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Chiari Malformation

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Incidental Findings of the Nervous System

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Abstract

Chiari malformation (CM) is a clinical condition ranging from herniation of the cerebellar tonsils through the foramen magnum to syrinx, hydrocephalus, spinal dysraphism, and encephalocele. The etiology and pathogenesis of these malformations are still not fully understood and are a matter of debate. Four different types of CM were defined by Hans Chiari. However, new variants such as type 3.5, type 5, and complex CM were distinguished subsequently. CM type 1, which is usually asymptomatic, is a neurological structural anomaly that includes cerebellar herniation along the foramen magnum. Because it is generally asymptomatic and imaging methods were not common, the rate of diagnosis was previously well below its prevalence. Diagnosis has become easier in recent years with the widespread and easy accessibility of imaging methods. Thanks to the increasing use of such methods for investigating cranial and cervical pathologies, the diagnosis of incidental tonsillar ectopia has also increased. Magnetic resonance imaging is the most useful and widely used method for diagnosing CM. There are surgical and conservative approaches to treatment selection, but the choice of treatment and surgical technique remains controversial. Prognosis for CM varies depending on the type and on individual pathologies.

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References

  1. Vernooij MW, Ikram MA, Tanghe HL, et al. Incidental findings on brain MRI in the general population. N Engl J Med. 2007;357(18):1821–8. https://doi.org/10.1056/NEJMoa070972.

    Article  CAS  PubMed  Google Scholar 

  2. Arnautovic A, Splavski B, Boop FA, Arnautovic KI. Pediatric and adult Chiari malformation Type I surgical series 1965-2013: a review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr. 2015;15(2):161–77. https://doi.org/10.3171/2014.10.PEDS14295.

    Article  PubMed  Google Scholar 

  3. Taylor FR, Larkins MV. Headache and Chiari I malformation: clinical presentation, diagnosis, and controversies in management. Curr Pain Headache Rep. 2002;6(4):331–7. https://doi.org/10.1007/s11916-002-0056-z.

    Article  PubMed  Google Scholar 

  4. Milhorat TH. Cerebrospinal fluid physiology. In: Milhorat TH, editor. Hydrocephalus and the cerebrospinal fluid. Baltimore: Williams & Wilkins; 1972. p. 1–41.

    Google Scholar 

  5. 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  PubMed Central  Google Scholar 

  6. Paul KS, Lye RH, Strang FA, Dutton J. Arnold-Chiari malformation. Review of 71 cases. J Neurosurg. 1983;58(2):183–7. https://doi.org/10.3171/jns.1983.58.2.0183.

    Article  CAS  PubMed  Google Scholar 

  7. Fisahn C, Shoja MM, Turgut M, Oskouian RJ, Oakes WJ, Tubbs RS. The Chiari 3.5 malformation: a review of the only reported case. Childs Nerv Syst. 2016;32(12):2317–9. https://doi.org/10.1007/s00381-016-3255-3.

    Article  PubMed  Google Scholar 

  8. Semple DA, McClure JH. Arnold-chiari malformation in pregnancy. Anaesthesia. 1996;51(6):580–2. https://doi.org/10.1111/j.1365-2044.1996.tb12570.x.

    Article  CAS  PubMed  Google Scholar 

  9. Tubbs RS, Turgut M. Defining the Chiari malformations: past and newer classifications. In: Tubbs RS, Turgut M, Oakes WJ, editors. The Chiari malformations. Springer Nature Switzerland AG; 2020. p. 21–39.

    Chapter  Google Scholar 

  10. Brockmeyer DL, Spader HS. Complex Chiari malformations in children: diagnosis and management. Neurosurg Clin N Am. 2015;26(4):555–60. https://doi.org/10.1016/j.nec.2015.06.002.

    Article  PubMed  Google Scholar 

  11. Turgut M, Sağıroğlu S. Suboccipital decompressive craniectomy in a case of complex Chiari malformation complicated with influenza a (H1N1) necrotizing encephalopathy. Childs Nerv Syst. 2020;36(7):1335–6. https://doi.org/10.1007/s00381-020-04659-7.

    Article  PubMed  Google Scholar 

  12. Tubbs RS, Muhleman M, Loukas M, Oakes WJ. A new form of herniation: the Chiari V malformation. Childs Nerv Syst. 2012;28(2):305–7. https://doi.org/10.1007/s00381-011-1616-5.

    Article  PubMed  Google Scholar 

  13. Cavender RK, Schmidt JH 3rd. Tonsillar ectopia and Chiari malformations: monozygotic triplets. Case report. J Neurosurg. 1995;82(3):497–500. https://doi.org/10.3171/jns.1995.82.3.0497.

    Article  CAS  PubMed  Google Scholar 

  14. Turgut M. Chiari type I malformation in two monozygotic twins. Br J Neurosurg. 2001;15(3):279–80. https://doi.org/10.1080/026886901750353773.

    Article  CAS  PubMed  Google Scholar 

  15. Speer MC, Enterline DS, Mehltretter L, et al. Review article: Chiari type I malformation with or without syringomyelia: prevalence and genetics. J Genet Couns. 2003;12(4):297–311. https://doi.org/10.1023/A:1023948921381.

    Article  PubMed  Google Scholar 

  16. Braca J, Hornyak M, Murali R. Hemifacial spasm in a patient with Marfan syndrome and Chiari I malformation. Case report. J Neurosurg. 2005;103(3):552–4. https://doi.org/10.3171/jns.2005.103.3.0552.

    Article  PubMed  Google Scholar 

  17. Newman PK, Wentzel J, Foster JB. HLA and syringomyelia. J Neuroimmunol. 1982;3(1):23–6. https://doi.org/10.1016/0165-5728(82)90015-7.

    Article  CAS  PubMed  Google Scholar 

  18. Elster AD, Chen MY. Chiari I malformations: clinical and radiologic reappraisal. Radiology. 1992;183(2):347–53. https://doi.org/10.1148/radiology.183.2.1561334.

    Article  CAS  PubMed  Google Scholar 

  19. Guo F, Turgut M. Precise management of Chiari malformation with Type I. Front Surg. 2022;9:850879. Published 2022 Mar 28. https://doi.org/10.3389/fsurg.2022.850879.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Krucoff MO, Cook S, Adogwa O, et al. Racial, socioeconomic, and gender disparities in the presentation, treatment, and outcomes of adult Chiari I malformations. World Neurosurg. 2017;97:431–7. https://doi.org/10.1016/j.wneu.2016.10.026.

    Article  PubMed  Google Scholar 

  21. McLone DG, Knepper PA. The cause of Chiari II malformation: a unified theory. Pediatr Neurosci. 1989;15(1):1–12. https://doi.org/10.1159/000120432.

    Article  CAS  PubMed  Google Scholar 

  22. Yadav YR, Parihar V, Sinha M. Lumbar peritoneal shunt. Neurol India. 2010;58(2):179–84. https://doi.org/10.4103/0028-3886.63778.

    Article  PubMed  Google Scholar 

  23. Banerji NK, Millar JH. Chiari malformation presenting in adult life. Its relationship to syringomyelia. Brain. 1974;97(1):157–68. https://doi.org/10.1093/brain/97.1.157.

    Article  CAS  PubMed  Google Scholar 

  24. 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 

  25. Ciaramitaro P, Ferraris M, Massaro F, Garbossa D. Clinical diagnosis-part I: what is really caused by Chiari I. Childs Nerv Syst. 2019;35(10):1673–9. https://doi.org/10.1007/s00381-019-04206-z.

    Article  PubMed  Google Scholar 

  26. Tachibana S, Harada K, Abe T, Yamada H, Yokota A. Syringomyelia secondary to tonsillar herniation caused by posterior fossa tumors. Surg Neurol. 1995;43(5):470–7. https://doi.org/10.1016/0090-3019(95)80092-u.

    Article  CAS  PubMed  Google Scholar 

  27. Chiari H. Ueber Veranderungen des Kleinhirns in folge von Hydrocephalie des Grosshirns. Dtsch Med Wochenschr. 1891;17:1172–5.

    Article  Google Scholar 

  28. Tubbs RS, Turgut M, Oakes WJ. A history of the Chiari malformations. In: Tubbs RS, Turgut M, Oakes WJ, editors. The Chiari malformations. Springer Nature Switzerland AG; 2020. p. 3–20.

    Chapter  Google Scholar 

  29. Gardner WJ, Goodall RJ. The surgical treatment of Arnold-Chiari malformation in adults; an explanation of its mechanism and importance of encephalography in diagnosis. J Neurosurg. 1950;7(3):199–206. https://doi.org/10.3171/jns.1950.7.3.0199.

    Article  CAS  PubMed  Google Scholar 

  30. Moscote-Salazar LR, Zabaleta-Churio N, Alcala-Cerra G, et al. Symptomatic Chiari malformation with syringomyelia after severe traumatic brain injury: case report. Bull Emerg Trauma. 2016;4(1):58–61.

    PubMed  PubMed Central  Google Scholar 

  31. 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. https://doi.org/10.3171/2014.10.SPINE14176.

    Article  PubMed  Google Scholar 

  32. Tubbs RS, Turgut M, Oakes WJ. Conclusions. In: Tubbs RS, Turgut M, Oakes WJ, editors. The Chiari malformations. Springer Nature Switzerland AG; 2020. p. 611–3.

    Chapter  Google Scholar 

  33. 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. https://doi.org/10.3171/jns.1997.86.1.0040.

    Article  CAS  PubMed  Google Scholar 

  34. 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. https://doi.org/10.1007/s00701-010-0636-3.

    Article  PubMed  Google Scholar 

  35. Acer N, Turgut M, Yılmaz S, Güler HS. Measurement of the volume of the posterior cranial fossa using MRI. In: Tubbs RS, Turgut M, Oakes WJ, editors. The chiari malformations. Springer Nature Switzerland AG; 2020. p. 329–39.

    Chapter  Google Scholar 

  36. Tubbs RS, McGirt MJ, Oakes WJ. Surgical experience in 130 pediatric patients with Chiari I malformations. J Neurosurg. 2003;99(2):291–6. https://doi.org/10.3171/jns.2003.99.2.0291.

    Article  PubMed  Google Scholar 

  37. Williams H. A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida. Cerebrospinal Fluid Res. 2008;5:7. Published 2008 Apr 11. https://doi.org/10.1186/1743-8454-5-7.

    Article  PubMed  PubMed Central  Google Scholar 

  38. Di Rocco C, Frassanito P, Massimi L, Peraio S. Hydrocephalus and Chiari type I malformation. Childs Nerv Syst. 2011;27(10):1653–64. https://doi.org/10.1007/s00381-011-1545-3.

    Article  PubMed  Google Scholar 

  39. Albert L Jr, Hirschfeld A. Acquired Chiari malformation secondary to hyperostosis of the skull: a case report and literature review. Surg Neurol. 2009;72(2):157–61. https://doi.org/10.1016/j.surneu.2008.02.030.

    Article  PubMed  Google Scholar 

  40. Pascual J, Oterino A, Berciano J. Headache in type I Chiari malformation. Neurology. 1992;42(8):1519–21. https://doi.org/10.1212/wnl.42.8.1519.

    Article  CAS  PubMed  Google Scholar 

  41. 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. https://doi.org/10.1097/00006123-199905000-00042.

    Article  CAS  PubMed  Google Scholar 

  42. Goodwin D, Halvorson AR. Chiari I malformation presenting as downbeat nystagmus: clinical presentation, diagnosis, and management. Optometry. 2012;83(2):80–6. Published 2012 Feb 15

    PubMed  Google Scholar 

  43. Dahdaleh NS, Menezes AH. Incomplete lateral medullary syndrome in a patient with Chiari malformation Type I presenting with combined trigeminal and vagal nerve dysfunction. J Neurosurg Pediatr. 2008;2(4):250–3. https://doi.org/10.3171/PED.2008.2.10.250.

    Article  PubMed  Google Scholar 

  44. Turgut M. Pierre-Robin syndrome associated with Chiari type I malformation. Childs Nerv Syst. 2004;20(1):3–7. https://doi.org/10.1007/s00381-003-0830-1.

    Article  PubMed  Google Scholar 

  45. Martins HA, Ribas VR, Lima MD, et al. Headache precipitated by Valsalva maneuvers in patients with congenital Chiari I malformation. Arq Neuropsiquiatr. 2010;68(3):406–9. https://doi.org/10.1590/s0004-282x2010000300015.

    Article  PubMed  Google Scholar 

  46. Martinot A, Hue V, Leclerc F, Vallee L, Closset M, Pruvo JP. Sudden death revealing Chiari type 1 malformation in two children. Intensive Care Med. 1993;19(2):73–4. https://doi.org/10.1007/BF01708364.

    Article  CAS  PubMed  Google Scholar 

  47. Tubbs RS, Oakes WJ. Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst. 2004;20(6):375–81. https://doi.org/10.1007/s00381-004-0969-4.

    Article  PubMed  Google Scholar 

  48. McLone DG, Dias MS. The Chiari II malformation: cause and impact. Childs Nerv Syst. 2003;19(7–8):540–50. https://doi.org/10.1007/s00381-003-0792-3.

    Article  PubMed  Google Scholar 

  49. Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO. Natural history of Chiari malformation type I following decision for conservative treatment. J Neurosurg Pediatr. 2011;8(2):214–21. https://doi.org/10.3171/2011.5.PEDS1122.

    Article  PubMed  Google Scholar 

  50. Turgut M, Tubbs RS. Chiari I malformation and craniosynostosis. In: Tubbs RS, Turgut M, Oakes WJ, editors. The Chiari Malformations. Springer Nature Switzerland AG; 2020. p. 239–59.

    Chapter  Google Scholar 

  51. Wu YW, Chin CT, Chan KM, Barkovich AJ, Ferriero DM. Pediatric Chiari I malformations: do clinical and radiologic features correlate? Neurology. 1999;53(6):1271–6. https://doi.org/10.1212/wnl.53.6.1271.

    Article  CAS  PubMed  Google Scholar 

  52. Jenkinson MD, Campbell S, Hayhurst C, et al. Cognitive and functional outcome in spina bifida-Chiari II malformation. Childs Nerv Syst. 2011;27(6):967–74. https://doi.org/10.1007/s00381-010-1368-7.

    Article  PubMed  Google Scholar 

  53. Dyste GN, Menezes AH, VanGilder JC. Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg. 1989;71(2):159–68. https://doi.org/10.3171/jns.1989.71.2.0159.

    Article  CAS  PubMed  Google Scholar 

  54. Badie B, Mendoza D, Batzdorf U. Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation. Neurosurgery. 1995;37(2):214–8. https://doi.org/10.1227/00006123-199508000-00004.

    Article  CAS  PubMed  Google Scholar 

  55. Killeen A, Roguski M, Chavez A, Heilman C, Hwang S. Non-operative outcomes in Chiari I malformation patients. J Clin Neurosci. 2015;22(1):133–8. https://doi.org/10.1016/j.jocn.2014.06.008.

    Article  PubMed  Google Scholar 

  56. Kalb S, Perez-Orribo L, Mahan M, Theodore N, Nakaji P, Bristol RE. Evaluation of operative procedures for symptomatic outcome after decompression surgery for Chiari type I malformation. J Clin Neurosci. 2012;19(9):1268–72. https://doi.org/10.1016/j.jocn.2012.01.025.

    Article  PubMed  Google Scholar 

  57. Giammattei L, Messerer M, Daniel RT, Aghakhani N, Parker F. Long-term outcome of surgical treatment of Chiari malformation without syringomyelia. J Neurosurg Sci. 2020;64(4):364–8. https://doi.org/10.23736/S0390-5616.17.04063-2.

    Article  PubMed  Google Scholar 

  58. Akbari SH, Limbrick DD Jr, Kim DH, et al. Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst. 2013;29(7):1143–54. https://doi.org/10.1007/s00381-013-2040-9.

    Article  PubMed  Google Scholar 

  59. Cameron AH. The Arnold-Chiari and other neuroanatomical malformations associated with spina bifida. J Pathol Bacteriol. 1957;73:195–211.

    Article  Google Scholar 

  60. Salman MS. Posterior fossa decompression and the cerebellum in Chiari type II malformation: a preliminary MRI study. Childs Nerv Syst. 2011;27(3):457–62. https://doi.org/10.1007/s00381-010-1359-8.

    Article  PubMed  PubMed Central  Google Scholar 

  61. Pollack IF, Pang D, Albright AL, Krieger D. Outcome following hindbrain decompression of symptomatic Chiari malformations in children previously treated with myelomeningocele closure and shunts. J Neurosurg. 1992;77(6):881–8. https://doi.org/10.3171/jns.1992.77.6.0881.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neurosurgery, Aydın Adnan Menderes University Faculty of Medicine, Efeler, Aydın, Turkey

    Mehmet Turgut & Ahmet Kürşat Kara

  2. Department of Histology and Embryology, Aydın Adnan Menderes University Health Sciences Institute, Efeler, Aydın, Turkey

    Mehmet Turgut

  3. Department of Neurosurgery, Tulane University School of Medicine, New Orleans, LA, USA

    R. Shane Tubbs

  4. Department of Structural and Cell Biology, Tulane University School of Medicine, New Orleans, LA, USA

    R. Shane Tubbs

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  1. Mehmet Turgut
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  3. R. Shane Tubbs
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Editors and Affiliations

  1. Department of Neurosurgery, Aydın Adnan Menderes University Faculty of Medicine, Aydın, Türkiye

    Mehmet Turgut

  2. Department of Neurosurgery, First Affiliated Hospital of Zhengzhou, Hena, China

    Fuyou Guo

  3. Department of Radiology, Ankara Medipol University Faculty of Medicine, Çankaya, Ankara, Türkiye

    Ahmet Tuncay Turgut

  4. Department of Neurosurgery Sanjay Gandhi, Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

    Sanjay Behari

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Turgut, M., Kara, A.K., Tubbs, R.S. (2023). Chiari Malformation. In: Turgut, M., Guo, F., Turgut, A.T., Behari, S. (eds) Incidental Findings of the Nervous System. Springer, Cham. https://doi.org/10.1007/978-3-031-42595-0_15

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