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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 features of hereditary hemorrhagic telangiectasia (HHT). Methods The clinical and imaging data of 6 HHT patients were analyzed systematically to analyze the imaging features of the disease. Six patients underwent abdominal CT scan and contrast-enhanced scan. Three patients underwent abdominal plain MRI and contrast-enhanced scan. Two patients underwent CT scan and enhanced CT scan. One patient underwent colonoscopy and one patient underwent DSA. Results CT and MRI showed pathological features including enlarged tortuous widening of hepatic and hepatic portal, enhancement of hepatic parenchymal nodules, dilatation of intrahepatic bile duct, arteriovenous malformation of liver and lung and splenic hemorrhage. DSA showed hepatic artery and mesenteric artery peripheral branches thickening, arteriovenous malformations, veins early. Conclusion HHT manifests as vascular dysfunction involving multiple organs with certain characteristics. Chest and abdomen CT and MRI scan are reliable methods to diagnose HHT.