Biliary Atresia

Question 1

What is Biliary Atresia?

Answer 1

• Biliary Atresia is a congenital condition where a section of the bile duct is narrowed or absent

Question 2

What is the pathophysiology of Biliary Atresia?

Answer 2

• Due to the bile duct narrowing/ being absent
• There is cholestasis whereby the bile cannot be transported from the liver to the bowel
• Conjugated bilirubin is normally excreted in the bile
• Therefore biliary atresia prevents the excretion of conjugated bilirubin

Question 3

How does Biliary Atresia present?

Answer 3

• After birth with significant jaundice due to high conjugated bilirubin levels
• Suspect biliary atresia in babies with persistent jaundice
• Investigations will show high levels of conjugated bilirubin

Question 4

What is the Managment for Biliary Atresia?

Answer 4

• 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 prolong survival
• However, often patients require a full liver transplant to resolve the condition