Regional anesthesia and muscle-wasting diseases in pediatrics: A focused educational review.

Abstract

The muscular dystrophies or muscle-wasting diseases include a diverse group of genetic disorders, which result in progressive degeneration of skeletal muscles, progressive muscle weakness, and comorbid multi-system involvement. Duchenne muscle dystrophy is the most common type of muscular dystrophy with a reported incidence of 1 in every 3500-6000 male live births in the United States. Given the progressive nature of these disorders, skeletal muscle weakness frequently progresses to loss of the ability to ambulate and perform functions of daily life. In addition to affecting the skeletal musculature, many muscular dystrophies have effects on both cardiac and smooth muscles. As respiratory muscles are one of the most frequently affected muscles in patients with muscular dystrophies, progressive respiratory insufficiency may occur with dependance on non-invasive forms of respiratory support. Given the progressive multi-system involvement associated with the muscular dystrophies, perioperative care and the use of general anesthetic agents and opioids may result in postoperative respiratory failure. In an effort to avoid the deleterious effects of anesthetic agents and opioids on hemodynamic and respiratory functions, regional anesthesia may be used as an adjunct to or instead of general anesthesia. This manuscript provides a literature review and educational summary regarding the use of regional anesthetic techniques in pediatric-aged patients with muscular dystrophies.