【摘要】 目的总结遗传性出血性毛细血管扩张症的临床和影像学特征。方法回顾性分析14例遗传性出血性毛细血管扩张症患者的临床表现和多排螺旋CT影像学资料。结果 14例患者中单一脏器受累6例,多脏器受累8例。其中肺部受累5例;肝脏受累12例,包括肝动脉-肝静脉分流6例,肝动脉-门静脉分流2例,门静脉-肝静脉分流4例;胰腺受累5例;脾脏受累1例;小肠受累1例。结论多排螺旋CT能够提高遗传性出血性毛细血管扩张症累及多脏器的检出率,特别是最大密度投影对于显示病变血管变异有优势,有助于临床诊断。更多还原

【Abstract】 Objective To study the image and clinical manifestations of hereditary hemorrhagic telangiectasia.Methods Image and clinical data derived from 14 cases of hereditary hemorrhagic telangiectasia were retrospectively studied and summarized. Results Single organ involvement was found in 6 cases while multiple organs involvement was found in the other 8 cases. Pulmonary arteriovenous malformations(PAVMs) were found in 5 patients in the lower lobe.Liver involvement was observed in 12 cases, with vascular abnormalities including arteriovenous shunt(6 cases), arterioportal shunt(2 cases) and portovenous shunt(4 cases). Pancreatic AVMs and telangiectases were found in 5 patients only during arterial phase. Diffuse telangiectases were present in the spleen of one patient. Small intestine involvement was detected in one patient. Conclusion MSCT can improve the positive detetion rate of multiple organs involved by hereditary hemorrhagic telangiectasia. Maximum intensity projection images have advantage in revealing vascular lesions resulted from hereditary hemorrhagic telangiectasia and can be used to characterize the associated vascular shunts.更多还原