|
|
|
|
Portosystemic encephalopathy may appear in the course of acute or chronic hepatocellular disease or as a complication of portosystemic shunting.Most of the patients in whom this complex organic brain syndrome develops have, in fact,elements of both liver disease and shunting through collaterals formed as a result of portal hypertension[1].Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu disease is an autosomal dominant disorder characterized by widespread fibrovascular malformations in the skin,mucous membranes,and internal organs leading to recurrent hemorrhage[ 2,3].Liver involvement is not uncommon[4-7].The occurrence of portosystemic encephalopathy in hereditary hemorrhagic telangiectasia is a rare event and has been attributed to severe hepatocellular disease or portal hypertension in most cases[8,9].We describe two patients with hereditary hemorrhagic telangiectasia and normal liver function,in whom portosystemic encephalopathy developed during bleeding episodes.The encephalopathy could only be explained by portosystemic shunting due to intrahepatic telangiectases. |
|