家族性结节性硬化病的影像学表现(附5例分析)

目的：探讨结节性硬化症的CT、MRI和超声表现特征。方法：搜集1个家系5例经临床证实的结节性硬化症，均行颅脑CT检查和肾脏超声检查，其中颅脑MRI检查1例，肾脏CT检查2例，回顾性分析其影像学特点。结果：结节性硬化症常累及皮肤、脑、肾脏等器官，主要影像学特征为室管膜下多发性结节，突入脑室生长，结节大于10mm，出现脑积水，即可诊断室管膜下巨细胞星形细胞瘤。本组5例，2例合并肾脏错构瘤，2例合并室管膜下巨细胞星形细胞瘤。结论：CT、MRI和超声检查可清晰显示结节性硬化症的大小、形态和延伸范围，多数能定性诊断，为临床治疗提供指导。

The imaging manifestations of familiar tuberous sclerosis

Objective:To investigate the CT,MRI and ultrasonographic manifestations of tuberous sclerosis.Methods:The CT and MRI findings of 5 cases from one family were proved by retrospectively analysis.Brain CT scans were performed in all cases,MRI in 1 case,and kindney CT scans in 2 cases.Results:Tuberous sclerosis frequently invoved skin,brain and kindney.On imaging,most lesions showed subendymal tuberosis,which diameter>10mm,and the water-head syndrome incur in case,the gigantocellular stellate tumor can definitely diagnosed,in 2 cases of the group kindney hamartoma was found and subendymal gigantocellular stellate tumor in 2 cases.Conclusion:CT ,MRI and ultrasonography not only display the size,shape and extent of tuberous sclerosis,but also determine the nature,which are helpful to diagnosis.