A 5-week-old term boy has persistent conjugated hyperbilirubinemia, acholic stools, and dark urine. Ultrasound shows no visible gallbladder. Liver biopsy reveals bile plugs within proliferated portal ductules, ductular proliferation, and early portal fibrosis. What is the most appropriate next step?

• A Ursodeoxycholic acid trial for six weeks before any surgery
• B Liver transplantation as first-line treatment
• C Hepatoportoenterostomy performed urgently ✓
• D Phenobarbital challenge to distinguish biliary atresia from neonatal hepatitis

Explanation

Extrahepatic biliary atresia is the commonest surgically correctable cause of neonatal cholestasis. Biopsy showing bile plugs and ductular proliferation supports atresia over neonatal hepatitis, where lobular disarray and giant cell transformation dominate. Kasai hepatoportoenterostomy must be done early, ideally before 60 days of age, because success falls sharply with delay; transplantation is reserved for failed portoenterostomy or late presentation. Drug trials waste critical time and are not diagnostic.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.