Pathology · Hepatobiliary and Pancreatic Pathology

A 3-week-old term infant has persistent jaundice with dark urine and acholic stools. Total bilirubin is 9 mg/dL with direct fraction 6 mg/dL. Intraoperative cholangiogram shows no opacification of the extrahepatic biliary tree. Liver biopsy is most likely to show:

  • A Paucity of interlobular bile ducts with a normal portal tract number
  • B Granulomatous destruction of interlobular bile ducts
  • C Marked bile ductular proliferation in expanded portal tracts with bile plugs
  • D Giant cell transformation confined to zone 3 hepatocytes without portal changes
Correct answer: C. Marked bile ductular proliferation in expanded portal tracts with bile plugs

Explanation

Extrahepatic biliary atresia presents as neonatal cholestasis with acholic stools and requires Kasai portoenterostomy before 8 weeks of age. The biopsy shows edematous, fibrotic portal tracts with striking bile ductular proliferation and bile plugs, reflecting complete mechanical obstruction. Granulomatous duct destruction defines primary biliary cholangitis, duct paucity characterizes Alagille syndrome and nonsyndromic paucity, and isolated giant cell change without ductular reaction suggests neonatal hepatitis, the chief biopsy differential here.

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

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