基础医学与临床 ›› 2025, Vol. 45 ›› Issue (12): 1653-1656.doi: 10.16352/j.issn.1001-6325.2025.12.1653

• 疑难病例 • 上一篇    下一篇

面肩肱型肌营养不良患者行直肠癌手术的麻醉管理一例

陈雯, 虞雪融*, 高鹏, 申乐   

  1. 中国医学科学院 北京协和医学院 北京协和医院 麻醉科,北京 100730
  • 收稿日期:2025-09-12 修回日期:2025-10-14 出版日期:2025-12-05 发布日期:2025-11-25
  • 通讯作者: *yuxuerong@pumch.cn

Anesthesia management for the surgical resection of rectal cancer in a patient with facioscapulohumeral muscular dystrophy:a case report

CHEN Wen, YU Xuerong*, GAO Peng, SHEN Le   

  1. Department of Anesthesiology, Peking Union Medical College Hospital, CAMS & PUMC, Beijing 100730, China
  • Received:2025-09-12 Revised:2025-10-14 Online:2025-12-05 Published:2025-11-25
  • Contact: *yuxuerong@pumch.cn

摘要: 目的 探讨患有罕见神经肌肉疾病面肩肱型肌营养不良(FSHD)同时合并有限制性通气功能障碍的患者接受手术治疗时的围术期麻醉管理策略。方法 回顾性分析一例FSHD合并中重度限制性通气功能障碍行直肠癌手术患者的临床资料,并对其临床表现、合并症、术前评估、术中麻醉管理及术后镇痛治疗进行分析与总结。结果 FSHD为罕见的遗传性疾病,术前多学科会诊评估至关重要。本例经多科会诊评估后,采用全静脉麻醉,术中予有创动脉监测、血气分析、体温监测,充分镇痛,密切呼吸监测,采用非保护通气策略;术毕充分吸痰膨肺肌力完全恢复后顺利拔管,同时在保证呼吸功能的基础上,充分镇痛并转入重症监护病房(ICU)监护,治疗效果满意后出院。结论 对FSHD等罕见神经肌肉疾病患者,术前需充分评估与优化,临床医生需了解患者相关并发症如限制性通气功能障碍,制定个体化围术期麻醉管理方案,术中加强监测如呼吸系统,维持围术期血流动力学稳定,避免缺氧及二氧化碳潴留,以降低并发症风险。

关键词: 肌营养不良, 限制性通气功能障碍, 手术治疗, 围手术期麻醉管理

Abstract: Objective To discuss the perioperative anesthesia management strategy for the surgical resection of rectal cancer in a patient with a rare neuromuscular disease-Facioscapulohumeral Muscular Dystrophy (FSHD) complicated by restrictive ventilatory dysfunction. Methods Clinical data of a patient with FSHD complicated by moderate-to-severe restrictive ventilatory dysfunction undergoing rectal cancer surgery were retrospectively collected; the clinical manifestations, complication, preoperative evaluation, intraoperative anesthesia management and postoperative pain treatment were analyzed and summarized. Results FSHD is a rare genetic disorder, and preopera-tive multidisciplinary evaluation is critical. In this case, total intravenous anesthesia was employed, with invasive arterial pressure monitoring, blood gas analysis, body temperature, sufficient analgesia, close respiratory monitoring and lung protective ventilation strategy after preoperative multidisciplinary evaluation. After thorough sputum suction, lung expansion, and complete recovery of muscle strength, the patient was successfully extubated; ensuring respiratory monitoring after surgery, sufficient analgesia was administered, and transferred to the ICU for monitoring, and ultimately discharged with satisfactory treatment results. Conclusions For patients with rare neuromuscular diseases such as FSHD, thorough preoperative evaluation and optimization are essential. Clinicians should be aware of related complications, such as restrictive ventilatory dysfunction, and develop individualized anesthesia plans. Intraoperative monitoring, particularly of the respiratory and hemodynamic systems, should aim to prevent hypoxia and carbon dioxide retention, thereby reducing the risk of complications.

Key words: muscular dystrophy, restrictive ventilatory dysfunction, surgical treatment, perioperative anesthesia management

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