Congenital hepatic fibrosis

Liver and intrahepatic bile ducts - nontumor
Developmental anomalies / cysts
Congenital hepatic fibrosis

Topic Completed: 1 April 2012

Minor changes: 11 March 2020

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

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Table of Contents

Cite this page: Arora K. Congenital hepatic fibrosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livercongenitalhepaticfibrosis.html. Accessed May 27th, 2020.

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

Images hosted on other servers:

Portal fibrosis with dilated biliary channels