Skip to main content

Advertisement

Log in

Atlas invagination through the foramen magnum: expanding the spectrum of craniovertebral junction malformations

  • Case Report
  • Published:
European Spine Journal Aims and scope Submit manuscript

Abstract

Purpose

Malformations of the craniovertebral junction (CVJ) range from mild, asymptomatic conditions to severe forms of instability with basilar invagination. Rarely, there have been accounts of forms of so-called paramedian basilar invagination, with abnormal bone masses invading the lateral portion of the foramen magnum. All these entities have been comprehensively classified both from an anatomical and embryological standpoint.

Methods

Here, we report a case of a unique CVJ malformation which is not included in any existing classification framework and could represent a novel pathologic entity. We also provide an overview of the pertinent literature.

Results

The patient was a 14-year-old boy with a recent onset of spastic tetraparesis. Radiological studies documented a malformation of the atlas which invaginated through the foramen magnum, causing anterolateral medullary incarceration. Surgical treatment involved posterior decompression with resection of the abnormal bone and occipito-cervical fusion.

Conclusion

Our report enriches the panorama of CVJ malformations, showing how anatomical knowledge and embryological insights constitute the basis for the correct assessment and treatment of these complex entities.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

References

  1. Pang D, Thompson DNP (2011) Embryology and bony malformations of the craniovertebral junction. Childs Nerv Syst 27:523–564. https://doi.org/10.1007/s00381-010-1358-9

    Article  PubMed  Google Scholar 

  2. Menezes AH, Fenoy KA (2009) Remnants of occipital vertebrae: proatlas segmentation abnormalities. Neurosurgery 64:945–954. https://doi.org/10.1227/01.NEU.0000345737.56767.B8

    Article  PubMed  Google Scholar 

  3. Gholve PA, Hosalkar HS, Ricchetti ET et al (2007) Occipitalization of the atlas in children: morphologic classification, associations, and clinical relevance. J Bone Jt Surg 89:571–578. https://doi.org/10.2106/JBJS.F.00527

    Article  Google Scholar 

  4. Menezes AH, Dlouhy BJ (2020) Atlas assimilation: spectrum of associated radiographic abnormalities, clinical presentation, and management in children below 10 years. Childs Nerv Syst 36:975–985. https://doi.org/10.1007/s00381-019-04488-3

    Article  PubMed  Google Scholar 

  5. Montrisaet R, Petcharunpaisan S (2020) Proatlas anomalies in craniofacial malformations: 5-year experience in King Chulalongkorn Memorial Hospital. Childs Nerv Syst 36:2829–2834. https://doi.org/10.1007/s00381-020-04599-2

    Article  PubMed  Google Scholar 

  6. Tubbs RS, Lingo PR, Mortazavi MM, Cohen-gadol AA (2013) Hypoplastic occipital condyle and third occipital condyle: review of their dysembryology: hypoplastic occipital condyle and TOC. Clin Anat 26:928–932. https://doi.org/10.1002/ca.22205

    Article  PubMed  Google Scholar 

  7. Muhleman M, Charran O, Matusz P et al (2012) The proatlas: a comprehensive review with clinical implications. Childs Nerv Syst 28:349–356. https://doi.org/10.1007/s00381-012-1698-8

    Article  PubMed  Google Scholar 

  8. Menezes AH (2008) Craniocervical developmental anatomy and its implications. Childs Nerv Syst 24:1109–1122. https://doi.org/10.1007/s00381-008-0600-1

    Article  PubMed  Google Scholar 

  9. Wang J, Zhu C, Xia H (2019) Management of unique basilar invagination combined with C1 prolapsing into the foramen magnum in children: report of 2 cases. World Neurosurg 127:92–96. https://doi.org/10.1016/j.wneu.2019.03.249

    Article  PubMed  Google Scholar 

  10. Yerramneni VK, Chandra PS, Kale SS et al (2011) A 6-year experience of 100 cases of pediatric bony craniovertebral junction abnormalities: treatment and outcomes. Pediatr Neurosurg 47:45–50. https://doi.org/10.1159/000329623

    Article  PubMed  Google Scholar 

  11. Wang S, Wang C, Liu Y et al (2009) Anomalous vertebral artery in craniovertebral junction with occipitalization of the atlas. Spine 34:2838–2842. https://doi.org/10.1097/BRS.0b013e3181b4fb8b

    Article  PubMed  Google Scholar 

  12. Ferreira EDZ, Botelho RV (2015) Atlas assimilation patterns in different types of adult craniocervical junction malformations. Spine 40:1763–1768. https://doi.org/10.1097/BRS.0000000000001045

    Article  PubMed  PubMed Central  Google Scholar 

  13. Ahmed R, Traynelis VC, Menezes AH (2008) Fusions at the craniovertebral junction. Childs Nerv Syst 24:1209–1224. https://doi.org/10.1007/s00381-008-0607-7

    Article  PubMed  Google Scholar 

  14. Chapman S, Weale P, Goldin JH et al (1991) The median cleft face syndrome with associated cleft mandible, bifid odontoid peg and agenesis of the anterior arch of atlas. Br J Oral Maxillofac Surg 29:279–281. https://doi.org/10.1016/0266-4356(91)90199-F

    Article  CAS  PubMed  Google Scholar 

  15. Wilting J, Ebensperger C, Müller Thomas S et al (1995) Pax-1 in the development of the cervico-occipital transitional zone. Anat Embryol (Berl). https://doi.org/10.1007/BF00184746

    Article  PubMed  Google Scholar 

  16. Smith CA, Tuan RS (1994) Human PAX gene expression and development of the vertebral column. Clin Orthop 302:241–250. https://doi.org/10.1097/00003086-199405000-00038

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Alberto Benato.

Ethics declarations

Conflict of interest

All authors declare no relevant financial or non-financial disclosures. There are no conflicts of interest. We received no funding for the present work.

Ethical approval

Approval of the institutional review board was not necessary for the present work. The parents of the patient provided informed consent for the anonymized publication of the data.

Additional information

Publisher's Note

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

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Benato, A., Offi, M., Bianchi, F. et al. Atlas invagination through the foramen magnum: expanding the spectrum of craniovertebral junction malformations. Eur Spine J 32, 2615–2621 (2023). https://doi.org/10.1007/s00586-022-07500-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00586-022-07500-8

Keywords

Navigation