Congenital Scoliosis

Congenital scoliosis is defined as a lateral deviation of the spine associated with one of a broad range of congenital vertebral malformations (CVMs) that can form during in utero development. It is distinct from other spinal deviations in which malformations do not occur and can present as an isolated spine anomaly or be associated with a large number of visceral organ and syndromic abnormalities. In Smith’s “Recognizable Patterns of Human Malformation,” over 40 syndromes have CVMs listed as one of the presenting features (1). The malformations are always present at birth, but the development of the scoliosis may occur over time. They can occur in any part of the spinal column and are believed to be a result of the disruption of the process of somatogenesis that occurs between the 5th and 8th weeks of gestation (2–8). These malformations are not unique to humans as several other species develop such abnormalities in response to teratogenic exposures or from genetic predispositions (9–14). Of great importance is the high incidence of associated abnormalities in other organ systems that can lead to significant adverse impacts upon the health and well-being of affected individuals. In addition to this is the realization that management of the developing deformity is often extremely challenging and, due to the variation of presentation, highly individualized. This chapter focuses upon children with congenital vertebral anomalies. We do not discuss infantile scoliosis or other conditions where there may be deformity without malformations. INCIDENCE/PREVALENCE