多系统病变的结节性硬化症MSCT表现分析

Analysis of MSCT Tuberous Sclerosis Complex Complicated with Multiple Systemiclesions

目的使用多层螺旋CT(MSCT)检查方法,探究结节性硬化症的多系统受累情况,以进一步提高对结节性硬化症合并皮肤、中枢神经、呼吸、消化及骨骼系统病变的MSCT表现的全面认识。方法对2013年4月至2020年12月在我院与成都大学附属医院经临床诊断为结节性硬化症伴多脏器病变的22例患者的MSCT表现进行回顾性分析,评价MSCT检查在合并多脏器病变的结节性硬化症临床诊治中的价值。结果室管膜下多发较对称的小结节以钙化为主22例;伴1例室管膜下巨细胞星形细胞瘤。合并肺部病变21例,主要为肺淋巴管平滑肌瘤病(LAM)和1例多灶性微小结节样肺细胞增生(MMPH),LAM表现为双肺随机分布大小不等的薄壁、清晰的含气囊腔,MMPH表现为双肺弥漫分布的边界清晰的小结节状密度增高影。合并双肾多发错构瘤21例,其中巨大肾脏血管平滑肌脂肪瘤(AML)4例,2例合并破裂出血,出血主要与病变大小密切相关。伴发肝脏AML 5例,其特征性改变为圆形或类圆形含脂肪密度肿块影。合并骨骼受累有8例,以脊柱多见,且以胸腰椎为主,表现为多个椎体及附件多发斑片状或结节状高密度致密影,其特征性改变为牙质样以椎弓硬化为著。1例伴面部皮肤受累,表现为皮脂腺瘤。结论MSCT在合并多脏器病变的结节性硬化症的诊断中发挥重要作用,其中合并中枢系统病变具有较明显的特征性,其他部位改变也具有一定的影像学特征,可结合其临床特点,作为诊断的重要依据。

Objective To explore the multisystem involvement of tuberous sclerosis by MSCT,so as to enhance comprehensive understanding of MSCT presentation in tuberous sclerosis complex complicated with lesions in the skin,central nervous system,respiratory,digestive and bone systems.Methods From April 2013 to December 2020,MSCT presentations of 22 patients who clinically diagnosed as tuberous sclerosis complex with multiple systemic lesions were retrospectively analyzed to evaluate MSCT examination in clinical diagnosis and treatment of tuberous sclerosis complex in multiple systemic lesions.Results There were 22 cases of multiple small symmetrical nodules,mainly characterized by calcification,in the subependyma zone.Among them,1 case was accompanied with subependymal giant cell astrocytoma.21 cases were complicated with pulmonary lesions,mainly characterized by pulmonary lymphangiomyomatosis(LAM),and 1 case was with multifocal micronodular pneumocyte hyperplasia(MMPH).LAM presented as randomly distributed thin walls of varying sizes and clear airsac-containing cavities in both lungs,and MMPH presented as diffusely distributed well-defined small nodular density enhancement shadow.21 cases were complicated with multiple hamartomas in both kidneys,including 4 cases of giant renal angiomyolipoma(AML),2 cases complicated with rupture hemorrhage which was mainly closely associated with lesion size.5 cases were AML of liver,with characteristic change of circular or quasi-circular lipid-containing density mass shadow.8 cases were complicated with bone involvement,mainly on spine(particularly thoracolumbar spine),which presented as multiple patches or nodules with high-density compact shadow on multiple vertebral bodies and adnexa,whose characteristic change being dentition characterized by vertebral arch sclerosis.1 case was accompanied with facial skin involvement,which presented as steatadenoma.Conclusion MSCT could play an important role to diagnose tuberous sclerosis complex complicated with multiple organ lesions.Complicated central system lesions would have obvious characteristics,and changes in other parts also have certain imaging characteristics,which couuld be combined with their clinical characteristics as an important basis for diagnosis.