Bulkley G B, Bass K N, Stephenson G R, Diener-West M, George S, Reilly P A, Baker R R, Drachman D B
Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Ann Surg. 1997 Sep;226(3):324-34; discussion 334-5. doi: 10.1097/00000658-199709000-00012.
The authors evaluated the response to extended cervicomediastinal thymectomy as a component of the integrated management of patients with myasthenia gravis in a large series of patients from a single institution. The authors evaluated the response to therapy with respect to a graded, multivariate, ordinal scale chosen to reflect the full range of the disease's manifestations.
A number of series, of varying sizes, describe the response of patients with myasthenia gravis to thymectomy primarily with respect to the bivariate endpoint of the presence or absence of "remission." These studies fail to describe the full spectrum of postoperative disease severity and have been unable to quantify definitively the influence of putative prognostic variables, nor to assess definitively the statistical significance of apparent improvements over time.
The authors evaluated 202 consecutive patients who underwent trans-sternal thymectomy for symptomatic myasthenia gravis from 1969 through 1996 at the Johns Hopkins Hospital. The response to surgery, combined with postoperative medical therapy, was evaluated by a standardized scale reflecting the full spectrum of the disease. These data were analyzed by a novel mean multivariate regression analysis, which allowed the quantification of the statistical significance of apparent responses over time and the evaluation of the independent influence of each of nine putative prognostic indicators.
There was no perioperative mortality and a 33% perioperative morbidity. There was a marked clinical response at 6 months to 1 year after surgery that was sustained for at least 10 years thereafter. The median increment of improvement was two (of five) classes. Eighty-six percent and 83% of the patients had improved by at least one class at 5 and 10 years, respectively. These changes were statistically significant (p < 0.001). Whereas the use of extended cervicomediastinal thymectomy was associated with a greater than twofold chance of improvement, compared to conventional trans-sternal thymectomy, neither the pathologic diagnosis (presence of thymoma) nor the age at surgery proved to be negative predictors of postoperative response.
Extended cervicomediastinal thymectomy is the procedure of choice as a component of the integrated management of myasthenia gravis, with significant improvement seen in the group as a whole, as well as in subgroups of patients with thymoma and those older than 40 years of age.
作者评估了扩大的颈纵隔胸腺切除术作为单一机构中大量重症肌无力患者综合治疗组成部分的疗效。作者根据一个分级、多变量、有序量表评估了治疗反应，该量表旨在反映疾病的全部表现范围。
许多规模各异的系列研究主要就“缓解”与否这一双变量终点描述了重症肌无力患者对胸腺切除术的反应。这些研究未能描述术后疾病严重程度的全貌，也无法明确量化假定的预后变量的影响，亦无法确切评估随时间推移明显改善的统计学意义。
作者评估了1969年至1996年在约翰霍普金斯医院因症状性重症肌无力接受经胸骨胸腺切除术的202例连续患者。通过反映疾病全貌的标准化量表评估手术联合术后药物治疗的反应。这些数据通过一种新颖的均值多变量回归分析进行分析，该分析能够量化随时间推移明显反应的统计学意义，并评估九个假定预后指标各自的独立影响。
围手术期无死亡病例，围手术期发病率为33%。术后6个月至1年有明显的临床反应，此后至少持续10年。改善的中位数增量为两级（共五级）。分别有86%和83%的患者在术后5年和10年至少改善了一级。这些变化具有统计学意义（p < 0.001）。与传统经胸骨胸腺切除术相比，扩大的颈纵隔胸腺切除术使改善的几率增加了两倍多，然而病理诊断（胸腺瘤的存在）和手术年龄均未被证明是术后反应的负面预测因素。
扩大的颈纵隔胸腺切除术是重症肌无力综合治疗的首选术式，在整个患者群体以及胸腺瘤患者亚组和40岁以上患者亚组中均可见显著改善。
