Aftabuddin M, Bhandari S
Professor Dr Md Aftabuddin, Professor & Chairman, Department of Cardiac Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.
Mymensingh Med J. 2016 Jul;25(3):536-41.
Myasthenic crisis is a life-threatening condition. We studied the demographic, frequency, causes and clinical presentation of isolated Myasthenic crisis, steps of treatment and to review our experience of extended thymectomy on patients with at least one episode myasthenic crisis. A prospective and retrospective study was conducted on patients with at least one episode of myasthenic crisis, from March 2010 to September 2014, at the Department of Cardiac Surgery, BSMMU, Dhaka, Bangladesh who were referred for thymectomy. Eighteen patients (13.6% of the total 132 patients with myasthenia gravis were admitted with single to multiple episodes of myasthenic crisis, median crisis was 2.5 episodes. Mean age of the patient was 35.5 (18-72) years with male predominance. All eighteen patients had undergone extended thymectomy after completion of 5 cycle plasmapheresis, of which 2 had experienced postoperative respiratory crisis, required invasive ventilator support for median 14 days. One patient required invasive ventilator support after third post operative day. Six patients had thymoma and 12 had thymic hyperplasia. Three patients needed Intravenous immunoglobin. Nine patients needed post operative anti acetylcholinesterase inhibitor after median 2.5 post days. Post thymectomy remission and decreases the frequency of myasthenic crisis was seen in follow up and post operative medication requirement reduced significantly as compared to the preoperative requirement. This report highlights that the patients who had extended thymectomy after episodes of myasthenia crisis are benefitted even in the histhopathology report does not confirmed thymoma. After thymectomy, there was remission of myasthenic crisis. Patients with myasthenic crisis should have judicious drug adjustments under supervision and should be treated aggressively during impending myasthenic crisis. With modern management of myasthenia gravis, early surgery with myasthenic crisis is safe with good long-term outcomes.
重症肌无力危象是一种危及生命的状况。我们研究了孤立性重症肌无力危象的人口统计学特征、发生率、病因及临床表现、治疗步骤，并回顾了我们对至少经历过一次重症肌无力危象的患者进行扩大胸腺切除术的经验。对2010年3月至2014年9月期间在孟加拉国达卡BSMMU心脏外科因胸腺切除术而转诊的至少经历过一次重症肌无力危象的患者进行了一项前瞻性和回顾性研究。18例患者（占132例重症肌无力患者总数的13.6%）因单次至多次重症肌无力危象入院，中位危象次数为2.5次。患者的平均年龄为35.5岁（18 - 72岁），男性居多。所有18例患者在完成5次血浆置换后均接受了扩大胸腺切除术，其中2例术后出现呼吸危象，需要有创呼吸机支持，中位时间为14天。1例患者在术后第3天需要有创呼吸机支持。6例患者有胸腺瘤，12例有胸腺增生。3例患者需要静脉注射免疫球蛋白。9例患者在术后中位2.5天需要使用抗乙酰胆碱酯酶抑制剂。在随访中发现，胸腺切除术后病情缓解，重症肌无力危象的发生率降低，与术前相比，术后药物需求显著减少。本报告强调，即使组织病理学报告未证实胸腺瘤，重症肌无力危象发作后接受扩大胸腺切除术的患者也能从中受益。胸腺切除术后，重症肌无力危象得到缓解。重症肌无力危象患者应在监督下谨慎调整药物，并在即将发生重症肌无力危象时积极治疗。随着现代重症肌无力管理方法的应用，伴有重症肌无力危象时早期手术是安全的，且长期效果良好。
