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。     

新的重症肌无力外科临床分型及分期在围手术期中的应用

目的 探讨围手术期应用新的重症肌无力（myasthenia gravis,MG）外科临床分型及分期降低术后肌无力危象（myasthenic crisis,MC）发生率的有效性。方法 回顾性分析2018年1月—2022年6月河南省人民医院重症肌无力综合诊疗中心收治的全身型MG患者的临床资料。在首次就诊、术前1 d、术后3 d采用重症肌无力日常活动能力（myasthenia gravis-activities of daily living,MG-ADL）评分及重症肌无力定量（quantification of the myasthenia gravis,QMG）评分评估患者状况。按照新的外科临床分型根据疾病发展过程将患者分为A组（Ⅱ型）、B组（Ⅲ型+Ⅳ型+Ⅴ型），所有患者均通过药物等干预将症状控制在缓解期或稳定期后行胸腔镜下胸腺（瘤）扩大切除术，分析术后MC发生率及手术的有效率。采用正态分布法和百分位数法计算QMG评分与MGADL评分单侧95%参考值范围。结果 纳入126例患者，其中男62例、女64例，年龄13～71(46.00±13.00)岁。A组95例，B组31例，两组患者术前基线资...     

电视胸腔镜手术治疗非胸腺瘤重症肌无力的中期疗效和生活质量

目的评估电视胸腔镜手术(video-assisted thoracoscopic surgery,VATS)治疗非胸腺瘤重症肌无力(nonthymomatous myasthenia gravis,NTMG)的中期疗效和生活质量,以合理选择手术方式。方法 2003年5月~2006年6月施行VATS胸腺扩大切除术治疗26例NTMG,术后3年随访采用术后MG相关症状变化DeFilippi分级和美国重症肌无力基金会MG评分(MGFA评分)评估术后疗效;采用欧洲癌症研究和治疗组织(European Organization for Research and Treatment ofCancer,EORTC)生活质量问卷(quality-of-life questionnaire,QLQ)评分评估术后生活质量。结果 26例手术顺利,无肌无力危象发生。术后1例并发肺炎,无围手术期死亡。术后3年随访时MG相关症状变化DeFilippi分级,1~3级26例,4~5级0例,总有效率为100%;MGFA评分(8.1±1.6)分优于术前(14.2±2.9)分(t=-9.391,P=0.000);EORTC-QLQ6项(包括躯体、角色、认知、情绪、社会、总生活质量)评分术后均高于术前,除社会功能评分外,差异均有统计学意义(P0.05)。结论 VATS胸腺扩大切除治疗NTMG,MG症状改善率高,生活质量好,术后并发症少,是治疗NTMG的一种有效方法,具有良好临床应用前景。     

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。     

115例重症肌无力患者胸腺切除手术的麻醉管理

目的 探讨重症肌无力(MG)患者胸腺切除手术的麻醉处理方法和分析术后需要呼吸支持的原因。方法 115例MG患者经胸骨正中劈开胸腺切除手术，MG分型：Ⅰ型12例，Ⅱa型20例，Ⅱb型68例，Ⅲ型10例，Ⅳ型5例。术前口服平时用量的溴吡斯的明；诱导用丙泊酚、芬太尼和琥珀胆碱；麻醉维持用氧气、氧化亚氮和异氟醚。术中不用非去极化的肌松药。拔管指征；患者完全清醒，抬头坚持5s，潮气量大于10ml／kg，吸气负压峰值大于-20cm H2O。术后6h内开始口服小剂量的溴吡斯的明。根据术后是否需要呼吸支持分成两组：术后立即拔管组和术后延迟拔管组。结果 101例在手术室内成功拔管，拔管率为87．8％；14例术后需呼吸支持6h至7d，术后延迟拔管率为12．2％；术后需要带气管导管和呼吸支持的患者随MG的临床分级增加而增高。结论 MG患者行胸腺切除术用丙泊酚、芬太尼和琥珀胆碱麻醉诱导，吸入氧气、氧化亚氮和异氟醚维持，是一种安全的麻醉方法；MG患者术后需要呼吸支持的发生率随MG的临床分级增加而增高。     

电视胸腔镜与胸骨正中切口行胸腺扩大切除治疗重症肌无力的对照研究

目的分析比较电视胸腔镜入路(VATS组)与胸骨正中切口(TS组)入路行胸腺扩大切除术治疗重症肌无力的临床特点及疗效,从而为重症肌无力患者手术方案的选择提供进一步的临床指导依据。方法回顾性分析李惠利医院心胸外科2010年1月至2017年12月113例重症肌无力患者行胸腺扩大切除手术的临床资料,其中电视胸腔镜组(VATS组)58例,胸骨正中切口组(TS组)55例。结果VATS组与TS组患者的一般临床资料无差异,VATS组与TS组患者比较:手术时间(分钟)、术中失血量(ml)、术后、口感染、肺部感染、术后重症肌无力危象发生率、住院天数(天)、总住院费用(元)、术前MGFA临床分型和术后病理分型等指标。VATS组患者手术时间(分钟)更短[(99.35±29.2)s vs.(112.53±32.2)s,P=0.04],手术失血量更少[(42.9±14.3)ml vs(139.0±15.1)ml,P=0.03],术后引流量更少[(139.0±18.1)ml vs(539.0±19.2)ml,P=0.01];住院时间更短[(4.0±0.3)天vs.(7.3±1.2)天,P=0.02];术后切口感染率及肺部感染更低;总住院费用更低;术后重症肌无力危象发生率,术前MGFA临床分型,术后病理分型等指标比较差异无统计学意义。结论电视胸腔镜入路相较胸骨正中切口入路行胸腺扩大切除术,明显减少失血量,术后恢复快,缩短住院时间,具有很好的安全性,可替代胸骨正中切口入路术。     

重症肌无力胸腺切除236例分析

目的　总结重症肌无力 (MG)胸腺切除的经验及治疗效果。　方法　回顾性分析我科 1978年 1月至2 0 0 2年 12月手术治疗 2 36例 MG患者术前、术后的处理 ,危象发生的有关因素及远期疗效。　结果　 2 36例术后发生危象 4 4例 (18.6 % ) ,危象的发生与术前准备、Osserm an临床分型、是否伴胸腺瘤密切相关。全组术后死亡 3例(1.3% ) ,1例死于误吸所致急性呼吸窘迫综合征 (ARDS) ,2例死于危象 ,占危象病例的 4 .5 % ,1、3、5年的有效率分别为 84 .6 %、91.0 %和 89.0 %。　结论　 MG行胸腺切除安全、有效 ,对术后发生危象高危因素的患者延期气管拔管可减少气管切开 ,部分胸骨劈开径路创伤小 ,其远期效果与文献报道其他径路无显著差别。     

电视胸腔镜与胸骨正中切口行胸腺扩大切除治疗胸腺瘤合并重症肌无力临床效果的比较

目的通过比较电视胸腔镜(Video-assisted thoracic surgery,VATs)和胸骨正中切口胸腺扩大切除术治疗胸腺瘤合并重症肌无力(MG)的临床效果,探讨电视胸腔镜胸腺扩大切除术的安全性及可行性。方法回顾性分析2012年1月至2016年6月本中心的33例胸腺瘤并MG患者的临床资料,其中15例行电视胸腔镜胸腺扩大切除术(VATs组),18例行胸骨正中入路胸腺扩大切除术(开放组)。结果两组的术前临床特征无显著差异。两组均无术中死亡,且VATs组无转开放发生。VATs组的术中出血量、术后并发症及术后住院天数均较开放组显著减少,且均有统计学意义(P0.05)。两组术后重症肌无力缓解情况无显著差别,其中VATs组的有效率为86.6%,开放组有效率为83.3%。结论电视胸腔镜胸腺扩大切除术治疗胸腺瘤并MG是安全、可行的,但其长期临床效果需进一步研究。     

胸腺瘤合并重症肌无力与单纯重症肌无力的临床特征及手术疗效分析

目的　探讨胸腺瘤合并重症肌无力 (MG)与单纯MG的临床特征及手术疗效。方法回顾性分析 1978年至 2 0 0 3年 2 4 3例MG患者临床资料 ,比较胸腺瘤合并MG组 (6 8例 )与单纯MG组 (175例 )的临床特点 ,以及术后并发症、缓解率、生存情况。结果　胸腺瘤合并MG组较单纯MG组年龄大 [平均年龄分别为 (41± 14 )与 (2 8± 16 )岁 ,t=6 .138,P =0 0 0 0 ]、病程短 (平均分别为 10、2 4个月 ,t =3 783,P =0 0 0 0 ) ,术后肌无力危象发生率高 [分别为 5 0 0 % (34/ 6 8)与 5 7% (10 / 175 ) ,χ2=6 4 77,P =0 0 0 0〗 ,两组Osserman分型差异无显著意义 (χ2 =7 6 78,P =0 10 4 )。胸腺瘤合并MG组肌无力症状完全缓解率、部分缓解率 ,术后 1年分别为 10 2 % (6例 )、6 2 7% (37例 ) ,术后 3年分别为 2 1 6 % (8例 )、75 7% (2 8例 ) ,低于单纯MG组 [术后 1年分别为 2 2 1% (30例 )、94 1% (12 8例 ) ,术后 3年分别为 4 4 4 % (44例 )、94 9% (94例 ) ,P值分别为 0 0 4 9、0 0 0 0、0 0 15、0 0 10 ];术后 5年两组比较肌无力症状完全缓解率、部分缓解率差异无显著意义 (P =0 4 5 7,P =0 6 99)。胸腺瘤合并MG组 3、5年生存率分别为 96 3%、84 4 % ,显著低于单纯MG组的 98     

胸腺瘤切除术后发生重症肌无力的危险因素分析

目的探讨胸腺瘤切除术后发生重症肌无力(myasthenia gravis,MG)患者的危险因素。方法回顾性分析新疆医科大学第一附属医院2002年6月至2015年5月行胸腺肿瘤切除术126例患者的临床资料,其中男51例、女7 5例,年龄31~73(51.71±14.06)岁。所有患者术前均无MG,术后病理确诊为胸腺瘤。分析患者术后发生MG的相关因素。结果 9例患者术后出现MG(7.1%)。不完整切除(P=0.024)、病理类型(P=0.048)、合并免疫疾病(P=0.024)、术后肺部感染(P=0.036)为术后发生MG的危险因素,术中未能完整切除者或侵袭性胸腺瘤术后放化疗(P=0.011)可降低术后MG的发生风险。结论肿瘤不完整切除、WHO病理分型A型和AB型、伴随免疫疾病、术后肺部感染患者行胸腺瘤切除术后易发生MG,术中未能完整切除者或侵袭性胸腺瘤术后应行放化疗。     

重症肌无力术后发生肌无力危象的危险因素分析

目的分析重症肌无力术后发生肌无力危象的影响因素。方法回顾性分析2006年6月至2019年6月首都医科大学宣武医院胸外科65例重症肌无力术后发生肌无力危象患者的临床资料,其中男31例(47.7%)、女34例(52.3%),年龄15~78(45.7±17.8)岁。分析患者的手术方式、手术时间、病理类型等与术后发生肌无力危象的关系。结果手术时间、病理类型是术后肌无力危象的影响因素。Osserman分型的受试者工作特征曲线下面积(AUC)为0.676,临界值为ⅡB型,灵敏度37.5%,特异性90.5%,约登指数0.280。Masaoka分期的AUC为0.682,临界值为Ⅱ期,灵敏度62.5%,特异性66.7%,约登指数0.292。出血量的AUC为0.658,临界值为90 mL,灵敏度87.5%,特异性69.6%,约登指数0.304。结论术前Osserman分型、病理类型、手术时间、出血量是发生术后肌无力危象的影响因素,因此充分的术前准备、快捷仔细的术中操作和积极的术后处理可减少术后肌无力危象的发生。     

Prediction of the need for postoperative mechanical ventilation in myasthenia gravis: Thymectomy compared to other surgical procedures

In a recent report Leventhal, Orkin, and Hirsh¹¹ described a scoring system felt to be of value in predicting the need for postoperative mechanical ventilation in patients with myasthenia gravis undergoing thymectomy. Leventhal,et al. identified four risk factors felt to have predictive value, namely: (1) duration of myasthenia gravis ≥6 years, (2) chronic respiratory disease, (3) dose of pyridostigmine ≥750 mg per day, and (4) vital capacity ≤2.9 litres. Forty-six patients with myasthenia gravis who received 68 general anaesthetics were studied retrospectively. They represented the past 10 years’ anaesthetic experience with myasthenia gravis at the Vancouver General Hospital. The patients were divided into two groups: (1) those who underwent thymectomy, and (2) those who underwent procedures other than thymectomy. Using the risk factors of Leventhal,et al., a predictive score was assessed for each patient; the time to postoperative tracheal extubation was also noted for each patient. From this study it was concluded that the scoring system proposed by Leventhal,et al. may have been of some value in predicting whether or not a particular patient undergoing thymectomy was likely to need ventilation postoperatively. In 41 myasthenics who had procedures other than thymectomy, however, this scoring system was found to be of no value.     

Multivariate determinants of the need for postoperative ventilation in myasthenia gravis

Purpose

Following transsternal thymectomy, up to 50% of patients may require postoperative ventilation. The aim of this study was to identify the variables most useful in predicting the myasthenic patient who needs postoperative mechanical ventilation.

Methods

We applied multivariate discriminant analysis to preoperative physical, historical, laboratory and intraoperative data of 51 myasthenic patients who underwent transcervical-transsternal thymectomy to select those variables most useful in predicting the postoperative need for mechanical ventilation. The receiver operating characteristic (ROC) curve was also used to describe the discrimination abilities and to explore the trade-offs between sensitivity and specificity of the model.

Results

Discriminant analysis identified seven risk factors that correlated with the need for postoperative ventilation: FVC, FEF_25–75%, MEF_50% and their percentages of the predicted values, as well as, sex. The model correctly predicted the actual ventilatory outcome in 88.2% of patients. The area under the ROC curve verified that our model correctly predicted the actual ventilatory outcome with a probability of 88.2%.

Conclusions

This model can be used for predicting the need for postoperative mechanical ventilation in myasthenia gravis patients.     

The prediction of the need for postoperative mechanical ventilation in patients with myasthenia gravis undergoing transsternal thymectomy

During a sixteen-year period, 51 patients with myasthenia gravis underwent trans-sternal thymectomy including extended resection of the adipose tissue around thymus. Using the criteria of risk factors described by Leventhal and Kimura, a predictive score was assessed for our 51 patients. Leventhal's scoring system achieved an accuracy of 54.3%, a sensitivity of 20.0% and a specificity of 63.9%. And, Kimura's scoring system achieved an accuracy of 75.0%, a sensitivity of 81.8% and a specificity of 73.0%. From this study it was concluded that Kimura's scoring system is statistically more adaptive for Japanese than Leventhal's scoring system, and the Leventhal's accuracy may have increased by adding "bulbar symptoms" and "preoperative crisis" as other risk factors and by decreasing "points" for "duration of myasthenia" and "pyridoxamine dosage", and the "Kimura's " accuracy may have increased by decreasing "7 points" for preoperative crisis. Consequently, patients who received postoperative ventilation were compared with the group who did not, with respect to the 19 factors. Evidence is that Osserman's type, bulbar symptoms, preoperative crisis and preoperative % VC were influenced to require the postoperative ventilation, but sex, age, thymic histology, duration of myasthenia and pyridoxamine dosage were not any influenced to require the postoperative ventilation.     

Myasthenia gravis: prognostic significance of clinical data in the prediction of post-thymectomy respiratory crises.

Medical records of 170 patients who had undergone thymectomy for myasthenia gravis were reviewed from the point of view of respiratory disturbances. In the group of patients requiring mechanical ventilation for over 24 hours after operation the incidence of high preoperative cholinesterase inhibitor intake, severe bulbar symptoms and severe myasthenia gravis with anamnestic respiratory crisis and cardiorespiratory disease were much higher than in the group of patients who could have their trachea extubation within 24 hours. The presence of a thymic tumour, patients' age over 50 years and the so-called precrisis have revealed differences between the two groups, while the patient groups were identical in mean age, duration of myasthenia gravis and sex distribution. The above clinical data are recommended to be considered in the evaluation of the need for postoperative mechanical ventilation or extubation and preparation of preventive tracheotomy.     

CT and myasthenia gravis: correlation between mediastinal imaging and histopathological findings

The surgical strategy in patients with myasthenia gravis (MG) is influenced by the suspicion of thymoma based on mediastinal imaging. Aim of this retrospective study was to analyse the accuracy of CT of the mediastinum in predicting the histological findings in patients with MG referred for thymectomy. Thirty-four CT-scans of MG patients referred for thymectomy between October 1989 and October 2003 were retrospectively evaluated by three cardio-thoracic surgeons and three radiologists. Data were analysed by Kappa statistics to judge inter-observer variance and were compared to the histopathological findings to determine predictive value. Observer agreement among the radiologists was fair (Kappa=0.28) and among the cardio-thoracic surgeons slight (Kappa=0.08). The average negative predictive value of no thymoma on CT was 91% (range 78-100%). The average positive predictive of thymoma on CT value was only 39% (range 29-58%). The average sensitivity of CT imaging in the study population was 75% (range 25-100%) and the average specificity was 62% (range 42-81%). In patients with MG undergoing thymectomy, CT is helpful in detecting thymoma, but the high inter-observer variation indicates that it remains difficult to distinguish lymphoid follicular hyperplasia from thymoma. This will influence the surgical strategy in patients with myasthenia gravis.     

Prediction of the need for postoperative mechanical ventilation in myasthenia gravis patients

The prediction of the need for postoperative mechanical ventilation in 23 myasthenia gravis patients, who had undergone thymectomy from January 1983 to December 1989, was evaluated. Five patients who had needed postoperative mechanical ventilation over 24 hours were compared with the patients in whom the tracheal tubes had been uneventfully extubated within 24 hours postoperatively. In this study, 47.8%, 78.3%, 91.3% of the patients were correctly predicted using the scoring systems proposed by Leventhal et al., Makii et al. and Kimura et al., respectively. The product of %VC by FEV1.0% was proposed in this study for the prediction of the need of postoperative mechanical ventilation in patients with myasthenia gravis. If the product is less than 8,300, the patient is expected to need postoperative mechanical ventilation. Nineteen patients out of 23 cases (82.6%) were predicted correctly by this value. Only one patient, who had been predicted to be in no need, was actually ventilated for 3 days. In conclusion, the product of %VC by FEV1.0% is a simple and practical method of predicting whether a patient with myasthenia gravis undergoing thymectomy requires postoperative mechanical ventilation or not.