Radiological and clinical predictors of scoliosis in patients with Chiari malformation type I and spinal cord syrinx from the Park-Reeves Syringomyelia Research Consortium

Jennifer M. Strahle MD1, Rukayat Taiwo MD1, Christine Averill MD1, James Torner MS, PhD2, Chevis N. Shannon DrPH, MBA, MPH3, Christopher M. Bonfield MD3, Gerald F. Tuite MD4, Tammy Bethel-Anderson1, Jerrel Rutlin5, Douglas L. Brockmeyer MD6, John C. Wellons III MD, MSPH3, Jeffrey R. Leonard MD7, Francesco T. Mangano DO8, James M. Johnston MD9, Manish N. Shah MD10, Bermans J. Iskandar MD11, Elizabeth C. Tyler-Kabara MD, PhD12, David J. Daniels MD, PhD13, Eric M. Jackson MD14, Gerald A. Grant MD15, Daniel E. Couture MD16, P. David Adelson MD17, Tord D. Alden MD18, Philipp R. Aldana MD19, Richard C. E. Anderson MD20, Nathan R. Selden MD, PhD21, Lissa C. Baird MD21, Karin Bierbrauer MD8, Joshua J. Chern MD, PhD22, William E. Whitehead MD23, Richard G. Ellenbogen MD24, Herbert E. Fuchs MD, PhD25, Daniel J. Guillaume MD26, Todd C. Hankinson MD, MBA27, Mark R. Iantosca MD28, W. Jerry Oakes MD9, Robert F. Keating MD29, Nickalus R. Khan MD30, Michael S. Muhlbauer MD30, J. Gordon McComb MD31, Arnold H. Menezes MD32, John Ragheb MD33, Jodi L. Smith PhD, MD34, Cormac O. Maher MD35, Stephanie Greene MD12, Michael Kelly MD36, Brent R. O’Neill MD27, Mark D. Krieger MD31, Mandeep Tamber MD, PhD37, Susan R. Durham MD, MS38, Greg Olavarria MD39, Scellig S. D. Stone MD, PhD40, Bruce A. Kaufman MD41, Gregory G. Heuer MD, PhD42, David F. Bauer MD43, Gregory Albert MD, MPH44, Jeffrey P. Greenfield MD, PhD45, Scott D. Wait MD46, Mark D. Van Poppel MD46, Ramin Eskandari MD47, Timothy Mapstone MD48, Joshua S. Shimony MD, PhD5, Ralph G. Dacey Jr. MD1, Matthew D. Smyth MD1, Tae Sung Park MD1, and David D. Limbrick Jr. MD, PhD1
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  • 1 Department of Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri;
  • | 2 Department of Epidemiology, University of Iowa, Iowa City, Iowa;
  • | 3 Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, Tennessee;
  • | 4 Department of Neurosurgery, Neuroscience Institute, All Children’s Hospital, St. Petersburg, Florida;
  • | 5 Department of Radiology, Washington University School of Medicine, St. Louis, Missouri;
  • | 6 Department of Pediatric Neurosurgery, University of Utah School of Medicine, Salt Lake City, Utah;
  • | 7 Department of Neurological Surgery, The Ohio State University College of Medicine, Columbus, Ohio;
  • | 8 Division of Pediatric Neurosurgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio;
  • | 9 Division of Neurosurgery, University of Alabama School of Medicine, Birmingham, Alabama;
  • | 10 Department of Pediatric Surgery and Neurosurgery, The University of Texas McGovern Medical School, Houston, Texas;
  • | 11 Department of Neurological Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin;
  • | 12 Department of Neurosurgery, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania;
  • | 13 Department of Neurosurgery, The Mayo Clinic, Rochester, Minnesota;
  • | 14 Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, Maryland;
  • | 15 Department of Neurosurgery, Stanford Child Health Research Institute, Stanford, California;
  • | 16 Department of Neurosurgery, Wake Forest Baptist Medical Center, Winston-Salem, North Carolina;
  • | 17 Department of Neurosurgery, Barrow Neurological Institute, Phoenix, Arizona;
  • | 18 Department of Pediatric Neurosurgery, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois;
  • | 19 Department of Pediatric Neurosurgery, University of Florida College of Medicine, Jacksonville, Florida;
  • | 20 Department of Neurological Surgery, Columbia University College of Physicians and Surgeons, New York, New York;
  • | 21 Department of Neurological Surgery, Oregon Health & Science University, Portland, Oregon;
  • | 22 Department of Neurosurgery, Children’s Healthcare of Atlanta, Georgia;
  • | 23 Department of Neurosurgery, Baylor College of Medicine, Houston, Texas;
  • | 24 Department of Neurosurgery, University of Washington Medicine, Seattle, Washington;
  • | 25 Department of Neurosurgery, Duke University School of Medicine, Durham, North Carolina;
  • | 26 Department of Neurosurgery, University of Minnesota Medical School, Minneapolis, Minnesota;
  • | 27 Department of Neurosurgery, Children’s Hospital Colorado, Aurora, Colorado;
  • | 28 Department of Neurosurgery, Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania;
  • | 29 Department of Neurosurgery, Children’s National Medical Center, Washington, DC;
  • | 30 Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, Tennessee;
  • | 31 Division of Neurosurgery, Children’s Hospital Los Angeles, California;
  • | 32 Department of Neurosurgery, University of Iowa Hospitals, Iowa City, Iowa;
  • | 33 Department of Pediatric Neurosurgery, Miami Children’s Hospital and University of Miami Miller School of Medicine, Miami, Florida;
  • | 34 Department of Neurological Surgery, Indiana University School of Medicine, Indianapolis, Indiana;
  • | 35 Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan;
  • | 36 Department of Orthopedic Surgery, Washington University School of Medicine, St. Louis, Missouri;
  • | 37 Department of Neurosurgery, The University of British Columbia, Vancouver, British Columbia, Canada;
  • | 38 Department of Neurosurgery, University of Vermont College of Medicine, Burlington, Vermont;
  • | 39 Arnold Palmer Hospital for Children, Orlando, Florida;
  • | 40 Department of Neurosurgery, Boston Children’s Hospital, Boston, Massachusetts;
  • | 41 Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, Wisconsin;
  • | 42 Division of Neurosurgery, Children’s Hospital of Philadelphia, Pennsylvania;
  • | 43 Department of Neurosurgery, Dartmouth Geisel School of Medicine, Hanover, New Hampshire;
  • | 44 Department of Neurosurgery, University of Arkansas for Medical Sciences, Little Rock, Arkansas;
  • | 45 Department of Neurological Surgery, Weill Cornell Medical Center, New York, New York;
  • | 46 Department of Neurological Surgery, Levine Children’s Hospital, Charlotte, North Carolina;
  • | 47 Department of Neurosurgery, Medical University of South Carolina, Charleston, South Carolina; and
  • | 48 Department of Neurosurgery, Oklahoma University Medical Center, Oklahoma City, Oklahoma
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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.

ABBREVIATIONS

AIS = adolescent idiopathic scoliosis; CM-1 = Chiari malformation type I; CXA = clivoaxial angle; pB–C2 = measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2; PRSRC = Park-Reeves Syringomyelia Research Consortium.

Mathematical model representation of cerebrovascular physiology for calibration-free, patient-specific, noninvasive and continuous intracranial pressure estimation. Anatomical drawing © Sara Jarret, CMI. Published with permission. See the article by Fanelli et al. (pp 509–519).

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