胸腺切除治疗重症肌无力的围手术期处理

我院 198 8年 1月～ 2 0 0 0年 12月对 61例重症肌无力患者进行了胸腺切除术及围术期处理 ,疗效较好 ,报告如下。临床姿料一、一般资料 :男性 3 7例 ,女性 2 4例 ;年龄 <15岁 8例 ,15～ 3 0岁 11例 ,3 0～ 45岁 16例 ,45～ 60岁 19例 ,>60岁 17例。分型 :眼肌型 49例 ,延髓肌型     

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。     

女性重症肌无力患者的外科治疗与围术期处理

目的总结女性重症肌无力(MG)患者的外科治疗及围术期处理经验。方法回顾性分析1979年1月至2007年12月收治的186例(包括川北医学院附属医院胸心血管外科41例,南方医科大学附属南方医院胸心血管外科145例)MG女性患者的临床资料,年龄5~64岁,平均年龄43.9岁。按Osserman临床分型,小儿MG45例,成人MG141例,其中眼肌型25例,轻度全身型63例,中度全身型29例,急性暴发型19例,晚期严重型5例。合并胸腺瘤166例,按Masaoka分期I期40例,Ⅱ期69例,Ⅲ期44例,Ⅳ期13例。152例患者经胸骨正中切口径路手术,行胸腺切除,纵隔淋巴结清扫术;34例胸腺无明显增生经颈部横切口行单纯胸腺切除术。结果全组无手术死亡。围术期发生MG危象22例,经相应的处理治愈。术后随访12个月以上165例,失访21例。痊愈30例(18.18%)、基本痊愈28例(16.97%)、显效60例(36.36%)、好转25例(15.15%)、无效22例(13.33%)。Ⅰ型、Ⅱa型、Ⅱb型、Ⅲ型和Ⅳ型有效率分别为100.00%、93.10%、90.00%、77.27%和53.85%。结论女性MG患者经胸腺切除术治疗效果良好;加强围术期处理,合理使用抗胆碱酯酶药物和激素,可减少各种MG危象的发生。     

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。     

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。     

310例重症肌无力的外科治疗

目的 评价胸腺切除术治疗重症肌无力的围手术期临床特点及疗效。方法 回顾性分析310例经胸腺切除术治疗重症肌无力患者的临床特点、手术方式、围手术期处理及疗效。以临床绝对评分及相对评分为治疗前后疗效主要判断标准。结果 全组无手术死亡,直接手术并发症8．7％(27／310)。术后病理检查,异位胸腺发生率38．7%,(120／310),胸腺增生达92．9％(288／310)。术后随访3个月以上者287例(随访率为92．6％),痊愈7．1％(22／310)、基本痊愈11．3％(35／310)、显效40．0％(124／310)、好转27．1％(84／310)、无效7．1％(22／310),总有效率85．5％(265／310)。其中随诊病例中Ⅰ型、Ⅱa型、Ⅱb型、Ⅲ型、Ⅳ型有效率分别为90．9％(20／22)、97．6％(40／41)、95．3％(162／170)、80．6％(29／36)和77．8％(14／18)。结论 全身型及部分眼肌型重症肌无力患者可首选扩大胸腺切除术治疗,疗效良好。     

重症肌无力外科治疗围术期的处理经验

重症肌无力 (ＭＧ)手术治疗时 ,围术期并发症多 ,甚至还可出现危象。我们在围术期处理等方面作了改进 ,1992年 3月至 1999年 3月外科治疗ＭＧ 37例 ,疗效好 ,无并发症 ,现总结报告如下。临床资料　本组 37例中男 19例 ,女 18例。年龄 2 6～ 5 8岁。病程 6～ 6 0个月。按Ｍｏｎｄｅｎ改良的Ｏｓｓｅｒｍａｎ分型〔1〕:Ｉ型 4例 ,ＩＩａ型 9例 ,ＩＩｂ型 17例 ,ＩＩＩ型 3例 ,ＩＶ型 4例。其中2例伴甲亢者 ,均为ＩＩｂ型 ,于ＭＧ手术后 2个月行甲状腺次全切除术 ;2 5例伴胸腺增生 ;12例伴胸腺瘤 ,按Ｍｏｎｄｅｎ肿瘤临床分期〔1〕:Ｉ期 7例 ,…     

重症肌无力的外科治疗

1986年10月～2004年1月，我科共收治重症肌无力病人37例，其中32例行手术治疗。我们体会胸腺及前纵隔脂肪组织清除术，围手术期处理，营养支持等综合治疗，是提高手术成功率的重要因素。     

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。     

免疫吸附疗法治疗晚发型重症肌无力

目的 观察蛋白A免疫吸附（IA）治疗对晚发型重症肌无力（MG）相关抗体的清除效果及短期临床疗效。 方法 随机选取25例晚发型MG患者，其中10例MG患者接受IA治疗，15例接受丙种球蛋白（0.4 g·kg-1·d-1）冲击治疗5 d。观察两组治疗前后血清中特异性标志物连接素抗体（Titin-ab）、抗乙酰胆碱受体抗体（AchR-ab）、突触前膜抗体（PrsmR-ab）的变化，同时测定治疗前后定量重症肌无力（QMG）评分。比较两组患者治疗有效率、临床症状缓解时间、呼吸机使用人数和平均住院天数的差异，并分析3种抗体水平的下降和QMG评分改善的相关性。 结果 与治疗前比较，治疗后免疫吸附组和丙种球蛋白组的Titin-ab（P/N值）、AchR-ab（P/N值）、PrsmR-ab（P/N值）均显著下降（均P < 0.05）。其中免疫吸附组的Titin-ab下降幅度显著大于丙种球蛋白组（54.7%±3.5%比19.9%±3.1%，P < 0.05）。免疫吸附组的QMG评分下降幅度显著大于丙种球蛋白组（42.4%±4.2%比23.8%±3.7%，P < 0.05）。免疫吸附组的治疗有效率显著高于丙种球蛋白组（70%比40%，P < 0.05）；临床症状开始缓解的时间也明显缩短[（5.38±0.42） d 比 （8.40±1.54） d，P < 0.01]；呼吸机使用人数（1/10 比 6/15，P < 0.05）和平均住院天数[（13.50±0.50） d比(16.00±0.50) d，P < 0.05]均低于丙种球蛋白组。相关分析显示Titin-ab的下降幅度和QMG评分下降呈正相关（r = 0.6315，P < 0.01）。 结论 免疫吸附疗法能快速有效清除晚发型重症肌无力患者体内的致病抗体，短期疗效优于丙种球蛋白。     

Role of thymectomy in the surgical treatment of myasthenia gravis

Of the 26 patients with myasthenia gravis undergone thymectomy, 11 cases had either benign or malignant thymoma as judged not only by hitological examination but also by their clinical and operative findings. Age of initial onset ranged from 13 to 64 years old. Fifteen out of 26 (58 per cent) benefited from thymectomy. Duration of the symptom from the onset to the operation and the presence or absence of the thymoma are not related to their outcome. Benign or malignant nature of thymoma should not be determined by histological examination alone but by combined evaluation of clinical and operative findings. Serial studies of serum immunoglobulin levels before and after thymectomy suggested that this disorder could be associated with humoral antibody (IgG). HLA typing of the patients with myasthenia gravis did not indicate the presence of any specific antigens.     

Steroid therapy without primary dose escalation for postthymectomy crisis in 2 thymomatous myasthenia gravis patients

We report 2 cases of thymomatous myasthenia gravis associated with postoperative crisis and medicated with steroid therapy using prednisolone without primary dose escalation. Two women, a 38 years old and the other 64 years old, underwent extended thymectomy under the diagnosis of myasthenia gravis associated with invasive thymoma. Bulbar symptoms in both were severe despite preoperative anticholinesterase medication. Myasthenic crises with an antiacetylcholine receptor antibody (anti-AchR Ab) elevation were encountered in the postoperative clinical course. Daily administrations of a large amount of prednisolone without primary dose escalation and the subsequent tapering therapy effectively improved myasthenic symptoms and decreased their anti-AchR Ab titer. We also discuss difficulties in treatment in these cases.     

Suxamethonium in myasthenia gravis

The effects of suxamethonium 1.0 and 0.5 mg/kg were studied in myasthenic patients. Resistance was not encountered at the 1.0 mg/kg dose but was seen at the lower dose. A non-depolarising type of block developed in patients in both groups. However, recovery from the non-depolarising block was not prolonged beyond the requirements of surgery. The neurophysiological basis for, and clinical implications of these findings are discussed.     

Sevoflurane as a sole anaesthetic for thymectomy in myasthenia gravis

Myasthenia gravis is a challenging situation for anaesthesiologists due to its neuromuscular involvement. The main concerns are respiratory muscle weakness and side effects due to a heavy dose of anticholinesterases. This limits the use of sedatives, hypnotics and muscle relaxants. Inhalational anaesthetics are best suited. We describe our experience with sevoflurane as a sole anaesthetic in a child having juvenile-type myasthenia gravis with thymoma, who underwent thymectomy by midsternal incision. Very smooth and short duration of induction (35 s) and easy intubation within 60 s without use of muscle relaxant were the remarkable features. Sevoflurane in oxygen and nitrous oxide (MAC=0.5-0.7) was used for maintenance of anaesthesia. Recovery was smooth and fast with no residual respiratory insufficiency. Hence we found sevoflurane to be a highly suitable agent for thymectomy in mysthenia gravis.     

Maternal myasthenia gravis: a cause for arthrogryposis multiplex congenita

Journal of Children's Orthopaedics - Arthrogryposis multiplex congenita (AMC) is a condition defined as contractures in more than two joints and in multiple body areas. The principal mechanism...     

Favorable results of thymectomy combined with prednisolone alternate-day administration in myasthenia gravis

Administration of anti-cholinesterase before operation and high doses of prednisolone after the operation are commonly prescribed in cases of thymectomy for the treatment of myasthenia gravis. Our policy is to carry out an extended thymectomy combined with pre- and post-operative administration of prednisolone on alternate days. Our method was used in the treatment of 50 patients with generalized myasthenia gravis, from January 1979 to December 1984. The results were examined in conjunction with the necessity for preoperative administration of an anti-cholinesterase preparation and also with factors influencing the follow-up result and postoperative process. This method made it feasible to perform surgery under conditions of stabilization of clinical symptoms and satisfactory postoperative management. A postoperative period of unstable symptoms could thus be avoided. The palliation rate at one year after the operation was 96 per cent, while the remission rate at 5 years after the operation was 70 per cent. The preoperative supplement of anti-cholinesterase and preoperative per cent forced vital capacity were considered to be predictive factors concerning the necessity of artificial respiration.     

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。     

Measurement of antiacetylcholine receptor antibody in patients with thymoma without myasthenia gravis complications

OBJECTIVE: Some patients with thymoma reported to show higher antiacetylcholine receptor antibody titers without the preoperative occurrence of myasthenia gravis and some have suffered postoperative complications of myasthenia gravis despite being negative for antiacetylcholine receptor antibody preoperatively. We evaluated changes in antiacetylcholine receptor antibody titers and the occurrence of myasthenia gravis in thymoma patients. METHODS: Subjects were 31 of 44 patients with thymoma undergoing thymothymectomy at Tokyo Women's Medical University Hospital between 1987 to 1999 in whom antiacetylcholine receptor antibody titers were measured preoperatively. We studied postoperative changes in antiacetylcholine receptor antibody titers and the presence or absence of myasthenia gravis. RESULTS: Eight patients were positive for antiacetylcholine receptor antibody preoperatively, suggesting the presence of subclinical myasthenia gravis. Neither postoperative changes in antiacetylcholine receptor antibody titers nor the occurrence of myasthenia gravis was observed in these 8 patients. Recurrent thymoma and rapid elevation of antiacetylcholine receptor antibody titers were observed postoperatively in 1 patient negative for antiacetylcholine receptor antibody preoperatively, resulting in manifestation of myasthenia gravis symptoms. CONCLUSION: We found no correlation between preoperative titers and myasthenia gravis symptoms. Rapid titer elevation indicates the occurrence of myasthenia gravis symptoms or the recurrence of thymoma.