胸腺扩大切除术治疗重症肌无力527例

目的总结胸腺扩大切除术治疗重症肌无力(myasthenia gravis,MG)的外科治疗经验,探讨MG外科治疗效果。方法回顾性分析我院1996年6月至2017年10月期间527例行胸腺扩大切除术的MG患者临床病例资料,其中男242例,女285例;年龄5~77岁,平均(52.6±13.7)岁。病程12天~18年。合并甲状腺功能亢进症22例,纯红细胞再生障碍性贫血7例,甲状腺机能减退症、肠激惹症、风湿性关节炎、强直性脊柱炎和血小板减少综合征各1例。改良OssermanⅠ型272例,Ⅱa型72例,Ⅱb型78例,Ⅲ型81例,Ⅳ型24例。所有患者肌疲劳试验、新斯的明试验阳性,胸部CT检查明确诊断。总结围手术期相关资料及术后随访情况。结果围手术期死亡3例,均为胸腺瘤合并MG,其中OssermanⅢ型MG 2例、Ⅳ型MG 1例。术后肌无力危象15例,其中OssermanⅡb型2例、Ⅲ型11例、Ⅳ型2例,气管切开7例。血浆置换70例,并发低渗综合征、下肢静脉血栓各2例。术后病理诊断胸腺增生293例(55.60%)、胸腺瘤207例(39.28%)、胸腺囊肿24例(4.55%)和胸腺萎缩3例(0.57%)。随访378例,平均随访(85.9±58.5)月;MG完全缓解、部分缓解、无变化和恶化患者分别为135例(35.71%)、192例(50.79%)、41例(10.85%)和10例(2.65%)。完全缓解率OssermanⅠ>Ⅱa>Ⅳ>Ⅱb>Ⅲ型,恶化率OssermanⅢ>Ⅳ>Ⅰ型。OssermanⅠ型外科治疗无效18例,术前病程>5~10年;恶化1例,为应用电视胸腔镜胸腺切除的眼肌型MG患者,遗留胸腺左叶未切除,6年后发展为重度全身型,再次手术切除遗留的左叶胸腺,证实左叶胸腺代偿性肥大增生。恶化患者中死亡6例,均为胸腺瘤合并MG,其中OssermanⅢ型5例、Ⅳ型1例;死亡原因为肌无力危象3例,快速停服溴吡斯的明3个月后突发呼吸骤停猝死2例,胆碱能危象1例。结论规范的胸腺扩大切除是治疗MG的有效方法,眼肌型MG尽早手术可有效降低其全身性转化风险。OssermanⅡb型以上MG易发生肌无力危象,围手术期采取综合处理措施有助于降低MG相关风险。重症术后远期可反复发生肌无力危象,须规律用药,并采取MG综合治疗措施。

Analysis of extended thymectomy for the treatment of myasthenia gravis of 527 cases

Objective To summarize the surgical treatment experience of extended thymectomy for myasthenia gravis(MG),and to explore the surgical treatment of MG.Methods Retrospectively analyzed the clinical data of 527 MG patients undergoing extended thymectomy in our hospital from June 1996 to October 2017,including 242 males and 285 females,aged 5 to 77 years,with a mean age of(52.6±13.7)years old.The course of illness was 12 days to 18 years.There were 22 cases of hyperthyroidism,7 cases of pure red blood cell aplastic anemia,1 case each of hypothyroidism,irritable bowel disease,rheumatoid arthritis,ankylosing spondylitis and thrombocytopenia syndrome.There were 272 cases of MG in OssermanⅠ,72 cases inⅡa,78 cases inⅡb,81 cases inⅢ,and 24 cases inⅣ,respectively.The muscle fatigue test and neostigmine test of all patients were positive,and the diagnosis was confirmed by chest CT examination.Meanwhile,summarize the perioperative data and postoperative follow-up.Results 3 cases died during the postoperative period,all of which were thymoma with MG,including 2 cases of OssermanⅢMG and 1 case ofⅣMG;15 cases of postoperative myasthenia crisis,including 2 OssermanⅡb cases,11 OssermanⅢcases and 2 OssermanⅣcases,also including 7 cases of tracheotomy;70 cases of plasma exchange,of whom 2 cases of hypotonic syndrome and 2 cases of lower extremity venous thrombosis.The postoperative pathological types were followed by thymic hyperplasia 293 cases(55.60%),thymoma 207 cases(39.28%),thymic cyst 24 cases(4.55%)and thymic atrophy 3 cases(0.57%)in descending order.378 cases were followed up with an average follow-up of(85.9±58.5)months;MG with complete remission,partial remission,no change and deterioration accounted for 135(35.71%),192(50.79%),41(10.85%)and 10(2.65%)cases,respectively.Complete remission rate ranked as Osserman typeⅠ>Ⅱa>Ⅳ>Ⅱb>Ⅲ,the deterioration rate from high to low was Osserman typeⅢ>Ⅳ>Ⅰ.18 OssermanⅠcases showed no effects,whose preoperative course of disease>5-10 years;1 OssermanⅠcase was worsening who initially was diagnosed with ophthalmic MG and underwent video-assisted thoracoscopic thymectomy.The left thymus lobe was left unresected and developed 6 years later.Another operation was performed to remove the left lobe thymus,confirming the compensatory hypertrophy of the left lobe thymus.Among the worsening patients,6 died,all of them were thymoma with MG,including 5 cases of typeⅢand one case of typeⅣ.The cause of death was omyasthenia crisis(3 cases),sudden respiratory arrest after 3 months of rapid stopping of brompistigmine(2 cases)and cholinergic crisis(1 case).Conclusion Standardized extended thymectomy is an effective method for the treatment of MG.Earlier surgery for ocular muscle type MG can effectively reduce the risk of generalization.MG with OssermanⅡb or higher is prone to myasthenia crisis.Comprehensive treatments should be taken to reduce MG-related risks.Myasthenia crisis can occur repeatedly in severe patients in the long term after surgery,requiring regular medication and comprehensive MG treatments.