结节性硬化症的颅脑及肾脏影像学表现与治疗

目的探讨结节性硬化症伴多脏器损害的影像学特点。方法回顾性分析19例结节性硬化症伴皮肤、大脑、肾脏、肝脏等多器官损害的病例资料、临床表现及影像学改变。结果 CT显示室管膜下多发胶质结节及皮层灰质结节呈等密度,钙化结节为高密度,室管膜下病灶多突入侧脑室内,伴有脑积水和肿瘤。MRI病变T1WI呈等或低信号,T2WI呈高信号,肿瘤的信号不均一。结论影像学检查室管膜下结节、皮质结节、内脏多发性错构瘤为特征,治疗方案应根据双肾肿瘤的大小、分布、发展情况及症状决定,手术时应注意保留正常肾组织,保护肾功能。

Imaging mannifestations and treatment of tuberous sclerosis in brain and Kidney

Objective Of tuberous sclerosis with multiple organ damage Imaging Features Methods Retrospective analysis 19 cases of tuberous sclerosis with skin,brain,kidney,liver damage,and other organs of the clinical data、clinical manifestation and image studies change.Results CT showed multiple subependymal nodules and cortical gray matter glial nodules were isodense,the calcification nodules were high density,Subependymal lesions were broke into the lateral ventricle,and tumors associated with hydrocephalus.MRI showed lesions,etc.,or low signal T1WI,T2W2 showed high signal,tumor signal heterogeneity.Conclusion Imaging subependymal nodules、cortical nodules、multiple hamartoma visceral were characterized,Kidney cancer treatment should be based on the size,distribution,development and symptoms of the decision,Surgery should pay attention to retain normal renal tissue,protection of renal function.