Clinical Manifestations and Diagnosis of Echinococcosis

14/2/2015
Clinicalmanifestationsanddiagnosisofechinococcosis
OfficialreprintfromUpToDate
www.uptodate.com2015UpToDate
Author
PedroLMoro,MD,MPH
SectionEditor
PeterFWeller,MD,FACP
DeputyEditor
ElinorLBaron,MD,DTMH
Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Jan2015.|Thistopiclastupdated:Oct21,2014.
INTRODUCTIONEchinococcaldiseaseiscausedbyinfectionwiththemetacestodestageofthetapeworm
Echinococcus,whichbelongstothefamilyTaeniidae.FourspeciesofEchinococcusproduceinfectionin
humansE.granulosusandE.multilocularisarethemostcommon,causingcysticechinococcosis(CE)and
alveolarechinococcosis(AE),respectively.Thetwootherspecies,E.vogeliandE.oligarthrus,cause
polycysticechinococcosisbuthaveonlyrarelybeenassociatedwithhumaninfection.
Theclinicalmanifestationsanddiagnosisofcysticandalveolarechinococcalinfectionwillbereviewedhere.
Theepidemiology,treatment,andpreventionofechinococcosisarediscussedseparately.(See"Epidemiology
andcontrolofechinococcosis"and"Treatmentofechinococcosis".)
CLINICALMANIFESTATIONSTheEchinococcusspecieshavedifferentgeographicdistributionsand
involvedifferenthosts.E.granulosusandE.multilocularisalsodifferintheirclinicalpresentation.(See
"Epidemiologyandcontrolofechinococcosis".)
EchinococcusgranulosusTheinitialphaseofprimaryinfectionisalwaysasymptomatic.Manyinfections
areacquiredinchildhoodbutdonotcauseclinicalmanifestationsuntiladulthood.Latentperiodsofmorethan
50yearsbeforesymptomsarisehavebeenreported.Whileapproximately50percentofdetectedcasesoccur
inasymptomaticpatients,manymorecasesremainundiagnosedorarefoundincidentallyatautopsy.
TheclinicalpresentationofE.granulosusinfectiondependsuponthesiteofthecystsandtheirsize.Small
and/orcalcifiedcystsmayremainasymptomaticindefinitely.However,symptomsduetomasseffectwithin
organs,obstructionofbloodorlymphaticflow,orcomplicationssuchasruptureorsecondarybacterial
infectionscanresult.
Cyststypicallyincreaseindiameteratarateofonetofivecentimetersperyear.However,cystgrowthrates
andtimecoursesarehighlyvariable[1,2].Hydatidcystsmaybefoundinalmostanysiteofthebody,either
fromprimaryinoculationorviasecondaryspread.Theliverisaffectedinapproximatelytwothirdsofpatients,
thelungsinapproximately25percent,andotherorgansincludingthebrain,muscle,kidneys,bone,heart,and
pancreasinasmallproportionofpatients.Singleorganinvolvementoccursin85to90percentofpatientswith
E.granulosusinfection,andonlyonecystisobservedinmorethan70percentofcases(image1).
LiverinvolvementE.granulosusinfectionoftheliverfrequentlyproducesnosymptoms.Therightlobe
isaffectedin60to85percentofcases.Significantsymptomsareunusualbeforethecysthasreachedatleast
10cmindiameter.Ifthecystsbecomelarge,hepatomegalywithorwithoutassociatedrightupperquadrant
pain,nausea,andvomitingcanresult(picture1).
E.granulosuscystscanruptureintothebiliarytreeandproducebiliarycolic,obstructivejaundice,cholangitis,
orpancreatitis.(See"Endoscopicdiagnosisandmanagementofbiliaryparasitosis".)
Pressureormasseffectsonthebileducts,portalandhepaticveins,orontheinferiorvenacavacanresultin
cholestasis,portalhypertension,venousobstruction,ortheBuddChiarisyndrome.(See"EtiologyoftheBudd
Chiarisyndrome".)
Livercystscanalsoruptureintotheperitoneum,causingperitonitis,ortransdiaphragmaticallyintothepleural
spaceorbronchialtree,causingpulmonaryhydatidosisorabronchialfistula.Secondarybacterialinfectionof
thecystscanresultinliverabscesses.(See"Pyogenicliverabscess".)
LunginvolvementThemostcommonsymptomsofpulmonarycysticechinococcosis(CE)describedin
theliteratureincludecough(53to62percent),chestpain(49to91percent),dyspnea(10to70percent),and
http://www.uptodate.com.ezproxy.uniandes.edu.co:8080/contents/clinicalmanifestationsanddiagnosisofechinococcosis?topicKey=ID%2F5669&elapse
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hemoptysis(12to21percent).Lessfrequentsymptomsincludemalaise,nauseaandvomiting,andthoracic
deformations[3,4].Themajorityofchildrenandadolescentswithlunglesionsareasymptomaticdespitehaving
lesionsofimpressivesize,assumedlybecauseofaweakerimmuneresponseandtherelativelyhigher
elasticityofthelungparenchymarelativetoolderpatients[3,5].
Cystscanbreakordevelopsecondarybacterialinfection.Thepresenceofthesecomplicationschangesthe
clinicalpresentation,eitherbycausingnewsymptomsorbyincreasingtheseverityofexistingsymptoms.The
principalcomplicationiscystrupture,withspillingofcystmaterialcontainingfragmentsoflarvaltissueand
protoscolicesintothebronchialtreeorthepleuralcavity.Bronchialtreeinvolvementcanleadtocough,chest
pain,hemoptysis,oremesispleuralcavityinvolvementcancausepneumothorax,pleuraleffusion,or
empyema.Secondarybacterialinfectionofthecystcanmanifestasapulmonaryabscesswithpoorlydefined
margins[68].
Approximately60percentofpulmonaryhydatiddiseaseaffectstherightlung,and50to60percentofcases
involvethelowerlobes[9].Multiplecystsarecommon.Approximately20percentofpatientswithlungcysts
alsohavelivercysts[10].Theratiooflungtoliverinvolvementishigherinchildrenthaninadults[10].
OtherorgansInvolvementoforgansoutsideoftheliverorlungisunusualbutcanleadtosignificant
morbidityandmortality.
Infectionoftheheartcanresultinmechanicalrupturewithwidespreaddisseminationorpericardial
tamponade[11,12].
Centralnervoussysteminvolvementcanleadtoseizuresorsignsofraisedintracranialpressure
infectionofthespinalcordcanresultinspinalcordcompression[13].
Cystsinthekidneycancausehematuriaorflankpain[14].Immunecomplexmediateddisease,
glomerulonephritisleadingtothenephroticsyndrome,andsecondaryamyloidosishavealsobeen
described[15,16].
Bonecystsareusuallyasymptomaticuntilapathologicfracturedevelopsthespine,pelvis,andlong
bonesaremostfrequentlyaffected[17].
Ocularcystsalsooccur[18,19].
CystruptureFeverandacutehypersensitivityreactions,includinganaphylaxis,maybetheprincipal
manifestationsofcystrupture.Hypersensitivityreactionsarerelatedtothereleaseofantigenicmaterialand
secondaryimmunologicreactions.(See"Anaphylaxis:Rapidrecognitionandtreatment".)
OutcomeTheoutcomeofinfectionvarieswiththestageofthedisease.Onestudyreportedonthelong
termoutcomeof33patientswithasymptomaticliverhydatidcysts[1].Thenaturalhistoryofinfectionwas
variable.Approximately15percentofpatientshadundergonesurgery10to12yearsaftertheinitialdiagnosis.
Amongpatientswhodidnotundergosurgery,75percentremainedasymptomatic57percentdidnothavea
changeinthesizeofthecystbyimaging.
Calcificationusuallyrequires5to10yearstodevelopandoccursmostcommonlywithhepaticcystsbutrarely
withpulmonaryorbonecysts.Totalcalcificationofthecystwallsuggeststhatthecystmaybenonviable.
EchinococcusmultilocularisInfectionduetoE.multilocularisisusuallysymptomatic,althoughthe
clinicalmanifestationsarefrequentlynonspecific.Themostcommonpresentingcomplaintsincludemalaise,
weightloss,andrightupperquadrantdiscomfortduetohepatomegaly.Cholestaticjaundice,cholangitis,portal
hypertension,andtheBuddChiarisyndromecanalsooccur.Theclinicalpresentationmaymimicthatof
hepatocellularcarcinoma.
Extrahepaticprimarydiseaseisveryrare(1percentofcases).Multiorgandiseasewasdescribedin13percent
ofcasesinoneseriesinwhichmetacestodesinvolvedthelungs,spleen,orbraininadditiontotheliver[20].
Immunodeficiency,suchasHIVortransplantation,mayacceleratethemanifestationsofalveolar
echinococcosis[21].
Ifleftuntreated,morethan90percentofpatientswilldiewithin10yearsoftheonsetofclinicalsymptoms,and
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virtually100percentwilldieby15years[22].Sincetreatmentwithalbendazolehasbeenintroduced,the
prognosishasimprovedconsiderably.Of117patientsfromFrancewhounderwentlongtermfollowup,the
actuarialsurvivalratewas88percent[23].Lifeexpectancyhasimproved,andpatientsundergoingradical
treatmenthaveabetteroutcomenowthan40yearsago.InSwitzerlandin1970,thelifeexpectancyforan
average54yearoldpatientwithechinococcosiswasestimatedtobereducedby18and21yearsformenand
women,respectivelyby2005,lifeexpectancywasreducedbyapproximately3.5and2.6yearsformenand
women,respectively[23].
DIAGNOSISBothcysticandalveolarEchinococcusmaybediagnosedwithacombinationofimagingand
serology[24].SerologicassaysforE.multilocularisinfectionaremoresensitiveandspecificthanforE.
granulosusinfection.ThefollowingdiscussionprimarilydealswiththemorecommonE.granulosusinfection,
exceptasindicated.Specificdiscussionrelatedtothediagnosisofalveolarechinococcosisfollowsbelow.
E.granulosusNonspecificleukopeniaorthrombocytopenia,mildeosinophilia,andnonspecificliverfunction
abnormalitiesmaybeobservedbutarenotdiagnostic.Eosinophiliaisobservedinfewerthan15percentof
casesandgenerallyoccursonlyifthereisleakageofantigenicmaterial.
ImagingHydatidcystsmaybevisualizedandevaluatedwithultrasonography,computedtomography
(CT),ormagneticresonanceimaging(MRI).Ultrasonographyisemployedmostwidelybecauseitiseasyto
performandrelativelyinexpensive.Portableultrasoundmachinesarefrequentlyusedforscreeningpatientsin
communitiesinwhichE.granulosusinfectionisendemic,sometimeswithconfirmatoryserologictestingto
maximizethediagnosticyield[25].However,CTorMRImaybeusefulforcircumstancesinwhichgreater
anatomicdetailisneededtoestablishthelocationandnumberofcysts,thepresenceorabsenceofdaughter
cysts,andpresenceofrupturedorcalcifiedcysts,whichareimportantforguidingmanagement(table1).
Plainradiographymaydemonstratecalcificationwithinacystbutcannotdetectuncalcifiedcystssoisnot
adequatefordefinitivediagnosticevaluation.
UltrasonographyThesensitivityofultrasonographyforevaluationofEchinococcusis90to95
percent[26,27].Themostcommonappearanceonultrasoundisananechoic,smooth,roundcyst,whichcan
bedifficulttodistinguishfromabenigncyst.Inthepresenceoflivercystmembranes,mixedechoescanbe
confusedwithanabscessorneoplasm.Inthepresenceofdaughtercysts,characteristicinternalseptationcan
beseen.
Shiftingthepatient'spositionduringultrasonographymaydemonstrate"hydatidsand,"whichconsists
predominantlyofhookletsandscolexesfromtheprotoscolices.Hydatiddiseaseisprobableinthesettingof
hydatidsand,innercystwallinfoldings,andseparationofthehydatidmembranefromthewallofthecyst
observedonultrasound[28].
Ultrasoundallowsclassificationofthecyst(s)asactive,transitional,orinactivebasedonbiologicactivity
suchcategorizationsmayinfluencethechoiceoftreatment(image2).Characteristicssuggestiveofaninactive
lesionincludeacollapsing,flattenedellipticalcyst(correspondstolowpressurewithinthecyst),detachmentof
thegerminallayerfromthecystwall("waterlilysign"),coarseechoeswithinthecyst,andcystwall
calcification[29,30].Cystswithacalcifiedrimmayhavean"eggshell"appearance.
Severalotherclassificationsystemsarebaseduponultrasoundappearance(image2):
TheWorldHealthOrganization(WHO)classificationcharacterizescystsbytypeandsize(table1)
[31,32].
TheGharbiclassificationdividescystsintofivetypes[33].TypeIcystsconsistofpurefluidtypeIIhave
afluidcollectionwithasplitwalltypeIIIcystscontaindaughtercysts(withorwithoutdegeneratedsolid
material)typeIVhaveaheterogeneousechopatternandtypeVhaveacalcifiedwall[33].
WHOcategoriesCE1andCE2areactivecysts.TypeCE1isunilocularandtypeCE2ismultilocularwith
daughtercysts(figure1).ClassCE3consistsofcyststhatarethoughttobedegenerating(transitionalgroup).
TherearetwotypesofCE3:CE3a,featuringthe"waterlily"signforfloatingmembranes,andCE3b,whichis
predominantlysolidwithdaughtercysts.Establishingwhetherdaughtercystsarepresentisimportantfor
guidingtreatment.Inaddition,nuclearmagneticresonancehasdemonstratedthatCE3aandCE3bhave
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differentmetaboliccharacteristics[34].ClassesCE4andCE5areconsideredinactive.Byultrasonography,
theyareechogenicwithincreasingdegreesofcalcificationandarenearlyalwaysnonviable.
Itisimportanttoestablishwhetherdaughtercysts(cyststhathaveinvolutedfromthewallandresidewithin
thelargercyst)arepresentandtodistinguishthemfrombroodcapsules,whichareattachedtothewallofthe
largercyst.Thisdistinctionisimportantforguidingtreatment.
Lungcystsmaybesingleormultiple,usuallydonotcalcify,rarelyleadtodaughtercystformation,andmay
containairifthecysthasruptured.
ComputedtomographyManyreportssuggestthatcomputedtomography(CT)hashigheroverall
sensitivitythanultrasonography(95to100percent)[26,27,35].CTisthebestmodefordeterminingthe
number,size,andanatomiclocationofthecystsandisbetterthanultrasoundfordetectionofextrahepatic
cysts.CTmayalsobeusedformonitoringlesionsduringtherapyandtodetectrecurrences(image1)[36].
CTmaybesuperiortoultrasonographyinassessingforcomplicationssuchasinfectionandintrabiliaryrupture
[37].Inonestudy,ultrasoundperformedbetterthanCTintheinvestigationofthecystwall,hydatidsand,
daughtercysts,andsplittingofthecystwall,whileCTwassuperiorfordetectinggasandminutecalcifications
withinthecysts,inattenuationmeasurement,andinanatomicmapping[30,38].
MagneticresonanceimagingMagneticresonanceimaging(MRI)hasnomajoradvantageoverCT
forevaluationofabdominalorpulmonaryhydatidcysts,exceptindefiningchangesintheintraand
extrahepaticvenoussystem[39].MRImaydelineatethecystcapsulebetterthanCTandmaybebetterat
diagnosingcomplications,particularlyforcystswithinfectionorbiliarycommunication.However,MRIis
usuallynotrequiredand,inmostinstances,isnotcosteffective[4042].
BothCTandMRIareusefulindiagnosingechinococcalinfectioninothersitessuchasinthebrain[43].
OtherOtherimagingtechniquessuchascholangiographymaybeindicatedtodiagnosebiliary
involvement,particularlyinpatientswithcholestaticjaundice.Endoscopicretrogradecholangiopancreatography
(ERCP)ormagneticresonancecholangiopancreatography(MRCP)isfrequentlyperformedinpatientswithliver
cystspriortointerventiontoascertainpotentialinvolvementofthebiliarysystemandtoguidethetreatment
approach.(See"Endoscopicdiagnosisandmanagementofbiliaryparasitosis".)
SerologicandantigenassaysSerologyisusefulforprimarydiagnosisandforfollowupaftertreatment
[20,44,45].AntibodydetectionismoresensitivethanantigendetectionfordiagnosisofE.granulosus[20].
LaboratoryserologictoolsDiagnosticserologictechniquesinclude:
Complementfixation
Indirecthemagglutination(IHA)
Indirectimmunofluorescence
Latexagglutination
Doublediffusionimmunoelectrophoresis
Countercurrentimmunoelectrophoresis(CIEP)
Radioimmunoassay(RIA)
Enzymelinkedimmunosorbentassay(ELISA)
Enzymelinkedimmunoelectrodiffusionassay(ELIEDA)
Timeresolvedfluoroimmunoassay(TRFLA)
Immunoblot
Thesensitivityandspecificityofanumberoftheserologictestshavebeencompared(table2).ELISAappears
tobethemostsensitiveandspecificoftheavailableassays[4651]:
Onestudyof79patientswithsurgicallyconfirmedpulmonaryhydatidosisdemonstratedthatIgGELISA
wasthemostsensitive(84percent),followedbyIgMELISA(62percent),passivehemagglutination(61
percent),latexagglutination(58percent),immunoelectrophoresis(51percent),andspecificIgEELISA(44
percent).Thespecificityofalltestswas98to100percent.SpecificIgGELISAhadthehighestnegative
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predictivevalue(93percent)[46].
Onestudycomparedeightserologictestsamong131patientswithE.granulosusinfection[49].IgG
ELISAwasthemostsensitive(94percent)andspecific(99percent)forthemajorityofcystlocations.
Onereportcomparedsixdifferentserologictestsforthediagnosisofcystichydatiddiseaseamong243
patientswithsurgicallyconfirmedinfection[50].ThetwoELISAtestsgaveidenticalresults,witha
sensitivityof89percentforlivercystsand78percentforlungcysts.Inthe39patientswithfalse
negativeresults,theuseofimmunoblottingonlyincreasedtheyieldby8percentELIEDAdidnotidentify
anyadditionalcases.
Themethodsmostfrequentlyemployedforinitialscreeningtests(usingcrudeantigenssuchashydatidfluidor
protoscolexextracts)areELISAandIHA.Confirmatorytestsusingspecificantigenscanthenbeperformed,
suchasimmunoelectrophoresisandimmunoblotting[48].Additionaltestsusingrecombinantorpurified
speciesspecificantigensmayalsobeusefuldiagnosis[52].
Simple,heatstable,inexpensivetests,suchashydatidantigendotimmunoassays,areoftenusedforfield
testingandpopulationscreening[53].ThedotELISAhasareportedsensitivityof88to96percentanda
specificityof90to98percent[5456].
TwomajorE.granulosusantigensutilizedinserologictestingincludeantigen5andantigenB:
Antigen5isamajorparasiteantigenfoundontheinneraspectofthegerminallayer,broodcapsule,and
protoscolices.Onlyafewstudieshaveassessedthevalueoftestsbasedonrecombinantantigen5,
whichhasrelativelylowspecificity,althoughitisusedquiteextensivelyfordiagnosisinclinicalpractice
[48].
AntigenBisahighlyimmunogenicpolymericlipoprotein.StudieshaveshowedthatantigenBshowsa
highdegreeofgeneticvariability[57].Itoffersgreaterspecificitythandetectionofantigen5however,
neitherantigenisspecificforE.granulosuspersedespiteaveryhighspecificityforechinococcal
infection[58,59].
ThesensitivityoftheseantigensinELISAassaysis60to90percentandthespecificityisusually
approximately90percent[60].Thesensitivityinimmunoblotandgeldiffusionassaysisapproximately90
percentwithaspecificityof97to100percent[59,61].Onestudydemonstratedthattheimmunoblotwiththe
antigenBrichfractionwaspositivein92percentofpatientswithE.granulosusbutwasalsopositivein79
percentofpatientswithE.multilocularis[62].Nocrossreactivitywasobservedwithserafrompatientswith
otherparasiticdiseases,malignancies,orhealthycontrols.
Avarietyofissuesinfluencetheratesoffalsepositiveandfalsenegativeserologicresults.First,thereisa
lackofstandardizationamongdifferentlaboratoriesforexample,onestudyshowedthatELISAusingantigen
Bhadasensitivityof63percent,whereasimmunoblottingusingthesameantigenhadasensitivityof80
percent[63].Second,methodsofantigenisolationandpurificationcaninfluencetheresults.Third,serological
assayswithhighsensitivityandspecificityfordiagnosisofcysticechinococcosisinclinicalsettingscanbe
lessusefulinepidemiologicalstudiestheseassaysmaydetectonlyhalfoflivercystsinfieldsurveysandas
fewas20percentoflungcysts[64,65].
Theutilityofserologycanbeimprovedbyusingacombinationoftestsorsequentialtesting[66].Tests
employinganumberofrecombinantantigensarebeingevaluatedtoimprovethesensitivityandspecificityof
thecommerciallyavailableserologictests.Ahighlysensitiveassay,usuallyanELISAorindirect
hemagglutinationtest,iscommonlyusedasaninitialscreen,followedbyahighlyspecificimmunoblotorgel
diffusionassayforconfirmation.Testingforspecificantibodies,suchasspecificIgG1orIgG4ratherthantotal
IgG,mayimprovespecificity[63,6769].
ClinicalfactorsAnegativeserologictestgenerallydoesnotruleoutechinococcosis.Thereisno
consistentcorrelationbetweenserologicresultsandthenumberorsizeofcysts[46].Ingeneral,livercysts
elicitanantibodyresponsemorefrequentlythanlungcysts.Overall,approximately85to95percentofliver
cystsand65percentoflungcystsareassociatedwithpositiveserology,althoughthisvarieswiththespecific
serologictestusedandcystactivity[47].Brain,eye,andspleniccystsoftendonotproducedetectable
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antibodies,whereasbonecystsfrequentlyareassociatedwithpositiveserology.Serologyislesslikelytobe
positivewithcystsatanysiteifthecystsareintact,calcified,ornonviable.
Thelikelihoodoffalsenegativeresultsisvariabledependingonthelesionsiteofthelesionandtheintegrity
andviabilityofthecyst.Antigenantibodycomplexesthat"mop"upallantibodiesmayleadtofalsenegative
reactions.Childrenandpregnantwomenmorefrequentlyhavenegativeserologythanotherpatientpopulations
[9].Falsepositivereactionsaremorelikelyinthepresenceofotherhelminthinfections(suchasTaenia
saginata,Taeniasolium,andparticularlyneurocysticercosis),cancer,andimmunedisorders.
AntigenassaysAvarietyofpurifiedorrecombinantdiagnosticantigenshavebeenevaluated.
Demonstrationofantigensincysticfluidorserumcanalsobeusedfordiagnosisofprimaryinfectionorrelapse
[66,70,71].However,upto50percentofpatientswithechinococcalcystsdonothavecirculatingantigens.
LatexagglutinationoradotELISAtodetectechinococcalantigensfromcystfluidhaveexcellentsensitivity
andspecificity[7274].Antigenassaysandtestsforcirculatingimmunecomplexesarenotwidelyavailablebut
maybecomeusefulsecondarytestsinthefuture[75].
E.multilocularisNonspecificleukopeniaorthrombocytopenia,mildeosinophilia,andnonspecificliver
functionabnormalitiesmaybedetectedbutarenotdiagnostic.Hypergammaglobulinemiaandelevatedserum
IgElevelsarepresentinmorethan50percentofcases.
ImagingThediagnosisofE.multilocularisisgenerallymadebyimagingtechniquesinconjunctionwith
serology.OnultrasoundorCT,thelesionsusuallyhaveanirregularcontourwithnowelldefinedwall,central
necrosis,andirregularintralesionalandwallcalcifications.Theymaybedifficulttodistinguishfromatumor,
butthepatient'soverallconditionisusuallybetterthanwouldbeexpectedforamalignancy.
Obstructionoftheinferiorvenacavaoroftheportalvenoussystemmaybeevident,whichmaybemoreeasily
appreciatedonMRI.Lung,brain,andbonelesionsmayalsobedetected.
SerologySerologictestsaremorereliablefordiagnosisofE.multilocularisinfectionthanforE.
granulosusinfectionsensitivityandspecificityratesare95to100percent[76].AspecificE.multilocularis
antigensuchastheaffinitypurifiedEm2antigenfromAEmetacestodesisoftenusedtheEm2ELISAcan
discriminatebetweenE.granulosusandE.multilocularisin95percentofcases.Serologyusuallyremains
positiveindefinitelyfollowingcompletesurgicalresection,serologymaynormalizewithinafewyears[77].The
Em2ELISAfrequentlybecomesnegativewithinfouryearsofsurgeryandbecomespositiveagaininthe
settingofarecurrence[76,78,79].AnEm2plusELISAassayusesadditionalspeciesspecificantigensithas
sensitivityandspecificityof97and99percent,respectively,andisalsousefulformonitoringrecurrence
followingsurgicalresection[8082].Thesetestsmaynotbereadilyavailableandmaybefoundonlyin
specializedcenters.
ELISAandimmunoblotstudiesusingEm18,an18kDprotoscolexantigen,aresensitiveandhighlyspecies
specific[62,8387].Theantigenisalsousefulfordifferentiatingbetweenactiveandinactiveinfectionandis
usefulforfollowupofpatientsontreatment[82,83,88,89].
Clinicalrecurrenceisfrequentlyassociatedwithrisingserologictiters.IgG1andIgG4antibodiesarethemost
sensitiveisotypesformonitoringsuccessoftherapy[83].
CystaspirationorbiopsyIntheabsenceofapositiveserologictest,percutaneousaspirationorbiopsy
mayberequiredtoconfirmthediagnosisbydemonstratingthepresenceofprotoscolices,hooklets,orhydatid
membranes.Percutaneousaspirationoflivercystcontentsisassociatedwithverylowratesofcomplications,
butthismethodofdiagnosisisgenerallyreservedforsituationswhenotherdiagnosticmethodsare
inconclusivebecauseofthepotentialforanaphylaxisandsecondaryspreadoftheinfection[9093].
Activecystshaveclear,wateryfluidcontainingscolicesandelevatedpressureinactivecystshavecloudy
fluidwithoutdetectablescolicesanddonothaveelevatedpressure[29].Inthesettingoflungcysts,
protoscolicesordegeneratedhookletsmaybedemonstrableinsputumorbronchialwashings.Avarietyof
stainingmethodstodetectparasiticmaterialcanbeused.Stainsforvisualizationofhydatidelementsinclude
RyantrichromebluestainandmodifiedBaxbystain.ZiehlNeelsenstainisalsousefulundergreenexcitation
light(546nm),thehydatidelementshaveafluorescentbrightredappearance[94].
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Ifaspirationisrequired,itshouldbeperformedunderultrasoundorCTguidancecomplicationscanbe
minimizedbyconcurrentadministrationofalbendazoleandpraziquantel[95].(See"Treatmentof
echinococcosis".)
PolymerasechainreactionPolymerasechainreaction(PCR)techniquesarelimitedtoresearchsettings
butmayplayadiagnosticroleinthefuture[96].DNAprobesusingSouthernhybridizationtestsarealsobeing
developed[80].
DIFFERENTIALDIAGNOSISIngeneral,anymassoccupyinglesionmayclinicallyresemblean
echinococcalcyst.Thedifferentialdiagnosisofcysticechinococcosisincludes[97]:
SimplebenigncystPatientswithsymptomaticlivercystmaypresentwithabdominaldiscomfort,pain,
ornausea.LivercystsaredistinguishedfromEchinococcusbyultrasonography.(See"Diagnosisand
managementofcysticlesionsoftheliver".)
HemangiomaHemangiomaisusuallyanincidentalfindingidentifiedinthesettingofradiographic
imagingorlaparotomythemostcommonsymptomsareabdominalpainandrightupperquadrant
fullness.Thediagnosisofhemangiomaisgenerallyestablishedradiographically.(See"Hepatic
hemangioma".)
HepatocellularcarcinomaPatientswithhepatocellularcarcinomaareusuallyasymptomaticintheearly
stagestheyaredistinguishedfrompatientswithcysticEchinococcusbasedonclinicalhistoryand
imaging.(See"Clinicalfeaturesanddiagnosisofprimaryhepatocellularcarcinoma".)
AbscessAliverorlungabscessmayresembleanEchinococcuscystclinicallyandradiographically.
Liverabscessisevaluatedbyaspirationlungabscessmaybeevaluatedviabronchoscopyoraspiration.
Inthesettingofsuspicionforechinococcosis,percutaneousaspirationorbiopsyshouldbereservedfor
situationswhenotherdiagnosticmethodsareinconclusivebecauseofthepotentialforanaphylaxisand
secondaryspreadoftheinfection.(See"Pyogenicliverabscess"and"Lungabscess".)
TuberculosisAcavitarytuberculosislesionmayresembleanEchinococcuscystonradiographic
imaging.Thediagnosisoftuberculosisisestablishedbasedonthepresenceofacidfastbacillionsmear
andculture.(See"Clinicalmanifestationsandevaluationofpulmonarytuberculosis".)
ThedifferentialdiagnosisofalveolarEchinococcusincludes:
CirrhosisPatientswithcirrhosismayhaveanorexia,weightloss,weakness,andfatiguepatientswith
alveolarEchinococcusmayhavemalaise,weightloss,andrightupperquadrantdiscomfort.Thetwoare
distinguishedbasedonradiographicimagingandlaboratorydata.(See"Cirrhosisinadults:Etiologies,
clinicalmanifestations,anddiagnosis".)
Malignancy(hepatocellularcarcinomaorlivermetastases)Patientswithlivertumorsaregenerally
distinguishedfromthosewithalveolarEchinococcusbasedonradiographicimaging.(See"Solidliver
lesions:Differentialdiagnosisandevaluation".)
SUMMARY
E.granulosusinfectionisinitiallyasymptomaticandmayremainsoformanyyears.Subsequentclinical
featuresandcomplicationsdependuponthesiteandsizeofthecyst(s).Theliverandlungsareaffected
inapproximately67and25percentofcases,respectively.Mostpatientshavesingleorganinvolvement,
andasinglecystispresentinmorethan70percentofcases.Thelongtermoutcomeisvariableand
manypatientsremainasymptomatic.(See'Echinococcusgranulosus'above.)
E.multilocularisinfectionismorelikelytobesymptomaticthanE.granulosusinfection.Themost
commonclinicalmanifestationsincluderightupperquadrantdiscomfort,malaise,andweightloss,and
thepicturemaymimicthatassociatedwithhepatocellularcarcinoma.Intheabsenceoftreatment,more
than90percentofpatientswithalveolarechinococcosisdiewithin10yearsoftheonsetofclinical
symptoms.(See'Echinococcusmultilocularis'above.)
Thediagnosisofechinococcosisistypicallyestablishedbyultrasoundimaging(table1andimage2)in
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combinationwithserologictesting(usuallyenzymelinkedimmunosorbentassay[ELISA]).Hydatid
diseaseisprobableinthesettingofultrasounddemonstratinginfoldingsoftheinnercystwall,separation
ofthehydatidmembranefromthewallofthecyst,orhydatidsand.E.multilocularislesionsmayhavean
irregularcontourandmaybedifficulttodifferentiatefromtumor.(See'Ultrasonography'above.)
Diagnosticjudgmentmusttakeintoconsiderationthelimitationsofserologictesting.Thelikelihoodofa
positiveserologydependsoncystlocationandviability.Patientswithlivercystsaremorelikelytobe
seropositivethanpatientswithlungcysts.Serologicassaysarelesslikelytobepositiveinthesettingof
calcifiedornonviablecysts.Inaddition,thesensitivityandspecificityofserologyisgreaterforE.
multilocularisthanforE.granulosus.(See'Serologicandantigenassays'above.)
Percutaneousaspirationorbiopsyshouldbereservedforsituationswhenotherdiagnosticmethodsare
inconclusivebecauseofthepotentialforanaphylaxisandsecondaryspreadoftheinfection.Ifaspiration
isrequired,itshouldbeperformedunderultrasoundorCTguidance.Complicationscanbeminimizedby
concurrentadministrationofalbendazoleandpraziquantel.(See'Cystaspirationorbiopsy'above.)
ACKNOWLEDGMENTTheeditorialstaffatUpToDate,Inc.wouldliketoacknowledgeDr.KarinLederand
Dr.PeterWeller,whocontributedtoanearlierversionofthistopicreview.
UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.
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WorldJHepatol20146:293.
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Topic5669Version12.0
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GRAPHICS
Echinococcalcystoftheliver
Acomputedtomogramshowsamultilocularcystintheliverofapatientwith
hydatiddisease.
Reproducedwithpermissionfrom:SunT.ParasiticDisorders:Pathology,Diagnosis,and
Management,2ndedition.Baltimore:LippincottWilliams&Wilkins,1999.Copyright1999
LippincottWilliams&Wilkins.
Graphic58845Version4.0
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14/2/2015
Macroscopicappearanceofechinococcalcystsin
livertissue
Reproducedwithpermissionfrom:CraigPS,McManusDP,LightlowlersMW,et
al.Preventionandcontrolofcysticechinococcosis.LancetInfectDis2007
7:385.Copyright2007Elsevier.
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WorldHealthOrganizationclassificationofcysticEchinococcus
(CE)andtreatmentstratifiedbycyststage
WHO
stage
CE1
CE2
Description
Stage
Unilocularunechoic
cysticlesionwith
doublelinesign
Active
Multiseptated,"rosette
like""honeycomb"cyst
Active
Preferred
treatment
Alternate
treatment
<5cm
Albendazole
alone
PAIR
>5cm
Albendazole+
PAIR
PAIR
Any
Albendazole+
eithermodified
Modified
catheterization
Size
catheterization
orsurgery
CE3a
Cystwithdetached
membranes(waterlily
Transitional
sign)
CE3b
Cystwithdaughter
Transitional
<5cm
Albendazole
alone
PAIR
>5cm
Albendazole+
PAIR
PAIR
Any
Albendazole+
Modified
eithermodified
catheterization
orsurgery
catheterization
cystsinsolidmatrix
CE4
Cystwithheterogenous
Inactive
Any
Observation
Inactive
Any
Observation
hypoechoic/hyperechoic
contentsnodaughter
cysts
CE5
Solidpluscalcifiedwall
Albendazoleisdosed10to15mg/kgperdayintwodivideddosestheusualdosefor
adultsis400mgtwicedaily.Durationoftherapyisdiscussedinthetext.
PAIR:Puncture,Aspiration,Injection,ReaspirationWHO:WorldHealthOrganization.
Datafrom:
1. JunghanssT,daSilvaAM,HortonJ,etal.Clinicalmanagementofcysticechinococcosis:state
oftheart,problems,andperspectives.AmJTropMedHyg200879:301.
2. BrunettiE,KernP,VuittonDA,WritingPanelfortheWHOIWGE.Expertconsensusforthe
diagnosisandtreatmentofcysticandalveolarechinococcosisinhumans.ActaTrop2010
114:1.
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Ultrasonographicclassificationofcystsduetocysticechinococcus
(PanelCL)Consistsofunilocular,cysticlesion(s)(CL)withuniformanechoiccontent.Thecystwallis
clearlyvisiblelesionsareusuallyroundbutmaybeoval.Iftheselesionsarecausedbycysticechino
anearlystageofdevelopmenttheyareusuallynotfertile.Definitivediagnosiscannotbemadebyult
findingsalone.
(PanelCE1)Consistsofunilocular,simplecystwithuniformanechoiccontent.Thecystwallisvisible
areroundoroval.Cystmayexhibitfineechoesduetoshiftingofbroodcapsulescalledhydatidsand
flakesign").
(PanelCE2)Consistsofmultivesicular,multiseptatedcysts.Thecystwallisnormallyvisiblelesions
oroval.Septationsproduce"wheellike"structures.Thepresenceofdaughtercystsisindicatedbyro
orhoneycomblikestructures.Daughtercystsmaypartlyorcompletelyfilltheunilocularmothercyst.
(PanelCE3)Consistsofaunilocularcystwhichmaycontaindaughtercysts.Anechoiccontentwithde
oflaminatedmembranefromthecystwallmaybevisibleasfloatingmembraneoras"waterlilysign"
indicativeofwavymembranesfloatingontopofremainingcystfluid.Thecystformmaybelessroun
ofdecreasedintracysticpressure.Thecystwhichmaydegeneratefurtherormaygiverisetodaughte
(PanelCE4)Consistsofheterogenoushypoechoicorhyperechoicdegenerativecontentsnodaughter
present.A"ballofwool"signmaybeseenwhichisindicativeofdegeneratingmembranes.Mostcysts
typearenotfertile.Definitivediagnosiscannotbemadebyultrasoundfindingsalone.
(PanelCE5)Cystscharacterizedbyathickcalcifiedwallthatisarchshaped,producingaconeshape
Thedegreeofcalcificationvariesfrompartialtocomplete.Thesecystsarenotfertileinmostcase.D
diagnosiscannotbemadebyultrasoundfindingsalone.
WorldHealthOrganizationInformalWorkingGrouponEchinococcosis(WHOIWGE)standardizedclassificationofE
cysts.PAIR:Puncture,Aspiration,Injection,ReAspiration:AnoptionforthetreatmentofCysticEchinococcosis.
WHO/CDS/CSR/APH/2001.6.WorldHealthOrganization2001.Copyright2001.
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Structureoftheechinococcalcyst
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Sensitivityofserologictestsforechinococcosisatdifferentsites
Siteoflesion
Liver
Sensitivityofserologictests
IgGELISA:80to90percent
IgEELISA:82to92percent
Latexagglutination:65to75percent
Hemagglutination:80to90percent
Immunoblot(usingantigen5and/oraBrichfraction):80to90percent
Enzymelinkedimmunotransferblot:80percent
Lung
IgGELISA:60to85percent
IgEELISA:45to70percent
Latexagglutination:50to70percent
Hemagglutination:50to70percent
Immunoblot(usingantigen5and/oraBrichfraction):55to70percent
Enzymelinkedimmunotransferblot:55percent
Ig:immunoglobulinELISA:enzymelinkedimmunosorbentassay.
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Disclosures
Disclosures:PedroLMoro,MD,MPHNothingtodisclose.PeterFWeller,MD,FACP
Grant/Research/ClinicalTrialSupport:NIH[EGPA(Mepolizumab)].Consultant/AdvisoryBoards:GSK
[EGPA(Mepolizumab)].ElinorLBaron,MD,DTMHEmployeeofUpToDate,Inc.
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,these
areaddressedbyvettingthroughamultilevelreviewprocess,andthroughrequirementsfor
referencestobeprovidedtosupportthecontent.Appropriatelyreferencedcontentisrequiredofall
authorsandmustconformtoUpToDatestandardsofevidence.
Conflictofinterestpolicy
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