Anaesthetic management in patients with Duchenne muscular dystrophy undergoing orthopaedic surgery

Abstract

CONTEXT: Patients with Duchenne muscular dystrophy are at increased risk of some anaesthesia-related hazards such as rhabdomyolysis, fever and hyperkalaemia. OBJECTIVES: To evaluate the management of anaesthesia in patients with Duchenne muscular dystrophy, including preoperative evaluation, intraoperative performance, critical events and postoperative care. DESIGN: We performed a retrospective case review study of anaesthesia in patients with Duchenne muscular dystrophy covering the period between April 2000 and December 2008. PATIENTS: 91 Duchenne patients undergoing 232 general anaesthetics for orthopaedic surgical interventions. SETTING: University hospital. RESULTS: Anaesthesia was performed using propofol, opioids and, if required, a non-depolarising muscle relaxant. Eight difficult direct laryngoscopies were reported. All patients undergoing spinal fusion surgery received transfusion of homologous blood products and required postoperative invasive ventilatory support for an average of 19 h. There was no severe anaesthesia-related complication and no case of unexplained fever or rhabdomyolysis. CONCLUSION: This retrospective survey confirms clinical experience that total intravenous anaesthesia can be used safely in Duchenne patients without major concern. Further prospective studies are necessary to establish evidence-based clinical guidelines for daily practice.