MR Imaging the Hepatic Manifestations and Complications of Hereditary Hemorrhagic Telangiectasia: A Pictorial Review

Christina Marie Chingkoe¹, Errol Colak¹, Vikram Prabhudesai¹, Marie Faughnan², Anish Kirpalani¹

Hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant disorder of variable penetrance, is characterized by the presence of mucocutaneous and visceral vascular malformations (VMs). Hepatic involvement occurs in 40% of patients with HHT. We perform liver MRI routinely in HHT patients to document hepatic VMs and to evaluate for hepatic complications of HHT. In this presentation, the MRI features of the hepatic manifestations of HHT are reviewed. These include intrahepatic shunting (arterioportal, arteriosystemic and portosystemic), discrete VMs (capillary telangiectasia, confluent vascular masses, arteriovenous malformations), focal nodular hyperplasia, sclerosing cholangitis from ischemic biliopathy, and potential complications (including hemosiderosis, cholangitis, intrahepatic abscess).