伴多器官受累的结节性硬化症影像学诊断

doi:10.12117/jccmi.2019.10.013

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摘要目的：探讨结节性硬化症（Tuberous sclerosis complex，TSC）伴多器官受累的影像学特点，提高对TSC影像学表现的认识。方法：回顾性分析10例经临床诊断的TSC患者的临床资料和影像学表现。结果：9例TSC伴有肺淋巴管平滑肌瘤病，CT表现为两肺弥漫大小不等薄壁囊腔。9例伴有多发骨质硬化改变，CT表现为多发结节状、斑片状骨质致密影。6例伴有肾脏血管平滑肌脂肪瘤，其中3例合并肝脏血管平滑肌脂肪瘤，1例合并多发肾囊肿。5例伴有特征性室管膜下结节，4例伴有脑白质异常信号改变，4例伴有皮质、皮质下结节。结论：TSC伴多器官受累具有一定的影像学特征，熟悉各系统病变的影像学表现，有助于提高TSC的诊断。

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	胡剑锋
	王宇泽
	李新春
	包盈莹
	雷强
	万齐
	邹乔

关键词 ：结节性硬化症, 体层摄影术, 螺旋计算机, 磁共振成像

Abstract： Objective: To investigate the imaging features of tuberous sclerosis complex(TSC) with multiple organ involvement which may help improve the diagnostic efficacy. Methods: Clinical data and imaging findings of 10 patients with TSC confirmed by clinic were restrospectively analyzed. Results: Pulmonary lymphangioleiomyomatosis was found in 9 cases which were characterized by diffuse thin-wall cysts of different sizes in both lungs. Nine cases had multiple sclerosis in bones which was characterized by multiple nodular or patchy high density shadows. Renal angiomyolipomas were found in 6 cases, among which hepatic angiomyolipomas were found in 3 cases and bilateral multiple renal cysts were found in 1 case. Five cases were accompanied with typical subependymal nodules, 4 cases with abnormal signal intensity in white matter, and 4 cases with cortical and subcortical tubers. Conclusion: TSC with multiple organ involvement demonstrates certain imaging features, which may help the diagnosis of TSC.

Key words： Tuberous sclerosis Tomography, spiral computed Magnetic resonance imaging

收稿日期: 2018-11-06

PACS:	Ｒ744.51
	Ｒ814.42
	Ｒ445.2

通讯作者: 李新春，广州医科大学附属第一医院放射科，510000。E-mail：xinchunli@163.com

作者简介: 胡剑锋（1995-），男，广东梅州人，在读硕士研究生。E-mail：544173068@qq.com

引用本文:

胡剑锋，王宇泽，李新春，包盈莹，雷强，万齐，邹乔. 伴多器官受累的结节性硬化症影像学诊断[J]. 中国临床医学影像杂志, 2019, 30(10): 738-741.
HU Jian-feng, WANG Yu-ze, LI Xin-chun, BAO Ying-ying, LEI Qiang, WAN Qi, ZOU Qiao. Imaging features of tuberous sclerosis with multiple organ involvement. JOURNAL OF CHINA MEDICAL IMAGING, 2019, 30(10): 738-741.

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http://www.jccmi.com.cn/CN/10.12117/jccmi.2019.10.013 或 http://www.jccmi.com.cn/CN/Y2019/V30/I10/738

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