Liver & intrahepatic bile ducts
Developmental anomalies / cysts
Congenital hepatic fibrosis
Author: Komal Arora, M.D.
Topic Completed: 1 April 2012
Minor changes: 11 March 2020
Copyright: 2002-2020, PathologyOutlines.com, Inc.
PubMed Search: Congenital hepatic fibrosis[TI] free full text[sb]
Table of Contents
Definition / general | Diagrams / tables | Clinical features | Gross description | Microscopic (histologic) description | Microscopic (histologic) imagesCite this page: Arora K. Congenital hepatic fibrosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livercongenitalhepaticfibrosis.html. Accessed August 14th, 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
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
