Hepatic involvement in hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease).

Abstract

Hepatic involvement in hereditary hemorrhagic telangiectasia is infrequent and poorly studied. We describe a 62-year-old woman with Rendu-Osler-Weber (ROW) disease and recurrent gastrointestinal bleeding episodes. Blood chemistry was consistent with the presence of cholestasis. Imaging studies revealed prominent vascular abnormalities in the liver and focal intrahepatic bile duct dilatations. The intimate anatomic relationship of the vascular abnormalities to the dilated bile ducts suggests that external vascular compression could have caused their dilatation. To our knowledge, this mechanism has not been proposed in the literature as a possible explanation of biliary dilatation in patients with ROW.