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Case of the Month: January 2023

posted: January 25, 2023

This is a 59 year old female with history of HIV who presented with upper gastrointestinal bleeding. Upper endoscopy was performed and shows esophageal and upper gastric varices. Liver U/S biopsy was performed along with portal pressure gradient. Histopathology slide is provided below.

View Final Diagnosis
Final Diagnosis: Congenital Hepatic Fibrosis
Congenital hepatic fibrosis (CHF) is a ductal plate malformation characterized by diffuse persistence of the ductal plate in the background of dense fibrous tissue. Clinically, CHF may be present in early life in patients with autosomal recessive polycystic kidney disease or in adulthood/older age as incidental hepatosplenomegaly with or without esophageal bleeding. Microscopically, the liver biopsy shows diffuse presence of irregularly shaped and branching bile ducts with open lumina in the background of fibrosis in portal tracts. Usually, the portal veins in CHF can be obliterated, rudimentary, or hypoplastic while hepatic arteries can be thickened, numerous, and coursing through the dense fibrotic stroma. Interestingly, there is no bile ductular proliferation, bile duct loss, significant portal inflammation, or marked cholestasis. Prominent portal fibrosis is present and can distend the portal tracts, distort them, and ultimately form bridging fibrosis which can histologically mimic cirrhosis.
  1. Congenital hepatic fibrosis and its mimics: a clinicopathologic study of 19 cases at a single institution

This case is contributed by Haidar Mejbel MD and Zaid Mahdi MD. Department of Pathology, Emory University Hospital.