Epidemiology
- Scoliosis in about 95% of boys with Duchenne muscular dystrophy without steroid treatment
- Corticosteroids reduce incidence and delay onset
Pathology
- Progressive proximal weakness and trunk muscle failure
- Progression accelerates after loss of ambulation
- Curves progress around 10 degrees per year once wheelchair bound
- Long C shaped thoracolumbar curves with pelvic obliquity
Clinical Features
- Sitting imbalance and pressure areas
- Declining respiratory function, with FVC falling over time
- Cardiomyopathy
- Gower sign and calf pseudohypertrophy
- Loss of ambulation around early adolescence
Investigations
- Sitting spine radiographs including pelvis
- Pulmonary function testing with FVC
- Echocardiography
- Overnight oximetry or sleep study for nocturnal hypoventilation
Management
- Bracing does not prevent progression
- Fusion indicated with curves of 25 degrees or more in non ambulators
- Operate while respiratory reserve is adequate, before FVC falls too low
- Posterior fusion from upper thoracic spine to pelvis in wheelchair bound patients
- Fusion improves sitting balance, comfort and nursing care
- Fusion may slow the decline in respiratory function, though evidence is weak
Complications
- Malignant hyperthermia like reactions and rhabdomyolysis, so avoid suxamethonium and volatile anaesthetics
- Heavy blood loss from poor vascular smooth muscle function
- Postoperative pneumonia and prolonged ventilation
- Cardiac arrhythmia and failure
- Pseudarthrosis and implant failure
- Loss of hand to mouth function if the spine is overcorrected or lordosis excessive
Reviewed by Professor Phong Tran, Head of Orthopaedic Surgery, Western Health. Last updated 10 October 2026.