A 3-week-old term infant has persistent jaundice since day 10. Total bilirubin is 9 mg/dL with conjugated fraction 6 mg/dL. Stools are pale and urine stains the nappy dark. Hepatobiliary scintigraphy shows no excretion into the gut. Intraoperative cholangiography shows no opacification of the extrahepatic biliary tree. The most likely diagnosis is:

• A Neonatal hepatitis
• B Physiological jaundice of the newborn
• C Gilbert syndrome
• D Extrahepatic biliary atresia ✓

Explanation

Conjugated hyperbilirubinaemia with acholic stools and complete failure of biliary excretion in the first month defines extrahepatic biliary atresia, caused by progressive inflammatory destruction of the extrahepatic ducts leading to fibrosis. Kasai portoenterostomy before about 60 days offers the best chance of bile flow, though many children still progress to cirrhosis. Physiological jaundice and Gilbert syndrome cause unconjugated hyperbilirubinaemia, and neonatal hepatitis usually retains some excretion.

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.