A case of arterial reconstruction for multiple hepatic artery aneurysms complicated by hereditary hemorrhagic telangiectasia

Abstract

Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant genetic disorder that manifests as mucous telangiectasia and arteriovenous malformations (AVMs) in the major organs. Although HHT type 2 often involves intrahepatic AVMs or aneurysms, which lead to high output and portal hypertension, extrahepatic aneurysm is a rare condition. Herein, we report a case of successful surgical intervention for an extrahepatic aneurysm in a patient with HHT type 2. Pathological findings of the resected aneurysm included hypoplasia of the media and absence of the intimal elastic lamina without atherosclerotic change. We believe that surgical resection is a favorable procedure for a patient with hepatic artery aneurysms concomitant with HHT.