结节性硬化症的特殊影像表现

The special imaging appearances of tuberous sclerosis complex

目的:探讨结节性硬化症的特殊影像表现特征,提高对本病的认识。方法:回顾性分析结节性硬化症35例,特殊影像表现9例,其中视网膜错构瘤3例,脑巨细胞星形细胞瘤3例,肾错构瘤2例,头皮下血管纤维瘤1例。CT平扫5例,平扫加增强3例,MRI平扫加增强扫描1例,手术病理证实5例。结果:视网膜错构瘤表现为视盘及其周围扁平或结节状向球内突出肿块,多伴有钙化;脑巨细胞星形细胞瘤发生在莫氏孔,向侧脑室内突出,常伴有钙化及脑积水;肾错构瘤表现为肾内含脂肪密度的肿块,多向肾轮廓外突出;头皮下血管纤维瘤表现为圆形或卵圆形软组织密度肿块。结论:CT及MRI检查对结节性硬化症的诊断具有重要价值,结合临床特点及脑内特征改变对其特殊表现可做出定性诊断。

Objective:To investigate the special imaging appearances of tuberous sclerosis complex(TSC)and to improve the understanding of the disease.Methods:Thirty-five cases of TSC were reviewed retrospectively.There were9cases with special imaging appearances,the number of retinal angiomyolipoma,subependymal giant cell astrocytoma,renal angiomyolipoma and scalp angiofibroma were3,3,2and1,respectively.Eight cases underwent CT plain scanning while3cases underwent CT contrast scanning,1case underwent MRI plain and contrast scanning.Five cases were proved by pathology.Results:Retinal angiomyolipoma was demonstrated as flattened or tuber mass with calcification in optic disc protruding into the eyeball.Subependymal giant cell astrocytoma originated in the region of Monro’s foramen,projecting in to ventricles,often accompany-ing with calcifications and obstructive hydrocephalus.Renal angiomyolipoma appeared mixed density mass associated fat tissue,mostly projecting outside of renal contour .Scalp angiofibroma demonstrated round and oval soft-tissue density mass.Con clu-sion:CT and MRI examinations have important value in the diagnosis of TSC,the characteristic imaging appearances with clinical features usually can make a specific TSC diagnosis.