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A Cervical Flexion-Extension MRI Study in Down Syndrome

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Abstract

Objective

To assess what kind of information MR examination in flexed and extended positions provides in Down syndrome subjects with suspected cranio-cervical instability.

Methods

Between 2005 and 2008, 35 subjects with DS were recruited in the study. Ethics committee approval was granted and a signed informed consent was obtained from the parents. All the subjects were affected by hypotonic status and ligament laxity established by clinical evaluation, but were asymptomatic about focal neurological symptoms due to medullar damage caused by cranio-cervical instability. Each patient underwent lateral supine radiographs and MR imaging in the neutral, active flexed and extended positions. For evaluating the atlanto-axial and atlanto-occipital joint stability, multiple measurements were calculated.

Results

A significant reduction of anterior subarachnoid space in flexed position was evident in DS subjects compared to healthy controls in neutral and flexed positions. Both, space available for cord and ligamentous thickness showed significant differences between DS subjects and healthy controls.

In DS subjects with occipito-cervical instability, the anterior subarachnoidal space reduction was significantly reduced in flexed position.

Conclusions

In DS subjects with asymptomatic cranio-cervical instability, anterior subarachnoidal evaluation and ligamentous status could add new information about the risk of spinal cord damage.

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References

  1. Amato C, Moschini M, Cioni M, Bianco M. Changes in the cranio-cervical junction in Down’s syndrome. Radiol Med. 1990;79:42–7.

    CAS  PubMed  Google Scholar 

  2. McKay SD, Al-Omari A, Tomlinson LA, Dormans JP. Review of cervical spine anomalies in genetic syndromes. Spine. 2012;37:E269–77.

    Article  PubMed  Google Scholar 

  3. Dedlow ER, Siddiqi S, Fillipps DJ, Kelly MN, Nackashi JA, Tuli SY. Symptomatic atlanto-axial instability in an adolescent with trisomy 21 (Down’s syndrome). Clin Pediatr. 2013;52:633–8.

    Article  Google Scholar 

  4. Hankinson TC, Anderson RCE. Craniovertebral junction abnormalities in Down syndrome. Neurosurgery. 2010;66:A32–8.

    Article  Google Scholar 

  5. American Academy of Pediatrics. Committee on sports medicine and fitness. Atlantoaxial instability in down syndrome: subject review. Pediatrics. 1995;96:151–4.

    Google Scholar 

  6. Pueschel SM. Should children with down syndrome be screened for atlantoaxial instability. Arch Pediatr Adolesc Med. 1998;152:123–5.

    Article  CAS  PubMed  Google Scholar 

  7. Pueschel SM, Herndon JM, Gelch MM, Senft KE, Scola FH, Goldberg MJ. Symptomatic atlantoaxial subluxation in persons with down syndrome. Pediatr Orthop. 1984;4:682–8.

    Article  CAS  Google Scholar 

  8. Pizzutillo PD, Herman MJ. Cervical spine issues in down syndrome. J Pediatr Orthop. 2005;25:253–9.

    Article  PubMed  Google Scholar 

  9. Rojas CA, Bertozzi JC, Martinez CR, Whitlow J. Reassessment of the craniocervical junction: normal values on CT. AJNR Am J Neuroradiol. 2007;28:1819–23.

    Article  CAS  PubMed  Google Scholar 

  10. Menezes AH. Specific entities affecting the craniocervical region: Down’s syndrome. Childs Nerv Syst. 2008;24:1165–8.

    Article  PubMed  Google Scholar 

  11. Taylor TK, Walter WL. Screening of children with down syndrome for atlantoaxial (C1-2) instability: another contentious health question. Med J Aust. 1996;165:448–50.

    CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  13. Harris JH Jr, Carson GC, Wagner LK. Radiologic diagnosis of traumatic occipitovertebral dissociation: 1. Normal occipitovertebral relationships on lateral radiographs of supine subjects. AJR Am J Roentgenol. 1994;162:881–6.

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

    Article  CAS  PubMed  Google Scholar 

  15. Pueschel SM, Moon AC, Scola FH. Computerized tomography in persons with Down syndrome and atlantoaxial instability. Spine. 1992;17:735–7.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  17. Smoker WR, Khanna G. Imaging the craniocervical junction. Childs Nerv Syst. 2008;24:1123–45.

    Article  PubMed  Google Scholar 

  18. Pueschel SM, Scola FH, Pezzullo JC. A longitudinal study of atlanto-dens relationship in asymptomatic individuals with Down syndrome. Pediatrics. 1992;89:1194–8.

    CAS  PubMed  Google Scholar 

  19. Cremers MJ, Bol E, de Roos F, van Gijn J. Risk of sports activities in children with Down’s syndrome and atlanto-axial instability. Lancet. 1993;342:511–4.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. San Raffaele Foundation Rome, Rehabilitation Facility Ceglie Messapica, Progetto Merit RBNE08E8CZ, Via della Pisana 235, 00163, Rome, Italy

    Andrea Romano & Marta Moraschi

  2. IRCSS San Raffaele Pisana, Department of Pediatrics, Rome, Italy

    Giorgio Albertini & Claudia Condoluci

  3. NESMOS, Department of Neuroradiology, S. Andrea Hospital, University Sapienza, Rome, Italy

    Danilo Guida, Luigi Maria Fantozzi & Alessandro Bozzao

  4. Department of Neurosciences, University of Modena and Reggio Emilia, Corso Canal Grande, 64, Modena, Italy

    Riccardo Cornia

  5. IRCSS San Raffaele Pisana, Department of Radiology, Rome, Italy

    Cristina Settecasi & Alberto Pierallini

Authors
  1. Andrea Romano
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  2. Giorgio Albertini
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  3. Danilo Guida
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  4. Riccardo Cornia
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  5. Cristina Settecasi
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  6. Claudia Condoluci
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  7. Marta Moraschi
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  8. Luigi Maria Fantozzi
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  9. Alessandro Bozzao
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  10. Alberto Pierallini
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Corresponding author

Correspondence to Andrea Romano.

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Romano, A., Albertini, G., Guida, D. et al. A Cervical Flexion-Extension MRI Study in Down Syndrome. Indian J Pediatr 82, 349–353 (2015). https://doi.org/10.1007/s12098-014-1549-6

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  • DOI: https://doi.org/10.1007/s12098-014-1549-6

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