The paper presents literature review on a rare congenital malformation of the spine and spinal cord. Key issues of etiology, classification, clinical presentation of caudal regression syndrome, and variants of conservative and surgical treatment are discussed.
To assess surgical treatment of children with congenital lumbar spine deformities associated with congenital developmental disorders of vertebrae and to select optimal approach to the treatment of such patients. Material and Methods. We have operated 16 patients aged from one year and 11 months to six years and three months with congenital scoliosis and kyphoscoliosis associated with developmental disorders of the lumbar vertebrae. Orthopedic status and the state of deformed spine before and after surgical intervention were evaluated based on data of clinical, neurological, and radiological examinations. Degree of immediate postoperative correction of congenital deformity and long-term outcomes after metal device removal were assessed. Results. Surgical treatment of patients younger than 3 years of age with isolated hemivertebra in the lumbar spine resulted in up to 86.0 % correction of scoliotic deformity and up to 77.5 % that of kyphotic deformity. The further child's growth is associated with a tendency toward spontaneous correction of residual deformity. In children of four to seven years old the mean correction of scoliosis reached 80.0 %, and that of kyphosis-87.5 %. The loss of correction was not observed during a short-time follow-up period. Conclusion. The optimal age for surgical treatment of congenital deformity in developmental vertebral disorders is the age below 3 years. Deformity correction at this age can provide a maximum approach of frontal and sagittal contours to their physiological norms.
The paper presents clinical observation of a patient with a rare congenital malformation of the distal spine and spinal cord-the caudal regression syndrome. The patient showed an absence of the lumbosacral spinal segment with neurological disorders and contractures of the lower extremities. The patient underwent reconstructive vertebral pelvic fusion surgery. Surgical treatment resulted in elimination of the lumbosacral spine instability, correction of kyphotic deformity, and creation of support ability of the spine.
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