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General Surgery · Paediatric hepatobiliary: biliary atresia
Sample itemA 5-week-old breastfed girl has persistent jaundice, pale stools and dark urine. Conjugated bilirubin is 7 mg/dL, ultrasound shows a triangular cord sign at the porta hepatis and a hepatobiliary scan shows no excretion into the bowel at 24 hours. What is the best management?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Intraoperative cholangiogram and Kasai portoenterostomy without delay
Biliary atresia is progressive obliteration of the extrahepatic ducts causing conjugated hyperbilirubinaemia in the neonate. The Kasai hepatoportoenterostomy gives the best drainage if done before about 8 weeks of age, and transplantation is reserved for failure of the Kasai or for late presentation. Delay worsens fibrosis and survival with the native liver.
Option by option
A. Intraoperative cholangiogram and Kasai portoenterostomy without delay (correct)
Correct. Early Kasai portoenterostomy, ideally before 8 weeks, offers the best chance of bile flow.
B. Observation and repeat bilirubin at 3 months
Waiting for 3 months allows irreversible cirrhosis and makes drainage surgery much less effective.
C. Liver transplantation as the primary procedure now
Transplantation is a salvage option after failed Kasai and not the first procedure in an infant who can still be drained.
D. Phenobarbitone and breastfeeding cessation only
Medical treatment cannot relieve a mechanical obliteration of the bile ducts.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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