Ianora Amato Antonio Stabile, Memeo Maurizio, Sabba Carlo, Cirulli Anna, Rotondo Antonio, Angelelli Giuseppe
Department of Radiology and HHT Centre, University Hospital, Policlinico of Bari, Piazza Giulio Cesare 11, 70124 Bari, Italy.
Radiology. 2004 Jan;230(1):250-9. doi: 10.1148/radiol.2301021745. Epub 2003 Nov 26.
To describe findings obtained with multi-detector row helical computed tomography (CT) of the liver in patients with hereditary hemorrhagic telangiectasia.
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.
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%).
Multi-detector row helical CT and reconstructions depict the complex hepatic vascular alterations typical of hereditary hemorrhagic telangiectasia.
描述遗传性出血性毛细血管扩张症患者肝脏的多排螺旋计算机断层扫描(CT)检查结果。
对70例连续患者(29例女性和41例男性;平均年龄48.5岁;年龄范围15 - 75岁)进行多期多排螺旋CT检查:64例被认为患有遗传性出血性毛细血管扩张症,6例疑似患有该病。通过使用造影剂团注试验来实现扫描延迟,以获取动脉早期、动脉晚期和门静脉期图像。然后进行多平面和血管造影重建。由两位放射科医生共同评估分流、肝脏灌注障碍、毛细血管扩张、其他血管病变、门静脉高压的间接征象以及血管解剖变异的存在情况。
70例患者中有52例(74%)存在肝脏血管异常。52例患者中只有4例(8%)有症状。52例患者中有27例(52%)存在动静脉分流,8例(15%)存在动体分流,17例(33%)同时存在两种分流类型。52例患者中有34例(65%)检测到实质灌注障碍。52例患者中有33例(63%)发现毛细血管扩张。52例患者中有13例(25%)发现大的融合性血管团块。52例患者中有31例(60%)检测到门静脉高压的CT间接征象,但只有1例有该病症的临床体征。7例患者(13%)检测到血管解剖变异。
多排螺旋CT及重建可描绘出遗传性出血性毛细血管扩张症典型的复杂肝脏血管改变。