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

Author: Komal Arora, M.D. (see Authors page)

Revised: 25 October 2017, last major update April 2012

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

Cite this page: Arora, K. Congenital hepatic fibrosis. PathologyOutlines.com website. http://pathologyoutlines.com/topic/livercongenitalhepaticfibrosis.html. Accessed December 14th, 2017.
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

Images hosted on other servers:

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