Tethered Cord Syndrome

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- Discussion:
    - tethered cord syndrome is more frequent in children who have a myelomeningocele
            at level of L4 & L5 as opposed to those who have a lesion at thoracic level;
    - diff dx:
          - hydromyelia:
                - while both a syrinx and tethered cord can cause scoliosis a
                        functioning ventriculoperitoneal shunt will tend to rule out
                        hydromyelia as a cause of an evolving scoliosis;

- Clinical Findings:
    - most common in children between 1 & 2 years;
    - increased lumbar lordosis;
    - back & buttock pain
    - spasticity in the sacral roots;
    - scoliosis;
            - may see rapid progression of scoliosis;
            - tethered cord may cause scoliosis is above level of paralysis or above
                  open defect of meningocele unlike a paralytic scoliosis;
            - development of scoliosis at young age may indicate tethered cord;

- Radiology:
    - MRI is effective in identifying hydromyelia & syringomyelia, but it is
          not as definitive in determining whether spinal cord is tethered;
    - CT - myelography is more accurate in identifying area of tethering;

- Treatment:
    - release may not result in improvement or resolution of scoliosis, but does arrest its progression
            and may facilitate non-op management;
    - in the report by K Pierz et al 2000, 21 patients with spinal dysraphism and scoliosis who had undergone
            a detethering procedure were retrospectively reviewed;
            - 3 patients had thoracic, 12 had lumbar, and six had sacral level myelomeningocele;
            - 3 patients (14%) had curves that improved, and six (29%) stabilized.
            - 12 patients (57%) progressed >10 degrees.
            - 86 percent of patients with initial curves of >40 degrees and 100% of thoracic level
                    patients went on to require spinal fusion;
            - 8 complications occurred in six patients;
            - although patients with curves <40 degrees may have some improvement after a detethering procedure, those
                    presenting with curves >40 degrees or thoracic neurologic levels had no improvement in their scoliosis.
            - ref: The effect of tethered cord release on scoliosis in myelomeningocele.
                          Pierz K, Banta J, Thomson J, Gahm N, Hartford J.   J Pediatr Orthop 2000 May-Jun;20(3):362-5

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