Management of Spinal Deformity in Cerebral Palsy

Abstract

Scoliosis is common in cerebral palsy (CP), typified by rapidly progressive curves that impact patient function and quality of life. With age, these curves become rigid, resulting in functional disabilities including sitting imbalance, decubitus ulcers, decreased socialisation, increased caregiver demands, and, in some cases, decreased pulmonary function.  The incidence of scoliosis has been correlated to disease severity, necessitating clinical and radiographic surveillance based on functional level according to the Gross Motor Function Classification System (GMFCS). The use of bracing for scoliosis in CP is to support the collapsing spine rather than to prevent curve progression, and should not be expected to alter natural history. Scoliosis correction surgery, however, is indicated for progressive curves greater than 40-50°, with the primary surgical goals being achieved with a balanced spine over a level pelvis, allowing for a more stable sitting platform and improved quality of life. Identifying and treating the causes of concomitant pelvic obliquity are important to achieve optimal sitting balance. Hip displacement and scoliosis are often coincident in CP, but the order of surgical management remains controversial. The mainstay of treatment for scoliosis involves posterior instrumentation and fusion from the upper thoracic spine to the pelvis. Though several options are available, the best evidence to date would suggest that segmental pedicle screw fixation achieves better curve correction and an improved risk profile over other implant choices. Often proposed as a benefit of scoliosis surgery in CP, the true impact of curve correction on pulmonary function has not been well studied and is currently unknown. Substantial comorbidities increase the peri-operative risk profile – including swallowing difficulties, aspiration risk, recurrent respiratory infections, epilepsy, and malnutrition – necessitating patient counseling and mitigating strategies to optimize surgical outcomes. Optimizing medical and nutritional management pre- and peri-operatively are important to tip the balance in favour of benefits over risks. The best evidence to date would suggest that scoliosis surgery improves quality of life and is warranted in spite of the risks involved.