Distal portal venopathy: a rare cause of liver transplantation and a disease with a protean clinical presentation

We report 5 cases of liver transplantation which showed phlebosclerotic les ions of the distal portal vein on he explant confirming a diagnosis of hepa toportal sclerosis. This lesion was associated with nodular regenerative hy perplasia (2 cases), incomplete septal cirrhosis (4 cases) and tumors (2 ca ses, 1 adenoma and 1 hepatocellular carcinoma). Indications for transplant were chronic liver failure (1 case), encephalopathy without liver insuffici ency (2 cases) on adenoma (1 case), a liver mass (1 case). Three patients o ut of 5 had a past history of surgical portacaval shunts to treat variceal bleeding non related to cirrhosis, one had a spontaneous portacaval shunt a nd 2 had undergone a splenectomy tor pancytopenia. The review of liver biop sies (4 cases out of 5) performed during surgery showed distal portal vein phlebosclerotic lesions. The diagnosis of hepatoportal sclerosis associated with complications, which is obvious retrospectively, is seldom made prior to transplantation. Portacaval shunts could play at least a partial role i n the progressive deterioration of the liver.