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Muscular Dystrophy
January 29, 2024
Pedsortho.com Staff
Muscular dystrophy (MD) encompasses a group of genetic disorders characterized by progressive weakness and degeneration of the skeletal muscles that control movement. There are several types of MD, with Duchenne Muscular Dystrophy (DMD) and Becker Muscular Dystrophy being among the most common. Orthopedic considerations are crucial in managing MD due to the direct impact of muscle weakness on the musculoskeletal system, leading to various complications that affect mobility, posture, and overall quality of life.
Orthopedic Complications in Muscular Dystrophy
Scoliosis: Progressive muscle weakness can lead to the development of scoliosis, a curvature of the spine, which is particularly common in non-ambulatory patients. Scoliosis can complicate sitting, breathing, and can lead to chronic pain.
Joint Contractures: Reduced mobility can result in joint contractures, where joints become stiff and locked in a bent or fixed position, further limiting movement and flexibility.
Bone Density and Fracture Risk: Individuals with MD often have lower bone density, making bones more fragile and increasing the risk of fractures. This is compounded by reduced mobility and the use of corticosteroids, which can also decrease bone density.
Foot and Ankle Deformities: Weakness in the muscles of the foot and ankle can lead to deformities such as pes planus (flat feet) or equinovarus (clubfoot), affecting gait and stability.
Orthopedic Management Strategies
Surveillance and Early Intervention: Regular monitoring by an orthopedic specialist is essential to identify and address musculoskeletal issues early. This includes routine assessments of posture, spine curvature, and joint mobility.
Physical Therapy: A cornerstone of managing MD, physical therapy aims to maintain muscle strength and flexibility for as long as possible, delay contracture development, and improve mobility.
Bracing and Orthotic Devices: Braces, splints, or orthotic devices can be used to support weakened muscles, correct deformities, and prevent contractures, particularly in the ankles and feet.
Surgical Interventions: Surgery may be considered for correcting severe scoliosis or releasing contractures that significantly impact function or quality of life. Spinal fusion may be necessary for severe scoliosis to stabilize the spine and alleviate pain.
Bone Health Management: Addressing bone health is critical. This may involve supplementation with calcium and vitamin D, and in some cases, medications to increase bone density. Weight-bearing exercises, as tolerated, can also help strengthen bones.
Fall Prevention: Due to the increased risk of fractures, measures to prevent falls are important. This can include home modifications, safe exercise programs, and the use of mobility aids.
Multidisciplinary Approach
Management of muscular dystrophy requires a multidisciplinary approach, involving neurologists, cardiologists, pulmonologists, orthopedic surgeons, physical therapists, and occupational therapists, among others. This team works together to address the wide range of challenges faced by individuals with MD, with orthopedic considerations being a critical component of comprehensive care. The goal is to maximize function, manage complications, and enhance the quality of life for those affected by muscular dystrophy.