[Hereditary hemorrhagic telangiectasia with liver involvement]

Abstract

Rendu-Osler-Weber syndrome, or hereditary hemorrhagic telangiectasia, is characterized by vascular alterations that tend to hemorrhage. We present a 56-year-old woman, with a diagnosis of chronic liver disease of unknown origin 15 years earlier, who came to our hospital as a liver transplantation candidate following hemorrhage due to esophageal varices. Rendu-Osler-Weber syndrome can affect the liver in several ways although vascular alterations can be distinguished from those produced in the liver parenchyma. The different types of vascular alterations in the liver may manifest as as telangiectasias, angiomas, aneurysms of the hepatic artery and fistulae between different vessels. This syndrome may also produce alterations in the liver parenchyma although the exact pathogenic mechanisms are unknown. There are three main forms of treatment: embolization of the branches of the hepatic artery, ligature of the hepatic artery and liver transplantation.