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Specific entities affecting the craniocervical region: Down’s syndrome

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Abstract

Introduction

The ligamentous laxities and bony abnormalities associated with Down’s syndrome, os odontoideum, achondroplasia, osteogenesis imperfecta, and basilar invagination have been discussed with the imaging of the craniocervical junction. These are significant and require suspicion when dealing with children with the previously mentioned entities.

Conclusion

Previous adverse surgical results in managing these patients reflect the lack of understanding of the underlying pathology and the appropriate surgical treatment.

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References

  1. Alvarez N, Rubin L (1986) Atlantoaxial instability in adults with Down syndrome: a clinical and radiological survey. Appl Res Ment Retard 7:67–78

    Article  PubMed  CAS  Google Scholar 

  2. Aoki N (1988) Atlantoaxial dislocation presenting as sudden onset of quadriplegia in Down’s syndrome. Surg Neurol 30(2):153–155

    Article  PubMed  CAS  Google Scholar 

  3. Arlet V, Rigault P, Padovani JP, Janklevicz P, Touzet P, Finidori G (1992) Atlantoaxial instability in children with trisomy 21: atlantoaxial (C1–C2) or occipitoaxial (O–C2) arthrodesis? Rev Chir Orthop Reparatrice Appar Mot 78(4):240–247

    PubMed  CAS  Google Scholar 

  4. Belichenko PV, Kleschevnikov AM, Salehi A, Epstein CJ, Mobley WC (2007) Synaptic and cognitive abnormalities in mouse models of Down syndrome: exploring genotype–phenotype relationships. J Comp Neurol 504(4):329–345

    Article  PubMed  CAS  Google Scholar 

  5. Braakhekke JP (1988) Symptomatic atlantoaxial dislocation in Down’s syndrome (letter). Ann Neurol 23:421

    Article  PubMed  CAS  Google Scholar 

  6. Brockmeyer D (1999) Down syndrome and craniovertebral instability. Topic review and treatment recommendations. Pediatr Neurosurg 31(2):71–77

    Article  PubMed  CAS  Google Scholar 

  7. Burke SW, French HG, Roberts JM, Johnston CE 2nd, Whitecloud TS 3rd, Edmunds JO Jr (1985) Chronic atlantoaxial instability in Down syndrome. J Bone Joint Surg (Am) 67:1356–1360

    CAS  Google Scholar 

  8. Cremers MJ, Beijer HJ (1993) No relation between general laxity and atlantoaxial instability in children with Down syndrome. J Pediatr Orthop 13(3):318–321

    PubMed  CAS  Google Scholar 

  9. Cremers MJ, Bol E, de Roos F, van Gijn J (1993) Risk of sports activities in children with Down’s syndrome and atlantoaxial instability. Lancet 342(8870):511–514

    Article  PubMed  CAS  Google Scholar 

  10. Cullen S, O’Connell E, Blake, Ward OC (1989) Atlantoaxial instability in Down’s syndrome: clinical and radiological screening. Ir Med J 82(2):64–65

    PubMed  CAS  Google Scholar 

  11. Devi BI, Shenoy SN, Panigrahi MK, Chandramouli BA, Das BS, Jayakumar PN (1997) Anomaly of arch of atlas—a rare cause of symptomatic canal stenosis in children. Pediatr Neurosurg 26:214–218

    Article  PubMed  CAS  Google Scholar 

  12. Doyle JS, Lauerman WC, Wood KB, Krause DR (1996) Complications and long-term outcome of upper cervical spine arthrodesis in patients with Down syndrome. Spine 21(10):1223–1231

    Article  PubMed  CAS  Google Scholar 

  13. Dzenitis AJ (1966) Spontaneous atlantoaxial dislocation in a mongoloid child with spinal cord compression. Case report. J Neurosurg 25:458–460

    PubMed  CAS  Google Scholar 

  14. El-Khoury GY, Clark CR, Dietz FR, Harre RG, Tozzi JE, Kathol MH (1986) Posterior atlanto-occipital subluxation in Down syndrome. Radiology 159:507–509

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  16. Herring JA (1982) Cervical instability in Down’s syndrome and juvenile rheumatoid arthritis. J Pediatr Orthop 2:205–207

    PubMed  CAS  Google Scholar 

  17. Locke AR, Gardner JI, Van Epps EF (1966) Atlas-dens interval in children. A survey based on 200 normal cervical spines. Am J Radiol 97:135–140

    CAS  Google Scholar 

  18. Martel W, Tishler JM (1966) Observations on the spine in mongoloidism. AJR 97:630–638

    CAS  Google Scholar 

  19. Menezes AH (2003) Developmental abnormalities of the craniovertebral junction. In: Winn HR (ed) Youman’s neurological surgery. vol. 3. 5th edn. Saunders, Philadelphia, pp 3331–3345

    Google Scholar 

  20. Menezes AH (2004) Evaluation and treatment of congenital and developmental anomalies of the cervical spine. J Neurosurg (Spine) 2:188–197

    Google Scholar 

  21. Menezes AH, Ryken TC (1992) Craniovertebral abnormalities in Down’s syndrome. Pediatr Neurosurg 18:24–33

    Article  PubMed  CAS  Google Scholar 

  22. Michejda M, Menolascino FJ (1975) Skull base abnormalities in Down’s syndrome. Ment Retard 131:24–26

    Google Scholar 

  23. Msall ME, Reese ME, DiGaudio K, Griswold K, Granger CV, Cooke RE (1990) Symptomatic atlantoaxial instability associated with medical and rehabilitative procedures in children with Down syndrome. Pediatrics 85:447–449

    PubMed  CAS  Google Scholar 

  24. Pueschel SM, Scola F (1987) Atlantoaxial instability in individuals with Down’s syndrome: Epidemiologic, radiographic, and clinical studies. Pediatrics 80:555–560

    PubMed  CAS  Google Scholar 

  25. Pueschel SM, Herndon JH, Gelch MM, Senft KE, Scola FH, Goldberg MJ (1984) Symptomatic atlantoaxial subluxation in persons with Down syndrome. J Pediatr Orthop 4(6):682–688

    PubMed  CAS  Google Scholar 

  26. Pueschel SM, Scola FH, Tupper TB, Pezzullo JC (1990) Skeletal anomalies of the upper cervical spine in children with Down’s syndrome. J Pediatr Orthop 10:607–611

    PubMed  CAS  Google Scholar 

  27. Rizzolo S, Lemos MJ, Mason DE (1995) Posterior spinal arthrodesis for atlantoaxial instability in Down syndrome. J Pediatr Orthop 15(4):543–548

    PubMed  CAS  Google Scholar 

  28. Ryken TC, Menezes AH (1994) Cervicomedullary compression in achondroplasia. J Neurosurg 81:43–48

    PubMed  CAS  Google Scholar 

  29. Sawin PD, Traynelis VC, Menezes AH (1998) A comparative analysis of fusion rates and donor-site morbidity for autogenic rib and iliac crest bone grafts in posterior cervical fusions. J Neurosurg 88:255–265

    Article  PubMed  CAS  Google Scholar 

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

    CAS  Google Scholar 

  31. Smith MD, Phillips WA, Hensinger RN (1991) Fusion of the upper cervical spine in children and adolescents. An analysis of 17 patients. Spine 16(7):695–701

    Article  PubMed  CAS  Google Scholar 

  32. Spitzer R, Rainowitch JY, Wybar KC (1961) A study of the abnormalities of the skull, teeth and lenses in mongolism. Can Med Assoc J 84:567–572

    Google Scholar 

  33. Taggard DA, Menezes AH, Ryken TC (1999) Instability of the craniovertebral junction and treatment outcomes in patients with Down’s syndrome. Neurosurg Focus 6(6):e3

    Article  PubMed  CAS  Google Scholar 

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

    CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  36. Una K, Kataoka O, Shiba R (1996) Occipitoatlantal and occipitoaxial hypermobility in Down syndrome. Spine 21(12):1430–1434

    Article  Google Scholar 

  37. Winell J, Burke SW (2003) Sports participation of children with Down syndrome. Orthop Clin North Am 34(3):439–443

    Article  PubMed  Google Scholar 

  38. Worley G, Shbarou R, Heffner AN, Belsito KM, Capone GT, Kishnani PS (2004) New onset focal weakness in children with Down syndrome. Am J Med Genet A 128(1):15–18

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Neurosurgery, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, 1824 JPP, Iowa City, IA, 52242, USA

    Arnold H. Menezes

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  1. Arnold H. Menezes
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Correspondence to Arnold H. Menezes.

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Menezes, A.H. Specific entities affecting the craniocervical region: Down’s syndrome. Childs Nerv Syst 24, 1165–1168 (2008). https://doi.org/10.1007/s00381-008-0603-y

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  • DOI: https://doi.org/10.1007/s00381-008-0603-y

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