A 45-day-old term infant has progressive jaundice since the second week of life, pale acholic stools, and dark urine. Ultrasound shows a small or absent gallbladder. Total bilirubin is 9 mg/dL with conjugated fraction predominant. What is the next best step?
- A Start ursodeoxycholic acid and review at 3 months
- B Intraoperative cholangiography with liver biopsy, followed by hepatoportoenterostomy if confirmed ✓
- C Hepatobiliary iminodiacetic acid (HIDA) scan alone to confirm diagnosis
- D Percutaneous transhepatic cholecystostomy for drainage
Correct answer: B. Intraoperative cholangiography with liver biopsy, followed by hepatoportoenterostomy if confirmed
Explanation
The triad of jaundice persisting beyond 14 days, pale stools, and dark urine with a predominantly conjugated hyperbilirubinaemia points to biliary atresia. Diagnosis is confirmed by intraoperative cholangiogram showing no contrast entry into the biliary tree, supported by liver biopsy showing bile duct proliferation and portal fibrosis. Treatment is Kasai hepatoportoenterostomy, and outcomes are best when performed before 60 days of age, making early referral mandatory rather than observation.
Reference: Ashcraft's Pediatric Surgery, 6th ed.
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