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.     

重症肌无力的围术期护理体会

目的探讨胸腺切除术治疗重症肌无力患者的围术期护理。方法回顾性分析和总结12例重症肌无力患者进行扩大胸腺切除术的围术期护理特点。结果12例病人无手术及住院死亡。8例病人肌无力症状缓解,3例症状较术前改善,1例术后肌无力症状无缓解,术后2年死亡。结论术前控制呼吸道感染改善肺功能,术后加强呼吸道护理,合理有效地使用抗胆碱酯酶药物,正确预防、判断及处理肌无力危象、胆碱能危象是护理关键,也是减少并发症、提高手术成功率的保证。     

胸腺瘤合并重症肌无力的外科治疗

目的报告1988年～2000年12月手术治疗36例胸腺瘤合并重症肌无力(MG)的结果.方法全部患者均行手术治疗,按Masaoka临床分期Ⅰ期6例,Ⅱ期15例,Ⅲ期12例,Ⅳ期3例.结果术后18例出现重症肌无力危象,均行气管切开及辅助呼吸,1例死于危象.术后随访半年～10年,3年内MG缓解率为72.2%,5年生存率为65.4%,10年生存率为24.8%.结论胸腺切除手术是治疗胸腺瘤合并重症肌无力的主要方法,术后肌无力危象发生率高,及时行气管切开及辅助呼吸是处理的关键.     

胸腺瘤合并重症肌无力患者的手术及围术期处理

目的 为提高胸腺瘤合并重症肌无力（MG）患者手术的安全性和治愈率，总结其临床经验。方法 1991年7月至2005年8月收治胸腺瘤合并MG23例，术前均给予肾上腺糖皮质激素和抗胆碱酯酶药物治疗，病情稳定后行胸腺扩大切除术，对发生MG危象患者予以气管切开或气管内插管，必要时使用呼吸机辅助呼吸。结果 全组无手术死亡，术后2例发生MG危象，经处理后痊愈。随访20例，失访3例，随访时间3个月～10年，缓解3例，明显改善11例，改善5例，无变化1例。其中1例胸腺瘤部分切除患者术后4个月死于肿瘤复发。结论 胸腺瘤合并MG患者除临床证实肿瘤无法切除或已胸外转移者外，其余均应手术治疗，完全切除胸腺瘤并清除前纵隔脂肪组织；正确的围术期处理是降低手术并发症及死亡率的关键。     

胸腺癌并重症肌无力危象的围手术期治疗

重症肌无力（MG）危象是胸腺瘤病人术后最严重的并发症之一,死亡率高,及时诊断、处理是抢救成功的关键。我们自1985年6月至1998年12月共手术治疗46例胸腺瘤合并MG的病人,15例术后发生MG危象,均抢救成菌,无围手术期死亡,现将抢救体会总结如下。     

重症肌无力病人胸腺切除围术期的处理

1978年1月到1994年10月，采用胸腺切除术治疗重症无力23例，1985年以前，围手术期处理不当，7例病人术后发生肌无力危象5例，胆碱能危象3例，3例因危象死亡，1985年以后，加强改进围手术期处理，16例病人术后发生肌无力危象3例，胆碱能危象1例，无手术死亡，本文对术前准备，术后处理要点，以及气管切开，呼吸机治疗在防治术后肌无力，胆碱能危象中的作用，进行了讨论。     

24例胸腺瘤伴重症肌无力围术期治疗体会

目的研究胸腺瘤伴重症肌无力患者的围术期治疗。方法对我科自1990年至2004年收治的24例胸腺瘤伴重症肌无力病人围术期治疗进行回顾性研究。结果本组中3例患者术后出现重症肌无力危象,治疗后痊愈;其余患者术后均顺利康复;全组无围术期死亡病例。结论降低手术并发症及死亡率的关键;合理使用抗胆碱酯酶药物和激素;尽早拔除气管插管,选用有效抗生素防治肺部感染;必要时气管切开、呼吸机辅助呼吸。     

重症肌无力外科治疗的围手术期处理

目的探讨重症肌无力（MG）外科治疗的围手术期处理方法及疗效。方法158例患者，男65例，女93例，年龄3—61岁，OssemanⅠ型93例，Ⅱa型38例，Ⅱb型17例，Ⅲ型10例。术前正确处理合并症，使用药物控制肌无力症状，术中完整切除胸腺并清扫前纵隔脂肪；术后联合使用抗胆碱酯酶药、激素及免疫抑制剂，应用呼吸机进行人工辅助呼吸，防治重症肌无力危象。结果全组患者均无手术或住院死亡，Ⅰ型MG患者术后早期症状缓解26例，明显改善19例，改善27例；Ⅱ、Ⅲ型MG患者术后早期症状缓解33例，明显改善21例；总有效率为76．6％。结论重症肌无力患者经充分的围手术期处理，可安全接受手术治疗，手术疗效好。     

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。     

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。     

Autoimmune myasthenia gravis: Recommendations for treatment and immunologic modulation

Opinion statement Treatment for myasthenia gravis should be individualized to each patient based on the clinical characteristics of myasthenia including the distribution, duration, and severity of weakness and resulting functional impairment; the risks for treatment complications related to age, gender, and medical comorbidities; and the presence of thymoma. Acetylcholinesterase inhibitors provide temporary, symptomatic treatment for all forms of myasthenia gravis. Immune modulators address the underlying autoimmune process in myasthenia gravis, but are associated with potential complications and side effects. Most patients with generalized myasthenia who have significant weakness beyond the ocular muscles and who remain symptomatic, despite treatment with cholinesterase inhibitors, are candidates for immune modulation. Although corticosteroids are effective for long-term immune modulation in myasthenia gravis, several more contemporary immunomodulators including azathioprine, cyclosporine, and mycophenolate mofetil have shown efficacy in myasthenia gravis and are used increasingly as first-line treatments and as steroid-sparing agents. Plasma exchange is used to achieve rapid improvement in patients with myasthenic crisis or exacerbation, to improve strength before a surgical procedure or thymectomy, and to minimize steroid-induced exacerbation in patients with oropharyngeal or respiratory muscle weakness. Intravenous immunoglobulin represents an alternative to plasma exchange in patients requiring relatively rapid short-term improvement in the setting of poor venous access. Because of a lack of controlled trials, the role of thymectomy in nonthymomatous myasthenia gravis is unclear, although evidence suggests that thymectomy increases the probability for myasthenic remission or improvement.     

Experience with programmed steroid treatment with thymectomy in nonthymomatous myasthenia gravis

BACKGROUND: The benefit of thymectomy in myasthenia gravis management is recognized but the perioperative course can fluctuate. The goal of this study was to assess the feasibility and clinical benefit of dose-escalated steroid therapy with thymectomy for nonthymomatous myasthenia gravis. METHODS: We reviewed the records of 69 myasthenia gravis patients who were followed up after undergoing transsternal thymectomy with extended anterior mediastinal dissection in our hospital between 1976-2000. Forty-eight patients in the programmed treatment group who had dose-escalated and de-escalated steroid therapy during the perioperative period comprised 17 patients with ocular myasthenia gravis and 31 patients with generalized myasthenia gravis. Clinical benefits and clinical remission, which was diagnosed when the patients were symptom-free without medications for at least 1 year, were compared with those of 21 patients in the occasional treatment group who received medications occasionally over the perioperative period. RESULTS: Postoperative respiratory failure and myasthenic crisis did not occur in the programmed treatment group but did occur in 6 patients in the occasional treatment group. Remission rates in the programmed treatment group (mean follow-up, 6.4 years) were 30% at 3 years, 38% at 5 years, and 46% at 10 years; rates in the occasional treatment group (mean follow-up, 9.6 years) were 25% at 3 years, 25% at 5 years, and 45% at 10 years. CONCLUSIONS: Programmed steroid therapy in patients with nonthymomatous myasthenia gravis is feasible and it provides clinical benefit when fluctuating symptoms occur during the perioperative period.     

胸骨部分劈开切口行扩大的胸腺切除术治疗重症肌无力

目的介绍胸骨部分劈开切口行扩大的胸腺切除术治疗重症肌无力的手术方法和效果。方法2000年1月至2005年12月,采用胸骨正中部分劈开切口,对32例重症肌无力患者进行了扩大的胸腺切除手术。结果6例肌无力症状完全缓解,19例症状显著改善,5例症状轻微改善,而仅有2例手术后症状完全没有好转。结论胸骨部分劈开切口是实施扩大的胸腺切除治疗重症肌无力的一种安全、有效的手术径路,并且对患者的创伤小,避免了手术后并发症,特别是肌无力危象的发生。     

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。     

High-dose steroids for perioperative management of patients with myasthenia gravis undergoing thymectomy. A preliminary report

To eliminate the morbidity associated with thymectomy, we used high-dose corticosteroids perioperatively in 32 patients who had Class II and III myasthenia gravis. All patients were extubated within 2 hours after the operation, and none required respiratory support postoperatively. The average stay in the intensive care unit was 20 hours and the average hospital stay, 6 days. Addition of high-dose corticosteroids to the perioperative managment of patients with myasthenia gravis undergoing thymectomy improved the immediate operative results and eliminated the morbidity associated with this procedure.     

Cervical thymectomy in the treatment of myasthenia gravis

Treatment modalities in myasthenia gravis consist of surgery, chemotherapy and plasmapheresis. Thymectomy can be accomplished either through a median sternotomy or through a small, transverse cervical incision. Forty patients who underwent cervical thymectomy for non-thymomatous myasthenia gravis were studied retrospectively. Twenty-six patients (65%) showed a favourable response to thymectomy and there were statistically significant improvements in myasthenic symptoms and reductions in medication requirements. Age, sex, duration of symptoms and thymic histology were not predictive of response to thymectomy. Operative mortality was zero and operative morbidity was minimal. During the last 6 years, only two of 22 patients required admission to the intensive care unit postoperatively. The postoperative hospital stay ranged from 2 to 23 days. Cervical thymectomy does not preclude later sternotomy in those patients who fail to respond favourably. We therefore recommend cervical thymectomy as the initial surgical procedure in the treatment of non-thymomatous myasthenia gravis.     

Surgical outcome of thymectomy for myasthenia gravis

Background The medical treatment for myasthenia gravis has been reported to have remission rates as low as 15%. Thymectomy on the other hand has been reported to have clinical remission rates up to 80% and therefore has become the accepted mode of treatment. Methods 57 patients diagnosed with myasthenia gravis underwent thymectomy at the Christian Medical College and Hospital, Vellore from January 1994 to December 2003. The aim of this study was to determine the outcome for myasthenia gravis after thymectomy. Results Our results indicated that female sex had a better over all prognosis, Ossermann stage, I, IIA, & III was associated with higher incidence of complete clinical remission and the response to thymectomy decreased with increasing Ossermann stage. Post operative medication requirement reduced significantly as compared to the preoperative requirement. Conclusion We therefore conclude that trans-sternal thymectomy was found to be beneficial to all patients of mild to moderate myasthenia gravis, with 70.2% patients showing improvement postoperatively. We also advocate thymectomy for ocular myasthenia gravis.     

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.     

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.     

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.