Skip to main content

Advertisement

SpringerLink
Log in

Pediatric Chiari I malformation: novel and traditional measurements associated with syrinx and surgery

  • Original Article
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

Multiple imaging parameters have been examined to estimate the presence of syrinx and the need for surgery in Chiari I patients (CM1); however, no consistent or definitive criteria have been proposed. The objective of this study was to review existing and identify novel radiological and clinical characteristics of CM1 patients that associate syrinx development and surgical intervention.

Methods

Patients with Chiari I malformation diagnosed on imaging between 0 and 18 years were retrospectively reviewed from January 1, 2007 to February 12, 2020. Participants were included if they had a baseline MRI of the head and spine prior to surgical intervention if required. Forty age-matched controls with cranial imaging were identified for comparison. Imaging parameters and clinical symptoms were recorded.

Results

A total of 122 CM1 patients were included in this study. Of the 122 patients, 28 (23%) had syrinx, and 27 (22%) had surgery. The following imaging parameters associated with syrinx and surgical intervention were identified: midbrain length (P < 0.001; P = 0.032), the obex position (P = 0.002; P < 0.001) and medullary kinking (P = 0.041; P < 0.001). Among the clinical features, the presence of overall pain (P = 0.017; P = 0.042), neck pain (P = 0.005; P = 0.027), and sensory dysfunction (P < 0.001) were found to be strongly associated with syrinx and surgery.

Conclusion

While further investigation is needed, these specific radiological and clinical parameters should be considered when evaluating CM1 patients and may be used to guide further management.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Alexander H, Tsering D, Myseros JS, Magge SN, Oluigbo C, Sanchez CE, Keating RF (2019) Management of Chiari I malformations: a paradigm in evolution. Child Nerv Syst. https://doi.org/10.1007/s00381-019-04265-2

    Article  Google Scholar 

  2. Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM (1986) Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol 7:795–799

    CAS  PubMed  PubMed Central  Google Scholar 

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

    Article  PubMed  Google Scholar 

  4. Chatrath A, Marino A, Taylor D, Elsarrag M, Soldozy S, Jane JA (2019) Chiari I malformation in children—the natural history. Child’s Nervous System. https://doi.org/10.1007/s00381-019-04310-0

    Article  PubMed  Google Scholar 

  5. McClugage SG, Oakes WJ (2019) The Chiari I malformation. J Neurosurg Pediatr. https://doi.org/10.3171/2019.5.PEDS18382

    Article  PubMed  Google Scholar 

  6. Pepper J, Elhabal A, Tsermoulas G, Flint G (2020) Symptom outcome after craniovertebral decompression for Chiari type 1 malformation without syringomyelia. Acta Neurochir. https://doi.org/10.1007/s00701-020-04631-z

    Article  PubMed  Google Scholar 

  7. Shoja MM, Tubbs RS, Oakes WJ (2013) Embryology and pathophysiology of the Chiari I and II malformations. In: The Chiari Malformations. Springer New York, New York, NY

  8. Steinbok P (2004) Clinical features of Chiari I malformations. Child Nerv Syst. https://doi.org/10.1007/s00381-003-0879-x

    Article  Google Scholar 

  9. Holly LT, Batzdorf U (2019) Chiari malformation and syringomyelia. J Neurosurg Spine. https://doi.org/10.3171/2019.7.SPINE181139

    Article  PubMed  Google Scholar 

  10. Attal N (2004) Effects of surgery on the sensory deficits of syringomyelia and predictors of outcome: a long term prospective study. J Neurol Neurosurg Psychiatry. https://doi.org/10.1136/jnnp.2003.026674

    Article  PubMed  PubMed Central  Google Scholar 

  11. Alford EN, Atchley TJ, Leon TJ, Laskay NMB, Arynchyna AA, Smith BP, Aban I, Johnston JM, Blount JP, Rozzelle CJ, Oakes WJ, Rocque BG (2021) Imaging characteristics associated with surgery in Chiari malformation type I. J Neurosurg Pediatr. https://doi.org/10.3171/2020.9.PEDS20347

  12. Atchley TJ, Alford EN, Rocque BG (2020) Systematic review and meta-analysis of imaging characteristics in Chiari I malformation: Does anything really matter? Child Nerv Syst. https://doi.org/10.1007/s00381-019-04398-4

    Article  Google Scholar 

  13. Gad KA, Yousem DM (2017) Syringohydromyelia in patients with Chiari i malformation: a retrospective analysis. Am J Neuroradiol 38:1833–1838. https://doi.org/10.3174/ajnr.A5290

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Haller G, Sadler B, Kuensting T, Lakshman N, Greenberg JK, Strahle JM, Park TS, Dobbs MB, Gurnett CA, Limbrick DD (2020) Obex position is associated with syringomyelia and use of posterior fossa decompression among patients with Chiari I malformation. J Neurosurg Pediatr. https://doi.org/10.3171/2020.2.PEDS19486

    Article  PubMed  PubMed Central  Google Scholar 

  15. Stovner LJ, Rinck P (1992) Syringomyelia in Chiari malformation. Neurosurgery. https://doi.org/10.1227/00006123-199211000-00013

    Article  PubMed  Google Scholar 

  16. R Core Team (2020) R: a language and environment for statistical computing. R Foundation for Statistical Computing, Vienna, Austria. https://www.R-project.org/

  17. Holm S (1979) A simple sequentially rejective multiple test procedure. Scand J Stat 6:65–70

    Google Scholar 

  18. Al-Habib A, al Abdulsalam H, Ahmed J, Albadr F, Alhothali W, Alzahrani A, Abojamea A, Altowim A, Ullah A, Alkubeyyer M (2020) Association between craniovertebral junction abnormalities and syringomyelia in patients with chiari malformation type-1. Neurosciences 25:308–315. https://doi.org/10.17712/nsj.2020.4.20200008

    Article  PubMed  PubMed Central  Google Scholar 

  19. Halvorson KG, Kellogg RT, Keachie KN, Grant GA, Muh CR, Waldau B (2016) Morphometric analysis of predictors of cervical syrinx formation in the setting of Chiari I malformation. Pediatr Neurosurg. https://doi.org/10.1159/000442991

    Article  PubMed  Google Scholar 

  20. Hiremath SB, Fitsiori A, Boto J, Torres C, Zakhari N, Dietemann JL, Meling TR, Vargas MI (2020) The perplexity surrounding chiari malformations - are we any wiser now? Am J Neuroradiol 41:1975–1981

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJL, Maher CO (2011) Chiari malformation type I and syrinx in children undergoing magnetic resonance imaging: Clinical article. J Neurosurg Pediatr 8:205–213. https://doi.org/10.3171/2011.5.PEDS1121

    Article  PubMed  Google Scholar 

  22. Wu YW, Chin CT, Chan KM, Barkovich AJ, Ferriero DM (1999) Pediatric Chiari I malformations: do clinical and radiologic features correlate? Neurology. https://doi.org/10.1212/WNL.53.6.1271

    Article  PubMed  Google Scholar 

  23. Yan H, Han X, Jin M, Liu Z, Xie D, Sha S, Qiu Y, Zhu Z (2016) Morphometric features of posterior cranial fossa are different between Chiari I malformation with and without syringomyelia. Eur Spine J 25:2202–2209. https://doi.org/10.1007/s00586-016-4410-y

    Article  PubMed  Google Scholar 

  24. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Wolpert C, Speer MC (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients

  25. Tubbs RS, Iskandar BJ, Bartolucci AA, Oakes WJ (2004) A critical analysis of the Chiari 1.5 malformation. J Neurosurg Pediatr 101. https://doi.org/10.3171/ped.2004.101.2.0179

  26. Tubbs RS, Wellons JC, Blount JP, Grabb PA, Oakes WJ (2003) Inclination of the odontoid process in the pediatric Chiari I malformation. J Neurosurg Spine. https://doi.org/10.3171/spi.2003.98.1.0043

    Article  Google Scholar 

  27. Tubbs RS, Lyerly MJ, Loukas M, Shoja MM, Oakes WJ (2007) The pediatric Chiari I malformation: a review. Child Nerv Syst. https://doi.org/10.1007/s00381-007-0428-0

    Article  Google Scholar 

  28. Sandoval-Garcia C, Iskandar BJ (2013) Research on the pathophysiology of Chiari I-related symptoms and syringomyelia, with emphasis on dynamic MRI techniques. In: The Chiari Malformations. Springer New York, New York, NY

  29. Koyanagi I, Houkin K (2010) Pathogenesis of syringomyelia associated with Chiari type 1 malformation: Review of evidences and proposal of a new hypothesis. Neurosurg Rev. https://doi.org/10.1007/s10143-010-0266-5

    Article  PubMed  Google Scholar 

  30. Bollo RJ, Riva-Cambrin J, Brockmeyer MM, Brockmeyer DL (2012) Complex Chiari malformations in children: an analysis of preoperative risk factors for occipitocervical fusion. J Neurosurg Pediatr. https://doi.org/10.3171/2012.3.PEDS11340

    Article  PubMed  Google Scholar 

  31. Strahle J, Smith BW, Martinez M, Bapuraj JR, Muraszko KM, Garton HJL, Maher CO (2015) The association between Chiari malformation type I, spinal syrinx, and scoliosis. J Neurosurg Pediatr 15:607–611. https://doi.org/10.3171/2014.11.PEDS14135

    Article  PubMed  Google Scholar 

  32. Moore HE, Moore KR (2014) Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation. Pediatr Radiol. https://doi.org/10.1007/s00247-014-3021-1

    Article  PubMed  Google Scholar 

  33. Aitken LA, Lindan CE, Sidney S, Gupta N, Barkovich AJ, Sorel M, Wu YW (2009) Chiari type I malformation in a pediatric population. Pediatr Neurol. https://doi.org/10.1016/j.pediatrneurol.2009.01.003

    Article  PubMed  PubMed Central  Google Scholar 

  34. Greenberg JK, Yarbrough CK, Radmanesh A, Godzik J, Yu M, Jeffe DB, Smyth MD, Park TS, Piccirillo JF, Limbrick DD (2015) The Chiari severity index. Neurosurgery. https://doi.org/10.1227/NEU.0000000000000608

    Article  PubMed  Google Scholar 

  35. Schijman E, Steinbok P (2004) International survey on the management of Chiari I malformation and syringomyelia. Child Nerv Syst. https://doi.org/10.1007/s00381-003-0882-2

    Article  Google Scholar 

  36. Papaker MG, Abdallah A, Cesme DH, Gönen G, Asiltürk M, Avyasov R, Sofuoğlu ÖE, Abdallah BG, Emel E (2020) Clinical and radiological evaluation of treated Chiari I adult patients: Retrospective study from two neurosurgical centers. Neurosurg Rev. https://doi.org/10.1007/s10143-020-01414-z

    Article  PubMed  Google Scholar 

  37. Holly LT, Batzdorf U (2002) Slitlike syrinx cavities: a persistent central canal. J Neurosurg Spine. https://doi.org/10.3171/spi.2002.97.2.0161

    Article  Google Scholar 

  38. Takamura Y, Kawasaki T, Takahashi A, Nunomura K, Tiba K, Hasunuma M, Itou T (2001) A craniocervical injury—induced syringomyelia caused by central canal dilation secondary to acquired tonsillar herniation. J Neurosurg Spine. https://doi.org/10.3171/spi.2001.95.1.0122

    Article  Google Scholar 

  39. Milhorat TH, Kotzen RM, Anzil AP (1994) Stenosis of central canal of spinal cord in man: Incidence and pathological findings in 232 autopsy cases. J Neurosurg. https://doi.org/10.3171/jns.1994.80.4.0716

    Article  PubMed  Google Scholar 

  40. Cai C, Oakes WJ (1997) Hindbrain herniation syndromes: the Chiari malformations (I and II)

  41. Cesmebasi A, Loukas M, Hogan E, Kralovic S, Tubbs RS, Cohen-Gadol AA (2015) The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat 28:184–194

    Article  PubMed  Google Scholar 

  42. Hunter JV, Youl BD, Moseley IF (1992) Neuro-radiology MRI demonstration of midbrain deformity in association with Chiari malformation

  43. el Gammal T, Mark E, Brooks B (1988) MR imaging of Chiari II malformation. Am J Roentgenol 150:163–170. https://doi.org/10.2214/ajr.150.1.163

    Article  Google Scholar 

  44. Olszewski AM, Proctor MR (2018) Headache, Chiari I malformation and foramen magnum decompression. Curr Opin Pediatr. https://doi.org/10.1097/MOP.0000000000000679

    Article  PubMed  Google Scholar 

  45. Saletti V, Esposito S, Frittoli M, Valentini LG, Chiapparini L, Bulgheroni S, Riva D (2011) Neurological pictures in paediatric Chiari I malformation. Neurol Sci. https://doi.org/10.1007/s10072-011-0744-8

    Article  PubMed  Google Scholar 

  46. Cahan LD, Bentson JR (1982) Considerations in the diagnosis and treatment of syringomyelia and the Chiari malformation. J Neurosurg. https://doi.org/10.3171/jns.1982.57.1.0024

    Article  PubMed  Google Scholar 

  47. Gamache FW Jr, Ducker TB (1990) Syringomyelia: a neurological and surgical spectrum. J Spinal Disord 1990 Dec;3(4):293-8. PMID: 2134442

Download references

Author information

Authors and Affiliations

  1. University of Ottawa, Ottawa, ON, Canada

    Maria F. Dien Esquivel, Neetika Gupta, Nagwa Wilson, Christian Alfred O’Brien, Maria Gladkikh & Albert Tu

  2. Department of Medical Imaging, Children’s Hospital of Eastern Ontario, Ottawa, ON, Canada

    Maria F. Dien Esquivel, Neetika Gupta & Nagwa Wilson

  3. Division of Neurosurgery, Children’s Hospital of Eastern Ontario, Ottawa, ON, Canada

    Albert Tu

  4. Research Institute, Children’s Hospital of Eastern Ontario, Ottawa, ON, Canada

    Nagwa Wilson, Nick Barrowman, Vid Bijelić & Albert Tu

  5. Northern Ontario School of Medicine, Thunder Bay, ON, Canada

    Christian Alfred O’Brien

Authors
  1. Maria F. Dien Esquivel
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Neetika Gupta
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Nagwa Wilson
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Christian Alfred O’Brien
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Maria Gladkikh
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Nick Barrowman
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Vid Bijelić
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Albert Tu
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Maria F. Dien Esquivel.

Ethics declarations

Ethics approval

The study was approved by CHEO REB#20/11X, file number 20200042.

Conflict of interest

The authors declare no competing interests.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Dien Esquivel, M.F., Gupta, N., Wilson, N. et al. Pediatric Chiari I malformation: novel and traditional measurements associated with syrinx and surgery. Childs Nerv Syst 38, 2119–2128 (2022). https://doi.org/10.1007/s00381-022-05644-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-022-05644-y

Keywords

Search

Navigation