The Journal of Bone and Joint Surgery, Vol 77, Issue 4 530-537, Copyright © 1995 by Journal of Bone and Joint Surgery, Inc
Segmental spinal dysgenesis. A disorder different from spinal agenesis
T Faciszewski, RB Winter, JE Lonstein, S Sane and D Erickson
Minnesota Spine Center, Minneapolis 55454-1419, USA.
We reviewed the clinical and roentgenographic findings, treatment, and
results for seventeen patients (six male and eleven female) who had
segmental spinal dysgenesis, a disorder frequently confused with, but
distinct from, lumbar and lumbosacral agenesis. The average age at the time
of presentation to the Minnesota Spine Center or the Gillette Children's
Hospital was two and a half years (range, newborn to twenty-one years), and
the average duration of follow-up 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 and four had weakness
of the lower extremities. An average of 2.6 procedures (range, one to five
procedures) was needed to obtain a solid fusion. 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 kyphosis, and stabilization of
neurological function were obtained in all patients. We recommend early
anterior and posterior arthrodesis in patients who have segmental spinal
dysgenesis, as the progressive kyphosis that inevitably develops often
results in neurological deficits.
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