Early onset scoliosis: current concepts and controversies

  • Nicholas D. FletcherEmail author
  • Robert W. Bruce
Pediatrics (M Glotzbecker, Section Editor)


Early Onset Scoliosis (EOS) may be associated with long-term pulmonary morbidity, which is not commonly seen in Adolescent Idiopathic Scoliosis. Initial evaluation is based on determining any underlying etiology related to congenital or syndromic conditions. Assessing the impact of scoliosis on thoracic development may help guide treatment, which is often required at a young age in these children to prevent irreversible pulmonary insufficiency. Treatment is based on multiple factors but may include non-surgical strategies, such as casting or bracing, along with growth-sparing surgical procedures using growing rods or chest wall expansion. Definitive fusion is rarely indicated in young patients. This chapter will cover the diagnosis, evaluation, and treatment of children with EOS.


Scoliosis Early onset scoliosis Thoracic insufficiency syndrome Infantile scoliosis Juvenile scoliosis Bracing Serial casting Mehta casting Risser casting Growing rods VEPTR Expansion thoracoplasty Pediatrics 



No potential conflicts of interest relevant to this article were reported.


Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. 1.
    Dickson RA. Early Onset Idiopahic Scoliosis, vol. 1. New York: Raven; 1994.Google Scholar
  2. 2.
    Redding GJ. Thoracic Insufficiency Syndrome. Vol 1: Springer; 2010.Google Scholar
  3. 3.
    Sharp JT, Druz WS, Balagot RC, et al. Total respiratory compliance in infants and children. J Appl Physiol. 1970;29(6):775–9.PubMedGoogle Scholar
  4. 4.
    Mechanisms and limits of induced postnatal lung growth. Am J Respir Crit Care Med. Aug 1 2004;170(3):319–343.Google Scholar
  5. 5.
    Campbell Jr RM, Smith MD, Mayes TC, et al. The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis. J Bone Joint Surg Am. 2003;85-A(3):399–408.PubMedGoogle Scholar
  6. 6.
    Corona J, Matsumoto H, Roye DP, Vitale MG. Measuring quality of life in children with early onset scoliosis: development and initial validation of the early onset scoliosis questionnaire. J Pediatr Orthop. 2011;31(2):180–5.PubMedCrossRefGoogle Scholar
  7. 7.
    • Vitale MG, Matsumoto H, Roye DP, Jr., et al. Health-related quality of life in children with thoracic insufficiency syndrome. J Pediatr Orthop. Mar 2008;28(2):239–243. This study found that children with EOS have health-related quality of life scores similar to or lower than children with asthma, epilepsy, heart disease, or childhood cancer.PubMedCrossRefGoogle Scholar
  8. 8.
    Caubet JF, Emans JB, Smith JT, et al. Increased hemoglobin levels in patients with early onset scoliosis: prevalence and effect of a treatment with Vertical Expandable Prosthetic Titanium Rib (VEPTR). Spine (Phila Pa 1976). 2009;34(23):2534–6.CrossRefGoogle Scholar
  9. 9.
    Striegl A, Chen ML, Kifle Y, et al. Sleep-disordered breathing in children with thoracic insufficiency syndrome. Pediatr Pulmonol. 2010;45(5):469–74.PubMedCrossRefGoogle Scholar
  10. 10.
    Cobb J. Outline for the study of scoliosis. Ann Arbor: J.W. Edwards; 1948.Google Scholar
  11. 11.
    Karol LA, Johnston C, Mladenov K, et al. Pulmonary function following early thoracic fusion in non-neuromuscular scoliosis. J Bone Joint Surg Am. 2008;90(6):1272–81.PubMedCrossRefGoogle Scholar
  12. 12.
    Mehta MH. Radiographic estimation of vertebral rotation in scoliosis. J Bone Joint Surg Br. 1973;55(3):513–20.PubMedGoogle Scholar
  13. 13.
    Mehta MH. The rib-vertebra angle in the early diagnosis between resolving and progressive infantile scoliosis. J Bone Joint Surg Br. 1972;54(2):230–43.PubMedGoogle Scholar
  14. 14.
    Abul-Kasim K, Overgaard A, Maly P, et al. Low-dose helical computed tomography (CT) in the perioperative workup of adolescent idiopathic scoliosis. Eur Radiol. 2009;19(3):610–8.PubMedCrossRefGoogle Scholar
  15. 15.
    Gollogly S, Smith JT, Campbell RM. Determining lung volume with three-dimensional reconstructions of CT scan data: A pilot study to evaluate the effects of expansion thoracoplasty on children with severe spinal deformities. J Pediatr Orthop. 2004;24(3):323–8.PubMedCrossRefGoogle Scholar
  16. 16.
    Gollogly S, Smith JT, White SK, et al. The volume of lung parenchyma as a function of age: a review of 1050 normal CT scans of the chest with three-dimensional volumetric reconstruction of the pulmonary system. Spine (Phila Pa 1976). 2004;29(18):2061–6.CrossRefGoogle Scholar
  17. 17.
    Dobbs MB, Lenke LG, Szymanski DA, et al. Prevalence of neural axis abnormalities in patients with infantile idiopathic scoliosis. J Bone Joint Surg Am. 2002;84-A(12):2230–4.PubMedGoogle Scholar
  18. 18.
    Belmont Jr PJ, Kuklo TR, Taylor KF, et al. Intraspinal anomalies associated with isolated congenital hemivertebra: the role of routine magnetic resonance imaging. J Bone Joint Surg Am. 2004;86-A(8):1704–10.PubMedGoogle Scholar
  19. 19.
    Rajasekaran S, Kamath V, Kiran R, Shetty AP. Intraspinal anomalies in scoliosis: An MRI analysis of 177 consecutive scoliosis patients. Indian J Orthop. 2010;44(1):57–63.PubMedCrossRefGoogle Scholar
  20. 20.
    Pahys JM, Samdani AF, Betz RR. Intraspinal anomalies in infantile idiopathic scoliosis: prevalence and role of magnetic resonance imaging. Spine (Phila Pa 1976). 2009;34(12):E434–8.CrossRefGoogle Scholar
  21. 21.
    Gupta P, Lenke LG, Bridwell KH. Incidence of neural axis abnormalities in infantile and juvenile patients with spinal deformity. Is a magnetic resonance image screening necessary? Spine (Phila Pa 1976). 1998;23(2):206–10.CrossRefGoogle Scholar
  22. 22.
    Smith JT, Dubosset, J. Imaging of the Growing Spine: Springer; 2010.Google Scholar
  23. 23.
    Campbell RMJ. Early Onset Scoliosis. Paper presented at: 4th Annual International Congress on Early Onset Scoliosis (ICEOS)2010; Toronto, Canada.Google Scholar
  24. 24.
    Goldberg CJ, Gillic I, Connaughton O, et al. Respiratory function and cosmesis at maturity in infantile-onset scoliosis. Spine (Phila Pa 1976). 2003;28(20):2397–406.CrossRefGoogle Scholar
  25. 25.
    Emans JB. Orthotic management of infantile and juvenile scoliosis. In: Akbarnia BAY, Muharrem; Thompson, George H, ed. The Growing Spine: Springer; 2010:372–388.Google Scholar
  26. 26.
    Mehta MH. Growth as a corrective force in the early treatment of progressive infantile scoliosis. J Bone Joint Surg Br. 2005;87(9):1237–47.PubMedCrossRefGoogle Scholar
  27. 27.
    • Sankar WN, Skaggs DL, Yazici M, et al. Lengthening of dual growing rods and the law of diminishing returns. Spine (Phila Pa 1976). May 1 2011;36(10):806–809. This study showed that the average T1-S1 length gained from a given growing rod lengthening decreased significantly with repeated lengthenings.CrossRefGoogle Scholar
  28. 28.
    D’Astous JL, Sanders JO. Casting and traction treatment methods for scoliosis. Orthop Clin North Am. 2007;38(4):477–84. v.PubMedCrossRefGoogle Scholar
  29. 29.
    Fletcher ND, McClung A, Rathjen KE, et al. Serial Casting as a delay tactic in the management of moderate to severe scoliosis. J Pediatr Orthop. In press 2011.Google Scholar
  30. 30.
    Luque ER. Paralytic scoliosis in growing children. Clin Orthop Relat Res. 1982;163:202–9.PubMedGoogle Scholar
  31. 31.
    Moe JH, Kharrat K, Winter RB, Cummine JL. Harrington instrumentation without fusion plus external orthotic support for the treatment of difficult curvature problems in young children. Clin Orthop Relat Res. 1984;185:35–45.PubMedGoogle Scholar
  32. 32.
    Harrington PR. Treatment of scoliosis. Correction and internal fixation by spine instrumentation. J Bone Joint Surg Am. 1962;44-A:591–610.PubMedGoogle Scholar
  33. 33.
    Akbarnia BA, Marks DS, Boachie-Adjei O, et al. Dual growing rod technique for the treatment of progressive early-onset scoliosis: a multicenter study. Spine (Phila Pa 1976). 2005;30(17 Suppl):S46–57.CrossRefGoogle Scholar
  34. 34.
    Akbarnia BA. Instrumentation with limited arthrodesis for the treatment of progressive early-onset scoliosis. Spine: State Art Rev. 2000;14(1):181–9.Google Scholar
  35. 35.
    Thompson GH, Akbarnia BA, Kostial P, et al. Comparison of single and dual growing rod techniques followed through definitive surgery: a preliminary study. Spine (Phila Pa 1976). 2005;30(18):2039–44.CrossRefGoogle Scholar
  36. 36.
    Bess S, Akbarnia BA, Thompson GH, et al. Complications of growing-rod treatment for early-onset scoliosis: analysis of one hundred and forty patients. J Bone Joint Surg Am. 2010;92(15):2533–43.PubMedCrossRefGoogle Scholar
  37. 37.
    • Noordeen HM, Shah SA, Elsebaie HB, et al. In vivo distraction force and length measurements of growing rods: Which factors influence on the ability to lengthen? Spine (Phila Pa 1976). Apr 7 2011. Similar to the above study, the authors found an increase distraction force required to achieve any additional length with repeated growing rod lengthenings. This was particularly notable after the 5 th lengthening.Google Scholar
  38. 38.
    Yang JS, McElroy MJ, Akbarnia BA, et al. Growing rods for spinal deformity: characterizing consensus and variation in current use. J Pediatr Orthop. 2010;30(3):264–70.PubMedCrossRefGoogle Scholar
  39. 39.
    Sankar WN, Acevedo DC, Skaggs DL. Comparison of complications among growing spinal implants. Spine (Phila Pa 1976). 2010;35(23):2091–6.Google Scholar
  40. 40.
    Farooq N, Garrido E, Altaf F, et al. Minimizing complications with single submuscular growing rods: a review of technique and results on 88 patients with minimum two-year follow-up. Spine (Phila Pa 1976). 2010;35(25):2252–8.CrossRefGoogle Scholar
  41. 41.
    Lee C, Myung KS, Skaggs DL. Proximal Junctional Kyphosis in Distraction-based Growing Rods. Annual meeting of the Scoliosis Research Society. Louisville, KY2011.Google Scholar
  42. 42.
    Campbell Jr RM, Hell-Vocke AK. Growth of the thoracic spine in congenital scoliosis after expansion thoracoplasty. J Bone Joint Surg Am. 2003;85-A(3):409–20.PubMedGoogle Scholar
  43. 43.
    Campbell Jr RM, Smith MD. Thoracic insufficiency syndrome and exotic scoliosis. J Bone Joint Surg Am. 2007;89 Suppl 1:108–22.PubMedCrossRefGoogle Scholar
  44. 44.
    Campbell Jr RM. Spine deformities in rare congenital syndromes: clinical issues. Spine (Phila Pa 1976). 2009;34(17):1815–27.CrossRefGoogle Scholar
  45. 45.
    Vitale MG, Gomez JA, Matsumoto H, Roye Jr DP. Variability of expert opinion in treatment of early-onset scoliosis. Clin Orthop Relat Res. 2011;469(5):1317–22.PubMedCrossRefGoogle Scholar
  46. 46.
    Emans JB, Caubet JF, Ordonez CL, et al. The treatment of spine and chest wall deformities with fused ribs by expansion thoracostomy and insertion of vertical expandable prosthetic titanium rib: growth of thoracic spine and improvement of lung volumes. Spine (Phila Pa 1976). 2005;30(17 Suppl):S58–68.CrossRefGoogle Scholar
  47. 47.
    Flynn JM, Ramirez N, Emans JB, et al. Is the vertebral expandable prosthetic titanium rib a surgical alternative in patients with spina bifida? Clin Orthop Relat Res. 2011;469(5):1291–6.PubMedCrossRefGoogle Scholar
  48. 48.
    Smith JT, Novais E. Treatment of Gibbus deformity associated with myelomeningocele in the young child with use of the vertical expandable prosthetic titanium rib (VEPTR): a case report. J Bone Joint Surg Am. 2010;92(12):2211–5.PubMedCrossRefGoogle Scholar
  49. 49.
    White KK, Song KM, Frost N, Daines BK. VEPTR growing rods for early-onset neuromuscular scoliosis: feasible and effective. Clin Orthop Relat Res. 2011;469(5):1335–41.PubMedCrossRefGoogle Scholar
  50. 50.
    Hasler CC, Mehrkens A, Hefti F. Efficacy and safety of VEPTR instrumentation for progressive spine deformities in young children without rib fusions. Eur Spine J. 2010;19(3):400–8.PubMedCrossRefGoogle Scholar
  51. 51.
    Reinker K, Simmons JW, Patil V, Stinson Z. Can VEPTR((R)) control progression of early-onset kyphoscoliosis? A cohort study of VEPTR((R)) patients with severe kyphoscoliosis. Clin Orthop Relat Res. 2011;469(5):1342–8.PubMedCrossRefGoogle Scholar
  52. 52.
    Skaggs DL, Sankar WN, Albrektson J, et al. Weight gain following vertical expandable prosthetic titanium ribs surgery in children with thoracic insufficiency syndrome. Spine (Phila Pa 1976). 2009;34(23):2530–3.CrossRefGoogle Scholar
  53. 53.
    • Redding GJ, Mayer OH. Structure-respiration function relationships before and after surgical treatment of early-onset scoliosis. Clin Orthop Relat Res. May 2011;469(5):1330–1334. This study found that ventillation and perfusion of the lung do not normalize after chest wall expansion surgery. These results highlight the difficulty in interpreting the pulmonary benefits of growth sparing spinal surgery.PubMedCrossRefGoogle Scholar
  54. 54.
    Mayer OH, Redding G. Early changes in pulmonary function after vertical expandable prosthetic titanium rib insertion in children with thoracic insufficiency syndrome. J Pediatr Orthop. 2009;29(1):35–8.PubMedCrossRefGoogle Scholar
  55. 55.
    Campbell Jr RM, Smith MD, Mayes TC, et al. The effect of opening wedge thoracostomy on thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis. J Bone Joint Surg Am. 2004;86-A(8):1659–74.PubMedGoogle Scholar
  56. 56.
    Smith JT. Bilateral rib-to-pelvis technique for managing early-onset scoliosis. Clin Orthop Relat Res. 2011;469(5):1349–55.PubMedCrossRefGoogle Scholar
  57. 57.
    Yaszay B, O’Brien M, Shufflebarger HL, et al. Efficacy of hemivertebra resection for congenital scoliosis: a multicenter retrospective comparison of three surgical techniques. Spine (Phila Pa 1976). 2011;36(24):2052–60.CrossRefGoogle Scholar
  58. 58.
    Ginsburg G, Mulconrey DS, Browdy J. Transpedicular hemiepiphysiodesis and posterior instrumentation as a treatment for congenital scoliosis. J Pediatr Orthop. 2007;27(4):387–91.PubMedCrossRefGoogle Scholar
  59. 59.
    Ruf M, Harms J. Posterior hemivertebra resection with transpedicular instrumentation: early correction in children aged 1 to 6 years. Spine (Phila Pa 1976). 2003;28(18):2132–8.CrossRefGoogle Scholar
  60. 60.
    Trobisch PD, Samdani A, Cahill P, Betz RR. Vertebral body stapling as an alternative in the treatment of idiopathic scoliosis. Oper Orthop Traumatol. 2011;23(3):227–31.PubMedCrossRefGoogle Scholar
  61. 61.
    Betz RR, Ranade A, Samdani AF, et al. Vertebral body stapling: a fusionless treatment option for a growing child with moderate idiopathic scoliosis. Spine (Phila Pa 1976). 2010;35(2):169–76.CrossRefGoogle Scholar
  62. 62.
    Betz RR, D’Andrea LP, Mulcahey MJ, Chafetz RS. Vertebral body stapling procedure for the treatment of scoliosis in the growing child. Clin Orthop Relat Res. 2005;434:55–60.PubMedCrossRefGoogle Scholar
  63. 63.
    Betz RR, Kim J, D’Andrea LP, et al. An innovative technique of vertebral body stapling for the treatment of patients with adolescent idiopathic scoliosis: a feasibility, safety, and utility study. Spine (Phila Pa 1976). 2003;28(20):S255–65.CrossRefGoogle Scholar
  64. 64.
    Newton PO, Upasani VV, Farnsworth CL, et al. Spinal growth modulation with use of a tether in an immature porcine model. J Bone Joint Surg Am. 2008;90(12):2695–706.PubMedCrossRefGoogle Scholar
  65. 65.
    Patel A, Schwab F, Lafage R, et al. Does removing the spinal tether in a porcine scoliosis model result in persistent deformity? A pilot study. Clin Orthop Relat Res. 2011;469(5):1368–74.PubMedCrossRefGoogle Scholar
  66. 66.
    Upasani VV, Farnsworth CL, Chambers RC, et al. Intervertebral disc health preservation after six months of spinal growth modulation. J Bone Joint Surg Am. 2011;93(15):1408–16.PubMedCrossRefGoogle Scholar
  67. 67.
    Luque ER. The anatomic basis and development of segmental spinal instrumentation. Spine (Phila Pa 1976). 1982;7(3):256–9.CrossRefGoogle Scholar
  68. 68.
    Luque ER. Segmental spinal instrumentation for correction of scoliosis. Clin Orthop Relat Res. 1982;163:192–8.PubMedGoogle Scholar
  69. 69.
    McCarthy RE, McCullough F, Luhmann SJ, Lenke LG. Greater than two years follow up Shilla growth enhancing system for the treatment of scoliosis in children. 2nd annual International Conference on Early Onset Scoliosis (ICEOS). Montreal, Canada2008.Google Scholar
  70. 70.
    McCarthy RE, Sucato D, Turner JL, et al. Shilla growing rods in a caprine animal model: a pilot study. Clin Orthop Relat Res. 2010;468(3):705–10.PubMedCrossRefGoogle Scholar
  71. 71.
    Akbarnia BA, Mundis GM, Jr., Salari P, et al. Innovation in Growing Rod Technique: A Study of Safety and Efficacy of a Magnetically Controlled Growing Rod in a Porcine Model*. Spine (Phila Pa 1976). Dec 3 2011.Google Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Emory University Orthopaedics and Spine CenterAtlantaUSA

Personalised recommendations