Department of Thoracic Surgery, Multidisciplinary Myasthenia Gravis Unit, Tor Vergata University, Rome, Italy.
J Thorac Cardiovasc Surg. 2012 Mar;143(3):601-6. doi: 10.1016/j.jtcvs.2011.04.050. Epub 2011 Dec 17.
The presence of ectopic thymic tissue has been considered one of the most significant predictors of poor outcome after thymectomy for myasthenia gravis, but the role of active ectopic tissue is unknown. The current study analyzed the importance of this factor on post-thymectomy outcome of patients with class III myasthenia gravis.
We retrospectively reviewed 106 patients with class III, anti-acetylcholine receptor antibody-positive, nonthymomatous myasthenia gravis (70 female, 36 male; mean age, 41 ± 17 years) who underwent transsternal extended thymectomy between 1980 and 2005. Quality of life was assessed from 1996 with the Short Form 36 questionnaire. Prognosticators were investigated using complete stable remission and normalized component summaries as end points.
Major morbidity rate was 5% with no perioperative mortality. Ectopic thymic tissue was detected in 51 patients (48%), 34 of whom (67%) presented germinal centers. Complete follow-up was available in 96 patients (mean 160 ± 91 months). Fifty-two patients (54%) achieved complete stable remission, and 20 patients (21%) presented clinical and pharmacologic improvement. Lack of postoperative improvement in physical and psychosocial domains was significantly correlated with active ectopic thymus. At Kaplan-Meier evaluation, duration of symptoms (>12 months) (P = .04), oropharyngeal involvement (P = .02), germinal centers (P = .03), ectopic thymus (P = .001), and active ectopic thymus (P < .0001) were negative predictors of complete stable remission. The presence of active ectopic thymus was the most significant negative predictor of complete stable remission at Cox regression (P = .03).
Extended thymectomy yields good outcome in patients with nonthymomatous class III myasthenia gravis. The presence of active ectopic thymus was the most significant predictor of poor outcome. These patients should be rigorously followed and undergo early aggressive therapy.
胸腺瘤外的胸腺组织的存在被认为是重症肌无力（MG）胸腺切除术后预后不良的最重要预测因素之一，但活性异位组织的作用尚不清楚。本研究分析了这一因素对 III 类 MG 患者胸腺切除术后的重要性。
我们回顾性分析了 1980 年至 2005 年间接受胸骨正中扩大胸腺切除术的 106 例 III 类、抗乙酰胆碱受体抗体阳性、非胸腺瘤性重症肌无力患者（70 例女性，36 例男性；平均年龄 41 ± 17 岁）。1996 年以来，采用 36 项简明健康调查问卷（Short Form 36 questionnaire）评估生活质量。以完全稳定缓解和正常化的成分总结作为终点，用预后分析来检测预测因子。
主要并发症发生率为 5%，无围手术期死亡。51 例患者（48%）检测到胸外胸腺组织，其中 34 例（67%）存在生发中心。96 例患者（平均随访 160 ± 91 个月）可进行完整随访。52 例（54%）患者达到完全稳定缓解，20 例（21%）患者出现临床和药物改善。术后物理和社会心理领域无改善与活性异位胸腺显著相关。在 Kaplan-Meier 评估中，症状持续时间（>12 个月）（P = 0.04）、口咽受累（P = 0.02）、生发中心（P = 0.03）、胸外胸腺（P = 0.001）和活性异位胸腺（P < 0.0001）是完全稳定缓解的阴性预测因子。Cox 回归分析显示，活性异位胸腺是完全稳定缓解的最显著阴性预测因子（P = 0.03）。
非胸腺瘤性 III 类重症肌无力患者行扩大胸腺切除术可获得良好的疗效。活性异位胸腺的存在是预后不良的最显著预测因子。这些患者应严格随访，并进行早期积极治疗。
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