Biliary Atresia

Question 1

What is biliary atresia?

Answer 1

Biliary atresia is a congenital condition where a section of the bile duct is either narrowed or absent. This results in cholestasis, where the bile cannot be transported from the liver to the bowel. Conjugated bilirubin is excreted in the bile, therefore biliary atresia prevents the excretion of conjugated bilirubin.

Question 2

Briefly describe the excretion of bilirubin

Answer 2

Question 3

What are the clinical features of biliary atresia?

Answer 3

Biliary atresia presents shortly after birth with significant jaundice due to high conjugated bilirubin levels. Suspect biliary atresia in babies with a persistent jaundice, lasting more than 14 days in term babies and 21 days in premature babies.

Question 4

What is the initial investigation of biliary atresia?

Answer 4

The initial investigation for possible biliary atresia is conjugated and unconjugated bilirubin.

Question 5

How does biliary atresia appear on blood tests?

Answer 5

A high proportion of conjugated bilirubin suggests the liver is processing the bilirubin for excretion (by conjugating it), but it is not able to excrete the conjugated bilirubin because it cannot flow through the biliary duct into the bowel.

Question 6

Briefly describe the management of biliary atresia

Answer 6

Management of biliary atresia is with surgery. The “Kasai portoenterostomy” involves attaching a section of the small intestine to the opening of the liver, where the bile duct normally attaches. This is somewhat successful and can clear the jaundice and prolong survival. Often patients require a full liver transplant to resolve the condition.