A 3-week-old term infant born after an uncomplicated pregnancy develops persistent jaundice. Total bilirubin is 9 mg/dL with direct fraction 6 mg/dL. Stools are pale clay-colored, urine stains the diaper dark. Ultrasound shows a small or absent gallbladder, and hepatobiliary scintigraphy shows no intestinal tracer passage. Intraoperative cholangiography confirms obliteration of the extrahepatic ducts. The definitive treatment is:
- A Kasai portoenterostomy before 60 days of age ✓
- B Ursodeoxycholic acid and fat-soluble vitamin supplementation
- C Phenobarbital priming followed by phototherapy
- D Medium-chain triglyceride formula feeding with observation
Explanation
This is extrahepatic biliary atresia, the leading cause of neonatal cholestasis requiring surgery. Progressive obliterative destruction of extrahepatic bile ducts produces acholic stools, conjugated hyperbilirubinemia, and eventual biliary cirrhosis. Kasai hepatoportoenterostomy establishes bile drainage and must be performed early, ideally before 60 days of life, as success falls sharply thereafter; most children still ultimately require liver transplantation. Ursodiol, phototherapy, and dietary measures are supportive only and delay definitive care, worsening outcomes.
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.