肝脏遗传性出血性毛细血管扩张症影像学表现

The imaging findings of hepatic hereditary hemorrhagic telangiectasia

目的:总结分析肝脏遗传性出血性毛细血管扩张症(HHT)的影像特征。方法:回顾分析11例经临床诊断的遗传性出血性毛细血管扩张症的影像学资料并总结其影像学特征。结果:11例病例均行CT平扫及多期增强扫描,其中2例做MR检查。11例病例中病灶CT平扫及MR T1WI图像表现正常,T2WI图像上表现为斑片云雾状略高信号灶。多期增强扫描图像上,11例病例或出现动脉期肝实质雪花样或棉絮状异常灌注,或出现肝脏大血管畸形,或两者同时存在;其中大血管畸形包括肝动脉畸形3例、肝动脉-肝静脉瘘3例、肝动脉-门静脉瘘2例,肝门脉-肝静脉瘘1例。4例病例同时肺部CT检查发现肺内动静脉畸形(AVM),其中2例单发,2例多发。结论:肝脏遗传性出血性毛细血管扩张症影像上表现为动脉期肝实质雪花样或棉絮状异常灌注及血管畸形,影像表现具有一定的特征性。

Objective:To summarize and analyze the imaging findings of hepatic hereditary hemorrhagic telangiectasia.Methods:The imaging information of 11 patients clinically-confirmed as hereditary hemorrhagic telangiectasia were retrospectively analyzed.The imaging findings were summarized.Results:11 patients all received CT plain scan and multi-phase enhanced scan,and 2 patients also received MR examination at the same time.11 cases showed normal in the CT plain images and the MR T1WI,while the lesions were patch cloudy and presented as hyper-signal on T2WI.In the multi-phase enhanced scan images,11 cases had the snow-pattern or cotton-like abnormal perfusion of hepatic parenchyma in arterial phase,or hepatic macrovascular malformation,or both.Vascular malformations included 3 cases of hepatic artery malformation,3 cases of arteriovenous shunting,2 cases of arterioportal shunting and 1 case of portovenous shunting.Meanwhile,arteriovenous malformation(AVM)lesions were found in the CT scan of the lungs of 4 patients.Among them,there were 2 cases with single lesion and 2 cases with multiple lesions.Conclusions:The snow-pattern or cotton-like abnormal perfusion of hepatic parenchyma in arterial phase and the hepatic macrovascular malformation are the characteristic imaging features of hepatic hereditary hemorrhagic telangiectasia.