Orthopaedic Management in Duchenne Muscular Dystrophy.

UNLABELLED

Duchenne muscular dystrophy (DMD) is the most common childhood muscular dystrophy and occurs primarily in males, affecting 1 in 3600-6000 live male births. The natural course of DMD results in a profound, progressive decline in muscle strength, requiring the use of a wheelchair, typically by age 13, and ultimately leading to fatal respiratory and cardiac dysfunction by young adulthood. Musculoskeletal care for patients with DMD often centers around preventing and managing contractures, fractures, and scoliosis. Medical considerations that affect musculoskeletal care include osteopenia and osteoporosis, endocrinopathies, and pulmonary diseases, which often affect perioperative care. This study aims to provide a detailed and updated review of current treatment options for DMD, highlighting the role of novel treatment options (eg gene therapy) that are changing the landscape of care for the DMD population.

KEY CONCEPTS

(1)Orthopaedic management of patients with Duchenne muscular dystrophy centers primarily around mobility preservation and managing contractures, scoliosis, and fractures.(2)Perioperative considerations include cardiology (ie preoperative echocardiogram for assessing cardiomyopathy progression), pulmonology (ie sleep-related breathing disorders that may result in compromised respiratory function intraoperatively), endocrinology (ie chronic glucocorticoid use that should not be paused in the perioperative period to prevent adrenal crisis), and anesthesiology (ie avoidance of depolarizing muscle relaxants).(3)As new gene-modifying treatments become available, the orthopaedic management of patients with Duchenne muscular dystrophy will continue to evolve rapidly.