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Spinal curvature is a common concern, as it develops in more than 50% of children with spinal muscular atrophy, most commonly in those who are nonambulatory. This may be addressed by surgical correction or positional support (e.g. bracing). The decision to perform surgical correction of complications such as scoliosis is based on the child’s spine curvature, pulmonary function, and bone maturity.1,2

POTENTIAL BENEFITS

CONSIDERATIONS

SURGERY

POTENTIAL BENEFITS

  • May improve sitting posture, balance, endurance, and cosmesis2
  • May slow respiratory decline2,3
  • May increase overall comfort, quality of life, and independence1

CONSIDERATIONS

  • May impact ability to perform lumbar puncture
  • Optimal timing of procedure is controversial3
  • Individuals with spinal muscular atrophy may be at higher risk of surgical complications than the general population3

BRACING

POTENTIAL BENEFITS

  • May improve sitting balance, endurance, and overall physical appearance3
  • More conservative approach than surgery that allows for further growth3

CONSIDERATIONS

  • Likely does not prevent or delay development of scoliosis3
  • May cause some discomfort1
  • Lung function may be adversely affected by rigid bracing in children ≤8 years of age
  • Expiratory lung volume may be lower with bracing4

References

1. Spinal Muscular Atrophy Clinical Research Center. Physical/occupational therapy. http://columbiasma.org/pt-ot.html. Updated March 14, 2013. Accessed April 18, 2016. 2. Wang CH, Finkel RS, Bertini ES, et al; and Participants of the International Conference on SMA Standard of Care. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007;22(8):1027-1049. 3. Mullender M, Blom N, De Kleuver M, et al. A Dutch guideline for the treatment of scoliosis in neuromuscular disorders. Scoliosis. 2008;3:14. 4. Tangsrud SE, Carlsen KC, Lund-Petersen I, Carlsen KH. Lung function measurements in young children with spinal muscular atrophy; a cross sectional survey on the effect of position and bracing. Arch Dis Child. 2001;84(6):521-524. 5. Skalsky AJ, McDonald CM. Prevention and management of limb contractures in neuromuscular diseases. Phys Med Rehabil Clin N Am. 2012;23(3):675-687. 6. Tsirikos AI, Baker ADL. Spinal muscular atrophy: classification, aetiology, and treatment of spinal deformity in children and adolescents. Curr Orthoped. 2006;20:430-445. 7. Lin W, Pierce A, Skalsky AJ, McDonald CM. Mobility-assistive technology in progressive neuromuscular disease. Phys Med Rehabil Clin N Am. 2012;23(4):885-894. 8. Jones MA, McEwen IR, Hansen L. Use of power mobility for a young child with spinal muscular atrophy. Phys Ther. 2003;83(3):253-262. 9. Arva J, Paleg G, Lange M, et al. RESNA position on the application of wheelchair standing devices. Assist Technol. 2009;21(3):161-168. 10. Hilliard K. Special Effect’s controllers enable every gamer to enjoy video games. http://www.gameinformer.com/b/features/archive/2014/06/25/creating-controllers-for-all-disabilities-special-effect-enables-every-gamer-to-enjoy-video-games.aspx. Game Informer website. Published June 25, 2014. Accessed August 1, 2016. 

Muscular Atrophy

The clinical spectrum of SMA is highly variable and often requires comprehensive medical care involving multiple disciplines.2