Skip to main content
Log in

Abnormal course of the vertebral artery at the craniovertebral junction in patients with Down syndrome visualized by three-dimensional CT angiography

  • Diagnostic Neuroradiology
  • Published:
Neuroradiology Aims and scope Submit manuscript

Abstract

Introduction

We determined the incidence of vertebral artery (VA) anomalies at the craniovertebral junction (CVJ) in patients with Down syndrome, and characterized the VA anomalies.

Methods

The course of the VA in 46 consecutive patients who were due to undergo posterior arthrodesis surgery at the CVJ were evaluated by three-dimensional CT angiography (3DCTA). Included were five patients with Down syndrome who suffered from myelopathy due to atlantoaxial subluxation. All five patients with Down syndrome also had a simultaneous congenital skeletal anomaly, either os odontoideum or ossiculum terminale.

Results

Of the five patients with Down syndrome, three had VA anomalies at the CVJ, two had fenestration and one had a persistent first intersegmental artery. Of the other 41 patients without Down syndrome, five had VA anomalies at the CVJ. The incidence of VA anomalies at the CVJ was much higher in patients with Down syndrome than in those without Down syndrome.

Conclusion

In planning surgery in patients with Down syndrome with symptomatic atlantoaxial subluxation and a congenital skeletal anomaly at the CVJ, we should consider the possible presence of VA anomalies. Preoperative 3DCTA allows us to precisely identify an anomalous VA and evaluate the possible risk of intraoperative VA injury in advance.

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

Similar content being viewed by others

References

  1. Tredwell SJ, Newman DE, Lockitch G (1990) Instability of the upper cervical spine in Down syndrome. J Pediatr Orthop 10:602–606

    PubMed  CAS  Google Scholar 

  2. Pueschel SM, Scola FH (1987) Atlantoaxial instability in individuals with Down syndrome: epidemiologic, radiographic, and clinical studies. Pediatrics 80:555–560

    PubMed  CAS  Google Scholar 

  3. Semine AA, Ertel AN, Goldberg MJ, Bull MJ (1978) Cervical-spine instability in children with Down syndrome (trisomy 21). J Bone Joint Surg Am 60:649–652

    PubMed  CAS  Google Scholar 

  4. Kobori M, Takahashi H, Mikawa Y (1986) Atlanto-axial dislocation in Down’s syndrome. Report of two cases requiring surgical correction. Spine 11:195–200

    Article  PubMed  CAS  Google Scholar 

  5. Taggard DA, Menezes AH, Ryken TC (2000) Treatment of Down syndrome-associated craniovertebral junction abnormalities. J Neurosurg 93:205–213

    PubMed  CAS  Google Scholar 

  6. Segal LS, Drummond DS, Zanotti RM, Ecker ML, Mubarak SJ (1991) Complications of posterior arthrodesis of the cervical spine in patients who have Down syndrome. J Bone Joint Surg Am 73:1547–1554

    PubMed  CAS  Google Scholar 

  7. Wright NM, Lauryssen C (1998) Vertebral artery injury in C1-2 transarticular screw fixation: results of a survey of the AANS/CNS section on disorders of the spine and peripheral nerves. American Association of Neurological Surgeons/Congress of Neurological Surgeons. J Neurosurg 88:634–640

    PubMed  CAS  Google Scholar 

  8. Tokuda K, Miyasaka K, Abe H, Abe S, Takei H, Sugimoto S, Tsuru M (1985) Anomalous atlantoaxial portions of vertebral and posterior inferior cerebellar arteries. Neuroradiology 27:410–413

    Article  PubMed  CAS  Google Scholar 

  9. Sato K, Watanabe T, Yoshimoto T, Kameyama M (1994) Magnetic resonance imaging of C2 segmental type of vertebral artery. Surg Neurol 41:45–51

    Article  PubMed  CAS  Google Scholar 

  10. Taneichi H, Suda K, Kajino T, Kaneda K (2005) Traumatically induced vertebral artery occlusion associated with cervical spine injuries: prospective study using magnetic resonance angiography. Spine 30:1955–1962

    Article  PubMed  Google Scholar 

  11. Yamazaki M, Koda M, Aramomi MA, Hashimoto M, Masaki Y, Okawa A (2005) Anomalous vertebral artery at the extraosseous and intraosseous regions of the craniovertebral junction: analysis by three-dimensional computed tomography angiography. Spine 30:2452–2457

    Article  PubMed  Google Scholar 

  12. Yamazaki M, Koda M, Yoneda M, Aiba A, Moriya H (2004) Anomalous vertebral artery at the craniovertebral junction in a patient with Down syndrome. Case report. J Neurosurg Spine 1:338–341

    Article  PubMed  Google Scholar 

  13. Yamazaki M, Okawa A, Aramomi MA, Hashimoto M, Masaki Y, Koda M (2004) Fenestration of vertebral artery at the craniovertebral junction in Down syndrome: a case report. Spine 29:E551–E554

    Article  PubMed  Google Scholar 

  14. Knox GE, ten Bensel RW (1972) Gastrointestinal malformations in Down’s Syndrome. Minn Med 55:542–544

    PubMed  CAS  Google Scholar 

  15. Shikata J, Yamamuro T, Mikawa Y, Iida H, Kobori M (1987) Surgical treatment of symptomatic atlantoaxial subluxation in Down’s syndrome. Clin Orthop Relat Res 220:111–118

    PubMed  Google Scholar 

  16. Nader-Sepahi A, Casey AT, Hayward R, Crockard HA, Thompson D (2005) Symptomatic atlantoaxial instability in Down syndrome. J Neurosurg 103:231–237

    PubMed  Google Scholar 

  17. Braakhekke JP, Gabreels FJ, Renier WO, van Rens TJ, Thijssen HO, Begeer JH (1985) Cranio-vertebral pathology in Down syndrome. Clin Neurol Neurosurg 87:173–179

    Article  PubMed  CAS  Google Scholar 

  18. Currarino G (2002) Segmentation defect in the midodontoid process and its possible relationship to the congenital type of os odontoideum. Pediatr Radiol 32:34–40

    Article  PubMed  Google Scholar 

  19. Lo RN, Leung MP, Lau KC, Yeung CY (1986) Abnormal radial artery in Down’s syndrome. Arch Dis Child 61:885–890

    PubMed  CAS  Google Scholar 

  20. Tandon R, Edwards JE (1973) Cardiac malformations associated with Down’s syndrome. Circulation 47:1349–1355

    PubMed  CAS  Google Scholar 

  21. Rathore MH, Sreenivasan VV (1989) Vertebral and right subclavian artery abnormalities in the Down syndrome. Am J Cardiol 63:1528–1529

    Article  PubMed  CAS  Google Scholar 

Download references

Conflict of interest statement

We declare that we have no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Masashi Yamazaki.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Yamazaki, M., Okawa, A., Hashimoto, M. et al. Abnormal course of the vertebral artery at the craniovertebral junction in patients with Down syndrome visualized by three-dimensional CT angiography. Neuroradiology 50, 485–490 (2008). https://doi.org/10.1007/s00234-008-0368-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00234-008-0368-8

Keywords

Navigation