European Spine Journal

, Volume 22, Issue 1, pp 29–35 | Cite as

Treatment strategies for severe C1C2 luxation due to congenital os odontoideum causing tetraplegia

  • C. M. BachEmail author
  • D. Arbab
  • M. Thaler
Grand Rounds



High-grade C1C2 luxation is a rare pathology. There is no clear evidence as to how to treat this deformity. There is only limited evidence about the different surgical techniques and possible approaches including advantages, disadvantages, and complications.


This is an uncommon case of a 13-year-old child with progressive, tetraplegia due to congenital os odontoideum with translational instability between C1 and C2, and progressive luxation of C2. An irreducible dislocation of the C0/C1 complex caused significant compression at the cervicomedullary junction and neurologic deficit. In this paper we highlight the different types of os odontoideum, a review of existing evidence of surgical correction. We will discuss the different treatment strategies which could be applied and the current solution will be described.


Continuous skeletal traction and translational reduction was achieved by a specially designed halo traction system including continuous skeletal traction in a wheelchair for 6 weeks. The surgical treatment consisted of a posterior only release, translational reduction and posterior instrumentation from C0 to C4 with a Y plate and homologous bone graft. Neurological deficits started to improve during halo traction. After surgery the patient was ambulatory without any assistance and reached a Frankel stage E. Postoperative X-rays and CT scan revealed complete reduction at the C1/C2 level and a decompressed cervicomedullary junction.


Treatment of severe C1C2 luxation is difficult with limited evidence in the literature. The current case shows a successful treatment strategy to reduce the deformity and lists alternative approaches.


Congenital os odontoideum C1C2 luxation Translational instability Neurologic deficit 


Conflict of interest



  1. 1.
    Matsui H, Imada K, Tsuji H (1997) Radiographic classification of os odontoideum and its clinical significance. Spine 22:1706–1709PubMedCrossRefGoogle Scholar
  2. 2.
    Menezes AH, Ryken TC (1992) Craniovertebral abnormalities in Down’s syndrome. Pediatr Neurosurg 18:24–33PubMedCrossRefGoogle Scholar
  3. 3.
    Pueschel SM, Scola FH, Tupper TB (1990) Skeletal anomalies of the upper cervical spine in children with Down’s syndrome. J Pediatr Orthop 10:607–611PubMedCrossRefGoogle Scholar
  4. 4.
    Fielding JW, Hensinger RN, Hawkins RJ (1980) Os odontoideum. J Bone Joint Surg Am 62:376–383PubMedGoogle Scholar
  5. 5.
    Fielding JW, Griffin PP (1974) Os odontoideum: an acquired lesion. J Bone Joint Surg Am 56:187–190PubMedGoogle Scholar
  6. 6.
    Kirlew KA, Hathout GM, Reiter SD, Gold RH (1993) Os odontoideum in identical twins: perspectives on etiology. Skeletal Radiol 22:525–527PubMedCrossRefGoogle Scholar
  7. 7.
    Klimo P Jr, Kan P, Hrao G, Apfelbaum R, Brockmeyer D (2008) Os odontoideum: presentation, diagnosis, and treatment in a series of 78 patients. J Neurosurg Spine 9:332–342PubMedCrossRefGoogle Scholar
  8. 8.
    Maeda T, Saito T, Harimaya K, Shuto T, Iwamoto Y (2004) Atlantoaxial instability in neck retraction and protrusion in patients with rheumatoid arthritis. Spine 29(7):757–762PubMedCrossRefGoogle Scholar
  9. 9.
    Spierings EL, Braakman R (1982) The management of os odontoideum. Analysis of 37 cases. J Bone Joint Surg Br 64B:422–428Google Scholar
  10. 10.
    Jeanneret B, Magerl F (1994) Primary posterior fusion C1/2 in odontoid fractures: indications, technique, and results of transarticular screw fixation. J Spinal Disord 5:464–475CrossRefGoogle Scholar
  11. 11.
    Watanabe M, Toyama Y, Fujimura Y (1996) Atlantoaxial instability in os odontoideum with myelopathy. Spine 21:1435–1439PubMedCrossRefGoogle Scholar
  12. 12.
    Choit RL, Jamieson DH, Reilly CW (2005) Os odontoideum: a significant radiographic finding. Pediatr Radiol 35:803–807PubMedCrossRefGoogle Scholar
  13. 13.
    Stevens JM, Chong WK, Barber C, Kendall BE, Crockard HA (1994) A new appraisal of abnormalities of the odontoid process associated with atlanto-axial subluxation and neurological disability. Brain 117:133–148PubMedCrossRefGoogle Scholar
  14. 14.
    Anderson RC, Ragel BT, Mocco J, Bohman LE, Brockmeyer DL (2007) Selection of a rigid internal fixation construct for stabilization at the craniovertebral junction in pediatric patients. J Neurosurg 107 (1 Suppl):36–42PubMedGoogle Scholar
  15. 15.
    Crawford NR, Hurlbert RJ (2002) Anatomy and biomechanics of the craniocervical junction. Semin Neurosurg 13:101–110CrossRefGoogle Scholar
  16. 16.
    Brockmeyer D, Apfelbaum R, Tippets R, Walker M, Carey L (1995) Pediatric cervical spine instrumentation using screw fixation. Pediatr Neurosurg 22:147–157PubMedCrossRefGoogle Scholar
  17. 17.
    Kim IS, Hong JT, Jang WY, Yang SH, Sung JH, Son BC, Lee S (2011) Surgical treatment of os odontoideum. J Clin Neurosci 18(4):481–484PubMedCrossRefGoogle Scholar
  18. 18.
    Menezes AH (2008) Surgical approaches: postoperative care and complications transoral-transpalatopharyngeal approach to the craniocervical junction. Childs Nerv Syst 24:1187–1193PubMedCrossRefGoogle Scholar
  19. 19.
    Limpaphayom N, Skaggs DL, McComb G, Krieger M, Tolo VT (2009) Complication of halo use in children. Spine 34(8):779–784PubMedCrossRefGoogle Scholar
  20. 20.
    Shirasaki N, Okada K, Oka S, Hosono N, Yonenobu K, Ono K (1991) Os odontoideum with posterior atlantoaxial instability. Spine 16(7):706–715PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  1. 1.Department of Orthopedics and Trauma, Spine ServiceK-Plus, Remigius Krankenhaus LeverkusenLeverkusenGermany
  2. 2.Department of Orthopedic SurgeryMedical University InnsbruckInnsbruckAustria

Personalised recommendations