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Spinal Pathology in Myelodysplasia

- Discussion:
     - Myelomeningocele Scoliosis may result from:
           - idiopathic causes;
           - myelomeningocele structural defect;
           - congenital defects
           - paralytic etiology;
- Complications:
    - Rapid Curve Progression:
         - may manifest as lower extremity spasticity;
         - MRI is helpful in evaluating these children;
         - Diff dx:
              - Hydrocephalus:
              - Tethered Cord Syndrome
              - Hydromyelia
              - Syringomyelia
              - Arnold Chiari Syndrome Compression
              - Distrubed Ventricular Shunt Mechanics
                     - is adressed by revising shunt or by releasing a tethered cord
- Orthotic Treatment:
    - attempts at bracing (TLSO) may fail & require subcutaneous rodding for very young children and fusion later;
- Operative Treatment of Scoliosis:
    - preop eval:
           - aggressive evaluation of urinary tract, treatment of urinary tract infection, & periop ATB are important in reducing wound infections;
           - treatment of the scoliosis without recognition of syringomyelia and Chiari malformation can lead to paraplegia;
    - paralytic Scoliosis:
           - treated w/ anterior arthrodesis combined w/ posterior arthrodesis and secure internal fixation, plus bone grafting;
    - Luque sublaminar wiring w/ fixation to sacrum;
           - curves > 60 deg require initial anterior release & fusion;
- Kyphosis in myelodyplasia:
     - resection of Kyphosis w/ local fusion or fusion to pelvis may be required;
     - surgery should be delayed as long as possible inorder to delay recurrence;
     - kyphectomy for severe congenital kyphosis in pts with thoracic-level Myelomeningocele is indicated for sitting imbalance or w/ skin
             problems occur over the apex;
     - patent shunt is essential to prevent acute hydrocephalus, which can result from spinal sac excision usually necessary with kyphectomy.