重症先天性肌无力综合征的产科和麻醉管理

先天性肌无力综合征是一组表现为神经肌肉传递障碍的异质性遗传疾病。尽管它与后天免疫性重症肌无力综合征具有共同特征——肌肉疲劳，但两者在病理生理、诊断、治疗和病程进展等方面存在着不同。本文报道1例28岁先天性肌无力综合征的产妇，在腰麻下行择期剖宫产术。患者由于肌力失衡和肌力减弱致脊柱侧弯和慢性呼吸衰竭，麻醉医师在超声引导下完成腰麻操作，手术中给予无创正压通气保持肺容积、有效地防止呼吸功能的恶化。

The congenital myasthenia syndromes form a heterogeneous group of genetic diseases character- ized by defective neuromuscular transmission. Although they have muscle fatigability in common with the acquired immune myasthenia syndrome, there are important pathophysiological, diagnostic, management and progression pattern differences between them. We report the management of a 28-yr-old patient with longstanding congenital myasthenia syndromes, who underwent an elective cesarean delivery under spinal anesthesia. Muscle imbalance plus weakness-related scoliosis and chronic respiratory failure complicated her management. Ultrasonography was used to facilitate the spinal anesthetic. Intraoperative noninvasive positive pressure ventilation maintained lung volumes effectively and prevented deterioration in respiratory function.