Ahamed Seyed, Swaminathan Ramji
Anaesthesiology, Amrith Hospital, Chennai, IND.
Anesthesiology, NMC Specialty Hospital, Abu Dhabi, ARE.
Cureus. 2023 Jan 31;15(1):e34442. doi: 10.7759/cureus.34442. eCollection 2023 Jan.
Patients with muscular dystrophies, especially those pauci-symptomatic presenting for surgery pose a complex problem for the anesthesiologist in preparing, optimizing and performing anesthesia. A myriad of complications including cardiac, respiratory, rhabdomyolysis, hyperkalemia, increased sensitivity to muscle relaxants etc., influence the anesthetic technique and recovery. Preoperative identification and appropriate choice of anesthesia technique can prevent most of the adverse events during anesthesia. We present a case of facioscapulohumeral dystrophy (FSHD) presenting for emergency appendectomy. Preoperative investigations and lung function were adequate. The patient underwent general anesthesia with propofol, cisatarcurium, and remifentanil and was maintained on total IV anesthesia for the duration of surgery. Continuous neuromuscular monitoring was carried out at two sites and the patient responded normally to intubating dose of cisatracurium and subsequent top-up doses, showing no increased sensitivity or need for dose reduction. The patient was hemodynamically stable with propofol and remifentanil infusions, with inhalational agents purposefully avoided. The patient was reversed with anticholinesterase and good train-of-four (TOF) ratio (>90%) was ensured before being shifted to recovery. The patient had a further uneventful course in the hospital.
患有肌肉萎缩症的患者，尤其是那些症状轻微而需接受手术的患者，给麻醉医生在准备、优化和实施麻醉方面带来了复杂的问题。包括心脏、呼吸、横纹肌溶解、高钾血症、对肌肉松弛剂敏感性增加等在内的众多并发症，都会影响麻醉技术和术后恢复。术前识别并合理选择麻醉技术可预防麻醉期间的大多数不良事件。我们报告一例因急诊阑尾切除术而就诊的面肩肱型肌营养不良症（FSHD）患者。术前检查和肺功能均正常。患者采用丙泊酚、顺式阿曲库铵和瑞芬太尼进行全身麻醉，并在手术全程维持全凭静脉麻醉。在两个部位进行连续神经肌肉监测，患者对顺式阿曲库铵的插管剂量及随后的追加剂量反应正常，未显示出敏感性增加或需要减少剂量。使用丙泊酚和瑞芬太尼输注时患者血流动力学稳定，特意避免使用吸入性麻醉药。使用抗胆碱酯酶药物使患者苏醒，并在转至恢复室之前确保有良好的四个成串刺激（TOF）比值（>90%）。患者在医院的后续病程顺利。
