Liver and intrahepatic bile ducts-nontumor
Congenital hepatic fibrosis
Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 23 April 2012, last major update April 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.
● 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
Embryological and molecular perspective
● Enlarged, abnormally shaped liver, relatively well-preserved hepatocellular function
● Entire liver affected by microscopic cysts
● Rarely macroscopic hepatic cysts
● Defective remodeling of the ductal plate, abnormal branching of the intrahepatic portal veins, progressive fibrosis of portal tracts (GeneReviews™ [Internet]; 1993-2008 Dec 09)
● 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
Portal fibrosis with dilated biliary channels
End of Liver and intrahepatic bile ducts-nontumor > Developmental anomalies/cysts > Congenital hepatic fibrosis
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