A 5-week-old full-term infant has persistent jaundice since the second week of life. Stools are pale clay coloured and urine stains the nappy dark yellow. Total bilirubin is 9 mg/dL with conjugated fraction of 7 mg/dL. Ultrasound shows an absent or atretic gallbladder. What is the most appropriate next step?

• A Ursodeoxycholic acid trial for four weeks and review
• B Hepatobiliary scintigraphy followed by intraoperative cholangiography and portoenterostomy if confirmed ✓
• C Percutaneous liver biopsy alone with annual follow-up
• D Reassurance, as this is breast milk jaundice

Explanation

Acholic stools, dark urine, and rising conjugated hyperbilirubinaemia beyond two weeks define biliary atresia until proven otherwise. Confirmation is by hepatobiliary scan showing no excretion into the gut and intraoperative cholangiography. Treatment is the Kasai hepatoportoenterostomy, which must be performed before 60 days of age for reasonable bile drainage; after 90 days success falls sharply. Breast milk jaundice is unconjugated, which kills that distractor. Ursodiol delays definitive surgery and worsens outcome.

Reference: Nelson Textbook of Pediatrics, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.