Obex position is associated with syringomyelia and use of posterior fossa decompression among patients with Chiari I malformation

• Published in: Journal of neurosurgery. Pediatrics Vol. 26; no. 1; pp. 45 - 52
• Main Authors: Haller, Gabe, Sadler, Brooke, Kuensting, Timothy, Lakshman, Nivan, Greenberg, Jacob K, Strahle, Jennifer M, Park, Tae Sung, Dobbs, Matthew B, Gurnett, Christina A, Limbrick, David D
• Format: Journal Article
• Language: English
• Published: United States 01-07-2020
• Subjects: syringomyelia; scoliosis; Chiari I malformation; obex position; tonsil position; headaches; posterior fossa decompression surgery
• ISSN: 1933-0707; 1933-0715; 1933-0715
• DOI: 10.3171/2020.2.PEDS19486

Chiari I malformation (CM-I) has traditionally been defined by measuring the position of the cerebellar tonsils relative to the foramen magnum. The relationships of tonsillar position to clinical presentation, syringomyelia, scoliosis, and the use of posterior fossa decompression (PFD) surgery have been studied extensively and yielded inconsistent results. Obex position has been proposed as a useful adjunctive descriptor for CM-I and may be associated with clinical disease severity.
A retrospective chart review was performed of 442 CM-I patients with MRI who presented for clinical evaluation between 2003 and 2018. Clinical and radiological variables were measured for all patients, including presence/location of headaches, Chiari Severity Index (CSI) grade, tonsil position, obex position, clival canal angle, pB-C2 distance, occipitalization of the atlas, basilar invagination, syringomyelia, syrinx diameter, scoliosis, and use of PFD. Radiological measurements were then used to predict clinical characteristics using regression and survival analyses, with performing PFD, the presence of a syrinx, and scoliosis as outcome variables.
Among the radiological measurements, tonsil position, obex position, and syringomyelia were each independently associated with use of PFD. Together, obex position, tonsil position, and syringomyelia (area under the curve [AUC] 89%) or obex position and tonsil position (AUC 85.4%) were more strongly associated with use of PFD than tonsil position alone (AUC 76%) (Pdiff = 3.4 × 10-6 and 6 × 10-4, respectively) but were only slightly more associated than obex position alone (AUC 82%) (Pdiff = 0.01 and 0.18, respectively). Additionally, obex position was significantly associated with occipital headaches, CSI grade, syringomyelia, and scoliosis, independent of tonsil position. Tonsil position was associated with each of these traits when analyzed alone but did not remain significantly associated with use of PFD when included in multivariate analyses with obex position.
Compared with tonsil position alone, obex position is more strongly associated with symptomatic CM-I, as measured by presence of a syrinx, scoliosis, or use of PFD surgery. These results support the role of obex position as a useful radiological measurement to inform the evaluation and potentially the management of CM-I.