结节性硬化症多系统损害的影像学分析

Imaging Analysis of Tuberous Sclerosis Complex with Multisystem Impairment

目的分析总结结节性硬化症伴发多系统损害的CT和MRI表现,提高对该病的认识。方法回顾性分析16例经临床确诊的结节性硬化症合并多系统损害的影像学特征。结果 16例均出现典型的室管膜下结节,其中钙化结节11例,非钙化结节5例;7例出现皮层及皮层下结节,6例可见脑白质信号改变,1例伴发室管膜下巨细胞星形细胞瘤;12例累及肾脏,表现为双肾多发大小不等血管平滑肌脂肪瘤,其中3例同时出现双肾多发囊肿;2例累及肝脏,表现为肝脏多发血管平滑肌脂肪瘤;2例累及肺,表现为肺淋巴管平滑肌瘤病;1例累及骨骼,表现为骨骼多发结节样及斑片状骨质硬化。结论了解结节性硬化症及其多系统损害的影像学表现有助于提高对该病的诊断。

Objective To analyze and summarize the CT and MRI manifestations of tuberous sclerosis complex(TSC)with multisystem impairment,and to improve the diagnostic recognition of the disease.Methods Imaging data of 16 TSC patients with multisystem impairment were analyzed retrospectively.Results Typical subependymal nodules were observed in all the 16 patients,including calcified nodules in 11 and noncalcified nodules in 5.Among the 16 patients,7 had cortical and subcortical tubers,6 had cerebral white matter abnormalities,and 1 had subependymal giant cell astrocytoma.In addition,bilateral multiple renal angiomyolipomas were found in 12 patients(bilateral multiple cysts in 3 patients),multiple hepatic angiomyolipomas in 2 patients,pulmonary lymphangioleiomyomatosis in 2 patients,and skeleton multiple nodules and patchy sclerosis in 1 patient.Conclusion Understanding of the imaging manifestations permits a more specific and accurate diagnosis of TSC with multisystem impairment.