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  • Author or Editor: Gerald F. Tuite Jr x
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Todd C. Hankinson, Gerald F. Tuite, Dagmara I. Moscoso, Leslie C. Robinson, James C. Torner, David D. Limbrick Jr., Tae Sung Park and Richard C. E. Anderson

OBJECTIVE

The distance to the ventral dura, perpendicular to the basion to C2 line (pB-C2), is commonly employed as a measure describing the anatomy of the craniovertebral junction. However, both the reliability among observers and the clinical utility of this measurement in the context of Chiari malformation Type I (CM-I) have been incompletely determined.

METHODS

Data were reviewed from the first 600 patients enrolled in the Park-Reeves Syringomyelia Research Consortium with CM-I and syringomyelia. Thirty-one cases were identified in which both CT and MRI studies were available for review. Three pediatric neurosurgeons independently determined pB-C2 values using common imaging sequences: MRI (T1-weighted and T2-weighted with and without the inclusion of retro-odontoid soft tissue) and CT. Values were compared and intraclass correlations were calculated among imaging modalities and observers.

RESULTS

Intraclass correlation of pB-C2 demonstrated strong agreement between observers (intraclass correlation coefficient [ICC] range 0.72–0.76). Measurement using T2-weighted MRI with the inclusion of retro-odontoid soft tissue showed no significant difference with measurement using T1-weighted MRI. Measurements using CT or T2-weighted MRI without retro-odontoid soft tissue differed by 1.6 mm (4.69 and 3.09 mm, respectively, p < 0.05) and were significantly shorter than those using the other 2 sequences.

Conclusions pB-C2 can be measured reliably by multiple observers in the context of pediatric CM-I with syringomeyelia. Measurement using T2-weighted MRI excluding retro-odontoid soft tissue closely approximates the value obtained using CT, which may allow for the less frequent use of CT in this patient population. Measurement using T2-weighted MRI including retro-odontoid soft tissue or using T1-weighted MRI yields a more complete assessment of the extent of ventral brainstem compression, but its association with clinical outcomes requires further study.

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Jennifer M. Strahle, Rukayat Taiwo, Christine Averill, James Torner, Chevis N. Shannon, Christopher M. Bonfield, Gerald F. Tuite, Tammy Bethel-Anderson, Jerrel Rutlin, Douglas L. Brockmeyer, John C. Wellons III, Jeffrey R. Leonard, Francesco T. Mangano, James M. Johnston, Manish N. Shah, Bermans J. Iskandar, Elizabeth C. Tyler-Kabara, David J. Daniels, Eric M. Jackson, Gerald A. Grant, Daniel E. Couture, P. David Adelson, Tord D. Alden, Philipp R. Aldana, Richard C. E. Anderson, Nathan R. Selden, Lissa C. Baird, Karin Bierbrauer, Joshua J. Chern, William E. Whitehead, Richard G. Ellenbogen, Herbert E. Fuchs, Daniel J. Guillaume, Todd C. Hankinson, Mark R. Iantosca, W. Jerry Oakes, Robert F. Keating, Nickalus R. Khan, Michael S. Muhlbauer, J. Gordon McComb, Arnold H. Menezes, John Ragheb, Jodi L. Smith, Cormac O. Maher, Stephanie Greene, Michael Kelly, Brent R. O’Neill, Mark D. Krieger, Mandeep Tamber, Susan R. Durham, Greg Olavarria, Scellig S. D. Stone, Bruce A. Kaufman, Gregory G. Heuer, David F. Bauer, Gregory Albert, Jeffrey P. Greenfield, Scott D. Wait, Mark D. Van Poppel, Ramin Eskandari, Timothy Mapstone, Joshua S. Shimony, Ralph G. Dacey Jr., Matthew D. Smyth, Tae Sung Park and David D. Limbrick Jr.

OBJECTIVE

Scoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors’ goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis.

METHODS

A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°).

RESULTS

Based on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB–C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude.

CONCLUSIONS

Syrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.