Yamashita Atsushi, Muramatsu Yuka, Matsuda Hiromi, Okamoto Hirotsugu
Department of Anesthesia, Kitasato University School of Mediceine, 1-15-1 Kitasato, Sagamihara, Kanagawa, 252-0375, Japan.
JA Clin Rep. 2022 Aug 31;8(1):70. doi: 10.1186/s40981-022-00560-1.
Congenital myasthenia syndrome is a heterogeneous disease with impaired neuromuscular transmission.
This report describes a 13-year-old child with congenital myasthenia syndrome who underwent surgery for scoliosis under general anesthesia. We used a small dose of rocuronium, neuromuscular transmission monitoring, and non-invasive positive pressure ventilation for postoperative respiratory management. There were no respiratory complications during the perioperative period.
As there are only a few reports on the anesthetic management of patients with congenital myasthenia syndrome, we applied the principles of managing autoimmune myasthenia gravis. The postoperative management described herein can prevent respiratory complications in patients with congenital myasthenia syndrome.
先天性肌无力综合征是一种神经肌肉传递受损的异质性疾病。
本报告描述了一名患有先天性肌无力综合征的13岁儿童，其在全身麻醉下接受了脊柱侧弯手术。我们使用了小剂量罗库溴铵、神经肌肉传递监测，并采用无创正压通气进行术后呼吸管理。围手术期未出现呼吸并发症。
由于关于先天性肌无力综合征患者麻醉管理的报道较少，我们应用了自身免疫性重症肌无力的管理原则。本文所述的术后管理可预防先天性肌无力综合征患者的呼吸并发症。
