结节性硬化症多系统受累患者临床与 CT 表现分析

Clinical and CT analysis of multiple system involvement in patients with multiple sclerosis

目的探讨结节性硬化症（ TSC）伴多系统受累患者的临床和CT表现特点。方法回顾性分析经临床确诊的10例TSC伴多系统受累患者的CT资料，观察病变的部位、大小、形状、分布等情况。结果纤维血管瘤和纤维瘤8例：面、腰、背部皮肤见直径0.3～1.2 cm多发粉红色及淡褐色透亮的蜡状皮疹；其中见甲周或甲下直径0.5～3.0 cm的纤维瘤3例。 TSC 10例：CT示两侧脑室室管膜下多发钙化结节，直径0.3～1.5 cm。肾血管平滑肌脂肪瘤10例：CT示两肾多发类圆形混杂密度灶，直径0.3～20.0 cm。肺淋巴管肌瘤病8例：CT示两肺散在薄壁囊腔影，直径0.5～2.0 cm。骨骼结节性硬化7例：CT示胸、腰、骶椎、肋骨、顶骨及下颌骨0.3～2.3 cm结节状致密影。肝血管平滑肌脂肪瘤3例：CT示肝内低密度团块影，密度不均，直径0.5～7.6 cm。心脏血管平滑肌脂肪瘤1例：CT示右房室壁条片状低密度灶，大小2.5 cm ×1.2 cm。结论 TSC伴多系统疾病患者CT表现具有特征性，CT表现结合临床可确定诊断。

Objective To investigate the clinical and CT features of the tuberous sclerosis complex （TSC） with multiple organ impairment.Methods Clinical presentation and CT imaging data of 10 clinically confirmed TSC patients with multiple organ impairment were retrospectively analyzed.The position, size, shape and distribution of lesions of these patients were observed .Results There were fibrous hemangioma and fibroma in skin in 8 of the 10 patients, with multiple pink and light brown translucent waxy skin rash of 0.3 -1.2 cm in diameter on the face , waist, back skin, scattered symmetrically in distribution , 3 of whom with fibroma in periungual fibromas underneath and around the nails of 0.5-3.0 cm in diameter.The sub-ependymal nodular sclerosis was found in 10 patients , and CT showed multiple calcification nodules of 0.3 -1.5 cm in diameter on both sides of the ventricles.The renal angiomyolipoma was found in 10 patients, and CT showed multiple circular hybrid density lesions of 0.3-20.0 cm in diameter in bilateral kidneys.There was pulmonary lymphangiomyomatosis in 8 patients, with CT showing scattered cystic lucency shadow in double lung , with thin walled, about 0.5-2.0 cm in diameter.The tuberous sclerosis was found in bones in 7 patients, and CT scan showed nodular opacities of 0.3-2.3 cm in diameter in thoracic , lumbar and sacral vertebrae , ribs, the parietal bone and the mandible , with clear boundary and homogeneous density involving symmetrical and irregular density in radix of the posterior arch of the vertebral body.The hepatic angiomyolipoma was found in 3 patients, and CT showed low density mass of 0.5-7.6 cm in diameter in liver , with uneven density.The heart angiomyolipoma was found in 1 patient , and CT showed streaked low density lesions with 2.5 cm ×1.2 cm in size and clear boundary in the right atrium and the right ventricular wall .Conclusions The CT performance of TSC with multiple system diseases has some certain specificity .The disease can be definitely diagnosed based on CT manifestations and clinical symptoms .