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The Relationship Between Basilar Invagination and Chiari Malformation Type I: A Narrative Review

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Part of the book series: Acta Neurochirurgica Supplement ((NEUROCHIRURGICA,volume 125))

Abstract

Basilar invagination (BI) and Chiari malformation type I CM-I) are the most common adult craniovertebral junction malformations, and they are frequently associated with each other and present synchronously. The relationship between BI and CM-I has remained incompletely understood, and the choice of surgical strategy has remained controversial. This brief review focuses on the different aspects of BI and CM-I, and further discusses the relationship between these two concomitant pathologies on the basis of the concepts proposed over the last three decades.

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References

  1. Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y. Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg. 1997;86(1):40–7.

    Article  CAS  PubMed  Google Scholar 

  2. Ferreira JA, Botelho RV. The odontoid process invagination in normal subjects, Chiari malformation and basilar invagination patients: pathophysiologic correlations with angular craniometry. Surg Neurol Int. 2015;6:118.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Milhorat TH, Nishikawa M, Kula RW, Dlugacz YD. Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as guide to clinical management. Acta Neurochir. 2010;152(7):1117–27.

    Article  PubMed  Google Scholar 

  4. Klimo P Jr, Rao G, Brockmeyer D. Congenital anomalies of the cervical spine. Neurosurg Clin N Am. 2007;18(3):463–78.

    Article  PubMed  Google Scholar 

  5. Menezes AH. Craniocervical developmental anatomy and its implications. Childs Nerv Syst. 2008;24(10):1109–22.

    Article  PubMed  Google Scholar 

  6. Chamberlain WE. Basilar impression (platybasia): a bizarre developmental anomaly of the occipital bone and upper cervical spine with striking and misleading neurologic manifestations. Yale J Biol Med. 1939;11(5):487–96.

    CAS  PubMed  PubMed Central  Google Scholar 

  7. Charnas LR, Marini JC. Communicating hydrocephalus, basilar invagination, and other neurologic features in osteogenesis imperfecta. Neurology. 1993;43(12):2603–8.

    Article  CAS  PubMed  Google Scholar 

  8. Smoker WR. Craniovertebral junction: normal anatomy, craniometry, and congenital anomalies. Radiographics. 1994;14(2):255–77.

    Article  CAS  PubMed  Google Scholar 

  9. Pinter NK, McVige J, Mechtler L. Basilar invagination, basilar impression, and platybasia: clinical and imaging aspects. Curr Pain Headache Rep. 2016;20(8):49.

    Article  PubMed  Google Scholar 

  10. Smoker WR, Khanna G. Imaging the craniocervical junction. Childs Nerv Syst. 2008;24(10):1123–45.

    Article  PubMed  Google Scholar 

  11. Cronin CG, Lohan DG, Mhuircheartigh JN, Meehan CP, Murphy J, Roche C. CT evaluation of Chamberlain’s, McGregor’s, and McRae’s skull-base lines. Clin Radiol. 2009;64(1):64–9.

    Article  CAS  PubMed  Google Scholar 

  12. Mc GM. The significance of certain measurements of the skull in the diagnosis of basilar impression. Br J Radiol. 1948;21(244):171–81.

    Article  Google Scholar 

  13. VanGilder JC, Menezes AH, Dolan KD. The craniovertebral junction and its abnormalities. New York: Futura; 1987.

    Google Scholar 

  14. Menezes AH, VanGilder JC. Anomalies of the craniovertebral junction. Philadelphia: Saunders; 1990.

    Google Scholar 

  15. McRae DL, Barnum AS. Occipitalization of the atlas. Am J Roentgenol Radium Ther Nucl Med. 1953;70(1):23–46.

    CAS  PubMed  Google Scholar 

  16. El Asri AC, Akhaddar A, Gazzaz M, et al. Dynamic CT scan of the craniovertebral junction: a role in the management of os odontoideum. Neurol Neurochir Pol. 2010;44(6):603–8.

    Article  PubMed  Google Scholar 

  17. Gupta V, Khandelwal N, Mathuria SN, Singh P, Pathak A, Suri S. Dynamic magnetic resonance imaging evaluation of craniovertebral junction abnormalities. J Comput Assist Tomogr. 2007;31(3):354–9.

    Article  PubMed  Google Scholar 

  18. Reijnierse M, Breedveld FC, Kroon HM, Hansen B, Pope TL, Bloem JL. Are magnetic resonance flexion views useful in evaluating the cervical spine of patients with rheumatoid arthritis? Skelet Radiol. 2000;29(2):85–9.

    Article  CAS  Google Scholar 

  19. Karhu JO, Parkkola RK, Koskinen SK. Evaluation of flexion/extension of the upper cervical spine in patients with rheumatoid arthritis: an MRI study with a dedicated positioning device compared to conventional radiographs. Acta Radiol. 2005;46(1):55–66.

    Article  CAS  PubMed  Google Scholar 

  20. Bassi P, Corona C, Contri P, Paiocchi A, Loiero M, Mangoni A. Congenital basilar impression: correlated neurological syndromes. Eur Neurol. 1992;32(4):238–43.

    Article  CAS  PubMed  Google Scholar 

  21. Erbengi A, Oge HK. Congenital malformations of the craniovertebral junction: classification and surgical treatment. Acta Neurochir. 1994;127(3–4):180–5.

    Article  CAS  PubMed  Google Scholar 

  22. Goel A, Desai K. Surgery for syringomyelia: an analysis based on 163 surgical cases. Acta Neurochir. 2000;142(3):293–301. discussion 301-292.

    Article  CAS  PubMed  Google Scholar 

  23. Sawin PD, Menezes AH. Basilar invagination in osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management. J Neurosurg. 1997;86(6):950–60.

    Article  CAS  PubMed  Google Scholar 

  24. Smith JS, Shaffrey CI, Abel MF, Menezes AH. Basilar invagination. Neurosurgery. 2010;66(3 Suppl):39–47.

    Article  PubMed  Google Scholar 

  25. Menezes AH. Evaluation and treatment of congenital and developmental anomalies of the cervical spine. Invited submission from the Joint Section Meeting on Disorders of the Spine and Peripheral Nerves, March 2004. J Neurosurg Spine. 2004;1(2):188–97.

    Article  PubMed  Google Scholar 

  26. Goel A, Bhatjiwale M, Desai K. Basilar invagination: a study based on 190 surgically treated patients. J Neurosurg. 1998;88(6):962–8.

    Article  CAS  PubMed  Google Scholar 

  27. Menezes AH. Surgical approaches: postoperative care and complications “transoral–transpalatopharyngeal approach to the craniocervical junction”. Childs Nerv Syst. 2008;24(10):1187–93.

    Article  PubMed  Google Scholar 

  28. Elster AD, Chen MY. Chiari I malformations: clinical and radiologic reappraisal. Radiology. 1992;183(2):347–53.

    Article  CAS  PubMed  Google Scholar 

  29. Aboulezz AO, Sartor K, Geyer CA, Gado MH. Position of cerebellar tonsils in the normal population and in patients with Chiari malformation: a quantitative approach with MR imaging. J Comput Assist Tomogr. 1985;9(6):1033–6.

    Article  CAS  PubMed  Google Scholar 

  30. Armonda RA, Citrin CM, Foley KT, Ellenbogen RG. Quantitative cine-mode magnetic resonance imaging of Chiari I malformations: an analysis of cerebrospinal fluid dynamics. Neurosurgery. 1994;35(2):214–23. discussion 223-214.

    Article  CAS  PubMed  Google Scholar 

  31. Milhorat TH, Chou MW, Trinidad EM, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44(5):1005–17.

    Article  CAS  PubMed  Google Scholar 

  32. Milhorat TH, Bolognese PA, Nishikawa M, et al. Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale. Surg Neurol. 2009;72(1):20–35.

    Article  PubMed  PubMed Central  Google Scholar 

  33. Cinalli G, Spennato P, Sainte-Rose C, et al. Chiari malformation in craniosynostosis. Childs Nerv Syst. 2005;21(10):889–901.

    Article  PubMed  Google Scholar 

  34. Atkinson JL, Weinshenker BG, Miller GM, Piepgras DG, Mokri B. Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases. J Neurosurg. 1998;88(2):237–42.

    Article  CAS  PubMed  Google Scholar 

  35. Elisevich K, Fontaine S, Bertrand G. Syringomyelia as a complication of Paget’s disease. Case report. J Neurosurg. 1987;66(4):611–3.

    Article  CAS  PubMed  Google Scholar 

  36. Lee M, Rezai AR, Wisoff JH. Acquired Chiari-I malformation and hydromyelia secondary to a giant craniopharyngioma. Pediatr Neurosurg. 1995;22(5):251–4.

    Article  CAS  PubMed  Google Scholar 

  37. Morioka T, Shono T, Nishio S, Yoshida K, Hasuo K, Fukui M. Acquired Chiari I malformation and syringomyelia associated with bilateral chronic subdural hematoma. Case report. J Neurosurg. 1995;83(3):556–8.

    Article  CAS  PubMed  Google Scholar 

  38. Rocque BG, Oakes WJ. Surgical treatment of Chiari I malformation. Neurosurg Clin N Am. 2015;26(4):527–31.

    Article  PubMed  Google Scholar 

  39. Fischbein R, Saling JR, Marty P, et al. Patient-reported Chiari malformation type I symptoms and diagnostic experiences: a report from the national Conquer Chiari Patient Registry database. Neurol Sci. 2015;36(9):1617–24.

    Article  PubMed  Google Scholar 

  40. Mueller DM, Oro JJ. Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari malformation type I with or without syringomyelia. J Am Acad Nurse Pract. 2004;16(3):134–8.

    Article  PubMed  Google Scholar 

  41. Hofkes SK, Iskandar BJ, Turski PA, Gentry LR, McCue JB, Haughton VM. Differentiation between symptomatic Chiari I malformation and asymptomatic tonsilar ectopia by using cerebrospinal fluid flow imaging: initial estimate of imaging accuracy. Radiology. 2007;245(2):532–40.

    Article  PubMed  Google Scholar 

  42. Tubbs RS, Lyerly MJ, Loukas M, Shoja MM, Oakes WJ. The pediatric Chiari I malformation: a review. Childs Nerv Syst. 2007;23(11):1239–50.

    Article  PubMed  Google Scholar 

  43. Panigrahi M, Reddy BP, Reddy AK, Reddy JJ. CSF flow study in Chiari I malformation. Childs Nerv Syst. 2004;20(5):336–40.

    Article  CAS  PubMed  Google Scholar 

  44. Tubbs RS. Definitions and anatomic considerations in Chiari I malformation and associated syringomyelia. Neurosurg Clin N Am. 2015;26(4):487–93.

    Article  PubMed  Google Scholar 

  45. Tubbs RS, Beckman J, Naftel RP, 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 

  46. Kagawa M, Jinnai T, Matsumoto Y, et al. Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia: case report. Surg Neurol. 2006;65(5):497–502. discussion 502

    Article  PubMed  Google Scholar 

  47. Rocque BG, George TM, Kestle J, Iskandar BJ. Treatment practices for Chiari malformation type I with syringomyelia: results of a survey of the American Society of Pediatric Neurosurgeons. J Neurosurg Pediatr. 2011;8(5):430–7.

    Article  PubMed  Google Scholar 

  48. Doughty KE, Tubbs RS, Webb D, Oakes WJ. Delayed resolution of Chiari I–associated hydromyelia after posterior fossa decompression: case report and review of the literature. Neurosurgery. 2004;55(3):711.

    Article  PubMed  Google Scholar 

  49. Klekamp J. Surgical treatment of Chiari I malformation—analysis of intraoperative findings, complications, and outcome for 371 foramen magnum decompressions. Neurosurgery. 2012;71(2):365–80. discussion 380.

    Article  PubMed  Google Scholar 

  50. Sakushima K, Tsuboi S, Yabe I, et al. Nationwide survey on the epidemiology of syringomyelia in Japan. J Neurol Sci. 2012;313(1–2):147–52.

    Article  PubMed  Google Scholar 

  51. Schijman E, Steinbok P. International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst. 2004;20(5):341–8.

    Article  PubMed  Google Scholar 

  52. Durham SR, Fjeld-Olenec K. Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation type I in pediatric patients: a meta-analysis. J Neurosurg Pediatr. 2008;2(1):42–9.

    Article  PubMed  Google Scholar 

  53. Hankinson T, Tubbs RS, Wellons JC. Duraplasty or not? An evidence-based review of the pediatric Chiari I malformation. Childs Nerv Syst. 2011;27(1):35–40.

    Article  PubMed  Google Scholar 

  54. Hayhurst C, Richards O, Zaki H, Findlay G, Pigott TJ. Hindbrain decompression for Chiari–syringomyelia complex: an outcome analysis comparing surgical techniques. Br J Neurosurg. 2008;22(1):86–91.

    Article  CAS  PubMed  Google Scholar 

  55. Menezes AH, Greenlee JD, Donovan KA. Honored guest presentation: lifetime experiences and where we are going: Chiari I with syringohydromyelia–controversies and development of decision trees. Clin Neurosurg. 2005;52:297–305.

    PubMed  Google Scholar 

  56. Milhorat TH, Bolognese PA. Tailored operative technique for Chiari type I malformation using intraoperative color Doppler ultrasonography. Neurosurgery. 2003;53(4):899–905. discussion 905-896.

    Article  PubMed  Google Scholar 

  57. Goel A. Is atlantoaxial instability the cause of Chiari malformation? Outcome analysis of 65 patients treated by atlantoaxial fixation. J Neurosurg Spine. 2015;22(2):116–27.

    Article  PubMed  Google Scholar 

  58. Goel A. Atlantoaxial facetal distraction spacers: indications and techniques. J Craniovertebr Junction Spine. 2016;7(3):127–8.

    Article  PubMed  PubMed Central  Google Scholar 

  59. Goel A. Basilar invagination, Chiari malformation, syringomyelia: a review. Neurol India. 2009;57(3):235–46.

    Article  PubMed  Google Scholar 

  60. Visocchi M, Trevisi G, Iacopino DG, Tamburrini G, Caldarelli M, Barbagallo GM. Odontoid process and clival regeneration with Chiari malformation worsening after transoral decompression: an unexpected and previously unreported cause of “accordion phenomenon”. Eur Spine J. 2015;24(Suppl 4):S564–8.

    Article  PubMed  Google Scholar 

  61. Klekamp J. Chiari I malformation with and without basilar invagination: a comparative study. Neurosurg Focus. 2015;38(4):E12.

    Article  PubMed  Google Scholar 

  62. Aghakhani N, Parker F, David P, et al. Long-term follow-up of Chiari-related syringomyelia in adults: analysis of 157 surgically treated cases. Neurosurgery. 2009;64(2):308–15. discussion 315

    Article  PubMed  Google Scholar 

  63. Arora P, Behari S, Banerji D, Chhabra DK, Jain VK. Factors influencing the outcome in symptomatic Chiari I malformation. Neurol India. 2004;52(4):470–4.

    PubMed  Google Scholar 

  64. Klekamp J. Neurological deterioration after foramen magnum decompression for Chiari malformation type I: old or new pathology? J Neurosurg Pediatr. 2012;10(6):538–47.

    Article  PubMed  Google Scholar 

  65. Naftel RP, Tubbs RS, Menendez JY, Wellons JC 3rd, Pollack IF, Oakes WJ. Worsening or development of syringomyelia following Chiari I decompression: case report. J Neurosurg Pediatr. 2013;12(4):351–6.

    Article  PubMed  Google Scholar 

  66. Goldstein HE, Anderson RC. Craniovertebral junction instability in the setting of Chiari I malformation. Neurosurg Clin N Am. 2015;26(4):561–9.

    Article  PubMed  Google Scholar 

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Liao, C. et al. (2019). The Relationship Between Basilar Invagination and Chiari Malformation Type I: A Narrative Review. In: Visocchi, M. (eds) New Trends in Craniovertebral Junction Surgery. Acta Neurochirurgica Supplement, vol 125. Springer, Cham. https://doi.org/10.1007/978-3-319-62515-7_16

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  • DOI: https://doi.org/10.1007/978-3-319-62515-7_16

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