Objective: The authors' objective was to better understand the anatomical load-bearing relationship between the atlantooccipital joint and the upper cervical spine and its influence on the clinical behavior of patients with Chiari malformation type I (CM-I) and craniocervical pathology.
Methods: In a single-center prospective study of patients younger than 18 years with CM-I from 2015 through 2017 (mean age 9.91 years), the authors measured the occipital condyle-C2 sagittal vertebral alignment (C-C2SVA; defined as the position of a plumb line from the midpoint of the occiput (C0)-C1 joint relative to the posterior aspect of the C2-3 disc space), the pB-C2 (a line perpendicular to a line from the basion to the posteroinferior aspect of the C2 body on sagittal MRI), and the CXA (clivoaxial angle). Control data from 30 patients without CM-I (mean age 8.97 years) were used for comparison. The primary outcome was the need for anterior odontoid resection and/or occipitocervical fusion with or without odontoid reduction. The secondary outcome was the need for two or more Chiari-related operations.
Results: Of the 60 consecutive patients with CM-I identified, 7 underwent anterior odontoid resection or occipitocervical fusion and 10 underwent ≥ 2 decompressive procedures. The mean C-C2SVA was greater in the overall CM-I group versus controls (3.68 vs 0.13 mm, p < 0.0001), as was the pB-C2 (7.7 vs 6.4 mm, p = 0.0092); the CXA was smaller (136° vs 148°, p < 0.0001). A C-C2SVA ≥ 5 mm was found in 35% of CM-I children and 3.3% of controls (p = 0.0006). The sensitivities and specificities for requiring ventral decompression/occipitocervical fusion were 100% and 74%, respectively, for C-C2SVA ≥ 5 mm; 71% and 94%, respectively, for CXA < 125°; and 71% and 75%, respectively, for pB-C2 ≥ 9 mm. The sensitivities and specificities for the need for ≥ 2 decompressive procedures were 60% and 70%, respectively, for C-C2SVA ≥ 5 mm; 50% and 94%, respectively, for CXA < 125°; and 60% and 76%, respectively, for pB-C2 ≥ 9 mm. The log-rank test demonstrated significant differences between C-C2SVA groups (p = 0.0007) for the primary outcome. A kappa value of 0.73 for C-C2SVA between raters indicated substantial agreement.
Conclusions: A novel screening measurement for craniocervical bony relationships, the C-C2SVA, is described. A significant difference in C-C2SVA between CM-I patients and controls was found. A C-C2SVA ≥ 5 mm is highly predictive of the need for occipitocervical fusion/ventral decompression in patients with CM-I. Further validation of this screening measurement is needed.
Keywords: Chiari malformation; cervical spine; clivoaxial angle; occipitoatlantal joint; pB–C2; pediatric; sagittal vertical alignment.