A multicenter validation of the condylar–C2 sagittal vertical alignment in Chiari malformation type I: a study using the Park-Reeves Syringomyelia Research Consortium

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  • 1 Division of Pediatric Neurosurgery, Primary Children’s Hospital, University of Utah, Salt Lake City, Utah;
  • | 2 Division of Neurosurgery, University of California, San Diego, California;
  • | 3 Department of Neurosurgery, Naval Medical Center San Diego, California;
  • | 4 Department of Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri; and
  • | 5 Division of Pediatric Neurosurgery, Texas Children’s Hospital, Houston, Texas
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OBJECTIVE

The condylar–C2 sagittal vertical alignment (C-C2SVA) describes the relationship between the occipitoatlantal joint and C2 in patients with Chiari malformation type I (CM-I). It has been suggested that a C-C2SVA ≥ 5 mm is predictive of the need for occipitocervical fusion (OCF) or ventral brainstem decompression (VBD). The authors’ objective was to validate the predictive utility of the C-C2SVA by using a large, multicenter cohort of patients.

METHODS

This validation study used a cohort of patients derived from the Park-Reeves Syringomyelia Research Consortium; patients < 21 years old with CM-I and syringomyelia treated from June 2011 to May 2016 were identified. The primary outcome was the need for OCF and/or VBD. After patients who required OCF and/or VBD were identified, 10 age- and sex-matched controls served as comparisons for each OCF/VBD patient. The C-C2SVA (defined as the position of a plumb line from the midpoint of the O–C1 joint relative to the posterior aspect of the C2–3 disc space), pBC2 (a line perpendicular to a line from the basion to the posteroinferior aspect of the C2 body), and clival-axial angle (CXA) were measured on sagittal MRI. The secondary outcome was the need for ≥ 2 CM-related operations.

RESULTS

Of the 206 patients identified, 20 underwent OCF/VBD and 14 underwent repeat posterior fossa decompression. A C-C2SVA ≥ 5 mm was 100% sensitive and 86% specific for requiring OCF/VBD, with a 12.6% misclassification rate, whereas CXA < 125° was 55% sensitive and 99% specific, and pBC2 ≥ 9 was 20% sensitive and 88% specific. Kaplan-Meier analysis demonstrated that there was a significantly shorter time to second decompression in children with C-C2SVA ≥ 5 mm (p = 0.0039). The mean C-C2SVA was greater (6.13 ± 1.28 vs 3.13 ± 1.95 mm, p < 0.0001), CXA was lower (126° ± 15.4° vs 145° ± 10.7°, p < 0.05), and pBC2 was similar (7.65 ± 1.79 vs 7.02 ± 1.26 mm, p = 0.31) among those who underwent OCF/VBD versus decompression only. The intraclass correlation coefficient for the continuous measurement of C-C2SVA was 0.52; the kappa value was 0.47 for the binary categorization of C-C2SVA ≥ 5 mm.

CONCLUSIONS

These results validated the C-C2SVA using a large, multicenter, external cohort with 100% sensitivity, 86% specificity, and a 12.6% misclassification rate. A C-C2SVA ≥ 5 mm is highly predictive of the need for OCF/VBD in patients with CM-I. The authors recommend that this measurement be considered among the tools to identify the “high-risk” CM-I phenotype.

ABBREVIATIONS

AP = anteroposterior; C-C2SVA = condylar–C2 sagittal vertical alignment; CM-I = Chiari malformation type I; CXA = clival-axial angle; ICC = intraclass correlation coefficient; OCF = occipitocervical fusion; pBC2 = line perpendicular to a line from the basion to the posteroinferior aspect of the C2 body; PRSRC = Park-Reeves Syringomyelia Research Consortium; VBD = ventral brainstem decompression.

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Contributor Notes

Correspondence Douglas L. Brockmeyer: Primary Children’s Hospital, University of Utah, Salt Lake City, UT. neuropub@hsc.utah.edu.

INCLUDE WHEN CITING Published online June 4, 2021; DOI: 10.3171/2020.12.PEDS20809.

Disclosures Dr. Limbrick reports receiving support of non–study-related clinical or research effort from Medtronic Inc. and Microbot Medical Inc.

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