遗传性出血性毛细血管扩张症累及肝脏的CT及DSA表现  

Hepatic involvement of hereditary hemorrhagic telangiectasia:its imaging findings on CT and DSA

作　　者：李艳[1] 吕传剑[1] 吴学军 吴青霞[1] 孙明华[1] 葛英辉[1] 

机构地区：[1]河南省人民医院放射科,郑州450003 [2]郑州市卫生学校外科教研室

出　　处：《介入放射学杂志》2013年第10期854-857,共4页Journal of Interventional Radiology

基　　金：河南省科技攻关项目(132102310423)

摘　　要：目的探讨遗传性出血性毛细血管扩张症(hereditary hemorrhagic telangiectasia,HHT)累及肝脏的CT和DSA表现。方法回顾性分析2005年9月至2012年10月6例HHT累及肝脏患者的临床及CT、DSA影像学资料。结果 CT增强表现为动脉期肝动脉及其分支迂曲扩张,肝静脉和门静脉早显、增粗。此期肝内见多发大小不等的灶状、小片状强化。门脉期部分病灶持续强化,同时肝实质强化,肝动脉密度减低,门静脉及肝静脉显示更清晰。CTA显示肝动脉变异。DSA表现为肝动脉及其分支明显增粗、迂曲;肝实质呈弥漫性团块状染色,肝静脉提前显影,门静脉主干及肝内分支增粗,还可见肝动脉变异以及肠系膜上动脉窃血。结论 CT增强及DSA在HHT肝脏受累有特征性表现,对HHT的诊断具有重要价值。Objective To investigate CT and digital subtraction angiography （DSA） features of liver involvement in hereditary hemorrhagic telangiectasia （HHT）. Methods Six patients of HTF with hepatic involvement, who were encountered at the hospital during the period from Sep. 2005 to Oct. 2012, were enrolled in this study. The clinical data as well as CT and DSA findings were retrospectively analyzed. Results Enhanced CT scanning showed that on arterial phase tortuous and dilated hepatic artery and its branches, as well as early visualization of thickened hepatic and portal veins could be seen. Muhiple focal and small patchy enhancement of different sizes were revealed in liver. On portal phase some lesions showed continuous enhancement, meanwhile the liver parenchyma became enhanced and hepatic arterial density decreased and the portal as well as the hepatic veins stood out more clearly. Computed tomography angiography （CTA） could demonstrate the anatomical variations of hepatic artery. On DSA the lesions were manifested as thickened and tortuous hepatic arteries with diffuse massive staining in liver parenchyma and early filling of the hepatic veins. Besides, thickened portal stem and its intrahepatic branches, abnormal hepatic artery as well as superior mesenteric artery steal syndrome could also be found. Conclusion On enhanced CT scanning and DSA, liver involvement in hereditary hemorrhagic telangiectasia has characteristic features, which are very helpful in diagnosing this disease.（J Intervent Radiol, 2013, 22： 854-857）

关 键 词：遗传性出血性毛细血管扩张症 肝脏 计算机断层扫描 血管造影 

分 类 号：R55[医药卫生—血液循环系统疾病]