Spina Bifida: The Management of Extremity Deformities in Myelomeningocele

  • Bülent Erol
  • Junichi Tamai


The spectrum of congenital neural tube defects, referred to as myelomeningocele (spina bifida), produces a wide variety of disabilities, ranging from benign defects in the skin of the back to devastating paralysis and deformity of the lower extremities, urinary and fecal incontinence, hydrocephalus, spasticity, and mental retardation. The care of children with myelomeningocele usually requires a multidisciplinary approach. Early intervention by a team of trained caregivers can significantly improve the disabilities and permit many children to be integrated into normal social environments with high levels of independence. This chapter describes the overall management of myelomeningocele with special emphasis on the orthopedic management.


Spina Bifida Stance Phase Flexion Contracture Pressure Sore Pelvic Obliquity 
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  1. 1.
    Lindseth RE (2001) Myelomeningocele. In: Morrissy RT, Weinstein SL (eds) Lovell and Winter’ s pediatric ortopaedics. Lippincott Williams & Wilkins, Philadelphia, pp 601–632Google Scholar
  2. 2.
    Lim R, Dias L, Vankoski S et al (1998) Valgus knee stress in lumbosacral myelomeningocele: a gait-analysis evaluation. J Pediatr Orthop 18:428–433PubMedCrossRefGoogle Scholar
  3. 3.
    Thomson JD, Ounpuu S, Davis RB et al (1999) The effects of ankle-foot orthoses on the ankle and knee in persons with myelomeningocele: an evaluation using threedimensional gait analysis. J Pediatr Orthop 19:27–33PubMedCrossRefGoogle Scholar
  4. 4.
    Vankoski S, Moore C, Statler KD et al (1997) The influence of forearm crutches on pelvic and hip kinematics in children with myelomeningocele: don’ t throw away crutches. Dev Med Child Neurol 39:614–619PubMedGoogle Scholar
  5. 5.
    Banta JV, Bonanni C, Prebluda J (1993) Latex anaphylaxis during spinal surgery in children with myelomeningocele. Dev Med Child Neurol 35:543–548PubMedGoogle Scholar
  6. 6.
    Mazon A, Nieto A, Estornell F et al (1997) Factors that influence the presence of symptoms caused by latex allergy in children with spina bifida. J Allergy Clin Immunology 99:600–604CrossRefGoogle Scholar
  7. 7.
    Tosi LL, Slater JE, Shaer C et al (1993) Latex allergy in spina bifida patients: prevalence and surgical implications. J Pediatr Orthop 13:709–712PubMedGoogle Scholar
  8. 8.
    Alenius H, Palosuo T, Kelly K et al (1993) Ig E reactivity to 14-kD and 27-kD natural rubber proteins in latex-allergic children with spina bifida and other congenital anomalies. Int Arch Allergy Immunol 102:61–66PubMedCrossRefGoogle Scholar
  9. 9.
    Degenhardt P, Golla S, Wahn F et al (2001) Latex allergy in pediatric surgery is dependent on repeated operations in the first year of life. J Pediatr Surg 36:1535–1539PubMedCrossRefGoogle Scholar
  10. 10.
    Herring JA (2002) Tachdjian’ s pediatric orthopaedics. WB Saunders Company, PhiladelphiaGoogle Scholar
  11. 11.
    Roye BD, Davidson RS (2004) Spina Bifida. In: Dormans JP (ed) Pediatric ortopaedics and sports medicine. The requisites in pediatrics. Mosby, Philadelphia, pp 417–436Google Scholar
  12. 12.
    Mazur JM, Menelaus MB, Hudson I et al (1986) Hand function in patients with spina bifida cystica. J Pediatr Orthop 6:442–447PubMedGoogle Scholar
  13. 13.
    Broughton NS, Graham G, Menelaus MB (1994) The high incidence of foot deformity in patients with highlevel spina bifida. J Bone Joint Surg 76(B):548–550Google Scholar
  14. 14.
    Frawley PA, Broughton NS, Menelaus MB (1998) Incidence and type of hindfoot deformities in patients with low-level spina bifida. J Pediatr Orthop 18:312–313PubMedCrossRefGoogle Scholar
  15. 15.
    Sharrard WJ, Grosfield I (1968) The management of deformity and paralysis of the foot in myelomeningocele. J Bone Joint Surg 50(B):456–465Google Scholar
  16. 16.
    Walker G (1971) The early management of varus feet in myelomeningocele. J Bone Joint Surg 53(B):462–467Google Scholar
  17. 17.
    Ponseti IV (1996) Congenital Clubfoot. Fundamentals of treatment. Oxford University Press, New YorkGoogle Scholar
  18. 18.
    Menelaus MB (1971) Talectomy for equinovarus deformity in arthrogryposis and spina bifida. J Bone Joint Surg 53(B): 468–473Google Scholar
  19. 19.
    de Carvalho Neto J, Dias LS, Gabrieli AP (1996) Congenital talipes equinovarus in spina bifida: treatment and results. J Pediatr Orthop 16:782–785PubMedGoogle Scholar
  20. 20.
    Sherk HH, Marchinski LJ, Clancy M et al (1989) Ground reaction forces on the plantar surface of the foot after talectomy in the myelomeningocele. J Pediatr Orthop 9:269–275PubMedGoogle Scholar
  21. 21.
    Maynard MJ, Weiner LS, Burke SW (1992) Neuropathic foot ulceration in patients with myelodysplasia. J Pediatr Orthop 12:786–788PubMedGoogle Scholar
  22. 22.
    Roye BD (2005) Neuromuscular Disorders. Myelo — meningocele. In: Dormans JP (ed) Pediatric Ortopaedics. Core knowledge in orthopaedics. Elsevier Mosby, Philadelphia, pp 483–504Google Scholar
  23. 23.
    Bliss DG, Menelaus MB (1986) The results of transfer of the tibialis anterior to the heel in patients who have a myelomeningocele. J Bone Joint Surg 68(A):1258–1264PubMedGoogle Scholar
  24. 24.
    Fraser RK, Hoffman EB (1991) Calcaneus deformity in the ambulant patient with myelomeningocele. J Bone Joint Surg 73(B):994–997Google Scholar
  25. 25.
    Rogrigues RC, Dias LS (1992) Calcaneus deformity in spina bifida: results of anterolateral release. J Pediatr Orthop 12:461–464Google Scholar
  26. 26.
    Janda JP, Skinner SR, Barto PS (1984) Posterior transfer of tibialis anterior in low-level myelodysplasia. Dev Med Child Neurol 26: 100–103PubMedGoogle Scholar
  27. 27.
    Drennan JC, Sharrard WJ (1971) The pathological anatomy of convex pes valgus. J Bone Joint Surg 53(B):455–461Google Scholar
  28. 28.
    Burkus JK, Moore DW, Raycroft JF (1983) Valgus deformity of the ankle in myelodysplastic patients. Correction of stapling of the medial part of the distal tibial physis. J Bone Joint Surg 65(A):1157–1162PubMedGoogle Scholar
  29. 29.
    Aronson DD, Middleton DL (1991) Extra-articular subtalar arthrodesis with cancellous bone graft and internal fixation for children with myelomeningocele. Dev Med Child Neurol 33:232–240PubMedGoogle Scholar
  30. 30.
    Lee YF, Grogan TJ, Moseley CF (1990) Extra-articular subtalar arthrodesis in myelodysplasia. Orthop Trans 14: 590–594Google Scholar
  31. 31.
    Bradley GW, Coleman SS (1981) The treatment of the calcaneocavus foot deformity. J Bone Joint Surg 63: 1159–1166PubMedGoogle Scholar
  32. 32.
    Dias LS, Jasty MJ, Collins P (1984) Rotational deformities of the lower limb in myelomeningocele: evaluation and treatment. J Bone Joint Surg 66(B):215–223PubMedGoogle Scholar
  33. 33.
    Fraser RK, Menelaus MB (1993) The management of tibial torsion in patients with spina bifida. J Bone Joint Surg 75(B):495–497Google Scholar
  34. 34.
    Wright JG, Menelaus MB, Broughton NS et al (1992) Lower extremity alignment in children with spina bifida. J Pediatr Orthop 12:232–234PubMedGoogle Scholar
  35. 35.
    Williams JJ, Graham GP, Dunne KB et al (1993) Late knee problems in myelomeningocele. J Pediatr Orthop 13:701–703PubMedGoogle Scholar
  36. 36.
    Wright JG, Menelaus MB, Broughton NS et al (1991) Natural history of knee contractures in myelomeningocele. J Pediatr Orthop 11:725–730PubMedGoogle Scholar
  37. 37.
    Dias LS (1982) Surgical management of knee contractures in myelomeningocele. J Pediatr Orthop 2:127–131PubMedGoogle Scholar
  38. 38.
    Marshall PD, Broughton NS, Menelaus MB et al (1996) Surgical release of knee flexion contractures in myelomeningocele. J Bone Joint Surg 78(B):912–916CrossRefGoogle Scholar
  39. 39.
    Menelaus M (1998). The knee. In: Broughton N, Menelaus M (eds) Menelaus’ orthopaedic management of spina bifida cystica. WB Saunders, Philadelphia, pp 129–134Google Scholar
  40. 40.
    Sandhu PS, Broughton NS, Menelaus MB (1995) Tenotomy of the ligamentum patellae in spina bifida: management of limited flexion range at the knee. J Bone Joint Surg 77(B): 832–833Google Scholar
  41. 41.
    Broughton N (1998). The hip. In: Broughton N, Menelaus M (eds) Menelaus’ orthopaedic management of spina bifida cystica. WB Saunders, Philadelphia, pp 135–144Google Scholar
  42. 42.
    Broughton NS, Menelaus MB, Cole WG et al (1993) The natural history of hip deformity in myelomeningocele. J Bone Joint Surg 75(B):760–763Google Scholar
  43. 43.
    Mazur JM, Shurtleff D, Menelaus M et al (1989) Orthopaedic management of high-level spina bifida: Early walking compared with early use of a wheelchair. J Bone Joint Surg 71(A):56–61PubMedGoogle Scholar
  44. 44.
    Fraser RK, Bourke HM, Broughton NS et al (1995) Unilateral dislocation of the hip in spina bifida: A longterm follow-up. J Bone Joint Surg 77(B):615–619Google Scholar
  45. 45.
    Menelaus MB (1980) Progress in the management of the paralytic hip in myelomeningocele. Orthop Clin North Am 11:17–30PubMedGoogle Scholar
  46. 46.
    Lough LK, Nielsen DH (1986) Ambulation of children with myelomeningocele-parapodium versus parapodium with Orlau swivel modification. Dev Med Child Neurol 28:489–497PubMedGoogle Scholar
  47. 47.
    McCall RE, Schmidt WT (1986) Clinical experience with the reciprocal gait orthosis in myelodysplasia. J Pediatr Orthop 6:157–161PubMedGoogle Scholar
  48. 48.
    Yngve DA, Douglas R, Roberts JM (1984) The reciprocating gait orthosis in myelomeningocele. J Pediatr Orthop 4:304–310PubMedGoogle Scholar
  49. 49.
    Lee EH, Carroll NC (1985) Hip stability and ambulatory status in myelomeningocele. J Pediatr Orthop 5:522–527PubMedGoogle Scholar
  50. 50.
    Bazih J, Gross RH (1981) Hip surgery in the lumbar level myelomeningocele patient. J Pediatr Orthop 1:405–411PubMedGoogle Scholar
  51. 51.
    Erol B, Bezer M, Kucukdurmaz F et al (2005) Surgical management of hip instabilities in children with spina bifida. Acta Orthop Traumatol Turc 39:16–22PubMedGoogle Scholar
  52. 52.
    Trivedi J, Thompson JD, Slakey JB et al (2002) Clinical and radiographic predictors of scoliosis in patients with myelomeningocele. J Bone Joint Surg 84(A):1389–1394PubMedGoogle Scholar
  53. 53.
    Heeg M, Broughton NS, Menelaus MB (1998) Bilateral dislocation of the hip in spina bifida: A long-term follow-up study. J Pediatr Orthop 18:434–436PubMedCrossRefGoogle Scholar
  54. 54.
    Tosi LL, Buck BD, Nason SS et al (1996) Dislocation of hip in myelomeningocele. The McKay hip stabilization. J Bone Joint Surg 78(A):664–673PubMedGoogle Scholar
  55. 55.
    Canale ST, Hammond NL III, Cotler JM et al (1975) Pelvic displacement osteotomy for chronic hip dislocation in myelodysplasia. J Bone Joint Surg 57:177–183PubMedGoogle Scholar
  56. 56.
    Stillwell A, Menelaus MB (1984) Walking ability after transplantation of the iliopsoas-a long term follow up. J Bone Joint Surg 66:656–659Google Scholar
  57. 57.
    Phillips DP, Lindseth RE (1992) Ambulation after transfer of adductors, external oblique, and tensor fascia lata in myelomeningocele. J Pediatr Orthop 12:712–717PubMedGoogle Scholar
  58. 58.
    Barnett J, Menelaus M (1998) Pressure sores and pathological fractures. In: Broughton N, Menelaus M (eds) Menelaus’ orthopaedic management of spina bifida cystica. WB Saunders, Philadelphia, pp 51–65Google Scholar
  59. 59.
    Quan A, Adams R, Ekmark E et al (1998). Bone mineral density in children with myelomeningocele. Pediatrics 102: E34PubMedCrossRefGoogle Scholar
  60. 60.
    Drummond DS, Moreau M, Cruess RL (1981) Postoperative neuropathic fractures in patients with myelomeningocele. Dev Med Child Neurol 23:147–150PubMedCrossRefGoogle Scholar
  61. 61.
    Kumar SJ, Cowell HR, Towsend P (1984) Physeal, metaphyseal, and diaphyseal injuries of the lower extremities in children with myelomeningocele. J Pediatr Orthop 4:25–27.PubMedGoogle Scholar
  62. 62.
    Parsch K (1991) Origin and treatment of fractures in spina bifida. Eur J Pediatr Surg 1:298–305PubMedGoogle Scholar
  63. 63.
    Nagarkatti DG, Banta JV, Thomson JD (2000) Charcot arthropathy in spina bifida. J Pediatr Orthop 20:82–87PubMedCrossRefGoogle Scholar
  64. 64.
    Phillips D (1998) Orthotics. In: Broughton N, Menelaus M (eds) Menelaus’ orthopaedic management of spina bifida cystica. WB Saunders, Philadelphia, pp 67–76Google Scholar

Copyright information

© Springer-Verlag Italia 2008

Authors and Affiliations

  • Bülent Erol
    • 1
  • Junichi Tamai
    • 2
  1. 1.Department of Orthopaedics and TraumatologyMarmara University Medical CenterIstanbulTurkey
  2. 2.Department of Orthopaedic SurgeryCincinnati Children’s Hospital Medical CenterCincinnatiUSA

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