalies!
hemisphericastrocytomascysticin 80%) Proposed causes:
(2) solid mass with cystic (= necrotic)center(4045%) (a) small posteriorfossa
(3) solidtumorwithoutnecrosis(<10%) (b) disproportionate CSF absorptionfrom
. cerebellarsigns: truncalataxia,dysdiadochokinesia subarachnoid sPinalspace
Location: originatingin midlinewith extensioninto (c) cerebellarovergrowth
cerebellarhemisphere(30%)> vermis> Associated with:
tonsils> brainstem (1) syringohydromyelia (20-30%)
r/ calcifications(20%): dense/ faint/ reticular/ punctate/ (2) hydrocephalus(25-44%)
globular;mostlyin solidvariety (3) malformation of skullbase + cervicalspine:
./ may developextremehydrocephalus(quitelargewhen (a) basilarimpression(25/")
finallysymptomatic) (b) craniovertebral fusion,eg, occipitalizationof C1
CT: (10/"), incompleteossificationof C1-ring(5%)
{ round/ oval cyst with densityof cyst fluid > CSF (c) Klippel-FeilanomalY(107d
r/ round/ oval/ plaquelikemuralnodulewith intense (d) platybasia
homogeneous enhancement 0 NOT associatedwith myelomeningocele!

. benigncerebellarectopia<3 mm of no clinical
consequence;3-5 mm of uncertainsignificance;
>5 mm clinicalsymptomslikely
. no symptomsin childhood(unlessassociatedwith
hydrocephalus / syringomyelia)
. may have cranialnervedysfunction/ dissociated
anesthesiaof lowerextremitiesin adulthood
r/ downwarddisplacement of cerebellartonsils+ medial
part of the inferiorlobesof the cerebellum5 mm
belowthe levelof the foramenmagnum
r/ inferiorpointingpeglike/ triangulartonsils
{ obliterationof cisternamagna
r/ elongationof 4th ventricle,whichremainsin normal
position
{ slightanteriorangulationof lowerbrainstem
Chiari ll Malformation (childhood)
= AFINOLD-CHIARI MALFORMATION
= rTlostcommonand seriouscomplexof anomalies
secondaryto a too small posteriorfossa involving
hindbrain,spine,mesoderm
HALLMARKis dysgenesisof hindbrainwith
(1) caudallydisplaced4th ventricle
(2) caudallydisplacedbrainstem
(3) tonsillar+ vermianherniationthroughforamen
m agnum
Associated with:
(a) spinalanomalies
(1) lumbarmyelomeningocele (>95%)
(2) syringohydromyelia
(b) supratentorialanomalies
(1) dysgenesisof corpuscallosum(gO-85%)
(2) obstructivehydrocephalus(50-9g%)following
closureof myelomeningocele
(3) absenceof septumpellucidum(40%)
(4) excessivecorticalgyration
(stenogyria= histologically
normalcortex;
polymicroglfia= histologically
abnormalcortex)
NOT associatedwith basilarimpression / C1-
assimilation / Klippel-Feil
deformityl
. newborn: respiratorydistress,apneicspells,
bradycardia,impairedswallowing,poor gag reflex,
retrocollis,spasticityof upperextremities
. teenager: gradualloss of function+ spasticityof
lowerextremities
Sk ullf ilm :
^/ Luckenschddel(mostprominentnear torcular
herophili/ vertex)in 85% = dysplasiaof
membranousskulldisappearing by 6 monthsof age
r/ scallopingof clivus+ posterioraspectof petrous
pyramids(frompressureof cerebellum)in 7O_gO%
, leadingto shorteningof IAC
r/ small posteriorfossa
{ enlargedforamenmagnum+ enlargedupperspinal
canalsecondaryto moldingin 7S/"
_ absent/ hypoplasticposteriorarch of C1 (70%)
r/
@ Supratentorial
^i hydrocephalus(ductof Sylviusdysfunctionalbut
probe patent);may not becomeevidentuntilafter
repairof myelomeningocele (90%)
BrainDisorders 269
r/ colpocephaly(= enlargementof occipitalhorns
+ atria)due to maldevelopedoccipitallobes
! hypoplasia / absenceof splenium+ rostrumof
corpuscallosum(80-90%)
. "bat-wing"
r/ configurationoi'frontalhornson coronal
views= frontalhornspointinginferiorlywith blunt
superolateralangle secondaryto prominent
impressions by enlargedcaudatenucleus
r/ "hourglassventricle"= small biconcave3rd
ventriclesecondaryto large massa intermedia
{ interdigitation of medialcorticalgyri (hypoplasi"
+ fenestration of falx in up to 100%)
r/ wide prepontine+ supracerebellar cisterns
r/ nonvisualization of aqueduct(in up to 7O%)
r/ stenogyria= multiplesmall closelyspacedgyri at
medialaspectof occipitallobe secondaryto
dysplasia(in up to 50%)
@ Cerebellum
r/ "cerebellarpeg" = protrusionof vermis
+ hemispheres throughforamenmagnum(gO%)
resulting in craniocaudal elongationof cerebellum
,
! hypoplasticpoorlydifferentiated cerebellum(poor
visualization of foliaon sagittalimages)secondary
to severedegeneration
r/ elongated/ obliteratedverticallyorientedthin-
tubed 4th ventriclewith narrowedAp diameter
, exitingbelowforamenmagnum(40%)
{ obliterationof CPA cistern+ cisternamagnaby
cerebellumgrowingaroundbrainstem
r/ dysplastictentoriumwith wide U-shapedincisura
, insertingcloseto foramenmagnum(gS%)
! 'lcqblbeaking" = fusionof midbraincolliculiinto a
singlebeak pointingposteriorly and invaginating
intocerebellum
./ V-shapedwidenedquadrigeminalplatecistern
, (dueto hypoplasiaof cingulate gyri)
! "toweringcerebellum" = "pseudomass,'=
cerebellarextensionabove incisuraof tentorium
^/ triplepeak configuration= cornersof cerebellum
wrappedaroundbrainstempointinganteriorly
, + laterally(on axialimages)
{ flattenedsuperiorportionof cerebellumsecondary
, to temporoparietal herniation
! verticalorientationof shortenedstraightsinus
@ Spinalcord
r/ medulla+ pons displacedinto cervicalcanal
{ 'cervicomedullary kffi" = herniationof medulla
posteriorto spinalcord (up to 70h) at levelof
dentateligaments
r/ widenedanteriorsubarachnoidspace at levelof
brainstem+ uppercervicalspine (40%)
r/ nP diameterof pons narrowed
{ uppercervicalnerve rootsascendtowardtheir exit
foramina
r/ syringohydromyelia
r/ low-lyingoftentetheredconus medullarisbelow L2
OB -U S :
^/ hydrocephalus
270 Radiology Review Manual

{ "bananasign"= cerebellumwrappedaround C H OR OID P LE X U S P A P ILLOMA

posteriorbrainstem+ obliteration of cisternamagna lncidence: 0.5-0.6% of all intracranialtumors;2-5/" of
secondary to small posterior
fossa + downward braintumorsin childhood
tractionof spinalcord in Chiarill malformation Age: 2O4O% <1 year of age; 86% <5 years of age;
middleage; in 75/" <2 yearsof age; M >> F
Chiarilll Malformation Path: largeaggregationof choroidalfrondsproducing
most severerare abnormality;probablyunrelatedto greatquantitiesof CSF; occasionally found
type I and ll Chiarimalformation incidentally on postmortemexamination
^/ low occipital/ high cervicalmeningomyelo- Pathophysiology:abnormalrate of CSF productionof
encephalocele 1.0mU mi n(normalrate= 0. 2 m Um in)
Prognosis; survivalusuallynot beyondinfancy . signsof increasedintracranialpressure
Location:
(a) glomusof choroidplexusin trigoneof lateral
ChiarilV Malformation ventricles,L > R (in children)
(b) 4th ventricle+ cerebellopontine angle (in adults)
extremelyrareanomalyprobablyerroneouslyincluded
as type of Chiarimalformation (c) 3rd ventricle(unusual)
r/ agenesisof cerebellum (d) mul ti P l ei nT" h
r/ hypoplasiaof pons r/ large mass with smoothlobulatedborder
{ small+ funnel-shaped posteriorfossa r/ smallfoci of calcifications(common)
! engulfmentof glomusof choroidplexus(distinctive
feature)
PLEXUSCYST
CHOROID ^/ asymmetricdiffuseventriculardilatation
= cyst arisingfromfoldingof neuroepithelium with trapping (CSF overproduction / decreasedabsorptionsecondary
of secretoryproducts+ desquamatedcells to obstructionof arachnoidgranulationsfrom repeated
lncidence; 0.9-3.6/"in sonographic population; 50% of occulthemorrhage)
autopsiedbrains r/ dilatationof temporalhorn in atriallocation(obstruction)
Histo: no epitheliallining,filledwith clearfluid+ debris r/ growthinto surroundingwhite matter(occasionally,
May be associated with: more commona featureof choroidplexuscarcinoma)
aneuploidy(76%with trisomy18, 17'/.withtrisomy21, CT:
syndrome) ^/ iso-/ mildlyhyperdense with intensehomogeneous
7'/.with triploidyi Klinefelter
0 ln absenceof other anomalies1"/oof fetuseswith enhancement on CECT
choroidplexuscystswill havetrisomy18! MR:
r/ isointense / slightlyhyperintense lesionon TlWl
0 ln presenceof other anomalies4ohof fetuseswith
choroidplexuscystswill havetrisomy18! + slightlyhypointenseon T2Wl relativeto white matter
{ surroundedby hypointense signalon TlWl
O 40-71% of autopsiedfetuseswith trisomy18 have
>10 mm in diameter + hyperintense signal on T2Wl (CSF)
choroidplexuscystsbilaterally
0 Riskof chromosomal abnormalitynot linkedto size, r/ intraventricular enhancingislandof tumoron Gd-DTPA
bilaterality,gestationalage at appearanceI US:
disappearance ./ echogenicmass adjacentto normalchoroidplexus
. usuallyasymptomatic A ngi o:
Location: frequentlyat levelof atrium;uni- / bilateral r/ suppliedby anterior+ posteriorchoroidalarteries
r/ single/ multipleroundanechoiccysts Cx: (1) transformation into malignantchoroidplexus
./ >3 mm in size (average4.5 mm, up to 25 mm) papilloma= choroidplexuscarcinoma
(2) hydrocephalus (in children)secondaryto
Cx: hydrocephalus(if cyst large)
Prognosis: 9O/" disappearby 28th week; may persist;in increasedintracranialpressurefrom CSF-
95/o of no significance overProduction
OB-management: Rx: surgicalremoval(24% operativemortality)cures
a choroidplexuscyst shouldstimulatea thorough hydrocePhalus
DDx: intraventricular meningioma, ependymoma,
sonographicexaminationat >19 weeks; if no other
sonographic abnormalities the yieldof
are identified, metastasis, cavernousangioma,xanthogranuloma,
abnormalkaryotypeis low so that the risk of trisomy18 astrocYtoma
(1:450-500)is lowerthan riskof fetallossdue to
amniocentesis(approximately1:200-300)
Risk of karyotype abnormality: SYNDROME
COCKAYNE
10 timeswith 1 additionaldefect = autosomalrecessivediffusedemyelinatingdisease
600 times with >2 additionaldefects Age: beginningat age 1
DDx: Choroidplexuspseudocystin the inferolateral . dwarfism
aspectof atrium(? corpusstriatum)on oblique . progressivephysical+ mentaldeterioration
coronalplane,whichelongatesby turning . retinalatrophy+ deafness
transducer { brain atrophy/ microcePhalY
 BrainDisorders 271

r/ calcifications
in basalganglia+ cerebellum 2. Contrecoup (180"oppositeto sideof impact)
d skeletalchangessuperficiallysimilarto progeria = broad areaof impactas a resultof movingbrain
DDx: Progeria againststationarycalvarium
Associatedwith: fall

COLLOIDCYST Location: multiplebilaterallesions;

lncidence: 2o/"of glialtumorsof ependymalorigin;
0.5-1% of CNS tumors
Histo: ciliated+ columnarepithelium; mucin-secreting;
squamouscellsof ependymalorigin;toughfibrous
capsule
Age: youngadults; M > F
. positionalheadaches(transientobstructionsecondary
to ball-valvemechanismat foramenof Monro)
. gait apraxia
. changein mentalstatus+ dementia(relatedto
increasedintracranialpressure)
. papilledema (maybecomemedicalemergencywith
acuteherniation)
Location: exclusivelyarisingfrom inferioraspectof
septumpellucidumprotrudingintoanterior
portionof 3rd ventriclebetweencolumnsof
fornix
{ + sellarerosion
r/ sphericaliso-/ hyperdenselesionon NCCTwith smooth
surface
./ ttuiocontents:
(a) in 20% similarto CSF (= isodense)
(b) in 80% mucinousfluid,proteinaceous debris,
hemosiderin, desquamated cells(= hyperdense)
r/ may show enhancementof border(OiapeOchoroid
, plexus/ capsule)
{ 3rd ventricularenlargement(to accommodatecyst
anteriorly)
r/ asymmetriclateralventricularenlargement(invariably)
./ occasionally widensseptumpellucidum
MR:
r/ lesionhyperintense on TlWl + hyperintenseon T2Wl
in 60% (relatedto largeproteinmolecules/
paramagnetic effectof magnesium,copper,iron in cyst)
DDx: meningioma,ependymomaof 3rd ventricle(rare)
with enhancement
CORTICAL
CONTUSION
= CEREBRAL CONTUSION = BRAINCONTUSION
= traumaticinjuryto corticalsurfaceof brain
lncidence.' most commontype of primaryintraaxial
lesion;in 21"/"of head traumapatients;
c hild re n :a d u l=ts2 :1
Pathogenesis: capillarydisruptionleadsto extravasation
of wholeblood,plasma(edema)and RBCs
Path: petechialhemorrhage(= admixtureof bloodwith
nativetissue)followedby liquefaction+ edema
afterf7 days,tissue necrosis
Mechanism: linearacceleration-deceleration
forces/
penetratingtrauma
1. Coup (sameside as impact)
= srTt?llarea of directimpacton stationarybrain
Associatedwith: skull fracture
- common: alonganterior+ lateral+ inferiorsurfaces
of frontallobe (in orbitofrontal,
inferiorfrontal,and
rectalgyri abovecribriformplate,planum
sphenoidale, lessersphenoidwing)and temporal
lobe (justabove petrousbone i posteriorto greater
sphenoidwing)
- lessfrequent:in parietal+ occipitallobes,cerebellar
hemispheres, vermis,cerebellartonsils
- often bilateral/ beneathan acutesubdural
hematoma
. confusion(mildinitialimpairment)
. focal cerebraldysfunction
. seizures,personality changes
. focalneurologicdeficits(latechanges)
CT (sensitiveonlyto hemorrhagein acutephase):
0 Look for scalp swellingto focus your attentionon the
locationof the coup!
r/ "saltand pepperlesion"= mottled/ speckleddensities
as focal/ multiple(29%)poorlydefinedareas of low
attenuationwith irregufarcontour(edema)intermixed
with a few tiny areas of increaseddensity(petechial
hemorrhage)
r/ diffusecerebralhypodensity+ swellingwithout
hemorrhagein immediateposttraumaticperiod
(commonin children)due to hyperemia/ ischemic
edema
ri somedegreeof contrastenhancement(leakingnew
capillaries)
r/ hemorrhageisodenseafter 2-3 weeks
r/ true extentof lesionsbecomesmoreevidentwith
progressionof edema + cell necrosis+ mass effect
over ensuingweeks
MR (bestmodalityfor initialdetectionof contusional
edema + accurateportrayalof extentof lesions):
r/ hemorrhagic lesions(detectedin 50% of all
contusions):
^/ initiattydecreasedintensity(deoxyhemoglobin of
acutehemorrhage) surroundedby hyperintense
edemaon T2Wl
r/ hyperintense on Tl Wl + T2Wl in subacutephase
(secondaryto Met-Hb)
r/ hyperintense gliosis+ hypointensehemosiderin on
T2Wl in chronicphase
r/ nonhemorrhagic lesionshypointense on TlWl
+ hyperintenseon T2Wl
Cx: (1) Progression to cerebralhematoma
(2) Encephalomalacia (= s6spped
brain)
(3) Porencephaly(= formationof cysticcavitylined
with glioticbrainand communicating with
ventricles/ subarachnoidspace)
(4) Hydrocephalusas a resultof adhesionscaused
by subarachnoid blood
272 Radiology Review Manual

CRANIOPHARYNGIOMA r/ lateraldisplacement,elevation,narrowingof
lncidence: 3-4h o{ all intracranialneoplasms;15% of supraclinoidsegmentof ICA
supratentorial + 50% of suprasellar tumorsin r/ posteriordisplacementof basilarartery
children; most common suprasellar mass DDx: (1) Epidermoid(no contrastenhancement)
lesion)
(2) Rathkecleftcyst (smallintrasellar
Origin: from epithelialrests alongvestigial
craniopharyngeal duct (Rathkecleft / pouch
withinintermediate lobeof pituitarygland)
Path: benigntumororiginating from neuroepithelium in
craniopharyngeal duct + primitivebuccalepithelium OF BRAIN
CYSTICERCOSIS
Histo: cystic(richin liquidcholesterol)/ complex/ solid Larva of pork tapeworm (Taeniasolium) frequently
Age: from birth-7thdecade;bimodalage distribution: involvingCNS, eyes,muscle,heart,fat tissue,skin
age peaks in 1st-2nd decade(75%)+ in Boute of lnfection:
5th decade(25%); M > F (1) Ingestionof ova by fecal-oralroutevia contaminated
. diabetesinsipidus(compression of pituitarygland) food / water or autoinfection;embryophoreis
. growthretardation(compressionof hypothalamus) dissolvedby gastricacid and enzymes+ oncosphere
. bitemporalhemianopia(compression of opticnerve is liberated
(2) Ingestionof undercooked contaminated pork
chiasm)
. headachesfrom hydrocephalus(compressionof containingcysticerci; tapewormdevelopsin intestinal
foramenof Monro/ aqueductof Sylvius) lumen+ releaseseggs
Organism:
Location: embryosinvadeintestinal wall + entercirculation
(a) pituitarystalk/ tubercinereum + disseminatein variespartsof body;embryodevelops
(b) suprasellar(20%) into a cysticercus(= comPlexwall surroundinga cavity
(c) intrasellar(10%) containingvesicularfluid+ scolex);followingingestion
(d) intra-and suprasellar(70/.) of cysticercusby definitivehost a tapewormdevelops
Ectopic craniopharYngioma: withinthe intestinaltract
(e) floorof anterior3rd ventricle(morecommonin lncidence: most commonparasiticinfectioninvolving
adults) CNS in develoPing countries;CNS
(f) sphenoidbone involvement in uPto 90%
S k ullf ilm s : Endemicto: Mexico,SouthAmerica,Africa,eastern
r/ normalsella (25%) EuroPe,Asia,Indonesia
^/ enlargedJ-shapedsellawith truncateddorsum Location: meninges(39%)esp. in basalcisterns,
{ thickening+ increaseddensityof laminadura in floor parenc hy ma (20/"), int raventric uIar (17'/"),
of sella(10%) mixed(23%),intrasPinal (1%)
r/ extensivesellardestruction(75%) Seeding: throughsubarachnoidspace
{ curvilinear /
/ flocculent/ stippledcalcifications + intraventricular sYstem
lamellarossification; calcificationsseen in youthin A. LARVALTISSUEINVASIONSTAGE
70-90%. in adults in 30-40% . asymPtomatic
CT: ri localizedfocus of edema on T2Wl
inhomogeneous
{ multilobulated suprasellarmass { nodulartissueenhancement
{ solid(15%) lmixed (30%)/ cysticlesion(54-75%) B . V E S IC U LA R S TA GE
= antigenetically inert,thereforewithoutinflammatory
[cysticappearancesecondaryto cholesterol,keratin,
necroticdebriswith higherdensitythan CSFI reaction/ circumferential edema
r/ enhancement of solidlesion,peripheralenhancement . asymptomatic
of cystic lesion r/ single/ multiplethin-wallednonenhancing 4-20 mm
r/ marginalhyperdenselesion(calcification / spherical cysts:
ossification) in 70-90% in childhoodtumors r/ centerwith clearfluidof CSF intensity
'V z-S mm muralnodule(= scolex)isointensewith
+ 30-50% of adulttumors
r/ + obstructivehydrocephalus brainParenchYma
{ extensioninto middle> anterior> posteriorcranial C. COLLOIDALSTAGE
fossa (25%) = scolexdies and its metabolicbreakdown(colloidal
MR (relativelyineffectivein demonstratingcalcifications): suspension)resultsin focalmeningoencephalitis
r/ mostlyhyperintense, on
but also iso-i hypointense with breakdownof blood-brainbarrier
Tl Wl (variablesecondaryto hemorrhage / . focalseizures(in endemiccountriesmostcommon
cholesterol-containi ng proteinaceous fluid) causeof adult-onset ePilePsY)
. headache,signsof increasedintracranialpressure
r/ markedlyhyperintenseon T2Wl
{ marginalenhancement of solidcomponentswith { avid ring-enhancing capsuleon TlWl
gadopentetate dimeglumine { centerhypointenseto white matterand hyperintense
A ngio: to CSF on TlWl + markedlyhyperintense on T2Wl
r/ usuatlyavascular (due to proteinaceousnatureof cyst ftuid)
 BrainDisorders 273

r/ hypointense muralnoduleon T2Wl with strong Prenatal screening:

, homogeneous enhancement antibodiesin 30-60% of pregnantwomen;
r/ with extensivewhitematteredema(DDx: primaryCMV infectionin 2.5./.of pregnantwomen
metastasiswithoutedema) Postnatal screening:
D. NODULAR-GRANULAR STAGE 10"hof neonatesexcretevirus:
= degeneration of cysticercus
with mineralization 1.6%of newbornsshed CMV in urine/ saliva
r/ graduallysubsidingperilesional edema . asymptomatic + subclinical(90%)
ri shrinkageof cyst becomingisointensewith brainon . symptomatic at birth(5-10%):
TlWl + hypointense on TzWl . sensorineural deafness,mentalretardation,
r/ isoattenuating lesionwith enhancement of thick neurologicdeficits,seizures
nodularringon CT . ocularabnormalities (15-50%): chorioretinitis,optic
E. CA LCI F I E DS T AGE neuritis,opticatrophy,hypoplasia+ colobomaof optic
= compl€teinvolutionof lesionwith continued nerve,anterioruveitis,anophthalmia, microphthalmia,
mineralization cataracts,cyclopia
.. asymptomatic/ posttreatmentseizures . jaundice,hemolyticanemia,thrombocytopenic
r/ smallfocal calcifications;may appearwithin purpura
8 monthsto 10 years after acute infection 0 Leadingcauseof braindisease+ hearingloss in
r/ "ricelike"musclecalcificationsrarelyvisible chi l dren!
. symptomaticin adults(in up to 15%):
RA DI O G RA P HlTCYP ES fever,pharyngitis, lymphadenopathy, polyarthritis
1. Parenchymal type r/ intrauterinegrowthretardation
r/ multiple/ solitarycysticlesionsup to 6 cm in size: ri hepatosplenomegaly (nontender)
, r/ largecysts are usuallymultiloculated { ascites
tr manyterminateas calcifiedgranulomata(larvae r/ hydrops
not dead unlesscompletelycalcified) r/ pneumonitis
^/ encephalitic form may occurin children @ CNS
2. Subarachnoid / racemose neurocysticercosis r/ periventricular subependymalcysts (= focal areasof
= infiltrationof basalcisterns+ sylvianfissures necrosis+ glialreaction)
associatedwith localmeningealinflammation / ./ intracranial calcifications:
fibrosis r/ periventricular postinflammatory calcifications
r/ lucentcysticlesionsup to severalcm in basal r/ scatteredcalcifications in basaiganglia+ thalami
cisterns(= rocernosecysts)with variable r/ nigntyechogenicthickenedwallsof ienticulostriate
enhancement, usuallylocatedin cerebellopontine vessels(= mineralized vasculopathy with
angle/ suprasellar cistern depositionof amorphousbasophilicmaterialin
Cx: hydrocephalus; scatteredinfarctions(due to arterialwalls)
vasculitisof basalperforatingvessels) V calcifications throughoutbrainparenchyma
3. Intraventricular neurocysticercosis r/ ventricular dilatation(dueto ventriculitis
/ obstruction
r/ obstructivehydrocephiluscausedby blockage by inflammatoryexudate/ brain atrophy)
withinvariousportionsof ventricular systemfrom ^/ intraventricular septa
solitary/ multiplecysts(OCCULTon CTI) r/ microcephaly(due to encephaloclastic effectof virus
4. Mixedtype (frequent) / disturbance of cell proliferation
resultingin brain
atrophy)
CYTOMEGALOVIRUS
INFECTION r/ lissencephaly, corticaldysplasia/ atrophy,
= double-stranded DNAviruswith replication insidecell heterotopia, polymicrogyria, schizencephaly (dueto
nucleuscausinga lytic productive/ fatentinfection; disturbedneuronalmigration)
memberof Herpesviridae family(withvaricella-zoster r/ severediffusehypoplasia/ dysplasiaof cerebellum
virus,Epstein-Barr virus,herpessimplexvirustypes 1 Dx: positiveviral culturewithinfirst 2 weeks of life
and 2) Rx: no effectivetreatmentfor maternalinfection
0 Most commonintrauterineinfection DDx: toxoplasmosis,teratoma,tuberoussclerosis,
lncidence: 0.4-2A% of liveborninfants;40,000babies Sturge-Weber syndrome,venoussinusthrombosis
born each year with CMV infection
Transmission: DAN DY-WALKER MALFORMATION
(a) horizontally by contactwith saliva/ urineor sexually = characterized by (1) enlargedposteriorfossawith high
(b) verticallyfrom motherto fetus transplacentally; positionof tentorium(2) dys-/ agenesisof cerebellar
spreadshematogenously throughoutfetus vermis(3) cysticdilatationof 4th ventriclefillingnearly
0 Severefetal morbidityif infectedduringfirst half of entireposteriorfossa
pregnancy! lncidence: 12/o of all congenitalhydrocephaly
Histo: necrotizinginflammatoryprocess Path: defectin vermisconnectingan ependyma-lined
Predilection: cMV has specialaffinityfor metabolically retrocerebellar cyst with 4th ventricle
activeneuroblastsof germinalmatrix (P A TH OGN OMON TC )
274 Radiology Review Manual
Cause: dysmorphogenesis of roof of 4th ventriclewith
failureto incorporatethe area membranacea
intodevelopingchoroidplexus;proposed
originallyas congenitalatresiaof foraminaof
Luschka(laterat)+ Magendie(median)not likely
sinceforaminaare not patentuntil4th month
Associatedanomalies:
- m idlineCN S a n o ma l i e s(i n > 6 0 % )
(1) Dysgenesisof corpuscallosum(20-25%),lipoma
of corpuscallosum
(2) Holoprosenceph aly (25%)
(3) Malformationof cerebralgyri (dysplasiaof
cingulategyrus)(25%)
(4) Cerebellarheterotopia+ malformationof
cerebellarfolia(25%)
(5) Malformation of inferiorolivarynucleus
(6) Hamartomaof tubercinereum
(7) Syringomyelia
(8) Cleft palate
(9) Occipitalencephalocele (<5%)
- otherCNS anomalies:
(1) Polymicrogyria I graymatterheterotopia
(2) Schizencephaly
(3) Lumbosacral meningocele
- non-CNSanomalies(25%)
(1) Polydactyly,syndactylY
(2) Klippel-Feil syndrome
(3) Corneliade Langesyndrome
(4) Cleft palate
(5) Facialangioma
(6) Cardiacanomalies
Sk ullf ilm :
./ largeskull secondaryto hydrocephalus
+ dolichocephaly
{ diastaticlambdoidsuture
^/ disproportionately largeexpandedposteriorfossa
{ torcularherophiliand lateralsinuseshigh above
lambdoidangle= torcular-lambdoid inversion
CT/US/MR:
{ absence/ hypoplasiaof cerebellarvermis:
total (25o/o),partial (75%)
r/ superiorlydisplacedsuperiorvermiscerebelli
{ small+ widelyseparatedcerebellarhemispheres
r/ anterior+ lateraldisplacementof + hypoplastic
cerebellarhemispheres
r/ largeposteriorfossa cyst with extensionthrough
foramenmagnum= diverticulum of roofless
4th ventricle
ri elevatedinsertionof tentoriumcerebelli
r/ cerebellarhemispheres in appositionwithout
intervening vermisfollowingshuntprocedure
^/ absenceof falx cerebelli
{ scaltopingof petrouspyramids
r/ ventriculomegaly (in72% open communication
3rd ventricle;in 39% patent4th ventricle;in 28/"
aqueductalstenosis;in 11"hincisuralobstruction);
presentprenatallyin 307",by 3 monthsof age in 75/"
r/ anteriordisplacementof pons
A ngio:
./ nigh positionof transversesinus
ri elevatedgreatvein of Galen
r/ elevatedposteriorcerebralvessels
r/ anterosuperiorlydisplacedsuperiorcerebellararteries
abovethe posteriorcerebralarteries
{ small / absentPICAwith high tonsillarloop
"keyhole
Cx: trappingof cyst abovetentorium=
configuration"
Prognosis; fetal demise in 66%; 22-50% mortality
during1stYearof life
DDx: (1) Posteriorfossa extra-axialcyst
(2) Arachnoidcyst (normal4th ventricle,patent
foramina,intactvermis)
(3) lsolated4th ventricle
(4) Megacisterna magna= giantcisternamagna
(enlargedposteriorfossa,enlargedcisterna
magna,intactvermis,normal4th ventricle)
(5) Porencephaly
Variant
Dandy-Walker
characterizedby
(5-10%) (1) variablehypoplasiaof posteroinferior portionof
vermisleadingto communication between
4th ventricleand cisternamagna
(2) cerebellardysgenesis
(3) cysticdilatationof 4th ventricle
(4) NO enlargementof posteriorfossa
0 Morecommonthan Dandy-Walker malformation;
accountstor 113of all posteriorfossa malformations
Cause: focalinsultto developingcerebellum
AssociatedCNS anomalies:
agenesisof corpuscallosum(21'/"),cerebralgyral
malformation (217o),heterotopia, holoprosencephaly
(10%),diencephalic cyst (10%),posteriorfossa
meningoencephalocele (10%)
Other associated anomalies :
polydactyly; cardiac,renal,facialanomalies;abnormal
karyotype(29%)
r/ +tn ventriclesmaller+ betterformed
{ retrocerebellar cyst smaller
i communicationbetweenretrocerebellar cyst and
subarachnoid spacethrougha patentforamenof
Magendiemay be Present
./ posteriorfossa smallerthan in usual Dandy-Walker
syndrome
OB-US:
r/ incompleteclosureof vermisis normaluntil
18 w eeksGA !
Complex
Dandy-Walker
= continuumof anomalies,includingDandy-Walker
malformation + Dandy-Walker variant+ megacisterna
magna,characterizedbypartial/ complete
with dysgenesisof vermiscerebelli
Cause: broad insultto alar platefrom a varietyof
abnormalities
Associatedwith:
A. InheritedgeneticsYndromes
- autosomalrecessive:
1. Meckel-Gruber sYndrome
 Brain Disorders 275

2. Ellis-vanCreveldsyndrome (1) axons resuftingin axonaltears foflowedby wallerian

3. Walker-Warburg syndrome degeneration
- autosomaldominant:
(2) smallwhite-matter vesselsresultingin small
1. X-linkedcerebellarhypoplasia petechialhemorrhages
2. Aicardisyndrome Path: muchof the injuryis only microscopic
B. Abnormatkaryotype(33%) Histo: multipleaxonalretractionballs (HALLMARK),
1. Duplications of chromosomes Sp,gp, gq numerousperivascular hemorrhages
2. T r is o m i e g
s , 19,1g . immediatesevereimpairmentof consciousness at time
C. Infection of impact
1. V ir us : C MV ,ru b e l l a . persistentvegetativestate
2. Protozoan:toxoplasmosis Location(accordingto severityof trauma):
D. Teratogen:alcohol,sodiumwarfarin (a) lobarwhitematterat corticomedullary junction
E. Multifactorial (67%): parasagittalregionof frontallobe
+ periventricularregionof temporallobe;
Pseudo-Dandy-Walker
Malformation occasionallyin parietal+ occipitallobes
= developingrhombencephalon during1sttrimester (b) internal+ externalcapsule/ basalganglia,corona
r/ fluid-filledspace in posterioraspectof fetal head radiata,cerebellarpeduncles
(c) corpuscallosum(21%): 314of lesionsat
undersurface of posteriorbody + splenium
DERMOID
OF CNS ri often associatedwith intraventricular
hemorrhage
= pilosebaceousmass linedwith skin appendages (d) brainstem: posterolateral quadrantsof midbrain
originating from inclusionof epithelialcells+ skin + upperpons;superiorcerebellarpeduncles
appendagesduringclosureof neuraltube especiallyvulnerable
lncidence: 1"/"of all intracranialtumors ! sparing of cortex
Path: ectodermal+ mesodermallesion= sgUornouS 4 ZOt"of lesionswith smallcentralareasof petechial
epithelium,mesodermalcells(hairfollicles,sweat hemorrhage
+ sebaceousglands) CT (negativein 30% of positiveMR cases):
Age: <30 years (appearsin adulthoodsecondaryto r/ foci of decreaseddensity(usuallyseen when >1.5cm
s l o w g r o w t h )M; < F in size)
Location: MR (mostsensitivemodality):
(a) spinalcanal(mostcommon):extra-/ intramedullary { multiplesmall oval lroundfoci of decreasedsignal
in lumbosacral region intensityon Tl Wl + increasedsignalon T2Wl
(b) posteriorfossa withinvermis/ 4th ventricle Prognosis:
(predilectionfor midline) (1) poordue to sequelae(maygo on to die withoutsigns
(c) posteriorto superiororbitalfissure,may be of highintracranial pressure)
associatedwith bone defect (2) brainatrophywith enlargementof sulci + ventricles
. bouts of chemicallbacterialmeningitispossible
r/ thick-walledinhomogeneous masswith focalareasof fat
{ mural/ centralcalcifications / bone (possible) DIFFUSE SCLEROSIS
{ may havesinustractto skin surface'(dermal sinus)if sporadic,youngadults,fulminantcourse
locatedin midlineat occipital/nasofrontal region ' dementia,deafness
r/ tat-tluidlevelif cyst rupturesinto ventricles,fat droplets r/ low-attenuation
regionsin both hemispheres
without
in subarachnoid space symmetry
./ tttOcontrastenhancement
MR:
r/ variointense on TlWl (hyperintense with contentsof D U R A L S IN U S TH R OMB OS IS
liquefiedcholesterolproducts) = VENOUS SINUSTHROMBOSIS
{ shortenedT1 + T2 relaxationtimes (= fat) 0 The radiologistmay be the firstto suggestthe diagnosisl
Cause:
DIFFUSE
AXONALINJURY A. ldiopathic= spontaneous(10-30%)
= WHITEMATTERSHEARINGINJURY B. Septiccauses(esp.in childhood):
lncidence: mostcommontype of primarytraumaticinjury mastoiditis,sub-/ epiduralempyema,meningitis,
_ in patientswith severehead trauma(49%) encephalitis, brainabscess,face + scalpcellulitis,
Cause: high-velocitytrauma (MVA)resultingin indirect septicemia
injurydue to rotational/angular(especially C. Asepticcauses:
coronal)acceleration/ decelerationforces (a) Tumorcompressing sinuses: meningioma,
(directimpactto head or fracturenot required) blasticcrisisof chronicmyelogenousleukemia
Pathogenesis: (b) Trauma: fracturethroughsinuswall,cranial
cortexand deep structuresmove at differentspeed surgery,jugularvein catheterization
causingshearingstressof (c) Low-flowstate: CHF, CHD, dehydration,shock
276 Radiology Review Manual

(d) Hypercoagulability: antiphospholipidsyndrome, r/ low signalintensityratherthan normalflow void

activatedC protein resistance,pregnancy, onTlWl
peripartumstate,oral contraceptives, (b) chronicthrombosis(when most cases are
polycythemia vera, idiopathic thrombocytosis, diagnosed)
thrombocytopenia, sicklecell disease, ./ hyperintense thrombuswithinsinuson TlWl
cryofibrinogenemia, d isseminated intravascular (dueto intra-and extracellular methemoglobin)
coagulopathy r/ iso-/ hyperintense thrombuson T2Wl (dueto
(e) Chemotherapy:eg, ARA-C,asparaginase extracellularmethemog lobin)
D. Unusualcauses: N.B.: hypointense thrombuson T2Wl (dueto
Behgetdisease.AIDS, ulcerativecolitis,systemic intracellularmethemoglobin) may mimic
lupuserythematosus,nephroticsyndrome flow void of a Patentdural sinus
r/ subcorticalhemorrhagicinfarcts(dueto retrograde
Associatedwith: CHD, antithrombinlll deficiency, extensionof thrombus)
proteinC resistance,proteinS disease, { wall-enhancement of thrombosedduralsinus
antiphospholipidsyndrome MR venography:
Pathophysiology: { absenceof flow-relatedenhancement
duralsinusthrombosisleadsto venouscongestion, Pitfall: hyperintense thrombuson TlWl time-of-
brain edema,sulcaleffacement,occasionally flightvenographycan simulateflow-related
hydrocephalus(due to decreasedCSF absorption) enhancement
. symptomsof increasedintracranialpressure: Angio:
headaches,nausea,vomiting,visualblurring { nonfitlingof thrombosedsinus
oftenconfusedwith: tensionheadaches,migraine r/ titlingof corticalveins,deep venoussystem,
. drowsiness,decreasedmentation,lethargy,obtundation cavernoussinus
. stroke symptomatology,seizures ^/ parasagittalhemorrhages(highlyspecificfor superior
. fever sagittalsinusthrombosis)secondaryto cortical
venousinfarction
Location: superiorsagittal> transverse> sigmoid> Prognosis; highmortalitY
straightsinus Rx: heparin(full recoverYin 70%)
NCCT (usuallysubtlefindings):
r/ hyperattenuating material(clottedblood)in sagittal SYNDROME
DYKE.DAVIDOFF.MASON
"cord = INFANTILE/ CONGENITAL
sinus/ straightsinus/ cerebralcorticalveins = = CEREBRAL HEMIATROPHY
sign"(20%) HEMIPLEGIA = SYNDROME OF HEMICONVULSIONS,
r/ compressionof lateralventriclesin 32/" (infarctioni A N, DE P ILE P S Y
H E MIP LE GIA
edema) = unilateralcerebralatrophywith ipsilateralsmall skull
r/ unilateral(213)/ bilateral(1/3)parenchymal Cause: insultto immaturebrainresultingin neuronal
hemorrhageinvolvinggray + white matter(20%) loss+ imPairedbraingroMh:
rl subduralcollection (a) prenatal: congenitalmalformation,infection,
{ strokp(oftenhemorrhagic) vascularinsult
CECT venography(30-40 sec delaY): (b) peflnata!: birthtrauma,anoxia,hypoxia,
./ "deltasign"/ "emptytriangle"= fillingdefectin straight intracranialhemorrhage
sinus/ superiorsagittalsinuswith outwardbowingof (c) postnatal:trauma,tumor,infection,
sinuswall (in 25-70%) prolongedfebrileseizures
Falsepositive: subduralhematoma/ empyema, Age: presentsin adolescence
. seizures
arachnoidgranulations
. hemiparesis (typicallyspastichemiplegia)
Falsenegative: partialvolumeaveraging,small
. mentalretardation
thrombus,recanalizedthrombus
r/ enlargementof thrombosedvein near obstruction r/ unilateralthickeningof skull
r/ shaggyirregularcontourof veins (= smallcollateral { unilateraldecreasein size of cranialfossa
veins enhancenear the obstructedvein) r/ unilateraloverdevelopment of sinuses
r/ gyral enhancementin peripheryof infarction(30- ! contraction of a hemisphere / lobe
40%) { compensatoryenlargementof adjacentventricle+ sulci
r/ intensetentorialenhancementsecondaryto with midlineshift
collaterals(rare)
r/ densetranscorticalmedullaryvein
Advantagesover MRI: shorter exam time DYSEMBRYOPLASTIC NEU ROEPITHELIAL
MR: TUMOR
r/ replacementof flow void by abnormalsignalintensity = benigntumorof neuroepithelial originarisingfrom
(a) acute thrombosis(firstfew days) cortical/ deep graYmatter
r/ clot isointenseto gray matteron TlWl (and Origin: derivedfrom secondarygerminallayers;
thereforeeasilymissed)+ hypointenseon T2Wl originallydiagnosedas low-gradeastrocytomas
 BrainDisorders 277

Histo: specificglioneuronal elementin a columnar EMPYEMA OF BRAIN

patternorientedperpendicularto corticalsurtace;
SubduralEmpyema
admixtureof astrocytes+ oligodendroglial 20/" of all intracranialbacterialinfections
elementsin association with ,,floating
neurons"and Cause: paranasalsinusitis,otitismedia,calvarial
mucinousdegeneration; +n'1u;linodular
architecture osteomyelitis,infectionaftercraniotomyor
Age: usually<20 years; M > F
. medicallyrefractorypartialseizures ventricularshuntplacement,penetrating
. neurologicdeficitsrare wound,contamination of meningitis-induced
subduraleffusion
Location: temporal(62%)/ frontal(31%) lobe;caudate
Location: frontal+ inferiorcranialspace in close
nucl e u sc; e re b e l l u mp;o n s proximityto paranasalsinuses;80% over
CT:
convexityextendingintointerhemispheric
r/ hypoattenuating mass + calcifications fissureor posteriorfossa
d remodelingof innertable of skulf
d nypo-/ isodensecrescentic/ lentiformzoneadjacent
MR:
to innertable
! corticalmasswithoutsurrounding vasogenicedema: r/ may show mass effect(sulcaleffacement,ventricular
r/ hypointenseon Tl Wl + hyperintenseon T2Wl
compression, shift)
r/ "soapbubble"/ megagyrusappearanceat cortical ^/ tnin curvilinearrim of enhancement(7-10 days later)
=
, margin enlargementof corticalsurface adjacentto brain
! contrastenhancement(in 33%) r/ severesinusitis/ mastoiditis(may be most significant
Prognosis; partial resectionstops seizure activity;rarely
indicator)
recur Mortality: 30% (neurosurgical emergency)
DDx: diffuseastrocytoma,ganglioglioma,
Cx: venousthrombosis,infarction,seizures,
oligodendroglioma
hemiparesis, hemianopia, aphasia,brainabscess
DDx: subacutei chronicsubduralhematoma
EMPTY SELLA SYNDROME
= extensionof subarachnoidspace into sellaturcica, EpiduralEmpyema
which becomesexposedto CSF pulsationssecondary Cause: same as above
to defectin diaphragmasellae;characterizedby normal . no neurologicdeficits(duraminimizespressure
/ moldedpituitarygland+ normalor enlargedsella exertedon brain)
(emptysella= misnomer) r/ tnict enhancingrim
lncidence: 24Yoin autopsy study

A. PRIMARYEMPTYSELLA(anatomicspectrum) ENCEPHALITIS
lncidence: 1loh of adultpopulation;M:F = 1:4 = term generallyreservedfor diffuseinflammatoryprocess
Probable causes: of viral etiology,most commonlyarthropod-borne
(1) pituitaryenlargement followedby regression arboviruses(Eastern+ Westernequineencephalitis,
duringpregnancy Californiavirusencephalitis, St. Louisencephalitis)
(2) involutionof a pituitarytumor r/ diffusemild cerebraledema
(3) congenitalweaknessof diaphragmasellae { smallinfarctions / hemorrhage(lessfrequent)
0 Occursmore frequentlyin patientswith increased r/ hyperintensity on T2Wl in areas of corticalinvolvement
intracranialpressure
. usuallyasymptomatic HerpesSimplexEncephalitis
(HSE)
. increasedriskfor CSF rhinorrhea = rT'lostcommoncause of nonepidemicnecrotizing
. NO endocrineabnormalities meningoencephalitis in immunocompetent individuals
B. SECONDARYEMPTYSELLA in USA
= postsurgical when diaphragmasellaehas been 0 Neurologicemergencydue to high morbidity+ mortality
disrupted Organism; HSV type | (in adults);HSV type il (in
. visualdisturbance neonatesfrom transplacentalinfection)
. headaches . precedingviralsyndrome
. mentalstatuschanges:confusion,disorientation,
r/ slowlyprogressivesymmetricallasymmetrical(double hallucination,personality change,aphasia
. low-gradefever, headache,seizures
, floor)enlargement of sella
r/ remodeledlaminadura remainsmineralized Location: inferomedialtemporal> frontal> parietal
{ small rim of pituitarytissuedisplacedposteriorly lobes;propensityfor limbicsystem
+ inferiorly (olfactorytract,temporallobes,cingulate
{ infundibulum sign = infundibulum extendsto floorof gyrus,insularcortex);initiallypredominantly
sella unilateral
DDx: cystictumor,large herniated3rd ventricle r/ mild patchyperipheralI gyral/ cisternalenhancement
(displacedinfundibulum) (50%),may persistfor severalmonths
278 Radiology Review Manual
CT:
^/ may be negativein first 3 days
{ poorlydefinedbilateralareasof mildlydecreased
attenuationin one / bothtemporallobes+ insulae
ri sparedputamenforms sharplydefinedconcave/
straightborder(DDx: infarction, glioma)
r/ mild mass effectwith compressionof lateral
ventricles+ loss of sylvianfissure(brainedema)
./ tendencyfor hemorrhage+ rapiddissemination in
brain
MR (studyof choice,positivewithin2 days):
./ increasedsignalintensityon T2Wl + mildto
moderatehypointensity on TlWl
r/ increasedsignalon diffusion-weighted images
(cytotoxicedema)
r/ smallfoci of hemorrhage(common)
NUC:
Agents: standardbrain imaging(eg, Tc-99m DTPA),
newerbrainagents(eg, l-123
iodoamphetamine / Tc-99m HMPAO)
SPECTimagingimprovessensitivity
{ characteristic focal increasein activityin temporal
lobeson brainscintigraphy (blood-brain barrier
breakdown)
Dx: (1) identification of viruswithinCSF (using
polymerasechain reactiontechnique)
(2) fluoresceinantibodystaining/ viralculturefrom
brain biopsy
Mortality: 3O-70%
Rx: adeninearabinoside
DDx: (1) Infarction(involveseithermedialor lateral
temporallobe, almostexclusivelyunilaterat)
(2) Low-gradeglioma
(3) Abscess
VirusEncephalitis
Humanlmmunodeficiency
oftenin combination with CMV encephalitis
Histo: microglialnodules+ perivascularmultinucleated
giantcellsaccompanying gliosisof deepwhite
+ gray matter
r/ predominantlycentralCNS atrophy
/ diffusewhite matter
r/ symmetricperiventricular
diseasewithoutmass effect(hypodenseon CT, high
intensityon T2Wl)
itis
Encephal
Postinfectious
followingexanthematous viralillness(measles,mumps,
rubella,smallpox,chickenpox,, Epstein-Barr virus,
varicella,pertussis)/ vaccination
Acute DisseminatedEncephalomyelitis
(ADEM)
= POSTVIRAL LEUKOENCEPHALOPATHY
= autoimmunereactionagainstpatient'swhite matter
. 7-14 days / severalweeks followingan
exanthematousviral infection/ vaccination
. confusion,headaches,fever
. seizures,focalneurologicdeficits
Histo: diffuseperivenousinflammatoryprocess
resultingin areasof demyelination
Location: subcorticalwhite matterof both
hemispheresasymmetricatlY
CT:
r/ multifocalhypodensewhite matterabnormalities
! sparingof corticalgray matter,occasionallydeep
gray matterinvolvement
ri no additionallesionson follow-upexam
MR:
r/ focal areas of hyperintensityon T2Wl
r/ may demonstratecontrastenhancement
Rx: corticosteroids resultin dramaticimprovement
Prognosis; completeresolutionof neurologicdeficits
within 1 month (80-90%)/ some
permanentneurologicdamage (10-20%)
DDx: simulatingmultiplesclerosis(rarelyrecurrent
episodesas in multiplesclerosis)
AcuteHemorrhagic Leukoencephalitis
= fulminantmyelinoclastic diseaseof CNS
= hyperacuteform of acutedisseminated
encephalomyelitis
Cause: immunoreactive diseasefollowing
prodromalillness(minorupperrespiratory
viral infection,ulcerativecolitis)
Path: markededema,brain softening
Histo: necrotizing angiitisof venules+ capillaries
withinwhite matterwith extravasationof
PMNs+ lymphocytes; fibrinoidnecrosisol
affectedcapillaries+ surrourldingtissues;
confluenthemorrhages with ball-and-ring
configuration due to diapedesisof RBCs
. progressivecoma, motordisturbance,speech
seizures
difficulty,
. pyrexia,leukocytosis
. pleocytosis, elevatedproteinin spinalfluid
Location: unilateraldisease;parietal+ posterior
frontalwhite matterat levelof centrum
semiovale(sparingsubcorticalU-fibers
+ cortex)> basalganglia,cerebellum,
brainstem,sPinalcord
rl rapiddevelopmentof profoundmass effect
resemblinginfarction
r/ multiplepunctatewhite matterhemorrhages
{ extensivehypoattenuation virtuallyconfinedto
hemispheric whitematter
Prognosis: usuallyresultsin death
DDx: (1) Herpessimplexencephalitis (cortical
lesionsin temporal+ inferiorfrontallobes
+ insularregion,no imagingfindingsuntil
3-5 daysafteronsetof significantsymptoms)
(2) Tumefactivemultiplesclerosis
(3) OsmoticdemYelination
(4) Toxicencephalopathy: lipophilicsolvent,
methanol
(5) Hypertensiveencephalopathy:eclampsia,
thromboticthrombocytopenic purpura
 BrainDisorders 279

EPENDYMOMA Rx: surgery(difficuftto resectdue to adherenceto

= in majoritybenignslow-growing neoplasmof mature surroundingbrain)+ radiation(partially
well-differentiated ependymalcellsliningthe ventricles radiosensitive)+ chemotherapy
lncidence; most commonlyin children;5-g% of all DDx of cerebellar ependymoma:
primaryCNS neoplasms;15/" of posterior (1) Astrocytoma(hypodense, displaces4th ventricle
fossatumorsin children;63/" of spinal from midline,cysticlucency,intramedullary)
intramedul larygliomas (2) Medulloblastoma (hyperdense, calcifications
in only
Histo: benignaggregatesof ependymocytesin form of 10%)
perivascularpseudorosettes; may have papillary (3) Trapped4th ventricle(no contrastenhancement)
pattern(difficultDDx from choroidplexuspapilloma)
Age: (a) supratentorial:at any age (atrium/ foramenof
Monro) EPIDERMOID
OF CNS
(b) posteriorfossa:<10 years;age peaksat 5 and = E P TD E R MOTD C ]Y S T
IN C LU S ION
34 y ea rs ; M :F = 0 .8 :1 = benigntumorwith extremelyslow lineargrowthresulting
Associatedwith: neurofibromatosis from desquamation of epithelialcellsfrom tumorwall
. increasedintracranialpressure(g0%) lncidence: 0.2-1.8/. of all primaryintracranial
neoplasms;most commoncongenital
Location: intracranial tumor
(a) infratentorial:
floorof 4th ventricle(70%of all Etiology: inclusionof ectodermalepithelialtissuefrom
intracranial
ependymomas) pharyngealpouchof Rathkei pluripotential cells
(b) supratentorial:frontal> parietal> temporoparietal duringclosureof neuraltube in Sthweek of fetal
juxtaventricularregion(uncommonly intraventricular), life (earlyinclusionresultsin midlinelesion,later
lateralventricle,3rd ventricle inclusionresultsin more laterallocation)
(c) conus(40-65%of all spinalintramedullary gliomas) Path: "pearlytumor"= well-defined solidlesionwith
in children: infratentorialin 70o/",supratentorialin 30"/o glisteningirregularnodularsurface;soft flaky
desquamatedkeratinaceousdebrisrich in
r/ smallcysticareasin 15-50% (centralnecrosis) cholesterol+ triglycerides = pRtMARy /
./ fine punctatemultifocalcalcifications(25-SO%) CONGENITALCHOLESTEATOMA
i/ intratumoral hemorrhage(10%) Histo: tumorlinedby simplestratifiedcuboidalsquamous
./ frequentlygrows into biain parenchymaextendingto epithelium; surroundedby thin bandof
corticalsurface(particularlyin frontal+ parietallobes) collagenous connectivetissue;tumorcenterof
^/ may invaginateinto ventricles lamellarappearancedue to desquamation
r/ expansionfrequentlythroughforamenof Luschkainto Age: 10-60 years,peak age in 4th-5th decade;tumor
cerebellopontine angle(15%)or throughforamenof slowlyexpandsoverdecadesby continued
Magendiecaudadintocisternamagna(up to 60%) desquamation of the liningthus becoming
( c HA RA CT E R tS T tC ) symptomatiicn adul thood;M:F = 1:1
r/ directinvasionof brainstem/ cerebellum(30-40%) . facialpain
r/ insinuation aroundbloodvessels+ cranialnerves . cranialnervepalsiesfrom CP angleepidermoids(50%)
r/ communicating hydrocephalus (100%)secondaryto . hydrocephalus in suprasellar epidermoids
proteinexudateelaboratedby tumor cloggingresorption . chemicalmeningitis(secondary to leakageof tumor
pathways contentsinto subarachnoidspace)in middlecranial
CT: fossaepidermoids
{ sharplymarginatedmultilobulated iso-/ slightly
hyperdense4th ventricularmass Site: midlinei paramidline; intradural(90%)/ extradural;
^/ tnln well-defined low-attenuationhalo (distended transspatialgrowth(= extensionfrom one into
effaced4th ventricle) anotherintracranial space)
r/ heterogeneous / moderatelyuniformenhancement of Location: (a) cerebellopontine angle(40o/",
accountfor
solidportions(80%) 5/" of C P angl etumors)
MR: (b) suprasellarregion,perimesencephalic
^/ low to intermediate heterogeneous signalintensityon cisterns(14%)
TlWI (c) in ventricles,brainstem,brainparenchyma
r/ hypointense tumormarginson Tl Wl + T2WI in 64/" (d) skullvault
(hemosiderin deposits) { soft lesionconformingto + moldingitselfaroundbrain
r/ foci of high-signalintensityon T2Wl (= necroticareas surfaces
/ cysts)+ low signalintensity(= calcification/ r/ may intimatelysurroundvessels+ cranialnervesrather
, hemorrhage) than displacingthem (limitedresectability)
d fluid-fluidlevelwithincysts ! littlemass effect,no edema/ hydrocephalus
r/ homogeneous Gd-DTPAenhancement of tumor ./ trlOcontrastenhancement
Cx: subarachnoid dissemination via CSF (rare)(DDx: r/ may be associatedwith dermalsinustract at occipital/
malignantependymoma, ependymoblastoma) nasofrontal regionif midlinein location
280 Radiology Review Manual
CT:
{ typicallylobulatedroundhomogeneousmasswith
densitysimilarto CSF (betweenwaterand -20 HU)
{ occasionattyhyperdensedue to high proteincontent,
saponification of keratinaceous debris,prior
hemorrhage into cyst,ferrocalcium i iron-containing
pigment,abundanceof PMNs
{ bony erosionwith sharplydefinedwell-corticated
margins
r/ calcification(25%)
r/ peripheralenhancement(perilesionalinflammation)
MR:
{ lamellatedonionskinappearancewith septations
(layer-on-layer accretionof desquamatedmaterial)
r/ "blackepidermoid" = signalintensitysimilarto CSF:
heterogeneously hypointense lesionon Tl Wl
+ hyperintense on T2Wl (dueto cholesterolin solid
crystallinestate+ keratinwithintumor+ CSF within
tumorinterstices)
{ "whiteepidermoid" (rare)= hyperintense on Tl Wl
+ isointenseon T2Wl due to presenceof triglycerides
+ polyunsaturated fatty acids
! hypointense on T2Wl (veryrare)due to calcification,
low hydration,viscoussecretion,paramagnetic iron-
containingpigment
A ngio:
r/ avascular
Cisternography:
r/ papillary/ frondlikesurfacewith contrastmaterial
extendingintotumorinterstices
'
Rx: surgicalresection(complicatedby adherenceto
surroundingbrain+ cranialnerves,spillageof cyst
contentswith chemicalmeningitis, CSF seeding
+ implantation)
DDx: arachnoidcyst (smoothsurface,earlierdiffusion),
cysticschwannoma,adenomatoid tumor,atypical
meningioma, chondroma,chondrosarcoma,
chordoma,calcifiedneurogenic tumor,teratoma,
calcified astrocytoma,ganglioglioma
EP I DURA L HE M AT OMA O F BR AIN
= EXTRADURAL HEMATOMA
= hematomawithinpotentialspace betweennaked inner
tableof skull+ calvarialperiosteum(innerdura layer),
which is bounddown firmlyto craniumat sutural
margins(= subperiosteal hematomaof innertable)
lncidence: 2o/oof all serioushead injuries;in <1% of all
childrenwith cranialtrauma;uncommonin
infants
Cause: impacton skull causes lacerationof periosteal
layerof outertable + linearfracture;temporary
inwarddisplacementof fragmentslacerates
meningealvesselsand stripsbothdurallayers
from innertablewhilethe innerlayer(meningeal
dura) remainsintact;blood accumulatesbetween
nakedinnertableand dura
Age: more commonin youngerpatients2040 years
(dura more easilystrippedaway from skull)
Associated with:
(1) skullfracturein 75-85 -95% (bestdemonstratedon
skullradiographs)
0 Skullfracturesfrequentlynot visiblein children
("ping-pong !
f racture")
(2) subduralhemorrhage
(3) contusion
Source of bleeding:
(a) lacerationof (middle)meningealartery(high
pressure)i meningealvein(lowpressure)adjacentto
innertablefrom calvarialfracture(91%)
(b) disruptionof duralvenoussinuses(transverse /
superiorsagittalsinus)with low pressure+ highflow
due to diastaticfractureof lambdoid/ coronalsuture
[majorcausein youngerchildren]
(c) avulsionof diploicveins/ marrowsinusoidsat points
of calvarialperforations
Time of presentation: within first few days of injury
(80%), 4-21 days (20%)
' transientlossof consciousness (= briefperiodof
unconsciousness from concussionof brainstem)
. "lucidinterval"(in <33%)
. delayedsomnolence(24-96 hoursafter accident)due
to accumulation of epiduralhematoma:
0 DANGEROUSbecauseof focal mass effect+ rapid
onset(neurosurgical emergencyunlesssmall)!
. progressive deterioration of consciousness to coma
. focalneurologicsigns: 3rd nervepalsy(signof cerebral
herniation),hemiparesis
0 Mostcommonlyclinicallysignificantif locatedin
temporoparietal region!
0 Only a minorityof skullfracturesacrossthe middle
meningealarterygrooveresultin epiduralhematomasl
Types:
I acute epiduralhematoma (58%)from arterial
bleeding
ll subacutehematoma (31%)
l l l chroni chematoma (11%)from venous
bleeding
Factorsdetermining the rateof epiduralexpansion:
injuryto arteryor vein,spasmof artery,containment
of bleedthroughpseudoaneurysm or tamponade,
decompression of hematomaintomeningeal+ diploic
veins or throughfractureinto scalp
Location:
(a) in 66% temporoparietal(mostoftenfrom laceration
of middlemeningealarterY)
(b) in 29"/"alfrontal pole, parieto-occpitalregion,
betweenoccipitallobes,posteriorfossa (mostoften
from lacerationof duralsinusesby fracture)
0 NO crossingof suturesunlessdiastaticfractureol
suturepresentl
C T:
r/ fractureline in area of epiduralhematoma
r/ expandingbiconvex(lenticular = elliptical)
extra-axial
fluidcollection(mostfrequent)= underhighpressure:
./ usuallydoes not cross suturelines

r/ separationof venoussinuses/ falx from innertable
of skull
0 The ONLY hemorrhagedisplacingfalx/ venous
sinusesawayfrom innertable!
r/ hematomausuallyhomogeneous:
^/ fresh extravasatedblood (30-50 HU) / coagulated
blood (50-80 HU) in acutestage
r/ rarelywith hypoattenuated "swirl"(due
to admixture
of fresh blood into clottedblood in activebleeding)
r/ mass effect("compressioncone effect")with
effacementof gyri + sulcifrom:
- epiduralhematoma(57%)
- hemorrhagiccontusion(29%)
- cerebraledematousswelling(a%)
r/ markedstretchingof vessels
r/ signs of arterialinjury(rare): contrastextravasation,
arteriovenous fistula,middlemeningealartery
occlusion,formationof pseudoaneurysm
MR:
r/ low intensityof fibrousdura materallows
differentiationof epiduralfrom subduralbloodin the
latesubacutephase(extracellular methemoglobin)
with hyperintensity on TlWl + T2Wl
Angio:
r/ meningealarteriesdisplacedawayfrom innertableof
s k ull
r/ pseudoaneurysm= extravasationof contrastmaterial
r/ arteriovenousfistulaat fractureline
Cx: herniation,coma,death(15-30%)
Rx: after surgicalevacuationreturnof ventricular
systemto midline
0 Epiduralhematomaat anothersite may be
unmaskedfollowingsurgicaldecompression!
DDx: Chronicsubduralhematoma(may havesimilar
biconvexshape,crossessuturelines,stopsat falx,
no associatedskullfracture,no displaceddura on
MRr)
GANGLION
CELLTUMOR
Gangliocytoma
= r?fe benigntumorcomposedof matureganglioncells
Prevalence: 0.1-0.5% of all braintumors
Age: children+ youngadults
Associatedwith: dysplastic+ malformedbrain
Cause: ? dysplasticbrain
Histo: purelyneuronaltumorcomposedof abnormal
matureganglioncellswithoutneoplasticglial
cells(= no immunoreactivity for glialfibrillary
acidicprotein)
Location: floorof 3rd ventricle> temporallobe>
cerebellum> parieto-occiptal region>
frontallobe > spinalcord
CT :
r/ hyperattenuating mass with littlemass effect
MR:
r/ iso-to hypointense on TlWl + T2Wl
^/ Origtrton protondensityimages
BrainDisorders 281
DysplasticCerebellarGangliocytoma
= LHERMITTE-DUCLOS DISEASE
Age: young adults;averageage of 34 years
Associatedwith: polydactyly,partialgigantism,
leontiasisossea,vascular
malformation
Strong associationwith: Cowden disease
(= autosomaldominanthamartomasyndrome
characterizedby mucocutaneous lesions,
macrocephaly, hamartomasand neoplasiaof
breast,thyroid,colon,genitourinaryorgans,CNS)
Path: disruptionof normalcerebellarlaminar
structure
Histo: hypertrophicganglioncells expanding
granularand molecularlayersof cerebellar
cortex+ abnormallyincreasedmyelination in
molecularlayers;markedreductionin
myelinationof centralwhite matterof
cerebellarfolia
. symptomsof increasedintracranialpressure
. slowlyprogressivecerebellarsyndrome (40%)
. megalencephaly (50%);mentalretardation
CT:
r/ nypo-/ isoattenuatinglesion
r/ calcification
uncommon
r/ thinningof skull
MR:
r/ striatedcerebellumsign = laminated/ lamellar
massof alternatingbandsof high + normalsignal
intensityon T2Wl
Rx: decompresionof ventricles+ resectionof mass
Ganglioglioma
= Uncofi]rnon slow-growing essentiallybenigntumor
composedof glial+ neuronalelements
Prevalence: 0.4-09.% of all intracranialneoplasms;
1-4% of all pediatricCNS neoplasms
Peak age: 10-20 years;in 80% <30 years of age;
M>F
Histo: containganglion+ glialelements:ganglioncells
(neurons)arisefrom primitiveneuroblasts
+ matureduringgrowth;usuallyastrocyticglial
cellspredominatein variousstagesof
neoplasticdifferentiation
. headaches
. medicallyrefractoryseizures:
0 Mostcommoncauseof chronictemporallobe
epilepsy
Location:frequentlyabovetentorium:in peripheryof
cerebralhemisphere[temporal(JB%)I
parietal(30%)/ frontal(18%) lobesl;
brainstem;cerebellum; pinealregion;spinal
cord; optic nerve;optic chiasm;ventricles;
localinvolvement of subarachnoid space
{ circumscribed slow-growing mass:
r/ solid(43%)/ cystic(5%) lsolid-cystic combination
(52/")
i/ calcifications(30%)
^/ tinteassociatedmass effect/ vasogenicedema
282 Radiology Review Manual

CT: Path: multilobulated appearance;quite extensive

r/ hypoattenuating (38%)/ mixedattenuation(32%)I vasogenicedema(transudation throughstructurally
isoattenuating (15%)mass
(15%) I hyperattenuating abnormaltumorvascularchannels);deeply
r/ + remodeling of skull infiltratingneoplasm;hemorrhage; necrosisis
r/ contrastenhancement(16-80%) essentialfor pathologicdiagnosis(HALLMARK)
0 Occasionallycompletelyundetectableby CT Histo: highlycellular,often bizarrelypleomorphic/
MR: undifferentiated multipolarastrocytes; common
ri variable(hypo-/ isointense)nonspecific MR mitoses+ prominentvascularendothelial
appearanceon TlWl proliferation;no capsule;pseudopalisading
{ commonlyat leastone hyperintense regionon (= viableneoplasticcellsformingan irregular
T2WI borderaroundnecroticdebrisas the tumor
r/ nonenhancing / ringlikei homogeneouslyintense outgrowsits btoodsuPPlY)
enhancement Subtypes:
Prognosis; favorable;malignantdegeneration(6%) (a) giantcell GBM = monstrocellular sarcoma
Rx: gross total resection(withresolutionof seizure (b) smallcell GBM = gliosarcorrl? = Feigintumor
activityin majorityof patients) Location:
(a) hemispheric: whitematterof centrumsemiovale:
Desmoplasticlnfantile Ganglioglioma frontal> temporallobes;commonin pons,thalamus,
= DESMOPLASTIC INFANTILE ASTROCYTOMA quadrigeminal region;relativesparingof basal
= SUPERFICIAL CEREBRAL ASTROCYTOMA ATTACHED ganglia+ gray matter
TO DURA DDx: solitarymetastasis,tumefactivedemyelinating
= uflcorTilTronvarietyof gangliogliomaexclusivelyin lesion("singular atypicalabscess
sclerosis"),
infants (b) callosal:"butterflyglioma"may grow exophytically
Age: <18 months(vastmajority);M:F = 2:1 intoventricle
Histo: spindlecell neoplasmwith oval / elongated (c) pg€IftI-fossa: pilocyticastrocytoma,brainstem
moderatelypleomorphicnuclei+ clustersof astrocytoma
largercellswith largeprominenteccentric (d) extraaxial: primaryleptomeningeal glioblastomatosis
nucleiand cytoplasmcontainingNisslbodies (e) multifocal:in 2-5%
. rapidlyincreasingheadcircumference Spread:
. seizureactivity(uncommon) (a) directextensionalongwhite mattertracts:
Location: frontal+ parietal> temporal> occipital corpuscallosum(36%),coronaradiata,cerebral
lobes peduncles,anteriorcommissure, arcuatefibers
rl exceptionallylarge heterogenousmass: "butterfly"
r/ readilycrossesmidline= glioma
{ slightlyhyperattenuating solidportiontypically (clue: invasionof septumpellucidum)
locatedalong corticalmargin r/ frontal+ temporalgliomastend to invadebasal
r/ cysticcomponents gangl i a
r/ intenseenhancementof solidcomponent ./ may invadepia,arachnoidand dura (mimicking
{ CHARACTERISTIC extensionof enhancement to meni ngi oma)
leptomeningeal margin(dueto firm duralattachment) (b) subependymalcarpetafter reachingthe surfaceof
^/ rare vasogenicedema the ventricles
r/ trtOcalcification (c) via CSF (<2%)
Prognosis; good (d) hematogenous(extremelyrare)
Rx: surgicalresection r/ osteoblasticbone lesion
N E C T:
Ganglioneuroma ./ inhomogeneous low-densitymasswith irregular
= ganglioncells predominateover glial cells shape+ poorlydefinedmargins(hypodensesolid
tumor/ cavitarynecrosis/ tumor cyst / peritumoral
"fingersof edema")
GLIOBLASTOMA MULTIFORME
Most malignantform of all gliomas/ astrocytomas; end r/ considerablemass effect:compression
stage of progressivesevereanaplasiaof preexisting + displacement cisterns,brain
of ventricles,
Gradel/ ll astrocytoma (notfrom embryologic glioblasts) parenchyma
lncidence: most commonprimarybraintumor; 50% of ./ iso- / hyperdenseportions(hemorrhage)in 5o/o
tumors;1-2% of all
all intracranial ./ rarelycalcifies(if coexistentwith lower-gradeglioma/
malignancies; 20,000casesper year after radio-or chemotheraPY)
Age: all ages; peak incidenceat 65-75 years; C E C T:
M:F = 3:2; morefrequentlyin whites Enhancement pattern:contrastenhancement due to
Genetics: Turcotsyndrome,neurofibromatosis type 1, breakdownof blood-brainbarrier/ neovascularity /
Li-Fraumeni syndrome(familialneoplasmsin areas of necrosis
variousorgansbasedon abnormalp53 tumor- (a) diffusehomogeneous enhancement
suppressorgene) (b) heterogeneous enhancement
 BrainDisorders 283

(c) ringpattern(occasionally enhancingmasswithin Oligodendroglioma 6%

t he r ing) Spongioblastomapolare 3%
(d) low-densitylesionwith contrastJluidlevel (leakage Mixedgliomas 3%
of contrast) Astroblastoma 2/"
{ almostalwaysringblushof variablethickness:
multiscalloped ("garland"),
round/ ovoid;may be seen Age peak; middleadult life
surrounding ventricles(subependymal spread);tumor Location: cerebralhemispheres;spinalcord;
, usuafly extends beyond marginsof enhancement brainstem+ cerebellum(in children)
{ sedimentation levelsecondaryto cellulardebris/
hemorrhage/ accumulatedcontrastmaterialin BrainstemGlioma
tumoralcyst lncidence: 1"/";12-15%of all pediatricbraintumors;
MR: 20-30% of infratentorialbrain tumorsin
^/ poorlydefinedlesionwith some mass effect/ children
vasogenicedema/ heterogeneity Histo: usuallyanaplasticastrocytoma/ glioblastoma
hemosiderin deposits(gradientecho images) multiformewith infiltration
alongfibertracts
hemorrhage(hypointensity on T2Wl and T2.-Wl) Age: in children+ youngadults;peakage g-19 years;
TlWl + gadolinium-DTPA enhancement separate M : F= 1 : 1
tumornodulesfrom surrounding edema,central . becomeclinicallyapparentearlybeforeventricular
necrosisand cyst formation obstructionoccurs
A ngio: . ipsilateral progressive multiplecranialnervepalsies
r/ wildlyirregularneovascularity+ earlydrainingveins . contralateralhemiparesis
{ avascularlesion . cerebellardysfunction:ataxia,nystagmus
PE T : . eventuallyrespiratory insufficiency
r/ increasein glucoseutilizationrate Location: pons > midbrain> medulla;oftenunilateral
Rx: surgery+ radiationtherapy+ chemotherapy at medullopontine junction
Prognosis; 16-18 monthspostoperative survival(frequent 0 Medullary+ mesencephalic gliomasare
tumorrecurrencedue to uncertainty during morebenignthan pontinegliomasl
surgeryabouttumormargins) Growthpattern:
(a) diffuseinfiltration
of brainstemwith symmetric
Multifocal GBM expansion+ rostrocaudal spreadintomedulla/
(1) Spreadof primaryGBM thalamus+ spreadto cerebellum
(2) Multipleareasof malignantdegeneration in diffuse (b) focallyexophyticgrowthinto adjacentcisterns
low-gradeastrocytoma("gliomatosis
cerebri',) (cerebel l oponti ne,preponti ne,ci ste r nam agna)
(3) lnherited/ acquiredgeneticabnormality ,
r/ asymmetricallyexpandedbrainstem
r/ flattening+ posteriordisplacementof 4th ventricle
+ aqueductof Sylvius
GL IO M A r/ compression of prepontine+ interpeduncular cistern
= malign?nttumorsof glialcellsgrowingalongwhite (in upwardtranstentorial herniation)
mattertracts,tendencyto increasein gradewith time; r/ paradoxicalwideningof CP anglecisternwith tumor
may be multifocal extensionintoCP angle
lncidence; 30-40% of all primaryintracranialtumors; r/ paradoxicalanteriordisplacementof 4th ventriclewith
50% of solitarysupratentorialmasses tumorextensionintocisternamagna
r/ contrastenhancement: CT:
0 Increasesin proportionto degreeof anaplasia r/ isodense/ hypodensemasswith indistinctmargins
0 Diminishedintensityof enhancement with steroid rl hyperdensefoci (= hemorrhage) uncommon
therapy r/ absent/ minimal/ patchycontrastenhancement
(50%)
CE LLO F O RI G I N { ringenhancementin necrotic/ cystictumors(most
1. Astrocyte Astrocytoma aggressive)
2. Oligodendrocyte Oligodendrogfioma r/ prominentenhancementin exophyticlesion
3. E pendy m a ......Ep e n d y m o m a ri hydrocephalusuncommon(becauseof early
4. M edu||ob|a s...................
t Me d u l l o b l a s to ma; symptomatology)
(PNET= primitive MR: (betterevaluationin subtlecases)
neuroectodermaltumor) ri hypointenseon Tl Wl + hyperintenseon T2Wl
5. Choroidplexus Choroidplexuspapilloma ! oftenonlysubtleenhancement
^/ + engulfmentof basilarartery
FRE Q UE NCY
O F IN T R AC R A N IAGLL IOMA S A ngi o:
Glioblastomamultiforme 51% d anteriordisplacementof basilar arLery+ anterior
Astrocytoma 25% pontomesencephalic vein
Ependymoma 6% r/ posteriordisplacementof precentralcerebellarvein
284 Radiology Review Manual

r/ posteriordisplacementof posteriormedullary . slowingof voluntarymovements,dysarthria

segmentsof PICA
+ supratonsillar . choreoathetotic movementdisorder
r/ lateraldisplacementof lateralmedullarysegmentof . progressive dementia
P I CA CT:
Prognosis; 10-30% S-yearsurvival rate ^/ low- (= tissuedestruction)/ high-density(= dystrophic
Rx: radiationtherapy calcification)foci in globuspallidus
DDx: focal encephalitis,resolvinghematoma,vascular MR:
malformation,tuberculoma, infarct,multiple { "eye-of-the-tiger" sign:
sclerosis,metastasi,lymphoma ri initiattyhypointenseglobuspaltiduson T2Wl (= iron
deposition)
/ ChiasmaticGlioma
Hypothalamic r/ latercentralhyperintensefoci on T2Wl (= tissue
Pointof originoftenundeterminable: hypothalamic destruction+ gliosis)
gliomasinvadechiasm,chiasmaticgliomasinvade
hypothalamus HAMARTOMA OF CNS
Incidence; 10-15/" of supratentorial tumorsin raretumor
children (a) sporadic
Age: 24years; M:F = 1:1 (b) associatedwith tuberoussclerosis;may degenerate
Associatedwith: von Recklinghausen disease into giant cell astrocytoma
(20-50%) Age: 0-30 years
. diminishedvisualacuity(50%)with optic atrophy Location: temporallobe, hamartomaof tuber cinereum,
. diencephalic syndrome(in up to 20o/o)tmarked subependymal in tuberoussclerosis
emaciation,pallor,unusualalertness,hyperactivity, r/ cyst with littlemass effect,possiblywith focal
euphoria calcifications
. obesechild { usuallyno enhancement
. sexualprecocity
. diabetesinsipidus
r/ obstructivehydrocephalus HEADTRAUMA
{ suprasellarhypodenselobulatedmass with dense = C N S TR A U MA
inhomogeneous enhancement lncidence: 0.2-0.3% annuallyin UnitedStatesare
r/ hypointense on TlWl + hyperintense on T2Wl significant; peak at 550/100,000peopleaged
{ cyst formation,necrosis,catcificationsrenderlesion 15-24 years;secondpeak >50 years of age
inhomogeneous Cause: motorvehicleaccidents(51%),tall (21"h),
DDx: hypothalamichamartoma assaultand violence(12/"),sportsand
recreation(10%)
Classification:
GLOBOIDCELLLEUKODYSTROPHY A. Primarytraumaticlesion
= KRABBE DISEASE (a) primaryneuronalinjurY
Cause: deficiencyof galactosylceramide beta- 1. Corticalcontusion
galactosidaseresultingin cerebroside 2. DiffuseaxonalinjurY
accumulation+ destructionof oligodendrocytes 3. SubcorticalgraYmatterinjurY
Dx: biochemicalassayfrom white blood cells/ skin = injuryto thalamus+ basalganglia
fibroblasts 4. PrimarybrainsteminjurY
Age: 3-6 months (b) primaryhemorrhages(frominjuryto a cerebral
. restlessness+ irritability artery/ vein / capillary)
. markedspasticity 1. Subduralhematoma
. optic atrophy 2. Epiduralhematoma
. hyperacusis 3. Intracerebral hematoma
{ symmetrichyperdenselesionsin thalami,caudate 4. Diffusehemorrhage(intraventricular,
nuclei,coronaradiata subarachnoid)
{ decreasedattenuationof white matter (c) primaryvascularinjuries
{ brain atrophywith enlargementof ventricles 1. Carotid-cavernous fistula
Prognosis; death within first few years of life 2. Arterialpseudoaneurysm
Location: branchesof ACA + MCA, intra-
DISEASE
HALLERVORDEN-SPATZ cavernousportionof lCA, PCom
= r?fe familialneurodegenerativemetabolicdisorderwith 3. Arterialdissection/ laceration/ occlusion
abnormaliron retentionin basalganglia 4. Duralsinuslaceration/ occlusion
Age: 2nd decadeof life (d) traumaticpia-arachnoid injury
Histo: hyperpigmentation + symmetricaldestructionof 1. PosttraumaticarachnoidcYst
globuspallidus+ substantianigra 2. Subduralhygroma
. progressivegait impairment+ rigidityof limbs (e) cranialnerve iniury

B. Secondarytraumaticlesion
. deteriorationof consciousness/ new neurologic
signssome time afterinitialinjury
1. Majorterritorialarterialinfarction
Cause: prolongedtranstentorial / subfalcine
herniationpinchingthe arteryagainsta
rigidduralmargin
Location: PCA,ACA territory
2 . Boundary+ terminalzone infarction
3 . Diffusehypoxicinjury
4 . Diffusebrain swelling/ edema
5 . Pressurenecrosisfrom brain herniation
Cause: increasedintracranialpressure
Location: cingulate,uncal,parahippocampal
gyri,cerebellartonsils
6. Secondary"delayed"hemorrhage
7. Secondarybrainsteminjury(mechanical
compression, secondary(Duret)hemorrhagein
tegmentumof rostralpons + midbrain,infarction
of median/ paramedianperforatingarteries,
necrosis)
8. Other(eg,fattyembolism,infection)
. Duret hemorrhage = hemorrhagein lateral
brainstemdue to massivetemporallobe herniation
Kernahorn notch = contusionof contralateral
brainstemcausedby pressureof free edge of
tentorium
Pathomechanism:
A. Directimpacton braindue to fracturei skull
distortion
{ scalp/ skullabnormal
{ superficialneuraldamagelocalizedto immediate
vicinityof calvarialinjury
1. Corticallacerationdue to depressedfracture
fragment
2. Epiduralhematoma
B. Indirectinjuryirrespectiveof skull deformation
! scalp/ skullnormal
(a) compression-rarefaction strain= changein cell
volumewithoutchangein shape(rare)
(b) shearstrain= changein shapewithoutchangein
volumeby
- rotationalaccelerationforces (morecommon)
r/ bilateralmultiplesuperficial/deep lesions
possiblyremotefrom the site of impact
1. Corticalcontusion(brainsurface)
2. Diffuseaxonalinjury(whitematter)
3. Brainstem+ deep gray matternuclei
- linearaccelerationforces(lesscommon)
1. Subduralhematoma
2. Smallsuperficial contusion
Prognosis: 10"/"fatal, 5-10% with residualdeficits
Centripetal approach in search of injuy:
A. Scalp
1. Scalpabrasion:not visible
2. Scalplaceration:air inclusion
3. Scalpcontusion:salt-and-pepper densities
B. Subgalealhematoma
Location: betweenperiosteumof outertable and
galea (= underneathscal' fat)
Brain Disorders 285
C. Skullfracture:
linear-, stellate-, depressed-, basilar-, eggshell-
D. Epiduralhematoma
E. Subduralhematoma
F. Subarachnoid hemorrhage
G. Braininjury
1. C ontusi on/edema
2. Brainhematoma
H. Ventricularhemorrhage
ExtracerebralHemorrhage
1. S ubduralhematoma
in adults: dura inseparable from skull
2. Epiduralhematoma
in children:dura easilystrippedawayfrom skuli
3. Subarachnoid hemorrhage
commonaccompanimentto severecerebraltrauma
IntracerebralHemorrhage
1. H ematoma
= bloodseparatingrelativelynormalneurons
(a) shear-strain
injury(mostcommon)
(b) blunt/ penetratingtrauma(bullet,ice pick,skull
fracturefragment)
lncidence: 2-160/oof trauma victims
Location: low frontal+ anteriortemporalwhite
matter/ basalganglia(80-90%)
. frequentlyno lossof consciousness
. developmentmay be delayedin Bh of head
, injuries
r/ well-defined homogeneously increaseddensity
2. Corticalcontusion
= blood mixedwith edematousbrain
./ poorlydefinedarea of mixed high and low
densities,may increasewith time
3. Intraventricular hemorrhage
= potentialcomplicationof any intracranial
hemorrhage
0 For earliestdetectionfocus on occipitalhorns!
OtherPosttraumaticLesions
1. P neumocephal us
2. Penetratingforeignbody
lndications for radiographic skull series:
Only in conjunction with positiveCT scanfindingsl
1. Evaluationof depressedskullfracture/ fractureof
base of the skull
lndications for CT:
1. Lossof consciousness (morethan transient)
2. Alteredmentalstatusduringobservation
3. Focalneurologicsigns
4. Clinicallysuspectedbasilarfracture
5. Depressedskullfracture(= eLrlgrtable of fragment
belowlevelof innertableof calvarium)
6. Penetrating wound(eg,bullet)
7. Suspectedacute subarachnoidhemorrhage,
epidural/ subdural/ parenchymalhematoma
286 Radiology Review Manual

CT reportmust address: Age: (a) adulthood(>80%): 20-50 years,averageage

ri midlineshift of 33 years; M > F
r/ localizedmass effect (b) childhood(<20/"): in von Hippel-Lindau disease
./ distortioni effacementof basal,perimesencephalic, (10-20% );gi rl s
suprasellar, quadrigeminal cisterns Associated with:
r/ pressureon brainstem,brainstemabnormality (a) von Hippel-Lindaudisease(in 20/"), may have
r/ hemorrhageI contusion: extraaxial,intraaxial, multiplehemangioblastomas (only 20% of patients
subarachnoid, intraventricular show other stigmata)
r/ edema: generalized/ localized (b) pheochromocytoma (oftenfamilial)
^/ hydrocephalus (c) syringomyelia
r/ presenceof foreignbodies,bullet,bone fragments, (d) spinalcord hemangioblastomas
air . headaches,ataxia,nausea,vomiting
r/ base of skull,face, orbit . erythrocythemia in 20/" (tumorelaboratesstimulant)
{ scalpswelling Location: paravermiancerebellarhemisphere(85%)>
spinalcord > cerebralhemisphere/
lndications for MR: brainstem;multiPlelesionsin 10%
1. Postconcussive symptomatology r/ solid(113)lcystic/ cystic+ muralnodule
2. Diagnosisof smallsub-/ epiduralhematoma r/ solid portionoften intenselyhemorrhagic
3. Suspecteddiffuseaxonal(shearing)injury,cortical r/ almostnevercalcifies
contusion,primarybrainsteminjury CT:
4. Vasculardamage(eg, pseudoaneurysm formation { cysticsharplymarginatedmass of CSF-density(2/3)
due to basilarskullfracture) { peripheralmuralnodulewith homogeneous
enhancement(50%)
Sequelae of head injury: { occasionally solidwith intensehomogeneous
1. Posttraumatic hydrocephalus (1/3) enhancement
= obstructionof CSF pathwayssecondaryto MR:
intracranialhemorrhage; developswithin3 months { well-demarcated tumor mass moderatelyhypointense
2. Generalized cerebralatrophy(1/3) on Tl W l + T2W l
= resultof ischemia+ hypoxia { hyperintense areason Tl Wl (= hemorrhage)
3. Encephalomalacia { hypointense areason TlWl + hyperintense areason
r/ focal areas of decreaseddensity,but usually T2Wl (= cyst formation)
higherdensitythan CSF r/ intralesionalvermiformareas of signaldropout
4. Pseudoporencephaly (= high-velocitybloodflow)
= CSF-filledspacecommunicating with ventricle/ { heterogeneous enhancement on Gd-DTPAwith
subarachnoidspace from cysticdegeneration nonenhancingfoci of cyst formation+ calcification
5. Subduralhygroma + rapidlyflowingblood
6. Leptomeningeal cyst { perilesional Gd-DTPAenhancingareasof slow-
= progressiveprotrusionof leptomeningesthrough flowingbloodvesselsfeeding+ drainingthe tumor
traumaticcalvarialdefect { peripheralhyperintense rim on T2Wl (= edema)
7. Cerebrospinal fluidleak A ngi o:
. rhinorrhea,otorrhea(indicatingbasilarfracture r/ denselystainedtumor niduswithincyst ("contrast
with meningealteaQ loading")
8. Posttraumaticabscess { stainingof entirerim of cyst
secondaryto (a) penetratinginjury(b) basilarskull { drainingvein
fracture(c) infectionof traumatichematoma
9. Parenchymal injury Prognosis: >85/" postsurgicalS-yearsurvival rate
brainatrophy,residualhemoglobindegradation DDx: (1) Cysticastrocytoma(>5 cm, calcifications,larger
products,wallerian-typeaxonaldegeneration, nodule,thick-walledlesion,no angiographic
demyelination, cavitation,microglialscarring contrastblushof muralnodule,no
Prognosis; up to 10% fatal; 5-10% with some degree of erythrocythemia)
neurologicdeficit (2) Arachnoidcyst (if mural nodulenot visualized)
Mortality: 251100,000per year (traffic-relatedin 20- (3) Metastasis(moresurroundingedema)
50/", gunshot20-40%; falls)

HEMATOMA OF BRAIN
HEMANGIOBLASTOMA OF CNS = INTRACEREBRAL HEMATOMA
= benignautosomaldominanttumorof vascularorigin Etiology:
lncidence: 1-2.5/" of all intracranialneoplasms;most A. Verycommon
commonprimaryinfratentorial neoplasmin (50%)
1. Chronichypertension
adults(10% of posteriorfossa tumors) Age: >60 years
 BrainDisorders 287

Location: externalcapsuleand basalganglia Resolution: resorptionfrom outsidetowardthe

(putamenin 65%)/ thalamus(25%), center;ratedependson size of
pons (5%)+ brainstem(10%), hematoma(usually1-6 weeks)
cerebellum(5/"), cerebral FALSE-NEGATIVE CT:
hemisphere(5%) f . impairedclotting
2. Trauma 2. anemi a
3. Aneurysm { iso-/ hypodensestage
4. Vascularmalformation:AVM, cavernous
hemangioma, venousangioma,capillary Hyperacute Cerebral Hemorrhage
telangiectasia Timeperiod: <24 hours
B . Com m on Substrate: fresh oxygenatedarterialblood contains
1. Hemorrhagic infarction= hemorrhagic 95% diamagnetic(= no unpaired
transformationof stroke electrons)intracellular
oxyhemoglobin
2. Amyloidangiopathy(20%): etdertypatients (Fer.)with higherwater contentsthan
3. Coagulopathy (5%): DlC, hemophilia, idiopathic whitematter;oxyhemoglobin persistsfor
thrombocytopenic purpura;aspirin,heparin, 6-12 hours)
coumadin NCCT:
4. Drugabuse(5%): amphetamines, cocaine, r/ homogeneous consolidated high-density
lesion
heroine (50-70 HU) with irregularwell-defined margins
5. Bleedingintotumor increasingin densityduringday 1-3 (hematoma
(a) primary:GBM, ependymoma, attenuation dependenton hemoglobin
oligodendroglioma, pituitaryadenoma concentration+ rate of clot retraction)
(b) metastatic:melanoma,choriocarcinoma, { usuallysurroundedby low attenuation(edema,
renalcancer,thyroidcancer,adenocarcinoma contusion)appearingwithin 24-48 hours
C. Uncommon (a) irregularshape in trauma
1. Venousinfarction (b) spherical+ solitaryin spontaneous
, hemorrhage
2. Eclampsia r/ less mass effectcomparedwith neoplasms
3. Septicemboli MR (lesssensitivethan CT duringfirsthours):
4. Vasculitis(especiallyfungal) ^/ titttedifferenceto normalbrain parenchyma
5. Encephalitis = centerof hematomaiso-to hypointense on Tl Wl
Stagesof CerebralHematomas , minimallyhyperintense
+ on T2Wl
r/ peripheralrim of hypointensity(= degradedblood
Progression: hematomagradually,,snowballs,'in size, productsas clue for presenceof hemorrhage)
dissectsalongwhite mattertracts;may
decompressintoventricularsystem/ Acute CerebralHematoma
subarachnoidspace Timeperiod: 1-3days

MR Appearanceof IntracerebralHematoma
Phase Age Compartment Hemoglobin Tl T2 Comments
Hyperacute<24 hr intracellular oxyhemoglobin iso hyper hyperacutebleedin <1 hr
deoxygenation
Acute 1 - 3d i n tra c e l l u l a r d e o x y h e mogl obi nhypo hypo withinclottedintacthypoxicRBCs
extracellular deoxyhemoglobiniso iso after lysisof RBCs
Subacute oxidation
early >3 d intracellular methemoglobin hyper hypo within intactRBCs insideretractingclot
late >7 d extracellular methemoglobin hyper hyper after lysisof RBCs
C hr onic > 14 d
center extracellular hemichromes iso hyper non-iron-containing
hemepigments
rim intracellular hemosiderin hypo hypo within macrophages,presentfor years
fibroustissue hypo hypo
.O.ru iso hyper
, "DD-BD-BB-OO
mnemonic: o
Dark-Dark acute 0-2 days deoxyhemoglobin
Bright-Dark early subacute 3-7 days intraceflular methemoglobin
Bright-Bright latesubacute g-14 days extracellu lar methemoglobin
Dark-Dark chronic >14 days hemosiderin
288 Radiology Review Manual
Substrate: paramagnetic(= 4 unpairedelectrons)
intracellular
deoxyhemoglobin (Fe'.);
deoxyhemoglobin persistsfor 3 days
MR:
{ slightlyhypo-/ isointenseon Tl Wl
(= paramagnetic deoxyhemoglobin withinclotted
intacthypoxicRBCs does not cause T1
shortening)
{ very hypointenseon T2Wl (progressive
concentrationof RBCs,blood clot retraction,and
fibrinproductionshortenT2)
^/ surrounding tissueisointenseon Tl Wl i
hyperintense on T2Wl (edema)
Early SubacuteCerebralHematoma
Time period: 3-7 days
Substrate: intracellularstronglyparamagnetic(= 5
unpairedelectrons)methemoglobin (Fe3.)
inhomogeneously distributedwithincells
NCCT:
r/ increasein size of hemorrhagicarea over days /
weeks
^/ nign-Oensitylesionwithin1stweek;oftenwith
layering
MR:
^/ very hyperintenseon Tl Wl (= oxidationof
deoxyhemoglobin to methemoglobin resultsin
markedshorteningof T1)
(a) beginningperipherally in parenchymal
hematomas
(b) beginningcentrallyin partiallythrombosed
aneurysm(oxygentensionhigherin lumen)
DDx: melanin,high-protein concentration,
flow-related enhancement, gadolinium-
basedcontrastagent
r/ very hypointense on T2Wl (= intracellular
methemoglobin causesT2 shortening)
Late SubacuteCerebralHematoma
Timeperiod: 7-14 days
Substrate: extracellularstronglyparamagneticmet-
hemoglobin(homogeneously distributed)
NCCT:
./ gradualdecreasein densityfrom peripheryinward
(1-2 HU per day) during2nd + 3rd week
CE CT :
{ peripheralrim enhancementat innerborderof
perilesionallucency(1-6 weeksafterinjury)in
80% (secondaryto blood-brainbarrierbreakdown
/ luxuryperfusion/ formationof hypervascular
granulationtissue)
{ ring blushmay be diminishedby administration
corticosteroids
M R:
^/ hyperintense on Tl Wl (= RBC lysisallowsfree
passageof water moleculesacrosscell
membrane)
./ hyperintenseon T2Wl (= comPartmentalization
methemoglobin is lostdue to RBC lysis)
{ surroundingedema isointenseon TlWl
on T2WI
+ hyperintense
Chronic CerebralHematoma
Timeperiod: >14 daYs
Substrate: superparamagnetic ferritin (= soluble
+ storedin intracellular compartment) and
hemosiderin(= insoluble+ storedin
lysosomes)cause markedfield
inhomogeneities
NCCT:
{ isodensehematomafrom 3rd-1Othweek with
perilesionalringof lucencY
CT:
r/ hypodensephase (4-6 weeks)secondaryto fluid
uptakeby osmosis
ri decreaseddensity(3-6 months)/ invisible
{ after10 weekslucenthematoma
(encephalomalacia due to proteolysis and
phagocytosis + surroundingatrophy)with ring
bl ush(D D x:tumor)
MR:
{ rim slightlyhypointense on TlWl + very
hypointense on T2Wl (= suPerParamagnetic
hemosiderin+ ferritinwithin macrophages);rim
graduallyincreasesoverweeksin thickness,
eventuallyfillsin entirehematoma= HALLMARK
r/ centerhyperintense on Tl Wl + T2Wl
(= extracellular methemoglobin of lysedRBCsjust
insidethe darkerhemosiderin ring);presentfor
monthsto 1 year
{ surroundinghyperintensity on T2Wl (= edema
+ serum extrudedfrom clot)with associatedmass
effectshouldresorbwithin4-6 weeks
(DDx: malignanthemorrhage)
Prognosisj (1) herniation(if 3-4 cm in size)
(2) death(if >5 cm in size)
Basal Ganglia Hematoma
= ruptureof smalldistalmicroaneurysmsin the
arteriesin patientswith poorly
lenticulostriate
controlledsystemicarterialhypertension
Cx: (1) Dissectioninto adjacentventricles(2/3)
(2) PorencephalY
(3) Atrophywith ipsilateralventriculardilatation
GRAYMATTER
HETEROTOPIC
= collectionof corticalneuronsin an abnormallocation
secondaryto arrestof migratingneuroblastsfrom
ventricularwalls to brain surfacebetween7-24 weeks
of GA
Frequency: 3o/"of healthypopulation
of May be associatedwith: agenesisof corpuscallosum,
aqueductalstenosis,
microcephaly, schizencePhalY
. seizures
Location:
(1) nodularform: usuallysymmetricbilaterally in
of subependymalregion/ periventricular white matter
with predilectionfor posterior+ anteriorhorns
 BrainDisorders 289

(2) laminarform: deep/ subcorticalregionswithinwhite 4. Cebocephaly= 2 hypoteloric orbits+ singlenostril

, matter(lesscommon) with smallflattenednose + absentnasalseptum
{ single/ multiplebilateralsubependymal nodulesalong 5. Mediancleftlip + cleftpalate+ hypotelorism
lateralventricles 6. Others:micrognathia, trigonocephaly (early
./ lrtOsurroundingedema,isointense with gray matteron closureof metopicsuture),microphthalmia,
all sequences,no contrastenhancement microcephaly
DDx: subependymal spreadof neoplasm,subependymal
hemorrhage, vascularmalformation,tuberous r/ thalamifused
sclerosis,intraventricular
meningioma, { protrusionof anteriorlyplacedfusedthalami+ basal
neurofibromatosis
, gangliainto monoventricle
I absenceof: septumpellucidum, 3rd ventricle,falx
cerebri,interhemispheric fissure,corpuscallosum.,
HOLOPROSENCEPHALY fornix,optic tracts,olfactorybulb (= arrhinencephaly),
= lack of cleavage/ diverticulation of the forebrain internalcerebralveins,superior+ inferiorstraight
(= prosencephalon) laterally(cerebralhemispheres), sagittalsinus,vein of Galen,tentorium,sylvian
transversely(telencephalon, diencephafon), horizontally fissure,opercularcortex
(optic+ olfactorystructures)as a consequenceof { crescent-shaped holoventricle= singlelargeventricle
arrestedlateralventriculargrowthin 6-weekembryo; withoutoccipitalor temporalhorns
corticalbraintissuedevelopsto cover the monoventricle r/ largedorsalcyst occupyingmost of calvarium
and fusesin the midline;posteriorpart of the , + widelycommunicating with singleventricle
monoventricle becomesenlargedand saclike ! "horseshoe" / "boomerang" configuration of brain
0 Septumpellucidumalwaysabsent! = peripheralrim of cerebralcortexdisplacedrostrally
lncidence; 1:16,000;M:F = 1.1 (coronalplane)
A. ALOBAR = no hemispheric development (a) pancakeconfiguration= cortexcovers
B. SEMILOBAR= some hemispheric development monoventricleto edge of dorsalcyst
C. LOBAR = frontaland temporallobation+ small (b) cup configuration= more cortexvisibleposteriorly
monoventricle (c) ballconfiguration = completecoveringof
Associatedwith: polyhydramnios(60%),renal+ cardiac monoventricle withoutdorsalcvst
anomalies;chromosomal anomalies
(predominantly trisomy13 + 18)
Associatedborderlinesyndromessecondaryto
diencephalic malformation
1. A nopht h a l m i a
2. Microphthalmia
3. Aplasiaof pituitarygtand
:
QQ@
Pancake Cup Ball
4. Olfactogenital dysplasia { midbrain,brainstem,cerebellumstructurally normal
5. Septo-opticdysplasia r/ pancakelikecerebrumin posteriorcranium
DDx: r/ cerebralmantlepachygyric
(1) Severehydrocephalus (roughlysymmetrically r/ midlinecleftsin maxilla+ palate
thinnedcortex) Prognosis; death within 1st year of life / stillborn
(2) Dandy-Walkercyst (normalsupratentorial ventricular DDx: massivehydrocephalus,hydranencephaly
system)
(3) Hydranencephaly (frontal+ parietalcortexmost SemilobarHoloprosencephaly
severelyaffected) = intermediate form with incompletecleavageof
(4) Agenesisof corpuscallosumwith midlinecyst prosencephalon (moremidlinedifferentiation
(lateralventricleswidelyseparatedwith pointed + beginningof sagittalseparation)
superolateralmargin) . mildfacialanomalies:midlinecleftlip + palate
. hypotelorism
Alobar Holoprosencephaly . mentalretardation
= extremeform in which the prosencephalon does not r/ singleventricularchamberwith partiallyformed
divide occipitalhorns+ rudimentary temporalhorns
. minimalmotoractivity,littlesensoryresponse r/ peripheralrim of braintissueis severalcm thick
(ineffective
brainfunction); seizures r/ partiallyfused thalamianteriorlysituated+ abnormally
. severefacialanomalies("theface predictsthe brain,'): rotatedresultingin small3rd ventricle
1. Normalface in 17o/" r/ absenceof septumpellucidum+ corpuscaltosum
2. Cyclopia(= midlinesingleorbit);may have + olfactorybulb
proboscis(= fleshysupraorbital prominence) r/ rudimentary falx cerebri+ interhemisphericfissure
+ absentnose form caudallywith partialseparationof occipitallobes
3. Ethmocephaly= 2 hypoteloricorbits+ proboscis r/ incompfetehippocampalformation
betweeneyes and absenceof nasalstructures Prognosis; infantssurvivefrequentlyinto adulthood
290 Radiology Review Manual
LobarHoloprosencephaly
= mildestform with two cerebralhemispheres+ two
distinctlateralventricles
0 May be part of septo-opticdysplasia!
. usuallynot associatedwith facialanomaliesexcept
for hypotelorism
. mild to severementalretardation,spasticity,athetoid
movements
r/ closelyapposedbodiesof lateralventricleswith
distinctoccipital+ frontalhorns
{ mild dilatationof lateralventricles
{ colpocephaly
r/ unseparatedfrontalhornsof angularsquaredshape
+ flat roof (on coronalimages)due to dysplastic
frontallobes
fissure
r/ dysplasticanteriorfalx + interhemispheric
r/ absenceof septumpellucidum+ sylvianfissures
{ corpuscallosumusuallypresent
r/ nippocampalformationnearlynormal
r/ basalganglia+ thalamimay be fused / separated
{ pachygyria(= abnormallywide + plumpgyri),
lissencePhalY (= o gYri)
Prognosis; survivalintoadulthood
OF BRAIN
HYDATIDDISEASE
= caninetapeworm(Echinococcus granulosus)in sheep-
and areas
cattle-grazing
Location: liver(60%),lung (25/"), CNS (2%)subcortical
{ usuallysingle,largeround,sharplymarginatedsmooth-
walledhypodensecyst
surroundingedema;no rim enhancement
{ no significant
{ devetopmentof daughter cysts (after rupture/ following
diagnosticpuncture)
HYDRANENCEPHALY
= liquefactionnecrosisof cerebralhemispheresreplaced
by a thin membranoussac of leptomeninges in outer
layer+ remnantsof cortexand white matterin inner
layer,filledwith CSF + necroticdebris
lncidence: O.2/" of infantautopsies
Etiology: absenceof supraclinoidICA system(? vascular
occlusion/ infectionwith toxoplasmosisor CMV)
= ultimateform of porencephaly
. seizures;respiratoryfailure;generalizedflaccidity
. decerebratestatewith vegetativeexistence
{ normalskullsize/ macrocrania / microcrania
{ completefillingof hemicranium with membranoussac
{ absenceof corticalmantle(inferomedialaspectof
temporallobe, inferioraspectof frontallobe,occipital
lobe may be identifiedin some patients)
^/ brainstemusuallyatrophic
{ cerebellumalmostalwaysintact
r/ thalamic,hypothalamic, mesencephalic structures
usuallypreserved+ projectinto cysticcavity
{ centralbraintissuecan be asymmetric
{ choroidplexuspresent
./ tatx cerebri+ tentoriumcerebelliusuallyintact,may be
deviatedin asymmetricinvolvement,may be incomplete
/ absent
Prognosis; not compatiblewith prolongedextrauterinelife
improvement
(no intellectual from shunting)
DDx: (1) Severehydrocephalus(some identifiablecortex
present)
(2) Alobarholoprosencephaly (facialmidline
anomalies)
(3) Schizencephaly(somesparedcorticalmantle)
HYDROCEPHALUS
= €XCesS of CSF due to imbalanceof CSF formation
+ absorptionresultingin increasedintraventricular
pressure
Pathophysiology:
A. Overproduction(rare)
B. lmpairedabsorption
1. Blockageof CSF flowwithinventricularsystem,
cisternamagna,basilarcisterns,cerebral
convexities
2. Blockageof arachnoidvilli/ lymphaticchannels
of cranialnerves,spinalnerves,adventitiaof
cerebralvessels
GompensatedhydrocephaluS= flew equilibrium
establishedat higherintracranialpressuredue to
openingof alternatepathways(arachnoidmembrane/
stromaof choroidplexus/ extracellularspace of
corticalmantle= transePendymal flow of CSF)
Skullfilm: signsof raisedintracranialpressure
A . Y OU N GIN FA N T/ N E W B OR N
r/ increasein craniofacialratio
./ butgingof anteriorfontanel
{ suturaldiastasis
r/ macrocephaly+ frontalbossing
"hammeredsilver"appearance
r/ "beatenbrass"=
= prominentdigitalimpressions (widerangeof
normalsin 4-10 yearsof age)
B. ADOLESCENT/ ADULT(changesin sellaturcica)
{ atrophyof anteriorwall of dorsumsellae
rl shorteningof the dorsumsellaeproducingpointed
appearance
of floorof sella
^/ erosion/ thinningi discontinuity
r/ depressionof floorof sellawith bulginginto
sphenoi dsi nus
{ enlargementof sellaturcica
DDx: osteoporoticsella (aging,excessivesteroid
hormone)
Signsfavoringhydrocephalusover white matteratrophy:
r/ commensuratedilatationof temporalhorn with lateral
ventricles(mostreliablesign)
^/ narrowingof ventricularangle (= angle between
anterior/ superiormarginsof frontalhornsat levelof
foramenof Monro)due to concentricenlargement:
ri Mict<eyMouseears on axialscans
{ enlargementof frontalhorn radius(= widestdiameter
of frontalhornstaken at 90" angleto long axis of
frontalhorn):
r/ roundingof frontalhornshape
 BrainDisorders 291

r/ enlargementof ventricularsystemdisproportionate to Noncommunicating Hydrocephatus

enlargementof corticalsulci (due to compressionof = INTRAVENTRICULAR HYDROCEPHALUS
braintissueagainstskull+ consequentsulcal = blockadeof CNS flow withinthe ventricularsystem
narrowing) with dilatationof ventriclesproximalto obstruction
r/ interstitialedemafrom transependymalflow of CSF: Pathogenesis: increasedCSF pressurecauses
r/ periventricular hypodensity ependymalflatteningwith breakdown
r/ rim of prolongedTl +T2 relaxationtimes
of CSF-brainbarrierleadingto
surroundinglateralventricles myelindestruction + compression of
r/ hydrocephalicdistortionof ventricles+ brain:
cerebralmantle(braindamage)
! atrialdiverticulum = herniationof ventricularwall Location:
throughchoroidalfissureof ventricular trigoneinto (a) Lateralventricularobstruction
. supracerebellar + quadrigeminal cisterns Cause: ependymoma,intraventricular glioma,
r/ dilatationof suprapinealiecess expandinginto menrngtoma
posteriorincisuralspace resultingin inferior (b) Foramenof Monroobstruction
displacement of pinealgland/ shorteningof tectumin Cause: 3rd ventricularcolloidcyst,tuber,
rostral-caudal direction/ elevationof vein of Galen papilloma,meningioma, septum
{ enlargementof anteriorrecessof 3rd ventricle pellucidumcyst/ glioma,fibrous
extendinginto suprasellarcistern membrane(postinfection), giantcell
astrocytoma
(c) Third ventricularobstruction
Cause: largepituitaryadenoma,teratoma,
ObstructiveHydrocephalus craniopharyngioma, gliomaof 3rd
= obstructionto normal CSF flow + absorption ventricle,hypothalamic glioma
(d) Aqueductafobstruction
Communicating Hydrocephatus Cause: Congenitalweb / atresia(often
= EXTRAVENTRICULAR HYDROCEPHALUS associatedwith Chiarimalformation),
= elevatedintraventricular pressuresecondaryto fenestratedaqueduct,tumor of
blockadebeyondthe outletof 4th ventriclewithin mesencephalon / pinealgland,
the subarachnoidpathways tentorialmeningioma, S/P intra-
lncidence: 38% of congenitalhydrocephaly ventricularhemorrhageor infection
Pathophysiology: (e) Fourthventricularobstruction
unimpededCSF flowthroughventricles;impeded Cause: Congenitalobstruction,Chiari
CSF flow overconvexitiesby adhesionsi impeded malformation, Dandy-Walker
reabsorptionby arachnoidvilli syndrome,inflammation(TB),tumor
Cause: within4th ventricle(ependymoma),
repetitivesubarachnoidmicrohemorrhage (most extrinsiccompressionof 4th ventricle
commoncause),immaturityof arachnoidvilli, (astrocytoma,medulloblastoma, large
meningealcarcinomatosis (medulloblastoma, CPA tumors, posteriorfossamass),
germinoma,leukemia,lymphoma,
adenocarcinoma), purulent/ tuberculousmeningitis,
subduralhematoma,craniosynostosis,
{ enrarsed,",Jil'?';fl
i:',33?:l
i,,.|"H:}ii'.;
occipitalhorns precedesenlargementof frontal
achondroplasia, Hurlersyndrome,venous horns)
obstruction(obliterationof superiorsagittalsinus), { effacedcerebralsulci
absenceof Pacchionigranulations ./ periventricular
edemawith indistinctmargins
r/ symmetricenlargemenlof lateral,3rd, and often
, (especiallyfrontalhorns)
4th ventricles ! radioisotopecisternography:no obstructionif
r/ dilatationof subarachnoidcisterns tracerreachesventricle
r/ normal/ effacedcerebralsulci { changein Rl indicatesincreasedintracranial
r/ symmetriclow attenuationof periventricular white pressure(ARl47-1327oversusB-29/" in normals)
, matter(transependymal CSF flow)
r/ delayedascentof radionuclidetracerover NonobstructiveHydrocephalus
convexities = secor'td?ryto rapidCSF production
r/ persistenceof radionuclidetracerin lateral Cause: Choroidplexuspapilloma
ventriclesfor up to 48 hours r/ ventriclenearpapillomaenlarges
Changesaftersuccessfulshunting: r/ intenseradionuclideuptakein papilloma
r/ diminishedsizeof ventricles+ increased r/ enlargedanterior/ posteriorchoroidalarteryand blush
prominenceof sulci
{ cranialvaultmay thicken CongenitalHydrocephalus
Cx: subduralhematoma(resultfrom = multifactorial
CNS malformation
duringthe 3rd / 4th
precipitous decompression) week after conception
292 Radiology Review Manual

Etiology: lnfantile Hyd rocephalus

(1) aqueductalstenosis(43%) . oculardisturbances:paralysisof upwardgaze,
(2) communicating hydrocephalus (38%) abducensnerveparesis,nystagmus,ptosis,
(3) Dandy-Walker syndrome(13%) diminishedpupittarylightresponse
(4) otheranatomiclesions(6%) . spasticityof lowerextremities(fromdisproportionate
(a) Geneticfactors: spina bifida,aqueductal stretchingof paracentralcorticospinalfibers)
stenosis(X-linkedrecessivetraitwith a 5Q/" Etiology:
"A VP-ShuntCan DecompressThe
recurrencerate for male fetuses),congenital mnemonic.'
atresiaof foraminaof Luschkaand Magendie HydrocephalicChild"
(Dandy-Walkersyndrome; autosomal Aqueductalstenosis
recessivetrait with 25h recurrencerate), Vein of Galenaneurysm
cerebellaragenesis,cloverleafskull,trisomy Postinfectious
13-18 Superiorvena cava obstruction
(b) Nongeneticetiology:tumorcompressing 3rd / Chiarill malformation
4th ventricle,obliterationof subarachnoid Dandy-Walkersyndrome
pathwaydue to infection(syphilis,CMV, Tumor
rubella,toxoplasmosis), proliferation of fibrous Hemorrhage
tissue(Hurlersyndrome),Chiarimalformations, Ghoroidplexuspapilloma
vein of Galenaneurysm,choroidplexus Doppler:
papilloma,vitaminA intoxication r/ Rt >O.A(signof increasedICP)in neonate:
lncidence: 0.3-1.8:1,000pregnancies ^/ Rl of 0.84 + 13% decreasingto 0.72 + 11"h after
Associated with: shunti ng
(a) Intracranial anomalies(37%): hypoplasiaof r/ nt >o.oS(signof increasedICP)in olderchildren
corpuscallosum,encephalocele,arachnoidcyst,
arteriovenousmalformation
(b) extracranial anomalies(63%): spinabifidain NormalPressureHydrocePhalus
25-30% (withspina bifidahydrocephalusis = N P H= A D A MS Y N D R OME
presentin 80%),renalagenesis,multicystic = pressuregradientbetweenventricle+ brain
dysplastickidney,VSD,tetralogyof Fallot,anal parenchymain spiteof normalCSF pressure
agenesis,malrotationof bowel,cleft lip / palate, 0 Potentiallytreatablecause of dementiain elderly!
Meckelsyndrome,gonadaldysgenesis, Cause: communicating hydrocephalus with incomplete
arthrogryposis, sirenomelia arachnoidalobstrruction from neonatal
( c ) c hr om o s o maaln o ma l i e s(1 1 % ):tri s o my18 + 21, hemorrhage,
intraventricular spontaneous
mosaicism,balancedtranslocation subarachnoid hemorrhage, intracranialtrauma,
. elevatedamnioticalpha-fetoprotein level infection,surgery,carcinomatosis
OB-US (assessmentdifficultpriorto 20 weeks GA as mnemonic: "PAM the HAM"
ventriclesordinarilyconstitutea largeportionof cranial Pagetdisease
vault): Aneurysm
r/ "danglingchoroidplexussign": Meningitis
r/ choroidplexusnot touchingmedial+ lateralwalls Hemorrhage(fromtrauma)
of lateralventricles Achondroplasia
./ downsidechoroidplexusfallingaway from medial Mucopolysaccharidosis
wall + hangingfromtela choroidea Pathophysiologyof CSF:
{ upsidechoroidfattingaway from lateralwall (?) brain pushedtowardcraniumfrom ventricular
r/ lateralwidthof ventricularatrium>10 mm (size enlargement; brainunableto expandduringsystole
usuallyconstantbetween16 weeks MA and term) thus compressinglateral+ 3rd ventricles+ expressing
0 88% of fetuseswith sonographically detected largeCSF volumethroughaqueduct;reversedynamic
neuralaxisanomalieshaveatrialwidth>10 mm "water-hammer" force of recurrent
duringdiastole;
r/ epO >95th percentile(usuallynot before3rd ventricularexpansiondamagesperiventricular tissues
trimester) Age: 50-70 years
r/ polyhydramnios (in 30%) . normalopeningpressureat lumbarpuncture
Recurrence rate: <4o/o . dementia,gait apraxia,urinaryincontinence
Mortality: (1)fetaldeathin24/" mnemonic: wacky, wobblYand wet
(2) neonataldeath in 17"/" r/ communicating hydrocephalus with prominent
Prognosis; poor with temporalhorns
(1) associatedanomalies r/ ventriclesdilatedout of proportionto any sulcal
(2) shiftof midline(porencephaly) enlargement
(3) head circumference>50 cm r/ upwardbowingof corpuscallosum
(4) absenceof cortex(hydranencephaly) r/ flatteningof corticalgyri againstinnertable of
(5) corticalthickness<10 mm calvarium(DDx: roundedgyri in generalizedatrophy)
 BrainDisorders 293

MR: ., elevatedopeningpressureson lumbarpuncture

r/ pronouncedaqueductalflow void (dueto r/ normalventricularsize/ pinchedventricles
diminishedcomplianceof normalpressure { increasedvolumeof subarachnoidspace
hydrocephalus)
r/ periventricular
hyperintensity(due to IN FA R C TION OF B R A IN
transependymalCSF flow) = braincell deathleadingto coagulationnecrosis
Rx: CSF shunting (only 50"/"improved) Cause: largevesselocclusionof ICA / MCA / pCA (SO%)
due to embolifrom atherosclerotic stenosis,
HYPOTHALAMIC HAMARTOMA small-vessellacunes(ZS%),cardiaccause(15%),
= HAMARTOMA OFTUBERCINEREUM blooddisorder(S%),non-arteriosclerotic (5%)
= r?re congenitalmalformationcomposedof normal 0 33% of TlAs will leadto infarction!
neuronaltissuearisingfrom posteriorhypothalamus in Pathophysiology:
regionof tubercinereum distalmicrostasisoccurswithin2 minutesafter
Age: <2 years of age; M > F occlusionof cerebralartery;regionalcerebralbloodflow
Histo: heterotopiccollectionof neurons,astrocytes, is acutelydecreasedin area of infarction+ remains
oligodendroglialcells(closelyresembling depressedfor severaldays at centerof infarct;arterial
histologicpatternof tuber cinereum) circulationtime may be prolongedin entirehemisphere;
. isosexualprecociouspuberty(due to LRH secretion)
rapiddevelopmentof vasodilatationdue to hypoxia,
. gelasticseizures,hyperactivity
hypercapnia,tissueacidosis;delayedfilling+ emptying
. neurodevelopmental delay of arterialchannelsin area of infarction(= arteriolar-
Location: mamillarybodies/ tubercinereumof thalamus, capillaryblock)well intovenousphase;by end of
rarelywithinhypothalamus itself 1st week regionalbloodflow commonlyincreasesto
r/ well-definedround/oval mass projectingfrom base of rateseven above those requiredfor metabolicneeds
, brain into suprasellar/ interpeduncular cistern (= hyperemicphase= luxUfyperfusion)
tr attachedto tubercinereum/ mamillarybodiesby thin Detection rate by CT:
, stalk (pedunculated) 80% for cortex+ mantle,55/"lor basalganglia,S4o/"tor
! remainstablein size over time; up to 4 cm in diameter posteriorfossa
CT: 0 Positivecorrelationbetweendegreeof clinicaldeficit
roundhomogeneous
mass isodensewith braintissue and CT sensitivity
NO enhancement CT sensitivity;
MR: on day of ictus: 48%
{ well-definedroundpedunculated
masssuspended 1-2 days later: 59%
fromtubercinereum/ mamillarybodies 7-10 days later: 66%
./ isointenseon Tl Wl + iso-/ slightlyhyperintense
on 10-11daysl ater: 74/o
T2Wl (imagingcharacteristics
of gray matter) Location: cerebrum:cerebellum = 19:1
{ no gadoliniumenhancement (a) supratentorial
- cerebralmantle(20%) in territoryof MCA (50%),
PCA (10%),watershedbetweenMCA + ACA
IDIOPATHICINTRACRANIALHYPERTENSION (7%), ACA(4%)
= PSEUDOTUMOR CEREBRI = BENIGN INTRACRANIAL - basalganglia+ internalcapsule(20%)
HYPERTENSTON (BtH) (b) infratentorial(10%)
secondaryto uppercerebellum(S%),lowercerebellum(g%),pons
(a) elevationin bloodvolume(85%) + medulla (2%)
(b) decreasein regionalcerebralbloodflow with
delayedCSF absorption(10%) HyperacutelschemicInfarction
Etiology: Timeperiod: <12 hours
1. Sinovenousocclusivedisease,SVC occlusion, CT (relativelyinsensitive+ nonspecific):
obstructionof dural sinus,obstructionof both internal r/ normal(in 10-G0%)
jugularveins
ri subtledecreasein attenuationwithinaffectedbrain
2. DuralAVM area:
3. S/P brain biopsywith edema V loss of gray-whitematterdifferentiation
4. Endocrinooathies r/ insularribbonsign = obscurationof lentiform
5. Hypervitaminosis A nucleusdue to decreasedattenuationwithin
6. Hypocalcemia insula(in 50-80% of MCA occlusions)
7. Menstrualdysfunction,pregnancy,menarche,birth { earlybrainswelling:
controlpills
, r/ hemisphericsulcaleffacement
8. Drugtherapy r/ "hyperdensemiddlecerebralarterysign" = ocUte
Predilectionfor: obese young to middle-agedwomen intraluminal
. headache thrombusof 80 HU (dueto extrusionof
. papilledema serumfrom thrombus)vs. 40 HU of flowingblood;
transientphenomenon
294 Radiology Review Manual
lncidence; 35-50Y. of acute MCA occlusions
DDx: high hematocritlevel,calcificationof vessel
wall
r/ calcifiedintraluminal embolus(rare)
MR (routinelypositiveby 4-6 hourspost ictus):
^/ hyperintensesignalon proton-densityimages
+ T2Wl involvingcorticalgray matter
./ loss of normalintravascularflow voids (similarto
hyperdenseMCA sign)
^/ stasisof contrastmaterialwithinaffectedarteries
r/ subtleparenchymalswellingwith sulcaleffacement
due to cytotoxicedema (= increasedintracellular
water)can be seen by 2 hours post ictus (beston
TlWl)
./ hyperintensesignalfrom less signalloss (due to
restrictedwater diffusibility)on diffusion-weighted
images(MOSTSENSITIVE,hyperintensity
maintainedfor 7-10 days,whichallows
discriminationof acutefrom older infarcts)
r/ ischemicpenumbf?= cotTlbination of perfusion
+ diffusion-weighted imagesallowsidentification of
areas at risk for infarction
NUC:
0 Newerimagingagents(eg,Tc-99mHM-PAO)may
be positivewithinminutesof the event,whileCT
and MR are normal
./ hemispherichypoperfusionthroughoutall phases
r/ defectcorrespondingto nonperfusedvascular
territorY
r/ "flip-flopsign"in radionuclide angiogram(15%)
= decreaseduptakeduringarterial+ capillary
phasefollowedby increaseduptakeduringvenous
phase
{ "luxuryperfusionsyndrome"(14/") = increased
perfusion
Rx: recombinanttissue plasminogenactivator(TPA)
Acutelschemiclnfarction
Histo: corticalcytotoxicedema (fromloss of vascular
autoregulation)followedby white matter
vasogenicedema
Early Acute lschemic Infarction
Time period: 12-24 hours
NCCT:
r/ low-densitybasalganglia
ri loss of differentiationbetweencorticalgray
matterand subjacentwhite matter:
r/ blurringof the clarityof internalcapsule
r/ "insularribbonsign"= hypodenseextreme
capsuleno longerdistinguishable from insular
coftex
{ subtlesulcaleffacement(8%)
CE CT :
r/ no iodineaccumulationin affectedcorticalregion
r/ meningealgyriformenhancement
MR:
{ subtlenarrowingof sulci
r/ blurringof gray-whitematterjunctionon T2- and
proton-densityimages
r/ increasein thicknessof cortex(= gyral swelling)
{ subtlelow-signalintensityon T1Wl, high-signal
intensityon T2Wl (maskingof gyral infarctson
heavilyT2Wl due to sulcalCSF intensity)
MR A :
r/ absenceof flow for infarcts>2 cm in diameter
Late Acute lschemiclnfarction
Timeperiod: 1-3-7 daYs
NCCT:
{ hypodensewedge-shapedlesionwith base at
cortexin a vasculardistribution(in 70%) due to
vasogenic+ cYtotoxicedema
r/ mass effect(23-75%): sulcaleffacement,
transtentorialherniation,displacedsubarachnoid
cisterns+ ventricles
./ "blandinfarct"may be transformedinto
hemorrhagicinfarctatter2-4 days (due to
leakageof bloodfrom ischemicallydamaged
capillaryendothelium followinglysisof
intraluminal clot + arterialreperfusion)
CECT:
r/ decreasedmeningeal+ intravascularcontrast
enhancement
^/ increasedparenchymalenhancement
MR:
r/ intravascularenhancementsign \77%)
= Gd-pentetateenhancementof corticalarterial
vesselsin area of brain injuryafter 1-3 days (due
to slow arterialbloodflow providedby collateral
circulationvia leptomeningeal anastomoses)
r/ meningealenhancement sign (33%)
= Gd-pentetate enhancement of meninges
adjacentto infarct after 2-6 days (due to
meningealinflammation)
A ngi o:
{ narrowedi occludedvesselssupplyingthe area
of infarction
r/ delayedfilling+ emptyingof involvedvessels
{ earlydrainingvein
r/ luxuryperfusionof infarctedarea (rare)= loss of
smallvesselautoregulation due to localincrease
i n pH
Subacutelschemiclnfarction
Time period: 7-30 days = paradoxicalphase with
resolutionof edema+ onsetof
coagulationnecrosis
NCCT:
ri "foggingphenomenon"= low-densityarea less
apparent
./ decreaseof mass effect+ ex vacuo dilatationof
ventricles(in 57%)
r/ + transientcalcification(especiallyin children)
CECT:
{ gyralblush+ ringenhancement(breakdownof
blood-brainbarrier+ luxuryperfusion)for
2-B weeks (in 6$-80% withinfirst 4 weeks)
{ no enhancementin 1/5 of Patients
 Brain Disorders 295

MR: Cause:
Histo: vasogenicedema (= increasedextraceilular (1) E mbol i sm
water)due to disruptionof blood-brain (2) Hypoperfusionm
barrier (3) Carbonmonoxidepoisoning
r/ hypointense on T1Wl, hyperintense on T2Wl (4) Drowning
! intravascular + meningealenhancement signs (5) Vasculopathy(hypertension,microvasculopathy,
resolvetowardend of 1st week aging)
r/ gyriformparenchymalGd-pentetateenhancement r/ dense homogeneousenhancementoutliningcaudate
0 Gyriformparenchymal enhancementpermits
, nucleus,putamen,globuspallidus,thalamus
differentiation
of subacutefrom chronicinfarction! ! denseroundnodularenhancement i peripheralring
enhancement

GhroniclschemicInfarction
Timeperiod: monthsto years (>30 days)
Histo:demyelination + gliosiscomplete(focalbrain LaminarNecrosis
atrophyafter 8 weeks) = ischemicchangesaffectingdeep layersof the cortex
,
r/ cerebralatrophy+ encephalomalacia + gliosis (layers3, 5, 6 very sensitiveto oxygendeprivation)
(HALLMARKS) MR:
r/ possiblecalcification (especially in children) (a) acute stage
NCCT: ./ linearcorticalhyperintensity
on Tl Wl
r/ cysticfoci of CSF density(= sn6sphalomalacia) in r/ contrastenhancement
vasculardistribution r/ white matteredemaon T2Wl
MR: (b) chronicstage
{ patctryregionwith increasedintensityon T2Wl r/ tnin hypointensecortex
r/ gliosis(hyperintense on T2WI)oftensurrounding r/ hyperintensewhite matter
encephalomalacic region r/ enlargementof CSF spaces
r/ walleriandegeneration(= antegradedegeneration
of axonssecondaryto neuronalinjury)of
corticospinaltracts in the wake of old large infarcts
that involvethe motorcortex LacunarInfarction
= srndlldeep infarctsin the distaldistributionof
penetratingvessels(lenticulostriate,
Hemorrhagic thalamoperforating, pontineperforatingarteries,
lnfarction
recurrentarteryof Heubner)
Etiology: lysisof embolus/ openingof collaterals/
Cause: occlusionof small penetratingend arteriesat
restorationof normalblood pressurefollowing
base of braindue to fibrinoiddegeneration
hypotension/ hypertension/ anticoagulation
Predisposed: hypertensive/diabeticpatients
causesextravasationin reperfusedischemic
lncidence: 20/" of cerebralinfarctions
brain
Path: lacune= c€lvitatedinfarctresultingin small hole
lncidence: 6o/"of clinicallydiagnosedbrain infarcts,
traversedby cobweblikefibrousstrands
20'/" of autopsiedbrain infarcts
Histo: "microatheroma" = hyalinization+ arteriolar
Path: petechialhemorrhagesin variousdegreesof
sclerosisresultingin thickeningof vesselwall
coalescence
+ luminalnarrowing
Location: corticomedullary junction . pure motori pure sensorystroke
CT: . ataxichemiparesis
r/ hyperdensity(5G-76HU) appearingwithina
Location: uppertwo{hirds of putamen> caudate>
previouslyimagedhypodenseacuteischemic
thalamus> pons > internalcapsule
infarct = hemorrhagictransformation(in 50-72%)
r/ small discretefoci of hypodensitybetween3 mm and
Falsenegative: hematomaisoattenuating if 15 mm i n si ze(most< 1 cm i n di ameter)
hematocrit<20A ./ nignerin signalintensitythan CSF (dueto marginal
MR:
r/ hypointense areaon T2Wl withinedemamarking , gliosis)
r/ unilateralpontineinfarctsare sharplymarginatedat
, gyri = deoxyhemoglobin of acute hemorrhage
mi dl i ne
ri hyperintense areaon Tl Wl = methemoglobin of
subacutehematoma

TIA and RIND

BasalGangliaInfarct
= occlusionof smallpenetratingarteriesat base of brain
(lenticulostriate/ thalamoperforati
ng arteries)
= lacunarinfarct(infarcts<1 cm in size)
r/ hypodensesmall lesionslocatedperipherallynearI
withincortexwithoutenhancement
{ lesionsdetectedin only 14/o,contralaterallesion
presentin 14/o (CT of marginalvalue)
296 Radiology Review Manual

PATIENTS
IN IMMUNOCOMPROMISED
INFECTION Rx: completesurgicalresection
Cause: underlyingmalignancy, collagendisease, DDx:
cancertherapy,AIDS,immunosuppressive (1) Intraventricularoligodendroglioma (no hemorrhage)
therapyin organtransplants (2) Astrocytoma(peritumoraledema in 20%)
Organism: Toxoplasma,Nocardia,Aspergillus,Candida, (3) Meningioma(almostexclusivelyin trigone,>30 years
Cryptococcus of age)
! poorlydefinedhypodensezoneswith rapidenlargement (4) Ependymoma(in + around4th ventricle/ trigone,in
in size + number,particularlyaffectingbasalganglia childhood)
+ centrumsemiovale(poorlylocalized+ encapsulated (5) Subependymoma (in + around4th ventricle,young
infectionwith poor prognosis) adults)
^/ ringi nodularenhancement(sufficient immune (6) Choroidplexuspapilloma(body+ posteriorhornof
lateralventricle,intenseenhancement, younger
defenses):Toxoplasma,Nocardia
r/ enhancementmay be bluntedby steroidRx patient)
AIDSmay be associatedwith: (7) Colloidcyst (anterior3rd ventriclei foramenof
thrombocytopenia, lymphoma,plasmacytoma, Kaposi Monro,calcifications uncommon)
sarcoma,progressivemultifocalleukoencephalopathy (8) Craniopharyngioma lar origin)
(extraventricu
(9) Teratoma+ dermoidcyst (fat attenuation)
IN I E NCE P HA LY
= complexdevelopmentalanomalycharacterizedby
(1) exaggeratedlordosis DISEASE
JAKOB.CREUTZFELDT
(2) rachischisis = f€lfetransmissiblediseasedevelopingoverweeks
"prion"= proteindevoidof functionalnucleic
(3) imperfectformationof skullbase at foramenmagnum Cause:
M:F= 1:4 acid;? slow-virusinfection
Associated with other anomalies in 84%: Age: olderadults
"spongiformencephalopathy"
anencephaly, encephalocele, hydrocephalus, cyclopia, Histo: classifiedas
absenceof mandible,cleft lip / palate,diaphragmatic . rapidlyprogressivedementia,ataxia,myoclonus
hernia,omphalocele, gastroschisis,
singleumbilical i/ hyperintenselesionsin headof caudatenucleus
artery,CHD, polycystickidneydisease,arthrogryposis, + putamen,bilaterallYon T2Wl
clubfoot ^/ ruOgadoliniumenhancement of lesions
r/ dorsalflexionof head ^/ trtOwhite matterinvolvement
r/ abnormallyshort+ deformedspine Prognosis: usuallyfatalwithin 1 year of onset
Prognosis; almost uniformlyfatal
DDx: (1) Anencephaly SYNDROME
JOUBERT
(2) Klippel-Feil
syndrome . episodichyperpnea
(3) Cervicalmyelomeningocele . abnormaleye movement
. ataxia,mentalretardation
LAR NEUROCYTOMA
INTRAVENTRICU Path: (1) nearlytotalaplasiaof cerebellarvermis
= INTRAVENTRICULAR NEUROBLASTOMA (2) dysplasia+ heterotopiaof cerebellarnuclei
= benignprimaryneoplasmof lateral+ 3rd ventricles (3) near total absenceof pyramidaldecussation
lncidence; unknown;tumor frequentlymistakenfor (4) anomaliesin structureof inferiorolivarynuclei,
intraventricular
oligodendroglioma descendingtrigeminaltract,solitaryfascicle,
Age: 2040 years dorsalcolumnnuclei
Histo: uniformroundcellswith centralroundnucleus ./ +tfrventricletriangle-shaped at mid-level+ bat-wing-
+ fine chromatinstipplingt perivascular shapedsuperiorly
pseudorosettes, (closely
focal microcalcifications { cerebellarhemispheres apposeone anotherin midline
resemblingoligodendroglioma but with neuronal { superiorcerebellarpedunclessurroundedby CSF
intosynapselike
differentiation junctions)
Location: body + frontalhorn of lateralventricle,may
extendinto 3rd ventricle LIPOMA
{ entirelyintraventricular well-circumscribed tumor, = congehitaltumordevelopingwithinsubarachnoid space
coarselycalcified(69%),containingcysticspaces (85%) as a resultof abnormaldifferentiation of the meninx
r/ mild to moderatecontrastenhancement primitiva(whichdifferentiates into pia mater,arachnoid,
{ attachmentto septumpellucidumCHARACTERISTIC inner meningeal layer of dura mater)
r/ + hemorrhageintotumor/ ventricle Incidence: <1ohof braintumors
^/ hydrocephalus Age: presentation in childhood/ adulthood
r/ peritumoraledema extremelyuncommon Associatedwith congenital anomalies:
MR: (a) in anteriorlocation:variousdegreesof agenesisof
r/ isointenserelativeto corticalgray matteron Tl Wl corpuscallosum(in 50-80%)
+ T2Wl with heterogeneousareas due to (b) in posteriorlocation(in <33%)
cysticspaces,vascularflow voids (62%)
calcifications, . asymptomaticin 50%
 BrainDisorders 297

Location: B . IN C OMP LE TELIS S E N C E P H A LY

(usuallyin subarachnoid space)callosalcistern
(25-50%),sylvianfissure,quadrigeminalcistern,
chiasmaticcistern,interpeduncular cistern,Cp angle
cistern,cerebellomedullary cistern,tubercinereum,
choroidplexusof lateralventricle
CT:
r/ well-circumscribedmasswith CT densityof -100 HU
r/ occasionallycalcifiedrim (esp.in corpuscallosum)
r/ no enhancement
MR:
r/ hyperintensemass on Tl Wl + less hyperintenseon
T2Wt (CHARACTERTSTTC)
Lipomaol CorpusGallosum
= cor'tgeflitalpericallosaltumor not actuallyinvolvingthe
corpuscallosumas a resultof faultydisjunctionof
neuroectodermfrom cutaneousectodermduring
processof neurulation
lncidencei approx.30% of intracraniallipomas
Associated with:
(1) anomaliesof corpuscallosum(30%with small
posteriorlipoma,90% with largeanteriorlipoma)
(2) frontalbone defect (frequent)= €flcephdlocele
(3) cutaneousfrontallipoma
. in 50% symptomatic:
. seizuredisorders,mentalretardation, dementia
. emotionallability,headaches
. hem iplegi a
P lainf ilm :
r/ midlinecalcificationwith associatedlucencyof fat
density
CT :
^/ area of markedhypodensityimmediatelysuperiorto
lateralventricleswith possibleextensioninferiorly
betweenventricles/ anteriorlyinto interhemispheric
fissure
r/ curvilinearperipherali nodularcentralcalcification
withinfibrouscapsule(morecommonin anterior
comparedwith posteriorlipomas)
MR:
r/ hyperintense midlinemasssuperior+ posteriorto
corpuscallosumon Tl Wl
r/ no callosalfibersdorsalto lipoma
r/ branchesof pericallosalarteryfrequentlycourse
throughlipoma
DDx: dermoid(denser,extraaxial),teratoma
= ore?sof both agyria+ pachygyria,pachygyricareas
most frequentlyin frontal+ temporalregions
Histo: thick gray + thin white matterwith only four
corticallayersl, lll, V, Vl (insteadof six layers)
Often associated with:
(1) CNS anomalies:microcephaly, hydrocephalus,
agenesisof corpuscallosum,hypoplastic thalami
(2) micromelia,clubfoot,polydactyly,camptodactyly,
syndactyly,duodenalatresia,micrognathia,
omphalocele, hepatosplenomegaly, cardiac+ renal
anomalies
. micrencephaly
. severementalretardation
. hypotonia+ occasionalmyoclonicspasm
. early seizuresrefractoryto medication
r/ smooththickenedcortexwith diminishedwhite matter
r/ figure-eightappearanceof cerebrumon axial images
due to shallowwidenedvertically orientedsylvianfissures
r/ absent/ shallowsulciand gyri (brainlookssimilarto that
in fetuses<23 weeks GA)
r/ middlecerebralarteriescloseto innertable of calvarium
(absenceof sulci)
r/ smallsplenium+ absentrostrumof corpuscallosum
r/ hypoplasticbrainstem(lackof formationof corticospinal
+ corticobulbartracts)
r/ ventriculomegaly (atrium+ occipitalhorns)
r/ midlineroundcalcification in area of septumpellucidum
(cHARACTERtSTtC)
r/ polyhydramnios (50%)
Prognosis; death by age 2
DDx: polymicrogyria(= formationof muftipfesmallgyri
mimickingpachygyriaon CT + MR, mostcommon
aroundsylvianfissures,broadthickenedgyri with
frequentgliosissubjacentto polymicrogyric cortex
as the most importantdifferentiating feature)
LYMPHOID HYPOPHYSITIS
= I?f€ inflammatory autoimmunedisorderwith
lymphocyticinfiltrationof pituitarygland
Associatedwith: thyrotoxicosis+ hypopituitarism
Age: almostexclusivelyin early postpartumwomen
. headaches, visionloss,inabilityto lactate/ to resume
normalmenses
r/ enlargedhomogeneously enhancingpituitarygland
Prognosis; spontaneousregression
Rx: steroids(reductionin pituitarysize on follow-up)

LISSENCEPHALY LYMPHOMA
= AGYRIA-PACHYGYRIA COMPLEX A. PRTMARY CNS LYMPHOMA(93%)
= "smoothbrain" = RETICULUM CELLSARCOMA = HISTIOCYTIC
= rTrostsevereof neuronalmigrationanomalies; LYMPHOMA = MICROGLIOMA
autosomalrecessivediseasewith abnormalcortical Risk: increased(350-fold)in immunocompromised
stratification patients:AIDS (6%),renaltransplant,Wiskott-
agyria = absenceof gyri on brain surface Aldrichsyndrome,immunoglobulin deficiencyA,
pachygyria = focal/ diffusearea of few broadflat gyri rheumatoidarthritis,progressivemultifocal
A. COMPLETELISSENCEPHALY = AGYRIA leukoencephalopathy
most frequentlyparieto-occipitalin location Associatedwith: intraocularlymphoma
298 Radiology Review Manual

B. SECONDARY(7./")= SYSTEMICLYMPHOMA ^/ nypo-to isointense / hyperintense (lesscommon)

Type: NHL > Hodgkindisease relativeto gray matteron T2Wl:
Location: tendencyfor dura mater+ leptomeninges { ring pattern(= centralnecrosiswith denselycellular
. pals iesof c ra n i a n "seaof edema")typicalin
l e rv e sl l l , Vl , V l l rim in hyperintense
r/ hydrocephalus immunocompromised Patients
r/ intensering-shaped contrastenhancement on TlWl
0 Primarylymphomais indistinguishable from secondary! ^/ irregularsinuous/ gyral-likecontrastenhancement or
Clues: (1) multicentric involvement of deep hemispheres homogeneous enhancement:
(2) association with immunosuppression { solidhomogeneous enhancementin
(3) rapid regressionwith corticosteroids / radiation immunocompetent Patient
therapy= "ghosttumor" ^/ irregularheterogeneous ringlikemass in
Prevalence: 0.3-2o/"of all intracranialtumors;7-15/" ot immunocompromised Patient
all primarybraintumors(equivalent to r/ periventricular enhancementis highlySPECIFIC
meningioma+ low-gradeastrocytoma);M > F (DDx:CMV ependymitis)
Peak age: 30-50 years; M:F = 2:1 A ngi o:
Histo: atypicalpleomorphicB-cellsmixedwith reactive ^/ avascularmass / tumor neovascularity
T-cellsinfiltratebloodvesselwalls+ clusterwithin ./ focal blush in late arterial-to-capillary phase persisting
perivascular(Virchow-Robin) spacessimulating well intovenousphase
vasculitis r/ arterialencasement
. symptomsof rapidlyenlargingmass (60%) ri dilateddeep medullaryveins
. symptomsof encephalitis(<25%) NUC:
. stroke(7%) ^/ increaseduptakeof C-11 methionineon PET
. cranialnervepalsy,demyelinating disease ./ increaseduptakeof thallium-2O1 on SPECT
r persorldlity changes,headaches,seizures Prognosis; mediansurvivalof 45 days for AIDS patients;
. cerebellarsigns,motordysfunction mediansurvivalof 3.3 monthsfor immuno-
. CSF cytologypositivein f25-437o: elevatedprotein, competentpatients;improvedwith radiation
mononuclear / blast/ otherlymphomacells therapy(4.5-20 months)+ chemotherapy
Location: supratentorial:posterior fossa= 3-9:1; DDx:
paramedianstructurespreferentially affected; A. Neoplasticdisorders
whitematter+ corpuscallosum(55%),deep (1) Glioma(maybe bilateralwith involvement of
centralgray matterof basalganglia basalganglia+ corpus callosum,may show
+ thalamus+ hypothalamus (17%),posterior densehomogeneous enhancement with
(1 1 % ),s p i n a cl o rd (1% );
f os sa+ c e re b e l l u m vascularity)
multicentricity in 1147"/" (2) Metastases(knownprimary,at gray-whitematter
Site: tendencyto abut ependyma+ meninges(12-30%); junction)
"butterflypattern"of frontallobe lymphoma;dural (3) Primitiveneuroectodermal tumor
involvementmay mimicmeningioma(rare) (4) Meni ngi oma
Spread: typicallyinfiltrating; may crossanatomic B. Infectious disease(multicentricity)
boundaries+ midline,diffuseleptomeningeal (1) Abscess,especiallytoxoplasmosis (largeedema)
spread;subependymalspread+ ventricular (2) Sarcoidosis
encasement (3) Tuberculosis
r/ commonlylargediscretesolitarylesion(57%) C. Demyelinating disease
0 LargelesionsuggestslymPhoma! (1) Mul ti pl escl erosi s
r/ small + symmetricmultiplenodularlesions(43-81%) (2) Progressivemultifocalleukoencephalopathy
r/ diffuselyinfiltrating lesionwith blurredmargins
r/ spontaneousregression(uniquefeature)
CT: SpinalEpiduralLymPhoma
{ usuallymildlyhyperdense(33%)/ occasionally (a) invasionof epiduralspacethroughintervertebral
isodense/ low-density area (leastcommon) foramenfrom paravertebrallymph nodes
r/ titttemass effectwith significantperitumoraledema (b) destructionof bone with vertebralcollapse(less
^/ homogeneously dense+ well-defined / irregular common)
+ patchyperiventricular contrastenhancement (c) directinvolvementof CNS (rare)
r/ commonlythick-walled ringenhancementin
immunocompetentpatient
MR (superiorto CT): Leukemia
{ well-demarcatedroundI oval/ gyral-shaped(rare) CNS affectedin 10/" of patientswith acute leukemia
MASS { enlargement of ventricles+ sulcidue to atrophy(31%)
r/ relativelylittlemass effectfor size r/ sulcal/ fissural/ cisternalenhancement(meningeal
r/ isointense/ slightlyhypointenserelativeto gray in 5Y"
infiltration)
matteron Tl Wl Prognosis; 3-5 months survival if untreated

MEDULLOBLASTOMA
0 Most malignantinfratentorial neoplasm;mostcommon
neoplasmof posteriorfossa in childhood(followedby
cerebellarpilocyticastrocytoma)
lncidence: 1F20/" of all pediatricintracranialtumors;
3040% of all posteriorfossa neoplasmsin
children;2-10% of all intracranial
gliomas
origin: from externalgranularlayerof inferiormedullary
velum (= roof of 4th ventricle)
Histo: completelyundifferentiated cells (S0%),
desmoplasticvariety(25%),glial/ neuronal
differentiation
(25%)
Age: 40% withinfirst5 yearsof life;7S/"in firstdecade;
betweenages 5 and 14 (Zlg ); betweenages 15
and 35 (1/3); M:F = 24j
. durationof symptoms<1 monthpriorto diagnosis:
nausea,vomiting,headache,increasing headsize,ataxia
Site: (a) vermiscerebelli+ roof of 4th ventricle(younger
age gr o u p )i n 9 1 %
(b) cerebellarhemisphere(olderage group)
Siz e: us ually> 2c m i n d i a m e te r
r/ well-definedvermianmass with wideningof space
betweencerebellartonsils
r/ encroachmenton 4th ventricle/ aqueductwith
hydrocephalus (85-95%)
! shift/ invaginationof 4th ventricle
! rapidgrowthwith extensioninto cerebellarhemisphere/
, brainstem(moreoften in adults)
r/ extensioninto cisternamagna+ uppercervicalcord,
occasionally throughforaminaof Luschkainto
cerebellopontine angle cistern
{ mild/ moderatesurrounding edema(90%)
CT:
Classicfeaturesin 53%:
r/ slightlyhyperdense(70%)/ isodense(ZO%)/ mixed
( 10%)les io n
r/ rapid intensehomogeneousenhancement(g7%)
due to usuallysolidtumor
Atypical features:
r/ cystic/ necroticareas (10-16%)with lack of
enhancement
r/ calcificationsin 13%
r/ hemorrhagein O%
{ supratentorialextension
MR:
r/ mixed/ hypointenseon Tl Wl
r/ hypo-/ iso-/ hyperintenseon T2Wl
r/ usuallyhomogeneousGd-DTpAenhancementwith
hypointense rim
{ cerebellarfoliablurred
Cx: (1) Subarachnoidmetastaticspread(30-100%)via
CSF pathwayto spinalcord + cauda equina
("dropmetastases"in 40y"),cerebral
convexities,sylvianfissure,suprasellarcistern,
retrogradeinto lateral+ 3rd ventricle
{ continuous"frosting"of tumor on pia
(2) MetastasesoutsideCNS (axialskeleton,lymph
nodes,lung)aftersurgery
Brain Disorders 299
Rx: surgery+ radiationtherapy(extremelyradiosensitive)
DDx of midline medulloblastoma:
ependymoma,astrocytoma(hypodense)
DDx of eccenlric medulloblastoma:
astrocytoma,meningioma,acousticneuroma
MENINGIOMA
lncidence; most.commonextraaxialtumor; 15-18% of
intracranial tumorsin adults;1-2/" of
primarybraintumorsin children;33%of all
incidentalintracranial neoplasms
Origin: derivedfrom meningothelial cells concentrated
in arachnoidvilli(= "arachnoidcap cells"),which
penetratethe dura (villiare numerousin large
duralsinuses,in smallerveins,alongroot
sleevesof exitingcranial+ spinalnerves,
choroidplexus)
Hi stologic cl assification :
- benignbehaviorpattern
(a) fibroblastictype = fibroustype
interwovenbandsof spindlecells + collagen
+ reticulinfibers
(b) transitionaltype = mixedtype
featuresof meningothelial + fibroblasticforms
- aggressiveimagingappearance
(c) meningothelial = syncytialtype
forminga syncytiumof closelypackedcellswith
indistinctborders
(d) angioblastic/ malignanttype
probablyhemangiopericytoma / hemangioblastoma
arisingfrom vascularpericytes
Age: peak incidence45 years (range35-70 years);rare
<20 years(in children>50% malignant,M > F); M:F
= 1 : 2t o 1 ; 4
Associatedwith: neurofibromatosis type 2 (multiple
meningiomas, occurrencein
childhood),basalcell nevussyndrome
0 10% of patientswith multiplemeningiomashave
type 2 neurofibromatosis !
0 Mostcommonradiation-induced CNS tumorwith
latencyperiodof 19-35 years varyingwith dosage!
Types:
(1) Globular meningioma(mostcommon):
compactroundedmass with invaginationof brain;
flat at base;contactto falx / tentorium/ basal duraI
convexitydura
(2) Meningiomaen plaque:
pronouncedhyperostosisof adjacentbone
particularlyalongbase of skull;difficultto distinguish
hyperostosis fromtumorcloakingthe innertable
(DDx: Pagetdisease,chronicosteomyelitis, fibrous
dysplasia,metastasis)
(3) Mufticentricmeningioma (2-9/o):
16/"in autopsyseries;tendencyto localizeto a
singlehemicranium;presentclinicallyat earlier age;
global/ mixed;CSF seedingis exceptional; in 50%
associatedwith neurofibromatosis type 2
300 Radiology Heview Manual

Location: r/ homogeneous/ heterogeneoustexture(tumor

A. Supratentorial(90%) vascularity,cysticchanges,calcifications)
(a) convexity= 121"t"1 hemisphere(20-34%) r/ arcuatebowingof white matter+ corticaleffacement
(b) parasagittal = medialhemisphere(18-22h): r/ tumor-braininterfaceof low-intensity vessels+ high-
falcinemeningiom a (5%)belowsuperiorsagittal intensitycerebrospinalcleft on T2Wl
sinus,usuallyextendingto bothsides r/ contrastenhancementfor 3-60 minuteson Tl Wl as
(c) sphenoidridge + middlecranialfossa (17-25%) high as 148/" over brain parenchyma
(d) frontobasalat olfactorygroove (10%) r/ "duraltail"sign = curvilinearareaof enhancement
B. Infratentorial (9-15%) taperingoff from the marginof tumoralongdural
(a) cerebellarconvexity(5%) surfacein 60% (= dural tumor infiltration/ reactive
(b) tentoriumcerebelli(24%) hypervascularity / reactivehyperplasticchanges)
(c) cerebellopontine angle (24%) { encasement+ narrowingof vessels
( d) c liv us( < 1 % ) A ngi o:
C. Spine (12%) { "mother-in-law" phenomenon(contrastmaterialshows
Atypicallocation: up early and stays late into venousphase)
"spoke-wheel"patternof tumor vascularity
(a) cerebellopontine angle(<5%) r/ "sunburst"/
(b) optic nervesheath(<2%) with hypervascularcloudlikestain
(c) intraventricular (2-5%\: 80% in lateral(L > R), 15% r/ earlydrainingvein (rare:perhapsin angioblastic
in 3rd, 5/"in 4th ventricle;from infoldingof meni ngi oma)
meningealtissueduringformationof choroidplexus r/ en plaquemeningiomais poorlyvascularized
0 Mostcommontrigonalintraventricular mass in Vascularsupply:
adulthood! A. Externalcarotidartery(almostalways):
(d) ectopic= extradural(<1%): intradiploic space,outer 1. vault: middlemeningealarterY
tableof skull,scalp,paranasalsinus,parotidgland, 2. sphenoidplane+ tuberculum:recurrent
parapharyngeal space,mediastinum, lung,adrenal meningealbranchof oPhthalmic a.
gland 3. tentorium:meningealbranchof
Plainfilm: meningohypophYseal trunk of ICA
r/ hyperostosisat site closeto / withinbone (exostosis, 4. clivus+ posteriorfossa: vertebralarteryI
enostosis,sclerosis) ascendingpharYngealarterY
0 Hyperostosisdoes NOT indicatetumor infiltration! 5. falx: partlymiddlemeningealartery+ others
./ blisteringat paranasalsinuses(ethmoid,sphenoid) B. lnternalcarotidartery(rare):
+ sclerosis(= pneumosinus dilatans) 1. intraventricular:choroidalvessels
r/ enlargedmeningealgrooves(if locationin vault), Cx: localinvasionof venoussinuses
enlargedforamenspinosum
{ calcification1=psammomabodies) A TY P TC AMEL N IN GIOMA (15% )
CT: 1. Low attenuationareaof necrosis,old hemorrhage,
r/ sharplydemarcatedwell-circumscribed slowly cystformation,fat (DDx:malignantglioma,
growingmass metastasis)
r/ wide attachmentto adjacentdura mater (a) Gystic meningioma (24%)
{ "corticalbuckling"of underlyingbrain Frequency; 55-65% in 1st year of life;
r/ hyperdense(70-75% due to psammomatous 10"/"in children
calcifications) / isodenselesionon NECT type | = intratumoralcentral/ eccentriccyst
{ calcificationsin circularI radialpattern in 20-25/" (ischemicnecrosis,microcYstic
(DDx:osteoma) degeneration,breakdownof
{ "intraosseousmeningiomg"= pellTloation of bone with hemorrhagicproducts); often
intra-and extracerebralsoft-tissuecomponent associatedwith meningothelial /
(DDx:fibrousdysplasia) microcystic / atypical/ malignant
r/ hyperostosisof adjacentbone (18%) histologicsubtypes
{ intenseuniformenhancement on CECT (absenceof type ll = extratumoralintraparenchymal cyst
blood-brain barrier) (arachnoidcyst / reactivegliosis/
r/ minimalperitumoral edema(in up to 75%): NO liquefactivenecrosisof adjacentbrain)
correlationbetweentumor size + amountof edema type lll = trappedCSF (DDx:cystic/ necrotic
(DDx: intraaxiallesion) gtioma)
{ cysticcomponent:majorin 2h, minorin 15/" (b) Lipoblastic / xanthomatous meningioma (5%)
metaplastic changeof meningothelial cellsinto
MR (100%detectionratewith gadoliniumDTPA): adipocytes
./ frypo-to isointenseon Tl Wl + iso- to hyperintenseon 2. Heterogeneous I ringenhancement(secondaryto
T2Wl (intensitydependson amountof cellularity blandtumor infarction/ necrosisin aggressive
versuscollagenelements) : histologicvariants/ true cyst formationfrom benign
{ tends to followcorticalsignalintensity fluidaccumulation)
 BrainDisorders 301

3. "En plaque"morphology ME N IN GITIS

4. "Commashape"= combination of semilunar = infectionof the pia mater+ arachnoid+ adjacentCSF
componentboundedby duralinterface+ spherical 1. Pachymeningitis: infectionof dura mater
componentgrowingbeyondduralmargin 2. Leptomeningitis: infectionof pia matter/ arachnoid
5. Sarcomatoustransformation with spreadover (mostcommon)+ CSF
hemisphere+ invasionof cerebralparenchyma . headaches,stiff neck
(leptomeningeal supply) . confusion,disorientation
6. Meningeal hemangiopericytoma . positiveCSF lab analysis
! multilobulatedcontour ROLEof CT and MR:
r/ narrowdural base / "mushroom"shape (1) to excludeparenchymal abscess,ventriculitis,
r/ largeintratumoral vascularsignals localizedempyema
r/ bone erosion (2) to evaluateparanasalsinuses/ temporalbone as
r/ prominentperitumoral edema sourceof infection
r/ multipleirregularfeedingvesselson angiogram (3) to monitorcomplications:hydrocephalus, subdural
effusion,infarction
Sphenoid Wing Meningioma
1. Hyperostotic meningiomaen plaque
. slowlyprogressiveunilateralpainlessexophthalmos
Purulent/ BacterialMeningitis
. numbnessin distribution of cranialnerveV.,+ V, Cause: otitismediai sinusitis
. headaches,seizures
Organism:
2. Meningiomaarisingfrom middlethirdof sphenoid (a)adul ts: Meni ngococcus,D i pl ococcus
ridge pneumoniae, Haemophilus influenzae,
. headaches,seizures
Neisseriameningitidis, Staphylococcus
r/ compression of regionalfrontal+ temporallobes aureus
3. Meningiomaarisingfrom clinoidprocess (b) children: Escherichia coli,Citrobacter,
r/ encasementof carotid+ middlecerebralarteries
B-hemolyticStreptococcus
ri compressionof optic nerve+ chiasm . fever,headache,seizures
4. Meningiomaof planumsphenoidale . alteredconsciousness,neck stiffness
r/ subfrontalgrowth+ posteriorgrowth into sella N E C T:
turcicaand clivus r/ often normal
r/ hyperostoticblisteringof planumsphenoidale r/ increaseddensityin subarachnoidspace
(increasedvascularity),esp. in children
r/ smallventriclessecondaryto diffusecerebral
SuprasellarMeningioma edema
lncidence: 10/" of all intracranialmeningiomas
C E C T:
Origin: from arachnoid+ dura alongtuberculum r/ markedcurvilinearmeningealenhancement over
sellae/ clinoids/ diaphragmasellae/ cerebrum(frontal+ parietallobes)and
cavernoussinus with secondaryextension interhemispheric + syfvianfissures
intosella; NOT from withinpituitaryfossa r/ obliteration
., hypothalamic/ pituitarydysfunction(rare) of basalcisternswith enhancement
(common)
r/ irregularhyperostosis = blisteringadjacentto sinus MR (mostsensitivemodality):
(HALLMARKof meningiomas at planumsphenoidale i r/ no abnormality on nonenhanced MR in mostcases
tuberculumsellae) r/ hyperintenseobliteratedbasal cisternson proton-
{ pneumatosissphenoidale= increasedpneumatization
densityimages+ intermediateintensityon Tl Wl
of sphenoidin areaof anteriorclinoids+ dorsum plaqueson T2Wl
r/ hyperintense
sellae(DDx:normalvariant)
r/ leptomeningeal enhancement with Gd-DTPA(in
r/ broad base of attachment
chronicinfection)
r/ intensehomogeneous enhancement(maybe Cx:
impossibleto differentiate from supraclinoid
carotid (1) C erebri ti s
, aneurysm on CT) (2) Ventriculitis= ependymitis(secondaryto
{ bloodsupply: posteriorethmoidalbranchesof retrogradespread)
ophthalmicartery,branchesof meningohypophyseal (3) Brainatrophy
trunk (4) Braininfarction(arteritis, venousthrombosis)
MR: (5) Subduraleffusion[sterilesubduraleffusion
r/ large mass isointenseto gray matteron Tl Wl
secondaryto H. influenzaemeningitis(in children)
+ T2Wl may turn intosubduralempyemal
r/ hyperintenseflattenedpituitarygland withinfloor of (6) Hydrocephalus (cellulardebrisblockingforamenof
s ella Monro,aqueduct,4th ventricular outlet/
r/ markedhomogeneous enhancement on TlWl intraventricular septa/ arachnoidadhesions)
DDx: metastasis, glioma,lymphoma (7) Cranialnervedysfunction
302 Radiology Review Manual

Prognosis: Mechanism for excitotoxicity-induced neuronal death :

0 Cerebralinfarction+ edemaare predictiveof poor seizurescause excessiveneuronaldepolarization,
outcome which causesoverproductionof excitoryamino acid
0 Enlargementof ventricles+ subarachnoidspaces neurotransmitters,which cause excessiveactivationof
+ subduraleffusionshave no predictivevalue N-methyl-D-aspartate receptors,which cause
Mortality: 10o/"torH. influenza+ meningococcus, unregulatedentry of Ca2*,which causesneuronal
30% for pneumococcus (5thcommoncause swellingwith cytotoxicedema
of death in childrenbetween1 and 4 years r/ increasedsignalintensity+ decreasedvolumeof
of age) hippocampuscomparedto contralateralside on T2Wl
DDx: meningealcarcinomatosis Associated limbic system findings:
{ ipsilateralatrophyof fornix (55%)
{ ipsilateralatrophyof mamillarybody (26%)
Associatedextrahippocampalabnormalities:
Meningitis
Granulomatous r/ increasedsignalintensityof anteriortemporallobe
Histo: thickexudate,perivascularinflammation, cortex (38%)
granulationtissue+ reactivefibrosis { cerebralhemiatrophy (1%)
(1) Tuberculousmeningitis= basilarmeningitis:
part of generalizedmiliarytuberculosis/ primary METACH LEUKODYSTROPHY
ROMATIC
tuberculousinfection = MLD = rTlostcommonhereditary(autosomalrecessive)
(2) Sarcoidosis(in 5% of sarcoidosiscases) leukodystrophy (dysmyelinatingdisorder)
Histo: granulomatousinfiltrationof Cause: deficiencyof arylsulfataseA resultingin severe
leptomeninges deficiencyof myelinlipidsulfatidewithin
{ nodularpattern(DDx from bacterialcauses) macrophages+ Schwanncells
./ ttrict<meningealplaquesover convexities Age of presentation: before age 3 (213),
(mimickingmeningioma) in adolescence(1/3)
{ markedenhancement A. LATE INFANTILEFORM
r/ may be associatedwith single/ multiple Age: 2nd year of life
intracerebralmasses . gaitdisorder+ strabismus
Cx: cranialnervepalsy,hypothalamic-pituitary . impairmentof speech
dysfunction, chronicmeningitis . spasticity+ tremor
(3) Fungalmeningitis:cryptococcosis, candidiasis, . intellectualdeterioration
coccidioidomycosis (endemic),blastomycosis, Prognosis: death within 4 years of onset
mucormycosis (diabetics),nocardiosis, B . JU V E N ILEFOR M
actinomycosis, aspergillosis (underchronic Age: 5-7 years
corticosteroid therapy) C . A D U LTFOR M
. acute life-threatening process/ chronicindolent . organicmentalsyndrome
disease . progressivecorticospinal,corticobulbar, cerebellar,
May be associatedwith: cerebritis,abscessformation extrapyramidal signs
^/ hydrocephalus
CT: r/ progressiveloss of hemisphericbraintissue
r/ obliterationof basalcisterns,sylvianfissure, CT:
suprasellarcistern(isodensecisternssecondaryto { symmetriclow densityof white matteradjacentto
fillingwith debris) ventricles(esp.centrumovale and frontalhorns)
{ intensecontrastenhancementof gyri + involved { progressiveatrophy
subarachnoidspaces r/ no contrastenhancement
r/ calcificationof meninges MR:
r/ decreasedattenuationof white matter r/ progressivesymmetricalareas of hypointensityon
MR: TlWI
.l nign-signalintensityof basilarcisternson T2Wl r/ hyperintensityon T2Wl (increasedwater)
{ enhancement with gadopentetate dimeglumine Prognosis; death within several years
Cx: (1) Hydrocephalus(obliterationof basalcisterns;
. blockingof CSF flow + CSF absorption) METASTASES TO BRAIN
(2) Infarction(due to arteritis) lncidence: 14-37% of all intracranialtumors
0 Most commoninfratentorial mass in the
adult
Metastaticprimary:
MESIAL TEMPORAL SCLEROSIS Six tumorsaccountlor 95/" of all brain metastases:
Cause: long-standingtemporallobe epilepsy 1. Bronchialcarcinoma@7%\ RARELYsquamous
Histo: markedneuronalloss throughouthippocampal cell carcinoma
subfieldswith relativesparingof the CA2 subfield 2. Breastcarcinoma(17"/")
 BrainDisorders 303

3. Gl-tracttumors(15%): colon,rectum r/ hypointensemass relativeto edema/ variable

4. Hypernephroma (10%) intensityon T2Wl (dueto hemorrhage, necrosis,cyst
5. Melanoma(8%) formation):
6. Choriocarcinoma r/ hypointensitymore pronouncedin melanoma
I n c hildhood: + mucinousadenocarcinoma (paramagnetic effect)
1. Leuk em i a/ l y mp h o ma r/ homogeneous / ringi nodularmiied enhancement
2. Neuroblastoma after Gd-DTPA;often more than one metastaticfocus
0 Brainmetastasesfrom sarcomasare exceptionally identifiedin regionof collidingedema
rare! ! asymmetricenhancement of dura with duralspread
r/ leptomeningeal enhancement(eg, in metastatlc
Hemorrhagic Metastasesto Brain (in 34%): ependymoma)
1. M alignant melanoma DDx: glioma(indistinct border,lesswell defined,lesser
2. Choriocarcinoma amountof vasogenicedema)
3. Oat cell carcinomaof lung
4. Renalcell carcinoma MalignantMelanomaMetastaticto Brain
q Thyroidcarcinoma Prevalence: 39% at autopsy
r/ hyperdensewithoutcontrast 0 No clearconsensuson contribution of paramagnetic
r/ hypervascularwith contrast effectof blood productsversus melanin
mnemonic.' "MATCH" 1. Melanoticpattern(in Za-Sa%\
M elanom a r/ hyperintense on TlWl + iso-/ hypointense on
Anaplasticlungcarcinoma T2WI
Thyroidcarcinoma Cause: free radicalsin melanin+ blood products
Ghoriocarcinoma 2. Amelanoticpattern(38%)
Hypernephroma r/ frypo-/ isointenseon Tl Wl + hyper-/ isointense
on T2Wl
Cystic Metastasisto Brain 3. Other patterns
1. S quam ousc e l lc a rc i n o m a
of lung ri isointenseon TlWl + hyperintense on T2Wl
2. Adenocarcinoma of lung
MICROCEPHALY
Calcified Metastasis to Brain = clinicalsyndromecharacterizedby a head
1. M uc in- pr od u c i n ge o p l a s m:Gl , b re a s t circumference belowthe normalrange
2. Cartilage-i bone-forming sarcoma lncidence: 1.6:1,000or 1:6,200-1:8,500births
3. Effectiveradiochemotherapy Etiology:
(1) Undiagnosed intrauterineinfection(toxoplasmosis,
Location: rubella,CMV, herpes,syphilis),toxicagents,drugs
(a) corticomedullary junctionof brain(mostcharacteristic) hypoxia,radiation,maternalphenylketonuria
(b) subarachnoid space= carcinomatous meningitis (2) Prematurecraniosynostosis
(15%) (3) Chromosomal abnormalities (trisomies13, 19,21)
(c) subependymalspread(frequentin breastcarcinoma) (4) Meckel-Grubersyndrome
(d) skull(5%) Often associated with:
N.B.: CORTICALMETASTASES micrencephaly, macrogyria,pachygyria,atrophyof
r/ minimal/no edema basal ganglia,decreasein dendriticarborization,
1 r"y not be identifiedon T2Wl holoprosencephaly
r/ contrast-enhancement essentialfor detection ^/ nc:Hc discrepancy
Presentation: r/ headcircumference <3 S.D. belowthe mean
- multiplelesions(2lg),singlelesion(1/3) { apelikeslopingof forehead
- cerebralhemispheres (57%),cerebellum(29%), r/ dilatationof lateralventricles
brainstem(SZ%) d poor growthof fetal cranium
- nodulardepositsto dura are common ./ intracranialcontentsmay not be visible(rare)
{ usuallywell-defined roundmasses: Prognosis.' normalto severe mental retardation
d multiplelesionsof differentsizes + locations (dependingon degreeof microcephaly)
{ surrounding edemausuallyexceedstumorvolume
CT: MIN E R A LIZIN G MIC R OA N GIOP A TH Y
r/ hypodenseon NECT (unlesshemorrhagic / = RADIATION-INDUCED LEUKOENCEPHALOPATHY
, hypercellular) = s€QUelae of radiotherapycombinedwith methotrexate
r/ solidenhancementin smalltumors/ ringlike therapyfor leukemia
enhancementin largetumors lncidence; in 25-30% after >9 months after treatment
MR (combinationof r2wl + contrast-enhanced rl wl offer Age: childhood
greatestsensitivity): Cause: depositionof calciumwithinsmallvesselsof
r/ hypointenseon Tl Wl previouslyirradiatedbrainparenchyma
304 Radiology Review Manual

. 85/" withoutneurologicdeficits - reactiveastrocytosis(= gliosis), initiallywith

CT: preservationof axons (= denudedaxons)
^/ tnin reticular/ serratedlinear/ punctatecalcifications resultingin scar (= whitematterplaque)
nearcorticomedullary junction,especiallyin basal (b) chronicstage: plaquesadvanceto fibrillarygliosis
ganglia+ frontaland posteriorparietallobes with reductionin inflammatory component
{ symmetriclow-attenuation processin white matter Clinicalforms: (a) relapsingremitting
nearcorticomedullary area (b) relapsingprogressive
MR: (c) chronicprogressive
{ confluentdiffuseperiventricular spreading
distribution . waxingand waningcoursewith
peripherally with an irregularscallopededge . numbness,dysesthesia, burningsensations
. signsof brainneoplasm:headaches,seizures,
dizziness,nausea,weakness,alteredmentalstatus
DISEASE
MOYAMOYA . ataxia,diplopia
= progressiveobstructivei occlusivecerebralarteritis . optic neuritis= retrobulbarpain, centralloss of vision,
affectingdistal ICA at bifurcationinto its branches afferentpupillarydefect(MarcusGunn pupil)
(anterior213of circleof Willis),usuallyinvolvingboth . trigeminalneuralgia(1-2%)
.@:
hemispheres
Etiology: unknown (1) C N S dysfuncti on
Age: predominantly in children+ youngadults (2) involvementof two / more partsof CNS
Path: endothelialhyperplasia+ fibrosiswithout (3) predominantwhite matterinvolvement
associatedinflammatoryreaction (4) two / moreepisodeslasting>24 hourslessthan 1
. headaches monthapart
. behavioraldisturbances (5) slow stepwiseprogression of signs+ symptoms
. recurrenthemipareticattacks (6) at onset10-50 yearsof age
^/ bilateralstenosis/ occlusionof supraclinoid portionof . RudlgKred-flags, (suggestsdiagnosisotherthan MS):
internalcarotidextendingto proximalportionsof middle (1) no eye fi ndi ngs
+ anteriorcerebralarteries (2) no clinicalremission
./ large networkof vesselsin basalganglia("puffof (3) totallylocaldisease
smoke")+ upperbrainstemfed by basilarartery,anterior (4) no sensoryfindings
+ middlecerebralarteries(dilatationof lenticulostriate (5) no bladderinvolvement
+ thalamoperforating arteries) (6) no CSF abnormality
r/ anastomosesbetweendural meningeal @ Brain
+ leptomeningeal arteries 0 Number+ extentof plaquescorrelatewith duration
Cx: subarachnoid hemorrhage(occasionally) of disease+ degreeof cognitiveimpairment
Location:
subependymal periventricularlocation(alonglateral
MoyamoyaSyndrome
neurocutaneous
Etiology: syndromes aspectsof atria+ occipitalhorns),corpuscallosum,
(neurofibromatosis),bacterialmeningitis, internalcapsule,centrumsemiovale,coronaradiata,
periarteritisnodosa,head trauma, optic nerves,chiasm,optictract,brainstem
tuberculosis,oral contraceptives, (ventrolateral aspectof pons at 5th nerve root entry),
atherosclerosis,sicklecell anemia cerebellarpeduncles,cerebellum;rathersymmetric
involvementof cerebralhemispheres;subcorticalU
fibers NOT spared
MULTIPLE SCLEROSIS rl lesionsize of 1-25 (majoritybetween5 and 10) mm:
= rnostfrequentform of chronicinflammatory .i large lesionsmay masqueradeas braintumors
demyelinating diseaseof unknownetiology,which r/ lesionsusuallywithoutmass effect/ edema unless
reducesthe lipidcontentand brainvolume; acute
characterizedby a relapsing+ remittingcourse { ovoidlesions(86%)orientedwith theirlongaxis
Prevalence: 6:10,000(higherfrequencyin cooler perpendicular walls(dueto perivenous
to ventricular
climates;increasedincidencewith positive demyelination; pathologicallydescribedas "Dawson
familyhistory) fingers")
Cause: ? virali autoimmunemechanism r/ chronicplaquesdo not enhance(dueto intactblood-
Peak age: 25-30 (rangeof 20-50) years; M:F = 2:3 brain barrier)
Histo: CT:
(a) acutestage: perivenularinflammation (at junctions r/ normalCT scan (18%)
of pial veins)with ^i nonspecificatrophyof brain (45%): enlarged
- hypercellularity of lipid-laden
(= infiltration ventricles,prominentsulci
macrophages+ lymphocytes) ri periventricular(nearatria)multifocalnonconfluent
- well-demarcated demyelination(destructionof lesionswith distinctmargins(locationnot always
oligodendrogliawith lossof myelinsheath) correlatingwell with sYmPtoms)
 BrainDisorders 305

(a) NECT: isodense/ lucent Rx: usuallyresponsiveto corticosteroids

(b) CECT: transientenhancement during DDx: (1) Acutedisseminated (history
encephalomyelitis
acute stage (activedemyelination) for about of recentviralillness,monophasiccourse,
2 weeks;may requiredoubledose of contrast; lesionslessconfluent,no masseffect/
ultimatelydisappearanceI permanentscar enhancement)
MR (modalityof choice;95% specific): (2) Adrenoleukodystrophy(bilaterally symmetric,
r/ well-marginated discretefoci of varyingsize with confluentlesions,parietallocation)
high-signal intensityon T2Wl + protondensity (3) Tumor,abscess,infarct
images(= lossof hydrophobic myelinproduces
increasein watercontent);hypointense on TlWl
i/ CO-OfpAenhancement of lesionson Tl Wl (up to N E ON A TA L IN TR A C R A N IA L H E MOR R H A GE
8 weeks followingacute demyelinationwith GerminlMatrixBleed
breakdownof blood-brainbarrier) = GERMINALMATRIX_RELATED
HEMORRHAGE
r/ lesionson undersurface of corpuscallosum Germinalmatrix
(CHARACTERISTTC sagittatimages) = highlyvasculargelatinoussubependymal tissue
@ Spinalcord adjacentto fateralventriclesin which the celfsthat
0 Mostcommondemyelinating processof spinalcord! composethe brainare generated;has its largest
0 ln 12% withoutcoexistentintracranial plaquesl volumearound26 weeksGA; decreasesin size
. number+ extentof plaquescorrelatewith degreeof with increasingfetalmaturity;usuallyinvolutesby
disability 32-34 weeks of gestation
Location: predilection for cervicalregion Location: greatestportionof germinalmatrixabove
Site: eccentricinvolvementof dorsal+ lateral caudatenucleusin floorof lateral
elementsabuttingsubarachnoid space ventricle,taperingas it sweepsfrom
! atrophicplaquesorientedalongspinalcord axis frontalhorn posteriorlyinto temporal
{ lengthof plaqueusuallylessthan 2 vertebralbody horn, roof of 3rd + 4th ventricle
segments+ width less than half of cross section Arterialsupply: via Heubnerarteryfrom ACA, striate
r/ acutetumefactiveMS = cord swelling branchesof MCA,anteriorchoroidal
+ enhancement a., perforating branchesfrom
DDx: (1) Cord tumor(follow-upafter6 weekswithout meningealaa.
decreasein size of lesion) Capillarynetwork:persistingimmaturevascularrete
(2) Infection = large irregularendothelial-lined
(3) Acutetransversemyelitis(afterviralillness/ channelsdevoidof connective
vaccination) tissuesupport(collagenand
Rx: steroids(incitingrapiddecreasein size of lesions muscle)
+ lossof enhancement) Venousdrainage:terminalw., choroidalv.,
DDx: thalamostriatev. courseanteriorly
(1) Whitematterischemicdisease(patients>50 yearsof + feed into internalcerebralv.
age, lesions<5 mm, not infratentorial) which has a posteriorcourse
(2) Acutedisseminated encephalomyelitis, subacute Risk factors:
sclerosingpanencephalitis (lesionsof similarage) (1) prematurity
(3) AIDS,CNS vasculitis,migraine,radiationinjury, (2) low birthweight
lymphoma,sarcoidosis, tuberculosis,systemiclupus ( 3 ) s e x ( M : F= 2 ; 1 ;
erythematosus,cysticercosis,metastases,multifocal (4) multiplegestations
glioma,neurofibromatosis, contusions (5) traumaat delivery
(6) prolongedlabor
MYELINOCLASTIC DIFFUSE SCLEROSIS (7) hyperosmolarity
= S CHI LDE RDI S EA SE (8) hypocoagulation
= rdredemyelinating disorderwith episodicrecurrence (9) pneumothorax
and remission (10)patentductusarteriosus
Age: children> adults; M:F = 1:1 Etiology: hypoxiawith loss of autoregulation
Histo: selectiveconfluentdemyelinationwith relative Pathogenesis: ruptureof friablevascularbed due to
axonalsparing,perivascular inflammatory (1) fluctuatingcerebralbloodflow in preterminfants
infiltrate,reactiveastrocytosi
s (indistinguishable with respiratorydistress
from multiplesclerosis) (2) increasein cerebralblood flow with
. hemiplegia, aphasia,ataxia,blindness (a) systemichypertension (pneumothorax, REM
. swallowingdifficulties, progressive dementia sleep,handling,trachealsuctioning,ligationof
. increasedintracranialpressure PDA,seizures,instillation of mydriatics)
Location: centrumsemiovale (b) rapidvolumeexpansion(blood,colloid,
./ largebilateralwhite matterlesionswith mass effect hyperosmolar glucose/ sodiumbicarbonate)
r/ enhancementwith lV contrastmaterial (c) hypercarbia (RDS,asphyxia)
306 Radiology Review Manual
(3) increasein cerebralvenouspressurewith labor
and delivery,asphyxia(= ippsirmentin exchange
of oxygenand carbondioxide),respiratory
disturbances
(4) decreasein cerebralbloodflow with systemic
hypotensionfollowedby reperfusion
(5) plateletand coagulation disturbance
lncidence; in prematureneonates<32 weeks of age;
in 4 3 /" o f i n fa n ts< 1 ,5 0 0g (i n 6 5 % of
500-700g infants,in 25'h of 7Q1-1,500g
infants); in up to 50% withoutprenatal
care, in 5-10% with prenatalcare
Location: regionof the caudatenucleusand
thalamostriategroove(= caudothalamic
notch)remainsmetabolically activethe
longest;in 80-90% in infants<28 weeksof
MA age
Timeof onset: 36o/"on firstday, 32o/"on secondday,
18% on first3 day of life;bY6th daY
91% of all intracranialbleedshave
occurred
G RA DES (Papileclassification)
I subependymal hemorrhageconfinedto
germinalmatrix(GMH)on one / both sides
tl subependymal hemorrhagerupturedinto
nondilatedventricle(lVH)
ill hemorrhage(lVH)with
intraventricular
ventricularenlargementr(a) mild,
(b) moderate,(c) severe
IV extensionof germinalmatrixhemorrhageinto
brainparenchyma(lPH)
Serial scans: 5-1O-dayintervals
US (100%sensitivity+ 91o/"specificity for lesions
>5 mm; 27o/"sensitivity+ 88/" specificityfor lesions
<5 mm):
Germinal matrix hemorrhage (gradel)
r/ well-definedovoid area of increasedechogenicity
(= fibrinmeshwithinclot)inferolateral to floorof
frontalhorn+ body of lateralventricle
r/ bulbousenlargement of caudothalamic groove
anteriorto terminationof choroidplexus
DDx: choroidplexus(attachedto inferomedial
aspectof ventricularfloor,taperstoward
caudothalamic groove,neveranteriorto
foramenof Monro)
{ resolvingbleeddevelopscentralsonolucency
./ outcome: (1) completeinvolution(2) thin
echogenicscar (3) subependymal cyst
Mild intraventricular hemorrhage (gradell)
r/ echogenicmaterialfillinga portionof lateral
ventricles(acutephase)becomingsonolucentin
a few weeks
rl clot may gravitateinto occipitalhorns
r/ verticalband of echogenicitybetweenthalamion
coronalscans (bloodin 3rd ventricle)
r/ irregularbulkychoroidplexus(clot layeredon
surfaceof choroidplexus)
r/ temporarilyincreasedechogenicityof ventricular
wall (= subependymalwhite halo between7 days
and 6 weeks after hemorrhagicevent)
Extensiveintraventricularhemorrhage(gradeIll)
cast of blood distendingthe
r/ intraventricular
lateralventricles
{ + extensionof hemorrhageintobasalcisterns,
cavumseptipellucidi
r/ hemorrhagebecomesprogressivelyless
echogenic
r/ temporarily thickenedechogenicwallsof
ventricles("ventriculitis")
Intraparenchymalhemorrhage(gradelV)
Cause: (a) extensionof hemorrhageoriginating
from germinalmatrix(unusual)
(b) separatehemorrhagewithininfarcted
periventriculartissue(frequent)
Location: on side of largestamountof lVH,
commonlylateralto frontalhorns/ in
parietallobe, rare in occipitallobe
+ thalamus
ri homogeneous highlyechogenic
intraparenchymal masswith irregularmargins
r/ centralhypoechogenicity (liquefyinghematoma
after10-14 days)
r/ retractedclot settlesto dependentposition
(3-4 weeks)
r/ completeresolutionby 8-10 weeks resultsin
anechoicarea (= porencephaliccyst)
CT:
Most sensitive+ definitemeansto definesite + extent
of hemorrhage, especiallyin subduralhemorrhage,
cerebralparenchymalhemorrhage,posteriorfossa
lesion
r/ hyperdensebleedonly visibleup to 7 days beforeit
becomesisodense
Cx:
(1) Posthemorrhagic hydrocephalus (30-70%)
0 Severityof hydrocephalus directlyproportional
to size of originalhemorrhage!
Cause:
(a) temporaryblockageof arachnoidvilli by
particulateblood clot (withindays),often
transientwith partial/ total resolution
(b) obliterativefibrosingarachnoiditis oftenin
cisterna magna (within weeks); frequently
leadsto permanentprogressiveventricular
dilatation(50%)
r/ thickenedechogenicventricular walls
Time of onset: by 14 days (in 80%)
. delayedclinicalsignsbecauseof compressible
prematurebrainParenchYma
r/ ventriculardilatation,particularlyaffectingthe
occipitalhorns(amountof compressible
immaturewhite matteris largerposteriorly)
DDx: ventriculomegaly secondaryto
periventricularcerebralatrophy
(occurringslowlyover severalweeks)

(2) Cyst formation
(a) cavitationof hemorrhage
(b) unilocularsubependymal cyst
(c) unilocularporencephalic
cyst
(3) Mentalretardation,cerebralpalsy
(4) Death in 25./"(lVH most commoncause of
neonataldeath)
Prognosis:
(1) Grade | + ll: good with normaldevelopmental
scores(12-18% risk of handicap)
(2) Grade lll + lV: 54h mortality;30-40% risk of
handicap(spasticdiplegia,spasticquadriparesis,
intellectualretardation)
ChoroidPlexusHemorrhage
affectsprimarilyfull-terminfants
Cause: birthtrauma,asphyxia,apnea,seizures
r/ echogenicityof choroidplexussame as hemorrhage
r/ nodularityof choroidplexus
r/ enlargement of choroidplexus>12 mm in Ap
diameter
r/ tett-rigntasymmetry>5 mm
r/ intraventricular
hemorrhagewithoutsubependymal
hemorrhage
Cx: intraventricularhemorrhage (25%)
Intracerebel
lar Hemorrhage
Cause:
(a) fullterm infant:traumaticdelivery,intermittent
positivepressureventilation,coagulopathy
(b) prematureinfant: subependymal germinalmatrix
hemorrhageup to 30 weeks gestation
lncidence: 16-21% of autopsies
r/ echogenicityof vermissame as hemorrhage
r/ echogenicmass in lessechogeniccerebellar
, hemisphere(coronalscan most useful)
r/ nonvisualization / deformityof 4th ventricle
r/ asymmetryin thicknessof paratentorialechogenicity
is a sign of subarachnoidhemorrhage
Prognosis; poor + frequentlyfatal
Intraventricu
lar Hemorrhage
Etiology:
(a) germinalmatrixhemorrhagerupturesthrough
ependymalliningat multiplesites
(b) bleedingfrom choroidplexus
Routeof hemorrhage:blooddissipatesthroughout
ventricularsystem+ aqueductof Sylvius,passes
throughforaminaof 4th ventricle,collectsin basilar
cisternof posteriorfossa
. seizures,dystonia,obtundation,intractableacidosis
. bulginganteriorfontanel,drop in hematocrit,
bloody/
, proteinaceousCSF
\i IVH usuallyclearedwithin7-14 days
Cx: (1) lntracerebral hemorrhage
(2) Hydrocephalus
BrainDisorders 307
PeriventricularLeukoencephalopathy
Periventri cuIar LeukomaIacia
= PVL = perinatalhypoxic-ischemic encephalopathy
= principalischemiclesionof the prematureinfant
characterizedby focal coagulationnecrosisof deep
white matteras a resultof ischemicinfarction
involvingthe watershed(= arterialborder)zones
betweencentraland peripheralvascularity
Vascularsupply:
(a) ventriculopetal branchespenetratingcerebrum
from pial surfaceare derivedfrom MCA + PCA
+ ACA
(b) ventriculofugal branchesextendingfrom
ventricularsurfaceare derivedfrom choroidal
arteries+ striatearteries
lncidence:
7-22% at autopsy(88% of infantsbetween900 and
2,200g survivingbeyond6 days);in 34.h of infants
<1,500g; in 59% of infantssurvivinglongerthan
1 week on assistedventilation;only 28% detected
by cranialsonography
Histo: edema,white matternecrosis,evolutionof
cysts+ cavities/ diminishedmyelin;
nonhemorrhagic : hemorrhagic PVL = 3:1
Pathogenesis:
immatureautoregulation of periventricular vessels
secondaryto deficientmuscularisof arterioleslimits
vasodilationin responseto hypoxemia
+ hypercapnia+ hypotensionof perinatalasphyxia
(hypoxic-ischemic encephalopathy)
. "cerebralpalsy"(in 6.5% of infants<1,800g):
. spasticdiplegia(81%)> quadriparesis (necrosis
of descendingfibersfrom motorcortex)
. choreoathetosis, ataxia
. * meflt?lretardation
. severevisual/ hearingimpairment
. convulsivedisorders
Location:
bilateralwhite mattersubjacentto externalangleof
lateralventricular trigones,involvingparticularly the
centrumsemiovale(frontalhorn + body),optic
(occipitalhorn),and acoustic(temporalhorn)
radiations
US (50% sensitivity+ 87"/"specificity):
Eaflychanges(2 days to 2 weeks after insult)
ri increasedperiventricular (PVE)
echogenicity
(DDx: echogenicperiventricular halo/ blushof
fibertracts in normalneonates,white matter
gliosis,corticalinfarctionextendingintodeep
white matter)
r/ bilateraloften asymmetriczones,occasionally
extendingto cortex
./ infrequentlyaccompaniedby IVH
Late changes(1-3-6 weeks afterdevelopmentof
echodensities):
i/ periventricular cystic PVL = cysticdegeneration
of ischemicareas(= multiplesmallnever
septatedperiventricular cysts in relationshipto
lateralventricles; the largerthe echodensities,
the soonerthe cyst formation)
308 Radiology Review Manual
ri brainatrophysecondaryto thinningof
periventricular whitematteralwaysat trigones,
occasionally involvingcentrumsemiovale
r/ ventriculomegaly (afterdisappearanceof cysts)
with irregularoutlineof body + trigoneof lateral
ventricles
^/ deep prominentsulciabuttingthe ventricles
with littlei no interposedwhite matter
(DDx:schizencephaly)
^/ enlargedinterhemispheric fissure
CT (not sensitivein early phase):
r/ periventricular hypodensity (DDx: immaturebrain
with increasedwater+ incompletemyelination)
MR (notsensitivein earlyphase):
r/ hypointenseareas on Tl Wl
{ hyperintenseperiventricular signalson T2Wl in
peritrigonal region
^/ thinningof posteriorbody + spleniumof corpus
callosum(= degeneration fibers)
of transcallosal
Prognosis:
majorneurologicprobtemi deathin up to 62h;
PVL localizedto frontallobesshow relativenormal
development; generalizedPVL resultsin neurologic
deficitsin closeto 100%
DDx: tissuedamagefrom ventriculitis (sequelaeof
meningitis), metabolicdisorders,in utero
ischemia(eg, maternalcocaineabuse)
Perivent ri cuIar Hemorrhagi c Infarcti on
= hemorrhagic necrosisof periventricularwhite
matter,usuallylarge+ asymmetric
lncidence; in 15-25% of infantswith IVH
Pathogenesis:
(a) germinalmatrixhemorrhagewith intraventricular
bloodclot (in 80%)
(b) ischemicperiventricular leukomalacia
leadto obstruction of terminalveinswith sequence
of venouscongestion+ thrombosis+ infarction
Histo: perivascular hemorrhageof medullaryveins
nearventricularangle
Associatedwith: the most severe cases of
hemorrhage
intraventricular
Age: peak occurrenceon 4th postnatalday
. spastichemiparesis(affectinglower + upper
extremities equally)/ asymmetricquadriparesis
(in 86% of survivors)
Location: lateralto externalangle of lateral
ventricleon sideof more markedIVH:
67"/ounilateral;33% bilateralbut
asymmetric
Earlychanges(hoursto days after major tVH):
"fan-
r/ unilateral/asymmetricbilateraltriangular
shaped"echodensities
r/ extensionfrom frontalto parieto-occipital
regions
in anteriorportionof
/ localized(particularly
lesion)
Latechanges:
r/ singlelargeclst = porencephaly
r/ bumpyventricle/ false accessoryventricle
Prognosis: 59ohoverallmortalitywith echodensities
>1 cm; in 64'/" major intellectualdeficits
Encephalomalacia
= nore extensivebraindamagethan PVL; may
includeall of white matterin subcortex+ cortex
Associated with:
(1) Neonatalasphyxia
(2) Vasospasm
(3) Inflammation of CNS
{ smallventricles(edema)with diffusedamage
{ increasedparenchymal echogenicity makingit
difficultto definenormalstructures
^/ decreasedvascularpulsations
^/ transcranialDoppler:
(a) groupI (goodprognosis)
^/ normalflow profile,normalvelocities,normal
resistiveindex
(b) groupll (guardedprognosis)
{ increasein peak-systolic+ end-diastolicflow
velocities+ decreasedresistiveindex
(c) group lll (unfavorableprognosis)
r/ reduceddiastolicflow + decreasedpeak
systolicand diastolicvelocities+ increased
resistiveindex
r/ ventricularenlargement+ atrophy
r/ extensivemulticysticencephalomalacia with cysts
oftennot communicating
NEUROBLASTOMA
Age at presentation: <2 years (50%); <4 years (75%);
<8 years (90%);peak age <3 years
. abdominalmass (45%)
. neurologicsigns(20%)
. bone pain llimp (20%)
. orbitalecchymosis / proptosis(12%)
. catecholamineproduction(95%)with paroxysmal
episodesof flushing,tachycardia, hypertension,
headaches,sweating,intractablediarrhea,acute
cerebellarencephalopathy
. positivebone marrowaspiration(70%)
Location: adrenalgland(67%),chest(13%),neck(5%),
intracranial (2%);commonlyinvolvement of
multipleskeletalsites
NUC (overallsensitivityof detectionbetterthan
radiography):
CAVE: symmetriclyticneuroblastomametastases
occurfrequentlyin metaphysealareaswhere
normalepiphysealactivityobscureslesions
^/ purelylytic lesionsmay presentas photopenicareas
{ softtissue uptakeof Tc-99mphosphatein 60%
^/ frequentlyGa-67 uptakein primarysite of
neuroblastoma
Prognosis: Z-yearsurvival(a) in 60% for age <1 year
(b) in 20"/"tor ages 1-2 years (c) in 10o/"tor
ages >2 years
A. PRIMARYCEREBRALNEUROBLASTOMA (rare)
Age: childhoodI earlyadolescence
{ largehypodenseI mixed-density masswith well-
definedmargins
 BrainDisorders 309

r/ intratumoralcoarsedense calcifications r/ intraspinal

lipoma
{ centralcystic/ necroticzones with hemorrhage Prognosis.' rapid deterioration+ death within 3 years of
Cx: metastasizesvia subarachnoidspaceto dura diagnosisdue to development of malignant
+ calvarium melanoma/ complication of hydrocephalus
B. SECONDARYNEUROBLASTOMA (common)
metastaticto:
@ liver NEUROFIBROMATOSIS
@ skeleton = autosomaldominantinheriteddisorder,probablyof
r/ osteolysiswith periostealnew-boneformation neuralcrest originaffectingall 3 germ cell layers,
{ suturaldiastasis capableof involvingany organsystem
^/ hair-on-endappearanceof skull Path: frequentlycombinationof
@ orbit: (1) pure neurofibromas (= tumorof nervesheath
r/ unilateralproptosis with involvementof nerve,nervefibers run
0 Neuroblastoma usuallynot metastaticto brain! throughmass)
(2) neurilemomas (= flervefibersdivergeand
OlfactoryNeuroblastoma courseover the surfaceof the tumor mass)
= vefy malignanttumorarisingfrom olfactorymucosa AS
Types: 1. Esthesioneuroepithelioma (a) localizedneurofibroma (mostcommon)
2. Esthesioneurocytoma Location: dermis+ subcutaneous tissue
3. Esthesioneuroblastoma = fibromamolluscum
./ mass in superiornasalcavitywith extensioninto (b) diffuseneurofibroma
ethmoid+ maxillarysinuses (c) plexiformneurofibroma = tortuoustangles/
Cx: distantmetastasesin 20% fusiformenlargement of peripheralnerves
(P A TH OGN OMON IC of N F1)
0 Often precedesdevelopmentof cutaneous
NEUROCUTANEOUS
MELANOSIS neurofibromas!
= tara sporadiccongenitalsyndromecharacterizedby Histo: proliferationof fibroblasts+ Schwanncells
largemultiplemelanocytic nevi(in 5-15%) + melanotic 0 Morefrequentinvolvement of deep largenerves(sciatic
lesionsof CNS (in a0-60%) nerve,brachialplexus)in NF1 in contradistinction to
Age: first 2 years of life (most);2nd l3rd decade(less isolatedneurofibromas withoutNF1|
) ; : F= 1 : 1
c o m m o n l yM
Cause: abnormalmigrationof melanocyteprecursors,
abnormalexpression of melanin-producinggenes PeripheralNeurofibromatosis(90%)
withinleptomeningeal cells,rapidproliferation
of = NEUROFIBROMATOSIS TYPE1 = NF1
melanin-producing leptomeningeal cells = VONRECKLINGHAUSEN DISEASE
Histo: abnormalabundanceof melanoticcells (whichare = dysplasiaof mesodermal+ neuroectodermal tissue
normallyfoundin basilarleptomeninges) with with potentialfor diffusesystemicinvolvement;
concomitantinfiftrationof perivascularspaces autosomaldominantwith abnormalitieslocalizedto
. increasedintracranialpressure the pericentromeric regionof chromosome17 (siteof
.. seizures,ataxia,cranialnerveVl + Vll palsies tumorsuppressorgene neurofibromin) ;
r/ frighattenuationof melaninpigmentson CT scan 50% spontaneousmutants;variableexpressivity
r/ hyperintense on T1Wl, hypointense on T2Wl mnemonic: von Recklinghausenhas lT letters
(paramagneticeffectof oxygen{ree radicalsin melanin) l nci dence: 1:2,000-4,000; M:F = 1:1; mostcom m on
r/ leptomeningeal melanosis= foci of abnormally of phakomatoses
thickenedleptomeninges 0 One of the mostcommongenetic
Location: inferiorsurfaceof cerebellum;inferior diseases!
surfaceof frontal,temporal,occipitallobes; Predisposing factor:
ventralaspectof pons, cerebra;peduncles, advancedpaternalage >35 years (2-foldincreasein
uppercervicalspinalcord new mutations)
i/ parenchymal melanosis(lesscommon) Diagnosticclinicalcriteria(at least two must be present):
Location: cerebellum, anteriortemporallobes (1) >6 caf6-au-laitspots>5 mm in greatestdiameter
(esp.amygdata) (>15 mm in postpubertal individuals)
r/ franklyhemorrhagicnecroticinvasivemass with (2) >2 subcutaneousneurofibromasof any type / one
transformation into malignantmelanoma plexiformneurofibroma
./ hydrocephalus (3) axillary/ inguinalfreckling
r/ posteriorfossa cyst (4) opticnerveglioma
r/ cerebellarhypoplasia (5) >2 Lischnodules(= pigmentediris hamartomas)
r/ Dandy-Walkermalformation (6) characteristicskeletallesions(eg,sphenoid
! syringomyelia dysplasia/ thinningof longbonecortex)
r/ intraspinalarachnoidcyst + pseudarthrosis
310 RadiologyReview Manual

relative(parent,sibling,child)with
(7) first-degree 7. CNS hamartomas(up to 75-90%)
peripheralneurofibromatosis = probablydysmyelinating lesions(may
resolve)
CLA S S I CT RI A D : Location: pons,basalganglia(most
( 1) Cut aneo u sl e s i o n s commonlyin globuspallidus),
(2) Skeletaldeformity thalamus,cerebellarwhitematter
0 Musculoskeletal predominatein
abnormalities ! muftiplefoci of isointensity on TlWl
NF1! + hyperintensity on T2Wl withoutmass
(3) Mentaldeficiency effect(= "unidentifiedbrightobjects")
May be associated with: 8. Vacuolar/ spongioticmyelinopathy(in 66%)
(1) MEA llb (pheochromocytoma + medullary Location: basalganglia(esp.in globus
carcinomaof thyroid+ multipleneuromas) pallidus),cerebellum,internal
(2) CHD (10 fold increase):pulmonaryvalve capsule,brainstem
stenosis,ASD, VSD, IHSS { nonenhancing hyperintense foci on T2Wl
A. CNS MANIFESTATIONS @ Spinal cord
@ Intracranial 1. Paraspinalneurofibromas
1. Opticpathwayglioma { tumorsof varyingsizes at nearlyevery level
isolatedto singleoptic nerve+ extensionto throughoutthe spinalcanal
other optic nerve,chiasm,optictracts r/ enlargement of neuralforaminadue to
"dumbbell"neurofibroma of spinalnerves
Histo: pilocyticastrocytomawith perineural/
subarachnoidspread(opticnerve is r/ fusiform/ sphericalmass:
embryologically part of hypothalamus { low-attenuation mass (20-30 HU) in up to
and developsgliomasinsteadof 73/" due to cysticdegeneration,
schwannomas) xanthomatousfeatures,confluentareas of
0 In up to 30% of all neurofibromatosis hypocellularity, lipid-richSchwanncells
patients r/ areasof higherattenuationdue to densely
0 10% of all optic nervegliomasare cellularcomponents1 collagen-rich regions
associatedwith neurofibromatosis r/ slightlyhyperintense to muscleon T1Wl,
hyperintense periphery+ hypointense core on
2. Cerebralgliomas T2WI
astrocytomasof tectum,brainstem, ./ hypoechoicwell-circumscribed cylindrical
gliomatosis cerebri(= Unusualconfluenceof lesion
astrocytomas) ! spinalcord displacedto contralateralside
3. Hydrocephalus 2. Lateral/ anteriorintrathoracicmeningocele
obstructionusuallyat aqueductof Sylvius = diverticulaof thecalsac extendingthrough
Cause: benignaqueductalstenosis,glioma widenedneuralforamina/ defectsin vertebra
of tectum/ tegmentumof Cause: dysplasiaof meningesfocallystretched
mesencephalon by CSF pulsations(dueto Pressure
4. Vasculardysplasia differencesbetweenthorax
= occlusion/ stenosisof distal internalcarotid + subarachnoid spacesuperimposed
artery,proximalmiddle/ anteriorcerebral on bone vertebraldefect)
artery Location: thoraciclevel (mostcommon)
r/ moyamoyaphenomenon(60-70%) ./ erosionof bony elementswith marked
5. Neurofibromas (= arisingfrom Schwanncells posteriorscalloPing
+ fibroblasts) of cranialnerveslll-XIl (most ./ wideningof neuralforamina(due to protrusion
c ommo n l yV + V l l l ) of spinalmeninges)
0 30% of patientswith solitaryneurofibromas DDx: mediastinalilungabscess
have NF1 B. SKELETALMANIFESTATTONS (in 25-a0%)
0 Virtuallyall patientswith multiple . dwarfismcausedby scoliosis
neurofibromas have NF1 @ Orbit
6. Craniofacial plexiformneurofibromas r/ Harlequinappearanceto orbit = partial
= locallyaggressivecongenitallesion absenceof greaterand lesserwing of
composedof tortuouscords of Schwann sphenoidbone + orbitalplate of frontalbone
cells,neurons+ collagenwith progression (failureof development of membranousbone)
along nerveof origin(usuatlysmall ./ hypoplasia+ elevationof lesserwing of
unidentified nerves) sphenoid
Location: commonlyorbitalapex,superior ./ defectin sphenoidbone+ extensionof middle
orbitalfissure cranialfossa structuresinto orbit
0 Plexiformneurofibromasare ./ concentricenlargementof opticforamen(optic
PATHOGNOMONIC for NF1 gl i oma)
 BrainDisorders 311

r/ enlargement of orbitalmargins+ superior r/ atrophicthinned/ absentfibulas

orbitalfissure(plexiformneurofibroma of r/ periostealdysplasia= traumaticsubperiosteal
peripheraland sympatheticnerveswithin orbit/ hemorrhagewith abnormaleasy detachmentof
opticnerveglioma) periosteumfrom bone
r/ sclerosisin the vicinityof opticforamen(optic r/ subendostealsclerosis
nervesheathmeningioma) ^/ bone erosionfrom periosteali soft-tissue
r/ deformity+ decreasedsize of ipsilateral neurofibromas
ethmoid+ maxillarysinus ./ intramedullary longitudinal
streaksof increased
@ Skull density
r/ macrocranium+ macroencephaly r/ multiplenonossifying fibromas/ fibroxanthomas
r/ left-sidedcalvarialdefectadjacentto lambdoid r/ single/ multiplecysticlesionswithinbone
suture= parietalmastoid(rare) (? deossification/ nonossifyingfibroma)
@ S pine ri focal gigantism= unilateral-overgrowth of a
{ sharplyangledfocalkyphoscoliosis (50%)in limb bone;markedenlargement of a digitin a
lowerthoracic+ lumbarspine;kyphosis hand / foot (overgrowthof ossificationcenter)
predominates over scoliosis;incidence
increaseswith age C . N E U R A LC R E S TTU MOR S
Cause: abnormaldevelopmentof vertebral 1. Pheochromocytoma: . hypertension in adults
bodies 2. Parathyroid adenomas: . hyperparathyroidism
r/ hypoplasiaof pedicles,transverse+ spinous D . V A S C U LA RLE S ION S
processes Schwanncell proliferation withinvesselwall
{ posteriorscallopingof vertebralbodiesdue to 1. Cranialarterystenosis
dural ectasia(secondaryto weakened 2. Renalarterystenosis:very proximal,funnel-
meningesallowingtransmission of normalCSF shaped(oneof the mostcommoncausesof
pulsations) hypertension in childhood)
r/ dumbbell-shaped enlargement of neural 3. Renalarteryaneurysm
foramina 4. Thoracic/ abdominalaorticcoarctation
@ Chestwall E. Gl TRACTMANTFESTATTONS (10-25%)
r/ numeroussmallwell-defined subcutaneous . pain,intestinalbleeding
neurofibromas . obstruction(simulatingHirschsprung diseasewith
r/ twisted"ribbonlike"ribs in upperthoracic plexiformneurofibromas of colon)
segmentsdue to bonedysplasia/ multiple Location: jejunum> stomach> ileum>
neurofibromasof intercostalnerves: duodenum; retroperitoneal / paraspinal
r/ localizedcorticalnotches/ depressionof Associatedwith: increasedprevalenceof carcinoid
inferiormarginsof ribs tumors+ Gl stromaltumors
(DDx:aorticcoarctation) (a) solitarypattern= singleneurofibroma, neuroma,
d chestwall mass invading/ eroding/ destroying ganglioneuroma, schwannoma
adjacentribs r/ subserosal/ submucosalfillingdefect
@ Lung ("mucosalganglioneu rofibromatosis")
., exertionaldyspnea (b) plexiformpattern= regionalenlargementof nerve
{ intrathoracic lateral+ anteriormeningoceles root trunks
r/ peripheralpulmonarynodule= pedunculated r/ mass effecton adjacentbarium-filledloops
intercostalneurofibromas r/ multipleeccentricpolypoidfillingdefects
r/ progressive pulmonaryinterstitial fibrosiswith involvingmesentericside of smallbowel
, lowerlungfieldpredominance (in up to Z0%) r/ mesentericfat trappedwithinentangled
r/ largethin-walledbullaewith asymmetricupper network(15-30 HU) CHARACTERISTTC
lobepredominance r/ multipleleiomyomas+ ulcer
@ Mediastinum Cx: intussusception
0 Neurogenictumorsaccounttor g"/"of primary F. GENITOURINARY MANIFESTATIONS (rare)
mediastinalmassesin adults+ 30% in children 1. Renalarterystenosis
r/ mediastinalmasses: r/ plexiformneurofibroma withvascularnarrowing
r/ well-marginated smoothround/ ellipticmass 2. Urinarybladdermass
r/ extensivefusiform/ infiltratingmass Origin: vesicoprostatic (male)/urethrovaginal
r/ paravertebralneurofibromas plexus(female)
@ Appendicular skeleton . symptomsof urinarytractobstruction:
r/ anterolateralbowingof lower half of tibia (most frequency,urgency,incontinence, hematuria,
common)/ fibula(frequent)/ upperextremity abdominalpain
(uncommon)secondaryto deossification r/ solitaryhypoechoicbladderwall mass
r/ pseudarthrosisafter bowingfracture r/ dittusebladderwall thickening;mass may
(particularlyin tibia)in 1st year of tife surrounduterus,vagina,sigmoidcolon
312 RadiologyReview Manual

"MlSME"
G. OCULARMANIFESTATIONS (6%) mnemonic.'
. pulsatileexophthalmos / unilateralproptosis MultipleInheritedSchwannomas
(herniation of subarachnoid space+ temporallobe Meni ngi omas
into orbit) Ependymomas
. buphthalmos
1. Plexiformneurofibroma (mostcommon) lncidence:1:50,000births
2. Pigmentediris hamartomas<2 mm (Lisch Etiotogy: deletionon the long arm of chromosome22;
nodules)in >907o,mostlybilateral;appear in in 50% new spontaneousmutation
childhood ; asymptomatic 0 Neurofibromatosis 2 is locatedon
3. Opticglioma: in 12/" of patients,in4/" bilateral; chromosome221
75'/. in 1st decade Symptomaticage: during 2nd lSrd decadeof life
{ extensionintoopticchiasm(up to 25%),optic Diagnostic criteria:
tracts + optic radiation ('1)bilateral8th cranialnervemasses
^/ increasedintensityon T2Wl if chiasm+ visual (2) first-degreerelativewith unilateral8th nerve mass,
pathwaysinvolved neurofibroma, meningioma, glioma(spinal
4. Periopticmeningioma ependymoma), schwannoma, juvenileposterior
5. Choroidalhamartoma:in50"/" of patients subcapsularlenticularoPacitY
H. S K I NM A N IF ES T AT ION S . NO Lischnodules,skeletaldysplasia,opticpathway
1. Caf6-au-lait spots glioma,vasculardysplasia,learningdisability
of "coastof California" type (= smoothoutline): . caf6-au-lait spots(<50%): pale,<5 in number
>6 in number>5 mm in greatestdiameter . cutaneousneurofibroma:minimalin size + number/
usuallydevelopwithin1styear of life/ >15 mm absent
in size in postpubertal individuals @ Intracranial
Histo:increasedmelaninpigmentin basal 1. Bilateralacousticschwannomas(srnequa non)
epidermallayer Site: superiorI inleriordivisionof vestibularn'
DDx: tuberoussclerosis,fibrousdysplasia r/ usuallyasymmetricin size
Location: axillaryfreckling(in 66%) 2. Schwannomaof othercranialnerves
Extent: often parallelsdiseaseseverity Frequency; trigeminaln. > facialn.
2. Cutaneousneurofibroma 0 NerveswithoutSchwanncellsare excluded:
beginto appeararoundearlychildhood/ puberty olfactorynerve,oPticnerve
subsequentto detectionof caf6-au-laitspots: 3. Multiplemeningiomas:intraventricular in choroid
(a) localized= fibroma mollusculrl = stringof plexusof trigone,parasagittal, sphenoidridge,
pearlsalongperipheralnerve olfactorygroove,along intracranialnerves
' firmwell-circumscribed movabletumor 4. Meningiomatosis = dura studdedwith
(b) plexiformneurofibroma = multilobulated i nnumerablsmal
e lmeni ngi omas
tortuousentanglement / interdigitating 5. Gliomaof ependYmal derivation
networkof tumor along a nerve + its branches @ S pi nal
. soft grittyoften hyperpigmented tumor . symptomsof cord comPression
feelinglike a "bag of worms"/ braidedropes A. Extramedullary
. may becomevery largehangingin a 1. Multipleparaspinalneurofibromas
pendulousfashionassociatedwith massive 2. Meningiomaof spinalcord (thoracicregion)
disfiguring enlargement of an extremity B. lntramedullarY
'1.
(= elephantiasis neuromatosa) Spinalcord ependymomas
{ + osseoushypertrophy (dueto chronic
hyperemia)
Rapid episodes of growth of neurofibromas: NEUROMA
puberty,pregnancy,malignancy Prevalence: 8"/"of all intracranialtumors
Age: 20-50 years
Cx: to malignant
malignanttransformation . slow growth;not painful
neurofibromas+ malignantschwannomas
(2-5-29%),glioma,xanthomatous leukemia VestibularSchwannoma
= ACOUSTIC NEUROMA = ACOUSTIC SCHWANNOMA
= N E U R ILE MMOMA
with BilateralAcoustic
Neurotibromatosis 0 Most common neoplasmof internalauditorycanal /
Neuromas cerebellopontine angle!
= NEUROFIBROMATOSIS TYPE2 = NF2= CENTRAL Prevalence: 6-10% of all intracranialtumors;85% of all
NEUROFIBROMATOSIS intracranialneuromas;60-90% of all
= tara autosomaldominantsyndromecharacterizedby cerebellopontineangletumors
propensityfor developingmultipleschwannomas, Age: (a) sporadictumor: 35-60 years; M:F = 1:2
meningiomas,and gliomasof ependymalderivation (b) type 2 neurofibromatosis:2nd decade

Histo:
encapsulated neoplasmcomposedof proliferating
fusiformSchwanncells with
(a) highlycellulardenseregions(AntoniA) with
reticulin+ collagen,and
(b) looseareaswith widelyseparatedcells (AntoniB)
in a reticulatedmyxoidmatrix;common
degenerativechangeswith cyst formation,
vascularfeatures,lipid-laden foam cells
May be associatedwith: central neurofibromatosis
0 Solitaryintracranialschwannomais associatedwith
type 2 neurofibromatosis in 5-25/.1
0 Bilateralacousticschwannomas allowa
presumptive diagnosisof type 2 neurofibromatosis!
. longhistoryof slowlyprogressive unilateral
sensorineural hearinglossaffectinghigh{requency
soundsmore severely(in 95%)
. t innit uspain
,
. diminishedcornealreflex
. unsteadiness, vertigo,ataxia,dizziness(<10%)
Doubling time: 2 years
Location:
(a) arisesfromwithininternalauditorycanal(lAC)in
80% /cochlea
(b) may arisein cerebellopontine anglecisternat
openingof IAC (= porusacusticus)with
intracanalicularextensionin 5"/.
Site: (a) in 85% from the vestibularportionof 8th nerve
(aroundvestibularganglionof Scarpa/ at the
glial-Schwann celljunction)posteriorto
cochlearportion
(b) in 15'/.fromthe cochlearportion
ri roundmasscenteredon longaxisof IAC forming
acute angleswith dural surfaceof petrousbone
r/ funnel-shaped componentextendinginto IAC
^/ tnC enlargement/ erosion(70-907j
r/ widening/ obliteration of ipsilateral
cerebellopontine
anglecistern
r/ shift/ asymmetryof 4th ventriclewith hydrocephalus
r/ degenerativechanges(cysticareas+ hemorrhage)
with tumors>2-3 cm
P lainf ilm :
r/ flaringporus acusticus
! erosionof IAC: a differencein canalheightof >2
mm is abnormal+ indicatesa schwannomain gTy"
CT :
./ isodensesmall/ hypodenselargesolidtumor
{ cystformationin tumor(= centril necrosisin 15/.
of largetumors)/ coexistentextramuralarachnoid
cyst adjacentto tumor
{ usuallyuniformlydensetumorenhancement with
small tumors(50% may be missedwithoutCECT)/
ringenhancement with largetumors
ri tttOcalcification
ri intrathecalcontrast/ carbondioxideinsufflation(for
tumors<5 mm)
MR (mostsensitivetestwith Gd-DTPAenhancement):
r/ iso-i slightlyhypointense on Tl Wl relativeto brain
{ intenselyenhancinghomogeneousmass/ ringlike
enhancement(if cystic)after Gd-DTPA
BrainDisorders 313
r/ hyperintense on T2Wl (DDx: meningiomaremains
hypo-/ isointense)
Angio:
ri elevation+ posteriordisplacementof anterior
inferiorcerebellarartery(AICA)on basalview
r/ elevationof the superiorcerebellarartery(large
tumors)
r/ displacementof basilararteryanteriorly/ posteriorly
+ contralateralside
./ compression/ posterior+ tateraldisplacementof
petrosalvein
r/ posteriordisplacement of choroidpointof PICA
r/ vascularsupplyfrequentlyfrom externalcarotid
arterybranches
r/ rarelyhypervascular tumorwithtumorblush
DDx: ossifyinghemangioma(bonyspiculations)
TrigeminalNeuroma
= TRIGEMINAL SCHWANNOMA
lncidence: 2-5% of intracranialneuromas,0.267oof
all braintumors
Origin: arisingfrom gasserianganglionwithin Meckel
cave at the most anteromedialportionof the
petrouspyramid/ trigeminalnerveroot
Age: 35- 60 years; M:F = 1:2
Symptomsof locationin middlecranialfossa:
. facialparesthesia/ hypesthesia
. exophthalmos, ophthalmoplegia
Symptomsof locationin posteriorcranialfossa:
. lacial nerve palsy
. hearingimpairment, tinnitus
. ataxia,nystagmus
Location: (in any segmentof trigeminalnerve)
(a) middlecranialfossa(46%)= gasS€fianganglion
(b) posteriorcranialfossa (29%)
(c) in both fossae (25%)
(d) pterygoidfossa lparanasalsinuses(10%)
r/ erosionof petroustip
r/ enlargement of contiguousfissures,foramina,canals
r/ dumbbell/ saddle-shaped mass (extensioninto
middlecranialfossa+ throughtentorialincisurainto
posteriorfossa)
r/ isodensemasswith denseinhomogeneous
enhancement(tumornecrosis+ cyst formation)
r/ distortionof ipsilateralquadrigeminal cistern
r/ displacement+ cutoffof posterior3rd ventricle
r/ anteriordisplacement of temporalhorn
r/ angiographically avascular/ hypervascularmass
OLIGODENDROGLIOMA
= uficorTlrTlonform of slowlygrowingglioma;presenting
with largesize at time of diagnosis
lncidence: 2-10"/. of intracranialgliomas;5-7.h of all
primaryintracranial neoplasms
Histo: mixedglial cells (50%),astrocyticcomponents
(30%);hemorrhage+ cyst formationinfrequent
Age: 30-50 years; adult:child = 8:1
. seizures
314 RadiologyReview Manual

Location: mostcommonlyin cerebralhemispheres r/ mild/ moderateprominenceof corticalsulci

(propensityfor peripheryof frontallobes) Prognosis; death in adolescence/ early adulthood
involvingcortex+ whitematter,thalamus,
corpuscallosum;occasionally around/ in P IC K D IS E A S E
ventricles("subependymal oligodendroglioma") = tata form of preseniledementiasimilarto Alzheimer
rare in cerebellum+ spinalcord disease;may be inheritedwith autosomaldominant
r/ large nodularclumpsof calcifications(in 45% on plain mode;M < F
film; in 70-90% on CT) r/ focal corticalatrophyof anteriorfrontal+ anterior
CT: temporallobes
r/ roundI ovalhypodenselesionwith mass effect(75%) r/ dilatationof frontal+ temporalhornsof lateralventricle
{ commonlyno / minimaltumorenhancement (75/"),
pronouncedin high-gradetumors
{ may be adherentto dura (mimickingmeningiomas) PINEAL CYST
r/ + erosionof innertable of skull = Str't?llnonneoplasticcyst of pinealgland
{ cysticchanges(uncommon) lncidence: 2540% on autopsy,4o/oon MRI
./ edema(in 50% of low-grade,in 80% of high-grade Types:
tumors) (a) developmental= persistenceof ependymal-lined
MR: pinealdiverticulum
r/ well-circumscribed heterogeneoushypo-/ isointense (b) degenerative = glial-lined
secondarycavitationwithin
lesionon Tl Wl + hyperintense foci on T2Wl area of gliosis
r/ tittteedema/ mass effect ' neverassociatedwith Parinaudsyndrome
r/ solid/ peripheral/ mixedmoderateenhancement . rarelycause of hydrocephalus(compression/ occlusion
r/ calcificationmay not be detected of aqueduct)
Cx: malignantmetaplasia+ CSF seeding . may be symptomaticwhen large
Prognosis: 46/" 1O-yearsurvivalrate with low-grade; r/ + calcification
20/" |O-yearsurvivalrate with high-grade CT:
DDx: (1) Astrocytoma(no largecalcifications) r/ normal-sized gland(80%),slightly>1 cm in 20o/",can
(2) Ganglioglioma (in temporallobes+ deep be >2 cm in size
cerebraltissues ./ isodenseto CSF in surroundingcistern(infrequently
(3) Ependymoma(enhancingtumor,oftenwith noted)
internalbleedingproducingfluid levels) M R :
(4) Glioblastoma (infiltrating,
enhancing,edema,no sharplymarginatedovoidmass in pinealregion
calcifications) slightimpressionon superiorcolliculi(sagittalimage)
isointenseto CSF on TlWl + slightlyhyperintense to
PARAGONIMIASIS OF BRAIN CSF on T2Wl (due to phase coherencein cysts but
= Orientallungfluke(Paragonimus westermani)producing not in movingCSF)
arachnoiditis, parenchymal granulomas, encapsulated may have highersignalintensitythan CSF due to high
abscesses proteincontent
r/ isodense/ inhomogeneous masses surrounded by contrast may diffusefrom enhancedrim of residual
edema pinealtissueintofluidcenter(no blood-brain barrier)
r/ ring enhancement on delayed sequence images
Prognosis;lackof growthoverlongtime

ER DISEASE
PELIZAEUS.MERZBACH PINEALGERMINOMA
= f?r€ X-linkedsudanophilicleukodystrophy(5 types with = DYSGERMINOMA = PINEALOMA = ATYPICAL TERATOMA
differenttimes of onset,rate of progression,genetic (formerinaccurate names)
transmission) 0 "pinealoma"= misnomer to anypinealmass
referring
Age: neonatalperiod = maligndntprimitivegerm cell neoplasm
. bizarrependularnystagmus+ headshaking lncidence: most commonpinealtumor (>50%of all
. cerebellarataxia pinealtumors,66% of pinealgerm cell
. slow psychomotordevelopment tumors)
CT: Histo: identicalto testicularseminoma+ ovarian
r/ hypodensewhite matter dysgerminoma, NO capsulefacilitatesinvasion
r/ progressivewhite matteratrophy A ge: 10-25years; M:F = 10:1to 33:1
MR: May be associatedwith: ectopic pinealoma= secondary
r/ lack of myelination(appearanceof newbornretained) focusin inferiorportionof
^/ hyperintense internalcapsule,opticradiations, 3rd ventricle
proximalcoronaradiataon TlWl . precociouspubertyfrequentin children<10 years of age
{ nearcompleteabsenceof hypointensity in . Parinaud syndrome = paralysisof upwardgaze
supratentorialregionon T2Wl (compression of mesencephalic tectum)
 BrainDisorders 315

Locationof germinomas: pinealgland(80%),suprasellar r/ may show heterogeneous / rimlikecontrast

region(20%),basalganglia, enhancement(limitedto solid-tissue areas)
thalamus Angio:
./ displacementof calcifiedpinealgland r/ elevationof internalcerebralvein
./ hydrocephalus(compressionof aqueductof Sylvius) r/ posteriordisplacementof precentralvein
{ well-definedlesionrestrictedto pinealgland C T:
{ may infiltratequadrigeminal plate/ thalamus r/ heterogeneousmass with fat, calcification,
cystic
CT: + solid areas
r/ infiltratingvariodense/ frequentlyhyperdense MR:
homogeneous mass (attenuation usuallysimilarto r/ variegatedappearanceon all pulsesequences:
gray matter) { hyperintenseareasof fat on Tl Wl with chemical
{ rarelypsammomatous withintumor,but
calcifications shift artifact
pinealcalcificationsin 100%(40%in normal Cx: chemicalmeningitis with spontaneousrupture
population)
r/ moderate/ markeduniformcontrastenhancement
MR: PINEALCELLTUMORS
./ round I lobularwelt-circumscribedrelatively { similarimagingappearance
homogeneousmass isointenseto gray matter: ./ peripherallydisplacedpreexistingnormalpineal
r/ intermediateintensityon Tl Wl calcificatiofl(= "explodedpinealpattern")
, r/ slightlyhypointensemass on T2Wl (occasionally)
! strongGd-DTPAenhancement
Cx: CSF seeding(frequent,CSF cytologymore Pineoblastoma
sensitivethan imaging,contrastMR of entire = highlymalignanttumorderivedfrom primitivepineal
neuroaxis) parenchymal cells
Rx: combinationof irradiation(veryradiosensitive) Histo: unencapsulated highlycellularprimitivesmall
+ chemotherapy(Adriamycin@, cisplatin, roundcelltumor(similarto medulloblastoma,
cyclophosphamide) neuroblastoma,retinoblastoma)
Prognosis: 75o/osurvivalafter radiationtherapy alone Age: any age, morecommonin children;M < F
{ usuallylargemass
PIN EAL TERATOCARCI NOMA C T:
= highlymalignantvariantof germ cell tumors r/ poorlymarginatediso- / slightlyhyperdensemass
Types: 1. Choriocarcinoma r/ may containdense tumor calcifications
2. Embryonalcell carcinoma ^/ intensehomogeneous contrastenhancement
3. Endodermalsinustumor MR:
Histo: arisingfrom primitivegerm cells,frequently ^/ iso-/ moderatelyhypointense on TlWl + iso-/
containingmorethan one cell type hyperintenseon T2Wl
Age: <20 years; males { densehomogeneous Gd-DTPAenhancement
. Parinaudsyndrome Spread:
. tumor markerselevatedin serum + CSF (1) directextensionposteriorlywith invasionof
r/ intratumoralhemorrhage(esp.choriocarcinoma) cerebellarvermis+ anteriorlyinto 3rd ventricle
r/ invasionof adjacentstructures (2) CSF seeding(frequent)alongmeninges/ via
{ intensehomogeneouscontrastenhancement ventricles
Cx: seedingvia CSF

PINEALTERATOMA Pineocytoma
= benigntumorcontainingone / all threegermcell layers = fdre slow-growingunencapsulated tumor composed
(pinealregionmost commonsite of teratomas) of maturepinealparenchymal cells
lncidence: 15ohof all pinealmasses(2nd most common A ge: any age; M:F = 1:1
tumorin pinealregion) { smal ltumor
Age: <20 years;M:F = 2:1 to 8:1 CT:
. Parinaudsyndroms= parollsis of upwardgaze { well-marginated slightlyhyperdense/ isodense
(compression / infiltration
of superiorcolliculi) mass
. hypothalamicsymptoms ^/ densefocaltumorcalcifications possible
. headache { well-definedmarkedhomogeneous enhancement
. somnolence(relatedto hydrocephalus) MR:
Location: pineal,parapineal,suprasellar,3rd ventricle ^/ intermediateintensityon TlWl + T2Wl
r/ well-definedrounded/ irregularlobulatedextremely may be isointense to CSF but containing
heterogenousmass of fat, cartilage,hair, linear/ trabeculations(DDx to pinealcyst)
nodularcalcifications+ cysts ./ mild to moderateGd-DTPAenhancement
0 Fat is absentin all otherpinealtumors! Cx: some metastasizevia CSF
3 16 Radiology
- - Review Manual

PI'TUITARY ADENOMA ./ central location; posterior lobe; usually <5 mm in

= benign slow-growing neoplasm arising from
adenohypophysis (= anterior lobe); most common tumor
of adenohypophysis
Prevalence: 5-1 0-1 8% of all intracranial neoplasms
pituitary hyperfunction / hypofunction / visual field defect
Former classification:
(a) Chromophobe adenoma (80%)
associated with: hypopituitarism
elevation of prolactin, TSH, GH serum levels
./ greatest sella enlargement; calcified in 5%
however: functioning microadenomas are part of
chromophobe adenomas
(b) Acidophilic / eosinophilic enoma (15%)
increased secretion of GH (acromegaly), prolactin,
TSH
./ tumor of intermediate size
(c) Basophilic adenoma (5%)
associated with ACTH secretion (Cushing
syndrome), LH, FSH
./ small tumor
Plain film: (LINREI-IABLE !)
./ enlargement of sella + sloping of sella floor
./ erosion of anterior + posterior clinoid processes
./ erosion of dorsum sellae
./ calcification in <10%
./ may present with mass in nasopharynx
Functioning Pituitary Adenoma
Adenoma may secrete multiple hormones!
1. PROLACTINOMA (30%)
rnost common of pituitary adenomas; approximately
50% of all cranial tumors at autopsy; M << F
prolactin levels do not closely correlate with tumor
size
0 Any mass compressing the hypothalamus /
pituitary stalk diminishes the tonic inhibitory effect
of dopaminergic factors, which originate there,
resulting in hyperprolactinemia!
Female:
Age: 15-44 years (during childbearing age)
infertility
amenorrhea
galactorrhea
elevated prolactin levels (normal <20 ng/mL)
0 >75% of patients with serum prolactin levels
>200 nglmL will show a pituitary tumor!
Male:
headache
impotence + decreased libido
visual disturbance
./ characteristic lateral location, anteriorly /
inferiorly; variable in size
Rx: bromocriptine
2. CORTICOTROPHIC ADENOMA (14%
Function: ACTH-secreting tumor
Age: 30-40 years; M:F = 1:3
Cushing disease
size
./ sampling of inferior petrosal sinuses (95%
diagnostic accuracy compared with 65% for MRI)
Rx: (1) s~~ppression by high doses of
dexamethasone of 8 mglday
(2) surgical resection difficult because ACTH
adenomas usually require resection of an
apparently normal gland (tumor small
+ usually not on surface)
3. SOMATO-TROPHICADENOMA (14%)
gigantism, acromegaly, elevated GH >10 ng/mL,
no rise in GH after administration of glucose / TRH
Histo: (a) densely granulated type
(b) sparsely granulated type: clinically more
aggressive
./ hypodense region, may be less well-defined,
variable size
4. GONADO-TROPH CELL ADENOMA (7%)
secretes follicle-stim~~~lating
hormone (FSH) /
luteinizing hormone (LH)
./ slow-growing often extending beyond sella
5. -THYROTROPH CELL ADENOMA (<lO/o)
secretes thyroid-stimulating hormone (TSH)
./ often large + invasive pituitary adenoma
6. PLURIHORMONAL PITUITARY ADEIVOMA (>5%)
CECT (dynamic bolus injection):
./ upward convexity of gland
./ increased height 210 mm
./ deviation of pituitary stalk
./ floor erosion of sella
./ gland asymmetry
./ focal hypodensity (most speci,Fic for adenoma)
./ shift of pituitary tuft Idensity change in region of
adenoma
MR:
highest sensitivity on coronal nonenhanced T I WI
(70%) + 3D FLASH sequence (69%) + combination of
both (90%)
0 113 of lesions are missed with enhancement
0 113 of lesions are missed without enhancement
./ focus of low signal intensity on T I WI
./ focus of high-signal intensity on T2WI
./ focal hypointensity within normally enhancing gland
DDx: simple pituitary cyst (= Rathke cleft cyst)
Nonfunctioning Pituitary Adenoma
1. NULL CELL ADENOMA
= hormonally inactive pit~~itary tumor with no
histologic / immunologic / ultrastructural markers
to indicate its cellular derivation
Prevalence: 17% of all pituitary tumors
Age: older patient
./ slow-growing
2. ONCOCYTOMA
Prevalence: 10% of all pit~litaryt ~ ~ m o r s
clinically + morphologically similar to null cell
adenoma

Pituitary Macroadenoma
= tumor > I 0 mm in size
Incidence: 10% (70-80% of pituitary adenomas);
M:F = 1: I
Age: 25-60 years
symptoms of mass effect: hypopituitarism, bitemporal
hemianopia (with superior extension), pituitary
apoplexy, hydrocephalus, cranial nerve involvement
(Ill, IV, VI)
usually endocrinologically inactive
Extension into: suprasellar cistern / cavernous sinus /
sphenoid sinus + nasopharynx (up to
67% are invasive)
v' occasionally tumor hemorrhage
v' lucent areas correspond to cysts / focal necrosis
v' invasion of cavernous sinus: encasement of carotid
artery (surest sign)
CT:
v' tumor isodense to brain tissue
v' erosion of bone (eg, floor of sella)
v' calcifications infrequent
MR: (allows differentiation from aneurysm)
v' homogeneous enhancement
cx:
(1) Obstructive hydrocephalus (at foramen of Monro)
(2) Encasement of carotid artery
(3) Pituitary apoplexy (rare)
DDx:
(1) Metastasis (more bone destruction, rapid growth)
(2) Pituitary abscess
Pituitary Microadenoma
= very small adenomas < I 0 mm
usually become clinically apparent by hormone
production (20-30% of all pituitary adenonias)
0 Prolactin elevation (>25 ng/mL in females)
4-8 x normal: adenoma demonstrated in 71%
>8 x normal: adenoma demonstrated in 100%
incidentaloma = nonfunctioning niicroadenoma /
pituitary cyst
v' NO imaging features to distinguish between different
types of adenomas
MRI:
v' small nonenhancing mass of hypointensity on pre-
and postcontrast T I WI
v' occasionally isointense on precontrast images
+ hyperintense on postcontrast images
-\I enhancement on delayed images
I/ focal bulge on surface of gland
v' focal depression of sellar floor
v' deviation of pituitary stalk
PITUITARY APOPLEXY
Cause: massive hemorrhage into pituitary adenoma
(especially in patients on bromocriptine for
pituitary adenonia) / dramatic necrosis / sudden
infarction of pituitary gland
0 25% of patients with pituitary hemorrhage will present
with apoplexy!
Brain Disorders 3 17
Sheehan syndrome = postpartum infarction of anterior
pi,tuitarygland
severe headache, nausea, vomiting
hypertension
stiff neck
sudden visual-field defect, ophthalmoplegia
obtundation (frequent)
hypopituitarisni (eg, secondary hypothyroidism)
0 Area of destruction must be >70% to produce pituitary
insufficiency!
v' enlargement of pituitary gla,nd
NCCT:
I/ increased density f fluid level
MR:
I/ bright signal from presence of hemoglobin on T I WI
with persistence over hyperintensity on T2WI
v' intermediate signal intensity from deoxyhemoglobin
on T I WI + T2WI
PORENCEPHALY
= focal cavity as a result of localized brain destruction
A. AGENETIC PORENCEPHALY
= Scl-~izencephaly (= true porencephaly)
B. ENCEPHALOCLASI-IC PORENCEPHALY
Time of injury: during first half of gestation
Histo: necrotic tissue completely reabsorbed without
surrounding glial reaction (= liquefaction
necrosis)
MR:
v' smooth-walled cavity filled with CSF on all pulse
sequences (= porencephalic cyst)
v' lined by white matter
C. ENCEPHALOMALACIA
= Pseudoporencephaly= Acquired porencephaly
Cause: infectious, vascular
Time of injury: after end of 2nd trimester (brain has
developed capacity for glial response)
Location: parasagittal watershed areas with sparing
of periventricl-~larregion + ventricular wall
CT:
v' hypodense regions
MR:
v' hypointense on T I WI + hyperintense on T2WI
I/ surrounding hyperintense rim on T2WI = gliosis)
v' glial septa coursing through cavity identified on T I WI
+ proton density images
US:
v' septations in cavity well visualized
PRIMITIVE NEUROECTODERMAL TUMOR
= PNET = PRIMARY CEREBRAL hlEUROBLASTOMA
= group of very undifferentiated tumors arising from
germinal matrix cells of primitive neural tube
Incidence: <50h of supratentorial neoplasms in children,
30% of posterior fossa tumors
Age: mainly in children <5 years of age; M:F = 1:I
Path: most undifferentiated form of malignant small cell
neoplasms grouped with Ewing sarcoma, Askin
tumor
 ReviewManual
318 RadiologY

Histo: highlycellulartumorscomposedof >90-95% of MR:

undifferentiatedcells(histologicallysimilarto { patchyhigh-intensitylesionsof white matteraway
medulloblastoma, pineoblastoma, peripheral iro, "p"ndyma in asymmetricdistributionon T2Wl
neuroblastoma) { sparingof corticalgraYmatter
. signs of increasedintracranialpressure/ seizures Prognosis;death u.suallywithin6 months
ODx in early stages: primary CNS lymphoma
Location:
(most
' ' supratentorial:deep cerebralwhite matter
(a)
commonly in lobe),
frontal pineal gland, in thalamic
REYESYNDROME
+ suprasellarterritories(leastfrequently) = hepatitis+ encephalitisfollowingviral YPperrespiratory
(b) posteriorfossa (= medulloblastoma) tract infectionwith Hx of largedoses of aspiriningestion
(c) outsideCNS:
Age: in children+ Youngadults
chestwall, ParasPinalregion . obtundationrapidlyprogressingto coma
kidneY
r/ initiatty(within2-3 days) smallventricles
./ large (hemispheric)heterogenousmass with tendency .r/ later progressiveenlargementof lateralventricles+ sulci
for necrosis(65%),cyst formation,calcifications(71%), { markedlydiminishedattenuationof white matter
hemorrhage(10%) Mortatity: 15-85% (from white matter edema
r/ tnin rim of edema + demYelination)
{ mild/ moderateenhancement of solidtumorportion
Dx: liverbioPsY
CT:
{ solidtumorportionshyperdense(dueto high nuclear LEUKO-
POSTERIOR
to cytoPlasmicratio)
REVERSIBLE
SYNDROME
ENCEPHALOPATHY
MR:
on TlWl + hyperintense on.T2Wl = HYPERTENSIVE ENCEPHALOPATHY
{ mildlyhypointense
r/ remarkablyinhomogeneous due to cyst formation 0 Emergencyconditionas patientmay proceedto
+ necrosis cerebiat infarctionand death if untreated!
{ areas of signaldropoutdue to calcifications Cause: acute rise in systemicblood pressure,
preeclampsia or eclampsia,following
r/ hyperintenseareason Tl Wl + variableintensity treatmentwith cyclosporine
(usuallyintermediate) on T2Wl due to hemorrhage immunosuppressive
FK-501,tacrolimus
A, cisPlatin,
r/ inhomogeneously moderatelyenhancingmasswith
tumor nodutes+ ringlikeareassurroundingcentral Pathophysiotogy: vasogenicedema due to loss of
autoregulation(? due to decreased
necrosisafter Gd-DTPA
seeding(15-40%) innervationof arteriesby autonomic
Cx: meningeal+ subarachnoid
fibers)
Location: white matterof posteriorhalf of brain
r/ nypodensewhite matteron NECT
MULTIFOCAL r/ tesionhypointenseon Tl Wl + hyperintenseon T2Wl
PROGRESSIVE images(due to a
ri lesionisointenseon diffusion-weighted
LEUKOENCEPHALOPATHY net effectof elevated diffusion from
coefficient
= PML = rapidlyprogressivefataldemyelinatingdiseasein
vasogenicedema + T2 shine-throughetfect)
patientswith impairedimmunesystem(chronic
{ no contrastenhancement
iymphocytic leukemia,lymphoma,Hodgkindisease,
carcinomatosis,AIDS,tuberculosis,sarcoidosis,organ
SARCOIDOSIS OF CNS
transPlant) = inflammatorymultisystemdiseasecharacterizedby
Etiology: virus infection(probablylatent papovavirus
= JC virus) noncaseatinggranulomas
lncidence: C-N-S involvementin 1-8% (in up to 15% of
Pathophysiotogy: destructionof oligodendrogliocytes
autoPsies)
leadingto areas of demYelination
+ edema Age: 2O4A years (morecommonin women + peopleof
inclusionbodieswithinswollen West Africandescent)
Histo: intranuclear . cranialneuropathy(facial> acoustic> optic > trigeminal
oligodendrocytes (viralparticlesin nuclei),
nerves)secondaryto granulomatousinfiltration
absenceof significantperivenousinflammation
+ leptomeningeal fibrosis(50-75%)
. progressiveneurologicdeficits,visualdisturbances,
. peripheralneuropathy+ myopathy
dementia,ataxia,spasticity . asepticmeningitis(20%)
. normalCSF fluid
. diffuseencephalopathy, dementia
Location: predilectionforparieto-occipital region
. pituitary+ hypothalamicdysfunction(eg, diabetes
Site: subcorticalwhite matterspreadingcentrally
^/ lttOcontrastenhancement insiPidusin 5-10%)
.gefl e| " | i zedl focal sei zures(heral dpoorer pr ognosis)
CT : . irultiple sclerosislikesymptoms(frommultifocal
r/ multicentricconfluentwhite matterlesionsof low
parenchYmalinvolvement)
attenuationwith scallopedbordersalongcortex . promptimprovementfollowingtherapywith steroids
^/ trtO mass effect
 BrainDisorders 319

Location: leptomeninges, dura mater,subarachnoid . rangeof normalmentationto severementalretardation

space,peripheralnerves,brain parenchyma, . blindnesspossible(opticnerve hypoplasiain 33%)
ventricularsystem Location: most commonlynear pre- and postcentralgyri
0 Affectsmeninges+ cranialnervesmore (sylvianfissure);uni-i (mostly)bilateral;in
oftenthan the brain! middlecerebralarterydistribution
@ Meningeal/ependymalinvasion r/ polymicrogyria / pachygyriaof cortexadjacentto cleft
./ diffusemeningealenhancement(mostcommon)/ r/ full-thickness cleftthroughhemispherewith irregular
meningealnodules(lesscommon) margins
Site: particularly in basalcisterns(suprasellar, { gray-matterliningof cleft(PATHOGNOMONIC)
sellar,subfrontalregions)with extensionto extendingthroughentirehemisphere
opticchiasm,hypothalamus, pituitarygland, { bilateraloften symmetricintracranialcysts,usually
cranialnerveswhere exitingbrainstem aroundsylvianfissure
Cx: communicating/ obstructivehydrocephalusis ./ asymmetricaldilatationof lateralventricleswith midline
the mostcommonfinding(fromarachnoiditis / shift
adhesions) r/ wide separationof lateralventricles+ squaringof frontal
DDx: carcinomatous/ fungal/ tuberculous lobes
m en i n g i ti s rl absenceof cavum septi pellucidi(80 - 90%) + corpus
^/ denseenhancement of falx + tentorium callosum
(granulomatous invasionof dura) Prognosisi severeintellectualimpairment,spastic
r/ solitary/ multipledura-basedmass tetraplegia,blindness
r/ ependymalenhancement DDx: (1) Pseudoporenceph€lly = ssqrired porencephaly
@ Parenchymaldisease(due to extensionfrom = localparenchymaldestructionsecondaryto
meningeal/ ventricular surfaces) vascular/ infectious/ traumaticinsult(almost
./ isodense/ hyperdensehomogeneously enhancing alwaysunilateral)
smallsingle/ multiplenodules(invasionof brain (2) Arachnoidcyst
parenchymavia perivascularspacesof Virchow- (3) Cystictumor
Robin)
Site: peripheryof parenchyma,intraspinal SEPTO.OPTIC DYSPLASIA
Cx: stenosis/ occlusionof bloodvessels = DeMORSIER SYNDROME
! smallvesselischemicchange = roreanteriormidlineanomalywith (1) hypoplasiaof
! lacunarinfarction(especiallybrainstem + basal optic nerves(2) hypoplasia/ absenceof septum
ganglia) pellucidum;oftenconsidereda mildform of lobar
^/ focal/ widespreadinfarctsof peripheralgray matter/ holoprosencephaly
at gray-whitematterjunction(periarteritis) M : F= 1 : 3
r/ reactivesubcorticalvasogenicedema Cause: insultbetween5-7th week of GA
Associatedwith: schizencephaly(50%)
' hypothalamic hypopituitarism(66%):
SC HI Z E NCE P HA L Y diabetesinsipidus(in 50%),growthretardation(deficient
= AGENETIC PORENCEPHALY = TRUEPORENCEPHALY secretionof growthhormone+ thyroidstimulating
= "splitbrain" hormone)
= full-thickness CSF-filledparenchymal cleftlinedby gray . diminishedvisualacuity(hypoplasia of opticdiscs),
matterextendingfrom subarachnoidspaceto nystagmus,occasionally hypotelorism
subependymaof lateralventricles . seizures,hypotonia
Frequency; 1:1,650 { smallopticcanals
Cause: segmentaldevelopmentalfailureof cell migration r/ hypoplasiaof opticnerves+ chiasm+ infundibulum
to form cerebralcortex/ vascularischemiaof ./ dilatationof chiasmatic+ suprasellarcisterns
portionof germinalmatrix i/ fused dilatedboxlikefrontalhornssquaredoff dorsally
Time of injury: 30-60 days of gestation + pointinginferiorly
Oftenassociatedwith: polymicrogyria, microcephaly, r/ bulbousdilatationof anteriorrecessof 3rd ventricte
gray matterheterotopia ./ hypoplastic/ absentseptumpellucidum
Types: ! thin corpuscallosum
(a) cleftswith fused lips
(maybe missedin imagingplanesparalleltothe S IN U S P E R IC R A N II
plane of cleft) = subperiosteal venousangiomasadherentto skulland
{ walls apposeone anotherobliteratingCSF space connectedby anomalousdiploicveinsto a sinus/
(b) cleftswith separated/ open lips corticalvein
r/ CSf fills cleftfrom lateralventricleto . soft painlessscalp mass that reducesunder
subarachnoidspace compression
. seizuredisorder Location: frontalbone
. mild/ moderatedevelopmental delay { calvarialthinning+ defect
320 Radiology Review Manual

CT: { subjacentwhite matterhypodenseon CT with slight

sessilesharplymarginatedhomogeneousdensely prolongationof T1 + T2 relaxationtimes (gliosis)
enhancingmassadjacentto outertableof skull, r/ choroidplexusenlargementipsilateral to
perforating
it and connectingit with anothersimilar angiomatosis
structurebeneaththe innertable thickeningof skull+ orbit(boneapposition
r/ ipsilateral
A ngio: as resultof subduralhematomasecondaryto brain
r/ extracalvarial
sinus may not opacifysecondaryto atroPhY)
slowflow { elevationof sphenoidwing + petrousridge
{ enlargedipsilateralparanasalsinuses+ mastoidair
cel l s
LEUKOENCEPHALOPATHY
SPONGIFORM r/ thickenedcalvarium(= wideningof diploicspace)
rare, hereditary @ oRBrrAL MANTFESTATION (30%)
Age: >40 years ipsilateralto nevusflammeus:
. deterioratingmentalfunction . congenitalglaucoma(30%)
r/ confluentareasof diminishedattenuation { choroidalhemangioma(71%)
^/ dilatation+ tortuosityof conjunctival+ episcleral
+ iris+ retinalvessels
SYNDROME
STURGE.WEBER-DIMITRI r/ buphthalmos
= ENCEPHALOTRIGEMINAL ANGIOMATOSIS Cx: retinaldetachment
= MENINGOFACIAL ANGIOMATOSIS @ vrscERAL MANTFESTATTON
= vascularmalformation with capillaryvenousangiomas localized/ diffuseangiomatousmalformation locatedin
involvingface,choroidof eye, leptomeninges intestine,kidneys,spleen,ovaries,thyroid,pancreas,
Cause: persistenceof transitoryprimordialsinusoidal l ungs
plexusstage of vesseldevelopment;usually DDx: Klippel-Trenaunay syndrome,Wyburn-Mason
sporadic syndrome
. seizures(80%) in 1st year of life: usuallyfocat involving
the side of the body contralateralto nevusflammeus
. mentaldeficiency(>50%) SUBARACHNOID HEMORRHAGE
. increasingcrossedhemiparesis(35-65%) Cause:
. hemiatrophyof body contralateralto facial nevus A. Spontaneous
(secondaryto hemiparesis) (1) rupturedaneurysm(72%)
. homonymoushemianopia (2) AV malformation (10%)
@ FACTALMANTFESTATTON (3) hypertensivehemorrhage
. congenitalfacialport-winestain(nevusflammeus) (4) hemorrhagefrom tumor
= telangiectasia of trigeminalregion;usually1st+ 2nd (5) embolichemorrhagic infarction
divisionof Sthnerve;usuallyunilateral (6) blooddyscrasia,anticoagulation therapy
- V, associatedwith occipitallobe angiomatosis (7) eclampsia
- V2associatedwith parietal lobe angiomatosis (8) intracranialinfection
- Vs associatedwith frontallobe angiomatosis (9) spinalvascularmalformation
@ cNS MANTFESTATTON (10)cryptogenicin 6oh(negative4-vessel
r/ leptomeningeal venousangiomasconfinedto pia angiography; seldomrecurrent)
mater B. Trauma(common)
Location: parietal> occipital> frontallobes concomitantto cerebralcontusion
Angio: (a) injuryto leptomeningealvessels at vertex
r/ capillaryblush (b) ruptureof majorintracerebralvessels(less
! abnormallylargeveinsin subependymal common)
+ periventricular regions Location: (a) focal,overlyingsite of contusion
r/ abnormaldeep medullaryveinsdraininginto (b) interhemisphericfissure,paralleling
internalcerebralvein (= venousshunt) falx cerebri
^/ failureto opacifysuperficialcorticalveins in (c) spreaddiffuselythroughout
calcifiedregion(markedlyslow blood flow / subarachnoid space(rarein trauma)
thrombosisof dysgeneticsuperficial veins)
r/ corticalhemiatrophy beneathmeningealangioma Pathophysiology: irritationof meningesby blood + extra
due to anoxia(steal) fluidvolumeincreasesintracranial
{ "tramtrack"gyriformcorticalcalcifications>2 years pressure
of age; in layers2-3(-4-5)of opposinggyri underlying . acute severeheadache("worstin life"),vomiting
pial angiomatosis; bilateralin up to 20% . alteredstateof consciousness: drowsiness, sleepiness,
Location: temporo-parieto-occipital area, stupor,restlessness,agitation,coma
occasionallyfrontal,rare in posterior . spectrophotometric analysisof CSF obtainedby lumbar
fossa puncture

NCCT (60-90% accuracyof detectiondependingon time
of scan;sensitivity dependson amountof blood;
accuracyhighwithin4-5 days of onset):
0 May occurin onlytwo locationsif subtle!
r/ increaseddensityin basalcisterns,superior
cerebellarcistern,sylvianfissure,corticalsulci,
intraventricular, intracerebral
{ alonginterhemispheric fissure= on lateralaspect
irregulardentatepatterndue to extensioninto
paramediansulciwith rapidclearingafterseveraldays
r/ corticalvein sign = visualizationof corticalveins
passingthroughextraaxialfluidcollection
MR (relatively insensitive withinfirst48 hours):
./ deoxyhemoglobin effectsnot appreciablein acute
phase(secondary to higheroxygentensionin CSF,
counterbalancing effectsof very long T2 of CSF,
pulsatileflow effectsof CSF)
r/ low-signalintensityon brain surfacesin recurrent
subarachnoid hemorrhages (hemosiderin deposition)
Prognosis; clinicalcoursedependson amountof
subarachnoid blood
Cx:
(1) Acuteobstructivehydrocephalus (in <1 week)
secondaryto intraventricular hemorrhage/
ependymitisobstructingaqueductof Sylviusor outlet
of 4th ventricle
(2) Delayedcommunicatinghydrocephalus(after
1 week) secondaryto fibroblasticproliferationin
subarachnoid spaceand arachnoidvilliinterfering
with CSF resorption
(3) Cerebralvasospasm+ infarction(developsafter
72 hours,at maximumbetween5-17 days,amount
of blood is prognosticparameter)
(4) Transtentorialherniation(cerebralhematoma,
hydrocephalus,infarction,brain edema)
SUBDURAL
HEMATOMA
OF BRAIN
= €lccurTrulation
of blood in potentialspace between
pia-arachnoid membrane(leptomeninges) + dura mater
(= "epiarachnoidspace")
lncidence: in 5/" of head traumapatients;in 15% of
closedhead injuries;in 65% of head injuries
with prolongedinterruption of consciousness
Age: accident-prone middleage; also in infants+ elderly
(largesubarachnoidspace with freedomto move in
cerebralatrophy)
Cause: severetrauma,hemorrhagic diathesis
Source of blood:
(1) pialcorticalarteries+ veins: directtrauma
= penetrating injury
(2) largecontusions:direct/ indirecttrauma
= "pulpedbrain";occasionally in bloodclotting
disorder/ duringanticoagulation therapy
(3) torn bridgingcorticalveins: indirectdue to sudden
de-/acceleration;alsowith forcefulcoughing/
sneezing/ vomitingin elderly
Elderlypredisposed: due to longerbridgingveinsin
senilebrainatrophy
BrainDisorders 321
0 No consistentrelationshipto skullfractures!
Pathogenesis:
differentialmovementof brain + adherentcorticalveins
with respectto skull + attacheddural sinusestears the
"bridgingveins"(= subduralveins),whichconnect
cerebralcortexto duralsinusesand travelthroughthe
subarachnoid and subduralspace
Location: hematomafreelyextendingacrosssuture
lines,limitedonly by interhemispheric fissure
and tentorium
. nonspecific headaches,nonlocalizing signs
. lethargy,confusion
. usuallynegativelumbarpuncture
. low-voltageEEG
CT:
r/ hyperdense65-90 HU (<1 week) / isodense20-40
HU (1-2 weeks)/ hypodense0-22 HU (3-4 weeks)
False-negative CT scan:
high-convexity location,beam-hardening artifact,
volumeaveragingwith highdensityof calvarium
obscuringflat "en plaque"hematoma,too narrow
windowsetting,isodensehematomadue to delayin
imaging10-20 days post injury/ due to low
hemoglobincontentof bloodI lackof clotting,CSF-
dilutionfrom associatedarachnoidtear
0 38% of smallsubduralhematomasare missed!
Aids in detectionof acutesubduralhematoma:
r/ thickeningof ipsilateral portionof skull
(hematomaof similarpixelbrightnessas bone)
r/ "subduralwindow"setting= window levelof
40 HU + windowwidthof 400 HU
r/ effacementof adjacentsulci
r/ sulci not traceableto brainsurface
{ ipsilateral ventricularcompression i distortion
r/ displacementof gray-whitematterinterface
away from ipsilateralinnertable
r/ midlineshift (oftengreaterthan width of
subduralhematomadue to underlyingbrain
contusion)
r/ contrastenhancementof cortexbut not of
subduralhematoma
Aids in detectionof bilateralsubduralhematomas:
r/ "parentheses" ventricles
r/ ventriclestoo smallfor patient'sage
MR: see HEMATOMAOF BRAIN
US (neonate):
^/ lineari ellipticalspace betweencranialvault + brain
r/ flattenedgyri + prominentsulci
r/ + distortionof ventricles,extensioninto
interhemispheric space
Limitations:
(a) convexityhematomamay be obscuredby pie-
shapeddisplay+ loss of near-fieldresolution
0 Use contralateraltranstemporalapproach!
(b) smallloculations may be missed
Prognosis; poor (due to associationwith other lesions)
DDx: (1) Arachnoidcyst (extensioninto sylvianfissure)
(2) Subarachnoidhemorrhage(extensionintosulci)
322 Radiology Review Manual
Acute SubduralHematoma
Usuallyfollowsseveretrauma,manifestswithinhours
after injury
Time frame: <7 days old
Associatedwith: underlyingbrain injury(50%)with
worse long-termprognosisthan
epiduralhematoma,skullfracture
(1%)
Location:
(a) over cerebralconvexity,frequentextensioninto
interhemispheric fissure,alongtentorialmargins,
beneathtemporal+ occipitallobes;NO crossingof
m idline
(b) bilateralin 15-25% of adults(commonin elderly)
and in 80-85% in infants
{ extraaxialperipheralcrescentic/ convexfluid
collectionbetweenskulland cerebralhemisphere
usuallywith:
! concaveinnermargin(hematomaminimally
pressingintobrainsubstance)
r/ convexouter marginfollowingnormalcontourof
cranialvault
{ hyperdensecollectionof 65-1OOHU, hypodenseif
hematocrit<29Y"
{ swirl sign = mixtureof clottedand unclottedblood
! occasionallywith blood-fluidlevel
{ aftersurgicalevacuation:underlyingparenchymal
injurybecomesmoreobvious
{ afterhealing:ventricular+ sulcalenlargement
Cx: Arteriovenousfistula(meningealartery+ vein
caughtin fractureline)
Prognosis.' may progressto subacute+ chronic stage /
may disappearspontaneously
Rx: evacuation,but with poor response(dueto high
uncontrollableintracranialpressurefrom
associatedinjuries)
Mortality: 35-50% (highernumberdue to associated
brain injury,mass effect,old age, bilateral
lesions,rapid rate of hematoma
accumulation, surgicalevacuation
>4 hours)
Interhemispheric Subdural Hematoma
Mostcommonacutefindingin childabuse(whiplash
forceson large head with weak neck muscles)
{ predominancefor posteriorportionof
interhemispheric
fissure
./ crescenticshapewith flat medialborder
! unilateralincreasedattenuationwith extension
alongcourseof tentorium
{ anteriorextensionto levelof genu of corpuscallosum
Subdural Hemorrhage in Newborn
Cause: mechanicaltraumaduringdelivery
(excessiveverticalmoldingof head)
1. Posteriorfossahemorrhage
(a) tentoriallacerationwith ruptureof vein of Galen
/ straightsinus/ transversesinus
(b) occipitalosteodiastasis= Sepdt?tionof
squamousportionfrom exoccipitalportionof
occipitalbone
r/ nign-Oensity thickeningof affectedtentorialleaf
extendingdown posteriorto cerebellar
hemisphere(betterseen on coronalviews)
r/ mildlyechogenicsubtentorial collection
Cx: deathfrom compressionof brainstem,
acutehydrocephalus
2. Supratentorial hemorrhage
(a) lacerationof falx nearjunctionwith tentorium
with ruptureof inferiorsagittalsinus(less
commonthan tentoriallaceration)
r/ hematomaovercorpuscallosumin inferior
aspectof interhemispheric fissure
(b) convexityhematomafrom ruptureof superficial
corticalveins
{ usuallyunilateralsubduralconvexity
hematomaaccompanied by subarachnoid
blood
r/ underlyingcerebralcontusion
{ sonographic visualization
of convexities
difficult
SubacuteSubduralHematoma
Time frame: 7-22 days
CT:
r/ isodensehematoma of 25-45 HU (1-3 weeks),
may be recognizableby mass effect:
{ effacementof corticalsulci
r/ deviationof lateralventricle
r/ midlineshift
r/ white matterbuckling
r/ displacement of gray-whitematterjunction
{ contrastenhancement of innermembrane
AID in Dx: contrastenhancementdefinescortical-
subduralinterface
MR (modalityof choicein subacutestage):
r/ nignsensitivity for Met-Hbon Tl Wl (superiorto CT
duringisodensephasefor smallsubdural
hematoma+ for hematomasorientedin the CT
scan plane,eg, tentorialsubduralhematoma):
r/ hyperintense on TlWl
ChronicSubduralHematoma
Time frame: >22 days old
Cause: mild unremembered headtrauma?
Pathogenesis: vessel fragilityaccountsfor repeated
episodesof rebleeding(in 10-30%)
followingminorinjuriesthat tear fragile
capillarybed withinneomembrane
surroundingsubduralhematoma
Predisposing factors:
alcoholism,increasedage, epilepsy,coagulopathy,
priorplacementof ventricularshunt
0 >75/" occur in patients>50 years of age!
Histo: hematomaenclosedby thick + vascular
membrane,which forms after 3-6 weeks
. historyof antecedenttraumaoften absent(2548%)

. ill-defined neurologicsigns+ symptoms:cognitive
deficit,behaviorafabnormality,nonspecificheadache
. progressiveneurologicdeficit
. low-voltageEEG,normalCSF
r/ often biconvexlenticular= mediallyconcave
configuration,esp. aftercompartmentalization
secondaryto formationof fibroussepta
r/ low-densityfesionof O-25 HU (= intermediate
attenuation betweenCSF + brain):
r/ differentattenuationswithincompartments
{ sometimesas low as CSF
r/ nign-Oensity componentsof collection(after
commonrebleeding)
r/ ttuid-sedimentation levels(sedimentedfresh blood
with proteinaceousfluid layeredabove)
{ displacement / absenceof sulci,displacement of
ventricles+ parenchyma
./ trlomidlineshift if bitaterat(25%)
{ absentcorticalvein sign = corticalveins seen along
peripheryof fluidcollectionwithoutpassingthroughit
(1-4 weeks after injury)
DDx: Acute epiduralhematoma(similarbiconvex
shape)
SUBDURAL
HYGROMA
= localizedCSF-fluidcollectionwithinsubduralspace;
commonin children
Cause: (a) resultof chronicsubduralhematoma
(b) traumatictear in arachnoidwith secondary
ball valve mechanism
Age: mostoftenin elderly+ youngchildren
Timeof onset: 6-30 days followingtrauma
r/ radiolucentcrescent-shaped collection(as in acute
subduralhematoma)
r/ no evidenceof blood products
(DDx to subduralhematoma)
MR:
r/ isointenseto CSF / hyperintense to CSF on Tl Wl
(increasedproteincontent)
Prognosis; often spontaneousresorption
DDx: (1) Enlargedsubarachnoidspace
(2) Subduralempyema
(3) Subduralhematoma
TERATOMA OF CNS
lncidence: A.5o/"of primaryintracranialneoplasms;2/"
of intracranial
tumorsbeforeage 15
Histo: mostlybenign,occasionally containingprimitive
elements+ highlymalignant
Location: pineal+ parapinealregion> floor of
3rd ventricle> posteriorfossa > spine
(associatedwith spina bifida)
ri heterogeneous midlinelesion,occasionally
homogeneous soft-tissuemass (DDx:astrocytoma)
,
r/ containsfat + calcium
./ hydrocephalus (common)
BrainDisorders 323
TOXOPLASMOSIS
OF BRAIN
Organism; obligate intracellularprotozoanparasite
Toxoplasmagondii,can live in any cell
exceptfor nonnucleated RBCs;felinesare
definitehost
lnfection: ingestionof undercookedmeat containing
cysts or sporulatedoocysts/ transplacental
transmission of trophozoites;
acquiredthrough
bloodtransfusion+ organtransplantation
Prevalence of seropositivity:
11-16'/" of urbanadultsin UnitedStates;
up to 90% of Europeanadults
Histo: inflammatorysolidi cysticgranulomasas a
resultof glial mesenchymalreactionsurrounded
by edea + microinfarctsdue to vasculitis
Affected tissue:
@ Gray + white matterof brain
0 Mostcommoncauseof focalCNS infectionin
patientswith AIDS!
@ Retina: mostcommonretinafinfectionin AIDS
@ Alveolarliningcells(4%):
mimicsPneumocystis cariniipneumonia
@ Heart(rare):
cardiactamponade/ biventricular failure
@ Skeletalmuscle
. asymptomatic
. lymphadenopathy
. malaise,fever
A. AIDS INFECTION= toxoplasmic encephalitis
= reactivationof a chroniclatentinfectionin >95%
Path: well-localized indolentgranulomatous processi
diffusenecrotizingencephalitis
. focal neurologicdeficitof subacuteonset (50-89%)
. seizures(15-25%)
. pseudotumor cerebrisyndrome
Location: basal ganglia(75%),scatteredthroughout
brainparenchymaat gray-whitematter
r/ murtipre,
i:iLi';iup to ss%)
tesions
withnodutar
/
thin-walled(common)ring enhancement
^/ surrounding whitematteredema
r/ double-dosedelayedCT scanswith higherdetection
rate for multiplelesions(64-72%)
r/ + hemorrhageand calcificationsatter therapy
Dx: improvement on therapywith pyrimethamine
+ sulfadiazine within1-2 weeks/ biopsy
DDx: CNS lymphoma(particularly with singlelesion)
0 Multiplelesionssuggesttoxoplasmosis!
B . IN TR A U TE R ININE FE C TION
Time of fetal infection: chancesof transplacental
transmission greaterin late
pregnancy
Screening:impractical due to highfalse-positiverate
. Toxoplasmagondiifoundin ventricular fluid
. chorioretinitis
' mentalretardation
r/ thickenedvault,suturesapposed/ overlapping
^/ hydrocephaluswith returnto normal/ persistenceof
, large head size
r/ intracerebralcalcificationsin posterioraspectof brain
324 Radiology Review Manual

{ multipleirregular,nodular/ cystlike/ curvilinear ./ loss of outlineof spinalcord in cervicothoracic

calcificationsin periventriculararea+ thalamus spine
+ basalganglia+ choroidplexus(= necroticfoci); { mattingof nerveroots in lumbarregion
bilateral;1-20 mm in size;increasingin number { nodularthicklinearintraduralenhancement of
+ size (usuallynot developedat time of birth) meni nges
OB-US (as early as 20 weeks MA): Cx: syringomyelia, syringobulbia
{ sonographic findingsin only36%
r/ evolvingsymmetricventriculomegaly Tuberculomaof Brain
r/ intracranialperiventricular+ hepaticdensities = feSUltof granulomaformationwithincerebral
{ increasedthicknessof placenta substance
r/ ascites lncidence: 0.15% of intracranialmassesin Western
0 Microcephalyis NOT a featureof toxoplasmosis! countries,30% in underdeveloped countries
Dx: elevatedtoxospecificlgM levelsin fetal Age: infant,smallchild,youngadult
blood Associatedwith: tuberculousmeningitisin 50%
Dx: demonstrationof elongatedteardrop-shaped . historyof previousextracranialTB (in 60%)
trophozoites in histologicsectionsof tissue Location: morecommonin posteriorfossa(62/"),
cerebellarhemispheres (frontal+ parietal
lobes);may be associatedwith tuberculous
TUBERCULOSIS meningitis
CranialTuberculousMeningitis { solitary(70%)/ multiple(30-60%)lesions;may be
Cause: ruptureof initialsubependymal/subpialfocus multiloculated
of tuberculosis(Richfocus)from earlier NCCT:
hematogenous dissemination intoCSF space ^/ isodense(72%)/ hyperdenserounded/ lobulated
Predisposed:in AIDS patients+ infants+ small lesionsof 0.5-4 cm in diameterwith mass effect
children(partof generalizedmiliary (e3%)
tuberculosis/ primarytuberculous r/ moderatesurroundingedema (72%) less marked
infection) than in pyogenicabscess
Location: basalcisterns,interhemisphericfissure, { centralcalcification(29%)
sylvianfissure,sulci of cerebralconvexities C E C T:
CT: ./ homogeneous enhancement
r/ iso-/ hyperattenuating meningesrelativeto basal { ringblush(nearlyall)with smooth/ slightlyshaggy
cisterns margins+ thickirregularwall aroundan isodense
r/ often homogeneouscontrastenhancement center(DDx: pyogenicabscesslessthick+ more
extendinginto interhemispheric fissures+ cortical regular)
surfaces r/ "targetsign" = centralcalcificationin isodense
MR: lesion+ ring-blush(DDx:giantaneurysm)
r/ normalat unenhancedSE (in early stage) r/ homogeneous blushin tuberculomaen plaque
rl distentionof affectedsubarachnoidspaceswith alongduralplane(6%) (DDx:meningiomaen
mildshorteningon T1 +T2 relaxation times plaque)
comparedwith CSF MR:
{ abnormalmeningealenhancementin basalcisterns (a) noncaseating granuloma
(mostpronounced) on gadolinium-enhanced T1Wl r/ hypointenserelativeto brain on Tl Wl
(correspondsto gelatinousexudate) ^/ hyperintenseon T2Wl
{ abnormalenhancement of choroidplexus { homogeneousnodularenhancement
+ ependymallining(rare) (b) caseatinggranuloma
Cx: (1) Communicating hydrocephalus (most r/ isointenseto markedlyhyperintenseon T2Wl
common)/ obstructivehydrocephalus(rare) { ri m enhancement on Tl W l
(2) lschemicinfarctsof basalganglia+ internal with a solidcenter
capsule(due to vascularcompression/ ^/ nypo-/ isointensecore on TlWl
occlusivepanarteritis in basalcisterns);MCA r/ iso- to hypointensecore on T2Wl
distributionmostfrequent { typicallyassociatedwith surrounding edema
DDx: infection(nontuberculous bacteria,virus,fungus, with necroticcenter
parasite),inflammatorydisease(rheumatoid { hyperintensecore on T2Wl
disease,sarcoidosis), neoplasia DDx: other CNS infection(esp.toxoplasmosis,
(meningiomatosis, CSF-seedingneoplasm) cysticercosis, fungus),lymphoma,atypical
meningioma, radiationnecrosis
S pinal T uber c u l o u s Me n i n g i ti s
MR:
{ cerebrospinal fluidloculiwith cord compression TUBEROUS
SCLEROSIS
r/ obliterationof spinalsubarachnoidspace: DISEASE= EPTPLOIA
= BOURNEVILLE
 Brain Disorders 325

= fleuroectodermal disordercharacterizedby uniformlyenhancing

hamartomatous tumors+ malformations otcurringin
brain,kidney,lung,skin,heartwith a spectrumof
phenotypicexpressions
CLASSICTRIAD(Vogt,1908)in only30% of patients:
(1) Adenomasebaceum
(2) Seizures
(3) Mentalretardation
mnemonic.' zits, fits, nitwits
Prevalence:1:10,000to 1:150,000livebirths
Cause: autosomaldominantwith low penetrance
(frequentskipsin generations);
gene loci9q34
and 16p13;spontaneousmutationsin 50-80%
Dx: it >2 of the followingsigns present
( 1) s eiz ur e
(2) calcifiedintraventriculartumor
(3) focal areas of increasedattenuationin cerebral/
cerebellarcortex
(4) wedge-shapedcalcifiedcortical-subcortical lesions
(5) hypomelanoticcutaneouslesions
(6) shagreenpatches
(7) multiplerenalcysts
(8) cardiacrhabdomyoma
(9) pulmonarylymphangiomyomatosis
(10) immediaterelativewith tuberoussclerosis
Prognosis: 30"h dead by age S;7Sh dead by age 20
@ c NS T NV O V EME N T
. myoclonicsizures(80-100%):ofen first+ most
commonsign of tuberoussclerosiswith onsetat
1st-2nd year,decreasingin frequencywith age
. mentalretardation(50-82%): mild to moderate(1/3)
moderateto severe(213);progressive;observedin
adulthood;commonif onset of seizuresbeforeage
5 years
1. Subependymal hamartomas
Location: alongventricularsurfaceof caudate
nucleus,on laminaof sulcusthalamo-
striatusimmediatelyposteriorto
foramenof Monro(mostoften),along
frontal+ temporalhornsor 3rd + 4th
ventricle(lesscommonly)
r/ multiplesubependymal nodulesof 1-12 mm:
r/ "candledrippings"appearance
r/ calcificationwith increasingage (in up to gg%)
MR:
r/ subependymal nodulesprotrudinginto
, adjacent ventricleisointensewith white matter
r/ iso- to hyperintenseon Tl Wl + hyper-and
hypointenseon T2Wl relativeto gray and white
matter
r/ minimalcontrastenhancement(in up to 56%)
2. Giant cell astrocytoma (in 15%)
= subependymal benigntumorwith tendencyfor
enlargement+ growthinto ventricle
lncidence; 5-15%; M:F = 1:1
Location: in the regionof foramenof Monro
^/ nypo-/ isodensewelt-demarcated roundedmass:
^/ Z-S cm in diameterwith intervalgrowth
{ partiallycalcified
frequentextensioninto frontalhorn / body of
lateralventricle
r/ hypo-/ isointenseon Tl Wl + hyperintense on
TWI
r/ hydrocephalus(obstructionat foramenof Monro)
Cx: degenerationinto highergrade astrocytoma
3. Gortical tubers (in 56%)
= CORTICAL/ SUBCORTICAL HAMARTOMAS
Histo: clustersof atypicalglial cells surrounded
by giantcellswith frequentcalcifications
(iI >2 years of age) = hamartomas
Frequency: multiple(75%); bilateral(30%)
./ largemisshapenbroadenedgyri with central
hypodenseregions(dueto abnormalmyelination)
r/ masslikei curvilinearcalcificationof cortical
tubers(in 15% <1 year of age, in 50"/"by age 10)
MR:
r/ relaxationtime similarto whitematter(if
uncalcified)
r/ multiplenoduleshyperintense on T2Wl
+ iso-to hypointense on Tl Wl (fibrillary
gliosis/
, demyelination)
r/ enhancementextremefyrare
4. Heterotopic gray matter islands in white matter
Histo: groupingof bizarreand giganticneuronal
cells associatedwith gliosis+ areas of
demyelination
Frequency; in up to 93%
r/ straight/ curvilinearbandsextendingradially
from ventricularwall
r/ wedge-shapedlesionwith apex at ventricularwall
r/ conglomeratemasses
r/ calcificationof all / part of nodule
{ may show contrastenhancement
CT:
r/ hypodensewell-defined regionswithincerebral
white matter
MR:
r/ iso-to hypointense regionon Tl Wl + well-
definedhyperintensearea on T2Wl relativeto
normalwhite matter
DDx of CNS /esions;
(1) lntrauterine CMV / Toxoplasmainfection(smaller
lesions,brain atrophy,microcephaly)
(2) Basalgangliacalcificationin hypoparathyroidism
I Fahrdisease(location)
(3) Sturge-Weber,calcifiedAVM (diffuseatrophy,not
focal)
(4) Heterotopicgray matter(alongmedialventricular
' wall, isodense,associatedwith agenesisof
corpuscallosum,Chiarimalformation)
@ S K ININ V OLV E ME N T
. Adenomasebaceum(80-90%) = wartlikenodulesof
brownishred coloraveraging4 mm in sizewith
bimalardistribution ("butterfly rash")
Age: firstdiscoveredat age 1-5 years;family history
in 30%
Path: small hamartomasfrom neuralelementswith
bloodvesselhyperplasia= angiofibromas
326 Radiology Review Manual

Location: nasolabial folds,eventuallycoversnose @ B ON EIN V OLV E ME N T

+ middleof cheeks r/ scleroticcalvarialpatches(45%)= "bone islands"
. Shagreenroughskin patches(80%)= "pigskin" involvingdiploe+ internaltable;frontal+ parietal
= "peaud'orange"= patchesof fibroushyperplasia; location
in intertriginous + lumbarlocation ./ thickeningof diploe(long-termphenytointherapy)
. Ash leaf patches= hypopigmentedmaculesshaped r/ bone islandsin pelvicbrim,vertebrae,longbones
like ash I spearmintleaf on trunk + extremities ! expansion+ sclerosisof rib (may be isolated)
(earliestmanifestation in infancy);may be visible { periostealthickeningof longbones
only underultraviolet light ! bone cystswith undulatingperiostealreactionin
. Ungualfibromas(15-50%): distalphalanges(mostcommon),metacarpals,
sub-/ periungualwith erosionof distaltuft metatarsals(DDx:sarcoid,neurofibromatosis)
. Caf6-au-laitspots: @ OTH E RV IS C E R A LIN V OLV E ME N T
incidencesimilarto that in generalpopulation 1. Adenomas+ lipomyomasof liver
DDx: neurofibromatosis type 1, fibrousdysplasia 2. Adenomasof pancreas
@ ocuLAR INVOLVEMENT 3. Tumorsof spleen
. Phakoma(>5%)= whitishdisk-shapedretinal @ VASCULARINVOLVEMENT (rare)
hamartoma= astrocyticprotiferationin / near optic r/ thoracic+ abdominalarterialaneurysms
disc,oftenmultiple+ usuallyin both eyes Path: vasculardysplasiawith intimal+ medial
{ small calcificationsin regionof optic nerve head abnormalities of largemuscular
{ opticnerveglioma + musculoelastic arteries
@ RE NA LI NV O L V EME N T
. usuallyasymptomatic U NI LATERAL MEGALENCEPHALY
. flankpain,hematuria = hamartomatousovergrowthof all/ part of a cerebral
. renalfailurein severecases (5%); hypertension hemispherewith neuronalmigrationdefects
0 75% of patientsdie from complicationsof renal . intractable seizuredisorderat earlyage, hemiplegia
failureby age 20 . developmentaldelay
1. Angiomyolipoma (38-89%): usuallymultiple r/ moderately/ markedenlargementof hemisphere
+ bilateral;<1 cm in diameter r/ ipsilateralventriculomegalyproportionateto
Cx: spontaneousretroperitonealhemorrhage enlargementof affectedhemisphere
(subcapsular / perinephrc) with shock { straightenedfrontalhorn of ipsilateralventriclepointing
2. Multiplecystsof varyingsize in cortex+ medulla anterolaterally
mimickingadultpolycystickidneydisease(15%) ^/ neuronalmigrationdefects:
Path: cystslinedby columnarepitheliumwith foci { polymicrogyria
of hyperplasiaprojectinginto cyst lumen ! pachygyria
{ polycysticinvolvementin infants r/ heterotopiaof gray matter
3. Renalcell carcinoma(1-3o/"),bilateralin 40o/o: { whitemattergliosis(lowdensityin whitematteron
usuallyduringadulthood CT, prolongedTl + T2 relaxationtimeson MR)
0 Only 17 documentedcasesin literatureby 1998 Rx: partial/ completehemisphericresection
Recommendation:
US evaluationevery 2-3 yearsbeforepuberty
+ yearlythereafterto identifygrowinglesions VEINOF GALENANEURYSM
@ LUNGT NV OL V EME N(1 T% ) = centralAVM directlydraininginto secondarilyenlarged
r/ interstitialfibrosisin lowerlungfields+ miliary vein of Galen(aneurysmis a misnomer)
nodularpatternmay progressto honeycomblung Anatomical types:
(lymphangioleiomyomatosis = strloothmuscle type 1 = AV fistulafed by enlargedarterialbranches
proliferationaroundbloodvessels) leadingto dilatationof vein Galen+ straight
r/ multiplebilateralsmallcystsin lungparenchyma sinus+ torcularheroPhili
! repeatedepisodesof spontaneouspneumothorax type 2 = angiomatousmalformationinvolvingbasal
(50%) ganglia+ thalamit midbraindrainingintovein
{ chylothorax of Galen
{ cor pulmonale type 3 = transitionalAVM with both features
@ HE A RTI NVOL V EME N T
Prevalence: decreaseswith increasingage (due to Feedingvessels:
spontaneoustumor regression+ better (a) posteriorcerebralartery,posteriorchoroidalartery
survivalof patientswithoutcardiactumor) (e0%)
. congenitalcardiomyopathy (b) anteriorcerebralartery+ anteriorchoroidalartery
{ circumscribed / diffusesubendocardial (c) middlecerebralartery+ lenticulostriate + thalamic
rhabdomyoma(in 5-30%) of ventricle(70%)/ atrium perforatingarteries(leastcommon)
(30%) Age at presentation: detectablein utero >30 weeks
r/ aorticaneurysm G A ; M : F= 2 : 1

(a) neonatalpattern(0-1 month)
. high-outputcardiacfailure(56%)due to massive
s hunt ing
(b) infantpattern(1-12 months)
. macrocraniafrom obstructivehydrocephalus
. seizures
(c) adult pattern(>1 year)
. headaches+ intracranialhemorrhage
. + hydrocephalus
. focal neurologicdeficits(5%)due to steal of blood
from surroundingstructures
. cranialbruit
May be associatedwith: porencephaly,nonimmune
r/ smoothtymarginat.ori[,{l:tff".. posteriorto indented
3rd ventricle
r/ prominentserpiginousnetworkin basalganglia,thalami,
midbrain
r/ dilatedstraight+ transversesinus+ torcularherophili
{ dilatationof lateral+ 3rd ventricle(97%)
NCCT:
r/ roundwell-circumscribed homogeneous slightly
hyperdensemass in regionof 3rd ventricularoutlet
r/ hyperdenseintracerebralhematoma(rupturedAVM)
r/ focal hypodensezones (ischemicchanges)
{ rim calcification (14%)
C E CT :
r/ markedhomogeneous enhancementof serpentine
structures+ vein of Galen+ straightsinus
OB - US :
{ mediantubularcysticspacewith high-velocity
turbulentflow demonstrated by pulsed/ colorDoppler
r/ brain infarction/ leukomalacia-(stealphenomenon
, with hypoperfusion)
r/ cardiacenlargement(high-output heartfailure)
r/ ditatedveins of head + neck
./ hydrocephalus(aqueductalobstruction/
posthemorrhagic impairmentof CSF absorption)
MR:
./ areasof signalvoid
An gio:
necessaryto definevascularanatomyfor surgical/
endovascularintervention
Cx: subarachnoidhemorrhage
Rx: ligation,excision,embolization of vesselsfrom
transtorcular/ transarterialapproach
Prognosis: 56o/"overall mortality;91% neonatalmortality
DDx: pinealtumor,arachnoid/ colloid/ porencephalic
VENO US A NG I O M A
= clusterof dilatedmedullaryveins,which draininto an
enlargedvein;bleedrarely
0 Can be considereda normalvariant!
Histo: venouschannelswithoutinternalelasticlamina,
separatedby glioticneuraltissuethat may calcify;
probablyrepresentingpersistentfetal venous
, system
r/ no arterialvessels
BrainDisorders 327
r/ "umbrella"configuration= multiplesmallradially
orientedveins at peripheryof lesionconvergingto a
singlelargervein
0 Associatedwith increasedincidenceof cavernous
angiomaswhich can bleed!
DDx: Sturge-Weberdisease(diffusepial angiomatosis
with venous-typecapillaries)
VENTRICULITIS
= EPENDYMITIS
= inflammation of ependymalliningof one / moreventricles
Cause: (1) ruptureof periventricular abscess(thinner
capsulewall medially)
(2) retrogradespreadof infectionfrom basal
cisterns
CECT (necessaryfor diagnosis):
^/ tnin uniformenhancement of involvedependymal
l i ni ng
rl often associatedwith intraventricular inflammatory
exudate+ septations
Cx: obstructivehydrocephalus(occlusionat foramenof
Monro/ aqueduct)
DDx: ependymalmetastases,lymphoma,infiltrating
gl i oma
VENTRICULOPERITONEAL
SHUNTMALFUNCTION
0 Peritoneumis an efficientsite of absorption
Components: ventriculostomy catheter,pressure-
sensitivevalve + reservoir,barium-
integratedsiliconeperitonealcatheter
. symptomsof increasedintracranialpressure: seizures,
headache,nausea,vomiting,lethargy,irritability
. abdominalpain, fever
. persistentbulgingof anteriorfontanel
. excessiverate of head growth
. slowedrefillof shunt reservoir
Mechanical
ShuntFailure
Cause: occlusionof catheterby choroidplexus/ glial
tissue,disconnectionof tubes
{ suturaldiastasis+ increasedsize of cranialcavity
r/ increasingventricular size:
cyst r/ intervalincreasesince last exam
r/ enlargement of temporalhorns(earliestfinding)
r/ preferentialenlargementof temporalhornsin infants
N.B.:
(1) no enlargement with scarringof ventricular
walls
(2) markedventriculardilatationdoes not
necessarilyindicateshunt malfunction
r/ shuntogram(by scintigram/ contrastradiography)
determinessite of obstruction
r/ brainedematrackingalongshunt+ withininterstices
of centrumsemiovale(withpartialobstruction)
r/ formationof white mattercyst surroundingventricular
catheter
328 RadiologyReviewManual

Obstruction of VP Shunt SubduralHematomalHygromaof VP Shunt

Location: ventricularend > peritonealend
Cause: pluggingof the catheterby brain
parenchyma/ choroidplexus/
proteinaceousmaterial/ tumor cells;
adhesionswithinPeritoneum
Tc-99malbumincolloid(injectedintoshunttubing
proximalto reservoir):
r/ no uptakewithinventricles+ normalperitoneal
activity(= proximalobstruction)
Contraststudy (injectionof nonioniccontrastmaterial
intoshuntreservoir):
r/ collectionof contrastmaterialat peritonealend of
shuntwithoutspillage(= distalobstruction)
Disconnection & Breaks of VP Shunt
Location: connectionof tubingto reservoir,at Y-
connectors,areas of great mobility(neck)
DDx: pseudo-disconnection due to radiolucenttube
components
Migration of VP Shunt
A. PROXIMALCATHETER:intosofttissuesof neck
/ unusuallocationswithinCNS
B. DISTALCATHETER:peritonealcavity,thorax,
abdominalwall,scrotum,perforationintoGl tract
Leakageof VP Shunt
= CSF escapewithoutcompletebreak/ disconnection
. palpablecystic mass
./ contrastverifiesleak site
Cause: precipitousdrainageof markedlyenlarged
ventricles
Age: usuallyseen in children>3 yearsof age with
relativelyfixed head size
Prognosis; small hematomasresolveon their own
Lesionof VP Shunt
Granulomatous
= tdta granulomatousreactionadjacentto shunttube
withinI nearventricle
massalongcourseof
./ irregularcontrast-enhancing
shunttube
Slit Ventricle Syndrome (0.9-3.3%)
= proximalshuntfailurefrom ventricular collapse
Cause: overdrainageof CSF, intermittentshunt
obstruction,decreasedintracranial
compliance,periventricularfibrosis,
intracranial hYPotension
lncidence: 0.9-3.3'/"
. intermittent / chronicheadaches,vomiting,malaise
. slowedrefillof shuntreservoir
{ small/ slitlikeventricles
V IS C E R A L LA R V A MIGR A N S OF B R A IN
roundwormnematode(Toxocaracanis)
{ smallcalcificnodules,especiallyin basalganglia
+ periventricular
DDx: tuberoussclerosis

CSFPseudocystof VP Shunt VON HIPPEL.LINDAU DISEASE

= vHL= RETINOCEREBELLAR ANGIOMATOSIS
{ shunttubingcoiledin an abdominalsoft-tissue
= inheritedneurocutaneous dysplasiacomplex;autosomal
MASS
US/ CT: dominant(genelocatedon chromosome3p25-p26)with
8O-1OO% penetrance+ variabledelayedexpressivity;
cathetertip
r/ cystsurrounding
groupedunderhereditaryphakomatosis (althoughthe
Cx: bowelobstruction
skin is not affected);in 20/"familial
Prevalence; 1:35,000-1:50,000
Age at onset: 2nd-3rddecade; M:F = 1:1
Infection of VP Shunt
Diagnostic criteria:
lncidence; 1-5-38% of CNS
(a) >1 hemangioblastoma
Timeof onset: within2 monthsof shuntplacement + visceralmanifestation
. intermittent (b) t hemangioblastoma
low-gradefever
. anemia, (c) 1 manifestation+ knownfamilyhistory
dehydration, hepatosplenomegaly
. stiffneck Subclassification(NIH):
type | = r€fl?l + pancreaticcysts,high risk for renal
. swelling+ redness overshuntingtract
. peritonitis cellcarcinoma,NO pheochromocytoma
type llA = pheochromocytoma, pancreaticisletcell
(= enlarged
r/ ventriculitis withirregular
ventricles
tumor (tYPicallYwithoutcYsts)
enhancing wall+ septations)
ventricular
type llB = pheochromocytoma + renal+ pancreatic
r/ meningitis(= enhancement of cerebral sulci)
cortical
disease
@ CNS MANIFESTATION
Abdominal Complicationsof VP Shunt Age at presentation: 25-35 Years
1. Ascites . signsof increasedintracranial pressure:headache,
2. Pseudocyst formation vomiting
wall
of viscus/ abdominal
3. Perforation . visionchanges: reactiveretinalinflammation with
4. Intestinal
obstruction exudate+ hemorrhage,retinaldetachment,
5. Metastases to peritoneum:germinoma, glaucoma,cataract,uveitis,decreasingvisual acuity,
medulloblastoma, glioblastoma
astrocytoma, eye pain
 Brain Disorders 329

. cerebellarsymptoms:vertigo,dysdiadochokinesia, Location: retrolabyrinthinetemporal bone

dysmetria,Rombergsign Site: endolymphaticsac
. spinalcord symptoms(uncommon):lossof r/ aggressivelytic lesioncontainingintratumoral
sensation,impairedproprioception osseousspicules+ areasof hemorrhage
'1.
Retinalangiomatosis = von Hippel tumor (>50%) r/ heterogeneous enhancement with hyperintense
earliestmanifestation of disease;multiplein up to areason Tl Wl + T2Wl (dueto hemorrhage)
66Vo,bilateralin up to 50% @ HEART
Histo: hemangioblastoma of retina 1. Rhabdomyoma
Dx: indirectophthalmoscopy+ fluorescein @ K ID N E Y S
, angiography . polycythemiadue to elevatederythropoietinlevel (in
! smalltumors rarelydetectedby imagingstudies 15%with hemangioblastoma, in 10/. with renalcell
r/ globe distortion carcinoma)
d tnict calcifiedretinaldensity(calcifiedangioma- 1. Corticalrenalcysts (75%)
inducedhematoma) multiple+ bilateral(maybe confusedwith adult
US: polycystickidneydisease)
r/ smallhyperechoic solidmasses,most in 2. Renalcell carcinoma(20-45/")
temporalretina Age: 20-50 years
Cx: (1) repeatedvitreoushemorrhage(frequent) r/ multicentricin 87"/o,bilateralin 10-75/", may
(2) exudativeretinaldetachmentposteriorly arisefrom cyst wall
2. Hemangioblastoma of CNS = Lindau tumor (40%) r/ sensitivity: 35% for angiography,37"/"for US,
= benignnonglialneoplasmas the mostcommonly 45/"for CT (dueto inabilityto reliablydistinguish
recognizedmanifestationof vHL disease betweencystic RCC, cancerwithincyst, atypical
Age: 15-40 years cyst)
Site: cerebellum(65%),brainstem(20%),spinal ^/ SOZ metastaticat time of discovery
c or d(1 5 % );m u l ti p l el e s i o n si n 1 0 -1 5%(may Prognosis; RCC is cause of death in 30-50% as
be metachronous) the secondmost frequentcause of
0 4-20% of singlehemangioblastomas occurin mortality!
von Hippel-Lindau disease! 3. Renaladenoma
CT: 4. Renalhemangioma
^/ largecysticlesionwith 3-15-mm muralnodule @ ADRENALpheochromocytoma (in up to 10-17o/o),
(75%) bilateralin up to 40%;confinedto certainfamilies
r / s olide n h a n c i n gl e s i o n(1 0 % ) @ EP|D|DYMIS
r/ enhancinglesionwith multiplecysticareas 1. Cystadenoma of epididymis
(15%) @ PANCREAS
r/ intensetumorblush/ blushingmuralnodule 1. Pancreaticcystadenoma/ cystadenocarcinoma
r/ tttOcalcifications(DDx:cysticastrocytoma 2. Pancreaticisletcelltumor
calcifiesin 25%) 3. Pancreatichemangioblastoma
MR (modalityof choice): 4. Pancreaticcysts (in 30-50%); incidencein
r/ hypointense cysticcomponenton TlWl autopsiesup to 72/"
(slightlyhyperintense to CSF due to protein { usuallymultipleand multilocular cystsin pancreatic
content);hyperintense on T2Wl body + tail
r/ smalltubularareasof flowvoid withinmural 0 Pancreaticcysts in a patientwith a familyhistoryof
nodule(= enlargedfeeding+ drainingvessels); von Hippel-Lindau diseaseare DIAGNOSTIC!
intensecontrastenhancement of muralnodule @ LIV E R
r/ slightlyhypointense solidlesionon TlWl; 1. Li verhemangi oma
hyperintense on T2Wl; intensecontrast 2. Adenoma
enhancement @ OTH E R S
A ngio: 1. P aragangl i oma
./ intensestainingof mural nodule("mother-in- 2. Cysts in virtuallyany organ: liver,spleen,adrenal,
law phenomenon" = tumorblushcomesearly, epididymis, omentum,mesentery,lung,bone
stays late,very dense)
{ presenceof feedingvessels
Prognosis; most frequentcause of morbidityand
mortality;frequentrecurrenceafter MU LTIP LEOR GA NN E OP LA S MS
incompleteresection @ Kidney : renalcell carcinoma(up to 40o/o),
@ LABYRINTH renalangioma(up to 45%)
1. Endolymphatic sac neoplasm @ Liver : adenoma,angioma
= aggressiveadenomatous tumorwith mixed @ Pancreas : cystadenomai adenocarcinoma
histologicfeatures @ Epididymis : adenoma
. sensorineural hearingloss @ Adrenalgland: pheochromocytoma
 Radiology Review Manual

MULTIPLE
ORGANCYSTS (2) Pancreas(in 9-72% often numerouscysts;second
most commonaffectedabdominalorgan)
corticalcystsin 75-100%at
(1)Kidney(usuallymultiple (3) Others: liver,spleen,omentum,mesentery,
earlyage,mostcommonabdominal manifestation) adrenals,lung,bone
epididymis,
 DIFFERENTIAL
DIAGNOSIS
OF ORBITALANDOCULARDISORDERS

OPHTHALMOPLEGIA Monocular Blindness,n Adulthood

Lesions
of 1. Opticneuritis
1. Oculomotor nerve(lll) 2. Vascularischemia
innervates medialrectus,superiorrectus,inferior (a) Amaurosisfugax = cholesterolembolifrom
rectus,inferiorobliquemuscle,pupilloconstrictor, internalcarotidarteryoccludingcentralretinal
levatorpalpebrae arteryand its branches
2. Trochlear nerve(lV) (b) Occultcerebrovascular malformationaffecting
innervates superior obliquemuscle the optic nerve
3. Abducens nerve(Vl) 3. Temporalarteritis
innervates lateralrectusmuscle 4. Malignantopticgliomaof adulthood

ANOPIA
[numbersreferto drawing] ORBIT
A. MONOCULARDEFECTS Spectrumof OrbitalDisorders
1 = rTlor'tocular
blindness(opticnerve lesionin fracture A. INFLAMMATORY DISEASE
of optic canal,amaurosisfugax) 1. Tissue-specific inflammation:
B. BILATERALHETERONYMOUS DEFECTS orbitalcellulitis,opticneuritis,scleritis,myositis,
2 = bitemporalhemianopia(chiasmaticlesion) Gravesdisease
C. BILATERALHOMONYMOUSDEFECTS 2. Panophthalmitis
3 = homonymoushemianopia 3. Pseudotumorof orbit
4 = upper right-sidedquadrantanopia B. CYSTICDISEASE
5 = centralhemianopticscotoma 1. Dermoidcyst
3,4,5 = rTrostcommontype of hemianopia(CVA,brain 2. Mucocele
tumor) 3. Retro-ocularcyst (developmental)

temporal

lateral
O I
monocular
blindness

OO
geniculate
nuclei of optic tract
thalamus )
bitemporal
hemianopia

OO 3
right-sided
homonymous
hemianopia

OO 4
upperright-sided
quadrantanopia

primary visual cortex

optic
radiation
OO centralhemianoptic
scotoma

Tlpesof Anopia
332 Radiology Review Manual

C. VASCULARDISEASE ExtraconalLesion
1. Cavernousangioma Extraconal-intraorbital Lesion
2. Capillaryangioma A. BENIGN TUMOR
3. Lymphangioma 1. Dermoidcyst
4. Varix 2. Teratoma
5. Carotid-cavernousfistula <1o/"of all pediatricorbitaltumors
D. T UM O RS ./ + areas of fat, caftilage,bone
1. Rhabdomyosarcoma { expansionof bony orbit+ bone defect
2. Optic nerveglioma 3 . Capillaryhemangioma
3. Meningioma 4 . Lymphangioma
4. Lymphoma 5 . Plexiformneurofibroma
5. Metastasis 6 . lnflammatoryorbitalpseudotumor
7 . HistiocytosisX
lesionusuallyarisesfrom bone
lntraconalLesion B. MALIGNANTTUMOR
mnemonic.' "Mel Met Rita MendingHems On Poor 1. Lymphomai leukemia
Charlie'sGrave" 2. Metastasis
Melanoma 3. Rhabdomyosarcoma
Metastasis
Retinoblastoma Extraconal -extraorbital Lesi on
M eningiom a A . FR OMS IN U S
Hemangioma maxillary/ sphenoidsinusesare rare locationsof
Opticglioma ori gi n
Pseudotumor 1. Tumor: squamouscell carcinoma(80%),
Cellulitis adenocarcinoma, adenoidcysticcarcinoma,
Grave disease lymphoma
2. Paranasalsinusitis:
lntraconal Lesion with Optic NerveInvolvement mostcommoncauseof orbitalinfection;
1. Opticnerveglioma Origin: from ethmoidsinuses(in children),
2. Optic nervesheathmeningioma(10% of orbital from frontalsinus (in adolescence)
neoplasm) Organism; Staphylococcus, Streptococcus,
3. Optic neuritis Pneumococcus
4. lnflammatory pseudotumor(maysurroundoptic r/ preseptal/ orbitaledema/ cellulitis
nerve) r/ subperiosteal/orbitalabscess
5. Sarcoidosis { mucormycosis(in diabetics)destroysbone
6. Intraorbitallymphoma(may surroundoptic nerve, + extendsinto cavernoussinus
older patient) Cx: (1) epiduralabscess(2) subdural
7. Elevatedintracranialpressure empyema(3) cavernoussinus
= distensionof optic sheath thrombosis(4) meningitis(5) cerebritis
r/ bilateraltortuousenlargedoptic nerve-sheath (6) brain abscess
complex 3. Mucocele
B . FR OMS K IN
Intraconal Lesion without Optic Nerve 1. Orbitalcellulitis
lnvolvement C. FROM LACRIMALGLAND
1. Cavernoushemangioma r/ mass arisingfrom superolateralaspectof orbit
2. Orbitalvarix
3. Carotid-cavernous fistula mnemonic.' "MOLD"
4. Arteriovenousmalformation Metastasis
leastcommonof orbitalvascularmalformations Others(rhabdomyosarcoma, sinus
lymphangioma,
(congenital, idiopathic,traumatic) lesion)
{ irregularlyshapedintenselyenhancingmass of Lymphoma,Lacrimalglandtumor
enlargedvessels Dermoid
r/ associatedwith dilatedsuperiorlinterior
ophthalmicvein ORBITAL MASS
5. Hematoma Orbital Mass in Childhood
6. Lymphangioma 1. Dermoid cyst 46%
7. Neurilemoma 2. Inflammatorylesion 16
r/ commonlyadjacentto superiororbitalfissure, 3. Dermolipoma 7%
inferiorto optic nerve hemangioma
4. Capillary 4%
r/ localbone erosion 5. Rhabdomyosarcoma 4"/"
 DifferentialDiagnosisof orbital and ocular Disorders sgg

6. Leukemia/ lymphoma 2% ExtraocularMuscleEnlargement

7. Optic nerveglioma Z% A . E N D OC R IN E
8. Lymphangioma 2oA 1. Gravesdisease (50%)
9. Cavernoushemangioma 1% 2. Acromegaly
mnemonic.' "LO VISHON" B . IN FLA MMA TION
Leukemia,Lymphoma 1. Myositis
. rapidonset of proptosis,erythemaof lids,
Optic nerveglioma
Vascularmalformation:hemangioma, conjunctivalinjection
lymphangioma
Inflammation Location: singlemuscle(in adults);multiple
Sarcoma: ie, rhabdomyosarcoma
Histiocytosis { enrarse.
Tif:i".',f$
ilJ3;iJ)
Orbitalpseudotumor,Osteoma r/ positiveresponseto steroids
Neuroblastoma 2. Orbitalcellulitis
3. Sjogrendisease,Wegenergranulomatosis,
lethal
PrimaryMalignantOrbitalTumors mi dl i negranul oma,S LE
4. Sarcoidosis
1. Retinoblastoma 86.0%
5. Foreign-bodyreaction
2. Rhabdomyosarcoma 8.1%
C . TU MOR
3. Uvealmelanoma 2 .3 %
1. P seudotumor
4. Sarcoma 1.7%
2. Rhabdomyosarcoma
3. Metastasis, lymphoma,leukemia
SecondaryMalignantOrbitalTumors D. VASCULAR
1. Leuk em ia 36.7% 1. Spontaneous / traumatichematoma
2. Sarcoma 14.3y" 2. Arteriovenousmalformation
3. Hodgkinlymphoma 11.0% 3. Carotid-cavernous sinusfistula
4. Neuroblastoma 9 .2 %
5. Wilmstumor 6 .7 %
6. Non-Hodgkin lymphoma 5 .6 % GLOBE
7. Histiocytosis 3 .9 %
8. Medulloblastoma
Spectrumof OcularDisorders
3 .5 %
A . C ON GE N ITA L
1. Persistenthyperplastic primaryvitreous
2. Coatsdisease
OrbitalCystic Lesion
3. Coloboma
1. Abscess 4. Congenitalcataract
2. Intraorbitalhematoma
B . V ITR E OR E TIN A L
3. Dermoidcyst
1. Vitreoushemorrhage
4. Lacrimalcyst
2. Retinaldetachment
5. Lymphangioma
3. Choroidaldetachment
6. Hydatidcyst
4. Endophthalmitis
5. Retinoschisis
OrbitalVascularTumors 6. Retrolentalfibroplasia
1. Orbitalvarix C . TU MOR
2. Arteriovenousmalformation 1. Retinoblastoma
3. Carotid-cavernousfistula 2. Choroidalhemangioma
4. Hemangioma:capillary lcavernous 3. Retinalangiomatosis
5. Bloodcyst 4. Melanocytoma
6. Arterialmalformation 5. Choroidalosteoma
7. Glomustumor D. TRAUMA
8. Hemangiopericytoma

Massin SuperolateralQuadrantof Orbit Microphthalmia

1. Lac r im algla n dtu m o r = cohg€rtital
underdevelopment i acquireddiminutionof
2. Dermoidcyst globe
3. Metastasis(breast,prostate,lung) A. BILATERALwith cataract
4. Lymphoma 1. C ongeni tal
rubel l a
5. Leukemicinfiltration of lacrimalgland 2. Persistenthyperplastic
vitreous
6. Sarcoidosis 3. Retinopathyof prematurity
7. Wegenergranulomatosis 4. Retinalfolds
8. Pseudotumor 5. Lowe syndrome
9. Frontalsinusmucocele { smallglobe+ smallorbit
334 Radiology Review Manual

B. UNILATERAL { smallflat / roundcalcificationat junctionof

1. Trauma/ surgery/ radiationtherapy retina+ optic nerve
2. lnflammation
with disorganizationof eye (phthisis ri bilateralinTlc/"
bulbi) 5. Scleralcalcifications
{ shrunkencalcifiedglobe+ normalorbit (a) in systemichypercalcemicstates (HPT,
hypervitaminosis D, sarcoidosis,secondaryto
Macrophthalmia chronicrenaldisease)
= enlargement of globe (b) in elderly:at insertionof extraocularmuscles
A. WITHOUTINTRAOCULAR MASS 6. Retrolentalfibroplasia
(a) generalized enlargement 7. Phthisis bulbi
1. Axialmyopia(mostcommoncause) secondaryto traumaor infection
r/ enlargementof globe in AP direction r/ small contractedcalcifieddisorganized
r/ + thinningof sclera nonfunctioning globe
2. Buphthalmos
3. Juvenileglaucoma mnemonic.' "NMR CT"
4. Connectivetissuedisorder: Marfan Neurofibromatosis
syndrome,Ehlers-Danlos syndrome,Weill- Melanoma(hyperdensemelanin)
Marchesanisyndrome(congenital Retinoblastoma
mesodermaldysmorphodystrophy), Choroidalosteoma
homocystinuria Tuberoussclerosis
{ "wavy"contourof sclera
(b) focalenlargement
1. Staphyloma Noncalcified Ocular Process
= s?ccuhtionof posteriorpole of globe (or 1. U vealmel anoma
berrylikeprotrusionof cornea) 2. Metastasis
Prevalence: increasingwith size of globe 86% of ocularlesionswithinglobe;usuallyin
Cause: axial myopia(temporalside of optic vascularchoroid
disc/ anteriorlyi alongequator), Origin: breast,lung, Gl tract,GU tract,
trauma,scleritis,necrotizing
infection , cutaneousmelanoma,neuroblastoma
r/ focalbulge+ thinningof sclera { bilateralin 30%
Cx: advancedchorioretinaldegeneration 3. Choroidalhemangioma
(77%),choroidretractionfrom opticdisc, 4. Vitreous lymphoma
posteriorvitreousdetachment,choroidal ./ diffuseill-definedsoft-tissuedensity
hemorrhage, retinaldetachment, 5. Developmental anomalies
cataract,glaucoma (a) Primary glaucoma = enlargementof eye
2. Apparentenlargementdue to contralateral secondaryto narrowingof Schlemmcanal
microphthalmia (b) Coloboma
B. WITH INTRAOCULAR MASS (c) Staphyloma
(rarecausefor enlargement)
(a) with calcifications: VitreousHemorrhage
1. Retinoblastoma Cause: trauma,surgicalintervention,arterial
(b) withoutcalcifications: hypertension,retinaldetachment,ocular
1. M e l a n o ma tumor,Coatsdisease
2. Metastasis . visuallossfrequent
US:
OcularLesion { numerousirregular,poorlydefinedlow-intensity
Intraocular Calcif ications echoes:
1. Retinoblastoma (>50%of all cases) { echogenicmaterialmovingfreelywithinvitreous
2. Astrocytichamartoma chamberduringeye movement
3. Choroidal osteoma { voluminoushyperechoic fibrinclotsnot fixedto
= fsfe juxtapapillarytumor of maturebone optic nerve (DDx to retinaldetachment)
Age: young woman; may be bilateral Prognosis; completeabsorption/ developmentof
r/ smallflat very dense curvilinearmass aligned vitreousmembranes(repetitiveepisodes)
with choroidalmarginof globe Cx: retinaldetachment(vitreoustractionsecondaryto
DDx: calcifiedchoroidalangioma fibrovascularingrowthfollowinghemorrhage)
4. Optic drusen
= ?ccr€tionsof hyalinematerialon i near surface DenseVitreousin PediatricAge Group
of opticdisc;oftenfamilial 1. Retinoblastoma
. headache,visualfielddefects primaryvitreous
hyperplastic
2. Persistent
. pseudopapilledema 3. Coatsdisease
 DifferentialDiagnosisof orbital and ocular Disorders sss

4. Norriedisease C . IN FLA MMA TION :

5. Retrolentalfibroplasia 1. Opticneuritis
6. Sclerosingendophthalmitis 2. Sarcoidosis
r/ fusiformthickening
= lens-shaped thickeningof nerve-sheath
complex
Leukokoria (a) with centrallucency: meningioma
= abnormalwhite/ pinkish/ yellowishpupillarylight
, (b) withoutcentrallucency:opticnerveglioma
reflex [from Greek leuko = white and koria = pupil] r/ excrescenticthickening
A . T UM O R = single/ multiplenodulesalongnerve-sheath
1. Retinoblastoma (mostcommoncause- 5S%) complexusuallydue to tumor
2. Retinalastrocytichamartoma(3%): r/ tubularenlargement
associatedwith tuberoussclerosis+ von = uniformenlargementof nerve-sheathcomplex
Recklinghausen disease (a) with centrallucency: subarachnoidprocess
3. Medulloepithelioma (rare) (metastases,perineuritis,meningioma,perineural
B . DE V E LO PME N T A L hemorrhage)
1. Persistenthyperplasticprimaryvitreous (b) withoutcentrallucency: papilledema,leukemia,
(2nd mostcommoncause- 2g%) lymphoma,sarcoid,opticnerveglioma
2. Coatsdisease(1G%)
3. Retrolentalfibroplasia(3-5%)
4. Colobomaof choroid/ opticdisc LACRIMAL GLAND
C. I NF E CT I O N
Lacrimal Gland Lesion
1. Uveitis
A. INFLAMMATION
2. Larvalgranulomatosis (16%)
D. DEGENERATIVE 1. Dacryoadenitis
1. Posteriorcataract 2. Mikulicz syndrome
= flonsp€cific
enlargement of lacrimal+ salivary
E. TRAUMA
glands
1. Retinopathy of prematurity(5%)
2. Organizedvitreoushemorrhage Associatedwith: sarcoidosis,lymphoma,
3. Long-standing retinaldetachment leukemia
3. Sjdgren syndrome
= lymphocyticinfiltrationof lacrimal+ salivary
Leukokoria in Normal-sized Eye glands
. decreasedlacrimation,xerostomia
A . CA LCI F I EDMA SS
1. Retinoblastoma Often associated with:
2. Retinalastrocytoma rheumatoidarthritis,systemiclupus
B . NO NCA L C IF IEMD AS S erythematosus, scleroderma, polymyositis
1. Toxocaralendophthalmitis 4. Sarcoidosis
2. Coatsdisease B. TUMOR
(a) benign: granuloma,cyst,benignmixedtumor
(= pleomorphic adenoma)
Leukokoria with Microphthalmia
(b) malignant:malignantmixedtumor(= pleomorphic
A. UNILATERAL
1. Persistenthyperplastic
primaryvitreous(pHpV) adenocarcinoma),adenoidcystic
B. BILATERAL carcinoma,lymphoma,metastasis
1. Retinopathy (rare)
of prematurity
2. BilateralPHPV
LacrimalGlandEnlargement
mnemonic.' "MELD"
Metastasis
OPTIC NERVE
Epithelialtumor
Optic Nerve Enlargement Lymphoidtumor
A. T UM O R: Dermoid
1. Opticnerveglioma
2. Opticnervesheathmeningioma Bilateral Lacrimal Gland Masses
3. lnfiltrationby leukemia/ lymphoma mnemonic.' "LACS"
B. FLUID: Lymphoma
1. Perineuralhematoma And
2. Papilledema of intracranial
hypertension Collagen-vascu
lar disease
3. Patuloussubarachnoidspace Sarcoidosis
336 RadiologyReviewManual

ANATOMYOF ORBIT

ORBITALCONNECTIONS Optic Ganal

SuperiorOrbitalFissure completelyformedby lesserwing of sphenoid
(Gray's
Boundaries AnatomY): Contents:
- medial : sphenoidbodY (a) nerve: oPticnerve(l)
- above : lesserwing of sphenoid= optic strut (b) vessel: oPhthalmica.
- below : greaterwing of sPhenoid
- lateral : small segmentof frontalbone NORMALORBITMEASUREMENTS
Contents: Muscles
(a) nerves: lll oculomotorn. medialrectusmuscle 4'1 + 0.5 mm
lV trochlearn. inferiorrectusmuscle 4.9 + 0.8 mm
V, ophthalmicbranchof trigeminaln.: superiorrectusmuscle 3.8 + 0.7 mm
(a) lacrimalnerve lateralrectusmuscle 2'9 + 0.6 mm
(b) frontalnerve superioroblique muscle 2'4 + 0.4 mm
Vl abducensn. Superiorophthalmicvein
sympatheticfilamentsof internalcarotid a x i a lC T . . . .1 . 8+ 0 . 5 m m
plexus c o r o n aCl T . . . . . . . . . . . . . . . 2 . 7+ 1 . 0m m
(b) veins: superior+ inferiorophthalmicvein Optic nerve sheath
(c) arteries: 1. meningealbranchof lacrimalartery retrobulbar............... . 5 ' 5+ 0 . 8m m
2. orbitalbranchof middlemeningeal waist 4.2 + 0.6 mm
arterY Globe position
behi ndi nterzygomatil icne " ..' .. 9.9+ 1.7mm
InferiorOrbitalFissure
Location: betweenfloor + lateralwall of orbit; ORBITALCOMPARTMENTS
connectswith pterygopalatine the orbitalseptum+ globedivideorbitinto
+ infratemporalfossa A . A N TE R IORC OMP A R TME N T
Contents: lids,lacrimalapparatus,anteriorsofttissues
(a) nerves: infraorbital + zygomaticnn. B . P OS TE R IOR C OMP A R TME N T
ganglion
branchesfrom pterygopalatine = RETROBULBAR SPACE
(b) veins: connectionbetween inferior v.
orbital the cone consistingof extraorbitalmuscles
+ pterygoidplexus + envelopeof fasciadividesretrobulbarspace into
(a) intraconalsPace
(b) extraconalsPace

superiorrectusm..
levator palpebrae
superior supenonsm.
ophthalmic v.
supenor
ophthalmic a. oblique m.

lateral
rectusm. medial
rectusm.

inferior
rectusm.
infraorbital n.

Coronal Orbital Tomogram through Midorbit

 Anatomyof Orbit 337

Orbital Spaces
globe: subdivided into anterior + posterior segments
by lens
optic nerve-
sheathcomplex: optic nerve surrounded by meningeal sheathas
extension from cerebral meninges
intraconal space: orbital fat, ophthalmic a., superior ophthalmic
v., nervesI, III,IV, V' VI
incomplete fenestratedmusculofascial system
extending from bony orbit to anterior third of
globe, consistsof extraocularmuscles
+ interconnecting fascia
extraconal space: between muscle cone + bony orbit containing
fat,lacrimal gland,lacrimal sac,portion of
inferior oblique m. superior ophthalmic v.
338 Radiology Review Manual

ORBITALANDOCULARDISORDERS

BUPHTHALMOS A ngi o:
= HYDROPHTHALMOS = MEGOPHTHALMOS r/ ipsilateralICA contrastinjectionshowswall of ICA to
= diffuseenlargement of eye in childrensecondaryto be incomplete
increasedintraocularpressure r/ contralateralICA contrastinjection+ compressionof
Cause: involvedICA
1. Congenital/ infantileglaucoma r/ earlyopacificationof veinsof cavernoussinus
2. Neurofibromatosis type 1: obstructionof canalof { retrogradeflow throughdilatedsuperiorophthalmicv.
Schlemmby membranes/ massescomposedof Rx: latex/ siliconeballoondetachedinsidecavernous
aberrantmesodermaltissue sinusto plug laceration(ocularsignsresolvewithin
3. Sturge-Weber-Dimitri syndrome 7-10 days)
4. Lowe (cerebrohepatorenal) syndrome
5. Ocularmesodermaldysplasia(eg,Axenfeldor CHOROIDAL DETACHMENT
Riegeranomalies) surgical
Cause: trauma, spontaneous
intervention,
6. Homocystinuria US:
7. Aniridia r/ two convexlines projectinginto the eye from
8. Acquiredglaucoma(rare) peripheryof globe+ advancingto ciliarybodywith
Pathophysiology: posteriorfixationoutsidethe opticdisk (= macula)
obstructionof canal of Schlemmlocatedbetween ri minimal/ no choroidalmembranemobilityduringeye
cornea+ iris leadsto decreasedresorptionof aqueous movement
humor(= €lnterior chamberfluid)with scleraldistension
r/ uniformlyenlargedglobewithoutmass of round/ oval
I bizarreshape C H OR OID A L H E MA N GIOMA
Rx: goniotomy(increasesthe angleof anterior = VdscUhrhamartoma
chambe|; trabeculotomy (lysesadhesions) Age: 10-20 years (mostcommonbenigntumor in adults)
May be associatedwith: Sturge-Webersyndrome
Location: posteriorpoletemporalto opticdisk (70%)
r/ O.S-g-mmsmalltumor
CAROTID.CAVERNOUS SINUS FISTULA ./ focalthickeningof posteriorwall of globe
= abnormalcommunicationbetweeninternalcarotidartery ^/ enhancement similarto choroid
+ veinsof cavernoussinus r/ retinaldetachment(frequent)
Etiology:
US:
(1) Trauma: lacerationof ICA withincavernoussinus homogeneous mass
{ hyperechoic
(a) usuallysecondaryto basal skullfracture
DDx: melanoma(choroidalexcavitation)
(cavernousICA + smallcavernousbranches
fixedto dura)
(b) penetrating trauma
(2) Spontaneous:ruptureof an intracavernous ICA COATSDISEASE
aneurysm = RETINAL TELANGIECTASIA
Route of drainage: = Pseudoglioma= col't9€I'lital idiopathicprimaryvascular
(a) superiorophthalmicvein (common) malformationof the retinacharacterizedby
(b) contralateralcavernoussinus (1) multipleabnormaltelangiectatic retinalvessels
(c) petrosalsinus (2) lackof blood-retinabarriercausingleakageof a
(d) corticalveins (rare) lipoproteinaceous exudateinto retina+ subretinal
. pulsatingexophthalmos, edema
chemosis,conjunctival spacewith secondarydetachmentof retina
. persistentorbitalbruit Age: 6-8 years(butpresentat birth); M:F = 2:1
. restrictedextraocularmovement . strabismus
. decreasein visiondue to increasein intraocular . may presentwith leukokoria(if retinamassively
pressure(50%)= indicationfor emergenttreatment detached)116%of leukokoriacasesl
. lossof vision,secondaryglaucoma
{ enlargededematousextraocularmuscles
. cholesterolcrystalsat funduscopy
r/ dilatationof superiorophthalmicvein I facialveins/
int er naljugu l avre i n Location: unilateralin 90%
r/ focal i diffuseenlargementof cavernoussinus Associatedwith: { retinaldetachment
r/ occasionally sellarerosion/ enlargement { slightmicrophthalmia
{ enlargementof superiororbitalfissure(in chronicphase) ./ trtOfocal mass / calcification(HALLMARK)
U S+ M R : US:
^/ arterialflow in cavernoussinus + superiorophthalmic { clumpyparticulateechoesin subretinalspace (due to
v ein cholesterolcrystalssuspendedin fluid)
 Orbitaland OcularDisorders 339

r/ vitreous+ subretinalhemorrhage(frequent) r/ well-definedcysticmass+ negative HU numbers

DDx: unilateralnoncalcifyingretinoblastoma(before r/ tfrict<
surrounding capsule
3 years of age, no microphthalmia) ^/ + expansion / erosionof bonyorbit
CT: US:
r/ unilateraldensevitreousin normal-sized globe r/ encapsulated heterogeneousmasswithvariable
MR: cysticcomponent
r/ hyperintensesubretinalexudateon Tl Wl + T2Wl MR:
(due to mixtureof protein+ lipid)/ hypointenseon r/ nignsignalintensity
on TlWl + T2Wl
, T2Wl (cholesterolcrystals+ membranouslipids)
{ abnormalenhancementof retinaat ora serrata+ of
detachedretinalleaves
DDx: (1) Persistenthyperplasticprimaryvitreous(thick ENDOPHTHALMITIS
tubularretrolentalmass) Infectious Endophthalmitis
(2) Retinopathyof prematurity Organism; bacteria(rarein childhood, trauma,
Rx: photocoagulation / cryotherapyto obliterate fungi,parasites
idiopathic),
telangiectasias(in early stages) Cause:
(a)exogenous endophthalmitis: mostcommonly
COLOBOMA relatedto eyeinjury/ surgery
Greek= to mutilatel
lkoloboun, (b)endogenous endophthalmitis:hematogenous
= incompleteclosureof embryonicchoroidalfissure spreadfromdistantsourceof infection
affectingeyelid/ lens liris lchoroid/ retina/ macula; US:
autosomaldominanttrait with variablepenetrance { medium- to high-intensityechoesdispersed
(30%)and expression;bilateralin 60% throughout vitreous(DDx:echoesin vitreous
Time of insult: 6th week of GA hemorrhage aremoremobile)
May be associatedwith: encephalocele,agenesisof CT:
callosum ^/ increased attenuationof vitreous
Location: in 50% oir"t.rl?tpus r/ uveal-scleralthickening
{ cysticoutpouching(= herniation)of vitreousat site of ^/ decreased attenuationof lens
optic nerveattachment
r/ smallglobe
DDx: microphthalmos with cyst = duplicationcyst, axial Sclerosing Endophthalmitis
(high)myopia = TOXOCARACANISENDOPHTHALMITIS
= granulomatous uveitisresultingin subretinalexudate,
retinaldetachment, organizedvitreous
CONGENITAL CATARACT Age: 2-6-12 years
= opacificationof lens Mode of infection:
Etiology: infection,hereditary playingin soilcontaminated by viableinfectiveeggs
Location: frequentlybilateral from dog excrement(commonin playgrounds)
US: Organism; helminthicnematodeToxocaracanis
^/ increasein thickness+ echogenicityof posteriorwall causing visceralI ocularlarva migrans
of lens+ intralenticular
echoes (0.5mm l ong,20 pm w i de);endem ic
throughoutworld;especiallycommonin
southeasternUnitedStates
DACRYOADENITIS Life cycle:
= infectionof lacrimalgland egg hatchesinto larvawithinintestinesof definitehost
Organism; staphylococci(mostcommon),mumps, (dog)+ developsinto adult worm; alternativelydog
infectiousmononucleosis,
influenza may eat infective-stagelarvaefrom intestines/
{ homogeneous enlargementof lacrimalgland visceraof otheranimals;in noncaninehost larvaewill
r/ + compressionof globe not developintoadultworm,but burrowthrough
intestinalwall and migrateto liver,lung,and other
tissueincludingbrain+ eye
DERMOID
CYSTOF ORBIT Pathophysiology:
Mostcommonbenignorbitaltumorin childhood(45%of migrationthroughhumantissueproducesa severe
all masses) eosinophilic reactionthat becomesgranulomatous;
Age: lstdecade spreadshematogenously to temporalchoroid
Histo: containskeratin,hair,stratifiedepithelium Path: retinaelevated+ distorted+ partiallyreplacedby
+ dermalappendageswithinthickcapsule;usually an inflammatory masscontainingabundant
arisesin fetalcleavageplanes(sutures) dense scar tissue;subjacentchoroidinfiltrated
Location: in anteriorextraconalorbit,uppertemporal with chronicinflammatory cellsincluding
quadrant(60%),upper nasalquadrant(25%) eosinophils;proteinaceoussubretinalexudate
340 Radiology Review Manual

. red "hot"eye, photophobia, pain STAGING(Werner'smodifiedclassification):

. anteriorchamberflarecells,keraticprecipitates Stage I eyelidretractionwithoutsymptoms
. vitreoussynechia Stage ll eyelidretractionwith symptoms
. vitreitis= €lccUrTrUlation
of cellulardebris in vitreous S tagel l l proptosis>22 mm withoutdiplopia
. leukokoria(16% of cases of childhoodleukokoria) Stage lV proptosis>22 mm with diplopia
. fever,hepatomegaly, pneumonitis, convulsions Stage V cornealulceration
. peripheralbloodeosinophilia Stage Vl lossof sight
Location: usuallyunilateral Location:
r/ eye of normalsize withoutcalcifications bilateralin 70-85%; singlemusclein 10%;asymmetrical
r/ secondaryretinaldetachment involvementin 10-30%; all musclesequallyaffected
US: with similarproportional enlargements; superiormuscle
{ hypoechoicmass in peripheralfundus groupmostcommonlywhen only singlemuscleinvolved
+ calcifications [formernotion: inferior> medial> superiorrectus
CT: muscle+ levatorpalpebrae> lateralrectusmuscle]
{ intravitrealmass mnemonic.' "l'M SLow"
thickening(granulomatous
focaluveoscleral lnferior
reactionaroundlarva)with contrastenhancement Medial
./ increaseddensityof vitreouscavity Superior
MR: Lateral
{ enhancinggranulomaisointenseto vitreouson r/ proptosis= globe protrusion>21 mm anteriorto
TlWI interzygomatic lineon axialscansat levelof lens
{ mass usuallyhyperintenserelativeto vitreouson { swellingof musclesmaximallyin midportion(relative
"Coke-bottle"
T2Wl,occasionally hypointense(dueto dense sparingof tendinousinsertionat globe)=
f ibroconnective tissue) srgn
Cx: retinaldetachment(dueto subretinalfluidi slightuveal-scleral thickening
vitreoretinaltraction),cataract apicalcrowding= orbitalapex involvedlate (pressureon
Dx: (1) Enzyme-linked immunosorbent assay(ELISA) opticnerve)
on bloodserum/ vitreous aspirate dilatationof superiorophthalmicvein (compromised
(2)Histologic
identification
of organism orbitalvenousdrainageat orbitalapex due to enlarged
DDx: retinoblastoma extraocularmuscles)
./ increasein diameterof retrobulbaroptic nervesheath
(duraldistensiondue to accumulation of CSF in
subarachnoidspacewith optic neuropathy)
GRAVESDISEASE
OF ORBIT ^/ increaseddensityof orbitalfat (late)
= THYROID OPHTHALMOPATHY = ENDOCRINE ^/ anteriordisplacementof lacrimalgland
EXOPHTHALMOS r/ intracranial fat herniationthroughsuperiorophthalmic
= increasein orbitalpressureproducesischemia,edema, fissure(bestcorrelationwith compressiveneuropathy
fibrosisof muscles MR:
Etiology: producedby long-actingthyroid-stimulating ri nignsignalintensityin enlargedeye muscleson T2Wl
factor (LATS);probablyimmunologiccross- (edemain acuteinflammation)
reactivityagainstantigenssharedby thyroid Prognosis; in 90% spontaneousresolutionwithin3-36
+ orbitaltissue months; in 10'/"decreasein visualacuity
Age: adulthood;5% youngerthan 15 years; M:F = 1:4 (cornealulceration/ optic neuropathy)
Histo: depositionof hygroscopicmucopolysaccharides Rx: short-and long-termsteroidtherapy,cyclosporine,
+ glycoprotein(early)+ collagen(late);infiltration radiation,surgicaldecompression, correctionof
by mast cells and lymphocytes,edema,muscle eyelidposition
fiber necrosis,lipomatosis,fatty degeneration DDx: pseudotumor(usuallyincludestendonof eye
Timeof onset: signs + symptomsusuallydevelopwithin muscles)
one year of the onset of hyperthyroidism
. proptosis H E MA N GIOMA OF OR B IT
0 Mostcommoncauseof uni-/ bilateralproptosisin adult! 0 Mostcommonbenignorbitaltumor
' lid lag = UPPgIeYelidretraction Location: 83-94%retrobulbar(intraconal)
. periorbital swelling { sharplydemarcatedoval mass in superior-temporal
. conjunctivalinjection portionof conus (2/3)often sparingorbitalapex
. restrictedocularmotility(correlateswith increasein ./ displacement(not involvement)of optic nerve
mean musclediameters) r/ expansionof bony orbit
. progressiveoptic neuropathy(5%) r/ uniform/ inhomogeneous (whenthrombosed)
. hyperthyroidism; euthyroidism(in 10-15%);severityof enhancement
orbitalinvolvementunrelatedto degreeof thyroid r/ smallcalcifications(phleboliths)
dysfunction { puddlingof contrastmaterialon angiography
 Orbrtaland OcularDisorders 341

US: r/ subperiostealfluidcollection
well-definedencapsulatedmass of intermediate r/ displacement of thickenedperiostealmembrane
echogenicity + increasedenhancement
r/ absent/ poor predominantly
venousflow r/ displacementof adjacentfat + extraocularmuscles
MR:
r/ hyperintensity
on Tl Wl + T2Wl
CapillaryHemangioma
of Orbit
mostcommonvasculartumorof orbitin children;
5-15% of all pediatricorbitalmasses Cellulitisof Orbit
Age: first 2 weeks of life;g5% in <6 monthsof age; = ?cUtebacterialinfection,often extendingfrom
M<F paranasalsinuses/ eyelids
Histo: proliferationof endothelialcells with multiple . limitationof ocularmovements
capillaries . fever
. proptosis,chemosis(= s6sma)of eyelid+ conjunctiva
Location: mostlyconfinedto extraconalspace
exaggeratedby crying r/ proptosis
. associatedwith skin angioma(90%)
r/ scleralthickening
Location: anteriorpart of orbit,occasionallyposterior
ri enlargement+ displacement of extraocular muscles
r/ masswith enhancement equalto / greaterihanorbital (frequently medialrectusmuscle)
muscle r/ increasedattenuationof retro-orbital fat + obliteration
r/ poorlymarginated(suggestingmalignantcause) of fat planes
! activityin radionuclideflow studies r/ opacification of ethmoid+ maxillarysinuses
US: (extension throughthin laminapapyraceaintoorbit)
{ poorlydefinedheterogeneousmass of intermediate { subperiostealabscess(withethmoiditis)
, echogenicity MR:
r/ abundantinternalflow decreasingwith age
r/ hypointense on TlWl + hyperintense on T2Wl
Prognosis; often increasein size for 6-10 months
r/ contrast-enhanced fat-suppressedimagesare most
followedby spontaneousinvolutionwithin sensitive
1-2 years US:
r/ diffusehypoechoicarea invadingretrobulbarfat
CavernousHemangioma
of Orbit Rx: antibiotics+ corticosteroids
Frequency;usuallytumor of adulthood; 12-157"of atl
Cx: orbitalabscess
orbitalmasses; 1-2% of childhoodorbital DDx: cannotbe differentiated from edema,chloroma.
masses leukemicinfiltrate
Age: 2040 years; F > M
Histo: largedilatedvenouschannelswith flattened
PreseptalCellulitis
endothelialcellssurroundedby fibrous = fibrousorbitalseptumresistsextensionof infection
pseudocapsule
. slowlyprogressiveunilateralproptosis,dipfopia, into posteriorcompartmentof orbit
{ thickeningof eyelids+ septum
diminishedvisualacuity(opticnervecompression)
r/ swellingof anteriororbitaltissueswith increased
density+ obliterationof fat planes
INFECTION
OF ORBIT Edemaof Orbit
Cause: bacterialinfectionextendingfrom paranasal
Location: usuallyconfinedto preseptalstructures
sinuses(especially ethmoid+ frontalsinuses), (eyelid,face);involvement of orbital
face,eyelid,nose,teeth,lacrimalsac through
structures(rare)
thin laminapapyracea+ valvelessfacialveins
{ swellingof eyelidslface
into orbit
^/ increasedattenuationof orbitalfat + obliterationof fat
Organism; staphylococci, streptococci,pneumococci
. lid edema,ocularpain,ophthalmoplegia planes
. fever,elevatedWBC r/ displacement+ enlargementof extraocularmuscles
MR:
Location: preseptal= periorbitalsoft tissue;
{ hyperintensity
on T2Wl
subperiosteal;peripheral= extraconalfat;
extraocularmuscles;central= intraconalfat;
opticnervecomplex;globe;lacrimalgland LY MP H A N GIOMA OF OR B IT
Cx: epiduralabscess,subduralempyema,cavernous lncidence: 3.5:100,000; 1-2% of orbitalchildhood
sinusthrombosis,cerebralabscess,osteomyelitis masses;8/" of expandingorbitallesions
Histo: dilatedlymphatics,dysplasticvenousvessels,
Abscessof Orbit smoothmuscle,areasof hemorrhage
Location: mostcommonlyin subperiosteal
spaceon (a) simple/ capillarylymphangioma
medialwall = lymphaticchannelsof capillarysize
342 RadiologyReviewManual

(b) cavernouslymphangioma TO ORBIT

METASTASIS
= dilatedmicroscopic channels Origin: only in 50% known;carcinomaof breast+ lung
(c) cystichygroma (adults);neuroblastoma> Ewingsarcoma,
= tTl?closcopic multilocularcysticmass leukemia,Wilmstumor(children)
Age: 1st decadeor later (meanage of 6 years) Location: 12/"intraorbital, especiallyin
86% intraocular
. proptosis(suddenproptosisfrom spontaneous posteriortemporalportionof uvea (vascular
intratumoral hemorrh?ge= cARDINALFEATURE; layerbetweenretina+ sclera)near macula;
exacerbatedduringupper respiratoryinfections[rare]) may be bilateral
. associatedwith lesionson lid,conjunctiva, cheek CT:
. coincidentlymphangiomatous cysts in oral mucosa ./ small areas of thickening+ increaseddensity
Location: usuallymedialto optic nervewith intra-and ,Vsubretinalfluid
extraconalcomponent,crossinganatomic
boundaries(conalfascia/ orbitalseptum); NORRIEDISEASE
may involveconjunctiva + lid = RETINAL DYSPLASIA
{ poorlydefinedmultilobulated inhomogeneous lesion = X-linkedrecessivedisease:? inheritedform of
r/ singlei multiplecystlikeareaswith rim enhancement persistenthyperplasticprimaryvitreous
(after hemorrhage)= blood cyst = "chocolatecyst" , seizures,mentalretardation(50%)
r/ areasof enhancement(= Venouschannels)/ ring , hearingloss,deafnessby age 4 (30%)
enhancement(afterhemorrhage) , bilateralleukokoria+ microphthalmia
{ rarelycontainsphleboliths(DDx: hemangioma, orbital . cataract,blindness(absenceof retinalganglioncells)
varix) r/ microphthalmia
r/ mild to moderateenlargementof orbit { densevitreouswith blood-fluidlevel
US: ^/ cone-shapedcentralretinaldetachment
r/ area of predominantlycysticheterogeneoustexture rl calcifications
with infiltrativeborders
MR: OCULARTRAUMA
r/ may show hematomaof variousdurationwithin lesion Types: (a) Simple/ complicatedcontusionwith rupture
of ocularwall
Prognosis; no involution,progressionslowswith (b) Simplei perforating injuryto the globe
terminationof body growth (c) Foreignbody
DDx: orbitalvarix . clinicalevaluation:testingof visualacuity,slit-lamp
evaluationof cornea+ anteriorsegment,intraocular
pressuremeasurement,f unduscopy
US (usedif ocularmediaopaquedue to vitreous
OF ORBIT
LYMPHOMA hemorrhage/ hyphema/ traumaticcataract)
Usuallypresentswithoutevidenceof systemicdisease; 1. H emorrhage
subsequentdevelopmentof systemicdiseasefrequent (a) vitreous hemorrhage (53%)
lncidence: 3rd most commoncause of proptosisafter . visuallossfrequent
orbitalpseudotumor+ cavernous { echogenicmaterialmovingfreelywithinvitreous
hem a n g i o mai n; 8 h o f l e u k e mi ai;n 3-4h of chamberduringeye movement
lymphoma Cx: retinaldetachment(vitreoustraction
Age: 50 years on average secondaryto fibrovascularingrowthfollowing
Type: usuallynon-Hodgkin B-celllymphoma;Burkitt hemorrhage)
lymphomawith orbit as pritnarymanifestation; Rx: vitrectomy
Hodgkindiseaserare (b) retrohyaloid hemorrhage (2%)
. painlessswellingof eyelid ^/ echogenicmaterialremainingbehinddetached
. exophthalmos(latein courseof disease) vitreouscapsuleduringeye movement
Location: extraconal(especiallylacrimalgland,anterior (c) hematomain retro-ocularspace
extraconalspace,retrobulbar)> intraconal> 2. Retinaldetachment
optic nerve-sheathcomplex;may be bilateral (a) total retinal detachment (18%)
"V" shapewith apex at optic
0 Lacrimalgland is a commonsite for leukemicinfiltratesl r/ slightlythick line of
Growth types: disk
(a) well-defined high-density mass (mostcommonly r/ retinaremainsbounddown at ora serrata
aboutlacrimalgland) (b) focal retinal detachment (2%)
(b) diffuseinfiltration(tendsto involveentireintraconal { elevatedimmobilelinecloseto scleraat
region) peripheryof globe
r/ slightto moderateenhancement 4. Vitreous detachment (11%)
US: ^/ tnin undulatemobileline movingaway from posterior
r/ solitary/ multiplehypoechoichomogeneousmasses aspectof globe duringeYemotion
with infiltrativeborders 5. Choroidaldetachment(5%)

6. Intraocular foreign body (7%)
US sensitivity: 95/" tor intraocular+ 50% for
intraorbitalforeignbody
Cx: siderosis(if metallic);endophthalmitis
7. Lens dislocation (3%)
8. Thickeningi ruptureof ocularwall
9. Vascularcomplications
(a) centralrenalarteryocclusion
(b) carotid-cavernous fistula
(c) fistulaof angularvein
OPTICNERVEGLIOMA
= JUVENILE PILOCYTIC ASTROCYTOMA
= fi'rostcommoncause of optic nerveenlargement
lncidence: 1"/oof all intracranialtumors,2/" of childhood
orbitalmasses;80% of primarytumorsof
optic nerve
Histo: proliferationof well-differentiatedastrocytes
= low-gradeglialneoplasm;mostcommonly
pilocyticastrocytoma(in children)
+ glioblastoma(in adults)
Age: 1st decade(80%);peak age around5 years;M < F
Associatedwith: neurofibromatosis in 10-50%
(+ bilateralopticgliomas)
0 15% of patientswith neurofibromatosis
have opticnervegliomas!
. decreasedvisualacuity,minimalaxialproptosis
r/ tubular/ fusiform/ excrescentic well-circumscribed
, enlargement of opticnerve
ri posteriorextensionalong optictracts in 60-70%
(indicatesnonresectability)
r/ calcifications(rare)
r/ same attenuationas normaloptic nerve;slightcontrast
enhancement
r/ ipsilateralopticcanal enlargement(g0%)>3 mm / 1 mm
differencecomparedwith contralateralside
US:
r/ well-defined homogeneousmassof medium
echogenicityinseparablefrom optic nerve
MR: moresensitivethan CT in detectingintracanalicular
+ intracranialextent
r/ isointenseto muscleon TlWl
r/ hyperintenseon T2Wl
DDx: opticnervesheathmeningioma(no intracranial
extensionalong optic pathway)
Malignant Optic Glioma of Adulthood
lncidence; extremelyrare;30 cases in this century
M eanage: 6t h d e c a d e ;M :F = 1 .3 :1 .0
Histo: anaplasticastrocytoma/ glioblastoma
multiforme
. rapidlyprogressive monocularvisuallossculminating
in monocularblindnesswithina few weeks
. with retrogradetumor extension:contralateral
, temporalhemianopia, polyuria,polydipsia
r/ focal/ diffuseenlargementof optic nerve
1 nypo-to isointenseon Tl Wl + hyperintenseon T2Wl
r/ obliterationof subarachnoidspace aroundaffected
portionof nerve
Orbitaland OcularDisorders 343
diffuseintenseenhancement of opticnerve
thickening+ abnormalenhancementof opticnerve
sheath
Tumorextension: opticchiasm,hypothalamus, basal
ganglia,brainstem,medialtemporal
lobes,leptomeninges, ependyma
Prognosis; <1-yearsurvivaldespiteaggressivetherapy
DDx: (1) Opticneuritis(demyelinating plaques
elsewhere)
(2) Periopticmeningioma(hypointense on T2Wl,
stippledcalcifications,
hyperostosis)
(3) Sarcoidosis, lymphoma,orbitalpseudotumor
(moderately/ markedlyhypointense on T2Wl)
OP TIC N E R V E S H E A TH ME N IN GIOMA
= P E R IOP TICME N IN GIOMA
lncidence: 10"/"of all intraorbitalneoplasms;<2o/"of
intracranial meningiomas
Age: middle-aged+ elderlyfemales;slightlymore
aggressivein children
Occasionally associated with:
neurofibromatosis (usuallyin teenagers)
Primaryorigin: arisingfrom arachnoidrests in the
meningealinvestiture of opticnerves
in orbit/ middlefossa
. progressiveloss of visualacuityover months(optic
atrophy),proptosis
r/ t enlargement of opticcanal
r/ tubular(mostcommonly)/ fusiform/ excrescentic
thickeningof opticnerve
r/ sphenoidbone hyperostosis
r/ frequentlycalcified(HIGHLYSUGGESTTVE)
US:
r/ hypoechoictumorwith irregularborder
CECT:enhancementis the rule
r/ dense linearbands (axialview) as "tramtracks"/
ringlike(coronalview)due to tumorenhancement
aroundnonenhancing opticnerve
r/ minimalextensionintoopticcanal(not uncommon)
MR:
ri extrinsicsoft-tissuemass surroundingoptic nerve
ri hypointense to fat on Tl Wl
OPTICNEURITIS
= flerVeinvolvement by inflammation,degeneration,
demyelination
Etiology: (1) multiplesclerosis(involvesoptic nervein 1/3)
(2) inflammation secondaryto ocularinfection
(3) degeneration (toxic,metabolic,nutritional)
(4) ischemia
(5) meningitis / encephalitis
0 45-80% of patientsdevelopmultiplesclerosiswithin
15 yearsof theirfirstepisodeof opticneuritis!
. ipsilateralorbitalpain on eye movement
. suddenonsetof unilaterallossof visionover several
hoursto severaldays
CT:
^/ normal/ mildlyenlargedopticnerve+ chiasm
^/ may show enhancement
344 RadiolagyReview Manual

MR: ^/ ttuid-ttuidlevelsfrom breakdownof recurrent

of opticnervewell
r/ mildenlargement+ enhancement hemorrhagein subhyaloid(betweenvitreous+ retina)
demonstratedon axialTlWl / subretinalspace(betweensensory+ pigment
Prognosis; spontaneousimprovementof visualacuity epi thel i um)
within1-2 weeks NO calcifications
MR:
hyperintense vitreousbody on Tl Wl + T2Wl from
PERSISTENT HYPERPLASTIC PRIMARY chronicblooddegradation products(methemoglobin) /
VITREOUS proteinaceous fluid
= ta;taconditionwith persistence+ proliferationof r/ nypo-to isointensethin triangularbandwith base
embryonichyaloidvascularsystemof primaryvitreous near opticdisc and apex at posteriorsurfaceof lens
due to arrestof normalregression r/ markedenhancementof fibrovascularmass within
May be associated with: vitreous
any severeocularmalformationI opticdysplasia/ Cx: (1) Glaucoma,cataractfrom recurrentspontaneous
intravitrealhemorrhage(dueto friablevessels)
t r is om y13
0 Bilaterality is a featureof a congenitalsyndrome(Norrie (2) Proliferationof embryonictissue
(3) Retinaldetachmentfrom organizinghemorrhage
disease,Warburgdisease)!
/ traction
- (4) Hydrops/ atrophyof globe + resorptionof lens
Primaryvitreous
= fibrillarectodermalmeshwork+ mesodermaltissue (5) Phthisisbulbi(scarredshrunkeneye)
consistingof embryonichyaloidvascularsystem;
appearsduring 1st monthof life;extendsbetween
lens + retina;involutesby 6th monthof gestation OF ORBIT
PSEUDOTUMOR
- Hyaloidartery = ID IOP A TH IC IN FLA MMA TORP YS E U D OTUM O R
= importantsourceof intraocularnutritionuntil8th = nongror'tulomatous inflammatoryprocessaffectingall
monthof gestation;arisesfrom dorsalophthalmic intraorbitalsoft tissues
arteryat 3rd week of gestation;grows anteriorlywith Etiology:
branchessupplyingvitreous+ posterioraspectof (a) cause not apparentat time of study: bacterial,viral,
lens foreignbody
- SecondaryI adultvitreous (b) systemicdiseasepresentlynot apparent:
beginsto form during3rd gestationalmonth;a watery sarcoidosis, collagen,endocrine
mass of loosecollagenfibers+ hyaluronicacid (c) idiopathic:probablyabnormalimmuneresponse
graduallyreplacesprimaryvitreous,which is reduced tncidence: 25h of all cases of unilateralexophthalmos;
to a smallS-shapedremnant(hyaloidcanal= Cloquet most commoncause of an intraorbitalmass
canal)and servesas lymphchannel l esi oni n adul t
Age: youngfemale
. unilateralleukokoria(2nd most commoncause)l2-3% Histo: lymphocyticinfiltrate
of leukokoriacases] May be associated with:
. seizures,mentaldeficiency,hearingloss Wegenergranulomatosis, sarcoidosis, f ibrosing
. t cataract mediastinitis, retroperitoneal fibrosis,thyroiditis,
. ophthalmoscopy:S-shapedtubularmass extending chotangitis, vasculitis,lYmPhoma
. unilateralpainfulophthalmoplegia
betweenposteriorsurfaceof lens + regionof optic nerve
. proptosis,chemosis,lid injection
head;lensopacitymay precludediagnosis
= srn€Illhypoplasticglobe . limitationof ocularmovement
ri microphthalmia
r/ retinaldetachment(due to vitreoretinaltractionin 30%) Location: retrobulbartat (76%),extraocularmuscle
US: (57%),optic nerve (38%),uveal-scleralarea
r/ hyperechoicband extendingfrom posteriorpole of (33%),lacrimalgland(5%)
globe to posteriorsurfaceof lens (= embryonicrest of (a) tumefactivetyPe (common)
primaryvitreous) { discrete/ poorlydefinedintra-/ extraconalmass
r/ centralanechoicline (= persistenthyaloidartery) = "pseudotumor"closeto surfacemarginof globe
visiblein casesof echogenicvitreoushemorrhage (b) myositictype (unusual)
! hyperechoicband extendingfrom papillato ora rl enlargementof one I more extraocularmuscles
serrata(= retinaldetachment) closeto insertionin globewith ill-defined margins
CT: { typicallyinvolvesmuscles+ tendoninsertions
./ enhancingcone-shapedcentralretrolentaldensity (DDxto Gravesdiseasewith muscleinvolvement
extendingfrom lensthroughvitreousbodyto backof onlY)
orbit,just lateralto oPticnerve { increaseddensityof retro-orbitalfat (may involve
r/ small optic nerve anteriorcomPartment)
! deformityof globe+ lens r/ thickeningand enhancementof scleranearTenon
{ hyperdensevitreous(fromprevioushemorrhage) capsule
 Orbitaland OcularDisorders 345

r/ enlargedlacrimalgland Types:
! proptosis (A)Nonheritable form (66%)
MR: (1) Sporadicpostzygoticsomaticmutation
r/ lesionisointenseto fat on T2Wl (subsequentgenerationsunaffected)
Prognosis: Mean age at presentation: 23 months
(1) remitting/ chronic+ progressive course { unilateraldisease
(2) rapiddramatic+ lastingresponseto steroidtherapy (2) Chromosomal anomaly
DDx: (1) Lymphoma(maybe confusedwith lymphoma = rT'tot'losomy 13 / deletionsof 13q
pathologically)
clinically,radiographically, Associatedwith: microcephaly,ear changes,
(2) Thyroidophthalmopathy(taperingof distal facialdysmorphism, mental
muscles,painlessproptosis) retardation,finger+ toe
(3) Radiationtherapy abnormalities, malformationof
genitalia
(B )H eri tablform
e
RETINALASTROCYTOMA (1) Heritablesporadicform (20-25%)
= low-gradeneoplasm/ hamartomaarisingfrom the nerve = sporadicgerminalmutation(50%chanceto
fiber layer of retina/ optic nerve,usuallyassociatedwith occurin subsequentgenerations)
tuberoussclerosis Mean age at presentation: 12 months
Etiology: tuberoussclerosis(53%);neurofibromatosis ./ bilateralretinoblastomas in 66%
type 1 (14%);sporadic(33%) (2) Familialretinoblastoma (5-10%)
Path: usuallymultiple+ bilateralin tuberoussclerosis; = autosomaldominantwith abnormality of band
(1) smallflat noncalcified lesionin
semitranslucent 14 in chromosome13 (95%penetrance)
posterior/ peripheralretina Mean age at presentation: 8 months
(2) "mulberry"fesion= raisedwhitetumor in { usually3 to 5 oculartumorsper eye
posteriorretinawith fine nodularitycontaining ^/ bilateraltumorsin 66%
calcifications + cysticfluidaccumulations Riskof secondarynonocularmalignancy:
Histo: spindle-shapedfibrousastrocytes osteo-, chondro-,fibrosarcoma, malignant
. leukokoria(3/" of all childhoodcasesof leukokoria) fibroushistiocytoma (20o/"riskwithin10 years,
. asymptomatic,progressiveloss of vision >90/" by 30 years of age)
Location: retinanearopticdisc Trilateral retinoblastoma (rarevariant)
r/ retinalmass+ enhancement = bilateralretinoblastomas + neuroectodermal
r/ typicallyunilateral(DDxto drusen) pinealtumor (pineoblastoma)
Cx: (1) Centralretinalvein occlusion+ secondary Quadrilateral retinoblastoma
hemorrhage = trilateralretinobfastoma + 4th focus in
(2) Neovascular glaucoma suprasellar cistern
(3) Extensivetumornecrosis
lncidence; 1:15,000-34,000 livebirths;mostcommon
intraocular neoplasmin childhood;1"/" oI all
pediatricmalignancies
RETINALDETACHMENT Age: meanage at presentation is 18 months;
Cause: trauma,
tumor,
exudative
/ inflammatory 98% i n chi l dren< 5 yearsof age; M:F = 1: 1
process,scar Path: (1) Exophyticform = proliferationinto subretinal
US: space with detachmentof retina+ invasionof
curvilinear area of highechogenicity
fixedat opticdisk vascularchoroid(hematogenousspread)
(= papilla)+ extendingto ora serrata (2) Endophytic form = centripetal tumorinvasion
V-shaped(withtotal detachment) causingfloatingislandsof tumorwithin
in one quadrantonly (partialdetachment) semiliquidvitreous+ anteriorchamber
thickfoldedretinawith lossof mobility(long-standing (3) Diffuseform = thin en-plaquelesionextending
detachment) alongretina
r/ subretinalspace normal/ occupiedby blood, Histo: (a) Flexner-Wintersteiner rosettes(in 50%)
inflammation / tumor(dependingon cause) = r'r€Ufor]€ll
cells line up aroundan empty central
DDx: vitreousmembranes,choroidaldetachment(point zone filledwith polysaccharides
of fixationnot at papilla) 0 Very specificfor retinoblastomas!
(b) Homer-Wright rosettes= fleUfon?lcellslineup
arounda centralarea containinga cobwebof
filaments(alsofoundin otherprimitive
RETINOBLASTOMA neuroectodermal tumors)
= fdfe malignantcongenitalintraocular
tumorarisingfrom (c) "fleurettes" = flowerlikegroupingsof tumorcells
primitivephotoreceptor
cellsof retina(includedin that form photoreceptorelements(specificfor
primitiveneuroectodermal tumorgroup) retinaldifferentiation)
346 Radiology Review Manual

. "cat'seye" - leukokoria(whitishmass behindlens)in RETROLENTAL FI BROPLASIA

60% = RETINOPATHY OF PREMATURITY
0 About 50/" of all childhoodleukokoriaare causedby = bilateraloften asymmetricpostnatalfibrovascular
retinoblastoma! organization of vitreoushumor,whichusuallyleadsto
. decreasedvisualacuity,heterochromiairidis retinaldetachment
. strabismus(crossedeyes),proptosis(lesscommon) Pathophysiology:
. hyphema retinalvascularization occursin 4th-9th monthsof fetal
. iris neovascularization, phthisisbulbi life progressingfromthe papillato the periphery;
. ocularpainfrom secondaryangle-closure glaucoma vascularization is incompletein prematureneonates
Location: posterolateral wall of globe(mostcommonly); especiallyin temporalsectors
60% unilateral; 40% bilateral+ frequently Predisposed: prematureinfantswithrespiratory
synchronous(90%bilateralin inheritedforms) distresssyndromerequiringprolonged
r/ normalocularsize oxygentherapy
US: Severitydirectlyrelatedto:
r/ heterogeneous hyperechoic solidintraocular
mass (1) degreeof prematurity
{ cysticappearanceupon tumor necrosis (2) birthweight
{ secondaryretinaldetachmentin all cases (3) amountof oxygenused in therapy
{ ac ous t ics h a d o w i n g(i n 7 5 /.) . leukokoriain severecases (tractionretinaldetachment,
r/ vitreoushemorrhagefrequent usuallybilateral+ temporal)[3-5% of all childhood
CT : leukokoriacasesl
{ solidsmoothlymarginatedlobulatedretrolental . Ophthalmoscopic stages:
hyperdensemass in endophytictype (rarerexophytic =
l"stase
type grows subretinallycausingretinaldetachment)
! partialpunctatei nodularcalcification(50-75-95%)
3ffJ55:i,T:"#[%:Li,;:'#$l,l:i:
avascularretina(fromspasm as a
0 Retinoblastoma is the mostcommoncauseof reactionto hyperoxygenation)
orbitalcalcifications! 2ndstage = dilatation+ elongation+ tortuosityof
^/ densevitreous(common) retinalvessels(afteroxygenwithdrawal)
r/ extraocularextension(in 25%): optic nerve 3'dstage = retinalneovascularization with growthinto
enlargement, abnormalsofttissuein orbit,intracranial vitreousleadsto vitreoushemorrhage
extension 4thstage = fibrosiswith retractionof fibrovascular
r/ contrastenhancementusual tissue+ retinaldetachment
r/ + macrophthalmia r/ bilateralmicrophthalmia + retinaldetachment
MR: US:
./ iso-to mildlyhyperintense tumoron TlWl relativeto r/ hyperechoictractsextendingfrom temporalside of
vitreous+ moderateto markedenhancement peripheryof retinato vitreousbehindthe lens
r/ distinctlyhypointense on T2Wl (similarto uveal CT:
melanoma) { densevitreousbilaterally(neovascularingrowth)
{ subretinalexudateusuallyhyperintense on Tl Wl { + dystrophiccalcifications in choroid+ lens (late
+ T2Wl (proteinaceous fluid) stage)
Cx: (1) Metastasesto: meninges(viasubarachnoid MR:
space),bone marrow,lung,liver,lymphnodes i/ hyperintense vitreouson TlWl + T2Wl (fromchronic
(2) Radiation-induced sarcomasdevelopin 15-20/" subretinal hemorrhage)
Prognosis; spontaneousregressionin 1"/"; r/ hypointenseretrolentalmass (appositionof detached
r/ calcifications= favorableprognosticsign leavesof retinadisplacedfrom retinalpigmentlayer)
r/ contrastenhancement= poor prognosticsign Prognosis:
Mortality: (1) spontaneousregressionof vitreous
(a) choroidalinvasion: 65/" if significant,24o/oif slight neovascularization (85-95%)+ retinaldetachment
(b) optic nerve invasion: (2) progressionto cicatricialstagecharacterizedby
< 1O h if n o t i n v a d e d formationof dense membraneof gray-white
15% rtthroughlaminacribrosa vascularizedtissuein retrolentalvitreous+ retinal
44% rt significantlyposteriorto laminacribrosa detachment+ microphthalmia
(c) marginof resectionnot free of tumor: >65/" DDx: (1) Retinoblastoma (calcifications
in eye of normal
DDx: (1) RetinorT'r? = retinocytoma(benignvariant) size)
(2) Toxocaracanis infection(no calcification)
(3) Retrolental fibroplasia(microphthalmia)
(4) Coatsdisease(subretinal exudation,no RHABDOMYOSARCOMA
calcification) Mostcommonprimarymalignantorbitaltumorin childhood
(5) Norriedisease(retinaldysplasia) 0 10%occurprimarilyin orbit
(6) Persistenthyperplasticprimaryvitreous 0 10% metastasizeto / invadeorbit
(hypoplasticglobe,no calcification) lncidence: 34o/" of all pediatricorbital masses
 Orbitaland OcularDisorders 347

Histo: arisingfrom undifferentiated mesenchymaof US:

orbitalsoft tissues(not from striatedmuscle) r/ smallflat hyperechoicsolid mass
(1) embryonaltype (75%) CT:
(2) alveolartype (1S%) r/ ilt-definedhyperdensethickeningof wall of globewith
(3) pleomorphic type (10%) inwardbulge
Age at presentation: average 7 years; g0% by 16 MR:
years of age; M > F { sharplycircumscribed hyperintense lesionon TlWl
Rarely associatedwith: neurofibromatosis (paramagneticpropertiesof melanin)
. rapidlyprogressiveexophthalmos+ proptosisof upper Metastasesto: globe,opticnerve;liver,lung,subcutis
t id
Location: superiororbit/ retrobulbar (71"/"),lid(22%),
conjunctiva(7%) VARIXOF ORBIT
{ largesoft-tissuedensitymass with ill-definedmargins Etiology: (a) Congenital:venous malformation/ venous
(extraocularmusclesnot involved) wall weakness
r/ + extensioninto preseptalspace,adjacentsinus,nasal (b) Acquired: intraorbital/ intracranialAVM
cavity,intracranialcavitywith bony erosion . intermittentexophthalmosassociatedwith straining
{ may show significantenhancement . frequentblindness
US: r/ involvement of superior/ inferiororbitalvein;phleboliths
{ heterogeneous well-definedirregularmass of low to rare
mediumechogenicity
1 r"y producebony erosionwithoutscleroticreaction
Metastases:lung, bone marrow,cervicallymph nodes tr enlargement of massduringValsalvamaneuver/
(rare) jugularvein compression
,
Prognosis; (1) 40% survivalafter exenteration tr well-defined markedlyenhancingmass
(2) 80-90% survivalafter radiationtherapy r/ spontaneous thrombosis(common)
(4,000-5,000rad) + chemotherapy US:
(vincristine,
cyclophosphamide, { anechoictubular/ ovalstructure+ thrombus
Adriamycin@) r/ venousflow increasingwith Valsalva
DDx: pseudotumor,lymphoma MR:
./ ftow void (rapidflow)i flow-relatedenhancement
UVEALMELANOMA (slowflow)
Most commonprimaryintraocularneopfasmin adult
Caucasian WARBURGDISEASE
Age: 50-70 years = autosomalrecessivesyndromecharacterizedby
Location: choroid(85-93%) > ciliarybody (4-9%) > iris (1) bilateralpersistenthyperplastic
primaryvitreous
(3-6%); almostalwaysunilateral (2) hydrocephalus,lissencephaly
. retinaldetachment,vitreoushemorrhage (3) mentalretardation
. astigmatism, glaucoma . bilateralleukokoria+ microphthalmia
348 Radiology Review Manual
 DIFFERENTIALDIAGNOSISOF EAR, NOSE,AND THROATDISORDERS

FACIALNERVEPARALYSIS membranouslabyrinth
A. I NT RA CRA N IAL SE G ME N T (1) enhancement:labyrinthitis, Cogan
(a) intraaxial syndrome(earlyphaseof autoimmune
brainstemglioma,metastasis, multiplesclerosis, interstitial
keratitis),intralabyrinthine
cerebrovascular accident,hemorrhage schwannoma, site of postinflammatory
. cranialnerveVl also involved perilymphatic fistula
(b) extraaxial (2) obliteration:labyrinthitis ossificans,
Cogan
CPA tumor(acousticneuroma,meningioma, syndrome(latephase)
epidermoid), CPA inflammation (sarcoidosis,
basilar (3) hemorrhage:trauma,labyrinthitis,
meningitis),
vertebrobasilar dolichoectasia,
AVM, coagulopathy,tumor fistulization
aneurysm (4) M6nidredisease(vertigo+ fluctuating
. cranialnerveVlll also involved sensorysensorineural hearingloss)
B. I NT RA T E M POR ALS EGME N T 0 MRI is the modalityof choice!
f racture, cholesteato
ma, paraganglio ma, hemang ioma, (b) neural/ retrocochlear SNHL (morecommon)
facialnerveschwannoma,metastasis,Bell palsy,otitis = abnormalities of neuronsof spiralganglion
media + centralauditorypathways
. lossof lacrimation, hyperacusis, lossof taste IAC / cerebellopontine angle
C. EXTRACRANIAL PAROTIDSEGMENT (1) neoplasticlesions:vestibular/ trigeminal
forcepsdelivery,penetratingfacialtrauma,parotid schwannoma(acousticneuroma in 1"/"),
surgery,parotidmalignancy, malignantotitisexterna meningioma, arachnoidcyst,epidermoid
. preservationof lacrimation,stapediusreflex,taste cyst,leptomeningeal carcinomatosis,
lymphoma,lipoma,hemangioma
EAR (2) nonneoplastic lesion: sarcoidosis,
HearingDeficit meningitis, vascularloop,siderosis
A . CO NDUCTIV E H EA R IN GL O S S intraaxialauditorypathway
. decreasein air conductionvia EAC, tympanic (brainstem, thalamus,temporallobe)
membrane,ossicularchain,ovalwindow(sound (1) i schemi cl esi on
via headphones) (2) neoplasticlesion
. rtofrTl?lboneconduction(soundvia bone (3) traumaticlesion
oscillator) (4) demyelinating lesion
(a) trauma: incudostapedial / malleoincudal 0 MRI is the modalityof choice!
subluxation ; incusdislocation; stapes
dislocation; stapes/ malleusfracture Pulsatile
Tinnitus
(b) destructionof ossicularchain: otitismedia t VascularTympanicMembrane
(c) restrictionof ossicularchain: fenestral = perceptionof a rhythmiccardiacsynchronous sound
otosclerosis of ringinglbuzzing / roaring
0 CT is the modalityof choice! A. No abnormality(20%)
B. SENSOBINEURAL HEARINGLOSS(mostcommon) B. Congenitalvascularvariants(21%)
. elevatedconductionthresholdsfor bone + air 1. AberrantICA
(a) sensoryi cochlear SNHL = damageto cochlea/ = feSUltof anastomosis of enlargedinferior
organof Corti(lesscommon) tympanicarterywith enlargedcaroticotympanic
bony labyrinth arterywhen cervicalICA is underdeveloped
(1) demineralization: otosclerosis 2. Dehiscentjugularbulb
(otospongiosis), osteogenesis imperfecta, 3. High-riding nondehiscent jugularbulb (= jugular
Pagetdisease,syphilis megabulb)
(2) congenitaldeformity:cochleardys-/ ^/ nighjugularbulbwith diverticulum projecting
aplasia,Michelanomaly,Mondini cephaladinto petroustemporalbone
dysplasia,enlargedvestibularaqueduct C. Acquiredvascularlesions(25%)
syndrome,X-linkedsensorineural hearing 1. D uralA V M
los 2. Extracranialarteriovenousfistula
(3) traumaticlesion: transversefracture, 3. High-gradestenoticvascularlesion:
perilymphaticfistula,cochlearconcussion carotidarteryatherosclerosis, f ibromuscular
(4) destructivelesion: inflammatory lesion, dysplasia,carotidarterydissection
neoplasticlesion 4. Aneurysminvolvinghorizontalsegmentof
0 CT is the modalityof choicel petrousICA
350 Radiology Review Manual

D. Temporalbonetumors(31%) D. MALIGNANTTUMOR
1. Paraganglioma(27"/"): 1. Squamouscell carcinoma
glomustympanicum, glomusjugulare . often long historyof chronicsuppurativeotitis
2. M ening i o ma media= "malignantotitis"
3. Hemangioma 2 . Basalcell carcinoma
E. Miscellaneous 3 . Melanoma,adenocarcinoma,adenoidcystic
1. Cholesterol granuloma carcinoma
4. Metastases
TemporalBoneSclerosis (a) hematogenous:breast,prostate,lung,
= otospongiosis
1. Otosclerosis kidney,thyroid
2. Pagetdisease= osteoporosis
circumscripta (b) directspread: skin, parotid,nasopharynx,
. sensorineural / mixedhearingloss (cochlear brain,meninges
involvement/ stapesfixationin oval window) (c) systemic: leukemia,lymphoma,myeloma
{ usuallylyticchangesbeginningin petrous 5 . HistiocytosisX: in 15'h of patients
pyramid+ progressinglaterally;otic capsulelast
to be affected
{ calvarialchanges+ basilarimpression MiddleEarMasses
3. Fibrous dysplasia A . C ON GE N ITA L
monostoticwith temporalbone involvement 1. Aberrant internal carotid artery
. painlessmastoidswelling . vasculartympanicmembrane
. conductivehearingloss (fromnarrowingof EAC / . pulsatiletinnitus
middleear) r/ tubularsoft-tissue densityenteringmiddleear
r/ homogeneously densethickenedbone (fibro- cavityposterolateral to cochlea,crossing
osseoustissue less densethan calvarialbone) mesotympanumalong cochlearpromontory,
{ expandedbone with preservedcortex exitinganteromedialto becomehorizontal
r/ tyticlesions(lessfrequent) portionof carotidcanal
r/ sparingof membranouslabyrinth,facialnerve { protrusioninto middleear withoutbony margin
canal,IAC is the rule 2. Dehiscentjugularbulb
4. Osteogenesisimperfecta B. INFLAMMATORY
r/ changessimilarto otosclerosis 1. Cholesteatoma
van der Hoeve syndrome = osteogenesis 2. Cholesterol granuloma
imperfecta+ otosclerosis+ blue sclera 3. Granulation tissue
5. M eningio ma r/ linearstrandspartiallyopacifyingmiddleear
6. Otosyphilis:labyrinthitis+ osteitis cavitywithoutbonYerosion
7. Metastasis C . B E N I G NT U M O R
8. Ossifyingfibroma 1. Adenomatous tumor(mixedpatterntype)
9. Osteosarcoma r/ intenseenhancement
1 0 . Osteopetrosis . { no osseousdestruction
2. Gl omustumor(mul ti pl e i n 10% ;8% ma lignant )
ExternalEar Masses (a) Glomustympanicum:at cochlearpromontory
A. CONGENITAL r/ seldomerodesbone
1. Atresia (b) Glomusjugulare: at jugularforamen
B. INFLAMMATORY ! invasionof middleear from below
1. Malignantexternalotitis { destructionof bony roof of jugularfossa
2. Keratosis obturans + bony spur separatingvein from carotid
bilateralprocessin associationwith chronic artery
sinusitis+ bronchiectasis 3. Facialnerveschwannoma
Age: <40 years . persistentBell palsy (in 5% causedby
3. Cholesteatoma neurinoma)
C. B E NI G NT U MOR Location: intracanalicular > IAC
1. Exostosis = surfer'sear r/ tubularmass in enlarged/ scallopedfacial
Cause: irritationby cold water canal
{ bony mass projectinginto EAC; often multiple 4. Ossifyinghemangioma
+ bilateral 5. Choristoma= ectoPicmaturesalivarytissue
2. Osteoma 6. Endolymphatic sac tumor
r/ may invadeadjacentbone; singlein EAC / ^/ arisesfrom regionof vestibularaqueduct
mastoid 7. Meni ngi oma
3. Ceruminoma D . MA LIGN A N T TU MOR
from apocrine+ sebaceousglands;bone erosion 1. Squamouscell carcinoma
mimicsmalignancy 2. Metastasis
 Differential Diagnosis of Ear, Nose, and Throat Disorders gTl

3. Rhabdomyosarcoma 3. Sinonasalpolyp
Location: orbit > nasopharynx> ear 4. Antrochoanal polyp
4. Adenocarcinoma (rare),adenoidcysticcarcinoma 5. Invertingpapilloma
6. S i nusi ti s
Masson Promontory 7. Carcinoma
[promontorY= bone over basalturn of cochlea]
1. G lom ust y m p a n i c u m GranulomatousLesions of Sinuses
2. Congenitalcholesteatoma A. Chronicirritants
3. Aberrantcarotidartery 1. Beryllium
4. Persistentstapedialartery 2. Chromate salts
B. Infection
InnerEar Masses 1. Tuberculosis
A . CO NG E N IT AL 2. Actinomycosis
1. Congenital/ primarycholesteatoma = epidermoid 3. Rhinoscleroma
tumor(3rdmostcommonCpA tumor) 4. Yaws
B. INFLAMMATION 5. Blastomycosis
1. Choles te roglra n u l o ma 6. Leprosy
2. Petrousapex mucocele 7. Rhinosporidiosis
C. T UM O R 8. Syphilis
1. G lom u sj u g u l a retu m o r 9. Leishmaniosis
2. Hemangioma, fibro-osseouslesion 10. Glanders
3. Metastasis C. Autoimmune disease
4. Facialnerveneurinoma 1. Wegener granulomatosis
5. LargeCPA tumors: acousticneuroma, D. Lymphoma-like lesions
meningioma(2nd mostcommonCpA tumor) 1. Midlinegranuloma
E. Unclassified
SINUSES 1. Sarcoidosis
Opacification
of MaxillarySinus
A. WITHOUT BONE DESTRUCTION HyperdenseSinus Secretions
1. Sinusaplasia/ hypoplasia 1. Inspissatedsecretions
Age: NOT routinelyvisualizedat birth,by age 6 2. Fungalsinusitis
antralfloor at levelof middleturbinate,by 3. Hemorrhage intosinus
a g e 1 5 o f a d u l ts i z e 4. Chronic sinusitis
infected
withbacteria (inparticular
Location: uni-/ bilateral in verylong-standingdisease/ cysticfibrosis)
{ depressionof orbitalfloorwith enlargement of
orbit Opacified Sinus & Expansion/ Destruction
r/ lateraldisplacementof lateralwall of nasal mnemonic;"PLUMPFACIES"
fossa with largeturbinate Plasmacytoma
2. Maxillarydentigerouscyst Lymphoma
usuallycontaininga tooth/ crown;withouttooth= Unknown etiology:Wegener
granulomatosis
primordialdentigerous cyst Mucocele
3. Ameloblastoma Polyp
4. Acutesinusitis Fibrousdysplasia,
Fibroma(ossifying)
r/ air-fluidlevel Aneurysmal bonecyst,Angiofibroma
B. WITH BONE DESTRUCTION Gancer
1. Maxillarysinustumor lnvertingpapilloma
2. Infection:aspergillosis, mucormycosis, TB, Esthesioneu roblastoma
syphilis Sarcoma:ie,rhabdomyosarcoma
3. Wegenergranulomatosis; lethalmidline
granuloma NOSE
4. Blowoutfracture Nasal Vault Masses
A. BENIGN
ParanasalSinusMasses 1. Sinonasal polyp
1. M uc oc ele 2. Invertedpapilloma
Cause: obstructionof a paranasalsinus 3. Hemangioma
r/ + bone remodeling/ sinusexpansion . historyof epistaxis
2. Mucus retentioncyst 4. fvogenicgranuloma
Cause: obstructionof small seromucinousgland ! pedunculated lobularmass
Location: commonlyin floorof maxilla 5. Granuloma gravidarum
r/ smoothlymarginatedsoft-tissuemass = I'l3S?l
hemangioma of pregnancy
352 RadiologyReviewManual

6. Hemangiopericytoma B. MALIGNAN'I
7. Juvenilenasopharyngeal angiofibroma 1. Squamouscellcarcinoma
{ arisesin superiornasopharynxwith extension ^i directextensionfrom pharyngealmucosal
intonosevia posteriorchoana space
B. MALIGNANT ri verticalextensionto skull base / hyoidbone
1. Ly m pho ma 2. SalivaryglandmalignancY
2. Melanoma
3. Vascularmetastasis
PharyngealMucosalSpaceMass
Mass in Nasopharynx 1. Asymmetricfossaof Rosenmuller
mnemonic; "NASALPIPE" = lateralpharyngealrecess= €lsYrTlffietry
in amount
Nasopharyngeal carcinoma of lymphoidtissue
Angiofibroma(juvenile) 2. Tonsillarabscess
. sore throat,fever,painfulswallowing
Spine/ skull fracture
Adenoids 3. Postinflammatory retentioncyst
Lymphoma ri t-2-cm well-circumscribed cysticmass
PoIYP 4. Postinflammatory calcification
. remotehistoryof severepharyngitis
lnfection
Plasmacytoma { multipleclumpsof calcification
Extensionof neoplasm(sphenoid/ ethmoidsinus ca.) 5. Benignmixedtumor
. pedunculated mass arisingfrom minorsalivary
CongenitalMidlineNasalMass glands
= resultof faultyregressionof embryologic dural { oval/ roundwell-circumscribed massprotruding
diverticulum throughforamencecum+ fonticulus into airway
frontalis(= hdsofrontalfontanel)from the prenasal 6. Squamouscell carcinoma
masswith epicentermedialto
r/ infiltrating
space
Frequency; 1:20,000to 1:40,000births + invadingparapharyngeal space
1. Dermoidcyst r/ middle-earfluid(eustachian tube malfunction)
r/ cervicaladenopathy
2. Epidermoidcyst
3. Nasalglioma= fl?s?lcerebralheterotopia 7. Non-HodgkinlymPhoma
4. Nasalencephalocele 8. Minorsalivarygtandmalignancy
5. Hemangioma / lymphangioma 9. Thornwaldtcyst
6. Dacryocystocele
7. Dacryocystitis
MasticatorSpaceMass
PHARYNX A BENIGN
SpaceMass
Parapharyngeal 1. Asymmetricaccessoryparotidgland
A . B E NI G N lncidence: 21"h of general population
1, Asymmetricpterygoidvenousplexus Location:usuallyon surfaceof massetermuscle
! racemose,enhancingarea alongmedialborder r/ prominentsalivaryglandtissue
of lateralpterygoidmuscle 2. BenignmasseterichYPertroPhY
2. Abscess Cause: bruxism(= nocturnalgnashingof teeth)
Origin: pharyngitis(mostcommon),dental r/ homogeneous enlargement of one / both
infection,parotidcalculusdisease, masseters
penetratingtrauma 3. Odontogenicabscess/ mandibularcysts
. bad dentition+ trismus
3. Atypicalsecondbranchialcleft cyst
Age: child/ youngadult 4. Lymphangioma, hemangioma
. protrudingparotidgland
. bulgingposterolateralpharyngealwall B. MALIGNANT
{ cysticmass projectingfrom deep marginof 1. Sarcoma(chondro-,osteo-,soft-tissue sarcoma,
faucialtonsiltowardskull base especiallyrhabdomyosarcoma in children)
4. Pleomorphic adenomaof ectopicsalivarytissue/ r/ infiltrating
masswith mandibulardestruction
of deep lobeof parotidgland(common) 2. Malignantschwannoma
5. Schwannoma,neurofibroma ^/ tubularmassalongcranialnerveV,
Origin: usuallyfrom cranialnerveX 3. Non-Hodgkin lymPhoma
r/ carotid artery pushed anteriorly 4. Infiltratingsquamouscell carcinoma
6. Paraganglioma r/ extendingfrom pharyngealmucosa
{ posteriorto carotidartery 5. Salivaryglandmalignancy(mucoepidermoid
r/ extremelyvascular(numerousflow voids) carcinoma,adenoidcysticcarcinoma)
7. Lipoma ^l extendingfrom parotidgland
 DifferentialDiagnosisof Ear, Nose,and ThroatDisorders SSa

N.B.: (1) checkcourseof V. to foramenovalefor LARYNX

skull base extensionto Meckelcave area VocalCord Paralysis
+ cavernoussinus 1. Birthinjury
(2) check for extensionto pterygopalatine
2. Arnold-Chiari malformation
fossa to infraorbitalfissureinto orbit 3. Intracranialtumor
4. Mediastinalmass / cyst
CarotidSpaceMass 5. Vascularring
A. VASCULARLESION 6. Thyroidectomy
1. Ectaticcommon/ internalcarotidartery 7. Malignancy
2. Carotidarteryaneurysm/ pseudoaneurysm r/ fixedvocal cords (fluoroscopy)
3. Asymmetricinternaljugularvein
4. Jugularveinthrombosis
B . B E N I G NT U M O R Epiglottic Enlargement
1. Paraganglioma (carotidbodytumor+ glomus A. NORMALVARIANT
jugulare+ glomusvagale)
1. Prominentnormalepiglottis
2. Schwannoma 2. Omega epiglottis
^/ displacementof carotidarteryanteromedially
B. INFLAMMATION
+ internaljugularvein posteriorly 1. Acute/ chronicepiglottitis
r/ well-encapsulated mass 2. Angioneurotic edema
3. Neurofibromaof cranialneryeslX. X. Xf 3. Stevens-Johnson syndrome
4. Branchialcleftcyst 4. Causticingestion
C. M A LI G NAN T U M O R
5. Radiationtherapy
1. Nodalmetastasisfrom squamouscell carcinoma
C. MASSES
to interiorjugularchain(common) 1. Epiglotticcyst
r/ encasementof carotidartery= inoperable 2. Aryepiglotticcyst
2. Non-Hodgkin lymphoma 3. Foreignbody
Retropharyngeal
SpaceMass
A . I NF E CT I ON AryepiglotticCyst
1. Reactivelymphadenopathy 1. Retentioncyst
r/ nodes>10 mm in diameter 2. Lymphangioma
2. Abscess: r/ bow-tieshape 3. Cystichygroma
B . B E N I G NT U M O R 4. Thyroglossalcyst
1. Hemangioma . may be symptomaticat birth
2. Lipoma r/ well-definedmass in aryepiglotticfold
C. M A LI G NA N T U M O R
1. Metastasisto retropharyngeal nodes
from nasopharyngeal squamouscefl carcinoma, LaryngealNeoplasms
melanoma,thyroidcarcinoma A. SQUAMOUS CELLCARCTNOMA
(e5-e8%)
N.B.: sentinelnodeof Rouviere(= lateral . endoscopically visibledue to mucosalinvolvement
retropharyngeal node)is an early sign of B. NON-SQUAMOUS CELL NEOPLASMS(2-5%)
nasopharyngeal cancerbeforeprimary mal i gnant:beni = gn1:1
mass becomesobvious (a) vasoformati ve tumor .......... . . . . 33%
2. Non-Hodgkin lymphoma Beucru
3. Directinvasionby squamouscell carcinoma 1. H emangi oma
2. Lymphangioma
PrevertebralSpaceMass 3. Angiofibroma
A . P S E UDO TU M O R 4. Angiomatosis
1. Anteriordisk herniation 5. Granulomapyogenicum
2. Vertebralbody osteophyte 6. Arteriovenous fistula
B . I NF LA M M A T IO N 7. Phlebectasia, telangiectasia
1. Vertebralbody osteomyelitis Mnlrcunrur
2. Abscess 1. Angiosarcoma (Kaposisarcoma)
r/ extensionfrom retropharyngeal space/ Locati on: epi gl otti s(mostfrequent )
osteomyelitis/ diskitis/ epiduralabscess) r/ intenselyenhancingmass
C. T UM O R 2. Hemangiopericytoma
1. Chor do m a ( b )c h o n d r o g e ntiuc m o r ...............20y"
2. Vertebralbody metastasis:lung, breast, 1. C hondroma
prostate,non-Hodgkin lymphoma,myeloma 2. Chondrosarcoma
r/ metastasesto prevertebralspace = inoperabfe 3. Osteosarcoma
354 Radiology Review Manual

( c ) hem at o p o i e tituc mo r ..............12% (d) Traumatic: Hematoma,foreignbody

1. Hodgkin/ non-Hodgkin lymphoma/ leukemia (e) Metabolic: HypothYroidism
2. Plasmacytoma
3. Pseudolymphoma Vallecular Narrowing
( d )s a l i v a r gy l a n dt u m o r ...............10% = valleySon each side of glossoepiglottic
folds
1. Pleomorphic adenoma betweenbase of tongue+ epiglottis
2. Adenoidcysticcarcinoma (a) Congenital: Congenitalcyst, ectopicthyroid,
3. Mucoepidermoidcarcinoma thyroglossalcyst
4. Adenocarcinoma (b) Inflammatory:Abscess
(e) fattytissuetumor ......7o/o (c) Neoplastic: Teratoma
1. Lipo m a (d) Traumatic: Foreignbody,hematoma
2. Liposarcoma
( f ) m et as ta s i s ......... .... ...7o/o Sup rag lottic Narrowing
skin (melanoma)> kidney> breast> lung> = arre?betweenepiglottisand true vocal cords
prostate> colon > stomach > ovary (a) Congenital: Aryepiglotticfold cyst
( g ) n e u r o g e n ti cu m o r. . . . . . . . . . . . . . .......5% (b) Inflammatory: Acute bacterialepiglottitis,
( h) m y oge n i ctu mo r .... ....2/" angioneuroticedema
( i) f ibr ohis ti o c y tu ti cm o r ................2/" (c) Neoplastic: Retentioncyst, cystichygroma,
neurofibroma
(d) Traumatic: Foreignbody,hematoma,radiation,
AIRWAYS causticingestion
InspiratoryStridorin Children (e) ldiopathic: LarYngomalacia
1. Croup
2. Congenitalsubglotticstenosis
3. Subglottichemangioma Glottic Narrowing
4. Airwayforeignbody = altal.of true vocal cords
5. Esophagealforeignbody (a) Congenital: Laryngealatresia,laryngeal
6. Epiglottitis stenosis,laryngealweb (anterior
commissure)
AinrvayObstructionin Children (b) Neoplastic: Laryngealpapillomatosis
Nasopharyngeal Narrowing (c) Neurogenic: Vocalcord paralysis(mostcommon)
(a) Congenital: Choanalatresia,choanalstenosis, (d) Traumatic: Foreignbody,hematoma
encephalocele
(b) lnflammatory: Adenoidalenlargement,polyps Subglottic Narrowing
(c) Neoplastic: Juvenileangiofibroma, = short segmentbetweenundersurfaceof true vocal
rhabdomyosarcoma, teratoma, cords+ inferiormarginof cricoidcartilageis the
neuroblastoma,lymphoepithelioma narrowestportionof child'sairway
(d) Traumatic: Foreignbody, hematoma,rhinolith (a)C ongeni tal : C ongeni tal subgl otti cst enosis
(b)l nfl ammatory:C rouP
Oropharyngeal Narrowing (c) Neoplastic: Hemangioma, papillomatosis
(a) Congenital: Glossoptosis+ micrognathia(Pierre (d) Traumatic: Acquiredstenosis(resultof
Robin,Goldenhar,TreacherCollins prolongedendotrachealintubation
syndrome),macroglossia i n 5/" ), granul oma
(cretinism,Beckwith-Wiedemann (e) ldiopathic: Mucocele= ITlucousretentioncyst
syndrome) (rarecomplicationof Prolonged
(b) Inflammatory: Abscess,tonsillarhypertrophy endotrachealintubation)
(c) Neoplastic: Lingulartumor/ cyst
(d) Traumatic: Hematoma,foreignbody
NECK
Retropharyngeal Narrowing Solid NeckMass
= potentialspace (normally<3/4 of AP diameterof Solid Neck MassIn Neonate
adjacentcervicalspine in infants/ <3 mm in older 1. Cystichygroma
children) 2. H emangi oma
(a) Congenital: Branchialcleft cyst, ectopicthyroid 3. Neuroblastoma
(b) lnflammatory: Retropharyngeal abscess 4. Teratoma
(c) Neoplastic: Cystichygroma(originatingin 5. colli
Fibromatosis
posteriorcervicaltrianglewith
extensiontoward midline+ into Solid Neck Mass in Childhood
mediastinum), neuroblastoma, 1. Lymphadenopathy
neurofibromatosis,hemangioma colli
2. Fibromatosis
 DifferentialDiagnosisof Ear, Nose,and rhroat Disorders gss

3. Aggressivefibromatosis @ S U B MA N D IB U LA R
4. Malignancy:neuroblastoma (mostcommon), skin,submandibular gland,baseof tongue
lymphoma,embryonalrhabdomyosarcoma @ P OS TE R TOR TR TA N GLE
5. Teratoma nasopharynx, base of tongue
6. Hem ang i o ma @ LATERALPHARYNGEAL
7. Cervicothoracic lipoblastomatosis nasopharynx,oropharynx
(a) lipoblastoma= circumscribedencapsulated
form (2/3) Congenital Cystic Lesions of Neck
(b) lipoblastomatosis = diffuseinfiltrativeform 1. Thyroglossal duct cyst
( 1/ 3) Location: anteriorcervicaltrianglecloseto midline
Histo: mature+ immaturefat cells betweenforamencecum + thyroid
r/ regionsof fat attenuationseparatedby septa i sthmus
{ NO enhancement 2. Lymphangioma/cystic hygroma
8. Lipoma Location: mostlyin posteriorcervicaltriangle,
9. Thyroidmass/ lingualthyroid occasionally in floorof mouthi tongue
10. Parathyroidadenoma 3. Branchialcleft anomalies
11. E c t opict h y m u s . often notedduring upper respiratoryinfection
(a) 2nd branchialcleftcyst
Lymph Node Enlargement of Neck Location: near angle of mandibleanteriorto
A . NO RM A LLY MP HN O D ES sternocleidomastoid muscle
(b) branchialcleftfistula
r/ few small oval hypoechoic
Location: apex of piriformsinusto thyroid
r/ + centrallinearechogenicity (= inysginating
hilar 4. Cervicaldermoid/ epidermoidcyst
fat)
Location: floorof mouth
{ largerin transversethan anteroposterior
5. Cervicalthymiccyst
dimension
6. Parathyroid cyst
B . CE RV I CA LAD EN IT IS
Age: 30-50 years
Location: posteriorcervicaltriangle . hormonallyinactive
1. Tuberculous adenitis
r/ noncolloidal cyst near lowerpoleof thyroidgland
r/ multichambered centrallyhypoattenuating mass 7. Cervical bronchogenic cyst
r/ tnicf enhancingrim
Cause: anomalousforegutdevelopment
r/ peripheralcalcifications
Histo: columnarciliatedpseudostratified
C. MALIGNANTLYMPHNODES
epitheliallining
r/ increasedanteroposterior diameter
M : F= 3 : 1
r/ prominentcalcifications suggestiveof medullary . drainingsinusin suprasternal
thyroidcancer notch/
r/ axialdiameterof >15 in jugulodigastric supraclavicular area
region/
r/ cyst up to 6 cm in diameter
>11 mm elsewhere(in squamouscell carcinoma)
r/ indentationof trachea
CT:
8. Laryngocele
r/ marginalenhancement
{ centralnecrosis(regardlessof size)
4 tuzzy bordersas sign of extracapsularextension Air-containing
Massesof Neck
1. Laryngocele
Low-density Nodeswith Peripheral 2. Trachealdiverticulum
arisingfrom anteriorwall of tracheaclose to thyroid
Enhancement
3. Zenkerdiverticulum
1. T uber c ulo s i s
4. Lateralpharyngealdiverticulum
2. Metastaticmalignancy
locatedin tonsillarfossa/ valleculai pyriformfossa
3. Lymphoma
4. Inflammatory conditions
SALIVARY GLANDS
Lymph Node Metastasisby Location ParotidGlandEnlargement
@ SUPRACLAVTCULAR A. LOCALIZEDINFLAMMATION / INFECTION
head & neck, lung,breast,esophagus 1. Chronicrecurrentsialadenitis
@ I NT E RNA LJ U G U L AR 2. S i al osi s
supraglotticlarynx,esophagus,thyroid 3. Sarcoidosis
@ M I DJ UG U L AR 4. Tuberculosis
tongue,pharynx,supraglotticlarynx 5. Cat-scratchfever
@ J UG ULO D T G A ST R T C 6. S yphi l i s
nasopharynx,oropharynx,tonsils,parotidgland, 7. Parotidabscess secondaryto acute bacterial
supraglotticlarynx (suppurative)sialadenitis
356 RadiologyReviewManual

8. Reactiveadenopathy 2 . Organificationdefect
9. Parotitis:mumps(mostcommonparotiddisease = deficientperoxidaseactivity,which catalyzesthe
H IV
in c hild re n ), oxidationof iodidebY HrO, to form
B . S Y S T E M ICA U T OIMMU N E R EL A T EDD IS E A S E monoiodotyrosine (MlT)/ diiodotyrosine (DlT)
1. Siogrendisease(= mYoepithelial sialadenitis) . highserumTSH
2. Mikuliczdisease . low serumTo
C. NEOPLASM . diffusesymmetricthYromegalY
Frequency: 90-957o occur in parotid gland, 5% in r/ frigl''thyroidaluptake of radioiodine/ pertechnetate
gl ands;
s u b m a n d i b u l a+rs u b l i n g u al r/ rapidl-131turnover
only 1Yo of all pediatric tumors! r/ positiveperchloratewashouttest
(a) benigntumor Pendred syndrome = autosomalrecessivetrait of
1. P le o m o rp h i/ cmo n o mo rp h iacd e n oma deficientperoxidaseregenerationcharacterizedby
2. Cystadenolymphoma (= Warthintumor) hypothyroidism + goiter+ nervedeafness
3. Benignlymphoepithelial cysts(AIDS) 3 . Deiodinase(dehalogenase) defect
4. Lipoma = deficientdeiodination of MIT / DIT to release
5. Facialnerveneurofibroma iodide,whichis reutilized to synthesizethyroid
6. Oncocytoma hormoneproduction
7. Parotidhemangioma . hypothyroidism
8. Angiolipoma . identification of MIT + DIT in serum+ urine
(b) primarymalignanttumor followingadministration of l-131
1. Mucoepidermoid carcinoma . "intrinsic" iodinedeficiencygoiter
2. Adenoidcysticcarcinoma(= cylindroma) r/ nignthyroidall-131uptake
3. Malignantmixedtumor r/ rapidintrathyroidal turnoverof l-131
4. Adenocarcinoma 4. Thyroxin-binding globulin(TBG)deficiency
5. Acinuscell carcinoma . abnormalTotransPort
6. Rhabdomyosarcoma . low boundserumToconcentration
(c) metastatictumor . euthyroid
0 Parotidglandundergoeslateencapsulation, 5. End-organresistanceto thyroidhormone
whichleadsto incorporation of lymphnodes! . highserumTo
1. S qu a m o u sc e l lc a rc i n o m a . euthyroid/ hYPothYroid
2. Melanomaof periauricular region . growthretardation
3. Non-Hodgkin lymphoma r/ goiter
4. Thyroidcarcinoma r/ stippledepiphyses
D. LY M P HO P R OL IF E R A T IVE D IS O R D E R
1. Ly m ph o ma/ l e u k e m i a
2. Primarynon-Hodgkin lymphoma(MALToma)
E . CO NG E N IT AL Hyperthyroidism
1. Firstbranchialcleftcyst = eXCess thyroidhormone
. tachycardia, weightloss,muscleweakness,anxiety,
Multiple Lesions of Parotid Gland decreased temperatu re tolerance
1. W ar t hi ntu mo r 1. Gravesdisease= toxicdiffusegoiter(most
2. Metastasesto lymphnodes: squamouscell common)
carcinomaof skin,malignantmelanoma,non- 2. Toxicmultinodular goiter
Hodgkinlymphoma 3. Solitarytoxic adenoma
3. Benignlymphoepithelial cysts(AIDS) 4. lodine-induced hyperthyroidism = Jod-Basedow
5. Thyroiditis
(a) Hashimotothyroiditis= chroniclymphocytic
THYROID thyroiditis
(b) Subacutethyroiditis= de Quervainthyroiditis
CongenitalDyshormonogenesis (c) Painlessthyroiditis
1. Trappingdefect US:
= defectivecellularuptakeof iodineinto thyroid, ^/ decreasein overallechogenicity
salivaryglands,gastricmucosa; r/ discretenodules(50%)
0 Highdosesof inorganiciodinefacilitatediffusion 6. Thyrotoxicosismedicamentosa / factitia
into thyroidpermittinga normalrate of thyroid surreptitiousself-administration of thyroidhormones
hormonesynthesis 7. Strumaovarii
0 Normalratioof iodineconcentrations for gastric = ovarianteratomacontainingthyroidtissue
juice:Plasma= 20:1 8. Hydatidiform mole/ choriocarcinoma / testicular
./ nearlyentiredose of administeredradioiodineis trophoblasticcarcinoma
excretedwithin24 hours = stimulation of thyroidby hCG

9. Pituitary = pituitary
hyperthyroidism neoplasm
. * ?clorTlegaly
' + hyperprolactinemia
10. Thyroidcarcinoma/ hyperfunctioning
metastases
very rare (25 cases)
Radioiodine Therapy for Hyperthyroidism
Dose: (a) empiric: 15-30mOi
(b) calculation(Y): 80-160 pOi/gram
Calculation:
Dose[mCi]= (gtandweight[gram]x y [pCi/gram])
divided by 24-houruptake
Hypothyroidism
A. PRIMARYHYPOTHYROTDTSM
(most
common)
= thyroid'sinabilityto producesufficientthyroid
hormone
1. Agenesisof thyroid
2. Congenitaldyshormonogenesis
3. Chronicthyroiditis
4. Previousradioiodinetherapy
5. Ectopicthyroid(1:4,000)
B. SECONDARYHYPOTHYROIDISM
= failureof anteriorpituitaryto releasesufficient
quantitiesof TSH
1. S heehans y n d ro m e
2. Headtrauma
3. Pituitarytumor(primary/ secondary)
4. Aneurysm
5. Surgery
C. TERTIARYi HYPOTHALAMIC HYPOTHYROIDISM
= failureof hypothalamusto producesufficient
amountsof TRH
Decreased/ No Uptakeof Radiotracer
A. B LO CK E DTR AP PIN GF U N C T ION
1. lodineload (mostcommon)
= dilutionof tracerwithinfloodediodinepool
(fromadministration of radiographiccontrast/
iodine-contai ning medication)
0 Suppressionusuallylastsfor 4 weeks!
2. Exogenousthyroidhormone(replacement
therapy)
suppressesTSH release
B. BLOCKEDORGANIFICATION
1. Antithyroidmedication(propylthiouracil
methimazole) / goitrogenicsubstances
r/ tc-ggm uptake not inhibited
C . DI F F US EP A R EN C H YMAD L ES T R U C T ION
1. Subacute/ chronicthyroiditis
D . HY P O T HY R OID ISM
1. Congenitalhypothyroidism
2. Surgical/ radioiodine ablation
3. Thyroidectopia(strumaovarii,intrathoracic
goiter)
DifferentialDiagnosisof Ear, Nose,and ThroatDisorders 357
mnemonic.' "H MITTE"
Hypothyroidism(congenital)
Medications:PTU,perchlorate, Cytomel,Synthroid,
Lugolsolution
lodineoverload(eg, after IVP)
Thyroidablation(surgery,radioiodine)
Thyroiditis(subacute/ chronic)
Ectopicthyroidhormoneproduction
IncreasedUptakeof Radiotracer
mnemonic.' "THRILLET"
Thyroiditis(earlyHashimoto)
Hyperthyroidism (diffuse/ nodular)
Reboundafterwithdrawalof antithyroidmedication
lodinestarvation
Low serumalbumin
Lithiumtherapy
Enzymedefect
ProminentPyramidalLobe
= distalremnantof thyroiddescenttract
1. Normalvariant: presentin 10o/"
2. Hyperthyroidism
3. Thyroiditis
4. S/P thyroidsurgery
DDx: esophagealactivityfrom salivaryexcretion
(disappearsafterglass of water)
Thyroid Calcif ications
= benigncalcifications = stromalcalcifications
in
adenoma
r/ coarsecalcificationswith roughoutline
r/ alignmentalongperipheryof lesion
r/ irregulardistribution
PsammomaBodies
= microcafcifications
(<1 mm) occur in 54/. of thyroid
neoplasms
r/ seen on xeroradiographyin g4o/o
1. Papillarycarcinoma 61%
2. Follicularcarcinoma 26%
3. Undifferentiatedcarcinoma137o
CysticAreasin Thyroid
15-25% of all thyroidnodules!
A. Anechoicfluid+ smoothregularwall:
1. Colloidaccumulation in goiter= colloidJilled
dilatedmacrofollicle
2. Simplecyst (extremelyuncommon)
(pTU)/ B. Solidparticles+ irregularoutline:
1. H emorrhagicol c l oi dnodul e
2. Hemorrhagicadenoma(30o/")
3. Necroticpapillarycancer(15%)
4. Liquefaction necrosisin adenoma/ goiter
5. Abscess
6. Cysticparathyroidtumor
. bloodyfluid= benign/ malignantlesion
. clearamberfluid= benignlesion
0 Cysticlesionsoftenyieldinsufficient numbersof cellsl
358 Radiology Review Manual

ThyroidNodule DiscordantThyroidNodule
lncidence; (increasingwith age) = nodulehyperfunctioning on Tc-99mpertechnetate
(a) 4-8% by palpation(>2 cm in 2o/",1-2cm in 57o, scan + hypofunctioningon l-131scan,whichindicates
< 1 c m i n 1 % ) ; M : F = 1: 4 reducedorganificationcaPacitY
(b) 50% by autopsy/ thyroidUS if clinicallynormal: Cause:
multiplein 38%, solitaryin 12'/"(occultsmall 1. Malignancy : follicular/ papillarycarcinoma
cancersfound in 4%) 2. Benignlesion: follicularadenoma/ adenomatous
A . T HY RO I DAD EN O MA hyperplasia
1. Adenomatousnodule(a2-77%) (autonomousnontoxicnoduleshave accelerated
2. Follicularadenoma (15-40%) iodineturnoverand dischargeradioiodine as
3. Ectopicparathyroidadenoma hormonewithin24 hours)
B , I NF LA M M AT ION / H EMOR R H AGE
1. lnflammatory lymphnode in subacute+ chronic Hot ThyroidNodule
thyroiditis lncidence: 8h of Tc-99mpertechnetatescans
2. Hemorrhage/ hematoma: frequentlyassociated 1. A denoma
with adenomas (a) Autonomousadenoma= TSH-independent
3. Abscess . euthyroid(80%),thyrotoxicosis(20%)
(8-17%\
c. cARcrNoMA { partial/ total suppressionof remainderof gland
0 Higherriskof malignancyif (b) Adenomatoushyperplasia = TSH-dependent
- patient<20 and >60 years of age secondaryto defectivethyroidhormone
- Hx of radiationtherapyto neck/ upperchest production
- familyHx of thyroidcanceri MEN syndrome 2. Thyroidcarcinoma(extremelyrare)
- new nodulein long-standing goiter ./ discordantuptake
1. Thyroidcarcinoma N.B.: any hot noduleon Tc-99mscan must be imaged
(a) papillarycarcinoma(70%) with l-123 to differentiatebetweenautonomous
(b) follicular(15%) or cancerouslesion
(c) medullarycarcinoma(5-10%)
(d) anaplasticcarcinoma(5%)
(e) thyroidlymphoma(5%) ColdThyroidNodule
2. Nonthyroidal neoplasm A . B E N IGNTU MOR
metastasisfrom breast,lung,kidney,malignant 1. N onfuncti oni ngadenoma
melanoma,Hodgkindisease 2. Cyst (11-20%)
3. Hurthlecell carcinoma nodule
3. lnvolutional
{ very thin hypoechoichalo 4. Parathyroidtumor
4. Carcinomain situ
r/ echogenicarea insidea goiternodule B. INFLAMMATORY MASS
1. Focalthyroiditis
Roleof fine-needle aspirationbiopsy(FNAB): 2. Granuloma
(large-needlebiopsyhas more complicationswith no 3. Abscess
increasein diagnosticyield)
0 FNAB as initialtestleadsto a betterselectionof TU MOR
C . MA LIGN A N T
patientsfor surgerythan any othertest! 1. C arci noma
Diagnosticaccuracyof 70-97"/": 2. Lymphoma
(a) 70-80% negative 3. Metastasis
(b) 10% positivespecimens(3-6% false-positive
rate often due to Hashimotothyroiditis) US featuresof cold nodule:
(c) 10-20% indeterminate r/ hypoechoic(71"h)
Up to 20% nondiagnostic(too few cells)material r/ isoechoic(22/.1
r/ mixedechogenicity$%)
Roleof imaging: r/ hyperechoic(3%)
0 lmagingcannotreliablydistinguishmalignant { cystic(rarelymalignant)
+ benignnodules! 0 A palpablecold nodulein a patientwith Graves
(a) radionuclide scanning diseasehas a highlikelihoodof malignancy$%)l
- usefulin indeterminatecytology
0 Hyperfunctioning noduleis almostalways "CATCH LAMP"
mnemonic:
ben i g n ! Golloidcyst
(b) ultrasound Adenoma(mostcommon)
- best methodto determinevolumeof nodule Thyroiditis
- usefulduringfollow-upto distinguishnodular Carcinoma
growthfrom intranodularhemorrhage Hematoma
 DifferentialDiagnosisof Ear, Nose,and ThroatDisorders 359

Lymphoma,Lymphnode (b) 1-6% for multiplenodules(DDx:multinodular

Abscess goiter)
Metastasis(kidney,breast) (c) with historyof neck irradiationin childhood
Parathyroid - solitarynodulefound in 70./"
(cancerousin 31%)
Probability of a cold nodule to represent thyroid cancer: - multiplenodulesfoundin ZSh
0 Solitarycold nodulesby scintigraphyare (cancerousin 37%)
multinodularby US in Z0-25%l - normalthyroidscan foundin S/"
(a) 15-25% for solitarycold nodule (cancerdetected in 20%\
360 RadiologyReviewManual

OF NECKORGANS
ANATOMYANDFUNCTION

posterior tongue mesopharynx

posterior rim of
aryepiglottic fold
vallecula
lateral wall of
vestibule aryepiglottic fold
laryngeal
surfaceof
false cord
larygeal ventricle epiglottis
tme cord

pyriform sinus -

during phonation during quiet breathing

Frontal Laryngogram During Phonation Lateral LarYngogram

PARANASAL SINUSES Ethmoidal bulla

Mucusproductionof 1 Uday; mucusblanketturns over
every 20-30 minutes;irritantsare propelledtoward
nasopharynxat a rate of 1 cm/minute
Maxillary Sinus
Size: 6-8 cm at birth
Walls: roof= floorof orbit;posteriorwall abuts
pterygopalatine fossa
Extension: 4-5 mm below levelof nasalcavityby age
12
entermiddle
Ostium: maxillaryostium+ infundibulum
meatuswithinposterioraspectof hiatus
additionalostiamay be present
semilunaris;
Plainfilm: presentat birth;visibleat 4-5 months;
completelydevelopedby 15 years of age
V ar iat ions :s i n u sh y p o p l a s i ian 9 h ; a p l a s i ai n 0-4h
Ethmoid Sinuses
Size: adultsize by age 12;3-18 air cellsper side
Walls: roof = floor of anteriorcranialfossa;
lateralwall = laminapapyracea
Plainfilm: very smallat birth;visibleat 1 year of age;
completelydevelopedby PubertY
(a) anteromedial ethmoid air cells
2-8 cells with a total area of 24 x 23 x 11 mm
Ostia: openinginto anterioraspectof hiatus
semilunarisof middlemeatus(anterior
group),openinginto ethmoidbulla (middle
gr o u p ).
Agger nasi cells
= anteriormostethmoidair cells in front of the
attachmentof middleturbinateto cribriformplate
nearthe lacrimalduct
= anterior,lateral+ inferiorto frontoethmoidal
recess= anteromedialmarginof orbit
Prevalence: Presentin >90%
= ethmoidalair cell above+ posteriorto
infundibulum located
+ hiatussemilunaris,
outside the lamina papyraceaat the lateralwall
of the middlemeatus
Haller cells
= anteriorethmoidair cellsinferolateralto
ethmoidalbulla,on lateralwall of infundibulum,
alonginferiormarginof orbit lroof of maxillary
sinus,protrudinginto maxillarysinus
Prevalence: 1A45%
(b) posterior ethmoid air cells
1-8 cells,largercells,totalareasmallerthan that of
anteromedialgrouP
Location:behindthe basal(= ground)lamellaof the
middleturbinate
Ostium: intosuperiormeatus/ suprememeatus,
ultimatelydrainingintosphenoethmoidal
recessof nasalcavitY
Onodi cell
= ITlostposteriorethmoidair cell pneumatizedinto
sphenoidbone t surroundingthe opticcanal
Location: superolateral to sphenoidsinus
FrontalSinus
Size: 28x24 x 20 mm in adults,rapidgrowthuntilthe
late teens
Walls: posteriorwall = anteriorcranialfossa;inferior
wall = anteriorportionof roof of orbit
Ostium: intofrontalrecessof middlemeatusvia
frontoethmoidalrecess(= nasofrontalduct)
Plainfilm: visibleat age 6 Years
Variations:sinusaplasiain up to 4% (in 90% with
Down sYndrome)
 Anatomyand Functionof NeckOrgans 361

SphenoidSinus
Size: 20x 23x 17 mminadults,
smallevagination
of ethmoid
sphenoethmoidal recessat birth,invasionof bulla
hiatus
sphenoidbone beginsat age 5 years;aerated semilunaris
extensionsinto pterygoidplates(44%)+ into
clinoidprocesses(19%) infundibulum uncmate
Walls: roof = floor of selfaturcica;anteriorwall shared process
with ethmoidsinuses;posteriorwall = clivus; maxillary
inferiorwall = roof of nasopharynx ostium middle
meatus
Ostium: 10 mm abovesinusfloorintosphenoethmoidal
recessposteriorto superiormeatusat levelof maxillary middle
sphenopalati ne foramen SlnuS turbinate
Plainfilm: appearsby 3 yearsof age; continuesto
inferior
grow posteriorly+ inferiorlyinto the sella turbinate
untiladulthood

OSTIOMEATAL
UNIT
= ore? of superomedial Coronal Scan of Ostiomeatal Unit
maxillarysinus+ middlemeatus
as the commonmucociliary drainagepathwayof frontal
maxillary,and anterior+ middleethmoidair cellsintothe
nose Ostia:
CoronalCT: visualizedon two or three3-mm-thick (1) multipleostiafrom anteriorethmoidair cells(at
sections its anterioraspect)
Components: (2) maxillaryostiuminfundibulum(at its posterior
1. Infundibulum aspect)
= flattenedconelikepassagebetweeninferomedial
borderof orbit/ ethmoidbulla(laterally) + uncinate Anatomicvariationspredisposingto ostiomeatalnarrowing:
process(medially)+ maxillarysinus(inferiorly) + 1. Conchabullosa(4-150/")= ?erdted/ pneumatized
hiatussemilunaris(superiorly) middleturbinate
2. Uncinateprocess cell = air cell withinverticalportionof
2. Intralamellar
= key bony structurein lateralnasalwall below middleturbinate
hiatussemilunarisin middlemeatusdefineshiatus 3. Oversizedethmoidbulla
semilunaris togetherwith adjacentethmoidbulla 4. Hallercells
r/ pneumatizedin <2.SVoof patients 5. Uncinateprocessbulla
3. E t hm oidbu l l a 6. Bowed nasalseptum
r/ locatedin cephaladrecessof middlemeatus 7. Paradoxicalmiddleturbinate= coflVeXity of turbinate
4. Hiat uss emi l u n a ri s directedtowardlateralnasalwall (10-26%)
finalsegmentfor drainageof maxillarysinus; 8. Deviationof uncinateprocess
locatedjust inferiorto ethmoidbullain middlemeatus 0 These conditionsare not diseasestatesper se!

frontal sinus
superior turbinate
posteriorethmoid ostia
ethmoid bulla +
middle ethmoid sphenoid sinus
ostia
sphenoethmoidalrecess

sphenopalatineforamen

anterior ethmoid
ostia
middle turbinate

inferior turbinate
nasolacrimal duct orifice maxillary ostium

View of Lateral Nasal Wall (turbinatesremoved)

362 Radiology Review Manual

CLEFTDEVELOPMENT
BRANCHIAL pharyngeal branchial arches
pouches (I-V) (1-4)
- 6 pairedbranchialarchesare responsiblefor formation
of lowerface + neck; recognizableby 4th week GA eustachiantube
- each branchialarch containsa centralcore of cartilage branchial clefts
tympanic cavity (Frv)
+ muscle,a bloodvessel,and a nerve mastoid antrum
- 5 ectodermal"clefts"/ grooveson outer aspectof neck
tonsillar fossa
+ 5 endodermalpharyngealpouchesseparatethe 6
archeswith a closingmembranelocatedat the palatine tonsil
interfacebetweenpouchesand clefts inferior cervical sinus
Formation:during4th-Gthweek of embryonic parathyroid III of His
development
pyriform sinus

thyroid
1st BranchialArch = maxillomandibular
arch
(a) largeventral/ mandibularprominence supenor
forms: mandible,incus,malleus,musclesof parathyroid IV
mastication thymus III
(b) smalldorsal/ maxillaryprominence ultimobronchial
thymus IV
forms: maxilla,zygoma,squamousportionof body
temporalbone,cheek,portionsof external
ear BranchialAPParatus
nerve: mandibulardivisionof trigeminalnerve
pouch forms: mastoidair cells + eustachiantube
cleft forms; externalauditorycanal + tympanic cavity
5th + 6th BranchialArches
2nd BranchialArch = hyoidarch cannotbe recognizedexternallY
nerue: recurrentlaryngealbranchof vagus nerve
nerve: facial nerve
arch forms; thyroidgland,stapes,portionsof external
ear, musclesof facialexpression
pouch forms: palatinetonsil+ tonsillarfossa ORALCAVITY
compriseslip, upper+ lowergingiva,buccalmucosa,hard
cleft involutescompletelyby 9th fetal week; 2nd arch
palate,floor of mouth,anterior213of tongue
overgrows2nd + 3rd + 4th cleftsto form ceruicalsinus,
which createsa tract that runs from supraclaviculararea
just lateralto carotidsheath,turns mediallyat OROPHARYNX
mandibularangle betweenexternal+ internalcarotid consistsof
artery,terminatesin tonsillarfossa (a) pharyngealwall betweennasopharynx
+ pharyngoePiglottic fold
3rd BranchialArch (b) soft palate
sinks into retrohyoiddePression (c) tonsillarregion
nerve: glossopharYngeal nerve (d) tonguebase
arch forms; glossoepiglottic fold, superiorconstrictor Borders:
m., internalcarotida., partsof hyoidbone (a) superior:soft palateand Passavantridge (= ridgeof
pouch forms: pharyngealmusclethat opposesthe soft palate
(a) thymusgland,whichdescendsinto mediastinum when soft palateis elevated)
by 9th fetal week (b) anterior:planethat joins the posteriorborderof soft
(b) inferiorparathyroidglandspassingdown with the palate,anteriortonsillarpillars,circumvallatepapillae
thymus (c) posterior:posteriorpharyngealwall
(d) inferior:vallecula
(e) lateral:tonsillarregionconsistingof anteriortonsillar
Ath BranchialArch pillar(= palatoglossusmuscle)+ palatine/ faucial
sunk into retrohyoiddePression tonsil+ posteriortonsillarpillar(= palatopharyngeus
nerve: superiorlaryngeal branchof vagus nerve muscle)
arch forms; epiglottis+ aryepiglotticfolds,thyroid
cartilage,cricothyroidm., left component HYPOPHARYNX
of aorticarch, rightcomponentof right = compdrtmentof aerodigestivetract betweenhyoid bone
proximalsubclaviana. + inferioraspectof cricoidcartilage
pouch forms: superiorparathyroidglands,apex of 1. P yri formsi nuses
piriformfossa = two symmetriclateralstalactitesof air hangingfrom
cleft forms; ultimobranchial body,which provides hypopharynxbehindlarYnx
parafollicular= "C" cellsof thyroid joint
- inferiorwall: levelof cricoarytenoid
 Anatomyand Functionof NeckOrgans g69

- anteromedialwall: lateralwall of aryepiglotticfold 3. Falsevocal cords

- lateralwall: abuts posteriorala of thyroidcartilage = vefltricularfolds= inferiorcontinuation
of
- posteriorwall: most lateralaspectof posterior aryepiglotticfolds = rnucosolsurfaceof
hypopharyngeal wall ventricularligaments;formingsuperiorborderof
2. postcricoid?feo = pharyngoesophageal junction laryngealventricle
extendsfrom levelof arytenoidcartilagesto inferior 4. Arytenoidcartilages
borderof cricoidcartilage 5. Aryepiglotticfolds
- anteriorwall of hypopharynx= posteriorwall = rT]ucos€ll
of reflectionsbetweencephaladportion
lower larynx = "pafty wall" (= arytenoidprocesses)of arytenoidcartilage
3. Posteriorhypopharyngeal wall + inferolateralmarginof epiglottis
extendsfrom levelof valleculaeto cricoarytenoid joints r/ soft-tissuefolds forming border between tateral
pyriformsinuses+ centrallaryngeallumen
LARYNX 6. Laryngealventricle
Verticallength: 44 mm (males),36 mm (females),at 4th_ = fusiformfossa boundedby crescenticedge of
6th cervicalvertebrae false cords superiorly+ straightmarginof true
A. SUPRAGLOTTIS cords inferiorly
extendsfrom tonguebase + valleculaeto laryngeal { generallynot visibleon axialscans
ventricle 7. Preepiglotticspace
1. V es t ibule { low-densitytissuebetweenanteriormarginof
= airspacewithinsupraglotticlarynx epiglottis+ thyroidcartilage
2. Epiglottis 8. Paralaryngealspace
= leaf-shapedcartilagethat functionsas a lid to r/ low-densitytissuebetweentrue + false cords and
endolarynx
, thyroidcartilage
(a) petiole= stem of epiglottis r/ continuouswith preepiglotticspace anteriorly
(b) thyroepiglotticligament= cohfl€ctspetioleto + aryepiglotticfolds superiorly
thyroidcartilageinferiorly
(c) hyoepiglotticligament= corn€cts epiglottisto B. GLOTTIS
hyoidbone anteriorly,coveredby a mucosalfold 1. True vocal cords
betweenthe valleculae(glossoepiglottic fold) = extendfrom vocal processof arytenoidcartilage
(d) "free margin"= superiorportionof epiglottis to anteriorcommissure

facial v.

hamulus
medial pterygoid m.

- masticator space
masseterm.
- parapharyngealspace
inferior alveolar n.

retromandibular v. - pharyngeal mucosal space

external carotid a.
- parotid space
facial n. (VII)
superior constrictor m.
styloid process
internal \',, 'carotid
\
carotid a. space
parotid gland jugular v. prevertebral m. 'prevertebral
\\ space
hypoglossaln. (XID retropharyngeal space
vagus n. (X)
glossopharyngeal n. (IX)

Thansaxial Scan through Level of Lower Nasopharynx

364 Radiology Review Manual

glossoepiglotticfold preepiglottic space

epiglottis
(free margin) thyroid notch strap muscle

submandibular paralaryngeal
gland space ---/_ epiglottis
(petiole)
hyoid bone
aryepiglottic
fold
vallecula=

=_-_-_
_-D
o0
pharyngo-
epiglottic
#-o fold

superior cornu of
thyroid cartilage py,rifonn sinus

Hyoid Bone Level High Supraglottic Level

thyroid notch thvroid notch

parapharyngeal
sternohyoid m. false vocal cord
thyroid lamina SDACC
'
arvtenord
omohyoid &
hyoepiglottic supenor
thyrohoid m. aryepiglottic
ligament process
fold
aryepiglottic pyriform
fold S1NUS
posterior
pharyngeal
wall

pharyngeal wall

Mid Supraglottic Level Low Supraglottic Level

anterior commissure anterior commissure

omohyoid m.
thyroid lamina thyroid m,
thyroid
CCA
true vocal cartilage
cord
IJV
cricoid
cartilage
/=.

arytenoid cricoarytenoid
cartilage joint
cricoid
cartilage H / longus capitis & colli m. sternocleidomastoidm.
nastoidm) - ngeusm.

Glottic Level Undersurfaceof True Cord

"E" I ./ <1 mm softtissuebehindthyroidcartilage(during

r/ vocal cords adductduringphonationof
breathholding
2. Anteriorcommissure
= midlinelaryngealmucosacoveringanterior
portionsof the true vocal cordswhere they abut
the laryngealsurfaceof the thyroidcartilage
abductionof vocal cordswith quiet breathing)
3. Posteriorcommissure
= midlinelaryngealmucosalsurfacebetween
attachmentof true vocal cordsto the arytenoid
cartilages
 Anatomyand Functionof NeckOrgans 365

C. SUBGLOTTIS PrevertebralSpace
extendsfrom undersurfaceof true vocal cords to = ffiajorhighwayfrom skull base to T4; posteriorto
inferiorsurfaceof cricoidcartilage retropharyngeal space
1. Conuselasticus Fascial borders:
= fibroelastic membraneextendingfrom cricoid (a) anteriorcompartmentof deep cervicalfascia:
cartilageto medialmarginof true vocal cords from one transverseprocessto the other anteriorly
+ forminglateralwall of subglottis in frontof longuscollimuscle
(b) posteriorcompartmentof deep cervicalfascia:
from transverseprocessposteriorlyto spinous
DEEPSPACESOF SUPRAHYOID
HEAD& NECK process
Contents:
MasticatorSpace prevertebral muscles(longuscolli)
= lateralto parapharyngeal space scalenemuscles
Fascia: vertebralartery+ vein
superficiallayerof deep cervicalfasciaencloses brachialplexus
musclesof mastication phrenicnerve
Contents:
musclesof mastication(medial+ lateralpterygoid
muscles,masseter,temporalismuscle)
CarotidSpace
ramus+ body of mandible Carotidfasciaextendsfrom skull base to aorticarch
Contents:
cranialnerveV,
(a) belowhyoidbone:
commoncarotidartery
PharyngealMucosalSpace
i nternal j ugul ar
vei n
adenoids,faucial+ lingualtonsils
cranialnerveX (vagusnerve)
superior+ middleconstrictormuscles
cervicalsympatheticplexus
salpingopharyngeal muscle (b) at levelof nasopharynx:
levatorpalatinimuscle
internalcarotidartery
torustubarius
i nternal j ugul ar
vei n
cranialnerveslX-Xll
i nternal j ugul ar
chai nof nodes
Parapharyngeal
Space
= triangular-shaped centrallylocatedspace; major ParotidSpace
verticalhighwayextendingfrom skull base to hyoid
Fascial borders:
Contents:
parotidglandwith Stensonduct
medial = middlelayerof deep cervicalfascia
intraparotidlymph nodes
lateral = suPerficiallayerof deep cervicalfascia
externalcarotid+ internalmaxillaryarteries
posterior = c?rotidsheath
retromandibularvein
Contents:
facialnerve
fat
internalmaxillaryartery
ascendingpharyngealartery
Submandibular
Space
pharyngealvenousplexus Contents:
submandibular glandwith Whartonduct
branchesof cranialnerveV"
facialartery+ vein
Vectors: if parapharyngealfat"iseffaced
cranialnerveXll
anteriorly = lesionin masticatorspace
medially = lesionin pharyngealmucosalspace
laterally = lesionin parotidspace TEMPORAL
BONE
posteriorly = lesionin carotidspace A. SQUAMOUSPORTION
= lateralwall of middlecranialfossa+ floorof temporal
fossa
Retropharyngeal
Space
= potentialspace posteriorto pharyngealmucosal
B. MASTOIDPORTION
space + anteriorto prevertebralspace;majorvertical 1. Mastoidantrum
highwayfrom skull base to T4 2. Aditusad antrum
Fascial borders: connectsepitympanum(= attic)of middleear cavity
mid + deep layersof cervicalfascia; to mastoidantrum
alar fascialaterally 3. Kdrnerseptum
Contents: = Srndllbony projectionextendinginferiorlyfrom
fat roof of mastoidantrumas part of petrosquamosal
medial+ lateralretropharyngeal nodes suturebetweenlateral+ medialmastoidair cells
366 Radiology Review Manual

supenor
semicircular canal
horizontal tensor tympanl m.

2nd turn of cochlea

lst turn of
cochlea

portion of facial nerve

facial nerve /
F
recess /
/ vestibularaqueduct
/
posterior genu
'hypotympanum
of facial nerve posterior semicircular canal

smus tympanl

pyramidal eminence

F-osrl

Coronal Tomogram of Temporal Bone Axial Tomogram of TemPoral Bone

Most Anterior Scan through Cochlea Scan at Vestibular Level Most Posterior Scan
through Round Window
Coronal Scan of Normal Right Ear

Most Superior Scan Scan through Vestibular Level Most Inferior Scan
through Lateral Semicircular Canal through Basilar T[rrn of Cochlea
Axiat Scan of Normal Right Ear
 Anatomy and Functionof Neck Organs 567

C. PETROUSPORTION= innerear IN N E R E A R
1. Tegmentympani 1. Cochlea
= foof of tympaniccavity
2112 turns,basalfirstturn opensinto roundwindow
2. Arcuateeminence posteriorly,encirclescentralbony axis of modiolus
= prominenceof bone over superiorsemicircular
2. Vestibule
canal = largestpart of membranouslabyrinthwith subunitsof
3. Internalauditorycanal(lAC) utricle+ saccule(notseparatelyvisualized);
(a) Porusacusticusinternus
separatedfrom middleear by oval window
= openingof internalauditorycanal
3. Semicircular canals
(b) Modiolus - superiorsemicircular canalformsconvexityof
= entranceto cochlea
arcuateeminence
(c) Cristafalciformis - posteriorsemicircular canalpointsposteriorly along
= horizontalbony septumin IAC
lineof petrousridge
4. Vestibularaqueduct - lateral/ horizontalsemicircular
= transmitsendolymphaticduct canaljutsinto
epitympanum
5. Cochlearaqueduct 4. Cochlearaqueduct
= transmitsperilymphaticduct
contains8-mm-longperilymphatic duct,extendsfrom
6. Petrousapex basalturn of cochleato lateralborderof jugular
= sepdf€ltedfrom clivusby petro-occipital
fissure foramenparallelingIAC
+ foramenlacerum Function: regulatesCSF + perilymphatic fluid
D. TYMPANICPORTION s Vestiburar"f,j::::["
1. Externalauditorycanal(EAC) encompassesendolymphaticduct, extendsfrom
medialborderformedby tympanicmembrane, vestibuleto endolymphaticsac
which attachessuperiorlyat scutum+ inferiorlyat Function: equilibration
of endolymphatic
fluid pressure
t y m panica n n u l u s
PAROTID
GLAND
E. STYLOIDPORTION
Embryology:
glandularcomponentarisesfrom ingrowthof local
proliferationof oral epithelium,which createsducts by
MIDDLEEAR 1Othweek GA; secretionsbeginby 18thweek GA
Borders: 0 Epithelialbuds brancharounddivisionsof facialnerve
- anteriorwall = carotidwall
- thus incorporatingit into parotidparenchyma
posteriorwall = m?stoidwall including 0 The only salivaryglandto becomeencapsulatedafter
(a) facial nerverecessfor descendingfacialnerve development of lymphaticsystemresultingin
(b) pyramidaleminencefor stapediusmuscle intraglandularlymph nodes and lymphvessefs
(c) sinustympani(ctinicallybtindspot) Location: wrapsaroundmandibularangle(within
- superiorwall = tegmentympani parotidspace)
- inferiorwall = jugularwall Anatomic divisions:
- lateralwall = tympanicmembrane (a) superficiallobe = fi't?inbulk of glandsuperficial
and
- medialwall = labyrinthine wall posteriorto massetermuscle;
separatedby facialnervefrom:
A. E P I T Y M P A N U M (b) deep lobe= smallextensionof glanddeepto angle
= tympaniccavityabovethe line drawn betweenthe
of mandible
inferiortip of scutum+ tympanicportionof facial (c) accessorylobe (20%)= superficialand lateralto
nerve massetermuscle+ anteriorto superficiallobe
Contents: malleushead, body + short processof drainingdirectlyintoparotidduct
ncus, Prussakspace (= of€a between Drainageroute: Stensonduct exitingabove upper2nd
ncus + lateralwall of epitympanum) molartooth
B. MESOTYMPANUM
= tympaniccavitybetweeninferiortip of scutum+ line
THYROID
GLAND
drawn parallelto inferioraspectof bony EAC
CT vafues: 70-120 HU
Contents: manubriumof malleus,long processof
incus,stapes,tensortympanimuscle
(innervatedby Vr), stapediusmuscle THYROID HORMONES
(innervated by Vll) Free hormone : To (0.03%)
T3 p.4%)
C. HYPOTYMPANUM Thyroxin-binding
globulin(TBG) : binds To
= shallowtroughin floorof middleear Q0%)
and T, (38%)
368 Radiology Review Manual

prealbumin(TBPA): bindsTo
Thyroxin-binding (10%) Location: superiordorsalsurfaceof thyroidgland/
and T, (27%) intrathyroidal
Albumin : bindsTo e0%)
and T. (35%) B . IN FE R IORP A R A TH Y R OID
GLA N D S
Embryology: derivedfrom 3rd pharyngealpouches
A. ELEVATIONOF TBG migratingcaudallywith thymus
1. P r egnan c y Location: anywherenear lin thyroid,carotid
2. Estrogenadministration bifurcation.lowerneck,mediastinum
3. Genetictrait
C . S U P E R N U ME R A RPYA R A TH Y R OID
GLA NDS
B . RE DUCT I O N IN T BG sth / 6th gland may occupyan ectopicsite
1. A ndr oge n s 0 Up to 12 parathyroids may be present!
2. Anabolicsteroids
3. Glucocorticoids Embryology; parathyroidglands develop by 6 weeks
4. Nephroticsyndrome GA + migrateinto neck at 8 weeks
5. Chronichepaticdisease S i z e :6 x 4 x 1 m m = 2 5 - 4 0 m 9

O F T , B IN D IN GT O T BG: s a l i c yl ates
C. I NHI B I T I O N glands:
Surgicalsuccessratesfor findingparathyroid
- 95% for initialcervicalexploration
PARATHYROID GLANDS - 60% for repeatsurgicalexploration
A . S UP E RI O RPA R A T H Y R OID
GL A N D S Causefor failure: overlookingan adenoma,multiple
Embryology: derivedfrom 4th pharyngealpouches, abnormalglands,diffusehyperplasia
descendingtogetherwith thyroid technique:
Localization
glandin closerelationship
to its US (75%sensitivity), subtraction
thallium-technetium
posterolaterallobes MR (88%sensitivity)
scintigraphy,

Duplexldentification

Criteria External Carotid Artery lnternal Carotid Artery

Size usuallysmallerthan ICA usuallylargerthanECA

Location orientedmedially+ anteriorlytowardface orientedlaterally+ posteriorly

towardmastoid
process(mnemonici IACvis-a-visECAis
positionedlikehelixvis-a-vistragusof yourear)
Branches givesoff arterialbranches(superiorthyroidala.
as 1st branch) NO arterialbranches

Waveform high-resistanceflowpatternsupplyingcapillary
bedsin skin+ muscre
i forwardsystoliccomponent
{ earlydiastolicflowrsversaloccasionally
lollowedby anothsrfoMardcomponent
J little/ no flowin latsdiastole
Maneuvsr oscillationsontemporaltapmaneuver
low-resistance flowpatternsupplyingcapillary
bed in brain
{ high-velocity forwardsystoliccomponsnt
{ sustainedstrongfoMardflowin diastole
{ stagnanteddywithflowreversaloppositeto
flowdividerin carotidbulb

EAR,NOSE,ANDTHROATDISORDERS
ADENOIDCYSTICCARCINOMA
= CYLINDROMA
lncidence: 4-15/" of all salivaryglandtumors
Histo: (a) cribriformsubtype,grade 1
(b) tubularsubtype,grade2
(c) solid/ basaloidsubtype,grade 3
0 Perineuralinvasionis typical!
Age: 3rd-9th decade;maximumbetween40 and
T 0 y e a r sM
; =F
Location:
@ Minorsalivaryglands (mostcommon;21-31r"hof
malignantneoplasmsin minorsalivaryglands)
0 Mostcommontumorof the minorsalivaryglandsl
. nasalobstruction + swelling
Location: oral cavity> pharynx> nose >
paranasalsinuses> trachea> larynx
@ Submandibular gland(15%of tumorsin this gland)
@ Parotidgland (2-G%of tumorsin this gland;arises
from peripheralparotidductswith propensityfor
perineuralspreadalongfacialnerve)
. hard mass + facialnerve pain/paralysis
{ infiltratingparotidmass
MR:
ri frypo-to hyperintense(highsignalcorrespondsto low
cellularity with a betterprognosis)on T2Wl
Metastases to: lung,cervicallymphnodes,bone,liver,
brain
Prognosis: slow growingbut relentlessmalignantcourse
with repeatrecurrences;the greaterthe
cellularity,the worse the prognosis(requires
entiretumor);60-69% S-yearsurvivalrate;
40% 1O-yearsurvival rate
Bx: repeatsurgicalexcision+ radiationtherapy
LaryngealAdenoidCysticCarcinoma
0.25-1Y"of all malignantlaryngealtumors
Histo: uniformsmallbasaloidcellswith largedeeply
stainingovoidnucleiarrangedin anastomosing
cords or islands
. coughingattacks,wheezing,hemoptysis
. paralysisof recurrentlaryngealnervedue to
propensityto invadenerves(CHARACTERISTIC)
. absenthistoryof cigarettesmoking
Location: subglottisat junctionwith trachea(g0%)
r/ extensivesubmucosaliumor spreadof entirelarynx
r/ invasionof cricoidcartilage,thyroid+ esophagus
i/ regionalneck nodes hard-lyever involved
ANG I O LI P O M A O F P AR O T ID GL A N D
= benignnodularlesionsimilarto ordinarylipomasexcept
for associatedangiomatousproliferation
Age: rare beforepuberty
CT:
r/ circumscribed (morecommon)/ infiltrating
mass
r/ markedenhancementaroundfatty compdnents
DDx: hemangiomawith fatty degeneration
Ear, Nose,and ThroatDisorders gig
APICAL PETROSITIS
= P E TR OUASP IC ITIS
chronic> acuteapicitis
Etiology: spreadfrom middleear + mastoidinfection;
requirespresenceof air cellsin petrousapices
(whichis found in B}.h of population)
Organism; Pseudomonas,Enterococcus
. Gradenigo syndrofit€ = otorrhea(otitismedia)+ retro-
orbitalpain (trigeminalpain) + 6th nervepalsy
! air cell opacification(fluidin ipsilateral
middleear
+ mastoid)
^/ bone destruction(osteomyelitis)
MR:
r/ enhancingmassaboutpetroustip
Cx: epiduralabscess;
cranialnervepalsy(abducens,trigeminal, vagus)
Mortality: up to 20"/. (priorto antibioticera)
Rx: intravenousantibiotics,myringotomy,surgery
B R A N C H IA L C LE FT A N OMA LIE S
= failureof involutionof branchialcleftsleadsto branchial
cleft cysts/ fistula/ sinustracts
First Branchial Cleft Cyst (5-8%)
= PAROTID LYMPHOEPITHELIAL CYST
Residualembryonictractbeginsnearsubmandibular
triangle+ ascendsthroughthe parotidgland,terminates
at junctionof cartilaginous+ bony externalauditorycanal
lncidence: 5-8/" of all branchialcleft anomalies(rare)
Age: middle-aged women
. enlargingmass near lowerpoleof parotidgland
. recurrentparotidabscesses
. + facialnervepalsy
. otorrhea(if cyst drainsinto EAC)
Pathologic classification (Work):
Type I duplicationanomalyof membranousEAC;
derivedfrom ectoderm+ linedwith
squamousepithelium; courseparallelto
EAC;medialto conchaof ear; no skin
appendages
Type ll cyst arisesfrom 1st branchialcleft
containingectodermand mesoderm
involvingEAC + pinna;skin appendages
(hairfollicles,sweatand sebaceousglands)
r/ cysticmass withingland or immediateperiparolid
region(superficial to / deep to parotidgland)
^i may extendinto adjacentfat-containing
parapharyngealspace+ connectionto EAC
DDx: inflammatoryparotidcyst, benigncysticparotid
tumor, necroticmetastaticlymphadenopathy
Second Branchial Gleft Cyst (95%)
= incompleteobliteration of 2nd branchialclefttract
(cervicalsinus of His) resultingin sinus tract/fistula i
cyst (75%)
lncidence: 95Y"of all branchialcleft anomalies
Age: 10-40 years; M = F
370 Radiology Review Manual
Cl assification (BaiIey):
Type I alonganteriorsurfaceof sternocleido-
mastoidmuscle,just deepto platysma
Type ll alonganteriorsurfaceof sternocleido-
mastoidmuscle,lateralto carotidspace,
posteriorto submandibular glandadhering
to the greatvessels(mostcommon)
Type lll extensionmediallybetweenbifurcation of
externaland internalcarotidarteriesto
lateralpharyngealwall
Type lV withinpharyngealmucosalspace
Path: 1-1O-cmlargethin-walledcyst,linedby stratified
squamousepitheliumoverlyinglymphoidtissue,
filledwith turbidyellowishfluid+ cholesterol
crystals
. historyof multipleparotidabscessesunresponsiveto
drainage+ antibiotics
. otorrhea(if connectedto externalauditorycanal)
Location:
anywherealonga linefrom the oropharyngeal
tonsillarfossato supraclavicular regionof neck;
classicallyat anteromedialborderof
sternocleidomastoid muscle+ lateralto carotidspace
+ at posteriormarginof submandibular gland;may be
in parapharyngealspace (afterextensionthrough
stylomandibular tunnel+ middleconstrictormuscle)
r/ oval/ roundcyst near mandibularangle
r/ displacement of sternocleidomastoid muscle
posteriorly,carotidartery+ jugularvein
posteromedially, submandibular glandanteriorly
i/ cyst may enlargeafter upper respiratorytract infection
/ injury
US:
r/ compressiblemass + internaldebris(due to
hemorrhage/ infection)obscuringits cysticnature
r/ lacf of internalflow
CT / M R:
./ "beaksign"= curvedrim of tissuepointingmedially
betweeninternal+ externalcarotidarteries
(PATHOGNOMONTC)
r/ slightenhancement of capsule
DDx: necroticneuraltumor,cervicalabscess,
submandibular glandcyst,cysticlymphangioma,
necroticmetastatic/ inflammatory
lymphadenopathy
Third BranchialFistula/ Cyst
= above superiorlaryngealnerve
lncidence; extremelyrare
lnternalopening: piriformsinus anteriorto fold formed
by internallaryngealnerve
Course: piercesthyrohyoidmembrane,runs over
hypoglossalnerve+ underglossopharyngeal
nerve,betweeninternal+ externalcarotid
arteries,caudolateral/ posterolateralto
proximalinternal+ commoncarotidarteries
Externalopening: at base of neck anteriorto
sternocleidomastoid muscle
{ unilocularcysticmass withinposteriorcervicalspace
FourthBranchialFistula
= belowsuperiorlaryngealnerve
Incidence: extremelyrare (R > L)
lnternalopening: apex of piriformsinus
Course: betweencricoid+ thyroidcartilage,below
cricothyroidmuscle,caudalcoursebetween
trachea+ carotidvessels,deep to clavicleinto
mediastinum,loopingforwardbelow aorta (left
side)/ rightsubclavianartery(rightside),
ascendingalongventralsurfaceof common
carotidartery,passingover hypoglossalnerve
Externalopening: at base of neck anteriorto
sternocleidomastoid muscle
to subclavianartery
+ anteroinferior
. recurrentepisodesof "suppurative thyroiditis"'/neck
abscesses
Site: 90% on leftside
CAROTIDARTERYANEURYSM
= €rr'r€urysm
of extracranialcarotidartery
Etiology:
1. Trauma
2. Infection(mycoticaneurysm)
3. Congenital(very rare): manifestationof connective
tissuedisorder(Ehlers-Danlos, Marfan,Kawasaki,
Mafuccisyndrome)
CAROTIDARTERYSTENOSIS
High-gradeICA stenosisis associatedwith increasedrisk
for TlA, stroke,carotidocclusion,embolismarisingfrom
thrombiformingat site of narrowing
lncreasedrisk for stroke:
(a) significantICA stenosis(compromised bloodflow)
Reductionof bloodflow occursat 50-60% diameter
stenosis175%area stenosis
0 2/" riskof strokewith nonsignificant stenosis
O 16% incidenceof strokewith significant stenosis
0 2% incidenceof subsequentstrokefollowing
endarterectomy
(b) intraplaque hemorrhage(embolicstroke)
Histo:
arteriosclerosis = genericterm for all structuralchanges
resultingin hardeningof the arterialwall
1. Diffuseintimalthickening
= growthof intimathroughmigrationof medial
smoothmusclecellsintosubendothelial space
throughfenestrations in internalelasticlamella
associatedwith increasingamountsof collagen,
elasticf ibers,glycosaminoglycans
Age: beginningat birthslowlyprogressing to adult
life
2. Atherosclerosis
= intimalpool of necrotic,proteinaceous+ fatty
substanceswithinhardenedarterialwall
Location: large+ medium-sized elasticand
musculararteries
 Ear, Nose,and ThroatDisorders 371

(a) fattystreak= superficialyellow-grayflat intimal

lesioncharacterizedby focal accumulationot
subendothelial smoothmusclecells+ lipid
deposits
(b) fibrousplaque= whitishprotrudinglesion
consistingof centralcore of lipid+ cell debris
surroundedby smoothmusclecells,collagen,
elasticfibers,proteoglycans; a fibrouscap
separatesthe lipidcore (= atheroma)from the
vessellumen
(c) complicatedlesion= fibrousplaquewith
degenerativechangessuch as calcification,
plaquehemorrhage, intimalulceration/ rupture,
muralthrombosis
Plaquehemorrhagefrom thin-walledbloodvessels
in vascularized plaquemay causeulceration,
thrombosis+ embolism,and luminalnarrowing ECSTmethod NASCETmethod
NASCET methoc
0 In 93% of symptomaticpatients
0 ln 27"/.of asymptomaticpatients % CarotidStenosis= (y-X) / y. 100
Plaque ulceration exposesthrombogenic ECST= EuropeanCarotidSurgeryTrial; NASCET= North
subendothelial collagen+ lipid-richmaterial AmericanSymptomaticCarotidEndarterectomy
Trial
0 Frequentin plaquesoccupying>g5% of lumen
0 12.5%strokeincidenceper year
3. Monckebergsclerosis= medialcalcification r/ Holmancarotidslim sign = diffusenarrowingof
4. Hypertensivearteriosclerosis
entire ICA distalto high-gradestenosisdue to
decreasein perfusionpressure
Predilection sites of arterial stenosr's;
occlusionof ICA
lncidenceof lesions @ Intracranial
Stenosis Occlusion carotidsiphonstenosis
RightICA origin retrogradeflow in ophthalmicarteryfilledfrom ECA
33.8% 8 .6%
Left ICA origin { smallvesselocclusion
34.1% 8 .7%
Rightvertebralarteryorigin focal areas of slow flow
18.4% 4 .8%
Left vertebralarteryorigin { earlydrainingvein = reactivehyperemia- "luxury
22.3% 2.2% perfusion"due to shuntingbetweenarterioles
Rightcarotidsiphon 6 .7 % 9 .0%
Left carotidsiphon + venulessurrounding an areaof ischemia
6 .6 % 9.2%
Basilarartery ICA-MCAsfowflow = delayed arrival+ washoutof
7 .7 % 0 . 8%
RightMCA ICA-MCAdistribution in comparisonto ECA
3.5% 2.2o/o
Left MCA 4.1% 2.1% Carotid endarterectomy:
Benefit: 17"/"reductionof ipsilateralstrokeat 2 years
Decreasein LuminalDiametervs. Cross-sectional
Area in patientswith >70% carotidstenosis
(NASCET= NorthAmericanSymptomatic
Decrease in Decrease in CarotidEndarterectomy Trial)
Lumen Diameter Cross-sectionalArea Risk: 1% mortality;2/"riskof intraoperative
20% 36% neurologicdeficit
40% 64%
60"/" 84% CarotidDuplexUltrasound
80"/" 96% lndications for carotid duplex US:
(1) Screeningfor suspectedextracranial carotid
Temporalcourse of carotid artery stenosis: disease
1. Stablestenosis(68%) (a) high-gradeflow-limitingstenosis
2. Progressivestenosisto >50%diameterreduction (b) low-gradestenosiswith hemorrhage
(25%) (2) Nonhemispheric neurologicsymptomatology
(3) Historyof transientischemicattack/ stroke
Angiography: (4) Asymptomaticcarotidbruit
@ Extracranial (5) Retinalcholesterol embolus
r/ smoothasymmetricalexcrescenceencroaching (6) Preoperativeevaluationbeforemajor
uponvessellumen cardiovascular surgery
! crater/ niche = ulceration (7) lntraoperativemonitoringof vascularpatency
! moundwithinbase of crater= ffiur?lthrombus duringendarterectomy
372 Radiology Review Manual

(8) Sequentialevaluationafter endarterectomy

- 300 peak systolic (9) Monitoringof knownplaqueduringmedical
U) velocity
treatment
peak diastolic
E zoo
, velocity -'
b |
-E roo Y -" ' Grading of Carotid Stenosis
= severityof stenosisis primarilygradedas a ratioof
lumendiameternarrowingNOT reductionin cross
10 20 30 40 50 60 t0 80 90 100 sectionalarea
Angiographicdiameterstenosis[7o]
Limitations:
1. Calcifications >1 cm in length
0 A jet associatedwith a >70"hstenosisusually
diastolic velocity ratio ---__* travelsat least 1 cm downstream!
i : t 0 high-gradestenosis
2. Contralateral
€>.
(.) = ipsilateralICA functionsas collateralwith
systolic velocity ratio
increasedbloodflow velocities
# ) 0 Use velocityratiosto compensatefor this
effect!
10 20 30 40 50 60 70 80 90 100
Accuracy of duplex scans: (in comparisonto
Angiographicdiameterstenosis[7o]
arteriographyfor ICA lesions)
Doppler Parametersin 91-94% sensitivity,85-99% specificityfor
Internal Carotid Artery Stenosis >50/" ICA diameterstenosis

DopplerSpectrumAnalYsis
DiameterStenosis ICNCCA Peak SYstolic VelocitY Peak Diastolic Velocity
Classification (%) Peak Systotic Ratio Peak Diastolic Ratio (cm/sec) kHzt (cm/sec) kHzt

Normal-mild 0-40 <1.5 < 2.6 <110 <3.5 <40 <1.5

>25
Moderate 41-59 <1.8 < 2.6 > 120 > 3.5 <40 <1.5
Severe 60-79 > 1 . 8 > 2.6 >130 >5.0 >40 >1.5
Critical 80-99 > 3 .7 > 5.5 >250 >8.0 >80-135 >4.5
t = basedon 5-MHzpulsedDopplercarrierfrequencyat 60'flow angle(Blackshear)

0-39 <1.8 < 2.4 < 11 0 <40

40-59 <1.8 < 2.4 <130 <40
60-79 >1.8 > 2.4 >130 >40
80-99 > 3 .7 > 5.5 >250 > 100
( B lut h)

0-50 <2"1 <125 <40

50-75 >2:1 125-225 40-1 00
75-90 >3:1 >5:1 225-325 > 100
>90 > 4 :1 > 9:1 >325 >100
>95 resistiveCCA distortion maybe decreased may be decreased
(Gosink)

1-15
16-49 (< 125) < 4.0
50-79 (> 125) > 4.0 (< 140) <4. 5
(Fell) 80-ee (>140) >4.5
(Strandness) occlusion no flow detected
of Washington)
t = based on 5MHz pulsed Dopplercarrierfrequencyat 60" flow angle(University
 Ear, Nose,and ThroatDisorders 373

Incorporating B-modeand Dopplerspectrumanalysis r/ increaseddiastolicflow in ECA (if ECA assumes

A. NO LESION the roleof primarysupplierof bloodto brain)
r/ peak systolicvelocity(PSV)< 1ZScm/sec r/ increasein peak systolicvelocitiesin
r/ clear windowundersystole contralateralICA (due to collateralflow)
^/ no spectralbroadening Limitations:
r/ no evidenceof plaque poorvisualization due to calcification,
tortuosity,
B . M I NI M A LD ISE AS E increaseddepth of artery,"high"bifurcation
= 0-15% diameterreduction
r/ PSv < 12s cm/sec
r/ clearwindowundersystole Common Carotid Waveform Analysis
r/ minimalspectralbroadeningin deceleration A. DISTALOBSTRUCTION
phase of systole r/ nign-pulsatility
waveform(pulsatilitychangesoccur
{ minimalplaque onlywith >80%stenosis)
C. M O DE RA T ED ISE AS E r/ reducedamplitude
= 16-49/" diameterreduction B. PROXTMAL OBSTRUCTION
{ peak systole<125 cm/sec ^/ low-amplitudedampedwaveform
r/ no windowundersystole
r/ poststenoticspectralbroadeningthroughout Hemodynamic Variations of Carotid Artery Stenosis
systole A. MORPHOLOGYOF STENOSIS
0 End-diastolic velocity(EDV)remainsnormalin 1. Degreeof stenosis:velocitiesincreaseup to a
<50/" d iameter reduction ! l umi naldi ameterof 1.0-1.5mm
r/ moderateplaque 2. Lengthof stenosis: peak velocitiesdecreasewith
D. S E V E RED ISE AS E= H E M O D YN AMIC A LLY lengthof stenosis
S I G NI F I C ANLTE SION ./ use the same angle+ steeringdirectionwhen
(a) 50-59% stenosis followinga patientfor diseaseprogression
'V psv 120-130 cm/s
B . P H Y S IOLOGIC V A R IA B ILITY
r/ rov 3o-40cm/s 0 A rangeof velocitiesmay be encounteredwith a
(b) 60-79% stenosis givendegreeof stenosis!
^/ pSv of 131-250cm/s 0 ICA/CCAratioobviateseffectsof physiologic
^/ eov of 4o-1oo cm/s variability!
(c) >60% stenosis 0 Compareleft with rightwaveformsto avoid errors!
r/ end diastolicvelocityof >80 cm/sec 0 Measurevolumeflow (moresensitivebecauseof
(d) 50-79% diameterreduction contralateralcompensatoryflow increase)
r/ peak velocityratioof ICA/CCA>1.5 Cause:
j nt"X systole>125 cm/s 1. Cardiacoutput
r/ markedpoststenoticspectralbroadening 2. Pulserate
throughoutcardiaccycle 3. Flow velocity: increasedwith obstructionin
(e) >7oo/o stenosis (benefitof endarterectomy collateralvessels,decreasedwith proximal
documentedin NASCETstudy) obstructionin same vessel
r/ peak systole>230 cm/s 4. Normalhelicalnatureof bloodflow with many
r/ end diastole>100cm/s differentvelocityvectors+ nonaxialbloodflow
r/ peak velocityratioof ICA/CCA>4.0 not detectableby color Dupleximaging
r/ peak systolicvelocityICA * end diastolic 5. Peripheralresistance
velocityCCA >15 6. Arterialcompliance
(f) 80-99% diameterreduction 7. Hypertension
{ pSv of >250 cm/s 8. Bloodviscosity
{ r ov o f > 1 0 0c m i s
r/ no windowundersystole
^/ poststenoticspectralbroadeningthroughout CarotidPlaque
systole FORMATIONTHEORY
r/ "stringsign"on colorDopplerwith slow-flow 1. Stagnanteddy that rotatesat outervessel margin
sensitivitysetting (oppositeto the flow dividerin areaof flow
E . O CCLUD EDVE SS EL separation+ low shear stress)leadsto net influx
r/ no signalin ICA on longitudinal / transverse of fluidintosubendothelial tissuewith progressive
images(colorsensitivity+ velocityscale must depositionof lipids+ smoothmusclecell
be set low enoughto clearlydiscernflow signals proliferation
withininternaljugularvein) 2. Increasedlikelihoodof intraplaque hemorrhage
r/ absenceof diastolicflow in CCA (high (vascularizationof plaquewith fragilevessels
impedanceflow) derivedfrom vasa vasorumI from lumen)
r/ diastolicflow reversalin CCA + fissuringfrom a criticalsize on
374 Radiology Review Manual
0 As the degreeof stenosisincreases,it is more
likelythat plaquesbecomedenser+ more
heterogeneous demonstratingan irregular
surface!
PLAQUEDENSITY
1. Hypoechoic= low-echogenicity plaque
= fibrofattyplaque/ hemorrhage
{ echogenicity lessthan sternocleidomastoid
muscle
r/ flow void / flow disturbanceon color Duplex
2. lsoechoicplaque
= sffioothmusclecell proliferation/ laminar
thrombus
{ echogenicityequalto sternocleidomastoid
muscle+ lowerthan adventitia
3. Hyperechoic = moderatelyechogenicplaque
= fibrousplaque
{ echogenicity higherthan sternocleidomastoid
muscle+ similarto adventitia
4. Calcification = stronglyechogenicplaque
r/ acousticshadowimpairsvisualizationof intima
ErrorsIn DuplexUltrasound
PLAQUETEXTURE
1. Homogeneous plaque= stableplaque
Histo: depositionof fatty streaks+ fibrous
tissue;rarelyshowsintraPlaque
hemorrhage/ ulcerations
Prognosis:
0 Neurologicdeficitsdevelopin 4oh
0 lpsilateralinfarctionon CT in 12'/.
0 lpsilateralsymptomsdevelopin 22'h
0 Progressive stenosisdevelopsin 18%
r/ homogeneousuniformecho patternwith smooth
surface(acousticimpedancesimilarto blood)
2. Heterogeneous plaque
= unstableplaque= mixtureof high,medium,and
low-levelechoeswith smooth/ irregularsurface;
may fissure/ tear resultingin intraplaque
hemorrhage/ ulceration+ thrombusformation
(embolus/ increasingstenosis)
B-modeultrasoundhas 90-947osensitivity,
7 5-88% specificity,90/" accuracyfor
intraplaquehemorrhage
Histo: lipid-ladenmacrophages,monocytes,
leukocytes,necroticdebris,cholesterol
crystals,calcifications
Prognosis:
0 Neurologicdeficitsdevelopin 27"h
0 lpsilateral infarctionon CT in 24h
0 lpsilateral symptomsdevelopin 50%
0 Progressivestenosisdevelopsin 77'/"
r/ anechoicareaswithinplaque(= hemorrhage/
lipiddeposition/ focal plaquedegeneration)
^/ heterogeneouscomplexecho pattern
PLAQUE SURFACECHARACTERISTICS
= US unreliabledue to poorvisualization
of intima
Categories: - smooth
- mildlyirregular
- markedlyirregular
- ulcerated
1. l nti malthi ckeni ng
Histo: fatty streaks
vesselwall
{ wavy / irregularlineparalleling
extending>1 mm intovessellumen
2. Ulceratedplaque
Accuracy: 60% sensitive,60-70% specific
0 The presenceof intraplaque hemorrhageis
much morecommonthan normallyappreciated
0 Neitherarteriographynor US has proved
reliable!
r/ isolatedcraterol >2 mm withinsurfaceof
plaquedemonstratedon transverse
+ longitudinal images
^/ reversedflow vorticesextendinginto plaque
craterdemonstratedby color Doppler
./ proximal+ distalundercutting of plaque
r/ anechoicareawithinplaqueextendingto
surface
1. Errorin properlocalization of stenosis(6%)
Cause: ECA stenosisplacedinto ICA / carotid
bifurcationor vice versa
2. MistakingpatentECA branchesfor carotid
bifurcation(4%)
Cause: completeocclusionof ICA not recognized
r/ disparityin positionof bifurcation
r/ no differencein pulsatilitywaveform
./ high-resistance waveformin CCA
3. Interpretererror in estimatingseverityof stenosis
(2.5%)
usuallyoverestimation, rarelyunderestimation
r/ absenceof one / morecomponentsfor diagnosis
which are
(a) significantelevationof peak velocity
(b) poststenoticturbulence
(c) extensionof high velocityinto diastole
4. Superimposition of ECA + ICA (2%)
Cause: strictcoronalorientationof ECA + ICA
r/ superimposition can be avoidedby rotationof
head to oppositeside
5. Severestenosismistakenfor occlusion
minimalflow not detectable;angiogramnecessary
with delayedimages
6. Weak signalsmisinterpreted as occlusion
7. Normal/ weak signalsin severestenosis
Cause: severestenosiscausesa decreasein
blood flow + peak velocitywith returnto
normalvelocitYlevels
r/ nign resistivityin CCA
8. Pointof maximumfrequencyshiftnot identified
Cause: extremelysmall lumen/ short segmentof
stenosis
r/ unexplained(poststenotic) coarseturbulence
{ ipsilateralECA collateralflow
^/ abnormalCCA resistivity

9. Stenosisobscuredby plaque/ strong Dopplershift
in overlyingvessel
10. Inaccessible stenosis
r/ abnormalCCA resistivity
r/ abnormaloculoplethysmography
11. Unreliable velocitymeasurements
(a) highervelocities:hypertension, severe
bradycardia,obstructivecontralateralcarotid
disease
(b) lowervelocities:arrhythmia, aorticvalvular
lesion, severecardiomyopathy, proximal
obstructive carotidlesion("tandemlesion"),
>95o/"ICA stenosis
(c) aliasing= highvelocitiesare displayedin
reverseddirectionbelowzero baselinedue to
Dopplerfrequencyexceedinghalfthe pulse
repetitionfrequency
Remedy: shift zero baseline,increasepulse
repetitionfrequency,increaseDoppler
angle,decreasetransducer frequency,
use continuous-wave Dopplerprobe
IN DI RE CTM E T H OD SO F E VA L U A T IO N
1. Oculoplethysmography (OpG)
= rn€osufement of ophthalmicarterypressure
+ pulse arrivaltime by air calibratedsystem
Contraindications: glaucoma,retinaldetachment,
recenteye surgery/ trauma,lens
implants
2. Periorbital bidirectional Doppler
= insonationof frontal+ supraorbitalarteriesto
assessflow directionaroundorbit and to detect
crossoverflowthroughthe circleof Willis(through
contra-and ipsilateral compression)
3. Transcraniaf Doppler
= insonationto establishflow directionin basal
cerebralarteriesthroughtemporalbone (MCA,
ACA, PCA,terminalportionof ICA),foramen
magnum(bothvertebralarteries,basilarartery),
orbit (carotidsiphon)
0 Nondiagnostic in up to 3S%!
CERVICAL
DERMOID
/ EPIDERMOID
CYST
Location: floorof mouth
(1) Cysticteratoma
teratoma= neoplasmwhosetissueis foreignto the
part of the body from which the tumor arises
(a) epidermoidcyst = epidermalinclusioncyst
= linedby simplesquamousepitheliumwithoutskin
appendages
(b) dermoidcyst
= epithelial-lined
cyst containinghairfollicles
+ sebaceous+ sweat glands
(c) teratoidcyst
= linedwith squamous/ respiratoryepithelium
containingderivativesof ectoderm+ endoderm
+ mesoderm(skinappendages,nervous/ Gl /
respiratorytissue)
Ear, Nose,and ThroatDisorders 375
(2) Nonteratomatoussequestration-typeepithelial-lined
cyst
Location:
- dorsumof nose in infants(mostcommon)
- midlineanteriorfloorof mouth:
(a) sublingualbetweenmylohyoidmuscle
+ tongue(DDx: inclusioncyst,ranula)
(b) submentalbetweenplatysma+ mylohyoid
muscle
CHOANAL ATRESIA
0 Mostcommoncauseof neonatalnasalobstruction!
Frequency; 1:5,000to 1:8,000neonates;M < F
Etiology: failure of perforationof oronasalmembrane,
which normallyperforatesby 7th week EGA
0 Associatedwith otheranomaliesin 50-75%:
acrophalyngosyndactyly, amnioticband syndrome,
malrotation of bowel,Crouzonsyndrome,fetalalcohol
syndrome,DiGeorgesyndrome,Treacher-Collins
syndrome,chromosome18 I 12 anomalies,polydactyly,
coloboma,facialcleft,CHD,TE fistula,craniosynostosis
Location: bilateral: unilateralatresia= 3:2
. respiratorydistressin bilateralchoanalatresia(relieved
by cryingin neonateswho are obligatenose breathers
duringfirst2-6 months)
. nasalstuffiness,rhinorrhea, infectionin unilateral
choanalatresia
Types:
A. OSSEOUS/ BONYSEPTATION(85-90%)
Cause: incompletecanalizationof choanae
B . ME MB R A N OU S E P TA TTON (10-15% )
Cause: incompleteresorptionof epithelialplugs
C . OS S E OME MB R A N OU S
CT (precededby vigoroussuctioning+ administration of
topicaldecongestant) :
r/ narrowingof posteriorchoanaeto a width of <3.4 mm
(in children<2 yearsof age)
r/ inwardbowingof posteriormaxilla
^i fusion/ thickeningof vomer
^/ bone / soft-tissueseptumextendingacrossthe
posteriorchoanae
Dx: nasalcathetercannotbe advancedto beyond32 mm
Cx: bilateralchoanalatresiais life-threatening
Rx: endoscopicperforation,choanalreconstruction
CHOLESTEATOMA
= KERATOMA
= epithelium-lined
sac filledwith keratindebrisleadingto
bone destructionby pressure+ demineralizingenzymes
Primary Cholesteatoma(2%)
= CONGENITAL CHOLESTEATOMA = EPIDERMOID CYST
= derivedfrom aberrantembryonicectodermalrests in
temporalbone (commonlypetrousapex)/ epidural
spacei meninges
. conductivehearinglossin childwith NO historyof
middleear inflammatorydisease
. cholesteatoma seenthroughintacttympanic
membrane
Associatedwith: EAC dysplasia
376 Radiology Review Manual

Location: Cx: (1) Intratemporal: ossiculardestruction, facial

(a) epitympanum nerveparalysis(1%),labyrinthine fistula,
(b) petrouspyramid:internalauditorycanalfirst automastoidectomy, completehearingloss
involved (2) Intracrani almeni
: ngi ti s,
si gmoi dsinus
(c) meninges: scoopedout appearanceof petrous thrombosis,temporallobeabscess,CSF
ridge rhi norrhea
(d) cerebellopontineangle: erosionof porus, DDx: chronicotitismedia,granulation tissue
shorteningof posteriorcanalwall = cholesterolgranuloma,brainherniation
(e) jugularfossa: erosionof posteroinferior
aspectof throughtegmendefect,neoplasm
petrouspyramid (rhabdomyosarcoma, squamouscell
carcinoma)

(98%)
SecondaryCholesteatoma CHOLESTEROL
GRANULOMA
= INFLAMMATORY CHOLESTEATOMA = ACQUIRED = CHOLESTEROLCYST
EPIDERMOID = €rcguired inflammatory lesionof petrousbone
Cause: Histo: cholesterolcrystalssurroundedby foreign-body
ingrowthof squamouscell epitheliumof EACthrough giantcells;embeddedin fibrousconnective
tympanicmembrane(= e?rdrum)secondaryto tissuewith varyingproportions of hemosiderin-
(a) repeatedepisodesof ear inflammationwith ladenmacrophages, chronicinflammatory
cells
invaginationof posterosuperior retractionpocket and bloodvessels;brownishfluidcontains
(b) marginalperforationof eardrum cholesterolcrystals+ blood (= "chocolatecyst")
Age: usually>40 years . blue (vascular)tympanicmembranewithoutpulsatile
. whitishpearlymass behindintacttympanic ti nni tus
membrane(invasionof middleear cavityand r/ ossiclesremainintact
mastoid)diagnosedotoscopicallyin 95% CT:
. facialparalysis(compressionof nerveVll at { nonenhancing middleear mass
geniculateganglion) MRI:
. conductivehearingloss (compromise of nerveVlll in r/ hyperintense signalon Tl Wl + T2Wl secondaryto
internalauditorycanal/ involvement of cochleaor methemoglobin (DDxto cholesteatoma, whichis
labyrinth) i soi ntenseto brai non Tl W l )
. severevertigo(labyrinthine fistula)
Types: C H R ON IC R E C U R R E N T S IA LA D E N ITIS
1. Pars flaccida cholesteatoma = Primaryacquired . painfulperiodicunilateralenlargement of parotidgland
cholesteatoma= Attic cholesteatoma(most . milkydischargemay be expressed
common) Sialography:
./ increasingwidth of attic ^/ Stensenduct irregularly enlarged/ sausage-shaped
./ initiattydestructionof lateralwall of attic, { pruningof distalparotidducts
particularlythe drum spur (scutum)with invasion r/ + calculi
of Prussakspace CT:
r/ extensionposteriorlythroughaditusad antrum r/ diffusetyenlargeddensegland
intomastoidantrum r/ ditatedStensenduct+ calculi
r/ destructionof Kornerseptum Cx: Mucocele
2. Pars tensa cholesteatoma = Secondaryacquired
cholesteatoma (lessfrequent) COGAN SYNDROME
r/ displacementof auditoryossicles = AUTOIMMUNE INTERSTITIAL KERATITIS
r/ erosionof ossicularchain: firstaffectinglong MR:
processof incus r/ membranouslabyrinthine
enhancement
nondependent homogeneousmass
perforationof tympanic membrane posterosuperiorly CROUP
(parsflaccida= Shrapnellmembrane) = ACUTELARYNGOTRACHEOBRONCHITIS = ACUTE
,Vpoorlypneumatizedmastoid(frequentassociation) VIRALSPASMODIC LARYNG ITIS
./ erosionof tegmentympani(withmoreextensive = lower respiratorytract infection
cholesteatoma)producingan extraduralmass Organism; parainfluenza,respiratorysyncytial virus
./ destructionof labyrinthine capsule(lesscommon) Age: >6 monthsof age, peak incidence2-3 years
involvingthe lateralsemicircular canalfirst . historyof viral lower respiratoryinfection
{ erosionof facialcanal . hoarsecry + "brassy"cough
MRI: . inspiratorydifficultywith stridor
^/ iso-/ hypointenserelativeto cortexon Tl Wl . fever
r/ no enhancement with Gd-DTPA(enhancement is r/ thickeningof vocal cords
relatedto granulationtissue) r/ l\ORtrlnl epiglottis+ aryepiglotticfolds
 Ear, Nose,and ThroatDisorders 377

r/ "steeplesign" = subglottic"invertedV" = symmetrical MR:

funnel-shaped narrowing1-1.Scm belowlowermargins r/ hypointense / hyperintense(sebaceousfluid)/
of pyriformsinuseson AP radiograph(lossof normal isointenserelativeto muscleon Tl Wl
"shouldering"
of air columncausedby mucosaledema r/ hyperintense on T2Wl + internallyheterogeneous
+ externalrestrictionby cricoid),accentuatedon Prognosis; malignantdegenerationinto squamouscell
expiration,paradoxicalinspiratorycollapse,less carcinomain 5./"
pronouncedduringexpiration DDx: ranula
r/ narrow+ indistinctsubglottictracheaon lateral
, radiograph
ri inspiratoryballooningof hypopharynx (nonspecific
sign DISSECTION OF CERVICOCEPHALIC ARTERIES
of any acute upperairwayobstruction) = hematomawithinmediasplittingoff the vesselwall and
r/ distensionof cervicaltracheaon expiration causinga falselumenwithinmedia
Prognosis; usuallyself-limiting lncidencei responsiblefor 5-20% of strokesin young
and middle-aged adult
Location: cervicalICA (60%),vertebralartery(20%),
both ICA + vertebralartery(10%);multiple
DACRYOCYSTOCELE simultaneous dissections(33%)
0 2nd mostcommoncauseof neonatalnasalobstruction Site: (a) subintimaldissection= closeto intima
(afterchoanalatresia) (b) subadventitialdissection= closeto adventitia
Cause: obstructionof nasolacrimalduct (imperforate r/ arterialnarrowing/ occlusion
Hasnermembranedistally,reasonfor proximal ri intimalflap
obstructionunknown) ./ pseudoaneurysm
., tenseblue-graymassat medialcanthus/ in nasalcavity r/ embolicdistalbranchocclusionof intracranial artery
r/ nasolacrimal duct dilatation US (50%accuracy):
^/ homogeneous well-defined massof fluidattenuation ^/ echogenicintimalflap
r/ enhancement of thin wall ./ echogenicthrombus
r/ superiordisplacement of inferiorturbinatebone ^/ dampened/ high-resistance Dopplerwaveform
r/ contralateralshift of nasalseptum C E C T:
Cx: dacryocystitis(postnatalinfectionwith adjacent r/ narrowing/ occlusionof contrast{illedartery
soft-tissueswelling+ enhancement), periorbital MR:
c ellulit is { periarterial rim of hyperintensesignalon Tl Wl
Rx: duct massage,duct probing,prophylactic antibiotics + iso-to hyperintense on T2Wl aroundflow void of
arterY(= intramuralhematoma)
./ pseudoenlargement of externaldiameterof artery
DERMOID
CYSTOF NECK Rx: early anticoagulanttherapy(to preventstroke)
0 7% of all dermoidinclusioncystsoccurin the headand
neck CarotidArtery Dissection
Age: 2nd-3rd decades;M = F 0 Twice as commonas vertebralarterydissection!
Path: circumscribedencapsulatedlesions,coveredby Etiology:
squamousepithelium,lumenfilledwith cheesy A . S P ON TA N E OU C
S A R OTIDD IS S E C TI O N
keratinaceous+ sebaceousmaterial (1) nonrecalledminor/ trivialtrauma(frequent)
Histo: epithelial-linedcyst containinghairfollicles (2) primaryarterialdisease(rare): Marfan
+ sebaceousglands+ sweatglands syndrome.fibromuscular dysplasia(in 15%),
. slowlygrowingsoft mobilemass in the suprahyoid cysticmedialnecrosis,collagenvascular
midline(no movementwith tongueprotrusion!) disease,homocysti nuria
Location:lateraleye brow > floorof mouth(11%) Associatedwith: hypertension(36%),smoking
Site: (a) sublingualspace(superiorto mylohyoidmuscle) (47"/"),migraine (11%)
= intraoralsurgicalapproach(morefrequent) B. TRAUMATICCAROTIDDISSECTION(rare)
(b) submandibular (inferiorto mylohyoidmuscle) blunt/ penetratingtrauma(automobile accident,
= externalsurgicalapproach boxing,accidentalhanging,diagnosticcarotid
S iz e: f ew m m - u p to 1 2 c m compression, manipulative therapy)
./ thin-walledunilocularmass Associatedwith: fracturethroughcarotidcanal
CT: lncidence: 2-5-20% of strokes in personsaged
./ homogeneous fluidmaterialof O-18 HU 40-60 years
r/ heterogeneousmass (due to variousgerminal Age: 18-76 years (66% between35 and 50 years)
components) . unremitting unilateralanteriorheadache(86%),neck
{ fluid-fluidlevel(dueto supernatant lipid) pain (25%)
{ "sack-of-marbles" appearance(= coalescenceof fat) . TIA/stroke (58%),amaurosisfugax(12%)
is PATHOGNOMONIC . oculosympathetic paresis= Hornersyndrome(52%)
{ rim enhancement frequent . bruit (48%)
378 Radiology Review Manual

Location: cervicalICA usuallyat levelof C1-2 (60%) Organism; Haemophilusinfluenzaetype B,

withina few cm of carotidbifurcation> Pneumococcus,Streptococcusgroup A
supraclinoid segmentof ICA;bilateral Age: >3 years,peak incidence6 years
carotiddissections(15%) . abruptonset of respiratorydistresswith inspiratory
Length: a few centimeters stridor
Angiography: . severedysphagia
{ stringsign = elongatedtaperedirregularluminal Location: purelysupraglottic lesion;associated
stenosisextendingto base of skull (76%) subglotticedema in 25'/"
r/ abruptluminalreconstitution at levelof bony carotid Lateralradiographshouldbe takenin erectpositiononly!
canal (42%) (frontalview irrelevant)
r/ fingerlike/ saccularaneurysm(40%),oftenin upper r/ enlargementof epiglottis+ thickeningof aryepiglottic
cervical/ subcranialregion folds
^/ intimat ftap (29%) { circumferential narrowingof subglotticportionof
r/ double-barrel lumensimilarto aorticdissection tracheaduringinspiration
(rare) ri ballooningof hypopharynx+ pyriformsinuses
{ slow ICA-MCAflow { cervicalkyphosis
^/ tapered"flamelike" / "radishtaillike"occlusion Cx: Mortaldangerof suffocationsecondaryto hazardof
(17"/"),oftendistalto carotidbulb completeainruayclosure;patientneedsto be
Cx: (1) Thromboemboli due to stenosis accompanied by physicianexperiencedin
(2) Subarachnoid hemorrhage(withintracranial endotracheal intubation
location)
(3) Secondaryaneurysm
Prognosis; complete/ excellentrecovery(8%) with
normalization in a few months;worseningin EXTERNAL AUDITORY CANAL DYSPLASIA
10% lncidence; 1:10,000births;familyhistoryin 14/"
Rx: best therapynot clear;anticoagulation (primary Etiology: (a) isolated(b) Trisomy13, 18, 21 (c) Turner
treatment),surgery,endovascular stentplacement syndrome(d) Maternalrubella(e) Craniofacial
dysostosis(f) Mandibulofacialdysostosis
S P E C TR U M
VertebralArtery Dissection 1. Stenosisof EAC
= hemorrhageinto wall of vertebralartery 2. Fibrousatresiaof EAC
Prevalence: unknown;up to 15/" of strokesin young 3. Bonyatresia(in positionof tympanicmembrane)
adults 4. Decreasedpneumatization of mastoid(mastoidcells
Etiology: beginto form in 7th fetalmonth)
(a) traumaticstretchingof arteryover lateralmass of 5. Decreasedsize labsence of tympaniccavity
C2 during rotationof head (chiropractic 6. Ossicularchanges(rotation,fusion,absence)
manipulation, bowling,tennis,archery) 7. Ectopicfacialnerve= anteriorlydisplacedvertical
(b) spontaneous (mastoid)portionof facial nervecanal
Predisposed;fibromusculardysplasia,Marfan 8. Decreasein numberof cochlearturns/ absenceof
syndrome,collagenvascular cochlea
disease,homocystinuria 9. Dilatationof lateralsemicircularcanal
. headache: occipital(>50%),frontal(20'/"),orbital . bilateralin29/"; M:F = 6:4
(20%) . pinnadeformity
. neck pain (30%) . stenotic/ absentauditorycanal
Location:at levelof C112(65%);bilateralvertebral Cx: congenitalcholesteatoma(infrequent)
arterydissections(5%);site of directtrauma
MR (modalityof choice):
r/ decreasedarteriallumen
r/ diminishedflow void EDULLARYPLASMACYTOMA
EXTRAM
r/ periarterialrim signalintensitychangeswith time Uncommonform;relativelybenigncourse(dissemination
( hem ogl o b i n ) may be foundmonths/ yearslateror not at all);
A ngio: questionable if precursorto multiplemyeloma
! taperingof artery/ intimaltlap lcompleteocclusion Age : 35-40 years; M:F = 2:1
Cx: stroke(in up to 95%) after hours/ weeks Location: air passages(50%)predominantly in upper
Prognosis; full recoverywith some residualdeficit(88%) nose and oral cavity;larynx;conjunctiva
(37%);lymphnodes(3%)
EPIGLOTTITIS . usuallynot associatedwith increasedimmunoglobulin
= ACUTEBACTERIAL EPIGLOTTITIS titeror amyloiddeposition
= life-threateninginfectionwith edemaof epiglottis { mass of one to severalcm in size with well-defined
+ aryepiglotticfolds lobulatedborder
 Ear, Nose,and ThroatDisorders 379

Classification: GOITER
1. Medullaryplasmacytoma AdenomatousGoiter
2. Multiplemyeloma: = MULTINODULAR GOITER
(a) scatteredinvolvementof bone US: (89%sensitivity, 73/" positive
84/" specificity,
(b) myelomatosis of bone predictivevalue,94/o negativepredictivevalue)
3. Extramedullary plasmacytoma r/ increasedsize + asymmetryof gland
DDx: { muftiple1-4-cmsolidnodules
( 1) M ULT T P LMY
E EL OMA { areasof hemorrhage+ necrosis
= malignantcoursewith soft-tissueinvolvement in { coarsecalcificationsmay occurwithinadenoma
50-73/": (secondaryto hemorrhage+ necrosis)
(a) microscopicinfiltration Cx: compressionof trachea
(b) enlargement of organs
(c) formationof tumormass (1/3)
. usuallyassociatedwith proteinabnormalities
DiffuseGoiter
. may have amyloiddeposition US:
{ increasein glandularsize,R lobe > L lobe
Age incidence: 50-85 years r/ trtOfocaltexturalchanges
0 Tendsto occur late in the courseof the disease
r/ calcificationsnot associatedwith nodules
and indicatesa poor prognosis(0-6% S-year
survival)
lodi ne-def iciency Goiter
Not a significantproblemin UnitedStatesbecauseof
FIBROMATOSIS COLLI supplemental iodinein food
= rdre form of infantilefibromatosisthat occurssolelyin Etiology: chronicTSH stimulation
sternocleidomastoid muscle . low serumT,
Cause: in >90% associatedwith birthtraumaduring ./ rrignl-131uitake
difficultdelivery/ forcepsdelivery
Pathology: compartmentsyndromewith pressurenecrosis Jod-Basedow Phenomenon (2%)
+ secondaryfibrosisof sternocleidomastoidm. = developmentof thyrotoxicosis(= €xc€Ssive
Age: 2nd to 4th weeks of life; M > F amountsof To synthesized+ released)if normal
. historyof difficultdelivery(forceps) dietaryintakeis resumed/ iodinatedcontrast
. firm soft-tissuemass in lower 1/3 of sternocleidomastoid mediumadministered
muscle,which may grow over2-4 additionalweeks lncidence: most commonin individualswith long-
. torticollis(14-20%)due to musclecontraction standingmultinodulargoiter
Location: lower 1/3 of sternocleidomastoid muscle Age: >50 years
affectingsternal+ clavicularheadsof the { muftinodular goiterwith in- / decreaseduptake
muscle;usuallyunilateral(R > L) (dependingon iodinepool)
r/ focal/ diffuseenlargement of sternocleidomastoid
m.
US: Toxic NodularGoiter
^/ homogeneously enlargedsternocleidomastoidm. = P LU MMED RIS E A S E
without atocal lesion = autonomousfunctionof one / more thyroidadenomas
{ well-/ ill-defined masswithinsternocleidomastoid m.: Peak age: 4-5th decade; M:F = 1:3
r/ nypo-to iso- to hyperechoicmass dependingon . elevatedTo
durationof disorder . suppressedTSH
MR: r/ nodularthyroidwitirhot nodule+ suppression of
^/ diffuseabnormalhighsignalintensity(greaterthan remainderof gland
that of fat) within muscleon T2Wl r/ stimulation scanwill disclosenormaluptakein
CT: remainderof gland
r/ isoattenuating homogeneous muscleenlargement ^/ increasedradioiodineuptakeby 24 hoursof
Prognosis; gradualspontaneousregressionby age 2 (in approximately80%
66%) with / withouttreatment Rx:
Rx: (1) musclestretchingexercise(2) surgeryin 10% (1) l-131treatmentwith empiricaldose of 25-29 mOi
DDx: (hypothyroidism in 5-30%)
(1) Neuroblastoma (heterogeneous solidmasswith (2) Surgery(hypothyroidism in 11%)
calcifications) (3) Percutaneousethanolinjection(hypothyroidismin
(2) Rhabdomyosarcoma <1o/o,
transientdamageof recurrentlaryngeal
(3) Lymphoma(well-definedround/oval massesalong nerve in 4%)
cervicallymphnodechain)
(4) Cystichygroma(anechoicregionwith septations) Intrathoracic
Goiter
(5) Branchialcleftcyst = extensionof cervicalthyroidtissue/ ectopicthyroid
(6) Hematoma tissue(rare)into mediastinum
380 Radiology Review Manual

lncidence: 5/" of resectedmediastinalmasses;most { cervicallymphadenopathy + enlargedadenoids

commoncauseof mediastinal masses; typicallyassociated
2/" of all goiters Prognosis; parotidinvolvementis associatedwith a better
. mostlyasymptomatic prognosisin H|V-positive
children!
. symptomsof tracheal+ esophageal+ recurrent
laryngealnervecompression HYPOPHARYNG EAL CARCINOMA
Location: Histo: squamouscell carcinoma
(a) retrosternal(80%)= in front of trachea May be associatedwith: Plummer-Vinson syndrome
(b) posteriordescending(20%)= behindtracheabut (= atrophicmucosa,achlorhydria, sideropenic anemia)
in frontof esophagus,caudalextentlimitedby affectingwomenin 90%
arch of azygosvein,exclusively on rightside of . sorethroat,intolerance to hot i cold liquids(earlysigns)
trachea . dysphagia,weightloss (latesigns)
r/ continuitywith cervicalthyroid llackof continuity(with . cervicaladenopathy(in 50% at presentation)
narrowfibrous/ vascularpedicle) Stage:
{ frequentfocal calcifications T1 tumorlimitedto one subsite
CT: T2 tumorinvolves>1 subsite/ adjacentsitewithout
r/ massof high HU + well-defined margins fixationof hemilarynx
./ inhomogeneoustexturewith low-densityareas T3 same as T2 with fixationof hemilarynx
(= degenerativecysticareas) T4 invasionof thyroidi cricoidcartilage/ soft tissue
! marked+ prolongedenhancement of neck

PyriformSinusCarcinoma
GRAVES DISEASE lncidence: 60/" of hypopharyngeal carcinomas
= DIFFUSE TOXICGOITER . may escapeclinicaldetectionif locatedat inferiortip;
= ?utoimmunedisorderwith thyroid-stimulating antibodies often originof "cervicaladenopathywith unknown
(LATS)producinghyperplasia+ hypertrophyof thyroid primary"(nextto primariesin lingual+ faucialtonsils
gland and nasopharynx)
Peak age: 3rd-4th decade; M:F = 1:7 r/ invasionof posteriorala of thyroidcartilage,
. elevatedT" + To
cricothyroidspace,soft tissueof neck in T4 lesion
. depressedTSH'production
Prognosis: poor due to early soft-tissueinvasion
. dermopothy= pretibialmyxedema(5%)
. ophthalmopathy = periorbital
edema,lid retraction, PostcricoidCarcinoma
ophthalmoplegia, proptosis,malignantexophthalmos lncidence: 25/" of hypopharyngeal carcinomas
{ diffusethyroidenlargement { difficultassessmentdue to varyingthicknessof
{ uniformlyincreaseduptake inferiorconstrictor+ prevertebralmuscles
r/ incidentalnodulessuperimposed on preexisting Prognosis: 25/" S-yearsurvival(worstprognosis)
adenomatousgoiter(5%)
US, (identicalto diffusegoiter)
PosteriorPharyngeal
WallCarcinoma
{ globalenlargement of 2-3 x the normalsize
Incidence: 15/" of hypopharyngealcarcinomas
r/ normal/ diffuselyhypoechoicpattern r/ invasionof retropharyngeal
spacewith extensioninto
r/ hyperemiaon color Doppler
oro- and nasopharynx
Rx: l-131treatments(foradults): r/ retropharyngeal adenopathy
Dose: 80-120 pCi/gof glandwith 100%uptake
(takinginto accountestimatedweightof
gland + measuredradioactiveiodine IN V E R TE D P A P ILLOMA
= INVERTING PAPILLOMA = ENDOPHYTIC PAPILLOMA
uptakefor 24 hours) = SQUAMOUS CELLPAPILLOMA = TRANSITIONAL CELL
Cx: 10-30% develophypothyroidism within PAPILLOMA = CYLINDRICAL EPITHELIOMA
1st year + 3/"lyear rate thereafter = SCHNEIDERIAN PAPILLOMA
lncidence: 4/" of all nasal neoplasms;most commonof
epithelialpapillomas;commonlyoccurring
HIV PAROTITIS after nasalsurgery
Histo: benignlymphoepithelial lesionconsistingof an Cause: unknown;association with human
intranodalcyst linedwith epithelialcells papillomavirus-1 1
US: Age: 40-60 years; M:F = 3-5:1
r/ multiplehypoechoic / anechoicareaswithout Path: vascularmasswith prominentmucouscyst
posterioracousticenhancement(70%) inclusionsinterspersed throughoutepithelium
r/ anechoiccysts (30%) Histo: hyperplastic epitheliuminvertsintounderlying
C T / M R: stromaratherthan in an exophyticdirection;high
r/ bilateralparotidgland enlargementwith intraglandular intracellularg lycogencontent
cystic+ solid masses 0 Squamouscell carcinomacoexistentin 5.5-27%l

Location: uniquelyunilateral(bilateralin <5%)
(a) most often arisingfrom the lateralnasalwall with
extensionintoethmoid/ maxillarysinuses,at
junctionof antrum+ ethmoidsinuses
(b) paranasalsinus(mostfrequentlymaxillaryantrum)
(c) nasalseptum(5.5-18%)
. unilateralnasalobstruction, epistaxis,postnasaldrip,
recurrentsinusitis,sinus headache
. distinctive absenceof allergichistory
r/ commonlyinvolvesantrum+ ethmoidsinus
{ wideningof infundibulum / outflowtractof antrum
r/ destructionof mediatantratwall/lamina papyraceaof
orbit,anteriorcranialfossa(pressurenecrosis)in up to
30%
ri septummay be bowedto oppositeside (NO invasion)
r/ homogeneous enhancement
MR:
r/ may have intermediateto low intensityon T2Wl
(DDx:squamouscell carcinoma,olfactory
neuroblastoma, melanoma,smallcell carcinoma)
Cx: (1) cellularatypia/ squamouscell carcinoma(10%)
(2) recurrencerate of 15-78./"
Rx: completesurgicalextirpation(lateralrhinotomywith
en blocexcisionof lateralnasalwall)
JUVENILEANGIOFIBROMA
= rTrost commonbenignnasopharyngeal tumor,can grow
to enormoussize and locallyinvadevitalstructures
lncidence: 0.5/" of all head and neck neoplasms
Age: teenagers(meanage of 15 years);almost
exclusivelyin males
. recurrent+ severeepistaxis(59%)
. r'r€rsdlspeechdue to nasalobstruction(91%)
. facialdeformity(lesscommon)
Location: nasopharynx/ posteriornares
Extension: posterolateral wall of nasalcavity;via
pterygopalatine fossa into retroantralregion/
orbit/ middlecranialfossa; laterallyinto
infratemporalfossa
r/ wideningof pterygopalatine fossa (90%)with anterior
bowingof posteriorantralwall
r/ invasionof sphenoidsinus(213)fromtumorerosion
throughfloorof sinus
r/ wideningof inferior+ superiororbitalfissures(spread
intoorbitvia inferiororbitalfissure+ into middlecranial
fossavia superiororbitalfissure)
r/ frigntyvascularnasopharyngeal mass (onlyenhances
on CT scan immediately afterbolusinjection);supplied
primarilyby internalmaxillaryartery
MR:
r/ intermediatesignalintensityon TlWl with discrete
punctateareas of hypointensity(secondaryto highly
vascularstroma)
NOTE: Biopsycontraindicated!
LABYRINTHITIS
Cause: viral infection(mumps,measles)> bacterial
infection> syphilis,autoimmune,
toxins
. suddenhearingloss,vertigo,tinnitus
Ear, Nose,and ThroatDisorders 381
MR: r/ faintdiffuseenhancement
of labyrinthon TlWl
(HALLMARK)
Ramsay-Huntsyndronte = herpeszosteroticus
. mucosalvesiclesof externalauditorycanal
^/ intracanalicular
8th nerveenhancement
Tympanogen
ic Labyrinthitis
Cause: agententersthroughoval lround windowin
middleear infection
Meningogenic
Labyrinthitis
Cause: agent propagatesalong IAC / cochlear
aqueductin meningitis
Location: oftenbilateral
Labyri nth itis Ossif icans
= LABYRINTHITIS OBLITERANS = SCLEROSING
LABYRINTHITIS = CALCIFIC/ OSSIFYING COCHLEITIS
Cause: suppurativeinfection(tympanogenic,
meningogenic, hematogenic) in 90%,trauma,
surgery,tumor,severeotosclerosis
Pathophysiology: progressivefibrosis+ ossificationof
granulationtissuewithinlabyrinth
. bi - / unilateralprofounddeafness
ri lossof normalfluidsignalwithinlabyrinthon T2Wl
(earlyin courseof disease)
ri innerear structuresfilledwith bone
LARYNGEAL CARCINOMA
98% of all malignantlaryngealtumors;in 2/o sarcomas
Riskfactors: smoking,alcoholabuse,airborneirritants
Histo: squamouscell carcinoma
Suggestiveof lymph node metastasis:
r/ lymph node >1.5 cm in cross section
r/ proximityto laryngealmass
r/ clusterof >3 lymphnodes6-15 mm in size
SupraglotticCarcinoma
lncidence: 20-30% of all laryngealcancers
Metastases: earlyto lymph nodesof deep cervical
chain, in 25-55% at time of presentation
. symptomaticlate in courseof disease(oftenT3 lT4)
Stage:
T1 tumorconfinedto site of origin
T2 involvementof adjacentsupraglotticsite /
glottiswithoutcord fixation
T3 tumorlimitedto larynxwith cord fixationor
extensionto postcricoidarea / medialwall of
pyriformsinus/ preepiglotticspace
T4 extensionbeyondlarynxwith involvement of
oropharynx(baseof tongue)/ soft tissueof
neck / thyroidcartilage
A . A N TE R IORC OMP A R TME N T
1. Epiglotticcarcinoma
{ circumferential relativelysymmetricgrowth
r/ extensioninto preepiglotticspace+ base of
tongue+ paraglotticspace
Prognosis; betterthan for tumorsof
posterolateral compartment
382 Radiology Review Manual

B. POSTEROLATERAL COMPARTMENT Rx: resection(local

function-preservinglaryngeal
1. Aryepiglottic fold (marginal supraglottic) recurrencemay be seen 10 years or more)
carcinoma DDx: benignchondroma
{ exophyticgroMh from medialsurfaceof
aryepiglotticfold HEMANGIOMA
LARYNGEAL
rl growthinto fixed portionof epiglottis Histo: cavernous/ capillarytype
+ paraglottic(= paralaryngeal)space . dark bluishred i palered compressibleswellingon
2. False vocal cord / laryngeal ventricle endoscopy
carcinoma { strongcontrastenhancement
r/ submucosalspreadinto paraglotticspace CT:
r/ + destructionof thyroidcartilage { phleboliths(PATHOGNOMONIC for cavernoustype)
r/ + involvementof true vocal cords MR:
Prognosis.' poorerthan for cancer of the anterior { very high signalintensityon T2Wl
compartment DDx: paraganglioma,hypervascularmetastasis(renal
adenocarcinoma)
GlotticCarcinoma
lncidence; 50-60"h of all laryngealcancers (10%)
InfantileLaryngealHemangioma
. early detectiondue to hoarseness = SUBGLOTTIC
HEMANGIOMA
Stage: 0 Most commonsubglotticsoft-tissuemass causing
T1 tumor confinedto vocal cord with normalmobility upper respiratorytract obstructionin neonates
T2 supra-/ subglotticextension+ impairedmobility A ge: < 6 months;M:F = 1:2
T3 fixationof true vocal cord . crouplikesymptoms(dyspnea,stridor)in neonatal
T4 destructionof thyroidcartilage/ extensionoutside period
larynx . hemangiomas elsewhere(skin,mucosalmembranes)
Patterns of tumor invasion: in 50%
(1) anteriorextensionintoanteriorcommissure Location: subglotticregion
{ >1 mm thicknessof anteriorcommissure { eccentricthickeningof subglotticportionof trachea
./ invasionof contralateralvocal cord via anterior (AP view)
commissure { arisesfrom posteriorwall belowtrue cords (lateral
(2) posteriorextensionto arytenoidcartilage,posterior view)
commissure,cricoarytenoid joint Rx: tracheostomy(waitingfor spontaneous
(3) subglotticextension regression)
{ tumor >5 mm inferiorto levelof vocal cords
(4) deep lateralextensioninto paralaryngealspace Adult LaryngealHemangioma
Prognosis; T1 carcinomararely metastasizes(0-2%) Location: supraglotticregion(isolated);associated
due to absenceof lymphaticswithintrue with extensivecervicofacialangiodysplasia
vocal cords M>F
Bx: laserexcision,cryotherapy,selective
SubglotticGarcinoma embolization
lncidence: 5/" of all laryngealcancers
. late detectiondue to minimalsymptomatology LARYNGEAL PAPILLOMATOSIS
Stage: = RECURRENT RESPIRATORY PAPILLOMATOSIS
T1 confinedto subglotticarea 0 Squamouspapillomais the mostcommonbenigntumor
T2 extensionto vocal cords+ mobility of the larynx!
T3 tumor confinedto larynx+ cord fixation Etiology: humanpapillomavirus types 6 + 11 (Papova
T4 cartilagedestruction/ extensionbeyondlarynx viruscausinggenitalcondylomaacuminatum)
Prognosis; poor due to early metastasesto cervical Histo: core of vascularconnectivetissuecoveredby
lymphnodes (in 25% at presentation) stratifiedsquamousePithelium
Age of onset: 1-54 years; M:F = 1:1;
LARYNGEAL CHONDROSARCOMA bimodaldistribution
0 Themostcommon ofthelarynx
sarcoma (a) <10 years(diffuseinvolvement) = iuvenile
Age: 50-70 years;M >> F laryngotracheal papillomatosis; probablycaused
. lobulatedsubmucosalmass by transmission from motherto childduringvaginal
Location: posteriorlaminaof cricoidcartilage(50-70%), delivery
thyroid cartilage (20-35%) (b) 21-50 years(usuallysinglepapilloma)
{ coarse/ stipptedintratumoralcalcifications . progressivehoarseness/ aphonia
{ + locallyinvasive . repeatedepisodesof respiratorydistress
MR: . inspiratorystridor,asthmalikesymptoms
{ very highsignalintensityof tumormatrixon T2Wl . cough
(corresponding to hyalinecartilage) . recurrentpneumonia

. hemoptysis
Location: (a) uvula,palate(b) vocal cord (c) subglottic
extension(50-70%) (d) pulmonary
involvement (1-6%)
r/ thickenedlumpycords
r/ bronchiectasis
Cx:
(1) Tracheobronchial papiilomatosis (Z-S%)
Cause: tracheostomy
Location:lower lobe + posteriorpredilection
r/ solid pulmonarynodulesin mid + posteriorlung
fields
r/ Z-S cm largethin-walledcavitywith 2-4 mm thick
nodularwall (fociof squamouspapillomasenlarge
centrifugally,undergocentrafnecrosis,cavitate)
r/ peripheralatelectasis* obstructivepneumonitis
(2) Pulmonary papillomatosis
from aerialdissemination (bronchoscopy,
laryngoscopy,trachealintubation)10 years after
initialdiagnosis
r/ irregularities
of tracheal/ bronchialwalls
r/ noncalcifiedgranulomataprogressingto cavitation
(3) Malignanttransformationinto invasivesquamouscell
carcinoma
Rx: CO, laser resection/ surgicalexcision
LARYNG
EAL PLASMACYTOMA
Age: 50-70 years; M >> F
Histo: largesheetsof uniformcellsindistinguishable
from normalplasmacells;markedamyloid
deposition(20%)
. pedunculated i slightlyprominentmassthat bleeds
easily
Location: epiglottis,true + falsevocal cords
CT :
! largesmoothlymarginatedhomogeneous mass
r/ no significant
contrastenhancement
LARYNGOCELE
= dilatedappendix/ sacculusof the laryngealventricle
extendingbeyondthe superiorborderof the thyroid
cartilage
lncidence; 1:2,500,000
Age: middle-aged men
Anatomy: laryngealventricleof Morgagniis a slitlike
cavitybetweentrue + false cords;alongthe
anteriorthirdof its roofarisesthe smallblind
mucosa-lined laryngealsacculeof Hilton/
laryngealappendix;it extendssuperiorly
betweenfalse vocal cord and aryepiglotticfold
medially+ thyroidcartilagelaterally;the
laryngealappendixis relativelylarge in
infancy;usuallyinvolutesby 6th year of life
Pathogenesis.'chronicincreasein intraglotticpressure
Cause: excessivecoughing,shouting,playingwind
instrument,blowingglass,obstruction of
appendicular ostium(= secondarylaryngocele)
by chronicgranulomatous disease/ laryngeal
neoplasm(15%)
Ear, Nose,and ThroatDisorders 383
N.B.: Almost50% of laryngoceles detectedwith plain
radiography containa laryngealcarcinomal
Histo: lined by pseudostratified columnarciliated
epithelium+ mixtureof submucosalserousand
mucousglands
Types:
(a) internal(40%)= in paraPharyngeal space confined
withinthyrohyoidmembrane
(b) external(26%)= protrusionthroughthyrohyoid
membraneat the pointof insertionof the
neurovascularbundle(superiorlaryngealnerve
+ vessels)presentingas lateralneckmass near
hyoidbonewith normalsize insidethe membrane
(c) mixed (44%)= internal+ externaldilatationof
sacculeon both sidesof thyrohyoidmembrane
. visiblein 10% of adultsduringphonation
. hoarseness / dysphagia/ stridor(internallaryngocele)
. compressible anteriorneckmassjust belowangleof
mandible(externallaryngocele)
. Brycesign = gurgling/ hissingsoundon compression
Site: unilateral(80%),bilateral(23%)
{ sharplydefinedround/ oval radiolucent areawithin
paralaryngealsoft tissues
r/ increasein sizeduringValsalvamaneuver
^/ decreasein size duringcompression
r/ cysticmass that can be followedto levelof ventricle
r/ may be filledwith fluid/ containair-fluidlevel
r/ O|nCTOSTIC= colrrectionbetweenair sac + airway
Cx: infection(laryngopyocele) in 8-107o,formationof
mucocele
DDx: laryngealcyst (linedby squamousepithelium);
lateralpharyngealdiverticulum (fillswith barium)
LARYNGOMALACIA
= immaturityof cartilage;most commoncause of stridorin
neonate+ younginfant
. only cause of stridorto get worse at rest
r/ hypercollapsiblelarynxduringinspiration(supraglottic
portiononly)
^/ backwardbent of epiglottis+ anteriorkink of
aryepiglottic
foldsduringinspiration
Prognosis; transient(disappearsby age 1 year)
LINGUAL
THYROID
= solidembryonicrestof thyroidtissue,whichremains
ectopicalongthe tract of thyroglossalduct
lncidence; in 10/. of autopsies(withintongue<3 mm);
M<<F
. may be onlyfunctioning thyroidtissue(70-80%)
. asymptomatic (usually)
. may enlargecausingdysphagia/ dyspnea
Location: midlinedorsumof tonguenearforamencecum
(majority),
thyroglossal duct,trachea
CT: r/ smallfocusof intrinsichighattenuation
Cx: malignancyin 3Y"(papillarycarcinoma)
LYMPHANGIOMA
= corg€flitallymphaticmalformation
tncidencer benign
tumors
of infancy
l3iil,lall
384 Radiology Review Manual

Age: presentat birth in 50-65%, in 80-90% evidentby CysticHygroma

age 2 (timeof greatestlymphaticgrowth); M = F = CYSTIC LYMPHANGIOMA
Lymphatic development: = single/ multiloculated fluid-filledcavitieson eitherside
endothelialbudsfrom veinsin jugularregionform of fetalneck+ head (localized form)+ trunk
confluentplexuses,whichdevelopinto rapidlyenlarging (generalized form)as the mostcommonform of
bilateraljuguloaxillary lymphsacs (7.5weeksGA); lymphangioma developingwithinlooseconnective
these fused lymphsacs extendcraniadand dorsolateral tissue
with extensiveoutgrowthof lymphvesselsin all lncidence: 1:6,000pregnancies
directions;connectionwith internaljugular vein at level Path: multipleenormouslydilatedcysticlymphatic
of confluencewith externaljugular vein persistson the channels;varyingbetweena few mm to >10 cm
left side in diametercontainingchylousfluid;separatedby
Pathogenesis: minimalinterveningstroma;may invadeadjacent
(1) earlysequestration of embryoniclymphatictissue softtissues/ muscleand surroundvessels
with failureto join centrallymphaticchannels Histo: cysticspaces linedby endothelialcells
(2) congenitalobstruction of lymphaticdrainagedue to + supportingconnectivetissuestroma
abnormalbuddingof lymphvessels(= loss of Associated with:
connection/ noncommunication of primordial jugular (a) chromosomal abnormalities in 60-80% (in
lymphaticsac withjugularvein) particularwhen detectedin 2nd trimester)
(1) Turnersyndrome(45 XO, mosaic)in 40-80/"
Classification(on basis of size of lymphaticspaces): (2) Tri somi es13, 18,21, 13q,18P ,22
(1) Cysticlymphangioma = cystichygroma (3) Noonansyndrome
(2) Cavernouslymphangioma (4) Distichiasis(= secondrow of hairbehind
= mildlydilatedcavernouslymphaticspaceswith eyelash)-lymphedema syndrome
cysts of intermediatesize (5) Familialpterygiumcolli
Location: tongue,floorof mouth,salivaryglands (6) Roberts,Cumming,Cowchocksyndrome
ri penetrationof contiguousstructures (7) Achondrogenesis tYPell
{ same signalintensitiesas cysticlymphangioma (8) Lethalpterygiumsyndrome
+ fibrousstromalcomponentof low intensityon (b) exposureto teratogens
T lW l + T 2 Wl (1) Fetalalcoholsyndrome
(3) Capillary/ simplelymphangioma (leastcommon) (2) aminopterin
= capillarY-sized lymphaticchannels (3) trimethadione
Location: epidermis+ dermisof proximallimbs Types:
(4) Vasculolymphatic malformation (1) Cystichygromawith abnormalperipheral
composedof lymphatic+ vascularelements, lymphaticsystem
eg, lymphangiohemangioma r/ lymphangioma in posteriorcompartment of neck
r/ septations(indicatehigh probabilityfor
Histo: endothelial-lined lymphaticchannelscontaining aneuploidy, developmentof hydrops,and
serous/ milkyfluid+ separatedby connective perinataldeath)
tissuestroma (2) Diffuselymphangiectasia
. asymptomatic(in majority)soft / semifirmmass i/ lymphangioma of chest+ extremities
. + dyspnea/ dysphagiawith encroachment upon r/ peripherallymphedema+ nonimmunehydrops
trachea,pharynx,esophagus (3) lsolatedcystichygroma
. rapid increasein size (from infection/ hemorrhage) (a) axillarylymphsac malformation
r/ lymphangioma restrictedto axilla
Location: anywherein developinglymphaticsystem; (b) jugularlymphsac malformation
mostlyin posteriorcervicaltriangle, r/ lymphangiomarestrictedto lateralneck
occasionally in floorof mouth/ tongue (c) internalthoracic+ paratracheallymphsac
(a) posteriortriangleof neck (mostcommon),with malformation
extensionintomediastinum in 3-10% r/ lymphangioma withinmediastinum
. visibleat birth in 65% (d) combinedlymph sac malformation
. clinicallyapparentby end of 2nd decadein 90% (e) thoracicduct malformation
(b) anteriormediastinum (<1%) ^/ thoracicduct cyst
(c) axilla,chestwall,groin . AF-AFP/ MS-AFPmay be elevated
Cx: infection,ainruaycompromise, chylothorax, Location: neck (frequentlyposteriorcervicalspace)
chylopericardium and lowerportionsof face (75-80%),
mediastinum (3-10/o, in 1/2 extensionfrom
Prognosis; spontaneousregression(10-15%) neck),axilla(20o/"), chestwall (14o/"),face
Rx: surgicalexcision(treatmentof choicebut difficult (10%), retroperitoneum (kidneys),
since mass does not followtissueplanes)with abdominalviscera(colon,spleen,liver),
recurrencerate of up to 15% groin,scrotum,skeleton

US:
./ thin-walledfluid-filled
structurewith multiplesepta
of variablethickness+ solidcystwall components
{ fluid-fluidlevelwith layeringhemorrhagic
component
isolatednuchalcysts
.Vwebbedneck(= pterygiumcolli)followinglater
communication withjugularveins
nonimmunehydrops(43%)
^/ progressiveperipheraledema
{ fetal ascites
oligo-i polyhydramnios/ normalamountof fluid
bradycardia
CT:
^/ poorlycircumscribed
multiloculatedmasses
ri homogeneousattenuationof fluid values/ higher
(afterinfection)
MR:
{ hyperintense on T2Wl
{ low to highsignalintensityon Tl Wl (dependingon
proteincontentof fluid)
{ may be hyperintenseon TlWl (due to clottedblood
/ high chylouslipidcontent)
{ + fluid-fluidlevel(if hemorrhagepresent)
Cx: (1) Compressionof airways/ esophagus
(2) Slow growthi suddenenlargement
(hemorrhage, inflammation)
Prognosis:
(1) Intrauterinedemise(33%)
(2) Mortalityof 100%with hydrops
(3) Spontaneousregression(10-1 5%)
0 Favorableprognosisfor localizedlesionsof anterior
neck+ axilla
0 Only 2-3% of fetuseswith posteriorcystichygroma
becomehealthylivingchildren!
DDx: twin sac of blightedovum,cervicalmeningocele,
encephalocele, cysticteratoma,nuchaledema,
branchialcleftcyst, vascularmalformation,
lipoma,abscess
PseudocysticHygroma
= P S E UDO ME M BR AN E
= ?n€choicspace borderedby specularreflectionon
posterioraspectof fetalneckduring1sttrimester
Cause: ? developingintegument
./ trtOprominentposteriornutgei internalseptations
MADELUNG
DISEASE
= BENIGN SYMMETRICAL LIPOMATOSIS
= r?re benignconditioncharacterizedby depositionof
massiveamountsof adiposetissuein neck,shoulders,
upperchest
Cx: trachealcompressionwith respiratorycompromise
MALIGNANT EXTERNAL OTITIS
= seVefebacterialinfectionof the soft tissues+ bonesof
base of skull
Organism; almostalwaysPseudomonasaeruginosa
Age: elderly
Predisposed: diabetesmellitus/ immunocompromised
Ear, Nose,and ThroatDisorders 385
. unrelenting otalgia,headache
. purulentotorrheaunresponsiveto topicalantibiotics
. may causemalfunction of nervesVll, lX, X, Xl
Location: at bone-cartilage junctionof EAC
Spread of infection:
(a) inferiorlyintosofttissuesinferiorto temporalbone,
parotidspace,nasopharyngealmasticatorspace
(b) posteriorlyinto mastoid
(c) anteriorlyintotemporomandibular joint
(d) mediallyintopetrousapex
CT:
r/ soft-tissuedensityin externalauditorycanal (100%)
./ ttuiOin mastoidi middteear (89%)
./ diseasearoundeustachiantube (64%)
r/ obliterationof fat planesbeneathtemporalbone (64%)
r/ involvementof parapharyngeal space (54%)
r/ masticatorspacedisease(27%)
./ mass effect in nasopharynx(S4e/")
^/ bone erosionof clivus(9%)
ri intracranialextension(9%)
Cx: bone destruction,osteomyelitis,abscess
Prognosis: 20ohrecurrencerate
DDx: malignantneoplasm
MUCOCELE
= efld stage of a chronicallyobstructedsinus
lncidence; most commonlesionto cause expansionof
paranasalsinus;increasedincidencein cystic
fibrosis
Etiology: obstructedparanasalsinusostium
Path: expandedsinus cyst linedby mucosawith
accumulated secretionsand desquamations
Age: usuallyadulthood
. historyof chronicnasalpolyposis+ pansinusitis
. commonlypresentwith unilateralproptosis
. decreasedvisualacuity,visualfield defect
. palpablemass in superomedialaspectof orbit (frontal
mucocele)
. intractableheadaches
Location:
mnemonic.' "fems"
frontal(60%)> ethmoid(30%)> maxillary(10%)>
sphenoid(rare)
r/ softtissue densitymass
^/ sinuscavityexpansion(DDx:neverin sinusitis)
r/ bone demineralization + remodelingat late stage but
NO bonedestruction(impossibleDDx from neoplasm)
{ surrounding zone of bonesclerosisi calcification
of
edgesof mucocele(fromchronicinfection)
ri macroscopiccalcificationin 5% (especiallywith
superimposed fungalinfection)
ri uniformenhancement of thin rim
US:
r/ homogeneoushypoechoicmass
MR:
{ signalintensityvarieswith state of hydration,protein
content,hemorrhage, air content,calcification,
fibrosis
{ hypointenseon Tl Wl + signalvoid on T2Wl due to
inspissateddebris+ fungus
386 Radiology Heview Manual
r/ peripheralenhancementpattern(DDx from solid
enhancementpatternof neoplasms)
Cx: (1) protrusionintoorbitdisplacingmedialrectus
musclelaterally
(2) expansionintosubarachnoid spaceresultingin
CSF leak
(3) mucopyocele= superimposedinfection(rare)
DDx: paranasalsinuscarcinoma,Aspergillusinfection
(enlargementof medialrectusmuscle+ optic nerve,
OTICCAPSULEDYSPLASIA
tocal ldiffuse areas of increasedattenuation),
chronicinfection,invertingpapilloma
CARCINOMA
MUCOEPIDERMOID
Path: arisesfrom intercalatedducts of seromucinous
glands
Histo: composedof a mixtureof 3 cells: mucin-secreting
cells+ squamouscells+ mucouscells;arranged
in cords/ sheets/ cysticconfiguration
Prognosis; variable(well-encapsulated low-gradeto
infiltratinghighlyaggressivemalignancy)
Rx: completesurgicalremoval
ParotidMucoepidermoid
Carcinoma
0 Mostcommonmalignantlesionof parotidgland
0 ln children:up to 35h of all salivaryglandtumorsare
malignant-60% are mucoepidermoid carcinomas
. rock-hardmass
. pain / itchingalongcourseof facial nerve
. facial nerveparalysis
CT:
{ may containcystic low-attenuating areas
r/ focal calcifications(rare)
(a) low-gradelesion
r/ well-circumscribed parotidmass
r/ nypo-to isointenseon Tl Wl
{ hyperintenseon T2Wl
Rx: wide localexcision
(b) high-gradelesion
r/ infiltratingpoorlymarginated,more solid,relatively
homogeneouslesionwith few cysticareas
Rx: wide blockexcision+ radicalneck dissection
LaryngealMucoepidermoid
Carcinoma
lncidence;-100 cases
M : F= 6 : 1
(mostcommon)
Location: epiglottis
NASAL GLIOMA
= misnomer (noneoplasticfeatures)= NASALCEREBRAL
HETEROTOPIA
= rate developmentalmass composedof dysplastic
sequesteredneurogenictissuethat has become
isolatedfrom the subarachnoidspace
Age: usuallyidentifiedat birth
Location: extranasal(60%);intranasal(30%);
combinationof intra-and extranasal(10%)
Site: unilateralright> left side
. no changein size duringcrying
{ may parallelthe rate of braingrowth
{ attachedto middleturbinatebone / nasalseptum
{ soft-tissuemass of glabella
{ attachedto brain by stalk (10-30%)
MR:
./ iso- / hypointenserelativeto gray matteron TlWl
r/ hyperintenseon T2Wl
CochlearAplasia
= Michel aplasia = Michelanomaly= agenesisof
osseous+ membranouslabyrinth(rare)
Cause: arresteddevelopmentat 4 weeks GA
. totalsensorineural hearingloss
./ regionof otic capsulenormallyoccupiedby cochleais
replacedby denselabyrinthine + pneumatizedbone
r/ ttat medialwall of middleear (= undeveloped
horizontalsemicircular canal)
{ hypoplasiaof internalauditorycanal
r/ dysptasiaof vestibule= markedenlargementinto
regionof lateral+ superiorsemicircular canals
DDx: labyrinthitisobliterans(no loss of lateralconvexity
of medialwall of middleear)
Single-cavity Cochlea
= s€lcculdrdefect/ cavityin otic capsulein the position
normallyoccupiedby cochleawithoutrecognizable
modiolus,osseousspirallamina,interscalar septum
. profoundhearinglossdiscoveredin earlychildhood
May be associatedwith: recurrentbacterialmeningitis,
perilymphaticfistulaof oval
window
./ cysticcochlea(= developedbasalturn, middle
+ apicalturnoccupycommonnondeveloped space)
InsufficientCochlearTurns
= norrfl?lbasilarturn + varyingdegreesof hypoplasiaof
middleand apicalturns
Mondini malformation
= absenceof anterior1 1/2 turns of cochleaoftenwith
preservationof the basilarturn
Cause: in utero insultat 7 weeks GA
Frequency: 2nd most commonimagingfindingin
childrenwith sensorineural hearingloss
. some high-frequencyhearingpreserved
. vertigo
. otorrhea,rhinorrhea, recurrentmeningitis
(perilymphaticfistulacausedby absence/ defectof
stapesfootplate)
! absenceof cochlearapex
May be associatedwith: deformityof vestibule
+ semicircularcanals
+ vestibularaqueduct
Anomalies of Membranous Labyrinth
Scheibedysplasia= abnormalcochlea+ saccule
Alexanderdysplasia= dysplasiaof basal turn
r/ normalCT findings
 Ear, Nose,and ThroatDisorders 387

SmallInternalAuditoryCanal ^/ bony proliferationin reparativescleroticphase

= decreasein the diameterof IAC due to hypoplasiai difficultto diagnosebecauseof same densityas
aplasiaof cochlearnerve (portionof cranialnerve cochlea
vril) DDx: Pagetdisease,osteogenesisimperfecta,syphilis
. totalsensorineuralhearingloss
r/ hypoplasticanteroinferior
quadrantof IAC
PAPILLARY ENDOLYMPHATIC SAC TUMOR
LargeVestibule = H E FFN ETU R MOR
Associatedwith: underdevelopedlateralsemicircular = adenomatous tumorof the temporalbone
canal Origin: epithelialliningof endolymphaticsac
. sensorineural hearingdeficit(mostcommoncause) Associatedwith: von Hippel-Lindaudisease(may have
r/ lateralsemicircularcanalsmaller bilateralpapillaryendolymphaticsac
r/ vestibuleextendsfurtherinto lateral+ superior neoplasms)
aspectsof otic capsule . hearingloss,facialnervepalsy,vestibulardysfunction
r/ solid + cysticcomponents
LargeVestibularAqueduct r/ surroundedby thin shell of reactivebone
= Enlargedvestibularaqueductsyndrome ./ may be hypervascular(suppliedby branchesof external
Age: manifestsaround3 years carotidartery)
Frequency: most commonimagingabnormality r/ intratumoralcalcifications= "bonesequestra"from
detectedin childrenwith sensorineural destructionof petrousbone
hearingloss r/ contrastenhancement
. unilateralcongenitaldeafness(commonlymissed) MR:
. vertigo,tinnitus(in 50%) r/ speckledpatternof hyperintensity on Tl Wl
Location: bilateralin 50-66% (mi mi cki ng
gl omus tumor)
r/ vestibularaqueduct >1.4-2 mm in diametermeasured ,
ri may containbloodproducts(hyperintense on Tl Wl
halfwaybetweenposteriorpetrousbone and common + hypointenseon T2Wl)
crus at levelof vestibule DDx: paraganglioma,cysticand papillary
r/ vestibularaqueductlargerthan superiorand posterior adenocarcinoma, chondroidlesions(benign
semicircular canals chondroma,low-gradechondrosarcoma,
chondromyxoid fibroma),cholesterolgranuloma,
OTOSCLEROSIS metastaticdisease
= OTOSPONGIOSIS
= Ieplacementof dense otic capsuleby highlyvascular
spongybone in activephase(misnomer) with
restorationof densityduringreparativescleroticphase PARAGANGLIOMA
Etiology: unknown;frequentlyhereditary = NONCHROMAFFIN PARAGANGLIOMA = GLOMUS TUMOR
Age: adolescent/ youngadultCaucasian;M:F = 1:2 (describesthericharborizationof bloodvesselsandnerves)
= CHEMODECTOMA (reflective
of thechemoreceptortissueof
A. STAPEDIAL= FENESTRALOTOSCLEROSTS = GLOMERULOCYTOMA
origin) = ENDOTHELIOMA
(80-e0%) = PERITHELIOMA = SYMPATHOBLASTOMA
Location: anteriorovalwindowmargin(= fissulaante = FIBROANGIOMA = SYMPATHETIC NEVI
fenestram);bilateralin 85% = f?fe neuroendocrine tumorarisingfrom paraganglionic
. tinnitusearly in course (2/3) tissuefoundbetweenbaseof skulland floorof pelvis;
. progressiveconductivehearingloss (stapesfixation belong to amine-precursor-uptakedecarboxylation
in oval window) (APUD)systemcharacterizedby cytoplasmicvesicles
{ ovalwindowtoo wide (lyticphase) containingcatecholamines
{ new boneformationon anteriorovalwindowmargin Paraganglion-- collectionof tissueof the extraadrenal
+ posteriorovalwindowmargin+ roundwindow neuroendocrine system,frequentlylocatednear nerves
r/ completepluggingof oval *indo* = obliterative and vessels,with specialchemoreceptorfunction
otosclerosis(in 2%) Origin: arisesfrom nonchromaffinparaganglioncells of
B. COCHLEAR= RETROFENESTRAL OTOSCLEROSIS neuroectodermal origin;differsfrom adrenal
(10-20%) medullaonly in its nonchromaffin feature
lnvariablyassociatedwith: fenestralotosclerosis Neuroendocrine system :
. progressivesensorineuralhearingloss (involvement (a) Adrenalparagangliomaarisingfrom adrenalmedulla
of otic capsule/ cytotoxicenzymediffusioninto fluid = pheochromocytoma
of membranouslabyrinth) (b) Extraadrenalparaganglioma
. Schwartzesign = reddishhue behindtympanic 1. Aorticosympathetic paragangliomaassociated
membranewhen promontoryinvolved with sympatheticchain + retroperitoneal ganglia
./ "doubleringi doublelucent';= lucenthaloaround 2. Parasympathetic paraganglioma including
cochlea(may appearas 3rd turn to cochlea)in early branchiomericchemodectoma,vagal + visceral
phase autonomicparaganglioma
388 Radiology Review Manual

Glenner classification of extraadrenalparagangliomas: 0 Chemodectoma is misnomer(notderivedfrom

(a) Branchiomeric distribution chemoreceptorcells)t
1. Associatedwith greatvesselsof chest + rl€ck Histo: nestsof epithelioid cells("Zellballen")
with
includingcarotidbody,glomusjugulare,glomus granulareosinophilic cytoplasmseparatedby
tympanicum trabeculatedvascularizedconnectivetissue
(b) Parasympathetic distribution 0 Chromaffin-positive granules
2. Associatedwith vagal nerve (= catecholamines) may be present
3. Associatedwith aorticosympathetic chain in Function of carotid body:
thoracolumbar regionfrom aorticarchto urinary 5 x 3 x 2 mm carotidbody regulatespulmonary
bladder,includingorganof Zuckerkandl ventilationthroughafferentinputby way of
4. Associatedwith visceralorgans glossopharyngeal nerveto the medullaryreticular
Histo: acidophil-epithelioid cellsin contactwith formation
endothelial cellsof a vessel;storageof Chemoreceptor: detectschangesin arterialpartial
catecholamines (usuallynonfunctioning) ; pressuresol O, + CO, + PH
histologically similarto pheochromocytoma Stimulus: hypoxia> hypercapnia> acidosis
Age: rangeof 6 monthsto 80 years;peak age in Effect: increasein respiratoryrate + tidal
5-6th decade; F:M = 4:1 volume;increasein sympathetic tone
Associatedwith: pheochromocytoma (heartrate, blood pressure,
. paroxysmal/ permanenthypertension(due to secretion vasoconstriction, elevated
of vasopressoramines)with headache,pallor, catecholamines)
perspiration, palpitations . painlesspulsatilefirm neckmass belowthe angleof
. tumor may secretecatecholamine(= functional the jaw, laterallymobilebut verticallyfixed
paraganglioma);proportionof hormonallyactive Location: within/ outsideadventitiallayerof CCA at
tumorshighfor pheochromocytomas, intermediate for levelof carotidbifurcation, commonlyalong
aorticosympathetic paragangliomas, low for posteromedial wall; bilateralin 5% with
parasympathetic paragangliomas sporadicoccurrence,in 32% with
. pheochromocytomas secretenorepinephrine autosomaldominanttransmission
+ epinephrine, extraadrenal paragangliomas secrete r/ enhancingoval masswith splayingof ICA + ECA
only norepinephrine, some paragangliomas produce above CCA bifurcation
dopamine ^1no narrowingof ICA / ECA caliber
. determination of free norepinephrine mostsensitivewith Extension: inferiorlyto lowercranialnerves+ pharynx;
gas chromatography / high-pressure liquidchromato- superiorlyto skull base + intracranialcavity
graphy(HPLC)performedon 24-hoururinespecimens Growthrate: about 5 mm/Year
Locationof functioningparagangliomas: Cx: malignanttransformationin 6% with metastases
(a) adrenalmedulla(>80%) to regionallymphnodes,brachialplexus,
(b) extraadrenalintraabdominal(8-16%) cerebellum,lung,bone,pancreas,thyroid,
(c) extraadrenalin head & neck (2-4%) kidney,breast
Fourprimarysitesin head& neck.chest:
1. carotidbody Tumor
GlomusTympanicum
2. jugularforamen Mostcommontumorin middleear
3. path of vagus nerve . hearingloss,pulsatiletinnitus
4. m idd l ee a r . reddishpurplemassbehindtympanicmembrane
Lesscommonsitesin head& neck: Location: tympanicplexuson cochlearpromontoryof
sellaturcica,pinealgland,cavernoussinus,larynx middleear
(laryngealbranchesof vagusnerve),orbit(ciliary CT (bonealgorithmPreferred):
ganglionof the eye),thyroidgland,nasopharynx, r/ globularsoft-tissuemass abuttingpromontory
mandible,soft palate,face,cheek ^/ intenseenhancement
(d) multipleparagangliomas in up to 20"/",particularly in r/ usuallysmallat presentation (earlyinvolvement
of
hereditarydisorders(multipleendocrineneoplasia ossicles)
syndromes,neuroectodermal syndromes): ./ erosion+ displacementof ossicles
Synchronousmulticentricity in 3-26o/": r/ inferiorwall of middleear cavityintact
(a) autosomaldominantin 25-35% A ngi o:
(b) nonhereditaryin <5% { difficultto visualizebecauseof small size
Cx: malignanttransformation in 2-10%
GlomusJugulareTumor
CarotidBodyTumor Mostcommontumorin jugularfossawith intracranial
Embryology: extension
derivedfrom mesodermof 3rd branchialarch + neural Glomusjugulotympanicum tumor= largeglomus
crest ectodermcells,which differentiateinto jugularetumorgrowingintothe middleear
sympathogonia (= forerunnerof paraganglioniccells) Origin: adventitiaof jugularvein
 Ear, Nose,and ThroatDisorders 389

. tinnitus,hearingloss (2) mucosaof cochlearpromontoryrelatedto

. vasculartympanicmembrane
tympanicbranchof glossopharyngeal nerve
Location: at dome of jugularbulb (Jacobsonnerve)
r/ soft-tissuemass in jugularbulb region/ (3) auricularbranchof vagus nerve(Arnoldnerve)
, hypotympanum/ middleear space . slow growing+ asymptomatic
ri intenseenhancement r/ spherical/ ovoid/ spindle-shapedmass with sharp
r/ destructionof posteroinferior petrouspyramid interfacingmarginsand homogeneous enhancement
, + corticojugularspine of jugularforamen { nignlyvascularmass* neovaicularity + intense
r/ destruction of ossicles(usuallyincus),otic capsule, tumorbl ush
posteromedialsurfaceof petrousbone Cx: malignanttransformationwith metastasesin 15% to
MR: regionallymphnodes+ lung (otherparagangliomas in
r/ "saltand pepper"appearancedue to multiplesmall 10%)
tumorvessels
Angio:(filmentireneckfor concurrentglomustumors!)
^/ hypervascularmass with persistenthomogeneous P A R A N A S A L S IN U S C A R C IN OMA
reticularstain Location: maxillarysinus (80%),nasalcavity(10%),
^/ invasion/ occlusionof jugularbulb by thrombus/
ethmoidsinus(5-6%),frontal+ sphenoid
t um or sinus(rare)
r/ suppliedby tympanicbranchof ascending
pharyngealartery,meningealbranchof occipital MaxillarySinusCarcinoma
artery,posteriorauriculararteryvia stylomastoid lncidence: 80% of all paranasalsinus carcinomas
branch,internalcarotid arlery,internalmaxillarya. Hista: squamouscell carcinoma(80%)
r/ arteriovenous shunting Age: >40 yearsin 95%; M:F = 2:1
Cx: malignanttransformation with metastasesto ., asymmetryof face, tumor in oral / nasalcavity
regionallymph nodes (in 2-4%) r/ bone destruction(in 90%) predominatesover
expansion
GlomusVagaleTumor r/ nodal metastasesin 1O-18%
= PARAGANGLIA OF VAGUSNERVE= VAGALBODY
TUMOR Nasopharyngeal
Carcinoma
Histo: dispersedwithinperineurium/ below nerve lncidence: 10"/oof paranasalsinuscarcinomas;
sheath/ betweennervefiber fascicles;not
0.25-05% of all malignanttumorsin
organizedinto a compactmass whites:M>F
Location:
Predisposed: Chinesepopulation
( 1)wit hininf e ri ogr a n g l i o n(= g a n g l i o nn o d o sum),
Histo: squamouscell carcinoma(>85%),
inferiorto baseof skullcloseto jugularforamen
nonkeratinizing ca., undifferentiatedca.
(mostcommonlocation)
Mean age: 40 years
(2) withinsuperiorganglion(= ganglionjugulare) . asymptomatic for a longtime
withinbase of skullat levelof jugularbulb . historyof chronicsinusitis/ nasalpolyps(15%)
(3) elsewherealongcourseof vagus nerve . unilateralnasalobstruction
Location: turbinates(50%)> septum> vestibule>
lnferior NodoseParaganglion posteriorchoanae> floor
{ spindle-shapedmass Extension:
./ compressionof internaljugular vein (a) lateral+ superior:throughsinusof Morgagni
r/ displacementof carotidvesselsanteromedially (= naturaldefectin superiorportionof lateral
r/ displacementof lateralpharyngealwall mediaily
nasopharyngeal wall) into cartilaginousportionof
r/ minimaldestruction of skull6ase eustachiantube + levatorveli palatinimuscle
+ masticatorspace and pre- and poststyloid
Superi or Jugular ParagangIion parapharyngeal spaces
r/ dumbbell-shaped mass + involvement of levator+ tensorveli palatini
r/ may encase/ displaceICA
muscle,3rd divisionof nerveV, petroclinoid
r/ extension:
fissure
(a) superiorlyinto posteriorcranialfossa + foramenlacerumof skullbase encasinginternal
+ compressionof brainstem
carotidartery
(b) inferiorlyinto infratemporal / parapharyngeal + cavernoussinus(alongICA/ mandibularnerve/
space (2/3) directskullbase invasion)
(c) mediallyto involvearch of atlas
(b) anterior:posteriornasalcavity+ pterygopalatine
(d) laterallyinto middleear structures
fossa
(e) posteriorlyinto mastoidair cells
(c) inferior(1/3):submucosalspreadalonglateral
Locationin temporalbone: pharyngealwall + anteriorand posteriortonsillar
( 1) dom eo f j u g u l a rb u l b pi l l ars
390 RadiologyReviewManual

{ polypoidor papillary(213) ADENOMA

PLEOMORPHIC
r/ bone invasion(1/3) = BENIGN MIXEDTUMOROF PAROTIS
MR: mnemonic: 80/" in parotidgland
r/ signalintensitysimilarto that of adjacentmucosa 80% in superficiallobe
80% benign
EthmoidSinusCarcinoma lncidence: 80% of all benignparotidtumors;3rd most
lncidence: 5-6/" of paranasalsinus carcinomas commontumorin pediatricparotidgland(after
Histo: squamouscell carcinoma(>90%),sarcoma, hemangioma+ lymphangioma)
adenocarcinoma, adenoidcysticcarcinoma; Histo: mixtureof epithelial+ myoepithelialcells
frequentlysecondarilyinvolvedfrom maxillary Age: usually>50 years
sinuscarcinoma . slow-growing hardpainlesslump in cheek
. nasalobstruction,bloodydischarge r/ round/ oval/ lobulatedsharplymarginatedmass
. anosmia,broadeningof nose { rarelydystrophiccalcifications
r/ variable,usuallymildcontrastenhancement
US:
PAROTID HEMANGIOMA r/ nypo-to isoechoicmass
Frequency: 90/" of parotidgland tumorsduring 1st year { + hyperechogenic shadowingfoci of calcifications
o f l i f e ;M < F CT:
Histo: capillarytype >> cavernoustype (in older ^/ homogeneous well-definedtumor(if small)
children) ^/ less well-definedwith low-densitycenterif large
. soft-tissuemass developingshortlyafter birthwith (mucoidmatrix,hemnorrhage, necrosis)
progressivegrowthpeakingat age 1-2 years MR:
. gradualspontaneousregressionusuallycompleteby r/ hypointense on TlWl + hyperintense masson T2Wl
adolescence r/ hyperintenseareas in center(mucoidmatrix)
US: Rx: facialnerve-sparingpartialparotidectomy
r/ hypoechoicmass relativeto parotidtissue
{ variabledegreeof abnormalflow RANULA
CT: = TTTUCUS retentioncyst due to obstructionof sublingual/
r/ occasionallyphleboliths adjacentminorsalivarygland
r/ well-defined masswith uniformintenseenhancement (a) simple: confinedto sublingualspace
MR: (b) diving: extendsto belowmylohyoidmuscleinto
{ low to intermediate signalintensityon shortTR submandibular space
r/ Origntsignalintensityon longTR
^/ flow voids due to prominentvasculature RAMSAY-HUNT SYNDROME
Rx: surgery,sclerotherapy,laserablation(therapyonly = HERPES ZOSTER OTICUS
with largesize + encroachmenton adjacent . vesiclesin mucosaof externalauditorycanal
structuresdue to spontaneousregression) 8th nerveenhancement
r/ intracanalicular

RETROPHARYNGEAL ABSCESS / HEMORRHAGE

PHARYNGEAL ABSCESS Etiology: upper respiratorytract infection,tonsillar
Etiology: spread of infectionfrom tonsils/ pharynx infection,perforatinginjuryof pharynx/
Age: children> adults esophagus,suppurationof infectedlymph node
. trismus(mostcommonpresentingsymptom)from
Organism; Staphylococcus, mixedflora
involvementof pterygoidmuscle Age: usually<1 year
. sore throat . fever, neck stiffness,dysphagia
. low-gradefever
{ thicknessof retropharyngeal space >314of AP diameter
./ isodense/ low-densitymass with unsharpmargins
of vertebralbody
{ rim enhancement r/ reversalof cervicallordosis
Cx: mycoticaneurysmof carotidartery(within10 days) { anteriordisplacementof airway
./ may containgas and gas-fluidlevel

PLASMA CELL GRANULOMA RHABDOMYOSARCOMA

= rarabenignpseudotumor Frequency:
Cause: ?hypersensitivity 5-10% of all malignantsolidtumorsin children
Histo: polyclonalinfiltrationof normalplasmacells <15 yearsof age (ranking4th afterCNS neoplasm,
mixedwith otherinflammatory cells neuroblastoma, Wilmstumor);3rdmostcommon
+ nonnecrotizing epithelialcell granulomas primarychildhoodmalignancyof head+ neck (following
Location: lung,Gl tract,salivaryglands,larynx braintumors+ retinoblastomas); 10-25'h of all
r/ largehomogeneous submucosalmass sarcomas;annualincidenceof 4.5:1,000,000 white
DDx: multiplemyeloma,solitaryplasmacytoma + 1.3:1,000,000bl ackchi l dren
 Ear, Nose,and ThroatDisorders ggl

0 Mostcommonsoft-tissue tumorin childrenl SARCOIDOSIS

Age: 2-5 years(peakprevalence);<10 years(70%); = 10:1
B l acks:W hi tes
M : F= 2 : 1 Location: eye, lacrimalglands,salivaryglands(30%),
Histo: larynx(5%),involvement
"blue of intra-and
undifferentiated cells"with scant cytoplasm extraparotidlymph nodes (rare)
+ primitive-appearing nuclei;commonperineural r/ granulomasmay enhance
invasion ./ enlargementof optic canal (opticneuritis)
(a) embryonalrhabdomyosarcoma (>50%) r/ thickeningof larynxwith enhancementof granulomas
subtype: polypoidalform = sarcomabotryoides r/ multiplesmallgranulomasof septum+ turbinates
= grapelike
(b) alveolarrhabdomyosarcoma (worstprognosis) HeerfordtSyndrome
(c) pleomorphicrhabdomyosarcoma (mostlyin adults)
. cranialnerve palsy (1) Parotidenlargement
0 May be the initial+ only manifestationof sarcoid
Location: head + neck (28-36o/o), trigone+ bladderneck . diffusebilateralpainlessenlargement(10-30%)
(18-21 %), orbit (10/"), extremities(1g-23%), . xerostomia
trunk (7-8%), retroperitoneu m (6-7./"), CT:
perineum+ anus (2"/"),othersites(7%)
r/ dittusetydensemultiplenoncavitating nodules
Site: paranasalsinus,middleear, nasopharyngeal
withinglandi enlargement of intraparotidlymph
musculature (1/3)especiallyin masticatorspace;
nodes
mostcommonprimaryextracranial tumorinvading (2) Uveitis
the cranialvaultin childhood (3) Facial nerve paralysis
Metastases: lymphnodes(50%),lung,bone
{ bulkynasopharyngeal mass
r/ extensionintocranialvaultthroughfissures+ foramina SIALOSIS
= nontendernoninflammatory recurrentenlargementof
(up to 35%) usuallyinvolvingcavernoussinus
parotidgland
r/ bone destructionby directinvasion
r/ uniformenhancement Cause: cirrhosis,alcoholism, diabetes,malnutrition,
hormonalinsufficiency(ovarian/ pancreaticI
CT:
thyroid),drugs(sulfisoxazole,
phenylbutazone),
r/ heterogenousmass isodenseto brain
r/ expandedforamen/ fissure radiationtherapy
Histo: serousacinarhypertrophy+ fatty replacementof
MR (imagingmodalityof choice):
r/ signalintensityintermediatebetweenmuscleand fat gl and
Sialography:
, on Tl Wl + hyperintense on T2Wl
r/ sparseperipheralducts
r/ diffusecontrastenhancement
CT:
Prognosis: 12.5/" S-yearsurvival
r/ enlarged/ normal-sized gland
./ diffuselydense gland in end stage

SINONASAL POLYPOSIS
= benignsinonasalmucosallesion
RHINOCEREBRAL
MUCORMYCOSIS Incidence: in 257"of patientswith allergicrhinitis;
= paranasalsinus infectioncausedby nonseptatedfungi in 15% of patientswith asthma
Rhizopusarrhizusand Rhizopusoryzae Cause: allergicrhinitis(atopichypersensitivity), asthma,
Spread: fungusfirst involvesnasalcavity,then extends cysticfibrosis(child),Kartagenersyndrome,
into maxillaryi ethmoidsinuses/ orbits/ nickelexposure,nonneoplastic hyperplasia of
intracraniallyalong ophthalmicartery/ cribriform inflamedmucousmembranes
plate (frontalsinusesare spared) Location: commonlymaxillaryantrum
Predisposed: r/ roundedmasseswithinnasalcavityenlargingsinus
(1) poorlycontrolleddiabetesmellitus(2) chronicrenal ostium
failure(3) cirrhosis(a) malnutrition(5) cancer r/ expansionof sinus
(6) prolongedantibiotictherapy(7) steroidtherapy { thinningof bonytrabeculae+ erosivechangesat
(8) cytotoxicdrug therapy(9) AIDS (10)extensiveburns anteriorskull base
. blackcrustingof nasalmucosa(in diabetics) { usuallyperipheral/ occasionally solidheterogeneous
. smallischemicareas(invasionof arterioles+ small enhancement
, arteries) DDx: cancer,fungalinfection
! nodularthickeninginvolvingnasalseptum+ turbinates
./ mucoperiosteal thickeningi cloudingof ethmoids AntrochoanalPolyp
r/ focal areas of bone destruction = benignantralpolyp,whichwidensthe sinusostium
Cx: (1) blindness(2) cranialnervepalsy(3) hemiparesis and extendsinto nasalcavity;5./" of all nasal polyps
Prognosis; high mortalityrate Age: teenagers+ young adults
392 Radiology Review Manual
r/ antralclouding
r/ ipsilateralnasal mass
{ smoothmass enlargingthe sinusostium
^/ trlOsinus expansion
Angiomatous Polyp
= derivativeof choanalpolyp (followingischemiaof
polypwith secondaryneovascularity along its surface)
DDx: juvenileangiofibroma(involvementof
pterygopalatine fossa)
SINUS I T I S
lncidence:
mostcommonparanasalsinusproblem;mostcommon
chronicdiseasediagnosedin UnitedStates(31,000,000
peopleaffectedeachyear);complicating commoncolds
in 0.5% (3-4 coldslyearin adults,6-8 coldslyearin
children)
Pathogenesis:
mucosalcongestionas a resultof viral infectionleadsto
appositionof mucosalsurfacesresultingin retentionof
secretionswith bacterialsuperinfection
(1) Obstruction of majorostia
(a) middlemeatusdrainingfrontal,maxillary,anterior
et hm oi ds i n u s
(b) sphenoethmoidal recessdrainingposterior
ethmoidsphenoidsinus
(2) lneffectivemucociliaryclearingsecondaryto contact
of two mucosalsurfaces
Predi sposing anatomic variants:
(1) greaterdegreeof nasalseptaldeviation
(2) horizontallyorienteduncinateprocess
NOT conchabullosa,paradoxicalturbinate,Haller
cells,uncinatepneumatization
Location:
(1) lnfundibularpattern(26%)
= isolatedobstruction
of inferiorinfundibulum
abovethe maxillarysinusostium
r/ timitedmaxillarysinusdisease
(2) Ostiomeatalunit pattern(25%)
r/ middlemeatusopacification
(3) Sphenoethmoidal recessobstruction(6%)
r/ sphenoid/ posteriorethmoidsinusinflammation
(4) Sinonasalpolyposispattern
r/ enlargementof ostia,thinningof adjacentbone
r/ air-fluidlevels
Plainfilms(Waters,Caldwell,lateral,submentalvertex
views):
1. Acute sinusitis
r/ air-fluidlevel[from retentionof secretions
secondaryto mucosalswellingleadingto ostial
dysfunctionl(5a%sensitive,92% specificin
maxillarysinus)
r/ total opacification
./ hyperintensesecretionson T2Wl (95%water
content+ 5o/"proteinaceousmacromolecules)
2. Chronic sinusitis
rl mucosalswelling>5 mm thick on Watersview
(99%sensitive,46% specificin maxillarysinus)
./ bone remodeling+ sclerosis(fromosteitis)
./ polyposis
.l hyperattenuating lesionon NCCT (due to
inspissated secretions/ fungaldisease)
i/ hypointense secretionson Tl Wl + T2Wl due to
inspissatedmaterialwith chronicobstruction
(D D x:ai r)
CT: to map bony anatomyfor surgicalplanning
MR:
sinusthickeningwith highsignalintensityon T2Wl
+ low intensityon TlWl
{ near solid secretionswith >28% proteinconcentration
are hypointense on bothTlWl + T2Wl simulatingair
^/ rim gadoliniumenhancement(DDxto neoplasms,
whichenhancecentrallY)
A . A LLE R GICS IN U S ITIS
Prevalence: 10% of PoPulation
r/ involvesmultiplesinuses
r/ bilaterally symmetric
{ uniformenhancement
r/ sinonasalpolyposis
B . B A C TE R IA L S IN U S ITIS
Organism:
(a) acute phase: Streptococcuspneumoniae
+ Haemophilus influenzae(>50%),beta-hemolytic
streptococcus,Moraxellacatarrhalis
(b) chronicphase: staphylococcus, streptococcus,
corynebacteria,Bacteroides,f usobacteria
r/ solitaryantraldisease(obstruction of sinusostium)
! uniformenhancement
C . MY C OTIC/ FU N GA LS IN U S IT]S
Organism; Aspergillusfumigatus,mucormycosis,
bipolaris,Drechslera, Curvularia,Candida
r/ polypoidlesion/ fungusball (= extramucosal
infectiondue to saprophyticgrowthon retained
secretions,usuallycausedby Aspergillus)
just
r/ infiltratingfungalsinusitis(in immune-competent
host)
^/ fulminantfungalsinusitis(aggressiveinfectionin
immune-compromised / diabetics)
individual
CT:
r/ punctatecalcifications(= calciumphosphate/
calciumsulfonatedepositionnear mycelium)
MR:
^/ dark on T2Wl secondaryto high fungal mycelial
iron,magnesium,manganesecontentfrom amino
acid metabolism
(DDx: inspissatedsecretions/ polypoiddisease)
Dx: failureto respondto antibiotictherapy
Cx:
(1) Mucousretenti on cyst(10% )
(2) Mucocele
(3) Orbitalextensionthroughneurovascular foramina,
dehiscences, or thin bones: orbitalcellulitis,
(4) Septicthrombophlebitis
extension:meningitis,
(5) Intracranial epiduralabscess,
subduralempyema,venoussinusthrombosis,
cerebralabscess
 Ear, Nose,and ThroatDisorders ggg

Rx: functionalendoscopicsinus surgery(amputationof Location: lacrimal+ salivaryglands;mucousglandsof

uncinateprocess,enlargementof infundibulum conjunctivae,nasalcavity,pharynx,larynx,
+ maxillaryostium,creationof commonchannelfor trachea,bronchi;extraglandular involvement
anteriorethmoidair cells,complete/ partial i n 5-10%
ethmoidectomy) @ Chest
r/ pulmonaryfibrosis(10-147o,mostcommonfinding)
r/ reticulonodular pattern(3-33-52%) involvinglower
lobes(= lymphocytic pneumonitis)
interstitial
SJ6GRENSYNDROME ! patchyconsolidation
= MYOEPITHELIAL SIALADENITIS ./ inspissated mucus
= autoimmunemultisystem disorder(= collagen-vascular r/ atelectasis
disease)characterizedby inflammation+ destructionof ./ recurrentpneumonia
exocrineglands leadingto drynessof mucous r/ bilaterallower lobe bronchiectasis
membranesaffecting r/ acutelocal llipoidpneumonia(secondaryto oils
(1)salivary+ lacrimalglands taken to combatdry mouth)
(2) mucosa+ submucosaof pharynx r/ t pleuraleffusion
(3)tracheobronchial tree H R C T:
(4) reticuloendothelial system r/ bronchiectasis
( 5) joint s ri bronchiolar inflammation
A. PRIMARYSJOGRENSYNDROME ./ increasedparenchymaltines
= dutoiffiriluneexocrinopathy
@ Parotidgland
(a) recurrentparotitisin children Sialogram:
(b) SICCASYNDROME= Mikuliczdisease ^/ nonobstructive sialectasia(ducts+ acinidestroyed
= x€tophthalmia + xerostomia by lymphocyticinfiftrates/ infection)
B. S E CO NDA R YS J OGR ENS YN D R O ME Stage | : punctatecontrastcollection<1 mm
Associated with: Stagell : globularcontrastcollection1-2 mm
(a) connectivetissuediseases Stage lll : cavitarycontrastcollection>2 mm
1. Rheumatoidarthritis(5S%) Stage lV : destructionof gland parenchyma
2. Systemiclupuserythematosus (2%) US:
3. Progressivesystemicsclerosis(0.S%) enlargedheterogenousgland with punctateareas
4. Psoriaticarthritis,primarybiliarycirrhosis of increasedechogenicity(= mucus-filledducts)
(0.5%) multiplescatteredcysts bilaterally(= sialectasis=
(b) lymphoproliferative disorders cysticdilatationof intraparotidducts + glands)
1. Lymphocytic interstitial
pneumonitis (Llp) increasedvascularity on colorDoppler
2. Pseudolymphoma (25%) MR:
3. Lymphoma(5%;44 x increasedrisk): mostly inhomogeneous "honeycomb""salt
/ and pepper"
B-celllymphoma appearance(= €lreasof low intensitybetween
4. Waldenstrommacroglobulinemia nodularparenchymaof high signalintensity)on
Age: 35-70 (mean57) years; M:F = 1;9 T2Wl / Gd-enhanced TlWl
Path: benignlymphoepithelioma Cx: Salivaryglandlymphoma(occursin significant
Histo: lymphocyticinfiltrateassociatedductaldilatation. numberof patients+ followsan aggressivecourse)
acinaratrophy,interstitialfibrosis(= parotid
destruction) SUBGLOTTIC
STENOSIS
. xerostomia(mostcommonsymptom)= atrophyof
A. CONGENITALSUBGLOTTICSTENOSIS
salivary+ parotidglandsleadingto diminishedsaliva . crouplikesymptoms,often self-limitingdisease
productionand drynessof mouth+ lips Location: 1-2 cm belowvocal cords
. xerophthalmia = drynessof eyes r/ circumferentialsymmetricalnarrowingof subglottic
= keratoconjunctivitis sicca = desiccationof cornea portionof tracheaduringinspiration
+ conjunctiva ./ wO changein degreeof narrowingwith expiration
. xerorhinia= drynessof nose
B . A C QU IR E DS U B GLOTTICS TE N OS ]S
. decreasedsweating
followingprolongedendotracheal intubation(in 5%)
. decreasedvaginalsecretions
. swellingof parotidgland:
- recurrentacuteepisodeswith tenderness;usually THORNWALDT
CYST
= midlinecongenitalpouch/ cyst linedby ectodermwithin
unilateral
- chronicglandularenlargement nasopharyngealmucosalspace
with superimposed Origin: persistentfocal adhesionbetween notochord
acute attacksof painlessprogressiveswelling
. rheumatoidfactor(positivein up to gS%) + ectodermextendingto the pharyngealtubercle
. ANA (positivein up to 80%) of the occipitalbone
. mitochondrialantibodies(6%) lncidence: 4/o of autopsies
Peak age: 15-30 years
394 Radiology Review Manual

. asymptomatic incidentalfinding r/ tnicf irregularrim + variablesignalintensityof fluid

. persistentnasopharyngeal drainage with inflammation
. halitosis Cx: (1) infection
. foul taste in mouth (2) thyroidcarcinoma(-a%): in 80% papillaryca"
Location: posteriorroof of nasopharynx (3) squamouscellcarcinoma(evenrareQ
r/ smoothlymarginatedcysticmass of few mm to 3 cm in Rx: Sistrunkprocedure(= r€sectionof centralportionof
size hyoidbone + core of tissuefollowingthe expected
^/ low density,not enhancing courseof entirethyroglossal duct)with a
^/ tto bone erosion 2.6% recurrencerate
Cx: infectionof cyst DDx: ectopicthyroid(no thyroidtissuein normallocation)
DDx: Rathkepouch (occursin craniopharyngeal canal
locatedanteriorly+ cephaladto Thornwaldtcyst)

ADENOMA
THYROID
DUCTCYST
THYROGLOSSAL withenhancement
./ round/ ovalmassof lowattenuation
tncidence; most commoncongenitalneck mass (70% of
2nd most
all congenitalneck anomalies);
commonbenignneckmass afterbenign Adenomatous Nodule (42-77%)
lymphadenopathY = COLLOID NODULE = ADENOMATOUS HYPERPLASIA
Embryogenesrs: = DEGENERATIVE INVOLUTED NODULE
thyroglossal duct = duct alongwhichthyroidgland Cytotogy: abundantcolloid+ benignfollicularcells with
descendsto its final positionfrom foramencecum at uniformslightlylargenuclei,arrangedin a
base of tongue (in 3rd week GA) passinganteriorto honeycombpattern(difficultDDx from
hyoidbone;duct makesa recurrentloopthrough/ folliculartumors)
posteriorto precursorof hyoidbone beforefinally { oftenmultiplenodulesby US / scintigraphy / surgery
descending;inferiorend becomespyramidallobeof r/ mostlyhypofunctioning, rarelyhyperfunctioning
thyroid;thyroidreachesfinal locationby 7 weeks GA; r/ solidform = incompletelyencapsulated,poorly
duct usuallyinvolutesby 8-1Othweek of fetallife demarcatednodulesmergingwith surrounding tissue
Histo: cyst linedby stratifiedsquamousepithelium/ { cystic form (= colloidcyst) = anechoicareas in nodule
ciliatedpseudostratified columnarepithelium (hemorrhage / colloiddegeneration
+ mucousglands;ectopicthyroidtissuein 5-62oh ! calcificdeposits
Age: <10 years in 50%; 2nd peak at 20-30 years;M=F
. enlargingpainlessmidlineneck mass
. cyst moves upwardwith tongueprotrusion Follicular Adenoma (15-40%)
. + historyof previousincisionand drainageof an = rnorroclonal tumorarisingfrom follicularepithelium
"abscess"in area of cyst Path: singlelesionwith well-developed fibrous
Location: suprahyoid(15%),at levelof hyoid(20/"), capsule
infrahyoid(65%) Histo subtypes:
Site: midline(75/"), paramedianwithin2 cm of midline (a) Simplecolloid(macrofollicular) adenoma:
frequentlyon left (25%) mostcommonform
Size: 1.5-3 cm (rangingfrom 0.5 to 6 cm) (b) Microfollicular(fetal)adenoma
{ midlinecyst with occasionalseptation (c) Embryonal(trabecular)adenoma
./ infrahyoidcyst is embeddedwithinstrapmuscle (d) Hurthle-cell (oxyphil/ oncocytic)adenoma:large
r/ infrahyoidstrap musclesbeak over edge of cyst singlepolygonalcellswith abundantgranular
US: cytoplasm+ uniformeccentricnuclei+ no colloid
r/ anechoiccyst (42%)in midline (e) Atypicaladenoma
r/ hypoechoicmass with fine to coarseinternalechoes (f) Adenomawith PaPillae
(= proteinaceousmaterial)+ increasedthrough (g) Signet-ringadenoma
transmission 0 5"/"of microfollicular adenomas,57" of Hurthle-cell
Scintigraphy: adenomas,2S/"of embryonaladenomasproveto
r/ uptakein functionalthyroidtissueof thyroglossalduct be follicularcancerswith carefulstudy!
cyst Functional status:
CT: (1)Toxi cadenoma
r/ smoothwell-circumscribed midlinemasswith thinwall (2) Toxic multinodular goiter= hyperfunctioning
^/ homogeneous attenuationof 10-18 HU / occasionally adenomawithinmultinodular goiter;usuallyoccurs
higher(dueto increasedproteincontent) i n nodul e> 2.5cm i n si ze
{ peripheralrim enhancement (3) Nonfunctioning adenoma
MR: ^/ mass with increased/ decreasedechogenicity
r/ cyst hypointenseon TlWt + hyperintenseon T2Wl r/ "halosign" = completehypoechoicring with regular
r/ nonenhancing rim (unlessinflamed) bordersurroundingisoechoicsolidmass

THYROID CARCINOMA
lncidence:13,000
newcancers/year
in United
States;
clinicallysilentcancersin up to 35% at
autopsy/ surgery(usuallypapillary
carcinomasof <1.0cm in size)
Age: <30 years; M > F
Types (in order of worseningprognosis):
papillary(50-80%) > follicutar(10-20%) > meduilary
(6-10%)> anaplastic
. historyof neck irradiation
. rapidgrowth
. stone-hardnodule
r/ hypoechoic/ hypoattenuating mass
r/ irregularill-definedborderwithouthalo
r/ l*lOhemorrhage/ liquefactionnecrosis
r/ ancillaryfindings:
r/ lymphadenopathy
r/ destructionof adjacentstructures
r/ loss of fat planes
r/ distantmetastasis
RA DI A T I O N- I ND U C ED
T H YR OD
I C AN C E R
Incidenceincreaseswith doses of thyroidalirradiation
from 6.5-1,500 rad (higherdoses are associatedwith
hypothyroidism)
Peak occurrence; 5-30 (up to 50) years post irradiation
Thyroidabnormalitiesin 2Oy":
(a) in 14/" adenomatous hyperplasia,follicular
adenoma,colloidnodules,thyroiditis
(b) in 6% thyroidcancer
0 Nondetectablemicroscopicfoci of cancerin
25'/" of patientsoperatedon for benigndisease!
0 In patientswith multiplecold nodulesfrequencyof
cancer is 40/"
DIAGNOSTIC WHOLE-BODYSCAN
Indication; to detect metastasesof thyroid carcinoma
aftertotal thyroidectomy;preferredover
bone scan (onlydetects4O%)for skeletal
metastases
0 Metastasesnot detectablein presenceof normal
functioningthyroidtissuebecauseuptakeis much
less in metastases
0 Tc-99mpertechnetateis uselessbecauseof high
backgroundactivity+ lack of organification
0 False-negativel-131scan in 24./"secondaryto
nonfunctioning metastases
Technique:
(1) low iodinedietfor 7 days = ovoidiodizedsalt;milk
and diary products;eggs; seafood;bread made
with iodatedough conditioners;,red food dyes;
restaurantfood; food containingiodizedsalt, sea
salt, iodates,iodines,algin,alginates,agar agar
(2) To replacementtherapydiscontinuedfor 6 weeks
(3) short-actingT, is administeredtor 4-6 weeks
(a) T. replacement therapydiscontinued 1O-14days
priorto whole-bodyscan
Ear, Nose,and ThroatDisorders ggs
(5) measurementof TSH levelto confirmadequate
elevation(TSH>30-50 mlU/mL;administration of
exogenousTSH not desirablebecauseof uneven
stimulation)
(6) oral administration
of 0.6-5-10 mOi l-131
(7) whole-bodyscan atter 2448-72 hours (low
backgroundactivity)
N.B.: posttherapyscan (1 week aftertherapeuticdose)
identifiesmorelesionsthan diagnosticscan
Normalsites of accumulation:nasopharynx,salivary
glands,stomach,colon,bladder,liver(l-131-labeled
thyroxinproducedby carcinomais metabolizedin
liver),breastsin lactatingwomen (breastfeedingmust
be terminatedafteradministration of l-131)
CONTRAINDICATEDduringpregnancy!
TREATMENTREGIMENfor folliculari papillarycancer:
(1) Surgery:totalthyroidectomy + modifiedradicalneck
dissection
(2) Postoperative radioiodine treatmentwith l-131if
diagnosticscan positive(multipletreatmentsare
usuallynecessary)
0 Radioiodinetherapyonly appropriatefor papillaryI
mixed/ follicularthyroidcarcinomas(NOTfor
medullaryor anaplasticcarcinomas)
(a) Ablation of thyroid tissue remnants
Time interual: 6 weeks after surgery
. no thyroidhormonereplacementfor S-4 weeks
Calculated dose:
= {(thyroidweight[gl x B0-1Z01tCitg) n%
uptakeof l-123by 24 hours)x 100
Estimated dose:
= 30-100mC i l -131oral l y
> rescan atler 3-7 days:
r/ no changefrom pre-ablation:on
suppression therapy
r/ new foci (in up to 16%): considertherapy
r/ decreaseduptake: may be due to ,,stunning"
(b) Treatment of metastases
Middle-of-the-road dose:
" 100 mCi for residualneckactivity
" 150 mOifor regionallymphnode metastases
" 175 mCi for lung metastases
" 200 mCi for bone metastases
Tumor dose:
150 mOiof l-131with an uptakeof 0.5% per
gram of tumortissueand a biologichalf-lifeof
4 dayswill produce25,000radsto tumor
0 Rapidturnoverrates may exist in some
metastases(lowerdose advisable)
0 Treatmentof largetumors incomplete(rangeof
beta radiationis a few mm)
Cx: radiationthyroiditis,radiationparotitis,Gl
symptoms(nausea,diarrhea),minimalbone
marrowdepression,leukemi a (2/"),
anaplastictransformation (uncommon), lung
fibrosis(withextensivepulmonarymetastases
and dose >200 mOi)
396 Radiology Review Manual

(3) ThyroidreplacementtheraPY Anaplastic Carcinoma of Thyroad (4-15%)

exogenousthyroidhormoneto suppressTSH Age: 6th-7thdecade; M:F = 1:1
stimulationof metastases r/ intrathoracicextensionin up to 50%
(4) Externalradiationtherapyfor anaplasticcarcinoma v., larynx
r/ + invasionof carotida., internaljugular
+ metastaseswithoutiodineuptake NUC:
FOLLOW-UP: thyroglobulin >50 ng/mLindicates ./ ruOradioiodineuptake
functioning metastasesfollowing CT:
completeablationof thyroidtissue ! masswith inhomogeneous attenuation
{ areasof necrosis(74%)
Papillary Carcinoma of Thyroid (60-70%) r/ calcifications(58%)
Peakage: Sthdecade; F > M { regionallymphadenopathy (74%)
Histo: unencapsulatedwell-differentiatedtumor Prognosis: 5/" S-yearsurvival;averagesurvivaltime of
(a) purelypapillary 6-12 months
(b) mixedwith follicularelements(morecommon,
especiallyunderage 40) Medullary Carcinoma of Thyroid (1-5-10%)
Metastases: sporadic/ familial
(1) Lymphogenic spreadto regionallymphnodes Histo: arisesfrom parafollicular C-cells,associated
(40/", in childrenalmost90%) with amyloiddepositionin primary
(2) Hematogenous spreadto lung (4/"), bone (rare) + metastaticsites
. carcinomaelaboratesthyroglobulin Mean age: 60 yearsfor sporadicvariety;
NUC: in adolescence with MEN
{ tumorusuallyconcentrates radioiodine (evensome May be associated with:
purelypapillarytumors) (1) MEN lls = pheochromocytoma + parathyroid
US: hyperplasia (SiPPlesYndrome)
r/ tumor of decreasedechogenicity (2) MEN llb = withoutparathyroid component
r/ purelysolid/ complexmass with areasof necrosis, Metastases: earlyspreadto lymphnodes(50%),lung,
hemorrhage, cysticdegeneration liver,bone
X-ray: . elevatedcalcitonin(fromtumorproduction) stimulated
{ punctate/ linearpsammomatouscalcificationsat by pentagastrin + calciuminfusion
tumorperiphery ^/ mass of 2-26 mm
Rx: lobectomy+ isthmectomyfor papillarycancer r/ granularcalcifications withinfibrousstroma/ amyloid
<1.5to 2.0 cm in size isolatedto one lobe masses(50%)
Prognosis: 90% 10-yearsurvivalfor occult NUC:
+ intrathyroidalcancer;60o/"10-year ./ lttOuptakeby radioiodine/ pertechnetate
cancer;worse
survivalfor extrathyroidal ./ frequentlyshows increaseduptakeof Tl-201
prognosiswith increasingage CT:
r/ mass of low attenuation(no iodineconcentration)
FolficularCarcinoma of Thyroid (20%) Prognosis:
Peak age: 5th decade; F > M 90% 10-yearsurvivalwithoutnodal metastases
Histo: encapsulatedwell-differentiatedtumorwithout 42% 1O-yearsurvivalwith nodalmetastases
papillaryelements;in 25'/. multifocal; Rx: total thyroidectomy+ modifiedradicalneck
cytologically impossibleto distinguish dissection
betweenwell-differentiated follicular
carcinoma+ follicularadenoma(vascular
invasionis only criteria) THYROIDITIS
Earlyhematogenousspreadto: HashimotoThyroiditis
(a) lung = CHRONIC LYMPHOCYTIC THYROIDITIS
(b) bone (30%): almostalwaysosteolytic(more Most frequentcause of goitroushypothyroidism in
frequentthan in papillarycarcinoma) adultsin the USA (iodinedeficiencyis the more
. carcinomaelaboratesthyroglobulin commoncause worldwide)
./ psammomabodies+ stromalcalciumdeposits Etiotogy: autoimmuneprocesswith markedfamilial
NUC: predisposition; antibodiesare typically
! usuatlyconcentratespertechnetate,but fails to present;functionalorganification defect
accumulatel-123 Peak age: 4th-sth decade; M > F
US: . firm rubberylobulargoiter
r/ indistinguishable from benignfollicularadenoma . gradualpainlessenlargement
Prognosis; slow growing;90% 1O-yearsurvivalwith . thyrotoxicosisin early stage (4%)
slight/ equivocalangioinvasion; . decreasedthyroidreserve
35% |O-yearsurvivalwith moderaleI . hypothyroidism at presentation(20%)
markedangioinvasion ./ moderateenlargementof both lobes (18%)
 Ear, Nose,and ThroatDisorders gg7

NUC: PainlessThyroiditis
r/ low traceruptake(occasionallyincreased)with poor Histo: resembleschroniclymphocyticthyroiditis
visualization(4%) . clinicalpresentation
similarto subacutethyroiditis
{ prominentpyramidallobe . NOT painful/ tender
r/ positiveperchloratewashouttest
r/ patchytracerdistribution
AcuteSuppurativeThyroiditis
{ multiple(40%)/ singtecotd defects(28%)/ normal
thyroid(8%)
US:
r/ focal/ diffuseenlargement;possiblyabscess
US:
^/ decreasedechogenicity
^/ initialtyheterogeneousdiffuselydecreased
echogenicity + slightlobulationof contour
r/ markedhyperemiaon color Doppler
WARTHIN
TUMOR
= PAPILLARY CYSTADENOMA LYMPHOMATOSUM
! laterdenselyechogenic(fibrosis)+ acoustical = ADENOLYMPHOMA
shadows
lncidence: 2nd most commonbenigntumor of parotid
Cx: hypothyroidism
gland;bilateralin 10%
Age: about50 years; M > F
Origin: from heterotopicsalivaryglandtissuewithin
parotidlymph nodes (directresultof
DeQuervai
n Thyroiditis incorporation of lymphaticelements
= SUBACUTE THYROIDITIS + heterotopic salivaryglandductalepithelium
Etiology: probablyviral withinintraparotid+ periparotidnodesduring
Histo: lymphocyticinfiltration+ granulomas+ foreign embryonicdevelopment
bodygiantcells Histo: CHARACTERISTIC doublelayerof oncocytes
Peak age: 2nd-5th decade; M:F = 1:5 (= epithelialcells)restingon a denselymphoid
. upper respiratorytract infectionprecedesonset of
stroma
symptomsby 2-3 weeks . slow-growingpainlessmass
. painfultendergland+ fever;only mildenlargement
Location: often in tail of parotidgland
. hyperthyroidism (50%)secondaryto severe r/ well-circumscribed single/ muftiflecystici solidlesion
destruction in parotidregionusually3-4 cm in size
. short-livedhypothyroidism(25%)secondaryto
0 Mostcommonlesionto manifestas unilateral
hormonedepletionof gland + multifocalmasses
NUC: 0 Most commonsalivaryneoplasmto manifestas
r/ abnormallylow radioiodineuptakewith clinicaland multiplemassesin one / both parotidglands
, laboratoryevidenceof hyperthyroidism MR:
1 poorvisualization of thyroid(initially) r/ hypointensecomparedwith fat / surroundingparotid
r/ single/ multiplehypofunctional areas (occasionally) tissueon T2Wl
^/ increaseduptakeduringphase of hypothyroidism NUC:
(lateevent) ^/ increaseduptakewith Tc-9gm,Tl-201,FDG
Cx: permanenthypothyroidism (rare) DDx: lymphoma,inflammatorydisease
Prognosis; usuallyfull recovery Rx: surgicalresection
398 Radiology Review Manual
 DIFFERENTIAL
DIAGNOSISOF CHESTDISORDERS

PULMONARYHEMORRHAGE D . IN FE C TION (pneumoni a)

A. WITHOUT
RENAL
DISEASE 1. C hroni cbronchi ti s
1. Bleedingdiathesis:leukemia,hemophilia, 2. Bronchiectasis,mouthful(15"/")
disseminated intravascular coagulation(DlC) 3. Tuberculosis (Rasmussenaneurysm)
2. P ulm onarey m b o l i s m, th ro mb o e mb o l i s m 4. Aspergillosis
3. Blunttrauma: contusion 5. Abscess
4. ldiopathicpulmonaryhemosiderosis 0 In 5-1 0%.of patientsno cause is foundl
5. LimitedWegenergranulomatosis 0 The two mostcommonidentifiable causesare
6. Infectious diseases bronchialcarcinoma+ bronchiectasis!
7. Drugs: amphotericin B, mitomycin,high-dose DDx: hematemesis(containingfood particles,dark
cyclophosphamide, cytarabine(ara-C), blood,acid pH)
D-penicillamine, anticoagulants, lymphangiography
B. W I T HRE NA LD IS EA SE ASPIRATION
1. Goodpasture syndrorTle = anti-basement membrane = intakeof solid/ liquidmaterialsintothe airwaysand
antibodydisease l ungs
2. Collagenvasculardisease+ systemicvasculitides: Predisposing factors:
SLE,Wegenergranulomatosis, polyarteritis 1. Alcoholism(mostcommonin adults)
nodosa,Henoch-Schonlein purpura,Behget 2. Generalanesthesia,lossof consciousness
disease 3. Structuralabnormalities of pharynx/ esophagus
3. Rapidlyprogressive glomerulonephritis + immune (congenitaI / acquiredtracheoesophageal
complexes + tracheopulmonary fistula),laryngectomy
C . H E M O R R H A G IPCN E U M O N I A 4. Neuromuscular disorders
1. B ac t er ia: L e g i o n n a i re s ' d i s e a s e 5. Deglutition abnormalities
2. Viruses: CMV, herpes,RockyMountainspotted Substrate:
fever,infectiousmononucleosis (a) foreignbodies
3. Fungi: Aspergillosis, mucormycosis (b) l i qui ds
D. BLEEDINGMETASTASIS:Choriocarcinoma gastricacid = Mendelsonsyndrome
. acute respiratorydistress water = fl€€tfdrowning
. hemoptysis(uncommon)
barium,water-solublecontrastmaterial
C X R:
^/ bilateralheterogeneous + homogeneous opacities lipoidpneumonia/ fire-eaterpneumonia
r/ focalconsolidation (lesscommon) mineraloil / cod liveroil = chronicexogenous
CT: l i poi dpneumoni a
r/ bilateralscatteredi diffuseareasof ground-glass (c) contaminatedsubstancesfrom oropharynx/ Gl tract
opacity
PULMONARY DISEASE ASSOCIATED WITH
CIGARETTE
SMOKING
Hemoptysis 1. Bronchogenic carcinoma
. frothysputum,brightred blood,alkalinepH 2. Chronicbronchitis
Source: bronchiala. (mostcommon),pulmonarya. 3. Centrilobular
emphysema
A. T UM O R 4. Panacinaremphysemawith alpha-1antitrypsin
1. Carcinoma(35%) deficiency
2. Bronchialadenoma 5. Respiratorybronchiolitis-associated
interstitial
lung
B . B RO NCHI A W
L A L L IN J U R Y disease
1. Foreignbody erosion 6. PulmonaryLangerhanscell histiocytosis
2. BronchoscopyI biopsy
C. VASCULAR HYPERSENSITIVITY
TO ORGANICDUSTS
1. COPD A . TR A C H E OB R ON C H IA
HLY P E R S E N S ITIV ITY
2. Pulmonaryemboluswith infarction largeparticlesreachingthe tracheobronchial mucosa
3. Venoushypertension (mostcommon): mitral (pollens,certainfungi,some animal/ insectepithelial
stenosis emanations)
4. Arteriovenousmalformation 1. Extrinsicasthma
5. Ruptureof pulmonaryarteryaneurysm: 2. Hypersensitivityaspergillosis
TB, vasculitis,
trauma,neoplasm,abscess,septic 3. Bronchocentric granulomatosis
embolus,indwellingcatheter 4. Byssinosisin cotton-wool workers
400 Radiology Review Manual

B. ALVEOLARHYPERSENSITIVITY Prognosis; deathfrom respiratoryfailurewithin

= HYPERSENSITIVITY PNEUMONITIS 3 monthsof onsetof sYmPtoms
= EXTRINSIC ALLERGIC ALVEOLITIS ./ diffusealveolardamage(mostcommon)
small particlesof <5 pm reachingalveoli ./ nonspecific pneumoniai BOOP:
interstitial
! subpleurallinear/ nodularopacities(5-30
DAMAGE
PULMONARY
DRUG.INDUCED mm) in lowerlungzonesoccurringafter
Histopathologic manifestations : 1-3 monthsfollowingbeginningof therapy
(a) Diffusealveolardamage DDx: metastases
bleomycin,busulfan,carmustine,mitomycin, 5. Taxoidderivatives= paclitaxel,docetaxel,
cyclophosphamide, melphalan,gold salts gemcitabine, topotecan,vinorelbine
(b) Nonspecificinterstitialpneumonia lJse: breastcancer,lung cancer,ovariancancer
amiodarone,methotrexate, carmustine,
chlorambucil B. NONCYTOTOXIC DRUGS
(c) Bronchiolitis obliteransorganizingpneumonia 1. A mi odarone
bleomycin,gold salts,methotrexate, amiodarone, = triiodinated benzofuran
nitrofurantoi n, penicillamine, sulfasalazine, use: refractoryventriculartachyarrhythmia
cyclophosphamide Toxicity: in 5-10%; risk increasedwith daily
(d) Eosinophilic pneumonia dose > 400 mg + in elderlY
penicillamine, sulfasalazi ne, nitrofurantoin, Prognosis; good afterdiscontinuation of drug
anti-inflammatory drugs, . pulmonaryinsufficiency after1-12 monthsin
nonsteroidal
paraaminosalicylic acid 14-18/" on long-termtheraPY
(e) Pulmonaryhemorrhage r/ nonspecific pneumonia(most
interstitial
anticoagulants, amphotericin B, cytarabine common)+ associatedBOOP:
(ara-C),penicillamine, cyclophosphamide ^/ alveolar+ interstitialinfiltrates(chronic
A. CYTOTOXICDRUGS(most importantgroup) presentation)
1. Cyclophosphamide r/ focalhomogeneous peripheralconsolidation
Use: varietyof malignancies,Wegener (acutePresentation):
granulomatosis, glomerulonephritis r/ attenuationvaluesof iodine(due to
Toxicity: afler 2 weeks - 13 years (mean, incorporationof amiodaroneintotype ll
3.5 years),no relationship to dose/ PneumocYtes)
durationof theraPY r/ pleuralthickening(inflammation) adjacentto
Prognosis; good afterdiscontinuation of therapy consolidation
rl diffusealveolardamage (mostcommon) r/ associatedhigh-attenuation of liverrelativeto
./ nonspecific interstitial pneumonia spleen
r/ aOOP (leastcommon) 2. Goldsalts
2. Busulfan= Myleran@ (forCML) lJse: inflammatorY arthritis
Toxicity: dose-dependent,after 3-4 years on Toxicity: in |'h within2-6 months
t h e d r u gi n 1 - 1 0 % . mucocutaneous lesions(30%)
r/ diffuselinearpattern(occasionally r/ diffusealveolardamage (common)
reticulonodular / nodularpattern) r/ nonspecificinterstitialpneumonia(common)
r/ partial/ completeclearingafterwithdrawalof ^/ eOoP (lesscommon)
drug 3. Methotrexate,Procarbazine
DDx: Pneumocystispneumonia,interstitial lJse: lung cancer,breastcancer,head and neck
leukemicinfiltrate epidermoidcancer,nonmetastatic
3. Nitrosoureos = c€lllrlustine (BCNU),lomustine osteosarcoma,advancedstage NHL,
(ccNU) AML, recalcitrantpsoriasis,severe
lJse: CNS glioma,IYmPhoma, mYeloma rheumatoidarthritis,PemPhigus)
Toxicity: in 50% afterdoses >1500 mg/m2; Toxicity: in 5-10%; not dose-related
sensitivityincreasedafter radiationRx Prognosis; usuallyself-limiteddespite
r/ diffusealveolardamage(mostcommon) continuationof theraPY
^/ nonspecific pneumonia:
interstitial . bloodeosinoPhilia (common)
r/ linear/ finelynodularopacities(following ./ nonspecific pneumonia(most
interstitial
treatmentof 2-3 Years) common)
r/ nign incidenceof pneumothorax r/ BOOP (lessfrequent)
4. BleomYcin { linear/ reticulonodular process(timedelayof
lJse: squamouscell carcinomaof neck/ cervix/ 12 days to 5 Years,usuallYearlY)
vagina,Hodgkinlymphoma,testicularca. ./ acinarfillingpattern(later)
Toxicity: at doses >300 mg (in 34%); increased ./ transienthilaradenopathy+ pleuraleffusion
risk with age + radiationtherapy+ high (on occasion)
oxygenconcentrations DDx: PneumocYstis Pneumonia
 DifferentialDiagnosisof ChestDisorders 401

4. Nitrofurantoin (Macrodantin@) HP"u = extravascularhydrostaticpressure

Use: urinarytract infection OP,u = intravascular
oncolicpressure
Toxicity: rare OP"u = extravascularoncoticpressure
. positivefor ANA + LE cells K,,n = conductanceof capillarywall = water resistanceof
(a) acutedisorderwithin2 weeksof administration: celljunction
capillaryendothelial
. fever,dyspnea,cough t = oncoticreflectioncoefficient= permeabilityof
. peripheraleosinophilia capillarymembraneto macromolecules
(morecommon)
Prognosis: prompt resolutionafter
Cause: disturbed equilibrium of net flow Fn"tbetween
withdrawalfrom drug fluid transudation/ exudation Qrrtand lymphatic
^/ diffusebilateralpredominantlybasal
absorption Qryn,'pn
heterogenousopacities
(b) chronicreactionwith interstitialfibrosis(less Fnet=Q,,,,-Qlyrpn
c om m o n )
. insidiousonsetof dyspnea+ cough 1. lncreasedhydrostatic pressureedema:
. may not be associatedwith peripheral bat wing,asymmetricdistribution, in acuteasthma,
eo s i n o p h i l i a postobstructive,in acuteand chronicpulmonary
r/ nonspecific interstitial
pneumonia embolism,in pulmonaryvenoocclusive disease,near
(common) drowning
r/ bilateralbasilarinterstitialopacities 2. Permeability edemawith diffusealveolardamage
= ARDS
C. O T HE RS 0 Not caused/ influencedby concurrentcardiac
1. Heroin,propoxyphene, methadone insufficiencyl
Toxicity: overdosefollowed by pulmonary 3. Permeability edemawithoutdiffusealveolardamage:
edema in 30-40% heroin-induced, high-altitude,
following
r/ bilateralwidespreadairspaceconsolidation administrationof cytokines
r/ aspirationpneumoniain SO-75% 4. Mixededemadue to increasedhydrostaticpressure
2. Salicylates + permeabilitychanges:
. asthma neurogenic, reperfusion,reexpansion, air embolism,
r/ pulmonaryedema(withchronicingestion) postpneumonectomy, lungreduction,afterlung
3. Intravenouscontrastmaterial transplantation
r/ pulmonaryedema
A . IN C R E A S E D
H Y D R OS TA TICP R E S S U REEDEM A
DISORDERS
WITHHEPATIC
ANDPULMONARY (a) cardiogenic(mostcommon)
MANIFESTATIONS = pulmonaryvenoushypertension
1. cr1-antitrypsin
deficiency Pulmonary capillary wedge pressure (pCWp):
2. Cysticfibrosis = reflectsleft atrialpressureand correlates
3. Hereditaryhemorrhagic telangiectasia well with radiologicfeaturesof CHF
4. Autoimmunedisease: primarybiliarycirrhosis, + pulmonaryvenoushypertension
rheumatoidarthritis,Hashimotothyroiditis, Sjogren 0 In acuteCHF radiologicfeaturesare delayed
syndrome,scleroderma, sarcoidosis in onsetand resolution
5. Drugswith toxic effectson lung and liver:
methotrexate, phenytoin,amiodarone
rcWP [mm Hg] Findings
PULMONARY
EDEMA
= abnormalaccumulation 5-12 normal
of fluidin the extravascular 12-17 cephalizationof pulmonaryvessels
compartmentsof the lung
(onlyin chronicconditions)
Pathophysiology (Starling equation) :
17-20 Kerleylines,subpleuraleffusions
transcapillaryflow dependenton
>25 alveolarfloodingedema
(1) hydrostaticpressure
(2) oncotic(= colloidosmotic)pressure
(3) capillarypermeability(the endothetialcells are 1. Heartdisease: leftventricular failure,mitral
relativelyimpermeable to proteinbut remain valve disease,left atrialmyxoma
permeableto waterand solutes;the tight 2. Pulmonaryvenousdisease:acute/ chronic
junctionsof alveolarepithetium
intercellular pulmonaryembolism,primaryvenoocclusive
remainnearlyimpermeable to waterand solutes) disease,mediastinal fibrosis
3. Pericardialdisease:pericardial effusion,
constrictivepericarditis(extremelyrare)
Qrl = K,n(HP,"- HP"u)- t(OPi"- OP"")
4. Drugs: antiarrhythmic drugs;drugs
Q,* = amountof fluidfilteredperunitareaperunittime depressingmyocardialcontractility(beta-
HP' = intravascular
hydrostaticpressure blocker)
402 Radiology Review Manual

ri flow inversion= "cephalization of pulmonary Unusualform of pulmonary edema = lur'tgedema

vessels"is only seen in longstanding left heart from unusualcauses
failure,neverin pulmonaryedemaof renal
failure/ overhydration i low oncoticpressure
(b) noncardiogenic Interstitial Pulmonary Edema
1. Ren a lfa i l u re = 1st phaseof pressureedemawith increasein
2. lV fluidoverload quantityof extracellularfluid
3. Hyperosmolar fluid(eg,contrastmedium) Cause: increasein meantransmuralarterial
(c) neurogenic pressureof 15-25 mm Hg
? sympathetic venoconstriction in { earlylossof definitionof subsegmental + segmental
cerebrovascular accident,headinjury,CNS vessels
tumor,postictalstate r/ mildenlargement of peribronchovascular spaces
B . DE CRE A S E D C O L L OIDOS M O T ICP R E SS U R E ^/ appearanceof Kerleylines
1. Hy popro te i n e mi a r/ subpleuraleffusions
2. Transfusion of crystalloid fluid ri progressive blurringof vesselsdue to central
3. Rapidreexpansion of lung migrationof edemaat lobar+ hilarlevels
C. I NCRE A SE D C AP IL L AR YP ER M EA BIL ITY r/ smallperipheralvesselsdifficultto identifydue to a
Endothelial injuryfrom decreasein lungradiolucency
(a) physicaltrauma: parenchymal contusion, 0 Often markeddissociationbetweenclinicalsigns
radiationtherapy + symptoms+ roentgenographic evidence
(b) aspirationinjury: 0 Nothingdifferentiates it from otherinterstitial
lesions
1. Mendelsonsyndrome(gastriccontents) 0 Does not necessarilydevelopbeforealveolar
2. Neardrowningin sea wateri freshwater pulmonaryedema
3. Aspirationof hypertoniccontrastmedia 0 NOT typicalfor bacterialpneumonia
(c) inhalationinjury:
1. Nitrogendioxide= silo-filler's disease
2. Smoke(pulmoharyedemamay be delayedby Alveolar Flooding Edema
2448 hours) = 2nd phase of pressureedema
3. Sulfurdioxide,hydrocarbons, carbon Cause: increasein mean transmuralarterial
m on o x i d eb, e ry l l i u m,
c a d m i u m,s i l i ca, pressureof >25 mm Hg + pressure-induced
dinitrogentetroxide,oxygen,chlorine, damageto alveolarepithelium
phosgene,ammonia,organophosphates r/ tiny nodularI acinarareas of increasedopacity
(d) injuryvia bloodstream r/ frankconsolidation
1. Vesselocclusion:shock(trauma,sepsis,
ARDS)or emboli(air,fat, amnioticfluid,
thrombus) Bat-Wing Edema (in <10%)
2. Circulating toxins: snakevenom,paraquat = centralnongravitational of alveolar
distribution
3. Dr u g s : h e ro i n mo
, rp h i n eme , th a d o ne, aspi ri n, edema
phenylbutazone,nitrofurantoin, chlorothiazide Cause: rapidlydevelopingseverecardiacfailure
4. Anaphylaxis:transfusionreaction,contrast (acutemitralinsufficiencyassociatedwith
mediumreaction,penicillin papillarymusclerupture,massiveMl, valve
5. Hypoxia: high altitude,acute largeairway leafletdestructionby septicendocarditis)or
obstruction renalfailure
r/ tungcortexsparedfrom fluid(dueto pumpingeffect
mnemonic; "ABCDEFGHI- PRN" of respiration/ contractilepropertyof alveolarsepta
Aspiration / mucopolysaccharide-filled perivascularmatrix)
B ur ns
Chemicals Asymmetric Distribution of PressureEdema
Drugs(heroin,nitrofurantoin,
salicylates) Cause: morphologic lungchangesin COPD,
Exudativeskin disorders hemodynamics, patientposition
Fluidoverload r/ tungapicesspared(= lungemphysemain heavy
Gram-negative shock smokers)
Heartfailure ./ upper + middleportionsof lung spared
Intracranial
condition (= end-stageTB, sarcoidosis, asbestosis)
Polyarteritisnodosa ./ predominantly RUL involvement (= mitral
Renaldisease refluxespreferentially
regurgitation into rightupper
Neardrowning pulmonaryvein)
gradienton CT in recumbent
r/ anteroposterior
Atypical pulmonary edema = lung edemawith an position
unusualradiologicappearance r/ unilateraledema in lateraldecubitusposition
 DifferentialDiagnosisof ChestDisorders 409

Pulmonary Edema with Acute Asthma r/ itt-detined

vessels+ peribronchialcuffing
Cause: airtrapping a positive
maintains { markedly asymmetric gravity-dependenidistribution
intraalveolarpressureand thus decreases of edema(motionless recumbent position
for hours
hydrostaticpressuregradient / days)
Pathogenesis: associatedwith severityof Mriller r/ resolution
within1 or 2 daysin uncomplicated
maneuver CASCS
r/ heterogenousedema (dueto nonuniformainuay Cx:
obstruction) (1) extensivecrushinjurieswith associatedmuscle
r/ peribronchialcuffing damageand ensuingrenalinsufficiency (from
r/ itt-defined
vessels motionless recumbency)
^/ enlargedill-definedhila (2) aspirationof gastriccontents
! patencyof narrowedairuvaysmaintained(due to Prognosis: 10o/omortalityrate
high negativepleuralpressurein forced inspiration)
Pulmonary Edemafollowing Administration of
PostobstructivePulmonary Edema Cytokines
Cause: followingrelief from an upper airway (a) intravenousinterleukin2 (lL-2):
obstruction(impactedforeignbody, enhancestumoricidal activityof naturalkillercells
laryngospasm, epiglottitis,
strangulation) in metastaticmelanoma+ RCC
Pathogenesis: (b) intraarterialtumor necrosisfactor:
(a) forcedinspirationcausesa high negative increasesproduction+ releaseof lL-2
intrathoracic
pressure(Mullermaneuver)and Frequency:in 75ohof lL-2 therapy;in 20o/oof tumor
increasesvenous return necrosisfactortherapy;in 2Sh of
(b) obstructioncreateshigh positiveintrathoracic recombinantIL-2 therapy
pressurethat impairsdevelopment of edema Pathophysiology:permeabilitydisruptionof capillary
{ septallines,peribronchial cuffing endothelialcells
! centralalveolaredema . 12 mm Hg increasein pulmonarycapillarywedge
./ normalheartsize
Prognosis: resolutionwithin2-3 days , pressure(directtoxic effecton myocardium)
r/ pulmonaryedema 1-5 days afterstart of therapy:
r/ bilateralsymmetricinterstitialedemawith thickened
Edema with Pulmonary Embotismk1o%) septallines
Cause: occlusionof pulmonaryarterialbed causes { peribronchialcuffing QSo/.)
redirectionof bloodflow and hypertension { small pleuraleffusions(40;p/")
in uninvolvedareas ! no alveolaredema (unlessassociatedcardiac
^/ areas of ground-glassattenuation insufficiency)
r/ sharplydemarcatedfrom areas of transparency
distal to occludedarteries High-altitude Pulmonary Edema
r/ associatedwith dilatedpulmonaryarteries(70%) Predisposed. young males after rapid ascent to
>3,000meters
Pulmonary Edema with pulmonary Cause: prolongedexposureto low partialoxygen
Venoocclusive Disease atmosphericpressure
Cause: organizedthrombiin smallveins causesan Pathophysiology: acute persistenthypoxiawith
increasein peripheralresistanceand endothelialleakage
hydrostaticpressure . prodromalacute mountainsickness
. rapidlyprogressivedyspnea,orthopnea . dyspneaat rest,coughwith frothypink sputum
. + hemoptysis . neurologicdisturbances (dueto brainedema)
. normal/ low pulmonarycapillarywedgepressure ., arterialoxygenlevelsas low as 38%
tr enlargedpulmonaryarteries r/ centralinterstitial
pulmonaryedema
r/ diffuseinterstitial
edema+ numerousKerleylines ! peribronchial cuffing
ri peribronchialcuffing r/ ill-definedvessels
d ditateOrightventricle r/ patctryairspaceconsolidation

Heroin-induced Pulmonary Edema NeurogenicPulmonary Edema

Hx: overdoseof opiates(almostexclusivelywith
heroin,rarelywith cocaine/,,crack")
Frequency: 15/" of cases of heroin overdose
Pathophysiology: depressionof medullary
respiratorycenterleadingto
r/widespr""o
put.r[IffiiilT'r.t|n consoIidatio
ns
Frequency;in up to 50% of severebraintrauma,
subarachnoid hemorrhage, stroke,status
epilepticus
Pathophysiology: modificationin neurovegetative
pathwayscausessuddenincrease
in pressurein pulmonaryvenules
with reducedvenousoutflow
404 Radiology Review Manual

. dyspnea,tachypnea,cyanosisshortlyafter brain PostpneumonectomyPulmonary Edema

insult+ rapiddisappearance = life-threatening
complication in the early
./ bilateralinhomogeneous i homogeneous airspace postoperativeperiodafter pneumonectomy(rarein
consolidations,in 50/o atlectingpredominantlythe lobectomyor lungreductionsurgery)
apices,disappearing within1-2 days Frequency: 2.5-5/"; R > Lpneumonectomy
Dx: by exclusion Riskfactors: excessiveadministration of fluidduring
DDx: fluid overload,postextubationedema surgery,transfusionof fresh trozen
plasma,arrhythmia,marked
postsurgicaldiuresis,low serum
colloidalosmoticpressure
Beperfusion Pulmonary Edema Pathophysiology: increasedcapillaryhydrostatic
Frequency: in up to 90-100% pressure,alteredcapillary
Cause: pulmonarythrombendarterectomy for permeability
massivepulmonaryembolism/ for webs . markeddyspneaduringfirst 2-3 postopdays
and segmentalstenosis ^/ nnOS-tikeoicture
Pathophysiology: rapid increasein blood flow Prognosis.' very high mortalityrate
+ pressure
. dyspnea,tachypnea,coughduringthe first
Pulmonary Edemaafter Lung Transplantation
2+48 hoursafter reperfusion in up to 97"/oduring first 3 days after
Frequency;
{ pulmonaryedemawithin2 days aftersurgery: surgery
r/ heterogeneousairspaceconsolidations, Pathophysiology: tissue hypoxia,disruptionof
predominantlyin the areasdistalto the recanalized pulmonarylymphaticdrainage,
vessels lungdenervation
r/ randomdistributionin up to 50%
{ progressivediffuseconfluentareasof increased
opacity,most pronouncedon postopday 5
{ returnto normal2 weeks after surgery
Reexpansion Pulmonary Edema
Cause: rapid reexpansionof a collapsedlung
followingevacuationof hydrothorax, UnilateralPulmonaryEdema
hemothoraxor pneumothorax A. IPSILATERAL = on side of preexisting
abnormality
Pathophysiology: prolongedlocal hypoxicevent, (a) fillingof airways
abruptrestorationof bloodflow, 1. Unilateralaspirationi pulmonarylavage
suddenmarkedincreasein 2. Bronchialobstruction(drownedlung)
pressure,diffuse
intrapleural 3. Pulmonarycontusion
alveolardamage (b) increasedpulmonaryvenouspressure
. frankrespiratory insufficiency:cough,dyspnea, 1. Unilateral venousobstruction
tachypnea,tachycardia,frothypink sputum 2. Prolongedlateraldecubitusposition
. may be asymptomatic (c) pulmonaryarterialoverload
r/ pulmonaryedemawithinreexpandedentirelung 1. Systemicartery-to-pulmonary arteryshunt
withint hour (in 6a%) (Waterston,Blalock-Taussig, Pott procedure)
./ increasein severitywithin 2448 hourswith slow 2. Rapidthoracentesis (rapidreexpansion)
resolutionover next 5-7 days B. CONTRALATERAL= oppositeto side of abnormality
Prognosis: ZOY"mortality (a) pulmonaryarterialobstruction
1. Congenitalabsencei hypoplasiaof
pulmonaryartery
Pulmonary Edemadue to Air Embolism 2. Unilateralarterialobstruction
Cause: usuallyiatrogeniccomplication 3. P ul monarythromboembol i sm
(neurosurgical procedurein sittingposition, (b) lossof lung parenchyma
placement/ manipulation of centralvenous 1. Swyer-James syndrome
line),rare in open/ closedchesttrauma 2. Unilateralemphysema
Pathophysiology:embolizedair bubblescause 3. Lobectomy
mechanicalobstruction of 4. Pleuraldisease
pulmonarymicrovasculature C . R IGH TU P P E RLOB E
. suddenonsetof chestpain,tachypnea,dyspnea PATHOGNOMONIC for mitralvalveregurgitation
. hypotension
r/ air bubblesin right-sidedcardiacchamberson
echocardiography Pulmonary Edema with Cardiomegaly
r/ interstitialedema 1. C ardi ogeni c
r/ bilateralperipheralalveolarareas of increased 2. Uremic(withcardiomegalyfrom pericardialeffusion/
opacity,predominantlyat lung bases hypertension)
 DifferentialDiagnosisof ChestDisorders 405

PulmonaryEdemawithoutCardiomegaly D. BILATERALLOWERLOBE PNEUMONIA

mnemonic.' "U DOPA" - Normalhost: anaerobic(aspiration)
Uremia - Compromisedhost: anaerobic(aspiration)
Drugs E. PERIPHERALPNEUMONIA
Overhydration - Noninfectious eosinophilic pneumonia
Pulmonaryhemorrhage Transmission:
Acute myocardialinfarction,Arrhythmia A. COMMUNITY-ACQUIRED PNEUMONIA
Organism.' viruses,S. pneumoniae,Mycoplasma
Noncardiogenic
PulmonaryEdema Mortality: 10%
mnemonic.- "Thealphabet" B. NOSOCOMIALPNEUMONIA
ARDS,Alveolarproteinosis, Aspiration,Anaphylaxis (a) Gram-negative organism(>50%): Klebsiella
Bleedingdiathesis,Bloodtransfusionreaction pneumoniae, P. aeruginosa,E. coli,Enterobacter
CNS (increasedpressure,trauma,surgery,CVA, (b) Gram-positiveorganism(10%): S. aureus,
cancer) S . pneumoni ae,
H . i nfl uenzae
Drowning(near),Drug reaction Complications:
Embolus(fat,thrombus) 1. E mpyema
Fluidoverload,Foreign-bodyinhalation 2. Pulmonaryabscess
Glomerulonephritis, Goodpasture
syndrome, 3. Cavitarynecrosis
Gastrografin aspiration 4. Pneumatocele
Highaltitude,Heroin,Hypoproteinemia 5. Pneumothorax
lnhalation(SO' smoke,CO, cadmium,silica) 6. Pyopneumothorax
7. Bronchopleural fistula
Narcotics,Nitrofurantoin
Oxygentoxicity BacterialPneumonia
Pancreatitis Lobar Pneumonia
= ALVEOLAR PNEUMONIA
Rapidreexpansionof pneumothoraxi removalof = pathogensreachperipheralair space,incite
pleuraleffusion
_ exudationof wateryedema into alveolarspace,
centrifugalspreadvia small ainruays, pores of Kohn
Transfusion
+ Lambertinto adjacentlobules+ segments
Ur em ia ^/ nonsegmental sublobarconsolidation
./ roundpneumonia(= uniforminvolvement of
PN E UM O NI A
"Classic"pneumoniapattern: contiguousalveoli)
(a) Streptococcus pneumoniae
1. Lobardistribution: Streptococcus pneumoniae (b) Klebsiellapneumoniae(moreaggressive); in
2. Bulgingfissure : Klebsiella immunocompromised + alcoholics
3. P ulm onar ey d e ma : V i ra lp n e u m o n i a , (c) any pneumoniain children
Pneumocystis pneumonia (d) atypicalmeasles
4. Pneumatocele : Staphylococcus r/ expansionof lobe with bulging
5. Alveolarnodules : Varicella,bronchogenic - of fissures
spread r/ lung necrosiswith cavitation
of TB r/ lack of volume loss
Distribution: DDx: Aspiration,pulmonaryembolus
A. SEGMENTAL/ LOBAR
- Normalhost: S. pneumoniae,Mycoplasma,
Lobular Pneumonia
virus = B R ON C H OP N E U MON IA
- Compromised host: S. pneumoniae = combination of interstitial
+ alveolardisease(injury
B . B RO NCHO P N E U MON IA startsin ainrvays,involvesbronchovascular
- Normalhost: Mycoplasma,virus, Streptococcus, bundle,
spillsintoalveoli,whichmay containedemafluid,
Staphylococcus, S. pneumoniae blood,leukocytes, hyalinemembranes,organisms)
- Compromisedhost: Gram-negative,
Organisms:
Streptococcus,Staphylococcus (a) Staphylococcus aureus,Pseudomonas
- Nosocomial:Gram-negative, pseudomonas, pneumoniae:thrombosisof lobularartery
Klebsiella,Staphylococcus brancheswith necrosis+ cavitation
- lmmunosuppressed: Gram-negative, (b) Streptococcus (pneumococcus), Klebsiella,
Staphylococcus, Nocardia,Legionella, Legionnaires' bacillus,Bacillusproteus,E. coli,
AspergiIlus, Phycomycetes anaerobes(Bacteroides+ Clostridia),Nocardia,
C. E X T E NS I V E
B IL AT E R A L
P N E U MON IA actinomycosis
- Normalhost: virus(eg, influenza), Legionella (c) Mycoplasma
- Compromisedhost: candidiasis, pneumocystis, r/ smallfluffyill-definedacinarnodules,whichenlarge
tuberculosis withtime
406 Radiology Review Manual

r/ lobar+ segmentaldensitieswith volumelossfrom 0 Atypicalmeaslespneumoniadoes NOT showthe

airwayobstructionsecondaryto bronchial findingsof viralpneumonias!
typicalradiographic
narrowing+ IT]UGUSplugging

Atypical BacterialPneumonia lnterstitialPneumonia

= bacterialinfectionwith radiographicappearanceof Acute lnterstitialPneumonia
viralpneumonia = NONBACTERIAL PNEUMONIA
Organism: 0 Initiallypredominantlyaffectinginterstitialtissues
(1) Mycoplasma Organisms: viruses,Mycoplasma,Pneumocystis
(2) Pertussis . often subacuteatypicalpneumonia
(3) Chlamydiatrachomatis r/ diffuseinterstitialprocesswith peribronchial
thickening
ViralPneumonia { segmentalilobardensities(mucusplugging
= infectionof bronchi+ peribronchial tissues + damage of surfactant-producing type 2 alveolar
Pathophysiology: cells)
tracheitis; bronchitis; bronchiolitis ; peribronchial
+ interstitial
septa infiltrates;injury to alveolarcells
Chronic lnterstitialPneumonia
= diversegroup of inflammatorydisordersthat can
with hyalinemembranes;necrosisof alveolarwalls
progressto PulmonarY fibrosis
with blood,edema,fibrin,macrophages in alveoli
Modified Liebow classification:
Organism: pneumoni a(U l P )
1. U suali ntersti ti al
(a) lnfluenzavirus: cavitarylesionconfirms
2. Desquamativeinterstitialpneumonia(DlP)
superimposed infection
3. Bronchiolitis obliteranswith organizing
(b) Coxsackievirus,echovirus,reovirus
pneumonia(BOOP)
(c) Parainfluenzavirus
added:
(d) Adenovirus
4. Acuteinterstitial pneumonia= Hamman-Rich
(e) RSV = respiratorysyncytialvirus (12%)
syndrome
(f) Rhinovirus(43%)
5. Nonspecific pneumonitis
interstitial
(g) Cytomegalic inclusionvirus: featuressuggestive
6. Respiratorybronchiolitis-associated interstitial
of bronchopneumonia
lungdisease
(h) Varicellai herpeszoster: 10% of adults;2-5 days
no l ongeri ncl uded:
after rash pneumonia(LlP)
1. Lymphoidinterstitial
( i) Rubeola(measles)= before/ with onsetof rash;
= potentialtymalignantlymphoproliferative
followingovertmeasles= giantcell pneumonia
disorder
(i) Mycoplasma(10%) 2. Giantcell interstitial pneumonia(GlP)
Path: necrosisof ciliatedepithelialcells,gobletcells, = mdnifestationof hard-metalpneumoconiosis
bronchialmucousglandswith frequent
involvement of peribronchial tissues+ interlobular
septa
Age: mostcommoncauseof pneumoniain children
Pneumonia
Gram-negative
In 50% causeof nosocomialnecrotizing pneumonias
under5 years of age
(includingstaphylococcal pneumonia)
Distribution: usuallybilateral
./ "dirtychest"= peribronchialcuffing+ opacification: Predisposed: elderly,debilitated,diabetes,alcoholism,
COPD, malignancy, Gram-
bronchitis,
{ parahilarperibronchial lineardensities(bronchial
positivepneumonia,treatmentwith
wall thickening)
r/ interstitialpattern antibiotics,respiratortheraPY
{ hyperaeration+ air trapping(due to bronchial Organisms:
+ bronchiolarnarrowingfrom edema+ secretions) 1. K l ebsi el l a
{ segmental+ subsegmental atelectasis(common) 2. Pseudomonas
{ airspacepattern(fromhemorrhagicedema)in 50% 3. E . col i
{ pleuraleffusion(20%) 4. Proteus
{ hilaradenopathy(3%) 5. H aemoP hi l us
^/ strikingabsenceof pneumatoceles,lung abscess, 6. Legi onel l a
r/ airspaceconsolidation (Klebsiella)
pneumothorax
{ radiographic resolutionlags2-3 weeksbehindclinical { spongyappearance(Pseudomonas)
Cx: (1) Bacterialsuperinfection(childbecomestoxic r/ affectingdependentlobes (poorcough reflexwithout
after a week of sickness,peripheral clearingof bronchialtree)
^/ bilateral
consolidations + pleuraleffusion)
(2) Bronchiectasis r/ cavitationcommon
(3) Unilateralhyperlucent lung,bronchiolitis Cx: (1) Exudate/ emPYema
(2) Bronchopleural fistula
obliterans
 Differential Diagnosis of Chest Disorders 407

MycoticInfectionsof Lung 4. Atopicasthma

A. INHEALTHY SUBJECTS 5. Bronchiolitis
obliterans
1. His t op l a s m o s i s 6. Sinusitis
2. Coccidioidomycosis 7. Bronchiectasis
3. Blastomycosis 8. Ciliarydysmotility
syndromes
B . O P P O RT U N IS T IC IN F EC T IO N 9. Pulmonary foreignbody
1. A s per g i l l o s i s
2. Mucormycosis(phycomycosis) EOSINOPHILIC
LUNG DISEASE
3. Candidiasis = PULMONARY INFILTRATION WITHBLOOD / TISSUE
Growth: (a) mycelialform E OS TN OP H tLtA (P tE )
(b) yeastform (dependingon environment) Classification:
Source of contamination: 1 . I D I O P A T H IE
CO S I N O P H I L ILCU N GD I S E A S E
(a) soil (a) Transientpulmonaryeosinophilia = Ldffler
(b) growthin moistareas (apartfrom Coccidioides) syndrome
(c) contaminatedbird i bat excreta . peripheraleosinophilia
(b)A cute/ chroni ceosi nophi l ipneumonia
c
CavitatingPneumonia . no peripheraleosinophilia
1. Staphylococcusaureus 2 . E O S I N O P H I L ILCU N GD I S E A S EO F S P E C I F I C
2. Haem oph i l u s i n fl u e n z a e ETIOLOGY
3. S . pneumo n i a e (a) drug induced:nitrofurantoin, penicillin,
otherGram-negative organisms(eg, Klebsiella) sulfonamides,ASA, tricyclicantidepressants,
hydrochlorothiazide, cromolynsodium,
Cavitating Opportunistic Infections mephenesin
A. FUNGALINFECTIONS (b) parasiteinduced: tropicaleosinophilia
1. A s pe rg i l l o s i s (ascariasis, schistosomiasis), strongyloidiasis,
2. Nocardiosis ancylostomiasis (hookworm), filariasis,
Toxocara
3. Mucormycosis (= phycomycosis) canis(viscerallarvamigrans),Dirofilaria immitis,
B . S E P T I CE M BOL I amebiasis(occasionally - in RLL + RML)
1. Anaerobicorganisms (c) fungusinduced:allergicbronchopulmonary
C. STAPHYLOCOCCAL ABSCESS aspergil losis,bronchocentric granulomatosis
D. TUBERCULOSIS (nummularform) (d) Pulmonaryeosinophilia with asthma
0 Repeatedinfectionsin same patientare not 3. E OS IN OP H ILIC LU N GD IS E A S EA S S OCI ATED
necessarily due to same organism! W ITHA N GITISt GR A N U LOMA TOS IS
DDx: Metastaticdiseasein carcinoma/ Hodgkin (a) Wegenergranulomatosis
lymphoma (b) Churg-Strauss syndrome
(c) Lymphomatoid granulomatosis
PulmonaryInfiltratesin Neonate (d) Bronchocentric granulomatosis
mnemonic; "l HEAR" (e) Necrotizing sarcoidgranulomatosis
Infection(pneumonia) (f) Polyarteritisnodosa
Hemorrhage (g) Rheumatoid disease
Edema (h) Scferoderma
Aspiration (i) Dermatomyositis
Respiratorydistresssyndrome (j) Sjogrensyndrome
(k)cRESr
RecurrentPneumoniain Childhood
A . I M M U N EP R O B L E M CONGENITALPULMONARYMALFORMATION
1. lm m un ed e fi c i e n c y = SEQUESTRATION SPECTRUM
2. Chronicgranulomatous diseaseof childhood 1. Congenitallobaremphysema
(males) 2. Bronchogenic cyst
3. Alpha 1-antitrypsin deficiency 3. Cysticadenomatoidmalformation
B. ASPIRATION 4. Bronchopulmonary sequestration
1. Gastroesophageal reflux 5. Hypogenetic lungsyndrome
2. H-typetracheoesophageal fistula 6. Pulmonaryarteriovenous malformation
3. Disorderof swallowingmechanism
4. Esophagealobstruction,impactedesophageal NEONATAL LUNGDISEASE
foreignbody Parenchymal
Lung Diseaseon 1st Dayof Life
C. UNDE RLYIN G L U N GD IS EA SE 0 Radiographicfindingsoverlap!
1. Sequestration 1. Transienttachypneaof newborn
2. Bronchopulmonary dysplasia 2. Respiratorydistresssyndrome
3. Cysticfibrosis 3. N eonatalpneumoni a
408 Radiology Review Manual

4. Meconiumaspirationsyndrome ABNORMAL LUNG PATTERNS

5. Prematurewith acceleratedlung maturity(PALM) 1. Mass
= any localizeddensitynot completelyborderedby
Air Leaks in Neonatal Chest fissures/ pleura
1. Pulmonary interstitial
emphysema 2. Consolidative(alveolar)pattern
2. Pneumomediastinum = colT'tlT'tonlyproducedby fillingof air spaceswith fluid
3. Pneumothorax (transudate / exudate)i cells/ othermaterial,ALSO
4. Gasbelowvisceral pleura by alveolarcollapse,airwayobstruction, confluent
! gasat lungbase/ againstfissure interstitialthickening
5. Pneumopericardium groundglass = hazYarea of increasedattenuation
6. Gasembolus to cardiacchambers/ bloodvessels not obscuringbronchovascular
structures
MediastinalShift & Abnormal Aeration in consolidation= m?Ik€dincreasein attenuation with
of underlyinganatomic
obliteration
Neonate
LUCENT LUNG features
A. SHIFTTOWARD
1. Diaphragmatic hernia 3. Interstitialpattern
2. Chylothorax 4. Vascularpattern
3. Cysticadenomatoid malformation (a) increasedvesselsize: CHF, pulmonaryarterial
hypertension, shuntvascularity,lymphangitic
B. SHIFTAWAYFROMLUCENTLUNG
1. Congenitallobaremphysema carcinomatosis
localizedpulmonary interstitial (b) decreasedvesselsize: emphysema,
2. Persistent
emphysema thromboembolism
3. Obstructionof main-stembronchus (by 5. Bronchialpattern
{ wall thickening:bronchitis, asthma,bronchiectasis
anomalous or dilatedvessel/ cardiacchamber)
r/ densitywithoutair bronchogram(= comPleteainruay
ReticulogranularDensities in Neonate obstruction)
1. Respiratory distresssyndrome (90%):premature r/ lucencyof air trapping(= partialairwayobstruction
infant,inadequate surfactant with ball-valvemechanism)
2. Prematurity with accelerated lung maturity(PALM)
= IMMATURE LUNG SYNDROME: prematureinfant, DIFFUSE LUNGDISEASE ON HRCT
normalsurfactant (dueto maternal steroidtherapy Patternsof DiffuseLungDiseaseon HRCT
I intrauterine
stress) maximum = 300pm
resolution
{ lunggranularity, almostclear 1. Linear densities = sePtalthickening
{ smallthymus (stress/ steroids) Cause: interstitialfluid/ fibrosis/ cellularinfiltrates
3. Transient tachypnea of the newborn (a) smoothseptalthickening:pulmonaryedema,
4. Neonatal group-B streptococcal pneumonia lymphangitic carcinomatosis
5. ldiopathic hypoglycemia (b) beadedseptai septalnodules:lymphangitic
6. Congestive heartfailure carcinomatosis
7. Earlypulmonary hemorrhage (c) irregularseptaimplyfibrosis
8. Infantof diabeticmother - distortedlobules:fibrosis
- no architectural distortionof lobules:edema/
Hyperinflationin Newborn infiltration
beyond8th ribposteriorly
r/ levelof inflation 2. Reticular densities
r/ depressed of hemidiaphragms
configuration best (a) predominantly subpleuralsmallreticularelements
judgedon LATview of 6-10 mm in diameterwith smallcystic
1. Fetalaspiration syndrome changes("honeYcombing")
2. Neonatal pneumonia Associatedwith: interstitialfibrosis,
3. Pulmonary hemorrhage lymphangioleiomYomatosis,
4. Congenital heartdisease amyloidosis
5. Transient tachypnea(mild) (b) fine diffuselydistributednetworkof 2-3-mm basic
elements
Hyperintlationin Child Associatedwith: miliaryTB, reactionsto
mnemonic; "BUMPFAD" methotrexate
Bronchiectasis Distribution:
Upperairwayobstruction - lowerlungzonesin subpleuralareas:
Mucoviscidosis idiopathicpulmonaryfibrosis,collagenvascular
Pneumonia (esp.staph) disease,asbestosis
Foreignbody(ball-valve
mechanism) - mi d l ungzone/ al l l ungzones: chronic
Asthma extrinsicallergicalveolitis
Dehydration(diarrhea,
acidosis) mid + upperlungzones: sarcoidosis
 Differential Diagnosis of Chest Disorders 409

3. Nodules (b) withoutwell-defined

walls
(a) interstitial nodules panlobular(panacinar),
centrilobular,
lymphangitic carcinomatosis, sarcoidosis, paraseptalemphysema
histiocytosisX, silicosis,coal worker
pneumoconiosis, tuberculosis, hypersensitivity HRCTof Bronchiolitis
pneumonitis, metastatic tumor,amyloidosis [CTfindings arenonspecific andmustbe interpreted in the
{ perihilarperibronchovascular, centrilobular, appropriate clinicalcontextl
interlobular septa,subpleuralnodules Cause:
(b) airspacenodules (a) infectionvia endobronchial spread:
lobularpneumonia,transbronchial spreadof TB, bacterial(mostcommon)
bronchiolitis obliteransorganizingpneumonia mycobacterial: classicTB, nonclassicM.
(BOOP),pulmonaryedema avium-intracellulare ("LadyWindermere
r/ ill-defined nodules,afew mm to 1 cm in size syndrome"commonlyin BML + fingufa)
r/ peribronchiolar + centrilobular viral: acuteinfectiousbronchiolitisin infants
Distribution: and youngchildrendue to RSV,adenovirus,
- alongbronchoarterial bundles+ interlobular mycoplasma
septa+ subpleural:sarcoidosis parasitic
- upperzone: silicosis,coal-worker's fungal
pneumoconiosis 0 Mostcommoncauseof tree-in-budappearance
- centrilobular:extrinsicallergicalveolitis (b) aspirationof infectedoral secretions/ other irritant
4. Ground-glass attenuation material/inertbarium: mineraldust ainruay
= hazy increasein lung opacitywithoutobscuration disease,extrinsicallergicalveolitis, chronic
of underlyingvessels bronchitis
0 Often indicativeof an acute,active,and potentially (c) immunologic deficiencyor impairedhostdefense:
treatableprocess! cysticfibrosis,dyskineticciliasyndrome
Cause: (d) cigarettesmoking
(a) earlyinterstitial lungdisease= minimalalveolar 1. Respiratory bronchiolitis
wall thickening (e) idiopathic
(b) alveolitis= minimalairspacefilling 1. Diffusepanbronchiolitis in Orientals
r/ areas of higherattenuationwith nodular/ 2. Follicularbronchiolitis:rheumatoidarthritis,
centrilobu lar distribution Sjdgrensyndrome
r/ pulmonaryvesselsuniformin size in areasof 3. Asthma
differingattenuation 4. Bronchiolitis obliterans
r/ increasein lungattenuation in low-and high- 5. Bronchiolitis obliteranswith organizing
attenuationareas on expiratoryHRCT pneumoni a
(c) edema= increasedcapillarybloodvolume
(d) partialatelectasis= partialcollapseof alveoli
(e) normalduringexpiration
Distribution:
- per iph e rai nl l o w e rl u n gz o n e s : D l p , Ul p
- mid + upperlungzones: sarcoidosis
- 3'9747y paving"appearance:alveolar Normal Bronchiole
proteinosis (<1 mm,not visibleon CT)
- mosaicperfusion:chronicthromboembolism,
bronchiolitisobliterans
5. Consolidation
= increasein lung opacitywith obscurationof
underlyingvessels+ air bronchograms
- subpleuralin mid + upperlungzones: chronic
eos inop h i l ipcn e u mo n i a ThickenedBronchiolar Dilated Bronchiole (>2 mm)
- subpleural+ peribronchial:BOOp Wall
- focal: bronchioloalveolar cell carcinoma.
lymphoma
- random: infection
6. Cystic airspaces
= circumscribed air-containing lesions
(a) with well-defined walls 66Tree-in'budt'Appearance
Bronchiolar
lar Impaction
Impaction
lymphangioleiomyomatosis, pulmonary = bronchiolar impaction +
Langerhans-cell granulomatosis, honeycomb clubbins
lung,bronchiectasis
Patternof BronchiolarDisease
410 Radiology Review Manual

A . DI RE CTS I G N S Cause:
{ ringliketubularstructuresin lung periphery 1. A i r traP P i ng
Cause: wall thickening r/ attenuationdifferencesare accentuatedon
r/ dilatationof bronchioles expiratoryHRCT
Cause: bronchiolectasis 2. Vascularobstruction
r/ Z- to 4-mm nodules/ branchinglinearstructuresin r/ increasein lungattenuation in low-and high-
lungperiphery attenuationareas on expiratoryHRCT
Cause: bronchiolar luminalimpactionwith pus,
mucus, granulomas, inflammatorY
exudate,fibrosis ALVEOLAR(CONSOLIDATIVE) PATTERN
B. INDIRECT SIGNS Classicappearanceof airspaceconsolidation:
"A2BCa"
{ subsegmentalatelectasis= wedge-shapedarea of mnemonic.'
ground-glass attenuation ^/ Acinarrosettes: roundedpoorlydefinednodulesin
{ air trapping= 41s4of decreasedattenuationfrom sizeof acini(6-10 mm), bestseen
collateralair drift/ ball-valveeffectdistalto at peripheryof oPacitY
occluded/ stenotic airway more prominent on ./ Rir alveologram / bronchogram
expiration: ^/ Buttertty/ bat-wingdistribution:perihilari bibasilar
DDx: physiologic air trappingwith a few lucent { Coalescent/ confluentcloudlikeill-defined opacities
secondarypulmonarylobules r/ Consolidation in diffuse,perihilar/ bibasilar,
^/ mosaic perfusion= scatteredareas of air segmental/ lobar,multifocal/ lobutardistribution
trapping r/ Changesoccurrapidly(labilei fleeting)
ri centrilobular emphysema= destructionof small H R C T:
airways+ surrounding parenchymain the center { poorlymarginateddensitieswithinprimarylobule(up
of the pulmonarylobule to 1 cm in size)
r/ centrilobular airspacenodule= acinarnodule r/ rapidcoalescence with neighboring lesionsin
- <1 cm ilt-definednoduleof ground-glass segmentaldistribution
attenuation(frominflammation withinalveolar { predominantly centrallocationwith sparingof
space)lessprominenton expiration subpleuralzones
Caus e: e x tri n s i c a l l e rg i c a l v e o l i ti s ,sarcoi dosi s { air bronchograms
(perivenular nodules),pneumoconiosis
(asbestosis, silicosis) Diffuse Airspace Disease
A. INFLAMMATORY EXUDATE= "PUS"
DDx: (1) Cysticlungdisease(thinseptumsurrounds 1. Lobarpneumoni a
area of air attenuation,centralvesselnot 2. Bronchopneumonia: especiallyGram-negative
present) organisms
(2) Panlobularemphysema(distortion of vascular 3. U nusualpneumoni as
+ septalarchitecture,bullae) (a) viral: extensivehemorrhagic edema
especiallyin immunocompromised patients
with hematologic malignancies + transplants
Tree-i n-bud appearance (b) Pneumocystis
= slndllpoorlydefinedcentrilobular nodules (c) fungal: Aspergillus, Candida,Cryptococcus,
+ branchingcentrilobularareasof increasedopacity Phycomycetes
occurringat multiplecontiguousbranchingsites (d) tuberculosis
Histo: severebronchiolarimpactionwith clubbing 4. Aspiration
of distalbronchioles analogousto "finger-in- B . H E MOR R H A GE = " B LOOD "
glove"appearance 1. Trauma: contusi on
1. Endobronchial spreadof activeTB 2. Pulmonaryembolism,thromboembolism
2. Viral,fungal,parasiticinfection 3. B l eedi ngdi athesi s:l eukemi a, hemophilia,
3. Allergicbronchopulmonary aspergillosis anticoagulants, DIC
4. Cysticfibrosis 4. Vasculitis:Wegenergranulomatosis,
5. Aspirationpneumonitis Goodpasture syndrome,SLE, mucormycosis,
6. Laryngotracheal papillomatosis aspergillosis, RockyMountainspottedfever,
infectiousmononucleosis
5. ldiopathicpulmonaryhemosiderosis
Mosaic Perfusion 6. Bleedingmetastases:choriocarcinoma
"WATER"
= patchworkof normaland air-attenuated segments c. TRANSUDATE=
./ vesselsin areas of low attenuationare smallerin 1. C ardi acedema
94% (due to differentialbloodflow) 2. Neurogenicedema
r/ normal / dilatedarteriesin areas of 3. Hypoproteinemia
hyperattenuation in 77"/. 4. Fluidoverload
 Differential
Diagnosisof ChestDisorders 411

5. Renalfailure LocalizedAirspaceDisease
6. Radiotherapy mnemonic.' "4P's& TAIL"
7. Shock Pneumonia
8. Toxicinhalation Pulmonaryedema
9. Drug reaction Pulmonarycontusion
10. Adult respiratorydistresssyndrome Pulmonaryinterstitial
edema
D. S E CRE T ION S = " PR O T E IN " Tuberculosis
1. Alveolarproteinosis Alveolarcell carcinoma
2. M uc usp l u g g i n g Infant
E. MALIGNANCY= "CELLS'' Lymphoma
1. Bronchioloalveolar cell carcinoma
2. Lymphoma
F. INTERSTITIAL DISEASEsimutatingairspace Acute Alveolarlnfiltrate
disease,eg, "alveolarsarcoid" mnemonic: "l 2 CHANGEFAST"
mnemonic: "AIRSPACED" Infarct
Aspiration Infection
Inhalation,Inflammatory Contusion
Renal(uremia) Hemorrhage
Sarcoidosis Aspiration
Proteinosis(alveolar) Neardrowning
Alveolarcell carcinoma Goodpasturesyndrome
Congestive(CHF) Edema
Emboli Fungus
Drug reaction,Drowning Allergicsensitivity
Shocklung
Tuberculosis
DiffusePulmonaryHemorrhage ChronicAlveolarInfiltrate
{ nonspecificdiffuse/ bilateralmultifocal
air-space mnemonic; "STALLAG"
opacities Sarcoidosis
1. Wegener granulomatosis: c-ANCA, upper Tuberculosis
respiratorytractinvolvement, renaldisease Alveolarcell carcinoma
2. Churg-Strauss syndrome Lymphoma
3. Systemic necrotizing vasculitis Li poi dpneumoni a
4. Collagen-vascular disease Alveolarproteinosis
(a)Systemic lupuserythematosus: granularpattern Goodpasturesyndrome
of immunecomplexes on tissuestains,
noncaseating granulomas, malarrash
(b) Rheumatoid arthritis CT AngiogramSign
(c) Seronegative juvenilerheumatoid arthritis = homogeneous low attenuationof lungconsolidation,
5. Goodpasture syndrome: antibasement membrane which allowsvesselsto be clearlyseen
antibodieswitha linearpatternon tissuestains 1. Lobarbronchioloalveolar cell carcinoma
6. lmmunoglobulin A nephropathy 2. Lobarpneumonia
7. Schdnlein-Henoch purpura 3. Pulmonarylymphoma
8. ldiopathicpulmonary hemorrhage 4. Extrinsiclipidpneumonia
9. ldiopathicglomerulonephritis 5. Pulmonaryinfarction
6. Pulmonaryedema

Air-space Opacification in Trauma

A. ACUTEPHASE INTERSTITIAL LUNG DISEASE
1. Pulmonarycontusion= hemorrhage intoalveoli = thickeningof lunginterstices
(= interlobular
septa)
2. Pulmonary = tearin lungparenchyma
laceration A. MAJORLYMPHATICTRUNKS
(a)sphericalhematofftd= filledwithblood 1. Lymphangiticcarcinomatosis
(b)traumaticpneumatocele = filledwithair 2. Congenitalpulmonarylymphangiectasia
pneumonia
3. Aspiration 3. Pulmonaryedema
4. Atelectasis
dueto splinting/ mucousplug 4. Alveolarproteinosis
5. Pulmonaryedema:cardiogenic / noncardiogenic B. PULMONARYVEINS(increasedpulmonaryvenous
B. SUBACUTE PHASE(>24hours)add pressure)
1. Fatembolism 1. Leftventricularfailure
2. Adultrespiratory
distresssyndrome 2. Venousobstructivedisease
412 RadiologyReviewManual

T ISS U EN E T WOR K
C O N N E C T IV E
C . S UP P O RT I N G septal
lungdiseasewith interlobular
F. lnterstitial
1. Interstitialedema thickening
2. Chronicinterstitial pneumonia 1. Lymphangi ti ccarci nomatosi s
3. Pneumoconioses pulmonarY
2. Interstitial edema
4. Collagen-vascular disease 3. Alveolarproteinosis
5. Interstitialfibrosis G. Eosinophilic syndrome
6. Amyloid H. Pulmonaryhemorrhage
7. Tumor infiltrationwithinconnectivetissue l. Vasculitis
8. Desmoplastic reactionto tumor
InterstitialLung Patternon CXR
Path: stereotypicalinflammatoryresponseof alveolar 1. LIN E A RP A TTE R N
wall to injury (a) Kerleylines= septallines
(a) acute phase: fluid + inflammatorycells exude into = thickenedconnectivesepta
alveolarspace,mononuclear cellsaccumulatein Path: accumulation of fluid/ tissue
edematousalveolarwall r/ KerleyA lines= relativelylong fine linear
(b) organizingphase: hyperplasia of type ll shadowsin upperlungs,deep withinlung
pneumocytesattemptto regeneratealveolar parenchymaradiatingfrom hila
epithelium, fibroblastsdepositcollagen ./ KerleyB lines= short horizontallyoriented
(c) chronicstage: densecollagenous fibroustissue peripherallinesextending+ perpendicularto
remodelsnormalpulmonaryarchitecture pleurain costophrenicangles+ retrosternal
Characteriz ing criteria : clear space
(a) zonaldistribution: (b) reticulations
upper/ lowerlungzones = innumerable interlacing linearopacities
axial (core)/ parenchymal(middle)/ peripheral suggestinga mesh/ network
"spiderweb / lacelike"
(b) volumeloss r/ KerleyC lines= fine
(c) time course polygonalopacitiesdistributedprimarilyin a
(d) interstitiallung pattern peripheral/ subpleurallocation
Path: pulmonaryfibrosis(lowerlobes),
CIassification scheme: hypersensitivitypneumonitis(upper
A. Interstitialpneumonias lobes)
p ln e u mo n i a(U l P)
1. Us ualin te rs ti ti a linearopacitiesin a central/ perihilar
ri ttrict<
2. Nonspecific interstitial pneumonia(NSIP) distribution
3. Acuteinterstitial pneumonia(AlP) Path: (a) dilatedthick-walledbronchiof
4. Alveolarmacrophagepneumonia(AMP) bronchiectasis, (b) cysts of lymphangio-
= desquamative interstitial pneumonia(DlP) leiomyomatosis I tuberoussclerosis
5. Bronchiolitis obliteransorganizingpneumonia 2 . N OD U LA R/ MILIA R YP A TTE R N
B. Diffuseinfiltrativediseasewith granulomas = srTt?ll well-defined innumerable uniform3-5-mm
1. Sarcoidosis noduleswith evendistribution
2. Hypersensitivity pneumonitis Path: diffusemetastaticdisease,infectious
c. Lymphocyticinterstitialpneumonia(LlP) granulomatous disease(TB,fungal),
D . Pneumoconioses noninfectiousgranulomatous disease
E , Interstitiallung diseasewith cysts (pneumoconioses, sarcoidosis, eosinophilic
1. Langerhanscell histiocytosis granuloma)
2. Lymphangioleiomyomatosis 3. DESTRUCTIVEFORM= honeycomblung

of CollagenVascularDisease
ThoracicManifestations
Ankylosing Dermatomyositis Progressive Rheumatoid Siogren SystemicLupus
Spondylitis Polymyositis Systemic Arthritis Syndrome Erythematosus
Sclerosis (sLE)

Pulmonaryfibrosis occasional common frequent frequent occasional occasional

Pleuraldisease frequent frequent
Diaphragmweakness frequent frequent
Aspirationpneumonia frequent frequent
Bronchiectasis occasional common
Apicalfibrosis treq*nt
Bronchiolitis
obliterans aorr*on
BOOP ao*on common
 DifferentialDiagnosisof ChestDisorders 413

Signsof Acute InterstitialDisease (b) Chronicslowlyprogressivewith volume loss

r/ peribronchialcuffing= thickenedbronchialwafl 1. Sarcoidosis(common)
, + peribronchialsheath(whenviewedend on) 2. Ankylosingspondylitis(rare)
r/ thickeningof interlobular
fissures 3. Sulfadrugs(rare)
r/ Kerleylines (c) Chronicslowlyprogressive withoutvolumeloss
r/ perihilarhaze= blurringof hilarshadows 1. Extrinsicallergicalveolitis
r/ blurringof pulmonaryvascularmarkings 2. E osi nophi l granul
ic oma
{ increaseddensityat lung bases 3. Aspirationpneumonia
r/ smallpleuraleffusions 4. Postradiation pneumonitis
5. RecurrentPneumocystis cariniipneumonia
Srgnsof Chronic lnterstitial Disease (PCP)in a patientreceivingaerosolized
r/ irregularvisceralpleuralsurface pentamidine prophylaxis
{ reticulations: mnemonic.' "SHIRT CAP"
r/ tine reticulations= e?rly potentiallyreversible/ Sarcoidosis
minimalirreversible alveolarseptalabnormality Histoplasmosis
(1) idiopathicpulmonaryfibrosis(basilar ldiopathic
predominance) Radiationtherapy
./ coarsereticulations Tuberculosis(postprimary)
in75/" relatedto environmental disease, Chronicextrinsicalveolitis
sarcoidosis,collagen-vascular disorders, Ankylosingspondylitis
chronicinterstitialpneumonia Progressivemassivefibrosis
i/ nodularity:
in 90% relatedto infectious / noninfectious
granulomatous process,metastaticmalignancy, Chronic Diffuse Infiltrative Lung Disease
= CHRONIC INTERSTITIAL LUNGDISEASE
, pneumoconioses, amyloidosis
= GENERALIZED
! linearity: INTERSTITIAL LUNGDISEASE
(1) cardiogenic / noncardiogenic interstitial Prevalence: up to 15/" of pulmonaryconditions
pulmonaryedema Cause: >200 describeddisorders;
r/ symmetriclinearity in only 25-30% known/ establishedetiology;
(2) lymphangitic malignancy 15-20 diseasescomprise>90/" of cases
./ asymmetriclinearity . dyspnea(primarycomplaint)
. dry basilarrales/ cracklesthat fail to clearwith
(3) diffusebronchialwall disorders(cysticfibrosis,
bronchiectasis, hypersensitivity asthma) coughi ng
r/ honeycombing= usuallysubpleuralclustered CXR:
cysticair spaces<1 cm in diameterwiththickwell- 0 Difficultto characterizedue to similarfindings
definedwalls set off againsta backgroundof 0 Differentiation into alveolar+ interstitialdiseaseis
increasedlungdensity(end-stagelung) unreliableas "interstitialdisease"invariablyinvolves
0 HRCT approximatelyO0%more sensitivethan CXFI alveoli+ vice versa
r/ + nonspecificabnormality
Distributionof InterstitialDisease mnemonic; "HIDE FACTS"
A . M I DLUNG/ PE R IH IL AR Hamman-Rich, Hemosiderosis
D ISE AS E
(a) Acute rapidlychanging Infection,lrradiation,ldiopathic
1. P ulm o n a ry Dust,Drugs
edema
2. Pneumocystis Eosinophilic granuloma,Edema
pneumonitis
3. Earlyextrinsicallergicalveolitis Fungal,Farmer'slung
(b) Chronicslowlyprogressive Aspiration(oil),Arthritis(rheumatoid,ankylosing
1. Lymphangitic spondylitis)
carcinomatosis
often unilateral,associatedwith adenopathy, Collagenvasculardisease
pleuraleffusion Tumor,TB, Tuberoussclerosis
B . P E RI P HE R AL
L U N GD IS EA SE Sarcoidosis,Scleroderma
(a) Acuterapidlychanging
1. Interstitial
pulmonaryedemawith KerleyB Zonal Predilection of Chronic Diffuse Lung
lines(mostcommon) Disease
2. Activefibrosingalveolitis GnnourcDlrruselrurtrRalveLurucDlsenseor
(b) Chronicslowlyprogressive UppenLuncZoHe
1. Secondarypulmonaryhemosiderosis = ZoflewithhigheroxygentensionandpH,
C. UP P E RLUN GD ISE AS E butlessefficient
lymphatic
drainage
(a) Chronicslowlyprogressive+ volume loss (a)inhalational
disease
1. Postprimary TB (common) 1. Silicosis
2. Silicosis(common) 2. Coalworkerpneumoconiosis
414 RadiologyReview Manual

3. Extrinsicallergicalveolitis 2. Lymphangitic carcinomatosis

4. Aspirationpneumonia 3, Chronicmedications
(b) granulomatousdisease 4. Neurofibromatosis
1. Sarcoidosis 5. Vasculitis
2. Langerhanscell histiocytosis(EG) 6. Silicosis
3. Postprimary TB (common) C . P E R IP H E R AC
L OMP A R TME N T
(c) others = pleurawith subpleuralconnectivetissue,
1. Cysticfibrosis interlobular septa,pulmonaryveins,lymphatics,
2. Ankylosingspondylitis walls of corticalalveoli
3. Chronicinterstitialpneumonia 1. Sarcoidosis
4. Sulfadrugs(rare) 2. Lymphangitic carcinomatosis
5. Postradiation pneumonitis 3. ldiopathicpulmonaryfibrosis
6. RecurrentPneumocystis cariniipneumonia 4. Collagenvasculardisease
(PCP) in a patientreceivingaerosolized 5. Rheumatoidarthritis
pentamidine prophylaxis
mnemonic: "CASSET"
Cysticfibrosis
Lung Volumes in Chronic Diffuse Lung
Ankylosingspondylitis
Silicosis Disease
Sarcoidosis LuHcDlsease
CnnoucDtrruselrurllrnalve wrn
Eosinophilic granuloma NonuelLuttcVolume
Tuberculosis, fungus 1. Sarcoidosis
2. Langerhanscell histiocytosis(in 66%)
3. Earlystage
Cnnonrc Drrruse Innltnalve LuHc Dtselse or Cnnouc Dtrruse lrurllrnltlve Lunc Dlseasewrn
Lowen LuHc Zorue lrucnraseoLurucVolun,te
= Zon€with greaterventilation, perfusion,and mnemanic; "ELECT"
lymphaticdrainage Emphysemawith interstitiallungdisease
1. ldiopathicpulmonaryfibrosis:usualinterstitial Lymphang ioleiomyomatosis
pneumonia(common) Eosinophilic granuloma(Langerhans) in 33%
2 . Lymphangiticcarcinomatosis Cysticfibrosis
3 . Collagenvasculardisease:scleroderma Tuberoussclerosis
(common) Cnnouc Dlrruse lrunlrnnrlve LuHc Dtselse wrn
4 . Asbestosis(posterioraspectof lung base)
Reouceo Luttc Volunne
5 . Lymphangioleiomyomatosi s
due to fibroticprocess
6 . Chronicaspirationpneumoniawith fibrosis
1. Systemiclupuserythematosus
(oftenregional+ unilateral)
2. Collagenvasculardisease(eg,scleroderma,
mnemonic: "BAD LASS RlF" polymyositis)
dermatomyositis,
Bronchiectasis
3. ldiopathicpulmonaryfibrosis
Aspiration pneumonias
4. Chronicinterstitial
Dermatomyositis
5. Asbestosis
Lymphangiticspread
Asbestosis
Sarcoidosis Pleural Diseasein Chronic Diffuse Lung
Scleroderma
Disease
Rheumatoidarthritis
CnnonrcDtrruselrurtlrnlrlve wtrn
LurucDlsense
ldiopathicpulmonaryfibrosis
Furadantin PneuuornoRAx
1. Lymphangioleiomyomatosis
2. Langerhanscell histiocytosis
CompartmentalPredilection of Chronic Diffuse 3. End-stagelungdisease
Lung Disease Cnnouc Dtrruse lmrtlrnnrlve LuHc Dlseasewrn
A. AXIALCOMPARTMENT Pleunnl Errustott
= peribronchial
vascularbundles+ lymphatics 1. Lymphangioleiomyomatosis
1. Sarcoidosis 2. Rheumatoidarthritis
2. Lymphangitic carcinomatosis 3. Systemiclupuserythematosus
3. Lymphoma 4. Mixedconnectivetissuedisorder
B . M I DDLE/ P AR EN C H YMAC
L OMP AR TME N T 5. Wegenergranulomatosis
= formedby alveolarwalls 6. Lymphangiticcarcinomatosis
1. Sarcoidosis 7. Pulmonaryedema
 DifferentialDiagnosisof ChestDisorders 4l s

Cxnouc Dlrruse lrurrrrRlnveLurucDrseasewlrn E . C ON N E C TIV E -TIS S UDEIS E A S E

Pleunnl Tnrcrerulruc reticulationsin fate stages
1. Asbestosis 1. R heumatoildung
2. Collagenvasculardisease 2. Scleroderma
3, Systemiclupuserythematosus
Lymphadenopathy in Chronic Diffuse Lung F. DRUG REACTIONS
Disease G. ID IOP A TH IC
1. S ilic os i s 1. Usualinterstitial pneumonitis(UlP)
2. Sarcoidosis 2. Desquamative pneumonitis(DlP)
interstitial
3. Lymphoma 3. Tuberoussclerosis:smoothmuscle
4. Lymphangitic carcinomatosis proliferation
4. Lymphangiomyomatosis
5. ldiopathicpulmonaryhemosiderosis
Diffuse Fine Reticulations 6. Alveolarproteinosis(fatecomplication)
Acute Diffuse Fine Reticulations 7. Amyloidosis
A . A CUT EI N T E R S T IT IAL ED EMA 8. Interstitial (chronicrenalfailure)
calcification
1. Congestiveheartfailure mnemonic; "LIFE lines"
2. Fluidoverload Lymphangitic spread
3. Ur em i a Inflammation / infection
4. Hypersensitivity Fibrosis
B . A CUT EIN T E R S T IT IAL P N E U MON IA Edema
1. Viralpneumonia(Hantavirus, CMV)
2. M y c o p l a s m a p n e u m o n i a CoarseReticulations
3. Pneumocystis cariniipneumonia = architecturaldestructionof interstitium= end-stage
mnemonic.' "HELP" scarringof lung= interstitial pulmonaryfibrosis
Hypersensitivity = honeycomb lung
Edema ^/ coarsereticularinterstitialdensitieswith intervening
Lymphoproliferative cysticspaces
Pneumonitis (viral) r/ roundedradiolucencies <1 cm in areasof increased
lungdensity
Chronic Diffuse Fine Reticutations r/ smalllungvolume(decreasedcompliance)
A. VENOUSOBSTRUCTION Cx: (1) Intercurrentpneumothoraces
1. Atherosclerotic heartdisease (2) Bronchogeniccarcinoma= sc€lfcarcinoma
2. Mitralstenosis Cause:
3. Left atrialmyxoma A. INHALATIONAL DISEASE
4. Pulmonaryvenoocclusive disease (a) Pneumoconioses
5. Sclerosingmediastinitis 1. Asbestosis:basilardistribution, shaggy
B. LYMPHATICOBSTRUCTION heart,pleuralthickening+ calcifications
1. Lymphangiectasia (pediatricpatient) 2. Silicosis:upperlobe predominance,
2. Mediastinal mass (lymphoma) + pl eural thi ckeni ng,
+ hi l arand m ediast inal
3. Lymphoma/ leukemia lymphadenopathy
4. Lymphangitic carcinomatosis: 3. Berylliosis
predominantly basilar distribution (b) Chemicalinhalation(late)
(a) bilateral(breast,stomach,colon,pancreas) 1. Silo-filler's
disease(nitrogendioxide)
(b) unilateral(lungtumor) 2. Sulfurdioxide,chlorine,phosgene,cadmium
5. Lymphocytic pneumonitis
interstitial (c) Extrinsicallergicalveolitis
C. INHALATIONAL DISEASE (= hypersensitivityto organicdusts)
1. Silicosis:smallnodules+ reticulations (d) Oxygentoxicity
2. Asbestosis:basilardistribution, pleural sequelaeof RDS therapywith oxygen
thickening+ calcifications (e) Chronicaspiration
3. Hard metals eg, mineraloil: localizedprocessin medial
4. Allergicalveolitis basalsegments/ middlelobe
D. GRANULOMATOUS DISEASE B . GR A N U LOMA TOUDSIS E A S E
from a nodularto a reticularpatternif 1. S arcoi dosi s
(a) nodulesline up alongbronchovascular 2. E osi nophi l granul
ic oma
bundles C. COLLAGEN-VASCULAR DISEASE
(b) interlobularseptashowfibroticchanges 1. R heumatoildung
1. Sarcoidosis:hilar+ mediastinal adenopathy 2. Scleroderma
(may have disappeared) 3. Ankylosingspondylitis:upperlobes
2. Eosinophilic granuloma:upperlobedistribution 4. SLE: rarelyproduceshoneycombing
416 RadiologyReview Manual

D. I A T RO G E N IC C . C E LLU LA RFILLIN GOF A IR S P A C E

1. Drug hypersensitivity 1. Desquamative pneumonia
interstitial
2. Radiotherapy 2. Pneumocystis cariniipneumonia
E . I DI O P AT H IC
1. Usualinterstitialpneumonitis(UlP)
honeycombingin 50/", severevolume loss in
45o/" Lung Disease
Reticulonodular
2. Desquamative pneumonitis(DlP)
interstitial mnemonic.' "PleaseDon't Eat StaleTuna Fish
honeycombing in 12.5/0,severevolumeloss in SandwichesEverYMorning"
23/ o Pneumoconiosis
3. Lymphangiomyomatosis Drugs
4. Tuberoussclerosis(rare) E osi nophi l granul
ic oma
5. Neurofibromatosis (rare) Sarcoidosis
6. Pulmonarycapillaryhemangiomatosis (rare) Tuberculosis
DDx: bronchiectasis, cavitarymetastases(rare) Fungaldisease
Schistosomiasis
Reticulations& Pleural Effusion Exanthem(measles,chickenPox)
A. ACUTE Metastases(thyroid)
1. Edema
2. Infection:viral, Mycoplasma(veryrare)
B . CHRO N IC BeticulonodularPattern& Lower Lobe
1. Congestiveheartfailure Predominance
2. Lymphangiticcarcinomatosis mnemonic.' "ClA"
3. Lymphoma/ leukemia Collagenvasculardisease
4. S LE ldiopathic
5. Rheumatoiddisease Asbestosis
6. Lymphangiectasia
7. Lymphangiomyomatosis
8. Asbestosis
NodutarLung Disease
Reticulations& Hilar AdenopathY = rouodmoderatelywell marginatedopacity<3 cm in
1. Sarcoidosis maximumdiameter
2. Silicosis A . GR A N U LOMA TOULU S N GD IS E A S E
Lymphoma/ leukemia (a) infections:eg, tuberculosis
3.
4. Lung primary: particularlyoat cell carcinoma (b) fungaldisease:eg, histoplasmosis
Metastases:lymphaticobstruction/ spread (c) silicosis
5.
6. Fungaldisease (d) vasculitis:eg, Wegenergranulomatosis
7. Tuberculosis B. NEOPLASM
8. Viralpneumonia(rarecombination) (a) metastaticlung diseases: eg, thyroidcancer
(b) lymphoma
(c) bronchioloalveolarcell carcinoma
ChronicInterstitialDiseaseSimulatingAirspace C . OTH E RD IS E A S E
Disease (a) drug-induced:methotrexate
A . RE P LA CE M ENO T F L U N GA R C H IT E C TU RB
EY (b) nongranulomatous vasculitis
AN INTERSTITIAL PROCESS (c) sarcoidosis
(a) neoplastic
Hodgkindisease,histiocytic lymphoma
(b) benigncellularinfiltrate MacronodularLung Disease
lymphocyticinterstitialpneumonia, { nodules>5 mm in diameter
pseudolymphoma mnemonic; "GAMMAWARPS"
(c) granulomatousdisease granuloma,fungus)
Granuloma(eosinophilic
alveolarsarcoidosis Abscess
(d) fibrosis Metastases
B. EXUDATIVEPHASEOF INTERSTITIAL Multiplemyeloma
P NE UM ON IA AVM
1. UIP Wegenerg ranulomatosis
2. Adult respiratorydistresssyndrome Amyloidosis
3. Radia ti o n p n e u mo n i ti s Rheumatoidlung
4. Drug reaction paragonimiasis)
Parasites(Echinococcus,
5, Reactionto noxiousgases Sarcoidosis
 DifferentialDiagnosisof ChestDisorders 417

FrrueNooumR DrseRserNFeenrlr Pnrrrrur

= discrete3-5-7-mm small roundfocal opacityof at 1. Tuberculosis
leastsoft-tissueattenuation 2. Fungalinfection(earlystage)
1. Granulomatous disease(miliarytuberculosis, 3. Pneumocystis
histoplasmosis) 4. Viralpneumonia
2. Hypersensitivity(organicdust)
3. Pneumoconiosis (inorganic
dust,thesaurosis End-stage Lung Disease
= prolongedhair spray exposure) = evidenceof honeycombing/ cysticchange/
4. Sarcoidosis conglomeratefibrosis
5. Metastases(thyroid,melanoma) A . D IS TR IB U TION
6. HistiocytosisX 1. Usualinterstitial pneumonia
7. Chickenpox ^/ subpleuraldistribution + lowerlobe
predominance
2. Asbestosis
r/ subpleuraldistribution+ lower lobe
Drrrusr FrueNooumn Dlsrnse& MlunRvNooules predominance + pleuralthickening
{ very small1-4-mm sharplydefinednodulesof 3. Sarcoidosis
interstitialdisease r/ subpleuralhoneycombing
(a) Inhalational disease r/ centralcysticbronchiectasis
1. Silicosis+ coalworker'spneumoconiosis r/ conglomeratefibrosis
2. Berylliosis { peribronchovascular distribution
3. Siderosis { upperlobepredominance
4. Extrinsicallergicalveolitis(chronicphase) 4. Extrinsicallergicalveolitis
(b) Granulomatous disease rl diffuserandomdistribution+ patchyareasof
1. E os i n o p h i l g
i cra n u l o ma ground-glassattenuation
2. Sarcoidosis(withcurrent/ previous B. CYSTICSPACESWITHWELL-DEFINED WALLS
adenopathy) 1. Langerhanscell histiocytosis
(c) Infectiousdisease r/ upperlobe predominance
1. B acte ri a : s a l m o n e l l a ,n o c a rd i o s i s 2. Lymphangioleiomyomatosis
2. Tuberculosis ./ no zonal predominance
3. Fungus: histoplasmosis,coccidioidomycosis, C. CONGLOMERATE FIBROTICMASSES
blastomycosis, aspergillosis (rare), 1. Sarcoidosis
cryptococcosis(rare) r/ peribronchovascu lar distribution
4. Virus: varicella(morecommonin 2. Silicosis
adults),Mycoplasmapneumonia r/ bronchisplayedaroundmasses
(d) Metastases 3. Talcosis
Thyroidcarcinoma,melanoma,adenocarcinoma r/ areas of high attenuation(= talc deposits)
of breast,stomach,colon,pancreas
(e) Alveolarmicrolithiasis (rare) HoneycombLung
(f) Bronchiolitis obliterans mnemonic;"SHIPS BOATS"
(g) Gaucherdisease
"TEMPEST" Sarcoidosis
mnemonic;
HistiocytosisX
Tuberculosis + fungaldisease ldiopathic(UlP)
Eosinophilic granuloma
Pneumoconiosis
Metastases(thyroid,lymphangitic
Scleroderma
carcinomatosis) Bleomycin,Busulfan
Pneumoconiosis, Parasites Oxygentoxicity
Embolismof oily contrast Arthritis(rheumatoid),
Sarcoidosis Amyloidosis, Allergicalveolitis
Tuberoussclerosis
Tuberoussclerosis,TB
Storagedisease(Gaucher)

FrrueNoouun DrsenserNAreente PRrrrrur PULMONARY NODULE/ MASS

1. lnhalational
disease SolitaryNodule/ Mass
2. Eosinophilicgranuloma Definition: any pulmonarylpleural sharplydefined
3. Sarcoidosis discretenearlycircularopacity
4. Metastases 2-30 mm in diameter = nodule
5. Fungalinfection(latestage) >30 mm in diameter = mass (>90%prevalence
6. Miliarytuberculosis
(rare) of malignancy)
418 Radiology Review Manual

lncidence; 150,000annuallyin USA E . D E V E LOP ME N TA/ LC ON GE N ITA L

(a) roentgenographicsurveyof low-riskpopulation: 1. Bronchogenic cyst (fluid-filled)
<5/" of nodulesare cancerous 2. Pulmonarysequestration
(b) on surgicalresection:40% malignanttumors, 3. Bronchialatresia
40% granulomas F. INHALATIONAL
1. Silicosis(conglomerate mass)
A . I NF LA M M AT ION / IN F E C T ION 2. Mucoidimpaction(allergicaspergillosis)
(a) infectious G. MTMTC K TN DG E N S ITIE S(20% )
1. G r a n u l o m a(m o s tc o m m o nl u n gm a ss): (a) Pseudotumor
sarcoidosis(1i3), tuberculosis, 1. Fl ui di n fi ssure
histoplasmosis, coccidioidomycosis, 2. Compositearea of increasedopacity
nocardiosis,cryptococcosis, talc, Dirofilaria (b) Mediastinalmass
immitis(dog heartworm), gumma,atypical (c) Chestwall lesion
measlesinfection 1. Nipple
2. Fluid-filledcavity: abscess,hydatidcyst, 2. Skintumor: mole,neurofibroma, lipoma,keloid
bronchiectatic cyst, bronchocele 3. Boneisland,rib osteochondroma
3. Mass in preformedcavity: fungusball, 4. Rib fracture/ osteoPhYte
mucoidimpaction 5. Pleuralplaque/ mass (mesothelioma)
4. Roundedatelectasis (d) Externalobject
5. Inflammatory pseudotumor:fibroxanthoma, 1. Electrocardiographic leadattachment
histiocytoma, plasmacell granuloma, 2. Buttons,snaps
sclerosinghemangioma
6. Paraffinoms= lipoidgranuloma mnemonic; "Big SolitaryPulmonaryMasses
7. Focalorganizingpneumonia CommonlyAppearHopelessAnd Lonely"
8. Rou n dp n e u mo n i a Bronchogen ic carcinoma
(b) noninfectious Solitarymetastasis,Sequestration
1. Rheumatoidarthritis Pseudotumor
2. Wegenergranulomatosis Mesothelioma
B. MALTGNANT TUMORS(30-40%) Gyst(bronchogenic, echinococcal)
neurenteric,
0 A solitarypulmonarynoduleis the initial Adenoma,Arteriovenous malformation
radiographicfindingin 20-30/" of patientswith Hamartoma,Histoplasmosis
lungcancer! Abscess,Actinomycosis
(a) Malignantprimariesof lung Lymphoma
1. Bronchogenic carcinoma(66%,2nd most
commonmass)
2. Primarypulmonarylymphoma Morphologic Evaluationof Solitary Pulmonary
3. Primarysarcomaof lung Nodule
4. Plasmacytoma(primary/ secondary) A . S IZE
5. Clearcell carcinoma,carcinoid,giantcell ca. 0 The smallerthe nodulethe more likelyit is
(b) Metastases(4th most commoncause) benignl
in adults: kidney,colon,ovary,testes r/ <zo mm nodule: in 80% benign
in children: Wilmstumor,osteogenicsarcoma, ./ >s0 mm nodule: likelymalignant
Ewingsarcoma,rhabdomyosarcoma N .B .: r/ tS X of mal i gnant nodul esare <10 m m
C. B E NI G NT U M O R S ^l qZ/" of malignantnodulesare <20 mm
(a) lung tissue : hamartoma(6%, 3rd most r/ nodule>3 cm is suspectfor malignancy
commonlung mass),chondroma B . MA R GIN/ E D GE
(b) fat tissue : lipoma(usuallypleurallesion) r/ smoothwell-definedmargin= likelybenign
(c) fibroustissue : fibroma 0 Mostl ybeni gn,i n 21' h mal i gnant
(d) muscletissue: leiomyoma ri corona radiata= irregularspiculatedmargin
(e) neuraltissue : schwannoma,neurofibroma, 0 In 89% malignant,in 557obenign
paraganglioma r/ pleuraltag
(f) lymphtissue : intrapulmonary lymphnode 0 ln 25h malignant , in 9"h benign
(g) deposits : amyloid,splenosis, endometrioma, C . C ON TOU R
extramedullary hematopoiesis { sharpl ymargi nated l esi onsare benignin79/ "
D. VASCULAR ./ lobulatednoduleimplies
1. Arteriovenous malformation (AVM),hemangioma (a) organizingmass
2. Hematoma (b) tumorwith multiplecelltypesgrowingat
3. Organizinginfarct differentrates (malignancy,hamartoma)
4. Pulmonaryvenousvarix 0 A lobulatedcontourfrequentlyimplies
5. Pseudoaneurysm of pulmonaryartery malignancY!
 Differential Diagnosis of Chest Disorders 4l g

0 Lobulatedgrowthoccursin 2S/" of benign (b) for benignnodules:<30 and > 400 days
nodules 0 Absenceof growthover a2-year period
{ vesselleadingto mass: pulmonaryvarix,AVM impliesa doublingtime of >730days
D. INTERNALATTENUATION Disadvantage:
r/ homogeneousattenuatio n in S5%"of benign (1) only 65% positivepredictivevalue
+ 20/" of malignantnodules - very slow growth:
r/ pseudocavitation (= smallfocal hypodense hamartoma,bronchialcarcinoid,
region)with air bronchogramsuggest inflammatory pseudotumor, granuloma,
bronchioloalveolar cell carcinoma/ lymphoma/ low-gradeadenocarcinoma, metastases
resolvingpneumonia from renalcell carcinoma
0 Air bronchogram in <2cm nodules: - very rapidgrowth:
in 65% malignant,in 5o/"benign osteosarcoma,choriocarcinoma, testicular
r/ OuObtetike areas of low attenuation: neoplasm,organizinginfectiousprocess,
bronchioloalveolar cell carcinoma(in S0%) infarct(thromboembolism, Wegener
E. CAVITATION granulomatosis)
{ a thin (<4 mm) smoothwall is benignin g4% (2) unreliablegrowthperceptionin nodules
{ a thick(>16 mm) irregularwall suggestsa < 10 mm: eg, a nodul ew i tha doublingt im eof
malignantnodule 6 monthsincreasesits diameterfrom 5 mm to
F. INTRANODULAR FAT (-40 to -120 HU) only 6.25 mm remainingradiologically stable
0 Fat is a reliableindicatorof a hamartomal better: volumetricgrowthassessment
r/ fat densityin up to 50% of hamartomas r/ decreasein sizewith time: benignlesion
G . I NT RA N OD U L AC RA L C IU M 0 Bronchogenic carcinomamay showtemporary
0 HRCTis 10-20 timesmoresensitivethan CXR! decreasein size due to infarction- necrosis-
^/ >eOOHU at CT densitometryindicates fibrosis- retractionsequence!
calcification
withina nodule(66%sensitive,
98% specificfor benigndisease)
0 38-63% of benignnodulesare not calcified!
r/ diffuseamorphous,rarelypunctate= malignant Clinical Assessment of lndeterminateSolitary
pattern
Pulmonary Nodule
r/ central,completelysolid,laminated:granuloma
by patientage (prevalenceof cancer<30 years is
of prior infection(TB / histoplasmosis) low),historyof priormalignancy,
presenting
^/ popcornlike= chondroidcaicificationin a
symptoms,smokinghistory
hamartomain 5-50%
r/ peripheralcalcification:granuloma,tumor
Calcifying malignant lung tumors:
carcinoid(up to g3%),lungcancer(up to 6%),
osteosarcoma,chondrosarcoma,
ManagementStrategiesof lndeterminate
metastatic
mucinous adenocarcinoma Solitary Pulmonary Nodule
H. SATELLITELESION A. BayesianAnalysis
= nodule(s)in association Likelihoodratio(LR)= probability
of malignancy
with largerperipheral
= LR of 1.0 meansa 50% chanceof malignancy
nodule
- in 99% due to inflammatorydisease(oftenTB)
- in 1% due to primarylungcancer
Characteristic / Feature Likelihood
Ratio
spiculatedmargin 5.54
size >3 cm 5.23
Growth RateAssessment of lndeterminate >70 years of age 4.16
Solitary Pulmonary Nodule malignantgrowthrate 3.40
= coffip?ringsize of noduleon currentimagewith that smoker 2.27
on priorimage upper lobe location 1.22
Best method: s i z e< 1 0 m m 0.52
earlyrepeatHRCT (resolution in x and y planesol smoothmargin 0.30
0.3 mm) in 1-4 weeksfor nodules>5 mm 30-39 years of age 0.24
measuringvolumeI area/ diameterof nodule neversmoked 0.19
Doubling time (= time requiredto doublein volume): 20-29 years of age 0.05
(a) for most malignantnodules: 30-400 days benigncalcification 0.01
= 26o/"increasein diameter benigngrowthrate 0.01
= 30 days: aggressivesmall cell cancer
= 90 days: squamouscellcarcinoma Odds of malignancy(Oddsca)= Sumof LR of
= 120 days: largecell carcinoma radiologicfeaturesor patientcharacteristics
= 150 days: aggressiveadenocarcinoma of malignancy(pCa)- Odds""I (1 +
Probability
= 180 days: averageadenocarcinoma Odds.")
420 Radiology Review Manual

B. DecisionAnalysis C . C E N TR A Li P E R IP H E R A L
= cost-effectivestrategyfor managementdecision 1. Li poma:
determinedby pCa (a) subpleural
pOa <0.05 observation (b) endobronchial
pOa >0.05and <0.6 biopsy D . P S E U D OTU MOR
pCa>0.60 immediatesurgicalresection 1. Fibroxanthoma/ xanthogranuloma
C. Contrast-enhanced Thin-section CT 2. Plasmacell granuloma
= degreeof enhancementdirectlyrelatedto 3. Sclerosinghemangioma
vascularity+ likelihoodof malignancy middle-aged woman,RMLi RLL (most
. 300 mg/ml iodineat 2 musec; commonly),may be multiPle
total dose 420 mglkg 4. Pseudolymphoma
. contiguoussectionsobtainedevery 30 seconds 5. Roundatelectasis
for 5 minutes 6. Pleuralpseudotumor= sccufilulationof pleural
r/ noduleenhancement of fluidwithininterlobarfissure
(a) .15 HU suggestsbenignlesion
(b) >20 HU indicatesmalignancy
(98% sensitive,73/" specific,85% accurate) LungTumorin Childhood
D. FDG PositronEmissionTomography 1. Metastatic(common)
= increasedglucosemetabolismin tumors 2. Blastoma
resultingin increasedaccumulation(= uptake 3. Mucoepidermoid carcinoma
and trappingof FDG-6-phosPhate) 4. Bronchogenic carcinoma
r/ no uptake= benignnodule(92-1007osensitive, 5. Hemangiopericytoma
52-100% specific,94% accurate) 6. Rhabdomyosarcoma
FN: carcinoidtumor, bronchioloalveolar
carcinoma;malignantlesion<10 mm
Probability of malignancy: <5/" LargePulmonaryMass
Precaution; radiographicfollow-up at mnemonic; "CAT PIES"
3-monthintervals Carcinoma(largecell,squamouscell,cannonball
FP: activeTB, histoplasmosis, rheumatoid metastasis
nodule Abscess
E. TransthoracicNeedleAspirationBiopsy Toruloma (Cryptococcus)
95-100% sensitivefor 10-1S-mmmalignancies; Pseudotumor, PlasmacYtoma
up to 91% sensitivefor establishing a benign Inflammatory
diagnosis Echinococcaldisease
Cx: pneumothorax(5-30%) with chesttube Sarcoma,Sequestration
placementin 15%;self-limiting hemorrhage

CavitatingLung Nodule
A. NEOPLASM
(a) Lungprimary:
BenignLungTumor 1. Squamouscell carcinoma(10%)
A. CENTRALLOCATION 2. Adenocarcinoma (9.5%)
1. B r onc h i apl o l y p 3. Bronchioloalveolarcarcinoma(rare)
2. Bronchialpapilloma 4. Hodgkindisease(rare)
3. Granular cell myoblastoma (b) Metastases(4% cavitate):
= cell of originfrom neuralcrest 1. Squamouscell carcinoma(2/3)
Age: middle-aged, esp. Blackwomen nasopharynx (males),cervix(females),
r/ endobronchiallesionin majorbronchi esophagus
B . P E RI P HE R AL L OC AT ION 2. Adenocarcinoma(colorectal)
1. Ham arto m a 3. Sarcoma: Ewingsarcoma,osteo-,myxo-,
2. Leiomyoma angiosarcoma
benignmetastasizing leiomyoma,historyof 4. Melanoma
hysterectomy 5. Seminoma,teratocarcinoma
3. Amyloidtumor 6. W i l mstumor
not associatedwith amyloidof other organs/ B. COLLAGEN-VASCULAR DISEASE
rheumatoidarthritisi myeloma 1. Pulmonaryangitis+ granulomatosis
4. Intrapulmonary lymphnode Wegenergranulomatosis + Wegenervariant
5. Arteriovenousmalformation 2. Rheumatoidnodules+ Caplansyndrome
6. Endometrioma, fibroma,neuraltumor, 3. S LE
chemodectoma 4. Periarteritisnodosa(rare)
 Differential Diagnosis of Chest Disorders 421

C. GRANULOMATOUS DISEASE ShaggyPulmonaryNodule

1. Histiocytosis X mnemonic: "ShaggySue Made LovingA ReallyWild
2. Sarcoidosis (rare) FantasyToday"
D. VASCULARDISEASE Sarcoidosis,alveolartype
1. Pulmonaryemboluswith infarction Septicemboli
2. Septicemboli(Staphylococcus aureus) Metastasis
E . I NF E CT I O N Lymphoma,Lung primary,Lymphomatoid
1. Bacterial:pneunnatoceles from staphylococcal
/ granulomatosis
Gram-negativepneumonia Alveolarcell carcinoma
2. Mycobacterial:TB Rheumatoidlung
3. Fungal: nocardiosis,cryptococcosis, Wegenergranulomatosis
coccidioidomycosis (in 10%),aspergillosis Fungus
4. Parasitic:echinococcosis(multiplein 20-30/"\, Tuberculosis
paragonimiasis
F. TRAUMA
Hemorrhagic
PulmonaryNodule
1. Traumaticlungcyst (afterhemorrhage)
! CT halo sign = central areaof soft-tissueattenuation
2. Hydrocarboningestion(lowerlobes)
surroundedby a haloof ground-glass attenuation
G . B RO NCHO P U L MON ARDYISE AS E
Causes:
1. I nf ec t e db u l l a
A . H E MOR R H A GIC IN FA R C TION
2. Cysticbronchiectasis
1. Earlyinvasiveaspergillosis
3. Communicating bronchogenic cyst 2. Mucormycosis
3. Hematogenouscandidiasis
mnemonic; "CAVITY"
4. Herpessimplex,CMV,varicella-zoster virus
Carcinoma(squamouscell),Cysticbronchiectasis
B. VASCULITIS
Autoimmunedisease(Wegenergranulomatosis,
1. Wegenergranulomatosis
rheumatoidlung)
C. FRAGILITYOF NEOVASCULAR TISSUE
Vascular(bland/ septicemboli)
1. Metastaticangiosarcoma
Infection(abscess,fungaldisease,TB, Echinococcus)
2. Metastaticchoriocarcinoma
Trauma
3. Bronchioloalveolar carcinoma
Young= congeflital(sequestration,
diaphragmatic 4. Lymphoma
hernia,bronchogeniccyst)
5. Kaposisarcoma
D . B R ON C H OA R TE R IAFISL TU LA
1. Coccidioidomycosis
2. Tuberculomaassociatedwith hemoptysis
Pulmonary Masswith Air Bronchogram E . TR A U MA
1. Bronchioloalveolar
carcinoma 1. Fol l ow i ng
l ungbi opsy
2. Lymphoma
3. Pseudolymphoma
MultiplePufmonaryNodulesand Masses
4. Kaposisarcoma
r/ homogeneous masseswith sharpborder
5. Blastomycosis r/ no air alveolo-/ bronchogram
A . TU MOR S
(a) malignant
Air-crescentSign 1. Metastases:
= air in a crescenticshape separatingthe outerwall
from breast,kidney,Gl tract,uterus,ovary,
of a nodule/ massfrom an innersequestrum testes,malignantmelanoma,sarcoma,Wilms
A . I NF E CT IO N tumor
1. Invasivepulmonaryaspergillosis 2. Lymphoma(rare)
2. Noninvasive mycetoma 3. Multipleprimarybronchogenic carcinomas
3. Echinococcallung cyst (synchronousin 1T"of all lung cancers)
4. Tuberculoma (b) benign
5. Rasmussenaneurysms(mostare too smallto 1. Hamartoma(rarelymultiple)
be identifiedon CXR) 2. Benignmetastasizing leiomyoma
6. Bacterialfungabscess+ pulmonarygangrene 3. AV malformations
B. CAVITATINGNEOPLASM 4. Amyloidosis
1. Primary/ metastaticcarcinoma/ sarcoma B. VASCULARLESIONS
2. Bronchialadenoma 1. Thromboemboli with organizinginfarcts
3. Cystichamartoma 2. Septicemboliwith organizedinfarcts
C. T RA UM A C. COLLAGEN-VASCULAR DISEASE
1. P ulm o n a ry
h e ma to ma 1. Wegenergranulomatosis: vasculitiswith
D. THROMBOEMBOLISM organizing infarcts
422 RadiologyReviewManual

2. Wegenervariants Lung Nodule

Pleura-based
3. Rheumatoidnodules:tendencyfor periphery, / sharplydefinedlesionmimickinga true
^/ itt-defined
occasionallycavitating pleuralmass
D. INFLAMMATORY GRANULOMAS { associatedlineardensitiesin lungparenchyma
1. Fungal:coccidioidomycosis, histoplasmosis, Cause:
cryptococcosis 1. Granuloma(fungus,tuberculosis)
2. Bacterial:nocardiosis,tuberculosis 2. Inflammatory Pseudotumor
3. Viral: atypicalmeasles 3. Metastasis
4. Parasites:hydatidcysts,paragonimiasis 4. Rheumatoidnodule
5. Sarcoidosis:large accumulationof interstitial 5. Pancoasttumor
granulomas 6. Lymphoma
6. lnflammatory pseudotumors:fibrous 7. Infarct:HamPtonhumP
histiocytoma,plasmacell granuloma,hyalinizing L AtelectaticPseudotumor
pulmonarynodules,pseudolymphoma
IntrathoracicMassof Low Attenuation
mnemonic.' "SLAM DA PlG" A. CYSTS
Sarcoidosis 1. Bronchogenic/ neurenteric/ pericardial
cyst
Lymphoma 2. Hydatiddisease
Alveolarproteinosis B. FATTYSUBSTRATE
Metastases 1. Hamartoma
Drugs 2. Lipoma
Alveolarcell carcinoma 3. TuberculouslYmPhnode
P neum onia s 4. Lymphadenopathy in Whippledisease
Infarcts C. NECROTICMASSES
Goodpasturesyndrome 1. Resolvinghematoma
2. TreatedlymPhoma
Multiple Cavitating Nodules/ Masses 3. Metastasesfrom ovary, stomach,testes
A. PULMONARYVASCULITIS
1. Wegenergranulomatosis
2. Necrotizing sarcoidgranulomatosis PNEUMOCONIOSIS
3. Bronchocentric granulomatosis = tissue reactionto the presenceof an accumulationof
B. METASTATICDISEASE dusti n the l ungs
particularlysquamoushistologictype Path: 1. Fibrosis
C. M ULT I FO C AL IN F EC T IO N (a) focal/ nodular(silicosis)
1. P s eu d o mo n a s (b) diffusefibrosis(asbestosis)
2. Tuberculosis 2. Aggregatesof particle-ladenmacrophages
3. Septicabscesses in inertdusts(iron,tin, barium)
D. MULTIPLEPULMONARYINFARCTS Types:
E . B RO NC H IEC T AS IS 1. S i l i cosi s
F. NEOPLASMS 2. CoalworkerPneumoconiosis
1. Ly m p h o ma 3. Siderosis
2. Multicentric bronchioalveolarcarcinoma 4. CarbonblackPneumoconiosis
G. COLLAGEN-VASCULAR DISEASE = burningof naturalgas + petroleumproducts(filler
1. Rheumatoidnodules in rubber,plastics,phonographrecords,inks,
H. GRANULOMATOUS DISEASE carbon paper, carbon electrodes)
1. Cysticform of sarcoidosis { tine reticulonodular patternwith lowerzone
2. Langerhanscell histiocytosis predominance
5. HardmetalPneumoconiosis
= alloyof tungsten,carbonand cobalt(occasionally
SmallPulmonary Nodules
mnemonic.' "MALTS" addingtitanium,tantalum,nickel,chromium)
r/ giantcell interstitial pneumonia,desquamative
Metastases(esp.thYroid)
Alveolarcell carcinoma interstitialpneumonia,interstitialpneumonia
6. Asbestos-related disease
Lymphoma,Leukemia
TB
Sarcoid
iosis Classification
Pneumocon
according to ILO (lnternational Labour Office)
A . TY P EOF OP A C ITIE S
Pulmonary Nodules& Pneumothorax
1. Silicosis,coalworker'spneumoconiosis
1. Osteosarcoma
nodularoPacities:
2. Wilmstumor
3. Histiocytosis P = < 1 ' 5m m
 DifferentialDiagnosisof ChestDisorders 4Zg

q = 1. 5 -3 m m Focal Area of Ground-glassAttenuation

| - 3-10 mm 1. Bronchioloalveolar cellcarcinoma
2.. Asbestosis 2. Pulmonaryinfiltratewith eosinophilia
syndrome
linearopacities: (a) simplepulmonaryeosinophilia
s = f in e (b) idiopathichypereosinophilicsyndrome
t = medium (c) parasiticinfection
U = c o a rs e /b l o tc h y 3. Lymphoma
B . P RO F US I O N
/ S EV ER IT Y 4. Hemorrhagic nodule
0 = nOrmal
1 = slight Opacification of Hemithorax
2 = moderate mnemonic.- "FATCHANCE"
3 = advanced Fibrothorax
intermediate grading: Adenomatoidmalformation
212= definitelymoderateprofusion Trauma(ie, hematoma)
2/3= moderate,possiblyadvancedprofusion Gollapse,Cardiomegaly
Hernia
Pneumoconiosis
with Mass Agenesisof lung
Anthracosi
licosiswith: Neoplasm(ie, mesothelioma)
1. Granuloma(histoplasmosis,TB, sarcoidosis) Consolidation
2. Bronchogenic carcinoma(incidencesame as in Effusion
generalpopulation)
3. Metastasis
4. Progressivemassivefibrosis
Atelectasis
5. Caplansyndrome(rheumatoid nodules) Cause:
A . TU MOR
PU LMONARY CALCI FICATIONS 1. Bronchogenic carcinoma(213of squamouscell
MultiplePulmonaryCalcifications carcinomaoccuras endobronchial masswith
persistent/ recurrentatelectasisor recurrent
A . I NF E CT I O N
pneumonia)
1. Histoplasmosis
2. Tuberculosis 2 . Bronchialcarcinoid
3. Chickenpoxpneumonia 3 . Metastases:primarytumorof kidney,colon,
B. INHALATIONAL rectum,breast,melanoma
DISEASE
1. S ilic os is 4. Lymphoma(usuallyas a latepresentation)
C. M I S CE LLA N E O U S 5 . Lipoma,granularcell myoblastoma, amyloid
1. Hypercalcemia tumor,fibroepithelialpolyp
2. Mitralstenosis B. INFLAMMATION
'1. Tuberculosis
3. Alveolarmicrolithiasis (endobronchial granuloma,
broncholith, bronchialstenosis)
2. Rightmiddlelobesyndrome(chronicright
CalcifiedPulmonaryNodules
middlelobeatelectasis)
mnemonic.' "HAM TV Station"
3. Sarcoidosis(endobronchial granuloma- rare)
Histoplasmosis, Hamartoma
C . M U C U SP L U G
Amyloid,Alveolarmicrolithiasis
1. Severechest/ abdominalpain (postoperative
Mitralstenosis,Metastasis(thyroid,osteosarcoma,
patient)
mucinouscarcinoma)
2. Respiratory depressantdrug (morphine;CNS
Tuberculosis
illness)
Varicella
3. Chronicbronchitis/ bronchiolitis obliterans
Silicosis
4. Asthma
0 central / laminated/ popcorn/ diffusecalcifications
5. Cysticfibrosis
are characteristic
of benignsolitarylung nodulesl 6. Bronchopneumonia (peribronchial
inflammation)
D . OTH E R
DENSELUNGLESIONS 1. Largeleftatrium(mitralstenosis+ left lower
Ground-glass
Attenuation lobe atelectasis)
1. Desquamative interstitial
pneumonia 2. Foreignbody (aspirationof food, endotracheal
2. Extrinsicallergicalveolitis intubation)
3. Sarcoidosis 3. Broncholithiasis
4. Usualinterstitialpneumonia 4. Amyloidosis
5. Alveolarproteinosis 5. Wegenergranulomatosis
6. Cryptogenicorganizingpneumonia 6. Bronchialtransection
424 Radiology Review Manual

r/ local increasein lung density Cicatrizing Atelectasis

r/ crowdingof pulmonaryvessels = parenchymal fibrosiscausingdecreasedlung
{ bronchialrearrangement vol ume
1. Tuberculosis / histoplasmosis (upperlobes)
r/ displacementof fissures
r/ displacement of hilus 2. Silicosis(uPPer lobes)
r/ mediastinalshift 3. Scleroderma (lowerlobes)
r/ elevationof hemidiaphragm 4. Radiationpneumonitis(nonanatomical
r/ cardiacrotation distribution)
{ approximationof ribs 5. ldiopathicPulmonarY fibrosis
{ compensatoryoverinflationof normallung
Discoid Atelectasis
"EPlC"
Types: mnemonic;
A. OBSTRUCTIVE ATELECTASIS E mbol us
Resorptive Atelectasis P neumoni a
Pathophysiology: InadequateinsPiration
sum of partialgas pressuresin venousblood Carcinoma,obstructing
perfusingatelectaticregionis less than
atmospheric pressure,which is responsible for Rounded Atelectasis
gradualresorptionof air trappeddistalto site of Cause: any type of pleuralinflammatory
obstruction;continuingsecretionintosmall reaction(asbestosas leadingcause)
airways leads to consolidation(postobstructive Pathomechanism:
pneumonitis / bacterialinfection) thickeningof visceralpleurawith progressive
Cause: bronchiolarobstructionby wrinkling+ foldingof subpleurallung
1. Tumor Location: posterobasalsubpleural
2. Stricture r/ round/ lentiformmass incompletely
3. Foreignbody surroundedbY lung
4. Mucusplug { increasedattenuationin peripheryof mass
5. Bronchialrupture { pleuralthickeningn vicinityof mass
. airlesscollapsewithinminutesto hours { curvingof vessels+ bronchitowardmass
MR: { air bronchogram withinmass
r/ nigf' signalintensityon T2Wl in atelectatic r/ lesionmay be stable/ enlarge
area
B. NONOBSTRUCTIVE ATELECTASIS Left Upper Lobe CollaPse
Pathophysiology: PA view:
pathwaybetweenbronchialsystem+ alveoliis r/ Luftsichelsign = sharplymarginatedparaaortic
maintainedbecausebronchiare less compliant crescentof hyperlucency(= hyperexpanded
than lung parenchyma+ remainpatent; superiorsegmentof LLL extendingtowardlung
secretionscan be eliminated+ convective apex + betweenaorticarch and atelectaticLUL)
airflowto distalbronchioles remains { hazy opacificationof left hilum + cardiacborder
. collapsedlung not completelyairless(up to 40/" { elevationof left hilum
residualaiQ { near horizontalcourseof leftmain bronchus
MR: { posterior+ leftwardrotationof heart
r/ low-signalintensityon T2Wl in atelectatic Lateralview:
area r/ retrosternalopacity
{ majorfissuredisplacedanteriorlyparalleling
anteriorchestwall
Passive Atelectasis DDx: (1) Herniationof right lung acrossmidline
= pleuralspace-occupying process (leftwarddisplacementof anteriorjunction
1. P n e u m o th o ra x l i ne)
2. Hydrothorax/ hemothorax (2) MedialPneumothorax
3. D i a p h ra g ma ti c h e rn i a
4. Pleuralmasses: metastases, mesothelioma Multifocal lll-detined Densities
= densities5-30 mm resultingin airspacefilling
Adhesive Atelectasis A . IN FE C TION
= decreasein surfactantproduction 1. BacterialbronchoPneumonia
1. Respiratorydistresssyndromeof the 2. Fungalpneumoni a:
newborn(hyalinemembranedisease) histoplasmosis,blastomycosis,actinomycosis,
2. Pulmonaryembolism:edema,hemorrhage, coccidioidomycosis,aspergillosis,
atelectasis cryptococcosis,mucormycosis,sporotrichosis
3. Intravenousinjectionof hydrocarbon 3. Viralpneumonia
 DifferentialDiagnosisof ChestDisorders 425

4. Tuberculosis(primaryinfection) B . P U LMON A R YE MB OLIS M

5. RockyMountainspottedfever (rarelymultiplellargerthan subsegmental)
6. Pneumocystiscarinii C. NEOPLASM
B. GRANULOMATOUS DISEASE 1. Obstructive pneumonia
1. Sarcoidosis(alveolarform secondaryto 2. Bronchioloalveolar cellcarcinoma
peribronchial granulomas) D. ATELECTASIS
2. E os ino p h i l g
i cra n u l o m a
C. VASCULAR
(a) thromboembolic disease ChronicInfiltrates
(b) septicemboli Chronic lnfiltrates in Childhood
(c) vascufitis mnemonic.'"ABC'S"
1. Wegenergranulomatosis Asthma, Agammaglobulinemia, Aspiration
2. Wegenervariants:limitedWegener, Bronchiectasis
lymphomatoid granulomatosis Cysticfibrosis
3. Infectious vasculitis= invasionof pulmonary Sequestration,intralobar
arteries: mucormycosis,invasiveform of
aspergillosis,RockyMountainspottedfever Chronic Multifocal lll-detined Opacities
4. Goodpasturesyndrome 1. Organi zi ng
pneumoni a
5. Scleroderma 2. Granulomatous disease
D. NE O P LA S T IC 3. Allergicalveolitis
1. Bronchioloalveolar cell carcinoma 4. Bronchioloalveolar cell carcinoma
= only primarylungtumorto producemultifocal 5. Lymphoma
ill-defined densitieswith air bronchograms
2. Alveolartype of lymphoma Chronic DiffuseConfluent Opacities
= massiveaccumulation of tumorcellsin 1. A l veol arprotei nosi s
interstitiumwith compressionatelectasis 2. Hemosiderosis
+ obstructive pneumonia 3. Sarcoidosis
3. Metastases
(a) Choriocarcinoma:hemorrhage(howeverrare)
(b) Vasculartumors: malignanthemangiomas lll-defined Opacities with Holes
4. Waldenstrdmmacroglobulinemia A . IN FE C TION
5. Angioblasticlymphadenopathy 1. Necrotizingpneumonias:
6. Mycosisfungoides Staphylococcus aureus,B-hemolytic
7. Amyloidtumor streptococcus,Klebsiellapneumoniae,E. coli,
E . I DI O P A T H IC
IN T E R S T IT IAL D IS EA SE Proteus,Pseudomonas,anaerobes
1. Lymphocytic interstitial pneumonitis(Llp) 2. Aspirationpneumonia:
2. Desquamative interstitial pneumonitis(Dlp) mixedGram-negative organisms
3. Pseudolymphoma = localizedform of Llp 3. Septicemboli
4. Usualinterstitial pneumonitis (Ulp) 4. Fungus:
F. INHALATIONAL DISEASE histoplasmosis,blastomycosis,
1. Allergicalveolitis:acutestage(eg,farmer'slung) coccidioidomycosis, cryptococcosis
2. Silicosis 5. Tuberculosis
3. E os inop h i l i c p n e u m o n i a B. NEOPLASM
G . DRUGRE A C T ION S 1. Primarylungcarcinoma
2. Lymphoma(cavitatesvery rarely)
DiffuseInfiltratesin lmmunocompromised C. VASCULAR+ COLLAGEN-VASCULAR DTSEASE
CancerPatient 1. Emboliwith infarction
mnemonic.'"FOLD" 2. Wegenergranulomatosis
Failure(CHF) 3. Necrobioticrheumatoidnodules
Opportu nisticinfection D. TRAUMA
Lymphangitic tumorspread 1. Contusionwith pneumatoceles
Drugreaction

Segmental& Lobar Densities RecurrentFleetingIntiltrates

A. PNEUMONIA 1. Lofflerdisease
1. Lobarpneumonia 2. Bronchopulmonary aspergillosis
/ bronchocentric
2. Lobularpneumonia granulomatosis
3. Acuteinterstitial
pneumonia 3. Asthma
4. Aspiration
pneumonia 4. Subacutebacterialendocarditiswith pulmonary
5. Primarytuberculosis emboli
426 Radiology Review Manual

TubularDensity C. CARDIACCAUSE of decreasedpulmonaryblood

A. Mucoidimpaction flow
B. Vascularmalformation shunt:
1. R i ghtto-l eft
malformation
1. Arteriovenous Tetralogyof Fallot(smallproximalpulmonary
2. Pulmonaryvarix vessels),pseudotruncus, truncustype lV,
Ebsteinmalformation, tricuspidatresia
2. Eisenmengerphysiologyof teft-to-right shunt:
Perihilar"Bat-wing"lnfiltrates ASD,VSD, PDA (dilatedproximalpulmonary
mnemonic; "Please,Please,Please,Study Light,
vessels)
Don'tGet All Uptight"
D. PULMONARYCAUSEof decreasedpulmonary
Pulmonaryedema
bloodflow
Proteinosis
(a) Decreaseof vascularbed:
Periarteritis
1. P ul monarY embol i sm
Sarcoidosis
bilateralityis rare; localizedareas of
Lymphoma (Westermark sign)
hyperlucencY
Drugs
(b) lncreasein air space:
Goodpasturesyndrome
1. Air trapping(reversible changes):
Alveolarcell carcinoma
acute asthmaticattack,acute bronchiolitis
Uremia (pediatricPatient)
2. EmphYsema
Peripheral"ReverseBat-wing"Infiltrates 3. Bulla
mnemonic.' "REDS" 4. B l eb
Resolvingpulmonaryedema emPhYsema
5. lnterstitial
E os inophil p
i cn e u m o n i a
Desquamative interstitial pneumonia
UnilateralHyperlucentLung
Sarcoidosis A. FAULTYRADIOLOGIC TECHNIQUE
1. Rotationof Patient
B. CHESTWALL DEFECT
LUCENTLUNGLESIONS 1. Mastectomy
PulmonaryOligemia 2. Absentpectoralismuscle(Polandsyndrome)
GeneralizedOligemia P U LMON A R Y
C . IN C R E A S E D A IR S P A CE
= reductionin pulmonarybloodvolume with decreasedpulmonarybloodflow
1. Aorticvalvedisease (a) Largeairwayobstructionwith air trapping
indicativeof markedlydiminishedstrokevolume @ BronchialcomPression:
+ cardiacoutput hilarmass (rare),cardiomegalY
r/ LV enlargement compressingLLL bronchus
2. Overpenetration of film = artifact @ Endobronchial obstructionwith air trapping
3. Deepinspiration + Valsalvamaneuver (collateral air drift):
4. Positivepressureventilation foreignbody,broncholith, bronchogenic
carcinoma,carcinoid,bronchialmucocele
(b) Small airwaYobstruction
Regional Oligemia
1. Bronchiolitis obliterans
A . DE CRE AS EIN BL OODVOL U M E
2. Swyer-James i MacleodsYndrome
1. PulmonaryarterialhYPoPlasia
3. Emphysema(particularly bullous
2. Mitralvalve disease
emPhYsema)
3. Pulmonaryembolism
4. Emphysema+ unilaterallungtransplant
4. Flowinversion(= oligemicbases+ hyperemic (in supinepatient)
(c) Pneumothorax
upper lobesin longstandingelevationof left
D. PULMONARYVASCULARCAUSEof decreased
heartpressure)
pulmonarybloodflow
B . I NCRE A SEIN AIR S PA C E S
1. PulmonarY arterYhYPoPlasia
1. Swyer-James syndrome
2. PulmonarY embolism
2. Regionalemphysema
3. CongenitallobaremPhYsema
3. Valvularair traPPing
4. CompensatorY overaeration
HyperlucentLung
Bilateral Hyperlucent Lung LocalizedLucentLung Defect
A. CAVITY= tissuenecrosiswith bronchialdrainage
A. FAULTYRADIOLOGICTECHNIQUE
(a) Infection
1. Overpenetrated film
BncrentnlPNEUMoNIA
B . DE CRE A SE DSOF TT ISS U E S
1. Pyogenicinfection= abscess= necrotizing
1. Thin body habitus
2. Bilateralmastectomy P neumoni a:
 DifferentialDiagnosisof ChestDisorders 427

Staphylococcus, Klebsiella, 3. Recurrentbacterialpneumonias

Pseudomonas, anaerobes,B-hemolytic r/ multiplethin-walledlucencieswith air-fluid
streptococcus,E. coli, mixedGram- levelsin lower lobes
negativeorganisms 4. Childhoodinfection:tuberculosis, pertussis
2. Aspirationpneumonia= gravitational 5. Allergicbronchopulmonary aspergillosis
(in
p n e u mo n i a : asthmaticpatients)
mixedGram-negative organisms, r/ involvement of proximalperihilarbronchi
anaerobes 6. Kartagenersyndrome(ciliarydysmotility)
GRnruulorvrATous tNFEcloN (b) Pneumatocele
1. Tuberculosis 1. Postinfectious pneumatocele
cavitationindicatesactiveinfectious 2. Traumaticpneumatocele:lunghematoma/
diseasewith risk for hematogenous/ hydrocarboninhalation
bronchogenicdissemination (c) Congenitallesion(rare)
2. Fungalinfection: 1. Multiplebronchogenic cysts
nocardiosis (in immunocompromised), 2. Intralobarsequestration:multicysticstructure
coccidioidomycosis (any lobe,desert in lowerlobes
Southwest),histoplasmosis, 3. Congenitalcysticadenomatoidmalformation
blastomycosis,mucormycosis, (CCAM)type I
sporotrichosis, aspergillosis, 4. Diaphragmatic hernia(congenital/ traumatic)
cryptococcosis (d) Centrilobular/ bullousemphysema
{ very thin-walledcavitiesless likelyto (e) Honeycomblung
followapicaldistributionof TB /
histoplasmosis
3. Sarcoidosis(stagelV, upper lobe MultipleLucentLung Lesions
predominance) for detailssee causesof localizedlucentlung defect
4. Angioinvasive organism(septiclung A. CAVITIES
infarctionfollowedby cavityformation): (a) Infection
Aspergillus, Mucorales,Candida, 1, Bacterialpneumonia:cavitatingpneumonia,
Torulosis,P. aeruginosa lungabscess
PannsrrrctNFESTATtorrr: hydatiddisease 2. Granulomatous infection:TB, sarcoidosis
3. Fungalinfection:coccidioidomycosis
(b) Neoplasm
4. Parasiticinfection:echinococcosis
PRrvnRvLUNG TUMoR:16/" of peripherallung 5. Protozoaninfection:pneumocystosis
cancers(in particularin squamouscell (b) Neoplasm
carcinoma(30%);also in bronchioloalveolar (c) Vascular
cell carcinoma 1. Thromboembolic+ septicinfarcts
M Ernsrnsrs(usuallymultiple) 2. Wegenergranulomatosis
1. Squamouscellcarcinoma(nasopharynx, 3. Rheumatoidarthritis
esophagus,cervix)in 213 4. Angioinvasive organism(septiclung infarction
2. Adenocarcinoma (lung,breast,Gl) followedby cavityformation):Aspergillus,
3. Osteosarcoma(rare) Mucorales,Candida,torulosis,p. aeruginosa
4. Melanoma B. CYSTS
5. Lymphoma(rare): with adenopathy; (a) Cysticbronchiectasis
cavitiesoften secondaryto opportunistic 1. Cysticfibrosis(moreobviousin upperlobes)
infectionwith nocardiosis+ cryptococcosis 2. Agammaglobulinemia (predisposed to
(c) Vascularocclusion recurrentbacterialinfections)
1. Infarct(thromboembolic, septic) 3. Recurrentbacterialpneumonias
2. Wegenergranulomatosis 4. Tuberculosis
3. Rheumatoidarthritis 5. Allergicbronchopulmonary (in
aspergillosis
(d) Inhalational asthmaticpatients)
1. Silicosiswith coalworker'spneumoconiosis (b) Pneumatoceles
- complicating tuberculosis (c) Congenitallesions(rare)
- ischemicnecrosisof centerof 1. Multiplebronchogenic cysts
conglomeratemass (rare) 2. Intralobarsequestration:
multicysticstructurein lowerlobes
B . CYST 3. Congenitalcysticadenomatoidmalformation
(a) Cysticbronchiectasis (CCAM)type I
1. Cysticfibrosis(moreobviousin upperlobes) 4. Diaphragmatic hernia(congenital/ traumatic)
2. Agammaglobulinemia (predisposedto (d) Centrilobular/ bullousemphysema: btebs,bullae
recurrentbacterialinfections) (e) Tuberoussclerosis+ lymphangiomyomatosis
428 Radiology Review Manual

(f) Honeycomblung 4. Tuberoussclerosis

(g) JuvenilepulmonarypolYPosis ^/ associatedskin abnormalities,mental
retardation, ePilePsY
5. Air-blockdisease(adultrespiratorydistress
syndrome,asthma,bronchiolitis, viral/
PulmonaryCyst
= fourd circumscribedspace surroundedby an bacterialPneumonia)
epithelial/ fibrouswall of uniform/ variedthickness E . R E MOD E LIN G OF LU N GA R C H ITE CTURE
= honeycombing of idiopathicpulmonaryfibrosis
containingair / liquid/ semisolid/ solidmaterial
A. CONGENITALCYST (= fibrosingalveolitis)
r/ S-t 0-mmsmallirregularthick-walled cysticair
1. Cysticadenomatoidmalformation
spaces usually of comparable diameter
2. CongenitallobaremPhYsema
3. Bronchialatresia surroundedby abnormallungparenchyma
{ predominantly peripheral+ basilardistribution
4. Bronchogeniccyst
5. Sequestration F. MU LTIFA C TOR IA/ LU N K N OW N
B. ACQUIREDCYST 1. Langerhanscell histiocYtosis
(traumatic / infectious) r/ cystswith walls of variablethickness
1. Pneumatocele
2. Pseudocyst(from interstitialemphysema) ri combinationof nodules+ cavitation
3. Hydatiddisease r/ septalthickening
^/ predominant distributionin upperlungzones
4. Bleb = cysticair collectionwithinvisceralpleura;
2. Lymphocytic pneumonia
interstitial
mostlyapicalwith narrowneck;associatedwith
r/ thickeningof interlobular septa
spontaneousPneumothorax
+ bronchovascular bundles
5. Bulla = sharplydemarcateddilatedair space
ri enlargedmediastinal nodes
withinlungparenchyma>1 cm in diameterwith
epithelialized wall <1 mm thickdue to destruction 3. K l i ppel -TrenaunaY sY ndrome
4. Juveniletracheolaryngeal papillomatosis
of alveoli(= air cyst in localizedi centrilobularI
panlobularemphysema) 5. Neurofibromatosis
. usuallyasymPtomatic rl cysticair spacespredominantlyapical
{ typicallyat lung apex
{ slow progressiveenlargement
Cx: (1) SPontaneous Pneumothorax CystlikePulmonary Lesions
"C.C.,I BAN WHIPS"
(2) "Vanishinglung"= large area of mnemanic.:
localizedemphysemacausing Coccidioidomycosis
atelectasis+ dyspnea Cysticadenomatoidmalformation
Rx: surgicalresectionif bulla >33/" of lnfection
hemithorax Bronchogenic Bowel
cyst,Bronchiectasis,
Abscess
Neoplasm
Multiple Pulmonary Cysts Wegenergranulomatosis
X
HydatidcYst,HistiocYtosis
A . I NF E CT IO N
1. Tuberculosis lnfarction
2. Pneumocystis cariniipneumoniain AIDS Pneumatocele
B. VASCULAR-EMBOLIC Sequestration
1. Cavitatingsepticemboli
r/ often seen at end of feedingvessel
2. Angioinvasive infection(invasivepulmonary
aspergillosis,candida,P. aeruginosa) Multiple Thin-walled Cavities
"BlTCH"
3. Pulmonaryvasculitis(Wegener mnemonic;
granulomatosis) Bullae+ pneumatoceles
C. DILATATIONOF BRONCHI= bronchiectasis lnfection(TB, cocci,staPh)
{ bronchialwall thickening Tumor (squamouscell carcinoma)
D. DI S RU PT ION O F E L AS T ICF IBE RN ETW OR K Cysts (traumatic,bronchogenic)
1. Centrilobular emphYsema Hydrocarboningestion
2. Panlobularemphysema
./ lobulararchitecturepreservedwith
bronchovascular bundlein centralposition, Masswithin CavitY
areas of lung destructionwithoutarcuate 1. Mycetoma= asPergilloma
contour 2. Tissuefragmentwithincarcinoma
3. Lymphangiomyomatosis 3. Necroticlung within abscess
r/ randomlyscatteredcysts in otherwise 4. DisintegratinghYdatidcYst
normallung 5. blood clot
IntracavitarY

MEDIASTINUM
AcuteMediastinal
Widening
1. Ruptureof aorta/ brachiocephalicarteries
2. Venoushemorrhage:traumatic/ iatrogenic
(malpositioningof centralvenousline)
3. Congestiveheartfailure(venousdilatation)
4. Ruptureof esophagus
5. Ruptureof thoracicduct
6. Atelectasisabuttingthe mediastinum
7. Magnification+ geometricdistortionon supine
radiograph(attemptat suspendedfull inspiration,no
rotation,10-15" caudalangulationof centralbeam)
Mediastinal Shift
= displacementof heart,trachea,aorta,hilarvessels
0 Expiration film,lateraldecubitusfilm (expandedlung
down),fluoroscopyhelpto determinesideof
abnormality
A . DE CRE A SE D L U N GVOL U M E
1. Atelectasis
2. Postoperative(lobectomy,pneumothorax)
3. Hypoplasticlung/ lobe
{ smallpulmonaryartery+ smallhilum
r/ decreasedperipheralpulmonaryvasculature
^/ irregularreticularvascularpattern(bronchial
origin)withoutconvergingon the hilum
4. Bronchiolitis obliterans= Swyer-James syndrome
B . I NCRE A S E D L U N GVOL U M E
= air trapping = retentionof excessgas in allI part
of the lung,especiallyduringexpiration, as a result
of (a) complete/ partialairwayobstruction,or
(b) focalabnormalities in pulmonarycompliance
@ Major bronchus
1. Foreignbody obstructingmain-stembronchus
(commonin children)with ball-valve
mechanism+ collateralair drift
d contralateralmediastinalshift increasing
with expiration
@ Emphysema
1. Bullousemphysema(localized form)
r/ largeavascularareaswith thin lines
2. Congenitallobaremphysema:only in infants
3. Interstitial emphysema
r/ patternof diffusecoarselines;
Cx of positivepressureventilationtherapy
@ Cysts/ masses
1. Bronchogenic cyst: with bronchialconnection
+ check-valvemechanism
2. Cysticadenomatoidmalformation
3. Largemass (pulmonary, mediastinal)
C. PLEURALSPACEABNORMALITY
1. Largeunilateralpleuraleffusion:
opaquehemithorax throughempyema,
congestivefailure,metastases
2. Tensionpneumothorax:
not alwayscompletecollapseof lung
3. Largediaphragmatichernia:
usuallydetectedin neonatalperiod
4. Large mass
Differential Diagnosis of Chest Disorders 429
D. Partialabsenceof pericardium/ pectusexcavatum
r/ shiftof heartwithoutshift of trachea,aorta,or
mediastinalborder
Pneumomediastinum
Frequency; in 1"/"of patientswith pneumothorax
Source of air:
A. INTRATHORACIC
1. Trachea,majorbronchi: blunt chesttrauma
2. Esophagus
3. Lung
(a) narrowed/ pluggedairways (mostcommon)
= air trappingin small ainnrays
as in asthma
(b) strainingagainstclosedglottis: vomiting,
parturition,weight-lifti
ng
(c) bluntchesttrauma
(d) alveolarrupture
4. Pleuralspace
B. EXTRATHORACIC
1. Headand neck: sinusfracture,dental
extraction
2. Intra-and retroperitoneum:perforationof
hollowviscus
Pathophysiology:
after alveolarruptureair tracksalong bronchovascular
sheath+ rupturesthroughfascialsheathat lung root
intomediastinum and facialplanesof the neck
, producingsubcutaneous emphysema
ri subcutaneous emphysema
r/ streakylucenciesof air in mediastinum (lookat
thoracicinleton PA + retrosternalspace on LAT film)
r/ "ringaroundarterysign"= air surrounding
intramediastinalsegmentof rightpulmonaryartery
(LAT view)
ri "tubulararterysign" = air adjacentto majoraortic
, branches,eg, left subclavian+ left commoncarotidaa.
r/ "continuous diaphragm"sign = air trappedposteriorto
pericardiumproduceslucencyconnectingboth domes
, of hemidiaphragms (frontalview)
{ "doublebronchialwall sign"= cleardepictionof
bronchialwallby air nextto and withina bronchus
^/ "V signof Naclerio"/ "extrapleural sign"= mediastinal
air extendinglaterallybetweenmediastinal pleurai
lowerthoracicaorta + diaphragm
r/ "spinnakersail"/ "thymicsail"iign in children= air
outliningthe thymus
r/ air in azygoesophageal recess
! air in pulmonaryligament= triangulargas collectionin
low mid chest
DDx:
A . OTH E RA IR C OLLE C TION S
medial/ subpulmonary pneumothorax (simulating
extrapleuralsign);pneumoperitoneum (simulating
extrapleuralsign); pneumopericardium
B. MISTAKENNORMALANATOMICSTRUCTURES
superioraspectof majorfissure(on lordoticview);
anteriorjunctionline;Machband effect
430 Radiology Review Manual

Spontaneous Pneumomedi astinum D. MULTIPLESYMMETRICLIPOMATOSIS

Age: neonates(0.05-1"h), 2nd-3rd decade rare entitywithoutinvolvementof anterior
Cause: mediastinal/ cardiophrenic/ paraspinalareas
1. Ruptureof marginallysituatedalveolifrom r/ compressionof trachea
sudden/ prolongedrisein intraalveolar pressure r/ periscapularlipomatousmasses
with subsequentdissection of air along
centrally
bronchovascular bundlesto hila (interstitial
emphysema)+ ruptureinto mediastinum:
Valsalvamaneuver,statusasthmaticus, MediastinalMass
Low-attenuation
aspirationpneumonia,hyalinemembrane A. FLUID
disease,measles,giantcell pneumonia, 1. Foregutcyst
coughing,vomiting,strenuousexercise, 2. Lymphocele
parturition,diabeticacidosis,crack cocaine 3. Seroma
inhalation= free-basing(mixingsolidcocaine 4. Hematoma
"smokeable") 5. Abscess
salt with a solventto renderit
2. Tumorerosionof trachea/ esophagus 6. Hydatiddisease
3. Pneumoperitoneum / retropneumoperitoneum B . LY MP HN OD E
= extensionfrom peritoneal/ retroperitoneal/ 1. TuberculouslYmPhnodes
deep fascialplanesof the neck 2. Metastasisfrom thyroid/ testiculartumor
3. Lymphoma: treated/ untreated
Traumatic Pneumomedi astin um (rare) C . P R IMA R YN E OP LA S M
Cause: 1. N eurogenitumor
c
emphysema
1. Pulmonaryinterstitial 2. Fat-containing neoPlasm
= disruptionof marginalalveoliwith gas
travelingtoward mediastinumdue to positive
pressureventilation
2. Bronchial/ trachealruPture Cyst
Mediastinal
{ commonlyassociatedwith pneumothorax = 21"hof all primarymediastinaltumors
3. Esophagealrupture:diabeticacidosis, @ Anteriormediastinum
alcoholic,BoerhaavesYndrome 1. Thymiccyst
4. latrogenic- accidental 2. Dermoidcyst
neck/ chest/ abdominalsurgery,subclavian 3. Parathyroidcyst (uncommonas mediastinal
mediastinoscopy,
vein catheterization, mass)
bronchoscopy,gastroscopy,recto-sigmoido- @ Middle mediastinum
colonoscopy,electrosurgerywith intestinalgas 1. PericardialcYst
explosion,positivepressureventilation, 2. Bronchogenic cYst
intubation, bariumenema @ Posterior mediastinum
1. Esophageal duPlication cYst
2. NeurentericcYst
Mediastinal Fat 3. Thoracic duct cYst
A. MEDIASTINAL LIPOMATOSIS rare,filledwith chYle
B. FAT HERNIATION Etiology: degenerative/ lymphangiomatous
= offierltdlfat herniatinginto chest 4. Transdiaphragmatic jejunalduplication
1. Foramenof Morgagni 5 . Cystichygroma
= cardiophrenic-angle mass,R >> L side 6 . Lateral meningocele
2. Foramenof Bochdalek = outpouching of leptomeningesthrough
= costoPhrenic-angle mass,almostalwayson left intervertebralforamen
3. Paraesophageal hernia= perigastricfat through Etiology: in 75h neurofibromatosis
phrenicoesophageal membrane r/ spinalabnormalities (kyphoscoliosis,
scalloping
CT: of dorsalvertebrae,enlargementof
r/ fat witfrfine lineardensities(= orn€ntalvessels) intervertebralforamen,pedicleerosion,
C. LI P O M A thinningof ribs)
un- / encapsulated with variableamountof fibrous 7. PosttraumaticlYmPhocele
septa = containedpleural/mediastinal lymphcollection
. historyof prolongedchylouschesttube
ri smooth+ sharplydefinedboundaries
DDx: Liposarcoma,lipoblastoma(infancy),fat- drainage
containingteratoma,thYmoliPoma Timeof onset: severalmonthsafter iniury
(inhomogeneous, higherCT numbers,poor 8. Hydatid cyst
demarcation, + invasionof surrounding Location: paravertebralgutter
structures) { erosionof ribs + vertebrae
 DifferentialDiagnosisof ChestDisorders 431

Frequeneyof DevelopmentalMediastinal Cyst Mlss rruRaloeRTnlancle

cyst= Foregut
1. Enterogenous cyst(45%): Raidertriangle= on LAT CXR formedby posterior
(a) Bronchogeniccyst (35%) wall of trachea+ thoracicvertebrae+ aorticarch
(b) Esophagealduplication cyst (15%) 1. Aberrantrightsubclavianarlery
(c) Neurentericcyst (leastcommon) 2. Aberrantleft subclaviana. with right aorticarch
2. Pericardialcyst (30%) 3. Aneurysms
3. Thymiccyst (10%) 4. Posteriordescendinggoiter
4. Nonspecificmesothelialcyst (10%) 5. Enlargedlymphnode
5. Cystichygroma(5%) 6. Esophagealmass / duplicationcyst

Anterior Mediastinal Mass

MediastinalMass mnemonic.'"4T's"
(excluding
hyperplastic glands,
thymus granulomas, Thymoma
lymphoma,metastases) Teratoma
1. Neurogenictumors (28%) : malignantin16/" Thyroidtumor/ goiter
2. Teratoidlesions (19%) : malignantin 15% Terriblelymphoma
3. Enterogenous cysts (16%) A . S OLIDTH Y MICLE S ION S
4. Thymomas (13%): malignantin46h 1. Thymoma(beni gn,mal i gnant):most com m on
5. Pericardialcysts ( 7%) 2. Normalthymus(neonate)
3. Thymichyperplasia (child)
A . B E NI G NM E D IAS T IN AM
L AS S 4. Thymiccarcinoma
0 66-75% of all mediastinal tumorsare benign(in all 5. Thymiccarcinoid
age groups) 6. Thymolipoma
0 88% discoveredincidentallyon routinechest x-ray 7. Lymphoma
B. MALIGNANTMEDIASTINAL MASS B . S OLIDTE R A TOIDLE S ION S
A 57% foundin association with symptoms(pain, 1. Teratoma
cough,shortnessof breath) 2. Embryonalcell carcinoma
0 80% of malignanttumorsare symptomatic 3. Choriocarcinoma
Cervicothoracic sign: 4. S emi noma
r/ posteriorsuperiormediastinalmassesare sharply C. THYROID/ PARATHYROTD
, outlinedby apicallung 1. Substernalthyroid/ intrathoracic goiter
r/ anteriorsuperiormediastinal massesextendinginto (10%of all mediastinal masses)
neck have unsharpborders 2. Thyroidadenoma/ carcinoma
3. Ectopic parathyroid adenoma:
ThoracicInlet Lesions ectopiain 1-3% (62-817oin anterior
1. Thyroidmass mediastinum / thymus,307owithinthyroid
1-3% of all thyroidectomies have a mediastinal tissue,8% in posteriorsuperiormediastinum)
component;113of goitersare intrathoracic D . LY MP HN OD E S
Location: anterior(80%)i posterior(20%) 1. Lymphoma(Hodgkin,NHL): may arisein
mediastinum thymus,morecommonin youngadults
r/ displacementof tracheaposteriorly+ laterally 2. Metastases
(anteriorgoiter) 3. Benignlymphnode hyperplasia
r/ displacementof tracheaanteriorly+ esophagus 4. Angioblastic lymphadenopathy
posteriorly+ laterally(posteriorgoiter) 5. Mediastinal lymphadenitis: sarcoidosis/
r/ inhomogeneous density(cysticipaces, high- granulomatousinfection
densityiodinecontentsof >100 HU) E. CARDIOVASCULAR
r/ focal calcifications(common) 1. Tortuousbrachiocephalic artery
r/ marked+ prolongedcontrastenhancement 2. Aneurysmof ascendingaorta
r/ connectionto thyroidgland 3. Aneurysmof sinusof Valsalva
r/ vasculardisplacement + compression 4. DilatedSVC
NUC (rarelyhelpfulas thyroidtissuemay be 5. Cardiactumor
nonf un c ti o n i n g ): 6. Epicardialfat-pad
{ + uptakeon l-123I l-131scan (pertechnetate F. CYSTS
sufficientwith moderngammacameras, 1. Cystichygroma
SPECT imagingmay be hetp{ut) 2. Bronchogeniccyst
2. Cystichygroma 3. Extralobarsequestration
3-1 0% involvemediastinum ; childhood 4. Thymiccystsi dermoidcysts
3. Lymphoma 5. Pericardialcyst: (a) true cyst
4. Othertumors: adenoma,carcinoma,ectopic (b) pericardial
diverticulum
thymoma 6. Pancreaticpseudocyst
432 Radiology Review Manual

G . O T HE R S 11. Bacteriallungabscess
1. Neuraltumor(vagus,phrenicnerve) (c) lnhalational diseaseadenopathy
2. Paraganglioma 1. Silicosis(eggshellcalcificationalso in
3. Hemangioma / lymphangioma sarcoidosis+ tuberculosis)
4. Mesenchymal tumor (fibroma,lipoma) 2. Coalworker'spneumoconiosis
5. Sternaltumors 3. Berylliosis
(a) metastasesfrom breast,bronchus,kidney, B. FOREGUTCYST
thyroid 1. Bronchogenic cyst: cartilage,
/ respiratory
(b) malignantprimary(chondrosarcoma, respiratoryepithelium
myeloma,lymphoma) 2. Entericclst = esophagealduplicationcyst
(c) benignprimary(chondroma, aneurysmal 3. Extralobarsequestration (anomalous feeding
bonecyst,giantcelltumor) vessel)
6. Primarylung/ pleuraltumor 4. Hiatalhernia
(invadingmediastinum) 5. Esophagealdiverticula:Zenker,traction,
7. Mediastinal lipomatosis: epiphrenic
(a) Cushingdisease C. PRIMARYTUMORS(infrequent)
(b) Corticosteroidtherapy 1. Carcinomaof trachea
8 . Morgagnihernia/ localizedeventration 2. Bronchogenic carcinoma
9 . Abscess 3. Esophageal tumor:
leiomyoma,carcinoma,leiomyosarcoma
Middle Mediastinal Mass 4. Mesothelioma
mnemonic.' "HABlT5" 5. Granularcell myoblastoma of trachea(rare)
Hernia,Hematoma D. VASCULARLESIONS
Aneurysm 1. Aneurysmof transverseaorta
Bronchogeniccyst / duplicationcyst 2. Distendedveins (SVC, azygosvein)
Inflammation (sarcoidosis, histoplasmosis, 3. Hematoma
coccidioidomycosis, primaryTB in children)
Tumors- rememberthe 5 L's: Suecnnrnll Spnce Lesloru
Lung,especiallyoat cell carcinoma 1. Enlargedlymphnodes
Lymphoma 2. Bronchogeniccyst
Leukemia 3. Pericardialeffusion
Leiomyoma 4. Enlargedleftatrium
Lymph nodehyperplasia 5. Esophagealmass
A. LYMPHNODES 6. Aorticaneurysm
0 90o/"of massesin the middlemediastinumare
malignant AonncopuLMoNARY WIt'toowMnss
(a) Neoplasticadenopathy 1. Adenopathy
1. L y mp h o ma(H o d g k i n :N H=l 2 :1 ) 2. Aneurysms:traumaticaorticpseudoaneurysm,
2. Leukemia(in 25%): lymphocytic > pulmonaryarteryaneurysm,ductus Botalli
granulocytic aneurysm,bronchialarteryaneurysm
3. Metastasis(bronchus,lung,upperGl, 4 . Bronchogenic cyst
prostate,kidney) 5 . Tumorof tracheobronchialtree
4. Angioimmunoblastic lymphadenopathy 6 . Esophageal tumor
(b) Inflammatoryadenopathy 7 . Neurogenictumor
1. Tuberculosis / histoplasmosis (may leadto 8 . Mediastinalabscess
fibrosingmediastinitis)
2. Blastomycosis(rare)/ coccidioidomycosis WroeuHo oF PARATRAcHEAL Splce
3. Sarcoidosis(predominant involvement of Normalwidth: <5 mm
paratrachealnodes) 1. Dilatedtortuousvessels(brachiocephalic
artery,
4. Viralpneumonia(particularly measles SVC, azygosvein)
+ cat-scratchfever) 2. Enlargedlymphnode
5. Infectiousmononucleosis / pertussis 3. B ronchogeni ccarci noma
p n e u mo n i a 4. Mediastinallipomatosis
6. Amyloidosis 5. Mediastinalhematoma
7. Plague/ tularemia 6. Bronchogenic cyst
8. Drug reaction
9. Giantlymphnode hyperplasia RernocnnDtAcSpAce or HouKNEcHTLestot't
= Castlemandisease 1. H i atalherni a
10. Connectivetissuedisease(rheumatoid, 2. Esophagealfesion
sLE) 3. Left ventricularaneurysm
 DifferentialDiagnosisof ChestDisorders 4gg

4. Pericardialcyst 4. Sarcoidosis(in 2%, typicallyasymptomatic

5. Bronchogenic cyst patient)
6. Aorticaneurysm C. VASCULARMASS
7. Vagal/ phrenicnerveneurofibroma 1. Aneurysmof descendingaorta(curvilinear
calcification; elderly)
Posterior Mediastinal Mass 2. Enlargedazygos + accessory hemiazygosvein
A. NEOPLASM 3. Esophagealvarices
Neunocruc ruMoR(largestgroup):30% malignant 4. Congenitalvascularanomalies:aberrant
(a) Tumorof peripheralnerveorigin subclavianartery,doubleaorticarch,
. morecommonin adulthood pulmonarysling,interruption of IVC with
^l AO"/"appearas roundmasseswith sulcus azygos / hemiazygoscontinuation
^/ lowerattenuationthan muscle(in 73%) D . TR A U MA
1. Schwannotnd = neurilemoma (82%): 1. Aorticaneurysm/ pseudoaneurysm
derivedfrom sheathof Schwannwithout 2. Hematoma
nervecells 3. Loculatedhemothorax
2. Neurofibroma (10%): containsSchwann 4. Traumaticpseudomeningocele
cells + nervecells,3rd + 4th decade E. FOREGUTCYST
3. Malignantschwannoma r/ cysts may demonstrateperipheralrimfike
(b) Tumorof sympathetic gangliaorigin calcifications
. morecommonin childhood 1. Bronchogenic cyst
./ gOZ are elongatedwith taperedborders 2. Entericcyst
1. Ganglioneuroma (29-gg%): second 3. Neurentericcyst
most commontumor of posterior 4. Extralobarsequestration
mediastinum after neurofibroma F, FATTY MASS
2. Neuroblastoma (15%): highlymalignant 1. B ochdal ek herni a
undifferentiatedsmallroundcelltumor 2. Mediastinal lipomatosis
originatingin sympathetic ganglia, 3. Fat-containing tumors: lipoma,liposarcoma,
<10 yearsof age teratoma(rare)
3. Ganglioneuroblastoma (14%): both G. OTH E R
features,spontaneousmaturation 1. Loculatedpleuraleffusion
possible 2. Pancreaticpseudocyst
(c) Tumorsof paraganglia origin(rare) 3. Lateralmeningocele (neurofibromatosis;
1. Chemodectoma = paraganglioma (4%) enlargedneuralforamen)
2. Pheochromocytoma 4. Extramedullary hematopoiesis:
r/ rib spreading,erosion,destruction in chronicbone marrowdeficiency;paraspinal
r/ enlargement of neuralforamina area rich in RES-elements
(dumbbelllesion) r/ splenomegaly; wideningof ribs
r/ scallopingof posterioraspectof vertebral 5. "Pseudomass" of the newborn
body
r/ scoliosis mnemonic.' "BELLMAN"
CT: r/ low-densitysoft-tissuemass (lipid Bochdalekhernia
contents) Extramedullary hematopoiesis
SprrurruMoR: metastases(eg, bronchogenic Lymphadenopathy
carcinoma,multiplemyeloma),ABC, Lymphangioma
chordoma,chondrosarcoma, Ewingsarcoma Meningocele (lateral)
LvtritpHOtrln Aneurysm
Neurogenictumor
lruvnsrve rHyMoMA
MrserucHvMAl TUMoR (fibroma,lipoma,leiomyoma) Cardi ophrenic-ang Ie Mass
HEvnuororun A. Lesionof pericardium
LvvpHnrucron,tn 1. Pericardialcyst
THynororuMoR 2. lntrapericardiacbronchogeniccyst
B . I NF LA M M A T IO/NIN F EC T IO N 3. Benignintrapericardiacneoplasm:
1. Infectiousspondylitis:pyogenic,tuberculous, teratoma,leiomyoma,hemangioma, lipoma
fungal 4. Mal i gnant
neopl asm:
r/ destructionof endplates+ disk space mesothelioma, metastasis(lung,breast,
r/ paravertebralsoft-tissuemass lymphoma,melanoma)
2. Mediastinitis B. Cardiaclesion: aneurysm
5. Pancreaticpseudocyst C. Others:massesarisingfrom lung,pleura,
3. Lymphoidhyperplasia diaphragm,abdomen
434 Radiology Review Manual

RrcHrCnnoropHRENrc-ANGle Mnss 3. Lymphoma

A. Heart (b) TNFLAMMATORY
1. Aneurysm(cardiacventricle,sinusof 1. Tuberculosis (primary)in 80%
Valsalva) 2. Fungalinfection:histoplasmosis,
2. Dilatedrightatrium coccidioidomycosis, blastomycosis
B. Peri-/ epicardium 3. Viralinfections:atypicalmeasles
1. Epicardialfat-pad/ lipoma(mostcommon 4. lnfectiousmononucleosis
cause) 5. Drug reaction
./ triangularopacityin cardiophrenic angle 6. Sarcoidosis(in 1-3%)
lessdensethan heart 7. Bilaterallungabscess
./ increasein size undercorticosteroid mnemonic.' "Fat Hila Suck"
treatment Fungus
2. Pericardialcyst Hodgkindisease
C. Diaphragm Squamous/ oat cell carcinoma
1. Diaphragmatic herniaof Morgagni D . B ILA TE R A L H ILA RA D E N OP A TH Y
2. Diaphragmatic lymphnode (esp.in Hodgkin (a) NEOPLASTIC
disease+ breastcancer) 1. Lymphoma (50%in Hodgkindisease)
D. Anteriormediastinalmass 2. Metastases
1. T hymo l i p o ma 3. Leukemi a
E . P r im a ryl u n gma s s 4. Primarybronchogenic carcinoma
F. Paracardiacvarices 5. Plasmacytoma
G. Enlargedlymphnode: lymphoma,metastasis (b) TNFLAMMATORY
(lung,breast,colon,ovary,melanoma) 1. Sarcoidosis(in 70-90%)
2. S i l i cosi s
HypervascularMediastinal Mass 3. HistiocytosisX
1. P ar agan g l i o m a 4. ldiopathicpulmonaryhemosiderosis
2. Metastasis:typicallyrenalcell carcinoma 5. Chronicberylliosis
3. Castlemandisease (c) IN FE C TIOU S
4. Hem ang i o m a 1. R ubel l a,E C H Ovi rus, vari cel l a,
mononucleosis
5. Sarcoma mnemonic.' "PleaseHelen Lick My PopsicleStick"
6. Tuberculosis PrimaryTB
7. Sarcoidosis Histoplasmosis
Lymphoma
Hilar Mass Metastases
A . LA RG EP U L M O N AR Y A R T E R IE S Pneumoconiosis
r/ enlargement of mainpulmonaryartery Sarcoidosis
r/ abruptchangein vesselcaliber
r/ enlargedpulmonaryarterycomparedwith Eggshell Calcification of Nodes
bronchus(in same bronchovascular bundle) A . P N E U MOC ON IOS IS
r/ cephalization 1. S i l i cosi s(5% )
./ enlargement of rightventricle(RAO45", LAo 60') 2. Coalworker'spneumoconiosis (1.3-6%)
Cause: not seen in: asbestosis, berylliosis,
talcosis,
1. Chronicobstructive disease(emphysema) baritosis
2. Chronicrestrictive interstitiallungdisease B. SARCOTDOSTS (5%)
(idiopathic fibrosis,cysticfibrosis,rheumatoid C . FU N GA L+ B A C TE R ILAIN FE C TION(rar e) :
arthritis,sarcoidosis) 1. Tubercul osi s
3. Pulmonaryembolicdisease(acutemassive/ 2. Histoplasmosis
chronic) 3. Coccidioidomycosis
4. ldiopathicpulmonaryhypertension D. FIBROSING MEDIASTINITIS
5. Left-sidedheartfailure+ mitralstenosis E. LYMPHOMAFOLLOWINGRADIATIONTHERAPY
6. Congenitalheartdiseasewith left-to-right shunt
(a) acyanotic:ASD, VSD, PDA of AzygosVein
Enlargement
(b) cyanotic(admixturelesions):transposition Normalazygosvein (on uprightCXR): <7 mm
of greatvessels,truncusarteriosus A . C OLLA TE R A CL IR C U LA TION
B. DUPLICATION CYST 1. Portalhypertension
C. UNI LA T ER A H L IL A RA D E N OP AT H Y 2. SVC obstruction/ compressionbelowazygos
(a) NEOPLASTIC vei n
1. Bronchogenic carcinoma(mostcommon) 3. IVC obstruction / compression
2. Metastases(lackof mediastinalinvolvement 4. InterruptedIVC with azygoscontinuation
exceptional) 5. Partialanomalousvenousreturn(rare)
 DifferentialDiagnosisof ChestDisorders 4Ss

6. Pregnancy 2. P ul monary sl i ng
7. Hepaticvein occlusion = ohorT't€llous left pulmonaryarteryarising
B . RI G HTA TR IALH Y PE R T E N S IO N from rightpulmonaryartery,passing
1. Right-sidedheartfailure betweentrachea+ esophagusen routeto
2. Constrictivepericarditis left lung
3. Largepericardialeffusion 3. Bronchogenic cyst
most commonbetweenesophagus+ trachea
at levelof carina
THYMUS
(b) inflammatory:abscess
ThymicMass (c) neoplastic:neurofibroma
1. T hy m om a (d) traumatic: esophagealforeignbody,
2. Thymolipoma
esophagealstricture,hematoma
3. Thymiccyst C, INTRINSICTBACHEALCAUSES
4. Thymiccarcinoid (a) Congenital:
1. Congenitaltrachealstenosis:
DiffuseThymicEnlargement generalized/ segmental
A . B E NI G N = completecartilaginous ring (insteadof
1. Thymichyperplasia horseshoeshape)
2. Intrathymichemorrhage 2. Congenitaltracheomalacia= immaturityof
3. Hem an g i o ma trachealcartilage= chondromalacia
4. Lymphangioma . expiratorystridor
B. MALIGNANTTHYMICINFILTRATION ^/ tracheafcollapseon expiration
. presenceof adenopathyelsewhere
(b) Neoplastic:papilloma,fibroma,hemangioma
r/ no pleuralimplants (c) Traumatic:acquiredstenosis(endotracheal
1. Leuk em i a + tracheostomy tubes),granuloma,acquired
2. Hodgkin/ non-Hodgkin lymphoma tracheomalacia(cartilage degenerationafter
3. Langerhanscell histiocytosis inflammation, extrinsicpressure,bronchial
neoplasia,TE fistula,toreignbody)

TRACHEA& BRONCHI
TrachealNarrowing
A . A NT E RI O RC O MP R E SS IO N TrachealTumor
(a) Congenital . asthmasymptomatology
1. Congenitalgoiter . hoarseness, cough
2. Innominatearterysyndrome . wheeze (inspiratorywith extrathoraciclesion,
. ablationof right radialpulse by rigid
expiratorywith intrathoraciclesion)
endoscopicpressure . hemoptysis
r/ posteriortrachealdisplacement
{ focalcollapseof tracheaat fluoroscopy A. MALIGNANT
r/ pulsatileindentationof anteriortrachealwall 1. Squamous-cell carcinoma(commonestprimary)
by innominatearteryon MRI 0 50o/oof all malignanttracheallesions
Rx: surgicalattachmentof innominate 2. Adenoidcysticcarcinoma= cylindroma
arteryto manubrium 3. Carcinoic
(b) lnflammatory 4. Mucoepidermoid carcinoma
1. Cervical/ mediastinal abscess 5. Metastasisfrom renalcell carcinoma,colon
(c) Neoplastic cancer,malignantmelanoma
1. Cervical/ intrathoracic teratoma 6. Lymphoma
r/ amorphouscalcifications+ ossifications 7. Plasmacytoma
2. Thymoma B - B E N IGNTU MOR
3. Thyroidtumors 1. Cartilaginous tumor(hamartoma)
4. Lymphoma 2. Squamouscell papilloma
(d) Traumatic:hematoma 3. Fibroma/ lipoma
B. POSTEHIORTRACHEALCOMPRESSION 4. Hemangioma
(a) Congenital 5. Granularcell myoblastoma
1. Vascularring C. INFLAMMATION
- complete: doubleaorticarch, rightaortic
1. Granulomatous disease:tuberculosis,
arch sarcoidosis, Wegenergranulomatosis
- incomplete:anomalousrightsubclaviana.
2. Inflammatory myoblasticpseudotumor
t indentation
ofesophasus 3. Amyloidtumor
lTj;il:r 4. Pseudotumor:inspissated mucus,foreignbody
436 Radiology Review Manual

Tumor
Endobronchial B ronchi al W al l Thi ckeni ng
1. Neuroendocrinetumor(typicali atypicalcarcinoid) 0 Apparentthicknessof bronchialwall varieswith lung
2. Mucoepidermoid carcinoma windowchosenon CT: a meanwindowthat is too
3. Adenoidcysticcarcinoma low can makethe bronchialwall appearabnormal!
4. Hamartoma A . P E R IB R ON C H OV A S C U LA R
5. Leiomyoma 1. Sarcoidosis
6. Myoblastoma 2. Lymphangitic carcinomatosis
7. Mucousglandadenoma 3. Kaposisarcoma
8. Squamouscell carcinoma 4. Lymphoma
5. Pulmonaryedema
BronchialObstruction B . B R ON C H IA W
L A LL
1. Foreignbody: mostcommonlyin youngchildren 1. Airwaydisease
2. Granulomatous disease:due to granulomaformation 2. Relapsingpolychondritis
in bronchialwall/ extrinsiccompressionby 3. Wegenergranulomatosis
adenopathy 4. Amyloidosis
3. Broncholiths= erosionof calcifiednodesinto C . MU C OS A LIN FE C TION
bronchiallumen 1. Croup
4. Stenosis/ atresia 2. Tuberculosis
5. Neoplasm 3. Fungaldisease
(a) Bronchogenic carcinoma 4. Aspergillosis
(b) Adenoidcysticcarcinoma
(c) Mucoepidermoid tumor B ronchol i thi asi s
(d) Hamartoma 1. Histoplasmosis
mnemonic; "MEATFACE" 2. Tuberculosis
Mucusplug 3. Cryptococcosis
Endobronchial granulomatous disease 4. Actinomycosis
Adenoma 5. Coccidioidomycosis
Tuberculosis { calcifiedlymph node within/ adjacentto affected
Foreignbody bronchus
Amyloid,Atresia(bronchial) { bronchialobstruction:atelectasis,airspacedisease,
Cancer(primary) bronchiectasis, air trapping
Endobronchialmetastasis r/ absenceof associatedsoft-tissuemass

Mucoidlmpaction PLEURA
= BRONCHIAL MUCOCELE = BRONCHOCELE Pneumothorax
= €lccurTrulation
of inspissatedsecretions(mucus/ pus / = €lccUrT'rUlation
of air in the pleuralspace
inflammatory products)withinbronchiallumen; Pathophysiology:disruptionof visceralpleura/ trauma
usuallyassociatedwith bronchialdilatation to parietalpleura
A. WITH BRONCHIALOBSTRUCTIONin the presence ' pleuriticbacki shoulderpain,dyspnea(in 80-90%)
of collateralair drift Etiology:
1. Bronchialobstructionby neoplasm: A. TRAUMATICPNEUMOTHORAX
bronchogenic carcinoma/ adenoma (a) penetrating trauma
2. Bronchialatresia (b) blunttrauma
B. WITHOUTBRONCHIALOBSTRUCTION 1. Ribfracture
1. Asthma(mostfrequentcause): esp.duringacute 2. Increasedintrathoracicpressureagainst
attackor convalescentphase closedglottis: lungcontusion/ laceration
2. Fluid-filledbronchiectasis: historyof childhood 3. Bronchialfracture
pneumonia;peripheraldistribution ^/ fallenlungsign = hilumof lungbelow
3. Bronchopulmonary aspergillosis:centralperihilar expectedlevelwithinchest cavity
bronchiectasis r/ persistentpneumothorax with functioning
4. Cysticfibrosis chesttube
5. Chronicbronchitis r/ mediastinalpneumothorax
(c) iatrogenic
Signet-ringSign tracheostomy,centralvenouscatheter,PEEP
= cfoss sectionof usuallythick-walledand dilated ventilator(3-16%),thoracicirradiation
ringlikebronchus+ branchof pulmonaryarteryas B. SPONTANEOUSPNEUMOTHORAX
adjacentroundsoft-tissueopacity 1. Primary/ idiopathic spontaneous
1. Bronchiectasis pneumothorax (80%)
2. Multifocalbronchioloalveolarcarcinoma Cause: ruptureof subpleuralblebs in apical
3. Metastaticadenocarcinoma regionof lung
 DifferentialDiagnosisof ChestDisorders 497

Age: 20-40 years;M:F = 8:1; esp. in patients Types:

with tall asthenicstature;mostlyin 1. Closedpneumothof€lX = intactthoraciccage
smokers 2. Open pneumothor?X = "sucking"chestwound
. chest pain (69%) 3. Tension pneumothorax
. dyspnea = €lccUrnulation of air withinpleuralspacedue to
Prognosisi recurrencein 30% on same side, free ingress+ limitedegressof air
in 10% on contralateral side Pathophysiology:
Rx: simpleaspiration(in >50% success)/ intrapleuralpressureexceedsatmospheric
tube thoracostomy(in 90% effective) pressurein lungduringexpiration(check-valve
2. Secondaryspontaneouspneumothorax(20%): mechanism)
(a) Air-trapping disease:spasmodicasthma, Frequency; in 3-5% of patientswith spontaneous
diffuseemphysema,Langerhans cell pneumothorax, higherin barotrauma
histiocytosis, lymphangiomyomatosis, r/ displacement of mediastinum / anteriorjunction
tuberoussclerosis,cysticfibrosis l i ne
0 Chronicobstructive pulmonarydiseaseis ./ Oeepsulcussign = on frontalview largerlateral
the mostcommonpredisposing disorder costodiaphragmatic recessthan on opposite
of secondaryspontaneouspneumothorax side
(b) Pulmonaryinfections:lungabscess, r/ diaphragmatic inversion
necrotizing pneumonia,hydatiddisease, r/ total/ subtotallungcollapse
pertussis,acutebacterialpneumonia, r/ collapseof SVC / IVC / rightheart border
Staphylococcus aureus,Pneumocystis (decreasedsystemicvenous return+ decreased
c a ri n i pi n e u m o n i a cardiacoutput)
(c) Granulomatous disease:tuberculosis, N .B .: Medi calemergency!
coccidioidomycosis, sarcoidosis, berylliosis 4. Tension hydropneumothorax
(d) Malignancy:primarylungcancer,lung ! sharpdelineation of visceralpleuralby dense
metastases(esp.osteosarcoma,pancreas, pleuralspace
adrenal,Wilmstumor) r/ mediastinalshiftto oppositeside
(e) Connectivetissuedisorder:scleroderma, r/ air-fluidlevelin pleuralspaceon erectCXR
rheumatoiddisease,Marfansyndrome, Pneumothorax size:
Ehlers-Danlos syndrome AverageInterpleuralDistance (AlD) = (A + B + C) + 3
(f) Pneumoconiosis: silicosis,berylliosis [in cm] convertsto percentageof pneumothorax
(g) Vasculardisease:pulmonaryinfarction see nomogramin drawing
(h) Catameniallkata, Greek= accordingto;
men , Greek = monthlpneumothorax
= tecUffentspontaneous AID Pneumo
pneumothorax
cm Vo Vo
duringmenstruationassociatedwith
erect supine
endometriosis of the diaphragm;R >> L
(i) Neonataldisease: meconiumaspiration,
respiratortherapyfor hyalinemembrane +i

disease 0.5 E
115
(j) Cx of honeycomblung: pulmonaryfibrosis, l-r
F
1.0
cysticfibrosis,sarcoidosis,scleroderma,
eosinophilic granuloma,interstitial 1.5 = t'
- n (
pneumonitis, Langerhans cell histiocytosis,
2.0 :
rheumatoidlung,idiopathicpulmonary
hemosiderosis, pulmonaryalveolar :'o
proteinosis, 2.5
biliarycirrhosis

mnemonic.' "THE CHEST SET"

3.0 i"
(+.r
Trauma 3.5 =oo
-
Honeycomblung,Hamman-Rich
Emphysema,Esophagealrupture
syndrome |r
C)
ts 4.0 iot
Chronicobstructivepulmonarydisease
4.5 i- )r)o
Hyalinemembranedisease
Endometriosis PA View of Right Chest 5.0 :
Spontaneous, Scleroderma
Tuberoussclerosis
Estimate of Pneumothorax Size
Sarcoma(osteo-),Sarcoidosis A = maximum apical interpleural distance
Eosinophilic
granuloma =
! fnterpleural distance at midpoint of upper half of iung
Tuberculosis+ fungus C = interpleural distance at midpoint of lower half of lung
438 Radiology Review Manual
Radiographicsigns in upright position:
{ white marginof visceralpleuraseparatedfrom
parietalpleura
DDx: skin fold, air trappedbetweenchestwall soft
tissues,hair braid)
{ absenceof vascularmarkingsbeyondvisceral
pleuralmargin
Radiographicsigns in supine position:
1. Anteromedial pneumothorax (earliestlocation)
{ outlineof medialdiaphragmundercardiac
silhouette
r/ sharpdelineation of mediastinal contours(SVC,
azygosvein, left subclavianarlery,anterior
junctionline,superiorpulmonaryvein,heart
border,lVC, deep anteriorcardiophrenic sulcus,
pericardialfat-pad)
2. Subpulmonicpneumothorax (secondmost
commonlocation)
{ hyperlucent upperabdominalquadrant
r/ deep lateralcostophrenicsulcus
{ sharplyoutlineddiaphragmin spiteof
parenchymal disease
r/ visualizationof anteriorcostophrenic sulcus
! visualization of inferiorsurfaceof lung
3. Apicolateralpneumothorax(leastcommon
location)
r/ visualization of visceralpleuralline
4. Posteromedial pneumothorax (in presenceof
lower lobe collapse)
r/ lucenttrianglewith vertexat hilum
^/ V-shapedbase delineatingcostovertebral
sulcus
5. Pneumothorax outlinespulmonaryligament
Prognosis; resorptionof pneumothoraxoccurs at a rate
of 1.25'/"per day (acceleratedbY
increasinginspiredoxygenconcentrations)
Rx: A pneumothorax>35o/"usuallyrequires
managementwith a chesttube!
PleuralEffusion
A. TRANSUDATE(proteinlevelof 1.5-2.5g/dl)
Pathophysiology: result of systemicabnormalities
causingan outpouringof low-
proteinfluid
(a) Increasedhydrostaticpressure
1. Congestiveheartfailure(in 65%)
bilateral(88%);right-sided(8%); left-sided
(4/"); leastamounton leftside due to cardiac
movement,whichstimulateslymphatic
resorotion
2. Constrictivepericarditis(in 60%)
(b) Decreasedcolloid-oncotic pressure
- decreasedproteinProduction
1. Cirrhosiswith ascites(in 6%):
right-sided(67%)
- proteinloss / hypervolemia
1. Nephroticsyndrome(21"/"),overhydration,
glomerulonephritis (55%),peritoneal
dialysis
2. Hypothyroidism
(c) Chylouseffusion
0 Most frequentcause of isolatedpleuraleffusion
in newbornwith 15-25% mortalitY!
. chylomicrons + lymphocytes in fluid
B. EXUDATE
Pathophysiology: increasedpermeabilityof
abnormalpleuralcapillarieswith
releaseof high-protein fluidinto
pleuralspace
Criteria:
. pleuralfluidtotal protein/ serumtotal protein
ratioof >0.5
. pleuralfluidLDH / serumLDH ratioof >0.6
. pleuralfluidLDH >213of upperlimitof normal
for serumLD H (upperl i mi tfor LD H -200 lU)
. pleuralfluidspecificgravity>1.016
. proteinlevel>3 g/dl
r/ effusionwith septation/ low-levelechoes
{ "splitpleura"signon CECT = thickenedenhancing
visceral+ parietalpleuraseparatedby fluid
ri extrapleuralfat thickeningof >2 mm + increased
attenuation(edema/ inflammation)
(a) Infection
1. Empyema
empyema necessitatis = chronicempyema
attemptingto decompressthroughchest
wall (in TB, actinornycosis,aspergillosis,
blastomycosis,nocardiosis)
2. Parapneumoniceffusion(in 40%)
= arY effusionassociatedwith pneumonia/
lung abscess/ bronchiectasis without
criteriafor an empyema
3. Tubercul osi(isn 1% ):
high proteincontent(75 g/dl), lymphocytes
>7O/o,positiveculture(onlyin 20-25%)
4. Fungi: Actinomyces, Nocardia
5. Parasites:amebiasis(secondary to liver
abscessin 15-20h), Echinococcus
6. Mycoplasma,rickettsia(in 20%)
(b) Malignantdisease(in 60%)
. positivecytologicresults
Pathogenesis:
- pleuralmetastases(increasedpleural
permeability)
- lymphaticobstruction(pleuralvessels,
mediastinal nodes,thoracicduct disruption)
- bronchialobstruction(lossof volume
+ resorptivesurface)
- hypoproteinemia (secondary to tumor
cachexia)
Cause: lung cancer(26-49/"), breastcancer
(8-24'/"),lymphoma (10-28'/., in 213
chylothorax),ovariancancer(10/"),
malignantmesothelioma containing
hyaluronicacid (5%)
Rx: sclerosingagents:doxYcYcline,
bleomYcin, talc
(c) Vascular
Pulmonaryemboli(in 15-30% of all embolic
events): often serosanguinous
 Differential Diagnosis of Chest Disorders 4gg

(d) Abdominaldisease r/ meniscus-shaped semicircular uppersurfacewith

1. Pancreatitis/ pancreaticpseudocysti lowestpointin midaxillary line
pancreaticopfeural fistula(in 2/3): { associatedcollapseof ipsilaterallung
r/ usuallyleft-sidedpleuraleffusion Massivepleuraleffusion:
. high amylaselevels ./ enlargement of ipsilateral
hemithorax
2. Boerhaavesyndrome: r/ displacementof mediastinumto contralateralside
left-sidedesophagealperforation ^/ severedepression/ flattening/ inversionof
3. Subphrenicabscess ipsilateral
hemidiaph ragm
r/ pleuraleffusion(79%) ri visibleair bronchogram
! elevation+ restrictionof diaphragmatic SubpuImonic/ subdiaphragmatic i infrapulmonary
motion(95%) pLeuraleffusion:
r/ basilarplatelikeatelectasis / pneumonitis ^/ peak of dome of pseudodiaphragm laterally
(7e%) positioned
4. Abdominaltumorwith ascites r/ acutelyangufatedcostophrenicangle
5. Meigs-Salmonsyndrome r/ increaseddistancebetweenstomachbubbleand
= primarypelvicneoplasms(ovarianfibroma, lung
thecoma,granulosacelltumor,Brenner V bluntedposteriorcostophrenic sulcus
tumor,cystadenoma, adenocarcinoma, { tnintriangularparamediastinal opacity
fibromyomaof uterus)causepleural (mediastinal extensionof pleuraleffusion)
effusionin 2-3o/o;ascites+ hydrothorax r/ flattenedpseudodiaphragmatic contouranterior
resolvewith tumor removal to majorfissure(on lateralCXR)
6. Endometriosis
7. B ilef i s tu l a C T:
(e) Collagen-vascular disease r/ tluiOoutsidediaphragm
1. Rheumatoidarthritis(in 3%): r/ ttuiOelevatingcrus of diaphragm
unilateral;R > L (in 75./"),recurrent r/ indistinctfluid-liverinterface
alternating sides;pleuraleffusionrelatively ^/ ttuiOposteromedial to liver(= bare area of liver)
unchangedin sizefor months;predominantly CAVE: "centraloval" sign of ascitesmay be seen in
in men; LOW GLUCOSEcontentof 20-50 subpulmonic effusionwith inverteddiaphragm
mg/dl (in 70-80%) withoutincreasefollowing
lV infusionof glucose(DDx: TB, metastatic
disease,parapneumonic effusion)
2. SLE (in 15-74%) Unilateral Pleural Effusion
mostcommoncollagenosis to give pleural 0 The majorityof massiveunilateralpleuraleffusions
effusion,bilateralin 50%; L > R are malignant(lymphoma,metastaticdisease,
r/ enlargementof cardiovascular silhouette(in primarylungcancer)!
35-50%) 1. N eopl asm
3. Wegenergranulomatosis (in 50%) 2. Infection:TB
4. Sjogrensyndrome 3. Collagenvasculardisease
5. Mixedconnectivetissuedisease 4. Subdiaphragmatic disease
6. Periarteritis nodosa 5. P ul monaryembol i
7. Postmyocardial infarctsyndrome 6. Trauma: fracturedrib
(f) Traumatic 7. Chylothorax
hemorrhagic, chylous,esophagealrupture,
thoracic/ abdominalsurgery,intrapleural infusion
= "infusothorax" (0.5%),radiationpneumonitis Lerr-sloeo Pleunnl Erruslor,r
(g) Miscelfaneous 1. Spontaneous ruptureof the esophagus
1. S ar c o i d o s i s 2. Dissectinganeurysmof the aorta
2. Uremicpleuritis(in 20% of uremicpatients) 3. Traumaticruptureof aortadistalto left
3. Drug-induced effusion subclavianartery
4. Transectionof distalthoracicduct
CX R: 5. Pancreatitis:left-sided(68%),right-sided
r/ first300 mL not visualizedon pA view (collectin (10/"), bilateral(22/")
subpulmonicregionfirst,then spillintoposterior 6. Pancreatic+ gastricneoplasm
costophrenic sinus)
{ lateraldecubitusviews may detectas littleas 25 mL
r/ hemidiaphragm + costophrenic sinusesobscured Rrcnr-sloeoPleunll Errusrolr
! extensionupwardaroundposterior> lateral> 1. Congestiveheartfailure
anteriorthoracicwall (mediastinal portionfixedby 2. Transectionof proximalthoracicduct
pulm onar yl i g a m e n+t h i l u m ) 3. Pancreatitis
440 RadiologyReviewManual

Pleural Effusion & Large Cardiac Silhouette C. VASCULAR

1. Congestiveheartfailure(mostcommon) i nfarct
1. P ul monary
r/ cardiomegaly 2. Arteriovenous malformation
{ prominenceof upperlobevessels+ constriction 3. Aorticdissection
of lower lobe vessels 4. Leakingatherosclerotic aneurysm
./ prominenthilarvessels D . MA LIGN A N C Y
{ interstitialedema (fine reticularpattern,Kerley 1. Mesothel i oma
lines,perihilarhaze,peribronchial thickening) 2. Lungcancer
{ alveolaredema(perihilarconfluentill-defined 3. Metastasis
densities,air bronchogram) 4. Leukemia
r/ "phantomtumor"= fluid localizedto interlobar E . OTH E R
pleuralfissure(in78% in righthorizontalfissure) 1. Catamenialhemorrhage
2. Pulmonaryembolus+ rightheartenlargement 2. Extramedullary hematopoiesis
3. Myocarditis/ pericarditiswith pleuritis r/ rapidlyenlarginghigh-attenuation pleuraleffusion
(a) viralinfection ^/ heterogeneousattenuation
(b) tuberculosis r/ hyperattenuating areasof debris
(c) rheumaticfever (poststreptococcal infection) ^/ ttuid-nematocritlevel
4. Tumor: metastatic, mesothelioma
5. Collagen-vascular disease SolitaryPleuralMass
(a) SLE (pleural+ pericardial effusion) = densitywith incompletemarginsand taperedsuperior
(b) rheumatoidarthritis + inferiorborders
1. Loculatedpleuraleffusion("vanishing tumor")
Pleural Effusion & Hilar Enlargement 2. Organizedempyema
1. Pulmonary embolus 3. Metastasis
2. Tumor 4. Localbenignmesothelioma
(a) bronchogeniccarcinoma 5. Subpleurallipoma: may erodeadjacentrib
(b) lymphoma 6. Hematoma
(c) metastasis 7. Mesothelial cyst
3. Tuberculosis 8. Neuraltumor: schwannoma,neurofibroma
4. Fungalinfection(rare) 9. Localizedfibroustumorof pleura
5. Sarcoidosis(very rare) 10. Fi bri n bodi es
= 34 cm largetumorlikeconcentrations of fibrin
Pleural Etrusion & SubsegmentalAtelectasis pleuraleffusions;usually
formingin serofibrinous
1. Postoperative(thoracotomy,splenectomy,renal near lung base
surgery)secondaryto thoracicsplinting+ mucous DDx: chestwall mass (rib destructionreliablesign of
pluggingof smallairway chestwall mass)
2. Pulmonaryembolus
3. Abdominalmass
4. Ascites MultiplePleuralDensities
5. Rib fractures r/ diffusepleuralthickeningwith lobulatedborders
1. Loculatedpleuraleffusion:
Pleural Effusion& Lobar Densities infectious,hemorrhagic, neoplastic
1. P neum o n i w
a i the m p y e ma 2. Pleuralplaques
2. Pulmonaryembolism 3. Metastasis(mostcommoncause)
3. Neoplasm Origin: lung (40%),breast(20'/"),lymphoma
(a) bronchogenic carcinoma(common) (10%),melanoma,ovary,uterus,Gl tract,
(b) lymphoma pancreas,sarcoma
4. Tuberculosis 0 Metastaticadenocarcinoma histologically
similarto
malignantmesothelioma!
4. Diffusemalignantmesothelioma
Hemothorax almostalwaysunilateral,associatedwith asbestos
A. TRAUMA exposure
1. Closed/ penetrating injury 5. Invasivethymoma(rare)
2. Surgery { contiguousspread,invasionof pleura,spreads
3. lnterventional procedures:thoracentesis,
pleural aroundlung
biopsy,catheterplacement ^/ lttOpleuraleffusion
B . B LE E DI N GD IA T H E SIS 6. Thoracic splenosis
1. Anticoagulanttherapy = autotransplantation of splenictissueto pleural
2. Thrombocytopenia spacefollowingthoracoabdominal trauma;
3. Factordeficiency discovered10-30 years later
 DifferentialDiagnosisof ChestDisorders 441

., asymptomatic/ recurrenthemoptysis ApicalCap

{ one or severalnodulesin leftpleurai fissures 1. lnflammatory process:TB, healedempyema
measuringseveralmm to 6 cm 2. Postradiationfibrosis
! pos it iv eTc -9 9 m-s u l fucro l l o i ds c a n ,i n d i um-111- 3. Neoplasm
labeledplatelets,Tc-99m-labeledheat-damaged 4. Vascularabnormality
RBCs 5. Mediastinalhemorrhage
6. Mediastinallipomatosis
mnemonic.' "MaryTyler Moore Likes Lemon" 7. Peripheralupperlobecollapse
Metastases(especiallyadenocarcinoma)
Thymoma(malignant)
Malignantmesothelioma Pleural Calcification
Loculatedpleuraleffusion A . IN FE C TION
Lymphoma 1. H eal edempyema
2. Tuberculosis (and Rx for TB: pneumothorax /
oleothorax),histoplasmosis
B . TR A U MA
1. Healedhemothorax= fibrothoraxl
Pleural Thickening . Hx of significant chesttrauma
A. TRAUMA r/ irregularplaquesof calciumusuallyin visceral
1. Fibrothorax(mostcommoncause) pleura
= orgdnizingeffusion/ hemothoraxlpyothorax r/ healedrib fracture
{ densefibrouslayerof approx.2 cm thickness; 2. Radiationtherapy
almostalwayson visceralpleura C. PNEUMOCONIOSIS
r/ frequentcalcificationon inneraspectof pleural 1. Asbestos-related pleuraldisease(most
Peel common):
B . I NF E CT I O N r/ combinationof basilarreticularinterstitial
1. Chronicempyema:over bases;historyof disease(<1/3)+ pleuralthickening
pneumonia;parenchymal scars r/ calcificationsof parietalpleurafrequently
2. Tuberculosis / histoplasmosis: lung apex; diagnostic(diaphragmatic surfaceof pleura,
associatedwith apicalcavity bilateralbut asymmetric)
3. Aspergilloma:in preexisting cavityconcomitant 2. Talcosis: similarto asbestos-related disease
with pleuralthickening 3. Bakelite
C. COLLAGEN.VASCULAR DISEASE 4. Muscovitemica
1. Rheumatoidarthritis:pleuraleffusionfailsto D . H Y P E R C A LC E MIA
resolve 1. Pancreatitis
D. I NHA LA T I O N ALD IS O R D E R 2. Secondaryhyperparathyroidism of chronicrenal
1. Asbestosexposure: lower lateralchestwall; failure/ scleroderma
basilarinterstitialdisease(<25%);thickeningof E . MIS C E LLA N E OU S
parietalpleurawith sparingof visceralpleura 1. Mineraloil aspiration
2. Talcosis 2. Pulmonaryinfarction
E . NE O P LA SM
1. Metastases:oftennodularappearance;may be mnemonic.' "TAFT"
obscuredby effusion Tuberculosis
2. Diffusemalignantmesothelioma Asbestosis
3. Pancoasttumor Fluid(effusion,empyema,hematoma)
F. OTHER Talc
1. Pleuralhyaloserositis
Path: hyalinesclerotictissue= cartilagelike
whitishsugar icingappearance
(Zuckerguss)with occasionalcalcification DIAPHRAGM
2. Mimickedby extrathoracic musculature,
1st + 2nd BilateralDiaphragmatic
Elevation
rib companionshadow,subpleuralfat, focal A. Shallowinspiration (mostfrequent)
scarringaroundold rib fractures B. Abdominalcauses
Obesity,pregnancy,ascites,largeabdominalmass
mnemonic.' "TRlNl" C. Pulmonarycauses
Trauma (healedhemothorax) (1) Bilateralatelectasis
Rheumatoidarthritis(collagenvasculardisease) (2) Restrictivepulmonarydisease(SLE)
Inhalationdisease(asbestosis,talcosis) D. Neuromuscular disease
Neoplasm (1) Myastheniagravis
Infection (2) Amyotrophiclateralsclerosis
442 Radiology Review Manual

UnilateralDiaphragmatic
Elevation C. TRAUMATIC
1. S ubpulmo n ipcl e u rael ffu s i o n 1. Hematoma
^/ dome of pseudodiaphragm migratestowardthe 2. Rib fracture
costophrenicangle and flattens D . IN FE C TIOU S
2. Alteredpulmonaryvolume cellulitis,pyomyositis,
abscess,necrotizing fasciitis
(a) Atelectasis 1. Actinomycosis (parenchymal pleural
infiltrate,
r/ associatedpulmonarydensity effusion,chestwall mass,rib destruction,
(b) Postoperativelobectomy/ pneumonectomy cutaneousfistulas)
r/ rib defects,metallicsutures 2. Aspergillosis,nocardiosis,
blastomycosis,
(c) Hypoplastic lung tuberculosis (rare)
r/ smallhemithorax(moreoftenon the right), 3. Pyogenic:Staphylococcus, Klebsiella
crowdingof ribs,mediastinal shift,absent/ E. CHESTWALL INVASION
smallpulmonaryartery,frequentlyassociated 1. Peripherallungcancer(eg, Pancoasttumor)
with dextrocardia + anomalouspulmonary 2. Recurrentbreastcancer
venousreturn 3. Lymphomatous nodes
3. Phrenicnerveparalysis r/ incompletebordersign (dueto obtuseangle)
(a) Primarylungtumor r/ smoothtaperingborders(tangentialviews)
(b) Malignantmediastinal tumor r/ tumorpediclesuggestsa benigntumor
(c) latrogenic
(d) ldiopathic
r/ paradoxicmotionon fluoroscopy(patientin lateral Lung Diseasewith ChestWall Extension
positionsniffing) A. Infectious
4. Abdominaldisease 1. Actinomycosis
(a) Subphrenicabscess: historyof surgery, 2. Nocardia
accompaniedby pleuraleffusion 3. Blastomycosis
(b) Distendedstomach/ colon 4. Tuberculosis
(c) Interpositionof colon B. Malignanttumor
(d) Liver mass (tumor,echinococcalcyst,abscess) 1. Bronchogenic carcinoma
5. Diaphragmatic hernia 2. Lymphoma
6. Eventration of diaphragm 3. Metastases
7. Traumaticruptureof diaphragm 4. Mesothelioma
associatedwith rib fractures,pulmonarycontusion, 5. Breastcarcinoma
hemothorax 6. Internalmammarynode
8. Diaphragmatic tumor C . B eni gntumor
Mesothelioma, fibroma,lipoma,lymphoma, 1. Capillaryhemangiomaof infancy
metastases 2. Cavernoushemangioma
3. Extrapleural
lipoma
4. Abscess
5. Hematoma

CHESTWALL
ChestWall Lesions ChestWallTumorsin Children
A. EXTERNAL Malignant Tumors of Chest Wattin Children
1. Cutaneouslesion: moles,neurofibroma 0 Morecommonthan benignprimarychestwall
2. Nipple s tumors!
3. Artifact 1. Ewingsarcomaof rib (mostcommon)
B. NEOPLASTIC (a) olderchild: rib involvementin7"/",
1. M es en c h y matul m o r predominant involvement of pelvis+ lower
(a) Lipoma(common):growingbetweenribs extremity
presentingas intrathoracic+ subcutaneous (b) child<10 years: rib involvement in 30%
mass;CT diagnostic) DDx: osteomyelitis, unusual-appearing fracture,
(b) Muscletumor,fibroma callus,directspreadof lunginfection
2. Neur a tu
l mo r 2. Rhabdomyosarcoma
Schwannoma,neurofibroma (mayeroderibs relativelycommonin children+ adolescents
inferiorlywith scleroticbone reaction),neuroma, { sclerosis/ destruction/ scallopingof cortex
neuroblastoma (localextensionto contiguousbone)
3. Vasculartumor ^/ may calcify
Hemangioma, lymphangioma, Metastasesto: lung,occasionally lymphnodes
hemangiopericytoma, aneurysm,falseaneurysm Prognosis: infiltrativegrowthwith high risk of
4. Bone tumor (see also Rib lesion) local recurrence
 Differential Diagnosis af Chest Disorders 443

3. Metastasis BEDSIDECHESTRADIOGRAPHY
(a) Neuroblastoma Unexpectedfindings: in 37-43%
1,0%presentas chestwall mass Changein diagnosticapproach/ therapy: in 27"/"
r/ may calcify lndications:
(b) Leukemia A. Apparatusposition+ complications
4. Askin tumor 1. Malposition of trachealtube (12%)
= PRIMITIVE NEUROECTODERMAL TUMOR r/ tube diametershouldbe 112toZlg of tracheal
= UflcorTlfi]ontumor probablyarisingfrom l umen
intercostalnervesin young Caucasianfemales 2. Malposition of centralvenousline (g%)
Path: neuroectodermal smallcell tumor . idealposition= originof SVC = centralto
containingneuron-specific enolase(may valves (= beyondupper marginof 1st rib)
also be found in neuroblastoma) 3. Malpositionof nasogastrictube
r/ rib destruction(occasionallyarisingfrom rib) in esophagealmalposition
25-63% bronchialintubation
r/ malignantpleuraleffusion
Metastasesto: bone, CNS, liver,adrenal r/ may not be on film if coiledin hypopharynx
DDx: Ewingsarcoma,lymphoma,chestwall 4. Swan-Ganzline (= balloon-directed line)
hamartomain infancy C x: pul monaryi nfarcti on,hemorr hage,
5. Chondro-/ osteosarcoma pseudoaneurysm formation,malposition
quite rare in pediatricpatients 5. Thoracostomytube
r/ break in radiopaquematerial(= msst proximal
side hole)shouldbe intrathoracic
r/ intrafissuralplacementmakestube ineffective
Benign Tumors of Chest Wall in Children B. Cardiopulmonary disease
A. OSSEOUS 1. P ul monary edema
1. Aneurysmal
bonecyst (a) cardiac(hydrostatic)
2. Chondroblastoma r/ usuallycardiomegaly
3. Enchondroma r/ Kerleylines
4. Osteoblastoma { pleuraleffusionfrequent
5. Osteochondroma r/ central/ diffuselung opacity
6. Osteoidosteoma r/ rapid onset + resolution
Often associated with systemic syndrome: (b) noncardiac(permeability)
neurofibromatosis, histiocytosis, r/ cardiomegalyrare
osteochondromatosis r/ Kerleylinesabsent
r/ corticalrib destruction+ soft-tissuemass V pleuraleffusionsunusual
B . S O F TT I S S U E ^/ diffuse/ peripherallung opacity
1. Lipoma r/ delayedonset + resolution
2. Hem a n g i o ma 2. Pleuraleffusion
3. Lymphangioma ^/ homogeneous densityover lowerlung
4. Teratoma ./ ttuiOover apexi in fissures
r/ intrafissuralpseudotumor
! not visiblein 30/"
3. Atelectasis
. mostcommonCXR abnormalityin ICU
PancoastSyndrome ./ lobar/ segmentalversus platelike
= sup€fiorsulcustumor invadingbrachialplexus 'V tett lung base (mostfrequent)
+ sympatheticstellateganglion { rapidtemporalchangepossible
CLI NI CA LT RI A D : 4. Alveolardisease= pflournorlio
1. lpsilateral arm pain . in 10/" of ICU patients,607"with ARDS
2. Musclewastingof hand impossibleDDx: ARDS,lobaratelectasis
3. Hornersyndrome= enophthalmos, ptosis,miosis, 5. Air leak (in 4-1 5/. ol ventilatedpatients)
anhidrosis r/ anteromedial / subpulmoniclocation
Cause: lung cancer(mostcommon),breastcancer, 6. Lungtrauma
multiplemyeloma,metastases, lymphoma, 7. Thoracicbleeding
mesothelioma 8. Mediastinal disease
444 Radiology Review Manual

FUNCTIONAND ANATOMYOF LUNG

20 mm

ap-P

RUL 1&3
post 3

2 ant
ant 2

25 mm

SS-RLL 6 sup
RML lat 4 LINGULA
SS-LLL 6
med 5

RLL LLL
7 10
10 mb
pb
pb

Bronchopulmonary Anatomy
A o = aortic arch RMS = right mainstem bronchus (2nd order bronchus)
A z = azygosvein LMS = left mainstem bronchus
T = trachea (lst order bronchus) IM = intermediate bronchus
SS-RLL = superior segmentright lower lobe SS-LLL = superior segmentleft lower lobe

RUL = right upper lobe LUL = left upper lobe (3rd order bronchus)
1 - apical l&3 = apicoposteriorsegment
2 = anterior 2 = anterior (4th order bronchus)
3 - posterior 4 = superior lingula
RML = right middle lobe J = inferior lineula
4 = lateral
J = medial
RLL = right lower lobe LLL= left lower lobe
6 = superior 6 = superior
7 = mediobasal 7&8 = anteromedial
$ = anterobasal 9 = laterobasal
9 = laterobasal l0 = posterobasal
10 = posterobasal
Order of lower lobe bronchi in frontal projection from lateral to medial:
"ALPm" = Anterior-Lateral-Posterior-medial
mnemonic
 Functionand Anatomyof Lung 445

lb int \
3b su \
lb ext \

2b sup

2b inf
-lvILB.-

-l
\4a
4b
RLLB

I
\\/
lb 7a 8a
8b-'
\
/-
Right Lung Left Lung
l- - RULB = right upper lobe bronchus LULB = left upper lobe bronchus
IB = intermediate bronchus CB = culminal bronchus
MLB = middle lobe bronchus LB = lingular bronchus
RLLB = right lower lobe bronchus LLLB = left lower lobe bronchus
BT = basilar truncus BT = basilar truncus
Br = subsuperior subsegment B* = subsuperiorsubsegment

Bronchial Tree in Lateral Projections

AIRWAYS ! earlyoriginof apicoposterior

LUL bronchus(less
Embryologyof Airways common)
first 5 weeks GA lung buds grow from ventralaspectof
primitiveforegut(fromcaudalend of Accessory Cardiac Bronchus
laryngotracheal grooveof primitive = true supernumerary anomalousbronchus
pharyngealfloor);pulmonaryagenesis M : F= 2 . 8 : 1
5th week GA trachea+ esophagusseparate r/ arisesfrom medialwall of bronchusintermedius
5-16 weeks formationof tracheobronchial tree priorto originof apicalsegmentalRLL bronchus
with bronchi,bronchioles, alveolar r/ caudalcoursetowardpericardium
ducts, alveoli;bronchogeniccyst r/ blind-endingpouchi ventilationof an accessory
(= abnormalbudding); pulmonary lobe
hypoplasia (= fewer than expected ParacardiacBronchus
bronchi) = oofrTl?lbronchusarisingfrom medialaspectof
16-24 weeks dramaticincreasein number lower lobe
+ complexityof airspacesand blood Prevalence: 5/" of patients
vessefs; small airways + reduction in
number and size of acini Airway
= cohductingbranchesfor the transportof air;
AnomalousBronchialDivision -300,000 branchingairwaysfrom tracheato
Tracheal Bronchus bronchiolewith an averageof 23 airwaygenerations
= bronchusof variablelengtharisingfrom lower Definition:
trachea bronchus = cartilagein wall
./ blind-ending
pouch/ aerationof a portionor all of the bronchiole = absenceof cartilage(after6 to 20
RUL divisionsof segmentalbronchus)
446 Radiology Review Manual

att n( hl , LSPV
RSPV_-Q\4'\' \I+LUL-PA

post , '((
)LMB

Level of Apical Segmental Bronchus Level of Right Upper Lobe Bronchus Level of Bronchus Intermedius

Level of Right Middle Lobe Level of Left Superior Segmental Level of Lower Lobe Bronchi

Anatomy of BronchovascularDivisions
Cross-sectional
Right Left
ant = anterior RUL pb - posterobasal RLL amb = anteromediobasalLLL ap-p = apicoposterior LUL
ap = apical RUL post = posterior RUL lb = laterobasal LLL LLL = left lower lobe
BI = bronchus intermedius RLL = right lower lobe LMB = left main bronchus LUL = left upper lobe
lat = lateral RML RML = right middle lobe pb = posterobasalLLL s-LLL= superiorsegment
mb = mediobasal RLL RUL = right upper lobe
med = medial RML s-RLL= superior segment ae = azygoesophagealrecess

RIPV / LIPV = right / left inferior pulmonary vein RIPA / LIPA = ngft lleft inferior pulmonary artery
RPA / LPA = nght lleft pulmonary artery RLL-PAs / LLL-PAs = nght lleft lower lobe pulmonary arteries
RUL-PA / LUL-PA = right / left upperlobepulmonaryartery RSPV / LSPV = right / left superior pulmonary vein

- membranous bronchiole= purelyair conducting HRCTof normallung(windowlevel-700 HU, window

- respiratory bronchiole= containingalveoliin w i dth1,000-1,500):
their walls { -azs + 18 HU at inspiration;
- lobularbronchiole= suppliessecondary i/ -oeo t 43 HU at expiration
pulmonarylobule;may branchinto3 or more ^/ atn orderbronchivisible = bronchi>2 mm in
terminalbronchioles diameter
- terminalbronchiole= lastgenerationof purely 0 Normallobularbronchioles not visible!
conductingbronchioles; each supplyingone
acinus Acinus
small airuvays= diameter<2 mm = sffioll = functionallymost importantsubunitof lung = sll
cartilaginous bronchi+ membranous parenchymaltissuedistalto one terminalbronchiole
and respiratorybronchioles;account comprising2-5 generationsof respiratorybronchioles
tor 25/" of airwayresistance + alveolarducts + alveolarsacs + alveoli
large airuvays= diameter>2 mm; accountfor 75h of . gas exchange
airway resistance r/ radiologicallynot visible
 FunctionandAnatomyof Lung 447

superior

Aortic Arch Level Left Pulmonary Artery Level

Right Pulmonary Artery Level Cardiac Ventricular Level

Cross-sectionalAnatomy of Lung Segments

[PrimaryPulmonary
Lobule] H R C T:
= alveolarduct + air spacesconnectedwith it r/ barelyvisiblefine linesof increasedattenuationin
contactwith pleura(= interlobular septa);best
SecondaryPulmonaryLobule developedin subpleuralareasof
= REID
LOBULE - UL + ML: anterior+ lateral+ juxtamediastinal
= sm?llestportionof lung surroundedby connective - LL: anterior+ diaphragmatic regions
tissuesepta = basicanatomic+ functionalpufmonary r/ dottit<eI linear/branching structures(= pufmonary
unitappearingas an irregularpolyhedron; separated arterioles)nearcenterof secondarypulmonary
from each otherby thin fibrousinterlobular
septa(100 lobule3-5 mm from pleura
pm); suppliedby 3-5 terminalbronchioles; contains
3-24 acini
Size: 10-25 mm in diameter
. visibleon surfaceof lung Surfactant
Contents: = surface-activematerialessentialfor normal
- centrally= 1s5u1tr core: branchesof terminal pulmonaryfunction
bronchioles(0.1 mm wall thicknessis belowthe Substrate:
resolutionof HRCT)+ pulmonaryarterioles(1 mm) phospholipids (phosphatidytchotine,
- peripherally (in interlobular
septa): pulmonaryvein phosphatidylglycerol),
otherfipids,cholesterol,
lung-
+ lymphvessels specificproteins
448 RadiologyReviewManual

interlobular terminal
septum bronchiole

respiratory
bronchiole:

lst
order

2nd
order

3rd
order

alveolar
ducts

Terminal Airways
The SecondaryPulmonary Lobule within the Secondary Pulmonary Lobule

Production: (b) musculararteries(50-1,000pm)

type ll pulmonaryalveolisynthesize+ transport accompanysubsegmentalairways+ terminal
+ secretelung surfactant;earliest productionaround bronchioles
18thweek of gestation(in amnioticfluid by r/ provideactivevasodilatation+ constriction
22nd week of gestation) (c) arterioles(15-150pm)
Action: accompanyrespiratorybronchioles+ alveolarducts
increaseslungcompliance,stabilizesalveoli, (d) capillarynetworkin alveolarwalls
enhancesalveolarfluid clearance,reversessurface (e) venules
tension,protectsagainstalveolarcollapseduring (f) pulmonaryveins
respiration,protectsepithelialcell surface,reduces coursethroughinterlobular fibroussepta
openingpressure+ precapillarytone 2. Bronchialcirculation(1% of cardiacoutput)
Origin: thoracicaorta,intercostalarteries(2 vessels
, for each lung)
LungInterstitium Suppty: esophagus,trachea,visceralpleura,lymph
nodes,extra-and intrapulmonary airways,
Division Components bronchovascular + neuralbundles,vasa
vasorumof Pulmonarycirculation
Axial sheaths
bronchovascular Course: tortuouspath alongperibronchialsheathof
lymphatics mainstemainrvayto terminalbronchioles
3. Anastomoses:throughmicrovascular connections;
Middle(parenchymal) alveolarwall(interalveolarseptum) r/ bronchialblood flow may increaseby 300% in the
weeksfollowingpulmonaryarteryembolization
Peripheral pleura
subpleuralconnectivetissue LUNGFUNCTION
interlobularsepta(enclosing LungVolumes& CaPacities
pulmonaryveins,lymPhatics, 1. Tidalvolume(TV)
walls of corticalalveoli) of gas movingin and out with each
= €trT'louflt
respiratorycycle
2. Residualvolume(RV)
PULMONARY CIRCULATION of gas remainingin the lung after a
= €lrTlouflt
pulmonary
1. Primary circulation maximalexpiration
pulmonaryarteriestravelalong lobar+ segmental 3. Total lung caPacitY(TLC)
bronchidown to subsegmental levelmatchingcaliber = gas containedin lungat the end of a maximal
of airways inspiration
(a) largeelasticpulmonaryarteries(500->1,000pm) 4. Vital capacity(VC)
accompanylobar+ segmentalbronchimatching = orrtourtof gas that can be expiredafter a maximal
caliberof airways inspirationwithoutforce
 FunctionandAnatomyof Lung 449

VC TLC 3. Flow-volume loop

= gas flow is plottedagainstthe actualvolumeof
A A
t l lungat whichthis flow is occurring
t l Usefulin identifyingobstructionin large airways
t l
t l
t l B. Resistancein small airways
t l Closingvolume= lungvolumeat whichdependent
r { l
l r l lung zones ceaseto ventilatebecauseof ainvay
I t l closurein smallairwaydiseaseor lossof lungelastic
l r l
recoil
l r l
. decreasein FEV,MMFR,MBC:
Y j r
r l (a) expiratoryairwayobstruction(reversibleas in
t l spasmodicasthma/ irreversibleas in
YV emphysema)
3 2 1 0 F R C (b) respiratorymuscleweakness
Lung Volumes and Capacities
DiffusingCapacity
= r€lteof gas transferacrossthe alveolocapillary
5. Functionalresidualcapacity(FRC)
= volumeof gas remainingin lungsat the end of a membranein relationto a constantpressure
quietexpiration differenceacrossit; measuredby the carbon
monoxidediffusionmethodDL^^.
Technique:
Changes In Lung Volumes " patientinspiresmaximallya gas with a knownsmall
A. DECREASED VC: concentrationof CO
1. Reduction in functioning lungtissuedueto " holdshis/herbreathfor 10 secondsand then slowly
(a)space-occupying process (eg,pneumonia, expiresto residualvolume(RV)
infarction) " an aliquotof the end-expired(alveolar)gas is
(b)surgical removal of lungtissue analyzedfor amountof CO absorbedduringbreath
2. Process reducing overallvolumeof thelungs
(eg,diffusepulmonary fibrosis) Measurement: in mL of CO absorbed/min/mm Hg
3. Inabilityto expandlungsdueto Reduction:
(a)muscular weakness (eg,poliomyelitis) 1. Ventilation / perfusioninequality:lessCO is taken
(b)increase in abdominal volume(eg,pregnancy) up by poorlyventilatedor poorlyperfusedareas
(c) pleurafeffusion
(eg,emphysema)
B. INCREASED FRCandRV: 2. Reductionof total surfacearea (eg, emphysema,
characteristic of airtrappingandoverinflation surgicalresection)
(eg,asthma, emphysema) 3. Reductionin permeability fromthickeningof
Associatedwith: increased TLC alveolarmembrane(eg,cellularinfiltration, edema,
r/ normallevelof inflationto 8 posteriorribs interstitialf ibrosis)
C. DECREASED FRCandRV: 4. Anemiawith lackof hemoglobin
1. Process reducing overallvolumeof lungs
(eg,diffusepulmonary fibrosis)
2. Process thatoccupies volumewithinalveoli
(eg,alveolar ArterialBloodGasAbnormalities
microlithiasis) . decreasedpulmonaryarterialO,:
3. Processthatelevates diaphragm (eg,ascites,
pregnancy), 1. Alveolarhypoventilation
usuallyassociated withdecreased
2. lmpaireddiffusion
TLC
3. Abnormalventilation/perfusion
ratios
4. Anatomicshunting
Flow Rates
A. Spirometric measurements: . elevatedpulmonaryarterialCO,:
1. Forcedexpiratory volume(FEV) 1. Alveolarhypoventilation
= ornoufltof air expiredduringa certainperiod
2. lmpairedventilation/ perfusionratios
( u s u a l l y+l 3 s e c ) ;
Normalvalues:FEV'= 83%;FEV3= g7"/o
2. Maximalmidexpiratory flowrate(MMFR) V/QInequality
= ?rllour^ttof gasexpiredduringthe middlehalfof A. NORMAL
forcedexpiratory volumecurve(largelyeffort (a) bloodflow decreasesrapidlyfrom base to apex
independent) (b) ventilationdecreasesless rapidlyfrom base to
lndicator of smallairwayresistance apex
450 Radiology Review Manual

0 V/Q is low at base and high at aPex Thymic weight:

0 PulmonaryarterialO, is substantiallyhigherat increasesfrom birthto age 11-12 years(22 ! 13 g in
apex neonate,34 + 15 g at puberty);ratioof thymicweightto
0 PulmonaryarterialCO2is substantially higherat body weightdecreaseswith age (involutionafter
base puberty,total fatty replacementafter age 60)
B. ABNORMAL 0 Atrophiesunderstress(dueto increasein
chieflyresultingfrom non-/ underventilatedlung endogenoussteroids)
regions(non-/ underperfused regionsdo not result Extent: from manubriumto 4th costalcartilage;may
in bloodgas disturbances) bulge into neck/ extenddown to diaphragm
CXR:
{ prominentnormalthymusvisiblein 50% of neonates
Compliance + infants0-2 years of age
= relationship of the changein intrapleural pressureto r/ notchsign = indentation at junctionof thymus+ heart
the volumeof gas that movesintothe lungs { sailsign = triangulardensityextendingfrom superior
A. DECREASEDCOMPLIANCE mediastinum, usuallYon rightside
edema,fibrosis,granulomatous infiltration { wave sign = rippledundulatedlateralborderdue to
B . I NCRE A SE D C O MP L IAN C E indentation by ribs
emphysema(faultyelasticarchitecture) { shapechangeswith respiration + position
./ neigfrtof diaphragmat TLC can providesome DDx: mediastinal mass,upperlobepneumonia,
indicationof lungcompliance,particularly valuablein atelectasis
sequentialroentgenograms for comparisonin: CT:
1. Diffuseinterstitial pulmonaryedema { measurement (perpendicular to axisof aorticarch):
2. Diffuseinterstitial pulmonaryfibrosis <18 mm beforeage20; <13 mm afterage 20
{ triangularshape like an arrowhead(62%),bilobed
(32%),singlelobe (6%)
M E DI A S T I NUM ./ musculardensityof 30 HU (beforepuberty)
"Quod per medium ./ ttat / concaveborderswith abundantfat (after
Terminology:Spigelius(1578-1626):
stat"= what sits in the middle puberty)
A . S UP E RI O RM ED IAS T IN AC L O MP AR T M EN T r/ detectedin 83% of subjects<50 years of age;
= thoracicinlet in 17'/. of subjects>50 years of age
B . I NF E RI O RM ED IAS T IN ACL OMP AR T M EN T US (supra-,trans-,parasternal approachin infants):
(a) anteriormediastinum { homogeneous finelygranularechotexture with some
= retrosternalregion echogenicstrands
(b) middlemediastinum { mildlyhypoechoicrelativeto liver,spleen,thyroid
= visceralregion r/ smoothwell-defined margin(dueto fibrouscapsule)
(c) posteriormediastinum ^/ nypo-i avascular
= containsesophagus,descendingaorta,
paraspinalregion
EctopicThymus
r/ solid mass
THYMUS r/ cysticmass (= endodermal-lined cavityof
Origin: residualthymictissuein neck in 1.8-21'/" thymopharyngeal duct/ cysticdegenerationof Hassall
Embryogenesis: corpusclesor glandularepithelium)
dorsal+ ventralwings of 3rd (and possibly4th) (1) Unilateral failureof thymicprimordiumto descend
branchialpouchbeginto form the primordiaof the ./ neckmassof thymictissueon one side of neck
inferiorparathyroidand thymicglandsat 4th-5th week absenceof normalthymiclobe
r/ ipsilateral
of gestation;both glandsseparatefrom pharyngealwall ri parathyroid tissuewithinectopicthymus
+ migratecaudallyand mediallywiththe thymuspulling (2) Smallrestof thymusleftbehindwithin
the inferiorparathyroid glandsalongthe thymopharyngeal tractduringmigration
thymopharyngeal tract;thymicprimordiumfuseswith its ^/ neck mass
contralateralcounterpartinferiorto thyroidgland;thymic ./ normallypositionedbilobedthymus
tail thins + disappearsby 8th week (3) Atypicallocation:trachea,skull base, intrathyroidal
 ChestDisorders 451

CHESTDISORDERS

ACUTEEOSINOPHILIC
PNEUMONIA <100 Cytomegalovirus, Herpessimplexvirus,
Etiology: idiopathic(no evidenceof infection/ exposure Mycobacteriumaviumcomplex(intestinal
to potentialantigens)with abruptincreasein CMV + MAI infection)
lung cytokines <50 AIDS-related lymphoma
Age: 32t 17 years;M > F Prognosis; mediansurvivalwith CD4 lymphocytecount
Histo: eosinophilicinfiltrates+ pulmonaryedema (from <50 cells/mm3 is 12 months
releaseof eosinophilicgranulesafteringvascular Transmissionby: intimatesexual contact,exposureto
permeability) contaminatedblood/ bloodybody
' acute respiratoryfailurein previouslyhealthyindividuals secretions
. markedlyelevatedfevelsof eosinophilsin Groups at risk:
bronchoalveolar lavagefluid 1. Homosexualmales(74%)
. no peripheraleosinophilia 2. lV drug abusers(16%)
. acute febrileillnessof 1-5 day's duration,myalgia 3. Recipientsof contaminatedblood products(S%)
r/ bilateralinterstitial+ air space opacities 4. Sexualpartnerof drug abuser+ bisexualman
r/ pleuraleffusion 5. Infantsbornto womaninfectedwithAIDS virus
Rx: lV corticosteroids 0 HIV antibodiespresentin >50% of homosexuals
Dx: bronchoscopywithbronchopulmonarylavage + 90o/oof lV drug abusersl
DDx: chroniceosinophilic pneumonia(infiltrates
with 0 Rateof heterosexual transmission is increasing!
peripheralpredominance)

AIDS CMV
= A CQ UI REIDM M U NDE EF IC IEN C
SYY N D R OME
= ultimatelyfatal diseasecharacterizedby HIV MAI
seropositivity,
specificopportunisticinfections,specific Lymphoma
malignantneoplasms(Kaposisarcoma,Burkitt
lymphoma,primarylymphomaof brain) Kaposisarcoma
= patientwith CD4 cell count <200 cells/pl (normalrange,
Fungal infection
800-1,200cells/pl)
lncidence: 2 millionAmericansare infectedwith HIV Pneumocystiscarinii pneumonia
+ 270,000haveAIDS (estimatein 1993);
M. tuberculosisatypical pattem
>50% developpulmonarydisease
Organism; humanimmunodeficiency virus(HlV) M. tuberculosisprimary pattem
= humanT-celllymphotropicvirus type lll
M. tuberculosisreinfection pattern
(HTLVlll) = lymphadenopathy-associated
virus (LAV) Bacterial pneumonia

Pathomechanism:
HIV retrovirusattachesto CD4 moleculeon surfaceof 600 400 200 100 50 0
T-helperlymphocytes+ macrophages+ microglialcells;
aftercellularinvasionHIV geneticinformation CD4 LymphocyteCountversusPulmonaryDisease
is
incorporated intocell'schromosomalDNA;virus
remainsdormantfor weeks to years;after an unknown CIinical classification :
stimulusfor viralreplication
CD4 lymphocytes are group I acuteHIV infectionwith seroconversion
destroyed(normalrangeof 800-1,000cells/mm3) and groupll asymptomatic HIV infection
othersbecomeinfectedleadingto impairmentof the grouplll persistentgeneralized lymphadenopathy
immunesystem;CD4 lymphocytenumberand function grouplV otherHIV disease
decreases(at an approximaterate of 50-g0 cells/year) - subgroupA constitutional
disease
- subgroupB neurologicdisease
AIDS-defining illnessrelatedto CD4 T-lymphocyte
count - subgroupC secondaryinfectiousdisease
[cells/pl]: - subgroupD secondarycancers
<400 extrapulmonaryMycobacteriumtuberculosis, - subgroupE otherconditions
Kaposisarcoma
<200 Candidaalbicans(thrush,hairy leukoplakia), AIDS-definingpulmonaryconditions(CDC, 1987):
Histoplasma capsulatum,Cryptosporidium (1) Tracheal/ bronchiali pulmonarycandidiasis
species,Pneumocystis cariniipneumonia, (2) PulmonaryCMV infection
non-Hodgkin lymphoma (3) Herpessimplexbronchitis/ pneumonitis
<150 cerebraltoxoplasmosis (4) Kaposisarcoma
452 Radiology Review Manual

/ Burkittlymphoma
(5) lmmunoblastic r/ atypicalTB patternwith diffusereticulari
cariniipneumonia
(6) Pneumocystis nodularinfiltrates(CD4 lymphocytecount of
<50 cells/mm3)
A. LYMPHADENOPATHY r/ adenopathyof low attenuationwith rim
Cause: enhancement on CECT
= HIV adenopathy
reactivefollicularhyperplasia (b) M. avium-intracellulare (5%)
(50%),AIDS-related lymphoma (20oh), . in patientswith low CD4 lymphocytecountonly
mycobacterialinfection(17"/"),Kaposisarcoma r/ diffusebilateralreticulonodularinfiltrates
infectionwith
(10/"), metastatictumor,opportunistic ./ adenopathy, miliarydisease
multipleorganisms,drug reaction (c) M. kansasiiand others
Location: mediastinum,axilla,retrocrural 4 . Bacterialpneumonia(5-30%):
(a) Haemophilus influenzae, Streptococcus
I ST ICINF EC T IO N
B . O P P O RT UN pneumoniae, Staphylococcus aureus
accountsfor majorityof pulmonarydisease r/ frequentlymultitobardistribution
0 Pulmonaryinfectionis oftenthe firstAIDS-defining . bacteremia(common)
illnes s ! (b) Nocardiapneumonia(<5%)
1. Pneumocystis cariniipneumonia(60-80%) usuallyoccursin cavitatingpneumonia
20-40% develop>1 episodeduringdisease r/ segmental/lobaralveolarinfiltrate
. CD4+T helperlymphocytecell count<200imm3 + cavitation+ ipsilateralpleuraleffusion
. subacuteinsidiousonsetwith malaise,minimal (c) Rhodococcus equii(aerobic,Gram negative)
cough r/ cavitarypneumonia
ri bilateralground-glassinfiltrateswithouteffusion/ (d) Bartonellahenselae(Gram negative)
adenopathy = bacillaryangiomatosis
^/ bilateralperihilarinterstitial infiltrates . cutaneouslesions
^/ diffusebilateralalveolarinfiltrates ./ nigntyvascularsmallpulmonarynodules
^/ frequentlyassociatedwith pneumatoceles r/ dramaticenhancement of enlargedlymphnodes
r/ apicalpredominance (in patientson prophylactic 5 . C MV pneumoni a
aerosolized pentamidine) most frequentinfectionfound at autopsy(49-81%)'
Mortality: in 25o/"fatal diagnosedbeforedeathin only 13-24/";
2. Fungaldisease(<5%) high combinedprevalencewith Kaposisarcoma
(a) Cryptococcusneoformanspneumonia (2-15%) ./ bilateralhazy infiltrates,focal nodules,masses
usuallyassociatedwith brain/ meningeal ! bronchiectasis / bronchialwall thickening
disease 6 . Toxoplasmosis
^/ segmentalinfiltrate+ superimposed ri coarseinterstitial/ nodularpattern
pulmonarynodules+ lymphadenopathy r/ focal areas of consolidation+ cavities
t pleuraleffusion DDx: indistinguishable from PCP
(b) Histoplasma capsulatum
r/ typicallydiffusenodularI miliarypatternat c. TU MOR
time of diagnosis 1. Kaposisarcoma(15%)
./ normalCXR in up to 35% Location: lung involvement(20%)precededby
(c) Coccidioides immitis widespreadskin + organinvolvement
./ diffuseinfiltrates+ thin-walledcavities Site: peribronchovascular (best
distribution
(d) Candidaalbicans appreciatedon CT)
(e) Aspergillus: lesscommon+ less invasivedue to { numerousfluffyill-definednodules/ asymmetric
relativepreservation of neutrophilicfunction clustersin a vagueperihilardistribution
invasivepulmonaryaspergillosis r/ interlobular septalthickening
chronicnecrotizingaspergillosis { pleuraleffusion(30%)
necrotizingtracheobronchitis ./ lymphadenopathy (10-35%)'latein disease
obstructingbronchopulmonary aspergillosis 2. AIDS-related lymphomaof B-cellorigin(2-5%)
3. Mycobacterialinfection(107"per year): primarilyimmunoblasticNHL / Burkittlymphoma/
(a) M. tuberculosis (increasing frequency): non-Burkitt lymphoma;occasionally Hodgkin
0 AIDS patientsare 500 times morelikelyto disease
becomeinfectedthan generalpopulation! Location: pulmonaryinvolvement(8-157"),CNS,
r/ postprimaryTB patternwith upper-lobe Gl tract,liver,sPleen,bone marrow
cavitatinginfiltrate(CD4 lymphocytecount of Site: primarilyextranodal
200-500 cells/mm3) r/ pleuraleffusion(50%)
r/ primaryTB patternwith lung infiltrate/ lung ./ nilar/ mediastinaladenopathy(25%);+ axillary/
masses+ hilar/ mediastinal supraclavicu lar / cervicaladenopathy
lymphadenopathy + pleuraleffusion(CD4 r/ solitaryi multiplewell-defined pulmonarynodules
lymphocytecount of 50-200 cells/mm3) (occasionallywith doublingtime of 4-6 weeks)
 ChestDisorders 453

^/ diffusebilateralreticulonodular heterogeneous (e) 7-14 days: extensivefibroblastic proliferation

in
opacities interstitium+ withinalveoli,rapidly
r/ alveolarinfiltrates progressing collagendeposition
r/ paraspinalmasses + fibrosis;almostinvariably
D . LY M P HO I DI N T E R S T IT IAL
PN EU M O N IT ]S associatedwith infection
Age: in children<13 yearsof age Predisposed:
E . S E P T I CE M B OL I hemorrhagic / septicshock,massivetrauma(pulmonary /
F. PREMATUREDEVELOPMENT OF BULLAE(40%) generalbody),acutepancreatitis, aspirationof liquid
with disposition to spontaneous pneumothorax gastriccontents,heroine/ methadoneintoxication,
massiveviralpneumonia,traumaticfat embolism,near-
A|DS-related
Complex(ARC) drowning,conditionsleadingto pulmonaryedema
= GENERALIZED LYMPHADENOPATHY SYNDROME mnemonic; "DICTIONARIES"
= prodromalphaseof HIV seropositivity, generalized Disseminatedintravascularcoagulation
lymphadenopathy, CNS diseasesotherthanthose lnfection
associatedwith AIDS Caughtdrowning
Time interval: approximately10 years between Trauma
seroconversion + clinicalAIDS Inhalants:smoke,phosgene,NOo
. weightloss,malaise,diarrhea O, toxicity
. fever,nightsweats,lymphadenopathy Narcotics+ otherdrugs
. lymphopenia with selectivedecreasein helperT-cells Aspiration
Radiation
Includespancreatitis
Emboli: amnioticfluid,fat
ADULTRESPIRATORY
DISTRESS Shock:septic,hemorrhagic, cardiogenic,
anaphylactic
SYNDROME
= SHOCKLUNG= POSTTRAUMATIC PULMONARY
I NS UF F I CI E N=CHYEMOR R H AGIC L U N GSY N D ROME . initiallyfew / no symptoms
= RESPIRATOR LUNG= STIFFLUNGSYNDROME = PUMP ' rapidlyprogressivedyspnea,tachypnea,cyanosis
LUNG= CONGESTIVE ATELECTASIS = OXYGEN TOXICITY . hypoxiaunresponsiveto oxygentherapy(due to
= SeVereunexpectedlife-threatening acute respiratory arteriovenous shunting)
distresscharacterizedby abruptonset of marked . no increasein pulmonarycapillarypressure
dyspnea,increasedrespiratoryeffort,severehypoxemia Stages (often overlapping):
associatedwith widespreadairspaceconsolidation 1st (exudative)stage
Etiology: interstitial
edemawith high proteincontentrapidly
= firost severe form of permeabilityedema associated fillingthe alveolarspaces
with diffusealveolardamage associatedwith hemorrhage+ ensuinghyaline
A . P RI M A RY= D IR E C TIN J U R Y membraneformation
= ARDS due to underlyingpulmonarydisease r/ interstitial
edema(withhighproteincontent)initially
= eXposUre to chemicalagents,infectious r/ perihilarareas of increasedopacityfollowingrapidiy
pathogens,gastricfluid,toxicgas r/ widespreadalveolarconsolidationwith
Associatedwith: pulmonaryconsolidation predominantly peripheralcorticaldistribution
B. SYSTEMICCONDITION ri air bronchogram
= ARDS due to extrapulmonary disease r/ gravitationalgradient(bestseen on CT) due to
= Systemicbiochemicalcascadecreatingoxidating dependentatelectasis
agents,inflammatory mediators,enzymesduring DDx: cardiogenicedema (cardiomegaly, apical
sepsis,pancreatitis,severetrauma,blood vasculardistribution, Kerleylines)
transfusion 2nd (proliferative) stage
Associatedwith: interstitialedema,alveolar organization of fibrinousexudate
collapse subsequentregeneration of alveolarlining
Histo: + thickeningof alveolarsepta
(a) up to 12 hours: fibrin+ plateletmicroemboli r/ inhomogeneous areasof ground-glass opacities
(b) 12-24 hours: interstitialedema 3rd (fibrotic)stage
(c) 24-48 hours: capillarycongestion,extensive varyingdegreesof scarring
interstitial+ alveolarproteinaceous r/ subpleuraland intrapulmonary cysts
edema+ hemorrhage, widespread Cx: pneumothorax
microatelectasis, destructionof type CXR:
I alveolarepithelialcells r/ ttlOcardiomegaly / pleuraleffusion
(d)s-7 days: extensivehyalinemembrane - up to 12 hours:
formation,hypertrophy r/ characteristic 12-hourdelaybetweenclinical
+ hyperplasiaof type ll alveolar onset of respiratoryfailureand CXR
l i n i n gc e l l s abnormalities
454 Radiology Review Manual

- 12-24 hours: 0 20% of homozygotic individuals neverdevelop

r/ patchyill-definedopacitiesthroughoutboth lungs clinicallyapparentemPhYsema
- 24-48 hours: . chronicsputumProduction (50%)
{ massiveairspaceconsolidationof both lungs { severepanacinaremphysemawith basilarpredominance
- 5-7 days: (dueto gravitational of pulmonaryblood
distribution
{ consolidation becomesinhomogeneous flow):
(resolutionof alveolaredema) r/ reductionin size + numberof pulmonaryvesselsin
{ localareasof consolidation (pneumonia) lowerlobes
- >7 days: { redistributionof bloodflow to unaffectedupper lung
^/ reticular/ bubblylung pattern(diffuseinterstitial ZONES
+ airspacefibrosis) { bullaeat both lungbases
{ markedflatteningof diaPhragm
Complication of continuouspositivepressureventilation { minimaldiaphragmatic excursion
(= barotrauma) ri multilobarcysticbronchiectasis(40%)
Path: { hepatopulmonarY sYndrome
(a) ruptureof alveolialongmarginsof interlobular Cx: hepaticcirrhosis(in homozygoticindividuals)
septa + vascularstructures(= parenchymal 0 Most commonmetabolicliverdiseasein children
pseudocyst) Prognosis: 15-20-yeardecrease in longevityin smokers
(b) air dissectionalonginterlobular septa relativeto nonsmokers
+ perivascularspaces (= interstitialemphysema)
./ "pseudoclearing" of RDS ALVEOLAR MICROLITHIASIS
(c) interstitialair rupturinginto pleuralspace = vefy rare disease of unknownetiologycharacterizedby
(= pneumothorax) i intomediastinum myriadof calcospherites (= tiny calculi)withinalveoli
(= pneumomediastinum) / into pericardial
cavity Age peak; 30-50 years; begins in early life; has been
(= pneumoperitoneum) i n utero; M:F = 1:1; i n 50% f am ilial
i denti fi ed
(d) interstitialair rupturinginto peritonealspace (restrictedto siblings)
(= pneumoperitoneum) pneumopericardium) / . usuallyasymptomatic(70%)
retroperitoneal spac€ (=pneuffioretroperitoneum) . dyspneaon exertion(reductionin residualvolume)
(e) air dissectingintoskin (= subcutaneous . cyanosis,clubbingof fingers
emphysema) . strikingdiscrepancybetweenstrikingradiographic
(f) air rupturingintovessel(= gas embolism) findingsand mildclinicalsymptoms
{ mottledair opacitiesoften outliningbronchovascular . NORMALserumcalcium+ phosphoruslevels
bundles { very fine,sharplydefined,sandlikemicronodulations
r/ largesubpleuralcystswithoutdefinablewall usuallyat (< 1 mm)
diaphragmatic + mediastinal surfacecompressing r/ diffuseinvolvement of both lungs
adjacentlung ./ intenseuptakeon bone scan
Rx: mechanicalventilatoryassistancewith positiveend- Prognosis:
expiratorypressure(to increaseoxygendiffusion) (a) late developmentof pulmonaryinsufficiency
secondaryto interstitialfibrosis
(b) diseasemay becomearrested
ALPHA.I ANTTTRYPSIN DEFICIENCY (c) microlithsmay continueto form/ enlarge
= ta;taautosomalrecessivedisorder DDx: "mainline"pulmonarygranulomatosis = lV abuseof
alpha-1antitrypsin(glycoprotein) is to >90% synthesized talc-containing drugs such as methadone (rarelyas
in hepatocytes+ releasedinto serum numerous+ scarring+ loss of volume)
Gene: codominantgene expressionon chromosome
14 with >100geneticvariantsof the protein;
most severehepatopulmonarymanifestations ALVEOLAR PROTEINOSIS
resultfrom homozygousP\ZZ phenotype = PULMONARY ALVEOLAR PROTEINOSIS (PAP)
Action: proteolyticinhibitorof neutrophilelastase, = €lccurTlulationof PAS-positivephospholipidmaterialin
trypsin,chymotrypsin, plasmin,thrombin, alveoli(= surfactant)
kallikrein,leukocytic+ bacterialproteases; Etiology: ?; associatedwith dust exposure
neutralizescirculatingproteolyticenzymes (eg,silicoproteinosis identical
is histologically
Modeof injuryfrom deficiency: to PAP),immunodeficiency, hematologic
PMNs + alveolarmacrophagessequesterinto lung + lymphaticmalignancies, AIDS,chemotherapy
during recurrentbacterialinfections+ release Pathophysiology:
neutrophilelastase,whichacts unopposed+ digests (a) overproduction of surfactantby granularpneumocytes
basementmembrane (b) defectiveclearanceof surfactantby alveolar
Age: earlyage of onset(20-30 years); [$;f = 1:1 macrophages
. rapid+ progressivedeteriorationof lung function: Histo: alveolifilledwith proteinaceousmaterial(the
. dyspneain 4th and Sth decade ONLY pure airspacedisease),normalinterstitium
 ChestDisorders 455

Age peak: 30-50 years (age range 2-70 years); usuallyfatal beforeradiographsobtained
M : F= 3 ; 1 may demonstratepulmonaryedema
. asymptomatic(10-20%)
. gradualonset of dyspnea+ cough
AMYLOIDOSIS
. weight loss,weakness,hemoptysis
= diseasecharacterizedby an extracellulardepositof
. defectin diffusingcapacity
proteinaceoustwistedB-pleatedsheet fibrilsof great
r/ "bat-wing"consoiidationoi ground-glasspattern, chemicaldiversity
predominantat bases Histo: protein(immunoglobulin) i polysaccharide
r/ smallacinarnodules+ coalescence + consolidation complex;affinityfor Congo red stain
r/ patchyperipheraliprimarilyunilateralinfiltrates (rare) @ Lunginvolvement
{ reticular/ reticulonodular / linearinterstitialpatternwith lncidence; 1oamyloidosis(in up to 70oh),
, Kerley B lines (late stage) 2" amyloidosis(rare)
r/ slow clearingover weeks or months
A. TRACHEOBRONCHTAL TypE (mostcommon)
r/ slow progression(1/3),remainingstable(2/3) . hemoptysis(mostfrequentcomplaint)
\i NO adenopathy,NO cardiomegaly, NO pleuraleffusion . stridor,cough,dyspnea,hoarseness, wheezing
HRCT : r/ multiplenodulesprotrudingfrom wall of trachea/
{ patchyground-glassopacity largebronchi
r/ smoothseptalthickening ^/ diffuserigid narrowingof a longtrachealsegment
Cx: infections(frequenilysecondaryto poorly r/ prominentbronchovascular markings
functioningmacrophages + excellentculture ri destructivepneumonitis
medium):Nocardiaasteroides(mostcommon), B . N OD U LA RTY P E
mycobacterial, fungal,pneumocystis, CMV A ge: > 60 yearsof age; M:F = 1:1
Prognosis: . usuallyasymptomatic
highlyvariablecoursewith clinicaland radiologic r/ mediastinal/hilaradenopathy
episodesof exacerbation+ remissions r/ solitary/ multipleparenchymal nodulesin a
(a) 50% improvement/ recovery peripheral / subpleurallocation+ central
(b) 30% deathwithinseveralyears underprogression
cafcification / ossification;slow growthover years
Rx: bronchopulmonary lavage r/ + pleuraleffusion
DDx: DDx: metastaticdisease,granulomatousdisease,
(a) duringacutephase: pulmonaryedema,diffuse
rheumatoidlung,sarcoidosis, mucoid
pneumonia,ARDS
impaction
(b) in chronicstage:
C. DIFFUSEPARENCHYMAL TypE (teastcommon)
1. ldiopathicpulmonaryhemosiderosis (boys, Age: >60 years of age
symmetricinvolvement of mid + lowerzones, . usuallyasymptomaticwith normalCXR
progressionto nodular+ linearpattern) . cough+ dyspneawith abnormalCXR
2. Hemosiderosis (bleedingdiathesis) ! widespreadsmallirregulardensities(exclusively
3. Pneumoconiosis interstitialinvolvement)t calcification
4. Hypersensitivity pneumonitis r/ may becomeconfluent+ honeycombing
5. Goodpasture syndrome(morerapidchanges, DDx: idiopathicinterstitial fibrosis,pneumoconiosis
renaldisease) (especially asbestosis), rheumatoidlung,
6. Desquamative interstitialpneumonia(,,ground_ Langerhanscell histiocytosis, scleroderma
glass"appearance,primarilybasilar
+ peripheral)
7. Pulmonaryalveolarmicrolithiasis (widespread ASBESTOS-RELATED
DISEASE
discreteintraalveolarcalcificationsprimarilyin
lungbases,rarefamilialdisease) lasbestos, Greek = inextinguishable = sevef?lfibrous
silicateminerafssharingthe propertyof heat resistancel
8. Sarcoidosis (usuallywith lymphadenopathy)
Substances:
9. Ly m pho m a
aspect(length-to-diameter) ratioeffectscarcinogenicity:
10. Bronchioloalveolar cell carcinoma(morefocal, eg, aspectratioof 32 = 8 pm long,0.25 pm wide
slowlyenlargingwith time)
commercialamphiboles:crocidolite, amosite
commercialserpentines(= nonamphiboles):
AMN I O T I C F LUI D EMB O L IS M
chrysotile(theonly mineralin the serpentine
= rT'toStcommoncause of maternalperipartumdeath group accountingfor >90% of asbestosused in
. dyspnea
. shockduringi afterlabor+ delivery the UnitedStates)
noncommercial contaminating amphiboles:
Pathogenesis;amnioticdebrisentersmaternalcirculation actinolite,
anthophyllite,tremolite
(a) relativelybenign:
ffi ,l'HJlary
embor
ization (1) chrysotile(whiteasbestos)in Canada
(2) anaphylactoidreaction (2) anthophyllite
in Finland,NorthAmerica
( 3) D tC (3) tremolite
456 RadiologyReviewManual
(b) relativelymalignant:
(1) crocidolite(blue/ blackasbestos)in South
Africa,Australia
(2) amosite(brownasbestos)
0 Very fine fibers (crocidolite)associatedwith largest
numberof pleuraldisease!
0 Asbestosfibersup to 100 pm in length
Occupational exposure :
(a) asbestosmining,milling,and processing
(b) insulationmanufacturing, textilemanufacturing,
construction, shipbuilding,gaskets,brakelinings
PleuralDisease
Asbestos-related
1. PleuralEffusion(21%)
= earliestasbestos-related pleuralabnormality,
frequentlyfollowedby diffusepleuralthickening
+ roundedatelectasis
Prevalence: 3/" (increaseswith increasing
levelsof asbestosexposure)
Latencyperiod: B-10 years after exposure
. benign asbestos PleurisY:
. may be associatedwith chest pain (1/3)
. usuallysmallsterile,serous/ hemorrhagic
exudate
{ recurrentbilateraleffusions
{ + plaqueformation
DDx: TB, mesothelioma
2. FocalPleuralPlaques(65%)
= hyalinizedcollagenin submesothelial
parietalpleura
lncidence; most common manifestationof
exposure;6'h of generalpopulationwill
show plaques
Latencyperiod: in 10% after 20 years; in 50%
after 40 years
His t o: d e n s e h y p o c e l l u l a ru n d u l a ti n gcol l agen
"basketweave"
fibersoften arrangedin a
patternt focal i massivecalcifications;
may containlarge numbersof asbestos
fibers (almostexclusivelychrysotile)
Location: bilateral+ multifocal;posterolateral
midportionof chestwall between7-1Oth
rib;aponeurotic portionof diaphragm;
mediastinum; followingrib contours;
apices+ costophrenicanglestypically
spared
Site: parietalpleura(visceralpleuratypically
sPared)
. asymptomatic,no functionalimpairment
{ usuallyfocalarea of pleuralthickening(<1 cm
thick)with edgesthickerthan centralportionsof
plaque i;n 4 8 h o n l yfi n d i n gi;n 4 1 % w i th
parenchymalchanges;stableover time
r/ no hilaradenopathy
{ usuallynot calcified
DDx: chestwall fat, rib fractures,rib companion
shadows
3. PleuralCalcification(21-25-60%)
0 HALLMARKof asbestosexPosure!
detected by radiographyin 25"/",by CT in 60%
Overall incidence: 20%
Latentperiod: >20 years to becomevisible;in
4c^hafter40 years
Histo: calcificationstartsin parietalpleura;calcium
depositsmay form withincenterof plaques
^/ denselinesparalleling the chestwall,
mediastinum, pericardium, diaphragm(bilateral
diaphragmatic calcificationswith clear
costophrenicanglesare (PATHOGNOMON lC)
r/ advancedcalcificationsare leaflikewith thick-
rollededges
DDx: talc exposure,hemothorax,empyema,
therapeuticpneumothoraxfor TB (often
extensivesheetlike,on visceral
unilateral,
pleura)
4. DiffusePleuralThickening(17%\
= sffioothuninterrupteddiffusethickeningof parietal
pleuraextendingover at least 114of chestwall
(visceralpleurainvolvedin 907o,but difficultto
demonstrate)
. may cause restrictionof pulmonaryfunction
May be associatedwith: rounded atelectasis
^/ bilateralprocesswith "shaggyheart"appearance
(20%)
r/ smooth;difficultto assesswhen vieweden face
r/ thickeningof interlobarfissures
{ focallythickeneddiaPhragm
{ obliteratedcostophrenicangles(minorityof cases)
layerof DDx: pleuralthickeningfrom parapneumonic
effusion,hemothorax, connectivetissue
disease
PulmonaryAsbestosis
= (termasbestosisreservedfor) chronicprogressive
diffuseinterstitialfibrosis
lncidence: in 49-52/" of industrialasbestosexposure
Latencyperiod: 40-45 years; dose-effectrelationship
Histo: interstitialfibrosisbeginsaroundrespiratory
bronchioles,then progressesto involveadjacent
alveoli
Diagnostic criteria:
1. ReliablehistorYof exPosure
2. Appropriatetime intervalbetweenexposure
+ detection
3. CXR evidence
4. Restrictivepatternof lung impairment
5. AbnormaldiffusingcaPacitY
6. Bilateralcracklesat posteriorlung bases,not
clearedby cough
. dyspnea
. restrictivepulmonaryfunctiontests: progressive
reductionof vital capacity+ diffusingcapacity
. asbestosbodiesin bronchoalveolar lavagefluid
Location: most severein lowerposteriorsubpleural
zones (concentration of asbestosfibers
beneathvisceralPleura)
{ smallirregularlinearopacities(NOT roundedas in
coal lsilica)progressfrom fine to coarsereticulation
{ confinedto lung bases,progressingsuperiorly
 ChestDisorders 457

rl septallines (= fibrousthickeningaroundsecondary r/ partialinterpositionof lung betweenpleura+ mass

lobules) r/ volume loss of affectedlobe t hyperlucencyof
r/ "shaggy"heartborder= obscurationsecondaryto adjacentlung
parenchymal+ pleuralchanges
r/ ill-defined
outlineof diaphragm Lung Gancerin Asbestos-related
Disease
r/ honeycombing (uncommon) lncidence: 20-25'/. of workers heavilyexposedto
^/ rarelymassivefibrosis,predominantlyat lung bases asbestos
withoutmigrationtowardhilum(DDxfrom silicosisi Occurrencerelatedto:
cwP) (a) cumulateddose of asbestosfibers
r/ ruOhilar adenopathy (b) smoking(synergisticcarcinogeniceffect)
i/ Ga-OZuptakegives a quantitativeindexof 0 Increasedrisk by factorof up to 100 in smokers
inflammatoryactivity versusa factorof 5 in nonsmokersl
HRCT: (c) preexistinginterstitialdisease
0 Obtainscan in prone positionto differentiatefrom (d) occupationalexposureto knowncarcinogen
gravity-related physiologicphenomena Latencyperiod: 25-35 years
r/ thickenedintralobularlinesas initialfinding(due to Associatedwith: increasedincidenceof gastric
centrilobular peribronchiolarfibrosis)
: carcrnoma
{ multiplesubpleuralcurvilinear branchinglines Histo: bronchioloalveolar cell carcinoma(most
("subpleuralpulmonaryarcades")= dotlike common); bronchogeniccarcinoma
reticulonodularitiesconnectedto the most (adenocarcinoma + squamouscell)
peripheralbranchof pulmonaryarlery Location: at lung base / in any locationif associated
Site: most prominentposteriorlyparallelto and with smoking
within1 cm of pleura
./ thickenedinterlobularseptallines (= interlobular MalignantMesothelioma
fibrotic/ edematousthickening): Risk: 10% over lifetimeof an asbestosworker;
r/ reticulation= networkof lineardensities,usually householdmembersof asbestosworker;
posteriorlyat lung bases residentsnear asbestosminesand plants
r/ parenchymalband = linear<5 cm long + several Latency period: 2O4O years
mm wide opacity,often extendingto pleura,which
may be thickened+ retractedat site of contact ASPERGILLOSIS
r/ patchyareas of ground-glassattenuation
Organism:
(= alveolarwall thickeningdue to fibrosis/ edema)
Aspergillusfumigatus= intenselyantigenicubiquitous
r/ honeycombing= multiplecysticspaces<1 cm in
soilfungusexistingas
diameterwith thickenedwalls (a) conidiophores= r€productiveform releasing
DDx: idiopathicpulmonaryfibrosis
thousandsof spores
(b) hyphae(= maturedspores)characterizedby45,
dichotomousbranchingpattern
AtelectaticAsbestosPseudotumor Occurrence:
= ROUNDED ATELECTASIS = "FOLDED LUNG" commonlyin sputumof normalpersons,abilityto invade
= infoldingof redundantpleuraaccompaniedby
arteries+ veins facilitatinghematogenousdissemination
segmental/ subsegmentalatelectasis
M : F= 3 : 1
0 The mostcommonof benignmassescausedby
Predisposed:
asbestosexposure! (a) preexistinglungdisease(tuberculosis,
Location: posteromedial/ posterolateralbasal region
bronchiectasis)
of lower lobes (mostcommon);frequently (b) impairmentof immunesystem (alcoholism,
bilateral
advancedage, malnutrition, concurrentmalignancy,
^/ Z.S-e cm focal subpleuralmass abuttinga regionof
poorlycontrolleddiabetes,cirrhosis,sepsis)
, thickenedpleura . positiveprecipitintest to Aspergillusantigen
! size + shape show littleprogression,occasionally . elevatedAspergillus-specific lgE, lgG-ELlSA,
decreasein size polymerasechain reactionidentification
CT:
r/ roundedI lentiform/ wedge-shapedperipheralmass _________>
+- Immune Status
r/ pleuralthickening+ calcificationalwayspresentand
frequentlygreatestnear mass Mild Severe
{ "crow'sfeet" = linearbands radiatingfrom mass into Hypersensitivity Normal immunosuppression immunosuppression
lung parenchyma(54%)
{ "vacuumcleaner"/ "comettail"sign lr tt
tt
l
l
= bronchovascular markingsemanatingfrom nodular v
Allergic
v
Saprophytic
v v
subpleuralmass+ coursingtowardipsilateral hilum Chronic Invasive
r/ "Swisscheese"air bronchogram Aspergillosis Aspergillosis Necrotizing Pulmonary
(18%) Aspergillosis Aspergillosis
458 Radiology Review Manual

Cx: disseminationto heart,brain,kidney,Gl tract, liver, (a) earlysigns

thyroid,spleen r/ single/ multipleill-definedperipheralopacities
'V Cf halosign = single/ multiple1-3-cmperipheral
0 Sputumculturesare diagnostically unreliablebecause
of normal(saprophytic)colonizationof upperairways! nodules(= necroticlung)with haloof ground-glass
attenuation(= hemorrhagic edema)
Aspergillosis
Noninvasive ri patchylocalizedbronchopneumonia
= SAPROPHYTIC ASPERGILLOSIS (b) signsof progression
= noninvasivecolonizationof preexistingcavity/ cyst in d enlargementof nodulesinto diffusebilateral
immunologically normalpatientswith cavitarydisease consolidation
[tuberculosis,sarcoidosis (common),bronchiectasis, { developmentinto largewedge-shapedpleural-
pulmonaryfibrosis,abscess,lungtrauma/ surgery, based lesions
ankylosingspondylitis, neurofibromatosis type 1, ^/ air-crescentsign (in up to 50%) = cavitationof
bullousemphysema,carcinoma] existingnodule(air crescentbetweenretracting
. sputumblood-streaked / severehemoptysis(a5-95%) sequestered necrotictissueand surrounding rim of
. elevatedserumprecipitins levelfor Aspergillus(50%) hemorrhagiclung parenchyma)1-3 weeks after
! solid roundgravity-dependent mass withinpreexisting granulocyterecovery
spherical/ ovoidthin-walledcavity(= Monadsign): 0 has betterprognosisthan consolidation without
Histo: mycetoma= aspergilloma= fungus ball cavitation(featureof resolutionphase)
= mosslikecollectionof intertwinedhyphae Prognosis; mortalityrate of 30-90%
mattedtogetherwith fibrin,mucus,cellular Dx: branchinghyphaeat tissueexamination; sputum
debriscolonizinga pulmonarycavity culturepositivein only 10%
^/ air-crescentsign = creSGeflt-shaped air space Rx: amphotericinB
separatingfungusballfrom nondependent cavitywall
ri fungusball may calcifyin scattered/ rimlikefashion
{ pleuralthickeningadjacentto preexistingcyst / cavity, Aspergillosis
AllergicBronchopulmonary
commonlyfirstsign beforevisualizingmycetoma = hypersensitivity towardaspergilliin patientswith long-
Dx: transthoracic needlebiopsy/ bronchialwashings standingasthma
DDx of other arganisms causing fungus ball: lncidence; in 1-2/" of patientswith asthma,in 10/" ot
Candidaalbicans,Pseudallescheria boydii, patientswith cysticfibrosis;mostcommon
Coccioidesimmitis,Nocardia,Actinomyces + clinicallyimportantform
Age: mostlyyoungpatients(beginsin childhood);
SemiinvasiveAspergillosis may be undiagnosed for 10-20 Years
= CHRONIC NECROTIZING ASPERGILLOSIS A. ACUTEALLERGICBRONCHOPULMONARY
= chroniccavitaryslowlyprogressivediseasein patients A S P E R GILLOS IS
with preexistinglung injury(COPD,radiationtherapy), Type I reaction= immediatehypersensitivity (lgE-
mild immunesuppression, (alcohol,
or debilitation mediated)
diabetes) Histo: alveolifilledwith eosinophils
. symptomsmimickingpulmonarytuberculosis B . C H R ON ICA LLE R GICB R ON C H OP U L M O NARY
./ progressiveconsolidation(usuallyupper lobe) A S P E R GILLOS IS
d developmentof aircrescentand fungusball Type lll reaction= delayedimmunecomplex
Dx: pathologicexamination demonstrating local response= Arthusreaction(lgG-mediated)
tissueinvasion Histo: bronchialdamagesecondaryto
Aspergillusantigenreactingwith lgG
InvasivePulmonaryAspergillosis antibodies,immunecomplexesactivate
= often fatal form in severelyimmunocompromised complementleadingto tissueinjury
patientswith absoluteneutrophilcountof <500 Pathophysiology:
Predisposed;most commonlyin lymphoma/ leukemia inhaledsporesare trappedin segmentalbronchiof
patientswith prolongedgranulocytopenia, individuals with asthma,germinate,and form hyphae;
after organtransplantation immunologic responsecoupledwith proteolytic
Path: endobronchialfungalproliferationfollowedby enzymescausespulmonaryinfiltrates + tissue
transbronchial vascularinvasioneventually damage+ centralbronchiectasis
causeswidespreadhemorrhage+ thrombosisof Criteria:
pulmonaryarterioles+ ischemictissuenecrosis (a) Primarydiagnosticcriteria:
+ systemicdissemination; fungusball = de- acronym: ARTEPICS
vitalizedsequestrumof lung infiltratedby fungi Asthma (84-96%)
. Hx of seriesof bacterialinfections+ unremitting fever Roentgenographic transientor fixedpulmonary
. pleuriticchestpain (mimickingemboli) infiltrates
. dyspnea,nonproductive cough Test for A. fumigatuspositive:immediateskin
. progressionof pulmonaryinfiltratesnot respondingto reaction
broad-spectru m antibiotics Eosinophiliain blood between8h and 40/"

Precipitatingantibodiesto A. fumigatus(70%)
lgE in serum elevated
Centralbronchiectasis(late manifestationthat
provesdiagnosis)
Serum-specific lgE and lgG A. fumigatuslevels
elevated
(b) Secondarydiagnosticcriteria(lesscommon):
1. Aspergillus fumigatusmyceliain sputum
2. Expectoration of brownsputumplugs (54%)
3. Arthus reaction(= late skin reactivitywith
erythema+ induration) to Aspergillusantigen
Staging:
I acute phasewith all primarydiagnosticcriteria
ll clearingof pulmonaryinfiltrateswith declininglgE
levels
lll all criteriaof stage I reappearafter emission
lV corticosteroiddependency
V irreversiblelung fibrosis
. flulikesymptoms:fever,headache,malaise,weight
loss,fleetingchestpain
r/ migratorypneumonitis= transientrecurrent"fleeting"
alveolarpatchysubsegmental/lobarinfiltrates in
upper lobes (50%),lower lobes(20/"), middlelobe
(7"/"),both lungs(65%);may persistfor >6 months
r/ centralvaricosei cysticbronchiectasis:
r/ "tramlike"bronchialwalls (edema)
r/ t-Z-cm ring shadows(= bronchuson end) around
hilum+ upperlobes(HALLMARK)
r/ "finger-in-glove/ toothpasteshadow"= V- or y-
shapedcentralmucusplugsin 2nd orderbronchiof
2.5-6 cm in lengthremainingfor months+ growing
in size
r/ lobarconsolidation(in gZ%)
r/ atelectasis(in 14%)with collateralair drift
r/ cavitation(in 14%)secondaryto postobstructive
abscess
./ hyperinflation(dueto bronchospasm)
! pulmonaryfibrosis+ retraction
r/ hilarelevationdue to lobarshrinkage
r/ emphysema
^/ ruOnUnL peripheralbronchi
{ UNUSUALare aspergilloma in cavity(T%),empyema,
pneumothorax
DDx: hypersensitivity pneumonitisor allergicasthma
(no hyphaein sputum,normallevelsof lgE + lgG
to A. fumigatus),tuberculosis,lipoidpneumonia,
Lofflersyndrome,bronchogeniccarcinoma
PleuralAspergillosis
= Aspergillus
empyema inpatients
withpulmonary
tuberculosis,bacterialempyema,bronchopleural
fistula
! pleuralthickening
ASPIRATION
OF SOLIDFOREIGN
BODY
@ Childhood
Age: in 50% <3 years
Delay of diagnosis: within 2-3 days (usual);weeks
to months(rare)
ChestDisorders 459
Source: in 85% vegetableorigin(lentil,beans,peas,
peanut,barleygrass),brokenfragmentsof
teeth
Location:almostexclusivelyin lowerlobes;R:L = 2:1
. varyingdegreesof cough
! obstructiveoverinflation(68%) + reflex
vasoconstriction
r/ atelectasis(1 4-53%)
r/ infiltrate(11%)
r/ radiopaqueforeignbody (9%)
r/ air trapping(expiratory/ lateraldecubitusfilm):
^/ lobar/ segmentaloverinflation
CT:
r/ low-attenuationintrabronchial material
(SUGGESTTVE)
NUC:
{ ventilationdefect(initialbreath)+ retention
(washout)
Cx: bronchiectasis(fromlong retention)
DDx: impactedesophagealforeignbody
@ Adulthood(unusual)
. oftenclinicallysilent
. massivelife-threatening hemoptysis
{ chronicvolume loss of affectedlobe
r/ recurrentpneumonia
{ bronchiectasis
r/ intrabronchial massformation(= chronic
inflammatoryreactionaroundinhaledmaterial)
r/ centrallylocatedmass + lobar/ segmentalcollapse
AspirationBronchiolitis
= chronicinflammatoryreactionto repeatedlyaspirated
foreignparticlesin the bronchioles
Predisposed;achalasia,Zenkerdiverticulum,
esophagealcarcinoma
Histo: resemblingdiffusepanbronchiolitis
. dysphagia,regurgitation, aspiration
r/ moderate/ markeddilatationof esophagus
r/ lobar/ segmental/ disseminated smallnodules
CT:
{ uni- / bilateralfoci of branchingareasof increased
attenuation:
r/ tree-in-budappearance
r/ mottledpoorlydefinedopacifiedacinarareas
ASPIRATION PNEUMONIA
Predisposing conditions:
(1) CNS disorders/ intoxication:alcoholism,mental
retardation,seizuredisorders,recentanesthesia
(2) Swallowingdisorders:esophagealmotility
disturbances,head + neck surgery
. low-gradefever
. productivecough
. chokingon swallowing
Location: gravity-dependent portionsof lung,posterior
segmentsof upper lobes + lower lobesin
bedriddenpatients,frequentlybilateral,right
middle+ lowerlobewith sparingof left lungis
common
460 Radiology Heview Manual

A. ACUTEASPIRATIONPNEUMONIA B. EXTRINSICASTHMA= ATOPICASTHMA

Cause: gastricacid,food particles,anaerobic Pathogenesis:
bacteriafrom Gl tract provokeedema, secondaryto antigensproducingan immediate
hemorrhage, inflammatory cellular hypersensitivity response(typel); reaginsensitizes
response,foreign-bodyreaction mast cellsto releasehistaminefollowedby
Gram-negativebacteria;Pseudomonas increasedvascularpermeability, edema,small
aeruginosa, Actinomycesisraelii musclecontraction; effectsprimarilybronchicausing
{ patchybronchopneumonic pattern airwayobstruction
r/ lobar/ segmentalconsolidation in dependentportion Nonoccupational alIergens:
r1 necrotizingpneumonia pollens,dog + cat fur, tamarindseed powder,castor
^/ abscessformation bean,fungalspores,grainweevil
B. CHRO NI CA S PIR AT ION P N E U MON IA Occupational allergens:
Cause: repeatedaspirationof foreignmaterial (a) naturalsubstances:wood dust,flour,grain,
from Gl tract over long time / mineraloil beans
(eg, in laxatives) (b) pharmaceuticals: ASA
antibiotics,
Associatedwith: Zenkerdiverticulum,esophageal (c) inorganicchemicals:nickel,platinum
stenosis,achalasia,TE fistula, Path: bronchialpluggingwith largeamountsof viscid
neuromuscular disturbances in tenaciousmucus(eosinophils, Charcot-Leyden
swallowing crystals),edematousbronchialwalls, hypertrophy
./ recurringsegmentalconsolidation of mucousglands+ smoothmuscle
./ progressionto interstitialscarring(= localized ACUTESIGNS:
honeycombappearance) . duringasthmaticattacklow valuesfor FEV + MMFR
r/ bronchopneumonic infiltratesof variablelocation and abnormalVIQ ratios
over months/ years . increasedresistanceto airflowdue to
! residualperibronchial scarring (a) smoothmusclecontractionin airwaywalls
UpperG l: (b) edema of ainrvaywall causedby inflammation
r/ abnormalswallowing/ aspiration (c) mucushypersecretion with airwayplugging
. normaldiffusingcapacity
./ hyperexpansion of lungs = seV€r€overinflation+ air
MendelsonSyndrome trapping:
= ospirationof gastricacid with a pH <2.5 r/ flatteneddiaphragmaticdome
Associatedwith: vomiting,gastroesophageal reflux, { deepenedretrosternalair space
achalasia,hiatalhernia { peribronchial cuffing(inflammation of airwaywall)
Pathophysiology: ri bronchialdilatation
acid rapidlydisseminatesthroughoutbronchialtree r/ localizedareas of hypoattenuation
+ lungparenchyma,incitesa chemicalpneumonitis C H R ON ICC H A N GE S :
within minutes;extentof injuryfrom mild bronchiolitis Normalchest x-ray in73o/",findingsof abnormalities
to hemorrhagic pulmonaryedemadependson pH dependon
+ aspiratedvolume (a) age of onset(<15yearsof age in 31%; >30 years
Location: with patientin recumbentposition: of age in none)
posteriorsegmentsof upper lobes (b) severityof asthma
+ superiorsegmentsof lower lobes r/ centralring shadows= bronchiectasis
ri bilateralperihilarill-defined
alveolarconsolidations ri scars (fromrecurrentinfections)
{ multifocalpatchyinfiltrates Cx:
./ segmental/ lobarconsolidationlocalizedto one / both (1) Pneumonia(2 x as frequentas in nonasthmatics)
lung bases r/ peripheralpneumonicinfiltrates (secondaryto
prosnosis' blockedairways)
3B Jtfr.i'fl,l],:i'i#5:,'il? l::XlllIo"o (2) Atelectasis(5-15%) from mucoidimpaction
(3) Pneumomediastinum (5%),pneumothorax,
p n e u m o n i ao, r AR D S
subcutaneousemphysema;predominantlyin children
(a) Emphysema
(5) Allergicbronchopulmonary aspergillosiswith central
ASTHMA
= episodicreversiblebronchoconstriction secondaryto bronchiectasis
to a varietyof stimuli
hypersensitivity
A. INTRINSICASTHMA ATYPICALMEASLESPNEUMONIA
Age: middleage = clinicalsyndromein patientswho have been previously
Pathogenesis: inadequatelyimmunizedwith killedrubeolavaccineand
probablyautoimmunephenomenoncausedby viral are subsequentlyexposedto the measlesvirus (= typs
respiratoryinfectionand often provokedby infection, notedin children
lll immunecomplexhypersensitivity);
exercise,pharmaceuticals; antigen
no environmental who have receivedlive vaccinebefore13 monthsof age
 ChestDisorders 461

. 2- lo 3-day prodromeof headache,fever,cough,malaise

A. ACUTEBERYLLTOSTS (25%)
. maculopapular rashbeginningon wrists+ ankles ./ pulmonaryedema followingan ovenryhelming
(sometimesabsent) exposure
. postinfectiousmigratoryarthralgias
B . C H R ON ICB E R Y LLIOS IS
historyof exposureto measles widespreadsystemicdiseaseof liver,spleen,lymph
^/ extensivenonsegmental consolidation,usuallybilateral nodes,kidney,myocardium, skin,skeletalmuscle;
{ hilaradenopathy(100%) removedfrom lungs + excretedvia kidneys
d pleuraleffusion(0-70%) Latentperiod: 5-15 years
nodulardensitiesof 0.5-10 cm in diameterin peripheral r/ tine nodularity(granulomas similarto sarcoidosis)
location,may calcifyand persistup to 30 months r/ irregularopacities,particularlysparingapices
+ bases
BARITOSIS ^/ hitar+ mediastinaladenopathy(may calcify)
= inhalationof nonfibrogenic bariumsulfate d emphysemain upperlobes+ intersiitial fibrosis
. asymptomatic ri pneumothorax in 10%
. normalpulmonaryfunction(benigncourse) H R C T:
r/ bilateralnodulari patchyopacities,denserthanbone r/ diffusesmallparenchymalnodules(57%)
(highatomicnumber) r/ septattines(50%)
r/ similarto calcifiednodules r/ patchesof ground-glassattenuatio n (gZ%)
1 tttOcor pulmonale,NO hilaradenopathy ^/ hitaradenopathy12|-SS|%), only in the presence
^/ regressionif patientremovedfrom exposure of parenchymal abnormalities
r/ bronchialwall thickening(46%)
{ pleuralirregularities(25%)
BEHQETSYNDROME DDx: (1) Nodularpulmonarysarcoidosis
= fore multisystemdiseaseof unknownorigin (indistinguishable)
characterizedby (2) Asbestosiswithouthilar adenopathy
(1) aphthousstomatitis
(2) genitalulceration
(3) iritis
. positivepathergytest = unusualhypersensitivity to BLASTOMYCOSIS
prickingwith formationof pustulesat site of needleprick = NORTH AMERICAN = GILCHRIST
BLASTOMYCOSIS
within 24-48 hours DISEASE = CHICAGO DISEASE
. skin changes:erythemanodosum,folliculitis, = ta.tesystemicmixed pyogenic+ granulomatousfungal
papulopustuf ar lesions infection
. arthritis,encephalitis
Organism; soil-bornsaprophyticdimorphicfungus
. epididymitis
Blastomyces dermatitidis, mycelialphasein
@ Chest (5%) soil + roundthick-walledyeastform with
r/ multipleperipheralsubpleuralopacities(dueto broad-based buddingin mammals
hemorrhage,necroticpufmonaryinfarctions) Geographic di stribution:
r/ increasedradiopacitynear hila (pulmonaryartery worldwide;endemicin central+ southeastern United
aneurysm) States(Ohio+ Mississippirivervalleys,vicinityof Great
@ Veins (25%) Lakes),Africa,Canada(northernOntario),Central
r/ largevein occlusion;may causeSVC syndrome + SouthAmerica(acquiredthroughactivitiesin woods)
r/ subcutaneousthrombopirlebitis Age: severalmonthsof age to 80 years (peakbetween
@ Arteries 25 and 50 years of age)
r/ arterialocclusion/ pulselessdisease Mode of infection:
r/ aneurysmof largearteries(in z%) inhalationof fungalconidia(primaryportalof entry);
spreadto extrapulmonary sites,eg, skin,bone (often
BERYLLIOSIS directextensionfrom skin lesionresembling
= chronicgranulomatous disorderas a resultof beryllium- actinomycosis), joints
specificcell-mediated immuneresponse(= delayed Predisposed: elderly,immunocompromised
hypersensitivity reactionafter exposureto acid salts Histo:
from extractionof berylliumoxide) (a) exudativephase: accumulation of numerous
Substance.' one of the lightestmetals(atomicweightg), neutrophilswith infectingorganism
markedheat resistance,great hardness, (b) proliferativephase: proliferationof epithelioid
fatigueresistance,no corrosion granulomas+ giantcellswith central
occupational exposure: fluorescentlight bulb factories microabscesses containingneutrophils and yeast
Histo: noncaseatinggranulomaswithin interstitium forms
+ along vessels+ in bronchialsubmucosa . mouth ulcers
' positiveberylliumlymphocytetransformation test (blood . fever,cough,weightloss,chestpain (majority)
test of T-lymphocyteresponseto beryllium) . crustedverrucouslesionson exposedbody areas
462 Radiology Review Manual

@ Lung BONE MARROW TRANSPLANTATION

. Clinicalpatternsfollowingpulmonaryinfection: = intravenous progenitorcells
infusionof hematopoietic
(a) severepulmonarYsYmPtoms from patient'sown marrow(autologoustransplant)/
(b) asymptomaticpulmonaryinfectionwith HLA-matcheddonor (allogenictransplant)to reestablish
spontaneousresolution marrowfunctionafter high-dosechemotherapyand total
(c) disseminateddiseaseto single/ multipleorgans body irradiationfor lymphoma,leukemia,anemia,
indolentfor severalYears multiplemyeloma,congenitalimmunologic defects,solid
(d) extrapulmonarymanifestationinvolvingmale tumors
GU system,skeleton,skin Cx: pulmonarycomplicationsin 40-60%
{ segmental/ lobarairspacediseasein lower lobes in
acute illness(26-61%) NeutropenicPhasePulmonaryComplications
r/ solitary/ multipleirregularnodularmasses/ satellite Time: 2-3 weeksaftertransplantation
lesionsin paramediastinal location 1. AngioinvasiveasPergillosis
r/ air bronchogramin area of consolidation / mass (87%) r/ nodulesurroundedby halo of ground-glass
r/ interstitialdisease attenuation(= fungal infectionspreadinginto lung
r/ cavitationif communicatingwith airway(13%) parenchymaand surrounding area of hemorrhagic
^/ nilar/ mediastinallymphnodeenlargement(<25%) infarction)
@ Bone r/ segmental/ subsegmentalconsolidation
r/ markeddestruction* surroundingsclerosis (= pulmonaryinfarction)
r/ periostealreactionin long bones,but not in short ! cavitationof nodulewith air-crescentsign (during
bones recoveryphasewith resolvingneutropenia)
r/ multipleosseouslesionsare frequent r/ .s-mm centrilobular nodulesto 5-cm peribronchial
r/ vertebralbodies + intervertebraldisks are destroyed consolidation(= airwayinvasionwith surrounding
(similarto tuberculosis) zone of hemorrhag e I organizingpneumonia)
^/ psoas abscess 2. Diffusealveolarhemorrhage(20%)
./ tyticskull lesions+ soft-tissueabscess . hemosiderin-laden macrophages on lavage
{ usuallymonarticular arthritis:knee > ankle> elbow r/ bilateralareasof ground-glassattenuationi
> wrist > hand consolidation
@ GU tract (20%): prostate,epididymis 3. Pulmonaryedema
Dx: (1) Cultureof organism Cause: infusionof largevolumesof fluid combined
(2) SiMerstain microscopyof tissues with cardiac+ renaldYsfunction
Prognosis; spontaneousresolutionof acute diseasein ^/ prominentpulmonaryvessels,interlobarseptal
up to 4 weeks;diseasemay reactivatefor up thickening,ground-glass attenuation,pleural
to 3 years effusions
Rx: (1) AmphotericinB lV: 8-10 weeks for noncavitary 4. Drug toxicitY
+ 10-12 weeksfor cavitarYlesions Cause: bleomycin,busulfan,bischloronitrosurea
(2) Ketoconazole (carmustine),methotrexate
DDx: otherpneumonias(ie, bacterial,tuberculous, ^/ bilateralareas of ground-glassattenuation/
fungal),pseudolymphoma, malignantneoplasm(ie, consolidation/ reticularattenuation(= fibrosis)
alveolarcell carcinoma,lymphoma,Kaposisarcoma)
EarlyPhasePulmonaryComplications
Time: upto 100daysaftertransplantation
1. C MV pneumoni a (23% )
BLUNTCHESTTRAUMA r/ multiplesmall nodules+ associatedareas of
lncidence: 100,000hospitaladmissions/year (in USA) consolidation+ ground-glassattenuation
Cause: high-speedmotorvehicleaccidents(70%) (= hemorrhagic nodules)
Type of injury: 2. Pneumocystis cariniipneumonia
1 . P n e u m o t h o r a. .x. . . . . . . . . . . . . . . . 6 9 % r/ diffuse/ predominantlyperihilar/ mosaicpatternof
2. Lungcontusion .67"/" ground-glass attenuationwith sparingof some
3 . R i bf r a c t u r e. . . . . . . . . . . . . . . . . . . . . . . 6 6 % secondarY PulmonarY lobules
4. Hem ot h o ra...........
x ........... 2 8 " /" pneumonia(12%)
3. ldiopathicinterstitial
5. F lailc he s t ..........1 4 " /" ^l nonspecific findings(diagnosisof exclusion)
6. T hor ac i cs p i n efra c tu re.....1 3 %
7. Clav ic l efra c tu re................ 13% LatePhasePulmonaryComplications
8 . S c a p u l af r a c t u r e. . . . . . . . . . . . .8. % ... Time: after100daysposttransplantation
9. Sternalfracture ...5"/" obliterans(in up to 1O%)
1. Bronchiolitis
10. Diaphra g m a tii nc j u ry...........5 % 2. BOOP
11. T r ac he o b ro n c h te i a la r.........2 " /" 3. Chronicgraft-versus-host disease
12. Vasculariniury ....2o/o infections,chronicaspiration,bronchiolitisobliterans,
13. E s opha g e aru l p tu re............ 1 o /o lymphoidinterstitial
Pneumonia
 ChestDisorders 463

BRONCHIAL
ADENOMA Age peak; Sth decade(rangeof 2nd-gth decade);
= misnomersecondaryto locallyinvasivefeatures, 4o/"occurin children+ adolescents;
tendencyfor recurrence,and occasionalmetastasisto M : F= 2 : 1 ; v e r yu n c o m m o inn B l a c k s
extrathoracic sites(10%)= low-grademalignancy Path:
Path: arisesfrom duct epitheliumof bronchialmucous originatesfrom neurosecretory cellsof bronchial
glands(predominant distribution of Kulchitsky cells mucosa(= Kulchitsky cells= erg€fltaffine
cells)just as
at bifurcationsof lobarbronchi) smallcell cancer;partof APUD (amineprecursor
lncidence; 6-1 0/" of all primarylung tumors uptakeand decarboxylation) system= chromaffin
Age: mean age of 95-45 years (range12-60 years); paraganglioma, whichproducesserotonin,ACTH,
90% occur<50 yearsof age; mostcommon norepinephrine, bombesin,calcitonin, ADH, bradykinin
pr im ar ylu n gtu m o ru n d e ra g e 1 6 ; M:F = 1:1; Pathologic cl assification :

Types:
Whites:Blacks

mnemonic.' "CAMP"
= 25:1
Kcc'
(KCC = Kulchitsky celt carcinoma)
")'
Carcinoid
Adenoidcysticcarcinoma= Cylindroma 6%
g0o/o =3i?]*i1.ffi'(;:':x'f:o
growth;usually<2.5cm in size + well-
Mucoepidermoid carcinoma go/" defi ned;youngerpati ent;M:F = 1: 10;
Pleomorphiccarcinoma 1% lymph node metastasesin 3%
Location: most commonlynear/ at bifurcationof lobarI KCC ll = atypical carcinoid (25%of carcinoid
segmentalbronchi;central:peripheral = 4:1 tumors);mass usually>2.5cm with well-
- 48% on right : RLL (20/"), RML (jO%),RUL (7"/"),
definedmargins;olderpatient;M:F = 3:1;
main rightbronchus(B%), lymph node metastasesin 40-50%;
intermediatebronchus(3%) metastasesto brain,liver,bone (in 30%)
-32% on left : LLL (19%),LUL (12/o),mainleft KCC lll = small cell carcinoma (mostaggressive);
b ro n c h u s(6 /" ),l i n g u l abr ro nchus rnediastinal lymphadenopathy; ill-defined
(1o/") tumormargi ns
. hemoptysis(40-50%) 0 Rarelycausefor carcinoidsyndromeor Cushing
. atypicalasthma syndrome!
. persistentcough . recurrentunifocalpneumonitis, hemoptysis
. recurrentobstructive pneumonia . wheezing,persistentcough,dyspnea,chestpain
. asymptomatic(10%) . carcinoidsyndrome(rare)
r/ completeobstruction/ air trappingin partialobstruction . endobronchial exophyticmass at endoscopy
, (rare)/ nonobstructive (10-15%) Location: 58-90% centralin lobar/ segmentalbronchi,
r/ obstructiveemphysema 10-42% peripheral;locatedin submucosa;
1 recurrentpostobstructive infection:pneumonitis, endobronchial / alongbronchialwall /
bronchiectasis, abscess exobronchial
r/ atelectasis/ consolidationof a lung/ lobe/ segment r/ potypoidtumor with averagesize of 2.2 cm
(78%) r/ mostextendthroughbronihialwall thus involving
r/ collateralair drift may preventatelectasis bronchiallumen+ parenchyma(= collarbuttonlesion)
r/ solitaryround loval slightlylobulatedpulmonarynodule r/ calcification/ ossificatio
n (26-33%): centralcarcinoid
( 1 9 % )o f 1 - 1 0 c m i n s i z e (43 ), peripheralcarcinoid(10%)
{ hilarenlargement / mediastinal widening r/ vasculartumorsuppliedby bronchialcirculation
= cefitralendo-/ exobronchialmass { cavitation(rare)
CT: r/ segmental/ lobaratelectasis
r/ well-marginated sharplydefinedmass r/ obstructivepneumonitis
r/ in close proximityto an adjacentbifurcationwith r/ bronchiectasis+ pulmonaryabscess
, splayingof bronchus Malignantpotential:low
r/ coarseperipheralcalcifications in 1/3 (cartilaginous / Metastases:
, bony transformation) (a) regionallymphnodesin 25/"
! may exhibitmarkedhomogeneous enhancement (b) distantlyin 5% (adrenal,liver,brain,skin,
Biopsy: riskysecondaryto highvascularityof tumor osteoblasticbone metastases)
Prognosis: 95/" S-yearsurvivalrale,TS/o1S-year Prognosis:
survivalrate after resection 95% S-yearsurvivalrate for classiccarcinoids;
57-66% S-yearsurvivalrate for atypicalcarcinoids

Carcinoid Cylindroma
= NE URO E ND OC RCIN
ARE C IN O MA = ADENOTD CYSTTC CARCTNOM
A (7%)
= slow-growing
low-grademalignanttumor Secondmostcommonprimarytumorof trachea
Incidence: 12-15% of all carcinoidtumors in the Path: mixedserous+ mucousglands;resembles
body;1-4o/oof all bronchialneoplasms salivaryglandtumor
464 Radiology Review Manual

Histo: Etiology:
Grade1: tubular+ cribriform;no solidsubtype A. Congenital
r/ entirelyintraluminal 1. Structuraldefectof bronchi:bronchialatresia,
Grade2: tubular+ cribriform; <20/o solidsubtype Williams-CamPbell sYndrome
r/ predominantlY intraluminal 2. Abnormalmucociliarytransport: Kartagener
Grade 3: solidsubtYPe>20o/o syndrome
r/ predominantly extraluminal 3. Abnormalsecretions:cysticfibrosis
Age peak; 4th-Sth decade B. Congenital/ acquiredimmunedeficiency(usually'
. typical Hx of refractory"asthma" lgG deficiency):chronicgranulomatous diseaseof
. hemoptysis, cough,stridor,wheezing childhood,alpha-1antitrypsin deficiency
. dysphagia,hoarseness C. Postinfectious childhoodpneumonias(after
r/ endotrachealmass with extratrachealextension necrotizingviralI bacterialbronchitis):measles,
Malignantpotential: whoopingcough,Swyer-James syndrome,allergic
more aggressivethan carcinoidwith propensityfor bronchopulmonary chronic
aspergillosis,
localinvasion+ distantmetastases(lung,bone' brain, granulomatous infection(TB)
liver)in 25% D. Distalto bronchialobstruction(dueto accumulation
Rx: trachealresection+ adjunctiveradiotherapy of secretions):neoplasm,inflammatory nodes,
Prognosis: 8.3 years mean survival foreignbody
E. Aspiration/ inhalation:gastriccontents/ inhaled
Carcinoma
Mucoepidermoid fumes(latecomPlication)
Path: squamouscells+ mucus-secreting columnar F. "Traction bronchiectasis" (due to increasedelastic
cells;resemblessalivaryglandtumor recoilwith bronchialdilatation+ mechanical
r/ may involvetrachea= locallyinvasivetumor distortionof bronchi):advancedpulmonaryfibrosis/
{ sessile/ polypoidendobronchial lesion radiation-induced lung injurY
G. Increasedinflationary pressure
PleomorphicAdenoma Classification:
= MIXEDTYPE(extremelyrare) 1. Gylindrical/ tubular / fusiform bronchiectasis
= mildlyand uniformlydilatedbronchi(leastsevere
tvpe)
ATRESIA
BRONCHIAL reversibleif associatedwith pulmonarycollapse
= localobliteration of proximallumenof a segmental Path: 16 subdivisions of bronchi
./ squareabruptendingwith lumenof uniform
bronchus
Praposed causesi diameterand samewidthas parentbronchus
(a) localinterruption of bronchialarterialperfusion>15 H R C T:
weeksGA (whenbronchialbranchingis complete) r/ "tram lines"of nontaperingair ways (horizontal
(b) tip of primitivebronchialbud separatesfrom bud and course)
continuesto develoP { "signet-ring sign"(verticalcourse)= cross
Path: normalbronchialtree distalto obstructionpatent sectionof dilatedbronchus+ branchof
and containingmucusplugs;alveolidistalto pulmonarYarterY
./ Y- or V-shapedareas of attenuation= mucous
obstructionair-filledthroughcollateralair drift
plugsfillingbronchiectatic segments
Associatedwith: lobaremphysema,cystic
adenomatoidmalformation 2. Varicose bronchiectasis
. minimalsymptoms,apparentlaterin childhood(mostby - moderatelydilatedand beadedbronchi(rare)
age 15) / adultlife Associatedwith: Swyer-Jamessyndrome
Location: apicoposterior segmentof LUL (>>RUL/ ML) Path: 4-8 subdivisionsof bronchi
r/ decreasedperfusion r/ beadedcontourwith normalpatterndistally
! overexpandedsegment(collateralair driftwith 3. Saccular / cystic bronchiectasis
= mark€dcysticdilatation(mostseveretype)
expiratory air-traPPing)
r/ fingerlikeopacitylateralto hilum (= rnUcusplug distalto Associatedwith: severebronchialinfection
atreticlumen)is CHARACTERISTIC Path: <5 subdivisionsof bronchi
OB-US (detected>24 weeks MA): ! progressive ballooningdilatationtowardperiphery
{ largeechogenicfetallung mass= fluid-filled lung with diameterof saccules>1 cm
r/ irregularconstrictionsmay be present
distalto obstruction
^/ dilatationof bronchion inspiration, collapseon
r/ ditatedfluid-filledbronchus
Rx: no treatmentbecausemostlyasymptomatic expiration
DDx: Congenitallobaremphysema(no mucusplug) r/ containsvariableamountsof pooledsecretions
H R C T:
r/ stringof cysts = "strirr9of pearls"(horizontal
BRONCHIECTASIS "clusterof grapes"
= localizedmostlyirreversibledilatationof bronchioften course)/ clusterof cysts =
with thickeningof the bronchialwatl { air-fluidlevel(frequent)
 ChestDisorders 465

Age: predominantfypediatricdisease (4) Connectivetissuedisorder: rheumatoidarthritis,

. chroniccough,excesssputumproduction
scleroderma, systemiclupuserythematosus
' recurrentinfectionwith expectorationof purulentsputum (5) Chronicrejection: lungtransplant,heart-lung
. shortnessof breath
transplant(30-50%)
. hemoptysis(50%) (6) Chronicgraft-versus-host disease: bone marrow
. frequentexacerbations+ resolutions(due to
transplant
superimposedinfections) (7) Cysticfibrosis(as a compficationof repeated
Associatedwith: obliterative+ inflammatorybronchiolitis episodesof pulmonaryinfection)
(in 85%) (8) ldiopathic(in immunocompetent patients)
Location: posteriorbasalsegmentsof lower lobes, Path: submucosaland peribronchiolar fibrosis
bilateral(50%),middlelobe/ lingula(10y"), = irreversiblefibrosisof small airwaywalls with
centralbronchiectasis in bronchopulmonary narrowing/ obliteration of airwaylumina(respiratory
aspergillosis bronchiole, alveolarduct,alveola)by granulation
CXR (37%sensitive): tissueof immaturefibroblastic plugs(Masson
r/ ditatedair-filledbronchi bodies)
r/ bronchialwall thickening Peakage: 40-60 years; M:F = 1:1
./ increasedbackgrounddensity . insidiousonsetof dyspneaover many months
r/ parenchymal volumeloss: ' obstructivepulmonaryfunctiontests
r/ crowdingof lung markings(if associatedwith . no responseto antibiotics
atelectasis) . persistentnonproductivecough,fever
r/ increasein size of lung markings(retained r/ normalCXR (in up to 40%)
, secretions) r/ hyperinflatedlungs= limiteddiseasewith connective
r/ lossof definitionof lung markings(peribronchial tissueplugsin airways
fibrosis) r/ bilateralscatteredheterogeneous+ homogeneous
r/ cysticspaces+ air-fluidlevels<2 cm in diameter opacities(typicallyperipheralin distribution; equally
(dilatedbronchi) distributedbetweenupper+ lower lobes)
r/ honeycombpattern(in severecases) r/ bronchiectasis
I compensatory hyperinflationof uninvolvedipsilateral r/ decreasedvascularity(reflexvasoconstriction)
lung HRCT (pairedexpiration-inspiration images:
HRCT (87-97% sensitive,93-1 00% specific): r/ "mosaicperfusion"of lobularair trafping (gS-1OO%)
r/ lack of bronchialtapering(in 80% = rT.lostsensitive = patchyareas of decreasedlung attenuation
f inding) alternating with areasof normalattenuation:
{ bronchialwall thickening ^i areasof decreasedattenuationcontainingvessels
r/ signetringsign = internildiameterof bronchuslarger of decreasedcaliber(due to alveolar
, than adjacentpulmonaryartery(in 60%) hypoventilation + secondaryvasoconstriction of
ri bronchivisiblewithin1 cm of pleuraff aS%) alveolidistalto bronchiolar obstruction)
./ mucus-filled dilatedbronchi(in 6%) r/ areasof increasedattenuationcontainingvessels
Cx: frequentrespiratoryinfections of increasedcaliber(uninvolved areaswith
DDx of CT appearance: compensatoryincreasedperfusion)
(1) Emphysematous blebs(no definablewall thickness, r/ bronchialwall thickening(87%)
subpleurallocation) r/ bronchiectasis(66-80{)
(2) "Reversiblebronchiectasis" = temporarydilatation r/ patchyair trappingon expiratoryscans (due to
duringpneumoniawith returnto normalwithin4-6 collateralairdriftinto postobstructive alveoli)= failure
months of volume/ attenuationchangebetweenexpiratory
+ inspiratoryimages
r/ poorlydefinednodularareas of consolidation
r/ "tree-in-bud"appearanceof bronchioles
BRONCHIOLITISOBLITERANS = centrilobularbranchingstructuresand nodules
= CONSTRICTIVE BRONCHIOLITIS = OBLITERATIVE causedby peribronchiolar thickening
B RO NCHI O LI T IS + bronchiolectasis with secretions(the only direct,but
= inflammationof bronchiolesleadingto (sometimes uncommonsign)
reversible)obstruction
of bronchiolar lumen r/ centrilobular ground-glass opacities
Etiology:
(1) Inhalation:1-3 weeks after exposureto toxic fumes Rx: steroids may stop progression
(isocyanates,phosgene,ammonia,sulfurdioxide, DDx: (1) Bacterial/ fungalpneumonia(responseto
chlorine) antibiotics,positivecultures)
(2) Postinfectious:Mycoplasma(chifdren),virus (older (2) Chroniceosinophilicpneumonia(youngfemafe,
individual);
see Swyer-James syndrome eosinophiliain213)
(3) Drugs: bleomycin,gold salts,cyclophosphamide, (3) Usualinterstitial
pneumonia(irregularopacities,
methotrexate,penicillamine decreasedlung volume)
466 Radiology Review Manual

OBLITERANSWITH ORGANIZING
BRONCHIOLITIS CARCINOMA
BRONCHIOLOALVEOLAR
= ALVEOLAR CELLCARCINOMA = BRONCHIOLAR
PNEUMONIA(BOOP)
= PROLIFERATIVE BRONCHIOLITIS = CRYPTOGENIC CARCINOMA
oRG A NI Z I NG PN EU M O N IT(C ISO P ) lncidence: 1.5-6% of all primarylung cancers
Prevalence: 20-301" of all chronicinfiltrativelung disease (increasingincidencelo ? 2O-25"/")
Cause: postobstructive pneumonia,organizingadult Etiotogy: developmentfrom type ll alveolarepithelialcells
respiratory distresssyndrome,lungcancer, Age: 40-70 years; M:F = 1:1 (strikingly high in women)
extrinsicallergicalveolitis, pulmonary Path: peripheralneoplasmarisingbeyonda recognizable
manifestation of collagenvasculardisease, bronchuswith tendencyto spreadlocallyusinglung
pulmonarydrugtoxicity,silo fillerdisease, structureas a stroma(= lepidic growth)
idiopathic(50%) Histo: subtypeofwell-differentiatedadenocarcinoma;
Path: granulation tissuepolypsfillingthe luminaof cuboidal/ columnarcellsgrow alongalveolar
alveolarductsand respiratory bronchioles walls+ septawithoutdisruptingthe lung
architecture or pulmonaryinterstitium (serving
(bronchiolitis obliterans) + variabledegreeof
infiltrationof interstitium and alveoliwith as "scaffolding" for tumorgrowth)
macrophages(organizingpneumonia) Subtypes:
obliteranscomponentnot present (a) mucinous(80%):mucin-secreting tall
0 Bronchiolitis
in up t o 1 /3 ! columnarpeglikebronchiolar cells;more
Histo: plugs of immaturefibroblasts(Massonbodies) tikely multicentric',26o/"S-yearsurvival rate
whichmay (b) nonmucinous (20%):cuboidaltype ll alveolar
coveredwith low cuboidalepithelium,
spreadthroughcollateralair drift pathways pneumocyteswith productionof surfactant/
Age: 40-70 years; M:F = 1:1 nonciliatedbronchiolar(Clara)cells;more
. clinical+ functional+ radiographic of
manifestation localized+ solitary;72% S-yearsurvivalrate
organizing pneumonia Riskfactors: localizedpulmonaryfibrosis(tuberculous
. nonproductive cough,dyspnea(1-4-monthhistory), scarring,pulmonaryinfarct)in 27"/",diffuse
fibroticdisease(scleroderma), previous
precededby a briefflulikeillnesswith sore throat (40"/"),
low-gradefever,malaise(in 33%) exogenousl i pi dpneumoni a
. late respiratorycrackles . historyof heavysmoking(25-50%)
. restrictive pulmonaryfunctiontests+ diminished . often asymptomatic(evenwith disseminateddisease)
diffusingcapacityon pulmonaryfunctiontests with insidiousonset
. unresponsive antibiotics . pleuriticchestpain (dueto peripherallocation)
to broad-spectrum
. no organismidentified . cough(35-60% ),hemoP tY si(11% s )
. bronchorrhea = abundantwhitemucoid/ watery
Location: mainlymid + lower lung zones;often
subpleural(50%)and peribronchiolar expectoration(5-27"h); can producehypovolemia
+ electrolyte depletion;unusual+ late manifestation only
distribution(30-50%)
with diffusebronchioloalveolar carcinoma
CXR:
. shortnessof breath(15%)
frequentlymixtureof:
. weightloss (13h),lever (8o/")
r/ uni- / bilateralpatchyalveolarairspaceconsolidation
(25-73/.), often subpleural Location: peripherally,beyonda recognizablebronchus
Spread: tracheobronchial dissemination = cellsdetach
r/ g-S mm nodules(up to 50%)
^/ irregularlinearopacities(15-42%) from primarytumor + attachto alveolarsepta
(5-31%) elsewherein ipsi-/ contralateral lung;
{ unilateralfocali lobarconsolidation
(1 3 % ) lymphogenous + hematogenous dissemination
{ pleur alt hic k e n i n g
{ cavitation/ pleuraleffusion(.5%) Metastases: involvingalmostany organ(in 50-60%);
HRCT : 33% of skeletalmetastasesare osteoblastic
ri patcnyairspaceconsolidation(80%) A. LOCALFORM (60-eo%)
(a) bilateralin 90% involvingall lungzones 1. Ground-glass attenuation
(b) subpleurallperibronchial in 50-60%
distribution = early stage (dueto lepidicgrowthpatternalong
ri patcnyground-glass opacities(dueto alveolitis) in 60% alveolarseptawith relativelackof acinarfilling)
'V S-S mm centrilobularnodules(30-50%) due to r/ ground-glass haziness
organizedpneumonia ^/ bubbtetike hyperlucencies / pseudocavitation
r/ air bronchograms = cylindrical bronchialdilatationin ^/ airwaydilatation
areasof airspaceconsolidation(36-74%) r/ lesionpersists/ progresseswithin6-8 weeks
r/ pleuraleffusion(28-35%) 2. Single mass (43%)
r/ adenopathy(27%) { well-circumscribed focalmass in peripheral I
Rx: improvementwith corticosteroid therapy(in 84% of subpleurallocationarisingbeyonda recognizable
patientswith idioPathic form) bronchus
Prognosis; persistentabnormalities(30%);10% mortality ./ "openbronchussign"= air bronchogram = tumor
due to progressive/ recurrentdisease / mucussurroundingaeratedbronchus
from open lung biopsy + narrowing/ stretching/ spreadingof bronchi
Dx: tissueexamination

{ "rabbitears"/ pleuraltags / triangularstrandI ,,lail
sign"(55%)= linearstrandsextendingfrom
noduleto pleura(desmoplasticreaction/ scarring
, granulomatous disease/ pleuralindrawing)
r/ spiculatedmargin= sunburstappearanci 1ZSX1
{ solitarycavitydue to centralnecrosis(7%)
0 2nd most commoncell type associatedwith
cavitationafter squamouscell carcinoma
./ pseudocavitation (= dilatationof intactair spaces
from desmoplasticreaction/ bronchiectasis
focal emphysema)in 50-60%
^/ heterogeneousattenuatio n (i7%)
r/ confinedto singlelobe
r/ rarelyevolvinginto diffuseform
r/ slowlyprogressivegrowthon serialradiographs
{ NO atelectasis
r/ negativeFDG PET resultsin 55%
Prognosis: 70Y"surgicalcure rate for tumor <S
c m; 4 -1 5 y e a rs ' s u rv i v atilm ew i th
s i n g l en o d u l e
B. DIFFUSEFORM= Pneumonicform (10-40%)
1. Diffuseconsolidation (30%)
r/ acinarairspaceconsolidation+ air bronchogram
+ poorlymarginatedborders
r/ airspaceconsolidationmay affectboth lungs
(mucussecretion)
r/ + cavitationwithinconsolidation
r/ "CT angiogramsign" = low-attenuation
consolidation does not obscurevessels(mucin-
producingsubtype)
2. Lobarform
r/ + expansionof a lobe with bulgingof interlobar
fissures
3. Multinodularform (27%)
{ muftiplebilateralpoorly/ well-defined
similarto metastaticdisease
! multiplepoorlydefinedareasof ground-glass
attenuation/ consolidation
r/ pleuraleffusion(B-10%)
Prognosis.' worse with extensiveconsolidationI
multifocal/bilateraldisease;deathwithin
3 years with diffusedisease
BRONCHOCENTRIC
GRANU
LOMATOSIS
Age: 4th-7th decade
. asthma with underlyingallergicbronchopulmonary
aspergillosis(33-50%)
. fever,nightsweats,cough,dyspnea,pleuriticchestpain
. seropositivearthritis(rare)
. ocularscleritis(rare)
Path: thick-walledectaticbronchi+ bronchioles
containingviscousmaterialof mucopurulent /
caseouscharacter
Histo:necrotizing granulomassurrounding smallainruays;
pulmonaryarteritisas a secondaryphenomenon
(1) largemassesof eosinophils in necroticzones,
associatedwith endobronchial mucusplugs,
eosinophilicpneumonia,Charcot-Leyden
crystals,fungalhyphaein granulomas(with
asthma)
ChestDisorders 467
(2) polymorphonuclear cell infiltratein necrotic
zones(withoutasthma)
Location: unilateral(75%);upper lobe predominance
r/ branchingopacities/ atelectasis( from mucoid
impaction)
r/ multiple/ solitarynodules/ masses
^/ ill-definedparenchymal consolidation
d + cavitation
Rx: corticosteroid therapy
/
BRONCHOG
ENICCARCINOMA
= LUNGCANCER = LUNGCARCINOMA
Most frequentcause of cancerdeathsin males (35% of all
cancerdeaths)and females(21"hof all cancerdeaths);
mostcommonmalignancyof men in the world;6th leading
cancerin womenworldwide
Prevalence:in 1991 161,000 new cases;143,000deaths
Age at diagnosis: 55-60 years (range 40-80 years);
M : F= 1 . 4 : 1
. asymptomatic(10-50%) usuallywith peripheraltumors
. symptomsof centraltumors:
. cough(75%),wheezing,pneumonia
. hemoptysis(50%),dysphagia(2%)
. symptomsof peripheraltumors:
. pleuriticI localchest pain, dyspnea,cough
. Pancoastsyndrome,superiorvena cava syndrome
. hoarseness
. symptomsof metastaticdisease(CNS,bone,liver,
adrenalgland)
. paraneoplastic syndromes:
. cachexiaof malignancy
. clubbing+ hypertrophicosteoarthropathy
. nonbacterial thromboticendocarditis
. migratorythrombophlebitis
nodules . ectopichormoneproduction:hypercalcemia,
syndromeof inappropriatesecretionof antidiuretic
hormone,Cushingsyndrome,gynecomastia,
acromegaly
Types:
1. Adenocarcinoma (50%)
0 Mostcommoncelltype seen in women
+ nonsmokers!
Intermediate malignantpotential(slowgrowth,high
incidenceof early metastases)
D oubl i ngti me: -150-180days
Histo: formationof glands i intracellular mucin
Subtype: bronchioloalveolarcarcinoma
Location: almostinvariablydevelopsin periphery;
frequentlyfoundin scars(tuberculosis,
infarction,scleroderma,bronchiectasis)
+ in closerelationto preexisting
bullae
{ solitaryperipheralsubpleuralmass (52%)I
alveolarinfiltrate / multiplenodules
may invadepleura+ grow circumferentially around
lungmimickingmalignantmesothelioma
upperlobedistribution (69%)
air broncho-i bronchiologram on HRCT (65%)
calcification in peripheryof mass (1%)
smoothmargin/ spiculatedmargindue to
desmoplasticreactionwith retractionof pleura
468 Radiology Review Manual
2. Squamous cell carcinom? = epidermoid
carcinoma (30-35%)
0 Stronglyassociatedwith cigarettesmoking
Histo: mimicsdifferentiation of the epidermisby
producingkeratin("epidermoid
carcinoma"); centralnecrosisis common
Histogenesis: chronicinflammationwith
squamousmetaplasia, progression
to dysplasia+ carcinomain situ
. positivesputumcytology
0 Mostcommoncelltype diagnosedthat is
radiologically occult!
. hypercalcemiafrom tumor-elaborated parathyroid
hormonelike substance
0 Slowestgrowthrate, lowestincidenceof distant
metastases
Doublingtime: -90 days
(a) Centrallocationwithinmain / lobar/ segmental
bronchus(2/3)
^/ largecentralmass + cavitation
{ distalatelectasist bulgingfissure(dueto mass)
r/ postobstructive pneumonia
0 All casesof pneumoniain adultsshouldbe
followedto completeradiologicresolution!
r/ airwayobstructionwith atelectasis(37%)
(b) Solitaryperipheralnodule(1/3)
r/ characteristiccavitation(in 7-10%)
0 Squamouscell carcinomais the most
commoncell type to cavitate!
./ invasionof chestwall
0 Squamouscell carcinomais the most
commoncelltype to cause Pancoasttumor!
3. Small cell undifferentiatedcarcinoma (15%)
0 Stronglyassociatedwith cigarettesmoking
Rapidgrowth+ high metastaticpotential(early
metastasesin 60-80% at time of diagnosis);
shouldbe regardedas systemicdisease
regardlessof stage;virtuallynever resectable
Doublingtime: -30 days
Path: arisesfrom bronchialmucosawith growth in
submucosa+ subsequentinvasionof
peribronchialconnectivetissue
Histo: small uniformoval cellswith scantcytoplasm;
nucleiwith stippledchromatin;numerous
mitoses+ largeareasof necrosis;in 20o/o
coexistentwith non-smallcell histologictypes
(mostfrequentlysquamouscell)
Subtype: oat cell cancerwith
hyperchromatic nuclei;? related
to Kulchitskycell carcinomas
. smooth-appearing mucosalsurfaceendoscopically
. ectopichormoneproduction:Cushingsyndrome,
inappropriatesecretionof ADH
0 Mostcommonprimarylungcancercausing
superiorvena caval obstruction(due to extrinsic
compression / endoluminal thrombosis/ invasion)l
Location: 90% centralwithinlobar/ mainstem
bronchus(primarytumorrarely
visualized)
r/ typicallylarge hilar lperihilarmass often
associatedwith mediastinal widening(from
adenopathy)
{ extensivenecrosis+ hemorrhage
{ smalllung lesion(rare)
Stagingevaluation:
CT of abdomen+ head,bonescintigraphy,
bilateralbone marrowbiopsies
4. Undifferentiatedlarge cell carcinoma (<5%)
0 Stronglyassociatedwith smoking
Intermediate malignantpotential;rapidgrowth
+ early distantmetastases
D oubl i ngti me: -120 days
Histo: tumor cellswith abundantcytoplasm+ large
nuclei+ prominentnucleoli;diagnosedper
exclusiondue to lackof squamous/ glandular
/ smallcell differentiation
Subtype: giantcell carcinomawith very
aggressivebehavior+ poor
prognosis
largebulkyusuallyperipheralmass >6 cm (50%)
largearea of necrosis
pleuralinvolvement
largebronchusinvolvedin centrallesion(50%)
RISK FACTORS:
(1) cigarettesmoking(squamouscell carcinoma+ small
cellcarcinoma)
- relatedto numberof cigarettessmoked,depth of
inhalation, age at whichsmokingbegan
0 85% of lung cancerdeathsare attributableto
cigarettesmoking!
0 Passivesmokingmay accountfor 25% of lung
cancersin nonsmokers!
(2) radongasr may be the 2nd leadingcausefor lung
cancerwith up to 20,000deathsper year
(3) industrialexposure:asbestos,uranium,arsenic,
chlormethyl ether
(4) concomitant disease:chronicpulmonaryscar
+ pulmonaryfibrosis
Scar carcinoma
7o/"of lung tumors',1"/"of autopsies
Origin: relatedto infarcts(>50%),tuberculosis
scar (<25%)
Histo: adenocarcinoma(72/"), squamouscell
carcinoma(18%)
Location: upperlobes(75%)
0 45%of all peripheralcancersoriginatein scars!
PRESENTATION
{ solitaryperipheralmass with coronaradiata/ pleural
tail sign/ satellitelesion
r/ cavitation(16%): usuallythick-walledwith irregular
innersurface;in 415secondaryto squamouscell
carcinoma,followedby bronchioloalveolar carcinoma
ri centralmass (38%): commonin smallcell carcinoma
ri unilateralhilarenlargement(secondaryto primary
tumori enlargedlymphnodes)
Nodeson CT: 0-10 mm negative,10-20 mm
>20 mm positive
indeterminate,

{ anterior+ middlemediastinal widening(suggests
smallcell carcinoma)
r/ segmental/ lobar/ lung atelectasis(gT%)secondary
to ainruayobstruction(particularlyin squamouscell
carcinoma)
r/ "S sign of Golden"- incompletelobarcollapsewith
, bulgingcontourproducedby primarycentraltumor
tr rat tail terminationof bronchus
r/ bronchialcuff sign = focali circumferential thickening
of bronchialwall imagedend-on(earlysign)
r/ localhyperaeration(due to check-valvetype
endobronchialobstruction,best on expiratoryview)
r/ mucoidimpactionof segmental/lobai bronchus(due
to endobronchialobstruction)
r/ persistentperipheralinfiltrate(30%)= postobstructive
pneumonitis
./ lttOair bronchogram
r/ pleuraleffusion(8^15%)
./ boneerosionof ribs/ spine(9%)
r/ involvement of mainpulmonaryartery(jg%); lobar
+ segmentalarteries(53%)may resultin additional
peripheralradiopacity(due to lung infarct)
{ calcificationin 7"/"on CT (histologically in 14%)
usuallyeccentric/ finelystippled
(a) preexistingfocusof calciumengulfedby tumor
(b) dystrophiccalciumwithintumornecrosis
(c) calciumdepositfrom secretoryfunctionof
carcinoma(eg, mucinousadenocarcinoma)
A ngio:
r/ bronchogenic carcinomasuppliedby bronchialarteries
r/ distortion/ stenosis/ occlusionof pulmonaryarterial
circulation
M ULT I P LEP RI M AR YL U N GC A N C ER S
lncidence: 0.72-3.5%;in 1/3 synchronous,inZl3
metachronous
0 10-32% of patientssurvivingresectionof a lung
cancerwill developa secondprimary!
Dx: biopsymandatoryfor propertherapybecausethe
tumor may have a differentcell type
PARANEOPLASTICMANIFESTATIONS
1. Carcinomatous neuromyopathy(4-15%)
2. Migratorythrombophlebitis
3. Hypertrophicpulmonaryosteoarthropathy
(3-5%)
4. Endocrinemanifestations(15%)usuallywith small
cell carcinoma:Cushingsyndrome,inappropriate
secretionof ADH, HPT,excessivegonadotropin
secretion
LOCATION
60-80% arise in segmentalbronchi
- central:smallcell carcinoma,squamouscell
carcinoma(sputumcytologypositivein 70%);arises
in centralairwayoften at pointsof bronchial
bifurcation,infiltratescircumferentially,
extendsalong
bronchialtree
- peripheral:adenocarcinoma, largecell carcinoma
- upperlobe:lowerlobe = rightlung:leftlung= 3.2
- mostcommonsite: anteriorsegmentof RUL
ChestDisorders 469
- Pancoasttumor (3%)= superiorpulmonarysulcus
tumor,frequentlysquamouscell carcinoma
. atrophyof musclesof ipsilateralupperextremity
due to lowerbrachialplexusinvolvement
. Hornersyndrome(enophthalmos,miosis,ptosis,
anhidrosis) due to sympatheticchain+ stellate
ganglioninvolvement
r/ apicalpleuralthickening/ mass
r/ + soft-tissueinvasion/ bone destruction
- SVC obstruction(5%): often in smallcell carcinoma
TNM STAGING
T1: <3 cm in diameter,surroundedby lung/ visceral
pleura
T2: >3 cm in diameter/ invasionof visceralpleuraI
lobaratelectasis/ obstructivepneumonitis/ at
least2 cm from carina
T3: tumor of any size; less than 2 cm from carina/
invasionof parietalpleura,chestwall,diaphragm,
mediastinalpleura,pericardium; pleuraleffusion
T4: invasionof heart,greatvessels,trachea,
esophagus,vertebralbody,carinai malignant
effusion
N1: peribronchial/ ipsilateralhilarnodes
N2: ipsilateral
mediastinal nodes
N3: contralateralhilar/ mediastinal nodes
STAGINGFOR SMALLCELL LUNGCANCER
Limiteddisease:
1. Primaryin one hemithorax
2. lpsilateralhilaradenopathy
3. lpsilateralsupraclavicular adenopathy
4. lpsi-and contralateralmediastinaladenopathy
5. Atelectasis
6. Paralysisof phrenic+ laryngealnerve
7. Smalleffusionwithoutmalignantcells
Extensivedisease(60-80%):
1. Contralateralhilaradenopathy
2. Contralateralsupraclavicular adenopathy
3. Chestwall infiltration
4. Carcinomatous pleuraleffusion
5. Lymphangitic carcinomatosis
6. Superiorvena cava syndrome
7. Metastasisto contralaterallung
8. Extrathoracicmetastasesto bone (38%),liver
(22-28%),bone marrow(17-23 ), CNS (B-1S%),
retroperitoneum (11"/"),otherlymphnodes
Prognosis: 7-11 monthsmediansurvival;1S-Z0o/o 2-
year disease-freesurvivalrate
SPREAD
1. Directlocalextension
2. Hematogenous (smallcell carcinoma)
3. Lymphaticspread(squamouscellcarcinoma);
tumor
in 10% of normal-sized
lymphnodes
4. Transbronchialspread-leastcommon
DISTANTMETASTASES
@ Bone
(a) Marrow: in 40h at time of presentation
470 Radiology Review Manual

(b) Grosslesionsin 1G-35%: A. MEDTASTTNAL BRONCHOGENTC CYST (86%)

Location: vertebrae(70%),pelvis(40%), Associatedwith: spinalabnormalities
femora(25%) M : F= 1 : 1
r/ osteolyticmetastases(3/4) . usuallyasymptomatic
r/ osteoblasticmetastases(14: . stridor,dysphagia
in smallcell carcinoma/ adenocarcinoma Location: pericarinal(52%),paratracheal (19%),
r/ occultmetastasesin 36% of bone scans (9/");
esophagealwal| (14/"), retrocardiac
@ Adrenals: in 37"/"at time of presentation usuallyon right
@ Brain: asymptomaticmetastaseson brainscan in { rarelycommunicate with tracheallumen
7% (30% at autopsy),in 213multiple ^/ may show esophagealcompression
@ Kidney,Gl tract,liver,abdominallymphnodes B. TNTRAPULMONARY BRONCHOGENIC CYST (14%)
@ Lung-to-lung metastases(in up to 10%,usuallyin M>F
late stage) . infection(75%)
Cx: . dyspnea,hemoptysis(mostcommon)
(1) Diaphragmatic elevation(phrenicnerveparalysis) Location: lower:upper lobe= 2'.1;usuallymedial
(2) Hoarseness(laryngealnerveinvolvement,left > right) third
(3) SVC obstruction(5%): lungcanceris causeof all r/ gOX will eventuallycontainair
SVC obstructionsin g0% DDx: solitarypulmonarynodule,cavitatedneoplasm,
(4) Pleuraleffusion(10%): malignant,parapneumonic, cavitatedpneumonia,lung abscess
lympho-obstructive
(5) Dysphagia:enlargednodes,esophagealinvasion
(6) Pericardialinvasion:pericardial effusion,localized BRONCHOPULMONARY DYSPLASIA
pericardial
thickening/ nodularmasses = RESPIRATOR LUNG
Prognosis.' mean survivaltime <6 months;10-15% = complicotionof prolongedrespiratortherapyof
overallS-yearsurvival;survivalat 40 intermittent PEEPwith highoxygenconcentration
months: squamouscell 30% > largecell Cause: oxygentoxicity+ barotrauma
16/" > adenocarcinoma15o/"> oat cell 1% Pathogenesis: hypoxia+ oxygen toxicity
Rx: - capillarywall damage,leakageof fluidinto interstitium
(1) Surgicalresectionfor non-smallcell histologictypes and pulmonaryedema
Unresectable: involvement of heart,greatvessels, Stage / (0-3 days):
trachea,esophagus,vertebralbody,malignant Path: loss of ciliatedcells + necrosisof bronchiolar
pleuraleffusion mucosa
(2) Adjuvantchemotherapy+ radiationtherapyin ./ nOS patternof hyalinemembranedisease
extensiveresectabledisease Stage II (4-10 days):
(3) Chemotherapy for smallcell carcinoma+ radiation Path: hyalinemembranes,eosinophilic exudate,
therapyfor bulkydisease,CNS metastases, spinal squamousmetaplasia, interstitial
edema
cord compression,SVC obstruction Associatedwith: congestivefailurefrom PDA
r/ completeopacificationwith air bronchogram
fibrosisof interstitium + groupsof emphysematous
alveoli
BRONCHOGENIC
CYST Stage lll (10-20 days):
= budding/ branchingabnormality of ventraldiverticulum Path: fewer hyalinemembranes,persistentinjuryof
of primitiveforegut(ventralsegment= tracheobronchial alveolarepithelium, exudationof macrophages
tree;dorsalsegment= esophagus)between26 and 40 { "spongy"/ "bubbly"coarselineardensities,esp. in
days of embryogenesis upper lobes
lncidence.' most common intrathoracicforegutcyst { hyperaerationof lung
(54-63% in surgicalseries) ./ lower lobe emphysema
Histo: thin-walledcyst filledwith mucoidmaterial,lined Stage lV (atler 1 month):
with columnarrespiratory epithelium,mucous Path: septalwall thickening,dilated+ tortuous
glands,cartilage,elastictissue,smoothmuscle lymphatics
. containsmucusI clearor turbidfluid { same patternas in stage lV
{ sharplyoutlinedroundi oval mass Prognosis: 40/" mortalityif not resolvedby 1 month
{ may containair{luidlevel . ol derchi l d
CT: Cx: (1) Increasedfrequencyof lower respiratorytract
r/ cyst contentsof water density(50%)/ higherdensity infections
(50%) (2) C or pul monal e
O B - US : (3) Focalatelectasis
{ singleunilocularpulmonarycyst (4) Asthmalikeclinicalpicture
r/ echogenicdistendedlungobstructedby bronchogenic (5) Rib fractures,rickets,renalcalcifications(from
cyst chronicf urosemidetherapy
 ChestDisorders 471

(6) Cholelithiasis (hyperatimentation Rx: tube thoracostomy,open drainage,decortication,

+ ? furosemide) thoracoplasty,
muscle-pedicleclosure,
(7) Focalareasof tracheomalacia, tracheat transbronchialocclusions
stenosis,acquiredlobaremphysema
Prognosis: BRONCHOPU
LMONARY
SEQUESTRATION
(1) Completeclearingover monthslyears (1lS) = coflg€flitalmalformationconsistingof
(2) Retainedlineardensitiesin upperlobeemphysema (1) nonfuncti oni ng
l ungsegment
(2e%) (2) no communication with tracheobronchial tree
DDx: (3) systemicarterialsupply
(a) conditionspresentat birth: lncidence; 0.15-6.4%of all congenitalpulmonary
( 1) Dif f us en e o n a tapl n e u m o n i a malformations; 1.1-1.8%of all pulmonary
(2) Meconiumaspiration resections
(3) Totalanomalouspulmonaryvenousreturn r/ usually>6 cm in size
(4) Congenitalpulmonarylymphangiectasia { round loval,smooth,well-defined solidhomogeneous
(b) conditionsdevelopingovertime: mass near diaphragmwith mass effect
(1) Recurrentpneumoniaswith scarring ^/ occasionally fingerlikeappendageposteriorly + medially
(gastroesophageal reflux,TE fistula,immune (anomalousvessel)
deficiency,etc) r/ contrastenhancement of sequestration at the same time
(2) Cysticfibrosis as thoracicaorta on rapidsequentialCT scans
(3) ldiopathicpulmonaryfibrosis r/ multiple/ singleair-fluidlevelsif infected
(c) conditionsnot apparentat birth: ./ surroundedby recurrentpulmonaryconsolidation in a
(1) Wilson-Mikity syndrome lowerlobethat neverclearscompletely
(2) Pulmonaryinterstitial emphysema r/ may communicate with esophagus/ stomach
(3) Patentductusarteriosus(uncommon 0 Pulmonarysequestration with communication to Gl tract
appearance) is termedbronchopulmonaryforegut malformation!
(4) Overhydration DDx: bronchiectasis, lungabscess,empyema,bronchial
(5) Perinatally acquiredviralinfection(especially atresia,congenitallobaremphysema,cystic
cMV) adenomatoid malformation, intrapulmonary
bronchogenic cyst,Swyer-James syndrome,
BRONCHOPLEURALFISTULA pneumonia,arteriovenous fistula,primary/
= BRONCHOPULMONARY FISTULA metastaticneoplasm,herniaof Bochdalek
= cofitrylunication
betweenthe bronchialsystem/ lung
parenchyma+ pleuralspace IntralobarSequestration(7s-96%)
Cause: = enclosedby visceralpleuraof affectedpulmonary
(a) Trauma lobe but separatedfrom bronchialtree
1. Complication of resectional
surgery Etiology: controversial
(pneumonectomy, lobectomy,bullectomy) (1) probablyacquiredin majorityof patients
2. Blunti penetrating trauma (2) earlyappearanceof congenitalaccessory
3. Barotrauma tracheobronchial bud leadsto incorporation within
(b) Lung necrosis one pleuralinvestment
1. Putridlungabscess Path: chronicinflammation fibrosis:multipleirregular
2. Necrotizing pneumonia:Klebsiella, H. cordlikeadhesionsto mediastinum, diaphragm,
influenzae,Staphylococcus, Streptococcus; parietalpleura;multiplecystsfilledwith lluid/
tuberculosis; fungus;Pneumocystis thickgelatinous/ purulentmaterial;vascular
3. Infarction sclerosis
(c) Airwaydisease Age at presentation: adulthood(50% >20 years);
1. Bronchiectasis (veryrare) M : F= 1 ; 1
2. Emphysemacomplicated by pneumonia/ Associatedwith congenitalanomaliesin 6-12%:
pneumothorax skeletaldeformities(4%):scoliosis,rib + vertebral
(d) Malignancy:lungcarcinomawith postobstructive anomalies;esophagobronchial a (a%);
diverticul
pneumonia/ tumornecrosisfollowingtherapy diaphragmatic hernia(3%);cardiac(including
. large/ persistentair leak
tetralogyof Fallot);renal:failureof ascent+ rotation;
. acute/ chronicempyema
cerebralanomalies;congenitalpulmonaryvenolobar
H RCT : syndrome
r/ directvisualizationof bronchopleuralfistula(in 5o%) . about 50% have symptomsby age 20; asymptomatic
r/ peripheralair + fluidcollection(indirectsign) in 15%
Dx: (1) Introduction of methyleneblue intopleural . pain,repeatedinfectionin same location(eg,
space,in 65% dye appearsin sputum recurrentacutelowerlobepneumonias)
(2) Sinography . high-output congestiveheartfailure(in neonatal
(3) Bronchography period)from L-to-Lshunt
472 RadiologyReviewManual

. cough+ sputumproduction, hemoptysis DDx of mass: neurogenictumor,lateralthoracic

Location: posterobasalsegments,rarelyupper lung / neningocele,extramedu llary
withinfissure;L:R = 3:2 rematopoiesis,pleuraltumor
CX R: DDx of cavity: lung abscess,necrotizingpneumonia,
{ recurrenti persistentpneumonialocalizedto lower fungal/ mycobacterial
Pneumonia,
lobe cavitatingneoPlasm,emPYema
rl cavitationand cysts+ fluid levels DDx of cysts: pulmonaryabscess,empyema,
0 Aerationof sequesteredlung via Kohn pores/
communication with tracheobronchialtree! "-
bronchiectasis,
a'
card
emPhYsema,

Bronchogram:
r/ ttO communication of rudimentarybronchialsystem :lli'j;.T,lfjfif;5pJ
of sequestrationwith tracheobronchialtree (rare
exceptions)
A ngio: ExtralobarSequestration(14-25%)
{ usuallysinglelargeartery(meandiameterof 6 mm) = €rcc€SSory lobe with its own pleuralsheath
"Rokitansky lobe"),whichpreventscollateralair
coursingthroughinferiorpulmonaryligamentfrom 1=
- distalthoracicaorta (73%) driftresultingin an airlessroundmass
- proximalabdominalaorta(22%) Etiotogy: developmentof an anomalousaccessoryi
- celiac/ splenicartery supernum erarytracheobronchial foregutbud
- intercostalartery(4%) Path: singleovoid/ rounded/ pyramidalairlesslesion
- anomalousbranchof coronaryartery between0.5 and 15 cm (generally3 to 6 cm) in
ri multipleaa. in 16% (withvesseldiameterof <3 mm) size
{ combinedsystemic+ pulmonaryarterialsupply Histo: resemblesnormallungwith diffusedilatationof
r/ venousdrainagevia bronchioles+ alveolarducts+ alveoli;dilatation
- normalpulmonaryveinsto L atrium(in 95%) of subpleural+ peribronchiolar lymphvessels;
- azygos lhemiazygosvv. / intercostalvv. / SVC coveredby mesotheliallayeroverlyingfibrous
into R atrium(in 5%) connectivetissue;congenitalcysticadenomatoid
CT: malformationtype ll is presentin 15-25/.
^/ single/ multiplethin-walledcystscontainingfluid/ lncidence: 0.5-6% of all congenitallung lesions
mucus/ pus / air-fluidleveli air alone Age: neonatalpresentation; 61% withinfirst6 months
ri mucus-impacted ectaticbronchi1=fat density)in of life;occasionally in utero; M:F = 4:1
sequesteredlung Associated with congenital anomalies in 1545%:
^/ emphysemaborderingnormallung (37%) @ Lung: congenitaldiaphragmatic hernia(20-30/o),
= postobstructive hyperinflationof sequestered eventration/ diaphragmaticparalysis(up to 60'/.),
lung cysticadenomatoid malformation(15-25%), lobar
./ homogeneous / inhomogeneous softtissuemass emphysema,bronchogenic cyst,pectus
with irregularborders excavatum,congenitalpulmonaryvenolobar
ri irregularenhancement(rare) syndrome
{ one / two anomaloussystemicarteriesarisingfrom 0 May coexist/ form part of spectrumwith CAM
aorta(DDx: AVM, interrupted pulmonaryartery, @ Heart: anomalouspulmonaryvenousreturn,
isolatedanomaly,chronicinfection/ inflammation cardiac/ pericardialanomalies(8%)
of lungor pleura,surgicallycreatedshunt) @ Gl tract: epiphrenicdiverticula(2/"), TE fistula
{ prematureatherosclerosis of anomalousarteries (1.5/"),duplicationof Gl tract,ectopicpancreas
0 Mucoidimpactionof bronchussurroundedby @ Others: renalanomaly,vertebralanomaly
hyperinflated lung is CHARACTERISTIC! . respiratorydistress+ cyanosis+ CHF in newborn
O B - US : (dueto shuntingof blood)
r/ sphericalhomogeneous highlyechogenicmass . feedingdifficulties
r/ anomaloussystemicarteryseen by color Doppler . asymptomatic (rarelybecomesinfected)in 10%
Cx: massivespontaneousnontraumaticpleural Locati on: L:R = 4:1;typi cal l w y i thi npl euraspace
l in
hemorrhage, chronicinflammation, fibrosis posteriorcostodiaphragmatic sulcus
betweendiaphragm+ lower lobe (63-77%);
monarySequestrations
Bronchopul mediastinum; withinpericardium; within/
belowdiaphragm(5-15%)
lntralobar Extralobar with bronchialtree);
r/ airless(NO communication
Prevalence 75% 25% in presenceof air connectionwith Gl tractis inferred
Pleuralinvestment visceralpleura own pleura ./ may containcysticareas
Venousdrainage pulmonaryveins systemicveins r/ mediastinal shift(if large)
Symptomatic adulthood first6 months Angio(diagnostic):
Etiology acquired develoPmental r/ arterialsupplyfrom
n. anomalies 15% 50% - aorta as single/ severalsmall branches(80%)
 ChestDisorders 473

- splenic,gastric,subclavian,intercostal branches CANDIDIASIS

(15%) Organism; ubiquitoushumansaprophyte (Candida
- pulmonaryartery(5%) albicansmost commonly)characterizedby
,
r,ivenousdrainagevia blastospheres(yeasts)admixedwith hyphae
- systemicveins (80%)to R heart (lVC, azygos, / pseudohyphae (conventional
stains)
hemiazygos,SVC, portalvein) At risk: patientwith lymphoreticular malignancy
- pulmonaryvein (25%) Entry: (a) aspiration
CX R: (b) hematogenous disseminationfrom Gl tract/
r/ singlewell-defined homogeneous triangularmass infectedcentralvenouscatheter
(mostcommonlylocatedadjacentto posterior . prolongedfever despitebroad-spectrum antibacterial
medialhemidiaphragm) coverage
./ ruOair bronchograms . cough,hemoptysis
r/ small"bump'n on hemidiaphragm / inferior r/ patchyairspaceconsolidationin lower lobe distribution
paravertebralregion r/ interstitialpattern
{ opaquehemithorax + ipsilateral
pleuraleffusion(if r/ diffusemicro-/ macronodulardisease
sequestrationlarge) { pteuraleffusion(25%)
+ air-fluidlevel
CT: CARDIOPU LMONARY SCHISTOSOMIASIS
^/ homogeneous
well-circumscribed
soft-tissue = form of parasiticembolism
densitymass (no bronchialcommunication) Organism; Schistosomamansoni(endemicin Middle
NUC (radionuclide angiography): East,Africa,Atlanticcoastof SouthAmerica,
r/ lackof perfusionduringpulmonaryphasefollowed Caribbean;S. japonicumand S. haematobium
by rapid perfusionin systemicphase (lesscommonly)
DDx: intrathoracickidney,scimitarsyndrome(with At risk: >5 years o{ continuousova secretion
systemicsupplyto affectedlung),hepatic Prerequisite: portal hypertensionwith periportalhepatic
herniationthroughdiaphragm fibrosis
O B - US : Cycle: eggs travelas embolivia portosystemic
0 The vast majorityin fetusesare extralobar! collateralpathwaysto lodge in pulmonary
{ conical/ triangularhomogeneous highlyechogenic musculararteriesand arterioles(50-150 pm in
mass (manyinterfacesfrom multiple diameter)
microscopically dilatedstructures) Pathogenesis:
r/ colorduplexmay demonstrate vascularsupply trappedeggs are antigenicand incitean obliterative
! polyhydramnios (? esophagealcompression, endarteritis(due to delayedhost hypersensitivity)
excessivefluid secretionby sequestration) Path: intra-and perivasculargranulomas,intimal
r/ tetat hydrops(? venouscompression): hyperplasia,medialhypertrophy,concentric
{ edema,ascites collagendepositionand fibrosisof vesselwalls;
rl hydrothorax(obstructedlymphatics+ veins in localizedalveolitiswith eosinophilicinfiltration;
torsedsequestration) pulmonaryinfarction
DDx for chest lesion: Age: 25-35 (rangeof 1-93) years
congenitalcysticadenomatoidmalformation, . graduallyworseninghepatosplenomegaly
neuroblastoma,teratoma,diaphragmatic hernia . dyspnea,cough,chestpain
DDx for infradiaphragmaticlesion: . severehypoxemia,cyanosis,digitalclubbing
neuroblastoma, teratoma,adrenalhemorrhage, CXR:
mesoblastic nephroma,foregutduplication r/ cardiomegaly
Cx: infection(in cases of communicationwith ^l centralpulmonaryarterialenlargement
bronchus/ Gl tract) r/ tiny scatteredlung nodulesoccasionally
Rx: resection(delineationof vascularsupplyhelpful) H R C T:
Prognosis; favorable(worseif pulmonaryhypoplasia r/ nodules,interstitial thickening
present);decreasesin size i disappearsin { patchyground-glassattenuation
up to 65% beforebirth r/ ditatationof right atrium+ rightventricle+ central
pulmonaryarteries
Cx: cor pulmonale(2-33%)
Rx: praziquantel, oxamniquine
Esophageal/ GastricLung
= fore variantof pulmonarysequestration
CASTLEMAN DISEASE
Age: infancy(as it is symptomatic) = ANGIOFOLLICULAR LYMPH NODEHYPERPLASIA = BENIGN
. cough relatedto feeding GIANTLYMPHNODEHYPERPLASIA = ANGIOMATOUS
. recurrentpulmonaryinfections LYMPHOID HAMARTOMA = LYMPHOID HAMARTOMA
{ communicationof bronchialtree of sequestered = diversegroup of rare lymphoproliferative
disordersof
lungwith esophagus/ stomach properties+ biologicbehavior
differinghistopathologic
474 Radiology Review Manual
Histo:
(a) hyaline-vascular Castlemandisease (76-91%)
lymphnode hyperplasia, hyalinization with involuted
germinalcenterspenetratedby capillaries,
prominentcapillaryproliferation with endothelial
hyperplasiain interfollicularareas
(b) plasmacell Castlemandisease(4-9-24%)
sheetsof plasmacellsbetweennormal/ enlarged
follicles;relativelyfew capillaries
Localized / Unicentric Angiofollicular Lymph
NodeHyperplasia
Cause: chronicviralantigenicstimulation with
reactivelymphoidhyperplasiai
developmentalgrowthdisturbanceof
lymphoidtissue
Age: all age groups(peakin 4th decade);M:F = 1:4
Histo: mostlyhyaline-vascular cell type
Location: middlemediastinum + hila,cervicallymph
nodes,mesenteric+ retroperitoneal
nooes
Morphologic types:
(a) solitarywell-circumscribed mass without
associatedadenopathy(50%)
(b) dominantmassdisplacing/ surrounding
contiguousstructures+ lymphadenopathy
(c) multipleenlargedlymphnodesconfinedto one
mediastinal compartment(10%)
. asymptomaticin 58-97%
. cough,dyspnea,hemoptysis
. lassitude,weightloss,fever
. growth retardation
. elevatedsedimentationrate
. lgG, lgM, lgA hypergammaglobulinemia (50%)
. refractorymicrocyticanemia
S iz e: up t o 16 c m i n d i a me te r
CT:
r/ sharplymarginatedsmooth/ lobulatedmass of
muscledensity
r/ spottycoarsecentralcalcifications(5-10%)
r/ enhancingrim (vascularcapsule)
./ intenseenhancementalmostequal to aorta (in
hyalin-vascular type)
r/ slightenhancement(in plasmacelltype)
r/ pleuraleffusion(uncommon)
MR:
^/ heterogeneousmass hyperintensecomparedwith
m us c leon T l Wl
r/ markedlyhyperintenseon T2Wl
./ flow voidsof feedingvesselssurroundingmass
A ngio:
^/ hypervascular masswith intensehomogeneous
blush(hyalin-vascular type)
r/ enlargedfeedingvesselsarisingfrom bronchial/
internalmammary/ intercostalarteries
./ some hypervascularity (plasmacell type)
DDx: indistinguishable from lymphoma
Prognosis: treatment-100%curative
Rx: (1) Completesurgicalresection
(2) Radiation+ steroidtherapy
Disseminated / Multicentric
/ Generalized
AngiofollicularLymphNodeHyperplasia
= potentially malignantlymphoproliferative disorder
Cause: disorderedimmunoregulationwithpolyclonal
plasmacellsfromviralinfectionwith
uncontrolled B-cellproliferation + interleukin-6
dysregulation
Mean age: 40-60 years; M:F = 2:1
Histo: mostlyplasmacell type (66%)with infiltration
of nodes by sheetsof matureplasmacells
Associated with:
(a) hyperplasiawithoutneuropathy
. fatigue,anorexia,skin lesions,CNS disorders
(b) hyperplasia with POEMS(polyneuropathy,
organomegaly, endocrinopathy, monoclonal
proteinemia,skin changes)syndrome
. skin lesions:hypertrichosis, hirsutism,
sclerodermatous thickening, hyperpig mentation,
hemangiomas
lymph . distalsymmetricsensorimotor neuropathy(50%)
. papilledema,pseudotumorcerebri(66%)
. monoclonallgG (75%)
(c) osteoscleroticmyeloma,Kaposisarcoma,AIDS
.i t-O cm largehomogeneous lymphnodesin multiple
/ invading mediastinal compartments
(40%) r/ variablemildcontrastenhancement
r/ peripheralmulticentric adenopathy
r/ hepatosplenomegaly
{ salivaryglandenlargement
r/ ascites
ri lymphocytic pneumonitis(LlP):
interstitial
^/ + itt-defined
centrilobular nodules
^/ ground-glassattenuation
ri air-spaceconsolidation
r/ cysts (due to partial airwayobstructionby
peribronchial + peribronchiolar LIP)
./ thickeningof bronchovascular bundles
Rx: surgicalresection,irradiation, systemic
chemotherapy+ corticosteroids
Prognosis.' mean survivalof 24-33 months
C H E MIC A L P N E U MON ITIS
= inhalationof noxiouschemicalsubstances
(a) organic:organophosphates,paraquat,polyvinyl
chloride,polymerfumes,smoke
(b) nonorganic:ammonia,hydrogensulfide,nitrogen
oxide,sulfurdioxide
(c) metal: cadmium,mercury,nickel,vanadium
Carbamates
= agriculturalinsecticidesfunctioning
as cholinesterase
inhibitor(similarto organophosphates) but with poor
penetration intoCNS
{ pulmonaryedemawith respiratory failure
Paraquat
= ?griculturalherbicide
Exposure: often intentionalingestion
 ChestDisorders 475

Pathophysiology: rapid accumulationin lungs with Pathophysiology: acute chemicalbronchiolitis

productionof superoxideradicals + pneumonitis followedby diffuse
damagingpulmonarycells alveolardamagewith hyaline
CXR (wide radiographicvariation): membraneformation
r/ no abnormality . pulmonaryfunctionimpairment
r/ increasedinterstitial/ granularopacities r/ perivascularhaziness+ fine reticularopacities
r/ pulmonaryedema r/ pulmonaryinterstitial
fibrosis
{ pneumomediastinum Prognosis.'acute inhalationpoisoningusualfyfatal
HRCT :
r/ bilateraldiffuseareas of ground-glassattenuation
evolvingintoconsolidation with bronchiectasis, C H R ON IC E OS IN OP H ILIC P N E U MON IA
irregularlines,tractionbronchiectasisof interstitial = 1'rurTl€fous
eosinophils,macrophages,histiocytes,
fibrosis lymphocytes,PMNswithinlung interstitium + alveolar
SACS
Etiology: unknown
HydrogenSulfide A g e : m i d d l e - a g eM; < F
= irritant+ chemicalasphyxiantgas . commonhistoryof atopia(may occur duringtherapeutic
lndustries; coal mines,tanneries,petroleum desensitization procedure)
manufacturingplants,geothermalpower . adult onset asthma(wheezing)
plants,aircraftfactories,sewer works, . highfever,malaise,dyspnea(DDxto Ldfflersyndrome)
rubberworks . peripheralblood eosinophilia(withrare exceptions)r/
Effect: toxic for respiratory(largequantitiescause homogeneous alveolarlunginfiltrates
with distribution
at
inhibitionof medullaryrespiratorycenter) lung periphery= "photographic negative"of pulmonary
+ neurologicsystems edema(bestseen on CT)
. smell of rotteneggs
r/ frequentlybilateralnonsegmental
. "knockdown"= brief loss of consciousnessdue to
{ unchangedfor many days / weeks (DDxto Ldffler
bronchialhyperpresponsiveness syndrome)
. determinationof urinethiosulfatelevels(to monitor ./ fast regressionof infiltratesundersteroids
occupationalexposure) Rx: dramaticresponseto steroidtherapy(within3-10
r/ pulmonaryedema days)

Ammonia C H R ON IC ME D IA S TIN ITIS

= highlysolublecorrosivegas acts as a mucosalirritant Etiology:
lndustries; productionof explosives,petroleum, (1) Granulomatous infection:histoplasmosis
(most
, agriculturalfertilizer,plastics frequent),tuberculosis,
actinomycosis,
Nocardia
! pulmonaryedema (2) Mediastinalgranuloma
Prognosis; completerecovery;bronchiectasis (3) Fibrosingmediastinitis
(4) Radiationtherapy
+ bronchiolitis
obliteransmay develop

Hydrocarbon Mediastinal
Granuloma
Exposure: ingestion/ aspiration(eg, accidental = relativelybenignmassivecoalescentadenitiswith
poisoningin children;fire-eating caseating/ noncaseatinglesions
performers) Cause: primarylymph node infection(commonly
Path: (a) acute phase: intraalveolar, intrabronchial, tuberculosis
/ histoplasmosis)
peribronchial,interstitial
accumulation of Histo: thin fibrouscapsulesurrounding granulomatous
inflammatorycells + edema lesion
(b) chronicphase(1-2 weeksafterinitialonset): r/ lymphadenopathy
proliferative
bronchiolitis,parenchymal DDx: fibrosingmediastinitis(infiltrative,
rare)
f ibrosis,pneumatoceleformation
{ uni-/ bilateralconsolidation,well-defined nodules FibrosingMediastinitis
{ pneumatoceles(fromcoalescingareas of bronchiolar = SCLEROSING MEDIASTINITIS = MEDIASTINAL
necrosis/ partialobstructionof bronchiallumen) COLLAGENOSIS = MEDIASTINAL FIBROSIS
= ut"tcorTlrnon
benigndisordercharacterizedby
Mercury proliferation
of densefibroustissuewithinmediastinum
Exposure: inhalationof mercuryvapor Cause: abnormalhost immuneresponseto
Industries; electrolysis,manufactureof thermometers, Histoplasma capsulatumantigen(organisms
cleaningof boilers,smeltingsilverfrom recoveredin 50%);autoimmunedisease,
dentalamalgamcontainingmercury methysergide-induced
476 Radiology Review Manual

May be associatedwith: retroperitonealfibrosis,orbital NUC:

pseudotumor, Riedelstruma ! unilateraldecreased/ absentperfusionwith normal
Path: ill-defined soft-tissuemasswith minimalI no ventilation(in focalhilarfibrosis)
apparentgranulomatousfoci rl largesegmental/ smallersubsegmentalunmatched
Histo: abundantpaucicellular fibroustissue perfusiondefects
infiltrating+ obliteratingadiposetissue r/ ventilationdefectsin lobar/ segmentalocclusion
Age: 2nd-5th decadeof life; M = F Angio(withtherapeuticintent):
. symptomsof centralairwayobstruction: { unilateral/ asymmetricnarrowingof central
. cough (41%),dyspnea(32%) pulmonaryarteries/ distalarterialcutoffs
. symptomsof pulmonaryvenousocclusion: { funnel-likepulmonaryvein stenosis/ obstruction /
. "pseudo-mitral stenosissyndrome"- progressive focal dilatationnear left atrium
exertionaldyspnea,hemoptysis(31%) Cx: (1) Compression of SVC (64%)+ pulmonary
. cor pulmonale(secondaryto pulmonaryarterial veins (4%)
hypertensioncausedby compressionof pulmonary (2) Chronicobstructivepneumonia(narrowingof
arteries/ veins) trachea/ centralbronchi)in 5%
. dysphagia(2%) (3) Esophagealstenosis(3%)
. superiorvena cava syndrome(6-39%) (4) Pulmonaryinfarcts+ fibrosis(narrowingof
. low leftatrialpressure+ widelydifferentialelevationof pulmonaryarterY)
pulmonarycapillarywedgepressures (5) Prominentintercostalarteries(narrowingof
Location: middlemediastinum (subcarinal pulmonaryarterY)
+ paratracheal regions)and hila Rx: resection,ketoconazole, steroidtherapy(limited
Site: right> leftside of mediastinum success)
CX R: DDx: (1) Bronchogenic carcinoma
^i nonspecific wideningof mediastinum: (2) Lymphoma
r/ distortionof normallyrecognizableinterfaces (3) Metastaticcarcinoma
r/ lobulated(in 86% calcified)paratracheallhilar (4) Mediastinalsarcoma
MASS
{ typicatlyunilateralpulmonaryarterialobstruction: SYNDROME
CHURG.STRAUSS
r/ enlargement of main pulmonaryartery+ right = ALLERGIC ANGITIS ANDGRANULOMATOSIS
heart = vationtof polyarteritisnodosain asthmaticpatients
r/ diminutionin size + quantityof vessels C LA S S ICTR IA D :
r/ localizedregionaloligemia (1) A l l ergi crhi ni ti sor asthma(phase1)
{ pulmonaryvenousobstruction: (2) Peripheralblood+ tissueeosinophilia with Ldffler
r/ peribronchialcuffing,septalthickening syndrome(phase2)
ri ipsilateralKerleyB lines (3) Systemicsmall-vessel granulomatous vasculitis
./ pulmonaryinfarct (phase3), usuallydevelopswithin3 yearsof onset
{ centralairwaynarrowing: of asthma
{ segmental/ lobaratelectasis Etiology: ? hypersensitivity responseto an inhaledagent
r/ recurrentpneumonia Age: 20-40 (mean28) years; M:F = 1:1
UGI: Path: (1) necrotizing vasculitis
^/ circumferentialnarrowing/ long-segmentstricture (2) eosinophilic tissueinfiltration
of esophagusat junctionof upper+ middlethirds (a) eosi nophi l ipneumoni
c a
^/ "downhill"esophagealvarices (b) eosinophilic gastroenteritis
"allergic"granulomas/
CT: (3) extravascular
focal mass (82%): eosinophilic abscesses
r/ calcifiedin 63%; in rightparatracheali subcarinal . allergicrhinitis,sinuspain,headaches,asthma
I hilar location . fever,malaise,gastrointestinal symptoms,arthralgias
r/ diffuselyinfiltrativeprocess(18%): . eosinophilia (almost100%): peripheraleosinophilia in
r/ softtissue attenuation,no calcification >3OYo
r/ obliterationof normalmediastinalfat planes . p-ANCA(perinuclearantineutrophil cytoplasmic
r/ encasement/ invasionof adjacentstructures autoantibodies) in 70%
mediastinal+ hilar nodal masses with coarse . elevatedrheumatoidfactorin 52h
calcifications { vascularaneurysms+ thrombosis
{ wedge-shaped peripheralconsolidation
of venous/ @ Lung: intraalveolar hemorrhage
arterialinfarction { normalCXR (25%)
MR: { oftentransientperipheralwidespreadnonsegmental
r/ heterogenousinfiltrativemass of intermediate air-spaceopacitieswithoutzonalpredominance
signalintensityon TlWl r/ diffusemiliarynodules:
{ mixtureof regionsof increased+ markedly r/ nodulesmay coalesceup to 2 cm (rare)
decreasedsignalintensityon T2Wl r/ cavitationis atypical(and suggestsinfection)
 ChestDisorders 477

r/ eosinophilic pleuraleffusions(29%) B. Trauma

HRCT: 1. Closedi penetrating chesttrauma/ birthtrauma
! consolidation / ground-glass attenuation(59%) (25%): latentperiodof 10 days
./ pulmonarynodules 2. Surgery(2nd most commoncause):
r/ interlobarseptalthickening esophagectomy/ cardiovascularsurgery,esp.
r/ bronchialwall thickening coarctationrepair(0.5%),retroperitoneal surgery,
@ Ol tract (20h): ulceration,hemorrhage,perforation neck surgery
. diarrhea,bleeding,obstruction
3. Subclavianvenouscatheter
r/ mesentericvasculitis C. Neoplasm(54%)
r/ bowelwall infiltrationby eosinophils 1. Lymphoma(mostcommoncause)
@ Heart(up to 47%): coronaryvasculitis,myocarditis, 2. Metastaticcancer
pericardialtamponade(accountingfor 50% of deaths) D. Fibrosingconditions
0 Higherfrequencyof cardiacinvolvementthan 1. Mediastinitis
Wegenergranulomatosis 2. Tuberculosis
@ CNS: diffuseneuritis,mononeuritis multiplex,cerebral 3. Filariasis(rare)
hemorrhage E. Obstructionof centralvenoussystem/ thoracicduct
@ Skin: palpablepurpura F. ldiopathic / cryptogenic(15%): mostcommoncause
@ Kidney: in neonatalperiod
., renalartery-inducedhypertension,hematuria
G. Transdiaphragmatic passageof chylousascites
r/ glomerulonephritis Age: in full-terminfants;may be presentin utero;
0 Less frequent+ less severerenaldiseasecompared M : F= 2 : 1
with Wegenergranulomatosis+ microscopic lncidence: 1:10,000deliveries
polyangitis May be associated with:
Prognosis: 85/" S-yearsurvival; death from cardiac/ Trisomy21, TE-fistula, extralobarlungsequestration,
intraabdominal complications,cerebral congenitalpulmonarylymphangiectasia
hemorrhage, renalfailure,statusasthmaticus . high in neutralfat + fattyacid (lowin cholesterol):
Rx: corticosteroids, cyclophosphamide . triglyceride level>110 mg/dl
. milkyviscoidfluid(chylomicrons) afteringestionof milk/
formulaand clearduringfasting
r/ usuallyunilateratloculaiedpleuraleffusion
(a) rightchylothorax due to duct disruptioninferiorto
CHYLOTHORAX T5-6 (morecommon)
= leakageof chyle(= lymphcontainingchylomicrons (b) left-sidedchylothoraxif duct disruptedabove T5-6
= sUSpeflded fat) from thoracicduct or its branchesinto r/ low attenuation(fat)i high attenuation(proteincontent)
pleuralspace secondaryto obstruction/ disruptionof r/ t leakageof lymphangiographic contrast
thoracicduct (in 2%) ! polyhydramnios (? resultof esophagealcompression)
Route of thoracic duct: Cx: (1) Pulmonaryhypoplasia
Origin: arisesfrom cisternachylianteriorIo Llr12 (2) Hydrops(congestiveheartfailuresecondaryto
(10-15 mm in diameterand 5-7 cm long) impairedvenousreturn)
Course: entersthoraxthroughaortichiatus;ascends Rx: (1) Thoracentesis (leadingto lossof calories,
in rightprevertebrallocation(betweenazygos lymphocytopenia, hypogammaglobu linemia)
vein + descendingaorta);swingsto left atT4_ (2) Total parenteralnutrition
6 posteriorto esophagus;ascendsfor a short (3) Thoracicduct ligation(if drainageexceeds
distancealong rightof aorta;crossesbehind 1,500mUdayfor adultsor 100 mUyr-age/day
aorticarch; runs ventrallyat T3 betweenleft for children>5 yearsof age;drainage>14 days)
commoncarotidartery+ left subclavianartery (4) Pleuroperitoneal shunt;tetracycline pleurodesis;
Termination: 3-5 cm aboveclavicleat venousangle mediastinal radiation;intrapleural
fibringlue;
(= junctionof left subclavian+ internal pleurectomy
jugularveins)
Variation: two (33%)or more (in up to 50%) main COALWORKER'S
PNEUMOCONIOSIS
ducts each consistingof up to g separate = CWP= ANTHRACOSIS = ANTHRACOSILICOSIS
c ha n n e l s = codl dust inhalationtaken up by alveolarmacrophages,
Etiology: in part clearedby mucociliaryaction(particlesize
A. Developmentaldefects >5 pm), in part depositedaroundbronchiofes+ alveoli,
1. Thoracicduct atresia coal dust in itselfis inert,but admixedsilicais fibrogenic
2. Lymphangiectasia
3. Lymphangioma SimpleCWP
4. Lymphangiomatosis (rare): mediastinal / thoracic = €lggregates
of coaldust= co?lmacules
cystichygromaof neckgrowinginto mediastinum (usually
<3 mm)
5. Lymphangioleiomyomatosis + tuberoussclerosis NO progressionin absenceof furtherexposure
478 Radiology Review Manual
Histo: developmentof reticulinfibersassociatedwith
bronchiolardilatation(focalemphysema)
+ bronchiolararterystenosis(decreased
capillaryperfusion)
. poor correlationbetweensymptoms,physiologic
findings+ roentgenogram
r/ smallround1-S-mmopacities,frequentlyin upper
lobes(radiographically only seenthrough
superpositionafter an exposureof >10 years)
r/ nodularitycorrelateswith amountof collagen(NOT
amountof coal dust)
Cx : (1) Chronicobstructivebronchitis
(2) Focalemphysema
(3) Cor pulmonale
coccrDrorDoMYcosls
Organism; dimorphicsoilfungusCoccidioides immitis;
arthrosporesin desertsoil spreadby wind
aerosolizedin dry dust;highlyinfectious
Geographic distribution:
endemicin southwestdesertof USA (San Joaquin
Valley,centralsouthernArizona,westernTexas,
southernNew Mexico)+ northernMexico+ in parts of
Central+ SouthAmerica;similarto histoplasmosis
Mode of infection;depositedin alveoliafter inhalation
+ maturationinto largethick-walled
spheruleswith releaseof hundreds
of endospores
Dx: (1) Cultureof organism
(2) Spherulesin pathologicmaterial(demonstrated
with Gomori-methenamine silverstain)
(3) Positiveskin test
(4) Complementfixationtiter
PrimaryCoccidioidomycosis
= ACUTERESPIRATORY COCCIDIOIDOMYCOSIS
. 60-80% asymptomatic
. "vall€yfever"= influenza-likesymptoms
. desertrheumatism(33%)mostcommonlyin ankle
. r?Sh,erythemanodosum/ multiforme(5-20%)
^/ segmental/lobarconsolidation
r/ patcnyinfiltratesmainlyin lower lobes (46+80%)
frequentlysubpleural+ abuttingfissures
{ peribronchial thickening
r/ hilaradenopathy(20%)
i pleuraleffusion (10%)
ChronicRespiratoryCoccidioidomycosis
Prevalence: 5/. of infectedpatients
. symptomsof postprimarytuberculosis
. hemoptysisin 50%
./ one / severalwell-definednodules
(= coccidioidomycoma) of 5-30 mm in size (in 5%)
{ persistent/ progressiveconsolidation
{ "grapeskin"thin-walledcavities(in 10-15%), in 90%
solitary,70'/oin anteriorsegmentof upper lobes
(DDx:TB), 3% ruptureintopleuralspacedue to
subpleurallocation(pneumothorax/ empyema/
persistentbronchopleural fistula)
^t
bronchiectasis
{ mediastinaladenoPathY(1O-20%\
Disseminated (in1%)
Goccidioidomycosis
= secoflddryphase of hematogenousspreadto
meninges,bones,skin,lymphnodes,subcutaneous
tissue,joints (excePtGl tract)
. skingranulomas/ abscesses
"miliary"lungpattern
{ micronodular
{ pericardialeffusion
LOBAREMPHYSEMA
CONGENITAL
= progressiveoverdistension of one / multiplelobes
M:F=3:1
Etiology:
(a) deficiency/ dysplasia/ immaturityof bronchial
cartilage
(b) endobronchial obstruction(mucosalfold/ web,
prolongedendotrachealintubation,inflammatory
exudate,insPissated mucus)
(c) bronchialcompression(PDA,aberrantleft
pulmonaryartery,puImonaryartery dilatation)
(d) polyalveolar/ macroalveolarhyperplasia
Associatedwith: CHD in 15% (PDA' VSD)
. respiratorydistress(90%)+ progressivecyanosiswithin
first 6 monthsof life
Location: LUL (42-43%), RML (32-35%), RUL (20%)'
two lobes (5%)
{ hazy masslikeopacityimmediatelyfollowingbirth
(delayedclearanceof lungfluidin emphysematous lobe
over 1-14 days)
{ air trapping
{ hyperlucentexpandedlobe (afterclearingof fluid)
r/ compressioncollapseof adjacentlobes
{ contralateralmediastinalshift
{ widelyseparatedvascularmarkings
Mortality: 10%
Rx: surgicalresection
CONG ENITAL LYMPHANGI ECTASIA
1. PRIMARYPULMONARYLYMPHANGIECTASIA (2/3)
= abnormaldevelopmentof lungs between14-20th
week of GA characterizedby anomalousdilatationof
pulmonarylymPhvessels
Path: subpleuralcysts,ectatictortuouslymph
channelsin pleura,interlobular septa+ along
bronchoarterial bundles;NO obstruction
Age: usuallymanifestat birth;50% stillborn;M = F
May be associatedwith: total anomalouspulmonary
venous return,hYPoPlastic
left heart,NoonansYndrome
. respiratorydistresswithinfew hoursof birth
Site: diffuseinvolvement of both lungs,occasionally
only in one / two lobes(withgood prognosis)
{ markedprominenceof coarseinterstitialmarkings
(simulatinginterstitialedema)
i/ hYPerinflation
{ scatteredradiolucentareas (dilatedairways)
r/ patchy areas of pneumonia+ atelectasis
r/ pneumothorax
 ChestDisorders 479

Prognosis: in diffuseform invariablyfatal at CT:

<2 monthsof age ovoid area of increaseddensityin posteriorright
2. GENERALIZEDLYMPHANGIECTASIA hemithorax(= dome of accessorydiaphragm)
= DI F F US EL Y M PH AN G IOMA B . MIN ORC OMP ON E N TS
-- proliferationof mainlylymphaticvascularspaces 1. Trachealtrifurcation (extremelyrare): 2 mainstem
with relentlesssystemicprogression bronchisupplythe rightlung
Age: children,youngadults 2. Eventrationof diaphragm
Location: widespreadvisceral+ skeletalinvolvement 3. Partialabsenceof diaphragm
r/ diffusepulmonaryinterstitial disease 4. Phreniccyst
{ chylouseffusionsin pleural+ pericardial spaces 5. Horseshoelung
tr + lyticbone lesions 6. Esophageal/gastriclung
^/ lymphangiographic poolingof contrastmaterialin 7. Anomaloussuperiorvena cava
dilatedlymphaticchannels/ lymphnodes 8. Absenceof leftpericardium
3. LOCALIZEDLYMPHANGIOMA 0 The mostconstantcomponentsof the syndromeare
= r?f€ benignusuallycysticlesion
hypogeneticlung + PAPVR!
Histo: collectionof dilated+ proliferatedlymph Associated with:
vessels(? hamartomai benignneoplasm/ (1) Vascularanomalies:hypoplastic artery,anomatous
focal sequestrationof ectaticlymphtissue) venousreturn,systemicarterialsupply
Age: first 3 years of life; M = F (2) Anomaliesof hemidiaphragmon affectedside:
. asymptomatic(33%)
{ retrosternalband on lateralCXR due to
. dyspnea(fromtrachealcompression)
mediastinal rotation
Location: neck (80%),mediastinum, axilla,extremity r/ phreniccyst
^/ discretefeaturelessmass r/ diaphragmatichernia
r/ may have chylous/ pleuraleffusion r/ accessoryhemidiaphragm
^/ may have lyticlesionin contiguousskeleton
(3) Hemivertebrae + scoliosis
Prognosis.'propensityfor localrecurrence (4) CHD (25-50%): secundum-type ASD, VSD,
DDx: hemangioma tetralogyof Fallot,PDA, coarctationof aorta,
4. SECONDARYLYMPHANGIECTASIA hypoplastic leftheart,double-outlet rightventricle,
Secondaryto elevatedpulmonaryvenouspressurein double-chambered rightatrium,endocardial cushion
cHD (TAPVR) defect,persistentleftSVC, pulmonarystenosis
. asymptomatic(40%)
. may have dyspneaI recurrentinfections
Locati on: ri ght-si ded predomi nance; M:F = 1.0: 1. 4
CONGENITAL
PULMONARY
VENOLOBAR { hypoplasia / aplasiaof one i morelobesof the lungwith
SYNDROME errorsof lobation(bilateralleft bronchialbranching
= uniqueform of lung hypoplasia / aplasiaaffectingone / pattern/ horseshoelung)
morelobesin a constellation of distinctlydifferent { "scimitarvein"(90%)= partialanomalouspulmonary
congenitalanomaliesof the thoraxthat oftenoccur venousreturn(commonlyinfradiaphragmatic into IVC/
t oget herM ; : F = 1 :1 .4 portalvein lhepaticvein/R atrium),on CXR seen only
A. MAJORCOMPONENTS in 1/3
1. Hypogenetic lung (69%): lobaragenesis/ aplasia/ { systemicarterialsupplyto abnormalsegmentmay be
hypoplasia presentfromthoracicaorta(bronchial, intercostal,
2. Partiafanomafouspulmonaryvenousreturn(31%) transpleural)or abdominalaorta (celiac artery,
= scimitarsyndrome
, transdiaphragmatic)
3. Absenceof pulmonaryartery(14%) ri reticulardensities(enlargedbronchial/ transpleural
4. Pulmonarysequestration(24%) arterialcollaterals)
5. Systemic arterializationof lung without { small hilus (absent/ small pulmonaryartery)
sequestration (10%) { smallrighthemithorax+ mediastinal shift
6. Absence/ interruptionof inferiorvena cava(7"/") ! hazinessof right heartborder
7. Duplication of diaphragm= ?cc€SSory diaphragm { cardiacdextroposition (in rightlunghypoplasia)
(7%) ! anomaliesof bony thorax/thoracic soft tissues
= thin membranein righthemithoraxfused { absentinferiorvena cava
anteriorlywith the diaphragmcoursing { rib hypoplasia / malsegmentation
posterosuperiorly to join with the posteriorchest { rib notching
wafl + trappingaltI part of RML / RLL CT:
r/ accessoryfissurelike obliquelineaboveright r/ smallhemithorax+ mediastinal shift
posteriorcostophrenic sinus(if trappedlungis r/ abnormalities of bronchialbranching
aerated) { anomalouslylocatedpulmonaryfissure
r/ solid mass alongposteriorright hemidiaphragm(if ! discontinuity of hemidiaphragm
trappedlung is unaerated) ri pulmonaryarterialhypoplasia
480 Radiology Review Manual

r/ hyparterialrightbronchus(insteadof eparteriat) Path: proliferationof bronchialstructuresat the expense

{ one / more vesselsincreasingin diametertoward of alveolarsacculardevelopment, modifiedby
diaphragm intercommunicating cysts of varioussize
r/ rind of subpleuralfattytissue in affectedhemithorax (adenomatoid overgrowthof terminalbronchioles,
r/ lack of normalvenousconfluenceof rightlung proliferationof smoothmusclein cyst wall,
DDx: meanderingpulmonaryvein, dextrocardia, absenceof cartilage)
hypoplasticlung,Swyer-Jamessyndrome TYPE | (50%):
Histo: single/ multiplelargecyst(s)>20 mm linedby
ciliatedpseudostratified columnarepithelium,
COSTOCHONDRITIS mucus-Producing cellsin 1/3
= rT'rusculoskeletal
infection Prognosis; excellentfollowingresection
lncidence: increasedwith lV drug abuse TYPE | (a0%):
Agents: Staphylococcus epidermidis,Streptococcus Histo: multiplecysts 5-12 mm linedby ciliated
pneumoniae, Candidaalbicans,Aspergillus cuboidal/ columnarePithelium
CT: Prognosis.' poor secondaryto associatedabnormalities
r/ soft-tissueswelling TY P E i l r (10% ):
r/ cartilagefragmentation,bone destruction Histo: solitarylargebulkyfirm massof bronchuslike
r/ low-attenuationcartilage structureslinedby ciliatedcuboidalepithelium
{ focal peripheralcartilaginouscalcification with 3-5-mm small microcYsts
Rx: surgicalexcision Prognosis.' poor secondaryto pulmonaryhypoplasia/
hydroPs
ln 25% associatedwith: cardiacmalformation,pectus
excavatum,renalagenesis,prune-belly syndrome,
jejunalatresia,chromosomal anomaly,
CRYPTOCOCCOSIS
= TORULOSIS = EUROPEAN BLASTOMYCOSIS bronchopu lmonarYsequestration
Age of detection:children,neonates,fetus; [/;p = 1:1
Organism.' encapsulatedunimorphicyeastlikefungus . respiratorydistress+ severecyanosisin first week of life
Cryptococcusneoformans;sphericalsingle-
(66%)/ withinfirstyear of life (90%)due to compression
buddingyeastcellwiththickcapsule,stains
with
with Indiaink;oftenin soil contaminated of normallung + airwaYs
pigeonexcreta
. superimposed chronicrecurrentinfection(10%)after
first year of life
Histo: granulomatouslesionwith caseousnecroticcenter
Location: equalfrequencyin all lobes(middlelobe
Predisposed: opportunisticinvaderin diabetics
rarelyaffected);more than one lobe involved
+ immunocompromised Patients
. low-grademeningitis(affinityto CNS); M:F = 4:1 in 20o/";mostlyunilateralwithoutside
preference
@ Lung
r/ well-circumscribed mass (40%)of 2-10 cm in CXR:
r/ almostalwaysunilateralexpansilemass with well-
diameter,usuallyperipherallocation
definedmargins(80%)(dueto retainedfetallungfluid
{ lobar/ segmentalconsolidation(35%)
/ type lll lesion)
r/ cavitation(15%)
r/ multipleair-/ occasionally cysts
fluid-filled
./ hilar/ mediastinaladenopathy(12%)
{ compressionof adjacentlung
r/ calcifications(extremelyrare)
{ contralateralshift of mediastinum(87%)
r/ interstitialpneumonia(rare,in AIDS patients)
r/ hypoplastic lung
ipsilateral
@ Musculoskeletal
(5-10%) { properpositionof abdominalviscera
{ osteomyelitis (latesign)
r/ spontaneous pneumothorax
r/ arthritis(rare,usuallyfrom extensionof
CT:
osteomyelitis)
0 Postnatally becomingobstructedand filledwith air
{ solitary/ multiplefluidor air-fluidfilledcystswiththin
walls
{ surrounding focalemphysematous changes
MALFORMATION
CYSTICADENOMATOID OB -U S :
= CAM = cong€oitalcysticabnormalityof the lung
{ singlelargecyst / multiplelargecysts of 2-10 cm in
characterizedby an intralobarmass of disorganized
diameter(TyPel)
pulmonarytissuecommunicatingwith bronchialtree
r/ multiplesmallcystsof 5-12 mm in diameter(typell)
+ havingnormalvascularsupply+ drainagebut delayed ./ large homogeneouslyhyperechoicmass compared
clearanceof fetal lung fluid
with liver(tyPelll)
lncidence: 25/o of congenitallung disorders;
{ contralateralmediastinalshift (89%)
95% of congenitalcysticlung lesions
{ polyhydramnios(2*75'/", ? from compressionof
Cause: arrestof normalbronchoalveolar differentiation
esophagusor increasedfluid productionby abnormal
between5th-7th week of gestationwith (6%)
lung)/ normaltluid(28%)/ oligohydramnios
overgrowthof terminalbronchioles
 ChestDisorders 4 8 1

r/ fetal ascites(62-7i%) . progressiverespiratoryinsufficiencydue to

./ fetat hydrops in gg-Bi% (decreasedvenous return
obstructivelung disease
from compressionof heart lvenacava) Location: predilectionfor apical+ posterior
Riskof recurrence: none
segmentsof upperlobes
Cx: ipsi-/ bilateralpulmonaryhypoplasia { "fingerlike" mucusplugging(mucoidimpactionin
Prognosis: 50/"premature,z5.hstillborn
0 Polyhydramnios, ascites,hydropsindicatea poor , dilatedbronchi)within1st monthof life
r/ subsegmental/ segmental/ lobaratelectasiswith
outcome! right upper lobe predominance(10%)
0 CAM becomessmallerin fetusesin manycasesand r/ progressivecylindricallcystic bronchiectasis(in
occasionally almostdisappearsby birth! 100%at >6 monthsof age)+ air-fluidlevelsdue to
DDx: (1) Congenitallobaremphysema prolongedmucuspluggingpreponderant in upper
(2) Diaphragmatic hernia lobes
(3) Bronchogeniccyst (smallsolitarycyst near
r/ parahilarlineardensities+ peribronchial cuffing
m idt in e ) r/ focal peripheralI generalizedhyperinflation
(4) Neurentericcyst
(5) Bronchialatresia , secondaryto collateralair drift into blockedairways)
r/ nitaradenopathy
(6) Bronchopulmonary sequestration(less r/ largepulmonaryarteries(pulmonaryarterial
frequentlyassociatedwith polyhydramnios / hypertension)
hydrops) { recurrentlocalpneumonitis(initiatedby
(7) Mediastinal/ pericardialteratoma staphylococcus / Haemophilus influenza,succeeded
by Pseudomonas)
CYSTICFIBROSIS { allergicbronchopulmonary aspergillosis (with
= MUCOVISCIDOSIS = FIBROCYSTIC DISEASE bronchialdilatation+ mucoidimpaction)
= autosomalrecessivemultisystemdiseasecharacterized CT:
by mucouspluggingof exocrineglandssecondaryto r/ cylindrical(varicose/ cystic)bronchiectasis
(a) dysfunctionof exocrineglandsforminga thick { peribronchial thickening
tenaciousmaterialobstructingconductingsystem r/ bronchiectatic cyst (= b?onchusdirectlyleading
(b) reducedmucociliarytransport intosacculation) in 56%
lncidence; 1:2,000-1:2,500livebirths;almostexclusively r/ interstitialcysts in 32h
in Caucasians(5% carrya CF mutantgene r/ emphysematous bulla(= peripheralair spacewith
allel e );u n u s u aifn B l a c k s(1 :1 7 ,0 0 0 ), longpleuralattachment+ withoutcommunication
Orientals,Polynesians to bronchus) i n 12%
0 The mostcommoninheriteddiseaseamong ^/ periseptalemphysema
CaucasianAmericansl mucusplugs = tubularstructures+ branching
Cause: cysticfibrosistransmembraneregulatorgene pattern
(CFTR)on longarm of chromosome7 buildsa { subsegmental / segmentalcollapse/ consolidations
defectiveion transportproteinfor an epithetial NUC:
chloridechannel;abnormaltransmembrane r/ matchedpatchyareas of decreasedventilation
conductancefor Cl- decreasesosmoticforces + perfusion
and thus luminalwater;>290differentgene Cx: (1) Pneumothorax (ruptureof bulla/ bleb),
mutations(in 70% AFsos) common+ recurrent
Screening (for 6 most common-mutations of CF gene): (2) Hemoptysis(parasitizedbronchialarteries
carrierdetectionrate of 8S/" of NorthernEuropeans, connectto pulmonaryarteries+ veins
90"/.of AshkenaziJews, 50% of AmericanBlacks resultingin AV fistulae)
Age at diagnosis: 1st year of life (7O/"),by age 4 years (3) Cor pulmonale
(80%),by age 12 years(90%);mean (4) Hypertrophicpulmonaryosteoarthropathy
age of 2.9 years;M:F = 1;1 (rare)
. elevatedconcentrations of sodium+ chloride Causeof death: massivemucusplugging(95%)
(>40 mmol/Lfor infants)in sweat Rx: intratracheal instillationof
. decreasedurinaryPABA excretion aerosolizedadenoviral
+ liposomalvector-CFTRgene preparations
. infertilityin males
. increasedsusceptibility @ Gl tract (85-90%)
to infectionby Staphylococcus . chronicobstipation
aureus+ Pseudomonas aeruginosa . failureto thrive
Prognosis; mediansurvivalof 28 years;pulmonary r/ gastroesophageal reflux(21-27%)due to transient
complications are the most predominant inappropriateloweresophagealsphincterrelaxation
cause of morbidityand death (90%) { meconiumplug syndrome 1iSV.,most common
@ Lung cause of colonicobstructionin the infant)
. chroniccough
r/ distalintestinalobstructionsyndrome(tO-t S-47%)
. recurrentpulmonaryinfections(reducedmucociliary = meconiumileusequivalentsyndrome(in older
clearanceencouragesPseudomonascolonization) child/ youngadult)
482 Radiology Review Manual

r/ meconiumileus(10-16/" at birth) Histo: dilatationof acini and ducts + clst formation

0 Earliestclinicalmanifestation of cysticfibrosis! . steatorrhea+ malabsorption+ fat intolerancedue to
r/ fibrosingcolonopathy= strictureof rightcolonwith exocrinepancreaticinsufficiency in 80-90% without
longitudinal shorteningsecondaryto high-dose affectingendocrine function (only after 98% of
lipasesupplementation pancreasis damaged)
^/ thickenednodularduodenalmucosalfolds(dueto 0 Cysticfibrosisis the most commoncause of
unbufferedgastricacid, productionof abnormal exocrinepancreaticinsufficiency in patients<30
mucus,BrunnerglandhyPertroPhY) yearsof age!
rl mild generalizedsmall boweldilatationwith diffuse . abdominalpain,bloating,flatulence, failureto thrive
distortion+ thickeningof mucosalfolds(at times . diabetesmellitus(secondaryto pancreaticfibrosis)
involvingcolon + rectum) increasingwith age (in 1% of children+ 13/ool
r/ largedistendedcolonwith mottledappearance adults):
(retainedbulkydry stool) . glucoseintolerance in 30-50%
^/ pneumatosisintestinalis of colon(5%)from air block . 1-2o/"requireinsulintheraPY
phenomenaof obstructivepulmonarydisease . acutepancreatitis(clinicallyrare)
^/ "microcolon" = colonof normallengthbut diminished ^/ diffusepancreaticatrophywithoutfatty replacement
caliber r/ lipomatouspseudohypertrophy of pancreas
ri "jejunizationof colon"= cosrS€redundant r/ generalizedincreasedechogenicity(70-100%)
+ hyperplasticcolonicmucosa(distendedcrypt r/ completei partialfatty replacement(-90 to -120
gobletcells) HU)
{ Crohn disease ri calcificchronicpancreatitis
appendicitis ! pancreaticcystosis = microscopic / 1-3-mmsmall
{ rectalprolapsebetween6 monthsand 3 years in cystsreplacingpancreas(common),occasionally
untreatedpatients(18-23%) macroscoPic cYstsuP to 12 cm
Cx: gastrointestinal perforationwith meconium @ S kul l
peritonitis(50%),volvulusof dilatedsegments, { sinusitiswith opacification of well-developed
bowelatresia,intussusception at an average ethmoi d,sphenoi dsi nuses
maxi l l ary,
age of 1 0 y e a rs(1 % ) r/ hypoplastic frontalsinuses
@ Liver OB -U S :
r/ steatosis(30%)due to untreatedmalabsorption, ^/ hyperechogenicbowel (in up to 60-70/" of fetuses
dietarydeficiencies, hepaticdysfunction, affectedwith cYsticfibrosis)
medications (= initialmanifestation in infants) Prognosis; mediansurvivalof 28 years;2.3 deaths/100
r/ focal (40%)/ multilobular(5-12/") biliarycirrhosis patientsfrom cardiorespiratory causes
from inspissated bile: (78 ), hePaticdisease(4%)
. signsof portalhypertension in multilobularform
(clinicallyin 4-6/", autopticin up to 50%)
r/ portalhypertension(in 1"h of biliarycirrhosis) DIAPHRAGMATIC HEFNIA
+ hepatosplenomegaly + hypersplenism Congenital DiaPhragmatic Hernia
@ Biliarytree = absenceof closureof the pleuroperitoneal fold by 9th
Histo: mucus-containing cysts in gallbladderwall week of gestationalage
. cholestasis(secondaryto CBD obstruction) Embryology:
. symptomsof gallbladder disease(3.6%) ventralcomponentof diaphragmformedby septum
r/ sludge (33%) transversumduring3rd-Sthweek GA; gradually
r/ chofelithiasis (12-24/"): mostlycholesterol stones extendsposteriorlyto envelopesophagus+ great
due to (1) interruptedenterohepaticcirculationafter vessels;fuses with foregutmesenteryto form the
ileal resection/ (2) ilealdysfunctionin distalintestinal posteromedialportionsof the diaphragmby 8th week
obstructionsyndrome GA; lateralmarginsof diaphragmdevelopfrom
r/ gallbladderatony musclesof the thoracicwall;the posterolaterally
r/ microgallbladder (25% at autopsy) locatedpleuroperitoneal foramina(Bochdalek)close
r/ thickenedtrabeculatedgallbladderwall last
r/ subepithelialcysts of gallbladderwall lncidence: 1: 2,2Q0-3,000livebirths(0.0a%);
r/ atresia/ stenosisof cysticduct M:F = 2:1; mostcommonintrathoracic fetal
@ Pancreas anomal y
Pathophysiology: duct obstructionfrom inspissated 0 DelaYedonsetfollowinggrouPB
secretions(= proteinPlugs)as a streptococcalinfection!
resultof precipitationof relatively Etiology:
insolubleproteins (1) delayedfusionof diaphragm(spontaneous self-
Path: progressiveductectasia,pancreaticatrophy, correctionmay occur)/ prematurereturnof bowel
increasedpancreaticlobulation,fibrosisdue to from its herniatedpositionwithinthe umbilical
recurrentacute pancreatitis,replacementby fat coelom
 ChestDisorders 483

(2) insultthat inhibits/ delaysnormalmigrationof the (3) Septumtransversumdefect= defectin central

gut + closureof the diaphragmbetweength and tendon
12thweek of embryogenesis (4) Hiatalhernia= coflg€nitally largeesophagealorifice
CI assification (Wiseman): (5) Eventration (5%) = upwarddisplacementof
L herniationearlyduringbronchialbranching abdominalcontentssecondaryto a congenitally thin
leadingto severebilateralpulmonary hypoplasticdiaphragm
hypoplasia;uniformlyfatal Unilateral eventration may be associated with:
ll. herniationduringdistalbronchialbranching Beckwith-Wiedemann syndrome,trisomy13,
leadingto unilateralpulmonaryhypoplasia; tri somy15,tri somy18
survivalpossible Bilateral eventration may be associatedwith:
lll. herniationlatein pregnancywith compression toxoplasmosis,CMV, arthrogryposis
of othenrvise normallung;excellentprognosis Location:anteromedial on right,total involvement
lV. postnatalherniationwith compressionof on l eftsi de; R :L = 5:1
otherwisenormallung;excellentprognosis r/ smalldiaphragmatic excursions
Associated anomalies in 20% of liveborn and in g0% of ! oftenlobulateddiaphragmatic contour
stillborn fetuses: . respiratorydistressin neonatalperiod(life-threatening
1. CNS (28%): neuraltube defects deficiencyof small ainruays + alveoli)
2. Gastrointestinal (20%): particularlymalrotation, . scaphoidabdomen
oral cleft,omphalocele Herniated organs:
3. Cardiovascular(g-29%) smallbowel(90%),stomach(60%),largebowel
4. Genitourinary (15%) (56%),spleen(54%),pancreas(24/"), kidney(12/"),
5. Chromosomal abnormalities (4%): trisomyjg + 21 adrenalgland,liver,gallbladder
6. Spinaldefects ./ bowel loops in chest
7. IUGR(withconcurrentmajorabnormalityin 90%) r/ contralateralshift of mediastinum+ heart
Location: L:R = 5-9:1 r/ complete(1-2%) I parlialabsenceof diaphragm
0 Right-sided herniasare frequentlyfatall r/ absenceof stomach,smallbowelin abdomen
(1) Bochdalekhernia (85-90%) ^/ passageof nasogastrictube underfluoroscopic
= posterolateral defectcausedby maldevelopment / controlenteringintrathoracicstomach
defectivefusionof the cephalicfold of the r/ incompleterotation+ anomalousmesenteric
pleuroperitoneal membranes attachmentof bowel
lncidence: 1:2,200-i2,500livebirths OB-US(diagnosispossibteby 18 weeksGA):
Location: left(80%),right(15%),bilateral(5%) r/ solid/ multicystic / complexchestmass
Herniatedorgans: ! mediastinal shift
(a) on left: omentalfat (6%),bowel,spleen,left { nonvisualization of fetalstomachbelowdiaphragm
lobe of liver,stomach(rare),kidney,pancreas ./ fetal stomachat levelof fetal heart
(b) on right: partof liver,gallbladder, smallbowel, r/ peristalsisof bowelwithinfetal chest (inconsistent)
kidney ./ paradoxicalmotionof diaphragmwith fetal
mnemonic.' "4 B's" breathing(defectin diaphragmsonographically not
Bochdalek visible)
Back (posteriorlocation) r/ scaphoidfetal abdomenwith reducedabdominal
Babies (age at presentation) circumference
Big (usuallylarge) ri herniatedliverfrequentlysurroundedby ascites
(2) Morgagni hernia (1-2%) r/ polyhydramnios (common,due to partial
= anteromedialparasternaldefect(spaceof Larrey) esophagealobstruction or heartfailure)/ normal
causedby maldevelopment of septum fluidvolumei oligohydramnios
transversum;R > L r/ swallowedfetal intestinalcontrastappearsin chest
lncidence: 1:100,000 (CT amniography confirmsdiagnosis)
Herniatedorgans: omentalfat, transversecolon, Cx: (1) Bilateralpulmonaryhypoplasia
liver (2) Persistentfetalcirculation(postsurgical
Often associated with: pulmonaryhypertension)
chromosomal abnormality, mentalretardation, Prognosis; (1) Stillbirth(35-50%)
heartdefects,pericardialdeficiency (2) Neonataldeath (3S%)
(a) abdominalvisceraI fal may herniateinto 0 Survivalis determinedby size of defect+ time of
pericardialsac entry + associatedanomalies(34% survivalrate if
(b) heart may herniateinto upperabdomen isolated,7o/"wilh associatedanomalies)
mnemonic.' "4 M's" Indicators for poor prognosis:
Morgagni large intrathoracicmass with markedmediastinal
Middle(anterior+ centrallocation) shift,IUGR,polyhydramnios, hydropsfetalis,
Mature(tendto presentin olderchildren) detection<25 weeks MA, intrathoracicliver,dilated
M inus c ul e(u s u a l l ys m a l l ) intrathoracicstomach,other malformations
484 Radiology Review Manual

Mortality: in 10% death beforesurgery; r/ hydrothoraxI hemothoraxindicatesstrangulation

40-50/" oPerativemortality; r/ fracturesof lower left ribs
(a) stomachintrathoracic vs. intraabdominal rl abnormalU-shapedcourseof nasogastrictube
= 60/" vs. 67o above suspectedlevelof hemidiaphragm
(b) polyhydramnios vs. normalamniotic N.B.: tube firstdips belowdiaphragm(rentspares
fluid = 89o/ovs. 45Y" esophagealhiatuswith gastroesophageal
DDx: congenitaladenomatoidmalformation, junctionremainingin its normalposition)
mediastinalcyst (bronchogenic,neurenteric, r/ locationof diaphragmmay be documentedby
thymic) 1. gas{illed bowelconstrictedat site of
diaphragmatic laceration
TraumaticDiaphragmaticHernia 2. bariumstudy
= DIAPHRAGMATIC RUPTURE CT (61% sensitive,87o/o sPecific):
Prevalence: 0.8-1.6'h of all blunttrauma;5/. of all 0 Best detectedon reformattedSAG + COR images!
diaphragmatic hernias,but 90% of all Associatedwith: abdominal+ pelvic injury in 90-94%
strangulated diaphragmatic hernias ! abruptdiscontinuityof hemidiaphragm(73-82%)
Etiology of traumatic rupture of diaphragm: r/ herniationof omentum/ bowel/ abdominalorgans
(a) blunttrauma (5-50%) due to markedincreasein into thorax (55%)
intraabdominal pressure:motorvehicleaccident r/ visualization of peritonealfat / abdominalviscera
(>90%),fall from height,bout of hyperemesis; L:R lateralto lungor diaphragm/ posteriorto crus of
= 3: 1, bi l a te raru
l p tu rei n < 3 .6 % hemidiaphragm
(b) penetrating trauma(50%): knife,bullet,repairof r/ "collarsign" = waistlikeconstrictionof visceraat
hiatushernia levelof diaPhragm(27%)
./ "absentdiaphragmsign"= failureto see diaphragm
0 Usually<1 cm in diameter;detectedat surgery
{ concurrentpneumothorax, pneumoperitoneum,
Herniation of organs (32-58%) in order of frequency:
stomach,colon,smallbowel,omentum,spleen, hemothorax, hemoPeritoneum
kidney,pancreas MR:
. may be asymptomatic for monthsi yearsfollowing ^/ interruption of hypointense bandof diaphragmatic
muscleoutlinedby hyperintense abdominal
trauma,onsetof symptomsmay be so longdelayed
that traumaticevent is forgotten + mediastinal fat
. virtuallyall becomeultimatelysymptomatic,most in Associated iniuries:
<3 years ^/ fracturesof lower ribs/ pelvis$2%)
. Bergqvist triad: r/ intraabdominal injuries(72%):
(1) rib fractures(2) fractureof spine/ pelvis { perforationof hollowviscus
(3) traumaticruptureof diaphragm r/ ruptureof spleen
Location: 77-90-98/" on left side; posterolateral Reasonsfor diagnostic misses:
portionof diaphragmmedialto spleenin a (1) left-sideddefectcoveredby omentum
radialorientation; medialcentraltendonwith (2) rightsideddefectsealedby liver
intrapericardial a (3A%)
herni (3) positivepressureventilation
Size: mosttearsare >10 cm in length (4) associatedinjuriesmask tear: atelectasis,pleural
CX R: effusion,lungcontusion,phrenicnerveparalysis
0 The first posttraumaticCXR is abnormalin 46-77"/" Cx: lifethreateningstrangulationof bowel/ stomach
but nonsPecific! occursin majoritY
0 90% of strangulated herniasare traumatic!
0 Positiveintrathoracicpressurefrom ventilationmay
delay herniation! Prognosis: 30"1"mortalityin unrecognizedcases
0 SerialCXRs may show progressive changes! DDx: eventration;diaphragmaticparalysis;normal
contour variantof acquireddiaphragmatic discontinuity
{ nonvisualization of diaphragmatic
r/ elevatedasymmetric/ irregularcontourof posteriorlyrelatedto congenitalBochdalekhernia
hemidiaphragm (6-11%)
Cave: cephaladmarginof bowelmay simulate
an elevateddiaphragm(lookfor haustra)
{ herniationof air-filledviscus: stomach,colon
r/ shiftof mediastinum + lungto oppositeside E MP H Y S E MA
r/ lowerlobe mass/ consolidation (herniatedsolid = groupof pulmonarydiseasescharacterized by
organ/ omentum/ airlessbowelloop) permanently enlargedair spacesdistalto terminal
^/ inhomogeneousmass with air-fluidlevel in left bronchiolesaccompaniedby destructionof alveolar
hemithorax walls + localelasticfiber network
"chronicobstructivepulmonarydisease
./ mushroomlike massof herniatedliverin right 0 The clinicalterm
hemithorax (COPD)"shouldnot be used in image interpretationllt
r/ "collarsign" = hourglassconstrictionof afferent+ encompasses:asthma,chronicbronchitis, emphysema!
efferentbowel looPsat orifice Prevalence: 1.65 millionpeoplein USA

Cause: imbalancein elastase-antielastase system(due
to increasein elastaseactivityin smokers/
o,-antiproteasedeficiency)causingproteolytic
destructionof elastinresultingin alveolarwall
destruction
. dyspneaon exertion
. irreversibleexpiratoryairflowobstruction(dueto
decreasedelasticrecoilfrom parenchymaldestruction)
. decreasedcarbonmonoxidediffusingcapacity
CXR (moderately sensitive,highlyspecific):
r/ hyperinflated lung (mostreliablesign):
./ low hemidiaphragm (= at lbelowTthanteriorrib)
./ ftat hemidiaphragm (= <1.5cm distancebetween
lineconnectingthe costo-and cardiophrenic angles
, + top of midhemidiaphragm)
ri retrosternalair space >2.5 cm
r/ "barrelchest"= enlargedanteroposterior chest
diameter
r/ saber-sheathtrachea
r/ pulmonaryvascularpruning+ distortion(+ pulmonary
arterialhypertension)
r/ right-heartenlargement
./ bullae
HRCT :
r/ well-definedareasof abnormallydecreased
attenuation withoutdefinablewall (<-910 HU)
Rx: lungvolumereductionsurgery
Centrilobular
Emphysema
= CENTRIACINAR EMPHYSEMA = PROXIMAL ACINAR
EMPHYSEMA
= emphysematouschangesefectivelyaffectingthe
acinusat the levelof 1st + 2nd generationsof
respiratorybronchioles(mostcommonform)
Path: normal+ emphysematousalveolarspaces
adjacentto each other
Histo: enlargementof respiratorybronchioles
+ destruction of centrilobular
alveolarseptain
the centerof the secondarypulmonarylobule;
CHARACTERISTICALLy surroundedby normal
lung;distalalveolispared;severityof
destructionvariesfrom lobuleto lobule
Predisposed: smokers (in up to 50%), coal workers
Cause: excessproteasewith smoking(elastaseis
containedin neutrophils + macrophages
foundin abundancein lungof smokers)
. blue bloater
Centrilobar Emphysema Panacinar Emphysema
ChestDisorders 485
Site: apicaland posteriorsegmentsof upperlobe
+ superiorsegmentof lowerlobe (relatively
greaterventilation-perfusion ratioin upperlobes
favorsdepositionof particulatematterand
releaseof elastasein upperlungs)
CXR (80% sensitivityfor moderate/ severestages): '
r/ irregularscatteredarea of radiolucency(beit
appreciated if lungopacifiedby edema/ pneumonia
i hemorrhage) = area of bullae,arterialdepletion
+ increasedmarkings
ri hyperinflated lung
H R C T:
r/ "emphysematous spaces"(= focal area of air
attenuation) >1 cm in diameterwith centraldot / line
(representingthe centrilobulararteryof secondary
pulmonarylobule)withoutdefinablewall and
surroundedby normallung
r/ pulmonaryvasculardistortion+ pruningwith lackof
juxtaposition of normallung (advancedstage)
PanacinarEmphysema
= PANLOBULAR EMPHYSEMA = D|PPUSE EMPHYSEMA
= GENERALIZED EMPHYSEMA
= emphysematous changeinvolvingthe entireacinus
= uniformnonselectivedestructionof all airspaces
throughoutboth lungs(rare)
Path: uniformenlargementof acinifrom respiratory
bronchioles to terminalalveoli(fromcenterto
peripheryof secondarypulmonarylobule)
secondaryto destructionof lung distalto
terminalbronchiole
Cause: autosomalrecessivealpha-1antitrypsin
deficiencyin 10-15% (proteolytic enzymes
carriedby leukocytesin bloodgradually
destroylungunlessinactivated by alpha-1
proteaseinhibitor)
Age: 6th-7th decade(3rd-4th decadein smokers)
. pinkpuffer
Site: affectswholelung,but moresevereat lung
bases (due to greaterbloodflow)
CXR:
r/ hyperinflatedlung
r/ decreasedpulmonaryvascularmarkings
{ lungdestructionextremelyuniform
H R C T:
^/ diffusesimplification of lungarchitecturewith
pulmonaryseptaland vasculardistortion+ pruning
(difficultto detectearly,ie, priorto considerable
, lungdestruction for lackof adjacentnormallung)
! paucityof vessels
r/ bullae
Paracicatricial
Emphysema
= P E R IFOC A L R E GU LAERMP H Y S E MA
/ IR
= airspaceenlargement+ lungdestruction developing
adjacentto areasof pulmonaryscarring
Usualcause: granulomatousinflammation,organized
pneumonia,pulmonaryinfarction
486 Radiology Review Manual

Path: no consistentrelationshipto any portionof Cx: multiloculation

secondarylobule/ acinus;frequentlyassociated Rx: chest tube drainage
with bronchiolectasisproducing"honeycomblung" lff organizingphase = recluitlTlentof fibroblasts
. littlefunctionalsignificance + capillariesresultsin depositionof collagen
CXR (rarelydetectable) : + granulation tissueon pleuralsurfaces= pleural
- "pleural peel/ pleuralrind"
^/ fine curvilinearreticularopacities+ interposed fibrosis
radiolucentareas Cx: limitedexPansionof lung
HRCT : Rx: decortication(withpersistentsepsisdespite
r/ low-attenuation areas adjacentto areasof fibrosis appropriateantibioticRx + drainageI
(diagnosableonly in the absenceof otherforms of persistentthick pleuralrind trapping
emphysema) underlYing lung)
CT:
r/ thickeningof parietalpleurain 60% on NECT,in 86%
ParaseptalEmphysema on CECT
= DISTAL AC]NAR/ LOCALIZED/ LINEAR EMPHYSEMA r/ increasedthickness+ densityof paraspinalsubcostal
= focalenlargement + destructionof air spacesin one tissue(inflammationof extrapleuralfat)
ri curvilinearenhancement of chestwall boundaryin
site in otherwisenormallung
96% (inflammatory hyperemiaof pleura)
Path: predominantinvolvementof alveolarducts
+ SACS r/ "splitpleura"sign = pleuralfluidbetweenenhancing
Site: withinsubpleurallungand
characteristically thickenedparietal+ visceralpleura
adjacentto interlobularsepta + vessels rl gas bubblesin pleuralspace (gas{ormingorganism/
bronchopleural f istula)
CX R:
r/ area of lucency,frequentlysharplydemarcated DDx: simple/ complicatedparapneumoniceffusion
from normallung (negativeGram + culturestain),malignanteffusion
./ bands of radiopacity(residualvessels/ interstitium) aftersclerotherapy,malignantinvasionof chest
may be Present wall, mesothelioma,pleuraltuberculosis,reactive
mesothelial hyperplasia, pleuraleffusionof
HRCT :
{ peripherallow-attenuation area with remainderof rheumatoiddisease
lung normal
Cx: spontaneous pneumothorax; bullaeformation

EXTRINSIC ALLERGIC ALVEOLITIS

EMPYEMA = HYPERSENSITIVITY PNEUMONITIS
= parapneumoniceffusioncharacterizedbypresenceof = characterizedby an inappropriatehost responseto
pus + positiveculture inhaledorganicallergensthat are often relatedto
patient'soccuPation
Organism; S. aureus,Gram-negative+ anaerobic
bacteria cause: inhalationof organicdust (= particulateorganism
. positiveGram stain / proteincomplex)typicallyof 1-2 pm (always
. positiveculture(anaerobicbacteriamost frequent) <5 pm) particlesize depositedin distalairspaces
Stage of lungactingas antigenfor a type lll + type lV
I exudativephase = inflammationof visceralpleura immunereaction
resultsin increasedcapillarypermeabilitywith Histo: diffusepredominantlymononuclearcell
weepingof proteinaceousfluid into pleuralspace inflammation of bronchioles(bronchiolitis)
+ pulmonaryparenchyma(alveolitis); ill-defined
= sterileexudate
. pH >7.2O granulomasof <1 mm in diameter
. glucose>40 mg/dl (2.2 mmol/L) . asymptomatic(1040%)
. L D H< 1 , 0 0 0l u / L . recurrentepisodesof fever,chills,dry cough,dyspnea
ll fibrinopurulentphase = ?cculTlulationof followingexposureafter6-hourinterval
withinpleural . resolutionof episodicsymptomsaftercessationof
inflammatory cellsand neutrophils
space + fibrin deposition on pleuralsurfaces exposure,abate spontaneouslyover 1-2 days
- earlystagell emPYema . insidiousonsetof graduallyprogressive dyspnea
. WBCs >5x10e/mm3, . reductionin vital capacity,diffusingcapacity,arterialPO,
but no grossPus
. pH between7.0 and7.2 . intracutaneousinjectionof antigenresultsin delayed
. glucoselevel>40 mg/dl hypersensitivitYreaction
. L D H < 1 , 0 0 0l u / L . pfes€rlceof serumprecipitins againstantigen
- late stage ll emPYema . positiveaerosolprovocationinhalationtest
' grosspus (WBC >15,000/cmt) = frankpus . markedlyincreasedcell count with often >50h
. pH <7.0 T-lymphocyteson bronchoalveolar lavage
. glucoselevel<40 mg/dL Location: predominantly midlungzones,occasionally
. LDH > 1 ,0 0 0l u /L lowerlungzones,rarelyupperlungzones

Specificantigens for immunecomplexdisease(type lll
= Arthusreaction):
1. Farmer's lung from moldyhay (Thermoactinomyces
vulgarisor Micropolyspora faeni)
2. Hypersensitivity pneumonitis from forced-air
equipment= Pandora'spneumonitiswith heating/
humidifying I airconditioning systems(thermophilic
actinomycetes)
3. Bird-fancier'slung, pigeonbreeder,slungfrom
proteinin bird serum/ excrements/ feathers
4. Mushroom worker's lung from mushroomcompost
(Thermoactinomyces vulgarisor Micropolyspora faeni)
5. Bagassosisfrom moldysugarcane in sugarmill
(contamination with Thermoactinomyces sacchari/
vulgarisand Micropolyspora faeni)
6. Malt worker's lung from maltdust (Aspergillus
clavatus)
7. Maple bark disease from moldy maplebark in saw
mill (Cryptostromacorticale)
8. Suberosisfrom moldycork dust (penicillium
frequentans)
L Sequoiosisfrom redwooddust (Graphiumspecies)
Thermoph iIic actinomycetes
= bacteria<1 pm in diameterwith morphologic
characteristics of fungi;foundin soil,grains,compost,
freshwater,forced-airheating,coolingsystem,
humidifier, air-conditioning system
lsocyanates
used for large-scaleproductionof polyurethane
polymersin the manufacture of flexible/ rigidfoams,
elastomers,adhesives,surfacecoating)
0 Principalcauseof occupational asthma!
Rx: mask,filter,industrialhygiene,alterations
in forced_
air ventilatorysystem,changein patient'shabits/
occupation/ environment
AcuteExtrinsicAllergicAlveolitis
= heavyexposureto incitingantigenin domestic,
occupational, atmospheric environment
Histo: fillingof air spacesby polymorphneutrophils
+ lymphocytes
Onset of symptoms after exposure: 4-g hours
. fever,chills,malaise,chesttightness,cough,
dyspnea
. scantymucoidexpectoration
. frontalheadache,arthralgia(common)
r/ trtoCXR abnormalitiesin gO-gSz
r/ diffuseacinarconsolidativepattern(edema+ exudate
, fillingalveoli)resolvingwithina few days
! lymphnodeenlargement(unusual,morecommon
with recurrence)
HRCT :
{ small+ mediumroundedopacities(largeactive
granulomas)
./ diffusedenseairspaceconsolidation
(confluent
collectionsof intraalveolarhistiocytes,interstitial
+ intraalveolar edema)
Dx: classicalpresentationof a known exposure
history+ typicalsymptoms+ detectionof serum
precipitins to suspectedantigen
Chest Disorders 487
SubacuteExtrinsicAllergicAlveolitis
= less intensebut continuousexposureto inhaled
antigens,usuallyin domesticenvironment
Histo: predominantlyinterstitiallymphocyticinfiltrate,
poorlydefinedgranulomas, cellular
bronchiolitis
Onset of symptomsafter exposure: weeks - months
. recurrentrespiratory/ systemicsymptoms:
. breathlessness uponexertion,fever+ cough
weightloss,muscle+ joint pain
^/ changesmay be completelyreversibleif presentless
than 1 year
r/ interstitial
nodular/ reticulonodularpattern
H R C T:
r/ poorlydefinedcentrilobular micronodules <5 mm
(cellularbronchiolitis+ smallgranulomas)
r/ widespreadpatchy/ diffuseground-glass
attenuation in 52/" (obstructive
pneumonitis, filling
of alveoliby large mononuclearcellinfiltrates)
r/ areas of decreaied attenuation+ mosaicperfusion
(86%)
ChronicExtrinsicAllergicAlveolitis
= prolongedinsidiousdust exposure
Onset of symptomsafter exposure: months- years
. insidiousprogressiveexertionaldyspnea
indistinguishable from idiopathicpulmonaryfibrosis
Histo: proliferationof epithelialcells+ predominantly
peribronchiolar interstitial
fibrosis
Location: usuallyin mid zones,relativesparingof
lungapices+ costophrenic sulci
r/ irregularlinearopacities(fibrosis)
r/ loss of lung volume (cicatrization atelectasis)
r/ pleuraleffusion(rare)
r/ lymphnodeenlargementmay occur
CT:
r/ fibrosisof middle+ Iowerlungzoneswith relative
sparingof lungbases:
r/ intralobularinterstitial
thickening
r/ irregularinterlobular septalthic[ening
^/ honeycombing
r/ tractionbronchiectasis
r/ focal air trapping/ diffuseemphysema
r/ coexistentsubaiutechanges(dueto continuing
exposure)
FAT EMBOLISM
= obstructionof pulmonaryvesselsby fat globules
followedby chemicalpneumonitis from unsaturated
plasmafatty acids producinghemorrhage/ edema
lncidence: in necropsyseriesin 67-g7% of patientswith
majorskeletaltrauma;however,symptomatic
fat embolismsyndromein 40" (M , F)
Onset: 24-72 hoursaftertrauma
. dyspnea(progressivepulmonaryinsufficiency)
. fever
. systemichypoxemia
. mentationchanges: headaches,confusion
488 Radiology Review Manual

. petechiae(50%)from coagulopathy(releaseof tissue ri abnormalpositionof endotrachealtube:

thromboplastin) r/ overdistension of tube cuff
r/ initlalchestfilm usuallynegative(normalup to 72 hours) r/ protrusionof tube wall beyondexpectedmarginsof
r/ platelikeatelectasis trachea
{ bilateraldiffusealveolarinfiltrates ! extraluminal positionof tip of tube
r/ consolidation (mayprogressto ARDS) Prognosis: 30/" mortality(in 15% within t hour)
NUC: Long-termCx: airwaystenosis/ bronchomalacia;
! mottledperipheralperfusiondefects(1-4 days after recurrentatelectasis / pneumonia
injury),laterenlargingsecondaryto pneumonic
infiltrates
GIANTCELLINTERSTITIALPNEUMONIA
PNEUMONIA
FOCALORGANIZING 0 Almost forhardmetalpneumoconiosis
pathognomonic
= UflresolVing pneumonia/ pneumoniawith incomplete r/ diffusemicronodularpattern
resolutionbeyondI weeks { reticularpattern;in advanceddiseasecoarseand
Prevalence: 5-100/"of all pneumonias(87%ot accompaniedby smallcysticspaces
pneumoniasresolvewithin4 weeks,127o r/ t lymphnodeenlargement
within4-8 weeks) H R C T:
Predisposingfactors: ? age, diabetes mellitus,chronic r/ bilateralareas of ground-glassattenuation
bronchitis,overuseof antibiotics { areas of consolidation
Histo: organizationof intraalveolarexudate+ thickening r/ extensivereticulations
of alveolarsepta/ chronicinflammatory change r/ tractionbronchiectasis
of bronchialmucosa+ obstructivelesionin
bronchioles with organization
. cough,sputum,fever,hemoptysis(in 1/4)
./ itl-defined localizedparenchymal abnormality with SYNDROME
GOODPASTURE
irregularmargin = ANTI-GLOMERULAR BASEMENT MEMBRANE ANTIBODY
r/ decreasein size of mass within3-4 weeks DISEASE
HRCT : = autoimmunediseasecharacterized by
r/ ttat/ ovoid lesionwith irregularmarginin subpleural (1) glomerulonephritis
location/ alongbronchovascular bundle (2) circulating antibodiesagainstglomerular+ alveolar
{ t satellitelesions(44%) + air bronchogram(22%) basement membrane
(3) pulmonaryhemorrhage
Pathogenesis:
/ BRONCHUS
OFTRACHEA
FRACTURE cytotoxicantibody-mediated disease= type ll
= TRACHEOBRONCHIAL TEAR hypersensitivity; alveolarbasementmembranebecomes
Cause: bluntchesttrauma(in 1.5%) antigenic(perhapsviraletiology);lgG / lgM antibody
. delayeddiagnosisis common with complementactivationcausescell destruction
Location:(a) mainstembronchuswithin2.5 cm of carina + pulmonaryhemorrhage, leadsto hemosiderin
(80%);R > L depositionand pulmonaryfibrosis
(b) just above carina(20%) Age peak: 26 years (range 17-78 years); M:F = 7:1
Associated injuries: . i ron-defi ci encyanemi a
r/ fractureof first 3 ribs (53-91"/o),rare in children . hepatosplenomegaly
r/ fractureof clavicle,sternum,scapula(40%) . systemichypertension
^/ pneumothorax(70%) @ Lung
r/ increasingmediastinal + subcutaneous emphysema . precedingupperrespiratoryinfection(in 2/3) + renal
r/ absenceof pleuraleffusion disease
r/ "fallenlungsign"= collaps€dlungdroopsto dependent . mild hemoptysis(72%)with hemosiderin-laden
positionperipherally(lossof anchoringsupportin macrophagesin sputum,commonlyprecedesthe
bronchialtransection) clinicalmanifestations of renaldiseaseby several
r/ inadequatereexpansionof lung despiteadequate months
placementof one / more chesttubes (due to largesize . cough,milddyspnea,basilarrales
of air leak) r/ extensivebilateralair-spaceconsolidation:
r/ elevationof hyoidbone above levelof C3 vertebralbody r/ symmetricconsolidation of perihilararea+ lung
/ elevationof greatercornu lo <2 cm from angle of bases with sparing of lung aPices
mandible(on LAT radiographof spine)due to infrahyoid { air bronchogram
musclerupture+ unopposedactionof suprahyoidmm. ! consolidationreplacedby interstitialpatternwithin
r/ atelectasis(may be late development) 2-3 days (dueto organizationof hemorrhage
CT: resultingin interlobularseptalthickening)
r/ focal peribronchialcollectionsof air ^/ hilarlymphnodesmay be enlargedduringacute
r/ discontinuity/ irregularityof bronchialwall episodes
 ChestDisorders 489

@ Kidney { ribs at peripherydeformedi eroded

' glomerulonephritis with lgG depositsin characteristic { significantamountof calcification/ ossification(DDx:
linearpatternin glomeruli
. hematuria , aneurysmalbone cyst)
r/ mass compressesunderlyinglung
Prognosis; deathwithin3 years (average6 months) Rx: resectioncurative
becauseof renal failure
Rx: cytotoxicchemotherapy,plasmapheresis, bilateral HAMARTOMA OF LUNG
nephrectomy = ffiostcommonbenigntumorof the lung
DDx: idiopathicpulmonaryhemosiderosis lncidence: 0.25/" in population(autopsy);6-g% of all
solitarypulmonaryneoplasms;77o/"of all
GRANULOMA
OF LUNG benignlungtumors
Cause: Etiology:
A. Sarcoidosis 1. Congenitalmalformation of a displacedbronchial
B. Non-sarcoid granulomatous disease anlage
(a) infectious 2. Hyperplasiaof normalstructures
bacterial:TB, gumma 3. C arti l agi nousneopl asm
opportunistic:cryptococcosis 4. Responseto inflammation
parasitic: Dirofilariaimmitis(dog heartworm) Path: solitarymass composedof tissuesnormallyfound
fungal: histoplasmosis, coccidioidomycosis, in this locationin abnormalquantity,mixture,and
nocardiosis arrangement
(b) noninfectious Histo: columnar,cuboidal,ciliatedepithelium, fat (in
foreignbody: talc,beryllium,algae,pollen, 50/"), bone,cartilage(predominates), muscle,
cellulose,lipids,abuseof nasallyinhaled vessels,fibroustissue,calcifications,plasma
drugs,aspirationof medication cellsoriginatingin fibrousconnectivetissue
angiocentriclymphoproliferative disease beneathmucousmembraneof bronchialwall
vasculitides Age peak; Sth+ 6th decade; M:F = 2:1 -3:1
extrinsicallergicalveolitis May be associated with:
Langerhanscell histiocytosis Carneytriad (pulmonarychondromatouslesion,gastric
pulmonaryhyalinizing granuloma leiomyosarcoma, functioningextraadrenal
peribronchial granuloma paraganglioma); pulmonaryhamartomasyndrome
chronicgranulomatousdiseaseof childhood . mostlyasymptomatic
Histo: epithelialcells, lymphocytes, macrophages, . hemoptysis(rare)
giantcellsof Langhanstype . cough,vague chest pain,fever (withpostobstructive
Frequency: constitutesthe majorityof solitarypulmonary pneumonitis)
nodules Location: 2/3 peripheraf; endobronchiafin 3-l 0-20%;
. nonproductivecough
multiplicity (rare)
. shortnessof breath
r/ roundsmoothiobulatedmass <4 cm (averages2.5 cm)
. spontaneouspneumothorax
{ calcificationin 15-20% (almostpathognomonicit ot
CX R: "popcorn"
0 cxR detectionrequiresmultiplegranulomas/ clusters , chondroid type)
r/ fat densityin 50% (D|AGNOST|C)
, of granulomas(individual granulomatoo small)! ! cavitation(extremelyrare)
r/ centralnidus of calcificationin a laminated/ diffuse r/ growthpatterns: slow / rapidi stablewith latergrowth
pattern { usuallydiameterincreaseby 1.5 mm/yeardoubing in
./ absenceof growthfor at least2 years size every 14 years
CT (mosteffectivein nodules<3 cm of diameter,with H R C T:
smoothdiscretemargins): ./ fat densitydetectabtein 34% (-BOto -120 HU)
r/ SO-OOZof pulmonarynodulesdemonstrate r/ calcium+ fat detectablein 1g"h
unsuspectedcalcificationby CT Transthoracicneedlebiopsy: 85% diagnosticaccuracy
DDx: carcinoma(in 10% eccentriccalcificationin DDx: lipoidpneumonia(ill-defined mass/ lunginfiltrate);
preexisting scar/ nearbygranuloma/ true intrinsic granulomatous disease,carcinoidtumor;metastatic
stippledcalcification in largerlesion) mucinousadenocarcinoma, amyloidoma
HAMARTOMA
OF CHESTWALL HEREDITARY
HEMORRHAGIC
TELANGIECTASIA
= MESENCHYMOMA (incorrect
as it impliesneoplasm) = RENDU.OSLER.WEBER SYNDROME
= focal overgrowthof normalskeletalelementswith a = group of autosomaldominantinheriteddisordersthat
benignself-limitedcourse;extremelyrare resultin a varietyof systemicfibrovasculardysplasias
4ge' 1st year of life affectingmucousmembranes,skin,lung,brain,Gl tract:
{ moderate/ largeextrapleuralwell-circumscribed mass (1) telangiectasias
affectingone / more ribs (2) arteriovenousmalformations(AV hemangiomas)
r/ ribs near centerof mass partially/ completelydestroyed (3) aneurysms
490 Radiology Review Manual
Etiotogy: gene that encodestransforminggrowthfactor
bindingProtein
Path: directconnectionsbetweenarteries+ veinswith
absenceof capillaries(telangiectases are small
AVMs)
(a) smalltelangiectasis = focaldilatationof
postcapillaryvenuleswith prominentstress
fibersin pericytesalongluminalborders
(b) fully developedtelangiectasis= markedly
dilated+ convolutedvenuleswith excessive
layersof smoothmusclewithoutelasticfibers
directlyconnectingto dilatedarterioles
. frequentbleedingintomucousmembranes,skin,lungs,
system(dueto
genitourinary system,gastrointestinal
vascularweakness)
of nasalmucosa)
@ Nose(telangiectasis
. recurrentepistaxis(32-85%): more severeover
time in 66%;beginsby age 10, presentby age 21 in
most cases;up to 45 episodesper month
@ Skin
. telangiectases= slil?ll red vascularblemishes
Age: presentin most cases by age 40; increasein
number+ sizewith age
Location: lips,tongue,palate,fingers,face,
conjunctiva, trunk,arms,nailbeds
Lung ( 5- 15% )
@
0 5-15% of patientswith hereditaryhemorrhagic
telangiectasia have pulmonaryAVMs
0 Up to 60% of patientswith pulmonaryAVMs have
hereditaryhemorrhagic telangiectasia
see PULMONARYARTERIALMALFORMATION
@ CNS (cerebralor spinalAVMs)
. subarachnoid hemorrhage
. seizure;paraparesis(lesscommon)
. headache
@ Gl tract(stomach,duodenum,smallbowel,colon)
occasionallyassociatedwith AVMs i angiodysplasia
. recurrentGl bleeding(in 5th-6thdecade)
@ Liver(8-31%)
./ hepatomegaly
^/ presenceof multipleAVMs (betweenhepaticartery
branches+ branchesof hepatic/ portalveins:
r/ simultaneousenhancementof hepaticarteries
+ veins
r/ multipletransientperipheralwedge-shaped
of hepaticenhancement on hepaticarterialphase
r/ widenedtortuoushepaticarteries
ri dilatationof hepaticveins
^/ diffusemottledcapillaryblush on angio
Cx: atypicalcirrhosis,portalhypertension,variceal
Gl hemorrhage, ascites,encephalopathy
Cx: (1) congestiveheartfailure(dueto AV shunting)
(2) cerebralabscess(fromparadoxicalemboli)
HISTOPLASMOSIS
Prevalence: nearly100% in endemicarea; up to 30% in
Central+ SouthAmerica,PuertoRico,West
Africa,SoutheastAsia
Organism; Histoplasma capsulatum= dimorphicfungus;
worldwidemost often in temperateclimates;
widespreadin soil enrichedby birddroppings
of centralNorthAmerica(endemicin Ohio,
Mississippi, St. LawrenceRivervalley;exists
as a spore in soil + transformsinto yeast form
at normalbodYtemPeratures
lnfection: inhalationof wind-bornespores(microconidia
of 2-G p.m,macroconidiaof 6-14 Frm),which
germinatewithinalveolireleasingyeastforms,
which are phagocytizedbut not killedby
macrophages; invasionof pulmonary
lymphatics with spreadto hilar+ mediastinal
lymphnodes;hematogenous dissemination of
parasitizedmacrophages throug hout
reticuloendothelialsystem(spleen!)
granulomas,
Path: sporesinciteformationof epithelioid
necrosis,calcification
Dx: (1) Cutture(sputum,lungtissue,urine,bone
marrow,lymPhnode)
(2) ldentificationof yeastforms stainedwith PAS i
Gomorimethenamine silver
(3) Complementfixationtest (absolutetiter of 1:64
or 4-foldrise in convalescenttiter suggestactive
/ recentinfection)
(4) Serumimmunodiffusion: agargel diffusiontest
(H precipitinband)
Rx: ketoconazole
PulmonaryHistoPlasmosis
A. ACUTEHISTOPLASMOSIS
. mostlyasymptomatic and self-limitingillness(in
ee.5% )
. fever,cough,malaisesimulatingviralupper
respiratoryinfection3 weeks after massive
inoculum/ in debilitatedpatients( infants,elderly)
. positiveskin test for histoplasmosis
^/ generalizedlymphadenoPathY
./ bilateralnonsegmental bronchopneumonic pattern
with tendencyto clearin one area + appearin
another
r/ multiplenoduleschanginginto hundredsof
punctatecalcifications(usually>4 mm) atter9-24
areas months
{ "targetlesion"= centralcalcificationis
P A TH OGN OMON IC
r/ tritar/ mediastinallymphnodeenlargement(DDx:
acuteviral lbacterialpneumonia)
^/ "popcorn"calcification of mediastinal lymphnodes
> 1 0m m
./ >5 spleniccalcifications (40%)
C T:
^i paratracheal/ subcarinalmass with regionsof
low attenuation(necrosis)+ enhancingsepta
B . C H R ON ICH IS TOP LA S MOS IS (0.03%)
Predisposed: individualswithchronicobstructive
pulmonarydisease
Age: adultmiddle-aged whitemen
Pathophysiology: hyperimmunereaction

. congh,low-gradefever,nightsweatssimulating
postprimarytuberculosis
, lymphocytes
I segmentalwedge-shapedperipheralconsolidation
of moth-eatenappearancefrom scatteredfoci of
emphysematouslung
r/ fibrosisin apicalposteriorsegmentsof upper
lobes (indistinguishablefrom postprimaryTB)
adjacentto emphysematousblebs
C. DISSEMINATED HISTOPLASMOSIS
Predisposed: impairedT-cellimmunity;AIDS
Prevalence: 1:50,000exposedindividuals
Pathophysiology: progression of exogenous
infection/ reactivationof latent
focus
. acute rapidlyfatal infection:
. fever,weightloss,anorexia,malaise
. cough (<S0%)
. abdominalpain,nausea,vomiting,diarrhea
. chronicintermittent illness:
. low-gradefever,weight loss,fatigue
. adrenalinsufficiency
r/ normatCXR (>50%)
{ miliary/ diffusereticulonodular patternrapidly
, progressingto diffuseairspaceopacification
r/ hilar + mediastinaladenopathy
r/ hepatosplenomegaly
Cx: arthritis(mostoften knee),tenosynovitis,
osteomyelitis
D. DELAYEDMANIFESTATIONS
r/ nistoptasmoma (= continuedgrowthof primary
focus at 0.5-2.8 mm/year)adjacentto pleura
+ typicallywith laminatedcalcificrings;
in 20% associatedwith: mediastinalgranulomas
r/ broncholithiasis
r/ mediastinal granuloma (morecommon)
= directinfectionof mediastinallymph nodes
Histo: involvednodeswith varyingdegreesof
centralcaseation+ calcification
. usuallyasymptomatic
Location: subcarinal/ rightparatracheal/ hilar
r/ widened
J:T'::,llr*slenra,seo
nodes
+ veins)
./ lobulatedmass of low-densitylymph nodes
3-10 cm in thicknesssurroundedby a 2_5_mm
thick fibrouscapsulecrisscrossedby irregularly
, shapedsepta (CHARACTER|ST|o)
, r/ displacementof SVC / esophagus
r/ fibrosingmediastinitis(lesscommon;
0 Organismrecoveredin only SO%l
HODGKINDISEASE
= diseaseof T cells
lncidence: 0.75/" of all cancersdiagnosedeach year;
4O/" of all lymphomas
Age: bimodal peaks at age 25-90 years and 75_g0
years
Histo: Reed-sternbergcell = binucleatecell with
prominentcentrallylocatednucleolus
ChestDisorders 491
(1) Lymphocytepredominance(5%)
= abundanceof normal-appearing
+ relativepaucityof abnormalcells
. often diagnosedin youngerpeople<35 years
. systemicsymptomsare uncommon
. frequentlyin early stage + localizeddisease
Prognosis; most favorablenaturalhistory
(2) Nodularsclerosis(78%)
= lymph nodestraversedby broad bands of
birefringent collagenseparatingnodules,which
consistof normallymphocytes, eosinophils,
plasmacells,and histiocytes
. 113with systemicsymptoms
r/ typicallylocalizedanteriormediastinalinvolvement
Prognosis; good
(3) Mixedcelfularity(17%)
= diffuseeffacementof lymph nodeswith
lymphocytes,eosinophils,plasmacells + relative
abundanceof atypicalmononuclearand Reed-
Sternbergcells;more commonlyadvancedstage
, presentationand older age
at
! morecommonlyabdominalthan mediastinal
Prognosis; lessfavorable
(4) Lymphocytedepletion(1%)
= paucityof normal-appearing lymphocytes
+ abundanceof abnormalmononuclear and Reed-
Sternbergcells;leastcommonsubtypewith worst
prognosis
Age: older patients
. systemicsymptoms
d disseminatedadvancedstage
Prognosis; rapidlyfatal
Ann Arbor Staging Classification:
Stage | = limitedto one / two contiguousanatomic
regionson same side of diaphragm
Stage ll = 2l anatomicregions/ two noncontiguous
regionson same side of diaphragm
Stage lll = on bothsidesof diaphragm,not extending
beyondlymphnodes,spleen(Stagelll"),
Waldeyer'sring
lll== ryi16extralymphaticorgan/ site
Stage lV = slgsn involvement(bone marrow,bone,
lung,pleura,liver,kidney,Gl tract,skin)
t lymphnodeinvolvement
E = extr?lymphaticsite
S = spfenicinvolvement
SubstageA = absenceof systemicsymptoms
SubstageB = fever,nightsweats,pruritus , >1O/oweight
loss in past 6 months
. painlesslymphadenopathy
. alcohol-induced pain
. unexplainedfevers,nightsweats,weightloss
. generalized pruritus
@ CHEST
At presentation: 67"/"with intrathoracicdisease
Sites of lymphoidaggregates:
1. Lymphnodesin mediastinum
2. Lymph nodesat bifurcationof 1st + 2nd order
bronchi
492 Radiology Review Manual

3. Encapsulated lymphoidcollections on thoracic DDx in treated Patients:

relapse,infection,radiationpneumonitis, drug-
surfacedeePto ParietalPleura
4. Unencapsulated nodulesat pointsof divisionsof inducedlungdisease
moredistallysituatedbronchi,bronchioles, and B. EXTRAPULMONARY MANIFESTATIONS
pulmonaryvessels 1. Mediastinal + hilarlymphadenopathy
Mostcommonmanifestation, presentin
5. Unencapsulated lymphoidaggregateswithin
peribronchial connectivetissue 90-99%, in thoraxcommonlymultiplelymph
6. Smallaccumulations of lymphocytes in nodegroupsinvolved
interlobularsepta + lymphaticchannels Location:
A. INTRAPULMONARY MANIFESTATIONS anteriormediastinal+ retrosternalnodes
Frequency: 6-1 1o/";in4.3% bilateral(more commonlyinvolved(DDx:sarcoidosis) ;
frequentin recurrentdisease) confinedto anteriormediastinum in 40/";
0 Mostcommonlyin nodularsclerosingtype 20% with mediastinalnodeshave hilar
0 Subsequentto hilaradenopathyin ipsilateral lung lymphadenopathy also;hilarlymphnodes
1. Bronchovascular form (mostcommontype of involvedbilaterallY in 50%
Spreadfrom anteriormediastinum to:
involvement)
{ coarsereticulonodular patterncontiguouswith othermediastinal locations,pleura,
mediastinum = directextensionfrom pericardium, chestwall
mediastinal nodesalonglymphatics 0 lnvolvement of multiplelymphnodegroupsin
{ nodularparenchYmallesions 95%!
r/ miliarynodules ./ CXR: on initialfilm adenopathyidentifiedin
r/ endobronchial involvement 50%
^/ lobaratelectasissecondaryto endobronchial r/ necroticlymphnodes(commonlynodular
obstruction(rare) sclerosingtYPe)
./ cavitationsecondaryto necrosis(rare) r/ lymph nodes may calcifyfollowingradiation/
2. Subpleuralform chemotheraPY
{ circumscribedsubpleuralmasses 2. Pleuraleffusion(13%)
{ pleuraleffusion(20-50%)from lymphatic 0 Not of Prognosticsignificance
obstruction Prognosis; usuallyresolvesfollowingtreatment
3. MassivePneumonicform (68%) 3. Pleuralmasses+ Plaques
.l dlffusenonsegmentalinfiltrate(pneumonic (a) sternalerosion
tvpe) (b) invasionof anteriorchestwall
{ massivelobar infiltrates(30%)
r/ homogeneous with
confluentinfiltrates Cx:
shaggYborders 1. SuperimPosed infection
r/ air bronchogram with bulgingborders:necrotizing
r/ consolidation
4. Nodularform bacterialPneumonia
r/ muttiptenodules<1 cm in diameter(DDx: r/ multiptenodularfoci: aspergillosis
metastaticdisease) + nocardiosis

Lymphoma
of Non-Hodgkin
Comparisonof HistologicClassifications

International Working Formulation Rappaport Cl assification

Low grade
A. SmatllymphocYtic Well-differentiatedlymphocytic
predominantly
B. Follicular, smallcleavedcell Nodular,poorlydifferentiatedlymphocytic
mixedsmalland largecell
C. Follicular, Nodular,mixed
Intermediategrade
predominantly
D. Follicular, largecell Nodular,histiocytic
E. Diffuse,smallcleavedcell Diffuse,poorlydifferentiatedlymphocytic
F. Diffuse,mixedsmalland largecell D.iffuse,mixed
G. Diffuse,largecell,cleavedor noncleaved
Highgrade
H. Diffuselargecell,immunoblastic
l. Small,noncleavedcell :
J. Lymphoblastic Undifferentiated
 ChestDisorders 493

r/ bilateraldiffuseconsolidation:pneumocystis
c ar in i i
r/ rapidlydevelopingcavitationwithin
consolidation:anaerobes/ fungus
Dx: by culture,sputumcytology,lung biopsy
2. Drug toxicity
EXTRANODALHODGKTN DTSEASE (15_30%)
@ BONE (5-20%)
0 Duringcourseof diseaseS-92% will developbone
marrowinvolvement
At presentation: 1-4/.; indicativeof widespread
aggressivediseasewith poor
prognosis
Location: dorsolumbarspine > pelvis> ribs > femora
sternum
r/ solitary(33%)/ potyostotic(66%) lesions:
r/ usuallywide ill-defined lesionedge/ sclerotic
, margin
{ lamellated/ "sunburst"periostealreaction
./ predominantly osteolyticwith blurredborders;
, rarely sclerotic/ mixed lytic-sclerotic
r/ fracturesoccur rarelyat presentation
r/ vertebralosteolysiswith collapse/ patchysclerosis/
"ivoryvertebra"
. / mixed lytic+ blasticlesion
r/ gouge defectof anteriorvertebralbody margin(due
to erosionby lymphnodes)
r/ osteolysisof sternum(due to its proximityto thoracic
lymphducts)
@ HE A D& NE C K(< 1 % )
. nasopharyngeal biopsypositivein 2C h
r/ thyroidmass as secondaryinvolvement(2%)
@ CNS ( unc om m o n )
Frequency; secondaryhematogenousinvolvement
in 0.2-0.5%
Location: supratentorial
cerebralcortex+ meninges
in inferioraspectof brain(mostfrequent)
./ leptomeningeal+ choroidplexusmasses
{ white mattermass,typicallyperiventricularlbasal
, ganglionic/ cerebellar
r/ paraneoplastic cerebellaratrophy
r/ epiduralmasswith spinalcordcompression(in
3-7.6%) from extensionof paraspinalnodesthrough
intervertebralneuralforamen:
r/ concomitantvertebralbone involvement (5242%)
@ THYMUS (30-56%)
0 Considereda "lymphnode"in staging
r/ remainsenlargedaftertreatmentin ggZ (dueto
recurrentdisease/ reboundhyperplasia/
persistenceof thymiccysts)
@ OHEST WALL (6.4%)
r/ infiltrationof parasternalsoft tissuesby direct
extensionfrom internalmammarynodes
^/ mass beneath/ betweenpectoralismuscles(rare)
@ HEART (75% at autopsy)
ri pericardialeffusion(withlarge mediastinalmass)
r/ invasionof pericardium + SVC
r/ pericardialnodularmass
@ LIVER(6-20%)
0 Primaryinvolvement very rare
Associatedwith: splenicdisease(almostinvariable)
^/ discretenodules(10%)= miliarylesionsof <10 mm
r/ diffusedisease(87%)= patchyirregularinfiltratesin
portal areas
@ SPLEEN
0 Considereda "nodalorgan"
Frequency; 30-40% at staging laparotomy
^/ diffuseinvolvement(not Oetectante by imaging)
+ splenomegaly
r/ hypoechoichypoattenuating noduleswith reduced
contrastenhancement
MR:
r/ nypo-/ isointensenoduleson TlWl
+ hyperintense on T2Wl
^/ reducedenhancement comparedwith normal
spleen
DDx: reactivesplenomegaly(in 30%)
@ PANCREAS(extremelyrare)
0 Secondaryto contiguouslymphnodedisease
@ ct TR A C T(10-15% )
@ Esophagus(extremelyrare):
r/ esophagealnodules/ irregularnarrowing
@ Stomach(9% of all intestinallymphomas):
^/ narrowrigidobstructivelesion(DDx:scirrhous
carcinoma)
r/ wall thickening+ smoothlylobulatedouterborder
@ Smallintestine:
. spruelikesymptoms,steatorrhea
d abundanceof desmoplasticreaction(DDx from
NHL)
{ infiltrating
(60%);potypoid(26%);ulcerated
(14%)
Prognosis.'poorerS-yearsurvivalrate than with other
formsof the disease
@ GU TRACT (extremelyrare)
r/ perirenalI renalmasses(due to invasionfrom
surroundingnodes)
Cx: increasedrisk for other malignanciesfrom
aggressivetherapy(acuteleukemia,NHL,
radiation-induced sarcoma)
HYDATIDDISEASE
= LUNGECHINOCOCCOSIS
0 Mostcommonsite of secondaryinvolvement in children
+ 2nd most frequentsite in adults
Source: hematogenousspreadfrom liver lesion
Frequency: 15-25% of hydatiddisease
. asymptomatic
. eosinophilia(<25%)
. suddencough attacks,hemoptysis,chest pain, fever
. expectorationof cyst fluid/ membranes/ scolices
. positiveCasoniskin test in 60%
. hypersensitivityreaction(if cyst ruptureoccurs)
Location: lowerlobesin 60%; bilateralin20/"
{ solitary(70-75%)/ muttipte(25-gO%)sharpty
circumscribedsphericalI ovoid masses
r/ sizeof 1-20 cm in diameter(16-20weeksdoublingtime)
494 Radiology Review Manual

r/ bronchusterminatesin dilatedblindpouch
r/ absenceof ipsilateralpulmonarytissue
masswithin
cavity
3. Pulmonary hypoplasia (38%)
= completelyformedbut congenitally smallbronchus
with rudimentary parenchyma+ smallvessels
Developmental causes:
water lily sign
resultingin intrauterine compression of chest
serpent slgn (a) ldiopathic(rare)
(b) Extrathoraciccompression(= Pottersyndrome)
1. Oligohydramnios (renalagenesis,bilateral
Cumbo sign
cysticrenaldisease,obstructiveuropathy,
prematureruptureof membranes)
menlscus slgn 2. Fetalascites
oval mass (c) Thoraciccage compression
1. Thoracicbonedysplasia(Jeune,
thanatophoricdystrophy,Ellis-vanCreveld,
severeachondroplasia)
2. Musculardisease
of Lung Echinococcosis
Presentations (d) lntrathoraciccompression
on Upright ChestRadiograph 1. Diaphragmatic defect
2. Excesspleuralfluid
r/ cyst communication with bronchialtree: 3. Large intrathoraciccyst / tumor
"doublearchsign,""moonsign,"
{ "meniscussign", CT:
"crescentsign" (5%) = thin radiolucentcrescentin r/ smallbronchus+ lobe
uppermostpart of cyst (dueto ruptureof pericystwith 0 Hypogenetic lungis the mostconstantcomponentof
air dissectingbetweenpericyst+ laminated congenital pulmonary venolobarsyndrome!
membrane) May be associatedwith: congenitaltrachealstenosis,
r/ air-fluidlevel= ruptureof all cyst wallswith air bronchitis,bronchiectasis
enteringthe endocyst Location: R:L = 3:1; RML (65%)> RUL (40%)> RLL
./ Cumbosign = air-fluidlevelinsideendocyst+ air (20%)> LUL (20%)> LLL (15%);multiple
betweenpericystand endocystwith an "onionpeel" lobes (45%)
appearance . usuallyasymptomatic (in isolatedhypogenetic lung)
r/ serpentsign = collapsedmembranesinsidecyst . exertionaldyspnea
outlinedby air (afterexpectorationof cyst contents) { smallipsilateral hemithorax+ elevatedhemidiaphragm
r/ "waterlily sign,""signof the Camalotte"= completely r/ diminishedpulmonaryvascularity on involvedside
collapsedcrumpledcyst membranefloatingon the { smallhilumon involvedside (absent/ smallpulmonary
. cyst fluid arterY)
r/ mass withincavity= cfurnpl€dmembranesfall to most i/ mediastinum+ heartshiftedtoward involvedside
dependentportionof cavityaftercomplete ./ indistinctcardiomediastinal borderon involvedside
expectorationof cyst fluid r/ diminishedradiolucency on involvedside
{ frydropneumothorax r/ large ipsilateralapicalcap + bluntedcostophrenicangle
r/ calcificationof cyst wall (0.7%) r/ broad retrosternalband of opacity(LAT view)
{ rib + vertebralerosion(rare)
r/ mediastinalcyst: posterior(65%),anterior(26/"), HorseshoeLung
middle(9%)mediastinum = UftcornrTlonvariantof hypogeneticlung syndromein
Cx: bacterialinfection(aftercyst rupture) whichRLL crossesmidlinebetweenesophagusand
heart+ fuses with oppositelung
LUNGSYNDROME
HYPOGENETIC r/ obliquefissurein leftlowerhemithorax(if both lungs
= collectivenamefor congenitalunderdevelopment of one separatedby pleurallayers)
/ more lobes of a lung separatedinto 3 forms: r/ pulmonaryvessels+ bronchicrossingmidline
1. Pulmonaryagenesis
= compl€teabsenceof a lobe + its bronchus IDIOPATHIC INTERSTITIAL PNEUMONIA
CT: AcuteInterstitialPneumonia
r/ missingbronchus+ lobe(s) = AtP = IACCELERATED INTERSTITIAL PNEUMONIA]
2. Pulmonaryaplasia = DIFFUSEALVEOLARDAMAGE= IDIOPATHIC ARDS
= rudimentary bronchusendingin blindpouch = ACUTEDIFFUSEINTERSTITIAL FIBROSIS= HAMMAN-
+ absenceof parenchyma+ vessels RICHSYNDROME
lnc idenc e; 1 :1 0 ,0 0 0R; :L = 1 :1 = rapidlY progressive fulminant disease of unknown
CT: etiology that usually occurs in previously healthy
r/ absenceof ipsilateralpulmonaryartery subjects + produces diffuse alveolar damage
 ChestDisorders 495

Path: temporallyhomogeneous organizingdiffuse . dyspnea+ dry cough(1-weekto S-yearhistory)

alveolardamage;littlematurecollagen . low-gradefever,malaise
deposition/ architecturaldistortion/ . decreaseddiffusingcapacityfor carbonmonoxide
honeycombing (as opposedto Ulp) Location: no zonalpredominance
Histo: (a)acuteexudativephase: thickeningof alveolar r/ normalCXR in 147o
wall due to alveolar/ interstitialedema r/ bibasilarirregularlinearopacities+ diffuse
+ inflammatory cells;extensiveatveolar
damagewith hyalinemembraneformation , heterogeneousairspaceconsolidation
ri normal/slightlydecreasedlungvolume
(mostprominentin 1stweek afterlung injury) CT:
(b) markedinterstitialfibroblastproliferationwith ^/ bilateralareasof scatteredground-glass
stabilizingnonprogressive scarring opacities(100%)
Mean age: 50 years; M = F r/ bibasilarairspaceconsolidation(71%)
. prodromalviralupperrespiratory infection:cough, r/ irregularlinearopacities(29%)
fever r/ bronchialdilatationin areasof consolidation
(71%)
. rapidlyincreasingdyspnea+ acute respiratoryfailure
r/ mediastinatlymphadenopath y (29%)
. requiresventilationwithindays to 1-4 weeks ^/ ttO honeycombing
. decreaseddiffusingcapacityfor carbonmonoxide
Prognosis: 11"/"overallmortality
Location: mainlylowerlungzones Rx: corticosteroids (clinical+ functional
Site: predominantlycentral/ subpleural(in 22%) + radiographicimprovementin 50-96%)
CXR: DDx: usual interstitialpneumonia(irregularreticular
^/ progressiveextensivebilateralhetero-/ pattern+ honeycombing involvingsubpleural
homogeneous airspaceopacification:symmetric, + lowerlungzones
bilateral,basilar
CT : Respiratory Bronchiolitis-lnterstitiat Lung
r/ diffuseextensivebilateralairspaceconsolidation(in
Disease
, 67%)with basalpredominance(similarto ARDS) = interstitial
pneumoniaof smokersin which
rl patchy(67%)/ diffuse(38%)bitateralground-gtass
respiratorybronchiolitisis associatedwith limited
opacities
peribronchiolarinterstitial
inflammation;? early
{ anteroposterior lung attenuationgradient
manifestationof DIP
r/ markedarchitecturaldistortion + honeycomblung if
Mean age: 36 years; M = F
fibrosisprogressive
Cause: heavycigarettesmoking
Dx: negativebacterialI viral/ fungalcultures;no
Histo: accumulationof brown-pigmented
inhalational exposureto noxiousagents;no
macrophages in respiratorybronchioles
pulmonarydrug toxicity
+ surrounding air spaces
Prognosis: deathwithin 1-6 months(60-90%); . milddyspnea+ cough
recoveryin 12/" . pulmonaryfunctiontest: mixedrestrictive
+ obstructive
r/ normalCXR (21%)
SubacuteInterstitialPneumonia ^/ diffusebibasilarsmalllinear+ nodularopacities
BOOP see BRONCH|OL|TISOBLTTERANS (71%)
r/ bibasilaratelectasis(12%)
r/ bronchialwall thickening
NonspecificlnterstitialPneumoniawith CT:
Fibrosis r/ scatteredground-glassopacities(66%)
= NONCLASSIFIABLE INTERSTITIAL PNEUMONIA r/ centrilobular
micronodules
= interstitialpneumonia that cannot be classifiedas r/ centrilobular
emphysema
UIP / DIP / acuteinterstitial pneumoniai BOOp Prognosis.' excellent(aftercessationof smoking/
Histo: temporaluniformityof corticoidtherapy)withoutprogressionto
(a) cellularinterstitial
infiltratewith little/ no end-stagelung fibrosis
fibrosis(48%)
(b) inflammation + fibrosis(38%) ChronicInterstitialPneumonia
(c) densefibrosisdominant(14%); = ORGANIZINGINTERSTITIAL
PNEUMONIA= CHRONIC
occasionally intraalveolar accumulation of DIFFUSESCLEROSINGALVEOLITIS
macrophages+ focal areas of bronchiolitis
obliteransorganizingpneumonia Usual lnterstitialPneumonia
Cause: collagenvasculardisease(16y"), = U I P= I D I O P A T H I P
CU L M O N A R Y
FIBROSIS
(IPF)
inhalational exposureto noxiousagents = MURALTYPEOF FIBROSINGALVEOLITIS
(17%),recentsurgery/ severepneumonia/ = CRYPTOGENIC FIBROSING ALVEOLITIS
ARDS (8%) = cofirrnonest (90%) form of idiopathic interstitial
Mean age: 46 years; M < F pneumonia (may represent late stage of Dlp)
496 Radiology Review Manual

Etiology: 50% idiopathic;25'htamilial;drug exposure ./ irregularlinearintralobular opacities(82%)with

(bleomycin, cyclophosphamide (Cytoxan@), architecturaldistortionof secondarypulmonary
busulfan,nitrofurantoin) ; 20-30% lobule
associatedwith collagenvasculardisease/ r/ interlobularseptalthickening(10%)
immunologic disorder(mostlyrheumatoid r/ subpleuralareasof honeycombingwith cystic
arthritis) spacesoutlinedby thick fibrouswalls (up to 96%)
Pathophysiology: r/ subpleurallines(= fibrosisi functionalatelectasis)
repetitiveepisodesof lung injuryto the alveolarwall r/ small peripheralconvolutedcysts (= traction
causingalveolito floodwith proteinaceous fluid bronchiectasis) in 50%
+ cellulardebris;incompletelysisof intraalveolar r/ ground-glass opacities(= diffuseinflammatory
fibrin;type ll pneumocytesregenerateoverthe mononuclear cell infiltratesof activedisease
intraalveolar collagenincorporating the fibrous + proliferation)
fibroblast in 65-76%
tissueintoalveolarsepta(= injury-inflammation- Cx: bronchogeniccarcinoma(morefrequent
fibrosissequence) occurrence)
Mean age: 64 years; M>F Rx: responseto steroidsin only 10-15%
Path: simultaneouspresenceof inflammatorycell Prognosis.' averagesurvivalof 3-6 years;45/o
infiltration+ fibroticalveolarwalls S-yearmortalityrate (overall87"/.);no
+ honeycombing + areasof normallung recovery
tissue (= temporalvariegation)
Histo: proteinaceousexudatein interstitium
+ hyalinemembraneformationin alveoli; Desquamativelnterstitial Pneumonia
necrosisof alveolarliningcellsfollowedby = DIP= DESQUAMATIVE TYPEOF FIBROSING
cellularinfiltration of mono-and lymphocytes ALVEOLITIS = ALVEOLAR MACROPHAGE PNEUMONIA
+ regeneration of alveolarlining;intraalveolar = secoodcommonest(althoughrare)form of
histiocytes ; proliferationof f ibroblasts interstitialpneumoniawith morebenigncoursethan
+ depositionof collagenfibers+ smooth UlP, may be self-limited diseaseor leadto UIP
muscleproliferation; progressive Mean age: 42years (approximately 8 years
disorganization of pulmonaryarchitecture Y ounger than i n U IP ); M > F
. progressive dyspnea,dry cough,fatigue(over1-3 Path: focal fillingof alveolarspaceswith foamy
years) histiocytes+ relativepreservationof lung
. "Velcro"rales = crepitations architecture + mildfibrosis(temporally uniform)
. clubbingof fingers(83%) Histo:alveolilinedby largecuboidalcells+ filledwith
. lymphocytosison bronchoalveolar lavage (marker heavyaccumulation of mononuclear cells
of alveolitis) (macrophages,NOT desquamatedalveolar
. pulmonaryfunctiontests: restrictivedefects cells);relativepreservationof alveolaranatomy;
+ decreaseddiffusingcapacityfor carbonmonoxide histologicuniformityfromfieldto field
Predisposed: smokers (historyin up to 90%)
. asymptomatic
r/ occasionallyground-glasspatternin early stage of
edema, . weightloss
alveolitis(alveolarwall injury,interstitial
proteinaceousexudate,hyalinemembranes, . dyspnea+ nonproductive cough(for6-12 months)
. clubbingof fingers
infiltrateof monocytes+ lymphocytes)in 15-62/"
^/ bilateraldiffuselinear/ smallirregularreticulations . mildpulmonaryfunctionabnormalities
(100%);basilar(85%)+ peripheral(59%) ^/ normalchest x-ray (3-22%)
r/ reticulonodular pattern= superimposition of linear ./ ground-glassalveolarpatternsparingcostophrenic
opacities ang les (25-33o/"),d iffuse g round-glass opacities
"shaggy" (15%)
r/ heart border
r/ honeycombing= hUlTleroUs cysticspaces(up to r/ linearirregularopacities(60%),bilateral+ basilar
74%) (46-73%)
r/ elevateddiaphragm= progressiveloss of lung ./ l ungnodul es(15% )
volume (45-75%) ./ honeycombing (13%)
./ t.S-g-mm diffuselydistributednodules(15-29%) ri preservedlungvolume
{ pleuraleffusion(+6/"), pleuralthickening(6%) HRCT:
^/ pneumothoraxin7"h (in late stages) Location: mainlymiddle+ lowerlungzones(73%);
r/ normalCXR (2-8%) bilateral+ sYmmetric(86%)
Site: predominantly subpleuraldistribution (59%)
HRCT (88%sensitive):
Location: lung bases (68-80%) r/ patchyground-glassattenuation
Site: predominantly subpleuralregions(79%) ri irregularlinearopacities(= fibrosis)
r/ patchydistributionwith areas of normal + architecturaldistortion(50%)
parenchyma,activealveolitis,early + late fibrosis ./ honeycombing+ tractionbronchiectasis(32%)
presentat the same time (HALLMARK) r/ fibrosisof lower lung zones in late stage
 ChestDisorders 497

Prognosis; better responseto corticosteroidRx than INFLAMMATORY

MYOBLASTIC
PSEUDOTUMOR
UIP (in 60-80%);mediansurvivalof 12 = PLASMA CELLGRANULOMA = INFLAMMATORY
years;5/o S-yearmortalityrate (overall PSEUDOTUMOR = (FtBROUS) H|ST|OCYTOMA
16-27 %); 70% 1O-yearsurvival = XANTHOMA = XANTHOFIBROMA = XANTHOGRANULOMA
= SCLEROSING HEMANGIOMA
Prevalence: <1"/oof all tumorsof lung + airways
IDIOPATHIC PULMONARY FIBROSIS Histo: composedof variableportionsof plasmacells,
= clinicalsyndrome lymphocytes,fibroblasts,bloodvessels;foamy
Age: 50-70 years; M > F histiocytes giantcells+ spindle
+ multinucleated
. dry cough,exertionaldyspnea cells(70%of tumor)groupedin
' "Velcro-type"inspiratorycrackles CHARACTERISTIC pinwheel/ whorledpattern
. digitalclubbing(25-50%) Age: young patient
. restrictivepulmonaryfunctiontests . asymptomatic
. decreasedtotal lung capacity+ functionalresidual . airwayobstruction(symptomsoften attributedto asthma
capacity+ residualvolume / pneumoni a)
. reduceddiffusingcapacityfor carbonmonoxide Location: lung> bronchus/ trachea> pleura
CX R: ./ smoothlymarginatedmass in trachea
r/ decreasedlung volume,progressiveover time Rx: surgicalexcision
r/ bibasilarsubpleuralreticulations
r/ honeycombing(90%)
r/ bibasilarground-glass appearance(uncommon) KARTAGENER
SYNDROME
r / s m allnodul e s(< 1 0 -1 S % ) = IMMOTILE / DYSMOTILE CILIASYNDROME
H RCT : lncidence: 1:40,00;high familialincidence
r/ patchybibasilarsubpleuralreticular(= irregularlinear) Etiology: abnormalmucociliaryfunctionsecondaryto
, opacities(= intralobular, interstitial
thickening) generalized deficiencyof dyneinarmsof cilia
! tractionbronchiectasis affectingrespiratoryepithelium,auditory
^/ honeycombing (90%) epithelium,sperm
r/ ground-glassopacities(occasionally) Triad: (1) Situsinversus(in 50%)
r/ discretenodules(occasionally) 0 50% of patientswith immotilecilia
r/ mildmediastinal lymphnodeenlargement(common) syndromehavesitusinversusl
Rx: corticosteroids, immunosuppressive/cytotoxic 0 Kartagenersyndromeis presentin 20/" of
agents(<10%respond);lungtransplantation patientswith situsinversus!
Prognosis: 30-50% S-yearsurvival (2) Nasalpolyposiswith chronicsinusitis
(3) Bronchiectasis
IDIOPATHIC PULMONARY HEMOSIDEROSIS . deafness
= IPH = probableautoimmuneprocesswith clinical . infertility
(abnormalspermtails)
+ radiologicremissions+ exacerbationscharacterized Associatedanomalies:
by eosinophilia + mastocytosis,immunoallergic reaction, transpositionof great vessels,tri- / bilocularheart,
pulmonaryhemorrhage, irondeficiencyanemia pyloricstenosis,postcricoidweb, epispadia
Age: (a) Chronicform: most commonly<10 years of age
(b) Acuteform (rare): in adults; M:F = 2:1 K LE B S IE LLA P N E U MON IA
. irondeficiencyanemia Mostcommoncauseof Gram-negative pneumonias;
. clubbingof fingers communityacquired
. hepatosplenomeg aly (25%) lncidence.' responsiblefor 5/" of adult pneumonias
. bilirubinemia Organism; Friedl6nderbacillus=encapsufated,
. recurrentepisodesof severehemoptysis nonmotile,Gram-negative rod
{ bilateralpatchyalveolar-filling pattern(= blood in Predisposed: elderly,debilitated,alcoholic,chroniclung
alveoli);initiallyfor 2-3 dayswith returnto normalin disease,malignancy
. bacteremiain 25%
, 10-12 days unlessepisoderepeated
ri reticularpattern(= depositionof hemosiderinin { propensityfor posteriorportionof upperlobe/ superior
, interstitialspace)later portionof lower lobe
ri moderatefibrosisafter repeatedepisodes r/ denselobarconsolidation
r/ nilarlymphnodesmay be enlargedduringacute r/ butgingof fissure(largeamountsof inflammatory
episodes , exudate)CHARACTERISTIC but unusual
Prognosis; death within 2-20 years (averagesurvival r/ empyema(one of the most commoncauses)
3 years) ri patchybronchopneumonia may be present
DDx: SECONDARYPULMONARYHEMOSTDEROSTS { uni-/ multilocular cavities(50%)appearingwithin4 days
causedby mitralvalve disease r/ pulmonarygangrene= infarctedtissue (rare)
ri septallines(NOT in idiopathicform) Cx: meningitis, pericarditis
ri lung ossifications(NOT in idiopathicform) Prognosis; mortality rate 25-50%
498 Radiology Review Manual

DDx: Acutepneumococcalpneumonia (bulging

of Evolutionary sequence:
fissures, pleural
+ cavityformation,
abscess nodule- cavitatednodule- thick-walled cyst- thin-
/ empyema
effusion frequent) walledcyst (secondaryto progressiveenlargement
+ air trappingof originalcavitarynodule)
./ itt-detined/ stellatenodules1-10 mm (granuloma
LANGERHANSCELL HISTIOCYTOSIS stage):
= LCH= EOSINOPHILIC GRANULOMA = HISTIOCYTOSIS X ^i dittusefine reticular/ reticulonodular pattern
= LANGERHANS CELLGRANULOMATOSIS (cellularinfiltrate)
= group of disorderscharacterizedby abnormalclonal ! cavitationof largenodules(rare)
proliferation of the Langerhanscell (fromthe monocyte- { "honeycomblung"= multiple1-5-cmcysts
macrophagecell line)resultingin granulomatous + subpleuralblebs (fibroticstage)
of lungs,bone,skin,lymphnodes,liver,
infiltration ^/ increasedlungvolumesin 1/3 (mostotherfibrotic
spleen,brain,kidneys,endocrineglands lungdiseaseshavedecreasedlungvolumes!)
Age: most frequentlyin 3rd-4th decade (range3 r/ pleuraleffusion(8%),hilaradenopathy(unusual)
m ont hsto 6 9 y e a rs );M :F = 4 :1 ; C a u c asi ans
>> ri thymicenlargement
Blacks HRCT (combination virtuallydiagnostic):
Histo: { complex/ branchingthin-walled cysts<5 mm in
granulomacontainingLangerhanscells,foamy size equally distributedin central + peripherallung
histiocytes, lymphocytes, plasmacells,eosinophils zones
Langerhanscell: { centrilobular peribronchiolar nodules
- dendriticantigen-presenting cell foundin basallayer ^/ intervening lungappearsnormal
of skin + in liver(Kupffercell),lymphnodes,spleen, DDx for nodules:
bone marrow,lung sarcoidosis,hypersensitivity pneumonitis,berylliosis,
- containsuniquemostlyrod-shapedcytoplasmatic TB, atypicalTB, metastases,silicosis,coal worker's
inclusionbodiesknownas Birbeckgranules pneumoconiosis
(identifiable onlywith electronmicroscopy) DDx for cysts:
@ PulmonaryLangerhansCell Histiocytosis emphysema,bronchiectasis, idiopathicpulmonary
Pathogenesis: fibrosis,lymphangiomYomatosis
heavycigarettesmokingin youngmen with Cx: 1. Recurrentpneumothoraxin 25"/"(trom
accumulation+ activationof Langerhanscells (90% ruptureof subPleuralcYsts)
smokers)as a resultof excessneuroendocrine cell C H A R A C TE R IS TIC
hyperplasia + secretionof bombazine-like peptides 2. PulmonarY hypertension
Path: 3. Superimposed Aspergillus fumigatus
multifocalgranulomatous infiltrationcenteredon infection
walls of bronchioles(= bronchiolitis) often extending
intosurroundingalveolarinterstitium with Prognosis; poor with multisystemdisease+ organ
subsequentbronchiolar destructionleadingto thick- dysfunction(especially with skin lesions);
walledcysts presumablycausedby check-valve (a) completei partialregression(13-55%)
(no necrosis); (b) stable(33%)
bronchialobstruction + pneumothorax
in end-stagediseasefoci of LCG are replacedby (") tv")
M'iXiLiv
fibroblasts formingCHARACTERISTIC stellate
"starfish"scarswith centralremnantsof persisting
fJ.?8;ffiJIJ
:TJfl failure
+ resPiratorY
inflammatory cells Mortality: 2-25o/o
0 CXR abnormalities moreseverethan clinical
symptoms+ pulmonaryfunctiontests! Rx: cessationof smoking,chemotherapy(vincristine
. asymptomatic(up to 25%) sulfate,prednisone,
methotrexate,
. nonproductivecough (75%) 6-mercaptoPurine)
. combinationof obstructive+ restrictivepulmonary
function:presentingwith pneumothorax in 15'/" DDx: sarcoidosis(equalsex distribution,always
. fatigue,weightloss,fever(15-30%) multisystem disease,not relatedto smoking,
. dyspnea(40%) erythemanodosum,bilateralhilar
. chest pain (25%)from pneumothorax/ eosinophilic lymphadenopathy, lung cavitation+ pneumothorax
gr anulom ai n ri b cells)
rare,epithelioid
. diabetesinsipidus(10-25%)
. lymphocytosis with predominance of T-suppressor
cellson bronchoalveolar lavage(DDx: excessof LE GION E LLA P N E U MON IA
T-helpercellsin sarcoidosis) = LEGIONNAIRES' DISEASE
Location: usuallybilaterally symmetric,upperlobe Organism; Legionellapneumophila,1-2 pm, aerobic,
predominance, sparingof costophrenic Gram-negative bacillus,weaklyacid-fast,
angles silver-i stain
mPregnation
 ChestDisorders 499

Predisposed: middle-aged/ elderly,immunosuppressed, 0 The degree+ type of tissuereactiondependon

alcoholism, chronicobstructivelung the frequencyof aspiration+ chemical
disease,diabetes,cancer,cardiovascular characterof the oil
disease,chronicrenalfailure,transplant H i sto: numerousl i pi d-l adenmacropha gesdist ending
recipients alveolarwalls+ interstitium, accumulation of
Transmission:directinhalation(air conditioningsystems) lipidmaterial,inflammatory cellularinfiltration,
Prevalence: 6/" of community-acquired pneumonias variableamountof fibrosis
Histo: leukocytoclastic fibrinopurulentpneumoniawith . mostlyasymptomatic
histiocytesin intraalveolarexudate . fever,constitutionalsymptoms
. fever . lipid-laden macrophagesin sputum/ lavagefluid
. absenceof sputum llack of purulence(22-78%) . oil dropletsin bronchialwashingi needleaspirate
Clue: involvement of otherorganswith Location: predilection for RML + lowerlobes
. diarrhea(0-25%), myalgia,toxic encephalopathy
r/ homogeneoussegmentalairspaceconsolidation
. liver+ renaldisease (mostcommon)
. hyponatremia(20%) r/ interstitialreticulonodular pattern(rare)
. elevatedserumtransaminase/ transpeptidase = circumscribedperipheralmass
r/ paraffinorT't?
levels (granulomatous reaction+ fibrosisoftencausing
. lack of quick responseto penicillin/
stellateappearance)
cephalosporin / aminoglycoside ri slow progression/ no change
Concomitantinfection (in S-10%): CT:
Streptococcus pneumoniae, Chlamydiapneumoniae, r/ diffuseground-glass opacityof centri-/ panlobular
Mycobacterium tuberculosis, pneumocystis carinii distribution (= acinarpattern)+ thickeningof
Location: unilateralI bilateral(lessfrequent);lobar/ interlobularsepta as earliestfinding
segmental r/ airspaceconsolidation(fillingof alveoliwith exudate
! patchybronchopneumonia (= multifocalconsolidation) + inflammatory cells)at 1 week
r/ moderatevolumeof pleuraleffusion(6-30-69%) r/ returnto ground-glassopacity(due to expectoration
r/ cavitation(rare) + lymphaticdrainageof lipiddropletsand
Cx: progressiverespiratoryfailure(mostcommon inflammatory cells)at24 weeks
cause of death;6% mortalityin healthypatients) { volumeloss+ fibrosisof interlobular septaand
Rx: erythromycin pleuraat 14-16 weeks
^/ mass of low-attenuation approachingthat of
subcutaneous fat (-150 to 50 HU)
LIPOID PNEUMONIA Dx: bronchoalveolar lavage,transbronchialbiopsy
AcuteExogenousLipoidPneumonia
= FIRE-EATER PNEUMONIA
Material: liquidparaffin,petroleum (hydrocarbons) LOFFLER
SYNDRoME
Cause: accidentalpoisoningin children,fire-eaters = disorderof unknownetiologycharacterizedby local
r/ ilt-detinednodularareas of increasedradiopacity areas of transientparenchymalconsolidationassociated
r/ pneumatoceles / thin-walledcollections
of air with bloodeosinophilia
Path: interstitial+ alveolaredema containinga large
ChronicExogenousLipoidpneumonia numberof lymphocytes
Etiology: aspiration/ inhalationof fatlikematerial . no / mildsymptoms
Types of oils: . eosi nophi l i a
(a) vegetableoil: sesameoil used in medical . historyof atopia
suspensionsfor the treatmentof constipation r/ single/ multipleareasof homogeneous ill-defined
(b) animaloil: cod liveroil (commonlygivento consolidation
children);squalene= derivativeof shark liveroil r/ uni- or bilateral,nonsegmental distribution,
(folkremedyin some Asiancountries);milk , predominantly in lung periphery
(c) mineraloil (mostcommon):as liquidparaffinin r/ transient+ shiftingin nature(changeswithinone to
nose drops (takenat bedtime)/ oral laxatives severaldays)
= inert pure hydrocarbonthat does not initiate Prognosisj may undergospontaneousremission
cough reflex
Predisposed: elderly,debilitated,neuromuscular
disease,swallowingabnormalities LUNG TRANSPLANT
(eg,scleroderma) lndications:
Path: poolof oil emulsifiedby lung lipase+ surrounded emphysema,cysticfibrosis,CHD, idiopathicpulmonary
by giant cell foreignbody reaction(mineraloil fibrosis,alpha-1antitrypsindeficiency,primary
aspiration)/ necrotizinghemorrhagic pulmonaryhypertension, sarcoidosis,pneumoconiosis,
bronchopneumonia (highercontentof free fatty malignancy
acid in animalfat aspiration) Suruivalrate: 90% 1-monthsurvival,7O/" 1-yearsurvival
500 Radiology Review Manual

Acute Rejectionof Lung Transplant HyperacuteRejectionof Lung Transplant

lncidence; 60-80% with 2-3 significantepisodesin = rejectionin casesof an immunoglobulin
G donor-
first3 months specificHLA antibodypositivecrossmatch
Histo: mononuclearcell infiltratearoundarteries, Path: acute diffusealveolardamage
veins,bronchioles, alveolarseptawith alveolar
edema(initially) + fibrinousexudate(later)
Timeof onset: first episode5-10 days after Infection
Posttransplantation
transplantation; occasionallyby 48 mucociliary
reduced
Cause: immunosuppression,
hours clearance,interruption of lymphaticdrainage,
. drop in arterialoxygenpressureWITHOUTinfection/ directcontactof transplantwith environment
airwayobstruction/ fluid overload via airways
. pyrexia,fatigue,decreasedexercisetolerance A . IN FE C TION OF LU N GTR A N S P LA N T
r/ heterogeneous opacitiesin perihilarareas Prevalence: 35-50%; majorcause of morbidity
r/ ground-glassattenuationon HRCT + mortalityin early postoperative
{ new increasingpleuraleffusion+ septalthickening period
(mostcommon,90% specific,68% sensitive) Cause: ? absentcough reflex,impaired
WITHOUTconcomitant signsof LV dysfunction mucociliarytransportin denervatedlung
(increasein cardiacsize lvascularpediclewidth/ Organism; bacteria(23/") > CMV > Aspergillus
vascular redistribution) > Pneumocystis
r/ subpleuraledema,peribronchial cuffing,airspace (1) within1st month: Gram-negative bacteria,
disease fungi (candidiasis,
Dx: (1) Transbronchial biopsy aspergillosis)
(2) Rapidimprovement of radiologic (2) after1st month: CMV, Pneumocystis carinii,
abnormalities aftertreatmentwith lV bolusof bacteria,fungi
corticosteroidsfor 3 days ' fever,leukocytosis
Rx: methylprednisolone, polyclonalT-cellantibody ^/ lobar/ multilobarconsolidation (dueto bacterial>
(antithymocyte globulin),monoclonalantibodies fungalpathogens)
(CD3,OKT3),lymphoidirradiation ^/ diffuseheterogeneous/ ground-glassopacities
(dueto viral ldisseminatedfungalpathogens)
AnastomoticComplications
of LungTransplant { nodularopacities(dueto fungal/ unusualbacterial
1. Airwaydehiscence(2-8%) pathogens/ CMV / septicemboli)
r/ presenceof extraluminalair collectionsat Cx: may progressrapidlyto respiratoryfailure
anastomoticsite (80%) + death
2. Airwaystricture Dx: transbronchial/ open biopsy(80% accurate)
DDx: telescopedanastomosis B. EXTRAPULMONARY INFECTION
Rx: laser resection,balloonbronchoplasty thoracotomy woundinfection,bacteremia, sepsis,
3. Vascularstenosis empyema,centralvenouslineinfection
4. Diaphragmatic herniafrom omentopexy
Procedure: omentalpedicleis harvestedat time of
transplantationthrougha small
diaphragmaticincision+ wrapped n LymphoproliferativeDisease
Posttransplantatio
aroundanastomosisto prevent lncidence: 4%
dehiscence Histo: spectrumfrom benignpolyclonalproliferationof
lymphoidtissueto non-Hodgkin lymphoma
ChronicRejectionof LungTransplant Associatedwith: Epstein-Barrvirus
Prevalence: 24/o Timeof onset: 1 monthto severalyears;relatedto
Path: obliterativebronchiolitis(36%),interstitial Immunosuppressrve regrmen
pneumonitis, rejection-mediated
vasculopathy ri solitary/ multiplediscretenodules
Timeof onset: 3-75 monthsaftertransplantation { mediastinal / hilarlymphadenopathy
persistentcoughingand wheezing
: slowlyworseningexertionaldyspnea
^/ increased/ diminishedlungvolumes ReperfusionEdema
central+ peripheralbronchiectasis = REIMPLANTATION RESPONSE
{ localizedairspacedisease = infiltrateappearingwithin48 hoursafter
partiatlobar atelectasis transplantation unrelatedto fluidoverload,LV failure,
^/ thin irregularareasof increasedopacity or rejection;diagnosedby
infection,atelectasis,
pleuralthickening exclusion
diminishedperipherallung markings Pathogenesr's;permeabilityedemadue to lymphatic
nodular/ reticularopacitiesassociatedwith disruption,pulmonarydenervation,
peribronchial thickening organischemia,trauma
 ChestDisorders 501

Histo: fluid accumulationin interstitiumconsistentwith occasionally chylousascites

noncardiogenic pulmonaryedema mediastinal+ retroperitoneal adenopathy(fromsmooth
Time course: manifestswithin24 hours,peaksat muscleproliferation)
2nd-4th postoperativeday, resolvesat HRCT:
variablerate rangingfrom days to r/ numerousrandomlyscatteredthin-walled(<2 mm)
1-2 weeks to months cysts of varioussizes (0.5-6 cm) surroundedby
. increasinghypoxiabeforeextubation;poor correlation normallungparenchyma(? due to air trappingdistal
betweenradiographicseverity+ physiologic to small ainrays narrowedby smoothmuscle
parameters proliferation / destruction of collagen+ elastinin the
Location: perihilarareas+ basalregions interstitiumfrom metalloproteinases elaboratedby
{ perihilarhaze lrapid uni-or bilateralheterogeneously LAM cells)
dense interstitialand/orairspacedisease r/ bronchovascular bundlesat peripheryof cyst walls
Dx: per excfusion(radiographicchangesnot due to r/ consolidations(due to hemorrhagefollowing
LV failure,hyperacuterejection,fluid overload, destruction of pulmonarymicrovasculature)
infection,atelectasis) { pericardialeffusion
r/ ditateOthoracicduct
r/ precarinal+ retrocrurallymph nodes
LYMPHANGIOMYOMATOSIS NUC (V/Qscan):
= LAM= LYMPHANGIOLEIOMYOMATOSIS r/ "speckling"= well-definedhot spots on ventilation
= tara disordercharacterizedby scan (presumablydue to accumulationof coalescing
(1) graduallyprogressive diffuseinterstitial
lung dropletsof DTPA aerosoltrappedin peripheralcysts)
disease in 66%
(2) recurrentchylouspleuraleffusions @ Abdomen
(3) recurrentpneumothoraces . bloating,increasedabdominalgirth,abdominalpain
Etiology: unknown,? forme frusteof tuberoussclerosis . perinealswelling,chylousvaginaldischarge
Age: 17-50 years,exclusivelyin womenof childbearing r/ lymphadenopathy of up to 4 cm (33%):
age Histo: replacementof lymph node with smooth
Histo: proliferation of atypicalsmoothmusclein pulmonary muscle
lymphaticvessels,bloodvessels,and airways ^/ centralhypoattenuating areasof -72to +50 HU
Pathogenesis: (dueto chylouslymphcollections / fat)
proliferatedsmoothmuscleobstructs(a) bronchioles r/ lymphangioleiomyoma (20%) = well-defined
(trappingof air, overinflation,formationof cysts, lobulatedcomplexlymphaticmass:
pneumothorax), (b) venules(pulmonaryedema, r/ vol umesof 10-1,500mL
hemorrhage, hemosiderosis), (c) lymphatics(thickening r/ hypoattenuating centerof 3-25 HU
of lymphatics,chylothorax) Path: smoothmuscleproliferationin walls of
May be associatedwith: Tuberoussclerosis(lung lymphaticsresultingin lymphaticdilatation
involvement in 1%) + muralthickening
. progressiveexertionaldyspnea+ cough Location: retroperitoneum > pelvis> chest > neck
. diseaseaggravatedby pregnancy+ oral contraceptives ri low-density ascites(10%)of -10 to +21 HU (dueto
. hemoptysis(30-40%),chyloptysis ruptureof overdistendedlymphcysts)
. radiologic-physiologic discrepancy= severeairflow ri dilatationof thoracicduct (10%)
obstruction(reduced FEV1,reducedratioof FEV' / ./ tatty liver masses(5%): AML / lipoma
FVC%)despiterelativelynormalfindingson CXR @ Kidneys
. near normalFVC and TLC . flankpain,hematuria
. combinationof restrictive+ obstructiveventilatory ! angiomyolipoma (50%):
defects: hypoxia,markedlyimpairedDlcoyo r/ occasionallylackingfat
. positiveimmunohistochemical stainingotLAtvtcellswith { multiplicity in <20h
HMB-45(monoclonal antibodyfor melanocytic lesion) r/ simplecysts (occasionallylargeenoughto leadto
renal insufficiency)
r/ classicsigns:
./ coarsereticularinterstitialpattern(causedby Dx: open/ transbronchial
lungbiopsy
summationof multiplecystwalls)
ri recurrentlargechylothoiax(20-50-75%) Prognosis; 8.S-yearsurvival rate of 38-78/"; death
^/ recurrentpneumothorax(40-50%at presentation;in within10 yearsfrom progressive pulmonary
, 80% duringcourseof disease) insufficiency
r/ normal/ increasedlungvolume DDx:
0 The only interstitial
lungdiseaseto developincrease (1) Tuberoussclerosis(corticaltubers,subependymal
in lungv olume ! nodules,retinalhamartomas, facialangiofibromas,
r/ KerleyB lines periungualfibromas,mentalretardation, epilepsy,
r/ pulmonarycysts(visibleif >1 cm) + honeycombing multiplerenalAML in 40-80%)
502 Radiology Review Manual

(2) Histiocytosis(cystsin upper213of lung with sparing { pleuraleffusion(30-50%)

of costophrenicangles,cyst walls more variablein r/ hilar/ mediastinallymphadenopathy (30-50%)
thickness,pulmonarynodules+ cavitation, septal Prognosis; death within 1 Year
thickening) DDx: (1) Fibrosingalveolitis(peripheral predominance)
(3) Emphysema(imperceptible cystwalls,cystsmay be (2) Extrinsicallergicalveolitis(no polygonal
segmentally distributed,lobulararchitecture structures,Pleuralchangesrare)
preservedwith bronchovascular bundlein central (3) Sarcoidosis(nodulesof irregularoutlinemore
position,areasof lungdestruction withoutarcuate frequentin upperlobes,polygonalstructures
contour) uncommon)
(4) ldiopathicpulmonaryfibrosis= fibrosingalveolitis
(smallirregularthick-walled cysts+ predominantly LY MP H OID IN TE R S TITIA L P N E U MON IA
peripheralinterstitial
thickening) = LYMPHOCYTIC INTERSTITIAL PNEUMONITIS = LIP
(5) Bronchiectasis (bronchialwall thickening) = benignlymphoproliferative disordercharacterizedby
(6) Neurofibromatosis (cysticair spacespredominantly diffuseinterstitiallymphocyticinfiltration(probably
in apicallocation) immunologic disorder)with highlyvariablecourse
Histo: extensiveinfiltrationof bronchovascular
LYMPHANGITIC CARCINOMATOSIS bundles,interlobularsepta,and pleuraby
= INTERSTITIAL CARCINOMA polyclonalmaturesmalllymphocytes + plasma
= tumorcell accumulation withinconnectivetissue cells;manycasesreclassified as lymphoma
(bronchovascular bundles,interlobularsepta,subpleural Associatedwith: Sjogrensyndrome,autoimmunethyroid
space,pulmonarylymphatics) from tumorembolization disease,AIDS,Castlemandisease,
of bloodvesselsfollowedby lymphaticobstruction, systemiclupuserYthematosus,
interstitialedema,and collagendeposition(fibrosisfrom myastheniagravis,perniciousanemia,
desmoplasticreactionwhen tumor cells extendinto chronicactivehepatitis
adjacentpulmonaryparenchyma) 0 lndicativeof AIDSwhen presentin childunder13 years
lncidence: 7"/" of all pulmonarymetastases of age!
carcinoma,carcinomaof . dyspnea+ cough
Tumororigin: bronchogenic
. cyanosis+ clubbing(50%)
breast(56%),stomach(46%),thyroid,
pancreas,larynx,cervix . enlargementof salivaryglands (20%)
. NO lymphocytosisor historyof atopia
mnemonic; "CertainCancersSpreadBy PluggingThe
Lymphatics" . monoclonalgammopathy(usuallylgM)
Cervix Distribution: bilateral,involvingall lungzones
Colon ./ fine reticularchangesin both lungs
Stomach r/ reticulonodular pattern
Breast r/ resemblingairspacedisease(in severeform)
Pancreas C T:
Thyroid ./ itt-definedcentrilobular nodules(100%)
Larynx { ground-glass attenuation (100%)
Path: (1) interstitial edema,(2) interstitial
fibrotic ri thickeningof bronchovascular bundles+ interlobular
changes,(3) lymphaticdilatation,(4) tumorcells septa (in majoritY)
withinconnectivetissueplanes { subpleuralsmall nodules(in majority)
. dyspnea(oftenprecedingradiographicabnormalities) { airspaceconsolidation
. rarelydry cough + hemoptysis { cysts (due to partialairwayobstructionby
Location: bilateral;unilateralif secondaryto lung primary peribronchial + peribronchiolar LIP)in 68%
CXR (accuracy23%): r/ mediastinal lymphnodeenlargement(50%)
r/ normalchest radiograph Prognosis:
r/ reticulardensities (a) recovery/ slowlyimproving/ stabledisease
./ coarsenedbronchovascular markings (b) progressivedisease(in 33%)
^/ KerleyA+Blines Rx: responsiveto steroids
{ s m alllungv o l u me DDx: Hypersensitivity pneumonitis(ground-glass
./ nitar adenopathy(20-50%) attenuation, small centrilobularnodules,NOT cystic
HRCT : airspaces/ thickeningof interlobular septaor
r/ well-defined smoothlythickenedpolygonalreticular bronchovascu lar bundles)
networkof 10-25 mm in diameter(= thickened
interlobularsepta) fOrM= PSEUDOLYMPHOMA
LOCAI|ZEd
"beaded"thickeningof
r/ irregular/ nodular=
interlobularsepta
{ centraldot withinsecondarypulmonarylobule LYMPHOMA
= thickenedcentrilobular bronchovascular bundle 0 7th leadingcause of deathfrom cancerin UnitedStates
{ subpleuralthickening Pathogenesis: ? viral cause
 ChestDisorders 503

HD: contiguousspreadrequiresscanningof abnormal . malaise,weightloss (35%)

area only . no specificserum markers
NHL: noncontiguous spreadrequiresscanningof chest, 0 Lesscommonlyfoundin lymphnodes,bone marrow,
abdomen,pelvis spleen
@ Thorax 0 Involvement of upperrespiratory
tract+ sinusesis very
0 Hodgkindiseasemorecommonin thoraxthan NHL unusual
at presentation (HD in 85%, NHL in 4S%) @ L u n g( 1 0 0 % )
1. Lymphadenopathy . fever (60%),cough (56%),dyspnea(Zg%)
anteriormediastinal, pretracheal, hilar,subcarinal, ri normalCXR
axillary,periesophageal, paracardiac, superior r/ diffusereticulonodularopacities(= granulomas)
diaphragmatic internalmammarylymphnodes r/ largemasslikeopacities(= granulomas* pulmonary
- anteriormediastinum:nodularsclerosingtype infarcts)
of HD (7 5 /" );M < F r/ multiplebilateralnodulesin middle+ lowerlobes
- posteriormediastinum:NHL (80%)
2. Lungparenchymainvolvement(HD in 12/o,NHL in d unilateralinvolvement Q1./"\
4%) { smallpleuraleffusions'(40%)
3. Pleural+ subpleurallymphoma(up to 30%) r/ hilar lymphadenopathy (25%)
@ Abdomen C T:
1. Periaorta i cd e n o p a t h y . . . . . . . . . . . . . . H D i n 2 5 % ^/ peripheralsubpleural0.6-8 cm largenodules/
N H L i n 49% MASSES
2. M es ent e riacd e n o p a th ..........
y H D i n 4/" ri centralcavitation(30%)
N H Li n 5 1 % r/ reticular/ nodularairspaceopacities(10-49%)
3. Liverinvolvement HD in g% @ Skin (3e-53%)
N H Li n 1 4 % . nodules,ulcers,maculopapular rash (20-39%)
r/ hepatomegaly with involvement.... HD in <30% @ cNS (37-53%)
N H L i n 57% ' neurologiccomplaints(21%)
HD: commonlydiffuseinfiltrating process @ Kidneys(32-40%)
NHL: diffuseinfiltrating / discretetumornodules Cx: lymphoma(12-47%)
4 . S p l e n i ci n v o l v e m e n t . . . . . . . . . . . . . HDin37% Mortality: in 53-90% from sepsis,respiratoryfailure,
N H Li n 4 1 % pulmonaryembolism,massivehemoptysis,
HD: mostcommonsite of abdominalinvolvement C N S l esi ons
NHL: 3rd mostcommonsite of abdominal
involvement; may be initialmanifestation in LYMPHOPROLIFERATIVE DISEASE AFTER
lar g ec e l lN H L TRANSPLANTATION
0 Staginglaparotomynecessaryas 2lS of tumor = abnormalproliferation of lymphoidcellsin
nodule s< 1 c m i n s i z e immunocompromised organtransplantrecipientsin a
5. Gastrointestinal involvement spectrumrangingfrom mild lymphoidhyperplasia to
in 10% of patientswith abdominallymphoma mal i gnant l ymphoma
(uncommonin HD, commonin histiocytic NHL); lncidence: 2o/"of all allograftrecipients:
NHL accountsfor 80% of all gastriclymphomas
bone marrowtransplantation .......0.6%
6. Renalinvolvement
renalgrafttransplantation ...........1-S%
late manifestation, mostcommonlyin NHL cardi actranspl antati on.......
... 1.8-20%
7. Adrenalinvolvement
l i vertranspl antati on ....... . 2%
m or ec om mo ni n N H L
lungtransplantation 6.2-9.4%
8. Extranodalinvolvement Pathophysiologyof B-cell (in 86%) origin:
morefrequentwith histologically diffuseformsof NHL f . infectionof B lymphocytes with EBV (Epstein-Barr
virus)causesincreasedproliferation of B cells
(= polyclonalB-cellexpansion)
2. lossof protectiveimmunecontrolby T cellsallows
LYMPHOMATOID G RAN U LOMATOSIS for uncontrolledproliferationof EBV-infectedB
= afigiocentric+ angiodestructive lymphoproliferative lymphocytes(= oligoclonalB-cellexpansion)
+ granulomatousdisease 3. geneticmutationtransformssome B cellsinto
Age: 7-85 (meanof 48) years; M:F = 2:1 malignantcells
Path: multiplesharplymarginatedmassesadjacentto a 0 14% of posttransplantation LPD is of T-cellorigin
bronchuscausingobstructive pneumonitis Time of onset: 2-5 months(mean)afterbone marrow,
Histo: angiocentricinfiltrateof atypicallymphoidcells (of lung,heart-lungtransplantation;
B-celllineagecontainingEpstein-Barr virus)with 23-32 months(mean)after kidney,heart,
vascularinvasion+ destruction; necroticlung livertransplantation
parenchymain highergradelesions;giantcells 0 Undercyclosporine / OKT3within
absent(DDxto Wegenergranulomatosis) 1 month
504 RadiologyReviewManual

Uniquefeatures: (1) chronicrenaldisease,renaltransplant(5%)

(a) predilectionfor extranodalsites (2) collagenvasculardisease,vasculitis
(b) varied morphologicappearance (3) hemolyticanemia
(c) strongi probablycausalassociationwith EBV (4) asthma
infection (5) dermatitis
(d) frequentabsenceof immunophenotypic / genotypic (6) Crohndisease
evidenceof monoclonality (7) myastheniagravis
(e) poor responseto cytolyticchemotherapyI irradiation (b) endogenoussteroidelevation:
. illnessresemblinginfectiousmononucleosis (1) adrenaltumor
= pharyngitis, fever,lymphadenopathy, (2) pituitarytumor/ hyperplasia = Cushingdisease
hepatosplenomegaly (3) ectopicACTH-production (carcinomaof the lung)
@ any site,includingCNS, lymphnodes,thorax,Gl tract, (c) obesity
or allograft . moon facies
@ Chest . buffalohump
ri well-circumscribed nodules+ low-attenuation center . supraclavicular + episternalfat
r/ patcnyair spaceconsolidation Location: uppermediastinum (common),cardiophrenic
r/ mediastinal/ hilarlymphadenopathy angles+ paraspinalareas(lesscommon)
@ Liver { uppermediastinal widening
r/ focal hepaticmass in orthotopiclivertransplant { paraspinalwidening
ri periportaladenopathy ^/ increasein epicardialfat-pads
{ symmetricslightlylobulatedextrapleural deposits
MECONIUM
ASPIRATION
SYNDROME extendingfrom apex to 9th rib laterally
= rTroStcommoncause of neonatalrespiratorydistressin OTH E RFE A TU R E S :
fullterm / postmatureinfants(hyalinemembrane rl osteoporosis
diseasemostcommoncausein prematureinfants) r/ fractures
Etiology: fetal circulatoryaccidentsi placental { asepticnecrosis
insufficiency/ postmaturityresultin perinatal ^/ increasedrectosacraldistance
hypoxia+ fetaldistresswith meconium
defecatedin utero MESOTHELIOMA
Pathosenesis;seve,f^:tffJ;$?Jli:,:i:t#ist#',";,
Benign Mesothelioma
= LOCALIZED FIBROUS MESOTHELIOMA = LOCALIZED
producesmediumand smallairway TUMOR = SOLITARY
FIBROUS OFTHEPLEURA FIBROUS
obstruction + chemicalpneumonitis TUMOROF PLEURA = BENIGN LOCALIZED MESO-
lncidence: 10o/o of all deliverieshave meconium-stained THELIOMA = BENIGN PLEURAL FIBROMA = FIBROSING
amnioticfluid, 1"/"of all deliverieshave MESOTHELIOMA = PLEURAL FIBROMYXOMA
respiratorydistress lncidence: <5o/oof all pleuraltumors
. cyanosis(rare) 0 No recognizedassociationwith asbestosexposure!
. persistent fetal circulation syndrom€ = rt€orr?tol Age: 3rd-8th decade;mean age of 50-60 years;
pulmonaryhypertension(secondaryto thick-walled M : F= 1 : 1
pulmonaryarterioles) + R-to-LshuntthroughPDA and Path: usuallysolitarymassarisingfrom visceralpleura
foramenovale + severecyanosis in 80% + parietalpleurain 20'/"
Rx: extracorporealmembraneoxygenation (major Histo: tumororiginatesfrom submesothelial
indicationbesidesdiaphragmatic hernia fibroblasts, linedby layerof mesothelial cells
+ neonatalpneumonia) (a) relativelyacellularfibroustissue
^/ large infant (b) roundedspindle-shaped denselypacked
./ bilateraldiffusegrosslypatchyopacities(atelectasis cel l s
+ consolidation) (c) resemblinghemangiopericytoma of lung
{ hyperinflation with areasof emphysema(airtrapping) . asymptomatic in 50%
./ spontaneouspneumothorax + pneumomediastinum . cough,fever,dyspnea,chestpain (largermass)
(25-40%) requiringno therapy . digitalclubbing(rare)+ hypertrophic pulmonary
,
r/ small pleuraleffusions(10-20%) osteoarthropathyin 20-35Y"
r/ trtOair bronchograms . episodichypoglycemia(4%)
r/ rapidclearingusuallywithin48 hours { sharplycircumscribed sphericallovoid lobularmass
Cx: morbidityfrom anoxicbraindamageis high of 2-30 cm in diameterlocatednear lung periphery/
adjacentto pleuralsurface/ withinfissure
M EDIASTINAL LI POMATOSIS { sessilewith smoothtaperedmargin(common)/
= €XC€SS UnenCapsulatedfat deposition pedunculated with obtuseangletowardchestwall
Etiology: (rare,benignfeature)
(a) exogenoussteroids(averagedaily dose of >30 mg r/ tumor may changein shape + locationupon alteration
pr ednis on e ): of patient'sposition(if pedunculated)

r/ areasof hemorrhage/ necrosismay be present
(favorsmalignancy)
r/ ipsilateralpleuraleffusion(rare)containinghyaluronic
acid
CT:
r/ substantialcontrastenhancement
r/ heterogeneous enhancement due to myxoid
degeneration+ hemorrhage
MR:
r/ hypointense on TlWl + hyperintense on T2Wl
Cx: malignantdegeneration in 37%
DDx: metastaticdeposit
Rx: excisionis curative(recurrencerate lowerfor
pedunculated versusnodulartumor)
MalignantMesothelioma
= DIFFUSE MALIGNANT MESOTHELIOMA
= uficorT'trT'ton
fatal neoplasmof serosalliningof pleural
cavity,peritoneum,or both
0 Mostcommonprimaryneoplasmof pleura!
Prevalence : 7-13:1,000,000 persons/year;
2,000-3,000cases/yearin USA
Etiology: asbestosexposure(13-1 00%);zeolite
(nonasbestos mineralfiber); chronic
inflammation (TB,empyema); irradiation
Carcinogenic potential :
proportionalto aspectratio (= length-to-diameter)
fiber and durabilityin humantissue:
crocidolite> amosite> chrysotile> actinolite,
anthophyllite, tremolite
0 Occupationalexposureof asbestosfound in only
40-80% of all cases!
0 5-10Y. o'tasbestosworkerswill develop
mesothelioma in theirlifetime(riskfactorof 30
comparedwith generalpopulation)
0 No relationto duration/ degreeof exposureor
smokinghistory
Latencyperiod: 20-35-45 years (earlierthan
asbestosis;laterthan asbestos-related
lungcancer)
Peak age: 50-70 years (66%); M:F = 24-6:1
Path: multipletumormassesinvolvingpredominantly
the parietalpleura+ to a fesserdegreethe
visceralpleura;progression to thicksheetlike/
confluentmassesresultingin lungencasement
Histo: (a) epithelioid(60%),(b) sarcomatoid(15.h\,
(c) biphasic(25%);intracellularasbestos
fibers in 25./"
Associatedwith: peritonealmesothelioma;
hypertrophicosteoarthropathy (10%)
Staging (Boutin modification of Butchart staging)
lA confinedto ipsilateralparietal/ diaphragmatic
pleura
lB + visceralpleura,lung , pericardium
ll invasionof chestwalli mediastinum (esophagus,
heart,contralateralpleura)or metastasesto
thoraciclymphnodes
lll penetration of diaphragmwith peritoneal
involvementor metastasesto extrathoracic
lymphnodes
ChestDisorders 505
lV distanthematogenousmetastases
Stageat presentation: ll in 50%, lllin2S/",1 in 18%,
lV in 4'/.
. nonpleuritic (56%)/ pleuriticchestpain (6%)
. dyspnea(53%)
. fever + chills+ sweats(30%)
. weakness,fatigue,malaise(30%)
. cough (24%),weight loss (22./"),anorexia(10%)
. expectorationof asbestosbodies(= fusiform
segmentedrodlikestructures= iron-proteindeposition
on asbestosfibers[a subsetof ferruginousbodies])
Spread:
(a) contiguous:chestwall,mediastinum, contralateral
chest,pericardium, diaphragm,peritonealcavity;
lymphatics, blood,lung
(b) lymphatic:hilar+ mediastinal(40%),celiac(8%),
axillary+ supraclavicular (1"/"),cervicalnodes
(c) hematogenous:lung,liver,kidney,adrenalgland
ri extensiveirregularlobulatedbulkypleural-based
massestypically>5 cm / pleuralthickening(00%)
! exudative/ hemorrhagic unilateralpleural-etiusion
(30-60-80%) withoutmediastinalshift ("frozen
hemithorax"= fixationby pleuralrind of neoplastic
tissue);effusioncontainshyaluronicacid in 80-100%;
bilateraleffusions(in 10%)
r/ distinctpleuralmass withouteffusion(<25%)
of r/ associatedpleuralplaquesin 50% = HALLMARKof
, asbestosexposure
! pleuralcalcifications(20%)
r/ circumferential encasement= involvementof all
pleuralsurfaces(mediastinum, pericardium, fissures)
as late manifestation
{ extensioninto interlobarfissures(40--AO%\
{ superficialinvasionof underlyingiung (primarilyas
extensionintointerlobular septa)
r/ rib destructionin20% (in advanceddisease)
r/ ascites(peritoneuminvolvedin 35%)
CT:
r/ pleuralthickening(92%)
r/ thickeningof interlobarfissure(86%)
r/ pleuraleffusion(74%)
{ contractionof affectedhemithorax(42%):
r/ ipsilateralmediastinalshift
r/ narrowedintercostalspaces
r/ elevationof ipsilateral hemidiaphragm
{ calcifiedpleuralplaques(20%)
MR (bestmodalityto determineresectability):
r/ minimallyhyperintenserelativeto muscleon Tl Wl
r/ moderatelyhyperintenserelativeto muscleon T2Wl
Metastasesto:
ipsilateral lung (60%),hilar+ mediastinal nodes,
contralateral lung+ pleura(rare),extensionthrough
chestwall + diaphragm
Prognosis: 10/" of occupationallyexposedindividuals
die of mesothelioma (in 50% pleural+ in
50% peritonealmesothelioma); mean
survivaltime of 5-11 months
DDx: pleuralfibrosisfrom infection(TB,fungal,
actinomycosis),f ibrothorax, empyema, metastatic
adenocarcinoma (differentiation impossible)
506 Radiology Review Manual

Dx: video-assistedthoracoscopicsurgery + others: synovialsarcoma,giantcelltumorof bone,

(postprocedural radiationtherapyof all entry lung metastasesfollowingradiation/
portsfor tumor seedingof needletrack 121%l\ chemotherapy

CavitatingLung Metastases
TO LUNG
METASTASIS Frequency: 4Y" (comparedwith 9% in primary
Pulmonarymetastasesoccurin 30% of all malignancies; bronchogenic carcinoma)
mostlyhematogenous Hista: squamouscellcarcinoma(10%),
Age: >50 years (in 87%) adenocarcinoma (9.5%)
mnemonic.' "squamousCell MetastasesTend to
F RE Q UE NCY : Cavitate"
Origin of pulmonary mets Probability of pulmonary mets Squamouscell carcinoma,Sarcoma
Colon
1. Breast 22% Kidney in 75% Mel anoma
2. K idney 11% Osteosarcoma in 75% cell carcinoma
Transitional
3. Headand neck 10% in
Choriocarcinoma 75% Cervix,duringChemotheraPy
4. Colorectal 9% Thyroid in 65%
5. Uterus 6% Melanoma in 60%
6. Pancreas 5% Breast in 55% HemorrhagicLung Metastases
7. Ovary 5% Prostate in 40% CT: ./ ill-definednoduleswith fuzzymargin+ halosign
8. Prostate 4% Headand neck in 30% (= SUrfoundingground-glass opacity)
9. Stomach 4% Esophagus in 20% 1. A ngi ocarci noma
2. Choriocarcinoma
lncidence of pulmonary metasfases; 3. Renalcell carcinoma
mnemonic.' "CHEST" 4. Mel anoma
Choriocarcinoma 60% 5. Thyroidcarcinoma
Hypernephroma i Wilmstumor 30 I 20%
Ewingsarcoma 18% EndobronchialMetastasis
Sarcoma(rhabdomyo-/ osteosarcoma)21 I 15% Frequency: 1"/"
Testiculartumor 12 ^/ subsegmental/ segmentalatelectasisor atelectasisof
entireunilaterallung
r/ multiplenodules(in75%) of varyingsizes(mosttypical), { roundendobronchial lesionon CT
82% subpleural 1. Bronchogenic carcinoma
^/ fine micronodular pattern:highlyvasculartumor(renal 2. Lymphoma
cell, breast,thyroid,prostatecarcinoma,bone sarcoma, 3. Renalcell carcinoma
choriocarcinoma) 4. Breastcancer
r/ pneumothorax(2%): especiallyin childrenwith 5. Colorectalcarcinoma
sarcoma+ frequentlywith osteosarcoma(due to
bronchopleural fistulacausedby subpleuralmetastasis) Lung Metastases in Childhood
CT: mnemonic.' "ROWE"
r/ noncalcified multiple(>10)roundlesions>2.5cm Rhabdomyosarcoma
likelyto be metastatic Osteosarcoma
ri connectionto pulmonaryarterialbranches(75%) W i l mstumor
Ewingsarcoma
SolitaryMetastaticLung Nodule
a primary
represents
lungnodule
0 A solitary lung Metastaseswith AirsPacePattern
= lepidicgrowthalongintactalveolarwallssimilarto
tumor in 62h in patientswith known Hx of neoplasm
0 0.4-5-9'/" of all solitarynodulesare metastatic;most bronchioloalveolarcarcinomamimickingpneumonia
likelyorigin: coloncarcinoma(30-40%),melanoma, r/ airspacenodules
osteosarcoma,renalcell carcinoma,bladdercancer, r/ consolidationwith air bronchogram
testiculartumor,breastcarcinoma r/ focal/extensiveground-glassopacities
1.Adenocarcinoma of Gl tract (10%)
Calcifying Lung Metastases (<1%) 2. Adenocarcinomaof breastI ovary
mnemonic.' "BOTTOM"
Breast
Osteo-/ chondrosarcoma SterilizedMetastasis
Thyroid(papillary) = persistenceof metastaticnodulewithoutsignificant
Testicular changein size afteradequatechemotherapy
Ovarian Histo: necroticnodule+ fibrosiswithoutviabletumor
Mucinousadenocarcinoma(colon) cells
 ChestDisorders 507

1. Choriocarcinoma Rx: erythromycin, azithromycin, tetracycline

2. Testicularcancer Cx: ? as an autoimmuneresponse
r/ growingteratomasyndrome= conV€rsionto a (1) Acutedisseminated encephalomyelitis
benignmatureteratoma (2) Cerebralarteriovenous occlusion
r/ pulmonarylacunae(= transformation intothin- (3) Erythemanodosum,erythemamultiforme
walledcavity)may persistfor years Stevens-Johnson syndrome
(4) pulmonary:Swyer-James syndrome,pulmonary
Metastasisof BenignTumorto Lung fibrosis,bronchiolitisobliterans,ARDS
1. Leiomyoma of uterus Prognosis: 20o/owith recurrentsymptomsof pharyngitis
2. Hydatidiformmole of uterus + bronchitist infiltrations
3. Giantcelltumorof bone DDx: viral infectionof lower respiratorytract,pertussis,
4. Chondroblastoma chlamydia(indistinguishable)
5. Pleomorphic adenomaof salivarygland
6. Meningioma NEARDROWNING
= asphyxiationdue to water inhalationfollowedby survival
METASTASIS
TO PLEURA for a minimumof 24 hours
1. Lung( 36% ) Stage 1:
2. Breast (25%) (a) acute laryngospasmafter inhalationof a small
3. Ly m phom a(1 0 % ) amountof water
4. Ovary (5%) ./ no roentgenographic abnormality
5. Stomach(2%) (b) prolongedlaryngospasm= "dry drowning"due to
negativepressureedemaarisingfrom a prolonged
MYCOPLASMA PNEUMONIA episodeof the Mullermaneuveras in postobstructive
= PR|MARY ATyptCAL pNEUMONtA (pAp) pulmonaryedema
0 Variedradiographic+ clinicalpicturel { Kerleylines,peribronchialcuffing
Commonestcauseof community-acquired nonbacterial ! patchyperihilaralveolarairspaceconsolidation
pneumoniawith a mildcourse(only2/" require Prognosis; resolutionwithin 24-48 hours (under
hospitalization),usuallylasts2-3 weeks;only 10% of therapy)
infectedsubjectsdeveloppneumonia Stage 2:
lncidence: 10-33% of all pneumonias;autumnpeak = laryngospasm+ swallowingof water into the stomach
Organism: Eatonagent = pleuropneumonia-like organism Stage 3:
(PPLO)= 350 Pm longPleomorPhic (a) persistentlaryngospasm withdry drowning(10-15%)
Mycoplasmapneumoniaewith lack of cell wall r/ pressureedema
Spread: direct contact/ aerosol (b) aspirationof water after hypoxia-induced relaxation
Age: most commonin ages 5-20 years (esp. in closed of laryngospasm(85-90%)
populations r/ permeabilityedema (due to hypoxia+ diffuse
Histo: peribronchial mononuclear (similarto
cell infiltrates alveolardamage)
viral lower respiratoryinfection) Cx: ARDS,aspirationof gastricfluid,infectionby
. incubationperiod: 1-2 weeks fresh-watersaprophyticbacteria
. gradualonsetbeginningwith pharyngitis, headache, 1. Sea-waterdrowning
myalgia(rhinorrhea + nasalcongestionuncommon) . hemoconcentration, hypovolemia
. mildsymptomsof dry cough+ low fever,malaise,otitis 2. Fresh-water drowning
. sputumwith PMNs but few bacteria . hemodilution, hypervolemia
. mild feukocytosis(20%) . hemolysis
. mostcommonrespiratory causeof cold agglutinin 3. Secondarydrowning
production(60%) (a) pneumoniadue to toxicdebris
0 Severityof radiologicfindingsdiscrepantto mild clinical (b) progressivepulmonaryedema
condition! 4. Dry drowningQ0a0%)
0 Pulmonaryinfiltrates show a significantlag time = laryngealspasm preventswater from entering
./ focal reticularinterstitialinfiltrate: r/ no roentgenographic abnormality
r/ unilobarfrom hiluminto lowerlobe as earliestchange Similarities of all 4 types:
(52%),bilobar(10%) . hypoxemia
{ parahilarperibronchialopacification(12%) . metabolicacidosis
! atelectasis(29%) ^/ centralextensivefluffyareasof increasedopacity
! alveolarinfiltrates: (alveolaredema indistinguishable from othertypes):
r/ patcnyinhomogeneousunilateral(L > R) airspace { tendencyfor opacitiesto coalesce
consolidationin segmentallowerlobe in S0o/", r/ hyalinemembraneformation= considefablelossof
bilateralin 1040"/" proteinfrom blood
r/ small pleuraleffusionsin 20./" Cx: pneumonia(due to aspiratedbacteria/ fungi/
r/ hilaradenopathy(7-22h) mycobacteria)
508 Radiology Review Manual

SARCOIDGRANULOMATOSIS
NECROTIZING r/ bilateralfocal/ diffuseareas of opacities(may initially
Etiology: ? variant of sarcoidosis appearsimilarto fetal aspirationsyndrome)
Age: 3rd -7th decade(meanage, 49 years); M:F = 1:2.2 r/ hyperaeration
Path: pleural+ subpleural+ peribronchovascular r/ may cause lobaratelectasis
scatterednodules/ conglomeratemasses+ central r/ may causepneumothoraxlpneumomediastinum
cavitation { pleuraleffusion(exceedingly rare)
Histo:confluentnoncaseatinggranulomas,extensive
necrosis,vasculitisof muscularpulmonaryarteries
+ veinswith frequentlytotalvascularocclusion, NOCARDIOSIS
bronchiolarobstruction, bronchiolitisobliterans, Organism; Gram-positiveacid-fastbacterium
obstructivepneumonitis resemblingfungus
. asymptomatic(15-40%) Predisposed: immunocomPromised
. cough,chestpain,dyspnea,fever,weightloss,fatigue nodules+ cavitation
{ muftiplepoorlyi well-defined
. uveitis,hypothalamic insufficiency(13%) r/ lobarconsolidation
0 Almostexclusivelyaffectslungs rl empyemawithoutsinustracts
r/ multiplebilateralsubpleural+ peribronchovascular r/ SVC obstruction(rare)
, pulmonarynodules
./ numerousill-defined parenchymal opacities
r/ + cavitation N ON .H OD GK IN LY MP H OMA
./ fritarlymphadenopathy (8-79/.) = NHL = diseaseof B cells
{ pleuralthickening lncidence: 3'h of all newlydiagnosedcancers;3rd most
0 No upperairwaydisease/ glomerulonephritis / systemic commoncancerin childhood(behind
vasculitis leukemia+ CNS neoplasms); 4 timesmore
Rx: corticosteroid therapyalone commonthan Hodgkindisease
DDx: sarcoidosis(highprevalenceof mediastinal+ hilar Predisposed:(40-100 times greaterrisk)congenital
lymphadenopathy, littlepropensityfor cavitation) immunodeficiency syndromes,organ
transPlant Patientsundergoing
immunosuppression, patientswith HIV
infection,collagenvasculardiseases
NEONATAL PNEUMONIA Age: all ages;medianage of 55 years;M:F = 1.4:1
Pathogenesis: . chest/ shoulderpain,dyspnea,dysphagia
(a) in utero infection(ascendingfrom prematurerupture . CHF, hypotension, SVC syndrome
of membranesor prolongedlaborI transplacental Modified Rappaport Cl assification :
route)= major risk factor = categorizationaccordingto histologicdistributionof
(b) aspirationof infectedvaginalsecretionsduring lymphomatous cells
delivery A. Nodularform = organizedin clusters
(c) infectionafter birth 1. Poorlydifferentiated lymphocytic (PDL)
Organism: 2. Mixed lymphocytic/ histiocytic(mixedcell)
(1) GroupB streptococcus (GBS)= IT'lost
common 3. Largecell (histiocYtic)
cause: in low-birth-weight prematureinfants;50% B. Diffuseform = distortionof tissuearchitecture
mortality 1. Well-differentiatedlymphocytic(WDL)
{ pulmonaryopacities(87%): 2. Intermediate-differentiated lymphocytic (lDL)
^/ appearanceidenticalto RDS (in 52%) 3. Poorlydifferentiatedlymphocytic(PDL)
! appearancesuggestingretainedlungfluid/ 4. Mixedlymphocytic largecell
/ histiocytic
focal infiltrates(35%) (DLCL);undifferentiated
(histiocytic) Burkitt
rl normalCXR (13%) lymphoma;undifferentiated non-Burkitt
r/ cardiomegaly (common) lymphoma(pleiomorphic); lymphoblastic (LBL);
r/ pleuraleffusions(in 213,but RAREin RDS) unclassified
Associatedwith: delayed onset of diaphragmatic Luke and Collins Classification:
hernia(evidencedby clinical = categorizationby morphologiccharacteristics of cell
deterioration) + cellof origin(T cell,B cell,non-B,non-Tcell)
Prognosis; often lethal Working Formulation Classification(Kiel / Lennert):
(2) Pneumococci:RDS-like = categorizationby grade
(3) Listeria:RDS-like A. Low grade
(4) Candida: progressiveconsolidation+ cavitation 1. Smalllymphocytic (3.6%)
(5) Chlamydiatrachomatis:bronchopneumonic pattern medianage 61 years,59% S-yearsurvival
(6) others: H. influenzae, Staphylococcus aureus, smallcleavedcell(22.5h)
2. Follicular,
E. coli,CMV, pneumocystis medianage 54 years,70"/"S-yearsurvival
. afebrile mixed(7.7%)
3. Follicular,
. lowerventilatorypressurerequirements median age 56 years, 50/" S-yearsurvival
 ChestDisorders 509

B. Intermediate grade Origin: B or T cell (in g0%) locatedoutsidemarrow;

1. Follicular, largecell (3.8%) (rarely)non-Band non-Tcells locatedwithin
medianage 55 years, 457.S-yearsurvival bone marrow
2. Diffuse,smallcleavedcell (6.9%) Age: medianage of 10 years;<15 yearsof age (most
medianage 58 years,33% S-yearsurvival common);unusual<5 yearsof age; M > F
3. Diffuse,mixed(6.7%) . chest pain, back pain,cough,dyspnea
medianage 58 years,38% S-yearsurvival . fever,anorexia,weight loss
4. Diffuse,largecell (19.7%) . + peripheralblood + bone marrowinvolvement
median age 57 years,3S/" S-yearsurvival (particularlyin lymphoblasticNHL):
C. Highgrade with lymphoblasticbone marrowinvolvementof <25/"
1. Largecell,immunoblastic (7.9%) patientis classifiedas havinglymphoma
medianage 51 years,32o/"S-yearsurvival
2. Lymphoblastic (4.2%) Staging (St. Jude):
medianage 17 years,26/" S-yearsurvivaf I singleextranodaltumor/ singleanatomicarea
3. Smallnoncleavedcell (5%) ll (a) singleextranodaltumor+ regionalnodes
medianage 30 years,2S/" S-yearsurvival (b) >2 nodalareason same sideof diaphragm
D. Miscellaneous (12%) (c) 2 singleextranodaltumors + nodeson same
composite,mycosisfungoides,histiocytic, side of diaphragm
extramedullaryplasmacytoma (d) primarygastrointestinal
tracttumor * nodes
Staging: same Ann Arbor systemas for Hodgkindisease lll (a) 2 singleextranodaltumorson oppositesidesof
Extranodalinvolvement: diaphragm
@ Cl tract: (b) >2 nodalareason both sidesof the diaphragm
stomach(3/"), smallbowel(S%),largebowel(2%), (c) primaryintrathoracictumors(mediastinum,
pancreas(0.7"/"),peritonealnodules+ ascites pleura,thymus)
(1.4%) (d) extensiveprimaryintraabdominal disease
@ Chest (40-50%): (e) paraspinal/ epiduraltumor
lung(6%),pleuralfluid(3.3%),pericardial fluid lV any of the above+ initialCNS / bone marrow
(0.7%),heart(0.2%) involvement
r/ trilar+ mediastinal adenopathy(DDx:sarcoidosis;
anteriornodesfavorlymphoma)
0 Nodesfrequentlynot involved!
r/ isolatedlymphnodesmay enhance(DDx: Differencesbetween Adult and Childhood NH
Castlemandisease)
./ tungnodules+ air bronchograms Characteristics Adult NHL Chitdhood
r/ pleuraleffusion Primarysite nodal extranodal
Prognosis.' unfavorable
Histology 50% follicular, diffuse
@ G U t r ac t( 1 0 % ):
kidneys(6/"), testes(1.2/"),ovaries(1.8%),uterus 50% diffuse
(1.2%) Grade low, intermediate, high
@ Bone (3.8%) high
@ cNS (2.4%) Histologicsubtype many three
@ B r eas t( 1. 2 % )
Sex predilection none 7O/" male
@ Skin (6.4%)
@ Headand neck (1.7%)
@ Liver(14%)
@ Spleen(41%) Prognosis: 80/" cure rate with multiple-agent
Nodalinvolvement:
chemotherapy
@ Paraaorticlymph nodes (49%) DDx: (1) Acutelymphocytic leukemia(>ZS/"
@ Mesentericlymphnodes(51%): lymphoblastswithinbone marrow)
predominantly in middlemediastinum, cardiophrenic (2) Hodgkindisease(contiguous spread,nodes
angle
are site of origin)
0 Singlelymphnode involvement is oftenthe only
manifestationof intrathoracicdisease! 1. Undifferentiated/ small noncleavedNHL (99%);
@ Splenichilarlymphnodes(59%) Path: non-Burkittlymphoma;Burkittlymphoma
0 Lymphography89% sensitive+ 86% specific . abdominalmass + ascites
. painsimilarto appendicitis
/ intussusception
N on- Hodgk in L y m p h o m a i n C h i l d h o o d Primarysite: abdomen(distalileum,cecum,
lncidence; 3rd most commonchildhoodmalignancy appendix);ovaries
(afterleukemia+ CNS tumors);7"h of all Commonsite: mesenteric, inguinal,iliacnodes;
malignancies in children<15 yearsof age CNS; bone marrow;kidney
510 RadiologyReview Manual

paraspinal
Raresite: orbit,supradiaphragmatic . cough(60-100%),hemoptysis(15-20%)
region,mediastinum,paranasalsinuses, . asthma,dysPnea
bone,testes,pulmonaryparenchYma . feverdistinctlyuncommon(10-13%)
Cx: "leukemictransformation"(= extensivebone . weakness+ weight loss (uPto 50%)
marrowinvolvement) . weekly positivetuberculinskin test
A. CLASSICALFORM
2. Lymphoblastic (T-cell) NHL (28%) Age: 6th-7th decade,in Whites (80-90%)' M > F
Primarysite: mediastinum (66%) Predisposing factors:
Commonsite: neck,thymus,liver,spleen,CNS, COPD (25-72%),previousTB (20-24%),interstitial
bone marrow,gonads lung disease(6%),smoking>30 pack-years(46%),
Raresite: subdiaphragmatic (ileum,cecum, alcoholabuse(4O/"),cardiovascular disease(36%)'
kidney,mesentery,retroperitoneum), chronicliverdisease(32%),previousgastrectomy
orbit,paranasalsinus,thyroid,parotid (18%)
. respiratorydistress,dYsPhagia Location: apical+ anteriorsegmentsof upper lobes
. SVC syndrome,pericardial tamponade r/ chronicfibronodular/ fibroproductive apicalopacities
f rom reactivationTB)
(indistinguishable
3. Large cell (histiocytic) NHL (26%) r/ cavitationin 80-95%
Origin: B cell,T cells (smallpercentage) { apicalpleuralthickeningin 37-56%
Location: nodal+ extranodal { additionalpatchynodularalveolaropacities(due to
Primarysite: variable(Waldeyerring, Peyer bronchogenicspread)in ipsi-/ contralaterallung in
patches) 40-70%
Commonsite: peripherallymphnodes,lung,bone, { adenopathy(0-4%)
brain,skin { pleuraleffusion(5-20%)
Rare site: hard palate,esophagus,trachea { typicallyNO hilarelevation
B. NONCLASSICALFORM (20-30%)
NONTUBERCULOUSMYCOBACTERIAL Age: 7th-8thdecade,86/"in Whites; p';'f = 1:4
Predisposingfactors; NONE
OF LUNG
INFECTION
Location: predominantly in middlelobe + lingula
= ATYPICALTUBERCULOSIS
Organisms: { mufiiplebilateralnodularopacitiesthroughoutboth
M. kansasii: lung infectionin subjectswith good lungsin randomdistribution
./ irregularcurvilinearinterstitialopacities(resembling
immunestatus
"swimmingpool granuloma" bronchiectasis)
M. marinum:
M. ulcerans: "Buruliulcer"in tropicalareas C. ASYMPTOMATICGRANULOMAS
in infants r/ clusterof similar-sized nodules
M. scrofulaceumtcervicallymphadenitis
D. ACHALASIA-RELATED INFECTION
M. aviumintracellulare: esp. in AIDS
with M. fortuitum-chelonei
Organism causing pulmonary disease (Runyon
classification): E . D IS S E MIN A TED IS E A S E
in immunocompromised patients:AIDS,transplant
ubiquitousorganismsas part of normalenvironmental
patients,lymphoproliferative disorders(esp. hairycell
flora
leukemia),steroid+ immunosuppressive therapy
1. Photochromogens
, . s i m i a e ,M .a s i a ti c u m
M . k ans asi iM CT:
. coloniesturn yellowwith exposureto light r/ multifocalbronchiectasis(79-94o/"),esp. middlelobe
0 70-80% of individualsfrom ruralareastest + l i ngul a
positiveon PPD-B(= antigenfrom M. kansasii)! r/ centrilobular nodulesof varyingsizes,usually<1 cm
2. Scotochromogens (= micronodules)in 76'97'/"
M. scrofulaceum, M. xenopi,M. szulgai,M. gordonae { bronchialwall thickening(97"/o)
. yellowcoloniesturn orangewith exposureto light ./ airspacedisease(76%)
3. Nonchromogens { cavitation(21'/"),esp. in upperlobes
M. avium-intracellulare, M. malmoense,M. terrae { interlobular septalthickening(12%)
. white/ beigecolonieswithoutcolorchange 0 Unfavorableresponseto antituberculous therapyis
4. Rapidgrowers suspiciousfor atyPicalTB!
M. fortuitum-chelonei DDx: M. tuberculosis (bronchiectasis lesscommon+ less
. appearin culturein 3-5 days (all othergroups extensive),bronchiolitisobliterans,sarcoidosis,
appearin culturein 2-4 weeks) fungaldisease
Histo: lesionsindistinguishable from M. tuberculosis
Source: soil,water,dairy products,bird droppings
lnfection: inhalationof aerosolizedwater droplets(M. PANBRONCHIOLITIS
avium-intracellulare complex),food aspiration = inflammatorylung disease,prevalentin Orientalsbut
in patientswith achalasia(M. fortuitum- rare in Europeans+ NorthAmericans
chelonei),Gl tract (in AIDS) Pathogenest's;unknown
 ChestDisorders 5 1 1

HRCT : PERICARDIAL
CYST
r/ centrilobularbranchingstructures(segmentsof Etiology:
bronchiolectasisfilledwith secretions)
+ nodules (1) defectin embryogenesis of coelomiccavities
surroundingrespiratory bronchioles (2) sequelaeof pericarditis
r/ mosaicperfusion
Histo: linedby singlelayerof mesothelialcells
r/ air trapping Age: 30-40 years; M:F = 3:2
r/ bronchialdilatation . asymptomatic(50%)
DDx: bronchiolitisobliterans Location:(a) cardiophrenic angle(75%),R:L = 3:1 l3;2,
25% higher;may extendinto majorfissure
PA RA G O NI M I A S IS OF L U N G (b) mediastinum (rare)
= parasiticdiseasecausedby trematodeparagonimus { sharplymarginatedround/ ovoidi triangularmass
(usuallyP. westermani= lungfluke)endemicto certain usually3-8 cm (range1-28 cm) in diameter
areasof East+ SoutheastAsia (China,Korea,Japan, r/ changein size + shape with respiration/body position
Thailand,Laos,Philippines, India) r/ attenuation valuesof 20-40 HU, occasionallyhigher
lnfection: ingestionof raw / incompletelycooked
freshwatercrab/crayfish infectedwith PNEUMATOCELE
metacercaria; larvaexistsin smallintestine = cvsticair collectionwithinlungparenchymadue to
+ penetratesthe intestinalwall + enters obstructiveoverinflation
peritonealcavity;larvapenetratesdiaphragm = regionalobstructiveemphysema
+ pleurato enterthe lung 0 Doesnot indicatedestructionof lungparenchyma
Cycle: from the final host (tiger,cat, dog, fox, weasel, 0 Occursduringhealingphase
opossum,human)eggs of worm pass to the 0 Appearsto enlargewhile patientimproves
outsidewith blood-streaked sputum;in freshwater 0 Frequentlymultiple
ciliatedembryos(miracidia) develop;they become Develop mental theories :
tailed larvae(cercariae)after invadinga fresh- (1) smallbronchioles undergoseveredistension
watersnail;when the infectedsnailis eatenby a secondaryto check-valveendobronchial/
crustacean, theirtailsdetachand they become peribronchial obstruction
300 pm encystedlarvae(metacercariae) (2) focus of necroticlung evacuatesthrougha bronchus
@ CNS narrowedby edemai inflammation;
. meningoencephalitis airspace
(in 25%) subsequently enlargesdue to check-valve
{ shell-like/ soap-bubble-like calcifications
of varying mechanismfrom enlargingpneumatocele /
size (-50%) inflammatoryexudate
CXR (pulmonarylesionsin 837o,pulmonary+ pleural (3) air from rupturedalveoli/ bronchiolesdissectsalong
lesionsin 44/", pleurallesionsin 17"/.): interstitialinterlobular tissueand accumulates
earlyfindings(lesionsoccur3-8 weeksafteringestion): betweenvisceralpleuraand lungparenchyma
{ uni-/ bilateralpneumo-/ hydropneumothoiax F7;/") = subpleuralemphysematousbulla
r/ uni- / bilateralpleuraleffuslon p-54%) = subpleuralair cyst
r/ focal patchymigratingairspaceconsolidation A . P N E U MA TOC E LE A S S OC IA TE DW ITH IN FECTI O N
(= worm migrationcausingfocalhemorrhagic Organism.' pneumococci,E. coli, Klebsiella,
pneum on i Q@ S % )
Staphylococcus (in childhood)
r/ lobar/ segmentalcollapse(airwayobstructionfrom ./ appearswithin1stweek,disappearswithin6 weeks
, egg granuloma/ intrusionof worm) .i thin-walled+ completelyair-filledcavity
r/ e-+-mm thick and 2-7-cm long linearopacities ^/ + air-fluidlevel+ wall thickening(duringinfection)
abuttingthe pleura(41%)due to worm migration r/ pneumothorax
track r/ spontaneousresotution(in most)
latefindings: B. TRAUMATICPNEUMATOCELE = PNEUMATOCYST
^/ tungcyst (cystformationfrom infarctionafter
Cause:
arteriolar/ venous obstructionby worm or egg; (a) air trappedwithinarea of pulmonarylacerationis
expansionof smallainvayby intraluminal parasite): initiallyobscuredby surrounding contusion
r/ thick-wailedcyst (due io fibrosis) (hematoma); pneumatocyst appearswithinhours
r/ "eclipseeffect"= eccefltficthickeningof cyst wall afterbluntchesttrauma
, (dueto intracysticone / two worms) (b) intensiveinflammatory responsefrom
r/ thin-walledcyst (whencyst connectedto airway)
hydrocarbon (furniturepolish,kerosene)
^/ tO-t5-mm nodules* masilikeconsolidation (z+;/.) inhalation/ ingestion
(due to cyst initiallymaskedby pericysticairspace r/ single/ multiplepneumatoceles
consolidation + cystfilledwith chocolate-colored r/ spontaneousresolutionover severalweeksto months
necroticfluid) C . " P U LV E R IZE D LU N G' '
r/ bronchiectasis(35%) Cause: severechesttrauma
DDx: tuberculosis (nodularslowlychanginglesion,residual r/ multiple5-1O-mmair cysts in an area of airspace
fibrosisaftertreatment, no subpleurallinearopacities) opacification
512 RadiologyReview Manual

PNEUMONIA
PNEUMOCOCCAL Predisposed:
MostcommonGram-positive pneumonia (1) debilitatedprematureinfants,childrenwith
90% community-acquired, 107o nosocomial hypogammagIobuIinemia (12%)
lncidence: 15/" ol all adulthoodpneumonias, (2) ArDS (60-80%)
(3) otherimmunocompromised patients:congenital
uncommonin child;peaksin winter+ early
spring;increasedduringinfluenzaepidemics immunodeficiency syndrome,lymphoproliferative
Organism; Streptococcuspneumoniae(formerly disorders,organtransplantrecipients(renal
Diplococcus pneumoniae), Gram-positive, in transplantpatientsin 10%),patientson longterm
pairs/ chains,encapsulated, capsular corticosteroid therapy(nephroticsyndrome,collagen
polysaccharide responsible for virulence vasculardisease),patientson cytotoxicdrugs[under
+ serotYPing therapyfor leukemia(40%\, lymphoma(16%)l
Susceptible;elderly,debilitated,alcoholics,CHF, COPD, 0 Often associatedwith simultaneousinfectionby CMV,
multiplemyeloma,hypogammaglobulinemia, Mycobacteriu m avium-intracel lulare,herpessimplex
. severe dyspnea + cyanosisover 3-5 days
functionalI surgicalasPlenia
. rustyblood-streakedsPutum . subacuteinsidiousonsetof malaise+ minimalcough
. left-shiftleukocytosis (frequentin AIDS patients)
. impairedpulmonaryfunction . respiratoryfailure(5-30%)
. WBC slightlyelevated(PMNs)
Location: usuallyinvolvesone lobeonly;biasfor lower
. lymphopenia (50%)heraldspoor prognosis
lobes + posteriorsegmentsof upper lobes
(bacteriaflow undergravitationalinfluenceto r/ normalCXR 10-39% in
most dependentportionsas in aspiration) ./ bilateraldiffusesymmetricfinelygranular/ reticular
{ extensiveairspaceconsolidationabuttingagainst interstitial/ airspaceinfiltrates(in 80%) with perihilar
+ basilardistribution (CHARACTERISTIC central
visceralpleura(lobari beyondconfinesof one lobe
throughporesof Kohn)CHARACTERISTIC location)
r/ slightexpansionof involvedlobes { responseto therapywithin5-7 days
{ prominentair bronchograms (20%) ri rapidprogressionto diffusealveolarhomogeneous
pattern(in some) consolidation (DDx:pulmonaryedema)
{ patchybronchopneumonic
r/ pleuraleffusion(parapneumonic transudate)uncommon \i air bronchogram
with antibiotictherapy fine / coarselinear/ reticularpattern= thickenedcoarse
r/ cavitation(rare,with type lll) interstitial lung markings(in healingphase)
disease, pleuraleffusion+ hilarlymphadenopathy (uncommon)
Variations(modifiedby bronchopulmonary
eg, chronicbronchitis, emPhysema) : ./ atypicalpattern(in 5%):
r/ bronchopneumonia-like pattern r/ isolatedlobardiseaseI focalparenchymalopacities
r/ effusionmay be only presentation(esp. in COPD) { tungnodules+ cavitation
{ empyema(withPersistentfever) ./ nilar/ mediastinal lymphadenopathy
- in children: r/ tnin- / thick-watledregular/ irregularcysts/ cavities
r/ roundpneumonia= sharplydefinedroundlesion with predilection for upperlobes+ subpleuralregions
Prognosisj promptresponseto antibiotics(if without { effectof prophylacticuse of aerosolizedpentamidine:
complications); 57omortalityrate
rl redistribution of infectionto upperlobes
Dx: blood culture(Positivein 30%) { cysticlungdisease
septicarthritis,empyema { spontaneouspneumothorax, frequentlybilateral(6-7%)
Cx: meningitis, endocarditis,
(now rarelyseen) ^/ disseminated extrapulmonary disease(1%)
{ punctate/ rimlikecalcifications in enlargedlymph
nodes+ abdominalviscera
PNEUMOCYSTOSIS CT:
= PNEUMOCYSTIS r/ patchworkpattern (56%)
CARINII PNEUMONIA
pneumoniain = bilateralasymmetricpatchymosaicappearance
0 Most commoncauseof interstitial
with sparingof segments/ subsegments of
immunocompromised patients,whichquicklyleadsto
pulmonarylobe
airspacedisease
Organism: { ground-glassPattern(26"/")
= bilateraldiffuseairspacedisease(fluid
ubiquitousobligateextracellularprotozoan/ fungus
+ inflammatory cellsin alveolarspace)in symmetric
Pneumocystis carinii
(a) trophozoitedevelopsinto a cyst distribution
(b) cyst producesup to eight daughtersporozoites, r/ interstitialpattern(18%)
= bilateralsymmetric/ asymmetric,linear/ reticular
which are releasedat maturity+ develop into
markings(thickening of lobularsepta)
trophozoites
Pathomechanism: { air-filledspaces(38%):
(a) pneumatoceles= thin-walledspaceswithoutlobar
trophozoiteattachesto cell membraneof type I alveolar
predilection resolvingwithin6 months
pneumocyteswith subsequentcell death + leakageof
proteinaceousfluid into alveolarspace (b) subpleuralbullae(dueto prematureemphysema)
 ChestDisorders 513

(c) thin-walledcysts (? check-valveobstructionof Location: almostexclusivelyrestrictedto posterior

small airwaysfrom aerosolizedpentamidine) segmentof upperlobe/ superiorsegmentof
, (d) necrosisof PCP granuloma lower lobe
r/ pneumothorax (13%) ^/ large>1 cm opacitiesinitiallyin middle+ upperlung
{ lymphadenopathy (19%) zones at peripheryof lung
r/ pleuraleffusion(18%) r/ discoidcontour(44%)= rTlossflat from frontto back
. { pulmonarynodules (thinopacityon lateralview, largeopacityon pA view),
usuallydue to malignancy(leukemia,lymphoma, medialborderoften ill-defined,lateralborderssharp
, Kaposisarcoma,metastasis)/ septicemboli + parallelto rib cage
{ pulmonarycavities r/ migrationtowardhilastartingat lungperiphery;bilateral
usuallydue to superimposed fungal/ mycobacterial
infection , symmetry
! apparentdecreasein nodularity(incorporation of
NUC: nodulesfrom surroundings)
r/ bilateraland diffuseGa-67 uptakewithoutmediastinal r/ cavitation(occasionally) due to ischemicnecrosis/
involvement priorto roentgenographic changes superimposed TB infection
DDx: TB / MAI infection(withmediastinal r/ bullousscar emphysema
involvement) r/ pulmonaryhypertension
Dx: (1) sputumcollection,(2) bronchoscopy with
lavage,(3) transbronchial / transthoraciclopen
lungB x PSEUDOLYMPHOMA
Prognosis; rapidfulminantdisease;death within2 weeks = reactivebenignlesion= localizedform of lymphocytic
Rx: co-trimoxazole lV, nebulizedpentamidine interstitialpneumonitis (LlP);no progressionto
lymphoma
Histo: aggregatesof plasmacells,reticulincells,large
PNEUMONECTOMY
CHEST + small lymphocyteswith preservedlymphoid
Earlysigns(within24 hours): architecture resemblinglymphomahistologically
r/ partialfillingof thorax withoutlymphnodeinvolvement
r/ ipsilateralmediastinalshift + diaphragmaticelevation Associatedwith: Sjdgren syndrome
Late signs (after2 months): . mostlyasymptomatic
{ completeobliterationof space r/ well-demarcated dense infiltrate
N.B.: Depressionof diaphragm/ shiftof mediastinum to r/ infiltratetypicallyin centrallocationextendingto visceral
contralateralside indicatesa bronchopleural fistula pleura
/ empyemai hemorrhagel { prominentair bronchogram
r/ l,lO lymphadenopathy
POSTOBSTRUCTIVE
PNEUMONIA Prognosis; occasionallyprogressionto non-Hodgkin
= chronicinflammatorydiseasedistalto bronchial lymphoma
obstruction Rx: most patientsrespondwell to steroidsinitially
Cause:
1. Bronchogenic carcinoma(mostcommonly)
2. Bronchialadenoma PSEUDOMONAS
PNEUMONIA
3. Granularcell myoblastoma(almostalwaystracheal = rnostdreadednosocomialinfectionbecauseof
lesion) resistanceto antibioticsin patientswith debilitating
4. Bronchostenosis diseaseson multipleantibiotics+ corticosteroids; rare in
Histo: "goldenpneumonia"= cholesterol pneumonia community
endogenouslipidpneumonia= mixtureof edema, Organism; Pseudomonasaeruginosa,Gram-negative
atelectasis,roundcell infiltration,bronchiectasis, . bradycardia
liberationof lipidmaterialfrom alveolar . temperaturewith morningpeaks
, pneumocytes secondaryto inflammatoryreaction r/ widespreadpatchybronchopneumonia (secondaryto
r/ frequentlyassociatedwith some degreeof atelectasis
, bacteremia; unlikeotherGram-negative pneumonias)
{ persistsunchangedfor weeks r/ predilectionfor lower lobes
^/ recurrentpneumoniain same regionafter antibiotic r/ extensivebilateralconsolidation
treatment r/ "spongelike pattern"with multiplenodules>2 cm
(= extensivenecrosiswith formationof multiple
PROGRESSIVE MASSIVE FIBROSIS abscesses)
= (pMF)= COMPLTCATED PNEUMOCONtOStS ! smallpleuraleffusions
= CONGLOMERATE ANTHRACOSILICOSIS
May develop/ progressaftercessationof dust exposure PU LMONARY ARTERIAL MALFORMATION
Path: avascularamorphouscentralmass of insoluble = PAVM= PULMONARY ARTERIOVENOUS
ANEURYSM
proteinsstabilizedby cross-links+ ill-defined = PULMONARYARTERIOVENOUS = PULMONARY
FISTULA
bundlesof coarsehyalinizedcollagenat periphery ANGIOMA= PULMONARY
TELANGIECTASIA
514 RadiologyReview Manual

= abnormalvascularcommunication betweenpulmonary CT (98% detectionrate):

arteryand vein (95%)or systemicarteryand pulmonary ^/ homogeneous circumscribed noncalcified nodule/
vein (5%) serpiginousmass up to severalcm in diameter
Etiology: ^/ vascularconnectionof mass with enlargedfeeding
(a) congenitaldefectof capillarystructure(common) artery+ drainingvein
(b) acquiredin cirrhosis(hepatogenic pulmonary r/ sequentialenhancement of feedingartery
angiodysplasia),cancer,trauma,surgery, + aneurysmalpart + efferentvein on dynamicCT
actinomycosis, schistosomiasis,TB (Rasmussen MR (if contraindicationto contrastmaterial/ if flow slow
aneurysm) due to partialthrombosis/ for follow-up)
Path: hemangiomaof cavernoustYPe r/ signalvoid on standardspin echo/ highsignal
Pathophysiology: intensityon GRASSimages
low-resistance extracardiacR-to-Lshunt (whichmay Angio(mostlyobviatedby MR / CT unlesssurgeryor
resultin paradoxical embolism);quantification
with embolization contemPlated) :
Tc-99m-labeled albuminmicrospheres by measuring 0 100%sensitivefor detectionof vessels>2 mm
fractionof dose reachingkidneYs Cx: CNS symptomsare commonlythe initial
Age: 3rd-4thdecade;manifestin adultlife,10% in manifestation
childhood (1) Cerebrovascular accident:stroke(18%),
transientischemicattack(37%)secondaryto
Occurrence: paradoxicalblandemboli
(a) isolatedabnormalitY(40%) (2) Brainabscess(5-9%) secondaryto loss of
(b) multiple(in 1i3) pulmonaryfilterfunctionfor septicemboli
associatedwith Rendu-Osler-Weber syndrome (3) Hemoptysis(13%)secondaryto ruptureof
(in 30-60-88%) = hereditary PAVMintobronchus,most commonpresenting
hemorrhagic telangiectasia sYmPtom
0 15-50% of patientswith Rendu-Osler-Weber (4) Hemothorax(9%) secondaryto ruptureof
diseasehavepulmonaryAVMs! subPleuralPAVM
Types: (5) Polycythemia
1. Simpletype (79%) Prognosis: 26/" morbidity,11% mortality
= singlefeedingarteryemptiesintoa bulbous Recommendation: screeningof first-degreerelatives
nonseptated aneurysmalsegmentwith a single DDx: solitary/ multiplepulmonarynodules
dr ainin gv e i n Rx: embolization with coils/ detachableballoons
2. Complexrype (21%)
= ffrofethan one feedingarteryemptiesinto
septatedaneurysmalsegmentwith morethan one PULMONARY CAPILLARY HEMANGIOMATOSIS
drainingvein = bilateralpulmonarydiseasebehavinglikea low-grade
. asymptomaticin 56% (until3rd-4th decade)if AVM nonmetastatic vascularneoplasmwith slowly
singleand <2 cm progressive pulmonaryhypertension
' orthodeoxia(= increasedhypoxemiawith PaO, Histo: sheetsof thin-walledcapillarybloodvessels
pulmonaryinterstitium
infiltrating + invading
<85 mm Hg in erect positiondue to gravitationalshiftof
pulmonarybloodflow to base of lung) pulmonaryvessels,bronchioles, and pleura
. cyanosiswith normal-sized heart(R{o-L shunt)in Pathomechanismof pulmonary hypertension:
25-50/", clubbing (a) venoocclusive phenomenonsecondaryto invasionof
. bruitover lesion(increasedduringinspiration) smallpulmonaryveins
. dyspneaon exertion(60-71%) (b) progressivevascularobliterationsecondaryto in situ
. hemoptysis(10-15%) thrombosis+ infarction
. palpitation, chestpain (c) pulmonaryscar formationsecondaryto recurrent
. NoCHF pulmonaryhemorrhage
Location: lower lobes (65-70%) > middlelobe > upper Age: 20-40 years
. dyspneaon exertion
lobes;bilateral(8-20%);medialthirdof lung
. cor pulmonale:jugularvenousdistension,pedal
r/ sharplydefined,lobulatedoval / round mass (90%)of
1 to severalcm in size ("coinlesion") edema,ECG signsof RV failure(DDx: pulmonary
{ cordlikebandsfrom mass to hilum (feedingartery venoocclusivedisease)
. elevatedPA pressures+ normalpulmonarywedge
+ drainingveins)
4 inZtg singlelesion,in 1/3 multiplelesions pressure
. hemoptysis+ pleuriticchestpain in 1/3
r/ enlargementwith advancingage
(DDx: pulmonarythromboembolic disease)
{ changein sizewith Valsalva/ Muellermaneuver/ erect
vs. recumbentposition(decreasewith Valsalva CXR:
maneuver) ^/ ditfusereticulonodular pattern
r/ phleboliths (occasionally) r/ focal areas of interstitialfibrosis(recurrentepisodesof
r/ increasedpulsationsof hilarvessels pulmonaryhemorrhage+ thromboticinfarction)
 ChestDisorders 515

CT: Pathogenesis:
r/ thickening+ nodularityof inter-and intralobular septa increasedvascularpermeability (ischemiccapillary
+ walls of pulmonaryveins endothelialinjury)+ reperfusion via bronchialcirculation
r/ areas of ground-glassattenuation(= increased causesintraalveolarextravasationof blood cells in a
perfusionto extensiveproliferatinghemangiomatous confinedarea with possibleprogressionto infarction
tissue) Co-condition to progress to infarction:
A ngio: CHF, highembolicburden,underlyingmalignancy,
{ combinationof increasedflow (to hemangiomatous diminishedbronchialflow (dueto shock,hypotension,
areas)+ decreasedflow (to regionsof thrombosis, chronicallyimpairedcirculation), vasodilatoruse,
infarction,and scarring) elevatedpulmonaryvenouspressure,interstitial edema
Prognosis; death atter 2-12-year intervalfrom onset of Prognosis; replacementby vascularfibroustissue
symptoms foldinginto a collagenousplatelikemass
Rx: bilaterallungtransplantation producingpleuralretraction
DDx: (1) Pulmonaryvenoocclusive disease
(2) ldiopathicinterstitial
fibrosis
(3) Primarypulmonaryhypertension (no increasein PULMONARYINTERSTITIAL
EMPHYSEMA
lungmarkings) = PIE = complication of respiratortherapywith pEEp
(4) Pulmonaryhemangiomatosis (onlyin children, Pathogenesis:
cavernoushemangiomasinvolvingseveral gas escapesfrom overdistendedalveolus,dissectsinto
organs) perivascular sheathsurrounding arteries,veins,and
lymphatics,tracks into mediastinumformingclustersof
blebs
. suddendeterioration in patient'sconditionduring
PULMONARY
CONTUSION respiratorytherapy
= rTtoSt commonmanifestationof blunt chesttrauma,esp. r/ elongatedlucenciesfollowingdistribution of
decelerationtrauma bronchovascular tree
Path: exudationof edema + blood into airspace r/ pseudocysts= circulardensitiescausedby subpleural
+ interstitium blebs
Timeof onset: apparentwithin6 hoursaftertrauma r/ bilateral,symmetricaldistribution
. clinicallyinapparent ri lobaroverdistension(occasionally)
. hemoptysis(50%) C x: (1)pneumomedi asti num, pneumothorax,
Location: posterior(in 60%) subcutaneous emphysema,
Site: directlydeep to site of impact/ contrecoup pneumopericardium, intracardiac
air,
! irregularpatchy/ diffusehomogeneous extensive pneumoperitoneum, pneumatosis intestinalis
consolidation(CT is more sensitive) (2) air-block phenomenon = buildupof pressurein
r/ opacitymay enlargefor 48-72 hours mediastinum / pericardialtamponadeimpeding
r/ rapid resolutionbeginning2449 hours,completewithin bloodflow in low-pressure pulmonaryveins
, 2-10 days causingdiminishedbloodreturnto heart
! overlyingrib fractures(frequent) (obstructionesp.duringexpiration); particularly
CT: commonin neonatalperiod
r/ nonsegmentalcoarseill-definedcrescentic(SO%)I r/ microcardia
amorphous(45%)opacificationof lung parenchyma
withoutcavitation
{ "subpleuralsparing"= 1 -2-mm rim of uniformly PULMONARY
LYMPHANGIOMATOSIS
nonopacified subpleuralportionof lung = increasednumberof communicating lymphaticchannels
Cx: pneumothorax r/ smooththickeningof bronchovascular bundles
DDx: fat embolism(1-2 days after injury) + interlobularsepta
C T:
r/ diffuseincreasedattenuationof mediastinalfat
r/ mild perihilarinfiltration
PULMONARY
INFARCTION { pleuraleffusion
= ischemiccoagulativenecrosisof lung parenchyma r/ pleuralthickening
Frequency: rare (due to protectiveeffect of collateraf
bloodflow from bronchialcirculation)
Path: dark necroticmaterial(withfaint ghostlike P U LMON A R Y MA IN LIN E GR A N U LOMA TOSI S
structuresof lungtissueremainingevidenton = PULMONARY TALCOSIS
histology)surroundedby a narrowrim of hyperemia = pulmonarymicroembolism in drug addictsdue to
+ inflammation chroniclV injectionof suspensionspreparedfrom
Cause: pulmonaryarteryocclusion(medium-to small- crushedtabletcompounds(talcis a commoninsoluble
sized vessel) additive)
516 RadiologyReview Manual

Drugs: amphetamines,methylphenidate hydrochloride
("Westcoast"),tripelenamine("bluevelvet"),
methadonehydrochloride,dilaudid,meperidi ne,
pentazocine, propylhexedrine, hydromorphone
hydrochloride
Pathogenesis: talc (= magnesiumsilicate)particlesincite
a pronouncedgranulomatous foreign-
body reaction+ subsequentfibrosisin
perivascu lar distribution
Path: multiplescatteredwhitishnodulesof 0.3-3 mm
converginginto grittyfibroticmassesin central
+ upper lungsmeasuringseveralcm
widespreadgranulomaspackedwith doubly
Histo:
refractiletalc particlesexpandingthe walls of
muscularpulmonaryarteriesand arterioles
+ perivascular connectivetissue+ alveolar
septa
' talc retinopathy(80%)= Srn€lll glisteningcrystals
. angiothrombotic pulmonaryhypertension + cor pulmonale
Earlychanges:
r/ widespreadmicronodularity of "pinpoint"size
(1-2-3 mm) with perihilari basilarpredominance
r/ well-defined nodulespredominantly in middlezones
Late changes:
./ lossof lungvolumeof upperlobes+ hilarelevation
+ hyperlucency at lung bases
r/ indistinctlymarginatedcoalescentopacitiessimilarto
progressivemassivefibrosis(DDx:in silicosisslightly
furtherawayfrom pulmonaryhila+ distinctmargin)
Cx: mycoticpulmonaryarteryaneurysm;right-sided
endocarditis with septicemboli;chronicrespiratory
failure;emphysema;systemictalc breakthroughto
liver+ spleen+ kidneYs+ retina
DDx of late changes:
(1) Progressive massivefibrosisof silicosisI coal
worker'spneumoconiosis
(2) Chronicsarcoidosis
Dx: lung biopsy
DISEASE
THROMBOEMBOLIC
PULMONARY
= PULMONARYEMBOLISM(PE)
Prevalence: 630,000Americanslyearwith missed/
delayeddiagnosisin 400,000,causingdeath
in 120,000;diagnosedin 1o/oof all hospitalized
patients;in 12-64/" at autopsy;in 9-56% of
patientswith deep venousthrombosis
Age: 60% >60 years of age
Cause: deep vein thrombosis(DVT)of extremities/
pelvis(>90%),rightatrialneoplasia/ thrombus,
thrombogenicintravenouscatheters,
endocarditis of tricuspid/ pulmonicvalves
Timeof onset: PE usuallyoccurswithinfirst 5-7 days of
thrombusformation
Predisposing factors:
primarythrombophlebitis (39%),immobilization(32%),
recentsurgery (31"/"),
venous (25/"),
insufficiency
recentfracture(15%),myocardialinfarction(12%),
(6%)
malignancy(8/"), CHF (5%),no predisposition
Pathophysiotogy: A clot from the deep veins of the leg
breaksoff + fragmentsin rightside of
heart+ showerslungwith emboli
varyingin size
0 On average>6-8 vesselsare embolized!
Class1 = <2Oh of pulmonaryarteriesoccluded
. asymPtomatic
. normalarterialblood gas levels
. normalpulmonary+ systemichemodynamics
Class 2 = 20-30% of pulmonaryarteriesocclude
. anxiety,hYPerventilation
. arterialPO, <80 torr
. PCO, <35 torr
Class3 = 30-50% of pulmonaryarteriesoccluded
. dyspnea,collaPse
. arterialPO, <65 torr
. arterialPCO, <30 torr
. elevatedcentralvenouspressure
Class 4 = >5}o/oof pulmonaryarteriesoccluded
. shock,dysPnea
. arterialPO, <50 torr
. arterialPCO, <30 torr
. elevatedcentralvenouspressure
. mean PA Pressure>20 mm Hg
. systolicbloodpressure<100 mm Hg
0 Clinicalpresentation is protean+ nonspecific!
0 False-positive clinicaldiagnosisin 62/"
. Classictriad (<33%):
(1) hemoptysis(25-34%) (2) pleuralfrictionrub
(3) thrombophlebitis
. symptoms(nonfatalPE versusfatal PE):
. pleuriticchestpain (88%vs' 10%)
. acute dyspnea(84%vs. 59%)
. apprehension(59% vs. 17"/")
. cough(53%vs.3% )
. hemoptysis(30% vs. 3%)
. sweats(27%vs.9%)
. syncope(13% vs. 27"/")
. signs(nonfatalPE versusfatal PE):
. respiratoryrate >16 (92% vs. 66%)
. ralesdue to loss of surfactant(58% vs.42"/.)
. tachycardi>a100bpm (44% vs.54h)
. temperature>37.8"C(43/" vs.30%)
. diaphoresis(36%vs. 10%)
. heartgallop(34%vs. 10%)
. phlebitis(32% vs. 7'h)
. heart murmur(23%)
. cyanosi s(19%vs. 12/" )
. ECG changes(83%),mostlynonspecific:
P-pulmonale, right-axisdeviation,rightbundlebranch
block,classicS1Q3T3 Pattern
. elevatedlevelsof fibrinopeptide-A (FPA)= Srnall
peptidesplitoff of fibrinogenduringfibringeneration
. positiveD-dimerassay (generatedduringclot lysis)
Locationof PE: bilateralemboli(in 45'/.),RT lungonly
(36%),LT lungonlY(18%);multiple
emboli[3-6 on average]in 65%
Distribution:RUL (16%),RML (9%)' RLL (25%),LUL
(14/"), LLL (26%)

Site: central= Segffi€r]tal/ larger(in 5g%);
peripheral= subsegmentalI smaller(in 42%);
in subsegmental branchesexclusively(in 30%)
0 Emboliare occlusivein 4Oo/ol
R E S O LUT I O N OF P E
(throughfibrinolysis + fragmentation):
in 8% by 24 hours,in 56% by 14 days,in 6g% by 6
weeks,in77"/. by 7 months;completein 65%,
incompletein 39% by 11 months,partialin 23yo,no
resolutionin 12Y.
0 Resolutionless favorablewith increasingage
+ cardiacdisease
0 Resolutionimprovedwith urokinase> heparinwithin
firstweek (after1 year 80% for both)
A. EMBOLTSM WITHOUTTNFARCTTON (90%)
Histo: hemorrhage+ edema
B. EMBOLTSM W|TH TNFARCTTON (10_60%)
= ary opacitydevelopingas a resultof thrombo-
embolicdisease;more likelyto developin presence
of cardiopulmonary diseasewith obstructionof
pulmonaryvenousoutflow(diagnosedin retrospect)
Histo: (1) incompleteinfarction= reversibletransient
hemorrhagic congestion/ edemausually
resolvingover severaldays to weeks
(2) completeinfarction= hemorrhagic
with necrosisof lungparenchymaremaining
permanently
CXR (33% sensitive,59% specific):
0 AbnormalnonspecificCXR in 84/": a normalCXR
has a negativepredictivevalue of only 74%l
r/ generalfindings(patientswith pE vs. no pE):
r/ atelectasis/ infiltrate(6g% vs. 4g%)
{ pleuraleffusion(48% vs. 31%)
r/ pleuralopacity(35%vs.21./")
r/ elevateddiaphragm(24%vs. 19%)
r/ decreasedvascuiarity(217ovs. 12:/")
{ prominentpulmonaryartery(17% usi,.Zai%l
r/ cardiomegaly (12%vs. 11%)
, r / pulm onary e d e m a (4 %v s . 1 3 % )
ri localfindings:
r/ Westermarksign = or€? of oligemia(due to
vasoconstriction distalto embolus)in 2-7%
r/ Fleischnersign = localwideningoiartery by
impactionof embolus(dueto distensionby clot/
pulmonaryhypertensiondevelopingsecondaryto
, peripheralembolization)
r/ "knucklesign"= abrupttaperingof an occluded
vesseldistally
r/ Fleischnerlines= long-lineshadows(fibroticscar)
from invaginationof pleuraat the base of the
collapseresultingin pseudofissure
r/ Hamptonhump = s€grT'tefltally distributedpleura-
based shallowwedge-shapedconsolidationwith
base againstpleuralsurface+ convexmedial
border:
r/ trtOair-bronchogram (hemorrhageinto alveoli)
{ + cavitation
! "meltingsign"= withinfew days to weeks
regressionfrom peripherytowardcenter
r/ subsequentnodular/ linearscar
ChestDisorders 517
./ thoracentesis:bloody(65%),predominantly pMNs
(61%),exudate(OS%)
NECT (purpose):
r/ depictionof acutechangesof pE:
r/ atelectasis/ linearbands(100%)
r/ pleuraleffusion(87%)
r/ consolidation(57%)
r/ ground-glassopacification (57%)
r/ Hampton'shump (50%)
r/ ditatedcentral/segmentalpulmonaryartery
r/ depictionof chronicchangesof pE (see betow)
'
r/ chestfindingsleadingto ilternativediagnosis
r/ localizationof volume-of-interest for CECT
CECT (53-88-100% sensitive,78-9 4-1OO%specific):
0 HelicalCT equalto angioin detectionof emboliwithin
proximalarteriesof <Sth/ 6th generation
0 Subsegmental intraluminalfillingdefects(in 2-30%)
usuallynot detectable!
0 Detectionpoor in middlelobe+ lingularbranches(in
18%)t
N.B.: evaluatethe vesseladjacentto a bronchus
r/ partialcentralfillingdefect in pulmonaryartery:
r/ peripheralrimlikecontrastenhancement
r/ "railwaytrack"sign = thromboembolusfloating
infarction freelywithinlumen
! partialmarginalfillingdefectin pulmonaryartery:
r/ muralfillingdefectof low attenuation
r/ completefillingdefectof low attenuationoccupying
entirearterialsection
PseudoJiIIing defects:
(1) Breathingartifactin tachypneicpatient
(2) Too short/ longscanningdelay
(3) Unilateralincreasein vascularresistance
(4) R-to-Lshunt
Technicalfailurerate: g4% due to severe dyspnea
NUC (VO sc€ln= guidefor angiographic evaluation)
interpretedin referenceto Bielloor plOpED criteria:
r/ low- / intermediate-probability scans (in 73%):
additionalstudiesrecommended
0 25-30% disagreementbetweenexpertreadersin
interpretingintermediate-and low-probability V/e
SCANS
^/ nign-probability scan: in 1Z/. normalangiogram
N.B.: V/Q abnormalities vary over time due to
autoregulation(hypoxicvasoconstriction,
hypercapneic bronchoconstriction)and resolution
Angio(>95%sensitive+ specific):
lndication:
(1) lndeterminate NUC scan (angiowithin24 hours)
(2) Mismatchbetweeninterpretation + clinicalfindings
(3) Significantriskfor anticoagulation + high
probabilityfor PE
(4) Priorto intervention:pulmonaryembolectomy,
caval ligation,cavalfilter placement
(5) Patientstoo ill to undergoV/e scan
Technique; AP & ipsilateralposteriorobliqueprojection
r/ intraluminaldefect (94%)
{ abruptterminationof pulmonaryarterialbranch
r/ pruning+ attenuation of branches
r/ wedge-shapedparenchymalhypovascularity
518 Radiology Review Manual
{ absenceof drainingvein in affectedsegment
r/ tortuousarterialcollaterals
Bisks in pulmonary angiograPhY:
(1) left bundlebranchblock: requirestemporary
pacingwire priorto right heartcatheterization
(2) marginalcardiacfunction:therapymustbe
availableto treat frank pulmonaryedema
(3) Rightventricularend diastolicpressure>20 mm
Hg: selectivecatheterization with occlusion
balloon
Cx of pulmonary angiography (1-2%):
arrhythmia,endocardialinjury,cardiacperforation,
cardiacarrest,contrastreaction
Mortality rate of pulmonary angiography: 0.2-0'5%
False-negativerate:
1-4-9% due to difficultyin visualizingsubsegmental
emboli (withonly 30% interobserveragreementabout
presenceof subsegmental emboli)
PulmonaryArterial
AcuteThromboembolic
Hypertension
0 Hypertension disappearsas embolilyse
. suddenonsetof chestPain
. acute dyspnea
. hemoptysisoccasionally
Mortality:
3:1,000 surgicalprocedures;200,000deathsin 1975;
7-10% of all autopsies(deathwithinfirsthourof PE in
most patients);26-30%rt untreated;3-10% if treated;
fatal if >60/" of pulmonarybed obstructed;healthy
patientsmay surviveobstructionof 50-60% of
vascularbed
Rx:
1. HeparinlV: 10,000-15,000unitsas initialdose;
8,000-10,000units/hourduringdiagnostic
evaluation; continuedfor 10-14 days
2. Streptokinase:better resultswith massive PE
3. Urokinase:slightlybetterthan streptokinase
4. Coumadin:maintainedfor at least3 months
(15% complicationrate)
PulmonaryArterial
ChronicThromboembolic
Hypertension
= CHRONIC THROMBOEMBOLIC DISEASE
Frequency: 1-5/" of patientswith acute pulmonary
thromboembolism
At risk: underlyingmalignancy,cardiovascular
disease,PulmonarY disease;M < F
Path: fibrouswebs and bands (= oroanized
thromboemboli), oftenwith overlyingrecent
thrombosis
Pathogenesis:
patentpulmonaryarteriesdevelopmedialhypertrophy
+ intimalthickening+ atheroscleroticplaquesin
responseto pressureelevation;bronchialarteries
may dilate + form extensivecollateralpathwaysto
minimizeareas of lung infarction
. may be clinicallysilent/ asymptomaticfor years
("honeymoonPeriod")
. historyof previousembolicepisodes
. recurrentacute/ gradualprogressiveexertional
dyspnea(DDx: interstitial lungdisease)
. chronicnonproductivecough,atypicalchest pain
. tachycardia,syncoPe
. elevatedpulmonaryarterialpressure(36-78 mm Hg),
normalpulmonarycapillarywedgepressure
. high right atrialpressures,reducedcardiacoutput
. lupusanticoagulant (11-24%)
CXR:
{ prominenceof rightside of heart
^/ asymmetricenlargementof centralpulmonary
arteries
r/ oligemicvascularityin patchydistribution
r/ triangular/ roundedopacity+ adjacentpleural
thickening(frompulmonaryinfarction)
! patchybilateralperihilaralveolaropacitiesof
"reperfusionedema"afterthrombendarterectomy
CT (77% sensitive):
{ cardiacchanges:
i/ hypertrophy+ enlargementof RA + RV
^/ vascularabnormalities:
{ main pulmonaryarterydiameter>28.6mm
r/ completefillingdefectat levelof stenosed
pulmonaryarterY
{ calcifiedclot
{ eccentricmuralarterialirregularities / nodules
{ arterialstenosis/ web
{ evidenceof recanalization
abruptnarrowing/ cutoffof distallobarI
segmentalbranches
decreasein caliberof smallbranches
+ narrowingof peripheralpulmonaryvessels
collateralsystemicsupplyof occludedpulmonary
arterialbed:
^/ bronchialarterydilatation+ tortuosity(77%)
withinmediastinum
r/ parenchymalabnormalities:
r/ wedge-shapedpleura-basedparenchymalbands
with tip pointingto hila,oftenmultiple,esp'
involvinglower lung (70%) = infarctedtissue
replacedby scar
r/ mosaicperfusionon HRCT:
{ scatteredgeometricareas of low attenuationin
55% (dueto oligemia)associatedwith vessels
of smallcross-sectional diameter
{ regionalsharplydemarcatedareasof high
attenuation(perfusedlung on backgroundof
oligemic/ nonPerfused lung)
primary pulmonary hypertension (more
[DDx:
diffusepatternof mosaicperfusion)l
r/ cylindricbronchialdilatationof segmental/
subsegmentalbronchi(64%) adjacentto stenotic/
obstructedpulmonaryarterialsegment
MR (lowestsensitivitY):
r/ discretefixed areas of low-to-mediumsignal
intensityon Tl Wl
Disadvantage: slow flow in centralvesselsmay
obscureembolicfixedsignal
NUC:
r/ VIO scan characteristically of high probability
 ChestDisorders 519

Angio(highestspecificity): Cx: subacutecor pulmonale(heraldsdeathwithin

r/ webs, bands
4-12 weeks)
{ stenotic/ absentarterialsegments
{ pouchlikefillingdefects MercuryEmbolism
r/ abruptcutoffsoftenconfinedto 1 l2 lungsegments Cause: accidental/suicidallV injection
r/ unilateralocclusion/ hypoperfusion Pathomechanism: intravascularmercurybecomes
r/ selectivebronchiatarteiiographyshows dilated encasedin thrombusor migratesinto
bronchialarterycollaterals(up to 30% of systemic pulmonaryinterstitium/ alveolar
bloodflow)fillingpulmonaryarteriesdownstream spacesresultingin significant
from sitesof occlusion
r/ markedlyelevatedpulmonaryarterypressure
Prognosis: 30./" S-yearsurvivalwith a mean pA
r/nisn-oensity
rine-?:1#:n:f.ffTil::res
in
symmetricdistribution
pressureof 30 mm Hg { mercurycollectionwithinapex of rightventricle
Rx: thrombendarterectomy (aO%operative
mortafity); suppfementalwarfarin anticoagulation PULMONARY
VENOUSVARIX
theraPY+ vasodilators = abnormaltortuosity+ dilatationof pulmonaryveinjust
beforeentranceinto left atrium
TumorEmbolism Etiology: congenital/associatedwith pulmonaryvenous
0 Diagnosisfrequentlymisseduntilpostmortemexam! hypertension
Frequency: 2-2G./"of patientswith known malignancy . usuallyasymptomatic;may cause hemoptysis
Primary: gastriccarcinoma(mostcommon),breast, Location: medialthirdof eitherlungbelowhilacloseto
prostate,lung,hepatocellular,ovarian, left atrium
osteosarcoma, lymphoma,choriocarcinoma r/ well-defined lobulatedround/oval mass
0 Rightatrialmyxoma+ RCC tend to { changein size duringValsalva/ Muellermaneuver
embolizeto largecentral+ segmental ! opacificationat same time as LA (on CECT)
pufmonaryarteriesl Risk: (1) death upon ruptureduringworseningheart
Pathogenesis: tumor cells form emboliin vena cava failure
subsequently occludesmallmuscular (2) sourceof cerebralemboli
pulmonaryarteries+ arterioles DDx: pulmonaryarteriovenousfistula
Histo: intravascularmalignantcells,acuteand
organizingplatelet{ibrin thrombi,small artery RADIATION PNEUMONITIS
intimalfibrosis,adjacentintrafymphatictumor = damageto lungsafter radiationtherapydependson:
. progressive dyspnea,cough,pleuriticchestpain (a) irradiatedlungvolume(mostimportant):
. hemoptysis,syncope
. asymptomaticin <25o/o of lung volume
. hypoxemia<50 mm Hg
(b) radiationdose (almostalwaysexceedscriticalvalue
CX R:
for tumoricidaldoses):
r/ enlargedcentralpulmonaryarteries - pneumonitisunusual if <20 Gy given in 2-3 weeks
{ cardiomegaly - pneumonitis
r/ ill-defined commonif >60 Gy givenin 5-6 weeks
nodular/ confluentperipheral - significantlyincreasedriskfor pneumonitis if daily
parenchymal opacities(withmultiplepulmonary dose fraction> 2.67 Gy
infarcts) (c) fractionationof dose
CT: (d) concurrent/ laterchemotherapy
r/ subpleurallinear+ wedge-shapedopacities(at Pathologicphases:
, sitesof pulmonaryinfarctions) (1) Exudativephase= edemafluid+ hyalinemembranes
r/ companionmanifestations: lymphadenopathy, (2) OrganizingI proliferativephase
pulmonaryvenoushypertension, lymphangitic (3) Fibroticphase = interstitialfibrosis
carcinomatosis Timeof onset: usually4-6 monthsaftertreatmenr
CECT: Location: confinedto radiationportals
^/ titlingdefectsin mainpulmonaryarterybranches
1. A C U TER A D IA TION P N E U MON ITIS
r/ multifocalbeading* dilatationof suOs-egmental
Onset: within4-8 (1-12) weeks after radiationRx
pulmonaryarteries
Path: depletionof surfactant(1 week to 1 monthlater),
NUC: plasmaexudation,desquamation
r/ multiplesmallsubsegmental of alveolar
unmatchedperfusion + bronchialcells
defects on V/Q scan . asymptomatic(majority)
Angio: . nonproductive cough,shortnessof breath,
r/ delayedarterialphase weakness,fever(insidiousonset)
! fillingdefects i occlusionsof subsegmental arterial . acute respiratoryfailure(rare)
branches r/ changesusuallywithinportalentryfields
^/ arterialwall irregularities
! patchy/ confluentconsolidation, may persistup to
r/ peripheralpruningof smallerarteries 1 month (exudativereaction)
520 Radiology Review Manual

{ atelectasis+ air bronchogram ./ diffusegranularityof reticulogranularpattern(coincides

r/ spontaneouspneumothorax(rare) with onsetof clinicalsigns)= visualizationof distended
CT: terminalbronchioles + alveolarductsagainsta
r/ homogeneousslighthazy increasein attenuation backgroundof alveolaratelectasis
obscuringvesseloutlines(2-4 monthsafter Rx) { prominentair bronchograms (distensionof compliant
{ coalescingpatchyconsolidations (1-12 months airways)
aftertherapy)not conformingto shape of portals r/ hypoaerationwith loss of lung vofume(counteractedby
r/ nonuniformdiscreteconsolidation (mostcommon; respiratortherapy)
3 monthsto 10 years aftertherapy)formingsharp ./ bilateral+ symmetricaldistribution
edge,which conformsto treatmentportals Prognosis: spontaneousclearingwithin7-10 days (mild
Prognosisi recovery/ progressionto death / fibrosis coursein untreatedsurvivors);death in 18/"
Rx: steroids Rx: exogenoussurfactantintratracheally resultsin rapid
2. CHRO NI CR AD IAT ION D A M AGE clearingof RDS
Onset: 9-12 (6-24) monthsafter radiationtherapy; DDx: (1) Diffusepneumoniaaccompanying sepsis
stabilizedby 1-2 years aftertherapy (2) Retainedfetallungfluid(firstfew hours)
Histo: permanentdamageof endothelial+ type I (3) Pulmonaryhemorrhage
alveolarcells (4) Pulmonaryvenouscongestion(eg,TAPVR,
May be associated with: pulmonaryvein atresia,hypoplasticleft heart)
(1) thymiccyst (5) Prematurewith acceleratedlung maturity
(2) calcifiedlymphnodes(in Hodgkindisease) (PALMbaby)
(3) pericarditis+ effusion(within3 years)
r/ severeloss of volume
r/ densefibrousstrandsfrom hilumto periphery A C U TE& S U B A C U TEC OMP LIC A TIONOFS RDS
r/ thickeningof pleura (a) Persistentpatencyof ductusarteriosus(PDA)
r/ pericardialeffusion oxygenstimulusis missingto closeduct;gradual
CT: decreasein pulmonaryresistance(by end of
r/ solidconsolidationwith parenchymaldistortion 1stweek)leadsto significantL-to-Rshunt
(due to radiationfibrosis+ atelectasis) (b) Barotrauma with air-blockphenomena
tractionbronchiectasis (c) Hemorrhage
,Vmediastinalshift 1. P ul monary hemorrhage
pleuralthickening 2. Cerebralhemorrhage
(d) Focalatelectasis(usuallyfrom mucus plug)
RESPIRATORY
DISTRESS OF
SYNDROME (e) Persistingfetal circulation
NEWBORN (f) Myocardialischemia
= RDS = HYALINE MEMBRANE DISEASE (g) Diffuseopacity
= ?cute pulmonarydisordercharacterizedby generalized 1. WorseningRDS (first1-2 days only)
2. Congestiveheartfailure(PDA,fluidoverload)
atelectasis,intrapuImonaryshunting, ventilation-
perfusionabnormalities, reducedlungcompliance 3. Pulmonaryhemorrhage
4. Superimposed pneumonitis
Cause: immaturesurfactantproduction(usuallybegins
at 18-20 weeks of gestationalage) leadsto 5. Massiveaspiration
6. Stage ll bronchopulmonary dysplasia
increasedalveolarsurfacetension+ decreased
alveolardistensibilitycausingacinaratelectasis 7. "Weaningeffect"from removalof endotracheal
(persistentcollapseof alveoli)+ dilatationof tube / diminishedventilatorpressure
8. Extracorporeal membraneoxygenation
terminalainrvays
(h) Disseminatedintravascularcoagulopathy
Histo: uniformlycollapsedalveoli+ variabledistensionof
linedby (i) Necrotizingenterocolitis
alveolarducts+ terminalbronchioles;
2'to proteinseepage (j) Acute renalfailure
fibrin("hyalinemembranes")
from damagedhypoxiccapillaries (k) Metabolicdisturbance(eg, hyperbilirubinemia,
Predisposed: perinatalasphyxia,cesareansection, hypocalcemia)
infantsof diabeticmothers,premature
i n fa n ts(< 1 ,0 0 0g i n 6 6 % ; 1 ,0 0 0g i n 50% ; C H R ON ICC OMP LIC A TIONOFS RDS
1 ,5 0 0g i n 16 /" ;2 ,0 0 0g i n 5 % ; 2 ,500g i n 1. Bronchopulmonary dysplasia(10-20%)
1%) 2. Subglotticstenosis(intubation)
Onset: <2-5 hoursafter birth,increasingin severity 3. Localizedinterstitialemphysema
lrom 24 to 48 hours,gradualimprovementafter 4. Hyperinflation
48- 7 2 h o u rs ; M:F = 1 .8 :1 5. Retrolentalfibroplasia
. abnormalretractionof chestwall 6. Malnutrition,rickets
. cyanosis(carbondioxideretention) 7. Lobaremphysema
. expiratorygrunting L Delayedonset of diaphragmatichernia
. increasedrespiratoryrate 9. Recurrentinspiratorytract infections
 ChestDisorders 521

RETAINED
FETALLUNGFLUID . low in sugar content(<30 mgidl) withoutrise
= NEONATAL WETLUNGDISEASE = TRANSIENT duringglucoseinfusion(75%)
RESPIHATORY DISTRESS OF THENEWBORN . low WBC high in lymphocytes
= TRANSIENT TACHYPNEA OFTHENEWBORN .
lncidence: 6Y"; most common cause of respiratory , positivefor rheumatoid factor,LDH, RA cells
{ pleuralthickening,usuallybilateral
distressin newborn
B . D TFFU S E tN TE R S T| T| AFtB
L R OS tS(30% )
Cause: cesareansection,precipitousdelivery,breech
Prevalence: 2-9% of patientswith rheumatoid
delivery,prematurity,maternaldiabetes
Pathophysiology: arthritis
. restrictiveventilatorydefect
delayedresorptionof fetal lung fluid (normalclearance
Location: lowerlobepredominance
occurs throughcapillaries(40%),lymphatics(g}y"),
Histo: depositionof lgM in alveolarsepta (DDx to
thoraciccompressionduringvaginaldelivery(30y");stiff
, IP F)
lungscauselaboredventilationuntilfluidis cleared
I punctate/ nodulardensities(mononuclear cell
Onset: within6 hoursof life;peaks at 1st day of life
' increasingrespiratoryrates duringfirst2-6 hours infiltratesin early stage)
of fife r/ reticulonodular densiiies
. intercostal+ sternalretraction
. normalbloodgases duringhyperoxygenation r/ mediumto coarsereticulations(maturefibroustissue
./ linearopacities+ perivascutainaze + thickenedfissures in laterstage):
r/ irregularinterlobular septalthickeningon HRCT,
+ interlobular septalthickening(interstitial
edema): predominantlyin peripheryof lower lung zones
, symmetricperihilarradiatingcongestion
r/
^/ honeycomblung (uncommonin late stagJ; witn
r/ mild hyperaeration
progressive volumeloss
tr mild cardiomegaly
r/ smallamountof pleuralfluid C . N E C R OB IOTIC N OD U LE S(rare)
= wefl-circumscribed nodufarmass in lung, pleura,
Prognosis; resolvingwithin 1-2^4 days (retrospective
pericardium identicalto subcutaneous nodules
diagnosis)
associatedwith advancedrheumatoidarthritis
DDx: (1) Normal(duringfirstseveralhoursof life)
Path: centralzone of eosinophilic fibrinoidnecrosis
(2) Diffusepneumonitis / sepsis surroundedby palisadingfibroblasts;nodule
(3) Mild meconiumaspirationsyndrome
often centeredon necroticinflamedblood vessel
(4) Alveolarphaseof RDS
(? vasculitisas initiallesion)
(5) "Drownednewbornsyndrome"= clearamniotic . subcutaneous nodules(samehistology)
fluidaspiration
Associatedwith: interstitiallung disease
(6) Pulmonaryvenouscongestion(eg, leftheart
r/ well-circumscribed usuallyrrtiipt. nodulesof 3-70
failure,overhydration,placentaltransfusion)
mm i n si ze
(7) Pulmonaryhemorrhage
r/ commonlylocatedin lung periphery
(8) Hyperviscosity syndrome= thick blood r/ cavitationwith thicksymmetricwalls + smoothinner
(9) lmmaturelungsyndrome= prern?ture with l i ni ng(i n 50% )
acceleratedlung maturity(PALMbaby) ^/ ttO calcification
D. CAPLANSYNDROME
= RHEUMATOID PNEUMOCON|OSTS
RHEUMATOID LUNG = pneumoconiosis + rheumatoidarthritisin coal
= autoimmune
disease
of unknown
pathogenesis workerswith rheumatoiddisease;
Prevalence: 2-54% of patientswith rheumatoidarthritis; = hypersensitivity reactionto irritatingdust particlesin
M:F = 5:1 (althoughincidenceof lungsof rheumatoidpatients
rheumatoidarthritis:M. F) lncidence: 2-6% of all men affectedby
. rheumatoidarthritis
pneumoconioses (exclusivelyin Wales)
Stage1: multifocalill-defined alveolarinfiltrates Path: disintegratingmacrophagesdeposita
Stage2: fine interstitialreticulations(histio-and pigmentedring of dust surroundingthe central
lymphocytes) necroticcore + zone of fibroblastspalisadingthe
Stage3: honeycombing zone of necrosis
A. PLEURALDISEASE(mostfrequentthoracic 0 NOT necessarilyevidenceof long-standing
manifestation) pneumoconiosis
. Hx of pleurisy(2i%) . concomitantwith joint manifestation(mostfrequent)/
Associatedwith: pericarditis,subcutaneousnodules may precedearthritisby severalyears
r/ pleuraleffusion(3%) with littlechangeover months: . concomitant with systemicrheumatoidnodules
r/ unilateral(g2%),may be loculated r/ rapidlydevelopingwell-definednodulesof S-50 mm
r/ most oftenwithoutoiher pulmonarychanges in size with a tendencyto appearin crops
' M : F= 9 ; 1
. usuallylate in the disease,but may antedate , predominantly in upperlobes+ in peripheryof lung
I nodulesmay remainunchanged/ increasein
rheumatoidarthritis numberI calcity/ result in thick-walledcavities
. exudate(withproteincontent>4 g/dl)
r/ backgroundof pneumoconiosis
522 Radiology Review Manual
r/ pleuraleffusion(may occur)
E . B RO NCHT AL A BN O R M AL T T T(3
E S0 % )
r/ bronchiectasis
r/ bronchiolitisobliterans(maybe transient
+ regardlessof penicillamine i goldtherapy):
{ mosaicpattern(= df€as of decreasedattenuation
+ vascularity) on end-inspiratory HRCT
r/ air trappingon end-expiratory HRCT
r/ bronchiolitis obliteransorganizingpneumonia
(BOOP):
^/ bilateralair-spaceconsolidationin peripheral/
peribronchial distribution
r/ follicularbronchiolitis(in 66%):
r/ small centrilobularnoduleswith patchyareas of
ground-glass attenuation
F . P ULM O NA R Y AR T ER IT IS
= fibroelastoid intimalproliferationof pulmonary
arteries
. pulmonaryarlerialhypertension+ cor pulmonale
G . CA RDI A CE N L AR G E M EN T
(pericarditis+ carditis/ congestiveheartfailure)
H . B O NEA B NO R M AL IT IEON S CXR
{ erosivearthritisof acromioclavicular joint,
sternoclavicular joint,shoulderjoint:
r/ resorptionof distalend of clavicles
r/ ankylosisof vertebralfacetjoints
r/ vertebralbody collapsedue to steroiduse
ROUNDPNEUMONIA
= NUM M ULAPRN EU M O N IA
= fairlysphericalpneumoniacausedby pyogenic
organisms
Organism; Haemophilusinfluenzae,Streptococcus,
Pneumococcus
Age: children>> adults
. cough,chestpain,fever
Location: alwaysposterior,usuallyin lowerlobes
r/ sphericalinfiltratewith slightlyfluffyborders+ air
bronchogram
r/ triangularinfiltrateabuttinga pleuralsurface(usually
seen on lateralview)
r/ rapidchangein size and shape
SARCOIDOSIS
= BOECKSARCOID lsarkos, Greek = flesh;sarcoid
= s?rcoln€l-like;
Caesar Boeckdescribesskin lesionsin
1seel
= immunologically mediatedmultisystem granulomatous
diseaseof unknownetiologywith variablepresentation,
progression, and prognosis
Prevalence:10-40:100,000in USA
Age peak: 20-40 years; M:F = 1:3 (morecommonin
women + peopleof West Africandescent);
AmericanBlacks:American Whites= 10:1
(rarein African/ SouthAmericanBlacks);
morecommonin bloodgroupA
Epidemiology:
found with varyingfrequencyin every countryin the
world;higherprevalencein temperateclimates
comparedto tropicalregions(<10/100,000)
lmmunology:
unknownantigenactivatesalveolarmacrophages,
whichrelease
- interleukin-1 (T-cellactivator)
- fibronectin(fibroblastchemotacticfactor)
- alveolarmacrophage-derived growthfactor
(stimulates fibrosis)
and activatesT lymphocytes,which release
- interleukin-2 (stimulatesgrowthof T-helper/
cytolyticcells)
- immuneinterferon(polyclonalB-cellactivator)
- monocytechemotacticfactor (attractscirculating
monocytesand stimulatesgranulomaformation)
Histo: alveolitis(earliestchanges);noncaseating
epithelioidgranulomas[composedof lymphocytes,
peripheralfibroblasts, multinucleatedgiantcellsl
with occasionalminimalcentralnecrosis
Location: alongcourseof lymphaticvessels:
subpleural, septal,perivascular,
peribronchial
DDx: indistinguishable from granulomasof
berylliosis,treatedTB, leprosy,fungal
disease,hypersensitivity pneumonitis,
Crohndisease,primarybiliarycirrhosis
. angiotensin-converting enzyme(ACE)elevatedin 70'/"
[ACE is a product of macrophages and an indicatorfor
the granulomaburdenof the bodyl
DDx: tuberculosis, leprosy,histoplasmosis, berylliosis,
cirrhosis,hyperthyroidism, diabetes
. hypercalcemia + hypercalciuria in 2-15% [resultof
hydroxylation of 1,25-dihydroxy vitaminD in macrophages
leadingto increasedintestinalresorptionof calciuml
. Kveim-Siltzbach test (positivein 70%) = intracutaneous
injectionof 0.1-0.2 mL of a previouslyvalidatedsaline
suspensionof humansarcoidspleeni lymphnodes,
rarelyused
. functionalpulmonaryimpairment(evenwith NO
radiographic abnormality) :
- reducedVC + FRC + TLC [fromgeneralized
reductionin lungvolumel
- low lung compliance[fromdiffuseinterstitialdisease]
- obstructiveairwaydisease[fromendobronchial
lesions,peribronchialfibrosis]
Dx: basedon a combination of clinical+ radiological
+ histologicfeaturesafter exclusionof other
i inflammatory
infectious entities
A. ACUTEFORM= L6fgren Syndrome (17%)
. fever+ malaise+ bilateralhilaradenopathy
. erythemanodosum
. arthralgiaof largejoints
. (occasionally) uveitis+ parotitis
B . C H R ON ICFOR M
. asymptomatic(50%)
. fever,malaise,weightloss
. dry cough + shortnessof breath(25%)
. hemoptysisin 4/o (fromendobronchial lesion/
vascularerosion/ cavitation)
 ChestDisorders 523

Stage at presentation: { bronchialstenosis(extrinsiccompressionby targe

0 normalchest radiograph 5%
I hilar+ mediastinal , lymphnodes/ endobronchial granulomas)
lymphadenopathy only SO% { bronchiectasis(scarringi fibrosis)
ll lymphadenopathy + parenchymaldisease gO% H R C T:
lll diffuseparenchymal diseaseonly 15% r/ irregularseptalthickening
lV pulmonaryfibrosis 20% ! perilymphaticnodules(= small nodufesalong
bronchoarterial bundlesand veins,in subpleural
Prognosis: + interlobularseptallymphaticsrepresenting
80% spontaneousremissionof stage 1 + 2 disease epithelioid cell granulomas)
75% completeresolutionof hilaradenopathy { tractionbronchiectasis(TyplCAL)
33% completeresolutionof parenchymaldisease ^/ ground-glassopacity(in alveolitisi
30% improvesignificantly r/ bullaein honeycombing
20% irreversiblepulmonaryfibrosis(may persist r/ irregular/ nodularbronihialwall thickening
unchangedfor >15 years) r/ air trapping
10% mortality(cor pulmonaleICNS / lung fibrosis/ Atypical manifestations (2i%) :
livercirrhosis) r/ pleuraleffusion(2%) = exudatewith
25% relapse (in S0% detectedby CXR) predominanceof lymphocytes,effusionclears in
2-3 months
@ Thoracicdisease(90%) r/ focal pleuralthickening
. mild symptomsin spite of extensiveradiographic
r/ solitary/ multiplepulm-onary nodules
changes(DIAGNOSTtCALLY StcN IFTCANT) r/ cavitationof nodules(0.67")
- adenopathyalone (49%)
r/ isolatedhilar/ mediastinal nodalenlargement
- adenopathy+ parenchymal disease(41%) r/ bronchostenosis (2%) with lobari segmental
- parenchymaldiseaseafone(16%)
atelectasis
Associatedwith: tuberculosisin up to 13% r/ pulmonaryarterialhypertension(periarterial
r/ intrathoraciclymohadenopathy (>g5%): granulomatosiswithoutextensivepulmonary
Location: fibrosis)
(a) "1-2-3sign" = Garlandtriad = symmetric Cx: ",1pneumothoraxsecondaryto chronic fung
bilateralhilar nodes + rightparatrachealand fibrosis(rare)
aortopulmonary windownodes (7S_gS%) r/ cardiomegalyfrom cor pulmonale(rare)
(b) isolatedunilateralhilarenlargement(1-g%) r/ aspergilloma formationin apicalbulla(in >S0%
(c) mediastinalnodesare regularlyenlargedon of stage lV disease)
CT Diagnostic criteria:
Prognosis; adenopathycommonlydecreasesas (1) compatibleclinical+ radiologicpicture

3il::i;XHii:
i:?Hi
fl:l'.H:;;,,'
32%; adenopathydoes not develop
(2) noncaseousepithelioid granulomason bronchiali
transbronchialbiopsy(diagnosticresultsin
60-95% and 80-95% respectively)
, subsequent to parenchymaldisease (3) negativeresultsof specialstains/ culturesfor
{ eggshellcalcificationof lymph nodes (in 3/" after other entities
, 5 years,in20"/" after 10 years) ASSESSMENTOF ACTIVITY
r/ parenchymaldisease(60y.);withoutadenopathyin (1) ACE titer(= angiotensinlconvertingenzyme)
16-200/": (2) Bronchoalveolar lavage: AO-SO'/" lymphocytes
0 Parenchymalgranulomasare invariablypresent with numberof T-suppressorlymphocytes
on open lung biopsyl 4-20 times above normal
Site: predominantly mid-zoneinvolvement (3) Galliumscan
r/ reticulonodularpattern (46%)
r/ uptakein lymph nodes + lung parenchyma
r/ acinarpattern(20%)= iil-detlned6-7-mm nodules
+ salivaryglands(correlateswith alveolitis
/ coalescentopacities + diseaseactivity);monitorof therapeutic
{ "alveolarI acinarsarcoidosis"(2-1Oo/o) = multiple response(indicatorof macrophageactivity)
nodules>10-50 mm (= coalescence of numerous 0 Extrathoracicmanifestations withoutintrathoracic
interstitialgranulomas): involvement occurin <10%!
^/ inoistincimargins
r/ t air bronchogram @ Abdominaldisease
r/ + cavitationoi occasionalnodule . strikinglyelevatedACE levelsin g1%
./ progressivefibrosiswith upper lobe retraction
@ Liver (pathologicinvolvementin 2fS9%\
, + bullae(20/.) ./ homogeneoushepatomegaly(1 B-2g%)
r/ irreversiblefibroticchangesof end-stagelung r/ 2-5-mm nodularlesionsin liverand spleenin
, disease(1F2A%) 5-15% (= coalescentgranulomata)occurring
r/ airwaydisease:
r/ trachealstenosis , within5 years of diagnosis
r/ abdominaladenopathy(meansize of 2.6 cm)
524 Radiology Review Manual

MR: Predisposed: lV drug abusers,alcoholism,

./ heterogeneous/ nodularhepatictextureand immunodeficiency, CHD,dermalinfection
periportalhighsignalintensityon T2Wl (cellulitis,carbuncles)
@ Spleen(pathologicinvolvementin 2+59%) Source:
! splenomegaly(20-33%) (a) infectedvenouscatheter/ pacemakerwires,
{ scatterednodularlesions(18%) arteriovenous shuntsfor hemodialysis, drug abuse
@ Lymphadenopathy (10-31%) producingsepticthrombophlebitis (eg, heroin
. frequentlyassociatedwith thoracicadenopathy addicts),pelvicthrombophlebitis, peritonsillar
{ mean lymphnodesize of 2.6 cm abscess, osteomYelitis
@ Pancreas (b) tricuspidvalveendocarditis (mostcommoncausein
r/ mass + pain mimickingpancreaticcarcinoma lV drug abusers)
@ Gastrointestinal disease: Age: majority<40 years
Location: anywherefrom esophagusto rectum . sepsis,cough,dysPnea,chestPain
@ Stomach(60 cases) . shakingchills,highfever,severesinustachycardia
{ polypoid/ nodularmass Location: predilection for lungbases
r/ + ulcer(simulating pepticulcerdisease) { multiplenondescript pulmonaryinfiltrates (initially)
r/ diffusefold thickening r/ migratoryinfiltrates(old ones heal, new ones appear)
r/ circumferential narrowing+ loss of antral r/ cavitation(frequent), usuallythin-walled
compliance(resembling scirrhouscarcinoma) r/ pleuraleffusion(rare)
@ Esophagus CT (moresensitivethan CXR):
r/ ptaquelikelesions,narrowing,aperistalsis { multipleperipheralparenchymal nodules+ cavitationi
@ Colon air bronchogram(83%)
./ annularinvolvement(tendsto be focal)with ^/ wedge-shaped subpleurallesionwith apex of lesion
obstruction directedtowardpulmonaryhilum(50%)
@ Genitourinary disease(0.2-5%): r/ feedingvesselsign = pulmonaryarteryleadingto
@ Kidney nodule(67%)
r/ renalcalculi r/ cavitation(50%),esp. in staphylococcalemboli
@ Scrotum(0.5%) r/ air bronchogram withinpulmonarynodule(28/.)
r/ hyperechoiclesionsof epididymis+ testis Cx: empyema(39%)
@ Skin disease(10-30%)
. erythemanodosum= multiplebilateraltender S ID E R OS IS
erythematousnodulesmostlyon anterioraspectof = inertironoxide/ metallicirondeposits
lower extremities: Path: iron phagocytosedby macrophagesin alveoli/
. often associatedwith fever + arthralgia respiratorybronchioles, eliminationfrom lungby
r/ nilarlymphnodeenlargement lymphaticcirculation
. lupuspernio= induratedbluishpurpleelevations Occupational exposure:
mainlyon nose + digits electricarc welding,oxyacetylenetorchworkers(iron
. skin plaques/ scars oxidein fumes),mining+ processingof ironores,
@ Peripherallymphnodeenlargement(30%) cutting/ burningof iron+ steel,foundryworkers,
@ Muscle(25%): myopathy grinders,fettlers,silverpolishers(jewelryindustry)
@ Myocardium(6-25%): ventriculararrhythmia,heart ./ diffusefine reticulonodular opacities(maydisappear
block,cardiomyopathy, congestivefailure,angina, after exposurediscontinued)
ventricularaneurysm { smallroundopacities(indistinguishable from silica/ coal)
@ Bone (6-15-20%): ^/ tttOsecondaryfibrosis+ NO hilar adenopathy(unless
{ denselyscleroticlesionsin spine,pelvis,ribs mixeddust inhalation as in siderosilicosis/
r/ lesionsof distal+ middlephalangesof hand + foot: silicosiderosis= mixed-dust pneumoconiosis)
. pain and swelling H R C T:
./ tyticlesionwith laceliketrabecularpattern ./ widespreadpoorlydefinedcentrilobular micronodules
{ sharplymarginatedcystlikearea of rarefaction r/ branchinglinearstructures
@ Eyes (5-25%): uveitis,photophobia, blurredvision, { extensiveground-glass attenuation withoutzonal
glaucoma(rare) predominance
@ CNS (9%): hypothalamus, basalgranulomatous DDx: silicosis(nodularopacitiesmoredense+ profuse)
meningitis, facialnervepalsy
@ Salivarygland (a%): bilateralparotidenlargement
SILICOSIS
= inhalationof silicondioxide;most prevalentsilicosisof
progressivenatureafterterminationof exposure;similar
SEPTIC PULMONARY EMBOLI to CWP (becauseof silicacomponentin CWP)
= lodgmentof an infectedthrombusin a pulmonaryartery Substance; crystallinesilica (quartz);one of the most
Organism; S.aureus,Streptococcus widespreadelementson earth
 ChestDisorders 525

Occupational expo sure: r/ multiplesubpleural

nodules
t unneling,m in i n gq, u a rry i n gs,to n ec u tti n gp, o l ishi ng, r/ "pseudoplaques"= aggregate
of subpleural
nodules
glassmanufacturing, foundrywork,sandblasting, r/ tractionbronchiectasiJ
pottery,bricklining,boilerscaling,vitreousenameling, r/ honeycombing
ceramicindustry
Dust deposition: dependenton ComplicatedSilicosis
(a) airflow: depositionof 1-S-pmparticles = PROGRESSIVE MASSIVE FIBROSIS
predominantly aroundrespiratory bronchioles in a = appearanceof largeopacities>1 cm in diameter
centrilobular locationwithinsecondarypulmonary Location: midzonei peripheryof upperlung migrating
lobule toward hila
(b) lymphaticclearance: relatedto pulmonaryarterial Distribution: oftenbilateralsymmetric+ nonsegmental
pressure(gravity-related verticalgradient)+ blood r/ conglomeratesausage-shapedmasseswith ill-
flow (higherbloodflowthroughLUL) + passive definedmargins(in advancedstages)
milkingof lymphaticsby respiratorymotion(lateral> ^/ compensatoryemphysemain unaffectedportion
anterior> posteriorchestwall) betweenmass + pleura
Path: small particlesengulfedby macrophages; r/ slow changeover years
liberationof silicaresultsin celldeath;2-3_mm { may calcify+ cavitate(ischemicnecrosis)
noduleswith layersof laminatedconnectivetissue
aroundsmallervessels Silicotuberculosis
Cx: predisposesto tuberculosis Doubtfulsynergisticrelationship betweensilicosis
DDx: coal workerpneumoconiosis(identicalradiographs) + tuberculosis
^/ titttechangeover yearswith intermittently
positive
AcuteSilicosis sputa
= SILICOPROTEINOSIS
= heavyexposureto respirablefree silicain enclosed CaplanSyndrome
spacewith minimal/no protectionof airways Morecommonin coalworker'spneumoconiosis
Histo: proliferationof type ll pneumatocytes
+ profusesurfactantproduction STAPHYLOCOCCAL PNEUMONIA
Exposuretime: as shorl as 6-g months Mostcommoncauseof bronchopneumonia
Associatedwith: increasedrisk to develop (a) commonnosocomialinfection(patientson antibiotic
autoimmunedisease drugsmostsusceptible)
Dis t r ibut ion:lu n g p e ri p h e ry (b) accountsfor 5.h of community-acquired pneumonias
Distribution: predominantly lowerlungzones;bilateral (esp.in infants+ elderly)
{ diffuseairspace/ ground-glassdisease 0 Secondaryinvaderto influenza(commonestcauseof
r/ air bronchograms deathduringinfluenzaepidemics)
HRCT : Organism; Staphylococcusaureus=Gram-positive,
{ patchyground-glassopacities appearsin clusters,coagulase-producing
r/ fine intralobularreticulations(= ,crazy paving") ,
! rapidspreadthroughlungs
Cx: infectionwith TB + atypicalmycobacteria ./ empyema(esp.in children)
Pragnosis.' often rapidly progressivewith death from r/ pneumothorax,pyopneumothorax
respiratoryfailure r/ abscessformation
DDx: alveolarproteinosis r/ bronchopleural fistula
A. inCHILDREN:
Chr onic S im ple Si l i c o s i s r/ rapidlydevelopinglobar/ multilobarconsolidation
At least 10-20 years of dust exposurebefore r/ pleuraleffusion(90%)
appearanceof roentgenograph ic abnormality { pneumatocele(40-GO%)
Location: upper+ posteriorlungzones B . i n A D U LTS :
r/ small2-5 (range,1-1O)-mmroundedopacities r/ patchyoftenconfluentbronchopneumonia of
! may calcifycentrallyin 5-10% (rathertypicalfor segmentaldistribution, bilateralin >60%
silicosis) r/ segmentalcollapse(airbronchograms absent)
r/ hilar+ mediastinal lymphadenopathy, may calcifyin r/ late developmentof thick-walled-lung abscess
5"/" ("eggshellpattern") (25-75%)
r/ + reticulonodular pattern r/ pleuraleffusioni empyema(50%)(DDxfrom other
H RCT : pneumoni as)
d nodulesof 3-10 mm in size Cx: meningitis,metastaticabscessto brain/ kidneys,
r/ thickenedintra-and interiobular lines acute endocarditis
r/ subpleuralcurvilinearlines(peribronchiolar fibrosis)
r/ ground-glasspattern= mild ihickeningof afveolar STREPTOCOCCAL
PNEUMONIA
wall + interlobular septa(fibrosis/ edema) lncidence: 1-5"/oof bacterialpneumonias(rarelyseen);
r/ parenchymal fibrous'bands mostcommonin wintermonths
526 Radiology Review Manual

Organism; GroupA B-hemolyticstreptococcus DDx: pulmonaryarteryatresia(uncommonin adults),

= Streptococcuspyogenes,Gram-positive localizedbullousemphysema(deviationof
cocciaPPearing in chains vessels),bronchialobstruction
Predisposed: newborns,followinginfectionwith measles
Associatedwith: delayedonset of diaphragmatichernia LUPUSERYTHEMATOSUS
SYSTEMIC
(in newborns) = sLE = ffiostprevalentof the potentiallygrave connective
. rarelVfollowstonsillitis+ pharyngitis tissuediseasescharacterizedby involvementof
./ patcrrybronchopneumonia vascularsystem,skin,serous+ synovialmembranes
Prevalence: 1:2,000;Blacks:Caucasians = 3:1;
r/ lower lobe predominance(similarto staphylococcus)
{ empyema increasedrisk in relatives
Cx: (1) Residualpleuralthickening(15%) Cause: localdepositionof antigen-antibody complexes/
(2) Bronchiectasis antibodiesinducingnecrotizing vasculitis(type
(3) Lung abscess lll immunecomplexphenomenon) of the small
(4) GlomerulonePhritis bloodvessels
Age: 16-41 years; M:F = 1:10 (womenof childbearing
SYNDROME
SWYER.JAMES age)
= MACLEOD SYNDROME = UNILATERAL LOBAREMPHYSEMA Diagnostic criteria:
= IDIOPATHIC UNILATERAL HYPERLUCENT LUNG 1) malarrash
= chroniccomplicationof bronchiolitis 2) discoidrash
Etiotogy: acuteviral bronchiolitisin infancyI early 3) photosensitivitY
childhood(adenovirus, RSV)preventing 4) oral ulcers
normaldeveloPment of lung 5) arthritis
Path: variantof postinfectious constrictivebronchiolitis 6) serositis
bronchiectasis,
bronchiolitis,
with acuteobliterative 7) antinuclearantibodies
distalairspacedestruction(developingin 7-30 8) renaldisease
months) 9) neurologicdisease
. asymptomatic (10) hematologic disease
. cough,dyspneaon exertion,hemoptysis (11) i mmunol ogidi c sorder
. historyof recurrentlower respiratorytract infections . fatigue,malaise,anorexia,fever,weightloss
duringchildhood . clinicallyheterogeneous due to differenttypesof serum
Location: one / both lungs(usuallyentirelung, antibodies
lobar/ subsegmental)
occasionally . antinuclearDNA antibodies(87%)
r1 hyperlucencyof one lung . hypergammaglobulinemia (77%)
./ diminishednumber+ size of pulmonaryvessels: . LE cells(= antigen-antibody complexesengulfedby
! smallipsilateral hilum(diminutedhilarvessels P MN s) i n78' /"
+ attenuatedarteries) . chronicfalse-positiveWassermanntest for syphilis
{ smallhemithoraxwith decreased/ normalvolume (24%)
(collateralair drift) . Sjogrensyndrome(frequent)
r/ air trappingduringexpiration . anemia(78%)
DDx: no air trappingwith proximalinterruptionof . leukopenia(66%)
pulmonaryartery(no hilum),hypogenetic lung . thrombocytoPenia (19%)
syndrome,pulmonarYembolus @ Skin changes (81%)
r/ mild cylindricalbronchiectasis with paucityof bronchial ' "butterflyrash"(= facialerythema),discoidlupus
"pruned
subdivisions(cutoffat 4th-5th generation- erythematosus,alopecia,photosensitivity
tree" bronchogram) . RaynaudPhenomenon (15%)
HRCT(mostusefulmodalitY): @ Thoracic involvement (30-70%)
./ bilateralareasof decreasedattenuation: 0 Affectsrespiratorysystemmore commonlythan any
{ areas of normallung attenuationwithin otherconnectivetissuedisease
hypoattenuatinglung . dyspnea,pleuriticchestpain (35%)
{ air trappingwithinhypoattenuating lung . respiratorydysfunction(>50%): single-breath
ri bronchiectasis diffusingcapacityfor carbonmonoxidemost
./ diminishedsize of pulmonaryvesselsin hyperlucent sensitiveindicator
areas (a) Pulmonarydisease
A ngio: Cause: chronicantibodydamageto alveolar-
{ "prunedtree" aPPearance capillarYmembrane
NUC (V/Qscan): { parenchymaloPacification:
{ matcheddefectsof perfusion+ ventilation(with r/ pneumonia(mostcommon)due to bacteria/
delayedwashout)in hyperlucentregions oPPortunistic organism
Bronchography: r/ lung hemorrhage
r/ ditatedbronchiwith sharplyterminatingsegments r/ pulmonaryedema
 ChestDisorders 527

r/ lupuspneumonitis(acuteform) = poorly r/ pneumatosisintestinafis, perforation

definedpatchyareas of increaseddensity r/ painfulascites
peripherallyat lung bases (alveolarpattern) r/ hepatomegaly, hepatitis,cirrhosis
secondaryto infection/ uremiain 10% ri splenomegaly
r/ cavitatingnodules(vasculitis) Prognosis; 60-90% 1O-yearsurvival;death from renal
{ pulmonaryfibrosis(OO%): failure/ sepsis/ CNS involvement
/
r/ interstitialreticulations
in lung peripheryof myocardialinfarction
lowerlungfields(chronicform)in B/"
{ fleetingplatelikeatelectasisin both bases Drug-induced
LupusErythematosus
(? infarctiondue to vasculitis)
(DtL)
' temporaryphenomenon
r/ progressiveloss of lung volume: Agents: procainamide,hydralazine,isoniazid,
r/ elevatedsluggishdiaphragms(dueto phenytoinaccountfor 90%
,
, diaphragmatic dysfunction) ri pufmonary+ pleuraldiseasemore commonthan in
./ hilar+ mediastinal lymphadenopathy (extremely S LE
rare)
(b) Pleuraldisease(50%)
TALCOSIS
r/ recurrentuni-/ bilateralpleuraleffusions(70%) = prolongedinhalationof magnesiumsilicatedust
, from pleuritis containingamphibolefibers (tremoliteand anthophyllite)
r/ pleuralthickening
and si l i ca
(c) Cardiovascular disease Talcosisresembles:
r/ pericardialeffusion(frompericarditis)
( 1) Asbestosis(indistinguishabte)
r/ cardiomegaly(primarylupuscardiomyopathy)
r/ massiveand bizarrepleuralplaques
@ Joints ^/ may encase lungwith calcification
. arthralgia(95%)
(2) Silicosis
r/ nonerosivearthritisof hands (characteristic) without r/ small rounded+ largeopacities
deformity ^/ fibrogenicprocessltrtOregressionafter removalof
r/ tumoralcalcinosis
patientfrom exposure)
@ Kidney
Prevalence; kidneysinvolvedin 100%with renal
diseasedevelopingin 30-50%
TERATOID
TUMOROF MEDIASTINUM
= MEDTASTTNAL GERMCELLTUMOR[= TERATOMA]
Histo: focal membranoproliferative
0 The anteriormediastinum is the mostcommon
glomerulonephritis
. renaffaifure(fibrinoidthickeningof basement extragonadal site of primarygerm celltumors(1-3% of
al l germcel ltumors)l
membrane)
r/ aneurysmsin interlobular + arcuatearteries(similar
; .mispIac;n{
Pathosenesis r,:iltff.::'J*
to but less frequentthan polyarteritisnodosa) ilil,.':!ffi
Tn
^/ normal/ decreasedrenalsize to gonad
./ hydronephrosis(due to detrusormusclespasmwith Incidence:
- adults: 15./"of anteriormediastinal tumors
vesicoureteralreflux/ fibrosisof ureterovesical - children: 24./.of anteriormediastinal
junction) tumors
0 16-28% of all mediastinalcystsl
US:
0 Occursin same frequencyas the usuallylarger
r/ t<idneyenlarged(early)/ diminutive(latestage)
thymomal
r/ increasedparenchymalechogenicity
0 1/3 of primaryneoplasmsin this area are in children
CT:
r/ multiplelinearhypoattenuating Classes:(1) Matureteratoma(solid)
bands (due to (2) Cysticteratoma(dermoidcyst)
vasculitis)
(3) lmmatureteratoma
Cx: (1) Nephroticsyndrome(common)
(4) Malignantteratoma(teratocarcinoma)
(2) Renalvein thrombosis(in 33%)
(5) Mixedteratoma
Prognosis; end-stagerenaldiseaseis commoncause
Location: mediastinum is 3rd mostcommonsite for
of death
teratoidlesions(aftergonadal+
@ Ct tract(in up to 50%)
. buccalerosions/ ulcerations sacrococcygeallocation); 5/" of all teratomas
. Gl tract bleeding occur in mediastinum,mostlyanterosuperiorly
r/ mesentericischemia:colitis,pseudoobstruction, (in only 1% posteriorly)
r/ ofteninseparable fromthymusgtanO
, ileus,thumbprinting, luminafnarrowing
{ motilitydisorderof loweresophagus(similarto
A. BENtcN TERATOTD TUMOR(75_86%)
scleroderma) = MATURETERATOMA
esophagitis + ulcers
= rTloSt
commonhistologictype
gastritis
1. Epidermoid(52%)= ectodermalderivatives
nodularityof folds
2. Dermoid(27%)= €cto-+ mesodermalderivatives
528 Radiology Review Manual

3. Teratoma(21o/")= €cto- + meso- + endodermal . asymptomatic(20-30%)

derivatives . chest pain lpressure,shortnessof breath,weight
Path: sphericallobulatedwell-encapsulated tumor; loss,hoarseness, dysPhagia, fever
typicallymulti-i unilocularcysticcavitieswith . SVC obstruction(10%)
clear/ yellow/ brownliquid . elevatedserumlevelsof hCG (7-18%)
Histo: . elevatedserum levelsof LDH (80%)correlate
(a) ectoderm: skin, sebaceousmaterial,hair,cysts withtumorburden+ rateof tumorgrowth
linedby squamousepithelium Metastases: to regionallymphnodes,lung,bone,
(b) mesoderm:bone,cartilage,muscle l i ver
(c) endoderm:Gl + respiratory tissue,mucusglands ^/ largebulkywell-marginated lobulatedmass
0 Tumorcapsulecommonlyhas remnantsof thymic r/ usuallyNO calcification
t is s ue! ./ homogeneous soft-tissue densitywith slight
0 Cystformationis typical(usuallylinedby mucus- enhancement
secretingtall epithelialcells)! Prognosis: 75-100% S-yearsurvival rate; death
Age: youngadults/ children;M = F f rom distantmetastases
. asymptomatic(in up to 53%) Rx: surgery+ radiationtherapy(very
. cough,dyspnea,chestpain,pulmonaryinfection, radiosensitive) t cisPlatin
respiratorydistress(due to compressionby large 2. Nonseminomatousmalignantgerm cell tumor
tumor) (a) embryonictissue
Location: (1) Embryonalcarcinoma
(a) anteriorsuperiormediastinum nearthymus/ (b) extraembryonic tissue
withinthymicparenchyma (1) Y ol ksac = endodermal si nustum or
(b) posteriormediastinum(rare= 3-8%) (2) Choriocarcinoma (leastfrequent)
r/ roundedmassbulginginto righti left hemithorax (c) combination = mixedgerm celltumor
sharplydemarcatedagainstadjacentlung Path: large unencapsulatedheterogeneoussoft-
{ variationsin density(mayall be present): tissue mass with tendencyfor invasionof
r/ tat-ttuidlevel(rarebut SPECIFIC) adjacentstructures
{ water density Age: during2nd-4thdecadeM:F = 9:1; in children
r/ homogeneoussoft-tissuedensity M= F
(indistinguishable from lymphoma/ thymoma) Associatedwith: Klinefeltersyndrome(in 2O/o),
r/ curvilinearperipheral/ centralcalcification hematologic malignancy
(2043/0,4 x morecommonin benignlesions)in . chestpain,dyspnea,cough,weightloss,fever,
tumorwall / substance,ossification in maturebone SVC syndrome(90-100%)
r/ visualization of tooth (PATHOGNOMONIC) . elevatedserum levelof o-fetoprotein(80%)with
{ often inseparablefrom thymicgland endodermalsinustumor/ embryonalcarcinoma
r/ enhancementof rim / tissuesepta . elevatedserum levelof LDH (60%)
Prognosis.' approx.100% S-yearsurvivalrate . elevatedserumlevelof hCG (30%)[DDx: lung
Rx: completesurgicalexcision cancer;hepatocellular carcinoma;
adenocarcinoma of pancreas,colon,stomachl
B. MALIGNANTTERATOIDTUMOR(14-20%) Metastasesto: lung, liver
Histo: similarto matureteratomabut with primitive/ rl largetumor of heterogeneoustexturewith central
immaturetissueelements;commonlyneural hemorrhage / necrosis
tissuearrangedin rosettes/ primitivetubules { well circumscribed i with irregularmargins
0 Teratocarcinoma / malignantteratoma= identicalto r/ enhancementof tumorperiphery
teratoma with componentsof seminoma, r/ lobulationsuggestsmalignancy
endodermalsinustumor,embryonalcarcinoma, r/ invasionof mediastinalstructures(SVC
choriocarcinoma, sarcoma,carcinoma obstructionis ominous)
1. Seminoma= germinorno= dysgerminoma r/ pleural/ pericardial effusion(fromlocalinvasion)
0 2nd mostcommonmediastinal germ celltumorl 0 Absenceof primarytesticulartumori
0 Mostcommonprimarymalignantgerm cell tumor retroperitoneal mass provesprimary!
of mediastinuml Rx: cisplatin-based chemotherapy+ tumor
lncidence: 2-6/" of all mediastinaltumors;5-13'/" resection
of all malignantmediastinal
tumors Prognosis: 50/"long-termsurvivors
Age: 3rd-4th decade;M >> F; white Cx:
Histo: uniformpolyhedral/ roundcells arranged (1) H emorrhage
in sheetsor formingsmalllobules (2) Pneumothorax (frombronchialobstruction with air
separatedby fibroussepta;varying trapping+ alveolarrupture)
amountsof maturelymphocytes (3) Respiratorydistress(rapidincreasein size from fluid
Path: large unencapsulated well-circumscribed production)with compressionof trachea/ SVC (SVC
mass syndrome)
 ChestDisorders 529

(4) Fistulaformationto aorta,SVC, esophagus (3) Neoplasticcyst (cysticteratoma,cysticdegeneration

(5) Huptureinto bronchus(expectoration of oily withina thymoma),S/P radiationtherapyfor Hodgkin
substance/ trichoptysisin S-14/",lipoidpneumonia) disease
(6) Ruptureintopericardium (pericardialeffusion), 0 No associationwith myastheniagravis/ neoplasial
pleuralcavity(pleuraleffusion) lncidencej very uncommonlesion;1-2% of mediastinal
DDx: thymoma MASSES
Age: 213in 1st decade;1/3 in 2nd + 3rd decades;M>F
Path: unilocularthin-walledcystwith thymictissue
THORACIC
PARAGANG
LIOMA Histo: squamous/ cuboidal/ respiratoryepitheliumin
= CHEMODECTOMA cystwall;lobulatedlymphoidtissuein cystwall
= f€lreneuraltumorarisingfrom paraganglionic tissue containingHassallcorpuscles; cholesterol
Age: 3rd-Sthdecade;M:F = 1:1 crystals;smallfoci of thyroidi parathyroidtissue
Path: extremelyvascularwell-marginated / irregular . commonlyasymptomatic slowlyenlargingpainless
mass that may adhereto / envelop/ invade MASS
adjacentmediastinal structures(bronchus,spinal . hoarseness,dysphagia,stridor,respiratorydistressin
canal) newborns
Histo: anastomosingcords of granule-storing chief cells . suddensymptomatic enlargement with Valsalva
arrangedin a trabecularpattern;identical maneuver/ hemorrhagelrecentviralinfection
appearancefor benignand malignanttumors Location:
May be associated with: (a) adjacentto carotidsheathfrom angle of mandibleto
syn-I metachronousadrenal/ extrathoracic thoracicinlet (alongpath of thymopharyngeal duct)
paragangliomas; multipleendocrineneoplasiatype 2; parallelto sternocleidomastoid muscle;L > R
bronchialcarcinoidtumor (b) anteriormediastinum
. asymptomatic r/ unilocularcystwiththin wallscontainingclearfluid/
. dyspnea,cough,chestpain,hemoptysis, neurologic multilocular cystwith thickwallscontainingturbidfluidor
deficits,SVC syndrome(if tumor large) gelatinousmaterial
. signs of excessivecatecholamineproduction:
r/ directextension/ fibrouscord along migratorytract of
hypertension,headache,tachycardia,palpitations, tremor thymictissueintomediastinum in 50%:through
Location: base of heart+ greatvessels(adjacentto thyrohyoid membrane into pyriform
, sinus
pericardium/ heart,withininteratrialseptum/
left atrialwall);paravertebralsulci 1 r"y show partiafwall cafcification(rare)
r/ low-density fluid(0-10 HU),may be higherdepending
CT: on cyst contents
r/ sharplymarginated5-7-cm middle/ posterior US:
mediastinalmass r/ typicallyanechoic
{ hypodenseareas due to extensivecystic DDx: branchialcleft cysts (no thymictissue),benign
degeneration / hemorrhage thymoma,teratoma,dermoidcyst,Hodgkin
r/ exuberantenhancement disease,non-Hodgkin lymphoma,pleuralfibroma
MR:
./ heterogeneousintermediatesignalintensitywith
THYMIC HYPERPLASIA
areas of signalvoid from ffowingbloodon Tl Wl 0 Mostcommonanteriormediastinal
'V nighsignaiintensity mass in pediatric
on T2Wl age groupthroughpuberty
NUC (l-123/ l-131metaiodobenzytguanidine):
Age: particularlyin young individual
r/ usefulfor localizationas retativelyspecific Histo: numerousactivelymphoidgerminalcenters
Angio(may precipitate cardiovascular crisis): Etiology:
r/ markedhypervascularity, multiplefeedingvessels 1. Hyperthyroidism (mostcommon),Gravesdisease,
r/ homogeneous capillaryblush treatmentof primaryhypothyroidism, idiopathic
Rx: surgicalexcisionwith preoperativeadministration of thyromegaly
a- or B-blockers(hypertensivecrisis,tachycardia, 2. Reboundhyperplasia in childrenrecoveringfrom
dysrhythmiaduringmanipulation) severeillness(eg, from burns),aftertreatmentfor
Cushingdisorder,afterchemotherapy
r/ thymusmay regrowmore than 50% (transient
THYMICCYST overgrowth,reduciblewith steroids)
Pathagenesis: 3. Myastheniagravis(65%)
(1) Congenitalcyst (persistenttubularremnantsof 3rd 4. Acromegaly
pharyngealpouch = thymopharyngeal duct, develops 5. Addisondisease
duringsth-Bth week of gestation) r/ normalthymusvisiblein S0%of neonates0-2 yearsof
(2) Acquiredreactivemultilocularcysts = progressive age
cysticdegenerationof thymic (Hassall)corpuscles r/ notch sign = indentationat junctionof thymus+ heart
+ thymicepithelialreticuluminducedby an { sail sign = triangulardensityextendingfiom superior
inflammatoryprocess:eg, HIV mediastinum
530 Radiology Review Manual
^/ wave sign = rippledborderdue to indentation
by ribs
r/ shape changeswith respiration+ position
THYMOLIPOMA
Incidence: 2-9"/" of thymic tumors
Age: 3-60 years(meanage of 22years);M:F = 1:1
Path: lobulatedpliableencapsulated tumorcapableof
growingto largesize (in 68% >500g, in 20/o
>2,000g, the largest>16 kg)
Histo: benignadult adiposetissue interspersedwith
areasof normal/ hyperplastic/ atrophicthymus
tissue(thymictissue<33o/"of tumormass)
. chestpain,dyspnea,cough(in 50%)
r/ largelesionsslumpinferiorlyfrom anteriormediastinum
towarddiaphragm
^/ may drapearoundheartenlargingcardiacsilhouetteon
frontalview
r/ apparentelevationof diaphragmon lateralview
^/ NO compression/ invasionof adjacentstructures
DDx: mediastinal lipoma(mostcommonof intrathoracic
fattytumors),liposarcoma
THYMOMA
0 Mostcommonprimaryneoplasmof anteriorsuperior
mediastinum
Age: majority>40 years; 70% occur in 5th-6th decade;
lessfrequentin youngadults,rare in children;
M : F= 1 : 1
Associatedwith: parathymicsyndromes(40%)such as
' Myasthenia gravis:
= autoimmunedisordercharacterized by antibodies
againstacetylcholinereceptorsof the postjunctional
musclemembrane:
. progressive weakness,fatigue
. fatigabilityof skeletalmusclesinnervatedby
cranialnerves,eg, ptosis,diplopia,dysphagia,
dysarthria, drooling,difficultywith chewing
. elevatedserum levelof anti-acetylcholine
receptorantibodies
0 10-15-25/" of patientswith myastheniagravis
havea thymoma(in 65% due to thymichyperplasia)
0 7-30-54o/"ol patientswith thymomahave
myastheniagravis;removalof thymictumoroften
resultsin symptomaticimprovement; myasthenia
gravismay developaftersurgicalthymomaexcision
Rx : edr o p h o n i u mc h l o ri d e
. Pure red cell aplasia= €Ifegel'rerativeanemia
= almosttotal absenceof marrowerythroblasts
+ blood reticulocytesresultingin severe
normochromic normocyticanemia
0 50% of patientswith red cell aplasiahavethymoma
0 5% of patientswith thymomadevelopred cell aplasia
. Acquiredhypogammaglobulinemia
0 10% of patientswith hypogammaglobulinemia have
thymoma
0 6% of patientswith thymomahave
hypogammaglobulinem ia
. Paraneoplastic syndromesoccurwiththymiccarcinoid
(10%): eg, Cushingsyndrome(ACTHproduction)
. chestpain,dyspnea,cough(33%)
Path:
round/ ovoidslow-growing primaryepithelialneoplasm
with smooth/ lobulatedsurfacedividedintolobulesby
fibroussepta;areas of hemorrhage+ necrosismay form
cysts
(a) encapsulated = thickfibrouscapsule+ calcifications
(b) locallyinvasive= microscopic foci outsidecapsule
(c) metastasizing= benigncytologicappearancewith
pleural+ pulmonaryparenchymal seeding
(d) thymiccarcinoma
Histo:
(a) biphasicthymoma(mostcommon)
= epithelial+ lymphoidelementsin equalamounts
(b) predominantly lymphocytic thymoma
-- >213of cells are lymphocytic
(c) predominantly epithelialthymoma
= >213of cellsare epithelial
0 Prognosisunrelatedto celltype!
. asymptomatic(50% discoveredincidentally)
. signs of mediastinalcompression(25-30%):
cough,dyspnea,chestpain,respiratory infection,
hoarseness(recurrentlaryngealn.),dysphagia
. signsof tumorinvasion(rare): SVC syndrome
Location: any anteriormediastinal locationbetween
thoracicinletand cardiophrenic angle;rare in
neck,othermediastinal compartments, lung
parenchyma, or tracheobronchial tree
S i z e : 1 - 1 0 c m ( u pt o 3 4 c m )
Noninvasive [Benign] Thymoma
Age peak; Sth-Othdecade,almostall are >25 years
of age
ri oval / round lobulatedsharplydemarcated
asymmetrichomogeneous massof softtissuedensity
(equalto muscle),usuallyon one side of the midline
{ abnormallywide mediastinum
^i displacement of heart+ greatvesselsposteriorly
C T:
r/ homogeneoussoft-tissuemass with smooth/
lobulatedborderpartially/ completelyoutlinedby fat
^/ homogeneous enhancement
^/ areasof decreasedattenuation(fibrosis,cysts,
hemorrhage, necrosis)
^/ amorphous,flocculentcentral/ curvilinear
peripheral calcification (5-25%)
MRI:
r/ isointenseto skeletalmuscleon Tl Wl
ri increasedsignalintensity(approachingthat of fat)
on T2Wl
.i ttuiOcharacteristics of cystswith high water content
Invasive[Malignant]Thymoma
0 Malignancydefinedaccordingto extentof invasion
into adjacentmediastinalfat + fascia!
Frequency: in 30-35o/"of thymomas
Stage I : intactcapsule
Stage ll : pericapsulargrowthinto mediastinalfat
Stage lll : invasionof surrounding organssuch as
lung,pericardium, SVC,aorta
 ChestDisorders 531

Stage lVa: disseminationwithinthoraciccavity lncidence: 0.5-1.5%

(metastasesto pleura+ lung in 6%) Age: discoveredin 3rd-Sthdecade
Stage lVb: distantmetastases(liver,bone,lymph . coughwith copioussputum

r/hete )
ros".i3l3?,[1H{,:,0'"''
r/ spreadby contiguityalong pleuralreflections,
extensionalong aorta reachingposteriormediastinum
/ crus of diaphragm/ retroperitoneum
, (transdiaphragmatic tumor extension)
! irregularinterfacewith lung
r/ unilateraldiffusenodularpleuralthickening/ pleural
massesencasinglung circumferentially
r/ vascularencroaChment
r/ pleuraleffusionUNCOMMON
DDx: malignantmesothelioma, lymphoma,thymic
carcinoma/ malignantgerm celltumor(older
male,no diffusepleuralseeding),peripherallung
carcinoma(no dominantmediastinalmass),
metastaticdisease(not unilateral)
Rx: radicalexcision+ adjuvantradiationtherapy
Prognosis; S-yearsurvivalof g3% for stage l, g6% for
stage 11,70/"for stagelll, 50% for stagelV;
2-12% rate of recurrencefor resected
encapsulatedthymomas
TORSION
OF LUNG
= tdta complicationof severechesttrauma
lncidencej rare (<30 cases)
Age: almostinvariablyin children
Cause: compressionof lowerthorax,tear on inferior
pulmonaryligament,completeness of fissures
Mechanism: compressionof lowerthoraxwith lung
twistedthrough180.; usuallyin presence
of a largeamountof pleural air lfluid
Associated with:
surgery(lobectomy),trauma,diaphragmatichernia,
pneumonia,pneumothorax, bronchus-obstructing tumor
Histo: + hemorrhagicinfarction+ excessiveair trapping
r/ collapsed/ consolidatedlobe in unusualposition
+ configuration:
r/ hilardisplacementof atelectatic-appearing lobe in an
inappropriatedirection
r/ changein positionof opacifiedlobe on sequential
radiographs
r/ alterationin normalcourseof pulmonaryvasculature:
r/ main lower lobe arterysweepingupwardtowardapex
{ rapidopacificationof an ipsilateratlobe from edema
+ hemorrhageinto airspacessecondaryto infarction
(DDx: pleuraleffusion)
r/ bronchialcutoff/ distortion
r/ lobarair trapping
{ lowerlungvesselsdiminutive
. shortnessof breathon exertion
. long historyof recurrentpneumonias
May be associatedwith: Ehlers-Danlossyndrome
r/ markeddilatationof trachea(>29 mm), rignt 1>eOmm;
+ l eft(> 15 mm) mai nstembronchi
r/ sacculatedoutline/ diverticulosisof tracheaon lateral
CXR (= protrusionof mucousmembranebetweenrings
, trachea)
of
{ may have emphysema,bullaein perihilarregion
TRACHEOBRONCHOPATHIA
OSTEOCHONDROPLASTICA
= f€lfebenigndiseasecharacterizedbymultiple
submucosalcartilaginous / osseousnodulesprojecting
intotracheobronchial lumen
Cause: unknown;may be due to chronicinflammation,
degenerativeprocess,irritationby oxygen/
chemical,metabolicdisturbance,amyloidosis,
tuberculosis, syphilis,heredity(highprevalence
i n Fi nl and)
Pathogenetic theo ries:
(1) Ecchondrosis / exostosisof cartilagerings
(2) cartilaginous / osseousmetaplasiaof internalelastic
fibrousmembraneof trachea
Path: foci of submucosafhyalinecartilagewith areas of
lamellarbone
Histo: adiposetissue+ calcifiedareaswith foci of bone
marrow;thinnednormaloverlyingmucosawith
inflammation + hemorrhage
Averageage: 50 years(11-78 years); M:F = 3:1
. usuallyasymptomatic (incidentally
diagnosed)
. dyspnea,productivecough,hoarseness,hemoptysis,
fever,recurrentpneumonia
Location: distal 213ol trachea,larynx,lobar/ segmental
bronchi,entirelengthof trachea;
sparesposteriormembraneof trachea
CXR:
r/ scalloped/ linearopacitiessurrounding+ narrowing
the trachea(beston lateralview)
CT:
r/ deformedthickenednarrowedtrachealwall
r/ irregularlyspaced1-3-mm calcificsubmucosal
nodulesof trachea+ bronchi(similarto plaques)
Dx: bronchoscopy
DDx: relapsingpolychondritis, tracheobronchial
amyloidosis(does not spare posteriormembranous
wall of trachea),sarcoidosis, papillomatosis,
tracheobronchomalacia

TRACHEOBRONCHOMEGALY TRAUMATIC
LUNGCYST
= M O U N I E R - K U HSNY N D R O M E
= primaryatrophy/ dysplasiaof supporting 4ge' children+ young adultsare particularlyprone
structures
of r/ thin-walledair-fitiedcivity FA|r,.4
r air-ftuidlevet
trachea+ majorbronchiwithabrupttransitionto normal precededby homogeneouswell-circumscribed mass
bronchiat 4th-5thdivision (hematoma)
532 RadiologyReviewManual

r/ oval / sphericallesionof 2-14 cm in diameter { "tree-in-bud"appearance= Sril?llpoorlydefined

r/ s ingle/ m ult ip l el e s i o n su; n i -o r mu l ti l o c u l a r nodules+ branchingcentrilobular
centrilobular
{ usuallysubpleuralunderpointof maximalinjury areasof increasedopacity(= severebronchiolar
r/ persistentup to 4 months+ progressivedecreasein impactionwith clubbingof distalbronchioles)
size (apparentwithin6 weeks) occurringat multiplecontiguousbranchingsites
{ bronchiectasis

TU B E R C U LOMA
TUBERCULOSIS = manifestationof primary/ postprimaryTB
Prevalence: 10 millionpeopleworldwide,activeTB roundi oval smoothsharplydefinedmass
developsin 5-1 0o/oof those exposed 0.5-4 cm in diameterremainingstablefor a longtime
Organism; Mycobacterium= acid-fastaerobic rods lobulatedmass (25%)
stainingred with carbol-fuchsin; M. { satellitelesions(80%)
tuberculosis(95%),atypicaltypes increasing: may calcify
M. avium-intracellulare, M. kansasii,M.
fortuitum CAVITARYTUBERCULOSIS
Susceptible: infants,pubertaladolescents,elderly, = hallmarkof reactivationtuberculosis
alcoholics,Blacks,diabetics,silicosis, = semisolidcaseousmaterialis expelledintobronchial
measles,AIDS (3040% infectedwith tree after lysis
HIV),sarcoidosis(in up to 13%) r/ moderatelythick-walledcavitywith smoothinner
At risk: immunocompromised, minorities,poor, surface
alcoholics, immigrantsfrom 3rd worldcountries, Cx:
prisoners,the aged,nursinghome residents, (1) Dissemination to otherbronchialsegments
homeless r/ multiplesmallacinarshadowsremotefrom
Pathologic phases: massiveconsolidation
(a) exudativereaction(initialreaction,presentfor (2) Colonization with Aspergillus
1 month) ^/ aspergilloma
(b) caseousnecrosis(after2-10 weeks with onset of
hypersensitivity)
(c) hyalinization = invasionof fibroblasts(granuloma
formationin 1-3 weeks)
(d) calcification/ ossification PrimaryPulmonaryTuberculosis
(e) chronicdestructive form in 1O/. (<1 year of age, Mode of infection; inhalationof infectedairborne
adolescents, youngadults) droplets
Spread: regionallymphnodes,hematogenous Age: most commonform in infants+ childhood;
dissemination, pleura,pericardium, upper increasinglyencounteredin adults(23-34% ot
lumbarvertebrae all adultcases)
Mortality: 1:100,000 . asymptomatic(91%)
. PositivePPD tuberculintest: 3 weeks after infection . symptomatic(5-10%)
. NegativePPD test: r/ one / more areas of homogeneousdense well-defined
1. Overwhelming tuberculousinfection(miliaryTB) airspaceconsolidation of 1-7 cm in diameterin
2. Sarcoidosis 2Y50-78% (requiresseveralweeks for complete
3. Corticosteroid therapy clearingwith antituberculous therapy):
4. Pregnancy r/ absentresponseto antibioticRx for "pneumonia"
5. Infectionwith atypicalMycobacterium Location: middlelobe,lowerlobes,anterior
FormerRx: plombagewith insertionof plasticpacks, segmentof uPPerlobes
eres
;oIeotho
rax r/ fine discretenodularareas of increasedopacity
:H;Lxi3'l';i;iTHffisph DDx: varicellapneumonia,histoplasmosis,
metastases, sarcoidosis,pneumoconiosis,
E NDO B RO NC H T AL(AC T N A RT)U B ER C U L O S IS hemosiderosis
0 Most commoncomplicationof tuberculouscavitation r/ in children:massivehilar(60%)/ paratracheal (4O%)
with activeorganismsspreadingvia airwaysfollowing / subcarinallymphadenopathy, in 80% on rightside;
caseousnecrosisof bronchialwall in adults: mediastinallymphadenopathy in 5-35-48%
Path: ulcerationof bronchialmucosafollowedby DDx of Lnn: metastases,histoplasmosis
fibrosisleadsto ri atelectasis(8-18%),esp. in rightlung (anterior
(a) bronchialstenosis(lobarconsolidation) segmentof upperlobe/ medialsegmentof middle
(b) bronchiectasis lobe)secondaryto
(c) acinarnodulesreflectingairwayspread (a) endobronchial tuberculosis
HRCT : (b) bronchial/ trachealcompressionby enlarged
r/ airspacenodules lymphnodes(68%)
 ChestDisorders 533

r/ pleuraleffusion(10%in childhood,23-3g% in Local Exudative Tuberculosis

adulthood)most commonly3-7 monthsafter initial { patchy/ confluentill-definedareas of acinar
exposure(fromsubpleuralfoci rupturinginto pleural consolidation (87-917o),commonlyinvolvingtwo /
space) moresegments(earliestfinding)
^/ pneumonicreaction(midor lowerlungzones)with ./ thin-walledcavitationwith smoothinnersurface
segmental/ lobarconsolidation (presentin more advanceddisease):
r/ calcifiedlung lesion(17%)/ parenchymalscar <5 mm r/ cavityundertension(air influx+ obstructed
= Ghon lesion
efflux)
r/ calcifiedlymphnode (36%)in hilus/ mediastinum r/ air-fluidlevelis strongevidencefor superimposed
{ Rankecomplex = Ghon lesion+ calcifiedlymph bacterial/ fungalinfection
node (22%) r/ air-crescentsign = mobileintracavitarymycetoma
r/ Simon focus = healedsite of primaryinfectionin lung r/ accentuateddrainagemarkingstoward ipsilateral
apex hilum
CT: r/ acinarnodularpattern(20%l due to bronchogenic
r/ tuberculousadenopathymay demonstratenecrotic spread
centerwith low attenuationafter enhancement r/ pleuraleffusion(18%)
Outcome of primary infection: CT:
1. lmmunitypreventsmultiplication of organism r/ micronodules in centrilobular
location(62%)
(containment of initialinfectionby delayed = solidcaseationmaterialin / surrounding the
hypersensitivityresponse+ granulomaformation terminal/ respiratory bronchioles
in 1-3 weeks) r/ interlobularseptalthickening(3a-Sa%)
2. Progressive primaryTB (inadequate immune = increasein lymphaticflow as inflammatory
mechanismwith localprogression)in 107o,most response/ impairedlymphaticdrainagedue to
commonin olderchildren/ teenagers hilarlymphadenopathy
3. Miliarytuberculosis (uncontrolled massive
hematogenous dissemination ovenruhelminghost
defensesystem) Local Fibroproductive Tuberculosrb
4. PostprimaryTB = reactiv€ltionTB (reactivationof
@ Parenchymal disease:
dormantorganismsafter asymptomaticyears)
{ sharplycircumscribed irregular+ angular
Prognosis: 3.6.h mortalityrate
masslikefibroticlesion(in up lo 7%)
Cx: (1) Bronchopleural fistula+ empyema ^/ thick-walledirregularcavitation(HALLMARK)
(2) Fibrosingmediastinitis
secondaryto expulsionof caseousnecrosisinto
ainruays,esp. in apical/ posteriorsegmentsof
upperlobes(rarein children,in up to 4S-51v"in
PostprimaryPulmonaryTuberculosis , adults),oftenmultiple(highlikelihoodof activity)
r/ reticularpulmonaryscars
= REACTIVATION TB = RECRUDESCENT TB r/ cicatrizationatelectasis= volumeloss in
= infectionunderthe influenceof acquired
affectedlobe
hypersensitivity and immunitysecondaryto longevity @ Airwayinvolvement:
of bacillus+ impairmentof cellularimmunity r/ bronchialstenosis::
lncidence: 1o/"per year in personswith normal r/ persistentsegmental/ lobarcollapse
immunity,up to 10/" in personswith r/ lobar hyperinflation
deficientT-cellimmunity { obstructivepneumonia
Age: predominantlyin adolescence+ adulthood r/ mucoidimpaction
Etiology: r/ tractionbronchiectasis in apical/ posterior
(a) reactivationof focus acquiredin childhood(90%) segmentsof upperlobes
(b) continuation of initialinfection @ Pleuralextension:
= pfogfesSive primarytuberculosis (rare) r/ pleuralthickening
(c) initialinfectionin individualvaccinatedwith BCG r/ apicalcap = pleuralrind = thickeningof layerol
Path: foci of caseousnecrosiswith surrounding extrapleural fat (3-25 mm) + pleuralthickening
edema,hemorrhage,mononuclearcell (1-3 mm)
infiltration;
formationof tubercles= occurnulation r/ air-fluidlevelin pleuralspace= bronchopleural
of epithelioid cells+ Langhansgiantcells; fistula
bronchialperforationleadsto intrabronchial r/ rim-enhancing/ calcifiedsoft-tissuemass of
dissemination (19-21%) chestwall with destructionof bone / costal
Site: 85% in apical+ posteriorsegmentsof upper [obe, cartilage
10% in superiorsegmentof lowerlobe,5% in r/ fistulizationto skin
mixed locations(anterior+ contiguoussegments
@ Lymphadenopathy:
of upperlobe);R > L (DDx: histoplasmosis tends { tuberculouslymphadenitis = enlargednodes
to affectanteriorsegment) with centralareas of low attenuation
534 Radiology Review Manual
{ calcifiedhilar lmediastinalnodes:
. broncholithiasis= erosioninto adjacentairway
./ Rasmussenaneurysm= aneurysmof terminal
branchesof pulmonaryarterywithinwall of TB
cavitysecondaryto inflammatorynecrosisof the
vesselwall (4% at autopsiesof cavitaryTB):
WEGENER
{ centralcavitynear hilum
r/ enlargementof centralsolidcomponentof cavity
{ opacificationof pseudoaneurysm on CT / angio
MiliaryPulmonaryTuberculosis
= mdsSivehematogenous dissemination of organisms
any time after primarYinfection
Cause:
(1) severeimmunodepression duringpostprimary
state of infection
(2) impaireddefensesduringprimaryinfection
= P RO G R ES SIV PE R IMA R YT B
lncidence: 2-3.5/" of TB infections
r/ chronicfocus often not identifiable
r/ radiographically recognizableafter6 weeks post
hematogenous dissemination
r/ generalized granulomatous smallfoci of
interstitial
pinpointto 2-3 mm size
{ rapidcompleteclearingwith appropriatetherapy
HRCT(earlierdetectionthan CXR):
r/ diffuselyscattereddiscrete1-2-mm nodules
Cx: disseminationvia bloodstreamaffectinglymph
nodes,liver,spleen,skeleton,kidneys,adrenals,
prostate,seminalvesicles,epididymis, fallopian
tubes,endometrium, meninges
UNILATERAL PULMONARY AG ENESIS
= ohe-sidedlack of primitivemesenchyme
Associated with:
anomaliesin 60% (higherif rightlung involved):PDA,
anomaliesof greatvessels,tetralogyof Fallot(left-sided
pulmonaryagenesis),bronchogenic cyst,congenital
diaphragmatic hernia,bone anomalies
. may be asymptomatic
. respiratoryinfections
./ completeopacityof hemithorax
r/ ipsilateralabsenceof pulmonaryartery+ vein
{ absentipsilateral mainstembronchus
r/ symmetricalchest cage with approximationof ribs
r/ overdistensionof contralaterallung
{ ipsilateralshiftof mediastinum+ diaphragm
PNEUMONIA
VARICELLA-ZOSTER
lncidence: 14/" overall;50% in hospitalizedadults
Age: >19 years (90%);3rd-Sthdecade (75%);contrasts
with low incidenceof varicellain this age group
. vesicularrash
{ patchydiffuseairspaceconsolidation
{ tendencyfor coalescencenear hila + lung bases
{ widespreadnodules(30%) representingscarring
widespreadthroughoutboth
./ tiny 2-3-mm calcifications
tungs(2%)
Cx: unilateraldiaphragmaticparalysis
Prognosis; 11% mortalitYrate
GRANULOMATOSIS
= probableautoimmunediseasecharacterized by
systemicnecrotizinggranulomatousdestructiveangitis
Path: peribronchial necrotizinggranulomas+ vasculitis
not intimatelyrelatedto arteries
Mean age of onset: 40 years (rangeof all ages);
M : F= 2 : 1
C LA S S IC TR IA D :
(1) respiratorytract granulomatousinflammation
(2) systemicsmall-vesselvasculitis
(3) necrotizing glomerulonephritis
. The mostcommonpresentingsymptomsare thoseof
upper respiratorytract involvement(in up to 67"/"):
. rhinitis,sinusitis,otitismedia
@ Pulmonarydisease(94%)
. stridor(fromtrachealinflammation+ sclerosis)
. intractablecough,occasionallywith hemoptysis
. fever,chest Pain,dYsPnea
Path: vasculitisof medium-sized and smallpulmonary
arteries+ veins+ capillaries, geographic
necrosis,granulomatous inflammation
./ bilateralinterstitialreticulonodular opacities,most
prominentat lungbases(earlieststage)
r/ widelydistributedirregularmasses/ nodulesof
varyingsizes(5 mm to 10 cm), especiallyin lower
lungfields(69%)usuallysparingapices:
{ usuallymultiplemasses,solitaryin up to 25%
r/ cavitationof noduleswith thick wall + irregular
shaggyinner lining(25-50%)
^/ bilateralmultifocalpatchyair-spaceopacities(in up
to 50%):
{ acuteairspacePneumonia
r/ intraalveolarpulmonaryhemorrhage
{ smooth/ nodularthickeningof subglottic/ tracheal/
bronchialwall producingstenosiswith oligemia
+ emphysema+ lobar/ segmentalatelectasis(60%)
{ pleuraleffusion(usuallyexudative)in 10-25-50%
r/ focal pteuralthickening
r/ nitar/ mediastinal lymphadenopathy (veryunusual)
r/ interstitialpulmonaryedema+ cardiomegaly(from
renalI cardiacinvolvement)
CT:
{ nodulesin peribronchovascular distribution:
r/ centralcavitationin nodules>2cm in diameter
r/ {eedingvesselsenteringnodules(= angiocentric
distribution)
r/ pleural-basedwedge-shapedlesions(= infarcts)
r/ Cf halo sign (= rim of ground-glassattenuation
surroundinga pulmonarylesion)due to
angiocentricparenchymalmicroinfarction
(nonsPecific)
r/ focal / elongatedsegmentsof tracheobronchial
stenosis+ intra-and extraluminalsoft-tissue
masses/ thickening
 ChestDisorders 535

Cx: (1) Dangerousainivaystenosis(15%of adults, M<F

50% of children) Prognosis; more favorablethan classicalWegener,s
(2) Massivelife-threatening pulmonary
hemorrhage Mi dl i ne Granul oma
(3) Spontaneous pneumothorax (rare) = mutilatinggranulomatous + neoplasticlesionslimited
@ Renaldisease(85%) to nose+ paranasalsinuseswith very poor prognosis;
focal glomerulonephritis in 20./"at presentation,as considereda variantof Wegenergranulomatosis
diseaseprogressesin 83% WITHOUTthe typicalgranulomatous + cellular
Histo: focal necrosis,crescentformation,paucity/ components
absenceof immunoglobulin deposits
@ Paranasalsinuses(91%)
Location: maxillaryantramostfrequently W I LLIA MS .C A MP B E LL S Y N D R OME
. s inuspain,p u ru l e nst i n u sd ra i n a g erh
, i n o rrhea = coflgenitalbronchialcartilagedeficiencyin the 4th-6th
r/ thickeningof mucousmembranesof paranasal
bronchialgenerationeitherdiffuseor restrictedto focal
s inus es
area
@ Nasopharynx(64%) H R C T:
. epistaxisfrom nasalmucosalulceration
r/ cysticbronchiectasis
distalto 3rd bronchialgeneration
. necrosisof nasalseptum
. saddlenose deformity r/ emphysematous lungdistalto bronchiectasG
r/ inspiratory
ballooning+ expiratorycollapseof dilated
^/ progressivedestructionof nasalcartilage+ bone
segments
, (DDx: relapsingpolychondritis)
r/ granulomatous massesfillingnasalcavities
@ Otherorganinvolvement: WILSON.MIKITY
(a) Joints (67%): migratorypolyarthropathy SYNDROME
= PULMONARY DYSMATURITY
(b) Ear (61%): otitismedia
= similarityto bronchopulmonary dysplasiain normal
(c) Eye (58%): ocularinflammation, proptosis preterminfantsbreathingroom air; rarelyencountered
(d) Skin + muscle(45%): inflammatory nodularskin
anymore
lesions,cutaneouspurpura
Predisposed: prematureinfants<1,500g who are
(e) Heart+ pericardium (12-28%):
i ni ti al l w
y el l
coronaryvasculitis,pancarditis, valvularlesions . gradualonsetof respiratory
Cx: acute pericarditis,dilatedcongestive distressbetween10 and 14
days
cardiomyopathy, acutevalvularinsufficiency
r/ hyperinflation
with pufmonaryedema, cardiacarrestdue to
r/ reticularpatternradiatingfrom both hila
ventriculararrhythmia, myocardialinfarction
(f) CNS (22%): centrali peripheralneuritis { smallbubblylucenciesthroughoutboth lungs(identical
(g) Splenicdisease to bronchopulmonary dysplasia)
Prognosis;resolutionover 12 months
( h) G l t r ac t( 10 % ):
. abdominalpain,diarrhea,bloodloss DDx: perinatally-acquired infection(especiallyCMV)
r/ ischemia,inflammation, ulceration, perforation
Cx: (1) Hypertension ZYGOMYCOSIS
(2) Uremia = PHYCOMYCOSIS
(3) Facialnerveparalysis = groupof severeopportunistic sinonasal+ pulmonary
Dx: (1) c-ANCA(cytoplasmic patternof antineutrophil diseasecausedby a varietyof Phycomycetes(soil
cytoplasmic autoantibodies): 96% sensitivefor fungi )
generalized disease,ggo/"specific Organism; ubiquitousMucor(mostcommon),Rhizopus,
(2) Lung lrenal biopsy Absidiawith broad nonseptatedhyphaeof
Prognosis; deathwithin2 years from renal (93%)I irregularbranchingpattern
respiratoryfailure;90-gS% mean S-year At risk: immunoincompetent host with
survivalundertreatment 1. lymphoproliferative malignanciesand
Rx: corticosteroids, cytotoxicdrugs (cyclophosphamide), leukemia
renal transplantation; 93% remissionwith therapy 2. acidoticdiabetesmellitus
DDx: Churg-Strauss(asthma, 47o/ocardiacinvolvement, 3. immunosuppression throughsteroids,
lesssevererenal+ sinusdisease,p-ANCA) antibioticsimmunosuppressive drugs(rare)
Entry: inhalationi aspirationfrom sinonasalcolonization
Path: angioinvasive behaviorsimilarto aspergillosis
A . R H ]N OC E R E B R AFOR
L M
LimitedWegenerGranulomatosis = involvement of paranasalsinuses(frontalsinus
= Wegenergranulomatosis
largelyconfinedto lung usuallyspared)with extensioninto:
WITHOUTrenal/upper airwayinvolvement (a) orbit= orbitalcellulitis
Dx: c-ANCA(96% sensitive,g9% specific) (b) base of skull= meningoencephalitis + cerebritis
536 Radiology Review Manual

B. PULMONARYFORM r/ rapidlyprogressive(oftenfatal)pneumonia
r/ segmentalhomogeneous consolidation Dx: cultureof fungusfrom biopsyspecimen/
{ cavitaryconsolidation+ air-crescentsign demonstrationwithinpathologicmaterial
r/ nodules(fromarterialthrombi+ infarction) DDx: aspergillosis
 DIFFERENTIAL
DIAGNOSISOF BREASTDISORDERS

VARIATIONS IN BREASTDEVELOPMENT AsvmmerntcBREAsTTlssue

UnilateralBreastDevelopment = greatervolume/ densityin one breastcompared
may exist 2 years before other breast becomes palpable with correspondingareain contralateralbreast

PrematureThelarche Denslry lH OruePno.leclor.r

= breastdevelopment <8 yearsof age = densityseen on only one standard
Cause: mammographic view
(1)isolated = mostlysubtleoverfunction
idiopathic of
pituitary-ovarian
axis Ancnrecrunal DrsroRTtoN
. NOgrowthspurt/ advanced boneage/ menses = focal area of distortedbreasttissue(spiculations
(2)centralprecociouspuberty with commonfocal point/ focal retraction/
r/ enlargement
of uterus+ ovaries tethering)withoutdefinablecentralmass
{ uni-/ bilateral
normalbreasttissue
Focnl Asvmuerntc Densmy
CongenitalAnomalies = focalasymmetricdensityseen on two mammo-
1. Polythelia graphicviews but not identifiedas a true mass
= rTlofe
thannormalnumberof nipples
2. Polymastia Diffuse Increase in Breast Density
= rnof€thannormalnumberof breasts r/ generalizedincreaseddensity
3. Amastia r/ skinthickening
= absence of mammary
glands r/ reticularpatternin subcutis
A. CANCER
1. "lnflammatory" breastcancer
2. Diffuseprimarynoninflammatory breastcancer
BREAST DENSITY 3. Diffusemetastaticbreastcancer
Asymmetric Breast Density 4. Lymphoma/ leukemia
A. BENIGN due to obstructivelymphedemaof breast
1. Postsurgicalscarring B . IN FE C TIOU S MA S TITIS
2. Noniatrogenictrauma usuallyin lactatingbreast
3. Postinflammatory fibrosis C . R A D IA TION
4. Radialscar (a) diffuseexudativeedemawithinweeks after
5. Ectopicbreasttissue beginningof radiationtherapy
6. Asymmetric breastdevelopment (b) indurationalfibrosismonthsafterradiationtherapy
7. Simplecyst D. EDEMA
8. Fibrocysticconditions: fibrosis
/ sclerosing 1. Lymphaticobstruction:extensiveaxillary/
adenosis intrathoracic
lymphadenopathy, mediastinal
/
9. Hormonal therapy:replacement, contraceptives anteriorchestwall tumor,axillarysurgery
B. MALIGNANT 2. Generalizedbody edema: congestiveneart
1. Invasiveductalcarcinoma: desmoplastic reaction failure(breastedemamay be unilateralif patient
2. Invasivelobularcarcinoma in lateraldecubitusposition),hypoalbuminemia
3. Tubularcarcinoma (renaldisease,livercirrhosis),fluidoverload
4. Primarylymphoma of breast E . H E MOR R H A GE
C. IMAGING PROBLEMS 1. Posttraumatic
1. Superimposed normalfibroglandular tissue 2. Anticoagulation therapy
2. Lesionobscured by overlapping dense 3. Bleedingdiathesis
parencnyma F. A C C ID E N TA LIN FU S IONOF FLU ID
3. Lesionoutside fieldof view intosubcutaneous tissue

Breast Imaging Reporting and Data System OVAL-SHAPED BREASTLESIONS

(BI-RADS) Categories Mammographic Evaluationof BreastMasses
0 Additionalimageevaluation
maybe necessary: Truemassorpseudomass?
off-angle
/ spotcompression
mammographic views; A . S IZE
ultrasound - well-defined
nodules<1.0 cm are of low riskfor
0 Unexplained abnormalities
warrantbiopsy cancer
538 Radiology Review Manual

- Il1'posl
likelybenign"nodulesapproaching1 cm 4. Invasiveductalcancernot otherwisespecified
shouldbe consideredfor ultrasound/ aspiration/ (rare)
biopsy 5. Pathologicintramammary lymphnode
B . S HA P E 6. Metastasesto breast: melanoma,lymphomaI
- increasein probability of malignancy: leukemia,lungcancer,hypernephroma
architecturaldistortion> irregular> lobulated>
oval > round Well-circumscribedDe Novo Massin Woman
C. MARGIN(mostimportantfactor) >40 Yearsof Age
- well-circumscribed masswith sharpabrupt 1. Cyst
transitionfrom surrounding tissueis almost 2. Papilloma
alwaysbenign 3. C arci noma
- rrftsls"signof apparentlucency= opticalillusion 4. Sarcoma(rare)
of Mach effect+ true radiolucenthalo is almost 5. Fibroadenoma (exceedingly rare)
always(92%)benignbut not pathognomonicfor 6. Metastasis(extremelyrare)
benignity
- microlobulated marginworrisomefor cancer
- obscuredmarginmay representinfiltrativecancer
- irregularill-defined
Fat-containing
BreastLesion
marginhas a high probability 0 Fat containedwithina lesionprovesbenignity!
of malignancy 1. Lipoma
- spiculatedmargindue to
2. Galactocele
(a) fibrousprojections extendingfrom main = fluidwith high lipidcontent(lastphase)
cancermass . during/ shortlyafter lactation
(b) previoussurgery
3. Traumaticlipidcyst = fat necrosis= oil cyst
(c) sclerosingduct hyperplasia(radialscar) . site of priorsurgery/ trauma
D. LOCATION 4. Focalcollectionof normalbreastfat
- intramammary lymphnodetypicallyin upper
outerquadrant(in 5% of all mammograms)
- largehamartoma+ abscesscommonin retro-/ Mixed Fat- and Water-densityLesion
1. Intramammary lymphnode
periareolarlocation
- sebaceouscyst in subcutaneoustissue 2. Galactocele
3. Hamartoma= lipofibroadenoma
E. X-RAYATTENUATION= DENSITY = fibroadenolipoma
- fat-containing lesionsare nevermalignant
- high-density 4. Smallsuperficial
hematoma
masssuspiciousfor carcinoma
(higherdensitythan equalvolumeof
Breast Lesion with Halo Sign
fibroglandular tissuedue to fibrosis)
F. NUMBER A . H IGH -D E N S ITY
LE S ION
- multiplicity = VeSSels+ parenchymal elementsnot visiblein
of identicallesionsdecreasesrisk
superimposed lesion
G . I NT E RV A LC H A N GE
- enlargingmass needsbiopsy 1. C yst
H. PATIENTRISKFACTORS 2. Sebaceouscyst
- increasingage increasesriskfor malignancy 3. Wart
- positivefamilyhistory B . LOW -D E N S ITY
LE S ION
- historyof previousabnormalbreastbiopsy = V€SSelS+ parenchymaseen superimposed on
- historyof extramammary malignancy lesion
1. Fibroadenoma
2. Galactocele
Well-circumscribed
BreastMass 3. Cystosarcoma phylloides
0 Well-defined nonpalpable lesionshave a 4% riskof
m alignanc y !
A. BENIGN Stellate/ SpiculatedBreastLesion
1. Cyst (a5%) = rn?SS/ architecturaldistortioncharacterizedby thin
2. Fibroadenoma linesradiatingfrom its margins
3. Sclerosingadenoma Riskof malignancy:
4. Intraductal papilloma(intracystic / solid) - 75% for nonpalpable spiculatedmasses
5. Galactocele - 32/" for nonpalpable irregularmasses
6. Sebaceouscyst A. PSEUDOSTELLATE STRUCTURE
7. Pseudoangiomatous stromalhyperplasia = SUMMATION SHADOWS
B. MALIGNANT causedby fortuitoussuperimposition of normal
1. M edull a ry
c a rc i n o ma fibrous+ glandularstructures;unveiledby rolled
2. Mucinouscarcinoma views,spot compressionviews + microfocus
3. lntracysticpapillarycarcinoma magnificationtechnique
 DifferentialDiagnosisof BreastDisorders SSg

B. "BLACKSTAR" { interspersedfatty elements

r/ groupsof fine fibrousstrandsbunchedtogether (b) summationartifact= chanceoverlapof normal
tr circular/ oval lucencieswithin center glandularbreaststructures
r/ changein appearanceon differentviews r/ failureto visualize"tumor"on morethan one
1. Radialsc?r= sclerosingduct hyperplasia view
2. Sclerosingadenosis 2. Siliconeinjections
3. Posttraumatic fat necrosis 3. Skin lesions
C. "WH]TESTAR" (a) Dermalnevus
r/ individualstraightdensespicules { sharp halo/ fissuredappearance
{ centralsolidtumor mass (b) Skin calcifications
r/ titttechangein differentviews r/ lucentcenter(clue)
(a) malignantfesions r/ superficiaflocation(tangentialviews)
1. Invasiveductalcarcinoma= scirrhous (c) Sebaceous/ epithelialinclusioncyst
carcinoma (d) Neurofibromatosis
= desmoplasticreaction+ secondary (e) Biopsyscar
retractionof surroundingstructures 4. Lymphedema
. clinicaldimensionslargerthan 5. Lymphnodes
mammographic size Frequency: 5.4% for intramammarynodes
d distinctcentraltumor mass with irregular Location: axilla,subcutaneoustissueof axillary
margins tail, laleralportionof pectoralismuscle,
r/ lengthof spiculesincreasewith tumor size intramammary(typicallyin upperouter
r/ localizedskin thickening/ retractionwhen quadrant)
, spiculaeextendto skin r/ ovoid/ bean-shapedmass(es)with fatty notch
r/ commonlyassociatedwith malignant-type representing hilum
calcifications r/ centralzone of radiolucency(fattyreplacementof
2. Infiltratinglobularcarcinoma center)surroundedby "crescent"rim of cortex
3. Tubularcarcinoma { usual l y< 1.5cm (upto 4 cm) i n si ze
4. Ductalcarcinomain situ r/ well-circumscribed with slightlylobulatedmargin
(b) benignlesions US:
1. Postoperativescar r/ reniformhypoechoicrim with echogeniccenter
. correlationwith history+ site of biopsy { echogenichilumfor entryand exitof vessels
{ scar diminishesin size + densityoueitime 6. H emangi oma
2. Postoperativehematoma
. clinicalinformation
{ short-termmammographicfollow-up Solid BreastLesionby Ultrasound
confirmscompleteresolution Malignant Sonographic Characteristics
3. Breastabscess
. clinicalinformation (accordingto data from A.T. Stavros)
r/ nign-Oensity lesionwith flamelikecontour US Characteristic Sens. Specif. PPV Rel. risk
4. Hyalinizedfibroadenoma with fibrosis Spiculation 36.0 99.4 91.8 5.5
r/ changingpatternwith differentprojections Tallerthan wide 41.6 98.1 81.2 4.9
d may be accompaniedby typicaicoarse Angularmargins 83.2 92.0 67.5 4.0
calcificationsof f ibroadenomas Acousticshadowing 48.8 947 64.9 3.9
5. Granularcell myoblastoma Branchpattern 29.6 96.6 64.0 3.8
6. Fibromatosis Markedlyhypoechoic 68.8 60.1 60.1 3.6
7. Extra-abdominal desmoid Calcifications 27.2 96.3 59.6 3.6
mnemonic; "STARFASH" Ductextension 24.8 95.2 50.8 3.0
Microlobulation 75.2 83.8 48.2 2.9
Summationshadow
Tumor(malignant) 0 Approximately5 malignantfeaturesare found per
Abscess cancer.The combination of 5 findingsincreasesthe
Radialscar sensitivityto 98.4%l
Fibroadenoma (hyalinized), Fat necrosis r/ spiculation = s;lgpnating straightlinesradiating
Adenosis(sclerosing) perpendicularly from surfaceof nodule
Scar (postoperative) (a) hypoechoicrelativeto echogenicfibroustissue
Hematoma(postoperative) (b) hyperechoicrelativeto surroundingfat
! tallerthan-widelesion= AP dimensiongreaterthan
Tumor-mimickingLesions craniocaudal / transversedimension
1. "Phantom
breast = simulated
tumor" mass V angularmargin= contourof junctionbetweenhypo-
(a)asymmetric
density or isoechoicsolidnoduleand surrounding tissueat
r/ scallopedconcave breast contour acute/ obtuse/ 90' angles
540 Radiology Review Manual

r/ acousticshadowingbehindall/ partof nodule ./ O.t-0.3 mm in size,irregular,sometimes

(= fibroelastichost responseto scirrhouscancer) mixedlinear+ punctate
{ centralpart of solid lesionvery hypoechoicwith Occurrence: secretorydisease,epithelial
respectto fat hyperplasia,atypicalductal
{ punctateechogeniccalcifications withinhypoechoic hyperplasia,intraductalcarcinoma
mass (acousticshadowingcommonlynot present) 2. Lobular microcalcifications
r/ radialextension/ branchpattern(= intraductal r/ smoothround,similarin size + density
componentof breastcancer) Occurrence:
r/ microlobulation = manysmalllobulations at surface cystichyperplasia, adenosis,sclerosing
of solidnodule adenosis,atypicallobularhyperplasia, lobular
carcinomain situ, cancerization of lobules
Benig n Sonographic Characteristics (= retrogrademigrationof ductalcarcinomato
r/ absenceof any malignantcharacteristics involvelobules),ductalcarcinomaobstructing
0 A singlemalignantfeatureprohibitsclassification egressof lobularcontents
of a noduleas benign! N.B.: lobularand ductalmicrocalcifications occur
r/ markedhyperechogenic well-circumscribednodule frequentlyin fibrocysticdisease+ breast
comparedwith fat = flonrlolstromalfibroustissue cancer!
(may representa palpablepseudomass/ fibrous (b) extramammary:arterialwall,ductwall,
ridge) fibroadenoma, oil cyst,skin,etc.
r/ smoothwell-circumscribed ellipsoidshape B . S IZE
r/ Z-g smoothwell-circumscribed gentlelobulations r/ malignantcalcifications usually<0.5 mm;
r/ tnin echogeniccapsule r a r e l y> 1 . 0m m
r/ kidney-shaped lesion= intramammary lymphnode C. NUMBER
0 lf specificbenignfeaturesare not foundthe lesion r/ <+-S calcificationsper 1 cm2have a low probability
is classifiedas indeterminate! for malignancy
D. MOR P H OLOGY
(accordingto datafromA.T.Stavros) (a) benign
US Characteristic Sens. Specif. NPV Rel. risk 1. Smoothroundcalcifications: formedin dilated
Hyperechoic 100.0 7.4 100.0 0.00 aciniof lobules
(3 lobulations 99.2 19.4 99.2 0.05 2. Solid/ lucent-centered spheres:usuallydue to
Ellipsoidshape 97.6 51.2 99.1 0.05 fat necrosis
Thin echogeniccapsule 95.2 76.0 98.8 0.07 3. Crescent-shaped calcificationsthat are concave
on horizontalbeam lateralprojection
= sedimentedmilk of calciumat bottomof cyst
BREASTCALCIFICATIONS 4. Lucent-centered calcifications: around
Indicativeof focallyactiveprocess;often requiringbiopsy accumulated debriswithinducts/ in skin
0 75-80% of biopsiedclustersof calcificationsrepresenta 5. Solid rod-shapedcalcifications / lucent-centered
benignprocess tubularcalcifications: formedwithin/ around
0 10-30% of microcalcifications in asymptomaticpatients normal/ ectaticducts
are associatedwith cancers 6. Eggshellcalcificationsin rim of breastcysts
Composition: hydroxyapatite / tricalciumphosphate/ 7. Calcifications with paralleltrack appearance
calciumoxalate = V€lscUlor calcifications
Resultsof breast biopsies for microcalcification: (b) malignant
(withoutany othermammographic findings) = calcifiedcellularsecretions/ necroticcancercells
(a) benignlesions(80%) withinducts
1. M as t op a thw y i th o u tp ro l i fe ra ti o........
n ....
44/" r/ calcificationsof
2. M as t op a thw y i thp ro l i fe ra ti o............
n ..... 28% vermicularform
3. Fibroadenoma........ ..4/" varyingin size
4. S olit aryp a p i l l o ma .....2% linear/ branchingshape
5 . M i s c e l l a n e o.u. s. . . . . . . .................2/" E . D IS TR IB U TION
(b) malignantlesions(20%) 1. Clusteredheterogeneous calcifications: adenosis,
1 . L o b u l acr a r c i n o m ian s i t u .......10% peripheralduct papilloma,hyperplasia, cancer
in 8/" no spatialrelationship to LCIS 2. Segmentalcalcifications withinsingleduct network:
2 . l n f i l t r a t i ncga r c i n o m a ................6% suspectfor multifocalcancerwithinlobe
3 . D u c t acl a r c i n o mian s i t u ...........4/" 3. Regional/ diffuselyscatteredcalcifications with
0 A positivebiopsyrate of >35o/"is desirablegoal! randomdistribution throughoutlargevolumesof
breast: almostalwaysbenign
A. LOCATION F. T I M EC O U R S E
(a) intramammary malignantcalcifications can remainstablefor >5 years!
1. Ductal microcalcifications G . D E N S ITY
 DifferentialDiagnosisof BreastDisorders S4l

Mal ignant Calcifications 6. Degeneratedfibroadenoma

1. Granularcalcifications= resemblingfinegrainsof r/ bizarre,coarse,sharplyoutlined,"popcornlike"
salt very dense calcificationwithindense mass
r/ amorphous,dotlike/ elongated,fragmented (= centralmyxoiddegeneration)
1 groupedvery closelytogether r/ eggshelltype calcification(= subcapsularmyxoid
r/ irregularin form,size,and density degeneration)
2. Casting calcifications = fragmentedcast of 7. Arterialcalcifications
calcifications withinducts r/ parallellinesof calcifications
r/ variablein size + length 8. Dermalcalcifications
r/ great variationin denlity withinindividualparticles Site: sebaceousglands
, + among adjacentparticles ./ hollowradiolucentcenter
r/ jaggedirregularcontour { polygonalshape
{ + Y-shapedbranchingpattern { peripherallocation(may projectdeep withinbreast
r/ clustered(t5 per focuswithinan area of 1 cm2) even on 2 views at 90. angles)
r/ linearorientationwhen caughtin tangent
r/ same size as skin pores
Benign Calcifications P roof: superfi ci al marki ngtechnique
1. Lobular calcifications = arisewithina spherical 9. Metastaticcalcifications
cavityof cystichyperplasia,sclerosingadenosis, Cause: 2' hyperparathyroidism (in up to 68%)
atypicallobularhyperplasia
r/ sharplyoutlined,homogeneous, solid,spherical
"pearl-like"
! littlevariationin size N IP P LE & S K IN
r/ numerous+ scattered NippleRetraction
r/ associatedwith considerablefibrosis 1. P osi ti onal
(a) adenosis 2. Relativeto inflammation
/ edemaof periareolar
r/ diffusecalcificationsinvolvingboth breasts tissue
symmetrically 3. Congenital
(b) periductalfibrosis 4. Acquired(carcinoma,ductalectasia)
r/ diffuse/ groupedcalcifications+ irregular
borders,simulatingmalignantprocess
2. Sedimentedmilkof calcium NippleDischarge
Frequency: 4/o Prevalence: 7.4 % of breastsurgeries
! multiple,bilateral,scattered/ occasionally Classification:
clusteredcalcifications withinmicrocysts A. PROVOKED
r/ smudge-likeparticlesat bottomof cyst on vertical postovulatorystate,duct ectasia,medication,
beam stimulationby exercise,breastself-examination,
r/ crescent-shaped on horizontalprojection sexualmanipulation
= "teacup-like"
B. SPONTANEOUS
3. Plasmacell mastitis= periductalmastitis (a) physiologic:pregnancy,lactation,
r/ sharplymarginatedcalcificationsof uniform galactorrhea,duct ectasia
, density= intraductalform (b) pathologic:benign/ malignantneoplasm,
! sharplymarginatedhollowcalcifications galactorrheadue to hyperprolactinemiafrom a
= periductalform pituitaryadenoma
4. Peripheraleggshellcalcifications C . U N ILA TE R A L
(a) with radiolucent lesion 0 Unilateralspontaneous
- liponecrosismicro-/ macrocysticacalcificans dischargeis significant
+ requiresinvestigation
I
(= fatty acids precipitateas calciumsoaps at D. BILATERAL
capsularsurface)as calcifiedfat necrosis/ 0 Expressedbilateralmultiporeblood-negative
calcifiedhematoma dischargeis physiologic and benign!
0 May mimicmalignantcalcificationsl
(b) with radiopaquelesion Type of discharge:
- degeneratedfibroadenoma
A. LACTATINGBREAST: galactorrhea
- macrocyst
B. NONLACTATING BREAST:
^/ frignuniformdensityin periphery (a) normal:
! usuallysubcutaneous 1. milky
I no associatedfibrosis 2. multicolored
sticky(blue,green,gray,
5. P apillom a brown,black)
r/ solitaryraspberryconfigurationin size of duct (b) abnormal:
r/ central/ retroareolar
3. purulent:antibiotics,
incision,drainage
542 RadiologyReviewManual

(c) surgicallysignificant(in 143% cancerous) { calcificationswith linearorientationtoward

4. clear/ watery: cancerin 33% subareolarareaa few mm long: rod-shaped/
5. bloody/ sanguineous:cancer 28/o, sausage-shaped / sphericalwith hollowcenter
6. pink/ serosanguineous: cancerin 13'h 3. Galactocele
7. yellow/ serous:cancerin 6% 4. Plasmacell mastitis
0 The most commoncause of bloodyand
serosanguineous dischargeis intraductal
papilloma! SkinThickeningof Breast
0 Exfoliativecytologynot helpful(truepositive Normal 0.8-3mm;mayexceed
skinthickness: 3 mm
in only 11V",falsenegativein 18%) region
ininframammary
A . LOC A LIZE D S K INTH IC K E N IN G
Siteof origin: 1. Trauma(priorbioPsY)
A. Lobules+ terminalduct lobularunit: 2. Carcinoma
1. Galactorrhea 3. Abscess
2. Fibrocysticchanges 4. Nonsuppurative mastitis
B. Largerlactiferousducts (collectingduct, 5. Dermatologicconditions
segmentalduct, subsegmentalduct) B . GE N E R A LIZE D S K INTH IC K E N IN G
1. Solitarypapilloma 0 Skin is thickenedinitiallyand to the greatestextent
2. Papillarycarcinoma in the lower dependentportionof breast!
3. Ductectasia { overallincreaseddensitywith coarsereticular
pattern(= dilatedlymphvessels+ interstitialfluid
GalactographyI ductography: triggeringfibrosis)
injectionof 0.2-0.3 mL of water-solublecontrast (a) Axillarylymphaticobstruction
material(Conray60@,lsovue@)throughstraightblunt 1. Primarybreastcancer
27-gaugepediatricsialographycannula(0.4-0.6 mm - advancedbreastcancer
outerdiameter)/ 3O-gaugecannula/ Jabczenski - invasivecomedocarcinoma in largearea
cannula(tip bent90') 0 Primarybreastcancernot necessarilyseen
Resultsof positive galactography: due to small size lhidden location(axillary
papilloma(48%),benignconditions(42/"), tai l ,behi ndni P P l e)!
intraductalcarcinoma (10%) 2. Primarymalignantlymphaticdisease
(eg, lymPhoma)
Contraindications to ductography : (b) Intradermal+ intramammaryobstructionof lymph
historyof severeallergyto iodinatedcontrast channels
material,inabilityof patientto cooperate 1. Lymphaticspreadof breastcancerfrom
(debilitatinganxiety,mentaldisorder),historyof contralateralside
priornipplesurgery 2. Inflammatorybreastcarcinoma= diffusely
invasiveductalcarcinoma
DDx of intraductal defects: (c) Mediastinallymphaticblockage
gas bubble,clot, inspissatedsecretions,solitary 1. Sarcoidosis
intraductalpapilloma,epithelialhyperplasticlesion, 2. Hodgkindisease
duct carcinoma 3. Advancedbronchial/ esophagealcarcinoma
4. Actinomycosis
Galactographic Filling Defect (d) Advancedgynecologicmalignancies
from thoracoepigastric collaterals
Single 1. Ovariancancer
Muttiptepapilloma 5.6% 14.O/" 2. Uterinecancer
Cancer 0.05% 9.7% (e) Inflammation
1. Acutemastitis
2. Retromamillary abscess
3. Fat necrosis
SecretoryDisease 4. RadiationtheraPY
1. Retainedlactiferoussecretions 5. ReductionmammoPlastY
resultof incomplete/ prolongedinvolutionof (f) Rightheartfailure
lactiferousducts may be unilateral(R t L) / migratingwith change
r/ branchingpatternof fat densityin dense breast in patientposition(to avoiddecubitusulcer)
(highlipidcontent) (g) Nephroticsyndrome,anasarca
2. Prolongedinspissationof secretion+ intraductal 1. D i al ysi s
debris 2. RenaltransPlant
= Mammaryduct ectasia (h) Subcutaneousextravasationof pleuralfluid
r/ duct dilatation followingthoracentesis

Lymphadenopathy
Radiographic features of normal lymph nodes:
. nonpalpable
r/ mass of low to moderatedensity
r/ sharplydefined
r/ roundto oval
r/ radiolucentfatty hilus (visiblein 78%)
./ <1 cm withinbreasttissue,<1.5cm withinaxilla
Intramammary Lymphadenopathy
= adenopathy>1 cm surroundedby breasttissue
N.B.: nodeslocatedhighwithinaxillarytail (= tail of
Spence)are mammographically difficultto
differentiate
from inferioraxillarylymphnodes
AxiIIary Lymphadenopathy
= solidnode >1.5cm in sizewithoutfattyhilum
N.B.: lymphnodesof up to 3 cm may be normalif
largelyreplacedby fat
A . M A LI G N AN T
1. Metastasisfrom breastcancerin 26./"
0 Primarybreastlesionmay not be found in
33% of cases!
2. Metastasesfrom non-breastprimary(lung,
melanoma,thyroid,Gl tract,ovary)
3. Lymphoproliferative disease: lymphoma/
chroniclymphocytic a (17%)
leukemi
0 Bilateralaxillarylymphadenopathy is
suggestiveof lymphoproliferativedisease!
B. BENIGN
1. Nonspecificbenignlymphadenopathy (29%)
2. Reactivenodal hyperplasia(breastinfection/
abscess/ biopsy)
3. Collagenvasculardisease: rheumatoid
arthritis,systemiclupuserythematosus
4. Granulomatous disease:sarcoidosis
5. Psoriasis
6. HIV-related adenopathy
7. Siliconeadenopathy
Radiographic features suspicious for malignancy:
{ size increaseof >100%over baseline
r/ size >3.3cm
r/ changein shape
r/ spiculation of margins
r/ intranodalmicrocalcifications (withouthistoryof
gold therapy)
r/ loss of radiolucentcenterI hilarnotch
r/ increasein density
DifferentialDiagnosisof BreastDisorders 543
MAMMOGRAPHY REPORTS
BreastlmagingReportingand DataSystem
(BrRD)
N = negative
thereis nothingto commenton; breastsare
symmetricalwithoutmasses,architectural
disturbances/ suspiciouscalcifications
B = beni gnfi ndi ng
confidentlylabeled,eg, calcifiedfibroadenoma,
multiplesecretorycalcifications, fat-containing
lesionsuch as oil cyst,lipoma,galactocele,
mixed-density hamartoma,intramammary lymph
node,implant
P = probablybenignfinding- shortintervalfollow-up
highprobability of benignwith radiologist's
preferenceto establishits stability
S = suspiciousabnormality - considerbiopsy
lesionwithoutcharacteristic morphologyof
cancerbut definiteprobability of beingmalignant
M = highlysuggestiveof malignancy
biopsyis mandatory
LexiconDescriptorsfor Reporting(ACR)
A. MASS
size
shape circular,oval,lobulated,irregular
margins circumscribed, lobulated,
obscured,indistinct,speculated
location based on face of clock + depth
in breast
associatedfindingsskin changes,calcifications,
nippleretraction,trabecular
thickening
attenuation relativeto an equalvolumeof
breasttissue: highdensity,
isodense,low density,fat
density
B. CALCIFICATIONS
type skin,vascular,coarse,rodlike,
eggshell,punctate,pleomorphic
number
size
distribution clustered,linear,segmental,
regional,scattered,multiple
groups
associatedfindingsskinchanges,nippleretraction,
architecturaldistortion,
trabecularthickening
544 Radiology Review Manual

BREASTANATOMYAND MAMMOGRAPHICTECHNIQUE

BREASTDEVELOPMENT StagelV (areolarmounding)

Embryology . secondarymounddevelops(verytransient)with
"Milkline"developsfrom ectodermalelements nipple+ areolaprojectingabovethe breasttissue
+ extendsfrom axillaryregionto groin;lack of { hyperechoic periareolarfibroglandular
tissue
regressionleadsto developmentof accessorybreast { prominentcentralhypoechoicnodule
tissue/ accessorynipples StageV (maturebreast)
. regressionof areolaforminga smoothcontourwith
TannerStages the rest of the breasttissue
{ hyperechoic glandulartissue
Stage| (prepubertal)
. nippleelevates ./ increasedsubcutaneousadiposetissueanteriorly
^/ ttlOhypoechoiccentralnodule
r/ ill-definedhyperechoicretroareolartissue
Stage ll
Cause: estrogenfor ductal+ progesteronefor BREASTANATOMY
lobuloalveolar
development Lobes
. palpablesubareolarbud = thelarchebeginswith 15-20 lobesdisposedradiallyaroundnipple,each lobe
onset of puberty(meanage, 9.8 years) has a main lactiferousduct of 2.0-4,5mm convergingat
. breasttissue+ nippleariseas a singlemoundof the nipplewith an openingin the centralportionof
tissue nipple
{ hyperechoicretroareolarnodule Mainduct: branchesdichotomously eventuallyforming
r/ centralstar-shaped/ linearhypoechoicarea (simple terminalductallobularunits
branchedducts) Histo:epithelialcells,myoepithelialcellssurroundedby
Stagelll extralobularconnectivetissuewith elasticfibers
. enlargement+ elevationof singlemound
{ hyperechoicglandulartissueextendingaway from TerminalDuctLobularUnit (TDLU)
retroareolararea (1) Extralobular terminalduct
{ centralspider-shapedhypoechoicarea Histo:linedby columnarcells+ prominentcoat of
elasticfibers+ outerlayerof myoepithelium
(2) Lobule
(a) intralobularterminalduct
elastic fibers Histo: linedby 2 layersof cuboidalcells + outer
layerof myoepithelium
(b) ductules/ acini
intralobular (c) intralobularconnectivetissue
connective Size: 1-8 mm (most1-2 mm) in diameter
C hange:
(a) reproductiveage: cyclicproliferation(up to time of
ovulation)+ cyclicinvolution(duringmenstruation)
(b) post menopause:regressionwith fatty
replacement
Significance:
TDLU is site of fibroadenoma, epithelialcyst,apocrine
metaplasia,adenosis(= proliferationof ductules
+ lobules),epitheliosis (= proliferation
of mammary
epithelialcellswithinpreexisting ducts+ lobules),
ductal+ lobularcarcinomain situ,infiltrating ductal
+ lobularcarcinoma
extralobular
connective
tissue Componentsof NormalBreastParenchyma
1. Nodulardensitiessurroundedby fat
(a) 1-2lnln = normallobules
extralobular (b) 3-g nrr'r= adenosis
terminal lobule
duct 2. Lineardensities
= ductsand theirbranches+ surrounding elastic
tissue
terminal ductal lobular unit 3 . Structurelessground-glassdensity
TerminalDuctalLobular Unit = stroma/ fibrosiswith concavecontours
 BreastAnatomyand MammographicTechnique 545

ParenchymalBreastPattern(Ldszt6
Tab60 (b) "No man's land"= fatty replacedarea between
PatternI posteriorborderof parenchyma+ chest wall on CC
namedQDY = quasidysplasia(for Wolfe projection
classification) (c) Medialhatf of breaston CC view
r/ concavecontourfrom Cooper'sligaments 3. Look for increasedretroareolardensity
r/ evenlyscattered1-2 mm nodulardensities 4. Look for parenchymalcontourretraction
(= normalterminalductallobularunits) 5. Look for architecturaldistortion
r/ oval-shaped/ circularlucentareas (= fatty 6. Lookfor straightlinessuperimposed on normal
replacement) scallopedcontour
Pattern ll 7. Compareleft with rightside
similarto N1 (wolfe) 8. Don'tstop lookingafterone lesionis found
r/ total fatty replacement
./ lttOnodulardensities
Pattern lll MAMMOG RAPHIC TECHNIQU E
similarto P1 (Wolfe)
{ normalparenchymaoccupying <25/" of breast BEAM QUALITY
volume in retroareolarlocation Molybdenumtarget materialwith characteristicemission
Pattern lV = adenosispattern peaksof 17.9 + 19.5 keV (loweraverageenergythan
similarto P2 (wolfe) tungsten)
Cause: hypertrophy+ hyperplasiaof aciniwithin
lobules FOCALSPOT
Histo: small ovoid proliferatingcellswith rare mitoses 0.1-0.4 mm (0.1 mm for magnification
views)
r/ scattered3-7 mm nodulardensities(= enlarged TUBE OUTPUT
terminalductallobularunits)= adenosis 80-100 mA
r/ ttricXlineardensities(= periductalelastictissue
proliferationwith fibrosis)= fibroadenosis E X P OS U R E
r/ no changewith increasingage (genetically (a) withoutgrid: 25 kV (optimumbetweencontrast
determined) + penetration),exposuretime of 1.0 seconds
Pattern V (b) with grid: 26-27 kY: exposuretime of 2.3 seconds
similarto DY (Wolfe) (c) microfocusmagnification:26-27 kV; 1.5-2.0 times
r/ uniformlydenseparenchymawith smoothcontour magnificationwith 16-30 cm air gap
(= extensivefibrosis) (d) specimenradiography:22-24 kV

MA M M O G RA P HIC F IL M R E AD IN G T E C H N IQU E FILTER

1. Comparewith earlierfilms (a) beryltiumwindow (absorbsless radiationthan glass
2. Scan "forbidden"areas tube)
(a) "MilkyWay" = 2-3 cm wide area parallelwiththe (b) molybdenumfilter(0.03mm): allowsmoreof lower
edge of the pectoralmuscleon MLO projection energyradiationto reachbreast

Pattern I Pattern II Pattern III Pattern[V Pattern V

Parenchymal Breast Pattern

546 Radiology Review Manual

REDUCTIONOF SCATTERRADIATION 3 . Air-gapeffect= increasedcontrastby reductionin

(1) adequatecompression(alsoimprovescontrast scatteredradiation
+ decreasesradiationdose) 4 . Visualeffect= improvedperceptionand analysisof
(2) beamcollimation to <8-10 cm smalldetail
(3) air gap with microfocusmagnification
(greaterspatialresolution,2-3Jold increasein
radiationexposure) lmageQuality
FactorsAffectingMammographic
(4) Movinggrid Radiographic Sharpness
grid if compressedbreast>5 cm I very dense breast = subjectiveimpressionof distinctness / perceptibility
(facilitatesperception,2-3{old increasein radiation of structureboundary/ edge
exposure) 1. Radiographiccontrast
= rn?gnitudeof opticaldensitydifferencebetween
S CRE E N- F I LM
C OMB IN A T IO N structureof interest+ surroundings influenced
(1) lntensifyingscreenphosphor by
singlescreensystems (a) subjectcontrast
(2) Film-screencontact = fotio of x-ray intensitytransmittedthrough
(3) Mammography filmwith minimalbasefog, sufficient one part of the breastto that transmitted
maximumdensity+ contrast througha more absorbingadjacentpart;
affectedby
FI LMP RO CE S SIN G - absorptiondifferencesin the breast
(1) Processingtime of 3 minutes(42-45 secondsin (thickness, density,atomicnumber)
developingfluid)superiorto 90-secondprocessorfor - radiationquality(targetmaterial,
double-emulsion film (whichcreates kilovoltage,f iltration)
underdevelopment + compensatory higherradiation - scatteredradiation(beamlimitation,grid,
exposure) compression)
(2) Developingtemperatureof 35"C (95'F) (b) receptorcontrast
(3) Developingfluidreplenishment rate: = colTtPortent of radiographiccontrastthat
450-500 mL replenisher per squaremeterof film determineshow the x-ray intensitypattern
will be relatedto the opticaldensitypattern
QUALITYCONTROL in the mammogram
(1) Processor(daily) affectedby
with sensito-/ densitometricmeasurements - filmtype
( a) bas ef o g < 0 .1 6 -0 .1 7 - processing(chemicals, temperature, time,
(b) maximumdensity>3.50 agitation)
(c) contrast>1.9-2.0 - photographic density
(2) X-ray unit (semiannually) - fog (storage,safelight,light leaks)
(a) beam quality 2. Radiographicblurring
(b) phototimer = lateralspreadingof a structuralboundary
(= distanceover which the opticaldensity
betweenthe structureand its surroundings
Average glandular dose: changes)
<0.6 mGy per breastfor nonmagnification film-screen (a) motion
mammogram(ACR accreditationrequirement) reducedby compression+ short exposuretime
Screen/filmtechnique(molybdenum target;0.03 mm (b) geometricblurring
molybdenumfilter,28 kVp): affectedby
mean absorbeddose: 0.05 rad for CC view - focalspot: size,shape,intensity
0.06 rad for LAT view distribution
- focus-objectdistance(= cone length)
Effective dose equivalent H,: - object-imagedistance
screen-filmmammography ... 0.11 mSv (c) receptorblurring
x er or adiogr a p hmi ca m m o g ra p h..........
y 0 .7 8mS v = lightdiffusion(= sPreadingof the light
c hes t .....0 .0 5mS v emittedby the screen)affectedby
sku11........ 0 . 1 5m S v - phosphorthickness+ particlesize
abdom en 1 .4 0mS v - light-absorbing dyes + pigments
lumbarspine 2.20 mSv - screen-filmcontact

Advantages of magnification mammography Radiographic Noise

1. Sharpnesseffect= increasedresolution = unwantedfluctuationin opticaldensity
2. Noiseeffect= noise reducedby a factorequal to the 1. Radiographicmottle
degreeof magnification = opticaldensityvariationsconsistof
 BreastAnatomyand MammographicTechnique 547

(a) receptorgraininess (c) structuremottle

= opticaldensityvariationfrom random
distribution of finitenumberof siMerhalide
grains
(b) quantummottle(principalcontributorto mottle)
= variationin opticaldensityfrom random
spatial distributionof x-ray quanta absorbed
in image receptor
affectedby
- film speed + contrast
- screenabsorption+ conversionefficiency
- lightdiffusion
- radiationquality
= opticaldensityfluctuationfrom nonuniformity
in the structureof the image receptor(eg,
phosphorlayerof intensifyingscreen)
2. Artifacts
= Ur'rw€lr'rted opticaldensityvariationsin the form
of blemisheson the mammogram
(a) improperfilm handling(static,crimpmarks,
f ingerprints,scratches)
(b) improperexposure(fog)
(c) improperprocessing(streaks,spots,
scratches)
(d) dirt + stains
548 Radiology Review Manual

BREASTDISORDERS

BREASTCANCER { soft-tissueabnormality only (10%)

lncidence: 1.5-4.5 cases per 1,000women per year
Origin: terminalductal lobularunit
invasive lobular
carcinoma
invasive ductal
carcinoma
tubular ca.
not otherwise
specified
medullary ca.
mucinous ca.
others
(eg, papillary ca.)
Distribution of Breast Cancers
in Screening Population
(numbersare percentages)
BR EA STC AN C E R(1 5 % )
A . NO NT NV A S T VE
= malignanttransformationof epithelialcellslining
mammaryducts+ lobulesconfinedwithin
boundariesof basementmembrane
Rx: few data are availableto provideinsightinto
propertreatment
1. Ductal carcinoma in situ (DCIS)
= intraductal carcinoma
Incidence: 10-2540/" in screening population;
70'/" of noninvasivecarcinomas
Age: most >55 years
Histo: heterogeneousgroup of malignancies
originating withinextralobularterminalduct
+ withoutinvasionof basementmembrane
Subgroups: comedocarcinoma, non-
comedocarcinomas (solid,
micropapillary,cribriform)
. may persistfor years withoutpalpable
abnormality(in screeningpopulation)
. palpablemass/ Pagetdiseaseof nipple/ nipple
discharge(in symptomaticpatients)
0 50% of DCISare >5 cm in size
0 Histologicsize of DCIS is independent of
histologicsubgroup
0 Almostall "comedo"type DCIScontainsignificant
microcalcifications
0 DCISofteninvolvesthe nipple+ subareolarducts
Spectrum of mammographic findings:
r/ calcificationsonly (72%)
r/ soft-tissueabnormality+ calcification(12%)
{ nonvisible(6%)
MR (40-100% sensitive):
r/ linearcontrastenhancement
Prognosis: 20-50/" develop invasivedisease 5-
10 yearsafterinitialdiagnosisof DCIS
Rx: (1) Simple/ modifiedmastectomy:cure rate
of almost 100%
(2) Localexcisionalone:
25/" rale of recurrencewithin26 months
in immediatevicinityof biopsysite
(3) Localexcision+ radiotherapy:
2-17"/. rate of recurrence
Treatmentproblems:
1. Occultinvasionin 5-20'/. of patients
2. Multifocality
(= >1 focusin same quadrantof breast)
3. Multicentricity
(= >1 focus in differentquadrantsof breast)in
14/" of lesions<25 mm, in 100%of lesions
>50 mm
4. Axillarymetastasesin 1-2oh
(a) high nucleargrade DCIS ("comedo type")
Prevalence: 60% of all DCIS
Precursorj none;one stagedevelopment
Path: "comedo"= pluglikeappearanceof
necroticmaterial that can be expressed
from the cut surface
Characteristics:
- nucleargrade: large/ intermediate nuclei,
numerousmitoses,aneuploidy
- growthpattern:predominantly solidcell
proliferation ; atypicallymicropapillary I
cribriform
- necrosis:extensive(HALLMARK)
- calcifications (90%): dystrophic/
amorphouswithinnecrosisin centerof
dilatedductalsystemoutliningmostof the
lobe in classicsolidgrowthpattern
. estrogen-+ progesterone-receptor negative
. overexpression of c-erbB-2 oncogene
productand P53 suppressorgene mutation
. oftensymptomaticlesionwith nipple
discharge
r/ ductalsystemenlargedto 300-350 pm
^/ linear/ branchingpatternof calcifications
scatteredin a largepart of lobe / whole lobe
^/ largesolid high-densitycastingcalcifications
(fragmented, coalesced,irregulaQin solid
growthpattern
"birchtree flowerlike"
{ "snakeskin-like"/
dottedcastingcalcifications within necrosis
of micropapillary / cribriformgrowthpattern
r/ palpabledominantmass withoutcalcifications
(veryunusual)
r/ nippledischarge(rare)

Prognosis; higherrecurrencerate than
noncomedo-group
(b) low nucleargrade DCIS (,,noncomedotype")
Prevalence: 40/" of all DCIS
Precursor lesion:
atypicalductalhyperptasia(ADH)with slight/
moderate/ severeatypia
0 52-56/o of ADH at core biopsy are
associatedwith malignancyat excision!
Characteristics:
- nucleargrade: monomorphicsmall round
nuclei,few / no mitoses
- growthpattern: predominanily
micropapillary / cribriform;atypicallysolid
cell proliferation(oftencoexist)
- necrosis:not presentin classic
micropapillary I cribriformgrowthpattern
- calcifications
(50%): laminatedi
psammoma-likedue to activesecretionby
malignantcellsintoduct lumen
{ fine granular"cottonball"calcificationsin
, micropapillarylcribriformgrowthpattern
! coarsegranular"crushedstone"/,,broken
needletip" / "arrowhead"calcificationsin less
commonsolidgrowthpattern
0 Sizeof "noncomedo" DCISoften
underestimatedmammographically
(? due to lowerdensityof calcificationsat
periphery of lesion)!
, of fluid
I palpabledominantmass withoutcalcifications
(intracystic papillarycarcinoma,multifocal
papillarycarcinomain situ)
r/ nonpalpableasymmetricdensitywith
architecturaldistortion
r/ occasionallyserous/ bloodynippledischarge
+ ductalfillingdefectson galactography
Risk of recurrence: Z./"
Prognosis: 30/" eventuallydevelop into
Invastvecancer
Dx: surgicalbiopsy
0 Core needlebiopsycouldresultin
diagnosisof only proliferativebreast
diseasethat is usuallyintermixed!
2. Lobular carcinoma in situ (LCIS)
= arisesin epitheliumof bluntductsof mammary
lobules
lncidence; 0.8-3.67"in screeningpopulation;
3-6% of all breastmalignancies;
25/" of noninvasivecarcinomas;high
incidenceduringreproductiveage but
decreasingwith age
Age: most 40-54 years (earlierthan DCIS/
invasivetumors)
Histo: monomorphous smallcell populationfilling
+ expandingductulesof the lobule
0 Synchronousinvasivecancerin 5%!
. not palpable
r/ mammographically occult
BreastDisorders 549
./ may atypicallypresentas a noncalcifiedmass (in
7"/"),calcifications + mass (in 10%),asymmetric
opacity(2%)
0 High frequencyof multicentricity (70%)
+ bilaterality(30%)!
Dx: incidentalmicroscopic findingdependingon
accidentof biopsy(performedfor unrelated
reasons+ findings)
Prognosis:
20-30% developinvasiveductal> lobular
carcinomawithin20 yearsafterinitialdiagnosis
0 1% per year lifetimerisk for invasive
malignancy
0 LCIS servesas a markerof increasedrisk for
developinginvasivecarcinomain eitherbreast!
Rx: recommendations rangefrom observation
(withfollow-upexaminationsevery
3-G months+ annualmammograms)
to
unilateral i bilateralsimplemastectomy
3. Intracystic papillary carcinoma in situ (0.5-2%)
= r?revariantof noncomedoDCIS
Age: usuallyolderpostmenopausal woman;peak
prevalencebetween34 and 52 years
Histo: papillaryfrondswithinthe walf of a
cysticailydilatedduct
. well-circumscribed + freelymovable
. aspirationyieldsstraw-colored/ dark red / brown
fluid(dueto rupturedcapillariesin cystwall/
necrosisof tumorcells);reaccumulation
within3-4 weeks
., fluidcytologynegativefor cancerin g0%
1 meantumorsizeof 1.9cm (range0.4-7.5 cm)
due to fast growth(fromaccumulation of fluid
+ proliferationof neoplasticcells)
^/ intracysticmass on pneumocystography
{ solid intracysticmass on US
r/ roundbenignappearingmasswith sharply
circumscribedlobulatedborderson
mammography
Rx: lumpectomy
Prognosis; 1O-yearsurvivalof 100%; 10-year
disease-freesurvivalrate of g1./"
DDx for mammogram;mucinous / medullaryca.,
hematoma,metastasis
B. |NVAS|VEBREASTCANCER(85%)
MR:
! peripheral/rim enhancement
1. Infiltrating/ invasiveductal carcinoma (65%)of
no specialtype / otherwisenot specified(NOS)
10% false-negative ratio
Histo:
grade | - well-differentiated
grade ll = moderatelydifferentiated
grade lll = poorlydifferentiated
. palpablein 70%
., largerby palpationthan on mammogram
r/ spiculatedmass (36%)is PRtNCtpALF|NDtNG
r/ malignantcalcifications(45-60%)
550 RadiologyReviewManual

2. Infiltratingi invasive lobular carcinoma (5-10%) 0 Fastestgrowingbreastcancerl

= neoplasmarisingfrom terminalductulesof breast Path: well-circumscribed mass with nodular
lobules architecture+ lobulatedcontour;central
0 2nd most commontype of breastcancer; necrosisis commonin largertumors;
30-50% of patientswill developa secondprimary reminiscentof medullarycavityof bone
in same / oppositebreastwithin20 years Histo: intenselymphoplasmocyticreaction
0 Most frequentlymissedbreastcancer(difficultto (reflectinghost resistance);propensityfor
detectmammographically with 19-
+ clinically) syncYtialgroMh; no glands
43% false-negative rate (occult in dense breast) tncidence: 11ohof breastcancersin women
Medianage: 45-56 years; 2/" of all ILC occur <35 years of age; 40-50% of medullary
in women <35 years cancers in women <50 Yearsof age
Path: multicentricity + bilaterality(in up to 1/3); Mean age: 46-54 Years
tendencyto grow aroundducts,vessels,and . softerthan averagebreastcancer
lobuleswithoutdestructionof anatomic r/ well-definedround/ oval noncalcifieduniformly
structures(targetoidgrowth);no substantial dense mass (hemorrhage)with lobulatedmargin
connectivetissuereaction { may have partial/ completehalo sign
Histo: 20Y"grade1,64o/" gradell, 16% grade lll US:
Metastases: Gl tract,gynecologicorgans, r/ hypoechoicmass with some degreeof through
peritoneu m, retroPeritoneum, transmission
carcinomatousmeningitis r/ distinct/ indistinctmargins
. palpablein 69%: r/ largecentralcysticcomPonent
. ateaof subtleskinthickening/ induration DDx: fibroadenoma
. large hard mass / fine nodularitY Prognosis: 92/" 1O-yearsurvivalrate
r/ architecturaldistortion(= retractionof normal
glandulartissuewith thickening+ disturbanceof 5. Mucinous / colloid carcinoma (1.5-2%)
fibroussepta)in 18-30% is MosT coMMoN Path:
M A M MOGR AP H IC F IN D IN G (a) pure form: aggregatesof tumor cells
Histo: straightsinglefile of uniformsmall cells surroundedby abundantpools of extracellular
with roundoval nuclei("lndianfiles") mucin(gelatinous / colloidfluid)
growingaroundducts resultingin (b) mixed form: containsareasof infiltrating
subtlechangesin architecture ductalcarcinomanot surroundedby mucin
{ irregularspiculatedmass >1 cm (16-28%) Age: 1"/"in women <35 years;7'/" of carcinomas
r/ poorlydefinedmass + spicules<1 cm (22%) in women >75 Years
. slow growthrate of Pureform
r/ asymmetricopacitY(= ill-definedarea of
increasedopacitywithoutcentraltumor nidus)in . "swish"/ "crush"sensationduringpalpation
v19% . 60o/oestrogen-receptor positive
r/ round/ ovoid mass with regularborders(1%) r/ well-circumscribed usuallylobulatedmass of
./ microcalcifications(0-24%\ round/ ovoid shaPe
{ retractionof skin (25%)+ nipple(26%) { pleomorphic clusteredlclumpedamorphous/
{ skinthickening punctatecalcifications(rare)
0 May be evidenton ONLY one standardview CC r/ may enlargefast (throughmucinproduction)
> ML > MLO view)! r/ solidmass on US
N.B.: difficulties in earlydiagnosisresultin Prognosis; favorable
disproportionate potentialfor malpractice
s u i ts ! 6. Papilfarycarcinoma (1-24%)
= rofe ductalcarcinomaformingpapillarystructures
3. Tubular carcinoma (6-8%) N.B.: Do not confusewith micropapillary / cribriform
= well-differentiated
form of ductalcarcinoma growthpatternof ductalcarcinoma
(a) low grade: bilateralin 1:3 Histo: multilayeredpapillaryprojections
(b) highgrade: bilateralin 1:300 extendingfrom vascularizedstalks;no
Associatedwith: lobularcarcinomain situ in 40% myoepithelial layer(as in benignlesions);
Mean age: 4049 years neurosecretory granules+ positiveCEA-
' positivefamilyhistorYin 40/" reactivityin 85% (absentin benignlesions)
' nonpalpable Types:
{ nigfr-opacitynodulewith spiculatedmargins (a) multipleintraductalcarcinomaswith papillary
4 4l mm in diameter;meandiameterof 8 mm configuration
DDx: radial scar (b) Intracysticpapillarycarcinoma
= in situ malignancy
4. Medullary carcinoma (2/") (c) invasivecarcinomawith papillarygrowth
= SOLIDCIRCUMSCRIBED CARCINOMA pattern(microscopicfrond formation)
 BreastDisorders 551

Age: 25-89 (mean50-60) years;peak age of ./ tumor mass + malignanttypecalcifications

40-75 years r/ diffusetyincreasedbreasidensity
. palpablemass (67%)
r/ stromalcoarsening(50%)
. nippledischarge(22-gS%)oftentingedwith blood ^/ thickeningof Cooperligaments
. rich in estrogenand progesteronereceptors r/ extensiveskinthickening(11%)
Location: singlenodulein centralportionof Dx: skinbiopsy
breast;multiplenodulesextendingfrom Prognosis: 2%"S-yearsurvival; median survivaltime
subareolararea to peripheryof breast of 7 months(untreated)
+ 1g months(after
r/ multinodularpattern(55%)= lobulatedmass / radicalmastectomy)
clusterof well-defined contiguousnodules DDx: breast abscess
r/ solitarywell-circumscribed round/ ovoidnodule
with averagediameterof 2-3 cm
{ usuallyconfinedto singlequadrant Epidemiology
of BreastCancer
ri associatedmicrocalcifications in 60% lncidence:
r/ multiplefillingdefects/ disruptionof an irregular 2-5 breastcancers/1,000 women;in USA >142,000
duct segment/ completeobstructionof duct new casesper year (of which25,000are in situ);
systemat galactography 25/" of all femalemalignancies
US: 0 One of 9 womenwill developbreastcancerduring
{ solidhypoechoicmasswith lobulatedsmooth her lifel
, margins+ acousticenhancement Age: 0.3-2% in women <30 years of age;
r/ + bloodflow on color Doppler
15/"in women<40 yearsof age;
Prognosis: g0/" S-yearsurvivalafter simple
85% in women >30 years of age
mastectomy+ axillarynode dissection Mortality: 43,000 deaths per year
DDx: solitarycentralduct papilloma;multiple 0 Deathrate has remainedstablefor past 60 yearsl
peripheralbenignpapillomas
Risk Factors(increasing risk):
c. P A G E TDT S E AS O
E F T H E N T P PL E
(5 % ) A. DEMOGRAPHIC FACTORS
. increasingage (6G%of cancersin women
D. INFLAMMATORY BREASTCARCINOMA >50 years):
= tumoremboliwithindermallymphatics prevalence of Cancer
Age
(angiolymphatic spread)
Prevalence: 1-4% of breast cancers 25 5:100,000 1: 1 9 , 6 0 8
Age: 52 years (on average) 40 80:100,000 1: 1 , 2 5 0
Histo: infiltratingductalcarcinoma 45 1075:100.000 1:93
Location: L > R breast;bilateralityin 30-55% 50 180:100,000 1:555
. rapidsymptomaticdevelopmentover 1i3 of breast 55 3030:100,000 1:33
surface: 60 240:100,000 1: 4 1 6
. palpabletumor(63%) Relative Risk Compared with Woman of Age 60:
. erythemaof skin (19-64%) 30 yearsof age 0.07 60 yearsof age 1.00
. peaud'orangeedemaof skin (19/.) 35 yearsof age 0,19 70 yearsof age 1.27
. nippleretraction(19%) 40 years of age 0.35 B0 years of age 1.45
. palpableaxillaryadenopathy(up to 91%) 50 yearsof age 0.71

Predictive Values of Radiographic Signs for Malignancy

1. C l a s s i cma mmo g ra p h fi i cn d i n gsof mal i gnancy + pal pabl eabnormal i ty...............
100%
(only3% of cancerspresentthis way)
2- elassicmammographic findingsof malignancy+ NO palpablefinding ..74%
(only6% of cancerspresentthis way)
3 . l n d e te rm i n a te ma mmo g ra p hifeatures c + pal pabl emass .......11%
4 . l n d e te rm i n a te ma s s+ n o p a l pabl efi ndi ng ................5%
5 . M a m m o g ra p h i c abl ley n i g nm a ss ..............2o/o
6. Asymmetricdensity(massquestionable) + clinicalfinding .......4%
7 . A s y m m e tridc e n s i ty(ma s sq u esti onabl e) + N o cl i ni calfi noi ng .................0%
8 . M i c ro c a l c i fi c a ti o+ncsl i n i c aal b normal i ty ............ ....25%
9 . M i c ro c a l c i fi c a ti o+nN s O c l i n i calabnormal i ty............ ..............2j %
(>3 punctateirregularmicrocalcifications in area <1 cm2)
1 0 . V e i nd i l a t a t i o.n. . . . . . . . ..................0%
11 . S k i nt h i c k e n i n g ..........0%
1 2 . D u c td i l a t a t i o.n. . . . . . . . ..................O"/"
552 RadiologyReviewManual

. Jewishwomen+ nuns F. MA MMOGR A P H IC FE A TU R E S

. . prominentduct pattern+ extremelydense
upper> lowersocialclass
. unmarried> marriedwomen
. Whites > Blacks after age 40
B . RE P ROD U C T IVE VA R IA BL E S
. nulliparous
> parous:
Relative Risk Compared with Nulliparous:
age at 1st pregnancY <19 Years 0.5
age at 1st pregnancy 20-30 Years
age at 1st pregnancy 30-34 years 1.0
age at 1st pregnancY >35 Years >1'0
. firstfull-termpregnancyafter age 35:.2x risk
. low parity> high paritY
. earlyage at menarche(<12Years):
relativerisk comparedwith onset of regular
ovulatorycycle:
Menarche<12 Menarche>12
3.7 1.6
immediately
1-4 years 2.3 1.6
. late age at menopause:
relativerisk comparedwith menopause
before age 44 years:
2.0
naturalmenopause>55 years of age
. early bilateralooPhorectomY:
relativerisk comparedwith menopause
between ages 45-49 Years:
artificialmenopauseat 50-54 years 1'34
artificialmenopausebeforeage 45 0.77
C. M ULT I PL EPR IM AR YC A N C ER S
. 4-5 x increasein risk for cancerin contralateral
breast
. increasedrisk after ovarian+ endometrial
cancer
D. FAMILYHISTORY
. breastcancerin first-degreerelative
Relativerisk comparedwith negativefamily Hx:
(+) for mother 1.8
2.5
(+) for sister
(+) for mother+ sister 5.6
. 25o/oof patientswith carcinomahave a positive
familyhistory
. carcinomatends to affectsuccessive
generations approx.10 yearsearlier
E . B E NI G NBR EA STD ISE AS E
. 2-4 x increasedrisk with atypicalhyperplasia:
relativerisk comparedwith no biopsy:
1.5
benignbreastdiseasein all patients
disease 0.9
nonproliferative
proliferativediseasewithoutatypia 1.6
3.5
f ibroadenoma+ hyPerPlasia
atypicalduct hyperPlasia(ADH)
4.4
no familyhistoryof breastcancer
familyhistoryof breastcancer 8.9
breasts accordingto Wolfe classification
N1 (0.14/"), P1 (0.52%),P2 (1-95%),DY
(5.22%)
G. RADIATIONEXPOSURE
excessriskof 3.5-6 casesper 1,000,000women
per year per rad after a minimumlatentperiodof
10 years(atomicbomb,fluoroscopy during
treatmentof tuberculosis, for
irradiation
postpartummastitis)
H . GE OGR A P H Y
. Western+ industrializednations(highest
incidence)
. Asia, LatinAmerica,Africa(decreasedrisk)
BreastCancerEvaluation
A. PRIMARY= LOCALIZINGSIGNSOF BREAST
CANCER
1. Dominantmassseen on two viewswith
(a\ spiculation = stellate/ star-burstappearance
(= fine linearstrandsof tumorextension+
desmoplastic response);"scirrhus"causedby:
(1) infiltratingductalcarcinoma(75% of all
invasivecancers)
(2) invasivelobularcarcinoma(occasionally)
/
{ massfeelslargerthan its mammographic
sonograPhic size
DDx: priorbiopsy/ trauma/ infection
(b) smooth border
(1) intracystic carcinoma(rare): subareolar
area; bloodYasPiration
(2) medullarycarcinoma:softtumor
(3) mucinousi colloidcarcinoma:softtumor
(4) PaPillarY carcinoma
r/ 'lelltale"signs: lobulation, smallcomettail,
flatteningof one side of the lesion,slight
irregularitY
^/ halo sign (= Mach band) may be present
DDx: cyst (sonographicevaluation)
(c) lobulation
(only
Appearancesimilarto fibroadenoma
characteristic may
calcifications exclude
malignancY)
0 The likelihoodof malignancyincreaseswith
numberof lobulations
. clinicalsize of mass > radiographic size
(Le Borgne'slaw)
2. Asymmetricdensity= star-shapedlesion
r/ distinctcentraltumor mass with volumetric
ratherthan planarappearance(additional
coned compressionviews!)
r/ denserrelativeto other areas
(= vessels+ trabeculaecannotbe seen within
high-densitY lesion)
./ fat does not traversedensity
{ coronaof sPicules
 BreastDisorders 553

r/ in any quadrant(but fatty replacementoccurs ! associatedwith ductalcalcifications

toward
last in upperouter quadrant) the nipple
DDx: postsurgicalfibrosis,traumaticfat DDx: nippleeczema
necrosis,sclerosingduct hyperplasia (c) nippledischarge
3. Microcalcifications . spontaneouspersistentdischarge
Associatedwith malignantmass by mammogram . need not be bloody
in 40/", pathologically with specialstainsin 60%, DDx: lactationaldischarge
on specimenradiographyin g6% 5. Abnormalveins
0 20% of clusteredmicrocalcifications represent venousdiameterratioof >1.4:1in 7Sy"ot
a malignantprocess! cancers; late sign + thus not very important
(a) shape: fragmented,irregularcontour, 6. Axillarynodes (signof advanced/ occultcancer)
polymorphic,castingrod-shapedwithout ! >1.5cm withoutfattycenter
polarity,Y-shapedbranchingpattern,granular DDx: reactivehyperplasia
"salt
and pepper"pattern,reticularpattern
(b) density; variousdensities LOCATIONOF BREASTMASSES
(c) size: 100-900 pm (usually);rarelyup to 2 mm benign+ malignantmassesare of similardistribution
(d) distribution;tight cluster over an area of @ upperouter quadrant(54%)
1 cm2or less is mostsuggestive; coursing @ upperinnerquadrant(14%)
alongductalsystemseen in ductalcarcinoma @ lowerouterquadrant(10%)
with comedoelements @ lower inner quadrant(7"/")
4. Architecturaldistortion @ retroareolar(15%)
due to desmoplasticreaction 0 Mediolateralobliqueview is importantpart of
r/ raggedirregularborder screeningbecauseit includeslargestportionof
DDx: postsurgicalfibrosis breasttissue+ considersmost commonlocationof
5. Intervalchange cancers!
(a) neodensity= de novo developingdensity(in
6% malignant) MetastaticBreastCancer
(b) enlargingmass (malignantin 10-15%) @ Axillary
lymphadenopathy
6. Enlargedsingleduct lncidence: 40-74/"
(low probabilityfor cancerin asymptomatic Riskfor positivenodes: 30% if primary>1 cm,
woman with normalbreastpalpation) 15o/"rt primary<1 cm
r/ solitarydilatedduct >3 cm long @ Bone
DDx: inspissateddebris/ blood,papilloma @ Liver
7. Diffuseincreasein density(latefinding) lncidence: 48-60/"
Cause: (1) pluggingof dermallymphatics with US: r/ hypoechoic(83%)/ hyperechoic (17%)
tumorcells masses
(2) less flatteningof sclerotic+ fibrous
elementsof neoplasmin comparison Screeningof Asymptomaticpatients
with morecompressible Definition of screening (World Health Organization):
fibroglandularbreasttissue A screeningtest must
B. SECONDARY= NONLOCALIZ]NG SIGNSOF (a) be adequatelysensitiveand specific
BREASTCANCER (b) be reproduciblein its results
1. Asymmetricthickening (c) identifypreviouslyundiagnoseddisease
2. Asymmetricducts,especiallyif discontinuous (d) be affordable
with subareolararea (e) be acceptableto the public
3. Skinchanges (f) includefollow-upservices
(a) retraction= dimplingof skin Guidelines of American Cancer Society, American
from desmoplasticreactioncausing College of Radiology, American Medicat Association,
shorteningof Cooperligaments/ direct National Cancer lnstitute:
extensionof tumorto skin 1. Breastself-examination to beginat age 20
DDx: trauma,biopsy,abscess,burns 2. Breastexaminationby physicianevery 3 years
(b) skin thickeningsecondaryto blocked between 20-40 years, in yearly intervalsafter age
lymphaticdrainage/ tumor in lymphatics 40
. peaud'orange
3. Baselinemammogrambetweenage 35-40;
DDx: normalin inframammary region follow-upscreeningbased upon parenchymal
4. NippleI areolarabnormalities pattern+ familyhistory
(a) retraction/ flatteningof nipple 4. Initialscreeningat 30 years if patienthas
DDx: normalvariant first-degreerelativewith breastcancerin
(b) Pagetdisease= ecZeffidtoidappearanceof premenopausalyears;follow-upscreeningbased
nipple+ areolain ductalcarcinoma upon parenchymalpattern
554 Radiology Review Manual
5. Mammographyat yearlyintervalsafter age 40
6. All womenwho have had priorbreastcancer
requireannualfollow-uP
Additionalrecommendations : 50-74 years
1. Screeningal2-year intervalsfor women
>70 years
2. Baselinemammogram10 yearsearlierthan age
of mother/ sisterwhen theircancerwas
diagnosed
Rate of detected abnormalities
30 abnormalities in 1,000screeningmammograms:
20-23 benignlesions
7-10 cancers
Acceptable recall rate for screening examination:
1Tohtor initialprevalencescreening;
5o/"tor subsequentincidencescreening
lnterval cancers:
10-20%of cancerssurfacebetweenannualscreenings
Roleof Mammography
Valueof ScreeningMammograqhy
lndication:
decreasein cancermortalitythroughearlier
detection+ intervention when tumorsize small
+ lymphnodesnegative;tumorgradeof no
prognosticsignificance in tumors<10 mm in size
1. HealthInsurancePlan(HlP) 1963-1969
randomizedcontrolledstudyof 62,000women
aged 40-64
. 25-30"/oreductionin mortalityin women
>50 years (followedfor 18 Years)
. 25o/"reductionin mortalityin women 40-49
years(followedfor 18 years);no significant
effectat 5- and 1O-Year follow-uP
. 19y"of cancersfoundby mammography alone
. 61"/"of cancersfound at physicalexamination
. effectivenessof screening<50 years of age is
uncertain
2. BreastCancerDetectionDemonstrationProject
( B CDD P )1 e 7 3 -1 9 8 0
4,443 cancersfound in 283,000asymptomatic
volunteers
. 41.6V"of cancersfound by mammography
alone (77"/.with negativenodes)
. 8.7"/"of cancersfound by physicalexamination
alone
. 59o/oof noninfiltrating cancersfound by
mammographyalone
. 25/" of cancerswere intraductal(vs. 5% in
previousseries)
. 21"hof cancersfound in women aged 4049
years (mammographyalonedetected35.4%)
. 51"/oof cancersfound with both mammography
+ physicalexamination
3. Two-countySwedishtrial 1977-1990
randomizedcontrolledstudyof 78,000womenin
studygroup+ 56,700in controlgroupaged
40-74 years
(a) singleMLO mammogramat2-year intervalsfor
women <50 years of age
(b) singleMLO mammogramat 3-yearintervalsfor
women >50 Yearsof age
. 4O/oreductionin mortality^7 yearsin women
. O/oreductionin mortalityat7 yearsin women
4049 years
4. Metaanalysisof combinedresultsof 5 Swedish
trialsfor women aged 39-49
. 2go/oreductionin breastcancermortalitywith
screeningmammogramsofferedat intervals
from 18 to 28 months
OCCULTVERSUSPALPABLECANCERS
27"/" are occultcancers(NO age difference)
Positiveaxillarynodes: occultcancers(19%);
palpablecancers(44%)
1O-yearsurvival: occultcancers(65%);patpable
cancers (25%)
Overall detection rate:
58-69% ;8% rt < 1 cm i n si ze
Mammographic accuracY:
887o correctlydiagnosedby radiologist
27"/" detectedonly by mammography
8% misinterPretations
4/o notdetected
15-30% positivepredictivevalue(nationalaverage):
25% PPV for women in 5th decade;50%
PPV for womenin 8th decade
MammographicallYMissedCancers
False-negative screeningmammogram= pathologic
diagnosisof breastcancerwithin 1 year after negative
mammogramwith the followingtypesof misses:
(a) lesioncould not be seen in retrospect(25-33%)
= "acutecancer"= c€lrlc€Isurfacingin screening
interval
(b) cancerundetectedby first readerbut correctly
identifiedbY secondreader(14%)
(c) visiblein retrospecton prior mammogram(61%)
Incidence: approx.4-15-34/" of all cancers;
approx.3 cancers:2,000 mammograms;
5-15-22/" of palpablebreastcancers
0 A secondreaderwill detectan additional5-15/" ot
cancers!
Cause:
1. lnterpretationerror (52%):
(a) benignappearance(18%): medullary
carcinoma,colloidcarcinoma,intracystic
ductal
papillarycarcinoma,some infiltrating
carcinomas
(b) presenton previousmammogram(17%)
(c) seen on one view onlY(9%)
(d) site of PreviousbioPsY(8%)
2. Observererror (30-43%):
(a) overlooked
(b) presenceof an obviousfindingleadsto
overlooking of a moresubtlelesion
= "satisfiedsearch"Phenomenon

(c) no knowledgeof clinicalfinding
(d) rushedinterpretation
(e) heavycaseload
(f) extraneousdistraction
(g) eye fatigue
(h) inexperience
3. Technicalerror(5%):
(a) inadequateradiographictechnique:
improperpositioni ng, inadequate
compression,under-/ overexposedimage,
poor screen-filmcontact,geometricmotion
blurring
(b) failureto image regionof interest
(c) suboptimalviewingconditions:inadequate
luminanceof view boxes,extraneousview
box light,high ambientroom light
4. Tumorbiology:
(a) smalltumorsize
(b) failureto incitedesmoplasticreaction
(eg,invasivelobularcarcinoma)
(c) limitations of screen-filmmammography in
physicallydense breasts
(d) no associatedmicrocalcifications
(approx.SIy" of cancers)
(e) developingsoft-tissueradiopacity
(f) stabilityof mammographic findings
0 Malignantcalcificationsmay be stablefor
up to 63 months
0 A mass may not changefor up to 4.5
years
Locationof missedcancers:
retroglandular area (33%),lateralparenchyma
(31%),central(18%),medial(13%),subareolar
(4%)
Radiation-induced Breast Carcin oma
0 Lifetimerisk with cumulativecarcinogeniceffect
relatedto age!
(a) women age <35: 7.5 additionalcancersper
1 millionirradiatedwomen per year per rad
(b) womenage >35: 3.5 additionalcancersper
1 millionirradiatedwomen per year per rad
Roleof BreastUltrasound
lndications:
0 Ultrasoundis no screeningtool!
A. TARGETEDEXAM
(1) Initialstudyof palpablelump in patient
<30 years of age / pregnant/ lactating
0 Ultrasoundwill not add usefulinformationin
an area that containsonly fattytissueon a
mammogram!
(2) Characterization of mammographic/ palpable
mass as fluid{illed/ solid
0 Ultrasoundwill add usefulinformationif there
is water-densitytissuein the area of
palpableabnormality!
0 Differentiationof cysticfrom solid lesionis
the principalroleof ultrasound!
BreastDisorders SSs
(3) additionalevaluationof nonpalpable
abnormality with uncertainmammographic
diagnosis
(4) searchfor focal lesionas cause for
mammographicasymmetricdensity
(5) confirmationof lesionseen in one
mammographicprojectiononly
B. WHOLE-BREASTEXAM
(1) Breastsecretions
(2) Suspectedleaksfrom siliconeimplant
(3) Follow-upof muftipfeknown mammographic/
sonographiclesions
(4) Radiographically dense breastwith strong
familyhistoryof breastcancer
(5) Metastasesthoughtto be of breastorigin,but
with negativeclinical+ ffiomffiographicexam
(6) Mammographynot possible: ,,radiophobic"
patient,bedriddenpatient,after mastectomy
C . IN TE R V E N TION APLR OC E D U R E
(1) Ultrasound-guided cyst aspiration
(2) Ultrasound-guided core biopsy
(3) Ultrasound-guided ductography,if
(a) secretionscannotbe expressed
(b) duct cannotbe cannulated
Accuracy: 98o/oaccuracy tor cysts; ggo/oaccuracy for
solidmasses;smallcarcinomashavethe
leastcharacteristicfeatures
Roleof BreastMRI
lndications: ambiguousmammographicfindings;
positiveclinicalexamination+ negative
mammographic/sonographic f indings;
stagingprior to excisionalbiopsy;
assessmentof residualdiseaseafter
excision;axillarynode malignancywith
unknownprimarysite
Sensitivity: 72-99-100%
MR:
r/ rapid increasein signalintensityafter contrast
injectionreachinga markedlyhigheramplitudethan
parenchymaltissue,followedby a plateau+ early
washout
DDx: fibroadenomain premenopausalpatient,
ductalhyperplasiat atypia,lobularneoplasia,
inflammatory disease,scar <6 monthsold in
nonirradiated breast,scar <19 monthsold in
irradiatedbreast,fibrocysticchange
(apocrinemetaplasia, sclerosingadenosis)
,
! intenseearlyrim / peripheralenhancement
, (+ central necrosis)
r/ malignantmass margination
Roleof StereotacticBiopsy
lndications:obviouslymalignantnonpalpablelesion,
ndeterminate likelybenignlesion,
anxietyover lesion
Types: well-definedsolid mass, indistinct/ spiculated
mass,clusteredmicrocalcifications
Advantage: single-stagesurgicalprocedure
556 Radiology Review Manual
Problematic; 3-5-mm small lesion,fine scattered
microcalcifications, indistinctdensity,
area of architecturaldistortion
Excision:
radialscar suspected(in up to 28/" associatedwith
tubularcarcinoma),lesioncloseto chestwall,lesion
in axillarytail, very superficiallesion,atypia/ atypical
hyperplasia(in 49-61% associatedwith malignancy),
carcinomain situ (in 9-20% associatedwith invasion),
branchingmicrocalcifications suggestiveof DCISwith
comedonecrosis
Sensitivity; 85-99% with core needle biopsy
(100%specific),68-93% with fine-
needleaspiration(88-100% specific)
Missrate: 3-8% for stereotacticbiopsy,S/" for surgery
BREASTCYST
tncidence.' most commonsinglecause of breastlumps
between35 and 55 Yearsof age
Age: any; most commonin later reproductiveyears
+ aroundmenopause
Histo: cyst wall linedby singlelayerof
(a) flattenedepithelialcells; cyst fluidwith Na./ K* ratio
>3
(b) epithelialcellswith apocrinemetaplasia(secretory
function);cyst fluidwith Na./K*ratio<3
Cause: fluid cannotbe absorbeddue to obstructionof
extralobular terminalduct by fibrosis/ intraductal
epithelialProliferation
. size changesover time
SimpleBreastCyst
r/ well-defined flattenedoval/ round(if underpressure)
mammographic mass+ surroundinghalo
(DDx: well-defined solidmass)
{ solitary/ multiPle
{ needteaspirationof fluid (proof)+ postaspiration
mammogramas new baseline
US (98-100%accuracY):
0 Correlatewith palpation/ mammogramas to size,
shape,location,surrounding tissuedensity!
r/ spherical/ovoidlesionwith anechoiccenter
r/ well-circumscribed thin echogeniccapsule
./ posterioracousticenhancement(may be difficultto
demonstratein smalli deeplysituatedcysts)
i/ tnin edge shadows
r/ occasionally multilocular+ thin septations/ cluster
of cysts
PNEUMOCYSTOGRAPHY (for symptomaticcysts):
{ air remainsmammographically detectablefor up to
3 weeks
rl therapeuticeffectof air insufflation(equalto
60-70% of aspiratedfluidvolume): no cyst
recurrencein 85-94% (4045% cyst recurrence
withoutair insufflation)
ComplexBreastCYst
= ar'tycyst that does not meet criteriaof simplecyst
Cause: fibrocysticchanges(vast majority),infection,
malignancY (extremelY rare)
0 0.3% of all breastcancersare intracystic
0 Patientswith apocrinecysts are at greater risk to
developbreastcancer!
{ uniformlythickwall + tenderness= inflammation /
infection
"foam"cyst)
./ diffuselow-levelinternalechoes(=
(a) with mobilityupon increasein power output
= subcellularmateriallike proteinglobs,floating
cholesterol crystals,cellulardebris
(b) withoutmobilityupon increasein poweroutput
= cellslikefoamymacrophages, apocrine
metaplasia, epithelialcells,pus, blood
r/ ftuid-debris level
Hx: aspirationto rule out blood/ pus
./ tnicf septation/ eccentricwall thickening
furthercharacterized by protrudingill-definedouter
margin,convexmicrolobulated innermargin("mural
nodule"),nonmobile mass with coarse heterogeneous
echotexture, CD flow withinthickening
Rx: treatedlikesolidnodule
r/ spongelikeclusterof microcysts
Rx: treatedlike solid nodule
Rx: completeaspiration(assuresbenigncause),core
needlebiopsy(if partially/ nonaspiratable)
DDx: artifactualscatterin superficial/deep smallcysts,
fibroadenoma, papilloma,carcinoma
CYST ASPIRATION
. inspectionof cYstfluid:
(a) normal:turbidgreenish/ grayish/ blackfluid
(b) abnormal: straw-coloredclear fluid / dark blood
^/ needlemoveswithinnonaspiratable complexcyst
r/ ttuiOwithoutbloodshouldbe discarded
.l Uloodyfluid shouldbe examinedcytologically
CARCINOMA OF MALE BREAST
lncidence: O.2o/o,1 ,400 new caseslyearwith 300 deaths;
0 9.7% of male breastcarcinomasoccur in
men with KlinefeltersYndrome!
Peak age: 60-69 Years
At risk: (maleswith increasedestrogenlevels)
1. Klinefelter syndrome(20-foldriskover normals):
XXY chromosomes
2. Liverdisease:cirrhosis,schistosomiasis, malnutrition
3. RadiationtheraPYto chest
4. Occupational heatexposure(diminished testicular
function)
5. Testicularatrophy:injury,mumps-orchitis,
undescended testis
6. Jewishbackground
7. FamilyhistorY
0 Gynecomastiais NOT a risk factorl
Histo: infiltratingductalcarcinoma
. firm painlessretroareolar/ upper-outer-quadrant mass
. breastswelling,bloodynippledischarge,retraction
Location: L > R breast;bilaterality is uncommon
{ resembles scirrhouscarcinoma of femalebreast
{ usuallylocatedeccentricallY
 BreastDisorders 557

! calcificationsfewer + more scattered+ more round DERMATOPATH

IC LYMPHADENOPATHY
+ larger = benignreactivelymphadenopathy
r/ enlargedaxillarynodes(in 50% at time of presentation) withinbreast
associatedwith cutaneousrashes
r/ metastasesto pleura,lung, bone, liver
Cause: exfoliativedermatitis,erythroderma,psoriasis,
Delayin diagnosisfrom onsetof symptoms: 6-1g months
atopicdermatitis, skin infection)
Rx: surgery,hormonalmanipulation (95%estrogen Histo: follicularpatternretained,germinalcenters
receptorand 75/" progesteronereceptorpositive) enlarged,enlargedparacortic al areawithpale-
Prognosis; S-yearsurvival rate for stage 1 = g2-1OO/o,
stainingcells(lymphocytes, Langerhanscells,
for stage 2 = 44-77o/o,tor stage B = 16-45"/o, interdigitatingreticulumcells)
for stage4 = 4-8h (sameas for women!) . mobilenontenderfirm subcutaneous nodules
DDx: breastabscess,gynecomastia,epidermalinclusion Location: oftenbitateral
cyst Site: predominantly upperouterquadrant
r/ regionalsubcentimet,er masseswith centrali peripheral
CHRONIC
ABSCESS
OF BREAST radiolucentnotches
= COLDABSCESSusuallyseen in lactatingwomen
. fever,pain, increasedWBC (clinicaldiagnosis)
. rapidresponseto antibiotics EPIDERMAL INCLUSION CYST
Location: mostcommonlyin central/subareolararea = benign
cutaneous
/ subcutaneous
lesion
./ itt-detined
massof increaseddensitywith flamelike Cause: congenital,metaplasia, trauma(needlebiopsy,
contour reductionmammoplasty), obstructedhairfollicle
./ secondarychangescommon: architecturaldistortion, Path: cyst filledwith keratin
nipple+ areolarretraction,lymphedema, skin Histo: stratifiedsquamousepithelium
, thickening,pathologicaxillarynodes . smoothroundnoduleattachedto skin with blackened
! liquefiedcentercan be aspirated pore,movableagainstunderlyingtissue
US: r/ circumscribedround/ oval iso- I hign-densitymass of
r/ anechoic/ nearlyanechoicarea with posterior 0.8-10.0cm i n di ameter
enhancement r/ may containheterogeneousmicrocalcifications
US:
CYSTOSARCOMA PHYLLOI DES { circumscribed hypoechoicsolidmassextendinginto
= GIANTFIBROADENOMA = ADENOSARCOMA dermis
= PHYLLODE TUMOR DDx: sebaceouscyst (epithelialcysts containing
= usuallybenigngiant form of intracanalicular sebaceousglands)
fibroadenoma
lncidence: 1: 6,300examinations; 0.3-1.5%of all FATNECROSIS
OF BREAST
breasttumors;3/" of all fibroadenomas = TBAUMATIC LIPIDCYST= OILCYST
Age: 5th-6th decade(meanage of 45 years, = dsepticsaponificationof fat by tissuelipaseafter local
occasionallyin women <20 years of age destructionof fat cells with releaseof lipids
Histo: similarto fibroadenomabut with increased + hemorrhage+ fibroticproliferation
cellularity+ pleomorphism (widevariationsin size, Etiology: directexternaltrauma(seatbelt injury),breast
shape,differentiation) of its stromalelements; biopsy,reductionmammoplasty, implant
fibroepithelialtumorwith leaflike(phylloides) removal,breastreconstruction, irradiation,
growthpattern= branchingprojectionsof tissue
nodularpanniculitis (Weber-Christian disease),
into cysticcavities;cavernousstructurescontain ductalectasiaof chronicmastitis
mucus;cysticdegeneration+ hemorrhage lncidence: 0.5o/"of breast biopsies
. rapidlyenlargingbreastmass;periodsof remission
At risk: middle-agedobesewomen with fatty pendulous
. sense of fullness
. huge,firm,mobile,discrete,lobulated,smoothmass breasts
. discoloration Histo: cavitywith oily materialsurroundedby ,1oam
of skin,wide veins,shiningskin cellS"(= lipid-laden macrophages)
^/ largenoncalcifiedmass with smoothpolylobulated . historyof trauma in 40/" (eg, prior surgery,radiation
marginsmimickingfibroadenoma >6 monthsago, reductionmammoplasty, lumpectomy)
! rapidgrowthto largesize (>6-8 cm), may fill entirebreast . usuallyclinicallyoccult
US: . firm,slightlyfixedtender/ painlessmass
./ ttuid-titted
cleftsin largetumors . skin retraction(50%)
Prognosis; limitedinvasionfrequentlyseen; 1S-ZO% . yellowishfatty fluid on aspiration
recurrencerate if not completelyexcised Location: anywhere;morecommonin superficial
Cx: in 5-10% degeneration into malignantfibrous periareolarregion;near biopsysite/ surgical
histiocytoma/ fibrosarcoma/ liposarcoma/ scar
chondrosarcoma / osteosarcomawith localinvasion r/ ill-definedirregularspiculateddense mass
+ hematogenous metastasesto lung,pleura,bone (indistinguishable from carcinomaif associatedwith
(axillarymetastasesquite rare) distortion,skin thickening,retraction)
558 RadiologyReviewManual

{ well-circumscribed mass with translucentareas at (a) peripheralsubcapsularmyxoiddegeneration

center(= homogeneousfat densityof oil cyst) { peripheralmarginalringlikecalcifications
surroundedby thin pseudocapsule (in old lesions) (b) centralmyxoiddegeneration
r/ calcifiesin 4-7"h (= liponecrosis macrocystica { "popcorn"type of calcification
(PATHOGNOMONIC)
calcificans): (c) calcificationswithinductalelements
{ occasionally curvilinear/ eggshellcalcificationin wall { pleomorphiclinear+ branchingpattern
r/ fine spiculesof low densityvary with proiection 0 Calcifications enlargeas soft-tissuecomponent
r/ localizedskin thickening/ retractionpossible regresses!
US: US:
i/ nypo-/ anechoicmasswith ill-/ well-defined margins r/ round(3%) loval mass (96%)with length-to-depth
+ acousticshadowing ratioof >1.4 (in carcinomasusually<1.4)
{ complexcystwith muralnodules/ echogenicbands r/ hypoechoicsimilarto fat lobules(80-96%)/
hyperechoici mixedpattern/ anechoic/ isoechoic
ristian Disease
Weber-Ch comparedwith adjacentfibroglandulartissue
= nonsuppurative
panniculitis
withrecurrent
boutsof ri homogeneous(48-89%)/ inhomogeneous(12-52/")
= areasof fat necrosis,involving
inflammation texture
subcutaneousfat + fat withininternalorgans { regular(57%)/ lobulated(15-31"/")I irregular
. accompaniedby fever+ nodulesover trunkand limbs (6-58%) contour
{ "humpand dip" sign = smallfocalcontourbulge
immediately contiguouswith a smallsulcus(57%)
FIBROADENOMA r/ intratumoralbrightechoes(10%)
= ADULT-TYPE FIBROADENOMA = ITr?cfoc€rlcifications
= estrogen-induced benigntumororiginating from TDLU; r/ posterioracousticenhancement(17-25%)/ acoustic
formsduringadolescence;pregnancy+ lactationare shadowwithoutcalcifications(9-1 1%)
growthstimulants;regressionaftermenopause(mucoid ^/ echogenichalo (capsule)with lateralshadowing
degeneration,hyalinization,involutionof epithelial MR:
components, calcification) ./ internalseptations
lncidence: 3rd most common type of breast lesion after
fibrocysticdisease+ carcinoma;most Juvenile/ Giant/ CellularFibroadenoma
commonbenignsolidtumorin womenof = fibroadenoma >5 cm in diameter/ weighing>500g
childbearing age Cause: hyperplasia+ distortionof normalbreast
Age: mean age of 30 years (range13-80 years); lobulessecondaryto hormonalimbalances
medianage 25 years;most commonbreasttumor betweenestradiol+ progesteronelevels
underage 25 years Age: any (mostlyin adolescentgirls)
Hormonal influence: Histo: more glandular+ more stromalcellularitythan
slightenlargementat end of menstrualcycle+ during adulttype of fibroadenoma;ductalepithelial
pregnancy;regressesafter menopause;may occur in hyperplasia
postmenopausal women receivingestrogen . rapidlyenlargingwell-circumscribednontendermass
replacementtherapy . dilatedsuperficialveins,stretchedskin
Histo: mixtureof proliferatedfibrousstroma+ epithelial r/ discretemass with roundedborders
ductalstructures DDx: medullary/ mucinous/ papillarycarcinoma/
(a) intracanalicular fibroadenoma compressing ducts carcinomawithinfibroadenoma
(b) pericanalicularfibroadenoma withoutduct
compression CHANGES
FIBROCYSTIC
(c) combination = MAZOPLASIA= MASTITISFIBROSACYSTICA= CHRONIC
. firm,smooth,sometimeslobulated,freelymovablemass CYSTICMASTITIS= CYSTICDISEASE= GENERALIZED
BREASTHYPERPLASIA = DESQUAMATED EPITHELIAL
. in 35% not palpable = MAMMARY
HYPERPLASIA = FIBROADENOMATOSIS
. NO skinfixation DISEASE= FIBROUS
DYSPLASIA= SCHIMMELBUSCH
. rarelytender/ painful MASTITIS= MAMMARYPROLIFERATIVE DISEASE
. clinicalsize = radiographic size 0 Not a diseasesincefoundin72/" of screening
Size: 1-5 cm (in 60%);multiplein 15-25'/.;bilateralin 4'/. population>55 years of age
r/ circular/ oval-shapedlesionof low density 0 The Collegeof AmericanPathologistssuggestsuse of
r/ nodular/ lobulatedcontourwhen larger(areaswith the term "fibrocysticchanges/ condition"in
differentgrowthrates) mammography reports!
{ smooth,discretemargins(indistinguishable from cysts lncidence; most commondiffusebreastdisorder;in 51"/"
whens m all) of 3,000autopsies
{ oftenwith "halo"sign Age: 35-55 years
r/ smoothlycontouredcalcificationsof high + fairlyequal Etiology: exaggerationof normalcyclicalproliferation
densityin 3/" due to necrosisfrom regressivechanges + involutionof the breastwith production
in olderpatients: + incompleteabsorptionof fluid by apocrinecells
 BreastDisorders 559

. asymptomaticin macrocysticdisease r/ "involutionaltype" calcifications= Veryfine punctate

. fullness,tenderness, pain in microcysticdisease calcifications evenlydistributed withinone / more lobes
. palpablenodules+ thickening againsta fatty background(from mild degreeof
. symptomsoccurwith ovulation;regressionwith hyperplasia in subsequently atrophiedglandulartissue)
pregnancy+ menopause US:
Histo: r/ ductalpattern,ductectasia,cysts,ill-definedfocal
(1) overgrowthof fibrousconnectivetissue= stromal lesions
fibrosis,fibroadenoma Risk for lnvasive Breast Carcinoma
(2) cysticdilatationof ducts + cyst formation(in 100% A . N O IN C R E A S E D R IS K
microscopic,in 20/" macroscopic) 1. Nonproliferative lesions:adenosis,floridadenosis,
(3) hyperplasia of ducts+ lobules+ acini= adenosis; apocrinemetaplasiawithoutatypia,macro-/
ductalpapillomatosis microcysts,duct ectasia,fibrosis,mild hyperplasia
(morethan 2 but not morethan 4 epithelialcells
^/ individualround/ ovoidcysts with discretesmooth deep),mastitis,periductalmastitis,squamous
m ar gins metaplasia
{ lobulatedmultilocular cyst 2. Fibroadenoma
r/ enlargednodularpattern(= fluid-distended lobules B . S LIGH TLYIN C R E A S E D R | S K(1.5-2ti me s) :
+ extensiveextralobularfibrousconnectivetissue 1. Moderate+ floridsolidi papillaryhyperplasia
, overgrowth) 2. Papillomawith fibrovascular core
! "teacup-like"curvilinear thin calcifications
with horizontal 3. Sclerosingadenosis
beam + low-densityroundcalcificationsin craniocaudal C . MOD E R A TE LY IN C R E A S E D R | S K(5 ti mes) :
projection= milk of calcium(4%) Ductal/ lobularatypicalhyperplasia (borderline
r/ "oysterpearl-like"/ psammoma-likecalcifications lesionwith some featuresof carcinomain situ)
D . H l c H R I S K( 8 - 1 1t i m e s ) :
1. Atypicalhyperplasia + familyhistoryof breast
cancer
2. Ductal/ lobularcarcinomain situ

Adenosis
Path: lobulocentric lesionderivedfrom TDLUwith
distortionand effacementof underlyinglobules
Histo: epithelialand myoepithelialproliferationof
sclerosinsadenosis
ductules+ lobuleswith nuclearpleomorphism
+ increasein cell size
^/ increasein size of TDLUsto 3-7 mm
{ "snowflakepattern"of widespreadill-definednodular
densities
r/ adenosislobulesare sonographically iso-to mildly
, hypoechoiccomparedwith fat
ri calcificationsless common+ extensivethan in
sclerosingadenosis

blunt duct adenosis

SclerosingAdenosis
fibrocystic change
Benign Diseasesof the Terminal Ductal Lobular Unit
Path: myoepithelialproliferation+ reactivestromal
fibrosis
Histo: stromalsclerosisinvolving>50% of all
TDLUs,whichbecomeelongated+ distorted
+ compressedby sclerosis
DDx: tubularcarcinoma(absenceof
basementmembrane+ myoepithelial
cells);radialscar (moreextensive
fibrosis+ centralfibrocollagenousscar)
. palpablemass (rare)= "adenosistumor"
Rarelyassociatedwith: lobularcarcinomain situ >
ductafcarcinomain situ
0 Sclerosingadenosisis not a risk factor/ precursorof
breastcancer!
560 Radiology Review Manual

{ calcificationsin 50% Cause: ? abruptsuppressionof lactation/ obstructed

(a) focal form milkduct
./ focal clusterof microcalcifications Age: occursduring/ shortlyafter lactation
{ focallydense breastappearingas a nodule/ . thickinspissated milkyfluid(colostrum)
spiculatedlesion Location: retroareolararea
(b) diffuseform ^/ largeradiopaquelesionof waterdensity(1stphase)
{ adenosis+ diffuselyscatteredcalcifications { smallerlesionof mixeddensity+ fat-waterlevelwith
(calcifications
in cysticallydilatedacinarstructure) horizontalbeam (2ndphase)
r/ diffuselydense breast { smallradiolucent lesionresemblinglipoma
DDx: other spiculatedlesions i/ + fluid-calcium
level
US:
Fibrosis ./ complexmass
with smooth
r/ round/ oval clusteredmicrocalcifications Dx: aspirationof milkyfluid
contours+ associatedfine granularcalcifications
fillinglobules
CELLTUMOR
GRANULAR
AtypicalLobularHyperplasia = GRANULAR CELLMYOBLASTOMA OF BREAST
= proliferationof roundcells of LCIS type growingalong = benigntumor,occasionally locallyinvasive
terminalducts in permeativefashion(pagetoidgroMh) + metastasizing
betweenbenignepithelium+ basalmyoepithelium Origin: ? Schwanncell,smoothmuscle,or
BUT NOTcompletely obliteratingterminalductallumina undifferentiated mesenchymalcelI
/ lobules
distending (as in lobularcarcinomain situ) Prevalence: 1:1,000primarybreastcarcinomas
, Age : 20-59 (mean35) years; more commonin Blacks
r/ no mammographiccorrelate
Histo: roundedgroupsof largecellswith smalldark
regularnuclei+ abundanteosinophilic granular
AtypicalDuctalHyperplasia
= low-gradeintraductalproliferationwith partial/ cytoplasm;not immunoreactive to cytokeratin
incompletelydevelopedfeaturesof noncomedoDCIS + epithelialmembraneantigenBUT to S-100
protein
r/ frequentcalcifications
DDx: carcinoma,lymphoma,metastasis
0 Fine-needle aspiratemay be difficultto
IntraductalPapillomatosis
= hyperplastic interpret!
polypoidlesionswithina duct
Location: tongue,skin,bronchialwall,subcutaneous
Age: perimenopausal
. spontaneousbloody/ serous/ serosanguinous breasttissue (6-8%)
nipple
Site: morecommonlyotherthan upperouterquadrant
discharge(mostcommoncauseof nippledischarge) . asymmetriclumpwith slow growth,hardness,skin
r/ small retroareolaropacity(= dilatedduct) extending
fixation/ retraction,ulceration
2-3 cm into breast . oftenfixedto pectoralisfascia
r/ intraluminalfillingdefecton galactography
r/ well-circumscribed spiculatedmass 1-3 cm in diameter
r/ stellateextensions(tumorinsinuating itselfinto
surroundingbreasttissue)
FIBROUS OFTHEBREAST
NODULE r/ may exhibitacousticshadow
= FIBROUS DISEASE OFTHEBREAST = FIBROUS DISEASE Rx: wide localexcision
= FIBROSISOFTHEBREAST = FIBROUS MASTOPATHY
= FIBROUS TUMOROFTHEBREAST
Frequency: 34/" of benignmasses;8/" of surgical
breastspecimens
Histo: focallydense collagenousstromasurrounding GYNECOMASTIA
= eXCeSSive developmentof breastin male
atrophicepithelium; NONSPECI FIC
Age: 20-50 years;only 8% postmenopausal Cause:
. palpable/ nonpalpable mass: edge mergesinto (1) H ormonal
(a) neonate:influencefrom maternalestrogens
surroundingdensetissue
Location: unilateral(80-85%)/ bilateral(15-20%) crossingplacenta
./ circumscribed(55%)/ indistinct(32%) margin (b) puberty: highestradiollevels
{ suggestiveof malignancy(11%): irregularshape, lncidence; in up to 60-75/" of healthy boys
spiculatedmargin,posterioracousticshadowing Age: 1 year after onset of puberty(13-14 years)
DDx: fibroadenoma,malignancy Prognosis: subsideswithin 1-2 years
(c) oldermen: declinein serumtestosterone levels
(d) hypogonadism (Klinefeltersyndrome,anorchism,
GALACTOCELE acquiredtesticularfailure(eg,testicularneoplasm)
= retentionof fatty materialin areasof cysticduct (e) tumors: adrenalcarcinoma,pituitaryadenoma,
dilatationappearingduring/ shortlyafter lactation testiculartumor, hyperthyroidism

(2) Systemicdisorders
advancedalcoholiccirrhosis,hemodialysis
renalfailure,chronicpulmonarydisease
(emphysema, TB), malnutrition
(3) Drug-induced
anabolicsteroids,estrogentreatmentfor prostate
cancer,digitalis,cimetidine, thiazide,spironolactone,
reserpine,isoniazid,ergotamine,marijuana
(4) Neoplasm: hepatoma(withestrogenproduction)
(5) ldiopathic
mnemonic.' "CODES"
Cirrhosis
Obesity
Digitalis
Estrogen
Spironolactone
lncidence: 85h of all male breast masses
Age: neonatalperiod,adolescentboys (4O/"),
men >50 years (32%)
Histo: increasednumberof ducts,proliferationof duct
epithelium,periductaledema,fibroplastic
adiposetissue
. palpablefirm mass>2 cm in subareolarregion
Location: bilateral(63%),left-sided(27%),right-sided
(10"/")
r/ mildprominenceof subareolarductsin flame-shaped
, distribution(focaltype)
{ homogeneouslydense breast(diffusetype)
DDx: pseudogynecomastia (= fs11yproliferationin
obesity)
HAMARTOMA OF BREAST
= FIBROADENOLIPOMA = LIPOFIBROADENOMA
= ADENOLIPOMA
lncidence: 2-16:10,000mammograms
Mean age: 45 (27-88) years
Histo: normal/ dysplasticmammarytissuecomposedof
densefibroustissue+ variableamountof fat,
delineatedfrom surrounding tissuewithouta true
capsule
. soft, often nonpalpable(60%)
Location: retroareolar(30%),
, upperouterquadrant(95%)
r/ round/ ovoid well-circumscribed mass usually> 3 cm
r/ mixeddensitywith mottledcenter(secondaryiotatl
= "sliceof sausage"pattern
r/ tnin smoothpseudocapsule (= thin layerof surrounding
fibroustissue)
{ peripheralradiolucentzone
{ may containcalcifications
DDx: liposarcoma,Cowdendisease
HEMATOMA
OF BREAST
Cause: (1) surgery/ biopsy(mostcommon)
(2)blunttrauma
(3)coagulopathy (leukemia,
thrombocytopenia)
(4) anticoagulanttherapy
{ well-definedovoid mass (= hemorrhagiccyst)
ill-defined
masswith diffuseincreaseddensity(edema
+ hemorrhage)
BreastDisorders 561
r/ adjacentskin thickening/ prominenceof reticular
in chronic structures
r/ regressionwithinseveralweeks leaving(a) no trace,
(b) architecturaldistortion,(c) incompleteresolution
r/ calcifications(occasionally)
US:
r/ hypoechoicmass with internalechoes
JUVENILE PAPILLOMATOSIS
Path: many aggregatedcysts with intersperseddense
stroma
Histo: cysts linedby flat duct epithefium/ epitheliumwith
apocrinemetaplasia, sclerosingadenosis,duct
stasis;markedpapillaryhyperplasiaof duct
epitheliumwith often extremeatypia
Mean age: 23 years (rangeof 1248 years)
. localizedpalpabletumor
. familyhistoryof breastcancerin Z8/" (affectedfirst-
degreerelativein 8%; in one / more relativesin 2g%\
Prognosis; developmentof synchronous(4%)/
stroma, metachronous(4%) breastcancerafter
8-9 years.
DDx: fibroadenoma
LACTATING
ADENOMA
= newlydiscoveredpainlessmassduring3rd trimesterof
pregnancyi in lactatingwoman
Etiology: ? variantof fibroadenoma/ tubularadenoma/
lobularhyperplasia or de novo neoplasm
Path: well-circumscribed yellowsphericalmass with
lobulatedsurface+ rubberyfirm textureand without
capsule
Histo: secretorylobuleslinedby granularand foamy to
vacuolatedcytoplasm+ separatedby delicate
connectivetissue
. firm freelymovablepainlessmass
r/ homogeneously hypoechoic/ isoechoicmass
1, posterioracousticenhancement(most)/ shadowing
r/ fibroussepta
Prognosis.' regressionaftercompletionof breastfeeding
DDx: breastcarcinoma(1:1,300-1:6,200pregnancies)
LIPOMAOF BREAST
= usuallysolitaryasymptomaticslow-growinglesion
Mean age: 45 years + postmenopause
. soft, freelymovable,well delineated
r/ usually>2 cm
r/ radiolucent lesioneasilyseen in densebreast;almost
invisiblein fatty breast
r/ discretethin radiopaqueline (= capsule),seen in most
of its circumference
./ displacementof adjacentbreastparenchyma
r/ calcificationwith fat necrosis(extremelyiare)
DDx: fat lobulesurroundedby trabeculae/ suspensory
ligaments
LYMPHOMA OF BREAST
A. PrimaryLymphoma
= extranodallymphomaof the breastwithoutprior
historyof lymphoma/ leukemia
562 Radiology Review Manual

Prevalence: 0.12-0.53o/"of all breastmalignancies; peripheryblendingwith normaltissue

2.2'/. of all extranodallymphomas ^/ multipteoften bilateraldense round/ oval calcifications
. asymptomatic with lucentcenter+ polarity(= orientation towardnipple)
B. SecondaryLymphoma (a) periductal
0 One of the most commontype of metastaticlesions ri oval / elongatedcalcifiedring arounddilatedducts
in the breast! withvery denseperiphery(surrounding depositsof
. fever,pain fibrosis+ fat necrosis)
Histo: B-cellNHL (majority), Hodgkindisease, (b) intraductal
"needle-shaped"
leukemia(CLL),plasmacytoma r/ tairtyuniformlinear,often
Age: 50-60 years; M < F calcificationsof wide caliber,occasionally
Location: right-sided predominance; 13% bilateral branching(withinducts/ confinedto ductwalls)
./ well/ incompletely circumscribed round/ oval lobulated r/ nippleretraction/ skinthickeningmay occur
mass / masses Sequelae: cholesterolgranuloma
^/ infiltratewith poorlydefinedborders DDx: breastcancer
./ ttO calcifications i spiculations
! skinthickening+ trabecularedema
ri bilateralaxillaryadenopathyin 30-50%
US: MAMMOPLASTY
r/ oval/ roundhomo-/ heterogeneously hypoechoic = COSMETICBREASTSURGERY
MASS / MASSES
r/ sharplydefined/ poorlydefinedborders
r/ posterioracousticshadowing/ enhancement AugmentationMammoPlastY
Prognosis: 3.4'/.S-yeardisease-freesurvivalfor all Mostfrequently plastic
performed in USA
surgery
stages;50% remissionrate with aggressive Frequency: 150,000proceduresin 1993 (80% for
chemotheraPy cosmeticreasons,2A/" for
Recurrence: mostlyin contralateralbreast/ other distant ; 2 millionAmerican
reconstruction)
sites womenhavebreastimplants(estimate)
DDx: circumscribedbreastcarcinoma,fibroadenoma, Methods:
phylloidestumor,metastaticdisease 1. Injectionaugmentation (no longerpracticed):
paraffin,silicone,fat from liposuction
Pseudolymphoma Cx: tissuenecrosisresultingin dense,hard,
= lymphoreticular response
asanovenruhelming
lesion tenderbreastmasses;lymphadenopathy;
to trauma infection;granuloma formation(= siliconoma)
2. lmplants
(a) spongelikemassesof lvalon,Etrheron,Teflon
(b) Siliconeelastomer(silastic)smooth/ textured
MAMMARYDUCTECTASIA si l i conegel / sal i n e:
shel lcontai ni ng
= PLASMA CELLMASTITIS = VARICOCELE TUMOROF >100varieties
BREAST = MASTITIS OBLITERANS = COMEDOMASTITIS - singlelumenof polymerized methyl
= PERIDUCTAL MASTITIS = SECRETORY DISEASE OF polysiloxane with smoothi texturedouter
BREAST
siliconeshelli polyurethane coating
= ta:taasepticinflammationof subareolararea - doubl el umenw i th i nnercoreof silicone
Pathogenesis (specuIative):
+ outerchamberof saline
(1) Stasisof intraductalsecretionleadsto duct dilatation - triPlelumen
+ leakageof inspissatedmaterialinto parenchyma
(c) expandableimplant+ intraluminal valves
givingriseto an asepticchemicalmastitis(periductal
= salineinjectioninto port with gradualtissue
mastitis);theextravasatedmaterialis rich in fatty
expansionfor breastreconstruction
acids = nontraumaticfat necrosis
Location: retroglandular I subpectoral
(2) Periductalinflammationcausesdamageto elastic
3. Autogenoustissuetransplantation
laminaof duct wall resultingin duct dilatation
(for breastreconstruction) with musculocutaneous
Histo: ductalectasia,heavilycalcifiedductalsecretions;
flaps: transverserectusabdominismuscle
of plasmacells+ giantcells
infiltration
(TRAM),latissimusdorsi,tensorfascialata,
+ eosinophils
gl uteusmaxi mus
Mean age: 54 years
. often asymptomatic Mammographic technique for implants:
. breastpain,nippledischarge,nippleretraction, 1. Two standardviews(CC and MLO views)for most
posteriorbreasttissue
mamillaryfistula,subareolarbreastmass
0 22-83% of fibroglandularbreasttissueobscured
Location: subareolar, oftenbilateral+ symmetric;may
by implantdependingon size of breast
be unilateral+ focal
+ locationof implant+ degreeof capsular
r/ dense triangularmass with apex towardnipple
contractionon standardviews!
r/ distendedductsconnectingto nipple

0 The false-negativerate of mammography
increasesfrom 10-20%lo 41o/oin patientswith
implants!
2. Two Eklund(= implantdisplacement) views
(CC and 90" LAT views)for compressionviews of
anteriorbreasttissue= ,,push-back" view = breast
tissuepulledanteriorlyin frontof implantwhile
implantis pushedposteriorfy+ superiorlythus
excludingmostof the implant
Cx of silicone-gel-filled implant:
1. Capsularfibrosis,calcification,contracture(1S-
50%): morefrequentwith retroglandular implants
. distortionof breastcontourwith hard capsule
r/ crenulatedcontour(US helpful)
r/ capsularcalcificationsat peripheryof prosthesis
{ fibrouscapsuledelineatedby US (unleaked
siliconeis echolucent)
2. lmplantmigration
Cause: overdistensionof implant pocket at
surgery
3. Ruptureof prosthesis
Prevalence: >50o/"after 12 years
. changein contour/ locationof implant
. flatteningof implant
., breastpain
r/ extracapsularsiliconein 11-23/o (97% specific,
5% sensitive)
4. "Gelbleed"- leakageof siliconethrough
semiporousbut intactbarriershell made of
siliconeelastomer
5. Localizedpain / paresthesia
6. ? development of autoimmunedisorders
(eg, scleroderma,lupuserythematosus)
7. lnfection/ hematomaformation
lntracapsularRupture (morecommon)
= brokenimplantcasingwith siliconeleakage
containedby intactfibrouscapsule
Mammo(11-23%sensitive,Bg-98%specific):
r/ butging/ peakingof implantcontour
US (59-70% sensitive,ST-g2o/"specific,49%
accurate):
r/ "stepladder"sign = seriesof parallelhorizontal
echogenicstraight/ curvilinearlines inside
implant(= collapsedimplantshellfloatingwithin
silicone gel)
,
{ heterogeneousaggregatesof low- to medium-
levelechogenicity(65% sensitive,57% specific)
N.B.: visualization of internallumenwithin
anechoicspacein double-lumen implants
can be confusedon US with intracapsular
rupture
MR (81-94% sensitive,gg-g7% specific,g4%
accurate):
r/ "linguine"sign = multiplehypointense wavy lines
within implant(= piecesof free-floatingcollapsed
envelopesurroundedby siliconegel)
BreastDisorders 563
r/ "invertedteardrop"/ "noose"/ "keyhole"I "larial
(= lasso)"sign = loop-shapedhypointense
structurecontiguouswith implantenvelope
(= smallfocalinvaginationof shellwith silicone
on eitherside)
{ = infoldedpolyurethane coat of a singlelumen
prosthesis
r/ hypointensesubcapsularlinesparallelingthe
fibrouscapsule(= minimallydisplacedruptured
shellas earlysign)(DDx:phase-encoding artifact
causedby motion)
Extracapsular Rupture
= extrusion+ migrationof siliconedropletsthrough
tear in both implant+ overlyingfibrouscapsule
. palpablebreastmasses
US:
{ "snowstofm"l"echogenic noise"pattern
= msrkedlyhyperechoicnodulewith wefl-defined
anterior+ indistinctechogenicnoiseposteriorly
(= free siliconedropletsmixedwith breast
tissup)
./ frightyechogenicarea with acousticshadowing
! hypoechoicmassesalmostindistinguishable from
cysts+ usuallysurroundedby echogenicnoise
(= largeto medium-sized collections
of free
silicone)with low-levelinternalechoes
MR:
./ discretehypointensefoci on fat-suppressedTlWl
+ hyperintensesignalon water-suppressed T2Wl
in continuitywith / separatefrom implant
Mammography:
r/ lobular/ sphericaldense area of opacities
adjacentto / separatefrom siliconeimplant
{ rim calcifications
ExtRlcepsulAR Spneno or Stlcone
Source: gel bleed,implantrupture(11r-29%)more
commonwiththinnershell+ olderimplants
. siliconelymphadenopathy
. paresthesiaof arm (from nerye impingement
secondaryto fibrosissurrounding silicone
migratedto axilla/ brachialplexus)
. siliconenippledischarge(rare)
; migrationto arm (+ constrictiveneuropathyof
radialnerve),subcutaneoustissueof lower
abdominalwall,inguinalcanal
r/ migrationto ipsilateralchestwall + axillarynodes
r/ siliconedropletsin breast
r/ granulomaformation(siliconoma)+ fibrosis
ReductionMammoplasty
r/ swirledarchitecturaldistortion(in inferiorbreastbest
seen on mediolateralview)
r/ postsurgicaldistortion
r/ residualisolatedislandsof breasttissue
r/ fat necrosis
r/ dystrophiccalcifications
r/ asymmetrictissueorientedin nonanatomicdistribution
564 Radiology Review Manual

. palpablemass/ thickeningof breastonly (14%)

MASTITIS
. tenderswollenred breast(DDx:inflammatory . + bloodynippledischarge+ itching
carcinoma) Histo: Pagetcell = largepleomorphiccellswith pale
. enlargedpainfulaxillarylymphnodes cytoplasmarisingin main secretoryducts and
. + febrile,elevatedESR, leukocytosis and
migratingintoepidermis;histologically
^/ diffuseincreaseddensity biologically similarto comedocarcinoma
r/ diffuseskinthickening Associated with:
r/ swellingof breast extensiveinvasive/ in situ ductalcarcinomalimitedto
{ enlargedaxillarylymph nodes one duct in subareolararea / remote+ multicentric
{ rapidresolutionunderantibiotictherapy { negativemammogramin 50%
{ nippleI areolar/ skin thickening
PuerperalMastitis i/ nippleretraction
= LACTATIONAL MASTITIS r/ dilatedduct
= usUdllvinterstitialinfectionduringlactationalperiod r/ linearlydistributedsubareolar/ diffusemalignant
(a) throughinfectednipplecracks microcalcifications
(b) hematogenous { discreteretroareolarsoft-tissuemass/ masses
(c) ascendingvia ducts = galactophoritis Dx: cytologicsmear of a weepingnipplesecretion/
Organism; Staphylococcus,Streptococcus excisionalbiopsyof a nipplelesion
Rx: incision+ drainage Prognosis; survivalrate with palpablemass similarto
ductcarcinoma;85-90% 10-year
infiltrating
Mastitis
Nonpuerperal survivalrate withoutpalpablemass; positive
axillarynodesin 0-13%
1. lnfectedcyst
2. Purulentmastitiswith abscessformation
3. Plasmacell mastitis PAPILLOMA OF BREAST
4. Nonspecificmastitis = usuallybenignproliferation of ductalepithelialtissue
Age: 30-77 years (juvenilepapillomatosis = 20-26
years);may occurin men
GranulomatousMastitis
1. F or eign- b o dgyra n u l o m a Histo: hyperplasticproliferationof ductalepithelium;
leprosy,syphilis, lesionmay be pedunculated / broad-based;
2. Specificdisease(TB,sarcoidosis,
actinomycosis, typhus) connectivetissuestalkcoveredby epithelialcells
in the form of apocrinemetaplasia/
proliferating
3. Parasiticdisease(hydatiddisease,cysticercosis,
filariasis,schistosomiasis) solid hyperplasiamay cause duct obstruction
+ distensionto form an intracysticpapilloma
DDx: invasivepapillarycarcinoma
TO BREAST
METASTASES
lncidence: 1o/o CentralSolitaryPaPilloma
Mean age: 43 years Location: subareolarwithinmajorduct
Primaries: leukemia/ lymphoma> malignantmelanoma NOT premalignant
> ovariancarcinoma> lung cancer> sarcoma . spontaneoususuallybloody/ serous(9480/") I clear
0 In up to 40/o no known historyof primary nippledischarge(52-88-100%):
cancer! 0 Mostcommoncauseof serous/ sanguineous
.
in children:rhabdomyosarcoma, leukemia, nippledischarge!
non-Hodgkinlymphoma . "triggerpoint"= nippledischargeproducedupon
i/ solitarymass (85%),esp. in upperouter quadrant compression of areawith papilloma
r/ multiplemasses . intermittent massdisappearing with discharge
{ skin adherence(25%)+ skin thickening rl negativemammogram/ intraductalnodulesin
{ axillarynode involvement (40%) subareolararea
./ asymmetrically dilatedsingleduct
OF THENIPPLE
PAGETDISEASE r/ subareolaramorphouscoarsecalcifications
[SirJamesPaget(1814-1899), surgeonandpathologist at St. r/ ditatedduct with obstructing/ distortingintraluminal
Bartholomew's Hospital,London,Engtand; in 18741
firstdescribed fillingdefecton ductography(= galactography)
= UflCOTT]rT'tOn
manifestationof breastcancercharacterized Cx: O-5-14/" frequencyof carcinomadevelopment
of the nippleepidermisby adenocarcinoma
by infiltration
Prevalence: 2-3/" of all breast cancers
. nipplechanges(32%): erythema,scaling,erosion, PeripheralMultiPlePaPillomas
ulceration,retractionof nippleand areola: Location: withinterminalductallobularunit;bilateral
0 Mediandelayof correctdiagnosisby 6-11 monthsas i n uPto 14%
featuressuggesta benigndiagnosisof eczema! ln 10-38% associated with:
. nipplechanges+ palpablemass / thickeningof breast atypicalductal hyperplasia,lobularcarcinomain situ,
(45%) papillary+ cribriformintraductalcancers,radialscar
 BreastDisorders 565

. nippledischarge(20%) , rarelypalpable
r/ roundI oval/ slightlylobulatedwell-circumscribed r/ meandiameterof 0.33 cm (range,0.1-0.6cm)
nodules r/ typicallyno centralmass (BUT:irregularnoncalcified
./ segmentaldistributionwith dilatedducts extending mass oftenwith architecturaldistortion)
from beneaththe nipple(20%) r/ variableappearancein differentprojections(= radial
r/ may be associateO witn coarsemicrocalcifications scarsare typicallyplanarin configuration)
Cx: 5% frequencyof carcinomadevelopment; r/ oval / circulartranslucentareas at center
increasedriskdependenton degreeof cellular { very thin longspicules,clumpedtogethercentrally
atypia ri radiolucentlinearstructures(= fat) parallelingspicules
Prognosis; in 24o/orecunenceafter surgicaltreatment ("blackstar" appearance)
ri no skin thickening/ retraction
PSEUDOANGIOMATOUS STROMAL HYPERPLASIA Rx: surgicalexcisionrequiredfor definitediagnosis
= benignproliferative lesionsof mammarystromain a DDx: carcinoma,postsurgicalscar, fat necrosis,
spectrumfrom focal incidentalfindingsto clinically fibromatosis, granularcell myoblastoma
+ mammographically evidentbreastmasses
Histo: (a) incidentalfocal microscopicfindingin 23/" ot
all breastspecimens SARCOMA OF BREAST
(b) tumoralform (rare) lncidence: 1"/"of malignantmammarylesions
Age: 45-55 years
TumoralFormof Pseudoangiomatous Stromal Histo: fibrosarcoma,rhabdomyosarcoma, osteogenic
Hyperplasia sarcoma,mixedmalignanttumorof the breast,
Age: 4-Sth decade (range 14-67 years) malignantfibrosarcoma and carcinoma,
Histo: proliferatingmyofibroblastscreatingslit-like liposarcoma
spacespositivefor CD34 + muscleactin; . rapidgrowth
similarin appearanceto low-grade r/ smooth/ lobulatedlargedense mass
angiosarcoma r/ well-defined outline
., singlecircumscribed palpablemass ./ palpatedsize similarto mammographic size
r/ well-circumscribed 5-6 (range,1-12)-cmmass
r/ growthover time + recurrenceafter excisionalbiopsy Angiosarcoma
US : = highlymalignantvascularbreasttumor
r/ hypoechoicsolidmasswith slightlyheterogeneous lncidence: 200 cases in world literature;0.04./"of all
echotexture malignantbreasttumors;8./" of all breast
r/ + smallcysticcomponent sarcomas
DDx: fibroadenoma, phylloidestumor Age: 3rd-4th decadeof life
Histo: hyperchromatic endothelialcells;networkof
RADIAL SCAR communicating vascularspaces
= SCLEROSING DUCTHYPERPLASIA = INDURATIVE stage l: cellswith largenucleoli
MASTOPATHY = FOCALFIBROUS DISEASE = BENIGN stagell: endothelial finingdisplayingtufting
SCLEROSING DUCTAL PROLIFERATION = INFILTRATING + intraluminal papillaryprojections
EPITHELIOSIS = NONENCAPSULATED SCLEROSING stage lll: mitoses,necrosis,marked
LESION hemorrhage
= benignproliferative breastlesion(malignantpotentialis Metastasis; hematogenousspreadto lung,skin,
controversial)unrelatedto prior surgery llrauma subcutaneous tissue,bone,liver,brain,
lncidence: 0.1-2.0/1,000screeningmammograms; in ovary;NOT lymphatic
2-16% of mastectomyspecimens . rapidlyenlargingpainlessimmobilebreastmass
Cause: ? localizedinflammatoryreaction,? chronic ! skin thickening+ nippleretraction
ischemiawith slow infarction ^/ largesolitarymasswith ill-defined nonspiculated
Path: "Scar"= scleroticcentercomposedOfacellular border
connectivetissue(= fibrosis)and elastindeposits US:
(= elastosis);entrappedductuleswith intact ri well-definedmultilobulated hypoechoicmasswith
myoepithelial layerin scleroticcore;coronaof hyperechoic areas(fromhemorrhage)
distortedducts + lobulescomposedof benign Prognosis: 1.9-2.1 years mean survival;14o/"overall
proliferations(sclerosingadenosis,ductal 3-yearsurvivalrate
hyperplasia,cyst formation,papiIlomatosis) Rx: simple mastectomywithout axillarylymph node
ln up to 50% associated with: dissection
tubularcarcinoma,comedocarcinoma,invasivelobular DDx: phylloidestumor,lactatingbreast,juvenile
carcinoma+ contralateralbreast cancer hypertrophy
0 Avoidfrozensection,core needlebiopsy,fine-needle 0 Frequentlymisdiagnosed as lymphangioma /
aspiration! hemangi oma!
566 Radiology Review Manual
 DIFFERENTIAL
DIAGNOSISOF CARDIOVASCULAR
DISORDERS

CONGENITAL
HEARTDISEASE

Approachto CongenitalHeartDisease
Acyanotic Cyanotic
i/ enlargedmainpulmonaryartery r/ concavemain pulmonaryartery

Increased PBF L-Rshunts Admixturelesions= bidirectior",,nrnO = r-*r,o*

+ increasedCff ratio VSD Transposition
ASD Truncus arteriosus
PDA TAPVR
ECD Tricuspidatresia(withoutRVOTobstruction)
PAPVR "Tingles"
(singleventricle
/ atrium)
Normal PBF LV outflow obstruction
+ normalC/T ratio AS
+ pulmonaryvenous Coarctation
hypertension Interruptedaorticarch
Hypoplasticleft heart
LV inflow obstruction
ObstructedTAPVR
Cor triatriatum
Pulmonaryvein atresia
CongenitalMV stenosis
Muscle disease
Cardiomyopathy
Myocarditis
AnomalousLCA

DecreasedPBF R\o-L shunts

+ normal C/T ratio + nonrestrictiveintracardiacshunt
Tetralogyof Fallot
Tricuspidatresia(with PS + nonrestrictive
ASD)
Pulmonaryatresia+ nonrestrictive VSD
+ increasedCff ratio + restrictiveintracardiacshunt
Pulmonaryatresia+ ASD withoutVSD
PS with ASD i patentforamenovale
Tricuspidatresia+ PS + restrictiveASD
Trilogyof Fallot
Ebsteinanomaly
Congenitaltricuspidinsufficiency

Incidence of CHD in Liveborn Infants Coarctationof aorta 5]%

Overallincidence: 8-9:1 000 livebirths Aorticstenosis 5.2%
0 MostcommonCHD: mitralvalveprolapse(S-20%), Tetralogyof Fallot 5.1%
bicuspidaorticvalve (2%) [usuallynot recognized Transposition 4J%
beforelate infancy/ childhoodl Endocardial cushiondefect 9.2%
0 ASD + VSD + PDA accountfor 4|oh of all CHD Hypoplasticrightventricle 2.2%
0 12 lesionsaccountfor 89% of all CHD Hypoplasticleftheart 1.3%
Ventricularseptaldefect 30.3% TAPVR 1.1"/"
Patentductusarteriosus 8.6% Truncusarteriosus 1i%
Pulmonary stenosis 7.4/" Singleventricle 03%
Septumsecundumdefect 6.7% Doubleoutletrightventricle 0.2%
568 Radiology Review Manual

High-riskpregnancy: ContinuousHeartMurmur
( 1) P r ev io u s i b l i n gw i thC H D : 2- 5% 1. PDA
(2) Previous2 siblingswith CHD: 10-15% 2. AP window
(3) One parentwith CHD: 2-10% 3. Rupturedsinusof Valsalvaaneurysm
Mostcommoncausesfor CHF + PVH in neonate: 4. Hemitruncus
1. Leftventricular failuredue to outflowobstruction 5. Coronaryarteriovenousfistula
2. Obstruction of pulmonaryvenousreturn

CHDPresentingin 1st Yearof Life Syndromeswith CHD

1. VSD
2. of greatvessels
d-transposition 5 p - (Cri-du-chat)Syndrome
3. Tetralogyof Fallot lncidence of CHD: 20%
4. lsolatedcoarctation
5. Patentductusarteriosus DiGeorge Syndrome
6. Hypoplasticleft heartsyndrome = congehitalabsenceof thymus+ parathyroid
glands
1. Conotruncalmalformation
CHD with Relatively Long Life 2. lnterruptedaorticarch
Congenitallesionscompatiblewith a relativelong life
are: Down Syndromo = MONGOLTSM = TRrsoMy21
1. Mildtetralogy:mildpulmonicstenosis+ small 1. Endocardial cushiondefect(25/")
VSD 2. MembranousVSD
2. Valvularpulmonicstenosis:with relativelynormal 3. OstiumprimumASD
pulmonarycirculation 4. A V communi s
3 . Transpositionof great vessels: some degreeof 5. Cleft mitralvalve
pulmonicstenosis+ largeVSD 6. P D A
4 . Truncusarteriosus:delicatebalancebetween 7. l l rib pairs (25%)
systemic+ pulmonarycirculation L Hypersegmented manubrium(90%)
5 . Truncusarteriosustype lV: largesystemic
collaterals Ellis-van CreveldSyndrome
6 . Tricuspidatresia+ transposition + pulmonic lncidence of CHD: 50%
stenosis . polydactyly
7. Eisenmenger complex { singleatrium
8 . Ebsteinanomaly
9 . Correctedtranspositionwithoutintracardiacshunt Holt-OramSyndrome
= UPPERLIMB-CARDIAC SYNDROME
Juxtaposition of Atrial Appendages lncidence of CHD: 50%
1. Tricuspid
atresiawithtransposition 1. ASD
2. Completetransposition 2. V S D
3. Corrected of greatarteries
transposition 3. Valvularpulmonarystenosis
4. DORV 4. Radialdysplasia

PresentingAge in CHD

Age Severe PVH PVH + Shunt Vascularity

0-2 days Hypoplasticleft heart Hypoplasticleft heart

Aorticatresia TAPVR abovediaphragm
TAPVRbelowdiaphragm Completetransposition
Myocardiopathy in IDM

3-7 days PDA in preterminfant

7-14 days CoA+VSD/PDA Coarctationof aorta (CoA)

Aorticvalve stenosis PeripheralAVM
PeripheralAVM
Endocardialf ibroelastosis
Anomalousleft coronaryartery
 DifferentialDiagnosisof Cardiovascular
Disorders 569

Hurler Syndrome SHUNTEVALUATION

Cardiomyopathy Evaluationof L-to-RShunts
A . A GE
lvemark Syndrome - lnfants:
lncidenceof CHD: 100% (1) l sol atedvsD
. asplenia
(2) VSD with CoA i PDA / AV canal
{ complexcardiacanomalies (3) P D A
(4) Ostiumprimum
Klippel-Feil Syndrome - Children/ adults:
lncidence of CHD: 5% ( 1 )A S D
1. Atrialseptaldefect (2) PartialAV canalwith competentmitralvalve
2. Coarctation (3) VSD / PDA with highpulmonaryresistance
(4) PDA withoutmurmur
Martan Syndrome = ARACHNoDAcTvLv B. SEX
1. Aorticsinusdilatation 99% chancefor ASD / PDA in femalepatient
2. Aorticaneurysm C. CHESTWALL ANALYSIS
3. Aorticinsufficiency r/ t t pair of ribs+ hypersegmentedmanubrium:
4. Pulmonaryaneurysm Down syndrome
r/ pectusexcavatum+ straightback syndrome
Noonan Syndrome + funnelchest: prolapsingmitralvalve
1. P ulm ona ry
s te n o s i s { rib notching
2. A S D D . C A R D IA CS ILH OU E TTE
3. Hypertrophiccardiomyopathy r/ absentpulmonarytrunk:
correctedtranspositionwith VSD; pink tetralogy
Osteogenesis Imperfecta r/ left-sidedascendingaorta:
1. Aorticvalveinsufficiency correctedtranspositionwith VSD
2. Mitralvalve insufficiency ./ tortuousdescendingaorta:
3. Pulmonicvalveinsufficiency aorticvalve incompetence+ ASD
r/ hugeheart:
Postrubella Syndrome persistentcompleteAV canal(PCAVC);VSD
. low birthweight
+ PDA; VSD + mitralvalve incompetence
. deafness
{ enlargedleftatrium:
. cataracts
intactatrialseptum;mitralregurgitation
. mentalretardation
(endocardialcushiondefect,prolapsingmitral
1. Peripheralpulmonicstenosis valve+ ASD)
2. Valvularpulmonicstenosis
3. Supravalvularaorticstenosis
4. PDA Differential Diagnosis of L-R Shunts

Trisomy 13-15 RA RV PA LA LV Prox. Ao

VSD,tetralogyof Fallot,DORV A S D t t t < + < - > < _ >
V S D < - + t t t t e +
Trisomy 16-18 PDA <+ +> t 1 t oftent
VSD,PDA,DORV
TurnerSyndrome(XO)= oVARIAN
DyscENESts
lncidenceof CHD: 35% Shunt with Normal LeftAtrium
1. Coarctationof the aorta (in 15%) A . P R E C A R D IA C
SHUNT
2. Bicuspidaorticvalve 1. Anomalouspulmonaryvenousconnection
3. Dissectinganeurysmof aorta B . IN TR A C A R D IASCH U N T
1. A S D (8% )
Williams Syndrome = tDtopATHtcHypERcALcEMtA 2. VSD (25%)
. peculiarelfinlikefacies C. POSTCARDIAC SHUNT
. mental+ physicalretardation 1. P D A (12% )
. hypercalcemia(not in all patients)
1. Supravalvular aorticstenosis(33%)
2. A S D,V S D Aortic Size in Shunts
3. Valvular+ peripheralpulmonaryarterystenosis A. EXTRACARDIAC SHUNTS
4. Aortichypoplasia,stenosesof more peripheral { aorta enlarged+ hyperpulsatile
arteries 1. PDA
570 Radiology Review Manual

B . P RE .A N D IN T R AC AR D IAS
CH U N T S
^/ aorta small but not hypoplastic
1. Anomalouspulmonaryvenousreturn
2. A S D
3. V S D
4. CommonAV canal
HEARTDISEASE
CYANOTIC
Chemicalcyanosis = PdO2<94/"
Clinicalcyanosis = P€lOz<85%
0 Decreasein hemoglobindelaysdetectability!
Most commoncause of cyanosis
- in newborn: transpositionof greatvessels
- in child: tetralogyof Fallot!
N.B.: tricuspidatresia= the greatmimicker
^/ R aorticarch: persistenttruncusarteriosus
^/ ductusinfundibulum:aorticatresia
ri pulmonarytrunkseen: supracardiac TAPVR;DORV;
tricuspidatresia;commonatrium
{ ascendingaortawith leftwardconvexity:single
ventricle
^/ ditatedazygosvein: commonatrium+ polysplenia
+ interruptedIVC;TAPVR to azygosvein
./ left-sidedSVC: verticalvein of TAPVR
r/ "waterfall"right hilum: singleventricle+ transposition
^/ largeleftatrium(rulesout TAPVR)
r/ prominentL heartborder: singleventriclewith
invertedrudimentaryR ventricle;levopositionof right
atrialappendage(tricuspidatresia+ transposition)
./ age of onset <2 days: aorticatresia

DecreasedPulmonaryBloodFlowwith Cyanosis
IncreasedPulmonaryBloodFlowwith Cyanosis = two componentsof
= ADMIXTURE LESIONS = bidirectional
shuntwith (a) impedanceof bloodflow throughrightheartdue to
2 components: obstruction/ atresiaat pulmonaryvalveI
(a) mixingof saturatedblood(L-Rshunt)and i nfundi bul um
unsaturatedblood(R-Lshunt) (b) Rto-L shunt
(b) NO obstructionto pulmonarybloodflow . pulmonarycirculationmaintainedthroughsystemic
Evaluation process: arteries/ PDA
r/ cardiomegaly r/ normal/ decreasedpulmonarybloodflow
r/ increasedpulmonarybloodflow ^/ concavemain pulmonaryartery
r/ concavemain pulmonaryartery: { cardiomegaly
r/ pn segmentabsent= transposition r/ restrictiveintracardiacR-to-Lshunt
r/ Pn segmentpresent: mnemonic: "P2 TETT"
(t t (=extracardiac
shunt) Pulmonicstenosiswith ASD
ltipnormal Pulmonicatresia
(b) L atriumenlarged(= intracardiacshunt) Tetralogyof Fallot
= truncusarteriosus Ebsteinanomaly
N.B.: Overcirculation+ cyanosis= Gomplete Tricuspidatresiawith pulmonicstenosis
transpositionuntil provenotherwise! Transpositionof greatvesselswith pulmonicstenosis
LE V E L
A . S H U N TA T V E N TR IC U LA R
A DM I X T UREL E SION S= T -L ES IO N S 1. Tetralogyof Fallot
mnemonic.'"5 T's + CAD" 2. Tetralogyphysiotogy(associatedwith pulmonary
Transpositionof greatvessels= comPleteTGV obstruction):
- complete/ correctedtransposition
t VSD
0 Most commoncausefor cyanosisin neonate - singleventricle
- DORV
Tricuspidatresiawith or withouttransposition
+ VSD - tricuspidatresia(PS in 75%)
0 2nd most commoncausefor cyanosisin - aspleniasyndrome
neonate ri prominentaortawith L / R aorticarch; inapparent
Truncusarteriosus pul monary trunk
Totalanomalouspulmonaryvenousreturn .i ruOnUnL R atrium(withouttricuspidregurgitation)
(TAPVR)abovediaphragm: r/ ruOnUnl-sized heart (secondaryto escape
(a) supracardiac mechanismintoaorta)
(b) cardiac(coronarysinus/ rightatrium) Clues:
"Tingle"= singleventricle 1. Skeletalanomaly(eg,scoliosis):tetralogy(90%)
Commonatrium 2. HepaticsymmetrY:asPlenia
Aortic atresia 3. Rightaorticarch: tetralogy,complete
Double-outletrightventricle(DORVtype | ) / transposition,tricusPidatresia
Taussig-Bing anomaly(DORVtype ll) 4. Aberrantrightsubclavianartery: tetralogy
Clues: 5. Leftwardconvexityof ascendingaorta: single
{ skeletalanomalies:Ellis-vanCreveldsyndrome ventriclewith invertedright rudimentary
(truncus/ commonatrium) ventricle,correctedtransposition,asplenia,
r/ polysplenia:commonatrium JAA (tricusPidvalve atresia)
 DifferentialDiagnosisof Cardiovascular
Disorders 571

B. SHUNTAT ATRIALLEVEL 2. Left ventricularinflowobstruction

mnemonic.'"PET" (a) at levelof peripheralpulmonaryveins:
1. Pulmonarystenosis
/ atresia
withintact pulmonaryvein stenosis/ atresia
ventricular
septum (b) at levelof left atrium: cor triatriatum
2. Ebsteinmalformation+ Uhl anomaly (c) at levelof mitralvalve:
3. Tricuspidatresia(ASD in 100%) supravalvularmitralstenosis,congenitalmitral
{ moderateto severecardiomegaly stenosis/ atresia,"parachute"mitralvalve
{ R atrialdilatation 1. Left ventricularoutflowobstruction
./ R ventricularenlargement(secondaryto massive (a) at level of aortic valve:
tricuspidincompetence) anatomicsubaorticstenosis,functional
r/ inapparentaorta subaorticstenosis(IHSS),valvularaortic
^/ lett aortic arch stenosis,hypoplasticleft heart,supravalvular
aorticstenosis
(b) at levelof aorta:
PulmonaryVenousHypertensionwith Cyanosis interruptionof aorticarch, coarctationof aorta
(a) during1stweek of life
B. CARDIOMYOPATHY
1. Hypoplasticleft heartsyndrome 1. Endocardialfibroelastosis
r/ markedcardiomegaly 2. Hypertrophiccardiomyopathy
2. TAPVRbelowdiaphragm 3. Glycogenstoragedisease
d normalcardiacsize C. HYPERDYNAMIC STATE
(b) during2nd week of life
1. NoncardiacAVM (cerebralAVM, vein of Galen
3. Aorticcoarctation aneurysm,largepulmonaryAVM,
4. Aorticatresia hemangioendothelioma of liver)
(c) during4-6th week of life 2. Thyrotoxicosis
5. Criticalaorticstenosis 3. Anemia
6. Endocardialfibroelastosis 4. Pregnancy
7. Anomalousoriginof LCA D. MYOCARDIALISCHEMIA
8. Atresiaof commonpulmonaryvein 1. Anomalousleft coronaryartery
2. Coronaryarterydisease(CAD)

ACYANOTIC HEARTDISEASE PULMONARY VASCULARITY

IncreasedPulmonaryBloodFlowwithout
Cyanosis
= indicatesL-R shuntwith increasedpulmonaryblood
flow (shuntvolume>40/")
A. WITH LEFTATRIALENLARGEMENT
indicatesshunt distalto mitralvalve = increased
volumewithoutescapedefect
1. VSD (25%): smallaortain intracardiac shunt
2. PDA (12oh): aorta+ pulmonaryarteryof equal
size in extracardiacshunt
3. Rupturedsinusof Valsalvaaneurysm(rare)
4. Coronaryarteriovenousfistula(very rare)
5. Aortopulmonarywindow(extremelyrare)
B. WITH NORMALLEFTATRIUM
indicatesshuntproximalto mitralvalve= volume
increasedwith escapemechanismthroughdefect
1. A S D ( 8% )
2. Partialanomalouspulmonaryvenousreturn
(PAPVR)+ sinusvenosusASD
3. Endocardial cushiondefect(ECD)(4%)
NormalPulmonaryBloodFlowwithoutCyanosis
A. OBSTRUCTIVE
LESION
1. Rightventricular outflowobstruction
(a) at levelof pulmonaryvalve: subvalvular/
valvular/ supravalvularpulmonicstenosis
(b) at levelof peripheralpulmonaryarteries:
peripheralpulmonarystenosis
NormalPulmonaryVasculature
A. VASCULARDISTRIBUTION
r/ pulmonaryvesselswithinupperperihilum
approximate1/3 of total vascularity
{ pulmonaryvesselswithinlowerperihilum
approximate213of total vascularity
B. VASCULARTAPERING
{ pulmonaryvesselstaperneartransitionof middle
1/3to outer1/3 of lung
C. VASCULARCALIBER
{ straight/ slightlyconcavemainpulmonaryartery
contour(mildconvexityis normalin youngfemales)
r/ pulmonarytrunk measures<4.5 cm (leftward
distancefrom verticalline at carinato most lateral
aspectof main pulmonaryarterycontour)
{ rightinterlobar / intermediate pulmonaryartery
measures10-15 mm i n mal esand 9-14 m m in
femaleson PA radiographs
r/ pulmonaryvesselsize <1-2 mm in extremelung
periphery
r/ arterieswithin 1st anteriorintercostalspace
measure<3 mm
Normal Pulmonary Vascularity & Normal-sized
Heart
mnemonic.' "MAN"
Myocardialischemia
Afterload(= pressureoverloadproblems)
Normal
572 RadiologyReviewManual

IncreasedPulmonaryVasculature 2. Persistenttruncusarteriosus(esp.type lV)

A. OVERCIRCULATION ./ diminished/ increasedbloodflow to eitherlung
= shuntvascularity= arterial+ venousovercirculation 3. Pulmonaryvalvularstenosis
(a) congenitalheartdisease(mostcommon) ./ increasedflow to left lung secondaryto jet
( 1) L- Rs h u n ts phenomenon
(2) Admixturecyanoticlesions
(b) high-flowsyndromes
(1) Thyrotoxicosis Dilatationof PulmonaryTrunk
(2) Anemia 1. ldiopathicdilatationof pulmonaryartery
(3) Pregnancy 2. Pulmonicvalvestenosis
(4) Peripheralarteriovenous fistula { poststenoticdilatationof trunk + left pulmonarya.
{ diameterof right descendingpulmonaryartery 3. Pulmonaryregurgitation
largerthan tracheajust above aorticknob (a) severepulmonicvalveinsufficiency
./ increasedsize of veins + arterieswith size larger (b) absenceof pulmonicvalve (may be associated
than accompanying bronchus(= "kissingcousin" with tetralogy)
sign),bestseen just abovehilaon AP view 4. CongenitalL-to-Rshunts
r/ enlargedhilarvessels(lateralview) 5. Pulmonaryarterialhypertension
r/ visualization of vesselsbelow1Othposteriorrib 6. Aneurysm:mycotic/ traumatic
B . P ULM O N A R Y VE N OU SH Y PE R T E N S IO N
rl redistribution of flow (notseen in youngerchildren)
r/ indistinctness of vesselswith Kerleylines Ratios
PulmonaryArtery-Bronchus
(= interstitialedema) = rdtioof diametersof end-onsegmentalpulmonary
r/ tine reticulatedpattern artery+ accompanying end-onbronchus
{ alveolaredema A . E R E C TC H E S TFILM
C. PRECAPILLARY HYPERTENSION 1. Normal(effectof gravity):
{ enlargedmain+ rightand leftpulmonaryarteries upperl ungzone 0 . 85+ 0. 15
{ abrupttaperingof pulmonaryarteries l ow erl ungzone 1. 34+ 0. 25
D. P RO M I N E N T S YS T EMIC/ AOR T O P U L MON A R Y 2. Equalized/ balancedredistribution pattern:
COLLATERALS upperl ungzone 1. 62t 0. 31
1. Tetralogyof Fallotwith pulmonaryatresia lowerlungzone 1.56t 0.28
(= pseudotruncus) Cause: systemicvolumeoverload
2. VSD + pulmonaryatresia(singleventricle, 3. Cephalizedredistribution pattern:
completetransposition,correctedtransposition) upperl ungzone 1. 50+ 0. 25
3. Pulmonary-systemic collaterals lowerlungzone 0.87+ 0.20
r/ coarsevascularpatternwith irregularbranching Cause: hydrostaticcardiogenicedema
arteries(fromaorta/ subclavianarteries) B . S U P I N EC H E S TF I L M
r/ smallcentralvesselsdespiteapparentincreasein 1. Normal(gravitational effectlost):
vascularity upperl ungzone 1. 01t 0. 13
l o w e rl u n gz o n e 1 . 0 5+ 0 . 1 3
DecreasedPulmonaryVascularity 2. DecompensatedCHF (invertedpattern/ plethora
= obstructionto pulmonaryflow pattern):
r/ vesselsreducedin size and number upperl ungzone 1. 49t 0. 31
r/ hyperlucentlungs lowerlungzone 0.96t 0.31
r/ smallpulmonaryarterysegment+ hilarvessels

PULMONARY ARTERY P U LMON A R Y H Y P E R TE N S ION

InvisibleMainPulmonaryArtery = sustainedpulmonaryarterialpressurein systole>25 mm
A. UNDERDEVELOPED= RVOT OBSTRUCTION Hg at rest/ >30 mm Hg duringexercise(in diastole
1. Tetralogyof Fallot >15 mm Hg, mean pressure>20 mm Hg) secondaryto
2. Hypoplastic rightheartsyndrome(tricuspid
/ area of the pulmonary
reductionin cross-sectional
pulmonaryatresia) vascularbed with concomitantincreasein pulmonary
ARTERY
B . M I S P LA C EDPU L M O N AR Y vascularresistance
1. Completetranspositionof greatvessels Cx: centralarterialthrombosis,premature
2. Persistent truncusarteriosus atherosclerosisof centralelastic+ muscular
pulmonaryarteries,aneurysmaldissectionof
Unequal Pulmonary Blood Flow pulmonaryarteries,hypertrophy+ dilatationof right
1. Tetralogyof Fallot side of heart
^/ diminishedflow on left side (hypoplastic/ stenotic Dx: clinicalassessmentof hemodynamicparameters,
pulmonaryarteryin 40%) medicalhistory,histologicfindings
 DifferentialDiagnosisof Cardiovascular
Disorders 573

PulmonaryArterialHypertension r/ main pulmonaryarterydiameter>2g mm (69%

= PAH= precapillary
pulmonary
hypertension sensitive,100% specific)
= changeslimitedto arterialside of pulmonary ! "pruning"of peripheratpulmonaryarteries
circulation = disproportionate increasein caliberof central
Pathogenesis: fibrousarteries+ decreasein caliberof smaller
A . HY P E R K IN E T IC C AU SE S musculararteries(fromsustainedincreasein flow
1. L- t o -Rs h u n t by a factor of >2)
2. High cardiacoutputstates: thyrotoxicosis, r/ mosaicperfusion
chronicanemia r/ normal-sizedheart/ right heartenlargement
B. OBLITERATIVE CAUSES d ttO increaseof pulsationsin middlethird of lung
(a) vasculap= precdpillary pulmonary r/ complications:
hypertension: ./ dilatationof bronchialarteries
1. Primaryplexogenicpulmonaryarteriopathy r/ subpleuralpulmonaryinfarcts(withelevated
= Primarypulmonaryhypertension (ppH) pulmonaryvenouspressure/ underlying
2. Arteritis(eg, Takayasu) malignancy)
3. Embolization r/ calcifiedplaquesof centralpulmonaryarteries
chronicthromboembolic disease (PATHOGNOMONTC)
tu m o r r/ dissection/ massiveihrombosisof central
V lymphangitic carcinomatosis pulmonaryarteries
garasites,eg, schistosomiasis
V hepatosplenomegaly Cor Pulmonale
mnemonic;"TICCS
BEV"
ffi iJ['i:'i,",.opac
ities Thoracicdeformity
r/ perihilarfibroticmasses fdiopathic
4. Persistentfetal circulation Chronicpulmonaryembolism
5. Pulmonarycapillaryhemangiomatosis COPD
(b) pleuropulmonic disease Shunt(ASD,VSD,etc)
1. Chronicinterstitial lungdisease Bronchiectasis
= cor pulmonale: Emphysema
COPD,emphysema,chronicbronchitis, Vasculitis
asthma,bronchiectasis, malignantinfiltrate,
granulomatous disease,cysticfibrosis,end- Pulmonary Venous Hypertension
stagefibroticlung,S/P lung resection, = INCREASED VENOUS PULMONARY PRESSURE
idiopathichemosiderosis, alveolar = VENOUS CONGESTION
proteinosis, alveolarmicrolithiasis = postcapillary
pulmonary = primary
hypertensisn
2. Pleuraldisease+ chestdeformity: findingslocatedwithinpulmonaryvenouscirculation
fibrothorax,thoracoplasty,kyphoscoliosis betweencapillarybed+ leftatrium
(c) vasoconstrictive Dx: uniform / widelyvariable of pulmonary
elevation
1. Chronicalveolarhypoxia wedgepressure
capillary (PCWP)>15mmHg
= hypoxicpulmonaryarterialhyperperfusion: Cause:
chronichighaltitude,sleepapnea,chronic A. LEFTVENTRICULAFI INFLOW TRACT
hypercapneadue to hypoventilation from OBSTRUCTION
neuromuscular disease/ obesity r/ normal-sized
heartwithrightventricutar
2. Portopulmonary hypertension(rare) hypertrophy
C. Chronicpulmonaryvenoushypertension r/ prominent
pulmonary trunk

Evaluationof PulmonaryVasculature
Normal PAH PVH Overload
Distribution
(UL:LL) 1:2 1:2 1 : 1o r 1 : 2 1:1
Vesseltapering middle:outer
third variablypruned outerthird outerthird
Vascularcaliber increased increased increased
Artery-bronchus
ratio
upperlungzone 0 . 8 5+ 0 . 1 5 1 . 5 0+ O . e S 1 . 6 2+ 0 . 3 1
lowerlungzone 1 .3 4+ 9 .2 5 0.87t0.20 1 . 5 6+ 0 . 2 8
Vascularmargins sharp sharp obscured obscured
574 Radiology Review Manual

@ proximalto mitralvalve: Histo:

./ normal-sizedleft atrium (a) primarychanges: venous medialhypertrophy
- pulmonaryveins markedthickeningof
+ intimalproliferation,
1. TAPVRbelowthe diaphragm venousinternalelasticlamina
2. Primarypulmonaryvenoocclusive (b) secondarychanges: capillarybed congestionwith
disease(PVOD) adjacentvascularproliferation,interlobularseptal
3. Stenosisof individualpulmonaryveins and pleuraledema+ fibrosis,lymphaticdilatation,
4. Atresiaof commonpulmonaryvein alveolarhemosiderosis, paraseptalvenous infarcts
- mediastinum adjacentto completevenousocclusion
1. Fibrosingmediastinitis (mayalso affect Cx: secondarypulmonary arterialhypertension
precapillaryvessels) { equalization of pulmonaryvascularity
2. Constrictivepericarditis (P C W P13-15 mm H g)
- left atrium { cephalizationof pulmonaryvascularity
1. Cor triatriatum (P C W P16-18 mm H g)
2. Left atrialmass: tumor,clot { interstitialpulmonaryedema (PCWP 19-24 mm Hg)
3. Supravalvularring of left atrium = fluidwithinperibronchovascular connectivetissue:
r/ peribronchial thickening/ cuffing
@ at mitralvalvelevel = mitralvalve stenosis ./ indistinctvessel margins
r/ enlargedleft atrium r/ t<erleyB lines= short horizontalreticulationswithin
1. Rheumaticmitralvalvestenosis lateralsubpleurallungbases
t regurgitation(99%) r/ KerleyA lines= S-4-cm-longlinesof interlobular
{ enlargedleft atrialappendage septalthickeningradiatingfrom hilato mid and
2. Congenitalmitralvalve stenosis upperlungzones
3. Parachutemitralvalve (= singlebulky { perihilarhaze= hilarinterstitialedema
papillarymuscle) ./ thickenedpleuralfissures/ pseudoeffusion = fluid
withinsubpleuralconnectivetissue
F A IL U R E
B . LE F TV E N T R IC U L A R { alveolarpulmonaryedema(PCWP>25 mm Hg)
(a) ABNORMALPRELOADwith secondarymitral = bilateralperihilarand basilarairspaceopacification
valve incompetence(= volumeoverload) { smallpleuraleffusions
1. Aorticvalve regurgitation
2. Eisenmenger syndrome(= Rto-L shuntin ABNORMAL HEART SIZE
vsD) Ratio
Cardiothoracic
3. High-output failure: = r?tioof cardiacdiameter(measuredas the sum of the
noncardiacAVM (cerebralAVM, vein of most rightward+ leftwardmarginsfrom the midline)to
Galenaneurysm,largepulmonaryAVM, innerthoracicmarginat its largestdiameter
hemangioendothelioma of liver,iatrogenic), <0.45 normal
thyrotoxicosis,anemia,pregnancy 0.45-0.55 mildcardiomegalY
(b) ABNORMALAFTERLOAD >0.55 moderate/ severecardiomegalY
(= pressureoverload)
= LV outflowtract obstruction Vascular Pedicle Width
1. Hypoplasticleft heartsyndrome = distanceon a horizontallinebetween(1) pointwhere
2. Aorticstenosis(supravalvular, valvular, rightmainstembronchus+ SVC crossand (2) point
anatomicsubaortic) where left subclavianarterycrosseshorizontalline
3. lnterruptedaorticarch 48+5mm normal
4. Coarctationof the aorta >53 mm edema,
in 60% of cardiogenic
(c) DISORDERSOF CONTRACTION AND in 85% of volumeoverload
RELAXATION
1. Endocardialfibroelastosis Abnormal Heart Chamber Dimensions
2. Glycogenstoragedisease(Pompedisease) A. LEFTVENTRICULAR VOLUMEOVERLOAD
3. Cardiacaneurysm 1. VSD
4. Cardiomyopathy 2. PDA
(a) congestive(alcohol) 3. Mitralincompetence
(b) hypertrophicobstructivecardiomyopathy 4. Aorticincompetence
(HOCM),particularly in IDM B. LEFTVENTRICULAR HYPERTROPHY
- asymmetricseptalhypertrophy(ASH) 1. Coarctation
- idiopathichypertrophicsubaortic 2. Aorticstenosis
stenosis(IHSS) C. RIGHTVENTRICULAR OVERLOAD
VOLUME
(d) MYOCARDIALISCHEMIA 1. ASD
1. Anomalousleft coronaryartery 2. PartialAPVR/ totalAPVR
2. Coronaryarterydisease(CAD) 3. TricuspidinsufficiencY
 DifferentialDiagnosisof CardiovascularDisorders S7S

4. Pulmonaryinsufficiency r/ posteriordisplacement
of LUL bronchus
5. Congenital/ acquiredabsenceof pericardium
[6. Ebsteinanomaly]- not truly RV Left VentricularEnlargement
D. R] G HTV E N T R IC U L AR
H Y PE R T R O P H Y 0 LV enlargesin posterior,inferior+ leftwarddirection
1. Pulmonaryvalvestenosis PA CXR:
2. Pulmonaryhypertension ^/ leftwarddisplacementof downturnedcardiac
3. Tetralogyof Fallot apex = left ventricularconfiguration
4. V S D r/ compressionof left hemidiaphragm+ gastric
E. FIXEDSUBVALVULAR AORTICSTENOSIS bubbl e
F. HY P O P LA ST ICL EF T/ R IG H TV EN T R IC LE , LAT CXR:
CO M M O NV EN T R IC L E ri increasedconvexityof posteroinferior cardiac
G. CONGESTIVECARDIOMYOPATHY margin
^/ posteriorcardiacmarginprojects>1.8cm
Right Atrial Enlargement posteriorto IVC measuredat a point 2 cm above
0 RA enlargesin rightward+ posteriordirection intersectionof IVC with right hemidiaphragm
P A CX R: (H ofman-R i gl rul
er e)
^/ >S.Scm from midlineto most lateralRA margin
^/ >Z.Scm from rightvertebralmargin Cardiomegaly
in Newborn
r/ >sOy"verticalfrbigntof RA comparedwith A. NONCARDIOGENIC
cardiovascular mediastinal height(fromtop of (a) metabolic:
aorticarch to base of heart) 1. l on i mbal ance
i n seruml evel sof sodium ,
LAT CXR: potassium,and calcium
r/ doubledensityon left side of heart 2. Hypoglycemia
(b) decreasedventilation
Right VentricularEnlargement 1. A sphyxi a
0 RV enlargesin anterior,superior+ leftward 2. Transienttachypnea
directioncausingfevorotationof heart 3. Perinatalbraindamage
P A CX R: (c) erythrocytefunction
r/ straightening/ convexityof left uppercardiac 1. Anemia
contour 2. Erythrocythemia
r/ upturnedcardiacapex (d) endocrine
r/ tett uppercardiacmarginparallelsleft mainstem 1. Glycogenstoragedisease
bronchus 2. Thyroiddisease: hypo-/ hyperthyroidism
^/ increaseddistancebetweenleft uppercardiac (e) infantof diabeticmother
margin+ leftmainstembronchus (f) arteriovenousfistula
r/ small appearanceof rotatedaorticarch + SVC 1. V ei nof Gal enaneurysm
^/ largeappearanceof main pulmonaryartery 2. Hepaticangioma
LAT CXR: 3. Chorioangioma
r/ prominentconvexityof anteriorheartborder>1/3 B . C A R D IOGE N IC
distancefrom anteriorcardiophrenic sulcusto 1. A rrhythmi a
sternalangle 2. Myo-i pericarditis
3. Cardiactumor
Left Atrial Enlargement 4. Myocardialinfarction
0 LA enlargesin multipledirections 5. Congenitalheartdisease
P A CX R:
r/ right retrocardiacdoubledensity NeonatalCardiacFailure
./ >Z.O(female)/ 7.5 (male)cm distancebetween A . LE FT-S ID E DOB S TR U C TIV LE
E S ION S
midpointof undersurface of leftmainstem 1. Segmentalhypoplasiaof aorta
bronchus+ right lateralLA shadow 2. Criticalcoarctationof the aorta
^/ >75'splayingof carinawith horizontalorientation 3. Aorticvalve stenosis
of distalleftmainstembronchus 4. Asymmetricalseptalhypertrophy/ hypertrophic
r/ enlargementof left atrialappendage obstructivecardiomyopathy
+ calcifications
(in 90% due to rheumaticheart 5. Mitralvalvestenosis
disease) 6. Cor triatriatum
LAT CXR: B. VOLUMEOVERLOAD
r/ increasedconvexityof posterosuperior cardiac 1. Congenitalmitralvalveincompetence
margin 2. Correctedtranspositionwith left (= tricuspid)AV
! posterosuperior atrialconvexitycrossesvertical valve incompetence
planeformedby trachealmidline+ upperlobe 3. Congenitaltricuspidinsufficiency
bronchus 4. Osti umpri mumA S D
576 Radiology Review Manual

C. MYOCARDIALDYSFUNCTION / ISCHEMIA ACOUIRED HEARTDISEASE

1. Nonobstructive cardiomyopathy = MVdisease
r/ m enlargement
2. Anomalousoriginof LCA from pulmonarytrunk aorta= ?ofticvalvedisease
r/ dilatedascending
3. Primaryendocardialfibroelastosis ./ nn enlargement= tricuspid
valvedisease
4. Glycogenstoragedisease(Pompedisease)
5. Myocarditis Pressure Overload
D. NONCARDIACLESIONS 1. SystemichyPertension
1. AV fistulas:hemangioendothelioma of liver, 2. Aorticstenosis
AV fistulaof brain,vein of Galenaneurysm,large 3. Mitralstenosis
pulmonaryAV fistula
2. Transienttachypneaof the newborn DecreasedCompliance
/ subarachnoid
3. Intraventricular hemorrhage 1. Myocardialinfarction
4. Neonatalhypoglycemia(low birthweight,infants 2. HypertrophiccardiomYoPathY
of diabeticmothers) cardiomYoPathY
3. Restrictive
5. Thyrotoxicosis(transplacental passageof LATS
hormone) Volume Overload
1. AorticinsufficiencY
Congestive Heart Failure & Cardiomegaly 2. MitralinsufficiencY
mnemonic.' "Ma McCae& Go." 3. TricuspidinsufficiencY
Myocardialinfarction
anem ia
Malformation AORTA
cardiomyopathy Enlarged Aorta
Coronaryarterydisease PACXR:
aortic insufficiency { aorticknob>4.0cm measured fromindented
effusion tracheato mostlateralmarginof aorta
Coarctation r/ rightconvexcontouraboveRA margin+ lateral
displacementof SVC(= dilatation aorta)
of ascending
Congenital Cardiomyopathy A. INCREASED VOLUME LOAD
mnemonic; "CAVEGl" 1. AorticinsufficiencY
Cysticmedialnecrosisof coronaryarteries 2. PDA
Aberrant left coronary artery/ Absent coronarya. B. POSTSTENOTIC D]LATATION
Viral myocarditis 1. Valvularaorticstenosis
Endocardialf ibroelastosis C. INCREASED INTRALUMINAL PRESSURE
Glycogenstoragedisease(PomPe) 1. Coarctation
Infantof diabeticmother/ lschemia 2. Systemic hyPertension

Approachto AcquiredHeartDisease
Mild-M oderate Cardiomegaly Mode rate-Severe CardiomegalY

Cff ratio 0.45-0.55 >0.55

LA enlargement pressure overload volume overload

mitralstenosis mitralinsufficiency
decreased LV compliance
hypertrophiccardiomyopathy
restrictivecardiomyopathY
Ascendingaortic
enlargement pressure overload volume overload
aorticstenosis aorticinsufficiency

NormalLA + aorta myocardial myocardial

acute infarction dilatedcardiomyopathY
hypertrophiccardiomyopathy ischemic cardiomyoPathY
restrictivecardiomyopathy
pericardial pericardial
constrictivepericarditis pericardialeffusion
 DifferentialDiagnosisof CardiovascularDisorders 577

D. M URA LW E AK N E SS/ IN F EC T IO N AorticCalcifications

1. Cysticmedianecrosis:Marfan/ Ehlers-Danlos Intimal Calcification
syndrome
Cause: part of atherosclerotic plaque
2. Congenitalaneurysm
Associatedwith: inflammatorycells,lipid,vascular
3. Syphiliticaortitis
smoothmusclecells
4. Mycoticaneurysm
Site: withinperimeterof internalelasticlamina
5. Atherosclerotic aneurysm(compromisedvasa r/ discretepunctatelesionof radiograph
vasorum)
E. LACERATIONOF AORTICWALL
1. Traumaticaneurysm
Medial Calcification
Cause: aging,diabetes,end-stagerenaldisease,
2. Dissectinghematoma
neuropathy, geneticsyndromes
Associatedwith: elastin+ vascularsmoothmuscle
Aortic WallThickening
cells
1. Intramural
hematoma r/ lineardepositalongelasticlamellaeresembling
= oorticdissectionwithoutintimaltear
railroadtracks (when severe)
2. Aortitis
segmentsof aorticarch + branchvessels
3. Atherosclerotic plaque
r/ irregularnarrowingof aorticlumen DoubleAorticArch
most common+ serioustype of a completevascular
4. Adherentthrombus
ring;usuallyisolatedcondition
Embryology: failureof regressionof eitherarch
0 1 lncidence: 55Y"of all vascular rings
Age: usuallydetectedin infancy
. usuallyasymptomatic
. stridor,dyspnea,recurrentpneumonia
RSA
. dysphagia(lesscommonthan respiratory symptoms,
more commonafter startingbaby on solids)
Location: descendingaorta in 75/" on left,in 2So/"on
rightside;smallerarch anteriorin 80%; right
arch larger+ more cephaladthan left in g0T"
./ two separatearchesarisefrom singleascending
aorta
./ each arch joins to form a commondescendingaorta
I tracheain midline:
^/ impressionsmay be presenton both sides of
trachea:usuallyR > L (in olderchildren)
r/ tracheanarrowedand displacedposteriorlywith
smallanteriorimpression
Esophagogram:
Edwards' Hypothetical Aortic Arch Development d broad horizontalposteriorindentationat the levelof
RSA = right subclavian a.
3rd / 4th thoracicvertebra(by rightarch crossing
AAo = ascending aorta
LSA = left subclavian a. DAo = descending aorta , obliquelyto join leftarch)
RCC = right common carotid a.
r/ bilateralesophagealindentationswith a reversed
Rd = right ductus
LCC = left common carotid a.
S-shapedconfiguration (= rightindentation higher
Ld = left ductus
0 = normal left aortic arch
than left)
1 = right aortic arch with mirror-image branching; ductus from
C T:
r/ "four-arterysign" = e?ch arch gives rise to 2 dorsal
pulmonary a. to left brachiocephalic /subclavian a. = no
vascular ring
subclavian+ 2 ventralcarotidarteriesevenly
2 = ri^ghtaortic arch with mirror-image branching; ductus from
spacedaroundtracheaon sectioncephaladto
pulmonary a. to descending aorta = complete vascular ring
aortic arch
DDx: rightarch with aberrantleft subclavianartery
3 = right aortic arch with aberrant left subclavian a.; ductus
(indistinguishable by esophagogram when
from pulmonary a. to descending aorta (most common
complete vascular ring)
dominantarch on rightside)
4 = Ieft aortic arch with aberrant right subclavian a.
5 = right aortic arch with aberrant left brachiocephalic ffi"ry; Right Aortic Arch
ductus from pulmonary a. to descending aorta (very lncidence: 1-2o/"
uncommon) Embryology; persistenceof right aortic arch and right
2 + 3 = right aortic arch with isolated left subclavian a. (very
descendingaorta + regressionof left
uncommon)
aortic arch
578 Radiology Review Manual

aortic
diverticulum
RS
ALS

RCC DA

LD
LCC

Right Aortic Arch with Aberrant Left Subclavian Artery Right Aortic Arch with Mirror-image Branching

RS

RCC

RPA

Double Aortic Arch Aberrant Left Pulmonary Artery

ALS = aberrantleft subclaviana. LS = left subclaviana. LCC = left common carotid a. LD = left ductus arteriosus
LPA = left pulmonarya. PT - pulmonarytrunk RCC = right common carotid a. RPA = right pulmonary a.
RS = right subclaviana.

Course: to rightof trachea+ esophagus,over right Right Aortic Arch

mainstembronchus;crosseslowerthoracic with Aberrant Left SubclavianArtery
spine;passesthroughleft hemidiaphragm = RAA with ALSA
= interruptionof embryonicleft arch betweenleft CCA
I NCI DE NCE O F R IGH TAOR T ICA R C H IN and left subclavianartery
CONGENITALHEARTDISEASE 0 Most commontype of rightaorticarch anomaly
1. T r unc usa rte ri o s u.............
s 35% 0 2nd most commoncause of vascularring after
2. P ulm ona ry a tre s i a.............. 25% doubleaorticarch
3 . T e t r a l o goyf F a l l o .t . . . . . . . . . . . . . 2 0 % lncidence: 1:2,500; 35-72% of right aorticarch
4. Tricuspidatresia 15% anomal i es
5. DORV .12% Associatedwith: congenitalheartdiseasein 5-120/":
6. TGV........ ..........8 . .%.. 1. Tetralogyof Fallot (213 = 8/")
7. Lar geV S D ....... .. 2 % 2. ASDt VSD (114 = 3/o)
3. Coarctation ( 1 1 1 2= 1 " / o )
Rareanomalies:
1. Correctedtransposition . usuallyasymptomatic(looseringaroundtrachea
2. Pseudotruncus + esophagus)
3. Asplenia . may be symptomaticin infancyI earlychildhood
4. Pinktetralogy provokedby bronchitis+ trachealedema
 DifferentialDiagnosisof Cardiovascular
. may be symptomaticin adulthoodprovokedby
torsion of aorta
r/ tettcommoncarotidarteryis first branchof
ascendingaorta
! left subclavianarteryarisesfrom descendingaorta
via the remnantof the left dorsalaorticroot
./ bulbousconfigurationof originof LSA (= remn?ntof
embryonicleft arch) = retroesophageal aortic
diverticulum = diverticulum of Kommerell(N.B.:
originallydescribedas diverticularoutpouching at
originof rightsubclavianarterywith left aorticarch):
{ small roundeddensityleft lateralto trachea
r/ impressionon leftside of esophagussimulatinga
doubleaorticarch (by aorticdiverticulumor
, ductus/ ligamentumarteriosum)
! vascularring (= left ductusextendsfrom aortic
diverticulumto left pulmonaryartery):
r/ impressionon trachealair shadow(by rightaortic
arch)
r/ rightesophagealindentation(by rightaorticarch)
r/ masslikedensitysilhouettingtop of aorticarch
just posteriorto tracheaon LAT CXR (by
aberrantleft subclavianartery)
r/ broad posteriorimpressionoh.esophagus(left
subclavianartery/ aorticdiverticulum)
{ small anteriorimpressionon trachea(Oytett
commoncarotidartery)
r/ descendingaortaon iight side
Right Aortic Arch with Mirror-imageBranching
2nd most commonaorticarch anomaly: 24-60!:"
= interruptionof embryonicleft arch betweenleft
subclavian arteryand descendingaorta; dorsal to
left ductusarteriosus
(a) Type 1 = interruptionof left aorticarch distalto
ductusarteriosus(common)
Associatedwith: cyanoticCHD in gg/":
1. Tetralogyof Failot (87%)
2. Multipledefects (7.5%)
3. Truncusarteriosus (2-6%)
4. Transposition (1-10%)
5. Tricuspidatresia (5%)
6. ASD r VSD (0.5%)
0 25% of patientswith tetralogyhave right
aorticarch!
Q 37% of patientswith truncusarteriosushave
rightaorticarch!
i/ NO vascularring, NO retroesophageal
component
! NO structureposteriorto trachea
r/ R arch impressionon trachealair shadow
{ NORMALbariumswallow
(b) Type 2 = interruptionof left aorticarch proximalto
ductusarteriosus(rare)
true vascularring (if duct persists);
rarelyassociatedwith CHD
Disorders S7g
Right Aortic Arch
with Isolated Left SubclavianArtery
0 3rd most commonrightaorticarch anomaly: 2o/o
= interruptionof embryonicleft arch between
(a) left CCA and left subclavianarteryand
(b) left ductusand descendingaorta
resultingin a connectionof left subclavianartery
with left pulmonaryartery
Associatedwith: tetralogyof Fallot
r/ tettcommoncarotidarteryarisesas the first branch
r/ lett subclavian arteryattachesto left putmonary
arterythroughPDA
{ NO vascularring,NO retroesophageal component
. congenitalsubclaviansteal syndrome
Right Aortic Arch
with Aberrant Left BrachiocephalicArtery
Similarin appearanceto R aorticarch + aberrantL
subclavianartery
Left Aortic Arch
Left Aortic Arch with Aberrant Right
SubclavianArtery
= rightsubclavianarteryarisesas 4th branchfrom
proximaldescendingaorta
lncidence: 0.f23/. of population;in 37"/"ot
Downsyndromechildrenwith CHD;
0 Mostcommoncongenitalaorticarch
anomaly!
Associatedwith: (1) Absentrecurrentpharyngealn.
(2) C H D i n 10-15%
Course: (a) behindesophagus(80%)
(b) betweenesophagus+ trachea (15%)
(c) anteriorto trachea(5%)
. asymptomatic / dysphagialusoria(rare)
r/ soft-tissueopacitycrossingthe esophagus
obliquelyupwardtowardthe rightshoulder
(PATHOGNOMONTC)
r/ masslikeopacityin right paratrachealregion
r/ roundedopacityarisingfrom superioraorticmargin
posteriorto trachea+ esophaguson LAT CXR
^/ ditatedoriginof aberrantrightlubclavianartery(in
up to 60%) = diverticulum of Kommerell
= reffiflontof embryonicrightarch
r/ unilateralL-sidedrib notch-ing (if aberrantright
subclavianarteryarisesdistalto coarctation)
AnomalouslnnominateArtery Compression
Syndrome
= origihof R innominatearteryto the left of trachea
coursingto the right
r/ anteriortrachealcompression
. ablationof rightradialpulseby rigidendoscopic
pressure
{ posteriortrachealdisplacement
! focal collapseof tracheaat fluoroscopy
r/ pulsatileindentationof anteriortrachealwall by
innominatearteryon MRI
580 Radiology Review Manual

J--r.

!-t-!

A. Anteriortrachealindentation+ largeposterior B . Anteriortrachealindentation

esophagealimpression: 1. Compression arterywith origin
by innominate
1. Doubleaorticarch more distalalongaorticarch
2. Rightaorticarch with aberrantleft subclavian 2. Compressionby.left.common carotidarterywith
+ leftductus/ ligamentum arteriosus originmoreproximalon arch
3. Left aorticarch with aberrantrightsubclavian 3. Commonoriginof innominateand leftcommon
+ rightductus/ ligamentum(extremelyrare) carotidartery

TJJ

#i
!--

c. Smallposterioresophagealimpression D. Posterior
tracheal + anterior
indentation esophageal
. dysphagialusoria(lusorius,Latin= playful) impression
1. Left aorticarch with aberrantrightsubclavian leftpulmonary
1. Aberrant artery
artery
2. Rightaorticarch with aberrantleft subclavian

Pattern of Vascular Compression of Esophagus and Trachea

Rx: surgicalattachmentof innominatearteryto { indentation on rightlateralesophagealwall (by

m anu b ri u m
Vascular Rings
= aflorn?lycharacterizedby encirclementof trachea
+ esophagusby aorticarch + branches
A. USUALLYSYMPTOMATICLESIONS
. chronicstridor,wheezing,recurrentpneumonia
. dysphagia,failureto thrive
1. Doubleaorticarchwith R descendingaorta
+ L ductusarteriosus
2. R aorticarch with R descendingaorta + aberrant
L subclavianartery+ persistentL ductus/
ligamentumarteriosum
N.B.: leftobliteratedductusarteriosus
(= ligament of Botallo ) passesfrom
L pulmonarya. to descendingaorta/
L subclaviana.
. symptoms+ radiographic findingsidenticalto
doubleaorticarch
aorticarch)
r/ impressionon the anterolateralesophageal
wall (by ligamentof Botallo)
ri originof L subclavianarteryfrequentlydilated
3. L archwith L descendingaorta+ R ductus/
l i gamentum
4. AberrantL pulmonaryartery- "pulmonarysling"
Frequency of CXR findings:
- frontalCXR:
r/ rightaorticarch (85%)
./ focal indentationof distaltrachea(73%)
lateralCXR:
{ anteriortrachealbowing(92%)
^/ increasedretrotrachealopacity(79%)
{ focaltrachealnarrowing(77%)
B. OCCASIONALLY SYMPTOMATICLESIONS
1. AnomalousR innominateartery
2. AnomalousL commoncarotidartery/ common
trunk
 DifferentialDiagnosisof cardiovascularDisorders sgl

3. R aorticarch with L descendingaorta + L ductus/ 2. Prolapsingaorticvalve vegetation

ligamentum
C. USUALLYASYMPTOMATIC LESIONS 3. NarrowedLVOT (<20 mm)
1. L aorticarch + aberrantR subcfavianarlery (a) Long-segmentsubaorticstenosis
2. L aorticarch with R descendingaorta r/ aorticvalve closurein early systolewith coarse
3. R aorticarch with R descendingaorta + mirror- fluttering
imagebranching ./ trigtr-trequency
flutterof mitralvalve in diastole
4. R aorticarch with R descendingaorta + aberrant (aorticregurgitation)
L subclavianartery d symmetricLV hypertrophy
5. R aorticarch with R descendingaorta + isolation (b) ASH / tHSS
of L subclavianartery { asymmetricalfy thickenedseptumbulginginto
6. R aorticarch with L descendingaorta + L ductus/ LV + LVOT
ligamentum { systolicanteriormotionof aML (SAM)
(c) Mitralstenosis
AorticStenosis (d) Endocardial cushiondefect
A. ACQUIRED
1. Takayasuaortitis
2. Radiationaortitis SITUS
3. Aorticdissection = "position/ site / location"referringto the positionof the
4. Infectedaorticaneurysmwith abscess atriaand viscerarelativeto the midline
5. Pseudoaneurysm from laceration (a) systemic/ rightatrium
6. Atherosclerosis(rare) r/ has a broad-basedappendage
7. Syphiliticaortitis(rare) r/ receivesbloodfrom IVC
B . CO NG E NIT A L r/ has terminalcrest + coarsepectinatemuscles
1. W illiam ss y n d ro me (b) pulmonary/left atrium
2. Neurofibromatosis ^/ has a small narrowappendage
3. Rubella r/ receivesbloodfrom pulmonaryveins
4. Mucopolysaccharidosis
5. Hypoplasticleft heartsyndrome A. SITUSSOLITUS= horffi?l/usualsitus
. on rightside: r/ systemicatrium
AbnormalLeft VentricularOutflowTract ^/ trilobedlung
LVOT = €lf€€lbetweenIVS + aML from aorticvalve r/ liver
cuspsto mitralvalve leaflets r/ gallbtadder
1. Membranoussubaorticstenosis r/ tvc
= cf€scert-shaped fibrousmembraneextending ' on leftside: r/ pulmonaryatrium
acrossLVOT + insertingat aML ri bilobedlung
r/ diffusenarrowingof LVbT r/ stomach
^/ abnormallinearechoesin LVOT space r/ singlespleen
(occasionally) r/ aorta

eparterial
bronchus

& 'RA )
LL

\lar

I 7T -4
ll
ii ,-/,2
,/ /"1
"r'/r/'

-r' ( r--.-
Situs Solitus Situs Inversus Left Isomerism Right Isomerism
anterior view posterior view posterior view

Situs Anomalies
582 Radiology Review Manual
Associated with:
(a) levocardia : 0.6-0.8%chancefor CHD
(b) dextrocardia: 95h chancefor CHD
B. S I T USI NV E R S U S
= mirror-image arrangement of situssolitus
Frequency: 0.01"/"
. on leftside: r/ systemicatrium
^/ trilobedlung
r/ liver
r/ gallbladder
./ tvc
. on rightside: r/ pulmonaryatrium
./ bilobedlung
r/ stomach
{ singlespleen
r/ aorta
Associated with:
(a) dextrocardia= situs inversustotalis(usual
variant):3-5% chancefor CHD, eg, Kartagener
syndrome(in 20%)
(b) levocardia(extremelyrare): 95'h chancefor
CHD
C. S I T USA M B IGU U S= H ET E R OT A XIA
= visceralmalposition+ dysmorphismassociatedwith
indeterminate atrialarrangement
Subclassification:
1. Aspleniasyndrome= doubleright-sidedness
= rightisomerism= lvemarksyndrome
r/ both lungs have 3 lobes
r/ eparterialbronchi= rn?inbronchuspasses
superiorto ipsilateralmain pulmonaryartery
2. Polysplenia syndrome= doubleleft-sidedness
= left isomerism
^/ notn lungshave2lobes
r/ hyparterialbronchi= m?in bronchuspasses
inferiorto ipsilateralmain pulmonaryartery
Associatedwith: CHD in 50-100%
Cardiac Malposition
(a) locationof heartotherthan within left hemothoraxin
situssolitus
(b) locationof heartwithin left hemithoraxwhen other
organsare ambiguous
0 Determinedby base-apexaxis; no assumptionis
made regardingcardiacchamber/ vessel
arrangement
A. POSITIONOF CARDIACAPEX
1. Levocardia= apex directedleftward
2. Dextrocardia= apex directedrightward
3. Mesocardia= vertical/ midlineheart(usuallywith
situssolitus)
{ atrialseptumcharacteristically bowed into left
atriumin cardiacsitus solituswith dextrocardia
+ cardiacsitusinversuswith levocardia(DDx:
juxtapositionedatrialappendages)
B . CA RDI A CD IS PL A C E M EN T
by extracardiacfactors(eg, lung hypoplasia,
pulmonarymass)
1. Dextroposition
suggestshypoplasiaof ipsilateralpulmonary
artery(PAPVRimpliesscimitarsyndrome)
2. Levoposition
3. Mesoposition
C . C A R D IA CIN V E R S ION
= alterationof normalrelationshipof chambers
1. D-bulboventricular loop
2. L-bulboventricular loop
D. TRANSPOSITION
= alterationof anterior-posterior
relationshipof great
vessels
CARDIACTUMOR
Prevalence: 0.001-0.03%(autopsyseries)
0 Rare often asymptomatictumorsuntilvery largel
. symptomsof cardiopulmonary diseases:
. congestiveheartfailure: dyspnea,orthopnea,
peripheraledema,paroxysmalnocturnaldyspnea
. palpitations,heartmurmur
. cough,chestpain
. symptomscausedby peripheralembolito cerebral/
systemic/ coronarycirculation:
. syncope
. weightloss,fever,malaise
Location: intracavitary(obstruction,emboli),intramural
(arrhythmia),pericardial(tamponade)
CXR:
{ cardiomegaly, pericardial
effusion
{ si gnsof C H F
{ abnormalcardiaccontour
{ pleuraleffusion
BenignHeartTumorin Adults
0 Morecommonthan malignantneoplasms
1. Myxoma(mostcommonprimarycardiactumor;50%
of all primarycardiactumors)
2. Papillaryfibroelastoma (10/" of all primarycardiac
tumors;mostcommonvalvulartumor)
3. Lipoma
4. Hydatidcyst (uncommon):
r/ localizedbulge of left cardiaccontour
{ curvilinear/ spottycalcifications(resembling
myocardialaneurysm)
Cx: may ruptureinto cardiacchamber/
pericardium
MalignantHeartTumors
Prevalence: 25/" of all cardiactumors in adults
10/" of all cardiactumorsin children
1. S arcoma:
0 Majorityof primarymalignantcardiacneoplasms!
0 2nd mostcommonprimarycardiacneoplasm
2. Rhabdomyosarcoma
0 Mostcommonprimaryin children
3. Metastaticdisease
(a) to peri-/ epicardium
lung > breast> lymphoma> leukemia(most
commonly)
 DifferentialDiagnosisof cardiovascularDisorders sgg

(b) myocardium:malignantmelanoma;secondary 1. Aortic valve

extensionto myocardium from epicardium . usuallyindicatessignificantaorticstenosis
(c) endocardiallintracavitary(in only 5%) Cause:
0 20-1000timesmorefrequentthan primarytumor! congenitalbicuspidvalve (70-85%)>
4 . Lymphoma(rare) atherosclerotic degeneration> rheumaticaortic
5 . Malignantteratoma stenosis(rare),syphilis,ankylosingspondylitis
6. Multiplecardiacmyxomas Location: above+ anteriorto a lineconnecting
carina+ anteriorcostophrenic angle
(lateralview)
CongenitalCardiacTumor (a) Congenitalbicuspidaorticvalvularstenosis
lncidence: 1:10.000 . calcium first detectedat an average age of
1. Rhabdomyoma (58%): usuallymultiplemasses 28 years
2. Teratoma(20%): intrapericardiac, extracardiac 0 In patients<30 years aorticvalvecalcifications
r/ multicysticmass
are mostlydue to a bicuspidaorticvalve!
3. Fibroma(12%): intramural r/ usuallyextensiveclusterof heavydense
may be associatedwith: Gorlin syndrome calcificdepositsassuminga nodularcontour
Location: free LV wall / interventricular
septum r/ poststenoticdilatationof iscending aorta
./ may be pedunculated
(b) lsolatedrheumaticaorticstenosis
r/ calcificationand cysticdegenerationcentrally . calciumfirst detectedat an averageage of
r/ tendencyfor slow growth 47 years
Cx: fetal hydropssecondaryto obstruction, 0 In patients30-60 years of age aorticvalve
pericardialeffusion,fetalarrhythmia,
fetal calcificationsuggestsrheumatic valvedisease!
death r/ clusterof heavydense calcificdeposits
4. Hemangioma(arisefrom RT atrium,pericardial
withoutbicuspidcontour
effusion,skin hemangiomas), lymphangioma, (c) Degenerativeaorticstenosis
neurofibroma, myxoma,mesothelioma: . calciumfirst detectedat an averageage of
^/ mass-occupying lesionimpinginguponcardiac 54 years
cavities 0 ln patients>65 years aorticvalve calcification
in 90% due to atherosclerosis!
CardiacTumorby Location ! curvilinear shapeof calciumoutliningtricuspid
A. ENDOCARDIAL / INTRACAVITARY leaflets
1. M y x om a r/ diffusedilatation+ tortuosityof aorta (NO
2. Thrombus poststenoticdilatation)
3. Myofibroblastic sarcomas(MFH,leiomyosarcoma, 2. Mitral valve leaflet
fibrosarcoma,myxosarcoma) Cause: rheumaticheartdisease(virtually
B. VALVULAR always),mitralvalve prolapse
1. Papillaryfibroelastoma Location: inferiorto a lineconnectingcarina
2. Vegetations + anteriorcostophrenicangle (on lateral
3. Thrombus view)
4. Myxoma . calciumfirst detectedin earlythirtieswhen
C. MYOCARDIAL/ INTRAMURAL patientsbecomeovertlysymptomatic
1. Rhabdomyoma:multifocal, highsignalon T2Wl { delicatecalcificationsimilarto coronaryarteries
2. Fibroma: alwayssolitary,calcifications, cystic (DDx: calciumin RCAi LCX)
degeneration,low signalon T2Wl r/ superior-to-inferior motion
D. E NDO -/ M Y O -i E PIC AR D IA L 3. Pulmonic valve
1. Lipoma Cause: tetralogyof Fallot,pulmonarystenosis,
2. Sarcoma atrialseptaldefect
3. Primarycardiaclymphoma:rightheart,multifocal, { calcificpatternsimilarto calcifiedmitralvalve
extensionintopericardium 4. Tricuspid valve (extremelyrare)
Cause: rheumaticheartdisease,septaldefect,
tricuspidvalve defect,infective
CAR DIAC CALCI FICATIONS endocarditis
Detectedby: @ A nnul us
fluoroscopy(at low-beamenergies<75 kVp is 57% = vdlv€ ringsserve as fibrousskeletonof the heartfor
sensitive)< digitalsubtractionfluoroscopy attachmentof myocardialfibers+ cardiacvalves
< conventionalCT < ultrafastCT (96% sensitive) 1. Mi tralannul us
@ Coronaryarteries Cause: degenerative(physiologicin elderfy)
@ Cardiacvalves Age: >65 years
0 Valvarcalcificationmeansstenosis- its amountis May be associatedwith: mitralvalve prolapse
proportionate to degreeand durationof stenosisl Commonlyassociatedwith: aortic valve calcium
584 Radiology Review Manual

r/ dense bandlikecalcificationstartingat posterior @ Endocardium

aspect+ progressinglaterallyfrequentlyforming Cause: cardiacaneurysm,thrombus, endocardial
a " r e v e r s e d Cl^" O l " U l " J fibroelastosis
Cx: mitralinsufficiency (dueto impairedanterior @ Pulmonaryartery
mitralleaflet),atrialfibrillation,heartblock Cause: severeprecapillarypulmonaryarterial
(due to infiltrationinto posteriorwall hYPertension, sYPhilis
conductionPathwaY) @ Ductusarteriosus
2. Aortic annulus (a) in adults: indicatespatencyof ductuswith
{ usuallyin combinationwith degenerativeaortic associatedlong-standingprecapillary
valve calcification pulmonaryhyPertension
3. Tricuspid annulus (b) in children: ductuslikelyclosed
Associatedwith: long-standingRV hypertension rl calciumdepositionin ligamentof Botallo
Location: rightAV groove
r/ bandlikeC-shapedconfiguration CoronaryArtery Calcification
@ Pericardium 0 The amountof coronarycalcificationcorrelateswith
Cause: idiopathicpericarditis, rheumatoidarthritis the extentof atherosclerosis!
(5%),tuberculosis, viral,chronicrenal 0 The absenceof calcificationimpliesthe absenceof
f ailure,radiotherapyof mediastinum angiographically significantcoronaryvesselnarrowingl
Location: calcificationover less pulsatileright-sided Cause: (1) arteriosclerosisof intima
chambersalong diaphragmaticsurface, (2) Mdnckebergmedialsclerosis(exceedingly
atrioventricular grooves,pulmonarytrunk rare)
0 50% of patientswith constrictivepericarditisshow Histo: calcifiedsubintimalplaques
pericardialcalcifications! PathophysiologY:
Cx: constrictivePericarditis injuryto endotheliumallowscirculatinghistiocytesto
@ Myocardium lodge in vesselwall where they are transformedinto
Caus e: in fa rc ti o n ,a n e u ry s m ,rh e u m a t i cfever, macrophages;these accumulatelipids("fattystreaks"
myocarditis beneathsurfaceendothelium); lipidscalcify;thethin
Frequency: in 8% post myocardialinfarction;M > F fibrouscap overlyinglipiddepositsmay rupture
Location: apex / anterolateralwall of LV (coincides allowingcirculating bloodto mounta thrombogenic
with LAD vasculardistribution+ typical reactionresultingin narrowingof lumen
locationof LV aneurYsms) 0 Calciumis depositedas calciumhydroxyapatite in
r/ tnin curvilinear contouroutlinesthe aneurysm hemorrhagic areaswithinatheromatous plaques!
{ shaggylaminatedcalcificationsuggestscalcification Location:"coronaryarterycalcificationtriangle"
of associatedmuralthrombus = triangularareaalong mid left heartborder,
./ coarseamorphouscalcificationsare causedby spine,and shoulderof LV containingleftmain
trauma,cardioversion, infection,endocardial fibrosis coronaryartery,proximalportionsof LAD
@ Interventricular septum + LCX calcificationsat autopsy: LAD (93%)'
Location: triangularfibrousarea betweenmitral LCX (77%),left main CA (70/"), RCA (69%)
+ tricuspidannuli(= trigonafibrosa) CXR (detectionrate uP lo 42/"):
representingthe basalsegmentof 0 Indicatingmore severecoronaryarterydisease
interventricular septum,closelyrelatedto i/ parallelcalcifiedlines (lateralview)
bundleof His Fluoroscopy:(promotedas inexpensivescreeningtest)
Always assaciated with: (a) asymptomaticPoPulation
heavycalcificationof mitralannulus/ aorticvalve - calcifications in34/" in asymptomatic male
Cx: heart block individuals
- in 35% of patientswith calcifications exercise
@ Left atrialwall
. Cause: rheumaticmitralvalve disease testwill be positive(withoutcalcifications only
(a) diffuseform in 4o/oPositive)
. patientusuallyin bilateralCHF + atrialfibrillation - calcificationsindicate>50/" stenosiswith
./ diffusesheetlikecalcificationstartingin the 72-7 6h sensitivity,78y" specificity); f requency
appendagesparingposterolateral wall on right of coronaryarterycalcifications with normal
side angiogramincreaseswith age; predictive
Cx: muralthrombusformation+ emboli values in population<50 years as good as
(b) localizedform exercisestresstest
r/ nodularcalcificscar in posteriorwall (= McOallum (b) symptomatic PoPulation
patch)due to injuryfrom a forcefuljetin mitral - in 54'h of symptomaticpatientswith ischemic
valve insufficiency heartdisease
@ Cardiactumor '10% 0 In symptomaticpatients94% specificityfor
atrialmyxoma(in calcified),rhabdomyoma, obstructivedisease(>75/" stenosis)of at least
fibroma,angioma,osteosarcoma,osteoclastoma one of the three majorvessels!
 DifferentialDiagnosisof Cardiovascular
Disorders 555

CT : r/ rapidlyappearingcardiomegaly+ normal
(a) electronbeam: thresholdof +130 HU pulmonaryvascularity
(b) spiralCT: thresholdof +90 HU ^/ "differentialdensitysign" = increasein lucencyat
Clinicaloutcome: heart marginsecondaryto slightdifferencein
(a) for coronarycalcificationsdetectedat fluoroscopy: contrastbetweenpericardialfluid + heart muscle
5.4% eventriskat l year(vs.2.1%without ^/ diminishedcardiacpulsations
calcification) E C H O:
(b) for electronbeam CT a calcificationscore of >100 r/ separationof epi- and pericardialechoesextending
is highlypredictiveto identifypatientswith events into diastole(rarelybehindLA)
Prognosis: 58/" S-yearsurvival rate with and B7"h ^/ volumeestimatesby M-mode:
withoutcalcifications (a) separationonly posteriorly= <300 mL
(b) separationthroughoutcardiaccycle
= 300-500 mL
(c) plusanteriorseparation= >1000mL
PERICARDIUM
PericardialEftusion
= pericardial fluid>50 mL Pneumopericardium
Etiology: Etiology: shearingmechanismof injuryof the heart
A. SEROUSFLUID= transudate duringblunttrauma
congestiveheartfailure, hypoalbuminemia, Path: tear in fibrouspericardium, usuallyalongthe
irradiation courseof the phrenicnerve,allows
B. BLOOD = hemopericardium pneumomediastinal air to enter
(a) iatrogenic:cardiacsurgery/ catheterization, r/ tnicf shaggysoft-tissuedensityof fibrouspericardium
anticoagulants,chemotherapy separatedby air from cardiacdensity
(b) trauma: penetrating/ nonpenetrating r/ air limitedto distributionof pericardialreflection
(c) acute myocardialinfarction/ rupture
(d) ruptureof ascendingaorta/ pulmonarytrunk
(e) coagulopathy PericardialTumor
(f) neoplasm: mesothelioma,sarcoma,teratoma, 1. Pericardialteratoma:benigntumorof infants
fibroma,angioma,metastasis(lung,breast, + children
lymphoma,leukemia,melanoma) 2. Pericardialmesothelioma:malignanttumorof
C. LY M P H adulthood
neoplasm,congenital,cardiothoracicsurgery, DDx: pericardialinvasion(sarcoma,lymphoma)
obstruction of hilum/ SVC
D. FIBRIN= eXudate
(a) infection:viral,pyogenic,TB
(b) uremia: 18/" in acuteuremia;51"/"inchronic VENACAVA
uremia;dialysispatient Venacavaanomalies
(c) collagendisease: rheumatoidarthritis,SLE, Circumaortic Left Renal Vein
acute rheumaticfever Prevalence: 1.5-8.7%
(d) hypersensitivity Etiology: persistenceof anterior intersubcardinal
mnemonic; "CUMTAPPIT RV" posteriorintersupracardinal anastomosis
Collagenvasculardisease r/ venouscollarencirclingaorta
Ur em ia r/ superiorleft renalvein crossesaorta anteriorly
Metastasis r/ inferiorleft renalvein receivesleft gonadalvein
Trauma + crossesaorta posteriorly1-2 cm belowthe
Acute myocardialinfarction superiorleft renalvein
Purulentinfection Significance: preoperativeplan for nephrectomy
Post Ml syndrome
ldiopathic DuplicatedIVC
Tuberculosis = DOUBLE IVC
Rheumatoidarthritis Prevalence: 0.2-3'/"
Virus Etiology: persistenceof both supracardinalveins
CX R: r/ small/ equal-sizedleft IVC formedby left iliacvein
r/ normalwith fluid <250 mL / in acute pericarditis r/ crossoverto right IVC via left renalvein / or more
{ "waterbottleconfiguration" = symmetrically inferiorly
enlargedcardiacsilhouette ./ crossoverusually anterior/ rarely posteriorto aorta
r/ loss of retrosternalclear space Significance: recurrentpulmonaryembolismafter
r/ "fat-padsign" = sep?r?tionof retrosternalfrom IVC filterplacement
epicardialfat line>2 mm (15%) DDx: leftgonadalv.la., inferiormesentericv.
586 Radiology Review Manual

Doualr IVC wrrn Rernoaonnc RrcHr Rerual Vetru Retroaortic Left Renal Vein
nno Azvcos CoNTNUAnoN or IVC Prevalence: 1.8-2.1%
Etiology: persistenceof left supracardinalv. and Etiology: persistenceof posteriorintersupracardinal
dorsallimbof renalcollar+ regressionof anastomosis + regressionof anterior
ventrallimb + failureof formationof right anastomosis
intersubcardinal
subcardinal-hepatic anastomosis r/ crossoverusuallybelow/ occasionallyat levelof
rightrenalvein
Douele IVC wmr Rernononlc Rlcnr Renal Velru
ANDHEMTAzYGos CorurruulrroHor IVC
Etiology: persistenceof left lumbar+ thoracic IVCObstruction
supracardinalv. + leftsuprasubcardinal A . IN TR IN S ICOB S TR U C TION
anastomosis + failureof formationof right (a) neoplastic(mostfrequent)
subcardinal-hepatic anastomosis 1. Renalcell carcinoma(in 10A), Wilmstumor
r/ rightIVC and rightrenalveinjoin the left IVC and 2. Adrenalcarcinoma,pheochromocytoma
continuecephaladas the hemiazygosvein 3. Pancreatic carcinoma,hePatic
r/ hemiazygosvein followsalternativepathways: adenocarcinoma
(a) crossesposteriorto aorta at T8/9 and joins 4. Metastaticdiseaseto retroperitoneal lymph
the rudimentary azygosvein nodes (carcinomaof ovary,cervix,prostate)
(b) continuescephalad+ joinscoronaryvein via (b) nonneoplastic
persistentleft SVC 1. l di opathi c
(c) accessoryhemiazygoscontinuationto left 2. Proximallyextendingthrombusfrom
brachiocephalic vein femoroiliac veins
r/ hepaticsegmentof IVC drainsinto rightatrium 3. Systemicdisorders:coagulopathy,Budd-
Chiarisyndrome,dehydration, infection
(pelvicinflammatory disease),sepsis,CHF
lnterrupted IVC with Azygos / Hemiazygos 4. Postoperative / traumaticphlebitis,ligation,
Continuation plication,clip,cavafilter,severeexertion
see AZYGOSCONTINUATION B . IN TR IN S ICC A V A LD IS E A S E
(a) neoplastic
Left IVC 1. Leiomyoma,leiomyosarcoma, endothelioma
= TRANSPOSITION OF IVC= SOLITARY LEFTIVC (b) nonneoplastic
Prevalence: 0.2-0.5% 1. C ongeni tal membrane
Etiology: persistenceof left + regressionof right C . E X TR IN S ICC OMP R E S S ION
supracardinal vein (a) neoplastic
d tett IVC usuallyjoins left renalvein 1. Retroperitoneal lymphadenopathy (adults)
r/ crossoveras left renalvein usuallyanteriorI rarely due to metastaticdisease,lymphoma,
posteriorto aorta granulomatous disease(TB)
DDx: left-sidedparaaorticadenopathy 2. Renal+ adrenaltumors(children)
Significance: difficulttransjugularaccessto 3. Hepaticmasses
infrarenalIVC filterplacement 4. Pancreatictumor
5. Tumor-induced desmoplastic reaction(eg,
PersistentLeft SVC metastaticcarcinoid)
= BILATERAL SVCs (b) nonneoplastic
Prevalence: 03% of generalpopulation; 1. Hepatomegaly
4.3-11"/"o'fpatientswith CHD 2. TortuousaortaI aorticaneurysm
Etiology: failureof regressionof left anterior 3. Retroperitoneal hematoma
+ commoncardinalveins+ leftsinushorn 4. Massiveascites
May be associatedwith: ASD, azygos continuation 5. Retroperitoneal fibrosis
of IVC D . FU N C TION AOB L S TR U C TION
Course: lateralto aorticarch, anteriorto left hilum 1. Pregnantuterus
r/ tett SVC drainsinto enlargedcoronarysinus 2. Valsalvamaneuver
(common) 3. StrainingI crying(in children)
./ tett SVC drainsinto LA (rare)creatinga R-to-L 4. Supinepositionwith largeabdominalmass
shunt(increasedprevalenceof CHD) E. COLLATERALPATHWAYS
{ hemiazygosarch formedby left superiorintercostal 1. Deeppathway:ascendinglumbarveinsto azygos
vein + persistentleft SVC (20%) vein (right)+ hemiazygosvein (left)
{ absent/ small left brachiocephalic vein (65%) + intravertebral, paraspinal,extravertebralplexus
r/ absenceof rightSVC (10-18%) (Batsonplexus)
{ anastomosisbetweenright+ left anteriorcardinal 2. Intermediate pathway:via periureteric plexus
veins(in 35%) + left gonadalvein to renalvein
 DifferentialDiagnosisof Cardiovascular
Disorders SB7

Medium- Sized-Vessel Vasculitis

(polyarteritisnodosa,Kawasakidisease) capillary
arteriole
arteries

Goodpasture syndrome

isolated cutaneousleukocytoclastic angiitis

Large-Vessel Vasculitis Henoch-Schdnlein

purpura,cryoglobulinemic
vasculitis
(eg,giant-cellarteritis,Takayasu
disease)

Small-VesselVasculitis
(microscopicpolyangiitis,Wegenergranulomatosis,
Churg-Strauss
syndrome)

Noninfectious Vasculitides

3. Superficialpathway: externaliliacvein to inferior CARDIAC SURGERY

epigastricvein + superiorepigastricvein + internal SurgicalProcedures
mammaryvein into subclavianvein A. AORTICOPULMONARY
WINDOW
SHUNT
4. Portalpathway: retrogradeflow throughinternal = side-to-sideanastomosisbetweenascending
iliacvein + hemorrhoidalplexusintoinferior aorta and left pulmonaryartery(reversible
mesentericvein + splenicvein into portalvein procedure)
0 Tetralogyof Fallot
VASCULITIS B. BLALOCK-HANLON PROCEDURE
= inflammationand necrosisof vesselwall = surgicalcreationof ASD
A. LARGE-VESSEL VASCULITIS 0 Completetransposition
1. Giantcell (temporal)arteritis C. BLALOCK-TAUSSIG SHUNT
2. Takayasudisease = end-to-sideanastomosisof subclavianarteryto
B . M E DI UM - S IZ ED -VE SS EL
VA SC U L IT IS pulmonaryartery,performedipsilateralto
1. Polyarteritis nodosa innominateartery/ oppositeto aorticarch
2. Kawasakidisease ModifiedBlalock-Taussig shuntusessyntheticgraft
3. Drug-induced vasculitis: materialsuch as polytetrafluoroethylene (Gore-
methamphetamine Tex@)in an end-to-sideanastomosisbetween
cocaine: neurovascular, cardiovascular subclavianartery+ ipsilateralbranchof pulmonary
complications,aorticdissection,venous artery
thrombosis,mesentericarterythrombosis,renal 0 Tetralogyof Fallot,tricuspidatresiawith pulmonic
infarction stenosis
C. SMALL-VESSEL VASCULITIS D. FONTANPROCEDURE
(a) ANCA-associated small-vesselvasculitis (1) externalconduitfrom rightatriumto pulmonary
(= antineutrophilcytoplasmicautoantibodies) trunk(= vehousreturnenterspulmonaryartery
1. Wegenergranulomatosis directly)
2. Churg-Strausssyndrome (2) closureof ASD: floor constructedfrom flap of
3. Microscopicpolyangitis atrialwall and roof from pieceof prosthetic
(b) immune-complex small-vesselvasculitis material
1. Henoch-Schdnlein purpura 0 Tricuspidatresia
2. Essentialcryoglobulinemic vasculitis E. G L E N NS H U N T
3. Cutaneousleukocytoclastic angitis = end-to-sideshunt betweendistalend of right
others: lupus,rheumatoid, Sjogren,Behget, pulmonaryarteryand SVC; reservedfor patients
Goodpasture,serumsickness,drug- with cardiacdefectsin which total correctionis not
induced,hypocomplementemic urticaria anticipated
(c) inflammatoryboweldiseasevasculitis 0 Tricuspidatresia
F. NORWOODPROCEDURE
Multiple Aneurysms (1) constructionof "neoaorta"from aorticarch
1. Polyarteritis
nodosa + descendingaorta + main pulmonaryartery
2. Rheumatoid vasculitis supplyingcoronaryand systemiccirculation
3. Systemiclupuserythematosus (2) communicationbetweenRV as systemicventricle
4. Churg-Strauss syndrome and systemiccirculation
588 Radiology Review Manual

LV+
pulmonary
trunk

Fontan Procedure Norwood Procedure Mustard Procedure

(lateralview into openedright atrium)

(3) shuntbetweeninnominateartery+ main 3. VSD repair:throughRA

pulmonaryarteryto controlpulmonaryarterial 4. Mitralvalvecommissurotomy
bloodflow B . O N L E F TS I D E
(4) excisionof distalductusarteriosus+ atrialseptum 1. PDA
to preventpulmonaryvenoushypertension 2. Coarctation
0 Hypoplasticleft heart syndrome 3. PA banding
G. POTTSHUNT 4. Mitralvalvecommissurotomy
= side-to-sideanastomosisbetweendescending 5. Systemic-PA shunt,Pott
shunt: Blalock-Taussig
aorta + left pulmonaryartery shunt
0 Tetralogyof Fallot
H. M US T A RDPR O C ED U R E
(a) removalof atrialseptum HeartValveProsthesis
(b) pericardialbaffleplacedinto commonatriumsuch 1. Starr-Edwards
that systemicvenous blood is reroutedinto left ri caged ball
ventricleand pulmonaryvenousreturninto right 0 Predictableperformancefrom large long-term
ventricleand aorta experience
0 Completetransposition 2. Bjark-Shiley / St. Jude
/ Lillehei-Kaster
L RASHKINDPROCEDURE = balloonatrialseptostomy r/ tiltingdisk
0 Completetransposition 0 Excellenthemodynamics, very low profile,durable
J. RASTELLIPROCEDURE 3. Hancock/ Carpentier-Edwards (= porcinexenograft)
externalconduit(Dacron)with porcinevalve lonescu-Shiley (= bovinexenograft)
connectingRV to pulmonarytrunk 0 Low incidenceof thromboembolism, no hemolysis,
0 Transposition centralflow, inaudible
K. WATERSTON-COOLEY SHUNT
= sideto-side anastomosisbetweenascendingaorta
and rightpulmonaryartery;
(a) extrapericardial (WATERSTON) PULSUSALTERNANS
(b) intrapericardial(COOLEY) = alternatingarterialpulseheightwith regularcardiac
0 Tetralogyof Fallot rhythm
1. lntrinsicmyocardialabnormality
PostoperativeThoracicDeformity severeleft ventriculardysfunction(CHF,aorticvalvular
A . O N R I G H TS I D E disease,hypothermia, hypocalcemia,hyperbaric
1. Systemic-PA shunt: Blalock-Taussig
shunt, stress,ischemia)
Waterston-Cooley shunt,Glennshunt,central 2. Alternatingend-diastolicvolumes
conduitshunt abnormalitiesin venousfilling+ return(obstructed
2. Atrialseptectomy:Blalock-Hanlon procedure venousreturn,IVC balloon
 Differential Diagnosis of CardiovascularDisorders

C'
I /

I
i /

ii

Central Venous Line Position

C = coronary sinus, M = middle cardiac vein, P = main pulmonary artery,pER = perforation
590 Radiology Review Manual

ANATOMYAND ECHOCARDIOGRAPHY
CARDIOVASCULAR

<12
PCWP

Normal Blood Pressures Development of Major Blood Vessels

PCWP = pulmonary capillary wedge pressure numbers refer to embryologic aortic arches
most portions of aortic archesI, II, V regress

PulmonaryValve
right cusp
anterior cusp

transverse - left cusp

pericardial
sinus supraventricularcrest

parietal band medial papillary

muscle

septal band

Tricuspid Valve
moderator band
anterior cusp
septal cusp
postenor cusp

posterior + anterior papillary muscles

Right Ventricle Viewed from Front

Demarcation between posteroinferior inflow portion and anterosuperior outflow portion
by prominent muscular bands forming an almost circular orifice
parietal band
crista supraventricularis
septomarginal trabeculae (- septal band + moderator band)
Anterior papillary muscle originates from moderator band!
Ao = aorta IVC = inferior vena cava PT - pulmonic trunk
RA = right atrium SVC = superior vena cava
 Cardiovascular
Anatomyand Echocardiography 591

v n a frequency
shift

t
I I IECG
1I,-^-r/+r/r
NormatHepaticWaveform rricuspiJf,leglrgitation Pulsatility

Type 2 Type 3
TFicuspidRegurgitation TricuspidRegurgitation
DopplerWaveformsof HepaticVeins
S wave = systolic wave resulting from negative RA pressure
causedby atrial relaxation + movement of tricuspid
anulus toward cardiac apex
v wave = resulting from elevated RA pressurecausedby RA
overfilling against a closed tricuspid valve; occurs
in <50Voof patients
D wave = diastolic wave resulting from negative RA pressure
causedby opening of tricuspid valve + blood flow
from RA into RV; equal to / smaller than S wave
a-wave = resulting from elevated RA pressurecausedby RA
contraction; in 66Voof patients
PULSATILITY
= ?SSesSrTlentof vascularresistance(increased
resistancereducesdiastolicflow)
0 Can be assessedin vesselstoo small/ tortuousto be
imaged(Dopplerangleunnecessary) !
0 lndex shouldbe calculatedfor each of severalcardiac
cycles(5 heartbeatsadequate)an averagevalue taken
S = A = maximalsystolicshift
D = B = end-diastolic
frequencyshift
1. Fullpulsatilityindexof Gosling(plr) = 1/A02SAi2
2. Simplifiedpulsatilityindex(pt) = (S - D)/m-ean
3. Resistanceindex(Rl) = Pourcelotindex
= (S - D)/S or 1 - (D/S)
4. Stuartindex = fuB ratio= SiD ratio
5. B/A ratio= B(100%)tA
HEART VALVE POSITIONS
PA CXR:
referenceline = obliquelinedrawnfrom distalleft
mainstembronchusto rightcardiophrenic angle
r/ aorticvalveresidesin profilesuperiorto tnis line
overlyingthe thoracicspine

/
' /l \l
rTV I
\ /
L V \ /

Heart Valve Positions

AoV = aorticvalve,_LA= .leftatrium,LY_=left ventricle,MV = mitral valve,pV - pulmonicvalve,
RA = right atrium,RV = right ventricie,TV = tricuspio"iru"
592 Radiology Review Manual

! pulmonicvalvejust inferiorto left mainstembronchus

r/ mitralvalve residesinferiorto this line centrally
locatedwithincardiacsilhouette
r/ tricuspidvalve inferiorto this line more basilarand
m idline
LAT CXR:
referenceline = obliquelinedrawnfrom carina/ right
pulmonaryarteryshadowto anteriorcardiophrenic
sulcus
{ aorticvalveresidessuperiorto this line
{ pulmonicvalve anterior+ superiorto aorticvalve
r/ mitralvalve residesinferoposteriorly to this line
Parasternal Long-Axis View
r/ tricuspidvalve inferiorto this line anteriorly
Ao = aorta PW = posterior wall
LA = left atrium RV = right ventricle
LVD = left ventricular diameter S = septum

Parasternal Long- And Short-Axis

1, 3, 5 = RV dimension 2 =LY dimension at mitral level
6 = aortic root 4 = LV dimension at papillary
7 =LA muscle level

Diasram Showing the Relationship of the Four

Cardiac Valvesin Cross Section

Apical 4-Chamber View aTL, pTL, sTL = anterior, posterior, septal tricuspid valve leaflets
aML, pML = anterior, posterior mitral valve leaflets
1 = LV long axis 4 =LAminor axis 7 = RA major axis = right, left, noncoronary cusps of aorta
rc, lc, nc (Ao)
2 =LY short axis 5 = RV long axis 8 = RA minor axis = right, left, anterior cusps of pulmonary artery
rc, lc, ac (PA)
3 = LAmajor axis 6 = RV short axis
 CardiovascularAnatomyand
Echocardiography 593

c o 1

LAPW

---,--F-.---_:
"'"t-'. :--1'=Ft RV q1;;'

'"S#=#fifr
T

Sweepof Transducerfrom Aorta toward Apex

Area 1: recognizedby parallelmotionof both aorticwalls (a) towardthe transducerduringsystole,
(b) away from the transducerduringdiastole. Left atrialposteriorwall (LAPW)does not
move becauseof mediastinalattachmentby pulmonaryveins.
Aorticvalve cusps (rightcoronary+ noncoronarylleft cusps)are positionedin middleof
aortaduringdiastole,open abruptlyduringsystoleat onset of ventricularejectionin a "box-
like"fashion.
Aortic+ LA dimensionare similarin mostcases.
Area2: Aortic-septalcontinuity= anterioraorticwall becomesinterventricular septum
Aortic-mitralcontinuity= posterioraorticwall becomesanteriormitralvalve leaflet
Mitralvalvewith typical"M" configuration duringdiastole;motionof aML towardtransducer
duringsystolesecondaryto movementof whole mitralvalve apparatus
Area 3: posteriormitralvalve leaflet(pML)= reciprocal"W-shaped"configuration;left ventricular
posteriorwall (LVPW)shows anteriormotionduringsystole.
Area 4: Chordaetendineaein continuitywith mitralvalve leafletsmergewith a thick posteriorband
of echoesrepresenting the posteromedial papillarymuscle(ppm).
594 Radiology Review Manual

aRVW
5 aRVW
\ RVOT RVOT

\ aAoW
\ LVOT
Ao
{
pAoW

LA
- / 1
LAPW
H!-^Ecc
ECG

Echocardiogram of Aortic Root Echocardiogram of Mitral Valve

= aortic root dimension, measuredat mitral valve excursion = opening

end-diastoleat R-wave of ECG 2.14.3 cm amplitude of anterior leaflet of mitral
increased in: aneurysm of aorta, valve (DE amplitude)......... .......2-3cm
aortic insufficiency decreasedin: nonpliable MV stenosis,
= aortic cusp separation: ...... I.7-2.5 cm low cardiac ouq)ut,
decreasedin: aortic stenosis, low compliance of LV
low stroke volume increasedin: MV prolapse,
increasedin: aortic insufficiency high flow through MV
J = left ventricular ejection time E to F slope = early diastolic
4 = left atrial diameter, measured at posterior motion of anterior leaflet ......7-I5 cm/sec
moment of mitral valve opening 2.34.4 cm decreasedin: mitral valve stenosis,
5 = eccentricity index of aortic valve low compliance of LV
cusps = ratio of anterior to posterior septal-mitral valve distance =
dimension(rarelyused) <1.3
.................. E point septalseparation .....2.94.1mm
4+I = ratio of LA-to-aortic root decreasedirz: ostium primum ASD,
dimension ....0.87-1.11 IHSS
aRVW = anterior right ventricular wall increasedin: dilated LV
RVOT = right ventricular outflow tract RV right ventricle
aAoW = anterior aortic wall IVS interventricular septum
Ao = aorta LVPW left ventricular posterior wall
pAoW = posterior aortic wall aML anterior mitral valve leaflet
LA = left atrium PML posterior mitral valve leaflet
LAPW = left atrial posterior wall aRVW anterior right ventricular wall
NCC = nOnCorOnarycUSp RVOT right ventricular outflow tract
RCC = right coronary cusp LVOT left ventricular outflow tract
ECG = electrocardiogram LA left atrium
ECG electrocardiogram
A point of atrial contraction
C closurepoint
DE opening secondaryto passive ventricular filling
CD systole with steady anterior drift of coapted leaflets
(passive movement secondaryto movement of
entire heart toward chest wall)

Mitral Valve in Mid-Diastole

 CardiovascularAnatomyand Echocardiography 595

AORTICARCHBRANCHING
PATTERNS
1. "Standard"branchingpattern(65-75%)
RVAW
brachiocephalic trunk,left CCA, left subclavianartery
RV 2 . Commonoriginof brachiocephalic trunk+ leftCCA
(13%)
V,u, 3 . Bovineaorticarch (9%)
= originof left CCA from brachiocephalic trunk
V I 4 . Vertebralartery(usuallyleft)arisingfrom aorticarch
(3%)
LV
5 . Leftand rightbrachiocephalic trunks(1%)
6 . Aberrantrightsubclavianarteryas the last branchof
the aorticarch (<1%)
LVPW
CervicalAortic Arch
Associatedwith: right aortic arch (in 2/3)
. pulsatileneck mass
. upperainruayobstruction
ECG ., dysphagia
Echocardiogram of Right and Left Ventricle ! mediastinal widening
1 = RV end-diastolic dimension ^/ absenceof normalaorticknob
(RVEDD) at R-wave of ECG 0.7_2.3 cm r/ aorticarch near lung apex
increasedin: RV volume overload r/ trachealdisplacementto oppositeside + anteriorly
2 = septal thickness = end-diastolicIVS { apparentcutoffof trachealair column(secondaryto
thicknessat R-wave of ECG 0.9 + 0.06 cm crossingof descendingaorta to side oppositeof arch)
decreasedin.- CAD DDx: carotidaneurysm
increased in: asymmetric septal
hypertrophy, IHSS AORTICISTHMUS VARIANTS
3 = LV end-diastolic dimension Aorticlsthmus
(LVEDD) ar R-wave of ECG 4.6 + 0.54 cm = fl?rfowingof the aorta in newbornbetweenleft
4 = LVPW thickness, measuredat end-
subclavianarteryand ductusarteriosus
diastole at peak of R-wave of ECG: 0.94 + 0.09 cm Age: up to 2 monthsof age
increased in: LV hypertrophy
- LV end-systolic
Pros
nos
is' cessatio
n
5
(LVESD)
dimension 3ill?:llffiX1"Tff
:f.1[:J:
.2.9 + 0.5 cm + increasedflow throughnarrowedregion
3 and 5 = fractional shortening of
internal diameter A orti c S pi ndl e (16% )
= (EDD - ESD)/EDD x 100 ...0.254.42 = corger]italnarrowingof the aorta at the ligamentum
I V S : L V P Wt h i c k n e s s . . . . . . . . .<. 1 . 9 arteriosumwith distalfusiformdilatation
RVAW = right ventricular anterior wall
RV = right ventricle
= interventricularseptum
DuctusDiverticulum
IVS
= localizedbulge along anteromedialaspectof aortic
LV = left ventricle
= left ventricular posterior wall
isthmus
LVPW
= endocardium
Origin: remnantof enlargedmouthof ductus
endo
epi = epicardium arteriosus/ resultof tractionfrom ligamentum
peri = pericardium arteriosum

Fractional shortening (FS) = [(end-diastolic size - systolic size)I

end-diastolicsizel x 100
- for LV = 2542Vo
- for IVS = 2842%o
- for LVPW = 36-100/0

Aortic Spindle Classical Atypical

Ductus Ductus
Diverticulum Diverticulum
Normal Aortic Arch in 45o LAO Projection
596 RadiologyReviewManual

Frequency: in 33h of infants,in g'/. of adults Left anterior descending (LAD)travelswithin

^/ focal bulgewith smoothuninterruptedmargins anteriorinterventriculargroove,givesbloodsupplyto
r/ gentlyslopingsymmetricshoulders(classicductus anterolateralPaPillarY muscle
diverticulum) Diagonatbranches(D1, D2, etc) arise from LAD
r/ shortersteeperslopesuperiorly+ moregentle and courseover anterolateralwall of LV
slopeinferiorly(atypicalductusdiverticulum) N.B.: Diagonalsfrom LAD
DDx: posttraumaticfalse aneurysm Septatbranches(S) for interventricularseptum
Left circumflex artery (LCX)travelswithinleft
trunk
Prominentbronchial-intercostal atrioventricularsulcus,terminatesat obtusemarginof
heart
ARTERIES Obtuse marginalbranches(OM1,OMz, etc) for
CORONARY
lateralwall of LV
Anatomyof GoronaryArteries Left atriatcircumflexartery (LACX)for atrium
R|GHTCORONARYARTERY(RCA)
sulcus,roundsthe
travelswithinrightatrioventricular sulcus
junctionof posterioratrioventricular
Crux =
acute marginof heart groove
+ posteriorinterventricular
Conus artery (CB) = 1st branchfrom RCA (in 50%
directlyfrom aorta)to supplyRVOT
Sinoatrial node artery (SANA)= 2nd branchfrom
CoronaryAnery TerritorY
= LAD
septum
RCA (in >50%)
anteriorwall = LAD
Acute / RV marginal branches (M1, M2, etc) have
lateralwall = LCX
an anteriorcourse
posteriorwall = RCA
Posterior descending artery (PDA)originatesfrom
inferior/ diaphragmaticwall = RCA
RCA near crux or from a distalacute marginal
apex + inferolateralwall = watershedareas
branch,suppliesposteriorthirdof ventricular
septum+ diaphragmaticsegmentof LV, gives
bloodsupplyto posteromedial papillarymuscle
node artery (AVNA) small branchto
CoronaryArterYDominance
Atrioventricular
determinedby the originof the posteriordescending
AV node
artery(PD),whichsuppliesthe inferiorportionof LV:
Posterolateralsegment arteries (PLSA)supply - from RCA in 85%
wall of LV
posterolateral - from LCX in 10%
- RCA + LCA = codominancelbalancedsupply(5%)
LEFT CORONARYARTERY(LCA)
Left main coronary artery (LM) 0.5-2.0 cm short
stem beforebi- / trifurcation
CoronaryArteriography
agents:
Contrast
1. Monomericioniccontrastmaterial:
(a) negativeinotropic= depressionof myocardial
of sodium
due to hyperosmolality
contractility
+ decreasein total calcium
(b) peripheralvasodilatation
2. Megluminediatrizoate(containssmallquantitiesof
sodiumcitrate+ EDTA)
3. Nonioniccontrastmaterial= slightincreasein LV
contractility
Dose: 3-10 mL
Mortality: 0.05%
Risk factors associated with death:
1. Multipleventricularprematurecontractions
2. Congestiveheartfailure
3. SystemichYPertension
4. Severetriple-vesselcoronaryarterydisease
(highestrisk)
5. LV ejectionfraction<30/o
6. Left main coronaryarterystenosis
Clues for proiection:
45-70" LAO:
r/ ribs slantingto left side of image
AP View of Heart and Coronary Arteries r/ catheterin descendingaortaon rightside of
(abbreviations in text) image
7
CardiovascularAnatomy and Echocardiography 597

I
-I LM

I
I I
, I
,
r M l T
t-ec
\
r $ wpt oMl
t d w ,
\ t r # *
\ I , T I \
, i6
I , I
, I I I
, I
I / I oM3 /
I ,
l r I
I d t
a
t ." I
tb

Right CoronaryArtery in RAO View Left Coronary Artery in RAO View

D'
I
oM1

L,\D

i _ _

Right CoronaryArtery in LAO View Left Coronary Artery in Caudocranial LAO

Right and Left Coronary Artery Angiograms

AVNA = atrioventricular node artery LCX = left circumflex artery
CB = conus branch artery Ml = lst acute marginal branch artery
Dl = 1st diagonal artery M2 = 2nd acute marginal branch artery
D2 = Znddiagonal artery OMl = 1st obtuse marginal artery
LACX = left atrial circumflex arterv OM2 = 2nd obtuse marginal artery
LM = left main coronary arterv PDA - posterior descending artery
LAD = left anterior descending'artery PLB - posterolateral branch ntery
598 Radiology Review Manual

15-30" RAO:
r/ ribsslantingto rightside of image
{ catheterin descendingaortaon left side of image
Technique:
0 20-30" of cranial/ caudalangulationvariablyused
Catheterin left coronaryorifice:
(a) LAO + caudocranial angulation:
proximal1/3 of LAD + originof firstdiagonal
branch
"spiderview":
(b) LAO + craniocaudalangulation=
LCA, proximal LCX, firstmarginal/ diagonal
branches
30o LAO view RAO view
(c) RAO + craniocaudal angulation:
Right Dominant System proximalthirdof LCX + originof its branches
(d) RAO + caudocranial angulation:
separationof LAD from diagonalbranches
Catheterin right coronary artery orifice: LAO t RAO
False-negative interPretation:
(1) eccentri cl esi oni n75' /"
(2) foreshorteningof vessel
(3) overlapof othervesselsremediedby angulated
projections:improveddiagnosis(50%),upgrade
to moresignificant stenosis(30%),lesion
unmasked(20%)

LAO view RAO view CoronaryArtery Collaterals

Left Dominant System A. INTRACORONARY COLLATERALS
= fillingof a distalportionof an occludedvesselfrom
the proximalPortion
{ tortuouscourseoutsidethe normalpath
ANA B. INTERCORONARY COLLATERALS
= between differentcoronaryarteries/ between
branchesof the same arterY
Location: on epicardialsurface,in atrial/
ventricularseptum,in mYocardium
1. ProximalRCAto distalRCA
(a) by way of acute marginalbranches
(b) from sinoatrialnode artery(SANA)to
node artery(AVNA)= Kugel
atrioventricular
collateral
LAO view RAO view 2. RCA to LAD
(a) betweenPDA and LAD throughventricular
Balanced Circulation
septum/ aroundapex
Coronary Artery Angiograms of Varying Dominance (b) conus artery(1st branchof RCA)to proximal
part of LAD
AVNA = atrioventricular node artery (c) acute marginalsof RCA to rightventricular
CB = conus branch artery
Dl = lst diagonal artery
branchesof LAD
DZ = 2nd diagonal artery 3 . DistalRCA to distalLcx
LACX = left atrial circumflex artery (a) posterolateralsegmentarteryof RCA to distal
LM = left main coronary afiery LCX (in AV groove)
LAD = left anterior descending artery (b) AVNA of RCA to LCX (throughatrialwall)
LCX = left circumflex artery
= (c) posterolateralbranchof RCA to obtuse
Ml lst acute marginal branch artery
MZ = 2nd acute marginal branch artery marginalbranchesof LCX (over left
OM1 = lst obtuse marginal artery ventricularwall)
posterolateral
OM2 = 2nd obtuse marginal artery 4 . ProximalLAD to distalLAD
PDA = posterior descendingartery (a) proximaldiagonaltodistaldiagonalarteryof
PLB - posterolateral branch artery
= posterolateral segment artery LAD
PLSA
S = septal branches (b) proximaldiagonalto LAD directly
SANA = sinoatrial node artery 5. LAD to obtusemarginalof LCX
7
Cardiovascular
Anatomyand Echocardiography 599

right superior anterior transversestnus

aortic recess superior
aortic recess

left
pulmonic lefr
recess pulmonic
TCCCSS
posterior right PA
superior
aortic
TECESS
right
pulmonic
right TECCSS
pulmonic
oblique
TCCESS
sinus

At Level of Pulmonarv
" Arterv Below Level of Right Pulmonary Artery
;.ricardial Sinuses & Recesses
asc Ao = ascending aorta main PA = main pulmonary artery =
descAo = descending aorta righ_tpA = right fuhonary artery ^Llly- l9f! superiorpulmonaryvein RAA- rightatrialappendage
RSPV= right superiorpulmonaryvein LAA = left atrial appenda[e
eso = esophagus SVC = superlorvena cava

PERICARDIUM C. Obliquesinus(89%.)
A . F I B RO USP ER IC A R D IU M ./ benindleft atrium+ anteriorto esophagus
= outerfibrouslayer { separatedfromtransversesinusby double
B . S E R O U SP E R I C A R D I U M reflectionof pericardium(and fat) betweenright
= innerseroussac formingthe pericardial cavity + leftsuperiorpulmonicveins
r/ contains20-25 mL of serousfluid 1. Posteriorpericardial recess(67%.)
(a) innerviscerallayer= epicardium / Oehinddistalrightpulmonaryartery+ medialto
. intimatelyconnectedto heart+ epicardialfat bronchusintermedius
(b) outer parietallayer DDx: lymphnodes,esophageal/ thymicprocess,
. linesfibrouspericardium vascularabnormality,pericardialcyst / tumor
* = p€fc€otages givedepiction
on HRCT
PericardialSinusesand Recesses EMBRYOGENESIS OF VENA CAVA
= extensionsof pericardialcavity
Time of development: 6-Bth week of embryoniclife
A. Recessesof pericardialcavityproper Origin:
1. Postcavalrecess(29%.) A. Vitelline(omphalomesenteric) venoussystem:
r/ benindand right lateralto SVC bloodfrom yolk sac to sinusvenosus
2. Rightpulmonicvein recess(2g%.) B. Umbilicalvenoussystem
r/ benindand right lateralto SVC bloodfrom chorionicvillito sinusvenosusvia ductus
3. Leftpulmonicvein recess(60%.) venosus
.i beninOand right lateralto SVC
C. Intraembryonic cardinalvenoussystem
B. Transversesinus continuousappearance+ regressionof 3 paired
r/ posteriorto ascendingaorta and pulmonarytrunk embryonicveins
+ above left atrium(95%.) (1) C ardi nalvei ns
1. Superioraorticrecess join to form commoncardinalvein,which enters
r/ alongascendingaorta;may be dividedinto left+ rightsinushorns
anterior,posterior,right lateralportion (a) anteriorcardinalveins
DDx: aorticdissectionon NECT drainthe cranialregion
2. Leftpulmonicrecess (b) posteriorcardinalveins
r/ below left pulmonaryartery+ posterolateral to drainbody of embryo+ mesonephros
proximalrightpulmonaryartery + anteriorextremities
3. Rightpulmonicrecess Location:dorsolateral partof urogenitalfold
ri below right pulm onary arlery+ above left atrium (2) Subcardinal veins
4. Inferioraorticrecess drainurogenitalsystemof metanephros
^/ betweenascendingaorta+ inferiorSVC / right
+ suprarenalglands
atrium Location:ventromedial to posteriorcardinalveins
r/ extendingdown to levelof aorticvalve
+ ventrolateralto aorta
600 Radiology Review Manual

intemal
jugular v
subclavian v.
left superior
brachio- intercostal v.
vitelline v. cephalic v.
supenor
accessory
subcardinal- left hom of hemiazygosv.
sinus venosus vena cava
hepatic
azygosv.

hepatic vv.

ductusvenosus hemiazygos v.
umbilical v.
right renal v.
intersubcardinal
supracardinal- anastomosis
subcardinal- '
left renal v.

kidney
sacrocardinal-
subcardinal
anastomosis
gonadal vv.

iliac vv.

DeveloDmentof the Maior VenousSvstem

vitelline vein + hepaticsegmentof IVCI right subcardinai-hepaticanastorirosis+ suprarenalsegmelt of M
right supracardinat-subcardinalanastomosis+ renal se€mentof M; right abdominalsupracardinalvein = infrarenal segmentof M
thoracrcsupracafcuralvems + azygosanonemuzygosvens

- Intersubcardinal anastomosesform anteriorto 2. Suprarenalsegment

aorta belowsuperiormesentericarteryand
connectleft+ rightsubcardinal veins
(3) Supracardinal veins
drain body wall via intercostalveins
Location:dorsomedialto posteriorcardinalvein
+ dorsolateralto aorta
- CR AN IAL
(a) azygosvein on the right
drains4-11 rightintercostal veins
(b) portionof superiorintercostalvein
drains2-3 left intercostalveins
(c) accessoryhemiazygos
drainsf7 Eft intercostalveins
(d) hemiazygosvein
drainsleft8-11 intercostal veins
- CAUDAL: lumbarveins
InferiorVenaCava
1. Hepatic= posthepatic
segment
Origin: terminalpart of rightvitellinevein
Origin: subcardinal-hepaticanastomosis
3. Renalsegment
Origin: part of rightsubcardinalvein
+ supracardinal-subcardinalanastomoses
4. Infrarenalsegment
Origin: rightsupracardinal/ sacrocardinalvein
VENOUSSYSTEMOF LOWEREXTREMITY
DeepVeinsof LowerExtremity
3 pairedstem veins of the calf accompanythe arteries
as venae commitantes+ anastomosefreelywith each
other:
1. Anterior tibial veins
drainingbloodfrom dorsumof foot,runningwithin
extensorcompartmentof lower leg closeto
interosseous membrane
2. Posterior tibial veins
formedby confluenceof superficial+ deep plantar
veinsbehindanklejoint
 Cardiovascular
Anatomyand Echocardiography 601

3. Peronealveins
directlybehind+ medialto fibula common femoral v.
4. Calfveins
(a) Soleal muscle veins
baggyvalvelessveinsin soleusmuscle deep femoral v.
(= sinusoidalveins); drainingintoposteriortibial
+ peronealveinsor lowerpart of poplitealvein femoral v.
(b) Gastrocnemius veins
thin straightveins with valves;draininginto lower
+ upper partsof poplitealvein popliteal v.
5. Popliteal vein
formedby stem veins of lower leg gastrocnemiusvv.
6. Femoral / superficial femoral vein
anterior tibial vv.
continuation of poplitealvein;receivesdeep femoral
vein about 9 cm below inguinalligament
7. Deep femoral vein peronealvv.
drainingtogetherwith superficial femoralvein into
commonfemoralvein; may connectto poplitealvein
(38%)
8. Common femoral vein
formedby confluenceof deep + superficialfemoral
vein; becomesexternaliliacvein as it passes
beneathinguinalligament Deep Venous System of Lower Extremity

SuperficialVeinsof LowerExtremity (b) Anterior supefficial tibial vein

1. Greatersaphenous vein (c) Posteromedialsuperficial thigh vein
formedby unionof veinsfrom medialsideof sole of often connectswith upper part of lesser
foot with medialdorsalveins;ascendsin front of saphenousvein
medialmalleolus;passesbehindmedialcondylesof (d) Anterolateral superficial thigh vein
tibia+ femur (e) Tributariesin fossa ovalis
(a) Posteriorarch vein - superficialinferiorepigastricvein
connectedto deep venoussystemby - superficial externalpudendalvein
communicatingveins - superficial circumflexiliacvein

greatersaphenousv.

anterolateral
superficial v. posteromedial
of thigh superficial v. of thigh

Dodd group
gastrocnemiuspoint
Boyd vein

anterior superficial soleuspoint

lateral communicatins v.
tibial v.
posterior lateral
posterior arch v. vein on calf

12 cm
lessersaphenousv.
greater saphenousv. 213 of leg communicating v.
5cm
Cockett group

submalleolar

IT communicating v.

SuperfrcialVenousSystemof Lower Extremity

602 Radiology Review Manual

2. Lesser saphenous vein B. LATERAL

originatesat outer borderof foot behindlateral
malleolusas continuationof dorsalvenousarch;
enterspoplitealvein betweenheadsof gastrocnemius
in poplitealfossawithin8 cm of kneejoint(60%)or
joins with greatersaphenousvein via posteromedial
/ anterolateralsuperficialthigh veins (20%)
Communicating= PertoratingVeins
> 100v einsin e a c hl e g
A . M E DI A L
1. Submalleolar communicatingvein
2. Cockett group
groupof 3 veinslocated7, 12,18 cm abovethe
tip of medialmalleolusconnectingposteriorarch
veinwith posteriortibialvein
3. Boyd vein
located10 cm belowkneeiointconnectingmain CONTENTS
trunkof greatersaphenousveinto posteriortibial
veins
4. Dodd group
groupof 1 or 2 veinspassingthroughHunter
canal(= subsartorial canal)to join greater
saphenousvein with superficial femoralvein
1. Lateralcommunicatingvein
locatedfrom just above lateralmalleolusto
junctionof lower-to-midthirdsof calf connecting
lessersaphenousvein with peronealveins
2. Posterior mid-calf communicating veins
locatedposteriorly5 + 12 cm above os calcis
joininglessersaphenousvein to peronealveins
3. Soleal + gastrocnemius Points
joiningshortsaphenousvein to soleal/
gastrocnemius veins
TRIANGLE
OF FEMORAL
mnemonic; "NAVEL"(from lateralto medial)
Nerve
Artery
Vein
Emptyspace
Lymphatics

common iliac a.
common iliac a. common iliac a.
intemal iliac a.
internal iliac a. internal iliac a.
extemal iliac a. external iliac a. extemal iliac a.
mon femoral a. common femoral a.
common femoral a.
femoral a. femoral a. deep femoral a.

superficial femoral a. superficial femoral a.

superficial femoral a.

Pelvic Arterial Anatomy (rightside)

 Cardiovascular
Disorders 603

CARDIOVASCULAR
DISORDERS

ABERRANT
LEFTPULMONARY
ARTERY Hemodynamics:
= PULMONARY ARTERY SLING with postnatalfall in pulmonaryarterialpressure
Embryology: failure of development/ obliterationof left perfusionof LCA drops (ischemicleft coronarybed),
6th aorticarch (= vascularpediclefor left collateralcirculationfrom RCA with flow reversalin LCA
lung);left lung parenchymamaintainsa - adequatecollateralcirculation= lifesaving
connectionwith rightlung leadingto - inadequatecollateralcirculation= myocardial
developmentof a collateralbranchof the infarction
rightpulmonaryarteryto supplythe left lung - largecollateralcirculation = L{o-R shuntwith volume
Site: left PA passesabove right mainstembronchus overloadof heart
+ betweentracheaand esophaguson its way to left . episodesof sweating,ashencolor(angina
lung symptomatology)
Age at presentation: neonate/ infant/ child . ECG: anterolateralinfarction
Associated with: . continuousmurmur(if collateralslarge)
(1) "napkin-ringtracheo"= absentpars membranacea ^/ dilatationof LV
(50%) r/ enlargementof LA
(2) PDA (mostcommon),ASD, persistentleft SVC r/ normalpulmonaryvascularity / redistribution
. stridor(mostcommon),wheezing,apneicspells, Rx:
cyanosis (1) Ligationof LCA at its originfrom pulmonarytrunk
. respiratoryinfection (2) Ligationof LCA + graft of feft subclavianartery to
. feedingproblems LCA
{ deviationof tracheato left (3) Creationof an AP window+ bafflefrom Ap window
r/ "inverted-T"appearanceof mainstembronchi to ostiumof LCA
= horizontalcoursesecondaryto loweroriginof right DDx: endocardialfibroelastosis, viral cardiomyopathy
mainstembronchus (NO shocklikesymptoms)
{ anteriorbowingof rightmainstembronchus
{ "carrot-shaped trachea"= florfowihgof tracheal ANOMALOUS PULMONARY VENOUSRETURN
diameterin caudaddirectionresultingin functional
TotalAnomalousPulmonaryVenousReturn
trachealstenosis
= TAPVR = admixturelesionbecauseof the
r/ obstructiveemphysema/ atelectasisof RUL + LUL
combinationof cyanosis+ increasedpulmonary
d low left hilum
./ separationof trachea+ esophagusat hilum by soft- vascularity(L-to-Rand R-to-Lshunt)
Embryology; anomalousconnectionbetween
tissuemass
pulmonaryveins and systemicveins
{ anteriorindentationon esophagram
secondaryto embryologicfailureof the
commonpulmonaryvein to join the
posteriorwall of the left atrium
Prevalence: 2/" of CHD
AMYLOIDOSIS Age: symptomaticin 1st year of life
= extracellulardepositsof insolublefibrillarprotein . cyanosis
. asymptomatic/ CHF (restrictivecardiomyopathy), Hemodynamics:
arrhythmia obstructionalongthe pulmonaryvenouspathway
CX R: R A e RV <+ Main PA <+
./ normal/ generalizedcardiomegaly Pulmvessels t
{ pulmonarycongestion L A e L V < + A o < - +
{ pulmonarydepositsof amyloid Associated with:
NUC: ASD / patentforamenovale (necessaryfor survival),
{ strikinguptakeof Tc-ggmpyrophosphategreaterthan bronchopulmonary sequestration, pulmonary
bone (50-90%) arteriovenousmalformation,cysticadenomatoid
EC HO : malformation
^/ granularsparklingappearanceof myocardium Overallprognosis: 75/o mortalityrate within 1 year of
{ LV wall thickening birth if untreated
r/ decreasedLV systolic+ diastolicfunction
Sup radiaphragmatic TAPVR
Type | = SUPRACARDIACTAPVR (Sz%)
ANOMALOUS LEFT CORONARY ARTERY = drainageinto leftbrachiocephalic vein/
= left coronaryarteryarisesfrom pulmonarytrunk (left right+ left persistentSVC / azygosvein;
sinusof Valsalva) <10/o obstructed
604 Radiology Review Manual

Type ll = 64g9lAC TAPVR(s0%) May be associated with:

= drainageintocoronarysinus(80%)/ RA (1) Atrialseptaldefect (25%)
Hemodynamics: (a) RUL pulmonaryvein entersSVC I RA (213)
- functionalL-to-Rshuntfrom pulmonaryveinsto frequentlyassociatedwith: sinusvenosustype
rightatrium ASD (e0%)
- increasedpulmonarybloodflow r/ nUl vein coursesin a horizontaldirection
(= overcirculation) (b) LUL pulmonaryvein entersbrachiocephalic
- ASD restoresoxygenatedbloodto left side vei n (1/3)
- normalsystemicvenouspressurewith frequentlyassociateduzrfh;ostium secundum
increasedflow throughwidenedSVC typeA S D (10-15% )
- after birth CHF secondaryto { verticalmediastinaldensitylateralto aortic
(a) mixtureof systemic+ pulmonaryvenous knob extendingupwardand mediallywith
bloodin RA smoothcurvilinearborder(DDx: persistent
(b) volumeoverloadof RV left SVC)
. cyanosis (2) Hypogenetic lungas a componentof congenital
. neckveinsundistended (shuntleveldistally) pulmonaryvenolobarsyndrome
. R ventricularheave (= increasedcontactof = SCIMITAR SYNDROME
enlargedRV with sternum) = part/ all of the hypogenetic lung is drainedby
. systolicejectionmurmur(largeshuntvolume) an anomalousvein
{ overallheartsize notablynormal: Anomalousvein drainsinto:
r/ slightlyenlargedRV (= volumeoverloadwithtime) - IVC belowrighthemidiaphragm (33%)
r/ normal/ enlargedRA - suprahepaticportionof IVC (22%)
{ normalLA (= ASD acts as escapevalve) - hepaticveins
r/ ditatedSVC + left verticalvein. - portalvei n(11% )
r/ "figureof 8" l"snowman"configuration of cardiac - azygosvein
silhouette(= dilatedSVC + left verticalvein) - coronarysinus
r/ pretrachealdensityon lateralfilm (= left vertical - rightatrium(22%)
vein) - leftatrium= "meandering pulmonaryvein"
^/ increasedpulmonarybloodflow (= overcirculation) 0 Drainageintosuprahepatic portionof IVC /
! absentconnectionof pulmonaryveinsto LA rightatriummay be a cluefor interruption
of intrahepaticportionof IVC!
May be associatedwith: systemicarterialization
Sub- / lnfradiaphragmatic TAPVR (12%) of the lungwithout
= t Y P elll sequestration
= drainageintoportalvein/ IVC / ductusvenosus/ Location: almostexclusively on rightside
left gastricvein with constrictionof descending ^i tubularstructureparalleling the rightheart
pulmonaryvein by diaphragmen routethrough borderin the configurationof a Turkishsword
esophagealhiatusleadingto pulmonaryvenous = "scimitar"(PA view)
hypertension+ RV pressureoverload;>90% . acyanotic
obstructed . ASD symptomatology
. intensecyanosis+ respiratorydistress(R-to-L r/ radiographic findingssimilarto ASD
shuntthroughASD) r/ anomalouscourseof drainingvein
Prognosis: death within a few days of life r/ enlargement of drainingsite:SVC, lVC, azygosvein
Associatedwith: aspleniasyndrome(80%), CECT:
polysplenia r/ nodular/ tubularopacity(= ?flomalousvein),which
r/ uniqueappearanceof pulmonaryedema opacifiesin phasewith pulmonaryvein
+ pulmonaryvenouscongestionwith normal-sized
, heart(DDx:hyalinemembranedisease)
tr low anteriorindentationon barium-filledesophagus
AORTICANEURYSM
Mixed Type ot TAPVR (6%) Cause:
= tYPelV 1. Atherosclerosis(73-80-90%): descendingaorta
= with variousconnectionsto R side of heart (6%) 2. Traumatic(15-20%): followingtransection;
descendingaorta
3. Congenital(2%)r aorticsinus,postcoarctation,
PartialAnomalousPulmonaryVenousReturn ductusdiverticulum
= PAPVR 4. Syphilis(19%): ascendingaorta+ arch
May occur in isolation 5. Mycotic= bacterialdissection;anywhere
N.B.: venousreturnalmostneverobstructed! 6. Cysticmedianecrosis(Marfan/ Ehlers-Danlos
Prevalence; 0.3-0.5% of patientswith CHD syndrome,annuloaortic ectasia):ascendingaorta

7. lnflammation of media+ adventitia:
Takayasuarteritis,giant cell arteritis,relapsing
polychondritis,rheumaticfever,rheumatoidarthritis,
ankylosingspondylitis, Reitersyndrome,psoriasis,
ulcerativecolitis,systemic lupuserythematosus,
scleroderma, Behgetdisease,radiation
8. Increasedpressure:
systemichypertension,aorticvalve stenosis
9. Abnormalvolumeload: severeaorticregurgitation
T RUEA NE URY S M
= permanentdilatationof all layersof weakenedbut
intactwall
FALSEANEURYSM
= focal perforationwith all layersof wall disrupted;
escapedbloodcontainedby adventitiai perivascular
connectivetissue+ organizedblood
FUSTFORM ANEURYSM(80%)
= circumferentialinvolvement
SACCULARANEURYSM
= involvementof portionof wall
AbdominalAorticAneurysm(AAA)
0 Thereis no consensusregardingthe definitionof an
atherosclerotic AAA!
= focalwidening>3 cm (ultrasound literature);
twicethe
size of normalaortaI >4 cm [Bergan, AnnSurg19g4]
Normalsize of abdominalaorta >50 years of age:
12- 19 m m in w o m e n ;1 4 -2 1m m i n m e n
Prevalence: 1.+8.2o/oin unselectedpopulation;in 6%
>80 years of age; in 6-20/" of patients
with signsof atherosclerotic disease;
M > F; Whites:Blacks = 3:1
Cause: ? genetic(10{old increasein riskas first-
degreerelativeof patientwith AAA); structural
defectof aorticwall causedby increased
proteolysis;copperdeficiency
Riskfactors: male sex, age >75 years,white race,
priorvasculardisease,hypertension,
cigarettesmoking,familyhistory,
hypercholesterolemia
Age: >60 years; M:F = 5-g:1
Associated with:
(a) visceral+ renal arteryaneurysm(2%)
(b) isolatediliac+ femoralarteryaneurysm(16%):
commoniliac(89%),internaliliac(10%),external
iliac( 1% )
(c) stenosis/ occlusionof celiactrunki SMA (22%)
(d) stenosisof renal artery(22-30%)
(e) occlusionof inferiormesentericartery(gO%)
(f) occlusionof lumbararteries(78%)
Growth rate of aneurysm of 3-6 cm in diameter:
0. 39c m ly e a r
. asymptomatic(30%)
. abdominalmass (26%)
. abdominalpain (37%)
0 lmagingshouldprovideinformation about
(a) the proximalextentof the aneurysm,which
determinesthe site of clampingof the aorta(origin
of renalarteries)
CardiovascularDisorders O0S
(b) the courseof the left renalvein (retroaortic?)!
Location: infrarenal(91-95%)with extensioninto iliac
arteries(66-70%)
Plainfilm: ! muralcalcification (75-86%)
US: ^/ >gg% accuracy in size measurement
NCCT:
r/ perianeurysmalfibrosis(10%),may cause ureteral
obstruction
{ "crescentsign"= peripheralhigh-attenuating
crescentin aneurysmwall (= acuteintramural
hematoma)= sign of impending rupture
CECT:
(a) rupturedaneurysm
./ anteriordisplacementof kidney
r/ extravasationof contrastmaterial
^/ ttuiOcollection/ hematomawithinposterior
pararenal+ perirenalspaces
r/ free intraperitonealfluid
r/ perirenal"cobwebs"
(b) containedleak
r/ laminatedmuralcalcification
r/ periaorticmass of mixed/ soft-tissuedensity
r/ lateral"draping"of aneurysmaroundvertebral
, body
r/ focaldiscontinuityof calcifications(unreliable)
^/ indistinctaorticwall (unreliable)
Angio(AP + LAT filming):
r/ focallywidenedaorticlumen>3 cm
r/ apparentnormalsize of lumensecondaryto mural
thrombus(11% )
r/ muralclot (807")
r/ slow antegradeflow of contrastmedium
Containedrupture = extraluminal hematomai cavity
ri absentparenchymalstain = dV?scUl€lr halo
r/ displacement+ stretchingof aorticbranches
Cx:
(1) Rupture(25%)
(a) into retroperitoneum:commonlyon left
(b) intoGl tract: massiveGl hemorrhage
(c) into IVC: rapidcardiacdecompensation
lncidence.' aneurysm<4 cm in 10"/",+S cm in
23/",5-7 cm in 25/o,7-10 cm in
46o/",>10 cm in 60/"
. suddensevereabdominalpain+ radiatinginto
back
. faintness,syncope,hypotension
Prognosis: 64-94h die before reachinghospital
lncreasedrisk: size >6 cm, growth >5 mm /
6 months,pain+ tenderness
0 The exact momentof ruptureis unpredictable!
0 Causeof deathin 1.3/" of men >65 years!
(2) Peripheralembolization
(3) Infection
(4) Spontaneous occlusionof aorta
Prognosis: 17o/"S-yearsurvivalwithoutsurgery,
50-60% S-yearsurvivalwith surgery
Rx: surgeryrecommendedif >5 cm in diameter;
+-5% surgicalmortalityfor nonruptured,30-80%
for rupturedaneurysm
606 Radiology Review Manual
Postoperative Cx:
(1) Leftcolonicischemia(1.6%)with 10% mortality
(2) Renalfailure(14%)
(3) 0-8% mortalityrate for electivesurgery
AtheroscleroticAneurysm
lncidence; leadingcause of thoracicaorticaneurysm
Histo: diseasedintimawith secondarydegeneration
+ fibrousreplacementof media;ultimatelywall
of aneurysmcomposedof acellular+ avascular
connectivetissue
Pathophysiology:
progressiveweakeningof media resultsin vessel
dilatation+ increasedtensionof vesselwall (law of
Laplace= tensilestressvarieswith productof blood
pressureand radiusof vessel);compromiseof mural
vascularnutrition(vasavasorum)causesfurther
degeneration+ progressivedilatation
A g e : e l d e r l y ;M > F
Location: distalabdominalaorta> iliaca. > popliteala.
> commonfemorala. > aortic+ descending
thoracicaorta > carotida.
Site: (1) infrarenalaorta (associatedwith thoracic
aneurysmin 29%)
(2) descendingthoracicaortadistalto left
subclavianartery
(3) thoracoabdominal
{ fusiform(80%),saccular(20%)
Cx: rupture(causeof death in 50%): usually
unrestrained+ fatal in thoraciclocation
Degenerative
Aneurysm
= medialdegeneration
Most commoncause of aneurysmin ascendingaorta
Cause: (1) geneticallytransmittedmetabolicdisorder:
Marfansyndrome,Ehlers-Danlos syndrome
(2) acquired: resultof repetitiveaorticinjury
+ repairassociatedwith aging
InflammatoryAortic Aneurysm
= definedas triad of
(1) thickenedwall of aneurysm
(2) extensiveperianeurysmal+ retroperitonealfibrosis
(3) dense adhesionsof adjacentabdominalorgans
Frequency; 3-10/" of all AAAs; M:F = 6:1 to 30:1
Mean age: 62-68 years
. abdominal/ back pain
. weight loss + anorexia(20-41%)
. elevatedESR (40-88%)
. tenderpulsatileabdominalmass (15-30%)
Comorbidities: arterial hypertension(34-69o/"),arlerial
occlusivedisease(1047"/"), diabetes
mellitus(3-13%),coronaryartery
disease(33-55%)
Size: usuallysmallat presentationbecauseof early
symptomatology
CT:
r/ rind of homogeneoussoft-tissuedensity
surroundingaorta anteriorly+ laterally
./ contrastenhancement(DDxfrom hematoma)
\i entrapmentof ureters(10-21%)
US:
{ sonolucenthalo aroundaorta
Cx: enlargement+ rupture(lowerrate than in
noninflammatoryaneurysm)
MycoticAneurysm
lncidence: 2.6'h of all abdominalaneurysms
A. PRIMARYMYCOTICANEURYSM(rare)
unassociatedwith any demonstrableintravascular
inflammatoryprocess
B. SECONDARYMYCOTICANEURYSM
= on€Ufysmdue to nonsyphilitic infection
Predisposing factors:
(1) lV drug abuse,(2) bacterialendocarditis (12%),
(3) immunocompromise (malignancy, alcoholism,
steroids,chemotherapy, autoimmunedisease,
diabetes),(4) atherosclerosis,(5) aortictrauma
causedby accidents/ aorticvalve surgery/
coronaryarterybypasssurgery/ arterial
catheterization
Mechanism:
(a) septicemiawith abscessformationvia vasa
vasorum
(b) septicemiawith abscessformationvia vessel
l umen
(c) directextensionof contiguousinfection
(d) preexistingintimalaceration(trauma,
atherosclerosis,coarctation)
Organism: S. aureus(53%),Salmonella(33-50%),
nonhemolyticStreptococcus,
Pneumococcus, Gonococcus,
Mycobacterium(contiguousspreadfrom
spine/ lymphnodes)
Histo: loss of intima+ destructionof internalelastic
lamella;varyingdegreesof destructionof
muscularisof media + adventitia
. frequentlyinsidious,fever
. positiveblood culturein 50%
Site: ascendingaorta > abdominalvisceralartery>
intracranialartery> lower/ upperextremityartery
./ true aneurysm(majority)
{ saccularstructurearisingeccentricallyfrom aorticwall
with rapidenlargement
{ interruptedring of aorticwall calcification
{ periaorticgas collection
{ adjacentvertebralosteomyelitis
{ adjacentreactivelymph node enlargement
Cx: (1) Life-threatening rupture+ hemorrhage(75%)
(2) Uncontrolledsepsisif untreated
Prognosis: 67o/"overallmortality
SyphiliticAneurysm
Spectrum:
1. Uncomplicated syphiliticaortitis
2. Syphiliticaorticaneurysm(mostlysaccular)
3. Syphiliticaorticvasculitis(aorticregurgitation)
 CardiovascuIar Disorders 607

lncidence: 12/" of patientswith untreatedsyphilis TraumaticAortic Pseudoaneurysm

Onset: 10-30 years after initialspirocheteinfection = CHRONIC AORTIC PSEUDOANEURYSM
Histo: chronicinflammationof aorticadventitia+ media 0 2nd most commonform of thoracicaorticaneurysm
beginningat vasa vasorum+ leadingto 0 Mostcommontype occurringin youngpatients
obstructionof vasa vasorumfollowedby lncidence: 2.5/" of patientswho surviveinitialtrauma
nutritional
impairmentof media+ lossof elastic of acute aortictransection
fibers+ smoothmusclefibers r/ usuallycalcified
. positivevenerealdiseaseresearchlaboratory(VDRL)
r/ may containthrombus
test
. positivemicrohemaggfutination Cx: (1) progressive enlargement
assay - Treponema (2) rupture(evenyears after insult)
pallidum(MHA-TP)test
Location: ascendingaorta (36%),aortic arch(34/o),
proximaldescendingaorta (25%),distal
Complicationsof Endovascular
Stent-GraftRepair
1. Endoleak(2-45%)
descendingaorta(S%),aorticsinuses(<1%) = leakageinto the aneurysmoutsidestent-graft
r/ asymmetricenlargementof aortlcsinuses(DDxio
Type 1 = incompletefixationof stent-graftto aortic
, medialdegeneration with symmetricenlargement)
wall at the proximali distalattachmentsite
r/ saccular(75%)/ fusiform(25%)aneurysm
Type 2 = retfogradeflow via parentartery
r/ pencil-thindystrophicaorticwail calcifitation(up to
(eg, lumbar/ inferiormesentericartery)
40%) most severein ascendingaorta,frequently
Type 3 = erndograftdefectwith disruptionof either
obscuredby thick coarseirregularcalcificationsof
metallicsupport/ tabric
secondaryatherosclerosis
Prognosis; enlargementof leak,aneurysmrupture
Prognosis; death in2/o, rupturein up to 4}%;death
2. Graftkinking
withinmonthsof onset of symptomsif
Cause: diminishing diameterof aneurysmafter
untreated
stent-graftimplantationalso decreases
lengthof aneurysm
Associatedwith: distalmigrationof stent-graft
ThoracicAorticAneurysm 3. Graftinfection
Mostcommonvascularcauseof mediastinal massl r/ intervaldevelopmentof perigraftsofttissue
0 10%of mediastinal massesare of vascularoriginl attenuationI air
Averagediameterof thoracicaorta (<4-S cm wid-e):
- aorticroot: Rx: antibiotics+ total excisionof infectedgraft
3.6 cm 4. Graftthrombosis(3-19%)
- ascendingaorta1 cm proximalto arch: 3.5
cm = intraluminal circular/ semicircularthrombus
- proximaldescendingaorta: 2 . 6c m
- middledescendingaorta: Prognosis.'spontaneousshrinkage,developmentof
2.5 cm completethrombosis
- distaldescendingaorta: 2.4 cm 5. Graftocclusion
Associatedwith: hypertension,coronary arlery 6. Showerembolism(4^17%)
disease,abdominalaneurysm Cause: muralthrombusdispersedby delivery
Mean age: 65 years; M:F = 3:1
. substernal/ backi shoulderpain (26%) system
. SVC syndrome(venouscompression) Prognosis; perioperativedeath
. dysphagia(esophageal 7. Colonnecrosis
compression) Cause: occlusionof inferiormesentericarteryby
. stridor,dyspnea(tracheobronchial compression)
., hoarseness(recurrentlaryngealnervecompression) stent-graft
8. Aorticdissection
! mediastinalmass with proximityto aorta
Cause: retrogradeinjuryby deliverysystem
r/ wide tortuousaorta
{ curvilinearperipheralcalcifications (75%)
r/ circumferential / crescenticmuralthrombus AORTIC DISSECTION
r/ Angio: may show normafcalibersecondaryto mural = spontaneouslongitudinal separationof aorticintima
t hr om bus + adventitiaby circulatingblood havinggainedaccessto
Cx: (1) Ruptureinto mediastinum, pericardium, either the mediaof the aorticwall splittingit in two
pleuralsac, extrapleuralspace Path: (a) transversetear in weakenedintima(95-97%)
r/ trign-anenuation fluid (b) no intimaltear (3-5-13%) = TNTRAMURAL
(2) Aortobronchopulmonary fistula HEMATOMAOF AORTA
! consolidation of lungadjacentto aneurysm Pathogenesis:
0 Mostaneurysmsrupturewhen >10 cm in size intimaltear resultsfrom combinationof followingfactors:
Prognosis; 1-yearsurvival57"/o,3-year survival26/o, (1) mediadegeneration decreasescohesiveness within
S-yearsurvival1g% (60%die from ruptured aorticwall
aneurysm,40"/"die from othercauses) (2) persistentaorticmotionsecondaryto a beatingheart
Rx: operativerepairconsideredif >6 cm in diameter stressesthe aorticwall
Surgicalmortality: 1O% (3) hydrodynamicforcesaccentuatedby hypertension
608 RadiologyReviewManual

lncidence; 3:1,000(morecommonthan all rupturesof DeBakeytype lll (50%) = descendingaortaonly

thoracic+ abdominalaortacombined);1:205 subtypelllA = up to diaphragm
autopsies;2,000 cases/yearin USA subtypelllB = belowdiaphragm
Peak age: 60 years (range 13-87 years); M:F = 3:1
Predisposed: (cysticmedial necrosis/ disease of aortic Stanfordtype A (60-70%) = ?SCeflding aorta+ arch
wall) in first4 cm in 90%
Startsin fusiformaneurysmsin 28% Stanfordtype B (30-40%) = descendingaortaonly
Doesnot occurin aneurysms<5 cm in diameter mnemonic; A affectsascendingaortaand arch;
Hypertension(60-90%) 9. Bicuspidaorticvalve B beginsbeyondbrachiocephalic vessels;
Marfansyndrome(16%) 10. S/P prostheticvalve l=ll+lll
E hler s - Da n l ossy n d ro me 1 1 . T ra u m a(ra re)
Relaps ing p o l y c h o n d ri ti sl 2 .C a th e te ri zati on Clinical classification :
Valvularaorticstenosis 13. Pregnancy (1) Acuteaorticdissection: <2 weeksold
Turnersyndrome 14. Aortitis(eg,SLE) (2) Chronicaorticdissection: >2 weeksold
Behgetdisease 15. Cocaineabuse
Coarctation NOT syphilis Locationof dissection(followinghelicalflow pattern):
ln women50% of dissections occurduringpregnancy! - on anterior+ right lateralwall of ascendingaortajust
distalto aorticvalve (65%)
. sharptearingintractableanterior/ posteriorchest pain - on superior+ posteriorwall of transverseaorticarch
(75-95%) radiatingto jaw, neck,low back (10%)
(DDx: myocardialinfarction) - on posterior+ left lateralwall of upperdescending
a
murmur+ bruit (65%)from aorticregurgitation aortadistalto left subclavianartery(20%)
a
asymmetricperipheralpulses+ blood pressures(59%) - moredistalaorta(5%)usuallyterminatingin leftiliac
a
absentfemoralpulses(25%),reappearingafter reentry artery(80%)/ right iliacartery(10%) [involvementof
o pulsedeficit:in up to 50% of type A dissection,in 16/" left renalarteryin 50%l
of type B dissection 0 An exit/ distaltear/ reentryoccursin 10%!
hemodynamic shock (25%)
neurologicdeficits(25%): hemiplegia,paraparesis(due Atypicalconfigurations of intimalflap:
to compromiseof anteriorspinalarteryof Adamkiewicz) r/ circumferential intimalflap due to dissectionof entire
persistentoliguria i nti ma
congestiveheartfailure(rare)due to acute aortic r/ filiformintimalflap creatingan extremelynarrowtrue
insufficiency lumen(+ ischemiccomplications)
. recurrentarrhythmias / rightbundlebranchblock r/ muralcalcification of falselumen(in chronic
. signsof pericardialtamponade:cloudedsensorium, dissection)
extremerestlessness,dyspnea,distendedneckveins ./ three-channelaorta (= Mercedes-Benzsign) due to
Types: two falsechannels
DeBakeytype | (29-34%) = €IScerdingaorta r/ intimointimalintussusception
+ portiondistalto arch
DeBakeytype ll (12-21%) = €lsc€I'lding aortaonly CXR (bestassessmentfrom comparisonwith serialfilms):
{ normalCXR in 25%
{ "calcificationsign" = inwarddisplacementof
atherosclerotic plaqueby >4-10 mm from outer aortic
contour(7"/"),can only be appliedto contourof
descendingaorta secondaryto projection,may be
misleadingin presenceof periaorticsoft-tissuemass /
hematoma
! disparityin size betweenascending+ descending
aorta
r/ irregularwavy contour/ indistinctoutlineof aorta
r/ wideningof superiormediastinum to >8 cm due to
hemorrhage / enlargingfalsechannel(40-80%)
r/ cardiacenlargement(LV hypertrophy/
hemopericardium)
r/ tettpleuraleffusion(27%)
r/ atelectasisof lower lobe
r/ rightwarddisplacementof trachea/ endotrachealtube

DeBakev Tvne I DeBakev Tvoe II DeBakev Tvpe III E C H O:

Stanford Tj'ire A Stanforil Tyfe A Stanforil T!'ire B
(a) transthoracicUS: 59-857osensitive+ 63-96%
Aortic Dissection specificfor type A dissection;poorerfor type B

(b) transesophagealUS: up to 9g% sensitive+ 77-g7"h
specific
(c) intravascularin conjunctionwith aortographyto
differentiatetrue from false lumen
r/ intimalflap (seenin morethan one view)
r/ pericardialfluid
r/ aorticinsufficiency
False-positives: reverberationechoes from
aneurysmalascendingaorta/ calcifiedatheromatous
plaque,postoperativeperiaortichematoma
Angio (86-88% sensitive,75-94% specific):
0 Aortography1st choice for finalconfirmation+ staging
becauseof contrastlimitation!
Superiorto any othertechniquein demonstrating
- entry + reentrypoints(in 50%)
- branchvesselinvolvement+ coronaryarteries
- aorticinsufficiency
r/ visualizationof intimal/ medial flap(75-7go/o)
= linear
radiolucencywithinopacifiedaorta
, "double
! barrelaorta"(87%)= opdcificationof two
aorticlumens
r/ abnormalcatheterpositionoutsideanticipatedaortic
course
{ compressionof true lumenby false channel(72-g1o/")
r/ aorticvalvularregurgitation13OZ;
{ increasein aorticwall thickness>6-10 mm
d obstructionof aorticbranches: left renalartery
(25-i30%)
r/ ulcerlikeprojectionscausedby truncatedbranches
{ slowerbloodflow in falselumen
False-negative:completethrombosisof false channel
(10%),intimalflap not tangentialto x-ray beam
False-positive:thickeningof aortic wall due to
aneurysm,aortitis,adjacentneoplasm/ hemorrhage
CECT (87-94% sensitive,B7-1OO%specific):
within4 hours (if patientrespondsrapidlyto medical
Rx);detectionas accurateas angiowith single-level
dynamicscanning
^/ crescentichigh-attenuation clotwithinfalselumen
! internallydisplacedintimalcalcification(DDx:
calcificationof thrombuson luminalsurfaceor within)
./ intimalflap separatingtwo aorticchannels(may be
seen withoutcontrastin anemicpatients)
False-negative:inadequatecontrastopacification,
thrombosedlumen misinterpreted as aorticaneurysm
with muralthrombus
False-positive:perivenousstreaks secondaryto beam
hardening+ motion,cardiaci aorticmotionartifacts,
opacifiednormalsinus of Valsalva,normalpericardial
recessmistakenfor thrombus,muralthrombusin a
fusiformaorticaneurysm,periaorticfibrosis,anemia
with apparenthigh attenuationof aorticwall
MR (95-100%sensitive,90-100%specific):
r/ intimalflap of mediumintensityoutlinedby signal
voids of rapidlyflowingblood
r/ intimalflap more difficultto detectin presenceof slow
flow/ thrombus
CardiovascularDisorders O0g
r/ "cobwebS"(= bandsof medialelasticlamellae
spanningthe junctionof the dissectingseptumwith
the outerwall of the false lumen)mark the false
l umeni n 80%
Cx: (1) Retrogradedissection(in Stanfordtype A)
(a) aorticinsufficiency
(b) occlusionof coronaryartery(g%)
(c) internalruptureinto RV, LA, vena cava,
pulmonaryarteryproducinglarge L-to-R
shunt
(2) Occlusion/ transientobstructionof majoraortic
branches(in up to 27"/")
(a) staticobstruction
./ ttap entersbranch-vesselorigin
(b) dynamicobstruction= flap sparesbranch-
vesseloriginbut coversit like a curtain
{ collapsedtrue lumenoutlinedby a
C-shapedflap envelopewhich is concave
towardfalselumen(ischemic configuration)
(3) Externalruptureof aorta into pleuralcavity/
pericardialsac: 70ohmortality(= most common
cause of death within24 hours)
(4) Development of aneurysm(15%)of the true/
falselumen
0 Organsmay receivetheir bloodsupplythrough
eitherthe true or falselumenor both!
Rx:
(1) Reducingpeak systolicpressureto 120-70 mm Hg
(adequatealonefor type lll = 3, whichrarely
progressesproximally):death from ruptureof aortic
aneurysmin 46/" of hypertensive+ 17"/"ol
normotensivepatients
Suruivalrate: 4A-7A%(with medical/ surgical
management)
(2) lmmediatesurgicalgraft reinforcementof aorticwall
(Type l, ll = A) preventingrupture+ progressive
aorticvalve insufficiency
Nonsurgical suruival rate: <10o/o
Postsurgical mortality: 10-35%
Cx: myocardialinfarction,stroke,respiratory
insufficiency, pulmonaryembolism,aortic
rupture,pseudoaneurysm, graft infection
Prognosis without Rx:
immediatedeath(3%); deathwithin: 1 day (20-30%),
1 week (50-62%),3weeks(60./"),1month(75%),
3 months(80%),1 year (80-95%)
Prognosis with Rx:
5-10% mortalityrate followingtimelysurgery;
40/" lO-yearsurvivalrate after leavinghospital
DDx: penetratingulcerof thoracicaorta (= atherosclerotic
lesionof mid-descending aortawith ulceration
extendingthroughintimaintoaorticmedia)
IntramuralAortic Hematoma(3-13%)
= oorticdissectionwithoutruptureof intima
Cause: hemorrhageof vasa vasorum
. signs+ symptomsidenticalto classicdissection
N E C T:
{ cuff / crescentof high attenuation
r/ displacementof intimalcalcification
610 RadiologyReview Manual

CECT: NUC:
r/ mural regionof low attenuationwith smoothborder r/ uptakeof Tc-99m hexametazinelabeledleukocytes
maintaining a constantcircumferential relationship (drawbacks:not performedquickly,hepatobiliary
with aorticwall excretion)
Aortography: notuseful! Prognosis: 17-75/"mortality; 30-50% morbidity
Cx: ulcerlikeprojectionwith progressionto open Dx: positiveculturefrom needleaspirate(incubation
dissection/ saccularor fusiformaneurysm periodshouldbe up to 14 days as organismsmay
Rx: (1) emergencysurgicalrepairfor type A be slow-growing)
hematoma(probablyrepresentsearly stage of
classicaorticdissection)
(2) observationfor type B hematoma(may heal AORTICREGURGITATION
completely) = AORTIC INSUFFICIENCY
DDx: Cause:
(1) Acutelythrombosedfalselumenof dissection A OR TICV A LV ED IS E A S E
A . IN TR IN S IC
(tendencyto spirallongitudinally aroundaorta) 1. Congenitalbicuspidvalve
(2) Atheromatous muralthrombus(irregularinternal 2. Rheumaticendocarditis
bordeQ 3. Bacterialendocarditis(perforation/ prolapseof
(3) Focalperiaorticsoft-tissuemass (irregularexternal cusp)
border) 4. Myxomatousvalve associatedwith cysticmedial
(a) idiopathicperiaorticfibrosis necrosis
(b) periaorticlymphoma 5. Aorticvalve prolapse
6. Prosthetic valve: mechanicalbreak,thrombosis,
paravalvularleak
A ORTA
B . P R IMA R YD IS E A S EOF A S C E N D IN G
(a) Dilatationof aorticannulus
AORTICPROSTHETIC
GRAFTINFECTION 1. Syphiliticaortitis
lncidence: 1.3-6% of prostheticgraft procedures 2. Rheumatoidarthritis
Classification: 3 1"iiffi[**'iJ,ll,*,
( 1) P E RT G RAFTTN F EC T T O(2N-6 % ) (5-1
o%)
. fever,chills,leukocytosis Reiterdisease
. groinswelling,heat,tenderness,pulsatilemass,
drainingsinustract
(2) AORTOENTERTC FTSTULA (0.6-2%)
4; :;:'ilx''i"?iHiii,.',',
5. Familialconnectivetissuedisease:
. acute/ chronicGl bleeding(maybe occult) mnemonic.'"HOME"
. sepsis Homocystinuria
. may be temporallyremote(up to 10 years): Osteogenesisimperfecta
mediantime of 3 years to manifestation(70% occur Marfansyndrome
after 1st year) "
. intracavitarysigns: malaise,back pain,fever,elevated (b)r-ace
rai,
l.':';?iil'.:.'Jl1':il
sedimentation rate,hydronephrosis, ischemiafrom 1. Deceleration
trauma
clotted graft 2. Hypertension
Normalpostoperativecourse:
{ ring of fat attenuationin early postoperativeperiod
<5 mm betweenaneurysmwall and graft
0 Completeresolutionof hematomaby 3 months
0 Disappearanceof ectopicgas completeby 4-7 weeks
CI (94% sensitive,85% specific,917oaccurate):
r/ perigraftfluid
r/ perigraftsoft-tissueattenuationwith indistinctnessof
graft margins
r/ ectopicgas (fistulouscommunication with bowel/
gas-producing organism)
r/ pseudoaneurysm(25%)
r/ focal bowelwall thickening(indicatesfistula)
./ >5 mm soft tissuebetweengraft + surroundingwrap
(beyond7th postoperativeweek) Mitral Valve in SevereAortic Regurgitation
./ focal discontinuityof calcifiedaneurysmalwrap The valve is almost completely closed before onset of ventricular
False positives: systole. Atrial contraction has little effect in reopening the valve.
perigrafthematomain early postoperativeperiod, Complete closure occurs with ventricular systole. A high-velocity
pseudoaneurysm(in 15-20%) flutter of aML is present in diastole.
 Cardiovascular
Disorders 6l l

Pathogenesis: progressiveenlargementof diastolic AORTICRUPTURE

+ systolicLV dimensionsresuftin
increasein myocardialfiber length
+ increasein strokevolume;
decompensationoccursif criticallimitof
fiber lengthis reached
. "water-hammerpulse"= twin-peakedpulse
' systolicejectionmurmur+ high-pitched
diastolicmurmur
. AustinFlintmurmUr= sefl mid-diastolic or presystolic
bruit
./ LV enlargement(cardiothoracic ratio>0.55)+ initially
normalpulmonaryvascularity(DDx: congestive
, cardiomyopathy, pericardialeffusion)
{ normalaorta (in intrinsicvalvedisease)
{ dilatationof aorta (in systemicdisease):
r/ + calcificationof ascendingaorta (in aorticwall
disease)
+ enlargedaorticarch + tortuousdescendingaorta
,{
r/ increasedpulsationsalong entireaorta
ECHO :
r/ aorticroot dilatation
r/ nign frequencyflutterof aML (occasionallypML)
AORTICSTENOSIS
, duringfirst2/3 of diastote(CHARACTERTSTTC)
r/ nign frequencydiastolicflutterof IVS (uncommon)
{ diastolicflutterof aorticvalve (SpEClFlC,but rare)
./ prematureaorticvalve opening(highdiastolicLV
pressure)
r/ decreasedMV opening(aML pushedposteriorlyby
, regurgitantaorticjet)
r/ prematureclosureof mitralvalve (highdiastolicLV
pressureproducesMV closurebeforebeginningof
, systolein severeacute aorticinsufficiency)
r/ LV dilatation+ largeamplitudeof LV wall motion
(volumeoverload,increasedejectionfraction):
End-systolicLV diameter Action
<50mm yearlyfollow-up
50-54mm 4- to 6-monthfollow-up
>55mm valve replacement
Doppler:
r/ slope of peak diastolicto end-diastolicvelocity
decrease>3 m/sec2in severeaorticregurgitation . asymptomaticfor many years
./ area of color Dopplerregurgitantflow . angina,syncope,heartfailure
r/ ratioof width of regurgitintOeamto width of aortic . systolicmurmur
root is good predictorof severity(colorDoppler) . carotidpulsusparvuset tardus
= blood feakagethroughaorticwall
1. Spontaneousruptureof aorticaneurysm
Pathogenesis: smallcleftsoccur at a fragilesite
withininnerthrombusgradually
expandingto outer layerof
thrombuswith graduafseepageof
flowingbloodinto muralthrombus
and aneurysmalwall
CT:
./ nign-attenuation
crescentsign (71%)
2. Spontaneousruptureof descendingthoracicaorta
Predisposed: hypertensionand atherosclerosis,
NO preformedaneurysml
Pathogenesis: pressureatrophyof media due to
overlyingintimal atheromatous
plaquecausinglocalizedballooning
of aorticwall priorto perforation
3. Traumaticrupture/ transectionof thoracicaorta
Cause: blunt trauma to thoracicaorta
Aorticvalve area decreasedto <0.8 cm2= 0.4 cm2lm2BSA
(normal2.5-3.5cm2)
A. ACQUIREDAORTICSTENOSIS
1. Rheumaticvalvulitis(almostinvariablyassociated
with mitralvalvedisease)
2. Fibrocalcificsenileaorticstenosis(degenerative)
B. CONGENITALAORTTCSTENOSTS (mostcommon)
= fiost frequentCHD associatedwith IUGR
1. Subvalvular AS (15-30%)
2. ValvularAS (G0-70%): degenerationof bicuspid
valve most commoncause
3. SupravalvularAS (rare)
Pathogenesrb:increasedgradientacross valve produces
-V hypertrophy and diminishedLV
compliance;increasedmusclemass may
outstripcoronaryblood supply
(subendocardial myocardialischemiawith
angina);LV decompensationleadsto LV
dilatation+ pulmonaryvenouscongestion

ValvularAortic Stenoses
Congenital Rheumatic Degenerative
Clinicallyapparent <30years 30-60 years >65 years
Valvecalcifications
first appearance 25 years 47 years 54 years
pattern nodular/ bicuspid nodular nodular/ tricuspid
on CXR >90"/"(40-65years) <1A"/" >90o/"(>65 years)
Aorticectasia ascending Ao ascendingAo entireAo
612 RadiologyReview Manual
- J r ' t F t \
Aortic Valve in Hypertrophic Subaortic Stenosis
duringmidsystolethe aorticvalve closessecondaryto subvalvular
obstruction
. diminishedaorticcomponentof 2nd heartsound
. suddendeath in severestenosis(20%)after exercise
(diminishedflow in coronaryarteriescausesventricular
dysrhythmias + fibrillation)
{ poststenoticdilatationof ascendingaorta (in 90% of
ac quir ed,in70 /" o f c o n g e n i taAS
l )
^/ normal-sized / enlargedLV (smallLV chamberwith thick
walls)
@ in adults>30 years
{ calcificationof aorticvalve (bestseen on RAO);
indicatesgradient>50 mm Hg
r/ discreteenlargement of ascendingaorta(NO
correlationwith severityof stenosis)
r/ calcification of mitralannulus
^/ "leftventricularconfiguration" = concavityalongmid
left lateralheartborder+ increasedconvexityalong
lower left lateralheartborder
@ in children/ youngadults
{ prominentascendingaorta
^/ tettventricularheartconfiguration
@ in infancy:
r/ tettventricularstresssyndrome
ECHO :
./ thickened+ calcifiedaorticvalvewith multipledense
cusp echoesthroughoutcardiaccycle (right>
noncoronary> left coronarycusp)
r/ decreasedseparationof leafletsin systolewith
r educ edope n i n go ri fi c e(1 3 -1 4m m = mi l dA S ;
8-12 rTuTr = moderateAS; <8 ITllTt = severe AS)
{ + domingin systole
r/ ditatedaorticroot
r/ increasedthicknessof LV wall (= concentricLV
hypertrophy)
r/ hyperdynamiccontractionof LV (in compensated
state)
! decreasedmitralEF slope (reducedLV comptiance)
^/ m enlargement
r/ increasedaorticvalve gradient(Doppler)
^/ decreasedaorticvalve area (unreliable)
DDx: calcificationof aorticannulusin elderly/ calcified
coronaryarteryostium (thickenedcusp echoes
only in diastole)
Prognosis; dependsonsymptomatology(angina,
syncope,CHF)
Aortic Valvular Stenosis
decreasedseparationof thickened deformed leaflets
SubvalvularAorticStenosis
= SUBAORTIC STENOSIS
(a) Anatomic/ fixed subaorticstenosis
Associatedwith: cardiacdefectsin 50% (usually
vsD)
Type I : thin 1-2-mm membranous
diaphragmatic stenosis,usuallylocated
within2 cm or lessof valveannulus
Typell : thickcollarlikestenosis
Typelll : irregularfibromuscular stenosis
TypelV : "tunnelsubaorticstenosis"= fixed
tunnel-likenarrowingof LVOT
= eXcesSive thickeningof only upper
ventricularseptumwith normalmitral
valvemotion
(b) Functional/dynamicsubaorticstenosis
1. Asymmetricseptalhypertrophy(ASH)
2. ldiopathichypertrophic subaorticstenosis(IHSS)
3. Hypertrophicobstructivecardiomyopathy (HOCM)
may occur in infantsof diabeticmothers
no dilatationof ascendingaorta
asymmetrically thickerventricularseptumthan free
wall of LV (95%)
normali smallleft+ rightventricularcavities(95%)
lucentsubaorticfillingdefect in systole
focal convexityof left upper-midcardiacmargin
= anterioraspectof ventricularseptum(rare)
E C H O:
^/ coarsesystolicflutterof valve cusps
{ openingof leafletsfollowedby rapid inwardmove in
mid systole,leafletsmay remainin partiallyclosed
positionthroughlatterportionof systole(to appose
bordersof the flow jet)
{ systolicanteriormotionof mitralvalve
Cx: mitralregurgitation(secondaryto abnormal
positionof anterolateralpapillarymuscle
preventingcompleteclosureof MV in systole)
Valvular Aortic Stenosis
= fusionof commissuresbetweencusps
Degree: mild: >0.7 cm2;moderate:0.5-0.7 cmz;
severe:<0.5cm2
 Cardiovascular Disorders 6l g

Congenital types: . clinically

resembles PDA
(a) bicuspid/ unicuspid(in 9S%): in 1-2% of CXR:
population;M > F; commonlyassociatedwith r/ shuntvascularity
coarctationof the aorta { cardiomegaly (LA+ LVenlarged)
(b) tricuspid(5%) r/ diminutive
aorticknob
(c) dysplasticthickenedaorticcusps ./ prominentpulmonarytrunk
{ valvularcalcifications(in 60%oi patients>24 years Angio(leftventriculogram
/ aortogramin Ap / LAO
of age) projection):
@ lN INFANTwith criticalaorticstenosis: r/ defectseveralmmaboveaorticvalve
. intractableCHF in first days / weeks of life with
r/ pulmonaryvalveidentified
(DDxto truncusarteriosus)
severedyspnea
. may simulateneonatalsepsis
Associatedwith: L-to-Rshunts(ASD,VSD)
d markedcardiomegaly(thickenedwall of LVy ARTERIOSCLEROSIS
OBLITERANS
^/ pulmonaryvenoui hypertension = A S O= H A R D E N INOFG TH EA R TE R IE S
r/ decreasedejectionfiaction Prevalence: 2.4 millionpeoplein USA; in 197g 12%of
r/ domingof thickenedvalve cusps autopsieshad ASO as leadingcause of
./ OitateOascendingaorta death(excludingMt)
Rx: emergencysurgicaldilatation Etiology: unknown
@ rNcHtLD: Contributing factors:
. asymptomaticuntil late in life aging,diabetes(1644%), hypertension,atherosclerosis
r/ normalpulmonaryvascularity Effectof hyperlipidemia:
r/ l-V configurationwith normalsize of heart (a) High-densitylipoproteins(HDL)have a protective
r/ largeposteriornoncoronary cusp,smallerfused effect: carry 25/. of bloodcholesterol
right+ left cusps (b) Low-density lipoproteins(LDL): carry60% of
^/ domingof thickenedvalve cusps bloodcholesterol
1 eccentricjet of contrast Histo: depositionof lipids,blood products,
{ poststenoticdilatationof ascendingaorta carbohydrates,beginsas disruptionof intimal
E CHO : surface;fatty streaks(as early as childhood);
{ increasein echoesfrom thickeneddeformed fibrousplaques(as earlyas 3rd decade);
, leaflets(maximalduringdiastole) thrombosis,ulceration, calcification,
aneurysm
ri decreasein leafletseparation Age: 50-70 years; M > F (aftermenopause)

CIinical cl assification :
SupravalvularAortic Stenosis (1) intermittentclaudication= ischemicsymptomswith
Types: exercise:calf,thigh,hip,buttock
(a) localizedhourglassnarrowingjust above aortic (2) ischemicsymptomsat rest (indicativeof
sinuses multisegment disease)
(b) discretefibrousmembraneabove sinusesof . cramping/ burning/ achingpain
Valsalva . cold extremity
(c) diffusetubularhypoplasiaof ascendingaorta . paresthesia
+ branchingarteries . trophicchanges: hair loss,thickenednails
Associatedwith: peripheralpS, valvular+ discrete . ulcer,gangrene
subvalvularAS, Marfansyndrome, . decreasedi absentpulses
Williamssyndrome,infantile Location: medium+ largearteries;frequentlyat
hypercalcemiasyndrome bifurcations;mostfrequent:
r/ small ascendingthoracicaorta - superficialfemoralarteryin adductorcanal
^/ dilatation+ tortuosityof coronaryarteries(may (diabetics+ nondiabetics)
undergoearly atherosclerotic degenerationsecondary - aortoiliacsegment(nondiabetics)
to high pressure) - tibioperoneal trunk(diabetics)
E CHO :
r/ narrowingof supravalvularaorticarea (normalroot Prognosis:
diameter:20-37 mm) accelerated by diabetes(34%will requireamputation),
r/ normalmovementof cusps hypertension, lipoprotein
abnormalities,heartdisease
(decreasedcardiacoutputresultingin increasedblood
viscosityfrom polycythemia),
chronicaddictionto
AORTOPULMONIC WINDOW tobacco(11/% will requireamputation), intermittent
= defectin septationprocesscharacterizedby large round claudication(5-7% requireamputationif nondiabetic
/ ovalcommunicationbetweenleft wall of ascending = 1-2/" per year),ischemiculceri restpain (19.6%
aorta+ rightwall of pulmonarytrunk requireamputation)
614 RadiologyReview Manual

ATRIALSEPTALDEFECT
0 Most commoncongenitalcardiacdefectin adults
>20 years of age!
lncidence: 8-14/" of all CHD; M:F = 1:4
Age: presentationfrequently> age 40 secondaryto
benigncourse
(a) mildlysymptomatic(60%): dyspnea,fatigue,
palpitations
(b) severelysymptomatic(30%): cyanosis,heart
failure
septum secundum
septum pnmum
Embryology:
coronarv smus
1. Septumprimum= €lt4th week membranegrows
Normal NewbornHeart from dorsalatrialwall towardendocardialcushions
Atrial septum consists of two components 2. Ostiumprimum= temporaryorificebetweenseptum
(a) right side: septum secundum (muscular, firm) with primum+ endocardial cushionscloseto AV valves;it
posterior opening = foramen ovale becomesobliteratedby 5th week
(b) left side: septum primum (fibrous, thin) with anterior
3. Ostiumsecundum= multiplesmallcoalescing
opening = ostium secundum
fenestrationsin centerof septumprimum
4. Septumsecundum= meffibr?nedevelopingon right
side of septumprimum+ coveringpart of ostium
septum secundum secundum
5. Foramenovale= orificelimitedby septumsecundum
+ septumprimum
6. Foramenovaleflap = loweredge of septumprimum
(patentin 6/", probe-patentin25"/");not considered
an ASD

A. OSTTUMSECUNDUMASD (60-70%)
= ex?ggeratedresorptiveprocessof septumprimum
septum primum leadsto absence/ fenestrationof the foramenovale
flap (Chiarinetwork)
Ostium Secundum Defect
Location: in the centerof the atrialchamberat fossa
ovalis
Size: largedefectof 1-3 cm in diameter
SVC May be associated with:
prolapsingmitralvalve (20-30%),pulmonaryvalve
stenosis,tricuspidatresia,TAPVR,hypoplasticleft
heart,interruptedaorticarch

B . OS TTU M P R IMU MA S D (30% )

= defectof atrioventricularendocardialcushion
Location: lowerend of septuminferiorto fossa ovalis
anomalous right upper lobe veins (at outletportionof atrialseptum)
Almost always associated with:
SinusVenosusDefect endocardialcushiondefects,cleft mitralvalve,
anteriorfascicularblock

c. srN U SV E N OS U SA S D (5% )
septumsecundum
= defectof the superiorinlet portionof the atrial
sePtum
Location: superiorto fossa ovalisnear entranceof
superiorvena cava (SVC straddlesASD)
Associatedwith: partialanomalouspulmonary
venousreturnin 90% (RUL
pulmonaryveinsconnectto SVC /
rightatrium),Holt-Oramsyndrome,
septum primum Ellis-vanCreveldsyndrome

Ostium Primum Defect SYNDROME= ASD + mitralSIENOSIS

D. LUTEMBACHER
 Cardiovascular
Disorders 6l s

Hemodynamics: A ngi o:
no hemodynamic perturbance in the fetus;afterbirth .l nn fills with contrastshortlyafter LA is opacified(on
physiologicincreasein LA pressurecreatesa L_to_R levophaseof pulmonaryangioin Ap or LAO
shunt(shuntvolumemay be 3-4 timesthat of systemic projection)
bloodflow) with volumeoverloadof RV leadingio RV { injectioninto RUL pulmonaryvein to visualizeexact
dilatation,rightheartfailure,pulmonaryhypertension; size + locationof ASD (LAO 45" + C-C 45")
diastolicpressuredifferencesin atriadetermine Prognosis:
directionof shunt;pulmonarypressureremainsnormal (1) Mortality:0.6% in 1st decade;0.7% in 2nd decade;
for decadesbeforeEisenmenger syndromesets in; 2.7"/"in 3rd decade;45% in 4th decade;5.4% in 5th
pulmonaryhypertension in youngadulthood(6%) decade;7.5% in 6th decade;medianage of death is
37 years
R A I RV T MainPA t (2) Spontaneous closure:22/"in infants<1 year;33/o
Pulmvessefs t betweenages 1 and2years;3%inchildren>4 years
L A < - > L V < + A o +/e Cx: (1) Tricuspidinsufficiency (secondary to dilatation
of AV ring)
(2) Mitralvalve prolapse
. repeatedrespiratoryinfections (3) Atrialfibrillation(in 20% 1st presentingsymptom
. feedingdifficulties in patients> age 40)
. arrhythmias Rx: (if vascularchangesstill reversible= resistanceof
. thromboembolism pulmonary-to-systemic system<0.2); 1"/osurgical
. asymptomatic;occasionallydiscoveredby routineCXR mortality
. rightventricularheave 1. Surgicalpatchclosure
' fixedsplittingof secondheartsound with accentuation 2. Rashkindfoam + stainlesssteel prosthesis
of pulmonarycomponent(ejectionmurmurgradelllVl)
heardat 2nd left intercostalspace along pA B E N E FIC IAALS D
' ECG: rightaxisdeviation+ somedegreeof rightbundle = SecUfldum type ASD servesan essential
branchblock compensatory functionin:
. exertionafdyspneaafterdevelopmentof pulmonary 1. Tricuspidatresia
arterialhypertension (= Eisenmenger syndrome) RA bloodreachespulmonaryvesselsvia ASD
. cyanosismay occur(shuntreversalto R-to_Lshunt), + PDA; improvementthroughRashkindprocedure
typicallyduring3rd-4th decade 2. TAPVR
. right heartfailurein patients>40 years significantshuntvolumeonly availablethroughASD
(V S D/P D A muchl essreti abte)
CX R: 3. Hypoplasticleft heart
r/ normal(if shunt<2 x systemicbloodflow) systemiccirculationmaintainedvia RV with
r/ overcirculation = increasein pulmonarybloodflow (if oxygenatedbloodfrom LA throughASD into RA
, pulmonary-to-systemic bloodflow >2:1)
! cardiomegaly:
AZYGOSCONTINUATION
OF IVC
! heartsmallcomparedwith pulmonaryvascularity = INTERRUPTED
= IVCWITHAZYGOS i HEMIAZYGOS
, c los ings h u n t CONTINUATION = ABSENCE OFTHEHEPATIC SEGMENT
ri heart largecomparedwith pulmonaryvascularity OFTHEIVC WITHAZYGOS CONTINUATION
= intercurrent myocardial/aorticdisease Prevalence: 0.6/"
r/ loss of visualizationof SVC (= clockwiserotationof Etiology: formationfailureof rightsubcardinal-hepatic
heartdue to RV hypertrophy) anastomosiswith atrophyof rightsubcardinal
{ small appearingaortawith normalaorticknob vein + shuntingof bloodfrom supracardinal_
ri normalsize of LA after shunt reversal(dueto subcardinalanastomosisto cranialportionof
immediatedecompression into RA) in Eisenmenger supracardinalvein (= retrocruralazygosvein)
syndrome: May be associated with:
r/ enlargement of PA + centralpulmonaryarteries polysplenia syndrome(morecommon),asplenia
rl nV enlargement syndrome(rare),indeterminate situs(= situsambiguus),
r/ "hilardance"= increasedpulsationsof central persistentleft SVC, dextrocardia,transposedabdominal
pulmonaryarteries(DDx:otherL-to-Rshunts) viscera,duplicatedlVC, retroaorticleft renalvein,
, congenitalpulmonaryvenolobarsyndrome
ECHO: r/ absenceof hepatic+ infrahepaticIVC:
^/ paradoxicalinterventricularseptalmotion(dueto r/ drainageof hepaticveinsinto rightatriumvia supra-/
volumeoverloadof RV) posthepatic segmentof IVC (N.B.: IVC shadow
r/ directvisualizationof n'SO1=lack of echoesof atrial presenton LAT CXR!)
, septum)in subcostalview ./ drainageof iliac+ renalveinsvia azygos/hemiazygos
! diastolicbloodflow from interatrial
septumcrossing vein:
RA + tricuspidvalve observedby color Doppler r/ rightrenalarterycrossesanteriorto ,,lVC,,onUS
616 RadiologyReview Manual

lncidence; <1"/"of all chronicvasculardiseases; more

commonin lsrael,Orient,India
Etiology: unknown
Histo:
(a) acute stage: multiplemicroabscesses withinfresh/
organizingthrombus;all layersof vesselwall
inflamedbut intact; internalelasticlaminamay be
l
/l-
i i
ilru damaged; multinucleated giantcellswithin
microabscesses (PATHOGNOMON lC)
(b) subacutestage: thrombusorganization with little
Aortic Valve Endocarditis residualinflammation
(c) chronicstage: lumenfilledwith organized
recanalized thrombus,fibrosisof adventitiabinds
r/ both gonadalveins drain into ipsilateralrenalvein (since togetherartery,vein, and nerve
postcardinal-subcardinalanastomosisdoes not Associatedwith: cigarettesmoking (95%)
contributeto formationof IVC) . instepclaudication+ distalulceration(symptomsabate
C X R: on cessationof smoking+ returnon its resumption)
{ enlargementof azygosarch to >7 mm . Raynaudphenomenon(33%)
r/ wideningof rightparaspinalstripecontiguouswith Location: legs (80%),arms (10-20%)
azygosarch (= enlargedparaspinal+ retrocrural Site: startsin palmar+ plantarvesselswith proximal
azygosveins) progression
{ wideningof leftparaspinalstripe(= enlarged ! superficial+ deep migratorythrombophlebitis (20-33%)
hemiazygosvein) { arterialocclusions,taperednarrowingof arteries
DDx: right-sidedparatrachealmass with retrocrural r/ abundantcorkscrew-shaped collaterals
adenopathy ^/ directcollateralfollowingthe path of the originalartery
(Martorell sign)in 80%
BACTERIAL ENDOCARDITIS { skip lesions= multiplesegmentsinvolvedwith portions
Predisposed: of arterialwall remainingunaffected
1. Rheumaticvalvedisease { absenceof generalizedarteriosclerosis I arterial
2. Mitralvalve prolapsewith mitralregurgitation calcifications(90%)
3. Aorticstenosis,mitralstenosis,aorticregurgitation,
mitralregurgitation
4. MostCHD (VSD,TOF) exceptostiumsecundum AORTA
BLUNTTRAUMATO THORACIC
ASD = AORTICLACERATION
5. Previousendocarditis = lacerationthat disruptsthe physicalintegrityof
6. Drugaddicts: >1 structurallayersof the aorta
endocarditisof tricuspidvalve causes multipleseptic Cause: rapiddeceleration(high-speedMVA >48 kmih
pulmonaryemboli with unrestraineddriveror ejectedpassenger,
7. Bicuspidaorticvalve: fall from heightt3 m) / crushingchestinjury
responsiblefor 50% of aorticvalvularbacterial Pathomechanism:horizontal/ verticaldeceleration,
endocarditis hydrostaticforce,osseouspinch
8. Prostheticvalve: Lengthof tear: circumferential tear (in majority)
4% incidenceof bacterialendocarditis
{ exaggeratedvalve motion(= disintegrationof Site: (a) Aorticisthmusjust distalto left subclavianartery
sutureline+ regurgitation) (88-95%): brachiocephalic arteries
+ ligamentumarteriosumfix aortain this region
ValveVegetations (b) Aorticarch with avulsionof brachiocephalic
E CHO : trunk(4.5%)
{ usuallydiscretefocal echodensitieswith sharp (c) Ascendingaorta immediatelyabove aorticvalve
edges;may showtuzzy/ shaggynonuniform (5-e%)
thickeningof cusps (vegetations)in systole Cx: aorticvalve rupture,coronaryartery
+ diastole laceration,hemopericardium + cardiac
r/ may appearas shaggyechoesthat prolapsewhen tamponade;NO mediastinal hematoma
the valve is closed(DDx to mitralvalve prolapse) (d) Diaphragmatic hiatus(1-3%)
0 Most often posteriorly(in noncircumferential tear)
DISEASE
BUERGER
= THROMBANGITIS OBLITERANS Extentof laceration:
= idiopathicrecurrentsegmentalobliterativevasculitisof 1. l ncompl ete rupture(15% )
small+ medium-sized peripheralarteries+ veins 0 Aorta goes on to rupturecompletelywithin24
(panangitis) hoursin 50% of patients!

- I NT I M A
(a) intimalhemorrhagewithouttear
(b) transverselacerationof intimawith
hemorrhage(= intimal tear ltlap
= traumatic aortic dissection)
- MEDIA
tear into mediawith subadventitialaccumulation
of blood (40-60%)
_ A DV E N T IT IA
(a) periaortichemorrhage+ aorticinjury
(b) traumaticfalse aneurysm= involvesintima
+ media + adventitiawith locallycontained
hematoma
2. Completerupture(85%)= transmuralextensionof
laceration= ?ottic transection
= traumatic aortic rupture
. exsanguination beforereachinga hospital
AcuteThoracicAorticInjury
Prevalence: 10-16-20"/"of allfatalitiesin high-speed
decelerationaccidents
. severechest pain: precordial(ascending
aorta),neck_
jaw (aorticarch),interscapular(descendingthoracic
aorta)
. anteriorchestwall contusion,dyspnea,dysphagia
. bloodpressurechanges:
. unexplained hypotension
. scapulothoracic syndrome= decreased/ absent
upperextremitypulses
. acute coarctationsyndrome= decreasedi
absent
lowerextremity+ normalupperextremitypulses
with upperextremityhypertension+ systolic
murmurin 2nd left parasternalinterspace
CX R:
0 A normalanteroposterior uprightCXR virtually
excludesacutethoracicaorticinjury
(96-98% negativepredictivevalue)!
N.B.: Thereare no plainCXR findingsof aorticinjury
(sinceaorticintegrityis maintainedby intaci
adventitia)!The sourcesof mediastinal
hematomaare frequentlythe azygos,
hemiazygos, internalthoracic,paraspinaland
intercostalvessels!
0 Aorticinjuryis the causeof mediastinal
hematomain only 12.5%l
r/ normaladmissionCXR ln 2g/. (radiographicsigns
may not developuntil6-36 hours): supineCXR is
very inaccuratefor mediastinalwidening
MoFtspecificsigns:
r/ deviationof nasogastric/ endotrachealtubeto
the rightof T3-T4 spinousprocess(12_1OO%
sensitive,80-95% specific)
r/ depressionof left mainstembronchus
anteroinferiorly>40" belowthe horizontal
+ toward right (53%)
r/ mediastinalwidening>8 cm at levefof originof left
subclavianartery(presentin 75-g2o/o:
53-93-1 00% sensitive,1-34-60% specific):
r/ mediastinafwidth to chestwidth >0.25
CardiovascuIar Disorders 617
ri indistinctaorticcontourat arch / descendingaorta
, (53-100%sensitive,21-SS% specific)
r/ obscurationof aortopulmonary window(40-jOO%
sensitive,56-8g% specific)
r/ widenedleft paraspinal"stripe">S mm
, (12-83% sensitive,89-97% specific)
r/ thickeningof rightparatrachealstripe>4-5 mm
, (= hematomabetweenpleura+ trachea)
r/ left / right "apicalpleural cap" sign in 37"/"
(= extrapleuralhematomaalong brachiocephalic
vessels)
r/ trachealcompression+ displacementtowardright
(61%)
{ rapidlyaccumulatingcommonlyleft-sided
hemothoraxwithoutevidentrib fracture(breakin
, rnediastinal pleura)
1 fracturesof 1st + 2nd rib (17%)
mnemonic.' "BAD MEAT"
Bronchusdepression(leftmain)
Aorticsilhouetteshaggy
Deathin 80-90%
Ittlediastinal widening
Enteric(nasogastric)tube displacement
Apical cap
Trachealshift
NECTscreening(55-100%sensitive,65-97% specific):
r/ obliterationof aorta-fatinterfacewith increased
attenuation(= mediastinalhematoma)
DDx: residualthymictissue,periaorticatelectasis,
pleuraleffusionadjacentto descending
aorta,volumeaveragingof pulmonaryartery
0 A negativeCT examinationfor mediastinal
hemorrhagehas an almost 1OO%NpV for aortic
injury!
0 All patientswith periaortic/ middle/ superior
mediastinalhemorrhagerequireaortographylSave
your contrastfor that study!
False positive:
residualthymictissue,atelectaticlung,pericardial
recess,patient motion,streak artifacts,paftial
volumeeffectwith pulmonaryartery
CECT (100%sensitive;81% specific;0-S9% false
positive;0.7% false negative):
Technique; 100-150 mL at 2 mlisec with
20-30 secondscanningdelay
Advantages:
(a) negativefindingswill obviateinvasiveangio
(b) unsuspectedinjuriesare discovered
(c) saves healthcare dollars
Disadvantages:
(a) CT delaysthe definitiveaortography+ surgery
(b) two contraststudiesare neededin the frequent
casesof mediastinalhemorrhage
(c) few data exist on accuracyof CT for branch
vessel injury
r/ intraluminal area of low attenuation:
r/ linear= intimalflap
r/ polypoid= clot
618 RadiologyReview Manual

r/ contourdeformityof inneraorticwall = intramural (b) with equivocalCXR: screeningCT of chest

hematoma (c) abnormalchestCT:
r/ aorticpseudoaneurysm angiography for confirmation i surgery
r/ pseudocoarctation = abrupttaperingof the diameter Prognosis:
of the descendingaortacomparedwith the (1) 80-90% fatal at scene of trauma
ascendingaorta (2) 10-20% reachhospital(due to formationof
r/ extravasationof contrastmaterial(rare) periaortichematoma+ falseaneurysmcontained
False positive: by adventitia+ surrounding connectivetissue)
linearmotionartifact(aorticvalve leaflets,wall of (a) withoutintervention:30% deadwithin6 hours;
ascendingaorta),volumeaveraging,prominent 40-50% dead within24 hours,90% dead
vessels,prominent
periaorticbronchial/ mediastinal within4 months;chronicfalseaneurysmmay
atheroma,smallductusdiverticulum developin 2-5/" at isthmus/ descendingaorta
(b) with surgicalrepair: 60-70% survive;surgical
Transesophageal echocardiography mortalityrate of 9-44'/" varieswith degreeof
(in 2-15% technicallyunsuccessful,57-63% sensitive, hemodynamic instability+ severityof
84-91% specific): associatedinjuries+ magnitudeof aortic
r/ intimalflap laceration
r/ intraluminal thickstripes Cx: postoperativeparaplegia(9%) due to
r/ pseudoaneurysm aorticcrossclamping>30 minutes
{ aorticocclusion(= pseudocoarctation)
{ fusiformaneurysm Aortic Pseudoaneurysm
ChronicPosttraumatic
r/ aorticwall hematoma = ?fleuIysmexistingfor >3 months(amountof wall
fibroplasia followingruptureusuallynot sufficientto
Aortography(almost100%sensitive,98% specific): preventsubsequentruptureuntilat least3 months
Technique;LAO + RAO projection;high-flowpigtail afterinitialtraumaticepisode)
catheter;50 mL at 35 mUsec lncidence: 2-5% of patientssurvivingaortic
True positive: transection >2f48 hours
ln 17-20'/" of patientswith mediastinalhematoma . symptom-freeperiodof monthsto years
angiodemonstrates acutetraumaticaorticinjury! (i n 11" /"> 10 years)
False negative: . delayedclinicalsymptoms(42%within5 years,857o
0 Smalltransverseintimaltearsmay be missed! within20 years): chestpain,backpain,dyspnea,
r/ resistancein advancingguide wire cough,hoarseness, dysphagia,systolicmurmur
r/ intimatirregularity,lineardefect,fillingdefect Location: descendingaortaat levelof lig. arteriosum
= intimalflap = posttraumatic dissection(5-10%) fillingthe aorticopulmonary window(most
^/ intramuralinjury: commonly)
ri thickeningof aorticwall r/ well-defined roundedmass in leftparamediastinal
r/ posttraumaticcoarctation region
r/ transmurallaceration: ./ + inferiordisplacement of leftmainstembronchus
^/ containedextravasation= traumaticfalse Cx: CHF, partialobstructionof aorticlumen,bacterial
aneurysm endocarditis,aortoesophageal fistula,aortic
i/ free extravasation= aorticrupture dissection,obstruction of tracheobronchial tree,
DDx: ductusdiverticulum (in 10'/. of normals),aortic systemicemboli
spindle,infundibula of brachiocephalic arterial Prognosis; enlargement+ eventualrupture;
branches;volumeaveragingwith leftbrachio- 1O-yearsurvivalrate: 85% with surgicalrepair,
cephalicvein / left superiorintercostalvein / 66% withoutsurgicalrepair
rightbronchialarteries(vs.intimalflap);artifact
from physiologic streaming/ mixingof contrast C A R D IA C FIB R OMA
material;atherosclerotic aorticulceration; = FIBROMATOSIS = FIBROUS HAMARTOMA
atheromatous plaque;syphiliticaorticaneurysm = FIBROELASTIC HAMARTOMA
= collg€hitalneoplasm / hamartomaof the heart
Recommendations for work-up : lncidence; 100 cases reported; 2nd most common
(1) Normalwell-defined mediastinal contourson CXR: benigncardiacneoplasmof childhood(after
no furtherimaging rhabdomyoma)
(2) Unstablepatient+ unequivocally abnormalCXR / Age: 0-56 years(meanage, 13 years);33% in children
strongclinicalevidenceof aorticinjury: <1 year of age / in utero;15/"in adolescents
angiography / emergencysurgery + adults
(3) Stablepatient lncreasedprevalencein: Gorlin (= [ssal cell nevus)
(a) with unequivocally
abnormalCXR: syndrome
CT of chest+ head + abdomen(whilewaiting . heartfailure,cardiacmurmur(33%),arrhythmia
for angiographic
examination) . NO embolism;asymptomatic (33%)

Path: 2-1O-cmlargesingleroundbulgingwell-
circumscribed tumorwithinventricularmyocardium;
foci of calcification/ ossification(50%)
Histo: collectionof fibroblastsinterspersedamong
largeamountsof collagen;numerouselastic
fibers (>50%);NO foci of cysticchange/
hemorrhage/ necrosis
Location: ventricularseptum> left ventricularfree wall
r/ cardiomegaly
r/ focal cardiacbulge (withtumor in free ventricularwall)
r/ t pericardialeffusion
E CHO :
r/ noncontractile echogenicheterogeneoussolid mass:
./ mean diameter"5 cm; may obliteratecardiac
chamber
{ multifocaldystrophiccentraltumor calcifications
{ affectedmyocardiumhypokinetic
DDx: focal hypertrophiccardiomyopathy,hypertrophy
of ventricularseptum
CT:
./ homogeneousmural mass of soft-issueattenuation
{ sharplymarginated/ infiltrative
{ calcifications(25%)
{ variableenhancement
MR:
./ iso-/ hyperintensehomogeneousdiscretemural
, mass/ myocardialthickeningon Tl Wl
r/ hypointenseon T2Wl
r/ no / littlehetero-or homogeneousenhancement
Prognosis:
(1) suddendeath(dueto invasion/ compression of
cardiacconductionsystemresultingin arrhythmia)
0 2nd most commonprimary cardiactumor
associatedwith suddendeath (afterendodermal
heterotopiaof AV node)
(2) may remainstablein size for years/ regress
Rx: surgicalexcision/ partialresection
DDx in infants: rhabdomyoma(multiplemasses)
DDx in children: rhabdomyosarcoma(no calcification,
cysticor necrotictumor,invasionof
pulmonaryveinsor pericardialspace)
CARDIACHEMANGIOMA
= fore benignvasculartumor of the heart
Prevalence: 5-10% of benigncardiactumors
Association: Kasabach-Merritt syndrome(multiple
systemichemangiomas,recurrent
thrombocytopenia, consumptive
coagulopathy)
Path: predominantlyintramuralspongymass / well_
circumscribedendocardial-based soft mass
growinginto pericardialspace;may containfat
Histo: capillary(= smallercapillary-like
vessels);
cavernous(= multiplethin-walleddilated
vessels);arteriovenous( = thick-walled
dysplasticarteries+ veins + capillaries)
. asymptomatic
. dyspneaon exertion,chestpain,right-sided
CHF
. arrhythmia,syncope,pericarditis,sudden
death
Disorders 6l g
Cardiovascular
{ + pericardialeffusion
US:
./ hyperechoicmass
CT:
./ heterogeneousintenselyenhancingmass
MR:
r/ intermediateintensityon Tl wl + hyperintenseon T2wl
A ngi o:
^/ vascularblush in capillary+ arteriovenoustype
r/ no enhancementfor cavernoustype
Prognosis; spontaneousregressionpossible
Rx: surgicalresection(for symptomaticlesion)
CARDIAC LIPOMA
= vsry rare benignneoplasm
lncidence; 60 reportedcases
Age: typicallyin adults
. mostlysymptomatic
. dyspnea(in intracavitarylipomasecondaryto bloodflow
obstruction,in pericardialfipomasecondaryto
displacement of lung)
. arrhythmia(involvementof conductionsystem)
Path: encapsulatedspherical/ ellipticalsolitarymass,
oftenvery large (up to 4,800 g) by the time the
cometo clinicalattention;multiplelipomasin CHD,
tuberoussclerosis
Histo: matureadipocytessurroundedby capsule
Location:
(a) broad-basedfrom epicardialsurfacegrowinginto
pericardialspace
(b) broad-basedfrom endocardialsurfacegrowinginto
cardiacchamber
(c) interatrialseptum
r/ cardiomegaly,globular-shaped heart
{ echogenic/ hypoechoicbroad-basednonmobilemass
r/ roundmass with smoothcontour
r/ homogeneous massof s -50 HU in cardiacchamberi
pericardialspace
{ homogeneousmass of increasedsignalintensity
+ a few thin septationson Tl Wi:
{ decreasingintensitywith fat saturation
r/ no enhancement
Rx: surgical resection
DDx: lipomatoushypertrophyof interatrialseptum
(infiltrative,
at levelof fossa ovaliswith sparingof
fossaovalis,>2 cm thickin transversedimension,
composedof brownfat, not a true neoplasm,
associatedwith advancedage + obesity)
CARDIACPARAGANGLIOMA
= extremelyrare,usuallybenignsporadicneoplasm
arisingfrom intrinsiccardiacsympatheticparaganglial
(chromaffin)cells
lncidence; <50 cases
Age: 18-85 (mean,40)years
. catecholamine-producing tumor(in the majority):
. headache,arterialhypertension,
palpitations,
flushing
. elevatedlevels of urinarynorepinephrine,
vanillylmandelicacid,total metanephrine
. elevatedlevelsof plasmanorepinephrine, epinephrine
620 Radiology Review Manual

Associated with: r/ pleuraleffusion,pericardial

effusion
(a) additionalparagangliomas(in 20%) in carotidbody, ./ focal cardiacmass
adrenalgland,bladder,paraaortic r/ pulmonaryconsolidation
(b) metastasesto bone (in 5%) Metastaticto: lung,lymphnodes,bone,liver,brain,
Path: 2-14 cm largeencapsulated/ poorlycircumscribed bowel,spleen,adrenalgland,pleura,
and infiltrativehighlyvascularmass;necroticin 60% diaphragm,kidney,thyroid,skin
Histo: monomorphictumor composedof nestsof Prognosisj mean survivalof 3 monthto 1 year
paraganglial cells(= "Zellballen")
surroundedby
sustentacular cells Angiosarcoma(37%)
Frequency; most commoncardiacsarcoma
Location: posteriorwall of left atrium> roof of left atrium Age: typicallyin middle-aged men
> atrialcavity> interatrialseptum> ventricle Path: frequentlyhemorrhagic+ necroticmass,often
Site : epicardialsurfaceof the base of the heartwith adherentto pericardium
tendencyto involvecoronaryarteries Histo: endothelial cellsliningill-definedvascular
CX R: spaces
r/ middlemediastinal mass splayingcarinasimulating . right-sided heartfailure,tamponade
left atrialenlargement(for typicallylocatedtumor) . fever,weightloss
E CHO : . bloodyfluidon pericardiocentesis (rarelywith
./ largeechogenicleft atrialmass malignantcells)
{ compressionof SVC, encasementof coronaryaa. Metastasesat presentation:in 66-89%
DDx: myxoma(broadbase of attachment,softer) Location: rightatrialfree wall + involvement of
NUC ( l- 131or t - 1 2 3MIB G ): pericardium (80%)
./ for total body imagingwith a sensitivityof 90% (a) well-defined massprotrudingintoa cardiacchamber
N E CT : { usuallyoriginating from rightatriumwith sparingof
r/ circumscribed heterogeneous
/ ill-defined mass: atrialseptum
r/ hypoattenuating ./ areasof centralnecrosiscommunicatingwith
{ isoattenuating to cardiacstructures(may be cardiacchamber
missed) r/ low-attenuation mass on CT
{ + tumor calcifications ./ heterogeneous contrastenhancement
r/ + extracardiacextension ./ heterogeneous MR signal:
CECT: { "cauliflowerappearance"= local nodular
0 Premedicatepatientwith alpha-and beta-blockersas hyperintenseareas interspersedwithinareas of
contrastmaterialcan triggera hypertensivecrisis! intermediate signalintensityon TlWl + T2Wl
ri markedlyenhancingmassadherentto / involvingleft (b) diffuselyinfiltrativemass extendingalong epicardial
atrium/ anteriorto aorticroot surface
{ centralarea of low attenuation(in 50%) from necrosis r/ obliteratedpericardialspace (hemorrhage
MR + necrotictumordebris)
{ massiso-/ hypointense to myocardiumon TlWl r/ "sunrayappearance"= linearcontrast
./ very hyperintensemass on T2Wl enhancement alongvascularlakeson MR
^/ intenseoftenheterogeneous enhancement Prognosis: 12-30 months survival

CARDIAC SARCOMAS Undifferentiated Sarcoma (24%)

0 Majorityof primarymalignantcardiacneoplasms! = PLEOMORPHIC SARCOMA = ROUNDCELLSARCOMA
0 2nd most commonprimarycardiacneoplasm = S P IN D LE
C E LLS A R C OMA
Mean age: 41 years;extremelyrare in infants+ children Age: 45 years (neonatesto elderlY)
(a) right-sidedheart inflowobstruction ' pulmonarycongestion
1. Angiosarcoma (37"/"): tumorin rightatrium Location: leftatrium
(b) mitralvalve obstruction(tumorin left atrium) r/ large irregularhypodenseintracavitarymass
2. Undifferentiated sarcoma(24%) r/ polypoidmass isointenseto myocardium
3. Malignantfibroushistiocytoma(11-24%) r/ thickening/ irregularity of myocardium(dueto tumor
4. Leiomyosarcoma(8-9%): tends to invade infiltration)
pulmonaryveins+ mitralvalve { tendencyto involvevalves
Age: 5-10 years earlierthan other sarcomas r/ hemorrhagic mass replacingthe pericardium(similar
5. Primarycardiacosteogenicsarcoma(3-9%) to angiosarcoma)
DDx: myxoma(at fossa ovalis)
. dyspnea,pericardial tamponade,arrhythmia, syncope,
peripheraledema,suddendeath CARDIAC TAMPONADE
. embolicphenomena,chestpain,pneumonia,fever = significant
compression of heartby fluidcontainedwithin
{ cardiomegaly sac resultingin impaireddiastolicfillingof
pericardial
./ cHp ventricles
 Cardiovascular
Disorders OZI

Cause: see PERTCARDTAL EFFUSTON . tendencyfor CHF when EF <40./"

. tachycardia
r/ global4-chamberenlargement
. pulsusparadoxus= exoggerationof normalpattern
{ poor ventricularcontraciility
= drop in systolicarterialpressure>10 mm Hg during
inspiration(secondaryto increasein rightheartfilling 1 fn enlargementwithouteniargementof LA appendage
^/ bilateralatrioventricular
valveJnsufficiency
duringinspiration at the expenseof left heartfilling) E C H O:
' elevatedcentrafvenouspressurewith distendedneck
r/ enlarged LV with global hypokinesis
veins { IVS and LVPW of equalthicknesswith decreased
. fallingbloodpressure
amplitudeof motion
. distantheartsoundsi frictionrub
^/ low-profile/ "miniaturized"
mitralvalve
. ECG: reducedvoltage,ST elevation,pR depression,
r/ mildlyenlargedLA (elevatedend-diastolic LV
nonspecificT-waveabnormalities pressure)
r/ normallungfields+ normalpulmonaryvascularity r/ enlargedhypokineticrightventricle
r/ rapidenlargementof heartsize
r/ distensionof SVC, lVC, hepatic+ renalveins
r/ periportaledema Hypertrophic Cardiomyopathy
r/ hepatomegaly = OBSTRUCTIVE CARDIOMYOPATHY
Doppler-US: = characterizedby nondilatedhypertrophyof left
r/ episodesof high-velocityhepatopetalflow separated ventriclein the absenceof cardiac/ systemicdisease
by longintervalsof minimalflow that would cause LV hypertrophy
ECHO : 1. S Y MME TR IC / C ON C E N TR IC
H Y P E R TRO PHY
r/ diastoliccollapseof RV (2-20%)
r/ cyclicalcollapseof eitheratrium (a) midventricular
Rx: pericardiocentesis/pericardial drainage (b) diffuse
(c) apical
2. ASYMMETRTC SEPTALHypERTROpHy(ASH)
CARDIAC
THROMBUS = IDIOPATHIC HYPERTROPHIC SUBAORTIC
A. Left AtrialThrombus STENOSIS (IHSS)= SUBAORTIC STENOSIS
Associatedwith: mitralvalve disease = HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY
. atrialfibrillation = basal septumof LV disproportionately thickened
Site: atrialappendage 3. APTCALHYPERTROPHY (2_3%)
! atrialdilatation = rnyocordialwall thickeningconfinedto apical
r/ irregular/ lobulatedborder portionof LV
r/ microcavitations . usuallyclinicallybenign
r/ laminatedappearance . giant invertedT wave
B. LeftVentricular Thrombus Left ventriculography:
Site: regionof ventricular dyskinesia/ aneurysm { spade-shapeddeformityof LV cavity
(fromprior myocardialinfarction) Hemodynamics:
r/ homogeneousattenuationon CT - LV hypertrophyleadsto subaorticstenosis,
! heterogeneous signalon SE MR images abnormaldiastolicfunction,myocardialischemia
r/ low-signalintensityon GRE MR images - rapidbloodflow throughnarrowoutflowtract
DDx: myxoma (heterogeneoustexture on CT) causesthe anteriorleafletof mitralvalve to
displaceanteriorlytowardseptumduringsystole
CARDIOMYOPATHY (Venturieffect)
- mitralregurgitation(fromdisplacedMV leaflet)
Dilatedand lschemicCardiomyopathy
= CONGESTIVE CARDIOMYOPATHY Etiology: autosomaldominanttransmission
Etiology: . exertionalangina+ dyspnea,fatigue
1. ldiopat hic . syncope,arrhythmia, suddendeath
2. Myocarditis:viruses,bacteria ri normalheartsize
3. Alcoholism
4. Pregnancy/ post partum 1 m enlargement with mitralinsufficiency(in 30%)
r/ prominentleft midheartborder(septalhypertrophy)
5. Endocardial fibroelastosis= thickened r/ t mitOpulmonaryvenoushypertension
endocardium+ reducedcontractility ECHO (modalityof choice):
6. Infantsof diabeticmothers ^/ tVS >14 mm thick;posterolateral wall >11 mm
7. lnbornerrorof metabolism:glycogenosis, thi ck;IV S :LV P Wthi ckness> 1.3:1
mucolipidosis,mucopolysaccharidosis r/ systolicanteriormotionof mitralvalve (SAM)
8. Coronaryarterydisease: myocardialinfarction,
anomalousoriginof left coronaryarlery,coronary , causingnarrowedLVOT in systole
! midsystolicclosureof aorticvalve
calcinosis ^/ increasedLVOT gradientwith late systolicpeaking
L Musculardystrophies on Doppler
622 Radiology Review Manual

A. LOCAL]ZEDCOARCTATION
= ADULT TYPE[former
/ JUXTADUCTAL
/ POSTDUCTAL
classification
l
= shortdiscretenarrowingcloseto ligamentum
arteriosum(mostcommontyPe)
0 Coexistentcardiacanomaliesuncommonl
Location: most frequentin juxtaductalportionof arch
. incidentalfindinglatein life
. ductususuallyclosed
SystolicAnterior Motion (SAM) of MV in IHSS r/ shelflikelesionat any point alongthe aorticarch
mitral valve leaflets move abruptly toward septum at a rate greater r/ narrowisthmusabovethe lesion
than the endocardium of the posterior wall; responsible for { poststenoticaorticdilatationdistally
obstruction to blood ejected from LV (for abbreviations seepage B. TUBULARHYPOPLASIA
= INFANTILE / PREDUCTAL i DIFFUSE TYPE[former
s94)
classificationl
= hypoplasiaof long segmentof aorticarch afterorigin
RestrictiveCardiomyopathy of innominateartery
Etiology: (a) idiopathic:endomyocardialfibroelastosis 0 Coexistentcardiacanomaliescommon!
(a) infiltrative
disease:amyloidosis, . CHF in neonatalperiod(in 50%)
glycogen,hemochromatosis, sarcoidosis
{ patentductusarteriosus
(b) constrictivepericarditis
Hemodynamics:
{ varyingdegreesof pulmonaryvenous hypertension fetus : no significantchangebecauseonly 10/" oI
r/ t LR enlargement cardiacoutputflowsthroughaorticisthmus
neonate:determinedby how rapidlythe ductuscloses;
VENOUSSTASISDISEASE
CHRONIC withoutconcurrentVSD overloadof LV leads
= CHRO NIV IN S U F F IC IEN C Y
CE N O U S to CHF in 2nd / 3rd week of life
= insufficiency lincompetenceof venousvalvesin deep Collateralcirculation: via subclavianartery and its
venoussystemof lower extremity branches:
Cause: - intercostals - internal
mammary
(a) postphlebiticvalvularincompetence:destructionof - anteriorspinalartery - scapular
artery
valve apparatusresultsin shortthickenedvalves - lateralthoracic - transverse
cervicalartery
secondaryto scar formation Associatedin 50% with:
(b) primaryvalvularincompetence:shallowelongated 1. Bicuspidaorticvalve (in 25-50%),which may result
redundantvalve cusps preventeffectiveclosure in calcificaorticvalve stenosis(after25 years of age)
Associatedwith: incompetentvenousvalvesin the calf + bacterialendocarditis
(secondaryto pressuredilatationfrom 2. Intracardiac malformations:
stasisin deep venoussystem)leading PDA (33%),VSD (15%),aorticstenosis,aortic
to superficialvein varicosities insufficiency,ASD,TGV, ostiumprimumdefect,
. edema,induration(= fluidexudationfrom increased
truncusarteriosus, double-outletrightventricle
capillarypressure) 3. Noncardiacmalformations(13%|
. ulceration(fromminortrauma+ decreaseddiffusionof
Turnersyndrome(13-1 5%)
oxygensecondaryto fibrindepositsaroundcapillaries) 4. Cerebralberryaneurysms
. skin hyperpigmentation (= breakdownproductsof 5. Mycoticaneurysmdistalto CoA
exudatedRBCs) Prognosis; 11% mortalitypriorto 6 monthsof age
., achingpain
Rx: ages 3-5 years are idealtime for operation(late
r/ venous refluxon descendingvenographywith Valsalva enoughto avoid restenosis+ early enoughbefore
(a\ 82% in deep venoussystemalone irreversiblehypertensionoccurs);surgical
(b) 2% in saphenousvein alone correctionpast 1 year of age decreasesoperative
(c) 16h in both mortalitydrastically;3-1 1% perioperativemortality
in75 /"
bilat er al
Grade:
1 = minimalincompetence = to levelof upperthigh
2 = mild incompetence = to levelof lowerthigh
3 = ffioderateincompetence= to levelof knee
4 = s€vete incompetence = to levelof calfveins

COARCTATION OF AORTA
= localizedobstructionat the junctionof aorticarch and
descendingaorta secondaryto a fibrousridge
protrudingintoaorticlumen
Localized Coarctation Tirbular Hypoplasia
M : F = 4: 1: r ar ei n B l a c k s

Procedures:
1. Resection+ end-to-endanastomosis
2. Patchangioplasty
3. Subclavianflap (Waldhausen procedure)usingleft
subclavianarteryas a flap
Postsurgical Cx:
1. Residualcoarctation(in 32%)
2. Subsequentobstruction(rare)
3. Mesentericarteritis:2-3 days after surgery
secondaryto paradoxicalhypertensionfrom
increasedplasmarenin
. abdominalpain,lossof bowelcontrol
4. Chronicpersistenthypertension
SymptomaticCoA CONGESTIVE HEARTFAILURE
0 second
mostcommon
cause
ofcHFin neonate
(after = increasein circulating
hypoplasticleft heart)
Time: (a) towardthe end of 1st week of life in ,,critical
stenosis"
(b) morecommonlypresentsin olderchild
. lowerextremitycyanosis(in tubularhypoplasia)
' leftventricularfailure(usuallytowardend of 1stweek
of life)
r/ generalizedcardiomegaly
r/ increasedpulmonaryvascularity(L-to-Rshunt
throughPDA / VSD)
{ pulmonaryvenoushypertension/ edema
{ "figure3 sign"hiddenby thymus
AsymptomaticGoA
. headaches(fromhypertension)
. claudication
(fromhypoperfusion)
^/ "figure3 sign" = indentationof left lateralmarginof
aorticarch in the regionof aortic-pulmonic window(at
site of coarctationand poststenoticdilatation)
{ "reverse3 sign"on bariumesophagram
r/ elevatedleft ventricularapex (secondaryto left
, ventricularhypertrophy)
r/ scallopedcontouringof soft-tissuesposteriorto
sternum(= dilatedtortuousinternalmammary
, arteries)on LAT CXR (in 2g%)
r/ dilatationof brachiocephalic vessels+ aorta proximal
to stenosis
r/ obscurationof superiormarginof aorticarch
r/ inferiorrib notching (in 7s%l mostlyin adultsover age
20; unusualbeforeage 6)
Location: ribs 3-9 (mostpronouncedin 3rd + 4th
ribs,lesspronouncedin lowerribs);1st
+ 2nd rib do not participatebecausethey
havearteriesoriginatingfromsubclaviana.
Site: central+ lateralthirdsof posteriorrib
(a) bilateral
(b) unilateralon left side: left aorticarch with
aberrantrightsubclavianarterybelow CoA
(c) unilateralon rightside: rightaorticarch
with anomalousleft subclavianarterybelow
CoA
Cardiovascular Disorders 623
CONGENITAL
ABSENCEOF PULMONARY
VALVE
Massiveregurgitationbetweenpulmonaryarteryand RV
ln 90% associatedwith: VSD, tetralogyof Fallot (50%)
. cyanosis(not in immediatenewbornperiod)
. repeatedepisodesof respiratorydistress
. conti nuous murmur
. ECG: rightventricularhypertrophy
r/ prominentmain,right,and leftpulmonaryartery
./ nV dilatation(increasedstrokevolume)
r/ partialobstructionof right/ left mainstembronchus
(compression
by vessel)
r/ right-sidedaorta (33%)
bloodvolumewith diminishing
cardiacfunctionleadsto elevationof microvascular
pressureof lung
lncidence; most commoncause of interstitial+ airspace
edemaof fungs
Cause:
(a) back pressurefrom LV: long-standing systemic
hypertension,aorticvalve disease,coronaryartery
disease,cardiomyopathy, myocardiafinfarction
(b) obstructionproximalto LV: mitralvalve disease.LA
myxoma,cor triatriatum
Histo:
(a) Interstitial
phase: fluidin looseconnectivetissue
aroundconductingairwaysand vessefs
+ engorgement of lymphatics
(b) Alveolarphase: increasein alveolarwall thickness
(c) Alveolarairspacephase: alveolifilledwith fluid
+ lossof alveolarvolume;pulmonaryfibrosisupon
organizationof intra-alveolarfibrin(if chronic)
,
./ large heart
1. Interstitial pulmonary edema (invariablyprecedes
alveolaredema)
. NO abnormalphysicalfinding
., hypoxemia(ventilation-perfusion inequality)
^/ lossof sharpdefinitionof vascularmarkings
r/ thickeningof interlobular septa(pulmonarfuenor.
, wedge pressure17-20 mm Hg)
! poorlydefinedincreasedbronchialwall thickness
r/ thickeningof interlobarfissures(dueto fluidin
subpleuralconnectivetissuelayer)
2. Airspace edema
Cause: acute LA pressureelevationwith volumeof
capillaryfiltrationexceedingthat of lymphatic
drainage
. severedyspnea/ orthopnea
. tachypnea+ cyanosis
. dry cough/ copiousfrothysputum
. hypoxemia(vascularshunting)
r/ poorlydefinedpatchyacinaropacities
ri coalescenceof acinarconsolidation, particularly
in
medialthirdof lung
r/ butterftyi bat-wingdistributionof consolidation
(= consolidatedhilum+ uninvolvedlungcortex)
624 Radiology Review Manual

3. Flow inversion { normalpulmonaryvascularity / pulmonaryvenous

Cause: chronicelevationof LA pressure(as in left hypertension(43%)
heartfailure/ mitralvalve disease) { straightening of heartborders:
Pathophysiology: { straighti concaveon rightside
long-standingelevationof LA pressurecausesan r/ squaredon left side
increasein atriovenousreflux;initiallythe increased ./ pericardialtenting
LA pressureis met with an increasedtonus of the LA r/ increasein ejectionfraction(smallEDV)
wall (= absenceof atrialenlargementin acute left r/ pleuraleffusion(34%bilateral,26% rightPE)
heartfailure);eventuallyLA enlargesincitinga CT:
protectiveatrial-puImonary-vascu lar reflex { epicardium= visceralpericardium >2 mm thick
vasospasm,which narrowsthe lower lobe vessels ^i dilatationof SVC + IVC
and decreasesatriovenousreflux { refluxof contrastintocoronarysinus
r/ basaloligemia ri flatteningof rightventricle+ curvatureof
r/ hyperemiaof upper lobes interventricular septumtoward left
N.B.: flow inversionis neverseen in pulmonary r/ pleuraleffusion+ ascites
edema of renalfailure/ overhydration/ low ECHO (nonspecific :
features)
oncoticpressure r/ thickeningof pericardium
4. Generalizedoligemia r/ rapidearlyfillingmotionfollowedby flat posteriorwall
Cause: aorticvalvulardisease motionduringdiastasisperiod1=periodbetween
early rapidfillingand atrialcontraction)
Cx: protein-losing enteropathy(increasedpressurein
IVC + portalvein)
PERICARDITIS
CONSTRICTIVE DDx: cardiactamponade,restrictivecardiomyopathy
= fibrousthickeningof pericardium interferingwith fillingof (eg,amyloid)
ventricularchambersthroughrestriction of heartmotion
Age: 30-50 years; M:F = 3:1
Etiology: ARTERYFISTULA
CORONARY
A . I DI O P A T H IC = single/ multiplefistulousconnectionsbetweena
B. INFECTIOUS / INFLAMMATORY coronaryartery(R t L) and other heartstructures
1. Viral(CoxsackieB) Abnormal communicationwith (>90% right heart):
2. Tuberculosis (formerlymostcommon) RV > RA > pulmonarytrunk> coronarysinus> SVC
3. Rheumatoidarthritis Hemodynamics: L{o-Rshunt; pulmonary:systemic
C. TRAUMATIC bl oodfl ow= < 1.5:1(usually)
1. Cardiacsurgery(mostcommon) { may have normalCXR (in smallshunts)
2. Radiotherapy to mediastinum { cardiomegaly + shuntvascularity(in largeshunts)
D. URE M I A= c h ro n i cre n a lfa i l u re A ngi o:
E. NEOPLASTIC= tumorinvasion r/ ditatedtortuouscoronarya. with anomalousconnection
Causes of acute pericarditis:
mnemonic.' "MUSlC" COR TRIATRIATUM
Myocardialinfarction(acute) = fdre congenitalanomalyin whicha fibromuscular
Uremia septumwith a singlestenotic/ fenestrated/ large
Surgery(cardiac) openingseparatesthe embryologic commonpulmonary
Infection vein fromthe leftatrium:
Cancer (1) proximal/ accessorychamberliesposteriorly
. dyspnea receivingpulmonaryveins
. abdominalenlargement(ascites+ hepatomegaly) (2) distal/ true atrialchamberlies anteriorlyconnected
. peripheraledema to left atrialappendage+ emptyinginto LV through
. pericardialknocksound= loudearly-diastolic sound mitralvalve
. neckvein distension Etiology: failureof commonpulmonaryvein to
. Kussmaulsign = failureof venouspressureto fall with incorporatenormallyinto left atrium
inspiration Associatedwith: ASD, PDA, anomalouspulmonary
. prominentX and Y descenton venouspressurecurve venousdrainage,leftSVC,VSD,
r/ linear/ plaquelikepericardialcalcifications(50-70/"): canal
tetralogyof Fallot,atrioventricular
predominantlyover RV, posteriorsurfaceof LV, in . dyspnea,heartfailure,failureto thrive
atrioventricu lar groove . clinicallysimilarto mitralvalvestenosis
r/ dilatationof SVC (77"/"),azygosvein (69%) r/ pulmonaryvenousdistention+ interstitial edema
{ smallatria;occasionally compensatory dilatationof + dilatationof pulmonarytrunkand pulmonaryarteries
nonconstricted portions,eg, LA enlargement(20%) (in severeobstruction)
r/ normal/ small-sizedheart(enlargement onlydue to r/ enlargedRA + RV
preexisting disease) { mildenlargement of LA

Angio:
^/ dividingmembraneon levophaseof pulmonary
arteriogram
Prognosis (if untreated):
usuallyfatal withinfirst 2 years of life;S0o/o2-year
survival;20h 2}-year survival
Rx: surgicalexcisionof obstructingmembrane
DEEPVEINTHROMBOSIS
= DVT
lncidence; 140,000-250,000new cases per year in
UnitedStateswith an estimatedsole / major
cause of 50,000-200,000deaths per year
(15%of in-hospitaldeaths);0-Z millionstasis
skin changes;in 05% causeof skin ulcers
Pathogenetic factors:
1. Hypercoagulability
2. Decreasedbloodflow / stasis
3. Intimalinjury
4. Decreasedfibrinolyticpotentialof veins
5. Plateletaggregation
Risk factors:
1. Surgery,esp.on legs/ pelvis: orthopedic(45-50%)
especiallytotalhip replacement >50%),gynecologic
(7-35/"), neurosurgery(18-20./"),urologic
(15-35%), generalsurgery(20-25%)
2. Severetrauma
3. Prolongedimmobilization: hemiplegicextremity,
paraplegia+ quadriplegia,casting/ orthopedic
appliances
4 . Malignancy(riskfactor2.5) = Trousseausyndrome
5 . Obesity(riskfactor1.5)
6 . Diabetes
7 . Pregnancy(riskfactor5.5) and for B-12 weeks
postpartum
8. Medication:birthcontrolpills,estrogen
replacement, tamoxifen(riskfactor3.2)
9. Decreasedcardiacfunction: congestiveheart
failure,myocardialinfarction(20-50%; risk factor
3. 5)
10. Age >40 years(riskfactor2.2)
11. Varicoseveins
12. PreviousDVT (riskfactorZ.S)
13. Patientswith bloodgroupA > bloodgroup0
14. Polycythemia
15. S m ok ing
Pathologic terminology :
brganizedthrombus"= transitionto a vascularized
lesionof connectivetissueadherentto vesselwall
"recanalized
thrombus"= vascularchannelnetwork
withinan organizedclot reducingit to septationsof
collagenand elasticfibersoftenlinedby endothelium
Location:
1. Dorsalveinsof calf (+ ascendingthrombosis)
2. lliofemoral
veins(+ descendingthrombosis)
3. Peripheral+ iliofemoralveinssimultaneously
CardiovascuIar Disorders 625
4. rare: internaliliacv., ovarianv., ascendinglumbarw.
L:R = 7:3 due to compression of leftcommoniliacv. by
leftcommoniliaca. (arterialpulsationsleadto chronic
endothelialinjurywith formationof intraluminal spur,
which is presentin 22o/"of autopsies+ in 90% of
patientswith DVT)
. Localsymptomsdue to obstruction / phlebitisusually
onlywhen (a) thrombusocclusive,(b) clot extendsinto
popliteal/moreproximalvein (14-78/" sensitivity,
4-21% specificity):
. warmth
. swelling(measurement of circumference)
. bfanchingof skin (phlegmasiadolensalba)/ blue leg
with completeobstruction(phlegmasia cerulea
dolens)
. deep crampypain in affectedextremity,worse in erect
position,improvedwhilewalking
. tendernessalong courseof affectedvein
. Homanssign = calf painwith dorsalflexionof foot
. Payrsign = pain uponcompression of sole of fool
0 213of deep vein thrombosesare clinicallysilent:
0 DVT diagnosedante mortemin <30%
0 Only 10-33% of patientswith fatal PE are
symptomaticfor DVT
0 ClinicallysuspectedDVT accuratein only 26-45.h:
0 DVT symptomatologydue to other causesin
15-35% of patients
0 Negativebilateralvenogramsin 30% of patientswith
angiographically detectedpulmonaryemboli(bigbang
theory= clot embolizesin toto to the lung leavingno
residualin vein)
Venography(89% sensitivity,97"/"specificity):
falsenegativein 11%,falsepositivein S/.;
study abortedi nondiagnosticin 5%
Risk: postvenography phlebitis(1-2%), contrast
reaction,contrastmaterial-induced skin slough,
nephropathy
r/ intraluminal fillingdefectconstanton all images
ri nonfillingof calfveins
r/ inadequatefillingof commonfemoralvein + external
+ commoniliacveins
B-ModeUS (88-100% sensitivity,92-100% specificity,
>,90V"accuracyfor DVT in thigh and poplitealveins):
! lackof completeluminalcollapsewith venous
compression(DDx:deformity+ scarringfrom prior
DVT;technicaldifficultiesin adductorcanal+ distal
deep femoral vein)
ri visualization
of clotwithinvein (DDx:slow flowing
blood;machinenoise)
",1.lSt" increasein diameterof commonfemoralvein
duringValsalva
r/ venousdiameterat leasttwice that of adjacentartery
suggeststhrombus<10 days old
DopplerUS:
r/ absenceof spontaneity(= any waveformrecording),
not reliablein peripheralveins
626 Radiology Review Manual

r/ continuousvenoussignal= absenceof phasicity Likelihood of pulmonary embolism:

(= no cyclicvariationin flow velocitywith respiration, 77o/ofor iliacveins, 35-67"h for femoropopliteal
ie, decreasein expiration+ increasein inspiration) is vein, 0-46"/ofor calf veins
suspiciousfor proximalobstruction (2) Postphlebitic syndrome(PPS)in 20/" of caseswith
r/ attenuation / absenceof augmentation (= no increase DVT (= recanalization to a smallerlumen,focalwall
in flow velocitywith distalcompression) indicates changes)due to valvularincompetence
venousocclusion/ compressionin intervening venous (3) Phlegmasia ceruleaI albadolens(= severely
segments impairedvenousdrainageresultingin gangrene)
r/ pulsatilevenousflow is a sign of congestiveheart
failurei pericardialeffusion/ cardiactamponade/ Prognosis; tibial/ peronealvenousthrombiresolve
pulmonaryembolismwith pulmonaryhypertension spontaneously in 40o/o,stabilizein 40/o,
propagateintopoplitealvein in 20h
VenousOcclusionPlethysmography : Prophylaxis; intermittentcompressionof legs,heparin,
- 87-95-1 00% sensitivity,92-100% specificityfor warfarin
above-kneeDVT Rx:
- 17-33% sensitivityfor below-kneeDVT (1) H epari nl V
= temporaryobstructionof venousoutflowby pneumatic (2) Systemicanticoagulation (warfarin)for >3 months
cuff aroundmid-thighinflatedabovevenouspressure decreasesriskof recurrentDVT in initial3 months
leadsto progressive increasein bloodvolumein from 50% to 3% + fatal pulmonaryembolismfrom
lower leg; upon releaseof cuff limb quicklyreturnsto 30% to 87"; necessityfor anticoagulation in DVT of
restingvolumewith promptvenousrunoff;limb blood calfveinsis controversial
volumechangesare measuredby impedance (3) Cavalfilter(10-15%)in patientswith
plethysmographyin which a weak alternatingcurrent contraindication / complication from anticoagulation
is passedthroughthe leg;the electricalresistance or progression of DVT / PE despiteadequate
variesinverselywith bloodvolume;the current anticoagulation
strengthis held constantand voltagechangesdirectly DDx: pseudothrombophlebitis (= signs+ symptomsof
reflectbloodvolumechanges DVT producedby poplitealcyst / traumatic
hematoma)
^/ initialrise in venousvolume(= veflouscapacitance)
dim inis he d RIGHTVENTRICLE
DOUBLE.OUTLET
r/ detayin venousoutflow= "fall"measuredat 3 = D OR V= TA U S S IG-B INHG
EART
seconds = rT'roSt
of the aorta + pulmonaryarteryarisefrom the RV
Falsepositives(6%): severecardiopulmonary secondaryto maldevelopment of conotruncus
disease,pelvicmass,reduced Type 1 = ?oft? posteriorto pulmonaryartery+ spiraling
arterialinflow course(mostfrequent)
False negatives: c a l fv e i nth ro m b o s i s.
smal l Type 2 = Taussig-Bingheart= ?ort? posteriorto
th ro mb u s pulmonaryartery+ parallelcourse
Type 3 = aortd anteriorto pulmonaryartery+ parallel
l-125-LabeledFibrinogen: course
- 90% sensitivefor calfveinthrombus Hemodynamics:
- 60-807" sensitivefor femoralvein thrombus fetus : no CHF in utero(in absenceof obstructing
- insensitive for thrombusin upperthigh/ pelvis otheranomalies)
Risk: resultsnot availablefor severaldays, neonate:ventricular work overloadleadsto CHF
transmission of viralinfection Associatedwith: VSD (100%),pulmonarystenosis
Falsepositives: hematoma,inflammation,wound,old (50%),PDA
smallthrombusisolatedin common ./ aorta overridingthe interventricularseptumwith
femoral/ iliacvein predominant connectionto RV
{ aorta posterior/ parallel/ anteriorto pulmonaryarlery
Cx: r/ l-v enlargement(volumeoverload)
(1) Pulmonaryembolism(50%): in 90% from lower
extremity/ pelvis;in 60% with proximal"free-floating" ANEURYSM
DUCTUSARTERIOSUS
/ "widow-maker" thrombus;occursusuallybetween = fusiformaneurysmof ductusarteriosus, usuallypatent
2nd to 4th (7th)day of thrombosis towardaorta+ completely/ incompletely occluded
Source of pulmonary emboli: towardpulmonaryartery
multiplesites(1/3),cryptogenicin 50%; lncidence: <100 cases
(a) lowerextremity (46%) Classification:
(b) inferiorvena cava (19%) (a) accordingto age: infantile,childhood,adulttype
(c) pelvicveins (16%) (b) accordingto causet congenital,infectious,traumatic
(d) muralheartthrombus (4.5%) Pathogenesis:? delay in closure,? myxoiddegeneration
(e)upperextremity (2%) of ductuswall,? abnormalelasticfibers

Age: most <2 monthsof age
. dyspnea,tachypnea,hoarseness
] pulmonaryarterydisplacedanteromedially
! distalaorticarch displacedlaterally
CXR:
^/ tett-sidedupper mediastinalmass in aorticopulmonary
window
r/ trachealdisplacementto right + anteriorlyi posteriorly
{ consolidationof adjacentlJng (compresiion,fibrosis,
hemorrhage)
CT: { contrast-enhancing mass in classiclocation
ECHO: ^/ cystic mass with pulsatileflow
Cx: rupture,dissection,infection,thromboembolic
disease,phrenicnervecompression
Prognosis; usuallyfatal (withoutpromptsurgery)
EBSTEIN ANOMALY
= downwarddisplacementof septal+ posteriorleafletsof
dysplastictricuspidvalve with ventriculardivisioninto
(a) a largesuperioratrializedportion(incorporating
of the RV into the RA) and
(b) a smallinferiorfunctionalchamberwith shortened
chordaetendineae
Etiology: chronic maternallithium intake (10%)
Hemodynamics:
tricuspidvalveinsufficiency leadsto tricuspid
regurgitation("ping-pong"volume)+ severelydilated
RA; RA dilatationstretchesinteratrialseptumcausing
incompetenceof foramenovale (R-to-Lshunt)
RA 1 RV J Main PA +>
Pulm.vessels <+lJ
L A < + L V < + A o €
Associatedwith: PDA,ASD (Rto-L shunt)
. t cyanosisin neonatalperiod(dependingon degree
R-to-Lshunt): may improve/ disappearpostnatallywith
decreasein pulmonaryarterialpressure
. CHF in utero/ in neonate(in SO%)
. systolicmurmur(tricuspidinsufficiency)
. Wolff-Parkinson-White syndrome(1O/") = paroxysmal
supraventricular tachycardia/ rightbundlebranchblock
(responsiblefor suddendeath)
Cause: conductionsystemdevelopsduringformation
of tricuspidvalve adjacentto it
r/ "boxlike/ funnel-like" cardiomegaty (enlargement of RA
,+RV)
r/ extremeRA enlargement(secondaryto insufficient
tricuspidvalve)
{ IVC + azygosdilatation(secondaryto tricuspid
regurgitation)
^/ hypoplasticaorta + pulmonarytrunk (the ONLy cyanotic
CHD to have this feature)
r/ normalLA
r/ calcificationof tricuspidvalve may occur
ECHO :
^/ large"sail-like"tricuspidvalve structurewithindilated
right heart
r/ tricuspidregurgitationidentifiedby Dopplerultrasound
Prognosis: 50/" infant mortality;13% operative
mortality;survivalinto adulthoodif valve
functionsnormally
Cardiovascular
Disorders 027
Rx: 1. Digitalis
+ diuretics
2. Tricuspidvalveprosthesis
EISENMENGERCOMPLEX
= E IS E N ME N GEDE RFE C T
= (1) highVSD t overridingaortawith hypoplastic crista
supraventricularis
(2) RV hypertrophy
and as consequence of increasedpulmonarybloodflow:
(3) dilatationof pulmonaryarlery+ branches
(4) intimalthickening+ sclerosisof smallpulmonary
arteries+ arterioles
. cyanosisappearsin 2nd + 3rd decadewith shunt
reversal
E IS E N ME N GE R S Y N D R OME
= E IS E N ME N GE RA C TION
RE
= development of highpulmonaryvascularresistance
part after many years of increasedpulmonarybloodflow
secondaryto L-to-Rshunt(ASD,pDA, VSD),which
leadsto a bidirectional (= balanced)shuntand ultimately
to R-to-Lshunt
Etiology:
pulmonarymicroscopicvesselsundergoreactive
muscularhypertrophy, endothelial thickening,in situ
thrombosis,tortuosity+ obliteration;once initiated,
pulmonaryhypertensionacceleratesthe vascular
reaction,thus increasingpulmonaryhypertension in a
viciouscycle with RV failure+ death
Path: adaptiveanastomoticpathwaysconnectplexiform
lesionsof pulmonaryarterialvesselsto bronchial
arteriessupplyingterminalbronchioles + vasa
of vasorumof pulmonaryarteries
Pathologic classification of severity (Heath & Edwards):
Grade | - medialhypertrophyof muscular
pulmonaryarteriesand arterioles
. potentiallyreversible
Grade ll = grade| + intimalproliferation in small
musculararteriesand arterioles
. potentiallyreversible
Grade lll = grade ll + intimallaminarfibrosis
+ progressivevesselobliteration
. borderlinefor reversibility
Grade lV = occlusionof vesselswith progressive
aneurysmaldilatationof small arteries
nearby
. irreversible
Grade V = tortuous"glomeruloid" channelswithin
proliferation of endothelialcells
(= plexiform+ angiomatoidlesions)
. irreversible
Grade Vf = thrombosis+ necrotizingarteritis
. irreversible
CXR:
./ pronounceddilatationof centralpulmonaryarteries
(pulmonarytrunk,main pulmonaryarlery,
intermediatebranches)
{ pruningof peripheralpulmonaryarteries
628 Radiology Review Manual

r/ enlargementof RV + RA (proportionate to volume Type 1 = chordaetendineaeof anteriorbridging

overload) leafletattachedto both sides of
./ m + LV returnto normalsize (withdecreaseof L-to-R ventricularsePtum
shuntdue to markedlyelevatedpulmonaryvascular Type 2 = chordaetendineaeof anteriorleaflet
resistance) attachedmediallyto anomalouspapillary
^/ normalpulmonaryveins (unlesssuperimposed musclewithinRV, but unattachedto
cardiacvolumeoverload): sePtum
r/ pulmonaryveinsNOT distended(NO increasein Type 3 = free-floatinganteriorleafletwith chordae
pulmonary bloodflow) attachmentsto sePtum;onlYtYPe
of pulmonaryveins (normal
r/ lttOredistribution becomingsYmPtomatic in infancY!
venouspressure) Associated with:
CT : (1) Downsyndrome:
r/ linearcalcification + thrombusin centralpulmonary in 25!. of trisomy21 an ECD is present;
arteries in 45'h of ECD trisomY21 is Present
r/ muralcalcification/ aneurysmaldilatationof ductus (2) Asplenia,PolYsPlenia
arteriosus(in cases of patentductusarteriosus) ri commonatrioventricular orifice
Dx: measurementof pulmonaryarterypressure+ flow r/ oval septaldefectconsistingof a low ASD + high
via catheter VSD
r/ atrialseptumsecundumusuallyspared("common
atrium"if absent)
DEFECT
CUSHION
ENDOCARDIAL { frequentlyassociatedwith mesocardia/ dextrocardia
= ECD= ATRIOVENTRICULAR SEPTAL DEFECT Hemodynamics:
= PERSISTENT OSTIUMATRIOVENTRICULARE UNE
COMM fetus : atrioventricular valvesfrequently
= PERSISTENT COMMON ATRIOVENTRICULAR CANAL incompetent leadingto regurgitation+ CHF
= persistenceof primitiveatrioventricular
canal neonate: L-to-Rshuntafterdecreaseof pulmonary
+ anomaliesof AV valves vascularresistanceresultingin pulmonary
hYPertension
A . I NCO M P LE T E IP AR T IALEC D . incompleterightbundlebranchblock(distortion of
= ( 1) O s t iu mp ri m u mAS D conductiontissue)
(2) Cleft in anteriormitralvalve leaflet/ trileaflet . left-anterior hemiblock
(3) Accessoryshort chordaetendineaearisingfrom CXR:
anteriorMV leafletinsertdirectlyinto crest of 0 Radiographic findingssimilarto ASD, but more
deficientventricularsePtum marked
^/ lett atrioventricular valve usuallyhas 3 leafletswith a ./ increasedpulmonaryvascularity(= shunt vascularity)
wide cleft betweenanterior+ septalleaflet r/ redistribution of pulmonarybloodflow (mitral
r/ "gooseneck"deformitysecondaryto downward regurgitation)
attachmentof anteriorMV leafletcloseto ! enlargedpulmonarY arterY
interventricular septumby accessorychordae r/ diminutiveaorta (secondaryto Lto-R shunt)
tendineae { cardiacenlargementout of proportionto pulmonary
^/ communicationbetweenLA-RA or LV-RA, vascularity(L-to-Rshunt+ mitralinsufficiency)
occasionallYLV-RV r/ enlargedRV + LV
{ rightatrioventricular valve usuallynormal ^/ enlargedRA (LV blood shuntedto RA)
r/ normal-sizedLA (secondaryto ASD)
B . T RA NS I T IO N AL / IN T E R ME D IA T E E C H O:
ATRIOVENTRICULAR CANAL(uncommon) ! visualization of ASD + VSD + valve+ site of insertion
= ( 1) O s t iu mp ri m u mA SD of chordae tendineae
(2) HighmembranousVSD r/ paradoxicalanteriorseptal motion(secondaryto
(3) Wide cleftsin septal leafletsof both AV valves ASD)
(4) Bridgingtissuebetweenanterior+ posterior r/ atrioventricular insufficiency+ shuntsidentifiedby
commonleafletof both AV valves Dopplerultrasound
A ngi o:
C. CO M P LE TEEC D = A V C OMMU N IS= C O MMONA V AP projection:
CANAL { gooseneckdeformityof LVOT (in diastole)
= (1) OstiumprimumASD above r/ cleft in anteriorleafletof mitralvalve (in systole)
(2) PosteriorinletVSD below ri mitralregurgitation
(3) One AV valve commonto RV + LV with Hepatoclavicular projectionin 45" LAO + C-C 45'
5-6 leaflets (= 4-chamberview):
(a) anteriorcommon"bridging"leaflet r/ best view to demonstrateLV-RAshunt
(b) two lateralleaflets r/ best view to demonstrateVSD (inflowtract +
(c) posteriorcommon"bridging"leaflet posteriorportionof interventricular septumin profile)
 CardiovascuIar Disorders 629

LAT projection: Site: main renalartery+ majorsegmental

r/ irregularappearanceof superiorsegmentof branches;oftenbilateral
anteriormitralvalve leafletover LVOT { narrowannularradiolucent band
Prognosis: 54/" survival rate at 6 months, 35/" at r/ poststenoticfusiformdilatation
12 months, 15/oat 24 months,4/o at Cx: dissection
5 years;91% long-termsurvivalwith primary 2. MEDTALFTBROPLASTA (60-85%)
intracardiacrepair,4-17% operativemortality = fibromuscular hyperplasia= medialfibroplasia
with microaneurysm
ENDOCARDIAL FIBROELASTOSIS Age: 20-50 years;typicallyaffectswomen
= diffuseendocardial thickeningof LV + LA from Path: multiplefibromuscular ridges+ severemural
depositionof collagen+ elastictissue thinningwith lossof smoothmuscle+ internal
Etiology: elasticlamina
(1) ? viralinfection Site: mid + distalrenalartery+ branches;usually
(2) Secondaryendocardialfibroelastosis bilateral
= subendocardialischemiain criticalLVOT r/ "string-of-beads" sign = alternatingareas of
obstruction:aorticstenosis,coarctation, stenoses(weblikeconstrictions)+ aneurysms
hypoplasticleft heartsyndrome (whichexceedthe normaldiameterof the artery)
. suddenonsetof CHF duringfirst6 monthsof life r/ singletubularfocalstenosis
r/ mitralinsufficiency: 3. ME D TA L (5-15% )
H Y P E R P LA S TA
(a) involvementof valve leaflets = FIBROMUSCULAR HYPERPLASIA
(b) shortening+ thickeningof chordaetendineae Path: smoothmuscle+ fibroustissue hyperplasia
(c) distortion+ fixationof papillarymuscles withinarterialmedia
r/ enlargedLV = dilatationof hypertrophiedLV from mitral Site: main renalarteryand branches
regurgitation ^/ tongsmoothconcentrictubularnarrowing
r/ restrictedLV motion DDx: Takayasuarteritis,sclerosingarteritis,vessel
r/ enlargedLA spasm,arterialhypoplasia
! pulmonaryvenouscongestion+ pulmonaryedema 4. P E R TME D ]AFTBL R OP LA S (20%
IA )
! LLL atelectasis(= compressionof left lower lobe = SUBADVENTITIAL FIBROPLASIA
bronchusby enlargedLA) Age: young females
Prognosis; mortalityalmost 100% by 2 years of age Path: fibroplasiaof outer 112of mediareplacing
externalelasticlamina
FIBROMUSCU
LAR DYSPLASIA Site: distal (mostlyright)main renal artery
= flor'totherosclerotic angiopathyof unknownpathogenesis r/ tongirregularstenosis
lncidence: <1"/"of cerebralangiographies;1,100 patients { beading= NO aneurysmformation(diameterof
reported(by 1982) beads not wider than normaldiameterof artery)
Age: children+ young adults<30-40 years;2lg >50 5. ME D TA L D IS S E C T| ON(5-10% )
y e a r s ;M : F= 1 : 3t o 1 : 9 Path: new channelin outer 1/3 of mediawithin
. hypertension externalelasticlamina
. progressiverenal insufficiency Site: main renalartery+ branches
Location: ^/ falsechannel,aneurysm
@ Cephalicarteries: 6. A D V E N TTTTA L R OP LA S (<
FTB TA1% )
cervical+ intracranialICA (85%),extracranialcarotid = SUBADVENTITIAL HYPERPLASIA
artery(30%), vertebral artery(7%); both anterior Path: adventitial+ periarterialproliferationin
+ posteriorcirculation(8%);bilateral(60-65%) fibrofattytissue
Associatedwith: brain ischemia(up to 50%), Site: main renalartery,largebranches
intracranial aneurysms(up to ^/ tongsegmentalstenosis
30%),intracranial tumors(30%), 7. ATYPICALFIBROMUSCULAR DYSPLASIA
bruits,trauma (= ? variantof intimalfibroplasia)
@ Abdominalaorta: r/ web = sffioothI corrugaledmass involvingonly
renalartery(60%),otheraorticbranches(in 1-2/": one wall of vessel+ projectinginto lumen
celiaca., hepatica., splenica., mesenterica., iliac DDx: atherosclerotic disease,posttraumatic
a. ) aneurysm
0 Simultaneous involvement of renali muscular
arteriesin 3% VARIANT: Segmental mediolytic arteriopathy
1. T NT |M AF L TB R OP L AS (1 -2 % )
TA = rdra noninflammatory diseaseof small + medium
= intimalhyperplasia arteries
. progressive Histo: focal segmentaldisruptionof medialsmooth
Path: circumferential / eccentricfibroustissue musclecellswith mediolysis
betweenintima+ internalelasticlamina { string-of-beads appearance
Age: children+ youngadults;M:F = 1:1 ./ irregularstenoses+ aneurysms
630 RadiologyReviewManual

HeterotaxySyndromes
Asplenia Polysplenia
= bilateralR sidedness = bilateralL sidedness

Clinical
Presentingage newborn/ infant infant/ adult
Sex predominance male female
Cyanosis severe usuallyabsent
Heartdisease severe moderate/ none (5-10%)
I Heinzbodies
Howell-Jolly present absent
Spleenscan no spleen multiplesmallspleens
CharacteristicECG none abnormalP-wavevector
Prognosis poor good
Mortality hi gh low
Plain radiograph
Lung vascularity decreased normal/ increased
Aortic arch rightI left righti left
Cardiacapex rightlleft l midline right/ left
Bronchi bilateraleparterial bilateralhyparterial
Minorfissure possiblybilateral none/ normal
Stomach midline/right/left right/ left
Liver symmetrical/R / L in variouspositions
Malrotationof bowel yes (microgastria) yes
Cardiography
Coronarysinus usuallyabsent sometimesabsent
Atrialseptum commonatrium(100%) ASD (84%)
AV valve atresia/ commonvalve normal labnormalMV
Singleventricle 44% infrequent
IVS VSD VSD common
Greatvessels (72%)
d- I l-transposition normalrelationship
Pulmonarystenosis the rule frequent
Pulmonaryveins TAPVR PAPVR(42%)TAPVR (6%)
Singlecoronaryartery 19/"
SVC bilateral(53%) bilateral(33%)
IVC-aortarelationship same side of spine normal
rvc normal interrupted(84%)/ normal
Azygosvein inapparent continuationR / L

Cx: dissection(in 3%), macroaneurysmformation, Location: chordaeto leafletfrom posteromedialpapillary

intramuralhemorrhage
Prognosis; tends to remainstable/ minimalprogression
of lesionsin 20ohcausingdeclinein renal
function
Rx: (1) Resectionof diseasedsegmentwith end-to-end
anastomosis
(2) Replacementby autogenousvein graft,excision
HETEROTAXY
+ repairby patch angioplasty
(3) Transluminalballoonangioplasty(90% success
rate with very low restenosisrate)
FLAIL MITRAL VALVE
Cause:
(1) rupturedchordaetendineaein rheumaticheart
disease,ischemicheartdisease,bacterial
endocarditis
(2) ruptureof head of papillarymusclein acute
myocardialinfarction,chesttrauma
muscle(singlevesselblood supply)
r/ deep holosystolicposteriormovement
r/ randomanarchicmotionpatternof flail parts in diastole
r/ excessivelylarge amplitudeof openingof aML
SYNDROME
lhetero, Greek = differentitaxis, Greek = dfr?I'lgement]
= CARDIOSPLENIC SYNDROMES
= situs ambiguuswith a spectrumof variouscongenital
truncalabnormalities+ frequentlycardiacmalformations
from aspleniato potysplenia
Embryology:
primarydefect in lateralizationwith disruptionof
completeseparationof cardiacchambersduring
20-30 days of gestation
lnheritance: multifactorial(autosomaldominant,
autosomalrecessive,X-linkedrecessive)
 Cardiovascular
Disorders 631

lndividualized approach of classification: @ Abdomen

describesall criticalstructuresby analyzing r/ absentspleen
(a) positionof atria r/ centrallylocated"bridging"
liver= hepaticsymmetry
(b) positionof venousdrainagebelowdiaphragm r/ stomachon right/ leftside/ in centralpositionand
relativeto midline small(microgastria)
(c) positionof aortarelativeto midline Prognosis: up to 80% mortalityby end of 1st year of life
(d) positionof the stomach+ presenceof malrotation
(e) positionof liver+ gallbladder PolyspleniaSyndrome
(f) positionof cardiacapex = BILATERAL LEFT-SIDEDNESS = LEFTISOMERISM
(g) presence,appearance, and numberof spleens Age: presentation in infancy/ adulthood;M < F
(h) presenceof bi- / trilobedlungs Associated with:
(a) CHD (>50%):
AspleniaSyndrome APVR (70%),dextrocardia(37"/"),ASD (37%),
= B I LA T E RARIGH
L T -SID ED N =
E SS
R IGH TIS O ME R IS M ECCD(43-65%),pulmonicvalvularstenosis
= IVEMARK SYNDROME (23%),TGA (13-17"/.),DORV (13-20%)
lncidence; 1:1,750-1:40,000livebirths;M > F . no / mildCHD in most patients
Associated with: (b) Gl abnormalities:
( a) CHD ( in 5 0 % ): esophagealatresia,TE fistula,gastricduplication,
TAPVR(almost100%),endocardial cushion preduodenal portalvein,duodenalwebs + atresia,
defect(85%),singleventricle(51%),TGA (58%), shortbowel,mobilececum,malrotation,
pulmonarystenosis/ atresia(70%),dextrocardia semiannular pancreas,biliaryatresia,absent
(42o/"),mesocardia,VSD, ASD, absentcoronary gallbladder
sinus,commonatrium,commonhepaticvein (c) GU anomal i es(15% ):
( b) G l anom a l i e s : renalagenesis,renalcysts,ovariancysts
Partial/ totalsitus inversus,annularpancreas, (d) Vertebralanomalies,commonceliactrunk-SMA
agenesisof gallbladder, ectopicliver,esophageal . CHF (dueto L-to-Rshunt)
varices,duplication+ hypoplasiaof stomach, . heartmurmur,occasionalcyanosis
Hirschsprung disease,hindgutduplication, . leftward/ superiorlydirectedP-wavevector
imperforateanus . heartblock(dueto ECCD)
(c) GU anomalies(15%): . extrahepaticbiliaryobstruction
Horseshoekidney,doublecollectingsystem, ri absenceof IVC (on LAT CXR)
hydroureter,cystickidney,fused/ horseshoe r/ largeazygosvein (on AP CXR) may mimicaorticarch
adrenal,absentleft adrenal,bilobedurinary @ Lung
bladder,bicornuateuterus r/ bilateralmorphologic left lungs(55-68%),normal
(d) Cleftlip / palate,scoliosis,singleumbilicalartery, (18/"), bilateralR-sidedlungs(7%)
lumbarmyelomeningocele r/ bilateralhyparterialbronchi(= arteriesprojecting
. cyanosisin neonatalperiod/ infancy(if severe
superiorto bronchion PA view + posteriorto
cyanoticCHD) tracheobronchial tree on LAT view)
. severerespiratorydistress
r/ normal/ increasedpulmonaryvascularity
. Howell-Jolly bodies= RBC inclusionsin patientswith r/ absenceof middlelobefissure
absentspleen @ Heart& great vessels
{ cardiacapex discordantfrom stomach+ liver ^/ bilateralpulmonaryI leftatria+ pointed,tubular,
r/ absentspleen(riskof sepsis) narrow-basedappendages
{ cardiacapex on R / in midline
@ Lung r/ bitateratSVC (50%)
^/ bilateraltrilobedlungs= bilateralminorfissures ^/ interruptionof hepaticsegmentof IVC with azygos
(sPECrFrC) I hemiazygoscontinuationin 65-70/. (MOST
^/ bilateraleparterialbronchi(MR / tomogram) C ON S TS TE NFtNT D tN G)
= pulmonaryarteriesinferiorto bronchion PA view
@ Abdominalheterotaxy(56%)
+ projectinganteriorto tracheaon LAT view r/ presenceof >2 spleens(usuallytwo major
./ diministredpulmonaryvascularityI pulmonary + indefinitenumberof splenules)locatedon both
venoushypertension (TAPVRbelowdiaphragm) sidesof the mesogastrium (esp.greatercurvature
of stomach)
@ Heart& greatvessels ri centrallylocatedliver = hepaticsymmetry
^/ bilateralsystemic/ rightatriawith broad-based ri absenceof gallbladder(50%)
, appendages ri stomachalwayson same side of spleen(s)
r/ ipsilaterality
of abdominalaorta + IVC r/ malrotationof bowel (80%)
= juxtaposed"piggybacked"IVC (aortausually ! preduodenalportalvein
posterior)(MOSTRELIABLEINDICATOR) OB-US:
r/ bilateralSVC { absenceof intrahepatic IVC
632 Radiology Review Manual

r/ aortaanteriorto spinein midline E C H O:

^/ "doublevessel"sign = 2 vesselsof similarsize in ./ normali enlargedLA
paraspinouslocationposteriorto heart= oort? ! slitlike/ small/ normalLV
+ azygosvein on left I rightside of spine { enlargedRA
Prognosis: 50h mortalityby 4 months; r/ herniation+ prolapseof foramenovaleflap into RA
75/" mortalityby 5 years; ^/ hypoplasticascendingaorta (<5 mm = aorticatresia)
90% mortalityby midadolescence ! absent/ grosslydistortedmitralvalve echoes
A ngi o:
HYPOPLASTIC LEFT HEART SYNDROME ri retrogradeflow in ascendingaorta + aorticarch
= SHONESYNDROME = AORTIC ATRESIA + coronaryarteriesvia PDA
= underdevelopment of left side of heartcharacterizedby ri stringlikeascendingaorta<6 mm in diameter
(a) hypoplastic/ atreticaorticvalve ri massiveenlargementof RV + RVOT
(b) hypoplastic/ atreticmitralvalve Prognosis.' almost 100%fatal by 6 weeks
(c) hypoplasticLV (due to endocardialfibroelastosis) Time of diagnosis: 32/" pre-,65Yo1-4 days postnatally
(d) hypoplasticascendingaorta Rx: (1) ProstaglandinE1 (patencyof ductusarteriosus)
(e) normallyrelatedgreat vessels (2) Hypoventilation(increasein CO, maintainshigh
Prevalence:0.2 / 1,000livebirths; M:F = 2:1 pulmonaryvascularresistance)
0 4th mostcommoncardiacmalformation manifesting
in (3) NitroprussidelV (decreasessystemicvascular
1st year of life (afterVSD, TGV, tetralogyof Fallot) resistance)
0 Mostcommoncauseof CHF in neonate (4) Norwoodprocedure= palliativeattempt
0 Responsiblefor 25'/" of all cardiacdeathsin 1st week (5) Cardiactransplant
of life
Hemodynamics: RIGHTVENTRICLE
HYPOPLASTIC
pulmonaryvenousblood in LA faces an atretic/ stenotic = PULMONARY ATRESIA WITHINTACT VENTRICULAR
MV (= pulmonaryvenousoutflowobstruction) and is S E P TU M
divertedto RA throughherniatedforamenovale/ ASD = underdeveloped rightventricledue to pulmonaryatresia
(Lto-R shunt);RV supplies(a) pulmonaryartery, in the presenceof an intactinterventricularseptum
(b) ductusarteriosus,(c) descendingaorta (antegrade Type | = small RV secondaryto competenttricuspid
flow), (d) aorticarch + ascendingaorta + coronary valve(morecommon)
circulation(retrogradeflow) leadingto RV work overload Type ll = normal I large RV secondaryto incompetent
+ CHF tricuspidvalve
Assaciatedmalfarmations: Hemodynamies:
coarctationof aorta,PDA, patentforamenovale,dilated fetus : L-to-Ratrialshuntthroughforamenovale;
pulmonaryartery,VSD,dilatedRA, enlargedRV, retrogradeflow throughductusarteriosus
double-outlet rightventricle,endocardial fibroelastosis intopulmonaryvascularbed
. severeCHF (RV volume+ pressureoverload): neonate : closureof ductusresultsin cyanosis,
. characteristically presentswithinfirstfew hoursof life acidosis,death
. ashengray colorI duskycomplexion(systemic rl small rightventricularcavity(apexof RV + LV shouldbe
underperfusion due to inadequateatrialL-to-Rshunt) at same level)
. myocardialischemia(decreasedperfusionof aorta r/ atresiaof pulmonaryvalve
"commoncoronaryartery"]+ coronaryarteries): r/ hypoplasticproximalpulmonary arlery
[=
. cardiogenicshock,metabolicacidosis(whenductus ./ secundumatrialseptaldefect(frequentlyassociated)
arteriosuscloses) Rx: prostaglandin E1 infusion+ valvotomy+ systemic-
CXR: pulmonaryarteryshunt
r/ hypoplastic / normal/ enlargedcardiacsilhouette:
rl prominentrightatrialborder IDIOPATHIC DILATATION OF PULMONARY
r/ + absenceof left ventricularsilhouette ARTERY
{ + thymicatrophy = CONGENITAL ANEURYSM OF PULMONARY ARTERY
r/ interstitial+ alveolarpulmonaryedema (due to A ge: adol escence; McF
pulmonaryvenoushypertension with severely . systolicejectionmurmur(in mostcases)
restrictiveinteratrialcommunicationin 80%) ^/ dilatedmain pulmonaryartery
r/ normalpulmonaryvasculature(withwide { normalperipheralpulmonaryvascularity
nonrestrictive interatrialcommunicationin 20%) { normalpulmonaryarterialpulsations
OB-US (may be missed<22weeks GA): ^/ tttOlateralization
of pulmonaryflow
r/ small left ventricularcavity(apexof LV and RV should Dx per exclusion:
be at same level) 1. Absenceof shunts,CHD, acquireddisease
r/ hypoplasticascendingaorta 2. NormalRV pressure
r/ aorticcoarctation(in 80%) 3. No significantpressuregradientacrosspulmonicvalve
^/ diastolicflow reversalin narrowascendingaorta is DDx: (1) Marfansyndrome
DIAGNOSTIC (2) Takayasuarteritis
 CardiovascuIar Disorders 633

INTERRUPTION
OF AORTICARCH Collateral supply:
= rdfe congenitalanomalyas a commoncauseof deathin 1. Arteriesarisingfrom arch + ascendingaorta
the neonatalperiod 2. Bronchialvessels
Trilogy: (1) Interrupted
aorticarch 3. Intercostalvessels
(2)vsD 4. Branchesfrom subclavianartery
(3) PDA (pulmonarybloodsupplieslowerpart of Location: usuallyoppositefrom aorticarch; R + L
body) pulmonaryarteryequallyinvolved
Associatedwith (in 1/3): CXR:
1. Bicuspidaorticvalve r/ hypoplastic ipsilateral
lung
2. Muscularsubaorticstenosis ri mediastinalshifttowardinvolvedfung
3. A S D r/ hemidiaphragmmay be elevated
4. Truncusarteriosus r/ small hyperlucentipsilateralchestwith narrowed
5. Transposition intercostalspaces
6. Completeanomalouspulmonaryvenousreturn { "comma-shaped" smalldistortedhilarshadow
. presentswith CHF ^/ asymmetryof pulmonaryvascularity
Location: r/ normalrespiratorymotion(normalaerationof
Type A: distalto left subclavianartery(42%) hypoplasticlung)
Type B: betweenleft CCA and subclavianartery NUC: { absentperfusionwith normalaeration
(53%)associatedwith: DiGeorgesyndrome Angio: r/ absentpulmonaryartery
, Type C: between innominateand left CCA (a%) Rx: surgicalanastomosisbetweenproximal+ distal
r/ dilatationof rightatrium+ ventricle pulmonaryartery(to preventprogressivepulmonary
r/ dilatationof pulmonaryartery hypertension with dyspnea,cyanosis,hemoptysis,
r/ ascendingaorta much smallerthan pulmonaryartery death)
{ arch formedby pulmonary arlery+ ductusarteriosus DDx: (1) Hemitruncus
givesthe appearanceof a low aorticarch (2) Swyer-James syndrome(ipsilateral air trapping,
! aorticknob absent reducedventilation+ perfusion)
r/ tracheain midline
1 tlO esophagealimpression
ri retrosternalclear space increased(smallsize of
, ascendingaorta) INTRAVENOUS
DRUGABUSE
ri increasedpulmonaryvascularity(L-to-Rshunt) Complicationssecondaryto:
Prognosis: 76/" dead at end of 1st month (a) directtoxic effectsof drugs or drug combinations
(eg, heroin+ cocaine/ Talwin)
INTEBRUPTION OF PULMONARY ABTERY (b) directtoxic effectsof adulterants[eg, heroinis mixed
= pulmonary
trunkcontinues
onlyasonelargeartery
to ("cut")with quinine,bakingsoda,sawdust]
one lungwhilesystemicaorticcollateralssupplythe (c) septicpreparation
otherside (d) injectiontechnique
Associatedwith: CHD (particularlyif interruptionon left (e) choiceof injectionsite (eg,"groinhit"intofemoral
s i d e ): vein;"pocketshot"intojugular,subclavian,
1. Tetralogyof Fallot brachiocephalic vein)
2. Scimitarsyndrome= cong€rlitalpulmonary A. Cardiovascularcomplications
venolobarsyndrome 1. Arterialpseudoaneurysm
3. P DA ,V S D may be followedby rupturewith exsanguination i
4. Pulmonaryhypertension lossof limb
2. Arteriovenous fistula
3. Arterialocclusion
(a) at injectionsitedue to intimaldamage,
thrombosis,spasm
(b) distalto injectionsite due to embolization,
spasm
4. Venousthrombosis
5. Intravenous migrationof needleto heart/ lungs
6. Embolization of infectiousagent/ foreignbody/ air
throughinadvertentarterialinjection("hitthe pink")
7. Endocarditis(mostcommonlyS. aureus)
42Vo 53Vo 4Vo B. Softtissue complications
Interruption of Aortic Arch 1. Hematoma/ abscess
LCC = left common carotid artery LS = left subclavian artery 2. Foreignbodies
= right common carotid arter/, RS = right subclavian artery, 3. Lymphadenopathy
FSq
PDA = patent ductus arteriosus 4. C el l ul i ti s
634 Radiology Review Manual

C. Skeletalcomplications (4) Asynchrony = disturbedtemporalsequenceof

1. Osteomyelitis contraction
(a) directcontamination:eg, pubicbone ("groin Coronaryangiography:1.2 millionproceduresper year
hit")/ clavicle("pocketshot")
(b) hematogenous:spinemostcommonlyaffected KAWASAKISYNDROME
2. Septicarthritis:spine,sacroiliac, sternoclavicular, = MUCOCUTANEOUS LYMPHNODESYNDROME
symphysispubis,acromioclavicular, hip,knee,wrist = scut€ febrilemultisystemvasculitisof unknowncause
D. Pleuropulmonary complications involvinglarge+ medium-sized + smallarterieswith a
1. Pneumothorax ("pocketshot") predilectionfor the coronaryarteries
2. Hemo-/ pyothorax lncidencei averageof 1.1:100,000populationper year
3. Septicpulmonaryemboli Histo: panvasculitis
E. Gastrointestinal complications Age: <5 years of age (in 85%); peak age of 1-2 years;
1. Severecolonicileus M : F= 1 . 5 : 1
2. Colonicpseudoobstruction Associatedwith: polyarthritis(30-50%), aseptic
3. Necrotizing enterocolitis meningitis(25%),hepatitis(5-10%),
4. Liverabscess pneumoni ti(5-10%
s )
F. Genitourinary complications . fever >5 days
1. Focal/ segmentalglomerulosclerosis (heroinabuser) . mucosalreddening(injectedfissuredlips,injected
2. Amyloidosis pharynx,strawberrytongue)in 99%
G. CNS complicalions . nonpurulentcervicallymphadenopathy (82%)
1. Spinalepiduralabscessin 5-18% (fromvertebral . maculopapular rashon extensorsurfaces(99%)
osteomyelitis) . bilateralnonpurulent conjunctivitis(96%)
2. Cord compression(fromcollapsedvertebralbody) . erythemaof palms+ soleswith desquamation (88%)
3. Cerebralinfarction(fromsubacutebacterial @ Cardiovascular system (1/3)
endocarditis, toxiceffectof drug,spasm,intimal 1. Coronaryarteryabnormality(15-25%)
"pocketshot")
damagefrom { coronaryarteryaneurysm:LCA (213),RCA (1/3);
4. Intracranial hemorrhage(fromtrauma, proximalsegmentin 70"/";487oregress,
hypertension, drugs,
injectionof anticholinergic 37o/"diminishin size
vasculitis,ruptureof mycoticaneurysm) ^/ coronaryarterystenosis(39%)due to thrombus
5. Meningitis, cerebralabscess formationin aneurysm+ intimalthickening
ri coronaryarteryocclusion(8%) in aneurysms
> 9 mm
2. Myocarditis(25%)
ISCHEMIC HEART DISEASE 3. Pericarditis
= coRoNARYARTERY DISEASE (CAD) 4. Valvulitis
lncidence; 1.5 million/year;leadingcause of death in 5. Atrioventricular conductiondisturbance
industrialnations r/ intestinalpseudoobstruction
Morbidity: 28.7 cases per 1,000 men per year r/ transientgallbladderhydroPs
Mortality: 3.1 deathsper 1,000men per year Prognosis: 0.4-3/"mortality (frommyocardialinfarction
Noninvasive testing: i myocarditiswith congestiveheartfailure/
1. Noninvasive testingis of marginalbenefitwhen ruptureof coronaryarteryaneurysm)
diseaseprevalenceis <0.2I >0.7 Rx: aspirin(100 mg/kgper day) + gammaglobulin
2. Concordantthallium-201 and stressECG are greater DDx: infantilepolyarteritis
predictorsof diseaseprobabilitythan eitherone used
aloneandiorwhen discordant
3. Sequentialthallium-201 and stressECG are most LEIOMYOSARCOMA OF IVC
mostcommonintravascular venoustumor
usefulto establishthe diagnosisof CAD when
pretestprevalenceis intermediate+ test resultsare Path: growthpatterns:extravascular,intravascular,
concordant combined
. shortnessof breath(decreasedcardiacreturn)
CX R:
. elevatedliverfunctions,jaundice
{ often normal
. lower-extremity edema
{ coronaryarterycalcification
followingacute r/ collateralpathwaysbypassingIVC: hemiazygos,
{ pulmonaryvenoushypertension
infarction(40%\ azygos
./ Lv aneurysm ! tumorextensionfrom IVC into rightatrium
E CHO : US:
^/ bloodflow in IVC / hepaticveins may be absent/
ri regionof dilatationwith disturbanceof wall movement
(1) Akinesis = flo wdll motion reversed/ turbulent(dependingon degreeof
(2) Hypokinesis= reducedwall motion obstruction)
(3) Dyskinesis = paradoxicalsystolicexpansion { tumorvascularity(DDxfrom thrombus)
 Cardiovascular
Disorders 635

CT: Trigger: drugs(eg,penicillin), microorganisms,

^/ contrastenhancement of tumor
Cx: Budd-Chiari syndrome (fromsudden/ gradual
occlusion
of hepaticveins/ IVC/ both)
DDx of tumor extension into right atrium:
renalcell carcinoma,hepatocellular carcinoma
LYMPHOMA OF HEART
Secondary Cardiac Lymphoma
lncidence: in 16-28% on autopsy;pericardial
involvementmorefrequent;morecommon
in immunocompromised patients
heterologous proteins,tumorantigens
. hemoptysis, hematuria,proteinuria
. abdominalpain, Gl bleeding,musclepain + weakness
. ANCA (antineutrophil cytoplasmic autoantibodies)
in
>80a
. negativeserologictests for hepatitisB
Location: skin,mucousmembranes,lung,brain,heart,
Gl tract,kidney,muscle
0 Mostcommoncauseof the pulmonary-renal syndromel
./ pulmonaryinfiltrates(due to capillaritii)
r/ glomerulonephritis(90%)
Rx: removalof offendingagent

Primary Cardiac Lymphoma

= lymphomathat involvesonly heart/ pericardiumat MITRALREGURGITATION
time of diagnosis(extremelyrare) = MITRAL INSUFFICIENCY
Age: 13-90 (mean,G0)years Cause:
Predisposed: immunocompromised patients, A. ACUTE
e s p .AID S 1. Spontaneous ruptureof chordaetendineae
Path: multiplefirm nodules;contiguousinvasionof 2. Myocardialinfarctionwith involvementof papillary
pericardium muscle(posteromedial > anterolateralpapillary
Histo: typicallyNHL: well-differentiated B-cell muscle)
lymphoma,follicularcentercell lymphoma, 3. Bacterialendocarditis
diffuselargecell lymphoma,undifferentiated 4. Periprosthetic valve leak
Burkitt-likelymphoma B. CHRONIC
. unresponsiverapidlyprogressiveheartfailure '1.
Acuterheumaticfever
. arrhythmia,cardiactamponade,SVC syndrome (a) isolated: frequentlyseen in children
. chest pain (b) uncommonin adults(mostlycombinedwith
Location; FIA> FIV> LV > LA > atrialseptum> stenosis)
ventricularseptum;>1 chamber(75%) 2. Mitralvalve prolapsesyndrome
CX R: 3. Atrial myxoma
{ cardiomegaly 4. Coronaryarterydisease
{ signsof CHF 5. ldiopathichypertrophic subaorticstenosis(IHSS)
{ massivepericardialeffusion 6. Myxomatousdegenerationof mitralvalve:
E CHO : eg, Marfansyndrome
ri hypoechoicmyocardialmassesin RA / RV 7. Mitralannuluscalcification
ri pericardialeffusion B. Functional / secondary(fromdilatationof mitral
CT: ring in any conditionwith severedilatationof LV)
{ nypo-/ isoattenuating massesrelativeto 9. Congenitalheartdisease: short/ abnormally
myocardium insertedchordaetendineae;persistentostium
ri heterogeneousenhancementof masses primumASD with cleft mitralvalve,corrected
MR: transposition with Ebstein-likeanomaly
{ poorlymarginatedheterogeneouslesionsof iso- to Hemodynamics:
hypointensityrelativeto myocardiumon Tl Wl backwardflow of bloodfrom LV into LA during LV
ri lesionsisointense to myocardiumon T2Wl systole;increasedvolumeof blood underelevated
r/ heterogeneousenhancementwith gadolinium pressurecausesdilatationof LA; markedincreasein LV
Dx: positivecytologyin pericardialfluid (in 6T%\ diastolicvolumewith littleincreasein LV diastolic
exploratorythoracotomywith biopsyof cardiac pressure(= increasein preloadwithoutincreasein
tissue afterload= elevatedejectionfraction)
Prognosisi very poor
A. ACUTEMITRALREGURGITATION
MICROSCOPIC POLYANGITIS r/ pulmonaryvenoushypertension with engorged
= MICROSCOPIC POLYARTERITIS = HYPERSENSITIVITY pulmonaryvesselsand cephalization (lessthan with
VASCULITIS = LEUKOCYTOCLASTIC VASCU LITIS mitralstenosis)
= pauci-immune necrotizingsmall-vessel angitiswithout ^/ symmetricinterstitialialveolarpulmonaryedema:
granulomatous inflammation r/ asymmetricright upper lobe edema (g%) due to
Path: necrotizingarteritisidenticalto polyarteritisnodosa preferentialflow of regurgitantjet into pulmonary
but in vesselssmallerthan arteries(= arterioles, vein of RUL (PATHOGNOMON|C)
venulesand capillaries) r/ timitedcardiacenlargement
636 Radiology Review Manual

Stage 2 : interstitialedema
S tage3 : al veol aredema
S tage4 : hemosi deri ndeposi ts+ ossi ficat ion
@ Left heart
r/ enlargedLA + wall calcification:
r/ "doubledensity"seen throughrightuppercardiac
border(AP view)
r/ butgeof superiorposteriorcardiacborderbelow
carina(lateralview)
ClassicMitral ValveStenosis { splayingof mainstembronchi
(for abbreviations
seepage594)
{ esophagusdisplacedtowardright + posteriorly
r/ ditatedleft atrialappendage(not presentwith
retractingclot),in 90% associatedwith rheumatic
B . CHRO NI CM IT R A LR E G U R G]T A T IO N heartdisease
r/ enlargedheart 0 Dilatationof left atrialappendage+ calcification
r/ massiveLA: = rheumaticheartdisease!
^/ m posteriorwall calcification(McOallumpatch) r/ calcificationof valve leafletsin 60% of severeMS,
{ enlargedLA appendage(with historyof previous usually>50 years of age (DDx: calcificationof mitral
rheumaticheartdisease) annul us)
r/ mitralannularcalcification(frequent) r/ normal/ undersizedLV
rl markedLV enlargement(cardiothoracic ratio >0.55) .l small aorta (due to decreaseof forwardcardiac
+ LV failure output)
ECHO: @ Rightheart
r/ Lv volumeoverload: ri prominentpulmonarYarlerysegment(precapillary
! normal-sized / enlargedLV hypertension)
{ increasedseptal+ posteriorwall motion r/ hypertrophyof RV
./ increasedEF slope
{ early closureof aorticvalve (LV strokevolume
partiallylost to LA)
./ m enlargement(in chronicMV insufficiency)
r/ nulgingof interatrial septumto the rightduringsystole
{ Doppleris onlydiagnostictool + allowsassessmentof
severity

MITRAL STENOSIS
Cause: rheumaticheartdisease(5-15 years after initial
episodeof rheumaticfever);carcinoid
syndrome; eosinophi ; rheumatoid
lic endocarditis
arthritis;SLE; massobstructingLV inflow
(tumor,atrialmyxoma,thrombus);congenital
1 y 1 .=f 1 : g
Hemodynamics:
rise in left atrial+ pulmonaryvascularpressure
throughoutsystoleand intodiastole;compensatory
dilationof LA + pulmonaryvenoushypertension;
developmentof medialhypertrophy + intimalsclerosisin
pulmonaryarteriolesleadsto postcapillarypulmonary
arterialhypertension;RV hypertrophy;tricuspid
regurgitation; RV dilatation;rightheartfailure Midsystolic Mitral Valve Prolapse
May be associatedwith: ASD = Lutembachersyndrome
(in 0.6%)causingL-to-Rshunt
. historyof rheumaticfever (in 50%)
. dyspneaon exertion,orthopnea,paroxysmalnocturnal
dyspnea
. atrialfibrillation
. systemicembolizationfrom thrombosisof atrial
appendage
Stages (accordingto degreeof pulmonaryvenous
hypertension):
Stage1 : lossof hilarangle,redistribution Holosystolic Mitral Valve Prolapse

r/ dilatationof RV (tricuspidinsufficiency / pulmonary
hypertension)
^/ increasein cardiothoracicratio
d diminutionof retrosternalclear space
r/ tVC pushedbackward(lateralview)
@ Lung
r/ pulmonaryvascularcephalization= redistribution
pulmonarybloodflow to upper lobes(postcapillary
pr es s ur e1 6 -1 9 mm H g )
r/ interstitial pulmonaryedema(postcapillary pressure
20-25 mm Hg)
DDx: interstitial fibrosis/ depositionof hemosiderin-
laden macrophages(= "browninduration")of
chronicmitralvalve stenosis
r/ alveolaredema (postcapillary pressure
25-30 mm Hg)
DDx: diffusealveolarhemorrhage= diffuse
confluentacinar/ ground-glass areasof
increasedopacitysparingthe lungperiphery
(= "window frame"effect)
! pulmonaryhemosiderosis:
{ 1- 3 m m i l l -d e fi n endo d u l e s
r/ fine / coarsereticularareas of increasedopacity
with biasfor middleand lowerlungs
r/ pulmonaryossification (3-l g/o) = denselycalcified
1-3-5 mm nodules(+ trabeculae)mainlyin middle
and lowerlungs
E CHO :
r/ thickeningof leafletstowardfree edge (fibrosis,
calcification)
r/ flatteningof EF slope = MV remainsopen throughout
diastoledue to persistently high LA pressure(crude
indexof severityof MV stenosis)
r/ diastolicanteriortrackingof pML in g0% (secondary
to diastolicanteriorpull by larger + moremobileaML)
r/ diastolicdomingof MV teiflets
r/ commissurefusion= increasedechodensity + mitralregurgitation)
decreasedleafletmotionat levelof commissure
r/ area reductionof MV orifice: normalwithin f6 cm2;
mild narrowingwith <2 cmz;severenarrowingwith
, <1 cmz(reproducibleto within0.3 cm2)
r/ shortening+ fibrosisof chordaetendineae
! abnormalseptalmotion= earlydiastolicdip of IVS
, due to rapidfillingof RV (in severeMV stenosis)
r/ slowedLV fillingpatternof small LV
r/ dilatationof LA (>5 cm increasesrisk of atrial
fibrillation + leftatrialthrombus)
./ Of openingamplitudereducedto <20 mm indicating
, lossof valvepliability(DDx: low cardiacoutputstate)
r/ absentA-wavecommon(atrialfibrillation)
^/ increasein valvegradient+ pressurehalftimeon
Doppler
Rx: (1) Commissurotomy if valvespliable+ calcium
absent+ MV regurgitation absent
(2) Valve replacementfor symptomaticpatientswith
severelystenoticvalves
DDx:
(1) Pseudomitral stenosisin decreasedLV compliance
(decreasedEF slope,normalleafletthickness
+ motion)
Cardiovascular
Disorders OST
(2) Rheumaticmitralinsufficiency (indistinguishable
findings+ evidenceof LV volumeoverload)
(3) LA myxoma(massbehindMV + in LA)
(4) Low cardiacoutput(apparentsmallvalveorifice)
LUTEMBACHER SYNDROME= rheumaticmitralvalve
of stenosis+ ASD
MITRAL VALVE PROLAPSE
lncidence: 2-6% of general population;S-20"/"of
youngwomen;? autosomaldominant
inheritance
Age: commonly14-30 years
Cause:
(1) "Floppymitralvalve"= elongationof cusps
+ chordaeleadingto redundantvalvetissue,which
prolapsesinto LA duringsystole
Associated with:
(a) Skeletalabnormalities:scoliosis,straightening
of thoracicspine,narrowanteroposterior chest
dimension,pectusexcavatumdeformityof
sternum
(b) Barlowsyndrome= straightback syndrome
(c) Marfansyndrome
(d) Tricuspidvalve prolapse
(e) Long-standing ASD
(f) Autosomaldominantpolycystickidneydisease
(2) SecondaryMV prolapse:
papillarymuscledysfunction, ruptureof chordae
tendineae,rheumaticmitralinsufficiency, primary
pulmonaryhypertension, ostiumsecundumASD
. arrhythmias, palpitation,
chestpain,light-headedness,
syncope
. responsiblefor midsystoliccfick+ late systolicmurmur
(whenassociatedwith mitralregurgitation)
r/ m not enlarged(unless".ro.Ltrd with significant
E C H O:
r/ interruption of CD linewith bulgetowardleftatrium:
r/ abruptmidsystolicposteriorbucklingof both leaflets
, (cfassicpattern)
r/ "hammocklike"pansystolicposteriorbowingof both
leaflets
r/ multiplescallopson mitralvalve leaflets(short-axis
parasternalview)
{ valve leafletsmay appearthickened(myxomatous
degeneration+ valve redundancy)
{ mitralvalveleafletspassing>2 mm posteriorto plane
of mitralannulus(apical4-chamberview)
r/ hyperactiveatrioventricular groove
r/ mitralannulusmay be dilated>4.7 cm2
DDx: (1) Pericardialeffusion(systolicposterior
displacementof MV leaflets+ entireheart)
(2) Bacterialendocarditis(mimickedby locally
thickened+ redundantleaflets)
MYOCARDIAL INFARCTION
lncidence: 1,500,000per year in USA resultingin
500,000deaths (50% occur in asymptomatic
individuals)
638 Radiology Review Manual

. atrioventricular block(commonwith inferiorwall B . ANEURYSM(12-15/" of survivors)

infarctionas AV nodalbranchoriginatesfrom RCA); C . MYOCARDIALRUPTURE(3.3%)
completeheart block has worse prognosisbecauseit . occursusuallyon 3rd-5th day post Ml
indicatesa largearea of infarction { enlargementof heart (slowleakageof blood into
C X R: pericardium)
r/ normal-sizedheart (84-95%) in acute phase if Prognosis.'cause of death in 13% of all infarctions;
previouslynormal almost 100%mortalitY
{ cardiomegaly:high incidenceof congestiveheart D . R U P TU R EOF P A P ILLA R Y MU S C LE(1% )
multiplemyocardial
failurewith anteriorwall infarction, from infarctionof posteromedialpapillarymusclein
infarctions,double-and triple-vessel CAD, LV inferiorMl (common)/ anterolateral papillarymusclein
aneurysm anterolateral Ml (uncommon)
CECT: . suddenonset ol massivemitralinsufficiency
{ perfusiondefectwithin60-90 secondsafter bolus . unresponsiveto medicalmanagement
injection { abruptonset of severepersistentpulmonaryedema
r/ delayedenhancementof infarctedtissuepeakingat { asymmetricPVH in right upper lobe
10-15 minutes (dueto accumulation of iodinein r/ minimalLV enlargement / normal-sized heart
ischemiccells),size of enhancedareacorrelateswell ./ trlOdilatationof LA (immediatedecompressioninto
with size of infarct pulmonaryveins)
Cx: (myocardiumis proneto ruptureduring Prognosis: 70"/"mortalitywithin 24 hours;
3rd-14th day post infarction) 80-90% within2 weeks
E. R U P TU R OF
E IN TE R V E N TR IC U LASRE P TUM
A. LEFTVENTRICULARFAILURE(60-70%) (0.t2%)
. "cardiacshock"= systolicpressure<90 mm Hg . occursusuallywithin4-21 days with rapidonset of
0 Signsof pulmonaryvenous hypertensionare a good L-to-Rshunt
predictorof mortality(>30%if present,<10'/. if . Swan-Ganzcatheterization:increasein oxygen
absent) contentof RV, capillarywedge pressuremay be
r/ progressiveenlargementof heart withinnormallimits
./ haziness+ indistinctness of pulmonaryarteries r/ right-sidedcardiacenlargement
ri increasein size of rightdescendingpulmonaryartery { engorgementof pulmonaryvasculature:
> 1 7m m r/ asymmetricPVH of right upper lobe
ri pleuraleffusion i/ ttlO pulmonaryedema (DDx to rupturedpapillary
r/ septallines muscle)
{ perihilap+ peripheralparenchymal clouding Prognosis: 24/" mortalitywithin24 hours;87% within
r/ alveolarpulmonaryedema 2 months;>90% in 1 Year
Mortality: 30-50% with mild LV failure;44/"with F. DRESSLERSYNDROME(<4%)
pulmonaryedema;80-100%with = POSTMYOCARDIAL INFARCTIONSYNDROME
cardiogenicshock;8% in absenceof LV Etiology: autoimmunereaction
failure Onset: 2-3 weeks (range 1 week-severalmonths)
followinginfarction
. relapsesoccur as late as 2 years after initialepisode
. fever
ri pericarditis+ pericardialeffusion
{ pleuritis+ pleuraleffusion
r/ pneumonitis

RightVentricularInfarction
0 Rightventricleinvolvedin 33% of left inferior
myocardialinfarction
^/ decreasedRV ejectionfraction
{ accumulationof Tc-99mpyrophosphate
Prognosis; in 50% RV ejectionfraction returnsto
normalwithin10 days
Cx: (1) cardiogenic shock(unusual)
Atrial Myxoma Prolapsinginto Mitral Valve (2) elevationof RA Pressure
Orifice (3) decreaseof pulmonaryarterypressure
Note the interval between the opening of aML and pML and the
moment that the tumor reachesits maximal anterior excursion at
point E when a slight additional opening of the aML results; aML MYXOMA
stays open during entire diastole as a result of obstruction to left = mostcommon primary
benign tumor(true
cardiac
atrial emptying. neoplasm)in adults,40-50/"of all cardiactumors
 Cardiovascular
Disorders 639

Age: 11-82 (mean50) years;90% of patientsare r/ decreasedpulmonaryvascularity

bet wee na g e s3 0 a n d 6 0 y e a rs ; M:F = 1 :1.7to 1:4 r/ pleuraleffusion(occasionally)
Classification: sporadic (most frequent); Cx: pulmonaryemboli
familialtype (meanage of 24 years); ECHO: (2D-ECHOis studyof choice)
complextype = Carneysyndrome r/ tumor attachedby narrowstalk
Path: (a) gelatinous, friable,papillary/ villous ^/ tumormobility:
pedunculated tumor r/ prolapseacrossAV valve duringdiastole
(b) round/ lobularsmoothsessiletumor(25%)with r/ tumordistensibility
firm surface r/ hyperechoicsphericalmass:
0 No infiltration of underlyingtissues! r/ internalhypoechoicareas (= hemorrhage,necrosis)
Histo: composedof myxomacells (= ovoid nucleuswith ri speckledechogenicfoci (= calcifications)
inconspicuous / largenucleoli+ abundant r/ frondlikesurfaceprojections
eosinophilic cytoplasm)) formingrings/ syncytia Doppler:
/ cords;hypocellular amorphousacid r/ valvularregurgitation
mucopolysaccharide matrixin areaswithout M-modefindingsof only historicalinterest:
fibrosis;coveredby a monolayerof endothelial ./ dense echoesappearingposteriorto aML soon
cells(= endocardial tumor) afteronset of diastole
Siz e: 0. 6- 12 ( m e a n ,5 .7 c) m r/ pML obscured
. short history+ rapidprogression ^/ tumor echoescan be tracedinto LA
. dyspnea,chestpain r/ ditatedtn
. constitutional symptoms(30%): r/ reducedE-F slope
. fever,myalgia,arthralgia, weightloss,lethargy C T:
. leukocytosis, anemia,elevatedESR,petechiae { well-defined sphericallovoid intraluminal
fillingdefect
. hypergammaglobulinemia ./ lobular/ smoothsurfacecontour
. positionalsymptoms(ie,changewith position)due to ^/ tumorattenuationlowerthan unopacified blood(due
hemodynamic obstruction : to gelatinouscomponent)
. arrhythmia(20h), heartmurmur r/ heterogeneous texture(due to hemorrhage,necrosis,
' congestiveheartfailure(valveobstruction) cystformation,fibrosis,calcification[16%],
. Syncope ossification)
. embolization(30-40%)to CNS, coronaryartery,aorta, MR:
kidney,spleen,extremities, pulmonaryartery(caused ^/ iso-/ hypointense on TlWl relativeto myocardium
by tumor fragments/ accumulatedthrombus) ./ heterogeneouscontrastenhancement(secondaryto
necroticareas)
Location: leftatrium(75-80%);rightatrium(10-20%); r/ markedlyhyperintense on T2Wl
ventricle(5%);biatrial(withgrowththrough { areas of decreasedsignalintensity(calcifications,
fossa ovalis) hemosiderin deposits)
Site: attachedto interatrialseptumby small stalk in fossa Rx: urgentsurgicalexcision+ valvuloplastyi valve
ovalis(75%)/ to wall of cardiacchambers/ to valve replacement
surfaces;may protrudeinto ventriclecausingpartial Prognosis; 5-1 4/" recurrencerate (multifocalmyxomas)
obstructionof atrioventricular
valve DDx: (1) Thrombus(mostcommonlyin LA + LV)
r/ smallmyxomasproduceno CXR findings (2) Othercardiactumors: sarcoma,malignant
r/ cardiomegaly mesenchymoma, metastasis,papillary
r/ atrialobstruction(mimickingvalvularstenosis) fibroelastoma(also arisesfrom narrowstalk)
r/ persistentdefectin atrium/ diastolicdefectin ventricle

A. LEFT ATRTALMYXOMA(75-80%)
with obstructionof mitralvalve: CarneyComplex
^/ pulmonaryvenoushypertension: = COMPLEX MYOMA
r/ pulmonaryvascularredistribution = autosomal-dominant inheriteddisorder
r/ interstitialedema Prevalence: 7Y"of all myxomas;150 patients
r/ enlargementof LA identifiedsince1985worldwide
1 NO enlargement of atrialappendage Age: youngerthan patientswith sporadicmyxoma
tr ossificlung nodules . endocrineoveractivity:
Cx: systemicemboli(27%) in 50% to CNS (stroke/ . Cushingsyndrome
"mycotic"aneurysm)
. sexualprecocity
B. RtcHT ATRTALMYXOMA(10-20%) . acromegaly
with obstruction of tricuspidvalve: (1) Cardiacmyxomas:multifocal(66%),outsideleft
^/ tumorcalcification:R > L atrium,recurringat an increasedrate after resection
{ enlargement of RA (2) Hyperpigmented skin lesions:lentigines,ephelides,
r/ prominentSVC, lVC, azygosvein bl uenevi
640 Radiology Review Manual

(3) Myxoidfibroadenomaof the breast 0 Anatomicclosuredue to subintimalfibrosis

(4) Psammomatous melanoticschwannoma + thrombosis:in 35% by 2 weeks;in 90% by
(5) Pituitaryadenoma 2 months;in 99% by 1 year
(6) Testiculartumor: largecalcifyingSertolicelltumor Hemodynamics of PDA:
(7) Primarypigmentednodularadrenocortical hyperplasia increasedvolumeof bloodflowsfrom aortathroughPDA
N.B.: not relatedto Carneytriad(pulmonary + pulmonaryarteryinto lungsand then to left sideof heart
hamartomas,extraadrenalparagangliomas, R A < - + RV <+ Main PA t
gastricleiomyosarcoma) Pulm.vessels 1
LA <-+/t LV <+lT Ao T
PAPI LLARY FI BROELASTOMA
= FIBROELEASTIC PAPILLOMA = PAPILLOMA / MYXOMA / . mostlyasymptomatic
FIBROMA OF VALVES = GIANTLAMBLEXCRESCENCE . congestiveheartfailure(rare)usuallyby 3 monthsof
= MYXOFIBROMA = HYALINE FIBROMA age if L-to-Rshuntlarge
= benignendocardialpapillomapredominantlyaffecting . continuousmurmur
cardiacvalves . boundingperipheralpulses(intraaortic pressurerunoff
Prevalence: 25'h of all cardiacvalvulartumors(most throughPDA)
commonvalvulartumor);10/" of all primary C X R (mi mi csV S D ):
cardiactumors(2ndmostcommonprimary r/ enlargedpulmonaryarterysegment
benigncardiacneoplasmafter myxoma) ./ increaseof pulmonaryvasculature;less flow directed
Meanage: 60 years; M:F = 1:1 to LU L
Cause: ? reactiveprocess,? hamartoma r/ enlargedascendingaorta + aorticarch (thymusmay
. mostlyasymptomatic(incidentalfindingat autopsy, obscurethis)
surgery,echocardiography, cardiaccatheterization) ./ m+LVenlargement
. chest pain,dyspnea,embolicevents(TlA / strokefrom { enlargedRV (onlywith pulmonaryhypertension)
thrombicollectingon tumor) { prominentductusinfundibulum (diverticulum)
. NO valvulardysfunction = prominencebetweenaorticknob + pulmonaryartery
Path: gelatinousmass with "sea anemone"appearance segment
due to multipledelicatebranchingpapillaryfronds ri obscuredaortopulmonary window
attachedto endocardiumby short pedicle r/ "railroadtrack"= calcifiedductusarteriosus
Histo: avascularpapillomacomposedof fibrouscore E C H O:
+ linedby a singlelayerof endothelium;scattered r/ tA:Ao ratio>1.2:1(signalizes significantL-to-Rshunt)
smoothmusclecellswithinpapillaryprojections A ngi o:
Location:aortic(29%)> mitral(25%)> tricuspid(17%) > r/ cathetercoursefrom RA to RV, main pulmonary
pulmonaryvalve(13%);nonvalvular endocardial artery,PDA, descendingaorta
surfaceof atrium/ ventricle(16%) { communicationfrom aorta (distalto left subclavian
Size: <1 cm in diameter(may be as largeas 5 cm) artery)to left pulmonaryarteryon AP / LAT / LAO
ECHO : aortogram
r/ <t.S cm homogeneous mobilepedunculated mass:
r/ elongatedstrandlikeprojection/ well-definedhead PDAin PrematureInfant
r/ CHRnnCTERISTIC stipplededgewith a "shimmer/ prematureinfantnot subjectto medialmuscular
vibration"at interfacebetweentumor and hypertrophyof small pulmonaryarterybranches(which
surroundingblood(DDx: amorphousthrombus) occursin normalinfantssubsequentto progressive
r/ flutters/ prolapseswith cardiacmotion hypoxiain 3rd trimester)
r/ turbulentbloodflow . CHF
Rx: surgicalexcisiont leafletrepair/ valve replacement Cause:
(a) pulmonaryarterypressureremainslow without
opposingany Lto-R shunts(PDA/ VSD)
PATENTDUCTUS
ARTERIOSUS (b) ductusarteriosusremainsopen secondaryto
= PDA = persistenceof left 6th aorticarch,which connects hypoxiain RDS
the left pulmonaryarterywith the descendingaorta { recurrenceof alveolarairspacefillingafter resolution
beyondthe originof the left subclavianartery of RDS
lncidence: 9o/"of all CHD; M:F = 1:2 { granularpatternof hyalinemembranedisease
Associatedwith: prematurity,birth asphyxia,high- becomesmore opaque
altitudebirths,rubellasyndrome, { enlargementof heart(maskedby positivepressure
coarctation, VSD,trisomy18 + 21 ventilation)
Normal ductus physiology in mature infant: Rx:
increasein arterialoxygenpressureleadsto constriction (a) Medicaltherapy:
+ closureof duct (1) supportiveoxygen,diuretics,digitalis
0 Functionalclosuredue to muscularcontraction within (2) avoidfluid overload(not to increaseshunt
10-15-48 hours volume)
 Disorders 641
Cardiovascular

(3) antiprostaglandins
= indomethacinopposes (2) Atheroma/ chronicintramuralthrombus
(low
prostaglandins,
which are potentduct dilators signalintensityon TlWl + T2Wl)
(b) Surgicalligation
PERICARDIAL
DEFECT
BeneficialPDA = failureof pericardialdevelopmentsecondaryto
= colT'tpeflsatory
effectof PDAin: prematureatrophyof the left duct of Cuvier(cardinal
1. Tetralogy of Fallot vein),whichfailsto nourishthe leftpleuropericardial
cyanosis usuallyoccursduringclosureof duct membrane
shortlyafterbirth Frequency; 1:13,000;M:F = 3:1
2. Eisenmenger pulmonary hypertension Age at detection: newbornto 81 years (mean 21 years)
PDAactsas escapevalveshuntingbloodto Location:
descending aorta A. PARTTAL ABSENCE(91%)
3, Interruptedaorticarch (a) completeabsenceon left side (35%)
supplyof lowerextremity via PDA (b) foraminaldefecton left side (35%)
(c) diaphragmaticpericardialaplasia (17%)
NonbeneficialPDA (d) foraminaldefecton rightside (4%)
in L-to-Rshunts(VSD,aortopulmonic
window)a PDA B. TOTALBILATERALABSENCE (9%)
increases shuntvolume ln 30% associated with:
(1) Bronchogenic cyst (30%)
(2) VSD, PDA, mitralstenosis
PENETRATINGAORTICULCER (3) Diaphragmatichernia,sequestration
= PENETRATING ATHEROSCLEROTIC ULCEROF THE . mostlyasymptomatic
AORTA . palpitations,tachycardia,dyspnea,dizziness,syncope
= atheromatous plaque characterized by ulceration that . positionaldiscomfortwhile lyingon left side
penetratesthe internalelasticlamina . nonspecificintermittentchest pain (lackof pericardial
Pathophysiology: cushioning, torsionof greatvessels,tensionon
atheromatousintimalplaqueprogressesto a deep pleuropericardial adhesions,pressureon coronary
atheromatousulcerthat penetratesthe elasticlamina arteriesby rim of pericardialdefect)
+ extendsinto media;hemorrhagewithinmediacauses . ECG: rightaxis deviation,rightbundlebranchblock
a communicating"double-barreled" / thrombosedaortic r/ size:
dissection;stretchingof aorticwall leadsto formationof - smallforaminaldefect = no abnormality
a saccularaorticaneurysm;aorticdissection/ aneurysm - largedefect = herniationof cardiac
may rupture structures/ lung
. may presentwith back pain completeabsence = levopositionof heart
Location: middle/ distalthirdof descendingthoracic { absenceof left pericardialfat-pad
aorla;occasionallyabdominalaorta rl levopositionof heartwith lack of visualizationof right
r/ extensiveatherosclerotic disease+ ectasia heart border
r/ tackof compressionof the aorticlumen r/ prominence / focalbulgein the areaof RVOT,main
CE CT : pulmonaryarlery,left atrialappendage
{ focallyulceratedplaque ! sharp margination+ elongationof left heartborder
r/ adjacentsubintimalhematoma(differentiation from r/ insinuationof lung betweenheart + left hemidiaphragm
intraluminal thrombus/ atherosclerotic plaquenot r/ insinuation of lungbetweenaorticknob + putmonarya.
possible): r/ increaseddistancebetweenheart + sternumsecondary
{ displacement of frequentlycalcifiedintimainwardly to absenceof sternopericardial ligament(cross-table
r/ thict<ening/ enhancementof adjacentaorticwall lateralprojection)
MR: { pneumopericardium followingpneumothorax
r/ deeplyulceratedaorticplaque ^1ruOtrachealdeviation
r/ subacutehematomain aorticwall of high signal Cx: cardiacstrangulation
intensityon TlWl + T2Wl (methemoglobin) either Rx: foraminaldefectrequiressurgerybecauseof
localizedor mimickingtype 3 dissection (a) herniation+ strangulationof left atrial
A ngio: appendage,(b) herniationof LA / LV
r/ ulceratedatherosclerotic plaque (1) Closureof defectwith pleuralflap
{ aorticwall thickening (2) Resectionof pericardium
Cx: (1) Aorticdissection(controversial)
(2) Saccularfalse aneurysmdue to incomplete PEBICARDIAL MESOTHELIOMA
rupture = malignantprimaryneoplasmarisingfrom mesothelial
(3) Spontaneouscompleteaorticrupture(40% risk cells of the pericardium
comparedwith 7"h risk in aorticdissection) lncidence: <1o/oof all mesotheliomas;50%of all primary
DDx: (1) Aorticdissection(intimalflap, patentfalse pericardialtumors
lum en ) Age: 2-78 (mean,46) years; M:F = 2:1
642 Radiology Review Manual

Path: multiplecoalescingpericardialmasseswith POLYARTERITISNODOSA

obliterationof pericardialspace;myocardial = PERIARTERITIS NODOSA = PAN
invasionis rare = systemicfibrinoidnecrotizing inflamination of medium-
Histo: biphasictumor composedof epithelialareas sized+ smallmusculararterieswithoutglomerulo-
formingtubulopapillary structures(resembling nephritisor vasculitisin arterioles,capillaries,venules
carcinoma)and spindledareas(resembling Frequency: f9 cases/million/year (rare);70 per million/
sarcoma) year in patientswith hepatitisB; M:F = 2:1
. chest pain, cough,dyspnea,palpitations Etiology: ? depositionof immunecomplexes
. signs of pericarditis,cardiactamponade Age: 18-81 (meanage,55) years
^/ irregulardiffusepericardial thickening Path: focalpanmuralnecrotizing vasculitis;mucoid
r/ cardiacencasementby soft-tissuemasses degeneration + fibrinoidnecrosisbeginswithin
r/ pericardialeffusion media;absenceof vasculitisin vesselsotherthan
CX R: arteries(DDx: necrotizingangitis,mycotic
r/ cardiacenlargementwith irregularcontour aneurysm)
^/ diffusemediastinal enlargement Histo: polymorphonuclear cell infiltratein all layersof
Rx: palliativesurgery+ radiationtherapy arterialwall + perivasculartissue(acutephase),
Prognosis; 6-1 2-monthsurvivalafter diagnosis mononuclear cell infiltrate,intimalproliferation,
thrombosis,perivascular inflammation (chronic
stage)
TERATOMA
PERICARDIAL Associatedwith: hepatitisB + HIV antigenemia
= benigngerm cell neoplasm . low-gradefever,malaise,abdominalpain,weightloss
Age: infants+ children . elevatedESR, thrombocytosis, anemia
Histo: derivativesof all 3 germ cell layers(neuroglia, . positivefor hepatitisB surfaceantigen(up to 30%)
cartilage,skeletalmuscle,liver,intestine, . positiveperinuclearANCA titers
pancreas,glandulartissue)
Location: withinpericardialsac connectedto a great Location: all organsmay be involved,kidney(70-90%),
vesselvia a pedicle;intramyocardial (rare) heart (65%),liver (50-60%),spleen (45o/"),
. respiratorydistress,cyanosis(dueto pericardial pancreas(25-35%),Gl tract,CNS
tamponade+ compressionof SVC, RA, aorticroot, PA) (cerebrovascular accident,seizure),skin
C X R:
^/ enlargedcardiomediastinal silhouette @ Kidney(involvedin 70-80-90%)
{ formedcalcifiedteeth . painlesshematuria
US: d irregularnephrogram
{ intrapericardial heterogeneous complexmultilocular r/ radiolucentcorticalareas
cysticmass: ^/ prolongedwashoutof contrastmaterial
^/ intrinsicechogenicfoci (= calcifications) ri multiplesmallintrarenalmicroaneurysms
r/ pericardialeffusion (at bifurcationof interlobar/ arcuatearteries)
r/ tetat hydrops(ascites,pleuraleffusion,subcutaneous { aneurysmsmay disappear(thrombosis) or appearin
edema,polyhydramnios) new locations
MR: { arterialnarrowing+ thrombosis(chronicstage/
./ large mass of heterogeneoussignalintensity healingstage)
Rx: emergentpericardiocentesis (life-threatening { muftiplesmallcorticalinfarcts
lesion);urgentsurgicalexcision C E C T:
Prognosis; good d lobulatedrenalcontour+ irregularthinning(dueto
priorcorticalinfarcts)
multiplehypoattenuating bands(arterialocclusion)
PERSISTENT FETAL CIRCULATION Cx: intrarenal/ subcapsularI perinephric
= PERSISTENT PULMONARY HYPERTENSION OFTHE hemorrhage(ruptureof aneurysm)
NEWBORN @ Chest (involved in 70%)
= delay in transitionfrom intra-to extrauterinepulmonary . CHF, myocardialinfarction
circulation { cardiacenlargement effusion(14%)
/ pericardial
Cause: primarydisorderrelatedto birth asphyxia, { pleuraleffusion(14%)
concurrentparenchymallung disease r/ pulmonaryvenousengorgement(21%)
(meconiumaspiration, pneumonia,pulmonary r/ massivepulmonaryedema (4%)
hemorrhage, hyalinemembranedisease, ./ lineardensitiesi platelikeatelectasis(10%)
pulmonaryhypoplasia),concurrent ./ wedge-shaped/ roundperipheralinfiltratesof
cardiovasculardisease,hypoxicmyocardial nonsegmental distribution(14%) (simulating
injury,hyperviscosity syndromes) thromboembolic diseasewith infarction)
labilePO, r/ cavitationmay occur
{ structurallynormalheart r/ interstitiallower lung field pneumonitis

@ Liver (50-66%)
{ prolongedwashoutof contrastmaterial(due to
increasein peripheralhepaticarterialresistance
+ hepaticinfarcts
@ Ot tract (50-70%)
Location: smallintestine> mesentery> colon
. abdominalpain,nausea,vomiting(66%)
{ ulcerformation,Gl bleeding(6%i
_ r/ bowelperforation(5%), intesiinaiinfarction(1.4%)
@ Skeletalmuscle(39%)
., myalgia,arthralgia(50%),limb claudication
1 aneurysmsof lumbar+ intercostal arteries(19%)
^/ lowerextremityischemia(16%)
@ Skin (20%)
. palpablepurpura,infection,ischemiculcer
. tendersubcutaneous nodules(15%)
. peripheralneuropathy(= mononeuritis multiplex)
Angiography(G1-89%sensitive,90% specific,55%ppV,
98% NPV, 80o/o true-positiverate):
r/ multiple(>10)aneurysmsof small+ medium-sized
arteriestypicallyat branchingpointsas a resultof
pannecrosisof the internalelasticfaminain 50_60%
( HA LLM A R K ):
r/ t-S mm saccularaneurysms in 60-75%
r/ fusiformaneurysms/ arierialectasia
0 Aneurysmsare found in 12-g4./"of patientswith
, polyarteritisnodosa
{ luminalirregularities (in up to 9O%)
r/ stenosesof arteries
{ arterialocclusions+ organ infarcts(gg%)
DDx: rheumatoidvasculitis,drug abuse,systemic
lupuserythematosus, Churg-Strausssyndrome
Dx: angiography,tissuebiopsy
Cx: renin-mediated hypertension, renalfailure,
hemorrhagesecondaryto aneurysmrupture(9"/"),
organinfarctiondue to vesselthrombosis,
gangreneof fingers/ toes
Prognosis: clinicalcourselastsseveralmonthsto
>1 year; relapsein 40/" with medianinterval
of 33 months;13/" S-yearsurvivalrate if
untreated
Rx: immunosuppression with corticosteroids
+ cyclophosphamide (increasesS-yearsurvivalrate
to 48-90%)
POPLITEAL ARTERY ENTRAPMENT SYNDROME
= poplitealarteryclassically windingmediallyand then
inferiorlyto the tendinousinsertionof the medialheadof
the gastrocnemius
lncidence; 35 cases in Americansurgicalliterature;
bilateralin up to 66%
Cause: anomalousdevelopment and courseof medial
head of gastrocnemiusmuscle,which attaches
to medialfemoralcondyleafterdevelopmentof
primitivepoplitealarteryin 2O-mmembryo
slingingaroundlateralaspectof popliteala.
Cardiovascular
Disorders 645
Pathophysiology:
flow unimpededwhen musclerelaxed;increasedarterial
angulationwith musclecontraction(early);progressive
intimalhyperplasia ("atheroma" = misnomer)due to
microtraumain area of repeatedarterialcompression;
ultimatelyocclusion/ thrombosiswithinaneurysm(late)
Age: <35 years in 68%; age peaks at 17 and47 years;
M : F= 9 ; 1
. slowlyprogressiveintermittentunilateralcalf
claudication (early)esp.duringperiodsof prolonged
standing
. acuteischemiaof leg with permanentocclusionof
popliteala. (late)
r/ posteriortibialpulseobliteratedduringactiveplantar
flexionagainstresistance
r/ pVn has 40% false-positiveresults
{ ankle-armindexreducedduringactivemuscle
contraction
r/ Dopplerwaveformsof posteriortibiala. diminished
duringmusclecontractions
Angio(biplanarviewswith hyperextended knee):
r/ medialdeviationof arteiy (29%),poplitealstenosis
(11"/"),poststenoticdilatation(8%)
Dx:
{ arteriographywith typicalmedialdeviationof popliteal
a. before+ aftergastrocnemiuscontraction
r/ popliteala. thrombosis/ occlusion
Cx: popliteala. aneurysm
DDx: cysticadventitialdiseaseof popliteala., arterial
embolism,prematurearteriosclerosis, popliteal
aneurysmwith thrombosis,popliteala. trauma,
popliteala. thrombosis,Buergerdisease,spinal
cord stenosis(= neurogenicclaudication)
P R IMA R Y P U LMON A R Y H Y P E R TE N S ION
= PLEXOGENIC PULMONARY ARTERIOPATHY
Diagnosis per exclusion:
clinicallyunexplainedprogressivepulmonaryarterial
hypertension withoutevidencefor thromboembolic
disease/ pulmonaryvenoocclusivedisease
At risk: portalhypertension(with/ withoutliverdisease),
collagenvasculardisease,HIV infection,
aminorexfumarate(appetitesuppressant)
ingestion
Histo: plexiform+ angiomatoidlesions= tortuous
channelswithin"glomeruloid"proliferation
of
endothelial
cells(75%);acute+ organizing
thrombi(S0%)
Age: 3rd decade; M:F = 1:3
. progressivedyspnea(60%)
. easyfatigability, syncope,angina
. hyperventilation, hemoptysis
.. Raynaudphenomenon
r/ rightventricularenlargement(dilatation+ hypertrophy)
./ dilatationof centralpulmonaryarteries
CXR:
r/ prominentcentralpulmonaryarteries:
r/ enlargedpulmonarytrunk
{ rightdescendingpulmonaryartery>25 mm wide
644 Radiology Review Manual

{ pulmonaryvascularitY: A ngi o:
r/ otigemia+ rapidlytaperingvessels rl nign positionof aorticarch
r/ overcirculation + vasculardistension ./ "figure3 sign" = flotchin descendingaorta at
CT: attachmentof short ligamentumarteriosum
{ enlargementof centralpulmonaryarteries: DDx: true coarctation,aneurysm,mediastinalmass
r/ diameterof mainpulmonaryartery> 29 mm
(87% sensitive,89% specific)measuredat scan
planeof bifurcation at rightangleto its longaxisjust ARTERYPSEUDOANEURYSM
PULMONARY
lateralto ascendingaorta = tear / disruptionof layersof vesselwall with
^/ segmentalartery-to-bronchus ratio>1:1 extravasationof bloodcontainedby adventitia/ clot /
r/ pulmonaryartery-to-aorta ratio (rPA)>1 compressedsurrounding tissue
r/ abruptlydiminishedcaliberof peripheralpulmonary Cause:
vessels(at outerto medialthird of lung mantle) A. TRAUMA
H RCT : 1. lmproperplacementof Swan-Ganzcatheter
r/ mosaicpatternof lung attenuation(due to regional 2. Blunt/ penetrating trauma
variationsin lungPerfusion): B. INFECTION:mycotic,syphilitic,mycobacterial
r/ hyperdenseareascontainlargecalibervessels C. VASCULARABNORMALITY: cysticmedialnecrosis,
r/ hypodenseareas containsmallcalibervessels BehEetdisease,MarfansYndrome
MR: D. OTHER: sePticemboli,neoPlasm
{ reversalof interventricular septalcurvature ' hemoptysis(= leakageof blood into bronchialtree)
r/ directIinearcorrelationbetweenmeanpulmonary CXR:
arterypressure(PAP)and ratioof main pulmonary r/ stable/ increasingfocallung mass
arterycaliberto descendingaorta (MPA/AO) CT:
{ abnormalintravascular signal(dueto slow arterial ! enhancingroundlung massisointenseto central
flow) in 92io on gated SE images pulmonarYarterY
NUC: Cx: 100%mortalitYwith ruPture
r/ normal/ low-probability V/Q scans
Angio:
r/ symmetricallyenlargedcentralarteries
r/ diffusepatternof abruptlytapering+ pruned ATRESIA
PULMONARY
subsegmental vessels = CONGENITAL ABSENCE OF PULMONARY ARTERY
r/ filamentous/ "corkscrew"peripheralarteries = atreticpulmonaryvalvewith underdeveloped pulmonary
r/ subpleuralcollaterals(occasionally) arterydistallY
Prognosis; death in 2-5 Years May be associatedwith: hypogeneticlung
Rx: vasodilators, calciumchannelblockers,diuretics, CXR:
anticoagulants; lung/ heart-lung transplantation ! smallhemithoraxof normalradiodensity
r/ mediastinalshiftto affectedside
PSEUDOCOARCTATION r/ elevationof ipsilateraldiaphragm
= A O RT I CK I NK I N G r/ reticularnetworkof vesselson affectedside (due to
= elongatedredundantthoracicaortawith acute kink / systemiccollateralcirculationfrom bronchialarteries)
anteriorbucklingjust distalto originof leftsubclavian { rib notchingfrom prominenceof intercostalarteries
arteryat lig. arteriosum (dueto largetranspleural collateralvessels)
= vdriantof coarctationwithouta pressuregradient OB -U S :
Age: 12-64 years r/ small/ enlarged/ normalrightventricle
Associated with: ^/ progressiveatrialenlargement(tricuspidregurgitation)
hypertension, bicuspidaorticvalve,PDA,VSD, aortici r/ flow reversalin ductusarteriosus+ main pulmonary
subaorticstenosis,singleventricle,ASD, anomaliesof artery(most reliable)
aorticarch branches
. asymptomatic
. ejectionmurmur PulmonaryAtresiawith lntactInterventricular
. NO pressuregradientacrossthe buckledsegment Septum
r/ anteromedialdeviationof aorta Associatedwith: ASD (R-to-Lshunt)
{ "chimney-shaped" high aorticarch (in children) Type | : no remainingRV, no tricuspidregurgitation
r/ rounded i oval soft-tissue mass in left paratracheal { moderatelyenlargedRA (dependingon
region+ superiorto presumednormallypositionedaortic size of ASD)
arch [secondaryto elongationof ascendingaorta Type tl : normalRV with tricuspidregurgitation
+ aorticarchl (in adults) r/ massiveenlargementof RA
{ anteriordisplacement of esophagus r/ cardiomegaly(LV, RA)
^/ lttO rib notchingi dilatationof brachiocephalic arteries/ { concave/ small pulmonaryarterysegment
LV enlargement/ poststenoticdilatation r/ diminisrredpulmonaryvascularity
 CardiovascularDisorders 645

PULMONARY
VENOOCCLUSIVE
DISEASE PULMONIC STENOSIS
= fibrousnarrowingof intrapulmonary veins;the Frequency: pulmonary arterystenosiswithout VSD in
postcapillarycounterpartof primarypulmonary 8./"of all CHD
hypertension Embryology: infundibulumformedfrom proximalportion
Cause: idiopathic(rarecondition);venousthrombosis of bulbiscordis;pulmonaryvalvesdevelop
initiatedby infection/ toxicexposure/ immune in 6-9th week from outgrowthof 3 tubercles
complexdeposition . mostlyasymptomatic
May be associated with: . cyanosis/ heartfailure
pregnancy,transplantation, drug toxicity(carmustine, ., loudsystolicejectionmurmur
bleomycin,mitomycin) ri systolicdomingof pulmonaryvalve(= incomplete
Hemodynamics: openi ng)
. elevatedpressurein rightatrium+ pulmonaryartery
r/ normal/ diminishedi increasedpulmonaryvascularity
. decreasedcardiacoutput
(dependingon presence+ natureof associated
. normal/ variablyelevatedcapillarywedge pressures
malformations)
' normalpressurein left atrium+ left ventricle(excludes
ri enlargedpulmonarytrunk+ leftpulmonaryartery
cardiacdiseaseas the causefor venoushypertension) (poststenoticdilatation)
r/ prominentleftpulmonaryartery+ normalright
Age: children(39%),adolescents;M:F = 1:1
Histo: , pulmonaryartery
r/ hypertrophyof RV with reducedsize of RV chamber:
(a) specificchanges: webs, recanalizedthrombus(in up r/ elevationof cardiacapex
to 95%),intimalfibrosisof pulmonaryveins; ^/ increasedconvexityof anteriorcardiacborderon LAO
"capillary
hemangiomatosis" = sheetsand nodular r/ diminutionof retrosternalclear space
collectionsof thin-walledcapillariesinvading r/ cor pulmonale
pulmonaryarteries+ veins+ bronchioles + pleura { mildenlargement of LA (reasonunknown)
(b) nonspecificchangesof venoushypertension: ! calcificationof pulmonaryvalvesin older adults (rare)
venousmedialhypertrophy, septaledema+ fibrosis, Prognosis; death at mean age of 21 years if untreated
paraseptalvenousinfarction, interstitial
+ pleural
lymphaticdilatation,intraalveolarhemosiderin-laden SubvalvularPulmonicStenosis
macrophages A . IN FU N D IB U LAP
. progressivedyspnea,hemoptysis RU LMON ICS TE N OS IS
. antecedentflulikesymptoms typicallyin tetralogyof Faflot
B. SUBINFUNDIBULA PRULMONIC STENOSIS
C X R: = hypertrophied anomalousmusclebundles
{ pulmonaryarterialhypertension crossingportionsof RV
r/ diffuseinterstitialpulmonaryedema Associatedwith: VSD (73-BS%)
r/ normal-sizedleft atrium
(a) low type: coursesdiagonallyfrom low anterior
r/ mediastinallymphadenopathy
septalside to cristaposteriorly
CT: (b) hightype: horizontaldefectacrossRV below
d markedlysmallcentralpulmonaryveins
i nfundi bul um
! centraland gravity-dependent parenchymalground_ r/ no dilatationof PA becauseof dissipationof RV force
glass attenuation
throughelongatedarea of obstruction
r/ smoothlythickenedinterlobularsepta
r/ pleuraleffusions
r/ normal-sizedleft atrium
ValvularPulmonicStenosis
1. C LA S S IC/ TY P IC A LP U LMON ICV A LV E
r/ centrilobular nodules
STENOS|S(e5%)
NUC:
= corTlrTlissuralfusionof pulmonarycusps
r/ patchydistributionof Tc-99mMAA (of ,,upstream,,
Age of presentation: childhood
pulmonaryarterialhypertension) . pul moni ccl i ck
Angio: . ECG: hypertrophy
r/ enlargedrightventricle+ centralpulmonaryarteries of RV
r/ tfrinmobiledome-shaped valve
! prolongedparenchymal phaseenhancement
ri let of contrastthrougl'rsmall centralorifice
! delayedfillingof normalpulmonaryveins .i ditateOmain + left pulmonaryartery
r/ normalto small left atrium
Rx: balloonvalvuloplasty
2. DySpLASTtCPULMONICVALVESTENOSTS (s%)
Prognosis; death within 3 years (no effectivetherapy)
= thickenedredundantdistortedcusps,immobile
Cx: potentiallyfatal pulmonaryedemafollowing
secondaryto myxomatoustissue
administration of vasodilatorsfor presumed . NO click
precapillarypulmonaryhypertension
r/ trlOpoststenoticdilatation
Dx: often missedinitiafly(clinicalpresentation
Rx: surgicalresectionof redundantvalve tissue
+ radiographic findingsmimicinterstitial lung Hemodynamics: obstructionof RV systolicejection
disease)
with pressureburdenon RV
646 Radiology Review Manual

RA ++II RV T MainPA t Cause: ? acquiredadrenoreceptorhypersensitivity

Pulm.vessels <+ LPA I May be associatedwith: reflex sympatheticdystrophy,
L A < + L V e Ao e) early stagesof autoimmune
CX R: disorders
{ normalpulmonaryvascularity Age: mostcommonin youngwomen
^/ normal-sizedheart . usuallyaffectsall fingersof both handsequally
A ngio: { normalsegmentalarm + digitpressuresat room
{ increasein trabecularpatternof RV temperature
(lateral
r/ hypertrophiedcristasupraventricularis { peakeddigitvolumepulse= raPidrisein systole,
projection) anacroticnotchjust beforethe peak,dicroticnotch
highon the downslope
PulmonicStenosis
Supravalvular PPG:
60% of all pulmonicvalve stenoses ./ flat-linetracingat low temperatures(10'-22'C) with
Siteof narrowing:pulmonarytrunk,pulmonary suddenreappearanceof normalwaveformat
bifurcation,one / both main 2+26C = "thresholdphenomenon"
pulmonaryarteries,lobarpulmonary
artery,segmentalpulmonaryartery RaynaudPhenomenon
Shapeof narrowing: = SECONDARY VASOSPASM WITHOBSTRUCTION
(a) localizedwith poststenoticdilatation = OBSTRUCTIVE FORMOF RAYNAUD SYNDROME
(b) longtubularhypoplasia = digitalarteryocclusiondue to stenoticprocessin
May be associated with: normallyconstrictingartery/ associatedwith an
(1) Valvularpulmonicstenosis,supravalvular aortic abnormallyhighbloodviscositY
stenosis,VSD, PDA, systemicarterialstenoses Cause:
(2) Familialperipheralpulmonicstenoses 1. Atherosclerosis (mostfrequent)
+ supravalvularaorticstenosis (a) embolization from an upstreamlesion
(3) Williams-Beuren syndrome:PS, supravalvular (b) occlusionof majorarteriessupplyingarm
AS, peculiarfacies 2. Arterialtrauma
(4) Ehlers-Danlos syndrome 3. End stageof manyautoimmunedisorders:
(5) Postrubellasyndrome:peripheralpulmonic eg, scleroderma, rheumatoidarthritis,systemic
stenoses,valvularpulmonicstenosis,PDA,low lupuserythematosus
birthweight,deafness,cataract,mentalretardation 4. Takayasudisease
(6) Tetralogyof Fallot/ criticalvalvularpulmonic 5. Buergerdisease
stenosis 6. Drugintoxication (ergot,methysergide)
7. Dysproteinemia
PeripheralPulmonaryArteryStenosis 8. Primarypulmonaryhypertension
Frequency: 5o/"of all pulmonaryarterystenoseswith 9. Myxedema
an intactventricularseptum . normalvasoconstrictive responseto cold
./ reducedsegmentalarm + digit pressuresat room
temperature
RAYNAUD SYNDROME PPG (76%sensitivity, :
92/" specificity)
= episodicdigitalischemiain responseto cold/ emotional r/ flat-lineI barelydetectabletracingat low
s t im uli temperaturewith gradualincreaseof amplitude
Pathogenesis: upon rewarming
(1) increasein vasoconstrictortone Hand magnification angiography:
(2) low blood pressure 1. Baselineangiogramwith ambienttemperature
(3) slightincreasein bloodviscosity 2. Stressangiogramimmediately following
(4) immunologic factors(4-81%) immersionof hand in ice water for 20 seconds
(5) cold provocation
. exaggeratedresponseof digitto cold / emotionalstress:
. numbness+ loss of tactileperception RHABDOMYOMA OF HEART
. demarcatedpallor/ cyanosis = benignmyocardialhamartoma
. hyperemicthrobbingduringrewarming Prevalence: most commoncardiactumor in infancy
. sclerodactyly + childhood(up to 90%)
. smallpainfululcersat tip of digit Age: usuallydiscovered<1 year of age
intramurallobulatednodule/
Path: well-circumscribed
RaynaudDisease multiple<1 mm nodules(= rhabdomyomatosis)
= PRIMARYVASOSPASM= SPASTICFORM OF RAYNAUD Histo: "spidercells"= enlargedvacuolatedcells with
SYNDROME highglycogencontent+ centralnucleus
= eX?gget'atedcold-induced constriction of smooth surroundedby clear cytoplasmand radial
muscle cells in otherwise normal artery extensions
 Cardiovascular
Disorders 647

Associatedwith: tuberoussclerosis(in 50_g6%); Site: rightsinus/ noncoronarysinus(>90%)

congenitalheartdisease 0 Rightsinususuallyrupturesinto RV, occasionally
. asymptomatic(incidentaldetectionat prenatal
US / into RA
screening) 0 Noncoronary
. murmur,arrhythmia sinusrupturesinto RA
. suddenretrosternal pain,dyspnea,continuousmurmur
' heartfailure(secondaryto obstructionof outflow
tract/ r/ shunt vascularity
reductionof enddiastolic volume/ decreased r/ cardiomegaly
contractility) r/ prominentascendingaorta
. supraventricular tachycardia(accessoryconductive
pathwayswithintumor)
S P LE N IC A R TE R Y A N E U R Y S M
Location: usuallymultiple;ventricular wall with = ffioStfrequentof visceralarteryaneurysms
intramuralgrowth+ tendencyto involve Etiology: medialdegenerationwith superimposed
interventricular
septum;atrialwall (rare) atherosclerosis,congenital,mycotic,
Size: up to 10 cm in diameter(average3-4 cm)
pancreatitis,trauma,portalhypertension
US (goodfor smallintramurallesions): (7-10% of casesdue to highflow rate)
r/ tetalnonimmunehydrops Predisposed: women with )2 pregnancies(gg%)
r/ solid echogenicseisile mass t intracavitary May be associatedwith: fibromusculardisease (in 2O%)
componentbulgingintoventricularoutflowtract/ M : F= 1 : 2
atrioventricularvalve . usuallyasymptomatic
r/ diffusemyocardialthickening(withmultiplesmall . pai n,Gl bl eedi ng
lesions) Location: intra-/ extrasplenic
MR (complimentary to US): r/ calcifiedwall of aneurysm(2/3)
^/ tumorisointenseon TlWl + hyperintense to Cx: ruptureof aneurysm(6-g%, higherduring
myocardiumon T2Wl pregnancy)especiallyif >1.5cm in diameter
Prognosisi may regressspontaneouslyin patients Mortality: up to 76%
<4 years of age DDx: renalarteryaneurysm,tortuoussplenicartery
Rx: surgicalexcisionfor life-threatening symptoms
DDx: fibroma(solitarycentrallycalcified+ cystictumor,in
SUBCLAVIAN
STEALSYNDROME
ventricularmyocardium, associatedwith Gorlin = stenosis/ obstructionof subclavianarterynear its origin
syndrome),teratoma(singleintrapericardial
with flow reversalin ipsilateralvertebralarteryat the
multicysticmass),hemangioma(arisefrom RT
expenseof the cerebralcirculation
atrium,pericardial effusion,skin hemangiomas) lncidence: 2.5o/"of all extracranialarterialocclusions
Etiology:
(a) congenital:interruption of aorticarch,preductal
infantilecoarctation,hypoplasiaof left aorticarch,
SIN G LE V E NT RI C L E
= UNI V E NT RI C U LHAERAR T hypoplasia/ atresia/ stenosisof an anomalousleft
= D OU BL IN
E L ETSIN GLE
VENTRICLE subclavianarterywith rightaorticarch,coarctation
= failureof development with aberrantsubclavianarteryarisingdistalto the
of interventricular
septum
+ absenceof one atrioventricular coarctation
valve (mitral/ tricuspid
(b) acquired:atherosclerosis (94%),dissecting
atresia)+ aortic/ pulmonicstenosis
Associatedwith: TGV or DORV aneurysm,chesttrauma,embolism,tumor
. conductiondefect (aberrantanatomyof conduction thrombosis,inflammatoryarteritis(Takayasu,
system) syphilitic),ligationof subclavianarteryin Blalock-
r/ two atrioventricular Taussigshunt,complicationof coarctationrepair,
valvesconnectedto a main
ventricularchamber radiationfibrosis
r/ tne singleventriclemay be a LV (g5%)/ RV / Age: average59-61 years; M:F = 3:1;
undetermined Whites:Blacks = 8:2
r/ a secondrudimentaryventricularchambermay be Associatedwith: additionallesionsof extracranial
present,which is locatedanteriorly(in left univentricle) arteriesin 81"/"
/ . lowersystolicblood pressureby >20-40 mm Hg on
posteriorly(in rightuniventricle)
:
ri rudimentarychamber+ connectionto one great artery affectedside
, . delayedweak / absentpulse in ipsilateralextremity
r/ may be associatedwith tricuspid/ mitralatresia
. signs of vertebrobasilar insufficien
cy (40%):
. syncopalepisodesinitiatedby exercisingthe ischemic
SINUS OF VALSALVA ANEURYSM arm
. headaches,nausea,vertigo,ataxia
= deficiencybetweenaorticmedia+ annulusfibrosisof
. mono-,hemi-,para-,quadriparesis, paralysis
aorticvalve resultingin distension+ eventualaneurysm . diplopia,dysphagia,dysarthria,paresthesiasaround
formation
mouth
Age: pubertyto 30 years of age . uni-/ bilateralhomonymoushemianopia
648 Radiology Review Manual

. signsof brachialinsufficiency (3-10%): . with malignancy:rapidprogression withinweeks,

. intermittent/ constantpain in affectedarm mostlymales, 40-60 Years of age
precipitatedby increasedactivityof that arm . proptosis,tearing
. paresthesia, weakness,coolness,numbness,burning . dyspnea,cyanosis,chestPain
in fingers+ hand . hematemesis (11%)
. fingertipnecrosis { superior mediastinalwidening$a%)
Location: L:R = 3:1 { encasement/ compression / occlusionof SVC
ColorDoppler: r/ ditatedcervical+ superficialthoracicveins (80%)
^/ reversalof vertebralartery flow, augmentedby ^/ svc thrombus
reactivehyperemia(bloodpressurecuff inflated NUC:
above systolicpressurefor 5 minutes)/ arm exercise r/ increasedtraceruptakein quadratelobe + posterior
A ngio: aspectof medialsegmentof leftlobe (umbilical
{ subclavianstenosis/ occlusion(aorticarch injection) pathwaytoward liverwhen injectedin upper
^/ reversalof vertebralarteryflow (selectiveinjectionof extremity)
contralateralsubclavian/ vertebralartery)
"falsesteal"= transientretrogradeflow in
CAVE:
contralateralvertebralarterycausedby
high-pressure injection SYPHILITIC AORTITIS
Rx: bypasssurgery,PTA (goodlong-termresults) = LUETIC AORTTTIS
lncidence: in 10-15/" of untreatedpatients(accounts
for deathin 1/3)
PartialSubclavianStealSyndrome Path: periaortitis(via lymphatics),mesaortitis(viavasa
= retrogradeflow in systole+ antegradeflow in diastole vasorum)= primarilydiseaseof medialeadingto
secondaryinjuryof intima,whichpredisposes the
intimato prematurecalcificatherosclerosis
Age: between40 and 65 Years
OccultSubclavianStealSYndrome
= reV€fseflow seen only after provocativemaneuvers, Site: ascendingaorta (36%),aortic arch (24o/"),
descendingaorta(5%),sinusof Valsalva(1"/"),
arm exerciseof 5 minutes/ 5 minutes
ie, ipsilateral
> systolicblood PulmonarY arterY
inflationof sphygmomanometer
pressurelevels r/ tnicf aorticwall (fibrous+ inflammatorytissue)
^/ saccular(75%)i fusiform(25/") dilatationof ascending
aorta
{ small saccularaneurysmsoften protrudefrom fusiform
SUPERIOR VENA CAVA SYNDROME
aneurysm
= obstructionof SVC with developmentof collateral
^/ tine pencil-like calcificationsof intima(15-20%)in
pathways late in disease
ascending aorta,
Etiology:
Cx: (1) stenosisof coronaryostia(intimalthickening)
(a) Malignantlesion(80-90%) (2) aorticregurgitation(syphiliticvalvulitis),rare
1. Bronchogenic carcinoma(>50%)
DDx: degenerativecalcificationof ascendingaorta (older
2. Lymphoma population, no aneurysm,no aorticregurgitation)
(b) Benignlesion
1. Granulomatous mediastinitis(usually
histoplasmosis, sarcoidosis,TB)
2. Substernalgoiter TAKAYASUARTERITIS
3. Ascendingaorticaneurysm = PULSELESS DISEASE = AORTITIS SYNDROME
4. Pacerwires/ centralvenouscatheters(23%) = AORTOARTERITIS = IDIOPATHIC MEDIALAORTOPATHY
5. ConstrictivePericarditis = AORTIC ARCHSYNDROME
= granulomatous inflammation of unknownpathogenesis
Collateral routes:
1. Esophageal venousplexus=
"downhillvarices" affectingsegmentsof aorta + majoraorticbranches
(predominantly uPPer 213) + pulmonaryarterieslimitedto personsusually<50
2. Azygos+ hemiazYgosveins years of age
3. Accessoryhemiazygos+ superiorintercostalveins 0 The only form of aortitisthat producesstenosis/
= "aorticnipple"(visualization
in normalpopulationin occlusionof the aorta!
5%) Etiology: probablycell-mediatedinflammation
4. Lateralthoracicveins + umbilicalvein Incidence: 2.6 new cases/million/year;
5. Vertebralveins 2.2/" (at autoPsY)
. head and neck edema (70%) Age: 12-66years; M:F = 1:8; especiallyin Orientals
. cutaneousenlargedvenouscollaterals Histo: (a) Acute stage: granulomatousinfiltrativeprocess
. headache,dizziness,syncope focusedon elasticfibersof mediaof arterialwall
. with benignetiology: sloweronset + progression,both consistingof multinucleated giantcells,
lymphocytes, histiocytes,plasmacells
sexes, 2540 years of age
 Cardiovascular
Disorders 649

(b) Fibroticstage (weeksto years): progressive @ Pulmonaryarteries(50-80%)

fibrosisof vesselwall resultingin constriction r/ dilatationof pulmonarytrunk(19%)
from intimalproliferation/ thromboticocclusion/ { nodularthrombi(3%)
aneurysmformation(fromextensivedestruction r/ "prunedtree"appearanceof pulmonaryarteries(OO%)
of elasticfibersin the media);ultimatelyleadsto r/ systemic-pulmonary arteryshunts
fibrosisof intima+ adventitia CXR:
0 Morphologically indistinguishable from temporal r/ widenedsupracardiacshadow>3.0 cm
arteritis! r/ wavy i scallopedappearanceof lateralmarginof
. prepulseless/ systemicphaseof a few monthsto a year
= Fronspecific , descendingaorta
systemicsigns + symptomsof fever,night ri aorticcalcification(15%)commonlyin aorticarch
sweats,weakness,weightloss, myalgia,arthralgia + descendingaorta
0 Mean intervalof 8 years betweenonset of symptoms ri focaldecreaseof pulmonaryvascularity
and diagnosis Cx: (1) Cerebrovascular accidents
. pulselessphase = signs + symptomsof ischemiaof limb (2) Heartfailuredue to aorticregurgitation
(claudication,pulsedeficit,bruits)+ renovascular DDx: atherosclerosis, temporalarteritis(CCA not
hypertension involved),fibromuscular dysplasia(in ICA not
. erythrocytesedimentationrate (ESR)>20 mm/hourin CCA),idiopathiccarotiddissection(lCA),syphilitic
80% aortitis(calcification
of ascendingaorta)
Location: Rx: steroids,angioplastyafter declineof active
TypeI classicpulselesstype = brachiocephatic inflammation
trunk + carotidarteries+ subclavianarteries
Typell combination of type | + lll
Typelll atvpicalcoarctationtype = thoracicand TEMPORAL AHTERITIS
= CRANIAL / GRANULOMATOUS ARTERITIS
abdominalaorta distalto arch + its major = POLYMYALGIA RHEUMATICA = GIANTCELLARTERITIS
branches (poorchoicebecause Takayasu diseaseis alsoa giantcell
TypelV dilatedtype = extensivedilatationof the arteritis)
lengthof the aorta + its branches = systemicgranulomatous vasculitislimitedto persons
Commonlyinvolved: left subclavianartery(<50%),left usually>50 years of age
commoncarotidartery(20%),brachiocephalic trunk, lncidence: 1.7 new cases lmillionlyear
renalarteries,celiactrunk,superiormesentericartery, Histo:
pulmonaryarteries(>50%) (a) acutestage: granulomatousinfiltrativeprocess
lnfrequentlyinvolved:axillary,brachial,vertebral,iliac focusedon elasticfibersof arterialwall consistingof
arteries(usuallybilaterally),coronaryarteries multinucleated giantcells,lymphocytes, histiocytes,
plasmacells
Angiography: (b) fibroticstage (weeksto years): progressivefibrosis
difficultcatheterization I risk of ischemiccomplications of vesselwall resultingin constrictionfrom intimal
, (increase in coagulation) proliferation/ thromboticocclusion/ aneurysm
r/ arterialwall thickening+ contrastenhancement formation
^l tutt-tnicXness calcification(chronicdisease) 0 Morphologically indistinguishablefrom Takayasu
r/ muralthrombi arteritis!
@ Aorta Age peak; 65-75 years; M:F = 1;3
r/ long+ diffuse/ short+ segmentalirregularstenosis/ . prodromalphaseof flulikeillnessof 1-3 weeks:
occlusionof majorbranchesof aorta near their . malaise,low-gradefever,weight loss,myalgia
origins . unilateralheadache(50-90%)
{ stenoticlesionsof descendingthoracicaorta > . chronicstaoe:
abdominalaorta . jaw claudication (whilechewing+ talking)
r/ frequentskippedlesions . palpabletendertemporalaftery
./ abundantcollateralization (latephase) . neuroophthalmic manifestations: visualimpairment /
./ aneurysmaldilatationof ascendingaorta + arch diplopia/ blindness
= diffuselydilatedlumenwith irregularcontours . polymyalgia rheumatica(50%)= intensemyalgiaof
{ fusiform/ saccularaorticaneurysms(10-15%) shoulder+ hip girdles
(commonin descendingthoracic+ abdominalaorta) . erythrocytesedimentationrate (ESR)of 40-1 40 mml
@ Brachiocephalic arteries hour (HALLMARK)
r/ multisegmented dilatationof carotidarteryproducing Location: any arteryof the body; mainlymedium-sized
, segmentalsepta branchesof aorticarch (10%),externalcarotid
r/ diffusehomogeneous circumferentialthickeningof arterybranches(particularly temporalartery);
vesselwall in proximalcommoncarotidartery extracranialarteriesbelow neck (9%):
r/ increasein flow velocity+ turbulence subclavian> axillary> brachial> profunda
./ distatCCA, lCA, ECA sparedwith dampened femoris> forearm> calf; commonlybilateral
waveforms + symmetric
650 RadiologyReviewManual

r/ long smoothstenoticarterialsegmentswith skip areas r/ coeur en sabot (boot-shapedheart)= enlargementof

r/ smoothtaperedocclusionswith abundanceof collateral rightventricle
supPly { pronouncedconcavityin regionof pulmonaryartery
r/ absenceof atherosclerotic changes trunk(small/ absentPA)
r/ aorticroot dilatation+ aorticvalve insufficiency r/ markedreductionin caliber+ numberof pulmonary
Dx: biopsyof palpabletemporalartery vessels:
Prognosis; diseasemay be self-limiting(1-2 years); ./ asymmetricpulmonaryvascularity
107" mortalitywithin2-3 years { reticularpatternwith horizontalcourseusuallyin
periphery(= prominentcollateralcirculation of
bronchialvessels+ pleuropulmonary connections)
OF FALLOT
TETRALOGY { enlargedaorta
= underdevelopment of pulmonaryinfundibulum r/ right-sidedaortic archin 25/"
secondaryto unequalpartitioning of the conotruncus OB -U S :
Frequency: 8ohof all CHD; most commonCHD with .i ditateOaorta overridingthe interventricular septum
cyanosisafter 1 year of life { usuallyperimembranous VSD
TETRAD: { mildlystenoticRV outflowtract
1. Obstruction outflowtract: usually
of rightventricular ^/ ruORV hypertrophyin midtrimester
of pulmonaryinfundibulum, occasionally of pulmonic E C H O:
valve r/ discontinuitybetweenanterioraorticwall
2. LargeVSD immediatelybelowaorticvalve + interventricular septum(= overridingof the aorta)
3. Rightventricularhypertrophysecondaryto elevated { small left atrium
RV systolicpressure ./ nV hypertrophywith small rightventricularoutflow
4. Overridingaortastraddlingthe VSD and receiving tract
bloodfrom both ventricles { wideningof the aorta
Embryology: r/ thickeningof rightventricular wall + interventricular
abnormalspiralingcaudadgrowthof truncoconalridges septum
in 3rd-4thweek causingunequalpartitioning of the Prognosis; spontaneoussurvivalwithoutsurgical
conotruncusintoa smallunderdeveloped anteromedial correcti oni n 50Y "up to ageT;i n 1O / "up t o
pulmonaryinfundibulum + largeposterolateral LV age 21
outflow tract Rx: surgeryin earlychildhood
Hemodynamics: (a) palliative
fetus : pulmonarybloodflow suppliedby 1. Blalock-Taussig shunt= end-to-side
retrogradeflow throughductusarteriosus anastomosisof subclavianto pulmonary
with absenceof RV hypertrophy/ IUGR arteryoppositeaorticarch (64% survivalrate
neonate: R-to-Lshuntbypassingpulmonary at 15 years, 55/" at 20 Years)
circulationwith decreasein systemic 2. Pott operationon left = dnastomosisof left
oxygensaturation(cyanosis); pressure PA with descendingaorta
overload+ hypertrophyof RV secondaryto 3. Waterston-Cooley procedure= anastomosis
pulmonic-inf undibular stenosis betweenascendingaorta + rightpulmonary
RA <+II RV T Main PA .1,
I
artery
Pulm.vessels J LPA t 4. Centralshunt= Rastelliprocedure= tubular
L A € L V < _ > A o 1 syntheticgraft betweenascendingaorta
+ pulmonaryarterY
Associated with: (b) correctiveopen cardiacsurgery= VSD-closure
1. Bicuspidpulmonicvalve(40%) + reconstruction of RV outflowtract by excision
2. Stenosisof left pulmonaryartery(40%) of obstructingtissue(82% survivalrate at
3. Rightaorticarch (25%) 15 years)
4. TE fistula Operativemortality: 3-10%
5. Down syndrome
6. Forkedribs,scoliosis PinkTetralogy
7. Anomaliesof coronaryarteriesin 10% (singleRCA/ = infundibular in VSD (3%)
hypertrophy
LAD from RCA)
. cyanosisby 3-4 monthsof age (concealedat birth by Pentalogy of Fallot
PDA) = tetralogy+ ASD
. dyspneaon exertion,clubbingof fingersand toes
. "squattingposition"when fatigued(increasespulmonary Trilogy of Fallot (infantilepresentation)
bloodflow) (1) severepulmonicvalvularstenosis
. "episodicspells"= lossof consciousness (2) hypertrophyof RV
. polycythemia, loweredPO, values,systolicmurmurin (3) ASD with Rto-L shunt (increasedpressurein RA
pulmonicarea forcesforamenovale open)
 Cardiovascular
Disorders 65l

THORACIC
OUTLETSYNDROME
= coffipressionof nerves,veins,and arteriesbetween
chest and arm
Cause:
A . CO NG E N IT AL
1. Cervicalrib
2. Scalenusminimusmuscle(rare)
extendingfrom transverseprocessof 7th cervical
vertebrato 1st rib with insertionbetweenbrachial
plexus+ subclavianartery
3. Anteriorscalenemuscle= scalenusanticus
syndrome(mostcommon)= widei abnormal
insertioni hypertrophyof muscle
4. Anomalous1st rib = uflUSU€llly straightcourse
with narrowingof costoclavicular space
B . A CQ UI RE D
1. Muscularbody habitus
= arterialcompressionin pectoralisminortunnel
2. Slenderbody habitus
with long neck,saggingshoulders
3. Fractureof clavicle/ 1st rib (94%)
with nonanatomic alignment/ exuberantcallus
4. Supraclavicular tumor/ lymphadenopathy
' pain in forearm+ hand that increasesupon
elevationof
arm
. paresthesias
of hand+ fingers(numbness,,,pinsand
needles")in g5/"
. decreasedskin temperature,discoloration hand
of
. intermittentclaudicationof fingers(fromischemia)
. hyperabductionmaReuverwith obliteration radial
of
pulse (34%)
. Raynaudphenomenon(0%| episodicconstriction
of
smallvessels
. supraclavicular bruit (15-30%)
Bidirectional
Doppler:
1. Adsonmaneuver(forscalenusanticusmuscle)
= holddeep inspiration whileneck is fullyextended
+ head turned toward ipsilateraland oppositeside
2. Costoclavicular maneuver(compressionbetween
clavicle+ 1st rib) = exaggeratedmilitarypositionwith
shouldersdrawn back and downward
3. Hyperabduction maneuver(compression by humeral
head/ pectoralisminor muscle)= extremity
monitoredthroughrangeof 1g0" abduction
{ completecessationof flow in one position
Photoplethysmog raphy:
1. Photopulsetransducersecuredto palmarsurfaceof
one fingertipof each hand
2. Arterialpulsationsrecordedwith arm in
(a) neutralposition
(b) extended90" to side
(c) 180' overthe head
(d) in "military"positionwith arms at 90. + shoulders
pressedback
,
! completedisappearanceof pulse in one position
Angio:
{ abnormalcourseof distalsubclavianartery
r/ focal stenosis/ occlusion
{ poststenoticdilatationof distalsubclavianartery
{ aneurysm
ri stresstest: bandlike/ concentricconstriction
{ muralthrombus+ distalembolization
^/ venousthrombosis/ obstruction
DDx: Cervicaldisk disease,radiculopathy,
spinalcord
tumor,traumato brachialplexus,arthritis,carpal
tunnelsyndrome,Pancoasttumor,peripheral
arterialocclusivedisease,aneurysm,causalgia,
thromboembolism, Raynauddisease,vasculitis
TRANSPOSITION OF GREATARTERIES
CompleteTranspositionof GreatArteries
= TGA= D-TRANSPOSITION
= greatvesselsoriginatefrom inappropriateventricle:
(1) aortaoriginating from RV with an infundibulum
(2) pulmonaryarteryoriginatingfrom LV
(3) normalpositionof atria + ventricles
Embryology:
failureof the aorticopulmonary septum(= truncoconal
ridges)to followa spiralcourse
lncidence: 10% of all CHD
VARIATIONS:
1. CompleteTGA + intactinterventricular septum
2. CompleteTGA + VSD: CHF due to vsD

ilvl,T,$"WR 3. CompleteTGA + VSD + pS: pS preventsCHF

= longestsurvival
Hemodynamics:
fetus : no hemodynamic compromisewith
double outlet RV anterior
normalbirthweight
(type II) = neonate: mixingof the 2 independent
Taussig-Bing anomaly circulations
necessaryfor survival
l-transposition Admixtureof bloodfrom both circulationsvia:
d-transposition = coffected (1) PDA (carriesaorticbloodintopulmonaryartery)
= complete primitive + patentforamenovale (allowssaturatedblood
truncus to enter RA from LA)
Prognosis.' worst when pDA closes
Variationsof Transpositions (2) vSD (in 50%)
652 Radiology Review Manual

R A l RV 1 Main PA <+ Associated with:

Pulm.vessels 1 (1) usuallyperimembranous VSD (in >50%)
LA <.) L V < - > A o < + (2) pulmonicstenosis(in 50%)
(3) anomalyof left (= tricuspid)atrioventricular valves
. cyanosis(mostcommoncausefor cyanosisin (Ebstein-like)
neonate)2nd most commoncause of cyanosisafter (4) dextrocardia (highincidence)
tetralogyof Fallot . NO cyanosis
. symptomatic1-2 weeks followingbirth . atrioventricular block(malalignment of atrial
CX R: + ventricularsepta)
{ "egg-on-its-side" appearanceof heart= I]€lllow CXR:
superiormediastinumsecondaryto hypoplastic r/ abnormalconvexity/ straightening in upperportion
thymus + hyperaeration+ abnormalrelationshipof of left heart border(ascendingaorta arisingfrom
greatvessels invertedRV)
r/ cardiacenlargementbeginning2 weeks after birth ri inapparentaorticknob + descendingaorta
{ rightheartenlargement (overlyingspine)
r/ enlargementof LA (withVSD) r/ inapparentpulmonarytrunk (rightwardposterior
{ absentpulmonarytrunk (99%)= PA located = P R E MIE RS IGN
posi ti on)
posteriorlyin midline r/ humpedcontourof lower left heartborderwith
^/ increasedpulmonarybloodflow (if not associated elevationabovediaphragm(anatomicRV)
with PS) { apicalnotch (= sePtalnotch)
r/ midlineaorta (30%)i ascendingaortawith ^/ increasedpulmonarybloodflow (if shuntpresent)
convexityto the right { pulmonaryvenoushypertension (if left-sidedAV
r/ rightaorticarch in 3% (difficultassessmentdue to valve incompetent)
midlineposition+ smallsize) r/ n enlargement
O B - US : A ngi o:
r/ great arteriesarise from ventriclesin a parallel { originalLV on rightside: smooth-walled, cylinder-/
fashion cone-shapedwith high recessemptyinginto aorta
r/ aorta anterior+ to rightof pulmonaryartery(in 60%; (= verous ventricle)
rarelyside by side) { originalRV on leftside: bulbous,triangularshape,
Prognosis; overall 70% survival rate at 1 week, trabeculatedchamberwith infundibularoutflowtract
50/" at 1 month,11o/"at 1 year by natural intopulmonarytrunk(= arterialventricle)
history OB -U S :
Rx: r/ great arteriesarisefrom ventriclesin a parallel
(1) Prostaglandin E1 administrationto maintainductal fashion
patency r/ aorticvalve separatedfrom tricuspidvalve by a
(2) RashkindprocedUre= balloonseptostomyto completeinfundibulum
createASD ri fibrouscontinuitybetweenpulmonicvalve+ mitral
(3) Blalock-Hanlon procedure= surgicalcreationof valve
ASD Prognosis; (unfavorablesecondaryto additional
(4) Mustardoperation(corrective)= removalof atrial cardiacdefects)40o/"1-yearsurvivalrate,
septum+ creationof intraatrialbaffledirectingthe 30/" 1O-yearsurvivalrate
pulmonaryvenous returnto RV + systemicvenous
returnto LV; 79% 1-yearsurvivalrate;64-89%
S-yearsurvival ATRESIA
TRICUSPID
2nd mostcommoncauseof pronouncedneonatal
cyanosis(aftertransposition)characterizedby
CorrectedTranspositionof GreatArteries (1) absenttricuspidvalve
= CONGENITALLY CORRECTED TRANSPOSITION (2) ASD
= L-TRANSPOSITION (3) smallVSD (in mostpatients)
= €lnorTl?lous loop
loopingof the bulboventricular Frequency: 1.5/" of all CHD
(= primordialventricles)associatedwith lack of spiral Embryology: imbalancedtissueproliferation+ resorption
rotationof conotruncalseptumcharacterizedby resultsin absenceof valvulartissue
(1) Transposition of greatarteries 1. TR IC U S P ID A TR E S IAW ITH OU TTR A N S PO SI TI O N
(2) Inversionof ventricles(LV on rightside,RV on left (80%)
s ide) : (a) withoutPS, (b) with PS, (c) with pulmonaryatresia
(a) RA connectedto morphologicLV 2. TR IC U S P IDA TR E S IAW ITHTR A N S P OS ITI O N
(b) LA connectedto morphologicRV (a) withoutPS, (b) with PS [mostfavorable
(3) AV valves+ coronaryarteriesfollowtheir combination], (c) with pulmonaryatresia
correspondi ng ventricles 0 UsuallysmallVSD + PS (75%)restrictpulmonaryblood
Hemodynamics: functionallycorrectedabnormality flow
 Cardiovascular
Disorders 659

Hemodynamics: TRICUSPID
INSUFFICIENCY
absenttricuspidvalve forcesbloodfrom an enlargedRA
Cause:
throughan ASD into LA (R-to-Lshunt);pulmonar blood 1. R i ghtventri cul ar
fai l ure(mostcommon)
flow limitedby pulmonaryvalvularstenosis
2. Carcinoidsyndrome
RA I RV J MainPA J 3. Bacterialendocarditis
Pulm.vessels v
4. Rheumaticheartdisease
L A T L V T A o 5. Congenitalheartdisease: Ebsteinanomaly,
. progressivecyanosisfrom birthon, increasingwith atrioventricular cushiondefect
r/ normal/ reducedpulmonaryvascularity
crying= OUTSTANDINGFEATURE(inverse
r/ cardiomegaly
relationshipbetweendegreeof cyanosis+ volumeof ./ nn + RV enlargement
pulmonarybloodflow) r/
. pansystolic distensionof IVC > SVC
murmur(VSD)
. ECG: left-axisdeviation
TROUSSEAU
SYNDROME
CXR (typicalcardiaccontour):
= PARANEOPLASTIC THROMBOEMBOLISM
r/ heartsize rangingfrom normalto moderately
lncidence: 1-11%; higherin terminallyill cancer
enlarged(dependingon volumeof pulmonaryblood
patients
flow and size of RA)
Tumors: mucin-secreting adenocarcinomaof Gl tract
r/ tett roundedcontour= enlargement+ hypertrophyof
and pancreas(mostcommon),lung,breast,
LV
r/ right roundedcontour= enlargedRA ovary, prostate
^/ ttat/ concavepulmonarysegient Pathogenesis: (?)
r/ normal/ decreasedpulmoniryvascularity (a) tumorsactivatecoagulation+ depressanticoagulant
function
r/ typicalflatteningof right heart borderwith
(b) cancercellscauseinjuryto endotheliallining,
transposition (in 1S%)
activateplatelets+ coagulation
Prognosis: may survivewell into early adulthood
Typeof lesion: (1) Venousthrombosis
Rx:
(1) Blalock-Taussig (2) Arterialthromboembolism
procedure(if pulmonarybloodflow
(3) Nonbacterialthromboticendocarditis
decreasedin infancy)
0 Patientswith thromboembolism have an increased
(2) Glennprocedure= shuntbetweenIVC + rightpA (if
incidenceof occultmalignancy!
totalcorrectionnot anticipated)
Prevalent criteria:
(3) Fontanprocedure= externalconduitfrom RA to - absenceof apparentcausefor thromboembolism
pulmonarytrunk+ closureof ASD (if pulmonary - age >50 years
vasculardiseasehas not developed) - multiplesitesof venousthrombosis
- simultaneous venous+ arterialthromboembolism
- resistanceto oral anticoagulanttherapy
- associatedother paraneoplastic syndromes
- regressionof thromboembolism with successful
treatmentof cancer
. disordersof consciousness(cerebralemboli)
. muscularpain + weakness(embolito skeletalmuscle)
., decompensated disseminated intravascularcoagulation
r/ deep vein thrombosis
r/ pulmonaryembolism
Type I r/ nonbacterialthromboticendocarditis(echocardiography)
Rx: (1) Heparin(moresuccessfulthan warfarin)
(2) Greenfieldfitter

TR U N C U S A R TE R IOS U S
= PERSISTENT TRUNCUS ARTERIOSUS = SINGLE OUTLET
OFTHEHEART
= failureof septationof the conotruncuscharacterizedby
(1) one greatarteryarisingfrom the heartgivingriseto
the coronary,pulmonary,and systemicarteries,and
straddlinga
(2) largeVSD
lncidence: 2/" of all CHD
Types:
Type I (50%) = main PA + aorta arisefrom common
VariationsinTruncusArteriosus truncalvalve
654 Radiology Review Manual

Type ll (25%) = both pulmonaryarteriesarisefrom Rx: Rastelliprocedure(30% no longeroperableat

back of trunk 4 years of age) = (a) artificialvalve placedhigh in
Type lll (10%)= both pulmonaryarteriesarisefrom RVOT and attachedvia a Dacrongraftto main
side of trunk pulmonaryartery(b) closureof VSD
Type lV = "pseudotruncus"
= absenceof
pulmonary pulmonary
arteries; supply Hemitruncus
fromsystemic arisingfrom
collaterals = r?Ieanomalycharacterizedby
descendingaorta (a)onepulmonary artery(commonly rightPA)arising
SubtypeA = infundibular VSD present fromtrunk
S ubt y peB = VS D a b s e n t (b)onepulmonary arteryarisingfromRV/ supplied
collaterals
by systemic
Associated with: with: PDA(80%),VSD,tetralogy
Associated (usually
(1) Rightaorticarch (in 35%) isolatedto leftPA)
0 rightaorticarch + cyanosis+ shunt vascularity . acyanotic
= T RUN C U S
(2) Forkedribs Pseudotruncus Arteriosus
Hemodynamics: = TRUNCUS TYPEIV
admixturelesion(R-to-Land L-to-RshuntacrossVSD) = sevel€formof tetralogy of Fallotwithatresiaof the
with volumeof pulmonarybloodflow inverselyrelatedto pulmonary trunk;entirepulmonary through
circulation
degreeof pulmonaryvascularresistance bronchialcollateralarteries(NOTa formof truncus
fetus : CHF onlywith incompetent valvesecondary arteriosusin itstruesense);characterized by
to massiveregurgitationfrom truncusto (1)pulmonary atresia
ventricles (2)VSDwithR-to-Lshunt
neonate: L-to-Rshuntafterdecreasein pulmonary (3) RV hypertrophy
resistance(massivediversionof flow to Associatedwith: rightaorticarchin 50%
pulmonarydistrict)leadsto CHF . cyanosis
(ventricularoverload)/ pulmonary { concavityin areaof pulmonary segment
hypertension withtime { commalike abnormal appearance of pulmonary aftery
RA <+ RV t Trunk t { absentnormalrightandleftpulmonary artery(lateral
Pulm.vessels 1 chestfilm)
L A < + L V t { esophageal posteriorly
indentation (dueto large
. moderatecyanosis(degreeinverselyrelatedto volume systemic collaterals)
of pulmonarybloodflow),apparentwith crying { prominent hilar+ intrapulmonary vessels(= sYstemic
. severeCHF withinfirst days / monthsof life (in large collaterals)
"coeuren sabot"= RV enlargement
R-to-Lshunt)
. systolicmurmur(similarto VSD) prominentascendingaortawith hyperpulsations
. earlydiastolicmurmur(withtruncalinsufficiency)
. wide pulse pressure ANEURYSM
VENTRICULAR
CX R: A. CONGENITALLEFTVENTRICULAR ANEURYSM
{ cardiomegaly: rare,youngBlackadult
./ increasedvolumeof both ventricles (1) S ubmi tral
type:
{ enlargedLA (50%)secondaryto increased r/ bulge at left middle/ uppercardiacborder
pulmonarybloodflow (2) Subaortictype:
r/ wide mediastinumdue to large"aorticshadow" ri small+ not visualized
= truncusarteriosus ri heartgreatlyenlarged(fromaorticinsufficiency)
r/ "waterfall/ hilarcommasign"= elevatedrighthilum B . A C OU IR E DLE FTV E N TR IC U LA AR N E URYSM
(30%);elevatedleft hilum(10%) = complicationof myocardialinfarction,Chagas
{ concavepulmonarysegment(50%) (type I has left disease
convexpulmonarysegment) . may be asymptomatic+ well toleratedfor years
r/ markedlyincreasedpulmonarybloodflow, may be . occasionallyassociatedwith persistentheartfailure,
asymmetric arrhythmia, peripheralembolization
E CHO :
r/ singlearterialvesseloverridingthe interventricular TrueVentricularAneurysm
septum(DDx:tetralogyof Fallot) = circumscribednoncontractile outpouchingof
r/ frequentlydysplasticsinglesemilunarvalvewith ventricularcavitywith broad mouth+ localized
3-6 leaflets(mostcommonly3 leaflets) dyskinesis
{ truncalvalve may be stenotic Cause: sequelaeof transmuralmyocardialinfarction
r/ truncalvalve insufficiencywith age (in 25%) Location:
Prognosis: 40oh6-monthssurvival rate, (a) left anterior+ anteroapical: readilydetected
20% 1-yearsurvival rate (anterior+ LAO views)
 Cardiovascular
Disorders 6Ss

(b) inferior+ inferoposterior:less readilydetected 2. SUPRACRISTAL = CONALVSD (5-8%)

(steepLAO + LpO views) 0 Cristasupraventricularis = invertedU-shaped
Detectionrate: 50% by fluoroscopy;g6%by muscularridgeposterior+ inferiorto pulmonary
radionuclide ventriculography; valve
(a) RV view - VSD just beneathpulmonaryvalve with
r/rocarizedburse"l';#;1ty.il'J,":','il"r:1":X?, valveformingpart of superiormarginof defect
, appearanceof mid left lateralmarginof heartborder (b) LV view = VSD just belowcommissurebetweenR
r/ localizedparadoxicalexpansionduringsystole
+ L aorticvalve cusps
(cHARACTERtSTtC) Cx: rightaorticvalve cusp may herniateinto VSD
r/ rim of calciumin fibroticwall (chronic),rare (= aorticinsufficiency)
r/ akinetic/ severelyhypokineticsegment 3. MU S C U LA RV S D (5-10% )
^/ tettventriculography in LAO, nnd is diagnostic May consistof multipleVSDs;borderedentirelyby
{ wide communication with heartchamber-(no neck) myocardium
Cx: wall thrombuswith embolization Location: (a) inletportion
Prognosis: rarelyruptures (b) trabecularportion
(c) infundibular/ outletportion
4. ATRIOVENTRICULAR CANALTYPE
Pseudoaneurysm
of Ventricle = E N D OC A R D IAC L U S H IONTY P E
= FALSE
ANEURYSM = leftventricular
rupture = POSTERTOR VSD (5_10%)
containedby fused layersof visceral+ parietal Location: adjacentto septal+ anteriorleafletof
pericardium/ extracardiactissue mitralvalve;rare as isolateddefect
(a) cardiacrupturewith localizedhematoma Hemodynamics:
containedby adherentpericardium; typicallyin the smallbidirectional shuntduringfetallife (similar
presenceof pericarditis pressuresin RV + LV);afterbirthpulmonaryarterial
(b) subacuterupturewith gradual/episodicbleeding pressuredecreases+ systemicarterialpressure
Etiology: trauma,myocardialinfarction increaseswith development of Lto-R shunt
Location: typicallyat posterolateral
/ diaphragmatic
wall of LV Classification:
^/ tett retrocardiacdoubledensity "Maladie
de Roger"
r/ diameterof mouthsmallerthan the largestdiameter = Sffi?llrestrictiveVSD with defect<1 cm; little/ no
of the globularaneurysm hemodynamic significancewith normalpulmonary
! delayedfilling arterypressure,normalpulmonaryvascular
Cx: high risk of delayedrupture(infrequentin true resistance
aneurysms) . asymptomatic
., holosystolicheart murmurat 4th left rib interspace
r/ normalplainfilm
Prognosis.' spontaneousclosure
VENTRICULAR
SEPTALDEFECT
Most commonCHD (25-30%): ModerateShunt
(a) isolatedin 20% VSD defect<75o/o of aorticdiameter(1-1.5 .r );
(b) with othercardiacanomaliesin S/" (pDA, systolicLV pressure> systolicRV pressure;
coarctationof aorta) intermediatepulmonary arterypressure;normal
0 AcyanoticL-to-Rshunt + rightaorticarch (in 2-S%\ pulmonaryvascularresistance
= VSD Heartsize t Main pA e to t
Embryology: Pulm.vessels<-+to t LA t
singleventricular chamberdividesintotwo by fusionof ', respiratoryinfections,milddyspnea
membranousportionof ventricularseptum r/ slightprominenceof pulmonaryvessels(45%
+ endocardial cushions+ bulbiscordis(= proximalpart shunt)
of truncusarteriosus)between4-gth week r/ slightenlargement of LA
1 . M E M B RA NO U S = P ER IM EMB R A N OUVS S D Prognosis: spontaneousclosurein largepercentage
(75-80%)
Location: posterior+ inferiorto crista Nonrestrictive
LargeShunt
supraventricu larisnear commissure VSD defect>75/o of aorticdiameter;systolicLV
betweenrightand posterior pressure= systolicRV pressure(pulmonary vascular
(= flor'tcoronary)
aorticvalve cusps disease+ hypertension increasesRV pressure);
May be associated with: pulmonaryarlerypressureapproachingsystemic
smallaneurysmsof membranousseptumcommonly levels;slightlyincreasedpulmonaryvascular
leadingto decreasein size of membranousVSD resistance;pulmonaryblood flow 2-4 x systemicflow;
(theirpresencedoes not necessarilypredicteventual Heartsize t Main pA t
completeclosure) Pulm.vesselst LA
656 Radiology Review Manual

. bouts of respiratoryinfections { enlargement of LA

. feedingproblems,failureto thrive { enlargementof pulmonaryarlerysegment
. CHF soon after birth (dueto RV overload) { enlargement of LV
r/ prominentpulmonarysegment+ vessels(= 5hun1 r/ nV hypertrophy
vascularity) ^/ increasein pulmonarybloodflow (if >45/" ot
r/ calcificationof pulmonaryarteries pulmonarybloodflow from systemiccirculation)
= PATHOGNOMONIC for pulmonaryarterial ./ Eisenmengerreaction
hypertension
r/ enlargementof LA + LV E C H O:
r/ normal/ smallthoracicaorta r/ prolapseof aorticvalve cusp (in supracristalVSD)
r/ deformityof aorticcusp (in membranousVSD)
Eisenmenger syndrome ./ lack of echoesin regionof interventricular
septum
largeVSD eventuallyleadsto shuntreversal(R{o-L with sharpedges(DDx:artifactual dropoutwith sound
increasein pulmonary
shunt)due to irreversible beam parallelto septum);muscularVSD difficultto
vascularresistance(= intima+ medialhyperplasia) see
when pulmonaryvascularresistance>0.75of r/ m enlargement
systemicvascularresistance
RA T RVl MainPA 1 A ngi o:
Pulm.vessels Jrt Projections:
LA tto<+ LV tto<+ Ao tto<+ (a) LAO 60' C-C 20" tor membranous+ anterior
Frequency: 10/o of large VSDs by 2 years of age muscularVSD
. cyanotic,but less symptomatic;CHF rare (b) LAO 45'C-C 45" (hepatoclavicular) for posterior
r/ eventualdecreaseof pulmonaryvesselcaliber endocardialcushion+ posteriormuscularVSD
{ eventualdecreasein size of LA + LV (c) RAO for supracristalVSD + assessmentof RVOT
^/ nVOf / pulmonaryvalvefill withoutfillingof RV
NATURALHISTORYOF VSD causingreductionin chamber(in supracristal VSD)
pulmonarybloodflow:
1. Spontaneous closure R x:
in 40'/" withinfirst 2 years of life;60% by 5 years (a) largeVSD + left heartfailureat 3 monthsof age: aim
(65%with muscularVSD, 25/.with membranous is to delayclosureuntilchildis 18 monthsof age;
V S D) ; wi thl a rg eVS D i n 1 0 o /"w; i ths ma l lVS D i n pulmonary-to-systemic bloodflow >2:1 requires
50% surgerybeforepulmonaryhypertensionbecomes
2. RVOT obstruction manifest
infundibularhypertrophyin 3% = pink tetrad 1. Digitalis+ diuretics
3. Prolapseof rightaorticvalve cusp 2. Pulmonaryarterybanding
= €lorticvalve insufficiency 3. Patchingof VSD: surgicalapproachthroughRA /
throughRV for supracristalVSD
CXR (withincreasein size of VSD): (b) smallVSDs withoutincreasein pulmonaryarterial
r/ variableappearancedue to variationsin defectsize pressureare followed
 DIFFERENTIALDIAGNOSISOF HEPATIC,BILIARY,PANCREATIC,AND SPLENICDISORDERS

RIGHT UPPER QUADRANT PAIN 8. Galactosemia

A . B I LEDUCT S B. MALIGNANCY
1. Biliarycolic/ bileduct obstruction 1. Lymphoma
2. Acute cholecystitis/ chofangitis 2. Diffusemetastases
B. LIVER 3. DiffuseHCC
1. Acutehepatitis:alcoholic,viral,drug-related,
toxic 4. Angiosarcoma
2. Hepaticabscess C . IN FLA MMA TION / IN FE C TION
3. Hepatictumor:metastases,hepatocellular 1. Hepatitis
carcinoma,hemangioma, focalnodularhyperplasia, 2. Mononucleosis
hepaticadenoma 3. MiliaryTB, histoplasmosis, sarcoid
4. Hemorrhagic cyst 4. Malaria
5. Hepaticcongestion:acute hepaticcongestion, 5. S yphi l i s
Budd-Chiari syndrome 6. Leptospirosis
6. Perihepatitis from gonococcal/chlamydialinfection 7. Chronicgranulomatous diseaseof childhood
(Fitz-Hugh-Curtissyndrome) 8. Sarcoidosis
C. PANCREAS D. VASCULAR
1. Acutepancreatitis 1. Passivecongestion
D . I NT E S T I NES E . OTH E R S
1. Acuteappendicitis 1. E arl yci rrhosi s
2. Peripyloric ulcer 2. Polycysticliverdisease
3. Smallbowelobstruction
4. lrritabfebowel
IncreasedLiverAttenuation
5. Colitis/ ileitis
Abnormaldepositsof substanceswith highatomic
6. Intestinal tumor
numbers
E. LUNG
A . IR ON
1. Pneumonia
(a) diffuseironaccumulation
2. Pulmonaryinfarction
1. GeneticI primaryhemochromatosis
F . K I DNE Y
2. Erythropoietichemochromatosis
1. Acutepyelonephritis
3. Bantusiderosis
2. Ureteralcalculus
4. Transfusionaliron overload
3. Renal/ perirenalabscess
(b) focal iron accumulation
4. Renalinfarction
1. Hemorrhagic metastases:choriocarcinoma,
5. Renaltumor
melanoma
G . O T HE RS
2. Hepaticadenoma
1. Costochondritis
3. Sideroticregenerativenodulesof cirrhosis
2. Herpeszoster
0 An iron-poorfocus withina sideroticnodule
LIVER on T2Wl suggestsHCC!
4. Focal hemochromatosis
DiffuseLiver Disease B . C OP P E R
1. Fattyliver Wilsondisease= hepatolenticufar degeneration
2. Cirrhosis = increasedcopperdepositsin liver+ basalganglia
3. Hepatitis c. toDrNE
4. Hemochromatosis Amiodarone(= antiarrhythmic drug with 37"/oiodine
5. Glycogenstoragedisease by weight)
6. B udd- Chia ri s y n d ro m e r/ gS-t45 HU (rangeof normalfor tiver30-70 HU)
D . GOLD
DiffuseHepaticEnlargement Colfoidalform of gold for therapyof rheumatoid
A. METABOLIC arthritis
1. Fattyinfiltration E. THOROTRAST
2. Amyloid Alpha-emitterwith atomicnumberof g0
3. Wilsondisease F. TH A LLIU M
4. Gaucherdisease Accidentali suicidalingestionof rodenticides (lethal
5. Von Gierkedisease dosei s 0.2-1.0gram)
6. Niem an n -Pi c k d i s e a s e G. ACUTEMASSIVEPROTEINDEPOSITS
7. Weber-Christian disease H. GLYCOGENSTORAGEDISEASE
658 Radiology Review Manual

"GG CHAT" (b) cholangiocellular(6%)

mnemonic.'
Goldtherapy 1. C hol angi ocarci noma
Glycogenstoragedisease 2. BiliarycYstadenocarcinoma
Gyclophosphamide B . ME S E N C H Y MATUL MOR
Hemochromatosis / hemosiderosis (a) tumor of bloodvessels
Amiodarone 1. A ngi osarcoma
Thorotrast 2. Epithelioidhemangioendothelioma
3. Kaposisarcoma
(b) othertumor
IncreaseIn LiverEchogenicity
Generalized 1. Embryonalsarcoma
1. Fattyliver 2. Fibrosarcoma
2. Steatohepatitis C . T U M O RO F M U S C L ET I S S U E
3. Cirrhosis(fibrosis+ fatty liver) 1. Leiomyosarcoma
4. Chronichepatitis 2. Embryonalrhabdomyosarcoma of the biliary
5. Vacuolardegeneration tree
D. MISCELLANEOUS
1. Carcinosarcoma
LiverMass 2. Teratoma
0 Hepaticmassesaccountonly for 5-6% of all 3. Yolk sac tumor
massesin children!
intraabdominal 4. Carcinoid
5. Squamouscarcinoma
Primary Benign Liver Tumor 6. PrimarylymPhoma
A . E P I T HEL IATLU M O R S
(a) hepatocellular
1. Regenerative nodules Solitary Liver Lesion
2. Adenomatoushyperplastic nodules A. BENIGN TUMOR
3. FocalnodularhYPerPlasia 1. Cavernoushemangioma
4. Hepaticadenoma 2. A denoma
(b) cholangiocellular 3. FocalnodularhYPerPlasia
1. Bileduct hamartoma/ adenoma B . IN FE C T]ON
2. Biliarycystadenoma 1. Pyogenicabscess
3. Papillaryadenoma 2. Echinococcal cYst
B . M E S E N C H YMATLU M O R S 3. InflammatorY Pseudotumor
(a) tumor of adiposetissue C . TR A U MA
1. H e p a ti cl i p o ma 1. H ematoma
2. Hepaticmyelolipoma 2. TraumaticcYst
3. HepaticangiomYoliPoma D . MA LIGN A N T TU MOR
(b) tumorof muscletissue 1. P ri marytumor
1. L e i o m y o ma 2. Metastasis
(c) tumor of bloodvessels E . OTH E R
h e ma n g i o e n d o th e l i o ma
1. I n fa n ti l e 1. Fattychange
2. Hemangioma 2. SimplecYst
3. PeliosishePatis
(d) mesothelial tumor
1. Be n i g nm e s o th e l i o ma SolrenY EcnooEttlcLlven Mlss
"HyperechoicFocalMasses
C. M I X E DT ISS U ET U M O R mnemonic;
1. Mesenchymal hamartoma Affectingthe Liver"
2. Benignteratoma Hematoma,HePatoma,Hemangioma,
D. MISCELLANEOUS Hemochromatosis
1. Adrenalresttumor Focalnodularhyperplasia,
Fattyinfiltration,
2. Pancreaticrest Fibrosis
Metastasis
Primary Malignant Liver Tumor Adenoma
0 Hepaticmalignancies are the mostcommonGl Lipoma
malignancyin children,but accounttor <2o/"of all
pediatricmalignancies I
T U MOR
A . E P I T H E L IAL LIven Mlss SunnouruoEDBYEcnocetttc Rlnrt
(a) hepatocellular esp.,
1. Metastasisl cysticisletcelltumor
1. Hepatoblastoma (7%) 2. Adenoma
2. Hepatocellular carcinoma(75%) 3. Hemangioma
 DifferentialDiagnosisof Hepatic,Bitiary,Pancreatic,and SptenicDisorders 659

Multiple Liver Lesions Vascular"Scar" Tumor of Liver

A. BENIGN TUMOR 1. Focalnodularhyperplasia
1. Cavernoushemangioma 2. Hepaticadenoma
2. Adenoma 3. Giantcavernoushemangioma
3. Regenerating hepaticnodules 4. Fibrolamellarcarcinomaof liver
4. Multiplebile duct hamartoma 5. Well-differentiated
hepatocellular
carcinoma
B . I NF E CTION 6. Hypervascularmetastasis
1. Multipleabscesses 7. lntrahepaticcholangiocarcinoma
2. Mycobacterial+ fungalinfection
3. Inflammatorypseudotumors Liver Masswith CapsularRetraction
C. CONGENITAL 1. Cholangiocarcinoma
1. Polycysticdisease 2. Fibrolamellar
carcinoma
2. Carolidisease or any hepaticmalignancy
D. MALIGNANCY
1. Metastases(mostcommonmalignantliver Low-density Massin Porta Hepatis
tumor) 1. Choledochal cyst
2. Multifocafhepatoma 2. Hepaticcyst
3. Lymphoma 3. Pancreaticpseudocyst
E . O T HE R 4. Enteric
duplication
1. Sarcoidosis 5. Hepaticarteryaneurysm
2. Simplecysts 6. Biloma
3. Langerhanscell histiocytosis(echogenic 7. Embryonal rhabdomyosarcoma
of biliarytree
nodules)
Low-density Hepatic Mass with Enhancement
1. Hepatoma
Butr's-eye Leslons or Lrven 2. Hypervascularmetastases (lesions
thatmaybe
1. Candidiasis(in immunocompromised) obscured
aftercontrastinjection:
2. Metastases pheochromocytoma,carcinoid, melanoma)
3. Lymphoma,leukemia 3. Cavernous hemangioma
4. Sarcoidosis 4. Focalnodularhyperplasia withcentralfibrousscar
5. Septicemboli 5. Hepaticadenoma
6. Other opportunisticinfections
7. Kaposisarcoma Fat-containing Liver Mass
1. Hepatoma
2. Angiomyolipoma
Mtranv HEpATospLENtcLEsroNs
1. Tuberculosis Hyperintense Liver Mass on TIWI
2. Metastases 1. Focalfat deposit
3. Fungalinfections 2. Highproteincontent
4. Sarcoidosis 3. Hemorrhage (methemoglobin)
5. Lymphoma 4. Melanoma metastasis
5. Paramagnetic contrastagents+ iodizedoil
Cystic Liver Lesion Hypervascular Liver Mass
A. NONNEOPLASTIC r/ detected
duringhepaticarterialphase
1. Congenitalhepaticcyst A. PRIMARY
2. Hematoma 1. Hepatocellular
carcinoma
3. Echinococcalcyst 2. Hemangioma
4. Abscess 3. Focalnodularhyperplasia
5. Cysticliverdisease 4. Hepaticadenoma
6. Autosomaldominantpolycysticdisease B. METASTASES
B. NEOPLASTIC 1. Neuroendocrinetumors:isletcell,carcinoid
1. Mesenchymal hamartoma 2. Renalcellcarcinoma
2. Undifferentiatedsarcoma(embryonalsarcoma) 3. Breastcarcinoma
3. Malignantmesenchymoma
4. Biliarycystadenoma/ cystadenocarcinoma Hepatic Galcification
0 <5/" of intrahepaticcysts are of biliaryoriginl A. INFECTION (mostcommoncause)
5. Lymphangioma 1. Granulomatous disease:tuberculosis
(4g/"),
6. Necroticneoplasm histoplasmosis,
brucellosis,
coccidioidomycosis
7. Cysticmetastasis(ovarian/ gastriccarcinoma) r/ calciuminvolves
entirelesion
660 RadiologyReviewManual

2. Echinococcalcyst (in 10-20%) 6. H E LLPsyndrome

r/ curvilinearI ringcalcification 7. Amyloidosis
3. CMV,toxoplasmosis, Pneumocystis carinii 8. Peliosishepatis
4. Chronicgranulomatous diseaseof childhood 9. Angiomyolipoma
5. Old pyogenic/ amebicabscess
6. Schistosomiasis, cysticercosis,filariasis,
paragonimiasis, Armilliferinfection, LIVERCIRCULATION
dracunculiasis Enhancement
TransientHepaticParenchymal
7. S y phi l i tigcu mma = H Y P E R P E R FU S IONA B N OR MA LITIEO SF LI VER
B. VASCULAR = ?fedsof earlyenhancement on arterial-dominant
1. Hepaticarteryaneurysm phasedue to decreasedportalbloodflow / formation
2. Portalvein thrombosis of intrahepaticarterioportalshuntsi increased
3. Hematoma aberrantdrainagethroughhepaticveins
C. B I LI A RY A. LOBARi SEGMENTAL
1. Intrahepatic calculi 1. Portalvein obstruction:
2. Ascariasis, clonorchiasis portalvein thrombosis, tumorinvasion,surgical
D . B E N I G NT U M O R S ligation
1. Congenitalcyst 2. Cirrhosiswith arterioportal shunt
2. Cavernoushemangioma 3. Hypervascular gallbladder disease
r/ largecoarsecentrallylocatedcalcification(in B . S U B S E GME N TA L
10-20%\ 1. Obstructionof peripheralportalbranches
3. Hepatocellularadenoma 2. Percutaneous needlebiopsy+ drainage
4. Capsuleof regenerating nodules procedure/ ethanolablation
5. Infantilehemangioendothelioma 3. Acutecholecystitis + cholangitis
E . P RI M A R YM AL IGN AN T U MOR c. SUBCAPSULAR
1. Fibrolamellar carcinoma(calcifiedin 1Y25%) (a) due to peripheralparenchymal compression
2. Hepatocellular carcinoma 1. R i bcompressi on
3. Hepatoblastoma (10-20%) 2. Perihepatic peritonealimplants
4. Intrahepatic cholangiocarcinoma (in 18%) 3. P seudomyxomaperi tonei
r/ calcificationaccompaniedby desmoplastic 4. Perihepatic fluidcollections
reaction (b) idiopathic/ unexplained
5. Epithelioid hemangioendothelioma D. P S E U D OLE S ION S
6. Cystadenocarcinoma = systemicvenousbloodflow drainingintohepatic
F . METASTATICTUMOR si nusoi ds
1. Mucin-producing neoplasm:carcinomaof colon, 1. Accessorycysticvein of gallbladder fossa
breast,stomach 2. Aberrantrightgastricvein
2. Ovariancarcinoma(psammomatous bodies) 3. Capsularveins
3. Melanoma,thyroidcarcinoma,pleural E . RETICULAR-MOSAICPATTERN
mesothelioma, chondro-and osteosarcoma, 1. C i rrhosi s
carcinoid,leiomyosarcoma, neuroblastoma 2. Hereditaryhemorrhagic telangiectasia
3. Hepaticvein obstruction
mnemonic: "4H TAG MAP"
Hepatoma ArterioportalShunt
Hemochromatosis = orgdrric/ functionalcommunicationbetweenhigh-
Hemangioma pressurehepaticarterialbranch+ low-pressureportal
Hydatiddisease venoussystem
Thorotrast Cause:
Abscess A. Primaryhepaticneoplasm
Granulomas(healed) 1. Hepatocellular carcinoma
Metastases 2. H emangi oma
Absentmnemonic 3. Cholangiocarcinoma
Porcelaingallbladder B. Metastatictumor
C. Hepatictrauma
1. B l untabdomi naltrauma
SpontaneousHepaticHemorrhage 2. latrogenic:biopsy,percutaneousabscess
1. Hepatocellular
carcinoma drainage,percutaneous biliarydrainage,
2. Hepatocellular
adenoma ethanolinjection
3. Focalnodularhyperplasia D. Cirrhosis
4. Hepatichemangioma E. Ruptureof hepaticarterypseudoaneurysm
5. lung,RCC,melanoma
Hepaticmetastases: F. Congenitalmalformation
 Afferential Diagnosis of Hepatic, Biliary, Pancreatic, and Splenic Disorders 661

Routes: 2. Hepaticartery-portalvein fistula

1. Macroscopic fistula 3. Arteriovenousshuntin cirrhosis
2. Transsinusoidal = betweenmicroscopic 4. Portal-to-hepatic
vein fistula
interlobular
arteriole+ portalvenule
3. Transvasal= via tumorthrombus
4. Transtumoral = via drainingvein from a
hypervascular tumor PortalVenousGas
5. Transplexal/peribiliary= via capillarynetwork
0 Shouldbe considereda life-threatening event and
surroundingbileducts
sign of bowelinfarction+ gangreneuntilproved
Pathophysiology:
otherwise!
shuntedcontrastmaterialenhancesa focal area of
Etiology:
liverparenchymabeforeadjacentparenchymais
A . IN TE S TIN AN
L E C R OS TS(i n74%of adut t s)
enhancedvia the usualsplanchnicroute
1. Bowelinfarctionsecondaryto arterialand
CECT (in hepaticarterialphase):
venousocclusions(vascularaccidents,
! pseudolesion = transientperipheralwedge-shaped
superiormesentericarterysyndrome)
hepaticparenchymal enhancement: 2. Ulcerativecolitis
ri smallshuntmay resemblenodularlesion
3. Necrotizingenterocolitisassociatedwith
r/ lesiondisappearsin portalvenousphase
mesentericarterialthrombosis
r/ enhancementof portalvein branch t main portal
4. Perforatedgastriculcer
vein from peripherywithoutenhancement of splenic B . GI OB S TR U C TION
vein/ superiormesentericvein
1. Smallbowelobstruction(duodenalatresia)
2. lmperforateanus
HepaticArtery Enlargement 3. Esophagealatresia
1. Cirrhosis(compensatoryresponseto decreased C . MIS C E LLA N E OU S
portalvenousflow) 1. Hemorrhagic pancreatitis
2. Intrahepatic arteriovenous shunting 2. Sigmoiddiverticulitis
(a) vascularneoplasm 3. Intraabdominal abscess
(b) hepaticartery-portalvein fistula 4. P neumoni a
Cause: biopsy,trauma 5. latrogenicinjectionof air duringendoscopy
r/ turbulenthigh-velocitylow-resistanceflow 6. Dead fetus
r/ soft-tissuebruit (= randomassignmentof color 7. Diabetes,diarrhea
in perivascular
softtissuedue to tissue
vibration) mnemonic.' "BE NICE"
r/ arterializedfrequentlyretrogradeflow in portal BE (airembolismduringdoublecontrastbarium
vein enema)
3. Hereditaryhemorrhagictelangiectasia Necrotizing enterocolitis
! largetortuousfeedingarterieswith high velocity+ Infarction(mesenteric)
aliasedflow Catheterizationof umbilicalvein
r/ multipledilatedvessels(representing AVMs) Erythroblastosisfetalis
./ largedrainingveins
{ areas of fattychange+ fibrosis Pathogenesis:
4. Chronicactivehepatitis 1. lntestinalwall alterationpermittingpassageof
intraluminalair intointestinalvenules:
Dampeningof HepaticVein DopplerWaveform (a) ulcerationof gastric,duodenal,bowelwall
= dampenedoscillations of hepaticveinsresembling (b) sloughingof epitheliallining
portalvein flow due to "shielding" of hepaticveins (c) enhancedmucosalpermeability
from activityof rightatrium eg, intestinalischemiawith bowelnecrosis(most
= "portalization" of hepaticvein flow pattern common),perforatedgastriccarcinoma/ ulcer,
A. Increasedlivertissuestiffness inflammatory boweldisease(Crohndisease,
1. Liv erc ir rh o s i s ulcerativecolitis)
2. Variousparenchymal abnormalitiesof liver Prognosis: 75-90/" mortalityrate within 1 week
B. Intrinsici extrinsicvenousobstruction of diagnosis
1. Budd-Chiari syndrome 2. Boweldistensionwith elevatedintraluminal
2. lnferiorvena cava obstruction pressurecausesminimalmucosaldisruption
3. Extrinsiccompressionof hepaticveins + permitspassageof intraluminalair into veins:
(a) iatrogenicdilatationof hollowviscus
PulsatilePortalVein (gastrostomy,sclerotherapy,ERCP,
= waveformpulsatilitywith >Zl3 changefrom peak to colonoscopy, bariumenema)
minimalvelocity (b) spontaneousparalyticileus,mechanical
1. Congestiveheartfailure obstruction,acutegastricdilatation
662 Radiology Review Manual

(c) blunttrauma(<1%)with acutepressure (c) hiatal,umbilical,inguinalhernias

changes (d) Zenker,epiphrenic, gastric,duodenal,jejunal
(d) barotrauma diverticulum
Prognosis; surgery often not indicated (e) gastriculcer,gastrocolicfistula
3. Intraabdominal sepsis (f) malabsorption, diarrhea
(a) ? gas from septicemiain branchesof (g) postoperativeileus,severetrauma
mesentericveins/ portalvein (pylephlebitis) (h) inflammation:acute pancreatitis,acute
(b) ? increasedintraluminal fermentationof peritonitis
carbohydratesdue to bacterialovergroMh 4. Deficiencyof bilesalts
(c) ? mesocolicabscesscausinginframesocolic Crohndisease,surgicalresectionof terminal
perforationdissectingbetweenperitoneal ileum,liverdisease,cholestyramine therapy,
leaflets abnormalcommunicationbetweenbiliarysystem
intra-or retroperitoneal
eg, diverticulitis, abscess/ and gastrointestinaltract
gangrene,TB B . IN TR IN S IC
GA LLB LA D D E RD IS E A S E
4. ldiopathic(15%) 1. Cholecystectomy
eg, organtransplantation (liver[18%],kidney, 2. Anomalousposition
bone marrow),pulmonarydisease(chronic 3. Obstructionof cysticduct
obstructivepulmonarydisease, 4. Chroniccholecystitis
bronchopneumonia, asthma),drugs(steroids,
cytostatics),seizure Oral Cholecystogram (OCG)
Composition of colonic gas: Dose: 6 x 0.5 g tablets2 hoursafter eveningmeal
methane,carbondioxide,oxygen,nitrogen,hydrogen A. PATIENTSELECTION
P lainf ilm : . bilirubin<5 mg% (not necessaryif due to
0 Substantialamountnecessaryfor detection hemolysis)
r/ branchinglineargas densities: 0 Contraindicated in seriousliverdisease!
r/ in peripheryof liver extendingto within2 cm of 0 Relativecontraindications in peritonitis,
livercapsule postoperativeileus,acute pancreatitis!
{ predominantlywithin more anteriorlylocatedleft B. TOXICITY
lobeof liver 1. Nausea+ vomiting(alsonotedin 29% on
./ pneumatosisof intestinalwall placebo)
CT: 2. lmmediateanaphylacticresponse
0 Small amountof gas detectable 3. Delayedhypotensivereaction(increasedrisk in
./ tubularareas of decreasedattenuationin periphery cirrhosis)
of liver 4. Renalfailure
./ gas in superior/ inferiormesentericveins of hyperthyroidism
5. Precipitation
{ gas in smallmesentericveinsat mesentericborder
of bowel Nonvisualization on US
of Gallbladder
US: 1. Statuspost cholecystectomy
0 Small amountof gas detectable 2. Obscuredby costalmargin
{ intenselyhyperechoicfociwithinlumenof portal 3. Anomalousposition(intrahepatic, subphrenic)
vein + liverparenchyma 4. Gallbladder carcinomareplacinggallbladder
Doppler: 5. Perforationof gallbladder
r/ tall sharp bidirectionalspikes(overloadingof 6. Congenitalabsence
Dopplerreceiverfrom strongreflectionof gas 7. Contractedgallbladder
bubblein bloodstream) superimposed on normal (a) nonfastingstatuswithoutstones
portalvein spectrum (b) in fastingstatuswith stones
DDx: pneumobilia(locatedcentrallywithinbileducts { wall-echo-shadow (WEStriad) interfaces
closeto liverhilum+ withinleft lobeof liver)

Shadowingin GallbladderFossa
GA LLB LA DDE R 1. WES (wall-echo-shadow) triad
Nonvisualization of Gallbladder on OCG 2. Gas in duodenum/ colonobscuringgallbladder
Peak opacificationof gallbladder:14-19 hours 3. Porcelaingallbladder
(13-35%of dose excretedin urine) 4. Emphysematouscholecystitis
A. EXTRABILIARY CAUSES 5. Cholecystoentericfistula
1. Failureto ingestcontrast 6. Statuspost ERCPwith retrogradeair injection
2. Fasting
3. Failureto reachabsorptivesurfaceof bowel High-density Bile
(a) vomiting,nasogastricsuction 1. Hemorrhagic cholecystitis
(b) esophagealI gastricobstruction 2. H emobi l i a
 Differential Diagnosis of Hepatic, Biliary, Pancreatic, and Splenic Disorders 669

3. Priorcontrastadministration Small Gallbladder

(a) vicariousexcretionof urographicagent 1. Chroniccholecystitis
(b) cholecystopaque 2. Cysticfibrosis:in 2S/" of patients
4. Milkof calciumbile 3. Congenitalhypoplasia / multiseptatedgallbladder
4. Postprandial
DisplacedGallbladder 5. Intrahepaticcholestasis(viral,drug-related)
A . NO RM A LI MP R E SS IO N
by duodenum/ colon(positional change)
B. HEPATICMASS Gal l bl adder W al l Thi ckeni ng
hepatoma,hemangioma, regeneratingnodule, Diffuse Gallbladder Wall Thickening
metastases,intrahepaticcyst, polycysticliver, = anteriorwall of gallbladder>3 mm
hydatiddisease,heparlobatum(tertiarysyphilis), A. INTRINSIC
granuloma,abscess 1. Acutecholecystitis
C. EXTRAHEPATIC MASS 2. Chroniccholecystitis (10-25%)
1. Retroperitonealtumor(renal,adrenal) 3. Xanthogranulomatous cholecystitis
2. Polycystickidney 4. Hyperplastic cholecystosis(in 91% diffuse)
3. Lymphoma 5. Gallbladder perforation
4. Lymph node metastasisto porta hepatis 6. Sepsis
5. Pancreaticpseudocyst 7. Gallbladder carcinoma(in 41% diffuse)
8. AIDS cholangiopathy (averageof 9 mm in up
Alterationin GallbladderSize to 55%)
Enlarged Gallbladder 9. Sclerosingcholangitis
= CHOLECYSTOMEGALY
= HYDROPSOF GALLBLADDER 10. Gallbladder varices
Size: 11. Chemoinfusion of hepaticartery(ischemia)
(a) infants<1 year: >3 cm in length B . E X TR IN S IC
(b) children: >7 cm in length 1. Hepatitis(in 80%)
(c) adults: >4x10cm 2. H ypoal bumi nemi a
A . O B S T RU C T ION 3. R enalfai l ure
1. Cysticduct obstruction(40%) 4. Rightheartfailure
(a) Hydrops: chroniccysticduct obstruction 5. Systemicvenoushypertension
+ distensionwith clearsterilemucus(white 6. Hepaticvenousobstruction
bile) 7. Ascites
(b) Empyema:acute/ chronicobstruction with 8. Multiplemyeloma
superinfection of bile 9. Portalnode lymphaticobstruction
2 . Cholelithiasis causingobstruction(32%) 10. C i rrhosi s
3 . Cholecystitis with cholelithiasis(11%) 11. A cutemyel ogenous l eukemi a
4. Courvoisierphenomenon(1Oyo)= secondaryto 12. B rucel l osi s
neoplasticprocessin pancreas/ duodenal 13. Graft-versus-host disease
papilla/ ampullaof Vater/ commonbileduct C . P H Y S IOLOGIC
5 . Pancreatitis = contractedgallbladderafter eating
6 . Infection:leptospirosis, ascariasis,
typhoid
fever,scarletfever,familialMediterranean fever Focal Gallbladder Wall Thickening
B. UNOBSTRUCTED (mosttyneuropathic) A. METABOLIC
1. S/P vagotomy 1. Metachromatic sulfatides
2. Diabetesmellitus 2. Hyperplasticcholecystoses
3. Alcoholism B . B E N I G NT U M O R
4. Appendicitis (in chitdren) 1. Adenoma:gfandularelements(0.2%)
5. Narcoticanalgesia 2. Papilloma:fingerlikeprojections
(0.2%)
6. WDHAsyndrome 3. Villoushyperplasia
7. Hyperalimentation 4. Fibroadenoma
8. Acromegaly 5. Cystadenoma:? premalignant
9. Kawasakisyndrome 6. N euri noma, hemangi oma
10. A nt ich o l i n e rg i c s 7. C arci noi tumor
d
11. Bedriddenpatientwith prolongedillness C . MA LIGN A N T TU MOR
1 2 . A I D S( i n 1 8 % ) 1. Carcinomaof gallbladder:adenocarcinoma /
13. Dehydration squamouscell carcinoma(in 59% focal)
14. Prolongedfasting 2. Leiomyosarcoma
15. Totalparenteralnutrition 3. Metastases:from malignantmelanoma(15%\,
16. S eps i s lung,kidney,esophagus,breast,carcinoid,
c. NoRMAL (2%) Kaposisarcoma,lymphoma,leukemia
664 Radiology Review Manual

/ IN F EC T IO N
D. I NF LA M M AT ION Gas in BiliaryTree= Pneumobilia
1. lnflammatory polyp: in chroniccholecystitis mnemonic.' "l GET UP"
2. Parasiticgranuloma:Ascarislumbricoides, Incompetentsphincterof Oddi (aftersphincterotomy
/
Paragonimus westermani,Clonorchis, passageof a gallstone)
filariasis,Schistosoma, Fasciola Gallstoneileus
3. Intramuralepithelialcyst / mucinousretention Emphysematouscholecystitis(actuallyin gallbladder)
cyst Trauma
4. Xanthogranulomatous (in 9%
cholecystitis Ulcer(duodenalulcerperforating intoCBD)
focal) Postoperative(eg, cholecystoenterostomy)
E. WALL-ADHERENT GALLSTONE= embedded
stone ^/ gas outlinescholedochus + gallbladder
F. HETEROTOPIC MUCOSA r/ peripheralbranchesof bile ducts not filled
1. Ectopicpancreatictissue
2. Ectopicgastricglands ObstructiveJaundicein Adult
3. Ectopicintestinalglands Etiology:
4. Ectopichepatictissue A . B E N IGND IS E A S E(76% )
5. Ectopicprostatictissue 1. Traumatici postoperativestricture(44%)
2. Catculi(21%)
Filling Defects of Gallbladder 3. Chronicpancreatitis (8%)
Fixed Filling Defectsof Gallbladder 4. Sclerosingcholangitis(1%)
"PANTS" 5. Recurrentpyogeniccholangitis
mnemonic.'
Polyp 6. Parasiticdisease(ascariasis)
Adenomyomatosis 7. Livercysts
Neurinoma 8. Aorticaneurysm
Tumor,primary/ secondary 9. Papillarystenosis
Stone,wall-adherent B. MALIGNANCY(24%)
1. Pancreaticcarcinoma(18%)
Mobile Intraluminal Massin Gallbladder 2. Ampullary/ duodenalcarcinoma(8%)
3. Cholangiocarcinoma (3%)
sludge
1. Tumefactive
4. Metastaticdisease(2%)
2. Bloodclot
stone from stomach,pancreas,lung,breast,colon,
3. Nonshadowing
lymphoma
Gomet-tailArtifactin Liverand Gallbladder Level and cause of obstruction:
A . LI V E R A. INTRAPANCREATIC
1. Foreignmetallicbody (eg,surgicalclip) 1. C hol edochol i thi asi s
2. lntrahepaticcalcification 0 Most commoncause of biliaryobstruction(in
3. P neumo b i l i a 15"/"ol patientswith cholelithiasis)!
4. Multiplebileduct hamartomo= Vor Meyenburg 2. Chronicpancreatitis
complex 3. Pancreaticcarcinoma
B. GALLBLADDER B. SUPRAPANCREATIC (5%)
1. Rokitansky-Aschoff sinus = betweenpancreas+ porta hePatis
2. lntramuralstone 1. Cholangiocarcinoma
3. Cholesterolosis of gallbladder 2. MetastaticadenoPathY
c. PORTAHEPATIS(5%)
Ligament
EchogenicFat in Hepatoduodenal 1. Klatskintumor
= sign of pericholecystic
inflammation 2. Spreadfrom adjacenttumor(GB, liver)
1. Cholecystitis 3. Surgicalstricture
2. Perforatedduodenalulcer D. INTRAHEPATIC
3. Pancreatitis 1. Cystadenoma, cystadenocarcinoma
4. Diverticulitis 2. Mirizzisyndrome
3. Carolidisease
4. Cholangitis:recurrentpyogeniccholangitis,
BILEDUCTS sclerosingcholangitis, AIDS cholangitis
Hemobilia
1. latrogenictrauma: percutaneous needlebiopsy, lncidenceof infectedbib in bite duct obstruction:
I biliarydrainage/
transhepaticcholangiography (a) incomplete/ partialobstructionin 64%
PortographY (b) completeobstructionin 10%
2. Blunt/ penetrating
trauma 0 Infectiontwice as high with biliarycalculithan with
3. Ruptureof aneurysm/ pseudoaneurysm malignantobstruction !
 DifferentialDiagnosisof Hepatic,Biliary,Pancreatic,and SplenicDisorders 665

Organism; E. coli (21%),Klebsiella(21%), NeonatalObstructiveJaundice

enterococci(18%),Proteus(15%) = Sevef€persistentjaundicein a child beyond
3-4 weeks of age
Iesf Sensitivity for Common Bile Duct Obstruction:
1. Intravenous cholangiography Cause:
dependson levelof bilirubin:<1 mg/dl in g4o/"; A . IN FE C TION
<2 mg/dLin 82Y.; <3 mg/dl in 40%; >4 mg/dl in (a) bacterial: E. coli, Listeriamonocytogenes
< 10y " (b) viral: TORCH,Coxsackievirus,
False-negative rate: 45% echovirus,adenovirus
Cx: adversereactionsin 4-1O/" B. METABOLIC
2. US (a) inherited: alpha-1antitrypsin deficiency,
88-90% sensitivityfor dilatationof CBD cysticfibrosis,galactosemia,
0 ln 27-95Y" correctlevelof obstruction hereditarytyrosinemia
determinedby US (b) acquired: inspissated bilesyndrome
0 In 23-81Tocorrectcause of obstruction = "bileplug"syndrome
determinedby US (= cholestasisdue to
./ CgO >4-6 mm / 1Oh of patient'sage in years
erythroblastosis); cholestasis
r/ increasein CBD size afterfatty meil due to total parenteralnutrition;
^/ "Swisscheesesign"= abundanceof fluid-filled
choledocholithiasis
structureson liversections C. BILIARYTRACTABNORMALITIES
r/ intrahepatic"double
channel,'/,,shotgun"sign (a) extrahepatic: biliaryobstruction/ hypoplasia/
= two paralleltubularstructurescomposedof
atresia,choledochalcyst,
portalvein + dilatedintrahepaticbile ducts spontaneousperforationof bile
r/ intrahepaticbile duct >2 mm / >4Oo of adjacent
duct
portalvein branch (b) intrahepatic: ductularhypoplasia/ atresia
False-negative:noldilatedin acuteobstruction(in D. IDIOPATHICNEONATALHEPATITIS
70/"), sclerosingcholangitis,
intermittentobstructionfrom 0 The 3 most commoncausesof jaundicein
choledocholithiasis neonatesare hepatitis,biliaryatresia,and
False-positive: dilatedhepaticarteryin cirrhosis/ choledochalcystl
portalhypertensioni hepatic mnemonic.' "CAN"
neoplasm,patientsafter Choledochalcyst
cholecystectomy Atresia
3. CT Neonatalhepatitis
100%visualization in tumorousobstruction,
60'/" in nontumorousobstruction NUO-imagingregimen:
4. NUC (1) Premedication with phenobarbital(5 mg/kg/day)
r/ delayed/ nonvisualization of biliarysystem over 5 days to inducehepaticmicrosomal
, (93% specificity) enzymes,whichenhanceuptakeand excretionof
! vicariousexcretionof tracerthroughkidneys certaincompoundsand increasebileflow
DDx: Hepatocellulardysfunction (delayed (2) IDA scintigraphy(50 pCi/kg;minimumof 1 mCi)
clearanceof cardiacblood pool) (3) lmagingat S-minuteintervalsfor t hour
+ al2,4, 6, 8,24 hours

Hyperbilirubinemia
in Intants
= UNCONJUGATED HYPERBILIRUBINEMIA Jaundice in Older Children
A. P HY S I O LO GIC A. DISEASEOF HEPATOCYTES
Frequency; in 60/" of fullterm infants,in g0% of (a) hepatitis
preterminfants 1. Acutehepatitis:infection,toxicagents,drugs
Course: increaseby day 2-3, peak by day 5-7 (up 2. Chronichepatitis
to 12 mg/dl in full-termbabies,up to (b) metabolic
14 mg/dl in prematureinfants) 1. W i l sondi sease
0 Breast-fedbabiesmay have an elevatedbilirubin 2. Cysticfibrosis
level untilthe end of 2nd week of lifel 3. Glycogenstoragedisease
B. NONPHYSIOLOGIC 4. Tyrosinemia
. ons€tof jaundicewithinfirst24 hours 5. Alpha-1antitrypsin deficiency
. persistent/ new-onsetjaundicein infants2 weeks B. OBSTRUCTION
of age (a) malignantneoplasm
. rise of serum bilirubin>5 mg/dl per 24 hours 1. Hepatoblastoma
. directbilirubinlevel>1 mg/dL 2. Hepatocellular carcinoma
666 Radiology Review Manual

3. Sarcomas:angiosarcoma, lymphosarcoma, 3. Fibroma,lipoma,neuroma

rhabdomyosarcoma of bile ducts, 4. Granularcell myoblastoma (= Schwann-cell-
undifferentiatedembryonalsarcoma derivedbiliarytumor)in youngblackwoman
4. Metastaticdisease: neuroblastoma, Wilms E . P A R A S ITE S
tumor,leukemia/lymphoma 1. Ascarislumbricoides:longlinearfillingdefect/
(b) benignneoplasm discretemass if coiled
1. Infantilehemangioendothelioma 2. Liverfluke(Clonorchis sinensis,Fasciola
2. Mesenchymal hamartoma hepatica) : intrahepatic
epithelialhyperplasia,
(c) benignstricture periductalfibrosis,cholangitis,
liverabscess,
(d) cholelithiasis (uncommon)
/ choledocholithiasis hepaticduct stones,commonduct obstruction
3. Schistosoma japonicum:portalvein infection
3. Hydatidcyst: after erosioninto biliarytree
LargeNonobstructed
CBD
1. Passageof stone (returnto normalafterdays to Echogenic Materialin Bile Ducts
weeks) 1. Calculi
2. Commonduct surgery(returnto normalin 30-50 2. Gas
days) 3. Blood
3. Postcholecystectomy dilatation(in up to 16%) 4. Tumor
4. lntestinalhypomotility 5. Parasites
5. Normalvariant(aging)
Fatty-mealsonography(to differentiatefrom obstruction
wilh 74/" sensitivity,100/" specificity) Bile DuctNarrowing
Method: peroralLipomul(1.5 mUkg) followedby A. BENIGNSTRTCTURE (44%)
100 mL of water[cholecystokinin causes (a) trauma
contractionof gallbladder,relaxationof 1. Postoperative stricture(95-99%)
sphincterof Oddi,increasein bile associatedwith cholecystectomy
secretionl,CBD measuredbeforeand 45 i 2. Blunt/ penetrating trauma
60 minutesafterstimulation 3. Hepaticarleryembolization
r/ titttechange/ decreasein size = flofrTl?lresponse 4. Infusionof chemotherapeutic agents
r/ increasein size >2 mm = partialobstruction (b) inflammation
1. S cl erosi ng
chol angi ti s
FillingDefectin Bile Ducts 2. Recurrentpyogeniccholangitis
A. ARTIFACT 3. Acute/ chronicpancreatitis
1. P s eud o c a l c u l u s 4. Pancreaticpseudocyst
(a) contractedsphincterof Boyden+ Oddi with 5. Perforated duodenalulcer
smootharcuatecontour 6. Erosionby biliarycalculus
(b) bridgeof tissuebetweencysticduct + CHD 7. Gallstones+ cholecystitis
(c) underfilling of cysticduct duringERCP 8. Abscess
(d) admixturedefectat cysticduct junction L Radiationtherapy
2. Air bubble: confirmedby positionalchanges 10. Papillarystenosis
3. Bloodclot: spheroidconfiguration, spontaneous 11. Acquiredimmunodeficiency syndrome
resolutionwith time (c) congenital
B . B I LI A RYC A L C U L I 1. Choledochal cyst
c. M I RI Z Z SI YN D R O ME B . MA LIGN A N T S TR IC TU R E
D . NEOPLASM 1. Pancreaticcarcinoma
(a) malignant 2. Ampullarycarcinoma
1. Cholangiocarcinoma: irregularstricture, 3. Cholangiocarcinoma
intraluminal polypoidmass 4. Compression by enlargedlymphnode
2. Metastatictumor (Gl tract,pancreas,breast, 5. Metastasis
melanoma,lymphoma)
3. Others: ampullarycarcinoma,hepatoma,
hamartoma,carcinoid,embryonal MultifocalIntrahepatic
Bile DuctStrictures
rhabdomyosarcoma of biliarytree 1. Primarysclerosingcholangitis
(b) benign 2. Ascendingcholangitis due to stricture/ stone/ bile
1. Papilloma(mostcommonbenignneoplasm) duct anomaly
Histo: vascularconnectivetissuecoveredby 3. Ori ental chol angi ohepati ti s
singlelayerof columnarepithelium 4. AIDS-related cholangitis
2. Adenoma 5. l schemi a
Histo: epithelialglandulartissue surrounded (a) floxuridinetreatment
by fibroustissue (b) hepaticarterialthrombosis(in livertransplant)
 DifferentialDiagnosisof Hepatic,Biliary,Pancreatic,and SplenicDisorders 667

6. Neoplasm CongenitalBiliaryCysts
(a) cholangiocarcinoma (Todani cl assification)
(b) metastases
l. Choledochalcyst (77-87%)
7. Previousbile surgery fA cysticdilatationof CBD
8. Congenitalbiliaryanomalies lB focalsegmentaldilatationof CBD
lC fusiformdilatationof CBD
ll. Diverticulum of extrahepatic
ducts(1.2-g%)
PapillaryStenosis
originating from CBD / CHD
Etiology:
r/ neck of diverticulumopen / closed
A . pRt M A R yp Ap tL L A R yS T EN O S | S(1 0 % )
lll. Choledochocele (1.4-6%)
1. Congenitalmalformation of papilla lV. Multiplesegmentalbile duct cysts
2. Sequelaeof acute/ chronicinflammation
IVA multipleintra-and extrahepaticbiliarycysts
3. Adenomyosis
+ sacculardilatationof CBD (19%)
B. SECONDARYpAplLLARy STENOSIS(90%)
IVB multipleextrahepaticbiliarycysts + normal
1. Mechanicaltraumaof stonepassage
intrahepaticbile ducts (rare)
(choIedochoIithiasis in 64/"; cholecysto Iithiasis V. Carolidisease= intrahepaticbiliarycysts
in 26%)
2. Functionalstenosis: associatedwith pancreas
divisum,historyof pancreatitis PANCREAS
3. Reflexspasm = papillarydyskinesia CongenitalPancreaticAnomalies
4. Scarfrom previoussurgicalmanipulation 1. Pancreasdivisum
5. Periampullaryneoplasm 2. Annularpancreas
r/ prestenoticdilatationof CBD 3. Agenesisof dorsalpancreas
r/ increasein pancreaticduct diameter(83%) May be associated with:
r/ long smoothnarrowinglbeak(fibroticstenosis) abnormalsitus,polysplenia,intestinalmalrotation
r/ prolongedbile-to-boweltransittime >45 minuteson
Tc-lDAscintigraphy
Pancreatic Calcitication
1. C H R ON ICP A N C R E A TITIS
Periampullary
Tumor Numerousirregularstippledcalcifications of varying
1. Pancreatic (BS%)
carcinoma size; predominantlyintraductal
2. Cholangiocarcinoma
of distalcommonbileduct(6%) (a) Alcoholicpancreatitis(in 20-50%):
3. Ampullary
tumor(4%) r/ calcificationslimitedto head I latl in 25"/"
4. Duodenalwalltumor (b) Biliarypancreatitis(in 2%)
adenocarcinoma,
adenoma, carcinoid,
smooth (c) Hereditarypancreatitis(in 35-60%):
muscletumor r/ roundcalcifications throughoutgland
(d) ldiopathicpancreatitis
Double-DuctSign (e) Pancreaticpseudocyst
= dilatationof commonbileduct+ pancreatic
duct 2. NEOPLASM
1 . Ampullary tumor(mostcommon) (a) Microcysticadenoma(in 33%):
2 . Otherperiampullarytumor { "sunburst"appearanceof calcifications
3 . Papillarystenosis (b) Macrocystic cystadenomaIn 15%):
4. Stone impactedin ampullaof Vater { amorphousperipheralcalcifications

TypeI TypeII TypeIII Type IVa Type IVb Type V

CholedochalCyst Diverticulum Choledochocele SaccularDilatation SaccularDilatation Caroli Disease
of CBD + of CBD
IntrahepaticDucts
Classificationof CongenitalBiliary Cysts
668 Radiology Review Manual

(c) Adenocarcinoma(in 2%| with "sunburst"pattern (b) Acinarcell origin

(d) Cavernouslymphangioma / hemangioma: 1. A ci narcel lcarci noma(1% )
r/ multiplephleboliths 2. Adenoma
(e) Metastasesfrom coloncancer (c) Indeterminate origin
3. I NT RA P A R E N C H Y M AL
H EMOR R H AGE 1. Pancreatoblastoma = infantilepancreatic
(a) Old hematoma/ abscess/ infarction carcinoma
(b) Ruptureof intrapancreatic aneurysm 2. Dermoidcyst
4. HYPERPARATHYROIDISM (in 20o/o): B . E N D OC R IN EN E OP LA S M
0 50% of patientsdevelopchronicpancreatitis (a) Nonfunctioning isletcelltumor
+ concomitantnephrocalcinosis (b) Functioning isletcelltumor
0 Indistinguishable from alcoholicpancreatitis 1 . I n s u l i n o m(aBc e l l s )
5. CY S T I CF IBR O S IS 2. Glucagonoma
Fine granularcalcificationsimplyadvanced 3. Gastrinoma(6 cells)
pancreaticfibrosis 4. Somatostatinoma
6. HE M O CH R O MA T O S IS 5. V l P oma(W D H Asyndrome)
7. KWASHIORKOR = juveniletropicalpancreatitis 6. "PP-oma"= pancreaticpolypeptide
0 Indistinguishable from alcoholicpancreatitis 7. Carcinoid
c. N ON E P ITH E LIAOR L IGIN
Atrophyof Pancreas (a) Primarytumor
1. Main pancreaticduct obstruction 1. P ri maryl ymphoma
2. Cysticfibrosis <1"/"of pancreaticneoplasms
0 Mostcommoncausein childhood! 2. Primitiveneuroectodermal tumor
3. Schwachman-Diamond syndrome 3. Rhabdomyosarcoma
4. Johanson-Blizzardsyndrome (= pancreatic (b) Metastases
nasalalar hypoplasia,
insufficiency, absenceof 1. Secondarylymphoma:
permanentteeth,shortstature,congenitaldeafness) ^/ largehomogeneous solidmass,
5. Hemochromatosis infrequentlywith centralcysticarea
6. Viralinfection r/ peripancreaticnodal masses
7. Malnutrition { peripancreatic vesselsdisplaced+ stretched
8. Cushingqyndrome,steroidtherapy,obesity 2. Primitiveneuroectodermal tumor
3. Kaposisarcoma
4. Renalcell carcinoma
PancreaticMass 5. Mel anoma
A. NEOPLASTIC 6. Lungcancer
1. Adenocarcinoma 7. Breastcancer
2. lsletcell tumor 8. Ovariancancer
3. Cystadenoma/ -carcinoma 9. Hepatocellular carcinoma
4. Solidand papillaryneoplasm 10. S arcoma
5. Lymphoma
B. INFLAMMATORY HypervascuIar Pancreatic Tumors
1. Acutepancreatitis A . P R IMA R Y
2. Pseudocyst lsletcelltumor,microcysticadenoma,solidand
3. Pancreaticabscess papillaryepithelialneoplasm
B. METASTASESfrom
PancreaticNeoplasm angiosarcoma, leiomyosarcoma, melanoma,
Origin: - in 99% exocrineductalepithelium carcinoid,renalcellcarcinoma,adrenal
- in 1o/oacinarportionof pancreaticglands carcinoma,thyroidcarcinoma
- in 0 .1 % ma l i g n a nat m p u l l a ry
tu morw i th
betterprognosis PancreaticGyst
A. INFLAMMATORY / INFECT]OUS
A. EXOCRINENEOPLASM (a) pseudocyst(85%): secondaryto obstructive
(a) Ductalcell origin tumor/ trauma/ acutepancreatitis (in 24h),
1. Ductaladenocarcinoma (90%) chronicpancreatitis(in 10-15%)[developwithin
2. Ductectatic mucinoustumor 10-20 days, consolidatedafter 6-8 weeksl
= fi ucin-hypersecreting
carcinoma (b) acquiredcyst:
3. Cysticneoplasm(10-15%) 1. Retentioncyst (= exudatewithinbursa
- serousmicrocystic neoplasm omentalisfrom acute pancreatitis)
- mucinousmacrocysticneoplasm 2. Parasiticcyst: Echinococcus multilocularis,
4. Solidand papillaryepithelialneoplasm(rare) amebiasis
5. Cysticchangesof von Hippel-Lindaudisease 3. Pancreaticabscess
 DifferentialDiagnosisof Hepatic,Biliary,Pancreatic,and SplenicDisorders OOg

B. CONGENITAL(rare) E. RENAL
(a) solitarytrue cyst 1. Transplantation
(b) multipletrue cysts (whenassociatedwith cystic 2. Renalinsufficiency
diseaseof the liver/ otherorgans): F. METABOLIC
1. Autosomaldominantpolycystickidney 1. Diabeticketoacidosis
disease(hepaticcysts in g0/. at autopsy) 2. Drugs
r/ nearlyalwaysassociatedwith renaicysts G. PNEUMONIA
2. Von Hippel-Lindau disease(pancreaticcysts H. SALIVARYGLANDLESION
in7 2 /" a t a u to p s yi;n o n l y2 5 % o n C T) 1. Faci altrauma
3. Cysticfibrosis 2. Mumps
C. NE O P LA ST IC
(a) cysticpancreaticneoplasm(S-1S%): S P LE E N
<5/" of all pancreatictumors Nonvisualization of Spleen
1. Serouscystadenoffl?= microcysticadenoma 1. Aspleniasyndrome
2. Mucinouscystictumors 2. P ol yspl eni asyndrome
- peripheralbranch-duct tumor= mucinous 3. Traumaticfragmentation of spleen
cystadenoma/ cystadenocarcinoma 4. Wanderingspleen
(= ffi€lcfocysticadenoma)
- mainduct tumor= intraductal papillary Small Spleen
mucinousadenoma/ adenocarcinoma 1. Infarcti on
3. Solidand papillaryepithelioid neoplasm 2. Celiacdisease
4. Cysticisletcell tumor (rare) 3. Congenital/ hereditaryhypoplasia
5. Pancreaticsarcoma(extremelyrare) 0 associatedwith recurrentbacterialinfections
(b) cysticmetastases(3-12'h at autopsy):
4. Fanconianemia
renalcell carcinoma,melanoma,lungtumors, 5. lrradiation
breastcarcinoma,hepatocellular carcinoma, 6. Partialsplenectomy
ovariancarcinoma 7. Polysplenia syndrome
(c) retroperitoneal lymphangioma / hemangioma 8. Atrophy
Microcystic Lesionof Pancreas Splenomegafy
- pancreaticlesionwith >6 cysts each <2 cm in size
{ inferiortip of spleenextendsbelowtip of rightlobeof
1. Pancreaticadenocarcinoma S0% liver
2. Microcystic adenoma 19% r/ np diameterof spleen>213of abdominaldiameter
3. Pancreatitis 11%
4. Metastases 6% A. CONGESTIVESPLENOMEGALY
5. Mucinouscystadenocarcinoma S% heartfailure,portalhypertension, cirrhosis,cystic
6. lsletcell carcinoma g% fibrosis,portal/ splenicvein thrombosis,acute
7. Lymphoma g% splenic sequestrationcrisisof sicklecell anemia
8. Sarcoma g% B . N E OP LA S M
leukemia,lymphoma,lymphoproliferative disease,
Hyperamylasemia Langerhanscell histiocytosis, metastases,primary
A. PANCREATIC neoplasm
1. Acute/ chronicpancreatitis C. STORAGEDISEASE
2. Pancreatic trauma Gaucherdisease,Niemann-Pick disease,
3. Pancreaticcarcinoma mucopolysaccharidoses, gargoylism,amyloidosis,
B. GASTROINTESTINAL diabetesmellitus,hemochromatosis
1. Perforatedpepticulcer D . IN FE C TION
2. Intestinalobstruction (a) bacterial:TB, subacutebacterialendocarditis,
3. Peritonitis typhoidfever,syphilis,brucellosis
4. Acute appendicitis (b) viral: hepatitis,infectiousmononucleosis
5. Afferentloopsyndrome (c) protozoal:echinococcosis, malaria,kala azar,
6. Mesentericischemia/ infarction Americanleishmaniosis
7. Portalveinthrombosis (d) fungal: histoplasmosis
C. TRAUMA E . H E MOLY TIC A N E MIA
1. Burns hemoglobinopathy,hereditaryspherocytos is, primary
2. Cerebraltrauma neutropenia, thromboticthrombocytopenic purpura,
3. Postoperative extracorporealmembraneoxygenation(due to RBC
D. OBSTETRICAL damage)
1. P r egna n c y F. EXTRAMEDULLARY HEMATOPOIESIS
2. Rupturedectopicpregnancy osteopetrosis,myelofibrosis
670 Radiology Review Manual

G. COLLAGENVASCULARDISEASE r/ hepatosplenomegaly
systemiclupuserythematosus,rheumatoidarthritis, { abdominaladenopathy(meansize of 2.6 cm)
Feltysyndrome 5. Gaucherdisease(islandsof RES cellsladenwith
H. S P LE NI CTR AU M A glucosylceramide)
I . O T HE RS 6. Inflammatory pseudotumor
1. Sarcoidosis 7. Lymphangioma
{ splenomegaly in up to 60% C . S P LE N ICIN FA R C TION
r/ inhomogeneousenhancementafter bolus
injection(multiple2-3-cm hypodensenodular
lesions) CysticSplenicLesion
{ necroticmass with focal calcifications A . C ON GE N ITA L
2. Hemodialysis 1. Epidermoidcyst = true cyst = congenitalcyst
3. Autoimmunelymphoproliferative syndrome
B. VASCULAR
SolitarySplenicLesion 1. Spleniclaceration / fracture
mnemonic.' "L'CHAIM" 2. Hematoma
Lymphoma 3. False cyst = posttraumaticcyst = rofrpohcreatic
Cyst pseudocystof the spleen
Hematoma,Hemangioma,Hamartoma 0 80% of all spleniccysts are pseudocysts
Abscess (= secondarycysts)
Infarct Cause: cysticend stage of trauma,infection,
Metastasis infarction
r/ internalechoesfrom debris
withincyst wall may resemble
r/ calcifications
Solid Splenic Lesion eggshell
A . M A LI G NA N T U M O R { smallersizethan true cyst
1. Lymphoma(Hodgkindisease,non-Hodgkin 4. Cysticdegenerationof infarct
lymphoma,primaryspleniclymphoma) (a) occlusionof splenica. / branches(hemolytic
0 Splenomegaly in non-Hodgkin
lymphoma anemia,endocarditis, SLE,arteritides,
indicatesinvolvementin most patients pancreaticcancer)
0 30% of patientswith splenomegalyhave no (b) venousthrombosisof splenicsinusoids
involvement from non-Hodgkinlymphoma (massivesplenomegaly)
0 30% of patientswith lymphomaof any kind 5. Peliosis
havesplenicinvolvement withoutsplenomegaly
{ homogeneoussplenomegaly(fromdiffuse C . IN FE C TION / IN FLA MMA TION
infiltration) 1. Pyogenicabscess
{ miliarynodules Prevalence: 0.14.7"/"
r/ large2-1O-cmnodules(10-25/") Cause: hematogenousspreadin sepsis(75%),
r/ nodesin splenichilum(50%)in NHL; penetratingtrauma(15%), infarction
uncommonin Hodgkindisease (10%)
2. Metastasis(7%) Predisposed;endocarditis,drug abuse,
melanoma(6-34/"), breastcarcinoma penetratingtrauma,neoplasm,
(12-21%),bronchogeniccarcinoma(9-18%), sicklecelldisease
coloncarcinoma(4/"), renalcell carcinoma(3%), . fever,chills,LUQ pain (in <50%)
ovary (8/"), prostate(6%),stomach(7"/"), ^/ irregularborderswithoutcapsule
pancreas,endometrialcancer ! gas bubbleswithinabscess
3. Angiosarcoma { rim enhancement
4. Malignantfibroushistiocytoma,leiomyosarcoma, Rx: 767osuccessrate for percutaneousdrain
fibrosarcoma 2. Microabscesses
5. Langerhanscell histiocytosis Organism; fungus (especiallyCandida,
r/ splenomegaly Aspergillus,Cryptococcus)
r/ multiplehypoechoicnodules(lessoften) Prevalence: 26/" of splenic abscesses
B . B E NI G NT U MOR Predisposed: immunocompromised patient
1. Hamartoma= splenoma r/ hepatosplenomegaly
2. Hemangioma r/ multipleround hypoechoic/ hypoattenuating
"target"lesionsof 5-10 mm often associated
3. Hematopoietic
4. Sarcoidosis with hepatic+ renal involvement
r/ nodularlesionsin liverand spleenin 5-15% ri "wheel-in-wheel" appearancewhen central
(= coalescentgranulomata)occurringwithin hyperechoicportionbecomesnecrotic
5 years of diagnosis + hypoechoic
 Differential Diagnosis of Hepatic, Biliary, Pancreatic, and Splenic Disorders 671

3. Granulomatous infection 2. Granuloma(mostcommon): histoplasmosis,

TB,
(a) Mycobacterium tuberculosis:miliaryTB brucellosis
r/ mildsplenomegaly uncommon B . C A P S U LA R& P A R E N C H Y MA L
(b) M. avium-intracellulare 1. Pyogenici tuberculous abscess
r/ markedsplenomegalyin 20./. 2. Pneumocystis cariniiinfection
4. Pneumocystis cariniiinfection 2. Infarction(multiple)
r/ splenomegaly + multiplehypoattenuatingfoci 3. Hematoma
5. Parasiticcyst (Echinococcus) C. VASCULAR
Prevalence: in <2h of patientswith hydatid 1. Splenicarterycalcification
disease 2. Splenicarteryaneurysm
Cause: systemicdissemination, intraperitoneal 3. Splenicinfarct
spreadof rupturedlivercyst D. CALCIFIEDCYSTWALL
r/ solitarycyst + subjacentdaughtercysts 1. Congenitalcyst
r/ nydatiOsand + infoldedmembranes 2. Posttraumaticcyst
./ + linearcalcification 3. Echinococcalcyst
6. Intrasplenicpancreaticpseudocyst 4. Cysticdermoid
Prevalence: in 1-5% of patientswith 5. E pi dermoi d
pancreatitis
mnemonic; "HlTCH"
D. CYSTICNEOPLASM Histoplasmosis (mostcommon)
1. Cav ern o u h
s e m a n g i o ma Infarct(sicklecell disease)
0 Mostcommonprimaryneoplasmof the spleen! Tuberculosis
2. Lymphangiomailymphangiomatosis Gyst(Echinococcus)
r/ septatesubcapsularcysticlesions Hematoma
3. Lymphoma(mostcommonmalignantneoplasm!)
4. Necroticmetastasis: lron Accumulationin Spleen
0 ln7"/" of patientswith widespreadmetastasis! A. DIFFUSE
malignantmelanoma(in 50%);breast,lung, 1. Multiplebloodtransfusions
ovarian,pancreatic, endometrial,
colonic, 2. Sicklecell anemia
prostatic,carcinoma;chondrosarcoma B. FOCAL
1. GamnaGandybodi es
E. TRUE CYST (withepithetiat lining) 2. Angiosarcoma
1. Congenitalcyst = epidermoidcyst
2. Parasiticcyst
HyperechoicSplenicSpots
F. FALSECYST= PSEUDOCYST(tackingepithetial 1. Granulomas:miliarytuberculosis,
histoplasmosis
lining) 2. P hl ebol i ths
1. Traumaticcyst 3. Lymphoma/ leukemia
2. Postinfarctcyst 4. Myelofibrosis
5. Gamna-Gandynodules(in portalhypertension)
IncreasedSplenicDensity
1. Sicklecell anemia(in 5% of sicklers)
2. Hemochromatosis SpontaneousSplenicRupture
3. Thorotrastexposure 1. Posttraumatic
delayed
rupture
4. Lymphangiography 2. Splenomegaly
3. Hemangioma
Splenic Calcification 4. Epidermoidcyst
A . DI S S E M IN A T ED 5. Peliosis
1. Phlebolith:visceralangiomatosis 6. Previoussplenicinfarction
672 Radiology Review Manual

ANATOMYOF LIVER,BILEDUCTS,ANDPANCREAS

cystic v. PORIAL V. coronary v. superior polar v.

pyloric v.
short gastric vv.
posterior superior pancreaticoduodenalv.

single pancreaticoduodenalv. splenic hilar vv.

subpyloric v.
gastrocolic trunk splenic v.

anterior superior pancreaticoduodenalv. pancreatic vv.

right superior colic v. inferior mesenteric v.
inferior pancreaticoduodenalv.
superior mesenteric v.

middle colic v.

gastroepiploic v.
right colic v.

omental v.

intestinal vv.

Extrahepatic Portal Vein Tfibutaries

- RPS absent horizontal

segment

LLS
\
I
I

Wg;ii.,j,.1
R A
iE'
G,
-sffirn

LLI ./

.t . a::
Variations of Intrahepatic Portal Venous System
(ZOVo)
A. LEFT PORTAL VEIN
1. Absenceof horizontal segment(0.2Vo)
B. RIGHT PORTAL VEIN
1. Trifurcation of main portal vein (ll%o)
Intrahepatic Portal Vein Branches 2. Origin of RP segmentfrom main portal vein (5Vo)
3. Origin of RA segmentfrom left portal vein(4Vo)
4. Absence of main right, RA, and RP portal segments

R A = right anterior segment RPI right posterior inferior LMI left median inferior
RAI = right anterior inferior RPS right posterior superior LMS left median superior
RAS = right anterior superior C caudatelobe LLI left lateral inferior
R P = right posterior segment L left portal vein LLS left lateral superior
 Anatomyof Liver,Bile Ducts,and Pancreas O7g

^^-
r1 . W
{tu
Wwww

5
il ,,
6

Hepatic Vein Junction Left Portal Vein

Level of
Right Portal Vein Splenic Vein

FunctionalSegmentalLiverAnatomy
Goldsmith & Woodburne Couinaud & Bismuth

CAUDATELOBE Caudatelobe 1
LEFTLOBE Left lateralsegment Left lateralsuperiorsubsegment 2
Left lateralinferiorsubsegment 3
Left medialsegment Leftmedialsuperiorsubsegment 4a
Leftmedialinferiorsubsegment 4b
RI G HTL OB E Rightanteriorsegment Rightanteriorinferiorsubsegment 5
Rightanteriorsuperiorsubsegment 8
Rightposteriorsegment Rightposteriorinferiorsubsegment 6
Rightposteriorsuperiorsubsegment 7
674 Radiology Review Manual

right, middle,left replaced replaced replaced

hepatic a. right hepatic a. right hepatic a. left hepatic a.

" left gastric a.

proper
hepatic a.

splenic a.
celiac
trunk

I
common
hepatic a.
\ sM^r.
Type1 (55Vo) Type2 (l0Vo) Type3 (lLVo) Type 4 (lVo)

accessory accessory accessory accessory common

left hepatic a. right hepatic a. right hepatic a. left hepatic a. hepatic a.
-t'""'rJ..
,

Type 5 (8%o)
I Type6 (7Vo) Type7 (LVo) Type9 (4.5Vo)

Michels Classification of Hepatic Arterial Anatomy

LIVER Type 2 (10%):

- commonhep.a. dividesintogastroduodenal
FunctionalSegmentalLiverAnatomy
basedon distributionof 3 major hepaticveins: + RT hep. a.
- LT hep. a. replacedto LT gastrica.
(a) middlehepaticvein
- middlehep.a. from RT heP.a.
dividesliverinto rightand left lobe
also separatedby main portalvein scissura(Cantlie Type3 (11% ):
- commonhep.a. dividesintogastroduodenal + LT
line)passingthroughIVC + longaxisof gallbladder)
(b) left hepaticvein hep.a.
- RT hep. a. replacedto superiormesenterica.
dividesleft lobe into medial+ lateralsectors
- middlehep.a. from LT heP.a.
(c) right hepaticvein
dividesright lobe into anterior+ posteriorsectors Type 4 (1%):
- commonhep.a. dividesintomiddlehep.a.
Eachof the four sectionsis furtherdivided:
by an imaginarytransverselinedrawnthroughthe right + gastroduodenala.
- RT hep. a. + LT hep. a. are both replaced
+ left portalvein into anterior+ posteriorsegments;the
segmentsare numberedcounterclockwise from IVC Type 5 (8%):
- accessoryLT hep. a. arisesfrom LT gastrica'
Hepatic Arterial Anatomy (Michelsclassification) Type 6 (7%):
- accessoryRT hep.a. arisesfrom superior
Type 1 (55%):
- celiactrunk trifurcatesinto LT gastrica. + splenic mesenterica.
a. + commonhep.a. Type7 (1% ):
- commonhep.a. dividesintogastroduodenal - accessoryRT + LT hePatica.
a.
+ properhep.a. Type 8 (2%):
- RT hep.a. + LT hep.a. arisefrom properhep. a. - combinationsof accessory+ replacedhepaticaa.
- middlehep. a. (supplyingcaudatelobe) arises Type I (4.5%):
- hepatictrunkreplacedto superiormesenterica.
from
(a) LT / RT hep. a. Type 10 (0.5% ):
- hepatictrunk replacedto LT gastrica.
(b) properhep.a. (in 10%)
 Anatomyof Liver,Bile Ducts,and Pancreas 675

AberrantHepaticArtery
= hepaticarterycoursingbetweenIVC + portalvein
1. Replacedright hepaticartery (50%)
2. Righthepaticarterywith early bifurcationof common
hepaticarteryinto right + left hepaticarteries(20%)
3. Accessoryrighthepaticartery(15%)
4. Replacement of entirehepatictrunkto SMA (15%)

Third Inflowto Liver

= aberrantveinssupplyingsmallareasof livertissue fissure for
+ communicatingwith intrahepaticportalvein lig. venosum
branches
Effect: focal decreaseof portalvein perfusionresulting
in areasof fat-sparing/ fat accumulation
1. Cholecystic veins
directlyenteringliversegments4 + 5
veinsjoiningthe parabiliary veinsvia triangleof Hepatic Fissures
Calot
2. Parabiliaryvenoussystem 2. Fissurefor ligamentumvenosum
= VefloUSnetworkwithinhepatoduodenalligament = invaginationof obliteratedductusvenosus
anteriorto main portalvein = embryologicconnectionof left portalvein with left
Tributaries: hepaticvein
cholecystic vein throughtriangleof Calot - separatescaudatelobe from left lobe of liver
pancreaticoduodenal vein - lesseromentumwithinfissureseparatesthe
rightgastric/ pyloricvein greatersac anteriorlyfrom lessersac posteriorly
r/ pseudolesionat dorsalaspectof segment4 3. Fissurefor gallbladder(GB)
3. Epigastric-paraumbilical venoussystem = shallowperitonealinvagination containingthe GB
= SrT't€lllveins aroundfalciformligamentdraining - dividesrightfrom left lobe of liver
anteriorpart of abdominalwall directlyinto liver 4. Transversefissure
Subgroups: = invaginationof hepaticpedicfeinto liver
(a) superiorvein of Sappey - containshorizontalportionof left + rightportal
drainsupperportionof falciformligament veins
+ medialpart of diaphragm 5. Accessoryfissures
entersperipheralleft portalvein branches (a) Rightinferioraccessoryfissure
communicateswith superiorepigastric = from gallbladderfossa/ just inferiorto it to
+ internalthoracicveins lateroinferior
marginof liver
(b) inferiorvein of Sappey (b) Others(rare)
drainslowerportionof falciformligament
entersperipheralleft portalvein branches Size of Liver
communicateswith branchesof inferior A . Y OU N GIN FA N T
epigastricvein aroundthe umbilicus right hepaticlobe shouldnot extend>1 cm below
(c) vein of Burow rightcostalmargin
terminatesin middleportionof collapsed B. CHILD
umb i l i c a l v e i n right hepaticlobe shouldnot extendbelow right
communicates with branchesof inferior costalmargin
epigastricvein aroundthe umbilicus C. ADULT
(d) intercalaryveins (a) midclavicularline (vertical/craniocaudadaxis):
interconnect vein of Burow+ inferiorvein of <13 cm = flo;ffi?l
Sappey 13.0-15.5cm = indeterminate (in 2S%)
>15.5cm = hepatomegaly (87%accuracy)
HepaticFissures (b) preaorticline<10 cm
1. Fissure teres= umbilical
forligamentum fissure (c) prerenalline<14 cm
= invagination of ligamentum teres= embryologic
remnantof obliteratedumbilicalvein connecting LiverEchogenicity& Attenuation
placentalvenousbloodwith left portalvein US: pancreatic> hepatic> renalechogenicity
- locatedat dorsalfree marginof falciform
CT: 40-70 HU (precontrast)
ligament CECT: earlyarterialphase (20 sec), late arterial
- runs into liverwith visceralperitoneum phase (30-40 sec), portalvenousphase
- dividesleft hepaticlobe into medial+ lateral
(60-70 sec);maximalenhancementat
segments(dividessubsegment3 from 4) 45-60 sec
676 RadiologyReviewManual

MaximumCross-sectional
Diameterof PortalVein (b) conjugated/ directbilirubin= water-soluble
( a)c hild< 10 y e a rso f a g e : 8 .5 m m Formation; conjugationin livercells
(b) 10-20 yearsof age: 10.0mm Metabolism;excretioninto bile; not reabsorbed
( c ) adult : 1 3 .0m m by intestinalmucosa+ excretedin
feces
Parameter
NormalHemodynamics of Liver Elevation:
Portalvein velocity: >11 cm/sec overproduction: hemolyticanemia,resorption
Congestionindex (= cfoss-sectionalarea of portalvein of hematoma,multipletransfusions
dividedby averagevelocity): 0.070+ 0.09 decreasedhepaticuptake:drugs,sepsis
Hepaticarteryresistiveindex: 0.60-0.64+ 0.06 decreasedconjugation: Gilbertsyndrome,
neonataljaundice,hepatitis,cirrhosis,sepsis
LiverTests decreasedexcretioninto bile: hepatitis,
A. Alkalinephosphatase(AP) cirrhosis,drug-induced cholestasis,sepsis,
Formation; bone, liver,intestine,placenta extrahepaticbiliaryobstruction
High increase: cholestasiswith extrahepaticbiliary E . Lacticdehydrogenase (LDH)
obstruction(confirmedby rise in nonspecific and thereforenot helpful
high increase: primaryor metastaticliver
1GT),drugs,granulomatous
disease(sarcoidosis), primary involvement
biliarycirrhosis,primary F. Alphafetoprotein(AFP)
+ secondarymalignancyof liver >400 ng/ml stronglysuggeststhat focal mass
Mild increase: all forms of liverdisease,heart representsa hepatocellular carcinoma
failure
B. Gamma-glutamyl transpeptidase(TGT) BILEDUCTS
very sensitivein almostall formsof liverdisease NormalSizeof Bile Ducts
Utility: confirmshepaticsourceof elevatedAP @ CBD at pointof maximumdiameter= free edge of
may indicatesignificant alcoholuse gastrohepaticligament(pointof leastconstraint):
C. Transaminases (a) adolescents& adults
high increase: viral ltoxin-inducedacute hepatitis (5 mm = florn?|,6-7 mm = equivocal;
(a) aspartateaminotransferase (AST;formerlyserum 28 mm = dilated
glutamicoxaloacetictransaminaseISGOTI) 0 In patient>60 years of age add 1 mm/decade
Formation; liver,muscle,kidney,pancreas,RBCs 0 Followingcholecystectomy up to 8 mm
(b) alanineaminotransferase (ALT;formerlyserum (b) neonates:<1 mm
glutamicpyruvictransaminase [SGPT]) (c) infantsup to 1 year of age: <2 mm
Formation; primarilyin liver (d) olderchildren:<4 mm
. ratherspecificelevationin liverdisease @ CHD at portahepatis+ CBD in headof pancreas:
D. B ilir ubin 5mm
helpsdifferentiate betweenvariouscausesof jaundice @ rightintrahepatic bileductjust proximalto CHD:
(a) unconjugated / indirectbilirubin= insolublein 2-3 mm I <40h of diameterof accompanyingportal
water vei n
Formation; breakdownof senescentRBCs @ Cysticduct diameter: 1.8 mm
Metabolismitightlyboundto albuminin vessels, averagelengthof 1-2 cm
activelytaken up by liver,cannotbe distalcysticduct posteriorto CBD (in 95/"), anterior
excretedby kidneys to CBD (in 5%)

&
Right Lateral Anterior Spiral Posterior Spiral Proximal Insertion Low Medial Low Lateral
Insertion Insertion Insertion fnsertion Insertion with a
Common Sheath
Anatomic Variants of Cvstic Duct Insertion
 Anatomyof Liver,Bile Ducts,and Pancreas 677

Bile DuctVariants (b) Mediolateral direction:

Prevalence: 2.4T" of autopsies; right lateral
| 3-1 8.5% of operativecholangiograms anteriorspiral
Significance: aberrantducts near cystic duct / posteriorspiral
gallbladderhave the greatestrisk of low lateral(withcommonsheath)
iatrogenicinjuryat cholecystectomy low medial(at I nearampullaof Vater)
Cx: (1) postoperativebile leak if severed (c) Insertionintointrahepatic bileduct
(2) segmentalbiliaryobstructionif ligated righthepaticduct (0.3%)
left hepaticduct (rare)
A . A B E RRAN TIN T R AH EP AT IC DUCT (d) absenceof cysticduct
may join CHD, CBD,cysticduct,righthepaticduct, r/ gallbladder drainsdirectlyintocommonbileduct
gallbladder
- majorrightsegmentalbileductjoinsextrahepatic
GA LLB LA D D E R
bile duct at / near cysticduct insertion(4-5%)
- cysticohepaticduct (1-2h) = doomalousright Size& Capacity& WallThickness
hepaticduct insertsinto cysticduct Length:
- anomalousleft hepaticducts: not susceptibleto (a) infant< 1 year old: 1.5-3 cm in length
injury+ thereforeof no clinicalsignificance (b) olderchild: 3-7 cm in tength
B. CYSTICDUCTENTERINGRIGHTHEPATICDUCT (c) adult: 7-10 cm in length;2-3.S cm in width
C. DUCT SO F L U S C H KA Capacity: 30-50 mL
= sflldllductsfrom hepaticbed drainingdirectlyinto Wall thickness: 2-3 mm
gallbladder Bile volume: 250-1,000 mL/day secretedby
D. DUP LI CA T ION OF C YS T ICD U C T/ C B D hepatocytes
+ duplication of gallbladder GB function: concentrationof bile throughabsorption
E. CONGENITALTRACHEOBILIARY FISTULA of 90% of water
= fistulouscommunication betweencarinaand left
hepaticduct Congenital Gallbladder Anomalies
. infantswith respiratorydistress Agenesis of Gallbladder
. productivecoughwith bilioussputum lncidence; 0.04-0.07 % (autopsy)
r/ pneumobilia Associated with:
common: rectovaginalfistula,imperforateanus,
hypoplasiaof scapula+ radius,intracardiac shunt
Variantsof CysticDuctInsertion rare: absenceof corpuscallosum,microcephaly,
Prevalence: variationsoccur in 1B-23/" atresiaof externalauditorycanal,tricuspid
(a) Craniocaudad direction: atresia,TE fistula,dextropositionof pancreas
proximalthird = commonhepaticduct high in + esophagus,absentspleen,highpositionof
porta hepatis cecum,polycystickidney
middlethirdof extrahepatic bileduct in 75%
distalthird of extrahepaticbile duct in 10% Hypoplastic Gallbladder
r/ cysticduct parallelsextrahepaticbile duct (a) congenital
(impliescommonfibroussheath) (b) associatedwith cysticfibrosis
Cx: duringcholecystectomy
(1) commonhepaticduct stricture Septationsof Gallbladder
(2) inadvertentligation/ transectionof A . LON GITU D IN ASLE P TA
extrahepaticbile duct 1. Duplication of gallbladder
(3) long cysticduct remnant = two separatelumens+ two cysticducts
lncidence: 1:3,000-1:12,000
2. Bifidgallbladder = doublegallbladder
= two separatelumenswith one cysticduct
2I-22Vo
3. Triplegallbladder(extremelyrare)
B. TRANSVERSESEPTA
1. lsolatedtransverseseptum
2. PHRYGIANCAP (2-6% of poputation)
= kinking/ foldingof fundus+ septum
3. Multiseptated gallbladder(rare)
= multiplecystlikecompartmentsconnectedby
Variants
right posterior segmentalduct ;';:;:;: right hepatic duct smallpores
Cx: stasis+ stone formation
right anterior segmental duct ffiffi left hepatic duct
C . GA LLB LA D D E R
D IV E R TIC U LU M
common hepatic duct = persistenceof cystohepaticduct
678 Radiology Review Manual

GallbladderEctopia
Most f reouentlocations:
(1) beneaththe left lobeof the liver> (2) intrahepatic
> (3) retrohepatic
Rare locations:
(4) withinfalciformligament,(5) withininterlobar
fissure,(6) suprahepatic(lodgedbetweensuperior
surfaceof right hepaticlobe + anteriorchest wall),
(7) withinanteriorabdominalwall,(8) transverse Pancreatic Diameters (on TRV image)
mesocolon,(9) retrorenal, (10) nearposteriorspine H = head = 1.0-2.0 cm
+ lVC, (11) intrathoracic
GB (inversionof liver) B = body = 0.4-1.0 cm
Associatedwith: eventrationof diaphragm T = tail = 0.8-2.2 cm
"FloatingGB"
= gallbladderwith looseperitonealreflections,may
herniatethroughforamenof Winslowintolesser { receives20-35 tributaries/ side branchesthat
sac enterat rightangles
"TorquedGB" { usuallydrainsthroughmajorpapilla
= resUltsin hydrops 0 Majordrainageroutein g1% of individuals
D. ACCESSORYPANCREATICDUCTOF SANTORINI
= proximalportionof dorsalduct,which has not
PANCREAS atrophied
Size 0 Presentin 44'/. of individuals
pancreatichead: 1.0-2.2cm E. AMPULLAOF VATER
pancreaticbody: 0.4-1.0cm = space within medialwall of secondportionof
pancreatictail: 0.8-1.8cm duodenumbelowsurfaceof papillaof Vater
F. MAJORDUODENALPAPTLLA = papillaof Vater
0 Drainageof commonbileduct in 100%
PancreaticDevelopment& Anatomy 0 Drainageof mainpancreaticductof Wirsungin 90%
duringthe 4th week of gestation2 endodermal G. MINORDUODENALPAPILLA(presentin 60%)
diverticulaform in the foregutnear its junctionwith the 0 Drainageof accessorypancreaticduct of Santorini
yolk sac 0 Drainageof mainpancreaticduct in 10%
- dorsaldiverticulumforms dorsalpancreas r/ locateda few cm orad to papillaof Vater
- ventraldiverticulum formsliver,gallbladder, bile
ducts,ventralpancreas JunctionVariants
Pancreaticobiliary
A. Angle betweenCBD + pancreaticduct:
A. DORSALANLAGE(in mesoduodenum) (a) usuallyacute at 5o-30'
Origin: arisesfrom dorsalwall of duodenum+ is (b) occasionallyabnormalat up to 90'
laterdisplacedto the left B. Sphincterof Oddi = sphincterof hepaticopancreatic
0 Formscranialportionof head + isthmus+ body ampul l a
+ tail of pancreas = ffiuscl€fibersencirclingthe CBD + pancreaticduct
- proneto atrophy(poor in polypeptides) at choledochoduodenal junction
r/ drainsto the minor papillathroughaccessoryduct
of Santorini
B. VENTRALANLAGE(belowprimordialliverbud)
Origin: ventralbud arisesfrom ventralwall of
duodenumand is composedof right + left
lobes(the left ventralbud regresses
completely),rotatesdorsallyand inferiorly accessorypancfeatlc dorsal anlage
+ then to the left of the duodenum+ fuses duct (Santorini)
with dorsalanlageduring6-7th week GA
0 Formscaudalportionof the pancreatichead mlnor
duodenal
+ uncinateprocess+ CBD
- not proneto atrophy(richin polypeptides) papilla
miun pancreatrc
r/ ventralduct drainswith CBD throughampullaof major duct (Wirsung)
Vater + becomesthe majordrainagepathwayfor duodenal
the entirepancreasafterfusionwith dorsalduct papilla
C. M A I NP A N C R EA T IC D U C TOF WIR SU N G
distalportionof dorsalduct connectswith ventral ampulla venffal anlage
duct; proximalportionof dorsalduct may disappear
r/ measures1-2-3 mm in diameter Embryologic Development of Pancreas
 Anatomyof Liver,Bile Ducts,and Pancreas 679

4::;:.::i S P LE E N
'7@t
Size of Spleen
in adults: 12 cm length,7-8 cm anteroposterior
diameter,3-4 cm thick;splenicindex
(LxWxH) of <480
in children: logarithmic increasein lengthwith
increasingage; formulafor length= 8.7
+ 0.31 x age (in years)
in infants(0-3 monthsof age): <6.0cm in length

Short Common No Common Long Common Weight of Spleen

Channel Channel Channel at birth: 1 5g
i n adul ts: 150 (100-265)g
Normal Union between CBD & Pancreatic Duct estimatedweight= splenicindexx 0.55

(a) choledochal sphincter(Boyden)= encirclesdistal Embryologyof Spleen

CBD
(b) pancreaticduct sphincter(in 33% separate)
(c) ampullarysphincter
C. Types of unionbetweenCBD + pancreaticduct:
1. Nor m al j u n c ti o=n u n i o ni n s i d ed u o d e n alw al l
(a) 2-10 (mean5) mm shortcommonchannel
(55-85%)with a diameterof 3-5 mm
(b) separateentrancesinto duodenum(42%)
(c) 8-15 mm longcommonchannel
2. Anomalousjunction= unionoutsideduodenal
wall beyondthe influenceof the sphincterof
Boyden(1.5-3.2%)
(a) pancreaticduct insertingintoCBD >15 mm
from entranceintoduodenum
(b) CBD insertinginto pancreaticduct
- spleenarisesfrom mesenchymalcells betweenlayers
of dorsalmesogastriumduringSthweek GA
- splenicprimordiumdifferentiates to form capsule,
connectivetissueframework,splenicparenchyma
- majorsite of hematopoiesisuntil28 weeks GA;
retainscapacityfor extramedullaryhematopoiesiswell
intoadultlife
^/ spleenrecognizableby 12th week GA (as fusionof
, mesenchymalaggregatesoccurs)
ri splenicclefts/ notches/ lobulesmay persist
r/ accessoryspleen(in up to 30% by auiopsy)
Histologyof Spleen
(a) RED PULP= flUrTlefous
vascularsinuses
(b) WHITEPULP= lymphoidfotlictes+ cellsof RES

--t--..--

DescendingPancreatic Duct Vertical Pancreatic Duct Sigmoid Pancreatic Duct Looped Pancreatic Duct
50Vo

Anomalous Union of PersistentDuct of santorini PersistentDuct of santorini Pancreas Di visum (7Vo)

Pancreatic Duct and Bile ductof Wirsungasgdor (44Vo)
Duct drainage rotte in91Vo duct of Santorini as ma.jor
drainage route ing%i

Variations in Pancreatic Duct Anatomy

680 Radiology Review Manual

Development: ratio of white to red pulp increaseswith (b) adult+ olderchild:

age + progressiveantigenicstimulation r/ tzWt-intensity:spleen> liver
./ tt Wl-intensity:liver> spleen> muscle

lmagingCharacteristicsof Spleen IRON METABOLISM

A. CTATTENUATION Totalbody iron: 5 g
(a) withoutenhancement: (a) functionaliron: 4 g
40-60 HU; 5-10 HU lessthan liver Location: hemoglobinof RBCs,myoglobinof
(b) with enhancement: muscle,variousenzymes
normalheterogeneous enhancement duringfirst (b) storediron: 1 g
minuteafter bolus injection(due to differentblood Location: hepatocytes,reticuloendothelial cells of
flow ratesthroughthe cords of the red + white liver(Kupffercells)+ spleen+ bone
pulp) marrow
{ arciform(alternatingbandsof high + low Absorption'. 1-Z mg/daythroughgut
attenuation)/ focal/ diffuseheterogeneity Transport: boundtotransferrin intravascularly
r/ heterogeneityresolvedin portalvenousphase Deposition:
(a) transferrin-transfer to :
B . M R S I G N A LIN T E N S IT Y hepatocytes,RBC precursorsin erythron,
directlyrelatedto ratioof whiteto red pulp parenchymal tissues(eg,muscle)
(a) neonate<8 monthsof age: (b) phagocytosisbY:
r/ f t Wl- and T2Wl-intensity:spleen< liver(due reticuloendothelial cells phagocytizesenescent
to predominanceof red PulP) erythrocytes(= extravascularhemolysis);RBC iron
DDx: hemochromatosis storedas ferritin/ releasedand boundto transferrin

anterior pararenal duodenum pancreas

space
peritoneum descending
g v 9 v
colon
9 g v $

lateroconal
fascia

perirenal space posterior renal fascia

(Gerota)

posterior pararenalspace transversalisfascia

Extraperitoneal Spaces
 Disordersof Liver,BiliaryTract,Pancreas,and Spleen 681
DISORDERS
OF LIVER,BILIARYTRACT,PANCREAS,
ANDSPLEEN
ACCESSORY
SPLEEN
= failureof coalescenceof severalsmalt mesodermal
buds in the dorsalmesogastrium that comprisethe
spleen
lncidence; 10-30% of population;multiple(up to 6) in
10%
0 Undergoeshypertrophyafter splenectomyand is
responsiblefor recurrenceof hematologicdisorders
(idiopathic thrombocytopenic purpura, hereditary
spherocytosis, acquiredautoimmunehemolyticanemia,
hypersplenism)
Location:
(a) nearsplenichilumalongthe courseof splenic
vessels(mostcommon)
(b) withinlayersof omentum(gastrosplenic ligament,
othersuspensoryligamentsof spleen)
(c) anywherein abdomen(eg, pancreas,pelvis)
(d) attachedto left ovary/ testis=splenogonadal
fusion (dueto closerelationshipbetweendeveloping
spleen+ mesonephros+ left gonadalanlage)
NUC (Tc-99msulfurcolloidscan/ spleen-specific Tc-ggm
denaturedRBCs):
! usually<1 cm in diameter
{ <10% identifiedwhen normalspleenpresent
Cx: diseaserecurrencedue to hypertrophyof accessory
spleenaftersplenectomyfor hypersplenism
AMPULLARY
TUMOR
= benign/ malignanttumorsarisingfrom glandular
epitheliumof ampullaof Vater
Age: 6th + 7th decade; M:F = 2:1
Path: averagediameterof <3 cm
Hista: (a)dysplasticepitheliumin glandular/ villous
structuresof tubular/ villousadenoma
(b) carcinomain situ
(c) invasivecarcinomaoftenwith desmoplastic
reaction
Associatedwith: familialadenomatouspolyposis
syndromes(eg,familialpolyposiscoli,
Gardnersyndrome)[100-200-fotdrisk],
coloncarcinoma
. malaise,epigastricpain,weightloss
. intestinalbleeding(tumorulceration)
. intermittentjaundice(ductalobstruction)
. gray "aluminum/ silver-colored" stools(3%)
. chills,fever,RUQ pain (ascendingcholangitis) in up to
20%
. endoscopy:tumor extendingthroughorifice(69%),
prominentpapilla/ submucosalmass (25/"), not
visualized(9%)
TNM staging:
T1 tumorconfinedto ampulla
T2 : tumorextending
intoduodenal
wall
T3 : invasionof pancreas<2 cm deep
T4 : invasionof pancreas>2 cm deep
lnternational Union Against Cancer Staging:
| - tumorconfinedto ampulla
ll = tumorextensionintoduodenalwall/ pancreas
lll = regionallymphnode involvement (Lnnstations
aroundhead + body of pancreas,anterior
+ posteriorpancreaticoduodenal, pyloric,
commonbileduct,proximalmesenteric)
lV = invasionof pancreas>2 cm deep
r/ tumorofteninapparentdue to smallsize
UGI:
r/ indentationof duodenallumenat papillaof Vaterwith
fi l l i ngdefect> 1.5cm
./ surfaceirregularity+ deep barium-filledcrevicesin
vi l l oustumor
Biliaryimaging:
r/ dilatationof most distalsegmentof commonbile duct
r/ stenosis(circumferential tumor growtharound
ampulla/ desmoplasticreaction)
r/ irregularpredominantlypolypoidfillingdefect
{ + pancreaticdilatation= double-ductsign (may be
absentif tumor small/ accessorypancreaticduct
decompressespancreaticsystem/ main pancreatic
duct drainsintominorpapilla)
EndoscopicUS (mostsensitivetechnique):
87% stagingaccuracy
Rx: Whippleprocedure(=pancreaticoduodenectomy)
Prognasis: 28-70"/" S-yearsurvivalfor ampullary
carcinomas(dependingon stage)
DDx:
1. Periampullary duodenaladenoma/ adenocarcinoma
(usuallylargerlesionwith significantintraduodenal
extension)
2. Choledochocele (cysticlesionfillingwith biliary
contrast)
3. Brunnerglandtumor,pancreaticrest ("myoepithelial
hamartoma"),leiomyoma,carcinoid(oftenproduce
somatostatin)
4. Duodenitis,pancreatitis
5. Stoneimpactionin ampulla
ANNULARPANCREAS
= secofldmostcommoncongenitalanomalywhereina
ringof normalpancreatictissueencirclesthe duodenum
secondaryto abnormalmigrationof ventralpancreas
(head+ uncinate)
lncidence: 1:20,000autopsies
Age at discovery: childhood(SZ%);adutthood(48%)
Associatedwith: othercongenitalanomalies(in 75%):
esophagealatresia,TE fistula,duodenalatresia/
. stenosis,duodenaldiaphragm,imperforate anus,
malrotation,Down syndrome
Location: 2nd portionof duodenum(85%);
1st/ 3rd portionof duodenum(15%)
. mostlyasymptomaticwith incidentaldiscovery
. neonate : persistentvomiting(duodenalobstruction)
682 Radiology Review Manual

. adult : nausea,vomiting(60%),abdominalpain BANTISYNDROME

(70%),hematemesis (10%),jaundice(50%) = NONCIRRHOTIC IDIOPATHIC PORTALHYPERTENSION
r/ polyhydramnios (in utero) = NONCIRRHOTIC PORTALFIBROSIS= HEPATOPORTAL
rl "doublebubble"= dilatedduodenalbulb + stomach SCLEROSIS
= Syndrome characterized by
r/ proximalduodenaldilatation
(1) splenomegalY
r/ enlargementof pancreatichead
(2) hypersplenism
UGI:
(3) portalhypertension
{ eccentricnarrowingwith lateralnotching+ medial
retractionof 2nd Partof duodenum Etiotogy: increasedportalvascularresistancepossibly
duodenum due to portalfibrosis+ obliterativevenopathy
r/ concentricnarrowingof mid-descending
of intrahepaticportalbranches
r/ reverseperistalsis,pyloricincompetency
CT: Histo: slightportalfibrosis,dilatationof sinusoids,intimal
r/ pancreatictissuesurrounding descendingduodenum thickeningwith eccentricsclerosisof peripheral
portalvein walls
ERCP (mostspecific)/ MR pancreatography:
Age: middle-aged women;rarein America+ Europebut
r/ normallylocatedmain duct in pancreaticbody + tail
common i n Indi a+ JaP an
r/ smallduct originating on anteriorleft+ passing
. elevatedportalvein pressure(withoutcirrhosis,
posteriorlyaroundduodenumcommunicateswith
parasites,venousocclusion)
mainduct (in 85%)
. normalliverfunctiontests
Cx: increasedincidenceof . cytopenia(dueto hypersplenism)
(1) periampullary pepticulcers
. nofrTr?l I slightlyelevatedhepaticvenouswedge
(2) pancreatitis(15-20%) usuallyconfinedto
pancreatichead and annulus pressure
Rx: gastrojejunostomy/duodenojejunostomy { esophagealvarices
./ patent hepaticveins
ri patentextrahepaticportalvein + multiplecollaterals
ASCARIASIS
Prognosis: 90/" S-yearsurvival;55% 3O-yearsurvival
Mostfrequenthelminthicinfectionin humans
Organism; Ascarislumbricoides,25-35cm long as
adultworm;lifesPanof 1 Year BILIARY CYSTADENOCARCINOMA
= BILEDUCTCYSTADENOCARCINOMA
Country: 644 millionhumansharborthe roundworm;
= tdte malignantmultilocular from
cystictumororiginating
70-90% in America;in UnitedStates
endemicin: Appalachianrange,southern biliarycystadenoma
+ Gulf coast states Histo: (a) with ovarianstroma(goodprognosis),in
femalesonlY
Prevalence: 25/"of world populationinfected
(b) withoutovarianstroma(bad prognosis)
(a) in UnitedStates: 12/"in blacks,1% in whites
. hemorrhagic internalfluid
(b) in partsof Africa,Asia, SouthAmerica: 90%
Cycle: i nodularitywith septationsare suggestiveof malignancy
ingestionof contaminatedwater/ soil / vegetable;larvae { coarsecalcifications
DDx: no imagedifferentiation from biliarycystadenoma
penetrateintestinalwall; migrateinto mesenteric
lymphatics+ veinsintoliver;reachlungvia rightheart
+ pulmonaryartery;maturein pulmonarycapillarybed BILIARY CYSTADENOMA
to 2-3 mm length;burrowintoalveoli;ascendin = BILEDUCTCYSTADENOMA
respiratorytract;are swallowedand again reachsmall = tartabenignpremalignant multilocularcystictumor
intestine,where they becomeadultworms whose eggs originatingin bileducts;probablyderivingfrom ectopic
leave the body by the fecal route nestsof PrimitivebiliarYtissue
. abnormalliverfunctiontests+ biliarycolic lncidence: 4.6/" of all intrahepaticcystsof bile duct origin
. hypereosinophilia only presentduringacutestageof Age: >30 years(82/"), peak incidencein Sthdecade;
larvalmigration M:F = 1:4; predomi nantlCYaucasi an
^/ bariumstudy Path: multilocular cystictumorcontainingproteinaceous
! cholangiography (49%) fluidwith well-defined thickcapsule
US: Histo: singlelayerof cuboidal/ tall columnarbiliary-
r/ tubularechogenicfillingdefectwith 2-4-mm wide type epitheliumwith papillaryprojections,
subepithelial stromaresembling that of the ovary
centralsonolucentline (= worm with digestivetract)
withindilatedcommonbile duct 0 Similarto mucinouscystictumorsof pancreas
Cx: (1) lntestinalobstruction + ovary
(2) Intermittent with acute
biliaryobstruction Location: intrahepatic:extrahepatic bileducts= 85:15;
cholangitis, pancreatitis
cholecystitis, right lobe (48%);left lobe (20-35%);both
(3) Liver abscess(rare) lobes(15-30%);gallbladder(rare)
. chronicabdominalPain
(4) Granulomatousstrictureof extrahepaticbile
. dyspepsia,anorexia,nausea+ vomiting
ducts (rare)
. jaundice
Rx: Mebendazole

. abdominalswellingwith palpablemass (90%)
{ massof 1.5-35cm in size
{ up to 11 litersof clear/ cloudy,serous/ mucinous/
gelatinous,purulent/ hemorrhagiclbiliousfluid
containinghemosiderin/ cholesterol/ necrosis
r/ papillaryexcrescences+ mural nodules
! septationsbetweencysts
US:
r/ ovoid multiloculatedanechoicmass with highly
echogenicseptations/ papillarygrowths
r/ may containfluid-fluidlevels
CT:
{ multiloculatedmass of near water density
{ contrastenhancementin wall + internalsepta
MB:
{ loculeswith variablesignalintensityon Tl Wl + T2Wl
dependingon theirproteincontent
A ngio:
r/ avascularmass with smallclustersof peripheral
abnormalvessels
{ stretching+ displacementof vessels
{ thin subtleblushof neovascularity in septa + wall
Cx: (1) malignanttransformationinto cystadeno-
carcinoma(indicatedby invasionof capsule)
(2) ruptureinto peritoneum/ retroperitoneum
Rx: surgicalresection(recurrencecommon)
DDx: liverabscess,echinococcalcyst, cystic
mesenchymal hamartoma(children+ young
adults),undifferentiatedsarcoma(children+ young
adults),necrotichepaticmetastasis,cysticprimary
hepatocellularcarcinoma
BI LIARY-ENTERIC FISTULA
lncidence: 5/" at cholecystectomy;0.5% at autopsy
Etiology: cholelithiasis(90%),acute/ chronic
cholecystitis,biliarytract carcinoma,regional
invasiveneoplasm,diverticulitis, inflammatory
boweldisease,pepticulcerdisease,
echinococcalcyst,trauma,congenital
communication
Communication with:
duodenum(70%),colon(26/"),stomach(4%),jejunum,
ileum,hepaticartery,portalvein (causeddeath of
lgnatiusLoyola),bronchialtree,pericardium, renal
pelvis,ureter,urinarybladder,vagina,ovary
A. CHOLECYSTODUODENAL FISTULA(51-80%)
1. Perforatedgallstone(90%):
associatedwith gallstoneileusin 20/"
2. Perforatedduodenalulcer (10%)
3. Surgicalanastomosis
4. Gallbladder carcinoma
B. CHOLECYSTOCOLTC FTSTULA (13_21%)
c . oHoLE DO C H OD U OD EN AL F T ST U L (1
A 3 -1 e % )
due to perforatedduodenalulcerdisease
D. MULTTPLEFTSTULAE(7%)
{ pneumobilia = branchingtubularradiolucencies, more
prominentcentrallywithinthe liver
r/ bariumfillingof biliarytree
r/ shrunkengallbladdermimickingpseudodiverticulum
duodenalbulb
Disordersof Liver,BiliaryTract,Pancreas,and Spleen 689
! multiplehyperechoicfoci with dirty shadowing
DDx: patuloussphincterof Oddi, ascendingcholangitis,
surgery(choledochoduodenostomy,
cholecystojejunostomy,sphincterotomy)
BUDD.CHIARI
SYNDROME
= syndromeof global/ segmentalhepaticvenousoutflow
obstruction
Cause:
A. tDtoPATHtC(66%)
B . TH R OMB OS IS
(a) hypercoagulable state: polycythemiarubravera
(1/3),oral contraceptives, pregnancy
+ postpartumstate,paroxysmalnocturnal
hemoglobulinuria (12%),sicklecell disease
mnemonic.' "5 P's"
Paroxysmalnocturnalhemoglobulinuria
Platelets(thrombocytosis)
Pill (birthcontrolpills)
Pregnancy
Polycythemiarubravera
(b) injuryto vesselwall: phlebitis,trauma,hepatic
radiationinjury,chemotherapeutic
+ immunosuppressive drugsin patientswith bone
marrowtransplants,venoocclusivediseasefrom
pyrrolizidine alkaloids(senecio)foundin
medicinalbushteas in Jamaica
C. NONTHROMBOTIC OBSTRUCTION
(a) Tumor growthinto IVC / hepaticveins (renalcell
carcinoma,hepatoma,adrenalcarcinoma,
metastasis,primaryleiomyosarcoma of IVC)
(b) Membranousobstructionof suprahepaticIVC
= IVC diaphragm(believedto be a congenital
web or an acquiredlesionfrom long-standing
IVC thrombosis);common in Oriental+ lndian
population(SouthAfrica,India,Japan,Korea);
very rare in Westerncountries
(c) Rightatrialtumor
(d) Constrictivepericarditis
(e) Rightheartfailure
Pathophysiology: hepaticvenous thrombosisleads to
elevationof sinusoidalpressure,
which causesdelayed/ reversed
portalvenous inflow,ascites,
alterationin hepaticmorphology
Age: all ages; M < F
. right upperquadrantpain
. shortnessof breath(dueto decreasedcardiacreturn)
. nonspecificelevatedtransaminases, jaundice
. lower-extremity edema
Location:
Type | : occlusionof IVC + hepaticveins
Type ll : occlusionof major hepaticveinst IVC
Type lll : occlusionof smallcentrilobar veins
r/ hepatosplenomegaly (earlysign)
r/ caudatelobe hypertrophy(88%) [DDx: cirrhosis]
r/ ascites
r/ gallbladder wall thickening>6 mm
of { nonvisualization of hepaticveins (75%)/ vein diameter
<3 mm (measured2 cm from IVC)
684 RadiologyReviewManual
{ communications betweenright/ middlehepaticvein and
inferiorrighthepaticvein
{ enlargedinferiorrighthepaticvein (18%)
r/ portalvein diameter>12 mm (in adults),>8 mm (in
children)
r/ visualizationof collateralpathways:
(a) portosystemic:paraumbilicalvein
(b) bypassingIVC: azygos,hemiazygos
r/ + narrowing/ obstructionof intrahepaticIVC
NE CT :
r/ globalliverenlargement+ diffusehypoattenuation
CT:
^/ "ttip+top"enhancementpattern:
^/ prominentenhancement of centralliver+ weak
enhancementof peripheralliveron earlyimages
./ enhancementof liverperiphery+ wash-outof
contrastfrom centralliveron delayedimages
ri normalenhancementof enlargedcaudatelobe (due
to separatevenousdrainagedirectlyinto IVC)
r/ mottledliverenhancementpattern(due to hepatic
congestion):
{ patchyliverenhancement(85%)with normalportal
bloodflow
r/ hypodensityin atrophicareas/ periphery(82%)
with inversionof portalbloodflow (= reversedportal
venousbloodflow due to increasedpostsinusoidal
pressureproducedby hepaticvenousobstruction/
rarelyinfarcts)
r/ failureto identifyhepaticveins
r/ hepaticvein thrombi(18-53%)
MRI:
r/ reductionin caliber/ completeabsenceof hepaticveins
{ "commasign"= multiplecomma-shaped intrahepatic
flow voids (due to intrahepaticcollaterals)
DopplerUS (85-100%sensitive,85% specific):
r/ one / more majorhepaticveins reducedin size to
<3 mm / filledwith thrombus/ not visualized
{ communicating venouscollaterals
intrahepatic
^/ decreased/ absent/ reversedblood flow in hepatic
veins
^/ ttat flow / loss of cardiacmodulationin hepaticveins
r/ demodulatedportalvenousflow = disappearanceof
portalvein velocityvariationswith breathing
./ slow flow (<11 cm/sec)/ hepatofugalflow in portal
vein
{ portalvein congestionindex>0.1
r/ portalvein thrombosis(20%)
{ compressionof IVC by enlargedliver/ caudatelobe
{ sluggish/ reversed/ absentbloodflow within IVC
{ hepaticarteryresistiveindex>0.75
NUC (Tc-99msulfurcolloid):
{ centralregionof normalactivity(hot caudatelobe)
surroundedby greatlydiminishedactivity(venous
drainageof hypertrophiedcaudatelobe into IVC by
separatevein)
r/ colloidshiftto spleen+ bone marrow
r/ wedge-shapedfocal peripheraldefects
Angio(inferiorvenocavography, hepaticvenography):
{ absenceof main hepaticveins
r/ spiderweb patternof collateral+ recanalizedveins
r/ nign-pressuregradientbetweeninfra-and
suprahepaticportionof IVC (due to enlargedliver)
^/ stretching+ drapingof intrahepaticarterieswith
hepatomegaly
./ inhomogeneous prolongedintensehepatogramwith
fine mottling
./ largelakesof sinusoidalcontrastaccumulation
Portography:
r/ centralhepaticenhancement(normalhepatopetal
flow)
! reversedportalflow in liverperiphery(suppliedonly
by hepaticartery)
\i bidirectional/ hepatofugalmain portalvein flow
Syndrome(1/3)
AcuteBudd-Chiari
0 Caudatelobe has not had time to hypertrophy!
. rapidonsetof abdominalpain (livercongestion)
. insidiousonset of intractableascites
r/ hepatomegaly withoutderangement of liverfunction
r/ ascites(97%)
CT:
./ diffusehypodensity on NECT
./ early enhancementof caudatelobe + central
portionaround IVC with decreasedenhancement
peripherally
hypodenseluminaof hepaticveinson CECT
decreasedattenuationof enhancingareaswith
patchyinhomogeneous enhancementin liver
peripheryon delayedscans
MR:
r/ peripheralliverparenchymaof moderatelylow
signalintensityon Tl Wl + moderatelyhighsignal
intensityon T2Wl comparedwith centralportion
^/ diminished+ mottledperipheralenhancement
Syndrome(2i3)
ChronicBudd-Chiari
. insidiousonsetof jaundice,intractable ascites
. portalhypertension, varicealbleeding
r/ enlargementof centralregion(= caudatelobe
+ adjacentcentralpart of right lobe + medialsegment
of left lobe
./ nonsegmentali lobaratrophyof affectedliver (due to
extensivefibrosis)with diminishedattenuation before
+ after contrastadministration
r/ progressivepatchyenhancementradiatingoutward
from majorportalvessels(on dynamicbolusCT)
r/ "reticulatedmosaic"enhancement= diffusepatchy
lobularenhancement separatedby irregularlinear
areasof low densityin centralarea
^/ delayedhomogeneous enhancement of entireliver
after severalminutes
r/ ascites
ColorDoppler:
^/ "bicolored"hepaticveins (due to intrahepatic
collateralpathways)are PATHOGNOMONIC
MR:
{ absenceof flow within hepaticveins
r/ minimaldifferencesin signalintensitybetween
centraland peripheralportionsof liver
r/ intrahepaticcollateralvessels
 Disorders of Liver, Biliary Tract, Pancreas, and Spteen 685

Dx: liverbiopsy r/ portatradiclescompletelysurroundedby dilatedbile

Rx: controlof asciteswith diuretics+ sodiumrestriction; ducts= c€ntraldot signon CT
anticoagulation,thrombolytictherapy,surgery/ r/ sludge lcalculi in dilatedducts
balloondilatation(dependingon etiology); Cholangiog raphy(diagnostic):
transjugularportosystemic shunt;orthotopicliver { segmentalsaccular/ beadedappearanceof
transplantation(for advancedcases) intrahepaticbile ducts extendingto peripheryof liver
r/ bridgeformationacrossdilatedlumina
r/ intraluminal bulbarprotrusions
CANDIDIASIS OF LIVER ^/ frequentectasiaof extrahepaticducts + CBD
= almostexclusively seen in immunocompromised Cx: (1) Bilestasiswith recurrentcholangitis
patients(acuteleukemia,chronicgranulomatous (2) Biliarycalculi
diseaseof childhood,renaltransplant, chemotherapy for (3) Liverabscess
myeloproliferative disorders) (4) Septicemia
Prevalence; at time of autopsyin S0-70/o of acute (5) Increasedriskfor cholangiocarcinoma
leukemia,in 50% of lymphomapatients
0 Mostcommonsystemicfungalinfection CHOLANGIOCARCINOMA
in immunocompromised patients! lncidence; 0.5-1"/"of all cancers,30% of hepatic
. abdominalpain primarymalignancies
. persistentfever in neutropenicpatientwhose leukocyte 0 Cholangiocarcinomas occur in 10:1 Sy" of patientswith
countis returningto normal primarysclerosingcholangitis!
. elevatedalkalinephosphatase Location:
./ hepatomegaly A . IN TR A H E P A TIC
r/ "target"/ "bull's-eye"sign = multiplesmallhypoechoic/ 1. PERIPHERALdistalto 2nd-orderbranches
hypoattenuating masseswith centersof increased 2. HILAR/ CENTRALat bifurcation i in 1st-order
echogenicity/ attenuationdistributedthroughoutliver branches= Klatskintumor
0 Bull's-eyelesionbecomesvisibleonlywhen confluenceof hepaticducts in 10-26%
neutropenia resolves! left / right hepaticduct in g-ig%
r/ hyperintense lesionson T2Wl B. EXTRAHEPATIC
NUC: commonhepaticduct in 14-97%
{ uniformuptake/ focal photopenicareas proximalCBD in 15-30%
r/ diminishedGa-67uptake distalCBD in 30-50%
Dx: biopsyevidenceof yeast/ pseudohyphaein central cysticduct in 6%
necroticportionof lesion Path:
DDx: metastases, lymphoma,leukemia,sarcoidosis, (a) exophytic (= mass-forming/ nodular)type:
septicemboli,otherinfections(MAl,CMV),Kaposi commonlyin peripheralcholangiocarcinoma
sarcoma ri largeirregularhypoattenuating mass
r/ stippled/ punctatehyperattenuating foci
./ ttrin rimlike/ thick bandlikeenhancementaround
CAROLI DISEASE the tumor(early)
= COMMUNICATING CAVERNOUS ECTASIA OF r/ progressiveconcentricfillingin of contrast(late)
INTRAHEPATIC DUCTS due to slow diffusioninto interstitialtumor spaces
= r?f€ congenitalprobablyautosomalrecessivedisorder (b) diffuse= infiltrative(periductal) type:
characterizedby segmentalsaccularcysticdilatationof commonlyin hilar+ extrahepatic cholangiocarcinoma
major intrahepaticbile ducts { muralthickening/ encirclingmassof bile ductwall
Etiology: (a) ? perinatalhepaticarteryocclusion r/ focal or diffusestricture/ completeobstructionof
(b) ? hypoplasia / aplasiaof fibromuscular
wall bileducts
components (c) polypo;6 = papillary(intraductal)type: infrequent
Age: childhood+ 2nd-3rddecade,occasionally in r/ intraluminal polypoidmass
i n f a n c y ;M : F= 1 : 1 (d) combination
Associated with: Histo: well/ moderately/ poorlydifferentiated ductal
benignrenaltubularectasia,medullaryspongekidney (mostcommon),papillary,mucinous,signet-ring
(in 80%),infantilepolycystickidneydisease, cell,mucoepidermoid, adenosquamous,
choledochalcyst (rare),congenitalhepaticfibrosis cystadenocarcinoma
. recurrentcramplikeupperabdominalpain Unusualmanifestation :
. fever,transientjaundice 1. Mucin-hypersecreting cholangiocarcinoma
., cirrhosis/ portalhypertension(very rare) ri severediffusedilatationof intra-and
r/ multiplecysticstructuresconvergingtoward porta extrahepaticbile ducts proximal+ distalto tumor
hepatisas eitherlocalizedi diffuselyscatteredcysts 2. Squamouscellcarcinoma
communicating with bileducts(DDx: polycysticliver = metaplastictransformationof adenocarcinoma
disease) cells
686 Radiology Review Manual

Predisposed: CT:
(1) Inflammatory boweldisease(10 x increasedrisk); r/ singlepredominantly homogeneous round/ oval
incidenceof 0.4-1.4/"in ulcerativecolitis:latent hypodensemass with irregularborders
periodof 15 years;tumorsusuallymulticentric r/ "peripheralwashoutsign"= early minimal/
+ predominantlyin extrahepaticsites;GB involved moderaterim enhancementwith progressive
in 15% (simultaneous presenceof gallstonesis concentricfillingand clearingof contrastmaterialin
rare) rim of lesionon delayedimages
(20-50%),
(2) Biliarylithiasis:cholecystolithiasis { markedhomogeneousdelayedenhancement(74%)
intrahepaticlithiasis(5-10%) MR:
(3) Primarysclerosingcholangitis(10%) { largecentralheterogeneoushypointensemass on
(4) Clonorchissinensisinfestation (Far East);most TlWI
commoncauseworldwide { hyperintenseperiphery(viabletumor)+ large
(5) Choledochalcyst / congenitalhepaticcyst / centralhypointensity(fibrosis)on T2Wl
congenitalbiliaryatresia { gadoliniumenhancement of lesion
(6) Ductalplatemalformation: Angiography:
Biliaryhamartoma { avascular/ hypo-/ hypervascularmass
Autosomaldominantpolycysticdisease { stretched/ encasedarteries(frequent)
Congenitalhepaticfibrosis ^/ neovascularity in 50%
Carolidisease(dueto chronicbiliarystasis) r/ lack of venousinvasion
(7) Papillomatosis of bileducts Prognosis: <20/" resectable; 30% 5-year survival
(8) Recurrentpyogeniccholangitis
(9) Choledochoenteric anastomosis KlatskinTumor
(10) Historyof othermalignancy(10%) = INTRAHEPATIC CENTRAL CHOLANGIOCARCINOMA
(11) Thorotrastexposure = tumor at confluenceof hepaticducts (up to 70'/" ot
(12) Alphcx-lantitrypsindeficiency cholangiocarcinomas)
Prognosis: mediansurvivalof 7 months,0-10/o S-year r/ directsignsof Klatskintumor:
survival r/ iso- to hyperechoiccentralporta hepatismass /
focal irregularityof ducts (for infiltrating
Cholangiocarcinoma
Intrahepatic cholangiocarcinoma = IT'lofe
commonsubtype)
= CHOLANGIOCELLULAR CARClNOMA ^/ polypoid/ smoothnodularintraluminalmass (for
lncidence: 113of all malignanciesoriginatingin the papillary+ nodulartypes of cholangiocarcinoma)
liver;8-1 3/" of all cholangiocarcinomas; with associatedmuralthickening
2nd mostcommonprimaryhepatictumor r/ indirectsignsof Klatskintumor:
after hepatoma ^/ segmentaldilatationwith nonunionof rightand
Histo: adenocarcinoma arisingfrom the epitheliumof a left ducts at porta hepatis+ normalcaliberof
smallintrahepatic bileduct with prominent extrahepaticducts
desmoplasticreaction(fibrosis);+ mucinand ^/ pressureeffect/ encasement/ invasion/
calcifications obliterationof portalvein and hepaticartery
Average age: 50-60 years; M > F r/ lobaratrophy(14%)= dilatedcrowdedducts
. abdominalpain (47%) extendingto liversurface+ geographicfatty
. palpablemass (e%) changein one lobe
. weightloss (18%)
. painlessjaundice(12%) IntrahepaticPeripheralCholangiocarcinoma
Spread: (a) localextensionalong duct . no jaundice
(b) localinfiltrationof liversubstance Location: right lobe Predilection
(c) metastaticspreadto regionallymph nodes r/ solitarymass (nodularform)withouthypoechoichalo
( i n1 5 % ) r/ diffuselyabnormallivertexture(infiltrativeform):
r/ mass of 5-20 cm in diameter { tumormore hypoechoicif <3 cm
r/ satellitenodulesin 65% { tumormore hyperechoic if >3 cm
r/ punctate/ chunkycalcificationsin 18% { well-marginated cysticmass (papillarymucin-
r/ calculiin biliarytree producingtumor)+ diffusehyperechoicflecksof
NUC: tumor calcification
r/ cold lesionon sulfurcolloid/ IDA scans ./ dilatationof bileductsperipheralto tumor(31%)
./ segmentalbiliaryobstruction DDx: metastaticadenocarcinoma / leiomyosarcoma;
! may show uptakeon galliumscan sclerosinghepatocellular carcinoma
US:
r/ ditatedbiliarytree
{ predominantlyhomo-/ heterogeneousmass noma
ExtrahepaticCholangiocarci
^/ nyper-(75%)/ iso-/ hypoechoic(14%)mass = BILE DUCTCARCINOMA
r/ muralthickening Age peak; 6th-7th decade, M:F = 3:2
 Disordersof Liver,BiliaryTract,pancreas,and Spteen 697

lncidence; <0.5%of autopsies;gO%of all Angiography:

cholangiocarcinomas; morefrequentin ^/ hypervasculartumor with neovascularity(SO%)
Far East r/ arterioarterial
collateralsalongthe courie of bile
Histo: well-differentiated sclerosingadenocarcinoma ducts associatedwith arterialobstruction
(2/3),anaplasticcarcinoma(11"/"), ./ poor / absenttumorstain
cystadenocarcinoma, adenoacanthoma, r/ displacement / encasement/ occlusionof hepatic
malignantadenoma,squamouscell artery+ portalvein
= €pidermoidcarcinoma,leiomyosarcoma Cx: (1) Obstructionleadingto biliarycirrhosis
. gradualonsetof fluctuatingpainlessjaundice
(2) Hepatomegaty
. c holangit is (10%) (3) Intrahepaticabscess(subdiaphragmatic,
. weightloss,fatigability
perihepatic,septicemia)
. intermittentepigastricpain
(a) Biliaryperitonitis
. elevatedbilirubin+ alkalinephosphatase
(5) Portalvein invasion
. enlargedtenderliver
Dx: endoscopicbrush biopsy(30-95% sensitive)
Growth pattern: Prognosis; mediansurvivalof 5 months;1.6% S-year
(1) Obstructivetype (70-AS%) survival;39% S-yearsurvivalfor
^/ U- I V-shapedobstructionwith nipple,rattail,
carcinomaof papillaof Vater
smoothI irregulartermination DDx: sclerosingcholangitis, AIDS cholangitis,
benign
(2) Stenotictype (10-25%) stricture,chronicpancreatitis,edematouspapilla,
r/ stricturedrigidlumenwith irregularmargins idiopathicinflammation of CBD
+ prestenotic dilatation
(3) Polypoid/ papiltaryrype (5-6%)
r/ intraluminal fillingdefectwith irregularmargins
Spread: (a) lymphaticspread: cystic+ CBD nodes CHOLANGITIS
(>32/"),celiacnodes(>16V.),
AcuteObstructivei AscendingGholangitis
peripancreatic nodes,superiormesenteric = biliaryduct obstructionassociatedwith biliaryinfection
nodes
Cause:
(b) infiftrationof liver (23./")
(a) benigndisease:
(c) peritoneat seeding(9%) (1) stricturefrom priorsurgery(36%)afterbileduct
(d) hematogenous (extremelyrare): liver,
explorationi bilioentericanastomosis,(2) calculi
peritoneum,lung
(30%),(3) sclerosingcholangitis, (4) obstructed
drainagecatheter,(5) parasiticinfestation
UGI:
(b) malignantdisease: ampullarycarcinoma
r/ infiltration / indentation of stomachi duodenum Types:
Cholangiography (PTCor ERC best modalityto depict
A . A C U TEN ON S U P P U R A TIVAES C E N D ING
bileduct neoplasm):
C H OLA N GITIS
r/ exophyticintraductal tumormass (46./"),2-5mm . bile remainsclear
in diameter . patientnontoxic
r/ frequentlylong/ rarelyshort concentricfocal
B . A C U TES U P P U R A TIVAES C E N D IN G
stricturein infiltrating sclerosingcholangitictype oH oLA N GtTtS(14% )
, with wall irregularities Associatedwith: obstructingbiliarystone or
r/ prestenoticdiffuse lfocal biliarydilatation(AA%)
./ progressionof ductalstricturei (100%) malignancy
. septicemia, CNS depression,lethargy,mental
US i CT :
confusion,shock(50%)
r/ dilatationof intrahepaticductswithoutextrahepatic
{ purulentmaterialfillsbiiiaryducts
ductdilatation
Prognosis: 100"hmortalityif not decompressed;
r/ failureto demonstratethe confluenceof L + R
40-60% mortafitywith treatment;
, hepaticducts 13-16% overallmortalityrate
r/ masswithin/ surrounding the ductsat pointof Organism.' gram-negativeentericbacteria= E. coli >
, obstruction (21% visible on US, 40% visibleon CT)
r/ infiltrating Klebsiella> Pseudomonas> Enterococci
tumorvisibleas highlyattenuatinglesion . recurrentepisodesof sepsis+ RUe pain
i n 2 2 / . o n C T , i n 1 S %o n U S . Charcottriad(70/"): fever+ chills+ jaundice
r/ exophytictumorvisiblein 100"/.on CT as low- . bile culturesin 90./"positivefor infection
attenuationmass,in 2g/" on US
r/ polypoidintraluminal r/ may have gas in biliarytree
tumorvisibleas isoechoic C E C T:
mass withinsurroundingbile in 100./"on US, in
r/ transienthepaticparenchymal enhancementin
25/" on CT
periportallocationon hepaticarterialphase
C E CT :
(= hyperemicchangesaroundbileducts)
r/ hyperattenuating lesionat delayedimaging(due to
Cx: miliaryhepaticabscessformation;secondary
delayedaccumulation+ washoutof fibrouscenter)
sclerosi ng cholangitis
688 Radiology Review Manual

AlDS-relatedCholangitis Histo:
= AIDSCHOLANGIOPATHY Stage 1: degenerationof epithelialbile duct cells
with lymphocytes
+ infiltration + neutrophils;
= infectiouscholangitischaracterizedby opportunistic
inflammation + scarring+ enlargement of
organisms
(protozoanparasite periportaltriads (pericholangitis)
Organism; Cryptosporidium
Stage2: fibrosis+ inflammation periportal
infiltrating
typicallyinfectingGl tractepithelium)' CMV
markedperiductalinflammatoryresponsewith parenchymawith piecemealnecrosisof
Histo:
interstitialedema + interstitialinflammatory hepatocytes;enlargementof portaltriads;
cell infiltrates+ necroticbiliaryepithelium bileductoPenia
. RUQ pain,fever,nausea,jaundice Stage3: portal-to-portal fibroussepta;severe
. elevatedWBC count degenerativechanges+ disappearanceof
. abnormalLFT (esp.serumalkalinephosphatase) bileducts;cholestasisin periportal
. opportunisticorganismisolatedfrom bile (in 50%) + paraseptalhePatocYtes
r/ irregularmild dilatationof intra-and extrahepaticbile Stage4: frankcirrhosis
ductsresemblingsclerosingcholangitis Associated with:
(1) lnflammatory boweldisease(ulcerative colitisin
US :
{ strictureof distalCBD / papillarystenosis(due to 50-74/o, Crohn diseasein 13%)
papillitis) 0 14% of patientswith inflammatorybowel
r/ echogenicnoduleat the distalend of the CBD diseasedevelopsecondarysclerosing
{ muralthickeningof gallbladder+ bileducts cholangitis!
{ periductalechogenicity (2) Cirrhosis,chronicactivehepatitis,pericholangitis,
{ + pericholecystic fluid fatty degeneration
CT : (3) Pancreatitis
/ mediastinalfibrosis
r/ "pseudogallstone" appearance= In€lrked (4) Retroperitoneal
circumferential edema of gallbladderwall + mucosal (5) Peyroniedisease
(6) Riedelthyroiditis, hypothyroidism
enhancement
r/ periportaledema (7) RetroorbitalPseudotumor
Cholangiography: (8) Sjogrensyndrome
' abnormalliverfunctiontests: serumbilirubin,serum
{ strictures+ beadingof centralintrahepaticbile
alkaline phosphatase,y-gIutamyltransf erase
ducts
. progressivechronic/ intermittentobstructivejaundice
{ pruningof peripheralbileducts
DDx: acalculouscholecystitis,papillarystenosis, (75%)
. historyof previousbiliarysurgery(53%)+ chronic/
sclerosingcholangitis
recurrentPancreatitis(14%)
. fever,nightsweats,chills,RUQ pain,pruritus
Gholangitis
Chemotherapy-induced (10-15% )
= inflammatoryfibrosingprocessaboutthe portaltriads Location:
simulatingprimarysclerosingcholangitis 1. CBD almostalwaYsinvolved
Predisposed: patientswith liver metastasesfrom 2. Intra- and extrahepaticducts (68-89%)
coloncancer 3. Cysticduct involvedin 15-18%
Cause: directeffectof hepaticarterialinfusionwith 4. lntrahepatic ductsonly (1-11-25%)
chemotherapeutic agents(eg,floxuridine)
/ 5. Extrahepatic ducts onlY(2-3%)
ischemiasecondaryto thrombosisof
intrahepaticarterialbranches r/ intrahepaticbile duct calculi(8-30%): soft black
./ oiteduct stricturesas early as 2 monthsaftertherapy crushablestones/ sandlikegrit
( in up t o 1 5 % ) US:
{ strictureof commonhepaticduct + sparingof distal nbrightlyechogenicPortaltriads
CBD { echogenicbiliarycasts/ punctatecoarse
calcificationsalong portalvein branches
PrimarySclerosingCholangitis ^/ t gallbladderwall thickening
= insidiousprogressiveobliterativefibrosinginflammation CT:
of the biliarytree causingmultifocalstrictures, bile dilatation,stenosis,pruning(decreased
duct obliteration,cholestasis,and biliarycirrhosis arborization),beadingof tortuousintrahepaticbile
Etiology: idiopathic,? autoimmuneprocess ducts = "tree-in-winter"
appearance(80%)
alteredbile acid metabolism
(speculative); ./ wall nodularity,ductwall thickening,muralcontrast
with increasein lithocholicacid by bacterial enhancementof extrahepaticbile ducts (100%)
overgrowth hepaticmetastases+ lymph nodes in porta hepatis
! subtlefoci of highattenuationin intrahepaticbile
Prevalence: 1o/"as commonas alcoholicliverdisease
Age: <45 years (213);mean 39 (range21-67) years; ducts
,Vlobaratrophyin preferentiallyaffectedportions
M : F= 7 : 3
 Disorders of Liver, Biliary Tract, Pancreas, and Spleen
Cholangiography:
r/ multifocalstrictureswith predilectionfor bifurcations
+ skip lesions(uninvolved duct segmentsof normal
caliber)involvingintra-and extrahepatic bileducts:
! CLASSlC"string-of-beads" appearance
(= alternatingsegmentsof dilatationand focal
annularstenoses)
r/ "prunedtree"appearance(= opacificationof
centralducts+ nonvisualization of peripheral
smallerradiclesdue to diffuseobstruction)
{ "cobblestone"appearance(= coarse nodular
muralirregularities) in 50%
d new strictures+ lengtheningof stricturesbetween
6 monthsand 6 years (<20%)
r/ minimalduct dilatationdue to periductal
inflammation + fibrosis
r/ markedductaldilatation(24%)
DDx: ascendingcholangitis,chofangiocarcinoma
r/ smalleccentricsaccularoulpouchingsldiverticula
pseudodiverticula) [up to 27%l = PATHOGNOMONTC
r/ webs = focal 1-2-mm-thickaieas of incomplete
circumferentialnarrowing
r/ anglesformedbetweenientral and peripheral
ducts changefrom acuteto obtuse
r/ polypoidmass (7%)
! gallbladderirregularities uncommon
MR:
r/ periportafintermediateintenbityon Tl Wl
+ hyperintense on T2Wl (dueto inflammation)
NUC (Tc-99m-lDAscan):
r/ multiplepersistentfocal areasof retentionin
distribution of intrahepatic biliarytree
r/ markedprolongationof hepaticclearance
r/ gallbladder visualizedonly in 70"/"
Cx: (1) Biliarycirrhosis(up to 49%)
(2) Portalhypertension
(3) Cholangiocarcinoma (clinicallyin 4-19%; in
7-360/"at autopsy lliver transplantation)
(4) Secondarycholangitis
Rx: (1) Palliative:ursodeoxycholic acid,dilatationof
dominantstrictures
(2) Curative:livertransplantation (4th leading
indication)
DDx:
(1) Sclerosingcholangiocarcinoma (progressive
cholangiographic changeswithinO.S-1.5yearsof
initialdiagnosis,markedductaldilatationupstream
from a dominantstricture,intraductalmass >1 cm
in diameter)
(2) Acuteascendingchotangitis (history)
(3) Primarybiliarycirrhosis(diseasetimitedto
intrahepaticducts,stricturesless pronounced,
pruning+ crowdingof bileducts,normalAMA titer)
(4) AIDScholangiopathy (sameon cholangiography)
RecurrentPyogenicCholangitis
= PRIMARYCHOLANGITIS = RECURRENTPYOGENIC
HEPATITIS = ORIENTALCHOLANGTO-
/ CHOLANGITIS
HEPATITIS= ORIENTALCHOLANGTTTS
= HONGKONG
DISEASE= INTRAHEPATICPIGMENTSTONEDISEASE
6g9
= chronicrecurrentparasiticcholangitisresultingin
progressivedestructivecholangiopathy+ liverfailure
Etiology: ? Clonorchissinensisinfestation,coliform
infectionof bile,portalbacteremia,malnutrition
lncidence; Srd most commoncause of an acute
abdomenin Hong Kong after appendicitis
and perforatedulcer;uncommonin United
States
Epidemiology; endemicto SoutheastAsia (South
China,Indochina, Taiwan,Japan,
K orea);A si an i mmi grants
i n Unit ed
States
Associated intrabiIiary infestation:
Clonorchissinensis,Ascarislumbricoides, E. coli
Path: pericholangitis, periductalabscesses,fibrosisof
bile duct walls, heavy infiltrationof portaltractsby
PMNs,intraductalbile pigmentcalculi
/ Age: 20-50 years; M:F = 1:1
. recurrentattacksof fever,chills,abdominalpain,
jaundice
Location: particularly in lateralsegmentof L lobe
+ posteriorsegmentof R lobe
markeddilatationof proximalintrahepaticducts
(3-4 mm) in 100%
r/ decreasedarborizationof intrahepaticradicles
r/ intra-and extrahepaticbile ducts titted*itn
nonshadowingsoft mudlikepigment(= calcium
bilirubinate) stones(74%)
r/ dilatationof CBD (68%)+ chotedochotithiasis (30%)
{ muftifocalbile duct strictures(22h)
r/ pneumobilia(3-52%)
r/ biloma
{ segmentalhepaticatrophy(36%)
! hepaticabscesses
C T:
./ frign-anenuation biliarycalculi
! enhancementof bile duct wall
Associated findings:
ri gallstones
r/ splenomegaly
r/ varices
ERCP:
0 Worseningof cholangitis/ sepsisif patientsdo not
receiveantibiotics!
r/ acutetapering+ straightening+ rigidityof bile ducts
{ decreasedarborization+ increasedbranching
angleof bile ducts
Cx: liverabscess(18%),splenomegaly (14%),biloma
(4%),panc reatit is (4./"),c ho Iang iocarcino ma
(2.5-6%)
Rx: endoscopicsphincterotomy,
choledochod uodenostomy
DDx: (1) Carolidisease(sacculardilatationof
intrahepaticbile ducts)
(2) Primarysclerosingcholangitis(focal
discontinuous bileduct dilatation)
(3) Clonorchiasis (biliaryductaldilatationlimited
to intrahepaticbile ducts)
690 Radiology Review Manual

SecondarySclerosingCholangitis { gallstones(83-98% sensitive,52-77"/"specific):

Cause:
(1) chronicbacterialcholangitis from bileduct stricture
/ choledocholithiasis
(2) ischemicbileduct damagefromtreatmentwith
floxuridine
(3) infectiouscholangiopathy in AIDS
(4) previousbiliarytract surgery
(5) congenitalbiliarytree anomalies
(6) bileduct neoplasm
ri impactedgallstonein GB neck/ cysticduct
r/ echogenicshadowingfat withinhepatoduodenal
ligament+ conspicuous colorDopplerflow (due
to inflammation)
DDx: bowelgas
r/ sludge
C ol orD oppl erU S :
{ visualizationof cysticartery>50/" of the lengthof
the gallbladder(30%sensitive,98% specific)

C E C T:
r/ distendedgallbladder
CHOLECYSTITIS r/ gallbladderwall thickness>3 mm
AcuteCalculousCholecystitis ./ increasedgallbladderwall attenuation
Etiology: (a) in 80-95% cystic duct obstructionby ri transientfocal increasedattenuationaround
impactedcalculus;85% disimpact gallbladderfossaon hepaticarterialphase(dueto
spontaneously if stone<3 mm hepaticarterialhyperemia+ earlyvenousdrainage)
(b) in 10o/"acalculouscholecystitis r/ hazinessof pericholecystic fat
Pathogenesis;chemicalirritationfrom concentrated ri pericholecystic fluid
bile,bacterialinfection,refluxof ^/ increasedattenuation of bile
pancreaticsecretions
Age peak; 5th-6thdecade;M:F = 1:3 NUC (86-97% sensitivity,73-100% specificity, 95-98%
Associatedwith: choledocholithiasis (15-25%) accuracy):
. persisting(>6 hours)RUQ pain radiatingto right = functionalinformation aboutgallbladder+ cystic
shoulder/ scapula/ interscapular area (DDx:biliary duct patency
colicusually<6 hours) 0 Tracer uptakehingeson adequatehepaticfunction
. nausea,vomiting,chills,fever + fastingstatus
. RUQ tenderness+ guarding ri nonvisualizationof GB during1st hour (in 83%)
. r leukocytosis,elevatedlevelsof alkaline = evidenceof cysticduct obstruction
phosphatase and transaminase and amylase ^/ nonvisualizationof GB by 4 hours(99%specific)
. mild hyperbilirubinemia (20%) ^i nonvisualizationof GB + CBD (in 13%)
. Murphysign = inspiratory arrestupon palpationof GB { pericholecystic rim sign (34%sensitive)on initial
area (falselypositivein 6/" of patientswith images= increasedhepaticactivityadjacentto
cholelithiasis) empty GB fossa (= localhepatocyteinflammation
Oral cholecystography: + hyperemiain transmuralprocess);57o/oPPY tor
r/ nonvisualization / poorvisualization of gallbladder gangrenousGB + 94o/oPPV for acutecholecystitis
US (81-1 00% sensitivity,60-1 00% speciticity,92/" ^/ increasedperfusionto GB fossaduring"arterial
P P V ,95% NP V): phase"(in up to 80%)
r/ t CA wall thickening>3 mm (45-727osensitive, Endpoint of imaging
76-88% specific): - when tracerfillsGB
r/ trazydelineationof GB wall - 4 hours of delayedimagingaftertracerinjection
./ "halosign"= GB wall lucency(in 8%) = 3-layered - 45 minutesaftermorphineinjection
configuration withsonolucent middlelayer(edema)
r/ striatedwall thickening(62%)= several False-positivescans(10-12/") = nonvisualization of
alternatingirregulardiscontinuous lucent GB withoutacutecholecystitis:
+ echogenicbandswithinGB wall (100%PPV) prolongedfasting,totalparenteralnutrition,
r/ Cg hydrops= distensionwith AP diameter>5 cm or hyperalimentation, recentfeeding<4-6 hoursprior
enlargement of greaterthan 4 x 10 cm to study,severeintercurrent illness,CBD
{ positivesonographicMurphysign (in 85-88%) obstruction, congenitalabsenceof GB, post-
= maximumtendernessduringcompression with cholecystectomy, carcinomaof GB, chronic
transducerdirectlyover gallbladder(63-94% cholecystitis,acutepancreatitis, alcoholicliver
sensitive,85-93% specific,72% NPV) disease,hepatocellular disease
False-negativesonographic Murphy sign: Reduction to 2% false-positivescans through:
lackof patientresponsiveness, pain medication, (1) delayedimagesup to 4 hours
inabilityto pressdirectlyon GB (positiondeepto (2) cholecystokinin(Sincalide@) injection15
liver/ protectedby ribs),GB wall necrosis minutespriorto study
r/ crescent-shaped / loculatedpericholecystic fluid (in (3) morphinelV (0.04mg/kg)at 40 minutes
20%) = inflammatoryintraperitoneal exudate/ + reimagingafter 20 minutes(contractionof
abscess pressure)
sphincterof Oddi + risein intrabiliary

False-negativescans (a.B%)= visualizationof GB
despiteacutecholecystitis:
rare calculous/ acalculouscholecystitiswithout
cysticduct obstruction
Cholangiography:
{ sharplydefinedfillingdefectin contrast-material
filledlumenof cysticduct
MR cholangiopancreatogram (highsensitivity)
: r/ sloughedmucosalmembrane
! low-signal-intensity defectsurroundedby high-
signal-intensity bile on T2Wl
Cx:
mnemonic; "GAME BEG"
Gangrene
Abscess (pericholecystic)
Mirizzisyndrome
Emphysematous cholecystitis
Bouveretsyndrome(= gallstoneerodesinto
duodenumleadingto duodenalobstruction)
Empyema
Gallstoneileus
(1) Gangreneof gallbladder
., positiveMurphysign (33%)
r/ shaggy,irregular,asymmetricwall (mucosal
ulcers,intraluminal hemorrhage, necrosis)
^/ hyperechoicfoci withinCe watt
, (microabscesses in Rokitansky-Aschoff sinuses)
r/ intraluminal pseudomembranes (gangrene)
r/ coarsenonshadowingnondepenJent-
echodensities(= sloughednecroticmucosa/
sludge/ pus / clottedbloodwithingallbladder)
(2) Perforationof gallbladder(in Z-20%)
(a) acutefree perforationwith peritonitiscausing
pericholecysticabscess in 33%
(b) subacutelocalizedperforationcausing
pericholecystic abscessin 4g%
(c) chronicperforationresultingin internalbiliary
fistulacausingpericholecysticabscessin 1g%
Location: most commonlyat fundus
{ gallstonelyingfree in peritonealcavity
r/ sonolucent/ complexcollectionsurrounding
r/ collectionin liveradjacentto gallbladder
(3) Empyemaof gallbtadder
r/ multiplemedium/ coarsehighlyreftective
intraluminalechoeswithoutshadowing/ layering
/ gravitydependence(purulentexudate/ debris)
AcuteAcalculousCholecystitis
Frequency; 5-1 5/" of all acute cholecystitiscases
Associatedwith: recent surgery in 50%
Etiology: probablycaused by decreasedblood flow
withincysticartery
(1) depressedmotility/ starvationin trauma,burns,
surgery,total parenteralnutrition,anesthesia,
positivepressureventilation,narcotics,shock,
vasoactiveamines,congestiveheartfailure,
arteriosclerosis,
polyarteritis
nodosa,SLE,
diabetesmellitus
(2) obstructionof cysticduct by extrinsicinflammation,
lymphadenopathy, metastases
Disordersof Liver,BiliaryTract,pancreas,and Spteen 691
(3) infection(onlyin 50%)from Salmonella, cholera,
Kawasakisyndrome
r/ thickenedgallbladder wall >4-5 mm
1 echogenicbile/ sludge
ri gallbladderdistension
r/ pericholecysticfluid in absenceof ascites
! subserosaledema
! Murphysign = pain + tendernesswith transducer
pressureoverthe gallbladder
r/ intramuralgas
r/ decreasedresponseto cholecystokinin
NUC: same criteriaas for calculouscholecystitis
Cx: gallbladderperforation, gangrene
Prognosis: 6.5/" mortalityrate
ChronicCholecystitis
0 Mostcommonform of gallbladderinflammation
r/ gallstones
{ smoothI irregularGB wall thickening(meanof 5 mm)
r/ meanvolumeof 42 mL
NUC:
r/ normalGB visualizationin majorityof patients
./ delayedGB visualization (1-ahours)
ri visualizationof bowelpriorto GB (sensitivity41o/o,
specificity90%)
r/ noncontractility/ decreasedresponseafter CCK
injection(decreasedGB ejectionfraction)
Emphysematous
Cholecystitis
= ischemiaof gallbladderwall + infectionwith gas-
producingorganisms
Etiology: small-vesseldisease with cystic artery
occlusion,complication of acute
cholecystitis
Organism; Clostridiumperfringens,Clostridium
welchii,E. coli,staphylococcus,
streptococcus
Age: >50 years; M:F = 5:1
GB Predisposed
:
:i33:l::
fT;:ffi}(9;lx|1il?
acalculouscysticduct obstruction
. WBC countmay be normal(1/3)
. pointtendernessrare (diabeticneuropathy)
P l ai nfi l m:
1 g". appears24-48 hoursafter onset of symptoms
r/ air-fluidlevelin GB lumen,air in GB wall within
24-48 hours after acute episode
^/ pneumobilia (rare)
US:
{ arclikehigh-levelechoesoutliningGB wall
{ cholecystolithiasis (50%)
Cx: gangrene(75%);gallbladderperforation(20%)
Mortality: 15%
DDx: (1) Entericfistula
(2) Incompetent sphincterof Oddi
(3) Air-containing
periduodenal abscess
(4) Periappendicealabscessin malpositioned
appendix
(5) Lipomatosisof gallbtadder
692 Radiology Review Manual

Gholecystitis
Xanthogranulomatous Classification:
= FIBROXANTHOGRANULOMATOUS INFLAMMATION malunionof pancreaticobiliary duct
= CEROID GRANULOMAS OFTHEGALLBLADDER Kimuratype | = pancreaticduct entersthe proximal/
= ur'rcolTlrTloninflammatorydiseaseof gallbladder mid CBD (10-58%)at rightangle
characterizedby presenceof multipleintramural Kimuratype ll = CBD drainsintopancreaticduct
nodules Prevalence:1:13,000admissions;highprevalencein
Etiology: Japanese/ Asian infants
ruptureof occludedRokitansky-Aschoff sinuseswith Age: <10 years(60%)+ youngadulthood;80/"
subsequentintramuralextravasationof inspissated diagnosedin childhood;7%duringpregnancy;
bile + mucinattractinghistiocytesto phagocytosethe occasionallydetectedup to 8th decade; M:F = 1:4
insolublecholesterol Histo: fibrouscystwall withoutepitheliallining
Incidence: 1-2h Associated with:
Age: 7Ih + 8th decade (1) dilatation,stenosisor atresiaof otherportionsof the
Histo: mixtureof ceroid (waxlike)xanthogranuloma biliarylree (2"/o)
with foamy histiocytes+ multinucleated foreign (2) gatlbladderanomaly(aptasia,doubleGB)
body giant cells + lymphocytes+ fibroblasts (3) failureof unionof left + right hepaticducts
containingareasof necrosis(in newerlesions) (4) pancreaticduct + accessoryhepaticbile ducts may
May be associatedwith: gallbladdercarcinoma(11%) drain into cyst
{ preservationof 2-3-mm thick mucosallining(in 82%) (5) polycysticliverdisease
r/ thickenedgatlbladderwall: 91% diffuse,97ofocal . Classictriad (20-30% of adult patients):
fat: in 45o/"focal,in 54/o
r/ infiltrationof pericholecystic (1) intermittentobstructivejaundice(33-50%)
0 Uncommoncauseof obstructive jaundice!
diffuse
r/ hepaticextension(45%) (2) recurrentRUQ colickypain (>75-90A), back pain
r/ biliaryobstruction(36%) (3) intermittent palpableRUQ abdominalmass (<25%)
r/ tymphadenopathy (36%) . recurrentfever,chills,weightloss,pruritus
US: Types:
^/ intramuralhypoechoicnodules (a) markedcysticdilatationof CBD + CHD
CT: (b) focal segmentaldilatationof CBD distally
r/ S-zO-mmsmallintramuralhypoattenuating nodules (c) cylindricdilatationof CBD + CHD
^/ poor / heterogeneouscontrastenhancement { size: diameterof 2 cm uP to 15 cm
DDx: gallbladdercarcinoma(in 59% focal,in 41"/" (thelargestcholedochalcystcontained13liters)
diffusethickeningof gallbladder wall,multiple r/ lrlO/ mild peripheralintrahepaticbile duct dilatation
masseswithinliver) ^/ may containstones/ sludge
UGI:
CYST
CHOLEDOCHAL r/ soft-tissuemass in RUQ
= CYSTICDILATATION OF EXTRAHEPATIC BILEDUCT r/ anteriordisplacement of 2nd portionof duodenum
aneurysmaldilatationof commonbile duct
= SegrTl€I'ttal + distalportionof stomach(on LAT view)
withoutinvolvementof gallbladder/ cysticduct; most r/ wideningof C-loopwith inferiordisplacementof
commoncongenitallesionof bileducts duodenum(on AP view)
Etiology: anomalousjunctionof pancreaticduct and US:
CBD proximalto duodenalpapilla;higher ./ ballooned/ fusiformcyst beneathporta hepatis
pressurein pancreaticduct and absentductal separatefrom gallbladder
sphincterallowsfree refluxof enzymesinto 0 Communicationwith commonhepatic/ intrahepatic
CBD resultingin weakeningof CBD wall ducts needsto be demonstrated!
r/ abruptchangeof caliberat junctionof dilated
segmentto normalducts
r/ intrahepatic bileduct dilatation(16%)secondaryto
stenosis
OB-US(earliestdiagnosisat25 weeksMA):
r/ right-sidedcyst in fetal abdomen+ adjacentdilated
hepaticducts
DDx: duodenalatresia;cyst of ovary,mesentery,
omentum,Pancreas,liver
N U Cw i th H ID A :
0 At timesthe choledochal cyst does not fill with
radionuclide!
{ photopenicareawithinliverthat fillswithin60 minutes
+ stasisof tracerwithincYst
Type I a TypeI b TypeI c lack of tracerpassageinto small intestine
Choledochal Cysts prominenthepaticductalactivity(dilatationof ducts)
 Disordersof Liver,BiliaryTract,Pancreas,and Spleen 693

DDx: often excludeshepaticcyst, pancreatic Rx: sphincterotomy i sphincteroplasty

pseudocyst, entericduplication, spontaneous DDx: choledochalcyst (involvesmore than only terminal
loculatedbiloma portionof CBD)
Cholangiography / MR cholangiography (confirms
diagnosis):
r/ anomalousjunctionof panreaticobiliary C H OLE LITH IA S IS
ductalsystem
./ ditatedintrahepaticbile ducts Prevalence:
r/ intraductalcalculi 25 millionadultsin UnitedStates;10/" of population
Cx: (1) Stonesin gallbladder, in CBD,withincyst,in + 2/" of chi l dren;
intrahepaticbiliarytree, in pancreaticduct increasingwith age (40% of women in 9th decade);
(8-50%) in 3rd decadeM:F = 2/.:4./.
(2) Malignanttransformation intobileduct in 7th decadeM:F = 10"h:25/"
carcinoma+ gallbladdercarcinoma(increasing Predisposingfactors.' "female,forty, tair,fat,fertile,
wit hag e ,< 1 " /" i n1 s td e c a d e ,T -1 4 /o> a ge 20) flatulent"
3) Recurrentpancreatitis(33%) Pathogenesis:
4) Chofangitisi cholecystitis(20%) supersaturation of bile constituents,most notably
5) Cyst rupturewith bile peritonitis (1.8%) cholesterol, relatedto defectsin biliarylipid
6) Bleeding metabolism; biliarydysmotility; prolongedintestinal
7) Biliarycirrhosis+ portalhypertension transit;aggravatedby sedentarylifestyle+ diet
8) Portalveinthrombosis (a) Hemolyticdisease
9) Hepaticabscess sicklecell disease(7-37%),hereditary
Rx: excisionof cyst + Roux-en-yhepaticojejunostomy spherocytosis (43-85/"), th alassemia, pernicious
DDx: mesenteric, omental,ovarian,renal,adrenal, anemia(16-20%),prostheticcardiacvalves+ mitral
hepatic,entericduplicationcyst, pancreatic stenosis(hemolysis), cirrhosis(hemolysis
pseudocyst,hydronephrotic kidney,hepaticartery secondaryto hypersplenism), Rhesus/ ABO blood
aneurysm,biloma(fromspontaneous perforation of groupincompatibility (perinatalperiod)
cBD) (b) Metabolicdisorder= disruptionof biliarylithogenic
index
diabetesmellitus,obesity,pancreaticdisease,cystic
fibrosis,hypercholesterolemia, type4
CHOLEDOCHOCELE hyperlipidem ia, hemosiderosis(20%),
= DUODENAL DUPLICATION CYST= ENTEROGENOUS CYST hyperparathyroidism, hypothyroidism, prolonged
OFAMPULLA OFVATERi DUODENUM = INTRADUODENAL use of estrogens/ progesterone,pregnancy
CHOLEDOCHAL CYST= DIVERTICULUM OF COMMON (c) Cholestasis
B I LEDUCT - hepaticdysfunction:hepatitis,neonatalsepsis
= cysticdilatationof the distal/ intramuralduodenal - biliarytree malformation:Carolidisease
portionof the CBD with herniationof CBD into - biliaryobstruction:parasiticinfection,benign/
duodenum(similarto ureterocele) malignantstrictures, foreignbodies(sutures,
Etiology: ascariasis)
( 1)c ongenit al : - prolongedfasting(totalparenterafnutrition)
(a) originatesfromtiny bud / diverticulum of distal - methadoneintake
CBD (foundin 5.7"/"of normalpopulation) (d) Intestinalmalabsorption
(b) stenosisof ductalorifice/ weaknessof ductalwall has a 10 x increasedrisk of stone formation
(2) acquired: - Inflammatory boweldisease:Crohndisease
stone passagefollowedby stenosis+ inflammation (28-34%)
Age: 33 years (manifestation usuallyin adulthood) - ileal resection
Types: (a) CBD terminatesin cyst,cyst drainsinto - bypasssurgery
duod e n u m(c o mmo n ) (e) Geneticpredisposition = familial
(b) cyst drainsinto adjacentintramuralportionof Navaho,Pima,ChippewaIndians
CBD (lesscommon) (f) Others
. biliarycolic,episodicjaundice,nausea,vomiting musculardystrophy
Associatedwith: stones/ sludge(frequent)
UGI: GALLSTONESIN NEONATE
r/ smoothwell-defined intraluminalduodenalfilling 0 Rarewithoutpredisposingfactors
, defectin regionof papilla Associatedwith: obstructivecongenitalbiliary
r/ changein shapewith compression / peristalsis anomaly,totalparenteralnutrition,
Cholangiography (diagnostic) : furosemide, Gl dysfunction(short-
r/ opacifiedsmoothclublike/ saclikedilatationof gut syndrome),prolongedfasting,
intramuralsegmentof CBD prolapsedintoduodenum phototherapy, dehydration,
Cx: pancreatitis,duodenalobstruction infection,hemolyticanemia
694 Radiology Review Manual

IN O L D ERC H IL D R EN
GA LLS T O NE S
Associatedwith: sicklecell disease,cysticfibrosis,
malabsorption, total parenteral
nutrition,Crohndisease,intestinal
resection,hemolyticanemia,
choledochalcyst
Composition:
A. CHOLESTEROL STONE(70%)
= ffioincomoonentof most calculi
r/ lucent(93%),calcified(7%)
r/ slightlyhypodensecomparedwith bile
(a) purecholesterol stones(10%):yellowish,soft
r/ buoyancyin contrast-enhanced bile
r/ densityof <100 HU
(b) mixtureof cholesterol+ calciumcarbonate/
(70%)
bilirubinate
r/ laminatedappearance
{ radiopaqueon plainfilm (15-20%)
B. PTGMENT STONE(30%)
. brown(common)= granularprecipitate of calcium
biIirubinate containing <25/" cholesterol(by
definition)
Cause: inflammationi infectionof gallbladder,
statuspost cholecystectomy
' black (lesscommon)= compact"lacquer"of
bilirubinderivatives with a highaffinityfor calcium
carbonate
r/ multipletiny faceted/ spiculatedhomogeneously
radiopaquestones
CT :
./ usuallydenserthan bile
Radiopacity:
ri lucentstones (84%):
cholesterol(85%),pigment(15%)
r/ calcifiedstones(15-20% on plainfilm, 60% on CT):
cholesterol(33%),pigment(67%)
Locationof calcium:
{ calciumphosphatedepositedcentrallywithin
cholesterolstones
r/ calciumcarbonatedepositedradiallywithinaging
cholesterol / peripherallyaroundcholesterol
+ pigmentedstones
FLOATTNG GALLSTONES (20-25%)
(a) relativelypure cholesterolstones
(b) gas-containing stones
(c) risein specificgravityof bile (1.03)from oral
cholecystopaques (specificgravityof 1.06)causing
stones (specificgravityof 1.05)to float
GAS-CONTAI N ING GALLSTONES
Mechanism: dehydrationof older stonesleadsto
internalshrinkage+ dendriticcracks
+ subsequentnitrogengas-fillingfrom
negativeinternalpressure
{ "crow-foot"= "Mercedes-Benz" sign = radiating
streaklikelucencieswithinstone,also responsiblefor
buoyancy
SLUDGE
= calcium-bilirubinate granules+ cholesterol crystals
associatedwith biliarystasis
Cause: prolongedfasting,parenteralnutrition,
hyperalimentation, hemolysis,extrahepatic
bile duct obstruction,cysticduct obstruction,
acute + chroniccholecystitis
r/ nonshadowing homogeneously echogenicmaterial:
{ fluid-sludge level
r/ "sludgeball"= tumefactivesludge:
{ slowlyshiftingwith repositioning of patient
DDx: gallbladdercancer
Prognosis; may cause acute cholecystitis
DDx: hemobiliawith blood clot, parasiticinfestation,
MUCUS
Cholecystolithiasis
. asymptomatic(60-65%);becomesymptomaticat a
rate of 2/" per year
. biliary colic (misnomer)due to transientobstruction
of cysticduct i commonbile duct developsin 33%
(18%overallriskin 20 years):
= ?cUt€RUQ / epigastric/ LUQ / precordial/ lower
abdominalpain increasingover seconds/ minutes
+ remainingfairlysteadyfor 1-3(-6) hours
associatedwith nausea+ vomiting
. no tendernessuponpalpation
Abdominalplainfilm (10-16%sensitive):
{ calcifiedgallstones
OCG (65-90% sensitive):
r/ tittingdefectin contrastedgallbladderlumen:
r/ allowsdetermination of size + numberof
gallstones
r/ demonstratescysticduct patency
r/ showscontractilityafter a fatty meal
r/ nonvisualization of gallbladder(25%)= inconclusive
CT (80%sensitive):
^/ hyperdensecalcifiedgallstonesin 607o
^/ hypodensecholesterolstones<140 HU = pure
cholesterolstone (= 280o/"cholesterolcontent):
0 InverserelationshipbetweenCT attenuation
number+ cholesterol content
{ gallstonesisointenseto bile in 21-24/" and thus
undetectable by CT (<30 HU)
US (91-98%sensitive;in 5/" falselynegative):
^/ Orignt(= highlyreflective)echo from anterior
surfaceof gallstonewithingallbladder:
{ markedposterioracousticshadowing
r/ mobileupon repositioning of patient(may
infrequentlybe adherentto wall)
r/ reverberationartifact
0 Small calcifications<2 mm may not shadow
./ nonvisualization of GB + collectionof echogenic
echoeswith acousticshadowing(15-25%):
{ "wall-echo-shadow" = "double-arcshadow"sign
= 2 echogeniccurvilinearparallellinesseparated
by sonolucentline (ie,anteriorGB wall + bile
+ stonewith acousticshadowing)
 Disorders of Liver, Biliary Tract, Pancreas, and Spleen 695

! focalnonshadowing opacities<5 mm in diameter NUC:

(in 70% gallstones) r/ delayedbowelactivitybeyond2 hours
False-negative US (5%): r/ persistenthepatic+ commonbile duct activityto
contractedGB, GB in anomalous/ unusuallocation, 24 hours
smallgallstone,gallstoneimpactedin GB neck/ r/ prominentductalactivitybeyondg0 minutes
cysticduct,immobilepatient,obesepatient, with visualization
of secondaryducts
extensiveRUQ bowelgas DDx: air bubbles,neoplasm,concentrated bile
Prognosis; stones<3 mm may pass throughcysticduct B . S TON EIN C Y S TICD U C TR E MN A N T:
Cx: acutecholecystitis (in 90"/"),choledocholithiasis, retainedin 0.4/" aftersurgeryfor choledocholithiasis
cholangitis,pancreatitis, duodenitis,biliaryfistula,
gallstoneileus,Mirizzisyndrome;cancerof GB
+ bile ducts (2-3 x more frequent)
CHRONIC
GRANULOMATOUS
DISEASE
OF
Cholangiolithiasis CHILDHOOD
A . CHO LE DO C H OL IT H IAS IS = fecessiVeX-linked(60%)/ autosomal@0%)
0 Mostcommoncauseof bileduct obstruction! immunodef iciencydisorderresultingin purulent
Etiology: (a) passedstonesoriginatingin GB infections+ granulomaformationprimarilyinvolving
(b) primarydevelopmentin intra-/ l ymphnodes,ski n,l ungs
extrahepaticducts Etiology: polymorphonuclear leukocytedysfunction
lncidence; in 12-15% of cholecystectomy patients; characterizedby inabilityto generatehydrogen
in 3-4h of postcholecystectomy peroxidecausingprolongedintracellular
patients;in75/" of patientswith chronic survivalof phagocytizedcatalase-positive
bileduct obstruction bacteriawith dissemination in
Risk indicators for CBD stone: reticuloendothelialsystem
(1) recenthistoryof jaundice Organism.' mostcommonlystaphylococcus, Serratia
(2) recenthistoryof pancreatitis marcescens, Nocardiaspecies,
(3) elevatedserumbilirubin>17 pmol/L mycobacteria, fungi
(4) elevatedserumamylase>120llJlL Path: chronicinfectionwith granulomaformation/
(5) dilatedCBD >6 mm (16%) caseation/ suppuration
(6) obscuredbileduct Age: onsetin childhood;M > F (moreseverein boys)
. recurrentepisodesof rightupperquadrantpain, . recurrentchronicinfections
jaundice,chills,fever (ZS-50%) . nitrobluetetrazolium test: low percentageof WBCs that
. elevatedtransaminase(75%) reducethe dye afterstimulationby phagocytosis /
. spontaneouspassagewith stones<6 mm size contactwith endotoxin(normally>90%)
Cholangiography (mostspecifictechnique): @ B one
r/ stone visualizationin g2o/" { osteomyelitis (commonlyof spine,ribs,metatarsals)
Peroperative cholangiography: @ Chest
prolongsoperationby 30 minutes; r/ chronicpneumonia
4% false-negatives; 4-10% false-positives ^/ rritarlymphadenopathy
r/ dependentroundfillingdefects r/ pleural+ pericardial effusions
DDx: air bubbles @ Liver
US (22-82% sensitive): r/ hepatosplenomegaly
r/ stonevisualizationin 1g-75% (more readilywith r/ hepaticabscess(mostcommonabdominalprocess)
CBD dilatation+ goodvisibilityof pancreatic r/ livercalcifications
head) @ Gl tract
r/ dilatedducts in 64-770/oiduct <g mm in . chronicdiarrheawith malabsorption
diameter in 24-36./" ., vomiting,anorexia,heartburn,weightloss
./ increaseddilatationof CBD with administration ri esophagealdysmotility, esophagitis, stricture
, of fatty meal/ cholecystokinin ./ gastricantralnarrowingt gastricoutletobstruction
r i no s t on ei n g a l l b l a d d e(1
r .2 -1 1 % ) ^/ perianalfistula+ abscess
CT,(88%sensitive,97% specific,g4ohaccurate): @ GU tract
{ stonevisualization in 75-88% (isoattenuating to . dysuria
bile in 12-25%) r/ cystitis
r/ targetsign = intraluminal masswith crescentic r/ obstructionof urethra+ ureters
, ring (= stone of soft-tissue density)in 85% @ Lymphnodes
{ subtlealternatinglow-and high-attenuation ./ suppurativelymphadenitis
rings(= mixedcholesterol-calcium stones) @ Skin
M RCP( 81- 1 0 0 %s e n s i ti v e8, 5 -1 0 0 %s p e c i fi c): . pyoderma
r/ dark fillingdefectwithinhyperintense fluid Rx: prophylactic long-termtrimethoprim-
(stonemustbe >2 mm in diameter) sulfamethoxazole + interferongammatherapy
696 Radiology Review Manual

CI RRHO S I S (b) cirrhoticnodule= fegel'l€fative nodulelargely/

= chronicliverdiseasecharacterizedby diffuse completelysurroundedby fibroussepta
parenchymalnecrosis,regenerationand scarringwith (c) dysplasticnodule[adenomatoushyperplasia]
abnormalreconstruction of preexistinglobular = clusterof hepatocytes >1 mm in diameterwith
architecture evidenceof dysplasia;commonin hepatitisB and C,
Etiology: alpha-1antitrypsin deficiency, tyrosinemia
A. TOXIC (d) hepatocellular carcinoma
(1) Alcoholicliverdiseasein 75/" Associatedwith: anemia,coagulopathy,
(2) Drug-induced(prolongedmethotrexate, hypoalbuminemia, cholelithiasis,
oxyphenisatin, alpha-methyldopa, nitrofurantoin, pepticulcerdisease,
pancreatitis,
isoniazid) diarrhea,hypogonadism
(3) lron overload(hemochromatosis, hemosiderosis) . anorexia,weakness,fatigue,weightloss
B . I NF LA M M AT ION . jaundice,continuouslow-gradefever
(1) Viralhepatitis . ascites,bleedingfrom esophagealvarices,hepatic
(2) Schistosomiasis encephalopathy
C. BILIARYOBSTRUCTION r/ enlarged(earlystage)/ normal/ shrunkenliver
(1) Cysticfibrosis r/ shrinkageof right lobe (segments5-8) and medial
(2) Inflammatory boweldisease segmentof left lobe (segments 4a + 4b) with
(3) Primarybiliarycirrhosis concomitanthypertrophyof lateralsegmentof left lobe
(4) Obstructiveinfantilecholangiopathy (segments2 + 3) and caudatelobe(segment1):
D. VASCULAR r/ ratioof caudateto right lobe >0.65on transverse
(1) ProlongedCHF = cardiaccirrhosis images[sensitivity43-84/", leastsensitivein
(2) Hepaticvenoocclusive disease(Budd-Chiari alcoholiccirrhosis,mostsensitivein cirrhosiscaused
syndrome) by hepatitisB; specificity100%; 26% sensitivity;
E . NUT RI T IO N AL 84-96% accuracyl(DDx: Budd-Chiarisyndrome)
(1) Intestinal bypass { diameterof quadratelobe (segment4) <30 mm
(2) Severesteatosis (= distancebetweenleft wall of gallbladderand
(3) Abetalipoproteinemia ascendingportionof left portalvein) due to selective
F . HE RE DI T A R Y atrophy(95% specific)
( 1) W ils o nd i s e a s e r/ widenedporta hepatis+ interlobarfissure
(2) Alpha-1antitrypsindeficiency r/ surfacenodularity+ indentations(regeneratingnodules)
(3) Juvenilepolycystickidneydisease { signsof portalhypertension
(4) Galactosemia ./ splenomegaly
(5) Type lV glycogenstoragedisease r/ ascites(failureof albuminsynthesis,overproduction of
(6) Hereditaryfructoseintolerance lymphdue to increasedhydrostaticpressurein
(7) Tyrosinemia sinusoids/ decreasedsplanchnicoutputdue to portal
(8) Hereditarytetany hypertension)
(9) Osler-Weber-Rendu syndrome r/ associatedwith fatty infiltration(in early cirrhosis)
( 10) F am i l i acl i rrh o s i s US (sensitivity65-80%; DDx: chronichepatitis,fatty
G . I DI O P A TH IC / C R YP T O GE N IC infiltration):
Heoaticsions:
Cirrhosisin children; chronic hepatitis,congenital r/ hepatomegaly(63%)
hepaticfibrosis,cysticfibrosis, { hypertrophyof caudatelobe (26%):
biliaryatresia,alpha-1antitrypsin ! ratioof width of caudatelobeto width of right
deficiency,tyrosinemia, hepaticlobe >0.65 (43-84% sensitive,
galactosemia, hemochromatosis, 100%specific)
Wilsondisease,schistosomiasis, ^/ surfacenodularity(88% sensitive,82-95% specific)
total parenteralnutrition ./ increasedhepaticparenchymalechogenicityin
Morphology: 66% (as a sign of superimposed fattyinfiltration):
(a) micronodular cirrhosis(<3 mm): usuallydue to ! increasedsound attenuation(9%)
alcoholism, biliaryobstruction, hemochromatosis, { decreased/ normaldefinitionof walls of portal
venousoutflowobstruction,previoussmall-bowel venules(signof associatedfatty infiltrationNOT
bypasssurgery,Indianchildhoodfibrosis of fibrosis)
(b) macronodular cirrhosis(3-15 mm, up to severalcm): ri heterogeneouscoarse(usually)/ fine echotexture
usuallydue to chronicviralhepatitis,Wilsondisease, (7%)
alpha-1antitrypsindeficiency { occasionaldepictionof isoechoicregenerative
(c) mixedcirrhosis nodules
Nodular lesions: r/ dilatationof hepaticarteries(increasedarterialflow)
(a) regenerativenodules = localizedproliferationof with demonstrationof intrahepaticarterialbranches
hepatocytes+ supportingstroma (DDx:dilatedbiliaryradicals)
 Disorders of Liver, Biliary Tract, Pancreas, and Spleen Og7

r/ increasein hepaticarteryresistanceafter meal NUC (Tc-99m-labeled sulfurcolloid):

ingestion r/ nigh blood pool activitysecondaryto slow clearance
r/ "portalization"
of hepaticvein waveform { colloidshiftto bone marrow+ spleen+ lung
= dampenedoscillations of hepaticveins r/ shrunkenliverwith littleor no activity+ splenomegaly
resemblingportalvein flow r/ mottledhepaticuptake(pseudotumors) on colloicf
Ext,rahepaticsigns: scan (normalactivityon IDA scans!)
{ splenomegaly r/ displacement of liver+ spleenfrom abdominalwall by
! ascites ascites
r/ signs of portalhypertension Cx: (1) Ascites: cause/ contributorto death in 50%
(2) Portalhypertension
CT: (3) Hepatocellularcarcinoma(in 7-12%)
native+ enhancedparenchymal
inhomogeneity (4) Cholangiocarcinoma
^/ decreasedattenuation(steatosis)in earlycirrhosis Fatality from:
,Visodensei hyperdense(siderotic)regenerative esophagealvaricealbleeding(in 25%),hepatorenal
nodules syndrome(10%), spontaneous bacterialperitonitis
^/ nodular/ lobulatedlivercontour (5-10%),complications from treatmentof ascites(10%)
predominantlyportalvenoussupplyto dysplastic
nodules
./ hypodensearea adjacentto portalvein (= peribiliary
cystsfrom obstructedextramuralperibiliaryglands)
{ rapidtaperingof intrahepaticportal+ hepaticvenous PrimaryBiliaryCirrhosis
branches = CHRONIC NONSUPPURATIVE DESTRUCTIVE
CECT: CHOLANGITIS
{ enlargedtortuoushepaticartery(compensatory Histo: idiopathicprogressivedestructivecholangitisof
increasein arterialbloodflow) interlobarand septalbile ducts,poftal fibrosis,
{ arterioportal shunts(throughtrans-sinusoidal shunts nodularregeneration, shrinkageof hepatic
in liverperiphery+ transplexalshuntswith parenchyma
hypertrophyof peribiliaryplexus)in hepaticarterial Age: 35-55 years; M:F = 1;9
phase: Associated autoimmune disorders:
{ poorlydemarcatedtransientperipheralwedge- rheumatoidarthritis,Hashimotothyroiditis, Sjogren
shapedhepaticparenchymalenhancement syndrome,scleroderma,sarcoidosis
DDx: hepatocellular carcinoma(defecton portal 0 66-100%of patientswith primarybiliarycirrhosis
venousphase) have sicca-complex signsof the Sjogrensyndrome
{ early retrogradeenhancementof portalvein . fatigue,pruritus
branches . xanthelasmai xanthoma(25%)
r/ hepatofugalflow . hyperpigmentation (50%)
Cause: with occlusionof small hepaticvenules . insidiousonsetof pruritus(60%)
the portalvein turnsfrom a supplying . lgM increased(95%)
vein intoa drainingvein . positiveantimitochondrial antibodies(AMA)in 85-100%
MR (problem-solving tool): r/ normalextrahepaticducts
r/ no alterationof liverparenchyma { cholelithiasis in 35-39%
d regeneratingnodules= hypolntenselesions(due to CT:
irondepositswithinnodules)with hyperintense septa r/ scattereddilatedintrahepaticducts with no
(due to vascularity)on T2Wl apparentconnectionto mainbileducts
{ dysplasticnodule= iso- / hyperintenseon Tl Wl r/ caudatelobe hypertrophy(in 98%):
+ iso-/ hypointense on T2Wl r/ hypertrophiedhyperattenuating caudatelobe
{ HCC nodule= hypo-i iso-/ hyperintense on Tl Wl surroundedby hypoattenuating rindlikerightlobe
+ usuallyhyperintenseon T2Wl with marked (pseudotumor)
enhancement duringarterialphase { atrophyof lateralsegmentof left hepaticlobe
r/ intrahepaticbiliarycalculi(ZO%)
Angio: NUC:
r/ stretchedhepaticarterybranches(earlyfinding) { markedprolongationof hepaticTc-g9m IDA
r/ enlargedtortuoushepaticarteries=,"corkscrewing" clearance
(increasein hepaticarterialflow) { uniformhepaticisotoperetention
r/ shuntingbetweenhepaticarteryand portalvein r/ normalvisualization of GB and majorbileductsin
r/ mottledparenchymalphase 100%
i/ delayedemptyinginto venousphase DDx: (1) Sclerosingcholangitis(youngmen)
tr pruningof hepaticvein branches(normallydepiction (2) CBD obstruction
of Sth order branches)= postsinusoidal
compression Prognosis.' mean survival6 (range 3-1 1) years after
by developingnodules onset of cholestaticsymptoms
698 Radiology Review Manual

Complicationsof End-StageLiver Disease . remittentincompleteobstruction+ bacterial

HepatopuImonary Syndrome
(1) chronicliverdisease
Dx:
gradient
(2) increasedalveolar-arterial
(3) intrapulmonary vasculardilatation
' hypoxemia(in 1i3 of decompensatedcirrhotic
patients)
Pathomechanism:
elevationof unknownvasoactivesubstancesin
cirrhoticpatientcausepulmonaryvasculardilatation
(from8-15 pm to 15-500 pm) + resultin diffusion-
perfusionmismatch
{ basilarnodular/ reticulonodularareasof increased
opacity(in 46-100%)
CONGENITAL BILIARY ATRESIA
{ ditatedarterioleswith an increasednumberof
terminalbranchesextendingto pleura
{ intrapulmonary arteriovenous shunt(demonstrated
with Tc-99mmacroaggregated albuminimaging,
microbubble echocardiography)
Hepatic Hydrothorax
= large pleuraleffusionin cirrhoticpatientwithout
primarypulmonary/ cardiacdisease
Prevalence: 10%
Mechanism: pressuregradientfavorsfluid
movementfrom peritonealto pleural
cavitythroughsmalldiaphragmatic
defects
in 17"/",bilateralin
{ pleuralfluid: rightin 67"/o,left
17o/o
PuImonary Hypertension
Prevalence: 0.73% in patientswith livercirrhosis
(versus013% in all patients)
Cause:
(a) thromboembolic:portalvenousthrombus
reacheslungthroughspontaneous / surgically
createdportosystemicshunts
(b) plexogenic:vasoactivesubstances(serotonin,
thromboxane,neuropeptideY, elastase)bypass
the liverthroughportosystemicshunts
Prognosis.'mean survivalof 15 months
superinfection
r/ multiplecrescent-/ stiletto-shaped fillingdefectswithin
bileducts:
{ echogenicfocus/ caston US
^/ dittusetythickenedbile ducts
Cx: (1) Bileduct obstruction(conglomerate of worms/
adenomatousProliferation)
(2) Calculusformation(stasis/ dead worms /
epithelialdebris)
(3) Jaundicein 8% (stonei stricture/ tumor)
(4) Generalized dilatationof bileducts (2%)
Etiology: ? variationof same infectiousprocessas in
neonatalhepatitiswith additionalcomponent
or vascularinjury
of sclerosingcholangitis
Prevalence: <10 in 100,000livebirths
Age: neonate;M:F = 2:1
Histo: periportalfibrosis,proliferation of small
intrahepatic bileducts,mixedinflammatory
infiltrates
ln 15%associatedwith: polysplenia,trisomy18
Types:
vascularinsult(extremelyrare)
I Focal= intrauterine
ll Intrahepatic biliaryatresia= paucityof intrahepatic
biteducts (uncommon)
lll Extrahepatic biliaryatresia= atresiaof CBD + patent
intrahepatic bileducts
Subtype1 = perinataltYPe(66%)
. jaundicedevelopsafter regressionof
physiologic jaundice
^/ Oiteduct remnantin porta hepatis
Subtype2 = embryonic/ fetal type (34%)
. normaldeclinein bilirubindoes not occur
^/ lttObile duct remnantin porta hepatis
Associatedwith: polysplenia(10-l 2%), intestinal
malrotation,azygoscontinuationof lVC,
symmetricbilobedliver,situsinversus,
preduodenalportalvein,anomaloushepatic
arteries,bilobedrightlung,complexCHD

CLONORCHIASIS

Mr
Rarelyof clinicalsignificance
Country: endemicto SoutheastAsia: Japan, Korea,
Central+ SouthChina,Taiwan,Indochina
Organism; Chineseliverfluke= Clonorchissinensis
Cycle: parasitecysts digestedby gastricjuice, larvae
migrateup the bileducts,remainin small
ru
Iw
intrahepatic ductsuntilmaturity(10-30 mm in
length),travelto largerductsto depositeggs
lnfection: snail + freshwaterfish serve as intermediate
hosts;infectionoccursby eatingraw fish;hog,
dog, cat, man are definitehosts
Path: (a) desquamation of epithelialbileduct liningwith
adenomatousproliferation of ducts+ thickening \
of duct walls(inflammation, necrosis,fibrosis) TypeA Type B
(b) bacterialsuperinfectionwith formationof liver
abscess
Biliary Atresia
 Disordersof Liver,BiliaryTract,Pancreas,and Spleen 699
US:
,Vnormal/ increasedsize of liver
^/ normal/ increasedliverechogenicity
^/ decreasedvisualizationof peripheralportalveins (due
to fibrosis)
{ "triangularcord"/ tubularechogenicstructurein porta
hepatis(dueto fibroustissue)= PATHOGNOMONIC
r/ gallbladderfindings:
.i nonvisualization of gallbladder
{ smallgallbladder<1.5cm in length+ varying
degreesof luminalcompromise(DDx: hepatitis)
{ normalgallbladder >1.5 cm in length(19%)when
atresiaof CBD distalto insertionof cysticduct
r/ Oiteduct findings:
r/ no dilatationof intrahepaticbile ducts (due to
, panductalsclerosis)
r/ + visualizationof bile duct remnantin porta hepatis
(dependingon type of biliaryatresia)
r/ smallfocal cysticdilatationof extrahepaticbile duct
(= choledochalcyst) = patentsegmentof CBD with
other parts beingoccludeddue to fibrosis
* communicationwith gallbladder/ intrahepaticbile
ducts
NUC [phenobarbital-augmented cholescintigraphy]
(90-97"/" sensitive,60-94% specific,75-90% accurate):
" preparationof patientwith 5 ng/kg/dphenobarbital
twice a day for 3-7 days to stimulatebiliarysecretion
(via inductionof hepaticenzymes+ increasein
conjugation+ excretionof bilirubin)
{ good hepaticactivitywithin5 min (infantsof
<3 monthsof age have a normalhepaticextraction
fraction)
r/ trtObiliaryexcretion:
{ NO visualizationof bowelon delayedimagesat
6 and 24 hours
{ delayedclearancefrom cardiacblood pool
{ increasedrenalexcretion+ bladderactivity
DDx: severehepatocellular dysfunction(DDx from
neonatalhepatitisimpossiblein the absenceof
small bowelactivity)requiresliverbiopsy
MR cholangiography:
r/ nonvisualization of extrahepaticbile ducts
{ atrophicgallbladder
r/ periportalthickening
Cholangiography (percutaneous i endoscopic/
intraoperative)
Liver Bx (60-97% accurate)
Rx: (1) Roux-en-Ycholedochojejunostomy (20%);
(2) Kasaiprocedure= portoenterostomy (80%)
(a) child <60 days of age: 91% successrate
(b) child between60 and 90 days of age: 50%
successrate (due to developingcirrhosis)
(c) child >90 days of age: 17o/osuccessrate
(3) Livertransplant
DDx:
(1) Neonatalhepatitis
(2) Sclerosingcholangitis
(3) Alagille syndrome = arteriohepatic dysplasia
(abnormalfacies,butterflyvertebra,pulmonic
stenosis,complexCHD)
CONGENITAL HEPATIC FIBROSIS
= coflg€rritalcirrhosiswith rapid+ fatal progression
Histo: fibroustissuewithin hepaticparenchymawith
excessnumbersof distortedterminalinterlobular
bile ducts + cyststhat rarelycommunicatewith bile
ducts
Age: usuallypresentin childhoodresultingin early
death
Associated with:
autosomalrecessivepolycystickidneydisease
(invariably),Meckel-Grubersyndrome,vaginalatresia,
tuberoussclerosis,nephronophthisis, medullarysponge
kidney(80%),autosomaldominantpolycystickidney
disease(rare)
. hepatosplenomegaly, portalhypertension
. predisposed to cholangitis+ calculi
./ "lollipop-tree"= ectasiaof peripheralbiliaryradicles
ri hepatosplenomegaly
r/ periportalfibrosis+ portosystemiccollaterals
Cx: portalhypertension,hepatocellular carcinoma,
cholangiocellularcarcinoma
ECHINOCOCCAL DISEASEOF LIVER
EchinococcusGranulosus
= HYDATID = unilocularform
DISEASE
= E. cysticus(morecommon);man is accidentalhost
(a) pastoral(European)form: dog is definitehost;
intermediatehostsare cattle,sheep,horses,hogs;
endemicin sheep-raising countries:Australia,
New Zealand,North+ East Africa,USSR,
Mediterraneancountries,Near + MiddleEast,
Japan,Argentina,Chile,Uruguay
(b) svlvatic(northern)form: wolf is definitehost:
intermediate hostsare deer,moose;endemicin
northwesternCanada,Alaska
Cycle: ingestionof contaminated material(eggspassed
in feces of dog / other carnivore);eggs hatch in
duodenum;larvaepenetrateintestinalwall
+ mesentericvenules;larvaecarriedinto portal
circulation; larvaeare filteredin capillaries
of
liver(firstlineof defense)> lung> otherorgans
Histo:
A. CYST FLUID= antigenicclear lpale yellowfluid
with neutralpH containingsodiumchloride,
proteins,glucose,ions,lipids,polysaccharides
Definite host
Parasitic Cycle of Echinococcus Granulosus
700 Radiology Review Manual

B. ENDOCYST(parasiticcomponentof capsule) r/ multivesicularcyst of "racemose"/ honeycomb

= innerGERMINATIVELAYER(resembling wet appearance= multiplesepta betweendaughter
tissuepaper)givingriseto brood capsules cysts insidemothercyst, CHARACTERISTIC but
(daughtervesicles),whichmay rare"
(a) remainattachedto cyst wall harboringup to r/ "wheelspoke"pattern= daughtercysts separated
400,000scolices by echogenicmaterialof hydatidmatrix
(b) detach+ form sedimentin cyst fluid = "hydatid composedof brokendaughtervesicles+ scolices
sand" + hydatidsand
(c) breakup into numerousself-contained r/ HtCHI-VSPECIFICserpentinelinearstructures
daughtercysts withinhydatidmatrix
C. ECTOCYST= CYST MEMBRANE= acellular r/ partial/ completedetachmentof endocystfrom
laminatedchitinlikesubstancesecretedby pericyst(due to decreasingintracysticpressureas
parasite,allowingpassageof nutrients a sign of degeneration/ traumai host responsei
D. PERICYST= densefibrousprotectivezone of host responseto therapy):
granulation tissuereplacingtissuenecrosis(from r/ localizedsplitin wall with floatingundulating
compressionby the expandingcyst);marginal membrane.CHARACTERISTIC but rare
vascularrim of 0.5-4 mm { "waterlilysign"= completedetachmentof
. pain / asymptomatic membrane
. recurrentjaundice+ biliarycolic (transientobstruction 0 Floatingmembranedoes not indicatedeath of
by membranefragments+ daughtercysts expelled parasite!
into biliarytree) r/ eggshellcalcification in cystwall (leastcommon)
. blood eosinophilia(20-50%) CT:
' urticaria+ anaphylaxis(foltowingrupture) r/ well-demarcatedroundlow-densitymass of fluid
. Tests: attenuation(3-30 HU):
1. Casoniintradermal test (60%sensitivity; { cystwall of highattenuation on NECT
may be falselypositive) ./ linearareas of increasedattenuation= detached
2. Complementfixationdoublediffusion laminatedmembrane
(65% sensitivity) ^/ round peripheralfluid collectionof lower
3. lmmunoelectrophoresis (mostspecific) attenuation(= daughtercYsts)
4. Indirecthemagglutination (85%sensitivity) ^/ enhancementof cyst wall + septations
Time to diagnosis: 11-81 (mean51) years { calcificationof cyst wall / internalsepta
Affected organs: MR:
tiver(73%);lung (14/"); peritoneum(12%);kidney r/ cyst with hypointenserim (= collagenouspericyst)
(3-6%);spleen(0.9-8%);CNS (1%);orbit(1%);bone on Tl Wl + T2Wl
(0.5a%); bladder;thyroid;prostate;heart r/ peripheralcystswithincyst hypointense on TlWl
Location: right lobe > left lobe of liver;multiplecysts + hyperintense on T2Wl (= daughtercysts)
in 2 0 /. ./ twistedlinearstructureswithinclst = collapsed
Size: up to 50 cm (averagesize of 5 cm), up to 16 liters parasiticmembrane
of fluid;grows2-3 cm annually A ngi o:
Stages of cyst growth: r/ avasculararcawith splayingof arteries
(1) Unilocular cyst ^/ natoof increaseddensityaroundcyst (inflammation
(2) Cyst with daughtervesicles/ daughtercysts / compressedliver)
(3) Partially/ completelycalcified Cholangiography:
P lainf ilm : r/ cyst may communicate with bileducts:righthepatic
! peripheralcrescentic/ curvilinear / polycyclic duct (55%),left hepaticduct (29%),CHD (9%),
calcifications (10-20-33%), locatedin pericyst: gallbladder(6'/"),CBD (1%)
0 Onlycompletecalcification of all layersimplies Percutaneousaspiration:
death of parasite! . fluid analysispositivefor hydatiddiseasein 7O"h
r/ pneumohydrocyst (infection/ communication with (fragmentsof laminatedmembranein 54/";
bronchialtree) scolicesin 15h; hookletsin 15%)
US: 0 Riskof anaphylactic shock(0.5%),asthma(3/"),
r/ complexheterogeneous mass mimickinga solid implantation of spilledprotoscoleces
mass (mostcommon):
0 Lookfor membranes / peripheral daughtervesicles Local Cx:
r/ well-defined anechoiccyst (common): (1) Rupture(50-90%)
{ cyst wall of doubleechogeniclinesseparatedby (a) contained= ruptureof laminatedmembrane
hypoechoiclayer of endocyst,pericystremainsintact
r/ "snowstormsign" = multipleinternalechogenic r/ floatingmembranes
foci settlingto most dependentportionof cyst (b) communicating= cYstcontentsescapes
(= hydatidsand) throughbiliary(5-15%)/ bronchialtree
 Disordersof Liver,Biliary Tract,Pancreas,and Spteen 701
(c) direct= tear of endocyst+ ectocyst+ pericyst
with cyst contentsspillingintopleural/
peritonealcavity(anaphylaxis,metastatic
hydatidosis)
(2) lnfection(5-8%) followingrupture
(3) Transdiaphragmatic arowth(0.6-16%) through
bare area of liver
(a) ruptureinto pleuralcavity
(b) seedingin pulmonaryparenchyma
(c) chronicbronchialfistula
(4) Perforation intohollowviscus(0.S%)
(5) Peritoneal seeding(13%)= encyst€dperitoneal
hydatidosis
(6) Compressionof vital structures(bileducts,pofial
v ein)
Rx: (1) Surgery(in 107orecurrence)
(2) Anthelmintics(albendazole,medendazole)
(3) Injectionof scolecidalagents(silvernitrate,
20 I 30% hypertonicsalinesolution,
45% cetrimidesolution,95% ethanol)
EchinococcusMultilocularis
= E. alveolaris= lesscommonbut moreaggressive
form of echinococcaldisease
Primaryhost: fox, wolf
Secondaryhost: rodents(moles,lemmings,wild
mice);domesticcat;dog
Endemicto: easternFrance,southernGermany,
westernAustria,muchof SovietUnion,
Japan,Alaska,Canada,some areasin
Turkey
lnfection: eatingwild fruitscontaminatedwith fox / wolf
feces;directcontactwith fox /wolt; contact
with dogs i cats that have ingestedinfested
rodents
Path: larvae proliferateby exogenousextension
+ penetration of surrounding tissue(= diffuse
+ infiltrative
processresemblingmalignancy);
chronicgranulomatous reactionwith central
necrosis,cavitation, calcification
Histo: daughtercystswith thick lamellarwall arisingon
outer surfaceof originalcyst, rarelycontaining
scolices
Location: liver(accessvia portalvein);widespread
hematogenous dissemination not uncommon
. clinicalmanifestation5-20 years after ingestion
. abdominaldiscomfort, jaundice,hepatomegaly
. eos inophili a
^/ aggressivegrowthpattern:
! geographicinfiltrating lesionwith ill-definedmargins
r/ invasionof lVC,diaphragm
r/ metastasesto lung,heait,brain(in 10%)
^/ faint / dense amorph-ous coalescentnodular/ flame-
shapedcalcifications(dystrophiccentralcalcifications
scatteredthroughoutnecrotic+ granulomatoustissue)
US:
^/ echogenicgeographicill-defined single/ multiple
solid masses
*
1 itt"grlar cysticareas
r/ propensityof spreadto liverhilum
C T:
^/ heterogeneoushypodensepoorlymarginated
infiltratingmasses
^/ pseudocysticnecroticregionsof near water density
surroundedby hyperdensesolidcomponent
r/ tittte/ no enhancement
A ngi o:
^/ intrahepaticarterialtapering+ obstruction
Cx: Budd-Chiarisyndrome,IVC thrombosis,portal
hypertension
Prognosis; fatal within 10-15 years (if left untreated)
DDx: hepatocellular carcinoma(biopsyl),large
hemangioma(characteristic enhancement
pattern),metastasis,epitheliaf
hemangioendothelioma
EMBRYONAL RHABDOMYOSARCOMA
OF
BILIARYTREE
= tdta tumormostcommonlyarisingfrom CBD
Medianage:3years;M>F
Path: intraluminalbiliarymass / cfusterof grapelike
masses(similarto rhabdomyosarcoma of bladder)
Histo: same as sarcomabotryoides
. malaise,fever,jaundice
. elevationof conjugatedbilirubin
Metastases(in up to 30%) to:
retroperitoneal+mesentericlymphnodes,lung
Location:commonbileduct (mostfrequently)
r/ 8-20-cm bulky heterogeneousmass in poita hepatis
r/ intrahepaticbile duct dilatation
./ displacement of duodenum,stomach,pancreas
Cholangiography:
^/ largebulkyintraluminal mass/ grapelikeclusterof
intraluminalmassesfocallydistendingcommonbile
duct + obstructingproximalbile ducts
EPIDERMOID
CYSTOFSPLEEN
= EPITHELIAL CYST= PRIMARY CYSTOF SPLEEN
lncidence: 10/" of all benignnonparasiticcysts
Cause: infoldingof peritonealmesothelium / collection
of peritonealmesothelialcells trappedwithin
splenicsulci
Histo: (1) mesotheliallining
(2) squamousepitheliallining= epidermoidcyst
= squamousmetaplasiafrom embryonic
inclusionswithinpreexisting mesothelial
surfaceepithelium
Age: 2nd-3rd decade(averageage of 18 years)
May be associatedwith: polycystickidney disease
(a) unilocular+ solitary(80%)
(b) multiple+ multilocular (20%)
! well-definedthin-walledanechoiclesionof water density
^/ averagesize of 10 cm
r/ peripheralseptations/ cyst wall trabeculations(in g6%)
r/ curvilinearcalcificationin wall (9-25%)
r/ may containcholesterolcrystais,fat, blood
Cx: trauma,rupture,infection
702 Radiology Review Manual
EP I T HE LI O I D HEMA N GIOE N D O T H EL IOMA
= f€lleprimarymalignantvasculartumor of liver(soft
tissue,bone,lung)
Age: averageage of 45 years;M:F = 1:2
Possiblyassociatedwith: oral contraceptives,exposure
to vinylchloride
Path: multifocalnodulesvaryingin size from a few mm
to severalcm involveboth lobesof the liver (due
to rapidperivascular extension);nodulesmay
coalescein liverperiphery
Histo: dendriticspindle-shaped cells + epithelioidround
cellsin a matrixof myxoid+ fibrousstroma;
neoplasticendothelial cellsinvadesinusoids
+ terminalhepatic+ portalveins cuttingoff the
tumor'sbloodsupply
. in 807o:abdominalpain,weakness,anorexia,jaundice
Metastasesto: spleen,mesentery,lymph nodes,lung,
bone
r/ multiplenodules(nodularform)
r/ peripheralsubcapsulargrowth (diffuseform)without
, deforminglivercontour
r/ increasedtumor vascularity
r/ hypertrophyof uninvolvedliver
P lainf ilm :
r/ hepaticcalcifications withinmyxoidstroma(15%)
US:
r/ typicallyhypoechoiclesions(due to centralcore of
myxoidstroma)
CT:
r/ low-attenuation masseson NECT,may become
isoattenuating with rest of liveron CECT (due to
vasoformativegrowth+ compensatoryhepaticarterial
flow with portalvein occlusion)
A ngio:
r/ hyper-and hypovascularity (dependentupon degree
of sclerosis+ hyalinization)
^/ invasion+ occlusionof portal+ hepaticveins
NUC:
./ decreasedperfusionto centralmyxoidtumor portion
+ increasedperfusionto cellularareas on sulfur
colloidscan
{ photopenicdefecton staticsulfurcolloidscan
{ NOT galliumavid
Prognosis: 20o/"die within 2years,207osurvivefor
5-28 years + treatment
DDx of multiplenodules: metastaticdisease
DDx of diffuseform: sclerosingcarcinoma,vaso-
occlusivedisease
FATTYLIVER
= FATTYINFILTRATION OFTHELIVER= HEPATIC
STEATOSIS
Cause:
A . M E T A B OL ICD E R A N GE M EN T
poorlycontrolleddiabetesmellitus(50%),obesity,
hyperlipidemia, acute fatty liverof pregnancy,protein
malnutrition,total parenteralhyperalimentation (TPN),
malabsorption(jejunoilealbypass),glycogenstorage
disease,glycogensynthetasedeficiency,cystic
fibrosis,Reye syndrome,corticosteroids, severe
trauma,chronicillness(TB, CHF)
hepatitis,
B. HEPATOTOXINS
alcohol(>50%),carbonchlorides,phosphorus,
amiodarone, chemotheraPy
Histo: hepatocyteswith largecytoplasmicfat vacuoles
containingtriglycerides;>57ofat of total liver
weight
. NO abnormalliverfunctiontests
{ rapidchangewithtime (fewdaysto >10 months)
dependingon clinicalimprovement(abstinence from
alcohol,improvednutrition)+ degreeof severity
DiffuseFatty lntiltration
r/ hepatomegaly(75-80%)/ normalsized liver
P l ai nfi l m:
r/ radiolucentliversign = enlargedradiolucentliver
US (sensitivity>90%,accuracy85-97%):
r/ increasedsound attenuation(scatteringof sound
beam) = poor definitionof posterioraspectof liver
r/ fine (moretypical)/ coarsenedhyperechogenicity
(comparedwith kidney)
./ impairedvisualizationof bordersof hepaticvessels
{ attenuationof sound beam (featureof fat, NOT
fibrosis)
CT:
{ areas of lowerattenuationthan normalportalvein /
IVC density
r/ reversalof liver-spleendensityrelationship(liver
densityis normally6-12 HU greaterthan spleen)
{ hyperdenseintrahepaticvessels
NUC:
Tc-99msulfurcolloidscan:
./ diffuseheterogeneousuptake(68%)
{ reversalof liver-spleenuptake(41%)
r/ increasedbone marrowuptake(41%)
Xe-133 ventilationscanl
{ increasedactivityduringwashoutphase (38%)
MR:
! slightlyincreasedsignalon TlWl + T2Wl; relatively
insensitive (10%fat by weightwill alterSE signal
intensities only by 5-15%)
./ fat turns blackwith Dixontechnique
FAT-SPAREDAREA in diffusefatty infiltration
Cause: directdrainageof systemicblood into liver
Location: (a) posterioredge of segment4 = anterior
to portalvein bifurcation(drainageof
aberrantgastricvein)
(b) nextto gallbladderbed (drainageof
cysticvein)
(c) subcapsularskiPareas
{ hypoechoicovoidi spherical/ sheetlikemass
^/ ttO mass effect(undisplacedcourseof vessels)
DDx: tumor mass
FocalFattyInfiltration
Etiotogy: ? vascularorigin,focaltissuehypoxia
 Disordersof Live4 Biliary Tract,Pancreas,and Spteen 703

Distribution:(a) lobar/ segmentaluniformlesions arterieswithinfibroussepta radiatingfrom the

(b) lobar/ segmentalnodularlesions centertowardthe periphery;absentportaltriads
(c) perihilarlesions + centralveins;difficultdifferentiation from
(d) diffusenodularlesions regenerativenodulesof cirrhosis
(e) diffusepatchylesions + hepatocellular adenoma
predominantly in centrilobar+ periportal Age peak; 3rd-4th decade(range:7 monthsto
regions,subcapsulardistributionmay be 75 years)i M:F = 1:2-4
due to variantsof bloodsupply(dueto Associatedwith: hepatichemangioma(in 23/"),
"thirdinflow"
from connectionbetween meningioma, astrocytoma, arterial
peripheralportalradicles+ perforating dysplasiaof otherorgansin case of
capsular/ accessorycysticveins) mul ti pl eFN H
Location: rightlobe,caudatelobe,perihilarregion . initiallyoften asymptomatic(in 50-90% incidental
r/ fan-shapedlobar/ segmentafdistributionwith discovery)
angulated/ interdigitating geographicmargins . vagueabdominalpain (10-15%)due to mass effect
./ lesionsextendto peripheryof liver . normalliverfunction
./ trtOmass effect(undisplaiedcourseof vessels,no . hepatomegaly/ abdominalmass
bulgingof livercontour) Location:rightlobe:leftlobe = 2:1
US: Size: <5 cm (in 85%)
d hyperechoicarea with poorlydefined/ sharp r/ well-circumscribed nonencapsulated nodularcirrhotic-
margins like mass in an otherwisenormalliver:
r/ multipleI rarelysingleechogenicnodules
r/ often near liversurface
simulatingmetastases(rare) r/ pedunculatedmass (in 5-20%)
CT : r/ multiplemasses(in 2O%)
r/ patctryareasof decreasedattenuationrangingfrom r/ centralscar containingarteriovenousmalformation
- 40 t o + 1 0 H U (D D x : l i v e rtu m o r)
^/ nigf'tyvasculartumor
r/ tttOcontrastenhancement
MR (notsensitivefor fat): , r/ hemorrhageis unlikely
ri calcificationsare EXTREMELYrare
r/ nighsignalon Tl Wi + low / isointensesignalon N E C T:
T2WI r/ iso-/ slightlyhypoattenuating homogeneous mass
NUC with colloid: C E C T:
{ no significant changeson sulfurcolloidimages ^/ transientintensehyperdensity(after30-60 sec) on
(SPECTimagingmay detectfocal fatty infiltration)
bolusinjectionfollowedrapidlyby isodensity:
DDx: primary/ secondaryhepatictumor ^/ hypodensemass duringpeaL portaluenousphase
r/ isodensemassduringequilibrium phase
FOCAL NODULAR HYPERPLASIA
= FNH = r?r€ benigncongenitalhamartomatous , 0 Lesionmay be missedwithoutprecontrast studyl
ri hypodensecentralstellatescdt = centralfibrouscore
malformationor reparativeprocessin areas of focal with radiatingfibroussepta(15-93%)(DDx:
inJ ur yS; P E CIF ICD IAGN O S ISR A R E L YP O S SIB LE fi brol amel l ar
H C C ):
lncidence: 2nd most commonbenigntumor of liverafter r/ t earlyenhancement of vesselstraversingcentral
hemangioma;4/"of all primaryhepatic scar
tumorsin pediatricpopulation,3_g/o in adult r/ hyperdensecentralscar on delayedimages
population;twice as commonas hepato- (delayedwashoutof contrastfrom myxomatous
cellularadenoma;only357 casesreported scar tissue)
Cause: (?) congenitalarteriovenousmalformation US:
triggersfocalhepatocellular hyperplasia owing r/ iso-/ hypo-/ hyperechoic(33%) homogeneousmass
to a regionalincreasein bloodflow ^/ hyperechoiccentralscar in 18.h
0 Oral contraceptives DO NOT causeFNH,but r/ displacementof hepaticvessels
exert a trophiceffecton its growth! Doppler:
Path: localized,well-delineated, usuallysolitary(90_gS%), ri enlargedafferentbloodvesselwith centralarterial
subcapsularmassof numeroussmalllobuleswithin hypervascularity + centrifugal fillingto the peripheryin
an otherwisenormalliver;no truecapsule;frequenily "spoke-wheel"pattern
centralfibrousscar in areaof interconnection of r/ largedrainingveinsat tumormargins
fibrousbands(HALLMARK)containingcentrallyan r/ may show high-velocity Dopplersignalswith arterial
arteriovenous malformation with spiderlike pulsatilityfrom arteriovenousshunts
branchessupplyingthe componentnodules NUC:
Histo: composedof multiplesphericalaggregatesof S ul furcol l oi dscan:
hepatocytesoften containingincreasedamounts r/ normaluptake(SO-70%),hot spot (7-10%)
of fat + triglycerides
+ glycogen;Kupffercells; 0 Only FNH containssufficientKupffercellsto
bile duct proliferationwithinfibroussepta causenormal/ increaseduptake(almost
withoutconnectionto biliarytree;thick-walled P A TH OcN OMON TC )!
704 Radiology Review Manual

{ cold spot (30-50%) (5) Hypervascularmetastasis(hypovascularduring

(DDx: hepaticadenoma,hemangioma, portalvenous phase,older patient)
hepatoblastoma, hepatocellular
liverherniation, (6) Intrahepatic
cholangiocarcinoma (lessvascular,
carcinoma) dominant large centralscar, metastases)
Tc-HIDA:
r/ normal/ increaseduptake(40-70%),coldspot (60%)
Tc-99m-taggedRBCs: CARCINOMA
GALLBLADDER
r/ increaseduptakeduringearly phase lncidence: 0.44.6% of biliarytract operations;most
r/ defect relativeto liveron delayedimages commonbiliarycancer(9 x morecommon
MR: than extrahepaticbile duct cancer);6th most
r/ usuallyhomogeneous signalintensityof lesion commongastrointestinal malignancy(after
r/ tt wl: colon,pancreas,stomach,liver,esophagus);
./ iso- to hypointense(94-100%) 3/" of all intestinalneoplasms;6,500deaths/
{ atypicallyhyperintenselesionin 6% year in UnitedStates
{ T2Wl: slightlyhyper-to isointense(94-100%) Demographics: most commonin lsrael,Bolivia,Chile,
{ centralscar northernJapan, New Mexico
r/ hypointense on Tl Wl Ethnicity: NativeAmericans+ HispanicAmericans
r/ hyperintense on T2Wl in75h (dueto vascular (associatedwith increasedprevalenceof
channels+ edema) gallstones)
r/ hypointenseon T2Wl in 25/" (absentor minimal Medianage: 73 years; M:F = 1:3-1:4
edema) 0 85% occur in 6th decadeor later!
CEMR: Riskfactors: increasedbody mass,femalegender,
^/ denseenhancementin arterialphase: postmenopausalstatus,cigarettesmoking,
r/ isointenseduringportalvenousphase chronicSalmonellatyphiinfection,
{ hyperintenseon delayedimages exposureto chemicals(rubber,
{ occasionally prolongedenhancement(dueto automobile, wood finishing,metal
entrapmentof Gd-DTPAby functioninghepatocytes fabricatingindustries)
insidetumorfollowedby 1% excretionintobiliary Associated with:
tree) (1) D i sorderof gal l bl adder:
^/ tate+ prolongedenhancementof centralscar (a) Cholelithiasis in 74-92h
r/ less uptakeof lV superparamagnetic iron oxidethan 0 Gallbladdercarcinomaoccursin only 1'/. of all
surroundingliver(uptakemechanismsimilarto that of patientswith gallstones!
sulfurcolloid) (b) Porcelaingallbladder(in 4-60%): prevalenceof
A ngio: gallbladder carcinomain 10-25% of autopsies
{ discretelymarginatedhypervascularmass (90%)with (c) Chroniccholecystitis
intensecapillaryblush/ hypovascular (10%) (d) Gallbladderpolyp: a polyp>2 cm is likely
{ enlargementof main feedingarterywith centralblood malignant!
supply(= "sPoke-wheel" patternin 33%) (2) Disorderof bileducts:
{ homogeneousparenchymalstain (a) Primarysclerosingcholangitis
r/ decreasedvascularityin centralstellatefibrousscar (b) Congenitalbiliaryanomalies:cysticdilatationof
Rx: (1) Discontinuation of oral contraceptives biliarytree,choledochal cyst,anomalousjunction
(2) Resectionof pedunculatedmass of pancreaticobiliary ducts, low insertionof cystic
(3) Diagnosticexcisionalbiopsyfor extensivetumor duct
(FNHseldomrequiressurgery) (3) lnflammatoryboweldisease(predominantly
Cx: rarelyrupturewith hemoperitoneum(increased ulcerativecolitis,lesscommonin Crohndisease)
incidencein patientson oral contraceptives - 14%) (4) Familialpolyposiscoli
DDx:
(1) Fibrolamellarcarcinoma(scarcalcified,metastases, lesion(68%),intraluminal
Path: diffuselyinfiltrating
retroperitoneal adenopathy,tumor hemorrhage polypoidgrowth(32%)
+ necrosiscausingpain,hypointense scaron T2Wl) : (")
Histo
(2) Hepaticadenoma(10 cm largetumor,symptomatic l'J;;,i:.'.i?#f*'r?'no"n.,,o,,,,
due to propensityfor hemorrhagein 507o,central gallbladderlumen)
scar atypical) intestinaltype (variantof well-differentiated
(3) WeIl-differentiatedhepatocelIular carcinoma (internal adenocarcinoma with intestinalglands)
necrosis+ hemorrhage, vascularinvasion, mucinous(5%,with >50% extracellular
metastases,rim-enhancement of pseudocapsule) muci n)
(4) Giantcavernoushemangioma(largertumor,may signet-ring cell (abundantintracytoplasmic
calcify,globularperipheralenhancement followedby muci n)
centripetalfilling,retentionof contraston delayed clear cell (well-definedcytoplasmic
images,CSF-likebehavioron MRI) borders)
 Disordersof Liver,BiliaryTract,Pancreas,and Spteen 705

(b) rare epithetiatcelttypes: { echogenicfoci = coexistinggallstones/ wall

adenosquamous carcinoma(3%) calcifications / tumoralcalcification
squamouscell carcinoma(1%) { tumorinseparable from liver
small(oat)cell carcinoma(0.5%,highly CT:
aggressive, t paraneoplastic Cushing r/ nypo-/ isoattenuating mass in gallbladder fossa:
syndrome) r/ low-attenuation areas of necrosis
undifferentiated carcinoma r/ areasof enhancement(= viabletumor)
(c) nonepithetial ceil types (2%): r/ subtleextensionbeyondwall of GB
carcinoid,carcinosarcoma, basal cell r/ invasionof tiverwith protrusionof anteriorsurfaceof
carcinoma,lymphoma medialsegmentof left lobe
Modified Nevin Stage: MR:
I mucosaonly (in situ carcinoma) r/ hypointensemass on TlWl + ill-definedearly contrast
ll mucosal+ muscularinvasion enhancement
lll mucosa+ muscularis+ serosa Metastases:in75-77./" at time of diagnosis
lV gallbladder wall + lymphnodes (a) directextension(mostcommonmode): invasionof
V hepatic/ distantmetastases liver(34-65-89%),duodenum(12-157o),colon
. Earlydiagnosisusuallyunsuspecteddue to lack of
(9-15oA),pancreas(G%),stomach,bile duct, right
specificsigns+ symptoms: kidney,abdominalwall
. historyof past GB disease(50%)
Cause: thin GB wall with onlya singlemuscle
. malaise,vomiting,weightfoss
layer+ no substantiallaminapropria
. chronicRUQ pain(5F76%)
+ perimuscular connectivetissue
. obstructivejaundice(35-74%)
continuouswith interlobular connective
. abnormalliverfunctiontests (20-75%)
tissueof liver
. * elevatedu-fetoproteinand CEA
(b) lymphaticspread(2641-75%):
Location: fundus(60%),body (gO%),neck (10%) cystic,pericholedochal, celiac,superiormesenteric,
Growthtypes: foramenof Winslow,paraaorticnodes,superior
r/ mass replacingthe gallbladder(40-65%) + posteriorpancreaticoduodenal
r/ thickeningof GB wail (20-go%)due to submucosal (c) intraperitoneal seeding(common)
spread: (d) hematogenous spread(lesscommon): liver,lung,
r/ focal (59%)/ diffuse(41%)wail thickening bones,heart,pancreas,kidney,adrenal,brain
DDx: acute/ chronicinflammation (usually<10 mm) (e) neuralspread(frequent):associatedwith more
r/ intraluminal polypoid/ fungating.cauliflower-like" aggressivetumors
mass with wide base (15-25%) (f) intraductalspread(leastcommon): particularlyin
r/ replacementof gallbladderby mass (57-70%) papillary adenocarcinoma
r/ pericholecystic infiltration:in l6/"focal, in 24/"diffuse Cx: perforationof gallbladder+ abscessformation
r/ dilatationof bitiarytree (38-70%): r/ gallstoneslocatedwithinabscess
r/ infiltrativetumor growthalongcysticduct Prognosis: 75o/"unresectableat presentation;average
r/ lymphnodeenlargement causingbiliaryobstruction survivalis 6 months;S/o 1-yearsurvivalrate;
{ intraductaltumor spread 6o/"S-yearsurvivalrate
r/ fine granular/ punctateflecksof calcification(mucinous DDx: (1) Xanthogranulomatous cholecystitis(lobulated
adenocarcinoma) mass fiflinggallbladder+ stones)
r/ lymph node enlaigementin porta hepatis (2) Acute/ chroniccholecystitis(generalized
N.B.: misdiagnosis by US / CT in 507o,especiallyin the gal l bl adder w al lthi ckeni ng< 10 mm)
presenceof gallstones (3) Livertumorinvadinggallbladder fossa
Abdominalradiograph: (4) Tumorsfrom adjacentorgans(pancreas,
r/ calcifiedgallstones duodenum)
r/ porcelaingallbladder (5) Metastases(melanoma,leukemia,lymphoma)
_ { RUO gas collection(afterinvasionof adjacentbowel) (6) Polyps:cholesterolpolyp,hyperplastic polyp,
Cholangiography: granulationpolyp
! malignantstricture/ obstructionof extrahepaticbile (7) Adenomyomatosis
ducts/ rightand left bile duct confluence,intrahepatic
, duct of right lobe GLYCOGEN
STORAGEDISEASE
r/ intraluminal GB fillingdefect(= tumor/ stones) = autosomalrecessivediseaseswith varyingseverityand
r/ mass displacing/ invadinggallbladde r clinicalsyndromes
r/ intraductalfillingdefects(= trror / stones) A . V ON GTE R K ED TS E A S (TY
E P Et)
US: Etiology: defect in glucose-G-phosphatase
r/ gallbladderreplacedby mass with irregularmargins with excess
depositionof glycogenin liver,kidney,
, + heterogenousechotexture(= 1grn.rnecrosis) intestines
r/ immobileintraluminal well-deiinedround/ oval mass Dx: failureof rise in bloodglucoseafterglucagon
DDx: tumefactivesludge administration
706 Radiology Review Manual

Age at presentation: infancY Pathophysiology:

r/ hepatomegaly absorbediron is selectively boundto transferrin;
US: increasedtransferrinsaturationin portalcirculation
.l increasedechogenicity(glycogenI fat) favors selectiveiron uptake by periportalhepatocytes
CT : as initialsite of ironaccumulation; RES cellsare
^/ increased(glycogen)/ normal/ decreased(fat) incapableof storingexcessiron
parenchymalattenuation Path: excessiron storedas crystallineiron oxide (ferric
Prognosis; death in infancy,may surviveinto oxyhydroxide) withincytoplasmic ferritin
adulthoodwith earlYtheraPY + lysosomal hemosiderin; iron overload affects
Cx: (1) Hepaticadenoma parenchymal cells(liver,pancreas,heart)NOT
(2) Hepatocellularcarcinoma Kupffercells/ RE cellsof bone marrow+ spleen
B . P O M P EDI S E A SE(T Y PEl l ) (abnormalfunctionof RES)
= abnormalmetabolismwith enlargement of . asymptomatic during1st decadeof disease
myocardialcellsdue to glycogendeposition;similar . hyperpigmentation (90%)
to endocardialf ibroelastosis . hepatomegaly (90%)
Etiology: defectin lysosomalglucosidase . arthralgias(50%)
{ massivecardiomegalywith CHF . diabetesmellitus(30%)secondaryto insulin
{ hepatomegaly resistanceby hepatocytes+ pancreaticB-celldamage
nosis'
Prog j?.'' from irondeposition
::ll::,*?T#;ffi3:;!,ji'l*i" . CHF + arrhythmia(15%)
. lossof libido,impotence,amenorrhea, testicular
beyondinfancy
c. coRl DISEASE(TYPElll) atrophy,lossof bodYhair
D. ANDERSEN DISEASE (TYPEIV) . liveriron index, 2 (= liverironconcentration
E. McARDLE DISEASE (TYPEV) [micromoles per gram of dry weight]per patient'sage)
F. HERSDISEASE (TYPEVl) MR:
(skeletalmuscle= good signalintensityreference)
ri significant signalloss in liveron T2Wl with signal
HEMOCHROMATOSIS intensityequalto backgroundnoise(paramagnetic
= eXc€ss irondeposition in variousparenchymal organs susceptibilityof ferritin+ ferricions leadsto
(liver,pancreas, spleen,kidneys,heart)leadingto profoundshorteningof T1 + T2 relaxationtimes of
cirrhosis withportalhypertension adjacentProtons)
Cause: excessirondePosition from r/ normalpancreaticsignalintensityin noncirrhotics
(a)increased Gl absorPtion: ./ pancreaticsignalintensityequalto / less than
1. Genetic hemochromatosis muscle(in 90% of cirrhoticpatients)
2. Erythropoietic hemochromatosis ^/ normalsignalintensityof spleen(in 86%)due to
3. Bantusiderosis abnormalRES function
(b)lV bloodtransfusion Dx: liverbiopsy
(c) intravascular (extrasplenic) hemolysis Cx: (1) Periportalfibrosisresultingin cirrhosis(if iron
CT (60% sensitivityfor iron): concentration>22,000pg/g of livertissue)
in liverdensity(upto (2) Hepatocellular carcinoma(14-30%)
r/ diffuseI rarelyfocalincrease
75-130HU) (3) Insulin-dependent diabetesmellitus(30-60%)
r/ depiction of portal+ hepaticveinsagainstbackground (4) Congestivecardiomyopathy(15%)
of hyperattenuating liveron NECT Rx: phlebotomies in precirrhotic stage
r/ dualenergyCT (at80 + 120kVp)canquantitate Prognosis.' normallife expectancywith early diagnosis
amountof irondeposition and treatment

SecondaryHemochromatosis
Genetic Hemochromatosis
= IDIOPATHIC / PRIMARY HEMOCHROMATOSIS
= €XC€SSive absorption+ parenchymalretentionof
dietaryiron that favors accumulationwithinnon-RES
organs(liver,pancreas,heart,pituitarygland)
Cause: autosomalrecessivedisorder(human-
leukocyteantigen[HLA]-linked abnormalgene
locatedon short arm of chromosome6) with
mucosaldefectin intestinalwall / increased
absorptionof intestinaliron
Prevalence: 1:22Owhitesof northernEuropean
ancestry;homozygotefrequencyup to
0.25-0.50%;heterozygotecarriers>10o/"
[= HEMOSIDEROSIS = increasedirondepositionwithout
organdamagel
Cause:
(1) Erythrogenic hemochromatosis = increased
absorptionof iron secondaryto erythroid
hyperplasiain ineffectiveerythropoiesis(eg,
thalassemia,NOT in sicklecell anemia)
Path: no excessKuPffercell iron
(2) Bantusiderosis= €XC€ssiVe dietaryiron from food
preparationin iron containers(Kaffirbeer)
(3) Transfusionaliron overlozfl= patientsreceiving
>40 unitsof blood (ironstoragecapacityof RES
= 10 g of i ron)

Path: iron depositioninitiallyin RES (phagocytosisof
intactRBC)with sparingof parenchymalcells of
pancreas;aftersaturationof RES storage
capacityparenchymalcells of other organs
accumulateiron (liver,pancreas,myocardium)
Age: 4th-sth decade; M:F = 16.1
. littleclinicalsignificance
MR:
r/ signalloss in liveron T2Wl with signalintensity
greaterthan backgroundnoise(ironin Kupffercells
, with sparingof parenchymallivercells)
! splenicsignalintensitylessthan muscle
! low signalintensityof bone marrow
HEPATIC
ABSCESS
= localizedcollectionof pus in the liverresultingfrom any
infectiousprocesswith destructionof the hepatic
parenchyma+ stroma
Types: pyogenic(85%),fungal (g/"), amebic(6%)
Location: multiplein S0/"
0 A pyogenicabscesstends to be centrally
located,an amebicabscessperipherallyl
r/ hepatomegaly
{ elevationof righthemidiaphragm
! pleuraleffusion
r/ right lower lobe atelectasis/ infiltration
r/ gas withinabscess(esp. Klebsiella)
MR:
r/ hypointense on TlWl + hyperintense on T2Wl (72%)
i/ perilesionatedema (gl%i
"double
r/ targetsign"on T2Wl = hyperintensecenter
(fluid)+ hypointense sharplymarginatedinnerring
(abscesswall) + hyperintensepoorlymarginatedring
(perilesionaledema)
{ rim enhancement(86%)
AmebicAbscess
Organism; Entamoebahistolytica
Etiology: spreadof viableamebaefrom colonto liver
via portalsystem
lncidence; in 1-25% of intestinalamebiasis
Age: 3rd-5th decade; M:F = 4:1
. amebicdysentery
. amebichepatitis(15%)
Location: liverabscess(rightlobe) in Z-25/";
systemicdissemination by invasionof
lymphatics/ portalsystem (rare);
liv e r:l u n g :b ra=i n10 0 :10 :1
Size: 2-12 cm; multipleliverabscessesin 25./,
r/ nonspecificvariableappearance
{ nodularityof abscesswall (60%)
r/ internalseptations(90%)
\i not gas-containing(unlesshepatobronchial
, hepatoentericfistulapresent)
r/ t disruptionof diaphragm
CT:
{ nonspecifichypoattenuating area
r/ enhancingwall
Disordersof Liver,BiliaryTract,pancreas,and Spteen 707
US:
r/ homogeneoushypoechoicarea
r/ posterioracousticenhancement
{ well-defined smooththin wall
NUC:
r/ sensitivityof sulfurcolloidscan is 9g%
{ photon-deficient area surroundedby rim of uptake
on Ga-67scan
Aspiration:
typicallyopaquereddish/ dirty brown/ pink material
("anchovypaste"/ "chocolatesauce"),usuallysterile,
parasiteconfinedto marginof abscess
Cx: (1) Diaphragmatic disruption(rare)is strongly
suggestiveof amebicabscess
(2) Fistulizationinto colon,rightadrenalgland,
bileducts,pericardium
Rx: conservativetreatmentwith chloroquine/
metronidazole(Flagyl@) ; percutaneousdrainage
for left hepaticabscess(spontaneousruptureinto
pericardium + tamponadepossible)
Prognosis; resolutionunder therapy may take from
1 monthto 2 years;permanentcysts may
remainbehind
PyogenicLiverAbscess
0 Most commontype of liverabscess
Organisms: E. coli, aerobicstreptococci,St. aureus,
anaerobicbacteria(45%)
lncidence; 0.016%
Etiology: (1) Ascendingcholangitisfrom obstructive
biliarytractdisease(malignant / benign)
(2) Portalphlebitis(suppurative appendicitis,
colitis,diverticulardisease)
(3) Infarction from sicklecelli embolism/
postembolization / septicemia
(4) Indwellingarterialcatheters
(5) Directspreadfrom contiguousinfection
(cholecystitis,pepticulcer,subphrenic
sepsis)
(6) Trauma(rupture,penetrating wounds,
biopsy,surgery)
(7) Cryptogenicin 4S/" (invasionof cystsi
dead tissueby pyogenicintestinalflora)
Age: 6th-7th decade; M > F
. pyrexia(79%)
. abdominalpain (68%)
. nocturnalsweating(43%)
. vomiting/ malaise(39%)
. jaundice(0-20%)
. positivebloodculture(S0%)
Location: solitaryabscessin rightlobe (40-75%), in
left lobe (2-10%);muttipleabscessesin 1O-
34-73% (moreoften of biliarythan
hematogenous origin)
/ US:
r/ hypoechoicroundlesionwithwell-defined mildly
echogenicrim
{ posterioracousticenhancement
r/ coarseclumpydebrisi low-levelechoesi fluid-
debrislevel
708 Radiology Review Manual

^/ intenselyechogenicreflectionswith reverberations Size: between6 and 30 cm in size (averagesizeof 8-10

(from gas) in 20-30% cm)
r/ roundwell-circumscribed pseudo-encapsulated mass
CT:
^/ inhomogeneous hypoattenuating (0-45 HU) single/ { intraparenchymal / pedunculated(in 10%)
multiloculated cavitY ./ unusual"nodule-in-nodule" appearancein largetumors
r/ "doubletargetsign" = wall-enhancement (DDx: hepatocellular carcinoma)
+ surroundinghypodensezone (6-30%) { occasionaleccentricdystrophiccalcifications
r/ "clustersign" = SeVelalabnormalfoci withinthe C T:
same anatomicarea;suggestiveof bitiaryorigin r/ round mass of decreaseddensity;areasof necrosis
ri air density (30-40%)
MR: ^/ hyperdenseareasof fresh intratumoralhemorrhage
r/ decreasedT1 signal+ increasedT2 signal (22-50/")
./ enhancement of peripheralrim C E C T:
{ transientenhancement on arterial-phase images(due
NUC:
^/ photon-deficient area on sulfurcolloid+ IDA scan to suPPlYbY hePatic arterY)
r/ Ga-02 citrateuptakein 80% ^/ iso- / hypoattenuating on delayed-phaseimages
^/ tn-t 11 taggedWBC uptakeis highlyspecific(since US:
WBCs normallygo to liver,may need sulfurcolloid { usuallysmallwell-demarcated solidheterogeneous
massof variableechogenicity (echogenic / complex
test for correlation)
hyper-and hYPoechoic):
( 1) S ep ti c e m i a r/ hyperechoic lesionwithwell-defined hypoechoicrim
Cx :
(2) Ruptureinto rightsubphrenicspace r/ anechoiccystic areas if large
(3) RuPtureintoabdominalcavitY MR:
(4) RuPtureintoPericardium ./ inhomogeneous on all pulsesequences
(5) Empyema (indistinguishablefrom HCC)
(6) Commonhepaticduct obstruction { often hyperintense areason TlWl (dueto presenceof
Mortatity: 20-80%;100% if unrecognized/ untreated fat-ladenhepatocytes/ hemorrhage)
r/ isointense(sheetsof hepatocytes)and hyperintense
areas(necrosis,hemorrhage) on T2Wl
HEPATIC ADENOMA
= HEPATOCELLULAR ADENOMA = LIVERCELLADENOMA NUC:
0 The mostfrequenthepatictumorin youngwomenafter r/ focalphotopeniclesionon sulfurcolloidscan
steroids! (becauselesioncomposedof hepatocytes
use of contracePtive
+ nonfunctioning Kupffercells)surroundedby rim of
Prevalence: half as commonas FNH
Path: pseudocapsule due to compression of livertissue increaseduptake(dueto compressionof adjacent
containingmultiplelargevessels;high incidenceof normallivercontainingKupffercells);may show
hemorrhage+ necrosis+ fattychange;no scar uptakeequalto / slightlylessthan liver(23h)
Histo: solitarysphericalbenigngrowthof hepatocytes; r/ usuallyincreasedactivityon HIDAscan
^/ ltlO galliumuptake
sheetsof hepatocyteswithoutportalveins or
centralveins;scatteredthin-walledvascular Angio:
{ usuallyhypervascular mass
c hann e l s+ b i l ec a n a l i c u l i ;d e c re a isnenumber
of abnormallyfunctioningKupffercells; r/ homogeneous but not intensestainin capillaryphase
hepatocytescontainincreasedamountsof { enlargedhepaticarterywith feedersat tumor
glYcogen+ fat periphery(50%)
,1/nypo-/ avascularregions(secondaryto hemorrhage/
Age: youngwomenin childbearing age; not seen in
malesunless on anabolic steroids; rarein children necrosis)
Associatedwith: oral contraceptives(2-5 x risk after rl neovascularity
S-yearuse, 7.5 x riskafter9-yearuse, CAVE: percutaneous biopsycarrieshigh riskof bleeding!
25 x risk>9-yearuse),steroids, Cx: (1) Spontaneoushemorrhagewith subcapsular
hematoma/ hemoPeritoneum (41%)
pregnancy,diabetesmellitus,type la
(2) Malignanttransformation (? contiguous
glycogenstoragedisease(von Gierke)
developmentof hepatocellular carcinoma)
in 607o,Fanconianemia
0 Pregnancymay increasetumor growthrate + lead to (3) Recurrenceafter resection
tumorrupturel Rx: surgicalresection(to preventrupture)
DDx: FNH,hemangioma, hepatocellular carcinoma
0 Tumor regressionmay occurwith dietarytherapyleading
to normalinsulin,glucagon,and serumglucoselevels
. asymptomatic(20%) HEPATIC ANGIOMYOLIPOMA
. RUQ pain as sign of mass effect(40%)/ intratumoralor = rdrebenignmesenchymal tumor
intraperitonealhemorrhage(40%) Associatedwith: tuberoussclerosis
. hepatomegaly Histo: smooth musclecells,fat, proliferatingblood
Location: right lobe of liver in subcapsularlocation(75%) vessels
 Disordersof Liver,BiliaryTract,Pancreas,and Spleen 709

. asymptomatic A ngi o:
rl intratumoral
fat is DIAGNOSTIC r/ hypervascularstain aroundtumor peripheryin late
rl soft-tissue
component mayenhance arterialphasewith puddling;NO arterialencasement
Cx: intratumoral hemorrhage CAVE: Biopsymay leadto massivebleedingin 16%!
Opt for open ratherthan percutaneousbiopsyl
HEPATICANGIOSARCOMA Prognosis; rapiddeteriorationwith mediansurvivalof
= HEMANGIOENDOTHELIAL SARCOMA = KUPFFER CELL 6 months(13 monthsunderchemotherapy)
SARCOMA = HEMANGIOSARCOMA DDx for multiple lesions: metastases
Prevalence:0.14-0.25per million;<2/" of all primaryliver DDx for single lesion: cavernoushemangioma
neoplasms;most commonsarcomaof liver
(followedby fibrosarcoma> malignant
fibrohistiocytoma > leiomyosarcoma) HEPATICCYST
Etiology: (a) thorotrast= thoriumdioxide(7-10%) with 0 Secondmostcommonbenignhepaticlesionafter
latent period of 15-24 years hemangioma
(b) arsenic Prevalence: 2-7"/.; increasingwith age; M < F
(c) polyvinylchloride(latentperiodof A. ACQUIREDHEPATICCYST
4-28 years) secondaryto trauma,inflammation, parasitic
Associatedwith: hemochromatosis,von infestation,neoplasia
Recklinghausen disease B. CONGENITALHEPATICCYST
Path: (a) multifocal/multinodular lesions(71%)of up to = defectivedevelopmentof aberrantintrahepaticbile
>5 cm in size ducts;derivedfrom bile duct hamartoma
(b) largesolitarymass with hemorrhage+ necrosis lncidence: liver cysts detected at autopsy in 50%;
Histo:
in 22/" detectedduringlife
(a) vesselslinedwith malignantendothelial cells Age of detection: sth-8th decade
(eg,sinusoids)causingatrophyof surrounding liver Histo: cysts surroundedby fibrouscapsule+ lined
(b) vasoformative= formingpoorlyorganizedvessels
by columnarepithelium,relatedto bileducts
(c) formingsolid nodulesof malignantspindlecells
withinportaltriads;no communication with
Age: 6th-7th decade;M:F = 4:1
. abdominalpain,weakness,fatigue,weightloss bileducts
Associated with:
. spontaneoushemoperitoneum (27%) (1) Tuberoussclerosis
. jaundice
(2) Polycystickidneydisease(25-33% have liver
. NO elevationof o-fetoprotein
cysts)
Earlymetastasesto: (3) Polycysticliverdisease:autosomaldominant;
lung,spleen(16%),portahepatisnodes,portalvein, M:F = 1:2;(50%havepolycystickidneydisease)
thyroid,peritonealcavity,bone marrow(rapidmetastatic . hepatomegaly (40%);pain (33%);jaundice(9%)
spread) Size of cyst: rangefrom microscopicto huge (average
r/ portalvein invasion
1.2 cm; in 25o/olargestcyst <1 cm; in 40%
rl hemorrhagicascites
largestcyst >4 cm; maximalsize of 20 cm);
P lainf ilm :
multiplecysts spreadthroughoutliver (in
r/ circumferential displacementof residualthorotrast 60%) / solitarycyst
NUC: r/ unilocularsimplecyst:
r/ single/ multiplephotopenicareason sulfurcolloid
r/ imperceptiblewall
scan ./ may show fluid-fluidinterface
r/ increasedgalliumuptake ,
r/ waterattenuation(0-10 HU)
{ perfusionblood pool mismatch(initialdecrease r/ "coldspot"on lDA, Ga-68,Tc-99msulfurcolloidscans
followedby slow increasein RBC concentration)as in Rx: sclerosingtherapywith minocyclinehydrochloride
hemangiomaon 3-phasered bloodcell scan (Dose:1 mg per 1-mLcyst contentup to 500 mg in
US: 10 mL of 0.9%sal i ne+ 10 mL 1% l i docaine)
r/ solid/ mixed mass with anechoicareas (hemorrhage/ followingcontrastopacificationof cyst to confirm
necrosis) absenceof communicationwith biliarylree /
multiple
nodules leakageinto peritonealcavity
CT:
hypodense
masseswithhigh-densityregions
(hemorrhage)
/ low-attenuation
regions(old
hemorrhage / necrosis) HEPATIC HEMANGIOMA
r/ strikingperipheralenhancement
on dynamicCT as in CavernousHemangioma
of Liver
largehemangioma = rTrost
commonbenignlivertumor (78%);secondmost
MR: commonlivertumor after metastases
^/ hypointenseon Tl Wl + hyperintense
on T2Wl lncidence: 1-4/"; autopsyincidence0.4-7.3%;
^/ peripheralGd-pentetateenhancement on TlWl increasedwith multiparity
710 RadiologyReview Manual
Cause:? enlarginghamartomapresentsince birth,
? true vascularneoplasm
Age: rarelyseen in young children; y'f = 1:5
Path: largevascularchannelsfilledwith slowly
circulating blood;linedby singlelayerof mature
flattenedendothelialcells separatedby thin
fibroussepta;no bile ducts;thrombosisof
vascularchannelscommonresultingin fibrosis
+ hemorrhage+ myxomatousdegeneration
+ calcifications
Pathophysiology: large blood volume with low blood
flow
Associatedwith: (1) Hemangiomasin other organs
(2) Focal nodularhyperplasia
(3) Rendu-Osler-Weber disease
' asymptomaticif tumor small (50-70%)
. may presentwith spontaneouslife-threatening
hemorrhageif large (5%)
. hepatomegaly
. may enlargeduringpregnancy
. abdominaldiscomfort+ pain (fromthrombosisin large
hemangioma)
. Kasabach-Merritt syndrome(= hemangioma
+ thrombocytopenia) rare
Location: frequentlyperipheral/ subcapsularin
posteriorright lobe of liver;ZO"hare
pedunculated; multiplein 10-20%
Size: <4 cm (90%);
>4-6-12 cm = giant cavernous hemangioma
r/ btoodsupplyfrom hepaticartery
r/ may have central areaof fibrosis= sfeds of
nonenhancement / cysticspace
/ nonfilling
(occurrenceincreaseswith age)
r/ centralseptalcalcifications withinareasof fibrosis/
phleboliths(5-20%)
US:
{ uniformlyhyperechoic(60-70%) mass due to
multipleinterfacescreatedby blood-filledspaces
separatedby fibrous septa
./ inhomogeneoushypoechoicmass (up to 40%) in
largerhemangiomas withwell-defined thick/ thin
echogeniclobulatedborderdue to hemorrhagic
necrosis,scarring,myxomatouschangecentrally
d homogenous(58-73%)/ heterogeneous(fibrosis,
thrombosis,hemorrhagicnecrosis)
ri hypoechoiccenterpossible
r/ may show acousticenhancemenl(37-77%)
r/ unchangedin size lappearance(82%)on
1-6-yearfollow-up
r/ no Dopplersignals/ signalswith peak velocityof
<50 cm/sec
CT (combinationof precontrastimages,good bolus,
dynamicscanning):
{ well-circumscribed spherical/ ovoidlow-density
MASS:
./ may have areas of higher/ lowerdensitywithin
MASS
{ typicalpatternof low densityon NECT + peripheral
enhancement+ completefill-inon delayedimages
3-30 minutespost lV bolus (55-89%):
r/ peripheral(72%)/ central(in 8%) / diffusedense
(in 8%) enhancement
r/ complete(75%)/ partial(24%) lno (2%) fill-into
isodensityin delaYedPhase
r/ rapidcontrastfilling(16%),more often in small
lesions(in 42% of hemangiomas <1 cm)
DDx: hypervascular tumor (do not remain
hyperattenuating on delayed-phaseimages)
{ centralscar may not enhanceat any time
MR (90-957"accuracY):
i spheroidi ovoid (87%) mass with smoothwell-
definedlobulatedmargins(87%);no capsule
d homogeneousinternalarchitectureif <4 cm,
hypointense internalinhomogeneities if >4 cm (due
to fibrosis)
r/ tt Wt: hypo-/ isointense
^/ tzWt: hyperintense"lightbulb"appearance(dueto
slowflowingblood)increasingwith echotime
(DDx: hepaticcyst, hyper-vascular tumor,necrotic
tumor,cysticneoPlasm)
! same enhancementPatternas CT:
r/ uniformenhancementat 1 secondin 40% of
smallhemangiomas <1.5cm aftergadolinium-
DTPA
{ peripheralnodularenhancement progressing
centripetallywith centrallyuniformenhancement
(50%)i persistenthypointensity(30%)
d mildlyhyperintenseon T2Wl for hyalinized
hemangioma+ lackof enhancement in early
phase+ slightperipheralenhancement in late
phase(DDx: malignanthepatictumor)
Angio (historical goldstandard):
{ dense opacificationof well-circumscribed, dilated,
irregular,punctatevascularlakes/ puddles in late
arterial+ capillaryphasestartingat peripheryin
ring-I C-shapedconfiguration
{ normal-sizedfeeders; AV shunting(very rare)
{ contrastpersistencelate into venousphase
NUC (95%accuracywith SPECT):
lndication: lesions>2 cm (detectablein 70-90%)
./ initiattycold lesionon Tc-99mlabeledRBC scans
(doseof 1$-20 mOi)with increasedactivityon
delayedimagesat 1-2 hours
r/ cold defecton sulfurcolloidscans
Bx: may be biopsiedsafelyprovidednormalliver is
presentbetweentumor + livercapsule
r/ nonpulsatileblood (73%)
r/ endothelialcellswithoutmalignancy(27%)
Prognosis.' no growth when <4 cm in diameter;giant
cavernoushemangiomasmay enlarge
Cx (rare): (1) Spontaneousrupture(4.5%)
(2) Abscessformation
(3) Kasabach-Merritt syndrome(platelet
sequestration)
DDx: hypervascularmalignantneoplasm/ metastasis
(quickhomogeneousfillingduringarterialphase
of smallhemangiomas)
 Disordersof Liver,BiliaryTract,pancreas,and Spteen 7 1 1

GiantHepaticCavernousHemangioma . Kasabach-Merrittsyndrome (in 11%)

= at leastone dimensionexceedingg cm (in literature = hemorrhagic diathesisdue to plateletsequestration
no agreementon size) by tumor/ disseminated intravascularcoagulation;
Associatedwith: coexistentsmaller<5 cm characterizedby an associationof hemangioma,or
h e m a n g i o ma
in 13% hemangioendothelioma, or angiosarcoma with
Histo: hemorrhage,thrombosis,extensive thrombocytopenia and purpura(secondaryto
hyalinization, liquefaction, fibrosis;central increasedsystemicf ibrinolysis)
cleft due to cysticdegeneration/ liquefaction Prognosis: tatal outcome in 20-30%
. RUQ pain/ fullness;abdominalmass . hemolyticanemia
US: Size: severalmm up to 20 cm (averagesize of 3 cm)
r/ heterogeneousmass r/ diffuseinvolvementof entireliver,raiely focal
NE CT : ^/ singlemass (50%)/ multiplemasses(50%)
r/ heterogeneoushypoattenuating mass with marked r/ enlargementof celiac+ hepaticarteries+ proximal
centralareas of low attenuation aorta
CECT: r/ rapiddecreasein aorticcaliberbelowceliactrunk
r/ earlyperipheralglobularenhancement r/ enlargedhepaticveins (increasedvenousflow)
{ incompletefillingof centralportions P l ai nfi l m:
MR: r/ fine speckled lfibrillarycalcificationsin 1 6-25%
r/ sharplymarginatedhypointensemass with clefilike (DDx: hepatoblastoma, hamartoma,metastatic
area of lowerintensityon Tl Wl neuroblastoma)
r/ markedlyhyperintenseclefilikearea with some US:
hypointenseinternalsepta insidea hyperintense r/ heterogeneouspredominantlyhypoechoic/
mass on T2Wl complex/ hyperechoic lesion
r/ cleftlikearea remainshypointense during r/ multiplesonolucentareas(= enlargingvascular
enhancement channelssecondaryto initialrapidgrowth)
DDx: metastasis,hepatocellular carcinoma, (DDx: mesenchymal hamartoma):
cholangiocarcinoma, hepaticadenoma,FNH, { vascularcomponentsdemonstiatedby color
focal fatty infiltration
, Duplex
r/ calcifications(in up to SO%)
Infantile Hemangioendothelioma of Liver OB -U S :
= INFANTILE HEPATIC HEMANGIOMA = CAPILLARY / r/ polyhydramnios + fetal hydrops
CAVERNOUS HEMANGIOMA N E C T:
0 Mostcommonbenignhepatictumorduringfirst6 ./ largewell-definedhypoattenuating mass
monthsof lifel r/ hemorrhage(notuncommon)
Histo: multipleanastomosingthick-walledvascular { calcifications(in up to 16%)
spacessimilarto cavernoushemangiomalined CECT (similarto cavernoushemangioma):
by plumpimmatureendothelial cellsin singleor { early peripheralenhancementilzt"l
(lessoften)multiplecell layers;areasof r/ variabledelayedcentralenhancement
extramedullary hematopoiesis / thrombi; MR:
scatteredbileducts;involutional changes r/ heterogeneous hypointense multinodular lesionon
(infarction,hemorrhage, necrosis,scarring) Tl Wl + hyperintense
, areas of hemorrhage
Classification: { varyingdegreesof hyperintensity on T2Wl
(a) Hemangioendothelioma type 1 (morecommon):
orderlyproliferation , (resembling adulthemangioma)
of smallbloodvessels r/ decreasingsignalintensitywith fibroticreplacement
(b) Hemangioendothelioma type 2: on T2Wl
more aggressivehistologicpattern NUC (sulfurcolloid,taggedRBC):
DDx: angiosarcoma r/ increasedflow in viableportionsof lesionduring
(c) Cavernoushemangioma: angiographic phase
dilatedvascularspaceslinedby flat endothelial r/ increasedactivitymixedwith centralphotopenic
cells areas (hemorrhage,necrosis,fibrosis)on delayed
0 Relationship to adultcavernoushemangioma taggedRBC images
unk no w n ! { photopenicdefecton delayedsulfurcolloidimages
Age at presentation: <6 months in gT/o, during Angio;
1st monthin 33yo,>1 year in S/"; r/ enlarged,tortuousfeedingarteriesand stretched
M : F= 1 : 1 . 4 - 1 : 2 intrahepaticvessels
. abdominalmass secondaryto hepatomegafy
r/ hypervascular tumorwith inhomogeneous stain;
. cutaneoushemangiomas(9-45-97%) occur
with clustersof smallabnormalvessels
multinodular form { poolingof contrastmaterialin sinusoidallakeswith
. may presentwith high-outputCHF secondary
to AV rapidclearingthroughearlydrainingveins(AV
shuntswithintumor(8 -1S-25%) shunti ng)
712 RadiologyReviewManual

Prognosis; rapidgrowthin first 6 monthsfollowedby Viral Markersof HePatitis

tendencyto involutewithin6-8 months;
32-75o/osurvival rate in complicatedcases Virus lesfs lnterpretation
Cx: (1) Congestiveheartfailure
(2) Hemorrhagic diathesis HAV Anti-HAVlgM acute hepatitis(can remain
(3) Obstructivejaundice positivefor >1 year)
(4) Hemoperitoneum (ruptureof tumor) Anti-HAVlgG past hepatitis,lifelongimmunitY
(5) Malignanttransformationinto angiosarcoma acute/ chronicdisease
HBV HBsAg
(rare) acuteinfection(if titer high);
Anti-HBclgM
Rx: (1) No treatmentif asymptomatic chronicinfection(if titer low)
I
(2) Reductionin size with steroids/ radiotherapY pasti recentHBV contact(maYbe
Anti-HBclgG
chemotheraPY only serumindicatorof Past
(3) Embolization infection)
(4) Surgicalresection/ livertransplantation HBe activeviral replication
DDx: (1) Hepatoblastoma (>1 year of age, elevated low / absentreplicativestate
Anti-HBe
cx{etoprotein,more heterogeneous) (typicallypresentin long-
(2) Mesenchymal hamartoma(usually standingHBV carriers)
multilocularcYsticmass) Anti-HBs imunityaftervaccination
(3) Metastaticneuroblastoma(elevated HBV-DNA activeviral replication
catecholamines in urine,adrenalmass,
multiplelivermasses) HCV Anti-HCV past / currentinfection
nonenhancing
R IB A test for variousviral comPonents
HCV-RNA activeviral rePlication
HEPATIC DISEASE
VENOOCCLUSIVE HDV Anti-HDVlgM acute/ chronicinfection
= occlusionof smallcentrilobularveins without Anti-HDVlgG chronicinfection(if titerhigh+ lgM
involvementof major hepaticveins positive);past infectionif titer
Etiotogy: radiationand chemotherapyin bone-marrow + lgM negative)
transplantpatients;bush tea (alkaloid) H D V -R N A activeviral rePlication
consumPtion in Jamaica
HEV Anti-HEVlgM acute hepatitis
{ main hepaticveins+ IVC normal
Anti-HEVlgG past hepatitis
./ bidirectionali reversedportalvenousflow
H E V -R N A viralreplication
{ gallbladder wall thickening

Diseases:
HEPATITIS
Cause: alcohol,medication,viral infection,NASH
(nonalcoholicsteatohePatitis)

Acute Hepatitis US:

. markedlyelevatedAST + ALT
. increasein serum-conjugated bilirubin
r/ hepatomegalyi normalsize of liver
i/ gallbladder wall thickening
r/ lymphadenopathy
CT:
./ periportallow attenuation(lymphedema)
US :
./ diftusedecreasein liverechogenicity
{ increasedbrightnessof portaltriads ("starrysky"
pattern)= centrilobularpatterndue to edema in
hepatocytes(DDx: leukemicinfiltrate,diffuse
lymphomatous involvement,toxicshocksyndrome)
r/ edemaof gallbladderfossa + gallbladderwall
thickening
r/ thickening+ increasein echogenicityof fat within
falciformligament,ligamentumvenosum,porta
hepatis,periportalconnectivetissue
ChronicHepatitis
= processpresentfor at least6 months
r/ increasedliverechogenicitY
{ coarseningof hepaticechotexture
r/ silhouetting/ loss of definitionof portalvenules
= decreasedvisualizationof the walls of the
peripheralPortalveins
^/ ruOsound attenuation
Cx: cirrhosis(10%for hepatitisB; 20-5OhIor
hepatitisC)
Neonatal Hepatitis
Cause:
A. INFECTION:virus,protozoa,spirochete,
toxoplasmosis, rubella,CMV, herpes,hepatitisA/
B , syP hi l i s
B. METABOLIC:alpha-1antitrypsin deficiency,
familialrecurrentcholestasis, errorsof metabolism
(nesidioblastosis = idiopathichyperinsulin
hypoglycemiaof infancY)
C . ID IOP A TH IC
Age: 1-4 weeks of age; M > F
 Disordersof Liver,BiliaryTract,Pancreas,and Spleen 713

Histo: multinucleatedgiant cells with hepatic Metastasesto: lung (frequent)

parenchymal disruption,relativelylittlebile Location: rightlobeof the liver
withinbileduct canaliculi { usuallysolitarymasswith an averagesize of 10-12 cm
US; r/ muftifocal(20%)
{ normal-sized / enlargedliver { coarsecalcifications / osseousmatrix(12-gO%)
^/ increasein parenchyrnalechogenicity US:
./ decreasedvisualizationof perpheralportalveins r/ large heterogeneousechogenicmass,oftenwith
r/ normalbile duct system calcifications,
occasionallycysticareas (necrosis/
r/ gallbladderof normal size lsmall (withdecreasein extramedullary hematopoiesis)
bilevolumein severehepatocellular dysfunction) CT:
^/ decreasein gallbladdersize after milk feeding { hypointense
tumorwith peripheralrim enhancement
(DDx: congenitalbiliaryatresia) MR:
NUC: inhomogeneously hypointense on TlWl with
Technique.'often performedafter pretreatmentwith hyperintense foci (hemorrhage)
phenobarbital(5 mg/kgx 5 days)to { inhomogeneously hyperintense with hypointense
maximizehepaticfunction bands (fibroussepta)on T2Wl
r/ normal/ decreasedhepatictraceraccumulation NUC:
{ prolongedclearanceof tracerfrom blood pool r/ photopenicdefect
{ bowel activitytainU delayedusuallyby 24 hours A ngi o:
(bestseen on lateralview; coveringliveractivity r/ hypervascularmass with dense stain
with leadshieldingis hetpfut) r/ markedneovascularity; NO AV-shunting
r/ gallbladdermay not be visualized r/ vascularlakesmay be present
Prognosis; spontaneousremission r/ avascularareas (secondaryto tumor necrosis)
DDx: biliaryatresia(NO small bowel activity) ./ may show caval involvement(= unpssectable)
Prognosis: 60Y"resectable;75/" mortality;better
RadiationHepatitis prognosisthan hepatoma;betterprognosis
Acute Radiation-i nduced Hepatitis for epithelialtype than mixedtype
Time of onset: 2-6 weeks aftercompletionof DDx: hemangioendothelioma (finegranular
radiationtherapywith dose calcifications),
metastaticneuroblastoma,
>3,500rad (35 Gy) mesenchymal hamartoma,hepatocellular
. abnormalliverfunctiontests carcinoma(>5 years of age, no calcifications)
. rightupperquadrantdiscomfort
{ hepatomegaly HEPATOCELLU LAR CARCINOMA
r/ ascites = HEPATOMA
Prognosis.' complete recoveryin majority = ffiostfrequentprimaryvisceralmalignancyin the world;
80-90% of all primarylivermalignancies;2nd most
Chronic Radiation-induced Hepatitis frequentmalignanthepatictumorin children(39%)after
r/ increasedattenuationin irradiatedparenchyma(no hepatoblastoma
fatty infiltration) lncidence; (a) in industrialized world: 0.2-0.8.h
^/ geographicareasof hypointensity on TlWl (b) in sub-SaharanAfrica,SoutheastAsia,
+ hyperintensity on T2Wl (dueto increasedwater Japan,Greece,ltaly: 5.5-20/"
content) Peak age: (a) industrialized world: Oth-7thdecade;
M:F = 2.5:1;fi brol amel l ar
subtype( in
HEPATOBLASTOMA 3-10%) belowage 40 years
lncidence: 3rd most commonabdominaltumor in (b) high incidenceareas: 30-40 years;
children;most frequentmalignanthepatic M : F= 5 : 1
tumorin infants+ children<3 yearsof age (c) in children: >5 years of age (peakat
lncidenceincreasedwith: hemihypertrophy,Beckwith 12-14 years); M:F = 4:3
syndrome Etiology:
Histo: (a) epithelialtype = smsll cells resembling 1. Cirrhosis(60-90%)
embryonal/fetalliver Latentperiod: 8 monthsto 14 years from onset of
(b) mixedtype = epithelialcells+ mesenchymal cirrhosis
cells(osteoid,cartilaginous, fibroustissue) lncidence of HCC:
Age: <3 years;<18 months(in 50%);peak age between - 44% in macronodular(= postnecrotic)cirrhosis
18 and 24 months;rangefrom newbornto due to hepatitisB virus,alcoholism,
1 5 y e a r s ;M : F= 2 : 1 hemochromatosis
. upperabdominalmass,weightloss,nausea,vomiting - 6% in micronodular cirrhosisdue to alcoholism
. jaundice,pain 0 5% of alcoholiccirrhoticsdevelopHCCI
. precociouspuberty(productionof endocrinesubstances) (a) alcohol (c) cardiac
. persistently+ markedlyelevatedcr-fetoprotein (66%) (b) hemochromatosis (d) biliaryatresia
714 RadiologyReview Manual

2. ChronichepatitisB / C: 12/" developHCC occasionally invasionof bileducts

3. Carcinogens calcificationsin ordinaryHCC (2-9-25%); however,
(a) aflatoxin commonin fibrolamellar (30-40%)and sclerosingHCC
(b) siderosis ./ hepatomegalyand ascites
(c) thorotrast ^/ tumor fatty metamorphosis(2-17o/o)
(d) oral contraceptives/ anabolicandrogens
4. lnbornerrorsof metabolism CT (sensitivity of 63% in cirrhosis,80% withoutcirrhosis):
(a) alpha-1antitrypsindeficiency r/ hypodensemass I rarelyisodense/ hyperdensein
(b) galactosemia fatty liver:
(c) type I glycogenstoragedisease(von Gierke) ^/ dominantmasswith satellitenodules
(d) Wilsondisease ^/ mosaicpattern= multiplenodularareaswith
(e) tyrosinosis differingattenuation on CECT (up to 63%)
mnemonic.' "WHATcauses HCC?" r/ diffuselyinfiltrating
neoplasm
Wilsondisease ^/ encapsulated HCC = circularzone of radiolucency
Hemochromatosis surroundingthe mass (12-32-67%)
Alpha-1-antitrypsindeficiency False-positive: confluentfibrosis, regenerativenodule
Tyrosinosis BiphasicCECT:
Hepatitis' { enhancementduringhepaticarterialphase (80%)
Cirrhosis(alcoholic,biliary,cardiac) ri decreasedattenuationduringportalvenous phase
Carcinogens(aflatoxin,sex hormones,thorotrast) with inhomogeneous areasof contrastaccumulation
r/ isodensityon delayedscans(10%)
Path: soft tumor due to lack of stroma,often hemorrhagic r/ tfrincontrast-enhancing capsule(50%)due to rapid
+ necrotic washout
Histo: HCC cells resemblehepatocytesin appearance r/ wedge-shapedareasof decreasedattenuation
+ structuralpattern(trabecular, pseudoglandular (segmental / lobarperfusiondefectsdue portalvein
= acin?[,compact,scirrhous); occlusionby tumorthrombus)
(a) expansiveencapsulatedHCC: collapsed CT with intraarterialethiodolinjection:
portalveinbranchesat capsule r/ hyperdensemass detectableas smallas 0.5 cm US
(b) infiltrativenonencapsulated HCC: portal (86-99% sensitivity,90-93% specificity,50-94o/"
venulescommunicate with tumoralsinusoids accuracy):
= often invasionof portal+ hepaticveins r/ hyperechoicHCC (13%)due to fatty metamorphosis
or markeddilatationof sinusoids
GROWTHPATTERN: ^/ hypoechoicHCC (26%)due to solidtumor
(a) solitary massive (27-50-59%): ./ UCC of mixedechogenicity (61%)due to
bulk in one (mostoftenright)lobewith satellite nonliquefactive tumornecrosis
nodules ^i Dopplerpeak velocitysignals>250 cm/sec
(b) multicentric smallnodular(15-25/.):
smallfoci of usually<2 cm (up to 5 cm) in both US:
hepaticlobes r/ variableechogenicity
(c) diffusemicroscopic(10-15-26%): r/ calcifications(rare)
tiny indistinctnodulescloselyresemblingcirrhosis MR:
Vascularsupply: hepaticartery,portalvein in 6% r/ hypointense(50%)/ iso- to hyperintense(withfatty
. a-fetoproteinelevatedin 75-90% (DDx: negative metamorphosis) on TlWl
o-fetoprotein in cholangiocarcinoma) ^/ ringsign = well-defined hypointensecapsuleon Tl Wl
. elevatedliverfunctiontests (24-44/"),doublelayerof innerhypointensity (fibrous
. persistentRUQ pain, hepatomegaly,ascites tissue)+ outerhyperintensity(compressed blood
. fever,weightloss,malaise vessels+ bile ducts)on T2Wl in expansivetype of
. Paraneoplastic syndromes: HCC
(a) sexual precocity/ gynecomastia r/ mitdlyhyperintenseon T2Wl
(b) hypercholesterolemia ^/ CO-OfpA enhancementperipherally(21"/")I centrally
(c) erythrocytosis(tumorproduceserythropoietin) (7%) lmixed (10%)/ no enhancement(21%)
(d) hypoglycemia .i improvedlesiondetectabilityafter intravenous
(e) hypercalcemia administration of superparamagneticironoxide
(f) carcinoidsyndrome NUC:
Metastasesto: lung (mostcommon=8o/"),adrenal,lymph r/ Sulfurcolloidscan: singlecold spot (70/"), multiple
nodes,bone defects(15-20%),heterogeneous distribution(10%)
r/ portalvein invasion(25-33-48%) r/ tc-HtOR scan: cold spot / atypicaluptakein 4o/"
r/ arterioportalshunting(4-63%) (delayedimages)
r/ invasionof hepaticvein (16%) I IVC (= Budd-Chiari r/ Gallium-scan:avid accumulation in 70-90% (in 63%
syndrome) greater,in 25h equal,in 12o/oless uptakethan liver)
 Disordersof Liver,BiliaryTract,Pancreas,and Spteen 7 1 5

A ngio: US:
r/ "threadand streaks"= linearparallelvascular { mixedechogenicity(60%)
channelscoursingalong portalvenous radiclesseen ./ centralhyperechoicscar (33-60%)
with portalvenousinvolvement CT:
r/ in differentiatedHCC: enlargedarterialfeeders, { mass of low attenuation
coarseneovascularity, vascularlakes,densetumor ./ enhancementof non-scarportion:
stain,arterioportalshunts r/ prominentheterogeneous enhancementin
r/ in anaplasticHCC: vascularencasement,fine arterial+ portalvenous phase
neovascularity, displacementof vessels+ corkscrew- r/ lesspronouncedenhancement duringequilibrium
likevesselsof cirrhosis phase
Prognosis: >90Y"overall mortality; l7oh resectability ./ delayedenhancementof scar (25%)
rate; 6 monthsaveragesurvivaltime; + pseudocapsule of compressedlivertissue(15%)
30% S-yearsurvivaltime MRI:
Cx: spontaneousrupture(in 8%) r/ large lobulatedmass
Rx: (1) Resection r/ ttwl:
(2) l-131antiferritin
lgc (remissionrate>4To/oupto r/ hypointense
(8G%)/ isointense(14%)
3 years) r/ homogeneous(BO%)/ heterogeneous(ZO%)
DDx: hepatocarcinoma, cholangiocarcinoma, focal r/ tewt:
nodularhyperplasia, hemangioma,hepaticadenoma r/ hyperintense/ heterogeneous(85%)
{ isointense / homogeneous (15%)
r/ hypointensecentralscar on Tl Wl + T2Wl
Fibrolamellar
Carcinomaof Liver A ngi o:
= urcorTlrTlon variantof hepatocellularcarcinoma r/ dense tumor stain
Prevalence: 1-g/" of all HCCs;up to 35% of HCCs in r/ enlargedfeedingarteries
patients<50 years of age { NO arteriovenous/ arterioportalshunting
Age: 5-69 (mean23) years;mostly2nd-3rd decade; r/ avascularcentralscar
M : F= 1 : 1 NUC:
Path: largewell-circumscribed lobulated r/ photopenicdefecton sulfurcolloidscan
nonencapsulated strikinglydesmoplastic tumor r/ increasedactivityduringarterialphase + photopenic
with calcifications+ fibrouscentralscar duringdelayedimagingon labeledRBCscan
Histo: large hepatocyte-like cells with granular Prognosis: 48ohresectabilityrate; 32 months average
eosinophilic cytoplasmgrowingin sheets/ survivaltime;67o/o S-yearsurvivaltime
cords/ trabeculaeseparatedby broad bands DDx: focal nodularhyperplasia(young+ middle-aged
of fibrousstromaarrangedin parallellamellae women,<5 cm in size,calcifications uncommon,
resultingin compartmentalized appearance isointenseto liveron all CT + MR imageswith
Riskfactors: NONE known;underlyingcirrhosisor pronouncedhomogeneous enhancement during
hepatitisin <So/o arterialphase,hyperintensecentralscar on
Demographics:less commonin Europe;rare in Japan T2Wl, uptakeof sulfurcolloid/ super
+ China paramagneticiron oxide)
. pain, cachexia;palpableRUe mass, hepatomegaly
. gynecomastia(rare)from conversionof androgensto HYPERPLASTIC CHOLECYSTOSIS
estrogensby tumor-elaborated enzymearomatase = varietyof degenerative+ proliferativechangesof
. jaundice(5%) from biliarycompression gallbladderwall characterizedby hyperconcentration,
. cr-fetoprotein usuallynegative/ mildlyelevatedto hyperexcitability,
and hyperexcretion
<200 ng/pL(in up to 10%) lncidence; 30-50% of all cholecystectomy specimens;
. transaminase levels<100 lU/L M:F=1:6
r/ partially/ completelyencapsulatedsolitarymass (in
80-e0%): Adenomyomatosis
of Gallbladder
r/ intrahepatic(S0%)/ pedunculated(2Oo/o) = increasein number+ heightof mucosalfolds
{ 5-20 (mean13) cm in diameter Histo: hyperplasia of epithelial+ muscularelements
r/ prominentcentralfibrousscar (45-60%) with mucosaloutpouching of epithelium-lined
{ capsularretraction(10%) cysticspacesinto (46%)or all the way through
{ punctate/ nodular/ stellatecalcificationslocated (30%)a thickenedmuscularlayeras tubules/
, withinscar (33-55%) crypts/ saccules(= intramuraldiverticula
r/ intratumoralhemorrhage+ necrosis(10%) = Rokitansky-Aschoff sinus);developwith
r/ vascularinvasion(<5*) increasingage
r/ mass + smallperipheralsatellitelesions(10-1S%) lncidence: 5o/oof all cholecystectomies
r/ diffusemultifocalmasses(<1%)
Age: >35 years; M:F = 1:3
r/ regionaladenopathy(50-70%): porta hepatis Associatedwith: (1) Gallstonesin 25-75%
r/ distantmetastases(20%): lung, peritonealimplants (2) Cholesterolosis in 33./"
716 Radiology Review Manual

(a) generalized form = ADENOMYOMATOSIS IN S P IS S A TE D B ILE S Y N D R OME

{ "pearlnecklace gallbladder"= tinYextraluminal cause of jaundicein neonate
= UfrcorT'trTlon
extensionsof contraston OCG (enhancedafter Associatedwith: massivehemolysis(Rh incompatibility)'
contraction) hemorrhage(intraabdominal,
r/ "comet-tail"= sound reverberationartifactbetween increased
intracranial,retroperitoneal),
cholesterolcrystalsin Rokitansky-Aschoff sinuses enterohepatic (Hirschsprung
circulation
(PATHOGNOMONIC) disease,intestinalatresia,stenosis)
(b) segmentalform US:
compartmentalization most often in neck/ distal 1i3 r/ sludgein gallbladder
(c) localizedform in fundus= ADENOMYOMA r/ sludgewithinbileducts+ partial/complete
r/ smoothsessilemass in GB fundus obstruction(affectedducts may blendwith
= solitaryadenomyoma+ extraluminal diverticula- surroundinghePaticParenchYma)
likeformation
(d) annularform
r/ "hourglass"configurationof GB with transverse TUMOROF
MUCINOUS
PAPILLARY
INTRADUCTAL
congenitalseptum PANCREAS
= IPMT= MUCINOUS DUCTAL ECTASIA = DUCTECTATIC
Cholesterolosis MUCINOUS CYSTIC TUMOROF PANCREAS
= abnormaldepositsof cholesterolestersin = INTRADUCTAL MUCIN.HYPERSECRETING NEOPLASM
= MU C TN _pR OD U C pAtNNGC R E A TTC
TU MOR= M UCTNO US
macrophages withinlaminapropria(foamcells)+ in
VILLOUS ADENOMATOSIS
mucosalepithelium
= taLteintraductaltumororiginating from epitheliallining
1. STRAWBERRYGALLBLADDER
= LIPIDCHOLECYSTITIS = CHOLESTEROSIS typifiedby voluminousmucinsecretions
= planarform = seedlikepatchy/ diffuse Path: conglomerationof communicatingcysts coveredby
a rim of normalpancreaticparenchyma+ thin
thickeningof the villoussurfacepattern
fibrouscapsule
(disseminated micronodules)
Histo: cysts representa dilatedduct linedwith
Associatedwith: cholesterolstones in 50-70h /
. not relatedto serumcholesterollevel innumerable papillaecoatedwith hyperplastic
atypical/ malignantepithelium(adenoma-
{ radiologically not demonstrable
carcinomasequence)
2. CHOLESTEROLPOLYP(90%) . recurrentepisodesof dull pain i acute pancreatitis(due
= polypoidform
= abnormaldepositof cholesterolester producing to impairedoutflowof pancreaticsecretions)
. viscosityof fluidgreaterthan normalserum(89%
a villouslikestructurecoveredwith a singlelayer
sensitive,100% sPecific)
of epitheliumand attachedvia a delicatestalk
Prognosis; low-grademalignancywith betterprognosis
Prevalence: 4%; most commonfixedfilling
than Pancreaticadenocarcinoma
defectof gallbladder
Rx: Whippleoperation(mainduct IPMT/ partial
Location: commonlyin middle113of gallbladder
pancreatectomy(branchduct IPMT)
r/ multiplesmallfillingdefects<10 mm in diameter
DDx: chronicobstructivepancreatitis,serous/ mucinous
DDx: papilloma,adenopapilloma, inflammatory
g ra n u l o m a cystictumors,PseudocYst

MainDuctIPMT
Age: 57 (range,34-75)Years; M:F = 1:1
^/ hyperechoic, hyperdense,T2-hypointense filling
defectwithindilatedduct (= enhancingpapillarymural
nodule/ gravity-dependent mucinglob)
ri dilatationof main pancreaticduct:
(a) dilatationof entiremain pancreaticduct
Main Duct IPMT ./ homogeneous hypoechoic, hypodense,
T1-hypointense and T2-hyperintense mainduct
SegmentalInvolvement Diffuse Involvement with
Branch Duct Dilatation r/ pancreaticparenchymalatrophy
r/ dilatationof branchducts (usuallyin pancreatic
tail + uncinateProcess)
^/ ditatationof major+ minorpapillabulginginto
duodenall umen
r/ + obstructionof CBD (due to tumor/ impacted
Branch Duct IPMT muci n)
Cx: pancreato-biliary / -duodenalfistula,
Microcvstic Pattern Macrocvstic Pattern pseudomyxoma peritonei
lntraductal Papillary Mucinous Ttrmor (IPMT) DDx: chronicobstructivepancreatitis
 Disorders of Liver, Biliary Tract; pancreas, and Spleen 717

(b) segmentaldilatationof main pancreaticduct US:

r/ cyst in pancreaticbody / tail + normalremaining ^/ echogenicmass
pancreaticparenchyma r/ strikingacousticrefraction(soundvelocityin soft
r/ cyst in pancreatichead + upstreamdilatationof tissue1,540m/sec,in fat 1,450m/sec)
main pancreaticduct Prognosis.' nomalignantpotential
DDx: peripheralmucinouscystictumor(main
duct almostalwaysnormal)
ERCP:
. thickjellylikemucusprotrudingfrom a bulging
LIVER TRANSPLANT
patulousduodenalpapilla lndication:
r/ pluggingof the papittaof Vater
chronicviralhepatitis:chronicactivehepatitis(4%in
r/ amorphousintraluminal fillingdefectsin main childhood)
pancreaticduct metabolicdisease:alpha-1antitrypsin deficiency
r/ usuallysmallmuralpolypoid/ flat tumor
(9% in childhood),hemochromatosis, Wilsondisease
r/ dilatedmain + branchpancreaticductswithout
cholestaticliverdisease:primarybiliarycirrhosis,
obstructiveductalstricture primarysclerosingcholangitis, biliaryatresia(52%in
N.B.: refluxof contrastmaterialdue to excessof childhood)
mucin/ patentpapillaryorificehindersfillingof autoimmunehepatitis
ductaltree cryptogenic cirrhosis(6% in childhood)
alcoholicliverdisease
BranchDuctIPMT acutefulminanthepaticfailure(11%in childhood):
Age: 63 (range,g7-76) years; M:F=1:1 viralhepatitis,drug-induced hepatitis(eg,by
. usuallyincidentalfindingwhen tumorsmall
acetaminophen, isoniazid),
hepatotoxins
. symptomsmimickingacute/ chronicpancreatitis
(eg, mushrooms)
Location: mainlyin uncinateprocess>> pancreatic Contraindications: AIDS, extrahepaticmalignant
tail > pancreaticbody tumors,activeIVDAi alcoholabuse
Path: macrocystic/ microcysticpattern;malignancy
suggestedby irregularthick wall + septa and NORMALPOSTTRANSPLANT FINDINGS
solidnodules (1) Periportaledema(21%)
r/ roundi ovoid small lobulatedintraductalmass Cause: lymphedemainearlyposttransplantation
(frequently not visualized): period(= dilatationof lymphaticchannels
{ dilatedmain pancreaticduct due to lackof normallymphaticdrainage)
r/ normalmain pancreaticduct (almostalwaysnormal r/ "periportalcollar"of low attenuationon CT
in s m allt u mo r)
0 Secretinadministration , + hyperechogenicity on US
distendsducts and ri resolutionwithinweeksto months
enhancesdetectionof communication with main (2) Fluidcollectionaroundfalciformligament(11"/o),at
, pancreatic duct! vascularanastomoses(liverhilum,IVC),biliary
! uni-/ multilocular cyst 10-20mm largewith sparse anastomosis,lessersac
septa (3) Smallrightpleuraleffusion
DDx: mucinouscystadenoma(no communication (4) Peri-/ subhepatichematoma/ free intraabdominal
with mainpancreaticduct);pseudocyst(no fl ui d
intraluminal fillingdefects)
r/ multiplethin septaseparatingfluid-filled lacunae
DDx: serouscystadenoma(no communication with VascularComplications
in LiverTransplant(9%)
main pancreaticduct) 0 Most freguentcause of graft loss
*..u"re pancreaticatrophy . liverfailure,bile leak,abdominalbleeding,septicemia
I
I protrusionof papillaintoduodenum 1. Anastomoticnarrowingof IVC/ portalvein
ERCP: 0 Discrepancies in caliberbetweendonor+ recipient
r/ contrastspillsfrom main duct into cysticallydilated vesselhave no pathologicsignificance!
branchducts . venous hypertensionof lower part of body
r/ elongatedband-/ threadlikeor nodularfilling . portalhypertension
defectsin dilatedducts (= depictionof mucin) r/ narrowingof portalvein + poststenoticdilatation
Cx: seedingto mainpancreaticduct resultingin main r/ 3-4-fold velocityincreasecomparedwith
duct IPMT prestenoticsegment
2. Thrombosis/ stenosisof portalvein (1-3%)
L IPO M A O F LI V E R Cause: faultysurgicaltechnique,vessel
Extremelyrare misalignment, differencesin vesselcaliber
. asymptomatic
creatingturbulentflow,hypercoagulable
May be associatedwith: tuberoussclerosis state,prior portalvein surgery,prior
Si z e: f ew m m - 1 3 c m thrombosisin recipientportalvein
718 RadiologyReview Manual

. portalhypertension,liverfailure,massiveascites, in LiverTransplant(6-34%)
BiliaryComplications
edema 0 Secondmost commoncause of liverdysfunctionafter
r/ titlingdefect/ focal narrowingat anastamosis rejection
Rx: percutaneous transluminal angioplasty+ stent Time of onset: withinfirst 3 months
placement,surgicalthfombectomy, venous 1. Biliaryobstruction
jump graft,creationof portosystemicshunt, (a) anastomoticstricture(extrahepatic)
retransplantation Cause: iatrogenictraumaresultingin ischemia
3 . Thrombosis/ stenosisof IVC (<1"/") + scar formation
. pleuraleffusions,hepatomegaly,ascites,extremity (b) nonanastomotic(intrahepatic) stricture
edema Cause: hepaticarterialthrombosis/ stenosis
r/ compressionof IVC (due to swellingof graft) (in 50%), prolongedpreservationtime,
r/ size discrepancybetweendonor + recipientIVC bacterialI vual cholangitis,rejection,
4. Hepaticarterystenosis(5-13%) recurrentprimarysclerosing
Location: at / near anastomoticsite cholangitis,cholangiocarcinoma,
Time of onset: within3 months kinkingof redundantCBD, sphincterof
r/ markedfocal increasein velocity Oddi dysfunction
>200-300 cm/sec+ poststenoticturbulence(in (c) tensionmucoceleof allograftcysticduct remnant
>50/" stenosis) Cause: ligationof cysticduct proximally
r/ intrahepatictarduset parvuswaveform= slowed + distally
systolicaccelerationtime (SAT >0.08sec) distalto r/ extrinsicmasscompressing CHD
stenosis(73% sensitive) ^/ ttuiOcollectionadjacentto CHD
./ diminishedpulsatility(Rl <0.5)due to ischemia Cx: ascendingcholangitis
DDx: normalin early post-transplantation period 2. Bileleak
r/ biliarydilatation(dueto stricture),infarction,biloma (a) T-tubeexitsite: 50% within10 days
Rx: revascularization surgery,balloonangioplasty (b) anastomoticsite of choledochocholedochostomy:
5 . Hepaticarterythrombosis(3-9-16% in adults, 70% within1st month
9-1942/" in children) (c) bile duct necrosis(hepaticarteryocclusion)
Riskfactors: significantcaliberdifferencebetween 0 The intrahepatic biliaryepitheliumis perfused
donor + recipientartery,preexisting solelyby the hePatic arterY!
celiacarterystenosis,prolongedcold (d) after liverbiopsy
ischemiaof donorliver,ABO blood (e) commonhepaticduct leak
rejection
type incompatibility, lncidence: 4.3-23%
Time of onset: usuallywithinfirst2 months 3. Stone/ sludgeformation
. Threetypes of clinicalpresentation: Cause: alterationin bile composition
(1) fulminanthepaticnecrosis+ rapiddeterioration
(2) bile leak,bile peritonitis,
bacteremia, sepsis LYMPHOMA OF LIVER
(3) relapsingbacteremia A. PRIMARYLYMPHOMA(rare)
r/ absenceof hepaticarteryflow { solidsolitarymass
False-positive DoppIer (10'/") : B. SECONDARYLYMPHOMA(common)
low flow state,smallvesselsize, severeliver autopticincidenceof liver involvement:
edema (in first 72 hours aftertransplantation, 60% in Hodgkindisease
viral hepatitis,rejection) 50% in non-Hodgkin lYmPhoma
False-negativeDoppler: arterialcollaterals Pattern:
{ muftiplehypoechoiclesionsinsliver periphery (a) infiltrativediffuse(mostcommon): no alterationin
(= infarcts) hepaticarchitecture
Mortality: 27-58% imaging
(b) focal nodular: detectableby cross-sectional
6. Hepaticarterypseudoaneurysm(uncommon) (c) combinationof diffuse+ nodular(3%)
Location: at vascularanastomosis Detectionrate (forCT, MRI): <10/o
Cx: massiveintraperitonealhemorrhage,portal
veinfistula,biliaryfistula ADENOMAOF PANCREAS
MACROCYSTIC
Rx: surgicalresection,embolization, exclusionby = MUCINOUS CYSTIC NEOPLASM = MUCINOUS
stent olacement CYSTADENOMA / CYSTADENOCARCINOMA
= thick-walled low-grademalignant
uni-/ multilocular
Parenchymal in LiverTransplant
Complications tumorcomposedof largemucin-containing cystic
1. Rejection spaces
0 Can ONLY be diagnosedwith liverbiopsy! Frequency: 10'/"of pancreaticcysts; 1"/"of pancreatic
2. Infarction(10%) neoplasms
{ may calcify Mean age: 50 years (range of 20-95 years); in 50%
^/ may liquefydevelopinginto intrahepaticbiloma between40-60 Years;
3. Graft infection M : F= 1: 1 9
 Disorders of Liver, Biliary Tract, Pancreas, and Spleen 719

Path: largesmoothround/ lobulatedmultiloculated cystic (2) Lymphangioma i hemangioma

mass encapsulatedby a layerof fibrousconnective (3) Variantsof ductaladenocarcinoma:
tissue (a) mucinouscolloidadenocarcinoma / ductectatic
Histo: similarto biliaryand ovarianmucinoustumors; mucinoustumor of pancreas= rT'tUCin-
cystslinedby tall columnar,mucin-producing hypersecreting carcinoma
cellssubtendedby a denselycellular (b) papillaryintraductaladenocarcinoma
mesenchymalstroma(reminiscentof ovarian (c) adenosquamous carcinoma:squamous
stroma),oftenin papillaryarrangement, lackof componentpredisposesto necrosis+ cystic
cellularglycogen degeneration
(a) mucinouscystadenoma (d) anaplasticadenocarcinoma:lymphadenopathy
(b) mucinous cystadenocarci o = stratified
norT'l + metastasesat time of presentation
papillaryepithelium (a) Solidand cysticpapillaryepithelioidneoplasm:
0 All mucinouscysticneoplasmsshouldbe hemorrhagiccysticchangesin 20%
consideredas malignantneoplasmsof (5) Cysticisletcelltumor: hypervascular component
low-grademalignantpotential (6) Cysticmetastases:historyof malignantdisease
(7) Atypicalserouscystadenoma:smallertumorwith
Location: often in pancreatictail (90%) lbody, greaternumberof smaflercysts
infrequentlyin head (8) Sarcoma
. asymptomatic (9) Infection:amebiasis,Echinococcus multilocularis
, abdominalpain, anorexia

r/ well-demarcated thick-walledmass of
2-36 (mean10-12)cm in diameter
r/ multi-/ unilocularlargecysts>2 cm with thin septa MESENCHYMAL HAMARTOMA OF LIVER
<2 mm: = r'?f€developmentalcysticlivertumor
0 A tumor with <6 cysts of >2 cm in diameteris in Histo: disorderedarrangement of primitivefluid-filled
93-95% a mucinouscysticneoplasm! mesenchyme,bile ducts,hepaticparenchyma;
r/ solid papillaryexcrescencesprotrudeinto the interiorof stromal/ cysticpredominancewith cysts of a few
tumor(signof malignancy) mm up to 14 cm i n si ze;no capsul e
r/ amorphousdiscontinuousperipheralmuralcalcifications Age peak: 15-24 months(rangefrom newbornto
( 10- 15%) 1 9 y e a r s ) ;M : F= 2 : 1
r/ hypovascularmass with sparseneovascularity . slow progressive abdominalenlargement
r/ vascularencasementand splenicvein occlusionmay be . + respiratorydistressand lowerextremityedema
present Location: rightlobe:leftlobe= 6:1; 20h pedunculated
r/ great propensityfor invasionof adjacentorgans Size: 5-29 (mean16) cm
US: r/ grosslydiscerniblecystsin 80%
r/ cysts may containlow-levelechoes US:
CT: { multipleroundedcysticareason an echogenic
r/ internalseptationsmay not be visualizedwithout background
contrastenhancement ^/ may appearsolid in youngerinfant(whencysts are
r/ cysts with attenuationvaluesof water; may have stillsmall)
differentlevelsof attenuationwithindifferentcystic CT:
cavities r/ multiplelucenciesof variablesize + attenuation
./ enhancementof cyst walls (dependingon compositionof stromalversuscystic
A ngio: elements)
r/ predominantlyavascularmass r/ hemorrhage(rare)
d cyst wall + solidcomponentsmay demonstratesmall r/ enhancement of stromalcomponent
areasof vascularblush+ neovascularity MR:
r/ displacementof surroundingarteries+ veins by cysts ri varyingsignalintensity(varyingconcentrationsof
Metastases: proteinin cysticpredominance type)/ hypointense on
r/ roundthick-walledcysticlesionsin liver T1Wl (mesenchymal predominance type)
Prognosis; invariabletransformationinto r/ markedhyperintensity of cysticlocules/ hypointense
cystadenocarcinoma fibrosison T2Wl
Rx: completesurgicalexcision(S-yearsurvivalrate of NUC:
74-90%\ ^/ one / moreareasof diminisheduptakeon sulfur
colloidscan
DDx: A ngi o:
(1) Pseudocyst:inflammatory changesin peripancreatic ri hypovascularmass
fat, pancreaticcalcifications,
temporalevolution, ^/ may show patchyareas of neovascularity
historyof alcoholism,elevatedlevelsof amylase r/ enlargedirregulartortuousfeedingvessels
720 Radiology Review Manual

TO GALLBLADDER
METASTASES (1) CT arteriography= angiographycatheterin hepatic
Organ of origin: artery,detectslesionsby virtueof increased
melanoma,renalcell carcinoma(latein courseof enhancement
disease),lymphoma(in AIDS),malignantfibrous (2) CT arterialportography= angiographycatheterin
histiocytoma SMA, detectshypodenselesionson a backgroundof
- in children: embryonalcell sarcoma, increasedenhancementof normalsurroundingsin
rhabdomyosarcoma portalvenousphase
CT-delayediodinescanning:
TO LIVER = CT performed4-6 hoursfollowingadministration of
METASTASES
0 Most commonmalignantlesionof the liver 60 mg iodineresultsin detectionof additionallesions
lncidence: the liver is the most common metastaticsite in 27"/"
afterregionallymphnodes;incidenceof Rx: Exclusioncriteriafor metastasectomy:
metastaticcarcinomais 20 x greaterthan (1) advancedstageof primarytumor
primarycarcinoma;metastasesrepresent (2\ "4 metastases
22/" of all livertumorsin patientswith (3) extrahepaticdisease
knownmalignancy (4) <30% normallivertissue/ functionavailable
Organ of origin: colon (42%), stomach (23%), pancreas after resection
( 2 1 % ) b, r e a s (t 1 4 ) , l u n g( 1 3 % )
r/ involvementof liver+ spleentypicalin leukemia/
lymphoma+ melanoma CalcifiedLiver Metastases
lN CHI LDRE N : n e u ro b l a s to ma W,i l mstu mo r lncidence: 2-3%
. hepatomegaly(70%) 1. Mucinouscarcinomaof Gl tract(colon,rectum,
. abnormalliverenzymes(50-75%) stomach)
Location: both lobes(77%),rightlobe (20/"\,left lobe (3%) 2. Endocrinepancreaticcarcinoma
Number: multiple(50-98%),solitary(2%) 3. Leiomyosarcoma, osteosarcoma
S iz e: >33"/"smallerthan 2 cm 4. Malignantmelanoma
Enhancementcharacteristics comparedwith normalliver: 5. Papillaryserousovariancystadenocarcinoma
r/ lesionenhancementduringarterialphase 6. Lymphoma
(metastasesare suppliedby hepaticartery) 7. Pleuralmesothelioma
r/ less enhancementduringportalvenousphase 8. Neuroblastoma
(metastaseshave a negligibleportalvenoussupply) 9. Breastcancer
r/ extracellularspace agentsaccumulatemore in tumor 10. Medullarycarcinomaof the thyroid
tissue (metastaseshave a largerinterstitialspace) 11. R enalcel lcarci noma
NUC: 80-95% sensitivityin lesions>1.5cm; lesions 12. Lungcarci noma
<1.5cm are frequentlymissed;sensitivity 13. Testicularcarcinoma
increaseswith metastaticdepositsize, peripheral "COBS"
location,and use of SPECT mnemonicfor mucinousadenocarcinoma:
NECT: importantfor hypervascular tumors(eg, renalcell Coloncarcinoma
carcinoma,carcinoid,isletcell tumors),which Ovariancarcinoma
may be obscuredby CECT Breastcarcinoma
CECT: Stomachcarcinoma
Technique:
optimalis bolustechniquewith dynamicincremental
scanning;sensitivityis decreasedrelativeto NCCT if HypervascularLiver Metastases
scansare obtainedduringequilibriumphaseof 1.Renalcell carcinoma
contrastadministration 2.Carcinoidtumor
rl circumferential bead-or bandlikeenhancement during Pancreaticisletcelltumor
3.
arterialphase+ peripheralwashout on delayedimages 4.Mel anoma
r/ no (35%),peripheral(37%),mixed (20%),central 5.Thyroidcancer
(8%)enhancement 6.Choriocarcinoma
rl completeisodensefill-inon delayedscansin 5/. 7.Ovariancystadenocarcinoma
(DDx: hemangioma) L Sarcomas
0 CT-sensitivity 88-90%; specificity99/"; lesionsof L Pheochromocytoma
approx.1 cm can usuallybe detected!
CT-Angiography (mostsensitiveimagingmodality): mnemonic.' "CHlMP"
lndication: patientswith potentiallyresectableisolated Carcinoid
liver metastasesi preoperativeto partial Hypernephroma
hepatectomyfor detectionof additional lsletcell carcinoma
metastases(additionallesionsdetectedin Melanoma
40-55%) Pheochromocytoma
 Disordersof Liver,BiliaryTract,Pancreas,and Spteen 721

HypovascularLiver Metastases METASTASES TO PANCREAS

1. Stomach Frequency; 3-10% (autopsy)
2. Colon Organof origin: renalcell carcinoma(30%),bronchogenic
3. Pancreas carcinoma(23/"), breastcarcinoma
4. Lung (12/"),soft-tissue
sarcoma(B%),colonic
5. Breast
(78%)
itary
{ sol /.trfriil,T?,'i3'
J:,::}"#3.f
flh
discretesmoothmargins
HemorrhagicLiver Metastases ./ ditfusepancreaticenlargement(5%)
mnemonic.' "CT BeOomesMR" CECT:
Coloncarcinoma r/ heterogeneously (60%)/ homogeneously (17%)
Thyroidcarcinoma hyperattenuatingrelativeto pancreas
Breastcarcinoma r/ hypoattenuatingrelativeto pancreas(20%)
Choriocarcinoma r/ isoattenuating
relativeto pancreas(5%)
Melanoma Concomitantintraabdominalmetastasesto:
Renalc ellc a rc i n o m a liver(36%),lymphnodes(30%),adrenalglands(30%)
DDx: ductalpancreaticadenocarcinoma(uniformly
nonenhancing mass,encasementof vessels)
EchogenicLiver Metastases
lncidence: ZS%
1. Coloniccarcinoma(mucinousadenocarcinoma)
54%
2. Hepatoma25% MICROCYSTIC ADENOMA OF PANCREAS
3. Treatedbreastcarcinoma217o = SEROUS CYSTADENOMA = GLYCOGEN-RICH
CYSTADENOMA
= benignlobulatedneoplasmcomposedof innumerable
Liver Metastasesof MixedEchogenicity smallcysts(1-20 mm) containingproteinaceous fluid
lncidence: 375% separatedby thin connectivetissuesepta
1. Breastcancer 31% lncidencei approximately50% of all cysticpancreatic
2. Rectalcancer 20% neoplasms
3. Lungcancer 17% Histo: cyst walls linedby cuboidal/ flat glycogen-rich
4. Stomachcancer 14% epithelialcellsderivedfrom centroacinar cellsof
5. Anaplasticcancer 11% pancreas(DDx:lymphangioma), thinfibrous
6. Cervical cancer S% pseudocapsule
7. Carcinoid 1% Age: 34-88 years;mean age 65 years;
82/" over 60 years of age; M:F = 1:4
Associatedwith: von Hippel-Lindausyndrome
Cystic Liver Metastases . pain,weightloss,jaundice
1.Mucinousovariancarcinoma . palpablemass
2.Coloniccarcinoma Location: any part of pancreasaffected,slight
3.Sarcoma , predominance for head
4.Melanoma r/ well-demarcatedlobulatedmass 1-25 (mean5) cm in
5.Lungcarcinoma diameterwith smoothi nodularcontour
6.Carcinoidtumor r/ innumerable small<2 cm cysts;uncommonlyfew large
cysts (in <5%) / cyst up to B cm in diameter
mnemonic.' "LC GOES" r/ prominentcentralstellatescar (CHARACTERISTIC)
Leiomyosarcoma(and other sarcomas) r/ amorphouscentralcalcifications (in 33% on plainfilm)in
Choriocarcinoma dystrophicarea of stellatecentralscar ("sunburst")
Gastriccarcinoma { pancreaticduct + CBD may be displaced,encased,or
Ovariancarcinoma obstructed
Endometrialcarcinoma US:
Smallcellcarcinoma r/ solidpredominantly echogenicmasswith mixed
hypoechoic+ echogenicareas
CT:
EchopenicLiver Metastases r/ attenuationvaluescloseto water
lncidence: 37.5./o ^/ contrastenhancement
1. Lymphoma 44% A ngi o:
2. Pancreas 36% r/ hypervascularmass with dilatedfeedingarteries,
3. Cervicalcancer 20% densetumorblush,prominentdrainingveins,
4. Lung (adenocarcinoma) neovascularity, occasionalAV shunting,NO vascular
5. Nasopharyngeal cancer encasement
722 Radiology Review Manual

MR: Histo: clusterof proliferatedbile ducts linedby single

r/ delayedenhancementof scar on contrast-enhanced layerof cuboidalcellsembeddedin
FLASHimages fibrocollagenous tissuewith singleramified
Prognosis.' nomalignantpotential lumen,communication with biliarysystem
Rx: surgicalexcisioni follow-upexaminations usuallyobliterated
Associatedwith: polycysticliverdisease
MILKOF CALCIUM
BILE S i z e : 0 . 1 - 1 0m m
= LIMYBILE= CALCIUM SOAP . asymptomatic
= precipitationof particulatematerialwith high ./ nonspecific imagingappearance
concentration of calciumcarbonate,calciumphosphate, C T:
calciumbilirubinate r/ multipleirregularhypodenselesionsof up to 10 mm
Associatedwith: chroniccholecystitis+ gallstone ri tittte/ no enhancement
obstructionof cysticduct US:
r/ diffuseopacificationof GB lumenwith dependent r/ multiplesmallcysts/ echogenicareas(if size not
layering resolved)up to 10 mm + comettailartifact
r/ usuallyfunctionless GB on oral cholecystogram MR:
US: rl hypointense on TlWl
^l intermediatefeaturesbetweensludge+ gallstones ./ iso-/ slightlyhyperintense on T2Wl
r/ hypointense aftergadopentetate dimeglumine
A ngi o:
M I RI Z Z I S Y NDR OME ri multipleareasof abnormalvascularityin form of small
= extrinsicright-sided compression of commonhepatic grapelikeclusterspersistingintovenousphase
duct by largegallstoneimpactedin cysticduct/ DDx: metastaticliverdisease,hepaticabscesses
gallbladdernecki cysticduct remnant;accompanied by
chronic inflamrnatoryreaction MU LTIP LE E N D OC R IN E N E OP LA S IA
Frequentlyassociatedwith: formationof fistula between = ME N= MU LTIP LEE N D OC R INAED E N OMA(MEA)
S
gallbladderand common = familialautosomaldominantadenomatoushyperplasia
hepaticduct characterized by neoplasiaof morethan one endocrine
. jaundice organ
r/ cysticduct courseusuallyparallelto CHD
r/ normalCBD below levelof impactedstone Theory: cellsof involvedprincipalorgansoriginatefrom
^/tRtno: neuralcrestand producepolypeptidehormones
(1) gallstoneimpactedin GB neck in cytoplasmic granules,whichallowamine
(2) dilatationof bile ducts above levelof cysticduct precursoruptakeand decarboxylation
(3) smoothcurvedsegmentalstenosisof CHD = A P U Dcel l s
Cholangiography: reminder:
{ partialobstruction of CHD due to external Type | = Wermersyndrome PPP
compressionon lateralside of duct / erodingstone Type ll = Sipplesyndrome(typellA) PMP
DDx: lymphadenopathy, neoplasmof GB / CHD Type l l l = Mucosalneuromasyndrome(typel lB) M PM

MEA Type I Type ll TyPe lll

MULTIPLE
BILEDUCTHAMARTOMA
= VONMEYENBURG Pituitaryadenoma +
COMPLEX
Parathyroidadenoma + +
lncidence; 0.15-2.8%of autopsies
Medullarythyroidcarcinoma + +
Etiology: failureof involutionof embryonicbile ducts
Pancreaticislandcelltumor +
Pheochromocytoma + +
Ganglioneuromatosis +

MENI Syndrome
= W E R ME S R Y N D R OME
= autosomaldominanttraitwith highpenetrance;
M : F= 1 : 1
Cause: geneticdefectin chromosome11
Organ involvement:
1. Parathyroid (97%):multiglandular
hyperplasia
Cystic Duct Stone Stone in Stone in Cystic 2. Pancreaticisletcell tumor(30-80%):
Gallbladder Neck Duct Remnant
0 Likelymultiple+ behavingmalignant!
Mirizzi Syndrome 0 Primarycauseof morbidity+ mortality!
 Disordersof Liver,BiliaryTract,Pancreas,and Spleen 723

(a) gastrinoffl€l
= Zollinger-Ellison
syndrome(most Prevalence: 4-9-1 4/"in autopsyseries;
commontype,in S0%),usuallymulticentric 2-8% in ERCPseries;
(b) insulinoma in normalpopulation;
(c) VlPoma= WDHH-syndrome (waterydiarrhea, ?-7%
12-26% in patientswith idiopathicrecurrent
hypokalemia, hypochlorhydria) pancreatitis
3. Anteriorpituitaryglandtumor (15-50%): Hypothesis.' relativeI actualfunctionalstenosisof minor
(a) nonfunctioning papillapredisposes to nonalcoholic
(b) prolactin,growthhormone,corticotropin, TSH recurrentpancreatitisin dorsalsegment
4. Combinationof parathyroid+ pancreas+ pituitary Age: youngi middle-agedaduft
involvement(40%) . chronicrelapsingpancreatitis (clinicalrelevance
5. Adrenocorticalhyperplasia(up to gg4}%) continuesto be debated)
6. Carcinoid Pancreatography:
7. Lipoma 0 The ONLY reliablemeansfor diagnosis
. usuallyasymptomatic
r/ contrastinjectioninto majorpapillademonstrates
May be associated with: CBD + only shortventralpancreaticduct with early
thyroidtumor (20/"), thymoma,buccalmucosal arborization
tumor,colonicpolyposis,M6n6trierdisease r/ contrastinjectioninto minorpapillafills dorsal
pancreaticduct
MENll Syndrome ^/ no communicationbetweenventral+ dorsalducts
= SIPPLE DISEASE = MENTypeilA
CT:
Organ involvement:
r/ obliquefat cleft betweenventral+ dorsalpancreas
1. Medullarycarcinomaof thyroid
(25%)
2. Pheochromocytoma:bilateralin S0%; ^/ failureto see unionof dorsal+ ventralpancreatic
m aligna ni tn 3 /"
ducts (rare)
diagnosedbefore(in 10%)/atter detection(in
17%)of medullarythyroidcarcinoma
PANCREATIC ACINAR CELL CARCINOMA
3. Parathyroidneoplasia
' + hyperparathyroidism = f?re neoplasmof exocrineorigin
Age: 40-81 (mean62) years;M:F = BG:14;87"/"
May be associatedwith: carcinoidtumors,Cushing
Caucasian
disease . increasedserum lipase+ amylase
. syndromeof elevatedlipase:
MENlll Syndrome . disseminatedsubcutaneous+ intraosseousfat
= MUCOSAL NEUROMA SYNDROME = MENTypeilB
necrosis(usuallydistalto knees/ elbows)
Organ involvement: . polyarthropathy
'1.
Medullarycarcinomaof thyroid . skin lesionsresemblingerythemanodosum
2. Pheochromocytoma . biliaryobstruction
3. Oral + intestinalneuroganglioneuromatosis distinctlyuncommon
r/ lobulatedwell-definedmass of 2-15 cm in diameter
0 Usuallyprecedesthe appearanceof thyroid
r/ tnin enhancingcapsule
carcinoma+ pheochromocytoma!
. long slenderextremities(Marfanoidappearance) ^/ tumornecrosisusuallypresent
. thickenedlips (dueto submucosalnodules) r/ moderatelyvasculartumor + neovascularity + arterial
. nodulardeformityof tongue(mucosalneuromasof and venousencasement
Prognosis; mediansurvivalof 7-9 months
tongueoften initiallydiagnosedby dentists)
. prognathism DDx: (1) pancreaticadenocarcinoma(small,irregular,
. corrl€?llimbusthickening locallyinvasive,withoutcapsule,biliary
. constipationalternatingwith diarrhea obstructionif locatedin head of pancreas)
(2) Nonfunctioning islet-cefltumor
@ Gl tract
(3) Microcysticcystadenoma
r/ thickened/ plaquelikecolonicwall
./ ditatedcolonwith abnormalhaustralmarkings (a) Solidand papillaryepithelialneoplasm
r/ alternatingareasof colonicspasm + dilatation (5) Oncocytictumor of pancreas
{ muftiplesubmucosalneuromasthroughoutsmall
bowel,may act as lead pointfor intussusception PANCREATIC
DUCTALADENOCARCINOMA
= DUCTCELLADENOCARCINOMA
PANCREAS DIVISUM 0 duct cells compriseonly 4o/oof pancreatictissue
= rnostcommonanatomicvariantof pancreasdue to lncidence; 80-957" of nonendocrinepancreatic
failureof fusionof the ventraland dorsalanlageat gth neoplasms;sth leadingcause of cancer
week of fetal life with main dorsalpancreaticduct death in the UnitedStates(27,000per year)
(Santorini) Etiology: alcoholabuse (4/"), diabetes(2 x more
drainingthroughminor(accessory) papilla
+ ventralpancreaticduct (Wirsung)with CBD draining frequentthan in generalpopulation,particularly
throughmajorpapilla in females),hereditarypancreatitis(in 40%);
cigarettesmoking(riskfactor 2x)
724 Radiology Review Manual

Path: scirrhousinfiltrativeadenocarcinoma with a dense ^/ ductdilatation(58%): 3/4 biductal,1l10isolatedto

cellularity+ sparsevascularitY one duct;dilatedpancreaticduct (67%);dilatedbile
Mean age at onset: 55 years; peak age in 7th decade; ducts (38%)
p 1 . p= 2 : 1 { atrophyof pancreaticbody + tail (20%)
STAGE | = confinedto pancreas r/ calcifications(2%)
ll = + regionallymphnode metastases { postobstructive pseudocyst(11%)
lll =+distantspread { obliterationof retropancreatic fat (50%)
0 At presentation r/ thickening of celiacaxis / SMA (invasionof
- 65% of patientshave advancedlocal perivascular lymPhatics) in 60%
disease / distant metastases ^/ ditatedcollateralveins (12"/")
- 21% of patientshave localizeddiseasewith r/ thickeningof Gerotafascia (5%)
spreadto regionallYmPhnodes { localtumorextensionposteriorly, intosplenichilum,
- 14/" of patientshave tumor confinedto into porta hePatis(68%)
pancreas { contiguousorganinvasion(duodenum,stomach,
Extension: mesentericroot)in 42/"
(a) localextensionbeyondmarginsof organ (68%): US:
posteriorly(96'/"),anteriorly(30%),into porta hepatis r/ hypoechoicpancreaticmass
, os p l e n i ch i l u m(1 3 % )
( 15" / " )int ^/ focal/ diffuse(10%)enlargement of pancreas
(b) invasionof adjacentorgans(42/"): { contourdeformityof gland;roundingof uncinate
duodenum> stomach> leftadrenalgland> spleen> process
root of small bowel mesenterY r/ dilatationof pancreatic+ biliaryduct
Metastases: liver (30-36%),regionallymph nodes MR (no diagnosticimprovement over CT):
>2 cm (15-28/"), ascitesfrom peritoneal r/ hypointense lesionon fat-suppressed TlWl
carcinomatosis (7-l 0%), lungs(pulmonary ^/ diminisfred enhancement on dynamiccontrastimages
nodules/ lymphangitic), pleura,bone Angiography(70% accuracY):
. weight loss,anorexia,fatigue ^/ hypovasculartumor/ neovascularity (50%)
. pain in hypochondrium radiatingto back r/ arterialencasement:SMA (33%),splenicartery
. obstructivejaundice(75%): most frequentcause of (14/o),cel i actrunk(11' /" ),hepati cartery(1 1'/ ") ,
malignantbiliaryobstruction gastroduodenal artery(3%),leftrenalartery(0.6%)
. new onset diabetes(25-50%),steatorrhea ^/ venousobstruction:splenicvein (34%),SMV (10%)
. thrombophlebitis ! venousencasement:SMV (23/"),splenicvein (15%)'
Location: pancreatichead F6a2%); body (26%);tail portalvein (4%)
(12%) Cholangiography:
Size: 2-10 cm (in 60% between4-6 cm) r/ "rattail/ nipplelike" occlusionof CBD
i nodularmass/ meniscuslike occlusionin ampullary
UGI: tumors
{ "antralpadding"= extrinsicindentationof the Pancreatog raphy (abnormalin 97"/"):
posteroinferiormarginof antrum ./ irregular,nodular,rat-tailed,eccentricobstruction
^/ "Frostberginverted-3"sign = inverted3 contourto the r/ localizedencasementwith prestenoticdilatation
medialportionof the duodenalsweep ^/ acinardefect
r/ spiculatedduodenalwall + traction+ fixation Prognosis:
(neoplasticinfiltrationof duodenalmucosa/ 1Oh 1-yearsurvival,2/" 3-yearsurvival,<1% S-year
desmoplasticresponse) survival;14 monthsmedialsurvivalaftercurative
./ irregular/ smoothnodularmasswith ampullary resection,8 monthsafterpalliativeresection,5 months
carcinoma withouttreatment;tumors resectablein only 8-15% at
BE: presentation,5hS-yearsurvivalrateaftersurgery
! localizedhaustralpadding/ flattening/ narrowingwith DDx: focal pancreatitis,isletcell carcinoma,metastasis,
serratedcontourat inferioraspectof transversecolon lymphoma,normalvariant
/ splenicflexure
r/ diffusetetheringthroughoutperitonealcavity(from
intraperitonealseeding) ISLETCELLTUMORS
PANCREATIC
Origin: embryonicneuroectoderm, derivativesof APUD
CT (99% detectionrate for dynamicCT scan; 89% in (amineprecursoruptakeand decarboxylation)
:
predicting nonresectability) cell line arisingfrom isletof Langerhans
{ pancreaticmass (95%)i diffuseenlargement(4%)I (APUDoma)
normalscan (1%) Prevalence: 1:1,000,000population/year;isolatedor part
^l mass with centralzone of diminishedattenuation of MEN lsyndrome(= Wermersyndrome)
(75- 83%) Path: (a) smalltumor: solidwell-demarcated
^/ pancreatic+ bile duct obstructionwithoutdetectable (b) largetumor: cysticchanges+ necrosis
mass (4%) + calcifications
 Disorders of Liver, Biliary Tract, Pancreas, and Spleen 725

Histo: sheetsof small roundcells + numerousstromal frequentlyin "gastrinomatriangle"(- triangledefined

vessels by porta hepatisas apex of triangle+ 2nd and 3rd
Averagetime from onset of symptomsto diagnosisis parts of duodenumas the base)
2.7 years ,
r/ averagetumor size3.4 cm (up to 15 cm)
Classification: (a) functional(85%) r/ occasionallycalcifications
(b) nonfunctional (belowthresholdof r/ homogeneous hypoechoicmass
detectability)
/ hypofunctional A ngi o:
Metastases:in 60-90% to liver+ regionallymph nodes ./ hypervascularlesion(70%)
0 Hyperechoiclivermetastasisis suggestiveof isletcell r/ hepaticvenoussamplingafter intraarterial
tumorratherthan pancreaticadenocarcinoma! stimulationwith secretin
{ calcificationshighlysuggestiveof malignancy CT:
NUC: r/ transientlyhyperdenseon dynamicCT (majority)
r/ somatostatinreceptorimagingwith octreotide ri thickeningof gastricrugalfolds
DDx: MR:
' (1) Pancreatic ductaladenocarcinoma (hypovascular, r/ low-intensitymass on fat-suppressed TlWl
smaller,encasementof SMA + celiactrunk) r/ diminishedcentral+ peripheralringenhancement
(2) Microcysticadenoma(benigntumor,smallcysts, r/ frign-intensity
mass on fat-suppresledT2Wl
olderwomen) Sensitivityof preoperativelocalization:
(3) Metastatictumor: renalcellcarcinoma(clinicalHx) 25o/"forUS, 35% for CT, 20o/"forMRl, 42-63/otor
(4) Solidand papillaryepithelialneoplasm(young transhepatic portalvenoussamplingfor gastrin,
female,hemorrhagic areas) 68-70% for selectiveangiography,77"/"for
(5) Paraganglioma arteriographycombinedwith intra-arterialinjectionof
(6) Sarcoma(rare) secretin
surgerycurativein 30%
AOTH-producing
Tumor
rarecauseof Cushingsyndrome
. increasedlevelof serum cortisol
. impairedglucosetolerance> centralobesity>
Glucagonoma
Uncommontumor; derivedfrom crcells; M < F
hypertension,oligomenorrhea > osteoporosis
> Associatedwith: MEN
purpura> striae> muscleatrophy . necrolyticerythemamigrans(erythematousmacules/
Prognosis; almostall malignantwith metastasesat papuleson lower extremity,groin,buttocks,face) in
time of diagnosis >70/" of patients
. diarrhea,diabetes,painfulglossitis,weightloss,
anemia
Gastrinoma . plasmaglucagonlevel> 1,000ng/L
2nd mostcommonisletcelltumor; in o cells/ 5 cells
Location: predominantly in pancreaticbody/ tail
Age: 8/"in patients<20 years; M > F
ri tumor size2.5-25 cm (mean6.4 cm) with solid
Path: (a) isletcell hyperplasia(10%)
+ necroticcomponents
(b) benignadenoma(30%): in 50% solitary,in
r/ hypervascular in 90%;successfulangiographic
50% multiple(especially in MEN t) localization
in 15%
(c) malignant(50-60%) wilh metastasesto liver,
Cx: deep veinthrombosis+ pulmonaryembolism
spleen,lymphnodes,bone Prognosis; in 60-80% malignanttransformation(liver
Associatedwith: MEN Type | (in 10-40%)
. Zolfinger-Ellison metastasesat time of diagnosisin 50%);
syndrome: severerecurrentpeptic 55/o S-yearsurvival rate
ulcerdisease(>90%),malabsorption, hypokalemia,
gastrichypersecretion, hyperacidity/ occasionally
hypoacidity,diarrhea(fromgastrichypersecretion)
Insulinoma
Most commonfunctioningisletcell tumor
0 Only 1:1,000patientswith pepticulcerdiseasehas
Age: 4th-6thdecade; M:F = 2:3
a gastrinoma!
. Gl bleeding Associatedwith: MEN type I
. elevatedserum levelsof gastrin Path: (a) singlebenignadenoma(80-90%)
(b) multipleadenomas/ microadenomatosis
Location:
(5-10%)
(a) 87% in pancreas(50% solitaryin head / tail)
(c) isletcell hyperplasia
(5-10%)
(b) ectopic(7-33%):
- duodenalwall (13%in medialwall of (d) malignantadenoma(5-10%)
. Whippletriad: starvationattack+ hypoglycemia
duodenum= gastrinomatriangle)
- peripancreaticnodes/ spleen (fastingglucose<50 mg/dl) + reliefby lV dextrose
. neuroglycopenic symptoms: headaches,confusion,
- stomach,jejunum
- omentum,retroperitoneum coma
. hypoglycemiaexacerbatedby fastingresultsin
- ovary
frequentmealsto avoidsymptoms
726 Radiology Review Manual

. sweating,palpitations,tremor (secondaryto { tumor size 0.6-20 cm (average>4 cm)

catecholaminereleasein responseto hypoglycemia) { hypervascular
. obesity Prognosis; 50-90% malignanttransformation;
. firm rubberypalpablemass at surgery(in >90%) metastaticdiseasein70'/" at time of initial
Location: no predilectionfor any part of pancreas, diagnosis
2-5Y" in ectopiclocation;10% multiple
(especially in MEN l) VlPoma
r/ averagetumor size 1-2 cm; <1.5cm in70/" = solitaUtumor liberatingVasoactiveIntestinalPeptides
US (20-757opreoperativeand 75-100% endoscopic actingdirectlyon cyclicadenosinemonophosphate
+ intraoperative :
sensitivity) (AMP)withinepithelialcellsof bowelrelaxingvascular
r/ round/ oval smoothlymarginatedsolid smoothmuscle;sporadicoccurrence
homogeneouslyhypoechoicmass Histo: adenoma/ hyPerPlasia
A ngio: M : F= 1 : 2
./ hypervascular tumor (66%): accurateangiographic . WDHA syndrome = watery diarrhea+ hypokalemia
localizationin 50-90% + achlorhydria(morerecently+ more accurately
{ transhepaticportalvenoussampling(correct describedas) WDHH syndrome = waterYdiarrhea
localizationin 95%) + hypokalemia+ hypochlorhydria = "pancreatic
r/ hepaticvenoussamplingafter intraarterial cholera" = Verner-Morrison syndrome
stimulation with calciumgluconate . dehydrationdue to massivediarrhea(>1 L/day)
C ECT (30-75% sensitivity) : Location:
^/ nypo-/ iso- / hyperattenuatinglesion (1) pancreas:from 6 cellspredominantly in
MR: pancreaticbodY/ tail
r/ low signalintensityon fat-suppressed Tl Wl (2) extrapancreatic:retroperitoneal ganglioblastoma,
r/ hyperintenseon T2Wl + dynamiccontrast- pheochromocytoma, lung, neuroblastoma(in
enhanced+ suppressedinversionrecoveryimages children)
Prognosis; malignanttransformationin 5-10% { averagesize 5-10 cm with solid+ necrotictissue
Rx: surgerycurative r1 mostlyhypervasculartumor
^/ dilatationof gallbladder
Prognosis; in 50-80% malignanttransformation
lsletCellTumor
Nonfunctioning DDx: smallcell carcinomaof lungi neuroblastoma may
lncidence; 3rd most commonisletcell tumor after alsocauseWDHHsYndrome
insulinoma+ gastrinoma;15-25"/"ol all
isletcelltumors
derivedfrom eithercror B cells PANCREATIC LIPOMATOSIS
Age: 24-74 (mean57) years = FATTYREPLACEMENT = FATTYINFILTRATION
. mostlyasymptomatic(hormonallyquiescent) = depositionof fat cellsin pancreaticparenchyma
. abdominalpain,jaundice,gastricvaricealbleeding Predisposing factors:
. palpablemass,gastricoutletobstruction 1. Atherosclerosis of elderlY
Location: predominantlyin pancreatichead 2. Obesity
{ tumor size 6-20 cm (>5 cm in 72%) with solid 3. Steroidtherapy
+ necroticcomponents 4. Diabetesmellitus
r/ coarse nodularcalcifications(20-25%) 5. Cushingsyndrome
.l Cf contrastenhancementin 83% 6. ChronicPancreatitis
./ hypoechoicmass 7. Main pancreaticduct obstruction
r/ late dense capillarystain 8. Cysticfibrosis(mostcommoncausein childhood)
^l large irregularpathologicvesselswith earlyvenous 9. Malnutrition/ dietarydeficiency
f illing 10. Hepaticdisease
Prognosis.' in 80-100% malignanttransformationwith 11. Hemochromatosis
metastasesto liver+ regionalnodes;60% 12. Viralinfection
3-yearsurvival;44h S-yearsurvival 13. Schwachman-Diamond sYndrome
Rx: may respondto systemicchemotherapy 14. Johanson-Blizzard sYndrome
r/ tatty replacementoften uneven:
Somatostatinoma r/ increasein AP diameterof pancreatichead with focal
Origin: derivedfrom 6 cells fatty replacement= lipomatouspseudohypertrophy
. inhibitorysyndrome= inhibitoryactionof somatostatin { prominentlylobulatedexternalcontour
on otherpancreatic+ bowelpeptides(growthhormone, US:
TSH, insulin,glucagon,gastricacid,pepsin,secretin) ./ increasedpancreaticechogenicity
. diabetes,cholelithiasis,
steatorrhea CT:
. elevatedlevelof somatostatin { "marbling"of pancreaticparenchyma/ total fatty
Location: predominantlyin pancreatichead replacement/ lipomatouspseudohypertrophy

PancreaticFattySparing
= sparingof fatty changein pancreatichead + uncinate
process(ventralpancreaticanlage)as initialstage in
pancreaticlipomatosis
Histo: ventralpancreaticanlagehas smaller+ more
denselypackedaciniwith scanty/ absent
interacinarfat
US:
i/ rounded/ triangularhypoechoicareawithin
pancreatichead/ uncinateprocess+ diffusely
increasedechogenicityin remainderof gland
CT:
r/ frigner-densityregionof pancreatichead + uncinate
processwith diffuselydecreasedattenuationof
pancreaticbody + tail
PANCREATIC
PSEUDOCYST
= collectionof pancreaticfluidencapsulated by fibrous
tissue
Etiology: (1) Acute pancreatitis; requires>4 weeksto
form; pseudocystsmaturein 6-g weeks
(2) Chronicpancreatitis
(3) Posttraumatic
(4) Pancreaticcancer
lncidence: 2-4/" in acute pancreatitis;
10-15% in chronicpancreatitis
Location: 2/3 withinpancreas
Atypicallocation(maydissectalongtissueplanesin 1/3):
(a) intraperitoneal:mesenteryof small bowel/
transversecoloni sigmoidcolon
(b) retroperitoneal: alongpsoasmuscle;may present
as groinmass/ in scrotum
(c) intraparenchymal: liver,spleen,kidney
(d) mediastinal(throughesophagealhiatus> aortic
hiatus> foramenof Morgagni> erosionthrough
diaphragm):may presentas neck mass
May communicate with: duodenum,stomach,spleen
Plainfilm/ contrastradiograph:
r/ smoothextrinsicindentationof posteriorwall of
stomachi innerduodenalsweep(80%)
r/ indentation / displacement of splenicflexure/
transversecolon (40%)
^/ downwarddisplacement of duodenojejunal
r/ gastricoutletobstruction
r/ splayingof renalcollectingsystem/ ureteral
obstruction
US (pseudocystdetectablein 50-g2./";g2-g6%accuracy):
! us uallys ingl e+ u n i l o c u l acry s t
! multilocular in 6%
^/ ttuid-debris level/ internalechoes(maycontain
sequester,bloodclot,cellulardebrisfrom autolysis)
r/ septations(rare;sign of infection/ hemorrhagej
r/ may increasein size (secondaryto hypertonicityof
fluid,communication withpancreatic duct,hemorrhage,
erosionof vessel)
r/ obstructionof pancreaticduct / CBD
CT:
r/ ttuiOin pseudocyst(0-30 HU)
r/ cyst wall calcification(extremelyrare)
Disorders of Liver, Biliary Tract, Pancreas, and Spleen 727
Pancreatography;
r/ communicationwith pancreaticduct in up to SO-70%
Indications for pseudocyst drainage:
pain,suspectedinfection,persistence of pseudocyst
>5 cm, increasingsize,biliary/ gastrointestinal
obstruction
Cx (in 40%):
1. Ruptureintoabdominalcavity,stomach,colon,
duodenum
2. Hemorrhage / formationof pseudoaneurysm
3. Infection= p?ncr€?tic abscess
. usuallyoccurs>4 weeks after acute pancreatitis
. symptomatology of infection
^/ gas bubbles(DDx:fistulouscommunication to Gl
tract)
./ increasein attenuationof fluid contents
Dx: transcutaneousneedleaspiration
4. Intestinalobstruction
Prognosis; spontaneousresolution(in 20-50%)
secondaryto ruptureinto Gl tractI
pancreatic/ bile duct
DDx: pancreaticcystadenoma,cystadenocarcinoma,
necroticpancreaticcarcinoma,fluid-filled bowel
loop,fluid-filledstomach,duodenaldiverticulum,
aneurysm
PANCREATIC TRANSPLANTATION
Complications: sepsis,rejection,pancreatitis,
pseudocyst,pancreaticabscess
(22%),anastomoticleak
Prognosis: 40/" survival rate >1 year
Graft-vesselThrombosisin Pancreatic
Transplant(2-19%)
A. Earlythrombosis(<1 monthaftertransplantation)
Cause: technicalerror in fashioninganastomosis,
microvasculardamagedue to preservation
injury
B. Late thrombosis(>1 monthaftertransplantation)
Cause: alloimmunearteritiswith gradualocclusion
of smallbloodvessels
junction Acute Rejectionof PancreaticTransplant
. focal tendernessover transplant
. measurement of urinary+ serumamylase,blood
glucose(nonspecific for diagnosisof rejection)
US:
r/ poor marginationof transplant
ri acousticinhomogeneity
ri dilatedpancreaticduct
PANCREATITIS
Cause:
A. IDtoPATHtC(20%)
B. ALCOHOLISM(<50%): acutepancreatitis(15%);
chronicpancreatitis
(70%)
C. CHOLELITHIASIS (<50%): acutepancreatitis
(75%);chronicpancreatitis(20%)
728 Radiology Review Manual

G . STRUCTURAL ABNORMALITI ES
splenic a. 1. Pancreasdivisum
0 fn 12-50% of caseswith no other underlying
abnormalities
2. Choledochocele
splenic v. H . D R U GS
Azathioprine,thiazide,furosemide, ethacrynicacid,
pancreatlc a. sulfonamides, phenformin,
tetracycline, steroids(eg,
renaltransplant), acetami
l-asparaginase, nophen,
donor iliac
bifurcation procainamide
I. MA LIGN A N C Y
Pancreaticcarcinoma(in 1%), metastases,
anastomotic lymphoma
branch J. MU LTIS Y S TE M C ON D ITION S
1. Sepsisand shock
SMA 2. Hemolytic-uremic sYndrome
dorsal
pancreatlc a. 3. Reyesyndrome
SMV 4. SystemiclupuserYthematosus

portal v. Theories of pathogenesis:

Refluxof bile / pancreaticenzymes/ duodenalsuccus
(a) terminalduct segmentsharedby commonbileduct
uto + pancreaticduct
Biu
(b) obstructionat papillaof Vaterfrom inflammatory
anterior pancreaticoduodenala.
stenosis,edema/ spasmof sphincterof Oddi,tumor,
posterior pancreaticoduodenala. periduodenal m
diverticulu
(c) incompetent sphincterof Oddi
Pancreatic TiansPlantation
EIA = external iliac artery, EIV = extemal iliac vein,
GDA - gastroduodenal artery,IMV = inferior mesenteric vein, Acute Pancreatitis
SMA = iuperior mesenteric artery, SMV = superior mesenteric vein = inflammatorydiseaseof pancreasproducing
temporarychangeswith potentialfor restorationof
normalanatomy+ functionfollowingresolution
Path:
1. IN TE R S TITIAELD E MA TOU S P A N C REATI TI S
D. M E T A B O L ICD ISOR D E R S (75-95%): edema,congestion,leukocytic
1. Hypercalcemia (10%),
in hyperparathyroidism infiltrates;mortalityrateof 4o/o
multiplemyeloma,amyloidosis, sarcoidosis 2. NECROTIZING PANCREATITIS (5-25%):
2. Hereditary pancreatitis: autosomaldominant, proteolyticdestructionof pancreaticparenchyma;
only Caucasiansaffected,most commoncause mortalityrate of 80-90%
of largesphericalpancreaticcalcificationsin (a) HEMORRHAGICPANCREATITIS:
childhood(in 50%),recurrentepisodesof + fat necrosisand hemorrhage(due to erosion
pancreatitis,developmentinto pancreatic of smallvessels)
carcinomain 20-40'/": pronounceddilatationof . fallinghematocrit
pancreaticduct; pseudocystformation(50%); (b) S U P P U R A TIV P
E A N C R E A TITIS:
associatedwith type l hypercholesterolemia + bacterialinfection
3. Hyperlipidemia types I and V A. Diffusepancreatitis(52%)
4. Cysticfibrosis B. Focalpancreatitis(48%): locationof head:tail= 3:2
E. INFECTION/ INFESTATION
1. Viralinfection(mumps,hepatitis,Coxsackie Mild Acute Pancreatitis(75%)
virus,mononucleosis) . minimal dysfunction
organ
2. Parasites(ascariasis,clonorchis) Path: interstitialedema
F . T RA UM A Prognosis: improvementwithin 48-72 hours
0 One of the most commoncausesof pancreatitis followingconservativetherapywith
in childhood! gradualdecreaseof elevatedenzymes
1. Penetrating ulcer Mortality: 1-5%
2. Blunt/ penetrating trauma
trauma;nonaccidental
3. Surgery(in 0.8%of Billroth-llresections,0.87"of Severe Acute Pancreatitis
splenectomies, surgery,
0.7%of choledochal 0 Developsshortlyafter onset of untreatedmild acute
0.4% of aorticgraft surgery) pancreatitis
 Disordersof Liver,BiliaryTract,Pancreas,and Spleen 729
. increasedabdominaltenderness,rebound
distension,hypoactivebowelsounds
Associatedwith: organfailure/ localcomplications
Path: pancreaticcell breakdown+ necrosis
Cx: acutefluid collection,pancreaticnecrosis,
pseudocyst,abscess
Acure Flulo Cor-leclorus (OO-5O%)
= early form of acute pseudocyst/ pancreatic
abscess
Path: lack of a definedwall of fibrous/ granulation
tissue;pancreatic phlegmon [misnomer,no
infectionl= solid boggy inflammatorymass
characterizedby edema,infiltrationof
inflammatorycells + necrosisof
retroperitoneal fat
Location: extensionintolessersac, anterior
pararenalspace,transverse
mesocolon,smallbowelmesentery,
retroperitoneum, pelvis
! nearO HU on CT
Prognosis; spontaneousregression(in 40-50%)
Parucnenlc Necnosls
= focal/ diffusearea of nonviablepancreatic
parenchyma
Path: clumpsof devitalizedpancreaticparenchyma
+ hemorrhagein pancreaticand
peripancreatic tissues
Histo: extensiveinterstitialfat necrosiswith vessel
damage+ necrosisof acinarcells,isletcells,
ductalsystem
Associatedwith: peripancreaticfat necrosis
r/ focal/ diffusewell-marginated zones of
unenhancedpancreaticparenchyma> 3 cm i
involving>30o/oof the pancreaticgland
Acure Pseuoocvsr
= collectionof pancreaticfluidenclosedby a wall of
fibrousgranulation tissue
Cause: acute pancreatitis,pancreatictrauma,
chronicpancreatitis
Time of onset: >4 weeks after acute pancreatitis
. amylase-rich fluid
Prognosis; spontaneousresolutionin 44h
PaHcnelnc ABScEss
= well-demarcated fluidcollectionof pus usually
closeto the pancreas
Time of onset: 2-4 weeks after severe acute
pancreatitis
Organism; most commonlydue to E. coli
. liquefiedtissuewith little/ no necrosis
r/ may containgas withinpancreaticbed in 30-50%
(DDxof gas: cutaneous/ entericfistula,ruptured
duodenum,iatrogenicgas collection)
DDx: infectednecrosis
Clinical stages:
| - EDEMATOUSPANCREATITTS (75%)
. rapid improvementfollowingconservative
therapy
. gradualdecreaseof elevatedenzymes
Mortality: 1-5%
II = PARTIALLYNECROTIZING PANCREATITIS
. delayed/ no responseto conservative therapy
. delayed/ no normalization of enzymes:
. hyperglycemiaof <200 mg/100mL
. hypocalcemiaof >4 mval/L
. base deficitof <4 mvaliL
. leukocytosisof <16,000
Mortality: 30-75%
III = TOTALLYNECROT]ZING PANCREATITIS
. deteriorationunderconseryativetherapy:
. hyperglycemia of >200 mg/100mL
. hypocalcemiaof <4 mval/L
. base deficit of >4 mvallL
. leukocytosis of >16,000
Mortality: 100% (40% by 2nd day,75o/"by
Sth day, 100%by 1Othday)
. acute abdominalpain (peakingaftera few hours,
resolvingin 2-3 days),nausea,vomiting
. raisedpancreaticamylase+ lipasein blood + urine
. increasedamylase-creatinine clearanceratio
. signsof hemorrhagic pancreatitis:
. Cullensign = periumbilicalecchymosis
. Grey-Turner sign = flankecchymosis
. Fox sign = infrainguinalecchymosis
. subcutaneousnodules+ fat necrosis+ polyarthritis
r/ NO findingson US I CT in 29/"
Abdominalfilm:
r/ "coloncutoff"sign (2-52%) = dilatedtransverse
colonwith abruptchangeto a gaslessdescending
colon(inflammation via phrenicocolicligament
causesspasm + obstructionat the splenicflexure
impingingon a paralytictransversecolon)
r/ "sentinelloop"(10-55%)= localizedsegmentof
gas-containingbowel in duodenum(in 2045%) I
terminalileum/ cecum
r/ "renalhalo"sign = water-densityof inflammationin
anteriorpararenalspace contrastswith perirenal
fat; morecommonon leftside
r/ mottledappearanceof peripancreatic area
(secondaryto fat necrosisin pancreaticbed,
mesentery,omentum)
{ intrapancreaticgas bubbles(fromacute gangrene/
suppurativepancreatitis)
./ "gaslessabdomen"= fluid-filledbowelassociated
with vomiting
! ascites
CXR (findingsin 14-71%):
{ pleuraleffusion(in 5%), usuallyleft-sided,
with
elevatedamylaselevels(in 85%)
! left-sideddiaphragmaticelevation
! left-sidedsubsegmentalatelectasis(20%)
{ parenchymalinfiltrates, pulmonaryinfarction
730 Radiology Review Manual

{ pulmonaryedema,ARDS CT (pancreatic visualization in 98%):

! pleuralempyema,pericardialeffusion r/ no detectablechangein size / appearance(29%)
r/ mediastinalabscess,mediastinalpseudocyst r/ enlargementof pancreaswith convexmargins
r/ / -pleural/-pulmonaryfistula
pancreatico-bronchial + indistinctness of gland+ parenchymal
heterogeneity:
UGI: r/ hypodense(5-20 HU) mass in phlegmonous
r/ esophagogastric varices(fromsplenicvein pancreatitis;may persistlong aftercomplete
obstruction) recovery
{ enlargedtortuousedematousrugalfoldsalong r/ hyperdenseareas(50-70 HU) in hemorrhagic
antrum+ greatercurvature(20%) pancreatitistor 24-48 hours
r/ wideningof retrogastricspace (frompancreatic rl thickeningof anteriorpararenalfascia
enlargement i inflammation in lessersac) ^/ "halosign"= sparingof perirenalspace
r/ diminishedduodenalperistalsis + edematousfolds ^/ non-contrast-enhancing parenchymaduringbolus
r/ wideningof duodenalsweep + downward injection(= pancreaticnecrosis)
displacement of ligamentof Treitz ^/ ttuiOcollection
r/ Poppelsign = edematousswellingof papilla Angiography:
r/ Frostberginverted-3sign = s€grT'rehtal narrowing { may be normal
with foldthickeningof duodenum ^/ hypovascularareas (15-56%)
r/ jejunal+ ilealfold thickening(proteolytic
spread ^/ hypervascularity + increasedparenchymalstain
alongmesentery) (12- 45%)
BE: ^/ venouscompressionsecondaryto edema
r/ narrowing,nodularity, folddistortionalonginferior ^/ formationof pseudoaneurysms (in 10% with chronic
haustralrow of transversecolon+ descending pancreatitis):splenicartery (50%),pancreatic
colon arcades,gastroduodenalartery
Cholangiography: Cx:
^/ tonggentlytaperednarrowingof CBD 1. P hl egmon(18% )
{ prestenotic biliarydilatation 2. Pseudocyst formation(10%)
r/ smooth/ irregularmucosalsurface 3. Hemorrhagic pancreatitis (2-5%)
4. Abscess(2-10%)
B onef ilm s( f in d i n g isn 6 % ): 5. Pancreaticascites
Cause: metastaticintramedullarylipolysis+ fat 6. Biliaryduct obstruction
necrosis+ trabecularbonedestruction 7. Thrombosisof splenicveini SMV
Time of onset: usually3-6 weeks after peak of 8. Pseudoaneurysm
clinicalpancreatitis (a) ruptureintopreexisting pseudocyst
r/ punchedout / permeativemottleddestructionof (b) digestionof arterialwall by enzymes
cancellousbone + endostealerosion lncidence; in up to 10% of severepancreatitis
r/ asepticnecrosisof femoral/ humeralheads Location: splenicartery(mostcommon),
r/ metaphysealinfarcts,predominantlyin distalfemur gastroduodenal, pancreatico-
+ proximaltibia duodenal,hepatic,left gastricartery
US (pancreaticvisualizationin 62-78/"): Mortality: 37"h for rupture, 16-50% for surgery
^/ hypoechoicdiffuse/ focal enlargementof pancreas 9. Thoracopancreatic fistula
^/ dilatationof pancreaticduct (if head focally (a) pancreaticopleural fistula
involved) (b) pancreaticopericardial fistula
^/ perivascularcloaking= sppgadof inflammatory (c) pancreaticoesophageal fistula
exudatealong perivascularspaces (d) pancreaticobronchial fistula
r/ extrapancreatic hypoechoicmass with good (e) mediastinalpseudocyst
acoustictransmission (= phlegmonous pancreatitis) Rx:
r/ ttuiOcollection:lessersac (60%),L > R anterior 1. Conservative (NPO,gastrictube,atropine,
pararenalspace (54h), posteriorpararenalspace analgesics, sedation,prophylactic for
antibiotics)
(18/"), aroundleft lobeof liver(16%), in spleen stage I
(9/"), mediastinum (3%),iliacfossa,along 2. Earlysurgeryin stagesll and lll
transversemesocolon/ mesentericleavesof small
intestine
Fateof fluidcollection:
(a) completeresolution
(b) pseudocystformation
(c) bacterialinfection= abscess Chronic Pancreatitis
^/ pseudocystformation(52%): extensioninto lesser = continuedinflammatorydiseaseof pancreas
sac,transversemesocolon,aroundkidney, characterizedby irreversiblepermanentdamageto
mediastinum, lowerquadrantsof abdomen anatomy+ function
 Disordersof Liver,BiliaryTract,Pancreas,and Spleen 731
Etiology:
A. CHRONICCALCIFYINGPANCREATITIS
1. Juvenile tropical pancreatitis = Kwashiorkor:
in equatorialthird-worldcountries,associated
with pure proteinmalnutrition,patientspresent
with diabetes+ chronicabdominalpain
2. Hereditarypancreatitis
3. Inbornerrorsof metabolism
4. Hyperlipidemia
5. Hypercalcemia
r/ proteinplugs/ calculiwithinductalsystem
B . CHRO N ICOB ST R U C T IVE PA N C R E ATITIS :
1. Congenital/acquiredlesionsof pancreatic duct
2. Trauma/ surgicalduct ligation
3. Sphincterof Oddidysfunction, ampullary
stenosis
4. Sclerosingcholangitis
5. ldiopathicfibrosingpancreatitis
6. Renalfailure
7. Slow growingampullarytumor
! dilatationof pancreaticduct
r/ normalsizedI focallyor diffuselyenlarged/
smallatrophicgland
! calcificationsuncommon
. acute exacerbationof epigastricpain (93%):
decreasingwith time due to progressivedestructionof
gland,usuallypainlessafter7 years
. jaundice(42%)from commonbile duct obstruction
. steatorrhea(80%)
. diabetesmellitus(58%)
. secretintest with decreasedamylase+ bicarbonatein
duodenalfluid
P lainf ilm :
r/ numerousirregularcalcifications(in 20-50/" of
alcoholicpancreatitis)PATHOGNOMONTC
UGI:
r/ displacement of stomach/ duodenumby
pseudocyst
r/ shrinkage/ fold indurationof stomach(DDx:linitis
plastica)
{ strictureof duodenum
Cholangiopancreatography (mostsensitiveimaging
modality):
{ side-branchectasia= slightductalectasia/
clubbingof side branches(minimaldisease)
! "nipping"= n?rrowingof the originsof side
branches
r/ dilatation>2 mm, tortuosity,wall rigidity,main
ductalstenosis(moderatedisease)
{ "beading,chainof lakes,stringof pearls"
= multifocaldilatation,stenosis,obstructionof main
, pancreaticduct + side branches(severedisease)
! intraductalfilfingdefectsdue to mucinousprotein
plugs/ c alc u l i /d e b ri s
! prolongedemptyingof contrastmaterial
! may have stenosis/ obstruction+ prestenotic
dilatationof CBD
./ tiltingof pseudocysts(<50%)
US / CT:
r/ irregular(73%)/ smooth(15%)/ beaded(12%)
. pancreaticductaldilatation(in 41-68%)
r/ smallatrophicgland (in 10-54%)
{ pancreaticmostlyintraductalcalcifications1+-66.7.;
r/ inhomogeneous glandwith increasedechogenicity
(62%)
r/ irregularpancreaticcontour(45-60%)
! focal (12-32/") /diffuse (2745%) pancreatic
enlargement duringflareup (DDx:pancreatic
carcinoma)
mostlymild biliaryductaldilatation(29%)
{ intra-/ peripancreaticpseudocysts(20-34%)
{ segmentalportalhypertension(= splenicvein
thrombosis+ splenomegaly) in 11./"
arterialpseudoaneurysm formation
peripancreaticfascialthickening+ blurringof organ
margi ns(16% )
ascites/ pleuraleffusion(9%)
MR:
{ lossof signalintensityon fat-suppressed Tl Wl
(fromloss of aqueousproteinin pancreaticacini
secondaryto fibrosis)
r/ diminishedcontrastenhancement(fromlossof
normalcapillarynetworkreplacedby fibroustissue)
Angiography:
r/ increasedtortuosity+ angulationof pancreatic
arcades+ intrahepaticarteries(88%)
r/ luminalirregularities/ focal fibroticarterialstenoses
(25-75%) / smooth beaded appearance
r/ irregularparenchymalstain
r/ venouscompression/ occlusion(20-50%)
r/ portoportalshunting+ gastricvariceswithout
esophagealvarices
Cx: pancreaticcarcinoma(2-4%),jaundice,
pseudocystformation,pancreaticascites,
thrombosisof splenic/ mesentericlportal vein
Rx: surgeryfor infectedpseudocyst,Gl bleedingfrom
portalhypertension, commonbileduct
obstruction,gastrointestinal obstruction
DDx: pancreaticcarcinoma(extrapancreatic spread)
PANCREATOBLASTOMA
= rdrechildhoodtumoroftenmisdiagnosed as
neuroblastoma/ hepatoblastoma
Age: <7 years
. palpablemass,anorexia,vomiting
{ up to 10 cm largewell-definedlobulatedsolid/
multiloculatedmass in regionof lessersac
PAPILLARY ADENOMA OF BILE DUCTS
= very rare benigntumor of biliarytract
Path: usuallysolitarytumor/ papillomatosis
with papillary
frondsextendingintolumen
Histo: columnarepitheliumsupportedby connective
tissuefrom laminapropria
. biliaryobstruction
Location: commonbileduct > right/ left hepaticduct
732 Radiology Review Manual

r/ usuallysmall intraductalmass { hepatomegaly, splenomegaly

r/ visualizedat cross-sectional
imagingonly if largeenough
Prognosis: high rate of recurrenceaftersurgicalresection
Cx: malignanttransformation (rare)
PASSIVEHEPATIC
CONGESTION
Cause: CHF, constrictivepericarditis
Pathophysiology: chroniccentralvenous hypertension
transmitted to hepaticsinusoids
resultsin centrilobular
congestion+
eventuallyhepaticatrophy,necrosis,
fibrosis
. abnormalliverfunctiontests
CT:
{ globallydelayedenhancement(36%)
r/ enhancementof portalveins + hepaticarteries+
immediately adjacentparenchyma(56%)
r/ "reticulatedmosaic"pattern= lobularpatchyareas of
enhancementseparatedby coarselinearregionsof
diminishedattenuation (100%)
./ diminishedperiportalattenuation(24%)
^/ diminishedattenuation aroundintrahepaticIVC (8%)
r/ prominentIVC + hepaticvein enhancement(dueto
contrastrefluxfrom rightatriuminto dilatedIVC)
DDx: Budd-Chiari syndrome(regional/lobular
distributionof reticulatedmosaicpattern,caudate
lobe hypertrophy)
PE LI O S I S
lpelios, Greek = purplel
= fore benigndisordercharacterizedby multipleblood-
filledcavitieswithinorgansof the RES
Cause: (a) ? acquired: chronicinfection(disseminated
TB), hepatotoxic drugs(androgen-anabolic
steroids,corticosteroids,
tamoxifencitrate,
chemotherapeutic agents,azathioprine, oral
contraceptives,thoriumdioxideinjection),
diabetesmellitus,chronicrenalfailure,
advancedmalignancy(Hodgkindisease,
myeloma,disseminated cancer)
(b) bacillarypeliosishepatisin AIDS (lesions
containbacilliof Rochalimaea species)
responsiveto antibiotics
(c) ? congenital:angiomatousmalformation
Histo: (1) Phlebectatic peliosishepatis(earlystage)
= endothelial-lined cysts(= ? dilatationof
centralveins)communicatingwith dilated
hepaticsinusoids+ compressionof
surroundingliver
(2) Parenchymal peliosishepatis(latestage)
= irregularlyshapedcystswithoutlining
communicating with dilatedhepaticsinusoids
+ areasof livercell necrosis
Associatedwith: hormonallyinducedbenign/ malignant
tumors
Location: liver(mostcommon),spleen,bone marrow,
lymphnodes,lungs)
Age: fetal life (rare)to adult life
. incidentaldiscovery
US:
r/ multipleindistinctareasof hypo-/ hyperechogenicity
C E C T:
r/ initiattyhypoattenuating, with passageof time
isoattenuating / enhancingroundlesions
MR:
{ mixedsignalintensitydue to repeatedhemorrhage
(deoxyhemoglobin + methemoglobin + siderotic
nodules)
A ngi o:
r/ multiplesmall(severalmm to 1.5cm) round
collectionsof contrastmediumscatteredthroughout
liver in late arterialphase of hepaticarteriogram
r/ t simultaneous opacificationof hepaticveins
Prognosis; reversibleafterdrug withdrawal/ progression
to hepaticfailure/ intraperitoneal
hemorrhageleadingto death
ABSCESS
PERICHOLECYSTIC
Cause: subacuteperforationof gallbladderwall
subsequentto gangrene+ infarctiondue to
acute cholecystitis
Prevalence: 2-20/"
Location:(a) gallbladderbed (mostcommon)
{ area of low-levelechoesin liveradjacent
to gallbladder
(b) intramural
{ smallarea of low-levelechoeswithin
thickenedgallbladder wall
(c) intraperitoneal
^/ area of low-levelechoeswithinperitoneal
cavityadjacentto gallbladder
Rx: (1) Emergencyoperation
(2) Antibiotictreatment+ electiveoperation
(3) Percutaneous abscessdrainage
P OR C E LA IN GA LLB LA D D E R
= calciumcarbonateincrustation of gallbladderwall
lncidence; 0.6-0.8/" of cholecystectomy patients;
M:F=1:5
Histo: (a) flakesof dystrophiccalciumwithinchronically
inflamed+ fibroticmuscularwall
(b) microlithsscattereddiffuselythroughout
mucosa,submucosa,glandularspaces,
Rokitansky-Aschoff sinuses
Associatedwith: gallstonesin 90%
' minimalsymptoms
r/ curvilinear(muscularis) / granular(mucosal)
in segmentof wall/ entirewall
calcifications
r/ nonfunctioning GB on oral cholecystogram
r/ nightyechogenicshadowingcurvilinear structurein GB
fossa (DDx:stone{illedcontractedGB)
r/ echogenicGB wall with littleacousticshadowing(DDx:
emphysematous cholecystitis)
r/ scatteredirregularclumpsof echoeswith posterior
acousticshadowing
Cx: 10-20% developcarcinomaof gallbladder
 Disordersof Liver,BiliaryTract,Pancreas,and Spleen 733

paraesophageal - sinusoidal
coronary 1. Hepatitis
retrogastric
2. Sicklecell disease
- postsinusoidal
1. Cirrhosis(mostfrequent):Laennec
cirrhosis,postnecroticcirrhosisfrom
hepatitis
para-
2. Venoocclusive diseaseof liver
umbilical
@ Posthepatic
1. B udd-C hi ari
syndrome
splenorenal 2. Constrictivepericarditis
shunt 3. CHF (tricuspidincompetence)

caput
medusae
K'u:-**, Pathophysiology:
continuedelevatedpressuredespiteformationof portal
venouscollateralvesselsmay be explainedby
mesenterlc (a) backwardflow theory= hypodynamicflow theory
= increasein sinusoidalpressuredue to deposition
hemorrhoidal
of collagenin the spacesof Disse+ hepatocyte
epigastric v. retroperitoneal- swelling
paravertebral . low / stagnantportalvenousflow rates
Portosystemic Collateral Vessels (b) forwardflow theory= hyperdynamicflow theory
in Portal Hypertension = splanchnicflow increasessecondaryto
mesentericvasodilators+ increasein cardiac
outputto preservehepaticperfusion+ intrahepatic
endogenousvasoconstrictors
. increasedportalvenousflow rates>15 mUmin/kg
PORTAL HYPERTENSION Flow direction:
= portalvenouspressure>10 mm Hg (a) hepatopetal(pefere, Latin= to seek)
. normalhepaticbloodflow of 550-900 mUmin (= 2S/" of (b) hepatofugal(fugere, Latin= to flee) = flow reversal
cardiacoutput)passesthroughportalsystem(2lB)+ Cause: intrahepaticarterioportalcommunications
throughhepaticartery(1/3) (insideportaltriadsvasa vasorumof portal
veins + hepaticarteriesconnectvia bile
Classification: duct capillariesto portalvein)
A . DY NA M I C/ H YP ER KIN ET IC P O R T AL . elevatedhepaticwedge pressure(HWP)= portal
HY P E RT E N S IO N venouspressure;normalvaluesseen in presinusoidal
congenital/ traumatic/ neoplasticarterioportalfistula portafhypertension
B . I NCRE A S ED POR T ALR E SIS T AN C E . caput medusae = drainagefrom paraumbilical+
@ Prehepatic omentalveinsthroughsuperficial veinsof chest(lateral
- portalvein thrombosis(portalphlebitis,oral thoracicvein to axillaryvein;superficialepigastricvein
contraceptives, coagulopathy,neoplastic to internalmammaryvein and subclavianvein)+
invasion,pancreatitis, neonatalomphalitis) abdominalwall (circumflex iliacvein and superficial
- portalvein compression(tumor,trauma, epigastricvein to femoralvein; inferiorepigastricvein to
lymphadenopathy, portalphlebosclerosis, externaliliacvein)
pancreaticpseudocyst) . hemorrhaging esophagealvarices(50%)
@ Intrahepatic(= obstructionof portalvenules)
- presinusoidal @ Splanchnicsystem:
1. Congenitalhepaticfibrosis { portalvein >13 mm (57% sensitivity , 1OO/o
2. ldiopathicnoncirrhoticfibrosis specificity)
3. Primarybiliarycirrhosis ^/ SttttV+ splenicvein >10 mm; coronaryvein >4 mm;
4. alpha-1antitrypsin deficiency recanalized umbilicalvein >3 mm (sizeof vessels
5. Wilsondisease not relatedto degreeof portalhypertensionor
6. Sarcoidliverdisease presenceof collaterals)
7. Toxicfibrosis(arsenic,copper,pVC) r/ loss of respiratoryincreaseof splanchnicvein
8. Reticuloendotheliosis diametersol <20/o (81% sensitive,100%specific)
9. Myelofibrosis r/ portalvein aneurysm
10. Feltysyndrome r/ portalvein thrombosis
11. S c h i s to s o m i a s i s r/ cavernoustransformationof portalvein
12. Cysticfibrosis r/ increasedechogenicity+ thickeningof portalvein
13. C h ro n i cm a l a ri a w al l s
734 Radiology Review Manual

DopplerUS: (d) retroperitoneal collaterals

r/ continuousmonophasicportalvenousflow pattern 1. Splenorenal / splenoadrenorenalshunt
withoutrespiratoryfluctuations 2. Gastrorenalshunt
r/ reductionof mean portalvein velocitiesto 7-12 3. Mesenterorenal shunt (betweenSMV + right
cm/sec (normally12-30 cm/sec) renalv.)
r/ loss of flow increasein portalvenoussystem 4. Mesenterogonadal shunt (betweenileocolicv.
duringexpiration + righttesticularv.)
r/ congestiveindex>0.13cm/sec (= ratioof area of 5. Splenocavalshunt (betweensplenicv. + left
portalvein dividedby flow velocity;67% sensitive) hypogastricv.)
r/ may have bidirectional/ hepatofugal(<10%)flow (e) intrahepaticshunt (portalv. to hepaticv.)
withinspontaneous splenorenal shunts(indicates
high incidenceof hepaticencephalopathy) @ Cruveilhier-von Baumgartensyndrome(20-35%)
r/ dilatedhepaticarterymay demonstrateelevated = recdholizedparaumbilicalveins (NOT recanalized
resistiveindex>0.78 umbilicalveins)
r/ hypoechoicchannelin ligamentum teres
@ Spontaneousportosystemicshunts: (a) size <2 mm (in 97% of normalsubjects;in 14o/"of
. high frequencyof hepaticencephalopathy patientswith portalhypertension)
! varices= serpontinetubularroundedstructures (b) size >2 mm @6%sensitivityfor portal
{ coronary(leftgastric)vein >5-6 mm (in 26/") hypertension)
r/ gallbladderwall varicesin thickenedgallbladderwall { arterialsignalon DopplerUS in 38%
(in 80% associatedwith portalvein thrombosis) { hepatofugal venousflow (82%sensitivity,lOC./"
specificityfor portalhypertension)
Type of Varices Frequency (%)
@ Spleen
Coronaryvenous 80-86 { splenomegaly(absencedoes not rule out portal
Esophageal 45-65 hypertension)
Paraumbilical 10-43 r/ sideroticGamna-Gandynodulesin 13/" (= smallfoci
Abdominalwall 30 of perifollicular
+ trabecularhemorrhage):
Perisplenic 30 r/ multiple3-8-mm low-intensityspots on FLASH /
Retrogastric/ gastric 2-27 GRASSimages
Paraesophageal 22 { mufiiplehyperechoicspots on US
Omental 20 { muftiplefaint calcificationson CT
Retroperitoneal-paravertebral 18 { ascites
Mesenteric 10 Cx: Acute gastrointestinal bleeding(mortalityof
Splenorenal 10 30-50% during1st bleeding)
Gastrorenal 7
SegmentalPortalHypertension
(a) connectionto SVC = Splenicvein occlusion/ superiormesentericvein
1. Esophageal varices(= subepithelial occlusion
+ submucosalveins)suppliedby anteriorbranch
of left gastricvein PortosystemicSurgicalConnections
2. Paraesophageal varices(endoscopically not A . N ON S E LE C TIVSEH U N T
visible)suppliedby posteriorbranchof coronary = decompressionof the entireportalsystemwith
(= left gastric)vein draininginto azygos increasedrisk of hepaticencephalopathy
+ hemiazygosw. + vertebralplexus 1. Portacavalshunt
0 NOT connectedto esophagealvaricesl = portalvein to IVC end-to-side/ sideto-side
r/ mediastinal/lung masson CXR in 5-8% 2. Mesocavalshunt
(b) connectionto pulmonarycirculation = syntheticgraft betweenSMV and IVC
1. Gastropulmonary shunt(betweengastric/ (a) short "H-graft"to posteriorwall of SMV
esophagealvv.and leftpericardiophrenic / (b) long "C-graft"to anteriorwall of SMV
inferiorpulmonaryw.) (c) directmesocavalshuntdividingIVC (rare)
r/ nodularityalong left cardiacborderon CXR 3. Mesorenalshunt
(c) retrogrademesentericflow 4. Mesoatrialshunt
1. Veins of Retzius(= €ln?stomoses betweenportal = polytetrafluoroethylene (PTFE)graft between
vein and IVC) anteriorwall of SMV superiorto pancreasand
- ileocolicveins- rightgonadalvein- IVC
rightatriumcoursingthroughabdomen
- pancreaticoduodenal vein - IVC + diaphragminto rightthoraciccavity
- proximalsmall left branchesof SMV - left
B . S E LE C TIV E SHUNT
gonadalvein - left renalvein = decompressionof partsof the portalsystemwith
- ileocolicveins- directlyinto IVC preservationof bloodflow to the liver
 Disorders of Liver, Biliary Tract, Pancreas, and Spleen 735

End-to-side Portocaval Side-to-sidePortocaval Mesocaval Shunt

i Splenorenal (Warren)
Shunt and Mesorenal Shunt Shunt
Surgical Portosystemic Shunts

0 Contraindicated in patientswith ascites 3. Peakbloodflowvelocitywithinproximal+ mid

1. Distalsplenorenal shunt= Warrenshunt(popular) + distalaspectsof stent
= Splenicvein to left renalvein 4. Hepaticartery: PSV, EDV,Rl

Doppler criteria for shunt patency: Pre-and post-T|PSbaselinestudyunderstablefasting

r/ increasedlocalvelocities conditions:
r/ turbulence+ severespectralbroadening Pre-TIPS Post-TIPS
r/ dilatationof recipientvein at shuntsite
r/ phasicflow patternin portaltributaries Portalvein velocity(cm/s) 10-30 40-60
r/ hepatofugalflow in intrahepaticportalvein Mean portalvein velocity(cm/s) 18 t 6 SS+ 7
branches Portalpressure(mm Hg) 37 t I 22+ 6
{ reductionin size + numberof portosystemic Shuntpeakvelocity(cm/s) 95 r 58
collaterals
{ reduction/ absenceof ascitesor splenomegaly r/ nign-vetocityturbulentflow (50-2ZOcm/s) at least
doublethat of pre-TlPSvalues
{ superimposedcardiac+ respiratoryvariations
TransjugularIntrahepatic r/ increasein hepaticarteryvelocitiesfrom77 cmls
Portosystemic
Shunt
(TtPS) (pre-TIPS)to 119 cm/s (post-TIPS)
^/ reversedflow directionwitninportalvein branches
= portaldecompressionthroughpercutaneously
Cx: (1) Shuntobstruction(38%)
established
shuntwith expandablemetallicstent
(2) Hepaticvein stenosis
betweenhepatic+ portalveinswithinthe liver
(3) Vascularinjury: hepaticartery
pseudoaneurysm, arterioportalfistula
lndication: patientswith esophageal+ gastricvariceal
(4) Intrahepatic/ subcapsularhematoma
hemorrhage/ refractoryascitesdue to
(5) Hemoperitoneum(due to penetrationof liver
advancedliverdiseasewith portal
capsule)
hypertension, hepatorenal
syndrome
rvpeorstent: (6) Transientbileductdilatation(dueto
1,1ilt,Hilr',i:3l,.fJif
ll;ii $?,.',, hemobilia)
(7) Bilecollection
stent
(8) Stentdislodgment with embolization to right
Shunt surveillance: at regular3-6-month intervalsfor
atrium,pulmonaryartery,internaljugularvein
A. MORPHOLOGY
Mortality: <2o/o(intraperitonealhemorrhage)
1. A s c it e s
2. Portosystemiccollaterals
TIPS failure
3. Size of spleen
Cause: acute thrombosis,improperstent
4. Diameterof stent(usually8-10 mm)
placement,intimalhyperplasia, hepaticvein
5. Configuration of stent:areasof narrowing
stenosis,changein stentconfiguration,
6. Extensionof stent into portal+ hepaticveins
bulgingof liverparenchymaintoshunt
B. HEMODYNAMICS
Prevalence: 31"/"at 1 year, 42ohat 2 years
1. Directionof flow in: extrahepatic
portalvein, RT . recurrentbleeding= shuntabnormality
+ LT portalvein,SMV,splenicvein,all 3 in 100%
A. >50%stenosis(in 30-80% within12 months)
hepaticveins,intrahepaticlVC, paraumbifical
r/ irregularfillingdefectsalongwall of shunton
vein,coronaryvein
color Doppler
2. Peakbloodflow velocitywithinmainportalvein
0 Pseudointimal hyperplasiais isoechoicto blood!
736 Radiology Review Manual
r/ graduatdecreasein shuntvelocityover 1-6
months(dueto intimalhyperplasia)
{ maximalshunt velocityof <60 cm/sec (>95%
sensitive+ specific)
{ in- I decreasein peak flow velocityin similar
locationwithinstent >50 cm/secrelativeto initial
baselinestudy
rl velocitytransitionzone withinstentwith flow
accelerationby a factorof 2
r/ decreasein maximalportalvein velocity>33%
from baseline
r/ reversalof portalvenousflow direction(100%
sensitive,92oh specific,71"/oPPV, 100/" NPV)
r/ loss of pulsatilityof portal/ shunt flow
{ changein flow directionin collateralveinsfrom
baseline
r/ retrogradeflow in RHV (developingstenosisof
right hepaticvenousoutflowtract)
{ developing/ worseningascites/splenomegaly
B. Occlusion
{ absentflow within shunt
r/ echogenicmaterialwithinstent
acute cause: prolongedProcedural
catheterization,leakageof bile into / around
stent
delayedcause: pseudointimal hyperplasia,
stent shorteningwith delayedstent expansion
PORTALVEINTHROMBOSIS
Etiology:
A. IDIOPATHIC(mostly):? neonatalsepsis
B. SECONDARY:
(1) Cirrhosis+ portalhypertension(5%)
(2) Malignancy:tumorinvasionby HCC,
cholangiocarcinoma, pancreaticcarcinoma,
gastriccarcinoma,metastasis/ extrinsic
compressionby tumor
(3) Trauma: umbilicalvenouscatheterization;
surgery;Cx of splenectomy(7"/",higherin
patientswith myeloproliferativedisorders)
(4) Hypercoagulable state: blooddyscrasia;clotting
disorder;estrogentherapy;severedehydration
(5) Intraperitonealinflammatoryprocess(portalvein
phlebitis):perinatalomphalitis;pancreatitis;
appendicitis;ascendingcholangitis
Age: predominantlychildren,young persons
. abdominalpain
. portalsystemicencephalopathy
. hematemesis(esophagealvarices)
Acute PortalVeinThrombosis
P lainf ilm :
{ hepatosplenomegaly
{ enlargedazygosvein
{ paraspinalvarices
UGI:
{ esophagealvarices
r/ thickeningof bowelwall
US:
{ echogenicmaterialwithinvessellumen(67%)
{ increasein portalvein diameter(57"/")
{ portosystemiccollateralcirculation(48%\
^/ enlargement of thrombosedsegment>15 mm
(38%)
^/ thickeningof lesseromentum
Doppler-US:
{ no flow on postprandial Dopplercolorscans:
0 Malignantthrombustendsto distendvein +
exhibitpulsatileflow, a blandthrombusdoes not!
{ decreasein hepaticarteryresistiveindex:
./ Rt <O.SO(in acute occlusiveportalvein
thrombosis)
r/ minimaldecrease/ normalRl (in chronicportal
veinthrombosis/ nonocclusive thrombosis)
N E C T:
{ decreasedattenuationof affectedhepatic
parenchyma(dueto edema,depletionof
hepatocytes,f ibrosis)
C E C T:
r/ transienthigh attenuationduringhepaticarterial
phase (due to increasedarterialflow)
^/ low-densitycenterof portalvein thrombus
surroundedby peripheralenhancement:
{ portalvein density20-30 HU less than aortic
density
MR:
{ absentflow void in portalarea + abnormalsignal
intensityin mainPortalvein
{ hyperintense thrombuson TlWl + T2Wl (if <5
weeks old)
./ tiltingdefecton MRA
Angio:
! "threadand streaks"sign of tumor thrombus
(streakycontrastopacificationof tumor vessels)
Cx: (1) Cavernoustransformation (19%)
(2) Hepaticinfarction
(3) Bowel infarction
GhronicPortalVeinThrombosis
Pathophysiology:
centralpart (caudatelobe + lateralsegment)is well
suppliedby collateralvenousvessels;the peripheral
zone (mainlyrightlobe)receiveslessportalvenous
blood resultingin increasedarterialflow
{ nonvisualization of extrahepaticportalvein (= fibrotic
portalvein)
r/ calcificationwithinclot / wall of portalvein
{ cavernoustransformation(= covernoma) of portal
vein:
{ presenceof a racemoseconglomerateof collateral
veinswith portalvenousflow linkingpancreas+
duodenum+ gallbladder fossa
{ splenomegaly
{ ascites
C E C T:
r/ peripheralscatteredareas of high attenuationin
liverduringhepaticarterialphase
 Disordersof Liver,BiliaryTract,Pancreas,and Spleen 737

US: . fever (65%)withoutevidenceof infection

,Vechogenic/ nonvisualized
portalvein . increasinglymphadenopathy + hepatosplenomegaly
MR: (46%)
./ hypointenseportalvein on Tl WI + hyperintense
on . weightloss (26%),abdominalpain (2G%)
T2Wl (2-18 monthsold) Location: bone marrow,lymphnodes,liver,spleen,
numerousabnormalflowvoidsin portahepatis bowel,lung,pleura,kidney,dura
Prognosis: mediansurvivaltime: 4 monthsfrom
POSTCHOLECYSTECTOMY SYNDROME diagnosisof lymphoma;14/"rateal
= SVrTtptoms
recurring
/ persisting
aftercholecystectomy remissionrate
lncidence:
mild recurrentsymptomsin g-2S%; severesymptomsin SCHISTOSOMIASIS
2.6-32% (resultof 1,930cholecystectomies): 0 Majorcause of portalhypertensionworldwide: 200
- completelycured(G1%) millionpeopleaffected
- satisfactoryimprovementwith Types:
(a) persistentmilddyspepsia(11%) A. SCHISTOSOMA MANSONI
(b) mild attacksof pain (24%) occursin >70 millioninhabitants of partsof Africa,
- failurewith
Caribbean,Arabicpeninsula,West Indies,northern
(a) occasionalattacksof severepain (S%) part of SouthAmerica
(b) continuousseveredistress(1.7%) B. SCHISTOSOMA JAPONICUM
(c) recurrentcholangitis(0.7%) coastalareas of China,Japan, Formosa,philippines,
Cause: Celebes
A . B I LI A RYCA U SE S C. SCHISTOSOMA HAEMATOBIUM
(a) lncompletesurgery in Africa,Mediterranean, SouthwestAsia
1. Gallbladder / cysticduct remnant -+ typicallyaffectsurinarytract
2. Retainedstone in cysticduct remnant(1%) Cycle:
3. OverlookedCBD stone (5%) cercariaeenter lymphatics+ blood systemvia thoracic
(b) Operativetrauma duct; larvaeare transportedinto mesentericcapillaries;
1. Bileduct stricture maturein portalsystem+ liver into worms;worms live in
2. Bile peritonitis pairsin copulawithinportalvein + tributaries for 10-15
3. Suturegranulomaof cysticduct remnant years;femaleswims againstblood flow to reachvenules
(c) Bile duct pathology of urinarybladder(S. haematobium) or intestine+
1. Fibrosisof sphincterof Oddi rectum(S. mansoni,S. japonicum);depositseggs in
2. Biliarydyskinesia wall of urinarybladderor intestines, eggs passwith
3. Biliaryfistula urine+ feces;hatchwithinwaterto releasemiracidia
4. Cysticduct mucocele which infectsnail hosts;cercariaeemergeafter
(d) Residualdiseasein neighboring structures maturationfrom snails
1. Pancreatitis lnfection: cercariaepenetratehumanskin / buccal
2. Hepatitis mucosafrom contaminatedwater (slow-
3. Cholangitis movingstreams,irrigationcanals,paddy
(e) Overlookedbile duct neoplasia fields,lakes)
B. EXTRABILIARY CAUSES(erroneouspreoperative Histo: granulomatousreaction+ fibrosisalong portalvein
diagnosis) branches
(a) Other Gl tract disease: . clinicallymildinfectionwith chroniccourse
1. lnad e q u a te d e n ti ti o n @ Liver& spleen(10%)
2. Hiatushernia r/ hepatosplenomegaly
3. Pepticulcer { portalvein dilatationin 73/" (= presinusoidal portal
4. Spasticcolon hypertension)
(b) Anxietystate,air swallowing { markeddiffusethickeningof echogenicwallsof
(c) Abdominalangina portalvenules= periportalfibrosis
(d) Carcinomaoutsidegallbladder 0 Schistosomainfectionis the most frequentcause
(e) Coronaryarterydisease of liverfibrosisworldwide!
{ normalparenchymal echogenicity+ smallperipheral
RICHTER
SYNDROME hyperechoicfoci in 50%
= developmentof largecell / diffusehistiocyticlymphoma r/ hyperechoicgallbladderbed
in patientswith CLL r/ thickenedgallbladderwall
Etiology: transformation / dedifferentiation
of CLL { peripheralseptal+ capsular"turtleback"
lymphocytes calcifications(Schistosomiasis japonicum)
lncideneein CLL patients: 3-10% @ Gl tract
Median age: 59 years { gastric+ esophagealvarices
Mediumtime intervalafterdiagnosisof CLL: 24 months r/ polypoidbowelwall masses(esp.in sigmoid)
738 Radiology Review Manual

{ granulomatouscolitis DDx: (1) Microcysticadenoma(innumerabletiny cysts,

{ strictureswith extensivepericolicinflammation olderage grouP)
Cx: ileus (2) Mucinouscysticneoplasm(largeuni-/
cysts,olderage group)
multilocular
(3) Nonfunctioningisletcell tumor (hypervascular)
SCHWACHMAN.DIAMOND SYN DROME (4) Pleomorphiccarcinomaof pancreas(smaller
= (ara probablyautosomalrecessivecondition tumorin olderPatient)
characterizedby congenitalabsenceof pancreatic (5) Pancreatoblastoma (childhoodtumor)
(6) Calcifiedhemorrhagic pseudocyst
exocrinetissue
0 2nd most frequentcause of exocrinepancreatic
insufficiency in childhoodaftercysticfibrosis! SPLENIC ANGIOSARCOMA
. pancreaticinsufficiency, steatorrhea lncidence: rare,<100 cases in literature
. recurrentrespiratoryand skin infections(secondaryto Cause: usuallynot due to thorotrastor toxic exposureto
bone marrowhypoplasia) vinylchloride/ arsenicas in liverangiosarcoma
. normalelectrolytesin sweat Age: 50-60 years
. failureto thrive . splenomegaly, abdominalPain
. tendsto improvewithtime { multiplenodulesof varyingsize usuallyenlargingthe
^/ total fatty replacementof pancreas spleen
r/ metaphysealchondrodysplasia resultingin dwarfism { solitarycomplexmass with variablecontrast
DDx: cysticfibrosis(pancreaticcalcifications, cyst enhancement
formation,abnormalsweattest) { metastasizesto liver (70%)
r/ spontaneousrupture(33%)
SO LI D A ND P A P IL L AR Y N E O P L AS M MR:
^/ focal/ diffusehypointensefoci on Tl Wl + T2Wl (iron
OF PANCREAS
= SOLIDANDCYSTIC TUMOR= PAPILLARY-CYSTIC depositionfrom hemorrhage)
NEOPLASM = SOLIDANDPAPILLARY EPITHELIAL Prognosis: 20"h survival rate after 6 months
NEOPLASM = HAMOUDI TUMOR
= r?re,low-grademalignanttumor;oftenmisclassified as
nonfunctioning isletcelltumor,cystadenoma, SPLENICHAMARTOMA
cystadenocarcinoma of pancreas = SPLENOMA
Prevalence: 0.17-2.7%of all nonendocrinepancreatic = tdtatypicallysinglenonneoplastic lesioncomposedof a
tumors mixture of normal splenicelements
Mean age: 25 (range10-74)years ; M:F = 1:9; Etiology: congenital
especiallyin blackand EastAsianpatients May be associatedwith: hamartomaselsewhereas in
Path: largewell-encapsulated mass with considerable tuberoussclerosis
hemorrhagicnecrosis+ cysticdegeneration Histo:(a) mixtureof white+ red pulp (mostcommon)
Histo: sheets+ cords of cells arrangedarounda (b) white pulp subtype= aberrantlymphoidtissue
fibrovascularstroma (c) red pulp subtype= aberrantcomplexof sinusoids
. vagueupperabdominaldiscomfortand pain . asymptomatic
. graduallyenlargingabdominalmass CT:
Location: tail of pancreas(mostfrequently) r/ attenuation equalto / hypodenseto splenictissue
r/ well-encapsulated inhomogeneousround/ lobulated r/ prolongedheterogenousenhancement
pancreaticmass with solid + cysticportions MR:
^/ may be completelycystic(whencomplicatedby r/ heterogeneously on T2Wl
hyperintense
extensivenecrosis+ internalhemorrhage) r/ diffuse heterogeneous enhancement, more
r/ ttuid-debrislevel (20%) homogeneous on delaYedimages
r/ mean diameterof 9 cm (range3-15 cm)
r/ t stippled/ punctate/ amorphousdystrophiccalcification S P LE N IC H E MA N GIOMA
(33%) Cause: congenital,arisingfrom sinusoidalepithelium
r/ hypovascularwith no contrastenhancement/ Prevalence; 0.03-14/. (autopsy); M > F
enhancementof solidtissueprojectingtowardcenterof 0 Mostcommonprimarysplenictumor!
MASS Age: 20-50 years
US: Histo: proliferation of vascularchannelslinedby single
{ echogenicmass with necroticcenter layer of endothelium;mostlyof cavernoustype;
MR: may containareasof infarction, hemorrhage,
{ highsignalintensityon Tl Wl (consistent
with thrombosis, fibrosis
hemorrhagic necrosis) Associatedwith: generalizedangiomatosisKlippel-
Prognosisj (1) excellentafter excision Tr6naunay-Webersyndrome,
(2) metastases(in 4%): omentum,tymph Beckwith-Wiedemann sYndrome,
nodes.liver Turnersyndrome
 Disordersof Liver,BiliaryTract,Pancreas,and Spleen 739

. asymptomaticlpain + fullnessin LUe

US:
r/ usuallysmallsinglelesion<4 cm, up to i7 cm in size { initiallyill-definedhypoechoiclesion(dueto
r/ foci of speckled/ snoMlakelikecalcifications inflammation,
edema,necrosis)
US: ^/ laterincreasingly
well-defined
echogeniclesion(due
{ well-marginated predominantlyhyperechoiclesion to organizationof infarctwith fibrosis)
CT: CT phases:
r/ predominantlycysticlesionis avascular (a) hyperacutephase (day 1)
! solid areas hypo-/ isodenseto normalspleenand r/ mottledarea of increasedattenuationon NECT
enhancing
, (hemorrhage)
MR: ! largefocal hyperattenuating lesionon CECT
1 nypo-/ isointenseon Tl Wl + hyperintense on T2Wl r/ mottledpatternof contrastenhancement
r/ hypointenseareasdue to hemosiderindeposits (b) acute (days 24) + subacutephase (days 4-8)
r/ progressivecentripetalenhancementwith persistent r/ focal progressivelymore well-demarcated areasof
uniformenhancement on delayedimages decreasedattenuationwithoutenhancement
NUC: (c) chronicphase (2-4 weeks)
r/ no uptakeof Tc-99msulfurcolloid ! size decreases+ attenuationreturnsto normal
Prognosis: slow growth,thus becomingsymptomaticin r/ completeresolution/ residualcontourdefect
adulthood ^/ areas of calcification
Cx: (1) Spontaneous splenicrupture(in up lo 2S%) Cx: acutefebrileillness,abscessformation,pseudocyst
(2) Kasabach-Merritt syndrome(= ?nemia, formation,splenicrupture,hemorrhage
thrombocytopenia, coagulopathy)with large
hem an g i o ma
(3) Portalhypertension
(4) Malignantdegeneration SPLENOSIS
= posttraumaticautotransplantation of splenictissueto
othersites
SPLENIC INFARCTION Age: young men with historyof trauma/ spfenectomy
0 Most commoncause of focaldefects! Time of detection: mean of 10 years (range of 6 months
Cause: to 32 years)aftertrauma
1. Embolic:bacterialendocarditis (responsiblein Location: diaphragmatic surface,liver,omentum,
50'/"),atherosclerosis with pfaqueemboli,cardiac mesentery,peritoneum,pleura(attachesto
thrombus(atrialfibrillation,leftventricular
thrombus), peritoneal/ pleuralsurface)
metastaticcarcinoma { multiplesmallencapsulated sessileimplants(few mm to
2. Localthrombosis:sicklecelldisease(leadingto 3 cm)
functionalasplenia),myelo-/ lymphoproliferative { demonstrated by Tc-99msulfurcolloid;In-111labeled
disorders(CMLmostcommon),polycythemia vera, platelets;Tc-99mheat-damagedRBC (bestdetection
myelofibrosiswith myeloidmetaplasia+ rate)
splenomegaly,Gaucherdisease,collagenvascular Significance:
disease,portalhypertension (1) protectsagainstinfectionin pediatricpatients
3. Vasculitis:periarteritisnodosa (2) may be confusedwith metastasesi lymphoma
4. Vascularcompromiseof splenicartery: focal (3) responsiblefor diseaserecurrenceaftersplenectomy
inflammatoryprocess(eg, pancreatitis), thrombus (eg, idiopathicthrombocytopenic purpura)
from splenicarteryaneurysm,splenictorsion DDx: accessoryspleen
5. Therapeuticcomplication:transcatheterhepatic
arterialembolization
mnemonic; "PSALMS"
Pancreaticcarcinoma,Pancreatitis
Sicklecell disease/ trait SPONTANEOUS
PERFORATION
OF COMMON
Adenocarcinomaof stomach BILEDUCT
Leukemia Pathogenesis;unknown(? CBD obstruction,localized
Mitralstenosiswith emboli muralmalformation,ischemia,trauma)
Subacutebacterialendocarditis Age: 5 weeks to 3 years of age
. vagueabdominaldistension
Anatomy: branchesof the splenic artery are . mildpersistenthyperbilirubinemia
noncommunicating end arteries . varyingacholicstools
. LUQ pain,fever US:
. elevatederythrocytesedimentationrate, leukocytosis r/ niliaryascites/ locutatedsubhepaticfluid
. abnormallactatedehydrogenaselevels r/ localizedpseudocholedochal cyst in porta hepatis
{ single/ multiplefocalwedge-shapedperipheraldefects Hepatobiliaryscintigraphy:
{ globalinfarction ! radioisotopediffuselythroughoutperitonealcavity
740 Radiology Review Manual

THOROTRASTOSIS (2) 2-2- nitro-4-trif Iuo ro-methy Ibenzoy|- 1,3-

Thorotrast= 25/" colloidalsuspensionof thoriumdioxide; cyclohexanedione (NTBC)inhibits4-
used as contrastagent betweenlate 1920sand mid hydroxyphenylpyruvate dioxygenase+ prevents
1950s,in particularfor cerebralangiographyand liver formationof maleylacetoacetate and
spleenimaging;chemicallyinertwith highatomic fumarylacetoacetate
numberof 90; >100,000peopleiniected (3) Livertransplantation(beforeHCC develops)
Thorium dioxide = consistsof 11 radioactive isotopes
(thorium-232is majorisotope);decayby meansof
alpha,beta,and gammaemission;biologichalf-lifeof UNDIFFERENTIATED OF LIVER
SARCOMA
1.34x 1010years;hepaticdose of 1,000-3,000rads in = EMBRYONALSARCOMA
20 years lncidence: 4th / 5th most commonlivertumor in
Distribution:phagocytosedby RES + depositedin liver PediatricPoPulation
(70'/"),spleen (30%),bone marrow, Age: <2 months(in 5%);6-10 years(in 52%);by
abdominallymph nodes (20%) 15 years(in 90%);up to 49 years; M:F = 1:1
^/ linearnetworkof metallicdensitycontrastmaterialin Histo: primitive undifferentiatedstellate/ spindle-shaped
spleen,lymphnodes,liver sarcomatous cellscloselypackedin whorls+
! spleenmay be shrunken/ nonfunctional sheets i scatteredlooselyin a myxoidground
Cx: hepaticfibrosis,angiosarcoma(50%), substancewith foci of hematopoiesis(50%)
noma,hepatocel lularcarcinoma . painfulRUQ mass and fever
cholangiocarci
(latencyperiodof 3-40 years;mean 26 years) . mild anemia+ leukocYtosis (50%)
. elevatedliverenzymes(33%)
. fever (5%)
Location: rightlobe (75%);left lobe (10%);both lobes
(15%)
TYROSINEMIA ^! z-14-21 cm in size
= f€lfeautosomalrecessivemetabolicdisorder r/ well-defined margins(fibrouspseudocapsule)
Country: increasedprevalencein Canadianprovinceof NUC:
Quebecand partsof Scandinavia ./ photodefect on sulfurcolloidscan
Biochemistry: deficiency of enzyme f umarylacetoacetase US / C T:
(laststep in catabolicpathwayof tyrosine, ^/ large intrahepaticmass with cysticareas up to 4 cm in
serummethionine, urinarysuccinylacetone); diameter(myxoidstroma+ necrosis+ hemorrhage)
elevatedlevelsof serumtyrosineas a r/ discordantfindingbetweenUS (solid)+ CT (cystlike)
precursorof dopamine,norepinephrine, A ngi o:
ep i n e p h ri n em, e l a n i nth
, Yro x i n ^1nypo-/ hypervascular with stretchingof vessels
A. ACUTEFORM { scatteredfoci of neovascularity
. fulminantliverfailure,oftenby 1 year of age Prognosis; mostlyresultsin death within 12 months
B . CHRO NI CF O R M DDx: mesenchymal hamartoma
= Fanconisyndromewith renaltubulardysfunction (a) solid lesion+ cysticdegeneration:
. vitaminD-resistantrickets hepatocellular carcinoma,fibrolamellar
. intermittentporphyria-like symptoms carcinoma,intrahepatic cholangiocarcinoma,
angiosarcoma, epithelioidhemangio-
' progressive liverfailurein earlychildhood endothelioma, othersarcomas,lymphoma,
. anemia,abnormalliverfunctiontests metastaticdisease,hepatocellular adenoma
. elevatedlevelsof cx-fetoprotein (b) solitarycysticlesion:
r/ hepatosplenomegaly biliarycystadenomaI biliarycarcinoma,cystic
{ micro-and macronodular cirrhosis(earlychildhood): degeneration of hepatocellular carcinoma,
r/ regeneratingnodulesof 2-20 mm: hyper-(mostly)/ bacterial/ parasiticabscess,metastaticdisease,
iso-/ hypoattenuating; hypo-/ occasionallyhyperechoic posttraumaticresolvinghematoma
r/ portalhypertension
^/ increasedechogenicity(fibrosis+ fatty infiltration)
./ nephromegalywith uniformlythickenedrenalcortices
r/ nephrocalcinosis WANDERING SPLEEN
PrenatalDx: enzymedeficiencydemonstrablein = ABERRANT / FLOATING / PTOTIC / DYSTOPIC
/ DRIFTING i
hepatocytes,skin fibroblasts,lymphocytes, DISPLACED / PROLAPSED SPLEEN
amniocytes = €Xc€sSively mobilespleenon an elongatedpedicle
Cx: hepatocellular carcinoma(in 37'/"beyond 2 years displacedfrom its usualpositionin LUQ
of age) Cause: embryologically absent/ malformed
Rx: (1) Dietrestrictedin phenylalanine + tyrosine gastrosplenic + splenorenalligaments;deficient
(alleviateskidneydamagebut does not prevent / lax abdominalmusculature(prune-belly
fatal outcome) syndrome,PregnancY)
 Disorders of Liver, Biliary Tract, Pancreas, and Spleen 741

Age: any (higherfrequencyin womenof childbearing elevatedresistiveindex in proximalsplenicartery

age) low attenuationwith heterogeneousenhancement
. asymptomatic mobileabdominal/pelvicmass on CT
. chronicvague lowerabdominal/ backpain
r/ whorledappearanceof twistedsplenicpedicle
. nausea,vomiting,eructation, flatulence 2 . Torsionwith arterialocclusion:hemorrhagic
. acute abdomen(withsplenicinfarctionfrom torsion)
infarction,subcapsular / intrasplenichemorrhage,
{ empty splenicfossa + associatedsofttissue mass in gangrene,degenerative cysts,functionalasplenism
centerof abdomen/ pelvis Gl complications:
r/ invertedmalpositionedstomach @ Stomach:compression, distension,volvulus,
r/ splenichilumoften locatedanteriorly tractiondiverticulum,varices
r/ displacedlargespleen(congestion duringtorsion) @ Smallbowel: dilatation, obstruction
Cx: @ Colon: compression, volvulus,laxity,ptosis
1. Torsionwith prolongedvenousocclusion:
perisplenitis,
localizedperitonitis,
adhesions,venous Rx: 1. Splenectomy(4% postsplenectomy
sepsis)
thrombosis,hypersplenism 2. Splenopexy
{ no flow withinspleenon DopplerUS 3. Conservativetreatment(if asymptomatic)
RadiologyReviewManual
 DIFFERENTIAL
DIAGNOSIS
OF GASTROINTESTINAL
DISORDERS

ACUTEABDOMEN IN CHILD low-ratebleeding

1. Intussusception 1. Inflammatory boweldisease
2. Appendicitis 2. B eni gn/ mal i gnant
tumor
3. Obstruction(previoussurgery,hernia) 3. Mesentericvarices
4. Acute gastroenteritis Rx:
5. Basilarpneumonia (1) lntraarterial
vasopressininfusion
Prognosis: 90/" initialcontrol rate; in 30%
GASTROINTESTINAL HEMORRHAGE recurrentbleeding
Moftality: approx. 10% (2) Transcatheterembolization
0 Bariumexamination shouldbe avoidedin acute Requiressuperselectivecatheterization using
bleeders! microcatheters+ microembolicagents
Source: Cx: 25Yorisk of bowel infarction+ stricture
A . UP P E RG A ST R O IN T ES T IN AHLE M O R R H A GE
= bleedingsite proximalto ligamentof Treitz
@ Esophagogastric junction Gastrointestinal Bleeding in lnfant
1. Esophageal varices(17%): 50% mortality (1) P epti cul cer
2. Mallory-Weiss syndrome(7-14%): very low (2) Varices
mortality (3) UlceratedMeckeldiverticulum
@ Stomach
1. Acutehemorrhagic gastritis(17_270/")
2. Gastriculcer(10%) Gastrointestinal Bleeding i n Chitd
3. Pyloroduodenal ulcer(17-25%) (1) Meckeldiverticulum
Mortality: <10o/oif underage 60; >S5%if over (2) Juvenilepolyp
age 60 (3) Inflammatoryboweldisease
@ Othercauses(14%): visceralarteryaneurysm,
vascularmalformation, neoplasm,vascular_
entericfistula IntramuralHemorrhage
Average mortality: B-10% A. VASCULITIS
Rx: 1. Henoch-Schonlein purpura
(1) Transcatheter embolization (methodof choice) B. TRAUMA
abundantcollateralsexceptfor postoperative C. COAGULATIONDEFECT
stomach 1. Anticoagulant therapy
(2) lntraarterialvasopressininfusion 2. Thrombocytopenia
(0.2-0.4U/min) 3. Disseminated intravascular
coagulation
Prognosis: controls 75./. of gastric mucosal D. DISEASESWITH COAGULATIONDEFECT
bleeding;high recurrencerate 1. H emophi l i a
B. LOWERGASTROINTESTINAL HEMORRHAGE 2. Leukemia,lymphoma
= bleedingsitedistalto ligamentof Treitz 3. Multiplemyeloma
@ Smallintestine 4. Metastaticcarcinoma
tumor (eg,leiomyoma,hemangioma, 5. ldiopathicthrombocytopenic purpura
metastases), ulcers,diverticula(eg, Meckel E. ISCHEMIA(oftenfatal)
diverticulum), inflammatory boweldisease . abdominalpain
(eg,Crohndisease),vascularmalformation, . melena
visceralarteryaneurysm,aortoentericfistula Site: submucosal/ intramurallmesenteric
@ Colorectal(70%) r/ "stackedcoin"/ "picketfence"appearanceof mucosal
massivebleeding folds(dueto symmetricinfiltration of submucosal
1. Diverticula (mostcommon): hemorrhage blood)
in 25"/"of patientswith diverticulosis; r/ 'thumbprinting" = roufldedpolypoidfillingdefect(due
spontaneous cessationof bleedingin to focal accumulationof hematomain bowelwall)
80%; recurrentbleedingin 25h r/ separation+ uncoilingof bowel loops
2. Colonicangiodysplasia = dilated r/ narrowingof lumen+ localizedfillingdefects
submucosalarteries+ veinsoverlying (asymmetric hematoma)
mucosalthinning(? secondaryto mucosal ^i no spasm/ irritability
ischemia) r/ mechanicalobstruction+ proximaldistensionof loops
3. Biopsy Prognosis; resolutionwithin2-6 weeks
744 Radiology Review Manual

GI ABNORMALITIES IN CHRONIC RENAL FAILURE 2. Lymphoma

3. Inflammatory boweldisease
AND RENAL TRANSPLANTATION
4. Pepticulcerdisease
@ Esophagus
B . H Y P E R TR OP H IEGAD S TR ICR U GA E
1. Esophagitis:candida,CMV, herPes
1. M6n6trierdisease
@ Stomach & duodenum
C . N ON U LC E R A TIVMU E C OS A LD IS E A S E
1. Gastritis
1. C el i acdi sease
r/ thickenedgastricfolds (38%)
2. Tropicalsprue
r/ edema+ erosions
Cause: 3. Whippledisease
(a) imbalanceof gastrinlevels+ gastricacid 4. AllergicgastroenteroPathY
5. Gastrocolic fistula
secretiondue to
(1) reducedremovalof gastrinfrom kidney 6. Villousadenomaof colon
with loss of corticalmass D. LYMPHATICOBSTRUCTION
(2) impairedacidfeedbackmechanism 1. IntestinallymPhangiectasia
(3) hYPochlorhYdria E . H E A R TD IS E A S E
(b) opportunisticinfection(eg, CMV) 1. Constrictive Pericarditis
2. Gastriculcer(3.5%) 2. TricuspidinsufficiencY
3. Duodenalulcer(2.4/")
4. Duodenitis(47%)
@ Colon
Malabsorption
More severely+ frequentlyaffectedafter renal = deficientabsorptionof any essentialfood materials
transplantation withinsmallbowel
1. Progressive distention+ pseudoobstruction
Contributingfactors: dehydration,alterationof A. PRIMARYMALABSORPTION
diet, inactivity,nonabsorbableantacids,high- = the digestiveabnormalityis the only abnormality
dose steroids present
2. lschemiccolitis 1. Celiacdisease= nontropical sprue
(a) primarydiseaseresponsiblefor end-stage 2. Tropicalsprue
renaldisease(eg,diabetes,vasculitis) 3. Disaccharidase deficiencies
(b) traumaof renaltransplantation
3. Diverticulitis B. SECONDARYMALABSORPTION
Contributingfactors: chronic obstipation, = occUrfiflgduringcourseof gastrointestinal disease
steroids,autonomicnervousdysfunction (a) enteric
4. Pseudomembranous colitis 1. W hi ppl edi sease
5. Uremiccolitis= t"tohsP€cific colitis 2. Parasites:hookworm,Giardia,fish
6. Spontaneouscolonicperforation taPeworm
Cause: nonocclusive ischemia,diverticula, 3. Mechanicaldefects:fistulas,blindloops,
duodenal+ gastriculcers adhesions,volvulus,shortcircuits
4. Neurologic:diabetes,functionaldiarrhea
@ Pancreas
1. Pancreatitis 5 . Inflammatory:enteritis(viral,bacterial,
Cause: hypercalcemia,steroids,infection, fungal,nonspecific)
immunosuppressive agents,trauma 6. Endocrine:Zollinger-Ellison syndrome
7 . Drugs: neomycin,phenindione, cathartics
@ General lupus'
1. G l hem o rrh a g e 8 . Collagendisease:scleroderma,
Cause: gastritis,ulcers,colonicdiverticula, polyarteritis
ischemicbowel,infectiouscolitis, 9 . Lymphoma
pseudomembranous colitis, 1 0 . Benign+ malignantsmallboweltumors
nonspecificcecal ulceration 1 1 .Vasculardisease
12. CHF, agammaglobulinemia, amYloid,
2. Bowelperforation(in 14% of transplantrecipignts)
intestinal
abetalipoproteinemia,
3. Opportunisticinfection
Organism;Candida,herpes,CMV, strongyloides lymphangiectasia
4. Malignancy (b) gastric
(a) skintumors vagotomy,gastrectomy,pyloroplasty,gastric
(b) lymphoma fistula(to jejunum,ileum,colon)
(c) pancreatic
pancreatitis,pancreatectomy, pancreaticcancer,
ENTEROPATHY cysticfibrosis
ng EnteroPathY
Protein-losi (d) hepatobiliary
A. DISEASEWITH MUCOSALULCERATION intra-and extrahepaticbiliaryobstruction,acute
1. Car c i n o m a + chronicliverdisease
 DifferentialDiagnosisof Gastrointestinat
Disorders 745

RoentgenographicSignsin Matabsorption Blood

r/ sIr,IRI-I-
BoWELWITHNoRMAL FoLDS+ FLUID Giardiasis
1. Maldigestion
(deficiency
of bilesalt/ pancreatic
enzymes)
2. Gastric
surgery ABDOMINAL MASS
3. Alactasia
Abdominal Mass in Neonate
^/ strlnll BowEL wtrH NoRMALFoLDS+ wET A. RENAL(55%)
1. Sprue 1. Hydronephrosis (ZS%)
2. Dermatitis
herpetiformis 2. Multicysticdysplastickidney(15%)
3. Polycystickidney
r/ oImTeD DRYSMALLBoWEL 4. Mesoblasticnephroma
1. Scleroderma 5. Renalvein thrombosis
2. Dermatomyositis B . GE N | TA L(15% )
3. Pseudoobstruction:
no peristaltic 1. Ovariancyst
activity
2. Hydrometrocolpos
r/ ourgD wET SMALLBowEL c. GASTROTNTESTTNAL (15%)
1. Sprue 1. Duplication
2. Obstruction 2. Volvulus
3. Blindloop 3. Cysticmeconiumperitonitis
4. Mesentericcyst
r/ rHtcxrNED srRAtcHTFoLDS+ DRySMALL D . N ON R E N A LR E TR OP E R TTON E(10%
AL )
BOWEL 1. Adrenalhemorrhage
1. Amyloidosis
(malabsorption 2. Neuroblastoma
is unusual)
2. Radiation 3. Teratoma
3. lschemia E. HEPATOBTLTARY (5%)
4. Lymphoma (rare) 1. Hemangioendothelioma
5. Macroglobulinemia
(rare) 2. Choledochalcyst
3. Hydropsof gallbladder
r/ rutcxrNED srRAtcHTFoLDS+ wET SMALL
BOWEL AbdominalMassin Infant& Child
1. Zollinger-Ellison
syndrome A. RENAL(55%)
2. Abetalipoproteinemia:
tareinherited
disease 1. Wilmstumor(22%)
characterized
by CNSdamage,retinal 2. Hydronephrosis (20%)
abnormalities,
steatorrhea,
acanthocytosis 3. Cysticrenal mass
4. Congenitalanomaly
r/ THIcTeNED NoDULAR IRREGULAR
FoLDS B. NONRENALRETROPERTTONEAL (23%)
+ DRYSMALLBOWEL 1. Neuroblastoma (21%)
1. Lymphoidhyperplasia 2. Teratoma
2. Lymphoma c. GASTROTNTESTTNAL (18%)
3. Crohndisease 1. Appendiceal abscess(10%)
4. Whippledisease 2. Hepatobiliary(G%)
5. Mastocytosis D . GE N | TA L(4% )
1. Ovariancyst / teratoma
r/ THIcTeNED NoDULAR IRREGULAR
FoLDS 2. Hydrometrocolpos
+ WETSMALLBOWEL
1. Lymphangiectasia
2. Giardiasis A B N OR MA L IN TR A A B D OMIN A L A IR
3. Whippledisease(rare) AbnormalAir Collection
1. Abnormallylocatedbowel
Small Bowel Nodularity with Malabsorption Chilaiditisyndrome(= colon interposedbetweenliver
mnemonic.'"Whatls HisMainAim?Lay Eggs,By and chestwall),inguinalhernia
God" 2. Pneumoperitoneum
Whipple disease 3. Retropneumoperitoneum
Intestinal
lymphangiectasia perforationof duodenum/ rectum/ ascending
Histiocytosis + descendingcolon,diverticulitis,
ulcerativedisease,
Mastocytosis endoscopicprocedure
Amyloidosis 4. Gas in bowelwall
Lymphoma, Lymphnodehyperplasia gastricpneumatosis,phlegmonous gastritis,
Edema endoscopy,ruptureof lungbulla
746 Radiology Review Manual

5. Gas withinabscess C. THROUGHFEMALEGENITALTRACTT

locatedin subphrenic,renal,perirenal,hepatic, (a) iatrogenic
pancreaticspace,lessersac 1. Perforationof uterus/ vagina
6. Gas in biliarysystem= pneumobilia 2. Culdocentesis
7. Gas in portalvenoussystem 3. Rubintest = tubal patencytest
4. Pelvicexamination
(b) spontaneous
Pneumoperitoneum 1. lntercourse,orogenitalinsufflation
Etiology: 2. D ouchi ng
A . DI S RUP T ION O F WA L LO F H O L L OWV ISC U S 3. Knee-chestexercise,waterskiing,horseback
(a) blunt/ penetrating trauma ri di ng
1. Perforating foreignbody (eg,thermometer
injuryto rectum,vaginalstimulatorin rectum) D . IN TR A P E R ITON E A L
1. Gas-forming Peritonitis
2. Compressorair directedtowardanus
(b) iatrogenicperforation 2. Ruptureof abscess
1. Laparoscopy/ laparotomy(58%): absorbed
in 1-24 days dependenton initialamountof Notet = asymptomaticspontaneous
withoutperitonitis
pneumoperitoneum
air introducedand body habitus(80%in
asthenic,25/"in obesePatients)
0 After 3 days free air shouldbe followedwith ! air in lesserperitonealsac
./ gas in scrotum(throughopen processusvaginalis)
s u s Pi c i o n !
2. Leakingsurgicalanastomosis
3. EndoscopicPerforation Largecollectionof gas:
4. E ne m ati p i n j u ry r/ abdominaldistension,no gastricair-fluidlevel
5. DiagnosticPneumoPeritoneum { "footballsign"= largepneumoperitoneum outlining
(c) diseasesof Gl tract entireabdominal cavitY
"bas-relief
1. Perforatedgastric/ duodenalulcer ri "doublewall sign"= "Riglersign"= sign"
2. PerforatedaPPendix = air on both sidesof bowelas intraluminal gas
3. Ingestedforeign-body perforation + free air outside(usuallyrequires>1,000mL of
4. Diverticulitis (rupturedMeckeldiverticulum / free intraperitonealgas + intraperitonealfluid)
sigmoiddiverticu lum,jejunaldiverticulosis) ^/ 'telltaletriangleSign"= triangularair pocket
5. Necrotizingenterocolitiswith perforation between3 looPsof bowel
6. Inflammatory boweldisease(eg,toxic { depictionof diaphragmatic muscleslips= two or
megacolon) three6-13-cm longand 8-10-mmwide arcuate
7. Obstructiont(gas traversingintactmucosa): soft-tissuebandsdirectedverticallyinferiorly
neoplasm,imperforateanus, Hirschsprung + archingparalleltodiaphragmatic dome superiorly
disease,meconiumileus { outlineof ligamentsof anteriorinferiorabdominal
8. Rupturedpneumatosis cystoidesintestinalist w al l :
with "balancedpneumoperitoneum" (= free r/ "invertedV sign"= outlineof both lateralumbilical
intraperitoneal air acts as tamponadeof ligaments(containing inferiorepigastricvessels)
pneumatosis cyststhus maintaining a balance r/ outlineof medialumbilicalligaments(obliterated
betweenintracysticair + pneumoperitoneum) umbilicalarteries)
9. ldiopathicgastricperforation = spontaneous ! "urachussign"= outlineof middleumbilicalligament
perforationin prematureinfants(congenital
gastricmuscularwall defect) RUQ gas (bestplaceto lookfor smallcollections):
r/ singlelargeareaof hyperlucency over the liver
B . T HRO UG HPE R IT ON EASU L RFACE r/ obliquelinearareaof hyperlucency outliningthe
(a) transperitonealmanipulation posteroinferiormarginof liver
1. Abdominalneedlebiopsyi catheter r/ doge'scap sign = triangularcollectionof gas in
placement Morisonpouch (posteriorhepatorenalspace)
2. Mistakenthoracentesis/ chesttube r/ outlineof falciformligament= longverticallineto
placement the rightof midlineextendingfrom ligamentum
3. Endoscopicbiopsy teresnotchto umbilicus;mostcommonstructure
(b) extensionfrom chestt outlined
1. Dissectionfrom pneumomediastinum (positive ./ ligamentumteressign = air outliningfissureof
pressurebreathing,ruptureof bulla/ bleb, ligamentumtereshepatis(= posteriorfree edge of
chest surgerY) falciformligament)seen as verticallyoriented
2. Bronchopleural fistula sharplydefinedslitlikeI ovalareaof hyperlucency
(c) ruptureof urinarybladder between1Othand 12thrib within2.5-4.0cm of right
(d) penetrating abdominalinjurY vertebralborder2-7 mm wide and 6-20 mm long
 DifferentialDiagnosisof Gastrointestinal
Disorders 747

r/ ligamentumteres notch = invertedV-shapedarea (a) intestinalobstruction :

, of hyperlucencyalong undersurfaceof liver pyloricstenosis,annularpancreas,imperforate
r/ "saddlebagI mustache/ cupolasign" = gas trapped anus,Hirschsprung disease,meconiumplug
belowcentraltendonof diaphragm syndrome,obstructingneoplasm
{ parahepaticair = gas bubblelateralto rightedge of (b) intestinal trauma:
liver endoscopy+ biopsy,biliarystent perforation,
sclerotherapy,bowelsurgery,postoperative
Pseudopneu
moperitoneu
m bowelanastomosis,penetrating/ blunt abdominal
- processmimickingfree air trauma,traumaof childabuse,intracatheter
A. ABDOMINALGAS jejunalfeedingtube, bariumenema
(a) gastrointestinalgas (c) infection/ inflammation:
1. Pseudo-wall sign = appositionof gas- pepticulcerdisease,intestinalparasites,
distendedbowel loops tuberculosis, peritonitis, inflammatory bowel
2. Chilaiditisyndrome disease(Crohndisease,ulcerativecolitis,
3. Diaphragmatic hernia pseudomembranous colitis),rupturedjejunal
4. Diverticulumof esophagus/ stomach/ diverticula,Whippledisease,systemicamyloidosis
duod e n u m C . IN C R E A S E D MU C OS A LP E R ME A B ILITY
(b) extraintestinal
gas Pathogenesis: defects in lymphoidtissue of bowel
1. Retroperitoneal air wall allowsbacterialgas to enter
2. Subdiaphragmatic abscess bowelwall
B . CHE S T (a) immunotherapy:
1. Pneumothorax graft-versus-host disease,organtransplantation,
2. Empyema bone marrowtransplantation
3. lrregularity
of diaphragm (b) others:
C. FAT AIDS enterocolitides, steroidtherapy,
1. Subdiaphragmatic intraperitoneal
fat chemotherapy,radiationtherapy,collagen
2. lnterpositionof omentalfat betweenliver vasculardisease(scleroderma,systemiclupus
+ diaphragm e rythematosus,periarteritisdermatomyositis),
intestinalbypassenteropathy,diabetesmellitus
Pneumoretroperitoneu
m D . P U LMON A R Y D IS E A S E
Cause:(1) Traumatic (usually
rupture duodenum) Pathogenesis: alveolarrupturewith air dissecting
(2) Perforationof duodenalulcer interstitiallyalong bronchovascular
(3) Gas abscessof pancreas(usuallyextends bundlesto mediastinum
into lessersac) + retroperitoneally alongvascular
(4) Urinarytract gas (trauma,infection) supplyof viscera
(5) Dissectedmediastinalair Chronicobstructive pulmonarydisease(chronic
r/ kidneyoutlinedby gas bronchitis, emphysema,bullousdiseaseof lung),
r/ outlineof psoas margin+ gas streaksin muscle asthma,cysticfibrosis,chesttrauma (barotrauma
bundles from artificialventilation,chesttube),increased
intrathoracicpressureassociatedwith retching
+ vomiting
Pneumatosis
Intestinal
is Path: (a) microvesicular type = 10-100-mmcysts/
= PNEUMATOSIS CYSTOIDES INTESTINALIS= BULLOUS bubbleswithinlaminapropria
EMPHYSEMA OFTHEINTEST]NE = INTESTINAL GAS (b) linear/ curvilineartype = streaksof gas
CYSTS= PERITONEAL LYMPHOPNEUMATOSIS orientedparallelto bowelwall
0 Attributedto at least 58 causativefactors! Location: any part of Gl tract; may be discontinuous
A . B O W E LNE C R O S IS/ GA N GR EN E with spreadto distantsites along mesentery
0 Most common+ life-threatening cause! Site: subserosa> submucosa> muscularis>
Pathogenesis: damage+ disruptionof mucosa mesentery;mesentericside >> antimesenteric
with entry of gas-formingbacteria side
into bowelwall (cystscontain50% r/ radiolucentclustersof cysts alongcontourof bowel
hydrogen= evidenceof bacterial wall (bestdemonstratedon CT)
origin) { segmentalmucosalnodularity(DDx:polyposis)
necrotizingenterocolitis,ischemia+ infarction { + pneumoperitoneum / pneumoretroperitoneum
(mesenteric thrombosis), neutropeniccolitis,sepsis, (asymptomaticlargepneumoperitoneum may persist
volvulus,emphysematousgastritis,causticingestion for monthsi years)
B . M UCO S A LD ISR U P T IO N r/ + gas in mesenteric+ portalvein
Pathogenesis: increasedintestinalgas pressure Prognosis:
leadsto overdistension and wide spectrumfrom innocuousto fatal;clinical
dissectionof gas into bowelwall outcomeimpossibleto predictbased on x-ray findings
748 Radiology Review Manual

0 Lineargas collectionsprobablyhave a more severe Locationof intraluminal lodgement:

connotation esophagus(68%),stomach(11.6"/"), smallbowel
0 Pneumatosis of the colonis likelyclinically ( 3 . 3 % )c, o l o n( 1 1 . 6 % )
insignificant D . TU MOR
0 Extentof pneumatosis is inverselyrelatedto 1. Mucoceleof appendix
severityof disease r/ crescent-shaped / circularcalcification
2. Mucinousadenocarcinoma of stomach/ colon
Appearancein Abdomenof Neonate
Soap-bubble = COLLOID CARCINOMA
1. Fecesin infantfed by mouth { smallmottled/ punctatecalcifications in
2. M ec oniumi l e u s : primarysite+ in regionallymphnode
gas mixedwith meconium,usuallyRLQ metastases,adjacentomentum,metastatic
3. M ec oniump l u g : liverfoci
gas in and aroundplug,in distribution
of colon 3. Gastric/ esophagealleiomyoma:calcifiesin 4/"
4. Necrotizing enterocolitis:submucosalpneumatosis 4. Lipoma
5. Atresia/ severestenosis:pneumatosis
6. Hirschsprung disease: Abdominal Wall Calcifications
impactedstool,sometimespneumatosis A . IN S OFTTIS S U E S
1. Hypercalcemic states
2. l di opathi ccal ci nosi s
ABDOMINAL CALCIFICATIONS & OPACITIES B . IN MU S C LE
(a) parasites:
OpaqueMaterialin Bowel = Taeniasolium
1. Cysticercosis
mnemonic.' "CHlPS"
^/ round/ slightlyelongatedcalcifications
Ghloralhydrate
2. Gui neaw orm= dracuncul i asi s
Heavy metals(lead)
{ stringlikecalcificationsup to 12 cm long
lron
(b) injectionsites
Phenothiazines
from quinine,bismuth,calciumgluconate,
Salicylates
cal ci umpeni ci l l i n
(c) myositisossificans
Diffuse Abdominal Calcifications C . I NS K I N
1. Cystadenomaof ovary 1. Soft-tissuenodules:papilloma,neurofibroma,
r/ granular,sandlikepsammomatous calcifications melanoma,nevi
2. Pseudomyxoma peritonei 2. Scar:
(a) pseudomucinousadenomaof ovary { lineardensity
(b) mucoceleof appendix 3. Colostomy/ ileostomY
3. Undifferentiatedabdominalmalignancy 4. Tattoo markings
4. Tuberculous peritonitis
{ mottledcalcificationssimulatingresidualbarium Abdominal Vascular Calcifications
5. Meconiumperitonitis A . A R TE R IE S
6. Oil granuloma 1. Atheromatous plaques
{ annular/ plaquelikecalcifications 2. Arterialcalcifications in diabetesmellitus
B. VEINS
Focal Alimentary Tract Calcifications phleboliths = calcifiedthrombus,generallyseen
A . E NT E RO L IT H S belowinterspinous line
1. Appendicolith:in 1O-15%ofacuteappendicitis 1. Normali varicoseveins
2. Stonein Meckeldiverticulum 2. H emangi oma
3. Diverticularstone C. LYMPHNODES
4. Rectalstone 1. Histoplasmosis / tuberculosis
5. Proximalto partialobstruction(eg,tuberculosis, 2. Chronicgranulomatous disease
Crohndisease) 3. Residuallymphographic contrast
B . M E S E NTE R IC
C A L C IF IC AT ION S 4. S i l i cosi s
1. Dystrophiccalcificationof omentalfat deposits
+ appendicesepiploicae(secondaryto infarction/ A B N OR MA L IN TR A A B D OMIN A L FLU ID
pancreatitis/ TB) Ascites
2. Cysts: mesentericcyst, hydatidcyst A. TRANSUDATE
3. Calcifiedmesentericlipoma (1) Cirrhosis(75%): poor prognosticsign
C. I NG E S T EDF O R EIGNBOD IES (2) Hypoproteinemia
trappedin appendix,diverticula,proximalto stricture (3) C H F
1. Calcifiedseeds+ pits (bezoar) (4) Constrictivepericarditis
2. Birdshot (5) Chronicrenalfailure
 DifferentialDiagnosisof Gastrointestinal
Disorders 749

(6) Budd-Chiarisyndrome D . GE N ITA L

B. EXUDATE 1. Rupturedovariancyst
(1) Carcinomatosis 2. Hydrometrocolpos
(2) Polyserositis E. HYDROPSFETALIS
(3) TB peritonitis 1. l mmunehydrops
(4) Pancreatitis 2. Nonimmunehydrops(usuallycardiaccauses)
(5) Meigssyndrome F. MIS C E LLA N E OU S
C. HE M O RRH AGIC / C H Y L O U SF L U ID 1. Chylousascites
2. Lymphangiectasia
Ea{y signs(accumulation in pelvis): 3. Congenitalsyphilis,trauma
ri roundcentraldensityin pelvis+ ill-defined bladder 4. ldiopathic
top
r/ thickeningof peritonealflankstripe Chylous Ascites
! space betweenproperitonealfat and gut >3 mm lN ADULTS: 1. Inflammatoryprocess (35%
Latg signs: 2. Tumor (30%
! Hellmersign = medialdisplacement of lateralliver 3. ldiopathic (23%
, margins 4. Trauma (11%
ri medialdisplacementof ascending+ descending
5. Congenital ( 1 %
colon lN CHILDREN: 1. Congenital (3e%
r/ obliterationof hepatic+ splenicangles
2. Inflammatoryprocess (15%
r/ butgingflanks 3. Trauma (12%)
r/ gray abdomen 4. Tumor ( 3%)
ri floatingcentralizedloops 5. ldiopathic (33%)
! separationof loops
FluidCollections
High-density Ascites mnemonic; "BLUSCHINGS"
1. Tuberculosis:20-45 HU; may be lower B i l oma
2. Ovariantumor Lymphocele,Lymphangioma, Lymphoma(atmost
3. Appendiceal
tumor anechoicby US)
U ri noma
Seroma
NeonatalAscites Cyst (pseudocyst,peritonealinclusioncyst)
A. GASTROINTESTINAL Hematoma(aneurysm,AVM)
(a) perforationof hollowviscus Infection,Infestation(empyema,abscess,
1. Meconiumperitonitis Echinococcus)
(b) inflammatory lesions Neoplasm(necrotic)
1. Meckeldiverticulum Gf tract(dilatedloops,ileus,duplication)
2. Appendicitis Serosa(ascites,pleuralfluid,pericardial
effusion)
(c) cyst rupture
1. Mesentericcyst Intraabdominal
Cyst in Childhood
2. Omentalcyst 1. Omentalcyst (greateromentum/ lessersac,
3. Choledochal cyst multilocular)
(d) bile leakage 2. Mesentericcyst (betweenleavesof small bowel
1. Biliaryobstruction mesentery)
2. Biliaryperforation 3. Choledochalcyst
B. PORTOHEPATIC 4. lntestinalduplication
(a) extrahepaticportalvein obstruction 5. Ovariancyst
1. Atresiaof veins 6. Pancreaticpseudocyst
2. Compressionby mass 7. Cysticrenaltumor
(b) intrahepaticportalvein obstruction 8. Abscess
1. Portalcirrhosis(neonatalhepatitis) 9. Meckeldiverticulum (communicates
with Gl tract)
2. Biliarycirrhosis(biliaryatresia) 10. Lymphangi oma
C. URINARYTRACT 11. Mesenterilcymphoma
0 Urineascites(mostcommoncause)from lower 12. Intramural tumor
urinarytract obstruction+ upper urinarytract
rupture:posterior/ anteriorurethralvalves, MECHANICAL INTESTINAL OBSTRUCTION
ureterovesical/ ureteropelvicjunction = occlusion/ constriction
of bowellumen
obstruction,renal/ bladderrupture,anterior Prevalence: 20h of acuteabdominaladmissions
urethraldiverticulum,bladderdiverticula, 80% smallbowelobstruction
neurogenicbladder,extrinsicbladdermass 20/" large bowel obstruction
750 Radiology Review Manual

Air Progressionin Neonates lntestinalObstruction in Neonate

withinminutesafterbirth . abdominaldistension
stomach
within3 hours . vomiting
entiresmallbowel
after 8-9 hours . failureto Passmeconium
sigmoidcolon
1. Duodenalatresia(50%),stenosis(40/"),web (10%)
Causeof AbsentGasin Neonate 2. Midgutvolvulus
1. Gl obstruction 3. Jejunal/ ilealatresia
in severerespiratory
ventilation
2. Mechanical 4. Meconi umi l eus
distress 5. MeconiumPlugsYndrome
gastricsuction
3. Continuous 6. Hirschsprung disease
7. Necrotizing enterocolitis
Cause of Delayed Passage of Gas in Neonate
1. TraumaticdeliverY NeoHarll OesrnucmoNwlrH Mtcnocololl
2. Septicemia 1. l l ealatresi a
3. Hypoglycemia 2. Distaljejunalatresia
4. Braindamage 3. Meconi umi l eus

Passage of Meconium Neonaral OesrnucrtoN wlrH Nonm* Cot-ot't

A. NORMAL 1. Meconi umP l ug
in 94"/owithin24 hours 2. Hirschsprung disease
in 99%within48 hours
exceptions: prematurity,severelyasphyxiated IntestinatObstruction in Infant & Child
terminfants 1. HypertrophicPYloricstenosis
B. DELAYED PASSAGE 2. Appendicitis
1. Hirschsprung disease 3. Intussusception
2. lleal/ jejunalatresia
3. Meconium ileus GastricOutletObstruction
4. Meconium plugsyndrome A. CONGEN]TALLESION
5. Colonatresia 1. Antralmucosaldiaphragm= antralweb
6. lmperforate anus 2. Gastricduplication:usuallyalonggreater
curvature,abdominalmassin infancy
3. HypertroPhic PYloricstenosis
Common Causes of Obstruction in Ghildren B. TNFLAMMATORY NARROWING
Nursery lntestinalatresia,midgutvolvulus, 1. Pepticulcerdisease:causein adultsin 60-65%
neconiumileus,Hirschsprung 2. Corrosivegastritis
disease.small bowelatresiawith 3. Crohndisease,sarcoidosis,syphilis,tuberculosis
me c o n i u mi l e u s ,m e c o n i u mP l ug C. MALIGNANTNARROWING
syndrome,smallleftcolonsYndrome, 1. Antralcarcinoma:causein adultsin 30-35%
imperforateanus,obstructionfrom 2. Scirrhouscarcinomaof pyloricchannel
duplicationcyst D. OTHERS
First3 months Hypertrophic pyloricstenosis,inguinal 1. ProlapsedantralPoIYP / mucosa
hernia,Hirschsprung disease,midgut 2. Bezoar
volvulus 3. Gastricvolvulus
*24months lleocolicintussusception 4. PostoPerative stomaledema
Childhood Appendicitis Abdominalplainfilm:
^/ largesmoothlymarginatedhomogeneousmass
Terminology: displacingtransversecolon + small bowel inferiorly
High obstruction = proximalto midileum ./ one / two air-fluidlevels
0 Rarelyneedsfurtherradiologicevaluation
. biliousvomiting(afterfirstfeeding)
. abdominaldistention DuodenalObstruction
^l few dilatedbowel loops A. CONGENITAL
Low obstruction = distalileum/ colon 1. A nnul arP ancreas
0 More difficultto accuratelylocalize 2. Peritonealbands= Ladd bands
0 Requirescontrastenemaexaminationto 3. Aberrantvessel
diagnosemicrocolon,positionof cecum,levelof B. INFLAMMATORY NARROWING
obstruction 1. Chronicduodenalulcerscar
. abdominaldistention+ vomiting 2. Acute pancreatitis:phlegmon,abscess,
. failureto pass meconium pseudocYst
r/ many dilatedintestinalloops 3. Acute cholecystitis:perforatedgallstone
 DifferentialDiagnosisof Gastrointestinat
Disorders 751

C. INTRAMURALHEMATOMA 2. Bezoar
1. Blunttrauma(accident,childabuse) 3. Gallstone
2. Anticoagulanttherapy 4. Inspissated milk
3. Blooddyscrasia 5. Bolusof Ascarislumbricoides
D. T UM O RA LN A R R O W IN G (b) afterbirth:
1. Primaryduodenaltumors 1. Meconi umi feus:
2. Tumorinvasionfrom pancreas,rightkidney, r/ microcolonin cysticfibrosis
lymphnodeenlargement 2. Meconi umi l eusequi val ent
E . E X T RI NS IC
C OMP R E SS IO N (c) other:
1. Aorticaneurysm 1. Intussuscepti on
2. Pseudoaneurysm 2. Tumor(rare): eg, lipoma
F . O T HE RS D . IN TR IN S IC B OW E LW A LL LE S ION
1. Superiormesentericarlerysyndromefrom (a) neoplasm
extensiveburns,body cast, rapidweightloss, 1. Adenocarcinoma
prolongedbed rest 2. Carcinoidtumor
2. Bezoar(in gastrectomizedpatient) 3. Lymphoma
mnemonic.' "VA BADD TU BADD' 4. Gastrointestinal stromaltumor
c hild adult (b) inflammatory lesion
Volvulus Tumor 1. C rohndi sease
Atresia Ulcer 2. Tubercufousenteritis
Bands Bands 3. Eosinophilicgastroenteritis
Annularpancreas Annularpancreas 4. Parasiticdisease
Duplication Duplication (c) vascularinsufficiency
Diverticulum Diverticulum 1. lschemia(arterial/ venousocclusion)
2. Radiationenteropathy
A bdom inalplai nfi l m : (d) intramuralhemorrhage
r/ double-bubble sign = air-fluidlevelsin stomach 1. B l unttrauma
+ duodenu m 2. Henoch-Schonlein purpura
r/ frequentlynormaldue to absenceof gas from 3. Anticoagulants
vomiting (e) strictures
1. Surgicalanastomosis
2. lrradiation
3. Potassiumchloridetablets
Jejunaland llealObstruction 4. Massivedepositionof amyloid
= SMALLBOWEL OBSTRUCTION (SBO)
Mortality: 55% (dictum:',Neverlet the sun rise or set Plainabdominalradiograph(50-66%sensitive):
on small-bowel obstruction") r/ "candycane" appearancein erect position
A. CONGENITAL = >3 distendedsmall bowel loops>3 cm with gas-
1. Jejunalatresia fluid levels( t3-5 hoursafter onset of obstruction)
,
2. lleal atresia/ stenosis r/ disparityin size betweenobstructedloops and
3. Entericduplication:locatedon antimesenteric contiguoussmallbowelloopsof normalcaliber
side,mostlyin ileum beyondsite of obstruction
4. Midgutvolvulusfrom arrestin rotation+ fixation { smallbowelpositionedin centerof abdomen
of smallbowelduringfetallife ! little/ no gas + stool in colonwith complete
5. Mesentericcyst from meconiumperitonitis: mechanicalobstructionafter 12-24 hours
locatedon mesentericside r/ "stretchsign" = et€ctilevalvulaeconniventes
6. Meckeldiverticulum completelyencirclebowellumen
B . E X T RI NS IC
BOW E LL E SION r/ "stepladderappearance"in low obstruction(the
1. Fibrousadhesions(50-75%)from previous greaterthe numberof dilatedbowelloops,the more
surgery(80%),peritonitis(1S%),congenital/ distalthe site of obstruction)
uncertaincause (5%) r/ "string-of-beads" indicateperistaltichyperactivityto
2. Her nia( 1 0 % ) overcomemechanicalobstruction
3. Volvulus r/ hyperactiveperistalsis/ aperistalsis= fatiguedsmall
4. Masses: extrinsicneoplasm(mostcommonly bowel
advancedperitonealcarcinomatosis), abscess, CAVE: little/ no gas in smallbowelfrom fluid-
aneurysm,hematoma,endometriosis distendedfoopsmay feadone to overlook
C. LUM I NA LO C C L U SION obstruction
(a) swallowed: Locationof obstruction:
1. Foreignbody: in children;mentallydisturbed/ (a) valvulaeconniventeshigh+ frequent= jejunum
disabledpatients (b) valvulaeconniventessparse/ absent= ileum
752 Radiology Review Manual
Plain abdominal radiographic categories:
1. Normal
= absenceof small intestinalgas / gas within3-4
variablyshapedloops<2.5 cm in diameter
2. Mildsmallbowelstasis
= single/ multipleloopsof 2.5-3 cm in diameter
with )3 airjluid levels
3. ProbableSBO pattern
loopswith air-
= dilatedmultiplegas-/ fluid-filled
fluid levels+ moderateamountof colonicgas
4. DefiniteSBO pattern
= clearlydisproportionate gaseous/ fluid
distensionof smallbowelrelativeto colonUGl:
r/ "snakehead"appearanc€= activeperistalsisforms
bulbousheadof bariumcolumnin an attemptto
overcomeobstruction
{ bariumappearsin colon>12 hours
:
Enteroclysisfor adhesiveobstruction
{ abruptchangein caliberof bowelwith normal
caliber/ collapsedboweldistalto obstruction
r/ stretchedfolds of normalpattern
{ angulated+ fixed bowel segment
Enteroclysis categories of SBO (Shrake):
(a) low-gradepartialSBO
= sufficientflow of contrastmaterialthrough
point of obstructionso that fold pattern
beyondobstructionis readilydefined
(b) high-gradepartialSBO
= stasis+ delay in arrivalof contrastso that
contrastmaterialis dilutedin distended
prestenoticloopwith minimalcontrastin
postobstructive loop leadingto difficultyin
definingfold patternaftertransitionpoint
(c) completeSBO
= flo Passageof contrastmaterial3'24 hours
after start of examination
CT (66% accurate,78/" specific,63% sensitive,181"/"
sensitivefor high-gradeobstruction,487osensitivefor
low-gradepartialobstructionl)
{ small boweldilatation>2.5 cm (not reliableto
distinguish from adynamicileus):
r/ "smallbowelfeces"sign = gas bubblesmixed
with particulatematterproximalto obstruction
^/ discrepantcaliberat transitionzone from dilatedto
nondilatedbowel:
r/ levelof obstructionbest determinedby relative
lengthsof dilatedversuscollapsedbowel
! passageof contrastmaterialthroughtransition
zone indicatesincompleteobstruction
DDx: adynamicileus(distension of entiresmall
bowel)
US:
r/ small bowel loopsdilated>3 cm
r/ lengthof dilatedsegment>10 cm
r/ increasedperistalsisof dilatedsegment(may
becomeparalyticin prolongedobstruction)
{ colon collapsed
Clo sed-loop Obstruction
= obstructionat two pointsalongthe courseof the
bowelat a singlesite usuallywith involvementof
mesenterY
0 Mostcommoncauseof strangulation!
Cause: adhesion(75%),incarcerated hernia
r/ fixationof bowelloop= no changein position:
r/ "coffeebean sign" = gas-filledloop
r/ "pseudotumor" = fluidjilledloop
r/ U- or C-shapeddilatedbowel loop on CT
r/ increasingintraluminal fluid
^/ "beaksign"= pointof obstruction on CT / UGI
"whirl = twisting of bowel + mesenteryon CT:
{ sign"
r/ stretchedmesentericvesselsconvergingtoward
site of obstruction/ torsion
Cx: volvulus
Strang uIated Obstruction
= impairedcirculationof obstructedsegment
Prevalence: 5-1A42% of patientswith SBO
At risk: patientswith acute complete/ high-grade
SBO; risk increasesovertime
TR IA D :
(1) closed-loopobstruction of the involvedsegment
(majorityof cases)
(2) mechanicalobstruction proximalto the involved
segment
(3) venouscongestionof the involvedloop
CT (63-100% detectionrate):
r/ slightcircumferential thickeningof bowelwall:
./ increasedwall attenuation
r/ target/ halo sign
{ serratedbeaklikenarrowingat site of obstruction
(32-100% specific)= closedloop with regional
mesentericvascularengorgement + bowelwali
thickeningat the obstructedsegment
./ unusualcourseof mesentericvasculature
^/ vascularcompromiseof affectedbowel:
{ poor/ no enhancement of bowelwall
( 1 o o %s P E C l F l c )
^l delayedprolongedenhancementof bowelwall
ri mesenterichazinessdue to edema (95/.
specific)
^/ diffuseengorgementof mesentericvasculature
r/ localizedmesentericfluid/ hemorrhage
r/ largeamountof ascites
^/ pneumatosisintestinalis
! gas in portalvein
Prognosis:
20-37% mortalityrate (comparedwith 5-8/"tor a
recentlyreducedsimpleobstruction)due to delay in
diagnosis:8/" for surgeryperformedin <36 hours,
25/o morlalityfor surgeryperformedin >36 hours
Acquired Small Bowel Obstructionin Childhood
"AAllMM"
mnemonic.'
Adhesions
Appendicitis
IntussuscePtion
lncarceratedhernia
 DifferentialDiagnosisof Gastrointestinat
Disorders 753

Malrotation 4. Hugelydistendedbladder
Meckefdiverticulum 5. Mesenteritis
6. Poorlyformedcolostomy
Small Bowel Obstruction in Adutthood (b) severeconstriction
mnemonic;"SHAVIT" 1. Volvulus(3rdmostcommoncause): sigmoid
Stone(gallstoneileus) colon,cecum,transversecolon,compound
Hernia(21%) volvulus(= ileosigmoid knot)
Adhesion(49%) 2. Hernia:transversecolonin diaphragmatic
Volvulus hernia,sigmoidcolonin left inguinalhernia
Intussusception 3. Adhesion
T um or( 16 % ) Abdominal plainlilm patterns:
(a) dilatedcolon only = competentileocecalvalve
(b) dilatedsmall bowel (25%) = incompetentileocecal
ColonicObstruction valve
lncidence: 25/" of all intestinalobstr.uctions (c) dilatedcolon + dilatedsmall bowel= ileocecal
A. NEONATALCOLONICOBSTRUCTION valve obstructionsecondaryto cecal
1. M ec on i u mp l u gs y n d ro m e overdistension
2. Colonicatresia r/ gas-fluidlevelsdistalto hepaticflexure(fluidis
3. Anorectalmalformation:rectalatresia, normalin cecum+ ascendingcolon);sign not valid
imperforateanus with diarrhea/ salinecatharsisI enema
4. Hirschsprung disease { cecummostdilatedportion(in7}%of cases);
5. Functionalcolonic immaturity (especially in criticalat 10 cm diameter(highprobability for
premies+ infantsof motherstreatedwith impendingperforation)
magnesiumor highdosesof sedatives/ opiates, 0 The lowerthe obstruction, the moreproximalthe
childrenwith septicemia,hypothyroidism, distension I
hypoglycemia, diabeticmothers) BE: Emergencybariumenemaof unpreparedcolon
- smallleftcolonsyndrome in suspectedobstruction!
- meconiumplug syndrome Contraindicated in toxicmegacolon,
B . LUM I NA LO B T U R AT ION pneumatosis intestinalis,
portalvein gas,
1. Fecalimpaction extraluminal gas
ri OuOOty patternof large mass of stool
2. Fecaloma ILEUS
3. Gallstone(in sigmoidnarrowedby diverticulitis) [ileus= stasis/ inabilityto push fluidalong(termdoes not
4. Intussusception distinguish betweenmechanicaland nonmechanical
C. BOWELWALL LESION causes)l
(a) malignant (60-70y" of obstructions): = ADYNAMIC / PARALYTIC / NONOBSTRUCTIVE TLEUS
predominantly in sigmoid = derangementimpairingproperdistalpropulsionof
(b) inflammatory intestinalcontents
1. Cr oh nd i s e a s e Cause:
2. Ulcerative colitis - in neonate:
3. Mesentericischemia 1. H yperbi l i rubi nemi a
4. Sigmoiddiverticulitis (1S%) 2. lntracranial hemorrhage
r/ stenoticsegment>6 cm 3. Aspirationpneumonia
5. Acute pancreatitis 4. Necrotizing enterocolitis
(c) infectious: 5. Aganglionosis
infectiousgranulomatousprocess - i n chi l d/ adul t:
1. Actinomycosis 1. Postoperative ileus
2. Tuberculosis . usuallyresolvesby 4th postoperativeday
3. Lymphogranulomavenereum 2. Visceralpain: obstructingureteralstone,
parasiticdisease commonbile duct stone,twistedovariancyst,
1. A m e b i a s i s bluntabdominal/ chesttrauma
2. Schistosomiasis 3. Intraabdominal inflammation
/ infection:
(d) wall hematoma: peritonitis,appendicitis,cholecystitis,
blunttrauma,coagulopathy pancreatitis, salpingitis,
abdominalabscess,
D. E X T RI NS I C hemolytic-uremic syndrome,gastroenteritis
(a) mass impression 4. lschemicboweldisease
1. E ndo me tri o s i s 5. Anticholinergic drugs: atropine,propantheline,
2. Largetumormass: prostate,bladder,uterus, morphine+.derivatives,tricyclicantidepressants,
tubes,ovaries dilantin,phenothiazines, hexamethonium
3. Pelvicabscess bromide
754 Radiology Review Manual

6. Neuromuscular disorder:diabetes, Age: neonatalperiod/ delayedfor months

hypothyroidism,porphyria,lead poisoning, + years
uremia,hypokalemia, amyloidosis, urticaria, ntestinal-hypoperistalsis
icrocolon-i
2. Megacystis-m
sprue,scleroderma, Chagasdisease,vagotomy, syndrome
myotonicdystrophy,CNS trauma,paraplegia,
quadriplegia ESOPHAGUS
7. Systemicdisease: septic/ hypovolemicshock, Contractions
Esophageal
urticaria 0 Esophagealmotoractivityneedsto be evaluatedin
8. Chestdisease: lowerlobe pneumonia,pleuritis, recumbentpositionwithoutinfluenceof gravity!
myocardialinfarction,acute pericarditis, PERISTALTTC EVENT= coordinatedcontractionsof
congestiveheartfailure esopnagus
disease: hemorrhage(spine
9. Retroperitoneal PERISTALTIC SEQUENCE= aboralstrippingwave
trauma),abscess clearingesophagus
A . P R IMA R YP E R IS TA LS IS
mnemonic.' "Rememberthe P's" = orderlyperistalticsequencewith progressive
Pancreatitis aboralstrippingtraversingentireesophaguswith
Pendicitis completeclearanceof barium;centrallymediated
Pepticulcer (medulla)swallowreflexvia glossopharyngeal
Perforation + vagalnerve;initiatedby swallowing
Peritonitis r/ rapidwave of inhibitionfollowedby slowerwave of
P neum onia contraction
Porphyria 0 Normalperistaltic sequencewill be interrupted by
Postoperative repetitiveswallowingbeforeperistalticsequenceis
Potassiumdeficiency completel
Pregnancy B . S E C ON D A R YP E R IS TA LS IS
Pyelonephritis = localperistalticwave identicalto primary
peristalsisbut elicitedthroughesophageal
. intestinalsoundsdecreasedi absent distension= SerSoIirT'lotor stretchreflex
. abdominaldistension 0 Esophagealmotilitycan be evaluatedwith barium
ri large+ smallbowel+ gastricdistension injectionthroughnasoesophageal tube despite
rl decreasedsmall bowel distensionon serialfilms patient'sinabilitYto swallow!
r/ delayedbut free passageof contrastmaterial C. TERTIARYCONTRACTIONS
Rx: not amenableto surgicalcorrection = nohPfoPulsive esophagealmotorevent
characterizedby disorderedup-and-down
Localizedlleus movementof boluswithoutclearingof esophagus
= isolateddistendedloop of smallI largebowel Cause:
= SENTINEL LOOP 1, PresbyesoPhagus
Often associatedwith: adjacentacute inflammatory 2. DiffuseesoPhageal sPasm
process 3. HYPeractive achalasia
Etiology: 4. Neuromuscular disease:
1. Acutepancreatitis: duodenum,jejunum, diabetesmellitus,parkinsonism,amyotrophic
transversecolon lateralsclerosis,multiplesclerosis,thyrotoxic
2. Acutecholecystitis: hepaticflexureof colon mYoPathY, mYotonicdYstroPhY
3. Acuteappendicitis: terminalileum,cecum 5. Obstruction of cardia:
4. Acutediverticulitis: descendingcolon neoplasm,distalesophagealstricture,benign
5. Acuteureteralcolic: Gl tractalongcourseof lesion,S/P rePairof hiatalhernia
ureter 0 Tertiaryactivitydoes not necessarilyimplya
significantmotilitydisturbance !
lntestinal Pseudoobstruction Age: in 5-1 0h of normaladultsduring
A . T RA NS I EN TPS EU D OOB ST R U C T IO N 4th-6th decade
1. Electrolyte imbalance (a) nonsegmental = partialluminalindentation
2. Renalfailure Location: in lower213of esoPhagus
3. Congestiveheartfailure { spontaneousrepetitivenonpropulsive
B . CHRO NICPS EU D OOB ST R U C T IO N contraction
1. Scleroderma { "yo-yo"motionof barium
2. Amyloidosis { "corkscrew"appearance= scalloped
PS EU D OOB ST R U C T IO N
C. I DI O P A TH IC configuration of bariumcolumn
"shishkebab"configuration
1. Chronicintestinalpseudoobstruction syndrome { "rosarybead"/
. persistently decreasedperistalsis+ clinical = colnPdftmentalization of bariumcolumn
obstruction r/ no lumen-obliterating contractions
 DifferentialDiagnosisof Gastrointestinat
Disorders 755

(b) segmental= luminalobliteration (rare) 5. B ul barpol i omyel i ti s

tr "Curling"= errdti6SegmentalCOntraCtiOnS 6. Cerebrovascular disease
{ "rosary-bead" appearance 7. Huntingtonchorea
8. Ganglioneuromatosis
AbnormalEsophagealperistalsis 9. Wilsondisease
A. PRIMARYMOTILITYDISORDERS 10. Friedreichataxia
1. Achalasia 11. Familialdysautonomia (Riley-Day)
2. Diffuse esophageal spasm 12. Stiff-mansyndrome
. severeintermittentpain while swallowing
r/ compartmentalization of esophagusby DiffuseEsophageal
Dilatation
numeroustertiarycontractions = ACHALASIA PATTERN = MEGAESOPHAGUS
Dx: extremelyhigh pressureson manometry A. ESOPHAGEALMOTIL]TYDISORDER
3. Presbyesophagus 1. fdiopathicachalasia
4. Chalasia 2. Chagasdisease:patientscommonlyfrom
5. CongenitalTE fistula SouthAmerica;often associatedwith
6. lntestinalpseudoobstruction megacolon+ cardiomegaly
B. SECONDARYMOTILITYDISORDERS 3. Postvagotomysyndrome
(a) connectivetissuedisease 4. Scleroderma
1. S c le ro d e rm a 5. Systemiclupuserythematosus
2. SLE 6. Presbyesophagus
3. Rheumatoidarthritis 7. Ehlers-Danlos syndrome
4. Polymyositis 8. Diabetici alcoholicneuropathy
5. Dermatomyositis L Anticholinergic drugs
6. Musculardystrophy 10. ldiopathicintestinalpseudoobstruction
(b) chemical/physicatinjury = degeneration of innervation
1. Reflux/ pepticesophagitis 11. Amyloidosis:associatedwith macroglossia,
2. S/P vagotomy thickenedsmallbowelfolds
3. Causticesophagitis 12. Esophagitis
4. Radiotherapy B. DISTALOBSTRUCTION
(c) infection 1. Infiltrating
lesionof distalesophagus/ gastric
- fungal: candidiasis cardia(eg, carcinoma)= pseudoachalasia
- parasitic: Chagasdisease
2. Benignstricture
- bacterial: TB, diphtheria
3. Extrinsiccompression
- viral: herpessimplex mnemonic,' "MA'STACO in a SHell"
(d) metabolicdisease Musculardisorder(eg,myastheniagravis)
1. Diabetesmellitus Achalasia
2. Amyloidosis Scleroderma
3. Alcoholism Trypanosomiasis (Chagasdisease)
4. Electrolytedisturbances Amyloidosis
(e) endocrinedisease Carcinoma
1. Myxedema Obstruction
2. Thyrotoxicosis Stricture(lye,potassium,tetracycline)
(f) neoplasm Hiatalhernia
(g) drug-related
atropine,propantheline, curare Air Esophagogram
(h) muscledisease 1. N ormalvari ant
1. Myotonicdystrophy 2. Scleroderma
2. Musculardystrophy 3. Distalobstruction:tumor,stricture,achalasia
3. Oculopharyngeal dystrophy 4. Thoracicsurgery
4. Myastheniagravis (disturbedmotilityonly in 5. Mediastinal inflammatory
disease
striatedmuscleof upper 1/3 of esophagus) 6. S/P total laryngectomy(esophagealspeech)
r/ persistentcollectionof bariumin'uppLrthirO 7. Endotrachealintubation+ PEEP
of esophagus
r/ findingsreversedby cholinesteraseinhibitor AbnormalEsophageal
Folds
edrophonium(Tensilon@) A. TRANSVERSEFOLDS
(i) neurologicdisease 1. Felineesophagus
1. P ar k i n s o n i s m frequentlyseen with gastroesophageal reflux;
2. Multiplesclerosis normallyfoundin cats
3. CNS neoplasm { transientcontractionof longitudinally
oriented
4. Amyotrophiclateralsclerosis muscularismucosae
756 Radiology Review Manual

2. Fixedtransversefolds Ulcer
Esophageal
due to scarringfrom refluxesophagitis A . P E P TIC
{ stepladderappearancein distalesophagus 1. Refluxesophagitis:scleroderma
B . LO NG I T U D IN AL FOLDS 2. BarrettesoPhagus
normal: 1-2 mm wide,bestseen in collapsed 3. Crohndisease
esophagus 4. Dermatologic disorders:benignmucous
rl >3 mm with submucosaledema/ inflammation membranepemphigoid, epidermolysisbullosa
1. Gastroesophageal reflux dystrophica, Behgetdisease
2. Opportunistic infection B . IN FE C TIOU S
3. Causticingestion 1. H erpes
4. lrradiation 2. Cytomegalovirus
DDx: (1) Varices C. CONTACTINJURYi EXTERNALINJURY
./ tortuous/ serpentinefolds that can be 1. Corrosives:alkali,stricturesin 50/"
effacedby esoPhagealdistension 2. Alcohol-induced esoPhagitis
(2) Varicoidcarcinoma 3. Drug-induced esoPhagitis
r/ tixed rigidfoldswith abruptdemarcation 4. Radiotherapy:smoothstricture>4,500rads
due to submucosalsPread r/ shallow/ deep ulcersconformingto radiation
Portal
5. Nasogastrictube
Inflammation
Esophageal r/ elongatedstricturein middle+ distal 1/3
A. CONTACTINJURY 6. EndoscoPicsclerotheraPY
(a) refluxrelated D . MA LIGN A N T
1. Pepticulcerdisease 1. Esophageal carcinoma
2. BarrettesoPhagus
Location:
3. Scleroderma (PatulousLES)
4. Nasogastric intubation @ UpperesoPhagus
1. Barrettulcerin isletsof gastricmucosa
(b) caustic
1. F ore i g nb o d y @ Midesophagus
1. HerPesesoPhagitis
2. Corrosives
2. CMV esoPhagitis
(c) thermic
3. Drug-inducedesoPhagitis
Habitualingestionof excessivelyhot meals/
liquids @ Distalesophagus
1. RefluxesoPhagitis
B . RA DI A T I O NIN J U R Y
2. CMV esoPhagitis
C. INFECTION
DDx:
1. Candid i a s i s
(1) Sacculation
2. Herpessimplexvirus/ CMV = outpouchingin distalesophagusdue to
3. Diphtheria
asymmetricscarringin refluxesophagitis
D. SYSTEMICDISEASE
(2) Esophagealintramuralpseudodiverticula
(a) dermatologicdisorders
. blisteringof skin + mucousmembranesin (3) Artifact
of barium
(a) tiny preciPitates
responseto minortrauma
(b) transientmucosalcrinklingin inadequate
1. Epidermolysis bullosadystrophica
distension
Histo: intraePidermal bullae
(c) irregularZ-line
2. Benignmucousmembranepemphigoid
= f?Ie diseaseof unknowncause
Histo: subepidermalbullaewithout Small EsophagealUlcer (<1 cm)
acantholysis 1. Herpes virustYPe
simPlex I
Age: 4th decade;M < F 2. Drug-induced
{ esophageallesions(in 2-13%) most 3. RefluxesoPhagitis
frequentat sites of relativestasis(aortic 4. Behgetsyndrome
knob,carina,GE junction): 5. Benignmucousmembranepmephigoid
^/ tfrinsmoothwebs arisingfrom anterior 6. Acuteradiationchange
asPect
{ stenosesof variablelength Large Esophageal Ulcer (>1 cm)
3. Pemphigusvulgaris 1. Cytomegalovirus
(b) others: vi rus
2. H umani mmunodefi ci encY
1. Cr o h nd i s e a s e 3. Carcinoma
2. Graft-versus-host disease 4. Drug-induced
3. Behgetdisease 5. BarrettesoPhagus
4. Eosinophilicgastroenteritis for varices
6. SclerotheraPY
 DifferentialDiagnosisof Gastrointestinal
Disorders 757

Double-barrelEsophagus Long SmoothEsophagealNarrowing

1. Dissectingintramuralhematomafrom emetogenic 1. Congenitalesophagealstenosis
injury { at junctionbetweenmiddle+ distalthird
2. Mallory-Weisstear { weblike/ tubularstenosisof 1 cm in length
trauma,esophagoscopy (in 0.ZS%),bougienage(in 2. Surgicalrepairof esophagealatresia
0.5%),ingestionof foreignbodies,spontaneous r/ interruptionof primaryperistalticwave at
(bleedingdiathesis) anastomosis
3. Intramuralabscess r/ secondarycontractionsmay produceretrograde
4. Intraluminal
diverticulum flow with aspiration
5. Esophageal duplication(if communication
with r/ impactionof food
esophageallumen present) 3. Causticburns= alkalineburns
4. Alendronate(= inhibitorof osteoclasticactivity)
5. Gastricacid: reflux,hyperemesisgravidarum
6. Intubation:reflux+ compromiseof circulation
Esophageal
Diverticulum 7. Radiotherapy for esophagealcarcinoma;tumorof
1. Zenkerdiverticulum (pharyngoesophageal) lung,breast,or thymus;lymphoma;metastasesto
2. Interbronchial diverticulum mediastinal lymphnodes
= tractiondiverticulum
Onset of stricture: usually4-8 monthspost Rx
responseto pullfrom fibrousadhesionsfollowing Dose: 3,000-5,000rad
lymphnode infection(TB),containsall 3 esophageal
8. Postinfectious: moniliasis(rare)
layers
Location: usuallyon rightanterolateralwall of Lower EsophagealNarrowing
interbronchial segment mnemonic.' "SPADE"
r/ calcifiedmediastinalnode! Scleroderma
3. Interaorticobronchial diverticulum Presbyesophagus
= thoracicpulsiondiverticulum Achalasia;Anticholi
nergics
.Location:
on leftanterolateral wall betweeninferior Diffuseesophagealspasm
borderof aorticarch + upper marginof left Esophagitis
main bronchus
4. Epiphrenic diverticulum (rare)
Location: usuallyon lateralesophagealwall, right> FocalEsophagealNarrowing
left,in distal10 cm 1. E sophageal w eb
r/ often associatedwith hiatushernia = 1-2-mmthick(verticallength)area of complete/
5. Intramuralesophagealpseudodiverticulosis incompletecircumferential
narrowing
r/ outpouching from mucosalglands 2. R i ng
= 5-10-mmthick(verticallength)areaof completei
incompletecircumferential
narrowing
Tracheobronchoesophageal
Fistula 3. Stricture
A . CO NG E NI T AL = >10 mm in verticallength
1. Congenitaltracheoesophageal fistula
B. MALIGNANCY(in o0%) mnemonic; "LETTERSMC"
1. Lungc a n c e r Lye ingestion
2. Metastasesto mediastinallymph nodes Esophagitis
3. Esophagealcancer Tumor
0 In 5-1 0h of patientswith advanced Tube (prolongednasogastric
intubation)
esophagealcancer Epidermolysis bullosa
4. Radiationtreatmentof mediastinal malignancy Radiation
C. TRAUMATIC Surgery,Scleroderma
1. Instrumentation (esophagoscopy, bougienage, Moni l i asi s
pneumaticdilatation) Congenital
2. Blunt ("crushinjury")/ penetratingchesttrauma
3. Surgery Midesophageal Stricture
4. Foreign-bodyperforation 1. Barrettesophagus
5. Corrosives 2. Radiationinjury
6. Postemeticrupture= Boerhaavesyndrome 3. Causticesophagitis
D. I NF E CT I OU /SIN F L A M M AT OR Y 4. Primarycarcinoma:squamouscell carcinoma
1. TB, syphilis,histoplasmosis, actinomycosis, 5. Metastaticcancer(fromsubcarinalnodes/ left
Crohndisease mainstembronchus)
2. Perforateddiverticulum 6. Drug-induced stricture(esp.potassiumchloride)
3. Pulmonarysequestration/ cyst 7. Esophagealintramuralpseudodiverticulosis
758 Radiology Review Manual
8. Dermatologicdisorder:benignmucousmembrane
pemphigoid,epidermolysisbullosa
disease
9. Graft-versus-host
Long DistalEsophageal Stricture
A. SEVERE ACIDEXPOSURE
1. Nasogastric intubation
2. Zollinger-Ellison syndrome
3. Alkalinerefluxesophagitis
B. INFLAMMATION
1. Cr ohnd i s e a s e
Short DistalEsophagealStricture
1. Refluxesophagitis
2. Carcinoma(adenocarcinoma)
3. Crohndisease
4. Schatzkiring
Esophageal Filling Defect
A . B E NI G NT U MOR S
<1ohof all esophagealtumors
(a) Submucosaltumor(75%)
= nonePithelial,intramural
1. Leiomyoma(50%of all benigntumors)
0 Mostcommonsubmucosalmass in
esophagus
2. Granularcell myoblastoma
3. Lipoma,fibroma,lipoma,fibrolipoma,
myxofibroma, hamartoma,hemangioma,
lymphangioma, neurofibroma, schwannoma,
rl primarywave stops at levelof tumor
r/ proximalesophagealdilatation
+ hypotonicity
./ rigid'esophagealwall
at site of tumoral
implant
./ disorganizedi altered/ effacedmucosal
folds arounddefect
r/ tumor shadowon tangentialview extending
beyondesophagealmargin
(b) Mucosaltumor(25%)= epithelial,intraluminal
1. Squamous papilloma
= ITloStcommonbenignmucosaltumor;rarely
multiple(esophageal papillomatosis)
{ smatlsessileslightlylobulatedpolyp
3. Fibrovascular polyp
Path: fibrovascular+ adiposetissue
Location:cervicalesophagusnear
cricopharyngeus
{ giantsausage-shaped intraluminalmass
Cx: regurgitationinto larynxcausessudden
death
2. Inflammatory esophagogastric polyp
= sentinelpolyp= bulboustip of thickened
gastricfold
Cause: sequelaeof chronicreflux
esophagitis
Prognosis: no malignantPotential
3. Adenoma
= originatesin Barrettmucosa
{ sessile/ pedunculated PoIYP
Cx: malignantdegeneration
4. Glycogenacanthosis
B. MALIGNANTTUMORS
1. Esophageal cancer
(a) squamous/ varicoidsquamouscell carcinoma
(b) adenocarcinoma
(c) spindlecell carcinoma:leiomyosarcoma,
carcinosarcoma, Pseudosarcoma
3. Carcinomaof cardia(gastriccancer)
4. Metastases:malignantmelanoma,lymphoma
lymphomas),stomach,
(<1"/"of gastrointestinal
lung,breast
C. VASCULAR
1. Varices
D . IN FE C TION / IN FLA MMA TION
1. Candida/ herpesesoPhagitis
2. Drug-induced inflammatoryreaction
E. CONGENITAL/ NORMALVARIANT
1. Prolapsedgastricfolds
2. Esophageal cYst
duPlication
(0.5-2.5%of all esophagealtumors)
F. FOR E IGNB OD IE S
1. Retainedfood particles(chickenbone,fish bone,
pins,coins,smalltoYs,meat)
2. Undissolvedeffervescentcrystals
3. Air bubbles
MucosalNodules/ Plaques
Esophageal
plaque = discreteirregular/ ovoid elevationbarely
protrudingabove mucosalsurface
nodule = srnollmore roundedelevation
1. Candidaesophagitis
2. Refluxesophagitis(earlystage)
3. Barrettesophagus
4. Glycogenacanthosis
5. Superficialspreadingcarcinoma
6. Artifacts(undissolvedeffervescentagent,air
bubbles,debris)
ExtrinsicEsophageallmpression
Cervical Causesof Esophageal Impression
A . OS S E OU SLE S ION S
1. Anteriormarginalosteophyte/ DISH
2. Anteriordisk herniation
3. Cervicaltrauma+ hematoma
4. Osteomyelitis
5. Bone neoPlasm
B. ESOPHAGEALWALL LESIONS
(a) muscle
1. Cricopharyngeus
2. Esophageal web
(b) vessel
1. Pharyngeal venousPlexus
2. Lymphnodeenlargement
 Differential Diagnosis of GastrointestinalDisorders 759

C. E NDO C R IN EOR G A N S Pancreatic

cancer: stomach/ duodenal
1. Thyroid/ parathyroid enlargement(benign/ sweep
malignant) Coloniccancer: greatergastriccurvature
2. Fibrotictractionafterthyroidectomy curvature
sreater
sastric
D. Retropharyngeal / mediastinalabscess B. BENIGN
,S#il:;icake:
(a) epithelial/ mucosaltumor(50%)
ThoracicCausesof EsophagealImpression 1. Hyperplastic polyp
A. NORMALINDENTATIONS 2. Adenomatouspolyp
aorticarch,left mainstembronchus,leftinferior 3. Brunnerglandhyperplasia
pulmonaryvein,diaphragmatic hiatus (b) mesenchymal tumor (50%)
B. ABNORMALVASCULATURE 1. Lei omyoma
right-sidedaorticarch, cervicalaorticarch, aortic 2. Ectopicpancreaticrest
unfolding,aortictortuosity,aorticaneurysm,
doubleaorticarch ("reverseS"), coarctationof MesenchymalTumors of GI Tract
aorta ("reversefigure3"), aberrantrightsubclavian A . S OMA TICS OFTTIS S U ETU MOR
artery (a) smoothmuscletumor
= arlerialusoria(semilunar/bayonet-shaped 1. True l ei omyoma
imprintuponposteriorwall of esophagus), 2. True leiomyosarcoma
aberrantleft pulmonaryartery(betweentrachea (b) neuraltumor
+ esophagus), anomalouspulmonaryvenous 0 4% of all benigngastrictumors
return(anterior), persistenttruncusarteriosus 1. S chw annoma
(posterior) 2. Neurofibroma
C. CA RDI A CC AU SE S 3. Plexosarcoma
(a) enlargement of chambers (c) lipocytictumor
leftatrial/ leftventricularenlargement: 1. Lipoma(2-3% of all benigngastrictumors)
mitraldisease(esophageal displacement 2. Liposarcoma
backward+ to the right) (d) vascular/ perivasculartissue
(b) pericardialmasses Q 2A of all benigngastrictumors
pericardialtumor/ cyst i effusion 1. Gl omustumor(mostcommon)
D. M E DI A S T IN AC L AU SE S 2. H emangi oma
mediastinaltumor,lymphadenopathy (metastatic, 3. Lymphangioma
tuberculous), inflammation, cyst B. GASTROINTESTINAL STROMALTUMOR
E . P ULM O N A R Y C AU SE S = S P IN D LE
C E LL/ E P ITH E LIOID
TU MOR
pulmonarytumor,bronchogenic cyst,atypical Origin: interstitialcell of Cajal
pulmonaryfibrosis(retraction) 0 Largestcategoryof primarynonepithelial
F. ESOPHAGEALABNORMALITIES neoplasms
1. Esophageal diverticulum
2. Paraesophageal hernia Calcified Gastric Tumor
3. Esophageal duplication 1. Mucinousadenocarcinoma: miliary/ punctate
2. Stromaltumors: amorphouscalcificatiens
3. Hemangioma:clustersof phleboliths
STOMACH
GastricTumor CongenitalGastricObstruction
Classiticationbasedon Biologic Behavior A. COMPLETEOBSTRUCTION
A . M A L|G N A N T(1 0 -1 5 % ) 1. Gastric atresia
1. Adenocarcinoma (>95%) Frequency; <'1"/" of all Gl obstructions
2. Lymphoma,mucosa-associated lymphoid May be associatedwith: epidermolysisbullosa
tissue (MALT) Site: antrum+ pylorus
3. Sarcoma: leiomyosarcoma, Kaposisarcoma . regurgitation of bile-freevomituswithinfirstfew
4. Carcinoidtumor hoursafter birth
5. Metastasis r/ "singlebubble"appearanceof air in stomach
(a) hematogenous:malignantmelanoma, r/ membranousmucosaldiaphragm
breastcancer 2. Congenitalperitonealbands
! one / moresubmucosalmasses 3. Annularpancreatictissue
r/ target/ bull's-eyelesionif centrally B. PARTIALGASTRICOUTLETOBSTRUCTION
ulcerated . cyclictransientpostprandial vomiting
r/ giant cavitatedlesion 1. Incompleteprepyloricdiaphragm
^/ tinitisplastica(usuallyin breastcancer) 2. Antralstenosis
(o) 3. Aberrantpancreatictissuein gastricantrum
1''T:i?J,?'J:1,",,'
rundus
sastric 4. Antralduplication cyst
760 Radiology Review Manual

WidenedRetrogastricSpace Pathophysiology: reflex paralysis

. abdominaldistension
A. PANCREATICMASSES(mostcommoncause)
. vascularcollapse(decreasedvenous return)
1. Acute+ chronicpancreatitis
. vomiting
2. Pancreaticpseudocyst
3. Pancreaticcystadenoma+ carcinoma r/ largestomachfilledwith air + fluid(up to 7,500mL)
B. OTHERRETROPERITONEAL MASSES { retentionof barium
1. S ar c om a ! absenti diminishedperistaltic activity
2. Renaltumor,adrenaltumor { patulouspylorus
3. Lymphnodeenlargement { frequentlydilatedduodenum
4. Abscess,hematoma DDx: gastricvolvulus,pyloricstenosis
C. GASTRICMASSES A. ACUTEGASTRICATONY
1. Leiomyoma,leiomyosarcoma (may developwithin 24-48 hours)
D. OTHERS 1. Acutegastricdilatation:secondaryto decreased
1. Aorticaneurysm arterialperfusion(ischemia,congestiveheart
2. Choledochal cyst failure)in old patients,usuallyfatal
3. Obesity 2. Postsurgicalatony,ureteralcatheterization
4. Postsurgicaldisruptions+ adhesions 3. lmmobilization: body cast,paraplegia,
5- Ascites postoPerativestate
6. Gross hepatomegaly+ enlargedcaudatelobe 4. Abdominaltrauma: especiallyback injury
7. Herniainvolvingomentum 5. Severepain: renal/ biliarycolic,migraine
headaches,severeburns
6. Infection:peritonitis,pancreatitis,appendicitis,
GaswithinStomachWall
A . NO NI NF EC T IO U S subphrenicabscess,sePticemia
1. Interstitial gastric emphysema B . C H R ON ICGA S TR ICA TON Y
- gas accumulation in submucosa/ subserosa/ 1. Neurologicabnormalities:braintumor,bulbar
or both poliomyelitis, vagotomy,tabes
2. Muscularabnormalities:scleroderma, muscular
Cause: air from an extrinsicsource
(a) obstructive(due to raisedintragastric dystroPhY
pressure):gastricoutletobstruction, 3. Drug-induced atony: atropine,morphine,heroin,
volvulus,overinflation duringgastroscopy, ganglionicblockingagents
profuseseverevomiting 4. Electrolyteimbalance:diabeticketoacidosis,
(b) pulmonary(due to rupture+ dissectionof hypercalcemia, hypocalcemia, hypokalemia,
subpleuralblebsin bullousemphysema hepaticcoma,uremia,mYxedema
alongesophagealwalli mediastinum): 5. Diabetesmellitus= gastroparesis diabeticorum
pulmonaryemphysema (0.08%incidence)
(c) traumatic(dueto mucosaltrauma): 6. Emotionaldistress
instrumentation of stomach,recent 7. Leadpoisoning
gastroduodenal surgery,endoscopy(1.6%) 8. Porphyria
. benignclinicalcoursewith spontaneous
resolution Narrowing of Stomach
r/ linearlucencyconformingto contourof a thin- = linitis plasticatype of stenosis
walleddistendedstomach A. MALIGNANCY
2. Cystic pneumatosis 1. Scirrhousgastriccarcinoma(involvingportion/
= PNEUMATOSIS CYSTOIDES INTESTINALIS all of stomach)
Cause: similarto interstitialgastricemphysema 2. H odgki nl ymphoma, NHL
. little/ no gastrointestinal symptoms 3. Metastaticinvolvement(carcinomaof breast,
r/ multiple1-2-mm gas-filledcystsin wall of pancreaticcarcinoma,coloniccarcinoma)
stomachand intestines B. INFLAMMATION
B . I NF E CT I O U S 1. Chronicgastriculcerdiseasewith intensespasm
1. Emphysematous gastritis 2. Pseudo-Billroth-lpatternof Crohndisease
predisposing:corrosivegastritis,acid ingestion, 3. Sarcoidosis
severenecrotizinggastroenteritis, r/ polypoidappearance,pylorichypertrophy
gastriculcerdiseasewith r/ gastriculcers,duodenaldeformity
intramuralperforation,gastric 4. Eosinophilic gastritis
carcinoma,volvulus,gastric 5. Polyarteritisnodosa
infarction 6. Stenosingantralgastritis/ hypertrophicpyloric
stenosis
GastricAtony C . IN FE C TION
= gastricretentionin the absenceof mechanical 1. Tertiarystageof sYPhilis
./ absentmucosalfolds + peristalsis
obstruction
 Differential Diagnosis of GastrointestinalDisorders 761

{ no changeover years B . IN FLA MMA TION / IN FE C TION

2. Tuberculosis (rare) 1. Granul oma
r/ hyperplasticnodules/ ulcerativelesion/ (1) Foreign-body granuloma
annularlesion (2) Sarcoidosis
r/ pyloricobstruction,may cross into duodenum (3) Crohndisease
3. Histoplasmosis (4) Tuberculosis
4. Actinomycosis (5) Histoplasmosis
5. Strongyloidiasis 2. Eosinophificgastritis
6. P hlegm o n o u s g a s tri ti s 3. Tertiarysyphilis: infiltrative/ ulcerative/
7. Toxoplasmosis tumoroustype
D. T RA UM A 4. Echinococcalcyst
1. Corrosivegastritis C. PANCREATICABNORMALITIES
2. Radiationinjury 1. Ectopicpancreas
3. Gastricfreezing 2. Annularpancreas
4. Hepaticarterialchemotherapyinfusion 3. Pancreaticpseudocyst
E . O T HE RS D . D E P OS ITS
1. Perigastric adhesions(normalmucosa,no 1. A myl oi d
intervalchange,normalperistalsis) 2. Endometriosis
2. Amyloidosis 3. Localizedhematoma
3. Pseudolyrnphoma E . OTH E R S
4. Exogastricmass (hepatomegaly, pancreatic 1. V ari ces(i e,fundal )
pseudocyst) 2. Duplications (4/" of all Gl tractduplications)
mnemonic; "SLIMRAGE"
Scirrhouscarcinomaof stomach
Lymphoma
Infiltrationfrom adjacentneoplasm
Metastasis(breastcarcinoma) GastricFillingDefects
Radiationtherapy A . IN TR IN S ICW A LLLE S ION S
Acids (corrosiveingestion) (a) benign(mostcommon)
Granulomatous disease(TB,sarcoidosis, Crohn) 1. Polyps: hyperplastic, adenomatous, villous,
Eosinophilicgastroenteritis hamartomatous(Peutz-Jeghers syndrome,
Cowdendisease)
Antral Narrowing 2. Leiomyoma
mnemonic; "SPICER" 3. Granulomatous lesions
Sarcoidosis,Syphilis (1) Eosinophilic granuloma
Pepticulcerdisease (2) Crohndisease
Infection(tuberculosis,
chronicgranulomatous (3) Tuberculosis
diseaseof childhood) (4) Sarcoidosis
Cancer(linitisplastica),Crohn disease,Caustic 4. Pseudolymphoma = benignreactive
ingestion proliferationof lymphoidtissue
Eosinophilicgastritis 5. Extramedullary hematopoiesis
Radiation 6. Ectopicpancreas
7. Gastricduplicationcyst
Intramural-extramucosal
Lesionsof Stomach 8. Intramuralhematoma
{ sharplydelineatedmarginal/contourdefect L Esophagogastric herniation
r/stretchedfolds over intactmucosa (b) malignant
{ acuteangleat margins 1. Gastriccarcinoma,lymphoma
{ may ulceratecentrally 2. Gastricsarcoma:leiomyosarcoma,
{ may becomepedunculatedand acquirepolypoid liposarcoma, leiomyoblastoma
appearanceover years 3. Gastricmetastases:melanoma,breast,
A. NEOPLASTIC pancreas,colon
1. Leiom y o ma (48% B . E X TR IN S ICIMP R E S S IONON
S S TOMA CH
2. Neur og e n itu
c mo rs (1 4 % in 70% nonneoplastic (extrinsicpseudotumorsin 20%)
3. Heterotopicpancreas (12% (a) normalorgans: organomegaly, tortuousaorta,
4. Fibroustumor (11% heart,cardiacaneurysm
5. Lipoma (7% (b) benignmasses:
6. Hem ang i o ma (7% cystsof pancreas,liver,spleen,adrenal,kidney;
7. G lom ust u mo r (rare gastricduplication,postoperativedeformity
8. Carcinoid (eg, Nissenfundoplication)
9. Metastatictumor (c) malignantmasses: enlargedceliacnodes
762 Radiology Review Manual

(d) inflammatory lesion: 2. Crohndisease

left subphrenicabscess/ hematoma 3. Sarcoidosis
- lateraldisplacement:enlargedliver,aortic 4. Infectiousgastritis:
aneurysm,enlargedceliacnodes bacterialinvasion,bacterialtoxinsfrom botulism,
- medialdisplacement:splenomegaly, mass in diphtheria, dysentery,typhoidfever,anisakiasis,
colonicsplenicflexure,cardiomegaly, TB , syphi l i s
subphrenicabscess 5. Pseudolymphoma
C. I NT RA LUM IN AGLA ST R ICMA SS ES B . MA LIGN A N C Y
1. B ez oar 1. Lymphoma
2. Foreignbodies: food,pills,bloodclot,gallstone 2. Gastriccarcinoma
D. TUMORSOF ADJACENTORGANS C . IN FILTR A TIVP ER OC E S S
1. Pancreatic carcinoma+ cystadenoma 1. E osi nophi l gastri
ic ti s
2. Livercarcinoma 2. Amyloidosis
3. Carcinomaof gallbladder D . P A N C R E A TIC D IS E A S E
4. Coloniccarcinoma 1. Pancreatitis
5. Renalcarcinoma 2. Directextensionfrom pancreaticcarcinoma
6. Adrenalcarcinoma E . OTH E R S
7. Lymphnodeinvolvement syndrome
1. Zol l i nger-E l l i son
E . T HI CK E N EDG A ST R ICF OL D S 2. M6n6trierdisease
3. Gastricvarices
FillingDetectof GastricRemnant mnemonic: "ZEALVOLUMESCaP3"
A . I A T RO G EN IC syndrome
Zol l i nger-E l l i son
surgicaldeformity/ plicationdefect,suturegranuloma Amyloidosis
B. INFLAMMATORY Lymphoidhyperplasia
bile refluxgastritis,hyperplastic polyps Varices
C. I NT US S U S C E PT ION Operativedefect
1. Jejunogastric intussusception Lymphoma
(efferentloop in 75o/",afferentloop in 25%) Ulcerdisease(peptic)
(a) acuteform: high intestinalobstruction,left M6n6trierdisease
hypochondriac mass,hematemesis Eosinophilic gastroenteritis
(b) chronicI intermittentform: may be self- Syphilis
reducing Crohn disease,Carcinoma,Corrosivegastritis
{ "coiledspring"appearanceof gastricfilling Pancreatitis,Pancreaticcarcinoma,
defect Postradiationgastritis
2. Gastrojejunal/ gastroduodenalmucosalprolapse
. often asymptomatic Bull's-eyeLesions
. bleedingpartialobstruction A . P R IMA R YN E OP LA S MS
D. NEOPLASTIC 1. Leiomyoma,leiomyosarcoma
1. Gastricstumpcarcinoma:>5 yearsafter 2. Lymphoma
resectionfor benigndisease;15% within10 3. Carcinoid
years;20/" after20 years 4. Primarycarcinoma
2. Recurrentcarcinoma(10%)secondaryto B. HEMATOGENOUS METASTASES
incompleteremovalof gastriccancer 1. Mal i gnantmel anoma
3. Malignancyat anastomosis(incomplete ^/ usuallyspareslargebowel
resection) 2. Breastcancer(15%)
E. INTRALUMINAL MATTER: bezoar ^/ scirrhousappearancein stomach
mnemonic; "PUBLICS" 3. Cancerof lung
Polyp(hyperplastic polypdue to bile reflux) 4. Renalcell carcinoma
Ulcer(anastomotic) 5. Kaposisarcoma
Bezoar,Blind loop syndrome 6. Bladdercarcinoma
Loop (afferentloop syndrome) C. ECTOPICPANCREAS
lntussusception at gastrojejunostomy in duodenum/ stomach
Cancer (recurrent,residual,de novo) D . E OS IN OP H ILICGR A N U LOMA
Surgicaldeformity,Suturegranuloma most frequentlyin stomach

ThickenedGastricFolds of Postoperative
Complications Stomach
A . I NF LA M MA T IO/NIN F EC T IO N 1. Fillingdefectof gastricremnant
1. Inflammatory gastritis: 2. Retainedgastricantrum
alcoholic,hypertrophic, antral,corrosive, 3. Dumpingsyndrome
postirradiation,gastriccooling 4. Afferentloop syndrome
 DifferentialDiagnosisof Gastrointestinal
Disorders 763

Billroth I Billroth II Whipple Roux-en-Y

Left-to-Right=Isoperistaltic Right-to-Left=Antiperistaltic
Vertical Silastic Vertical Ring Roux-en-Y
Ring Gastroplasty Gastric Bypass Loop-typeGastroenterostomy

Roux-en-Y Gastrojejunostomy Lc
Loop Gastrojejunostomy
Shoemaker Retrocolic (Polya) GastricBypass
Gastric SurgicalProcedures

5. Stomalobstruction LesionsInvolvingStomachand Duodenum

(a) temporaryreversible:edemaof sutureline, = Transpyloriclnvolvement
abscess/ hematoma,potassiumdeficiency, 1. Lymphoma:in up Io 4Q'/.of patientswith lymphoma
inadequateelectrolytereplacement, 2. Gastriccarcinoma: in 5-25o/".but 50 x more
hypoprotei
nemia,hypoacidity commonthan lymphoma
(b) late mechanical:stomalulcer (75%)
3. Pepticulcerdisease
mnemonic; "LOBULATING" 4. Tuberculosis:in 10o/"ofgastricTB
Leaks (early) 5. Crohndisease:pseudo-Billroth-l pattern
Obstruction(early) 6. Strongyloidiasis
Bezoar gastroenteritis
7. Eosinophilic
Ulcer(especially marginal)
Loop (afferentloop syndrome) DUODENUM
Anemia (macrocyticsecondaryto decreasedintrinsic GongenitalDuodenalObstruction
factor) . bile-stained
vomiting delayeduntilafterfirstfeeding
Tumor(? increasedincidence) progressively
andincreasing
Intussusception r/ "doublebubble"sign
Not feelingwell aftermeals(dumpingsyndrome) 1. Duodenal atresia/ severestenosis
Gastritis(bile reflux) 2. Annular pancreas
764 Radiology Review Manual

3. Midgutvolvulus E . V A S C U LA RD IS OR D E R
4. Duodenalweb duodenalvarices,mesentericarterialcollaterals,
5. Ladd bands intramuralhemorrhage(trauma,Schonlein-Henoch
6. Preduodenalportalvein purpura),chronicduodenalcongestion(congestive
7. Duodenalduplicationcyst heartfailure,portalvenoushypertension);
lymphangiectasia
ExtrinsicPressureEffecton Duodenum F. H Y P OP R OTE IN E MIA
A. BILEDUCTS nephroticsyndrome,M6n6trierdisease,protein-
normalimpression, cyst
dilatedCBD, choledochal losingenteropathy
B . G A LLB LAD D E R G. GLA N D U LA RE N LA R GE ME N T
normalimpression, gallbladderhydrops,Courvoisier Brunnergland hyperplasia,cysticfibrosis
phenomenon, gallbladdercarcinoma,pericholecystic
AbSCCSS
mnemonic.' "BAD HELP"
C. LI V E R Brunnerglandhyperplasia
hepatomegaly,hypertrophiedcaudatelobe, Amyloidosis
anomaloushepaticlobe,hepaticcyst,hepatictumor Duodenitis(Z-Esyndrome,PePtic)
D. RI G HTK I D N E Y Hemorrhage
bifidcollectingsystem,hydronephrosis, multiple Edema,Ectopicpancreas
renalcysts,polycystickidneydisease,hypernephroma Lymphoma
E . R I G H TA D R E N A L Pancreatitis,Parasites
adrenalcarcinoma,enlargementin Addisondisease
F. GOLON
duodenocolic appositiondue to anomalous Duodenal Filling Defect
peritonealfixation,carcinomaof hepaticflexure A . E X TR IN S IC
G . V E S S E LS gallbladder impression, gas{illed
CBD impression,
lymphadenopathy, duodenalvarices,dilatedarterial diverticulum
collaterals, aorticaneurysm,intramural / mesenteric B . IN TR IN S IC TO W A LL
hematoma (a) benignneoplasticmass
1. Adenoma
WidenedDuodenalSweep 2. Leiomyoma
A. NORMALVARIANT 3. Lipoma
L ES IO N
B . P A NCR E AT IC 4. Hamartoma(Peutz-Jeghers syndrome)
1. Acutepancreatitis 5. ProlapsedantralPolyp
2. Chronicpancreatitis 6. Brunnerglandadenoma
3. Pancreaticpseudocyst 7. V i l l ousadenoma
4. Pancreaticcarcinoma 8. lsletcelltumor
5. Metastasisto pancreas 9. Gangliocytic paraganglioma
6. Pancreaticcystadenoma (b) malignantneoplasticmass
C. VASCULARLESION 1. Carcinoidtumor
1. Lymphnodeenlargement: lymphoma, 2. Adenocarcinoma
metastasis,
inflammation 3. Ampullarycarcinoma
2. Cysticlymphangiomaof the mesentery 4. Lymphoma
D. RETROPERITONEAL MASS 5. Sarcoma
1. Aorticaneurysm 6. Metastasis(stomach,pancreas,gallbladder,
2. Choledochalcyst colon,kidney,melanoma)
7. Retroperitoneal lymphnodeinvolvement
Thickened Duodenal Folds (c) nonneoplastic mass
A. INFLAMMATION 1. Papillaof Vater
(a) withinbowelwall: 2. Choledochocele
pepticulcerdisease,Zollinger-Ellison syndrome, 3. Duplication cyst
regionalenteritis,lymphoidhyperplasia, uremia 4. Pancreaticpseudocyst
(b) surrounding bowelwall: 5. Duodenalvarix
pancreatitis,cholecystitis 6. MesentericarterYcollaterals
B . I NF E CT ION 7. lntramuralhematoma
giardiasis,TB, strongyloidiasis, celiacdisease 8. Adjacentabscess,stitchabscess
C. NEOPLASIA 9. Ectopicpancreas,heterotopicgastric
lymphoma,metastasesto peripancreatic nodes mucosa
D IS O R D E R
D. DI F F US EIN F IL T R A T IVE 10. Prolapsedantralmucosa
Whippledisease,amyloidosis, mastocytosis, 11. Brunnerglandhyperplasia
eosinophilicenteritis,intestinallymphangiectasia 12. Benignlymphoidhyperplasia
 DifferentialDiagnosisof Gastrointestinal
Disorders 765

C. I NT RA LU MIN AL DuodenalNarrowing
1. B loodc l o t A. DEVELOPMENTAL ANOMALIES
2. Foreignbody: fruitpit,gallstone,feedingtube 1. Duodenalatresia
2. Congenitalweb / duodenaldiaphragm
3. Intraluminaldiverticulum
DuodenalTumor 4. Duodenalduplication cyst
Benign Duodenal Tumors 5. Annularpancreas
1. Leiom y o m a (27% 6. Midgutvolvulus,peritonealbands(Laddbands)
2. Adenomatouspolyp (21o/" B. INTRINSIC DISORDERS
3. Lipoma (21% (a) inflammation / infection
4. Brunnerglandadenoma ( 1 7 % 1. P ostbul barul cer
5. Angiomatous tumor (6% 2. Crohndisease
6. Ectopicpancreas (2% 3. Sprue
7. Duodenalcyst (2% 4. Tuberculosis
8. Neurofibroma (2% 5. Strongyloidiasis
9. Hamartoma (2% (b) tumor
duodenal/ ampullarymalignancy
C. DISEASEIN ADJACENTSTRUCTURES
Malignant Duodenal Tumors 1. Pancreatitis,pseudocyst, pancreaticcarcinoma
1. Adenocarcinoma (73%) 2. Cholecystitis
Location:40/"in duodenum,mostoftenin 2nd 3. Contiguousabscess
+ 3rd portion= periampullary neoplasm 4. Metastasesto pancreaticoduodenal nodes
(a) suprapapillary:apt to cause (lymphoma,lungcancer,breastcancer)
obstruction+ bleeding D. TRAUMA
(b) peripapillary:extrahepaticjaundice 1. Duodenalrupture
(c) intrapapillary:
Gl bleeding 2. Intramuralhematoma
Predisposed;Gardnersyndrome,celiacdisease E. VASCULAR
May be associatedwith: peutz-Jegherssyndrome 1. Superiormesentericarterysyndrome
r/ annular/ polypoid/ ulcerative 2. Aorticoduodenal fistula
Metastases: regionallymph nodes (2/3) 3. Preduodenal portalvein (anteriorto descending
DDx: (1) Primarybifeduct carcinoma duodenum)
(2) Ampullarycarcinoma
2. Leiomyosarcoma (14%) DilatedDuodenum
most often beyond 1st portionof duodenum Megaduodenum = rT'tdfk€d dilatationof entire C-loop
{ up to 20 cm in size Megabulbus = dilatationof duodenalbulb only
r/ frequentlyulceratedexophyticmass A. VASCULARCOMPRESSION
3. Carcinoid(11%)
superiormesenteric arterysyndrome, abdominal
4. Lymphoma(2%)
aorticaneurysm,aorticoduodenal fistula
! markedwall thickening B. PRIMARYDUODENALATONY
./ Outt<y
periduodenallymphadenopathy (a) scleroderma,dermatomyositis, SLE
(b) Chagasdisease,aganglionosis, neuropathy,
surgical/ chemicalvagotomy
Enlargement
of Papillaof Vater (c) focal ileus: pancreatitis,cholecystitis,peptic
A. Normalvariant ulcerdisease,trauma
identifiedin 60% of UGI series;atypicallocationin (d) alteredemotionalstatus,chronicidiopathic
3r d por t iono f d u o d e n u mi n B/" ;1 .5 c m i n d iameter intestinalpseudoobstruction
in 1"/"of normals C. INFLAMMATORY / NEOPLASTICINDURATION OF
B. Papillaryedema ME S E N TE R IC R OOT
1. lmpactedstone Crohndisease,tuberculousenteritis,pancreatitis,
2. Pancreatitis(Poppelsign) pepticulcerdisease,strongyloidiasis, metastatic
3. Acuteduodenalulcerdisease disease
4. Papillitis D . FLU IDD IS TE N S ION
C. Perivaterianneoplasms celiacdisease,Zollinger-Ellison syndrome
= tumor mass + lymphaticobstruction
1. Adenocarcinoma PostbulbarUlceration
2. Adenomatouspolyp (premalignantlesion)
1. Benignpostbulbarpepticulcer
^/ irregularsurface* eroiions
{ medialaspectof upper2nd portion
D. Lesionssimulatingenlargedpapilla r/ incisurapointingto ulcer
1. Benignspindlecell tumor
{ occasionally bariumrefluxintocommonbileduct
2. Ectopicpancreatictissue
{ ring stricture
766 Hadiology Review Manual

r/ stress-and drug-induced ulcershealwithout 6. Giantduodenalulcer

deformity
2. Zollinger-Ellisonsyndrome SmallBowelUlcer
r/ multipleulcersdistalto duodenalbulb Aphthous Ulcersof Small Bowel
r/ thickeningof folds + hypersecretion A . IN FE C TION
3. Leiomyoma (25%)
1. Yersiniaenterocolitis
4. Malignanttumors: 2. Salmonellosis
(a) primaries 3. Tuberculosis
adenocarcinoma, lymphoma,sarcoma 4. Rickettsiosis
(b) contiguousspread B. INFLAMMATION
pancreas,colon,kidney,gallbladder 1. Crohndisease(22%)
(c) hematogenousspread 2. Behgetsyndrome
melanoma,Kaposisarcoma 3. Reitersyndrome
(d) lymphogenic spread 4. Ankylosingspondylitis
metastasesto periduodenallymph nodes
5. Granulomatous disease:Crohndisease,TB Large NonstenoticUlcersof Small Bowel
6. Aorticoduodenal fistula ulcer 47"/"incidence
1. Primarynonspecific
7. Mimickers:ectopicpancreas,diverticulum 2. Yersiniosis 33%
3. Crohndisease 30/o
4. Tuberculosis 18%
SMALL BOWEL 5. Salmonellosis/ shigellosis 7o/o
AnatomicPredilectionfor IntestinalInvolvement 6. Meckeldiverticulum 5%
jejunum diverticulosis,
@ proximal giardiasis,
adenocarcinoma,WhiPPle MultipleSmallBowel Ulcers
disease,ZE syndrome,celiac A . D R U GS
disease 1. Potassiumtablets
@ distalileum TB,infectious
Crohndisease, 2. Steroids
enteritis,lymphoma, 3. Nonsteroidal antiinflammatorydrugs
carcinoid,metastases B. INFECT]ON/ INFLAMMATION
@ mesentericborder Crohndisease,
diverticulosis, 1. BacillarydysenterY
mesenterichematoma, 2. lschemicenteritis
spreadof tumor
intraperitoneal 3. Ulcerativejejunoileitisas complicationof celiac
@ antimesentericborder Meckeldiverticulum, disease
sacculationsin scleroderma, C . TU MOR
hematogenousmetastases 1. N eopl asms
2. IntestinallYmPhoma

IncreasedFluidwithinSmallBowel Cavitary Small Bowel Lesion

1. lnges t ion TUMOR
A. PRIMARY
2. Resection/ removalof stomach 1. Lymphoma(exoenteric form)
3. Small-bowelobstruction 2. Leiomyosarcoma(exoentericform)
4. Enteritis 3. Primaryadenocarcinoma
5. Malabsorption:celiacdisease,Whippledisease B. METASTASIS
6. Peritonealcarcinomatosis mel anoma
1. Mal i gnant
2. Lungcancer
C. INFLAMMATION
SmallBowelDiverticula with abscess(Meckel,jejunal)
1. Diverticulitis
A. TRUEDIVERTICULA 2. Communicating cyst
duplication
(a) Duodenaldiverticula
1. Racemosediverticula:bizarre,lobulated
2. Giantdiverticula Separationof BowelLooPs
diverticula:resultof congenital
3. Intraluminal A. INFILTRATIONOFBOWELWALL/ MESENTERY
web / diaphragm (a) inflammation / infection
(b) Jejunaldiverticulosis 1. Crohndisease
(c) Meckeldiverticulum 2. TB
B . P S E UDOD IVE R T ]C U L A 3. RadiationinjurY
1. Scleroderma 4. Retractilemesenteritis
2. Crohndisease 5. lntraperitoneal abscess
3. Lymphoma (b) deposits
4. Mesentericischemia 1. Intestinalhemorrhage / mesentericvascular
5. Communicating ilealduplication
 DifferentialDiagnosisof Gastrointestinal
Disorders 767

occlusion C . V A S C U LA RC OMP R OMIS E

2. Whippledisease 1. Mesentericischemia(atheroscferosis)
3. Amyloidosis 2. Acute radiationenteritis
(c) tumor 3. Amyloidosis
1. Carcinoidtumor: localreleaseof serotonin 4. SLE
responsible for muscularthickening+ D. BOWELWALL DESTRUCTION
fibroplasticproliferation= desmoplastic 1. Lymphoma
reaction 2. Scleroderma(smoothmuscleatrophy)
2. Primarycarcinomaof smallbowel(unusual 3. Dermatomyositis
presentation)
3. Lymphoma
4. Neurofibromatosis AbnormalSmallBowelFolds
Abnormal Folds + lncreasedIntraluminal Fluid
B . A S CI T E S/ IN T R AP ER IT O N EABLL EE D IN G 1. Malabsorption syndrome
hepaticcirrhosis(75/"), peritonitis, peritoneal 2. Crohndisease,infectiousenteritis
carcinomatosis,congestiveheartf aiIure, constrictive 3. Parasiticinfestation
/ giardiasis
pericarditis,primary/ metastaticlymphaticdisease 4. lschemiaproximalto an obstruction
C. EXTRINSICMASS 5. Zollinger-Ellison
syndrome
1. Intraperitoneal spreadof tumor: peritoneal 6. Lymphangiectasia, mesentericlymphadenopathy
mesothelioma, mesenterictumors(fibroma,
lipoma,fibrosarcoma,leiomyosarcoma, Thickened Folds of Stamach & Small Bowel
malignantmesentericlymphoidtumor,
1. Lymphoma
metastases)
2. Crohndisease
2. lnterloopabscesses/ loculatedfluidcollection
3. Eosinophilicgastroenteritis
3. Endometriosis
4. Zollinger-Ellison
syndrome
4. Retractilemesenteritis(fibrosis,fatty infiltration,
5. M6n6trierdisease
pannicu l i ti s )
6. Cirrhosis= gastricvarices+ hypoproteinemia
5. Mesentericfat deposits
7. Amyloidosis
6. Fibrofattyproliferation:Crohndisease,
8. Whippledisease
mesentericpanniculitis
9. Systemicsclerosis
NormalSmallBowelFolds& Diarrhea Thickened Smooth Folds t Dilatation
1. Pancreaticinsufficiency
A . E D E MA
2. Lactasedeficiency
(a) hypoproteinemia
3. Lymphoma/ pseudolymphoma
1. C i rrhosi s
2. Nephroticsyndrome
DilatedSmallBowel& NormalFolds 3. Protein-losing enteropathy(celiacdisease,
mnemonic.' "SOS" Whippledisease)
Sprue (b) increasedcapillarypermeability
Obstruction 1. Angioneurotic edema
Scleroderma 2. Gastroenteritis
A . E X CE S S I V E
F L U ID (c) increasedhydrostaticpressure
(a) mechanicalobstruction 1. Portalvenoushypertension
due to adhesion,hernia,neoplasm (d) Zollinger-Ellison syndrome
r/ "string-of-beads sign" = air bubblesbetween B . H E MOR R H A GE
mucosalfoldsin a fluid-filled smallbowel (a) vesselinjury
r/ "pseudotumor sign"= closed-loopobstruction 1. l schemi a
(b) malabsorptionsyndromes 2. Infarction
1. Celiacdisease,tropical+ nontropical sprue 3. Trauma
2. Lactasedeficiency (b) vasculitis
B. BOWELWALL PARALYSIS 1. Connectivetissuedisease
= functionalileus= adynamicileus
2. Henoch-Schonlein purpura
1. Surgicalvagotomy 3. Thrombangitisobliterans,irradiation
2. Chemicalvagotomyfrom drug effects: (c) hypocoagulability
atropine-like substances,morphine,L-dopa, 1. Hemophilia
glucagon 2. Anticoagulanttherapy
3. Chagasdisease 3. Hypofibrinogemia
4. Metabolic:hypokalemia, diabetes 4. Circulating anticoagulants
5. Intrinsic+ extrinsicintraabdominal inflammation 5. Fibrinolyticsystemactivation
6. Chronicidiopathicpseudoobstruction 6. ldiopathicthrombocytopenic purpura
768 Radiology Review Manual

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 DifferentialDiagnosisof Gastrointestinat
Disorders 769

7. Coagulation defects(leukemia,lymphoma, Amyloidosis, Agammaglobulinemia

multiplemyeloma,metastaticcarcinoma) Mastocytosis,Malabsorption
8. Hypoprothrombinemia Soft-tissueneoplasm(carcinoid,lipoma)
C. LYMPHATICBLOCKAGE
1. T um o ri n fi l tra ti o nl y: mp h o ma , TetheredFolds
pseudolymphoma = indicativeof desmoplasticreaction
2. lrradiation r/ kinking,angulation,tethering,separationof bowel
3. Mesentericfibrosis loops
4. Intestinallymphangiectasia 1. C arci noi d
5. Whippledisease 2. Postoperativein Gardnersyndrome
D. DE P O S IT S 3. Retractilemesenteritis
1. E os in o p h i l e
i cn te ri ti s 4. Hodgkindisease
2. Pneumatosisintestinalis 5. Peritonealimplants
3. Amyloidosis 6. Endometriosis
4. Abetalipoproteinemia 7. Tuberculous peritonitis
5. Crohndisease L Mesothelioma
6. Graft-versus-host disease 9. Postoperativeadhesions
7. lmmunologic deficiency:hypo-/
dysgammaglobulinemia Atrophyof SmallBowelFolds
1. Chronicmalabsorption: celiacdisease
Thickenedlrregular Foldst Dilatation 2. Chronicischemicchanges: radiationinjury,
A. INFLAMMATION amyloidosis
1. Crohndisease 3. Crohndiseasein burned-outstage
B. NEOPLASTIC 4. Parasiticinfestation:strongyloidiasis
1. Lymphoma,pseudolymphoma 5. Graft-versus-hostdisease
C. I NF E CTIO N
(a) protozoan Ribbonlike Small Bowel
giardiasis, strongyloidiasis, hookworm = featureless/ tubularnatureof small bowelwith
(b) bacterial effacementof folds
Yersiniaenterocolitica, typhoidfever,
tuberculosis 1. Graft-versus-hostdisease
(c) fungal: histoplasmosis 2. Celiacdisease
(d) AIDS-relatedinfection 3. Small bowel infection(eg, viral enteritis)
D. I DI O P A T H IC 4. Injuryfrom radiation/ corrosivemedication
(a) lymphaticdilatation 5. Allergy(eg,soybeans)
1. Lymphangiectasia 6. lschemia
2. Inflammatory process,tumorgrowth, 7. Amyloid,mastocytosis
irradiationfibrosis 8. Lymphoma,pseudolymphoma
3. Whippledisease 9. Crohndisease
(b) cellularinfiltration
1. Eosinophilic enteritis DelayedSmallBowelTransit
2. Mastocytosis = transittime >6 hours
(c) deposits mnemonic; "SPATSDlD"
1. Z o l l i n g e r-E l l i ssoynn d ro m e Scleroderma
2. Amyloidosis Potassium(hypokalem ia)
3. Alpha chaindisease: defectivesecretory Anxiety
lgA system Thyroid(hypothyroidism)
4. A-B-lipoproteinemia: recessive,retinitis Sprue
pigmentosa,neurologicdisease Diabetes(poorlycontrolled)
5. A-a-lipoproteinemia ldiopathic
6. Fibrocysticdiseaseof the pancreas Drugs(opiates,atropine,phenothiazine)
7. Polyposissyndrome
ConstrictingLesionof SmallBowel
mnemonic.' "G. WILLIAMS' 1. Primaryadenocarcinoma (proximaljejunum)
Giardiasis 2. Carcinoid(distalileum)
Whippledisease,Waldenstrommacroglobulinemia 3. Lymphoma,metastasis
lschemia 4. Endometriosis
Lymphangiectasia 5. Adhesion,mucosaldiaphragm
Lymphoma 6. Strictures:Crohndisease,radiationenteritis,
Inflammation ischemia,potassiumchloridetablets
770 Radiology Review Manual

Multiple Stenotic Lesionsof Small Bowel 3. Kaposisarcoma

1. Crohndisease 4. Submucosalmetastases:melanoma> lung>
2. End-stageradiationenteritis breast> choriocarcinoma > kidney> stomach,
3. Metastaticcarcinoma uterus,ovary,Pancreas
4. Endometriosis D . IN TR A LU MIN A L
5. gastroenteritis
Eosinophilic 1. Gal l stones
6. Tuberculosis 2. Foreignbodies,food particles,seeds,pills
7. Drug-induced (eg,potassiumchloridetablets, 3. Parasites:ascariasis,strongyloidiasis,
NSAIDS) hookworm,taPeworm

SmallBowelFillingDefects Sandlike Lucenciesof SmallBowel

Solitary Filling Defectof Small Bowel 1. Waldenstrommacroglobulinemia
A. INTRINSIC TOBOWEL WALL 2. Mastocytosis
(a) benignneoplasm:leiomyoma(97"/"), 3, Histoplasmosis
adenoma,lipoma,hemangioma, neurofibroma 4. NodularlymphoidhYPerPlasia
(b) malignantprimary:adenocarcinoma, 5. lntestinallymPhangiectasia
6. Eosinophilic gastroenteritis
lymphoma(desmoplasticresponse),sarcoma,
carcinoid 7. Lymphoma
(c) metastases:from melanoma,lung,kidney, 8. Crohndisease
breast 9. Whippledisease
(d) inflammation:inflammatorypseudotumor 10. Yersiniaenterocolitis
(e) infection:parasites 11. Cronkhite-Canada sYndrome
B. EXTRINSICTO BOWELWALL 12. Cysticfibrosis
1. Duplication cyst 13. Foodparticles/ gas bubbles
14. Strongyloides stercoralis
2. Endometrioma
C. I NT RA L U M IN A L
1. Gallstoneileus
2. Parasites(ascariasis,strongyloidiasis) SmallBowelTumors
3. lnvertedMeckeldiverticulum Incidence: 1:100,000;1.5-6/" of all Gl neoplasms
4. Bloodclot Mal i gnant:beni =
gn1:1
Symptomaticmalignant:symptomatic benign= 3:1
5. Foreignbody,bezoar,Pills,seeds
Locationof smallbowelPrimaries:
ileum(41"/"),jejunum (36%),duodenum(18%)
Multiple Filling Defects of Small Bowel
A. POLYPOSISSYNDROMES R OE N TGE N OG R A P HIC A P P E A R A N C E :
1. Peutz-Jeghers syndrome (1) pedunculated intraluminal tumor,usually
2. Gardnersyndrome originating from mucosa
3. Disseminated polyposis
gastrointestinal ! smoothI irregularsurfacewithoutvisible
4. Generalized juvenilepolyposis
gastrointestinal mucosalPattern
5. Cronkhite-Canada syndrome ./ moveswithinintestinallumentwicethe lengthof
B . B E NI G NT U M O R S the stalk
1. Multiplesimpleadenomatous polyps (2) sessileintraluminal tumorwithoutstalk,usually
2. Hemangioma, blue rubberbleb nevus from tissuesoutsidemucosa
syndrome r/ smooth/ irregularsurfacewithoutvisibte
3. Leiomyoma,neurofibroma, liPoma mucosalPattern
4. Nodularlymphoidhyperplasia (3) intra-/ extramuraltumor
= horffi?lterminalileumin children r/ base of tumor greaterthan any part projecting
+ adolescents;may be associatedwith i ntothe l umen
dysgammaglobulinemia ./ mucosalpatternvisible,may be stretched
r/ symmetricfairlysharplydemarcatedfilling (4) serosaltumor
defects r/ displacementof adjacentloops
5. Varices(= multiplephlebectasia in jeiunum, r/ small bowelobstruction(rare)
oral mucosa,tongue,scrotum) { coil-springpatternof intussusceptum
C. MALIGNANTTUMORS C T: smal lbow el w al > l 1.5cm thi ck
1. Carcinoidtumor Cx: small-bowelobstruction(in up to 10%)
2. Lymphoma
(a) primarylymphoma(rarelymultiple) Benign Small Bowel Tumors
(b) secondarylymphoma: gastrointestinal . asymptomatic(80%)
involvementin 63% of disseminated . melena,intermittent
abdominalpain,weakness
disease;19% in smallintestine . palpableabdominalmass (20%)
 DifferentialDiagnosisof Gastrointestinat
Disorders 771

Types: 6. Catharticabuse
1. Leiomyoma (36-49%) D. PROLAPSE
Location: anysegment (a) antegrade: indistinguishable
from lipomatosis
/
2. Adenoma(15-20%) prolapsingmucosa/ neoplasm
3. Lipoma(1+16%) (b) retrograde
Location: duodenum(92/"),jejunum
(17"/"), E. INTUSSUSCEPTION
t ") F. LY MP H OIDH Y P E R P LA S IA
r/rat-dens,,,i'5lt{u
4. Hemangioma(19-160/") ConedCecum
5. Lymphangioma (5%) A. INFLAMMATION
Location: duodenum> jejunum> ileum 1. C rohndi sease
6. Neurogenictumor(1%) r/ involvement of ascendingcolon+ terminal
i l eum
Malignant Small Bowel Tumors 2. Ulcerativecolitis
At risk: Crohndisease,celiacdisease,polyposis d backwashileitisfin 10/")
syndromes,historyof small-bowel diverting { gapingileocecalvalve
surgery 3. Appendicitis
. asymptomatic(10-30%) 4. Typhlitis
. pain due to intermittentobstruction(gO%) 5. Perforatedcecaldiverticulum
. weight loss (66%) B . IN FE C TION
. gastrointestinalblood loss (50%) 1. Tuberculosis
. palpableabdominalmass (50%) r/ colonicinvolvement moreprominentthan that
of termi nali l eum
PRlmanvMaucnnrurSulll Bowel Turuon 2. Amebiasis
1. Carcinoid(25-41%) r/ involvementof cecum in 90% of amebiasis
0 Mostcommonprimarysmallboweltumorl r/ thickenedileocecalvalvefixed in open position
Location: predominantlydistal ileum r/ refluxinto normalterminalileum
r/ calcifiedmesentericmass on CT { skip lesionsin colon
2. Adenocarcinoma (25-26%) 3. Actinomycosis
Location: duodenum(48/"),jejunum(44/"), . palpableabdominalmass
ileum (8%) . indolentsinustractsin abdominalwall
3. Lymphoma(16-17%) 4. Blastomycosis
r/ aneurysmaldilatation 5. Anisakiasis
4. Gastrointestinal stromaltumor (GIST) 6. Typhoid,Yersinia
= leiomyosarcoma(g-10%) C . TU MOR
Location: ileum(50%) 1. Carcinomaof the cecum
5 Vascularmalignancy(1%) 2. Metastasisto cecum
6. Fibrosarcoma (0.3%)
CecalFillingDefect
SecoruonnvMlucrunrur Smau Bowel Tuuon A . A B N OR MA LITIEOFS TH E A P P E N D IX
0 Mostcommonneoplasmof smallintestines! 1. Acuteappendicitis / appendiceal abscess
2. Crohndisease
CECUM 3. Invertedappendiceal stump/ appendiceal
lleocecalValveAbnormalities intussusception
A. -Lipomatosis: >40 years of age, female 4. Mucocele
r/ stellate/ rosettepattern 5. Myxoglobulosis
B. NEOPLASM 6. Appendicealneoplasm:carcinoidtumor(gO%),
1. Lipoma,adenomatous polyp,villousadenoma leiomyoma,neuroma,lipoma,adenocarcinoma,
2. Carcinoidtumor metastasis
3. Adenocarcinoma: 2/" of all coloniccancers B . C OLON ICLE S ION
4. Lymphoma:ofteninvolvingterminalileum 1. A meboma
C. INFLAMMATION 2. Primarycecal neoplasm
1. Crohndisease 3. lleocolicintussusception
2. Ulcerativecolitis 4. Lipomatosisof ileocecalvalve
{ patulousvalve,fixed in open position C. UNUSUALABNORMALITIES
3. Tuberculosis 1. lleocecaldiverticulitis
(in 50% < age 30 years)
4. Amebiasis 2. Solitarybenignulcerof the cecum
r/ terminalileum not involved(in USA) 3. Adherentfecolith(eg, in cysticfibrosis)
5. Typhoidfever,anisakiasis, schistosomiasis, 4. Endometriosis
actinomycosis 5. Burkittlymphoma
772 Radiology Review Manual

mnemonic; "CECUMTIPSALE" 3. IN FE C TION

Carcinoma acuteamebiasis,schistosomiasis, strongyloidiasis,
Enteritis cytomegalovirus (in renal transplant recipients),
Carcinoid pseudomembranous colitis
Ulcerativecolitis 4. MA LIGN A N TLE S ION S
Mucoceleof appendix localizedprimarylymphoma,hematogenous
Tuberculosis metastases
IntussuscePtion 5. MIS C E LLA N E OU S
Periappendiceal abscess endometriosis, amyloidosis, pneumatosis intestinalis,
Stumpof the aPPendix diverticulitis,
diverticulosis, hereditaryangioneurotic
Ameboma edema
"PSALMll"
Lymphoma mnemonic;
Endometriosis Pseudomembranous colitis
Schistosomiasis
Appendiceal I ntussusceqtion Amebiccolitis
1. M uc oc e l e Lymphoma
2. Endometrioma Metastases(to colon)
3. Fecolith lschemiccolitis
4. Foreignbody Inflammatoryboweldisease
5. Polyp(juvenile,inflammatorY)
6. Papilloma GolonicUrticariaPattern
7. Adenoma/ adenocarcinoma A. OBSTRUCTION
8. Carcinoidtumor 1. Obstructing carcinoma
9. Postappendectomy stumP 2. Cecalvolvulus
3. C ol oni ci l eus
on CT
PericecalFat-Stranding B . IS C H E MIA
1. A ppendici ti s C. INFECTION/ INFLAMMATION
2. Crohndisease 1. Yersiniaenterocolitis
3. Tuboovarianabscess 2. Herpes
4. Cecaldiverticulitis 3. Crohndisease
5. Perforatedcecal carcinoma D . U R TIC A R IA

COLON ColonicUlcers
GolonCutoffSign A . ID IOP A TH IC
= abrupttermination of colonicgas columnat splenic 1. Ulcerativecolitis
flexurewith decompressionof the distalcolondue to 2. Crohncolitis
spasm+ obstruction at the splenicflexureimpinging B. ISCHEMIC
on a paralytictransversecolon 1. l schemi ccol i ti s
A. IMPINGEMENT VIA PHRENICOCOLIC LIGAMENT C . TR A U MA TIC
1. Acute pancreatitis/ postpancreatitic
stricture 1. R adi ati oni nj urY
2. Pancreatici gastriccarcinoma 2. Causticcolitis
3. Hemorrhagefrom ruptureof splenicartery/ D. NEOPLASTIC
abdominalaorticaneurysm 1. Primarycoloniccarcinoma
B . CO LO NI CD ISE AS E 2. Metastases(prostate,stomach,lymphoma,
1. Coloncancer leukemia)
2. Mesentericthrombosis E. INFLAMMATORY
3. lschemiccolitis 1. Pseudomembranous colitis
4. Perforatedappendicitis(in 20%) 2. Pancreatitis
N.B.: amputationof gas at the hepaticflexure 3. Diverticulitis
due to spasticascendingcolon 4. Behgetsyndrome
5. SolitaryrectalulcersYndrome
ColonicThumbprinting 6. Nonspecific benignulceration
= sharplydefinedfingerlikemarginalindentations at F. IN FE C TION
contoursof wall (a) protozoan
1. I S CHE M IA= l s c h e mi c o l i ti s 1. A mebi asi s
occlusivevasculardisease,hypercoagulability state, 2. Schistosomiasis
hemorrhageinto bowelwall (bleedingdiathesis, 3. StrongYloidiasis
anticoagulants), traumaticintramuralhematoma (b) bacterial
2. I NF LA MM AT ION sal monel l osi s
1. S hi gel l osi s,
ulcerativecolitis,Crohncolitis 2. Staphylococcal colitis
 DifferentialDiagnosisof Gastrointestinat
Disorders 773

3. Tuberculosis (c) ischemic

4. Gonorrhealproctitis 1. l schemi ccol i ti s
5. Yersiniacolitis (d) traumatic
6. Campylobacterfetus colitis 1. Radiationinjury
(c) fungal 2. Catharticcolon
histoplasmosis,mucormycosis, actinomycosis, 3. Causticcolitis
candidiasis B . MA LIGN A N T LE S ION
(d) viral (a) primary
1. Lymphogranuloma venereum . 1. Coloniccarcinoma(annulari scirrhous)
2. Herpesproctocolitis 2. Complication of ulcerativecolitis+ Crohn
3. Cytomegalovirus (transpfants) colitis
(b) metastatic:
Aphthous Ulcersof Colon from prostate,cervix,uterus,kidney,stomach,
1. Crohndisease pancreas,primaryintraperitoneal sarcoma
2. Amebiccolitis - hematogenous(eg, breast)
3. Yersinia enterocolitis - lymphangitic spread
Organism; Gram-negative - peritonealseeding
. fever,diarrhea,RLe pain
C . E X TR IN S ICP R OC E S S
Location: terminalileum (a) inflammation
^/ thickenedfolds + ulceration 1. Retractilemesenteritis
d lymphoidnodularhyperplasia 2. Diverticulitis
4. Salmonella, shigellainfection 3. Pancreatitis
5. Herpesvirusinfection (b) deposits
6. Behgetsyndrome 1. Amyloidosis
7. Lymphoma 2. Endometriosis
8. ls c hem ia 3. Pelviclipomatosis
D . P OS TS U R GIC A L
MultipleBull's-eyeLesionsof ColonicWall 1. Adhesivebands
mnemonic; "MaCKCLaN" 2. Surgicalanastomosis
Melanomaand E . N OR MA L
Carcinoma 1. C annonpoi nt
Kaposisarcoma
Carcinoid
Lymphomaand
Neurofibromatosis Localized Colonic Narrowing
mnemonic; "SCAREDCELL-MATE"
Double-tracking
of Colon Schistosomiasis
= longitudinal
extraluminal
tracksparalleling
the colon Carcinoid
1. Diverticulitis:
generally8-6 cm in length Actinomycosis
2. Crohndisease:generally>10 cm Radiation
3. Ulcerativecolitis Endometriosis
4. Primarycarcinoma:wider+ more irregular Diverticulitis
Colitis
ColonicNarrowing Extrinsiclesion
A . CHRO NI CS T AGEOF A N Y U L C ER AT IN G
COLITIS Lymphoma
(a) inflammatory: Lymphogranulomavenereum
1. Ulcerativecolitis Metastasis
2. Crohncolitis Adenocarcinoma
3. Solitaryrectalulcersyndrome Tuberculosis
4. Nonspecificbenignulcer Entamoebahistolytica
(b) infectious:
1. A m eb i a s i s
2. Schistosomiasis Microcolon
3. Bacillarydysentery mnemonic.' "Ml MCA"
4. Tuberculosis Meconiumileus,Meconiumperitonitis(cystic
5. Fungaldisease fibrosis)
6. Lymphogranulomavenereum lleal ljejunal atresia
7. Herpeszoster Megacystis-microcolon-hypoperistalsis
syndrome
8. Cytomegalovirus Colonicatresia(distalto atreticsegment)
9. Strongyloides Aganglionosis(Hirschsprungdisease)
774 Hadiology Review Manual

ColonicFillingDefects 3. Diverticulitis
4. Foreign-bodyPerforation
Submucosal Tumor of Colon BODY
1. Lipoma E. NONSESSILEINTRALUMINAL
1. Fecalimpaction
2. Carcinoid
Leiomyoma 2. Foreignbody
3.
hemangioma
Lymphangioma, 3. Gallstone
4.
4. Bolusof Ascarisworms
F. MISCELLANEOUS
Single Colonic Filling Defect
1, E ndometri osi s
A . B E NI G NT U MOR
3rd mostcommonbenigntumor
1. P oly p
Location: sigmoidcolon,rectosigmoid
(hyperplastic,adenomatous,villousadenoma,
junction(at levelof cul-de-sac)
villoglandulafl; mostcommonbenigntumor . may cause bleeding(afterinvasionof
2. Lipoma
mucosa)
Mostcommonintramuraltumor;2nd most
2 . LocalizedamytoiddePosition
commonbenigntumor;M < F
3 . Suturegranuloma
Location: ascendingcolon + cecum > left side
4. Intussusception
of colon
5 . Pseudotumor(adhesions,fibrousbands)
3. Carcinoid:10% metastasize
6. Colitiscysticaprofunda
4. Spindlecelltumor
(leiomyoma, 4th most
fibroma,neurofibroma);
commonbenigntumor;rectum> cecum
Multiple Colonic Filling Defects
5. Lymphangioma, hemangioma A. NEOPLASMS
(a) polyposissyndrome:
B . M A LI G N AN T U M O R
(a) primarytumor: familialpolyposis,Gardnersyndrome,Peutz-
Jegherssyndrome,Turcotsyndrome,juvenile
carcinoma,sarcoma
polyposissyndrome,disseminated
(b) secondarY tumor:
polyps,multipleadenomatous
gastrointestinal
metastases(breast,stomach,lung,pancreas,
polyps
kidney,femalegenitaltract),lymphoma,
(b) hematogenousmetastases:
invasionby adjacenttumors
from breast,lung,stomach,ovary,pancreas,
C. I NF E CT IO N
1. A m e b o ma uterus
TB (c) multipletumors
2. Polypoidgranuloma:schistosomiasis,
beni gn:
D. INFLAMMATION
pseudopolyp:ulcerativecolitis, neurofibromatosis,coloniclipomatosis,
1. lnflammatory
multiplehamartomasyndrome(Cowden
Crohndisease
abscess disease)
2. Periappendiceal

Differential Diagnosis of Colonic Polyps

Single Polyp MultiPle PolYPs

Neoplastic(10 %)
- epithelial 1. Tubular adenoma 1. Familialmultiplepolyposis
(adenomatous) adenoma
2. Tubulovillous 2. Adenomatosis of Gl tract
3. Villousadenoma 3. Gardnersyndrome
4. TurcotsYndrome
- nonepithelial 1. Carcinoid
2. Leiomyoma
3. Liooma
4. Hemangioma, lYmPhangioma
5. Fibroma.neurofibroma
Nonneoplastic (90%)
- unclassified 1. Hyperplasticpolyp polyposis
1. Hyperplastic
: hamartomatous 1. Juvenilepolyps 1. Juvenilepotyposis
2. Peutz-JegherssYndrome
3. Cronkhite-Canada sYndrome
- inflammatory 1. Benignlymphoidpolyp 1. Ulcerativecolitis
2. FibroidgranulationpolyP
 DifferentialDiagnosisof Gastrointestinat
Disorders 775

malignant: 3. Postinflammatory (fitiform) polyp

lymphoma,leukemia,adenocarcinoma = fingerlikeprojectionof submucosacoveredby
B. INFLAMMATORY PSEUDOPOLYPS mucosaon all sidesfollowinghealing
ulcerativecolitis,Crohncolitis,ischemiccolitis, + regeneration of inflammatory (mostcommon
amebiasis,schistosomiasis, strongyloidiasis, in ulcerativecolitis)/ ischemic/ infectiousbowel
trichuriasis
disease
C. ARTIFACTS
Hi stologic classification :
feces,air bubbles,oil bubbles,mucousstrands,
A. ADENOMATOUSPOLYPS
ingestedforeignbody (eg,corn kernels) = Familial adenomatous polyposis syndrome
D. M I S CE L L A N E O U S
Cause: abnormalityon chromosome5
nodularlymphoidhyperplasia, lymphoidfollicular Cx: adenomatouspolypsare premalignant
pattern,hemorrhoids, pneumatosis
diverticula,
eventuallyfeadingto colorectalcarcinoma
intestinalis,colitiscysticaprofunda,colonic
1. Fami l i al(mutti pte)
potyposi s
urticaria,submucosalcolonicedemasecondaryto
2. Gardnersyndrome
obstruction, cysticfibrosis,amyloidosis,ulcerative 3. Turcotsyndrome
pseudopolyps,proximafto obstruction
B. HAMARTOMATOUSPOLYPS
mnemonic.' "MILL P3" = Hamartomatous polyposis syndromes
Metastases(to colon)
1. Peutz-Jeghers syndrome(mostin smallbowel).
lschemia(thumbprinting)
2. Cowdendisease*
Lymphoma
3. Juvenilepolyposis.
Lymphoidhyperplasia
4. Cronkhite-Canada syndrome
Polyposis
5. Bannayan-Riley-Ruvalcaba syndrome
Pseudopolyposis (withinflammatorybowel * = increased prevalence of coexisting
adenomas and
disease); Pneumatosiscystoides adenoma-carcinoma sequence
C. POLYPOSISLOOK-ALIKES
1. Inflammatory polyposis
Carpet Lesionsof Colon 2. Lymphoidhyperplasia
= flat lobulatedlesionswith alterationof surface 3. Lymphoma
texture+ little/ no protrusionintolumen 4. Metastases
Location: rectum> cecum> ascendingcolon 5. Pneumatosis coli
Cause:
A. NEOPLASMS PolyposisSyndromes
1. T ubul a /r tu b u l o v i l l o u/ sv i l l o u sa d e n o ma = rTlofethan 100 polypsin number
2. Familialpolyposis Mode of transmission:
3. Adenocarcinoma A . H E R E D ITA R Y
4. Submucosaltumor spread(fromadjacent (a) autosomaldominant
carcinoma) 1. Fami l i al(mutti pte)
potyposi s
B . M I S CE L L AN EOU S
2. Gardnersyndrome
1. Nonspecific follicularproctitis 3. Peutz-Jeghers syndrome
2. Biopsysite 4. Juvenilepolyposiscoli
3. Endometriosis (b) autosomalrecessive
4. Rectalvarices 1. Turcotsyndrome
5. Colonicurticaria B . N ON H E R E D ITA R Y
1. Cronkhite-Canada syndrome
2. Juvenilepolyposis
ColonicPolyp
Terminology:
1. P oly p MURAL STRATIFICATION OF INTESTINAL TRACT
= rTr€lss
projectinginto the lumenof a hollow = abnormalseparationof bowel layerson cross-sectional
viscusabovethe levelof the mucosa;usually imaging
arisesfrom mucosa,may derivefrom C E C T:
submucosa/ muscularispropria r/ "doubelhalo/ target"sign duringarterialphase:
(a) neoplastic:adenomaI carcinoma
r/ contrastenhancemenlof innerlayer:
(b) nonneoplastic:hamartoma/ inflammatory (1) mucosa+ (2) muscularispropria
polyp ^/ interposededema (water)/ hemorrhage(blood)i
2. Pseudopolyp inflammatorycell infiltrate(pus,cells)/ fatty
= scatteredislandof inflamededematousmucosa proliferation:
on a backgroundof denudedmucosa (3) submucosa
(a) pseudopolyposis of ulcerativecolitis r/ contrastenhancementof outer layer:
(b) "cobblestoning"of Crohndisease (4) muscularispropria+ (5) serosa
776 RadiologyReviewManual

Cause: 7. lntramuralhemorrhage
0 Tumor has not been reportedto cause stratificationl
A . E DE M A
^/ low-density/ water-densityseparation RECTUM & ANUS
1. Ulcerativecolitis(50%): rectum RectalNarrowing
2. Proximalto obstructingtumor / intussusception 1. Pelviclipomatosis + fibrolipomatosis
CELL INFILTRATE 2. Lymphogranuloma venereum
B. INFLAMMATORY
1. Crohndisease(in uP to 50%) 3. RadiationinjurYof rectum
2. Mycobacteriumtuberculosis 4. Chroniculcerativecolitis
3. Eosinophilicenteritis
4. Cytomegalovirus EnlargedPresacralSPace
5. Clos t ri d i u m d i ffi c i l e Normalwidth<5 mm in 95%; abnormalwidth>10 mm
6. EntamoebahistolYtica A. RECTALINFLAMMATION / INFECTION
7. Vibriocholera ulcerativecolitis,Crohncolitis,idiopathic
8. S higel l a proctosigmoiditis,radiationtherapy
9. Escherichia coli B . R E C TA LIN FE C TION
C. ISCHEMIA/ INFARCTION 1. Proctitis(TB,amebiasis,lymphogranuloma
1. Arterialobstruction: plaque
thromboembolism, venereum,radiation,ischemia)
thrombus 2. Diverticulitis
2. PeripheralvasculoPathY C . B E N IGN R E C TA LTU MOR
3. Venousobstruction:thrombosis,boweltorsion, 1. Developmental cyst (dermoid,entericcyst,tailgut
closed-looPobstruction cYst)
4. Hypoperfusion:proximalarterialstenosis 2. Lipoma,neurofibroma, hemangioendothelioma
potentiatedby myocardialinfarction,bradycardia, 3. EPidermalcYst
dehydration 4. RectalduPlication
N.B.: closed-loopobstructionwith signsof bowel D. MALIGNANTRECTALTUMOR
infarctionis a surgicalcondition! 1. Adenocarcinoma, cloacogenic carcinoma
r/ signsof bowel infarction: 2. Lymphoma,sarcoma,lymph node metastases
r/ free peritonealfluid 3. Prostaticcarcinoma,bladdertumors,cervical
{ asymmetricbowelwall enhancement cancer,ovariancancer
rl persistentenhancementof bowelwall / E. BODY FLUIDS/ DEPOSITS
segmentalarteries 1. Hematoma:surgery,sacralfracture
r/ arterial/ venousfillingdefects 2. Pus: perforatedappendix,presacralabscess
^/ increaseddensityof mesentery 3. Serum: edema,venousthrombosis
r/ bowelobstruction 4. Depositof fat: pelviclipomatosis, Cushing
D. INTESTINALWALL HEMORRHAGE disease
1. Anticoagulation 5. Depositof amYloid:amYloidosis
2. Blooddyscrasia:thrombocytopenic purpura F. SACRALTUMOR
3. Blunttrauma 1. Sacrococcygeal teratoma,anteriorsacral
{ "snow-cone"appearanceof duodenum meningocele
2. Chordoma,metastasisto sacrum
Golitis
Pseudomembranous G. MIS C E LLA N E OU S
1. Clostridiumdifficile 1. Inguinalherniacontainingsegmentof colon
2. lschemiccolitis:acute/ subacute 2. ColitiscYsticaProfunda
3. Staphylococcus 3. PelvicliPomatosis
4. S higella
5. Pseudomonas aeruginosa Lesionsof lschiorectalFossa
6. Drugs: chlorpropamide, mercuriccompounds,gold, A. Congenitaland developmental anomalies
N SA ID S 1. Gartnerduct cYst
2. Klippel-Trenaunay sYndrome
AccordionSign 3. Tailgutcyst
= gross irregularpolypoidthickeningof colonicwallwith B. lnflammatoryand hemorrhagiclesions
wide separationof inner+ outerwalls 1. Fi stul ai n ano
1. Radiation-induced colitis 2. lschiorectal/ perirectalabscess
3. Extraperitoneal pelvichematoma
2. lschemiccolitis
3. Infectiouscolitis:Clostridiumdifficile,tuberculosis 4. RectalPerforation
4. Typhlitis,neutropeniccolitis C. SecondaryneoPlasm
5. lnflammation:Crohndisease,ulcerativecolitis per directextension/ hematogenousspread:
0 The only 2 conditionswith wall thickening>10 mm anorectallprostatic/ pelvic/ sacraltumor;lung
6. Lymphangiectasia cancer;melanoma;lYmPhoma
 DifferentialDiagnosisof GastrointestinalDisorders 777

D. Primaryneopfasm MesentericMass
1. Aggressiveangiomyxoma A . R OU N DS OLIDMA S S E S
2. Lipoma 0 Benignprimarytumorsare morecommonthan
3. Plexiformneurofibroma malignantprimarytumorsl
4. Anal adenocarcinoma 0 Secondaryneoplasmsmore frequentthan primary
5. Squamouscellcarcinoma 0 Cystictumorsmorecommonthan solidtumors!
0 Malignantsolidtumors have a tendencyto be
PERITONEUM locatednear root of mesentery,benignsolid
PeritonealMass tumorsin peripherynear bowel!
A. SOLIDMASS 1. Metastasesespeciallyfrom colon,ovary (most
1. Peritonealmesothelioma frequentneoplasmof mesentery)
2. Peritonbalcarcinomatosis 2. Lymphoma
B. INFILTRATIVE PATTERN 3. Leiomyosarcoma(morefrequentthan
1. Peritonealmesothelioma leiomyoma)
C. CYSTICMASS 4. Neuraltumor(neurofibroma, ganglioneuroma)
1. Cysticmesothelioma 5. Lipoma(uncommon), lipomatosis,
liposarcoma
2. Pseudomyxomaperitonei 6. Fibroushistiocytoma
3. Bacterial/ mycobacterialinfection 7. H emangi oma
8. Desmoidtumor(mostcommonprimary)
MESENTERY & OMENTUM 9. Desmoplastic smallroundcelltumorof peritoneum
Short mesentery B . ILL.D E FIN E DMA S S E S
= shortenedline of fixation 1. Metastases(ovary)
1. Malrotation+ midgutvolvulus 2. Lymphoma
2. Omphalocele 3. Fibromatosis,fibrosingmesenteritis(associated
3. Gastroschisis with Gardnersyndrome)
4. Congenitaldiaphragmatic hernia 4. Lipodystrophy
5. Asplenia+ polysplenia 5. Mesentericpanniculitis
C. STELLATEMASSES
"Applepeel" 1. Peritonealmesothelioma
SmallBowel
= distalsmall intestinesspirafsaroundits vascular 2. Retractilemesenteritis
supplyresemblingan applepeel resultingin a very 3. Fibroticreactionof carcinoid
shortintestine 4. Radiationtherapy
1. Proximaljejunalatresia 5. Desmoidtumor
2. Absenceof distalsuperiormesentericartery 6. Hodgkindisease
3. Shorteningof small boweldistalto atresia 7. Tuberculousperitonitis
4. Absenceof dorsalmesentery 8. Ovarianmetastases
Cx: propensitytowardnecrotizingenterocolitis 9. Diverticulitis
Prognosis; high mortality 10. Pancreatitis
0 A calcifiedmesentericmass suggestscarcinoid
tumor!
OmentalMass D. LOCULATEDCYSTTCMASSES(2/3)
0 33%of primary
omental
tumors
aremalignant! 1. Cysticlymphangioma (mostcommon)
0 Secondaryneoplasmsare more frequentthan 2. Pseudomyxoma peritonei
primaryl 3. Cysticmesothelioma
A. SOLIDMASS 4. Mesentericcyst
(a) benign 5. Mesenterichematoma
1, Leiomyoma 6. Benigncysticteratoma
2. Lipoma 7. Cysticspindlecelltumor(= centrallynecrotic
3. Neurofibroma leiomyoma/ leiomyosarcoma)
(b) malignant
1. Leiomyosarcoma Mesenteric/ OmentalCysts
2. Liposarcoma = ,'BUBBLES OFTHEBELLY',
3. Fibrosarcoma 0 The firststep is to determinethe organof originl
4. Lymphoma 1. Lymphangi oma
5. Peritonealmesothelioma 2. Nonpancreaticpseudocyst
6. Hemangiopericytoma = s€Quelaeof mesenteric/ omentalhematoma/
7. Metastases abscess
(c) Infection:tuberculosis Path: thick-walled,usuallyseptatedcystic mass
B. CYSTICMASS with hemorrhagic / purulentcontents
1. Hem at o ma 3. Duplicationcyst
778 Radiology Review Manual

4. Mesothelialcyst @ Gastrohepatic ligamentnodes

5. Entericcyst = suP€riorportionof lesseromentumsuspending
stomachfrom liver
Mesenteric Edema / Congestion Abnormalsize: >8 mm
r/ increaseof mesentericfat attenuationto -40 to Common cause: carcinomaof lessercurvatureof
_60 HU stomach,distalesoPhagus,
r/ loss of sharp interfacesbetweenmesentericvessels lymphoma,pancreaticcancer,
+ fat melanoma,colon+ breastcancer
A . S Y S T E M I CF L U IDO V ER L O A D DDx: coronaryvarices
1. Hy poal b u mi n e m i a @ Portahepatisnodes
2. Livercirrhosis = in pofia hepatisextendingdown hepatoduodenal
3. Nephrosis ligament,anterior+ posteriorto portalvein
4. Heartfailure Abnormalsize: >6 mm
B . LO CA LV E SS ELD ISE AS E Commoncause: carcinomaof gallbladder+ biliary
1. Portalveinthrombosis tree,liver,stomach,Pancreas,
2. Mesentericvein I arterythrombosis colon,lung,breast
3. Vasculitis Cx: high extrahepaticbiliaryobstruction
4. SMA dissection @ Pancreaticoduodenal nodes
= betweenduodenalsweep + pancreatichead
C. CE LL I NF]L T R A T E
1. Malignantneoplasm anteriorto IVC
2. lnflammation Abnormalsize: >10 mm
3. Trauma(smallhemorrhage) Commoncause: lymphoma,pancreatichead,
colon,stomach,lung,breast
cancer
UmbilicalTumor
@ Perisplenic nodes
A. PRTMARY (38%)
= i n spl eni chi l um
benign/ malignantneoplasm,skintumor
A bnormalsi ze: > 10 mm
B. METASTASES(30%)
= "SisterJosephnodule" Commoncause: NHL, leukemia,smallbowel
. firm painfulnodule neoplasm,ovariancancer,
. * ulcerationwith serosanguinous / purulent carcinomaof right/ transverse
colon
discharge
@ Retroperitoneal nodes
Cause: gastrointestinal cancer(50%),
(25%),ovariancancer, = periaortic,pericaval,interaortocaval
undetermined
A bnormalsi ze: > 10 mm
pancreaticcancer,smallcell carcinomaof
lung (very rare) Commoncause: lymphoma,renalcell,testicular,
cervical,prostaticcarcinomas
Spread:
(a) directextensionfrom anteriorperitoneal
surface @ Celiacand superiormesentericarterynodes
= preaorticnodes
(b) extensionalongembryonicremnants:
Abnormalsize: >10 mm
falciform,medianumbilical,
omphalomesenteric ligaments Commoncause: any intraabdominalneoplasm
(c) hematogenous
(d) retrogradelymphaticflow from inguinal, @ Pelvicnodes
= alongcommon,external+ internaliliacvessels
axillary,paraaorticnodes
A bnormalsi ze: > 15 mm
(e) iatrogenic:laparoscopictract,tract of
percutaneousneedlebiopsy Commoncause: carcinomaof bladder,prostate,
cervix,uterus,rectum
C. NO NNE O P L AS T IC
1. Endometriosis (32%)
2. G r anu l o m a
3. lncarcerated hernia Enlarged Lymph Node with Low'density Center
Mycobacterium
1. Tuberculosis, avium-intracellulare
ABDOMINAL LYMPHADENOPATHY 2. Pyogenicinfection
RegionalPatternsof Lymphadenopathy 3. Whippledisease
@ Retrocruralnodes 4. Lymphoma
Abnormalsize: >6 mm 5. Metastaticdiseaseafter radiation+ chemotherapy
Commoncause: lung carcinoma,mesothelioma, 6. Lymphangioleiomyomatosis
lymphoma tYPeI
7. Neurofibromatosis
 Anatomyand Functionof Gastrointestinal
Tract 779

ANATOMYAND FUNCTION
OF GASTROINTESTINAL
TRACT

GASTROINTESTINAL HORMON
ES Producedin: antral cells + G cells of pancreas
Cholecystokinin Released by:
= CCK = 33 amino acid residues(formername: (a) vagal stimulation,gastricdistension
Pancreozymin);the 5 C-terminalaminoacidsare (b) short-chainalcohol(ethanol,propanol)
identicalto those of gastrin,causingsimilareffectsas (c) aminoacids(glycine,B-alanine)
gastrin (d) caffeine
Producedin: duodenal+ upper intestinafmucosa (e) hypercalcemia
Releasedby: fatty acids, some amino acids mediatedby neuroendocrine cholinergicreflexes
(phenylalanine, methionine),hydrogen lnhibitedby: drop in pH of antral mucosa to <3.5
ions Effects:
Effects: @ Stomach:
@ Stomach (1) stimulationof gastricHCI secretionfrom parietal
(1) weaklystimulatesHCIsecretion cells,whichin turn:
(2) givenalone: inhibitsgastrin,whichleadsto (2) increasespepsinogenproductionby chiefcells
decreasein HCI production throughlocal reflex
(3) stimulatespepsinsecretion (3) increasein antralmotility
(4) stimulatesgastricmotility (4) trophiceffecton gastricmucosa(parietalcell
@ Pancreas hyperplasia)
(1) stimulatessecretionof pancreaticenzymes @ Pancreas
(= Pancreozymin) (1) strongincreasein enzymeoutput
(2) stimulatesbicarbonatesecretion(weaklyby (2) weaklystimulatesfluid + bicarbonateoutput
directeffect;stronglythroughpotentiating (3) stimulatesinsulinrelease
effect on secretin) @ Liver
(3) stimulatesinsulinrelease (1) water + bicarbonatesecretion
@ Liver @ Intestine
(1) stimulateswater + bicarbonatesecretion (1) stimulatessecretionof Brunnerglands
@ Intestine (2) increasesmotility
(1) stimulatessecretionof Brunnerglands @ Gallbladder
(2) increasesmotility (1) stimulatescontraction
@ Biliarytract @ Esophagus
(1) strongstimulatorof gallbladdercontraction (1) increasesrestingpressureof LES
(2) relaxationof sphincterof Oddi
Medication:
sincalide(Kinevac@) = cholecystokinin-C-terminal
octapeptide
Use: may be used to emptygallbladderabout Glucagon
30 minutes(to 4 hours)beforetracerinjection Producedin: o cells (and B cells) of pancreas
in patientson prolongedfasting(gallbladder Releasedby: low blood glucose levels
atony + retainedbile and sludgesecondaryto Effects:
absenceof endogenouslyproducedCCK) @ Intestines
Dose for radiologic imaging: (1) lowerspressureof GE sphincter
slow lV injectionof 0.02 pg/kg Kinevac@over (2) hypotoniceffecton duodenum> jejunum>
>3 minutes stomach> colon
Usefulin; (a) patientfasting>24hours/ on total @ Hormones
parenteralnutrition (1) releasescatecholamines from the adrenalgland
(b) acalculouscholecystitis that paralyzeintestinalsmoothmuscle
(c) chronicGB dysfunction (2) increasesseruminsulin+ glucoselevels
Side effect: increasein biliary-to-bowel transittime, (mobilizationof hepaticglycogen)
nausea,abdominalcramps @ Biliarytract
(1) increasesbileflow
(2) relaxesgallbladder+ sphincterof Oddi
Dose for radiologic imaging: 1 mg maximum
Gastrin 0 lV administration
= 17 aminoacid peptideamide; causesa quick response+ rapid
dissipation of action!
PENTAGASTRIN 0 lM administration prolongsonset+ increaseslength
= ocyl derivativeof the biologicactiveC-terminal
of action!
tetrapeptideamide Half-life: 3-6 minutes
780 Radiology Review Manual

Side effects: nausea+ vomiting,weakness, @ Esophagus

dizziness(delayedonsetof (1) opensLE S
1.54 hoursafterlM administration)
Contraindication: EMBRYOLOGY OF ALIMENTARY TRACT
(1) hypersensitivity/ allergyto glucagon:urticaria, Origin: as a pouchlikeextensionof the yolk sac
periorbitaledema,respiratorydistress, Developmentof Gltube:
hypotension, coronaryarteryspasm(?), continuoustubularstructureby 6 weeksGA;
circulatoryarrest dividedinto:
(2) known hypertensiveresponseto glucagon (a) foregut(suppliedby celiacartery)
(3) pheochromocytoma:glucagonstimulatesrelease (b) midgut(suppliedby superiormesentericartery)
of catecholamines (c) hindgut(suppliedby inferiormesentericartery)
(4) insulinoma:insulin-releasing effectmay resultin Rotation about omphalomesenteric vesselsby 270':
hypoglycemia <6 weeksGA
(5) glucagonoma 90ocounterclockwise rotationof duodenojejunal
(6) poorlycontrolleddiabetesmellitus segmenttowardthe rightof SMA
90' counterclockwise rotationof ileocolicsegment
Secretin towardthe left of SMA
Producedin: duodenal mucosa during6th week GA
Releasedby: hydrogenions providinga pH <4.5 additional90" counterclockwise rotationof duodenum
Effects: posteriorto SMA
@ Stomach remainderof midgutwithinumbilicalcord
(1) inhibitsgastrinactivity,whichleadsto decrease 10-12w eeksGA
in HCI secretion intestinesslidesback into peritonealcavity
(2) stimulatespepsinogensecretionby chief cells final90' counterclockwise rotationof duodenum
(potentpepsigogue) final 180"counterclockwise rotationof cecum
(3) decreasesgastricand duodenalmotility Peritonealfixationof small bowel
+ contractionof pyloricsphincter broad-basedmesenteryextendingfrom ligamentof
@ Pancreas Treitzto ileocecalvalve
(1) increasesalkalinepancreaticsecretions
(NaHCOr)
ESOPHAGUS
(2) weaklystimulatesenzymesecretion
(3) stimulatesinsulinrelease Anatomy
LowerEsoPhageal
A. EsophagealVestibule
@ Liver
(1) stimulateswater + bicarbonatesecretion(most = s?ccUl€lrterminationof loweresophaguswith
junction
upperboundaryat tubulovestibular
potentcholeretic)
+ lowerboundaryat esophagogastric junction
@ Intestine
(1) stimulatessecretionof Brunnerglands r/ collapsedduringrestingstate
{ assumesbulbousconfiguration with swallowing
(2) inhibitsmotility

Rotation of duodenum + Rotation of duodenum by Final 90' Final position of

distal large bowel by 90' an additional 90" counterclockwise normal bowel rotation
counterclockwise counterclockwise rotation of duodenum
Stagesof Intestinal Rotation
 Anatomyand Functionof Gastrointestinal
Tract 781

left sastric a.
short gastric aa.
right gastric a.
left hepatic a.

right hepatic a.

cystic a.
proper hepatic a.

gastroduodenala. great pancreatic a.

dorsal pancreatic a.
pancreatico-
duodenal arcade transversepancreatic a.
- anterior superior a.

left gastroepiploic a.
- posterior superior a.

- posterior inferior a.

right gastroepiploica.
- anterior inferior a.

i nferior pancreaticoduodenal
a.

Blood Supply of Stomach,Duodenum, and pancreas

left branch of accessory

middle colic a.\ middle colic a.

Griffrth point

arc of Riolan

ascending branch
middle colic a. of left colic a.

right colic a. marginal artery

of Drummond

left colic a.
ileocolic a.
superior
rectal a.

inferior
mesenteric a.

Blood Intestine
782 Radiology Review Manual

(a) tubulovestibularjunction= A level= junction CNS involved: cranialnervesV, Vll, lX, X, Xll;5 ceruical
betweentubularand saccularesophagus nerves;cortical+ subcorticalpathways;
(b) phrenicampulla= bell-shapedpart above midbrain;brainstem
diaphragm(termshouldbe discardedbecauseof Musclesinvolved: 32 groupsof muscles
dynamicchangesof configuration) Developmental: swallowingas earlyas 11 weeksGA;
(c) submergedsegment= infrahiatalpart of sucklingat 18-24 weeks GA; nonnutritive
esophagus suckingat27-28 weeks GA; single
{ widening/ disappearanceis indicativeof breathsuckingat 35-36 weeks GA
gastroesophageal refluxdisease(GERD) Phases:
B. GastroesophagealJunction 1. Oral preparatoryPhase
Site: at upperlevelof gastricslingfibers,straddles { tood chewed+ mixedwith saliva
cardiacincisurademarcatingthe left lateral 2. Oral phase
marginof GE junction r/ bolus propelledposteriorlyto tongue
C. Z line = B level= zigzag-shaped squamocolumnar Path:
junc t ionlin e (a) spillagefrom mouth
not acceptablecriterionfor locatingGE junction (b) smallbolusformation
Site: 1-2 cm abovegastricsling fibers (c) tonguetremor
D. Lower Esophageal SPhincter (d) incompletetongueelevation
= physiologic2-4-cm high pressurezone (e) earlyspillageintovalleculaepriorto initiationof
correspondingto esophagealvestibule swallow
i/ tigtrttyclosedduringrestingstate 3. PharyngealPhase
r/ assumesbulbousconfigurationwith swallowing { elevationof soft palate+ valleculae(to seal
nasopharynx)
ri elevationof larynx(to closevestibule)
MuscularRingsof EsoPhagus r/ relaxationof cricopharyngeal muscle
A Ring r/ contractionof lateralpharyngealwall
= contracted/ hypertrophiedmusclesin responseto Path:
incompetent GE sphincter (a) nasoPharYngeal reflux
. rarelysymptomatic/ dYsPhagia (b) laryngealpenetration1=contrastmaterial
Location: at tubulovestibular junction= superior enterslarYngeal vestibule
aspectof vestibule Cause: delaYedelevationof larYnx
r/ usually2 cm proximalto GE junctionat upperend (c) trachealaspiration(= contrastmaterialenters
of vestibule airwaybelowvocal cord level)
r/ varies in caliberduringthe same examination,may Cause: detayedelevationof larynx,delayed
disappearon maximumdistension pharyngealtransittime, decreased
^/ broad smoothnarrowingwith thick roundedmargins clearanceof boluswith residualin
{ visibleonly if tubularesophagusabove+ vestibule vallecula+ pyriformsinusspillinginto
below are distended larynx+ trachea
4. EsophagealPhase
B Ring { contractionof cricopharyngeal muscle
= slingfibersrepresentinga U-shapedthickeningof ./ bolustransferinto esophagus
inner musclelayerswith open arm of U toward Path: cricopharyngeal achalasia(with refluxof bolus
lessercurvature= inferioraspectof vestibule intooropharynx/ poolingin pyriformsinus)
Location: < 2 cm from hiatalmargins
{ only visiblewhen esophagogastric junctionis above STOMACH
hiatus GastricCells
^/ tnin ledge-likeringjust belowthe mucosaljunction 1. C hi efcel l s
(Z line) = peptic/ zymogeniccells
Location: bodY+ fundus
produce: PePsinogen
FUNCTION
SWALLOWING 2. Parietalcells
Technique; videofluoroscopy of a modifiedbarium = oxynticcells
swallowstudy to assesshandlingof bolus Location: bodY+ fundus
(withconsistencyof nectarliquid,honey produce: H*, Cl-,intrinsicfactor,prostaglandins
liquid,pureedfood, soft solidfood, hard 3. Mucousneckcells
solidfood);preferrablytogetherwith speech produce: mucoprotein,mucopolysaccharide,
pathologist sulfate
aminopolysaccharide
Dacument patientbehavior+ reaction: 4. Argentaffinecells
episodeof refusal,cough,silentaspiration, apnea, = enteroendocrine cells
bradycardia Location: bodY+ fundus
 Anatomyand Functionof Gastrointestinal
Tract 783

produce: glucagon-like substance(A-cells), DuodenalSegments

somatostatin(D-cells),vasoactive (1) duodenalbulb + shortpostbulbarsegment:
intestinalpolypeptide (D,-cells), intraperitoneal+ freely movable
5-hydroxytryptam ine (EC-cells) (2) descendingduodenum:
5. G-cells retroperitonealattachedto head of pancreas
Location: pylorus (3) horizontal= transversesegment:
produce: gastrin
retroperitonealcrossingthe spine
(4) ascendingportion
Effectof BilateralVagotomy retroperitonealascendingto levelof duodenojejunal
= cholinergic
denervation junction
(1) decreasedMOTILITyof stomach+ intestines V A R IA TION S :
(2) decreasedGASTRTC SECRETTON (1) "mobileduodenum"/ "water-trap duodenum"
(3) decreasedTONE OF GALLBLADDER+ biteducts = longpostbulbarsegmentwith undulation/
(4) increasedTONE OF SpHTNCTERS (Oddi+ lower redundancy
esophagealsphincter) (2) duodenuminversum/ duodenumreflexum
= distalduodenumascendsto the rightof spineto
Pylorus the levelof duodenalbulb+ then crossesspine
= fan-shapedspecialized circularmusclefiberswith: horizontally+ fixatedin normallocation
(a) distalsphinctericloop = rightcanalisloop
r/ correspondsto radiologicpyloricsphincter
(b) proximalsphinctericloop= leftcanalisloop SmallBowelFolds
r/ 2 cm proximalto distalsphinctericloopon 0 Circularsmallbowelfolds= foldsof Kerckring
greatercurvature(seenduringcomplete = valvulaeconniventes= two mucosallayersaround
relaxation) a core of submucosa
(c) torus A . N OR MA LFOLDTH IC K N E S S
= fibersof both sphinctersconvergeon the lesser @ jejunum 1.7-2.0mm > 2.5mm pat hologic
curvatureside to form a muscularprominence; @ ileum 1.4-1.7mm > 2.0mm pat hologic
prolapseof mucosabetweensphinctericloops B. NORMAL NUMBEROF FOLDS
, producesa nichesimulatingulcer @ jejunum f7 linch
tr pyloricchannel5-10 mm long,wall thicknessof @ ileum 2-4 linch
4-8 mm C. NORMALFOLD HEIGHT
r/ concentricindentationof the base of the duodenal @ jejunum 3.5-7.0mm
bulb @ ileum 2.0-3.5mm
D . N OR MA LLU ME ND IA ME TE R
@ upperjejunum 3.0-4.0 cm >4.5cm pathologic
SMALLBOWEL @ lowerjejunum 2.5-3.5 cm >4.0cm pathologic
0 Longesttubularorganin body measuring550-600cm @ ileum 2.0-2.8cm >3.0cm pathologic
(18-22 feet) in length
Segments: R U LEOF 3' s:
duodenumof 25-30 cm in length 0 Wallthickness<3 mm
jejunumof 10-12 feet in length(= proximal60%) 0 Valvulaeconniventes
<3 mm
ileumof 6-8 feet in length(= distal4O%) 0 Diameter<3 cm
Mesentery, risament
ofrreitz 0 Air-fluidlevels<3
jffiHeen
i1,3i'.:U?
NormalBowelCaliber
torus mnemonic: "3-6-9-12"
3 cm maximalsize of smallbowel
6 cm maximalsize of transversecolon
9 cm maximalsizeof cecum
12 cm maximalcaliberof cecum beforeit may burst

SmallBowelPeristalsis
A . ]N C R E A S E D
1. Vagalstimulation
2. Acetylcholine
right left 3. Anticholinesterase
(eg, neostigmine)
loop 4. Cholecystokinin
B. DECREASED
1. Atropine(eg, Pro-Banthine@)
Pyloric MuscularAnatomy 2. Bilateralvagotomy
784 Radiology Review Manual

INTESTINAL FUNCTION rectalprolapse = descentof entirethicknessof rectal

wall throughanalverge
IntestinalGas
rectalintussuscePtion
A . I NF LUX
--....2L = descentof the entirethicknessof the rectalwall
1. Aerophagia.............
possiblyextendinginto anal canal;starting6-11 cm
2. Liberationfrom intestinaltract
(a) neutralization of bicarbonatein above anus; accompaniedby formationof a circular
" ..8 L indentation forminga ring Pocket
s ec re ti o n(C s O,)
^/ infoldingof <3 mm in width/ > 3 mm in width/
(b) bacterialfermentation(CO2,H2,
. . . . . . . . . . 1. .5. .L intraluminal narrowing/ descentintoanalcanali
c H 4 'H 2 S )
from blood (N, O, CO. externalProlaPse
3. Diffusion )
B . E F F LUX
1. Diffusion{rom intestinesinto blood PERITONEUM
a n d e x p u l s i ofnr o ml u n g. . . . . . . . . ' . . . 5 0L PeritonealSpaces
2. E x puls i o fro
n ma n u s .' ..2 L Definitions:
Ligament = formedby two foldsof peritoneum
supportinga structurewithinthe
IntestinalFluid peritonealcavitY
A . I NF LUX
1 . O r a li n g e s t i o.n. . . . . . . . ."2-5L Omentum = sPecialized structureconnecting
..8.2 L stomachto an additionalstructure
2. lntestinal secretions
1 .5 L Mesentery= two peritonealfolds connectinga portion
s aliv a
"""" 0'5 L of bowelto the retroPeritoneum
b i l e" " " " '
gastricsecretions 2.5 L Embryology:
pancreaticsecretions '....0'7 L abovetransversemesocolon:
.......3 .0L A . R IGH TP E R ITON E AS LPACE
int es t in asle c re ti o n s
formsperihepatic space+ lessersac:
B . E F F LUX
" ' . . . 0 . 1L 1. RightsubPhrenic space:
1. Peranal - locatedbetweenright hepaticlobe
2. lntestinalresorption(primarilyin ileum
.' 10.6L + diaPhragm
+ as c e n d i n g colon) - limitedposteriorlyby rightsuperiorreflection
of coronarylig.+ righttriangularligament
Defecography/ EvacuationProctography 2. RightsubhePatic space:
evacuationtime = 15 (range5-40) seconds -
anorectalangle = "n9le formedbetweencentralaxis :'"iff$,iJ?,nn, rocated
space:
subhepatic
of anal canal+ lineParallelto just posteriorto PortahePatis,
posteriorwall of rectum communicating with lessersac via
^/90'at restand duringvoluntary epiploicforamen(= foramenof Winslow)
contraction(squeezemaneuver)
r/ more obtuseduringdefecation
straining(void)
anorectaljunction= pointof taper of distalrectal
ampullaas it mergeswiththe anal
canal; positionof anorectaljunction
referencedto Planeof ischial
tuberosities= 0-3.5 cm; elevation
duringsqueezeof 0-4.5 cm;
elevationduringvoid of -3.0-0 cm
rectovaginalspace = space betweenvaginaand symphysis
rectum pubis
penneum = oled betweenexternalgenital
organsand anal verge
rectocele= rTl€?sUfement depth of
of anteroposterior
rectocele
convexwall protrusionextendingbeyond
expectedmarginof normalrectalwall
small <2 cm;
moderate = 2-4 cmi :--l
large >4 cm plane of ischial tuberosity - -
peritoneocele = extensionof rectouterineexcavation
to belowupperthirdof vagina;
containingliquid/ bowel/ omentum
enterocele = bowel presentin peritoneocele Defecographic Measurements
 Anatomyand Functionof Gastrointestinal
Tract 7gs

. posteriorrightsubhepaticspace . perisplenic space:

= Morisonpouch = hepatorenalfossa boundedinferiorlyby phrenicocolic lig.
0 Mostdependentportionof the . subhepaticspace = gastrohepaticrecess:
abdomenin supinepatient! locatedbetweenlateralsegmentof left
3. Bare area of liver hepaticlobe + stomach
- situatedbetweenreflectionsof right+ left - separatedfrom rightsubphrenicspace by
coronaryfigaments falciformligament
- continuouswith rightanteriorpararenal
2. Lefttriangularligament:
space - forms from coalescenceof superiorand
4. Lessersac: inferiorreflectionsof left coronaryligament
. superiorrecess: - locatedalong superioraspectof left hepatic
- surroundsmedialaspectof caudatelobe
lobe
- separatedfrom splenicrecessby
C. DORSALMESENTERYgivesriseto:
gastropancreatic fold 1. Gastrophrenic ligament
. splenicrecessl - coursesthroughimmediatesubphrenic
- extendsacrossmidlineto splenichilum
space
. inferiorrecess: - suspendsstomachfrom dome of
- separatesstomachfrom pancreas
diaphragm
+ transversemesocolon 2. Gastropancreatic ligament
- anteriorlycoveredby lesseromentum - formedby proximalleft gastricartery
5. Lesseromentum= combination of - attachesposterioraspectof gastricfundus
gastrohepaticligament+ hepatoduodenal to retroperitoneum
ligament partiallyseparatessuperiorrecessof lesser
6. Righttriangularligament: sac from spfenicrecess
- forms from coalescenceof superiorand
3. Phrenicocolic ligament
inferiorreflections
of rightcoronaryligament - majorsuspensoryligamentof spleen
- dividesposterioraspectof rightperihepatic - attachesproximaldescendingcolonto left
spaceinto rightsubphrenicspace hemidiaphragm
+ posteriorrightsubhepaticspace - separatesleft subphrenicspace from left
B. LEFT PERITONEALSPACE paracolicgutter
forms left subphrenicspace 4. Gastrosplenic ligament
1. Leftsubphrenicspace: - remnantof dorsal mesentery
- artificially dividedinto - connectsgreatercurvatureof stomachwith
. immediatesubphrenicspace:
spl eni chi l um
betweendiaphragm+ gastricfundus - containsshort gastricvessels

falciform lis.

right left
subphrenic space subphrenic space

gastrohepaticlig.

gastrospleniclig.

splenorenal lig.

Ligaments and Peritoneal Spacesin Upper Abdomen

786 Radiology Review Manual
5. Splenorenalligament
- connectsposterioraspect of spleen to
anteriorpararenalsPace
- contributesto left lateral+ posteriorborder
of lessersac
- enclosestail of pancreas+ distalsplenic
artery+ proximalsPlenicvein
6. G as t ro c o l i c l i g a m e n t
- formsportionof anteriorborderof lesser
sac
- formssuperioraspectof greateromentum
- connectsgreatercurvatureof stomachwith
superioraspectof transversecolon
- containsgastroepiploic vessels
D. VENTRALMESENTERYgivesriseto:
1. F alci fo rm ligament
= sickle-shapedfold composedof two layersof
peritoneum
- attachesventralsurfaceof liverto anterior
abdominalwall
- its right layercontinuesinto the superior
layerof the coronaryligament,its left layer
continuesintothe anteriorlayerof the left
triangularligament
- containsligamentumteres (= obliterated
umbilicalvein)in its free inferoposterior
margin
- continuouswith fissurefor ligamentum
venosum
2. Gastrohepatic ligament:
- arisesin fissureof ligamentumvenosum
- connectsmedialaspectof liverto lesser
curvatureof stomachas part of lesser
o me n tu m
- containsleft gastricartery,coronaryvein,
lymphnodes
3. Hepatoduodenalligament:
- formsinferioredge of gastrohepatic
ligament
- forms anteriormarginof epiploicforamen
- extendsfrom proximalduodenumto porta
hepatis
- containscommonhepaticduct,common
bile duct, hepaticarlery,portalvein
belowtransversemesocolon:
A. VENTRALMESENTERYregresses
forms:
B . D OR S A LME S E N TE R Y
1. Transversemesocolon:
- suspendstransversecolonfrom
retroperitoneum alonganteroinferior edge
of pancreas
- formsposteroinferior borderof lessersac
- containsmiddlecolicvessels
2. SmallbowelmesenterY:
- suspendssmallbowelfrom retroperitoneum
- extendsfrom ligamentof Treitzto ileocecal
valve
- containssuperiormesentericvessels
+ lymphnodes
3. Sigmoidmesocolon:
- attachessigmoidcolonto posteriorpelvic
wall
- containssigmoid+ hemorrhoidal vessels
4. Greateromentum:
- inferiorcontinuation of gastrocolicligament
- formedby doublereflectionof dorsal
mesogastriumthus composedof 4 layersof
peritoneum
5. Superior+ inferiorileocecalrecesses:
- locatedabove + belowterminalileum
6. RetrocecalsPace:
- presentonly if peritoneumreflectsposterior
to cecum
7. Right+ leftparacolicgutters:
- locatedlateralto ascending+ descending
colon
B. lntersigmoid recess:
- locatedalong undersurfaceof sigmoid
mesocolon

GASTROINTESTINAL
DISORDERS
ACHALASIA ADENOMA OF SMALL BOWEL
= failureof organizedperistalsis+ relaxationat levelof
loweresophagealsphincter
Etiology: (a) idiopathic:abnormalityof Auerbachplexus
/ medullarydorsalnucleus;? neurotropic
virus,? gastrinhypersensitivity
(b) Chagasdisease
r/ megaesophagus = dilatationof esophagusbeginningin
, upper1/3,ultimatelyentirelength
{ absenceof primaryperistalsisbelow levelof ADENOMATOUS
, cricopharyngeus
r/ nonperistaltic contractions
r/ "bird-beak" l"rattail" deformity= y-g6apedconical
+ symmetrictaperingof stenoticsegmentwith most
markednarrowingat GE junction
r/ Hurstphenomenon= temporarytransitthroughcardia
when hydrostaticpressureof bariumcolumnis above
, tonic LES pressure
{ suddenesophagealemptyingafter ingestionof
carbonatedbeverage(eg, Coke)
r/ "vigorousachalasia-" ='nimerous tertiarycontractionsin
nondilateddistalesophagusof early achalasia
r/ promptrelaxationof LES upon amyl nitrateinhalation
(smooth-muscle relaxant)
C X R:
r/ rightconvexopacitybehindright heartborder;
occasionallyleft convexopacityif thoracicaorta
tortuous
r/ rightconvexopacitymay be tetheredby azygosarch
allowingfor greaterdilatationabove + below
r/ air-fluidlevel (stasisin thoracicesophagusfilledwith
retainedsecretions+ alimentaryresidue)
{ small/ absentgastricair bubble
{ anteriordisplaCement + bowingof trachea(LAT view)
{ patchybilateralalveolaropacitiesresemblingacute/
chronicaspirationpneumonia(M. fortuitum-chelonei
infection)
Cx: esophagealcarcinomain 2-7% (usually
midesophagus)
Rx: pneumaticdilatation/ surgicalmyotomy
DDx: (1) Neoplasm(separationof gastricfundusfrom
diaphragm;normalperistalsis;asymmetric
tapering)
(2) Pepticstrictureof esophagus
Secondary Achalasia
= carcinomaof cardia/ gastricfundusinvading
esophagus
Age: >50 years
. durationof symptomsfor <6 months
r/ irregular/ asymmetricnarrowing
{ abrupttransition
r/ associatedfundallesion
Gastrointestinal Disorders 787
Location: duodenum(21%),jejunum(36%),ileum(49%)
esp. ileocecalvalve
Histo: (1) Hamartomatouspolyp (77o/"),multiplein 47o/o,
1/3 of multipfelesionsassociatedwith peutz-
Jegherssyndrome
(2) adenomatouspolyp(19/"), may have malignant
potential
(3) polypoidgastricheterotopic tumor(10%)
COLONICPOLYP
= EPITHELIALPOLYP
Mostcommonbenigncolonictumor(68-79/")
Predisposed: previouslydetected polyp I cancer;family
historyof polypsI cancer;idiopathic
inflammatory boweldisease;Peutz-
Jegherssyndrome;Gardnersyndrome;
'Jfl
prevat
ence
: r.j""'T n'#::i 0o/"inzth
decade
:
260/"in 9th decade
Location: rectum(21-34%);sigmoid(26-gg%);
descendingcolon(6-18%);transversecolon
(12-13/"); ascendingcolon (9-12%); multiple
in 35-50% (usually<5-10 in number)
Histo:
1. Tubularadenoma(75%)
= cylindrical glandularformationlinedby stratified
columnarepithelium+ containingnestsof
epitheliumwithinlaminapropria
r/ usual l y< 10 mm i n di ameter
{ oftenpedunculated if >10 mm
malignantpotential: <10 mm in 1"/":10-20 mm in
10/"; >20 mm in 35%
2. Tubul ovi l l ousadenoma(15% )
= mixturebetweentubular+ villousadenoma
malignantpotential: <10 mm in 4y"; 10-20 mm in
7o/"i>20 mm in 46%
3. Villousadenoma(10%)
= thin frondlikesurfaceprojections(,Villousfronds,')
. potassiumdepletion
r/ often >20 mm in diameterwith papillarysurface
r/ often broad-basedsessilelesion
malignantpotential: <10 mm in 10"/";10-20mm in
10"/";>20 mm in 53%
Adenoma size & incidence of malignancy:
<5 mm in 0.5%;5-9 mm in 1"/";10-20mm in 5-10"/":
>20 mm in 10-50% mafignant
0 Invasivecarcinoma(= penetration of muscularis
mucosa):
(a) rarein a pedunculated adenomaof <15 mm
(b) in 30.h of villousadenomasof >50 mm
0 All polyps>10 mm shouldbe removed!
0 Time for adenoma-carcinoma sequenceprobably
averages10-15 years!
788 Radiology Review Manual

Probability of coexistent colonic growth: Cause: gastrojejunostomy with left-to-rightanastomosis

- synchronous adenomain 50% (= proximaljejunal loop attachedto greater
- metachronousadenomain 30-40% curvatureinsteadof lessercurvature),
- synchronous adenocarcinoma in 1'S-5% mechanicalfactors(intussusception, adhesion,
- metachronous adenocarcinoma in 5-10% kinking),inflammatory disease,neoplastic
. asymptomatic(75%) infiltrationof local mesenteryor anastomosis,
. diarrhea,abdominalPain idiopathicmotordYsfunction
. peranalhemorrhage(67%) . postprandial epigastricfullnessrelievedby bilious
Colonoscopy(incompletein 16-43%) vomiting
BE: . vitamin8.,, deficiencywith megaloblastic anemia
Sensitivity of DCBE in detecting polyps: . afferentlciSpwith abnormalbacterialflora (Gram
<10 mm 80-83%; >10 mm 96-97"/";all84-88h; negative,resemblingcolonin quality+ quantity)
rateof detectionof polyps<10 mm higherwith DCBE Abdominalplainfilm:
than SCBE r/ normalin 85% (no air in lumenof afferentloop)
./ sessileflat / roundpolyp UGI:
{ pedunculated polyp: stalk>2 cm in lengthalmost ^/ preferentialemptyingof stomachinto proximalloop
alwaysindicativeof a benignpolyp r/ proximalloop stasis
{ suggestiveof malignancy:irregularlobulatedsurface, r/ regurgitation
broad base = width of the base greaterthan height, CT:
retractionof colonicwall = dimpling/ indentation / ! roundedwater-densitymassesadjacentto head + tail
puckeringat base of tumor, intervalgrowth of pancreasforminga U-shapedloop
{ lacelike/ reticularsurfacepatternCHARACTERISTIC r/ oral contrastmaterialmay not enter loop
for villousadenoma(occasionally in tubularadenoma) r/ may resultin biliaryobstruction(increasedpressureat
DDx: (1) Nonneoplastic: hyperplastic polyp,inflammatory amPulla)
pseudopolyp, lymphoidtissue,ameboma, Rx: antibiotictherapy
tuberculoma, foreign-body granuloma,
malacoplakia,heterotopia,hamartoma
(2) Neoplasticsubepithelial:lipoma,leiomyoma, AIDS
neurofibroma, hemangioma,lymphangioma, involvement
0 Gastrointestinal due to opportunistic
endothelioma, myeloblastoma, sarcoma, +
infections AIDS-associatedneoplasms!
lymphoma,entericcyst,duplication, varix, at multiplesiteswith single/
0 Pathologicabnormalities
pneumatosis, hematoma,endometriosis organismsare frequent!
severalopportunistic

ADENOCARCINOMA OF SMALL BOWEL A. VIRALPATHOGENS

Frequency: about50 x less commonthan colonic 1. Cytomegalovirusinfection
carcinoma 0 Mostcommoncauseof life{hreatening
Riskfactors: Crohn disease,sprue, Peutz-Jeghers opportunistic viralinfectionin AIDS patients!
syndrome,Lynchsyndromell, congenital Organism; double-stranded DNA virus of the
bowelduplication, ileostomy,duodenal/ herpesfamily
jejunalbypasssurgery lnfection: ubiquitousamong humansoccurringat
Histo: mostlymoderatelyto well differentiated; may an early age in populationswith poor
arisein villoustumors/ de novo;no correlation sanitation+ crowdedlivingconditions
betweensize and invasiveness 0 Resultof reactivationof latentvirus in previously
Location: duodenum(-50%, especiallynearampulla), infectedhost!
jeju n u m> i l e u m Prevalence: 13'/. of all gastrointestinal diseasesin
"overhangingedges"(60%) AIDS patients
{ annularstricturewith
r/ lobulated/ ovoid polypoidsessilemass (41%) Path: infectionof endothelialcells leadsto small
0 Duodenaltumorstend to be papillary/ polypoid! vesselvasculitisresultingin hemorrhage,
r/ ulceratedmass (27%) ischemicnecrosis,ulceration
CT: Histo: largemononuclear epithelial/ endothelial
r/ soft-tissuemass with heterogeneousattenuation cellsthat containintranuclear /
r/ moderatecontrastenhancement cytoplasmatic inclusionswith surrounding
Cx: intussusception inflammation
DDx: lymphoma(lymphadenopathy morebulky) Location: colon> smallbowel(terminalileum)>
esophagus> stomach
AFFERENT LOOP SYNDROME @ Esophagus
= PROXIMAL { single/ multiplelargesuperficial ulcers
LOOP/ BLINDLOOPSYNDROME
= partialintermittentobstructionof afferentloop leadingto @ Small bowel
overdistensionof loop by gastricjuices after Billroth-ll r/ luminalnarrowingsecondaryto markedbowel
gastrojejunostomy wall thickening

./ thickenedirregularfolds(vasculitisleadingto
thrombosis+ ischemia)
r/ penetratingulcer+ pe*oration
r/ CtrlV pseudotumor(uncommon)
@ Colon (CMVcolitis)
. hematochezia,crampyabdominalpain,fever
r/ tindingsof toxic megacolon
r/ discretesmallwell-defined nodules(similarto
fymphoidnodularhyperplasia)throughout
entirecolon
r/ aphthousulcerson backgroundof normal
mucosa
r/ markedbowelwall thickening
r/ double-ring/ targetsign on Ct lOueto
increasedsubmucosaledema)
r/ ascites
^/ inflammationof pericolonicfat + fascia
Rx: ganciclovir(effectiveinTS%)
2. Herpes simplex virus infection
0 Resultof reactivationof latentvirus in previously
infectedhost
Organism; neurotropicDNA virus of herpesfamily
Prevalence: 7Oo/" for type 1, 1G/ofor type 2
(endemicin UnitedStates);type 2
much morecommonin AIDS
lnfection: directinoculationthroughmucous
membranecontact;from dormant
state in root gangliareactivated
+ transportedvia efferentnervesto
mucocutaneoussurface
Location: oral cavity,esophagus,rectum,anus
r/ multiplesmalldiscreteulcers-
3. HIV infection
0 Not an AlDS-defining illness!
Infection: acute HIV-infection with transient
immunosuppression i duringAIDS
r/ >2-cmlargesolitaryulcerin the mid-or distal
esophagus(HlV-infected cellscausealterations
in cytokinesresultingin infiltration
of
inflammatorycells intcjsubmucosa+ destruction
of mucosa)
Rx: corticosteroids
B . FUNGALPATHOGENS
1. Candidiasi s
0 The absenceof thrushdoes not excludethe
diagnosisof candidaesophagitis!
Organism; commensalfungusCandidaalbicans
Prevalence: 10-20"/" (in United States);up to g0%
in developingcountries
Location: oral cavity,esophagus
r/ discretelinear/ irregularlongltudinally oriented
fillingdefectsin esophagus
Cx: disseminatedsystemiccandidiasis(rare
+ indicativeof granulocytopenia from
chemotherapy I directinoculationvia
catheter)
Gastrointestinal Disorders 789
2. Histoplasmosis
Organism; dimorphicopportunisticfungus
Prevalence: 10o/"Gl involvementwith
disseminated histoplasmosis in Al DS
patients
L<lcation: colon > terminalileum
{ segmentalinflammation / applecore lesion/
bowelstricture
r/ hepatosplenomegaly
r/ mesentericlymphadenopathy
^/ diffusehypoattenuation
of spleen
C. PROTOZOANPATHOGENS
1. Cryptosporidiosis
0 One of the most commoncausesof enteric
+ biliarydiseasein AIDS patientsl
Organism: intracellularparasiteCryptosporidium
Prevalence; isolatedin6% of all patientswith
AIDS;in 16h (in UnitedStates)+ in
up to 48/" (in developingcountries)in
patientswith diarrhea
. severecholeralike debilitating diarrheawith fluid
loss of 10-17 L/day
Location: jejunum> othersmallbowel> stomach
> colon
r/ Cryptosporidium antritis(= ?rea of focal gastric
thickening+ ulceration)
r/ small boweldilatation(increasedsecretions)
r/ regularfoldthickening+ effacement(atrophy,
, blunting,fusion,lossof villi)
! "toothpaste"appearanceof small bowel
(mimickingsprue)
^/ dilutionof barium(hypersecretion)
r/ markedantralnarrowing(extensiveinflammation)
^/ ntOS-related cholangitis
Dx: microscopicidentificationin stool/ biopsy
2. Pneumocystosis
0 Likelyto occur in patientstreatedwith
aerosolized pentamidine !
Organism; eukaryoticmicrobePneumocystis
cannrl
Prevalence.'pulmonaryinfectionin T5o/"of AIDS
patients;in <1Yodissemination
Location: liver,spleen,lymphnodes
r/ hepatic+ splenic+ nodal punctatecalcifications
{ multipletiny echogenicfoci in spleen
{ multiplelow-attenuation lesionsof varyingsize in
spleen(foamyeosinophilic material)with
subsequentlyprogressiverimlike/ punctate
calcifications
D. BACTERIALPATHOGENS
1. Tubercul osi s
0 Most commoncause of seriousH|V-related
infectionworldwidewith tendencyto occurearlier
than otherAIDS-defining opportunisticinfections!
Prevalence: 4/o (in United States) + 43% (in
developingcountries)of HIV-infected
persons
790 Radiology Review Manual

lnfection: swallowingof infectedsputum; with contrast

{ abdominallymphadenopathy
hematogenous spreadfrom pulmonary enhancement
focus;directextensionfrom lymph
node 2. lsospora belli
Location: lymphnodes,liver,spleen,peritoneum, 0 Infectionresemblescryptosporidiosis
Gl tract (especiallyileum,colon, Organism; protozoanPathogen
ileocecalvalve) Histo: oval oocystswithinbowel lumen/
r/ low-attenuationmesentericlymphadenopathy epithelialcells;localizedinflammation;
(suggestiveof necrosis) fold atroPhY
r/ segmentalulceration Location: smallintestine
r/ inflammatorystricture . severewaterydiarrhea
{ hypertrophiclesionresemblingpolyp or mass r/ toto thickening

2. Mycobacterium avium complex infection F. AIDS-ASSOCIATED NEOPLASMS

= PSEUDO-WHIPPLE DISEASE in AIDS 1. Kaposisarcoma
0 Most commonopportunisticinfectionof bacterial
originin AIDS patients! 2. Non-HodgkinlymPhoma
0 Most commonnontuberculousmycobacterial 0 2nd most commonAIDS-associated neoplasm
infectionin AIDS patients! Prevalence: in 4-10"/"of AIDS patients(60 times
Organism; facultativeintracellularacid-fast higherriskcomparedwith general
bacillusM. avium/ M. intracellulare population); occursin all AIDS risk
lnfection: invasionof Peyerpatches+ adjacent groups
mesentericlymphnodes Histo: multiclonal B-celllymphomaof highor
Histo: true granulomaswith Langhansgiant intermediategrade
cells and caseousnecrosisare rare . at initialpresentation widelydisseminated
becauseinfectionoccursin patientswith diseaseoftenwith extranodalinvolvement
advanceddiseaseand a CD4 cell count Location: CNS, bone marrow,Gl tract (stomach,
of <100/pL smallbowel)
. diarrhea,malabsorption (similarclinicalpictureas @ Stomach
in Whipplediseasecausedby Mycobacterium { circumferential llocal wall thickening
avium-intracellulare) { muralmass+ ulceration
Location: jejunum(mostcommon) @ Smallbowel
{ milddilatationof middle+ distalsmallbowel ri diffuse/ focalwall thickening
r/ wall thickeningof small bowel loops r/ excavatedmass
^/ diffuseirregularmucosalfoldthickeningand { solitary/ multipleliverlesions
nodularitywithoutulceration
r/ mesenteric+ retroperitoneal lymphadenopathy Differential diagnostic considerations:
(1.0-1.5cm in size)with homogeneous soft- 1. Splenomegaly (31-45%)
tissueattenuationcausingsegmentalseparation Cause: nonspecific(most),lymphoma,infection
of smallbowelloops (M. avium-intracellulare,P. carinii)
{ hepatosplenomegaly 2. Lymphadenopathy (21-60%)
r/ multipletiny echogenicfoci in liver+ spleen Cause: reactivehyperplasia(most),Kaposi
(occasionallylarge hypoechoic/ low-attenuation sarcoma,lymPhoma,infections
lesions) Size: <3 cm in diameter(in 95%)
Dx: (1) Visualization of largenumbersof 3. Hepatomegaly(20%)
acid-fastbacilliin foamy
intracellular Cause: nonspecific,hepatitis,fatty infiltration,
histiocytesof tissuespecimens lymphoma,Kaposisarcoma
(2) Tissueculture 4. AIDS-related cholangioPathY:
DDx: Whippledisease(positivewith periodicacid- Organism; CMV, CryPtosPoridium
Schiffstainjust like M. avium,but not with r/ papillarystenosisof CBD
acid-faststain,responsiveto tetracyclines) ./ dilatationof extra-and intrahepaticbile ducts
ri periductalfibrosis
E . O T HE RI NFE C T ION S of bileductsresembling
r/ strictures+ irregularities
1. Bacillaryangiomatosis primarysclerosingcholangitis
Organism; RickettsialesBartonellahenselae ri intraluminalpolypoidfillingdefects
Histo: characteristicpatternof vascular 5. AIDS-related esoPhagitis:
proliferation
with bacilli Organism; Candida,herpessimplex,CMV
Location: cutis (mimickingKaposisarcoma), rl giantesophagealulcer: HIV (76%),CMV (14%\
liver,spleen,lymphnodes r/ esophagealfistula/ perforation:tuberculosis,
{ peliosis(blood{illedcysticspaces)of liver/ spleen actinomycosis
 Gastrointestinal Disorders 791

6. Gastritis AMYLOIDOSIS
Organism; CMV (GE junction+ prepyloricantrum), = group of heterogeneousdisorderscausedby interstitial
Cryptosporidium(antrum) depositsof a protein-polysaccharide
in variousorgans
7. AIDS enteritis leadingto hypoxia,mucosaledema,hemorrhage,
Organism; Cryptosporidium, M. avium complex ulceration,
mucosalatrophy,muscleatrophy
8. A I DSc olit i s
- ischemicbowel
Cause: (a) prolongedantigenicstimulation
of RES by
- acute appendicitis
chronicinfection
- neutropenic colitis (b) disorderof immunoincompetence
- pseudomembranous colitis (c) aging
- infectiouscolitisi ileitis
(d) idiopathic
L Bowelobstruction
(a) infection H i sto: amorphouseosi nophi l i chyal i nemat er ial
(b) intussusception: Kaposisarcoma,lymphoma depositedaroundterminalbloodvessels,stains
with Congo red + crystalviolet;green
AM E B I A S I S birefringence underpolarizinglight;amyloid
= primaryinfectionof the colonby protozoanEntamoeba fibrilshave B-pleatedsheet structure
histolytica (= B fibrilloses)
Countries:worldwidedistribution, mostcommonin warm
climates;SouthAfrica,Egypt,India,Asia, Biochemical classification (1979):
Central+ SouthAmerica(20%);UnitedStates 1. A L amyl oi dosi s
(5%) (A = amyl oi dosi s,
L = l i ghtchai ni mmunog lobulin)
Route: contaminatedfood /water (humancyst carriers); . monoclonalproteinin serum+ urine
cyst dissolvesin small bowel;trophozoitessetile . occursin primaryamyloidosis + myeloma-
in colon;proteolytic enzymes+ hyaluronidase associatedamyloidosis
lyse intestinalepithelium;may embolizeinto Histo: massivedepositsin muscularismucosae
portalvenous+ systemicblood system submucosa
Histo: amebicinvasionof mucosa+ submucosacausing r/ thickeningof foldswith polyps/ largenodules
tiny ulcers,whichspreadbeneathmucosa 2. SAA amyloidosis(S = Serum,AA = amyloidA)
+ merge into largerareasof necrosis;mucosal . occursin secondafy= toactiveamyloidosis
sloughing;secondarybacterialinfection Histo: expansionof laminapropria
. asymptomatic for monthsi years r/ coarsemucosalpattern+ innumerable fine
. acute attacksof diarrhea(loosemucoidbloodstained granularelevations
stools) 3. AF amyloidosis (A = amyloid,F = familial)
. fever, headache,nausea . AF prealbuminas precursorof fibrils
Location; (areasof relativestasis)rightcolon + cecum . occursin familialamyloidosis
(90%)> hepatic+ splenicflexures> 4. AS amyloidosis(A = amyloid,S = senile)
rectosigmoid . AS prealbuminas precursorof fibrils
r/ loss of normalhaustralpatternwith granular . occursin senileamyloidosis
, appearance(edema,punctateulcers) r/ massiveamyloiddeposition
{ "collarbutton" ulcers 5. AH amyloidosis(A = amyloid,H = hemodialysis)
^/ cone-shapedcecum . 0, microglobulinas precursorof fibrils
r/ severalcm long stenosisof bowel lumen in transverse 6. AE amyloidosis(A = amyloid,E = endocrine)
colon,sigmoidcolon,flexures(resultof healing . calcitoninproducedby medullarythyroid
, + fibrosis);in multiplesegments carcinomais precursorof fibrils
ri ameboma= hyperplasticgranulomawith bacteriaf
invasionof amebicabscess;usuallyannular Reimann classification (1 935):
+ constricting / intramuralmass/ cavitycontinuouswith 1. Primary= idiopathicamyloidosis
. bowel lumen; shrinkageundertherapyin 3-4 weeks = probablyautosomaldominantinheritance with
^/ ileocecalvalve thickened+ fixed in ofen positionwith immunologically determineddysfunctionof plasma
reflux cells
r/ involvement of distalileum(10%) . absenceof discerniblepreceding/ concurrent
Dx: stool examination/ rectalbiopsy disease
Cx: (1) Toxic megacolonwith perforation Location: (predominant involvementofconnective
(2) Amebicabscessin liver(Z%),brain,lung tissues+ mesenchymal organs)heart
(transdiaphragmatic spreadof infection), (90%),lung (30-70%),liver(35%),
pericolic,ischiorectal, subphrenicspace spleen(40'h), kidneys(35%),adrenals,
(3) lntussusception in children(dueto ameboma) tongue(40/"), Gl tract(70%),skin
(4) Fistulaformation(colovesical,rectovesical,
rectovaginal, enterocolic) r/tendenc,
; ;::',T:'l
J3311*.,
792 Radiology Review Manual

2. Secondaryamyloidosis(mostcommonform) ^/ pseudoobstruction = physical+ plain-filmfindings

. following/ coexistentwith prolongedinfectious / suggestingmechanicalobstruction with patent
inflammatoryprocesses large+ small bowel on barium examination
Cause: rheumatoidarthritis(in 2O/.),Stilldisease, (involvement of myentericplexus)
tuberculosis,osteomyelitis,leprosy, Cx: small bowel infarction
chronicpyelonephritis, bronchiectasis, @ C ol on(27% ):
ulcerativecolitis,Waldenstrdmmacro- ./ pseudopolyps in colon
globulinemia, familialMediterranean fever, @ B one:
lymphoreticular malignancy, paraplegia { bone cysts
Location: spleen,liver,kidneys(>80%),breast, @ Liver:
tongue,Gl tract,connectivetissue Path: extracellulardepositionof amyloidin the spaces
{ small amyloiddeposits of Disse (= narrowgaps betweenendothelial
3. Amyloidosisassociatedwith multiplemyeloma liningsof sinusoidsand hepatocytes of hepatic
. may precededevelopment of multiplemyeloma lamina)with progressive encroachment on
lncidence; 10-15% hepaticparenchymal cells+ sinusoids
{ primaryamyloidosiswith osteolyticlesionsin . hepaticfunctionusuallypreserved
myelomatous disease C T:
4. Tumor-forming / organ-limitedamyloidosis r/ hepatomegaly
. relatedto primarytype r/ regionsof low attenuationwith decreasedcontrast
(a) hereditaty= familialamyloidosis enhancement
(b) senileamyloidosis (limitedto heart lbrain I @ S pl een:
pancreas/ spleen) Histo:(a) nodularform involvinglymphfollicles
r/ large localizedmasses (b) diffuseform infiltratingred pulp
ri discretemasses
0 Gl involvementin primarymorecommonthan in r/ splenomegaly (4-13%)
secondaryamyloidosis ! MR:
. malabsorption(diarrhea,proteinloss) ./ fZ values significantlylowerthan normal
. occultGl bleeding Cx: spontaneoussplenicrupture(fromvascular
. obstruction fragility+ acquiredcoagulopathy)
. macroglossia Dx: by rectal/ gingivalbioPsY
DDx: Whippledisease,intestinallymphangiectasia,
@ Esophagus(11%) lymphosarcoma
^/ loss of peristalsis
./ megaesophagus ANGIODYSPLASIA OF COLON
@ Stomach(37%) = VASCULAR ECTASIA = ARTERIOVENOUS MALFORMATION
. postprandialepigastricpain + heartburn Cause: ?; acquiredlesion
. acute erosivehemorrhagicgastritis Associatedwith: aortic stenosis (20%)
(a) diftuseinfiltrativeform Incidence at autopsy: 2o/o
./ small-sized stomachwith rigidity+ lossof Age: majority>55 years
simulatinglinitisplastica(from
distensibility Location:(a) cecum+ ascendingcolon(majority)
thickeningof gastricwall) (b) descending+ sigmoidcolon (25%)
rl effaced rugal pattern Site: usuallyat antimesenteric border
./ diminisned/ absentperistalsis . chronicintermittent low-gradebleeding
{ markedretentionof food . occasionally massivebleeding
(b) localizedinfiltration(oftenlocatedin antrum) { "arterialtuft" = clusterof vesselsduringarterialphase
ri irregularlynarrowed+ rigidantrum alongantimesenteric border
r/ thickenedrugae { earlyopacification of drainingileocolicvein
{ superficialerosions/ ulcerations { denselyopacifieddilatedtortuousileocolicvein into late
(c) amyloidoma= well-definedsubmucosalmass venousphase
@ Small bowel (74%) r/ contrastextravasation(unusual)
(a) diffuseform (morecommon)
{ diffuseuniformthickeningof valvulae ANISAKIASIS
conniventesin entiresmall bowel = parasiticdiseaseof Gl tract
r/ broadenedflat undulatedmucosalfolds (mucosal Cause: ingestionof Anisakislarvaepresentin raw I
atrophy) undercooked fish (mackerel,cod, pollack,
r/ "jejunalization"
of ileum herring,whiting,bonito,squid)consumedas
r/ impairedintestinalmotility sashi misushi
, ,cevi che,l omi -l omi
r/ smallboweldilatation Organism.' worm with straight/ serpentine/ circular
(b) localizedform (lesscommon) threadlikeappearance
i/ multiplepea- / marble-sizeddeposits 0 Site of penetrationby larvaedeterminesclinicalforml
 Gastroi ntestinal Disorders 793

@ Gastricanisakiasis rectovesicalseptum+ cloacalmembrane;anal

. acutegastricpain,nausea,vomitinga few hours membrane rupturesby 9th week
after ingestion(DDx: acutegastritis,pepticulcer, ln 48% associatedwith: (part of VACTERL syndrome)
food poisoning,neoplasia) (1) GU anomalies(20%):
. eosinophilia renalagenesis/ ectopia,vesicoureteral reflux,
r/ mucosaledema obstruction, hypospadia(3.1%); M > F
r/ about 3-cm-longthreadlikefilfingdefects(= larvae) (2) Lumbosacrafsegmentationanomalies(30%):
@ lntestinalanisakiasis dysplasia,agenesis,hemivertebrae
. diffuseabdominaltenderness/ colickyabdominal ( 3 )G l a n o m a l i e(s1 1 % ) :
pain,nausea,vomiting(DDx: acuteappendicitis, esophagealatresia+ tracheoesophageal fistula
regionalenteritis,intussusception, ileus,diverticulitis, (4%),duodenalatresia/ stenosis
neoplasia) (4) Cardiovascular anomalies(8%)
. leukocytosiswithouteosinophilia(frequent) (5) Abdominalwall (2%)
His t o: m a rk e d e d e m a ,e o s i n o p h i l i c i n fi l trates, (6) Cleftlip-cleftpalate(1.6%)
granulomaformation (7) D ow nsyndrome(1.5% )
r/ thickenedfolds (8) Meningomyelocele (0.5%)+ occultmyelodysplasia
r/ disappearanceof Kerckringfolds (9) Others(8%)
r/ thumbprinting/ saw-toothappearance
r/ irregularluminalnarrowing ANTRALMUCOSALDIAPHRAGM
r/ eosinophilicascites(DDx: eosinophilic = ANTRAL WEB
gastroenteritis,
hypereosinophilic syndrome) Age range; 3 monthsto 80 years
Cx: ileus Associatedwith: gastric ulcer (30-50%)
@ Colonicanisakiasis(rare) . symptomaticif opening<1 cm
DDx: colonictumor Location: usually1.5 cm from pylorus(range0-7 cm)
r/ constantsymmetricband of 2-3 mm thickness
ANORECTAL MALFORMATION traversingthe antrumperpendicular to longaxis of
(1) Rectalatresia stomach
= open anus + atreticrectalsegmentsuperiorto anus r/ "doublebulb"appearance(in profile)
+ no fistula ./ concentric/ eccentricorifice
(2) Ectopicanus r/ normalperistalticactivity
= fistulousopeningof boweldue to failureof terminal
bowelto descendnormally
Site of arrest: high/ low colon arrest= above/ below A P P E N D IC ITIS
puborectalsling Prevalence: 1-4% in childrenwith acute abdominalpain
. rnostcommonanomalyof anorectalsegment
Lifetimerisk: 7-9% in Western world population
. analdimple+ externalsphincterin normalposition
Etiology: obstructionof appendiceallumenby lymphoid
Locationof fistula:perineum,vestibule,vagina, hyperplasia (60%),fecolith(33%),foreign
urethra,bladder,cloaca bodies(4/"), stricture,tumor,parasite;Crohn
r/ low small bowel/ colonicobstruction disease(in 25%)
\i "M" line accuratelyrepresentslevelof puborectal Cause: luminalobstruction from
muscle= linedrawnhorizontally throughjunctionof (a) fecolith(11-52%) = hard crushableconcretionsfrom
lower 113+ upper213of ischiumon lateral inspissationof fecal material+ inorganicsalts
radiograph (b) appendiceal calculus= hard noncrushable calcified
(3) lmperforateanus stone(7-15%)
= blindendingof terminalbowel+ no fistula (c) lymphoidhyperplasia
(4) Cloacalmalformation (d) foreignbody
(5) Cloacalexstrophy (e) parasite
Embryology: (f) primarytumor: carcinoid,adenocarcinoma, Kaposi
duringweeks 3 and 4 the dorsalpart of the yolk sac sarcoma,lymphoma
folds are incorporatedinto embryoformingthe primitive (g) metastatictumor: colon cancer,breastcancer
hindgut consistingof distalpart of transverse Pathogenesis:
+ descending+ sigmoidcolon,rectum,superiorportion continuedsecretionof mucusin appendiceal obstruction
of analcanal,epitheliumof urinarybladder,and mostof elevatesintraluminal pressure+ distendslumen;venous
the urethra; at 4 weeks the transverserectovesical engorgement+ arterialcompromise+ tissueischemia
septum descendscaudallybetweenallantoisand ensuesafterintraluminal pressureexceedscapillary
hindgutdividing the cloaca into urogenitatsinus perfusionpressure
ventrally+ anorectalcanal dorsally; by 7th week the Peak age: 2nd decade;thereafterdecliningincidence;
rectovaginalseptumfuseswith cloacalmembrane M:F = 3:2 (in teens/ youngadults,thereafter
creatinga urogenitalmembrane ventrally+ anal 1: 1 )
membranedorsally;perineumis formedby fusionof 0 Rare underthe age of 2 yearsl
794 Radiology Review Manual

B0% clinicalaccuracy(78-92% in males,58-85% in r/ pneumoperitoneum (rare)

females): r/ loss of fat planes:
Diagnostic dilemma (20-35%) : r/ focal increasein thicknessof lateralabdominalwall
in elderly,ovulatingwomen,infants/ youngchildren in 32/" (= edema betweenproperitonealfat line
O 32-45% rate of misdiagnosisin women between + cecum)
ages 20-40! r/ loss of properitonealfat line
0 5-25% false-negativeappendectomyrate for r/ loss of pelvicfat planesaroundthe bladderI righl
pediatricpopulation! obturator(= fluid/ pus in cul-de-sac)
pain r/ lossof definitionof rightinferiorhepaticoutline
. mildpoorlylocalizedvisceralpain of 4-6 hours (= free peritonealfluid)
durationreferredto epigastrium+ periumbilical ^/ distortionof psoas margin+ flank stripes
region { scoliosis(dueto muscleirritation)
. crampypain migratesinto RLQ pain over appendix BE i UGI (accuracy50-84%):
= McBurneysign (72%)and becomescontinuous ^/ failureto fill appendixwith barium(normalfindingin
+ more severe(somaticpain) up to 35%)
anorexia,nausea,vomiting(40%) r/ indentationalong medialwall of cecum (= edema at
afebrile/ low-gradefever (56%) base of appendix/ mattedomentum/ periappendiceal
0 Suspectperforationwith temperature>38.3"C abscess)
leukocytosiswith left shift (88%)
Graded-compression US (85% sensitive,92o/"specific,
"MANTRELS"score of 10 78-96% accurate,91-94/" PPV, 89-97% NPV):
Clinicalscoring system:
Migrationof pain to RLQ 0 Nondiagnostic studyin 4h due to inadequate
Anorexia compression of RLQ
N a u s e aa n dv o m i t i n g. . . . . . . . . . . . . . . . 0 Usefulin ovulatingwomen(false-negative
T e n d e r n e sisn R L Q . . . . . . . . . . . . . .2. . . . . appendectomyrate in males 157",in females35%)
Rebounp d a i n. . . . . . . . . . . . . . . . . . . .1. . . . . . . . + infants/ children
E lev at edt em p e ra tu re ................ 1 { visualization of noncompressible appendixas a blind-
L e u k o c y t o s .i .s. . . . . . . . . . . . . . . . . . .2. . . . . endingtubularaperistalticstructure(seen only in 2o/"
S hif tof W B C c o u n tto l e ft.......... 1 of normaladults,but in 50% of normalchildren)
r/ laminatedwall with targetappearanceof >6 mm in
Location: totaldiameteron crosssection(81%SPECIFIC)/
(a) base of appendix: posteromedialwall of cecum muralwall thickness>2 mm
3 cm below ileocecalvalve ^/ lumenmay be distendedwith anechoic/ hyperechoic
subcecal,retroileal,
(b) tip of appendix:retrocecal, material
preileal,withinpelvis(30%),extraperitoneal(5%) ^/ pericecal/ periappendiceal fluid
^/ increasedperiappendiceal echogenicity(= infiltration
Abdominalplainfilm (abnormalities seen in <50%): of mesoappendix / pericecalfat)
0 Plain-filmfindingsbecomemoredistinctiveafter { enlargedmesentericlymphnodes
perforation,while clinicalfindingssubside/ simulate ./ tossof wall layers= gangrenousappendix
otherdiseases! r/ perforatedappendix(23-73/"):
{ usuallylaminatedcalcifiedappendicolith in RLQ (in r/ lossof echogenicsubmucosallayer
7-15%\ r/ appendixno longervisualized(40-60%)
0 Appendicolith + abdominalpain =90"h probability r/ loculatedperiappendiceal / pelvicfluidcollection
of acuteappendicitisl + gas bubbles(= abscess)
0 Appendicolithin acute appendicitismeans a high r/ prominenthyperechoicmesoappendix / pericecalfat
probabilityfor gangrene/ perforation! r/ visualization of appendicolith (6%)= bright
{ cecalchanges: echogenicfocuswith cleandistalacousticshadowing
rl thickeningof cecalwall { gas bubbleslocalizedto perforationsite
{ water-densitymass + paucityi absenceof intestinal ri hypoechoiczoneswith poor margination within
gas in RLQ (in 24/" of perforations) inflamedfat 1=phlegmonous appendicitis)
{ "cecalileus"= gas-fluidlevelin cecumin gangrene { sympathetic thickeningof adjacentterminalileum
(= local paralysis) + ascendingcolon
{ colon cutoffsign = amputationof gas at the hepatic False-negative US:
flexure(in 20% of perforations)due to spastic (a) failureto visualizeappendix
ascendingcolon - inabilityof adequatecompression
r/ small bowel obstructionpattern= slnall bowel - aberrantlocationof appendix(eg, retrocecal)
dilatationwith air-fluidlevels(in 43o/"of perforations) - appendicealperforation
r/ extraluminal gas (in 33% of perforations): (b) earlyinflammation limitedto appendiceal tip
^/ gas loculation False-positiveUS:
r/ mottledbacteriogenicgas (a) normalappendixmistakenfor appendicitis

(b) alternatediagnosis:Crohndisease,pelvic
inflammatorydisease,inflamedMeckel
diverticulum
(c) spontaneousresolutionof acute appendicitis
ColorDopplerUS:
./ increasedconspicuity(= inspsasein size + number)of
circumferential
vesselsin and aroundthe wall of the
aPPendix(= hyperemia)
r/ decreasedresistanceof arterialwaveforms
r/ continuous/ pulsatilevenousflow
r/ decreased/no perfusion= gangrenousappendicitis
CT (87-100% sensitive,89-98% specific,g3-98%
accurate,92-98% PPV, 95-1 00% NPV):
r/ normalappendixvisualizedin 67-100%:
\l 1-2 cm below ileocecaljunctionfrom posteromedial
aspectof cecumwith a diameterof up to 10 mm
./ abnormalappendix:
r/ distendedlumen(appendix>7 mm in diameter)
r/ circumferential wall thickening
./ homogeneously enhancing*Lttt mural
stratification("targetsign")
{ appendicolith = homogeneous / ringlikecalcification
(25%)
{ distalappendicitis = abnormaltipof appendix
+ normalproximalappendixand normalcecal apex
! periappendicular inflammation (98%):
r/ linearstreakydensitiesin periappendicular / Organism; Ascarislumbricoides= roundwormparasite,
pericecal/ mesentericlpelvic fat
! subtlecloudingof mesentery
./ localfascialthickening
{ free peritonealfluid
r/ mesentericlymphadenopathy
r/ circumferential / focal cecal apicalthickening(80%):
! "arrowheadsign"= funnelof contrastmediumin
cecumsymmetrically centeringaboutoccluded
orificeof appendix
{ perforationof appendix:
r/ nonvisualization of appendix(due to fragmentation)
./ pericecalphlegmon= pericecalsoft-tissuemass
(DDx: ileocolitis with secondaryinflammation
appendix)
r/ pericecal/mesentericlpelvicabscess= poorly
encapsulated single/ multiplefluidcollectionwith
air I extravasatedcontrastmaterial
{ appendicolith
r/ extraluminal air
./ markedileocecalthickening
{ localizedlymphadenopathy
r/ peritonitis
{ small-bowelobstruction
False-negative CT:
(a) overlappingrangein maximalappendiceal
diameterbetweeninflamed+ uninflamedappendix
(b) appendixmistakenfor unopacifiedbowel
(c) inflammationlimitedto appendicealtip
Prognosis:
(1) mildacuteappendicitis may resolvespontaneously
(afterreliefof incitingobstruction)
Gastrointestinal
Disorders 795
(2) recurrentappendicitis (10%) = repeatedsimilar
episodicattacksof RLQ pain leadingto
appendectomy+ showingacute inflammation
(3) chronicappendicitis (1%)= RLQ painof >3 weeks
+ no alternativediagnosis+ chronicactive
inflammationon histology+ reliefof symptomsafter
appendectomy
(4) Mortalityrate of 1"/"(associatedwith perforation)
Cx: perforation(13-30-737"),abscessformation,
peritonitis,
woundinfection,sepsis,infertility,
adhesions,bowelobstruction,death
Rx: findingof appendicolith is sufficientevidenceto
performprophylacticappendectomyin
asymptomaticpatients(50% have perforationI
abscessformationat surgery)
DDx: colitis,diverticulitis,
epiploicappendagitis, small
bowelobstruction, infectiousenteritis,duodenal
ulcer,pancreatitis, intussusception, Crohndisease,
mesentericlymphadenitis, ovariantorsion,pelvic
inflammatorydisease
0 Only 22-38% of childrenreferredfor suspected
appendicitisactuallyhave appendicitis
ASCARIASIS
= rT'rost
commonparasiticinfectionin world;cosmopolitan
occurrence; endemicalongGulf Coast,Ozark
Mountains,Nigeria,SoutheastAsia
15-35 cm in length;productionof 200,000
eggs daily
Cycle: infectionby contaminatedsoil, eggs hatch in
duodenum,larvaepenetrateintovenules/
lymphatics,carriedto lungs,migrateto alveoli
and up the bronchialtree,swallowed,
maturationin jejunumwithin2.5 months
Age: childrenage 1-10 years
. colic
. eosi nophi l i a
. appendicitis
. hematemesis / pneumonitis
of . jaundice(if bileductsinfested)
Location: jejunum> ileum(99%),duodenum,stomach,
CBD, pancreaticduct
r/ t S-gS-cm-long tubularfillingdefects
r/ barium-filledentericcanaloutlinedwithinAscaris
r/ whirledappearance,occasionallyin coiledclusters
("bolusof worms")
Cx: (1) Perforationof bowel
(2) Mechanicalobstruction
BANNAYAN.RILEY.RUVALCABA SYNDROME
= RUVALCABA-MYHRE-SMITH SYNDROME
Cause: autosomaldominanttransmission
. pigmentedgenitallesions
r/ hamartomatousintestinalpolyps(in 45%): usuallyin
distalileum+ colon
^/ macrocephaly
{ subcutaneousand viscerallipomas+ hemangiomas
796 Radiology Review Manual

BARRETTESOPHAGUS 3. Adenocarcinoma (0-10-46%;)40{old higher

= BARRETT SYNDROME riskthan generalpopulation
= replacement of stratifiedsquamousepitheliumby r/ plaquelike/ focalirregularity
/ nodularity
/
metaplastic columnarepithelium(Barrettepithelium) sessilepolyps
containinggobletcellsbut no parietalcells Rx: (1) stopsmoking,avoidbedtimesnacks+ foods
Cause: chronicgastroesophageal refluxwith epithelial that lower LES pressure,lose excessweight
injuryfrom esophagitis (2) suppressgastricacidity: antacids,Hr-receptor
Contributing factors: antagonists(cimetidine, ranitidine,famotidine),
geneticinfluence,reducedLES pressure,transientLES H*K*-adenosintriphosphatase inhibitor
relaxation, hiatalhernia,delayedacidclearance, (omeprazole)
reducedacid sensitivity,duodenogastroesophageal (3) improveLES pressure:metoclopramide,
reflux,alcohol,tobacco,chemotherapy, scleroderma bethanechol
(37"/.),S/P repairof esophagealatresia/ (4) esophagealresectionin high-gradedysplasia
esophagogastric resection/ Helleresophagomyotomy
Histo: (1) specialized columnarepithelium(proximal) BEHQET
SYNDROME
(2) junctional-type epithelium(distalto above) = urcofi]rTron
chronicmultisystem inflammatory disorder
(3) fundic-type epithelium(mostdistally) of unknownetiologywith relapsingcoursecharacterized
Prevalence: in general0.34/"; 7-10'/" of patientswith by mucocutaneous-ocular symptomsas a triad of
advancedchronicrefluxesophagitis aphthousstomatitis, genitalulcers,ocularinflammation
Associatedwith: moderate+ severeesophagitis(94%), Countries: worldwide,most commonin eastern
no / mildesophagitis(6%) Mediterranean countries.easternrim of Asia
Age: 0-15 years and 40-88 years (meanof 55 years); Histo: nonspecific necrotizing vasculitiswithdeposition
M > F; mainlyamongWhites of immunecomplexesin wallsof smallblood
. dysphagia(due to esophagealstricture) vessels
. signsof refluxesophagitis:heartburn,substernalchest Age at onset: 3rd decade; M:F = 2:1
pain,regurgitation Majorcriteria: buccal+ genitalulceration, ocular
. low-gradeupperintestinalbleeding inflammation, skin lesions
. asymptomatic Minorcriteria: thrombophlebitis, Gl + CNS lesions
Location: middleto loweresophagus arthritis,familyhistory
N.B.: the squamocolumnar junctiondoes not . abdominalpain + diarrhea(50%)
coincidewith the GE junction,is @ Mucocutaneous: aphthousstomatitis, papules,
irregularand lies >2-3 cm orad from pustules,vesicles,folliculitis,
the gastroesophageal ju nction erythemanodosum-like lesions
Distribution: circumferential/focal @ Genital: ulcerson penis+ scrotum/ vulva+ vagina
{ several-cm-long stricture(71%)in midesophagus (40%) @ Ocul ar: rel apsi ngi ri docycl i ti s,hypopyon, chor oidit is,
or loweresophagus(60%)[DDx: pepticstricturewithout papillitis,retinalvasculitis
Barrettesophagusl @ Articular: mild nondestructive arthritis
./ largedeep wide-mouthedpepticulcer (= Barrettulcer) @ Vascular: migratorythrombophlebitis
at upwardlydisplacedsquamocolumnar junction/ within @ CNS: chroni cmeni ngoencephal i ti s
c olum narepith e l i u m DDx: Reitersyndrome,Stevens-Johnson syndrome,
^/ fine reticularmucosalpattern(3-30%) resemblingareae SLE, ulcerativecolitis,ankylosingspondylitis
gastricaeof the stomach= netlikeweb of barium-filled
groovessurroundingsmalltufts of mucosa;located IntestinalBehgetDisease
distallyfrom stricture(DDx: gastroesophageal reflux, = largedeeplypenetrating intestinalulcers(HALLMARK)
monilial+ viral esophagitis, superficial spreading lncidence: 1040/"
carcinoma) Location: terminalileum,cecum,ascendingcolon,
r/ thickenedirregularmucosalfolds (28-86%) transversecolon
r/ tine granularmucosalpattern(DDx: refluxesophagitis, r/ deep roundulcerssimilarin appearanceto peptic
acanthosis, leukoplakia, supedicialspreadingcarcinoma, ulcersof stomach/ duodenum
moniliasis/ herpessimplex/ CMV esophagitis) r/ multipleshallow/ longitudinal / aphthoidulcers
r/ gastroesophageal reflux(45-63%) C T:
r/ distalesophagealwidening(3a-66%; due to abnormal r/ polypoidlesion/ thickenedbowelwall (mural
motility) edema associatedwith deep ulcer penetration)
ri hiatal hernia (75-94%) r/ contrastenhancement(71%)
r/ uptakeof Tc-99mpertechnetateby columnarepithelium r/ minimallymphadenopathy, mostly<10 mm
Dx: velvetypinkishred appearanceof gastric-type Cx (56%): panperitonitis with high mortalitydue to
mucosaextendingfrom gastricmucosainto distal tendencyfor perforationat multiplesites;
esophagus(endoscopywith biopsy) fistula;hemorrhage
Cx: (1) Ulceration + penetration into mediastinum Prognosis; recurrencein 40-45% adjacentto surgical
(2) Stricture anastomosis
 Gastrointestinal
Disorders 797

DDx: ulcerativecolitis,Crohndisease MR:

r/ hyperintenselesionson T2Wl (dueto slow flow/
BEZOAR thrombosis)
lpadzahr, = antidote,
Persian counterpoison] Cx: intussusception, volvulus;pressureerosionof bone,
= persistentconcretionsof foreignmattercomposedof osseous+ soft-tissuehypertrophy(secondaryto
accumulatedingestedmaterialin intestines hypervascularity)
lncidence: 0A% (largeendoscopicseries) DDx:
Etiology: materialunable to exit stomach because of (1) Mafuccisyndrome(dyschondroplasia
largesize,indigestibility,
gastricoutlet + osteochondromas + vascularmalformations)
obstruction,poor gastricmotility(diabetes, (2) Klippel-Trenaunay-Weber syndrome(portwine stain,
mixedconnectivetissuedisease,myotonic vascularmalformations, limb hypertrophy)
dystrophy,hypothyroidism) (3) Kasabach-Merrittsyndrome(largevascular
Predisposition: malformations+ consumptive coagulopathy)
previousgastricsurgery (vagotomy,pyloroplasty, (4) Kaposisarcoma
antrectomy,partialgastrectomy),inadequatechewing, (5) Peutz-Jegherssyndrome(congenital polyposis
missingteeth,dentures,massiveoverindulgence of food + melanoticcutaneouslesions)
with highfibercontents (6) Gardnersyndrome(soft-tissuetumors+ sebaceous
. anorexia,bloating,early satietyI may be asymptomatic cysts)

Phytobezoar BLUNTABDOMINAL
TRAUMA
lncidence: 55h of all bezoars CT is imagingmethodof choicefor evaluationof stable
= poorlydigestedfibers,skin + seedsof fruitsand patients
vegetablesusuallyformingin stomach,may become US imagingin the detectionof intraabdominal
injury:
impactedin smallbowel 86% sensitive,99% specific,98o/oaccurate
. historyof recentingestionof pulpyfoods
Food: oranges,persimmons(mostcommon,unripe Hemoperitoneum
persimmonscontainthe tanninshibuolthat Frequency: 29-34% of patientswith abdominal
forms a gluelikecoagulumaftercontactwith visceralinjuryhave no hemoperitoneum
diluteacid) Location: paracolicgutters, pelvis
Site of impaction: stomach,jejunum,ileum CT (negativepredictivevalue of 99.6%):
r/ intraluminal fillingdefectwithoutconstantsite of Attenuation values of blood:
attachmentto bowelwall duringlV contrast administration
andassuming an initially
r/ intersticesfilledwith barium normalhematocrit withoutsignificant
dilutionfrom
r/ coiled-springappearance(rare) intraperitonealfluid(ascites,
urine,succus,lavagefluid)
r/ partial/ completeobstruction - serum (afterhematocriteffect) 0-20 HU
Cx: decubitusulceration+ pressurenecrosisof bowel - fresh unclottedblood 30-45HU
wall,perforation, peritonitis - clottedblood 60-100HU
DDx: lobulated/ villousadenoma,leiomyosarcoma, - activearterialextravasation > 1 8 0H U
metastaticmelanoma,intussusception { "sentinelclot"sign = the highestattenuation value
of bloodclot marksthe anatomicsite of visceral
Trichobezoar injury
, thrix,Greek= hairl
Itrikho- ./ nign-Oensity activearterialextravasationalways
80% are < age 30, almostexclusively in females; surroundedby lower-densityhematoma
Associatedwith: gastric ulcer in 24-70/" (DDx: extravasatedoral contrastis not surrounded
by lower-densitymaterial)
US:
BLUERUBBER
BLEBNEVUSSYNDROME { usuallyanechoicfluidaccumulation in subhepatic
= f?re disordercharacterizedby vascularhamartomasof space(= Morisonpouch)> pouchof Douglas/
skin + visceralhemangiomas predominantlyafflicting paravesicalspace > betweenbowel loops
the Gl tract (but afso liver,spleen,heart,skeletal DDx: bowel contents,urine,bile,ascites
muscle,lung,kidney,thyroid,eyes,CNS) r/ hemoperitoneum score = depth of largestfluid
Etiology: sporadic/ autosomaldominant collectionin cm + 1 pointfor each additionalsite
Path: thin layerof connectivetissue+ singlelayerof with fluid (scoreol <2 managedconservatively)
endothelial cellssurroundingblood-filled ectatic r/ hyperechoic/ occasionallyisoechoicmasses
vessels (= intraperitoneal clot)
. red to deep blue soft painlesscutaneouslesions Prognosis: 17"/"of patientswithouthemoperitoneum
evacuatingunderpressure+ slow refilling(commonly requiresurgical/ angiographic intervention
presentat birth+ increasein size and numberwith age) 0 Peritoneallavagecannotquantifyamountof
.. irondeficiency anemia(dueto spontaneous hemorrhage) hemoperitoneum + resultsin a 19-39% rate of
r/ nodularfillingdefectsthroughoutsmallbowel nontherapeutic surgeries
798 Radiology Review Manual
= Hypoperfusion
Hypovolemia complex
r/ "collapsedcava" sign = persistentflatteningof IVC
(due to decreasedvenous return)
N.B.: abortCT examination as shockis imminent!
,Vsmall hypodensespleen(decreasedenhancement)
./ small aorta + mesentericarteries(due to intense
vasoconstriction)
{ shock nephrogram= lack of renalcontrastexcretion
{ "shockbowel"= dilatationof fluid-filledintestines
+ generalized thickeningof smallbowelfolds
+ increasedenhancement(dueto vasoconstriction of
mesentericvessels)
r/ markedenhancementof adrenalgland
./ intensepancreaticenhancement
BluntTraumaTo Spleen
0 Most frequentlyinjuredintraperitoneal organ in blunt
abdominaltrauma
Associatedwith: other solidvisceral/ bowel injuries
(29%);lower rib fracturesin 44o/",
injuryto left kidneyin 10/", injuryto
leftdiaphragmin 2/"
0 20% of left rib fractureshave splenicinjuryl
0 25% of left renal injuryhave splenicinjury!
Technique; scanningdelay of 60-70 secondsto avoid
heterogeneous splenicenhancement
Categoriesof SplenicInjury
Grade lnjury Description
hematoma subcapsular<25o/o surfacearea
laceration capsulartear <1 cm parenchymaldepth
tl hematoma subcapsular25-50/" surface area;
intraparenchymal <5 cm in diameter
laceration 1-3 cm deepwithoutinvolvement of
trabecularvessel
ill hematoma subcapsular>50/" surfacearea;
rupturedsubcapsular/ parenchymal ;
intraparenchymal >10 cm / expanding
laceration >3 cm parenchymaldepth/ involvement
of trabecularvessels
IV laceration involvingsegmental/hilarvesselswith
devascularization
of >25o/o
laceration completelyshatteredspleen
vascular total splenicdevascularization
CECT (95% accurate):
0 CT not reliableto determineneed for surgical
intervention!
r/ hemoperitoneum (indicatesdisruptionof splenic
capsule)
{ "sentinelclot" (= afea of >60 HU adjacentto
spleen)sensitivepredictorof splenicinjury
= perisplenic hematoma
r/ nign-attenuation area (80-370 HU) = active
extravasation / pseudoaneurysm : 0 Left lobe injurymore often associatedwith
N.B.: activeextravasationof contrastmaterial
usuallyrequiressurgery
./ mottledparenchymalenhancement= contusion
nhypoattenuatingline connectingopposingvisceral
surfaces= linearparenchymaldefect= splenic
laceration:
r/ almostalwaysassociatedwith hemoperitoneum
crescenticregionof low attenuationalong splenic
marginflattening/ indenting/ compressing the
normalparenchyma= subcapsular hematoma
{ roundhypodenseinhomogeneous region
t hyperdenseclot = intrasplenic hematoma
{ hypoattenuating hematomawith complete
separationof splenicfragments= laceration
traversingtwo capsularsurfaces= splenic fracture
{ multiplelacerations= "shattered spleen"
US:
^/ hyperechoicintraparenchymal
region(= dcute
hematoma/ laceration)
^/ anechoicintralesional
collection(= brisk
hemorrhage)
{ diffuselyheterogeneousparenchymalpattern
containinghyper-and hypoechoicareas
(= extensivesplenicinjury)
r/ loss of normalorgancontour(= perisplenicclot)
Sequelae:
(1) scar/ fibrosis
(2) splenicpseudocyst(20-30 HU)
(3) pseudoaneurysm formation
(4) delayedsplenicrupture
= hemorrhage>48 hoursaftertrauma
Cause: subcapsularhematoma
Prevalence: 0.3-20% of bluntsplenicinjuries
Timeof onset: in70/o within2 weeksof injury,in
90% within4 weeks of injury
Prognosis: 52o/osurgery (splenectomy[8%],
splenorrhaphy),48/" nonsurgical
management
Rx: up to 91"/"of stablepatientscan be treated
conservatively with observation;transcatheter
embolization
DDx: (1) Normallobulation/ spleniccleft(smoothly
contoured,mediallylocated)
(2) Adjacentunopacifiedjejunumsimulating
splenictissue
(3) Earlydifferentialenhancementof red and
white pulp (scanobtainedwithin20-50
seconds)
(4) Perisplenic fluidfrom ascites/ urine/ succus/
bile / lavage
Blunt TraumaTo Liver (20%)
0 Secondmost frequentlyinjuredintraabdominal viscus
Associatedwith: splenic injury in 45h
. clinicalmanifestationoften delayedby days / weeks
L o c a t i o n :R > L l o b e
Site: perivascular, parallelingright+ middlehepatic
arteries+ posteriorbranchesof rightportalvein,
avulsionof righthepaticvein from IVC (13%)
damageto duodenum,pancreas,transverse
colon
 Gastrointestinal Disorders 799

r/ widespreadheterogeneousliverechogenicity
Categoriesof LiverInjury
+ absenceof normalvascularpattern(= global
Grade lnjury Description parenchymalinjury)
I hematoma subcapsular Cx: in up to 20%
<10o/"surfacearea
(1) delayedrupture(rare)
laceration capsulartear <1 cm parenchymaldepth
(2) hemobi l i a
ll hematoma subcapsular10-50% surfacearea; (3) arteriovenous fistula/ pseudoaneurysm
intraparenchymal<10 cm in diameter (4) biloma+ infection
laceration 1-3 cm deep and <10 cm long (5) superinfection of hematoma/ devascularized
lll hematoma subcapsular>50o/"surface arca; hepaticparenchyma
rupturedsubcapsulari parenchymal; Rx: conservativetreatmentin up to 80% in adults
intraparenchymal>10 cm / expanding + 97"hin children;transcatheter embolization
laceration >3 cm parenchymaldepth Healing: 1-6-15 months
DDx: (1) beam-hardening artifactfrom adjacentribs/
fV laceration parenchymaldisruption25-75/. of lobe;
from air-contrastlevel in stomach
1-3 Couinaudsegmentsin singleI
(2) Focalfatty infiltration
V laceration disruption>75/" of singlelobe;
>3 Couinaudsegmentsin singlelobe BluntTraumato Gallbladder
(2%)
vascular juxtahepatic venousinjury(HV, IVC) Associatedwith: injuryto liver,duodenum
Vl vascular hepaticavulsion r/ pericholecystic fluid (extraperitoneallocationof GB)
r/ free intraperitoneal fluid
CECT: C E C T:
r/ hypoattenuating hematoma: ri blurredcontourof GB
^/ focalthickening/ discontinuity of GB wall
r/ lenticularconfiguration (= subcapsular
hematoma) ^/ intraluminalenhancingmucosalflap
, usually resolving within6-8 weeks
r/ irregularlinearbranchingi roundregionsof low { hyperattenuating bloodwithinGB lumen
attenuation= laceration ./ mass effecton adjacentduodenum
r/ focal/ diffuseperiportaltracking(in up to 22o/") r/ collapsedGB = GB rupture
due to dissectinghemorrhage r/ focal periportaltracking= GB rupture
/ bile/ dilated
periportallymphatics(secondaryto elevated US:
centralvenouspressure/ injuryto lymphatics) ri focal hypoechoicthickening
r/ alterationin distributionof vessels+ ducts r/ echogenicmasswithinGB lumen
r/ hypodensewedge extendingto liversurface= focal
hepaticdevascularization BluntTraumato Gl Tract(5%)
{ focal hyperdense(80-350 HU) area = active Causein children:MVA (lap belts),bicyclehandlebar,
hemorrhage / pseudoaneurysm childabuse
r/ hemoperitoneum (inabilityof liverveinsto contract) May be associatedwith: Chance fracture;traumatic
! intrahepatic / subcapsular gas (usuallydue to hernia(disruption
of the
necrosis) rectusabdominismuscle)
US: Location: jejunumdistalto ligamentof Treitz>
r/ localizedarea of increasedintraparenchymal duodenum> ascendingcolonat ileocecal
echogenicity (= 2su1ehematoma/ laceration) valve> descendingcolon
. abdominaltenderness(100%sensitive)
. increasedtemperature+ heart rate
. decreasedurineoutput over24 hours
1 27o . lap beltecchymosis

Y--
z Jvo N E C T:
{-
./ pneumoperitoneum (15-32%):
Vt-**@ r/ smallgas bubblesanteriorlynear liver ltrapped
withinleavesof mesentery(withsmallbowel
perforation)
I nr" r/ retroperitoneal air (withdisruptionof duodenum/

ffi
colon)
r/ hypodensefree fluid (58-85%),particularlyin
!,tr 5 interlooplocationdue to perforation
\ : 'ri;:i:,
i:;
l : ; : : 1. ; 4Vo DDx: parenchymalorgan injury/ osseousinjury/
l6 :',li,i:,'i;
largevessel injury/ bladderperforation
1 9 %.:
::.!y'

r/ sentinelclot sign adjacentto bowel

C ECT (88-92% sensitive):
Distribution of Tiaumatic Hepatic Lesions r/ focal discontinuityof bowelwall = directevidence
800 Radiology Review Manual
r/ focalbowelwall thickening> 3 mm (= intramural
hematoma(75%)/ vascularcompromiseand
inflammation due to spillingof bowelcontents):
r/ + intestinalobstruction
r/ hyperdensecontrastenhancementof injuredbowel
wall = delayedvenoustransittime (20%)
r/ strandingof mesentery= streokyhyperattenuating
infiltration/ fluid at mesentericroot (due to
hemorrhage+ inflammatoryresponse)
{ mesenterichematoma(39%)
r/ extravasationof oral contrastmaterial(uncommon),
most dense near perforation
DDx: hyperattenuating blood,extravasating
vascularcontrastmaterial,leak of contrast
materialfrom urinarytract
r/ mesentericpseudoaneurysm
US :
r/ nonspecificfree fluid (98% sensitive)
N.B.: clinicalsigns+ symptomsmay be delayedfor
24 hours (increasingmortalityto 65%)
Blunt Traumato Pancreas(3%)
Mechanism: compressionagainstvertebralcolumn
with shear acrosspancreaticneck
lncidence: <10/" of childhoodtrauma
Cause: motorvehicleaccident,fall onto handlebars
of a bicycle,childabuse
Associatedwith: injuryto liver,duodenum
Classification:
I minorcontusion/ hematoma,capsule+ major
duct intact
ll parenchymal injurywithoutmajorduct injury
lll majorductalinjury
lV severecrush injury
Location: junctionof body + tail
{ posttraumaticpancreatitis:
{ edema/ fluid in peripancreatic fat
{ focal/ diffusepancreaticenlargement
r/ irregularityof pancreaticcontour
r/ area of low-attenuation = contusion/ laceration
(actualsite of lacerationdifficultto visualize)
r/ ttuiOaroundsuperiormesentericartery
r/ ttuiOin transversemesocolon/ lessersac
r/ ttuiObetweenpancreasand splenicvein
r/ thickeningof anteriorpararenalfascia
N.B.: 24-48-hourdelayedscansuncoverfindingsnot
presentearlier
Rx: | + ll conservativemanagement;
lll + lV needsurgerywithin24 hours
Cx: recurrentpancreatitis,pseudocyst,
pseudoaneurysm, fistula,abscess(attendant
mortalityof 20%)
BOERHAAVE
SYNDROME
= completetransmuraldisruptionof esophagealwall with
extrusionof gastriccontentinto mediastinum/ pleural
spacesecondaryto food bolus impaction
. forcefulvomitingwith suddenonset of pain (substernal,
leftchest,in neck,pleuritic,abdominal)
. dyspnea
. NO hematemesis(bloodescapesoutsideesophageal
lumen)
{ rent of 2-5 cm in length,2-3 cm above GE junction,
predominantlyon left posterolateralwall
{ pleuraleffusionon left >> rightside/ hydropneumothorax
{ pneumomediastinum (singlemost importantplain-film
finding),pneumopericardium, subcutaneous air
r/ "V signof Naclerio"= localizedmediastinalemphysema
with air betweenlowerthoracicaorta + diaphragm
r/ mediastinal widening
{ air-fluidlevelwithinmediastinum
{ extravasationof contrastmediuminto mediastinum/
pleura
GLANDHYPERPLASIA
BRUNNER
= BRUNNER GLANDHAMARTOMA
lncidence: 1.2h of all gastricpolyps
Etiology: responseto increasedacid secretion
Histo: diffuselyenlargedhyperplasticglandswith Swiss
cheeseappearance
Physiology; secretea clearviscousalkalinemucus into
cryptsof Lieberktlhn
MOR P H OLOGIC TY P E S :
1. Diffusenodularhyperplasia
2. Circumscribed nodularhyperplasia:
in suprapapillary portion
3. Singleadenomatoushyperplastic polyp:
i n duodenalbul b
Location: duodenum,prepyloricregion(duodenalglands
beginin vicinityof pylorusextendingdistally
withinproximal213of duodenum)
{ multiplenodularfillingdefects(usuallylimitedto
1st portionof duodenum)with "cobblestone
appearance" (mostcommonfinding)
DDx: polyposissyndromes,lymphoidhyperplasia,
gastricmucosa,nodularduodenitis
heterotopic
{ occasionally singlemass up to 5 cm + centralulceration
DDx: adenomatouspolyp,varioussubmucosaltumors
Cx: Gl bleeding,obstruction, intussusception
BURKITT LYMPHOMA
= rTrostcommontype of pediatricnon-Hodgkinlymphoma;
initially
describedin a7-yearoldUgandan childin 1958by
DennisBurkitt,a Britishsurgeon
Etiology: tumor from undifferentiated small noncleaved
B-cell-derivedlymphocytes
Path: resemblanceto Hodgkindisease
Histo: characteristic"starrysky" pattern
. fastestgrowingtumorin humanswith a potential
doublingtime of 24 hours
. paraplegia
. NO peripheralleukemia
r/ conspicuousabsenceof lymph node disease
r/ tendencyto permeate/ destroybone
MR:
r/ isointenseto muscleon Tl Wl + T2Wl
r/ intensehomogeneous enhancement
 Gastrointestinal
Disorders 801

A . E NDE M I CF OR MO F B U R KIT TL YMP H OMA Path: firm yellowsubmucosalnodulearisingfrom

Endemic in areas with malaria: argentophilKulchitsky cellsin the cryptsof
sub-Saharan Africa,New Guinea(exposureto Lieberktlhn(= ?rgontaffinoma due to affinityfor
Plasmodiumfalciparumhas a synergisticeffect silverstain);invasionintomesenteryincitesan
causinga markeddecreasein T-cellsurveillance) intensefibroticreaction
lncidence in central Africa: Histo: low-grademalignancy= fesembleadeno-
50-80% of all childhoodneoplasms carcinomasbut do not have their aggressive
Associatedwith: Epstein-Barrvirus infectionin 95% behavior;malignantthroughinvasionof muscularis
(implicatedas a B-cellmitogenin Biochemistry:
the oncogenesis) tumorelaborates(1) ACTH,(2) histamine,(3) bradykinin
Age: 3-10 years (4) kallikrein,(5) serotonin= S-hydroxytryptamine (from
@ Mandible(first)/ maxillaltacialbones tryptophanover S-hydroxytryptophan), which is
. jaw mass metabolized in liverby monamineoxidaseinto
. exophthalmos(orbitalextension) S-hydroxyindole aceticacid (5-HIAA)and excretedin
r/ grosslydestructivelesion,spiculesof bone urine;5-hydroxytryptophan is destroyedin pulmonary
, growingat rightangles circulation
{ largesoft{issue mass Associatedwith: other synchronousi metachronous
@ Other skeleton(multifocalin 10%) malignancies(36% at necropsy)
{ reminiscent of Ewingtumor/ reticulumcell
sarcoma . asymptomatic(66%)
r/ lamellatedperiostealreactionaroundmajorlong . abdominalpain/ obstruction(19%)
bones . nausea,weightloss (16%)
B . NO NE NDE MIC / SP O R AD ICF OR MOF B U R K ITT . palpablemass (14%)
LYMPHOMA . Gl bleeding
lncidence in Europe + NorthAmerica: . Garcinoid syndrome (in 7"/"of small bowelcarcinoids):
3545% of all pediatricNHL;3/" of all childhood = cor'rstellation of clinicalfindingsrelatedto hormone
tumors secretionby tumor
Age: 6-15 years Cause: excessserotoninlevelswhen the metabolic
. Epstein-Barr virusgenomefoundin only a minority pathwayto S-HIAA(in liver)is bypassed
@ Abdomen(69%) (a) with liver metastases
tumorsof small bowel (Peyerpatchesof terminal (b) with primarypulmonaryI ovariancarcinoids
ileum),mesentery . recurrentdiarrhea(70%)
. abdominalmass . right-sidedendocardialfibroelastosis(35%) resulting
. intestinalobstruction in tricuspidregurgitation+ pulmonaryvalve stenosis
{ usuallyintraabdominal extranodalinvolvement + right heartfailure
with sparingof spleen . attacksprecipitatedby ingestionof food / alcohol
,
r/ well-defined sharplymarginatedhomogeneous . asthmaticwheezingfrom bronchospasm (15%)
tumors(75/") . desquamative skin lesions(5%)
{ ascites(13%) . peflagra (7oh)trom niacindeficiencyas a resultof
@ Genitourinarytract (20%) preferentialconversionof dietarytryptophanto
ovary,uterus,kidneys,retroperitoneum serotoninratherthan niacin
r/ renal masses/ diffuseenlargement(5%) . multipletelangiectasias(25%)
{ hydronephrosis(28%) . nausea& vomiting,fever
@ Chest . hypotension(vasomotorinstability)
r/ pleuraleffusion(mostcommonchestabnormality) . cutaneousflushing(rare)
@ CNS . excessS-HIAAin urine
r/ meningealinfiltration (mostcommonly) Prognosis:carcinoidsyndromehas a highermorbidity
! cavernoussinusinvasion & mortalitythan doesthe tumoritself!
{ supra-and parasellartumor
! epiduralspinalmass+ spinalcord compression RULEOF 1/3: 0 1/3 occurin smallbowel
@ Others 0 1/3 have metastases
salivaryglands,thyroid,bone marrow 0 1/3 are multiple
Rx: dramatic responseto chemotherapy 0 1/3 havea secondmalignancy
Prognosis: long-termsurvivalin 50% Metastases:
to lymphnodes,liver(in 90% of patientswith carcinoid
C A RCI NO I D syndrome),lung,bone (osteoblastic)
= rT'rostcommonprimarytumor of small bowel + appendix (a) incidenceversustumorsize
(>95%of all carcinoids);
belongsto APUDomas(like tumorof <1 cm (in75%) metastasizes in 2%
pheochromocytoma, medullarycarcinomaof thyroid, tumor of 1-2 cm (in 20%) metastasizesin 50%
isletcelltumorsof pancreas);M:F = 2:1 tumor of >2 cm (in 5%) metastasizesin 85%
802 Radiology Review Manual

(b) incidenceversuslocation r/ hypoattenuating livermetastases:

tumor in ileum (in 28%) metastasizesin 35% ^/ may appearhyperdensein arterialphase
tumor in appendix (in 46%\ metastasizesin 3% { may becomeisodenseafter slow contrastinfusion
tumorin rectum (in 17%) metastasizes in 1o/o A ngi o:
Liver metastasesseen: best / (only) on. { "sunburst"appearance:
(a) NECT 35% (3%) ^/ Xinkingof small-and medium-sized vesselswith a
( b) CE CTin H A P ..3 5 % (1 4 % ) stellateconfiguration
(c) CECT in PVP ..30% (3%) { simulatedhypervascularity withthickening
HAP = hepaticarterial-dominant
phase of triplephase CT + foreshortening of mesentericvesselssecondary
PVP = portalvenous-dominantphase of triplephase CT
to fibroticretraction
Location: betweengastriccardiaand anus { mesentericischemia:
@ Appendix(30-45%) { arterialbranchstenosesfrom encasementof
Commonlybenign: slowgrowth,rarely medium-sized vessels(dueto elasticvascular
metastasizing sclerosiswith locallyelevatedserotoninlevels)
lncidenceat surgery: 0.03-0.7% ./ venousocclusion/ mesentericvarices
Site: tip (70%),middle(20/"), base (10%)of ^/ tumor may be identifiedas hypervascularmass
appendix N U C ( l - 1 2 3M I B Gi m a g i n g ) :
. symptomsof appendicitis r/ uptakein 44-63% (higherf requencyof radiotracer
r/ invasionof mesoappendix (11%) uptakein midgutcarcinoids+ with elevatedserotonin
@ Small bowel (25-35%) levels)
lncidence: 25/" of all small boweltumors US:
Loc at ion: i l e u m(9 1 % );j e j u n u m(7 " /" ),duodenum r/ persistentfluid-distended appendixwithouttypical
(2%);multiplein 15-35% signsof appendicitis
0 75% of patientswith symptomaticspreadhave Prognosis; slow progressionwith averagesurvivaltime of
midgutcarcinoids 3.2 years afterdiagnosisof livermetastases
0 Duodenalcarcinoidsare associatedwith multiple Rx: Somatostatin / SMS 201-995:chemoembolization
endocrineneoplasia of hepaticarteries
@ Rectum(10-15%): metastasize in 10% DDx: oat-cellcarcinoma,pancreaticcarcinoma,
@ Colon(5%): ascendingcolon,oftenmalignant medullarythyroidcarcinoma,retractilemesenteritis,
@ Stomach(<3%):lessercurvatureof distalantrum desmoplastic carcinomaI lymphoma
./ one / more 1-4-cm submucosalmasses:
, r/ bull'seye appearancewhen ulcerated CATHARTIC
COLON
r/ one / moresessile/ pedunculated polyps = prolongeduse of stimulant-irritantcathartics
@ Otherorgans(5%): bronchus,thyroid,pancreas, ( t15 years)resultingin neuromuscular incoordination
biliarytract,teratomas(ovarian,sacrococcygeal, from chronicallyincreasedmuscularactivity+ tonus
testicular) Agents: castoroil, senna,phenolphthalein,cascara,
@ may be multicentric podophyl l um,al oi n
Location: involvement of colonproximalto splenic
UGI: flexure
{ smallsmoothsubmucosalmass (usually<2 cm) r/ effacedmucosawith flattenedsmoothsurface
impingingeccentrically on lumen r/ diminished/ absenthaustrations
r/ desmoplasticresponseof mesenteryfrom locallyhigh r/ "pseudostrictures"= sffioothlytaperedareas of
levelsof serotonincauses: narrowingare typical(sustainedtonusof circular
r/ angulation+ kinkingof loopsleadingto obstruction muscles)
( DT A G N OS T tC ) r/ poor evacuationof barium
{ spiculated/ tetheredappearanceof mucosalfolds r/ flanened+ gaping ileocecalvalve
! mattingof multipleloops r/ shortenedbut distensible ascendingcolon
r/ separationof loopsdue to large mesenteric DDx: "burned-out"ulcerativecolitiswith right-sided
metastases predominance (verysimilar)
CT:
focal calcifiedmesentericmass surroundedby CHAGAS DISEASE
thickenedmesentery = damageof ganglioncellsby neurotoxinliberatedfrom
rl stellateradiatingpattern+ beadingof mesenteric protozoaTrypanosoma cruziresultingin aperistalsis
of
neurovascular bundles(desmoplastic reaction) Gl tract+ dilatation
r/ retraction+ shorteningof mesenteryr/displacement Endemicto Central+ SouthAmerica(esp.easternBrazil)
+ kinking+ separationof adjacentbowel loops Histo: decreasednumberof cellsin medullarydorsal
{ segmentalthickeningof adjacentbowel loops motornucleus+ Walleriandegeneration of vagus
(encasement of mesentericvesselsleadsto chronic cellsin myenteric
+ decrease/ lossof argyrophilic
ischemia) plexusof Auerbach
r/ low-densitylymphadenopathy (due to necrosis) Peakage: 30-50 years; M:F = 1:1

.intermittent/ persistentdysphagia
'odynophagia(= fear of swallowing)
.foul breath,regurgitation,aspiration
'Mecholyltest: abnormalresponseindicativeof deficient
innervation; 2.5-10 mg methacholine subcutaneously
followedby severetetanicnonperistalticcontraction
2-5 minutesafterinjection,commonlyin distalhalfof
esophagus,accompaniedby severepain
@ Dilatativecardiomyopathy(myocarditis)
@ Megacolon(bowelsmove at intervalsof g days to
5 months)
Cx: impactedfeces,sigmoidvolvulus
@ Esophagus:changesas in achalasia
CHALASIA
= continuouslyrelaxedsphincterwith free refluxin the
absenceof a slidinghernia
Etiology: elevatedsubmergedsegment
Causes: (1) Delayeddevelopmentof esophagogastric
regionin newborns
(2) Scleroderma, Raynauddisease
(3) S/P forcefuldilatation/ myotomyfor
achalasia
./ free / easilyinducedreflux
CHRONIC IDIOPATHIC INTESTINAL
PSEUDOOBSTRUCTION
= nonpropulsive
intestine
characterized
byimpaired
responseto intestinaldilatationwithoutdefinablecause;
? autosomaldominant
Age: all ages,M:F = 1 :1
' recurrentattacksof abdominaldistension,periumbilical
pain,nausea,vomiting,constipation
{ mildto markedgaseousdistensionof duodenum
+ proximalsmallbowel
i/ esophagealdilation+ hypoperistalsis (lowerthird)
ri excessiveduodenaldilation(DDx:megaduodenum,
, superiormesentericarterysyndrome)
r/ ligamentof Treitzmay be placedlowerthan usual
! delayedtransitof bariumthroughaffectedsegments
tr disorderedmotoractivity(fluoroscopy)
COLITISCYSTICAPROFUNDA
= r?re benignconditioncharacterizedby submucosal
mucus-containing cysts linedby normalcolonic
epit helium
Etiology: probablyrelatedto chronicinflammation
Associatedwith: solitaryrectalulcersyndrome(in
localizedform)
Age: primarilydiseaseof young adults
. brief periodsof brightred rectalbleeding
. mucous/ bloodydischarge
. intermittentdiarrhea
Location:(a) localizedto rectum(mostcommonly)i
sigmoid
(b) generalizedcolonicprocess(lesscommon)
! nodularpolypoid/ cauliflower-like lesions<2 cm in size.
containingno gas
Gastrointestinal
Disorders 809
V spiculations
mimickingulcers(barium-filled
clefts
betweennodules)
DDx: pneumatosis(rarelyaffectsrectum)
COLONIC ATRESIA
lncidence: less commonthan ilealatresia
Plainradiograph:
r/ massivedilatationof colon proximalto obstruction
r/ mottledpatternof gas + feces proximalto pointof
atresia
DDx: often indistinguishable from obstructionof distal
i l eum
BE:
r/ functionalmicrocolon
r/ obstructionto retrogradeflow of barium
US:
r/ dilatedhyperechoicdistalsmall bowel + proximal
colon(fromretainedmeconium)
COLORECTAL
CARCINOMA
Mostcommoncancerof Gl tract;3rd mostcommonly
diagnosedmalignancyin developedcountriesin men
(afterlung + prostatecancer)and women (afterlung
+ breastcancer);2nd leadingcauseof cancerdeaths
lncidence: 11"hof all newlydiagnosedcancers;13% of
all cancerdeaths;150,000new cases/year
with 57,000deathsin UnitedStates(1ggg);
6% lifetimeprobabilityof any White personto
developcolorectalcancer; 3:100,000in
30-34-year-olds;532:100,000for
>85-year-olds
Lifetime probability: 4o/"
Risk factors:
1. Personalhistoryof colonicadenoma/ carcinoma
- malignancyin 5/" of tubularadenomas
- malignancyin 30-40% of villousadenomas
Proofof adenoma-carcinoma sequence:
(a) frequentcoexistence
of adenoma+ carcinoma
(b) similardistribution
withincolon
(c) consistentproportionalprevalencein
populationhavingvariedmagnitudesof colon
cancerrisk
(d) increasedfrequencyof carcinomain patients
with adenomas
(e) reductionof cancerincidencefollowing
endoscopicremovalof polyps
(f) all patientswith familialadenomatous
polyposissyndromedevelopcoloncarcinomaif
colonnot removed
(g) similarityof DNA + chromosomal constitution
0 93% of colorectalcarcinomasarisefrom
adenomatouspolyp!
0 A patientwith one adenomahas agh chanceof
havinga colorectalcarcinomain next 15 yearsl
0 lt takesabout7 yearsfor a 1-cmadenomato
becomean invasivecancerl
0 5% of adenomas5 mm in sizedevelopinto
invasivecancers(5 mm is consideredcriticalmass
of intraepithelialneoplasia)I
804 Radiology Review Manual

2. Familyhistoryof benign/ malignantcolorectaltumors Staging (UICC-AJCC Colorectal Cancer Staging System):

in first-degreerelatives(3-5 x risk) Stage Grouping S-year Survival
3. Personalhistoryof ovarian/ endometrial/ breast
cancer 0 Tis N0 M0 >95%
4. Dysplasiaof colonwithinflat mucosa I T1 N0 M0 75-100%
5. Inflammatory boweldisease: T2 NO MO
(a) Ulcerative colitis(3-5% incidence;cumulative ll T3 N0 M0 50-75%
incidenceof 26/" after 25 years of colitic T4 NO MO
symptoms) lll any T N1 M0 30-50%
(b) Crohn diseaseaffectingthe colon + rectum any T N 2,3 M0
(particularlyin bypassedloops/ in vicinityof lV any T any N M1 <10o/o
chronicfistula) Legend:
Tis carcinomainsitu
Time delay: >8-10 yearsof colitis
T1 invasion
of submucosa
Underlyinglesion: dysplasiawithinflat mucosa f2 of muscularis
invasion propria
6. Prominentlymphoidfollicularpattern T3 invasion
of subserosa / pericolic
tissue
7. Pelvicirradiation f4 of visceralperitoneum
invasion / otherorgans
8. Ureterosigmoidostomy N1 1-3 pericolic
Lnn
Environmental risk factors: N2 >4 pericolic
Lnn
(a) low fiberdiet: preventsrapidtransittime thus N3 anyLnnalongcourseof a vascular trunk
increasingcontacttime betweenpotentialtoxins
and colonicmucosa Metastases(lymphatic/ hematogenous venous):
(b) increasedingestionof fat + animalprotein 1. Liver(75'h;15-20/" at time of surgery)due to portal
(c) obesity venousdrainageroute
(d) asbestosworker ' 2. Mesentery+ mesentericnodes(10-15%)
Genetic risk factors (6% of colorectal carcinomas): 3. Adrenal(10-14%)
(a) familialadenomatous polyposissyndrome: 4. Lung (5-50%)
familialpolyposis,Gardnersyndrome,Turcot 5. Ovary (3-8%) = Krukenbergtumor
syndrome 6. Psoasmuscletumordeposit
Age: approximatelyat 40 years 7. Peritonealmetastases:
(b) certainhamartomatouspolyposissyndromes: (a) malignantascites: usuallyassociatedwith poorly
Peutz-Jeghers syndrome,juvenilepolyposis, coloniccarcinoma
differentiated
Cowdendisease (b) pseudomyxoma peritonei(<5%): low-grade
(c) hereditarynonpolyposis coloncancersyndrome colonicadenocarci noma
= Lynchsyndrome(see below) 8. Bone(5%)
Screening recommendations (American Cancer Society): 9. Brain(5%)
as / more effectivethan mammographicscreening 0 Becauseof absenceof lymphaticsin laminapropria
(a) for persons>50 yearsof age: annualfecaloccult- coloncancerwill not metastasizeuntil it penetrates
bloodtest + sigmoidoscopy/ BE every 3-5 years the muscularismucosa!
(b) for first-degreerelativesof patientswith coloncancer . rectalbleeding,irondeficiencyanernia
screeningshouldstart at age 40 . changein bowel habits/ caliberof stools
Age: medianage of 71 yearsfor colon cancer;median . obstruction(poorprognosticindicator)
age of 69 years for rectalcancer; M:F = 3:2 . hydronephrosis (13%)
Histo: (1) Adenocarcinoma with varieddegreesof . positivefecal occultbloodtesting(2-6% positive-result
differentiation rate ; 5-10/" positivepredictivevalue;failsto detect
(2) Mucinouscarcinoma(uncommon) 30-50% of colorectalcarcinomas+ up to 75/" ot
(3) Squamouscellcarcinoma+ adenoacanthoma adenomas):Hemoccult(hematein), Hemoquant
(rare) (porphyrins), Haemselect(hemoglobin)
. progressive elevationof carcinoembryonic antigen
Staging (modified Dukes= Astler-Coller classification):
(CEA)>10 pg/L indicativeof recurrent/ metastatic
Stage Findings disease
A limitedto mucosa . waterydiarrhea+ potassiumdepletion/ excessive
secretionof mucus+ hypoalbuminemia (in largemucin-
B involvementof muscularispropria
secretingvilloustumor)
B1 extensioninto muscularispropria
B2 extensionthroughmuscularispropriainto
Location: "aginggut" = numberof right-sided lesions
serosa/ mesentericfat (35%)
increasingwith age ("changing
distribution")
c lymph node metastases(50%) (a) left colon (52-61%):
c1 + growthlimitedto bowelwall rectum(15-33 -41"/"),sigmoid(20-37"/"),
c2 + growthextendinginto adiposetissue descendi ng col on(10-11% )
distantmetastases ^/ commonlyannularstrictureswith obstruction
 Gastrointestinal
Disorders 805

(b) rightcolon: Recurrencein 1/3 of patients:

transversecolon(12/"), ascendingcolon(g-16%), (a) localrecurrenceat lineof anastomosis(60%)
cecum(8-10%) within 1 year after resectionin 50/o,within2 years
r/ commonlypolypoidlesionswith chronicbleeding after resectionin 70-80%
+ intussusception (b) distantmetastases(26%)
(c) local recurrence+ metastases(14%)
Colonoscopy:cecumnot visualizedin 10-36%;failsro Risk afterdetectionof colon cancer:
detect 12/" of colonicpolyps(10% in areas never reached 5/" Ior synchronous coloncancer
by colonoscope) 14h for synchronous cancerwith "sentinelpolyp"
Cx: perforation in 0.2/" (0.02%for BE);deathin 35/" for additionaladenomatouspolyp
1 : 5 , 0 0 0( 1 : 5 0 , 0 0f0o r B E ) 3'/o for metachronouscolon cancer
4o/" tor extracolonicmalignancy
BE (sensitivitiesin detectionof polyps >1 cm:SCBE Cx: (1) Obstruction(frequently in descending+ sigmoid
77-940/",DCBE 82-97%; for polyps<1 cm: SCBE colon)
18-72/", DCBE61-93%): (2) Perforation
r/ fungatingpolypoidcarcinoma: (3) Intussusception
. chronicbleeding,intussusception (4) Abscessformation
r/ annularulceratingcarcinoma: "dpplecore lesion', (5) Fistulaformation
= annularconstriction is a resultof tumorgrowing (6) Pneumatosis cystoidesintestinalis
alongthe lymphaticchannels,whichparallelthe (7) Pseudomyxoma peritonei(fromlow-grade
circularmusclefibersof the innerlayerof the adenocarcinoma of colon)
muscularispropria;longitudinalgrowthis limited Rx: (1) Localsurgicalexcision/ polypectomytor stage I
with abrupttransitionto normalmucosa disease
. colonicobstruction (2) Righti left hemicolectomy with eventual
{ "saddlelesion"= growthcharacteristics between anastomosis of proximal+ distalexcisionsites
polypoidmass+ annularconstricting lesion (3) Low anteriorresection:>2 cm of rectummust
r/ scirrhouscarcinoma(signet-ring type) remainto anastomosethe colon
= long-segment stricturewithoutsignificantmucosal (4) Abdominoperineal resectionwith colostomyfor
abnormality similarto linitisplasticadue to diffuse low rectalcarcinoma
circumferential+ longitudinal tumorinfiltrationwithin (5) Adjuvantchemotherapyfor stage ll disease
the loosesubmucosaltissuebetweenmuscularis (fluorouracil/ levamisole)
mucosa+ muscularispropria DDx: (1) Prolapsingileocecalvalve(changeon palpation)
. oftenseen in ulcerativecolitis (2) Spasm (intactmucosa,releasedby
{ curvilinear/ mottledcalcifications(rare)are propantheline bromide)
CHARACTERISTIC of mucinousadenocarcinoma (3) Diverticulitis

CT (48-90% stagingaccuracy,25-79% for lymphnode

metastases): LynchSyndrome
CT staging(pooraccuracycomparedwith Astler-Coller = HEREDITARY NONPOLYPOSIS COLORECTAL CANCER
classification): SYNDROME
Stage 1 intramuralpolypoidmass = familieswith high incidenceof colorectalcancers
Stage2 thickeningof bowelwall + increasedincidenceof synchronous and
Stage3 slightinvasionof surrounding tissues metachronouscolorectalcancers
Stage4 massiveinvasionof surrounding tissue Amsterdam criteria:
+ adjacentorgansi distantmetastases (a) >3 familymembersof whom 2 are 1st degree
r/ low-densitymass + low-densitylymph nodes in relativesof the third
mucinousadenocarcinoma (= >50% of tumor (b) familymembersin >2 generations
composedof extracellular mucin) (c) one familymemberdiagnosed<50 yearsof age
! psammomatous calcificationsin mucinous A. Lynch I = flo associatedextracoloniccancer
adenocarcinoma B. Lynchll = ossociated with extracolonic
malignancy:
r/ signsof Lnn involvement:singlelymphnode>1 cm in transitionalcell carcinomaof ureter
diameter/ clusterof )3 nodes<1 cm / nodeof any + renalpelvis;adenocarcinoma of
sizewithinmesentery
MR (stagingaccuracyof 73o/o,40%
node metastases)
sensitivity
for lymph ;:ffi:l ffi;,'J?[3i
?,:il
ll]#],31
;n,.
malignancy;carcinomaof skin + larynx
Etiology: autosomaldominantabnormalityof
Prognosis: chromosome2 with defectin DNA
Survivalrate of 40-50/" overallin 5 years (unchanged replication-repairprocess
over past 40 years);80-90% with Duke A; 70% with (a) acceleratedadenoma-carci noma sequence
Duk eB ; 33% w i th D u k eC :S./" w i thD u k eD (b) dysplasiain flat mucosaof colon
806 Radiology Review Manual

Prevalence: 5-10% of patientswith coloncancer; r/ hypoechoicperirectallymph nodes (= tumor

5 x morecommonthan familial involvement)
adenomatousPolYPosis sYndrome
Mean age: 45 years VOLVULUS
COLONIC
Location: 70/" proximalto splenicflexure = ITloSt
commonform of volvulus
Prognosis; better stage for stage than in other Incidence: 10"/"of large-bowelobstruction
cancers(S-yearsurvivalrate of 65%
versus44hin sporadiccases) CecalVolvulus
Surveillance: colonoscopyevery 1-2 years from ages = VOLVULUS OFCECUM
22-35 years lncidence: 40h of colonicvolvulus
Cause: suddendistensionby trauma,pressure,
constipation,distalcolonicobstruction
RectalCancer Associatedwith: malrotation+ long mesentery
lncidence: 45,000 rectalcancers/yearin UnitedStates resultingin poor fixationof right
Pathologic staging of rectal cancer: colon (10-25% of PoPulation)
Pathophysiologyof vascular compromise:
Astler-Coller/TNM Description S-year Suruival (1) Acutemesenterictorsion+ strangulation causes
A T1,N0,M0 limitedto submucosa 80% arterial+ venousobstruction
B1 T2,N0,M0 propria
limitedto muscularis 70% (2) Gradualdistension+ increasein intraluminal
82 T3,N0,M0 transmuralextension 60-65% pressureinterfereswith blood supply;perforation
C1 T2,N1,M0 nodes(+),into muscularis 3545% in 65%
C2 T3,N1,M0 nodes(+),transmural 25% Age peak: 2040 Years; M > F
f4 invasionof adjacentorgans ^/ cecalgaseousdistension:
D M1 distantmetastasis <25/"
./ cecum rotatesanteriorto ascendingcolon = ceGdl
bascule [French= seesaw](type I cecalvolvulus)
Hematogenous metastasis : r/ "kidney-shaped" distendedcecum rotatesinto left
dualvenousdrainageintoportal+ systemicveins upper quadrant(type ll cecalvolvulus)
^/ may have lung withoutlivermetastases r/ taperedend of bariumcolumnpointstowardtorsion
Riskof recurrence: Cx: (1) cecaldistension>10-12 cm meansriskof
5% for T1 bowel Perforation/ infarction
1O / o lor T 2 3 3 % fo r T 1,N 1 + T 2 N 1 (2) Abdominal compartment syndrome
25% for T3 66% for T3N1 = increasein abdominalpressurediminishes
50% for T4 respiratoryfunction+ cardiacoutput
Staging accuracy:
(1) Digitalrectalexamination:68-75-83/"; limitedto SigmoidVolvulus
lesionswithin10 cm of analverge = VOLVULUS OFSIGMOID
(2) CT: 48-72-927o,betterfor more extensive Cause: sigmoidtwistson mesentericaxis
regionalspread;25-73% for lymph node Age: usuallyin elderly/ psychiatricallydisturbed
involvement Degreeof torsion:360" (50%),180" (35%),540' (10%)
(3) MR: 7+84-93% with tendencyfor overstaging r/ greatlydistendedparalyzedloop with fluid-fluidlevels,
(4) Transrectalultrasound:6+77-94% with mainlyon leftside,extendingtowarddiaphragm
tendencyfor overstaging; limitedto lesions<14 cm (erectfilm)
from anal verge + nonstenoticlesions;50-83% { "coffee-beansign" = distinctmidlinecrease
sensitivityfor lymph node involvement correspondingto mesentericroot in largelygas-
TransrectalUS (817oaccuracy): distendedloop (suPine)
Normal layers: ./ "bird-of-preysign" = taperedhooklikeend of barium
(a) hyperechoicinterfaceof balloon+ mucosa col umn
(b) hypoechoicmucosa+ muscularismucosa C T:
(c) hyperechoicsubmucosa { "whirlsign" = tightlytorsionedmesenteryformedby
(d) hypoechoicmuscularispropria twistedafferent+ efferentlooP
(e) hyperechoicserosa
{ hypoechoicmassdisruptingrectalwall:
{ no interruption of hyperechoic submucosa CONGENITAL ESOPHAGESOPHAGEAL ATRESIA
= tumorconfinedto mucosa+ submucosa & TRACHEOESOPHAGEAL FISTULA
{ no interruptionof hyperechoicserosa= tumor = complexof congenitalanomaliescharacterizedby failed
confinedto rectalwall / incompleteformationof the tubularesophagusor an
r/ break in outermosthyperechoiclayer = tumor abnormalcommunicationbetweenesophagus+ trachea
penetratesinto perirectalfat Cause: developmentaldisorderin formationand
r/ irregularserratedouter borderof muscularis separationof primitiveforegutinto trachea
propria(pseudopodia throughserosa) + esoPhagus / vascularcomPromise
 Gastrointestinal
Disorders 807

Embryology: ' droolingfrom excessiveaccumulationof pharyngeal

primitiveforeguttube developslateralwall foldsthat secretions(esophagealatresia= EA)
may incompletely connectat any pointleavinga . obligatoryregurgitationof ingestedfluids(EA)
fistulouscommunication;occursat 3rd-Sthweek of . aspirationwith coughing+ chokingduringfeeding(TEF)
intrauterine life . recurrentpneumonia+ progressiverespiratorydistress
lncidence: 1:2,000-4,000livebirths;most common of variableseverity(tracheoesophageal fistula= TEF) in
sporadiccongenitalanomalydiagnosedin neonate
childhood
Risk of recurrence in sibling: i% Location: betweenupper1/3 + lower1/3 of esophagus
Associatedanomalies (17-56-70%): just above carina
1. Gastrointestinal (20-25%): imperforateanus, pyloric
stenosis,duodenalatresia,annularpancreas @ Mediastinum
2. Cardiac(15-39%): patentductusarteriosus, ASD, ! "coiledtube"= inabilityto passfeedingtube into
VSD, right-sidedaorticarch (5%) stomach(esophagealatresia)
3. Musculoskeletal Qa%): radialray hypoplasia, r/ retrotrachealair-distendedpouch of proximal
vertebralanomalies esophaguscausingcompression / displacement
of
4. Genitourinary (12%): unilateralrenalagenesis trachea
5. Chr om os o ma(3 l -1 9 % ):tri s o m y1 g ,2 1, 1 g r/ non-/ hypoperistaltic esophagealsegment
0 Trisomy18 is presentin 75-100%of fetuses+ in (6-15 cm) in midesophagus
3-4% of neonateswith esophagealatresial r/ food impaction
@ Abdomen
mnemonic; "ARTICLES" ^/ gaslessabdomen(esophagealatresia+ proximalTE
Anal atresia fistula=typesA+B)
Renalanomalies r/ abdomendistendedby bowelgas in 90% (distalTE
TE fistula fistula/ H-typefistula= types C + D)
Intestinalatresia/ malrotation @ Chest
Gardiacanomaly(PDA, VSD) r/ bronchopneumonia with patchyairspaceopacity,
Limb anomalies(radialray hypoplasia,
polydactyly) esp. in dependentupperlobes(in 50%)
Esophagealatresia
Spinalanomalies OB-US(anomaliesnot identifiedbefore24 weeksGA):
{ polyhydramnios in 33-60%:
mnemonic.' "VACTERL" 0 TE-fistulawith esophagealatresiais causeof
Vertebralanomalies polyhydramnios in onlyS%!
Anorectalanomaly r/ absenceof fluid-distended stomach(in 1O-41"/o;in
Cardiovascular anomalies remainingcasesTE-fistula/ gastricsecretionsallow
Tracheo- somegastricdistension)
Esophageal fistula r/ reducedintraluminal fluidin fetalgut
Renalanomalies r/ smallabdomen(birthweight<1Othpercentilein 4O%)
Limb anomalies r/ distendedproximalpouchof atreticesophagus

w
EsophagealAtresia Ejopltuggal4tresia+ EsophagealAtresia + Esophageal
Atresia + TE Fistula without
Proximal rE Fistula DistafTE Fistula proximafand DistalrE EsophagealAtresia
Fistula
L0Vo lVo 77Vo 27o l0Vo
EsophagealAtresia with Thacheo-esophagealFistula
808 Radiology Review Manual

Cx after repair: EALACHALASIA

CRICOPHARYNG
(1) Anastomoticleak = hypertrophy of cricopharyngeus muscle(= uPPer
(2) RecurrentTE fistula esophagealsphincter)with failureof completerelaxation
(3) Aspirationpneumoniasecondaryto Etiology:
(a) esophagealstricture 1. Normalvariantwithoutsymptoms:seen in 5-10/" of
(b) disorderedesophagealmotilitydistalto TE fistula adults
(c) gastroesophageal reflux 2. Compensatorymechanismto gastroesophageal
DDx: pharyngealpseudodiverticulum (traumatic reflux
perforationof posteriorpharynxfrom fingerinsertion 3. Neuromuscular dysfunctionof deglutition
intooropharynxduringdelivery/ tube insertion) (a) primaryneuraldisorders:
brainstemdisorder(bulbarpoliomyelitis,
EsophagealAtresiawithout Fistula= TypeA syringomyelia, multiplesclerosis,amyotrophic
Frequency: 8-10/. central/peripheralnervepalsy;
lateralsclerosis);
Associatedanomaliesin 17% (mostlyDownsyndrome cerebrovascular occlusivedisease;Huntington
+ other atresiasof Gl tract) chorea
(b) primarymuscledisorder:
EsophagealAtresiawith Fistula myotonicdystroPhY; PolymYositis;
Associatedanomaliesin 30% (mostlycardiovascular) dermatomyositis; sarcoidosis;myopathies
Type B = esophagealatresia+ proximalTE fistula secondaryto steroids/ thyroiddysfunction;
Frequency: 0.9-1"h oculopharyngeal mYoPathY
Type C = esophagealatresia+ distalTE fistula (c) myoneuraljunctiondisorder:
Frequency; 53-86% myastheniagravis; diphtheria;tetanus
. mostlyasymptomatic
Type D = esophagealatresia+ proximaland distalTE
. dysphagia
fistula
Frequency: 1-2.1oh 0 Cineradiography / videotaperecordingrequiredfor
demonstration!
Fistulawithout Atresia r/ distensionof proximalesophagus+ pharynx
Tracheoesophageal
= H-shapedfistula= Type E r/ smoothlyoutlinedshelf-i liplikeprojectionposteriorlyat
levelof cricoid(= pharyngoesophagealjunction) = level
Frequency: 6-10h
Associatedanomaliesin 23% (mostlycardiovascular) of C5/6
. feedingdifficultieswith choking ./ bariummay overflowinto larynx+ trachea
. diagnosismay not be made for severalyears Cx: Zenker diverticula
Rx: cricoPharyngeal mYotomY
r/ fistulacoursesfonrvardand upwardfrom esophagus

mnemonic:
type A = esophagealatresia - NO fistula = 10o/o
type B = esophagealatresia - PROX fistula = 1o/o
type C = esophagealatresia - DIST fistula = 80o/" COWDENDISEASE
= MULTIPLE HAMARTOMA SYNDROME
t y peD= es op h a g e aal tre s i a-PR O X + D IS T = 1o/o
- NO atresia = 1O/o = autosomaldominantdiseasewith highpenetrance
type E = Htype fistula
characterizedby multiplehamartomas+ neoplasmsof
endodermal,ectodermal,mesodermalorigin
CONGENITAL INTESTINAL ATRESIA lncidence: 160 cases rePorted
lncidence; 1:300livebirths Cause: susceptibility gene on long arm of chromosome
Cause: usuallysporadicvascularaccidents(primary/ 10(10q23)
secondaryto volvulusor gastroschisis) Age: 2nd decade
Location: jejunum+ ileum (70%),duodenum(25%), @ Mucocutaneoustumors
colon(5%);may involvemultiplesites . facialpapules
./ 'triplebubblesign"= intraluminal gas in stomach . oral papillomas(lips,gingiva,tongue)
+ duodenalbulb + proximaljejunum as pathognomonic . palmoplantarkeratosis,acral keratosis
sign for jejunalatresia @ CNS neoplasia
r/ bulbousbowelsegmentsign = dilatedloopof boweljust meni ngi oma;gl i oma
proximalto site of atresia(due to prolongedimpactionof Associatedwith: dysplasticcerebellargangliocytoma
intestinalcontents)with curvilineartermination . macrocePhaly
^/ gaslesslower abdomen(gut usuallyair{illed by 4 hours @ Breastlesions(in 50%):
after birth) r/ fibrocysticdisease+ fibroadenomas
r/ meconiumperitonitis(6%) ./ breast cancer (20-30h): often bilateral+ ductal
r/ polyhydramnios (in 50% with duodenallproximaljejunal @ Gl tract
atresia;rarelyin ileal/ colonicatresia) { multiplehamartomatouspotyps(in 30-60%'
Prognosis: 88o/"survivalfor isolatedatresia commonlyin rectosigmoid)

@ Thyroidabnormalities(in 60-70%):
r/ adenomas+ goiter
r/ follicularthyroidadenocarcinoma(g-4%)
@ Genitourinary lesions
@ Skeletalabnormalities
CROHNDISEASE
= REGIONAL ENTERITIS
= diseaseof unknownetiologywith prolonged
+ unpredictablecoursecharacterizedby discontinuous
+ asymmetricinvolvementof entireGl tract
Prevalence: 2-3:100,000white adults
Path: transmuralinflammation (noncaseatinggranuloma
with Langhansgiantcellsand epithelioid
celrs,
edema,fibrosis); obstructivelymphedema
+ enlargement of submucosallymphoidfollicles;
ulcerationof mucosaoverlyinglymphoidfollicles
Age: onsetbetween15 and 30 years; M:F = 1;1
. recurrentepisodesof diarrhea
. colicky/ steadyabdominalpain
. low-gradefever
. weightloss,anorexia
. occultblood+ anemia
. perianalabscessi fistula(40%)
. malabsorption(30%)
Associatedwith: erythemanodosum,pyoderma
gangrenosum
INTESTINAL MANIFESTATIONS
@ Esophagus(3%)
r/ "aphthous"ulcers(early)
r/ esophagitis,stricture,fistula(late)
@ Stomach(1-2%) = granufomatousgastritis
r/ aphthousulcers(= pinpointerosions)
r/ pseudo-postBillroth-lappearance
{ "ram'shornsign"= poorlydistensible smooth
tubularnarrowedantrum+ widenedpylorus
+ narrowduodenalbulb
r/ cobblestoneappearanceof mucosa
r/ antral-duodenai fistula
@ Duodenum(f10%)
almostalwaysassociatedwith gastricinvolvement
Location: duodenalbulb + proximalhalfof
duodenum
{ superficialerosions/ aphthoidulcers(earlylesion)
r/ thickenedduodenalfolds
@ Smallbowel(80%)= REGIONALENTER|T|S
terminalileum(alone/ in combinationin 9S%);
jejunum/ ileum(1S-5S%)
r/ thickening+ slightnodularityof circularfolds
r/ aphthousulcers
r/ cobblestonemucosa/ ulceration
./ commonlyassociatedwith medialcecal defect
@ Colon (22-55%) = GRANULOMATOUSCOLIT|S
Location: particularlyon rightside with rectum
^/tiny1-r-#.sJffi
llJll"l'lllL'.?3
firl"phoid
follicularpattern)
Gastroi ntestinal Disorders 809
r/ aphthousulcerswith "target/ bull's-eye"
appearance
r/ "transverse stripesign"= 1-cm-longstraight
stripesrepresentingcontrastmediumwithindeep
groovesof coarsemucosalfolds
{ long fistuloustractsparallelto bowel lumen
@ Appendicitis(20%)
@ Rectum(14-50%)
r/ deep / collarbuttonulcers
r/ rectalsinustracts
Phases:
(a) Earliestchanges
r/ nodularenlargement of lymphoidfollicles
r/ blunting/ flattening/ distortion/ straightening /
thickeningof valvulaeconniventes(obstructive
, lymphedema, usuallyfirstseen in terminalileum)
{ aphthousulcers= noduleswith shallowcentral
bariumcollectionup to 5 mm in diameter
Location:duodenalbulb,secondportionof
duodenum, termi nali l eum
(b) Advancednonstenoticphase
r/ skip lesions(90%)= discontinuous involvement
with interveningnormafareas
r/ cobblestoneappearance= serpiginous
longitudinal+ transverseulcersseparatedby
areasof edema
r/ tni* + bluntedsmall bowel folds (inflammatory
infiltration
of laminapropria+ submucosa)
r/ straightening+ rigidityof small bowel loopswith
luminalnarrowing(spasm+ submucosaledema)
r/ separation+ displacementof small bowel loops
(fromlymphedematous wall thickening/ increase
in mesentericfat / enlargedmesentericlymph
, nodes/ perforationwith abscessformation)
r/ pseudopolyps= islandsof hyperplasticmucosa
betweendenudedmucosa
^/ inflammatorypolypoidmasses
r/ sessile/ pedunculated/ filiformpostinflammatory
pofyps
./ diffusemucosalgranularity due to 0.5-1-mm
roundlucencies(= blunted+ fusedvilliseen en
face)
r/ pseudodiverticula = pseudosacculations = bulging
area of normalwall oppositeaffectedscarredwall
on antimesenteric side
(c) Stenoticphase
r/ "stringsign" = strictures(in 217o,most frequently
in terminalileum)/ markednarrowingof rigidloops
r/ normalproximalloops may be dilatedwith stasis
ulcers+ fecoliths
CT:
r/ homogeneous densityof thickenedbowelwall (DDx:
ulcerativecolitiswith heterogeneousattenuation):
r/ "doublehalo configuration"(50%)= intestinal
lumensurroundedby innerringof low attenuation
(= edematousmucosa)+ outer ring of soft-tissue
density(= thickenedfibroticmuscularis+ serosa)
(DDx:radiationenteritis,ischemia,mesenteric
venousthrombosis,acute pancreatitis)
810 RadiologyReview Manual

{ skip areas of asymmetricbowelwall thickeningof (5) Radiationileitis(appropriate

history)
11 (range10-20) mm in 82% (DDx:ulcerative (6) Lymphoma(no spasm,luminalnarrowingis
colitiswith a meanthicknessof 8 mm) uncommon,tumornodules)
^/ "creepingfat" = massiveproliferationof mesentericfat (7) Carcinoidtumor (tumornodules)
(40%)with mass effectseparatingsmall bowel loops (8) Eosinophilicgastroenteritis
^/ "Comb"sign = vasculardilatation+ tortuosity (9) Potassiumstricture
indicativeof activedisease
r/ luminalnarrowing+ proximatdilatation EXTRAINTESTINAL MANIFESTATIONS
r/ fistula/ sinustract (15-40%) @ Hepatobiliary
r/ mesentericadenopathy(18%) 1. Fattyinfiltration of liver(steroidtherapy,
^/ mesentericabscessin 15-20% (DDx:postoperative hyperalimentation)
blindloop) 2. Hepaticabscess
US: 3. Gallstones(15-3a%): predominantly cholesterol;
{ "pseudokidney" / targetsign = thickeningof bowelwall Risk: 3-5 x higherriskthan expected;risk
(22-65-89%) ot 5-20 mm (DDx: ulcerativecolitis) correlateswith lengthof diseasedileumi
ri circumferential diffuselyhypoechoicbowelwall with resectedileum/ durationof disease
lossof normallayering(dueto transmuraledema, Cause: interrupted enterohepaticcirculation
inflammation,f ibrosis) with malabsorption of bilesaltsin
r/ rigid+ noncompressible bowelsegmentwith termi nali l eum
reduction/ lossof peristalsis 4. Acute cholecystitis
^/ hyperemiaof gut wall + adjacentfat on color Doppler 5. Sclerosingcholangitis(10%)+ hepatoma
./ inflammatory mass= phlegmon(14/"), abscess(4%) 6. Bileduct + gallbladder carcinoma
./ distendedfluid-filledloops (12"/") @ GenitourinarY
! hypoechoicfistuloustract 1. Urolithiasis (5-10%): oxalate(steatorrhea leads
Prognosis.' recurrencerate of up to 39% after resection to excesscolonicabsorptionof oxalate)i urate
(commonlyat the siteof the new terminal stones
ileum,most frequentlyduringfirst 2 years 2. Hydronephrosis
after resection);mortalityrate of 7ohaI 3. Renalamyloidosis
5 years,12h at 10 yearsafter1st resection 4. Focalcystitis
Cx: (1) Fistula(33%): 5. lleoureteral / ileovesical fistula(5-20%)
(a) enterocolic: @ Musculoskeletal
most frequentlybetweenileum and cecum . digitalclubbing(11-40%)
(b) enterocutaneous (8-21%): . mildself-limiting seronegative peripheralmigratory
rectum-to-ski n ; rectumto-vagina arthritis(15-22%): may precedeboweldiseasein
(c) perinealfistula+ sinustracts 10%;severity+ coursecorrelateswell with
0 Crohndiseaseis 3rd mostcommoncauseof severityof intestinaldisease;resectionof
fistula/ sinustracts (DDx: iatrogenic[most diseasedbowel leadsto regressionof symptoms
commoncause],diverticula[2nd most 1. Hypertrophic osteoarthropathy
commoncausel)! 2. Ankylosingspondylitis(in 3-16%)
(2) Intramuralsinustracts 0 Axialskeletalinvolvement usuallyprecedes
(3) Abscess(DDx: acute appendicitis) onsetof Gl sYmPtoms!
(4) Free perforation(1-2%) . unrelatedin severityi courseto activitylevelof
(5) Toxic megacolon boweldisease
(6) Smallbowelobstruction(15%) r/ symmetricbilateralsacroiliitis
(7) Hydronephrosis (fromuretericcompression, r/ spondylitiswith syndesmophytes
generallyon rightside) 3. Peripheralerosivearthritis
(8) Adenocarcinoma in
in ileum/ colon(particularly r/ smallmarginalerosions
bypassedloops/ in vicinityof chronicfistula) r/ Periostitis
Q +20 x increasedriskof colonicadeno- ./ propensityfor osseousankylosis
carcinomacomparedwith generalpopulation 4. Avascularnecrosisof femoralhead (steroidRx)
with a latencyperiodof 25-30 years! 5. Pelvicosteomyelitis (contiguous involvement)
(9) Lymphomain large+ smallbowel 6. Septicarthritis
DDx: (1) Yersinia(in terminalileum,resolution within 7. Muscleabscess
3-4 months) 8. Retardedskeletalgrowth+ maturation
(2) Tuberculosis (moresevereinvolvement of @ Erythemanodosum,uveitis
cecum,PulmonarY TB)
(3) Actinomycosis, histoplasmosis, blastomycosis, CRONKHITE.CANADA SYN DROME
anisakiasis = hoflheoplasticnonhereditarypolyps(as in juvenile
(4) Segmentalinfarction(acuteonset,elderly polyposis)associatedwith ectodermalabnormalities;no
Patient) familialpredisposition

lncidence: >100 cases described
Histo: hamartomatouspolypsresemblingjuvenile/
retentionpolyps= multiplecysticspacesfilledwith
mucinsecondaryto degenerativechanges;
expansion+ inflammationof laminapropria
Age: 62 years (range 42-75 years); M < F
. exudativeprotein-losing enteropathy
. diarrhea(disaccharidase deficiency,bacterial
overgrowthin small intestine)
. severeweight loss,anorexia
. abdominalpain
. nail atrophy
. brownishmaculesof hand + feet
. alopecia
{ multiplepolyps
r/ thickenedgastricrugae
Location: stomach(100%);smallbowel(>50%);colon
( 10 0 % )
Prognosis; rapidlyfatal in women within6-18 months
(cachexia);tendencytoward remissionin
men
DESMOPLASTIC
SMALLROUNDCELLTUMOR
-- INTRAABDOMINAL DESMOPLASTIC SMALL ROUNDCELL
TUMOROF PERITONEUM
= highly malignant tumor belonging to a generic group of
smallroundblue celltumors(Ewingsarcoma,
neuroblastoma,
Wilmstumor, rhabdomyosarcoma,
primitiveneuroendocrine tumor)
lncidence; <50 cases in literature
Medianage: 21 years;affectschildren+ young adults;
up to 6th decade;M:F = 4:1
Origin: mesothelial/submesotheliallsubserosal
mesenchymeof the abdominalcavity
Histo: islandsof smallblue cells surroundedby fibrous
stroma;immunohistochemically positivefor
epithelial,neural,muscularmarkers(cytoplasmic
keratin+ desmin);abnormalchromosome11
. gastrointestinal / genitourinarydiscomfortI pain
. abdominaldistension
. palpableabdominalmass
Location: mesentery(spreadto omentum),
retroperitoneum, paratesticular,
posterior
mediastinum, pleura,meninges
{ multiplescatterednecrotictumor massesin abdomen
t pelviswith no definitevisceralorgan of origin:
r/ nodularperitonealthickening
r/ serosal hepatic metastases
, punctateperitonealcalcifications
! ./ OuOnty granularileocecalsoft-tissuemass in RLQ
{ enlargedtymphnodes
{ scant ascites
Cx: hydronephrosis,bowelobstruction
Prognosisi mean survivaltime of 17 months
DDx:
(a) in infants/ adolescents:rhabdomyosarcoma;
neuroblastoma; mesentericcarcinoid;Burkitt
lymphoma
GastrointestinalDisorders811
(b) in adults:diffuseomental/peritonealcarcinomatosis
(carcinomaof stomach,colon, ovary,pancreas);
melanoma;leiomyosarcoma; lymphoma;desmoid
tumor;mesothelioma; tumefactive tuberculosis;
actinomycosis;Castlemandisease
DIAPHRAGM DISEASE
= slndllbowelwebs due to NSAIDs
Effectof NSAID: gastricirritation,ulcerationof small
intestines
Frequency: in 10% of patientsreceivinglong-term
NSAIDtherapy
Path: foci of submucosalfibrosiswith interruptionof
adjacentmuscularismucosae
. blood + proteinloss
. intermittentintestinalobstruction
Location: ileum> jejunum
Enteroclysis:
{ multipleconcentricdiaphragm-likestrictures
DDx: Crohndisease
DISACCHARIDASE
DEFICIENCY
= eflZym€deficienciesfor any of the disaccharides
(maltose,lactose,etc.)
A . P R IMA R Y
B. SECONDARYto otherdiseases(eg,Crohndisease)
Pathophysiology:
(a) unabsorbeddisaccharidesproduceosmoticdiarrhea
(b) bacterialfermentationproducesshort-chainvolatile
fatty acidscausingfurtherosmotic+ irritantdiarrhea
,
I normalsmall bowelserieswithoutadded lactose
r/ abnormalsmall bowelseriesdone with lactose(50 g
addedto 600 cm3of bariumsuspension)
{ small+ targeboweldistension
r/ dilutionof barium
r/ shorteningof transittime
DISTAL INTESTINAL OBSTRUCTION SYNDROME
= MECONIUM ILEUSEQUIVALENT
= impactionof inspissatedstool in distalpart of ileum
+ proximalpart of colon
Prevalence: 7-1541% of children/ adolescentswith
cysticfibrosis;2/"in patients<5 yearsof age
Cause: tenaciousintestinalmucus,steatorrheadue to
pancreaticinsufficiency, undigestedfood residue,
disorderedintestinalmotilitywith increasein
intestinaltransittime, fecal stasis,dehydration
Age: 2nd-3rd decade of life
. recurrentboutsof colickyabdominalpain (fromfecal
impaction/ constipation)in RLQ
. palpablececal mass
{ partial/ completesmall bowelobstruction(due to
puttylikefecal materialin terminalileumi rightcolon)
r/ thickeningof mucosalfolds
{ cysticfibrosisof lung
CT:
Location:cecum > ascendingcolon > transversecolon
> descendingcolon (contiguousinvolvement)
diffusecolonicthickening
812 RadiologyReviewManual

r/ muralstriation(50%) ./ may be pointed,attenuated,irregularwith variable

r/ mesentericsoft-tissueinfiltration(100%) fi l l i ng
{ increasedpericolonicfat (60%) ! circularlinewith sharpouteredge + fuzzyblurred
Cx: intussusception, volvulus inneredge (en faceview in doublecontrastBE)
Rx: stool softeners,oral polyethyleneglycol-electrolytd ri Giant sigmoid diverticulurr = largegas-containing
solution(Golytely@), increasingdose of pancreatic cyst (air entrapmentsecondaryto ball-valve
enzymesupplements,mucolyticagents mechanism)arisingin leftiliacfossa
(N-acetylcysteine) orally/ with Gastrografin@ C T:
enema { roundedoutpouchings containingair t contrast
DDx: appendicitis,partialintestinalobstruction(adhesion material(= diverticula)
/ stricturefrom previousbowelsurgery) r/ circumferential sawtooth-likethickeningof colonic
haustra+ distortedluminalcontour(= rnuscular
DISEASE
DIVERTICULAR OF COLON hypertrophy)
= oVeroctivity
of smoothmusclecausingherniationof Cx: bleeding(usuallyrightcolon),diverticulitis
mucosa+ submucosathroughmusclelayers (usuallysigmoidcolon)
lncidence: 5-10% in Sth decade;3348% over age 50;
50-65% Past7th decade; M:F = 1:1; most
commonafflictionof colon in developed ColonicDiverticulitis
countries = perforationof diverticulumwith intramural/ localized
Cause: decreasedfecal bulk (diet high in refinedfiber pericolicinflammatorymass
+ low in roughage) lncidence: 5o/oof population;in 10-35% of diverticular
Location: in 80% in sigmoid(= fl€llrowestcolonic disease;increasingfrequencywith age (in
segmentwith highestpressure);in 17"h 5-10% >45 Yearsof age, in 80%
distributedover entirecolon; in 4-l2hisolaIed >85 Yearsof age)
to cecum/ ascendingcolon Pathogenesis;mucosalabrasionfrom inspissatedfecal
materialleadsto perforationof thin wall
PrediverticularDiseaseof Colon . pain + localtenderness+ mass in LLQ
= longitudinal + circularsmoothmusclethickeningwith . fever(25/"),leukocytosis (36%)
redundancyof folds secondaryto myostatic . clinicalmisdiagnosisrate of 34-67"/"
contracture Location: sigmoidcolon(95%),cecum(4%)
{ "saw-toothsign" = ctowding+ thickeningof haustral r/ localizedileus
folds (shorteningof colonicsegment) { + patternof small bowelobstruction(kinking/ edema
r/ plumpmarginalindentations if small bowel adheresto abscess)
{ superimposedmusclespasm (relievedby { extraluminalgas in abscess/ fistula
antispasmodics) r/ pneumoperitoneum (rare)
DDx: hemorrhage; ischemia;radiationchanges; BE (77-86% sensitive):
pseudomembranous colitis ./ focal area of eccentricluminalnarrowingcausedby
pericolic/ intramuralinflammatorymass:
ColonicDiverticulosis ./ annularlesionmimickingcarcinoma
= ?cguiredherniations of mucosa+ muscularis
mucosaethroughthe muscularispropriawith wall
componentsof mucosa,submucosa,serosa= false mesentery
diverticulaof pulsiontype
Location: predominantlyleft-sidedcolon taeniamesocolica
Site:
(a) lateraldiverticulaarise betweenmesenteric
circular muscle
+ antimesenteric teniaeon oppositesides
(b) antimesentericintertaenialdiverticulaoppositeof
mesentericside
lntramuraltype vasa recta (= nutrientarteries)pass lateral
diverticulum
throughthe circularmuscle(weaknessin muscular
wall) and are carriedover the fundusof the diverticula
as it enlarges
^/ size: initiallytiny (3-1O-mm)V-shapedprotrusions
increasingup to severalcm in diameter taenia omentalis taenia libera
r/ OuOOty appearanceof air-containing diverticula
r/ residualbariumwithindiverticulafrom previousstudy antimesenteric
{ spiky irregularoutline(antimesenteric intertaenial intertaenial diverticula
ridge is typicalsite for intramuraldiverticula)
r/ smoothdome-shapedappendageswith a short neck Cross Section through Colon
 Gastroi ntestinal Disorders 813

r/ markedthickening+ distortionof mucosalfolds ColonicDiverticularHemorrhage

r/ tetheredspiculatedmucosalfolds Not relatedto diverticulitis
r/ centrallyamputateddiverticulum lncidence: ]n 347"/" of diverticulosis
! extraluminal contrasl= peridiverticulitis: Location: 75/"located in ascendingcolon(largerneck
r/ ,,double-tracking" = pericolonic longitudinal
sinus dome of diverticula)
tract . massiverectalhemorrhagewithoutpain
r/ pericoloniccollection= peridiverticular abscess r/ extravasationof radionuclidetracers
^/ fistulato bladder/ smalibowel/ vagina
{ angiographic contrastpoolingin bowellumen
CT,(79-93% sensitive, 77o/"specific): Rx: (1) transcatheter infusionof vasoconstrictive
r/ inflameddiverticulum: agents(Pitressin@)
r/ pericolicfat stranding= poorlymarginatedhazy (2) embolizationwith Gelfoam@
area of increasedattenuation+ fine linearstrands
, withinpericolicfat (98%) RightColonicDiverticulitis
r/ diverticula(84%)= flask-shapedstructures
= congehitaltrue diverticulum
projectingthroughcolonicwall + filledwith air /
Frequency: 1:34-1:300appendectomies
barium/ fecal material
"centipede" Age: any; peak prevalenceat 35-45 years of age
, r/ sign = hyperemicengorgedvasa recta . protractedmild pain; palpablemass in 33%
r/ bowelwall thickening:
r/ solitarydiverticulum
containinga'12-mmfecolith
r/ circumferential bowelwall thickeningof >4 mm surroundedby inflamedfat
(70%)
r/ markedcircumferential colonicwall thickening
r/ focallythickened+ inflamedcolonicwall
Cx: pericolonicabscess
! "arrowheadsign"= funnelof intraluminal contrast Prognosis; spontaneousevacuationinto coloniclumen
mediumI air in focallythickenedcofonicwall
Rx: conservative
centeringaboutoccludedorificeof inflamed
DDx: appendicitis
diverticulum (27%)
r/ ttuiOcollection:
DUMPING
SYNDROME
r/ frank abscess(47%)= ceotralliquid/ gas
= earlypostprandialvascularsymptomatology of sweating,
^/ ttuiO+ air of peritonitis(16%)
flushing,palpitation,
feelingof weaknessand dizziness
r/ ttuiOat root of sigmoidmesentery
Pathophysiology: rapidenteringof hypertonicsolution
r/ tract formation:
r/ fistulaformation(a%\ mostcommonly intojejunumresultingin fluidshiftfrom
colovesical, bfoodcompartmentinto small bowel
alsocolovaginal, coloenteric, lncidence: 1-5h; M:F = 2:1
colocutaneous
0 Roentgenologic findingsnot diagnosticl
r/ intramuralsinustracts (9%)
r/ fecolith r/ rapidemptyingof bariuminto small bowel (= loss of
r/ colonicobstruction(12%) gastricreservoirfunction)
Rx: lyingdown,diet
r/ ureteralobstructio n (7%)
DDx: late postprandialhypoglycemia(90-120 minutes
US (85-98% sensitive,80-g7o/ospecific):
after eating)
r/ thickeningof bowelwall = >4 mm distancebetween
echogeniclumeninterfaceand serosa
r/ diverticula= roundI ovalhypo-/ hyperechoicfoci DUODENAL
ATRESIA
protrudingfrom colonicwallwith focaldisruptionof = ffiostcommoncauseof congenitalduodenal
obstruction;secondmostcommonsiteof
, normallayercontinuity+ internalacousticshadowing gastrointestinalatresiasafter ileum
r/ inflammatory pericolicfat = regionallyincreased
echogenicity adjacentto colonicwall+ ill-defined lncidence; 1:10,000;M:F = 1:1
hypoechoiczones Etiology: defectivevacuolizationof duodenumbetween
r/ pericolicabscess 6th and 11th weeks of fetal life; rarelyfrom
Prognosis; (a) self-limiting(usually) vascularinsult(extentof obstructionusually
(b) transmuralperforation involveslargerregionswith vascularinsult)
(c) superficialulceration Age at presentation: first few days of life
. persistentbiliousvomitinga few hoursafterbirthi
(d) chronicabscess
Cx: (1) Colonicobstruction following1st feeding(75%)
. rapiddeteriorationsecondaryto loss of fluids
(2) Fistulato bladder/ vagina/ smallbowel
(3) Free perforation(rare) + electrolytes
DDx: (1) Colonicneoplasm(shortersegment,heaped- lsolatedsporadicanomaly(30-52%)
up margins,ulceratedmucosa) Associated anomalies (in 50-60%):
(2) Crohncolitis(double-tracking (1) Downsyndrome(20-gg%);
longerthan
1 0c m ) 0 25% of fetuseswith duodenalatresiahave Down
Rx: antibiotics, surgery(in 2E/"), percutaneous syndrome!
abscessdrainage 0 <5/o of fetuseswith Down syndromehave
duodenalatresia!
814 Radiology Review Manual

(2) CHD (8-30-50%): endocardialcushiondefect,VSD Location:(a) bulbar(95%):

(3) Gastrointestinal anomalies(26%): anteriorwall (50%),posteriorwall (23Yo),
esophagealatresia,biliaryatresia,duodenal inferiorwall (22/"),superiorwall (5%)
duplication,imperforateanus, smallbowelatresia, r/ bulbardeformityin 85%
malrotation, Laddbands,Meckeldiverticulum, (b) P ostbul bar(3-5% ): M:F = 7:1;
transposedliver,annularpancreas(20%), majorityon medialwall above papilla;
preduodenal portalvein . hemorrhagein 66%
(4) Urinarytract anomalies(8%) ^/ edema + spasm maYobscureulcer
(5) Vertebral+ rib anomalies(37%) { smoothroundedindentationof lateralwall
Location:(a) usuallydistalto ampullaof Vater (80%) r/ frequently cm roundi ovoid(5% linear)ulcerniche
<1
(b) proximalduodenum(20%) r/ "kissingulcers"= ulcersoppositefrom each otheron
{ "double bubblesign" = gas-fluidlevelsin duodenalbulb anterior+ posteriorwall
+ gastricfundus r/ giantduodenalulcer>2cm (rare)with highermorbidity
r/ total absenceof intestinalgas in smalli largebowel + mortality;may be overlookedby simulatinga normal/
r/ colonof normalcaliber deformedscarredduodenalbulb
OB-US (usuallynot identifiedpriorto 24 weeks GA): i "cloverleafdeformity,hourglassstenosis"(healedstage)
. * elevatedAFP with prestenoticdilatationof recesses
r/ "doublebubblesign"= simultaneous distensionof Cx: (1) Obstruction(5%)
stomach+ 1st portionof duodenum,continuityof fluid (2) Perforation (<10%): anterior> posteriorwall;
betweenstomach+ duodenummust be fistulato gallbladder
demonstrated (3) Penetration(<5%)= sealedperforation
^/ increasedgastricperistalsis (4) Hemorrhage(15%): melena> hematemesis
r/ polyhydramnios in 3rd trimester(100%) Rx: antralresection(Billrothl) + vagotomy
Prognosis: 36/" mortalityin neonates
DDx: (1) Prominentincisuraangulariscausing
bidissectionof stomach VARICES
DUODENAL
(2) Choledochalcyst = dilatedcollateralveinssecondaryto portalhypertension
(3) Annularpancreas (posteriorsuperiorpancreaticoduodenal vein)
(4) Peritonealbands { lobulatedfillingdefects(bestdemonstratedin prone
(5) Intestinal
duplication position,maximalluminaldistensionwill obliteratethem)
Cx: prematurity(a0%) secondaryto preterm labor r/ commonlyassociatedwith fundal+ esophagealvarices
relatedto polyhydram nios

DIVERTICULUM
DUODENAL CYST
DUPLICATION
lncidence: 1-5/" of Gl studies;22/oof autopsies = UncorTlrTlon congenitalanomalyfound anywherealong
A . P RI M A RYD ]VE R T IC U L U M alimentary tract from tongueto anus
= fitUCos€llprolapsethroughmuscularispropria lncidence: 15% of pediatricabdominalmassesare
posteriorly(8%), lateralwall (4%) gastrointestinal duplicationcysts
B . S E CO NDA R YD IV ER T IC U L U M Theories of formation:
= all layersof duodenalwall = true diverticulum
as (1) Abortivetwinning
complication of duodenal/ periduodenal (2) Persistent embryologic diverticula
inflammation (3) Split notochord
Location: almostinvariablyin 1st portionof
duodenum
. mostlyasymptomatic
Cx: (1) Perforation+ Peritonitis
(2) Bowelobstruction
(3) Biliaryobstruction
(4) Bleeding
(5) Diverticutitis

DUODENAL
ULCER
lncidence; 200,000cases/year;2-3x morefrequent
than gastriculcers; M:F = 3:1
Pathophysiology: too much acid in duodenumfrom
(a) abnormallyhigh gastricsecretion
(b) inadequateneutralization
Predisposed: cortisonetherapy,severe cerebralinjury,
aftersurgery,chronicobstructive
pulmonarydisease Duodenal Diverticula
 Gastrointestinal
Disorders 815

(4) Aberrantluminalrecanalization (Bremer):foregut DDx:

epitheliumgrowsand obliterateslumen(solidstage (1) Omentalcyst (greateromentum/ lessersac,
for esophagus,smallbowel,colon);laterproduces mul ti l ocul ar)
secretionsthat form vacuolesin the intercellular (2) Mesentericcyst (betweenleavesof small bowel
space;vacuolesline up longitudinally and coalesce mesentery)
to form new lumen;failureof an aberrantvacuoleto (3) Choledochalcyst
coalescecreatesa wall cyst (4) Ovariancyst
(5) Intrauterinevascularaccident(Favara) (5) Pancreaticpseudocyst
associatedwith alimentarytract atresiain g% (6) Cysticrenaltumor
Age: presentationoften in infancyI earlychildhood (7) Abscess
Path: sphericalcyst / tubularstructurelocatedin / (8) Meckeldiverticulum (communicates
with Gl tract)
immediatelyadjacentto gastrointestinal tract; (9) Lymphangioma
sharesa commonmusclewall + bloodsupply;has (10) Mesentericlymphoma
a separatemucosallining;cyst contentsare (11) lntramura t ul m o r
usuallyserous
Histo: smoothmusclewall + linedwith alimentarytract ColonicDuplicationCyst
mucosa;ectopicmucosa;squamous,transitional, lncidence: 13o/o of all alimentarytract duplications
ciliatedmucosa;lymphoidaggregates;ganglion A. CySTtCCOLONTCDUPL|CAT|ON(7%)
cells Path: closedsphericalcyst; containsgastric
0 Gastricmucosa+ pancreatictissueare the only mucosain 2o/"+ ectopicpancreatictissuein
ectopictissuesof clinicalimportance! 5Y"
. respiratorydistress(withesophagealduplication) . abdominalmass,bowelobstruction
. palpableabdominalmass / constipation
. Gl hemorrhage
. nausea,emesis(dueto partial/completeobstruction)
Location: cecum(40%)+ intussusception
tl ar lintestinalmattercan entercyst (20%)
Location: ileum(30-33%),esophagus(17-20%),colon B. COLORECTALTUBULARDUPL|CAT|ON(6%)
(13-30%),jejunum(10-13%),stomach(7"/"), = D U P LIC A TION OF TH E H IN D GU T
pylorus(4%),duodenum(4-SA), ileocecal = double-barreled duplicationinvolvingpart/ all of
junction(4/"), rectum(4%);
largebowelwith "twin"segmenton mesentericI
0 In 7-15Y"concomitant duplications antimesenteric side
elsewherein the alimentarytract! Symptomaticage: neonatalperiod/ infancy;
Site: on mesentericaspectof alimentarycanal
M : F= 1 : 2
Morphology: May be associated with:
(a) largespherical/saccularcyst (82%) rectogenital/ rectourinaryfistula,duplicationof
(b) small intramuralcyst internal/ externalgenitalia,vertebralanomalies,
(c) tubularsausage-shaped cyst (18%): commonly multisystem congenitalanomalycomplex
alongsmall+ largebowel;frequentlycommunicates . bowelobstruction/ constipation
with lumenof adjacentgut . passageof fecesthroughvagina
BE: r/ simultaneous opacification of true + twin colon
mass extrinsicto bowel lumen ri duplicationmay terminateat
US: (a) 2nd functionalanus
elongatedtubular/ sphericalcysticmass: (b) imperforateperinealorifice
r/ sonolucentmasswith goodthroughtransmission (c) fistulouscommunication with GU tract
(dueto clearfluidcontent) C . D OU B LEA P P E N D IX
r/ echogenicmass (dueto hemorrhage+ inspissated
material)
m us c ularr im s i g n(= e c h o g e n i icn n e rm u c o sall i ni ng DuodenalDuplicationCyst
+ hypoechoicouter rim) in 47o/"
lncidence: 5/" of all alimentarytract duplications
cyst parallelingnormalbowellumen
Path: noncommunicating sphericalcyst;may contain
CT:
ectopicgastricmucosain 21"/",small bowel
smoothlyroundedfluid-filledcyst/ tubularstructure mucosa,pancreatictissue
thin slightlyenhancingwall . obstructivesymptoms,palpableabdominalmass
MR: . hemorrhage(dueto pepticulceration)
heterogeneous signalintensityof intracystic
fluidon . jaundice(dueto biliaryobstruction)
TlWl + homogeneoushighsignalintensityon T2Wl . pancreatitis(due to ectopicpancreatictissue)
Cx: bowelobstruction,intussusception(due to cyst at
Site: on mesentericsideof anteriorwall of 1st
ileocecaljunction),
smallbowelvolvulus(dueto + 2nd portionof duodenum
weightof duplication),
bleeding(dueto presenceof ./ mass in concavityof duodenalC-loop
gastricmucosa/ pressurenecrosisof adjacent ./ compression + displacement of 1st l2nd portionof
mucosaby cyst expansion/ from intussusception)
duodenumsuperiorly+ anteriorly
816 RadiologyReview Manual
Cholangiography:
r/ may communicatewith pancreaticductalsystem
throughthe aberrantduct of an accessorylobe
Cx: pancreatitisfrom perforationof duplicationcyst
DDx: pancreaticcyst, pancreaticpseudocyst,
choledochalcyst,choledochocele, duodenal
intramuraltumor,pancreatictumor
EsophagealDuplicationCyst
arisesfrom foregut
lncidence: 10-20/" of all alimentarytract duplications;
0.Y2.5% of all esoPhageal
M : F= 2 : 1
Path: containsectopicgastricmucosain 43/"
Histo: containsno cartilage,linedby alimentarytract
mucosa
Associatedwith: vertebralanomalies(spinabifida,
hemivertebra,f usiondefects),
esophagealatresia,smallbowel
duplication (18%)
Location: adjacentto esophagus/ withinesophageal
musculatureat any level,paraspinal
; :L = 2 :1 ;i n ri g h tp l e u raspace
po s i ti o nR
detachedfrom esoPhagus(rare)
A. CERVTCAL ESOPHAGUS(23%)
. asymptomaticenlarginglateralneck mass
. upperainruayobstructionin newborn
DDx: thyroglossalduct cyst, branchialcleft cyst,
cystichygroma,cervicaltumor,cervical
lymphadenopathy
B . M T DE S OP H A G U(1
S7 % )
. severeupperairwayobstructionin early infancy
DDx: bronchogeniccyst, neurentericcyst,
intramuralesophagealtumor
c. DrsrAL ESOPHAGUS(60%)
. frequentlyasymptomatic
Location: paraspinal
DDx: bronchogeniccyst, neurentericcyst,
intramuralesophagealtumor
r/ thick-walledclosedsphericalcyst, almostnever
communicating
CX R:
r/ posteriormediastinalmass + air{luid level
r/ lobarconsolidation+ centralcavitation(from
autodigestionof lung tissueby gastricsecretions)
r/ thoracicvertebralanomalies
UGI:
r/ displacementof esophagusby paraesophageal
MASS
r/ intramuralextramucosalmass
US:
cyst + innermucosallining
r/ hypoechoicfluid-filled
CT:
{ sharplymarginatedhomogeneousnear-water
densitymasswithoutenhancement
Cx: (1) Pepticulceration(secondaryto gastric
mucosa)
(2) Perforation(secondaryto penetratingulcer)
(3) Hematemesis (fromerosionintoesophagus)
(4) Hemoptysis+ autodigestionof pulmonary
tissue(fromerosionintotracheobronchial
tree)
GastricDuplicationCYst
= intramuralgastriccyst linedwith secretoryepithelium
lncidence: 7"/" of all alimentarytract duplications
Path: noncommunicating sphericalcyst (majority);
may communicatewith aberrantpancreatic
duct; ectopicpancreatictissuefound in 37"/o
masses; Symptomaticage: infancy; in75'/" detectedbeforeage
1 2 ; M : F= 1 : 2
. pain (fromoverdistension of cyst, rupturewith
peritonitis,pepticulcerformation,internalpancreatitis)
. vomiting,anemia,fever
. symptomsmimickingcongenitalhypertrophic pyloric
stenosis(if duplicationin antrum/ pylorus)
Most commonsite: greatercurvature(65%)
r/ paragastric cysticmass up to 12 cm in size,indenting
greatercurvature
r/ seldomcommunicateswith main gastriclumenat one
l or both ends
{ may enlar,ge+ ulcerate
^/ Tc-ggmuptake
US:
r/ cyst with two wall layers: innerechogeniclayerof
mucosa+ outer hypoechoiclayer of muscle
r/ clear/ debris-containing fluid
Cx: (1) Partial/ completesmall bowelobstruction
(2) Relapsingpancreatitis(withductal
communication)
(3) Ulceration, perforation, fistulaformation
DDx: pancreaticcyst, pancreaticpseudocyst,
mesentericcyst,leiomyoma,adenomatouspolyp,
hamartoma,lipoma,neurofibroma,teratoma
RectalDuplicationCYst
lncidence: 4o/"of all alimentarytract duplications
Path: sphericalfluid-filledcyst;may containduodenal/
gastricmucosa+ pancreatictissue
Site: posteriorto rectum/ anus
{ communicationwith rectum/ perianalfistula(in 20%)
Symptomaticage: childhood
. constipation + fecalsoiling
. palpableretrorectal/ retroanalmass
. intractableexcoriationof perianalskin (withchronic
perianalfistula)
{ cysticmass; may be echogenic(due to solid material
+ gas from communication with rectum)
DDx: anteriormeningocele,sacrococcygeal teratoma,
retrorectalabscess,pilonidalcyst, sacralbone
tumor
CYst
SmallBowelDuPlication
lncidence: most commonof all alimentarytract
duPlications
Symptomaticage: neonatll,P:liod (1/3); <2 years of
age (in 72%)
 Gastrointestinal
Disorders 817

Path: containsectopicgastricmucosain 24./";ectopic Age: in children+ youngadultswith allergy

pancreatictissue in jejunum(8%) + eosi nophi l i a
May be associatedwith: small bowel atresia A . E OS IN OP H ILIC GR A N U LOMA
. neonatalbowel obstruction = FIBROUS POLYPOID LESION
. intussusception, palpablemass = INFLAMMATORY PSEUDOTUMOR
. acuteabdominalpain,hemorrhage = localizedform / circumscribedtype
Location: ileum(33%),jejunum(10%),ileocecal(4%) Location: almostexclusivelyin stomach(most
r/ low small bowelobstruction+ soft-tissuemass commonin antrum+ pylorus)
r/ cyst may serve as lead pointfor intussusception r/ submucosalpolypoidmass / pedunculatedpolyp
DDx: mesentericcyst, pancreaticpseudocyst,omental B . E OS IN OP H ILIC GA S TR OE N TE R ITIS
cyst, exophytichepaticcyst,ovariancyst = diffusetype
= eosinophilic infiltration
of mucosa,submucosa,and
Thoracoabdominal
Duplication muscularlayersof smallintestine+ stomachby
= FOREGUTDUPLICATION matureeosinophils(? gastricpendantto Loffler
= long tubularcyst closedat its cranialend, passing syndrome)
throughdiaphragmthroughits own hiatus,in 60% . recurrentepisodesof abdominalpain,diarrhea,vomiting
communicating with normalduodenumi jejunumI . weightloss
ileum . hematemesis(from ulceration)
lncidence: 2'/" of all alimentarytract duplications . peripheraleosinophilia, anemia
Assaciatedwith: thoracicvertebralanomalies . historyof systemicallergy/ food allergy
Histo: gastricmucosain 29/" Location: entiresmallbowel(particularly jejunum),distal
Symptomaticage: 50% during neonatalperiod; stomach,omentum,mesentery
80% within1styear of life Site: (a) mucosal
' severerespiratorydistress (b) muscular
. chestpain,Gl bleeding,anemia (c) serosal(rare)
^1tubularrightposteriormediastinalmass + air @ Stomach(almostalwayslimitedto antrum)
r/ thoracicvertebralanomaly ri "wet stomach"
r/ contrastmaterialmay entbrthroughdistalconnection r/ ulcersare rare
(a) mucosaltype
ECTOPIC
PANCREAS ./ enlargedgastricrugaei cobblestonenodules/
= PANCREATIC REST= MYOEPITHELIAL HAMARTOMA polyps
lncidence: 2-10/" of autopsies; M:F = 2:1 (b) musculartype
. asymptomatic ^/ thickened+ rigidwallwith narrowedgastric
Location:lesionsmay be multiple antrum/ pylorus
(a) greatercurvatureof antrum 1-6 cm from pylorus, { Outt<y intraluminal mass up to 9 cm in size
pylorus,duodenalbulb,proximaljejunum(in 80%) Cx: pyloricobstruction
(b) ileum,Meckeldiverticulum DDx: hypertrophicgastritis,lymphoma,carcinoma
r/ smoothcone-/ nipple-shaped submucosalnodule @ Small bowel (involvedin 50%)
1-5 cm in size i/ separationof smallbowel loops
{ centralumbilicationrepresentingorificeof filiformduct (a) mucosaltype
. malabsorption+ hypoproteinemia
ENTERIC CYST r/ thickening+ distortionof folds predominantlyin
= cystlinedbygastrointestinal
mucosa
without
bowelwall jejunum
Etiology: migrationof small bowel/ colonicdiverticulum (b) submucosal/ musculartype
into mesentery/ mesocolon { motilitydisturbance
Path: unilocularthin smooth-walledcyst with serous r/ small-bowelobstruction
contentslinedby entericepithelium+ thinfibrous r/ effacementof mucosalpattern+ narrowingof
wall l umen
US: (c) serosaltype
r/ hypoechoiccysticmass,occasionallywith septations { ascites
DDx: duplicationcyst (reduplication
of bowelwall) Prognosis; tendencytowardspontaneousremission
Rx: steroidsI removalof sensitizingagent
EOSINOPHILIC GASTROENTERITIS
= uncorT'rrT'ron
self-limitedform of gastroenteritis
with
remissions+ exacerbationscharacterized by infiltration E P IP LOIC A P P E N D A GITIS
of eosinophilicleukocytesinto stomach/ small bowel = r?f€ inflammation of one of the 100 epiploicappendages
wall + usuallymarkedperipheraleosinophilia Cause: (a) primary: torsion(exercise),venous
Cause: unknown thrombosis
Histo: fibroustissue+ eosinophilicinfiltrateof (b) secondary: inflammationof adjacentorgan
gastrointestinal
mucosa (eg,diverticulitis,
appendicitis)
818 RadiologyReview Manual

Histo: acute infarctionwith fat necrosis,inflammation, CT staging(Moss):

thrombosedvesselswith hemorrhagicsuffusion Stage 1 intraluminal tumori localizedwall thickening
. abruptonsetof localizedabdominalpain (RLO in 50%), of 3-5 mm
graduallyresolvingover 3-7 days Stage2 localized/ circumferential wall thickening
. palpablemass (10-30%) > 5 mm
- + p€fitonealsigns Stage3 contiguousspreadinto adjacent
. oorrr?l/ mildlyincreasedWBCs mediastinum (trachea,bronchi,aorta,
0 Almost neversuspectedpreoperatively! pericardium)
Location: anterolaterally I (occasionally)
anteromedially ^/ loss of fat planes(nonspecificdue to
to ascending/ descendingi sigmoidcolon cachexia,often still resectable)
US: ^/ mass in contactwith aorta >90" arc (in
r/ solid hyperechoicnoncompressible ovoid mass 20-7 0% still resectable)
r/ hypoechoicmargin(93%) r/ displacement/ compressionof airway
CT: (90-100% accuracYfor invasion)
r/ pericolicoval-shapedpedunculatedmass, 1-4 cm in ./ esophagotracheal / -bronchialfistula
diameter,with fat attenuation(approx.-60 HU): (unresectable)
r/ hyperattenuating peripheralrim Stage4 distantmetastases
{ internalfat stranding r/ enlargedabdominallymph nodes
r/ periappendigeal fat infiltration >10 mm (12-85% accuracy)
d thickeningof adjacentvisceralperitoneallining(93%) ./ hepatic,pulmonary,adrenalmetastases
Prognosis: spontaneousresolution r/ directerosionof vertebralbody
Rx: conservativemanagement ./ tumor>3 cm wide = highfrequencyof
DDx: torsion/ infarctionof greateromentum,diverticulitis, extraesophageal sPread
appendicitis Histo:
(1) Squamouscell carcinoma(50-70%)
(2) Adenocarcinoma (30-50%)arisingfrom mucosal/
ESOPHAGEAL
CANCER submucosalglandsor heterotopicgastricmucosaor
lncidence: <1o/"of all cancers;4-1O/" ol all Gl columnar-lined epithelium(Barrett)
malignancies; 11,000cases/year(USA in (a) in 70o/otrom Barrettesophagus
1 9 9 4 ) ;M : F= 4 : 1; (b) gastricadenocarcinomainvolvingGE junction
Blacks:Whites = 2:1 (c) in submucosal/ deep esophagealglands
High-riskregions: lran, parts of Africa, ltaly, China (d) ectopicgastricmucosain esophagus
Predisposing factors: 0 2,500new caseseach year;M:F = 7:1
achalasia(riskfactorof 1,000x), asbestosis,Barrett ^/ tendencyto invadegastriccardia+ fundus
esophagus,celiacdisease,radiationexposure,caustic (3) Spindle-cell squamouscarcinoma= carcinosarcoma
stricture(riskfactorof 1,000x), Plummer-Vinson = pseudosarcoma
syndrome,tannins,alcohol,tobacco,historyof oral/ Histo: squamous+ sarcomatouselements
pharyngealcancer,tylosispalmariset plantaris Age: in men >45 years
Location: usuallymiddlethirdof esophagus
mnemonic; "BELCHSPAT" ri largebulky polypoidsmooth,lobulated,scalloped
Barrettesophagus intraluminal mass;may be pedunculated
EIOH abuse DDx: lymphoma,other sarcomas
Lye stricture (4) Mucoepidermoid carcinoma,adenoidcystic
Geliacdisease carcinoma
Head and neck tumor (5) Leiomyosarcoma, rhabdomyosarcoma,
Smoking fibrosarcoma, malignantlymphoma
Plummer-Vinson syndrome (6) Secondarytumorinvolvement from: thyroid,larynx
Achalasia,Asbestosis (7) Metastasisfrom: breast,melanoma,Gl tract
Tylosis
. dysphagia(87-95%)of <6 months'duration
Cancer Staging: . w ei ghtl oss(71% )
TNM system: . retrosternalpain (46%)
T1 tumorinvadeslaminapropria/ submucosa . regurgitation(29%)
f2 tumorinvadesmuscularispropria Location: upper1/3 (15-20%);middle113(374a%);
T3 tumor invadesadventitia lower 113(3843%)
T4 tumor invadesadjacentstructures
Radiologic types:
S t ageI = T 1 ,N 0 ,M 0 Sta g el l l = T 3 , N 1,M0 (1) Polypoid/ fungatingform (mostcommon)
StagellA = T213,N0,M0 or T4,N0/1,M0 r/ sessilei pedunculatedtumor with lobulated
S t agellB = f1 1 2 ,N 1 ,M 0 Sta g el V = T 1 - 4,N 0i 1,M1 surface
 Gastrointestinal
Disorders 819

r/ protruding,irregular,polycyclic, overhanging, ! muftipfetiny rounded/ flask-shapedbariumcollections

steplike"applecore" lesion in longitudinal rowsparallelto long axisof esophagus:
(2) Ulceratingform ^/ appearto "float"outsideesophaguswithoutapparent
r/ largeulcernichewithinbulgingmass communication with lumen
(3) Infiltratingform { esophagealstricture:
{ gradualnarrowingwith smoothtransition r/ short stricturein distalesophagus(common)
(DDx: benignstricture) r/ long stricturein cervical/ uppe?thoracicesophagus
(4) Varicoidform = superficialspreadingcarcinoma (classic)
Histo: longitudinalextensionconfinedto mucosa
/ submucosa ESOPHAGEAL
PERFORATION
./ focal area of confluentmucosalnodules/ plaques = ESOPHAGEAL RUPTURE
DDx: Candidaesophagitis Cause:
Metastases:
(1) latrogenicinjury(mostcommoncause,SS%):
(a) lymphogenic:anteriorjugularchain
complication of endoscopy,dilatationof stricture,
+ supraclavicular nodes(primaryin upper1/3); bougie,disruptionof suturelinefollowingsurgical
paraesophageal+ subdiaphragmatic nodes (primary anastomosis,attemptedintubation
in middle1/3);mediastinal + paracardial
+ celiac (2) Spontaneousrupture= Boerhaavesyndrome(15%):
trunknodes(primaryin lower1/3)
emetogenicinjuryof the esophagusfrom sudden
(b) hematogenous:lung,liver,adrenalgland
increasein intraabdominal pressure+ relaxationof
C X R:
distalesophagealsphincterin the presenceof a
r/ widenedazygoesophageal recess with convexity moderateto largeamountof gastriccontents
toward right lung (in 30% of distal+ midesophageal (3) Closedchesttrauma(10%)
, cancers) lncidence: 1"/"of all blunt chesttrauma
! thickeningof posteriortrachealstripe+ right
Location:
paratrachealstripe>4 mm (if tumor locatedin upper
(a) cervical/ upperthoracicesophagus(82%)
thirdof esophagus) (b) just above gastroesophageal junctionalong
r/ widenedmediastinum
posterolateralwall on the left side
{ trachealdeviation (4) Esophageal carcinoma
{ posteriortrachealindentation/ mass (5) Retainedforeignbody (14o/"): coin,aluminumpop-
r/ retrocardiacmass
tops,metallicbutton,safetypin, invisibleplastictoy)
r/ esophagealair-fluidlevel
leadingto perforation(in pediatricage group)
r/ lobulatedmass extendinginto gastricair bubble
(6) Barrettulcer
r/ repeatedaspirationpner-^oniJ lwittr . pain,dysphagia,odynophagia
tracheoesophageal f istula) . rapidonset of ovenruhelming sepsis: fever,tachycardia,
Cx: fistulaformationto trachea(S-10%)/bronchj /
hypotension, shock
mediastinum
Prognosis: 3-5-20"/o S-yearsurvival rate; O/oS-year P l ai nfi l m(normali n 9-12/" ):
survivalrate for cancerof cervical { extensivepneumomediastinum
esophagus { V signof Naclerio= extrapleural air withinlower
Mean survivaltime:
mediastinum betweenparietalpleura+ diaphragm
90 days with subdiaphragmatic lymphadenopathy
180 days with localinvasion+ abdominalmetastases , (usuallyon left)
ri subcutaneousemphysemaof the neck
480 days withoutevidenceof invasion/ metastases
r/ delayedwideningof the mediastinum (secondaryto
Rx: (1) chemotherapy (fluorouracil,
cisplatin,bleomycin mediastinitis)
sulfate,mitomycin)+ surgery r/ hydrothorax(afterruptureinto pleuralcavity),usually
(2) chemotherapy + irradiation
(-4,000cGy) unilateralon left side
(3) chemotherapy + irradiation
+ surgery r/ hydropneumothorax (oftennot initiallyseen)
Operative mortality: 3-8y" r/ tett lower lobe atelectasis
r/ confirmationwith contraststudy (gO%of contrast
esophagogramsare positive)
ESOPHAGEAL INTRAMURAL C T:
./ focal extraluminalair collectionat site of tear (g2%;
PSEUDODIVERTICULOSIS
= dilatedexcretoryducts of deep mucousglands most usefulsign)
r/ periesophageal / mediastinal
hematoma/ fluid(92%)
Etiology: uncertain
{ pleuraleffusion(75%)
lncidence: about 100 cases in world literature
r/ esophagealwall thickening
Site: diffuse/ segmentalinvolvement
ln 90% associated with: r/ extravasationof oral contrast material
Esophagography with:
diabetes,alcoholism, any severeesophagitis(most
(1) water-soluble contrastmaterial(10%false-negative
often reflux/ Candida),esophagealstricture
results)
820 Radiology Review Manual

(2) barium(if resultwith water-solublematerial (c) in LAO projectionwith patientrecumbent/ in

negative) Trendelenburg position+ Valsalvamaneuver/ deep
inspiration
A. UP P E R/ M I D -E SOP H A G E AL
P ER F O R AT ION P l ai nfi l m:
Location:at levelof cricopharyngeus muscle(most ^/ lobulatedmassesin posteriormediastinum (visiblein
frequent) 5-8% of patientswith varices)
{ wideningof uppermediastinum r/ silhouettingof descendingaorta
r/ right-sidedhydrothorax ^/ abnormalconvexcontourof azygoesophageal recess
B. DISTALESOPHAGEALPERFORATION (more at levelof gastroesophageal junction
common,but not in bluntchesttrauma) UGI:
Cause: biopsy,dilatationof stricture,Boerhaave r/ thickenedsinuousinterrupted mucosalfolds(earliest
syndrome si gn)
./ teft-sidedhydrothorax { tortuousradiolucencies of variablesize + location
./ titttemediastinalchanges ^/ "worm-eaten"smoothlobulatedfillingdefects
Cx: (1) Acutemediastinitis ./ findingsmay be accentuatedaftersclerotherapy
(2) Obstructionof SVC C T:
(3) Mediastinalabscess r/ thickenedesophagealwall + lobulatedouter contour
Prognosis: 20-60/" mortality { scallopedesophagealluminalmasses
r/ right-/ left-sidedsoft-tissuemasses
(= paraesophageal varices)
ESOPHAGEAL VARICES r/ markedenhancement followingdynamicCT
= plexusesformedby dilatedsubepithelial veins
+ submucosalveins+ dilatedvenaecommitantes of the Cx: bleedingin 28/. within3 years;exsanguinationin
vagusnervesoutsidethe tunicamuscularis 10-15%
Anatomy: DDx: varicoidcarcinomaof esophagus
(a) anteriorbranchconnectedto left gastricvein
(b) posteriorbranchconnectedto azygos+ hemiazygos
system ESOPHAGEAL WEB
A . UP HI LLV A R IC E S = compl€te/ incompletecircumferential narrowingcaused
= collateralbloodflow from portalvein via azygosvein by 1-2-mmthick(verticallength)mucosalmembrane
intoSVC (usuallyloweresophagusdrainsvia left projectingintoesophageallumen;coveredby
gastricvein intoportalvein) squamousepitheliumon superior+ inferiorsurfaces
Cause: Age: middle-agedfemales
(a) intrahepaticobstructionfrom cirrhosis ? Association with:
0 ln <5% of patientswith portalhypertension Plummer-Vinson syndrome = Paterson-Kelly
(b) splenicveinthrombosis(usuallygastricvarices) syndrome(irondeficiencyanemia,stomatitis, glossitis,
(c) obstructionof hepaticveins dysphagia,thyroiddisorder,spoon-shaped nails)
(d) IVC obstructionbelow hepaticveins "BlEP"
Cause: mnemonic.'
(e) IVC obstructionabove hepaticvein entrance/ B-ring(Schatzkiring)
CHF ldiopathic(= transversemucosalfold)
(f) markedsplenomegalylsplenic Epidermolysis bullosa
hemangiomatosis (rare) Plummer-Vinson disease
r/ varicesin lower half of esophagus Path: hyperkeratosis + chronicinflammationof
B . DO W NHI LLV AR IC E S submucosa
= collateralbloodflow from SVC via azygosvein into . mostlyasymptomatic (unlessseverelystenosing)
IVC / portalvenoussystem(upperesophagus Location: in cervicalesophagusnearcricopharyngeus
usuallydrainsvia azygosvein into SVC) (mostcommon)> thoracicesophagus;
Cause: obstructionof superiorvena cava distalto occasionally multiple
entry of azygosvein (= superiorvena cava { visualizedduringmaximaldistension(in onetenth of a
syndrome)mostcommonlydue to lung second)
cancer,lymphoma,retrosternal goiter, ri arisesat rightanglesfrom anterioresophagealwall
thymoma,mediastinal fibrosis r/ ttrindelicatemembraneof uniformthicknessof <3 mm
r/ varicesin upper1/3 of esophagus
E X A M I NA T I OT
NEC H N IQ U E Cx: high riskof upperesophageal+ hypopharyngeal
(a) smallamountof barium(notto obscurevarices) carcinoma
(b) relaxationof esophagus(not to compressvarices): Rx: (1) balloondilatation
refrainfrom swallowingbecausesucceeding (2) bougienageduringesophagoscopy
swallowinitiatesa primaryperistalticwave that lasts DDx: stricture(circumferential+ thicker= 1-2-mm thick
for 10-30 seconds:sustainedValsalvamaneuver [verticallength]area of complete / incomplete
precludesfrom swallowing circumferentialnarrowing
 Gastrointestinal
Disorders 821

ESOPHAGITIS ./ narrowedlumen(fromspasm,pseudomembranes,
Acute Esophagitis markededema)
mnemonicfor cause: "CRlER" r/ "intramural = multipletiny indentations
diverticulosis"
Corrosives,Crohndisease + protrusions
Reflux r/ sluggishi absentprimaryperistalsis
Infection,Intubation r/ strictures(rare)
Epidermolysisbullosa r/ mycetomaresemblinglargeintraluminal tumor(rare)
Radiationtherapy Diagnosticsensitivity: endoscopy(97"/"),double
^/ thickened>3-mm-widefoldswith irregularlobulated contrast(88%),single
contour contrast(55%)
./ mucosalnodularity(= multipleulcerations Cx: (1) Systemiccandidiasis("microabscesses" in
+ interveningedema) liver,spleen,kidney)
r/ erosions (2) Gastricbezoardue to largefungusball (after
r/ verticallyorientedulcersusually3-10 mm in length long-standing esophagealcandidiasis)
^/ inflammatoryesophagogastric polyp = proximal Rx: ketoconazole/ fluconazole
gastricfold extendingacrossesophagogastric DDx: glycogenacanthosis,refluxesophagitis,
junction(rare) superficialspreadingcarcinoma,artifacts
r/ abnormalmotility (undissolvedeffervescentcrystals,air bubbles,
retainedfood particles),herpesesophagitis,
acutecausticingestion,intramuralpseudo-
GandidaEsophagitis diverticulosis,squamouspapillomatosis, Barrett
= MONILIASIS = CANDIDIASIS esophagus,epidermolysis bullosa,varices
0 Mostcommoncauseof infectiousesophagitis!
Organism; C. albicans,C. tropicalis;endogenous
(majority)/ transmittedby anotherhuman/ GausticEsophagitis
animal;oftendiscoveredin diseasedskin, = CORROSIVE ESOPHAGITIS
Gl tract,sputum,femalegenitaltract,urine Corrosive agents:
with an indwellingFoleycatheter lye (sodiumhydroxide), washingsoda (sodium
Predisposed: carbonate),householdcleaners,iodine,silvernitrate,
(a) individualswith depressedimmunity:hematologic householdbleaches,Clinitest@ tablets(tendto be
disease,renaltransplant,leukemia,chronic neutralizedby gastricacid)
debilitatingdisease,diabetesmellitus,steroids, 0 Severityof injurydependenton contacttime
chemotherapy, radiotherapy,AIDS + concentrationof corrosivematerial!
0 Mostcommontype of fungifoundwith Associatedwith: injury to pharynx+ stomach (7-8%):
opportunisticinfections! antralburnsmorecommonwith acid
(b) delayedesophagealemptying; scleroderma, (bufferingeffectof gastricacid on
strictures,
achalasia,S/P fundoplication alkali)
(c) antibiotics Location: middle+ lowerthirdsof esophagus
Path: patchy,creamy-whiteplaquescoveringa friable Stage | : acute necrosisfrom proteincoagulation
erythematousmucosa r/ mucosalblurring(edema)
Histo: mucosalplaques= necroticepithelialdebris r/ diffuselyatonic+ dilatedesophagus
+ fungalcolonies r/ tertiarycontractions/ spasm
' dysphagia(= 6;111sulty swallowing) Stage ll : frank ulcerationin 3-5 days
. severeodynophagia(= painfulswallowingfrom { ulceration+ pseudomembranes
segmentalspasm) Stage lll : scarring+ stricturefrom fibroblasticactivity
" intenseretro-/ substernalpain ./ tongsegmentalstrictureafter 10 days
. associatedwiththrush(= oropharyngeal moniliasis)in when acute edemasubsides(7-30%)
20-50-80%
Cx: (1) Esophageal / gastricperforation
during
Location: predilection for upper 112of esophagus ulcerativestage
r/ involvementof long esophagealsegments: (2) Squamouscell carcinomain injuredsegment
{ "cobblestone" appearance= rT'tUoosalnodularityin Rx: dilatationprocedure/ esophagealreplacement
, early stage (fromgrowthof colonieson surface) surgery
r/ longitudinalplaques= groupingof tiny 1-2-mm
nodularfillingdefectswith linearorientation
(= heaped-upareasof mucosalplaques) ChronicEsophagitis
{ shaggylfuzzy / serratedcontour(fromcoalescent ^/ luminalnarrowingwith taperedtransitionto normal
plaques,pseudomembranes, erosions,ulcerations, + proximaldilatation
intramuralhemorrhage) in fulminantcandidiasisof r/ circumferential
/ eccentricstricture
A I DS ^/ sacculations= pseudodiverticula
822 RadiologyReviewManual

Esophagitis
Drug-induced r/ singlemarginalulcer/ erosionat or adjacentto
= contactesophagitisdue to oral medications= "pill gastroesophageal ju nction
esophagitis" r/ multipleareas of superficialulcerationin distal
Agents: antibiotics(tetracycline, doxycycline), esophagus
quinidine,potassiumchloride,nonsteroidal { prominentmucosalfold endingin polypoid
antiinflammatory agents(aspirin),ascorbic protuberance withinhiatalhernia/ cardia
acid,alprenololchloride,emepronium ^/ interruption of primaryperistalsis at inflamedsegment
bromide,alendronate(= inhibitorof ./ nonperistaltic waves in distalesophagusfollowing
osteoclasticactivity) deglutition(85%)
. severeodynophagia ./ incompleterelaxationof LES (75%),incompetent
. historyof takingmedicationwith little/ no water sphincter(33%)
immediately beforegoingto bed { acid test = abnormalmotilityelicitedby acid barium
. rapidclinicalimprovementafterwithdrawalof ( p H1 . 7 )
offendingagent r/ "felinization" = transverseridgesof esophagus
Location: midesophagusat site of normalextrinsic secondaryto contractionof muscularismucosae
impressionsby aortic archI left mainstem (similarto cat esophagus)
bronchus/ leftatrium NUC (pertechnetate):
r/ localizedclusterof tiny ulcersdistributed { esophagealactivity(Barrettesophagussimilarto
circumferentially(mostcommonly) ectopicgastricmucosa)
r/ superficialsolitary/ severaldiscreteulcers Reflux fesfs;
Prognosis; ulcersheal within7-10 days aftercessation 1. Reflux of bariumin RPO position,may be elicited
of offendingmedication by coughing/ deep respiratorymovements/
DDx: herpesesophagitis(immunosuppressed patient, swallowingof saliva+ water / anteflexionin erect
lesslocalized); refluxesophagitis(heartburn, position:only in 50% accurate
distalesophagusnearesophagogastric junction) 2. Water-siphontest: in 5/" false negative;large
numberof falsepositives
3. Tuttletest = measurement of esophagealpH:
RefluxEsophagitis 967oaccurate
= esophogealinflammationsecondaryto refluxof acid- 4. Radionuclide gastroesophageal refluxtest
pepticcontentsof the stomach;refluxoccursif resting (typicallycombinedwith gastricemptyingtest):
pressureof LES <5 mm Hg (may be normalevent if Technique; ROI drawn over distal esophagus
followedby rapidclearing) + comparedwith time-activitycurve
Prevalence: in 2c^hof gastroesophageal reflux over stomach,scaledlo 4/"
Histo: basalcell hyperplasiawith wall thickening { esophagealactivity>4ohstomachactivity
+ thinningof epithelium, mucosaledema Cx of reflux:
+ erosions,inflammatoryinfiltrate (a) from acid + pepsinactingon esophagealmucosa:
Determinants:(1) Frequencyof reflux 1. Motilitydisturbance
(2) Adequacyof clearingmechanism 2. Stricture
(3) Volumeof refluxedmaterial 3. Schatzkiring
(4) Potencyof refluxedmaterial 4. Barrettesophagus
(5) Tissue resistance 5. lron-deficiency anemia
Refluxpreventingfeatures: 6. Reflux/ pepticesoPhagitis
(1) Loweresophagealsphincter (b) from aspirationof gastriccontents
(2) Phrenoesophageal membrane 1. Acuteaspirationpneumonia
(3) Lengthof subdiaphragmatic esophagus 2. Mendelsonsyndrome
(4) Gastroesophageal angleof His (70-110") 3. Pulmonaryfibrosis
May be associatedwith: slidinghiatalhernia(in most
patients),scleroderma, ViralEsophagitis
nasogastricintubation Predisposed: immunocompromised, eg, underlying
. heartburn,epigastricdiscomfort malignancy, illness,radiation
debilitating
. choking,globushystericus treatment,steroids,chemotherapy,
. retrosternalpain AIDS
. thoracic/ cervicaldysphagia
Site: usuallylower1/3/ lower112with continuous Cytomegalovir us EsoPhagitis
diseaseextendingproximallyfrom GE junction Organism; memberof herpesvirusgroup
{ segmentalesophagealnarrowing(edema/ spasm/ Associatedwith: AIDS
stricture) . severeodynophagia
r/ poorlydefinedtiny mucosalelevations("mucosal r/ diffusetynormalmucosalbackground
granularity")on thickened/ nodularlongitudinal folds r/ one / more giant ovoidflat ulcers(up to severalcm
(mucosaledema+ inflammation) in earlystages in size) near gastroesophageal junction

i/ discretesmallsuperficial
ulcersindistinguishable
from herpesesophagitis(uncommon)
Rx: ganciclovir(relativelytoxic)
Dx: endoscopicbrushings,biopsyspecimen,
cultures
HerpesEsophagitis
0 2nd mostcommoncauseof opportunistic infection!
Organism; Herpessimplexvirus type | (DNA core
virus)secretedin salivaof 2"/"of healthy
population
Age: 15-30 years; usuallymales
Predisposed: immunosuppressedpatient
. historyof recentexposureto sexualpartnerswith
herpeticlesionson lips/ buccalmucosa
. flulikeprodromeof 3-10 days (headaches, fever,
sore throat,upper respiratorysymptoms,myalgia)
. severeacutedysphagia/ odynophagia
May be associatedwith: oropharyngealherpetic
lesions/ oropharyngeal
Location:
midesopharr:iH:iXliLr'"in
bronchus)
r/ initiatty
vesicles/ blistersthat subsequently rupture
r/ multiplesmalldiscretesuperficialpunctateI roundI
linear/ serpentine/ stellate(often"diamond-
shaped")ulcerssurroundedby radiolucenthalosof
edematousmucosa(in >50%)
./ interveningmucosanormal(withoutplaques)
r/ multipleplaquelikelesions(onlywith severe.
infection)
Dx: risingserumtiterfor HSV type l, viralculture,
biopsy(immunofluorescent stainingfor HSV
antigen,demonstration of intranuclear
inclusions)
Rx: oral lintravenousacyclovir
Prognosis; resolutionof symptomsin 3-14 days
DDx: drug-induced esophagitis, Crohndisease,
esophagealintramuralpseudodiverticulosis
Human lmmunodeficiency Virus Esophagitis
. maculopapularrash + ulcersof soft palate
(occasionally)
. recefltseroconversion / knownAIDS
^/ one / moregiant(>1 cm) flat ovoid/ diamond-
shapedulcers(at time of seroconversion)
indistinguishable
from CMV esophagitis
Dx: per exclusion(brushings, biopsies,cultures
negativefor CMV)
Rx: oral steroids
DDx: CMV esophagitis,mycobacterialesophagitis,
actinomycosis, potassiumchloride,quinidine,
causticingestion,nasogastric intubation,
radiationtherapy,endoscopicsclerotherapy
FAMILIAL ADENOMATOUS POLYPOSIS
= FAMILIAL MULTIPLE POLYPOSIS
= autosomaldominantdiseasewith 80% penetrance
(genefor familialpolyposislocalizedon chromosome5);
sporadicoccurrencein 1/3
Gastrointestinal
Disorders 823
lncidence: 1:7,000to 1:24,000livebirths
Histo: tubular/ villotubularadenomatouspolyps;usually
about1,000adenomas
Age: polypsappeararoundpuberty
. familyhistoryof colonicpolyps(66%)
0 Screeningof familymembersafterpuberty!
. clinicalsymptomsbeginduring3rd-4thdecade(range
5-55 years)
. vagueabdominalpain,weightloss
. diarrhea,bloodystools
. protein-losing enteropathy(occasionally)
Associatedwith: (1) Hamartomasof stomachin 4g%
(2) Adenomasof duodenumin 25%
(3) Periampullary carcinoma
r/ "carpetof polyps"= myriadof 2-3 mm (up to 2 cm)
polypoidlesions
@ Colon(100%): more numerousin distalcolon;always
affectingrectum
r/ normalhaustralpattern
@ Stomach(5%)
@ Smallbowel(.5%)
Cx: malignanttransformation: colon> stomach> small
bowel (in 12% by 5 years;in 3A"/"by 10 years;in
100%by 20 yearsafterdiagnosis;age at
carcinomatous developmentusually2040 years;
multiplecarcinomasin 48%)
0 Periampullarycarcinomais the most common
cause of death after prophylacticcolectomy!
Rx: prophylactictotal colectomyin late teensI early
twentiesbeforesymptomsdevelop+
(1) Permanentileostomy
(2) Continentendorectalpull-through pouch
(3) Kockpouch(= distalileumformedintoa one-
way valve by invaginatingthe bowel at skin site)
DDx: other polyposes,lymphoidhyperplasia,
lymphosarcoma, ulcerativecolitiswith inflammatory
pseudopolyps
GALLSTONE
ILEUS
lncidence: 0.4-5/" of all intestinalobstructions(20% of
obstructionin patients>65 years; 24./"ot
obstructions in patients>70 years);develops
in <1"/"of patientswith cholelithiasis; in 1 of
6 perforations; riskincreaseswith age
Etiology: biliarydisease(90%),pepticulcerdisease,
cancer,trauma
Age: average65-75 years; M:F = 1:4 -1:7
. previoushistoryof gallbladder disease
. intermittent episodesof acutecolickyabdominalpain
(20-30%)
. nausea,vomiting,fever,distension,obstipation
r/ ni gl er tri ad on pl ai nfi l m(i n 10% ):
1. Partial/ completeintestinalobstruction(usuallysmall
bowel),"stringof rosarybeads"= multiplesmall
amountsof air trappedbetweendilated+ stretched
valvulaeconniventes(in 86%)
2. Gas in biliarytree (in 69%)
3. Ectopiccalcifiedgallstone(in 25%): stonesare
commonl y> 2.5cm i n di ameter
r/ changein positionof previouslyidentifiedgallstone
824 Radiology Review Manual
U G I/ B E :
{ well-containedlocalizedbariumcollectionlateralto
firstportionof duodenum(barium-filled collapsedGB
+ possiblybiliaryducts)
F istulous communication :
Cholecystoduodenal (60%),choledochoduodenal,
cholecystocol ic, choledochocolic, cholecystogastric
{ identification of site of obstruction:terminalileum
(60-70h), proximalileum(25/"), distalileum(10%),
pylorus,sigmoid,duodenum(Bouveretsyndrome)
Cx: recurrentgallstoneileusin t10% (additional
calculimoreproximally)
Prognosis; highmortality
PARAGANGLIOMA
GANGLIOCYTIC
= r?fe benigntumorof the Gl tract
Frequency; <100 cases reported
Origin: pancreaticendocrinerest that remainedwhen
the ventralprimordiumrotatedaroundthe
duode n u m
Age: 50-60 years of age; [/;P = 2:1
Location: almostexclusivelyin 2nd portionof duodenum
nearthe ampullaof Vateron the medial/
lateralwall of duodenum
. Gl hemorrhage, abdominalpain
r/ polypoidsmooth-surfaced intraluminalmass
{ homogeneously enhancingmural/ extrinsicsolidmass
of soft-tissueattenuation
r/ well-circumscribed hypoechoicmasscontiguouswith
bowel
r/ no biliaryduct dilatation
DDx: adenocarcinoma (biliaryduct dilatation,
hypovascular),leiomyosarcoma(cysticinternal
hemorrhageI necrosis),hemangioma, duplication
cyst, choledochalcyst, lipoma,hamartoma,
inflammatory fibroidpolyp(distalsmallbowel),
lymphoma(isolatedin stomachand ileum)
GARDNER
SYNDROME
= autosomaldominantdisease(? variantof familial
polyposis)characterizedby a triad of
(1) colonicpolyposis
(2) osteomas
(3) soft-tissuetumors
Cause: adenomatouspolyposisgene on chromosome
5-q21;in 20/" new mutations
0 Familialpolyposis+ Gardnersyndromemay
occurin the samefamily!
Histo: adenomatouspolyps
Age: 15-30 years (2 months- 70 years)
Associatedwith: ? MEA complex
(1) periampullary / duodenalcarcinoma(12%\
(2) papillarythyroidcarcinoma(oftenmulticentric)
(3) adrenaladenoma/ carcinoma
(4) parathyroidadenoma
(5) pituitarychromophobeadenoma
(6) carcinoid,adenomaof smallbowel
(7) retroperitoneal leiomyoma
0 Extraintestinal manifestations occurusuallyearlierthan
in intestinalpolyposis!
. skin pigmentation
. crampingabdominalpain
. weightloss,diarrhea
@ Polyposis
Location:colon('100%),stomach(5-68%),duodenum
(90%),smallbowel(<5%)
r/ multiplecolonicpolypsappearingduringpuberty,
increasingin numberduring3rd-4thdecade
silent r/ lymphoidhyperplasia of terminalileum
r/ hamartomasof stomach
{ intussusception
Cx: small bowel/ colonicobstruction
@ Soft-tissuetumors
(a) sebaceous/ epidermoidinclusioncysts(scalp,
back,face, extremities)
(b) fibroma,lipoma,leiomyoma,neurofibroma
(c) desmoidtumors(3-29%); peritonealadhesions
(desmoplastic tendency);mesentericfibrosis,
retroperitoneal fibrosis
. urinarytract obstruction
(d) mammaryfibromatosis
(e) markedkeloidformation,hypertrophiedscars
(anteriorabdominalwall)arise1-3 yearsafter
surgery
@ Osteomatosisof membranousbone (50%)
Location: calvarium,mandible(81%),maxilla,ribs,
long bones
@ Longbones
rl localizedwavy corticalthickening/ exostoses
r/ slightshortening+ bowing
@ Teeth
{ odontoma,unerupted/ supernumerary teeth,
hypercementosis
{ tendencytoward numerouscaries(dentalprosthesis
at early age)
Cx: malignanttransformation of colonicpolypsin 100%
(averageage at death is 41 years if untreated)
Prophylaxis; gastrointestinal surveillance,thyroid
screening,ophthalmologic evaluationfor
retinalpigmentation anomalies;screening
of familymembersstartingat age 15
Rx: prophylactictotal colectomyat about20 years of
age
GASTRICCARCINOMA
0 3rd most commonGl malignancyaftercolorectal
+ pancreaticcancer,6th leadingcause of cancerdeaths
Prevalence; declining;24,000cases/yearin USA
Riskfactors;smoking,nitrites,nitrates,pickledvegetables
Predisposing factors:
H. pylorigastritis,chronicatrophicgastritis,
adenomatous+ villouspolyp(7-27% are malignant),
gastrojejunostomy, partialgastrectomy(Billrothll >
Billrothl), perniciousanemia(riskfactorof 2), M6n6trier
disease(?)
Histo: adenocarcinoma(95%);rarelysquamouscell
carcinoma/ adenoacanthoma
 Gastrointestinal
Disorders 825

Staging: { wall thickness>6 mm with gas distension+ 13 mm

T1 tumorlimitedto mucosa/ submucosa with positivecontrastmaterialdistension:
T2 tumorinvolvesmuscle/ serosa ./ diffuselow attenuationin mucinouscarcinoma
T3 tumor penetratesthroughserosa ./ increaseddensityin perigastricfat
T4a invasionof adjacentcontiguoustissues ./ enhancementexclusively in linitisplasticatype
T4b invasionof adjacentorgans,diaphragm, i/ nodulesof serosalsurface(= dilatedsurface
abdominalwall lymphatics)
N1 involvement of perigastricnodeswithin3 cm of i/ diameterof esophagusat gastroesophagealjunction
primaryalonggreater/ lessercurvature largerthan adjacentaorta(DDx:hiatalhernia)
N2 involvement of regionalnodes>3 cm from r/ lymphadenopathy below levelof renalpedicle(3%)
primaryalongbranchesof celiacaxis Metastases:
N3 paraaortic,hepatoduodenal, retropancreatic, 1. alongperitonealligaments
mesentericnodes (a) gastrocoliclig.: transversecolon,pancreas
M1 distantmetastases (b) gastrohepatic + hepatoduodenal lig.: liver
Location: mostlydistalthird of stomach+ cardia;60% on 2. locallymph nodes
lessercurvature,10% on greatercurvature; 3. hematogenous:liver(mostcommon),adrenals,
esophagogastric junctionin 30%;transpyloric ovaries,bone (1.8%),lymphangitic carcinomatosis
of
spreadin 5-25/" (for lymphoma40o/") lung (rare)
Probabilityof malignancyof an ulcer: at lessercurvature 4. peritonealseeding:
10-15/", at greatercurvature70"/o,in fundus90% on rectalwall = Blumershelf
Morphology: on ovaries = Krukenberg tumor
1. Polypoid/ fungatingcarcinoma 5. left supraclavicular lymph node = Virchownode
2. Ulcerating/ penetratingcarcinoma(70%) Prognosis:
3. Infiltrating / scirrhouscarcinoma(S-1S%) overallS-yearsurvivalrate of 5-18o/",mean survival
= linitisplastica time of 7-8 months;
Histo: frequentlysignetring cell type + increase - 85% S-yearsurvivalin stage Tl
in fibroustissue - 52% S-yearsurvivalin stage T2
Location: antrum,fundus+ body (38%) - 47% S-yearsurvivalin stage T3
r/ firmness,rigidity,reducedcapaiity of stomach, - 17o/o S-yearsurvivalin stageN1-2
aperistalsisin involvedarea 5% S-yearsurvivalin stage N3
r/ granular/ polypoidfoldswith encirclinggroMh
4. Superficial spreadingcarcinoma EarlyGastricCancer(20%)
= confinedto mucosa/ submucosa;S-yearsurvival = invasionlimitedto mucosa+ submucosa(T1 lesion)
of 90% regardlessof lymphnode involvement
r/ patch of nodularity Classificationof Japan Research Society for Gastric
r/ tittteloss of elasticity Cancer:
5. Advancedbulkycarcinoma Type I protrudedtype = >5 mm in heightwith
. Gl bleeding,abdominalpain,weightloss protrusioninto gastriclumen (10-20%)
UGI:
Type ll superficialtype = <5 mm in height
r/ rigidity
lla slightlyelevatedsurface(10-20%)
! fillingdefect
llb flat / almostunrecognizable (2/")
r/ amputationof folds+ ulceration+ stenosis
llc slightlydepressedsurface(50-00%)
r/ miliary/ punctatecalcifications(mucinous
adenocarcinoma) Type lll excavated/ ulceratedtype (5-10%)
CT:
{ irregularnodularluminalsurface Advanced Gastric Cancer (T2 lesionand higher)
{ asymmetricthickeningof folds Bormann clas sification:
mass of uniformdensity lvarying attenuation Type 1 broad-basedelevatedpolypoidlesion
Type 2 elevatedlesion+ ulceration+ well-
demarcatedmargin
PrognosticParametersof GastricCarcinoma Type 3 elevatedlesion+ ulceration+ ill-defined
margrn
Tumor Metastases Limited to S-Year
Size Submucosa Survival Rate Type 4 ill-defined
flat lesion
Type 5 unclassified,no apparentelevation
1 cm 11% 87%
2 cm 25Yo 70% O7%
3 cm 45/" gS%
gg% GA S TR IC D IV E R TIC U LU M
4 cm 59% 60%
>4 cm 72/" stomachis leastcommonsite of diverticula
33%
lncidence; 1:600-2,400of UGI studies
826 Radiology Review Manual

Etiology: (a) traction secondaryto scarring/ periantral DDx: (1) M6n6trierdisease(antrumspared)

= true diverticulum
inflammation polyp(peripheral
(2) Eosinophilic linitis
eosinophilia,
(b) pulsion(lesscommon)= falsediverticulum plasticaappearance,small bowelchanges)
Age: beyond40 years (3) Lymphoma
Often associatedwith: aberrantpancreasin antral location (4) Carcinoma
Location: juxtacardiacon posteriorwall (75%),prepyloric
(15-22%),greatercurve (3%)
r/ pliability+ varyingdegreesof distension GASTRICULCER
{ nO mass,edemaor rigidityof adjacentfolds BenignGastricUlcer
DDx: small ulcer in intramural-extramucosal mass 95% of all gastriculcers
Cause:
GASTRICPOLYP A. HORMONAL
lncidence: 1.5-5/", most commonbenigngastrictumor syndrome
1. Zol l i nger-E l l i son
Associatedwith: hyperacidity+ ulcers,chronicatrophic 2. Hyperparathyroidism (in 13-2a%)
gastritis,gastriccarcinoma duodenum:stomach = 4' .1i M:F = 3: 1
A. NONNEOPLASTIC 0 Duodenalulcerspredominate in femalesl
1. Inflammatory polyp of stomach (75-90%) 0 Gastriculcerspredominate in males!
= HYPERPLASTIC POLYP= REGENERATIVE POLYP . absenceof gastrichypersecretion
Histo: cysticallydilatedglandslinedby gastric 3. Steroid-induced ulcer
epithelium+ acuteand chronic gastric> duodenallocation;
inflammatory infiltrates in laminapropria frequentlymultiple+ deep ulcers;
Associatedwith: chronicatrophicgastritis, commonlyassociatedwith erosions
p e rn rc ro uasn e m l a . bleeding(in 1/3)
Location: predominantly in fundus+ body; 4. Stress,severeprolongedillness
usuallymultiple 5. Cerebraldisease= Cushingulcer
r/ sharplydelineatedpolypwith smoothcircular 6. Curlingulcer(burns)(in 0.09-2.6%)
border 7. Retainedgastricantrum
r/ "Mexicanhat sign" = stalkseen en face overlying 8. U remi a
the head of polyp B . IN FLA MMA TION
{ sessile/ pedunculated 1. P epti cul cerdi sease
r/ usually<2 cm in diameterwithoutprogression 2. Gastritis
r/ no contourdefectof stomach 3. Radiation-induced ulcer
Prognosisj no malignantpotential 4. lntubation
2. Hamartomatous polyp of stomach (rare) 5. Stasisulcerproximalto pyloric/ duodenal
Histo: denselypackedgastricglands+ bundles obstruction
of smoothmuscle C . B E N IGNMA S S
Associatedwith: Peutz-Jegherssyndrome 1. Lei omyoma
r/ sessile/ pedunculated 2. Granulomatous disease
r/ usually<2cm in diameter 3. Pseudolymphoma (lymphoidhyperplasia)
3. Retention polyp of stomach (rare) D. DRUGS
Histo: dilatedcysticglands+ stroma ASA: greatercurvature
Associatedwith: Cronkhite-Canadasyndrome Pathophysiology:
B. NEOPLASTIC disruptedmucosalbarrier(Helicobacter pylori)with
1. Adenomatous polyp of stomach (10-20%) vulnerability to acid+ secretionof largevolumeof
= true neoplasmwith malignantpotential gastricjuicecontaininglittleacid
(10-80%,increasingwith size) lncidence; 5:10,000;100,000/year (UnitedStates)
Age: increasingincidencewith age; M:F = 2:1 Age peak; 55-65 years; M:F = 1:1
Histo: intestinalmetaplasia(common)+ marked Multiplicity:
cellularatypism (a) multiplein 2-8/" (17-24%at autopsy),especially
Associatedwith: Gardnersyndrome;coexistent in patientson aspirin
with gastriccarcinomain35h
Location: morecommonlyin antrum(antrum
sparedin Gardnersyndrome) GastricUlcer
r/ broad-based elliptical/ mushroom-shaped polyp
Sign Benign Malignant
+ pedicle;usuallysolitary
{ usually>2 cm in diameter(in 80%) Crater round,ovoid irregular
r/ smooth/ irregularlobulatedcontour Radiatingfolds symmetric nodular,clubbed,{used
2. Villous polyp of stomach (rare) Areae gastricae preserved destroyed
{ trabeculated / lobulatedslightlyirregularcontour Projection outsidelumen insidelumen
Cx: malignanttransformation Ulcermound smooth rollededge

(b) coexistentduodenalulcerin 5-64/";
gastric:duodenal = 1:3 (adults)= 1:7 (children)
. abdominalpain: in 30% at night,in 25/" precipitated
by food
Location: lessercurvatureat junctionof corpus
+ antrumwithin7 cm from pylorus;proximal
half of stomachin older patients(geriatric
ulcer);adjacentto GE junctionwithinhiatal
he rn i a
{ ulcersize usually<2 cm (range1-250 mm);in 4/o
>40 mm
^/ round/ ovoidi linearshape
{ Haudekniche= conical/collarbutton-shaped barium
coflectionprojectingoutsidegastric contour (profife
view)
r/ Hamptonline= 1-mmthin straightlucentline
traversingthe orificeof the ulcerniche(seenon
profileview + with littlegastricdistension)= ledgeof
touchingoverhanging gastricmucosaof undermined
benignulcer
r/ ulcercollar= Srnooththick lucentband interposed
betweenthe nicheand gastriclumen (thickenedrim of
, edematousgastricwall) in well-distendedstomach
! ulcer mound = srT'looth, sharplydelineated,gently
slopingextensivetissuemasssurrounding a benign
ulcer(edema+ lackof wall distensibility) in well-
distendedstomach
{ ulcercrater= foUfld/ oval bariumcollectionwith
smoothborderon dependentside (en face view)
r/ nalo defect= wid€ lucentband symmetrically
surroundingulcer resemblingextensiveulcer mound
(vieweden face)
{ ring shadow: ulceron nondependentside (en face
view)
r/ radiatingthick folds extendingdirectlyto crateredge
fusingwith the effacedmarginalfold of the ulcercollar
/ haloof ulcermound
./ incisuradefect= srTlooth, deep, narrow,sharp
indentationon greatercurvatureoppositea nicheon
lessercurvatureat / slightlybelowthe levelof the
ulcer (spasticcontractionof circularmusclefibers)
Prognosis; healingin 50% by 3 weeks,in 100% by 6-
8 weeks;slowerhealingin olderpatients;
onlycompletehealingprovesbenignancy
Cx: bleeding,perforation,fistula
0 Most commoncause of gastrocolicfistula!
MalignantGastricUlcer
lncidence: 5/" of ulcersare malignant
Cause:
1. Gastriccarcinoma
2. Lymphoma(2% of all gastricneoplasms)
{ mufiipleulcerswith aneurysmalappearance
3. Leiomyosarcoma, neurogenicsarcoma,
f ibrosarcoma,liposarcoma
4. Metastases
(a) hematogenic:malignantmelanoma,breast
cancer,lungcancer
(b) per continuum:pancreas,colon,kidney
Prognosis; partial healing may occur
Gastrointestinal
Disorders 827
Location: anywherewithinstomach;fundal ulcers
above levelof cardiaare usuallymalignant
r/ ulcerlocationwithingastriclumen,ie, not projecting
, beyond expectedmarginof stomach (profileview)
ri eccentricallylocatedulcerwithinthe tumor
r/ irregularlyshapedulcer
r/ shallowulcerwith width greaterthan depth
r/ nodularulcerfloor
r/ abrupttransitionbetweennormalmucosa
+ abnormaltissueat some distance(usually24 cm)
from ulceredge
r/ rolled/ rounded/ shoulderededgessurrounding
ulcer
{ nodularirregularfoldsapproachingulcerwith fused/
clubbed/ amputatedtips
{ rigidityllack of distensibility
r/ associatedlarge irregularmass
r/ Carman meniscus sign = curvilinearlens-shaped
intraluminalform of craterwith convexityof crescent
towardgastricwall and concavitytowardgastric
lumen(profileview,usuallyundercompression)
foundin specifictype of ulceratingcarcinoma,seen
only infrequently;wall aspectcan also be concavei
flat
r/ firnin meniscus complex = Carmansign
(appearanceof crater)+ radiolucentslightlyelevated
rolledborder
GASTRIC
VARICES
Cause: portalhypertension(varicesseen in 2-78%);
splenicvein obstruction(frompancreatitis,
pancreaticcarcinoma,pseudocyst)
Location:(a) esophagogastric junction(mostcommon)
(b) along lessercurvature(in 11-75"/"of
patientswith portalhypertensioni cirrhosis)
Feedingvessels:
1. Leftgastricvein (betweensplenicvein + stomach)
2. Shortgastricveins(betweenspleen+ fundus)
3. Retrogastric vein (betweensplenicvein
+ esophagogastric junction)
. increasedprevalenceof portosystemicencephalopathy
r/ bariumstudy: 65-89% rate of detection:
r/ lobulatedfolds/ polypoidmassesin fundus
r/ endoscopy:most practicalmethod
{ splenicportography
r/ hepatofugalbloodflow along SMV into left gastric
+ splenicvein
Cx: varicealbleedingin 3-10-36%
0 Gastricvaricesbleed less frequentlybut more
severelythan esophagealvarices!
GASTRIC
VOLVULUS
= abnormaldegreeof rotationof one part of stomach
aroundanotherpart,usuallyrequires>180otwistingto
producecompleteobstruction
Etiology: (a) abnormalityof suspensoryligaments
(hepatic,splenic,colic,phrenic)
(b) unusuallylonggastrohepatic + gastrocolic
mesenteries
828 Radiology Review Manual
Usuallyassociatedwith: diaphragmaticabnormality:
1. Paraesophageal hiatusherniain 33%
2. Eventration
Types:
A. ORGANOAXIALVOLVULUS
rotationarounda lineextendingfrom cardiato
pylorus
B. MESENTEROAXIAL VOLVULUS
rotationaroundan axis extendingfrom lesserto
greatercurvature
. severeepigastricpain
. vigorousattemptsto vomit withoutresults
. inabilityto pass tube into stomach
{ massivelydistendedstomachin LUQ extendinginto
chest
r/ incomplete / absententranceof bariumintostomach
r/ bariumdemonstratesarea of twist
Cx: intramuralemphysema,perforation
DDx: gastricatony,acutegastricdilatation,pyloric
obstruction
GASTRITIS
GorrosiveGastritis
Agents:
(a) acid, formaldehyde
. clinicallyusuallysilent
Location: esophagususuallyunharmed,severe
gastricdamage,duodenummay be
involved(newerpotentmaterials
cause atypicaldistribution)
(b) alkaline
Location: pylorus+ antrummost frequently
involved
A. ACUTECHANGES(edema+ mucosalsloughing)
r/ markedenlargementof gastricrugae+ erosions/
ulceration
r/ completecessationof motoractivity
r/ gas in portalvenoussystem
Cx: perforation
B , CHRO NI CC H AN G E S
r/ firm thicknonpliable wall
r/ stenotici incontinent pylorus(if involved)
! gastricoutletobstruction(cicatrization) after
3-10 weeks
Emphysematous
Gastritis
= r?re but severeform of widespreadphlegmonous
gastritissubsequentto mucosaldisruption
characterizedby gas in wall of stomach
Cause of mucosal disruption:
ingestionof toxic/ corrosivesubstances(37"/"),
alcoholabuse (22"/"),gastroenteritis(157o),recent
abdominalsurgery(15/"), gastricinfarction,
necrotizingenterocolitis,ulcer,acute pancreatitis,
adenocarci noma of stomach,phytobezoar,leukemia,
diabetesmellitus,disseminated strongyloidiasis,
gastricmuormycosis,after ingestionof largeamounts
of carbonatedbeverages
Histo: bacterialinvasionof submucosa+ subserosa
Organism; hemolyticstreptococcus,Clostridium
welchii,Clostridium perfringens, E. coli,
S. aureus,enterobacter, Pseudomonas
aeruginosa
. explosiveonsetof severeabdominalpain
. nausea,diarrhea,chills,fever,leukocytosis
. bloodyfoul-smelling emesist PATHOGNOMONIC
vomitingof a necroticcast of stomach(due to
dissectionalongplaneof muscularismucosae)
CT:
ri gastricwall thickening(DDx:emphysematous
gastritis)
Plainradiographs:
./ innumerable smallgas bubblessilhouetting the
stomachin a mottledfashionwithoutpositional
change
./ thickeningof rugalfolds
{ + portalvenousgas
GI:
{ cobblestone appearanceof mucosaon upperGl
r/ intramuralpenetrationof contrastmaterial
Cx: cicatricialstenosis(21"/"),sinustract formation
Prognosis; 60-80%mortality
Rx: broad-spectrum antibiotics+ intravenousfluids;
emergentsurgeryfor acute perforation
ErosiveGastritis
= HEMORRHAGIC GASTRITIS
lncidence: 0.5-1 0'/. of Gl studies
Etiology (in 50% without causativefactors):
(1) Pepticdisease:emotionalstress,alcohol,acid,
corrosives,severeburns,anti-inflammatory agents
(aspirin,steroids,phenylbutazone, indomethacin)
(2) Infection:herpessimplexvirus,CMV, Candida
(3) Crohndisease:aphthoidulcersidenticalin
appearanceto varioliforrnerosions
Histo: epithelialdefectnot penetratingbeyond
muscularismucosae
. 10-20ohof all Gl hemorrhages (usuallywithout
significant bloodloss)
. vaguedyspepsia,ulcerlikesymptoms
Location: antrum,rarelyextendingintofundus;aligned
on surfaceof gastricrugalfolds
r/ varioliform completeerosion(95%)= tiny fleckof
bariumsurroundedby radiolucent halo ("target
lesion")<5 mm, usuallymultiple
{ incompleteerosion(5%)= linearstreaks/ dots of
bariumwithoutsurroundingmoundof edema/
inflammation
{ nodularity / scallopingof prominentantralfolds
r/ contiguousduodenaldiseasemay be present
poor peristalsisI alony,delayed
^/ timiteddistensibility,
gastricemptying
Gastritis
Phlegmonous
Etiology: septicemia,localabscess,postoperative
stomach,complicationof gastriculcer/
cancer
Organism: Streptococcus

Path: multiplegastricwall abscesses,which may GLYCOGEN
communicate with lumen
. severefulminatingillness
. patientmay vomit pus
Location: usuallylimitedto stomachnot extending
beyondpylorus;submucosais the most
severelyaffectedgastriclayer
r/ bariumdissectionintosubmucosa+ serosa
GIARDIASIS
= ovefgfowthof commensalparasiteGiardialamblia
Organism:
Giardialamblia(flagellated protozoan); oftenharmless
contaminant of duodenum+ jejunumin motileform
(= trophozoite)attachedto mucosaby suctiondisk,
nonmotileform (= cyst) shed in feces;capableof
pathogenicbehaviorwith invasionof gut wall
lncidence: 1.5-2% of populationin UnitedStates,
infests4-16% of inhabitantsof tropical
countries,foundin3-20/. of childrenrn
partsof southernUnitedStates
Predisposed: alteredimmunemechanism
(dysgammaglobulinemia, nodular
lymphoidhyperplasia of ileum) GRAFT.VERSUS.HOST
Histo: bluntedvilli (may be misdiagnosedas celiac
diseaseespeciallyin children),cellularinfiltrateof
acute + chronicinflammationin laminapropria
. abdominalpain,weightloss,failureto thrive(especially
in c hildr en)
. spectrumfrom asymptomaticto severedebilitating
diarrhea,steatorrhea(relatedto numberof organisms)
. reducedfat absorption(simulating celiacdisease)
Location: most pronouncedin duodenum+ jejunum
r/ thickeneddistortedmucosalfoldsin duodenum
+ jejunum(mucosaledema)with normalileum
r/ markedspasm + irritabilitywith rapidchangein direction
+ configuration of folds
r/ hypersecretion with blurring+ indistinctness of folds
^/ hyperperistalsis with rapidiransittime
^/ segmentation of barium(frommotilitydisturbance
+ excessintraluminal fluid)
r/ t lympnoidhyperplasia(associatedwith immunoglobulin
deficiencystate)
. Dx: (1) Detectionof Giardialambliacystsin formed
feces or trophozoitesin diarrhealstools
(2) Trophozoitesin duodenalaspirate/ jejunal
biopsy
DDx: Strongyloides/ hookworminfection
Rx: quinacrine(Atabrine@)
GLOMUSTUMOROF STOMACH
0 Mostcommonbenignvasculargastrictumor
Histo: dilatedirregularfyshapedthin-walledvessels
(= modifiedcapillaries)coveredby nests/
strands/ sheetsof glomuscells
. asymptomatic / upperGl bleeding
Location: gastricantrum
r/ singlesmoothsubmucosalmass+ ulceration
r/ tiny flecksof calcification(occasionally)
r/ strongenhancementin earlyarterialfhase
GastrointestinalDisorders 829
ACANTHOSIS
= benigndegenerative conditionwith accumulation
of
cellularglycogenwithinsquamousepithelialliningof
esophagus
lncidence.' in up to 15% of endoscopedpatients
Etiology: unknown
Age: middle-aged / elderlyindividuals
Histo: hyperplasia+ hypertrophyof squamousmucosal
cellssecondaryto increasedglycogen;no
malignantpotential
. asymptomatic
. whiteoval mucosalplaquesof 2-15 mm in diameteron
otherwisenormalappearingmucosa
Location: middle(common)/ upperesophagus,
in randomdistribution
{ multiple1-3-mm roundednodules/ plaques
Dx: biopsy
DDx: candidaesophagitis(lesionsdisappearunder
treatmentin contrastto glycogenacanthosis),reflux
esophagitis
DISEASE
= T lymphocytesfrom donor bone marrowcause selected
epithelialdamageof recipienttargetorgans
Bone marrowtransplantation for treatmentof:
leukemia,lymphoma,aplasticanemia,immunologic
deficit,metabolicdisordersof hematopoieticsystem,
some metastaticdisease
lncidence: 30-7A% of patientswith allogeneic(= donor
geneticallydifferentfrom host)transplant
Targetorgans: Gl tract(smallbowel),skin,liver
@ S ki n
. maculopapular rashon face,trunk,extremities
@ Liver
. elevationof hepaticenzymes+ liverfailure
Gf tract
@
. profusesecretorydiarrhea
. abdominalcramping,fever,nausea,vomiting
Path: severemucosalatrophy/ destruction
^/ shaggyfold thickening
r/ "ribbonbowel"= srT'l?ll bowelfold effacementwith
tubularappearance(DDx: viralenteritis,ischemia,
celiacdisease,radiation,soybeanallergy)
{ lossof haustration, spasm,edema,ulceiation,
granularmucosalpatternof colon(simulating
ulcerativecolitis)
r/ small bowel"cast"= prolongedcoatingof
abnormalbowelfor hoursto days
ri circularcollectionsof contrastmaterialon cross
section+ paralleltrackson longitudinalsection
./ severelydecreasedtransit time
C T:
./ abnormallyenhancingthin layerof mucosa
diffuselyinvolvingsmall+ largebowel
^/ ftuid-titted
distendedpoorlyopacifiedbowel (oral
contrastmaterialnot given!)
r/ barium(frompreviouscontrastenema)may
becomeincorporatedinto bowelwall
830 Radiology Review Manual

Cx: infectionwith opportunisticorganisms,eg, Candida Precipitatedby: bacteriallviral infection,allergies,insect

albicans,herpesvirus,invasivefungalorganisms, sting,drugs(eg,penicillin, sulfonamides,
CMV, varicella-zoster virus, Epstein-Barrvirus, aspirin),certainfoods
hepatitisviruses,rotavirus,adenovirus,Coxsackie Cause: depositionof lgA-dominant immunecomplexes
virusA and B, P. carinii,pneumococcus in venules,capillaries,and arterioles
Prognosis; fatal in up to 15% (due to opportunistic Age: children(peakage of 5, range3-10 years)+ adults
infections) >20 years (in uP to 30%)
Rx: steroids+ cyclosporine . most frequentmanifestations:
DDx: superinfection with enteroviruses . purpuricskin rashon legs+ extensorsurfaceson arms
. colickyabdominalpain+ Gl bleeding
. microscopichematuria+ proteinuriain 50/" (from
HELICOBACTER PYLORI INFECTION glomerulonephritis
proliferative with lgA deposits
Organism; worldwideGram-negativespiral-shaped demonstratedby immunofluorescence)
bacillus[formerlyCampylobacterpylori] . often beginsas an upperrespiratorytract infection
Prevalence: increasingwith age; >50% of Americans . arthralgiasof largejoints
>60 years of age r/ multifocalbowelwall thickening(due to intramural
Path: surfaceepithelialdamage+ inflammationwith hemorrhage+ edema)
mucosalinfiltration by neutrophils,plasmacells, Cx: (1) Bowel infarct/ perforation/ irreducible
and lymphoidnodules intussusception (3-5%)
Location: gastric antrum > proximalhalf of stomach (2) Renalinsufficiency(10-207o),end-stagerenal
Site: beneathmucuslayeron surfaceepithelialcells disease(5%)
. asymptomatic(vast majority) Rx: high doses of corticosteroids + azathioprine
. dyspepsia,epigastricpain
{ gastritis(75% prevalenceof H. pylori): H E R N IA
r/ thickenedgastricfolds Prevalence: 10% of SBO; 2nd most commoncause of
{ polypoidgastritismimickingmalignanttumor smallbowelobstruction!
{ enlargedareae gastricae Namenclature: indicatesanatomicsite of its orifice
r/ gastriculcer (60-80% prevalenceof H. pylori)
r/ duodenalulcer(90-100%prevalenceof H. pylori) ExternalHernia
Dx: (1) Endoscopicbrushings+ biopsy = bowelextendingoutsidethe abdominalcavity
(2) Breathtest measuringureaseactivityafter lncidence: 95o/oof all hernias
ingestionof carbon-14-labeled urea Location:
(3) Serologictest for lgG antibodies @ Ventral
Rx: tripletherapy(= bismuth+ metronidazole 1. PostoPerative hernia
+ tetracycline/ amoxicillin)resultsin 95% cure rate 2. Trocarsite hernia
alter 2 weeks of therapy lncidence: 1-3.6%
r/ often Richtertype hernia
2. Umbilicalhernia
HEMANGIOMA OF SMALL BOWEL 3. Epigastrichernia
lncidence: 7-10"/" of all benignsmall boweltumors 4. Spigelianhernia
lncreasedincidencein: Turner syndrome,tuberous Frequency: 2o/oof anteriorabdominalhernias
sclerosis,Osler-Weber-Rendu = acquiredventrolateralherniathroughdefect
disease in aponeurosisbetweentransverseand
. symptomatic(80%): rectusmuscleof abdomenat junctionof
. acute intermittentseverebleeding(melena) semilunar+ arcuatelinesbelowumbilicus
. acute/ chroniclife-threatening anemia ^/ herniasac dissectslaterallyto rectus
. intestinalobstruction,intussusception,perforation abdominismusclethrougha fibrousgroove
Path: submucosalsoft infiltrativepolypoidmass (= semicircular/ spigelianline)
Location: jejunum(55%),ileum(42%),duodenum(2%) r/ herniasac lies beneathan intactexternal
{ muftiplesessilecompressible fillingdefects
intraluminal obliqueaPoneurosis
{ nodularsegmentalmucosalabnormality @ Diaphragm
./ t phtenolithsin intestinatwalt 1. Bochdalekhernia
CT : 2. Morgagnihernia
r/ well-circumscribedlobulatedmass of mixed @ Lumbar
attenuationsuppliedby largeartery 1. Grynfelt(uppe|
2. Petitlumbartriangle(lower)
@ Pelvicfloor
PURPU
HENOCH-SCHONLEIN RA 1. Obturatorforamen
= rnostcommonsystemicallergichypersensitivity-related r/ hernia betweenpectineus+ external
acute small-vesselvasculitisin children obturatormuscles
 Gastrointestinal
Disorders 831

2. Sciaticnotch r/ mass effectwith displacementof other abdominal

3. Perinealhernia(rare) organs
(a) anteriorperinealhernia= defectof Cx: volvulus
urogenitaldiaphragmanteriorto superficial
transverseperinealm. + lateralto Paraduodenal Hernia (59%)
bulbocavernosus m. + medialto = coog€hitaldefectin descendingmesocolon
ischiocavernosus m. (onlyin females) . frequentlyasymptomatic
(b) posteriorperinealhernia= defectin levator (a) LEFT throughfossa of Landzert(75%)
ani m. / betweenlevatorani m. and Location:
coccygeusm. posteriorto superficial to the left of 4th portionof duodenumat
transverseperinealm. duodenujejunal junction(paraduodenal fossa
r/ defecatingproctography = confluentzone of descendingmesocolon
@ Groin + transversemesocolon+ smallbowel
1. I ngu i n ahl e rn i a mesentery
(a) directinguinalhernia r/ clusterof dilatedsmall-bowelloopsbetween
= defectin Hesselbach triangle(bounded pancreas+ stomach
by inguinalligamentinferiorly, inferior ^/ displacementof gastricwall anteriorly
epigastricarterysuperolaterally, fused ^/ displacement of duodenojejunal flexure
aponeuroses of internaloblique + transversecolon inferiorly
+ transverseabdominalmusclesmedially) r/ engorgedcrowdedvesselsat entranceof hernia
{ medialto inferiorepigastricvessels sac
r/ herniacontainsbowel,mesentericfat, C T:
vessels r/ encapsulated bowelloopdisplacingthe inferior
(b) indirectinguinalhernia mesentericvein (= landmarkof the rightmargin
r/ lateralto inferiorepigastricvessels of the descendingmesocolon)anterolaterally
originating at deep inguinalring (b) RIGHTthroughmesentericoparietal fossa of
2. Femoralhernia Waldeyer(25%)
r/ medialto femoralvein withinfemoralcanal Predisposed; nonrotationof small bowel
Cx: high probabilityof incarceration Location: behindthe rootof smallbowel
3. Richter hernia = entrapmentof antimesenteric mesenterycaudalto SMA and
borderof bowelin herniaorifice,usuallyseen inferiorto 3rd portionof duodenumon
in olderwomenwith femoralhernias rightside
. no palpablemass = difficultto diagnose
CT:
r/ partialobstructionwith patentOowLtlumen r/ encapsulatedbowel loop displacingthe right
colicvein (= landmarkof left marginof the
InternalHernia ascendingmesocolon)anteriorly
= herniationof bowelthrougha developmental/ { loopingof smallintestinebehindSMA + SMV
surgicallycreateddefectof the peritoneum, omentum, belowtransverseportionof duodenum
mesenteryor throughan adhesiveband 'V StvtVlocatedventral
+ to left of SMA
lncidence: 5/" of all hernias,responsiblelor <j"/o of r/ absenceof normalhorizontalduodenum
mechanicalsmallbowelobstruction Cx: partial/completeobstruction of smallintestine
Classificationof internal hernias: (in 50%)
(a) retroperitoneal: usuallycongenitalcontaininga
hernialsac Lesser Sac Hernia (<10%)
1. paraduodenal (ligamentof Treitz) throughforamenof Winslowin retrogastric location
2. foramenof Winslow lnvaginated gut:
3. intersigmoid ileum> jejunum,cecum,appendix,ascending
4. pericecal/ ifeocolic colon, Meckeldiverticulum,gallbladder,greater
5. supravesical omentum
(b) anteperitoneal: ^/ gas-containingbowelloopsin centerof upper
smallgroupof herniaswithouta peritonealsac abdomen
1. transmesenteric (transverse
i sigmoid ^/ distendedsmall bowel loopsoccupyingspace
mesocolon) betweenstomach+ liver
2. transomental
3. pelvic(includingbroadligament)
. intermittent nausea,abdominalpain,distension Iatrogenic Hernia
(madeworse by eating+ standingand relievedby TnarusnnesENTERrc Henua
fasting+ assuminga recumbentposition) Cause: fenestrationof transversemesocolonin
r/ signsof bowelobstruction(onlyduringsymptomatic constructionof Roux-en-Yloop
period) r/ clusterof small-bowelloops (70%)
832 Radiology Review Manual

{ clusteredsmallboweloutsidecoloncompressed Totally lntrathoracic Stomach

againstabdominalwallwithoutoverlying
omental = defectin centraltendonof diaphragmin
fat (85%) combination with slightvolvulusin transverseaxis
of colon(92%)
{ centraldisplacement of stomachbehindheart
{ displacement trunk(85%)
of mesenteric { cardiamay be intrathoracic (usually)/
{ engorged vessels(85%)
mesenteric subdiaphragmatic
r/ greatgastriccurvatureeitheron right/ left side
HenHnrHRoucH Bnolo Ltcauerur (veryrare)
duringsurgeryor
/ fenestration
afterlaceration Congenitally Short EsoPhagus
duringpregnancy veryrare)
(nottruehernia,
= gastricectopyby lack of lengtheningof esophagus
r/ nonreducible gastricsegment(in erect
intrathoracic
Hiatal Hernia / supineposition)
Associated (25%),reflux
with: diverticulosis { cylindrical/roundintrathoracicsegmentwith large
esophagitis(25%),duodenalulcer sinuousfolds
(2O ), gallstones
(18%) r/ shortstraightesophagus
r/ circularnarrowingat gastroesophageal junction,
Sliding Hiatal Hernia (ee%) frequentlywith ulcer
= AXIALHERNIA = CONCENTRIC HERNIA r/ gastroesophageal reflux
= eSoPh?gogastric junctionremainsin chestwith
portionof peritonealsac formingpart of wall of
hernia UmbilicalHernia
Etiology: ruptureof phrenicoesophageal membrane = protrusion
of abdominal / fatintoanterior
contents
due to repetitivestretchingwith swallowing abdominalwall via umbilicalring
Incidence; increasingwith age Prevalence: 4/" of all hernias; M < F
{ reduciblein erect position Cause: failedclosureof umbilicalring,obesity,
{ epiphrenicbulge= entirevestibule+ sleeveof multiplepregnancies, intraabdominal masses,
stomachare intrathoracic liverfailure,increasedintraabdominal
./ distancebetweenB ring (if visible)and hiatal pressure,weak abdominalwall
margin>2 cm r/ may containfat / small bowel/ colon
r/ peristalsisceasesabove hiatus(end of peristaltic r/ herniationof antimesenteric borderof intestine
wave delineatesesophagogastric junction) (Richterhernia)
{ tortuousesophagushavingan eccentricjunction ^/ Meckeldiverticulum in hernialsac (Littrehernia)
with hernia Cx: strangulation,incarceration
{ numerouscoarsethick gastricfolds within DDx: paraumbilical, spigelian,epigastric,incisional
suprahiatalpouch(>6 longitudinal folds) herni a
{ + gastroesophageal reflux
CT:
^/ dehiscenceof diaphragmatic crura>15 mm H IR S C H S P R U N G D IS E A S E
r/ pseudomasswithin/ above esophagealhiatus = AGANGLIONOSIS OFTHECOLON= AGANGLIONIC
ri increasein fat surroundingdistalesophagus MEGACOLON
(= herniationof omentumthrough = absenceof parasympathetic gangliain muscle
phrenicoesophageal ligament) (Meissnerplexus)+ submucosallayers(Auerbach
DDx: normaltemporarycephaladmotionof plexus)secondaryto an arrestof craniocaudalmigration
esophagogastric junctionby 1-2 cm into chest of neuroblastsalongvagal trunksbefore 12thweek
due to contraction muscleduring
of longitudinal leadingto relaxationfailureof the aganglionic segment
esophagealperistalsis lncidence: 1:5,000-8,000live births;15-20% of all
neonatalbowelobstructions; usuallysporadic;
fami l i ali n 4/"
ParaesophagealHernia 1lx1 Age: duringfirst 6 weeks of life of a full-terminfant
= ROLLING HIATAL HERNIA = PARAHIATALHERNIA (70-80%); M:F = 4-l9i; extremelYrare in
= portionof stomachsuperiorlydisplacedinto thorax prematureinfants
with esophagogastric junctionremainingin Associatedwith: trisomY21 (in 2%)
subdiaphragmatic position Location: at varyingdistancesproximalto anus,
r/ cardiain normalposition usuallYrectosigmoid (in 80%)
r/ herniationof portionof stomachanteriorto (a) ultrashortsegment(= in1sl.alsphincter)..(veryrare)
esophagus (b) shortsegmentdisease ....(80%)
{ frequentlynonreducible (c) longsegmentdisease .....(15%)
{ may be associatedwith gastriculcerof lesser (d)totalcol oni cagangl i onosi........
s . . . . . . ( 5%)
curvatureat levelof diaphragmatichiatus (e) skip aganglionosis = sparingof rectum....(veryrare)
 Gastrointestinal
Disorders 833

. failureto pass meconiumwithinfirst 48 hoursof life . palpableolive-shapedmass (80% sensitivein

. intermittentconstipation+ paradoxicaldiarrhea(25%)
experiencedhands,up to 14o/otalse positive)
. biliousvomiting,abdominaldistention . nasogastricaspirate>10 mL (92% sensitive,86yo
. rectalmanometrywith absenceof spike activity
specific)
r/ generalizedgaseousdistentionof bowel loops UGI (95%sensitivity):
BE: Precautions:(1) emptystomachvia nasogastric tube
r/ short patentcolon usuallyof normalcaliber beforestudy
r/ invertedcone shape at transitionbetweenabnormal (2) removecontrastat end of study
+ normalbowel(MOSTCHARACTERTSTTC): r/ elongation+ narrowingof pyloriccanal (2-4 cm in
{ "transition zone"= aganglionic segmentappearing l ength):
normalin size (seenin 50% during1st week of life) r/ "double/ tripletrack sign" = crowdingof mucosal
{ dilatationof large+ small bowelaborallyfrom folds in pyloricchannel
transitionzone r/ "stringsign"= passingof smallbariumstreak
{ normal-appearing rectumin 33% throughpyloricchannel
r/ markedretentionof bariumon delayed ! Twiningrecess= "diamondsign"= transient
postevacuation films after 1Z-24 hours triangulartentlikecleft lniche in midportionof
^/ t o-t 5-cm segmentof persistentcorrugated/ pyloriccanalwith apex pointinginferiorly
convolutedrectum(= abnormaluncoordinated secondaryto mucosalbulgingbetweentwo
contractionsof the aganglionicportionof colon)in separatedhypertrophiedmusclebundleson the
31% (DDx:colitis,milkallergy,normalintermittent greatercurvatureside withinpyloricchannel
spasm of rectum) r/ abnormalconfiguration of antrum:
N.B.: avoiddigitalexami cleansingenemapriorto r/ "pyloricteat" = outpouchingalong lesser
radiographicstudies! curvaturedue to disruptionof antralperistalsis
O B - US : r/ "antralbeaking"= fi't€lssimpressionupon antrum
r/ dilatedsmall bowel/ dilatedcolon with streakof bariumpointingtowardpyloric
Cx: (1) Necrotizing enterocolitis channel
(2) Cecal perforation(secondaryto stasis, ./ t<irt<tinsign = "mushroomsign"= indentationof
distension,ischemia) baseof bulb (in 50%)
(3) Obstructiveuropathy r/ gastricdistensionwith fluid
Dx: suctionmucosalbiopsyof rectum(increased r/ activegastrichyperperistalsis:
acetyfchofinesteraseactivity) r/ "caterpillarsign" = gastrichyperperistaltic waves
Rx: (1) Swensonpull-through procedure US:
(2) Duhameloperation r/ "targetsign"= hypoechoicring of hypertrophied
(3) Soave procedure pyloricmusclearoundechogenicmucosacentrally
on cross-section
r/ elongatedpyloruswith thickenedmuscle:
HYPERPLASTIC
POLYPOF COLON { pyloricmusclewall thickness>3 mm
= intestinalmetaplasiaconsistingof mucousglandslined
{ pyloricvolume>1.4cm3(= 114rux [maximum
by a singlelayerof columnarepithelium;NO malignant pyloricdiameterl2x pyloriclength);most criteria
potential
independentof contractedor relaxedstate (gg%
Path: infoldingof epitheliumintothe glandularlumen
false negative)
Location: rectosigmoid r/ pyloriclength(mm) + 3.64 x musclethickness
{ smoothroundedsessileelevation (mm)> 25
r/ usually<5 mm in diameter

HYPERTROPHIC PYLORIC STENOSTS

= idiopathichypertrophyand hyperplasiaof circular double / triple
musclefibersof pyloruswith proximalextensioninto track sign
gastricantrum
lncidence; 3:1,000;M:F = 4-S:1
Etiology: inheritedas a dominantpolygenictrait;
increasedincidencein firstbornboys;acquired
ratherthan congenitalcondition

InfantileFormof HypertrophicpyloricStenosis
Age: manifestationbetween2-8 weeks of life
. nonbiliousprojectilevomiting(sourformula/ clear
gastriccontents)with progressionover a periodof beak sisn
severalweeks after birth (15-20%)
. positivefamilyhistory
Hypertrophic Pyloric Stenosis
834 Radiology Review Manual

{ pylorictransversediameter>13 mm with pyloric D E FE C T(l eastcommon)

B . IN TE R ME D | A TE
channelclosed = bowel ends withinlevatormuscleas a resultof
r/ elongatedpyloriccanal>17 mm in length abnormalityin posteriormigrationof rectum
r/ "cervixsign"= indentation
of musclemasson fluid- . fistulaopeninglow in vagina/ vestibule
filledantrumon longitudinal
section Rx: 2- / 3-stageoperation
r/ "antralnipplesign"= redundantpyloricchannel C . H IGHA N OMA LY
mucosaprotrudingintogastricantrum = bowelends above levatorsling; M > F
r/ exaggeratedperistalticwaves . fistulousconnectionto perineum/ vagina/ posterior
r/ delayedgastricemptyingof fluid into duodenum urethra(airin bladderin males;air in vaginain
females)
Cx: hypochloremicmetabolicalkalosis Cx: associatedmalformationsmore common+ more
DDx: severe
1. lnfantilepylorospasm Rx: multiplesurgicalProcedures
r/ musclethicknessbetween1.5 and 2 mm r/ distancebetweenrectalair and skin will not accurately
r/ variablecaliberof antralnarrowing outlinethe extentof atreticrectumand anus (varying
{ antraloeristalsis lengthduringcryingwith increasein abdominalpressure
r/ detayedgastricemptying + contractionof levatorani muscle)
r/ elongationof pylorus US:
Prognosis; resolvesin several days / ? early r/ <t S mm distancebetweenanal dimple+ distalrectal
stage of evolvingpyloricstenosis pouchon transperineal imagesindicateslow lesion
Rx: effectivewith metachlopromide / bentyl OB-US (earliestdetectionby 2O-29weeks GA):
2. Gastritis/ milkallergy . absent/ low disaccharidase levelin amnioticfluid
r/ circumferential/ eccentricthickeningof antral r/ ditatedcolon in lowerpelviswith U- / S-shaped
mucosa>2-3 mm configuration+ intraluminal calcifications
3. Duodenalobstruction from midgutvolvulus r/ normalamnioticfluid(unlessalsoTE fistula)
^/ distendeddescendingduodenum { absenceof anal characteristics (= hypoechoiccircular
{ reversatof SMA and SMV relationship rim with centralechogenicstripe)
r/ whirlpoolsign = twistingof smallbowel
mesenterY COLITIS
INFECTIOUS
4. Gastricdiaphragm Cause:
Y ersi n ia,
S al monel l a,
(1) bacteri um:S hi gel l a,
Adult Formof HypertrophicPyloricStenosis Campylobacter, Staphylococcus, Chlamydia
Cause: secondaryto mild infantileform trachomatis, amebiasis,tuberculosis
. acute obstructivesymptomsuncommon (2) fungus: histoptasmosis, mucormycosis,
. nausea, intermittent vomiting actinomycosis
. postprandialdistress,heartburn (3) virus: herpesvirus, CMV, rotavirus
Associated with: Location:
(1) pepticulcerdisease(in 50-74%) (prolonged diftuseinvolvement = CMV, E. coli
gastrinproductionsecondaryto stasisof food) limitedto rightcolon= Shigella,Salmonella
(2) chronicgastritis(54%) descending+ sigmoidcolon= schistosomiasis
rectosigmoid = gonorrhea,herpesvirus, C'
r/ persistentelongation(2-4 cm) + concentricnarrowing
of pyloricchannel trachomatis(lymphogranuloma venereum)
{ parallel+ preservedmucosalfolds CT:
{ antispasmodics show no effecton narrowing wall thickening+ low attenuation
{ proximalbenignulcer(74/"),usuallynear incisura homogeneous wall enhancement
multipleair-fluidlevels
inflammation of pericolicfat
FocalPyloricHypertrophy ascites
= T O RUSHY PE R P L AS IA Dx: cl i ni cal
= localizedmusclehypertrophyon the lessercurvature
= milderatypicalform of HPS INTESTINALLYMPHANGIECTASIA
^/ flatteningof distallessercurvature LYMPHANGIECTASIA
A. CONGENITAL
= PRIMARY PROTEIN.LOSING ENTEROPATHY
= generalized congenitalmalformationof lymphatic
ANUS
IMPERFORATE systemwith atresiaof the thoracicduct + gross
Prevalence: 1:5,000live births dilatationof smallbowellymphatics; usually
A. LOW ANOMALY(55%) sporadic;may be inherited
= bowel has passedthroughlevatorsling Age: presentationbefore30 Years
. fistulato perineum/ vulva . asymmetricgeneralizedlymphedema(dueto
Rx: readilyreparable protein-losing enteropathywith hypoproteinemia)
 Gastrointestinal
Disorders 835

. chylouspleuraleffusions(45%) ^/ "halo"sign= duodenal

mucosacoversouter+ innerwall
' diarrhea(60%),steatorrhea(20%) of diverticulum
. v om it ing( 1 5 % )
. abdominalpain (15%)+ distension INTRAMURALESOPHAGEALRUPTURE
. decreasedalbumin+ globulin = DISSECTING INTRAMURALHEMATOMA
. lymphocytopenia (90%) = rnUcosdltear with dissectinghemorrhageinto
. decreasedserumfibrinogen,transferrin, submucosaand involvement of venousplexus
ceruloplasmin . hematemesis
B . A CQ UI RE DL YMP H A N GIE C T A SIA r/ intramuralhematomasimulatesretainedsolidmaterial
Causes leading to dilatation of intestinaltymphatics: w i thi nl umen
1. Mesentericadenitis ^/ "mucosalstripesign" = dissectedmucosafloatingwithin
2. Retroperitoneal fibrosis l umen
3. Diffusesmallbowellymphoma
4. Pancreatitis INTUSSUSCEPTION
5. Pericardialeffusionwith obstructionof thoracic = telescope-like invagination or prolapseof a segmentof
duct intestinaltract (= intussusceptum = donor loop) into the
. peripheraledema/ anasarca(KEy SYMPTOM)
lumenof the adjacentintestine(= intussuscipiens
. chylous+ serouseffusion = receivingloop)
. diarrhea,vomiting,abdominalpain,malabsorption,
0 The intussuscipiens containsthe foldedintussusceptum
steatorrhea with enteringlimb+ returninglimb+ theirmesentery
. hypoproteinemia secondaryto proteinloss into
intestinallumen A . tN C H TLD R E (94%
N )
Path: dilatationof lymphvesselsin mucosa lncidence: 2-4:1,000 live births;most common
+ submucosa+ abundanceof foamy fat-staining abdominalemergencyof earlychildhood
, macrophages(negativefor pAS) 0 Leadingcause of acquiredbowelobstructionin
ri diffusesymmetricmarkedenlargementof folds in chi l dhood!
jejunum+ ileum(dueto dilatedintestinallymphatics Etiology:
+ hypoproteinemic edema) (1) idiopathic(over95%): mucosaledema
r/ slightseparation+ rigidityof folds + lymphoidhyperplasia followingviral
! dilutionof bariumcolumn(considerable increasein gastroenteritis;predominantlyat ileocecalvalve
intestinalsecretionsfrom malabsorption) (2) leadpoint(5%):
r/ no / mild dilatationof bowel (a) infants<3 months: Meckeldiverticulum (most
Lymphangiogram (notalwaysdiagnostic): common),duplication cyst
{ hypoplasiaof lowerextremitylymphatics (b) children>3 yearsof age: Burkittlymphoma,
tr occlusionof thoracicduct i largetortuousthoracicduct polypin Peutz-Jeghers syndrome,polypoid
r/ obstructionof cisternachyli with backflowinto hemangioma, enterogenous cyst,ectopic
mesenteric+ intestinallymphatics pancreas,suturegranuloma,periappendicitis,
r/ hypoplasticlymph nodes Henoch-Schonlein purpura,coagulopathy,
Dx: small bowel biopsy(dilatedlymphaticsin lamina inspissated meconium
propria+ vascularcore) mnemonic.' "H DIMPL"
Rx: low-fatdiet with medium-chaintriglycerides(direct Henoch-Schonlein purpura
absorptioninto portalvenoussystem) Duplication
DDx: (1) Whippledisease(moresegmentation ldiopathic
+ fragmentation,wild folds) Meckeldiverticulum
(2) Amyloidosis(edema+ secretionsusually Potyp
absent) Lymphosarcoma
(3) Hypoalbuminemia (lesspronouncedsymmetric Age: peak incidencebetween6 months and 2 years;
thickeningof folds,lessprominentsecretions) 3-9 monthsg0%); <1 year (50%);<2years
(75% );> 3 years(< 10% );M:F = 2:1
INTRALUMINAL
DUODENAL
DIVERTICULUM . abruptonset of violentcrampy pain (g4%)
= cor'rgenitallesionsecondaryto elongationof an . vomiting(91%)
incompleteduodenaldiaphragm . red "currantjelly"stools/ hematochezia (66%)
Age at presentation: in young adult usuallyonly after>48 hoursduration
. easy satiety . palpableabdominalmass (59%)
. vomiting . diarrhea
. upperabdominalcrampingpain . restlessness,pallor,fever
Localion: 2nd-3rd portionof duodenum Types: ileocolic(75-95%) > iteoileocotic (9%) >
r/ barium-filledsac withinduodenallumen(pathognomonic ileoileal(4%)> colocolic
piCture)= "WindSOCk, Cgmma,teardrOp"appearan.e Location: frexure
r/ anchoredto the lateralwall of the duodenum I*::"ffi?:ffi ["#i'.
836 Radiology Review Manual

Cx: vascularcompromisesecondaryto Plainfilm (40-90% accuracY):

of mesentery(hemorrhage,
incorporation r/ no abnormalityin 25/"
acuteinflammation)
infarction, { abdominalsoft-tissue mass (50-60%),usuallyin RUQ:
r/ targetsign = soft-tissuemass with concentric
B. rN ADULTS(6%) circularareas of lucency(due to mesentericfat of
0 Accountsfor <160/0 of all bowelobstructions! intussusceptum)
Etiology: ^/ meniscussign = clescefltof gas withincolonic
(a) specificcause (80%): lumenthat outlinesthe apex of the intussusceptum
1 . T um or : benig n(1 /3 ),m a l i g n a n(1t /5 ) ^/ loss of inferiorhepaticmargin
2. Postsurgical changes(1/3): adhesions ./ tittteair in smallintestine/ gaslessabdomen
adjacentto sutures / submucosalbowel ! air in displacedappendix
edema/ discoordinated motilitY r/ small bowelobstruction(25%)
3. Invaginated (= inverted)Meckeldiverticulum Antegradebariumstudy:
4. Prolapsedgastricmucosa r/ "coiledspring"appearance
5. Aberrantpancreas r/ beaklikeabruptnarrowingof bariumcolumn
6. Foreignbody,feedingtube demonstrating a centralchannel
7. Chroniculcer(TB,tyPhoid) DiagnosticEnema:
8. Priorgastroenteritis Indication: unusualage of child (<2 months,>4 years),
9. GastroenterostomY, trauma highfever,Peritoneal signs
spontaneous withoutanatomiclead point: Contraindication: free air
celiacdisease,scleroderma, Whippledisease, r/ meniscussign = cofiVeXintracolicmass = rouflded
fasting,anxiety,agonalstate apex of intussusceptum protrudesinto contrastcolumn
(b) idiopathic(20%) { "coiledspring"sign = edematousmucosalfoldsof
. recurrentepisodesof colickypain,nausea,vomiting returninglimbof intussusceptum outlinedby contrast
. abdominaltenderness,distention materialwithinlumenof colon
. changein bowelhabits US (98-100%sensitive,88-100%specific):
. palpablemass (in uP to 50%) { readilydetectablemass over 5.0 x 2.5 cm
. bloodystool (in majoritY) { "crescent-in-doughnut / targeti bull'seye sign"(on
Location: ileoileal(40/") > ileocolic(15%) transversescan) = corlc€I]tricringsof alternating
smallbowel(55%): benignneoplasm(40/"), hypoechoic+ hyperechoiclayers(= intussuscipiens)
malignantneoplasm(17'/"), with centralhyperechoicportiofl(= Inesenteryof
nonneoplastic(43%) intussusceptum)
colon @5%\ malignantneoplasm(48/"), ./ "pseudokidney / sandwichlhay fork sign"(on
benignneoplasm(21o/"), longitudinal scan)= hypoechoiclayerson each side of
n o n n e o p l a s ti(3
c 1%) echogeniccenterof mesentericfat
r/ peritonealfluid trappedinsideintussusception in
<15o/" (associated with +
irreducibility ischemia)
ri echogenicmesenterycontainslymphnodes+ ceco-
serosalsurfaces appendicealcomplexcloseto base of intussusception
r/ color Dopplerdemonstratesmesentericvessels
draggedbetweenentering+ returningwall of
intussusceptum
0 Absenceof bloodflow withinthe intussusceptum
suggestsbowel necrosis(47%)l
0 Presenceof bloodflow withinthe intussusceptum is
a good predictorof reducibilitY!
CT:
{ "multipleconcentricrings"= 3 concentriccylinders
(centralcylinder= c?l'l?l+ wall of intussusceptum;
middlecylinder= clesc€ntof mesentericfat; outer
cylinder= returningintussusceptum + intussuscipiens)
./ proximalobstruction

HYDROSTATIC / PNEUMATICREDUCTION
I intussuscipiens= receptor mucosal surfaces O <1"/omortalityif reductionoccurs<24 hoursafteronset!
in contact 70-85%
m returning il-u of intuisusceptum Overall success rate:
. \ - mesentery Contraindications:pneumoperitoneum, peritonitis,
ffi enteing limb of intussusceptum hypovolemic shock
],,i,:;fo6v791
lUmen
0 Obtainabdominalradiographto document
Anatomy of Intussusception absenceof perforationbeforereduction!
 Gastrointestinal Disorders 837

Technique: (b) thrombosis:cardiovascular disease,myocardial

(1) Sedation(debated)with morphinesutfate infarction, digitalistreatment,arrhythmia, oral
(0.2 mg/kglM) / fentanytcitratetV (straining contraceptives, hypotensiveepisode,collagen
increasesintraluminal pressureof distended vasculardisease,sicklecell disease,hemolytic-
colon) uremicsyndrome
(2) Anal sealwith 24-F Foleycatheter+ balloon (c) trauma: aortoiliacreconstruction(2%)with ligation
inflationto size equalto interpediculate
distanceof of lMA, cardiacsurgery
L5; balloonpulleddown to levatorsling;tapedto (d) idiopathic / spontaneous:mainlyin elderly
buttocks;both buttocksfirmlytaped together mnemonic; "VlNTS"
(3) 60% wt/vol barium sulfatewith containerbetween Vasculitis
24-36 inchesabove levelof anus Incarceration (hernia,volvulus)
(4) Maximally3 attemptsfor 3 minuteseach Nonocclusive ischemia(shock,CHF)
(5) Manualmanipulation increasescolonicpressure Thrombosis(atherosclerosis, emboli,polyqythemia
(6) Reductionshouldbe accomplished within10 vera,hyperviscosity)
minutes Spontaneous
(7) Extensiverefluxintosmallboweldesirableto Age: usually>50 years; M = F
excluderesidualileoilealintussusception . abruptonsetof lowerabdominalpain+ rectalbleeding
. abdominaltenderness, diarrhea
"Rule of . lack of sepsis
3s":
( 1)3. 5 f eet (1 0 5c m) a b o v eta b te(= 1 2 0 m m H g) . negativestoolcultures
(2) 3 attempts Location:segmentalinvolvement of any part of colon;
(3) 3 minutesbetweenattempts(delayallows entirecolon(11%);rightcolon(30%);transverse
venouscongestion+ edemato subside) cofon(9%);leftcolon(4G-90%);sigmoidcolon
Alternativemedium: (4%);rectumspared;
(1) 1:4 Gastrografin@-water solutionraisedto a mostcommonlyaffectedsegments:
heightof 5 feet (150cm) (a) Griffith point (80%) = junctionbetween
(2) air: delivershigherintracolonicpressures, distributionof superior+ inferiormesenteric
faster,lessfluoroscopic time,smallertears, arteriesat the splenicflexure
less contaminationof peritonealcavity (b) point of Sudeck = anastomoticplexus
(3) Ultrasound-guided salineenema: no limitto betweeninferiormesentericartery
proceduretime, low perforationrate + hypogastricvascularsupplyat
rectosigmoid junction
Cx: perforation(0.+S%; colonicburstingpressure Mean lengthof segmentalinvolvement:1g cm
-200 mm Hg);reductionof nonviablebowel;
P l ai nfi l m(usual l ynormal ):
incompletereduction;missedlead point ri segmentalthumbprinting = marginalindentations on
Prognosis; 3.5-10% rate of recurrence mesentericside (rarefindingon plainfilm)
B E (i n 90% abnormal ):
ISC HE M I C CO LI TIS 0 Singlecontrastmay effacethumbprinting, but double
= ftonocclusive ischemicdisease(majormesenteric contrastoverallis moresensitive!
vesselsusuallypatent)characterizedby acute onset ^/ thumbprinting (75%)due to submucosalhemorrhage
+ rapidclinicaland radiographicevolutionarychanges + edema
Etiology: decrease in blood flow to 20/. of normalflow r/ transverseridging= markedlyenlargedmucosalfolds
associatedwith smallvesseldisease(hypoxia) (spasm),some wall pliabilityis preserved
+ reperfusioninjurywhen blood flow is ri serratedmucosa= inflammatoryedema + superficial
reestablished; injurymoresevereif terminal longitudinal / circumferential ulceration
vascularbranchesobstructedratherthan r/ deep penetratingulcers(late)
proximalmesentericarcades CT (detectionrate of 26-39%):
Path: (a) mucosalcongestion,patchynecrosis, r/ symmetric/ lobulatedsegmentalthickeningof colonic
ulcerations + submucosaledemaand wall between2 and 20 (mean8) mm:
hemorrhage(mucosa+ submucosamost r/ shaggyconfiguration + alternatelayersof highand
sensitiveto ischemia) low attenuation (doublehalosign)+ markedpericolic
0 Earlymucosalinjuryis reversible streakiness of edema(wetappearancein 61%)
(b) injuryof muscularispropria(aftersevere ri sharplydefinedhomogeneously enhancingwail
+ prolongedischemia)can leadto transmural + mildmuralthickening(dryappearancein 33%)
necrosis r/ loss of haustralmarkings
0 Fibroticstricture,perforation,severesepsis r/ irregularnarrowedatoniclumen(= thumbprinting)
Precipitating factors: V curvilinearcollectionof intramuralgas (6%) suggests
(a) bowelobstruction:volvulus,carcinoma(proximal bowel infarction
dilatationwith increasedintraluminal pressureand r/ portal+ mesentericvenousair
reducedbloodflow) r/ btoodclot in SMA / SMV
838 Radiology Review Manual

US: Size: a few milimeters to >10 cm

{ absence/ barelyvisiblecolorflow
{ absenceof arterialsignals
r/ nonstratified(= indistinctlayers)thickenedbowelwall
>3 mm
Angio(findingssimilarto inflammatory disease):
^/ normal/ stightlyattenuatedarterialsupply
r/ mild accelerationof arteriovenoustransittime
{ smalltortuousectaticdrainingveins
Prognosis:
(1) Transientischemi€I = corTlPlete
resolutionwithin
1-3 months(76%)
(2) Stricturing ischemia= incompletedelayedhealing
r/ narrowedfoldlesssegmentof severalcm in length
with smoothtaperingmargins
(3) Gangrenewith necrosis+ perforation(extremely
unc om m o n )
(4) Mortalityrateof 11-36%
DDx: (a) dry appearance:ulcerative/ granulomatous
colitis
(b) wet appearance:pseudomembranous colitis,
CMV colitis
JEJUNAL ATRESIA
0 Air may be injectedthroughnasogastrictube
0 BE to exclude2nd and 3rd areasof atresia
Cause: intrauterineischemicinjuryto developinggut
Age: majoritypresentingduring1st day of life
ln 25% associatedwith: malrotation,volvulus,
gastroschisis,
omPhalocele
. biliousvomiting,abdominaldistension,failureto pass
meconium
P lainf ilm :
N.B.: difficultto tell colonfrom smallbowelin neonate
r/ z-g dilated bowel loops
{ absenceof gas in lower portionof abdomen
BE:
Purpose:to excludelarge-bowelcausesof obstruction,
show anatomicalsize of colon,demonstrate
m e c o n i u mi l e u s
^/ microcolon/ smallcolon/ colonof normalcaliber(due
to sufficientintestinalsecretionsin remainingsmall
bowel)
Cx: meconiumperitonitis(5%)
DISEASE
DIVERTICULAR
JEJUNOILEAL
= JEJUNAL DIVERTICULOSIS
= f?r€stform of gastrointestinal diverticulardisease
Cause: disorderedcontractionsof smoothmuscle
resultsin increasedintraluminal pressureand
mucosalherniation(= pulsiondiverticula= false
diverticula)
lncidence: 0.5-1.1-2.3/" on UGI;0.34.5% of autopsy
; >F
s e ri e s M
Age: 6th-7th decades
Location: 80% in jejunum,15'/"in ileum(usually
solitary),5o/"in jejunum+ ileum
Site: on mesentericbordernear entranceof vasa recti
. intermittentupper abdominalpain,flatulence,episodes
of diarrhea(30%)
P l a i nf i l m :
r/ air-fluidlevelsin multiplediverticula
{ slightdilatationof intestinalloopsin areaof diverticula
BE:
r/ may not fill (narrowneck / stagnantsecretions)
{ trappedbariumon delayedfilm atler24 hours
Cx:
(1) Blindloop syndromewith bacterialovergrowth
. steatorrhea, diarrhea,malabsorption, weightloss
. megaloblasticanemia(overgrowthof coliform
bacterialeadsto deconjugationof bile acids
metabolismof vitamin812)
+ intraluminal
(2) Free perforation= leadingcause of pneumo-
peritoneum without peritonitis (2140% mortality)
(3) Hemorrhage(few cases)
(4) Diverticulitis
(5) Intestinalobstruction(enterolith ileus)
JU V E N ILE P OLY P OS IS
= f?f€ autosomaldominantdiseasewith variable
penetrancecharacterizedby developmentof multiple
(>5)juvenilepolypsin Gl tract
0 Mostcommonfamilial/ nonfamilial colonicpolypin
children(75%)l
Categories:
A. Juvenilepolyposisof infancY
Age: 4-6 years(range1-10 years); M:F = 3:2
. protein-losing enteropathy, diarrhea,hemorrhage
. rectalprolaPse
r/ intussusception
B. Colonic& generalized juvenilepolyposis
Age: in 85'h manifestedby 20 years of age
. prolapseof polYP/ rectum
. rectalbleeding,anemia
Path: hamartomatouspolyps;adenomasmay coexist
Histo: little/ no smoothmuscle;hyperplasia of mucous
glands;retentioncystsdevelopwith obstruction
of gland orifices(multiplemucin{illedspaces);
edematousinflamedexpandedlaminapropria
DDx: familialadenomatouspolyposis,Peutz-
Jegherssyndrome
. rectalbleeding(95%)most commonlyas intermittent
brightred hematochezia
. anemia,pain
. diarrhea,constipation
. abdominalpain (fromintussusception)
. rectalprolapse(rare)
Location: rectosigmoid(80%);rare in small bowel
+ stomach;not in esoPhagus
{ solitarypolyp (75%);multiplepolyps (1/3)of smooth
roundcontour
r/ lesionof pinpointsize / up to severalcm in diameter
r/ invariablyon stalkof variablelength
Dx: (1) Any numberof polypswith familyhistory
(2) Polypsthroughoutthe Gl tract
(3) >5-10 PolYPs in colon
Cx: colorectalcancerby 35 years of age (in 15%)
DDx: solitaryjuvenilepolyps(<5 polyps,17oprevalence
in children)
 Gastrointestinal
Disorders 839

KAPOSI SARCOMA
= multicentric
LADDBANDS
malignantneoplasmoriginating from = cohger'rital
peritonealbands extendingfrom cecum/
endothelialcellsof lymphatic/ bloodvessels hepaticflexureover anteriorsurfaceof 2nd / 3rd portion
Cause: HIV regulatoryprotein(trans-activator target of duodenumcausingduodenalobstruction at its
ITATI)importantfor viral replicationis thoughtto 2nd portion(evenwithoutvolvulus)
cause proliferationof Kaposisarcomacells
Associatedwith: malrotation
lncidence; most commonAIDS-relatedneoplasm
r/ obliqueterminationof duodenalcontrastcolumn
(10-20-34%); in 51"/"of homosexual/
bisexualmen with AIDS;rare in hemophiliacs; LEIOMYOMA
M : F= 5 g ' 1
Location: 213occur in stomach
Histo: proliferationof spindlecells with numerous
Path: arisingfrom muscularispropria/ submucosa/
extravasatedRBCs located in clefts between
muscularismucosae/ smoothmuscleof blood
stromalcells
vesselswithinwall of viscus
Types:
Histo: intersectingbandsof muscle+ fibroustissuein a
1. ClassicKaposisarcoma
well-defined capsule
affectingmen of Mediterraneanoriginin 7th decade
{ difficultto differentiatefrom leiomyosarcoma
2. CentralAfricanKaposisarcoma
DDx: fibroma,neurofibroma, hemangioma
3. A|DS-relatedKaposisarcoma
EsophagealLeiomyomatosis
@ St<in(mostfrequentsite) Age: 6-18 (meanof 11) years;M > F
. multiplebluishred slightlyelevatedskin lesions
Cause: (1) sporadic(50%)
@ Lymphnodes(2nd mostfrequentsite): (2) familialdisease(20%): leiomyomasof
r/ abdominal+ pelviclymphadenopathy with high uterus,vulva,tracheobronchial tree,small
contrast-enhancement (secondaryto vascularity)
bowel,rectum
Associatedwith high frequencyof Gl tract involvement
(3) Alportsyndrome(30%)= nephritis,high-
@ Ot tract(40-50/",3rd mostfrequentsite): frequencysensorineuralhearingloss,
. usuallyclinicallysilent
. concurrentwith I attercutaneousdisease congenitalcataract
Site: distalthirdi halfof esophagus+ extensioninto
0 Gl tractis the only site of involvement in <S/ol proximalstomach
Location: anywherewithinGl tract;often multifocal ., slowlyprogressivedysphagiaover years
^/ thickenednodularfolds
ri smoothtaperednarrowingof distalesophagusover
r/ multiplesubmucosalnodules+ centralumbilication
an averagelengthof 6 cm
r/ polypoidalmass r/ decreased/ absentesophagealperistalsis
{ infiltrating linitisplasticalesion(rare) r/ smoothrelativelysymmetriCdefect at cardia (from
@ Liver (34% at autopsy) thickenedmusclebulgingintogastricfundus)
infrequentlycontributesto morbidity+ mortality
CT:
r/ multiple5-12-mm noduleshypeiechoicon US,
r/ markedcircumferential wall thickeningof up to 4 cm
hypoattenuating on NECTiCECTindistinguishable from mass with relativelylow soft-tissueattenuation
from multiplehemangiomas
DDx: (1) Primaryachalasia(shorternarrowedsegment)
DDx: metastaticdisease,fungal microabscesses,
(2) Secondaryachalasia(olderindividual, recent
multipleareasof bacillaryangiomatosis
onset of dysphagia)
(= swollenvenouslakesin liver)
(3) Stricturefrom refluxesophagitis
@ Lung (18-47% of patientswith cutaneoussarcoma): (4) ldiopathicmuscularhypertrophy
= latecomplication of the
of AIDS esophagus(in late adulthood,corkscrew
Site: peribronchial + perivascular axialinterstitium appearanceof esophaguswith nonperistaltic
, (91%); middle / lower lung zones (92%) contractions,cardiararelyinvolved)
1 coarseningof bronchovascular bundles:
tr tram track opacities Leiomyomaof Esophagus
r/ peribronchial cuffing 0 Mostcommonbenignsubmucosal tumorof esophagus
r/ septallines (38-7io/o) lncidence: 1:1,119 (autopsystudy);
r/ centralperihilarcoalescentconsolidation 50% of all benignesophagealtumors
+ air bronchograms in 45h (= confluenttumor) A ge: youngadul ts;3/" i n chi l dren;M > F
r/ small(50%) tlarge(28%)pulmonarynodutes . usuallyasymptomatic(due to slow growth)
, (= tumor proliferationextendinginto parenchyma) . dysphagia,odynophagia, dyspepsia
r/ pleuraleffusion(33-67"/"),chylothorax(rare) . hematemesis if large(rare)
r/ moderatelymphadenopaihy ('16%) Site: frequentlylower+ mid 1i3 of esophagus;
@ Lowerextremities intramural;multipleleiomyomasin 3-4%
^/ tyticcorticallesion ^l Z-15-cmlargesmoothwell-definedintramuralmass
r/ subcutaneous nodules causingeccentricthickeningof wall + deformityof
Dx: visualization+ biopsyof mass with red-purplecolor l umen
840 Radiology Review Manual

r/ may have coarsecalcifications: of Stomach

Leiomyosarcoma
0 Leiomyomais the only calcifyingesophagealtumor! lncidence; 0.1-3% of all gastricmalignancies
{ ulcerationextremelyuncommon Age: 10-73 Years; M > F
Histo: pleomorphism,hypercellularity, mitoticfigures,
{ uniformsofttissuedensity cysticdegeneration,necrosis
r/ diffusecontrastenhancement . Gl bleeding(fromulceration)
CAVE: high percentagemisdiagnosedas extrinsic . obstruction
les io n ! Metastases:
(a) hematogenous to liver,lung,peritoneum; rarelyto
Leiomyomaof SmallBowel bone + soft tissue
Mostcommonbenigntumorof smallbowel (b) directextensioninto omentum,retroperitoneum
Location: duodenum(21'/"),jejunum(48%),ileum (c) lymph nodes (rare)
( 31 % );s i n g l ei n 9 7 ' h Location: 90% in fundusi body of stomach
mainlyserosal(50%),mainlyintraluminal (20%), Site: anterior/ posteriorwall; endo-/ exogastric
intramural (10%) { averagesize of 12 cm
Size: <5 cm (50%),5-10cm (25%),>10 cm (25/") { intramuralmass
{ small ulcer + largebarium-filledcavity(central r/ may be pedunculated
necrosis+ communication with lumen) { large massestend to be exogastric
{ hypervascular { very frequentlyulcerated
CT:
r/ lobulatedirregularoutline
Leiomyomaof Stomach r/ heterogenousexogastricmass with centralzones
0 2ndmostcommonbenign gastric
tumor(after
gastric of low density(necrosiswith liquefaction)
polyp),mostcommonof calcifiedbenigntumors { air / positivecontrastwithintumor (= ulceration)
Location: pars media (39%),antrum(26'/"),pylorus { dystrophiccalcifications
(12/o),fundus(12%),cardia(10%)
Site: intraluminal submucosal(60%),exophytic
subserosal (35%), combinedintramural- CarneySyndrome
extramuraldumbbelltype mass (5%) leiomyosarcoma
Triadof (1) Gastricepithelioid
0 90% of all submucosaltumors! (2) Functioning paraganglioma
extraadrenal
{ averagesize of 4.5 cm (3) PulmonarY chondromas
{ ovoidhass with smoothmargiir+ smoothsurface lncidence: 24 patientsreported; M:F = 1:11
(mostfrequentlY)
^/ forms rightanglewith gastricwall
{ ulceratedin 50% LIPOMA
r/ pedunculated intraluminal tumorin submucosal = benignsubmucosaltumorcomposedof matureadipose
growth(rare) tissue
{ "icebergphenomenon"= large extraluminal 0 Mostcommonsubmucosaltumorin colon
componentin subserosalgrowth lncidence: in colon in 0.25/" (autopsy);2-3% of benign
r/ calcifiesin 4oh gastrictumors
Cx: (1) Hemorrhage(acute/ chronic) Location: colon (particularlycecum + ascendingcolon)>
(2) Obstruction(tumorbulk / intussusception) duodenum> ileum> stomach(gastricantrum)
(3) Infection > jejunum> esoPhagus
(4) Fistulization / perforation . asymptomatic
(5) Malignantdegeneration . crampypain,hemorrhage(rare)
( beni g n :m a l i g n a=n3t :1 ) { smooth,sharplyoutlined,round/ ovoidglobularmassof
. 1-3 cm in diameter
r/ shortthick pediclein 1/3 causedby repeatedperistaltic
LEIOMYOSARCOMA activity(proneto intussuscePt)
of SmallBowel
Leiomyosarcoma r/ markedradiolucency
{ changein shape+ sizeon compression due to softness:
Location: duodenum(26%),jejunum(34o/"), ileum
(40%) { "squeezesign" = s€lUSoge-shaped mass on
{ usually>6 cm in size postevacuationradiograPhs
r/ nodularmass: intraluminal (10%),intraluminal CT:
pedunculated(5%), intramural(15/"\, chieflyextrinsic i/ well-circumscribed submucosalmass of uniformfat
(66%) densitY
Cx: (1) IntussuscePtion (rare)
{ mucosamay be stretched+ ulcerated(50%)
r/ may show centralulcer pit / fistulacommunicating (2) Ulceration(frompressurenecrosisof overlying
with a large necroticcenter mucosaby largeliPoma;rare)
{ intussusception Prognosis; NO liposarcomatousdegeneration
 Gastrointestinal
Disorders 841

LYMPHANGIOMA (3) Giardialambliainfection(90%)

= congenitalmalformation of lymphaticvessels (4) Functional
thyroidabnormalities
Path: usuallymultiloculatedlargethin-walledcystic Location: primarilyjejunum,may involveentiresmall
masswith chylous/ serous/ hemorrhagic fluid bowel,ascendingcolon+ hepaticflexure,
contents seldomin sigmoid/ rectum
Location: mesentery;rarelyaffectingGl tract . malabsorption(diarrhea+ steatorrhea)
r/ proximalboweldilatation(in partialbowelobstruction) . low serumconcentrations of lgA, lgG, lgM
US: r/ mucosastuddedwith innumerable-1-3-hm small
r/ multiseptatedcysticmass with lobules uniformpolypoidlesions
r/ fluidanechoic/ with internalechoes/ sedimentation { lesionsmay be umbilicated (uncommon)
CT :
r/ cysticmass with contentsof water-to fat-density LYMPHOMA OF GASTROINTESTINAL TRACT
MR: Classification:
r/ serouscontents:hypointense on TlWl A. PRIMARYLYMPHOMAOF BOWEL
+ hyperintenseon T2Wl (a) localized (b) diffuse
r/ hemorrhage/ fat: hyperintense on TlWl + T2Wl Predisposed: Arabs + Middle EasternJews
Rx: surgery(difficultdue to intimateattachmentto Associatedwith: celiac disease
bowelwall) B. SECONDARYINTESTINALLYMPHOMA
as part of generalizedsystemicprocess
LYMPHOGRANULOMA
VENEREUM
= LGV = sexudllytransmitteddiseasecausedby virus lncidence: 4-20% of all NHL;10/" of patientswith
Chlamydiatrachomatisproducinga nonspeciiic abdominallymphomahavebowelinvolvement
granulomatousinflammatoryresponsein infected At risk: long-standingceliacdisease,AIDS, systemic
mucosa(mononuclearcells + macrophages),perirectal lupuserythematosus, Crohndisease,historyof
lymphaticinvasion chemotherapy
Location: rectum,may extendto sigmoid+ descending Median age: 60 years
colon Histo:
M : F= 3 . 4 : 1 (1) T-cellmalignantlymphoma(in celiacdisease)
./ narrowing+ shortening+ straightening of rectosigmoid (2) B-celllymphoma
r/ wideningof retrorectalspace (3) lmmunoproliferative smallintestinaldisease
r/ irregularityof mucosa+ ulcerations (= Mediterranean lymphoma)
r/ paracolicabscess (4) Low-gradeB-celllymphoma(= low-grademucosa-
r/ fistulato pericolicarea, rectum,vagina (common) associatedlymphoidtissue= MALT lymphoma)
Rx: tetracyclineseffectivein acute phase before 50-72% of all primarygastriclymphomas;
scarringhas occurred Associatedwith: Helicobacterpylori gastritisin
90% (may regresscompletely
LYMPHOID HYPERPLASIA after antibiotictherapy)
lncidence: normalvariantin 13/" of BE examinations (5) Follicularlymphoma
Histo: hyperplasticlymphfolliclesin laminapropria (6) Burkittlymphoma(in children)
(Peyerpatches),probablycompensatoryattempt (7) Mantlecell lymphoma
for immunoglobulin deficiency (8) Hodgkindisease(<15%)
Etiology: May be associatedwith: enlargementof extraabdominal
( 1) Nor m alin c h i l di y o u n ga d u l t lymphnodes,malabsorption
(2) self-limitinglocal/ systemicinflammation / infectioni Radiographic types:
allergy 1. Polypoid/ nodular(47%)
(3) May be relatedto immunodeficiency i r/ enlargednodularfolds
dysgammaglobulinemia with smallbowel 2. Ulcerative(42%)
involvement ! ulcerativelesions,may be complicatedby
Age: (a) generallyin children<2 years perforation
(b) in adultsinvariablyassociatedwith late onset r/ aneurysmalconfiguration
immunoglobulin deficiency(lgA,lgM) 3. Diffuselyinfiltrating
(11%)
Associatedwith: splenomegaly,largetonsils, r/ diffusehoselikethickeningof bowelwall
eczematousdermatitis,achlorhydria, r/ decreased/ absentperistalsis
perniciousanemia,acutepancreatitis,
coloniccarcinoma CT staging:
At risk for: Stage I tumor confinedto bowelwall
( 1) G ood s y ndr o me(1 0 % ) Stage ll limitedto local nodes
= gastriccarcinoma+ benignthymoma+ lymphoid Stage lll widespreadnodaldisease
hyperplasia Stage lV disseminatedto bone marrow,liver,other
(2) Respiratoryinfections organs
842 Radiology Review Manual

10-25/" of NHL are extranodal;stomach 2. S i ngl e/ mul ti pl epol ypoi dmucosal isub m ucosal
(50%)> smallbowel> colon> esophagus; MASSES
multicentric in 10-50% ^/ cobblestonedefectsdue to lymphomatous
r/ enlargement of spleen polyps
of regionallymphnodes ^/ nodulesmay ulcerate
r/ nutry enlargement
./ may causeintussuscePtion
@ Esophagus
leastcommonsite of Gl involvement (in <1%) ri sprue Pattern
Stomach 3. Endoexoenteric mass
@
1-5% of all gastricmalignancies; mostcommonsite of ^/ largemasswith only smallintramural
extranodallymphoma (25%);most frequentsite of comPonent
{ t ulcer+ fistulas+ aneurysmatic dilatation
involvementby NHL (50%);isolatedprimarygastric
m alignancy in 10% 4. Mesenteric/ retroperitonealadenopathy
for any particularregionof ! single/ multipleextraluminalmasses
Location: no predilection
stomach displacingbowel
Site: arisesin lymphoidtissueof laminapropriathat confluentmassengulfing+ encasing
^/ ill-defined
forms secondaryto chronicHelicobacterpylori multiplelooPsof adjacentbowel
gastritis(normallygastricmucosahas no r/ "sandwichconfiguration"= IT'l?sS
surrounding
lymphoidtissuel) mesentericvesselsthat are separatedby
Directextensioninto: pancreas,spleen,transverse perivascularfat
colon,liver ^/ conglomeratemantleof retroperitoneal
r/ pliantgastricwall + mesentericmass
r/ duodenumoften affectedwhen antruminvolved
r/ circumscribedmass with endogastric/ exogastric @ C ol on
(25%) growth Lesscommonlyinvolvedthan stomachi smallbowel;
^/ broadtortuousmucosalfolds over largeportionsof 1.5%of all abdominallYmPhomas
stomach(diffuseform): Location: cecummostcommonlyinvolved(85%)
r/ singlemass > diffuseinfiltration> polypoidIesion
r/ polypoid/ nodular
r/ largeirregularulcer r/ paradoxicaldilatation
r/ rarelyluminalnarrowing ./ gross muralcircumferential / focal soft-tissue
CT: thickening(averagesize of 5 cm)
./ diffuseinvolvementof entirestomach(50%), r/ slightenhancement
typicallymore than half of gastriccircumference ^/ massiveregional+ distantmesenteric
{ segmentalinvolvement(15%) adenoPathY
+ retroperitoneal
r/ ulceratedmass (8%) DDx: frequentlyresemblesinflammatorydisease/
r/ averagewall thicknessof 4-5 cm polyposis
{ luminalirregularity (66%)
r/ hyperrugosity(58%) Prognosis: (a) 71-82%2-year survival rate in isolated
Prognosis: 55/" S-yearsurvival rate after resection bowellymphoma
DDx: gastricadenocarcinoma(wallthickeningless (b) 0% 2-yearsurvivalrate in stage lV
pronounced,perigastricfat plane not likely diseasewith bowelinvolvement
preserved,luminalnarrowing,rigidwall,smaller
lymph nodesabovethe levelof renalveins) Cx during chemotherapy: perforation(940/"),
hemorrhage
@ Smallbowel
1/5 of all smallbowelmalignancies; mostcommon
malignantsmallboweltumor;multiplesitesof
involvement in 1/5;mostcommoncauseof MALIGNANT MELANOMA
intussusception in children>6 years = developsfrom melanocytesderivedfrom neuralcrest
Location: ileum(51%), jejunum(47%\,duodenum cells,arisingin preexisting benignnevi (in 20%)
(2%), lncidence: l"h of all cancers; increasingat 3'9% per year
Site: arisingfrom lymphoidpatchesof Peyer Peak prevalence: 40-60 Yearsof age
Types: Riskfactors: dysplasticmole,atypicalmelanocytic
1. Infiltrating lymphomawith plaquelikeinvolvement hyperplasia, xerodermapigmentosum,
of wall >5 cm in length(80%)l>10 cm in length melanomain first-degree relative,sun-
(20%)(DDx: Crohndisease) sensitivephenotype,excessivesun
r/ t ulceration(considerableexcavation) exposure
^/ desmoplasticresponse Primarysites: skin,mucousmembranes,
^/ thickenedvalvulaewith corrugatedappearance leptomeninges, eye
^/ aneurysmaldilatation(secondaryto destruction . areas of red / white/ blue in additionto brown and black
of autonomicnerveplexus+ muscle/ tumor colorsof benignnevi
necrosis) . irregularborderswith notching+ strikingprotrusions

@ Stin primary
Clark staging:
Level I alltumorcellsabovebasementmembrane
(in situ lesion)
Level ll tumor extendsto papillarydermis
Level lll tumor extendsto interfacebetween
papillary+ reticulardermis
Level lV tumor extendsbetweenbundlesof
collagenof reticulardermis
Level V tumor invasionof subcutaneoustissue (in
87o/"metastatic)
Breslow staging:
thin <0.75mm depthof invasion
intermediate 0.76-0.99 mm depth of invasion
thick >4 mm depthof invasion
METASTASES:
latentperiodof 2-20 years after initialdiagnosis
(mostcommonly2-S years)
Primarysite: head + neck (7g%),eye (77%), Gt)
system (67"/,),Gl tract (in up to 60%)
@ Lymphadenopathy
- in 23% with levelll + lV
- in 75% with levelV
. sentinelnode biopsy:
r/ intraoperativeintradermalinjectionof dye
r/ preoperativelymphoscintigraphy
@ Bone(7-17%)
Prevalence: 304C,/" at autopsy
. often initialmanifestationof recurrence
. poor prognosis
r/ predominantlyosteolytic
Location: axialskeleton(g0%),ribs (39%)
@ Lung (70% at autopsy)
most commonsite of relapse;
respiratoryfailuremost commoncause of death
@ Liver (17-23%;58-66% at autopsy)
{ single/ multiplelesions0.5-15 cm in size
{ largerlesionoften necrotic
^/ may be partiallycalcified
@ Spleen(1-5%;33/. at autopsy)
r/ single/ multiplelesionsof variablesize
r/ solid/ cystic
@ Cl tract + mesentery(+B%)
. abdominalpain,Gl bleeding
Location: small intestine(35-50%),colon (14-ZO/"),
stomach (7-20%)
r/ multiplesubmucosalnodules* ,,bull's-eye
, appearance= Geotralulceration
! irregularamorphouscavity(exoentericgrowth)
r/ intussusception (10-ZO%i
@ Kidney(up to 35% at autopsy)
@ Adrenal(11"/",up to S0% at autopsy)
@ Subcutis
MR of melanoticmelanoma:
{ nypo-/ isointenseon TlWl + T2Wl + STIR images
(mostcommonly)
hyperintenseon Tl Wl + hypointenseon T2Wl (dueto
T1-shortening
effectof the paramagneticmetalsiron
+ copperboundto melanin)
Disorders g4g
Gastrointestinal
DDx: melanotic/ amelanotichemorrhagictumor
(hyperintenseon Tl Wl + iso-/ hyperintenseon
T2Wt)
Prognosis: 3040/" eventually die from this tumor
MALLORY.WEISS SYN DROM E
= rTlucos?l + submucosaltear with involvementof venous
plexus
Pathophysiology: violent projectionof gastric contents
againstloweresophagus
Age: 30-60 years; M > F
Predisposed: alcoholics
. historyof repeatedvomitingpriorto hematemesis
. massivepainlesshematemesis
Location: at / abovel below(76%)esophagogastric
junction
r/ longitudinalsingletear in 77o/o,
in 2Jh multipletears
r/ extravasationof barium
Angio:
r/ bleedingsite at gastriccardia
DDx: pepticulcer/ ulcerativegastritis
MALROTATION
= abnormalpositionof gut secondaryto a narrow
mesentericattachmentas a resultof arrestin the
embryologicdevelopmentof gut rotation+ fixation
Embryology:
duodenojejunal + ileocolicsegmentsof primitive
digestivetube rotateby 270" in a counterclockwise
directionaboutthe omphalomesenteric vesselsto cross
beneaththe vessels(futureSMA + SMV);normallyLUe
fixationat ligamentof Treitz(an extensionof the right
crus of diaphragm)+ fibroustissuearoundceliacartery,
locatedto left of L2) + RLQ fixationof cecum
Abnormalfixationof mesentery:shorterthan usual,its
upperpointbelowthe normalpositionof ligamentof
Treitz,its lower pointsuperior+ medialto normalcecal
position
Definition:
Nonrotation
= midgutloop returnsto peritonealcavitywithout
rotationresultingin weak peritonealfixation
Frequencyi common
. generallyasymptomatic:oftenincidentalfindingin
/ target" olderchildren+ adults
./ SMn to rightof SMV
^/ targeintestineon left + small intestineon right
Cx: volvulus(as a resultof localclockwiserotation)
with "whirlsign"aroundSMA
Incomplete rotation
= failureof midgutloopto completefinalg0. of
rotation
. prearterialsegmentof midgutreentersabdomen
firsttoward left side
{ cecumjust inferiorto pylorus
Cx: duodenalobstruction(peritonealbands pass
over duodenum)
844 Radiology Review Manual

Normal Duodenal Position Nonrotation of Duodenum Corkscrew Duodenum + Jejunum

Partial Duodenal Rotation with Partial Duodenal Rotation with Redundant-Duodenum Malrotation
Jejunum in Right Upper Quadrant Duodenojejunal Junction over Right Pedicle to Right of Spine

Malrotation

Reversed rotation ^/ duodenojejunaljunction to leftof spinebut low

Frequency.' rare ^/ duodenalredundancy to rightof spine
. postarterialsegmentof midgutreentersabdomen ./ Z-shapeconfiguration of duodenum+ jejunum
first ri small bowelon right + colon on left side of abdomen
= cecurTlmigratesfirst passingbehindSMA toward (in 0.2% incidentalfindingin adults)
rightthus unwindingthe normalcounterclockwise r/ abnormalpositionof duodenum+ cecum (84%)
rotationof the first stagewith additionalfinal 90" ./ normalpositionof duodenum(3%)
clockwiserotation r/ normalpositionof cecum(in 5-20%)
r/ duodenumanteriorto SMA DDx: mobilececum (15%)
r/ transversecolonbehindduodenum+ SMA CT:
Cx: obstructionof transversecolon by pressure r/ Sfuv positionedto left of SMA (80%)
from SMA r/ aplastic/ hypoplasticuncinateprocessof pancreas
Cx: midintestinal / midgutvolvulus,duodenal
Associatedwith: urinarypseudoobstruction, prune- Laddbands,internalherniation
obstruction,
bellysyndrome,cloacalexstroPhY
. symptomsof partial/ completeproximalbowel MASTOCYTOSIS
obstruction: = URTICARIA PIGMENTOSA
. vomiting(77%of neonates;in 39% within1stweek of = systemicdiseasewith mastcell proliferation in skin and
life) RES (laminapropriaof smallbowel;bone;lymphnodes;
. recurrentattacksof vomiting+ distension(in older liver;spleen)associatedwith eosinophils + lymphocytes
children) Age: <6 monthsold (in 50%)
Bariummeal & bariumenema: Associatedwith: myeloproliferative disorders,acute
Purpose: guessthe locationof abnormalperitoneal leukemia,malignant
nonlymphatic
fixationfrom positionof bowel! lymphoma,mastcell leukemia
r/ clearlyabnormalpositionof duodenum(81%): Categories:
r/ duodenum+ jejunumto the rightof spine(30%) I indolentmastocytosis(mostfrequent)
{ corkscrewduodenum+ jejunum(29/") ll mastocytosisassociatedwith myeloproliferative /
r/ duodenojejunaljunctionlow + in midline(22%) myelodysplastic hematologicdisorder
{ unusualabnormalpositionof duodenum(16%): lll aggressive/ lymphadenopathic mastocytosiswith
r/ duodenojejunaljunctionover rightpedicle eosi noP hi l i a
 Gastrointestinal
Disorders 845

lV mastcell leukemia(rare) Histo: containsectopicmucosain 50%: gastric/

. diarrhea,malabsorption, steatorrhea,anorexia pancreatic/ colonicmucosa
. urticariapigmentosa= cutaneousform (in g0-90%):
0 Frequencyof ectopicgastricmucosa:
. hyperpigmented skin lesionsexhibiting*whealand 15-34% overall;60% in symptomaticchildren;
flare"phenomenonwhen disturbed in >95%with Gl hemorrhage
. abdominalpain,nausea,vomiting Location: withinterminal6 feet of ileum(= 30-90 cm
. tachycardia, asthma,flushing,gastrointestinal upset, from ileocecalvalve);ing4/" on
headache,pruritus(due to liberationof histamine/ antimesenteric border
prostaglandinDr) . asymptomatic(20-40%)
causedby: physicalexertion,heat,certainfoods,
alcohol,nonsteroidalantiinflammatory R U LEOF 2s: (1) i n 2" h ol popul ati on
drugs (2) symptomaticusuallybefore age 2
. pancytopenia (chronicneutropenia) (3) locatedwithin 2feetof ileocecalvalve
@ Skeletalinvolvement(70%) (4) lengthof 2 inches
. bone and jointpain NUC (>85%sensitive,>g|h specific,>83-88%accurate):
r/ osteoporosis(dueto releaseof heparin 0 Tc-99mpertechnetateis excretedby mucoidcells of
, + prostaglandinby mast cells activatingosteoclasts) gastricmucosa,excretionis not dependenton
r/ scatteredwell-definedscleroticfoci with focal/ presenceof parietalcells
diffuseinvolvement (dueto releaseof histamineby N.B.: sensitivity dropsafteradolescence, because
mast cells promotingosteoblasticactivity);often patientsasymptomaticthroughoutchildhoodare
alternatingwith areas of bone rarefaction less likelyto have ectopicgastricmucosa
Predilectedsites: skull,spine,ribs, pelvis,humerus, Preparation:
femur (1) No irritativemeasurestor 48 hours (contrast
@ Reticuloendothelial system studies,endoscopy,cathartics, enemas,drugs
r/ hepatomegaly irritatingGl tract)
ri splenomegaty(49-61%) (2) Fastingfor 3-6 hours (resultsin decreasedgastric
r/ lymphadenopathy:retroperitoneal, periportal, secretion+ diminishedbowelperistalsis)
mesenteric (3) Evacuationof bowel+ bladderpriorto study
r/ guOO-Chiari hepaticvenoocclusivedisease Dose: 5-10-20 mCi (100 pOi/kg)Tc-99m
{ reversedportalvenousflow pertechnetate(adultdose!)
{ cavernoustransformationof portalvein Radiationdose: 0.54 radl2 mCi for thyroid;
@ Abdomen 0.3 radl2 mOifor largeintestine;
. nausea,vomiting,diarrhea
0.2 radl2 mCi for stomach
^/ thickeningof omentum,+ mesentery lmaging: immediatecontinuousanteriorimagingfor
r/ ascites: (a) transudativesecondaryto liverdisease 30-45 minutes/ serialimagesin 5-1O-minute
(b) exudativefrom mast cell proliferationof intervalsfor up to t hour
peritoneum r/ smallfocalcollectionof tracer in RLe appearingat
@ Smallbowel the same time / shortlyafter gastricactivity
r/ generalizedirregulardistortednodularthickened r/ traceractivityincreasesin intensitywith time parallel
foldst wallthickening(dueto infiltrationby mast to that of stomach
, cells,lymphocytes, plasmacells) ./ improvedvisualization through
{ diffusepatternof 2-S-mm sandlikemucosalnodules (a) pentagastrin= stimulatesuptake(6 pg/kg SC
! urticaria-likelesionsof gastric+ intestinalmucosa 20 minutespriorto pertechnetate)
Dx: skin/ bone marrowbiopsy;jejunalbiopsy (b) cimetidine= inhibitssecretion(maximum300 mg/
demonstratesan excessof mast cells dose lV t hourprior)
Cx: (1) Pepticulcerdisease(releaseof histamine (c) glucagon= decreasesperistalsis(50 pg/kg lM
increasesgastricacid secretion) 5-10 minutesprior)
(2) Leukemia ! poor visualizationwith use of perchlorate+ atropine
Rx: antihistamines, histaminedecarboxylase inhibitors, (= depresseduptake)
sodiumchromoglycase; steroids;splenectomy(for False-positive resu lts:
symptomatic splenomegaly / hypersplenism) (1) Ectopicgastricmucosain gastrogenic cyst,enteric
DDx: carcinoid,pheochromocytoma duplication, normalsmallbowel,Barrettesophagus
(2) Increasedbloodpool in AVM, hemangioma,
hypervascular tumor,aneurysm
MECKELDIVERTICULUM (3) Duodenalulcer,ulcerativecolitis,Crohndisease,
= persistenceof the omphalomesenteric duct (= vitelline appendicitis, laxativeabuse
duct),whichusuallyobliterates
by 5th embryonicweek (4) lntussusception, intestinalobstruction,
volvulus
0 Most commoncongenitalabnormalityof the Gl tracil (5) Urinarytractobstruction, calicealdiverticulum
lncidence: 0.3-2-3% of population(at autopsy) (6) Anteriormeningomyelocele
Age: majorityin children<10 yearsof age; M:F = 3:1 (7) Poortechnique
846 Radiology Review Manual

mnemonic.' "HA GUIDI' { multipleroundI ovalfillingdefectsin distalileum+ colon

Hemangioma r/ functionalmicrocolon(unusedcolon in antenatal
Appendicitis obstruction)
Gastricectopia OB -U S :
Urinaryobstruction ^/ unusualechogenicintraluminal areasin smallbowel
Intussusception (DDx: normaltransientinspissated meconium)
Duplicationof bowel { usuallypolyhydramnios
Inflammatoryboweldisease r/ ttuid-titteddilatedsmallbowel
Cx (in 40-50%): volvulus,ischemia,necrosis,stenosis,
False-negative results: atresia,perforation,meconium
(1) lnsufficientmass of ectopicgastricmucosa peritonitis,pseudocyst
activity(hemorrhage
(2) Dilutionof intraluminal i Rx: (1) Nonioniccontrastmediaenema(becauseof risk
hypersecretion) of bowelPerforation)
mnemonic.' "MlS" (2) 17% Hypaque/ Conrayenema mixedwith
Malrotationof ileum acetylcystei ne (MucomYst@)
lrritablebowel in RLQ (rapidtransit) (3) Gastrografin@ enemawith Tween 80 (attention
Small amountof ectopicgastricmucosa to fluid + electrolytebalance)
, DDx: Hirschsprungdisease,small bowelatresiawith
Enteroclysis: meconiumileus,meconiumplug syndrome,small
r/ elongated,smoothlymarginated, clublike,intraluminal left colon syndrome,imperforateanus, obstruction
mass parallelto long axis of distalileum = inverted from duplication cyst
Meckeldiverticulu m (20%)
./ O.S-ZO-cm-long blind pouchon the antimesenteric
borderof ileumwith junctionalfold pattern ME C ON IU M P E R ITON ITIS
Angio (59% accuracy): = sterilechemicalperitonitissecondaryto perforationof
./ presenceof vitellineartery(= onomalousend branch bowelproximalto high-grade/ completeobstructionthat
of superiormesentericartery)is PATHOGNOMONIC seals in uterodue to inflammatoryresponse
Cx (in 20%): lncidence; 1:35,000livebirths
(1) Gl bleedingsecondaryto ulceration(in 95% due to Age: antenatalperforationafter 3rd monthof gestation
ectopicgastricmucosa) Cause:
(2) Acute diverticulitis (1) Atresia(secondaryto ischemicevent) (50%)
(3) Intestinalobstructionsecondaryto intussusception (a) of smallbowel(usuallyileumor jejunum)
(diverticulumacts as lead point)/ volvulus(when (b) of colon(uncommon)
omphalomesenteric diverticulumattachedto (2) Bowelobstruction(46%)
umbilicusby fibrousband) (a) meconiumileus
(4) Malignanttumor(rare): carcinoma,sarcoma, (b) volvulus,internalhernia
carcinoid (c) intussusception, congenitalbands,Meckel
(5) Chronicabdominalpain diverticulum
(3) Hydrometrocolpos
0 Meconiumperitonitis due to cysticfibrosis
ILEUS
MECONIUM diagnosedin uteroin 8/" + at birthin 15-40%!
= srn?llbowel obstructionsecondaryto desiccated 0 Intraperitoneal meconiummay calcifywithin
meconiumpelletsimpactedin distalileum 24 hours!
Age: may developin utero(in 15%) Types:
Associated with: (a) fibroadhesive type (mostcommon):
cysticfibrosiswith tenacious+ sticky meconiumdue to = intensechemicalreactionof peritoneum, which
deficiencyof pancreaticsecretions(in almost 100%) sealsoff the Perforation
0 Earliestclinicalmanifestationof cysticfibrosis! . no evidencefor activeleak at birth
0 Virtuallyall infantswith meconiumileusproveto have { dense mass with calciumdePosits
cysticfibrosis { calcificplaquesscatteredthroughoutperitoneal
0 10-15/" of infantswith cysticfibrosispresentwith cavitY
m ec oniumi l e u s ! (b) cystictype:
. abdominaldistension,biliousemesis = cysticcavityformedby fixationof bowel loops
. failureto pass meconiumwithin48 hours surrounding the perforation
site,whichcontinues
to leak meconium
^/ numerousdilatedsmallbowelloopswithoutair{luid { cyst outlinedbY calcificrim
levels(fluidnot present) (c) generalized type:
r/ "bubbly"I "frolhy"appearanceof intestinalcontents . perforationoccursimmediatelyantenatally
^/ "soap-bubble" / "applesauce"appearancein RLQ (in . active leakageof bowel contents
50-66%) due to admixtureof gas with meconium r/ complicatedascites
 Gastrointestinal Disorders 847

! intraabdominal calcifications
(conspicuouslyabsentin MELANOSIS COLI
cysticfibrosis): = benignbrown-black discoloration
of colonicmucosa
r/ peripherallycalcifiedpseudocysts Incidence: 10o/"of autopsies
{ smallflecksof calcificationsscatteredthroughout Cause: ? chronicanthracenecatharticusage
abdomen . asymptomatic
^/ largeraggregatesof calcificationsalong inferior
Prognosis.' nomalignantpotential
surfaceof liverI flank/ processusvaginalis/ scrotum
r/ obstructiveroentgensignsfollowingbirth
MENETRIER
DISEASE
r/ separationof bowefloopsby fluid = GIANTHYPERTROPHIC GASTBITIS = HYPERPLASTIC
r/ microcolon= "unusedcolon;'
GASTROPATHY
r/ meconiumhydroceleproducinglabialmass = characterizedby excessivemucus productionand
US: TRIADof (1) Giantmucosalhypertrophy
r/ nignlyechogeniclinear/ clumpedfociwith posterior (2) Hypoproteinemia
acousticshadowingin scrotum (3) Hypochlorhydria
r/ "snowstormappearance"= highlyechogenicmaterial
Path: mucosalthicknessup to 6 mm (normalrange:
, throughoutabdomenin betweenbowel loops 0 . 6 - 1 . 0m m )
{ ill-/ well-defined homo-/ heterogeneous encysted Histo: hyperplasiaof glandulartissue+ microcyst
collectionsof meconium formation
O B - US : Age: 20-70 years; M:F = 2:1
r/ polyhydramnios (64-71%) Associatedwith: benigngastriculcer (19-72%)
r/ fetal ascites (54-57%) . epigastricpain,vomiting,weightloss
r/ bowet dilatation(27-29%) . gastrointestinal bleeding
r/ intraabdominal brightechogenicmass . protein-losing enteropathywith hypoproteinemia
r/ multiplelineari clumpedtoii ot calcifications(g4%); + peripheraledema
, may developwithin 12 hoursafter perforation . absent/ decreasedacid secretion(>50%)
r/ meconiumpseudoclst= well-defined hypoechoic Location:throughoutfundus+ body,particularly prominent
mass surroundedby an echogeniccalcifiedwall alonggreatercurvature,antrumusuallyspared
(= containedperforation) (DDxto lymphoma:usuallyin antrum)
DDx: (1) Intraabdominal teratoma r/ markedlyenlarged+ tortuousgasiricrugae in spite of
(2) Fetalgallstones