Kim O M O, Elliott D
Department of Anaesthesia, Westmead Hospital, Sydney, New South Wales, Australia.
Anaesth Intensive Care. 2010 Jul;38(4):744-7. doi: 10.1177/0310057X1003800419.
Emery-Dreifuss muscular dystrophy is a rare form of muscular dystrophy involving both cardiac and skeletal muscles. Cardiac involvement frequently leads to dilated cardiomyopathy, arrhythmias and may precipitate sudden cardiac death. Skeletal involvement is characterised by early contractures and muscle weakness in the humeroperoneal distribution. We describe the anaesthetic management of a 29-year-old patient with Emery-Dreifuss muscular dystrophy presenting for elective caesarean section and discuss the disorder and its potential anaesthetic implications.
埃默里-德赖富斯肌营养不良症是一种罕见的肌营养不良症，累及心肌和骨骼肌。心脏受累常导致扩张型心肌病、心律失常，并可能引发心源性猝死。骨骼受累的特征是肱骨-腓骨分布区出现早期挛缩和肌肉无力。我们描述了一名29岁埃默里-德赖富斯肌营养不良症患者择期剖宫产的麻醉管理，并讨论了该疾病及其潜在的麻醉影响。
