遗传性出血性毛细血管扩张症的综合影像学分析  

Comprehensive Imaging Analysis of Hereditary Hemorrhagic Telangiectasia

作　　者：郝传玺[1] 金龙[1] 陈尘[1] 高健[1] 胡立宝 洪楠[1] 

机构地区：[1]北京大学人民医院放射科,100044

出　　处：《临床放射学杂志》2014年第3期373-376,共4页Journal of Clinical Radiology

摘　　要：目的探讨遗传性出血性毛细血管扩张症(HHT)的影像学特征。方法结合6例HHT患者的临床及影像资料,系统分析该病的影像学特征。6例患者均行腹部CT平扫及增强扫描,3例行上腹部MRI平扫及增强扫描,2例行胸部CT平扫及增强扫描,1例行肠镜检查,1例行DSA检查。结果 CT及MRI检查示病变特征包括:肝大、肝门区血管迂曲增宽紊乱、肝实质结节样强化、肝内胆管扩张、肝肺内动静脉畸形及脾大出血等。DSA示肝动脉及肠系膜动脉末梢分支增粗,动静脉畸形,静脉早显。结论 HHT表现为累及多脏器的血管发育异常,具有一定特征性,胸腹部CT及MRI扫描是诊断HHT的可靠方法。Objective To investigate the imaging manifestations of hereditary hemorrhagic telangiectasia （HHT）. Methods The imaging features of six HHT patients were analyzed and the related clinical data were reviewed. Plain and contrast enhanced CT scan were conducted in all of six patients, three of them underwent MR scan, two of them performed plain and contrast enhanced CT scan, one had gastroscopy examination, and one carried out angiography. Results CT and MR features included hepatomegaly, expanding, circuiting, mussy vessel in porta hepatis, nodular heterogenous parenchymal enhancement pattern, intrahepatic arteriovenous malformation, early filling of intraheptic vein, pulmonary arteriovenous malformations, intrahepatic biliary dilation, splenomegaly with hemangioma and bleeding; DSA showed dilation of arteriole endings in hepatic artery and mesenteric artery, early filling of draining vein. Conclusion Hereditary hemorrhagic telangiectasia involves abnormal vascular development of multiple organs with certain characteristics; conventional and enhanced CT and MRI scan are reliable imaging methods for diagnosis of hereditary hemorrhagic telangiectasia.

关 键 词：遗传性出血性毛细血管扩张症 计算机断层扫描 磁共振成像 数字减影血管造影 

分 类 号：R596.1[医药卫生—内科学] R445.2[医药卫生—临床医学] R816.5