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Tethered Cord Syndrome

- 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 Pierz K, 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.