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The Management of Disorders of the Child’s Cervical Spine pp 291–301Cite as

Chiari Malformations and Foramen Magnum Stenosis

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Abstract

Chiari types 1 and 2 are congenital hindbrain abnormalities frequently encountered by the pediatric neurosurgeon and orthopedic surgeon. The entities will be defined and common associated findings discussed. Options for surgical intervention, potential complications, and outcomes will be described in this review.

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References

  1. Chairi H. Uber veranderungen des kleinhirns, des pons und der medulla oblongata infolge von congenitaler hydrocephalie des gross hirsn. Deutsche Medizinische Woschenschrift. 1891;17:1172–5. [German].

    Article  Google Scholar 

  2. Chiari H. Uber veranderungen des kleinhirns, des pons und der medulla oblongata infolge von congenitaler hydrocephalie des grosshirns. Denkschriten der Akademieder Wissenschaften Wien. 1986;63:71–116. [German].

    Google Scholar 

  3. Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC 3rd, Rozzelle CJ, et al. Institutional experience with 500 cases of surgically treated pediatric Chiari malformation type I. J Neurosurg Pediatr. 2011;7(3):248–56.

    Article  PubMed  Google Scholar 

  4. Dauser RC, DiPiertro MA, Venes JL. Symptomatic Chiari I malformation in childhood; a report of 7 cases. Pediatr Neurosci. 1988;14:184–90.

    Article  CAS  PubMed  Google Scholar 

  5. Milhorta TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44(5):1005–17.

    Article  Google Scholar 

  6. Iskandar BJ, Oakes J. Chiari malformations. In: Albright AL, Pollack IF, Adelson PD, editors. Principles and Practice of Pediatric Neurosurgery. New York: Thieme; 1999. p. 165–87.

    Google Scholar 

  7. Hankinson T, Tuibbs RS, Wellons JC. Duraplasty or not? An evidence-bases review of pediatric Chiari I malformation. Childs Nerv Syst. 2011;27:35–40.

    Article  PubMed  Google Scholar 

  8. Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM. Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol. 1986;7(5):795–9.

    CAS  PubMed  Google Scholar 

  9. Tubbs RS, Wellons JC 3rd, Blount JP, et al. Inclination of the odontoid process in the pediatric Chiari I malformation. J Neurosurg. 2003;98(1 Suppl):43–9.

    PubMed  Google Scholar 

  10. Sl B, Drvaric DM, Roberts JM, et al. Scoliosis in syringomyelia. Orthopedics. 1989;12:335–7.

    Google Scholar 

  11. Vega A, Quintana F, Berciano J. Basichoondrocranium anomalies in adult Chiari type I malformation: a morphometric study. J Neurol Sci. 1990;99:137–45.

    Article  CAS  PubMed  Google Scholar 

  12. Williams B. Pathogenesis of syringomyelia. In: Batzdorf U, editor. Syringomyelia: current concepts in diagnosis and treatment. Current neurosurgical practice series, vol. 4. Baltimore: Williams & Wilkins; 1991. p. 59–90.

    Google Scholar 

  13. Williams B. Simultaneous cerebral and spinal fluid pressure records 2. Cerebrospinal dissociation with lesions at the foramen magnum. Acta Neurochir [Wein]. 1981;59:123–42.

    Article  CAS  Google Scholar 

  14. Williams B. Syringomyelia. Neurosurg Clin N Am. 1990;1:653–85.

    CAS  PubMed  Google Scholar 

  15. Oakes WJ, Tubbs RS. Chiari malformation. In: Winn HR, editor. Youmans neurological surgery: a comprehensive guide to diagnosis and management of neurological problems. Ed 5 ed. Philadelphia: WB Saunders; 2003. p. 3347–61.

    Google Scholar 

  16. Oldfield EH, Muraszko K, Shawker TH, Patronas NJ. Pathophysiology of hydrosyringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implication of diagnosis and treatment. J Neurosurg. 1994;80(1):3–15.

    Article  CAS  PubMed  Google Scholar 

  17. Pillay PK, Awad IA, Little JR, Hahn JF. Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance. Neurosurgery. 1991;28(5):639–45.

    Article  CAS  PubMed  Google Scholar 

  18. Tubbs RS, Shoja MM, Ardalan MR, Shokouhi G, Loukas M. Hindbrain herniation: a review of embryological theories. Ital J Anat Embryol. 2008;113(1):37–46.

    PubMed  Google Scholar 

  19. Ogiwara H, Morota N. Surgical decompression without dural opening for symptomatic Chiari type II malformation in young infants. Childs Nerv Syst. 2013;29:1563–7.

    Article  PubMed  Google Scholar 

  20. Isu T, Chono Y, Iwasaki Y, Koyanagi I, Akino M, Abe H, et al. Scoliosis associated with syringomyelia presenting in children. Childs Nerv Syst. 1992;8(2):97–100.

    Article  CAS  PubMed  Google Scholar 

  21. Muhonen MG, Menezes AH, Sawin PD, Weinstein SL. Scoliosis in pediatric Chiari malformations without myelodysplasia. J Neurosurg. 1992;77(1):69–77.

    Article  CAS  PubMed  Google Scholar 

  22. Badie B, Mendoza D, Batzdorf U. Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation. Neurosurgery. 1995;37:214–8.

    Article  CAS  PubMed  Google Scholar 

  23. Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship myelocele. J Neurol Neurosurg Psychiatry. 1965;28:247–59.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Penfield W, Coburn DF. Arnold-Chiari malformation and its operative treatment. Arch Neurol Psychiatr. 1938;40:328–36.

    Article  Google Scholar 

  25. Litvack ZN, Lindsay RA, Selden NR. Dura splitting decompression for Chiari I malformation in pediatric patients: clinical outcomes, healthcare costs, and resource utilization. Neurosurgery. 2013;72:922–9.

    Article  PubMed  Google Scholar 

  26. Horton WA, Hall JG, Hecht JT. Achondroplasia. Lancet. 2007;370(9582):162–72.

    Article  CAS  PubMed  Google Scholar 

  27. Bagley CA, Pindrik JA, Bookland MJ, Camara-Quintana JQ, Carson BS. Cervicomedullary decompression for foramen magnum stenosis in achondroplasia. J Neurosurg. 2006;104(3 Suppl):166–72.

    PubMed  Google Scholar 

  28. Keiper GL Jr, Koch B, Crome KR. Achondroplasia and cervicomedullary compression: prospective evaluation and surgical treatment. Pediatr Neurosurg. 1999;31(2):78–83.

    Article  PubMed  Google Scholar 

  29. Brockmeyer D, Gollogly S, Smith JT. Scoliosis associated with Chiari I malformations: effects of suboccipital decompression on scoliosis curve progression: a preliminary study. Spine. 1976;28:2505–9.

    Article  Google Scholar 

  30. Attenello FJ, McGirt MJ, Garces-Ambrossi GL, Chaichana KL, Carson B, Jallo GI. Suboccipital decompression for Chiari I malformation: outcome comparison of duraplasty with expanded polytetrafluoroethylene dural substitute versus pericranial autograft. Childs Nerv Syst. 2009;25(2):183–90.

    Article  PubMed  Google Scholar 

  31. McLone DG, Dias MS. The Chiari II malformation: cause and impact. Childs Nerv Syst. 2003;19:540–50.

    Article  PubMed  Google Scholar 

  32. Stevenson KL. Chari type II malformation: past, present and future. Neurosurg Focus. 2004;16(2):1–7.

    Article  Google Scholar 

  33. LaMarca F, Herman M, Grant JA, McLone DG. Presentation and management of hydromyelia in children with Chairi type II malformation. Pediatr Neurosurg. 1997;26(2):57–67.

    Article  CAS  Google Scholar 

  34. Akbari SH, Limbrick DD Jr, Kim DH, Narayan P, Leonard JR, Smyth MD, et al. Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst. 2013;29(7):1143–54.

    Article  PubMed  Google Scholar 

  35. James HE, Brant A. Treatment of Chiari malformation with bone decompression without durotomy in children and young adults. Childs Nerv Syst. 2002;18(5):202–6.

    Article  PubMed  Google Scholar 

  36. Samdani AF, Fine AL, Sagoo SS, Shah SC, Cahill PJ, Clements DH, et al. A patient with myelomeningocele: is untethering necessary before scoliosis correction? Neurosurg Focus. 2010;29(1):E8.

    Article  PubMed  Google Scholar 

  37. Adzick NS, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson MP, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364(11):993–1004.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Author information

Authors and Affiliations

  1. Pediatric Neurosurgery, Arnold Palmer Hospital, Orlando, FL, USA

    Christopher A. Gegg MD, FAANS, FACS

  2. Neurosurgery, Arnold Palmer Hospital for Children, Orlando, FL, USA

    Greg Olavarria MD

Authors
  1. Christopher A. Gegg MD, FAANS, FACS
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  2. Greg Olavarria MD
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Corresponding author

Correspondence to Christopher A. Gegg MD, FAANS, FACS .

Editor information

Editors and Affiliations

  1. Harvard Medical School, Boston, Massachusetts, USA

    Daniel J. Hedequist

  2. Nemours/Alfred I. DuPont Hospital for Children, Wilmington, Delaware, USA

    Suken A. Shah

  3. Rady’s Children’s Hospital Associate, San Diego, California, USA

    Burt Yaszay

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Gegg, C.A., Olavarria, G. (2018). Chiari Malformations and Foramen Magnum Stenosis. In: Hedequist, D., Shah, S., Yaszay, B. (eds) The Management of Disorders of the Child’s Cervical Spine. Springer, Boston, MA. https://doi.org/10.1007/978-1-4939-7491-7_20

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  • DOI: https://doi.org/10.1007/978-1-4939-7491-7_20

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  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4939-7489-4

  • Online ISBN: 978-1-4939-7491-7

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