Skip to main content
SpringerLink
Log in

Chiari malformation and atlantoaxial instability: problems of co-existence

  • Special Annual Issue
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Background

Association of Chiari malformation and atlantoaxial subluxation varies. There is a complex relationship between the two, bony and soft tissue pathologies.

Methods

This is a review of various articles available from the literature on the management of Chiari and its association with atlantoaxial instability.

Results

We have an experience of operating on 86 cases of paediatric atlantoaxial subluxation, of which 12 had Chiari malformation diagnosed preoperatively (13.95%). Of the 76 children with Chiari malformations operated on by us, 11 had associated atlantoaxial subluxation diagnosed on imaging (14.47%).

Conclusions

Re-alignment and reduction with fixation may be effective in achieving decompression in cases where reduction is possible from posterior approach. In these cases, posterior fixation is all that is required. If reduction is not possible from posterior and there is “fixed” ventral compression, anterior decompression needs to be combined with posterior fixation. In most cases, direct posterior decompression is warranted.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Levy WJ, Mason L, Hahn JF (1983) Chiari malformation presenting in adults: a surgical experience in 127 cases. Neurosurgery 12:377–390

    Article  CAS  PubMed  Google Scholar 

  2. Dyste GN, Menezes AH, VanGilder JC (1989) Symptomatic Chiari malformations. J Neurosurg 71:159–168

    Article  CAS  PubMed  Google Scholar 

  3. Menezes AH (1995) Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): data base analysis. Pediatr Neurosurg 23:260–269

    Article  CAS  PubMed  Google Scholar 

  4. Milhorat TH, Chou MW, Trinidad EM et al (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44:1005–1017

    Article  CAS  PubMed  Google Scholar 

  5. Menezes AH, Greenlee JDW, Donovan KA (2005) Honored guest presentation: lifetime experiences and where we are going: Chiari I with syringohydromyelia--controversies and development of decision trees. Clin Neurosurg 52:297–305

    PubMed  Google Scholar 

  6. Caetano de Barros M, Farias W, Ataíde L, Lins S (1968) Basilar impression and Arnold-Chiari malformation. A study of 66 cases. J Neurol Neurosurg Psychiatry 31:596–605

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Pang D, Thompson DNP (2011) Embryology and bony malformations of the craniovertebral junction. Childs Nerv Syst 27:523–564

    Article  PubMed  Google Scholar 

  8. van Gilder JCMA (1983) Craniovertebral junction abnormalities. Clin Neurosurg 30:514–530

    Article  Google Scholar 

  9. Asakawa H, Yanaka K, Narushima K et al (1999) Anomaly of the axis causing cervical myelopathy. J Neurosurg Spine 91:121–123

    Article  CAS  Google Scholar 

  10. Behari S, Kiran Kumar MV, Banerji D et al (2004) Case report atlantoaxial dislocation associated with the maldevelopment of the posterior neural arch of axis causing compressive myelopathy. Neurol India 52(4):489

    PubMed  Google Scholar 

  11. Lufkin T, Mark M, Hart CP et al (1992) Homeotic transformation of the occipital bones of the skull by ectopic expression of a homeobox gene. Nature 359:835–841

    Article  CAS  PubMed  Google Scholar 

  12. Condie BG, Capecchi MR (1993) Mice homozygous for a targeted disruption of Hoxd-3 (Hox-4.1) exhibit anterior transformations of the first and second cervical vertebrae, the atlas and the axis. Development 119:579–595

    CAS  PubMed  Google Scholar 

  13. Dietrich S, Kessel M (1997) The vertebral column. In: Thorogood P (ed) Embryos, genes and birth defects. Wiley, Chichester, pp 281–30214

    Google Scholar 

  14. Menezes AH (2008) Craniocervical developmental anatomy and its implications. Childs Nerv Syst 24:1109–1122

    Article  PubMed  Google Scholar 

  15. Chisaka O, Capecchi MR (1991) Regionally restricted developmental defects resulting from targeted disruption of the mouse homeobox gene hox-1.5. Nature 350:473–479

    Article  CAS  PubMed  Google Scholar 

  16. Kessel M (1993) Reversal of axonal pathways from rhombomere 3 correlates with extra hox expression domains. Neuron 10:379–393

    Article  CAS  PubMed  Google Scholar 

  17. Koseki H, Wallin J, Wilting J, Mizutani Y, Kispert A, Ebensperger C, Herrmann BG, Christ B, Balling R (1993) A role for Pax-1 as a mediator of notochordal signals during the dorsoventral specification of vertebrae. Development 119:649–660

    CAS  PubMed  Google Scholar 

  18. Kimura Y, Matsunami H, Inoue T et al (1995) Cadherin-11 expressed in association with mesenchymal morphogenesis in the head, somite, and limb bud of early mouse embryos. Dev Biol 169:347–358

    Article  CAS  PubMed  Google Scholar 

  19. Keynes RJ, Stern CD (1988) Mechanisms of vertebrate segmentation. Development 103:413–429

    CAS  PubMed  Google Scholar 

  20. Erdil H, Yildiz N, Cimen M (2003) Congenital fusion of cervical vertebrae and its clinical significance. J Anat Soc India 52:125–127

    Google Scholar 

  21. Saraga-Babić M, Saraga M (1993) Role of the notochord in the development of cephalic structures in normal and anencephalic human fetuses. Virchows Arch A Pathol Anat Histopathol 422:161–168

    Article  PubMed  Google Scholar 

  22. Sakai Y, Meno C, Fujii H et al (2001) The retinoic acid-inactivating enzyme CYP26 is essential for establishing an uneven distribution of retinoic acid along the anterio-posterior axis within the mouse. Genes Dev 15:213–225

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Kashyap V, Gudas L, Brenet F et al (2011) Epigenomic reorganization of the clustered Hox genes in embryonic stem cells induced by retinoic acid. J Biol Chem 2(86):3250–3265

    Article  CAS  Google Scholar 

  24. Wellik DM, Capecchi MR (2003) Hox10 and Hox11 genes are required to globally pattern the mammalian skeleton. Science (80- ) 301:363–367

    Article  CAS  Google Scholar 

  25. Wellik DM (2009) Hox genes and vertebrate axial pattern. Curr Top Dev Biol 88:257–284

    Article  CAS  PubMed  Google Scholar 

  26. Mallo M, Wellik D, Deschamps J (2010) Hox genes and regional patterning of the vertebrate body plan. Dev Biol 344:7–15

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. McGaughran JM, Oates A, Donnai D et al (2003) Mutations in PAX1 may be associated with Klippel–Feil syndrome. Eur J Hum Genet 11:468–474

    Article  CAS  PubMed  Google Scholar 

  28. Soshnikova N, Dewaele R, Janvier P et al (2013) Duplications of hox gene clusters and the emergence of vertebrates. Dev Biol 378:194–199

    Article  CAS  PubMed  Google Scholar 

  29. Jukkola T, Trokovic R, Maj P et al (2005) Meox1Cre: a mouse line expressing Cre recombinase in somitic mesoderm. Genesis 43:148–153

    Article  CAS  PubMed  Google Scholar 

  30. Abu-Abed S, Dollé P, Metzger D et al (2001) The retinoic acid-metabolizing enzyme, CYP26A1, is essential for normal hindbrain patterning, vertebral identity, and development of posterior structures. Genes Dev 15:226–240

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Mardani M, Borujeni MJS, Esfandiary E (2016) Congenital fusion of cervical vertebrae: a review on embryological etiology. Rev Clin Med 3:148–153

    Google Scholar 

  32. Chatterjee S (2018) The chicken or the egg - pancervical fusion with atlantoaxial dislocation. Neurol India 66:153

    Article  PubMed  Google Scholar 

  33. Nishikawa M, Sakamoto H, Hakuba A et al (1997) Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg 86:40–47

    Article  CAS  PubMed  Google Scholar 

  34. Moore KL, Persaud TVN, Torchia MG (1998) The developing human : clinically oriented embryology, 6th edn. WB Saunders, Philadelphia, pp 405–426

    Google Scholar 

  35. Wackenheim A (1974) Roentgen diagnosis of the craniovertebral region. Springer-Verlag, Berlin, pp 353–379

    Google Scholar 

  36. Shapiro R, Robinson F (1976) Anomalies of the craniovertebral border. Am J Roentgenol 127:281–287

    Article  CAS  Google Scholar 

  37. Di Lorenzo N, Fortuna A, Guidetti B (1982) Craniovertebral junction malformations. J Neurosurg 57:603–608

    Article  PubMed  Google Scholar 

  38. Mesiwala AH, Shaffrey CI, Gruss JS, Ellenbogen RG (2001) Atypical hemifacial microsomia associated with Chiari I malformation and syrinx: further evidence indicating that Chiari I malformation is a disorder of the paraaxial mesoderm. J Neurosurg 95:1034–1039

    Article  CAS  PubMed  Google Scholar 

  39. Kagawa M, Jinnai T, Matsumoto Y et al (2006) Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia: case report. Surg Neurol 65:497–502

    Article  PubMed  Google Scholar 

  40. Fenoy AJ, Menezes AH, Fenoy KA (2008) Craniocervical junction fusions in patients with hindbrain herniation and syringohydromyelia. J Neurosurg Spine 9:1–9

    Article  PubMed  Google Scholar 

  41. Goel A (2009) Basilar invagination, Chiari malformation, syringomyelia: a review. Neurol India 57:235

    Article  PubMed  Google Scholar 

  42. Achawal AGS, Goel SA (1995) The surgical treatment of chiari malformation associated with atlantoaxial dislocation. Br J Neurosurg 9:67–72

    Article  PubMed  Google Scholar 

  43. Behari S, Kalra SK, Kiran Kumar MV et al (2007) Chiari I malformation associated with atlanto-axial dislocation: focussing on the anterior cervico-medullary compression. Acta Neurochir 149:41–50

    Article  CAS  PubMed  Google Scholar 

  44. Kumar R, Kalra SK, Vaid VK, Mahapatra AK (2008) Chiari I malformation: surgical experience over a decade of management. Br J Neurosurg 22:409–414

    Article  CAS  PubMed  Google Scholar 

  45. Galarza M, Martínez-Lage JF, Ham S, Sood S (2011) Cerebral anomalies and Chiari type 1 malformation. Pediatr Neurosurg 46:442–449

    Article  Google Scholar 

  46. Dlouhy BJ, Policeni BA, Menezes AH (2017) Reduction of atlantoaxial dislocation prevented by pathological position of the transverse ligament in fixed, irreducible os odontoideum: operative illustrations and radiographic correlates in 41 patients. J Neurosurg Spine 27:20–28

    Article  PubMed  Google Scholar 

  47. Goel A, Gore S, Shah A et al (2018) Atlantoaxial fixation for Chiari 1 formation in pediatric age-group patients: report of treatment in 33 patients. World Neurosurg 111:e668–e677

    Article  PubMed  Google Scholar 

  48. Goel A (2015) Is atlantoaxial instability the cause of Chiari malformation? Outcome analysis of 65 patients treated by atlantoaxial fixation. J Neurosurg Spine 22:116–127

    Article  PubMed  Google Scholar 

  49. Goel A (2014) Is Chiari malformation nature’s protective “air-bag” ? Is its presence diagnostic of atlantoaxial instability? J Craniovertebr Junction Spine 5:107–109

    Article  PubMed  PubMed Central  Google Scholar 

  50. Joaquim A, Tedeschi H, Ps C (2019) Controversies in the surgical management of congenital craniocervical junction disorders – a critical review. Neurol India 66:1003

    Google Scholar 

  51. Menezes AH (2011) Current opinions for treatment of symptomatic hindbrain herniation or Chiari type I malformation. World Neurosurg 75:226–228

    Article  PubMed  Google Scholar 

  52. Bollo R, Riva-Cambrin J, … Brockmeyer DL (2012) Complex Chiari malformations in children: an analysis of preoperative risk factors for occipitocervical fusion. Pediatrics 10:134–41

  53. Salunke P, Sura S, Futane S et al (2012) Ventral compression in adult patients with Chiari 1 malformation sans basilar invagiantion: cause and management. Acta Neurochir 154:147–152

    Article  PubMed  Google Scholar 

  54. Rahman A (2015) Letter to the editor: does atlantoaxial dislocation really cause Chiari? J Neurosurg Spine 23:393–394

    Article  PubMed  Google Scholar 

  55. Menezes AH (2012) Craniovertebral junction abnormalities with hindbrain herniation and syringomyelia: regression of syringomyelia after removal of ventral craniovertebral junction compression. J Neurosurg 116:301–309

    Article  PubMed  Google Scholar 

  56. Menezes AH, VanGilder JC, Graf CJ, McDonnell DE (1980) Craniocervical abnormalities. A comprehensive surgical approach. J Neurosurg 53:444–455

    Article  CAS  PubMed  Google Scholar 

  57. Müller F, O’Rahilly R (1991) Development of anencephaly and its variants. Am J Anat 190:193–218

    Article  PubMed  Google Scholar 

  58. Kohno K, Sakaki S, Shiraishi T et al (1990) Successful treatment of adult Arnold-Chiari malformation associated with basilar impression and syringomyelia by the transoral anterior approach. Surg Neurol 33:284–287

    Article  CAS  PubMed  Google Scholar 

  59. Klekamp J (2015) Chiari I malformation with and without basilar invagination: a comparative study. Neurosurg Focus 38:E12

    Article  PubMed  Google Scholar 

  60. Hwang SW, Heilman CB, Riesenburger RI, Kryzanski J (2008) C1–C2 arthrodesis after transoral odontoidectomy and suboccipital craniectomy for ventral brain stem compression in Chiari I patients. Eur Spine J 17:1211–1217

    Article  PubMed  PubMed Central  Google Scholar 

  61. Jea A (2015) Editorial: Chiari malformation I surgically treated with atlantoaxial fixation. J Neurosurg Spine 22:113–115

    Article  PubMed  Google Scholar 

  62. Hankinson TC, Klimo P, Feldstein NA et al (2007) Chiari malformations, syringohydromyelia and scoliosis. Neurosurg Clin N Am 18:549–568

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurosurgery, VIMS and Park Clinic, Park Clinic, 4, Gorky Terrace road, Elgin, Kolkata, 700017, India

    Sandip Chatterjee

  2. Department of Neurosurgery, Park Clinic, Kolkata, 17, India

    Pankaj Shivhare & Shyam Gopal Verma

Authors
  1. Sandip Chatterjee
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Pankaj Shivhare
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Shyam Gopal Verma
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sandip Chatterjee.

Ethics declarations

Conflict of interest

None.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Presentation at a meeting

None.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Chatterjee, S., Shivhare, P. & Verma, S.G. Chiari malformation and atlantoaxial instability: problems of co-existence. Childs Nerv Syst 35, 1755–1761 (2019). https://doi.org/10.1007/s00381-019-04284-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-019-04284-z

Keywords

Search

Navigation