Segmental spinal dysgenesis: early neurologic deterioration and treatment.

Segmental spinal dysgenesis is a rare congenital condition of the lumbar or thoracolumbar spine that can be associated with significant progressive or permanent neurologic defects, including paraplegia. In the past, both bracing and surgery have been recommended. Of the seven children we have seen with this problem, three have lost motor function between presentation and the time of surgery. Our experience suggests that to prevent catastrophic neurologic deterioration, surgery is indicated once the diagnosis is made.