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

  • Chenlong Liao
  • Massimiliano Visocchi
  • Wenchuan Zhang
  • Shiting Li
  • Min Yang
  • Wenxiang Zhong
  • Pengfei Liu
Part of the Acta Neurochirurgica Supplement book series (NEUROCHIRURGICA, volume 125)


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.


Basilar invagination Chiari I malformation Craniovertebral junction Posterior cranial fossa 


  1. 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.PubMedCrossRefGoogle Scholar
  2. 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.PubMedPubMedCentralCrossRefGoogle Scholar
  3. 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.PubMedCrossRefGoogle Scholar
  4. 4.
    Klimo P Jr, Rao G, Brockmeyer D. Congenital anomalies of the cervical spine. Neurosurg Clin N Am. 2007;18(3):463–78.PubMedCrossRefGoogle Scholar
  5. 5.
    Menezes AH. Craniocervical developmental anatomy and its implications. Childs Nerv Syst. 2008;24(10):1109–22.PubMedCrossRefGoogle Scholar
  6. 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.PubMedPubMedCentralGoogle Scholar
  7. 7.
    Charnas LR, Marini JC. Communicating hydrocephalus, basilar invagination, and other neurologic features in osteogenesis imperfecta. Neurology. 1993;43(12):2603–8.PubMedCrossRefGoogle Scholar
  8. 8.
    Smoker WR. Craniovertebral junction: normal anatomy, craniometry, and congenital anomalies. Radiographics. 1994;14(2):255–77.PubMedCrossRefGoogle Scholar
  9. 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.PubMedCrossRefGoogle Scholar
  10. 10.
    Smoker WR, Khanna G. Imaging the craniocervical junction. Childs Nerv Syst. 2008;24(10):1123–45.PubMedCrossRefGoogle Scholar
  11. 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.PubMedCrossRefGoogle Scholar
  12. 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.CrossRefGoogle Scholar
  13. 13.
    VanGilder JC, Menezes AH, Dolan KD. The craniovertebral junction and its abnormalities. New York: Futura; 1987.Google Scholar
  14. 14.
    Menezes AH, VanGilder JC. Anomalies of the craniovertebral junction. Philadelphia: Saunders; 1990.Google Scholar
  15. 15.
    McRae DL, Barnum AS. Occipitalization of the atlas. Am J Roentgenol Radium Ther Nucl Med. 1953;70(1):23–46.PubMedGoogle Scholar
  16. 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.PubMedCrossRefGoogle Scholar
  17. 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.PubMedCrossRefGoogle Scholar
  18. 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.CrossRefGoogle Scholar
  19. 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.PubMedCrossRefGoogle Scholar
  20. 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.PubMedCrossRefGoogle Scholar
  21. 21.
    Erbengi A, Oge HK. Congenital malformations of the craniovertebral junction: classification and surgical treatment. Acta Neurochir. 1994;127(3–4):180–5.PubMedCrossRefGoogle Scholar
  22. 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.PubMedCrossRefGoogle Scholar
  23. 23.
    Sawin PD, Menezes AH. Basilar invagination in osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management. J Neurosurg. 1997;86(6):950–60.PubMedCrossRefGoogle Scholar
  24. 24.
    Smith JS, Shaffrey CI, Abel MF, Menezes AH. Basilar invagination. Neurosurgery. 2010;66(3 Suppl):39–47.PubMedCrossRefGoogle Scholar
  25. 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.PubMedCrossRefGoogle Scholar
  26. 26.
    Goel A, Bhatjiwale M, Desai K. Basilar invagination: a study based on 190 surgically treated patients. J Neurosurg. 1998;88(6):962–8.PubMedCrossRefGoogle Scholar
  27. 27.
    Menezes AH. Surgical approaches: postoperative care and complications “transoral–transpalatopharyngeal approach to the craniocervical junction”. Childs Nerv Syst. 2008;24(10):1187–93.PubMedCrossRefGoogle Scholar
  28. 28.
    Elster AD, Chen MY. Chiari I malformations: clinical and radiologic reappraisal. Radiology. 1992;183(2):347–53.PubMedCrossRefGoogle Scholar
  29. 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.PubMedCrossRefGoogle Scholar
  30. 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.PubMedCrossRefGoogle Scholar
  31. 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.PubMedCrossRefGoogle Scholar
  32. 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.PubMedPubMedCentralCrossRefGoogle Scholar
  33. 33.
    Cinalli G, Spennato P, Sainte-Rose C, et al. Chiari malformation in craniosynostosis. Childs Nerv Syst. 2005;21(10):889–901.PubMedCrossRefGoogle Scholar
  34. 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.PubMedCrossRefGoogle Scholar
  35. 35.
    Elisevich K, Fontaine S, Bertrand G. Syringomyelia as a complication of Paget’s disease. Case report. J Neurosurg. 1987;66(4):611–3.PubMedCrossRefGoogle Scholar
  36. 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.PubMedCrossRefGoogle Scholar
  37. 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.PubMedCrossRefGoogle Scholar
  38. 38.
    Rocque BG, Oakes WJ. Surgical treatment of Chiari I malformation. Neurosurg Clin N Am. 2015;26(4):527–31.PubMedCrossRefGoogle Scholar
  39. 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.PubMedCrossRefGoogle Scholar
  40. 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.PubMedCrossRefGoogle Scholar
  41. 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.PubMedCrossRefGoogle Scholar
  42. 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.PubMedCrossRefGoogle Scholar
  43. 43.
    Panigrahi M, Reddy BP, Reddy AK, Reddy JJ. CSF flow study in Chiari I malformation. Childs Nerv Syst. 2004;20(5):336–40.PubMedCrossRefGoogle Scholar
  44. 44.
    Tubbs RS. Definitions and anatomic considerations in Chiari I malformation and associated syringomyelia. Neurosurg Clin N Am. 2015;26(4):487–93.PubMedCrossRefGoogle Scholar
  45. 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.PubMedCrossRefGoogle Scholar
  46. 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 502PubMedCrossRefGoogle Scholar
  47. 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.PubMedCrossRefGoogle Scholar
  48. 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.PubMedCrossRefGoogle Scholar
  49. 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.PubMedCrossRefGoogle Scholar
  50. 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.PubMedCrossRefGoogle Scholar
  51. 51.
    Schijman E, Steinbok P. International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst. 2004;20(5):341–8.PubMedCrossRefGoogle Scholar
  52. 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.PubMedCrossRefGoogle Scholar
  53. 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.PubMedCrossRefGoogle Scholar
  54. 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.PubMedCrossRefGoogle Scholar
  55. 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.PubMedGoogle Scholar
  56. 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.PubMedCrossRefGoogle Scholar
  57. 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.PubMedCrossRefGoogle Scholar
  58. 58.
    Goel A. Atlantoaxial facetal distraction spacers: indications and techniques. J Craniovertebr Junction Spine. 2016;7(3):127–8.PubMedPubMedCentralCrossRefGoogle Scholar
  59. 59.
    Goel A. Basilar invagination, Chiari malformation, syringomyelia: a review. Neurol India. 2009;57(3):235–46.PubMedCrossRefGoogle Scholar
  60. 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.PubMedCrossRefGoogle Scholar
  61. 61.
    Klekamp J. Chiari I malformation with and without basilar invagination: a comparative study. Neurosurg Focus. 2015;38(4):E12.PubMedCrossRefGoogle Scholar
  62. 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 315PubMedCrossRefGoogle Scholar
  63. 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.PubMedGoogle Scholar
  64. 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.PubMedCrossRefGoogle Scholar
  65. 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.PubMedCrossRefGoogle Scholar
  66. 66.
    Goldstein HE, Anderson RC. Craniovertebral junction instability in the setting of Chiari I malformation. Neurosurg Clin N Am. 2015;26(4):561–9.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  • Chenlong Liao
    • 1
  • Massimiliano Visocchi
    • 2
  • Wenchuan Zhang
    • 1
  • Shiting Li
    • 1
  • Min Yang
    • 1
  • Wenxiang Zhong
    • 1
  • Pengfei Liu
    • 1
  1. 1.Department of NeurosurgeryXinhua Hospital, Affiliated to Shanghai Jiao Tong University School of MedicineShanghaiP. R. China
  2. 2.Department of NeurosurgeryCatholic University School of MedicineRomeItaly

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