Gastro - Biliary Atresia

Question 1

What is biliary atresia?

Answer 1

Congenital condition where section of bile duct is narrowed or absent

Question 2

What does biliary atresia result in?

Answer 2

Cholestasis

Bile can’t be transported from liver to bowel

Conjugated bilirubin excreted in bile

Question 3

How does biliary atresia present?

Answer 3

Shortly after birth
Significant jaundice due to high conjugated bilirubin levels

Question 4

When should biliary atresia be suspected?

Answer 4

Babies with persistent jaundice

More than 14 days in term babies

More than 21 days in premature babies

Question 5

What is the initial investigation for biliary atresia?

Answer 5

Conjugated and unconjugated bilirubin levels

High conjugated bilirubin suggests liver processing it (by conjugating it) not able to excrete it as unable to flow through biliary duct to bowel

Question 6

What are the majority of cases of jaundice in new-borns caused by?

Answer 6

Breast milk jaundice

More serious cases e.g. biliary atresia, need to exclude by measuring conjugated bilirubin

Question 7

How is biliary atresia managed?

Answer 7

Kasai portoenterostomy

Question 8

What is a Kasai portoenterostomy

Answer 8

Attaching section of small intestine to opening of the liver where bile duct normally attaches

Good results and can clear jaundice

Patients will then go on to require full liver transplant to resolve the condition