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Pediatric Rotation

Kawasakis disease
Kawasaki disease or syndrome is a generalized
vasculitis of unknown etiology and a leading
cause of acquired heart disease among
children living in developed countries.
Affects children (6 months-4 years).

2
Kawasaki disease may be diagnosed with fewer than 4 of
these features if coronary artery aneurysms are detected

KAWASAKI DISEASE: DIAGNOSTIC CRITERIA


Duration of 5 days or more plus 4 of the
Fever
following
1. Conjunctivitis Bulbar, non-suppurative, bilateral

2. Lymphadenopathy Cervical >1.5cm

3. Rash Polymorphous, non vesicles or crusts


4. Changes of lips or Red cracked lips; "strawberry tongue; diffuse
oral mucosa erythema of oropharynx
Initial stage: erythema and oedema of palms
5. Changes of and soles Convalescent stage: peeling of skin
extremities from fingertips
3
The picture shows typical bilateral
conjunctivitis and red lips in a four-year old
boy with Kawasaki Disease.

4
Cervical lymphoadenopathy >1.5 cm

5
Rash: polymorphous, non-vesicle:
(polymorphous exanthem)

6
Changes of lips or oral mucosa

Strawberry tongue Red cracked lips


7
Changes in extremities

Erythema and Peeling of skin from


oedema of palms fingertips
8
Coronary artery aneurysm in KD:
20-25% of untreated
children have it.
The principal cause of
death from Kawasaki
disease is myocardial
infarction.
Coronary angiograms in a six-year-old
boy with Kawasaki disease. (Left) Treatment: IVIg +
Hugely dilated left descending (LAD)
artery with obstruction. (Right) Very aspirin.
dilated right coronary artery (RCA) with
an area of severe narrowing
9
Nephrotic Syndrome:
Periorbital edema.
Scrotal, vulval,
ankle and leg
edema.
Ascitis.
Breathlessness due
to pleural effusion.

10
Napkin Rash: Diper rash
Causes:
- Fungal infections. (satellite
Lesion)
- Bacterial infection. (Staph.
aureus commonly cause papules).
- Contact dermatitis.
How to differentiate by examination???
We look to the skin folds if they spared, this comes
with contact dermatitis because this fold during
wearing diper will not come in contact with the
irritant, while in fungal infection, they will be
affected. 11
12
Erythomelalgia:
a rare condition that is characterized by the
triad of redness, increased temperature, and
intense pain of feet or hands.
Common in 10-20 age group.
Livido reticularis: one of the physical signs.
It is a common cutaneous finding consisting of a
mottled reticulated vascular pattern that appears like
a lace-like purplish discoloration of the lower
extremities. The discoloration is caused by swelling
of the medium veins (not small) in the skin, which
makes them more visible.
13
14
Periorbital cellulitis:
Fever, swelling, tenderness and
discoloration (erythema) of the eyelid.
How can differentiate if it is orbital or periorbital the
eye???
We examine the movement of extra-occular muscles,
if it is affected, then it is orbital.
There is also proptosis and visual acuity.
If the orbital involvement suspected, CT scan has to be
done to confirm the involvement of the eye because
it can result in eye damage.
15
Periorbital cellulites:
It is almost always unilateral.
It can spread from Para-nasal sinusitis or dental
abscess in older children.
Water's view if done, it can show complete opacity in
the sinus, which can result in secondary peri-orbital
cellulites.
Common organism of sinusitis:
- 90% Bacterial: (Streptococcus, H. influenza type B
and non type)
IV antibiotics should be given to prevent spread to
the orbit (orbital cellulites)
16
Waters view: Para-nasal sinusitis

17
Congenital Hypothyroidism:
Causes:
1. Anatomic defect in the
gland.
2. Inborn error in thyroid
metabolism.
3. Iodine deficiency.
Female: male ratio 2:1.

18
Clinical Features:
Protruding tongue. (due to
macroglossia).
Flat nasal bridge.
Umbilical hernia.
Constipation/jaundice.
Hypotonia.

A infant with cretinism, hypotonic posture, coarse facial features, and


umbilical hernia.

19
Diagnosis:
free T4 and TSH.
X-ray findings:
Absence of epiphesis
Treatment:
Eltroxyin (life-long).

20
Ptosis:
Unilateral ptosis:
Horner's Syndrome
& periorbital
cellulites.
Bilateral ptosis (in
children):
Myasthenia
gravis.(affect the
thymus in children).
21
Treacher Collin Syndrome:
Clinical features:
- Down-slanting eye.
- Hypertolirsm.
- Bulky nose.
- Underdeveloped
external ear and low-
set ear.
- Mentally is good IQ.
Treatment: Plastic
surgeries 22
Osteogenesis Imperfecta:
Brittle bone disease

Blue Sclera 23
Cause: Heterogeneous group of disorders of
type I collagen synthesis.
Mode of inheritance:
Dominant in type I and IV.
Recessive in type II and III.
Genetic abnormality in chromosome 7 and 17.

24
Albinism: Achromasia
* It is a congenital
disorder characterized
by the complete or
partial absence of
pigment in the skin,
hair and eyes due to
absence or defect of an
enzyme involved in the
production of melanin.
* AR.
* Associated vision
defects: photophobia,
nystagmus &
astigmatism 25
Types:
- Oculocutanous.
- Ocular.
- Partial.

26
Amigous Genitalia:
It is different if it
is term or
preterm; in
preterm, the
genitalia still
underdeveloped
(not amigous).

27
Scrotal Swelling:
Hydrocele vs. Torsion of testes:

Congested scrotum + empty side.


28
Teratome:

29
Imperforate Hymen:

30
Imperforated anus: Anal atresia

31
Cephalohematoma: firm & contain blood
Caput: soft tissue edema can cross the sutures

32
Bilateral Cephalohematoma:

33
Hydrocephalus:
Congenital H:
commonly
caused by
congenital
infection
(toxoplasmosis)
Acquired H:
meningitis.

34
Vacuum extraction: Chignon

35
Otoscope:

36
Normal Eardrum

Cone of light.
Clear tympanic
membrane.
Handle of mallus.
No blood vessels.

37
Otitis Media:

Acute otitis media:


The earliest visible otoscopic change in acute suppurative otitis media
consists of redness, edema, and bulging and loss of light reflection. 38
Otitis Media (OM):
At 6-12 months of age.
Infant and children are prone to OM, Why????
Because of short, horizontal and poorly
functioning Eustachian tube.
Clinical features:
- Pain in ear.
- Fever.
- Irritability.
- Pulling at the affected ear.
- Occasionally, there is perforation with pus.
39
Perforation
Otorrhea:

Otorrhoea:
This patient has a long-standing perforation in the right tympanic membrane
developed an acute otitis media following a severe viral upper respiratory
infection.
A mixture of creamy white purulent material and clear mucus can be seen
draining into the external canal through the large antero-inferior perforation.
40
Glue ear (serous otitis media)
Recurrent ear infections
otitis media with
effusion.
Tympanic membrane:
dull, retracted, often
with a fluid level.
Resolve spontaneously.
It is the most common
cause of conductive
hearing loss. 41
1 year old girl is brought to the clinic by her mother with complaints of
fever, irritability and general fussiness. The mother noticed that her
daughter tends to hold and pull on her left ear more often than
expected. Review of systems revealed she has developed rhinorrhea
and nasal congestion for the past few days. Her temperature today is
102F. Otoscopic examination reveals bulging and erythematous
tympanic membrane with decreased mobility on air compression.

Which of the following is the most likely organism responsible for the
patients condition?
A) Hemophilus Influenza
B) Streptococcous pneumoniae
C) Moraxella catarrhalis
D) Pseudomonas aeruginosa
E) Group A Streptococcus
F) Staphylococcus aureus
G) Respiratory syncytial virus

42
Tonsillectomy:
Indications of tonsillectomy:
- Recurrent tonsillitis.
- Paratonsillar abscess (Quinsy).
- Obstructive sleep apnea.

43
Anopthalmia:

44
Micro-opthalmia:

45
Cataract:

46
Gonorrhea:
Copious, yellow
eye secretion.
Transmitted
form the
mother during
delivery .

47
Visual Impairment present w/
Loss of red reflex from cataract.
White reflex in pupil may be due to:
Retinoblastome.
Cataract.
Retinopathy of prematurity.
Not smiling at 6 weeks.
Nystagmus.
Squint.
Photophobia.
48
Preauricular Tag:
If present,
Renal U/S
has to be
done.

49
Hemangioma
On the eyelids :
fades within 1
year.
On the neck: do
not disappear &
covered with
the hair.

50
Capillary Hemangioma: (stork bites)
Pink macula on the upper eyelids, mid-forehead
and nape of the neck.
Arises from the distention of the dermal
capillaries.
Cavernous Hemangioma: (Strawberry naevus)
Not present at birth (appears in 1st month)
More common in preterm.
Increases in size until 3-9 months, then starts to
regress gradually.
No treatment required unless interferes with
vision.
51
Cleft lip and palate:

52
Natal teeth:
1. May present at
birth.
2. Need to be
removed
to avoid aspiration

53
Ranula: subglossal cyst.

54
Tongue tie:

55
Oral Thrush It is caused by the
overgrowth of the yeast
candida albicans.
Found normally in the
mouth or GI tract but their
amount depend on the
immune system and some of
the good normal flora that
prevent them from growing.
- Can overgrow after receiving
antibiotic that kills also the
good bacteria
- In infants whose immune
system still underdeveloped.
56
Facial nerve palsy:

57
Sebaceous gland hyperplasia:
White spots on the nose

58
Milia: white pimples on the cheeks
and nose.

59
Jaundice: direct vs. indirect.

Direct: greenish Indirect: Orange


60
Cystic Hygroma

61
Goiter:

Most common
cause in children
Hashimotos
thyroiditis.

62
Amniotic bands:
A remnant of
amniotic sac
that can
constrict
limbs and can
lead to
gangrene and
amputation.

63
Polydactyl >5 fingers

6 fingers

64
Zygodactyls: webbed fingers.
Syndactyly: joined fingers.

65
Sucking Blisters:

Commonly
in post-term
newborn
due to
sucking in
utero.

66
Equinovarus:

67
Subcutaneous nodules in
Rheumatic fever

Painless, pea-sized,
hard.
Mainly on extensor
surface.

68
MORO reflex: ABSCENCE
Erbs palsy.
Fractured
limb.

69
Exomphalos (Omphalocele):
Abdominal contents
protrude through the
umbilical ring.
Covered with
transparent sac
(amniotic membrane
and peritoneum.
Associated with other
major congenital
abnormalities.

70
Gastroschisis:
Bowels protrudes through a
defect in the anterior abdominal
wall adjacent to the umbilicus.
No covering sac.
Not associated with other
congenital abnormalities.
Antenatal U/S:
Will shows free loops of small
bowel in the amniotic fluid.
71
Prune Belly Syndrome: absent
musculature syndrome
No anterior
abdominal wall
muscles.
Frequently,
associated with large
bladder & dilated
ureters.

72
Exstrophy of the bladder:

Due to failure of
fusion of
infraumbilical midline
structure.

73
Meningomyelocele:
Potential problems associated with MMC:
- Urinary retention w/ overflow.
- Nephropathy due to VUR.
- Constipation w/ fecal overflow.
- Spastic diplagia.
- Hydrocephalus.
- Meningitis.
Antenatal diagnosis: serum fetoprotein
and amniotic fetoprotein+ Anomaly scan.
74
Mongolian spots:
Dermal melanocytic nevi

Management: none only reassure the parents


Classic site: over the sacrum. 75
Birth Injury:
Etiology:
Malposition:
Too large for the pelvic outlet
(e.g. Macrosmnia).
Instrumental deliveries (forceps
or vacuum).

77
Soft tissue injuries:
Caput succedaneum.
bruising and edema of presenting part that can
cross the sutures.
Resolve in a few days.
Cephalohematoma.
Hematoma from bleeding below the periosteum,
confined within the margins of skull sutures. (esp.
parietal bone).
the center feels soft.
resolves over several weeks.
occasionally accompanied by linear skull fracture.
78
79
Chignon:
bruising and edema from vacuum delivery.
Bruising:
to the face: face presentation.
to genitalia and buttocks: breech presentation.
suckling bruise: post-term and in term infant.
Subaponeuroticn hemorrhage.
uncommon.
diffuse, boggy swelling of scalp.
can cause severe blood loss hypovolemic
shock.
Forceps marks: 80
Nerve Palsies:
Brachial nerve palsy: traction of brachial
plexus nerve root.
1. Upper nerve root (C5-C6) injury Erbs
palsy
May accompanied by phrenic nerve palsy
elevated diaphragm.
Affected arm lies straight, limp and with hand
pronated and the fingers flexed (waiters tip
position).

81
2. Lower nerve root Klumpkes palsy.
If not resolved within 6 weeks, should be
referred to an orthopedic surgeon (90%
recover within 2 yrs.
Facial nerve palsy:
Compression by forceps blades.
Or against mathers ischial spine.
Unilateral .
Facial weakness on crying.
Eye remain open. (Methyle cellulose may be
needed)
Usually transient.
82
Fractures:
Clavicle:
Usually from Shoulder dystocia.
Presentation:
Snap may be heard at delivery,
Reduced arm movement.
Lump from callus formation over clavicle at several
days of age.
Excellent prognosis.

83
Humerus/femur:
Usually mid-shaft.
Breech presentation: femur
Shoulder dystocia: humerus.
Presentation:
Deformity.
Reduced movement of the limb.
Pain on movement.
Heals rapidly with immobilization.

84
Cerebro-vascular disease:
Cerebral hemorrhage:
1. Extra (epi) dural H.
2. Subdural H.
3. Subarachnoid H.
Stroke.

85
Extradural Hemorrhage:

Head trauma skull fracture and arterial or


venous bleeding into the epidural space.
Diagnosis: CT scan.
Management:
- Correction of hypovolemia.
- Surgical evacuation of the hematoma 86
Subdural Hemorrhage:
Results from tears in the
veins in the subdural
space.
Characteristic lesion of
non-accidental injury (e.g.
shaking). Shaken baby
syndrome.
Retinal hemorrhage.
Seen after fall from a
height. 87
Subarachnoid Hemorrhage:
More common in adult.
Presentation: acute
onset headache, neck
stiffness and occasio.
fever.
Seizures & coma.
Retinal hemorrhage.
Due to aneurysm or AV
malformation.
88
Intracerebral hemorrhage:

89
Stroke:
Causes:
- Vascular.
- Thromboembolic.
- Hemorrhagic.
Anterior circulation >
posterior circulation.

90
Neural tube defects
Anencephaly:
Failure of the
development of most
of the cranium &
brain.
Affected children are
stillborn or die after
birth.
Detected on antenatal
care termination.
92
Encephalocele:

Herniation of the
brain and meninges
through a midline
skull defect.

93
Spina Bifida Occulta:
Failure of the fusion of
the vertebral arches.
Often incidental findings
on x-ray.
Associated overlying skin
lesion (hair tuft).
Usually in lumbar region.
May be tethering of the
cord neuro. deficit
of bladder & lower limbs.
94
Meningocele:

Good prognosis.
Surgical repair.

95
Meningomyelocele:
Can cause:
- Paralysis of legs.
- Loss of sensation.
- Neurogenic bladder.
- Neurogenic bowel.
- Scoliosis.
- Hydrocephalus. (Arnold-
chairi malformation).

96
Hydrocephalus:
Non-communicating (obstructive): within
ventricular system or aquaduct.
Communicating: at arachnoid villi, the site of
absorption of CSF.
Clinical features:
1. HC disproportionally large.
2. Skull sutures separated.
3. Anterior fontanelle bulge.
4. Scalp veins are distended.
5. Advanced sign: sun-setting sign. 97
Sun-setting sign:
Downward deviation of
the eyes.

Management:
Insertion of ventriculo-peritonial shunt.
Complication: infection or blockage.
98
INSTRUMENTS:
Inhaler/spacer device:

100
Peak flow meter

101
102
Pulse Oximeter

103
Nebulizer Mask

104
O2 Mask

105
NG Tube:
Nasogastric
tube

106
NJ Tube:
Nasojeujenal
Tube

107
Suctions:

108
Foleys Catheter:

109
Syndromes:
Downs Syndrome
Trisomy 21.
Incidence: 1 in 650.
Genetics:
- Non-disjunction. (Most common) related to maternal age;
however, most babies are born to young mothers.
The risk of recurrence in this type (1 in 200)
- Robertsonian translocation: unbalanced (parental
chromosomal analysis is essential because one of them can be
carrier of balanced translocation)
The risk of recurrence is 10-15% (if mother carrier).
- Mosaicism: some cells normal and some have trisomy 21
111
Screening test:
Blood test which measures certain chemicals
in the mother: triple test (15-22 wks): Free -
HCG, fetoprotein and estriol
Nuchal thickening on U/S: thickening of the
fat pad at the back of the neck (10-14 wks).
Not diagnostic only estimate the level of risk.
Amniocentesis or chorionic villous sampling
(done for high risk): chromosomal study.
Diagnostic
112
General Appearance:

Generalized
hypotonia (the
majority are
hypoptonic)
Marked head
lag

113
Facial features:

The pupil demonstrate a light smudgy opacity in the iris,


called a Brushfield spot + prominent epicanthic fold
(a fold of skin crossing the inner edge of the palpebral
fissure).
114
Up-slanting eye.
Flat nasal bridge.
Small low set
ear.
flat facial profile.
Flat occipt
(barchycephaly).
3rd frontanelle.
Short neck.
Protruding
tongue (due to
small oral cavity).

115
Hand Features:

Brachydactyly: short broad hands


Clinodactyly: incurved little finder
116
Foot features:

Wide
sanddle gap
(between 1st
and 2nd toes).

117
Immediate medical complication:
Congenital heart defects (40%). life threatening
mainly AVSD, ASD, VSD and tetralogy of Fallot.
Duodenal atresia: projectile vomiting. life
threatening

Hirschsprungs disease. a developmental disorder


of the enteric nervous system (Both the myenteric
(Auerbach) plexus and the submucosal (Meissner)
plexus are absent, resulting in reduced bowel
peristalsis in the distal colon resulting in a functional
obstruction.
118
Duodenal Atresia:

Double bubble sign on x-ray.


119
Hirschsprungs disease.

120
Hirschsprungs disease.
Absence of ganglion cells from the myenteric
and submucosal plexuses in the large bowel
(esp. rectosigmoid)
Presentation: usually in neonates with
intestinal obstruction. (failure to pass
meconium in the first 24 hrs of life + abdo.
distention + bile-stained vomiting).
In later childhood: chronic constipation +
abdo. distention.
Treatment: surgery 1st colostomy then
anastomosis btw normal bowel and anus. 121
Late medical complications:
Delayed development.
- Motor milestones.
- Moderate to severe learning difficulties.

Small stature.
risk of infections.
Hearing problems: deafness either sensorineural or
conductive.
Visual problems: Cataract, squint, myopia.
risk of leukemia and tumors.
Risk of atlantoaxial instability.
Hypothyroidism, DM and celiac disease.
Epilepsy.
Alzheimers disease.
122
Causes of premature death:
Cardiac defects.
Respiratory disorders.
Alzheimer's disease.
Patient with DS should be screened for:
Hypothyroidism.
Hearing problem because they can result in
developmental delay.

123
Turners Syndrome:
Chromosomal disorder.
Monosomy:X 45, Karyotype.
(missing X or Y)

Female.
Turners-like Syndrome:
Noonan Syndrome.

124
General features:
Short stature (they use for them special growth
chart).

125
126
Short stature.
Webbed-neck.
Low-hair line.
Shield-shaped
chest.
Wide-spaced
nipple.
Wide-carrying
angle.
127
Lymphodema:
Lead to webbed neck
in the Turners
Syndrome.
Any newborn with
swollen feet, hands
and postnuchal
should have
karyotyping.
Antenatal detection
by U/S: lymphoedema
& cystic hygroma 128
Cardiovascular malformations:
Coarctation of aorta
Patient need
echo for
screening.
Patient
asymptomatic
until the
closure of PDA.

129
Coarctation of the aorta:

An x-ray shows
rib notching.

130
Noonans Syndrome
Turner-like Syndrome.
Autosomal Dominant genetic disorder.
Mutation in 4 genes:
- PTPN11 gene. (50%)
- KRAS gene.
- RAF1 gene
- SOS1 gene
With: Short stature, dysmorphic face, webbed
neck and cardiac defect (esp. pulmonary
stenosis). 131
132
Characteristic facial features

133
Other manifestations

134
Achondroplasia:
Autosomal dominant:
Spontaneous new mutation.
Disproportional short
stature. (Specifically the limbs
are disporportionately small
when compared to the trunk.
Within the limbs the proximal segment is shorter causing
rhizomelia).

135
136
Other symptoms are as listed below:
Abnormal hand appearance with persistent space
between the long and ring fingers
Fingers are short and broad (stubby appearance)
Bowed legs
Decreased muscle tone
Prominent forehead (frontal bossing)
The mid-face underdeveloped (maxillary hypoplasia)
Short stature (significantly below the average height
for a person of the same age and sex)
Spine curvatures called kyphosis and lordosis
137
X-rays Findings:

Long bones are relatively


plump and short.
Proximal humerus and
femur in infancy have
distinctive rectangular or
translucent ovals that
change by 2 years.
Fibulas tend to be longer
than tibias.

138
Diagnosis:
The diagnosis of achondroplasia can also be
made prenatally either by ultrasound or by
prenatal DNA testing. (A sample of tissue from a
fetus is obtained by either chorionic villa sampling
(CVS) or by amniocentesis).

139
Associated Medical problems:
Foramen magnum stenosis cord
compression or compressive myelopathy
sudden death.
Sleep apneas.
Hydrocephalus.

140
Cornelia De nyle Syndrome
Confluent eyebrows
(synophrys)
Long curly eyelashes.
Low anterior and
posterior hairline.
Underdeveloped orbital
arches.
Long philtrum.

141
Congenital heart disease:
1% of newborn infants.
10% of all congenital anomalies.
Risk factors:
- Maternal DM.
- Genetic syndromes (e.g. NS).
- Hx of previous infant with CHD.

142
Neonatal presentations in CHD:
Shunt (Respiratory distress).
Cyanosis with blood flow.
Cyanosis with N/ blood flow.
Shock.

143
Shunt: Respiratory Distress
VSD, PDA, ASD: rarely cause symptoms in 1st
few days unless (+1 lesion).
AV canal, aortico-pulmonary window, TAPVR
w/o obstruction.
TAPVR w/ obstruction: respiratory signs occur
immediately after birth. (require ER
intervention).
Truncus arteriosus.

144
Ventricular Septal Defect:

145
VSD
The most common cardiac defect.(25-40%)
Can be isolated or in combination with other
congenital anomalies.
The clinical presentation depends on the
severity of the shunt (size of defect & status of
the pulmonary vasculature).
- Small defect: no symptoms usually.

146
Left-to-right shunting:

1.LV volume
overload.
2. pulmonary blood
flow.
3. systemic cardiac
output

147
Atrial Septal Defect:

148
ASD:
4 Types:
1. Ostium secundum defect.
2. Ostium primum defect.
3. Sinus venosus defect.
4. Coronary sinus septal defect.

149
Patent Ductus Arteriosus:
Patent ductus
arteriosus (PDA) is
the persistence (>
10 days after birth)
of a normal fetal
structure between
the left pulmonary
artery and the
descending aorta

150
PDA:
5-10% of all CHD.
The ductus arteriosus is a blood vessel in an
unborn baby (fetus) that connects the
pulmonary artery, which carries blood to the
lungs, and the aorta, which carries blood to
the body. This allows blood flow to bypass the
lungs. (The baby does not need blood to flow
through the lungs before it is born, because
the baby is not breathing air.)
151
PDA:
Normally, this blood vessel closes shortly after
birth; when it does not close, it is known as a
patent (open) ductus arteriosus. Extra blood
from the aorta then flows into the lungs and
back to the heart, rather than out into the
body. In severe cases, this can stress the heart,
increase pressure in the pulmonary artery that
carries blood to the lungs, and cause fluid to
build up in the lungs.
152
Clinical presentation:
Small PDA: no symptoms.
Mild-to-moderate shunt: horse
crying, cough, LRTI and atelectasis, or
pneumonia.
Large PDA: CHF with dyspnea and
poor weight gain or failure to thrive

153
Complications of untreated PDA
include: bacterial endocarditis, late
congestive heart failure (CHF), and
the development of pulmonary
vascular obstructive disease.

154
Total anomalous pulmonary
venous return (TAPVR):

155
TAPVR:
The pulmonary veins, which normally drain
into the LA, in this condition, they drain into
either systemic vein (e.g. SVC) or directly into
the RA.

Drain into
RA Normal
drainage

156
TAPVR:
An atrial septal defect or patent foramen
ovale, considered part of the complex, serves a
vital function in this condition for maintaining
left ventricular output.
Clinical presentation:
- Patients without pulmonary venous
obstruction failure to thrive, dyspnea on
exertion and recurrent infections.
- Patient with obstruction severe cyanosis
and significant respiratory distress.
157
Truncus Arteriosus:

158
TA:
One valve: truncal valve.

159
Management:
O2 is rarely helpful and may worsen
shunting.
Correct anemia (if present), limit sodium
and fluid intake, administer diuretics,
optimize nutrition.
Early surgery may be necessary for some.

160
Radiology:

161
Respiratory System: Normal CXR
Children are not just small adults and this is important when
interpreting a child's X-ray. Such matters as identification of
the patient are still important. A child, especially if small, is
more likely to be unable to comply with instructions such as
keeping still, not rotating and holding deep inspiration.
Technical considerations such as rotation and under or over
penetration of the film still merit attention and they are more
likely to be unsatisfactory. A child is more likely to be laid
down and have an AP film with the radiographer trying to
catch the picture at full inspiration. This is even more difficult
with tachypnoea.

162
Assess lung volume
Count down the anterior rib ends to the one that meets the
middle of the hemidiaphragm. A good inspiratory film should
have the anterior end of the 5th or 6th rib meeting the middle
of the diaphragm. More than six anterior ribs shows
hyperinflation. Fewer than five indicates an expiratory film or
underinflation.

Tachypnoea in infants causes trapping of air. Expiration


compresses the airways, increasing resistance and, especially
under 18 months, air enters more easily than it leaves and is
trapped, causing hyperinflation. Bronchiolitis, heart failure
and fluid overload are all causes.

With underinflation, the 3rd or 4th anterior rib crosses the


diaphragm. This makes normal lungs appear opaque and a
normal heart appears enlarged. 163
Positioning
Sick children, especially if small, may not be
cooperative with being positioned. Check if
the anterior ends of the ribs are equal
distances from the spine. Rotation to the right
makes the heart appear central, and rotation
to the left makes the heart look large and can
make the right heart border disappear.

164
Lung density
Divide the lungs into upper, middle, and lower zones and
compare the two sides. Infection can cause consolidation, as
in an adult. Collapse implies loss of volume and has various
causes. The lung is dense because the air has been lost. In
children, the cause is usually in the airway, such as an
intraluminal foreign body or a mucous plug. Complete
obstruction of the airway results in reabsorption of air in the
affected lobe or segment. Collapse can also be due to extrinsic
compression such as a mediastinal mass or a pneumothorax.

Differentiating between collapse and consolidation can be


difficult or impossible, as both are denser. Collapse may pull
across the mediastinum and deviate the trachea. This is
important, as pneumonia is treated with antibiotics but
collapse may require bronchoscopy to find and remove an
obstruction. 165
Pleural effusion
The features of effusion have already been
noted for adults. In children, unilateral
effusion usually indicates infection whilst
bilateral effusion occurs with
hypoalbuminaemia as in nephrotic syndrome.

Bronchial wall thickening is a common finding


on children's X-rays. Look for "tram track"
parallel lines around the hila. The usual causes
are viral infection or asthma but this is a
common finding with cystic fibrosis.
166
Heart and mediastinum
The anterior mediastinum, in front of the heart, contains the
thymus gland. It appears largest at about 2 years old but it
continues to grow into adolescence. It grows less fast than the
rest of the body and so becomes relatively smaller. The right
lobe of the lung can rest on the horizontal fissure, which is
often called the sail sign.

Assessment of the heart includes assessment of size, shape,


position and pulmonary circulation. The cardiothoracic ratio is
usually about 50% but can be more in the first year of life and
a large thymus can make assessment difficult, as will a film in
poor inspiration. As with adults, one third should be to the
left of centre and two thirds to the right. Assessment of
pulmonary circulation can be important in congenital heart
disease but can be very difficult in practice.
167
Head and Neck X-ray

168
Case:1 Thickened pre-vertebral soft tissue at the level
of C1-C4(space normally =/< vertebral width)
D.Dx: viral croup, epiglottitis or abscess

Pre-vertebral or retropharyngeal abscess. 169


Epiglottitis:
Thumb sign.
because of
H. influenza
vaccine.

170
Croup:
Narrowing of
the trachea.

171
Normal CXR

172
Pneumonia:

173
Normal Thymus:

174
Bronchiectesis:
Frontal chest x-
ray in cystic
fibrosis shows
diffuse interstitial
disease
with
bronchiectasis
and nodular
densities of
mucoid impaction

175
Pneumothorax:

176
Pneumothorax:
presence of free air in the intrapleural space (between parietal and viseral
pleura)
types:
Spontaneous (primary) pneumothorax.
Due to non-traumatic rupture of alveolous, bronchiole, or bleb
No underlying pulmonary pathology present
Often due to rupture of small subpleural cyst or bleb
Occurs primarily in young, healthy patients (ages 15-40yo), with tall
and thin body habitus
Secondary spontaneous pneumothorax from underlying lung pathology
COPD, asthma, cystic fibrosis,
infections: necrotizing bacterial pneumonia, TB, fungal pneumonia,
PCP
neoplasm
Interstitial lung disease: sarcoid, pulmonary fibrosis, pneumoconioses
177
Trauma
broken rib, ruptures bronchus, blunt trauma, penetrating wound, etc.
Iatrogenic
central line placement, etc
seen in ~3% of ICU patients
"Tension pneumothorax"
Air continues to enter pleural space through broncoalveolar disruption
and becomes trapped via ball-valve mechanism
Intraplural pressure then increases
venous return to right heart decreases, resulting in decreased cardiac
output
mediastinum can shift toward uninvolved side, which further
interferes with right atrial filling. May note tracheal deviation as well.
V/Q mismatch results in hypoxia

178
Lung abscess: Air-fluid level.
Causative organisms:
- Staph.
- H. influenza.
- Klebsiella pneumonia.
- Mycobacterium TB.
- Anarobes.
Complication:
- Bronchectasis.
- Sepsis.
- Empyema.
179
Meconium Aspiration:
Post-term neonate.

180
Bronchopulmonary Dysplasia:
Hx of previous prolonged Ventilation
Chest x-ray findings:
1. ET tube indicates
persistant ventilatory
requirement.
2. Cystic changes.
3. Thickening of
bronchial wall.
4. Diffuse reticular
opacity.
5. Interstial edema.

181
Pulmonary Interstitial Eymphezyema.
Hx of being on ventilation for 3 days.

182
Respiratory Distress Syndrome
(Hyaline membrane disease):

Ground glass
appearance + air
bronchogram

183
Endotracheal (ET) tube:

184
ET tube:

185
Dextrocardia:

186
Diaphragmatic hernia:

187
Diaphragmatic hernia:
Common in LT-side and posterior-lateral (Bochdalek
hernia)

Deviation of the
trachea and
mediastinum.
Absence of the
diaphragm.

188
Congenital lobar emphysema:
Congenital lobar emphysema
of the right upper and
right middle lobes. Note
hyperinflated and
hyperlucent right
lung, shift of the
mediastinum to the left,
herniation of the
emphysematous lung to the
left, and atelectasis of the
left lobe.

189
Round (Spherical) Pneumonia:

190
CT scan with brain abscess:

Pulmonary TB
brain abscess
constructive
hydrocephelus.
Ring enhancing
lesion.

191
Brain CT with multiple
calcification:

CMV infection.

192
GIT system: Normal Abdo. x-ray

193
Tracheo-esophygeal Fistule:
This newborn had feeding
difficulty and respiratory
distress after birth. A chest
x-ray is shown. The
oro-gastric tube tip is
consistent with a location
in the right lower lobe
bronchus. There is
consolidation of the right
lung. There is gas in the
Wide-caliber feeding intestinal tract. The
tube not entering findings are consistent
through the esophagus with a double tracheo
due to blinded end and -esophageal fistula
fails to reach the or an H-type fistula.
stomach.
194
Esophageal atresia:
Usually associated with T-E fistula.
Associated with polyhydramnious during
pregnancy. (bcz fetus doesnt swallow
amniotic fluids).
Clinical presentation (if not detected at
birth):
- Persistent salivation.
- Drooling from the mouth.
- Choking and cyanotic episodes.
50% of cases have other congenital abno.
(e.g. VACTERL association) 195
Small bowel obstruction:
Clinical presentation:
- Persistent, bile-stained vomiting bilious. (unless
above the ampulla of Vater)
- Abdominal distention (the more the distal the
obstruction).
Causes:
- Atresia or stenosis of duodenum (Downs Syn.).
- Atresia or stenosis of jejunum/ileum.
- Malrotation with volvulus (can lead to infarction).
- Meconium ileus/plug.
196
Duodenal Atresia:
An emergency

Double
Bubble
sign

197
Intussusceptions:
Intussusceptions is the invagination of a
proximal segment of bowel into the distal
bowel lumen.
The commonest occurrence is a segment of
ileum moving into the cecum & colon through
the ileo-caecal valve.
Any age but commonly occurs in the 2 month
to 2 years (peak 5 to 9 months).
M:F = 2:1.
The commonest cause of intestinal obstruction
in infants after neonatal period. 198
Clinical Presentation:
Severe colicky pain and pallor (esp. around the
mouth) and drawing up the legs.
Sausage-shaped mass in the abdomen.
Passing of redcurrant jelly stool (blood and
mucus in the stool).
Abdo. Distention and shock.

199
Intussusceptions
Plain abdominal X-ray
Performed to exclude perforation or bowel
obstruction.
A normal AXR does not exclude intussusception.
Signs of intussusception on a plain Xray
include:
Target sign - 2 concentric circular radiolucent lines
usually in the right upper quadrant.
Crescent sign - a crescent-shaped lucency usually
in the left upper quadrant with a soft tissue mass.
200
Intussusceptions: AXR

201
Intussusceptions: U/S

Doughnut
Sign

202
Treatment:
Rectal air insufflation: if no peritonitis.
Surgical reduction:
If there is perotinitis.
Failure of rectal air insufflation.

203
Intestinal Obstruction:

204
Perforation:

Air under the


diaphragm
(pneumoperitonium)

205
Pyloric Stenosis:
Clinical presentation:
At 2-7 weeks of age.
Boy. (esp. first born)
Projectile vomiting.(gradual progressive).
The child always hungry.
O/E: small abdominal mass-like olive with visible
gastric peristalsis.
Investigation:
Lab: Low HCL metabolic alkalosis and K+. (loss of gastric HCL).
Imaging: U/S.
Treatment: fluid and electrolytes rep. + pyloromyotomy. 206
Pyloric Stenosis:

207
MSK
Multiple fractures
with callus formation:
- Child abuse.
- Osteogenesis
imperfecta. (could be
intrauterine
fractures).

208
209
Types of Child abuse.
Physically abused.
Neglect.
Emotional abuse.
Sexual abuse.
Non-accidental poisoning.
Fabricating or induced illness.

210
Presentation of physical abuse:
Bruises: most common presentation.
Non-accidental head injuries: vigorous shaking
subdural hemorrhage.
apnea hypoxic-ischemic damage
- Retinal hemorrhage.
Abdominal injuries: visceral injuries.
Burns.
Fractures: ribs, if trauma & bone disease
excluded. (due to squeezing & usually
posterior)
211
Battered Child Syndrome:
Child abuse:
Posterior rib usually.

212
Rickets:

Active: no
line of
calcification
Healing
Healed

213
Active:
Healing: line of
calcification
Healed

214
Active:
Healing
Healed:
well-
demarcated

215
Urology:
IVU:

Duplex ureter:

217
Horseshoe kidney

218
MCUG:
MCUG procedure.
Posterior urethral
valve.
Male present with
interrupting poor
urine stream.
On U/S: bilateral
hydronephrosis &
hydroureter.
219
MCUG procedure.
Bilateral
vescioureteric reflux
with ureteric
dilatation.

220
Nephroblastoma: Wilms tumor
The commonest renal
tumor in childhood.
80% present before 5 yrs
of age.
Abdominal mass,
incidentally.
5% bilateral.
Treatment: chemo
nephrectomy chemo..
Prognosis: good. 221
Neuroblastoma:

222
Growth Charts:
GC#1:
Steroid administration(typical) in
nephrotic syndrome because of high
dose requirement.

224
225
GC#2:
Child abuse due to:
- Sick child with chronic illness.
- Postpartum depression.

226
227
GC#3:

Craniopharyngioma (a tumor
affecting the pituitary region):
GH

228
229
GC#4:
Deceleration in height and acceleration of
weight: Endocrine:
- Hypothyroidism.
- Corticosteroid use.
There is improvement with treatment (Eltroxine)
or stop of the steroid use.

230
231
GC#5:
Familial: following growth centile within
predicted range for parental height.
IUGR or extreme prematurity.

232
233
GC#6:

234
235
GC#7:

236
237
GC#8:

238
239
E-loop Questions:
1.HSP:
Skin rash was noted when a 11-years
old girl admitted with generalized acute
abdominal pain:

What the Dx? HSP


Two possible causes of the abdominal
pain.
- Gut wall edema and hemorrhage.
- Acute intussusception.
Four potential complication of the
condition:
1. Arthropathy.
2. Renal disease: nephritis, nephrotic
syndrome, renal failure.
3. CNS disease: seizure, paresis, rarely
coma.
4. Testicular torsion.

241
HSP:
Medium-sized BV vasculitic condition often
preceded by URTI.
RF and ANA: -ve.
LL extensor surface are most commonly
affected.
Arthritis/arthralgia: transient and non-
migratory.

242
2.Intussusception
Name this radiological
procedure?
Barium enema
What is the Dx?
Intussusception
Contraindication to
performing this
procedure?
Evidence of perforation
and peritonitis.

243
3.Congenital Adrenal hyperplasia:

244
4.Twin-twin transfusion
These twins were born at a gestation of 37 weeks:

Explain the different appearance?


Twin-twin transfusion
What is the important initial investigation?
Hematocrit
List 3 potential complications:
Polycythemic infant:
- Hyperbilirubinemia
- Hypoglycemia.
- Hypocalcemia.
- Acidosis
- Cardiac failure.
Anemic infant:
- Cardiac failure.
- Hypoalbuminemia.
- Poor growth.

245
5.Erythema nodosum (EN)
Teenage girl has acutely
painful legs. She has
recently suffered from
mild cold. Her only
medication is mefenamic
acid and OCP; both for
menorrhagia.

246
What is the Dx:
Erythema nodosum.
Causes:
- Infection : streptococcal, mycoplasma,
mycobacterial, salmonella, parasites, EBV,
varicella, herpes.
- Drugs : Sulphonamides, OCP.
- IBD.
- Sarcoidosis.
- SLE

247
EN rare below 6 years of age.
Female predominance.
Lesion: red, shiny, painful and symmetrical.
Pathology: immune-complex mediated
reaction in the subcutaneous tissues around
large blood vessels producing nodules and
plaques, mainly in the tibial aspect.

248
6.Erythema Marginatum:
Comprises erythematous
ring with pale centers of
normal skin.
Only occasionally appear in
acute rheumatic fever
(ARF). a major criterion
Other criteria of ARF are:
- Polyarthritis.
- Carditis.
- Subcutaneous nodules.
- Chorea.

249
7.Sickle chest Syndrome

250
Giant hairy naevus:

It becomes malignant
in 30% of cases.
Surgical removal may
need skin grafting.

251
This child was brought to ER
Urticaria: with difficulty in breathing
after ingestion of strawberry.
O/E there were rash and
stridor.
What is the Dx? Urticaria.
What is ER management?
IV administration of
hydrocortisone and
antihistamine.
Airway management if
upper airway obstruction
persist
252
Urticaria (U):
Acute U:
- Due to exposure to an allergen or viral infection.
- May involves the deep tissue (swelling of lips) or
soft tissue around the eyes (angioedema) &
anaphlaxis.
Chronic U: > 6 wks
- Usually non-allergen.
- Most are idiopathic.

253
Clubbing and peripheral cyanosis:
Causes:
- Cystic fibrosis.
- Bronchectasis.
- Cyanotic cardiac
disease.
- IBD.
- Empyema.

254
Infections:
Meningitis:
Inflammation of the meninges.
Causes:
- Viral: most common (self-resolving)
- Bacterial: may have severe consequences.
- Other causes (non-infectious):
malignancy/autoimmune diseases.

256
Bacterial meningitis:
Pathophysiology:
- Usually after bacteremia.
- Damage due to the host response not
the organism.
- Inflammatory response leads to cerebral
edema, ICP and CBF.

257
Organisms: depend on the age.

Neonate -3 months GBS.


E coli,
Listeria monocytogenes.
1 month-6 years Nisseria meningitidis.
Streptococcus
pneumonia.
H. inflenza.
> 6 years Nisseria meningitidis.
Streptococcus
pneumonia

258
Clinical Presentation:
Early signs and symptoms are non-specific.
Infant and young children:
Combination of fever, poor feeding, vomiting,
irritability, lethargy, drowsiness, seizures or
consciousness.
Late signs: bulging fontanelle, neck stiffness and
opisthotonos.
Signs associated with neck stiffness: Brudzinskis
sign and Kerning's sign.
Older children, who can talk can describe
headache, neck stiffness and photophobia.
259
Opisthotonos
Infant lying with
an arched back.

260
Brudzinskis sign
Flexion of the
neck with the
child supine
causes flexion of
the knees and
hips.

261
Kerning's sign
With child lying
supine and with
the hips and knees
flexed, there is back
pain on extension
of the knee.

262
Septicemia: signs of shock e.g.
tachycardia, prolonged capillary refill
time, oligouria and hypotension.
Purpura (in meningiococcal
pneumoniae).

263
Contraindication of LP:
Ill patient.
Focal neurological signs.
Signs of ICP e.g. coma, high BP, low heart
rate or papillodema.
Coagulopathy.
Thrombocytopenia.
Local infection at site of LP.

264
Changes in CSF in meningitis:
Etiology Appearanc WBC Protein Glucose
e

Bacterial Turbid Polymorph


Viral Clear Lymphocyte N/ N/


(initially
polymorph)

TB Turbid/clea Lymphocyte
r/viscous

265
Types of Skin lesions:
Types of Skin lesion:
Asmaa Ghazi Al-Adwani
Macule:

268
Papule:

269
Nodule:

270
Vesicle:

271
Bulla:

272
Pustule:

273
Scale:

274
Ulcer:

275
Wheal:

276
Plaque:

277
Macule:

278
Papule:

279
Nodule:

280
Vesicle:

281
Bulla:

282
Pustule:

283
Scale:

284
Ulcer:

285
Wheal:

286
Plaque:

287

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