遗传性出血性毛细血管扩张症的MDCT影像学表现及临床意义  被引量:4

MDCT Findings of Hereditary Hemorrhagic Telangiectasia and Its Clinical Significance

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作  者:周仕恩[1] 宋文艳 何汇忱[1] 

机构地区:[1]北京民航总医院放射科,100123 [2]北京佑安医院,100679

出  处:《临床放射学杂志》2014年第12期1873-1877,共5页Journal of Clinical Radiology

摘  要: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 MDCT findings of hereditary hemorrhagic telangiectasia involving muhiple organs and to discuss their clinical significance. Methods The imaging materials of 11 patients with clinically-confirmed hereditary hemorrhagic telangiectasia were retrospectively analyzed, and the imaging features were summarized. Results Of the total 11 patients, the lesion involved single organ in 5 and involved multiple organs in 6. In the four cases with pulmonary involvement, the lesions were distributed in the bilateral lower lobes, which were characterized by single or multiple masses or nodular shadows that showed synchronous enhancement with pulmonary artery. Tortuous and thickened tumorfeeding arteries and draining veins could also be seen. In the nine cases the liver was involved. The imaging findings included ( 1 ) hepatic artery-hepatic vein shunt ( n = 4) : tortuous and thickened hepatic artery as well as early visualization of hepatic vein, diffuse or scattered small patchy enhanced foci with blur edge could be observed, and the lesions were isodensity in portal phase ; (2) hepatic artery-portal vein shunt ( n = 1 ) : tortuous and thickened hepatic artery as well as early visualization of dilated portal vein, diffuse or scattered small patchy enhanced loci with blur edge could be observed, and the lesions were iso-density in portal phase; (3) hepatic artery-hepatic vein shunt complicated by hepatic artery-portal vein shunt ( mixed type, n = 3) ; (4) portal vein-hepatic vein shunt ( n = 1 ) : thickened hepatic artery in arterial phase and dilated portal vein in portal phase were seen, small patchy enhanced foci with blur edge was found, and lesions were slight hyper-density in delayed phase. The lesion was located at pancreas in 3 cases : multiple small nodular enhanced foci at pancreatic body and tail were seen in arterial phase ( n = 1 ), which was iso-density in portal phase ; multiple vascular ectasia around the pancreatic head was found in one case;

关 键 词:遗传性出血性毛细血管扩张症 器官受累 体层摄影术 X线计算机 

分 类 号:R543.7[医药卫生—心血管疾病]

 

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