SEGMENTAL SPINAL DYSGENESIS - A DISORDER DIFFERENT FROM SPINAL AGENESIS

We reviewed the clinical and roentgenographic findings, treatment, and results for seventeen patients (six male and eleven female) who had s egmental spinal dysgenesis, a disorder frequently confused with, but d istinct from, lumbar and lumbosacral agenesis. The average age at the time of presentation to the Minnesota Spine Center or the Gillette Chi ldren's Hospital was two and a half years (range, newborn to twenty-on e years), and the average duration of followup was eight years (range, five months to twenty-two years). At the time of the diagnosis, eight patients had neurological deficits: seven had a neurogenic bladder an d four had weakness of the lower extremities. An average of 2.6 proced ures (range, one to five procedures) was needed to obtain a solid fusi on. Decompression of the stenotic canal was performed in ten patients, and it was followed by an improvement in neurological function in two of them. A solid fusion of the spine, arrest of the progressive kypho sis, and stabilization of neurological function were obtained in all p atients. We recommend early anterior and posterior arthrodesis in pati ents who have segmental spinal dysgenesis, as the progressive kyphosis that inevitably develops often results in neurological deficits.