Perioperative anesthetic management in patients with Duchenne muscular dystrophy

Abstract

SUMMARY D uchenne muscular dystrophy (DMD) is a severe, progressive disease that affects 1 in 3600–6000 live male births. It is characterised by delayed milestones, progressive muscle weakness with calf pseudohypertrophy, mental retardation in one third and the development of a cardiomyopathy. Over 50% of patients will have a clinically relevant dilated cardiomyopathy by 15 years of age. Death is expected in early to mid-adulthood because of progressive cardiomyopathy or respiratory insufficiency. 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 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 patient requires a multispecialty approach to insure operative success. Duchenne muscular dystrophy are associated with life-threatening perioperative complications, including rhabdomyol-ysis, hyperkalemia, and hyperthermia. Current recommendations contraindicate use of succinylcholine and volatile anesthetics; however, the latter recommendation remains controversial.