Liver & intrahepatic bile ducts
Developmental anomalies / cysts
Congenital hepatic fibrosis

Topic Completed: 1 April 2012

Minor changes: 7 May 2021

Copyright: 2002-2021,, Inc.

PubMed Search: Congenital hepatic fibrosis[TI] free full text[sb]

Komal Arora, M.D.
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Cite this page: Arora K. Congenital hepatic fibrosis. website. Accessed May 12th, 2021.
Definition / general
  • Progressive lesion associated with autosomal recessive infantile polycystic kidney disease, mildly associated with autosomal dominant polycystic kidney disease
  • Often diagnosed in adolescents with portal hypertension
  • Complications: portal hypertension resulting in splenomegaly, hypersplenism, gastroesophageal varices, cholangitis
Diagrams / tables

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Pathogenesis: embryological and molecular perspective

Clinical features
  • Enlarged, abnormally shaped liver, relatively well preserved hepatocellular function
Gross description
  • Entire liver affected by microscopic cysts
  • Rarely macroscopic hepatic cysts
Microscopic (histologic) description
  • Defective remodeling of the ductal plate, abnormal branching of the intrahepatic portal veins, progressive fibrosis of portal tracts (GeneReviews: Congenital Hepatic Fibrosis Overview [Accessed 25 October 2017])
  • Anastomosing biliary channels in irregular, bland fibrous stroma, continuous with biliary tree, hepatocyte nodules with central veins and normal architecture
  • Marked proliferation of bile ductules
  • Angulated bile ducts often with inspissated bile in fibrotic portal tracts with portal - portal bridging fibrosis
  • No inflammation, no regenerative nodules
Microscopic (histologic) images

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Portal fibrosis with dilated biliary channels

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