Anesth Prog. 2024 May 3;71(1):44-52. doi: 10.2344/673191.
Muscular dystrophy encompasses a group of genetic conditions with progressive muscle damage and weakness. Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are X-linked recessive disorders that affect the production of the protein dystrophin. Emery-Dreifuss muscular dystrophy (EDMD) is typically an X-linked-recessive disorder involving the gene that codes for emerin. Facioscapulohumeral muscular dystrophy and oculopharyngeal muscular dystrophy (OPMD) are both autosomal dominant disorders. Although commonly mistaken as a condition in which patients are susceptible to malignant hyperthermia with volatile inhalational anesthetics, muscular dystrophy is more closely associated with rhabdomyolysis. Providers developing an anesthetic plan for dental patients with muscular dystrophy must take into consideration the patient's baseline cardiac and pulmonary function as well as the potential for abnormalities. Nondepolarizing neuromuscular blocker use is safe but likely to result in prolonged skeletal muscle relaxation. Succinylcholine and volatile anesthetics are generally contraindicated due to the risks of rhabdomyolysis and hyperkalemia with subsequent ventricular fibrillation, cardiac arrest, and death if left untreated. In-depth understanding of the more commonly encountered forms of muscular dystrophy is vital to providing safe and effective ambulatory anesthesia care for patients undergoing dental treatment outside the traditional hospital operating room setting.
肌肉萎缩症包括一组具有进行性肌肉损伤和无力的遗传疾病。杜氏肌营养不良症（DMD）和贝克肌营养不良症（BMD）是 X 连锁隐性疾病，影响肌营养不良蛋白的产生。面肩肱型肌营养不良症（EDMD）通常是一种 X 连锁隐性疾病，涉及编码核纤层蛋白的基因。面肩肱型肌营养不良症和眼咽型肌营养不良症（OPMD）都是常染色体显性疾病。尽管常被误认为是一种患者易患挥发性吸入麻醉剂恶性高热的疾病，但肌肉萎缩症与横纹肌溶解症的关系更为密切。为患有肌肉萎缩症的牙科患者制定麻醉计划的医生必须考虑到患者的基线心肺功能以及潜在的异常情况。非去极化神经肌肉阻滞剂的使用是安全的，但可能导致骨骼肌松弛时间延长。琥珀酰胆碱和挥发性麻醉剂通常是禁忌的，因为横纹肌溶解症和高钾血症会导致随后的心室颤动、心脏骤停和死亡，如果不治疗的话。深入了解更为常见的肌肉萎缩症类型对于在传统医院手术室环境之外进行牙科治疗的患者提供安全有效的门诊麻醉护理至关重要。
