【摘要】 目的探讨多系统损害结节性硬化症(TSC)的CT影像学特点及临床表现,分析两者结合在TSC诊断中的应用价值。方法回顾性分析经临床或病理确诊的11例TSC患者的CT平扫表现及临床特点。结果 11例患者中仅1例同时具有TSC典型三联征(癫痫、智力低下及面部皮脂腺瘤),其余10例仅有1种或2种。所有患者均进行了头部或胸腹部CT平扫,11例患者均有室管膜下结节,1例有室管膜下巨细胞星形细胞瘤,4例有肺部非钙化性小结节,8例有肾脏血管平滑肌脂肪瘤,4例有肝脏血管平滑肌脂肪瘤,5例有骨骼受累。结论结合TSC的特征性临床表现及CT平扫征象,可对TSC的确诊提供重要的诊断依据,特别是以癫痫为首发症状且头颅CT平扫有室管膜下结节的患者,更加高度预示了本病的发生。更多还原

【Abstract】 Objective To make an exploration of the plain CT scanning features and clinical features of multiorgan impairment with tuberous sclerosis complex(TSC) to investigate the combination of both features in diagnosis of TSC. Methods The plain CT scan imaging findings and the clinical features of the 11 TSC patients who had been diagnosed pathologically or clinically were reviewed. Results Of 11 patients with TSC, one typical triad(epilepsy, mental retardation and facial sebaceous adenoma) was found and the remaining ten cases had only 1 or 2 symptoms separately. All the eleven patients underwent head or chest orabdominal multi-slice spiral plain CT scan. As a result, sub-ependymal nodules were detected in every case, sub-ependymal giant cell astrocytoma was detected in 1 case, and non-calcified pulmonary nodules were revealed in 4 cases. Eight patients had renal angiomyolipomas, while four had liver angiomyolipomas. Bone involvement was discovered in 5 cases. Conclusion The combination of plain CT scan imaging findings and clinical features of TSC offered sufficient basis for the diagnosis of multi-organ impairment of TSC, especially for the epilepsy patient from whom the subependymal nodules were revealed by using plain CT scan as a useful indication of the diagnosis of TSC.更多还原