Hereditary hemorrhagic telangiectasia: Multi-detector row helical CT assessment of hepatic involvement

PURPOSE: To describe findings obtained with multi-detector row helical computed tomography (CT) of the liver in patients with hereditary hemorrhagic telangiectasia. MATERIALS AND METHODS: Multiphasic multi-detector row helical CT was performed in 70 consecutive patients (29 females and 41 males; mean age, 48.5 years; age range, 15-75 years): 64 considered to have hereditary hemorrhagic telangiectasia and six suspected of having the disease. Scanning delay was achieved by using a test bolus of contrast medium to obtain early arterial phase, late arterial phase, and portal venous phase images. Multiplanar and angiographic reconstructions were then generated. The presence of shunts, hepatic perfusion disorders, telangiectases, other vascular lesions, indirect signs of portal hypertension, and vascular anatomic variants were evaluated by two radiologists in consensus. RESULTS: Fifty-two of 70 (74%) patients had hepatic vascular abnormalities. Only four of 52 (8%) patients were symptomatic. Arterioportal shunts were present in 27 of 52 (52%) patients, arteriosystemic shunts in eight of 52 (15%), and both shunt types in 17 of 52 (33%). In 34 of 52 (65%) patients, parenchymal perfusion disorders were detected. Telangiectases were found in 33 of 52 (63%) patients. Large confluent vascular masses were identified in 13 of 52 (25%) patients. In 31 of 52 (60%) patients, indirect CT signs of portal hypertension were detected, but only one had clinical signs of this condition. Vascular anatomic variants were detected in seven patients (13%). CONCLUSION: Multi-detector row helical CT and reconstructions depict the complex hepatic vascular alterations typical of hereditary hemorrhagic telangiectasia. (C) RSNA, 2003.