A 6-week-old full-term girl has persistent jaundice since the second week of life. Stools are pale and urine stains the nappy dark. Total bilirubin is 9 mg/dL with a conjugated fraction of 7 mg/dL. Hepatobiliary scintigraphy shows no intestinal excretion. What is the most appropriate next step?

• A Ursodeoxycholic acid trial for 8 weeks before any surgery
• B Referral for primary liver transplantation as first-line treatment
• C Percutaneous liver biopsy followed by observation until 6 months of age
• D Intraoperative cholangiography with proceeding to hepatoportoenterostomy if the diagnosis is confirmed ✓

Explanation

Conjugated jaundice with acholic stools and non-excreting scintigraphy in a 6-week-old indicates biliary atresia. The Kasai hepatoportoenterostomy must be performed early, ideally before 60 days of age, because success falls sharply with delay, so operative cholangiography and portoenterostomy should not be postponed. Transplantation is reserved for failed Kasai or late presentation, not first-line. Medical therapy alone cannot restore bile flow in atresia.

Reference: Bailey and Love's Short Practice of Surgery, 27th ed.

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Written and medically reviewed by the StethoPrep medical team.