Skip to main content

Advertisement

SpringerLink
Log in

Long-term outcomes for children with an incidentally discovered Chiari malformation type 1: what is the clinical significance?

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

Abstract

Objectives

Chiari malformation type 1 (CM1) is an increasingly common incidental finding on magnetic resonance imaging (MRI). The proportion of children with an incidentally discovered CM1 who upon further evaluation require operative intervention for previously unrecognized signs and symptoms of neurological compromise or significant radiographic findings (syringomyelia) is unclear. An extensive long-term single-institution patient series was evaluated to better clarify the likelihood of surgery in patients who present with an incidentally discovered CM1.

Methods

This study was conducted using prospective data for patients up to 18 years old that were evaluated for a CM1 at a large tertiary pediatric neurosurgery clinic between February 2009 and June 2019. Patients were excluded if they did not have an incidentally discovered CM1 and at least 12 months of clinical follow-up.

Results

A total of 218 consecutive patients were included in this study. The mean age at the initial neurosurgical evaluation was 6.5 years (range 5 months to 18.4 years), and the mean duration of clinical follow-up was 40.6 months (range 12 to 114 months). Initial MR imaging was most commonly obtained for the evaluation of seizures (15.1%), nonspecific headaches (not occipital or tussive) (14.7%), trauma (9.6%), and developmental delay (7.8%). Of the patients studied that eventually required surgery, we identified two groups: those operated before 6 months since presentation and those operated after 6 months. A total of 36 patients (16.5%) underwent a decompression with 22 patients (61.1%) receiving surgery within 6 months and the remaining 14 patients (38.9%) beyond 6 months. Patients undergoing early surgery (10.1%) initially presented with a significant syrinx or were noted to have an occult neurological dysfunction, whereas a smaller subset of patients (6.4%) eventually required surgery over time due to the development of new symptoms or a de novo syrinx. Only the presence of syringomyelia was statistically significant for the need of a surgical intervention, while age, sex and degree of tonsillar herniation were not.

Conclusion

Evaluation of a large group of patients with an incidentally discovered Chiari malformation demonstrated that most patients may be managed conservatively, especially in the absence of syringomyelia. However, there is a subset of patients who will go on to develop a de novo syrinx or neurological symptoms that are new or progressive during follow-up, which should be evaluated by imaging of the brain and spinal cord. The presence of syringomyelia was associated with need for early surgical intervention. However, for patients without syringomyelia, surgical intervention is uncommon but may be delayed up to several years after presentation; therefore, long-term clinical follow-up is recommended.

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
Fig. 5

Similar content being viewed by others

References

  1. Aboulezz AO, Sartor K, Geyer CA, Gado MH (1985) Position of cerebellar tonsils in the normal population and in patients with Chiari malformation: a quantitative approach with MR imaging. J Comput Assist Tomogr 9:1033–1036. https://doi.org/10.1097/00004728-198511000-00005

    Article  CAS  PubMed  Google Scholar 

  2. 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 40:449–454. https://doi.org/10.1016/j.pediatrneurol.2009.01.003

    Article  PubMed  PubMed Central  Google Scholar 

  3. Alexander H, Tsering D, Myseros JS, Magge SN, Oluigbo C, Sanchez CE, Keating RF (2019) Management of Chiari I malformations: a paradigm in evolution. Childs Nerv Syst 35:1809–1826. https://doi.org/10.1007/s00381-019-04265-2

    Article  CAS  PubMed  Google Scholar 

  4. 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 15:161–177. https://doi.org/10.3171/2014.10.PEDS14295

    Article  PubMed  Google Scholar 

  5. 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  Google Scholar 

  6. Chatrath A, Marino A, Taylor D, Elsarrag M, Soldozy S, Jane JA Jr (2019) Chiari I malformation in children-the natural history. Childs Nerv Syst 35:1793–1799. https://doi.org/10.1007/s00381-019-04310-0

    Article  PubMed  Google Scholar 

  7. Ciaramitaro P, Ferraris M, Massaro F, Garbossa D (2019) Clinical diagnosis-part I: what is really caused by Chiari I. Childs Nerv Syst 35:1673–1679. https://doi.org/10.1007/s00381-019-04206-z

    Article  PubMed  Google Scholar 

  8. Elster AD, Chen MY (1992) Chiari I malformations: clinical and radiologic reappraisal. Radiology 183:347–353. https://doi.org/10.1148/radiology.183.2.1561334

    Article  CAS  PubMed  Google Scholar 

  9. Haroun RI, Guarnieri M, Meadow JJ, Kraut M, Carson BS (2000) Current opinions for the treatment of syringomyelia and Chiari malformations: survey of the pediatric section of the American Association of Neurological Surgeons. Pediatr Neurosurg 33:311–317. https://doi.org/10.1159/000055977

    Article  CAS  PubMed  Google Scholar 

  10. Headache Classification Committee of the International Headache Society (IHS) (2018) The International Classification of Headache Disorders, 3rd edition. Cephalalgia 38:1–211. https://doi.org/10.1177/0333102417738202

    Article  Google Scholar 

  11. Hersh DS, Groves ML, Boop FA (2019) Management of Chiari malformations: opinions from different centers-a review. Childs Nerv Syst 35:1869–1873. https://doi.org/10.1007/s00381-019-04176-2

    Article  PubMed  Google Scholar 

  12. Khalsa SSS, Geh N, Martin BA, Allen PA, Strahle J, Loth F, Habtzghi D, Urbizu Serrano A, McQuaide D, Garton HJL, Muraszko KM, Maher CO (2018) Morphometric and volumetric comparison of 102 children with symptomatic and asymptomatic Chiari malformation type I. J Neurosurg Pediatr 21:65–71. https://doi.org/10.3171/2017.8.PEDS17345

    Article  PubMed  Google Scholar 

  13. Leon TJ, Kuhn EN, Arynchyna AA, Smith BP, Tubbs RS, Johnston JM, Blount JP, Rozzelle CJ, Oakes WJ, Rocque BG (2019) Patients with "benign" Chiari I malformations require surgical decompression at a low rate. J Neurosurg Pediatr 23:498–506. https://doi.org/10.3171/2018.10.PEDS18407

    Article  PubMed  Google Scholar 

  14. Maher CO, Piatt JH Jr, Section on Neurologic Surgery AAoP (2015) Incidental findings on brain and spine imaging in children. Pediatrics 135:e1084–e1096. https://doi.org/10.1542/peds.2015-0071

    Article  PubMed  Google Scholar 

  15. Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS (2000) Asymptomatic Chiari type I malformations identified on magnetic resonance imaging. J Neurosurg 92:920–926. https://doi.org/10.3171/jns.2000.92.6.0920

    Article  CAS  PubMed  Google Scholar 

  16. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, Speer MC (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44:1005–1017. https://doi.org/10.1097/00006123-199905000-00042

    Article  CAS  PubMed  Google Scholar 

  17. Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, Tamburrini G, Di Rocco C (2008) The natural history of the Chiari type I anomaly. J Neurosurg Pediatr 2:179–187. https://doi.org/10.3171/PED/2008/2/9/179

    Article  PubMed  Google Scholar 

  18. Olszewski AM, Proctor MR (2018) Headache, Chiari I malformation and foramen magnum decompression. Curr Opin Pediatr 30:786–790. https://doi.org/10.1097/MOP.0000000000000679

    Article  PubMed  Google Scholar 

  19. Schijman E, Steinbok P (2004) International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst 20:341–348. https://doi.org/10.1007/s00381-003-0882-2

    Article  PubMed  Google Scholar 

  20. Shimoji K, Hara T, Ohara Y (2019) Controversies related to pediatric Chiari I malformation. Childs Nerv Syst 35:1695–1699. https://doi.org/10.1007/s00381-019-04243-8

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  22. Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO (2011) Natural history of Chiari malformation type I following decision for conservative treatment. J Neurosurg Pediatr 8:214–221. https://doi.org/10.3171/2011.5.PEDS1122

    Article  PubMed  Google Scholar 

  23. Vernooij MW, Ikram MA, Tanghe HL, Vincent AJ, Hofman A, Krestin GP, Niessen WJ, Breteler MM, van der Lugt A (2007) Incidental findings on brain MRI in the general population. N Engl J Med 357:1821–1828. https://doi.org/10.1056/NEJMoa070972

    Article  CAS  PubMed  Google Scholar 

  24. Wu YW, Chin CT, Chan KM, Barkovich AJ, Ferriero DM (1999) Pediatric Chiari I malformations: do clinical and radiologic features correlate? Neurology 53:1271–1276. https://doi.org/10.1212/wnl.53.6.1271

    Article  CAS  PubMed  Google Scholar 

Download references

Funding

The authors would like to acknowledge the support of the American Syringomyelia & Chiari Alliance Project (ASAP) for the grant support in the above clinical research at the Children’s National Hospital.

Author information

Authors and Affiliations

  1. Division of Neurosurgery, Children’s National Medical Center, 111 Michigan Ave NW, Washington, DC, 20010, USA

    Laurence Davidson, Tiffany N. Phan, John S. Myseros, Suresh N. Magge, Chima Oluigbo, Carlos E. Sanchez & Robert F. Keating

Authors
  1. Laurence Davidson
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Tiffany N. Phan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. John S. Myseros
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Suresh N. Magge
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Chima Oluigbo
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Carlos E. Sanchez
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Robert F. Keating
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Laurence Davidson.

Ethics declarations

Conflict of interest

The authors have no personal or institutional financial interest in drugs, materials, or devices described in this manuscript.

Additional information

Publisher’s note

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

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Davidson, L., Phan, T.N., Myseros, J.S. et al. Long-term outcomes for children with an incidentally discovered Chiari malformation type 1: what is the clinical significance?. Childs Nerv Syst 37, 1191–1197 (2021). https://doi.org/10.1007/s00381-020-04980-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-020-04980-1

Keywords

Search

Navigation