ARRS 2022 Abstracts

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E2051. Chiari I Malformation in Pediatric Population With and Without Syrinx: What to Measure?
Authors
  1. Maria Dien Esquivel; Children's Hospital of Eastern Ontario; University of Ottawa
  2. Neetika Gupta ; Children's Hospital of Eastern Ontario; University of Ottawa
  3. Albert Tu; Children's Hospital of Eastern Ontario; University of Ottawa
  4. Vid Bijelic; Children's Hospital of Eastern Ontario
  5. Maria Gladkikh; University of Ottawa
  6. Christian O'Brien; University of Ottawa
  7. Nagwa Wilson; Children's Hospital of Eastern Ontario; University of Ottawa
Objective:
Multiple imaging parameters have been examined in previous studies to predict syrinx in patients with Chiari I malformation (CM1) including skull base angle, degree of tonsillar descent, tonsillar shape, and obex position; however, no definitive criteria have been proposed. The objective of this study was to compare clinical and radiological characteristics of patients with CM1 and syrinx versus those without syrinx and matched controls.

Materials and Methods:
This is a retrospective study of patients between 0–18 years old with CM1 with and without syrinx identified between January 1, 2007 and February 12, 2020 who were seen at a quaternary pediatric hospital. Participants were included only if they had a baseline MRI of the head and spine between January 1, 2007 and February 12, 2020 prior to surgical intervention if offered. Patients with syndromic features noted on imaging or clinical history were excluded. Forty age-matched controls were identified for comparison. Multiple imaging parameters and clinical symptoms were recorded.

Results:
A total cohort of 122 patients with Chiari I malformation were included in this study. Of the 122 patients, 94 (77%) had no syrinx and 28 (23%) had syrinx. Among the radiological variables evaluated, we found that the length of the midbrain (p < 0.001), the length of the supraoccipital (p = 0.030), the position of the obex (p = 0.004), the presence of scoliosis (p < 0.001), kinking of the medulla (p = 0.041), the prominence of the central canal (p = 0.032), and the retroversion angle of the dens (p = 0.006) were statistically significant when comparing patients with CM1 with and without syrinx. Among the clinical features, the presence of neck pain (p = 0.005) and sensation dysfunction (p < 0.001) were the only statistically significant findings associated with syrinx between the three groups.

Conclusion:
The pathophysiology of syrinx formation in children with CM1 is not well understood. In our study, we describe radiographic and clinical data that are unique to patients with CM1 and syrinx. These specific radiographic and clinical parameters are predictive of the presence of syrinx. In clinical practice, patients for whom a CM1 has been identified, the presence of these radiographic and clinical findings should prompt further investigation for a syrinx, or close monitoring for development of syrinx.