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An Updated Algorithm for Radiographic Screening of Upper Cervical Instability in Patients With Down Syndrome

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Abstract

Study Design

Retrospective evaluation of cervical spine images from 2006–2012 for the purposes of “screening” children with Down syndrome for instability.

Objective

To determine whether a full series of cervical spine images including flexion/extension lateral (FEL) radiographs was needed to avoid missing upper cervical instability.

Summary of Background Data

The best algorithm, measurements, and criteria for screening children with Down syndrome for upper cervical instability are controversial. Many authors have recommended obtaining flexion and extension views. We noted that patients who require surgical stabilization due to myelopathy or cord compression typically have grossly abnormal radiographic measurements on the neutral upright lateral (NUL) cervical spine radiograph.

Methods

The atlanto-dental interval, space available for cord, and basion axial interval were measured on all films. The Weisel-Rothman measurement was made in the FEL series. Clinical outcome of those with abnormal measurements were reviewed. Sensitivity, specificity, and positive and negative predictive values of NUL and FEL radiographs for identifying clinically significant cervical spine instability were calculated.

Results

A total of 240 cervical spine series in 213 patients with Down syndrome between the ages of 4 months and 25 years were reviewed. One hundred seventy-two children had an NUL view, and 88 of these patients also had FEL views. Only one of 88 patients was found to have an abnormal atlanto-dental interval (≥6 mm), space available for cord at C1 (≤14 mm), or basion axial interval (>12 mm) on an FEL series that did not have an abnormal measurement on the NUL radiograph. This patient had no evidence of cord compression or myelopathy.

Conclusions

Obtaining a single NUL radiograph is an efficient method for radiographic screening of cervical spine instability. Further evaluation may be required if abnormal measurements are identified on the NUL radiograph. We also propose new “normal” values for the common radiographic measurements used in assessing risk of cervical spine instability in patients with Down syndrome.

Level of Evidence

Level IV.

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References

  1. American Academy of Pediatrics, Committee on Sports Medicine and Fitness. Atlantoaxial instability in Down syndrome: subject review. Pediatrics 1995;96:151–4.

    Google Scholar 

  2. Burke SW, French HG, Roberts JM, et al. Chronic atlanto-axial instability in Down syndrome. J Bone Joint Surg Am 1985;67:1356–60.

    Article  CAS  Google Scholar 

  3. Tredwell SJ, Newman DE, Lockitch G. Instability of the upper cervical spine in Down syndrome. J Pediatr Orthop 1990;10:602–6.

    Article  CAS  Google Scholar 

  4. Spitzer R, Rabinowitch JY, Wybor KC. A study of the abnormalities of skull, teeth, and lenses in mongolism. Can Med Assoc J 1961;84:567.

    CAS  PubMed  PubMed Central  Google Scholar 

  5. Matsuda Y, Sano N, Watanabe S, et al. Clinical predictors and radiological reliability in subjects with Down’s syndrome. Spine 1995;20:2283–6.

    Article  CAS  Google Scholar 

  6. Pueschel SM, Scola FH. Atlantoaxial instability in individuals with Down syndrome: epidemiologic, radiographic, and clinical studies. Pediatrics 1987;80:555–60.

    CAS  PubMed  Google Scholar 

  7. Selby KA, Newton RW, Gupta S, et al. Clinical predictors and radiological reliability in atlantoaxial subluxation in Down’s syndrome. Arch Dis Child 1991;66:876–8.

    Article  CAS  Google Scholar 

  8. American Academy of Pediatrics, Committee on Sports Medicine. Atlantoaxial instability in Down syndrome: subject review. Pediatrics 1984;74:152–4.

    Google Scholar 

  9. Wellborn CC, Sturm PF, Hatch RS, et al. Intra-observer reproducibility and inter-observer reliability of cervical spine measurements. J Pediatr Orthop 2000;20:66–70.

    CAS  PubMed  Google Scholar 

  10. Karol LA, Sheffield EG, Crawford K, et al. Reproducibility in the measurement of atlanto-occipital instability in children with Down syndrome. Spine 1996;21:2463–8.

    Article  CAS  Google Scholar 

  11. Oda T, Yonenobu K, Fujimura Y, et al. Diagnostic validity of space available for the spinal cord at C1 level for cervical myelopathy in patients with rheumatoid arthritis. Spine 2009;34:1395–8.

    Article  Google Scholar 

  12. Harris JH, Carson GC, Wagner LK. Radiologic diagnosis of traumatic occipitovertebral dissociation. AJR Am J Roentgenol 1994;162:887–92.

    Article  Google Scholar 

  13. Hakinson TC, Anderson RCE. Craniovertebral junction abnormalities in Down syndrome. Neurosurgery 2010;66A32–8.

    Article  Google Scholar 

  14. Wiesel SW, Rothman RH. Occipitoatlantal hypermobility. Spine 1979;4:187–91.

    Article  CAS  Google Scholar 

  15. Nakamura N, Inaba Y, Oba M, et al. Novel 2 radiographical measurements for atlantoaxial instability in children with Down syndrome. Spine 2014;39:E1566–74.

    Article  Google Scholar 

  16. Gabriel KR, Mason DE, Carango P. Occipito-atlantal translation in Down’s syndrome. Spine 1990;15:997–1002.

    Article  CAS  Google Scholar 

  17. Ferguson RL, Putney ME, Allen BL, et al. Comparison of neurological deficits with atlanto-dens intervals in patients with Down syndrome. J Spinal Disord 1997;10:246–52.

    Article  CAS  Google Scholar 

  18. Harley EH, Collins MD. Neurologic sequelae secondary to atlantoaxial instability in Down syndrome. Implications in otolaryngologic surgery. Arch Otolaryngol Head Neck Surg 1994;120:159–65.

    Article  CAS  Google Scholar 

  19. Powell JF, Woodcock T, Luscombe FE. Atlantoaxial subluxation in Down syndrome. Anaesthesia 1990;45:1049–51.

    Article  CAS  Google Scholar 

  20. Rosenbaum DM, Blumhagen JD, King HA. Atlantooccipital instability in Down syndrome. AJR Am J Roentgenol 1986;146:1269–72.

    Article  CAS  Google Scholar 

  21. Brockmeyer D. Down syndrome and craniovertebral instability. Topic review and treatment recommendations. Pediatr Neurosurg 1999;31:71–7.

    Article  CAS  Google Scholar 

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Authors and Affiliations

Authors

Corresponding author

Correspondence to Maryse Bouchard MD, MSc, FRCSC.

Additional information

Author disclosures: MB (Orthopaediatrics Inc, Nuvasive, Novadip, unrelated), JMB (Johnson&Johnson, unrelated), VB (none), WFK (none).

IRB approval: This study was approved by Seattle Children’s Hospital, PIROSTUDY13906.

Funding: There were no funding sources.

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Bouchard, M., Bauer, J.M., Bompadre, V. et al. An Updated Algorithm for Radiographic Screening of Upper Cervical Instability in Patients With Down Syndrome. Spine Deform 7, 950–956 (2019). https://doi.org/10.1016/j.jspd.2019.01.012

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  • DOI: https://doi.org/10.1016/j.jspd.2019.01.012

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