脊髓性肌萎缩小儿的麻醉管理

Anesthetic management of spinal muscular atrophy in children

脊髓性肌萎缩(spinal muscular atrophy,SMA)是一种罕见的神经肌肉性疾病,是儿童期最常见的遗传致死性疾病之一。这类患儿因为全身肌肉萎缩及其他畸形的存在,麻醉存在一定的难度。文章介绍该疾病的发病机制和分型、麻醉管理(麻醉前评估、麻醉方式和药物的选择、术后监护及镇痛)及其治疗进展对麻醉的影响,为拟行手术的SMA患儿的围手术期管理提供参考。

Spinal muscular atrophy(SMA)is the most common inherited lethal disease in childhood.The anesthetic management of these children is often difficult as a result of muscle weakness and other malformations.This paper briefly describes the pathogenesis and patterns,anesthetic management(including pre-anesthetic assessment,anesthesia methods'and drugs'selection,post-anesthetic monitor and analgesia)and the effect of recent therapy advances on anesthesia,to provide some ideas for anesthetists to manage the children of SMA who are undergoing surgery.