The site of the Media Sphera Publishers contains materials intended solely for healthcare professionals.
By closing this message, you confirm that you are a certified medical professional or a student of a medical educational institution.

Bogdanov É.I.

Kazan State Medical University;
Republican Clinical Hospital

Clinical significance of subthreshold cerebellar tonsil ectopia into foramen magnum and Chiari malformation type 0

Authors:

Bogdanov É.I.

More about the authors

Journal: Burdenko's Journal of Neurosurgery. 2022;86(3): 92‑98

Read: 9508 times


To cite this article:

Bogdanov ÉI. Clinical significance of subthreshold cerebellar tonsil ectopia into foramen magnum and Chiari malformation type 0. Burdenko's Journal of Neurosurgery. 2022;86(3):92‑98. (In Russ., In Engl.)
https://doi.org/10.17116/neiro20228603192

Recommended articles:
Drop atta­cks: diagnostic crossovers and rare causes. S.S. Korsakov Journal of Neurology and Psychiatry. 2026;(3):174-179

References:

  1. Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM. Significance of cerebellar tonsillar position on MR. AJNR. American Journal of Neuroradiology. 1986;7(5):795-799. 
  2. Elster AD, Chen MY. Chiari I malformations: clinical and radiologic reappraisal. Radiology. 1992;183(2):347-353.  https://doi.org/10.1148/radiology.183.2.1561334
  3. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, Speer MC. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44(5):1005-1017. https://doi.org/10.1097/00006123-199905000-00042
  4. Milhorat TH, Nishikawa M, Kula RW, Dlugacz YD. Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as guide to clinical management. Acta Neurochirurgica. 2010;152(7):1117-1127. https://doi.org/10.1007/s00701-010-0636-3
  5. Bolognese PA, Brodbelt A, Bloom AB, Kula RW. Chiari I Malformation: Opinions on Diagnostic Trends and Controversies from a Panel of 63 International Experts. World Neurosurgery. 2019;130:9-16.  https://doi.org/10.1016/j.wneu.2019.05.098
  6. Azahraa Haddad F, Qaisi I, Joudeh N, Dajani H, Jumah F, Elmashala A, Adeeb N, Chern JJ, Tubbs RS. The newer classifications of the hiari malformations with clarifications: An anatomical review. Clinical Anatomy (New York, N.Y.). 2018;31(3):314-322.  https://doi.org/10.1002/ca.23051
  7. Morgenstern PF, Tosi U, Uribe-Cardenas R, Greenfield JP. Ventrolateral Tonsillar Position Defines Novel Chiari 0.5 Classification. World Neurosurgery. 2020;136:444-453.  https://doi.org/10.1016/j.wneu.2020.01.147
  8. Fayzutdinova AT, Bogdanov EI. Clinical and radiological substantiation of the allocation of subtypes of primary Chiari malformation type 1. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova. 2020;120(8):64-69. (In Russ.). https://doi.org/10.17116/jnevro202012008164
  9. Markunas CA, Tubbs RS, Moftakhar R, Ashley-Koch AE, Gregory SG, Oakes WJ, Speer MC, Iskandar BJ. Clinical, radiological, and genetic similarities between patients with Chiari Type I and Type 0 malformations. Journal of Neurosurgery. Pediatrics. 2012;9(4):372-378.  https://doi.org/10.3171/2011.12.PEDS11113
  10. Musolf AM, Ho WSC, Long KA, Zhuang Z, Argersinger DP, Sun H, Moiz BA, Simpson CL, Mendelevich EG, Bogdanov EI, Bailey-Wilson JE, Heiss JD. Small posterior fossa in Chiari I malformation affected families is significantly linked to 1q43-44 and 12q23-24.11 using whole exome sequencing. European Journal of Human Genetics: EJHG. 2019;27(10):1599-1610. https://doi.org/10.1038/s41431-019-0457-7
  11. Urbizu A, Martin BA, Moncho D, Rovira A, Poca MA, Sahuquillo J, Macaya A, Español MI. Machine learning applied to neuroimaging for diagnosis of adult classic Chiari malformation: role of the basion as a key morphometric indicator. Journal of Neurosurgery. 2018;129(3):779-791.  https://doi.org/10.3171/2017.3.JNS162479
  12. Heffez DS, Broderick J, Connor M, Mitchell M, Galezowska J, Golchini R, Ghorai J. Is there a relationship between the extent of tonsillar ectopia and the severity of the clinical Chiari syndrome? Acta Neurochirurgica. 2020;162(7):1531-1538. https://doi.org/10.1007/s00701-019-04171-1
  13. Sekula RF Jr, Jannetta PJ, Casey KF, Marchan EM, Sekula LK, McCrady CS. Dimensions of the posterior fossa in patients symptomatic for Chiari I malformation but without cerebellar tonsillar descent. Cerebrospinal Fluid Research. 2005;2:11.  https://doi.org/10.1186/1743-8454-2-11
  14. Ciaramitaro P, Ferraris M, Massaro F, Garbossa D. Clinical diagnosis-part I: what is really caused by Chiari I. Child’s Nervous System: ChNS. 2019;35(10):1673-1679. https://doi.org/10.1007/s00381-019-04206-z
  15. Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO. Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. Journal of Neurosurgery. Pediatrics. 2011;8(2):205-213.  https://doi.org/10.3171/2011.5.PEDS1121
  16. Oldfield EH. Pathogenesis of Chiari I — Pathophysiology of Syringomyelia: Implications for Therapy: A Summary of 3 Decades of Clinical Research. Neurosurgery. 2017;64(CN suppl 1):66-77.  https://doi.org/:10.1093/neuros/nyx377
  17. Tubbs RS, Elton S, Grabb P, Dockery SE, Bartolucci AA, Oakes WJ Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery. 2001;48(5):1050-1055. https://doi.org/10.1097/00006123-200105000-00016
  18. Chern JJ, Gordon AJ, Mortazavi MM, Tubbs RS, Oakes WJ Pediatric Chiari malformation Type 0: a 12-year institutional experience. Journal of Neurosurgery. Pediatrics. 2011;8(1):1-5.  https://doi.org/:10.3171/2011.4.PEDS10528
  19. Kyoshima K, Kuroyanagi T, Oya F, Kamijo Y, El-Noamany H, Kobayashi S. Syringomyelia without hindbrain herniation: tight cisterna magna. Report of four cases and a review of the literature. Journal of Neurosurgery. 2002;96 (2 Suppl):239-249.  https://doi.org/10.3171/spi.2002.96.2.0239
  20. Bogdanov EI, Heiss JD, Mendelevich EG, Mikhaylov IM, Haass A. Clinical and neuroimaging features of «idiopathic» syringomyelia. Neurology. 2004;62(5):791-794.  https://doi.org/10.1212/01.wnl.0000113746.47997.ce
  21. Ozsoy KM, Oktay K, Cetinalp NE, Gezercan Y, Erman T. The Role of Cine Flow Magnetic Resonance Imaging in Patients with Chiari 0 Malformation. Turkish Neurosurgery. 2018;28(2):251-256.  https://doi.org/10.5137/1019-5149.JTN.19049-16.2
  22. Petit-Lacour MC, Lasjaunias P, Iffenecker C, Benoudiba F, Hadj Rabia M, Hurth M, Doyon D. Visibility of the central canal on MRI. Neuroradiology. 2000;42(10):756-761.  https://doi.org/10.1007/s002340000373
  23. Bogdanov EI, Ibatullin MM, Mendelevich EG. Spontaneous drainage in syringomyelia: magnetic resonance imaging findings. Neuroradiology. 2000;42(9):676-678.  https://doi.org/10.1007/s002340000358
  24. Bogdanov EI, Mendelevich EG. Syrinx size and duration of symptoms predict the pace of progressive myelopathy: retrospective analysis of 103 unoperated cases with craniocervical junction malformations and syringomyelia. Clinical Neurology and Neurosurgery. 2002;104(2):90-97.  https://doi.org/10.1016/s0303-8467(01)00189-5
  25. Kyoshima K, Bogdanov EI. Spontaneous resolution of syringomyelia: report of two cases and review of the literature. Neurosurgery. 2003;53(3):762-769.  https://doi.org/10.1227/01.neu.0000079629.05048.a2
  26. Bogdanov EI, Heiss JD, Mendelevich EG. The post-syrinx syndrome: stable central myelopathy and collapsed or absent syrinx. Journal of Neurology. 2006;253(6):707-713.  https://doi.org/10.1007/s00415-006-0091-5
  27. Lobzin SV, Iurkina EA, Lobzina AS. Clinical, neurological and neuroimaging criteria for the diagnosis of Chiari malformation 0. Vestnik Rossijskoj voenno-meditsinskoj akademii. 2016;55(3):74-77. (In Russ.).
  28. Fischbein R, Saling JR, Marty P, Kropp D, Meeker J, Amerine J, Chyatte MR. Patient-reported Chiari malformation type I symptoms and diagnostic experiences: a report from the national Conquer Chiari Patient Registry database. Neurological Sciences. 2015;36(9):1617-1624. https://doi.org/10.1007/s10072-015-2219-9
  29. Smith BW, Strahle J, Bapuraj JR, Muraszko KM, Garton HJ, Maher CO. Distribution of cerebellar tonsil position: implications for understanding Chiari malformation. Journal of Neurosurgery. 2013;119(3):812-819.  https://doi.org/10.3171/2013.5.JNS121825
  30. Bogdanov EI, Faizutdinova AT, Mendelevich EG, Sozinov AS, Heiss JD. Epidemiology of Symptomatic Chiari Malformation in Tatarstan: Regional and Ethnic Differences in Prevalence. Neurosurgery. 2019;84(5):1090-1097. https://doi.org/10.1093/neuros/nyy175
  31. Taylor DG, Chatrath A, Mastorakos P, Paisan G, Chen CJ, Buell TJ, Jane JA. Cerebrospinal fluid area and syringogenesis in Chiari malformation type I. Journal of Neurosurgery. 2020;134(3):825-830.  https://doi.org/10.3171/2019.11.JNS191439
  32. Botelho RV, Heringer LC, Botelho PB, Lopes RA, Waisberg J. Posterior Fossa Dimensions of Chiari Malformation Patients Compared with Normal Subjects: Systematic Review and Meta-Analysis. World Neurosurgery. 2020;138:521-529.  https://doi.org/10.1016/j.wneu.2020.02.182
  33. Moncho D, Poca MA, Minoves T, Ferré A, Cañas V, Sahuquillo J. Are evoked potentials clinically useful in the study of patients with Chiari malformation Type 1? Journal of Neurosurgery. 2017;126(2):606-619.  https://doi.org/10.3171/2015.11.JNS151764
  34. Nishikawa M, Bolognese PA, Kula RW, Ikuno H, Ohata K. Pathogenesis and Classification of Chiari Malformation Type I Based on the Mechanism of Ptosis of the Brain Stem and Cerebellum: A Morphometric Study of the Posterior Cranial Fossa and Craniovertebral Junction. Journal of Neurological Surgery Part B: Skull Base. 2021;82(3):277-284.  https://doi.org/10.1055/s-0039-1691832
  35. Bogdanov EI, Faizutdinova AT, Heiss JD. Posterior Cranial Fossa and Cervical Spine Morphometric Abnormalities in Symptomatic Chiari Type 0 and Chiari Type 1 Malformation Patients with and without Syringomyelia. Acta Neurochirurgica. 2021;163(11):3051-3064. https://doi.org/10.1007/s00701-021-04941-w
  36. Nishikawa M, Bolognese PA, Kula RW, Ikuno H, Takami T, Ohata K. Surgical Management of Chiari Malformations: Preliminary Results of Surgery According to the Mechanisms of Ptosis of the Brain Stem and Cerebellum. Journal of Neurological Surgery Part B: Skull Base. 2021;82(2):264-272.  https://doi.org/10.1055/s-0039-1697977
  37. Heffez DS, Golchini R, Ghorai J, Cohen B. Operative findings and surgical outcomes in patients undergoing Chiari 1 malformation decompression: relationship to the extent of tonsillar ectopia. Acta Neurochirurgica. 2020;162(7):1539-1547. https://doi.org/10.1007/s00701-019-04172-0
  38. Thimineur M, Kitaj M, Kravitz E, Kalizewski T, Sood P. Functional abnormalities of the cervical cord and lower medulla and their effect on pain: observations in chronic pain patients with incidental mild Chiari I malformation and moderate to severe cervical cord compression. The Clinical Journal of Pain. 2002;18(3):171-179.  https://doi.org/10.1097/00002508-200205000-00006
  39. Thakar S, Kurudi Siddappa A, Aryan S, Mohan D, Sai Kiran NA, Hegde AS Does the mesodermal derangement in Chiari Type I malformation extend to the cervical spine? Evidence from an analytical morphometric study on cervical paraspinal muscles. Journal of Neurosurgery. Spine. 2017;27(4):421-427.  https://doi.org/10.3171/2016.12.SPINE16914
  40. Tubbs R, Turgut M, Oakes W, eds. The Chiari Malformations. Springer, Cham; 2020. https://doi.org/10.1007/978-3-030-44862-2
  41. Wilkinson DA, Johnson K, Garton HJ, Muraszko KM, Maher CO. Trends in surgical treatment of Chiari malformation Type I in the United States. Journal of Neurosurgery. Pediatrics. 2017;19(2):208-216.  https://doi.org/:10.3171/2016.8.PEDS16273
  42. Whitney N, Sun H, Pollock JM, Ross DA. The human foramen magnum — normal anatomy of the cisterna magna in adults. Neuroradiology. 2013;55(11):1333-1339. https://doi.org/10.1007/s00234-013-1269-z
  43. Boyles AL, Enterline DS, Hammock PH, Siegel DG, Slifer SH, Mehltretter L, Gilbert JR, Hu-Lince D, Stephan D, Batzdorf U, Benzel E, Ellenbogen R, Green BA, Kula R, Menezes A, Mueller D, Oro’ JJ, Iskandar BJ, George TM, Milhorat TH, Speer MC. Phenotypic definition of Chiari type I malformation coupled with high-density SNP genome screen shows significant evidence for linkage to regions on chromosomes 9 and 15. American Journal of Medical Genetics. Part A. 2006;140(24):2776-2785. https://doi.org/10.1002/ajmg.a.31546
  44. Mueller DM, Oro’ JJ. Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari malformation type I with or without syringomyelia. Journal of the American Academy of Nurse Practitioners. 2004;16(3):134-138.  https://doi.org/10.1111/j.1745-7599.2004.tb00384.x
  45. Kumar A, Agrawal M, Prakash S, Somorendra S, Singh PK, Garg A, Singh M, Sharma BS. Acute Foramen Magnum Syndrome Following Single Diagnostic Lumbar Puncture: Consequence of a Small Posterior Fossa? World Neurosurgery. 2016;91:677.e1-677.e6777. https://doi.org/10.1016/j.wneu.2016.04.111
  46. Neufeld EA, Menacho ST, Shah LM. Craniocervical Junction and Posterior Fossa Dimensions can Affect Need for Decompressive Craniectomy in Posterior Cranial Fossa Hemorrhage. World Neurosurgery. 2019;127:570-577.  https://doi.org/10.1016/j.wneu.2019.03.208
  47. Chen YY, Lirng JF, Fuh JL, Chang FC, Cheng HC, Wang SJ. Primary cough headache is associated with posterior fossa crowdedness: A morphometric MRI study. Cephalalgia. 2004;24(9):694-699.  https://doi.org/10.1111/j.1468-2982.2004.00739.x
  48. Bolognese PA, Brodbelt A, Bloom AB, Kula RW. Professional Profiles, Technical Preferences, Surgical Opinions, and Management of Clinical Scenarios from a Panel of 63 International Experts in the Field of Chiari I Malformation. World Neurosurgery. 2020;140:14-22.  https://doi.org/10.1016/j.wneu.2020.03.119
  49. Zuev AA, Kostenko GV. Treatment of syringomyelia in patients with arachnopathy at the level of craniovertebral junction. Burdenko’s Journal of Neurosurgery. 2021;85(1):10-18. (In Russ.). https://doi.org/10.17116/neiro20218501110

Email Confirmation

An email was sent to test@gmail.com with a confirmation link. Follow the link from the letter to complete the registration on the site.

Email Confirmation

We use cооkies to improve the performance of the site. By staying on our site, you agree to the terms of use of cооkies. To view our Privacy and Cookie Policy, please. click here.