TY - JOUR
T1 - Radiological and clinical predictors of scoliosis in patients with chiari malformation type i and spinal cord syrinx from the park-reeves syringomyelia research consortium
AU - Strahle, Jennifer M.
AU - Taiwo, Rukayat
AU - Averill, Christine
AU - Torner, James
AU - Shannon, Chevis N.
AU - Bonfield, Christopher M.
AU - Tuite, Gerald F.
AU - Bethel-Anderson, Tammy
AU - Rutlin, Jerrel
AU - Brockmeyer, Douglas L.
AU - Wellons, John C.
AU - Leonard, Jeffrey R.
AU - Mangano, Francesco T.
AU - Johnston, James M.
AU - Shah, Manish N.
AU - Iskandar, Bermans J.
AU - Tyler-Kabara, Elizabeth C.
AU - Daniels, David J.
AU - Jackson, Eric M.
AU - Grant, Gerald A.
AU - Couture, Daniel E.
AU - Adelson, P. David
AU - Alden, Tord D.
AU - Aldana, Philipp R.
AU - Anderson, Richard C.E.
AU - Selden, Nathan R.
AU - Baird, Lissa C.
AU - Bierbrauer, Karin
AU - Chern, Joshua J.
AU - Whitehead, William E.
AU - Ellenbogen, Richard G.
AU - Fuchs, Herbert E.
AU - Guillaume, Daniel J.
AU - Hankinson, Todd C.
AU - Iantosca, Mark R.
AU - Oakes, W. Jerry
AU - Keating, Robert F.
AU - Khan, Nickalus R.
AU - Muhlbauer, Michael S.
AU - McComb, J. Gordon
AU - Menezes, Arnold H.
AU - Ragheb, John
AU - Smith, Jodi L.
AU - Maher, Cormac O.
AU - Greene, Stephanie
AU - Kelly, Michael
AU - O'Neill, Brent R.
AU - Krieger, Mark D.
AU - Tamber, Mandeep
AU - Durham, Susan R.
AU - Olavarria, Greg
AU - Stone, Scellig S.D.
AU - Kaufman, Bruce A.
AU - Heuer, Gregory G.
AU - Bauer, David F.
AU - Albert, Gregory
AU - Greenfield, Jeffrey P.
AU - Wait, Scott D.
AU - Van Poppel, Mark D.
AU - Eskandari, Ramin
AU - Mapstone, Timothy
AU - Shimony, Joshua S.
AU - Dacey, Ralph G.
AU - Smyth, Matthew D.
AU - Park, Tae Sung
AU - Limbrick, David D.
N1 - Funding Information:
We would like to acknowledge the Reeves family and their friends and family for their generosity, which is responsible for the Park-Reeves Syringomyelia Research Consortium. Research reported in this publication was also supported by the Eunice Kennedy Shriver National Institute of Child Health & Human Development of the National Institutes of Health under award number U54 HD087011 to the Intellectual and Developmental Disabilities Research Center at Washington University. We would like to thank Michael Lehmkuhl for assistance in database management.
Publisher Copyright:
© AANS 2019.
PY - 2019
Y1 - 2019
N2 - 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.
AB - 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.
KW - Chiari
KW - Deformity
KW - Scoliosis
KW - Spine
KW - Syringomyelia
KW - Tonsil
UR - http://www.scopus.com/inward/record.url?scp=85074646365&partnerID=8YFLogxK
U2 - 10.3171/2019.5.PEDS18527
DO - 10.3171/2019.5.PEDS18527
M3 - Article
C2 - 31419800
AN - SCOPUS:85074646365
SN - 1933-0707
VL - 24
SP - 520
EP - 527
JO - Journal of Neurosurgery: Pediatrics
JF - Journal of Neurosurgery: Pediatrics
IS - 5
ER -