Orthopedic management in Duchenne muscular dystrophy

This article provides a comprehensive overview of the orthopedic management strategies in Duchenne muscular dystrophy (DMD). DMD manifests with severe muscle wasting, respiratory insufficiency, and cardiomyopathy, with initial symptoms emerging in early childhood. By the age of 10-12 years, most patients require wheelchairs due to muscle degeneration, which often leads to spinal deformities and joint contractures. The absence of dystrophin causes the breakdown of the dystrophin glycoprotein complex, causingmuscle degeneration, necrosis, and fibrosis. Current treatments focus on symptom management, including physical therapy, corticosteroids, orthopedic surgery, ventilatory and nutritional support, and cardiac care. Long-term glucocorticoid therapy can extend mobility and life expectancy, delay the need for ventilation, and reduce the rates of scoliosis surgery. Orthopedic care focuses on prevention to preserve motor function and bone health and involves an interdisciplinary team, early use of orthotic devices, and promotion of proper posture. Common deformities include varus equinus, hip flexor retractions, scoliosis, and pelvic obliquity. Scoliosis management involves posterior fusion surgery extended to the pelvis or limited to the L5 vertebra. Preoperative evaluation should include assessing the risk of hip flexion contracture decompensation following arthrodesis. There now seems to be increasing consensus favoring lower extremity surgery only in exceptional cases. This shift is due to the limited success of surgery, with frequent recurrence of muscle retractions and minimal expected benefits. Fracture management involves bisphosphonate therapy post-fracture, with vertebral compression and lower limb fracture being the most prevalent.