A 6-week-old term infant presents with persistent jaundice, pale acholic stools, and dark urine. Total bilirubin is 8 mg/dL with conjugated fraction 6 mg/dL. Ultrasound shows an absent or atretic gallbladder, and hepatobiliary scintigraphy shows no intestinal excretion. What is the definitive next step?

• A Ursodeoxycholic acid therapy and repeat LFTs at 3 months
• B Cholecystostomy for external drainage of bile
• C Percutaneous liver biopsy followed by Kasai portoenterostomy without delay ✓
• D Orthotopic liver transplantation as primary treatment

Explanation

Biliary atresia is the commonest cause of obstructive neonatal jaundice requiring surgery. The combination of conjugated hyperbilirubinaemia, acholic stools, and non-excreting HIDA scan demands urgent workup, and percutaneous biopsy showing bile duct proliferation with bile plugs confirms the diagnosis. The Kasai hepatoportoenterostomy should be done before 60 days of age because success falls sharply after that window. Transplantation is reserved for failed Kasai or late presentation, and medical therapy alone is never adequate.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.