Abstract

Myasthenia gravis (MG) is an autoimmune neuromuscular junction disorder and rarely coexists with aortic aneurysms requiring open repair. A 66-year-old patient with MG underwent extended thoraco-abdominal aortic aneurysm (TAAA) repair 16 years after onset of type-B acute aortic dissection. At 62 years, the patient was diagnosed with MG (MGFA class IIIa) from positive anti-acetylcholine receptor antibody without thymoma. Preoperatively, MG was well-controlled by prednisolone, cyclosporin and pyridostigmine. Extent II TAAA repair was performed under general anaesthesia maintained by total intravenous anaesthesia. Transcranial motor-evoked potential and somatosensory-evoked potential were applied to monitor intraoperative spinal cord ischaemia and muscle weakness. Amplitudes of motor-evoked potential and somatosensory-evoked potential attenuated intraoperatively but normalized after reperfusion from the reconstructed tube graft. Perioperative steroid coverage was given against surgical stress. The patient was weaned from mechanical ventilatory support on postoperative day 7. No signs of spinal cord ischaemia or muscle weakness were seen.

中文翻译：

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重症肌无力患者开放胸腹主动脉瘤修复术

摘要

重症肌无力 (MG) 是一种自身免疫性神经肌肉接头疾病，很少与需要开放修复的主动脉瘤共存。一名 66 岁的 MG 患者在 B 型急性主动脉夹层发病 16 年后接受了扩大的胸腹主动脉瘤 (TAAA) 修复。62 岁时，患者被诊断为 MG（MGFA IIIa 类），抗乙酰胆碱受体抗体阳性，无胸腺瘤。术前，强的松龙、环孢素和吡啶斯的明控制了MG。Extent II TAAA 修复在全静脉麻醉维持的全身麻醉下进行。应用经颅运动诱发电位和体感诱发电位监测术中脊髓缺血和肌肉无力。运动诱发电位和体感诱发电位的幅度在术中减弱，但在重建管移植物再灌注后恢复正常。针对手术压力给予围手术期类固醇覆盖。患者在术后第 7 天停止使用机械通气支持。没有发现脊髓缺血或肌肉无力的迹象。