Chapter 31 - Duchenne Muscular Dystrophy

Summary

Duchenne muscular dystrophy is a progressive neuromuscular disorder that is inherited in an X-linked recessive pattern, therefore predominantly affecting the male offspring of maternal carriers. Duchenne muscular dystrophy is characterized by progressive muscle weakness and damage that impacts both skeletal and cardiac muscle tissue. Symptoms typically manifest as clumsiness, weakness, or failure to meet gross motor milestones by 3–5 years of age. Chest wall and diaphragmatic weakness eventually result in respiratory insufficiency and the need for respiratory support in the late teens to early twenties. In the past, respiratory insufficiency and infections were the leading cause of death for patients with Duchenne muscular dystrophy, but now, with early noninvasive respiratory support, heart failure and arrhythmias are often life limiting. The combination of respiratory and cardiac impairment makes these patients challenging to care for in the perioperative environment. This chapter discusses the anesthetic and perioperative management of a Duchenne muscular dystrophy patient requiring implantation of a cardioverter defibrillator.