Peadiatrics 3

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www.onlinembbs.com PAEDIATRICS 53
This 3 month old child died with extrahepatic biliary

atresia, a disease in which there is inflammation with
stricture of hepatic or common bile ducts.
This leads to marked cholestasis with intrahepatic bile
duct proliferation, fibrosis, and cirrhosis . This liver
was rock hard.
• Biliary atresia: The dark green color comes from formalin acting on bile
– is a disorder of unknown cause where a destructive pigments in the liver from marked cholestasis, turning
sclerosing inflammatory process causes obstruction of the bilrubin to biliverdin.
extrahepatic bile ducts and extends into the major
intrahepatic bile ducts.
– By 6-10 weeks of age the portal blood pressure is
Increased.
– Cirrhosis rapidly develops.
Clinical features :
• The first sign is prolongation of jaundice.
• The urine is always yellow.
• The stools contain no yellow or green pigment,
• Conjugated hyperbilirubenemia
• Acholic stools
Microscopically, extrahepatic biliary atresia leads to this

appearance in the liver, with numerous brown-green
bile plugs, bile duct proliferation (seen at lower
center), and extensive fibrosis. If a large enough bile
duct can be found to anastomose and provide bile drainage,
then surgery can be curative
• Biliary atresia must be -considered in any infant remaining Seen here is the major differential diagnosis of biliary
jaundiced beyond 14 days of age. atresia: this is neonatal giant cell hepatitis . There is
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lobular disarray with focal hepatocyte necrosis, giant Choledochal cyst
cell transformation, lymphocytic infiltration, Kupffer • In this disorder there is enlargement or dilatation of part
cell hyperplasia, and cholestasis (not seen here). or all the extrahepatic biliary system.
Neonatal hepatitis may be idiopathic or of viral origin. Many • Bite flow may be obstructed with intrahepatic changes
neonates recover in a couple of months similar to those of biliary atresia, progressing to cirrhosis.
• More common in females.
• Percutaneous liver biopsy showing the features • Recurrent abdominal pain
described above in all portal tracts is suggestive • Episodic jaundice
• Diagnosis of is confirmed at laparotomy • Right upper quadrant mass
• Complications : Cholangitis occurs in a significant minority • Or silent upto childhood are features
of cases in the first 2 years after surgery.
• Portal hypertension is present in almost all cases
• The prognosis been transformed by the Kasai operation

and with liver transplantation
Operative finding of choledochal cyst
Contrast-enhanced CT showing the lower end of the
choledochal cyst (arrow) with calcification (*) at
the uncinate process of the pancreas
Radionuclide (Disida) scan of choledochal cyst, the collection

of radionuclide is demonstrated in a cyst-like common bile
duct
Anatomy of the liver, duodenum, gallbladder, hepatic
ducts and common bile duct
There are five main variants of choledochol cysts

Type I - fusiform (spindle shaped) dilation of the
common bile duct and common hepatic duct
Type II - a sac like duct off the side wall of a biliary duct
Type III - cystic outpouching of the biliary duct into the
duodenum
Type IV - dilation of the hepatic ducts both inside and outside
the liver
Type V - dilation of the hepatic ducts inside the liver
Operative cholangiogram revealed fusiform dilatation of

common bile duct - choledochal cyst
• Type 1 (dilatation of common bile duct) I is commonest
Operative finding of large choledochal cyst
Complications Carolis disease:
• Cholangitis, • Congenital, autosomal recessive disorder.
• cyst rupture, • Communicating cavernous ectasia of intrahepatic biliary
• pancreatitis, ducts.
• gallstones and – it differentiates the disease from choledochal cyst.
• carcinoma of the cyst wall
Caroli’s disease. An axial T2-weighted MRI scan of the liver

shows several cystic lesions that are localized to
segment 7 of the liver in this patient with Caroli’s
disease.
These represent saccular dilatations of the biliary tree;
the largest lesion is noted to contain a small amount
of sludge.
• In infancy it presents with features of neonatal cholestasis. Saccular bile duct dilatation in Caroli disease
• In older children there may be recurrent upper
Saccular intrahepatic duct dilatation with normal sized
abdominal pain, recurrent jaundice and/or a
choledochal duct in Caroli disease
palpable cystic mass but the classical triad is rare.
• The treatment is surgical removal with biliary drainage via

a Roux-en-Y loop.
A very important sign is the central dot sign.( The central
dot corresponds to the portal vein that is surrounded
by dilated bile ducts.
On the left we see a dot within the dilated ducts.( When
we put on the color doppler, we will notice that these
structures contain blood flow and represent portal
veins.
Cirrhosis in a patient with Caroli disease
the gross specimen demonstrates dilated bile ducts and ductal

plate malformation was present microscopically.
• Cystic structures(Multiple) converging towards porta

LEFT: Infiltrating cholangiocarcinoma with stricture in
hepatis
a patient with Caroli disease ( RIGHT: Intraductal
• Central dot sign on CT Scan
cholangiocarcinoma
• Cholangiography most diagnostic
Common causes of chronic diahrrea
• Lactase deficiency
• Cows milk intolerance
• Irritable bowels
• Inflammatory bowel
• Giardiasis
• Cystic fibrosis
• Celiac disease
Pneumatosis intestinalis
• Pneumatosis intestinalis, defined as gas in the bowel wall,
is often first identified on abdominal radiographs or
computed tomography (CT) scans.
• It is a radiographic finding and not a diagnosis, as the

etiology varies from benign conditions to fulminant
gastrointestinal disease.
The findings are:
Medullary sponge kidney with stone Congenital hepatic
fibrosis and cystic • In neonates, pneumatosis is usually secondary to NEC and
formation (red arrow)
(Poly cystic) kidney indicates a later stage of the disease.
Mild bile duct dilatation (blue arrow)
Round hypoattenuating mass in the disease are associations
right liver lobe
• Pneumatosis intestinalis is considered an “ominous

finding” in ischemia , especially in association with
portomesenteric venous gas
Necrotising enterocolitis. This is a serious bowel infection

in new borns
• It is commoner in low birth weight infants and may
present with signs of bowel obstruction.
• These are severe abdominal distension with
tenderness and bile stained vomiting.
• Look for blood stained stools.
Pneumatosis intestinalis occurs in 2 forms.

• NEC is seen almost exclusively in premature infants and • Primary pneumatosis intestinalis (15% of cases) is a
is associated with bowel ischemia caused by b a c te r i a l benign idiopathic condition in which multiple thin-
invasion and hyperosmolar feeds. walled cysts develop in the submucosa or subserosa
of the colon.
• Usually, this form has no associated symptoms, and
the cysts may be foun d incidentally thro ugh
radiography or endoscopy.
• When the cysts protrude into the lumen, they may mimic
polyps or carcinomas , as shown on barium enema studies
• Mesenteric vascular occlusion leads to ischemic damage

of the mucosa, which allows luminal gas and bacteria to . This primary form is often termed
invade the bowel wall. pneumatosis cystoides intestinalis
The secondary form (85% of cases) is associated with • Of the cases, 50% are associated with the CHARGE
obstructive pulmonary disease, as well as with obstructive syndrome - choanal atresia with ear, eye, heart and genital
and necrotic gastrointestinal disease. defects.
• Gasping respiration in a neonate is suggestive of choanal
atresia.
• Bilateral choanal atresia is a neonatal emergency.
The disease is seen in other conditions, including

• chronic obstructive pulmonary disease,
• connective tissue disorders,
• infectious enteritis,
• celiac disease,
• leukemia,
• amyloidosis,
• and acquired immunodeficiency syndrome (AIDS); • The diagnosis is by suspicion, by inability to pass a catheter
• It is also found in association with organ transplantation, along the nose and confirmation by endoscopic
steroid use, and chemotherapy. examination.
• The treatment consists of establishment of an airway either
• Pediatric Pulmonology oral or orotracheal.
CHOANAL ATRESIA
• This rare anomaly is due to failure of breakdown of
the nasobuccal membrane which normally occurs at
weeks’ fetal development. Transient tachypenea of new born
• Self Limited ,
• seen after caesarean section,
• Tachypnea, mild retractions and grunting seen.
• CXR shows fluid in fissure and prominent vascular markings,
Air bronchogram and reticular shadows.
Radiograph of a neonate at age 2 days. Cardiomegaly

has disappeared . Pulmonary parenchymal abnormalities
are diminishing, but perihilar, streaky markings persist.
- Transient Tachypnoea of the Newborn (TTN, also called
Retained Fetal Lung Fluid or “Wet Lung”) is a diagnosis of
exclusion.
-Typical radiologic features are ill-defined but include:

1-Increased centr al v ascula r markings (“sta r-burst”
appearance)
2-Hyperaeration
3-Evidence of interstitial and pleural fluid
4-Prominent interlobar fissures
5-Cardiomegaly
Radiograph of a neonate at age 4 days. Normal

heart size and clear lungs are seen .
Meconium aspiration syndrome

• Most common cause of neonatal respiratory distress
in full-term/postmature infants
• Hyaline membrane disease most common cause in
premature infants
Radiograph of a neonate at age 6 hours.

Overaeration and streaky, bilateral, pulmonary
interstitial opacities and prominent perihilar
interstitial markings are seen along with mild
cardiomegaly.
Etiology
• Fetal circulatory accidents/placental insufficiency /
postmaturity result in perinatal hypoxia and fetal distress
• Meconium defecated in utero triggered by vagal response
Frontal chest shows large, ropey and strand-like

densities in (a post-mature infant consistent with
• Pathogenesis Meconium Aspiration Syndrome
– Meconium products produce bronchial obstruction and air-
trapping Diaphragmatic hernia
– Chemical pneumonitis • The commonest herniation is the Bochdalek type, a
• Incidence posterolateral defect, possibly a failure of closure of
– 10% of all deliveries have meconium-stained amniotic fluid the pleuroperitoneal canal.
– 1% of all deliveries have respiratory distress
• CXR shows patchy infiltration
• Mechanical ventilation, , ECMO are used
Imaging findings
• Bilateral diffuse grossly patchy opacities (atelectasis and
consolidation)
• Hyperinflation of lungs
• Areas of emphysema (air-trapping)
• Spontaneous pneumothorax and pneumomediastinum
• 25% requiring no therapy • A hernia through the Foramen of Morgagni (Retrosternal)
• Small pleural effusions (20%) is less common in neonates. This defect is retrosternal,
• No air bronchograms to the right or left of the midline.
• Rapid clearing usually within 48 hours • The third site for herniation is the Esophageal hiatus - the
so-called hiatus hernia.
- Majority present with respiratory distress – cyanosis,
dyspnea and tachypnea – either immediately after birth
or within a few hours.
- Most common on left side.
Eventration of the diaphragm

• This is due to a deficiency in the muscle of the diaphragm.
• The thin layer becomes attenuated and bulges up into
the thorax.
• Extensive eventrations are similar to diaphragmatic hernia
presenting in the neonatal period.
• Smaller eventrations present later and require localized
plication.
• Examination reveals a scaphoid abdomen, bowel sounds
• Congenital diaphragmatic hernia are present along
on auscultation of the affected side of the chest and a
with polyhydraminos
shift of the apex beat to the right in the case of a
left-sided hernia.
• Once air has been swallowed after birth a chest X-ray

confirms the diagnosis, showing gas filled loops of bowel
on the affected side of the chest with displacement of
the mediastinum to the opposite side
Congenital diaphragmatic hernia:

• Develop symptoms of respiratory distress, either in the
delivery room or shortly thereafter, as the bowel in the
chest becomes distended with swallowed air.
• This further compresses the ipsilateral lung (which is
often hypoplastic) and causes a mediastinal shift,
interfering with ventilation of the contralateral lung.
• The infant appears

• dyspneic,
• tachypneic, and
• cyanotic and has severe retractions with an
increased chest diameter and
• a relatively scaphoid abdomen
• Treatment: A nasogastric tube is passed to reduce the • Bowel sounds may be heard on auscultation of the
gaseous distention of the bowel with air. affected chest.
HM is made of fibrin
• It is due to surfactant deficiency resulting in poor lung
compliance and atelectasis
• Seen in diabetics, premature babies In addition to the
• An anteroposterior and lateral chest x-ray demonstrates early onset of respiratory distress, clinical features include
air-filled abdominal viscera in the chest, confirming the - Tachypnea,
diagnosis - intercostal and sternal retractions, expiratory grunt,
- Nasal flaring
- Decreased lung compliance.
• Four radiographic features characteristic of HMD are:

- Reduced lung volume, with expansion only to the fifth,
sixth or seventh thoracic vertebrae rather than to the
eighth or ninth, as found in the normally expanded chest.
The reduced lung expansion is a consequence of
atelectasis, which occurs due to a tack of surfactant.
HMD (Hyaline Membrane Disease)
- Also, the diaphragms will appear unusually high and
• Most neonates with HMD demonstrate clinical findings of
sometimes domed, and the intercostal spaces will appear
respiratory distress in the delivery room or during the first
narrow,
few hours of life.The condition starts with an increase in
breathing rate, with respiration then becoming labored
and more rapid.
Air bronchograms, or the outlines of air-filled secondary and • Confirmed by Chest x ray.
tertiary bronchi seen over abnormal lung fields. Because • L/S >2 indicates lung maturity
normally expanded lungs and the overlying bronchi are of
air density, air bronchograms are not generally seen in
the lung fields on a normal chest x-ray.
- A reticulogranular/ reticulo nodular pattern, or
ground glass appearance , uniformly distributed
throughout both lung fields. Because of surfactant
deficiency, some alveoli in the lung collapse from high
surface tension.
- The alveoli that remain distended have lower surface

tension. Air entering the lung goes only to the area of Pulmonary alveolar proteinosis (PAP)
lowest pressure, creating the image of ground glass • PAP presents in infancy with progressive respiratory
appearence. distress.
• In some children it may be related to a form of immune
deficiency such as thymic atrophy, lymphopenia and
immunoglobulin deficiency.
• CXR shows a ground glass appearance, and
• Increased lung opacification . When diffuse lung

opacification becomes visible, it usually is the result of
nonexpanded alveoli with little or no terminal airway
aeration.
• The heart borders may be visible initially, but the x-ray
may progress to a complete loss of visualization of the
heart borders, called a “whiteout”. • HRCT a typical cobblestone appearance.
- Suspect this condition in a neonate with respiratory • Characteristically there is a short prodromal upper
distress not responding to surfactant & positive history of respiratory tract illness with coryzal symptoms and low-
past baby ‘s death similarly. grade pyrexia, followed by a relatively sudden onset of
tachypnea, hypoxia, a moist cough and difficulty with
feeding.
Bronchiolitis:
• Caused by RSV mainly. Increased work of breathing is reflected by suprasternal,
• Commonly in children less than 2 years, subcostal and intercostal recession with head bobbing and
• Self limiting. nasal flaring.
• Ribavarin DOC. The predominant feature on auscultation is crackles with, or
• Leads to asthma without the presence of wheeze.
• Humidified oxygen As the chest becomes hyperinflated, the liver is displaced
and is often easily palpable in the abdomen.
• The infecting organism in around 75% cases is respiratory

syncytial virus (RSV) and H.influenza. More common in
boys.
• Infants at high risk of severe RSV include those born
prematurely, and those with cardiovascular disease,
chronic respiratory disease including bronchopulmonary
dysplasia (BPD) and cystic fibrosis or immunosuppression. • CXR show hyperinflation with multiple areas of interstitial
infiltration
• Current therapy for RSV Bronchiolitis is essentially
supportive, involving maintenance of hydration and oxygen
status. Nasogastric or intravenous feeding is required if
the baby is unable to suck.
• May lead to asthma in later life.
• Ribavirin inhibits viral replication and is the antiviral agent
available against RSV.
• Oxygen is helpful.
• RSV immunoglobulin has no role in acute attacks.
• Antibiotics are not used initially
• Long term oxygen dependency is common.

• Improvement after 2-3 years may be seen.
• The condition is usually stable once the initial effects of
the devastating infection have burnt out, but some may
go on to require lung transplantation .
• OB developing after lung transplant is eventually fatal,
and the results of a second transplantation are very poor.
Follicular bronchiolitis
- Follicular bronchiolitis (FB) is part of the spectrum of
Obliterative bronchiolitis lymphoid disorders of the lung, which includes lymphoid
• Obliterative bronchiolitis (OB) has been described in interstitial pneumonia (LIP).
association with infections caused by adenovirus types 3, - There is a polyclonal expansion of lymphoid tissue,
7 and 21; measles, pertussis, influenza A, mycoplasma; probably part of the bronchus associated lymphoid
after lung transplantation; and secondary to chronic tissue (BALT), which causes small airway obstruction.
aspiration. - Presentation is with chronic respiratory distress, and HRCT
• The clinical course is one of cough, wheeze and demonstrates air trapping, sometimes with associated
tachypnea in an infant or young child which may be infiltrates.
static if the cause is an infection, or progress at a variable
rate to respiratory failure and failure to thrive if there is
ongoing aspiration.
Integrated management of childhood illness includes:

• Malaria
• Diahorrea
• Acute respiratory infections.
• Chest radiography initially shows generalized hyperinflation.

• HRCT demonstrates patchy air trapping, particularly if the
scan is expiratory.
• Focal hyperlucency involving a lobe or a whole lung
represents the radiological appearance of Macleod’s
syndrome, in which the affected lung appears small
and hyperlucent.
• Effusions are U/L & never associated with segmental
Bronchiolitis 0-18 Respiratory Hyperinflati pulmonary lesion & rare in disseminated TB
months syncitial on • MC form of cardiac TB is pericarditis
virus • MC bone & joint involvement is the vertebra
Lary ngotracheob 1 -2 Para influenza Steeple

ronchitis years virus sign
• Most serious complication in children is TB of CNS &

is fatal without treatment
• MC site of CNS involvement is the brainstem
Epiglottitis 2-5 years H influenza Thumb sign
TB in children: • MC form of extrapulmonary TB in children is TB of superficial

• MC (most common) symptom in primary pulmonary TB in lymph nodes,referred to as scrofula
children - Non-productive cough • Genitourinary TB is rare in children because incubation
• TB pleural effusion is infrequent in children <6yrs a rare period is several yrs or longer
in children <2yrs.
Paediatric Cardiology Right to left shunts: TOF
• Transposition of great vessels
A 10 year old girt with pulmonary flow systolic ASD • Tricuspid Atresia
murmur and fixed split second hear sound Left To Right Shunts:
• A5D
• VSD
• PDA
A 3 year old girl with failure to thrive has pan VSD

systolic murmur on left sternal
border with prominent pulmonary vascular
markings
Cardiac tumors in childhood:

• Rhabdomyoma
• Myxoma
• Fibroma
A 5 day old girl with machinery like heart PDA

murmur and bounding peripheral pulses
Rhabdomyoma of heart (cardiac Rhabdomyoma ) in left ventricle
ASD;
• Commonest: Ostium secondum defects
• Systolic ejection murmur in left mid and upper sternal
border.
→ A wide fixed split of S2.
→ Left parasternal heave is seen
→ CXR: Enlarged RA, RV not LA
A 6 year old with clubbing, cyanosis improved T etral → Seen in Holt Oram Syndrome
by squatting, systolic ejection murmur in 3 rd ogy
intercostal space with right ventricular of
hypertrophy Fallot
■ Most common type of ■ Ostium secondum type.

ASD is
Parasternal short axis: RV dilation with RV pressure

overload as evidenced by M.
■ ASD+single atrium= ■ "Ellis Crevald Syndrome.
Transesophageal echocardiogram: Moderate-large ASD

with left-to-right shunt across the interatrial
septum.
■ ASD+Mitral stenosis= ■ "Lutembachers syndrome
Left to right shunt across a Apical 4-chamber view.

distended atrial septum in
the same patient. Electrocardiography in ASD
Mitral stenosis. • Characteristic findings in patients with secundum ASD
are a normal sinus rhythm, right-axis deviation , and an
rSR’ pattern in V1, an interventricular conduction delay or
■ ASD+Bony ■ "Holt Oram Syndrome
right bundle branch block (which represents delayed
abnormalities=
posterobasal activation of the ventricular septum and
enlargement of the right ventricular outflow tract).
• Left-axis deviation and an rSR’ pattern in V1, an
interventricular conduction delay or right bundle branch
block suggests an ostium primum defect.
The right atrium is not enlarged in the other two

Incomplete right bundle branch block (IRBBB) and varieties of Eisenmenger syndrome.
left axis deviation are the ECG features of primum
ASD. VSD:
In secundum ASD, the ECG features are IRBBB and right • Holosystolic murmur at left lower sternal border.
axis deviation • Thrill associated.
• LVF, Biventricular hypertrophy with left axis deviation.
• Close spontaneously in 30-50% cases.
The right atrium is enlarged due to volume overload.

This will manifest as tall P waves (more than 2-3 mm).
RVH is also noted, typically as rsR’ pattern of the QRS
complexes in the right chest leads.
• Left-axis deviation and negative P wave in lead III suggest

sinus venosus defect.
• Increasing pulmonary hypertension can cause loss of
the rSR’ pattern in V1 and a tall monophasic R wave with
a deeply inverted T wave. PDA:
→ Failure of closure of Ductus arteriosus
• A prolonged P-R interval can be seen in familial ASD or → Blood flows from “Aorta to Pulmonary artery “both during
ostium primum secondary to left atrial enlargement and systole and diastole.
an increased distance for internodal conduction produced → Wide pulse pressure
by the defect itself. Displacement of the AV node in a → Seen in Rubella
posteroinferior direction in some patients or an enlarged → Hypoxia and prematurity predispose
right atrium has also been reported.
• The main pulmonary artery (MPA) is grossly dilated .

• The right pulmonary artery (RPA) is also quite enlarged.
• Right atrial enlargement is seen as a shift of the cardiac
contour to the right of the spine.
• Pulmonary vascularity is increased and prominent
end on vessels (End on) are also seen.
• Apex is upwards , suggesting a right ventricular
configuration.
• All features suggest a large secundum atrial septal
defect with a large left to right shunt producing severe
pulmonary hypertension.
• Cardiomegaly on chest x-ray is suggestive of atrial septal

defect in Eisenmenger syndrome, while it is unlikely in
ventricular septal defect and patent ductus arteriosus.
Cardiomegaly is mainly due to the grossly dilated right
atrium in atrial septal defect.
“Differential cyanosis” is seen. Closure of a PDA by coil catheterization. (A)
Injection into the aorta reveals a large PDA at
baseline. (B) Following placement of a coil the
angiographic dye no longer crosses into the
pulmonary artery confirming ductal closure. (MPA
= main pulmonary artery, PDA = patent ductus
arteriosus, DA = descending aorta)
Coarctation of Aorta:
• Mostly below origin of left subclavlan artery.
• AcyanotIc
→ Seen in Turners syndrome and associated with bicuspid
aortic valve.
• Upper extr emity Hypertension headache,
dizziness,intermittent claudication
55-year-old gentleman with unrepaired large PDA , • Lower Extremity Hypotension.
Eisenmenger’s syndrome, and right-to-left • Rib notching due to Increased flow through collaterals.
shunting at the ductal level producing differential
cyanosis. The transcutaneous oxygen saturation in the
fingers was 92 % and in the toes 84 %. The central
hematocrit was 65 %. Clubbing is more apparent in
the toes than in the fingers.
→ Machinery murmur” which is continuous.

Heard at 2nd left intercostal space.1
• Rib notching with double bulging is seen.
→ CXR: Prominent Pulmonary artery.
• Cause of death is usually complication of hypertension,
→ PG inhibitors stimulate closure of PDA
CHF, Endocarditis.
Trilogy Of Fallot:
• Pulmonary valve stenosis
• Atrial septal defect
• Right ventricular hypertrophy.
- Tetralogy of Fallot:
Ventricular septal defect, communication between the two
ventricles
• Pulmonary stenosis, narrowing at the pulmonary valve or
at the level of right ventricular infundibulum, which lies
just below the pulmonary valve
• Over-riding of the aorta, the aorta being positioned over
the ventricular septal defect instead of in the left ventricle
• Right ventricular hypertrophy,
- Pentalogy of Fallot:
• The four characteristics of Fallot’s tetralogy syndrome, plus
a patent foramen ovate or atrial septal defect.
Complications of TOF
1) Erythrocytosis
2) Brain abscesses
3) Acute Gouty Arthritis
4) Infective Endocarditis
5) Cerebrovascular Thrombosis
6) Delayed puberty
TOF: Tetrology of Fallot
– Boot shaped heart Corn en Sabot heart decreased
Pulmonary blood flow. Ejection systolic mumur, single S2,
– Associated with right sided aortic arch
– Right axis deviation and RVH.
– Blue spells seen.
TGA (Transposition of Great Vessels)

• Aorta arises from Right Ventricle and Pulmonary artery
from Left ventricle.
• Common in infants of diabetic mothers.
• Presents with cyanosis in first 24 hours of life.
• Egg on string appearance-
• PGE , is used.
• Tet spells ‘Hypoxic spell are characterised by :

1) period of uncontrolled crying
2) rapid and deep breathing
3) deepening of cyanosis
4) decreased in intensity of heart murmurs
5) limpness
6) convulsions
7) rarely death.
note the narrowness of the “aortic knob” and the lack of the • Most common Type is supracardiac
pulmonary artery segment on the PA xray. • Infra cardiac type is invariably always obstructive.
• Snow man pattern of figure 8 occurs
Tricuspid atresia:
• Single 52 MURMURS:
• Pansystolic murmur • Carey Coombs Murmur: Transient Soft mid diastolic murmur
• LVH and LAD. of Acute Rheumatic fever due to Mitral valvulitis, low
pitched
• Graham Steel Murmur: Early diastolic murmur of Pulmonary
Regurgitation.
• Means Murmu r: Pulmonary S ystoli c murmur in
Thyrotoxicosis.
• Sea Gulls Murmur: Aortic Regurgitation murmur.
• Austin Flint Murmur: Murmur of Aortic regurgitation

– Classically, it is described as being the result of mitral valve
leaftlet displacement and turbulent mixing of antegrade
mitral flow and retrograde aortic flow:
– Displacement: The blood jets from the aortic regurgitation
strike the anterior leaflet of the mitral valve, which often
results in premature closure of the mitral leaflets. This
can be mistaken for mitral stenosis
ENDOCARDIUM is most commonly Involved.

• Involvement of Tricuspid valve cause TRICUSPID
REGURGITATION
• Involvement of Pulmonary valve cause PULMONARY
STENOSIS
• MITRAL VALVE is most commonly involved valve on left
side.
Total anomalous pulmonary venous return (TAPVC) • If Cardiac Lesions are present in Carcinoid
• Pulmonary veins dr ain back into systemic venous • Syndrome, it causes Heart Failure in 80% Patients.
circulation. • Cardiac effects in carcinoid is due to serotonin
• Right atrial blood goes to left atrium through foraman
ovale/ ASD.
• Pulmonary hypertension occurs. Carcinoid syndrome & Heart
Major cardiovascular manifestations of some Iron deficiency anemia
important diseases • Commonest type of anemia in children.
• Iron deficiency in children can be due to causes slightly
■ Progeria: Accelerated different from adults:
atherosclerosis – Cows milk( Poor source of iron)
■ Cystic fibrosis: Cor pulmonale – Hookworm infestation
■ Freidrechs Ataxia: Cardiomyopathy – Polyposis colon
– Meckels diverticulum
■ Williams syndrome: Supravalvular Aortic – Malabsorption syndrome
stenosis – Prematurity and low birth weight
■ Cornelia de lange VSD
syndrome:
Most common associations
• Turners syndrome • Coarctation of Aorta

• Noonan Syndrome. • Pulmonary stenosis
• Congenital Rubella • PDA
• Marfan syndrome • MVP

• Williams syndrome • Supravalvular Aortic
stenosis
• Digeorges • Aortic arch
Syndrome abnormalities
• Paediatric Hematology • Red cell distribution width (RDW) in iron deficiency
anemia is increased.
In the BM(Bone Marrow) first morphologically recognizable
precursor is the PROnormoblast and “not normoblast”.
• This cell can undergo 4-5 cell divisions that result in
production of 16-32 mature RBCs
• Iron supplementation should be started to a term breast

fed baby at 6 weeks
• Increased reticulocyte is the first indicator of response
after iron therapy
• Iron requirement: 4x wt(kg)x Hb deficit(gm/dl)
• Erythropoietin is produced by the peritubular cells within
the kidney.
• Haemoglobin first to appear in fetus: Hb Gowers
• Switch over of fetal Hb to adult Hb begins at 14 weeks
gestation.
• Fetal Hb is completely replaced by adult Hb by 6 months
• Bone marrow hyperactivity causes thickening of the cranial
Iron Thale Siderobi Chronic bones and malar eminences, producing “hemolytic facies.
defice ssmia astic anemia
ncy anemia
Sr Fe ↓ n n ↓
TIBC ↑ n n ↓
%Saturati ↓ ↑ ↑ ↓
on
Ferritin ↓ ↑ ↑ ↑
• Hypochromic microcytic anemia with ↓sr Fe, ↑ TIBC: Iron
deficiency anemia.
• ↓ Sr Ferritin is the early indicator of iron deficiency.
Long-bone involvement makes pathologic fractures common.

• Osmotic fragility decreased. NOT INCREASED.
Thalassemia Major and Minor

• A group of chronic, inherited, microcytic anemias
characterized by defective Hb synthesis and ineffective
erythropoiesis;
• Growth rates are impaired, and puberty may be significantly
• Symptoms of severe anemia occur in b- thalassemia major delayed or absent.
(Cooley’s anemia). “Clinical features result from anemia, • Fe deposits in heart muscle may cause dysfunction and
markedly expanded marrow space, and transfusional and ultimately heart failure.
absorptive Fe overload. Patients are jaundiced, and leg • Hair on end appearance seen.
ulcers and cholelithiasis occur {as in sickle cell anemia).
• Splenomegaly is common, and the spleen may be huge.
If splenic sequestration develops, the survival time of
transfused, normal RBCs is shortened.
• Hepatic siderosis occurs typically, leading to functional

impairment and cirrhosis.
• Electrophoresis is the diagnostic test
• There are 4 major types of globins labeled as alpha (α),
beta (β), gamma (γ), and delta (δ). The dominant
hemoglobin in adults (hemoglobin A) is composed of 2
• T arget cells: Liver Diseases,
alpha and 2 beta chains Thalessmia
• Minor forms of hemoglobins constitute a small percentage
of the normal blood and are referred to as hemoglobin F
(fetal), composed of 2 alpha chains and 2 gamma
chains, and hemoglobin A2, composed of 2 alpha
chains and 2 delta chains.
• Helmet cells: DIC, Microangipathic

Hemolytic anemia
Peripheral smear from a patient with beta-zero thalassemia

major showing more marked microcytosis (M) and
anisopoikilocytosis (P) than in thalassemia minor.
Target cells (T) and hypochromia are prominent
• Burr cells: Uremia
Peripheral smear in beta-zero thalassemia minor showing

microcytes (M), target cells (T), and poikilocytes.
• T ear drop cells: Myelofibrosis,

Myelopthisic anemia
• Bite cells: G6PD Deficency
• Smudge cells: CLL

• Von Willebrand disease is characterised by platelet adhesion
defect
VonWillebrands factor) levels which is synthesized in platelets

is low.
• Types
• type 1: partial reduction in vWF
• type 2: abnormal form of vWF
• type 3: total lack of vWF
Investigation
• prolonged bleeding time
• APTT may be prolonged
Von Willebrand’s disease • factor VIII levels may be moderately reduced
• Von Willebrand’s disease is the most common inherited • defective platelet aggregation with ristocetin
bleeding disorder.
• It is inherited in an autosomal dominant fashion and Ristocetin induced platelet agglutination
characteristically behaves like a platelet disorder i.e.
epistaxis a nd menorrhagia are co mmon whilst • In an unknown fashion, the antibiotic ristocetin causes
haemoarthroses and muscle haematomas are rare von Willebrand factor to bind the platelet receptor
glycoprotein Ib (GpIb), so when ristocetin is added to
normal blood, it causes agglutination.
Role of von Willebrand factor

• large glycoprotein which forms massive multimers up to
1.000.000 Da in size
• promotes platelet adhesion to damaged endothelium
• carrier molecule for factor VIII • In von Willebrand disease, where von Willebrand factor is
absent or defective, abnormal agglutination occurs:
• In type 1 vWD: hypoactive agglutination occurs
• In type 2A vWD: hypoactive agglutination occurs
• In type 2B vWD: hyperactive agglutination occurs
• In type 2M vWD: hypoactive agglutination occurs
activity[2]
• In type 2N vWD: normal agglutination occurs
• In type 3 vWD: no agglutination occurs
• Treatment:
– Factor VIII cryoprecipitate
– -desamino-8 -D-arginine v asopres sin, DDA VP)
(desmopressin acetate,
• On examination of the bone marrow, an increase in the

production of megakaryocytes may be observed and may
help in establishing a diagnosis of ITP.
Some of the
megakaryocytes
appear relatively
immature with
hypolobulated nuclei
and hypogranular
cytoplasm.
• Platelet transfusions are not helpful in immunogenic variety
ITP
In many cases, the cause is not actually idiopathic but
autoimmune, with antibodies against platelets being
detected in approximately 60 percent of patients.
Most often these antibodies are against platelet membrane
glycoproteins II b-llla or Ib , and are of the IgG type.
Thrombotic Thrombocytopenic Purpura:
There is widespread formation of platelet thrombi.
• The diagnosis of ITP is a process of exclusion.

• In approximately one percent of cases, autoimmune
hemotytic anemia and immune thrombocytopenic purpura
coexist, which is a condition called “Evans syndrome”.
• Bleeding time is prolonged in ITP patients.
• Lactate dehydrogenase levels are generally used to
monitor disease activity.
• Plasmapheresis may need to be continued for 1–8
weeks before patients with idiopathic TTP cease to
consume plat elets and begin to nor malize their
hemoglobin.
Overwhelming Post Splenectomy Infection (OPSI)

• Infection due to encapsulated bacteria
• Almost 50% due to strep. Pneumoniae
• Other organisms include: Haemophilus influenzae .Neisseria
meningitidis
• Occurs post splenectomy in 4% patients without
prophylaxis
Two arterioles in a case of typical HUS with severe • Mortality of OPSI is approximately 50%
vascular involvement. The vascular lumen is • Greatest risk in first 2 years post op.
extremely narrowed due to miointimal proliferation and
vascular wall thickening.
Pentad of signs” is:

• Fever
• Thrombocytopenia
• Microangiopathic Hemolytic Anemia
• Neurological symptoms
• Renal failure
• Lab features:
• Decreased Platelet count and increased Bleeding time.
• Normal PT and PTT
• Normal complement
• Plasmapheresis has become the treatment of choice

for TTP.
• This is an exchange transfusion involving removal of the
patient’s blood plasma through apheresis and replacement
with donor plasma (fresh frozen plasma or
cryosupernatant); the procedure has to be repeated daily
to eliminate the inhibitor and abate the symptoms. If
apheresis is not available, fresh frozen plasma can be
infused, but the volume able to be given safely is limited
due to the danger of fluid overload.

Peadiatrics 3

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Peadiatrics 3

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Dr.

Murali Bharadwaz Classes for MD/MS Entrance Coaching Programme

This 3 month old child died with extrahepatic biliary

Microscopically, extrahepatic biliary atresia leads to this

• The prognosis been transformed by the Kasai operation

Operative finding of choledochal cyst

Radionuclide (Disida) scan of choledochal cyst, the collection

There are five main variants of choledochol cysts

Operative cholangiogram revealed fusiform dilatation of

• Type 1 (dilatation of common bile duct) I is commonest

Operative finding of large choledochal cyst

Caroli’s disease. An axial T2-weighted MRI scan of the liver

• The treatment is surgical removal with biliary drainage via

Cirrhosis in a patient with Caroli disease

the gross specimen demonstrates dilated bile ducts and ductal

• Cystic structures(Multiple) converging towards porta

• It is a radiographic finding and not a diagnosis, as the

• Pneumatosis intestinalis is considered an “ominous

Necrotising enterocolitis. This is a serious bowel infection

Pneumatosis intestinalis occurs in 2 forms.

• Mesenteric vascular occlusion leads to ischemic damage

The disease is seen in other conditions, including

Radiograph of a neonate at age 2 days. Cardiomegaly

-Typical radiologic features are ill-defined but include:

Radiograph of a neonate at age 4 days. Normal

Meconium aspiration syndrome

Radiograph of a neonate at age 6 hours.

Frontal chest shows large, ropey and strand-like

Eventration of the diaphragm

• Once air has been swallowed after birth a chest X-ray

Congenital diaphragmatic hernia:

• The infant appears

• Four radiographic features characteristic of HMD are:

- The alveoli that remain distended have lower surface

• Increased lung opacification . When diffuse lung

• The infecting organism in around 75% cases is respiratory

• Long term oxygen dependency is common.

Integrated management of childhood illness includes:

• Chest radiography initially shows generalized hyperinflation.

Lary ngotracheob 1 -2 Para influenza Steeple

• Most serious complication in children is TB of CNS &

Epiglottitis 2-5 years H influenza Thumb sign

TB in children: • MC form of extrapulmonary TB in children is TB of superficial

A 3 year old girl with failure to thrive has pan VSD

Cardiac tumors in childhood:

A 5 day old girl with machinery like heart PDA

Rhabdomyoma of heart (cardiac Rhabdomyoma ) in left ventricle

■ Most common type of ■ Ostium secondum type.

Parasternal short axis: RV dilation with RV pressure

Transesophageal echocardiogram: Moderate-large ASD

■ ASD+Mitral stenosis= ■ "Lutembachers syndrome

Left to right shunt across a Apical 4-chamber view.

The right atrium is not enlarged in the other two

The right atrium is enlarged due to volume overload.

• Left-axis deviation and negative P wave in lead III suggest

• The main pulmonary artery (MPA) is grossly dilated .

• Cardiomegaly on chest x-ray is suggestive of atrial septal

→ Machinery murmur” which is continuous.

TGA (Transposition of Great Vessels)

• Tet spells ‘Hypoxic spell are characterised by :

• Austin Flint Murmur: Murmur of Aortic regurgitation

ENDOCARDIUM is most commonly Involved.

• Turners syndrome • Coarctation of Aorta