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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。
Objective To summarize the clinical and imaging features of hereditary hemorrhagic telangiectasia. Methods Retrospective analysis of 14 patients with hereditary hemorrhagic telangiectasia clinical manifestations and multi-slice spiral CT imaging data. Results 14 cases of single organ involvement in 6 cases, multiple organ involvement in 8 cases. Including lung involvement in 5 cases; liver involvement in 12 cases, including hepatic artery - hepatic vein shunt in 6 cases, hepatic artery - portal vein shunt in 2 cases, portal vein - hepatic vein shunt in 4 cases; pancreatic involvement in 5 cases; spleen involvement in 1 case; 1 case. Conclusion MSCT can improve the detection rate of hereditary hemorrhagic telangiectasia involving multiple organs, especially the maximum density projection has the advantage of showing the vascular variation of the lesion, which is helpful for the clinical diagnosis.