Variants of segmental spinal dysgenesis: literature review and our own experience of treatment

Purpose The purpose of the work was to study the clinical and neuroradiological features of segmental spinal dysgenesis (SSD), congenital anomaly with local hypogenesis of a spinal motion segment and the spinal cord; as well as the selection of an optimal tactics of treating this anomaly. Methods The authors reviewed the literature and present seven clinical cases of their own practice. Results The severity of clinical deficit correlates with the degree of morphological changes in the spinal cord, the level of dysgenesis, as well as with the presence of combined and concomitant developmental anomalies. Early decompression of neural structures and spine stabilization play an important role in improvement of treatment results but the immature bone structures in infants limit the possibilities of early instrumented fixation. Conclusion SSD is a specific developmental anomaly of the spine and spinal cord that features some clinical and radiological peculiarities. This anomaly fills the gap between the infant thoracolumbar kyphosis with segmental subluxation and the syndrome of caudal regression. Early diagnosis and individual approach to determination of an optimal period of surgical treatment and tactics are required in each case.