Scoliosis in children with myelomeningocele occurs frequently and is multifactorial in origin. The aim of this paper is to review the evidence regarding the causal association, if any, between isolated scoliosis and spinal cord tethering, Chiari malformation, or syringomyelia in this population.
A Medline search of the English-language literature from 1966 to 2002 was undertaken. All of the pertinent articles that were identified underwent careful review to determine the strength of the evidence supporting the claim that isolated scoliosis in children with myelomeningocele is related to spinal cord tethering, Chiari malformation, or syringomyelia. The strength of the evidence was graded using the Oxford Centre for Evidence-Based Medicine Levels of Evidence (May 2001). A survey was also sent to practicing pediatric neurosurgeons and the returned responses were evaluated.
All available studies were classified as Level 4 studies (case series and flawed cohort and case-control studies). Based on the strength of the available data, there may be an association between spinal cord tethering and scoliosis, particularly in those patients with upper lumbar lesions and spinal curves less than 45°. There is little evidence to support a causal relationship between scoliosis and Chiari malformation or syringomyelia in this population.