Effective therapy for hepatic M. Osier with systemic hypercirculation by ligation of the hepatic artery and subsequent liver transplantation

Abstract

Hereditary hemorrhagic teleangiectasia, or M. Osler (OslerWeber-Rendu disease), is an autosoma1 dominant, systemic fibrovascular dysplasia. This may lead to increased liver blood flow from arteriovenous fistulas. A 45-year-old woman with a known M. Osler was admitted for liver transplantation. On admission, exertional dyspnea was the predominant symptom. Radiological investigations revealed multiple intrahepatic arteriovenous fistulas and consecutive high-output heart failure. Laboratory findings revealed remarkably elevated bilirubin and alkaline phosphatase. To alleviate the high-output cardiac failure, the hepatic artery was ligated. Fourteen months later, the patient presented again with increased levels of bilirubin and recurrent bleeding episodes from esophageal 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, however, is recommended only for patients with normal liver function and carries a considerable risk of bile duct necrosis.

Hepatic involvement in hereditary hemorrhagic telangiectasia: An unusual indication for liver transplantation

“…Total hepatectomy and liver transplantation as the only chance for survival has been described once before in the literature. 10 In our case, the decision for liver transplantation was taken in view of the panhepatic involvement with biliary dilatation and cholangitis that could not be controlled medically. It was decided that because the liver was the source of the systemic sepsis, total removal of the liver was the only chance of survival for the patient.…”