The Development of Spinal Deformity in Patients with Duchenne Muscular Dystrophy: Clinical Assessment, Surgical...

Duchenne muscular dystrophy (DMD) causes progressive skeletal, respiratory and cardiac muscle weakness in affected males. Most DMD patients develop scoliosis following loss of ambulation. This narrative review describes recommendations for the management of scoliosis in DMD patients using a review of the current literature evidence and a consensus review by the DMD Care UK Spinal Surgery Working Group. Advances in medical treatments have improved life expectancy for DMD patients. Spinal bracing is not effective in preventing the deterioration of scoliosis. Seating and wheelchair adaptations can provide postural support. The multidisciplinary assessment of patients with DMD requiring treatment for scoliosis is reviewed, with particular focus on bone, cardiac and respiratory health. The indications, surgical techniques, and type of spinal instrumentation for surgical management for progressively severe scoliosis with or without pelvic obliquity are discussed. Anaesthetic techniques, intraoperative neuromonitoring, perioperative care, and postoperative management in the ICU are discussed for the optimal management of DMD patients undergoing surgery to correct spinal deformity. Finally, regional and holistic functional assessments, patient satisfaction and long-term health, quality of life, and life expectancy for DMD patients undergoing treatment for spinal deformity are reviewed.