A 3-week-old term infant has persistent jaundice, pale acholic stools, and dark urine. Fractionated bilirubin shows conjugated hyperbilirubinemia. Intraoperative cholangiogram confirms complete obliteration of the extrahepatic biliary tree. The definitive management is:

• A Ursodeoxycholic acid therapy with fat-soluble vitamin supplementation alone
• B Liver transplantation deferred until the child reaches 5 years of age
• C Phenobarbital priming followed by phototherapy
• D Kasai portoenterostomy performed before 60 days of age ✓

Explanation

Biliary atresia is the most common surgically correctable cause of neonatal conjugated hyperbilirubinemia, characterized by progressive fibrosing obliteration of extrahepatic ducts with bile plugs, ductular proliferation, and portal edema on biopsy. Kasai hepatoportoenterostomy restores some bile flow and must be done early, ideally before 60 days, because success falls sharply afterward; many children still need eventual transplantation.

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.