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。     

扩大胸腺切除术治疗重症肌无力的临床研究

目的:探讨重症肌无力的外科治疗和围术期处理方法。方法:采取扩大的胸腺切除和纵隔脂肪组织清扫手术治疗重症肌无力89例,其中单纯重症肌无力48例,胸腺瘤合并重症肌无力41例,其中1例伴发甲状腺机能亢进,27例有肌无力危象发作史。Osserman分型:Ⅰ型21例,Ⅱa型17例,Ⅱb型31例,Ⅲ型15例,Ⅳ5例。1987年以前手术采用前胸二或三肋间横切口或右胸前外侧切口入路手术。1988年以后采用胸骨正中入路施术。术前均口服吡啶期的明每天180～600mg分次服用,Ⅱb型以上病加服强的松每天30～60mg或90mg隔日一次顿服。手术当日和术后抗胆碱酯酶药物给药的剂量、方法、时间不变。术日激素采用氢考300～400mg静滴。危重病人采取预防性气管切开或延迟气管插管和呼吸机辅助呼吸,以帮助病人渡过手术创伤期和危象发生期。结果:全组13例术后12～22小时之间发生肌无力危象,死亡3例,死亡率3.4％。其余均治愈出院。对32例病人随访12个月～120个月,缓解6例,明显改善13例,改善4例,无效1例,差2例,失访6例。结论:正中入路施行扩大胸腺切除和纵隔脂肪组织清扫效果可靠,围术期合理抗胆碱酯酶药物和激素是手术成功的前提,适时的气管切开和延迟气管插管拔管行呼吸机辅助呼吸为病人提供了渡过手术创伤期的安全保证。     

胸腺切除术治疗重症肌无力23例

目的:通过重症肌无力合并胸腺瘤及胸腺增生23例治疗体会的总结,探讨重症肌无力合并胸腺瘤及胸腺增生的外科治疗方法.方法:对23例重症肌无力合并胸腺瘤及胸腺增生患者的临床资料进行回顾性分析与总结.结果:全组均行手术切除胸腺,死亡3例,2例死于肌无力危象,1例死于成人呼吸窘迫综合征.随访1～8年,手术效果较好.结论:重症肌无力是一种自身免疫性疾病,胸腺被认为是参与发病的重要组织,胸腺切除是治疗重症肌无力的有效方法,如能尽早手术治疗,将能获得较好的疗效.     

胸腔镜胸腺切除术治疗重症肌无力

2002年2月至2005年5月，我们采用胸腔镜行胸腺切除治疗重症肌无力（MG）病人70例，现总结报道如下。     

胸腺扩大切除术治疗重症肌无力30例

目的 探讨重症肌无力外科治疗方法。方法:30例重症肌无力患者均行胸腺扩大切除术,术后机械通气1—7天(平均3天)。结果:术后出血、右气胸及伤口感染各1例;肌无力危象3例,2例缓解,1例死亡。术后随访29例中,症状明显缓解者15例,改善12例,无变化2例。结论:本术式切除胸腺组织较彻底,未增加手术难度及并发症,临床效果良好。     

胸腔镜胸腺扩大切除治疗非胸腺瘤性重症肌无力

目的：探讨胸腔镜辅助治疗非胸腺瘤性重症肌无力（ non-thymoma myasthenia gravis ,non-TMG）的价值。方法2007年1月～2011年8月,对62例non-TMG行胸腔镜辅助胸腺扩大切除术。男24例,女38例。年龄18～67岁,平均31岁。根据改良Osserman分型,Ⅰ型17例,Ⅱa型28例,Ⅱb型15例,Ⅲ型2例。按美国重症肌无力协会标准（ MGFA）进行疗效判定。结果全组无手术死亡,手术时间40～150 min,平均110 min。术后保留胸腔引流管时间1～5 d,平均2．0 d。术后7天内脱离呼吸机57例,超过7天5例。62例术后病理诊断结果均显示胸腺增生,合并胸腺外脂肪（心包及双侧心膈角）内查见胸腺小体22例。术后随访2～5年,平均4．2年。完全稳定缓解31例,药物缓解17例,微小症状表现6例,改善8例。结论胸部CT扫描尚不能根据胸腺本身“体积”大小判断胸腺增生与否。术中应重视迷走胸腺组织清除。胸腔镜下胸腺扩大切除术是治疗non-TMG有效的手段。     

286例重症肌无力胸腺切除术疗效分析

重症肌无力（MG）外科治疗效果已被公认。1978年至2006年4月，我们行胸腺切除治疗重症肌无力286例，现报道如下。[第一段]     

胸腺切除治疗重症肌无力34例体会

自１９８０年４月至１９９５年９月，采用胸腺切除治疗重症肌无力３４例，年龄５～６２岁，术后早期死亡１例，死亡率２．９４％，３３例随访３个月至１２年，１年内缓解和改善率为８７．９％（２９／３３），１～３年缓解和改善率为８８．５％（２３／２６），３年以上缓解和改善率为８０．０％（４／５）。随访结果表明胸腺切除治疗重症无 力效果满意，延迟拔除气管插管或早期行气管切开术，是防治术后重症肌无力危象的有效措施。     

扩大胸腺切除治疗重症肌无力的结果分析

目的：总结1990年2月至1999年10月手术治疗重症肌无力的随访结果，并分析其预后因素。方法：54例病人均行扩大胸腺切除术，按改良Osserman标准分为I型15例，Ⅱa型14例，Ⅱb型16例，Ⅲ型9例，随访结果按完全缓解，改善，无效，差进行评价。结果：手术后完全缓解29．6％，改善48．1％，无效13．0％，差9．3％，手术疗效与术前病程长短及Osserman分型有关，与性别，年龄，胸腺病理类型无关，结论：扩大胸腺切除术是治疗重症肌无力的有效方法，对重症肌无力病人应及早手术治疗。     

Thymoma appearing 10 years after an extended thymectomy for myasthenia gravis

Occurrence of thymoma after an extended thymectomy through a full median sternotomy for nonthymomatous thymectomy has been very rarely reported. A 60-year-old male patient who had myasthenia gravis (MG) for 11 years had an extended thymectomy operation with a pathology of thymic hyperplasia and developed a mass in the aortopulmonary window. We resected the mass via anterior left thoracotomy by dividing the ductus arteriosus and mobilizing the aorta. Cord vocal augmentation procedure was done due to the resection of the left recurrent laryngeal nerve.     

Inclusion of the transcervical approach in video-assisted thoracoscopic extended thymectomy (VATET) for myasthenia gravis: a prospective trial

Background Because evidence-based data regarding the quality of video-assisted thoracoscopic thymectomy for the treatment of myasthenia gravis are lacking, a prospective trial comparing three different operative approaches was conducted to evaluate their efficacy. Methods This prospective study enrolled 20 consecutive patients with nonthymomatous myasthenia gravis. A series of three approaches for bilateral video-assisted thoracoscopic extended thymectomy (VATET) using the anterior chest wall–lifting method (original), the original method with a flexed-neck position (modified), and the original method with a transcervical approach (final) were prospectively performed in each patient for quantitative and pathologic evaluation of the residual thymus after each approach. Results Complete VATET required 242 ± 48 min, with the transcervical procedure requiring 23 ± 12 min. After the modified method, the residual thymus in the cervical region was 1.5 cm in size and weighed 0.8 g (0.8% of the entire thymus), as compared with a size of 2.2 cm and a weight of 1.3 g (3.2%) after the original method. Each value is the result of comparison with the final method. Histopathologic studies showed residual tissue in the germinal center as well as Hassall’s corpuscles in more than 70% of cases. Conclusion The findings show that VATET without the transcervical approach could be an immunologically incomplete treatment for myasthenia gravis. Therefore, the transcervical approach should be included in VATET procedures to ensure radicality.     

胸腔镜胸腺扩大切除加颈部切口治疗重症肌无力

目的 探讨胸腔镜胸腺扩大切除加颈部切口治疗重症肌无力的可行性和术后疗效.方法 2005年1月至2006年2月采用胸腔镜胸腺切除加颈部切口治疗重症肌无力患者41例,其中男性18例,女性23例,年龄18～67岁.病程1个月～3年.术中除完整切除胸腺外,还清除前纵隔区域及颈根部的异位胸腺和脂肪组织.结果 全组41例手术完全在电视胸腔镜下完成,平均手术时间162 min.术后发生肌无力危象7例.术后病理学诊断:4例颈部发现残存胸腺上极,35例患者气管前胸骨上间隙有肿大淋巴结(3～17枚).所有患者均痊愈出院.术后随访观察2年,完全缓解率41.4%,有效率达85.4%.结论 胸腔镜下胸腺切除术治疗重症肌无力能取得较为理想的治疗效果,胸腔镜扩大胸腺切除加颈部切口对于颈根部异位胸腺的清除是一补充.     

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。     

Video-assisted thymectomy for myasthenia gravis: an update of a single institution experience

Objective: Video-assisted thymectomy was introduced in 1992 as a minimally invasive alternative for the treatment of myasthenia gravis. As experience with this technique is limited and follow-up short, we present this expanded and updated experience for purposes of validation of the technique. Methods: Thirty-eight video-assisted thymectomies for myasthenia gravis were performed in our institution between March 1992 and March 2002. Two patients were lost to follow-up. We analyzed clinical results of 36 patients (14 males and 22 females) with a mean age of 41.2 years. Preoperative clinical staging was assessed by the newly recommended Myasthenia Gravis Foundation of America Clinical Classification. Clinical status at follow-up was assessed by the Myasthenia Gravis Foundation of America Postintervention Status classification. Results: There was no perioperative mortality or long-term morbidity. One of 38 (2.6%) patients required conversion to limited thoracotomy for bleeding. The mean length of hospital stay was 1.64 days (range 0–8 days) with a median stay of 1 day. The mean length of follow-up is 53.24 months (range 4–126 months). Overall clinical improvement at follow-up was observed in 30 of 36 (83.0%) patients, with five of 36 (14.0%) patients in complete stable remission. Conclusions: Video-assisted thymectomy for myasthenia gravis provides acceptable clinical long-term results by a minimally invasive approach comparable to standard surgical approaches to the disease. The presented data is reported in accordance with the new guidelines by Myasthenia Gravis Foundation of America Task Force for valid comparison with future studies.     

横断胸骨扩大胸腺切除治疗全身型重症肌无力

目的探讨经横断胸骨入路行扩大胸腺切除治疗重症肌无力的临床疗效。了解该入路的特点及临床疗效有助于该领域胸外科医师在临床实践中进行更好的临床决策。 方法对1998至2008年在复旦大学附属华山医院胸心外科行横断胸骨治疗全身型重症肌无力的211例患者的临床资料进行回顾性研究,分析患者术中和术后基本情况。并对术后5年进行随访的患者根据是否合并胸腺瘤进行分组,采用χ²检验比较两组间的疗效。 结果经横断胸骨入路能满足对合并Masaoka-Koga Ⅰ期及Ⅱ期胸腺瘤患者及无瘤患者实施扩大胸腺切除的需要,手术并发症较低,便于围术期护理,恢复迅速。对173例患者进行术后5年随访,总缓解率达到79.8%(138/173),无肿瘤复发。合并胸腺瘤组的总缓解率为81.9%(59/72),未合并胸腺瘤组的总缓解率为78.2%(79/101),两组间比较差异无统计学意义(χ²＝0.362,P＝0.548)。 结论与其他常用手术入路相比,横断胸骨入路的特点值得该领域胸外科医师关注。     

电视胸腔镜胸腺扩大切除治疗重症肌无力107例临床分析

目的探讨电视胸腔镜下胸腺扩大切除术治疗重症肌无力的临床效果。方法对1995年6月至2004年6月,台湾长庚纪念医院胸外科和北京大学人民医院胸外科根据临床表现及肌电图检查诊断证实为单纯重症肌无力,行电视胸腔镜胸腺完整切除及前纵隔脂肪组织廓清术治疗的107例患者的临床资料进行回顾分析。结果术后随访1—98个月,完全缓解34例,改善55例,全组完全缓解及改善率为83％,无手术死亡病例。结论电视胸腔镜下手术切除胸腺及前纵隔脂肪组织治疗重症肌无力临床效果良好,安全,创伤较小。     

Matched-pair comparison of three different approaches for thymectomy in myasthenia gravis

Background: Because of the lack of evidence-based data comparing different techniques for thymectomy (Thx), a matched-pair study was conducted to evaluate the role of thoracoscopic Thx (tThx) more objectively. Methods: Of 182 patients who underwent Thx, 3 groups according to the operation technique were matched for myasthenia gravis (MG) without thymoma, age, gender, and severity of MG. Twenty patients each who had Thx through anterolateral thoracotomy (aThx), extended median sternotomy (sThx), and tThx were compared for length of operation, postoperative morbidity, complete remission, quantification of improvement of MG, and cosmetic results. Results: Complete tThx required 197 ± 35 min as compared to 113 ± 43 min for sThx and 82 ± 27 min for aThx (P < 0.001). With zero mortality the overall postoperative morbidity rate was 25% for sThx versus 15% for aThx and 5% for tThx (P < 0.05). There was no difference in complete remission of MG. The median activities of daily living (ADL) scores improved by 6.0 (1–19) after tThx, 5.5 (2–4) after sThx, and 7.5 (0–12) after aThx. Best cosmetic results were achieved after tThx. Conclusions: There was adequate cumulative medium-term improvement of MG and less postoperative morbidity after tThx, which may become the preferred technique for Thx.