Hereditary-hemorrhagic telangiectasia: one-step magnetic resonance examination in evaluation of liver involvement

To describe the magnetic resonance imaging (MRI) features of hepatic involvement in hereditary-hemorrhagic telangiectasia (HHT) and to determine the interobserver agreement for all of them.

Twenty-three consecutive patients (including 17 women, mean age: 55 years) with HHT, according to the Curaçao criteria, underwent prospective MRI of the liver, including parenchymal, angiographic and biliary sequences, in one step. The scans were analyzed to determine the presence of vascular, biliary and parenchymal abnormalities (Mann-Whitney U test, kappa). The diameters of the hepatic vessels in the 23 patients were compared with those of 23 subjects with no signs of HHT or vascular or liver disease.

MRI of the liver was abnormal in 21 patients with suspected HHT (91%). Vascular abnormalities were found in 21 patients (91%), consisting of marked dilatation of the hepatic artery (N=14), intrahepatic telangiectases (N=21), arteriosystemic venous shunting (N=19), arterioportal shunting (N=11) and aneurysms of the hepatic artery (N=3). Regenerative nodular hyperplasia was identified in 17 patients (74%) and ischemic cholangitis in nine (39%). No such lesions were found in the controls. The diameter of the hepatic artery proper was greater in patients with HHT than in the controls: 8.69+/-1.63 mm versus 5.17+/-0.44 mm, respectively (P<0.05). Good interobserver agreement was found with parenchymal and vascular abnormalities (0.62) and moderate interobserver agreement (0.42) with biliary abnormalities.

One-step MRI of the liver appears to be an excellent tool for the evaluation of liver involvement in HHT, revealing vascular abnormalities, telangiectases, arteriovenous shunting, focal-liver lesions and ischemic cholangitis.