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Updated: Dec 18 2016

Neuromuscular Scoliosis

Introduction
  • Defined as an irregular spinal curvature caused by disorders of the brain, spinal cord, and muscular system.
  • Neurogenic curves (relative to idiopathic curves) tend to be
    • more rapidly progressive
    • may progress after maturity
    • associated with pelvic obliquity
    • are longer and involve more vertebrae (may involve cervical vertebrae)
    • have a higher rate of pulmonary complications with surgery
Classification & Treatment
  • Outcomes
    • surgical correction of spinal fusion is only treatment that has a documented beneficial impact on deformity
    • parents and caretakers report excellent improvement in the child's quality of life after deformity correction
    • Increased risk of wound complications with: 
      • Poor nutritional status (serum albumin <3.5 g/dL)
      • Immunocompromised status (WBC <1,500 cells/ucL)
      • Presence of a ventriculoperitoneal (VP) shunt
      • Severe spastic quadriplegia nonambulatory status with seizures
Classification and Treatment
(for more detail go to disease content)
Category
Disease
Nonoperative Treatment
Operative Treatment
Upper motor neuron Cerebral palsy  Boston-type underarm bracing until puberty (age 10-12) and wheelchair modification
  • Group I treat with PSF with instumentation
  • Group II treat with PSF +/- ASF with instrumentation and fusion to pelvis (Luque-Galveston)
  • Indications for surgery
    • curve > 50°
    • worsening pelvic obliquity with sitting imbalance
  Rett Syndrome   Bracing for C-shaped curves
  • Bracing
    • C shaped curves
  • Posterior Spinal Fusion indications
    • thoracic curve that intefers with sitting and balance
    • C shaped curves that do not respond to bracing
Muscle Weakness Spinal muscular atrophy  Boston-type underarm bracing until puberty (age 10-12)
  • Treat with PSF with fusion to pelvis for improved wheelchair sitting
    • address hip contractures before PSF
    • may lead to temporary loss of upper extremity function
Muscular dystrophy  bracing is contraindicated
  • PSF with or without fusion to pelvis (fusion to pelvis is controversial)
    • indications
      • absolute curve > 20°
      • progressive curve
    • Treat early before pulmonary function declines (curve from 20 to 30°)
Paralytic Syndromes Spinal bifida and spinal cord injuries  bracing is contraindicated
  • ASF/PSF with instumentation and pelvic fixation
    • anterior fusion required because minimal posterior element to obtain fusion
Polio  Boston-type underarm bracing until puberty (age 10-12)
  • PSF

 
 
 
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