Congenital scoliosis: management and future directions.

Congenital scoliosis remains an interesting and challenging diagnostic entity. Vertebral absence, partial formation or lack of segmentation may cause asymmetrical growth and resultant deformity. Because of the high frequency of associated anomalies within and outside the spine, a detailed history and physical examination are mandatory. Maternal, perinatal history, family history, and developmental milestones must be fully explored. Plain radiographs remain standard for diagnosis of congenital anomalies and measuring curve magnitude, progression and perhaps growth potential of the vertebral anomaly. Preoperative CT scan defines the anatomy and avoids any unexpected intraoperative posterior element deficiencies. MRI can exclude associated conditions of the spine, cranio-cervical junction and viscera. The recognition of curves with a bad prognosis at an early stage is pertinent to prevent curve progression and possible neurological complications. The goal of surgery is to achieve a straight spine and a physiological sagittal profile while maintaining flexibility, to arrest progression of the curve with a short fusion segment preserving as much normal spinal growth as possible. Developments in gene research continue to be promising and may potentially lead to early detection of congenital vertebral malformations.