重症肌无力胸腺切除围术期处理

胸腺切除治疗重症肌无力是常用的方法,并使70％的病者在手术治疗后可望症状改善,然而胸腺切除术后呼吸功能处理的好坏,与胸腺切除的疗效直接相关,本院胸外科自1989年1月至1993年12月共对99例     

胸腺切除治疗重症肌无力

我科自1966年3月至1991年7月采用胸腺切除术治疗重症肌无力63例。现将我们的体会总结如下: 临床资料本组男27例,女36例。年龄<10岁者11例,11～20岁7例,21～30岁13例,31～40岁13例,41～50岁8例,51～60岁10例,>61岁1例。按Monden改良的Osserman分型:重症肌无力仅限于眼肌者为Ⅰ型;轻度累及全身者为Ⅱ_A型;严重累及全身者为Ⅱ_B型;急性者为Ⅲ型。本组Ⅰ型16例,Ⅱ_A型18例,Ⅱ_B型20例,Ⅲ型9例。病期最短者1个月,最长者15年,平均1年9个月。全部病人都进行了胸部X线正侧位照片,发现胸腺瘤9例。纵隔增宽1例,胸腺未见异常53例。CT扫描7例,诊断胸腺瘤4例,胸腺增生2例,胸腺未见异常1例。     

胸腺切除治疗22例重症肌无力的疗效观察

重症肌无力 (ＭＧ)的发病与胸腺关系密切 ,15 %～ 30 %的ＭＧ患者合并胸腺增生或胸腺瘤。以往ＭＧ的治疗以药物为主 ,我们自 2 0 0 0年至 2 0 0 2年对 2 2例ＭＧ患者进行了胸腺切除治疗 ,现总结如下。1　资料与方法　1.1　一般资料　成人组 13例 ,男 7例 ,女 6例 ,年龄 2 2～ 6 3岁 ,平均 4 1.2岁。儿童组 9例 ,男 2例 ,女 7例 ,年龄 2～ 13岁 ,平均 7.6岁。眼肌型 14例 (成人 5例 ,儿童 9例 ) ,全身型 5例 ,球麻痹型 3例 ,均为成人。胸腺瘤 11例 (良性 8例 ,恶性 3例 ) ,均为成人 ;胸腺增生 9例 (成人 1例 ,儿童 8例 ) ;正常胸腺组织 2例 (…     

行胸腺切除重症肌无力患者临床分析

目的探讨重症肌无力患者的临床特点、诊断方法和治疗效果。方法收集本院2003-09—2010-09收治的122例重症肌无力行胸腺切除患者的临床资料,术后随访2a观察并发症及病死率。结果最常见的临床表现为眼睑下垂(77.0%)和上肢无力(70.5%),CT扫描显示51.6%患者胸腺增生,11.5%患者存在胸腺瘤。术后并发症发生率为13.1%,病死率6.6%。结论行胸腺切除术后重症肌无力患者有较高的病死率,应进行更谨慎的术前评估和治疗。     

胸腺瘤并重症肌无力危象围手术期护理

我科自2000-12～2006-12共手术治疗46例胸腺瘤合并重症肌无力(MG)病人,20例术后发生危象,均抢救成功,无围手术期死亡.     

药物及胸腺切除手术治疗重症肌无力的疗效分析

目的探讨伴有不同胸腺情况的重症肌无力(MG)患者对药物及胸腺切除术(TX)的疗效。方法对我院确诊的71例MG患者进行回顾性分析,根据胸腺CT情况将患者分为胸腺CT正常组、合并胸腺增生组、合并胸腺瘤组及合并胸腺癌组,采用Fisher精确检验法比较各组对两种治疗方法的疗效。结果胸腺CT正常的MG患者多采取药物治疗,缓解率为52%,有效率为88.5%;合并胸腺增生、胸腺瘤、胸腺癌的MG患者多采取了TX治疗。合并胸腺增生组的术后缓解率(62.5%)明显高于合并胸腺瘤组(8.3%)(P<0.05);合并胸腺增生组的术后有效率(93.7%)与合并胸腺瘤组差异无显著性(83.3%)(P>0.05)。结论合并胸腺增生的MG患者TX的术后疗效优于合并胸腺瘤者。     

重症肌无力围手术期处理69例临床分析

本文总结我院2005-01～2009-12收治的69例重症肌无力（MG）患者的围手术期处理体会。     

重症肌无力术围手术期护理体会

重症肌无力是一种由于神经、肌肉接头间传递功能障碍所引起慢性自身免疫性疾病,临床表现为受累横纹肌易于非正常性疲劳,而且休息后可以缓解。胸腺切除治疗重症肌无力是治疗重症肌无力一种有效的方法,但是由于麻醉及手术创伤的刺激,一些患者可能会在围手术期出现重症肌无     

重症肌无力术围手术期护理体会

重症肌无力是一种由于神经、肌肉接头间传递功能障碍所引起慢性自身免疫性疾病,临床表现为受累横纹肌易于“非正常性”疲劳,而且休息后可以缓解。胸腺切除治疗重症肌无力是治疗重症肌无力一种有效的方法,但是由于麻醉及手术创伤的刺激,     

电视胸腔镜下胸腺扩大切除术治疗重症肌无力的围术期管理

重症肌无力(myasthenia gravis,MG)是一种以横纹肌神经肌肉传导障碍为特点的自身免疫性疾病,与胸腺异常关系密切.MG有多种治疗方法[1],其中电视胸腔镜手术目前已得到普及.此文作者2004-10-2008-12成功为51例MG患者进行了电视胸腔镜胸腺扩大切除术并取得较好近期效果,此文对其围术期管理进行报道.     

重症肌无力行胸腺切除术后合并视神经脊髓炎1例

重症肌无力（myasthenia gravis,MG）和视神经脊髓炎（neuromyelitis optica,NMO）均为较少见的神经系统自身免疫性疾病,且两病极少在同一个体先后出现。首都医科大学宣武医院神经内科收治1例重症肌无力患者行胸腺切除术后4个月合并视神经脊髓炎患者,现报道如下。     

Sleep apnoea in well-controlled myasthenia gravis and the effect of thymectomy

We conducted overnight polysomnographic sleep studies of 16 patients (5 men and 11 women) with clinically well-controlled myasthenia gravis (MG). The subtypes of MG were IIA (3 patients), IIB (11 patients), IV (1 patient) and V (1 patient). Twelve patients were found on polysomnography to have obstructive and/or central types of the sleep apnoeas (SA). Their mean age was 42.4, SD 16.4 years, and the mean duration of MG was 7.4, SD 6.96 years. SA was not detected in 4 patients whose mean age was 30.8, SD 10.71 years and who had manifested MG for a mean duration of only 0.9, SD 0.65 years. Thus, patients with a longer duration of MG tended to have more SA. In 9 of the 12 SA patients, polysomnographic studies were repeated following thymectomy. SA had resolved in 6 patients, but persisted in 3. These findings suggest that SA is a possible clinical manifestiation of MG and that nocturnal dysfunction of both peripheral and central colinergic systems may be involved. Received: 10 April 1996 Received in revised form: 12 September 1997 Accpeted: 1 October 1997     

Effects of thymectomy on late-onset myasthenia gravis without thymoma

OBJECTIVES: This study aims to investigate whether thymectomy is beneficial for late-onset (>50 years) myasthenia gravis patients with no thymoma, particularly for those with mild generalized weakness. PATIENTS AND METHODS: A total of 34 patients were included in the study. The clinical course and long-term outcomes over 2 years were reviewed in 20 patients who underwent thymectomy and in 14 without thymectomy. RESULTS: Of the 34 patients, 20 (59%) underwent thymectomy. Thymectomized patients had more severe disability at entry than non-thymectomized patients, but outcome measures did not significantly differ between the two patient groups. Moreover, subgroup analyses including 22 patients with mild generalized weakness at entry showed that the thymectomized group (n=10) showed a greater percentage of clinical remission (no symptoms; 50% versus 17%; p=0.11) and a lower frequency of the presence of generalized symptoms (30% versus 75%; p<0.05) than the non-thymectomized group (n=12) at the end of follow-up (means 9.6 years after onset). CONCLUSIONS: Thymectomy is a potentially effective treatment for late onset, non-thymomatous patients with mild generalized myasthenia gravis.     

Evaluation of results of thymectomy in myasthenia gravis

Summary The results of thymectomy carried out in 150 cases of myasthenia gravis are discussed. In a group of 123 cases followed for 1 to 5 years after the operation, full remission was observed in 24.4% of cases, significant improvement in 36.6%, slight improvement in 24.4% and no improvement in 8.1%, while deterioration occurred in 1.6% of patients. No correlation was found between the result of the operation and the age and sex of patients, but better results were achieved in those treated surgically rather soon after the onset of symptoms. This correlation was particularly evident in the group with full remissions. The results obtained in the cases without thymic tumors were better than in the cases with tumors. No correlation was noted between the results of the operation and the histological characteristics of the thymus in the group with thymic hyperplasia and in the group with thymic atrophy. The surgically treated group (150 cases), compared with the conservatively treated group (75 cases), showed the superiority of the surgical method (lower rate of death and deterioration, higher rate of improvement and remission).In discussing the indications for surgical treatment the authors emphasize that advances in anaesthesiology in recent years have reduced the risk of operation. It is suggested that the indications for surgical treatment should be expanded and operations should be performed as early as possible after the onset of clinical manifestations without regard to the age and sex of the patient. Operation should not be considered in cases belonging to group 1, 2a (sometimes 2b) only, with duration of the disease over 8–10 years and with little or no progression of the process, if the presence of a thymic tumor has been excluded.This work was supported by NIH., N.I.N.D.S.—Bethesda (agreement 05-002-1).     

Anti-acetylcholine receptor antibodies in myasthenia gravis. Part 3. The effect of thymectomy

The clinical condition and the serum levels of antibodies to acetylcholine receptor protein were followed in 30 patients with myasthenia gravis before and in a period varying from 2 to 4 (mean 3) yr after thymectomy. Twenty-five patients improved in the 2 yr following thymectomy. A highly significant correlation was found between the change in clinical condition and the change in antibody levels. Only 3 patients improved without a fall of antibody level. Prethymectomy antibody levels were positively correlated with the severity of the clinical condition and with the degree of thymus hypertrophy.     

胸腺切除术对重症肌无力患者电生理学指标和临床评分的影响

目的评价胸腺切除术对重症肌无力(MG)的近期疗效。方法对15例MG在胸腺切除术前(平均9天)及术后(平均11天)依次进行了临床绝对评分、右侧腋神经低频重复电刺激(RNS)和右侧伸指总肌单纤维肌电图(SFEMG)检查。结果手术后SFEMG和RNS异常率分别为100%和73%,手术后平均颤抖(jitter)值、异常电位对比例、阻滞电位对比例和临床绝对评分都较手术前有显著的降低;病理为胸腺瘤5例,手术前后平均jitter值、阻滞电位对比例和临床绝对评分均无显著差异,胸腺增生9例,手术后阻滞电位对比例和临床绝对评分较术前显著减低。结论MG患者胸腺切除术后在临床表现和电生理学指标上都有一定程度的改善,但此时大多数患者仍有临床症状和电生理学指标的异常,需要进一步的免疫调节治疗;MG并发胸腺瘤者可能手术后近期疗效差,并发胸腺增生者可能疗效相对较好。     

Myasthenia gravis appearing after thymectomy

We aimed to evaluate the clinical characteristics of patients with postoperative myasthenia gravis (MG). We retrospectively studied the data of 174 thymoma patients treated between 1990 and 2008 in Xiangya Hospital. Six of 125 patients without preoperative MG (4.8%) developed postoperative MG. The anti-acetylcholine-receptor binding antibody (ARAb) titers were elevated preoperatively in 22 of the 125 patients (17.6%) who did not have preoperative MG (range, 0.5-67.6 nmol/L). Four of six patients with postoperative MG had positive ARAb levels preoperatively. Serum titers were exacerbated in all six patients at the onset of postoperative MG. Postoperative MG was responsive to anti-cholinesterase compounds and/or steroids. We concluded that a thymectomy did not prevent postoperative MG. Exacerbated ARAb levels after thymectomy suggested an extrathymic production of ARAb. We suggest that a rise in the ARAb titer might be a risk indictor for post-thymectomy MG.     

重症肌无力病人胸腺切除术后血清自身抗体水平变化和临床转归分析

６９例重症肌无力（ＭＧ）病人行胸腺切除术，术前血清乙酰胆碱受体抗体水平均高于正常，术后半年抗体水平明显下降，胸腺增生组（２０例）尤为显著．胸腺瘤组（４９例）术前胸腺瘤相关抗体水平增高，但术后半年内无明显变化．６９例ＭＧ患者胸腺切除术后４年，症状总缓解率为７６．８１％，其中胸腺增生组９５％，胸腺瘤组６９．３９％。作者认为，对于伴有胸腺增生或胸腺瘤的ＭＧ患者，胸腺切除术是有效的，应该列为治疗的第一选择。     

Treatment of myasthenia gravis

Summary In the treatment of myasthenia gravis (MG) considerable progress has recently been achieved. Our experience is based on the observation of 139 patients with an average follow-up of 3 years and 4 months.A treatment plan and results are presented.Indications for thymectomy: all cases of MG in adult life, apart from ocular myasthenia without radiological thymoma and without electrophysiological and pharmacological signs of generalization; before puberty only cases with radiological thymoma and severely incapacitating or life-threatening signs.Median sternotomy is preferable for thymoma, the transcervical approach with a sternal split for non-neoplastic thymus. Mediastinal radiotherapy is indicated after removal of an invasive or adhesive thymoma.Indications for corticosteroids: 1) before thymectomy: respiratory weakness; 2) soon after thymectomy: life-threatening signs; 3) later after thymectomy: incapacitating or life-threatening signs; 4) as an alternative to thymectomy: when surgery cannot be performed or it is not indicated. Oral Prednisone was nearly always preferred: alternate-day high single dose (75 to 115 mg) has given good results in most cases even if in some cases a small dose was required in the off day; inversely a lower alternate-day or daily dose was often sufficient.Long-term results: following this schedule for adult patients good results were scored in 67% of thymomas, in 94% of hyperplasias, and in 62% of unthymectomized patients: in prepuberal life the few cases of severe MG have all shown a favorable evolution.

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Zusammenfassung Bei der Behandlung der Myasthenie wurden in letzter Zeit beachtliche Fortschritte erzielt. Wir teilen hier unsere Erfahrungen anhand von 139 Patienten mit einer durchschnittlichen Katamnese von 3 Jahren und 4 Monaten mit.Wir betrachten als Indikationen für eine Thymektomie: alle Fälle von Myasthenie beim Erwachsenen mit Ausnahme der rein okulären Formen ohne radiologisch nachweisbares Thymom und ohne elektrophysiologische oder pharmakologische Zeichen einer Generalisierung; bei Kindern vor der Pubertät empfehlen wir die Thymektomie nur in Fällen mit radiologisch nachweisbarem Thymom und mit schwerer Beeinträchtigung oder gar Lebensgefährdung durch die Symptome.Die mediane Sternotomie ist beim Thymom vorzuziehen, der transzervikale Zugang mit Spaltung des Sternums für die nicht neoplastischen Thymusvergrößerungen. Mediastinale Strahlentherapie ist nach Exstirpation eines invasiven Thymoms oder eines Thymoms mit Adhäsionen angezeigt.Als Indikation für die Corticosteroidtherapie betrachten wir: 1. wenn vor der Thymektomie Atemstörungen bestehen; 2. wenn bald nach der Thymektomie lebensbedrohliche Symptome auftreten; 3. wenn später nach Thymektomie nennenswert behindernde oder lebensbedrohliche Symptome in Erscheinung treten; 4. als Alternative zur Thymektomie, wenn diese nicht durchgeführt werden kann oder nicht indiziert ist. Die orale Prednisontherapie wurde fast immer vorgezogen: wir gaben an alternierenden Tagen jeweils hohe Einzeldosen (75–115 mg) mit gutem Erfolg in den meisten Fällen. In gewissen Fällen war eine kleine Dosis an den Tagen zwischen der Hauptdose aber genügend, ebenso in Einzelfällen eine allgemein niedrigere Dosierung. Die Langzeiterfolge mit diesem therapeutischen Vorgehen betrugen 67% gute Ergebnisse bei Thymomen und 94% bei Thymushyperplasie. Unter den nicht thymektomierten Patienten wiesen 62% ein gutes Ergebnis auf. Vor der Pubertät zeigten die allerdings wenigen Fälle schwerer Myasthenie alle ein gutes Ansprechen auf die Therapie.

     

Thymectomy in the treatment of myasthenia gravis: report of 247 patients

Summary We made a retrospective assessment of the long-term outcome in 247 consecutive patients with myasthenia gravis (MG) who underwent thymectomy in the period January 1971–December 1985. In 84 cases a thymoma was found at surgery, while 163 patients had a non-neoplastic thymus. The duration of symptoms before surgery, the age at onset of the disease and the presence of germinal centres in the thymus did not appear to influence the prognosis. Patients with a non-neoplastic thymus showed a better response to thymectomy. Thymoma was associated with more severe disease and with a higher mortality; moreover, more thymoma patients required corticosteroid treatment in order to achieve good therapeutic results. In our opinion, thymectomy is indicated in the treatment of generalized MG, while ocular myasthenia seems not to be improved by the removal of the thymus.