Kosin Med J.  2020 Jun;35(1):64-68. 10.7180/kmj.2020.35.1.64.

General Anesthesia for a Patient with GNEMyopathy: a case report

• Kang C
• An Jh
• Kwon JY
• Hwang BY

• KMID: 2503731
• DOI: http://doi.org/10.7180/kmj.2020.35.1.64

Abstract

GNE, or bifunctional UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase, myopathy presents with symptoms of foot drop, followed by lower and upper extremity muscle weaknesses and sparing of the quadriceps. Myopathies usually increase the risks of complications related to general anesthesia. The anesthetic management of patients with GNEmyopathy has not been previously reported. Herein, we report a case of GNEmyopathy in a 37-year-old woman and discuss anesthetic considerations for elective laparoscopic hysterectomy and bilateral salpingectomy, focusing on the postoperative airway management. We avoided administering neuromuscular-blocking agents and instead used a laryngeal mask airway. The anesthetic management combining the use of a laryngeal mask airway and desflurane without neuromuscular-blocking agents provided sufficient abdominal and diaphragmatic muscle relaxations for sustaining the pneumoperitoneum for laparoscopic surgery.

Keyword

GNE myopathy; Laryngeal mask airway; Neuromuscular Blocking Agents

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