Correlation of anomalous vascular and osseous anatomy in congenital kyphosis - Type I - Case report

Congenital kyphosis Type I results from failure of formation of the vertebral body (VB). The clinical course is characterized by progressive deformity and neurological dysfunction unless the patient undergoes fusion. It has been postulated that failure of VB formation may be secondary to anomalies of the segmental arteries arising from the aorta. There is limited clinical evidence in the literature, however, to support this hypothesis. The authors report on the case of an adult patient with congenital kyphosis Type I in whom spinal angiography correlated vascular with vertebral anomalies. This 32-year-old man presented with back pain, lower-extremity paresthesias, and incomplete voiding, which had progressed over several years. Imaging revealed that the L-1 and L-2 vertebrae had not fully formed, causing kyphotie deformity such that the anterior portion of T-12 nearly articulated with that of L-3; this anomaly produced significant mobility in flexion and extension. Magnetic resonance imaging revealed distal spinal cord atrophy and signal change on T-2-weighted images. Spinal angiography showed that the right and left segmental arteries at both L- 1 and L-2 arose from a common trunk from the aorta. This was confirmed during subsequent T12-L3 anterior-posterior fusion. Postoperatively the patient experienced relief of pain and remained neurologically stable throughout a 3-year follow-up period. The presence of corresponding anomalies of the segmental arteries and vertebrae in this case of congenital kyphosis Type I indicates that a relationship may exist in the genesis of aberrant spinal vertebral and vascular structures.