Biliary Atresia

Question 1

What is biliary atresia?

Answer 1

A congenital disorder where part of the bile duct is narrowed or missing leading to hyperbilirubinaemia

Question 2

What is the presentation of biliary atresia?

Answer 2

Prolonged jaundice - more than 14 days
Signs of biliary obstruction
- Pale stools
- Dark urine

Question 3

What investigations are performed to diagnose biliary atresia?

Answer 3

Bloods - high conjugated bilirubin
Deranged LFTs
Choliangiography (XR) - abnormal structure of the biliary tree

Question 4

What is the definitive investigation in the diagnosis of biliary atresia?

Answer 4

Choliangiography - absence of biliary tree will be seen

Question 5

What kind of bilirubinaemia is seen in biliary atresia?

Answer 5

Conjugated

Question 6

What may be found on examination of a baby with biliary atresia?

Answer 6

RUQ and LUQ masses due to hepatomegaly and splenomegaly

Question 7

What is the management of biliary atresia?

Answer 7

Surgery to create a new pathway from the liver to the gut
- Kasai procedure