Biliary atresia

Question 1

What is biliary atresia?

Answer 1

A congenital condition where a section of the bile duct is either narrowed or absent, resulting in cholestasis where the bile can’t be transported from the liver to the bowel.

Biliary atresia prevents the excretion of conjugated bilirubin.

Question 2

How and when does biliary atresia present?

Answer 2

Shortly after birth with significant jaundice due to high conjugated bilirubin levels, dark urine, and pale stools.

Question 3

What are the examination findings in biliary atresia?

Answer 3

Hepato and splenomegaly, jaundice, and abnormal growth.

Question 4

When should biliary atresia be suspected?

Answer 4

Persistent jaundice lasting more than 14 days in term babies and 21 days in preterm babies.

Question 5

What investigations are done for biliary atresia?

Answer 5

Levels of conjugated and unconjugated bilirubin; a high proportion of conjugated bilirubin suggests the liver is processing but unable to excrete it.

Sweat chloride test: CF often involves the biliary tract. USS of the biliary tree and liver may show distension and tract abnormalities.

Question 6

What are some other causes of neonatal jaundice?

Answer 6

The majority of cases are benign (e.g., breast milk jaundice), but more serious cases like biliary atresia must be excluded by measuring the conjugated bilirubin levels.

Question 7

What is the management for biliary atresia?

Answer 7

Surgery: kasai portoenterostomy, which involves removing the damaged bile ducts and attaching a section of the small intestine to the opening of the liver. This can clear jaundice and prolong survival, but often patients need a full liver transplant.