6例结节性硬化症脑外病变的CT表现

CT manifestations of the extracerebral lesions of tuberous sclerosis complex

目的分析结节性硬化症脑外病变的影像学表现,提高对结节性硬化症脑外病变影像学表现认识。方法收集6例符合临床诊断标准的结节性硬化症患者脑外病变的影像学资料,其中腹部CT扫描6例,胸部CT扫描1例。并复习相关文献。结果 6例结节性硬化患者中,伴双肾多发错构瘤者6例,其中2例发生自发性出血;伴骨骼多发硬化结节者6例;伴肝脏错构瘤者3例;伴肺部病变者1例。结论结节性硬化症脑外病变影像学表现多种多样,以双侧肾脏错构瘤伴骨骼多发硬化结节具有特征性。

Objective To analyse the imaging features of extracerebral lesions of tuberous sclerosis complex and improve the cognition of their imaging manifestations. Methods Extracerebral imaging findings（ 6 cases in upper abdomen and 1case in chest） of 6 patients with clinically proved tuberous sclerosis complex were collected. And the relevant literatures were reviewed. Results All cases had multiple renal hamartomas,in which 2 cases with spontaneous bleeding,and multiple sclerotic nodules of bones. However,among the 6 patients,3 cases had multiple hepatic hamartomas and 1 case had pulmonary lymphangioleiomyomatosis. Conclusion The imaging manifestations of the extracerebral lesions of tuberous sclerosis complex were varied,and multiple renal hamartomas accompany with multiple sclerotic nodules in bones was distinctive.