外科治疗重症肌无力的疗效分析

目的评价外科治疗重症肌无力的效果,探讨影响术后肌无力危象发生的因素以及预防治疗要点。方法回顾性分析1985年6月至2005年6月78例接受外科治疗重症肌无力患者的临床资料,对影响术后肌无力危象发生及程度的因素进行分析,比较不同围手术期处理方案的疗效差异。结果肌无力症状完全缓解21例,明显改善38例,改善11例,无变化8例。病程长短、血清抗乙酰胆碱受体抗体水平、Osserman分期和胸腺病理类型均是术后肌无力危象发生的独立相关危险因素。在围手术期处理方面,新方案疗效明显优于旧方案。结论外科治疗重症肌无力具有良好的效果和可行性。     

电视胸腔镜下胸腺切除术治疗重症肌无力

目的探讨电视胸腔镜手术(VATS)下行胸腺切除治疗重症肌无力的可行性和治疗效果。方法19例重症肌无力患者,按照Osserman临床分型标准,Ⅰ型10例,Ⅱa型5例,Ⅱb型3例,Ⅲ型1例,均在VATS下行胸腺扩大切除术。结果19例患者均顺利完成手术,无手术死亡,平均手术时间120min,术中出血量均小于100ml,术后发生重症肌无力危象3例,经及时治疗治愈。术后对所有患者均进行了随访,随访时间>6个月,重症肌无力病情完全缓解9例,好转6例,无变化4例,总有效率79%(15/19)。结论在VATS下行胸腺扩大切除术治疗重症肌无力是可行的,且创伤小、疼痛轻,以期望减少术后并发症。     

胸腺瘤合并重症肌无力患者术后远期疗效及影响因素分析

目的探讨胸腺瘤合并重症肌无力(myasthenia gravis,MG)患者术后的远期疗效及其影响因素。方法回顾性分析2002年6月至2014年12月在我院行胸腺扩大切除术的63例胸腺瘤合并重症肌无力的患者的临床资料及随访结果,其中男26例、女37例,平均年龄(54.51±12.62)岁。运用生存分析模型对性别、手术时年龄、术前病程、伴有其他疾病、危象史、术前激素服用时间、Osserman分型、Masaoka分期、WHO病理分型、手术路径、肿瘤大小等因素对术后疗效的影响进行统计学分析。结果平均随访时间35(5~96)个月。随访期间,12例(19%)患者完全缓解,39例(62%)部分缓解,7例(11%)病情稳定,5例(8%)加重,总有效率为81%。Log-rank分析显示术前病程(P=0.027)、肌无力危象史(P=0.035)和Osserman分型(P=0.018)与术后MG未完全缓解有关,Cox回归分析显示术前病程(P=0.001)、Osserman分型(P=0.012)是术后重症肌无力未完全缓解的独立危险因素。结论胸腺扩大根治术是治疗胸腺瘤合并重症肌无力的有效方式,但术前病程≥12个月和Osserman分型ⅡB、Ⅲ和Ⅳ型的胸腺瘤合并重症肌无力患者术后肌无力症状不易完全缓解。     

胸腺切除术治疗重症肌无力102例分析

目的总结胸腺切除术治疗重症肌无力（myasthenia gravis，MG）的经验。方法回顾1990年1月～2004年12月对102例MG患者行胸腺切除术的临床资料，从性别、年龄、病程、Osserman分型、纵隔脂肪组织清扫程度和病理结果等6个方面统计分析影响疗效的因素。结果102例患者术后发生并发症22例（21．6％），其中危象14例（13．7％），死亡2例（2．0％），1例死于肺部感染，1例放弃治疗。术后平均随访5年，治愈率为29．4％（30／102），总有效率为82．4％（84／102）。Osserman分型、纵隔脂肪组织清扫程度和病理结果对有效率有影响（P〈0．05）。结论扩大胸腺切除术是治疗MG安全、有效的方法，而围手术期处理是综合治疗MG的重要组成部分；Osserman分型、纵隔脂肪组织清扫程度和病理结果是影响手术疗效的重要因素。     

重症肌无力协会临床分型及定量评分的应用体会

目的探讨美国重症肌无力协会(MGFA)临床分型及定量评分(QMG)的临床意义。方法对2001年11月至2003年3月手术治疗的重症肌无力病人36例，分别按Osserman分型与MGFA分型并作比较，以探讨手术治疗前后OMG定量评分的变化，及与临床分型、近期疗效的关系。结果。MGFA临床分型中的Ⅰ型与Osserman Ⅰ型相同，Osserman Ⅱa型与Ⅱb型分别包含不同的MGFA临床类型；治疗后QMG评分随时间呈明显降低；不同疗效病人其QMG有显著差异，与Osserman疗效评分相一致。结论美国重症肌无力协会临床分型较Osserman分型更为细致、客观，其定量评分准确反映出治疗前后病情的变化，值得在临床上推广应用，以加强对重症肌无力的临床研究与协作。     

重症肌无力合并胸腺瘤手术治疗的近期疗效及危象发生的临床分析

目的观察重症肌无力合并胸腺瘤的手术治疗的近期疗效及危象发生的情况，分析疗效和危象的相关因素。方法1994年1月至2005年1月手术治疗重症肌无力（MG）436例，其中58例合并胸腺瘤。肌无力按改良Osserman分型，Ⅰ型17例，Ⅱa型23例，Ⅱb型12例，Ⅲ型6例；胸腺瘤Masaoka分期Ⅰ期30例，Ⅱ期18，Ⅲ期7例，Ⅳ期3例。对术后近期疗效和发生危象发生情况进行统计分析。结果手术后缓解16例（27．59％），无效18例（31．03％），恶化11例（18．97％），危象及死亡13例（22．41％）。Logistic回归分析发现，MG合并胸腺瘤者发生危象的可能性是无胸腺瘤者的1．286倍，MG伴有胸腺瘤者、MGⅡ型及以上者是术后发生危象的高危人群。Ⅰ型与Ⅱ型及以上者术后近期的危象发生率差异有统计学意义（P〈0．05）。Ⅰ期与Ⅱ期及以上胸腺瘤者术后近期的危象发生率差异无统计学意义（P〉0．05）。结论MG合并胸腺瘤的病人术后危象的发生率明显增加，MGⅡ型及以上型术后更容易发生危象，危象的发生与胸腺瘤的病理分期无相关性。     

糖皮质激素联合丙种球蛋白短程冲击治疗预防重症肌无力术后危象

重症肌无力(myasthenia gravis,MG) 是一种以神经肌肉传导障碍为特征的自身免疫性疾病,非手术治疗方法主要包括应用抗胆碱酯酶药、免疫抑制剂、丙种球蛋白及血浆置换,手术切除胸腺后疾病的缓解率可达80%.手术治疗的围术期并发症较多,术后肌无力危象发生率约6%～25%.2001年以来,我们采用糖皮质激素联合丙种球蛋白短程冲击治疗预防全身型MG胸腺切除术后肌无力危象和其他呼吸道并发症,明显减少了围术期并发症的发生,提高了手术治疗的效果,现总结报告如下.     

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。     

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。     

胸腺瘤合并重症肌无力术后发生肌无力危象处理体会

目的探讨胸腺瘤切除术后发生重症肌无力危象的原因及治疗方法。方法回顾分析2000年1月至2007年6月我科收治的43例胸腺瘤合并重症肌无力患者中11例发生术后危象的病例资料。结果11例患者中除l例放弃治疗外,其余均治愈出院。术后危象发生率25.5%(11/43),重症肌无力危象主要发生于术后早期。术前准备不充分,手术的创伤,服用抗胆碱酯酶药物剂量不当,感染(尤其是肺部感染)是围术期发生危象的诱因。结论加强围术期管理,可以改善危象的预后,减少发病率和病死率。     

MGFA分型及QMG评分预测重症肌无力患者术后延迟拔管的价值

目的探讨美国重症肌无力协会基于定量测试的临床分型((MGFA分型)及定量评分(QMG评分)对重症肌无力(MG)患者胸腺切除术后延迟拔管的预测价值。方法以我院2007年1月至2012年2月确诊为MG行胸骨正中切口胸腺切除手术的61例患者为研究对象,根据术后情况分为正常拔管组(47例)和延迟拔管组(14例),比较两组性别、年龄、术前MGFA临床分型、QMG评分、肝肾功能、电解质、术前新斯的明及强的松用量等情况。绘制术前MGFA分型及QMG评分的受试者工作特征(ROC)曲线,计算QMG评分的最佳临界值,同时对MGFA分型及QMG评分预测术后延迟拔管的敏感度与特异度进行比较。结果延迟拔管组(14例)在术毕麻醉苏醒后需呼吸支持或拔管后48h内再次插管,延迟拔管率为22.95%。MGFA分型、QMG评分预测术后延迟拔管的ROC曲线下面积(AUC)分别为0.723、0.866,以QMG评分8.5为阈值,预测延迟拔管的灵敏度为78.6%,特异度为87.2%,而MGFA分型预测的灵敏度为78.5%,特异度为63.8%。结论术前MGFA分型、QMG评分可作为术后延迟拔管的预测指标。     

重症肌无力病人胸腺切除术后危象发生的多因素分析

目的 探讨重症肌无力(MG)病人胸腺切除术后危象发生的危险因素.方法 回顾性分析1995年7月至2009年12月确诊为MG者进行胸腺扩大切除术84例的临床资料.对性别、年龄、术前危象史、Ossermen分型、术前是否使用激素和(或)抗胆碱酯酶药物、手术方式、手术时间、胸腺瘤、病?　 理类型、输血和病程、术后肺部感染进行多因素Logistic回归分析.结果 术后出现MG危象24例(28.6%).危象的发生与术前危象史、Ossermen分型、手术方式、手术时间有关.结论 有术前危象史、OssermenⅡb型及以上、传统手术方式和手术时间长均是易发生术后危象的独立危险因素.

Abstract：

Objective To evaluate the risk factors of myasthenic crisis(MC) after thymectomy for myasthenia gravis (MG) Methods A retrospective study was conducted in 84 patients with MG from July 1995 to December 2009. The following factors were evaluated: sex, age, history of preoperative myasthenic crisis, Osssrmen classification, use steroid hormones and anticholinesterase drugs before operation, operation methods, operation time, thymoma, pathology, transfusion, course of disease, postopertive lung infection,etc. Multivariate logistic regrossion analysis was used to find the predictors of postoperative myasthenic crisis. Results Twenty-four patients(28.6%) developed myasthenic crisis after thymectomy. Statistics showed that history of preoperative myasthenic crisis, Ossermen classification, operation methods, operation time were related to postoperative myasthenic crisis. Conclusion Preoperative myastheric crisis, Ossermen classification≥I b, traditional operation methods, long opertion time are the independent risk factors for myasthenic crisis after thymectomy.     

Prognostic factors for myasthenic crisis after transsternal thymectomy in patients with myasthenia gravis

OBJECTIVE: The purpose of this study was to assess which clinical features of patients with myasthenia gravis predict postoperative respiratory problems due to myasthenic crisis after transsternal thymectomy. METHODS: One hundred twenty-two patients who underwent transsternal thymectomy in our institute were analyzed retrospectively. Fourteen of those experienced myasthenic crisis and required prolonged (48 hours or more) postoperative mechanical ventilation. The following factors were evaluated: sex, age, body mass index, grade of symptom, disease interval, existence of thymoma, history of preoperative crisis, doses of anticholinesterase drugs, steroid use, pulmonary function, serum anti-acetylcholine receptor antibody, history of pulmonary disease, presence of other disease, operation time, and blood loss. RESULTS: Univariate analysis revealed preoperative bulbar symptoms (odds ratio = 14.246, P =.001), history of preoperative myasthenic crisis (7.091,.018), and preoperative serum level of anti-acetylcholine receptor antibody > 100 nmol/L (4.098,.044) were prognostic factors for postoperative myasthenic crisis. On the other hand, multivariate logistic regression analysis revealed preoperative bulbar symptoms (33.333,.004), preoperative serum level of anti-acetylcholine receptor antibody > 100 nmol/L (7.874,.020), and intraoperative blood loss > 1000 mL (18.519,.048) were prognostic factors for postoperative myasthenic crisis. CONCLUSIONS: In this study, postoperative myasthenic crisis after transsternal thymectomy in 122 patients with myasthenia gravis was affected by the existence of preoperative bulbar symptoms, history of preoperative myasthenic crisis, preoperative serum level of anti-acetylcholine receptor antibody > 100 nmol/L, and intraoperative blood loss > 1000 mL. Meticulous preoperative and postoperative care should be carried out to prevent postoperative myasthenic crisis in patients with these prognostic factors.     

Experience in the treatment of acute fulminating myasthenia gravis]

During past 15 years, 188 myasthenia gravis (MG) patients underwent thymomectomy or thymectomy with extended resection of the adipose tissue around the thymus in our institution. Four of 188 patients (2%) had to be given respiratory support within 2 months after the onset of MG. We defined this MG as the acute fulminating type. There were 2 male and 2 female patients, ranging in age from 22 to 44 years (average, 32.5 years). Three of these patients had thymoma. One of these patients was post-thymomectomy myasthenia gravis and another patient was d-penicillamine induced myasthenia gravis. Two patients admitted on respirator for respiratory crisis. All patients underwent operation within 2 weeks after admission. The duration of respiratory support ranged from 10 to 120 days (mean 44) after operation. The period of the hospital stay ranged from 8 months to 2 years 1 month (mean 1 year 5 months). During acute stage after operation, the patients needed ACTH, steroid, immunosuppressants (azathioprine, Bredinin) and plasma pheresis depending on their severity of myasthenic symptoms. In 2 patients dose of steroid could be reduced without deterioration of the symptoms. They are doing well with small dose of steroid. In one patient plasma pheresis was performed 6 years after thymectomy. Various symptoms due to myasthenia gravis and rheumatoid arthritis has recurred 10 years after thymectomy. The titer of acetylcholine receptor antibody of this patient has been continuing in high level.     

Non-small-cell lung cancer associated with non-thymomatous myasthenia gravis

A non-small-cell lung cancer without distant metastases was incidentally found in a 77-year-old man who had suffered from myasthenia gravis (MG) without thymoma. The patient’s condition was stabilized by oral pyridostigmine bromide which he had taken during the past 6 years. He simultaneously underwent thymectomy and left lower lobectomy with regional lymph node dissection. Although postoperative myasthenic crisis occurred, mechanical ventilation and intravenous steroid pulse relieved the patient and the symptoms improved thereafter. Cases of operable lung cancer with non-thymomatous MG have rarely been reported and the appropriate therapeutic strategy for such cases remains to be debated. Their causal association remains to be identified, whereas some studies have implied that immune disorder due to the abnormal thymus might possibly enhance the oncogenesis of extrathymic malignancies. Myasthenic crisis should also be taken into account in postoperative management of MG patients who simultaneously undergo thymectomy and lobectomy for synchronous lung cancer.     

Surgical treatment for myasthenia gravis.

A new surgical technique for thymectomy is presented. Three hundred and seventeen patients with myasthenia gravis and 20 with thymomas who had myasthenic symptoms were operated on. The new surgical approach--a small transverse sternotomy--was used in 257 cases (in 240 patients with myasthenia gravis and 17 with thymomas) and conventional median sternotomy in 80. In myasthenic patients small transverse sternotomy enabled radical thymectomy to be performed with an uneventful postoperative course and very good cosmetic results. There were no hospital deaths among patients with myasthenia gravis after thymectomy. The long term results, assessed after 18-24 months, were good: the total remission rate was 39.5%, and there was a great improvement in 48.5% and an improvement in 9%. After thymectomy about 30% of patients received supplementary treatment with prednisone. A correlation between the duration of symptoms and the result of thymectomy was established: the shorter the duration of myasthenia gravis the better the results. In the small group of 20 patients with thymomas two died in hospital. In 12 patients with encapsulated thymic tumours the long term results were similar to those in patients with myasthenia gravis, whereas in patients with infiltrating thymic tumours the results were unsatisfactory.     

Combined operation for myasthenia gravis and coronary artery disease

A 57-year-old man with ocular myasthenia gravis was admitted to our hospital because of acute respiratory insufficiency associated with myasthenic crisis. He had a history of unstable angina indicated percutaneous coronary artery angioplasty. He was diagnosed with generalized nonthymomatous myasthenia gravis and a triple vessel coronary artery disease. We conducted a simultaneous surgical intervention, including extended thymectomy and coronary artery bypass grafting, using a standard cardiopulmonary bypass via median sternotomy. The patient had already been immunocompromised at surgery for having diabetes, and postoperative long-term steroid therapy. In this rare and special condition, a meticulous overall therapeutic strategy was needed in order to avoid myasthenic crisis and prepare for the worst case scenario of mediastinitis.