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胸腺瘤切除术后难治性重症肌无力,发病时处于 MGFA 分期 V:病例报告。

Refractory post-thymectomy myasthenia gravis with onset at MGFA stage V: a case report.

机构信息

Department of Cardiothoracic Surgery, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.

出版信息

J Cardiothorac Surg. 2022 May 12;17(1):115. doi: 10.1186/s13019-022-01872-0.

Abstract

BACKGROUND

Post-thymectomy myasthenia gravis (PTMG) is defined as thymoma patients without signs of myasthenia gravis (MG) pre-operation, but develop MG after radical surgical resection. PTMG might be misdiagnosed not only because of its rare incidence, but also the uncertain interval between the removal of thymoma and the new onset MG. Additionally, some surgeons and anesthesiologists pay less attention to those asymptomatic thymoma patients in perioperative management, leading to the neglect of new onset PTMG, and miss the best time to treat it.

CASE PRESENTATION

Majority of cases of PTMG with onset at stage I-II on the basis of Myasthenia Gravis Foundation of America (MGFA) classification have been reported, but rarely at stage V, which requiring intubation or non-invasive ventilation to avoid intubation. Herein, we presented a 70-year-old male with PTMG onset at MGFA stage V, meanwhile, he had severe pulmonary infection interfering with the diagnosis of PTMG, and eventually progressed to refractory PTMG, which requiring much more expensive treatments and longer hospital stays.

CONCLUSION

In the perioperative management of asymptomatic thymoma patients, careful preoperative evaluation including physical examination, electrophysiological test and acetylcholine receptor antibodies (AChR-Ab) level should be done to identify subclinical MG. Complete resection should be performed during thymectomy, if not, additional postoperative adjuvant therapy is neccessary to avoid recurrence. It's important to identify PTMG at a early stage, especially when being interfered with by postoperative complications, such as lung infection, so that treatments could be initiated as soon as possible to avoid developing to refractory PTMG.

摘要

背景

胸腺瘤切除术后重症肌无力(PTMG)定义为术前无重症肌无力(MG)征象的胸腺瘤患者,但在根治性外科切除术后发生 MG。PTMG 不仅由于其罕见的发病率,而且由于胸腺瘤切除与新发 MG 之间的不确定间隔,可能会被误诊。此外,一些外科医生和麻醉师在围手术期管理中对无症状胸腺瘤患者关注较少,导致对新发 PTMG 的忽视,并错过治疗的最佳时机。

病例介绍

大多数 PTMG 病例在 MGFA 分类的 I-II 期发病,但很少在 V 期发病,需要插管或无创通气以避免插管。在此,我们报告了一例 70 岁男性,新发 PTMG 为 MGFA V 期,同时伴有严重肺部感染,干扰了 PTMG 的诊断,最终进展为难治性 PTMG,需要更昂贵的治疗和更长的住院时间。

结论

在无症状胸腺瘤患者的围手术期管理中,应进行仔细的术前评估,包括体格检查、电生理检查和乙酰胆碱受体抗体(AChR-Ab)水平,以识别亚临床 MG。如果未完全切除胸腺瘤,应在术后进行辅助治疗,以避免复发。重要的是要在早期识别 PTMG,特别是在术后并发症(如肺部感染)干扰的情况下,以便尽快开始治疗,避免发展为难治性 PTMG。

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