Hepatic involvement in hereditary hemorrhagic telangiectasia - Helical computed tomography features in 24 consecutive patients

Objective: Among the various organs that may be affected by hereditary hemorrhagic telangiectasia (HHT), the liver can show various degrees of vascular and parenchymal involvement. The purpose of this prospective study comprising a large series of patients was to reassess the computed tomography (CT) features of hepatic involvement in HHT using helical CT. Methods: Twenty-four consecutive patients with HHT had prospective helical CT of the liver, including noncontrast, arterial-dominant, and portal-dominant phases. The CT images were analyzed by 2 readers in consensus to determine the presence of vascular and parenchymal abnormalities. The diameter of the proper hepatic artery in these 24 patients was compared with that in 24 healthy subjects (Student t test). Results: Helical CT was normal in 5 patients (21%) and abnormal in 19 patients (79%). Vascular abnormalities were found in 16 patients (67%), consisting of marked dilatation of the hepatic artery (n = 16), intrahepatic telangiectases (n = 12), arteriovenous shunting (n = 5), and arterioportal shunting (n = 3). The diameter of the proper hepatic artery was greater in the patients with HHT than in control subjects (6.12 +/- 2.52 mm vs. 3.29 +/- 0.65 mm, respectively; P < 0.05). Helical CT showed nodular hyperplasia in 1 patient with vascular and parenchymal abnormalities, cavernous hemangiomas in 2 patients (I ill a patient with an enlarged hepatic artery, intrahepatic telangiectases, and arteriovenous shunting and 1 in a patient with an isolated enlarged hepatic artery), and biliary cysts in 3 patients (2 biliary cysts were present in 2 patients with an enlarged hepatic artery and intrahepatic telangiectases, and 1 biliary cyst was present without any manifestations in the third patient). Conclusions: Liver involvement in HHT is associated with a constellation of findings on helical CT, including significant dilatation of the proper hepatic artery, telangiectases, arteriovenous shunting, and focal liver lesions. Familiarity with these findings will result in more accurate diagnosis and allows better therapeutic options if necessary.