老年及老年前期重症肌无力的手术治疗(附23例报告)

目的总结胸腺切除术治疗老年及老年前期重症肌无力的临床效果。方法对1981年～2001年在我院行胸腺切除治疗的23例老年及老年前期重症肌无力患者的手术疗效进行回顾分析。结果在所有重症肌无力患者中老年及老年前期患者所占比例小,男性为多见;全身型为主,合并胸腺瘤及危象者多;手术总有效率43.5％。结论老年及老年前期重症肌无力的手术疗效较青壮年为差,但仍是可选择的一种重要方法。     

Epidemiological study of myasthenia gravis in Sardinia, Italy (1958–1986)

From 1.1.1958 to 31.12.1986, 110 cases of MG were observed in Sardinia, with a mean annual incidence of 2.5 x 1,000,000 inhabitants and prevalence rates of 7.5, 17.6, 31.4 and 45.0 x 1,000,000 inhabitants respectively (prevalence days: 15.10.1961, 24.10.1971, 25.10.1981 and 31.12.1986). The disease was found to be more frequent in women. There were no differences in the distribution of MG in various areas of the island. The muscle group more frequently involved at onset was the ocular. In 6.4% of patients an association with thyroid disorders was observed. The mortality of MG patients was significantly higher than expected. Removal of the thymus, carried out in 58 patients, was shown to be useful in the treatment of the disease, particularly in patients without thymomas. No familial cases were observed.     

Exacerbation of myasthenia gravis after removal of thymomas

It appeared from this prospective and non-randomized study, that the removal of thymomas in myasthenia gravis (MG) patients resulted in rapid exacerbation of the clinical severity of the disease and of anti-acetylcholine receptor antibodies titres, which peaked after about 300 days and continued for up to 2 years. Long-term follow-up after thymomectomy (mean duration ± SEM after surgery 5.5 ± 0.8 years) showed that the immunological and clinical state observed prior to surgery was eventually restored, but long-term benefit attributable to surgery could not be demonstrated. Non-thymoma MG cases, however, exhibited post-operative amelioration in clinical course and decreasing antibodies titres, both of which were already significant one year after surgery, and additional improvement was observed at the time of long-term follow-up (mean 4.3 ± 0.5 years). Furthermore, the prognosis for MG patients not operated on was also favourable. It is suggested that the occurrence of thymomas is linked to genetic factors and that neoplasia of the thymus may be part of immunoregulatory mechanisms with predominance of inhibition.     

Effects of surgical treatment in thymoma with myasthenia gravis: our experience in 103 patients.

A retrospective study of 103 thymectomies examines the effects of the integration of surgical and medical therapy in patients affected by myasthenia gravis accompanied by thymoma. An extended thymectomy via a median longitudinal sternotomy was used in 102 patients. The operative mortality was 4.85% (5/103 patients), the 10-year survival rate was 78% with a recurrence rate of 3.06% (3/98). Neoplastic infiltration and postoperative radiotherapy did not influence the survival rate. There was no correlation between a preoperative Osserman's class better than III and postoperative outcome. The improvement of medical treatment, and anaesthesiological and intensive care techniques resulted in a decrease of the operative mortality and long-term death rate during the last 10 years of our 20-year series. Extended thymectomy via sternotomy is the best intervention in patients with myasthenia gravis associated with thymoma judged by the low operative mortality and the favorable 10-year survival rates.     

Antibodies to acetylcholine receptors in myasthenia gravis. In vitro synthesis by peripheral blood lymphocytes before and after thymectomy.

Pokeweed mitogen (PWM)-driven in vitro synthesis of antibodies to the acetylcholine receptor (PSA) was studied in non-thymoma patients with myasthenia gravis. In a group of 46 patients, the occurrence of PSA was related to the presence of the thymus or, in operated patients, the absence of a clinical effect of thymectomy. Sixteen patients were followed before and soon after thymectomy. PSA disappeared in all patients, at least temporarily, between 6 weeks and 1 year afterwards, independent of the clinical course and eventual clinical effect of the operation. A recurrence was found only in one of the five patients who derived no benefit from the operation. These findings support the hypothesis that the therapeutic effect of thymectomy can be explained by removal of a source of autoreactive lymphocytes. There was no correlation between the changes in serum levels of a-AChR and clinical improvement, suggesting a minor role of circulating peripheral blood lymphocytes (PBL) and the thymus in the total production of a-AChR.     

胸腺切除治疗重症肌无力远期疗效及影响因素分析

目的 报道胸腺切除治疗重症肌无力的远期疗效并对影响远期效果的若干因素进行多因素分析。方法 回顾1995年1月至1999年12月行胸腺切除的154例重症肌无力患者的病史资料及随访结果，通过logistic回归对性别、年龄、术前病程、改良Osserman分型、Besinger评分、胸腺病理、术前激素用量、AchRab和PsmRab水平等因素对术后疗效的影响进行多因素分析。结果 中位随访期为7．3（5．3—10．0）年。2例患者在随访期内死亡。152例存活患者在末次随访时有12例（7．9％）达到完全缓解，116例（76．3％）达到部分缓解，12例（7．9％）病情稳定，12例（7．9％）病情加重，总有效率为84．2％。Logistic回归分析表明Besinger评分（回归系数=0．057，P=0．020）和胸腺病理（回归系数=-1．559，P=0．012）与远期疗效显著相关，Besinger评分较高或不伴有胸腺瘤的患者预后较好。结论 胸腺切除是治疗重症肌无力的有效手段，Besinger评分和胸腺病理是远期疗效的独立预后因素，Besinger评分较高或不伴有胸腺瘤的患者预后较好。改良Osserman分型与预后的关系需要进一步研究。     

VATS胸腺扩大切除术治疗重症肌无力临床分析

目的评价电视胸腔镜(video-assistant thorascope,VATS)下胸腺扩大切除术治疗重症肌无力(myasthenia gravis,MG)的手术方法和效果。方法回顾性分析36例MG患者的临床资料,其中合并胸腺增生26例,胸腺瘤10例,均行VATS胸腺瘤、胸腺扩大切除术。根据临床Osserman分型:Ⅰ型3例,Ⅱa型22例,Ⅱb型8例,Ⅲ型3例。结果本组手术全部经胸腔镜完成,无中转开胸者。全组手术时间平均80(50~120)min,术中出血量平均80(10~150)ml,留置胸腔引流管1~3 d,引流量平均150(100~500)ml,6例未留置胸管者术后3 d经CT扫描估计胸液量均<200 ml。住院时间3~6 d,术后未出现进行性血胸,无肺及膈神经损伤,无肌无力危象、胆碱能危象发生,无围手术期死亡病例。术后病理:胸腺增生26例,A型胸腺瘤6例,B1型2例,B3型2例(WHO分型)。所有患者随访3~12个月,完全缓解6例(Ⅰ型2例、Ⅱa型4例),占16.7%;部分缓解28例(Ⅰ型1例、Ⅱa型18例、Ⅱb型7例、Ⅲ型2例),占77.8%;稳定2例(Ⅱb型1例、Ⅲ型1例),占5.6%。结论 MG患者行VATS胸腺瘤、胸腺扩大切除术安全、可行,效果理想。     

单孔与三孔剑突下胸腔镜全胸腺切除术的回顾性队列研究

目的比较单孔与三孔剑突下胸腔镜(subxiphoid video-assisted thoracoscopic surgery,XVATS)全胸腺切除术的临床特点。方法回顾性分析2017年1月至2019年5月在徐州市中心医院连续开展的60例XVATS全胸腺切除术患者的临床资料,其中男29例、女31例,年龄53.1(27.0~76.0)岁。依据手术方式不同分为单孔XVATS组(30例)与三孔XVATS组(30例)。比较两组临床效果。结果两组患者年龄、性别、体重指数、肿瘤长径、术中出血量、术后胸腔引流管留置时间、胸腔引流量与术后住院时间等差异无统计学意义(P均>0.05)。全组无围手术期死亡、中转开胸、血栓或纵隔感染病例。单孔XVATS组患者手术时间显著长于三孔XVATS组,差异有统计学意义[(87.5±19.0)min vs.(75.8±15.7)min,P=0.012];此外,单孔组术后3~14 d疼痛评分显著低于三孔XVATS组(P=0.001)。结论单孔XVATS全胸腺切除术是可行的,患者术后疼痛较三孔组患者轻,但开展初期手术时间较长。     

延髓型重症肌无力喉肌电指标变化的临床价值

目的探讨延髓型重症肌无力患者电视胸腔镜下胸腺扩大切除术（video-assisted thoracoscopic extended thymectomy,VATET）后喉肌电指标测定的临床价值。方法回顾分析2002年6月～2011年7月我科36例延髓型重症肌无力（延髓型组）行VATET的临床资料,对术前后喉肌电指标进行分析,选取同期36例健康受试者作为对照组。结果延髓型组术前喉内肌肌电振幅（210．68±75．42）μV,较对照组（309．40±55．33）μV明显缩窄（t=6．332,P=0．000）;时程（7．68±0．57）ms较对照（5．42±0．34）mB明显延长（t=-20．431,P=0．000）;募集相最大电位（1132．18±467．19）μV较对照组（1675．00±532．85）μV明显缩窄（t=4．596,P=0．000）,术后2组比较仅时程有明显差异（t=6．375,P=0．000）。延髓型组喉内肌群术后肌电振幅（312．91±69．43）μV,较术前（210．68±75．42）μV明显变宽（t=-5．983,P=0．000）;术后时程（6．19±0．64）m8较术前（7．68±0．57）m8明显缩短（t=10．431,P=0．000）;术后募集相最大电位（1557．67±521．45）斗V较术前（1132．18±467．1）μV明显变宽（t=-3．646,P=0．000）;喉外肌仅肌电振幅差异显著[（139．36±74．26）μV vs．（102．75±63．22）μV,t=-2．252,P=0．027]。术后喉肌电图正常的患者疗效有效率89．3％（25／28）,喉肌电图异常的患者疗效有效率37．5％（3／8）,2组比较有统计学差异（χ2=6．891,P=0．009）。结论延髓型重症肌无力患者肌电指标异常,VATET可有效改善各项指标,缓解相关的临床症状。