Management of Congenital Scoliosis

Abstract

Congenital Scoliosis is an inborn deformity of the spine due to presence of vertebral anomalies causing an imbalance in the longitudinal growth of the spine in the coronal plane. These anomalies have no certain etiology, but recent studies were made

experimentally on mouse using genetic mutants, confirmed the possibility of genetic mutants to cause congenital deformities of the spine, other studies said that certain environmental factors may be the cause of the deformities especially when their insult occurs during the early pregnancy. At the time of formation of the spinal cord between the sixth and eighth weeks of gestation, the centers of chondrification in the membranous portion of the mesenchyme begins to ossify, central nervous system, cardio-pulmonary system and genito­ urinary tract begin to develop, so errors in the development in this stage of intra-uterine life, causing the vertebral malformations and the other associated systemic anomalies. The vertebral anomalies, which may occur, are failure in formation (hemivertebra or wedge vertebra), failure of segmentation (block vertebrae or unilateral bar) or mixed anomalies which is by far the commonest presentation. Prognosis and rate of curve progress in congenital scoliosis depend primarily on the

type of vertebral anomalies present. The worst prognosis is of unsegmented bar with contralateral hemivertebra, then bar only, hemivertebra, wedge vertebra and the least anomaly to progress is block vertebrae. Other anomalies which are frequently coexist with congenital scoliosis are congenital

kyphoscoliosis, spinal dysraphism complexes (like tethered cord, diastematomyelia and Syringomyelia), Klipple Feil syndrome, genito-urinary anomalies, congenital heart diseases, respiratory system anomalies and other less common anomalies. As the congenital scoliosis is mostly a rapidly progressive deformity, it is too important to diagnose it as early as possible. Reaching the diagnosis either clinically or by the recent imaging methods is the first step for early interference to prevent progression of curves, which makes the late interference more difficult with lower outcome. Clinical evaluation includes good history taking to determine possible cause of deformity, age of onset, rate of general growth, rate of deformity progression and other associated anomalies. Examination should include general examination (cardio-pulmonary function, study of growth factors) and orthopedic examination which includes trunk alignment (asymmetry of rib cage, shoulders and pelvis), neurological examination, skin and extremities examination.