PDF(Pubmed)
Abstract：
BACKGROUND: Nemaline myopathy (NM) is a congenital disease characterized by nonprogressive or slowly progressing muscle weakness and may increase the risk of anesthesia in case of respiratory muscle or cardiac involvement. Care should be taken to prevent respiratory failure after surgery.
METHODS: A 35-year-old man with NM, who had difficult airway, restrictive ventilatory pattern, and pulmonary hypertension, required general anesthesia for surgery because of limited mouth opening.
METHODS: The patient was diagnosed with NM (ACTA1 mutation) and coronoid hyperplasia.
METHODS: Awake fiberoptic nasal intubation was performed following preparations for analgesia. General anesthesia was maintained using inhalational anesthetics and opioids without using neuromuscular blocking agents.
RESULTS: General anesthesia remained well maintained during surgery, with no movement or spontaneous breathing of the patient and he recovered from anesthesia uneventfully without complications.
CONCLUSIONS: This report highlights the safe performance of anesthesia induction and recovery in a case where anesthesia management is necessary for surgery in a patient of NM at a high risk of anesthesia-related complications.
METHODS: A 35-year-old man with NM, who had difficult airway, restrictive ventilatory pattern, and pulmonary hypertension, required general anesthesia for surgery because of limited mouth opening.
METHODS: The patient was diagnosed with NM (ACTA1 mutation) and coronoid hyperplasia.
METHODS: Awake fiberoptic nasal intubation was performed following preparations for analgesia. General anesthesia was maintained using inhalational anesthetics and opioids without using neuromuscular blocking agents.
RESULTS: General anesthesia remained well maintained during surgery, with no movement or spontaneous breathing of the patient and he recovered from anesthesia uneventfully without complications.
CONCLUSIONS: This report highlights the safe performance of anesthesia induction and recovery in a case where anesthesia management is necessary for surgery in a patient of NM at a high risk of anesthesia-related complications.
摘要：
背景：神经肌病（NM）是一种先天性疾病，其特征是非进行性或缓慢进行性肌无力，在呼吸肌或心脏受累的情况下可能会增加麻醉的风险。手术后应注意预防呼吸衰竭。
方法：一名患有NM的35岁男子，谁有困难的气道，限制性通气模式，肺动脉高压,由于张口有限，手术需要全身麻醉。
方法：患者被诊断为NM（ACTA1突变）和冠状增生。
方法：在镇痛准备后进行Awake纤维鼻插管。使用吸入麻醉药和阿片类药物维持全身麻醉，而不使用神经肌肉阻断剂。
结果：手术期间全身麻醉维持良好，患者无运动或自主呼吸，麻醉恢复顺利，无并发症。
结论：本报告强调了在麻醉相关并发症高风险的NM患者需要进行手术麻醉管理的情况下，麻醉诱导和恢复的安全性。
方法：一名患有NM的35岁男子，谁有困难的气道，限制性通气模式，肺动脉高压,由于张口有限，手术需要全身麻醉。
方法：患者被诊断为NM（ACTA1突变）和冠状增生。
方法：在镇痛准备后进行Awake纤维鼻插管。使用吸入麻醉药和阿片类药物维持全身麻醉，而不使用神经肌肉阻断剂。
结果：手术期间全身麻醉维持良好，患者无运动或自主呼吸，麻醉恢复顺利，无并发症。
结论：本报告强调了在麻醉相关并发症高风险的NM患者需要进行手术麻醉管理的情况下，麻醉诱导和恢复的安全性。
