Biliary Atresia - Short Note - Paediatrics

Biliary atresia is not very common. However it's the major differential diagnosis of Obstructive jaundice in a neonate. Here are the common DD for obstructive jaundice,

• Biliary atresia
• Neonatal hepatitis
• Choledocal cyst
• Tyrosinaemia
• Alpha 1 antitrypsin deficiency
  

Biliary atresia

    • This occurs in 1 in 14 000 live births.
    • It is a progressive disease, in which there is destruction or absence of the extra-hepatic biliary tree and intrahepatic biliary ducts.
    • This leads to chronic liver failure and death unless surgical intervention is performed.

Clinical features

    • Normal birth weight but fail to thrive as the disease progresses.
    • They are usually mildly jaundiced
    • Pale stools
    • Dark Urine
    • Hepatomegaly
    • Splenomegaly (secondary to portal hypertension)
    • Features of cirrhosis may be present in advance cases (clubbing, palmar erythema, spider nevi, gynaecomastia, caput medusa)

Investigations

    • Liver function tests - little value in the differential diagnosis.

    • Fasting abdominal ultrasound may demonstrate a contracted or absent gallbladder

    • Radioisotope scan with TIBIDA shows good uptake by the liver, but no excretion into the bowel.

    • Liver biopsy demonstrates features of extrahepatic biliary obstruction

    • Diagnosis is confirmed at laparotomy
 

Management

Surgical bypass of the fibrotic ducts, hepatoportoenterostomy (Kasai procedure)  -loop of jejunum is anastomosed to the cut surface of the porta hepatis, facilitating drainage of bile from any remaining patent ductules.

(If surgery is performed before the age of 60 days, 80% of children achieve bile drainage. The success rate diminishes with increasing age)

Postoperative complications  -cholangitis
                                               -malabsorption of fats and fat-soluble vitamins

                                               -cirrhosis and portal hypertension can occur even after Kasai procedure

If the operation is unsuccessful, liver transplantation  (Biliary atresia is the single most common indication for liver transplantation in the paediatric age group.)

Answer the following question.

01. A 3 week old breast fed infant has deep jaundice. On physical examination the liver is 3cm below the costal margin. What is the most important laboratory test in this child to diagnose the condition at this time?

a. Serum ceruloplasmin level

b. Direct and total bilirubin levels

c. Hepatic ultasonography

d. Full blood count

e. Urine urobilinogen level

Comment your answer.