Liver transplantation as definitive therapy for complications after arterial embolization for hepatic manifestations of hereditary hemorrhagic telangiectasia

Abstract

Experience with hepatic artery embolization for the treatment of symptomatic hepatic arteriovenous malformations (AVMs) in Rendu-Osler-Weber disease is limited. We report 2 cases of hepatic AVMs that caused mesenteric angina-like symptoms that were treated with embolization. Both patients developed parenchymal and bile duct necrosis, intrahepatic bilomas, and refractory biliary sepsis, subsequently leading to liver failure. We hypothesize that the pathophysiological cause of biliary necrosis in this setting is similar to that which occurs in the setting of hepatic artery thrombosis of the liver allograft. Progressive liver failure in these patients was treated successfully by liver transplantation. Liver transplantation offers definitive therapy by removing the source of ongoing sepsis, restoring normal liver function, and eliminating the intrahepatic AV shunt.