CASE-REPORT - SECONDARY BILIARY-CIRRHOSIS POSSIBLY RELATED TO CONGENITAL HEPATIC-FIBROSIS - EVIDENCE FOR DECREASED NUMBER OF PORTAL BRANCH VEINS AND HYPERTROPHIC PERIBILIARY VASCULAR PLEXUS

A 30-year-old man with presinusoidal portal hypertension was transplan ted for cryptogenic cirrhosis. On the explanted liver, few intrahepati c stones, biliary cirrhosis, chronic cholangitis of the large bile duc ts and a peculiar proliferation of small dilated bile ducts at the per iphery of the portal tracts led to the diagnosis of secondary biliary cirrhosis and cholangitis, possibly linked to ductal plate malformatio n, including congenital hepatic fibrosis associated with a minor form of Caroli's disease. Ex vivo portogram and histology showed the paucit y of portal vein branches and the hypertrophy of the peribiliary vascu lar plexus. This hypertrophy, which has been reported in livers with p resinusoidal hypertension, is another indirect argument to suggest the diagnosis of congenital hepatic fibrosis.