de Lédinghen V, Le Bail B, Trillaud H, Bernard P H, Saric J, Balabaud C, Bioulac-Sage P
Unité Fonctionnelle de Transplantation Hépatique, Hôpital Pellegrin, CHU Bordeaux, France.
J Gastroenterol Hepatol. 1998 Jul;13(7):720-4. doi: 10.1111/j.1440-1746.1998.tb00720.x.
A 30-year-old man with presinusoidal portal hypertension was transplanted for cryptogenic cirrhosis. On the explanted liver, few intrahepatic stones, biliary cirrhosis, chronic cholangitis of the large bile ducts and a peculiar proliferation of small dilated bile ducts at the periphery of the portal tracts led to the diagnosis of secondary biliary cirrhosis and cholangitis, possibly linked to ductal plate malformation, including congenital hepatic fibrosis associated with a minor form of Caroli's disease. Ex vivo portogram and histology showed the paucity of portal vein branches and the hypertrophy of the peribiliary vascular plexus. This hypertrophy, which has been reported in livers with presinusoidal hypertension, is another indirect argument to suggest the diagnosis of congenital hepatic fibrosis.
