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目的探讨遗传性出血性毛细血管扩张累及多脏器的MDCT表现及临床意义。方法回顾分析11例经临床明确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特征。结果 11例患者单脏器受累5例,多脏器受累6例。肺脏4例,分布于双肺下叶,表现为与肺动脉同步强化的单发或多发团块状或结节状阴影,并可见迂曲增粗的供血动脉及引流静脉。肝脏9例,包括:(1)肝动脉-肝静脉分流4例:动脉期见增粗迂曲的肝动脉及提前显影的肝静脉;肝实质弥漫性或散在性分布小斑片状强化灶,边缘模糊,门静脉期呈等密度;(2)肝动脉-门静脉分流1例:动脉期见扩张迂曲的肝动脉及提前显影的扩张门静脉;肝实质少量小斑片状强化灶,边缘模糊,门静脉期呈等密度;(3)肝动脉-肝静脉分流合并肝动脉-门静脉分流(混合型)3例;(4)门静脉-肝静脉分流1例:动脉期未见扩张肝动脉,门静脉期见扩张门静脉及其远端分支旁小斑片状强化灶,边缘模糊,延迟期呈稍高密度。胰腺3例:动脉期胰体及胰尾部多发小结节强化灶1例,门静脉期呈等密度;胰头周围多发血管瘤样扩张1例;1例呈混合型表现。脾脏1例,动脉期、门静脉期均可见脾实质弥漫性斑片状、片状强化灶,延迟期呈等密度。胃肠道1例,表现为结肠脾曲及左下腹小肠壁外侧多发异常扩张毛细血管网。结论遗传性出血性毛细血管扩张症累及多脏器的影像表现具有特征性,充分认识其影像表现并结合临床对其诊断及鉴别诊断具有重要意义。
Objective To investigate the clinical manifestations and clinical significance of hereditary hemorrhagic telangiectasia involving multiple organs in MDCT. Methods Retrospective analysis of 11 cases of clinically diagnosed hereditary hemorrhagic telangiectasia imaging data and summarize the image features. Results Eleven patients had single organ involvement in 5 cases and multiple organ involvement in 6 cases. 4 cases of lungs, located in the lower lobe of the lungs, the performance of simultaneous enhancement of the pulmonary artery with multiple or massive lumps or nodular shadows, and see tortuous thickened donor artery and drainage veins. The liver in 9 cases, including: (1) hepatic artery - hepatic venous shunt in 4 cases: the arteries see the thickening tortuous hepatic artery and hepatic vein in advance of the development; diffuse or diffuse distribution of liver parenchyma patchy lesions, the edge (2) hepatic artery - portal vein shunt in 1 case: arterial phase see the expansion of tortuous hepatic artery and dilated portal vein in advance; a small amount of liver parenchyma lesser patches, edge fuzzy, portal vein was (3) hepatic artery - hepatic shunt with hepatic artery - portal vein shunt (mixed type) in 3 cases; (4) portal vein - hepatic vein shunt in 1 case: no dilatation of hepatic artery in arterial phase, portal vein in dilated portal vein Side of the small branches of the lesion near the edge of enhanced focus, fuzzy edge, the delay was slightly higher density. 3 cases of pancreas: multiple nodules in the pancreas and tail of the pancreas in the arterial phase showed enhanced nodules in 1 case. The portal vein phase showed isodense density. One case had multiple hemangioma-like dilatation around the pancreas. One case showed mixed appearance. Spleen in 1 case, arterial phase, portal vein can be seen diffuse patchy splenomegaly, sheet-like focal enhancement, delay was equal density. One case of gastrointestinal tract, the performance of the spleen and left colon of the left lower quadrant of the wall of multiple abnormal expansion of capillary network. Conclusion The hereditary hemorrhagic telangiectasia is characterized by multiple organ manifestations. It is of great importance to fully understand the imaging manifestations and combine with clinical diagnosis and differential diagnosis.