Congenital Scoliosis

Abstract

Congenital scoliosis is a subset of early-onset scoliosis. This is due to abnormal normal vertebral development during the fourth to sixth week of gestation. Congenital scoliosis is classified as a failure of formation of the vertebra (e.g., hemivertebrae), failure of segmentation of the vertebra (e.g., unsegmented bars), or mixed. Asymmetrical spinal growth and progressive spinal deformity may occur due to vertebral absence, partial formation, or lack of segmentation. A detailed history, examination, and investigations are mandated, as these children have a higher prevalence of associated anomalies within and outside the spine. A thorough history includes maternal and perinatal history, family history, and developmental milestones. Plain radiographs remain standard for the evaluation of congenital vertebral anomalies and measuring curve magnitude, progression, and perhaps growth potential of the vertebral anomaly. A preoperative CT scan delineates the anomaly and aids surgical planning. MRI, ultrasound, and echocardiogram can exclude associated neuro spinal axis and visceral abnormalities. Early recognition and prompt intervention are mandated. Treatment aims to control curve progression and allow maximum spinal column growth and thoracic volume development for normal pulmonary function. Observation and casting or bracing have a limited role. Standard surgical procedures include convex hemiepiphysiodesis, hemivertebra excision, and short fusion or growing spine techniques.