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Natural history of Chiari I malformation with syrinx and dilatation of the central canal in the pediatric population: the CHEO experience

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Abstract

Purpose

Given that syrinx is often considered an indication of surgery in children with Chiari I malformation (CM1), understanding of the natural history of these patients is very challenging. In this study, we investigate the natural history of children with CM1 that have syrinx and/or prominence of the central canal on presentation.

Methods

All pediatric Chiari I patients who had syrinx and/or prominence of the central canal who underwent MR imaging of the head and spine from 2007 to 2020 were reviewed. Patients were divided into 3 groups (early surgery, delayed surgery, and conservative management). We focused on those patients who did not initially undergo surgery and had at least 1 year of clinical follow-up. We assessed if there were any radiological features that would correlate with need for delayed surgical intervention.

Results

Thirty-seven patients met the inclusion criteria. Twenty-one patients were female and 16 were male. The mean age at presentation was 8.7 (5.8 SD). Fourteen (38%) patients had early surgical intervention, with a mean of 2.5 months after initial presentation, 8 (16%) had delayed surgery due to new or progressive neurological symptoms and 46% of patients did not require intervention during follow-up. The length of tonsillar herniation and the position of the obex were associated with the need of surgery in patients who were initially treated conservatively.

Conclusion

In pediatric patients with CM1 with syringomyelia and prominence of the central canal, conservative treatment is initially appropriate when symptoms are absent or mild. Close follow-up of patients with CM1 and dilatation of the central canal who have an obex position below the foramen magnum and greater tonsillar herniation is suggested, as these patients show a trend towards clinical deterioration over time and may require earlier surgical intervention.

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Availability of data and materials

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

Abbreviations

CM1:

Chiari I malformation

PFD:

Posterior fossa decompression

MRI:

Magnetic resonance imaging

CHEO:

Children’s Hospital of Eastern Ontario

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Author information

Authors and Affiliations

  1. Department of Medical Imaging, CHEO, University of Ottawa, Ottawa, ON, Canada

    Maria Fernanda Dien Esquivel, Neetika Gupta & Nagwa Wilson

  2. University of Ottawa, Ottawa, ON, Canada

    Christian Alfred O’Brien

  3. CHEO Research Institute, CHEO, University of Ottawa, Ottawa, ON, Canada

    Vid Bijelić & Nick Barrowman

  4. Department of Neurosurgery, CHEO, University of Ottawa, Ottawa, ON, Canada

    Albert Tu

Authors
  1. Maria Fernanda Dien Esquivel
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  2. Neetika Gupta
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  3. Christian Alfred O’Brien
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  4. Vid Bijelić
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  5. Nick Barrowman
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  6. Nagwa Wilson
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  7. Albert Tu
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Contributions

M.D.E. collected imaging and clinical data and wrote the main manuscript text. N.G contributed in the imaging data collection. C.A.O. contributed in the clinical data collection. V.B. performed the statistical analysis. N.B. performed the statistical analysis. N.W. conceived and supervised the study and contributed in the imaging data collection. A.T. conceived and supervised the study and contributed in the clinical data collection. All authors reviewed the manuscript.

Corresponding author

Correspondence to Maria Fernanda Dien Esquivel.

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Ethics approval

The study was approved by CHEO REB#20/11X, File Number 20200042.

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The authors declare no competing interests.

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Esquivel, M.F.D., Gupta, N., O’Brien, C.A. et al. Natural history of Chiari I malformation with syrinx and dilatation of the central canal in the pediatric population: the CHEO experience. Childs Nerv Syst 40, 1443–1448 (2024). https://doi.org/10.1007/s00381-023-06273-9

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