EFFECTIVE THERAPY FOR HEPATIC OSLER,M. WITH SYSTEMIC HYPERCIRCULATIONBY LIGATION OF THE HEPATIC-ARTERY AND SUBSEQUENT LIVER-TRANSPLANTATION

Hereditary hemorrhagic teleangiectasia, or M. Osler (Osler-Weber-Rendu disease), is an autosomal dominant, systemic fibrovascular dysplasia. This may lead to increased liver blood now from arteriovenous fistula s. A 45-year-old woman with a known M. Osler was admitted for liver tr ansplantation. On admission, exertional dyspnea was the Predominant sy mptom. Radiological investigations revealed multiple intrahepatic arte riovenous fistulas and consecutive high-output heart failure. Laborato ry findings revealed remarkably elevated bilirubin and alkaline phosph atase. To alleviate the high-output cardiac failure, the hepatic arter y was ligated. Fourteen months later, the patient presented again with increased levels of bilirubin and recurrent bleeding episodes from es ophageal varices grade IV. The patient underwent liver transplantation and posttransplant recovery was excellent. A hyperdynamic circulatory state due to a hepatic M. Osler has been treated in several cases by ligation or embolization of the hepatic artery. This procedure, howeve r, is recommended only for patients with normal liver function and car ries a considerable risk of bile duct necrosis.