Skip to main content

Introduction to Neuromuscular Scoliosis

  • Chapter
  • First Online:
Paediatric Scoliosis

Abstract

Neuromuscular spinal deformities are a heterogeneous group of conditions in which the pathogenesis of the underlying neurologic or muscular disorder determines the characteristics of each case. The incidence of scoliosis in neuromuscular diseases ranges between 25 and 90% compared to idiopathic scoliosis in 2 and 4% of the general population [1]. Spinal deformity usually appears at an early age, rapidly progresses, and progresses even after attaining skeletal maturity [2]. The treatment plan depends on the magnitude of the deformity, the patient’s quality of life, and the natural history of neuromuscular disease. Preoperative optimization of the patient’s comorbid conditions is crucial. Surgery is associated with high complication rates, repeated procedures, and prolonged hospitalization.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Sarwark J, Sarwahi V. New strategies and decision making in the management of neuromuscular scoliosis. Orthop Clin North Am. 2007;38(4):485–96, v.

    Article  PubMed  Google Scholar 

  2. McCarthy RE. Management of neuromuscular scoliosis. Orthop Clin North Am. 1999;30(3):435–49, viii.

    Article  CAS  PubMed  Google Scholar 

  3. Vialle R, Thévenin-Lemoine C, Mary P. Neuromuscular scoliosis. Orthop Traumatol Surg Res. 2013;99(1 Suppl):S124–39.

    Article  CAS  PubMed  Google Scholar 

  4. Williams BA, Matsumoto H, McCalla DJ, Akbarnia BA, Blakemore LC, Betz RR, et al. Development and initial validation of the classification of early-onset scoliosis (C-EOS). J Bone Joint Surg Am. 2014;96(16):1359–67.

    Article  PubMed  Google Scholar 

  5. Berven S, Bradford DS. Neuromuscular scoliosis: causes of deformity and principles for evaluation and management. Semin Neurol. 2002;22(2):167–78.

    Article  PubMed  Google Scholar 

  6. Barsdorf AI, Sproule DM, Kaufmann P. Scoliosis surgery in children with neuromuscular disease: findings from the US National Inpatient Sample, 1997 to 2003. Arch Neurol. 2010;67(2):231–5.

    Article  PubMed  Google Scholar 

  7. Lonstein JE, Akbarnia A. Operative treatment of spinal deformities in patients with cerebral palsy or mental retardation. An analysis of one hundred and seven cases. J Bone Joint Surg Am. 1983;65(1):43–55.

    Article  CAS  PubMed  Google Scholar 

  8. Dayer R, Ouellet JA, Saran N. Pelvic fixation for neuromuscular scoliosis deformity correction. Curr Rev Musculoskelet Med. 2012;5(2):91–101.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Mercado E, Alman B, Wright JG. Does spinal fusion influence quality of life in neuromuscular scoliosis? Spine (Phila Pa 1976). 2007;32(19 Suppl):S120–5.

    Article  PubMed  Google Scholar 

  10. Shore BJ, Yu X, Desai S, Selber P, Wolfe R, Graham HK. Adductor surgery to prevent hip displacement in children with cerebral palsy: the predictive role of the gross motor function classification system. J Bone Joint Surg Am. 2012;94(4):326–34.

    Article  PubMed  Google Scholar 

  11. Soo B, Howard JJ, Boyd RN, Reid SM, Lanigan A, Wolfe R, et al. Hip displacement in cerebral palsy. J Bone Joint Surg Am. 2006;88(1):121–9.

    PubMed  Google Scholar 

  12. Kilmer DD. The role of exercise in neuromuscular disease. Phys Med Rehabil Clin N Am. 1998;9(1):115–25, vi.

    Article  CAS  PubMed  Google Scholar 

  13. Olafsson Y, Saraste H, Al-Dabbagh Z. Brace treatment in neuromuscular spine deformity. J Pediatr Orthop. 1999;19(3):376–9.

    Article  CAS  PubMed  Google Scholar 

  14. Prior TW, Leach ME, Finanger E. Spinal muscular atrophy. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJ, Gripp KW, et al., editors. GeneReviews® [Internet]. Seattle: University of Washington; 1993 [cited 2022 May 29]. http://www.ncbi.nlm.nih.gov/books/NBK1352/.

  15. Downs J, Bergman A, Carter P, Anderson A, Palmer GM, Roye D, et al. Guidelines for management of scoliosis in Rett syndrome patients based on expert consensus and clinical evidence. Spine (Phila Pa 1976). 2009;34(17):E607–17.

    Article  PubMed  Google Scholar 

  16. Dooley JM, Gordon KE, MacSween JM. Impact of steroids on surgical experiences of patients with Duchenne muscular dystrophy. Pediatr Neurol. 2010;43(3):173–6.

    Article  PubMed  Google Scholar 

  17. Lebel DE, Corston JA, McAdam LC, Biggar WD, Alman BA. Glucocorticoid treatment for the prevention of scoliosis in children with Duchenne muscular dystrophy: long-term follow-up. J Bone Joint Surg Am. 2013;95(12):1057–61.

    Article  PubMed  Google Scholar 

  18. Houde S, Filiatrault M, Fournier A, Dubé J, D’Arcy S, Bérubé D, et al. Deflazacort use in Duchenne muscular dystrophy: an 8-year follow-up. Pediatr Neurol. 2008;38(3):200–6.

    Article  PubMed  Google Scholar 

  19. Murphy RF, Mooney JF. Current concepts in neuromuscular scoliosis. Curr Rev Musculoskelet Med. 2019;12(2):220–7.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Larsson ELC, Aaro SI, Normelli HCM, Oberg BE. Long-term follow-up of functioning after spinal surgery in patients with neuromuscular scoliosis. Spine (Phila Pa 1976). 2005;30(19):2145–52.

    Article  PubMed  Google Scholar 

  21. Obid P, Bevot A, Goll A, Leichtle C, WĂ¼lker N, Niemeyer T. Quality of life after surgery for neuromuscular scoliosis. Orthop Rev. 2013;5(1):e1.

    Google Scholar 

  22. Howard JJ, Farrelly J. Evidence-based treatment of neuromuscular scoliosis. In: Alshryda S, Huntley JS, Banaszkiewicz PA, editors. Paediatric orthopaedics: an evidence-based approach to clinical questions [Internet]. Cham: Springer International Publishing; 2017 [cited 2022 Mar 15]. p. 213–228. https://doi.org/10.1007/978-3-319-41142-2_24.

  23. Modi HN, Suh SW, Yang JH, Cho JW, Hong JY, Singh SU, et al. Surgical complications in neuromuscular scoliosis operated with posterior- only approach using pedicle screw fixation. Scoliosis. 2009;4:11.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Caekebeke P, Moke L, Moens P. Sublaminar devices for the correction of scoliosis: metal wire versus polyester tape. Acta Orthop Belg. 2013;79:216.

    PubMed  Google Scholar 

  25. Watanabe K, Lenke LG, Bridwell KH, Kim YJ, Watanabe K, Kim YW, et al. Comparison of radiographic outcomes for the treatment of scoliotic curves greater than 100 degrees: wires versus hooks versus screws. Spine (Phila Pa 1976). 2008;33(10):1084–92.

    Article  PubMed  Google Scholar 

  26. Sedra F, Shafafy R, Sadek AR, Aftab S, Montgomery A, Nadarajah R. Perioperative optimization of patients with neuromuscular disorders undergoing scoliosis corrective surgery: a multidisciplinary team approach. Global Spine J. 2021;11(2):240–8.

    Article  PubMed  Google Scholar 

  27. Lonstein JE, Koop SE, Novachek TF, Perra JH. Results and complications after spinal fusion for neuromuscular scoliosis in cerebral palsy and static encephalopathy using Luque Galveston instrumentation: experience in 93 patients. Spine (Phila Pa 1976). 2012;37(7):583–91.

    Article  PubMed  Google Scholar 

  28. Modi HN, Suh SW, Song HR, Fernandez HM, Yang JH. Treatment of neuromuscular scoliosis with posterior-only pedicle screw fixation. J Orthop Surg. 2008;3:23.

    Article  PubMed Central  Google Scholar 

  29. Mattila M, Jalanko T, Puisto V, Pajulo O, Helenius IJ. Hybrid versus total pedicle screw instrumentation in patients undergoing surgery for neuromuscular scoliosis: a comparative study with matched cohorts. J Bone Joint Surg Br. 2012;94(10):1393–8.

    Article  CAS  PubMed  Google Scholar 

  30. Allen BL, Ferguson RL. A 1988 perspective on the Galveston technique of pelvic fixation. Orthop Clin North Am. 1988;19(2):409–18.

    Article  PubMed  Google Scholar 

  31. Bell DF, Moseley CF, Koreska J. Unit rod segmental spinal instrumentation in the management of patients with progressive neuromuscular spinal deformity. Spine (Phila Pa 1976). 1989;14(12):1301–7.

    Article  CAS  PubMed  Google Scholar 

  32. Peelle MW, Lenke LG, Bridwell KH, Sides B. Comparison of pelvic fixation techniques in neuromuscular spinal deformity correction: Galveston rod versus iliac and lumbosacral screws. Spine (Phila Pa 1976). 2006;31(20):2392–8; discussion 2399.

    Article  PubMed  Google Scholar 

  33. Phillips JH, Gutheil JP, Knapp DR. Iliac screw fixation in neuromuscular scoliosis. Spine (Phila Pa 1976). 2007;32(14):1566–70.

    Article  PubMed  Google Scholar 

  34. Chang TL, Sponseller PD, Kebaish KM, Fishman EK. Low profile pelvic fixation: anatomic parameters for sacral alar-iliac fixation versus traditional iliac fixation. Spine (Phila Pa 1976). 2009;34(5):436–40.

    Article  PubMed  Google Scholar 

  35. Wu Z, Schwend RM, Anderson JT, Marasigan JAM, Price NJ. Iliac screw instrumentation to the pelvis in children with neuromuscular and syndromic scoliosis. No lateral connectors and respect sagittal balance. Spine Deform. 2021;9(4):1115–23.

    Article  PubMed  Google Scholar 

  36. Modi HN, Suh SW, Song HR, Yang JH, Jajodia N. Evaluation of pelvic fixation in neuromuscular scoliosis: a retrospective study in 55 patients. Int Orthop. 2010;34(1):89–96.

    Article  PubMed  Google Scholar 

  37. Mehta SS, Modi HN, Srinivasalu S, Suh SW, Yi JW, Cho JW, et al. Pedicle screw-only constructs with lumbar or pelvic fixation for spinal stabilization in patients with Duchenne muscular dystrophy. J Spinal Disord Tech. 2009;22(6):428–33.

    Article  PubMed  Google Scholar 

  38. Saito W, Inoue G, Shirasawa E, Imura T, Nakazawa T, Miyagi M, et al. Limitations of posterior spinal fusion to L5 for flaccid neuromuscular scoliosis focusing on pelvic obliquity. Spine Deform. 2021;9(2):559–65.

    Article  PubMed  Google Scholar 

  39. Wild A, Haak H, Kumar M, Krauspe R. Is sacral instrumentation mandatory to address pelvic obliquity in neuromuscular thoracolumbar scoliosis due to myelomeningocele? Spine (Phila Pa 1976). 2001;26(14):E325–9.

    Article  CAS  PubMed  Google Scholar 

  40. McCall RE, Hayes B. Long-term outcome in neuromuscular scoliosis fused only to lumbar 5. Spine (Phila Pa 1976). 2005;30(18):2056–60.

    Article  PubMed  Google Scholar 

  41. Shao ZX, Fang X, Lv QB, Hu ZC, Shao SY, Hu YB, et al. Comparison of combined anterior-posterior approach versus posterior-only approach in neuromuscular scoliosis: a systematic review and meta-analysis. Eur Spine J. 2018;27(9):2213–22.

    Article  PubMed  Google Scholar 

  42. Moon ES, Nanda A, Park JO, Moon SH, Lee HM, Kim JY, et al. Pelvic obliquity in neuromuscular scoliosis: radiologic comparative results of single-stage posterior versus two-stage anterior and posterior approach. Spine (Phila Pa 1976). 2011;36(2):146–52.

    Article  PubMed  Google Scholar 

  43. Tsirikos AI, Chang WN, Dabney KW, Miller F. Comparison of one-stage versus two-stage anteroposterior spinal fusion in pediatric patients with cerebral palsy and neuromuscular scoliosis. Spine (Phila Pa 1976). 2003;28(12):1300–5.

    Article  PubMed  Google Scholar 

  44. LaMothe JM, Al Sayegh S, Parsons DL, Ferri-de-Barros F. The use of intraoperative traction in pediatric scoliosis surgery: a systematic review. Spine Deform. 2015;3(1):45–51.

    Article  CAS  PubMed  Google Scholar 

  45. Hammett TC, Boreham B, Quraishi NA, Mehdian SMH. Intraoperative spinal cord monitoring during the surgical correction of scoliosis due to cerebral palsy and other neuromuscular disorders. Eur Spine J. 2013;22(Suppl 1):S38–41.

    Article  PubMed  Google Scholar 

  46. Fehlings MG, Kelleher MO. Intraoperative monitoring during spinal surgery for neuromuscular scoliosis. Nat Clin Pract Neurol. 2007;3(6):318–9.

    Article  PubMed  Google Scholar 

  47. Reames DL, Smith JS, Fu KMG, Polly DW, Ames CP, Berven SH, et al. Complications in the surgical treatment of 19,360 cases of pediatric scoliosis: a review of the Scoliosis Research Society morbidity and mortality database. Spine (Phila Pa 1976). 2011;36(18):1484–91.

    Article  PubMed  Google Scholar 

  48. Kannan S, Meert KL, Mooney JF, Hillman-Wiseman C, Warrier I. Bleeding and coagulation changes during spinal fusion surgery: a comparison of neuromuscular and idiopathic scoliosis patients. Pediatr Crit Care Med. 2002;3(4):364–9.

    Article  PubMed  Google Scholar 

  49. Brenn BR, Theroux MC, Dabney KW, Miller F. Clotting parameters and thromboelastography in children with neuromuscular and idiopathic scoliosis undergoing posterior spinal fusion. Spine (Phila Pa 1976). 2004;29(15):E310–4.

    Article  PubMed  Google Scholar 

  50. Kasimian S, Skaggs DL, Sankar WN, Farlo J, Goodarzi M, Tolo VT. Aprotinin in pediatric neuromuscular scoliosis surgery. Eur Spine J. 2008;17(12):1671–5.

    Article  PubMed  PubMed Central  Google Scholar 

  51. Vitale MG, Privitera DM, Matsumoto H, Gomez JA, Waters LM, Hyman JE, et al. Efficacy of preoperative erythropoietin administration in pediatric neuromuscular scoliosis patients. Spine (Phila Pa 1976). 2007;32(24):2662–7.

    Article  PubMed  Google Scholar 

  52. Sponseller PD, LaPorte DM, Hungerford MW, Eck K, Bridwell KH, Lenke LG. Deep wound infections after neuromuscular scoliosis surgery: a multicenter study of risk factors and treatment outcomes. Spine (Phila Pa 1976). 2000;25(19):2461–6.

    Article  CAS  PubMed  Google Scholar 

  53. Duckworth AD, Mitchell MJ, Tsirikos AI. Incidence and risk factors for postoperative complications after scoliosis surgery in patients with Duchenne muscular dystrophy: a comparison with other neuromuscular conditions. Bone Joint J. 2014;96-B(7):943–9.

    Article  CAS  PubMed  Google Scholar 

  54. Ersberg A, Gerdhem P. Pre- and postoperative quality of life in patients treated for scoliosis. Acta Orthop. 2013;84(6):537–43.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to K. V. Nikhil .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2023 The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd.

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Nikhil, K.V., Vinod, V., Abraham, G. (2023). Introduction to Neuromuscular Scoliosis. In: Zacharia, B., Raja, S.D.C., KV, N. (eds) Paediatric Scoliosis . Springer, Singapore. https://doi.org/10.1007/978-981-99-3017-3_32

Download citation

  • DOI: https://doi.org/10.1007/978-981-99-3017-3_32

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-99-3016-6

  • Online ISBN: 978-981-99-3017-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics