Cripe Linda H, Tobias Joseph D
The Department of Pediatrics, Nationwide Children's Hospital, Columbus, OH 43205-2664, USA.
Paediatr Anaesth. 2013 Sep;23(9):777-84. doi: 10.1111/pan.12229. Epub 2013 Jul 19.
Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) is a progressive multisystem neuromuscular disorder. In addition to the skeletal muscle, the myocardium in the DMD/BMD patient is dystrophin deficient which results in a progressive cardiomyopathy. The myopathic myocardium poses significant risk of increased morbidity and mortality at the time of major surgical procedures. Careful attention must be given to the DMD/BMD patient during the intraoperative and postoperative period. Anesthesia selection is critical and anesthetics should be avoided which have been shown to be harmful in this patient population. Preanesthesia assessment should include cardiac consultation and detailed preoperative evaluation. Intraoperative management needs to insure that the weakened myocardium is not compromised by physiologic changes such as hypotension or major fluid shifts. Finally, attention to the cardiac status of the patient must continue into the postoperative period. The surgical care of the DMD/BMD patient requires a multispecialty approach to insure operative success.
杜氏肌营养不良症/贝克肌营养不良症(DMD/BMD)是一种进行性多系统神经肌肉疾病。除骨骼肌外,DMD/BMD患者的心肌缺乏抗肌萎缩蛋白,这会导致进行性心肌病。肌病性心肌在重大外科手术时会带来显著的发病率和死亡率增加的风险。在术中及术后期间,必须密切关注DMD/BMD患者。麻醉选择至关重要,应避免使用已证明对该患者群体有害的麻醉剂。麻醉前评估应包括心脏会诊和详细的术前评估。术中管理需要确保虚弱的心肌不会因低血压或大量液体转移等生理变化而受到损害。最后,对患者心脏状况的关注必须持续到术后阶段。DMD/BMD患者的手术护理需要多专业方法以确保手术成功。