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Comparison of spinal deformity in children with Chiari I malformation with and without syringomyelia: matched cohort study

European Spine Journal volume 25, pages 619–626 (2016)Cite this article

Abstract

Purpose

To describe curve patterns in patients with Chiari malformation I (CIM) without syringomyelia, and compare to patients with Chiari malformation with syringomyelia (CIM + SM).

Methods

Review of medical records from 2000 to 2013 at a single institution was performed to identify CIM patients with scoliosis. Patients with CIM were matched (1:1) by age and gender to CIM + SM. Radiographic curve patterns, MRI-based craniovertebral junction parameters, and associated neurological signs were compared between the two cohorts.

Results

Eighteen patients with CIM-associated scoliosis in the absence of syringomyelia were identified; 14 (78 %) were female, with mean age of 11.5 ± 4.5 years. Mean tonsillar descent was 9.9 ± 4.1 mm in the CIM group and 9.1 ± 3.0 mm in the CIM + SM group (p = 0.57). Average syrinx diameter in the CIM + SM group was 9.0 ± 2.7 mm. CIM patients demonstrated less severe scoliotic curves (32.1° vs. 46.1°, p = 0.04), despite comparable thoracic kyphosis (43.7° vs. 49.6°, p = 0.85). Two (11 %) patients with CIM demonstrated thoracic apex left deformities compared to 9/18 (50 %) in the CIM + SM cohort (p = 0.01). Neurological abnormalities were only observed in the group with syringomyelia (6/18, or 33 %; p = 0.007).

Conclusion

In the largest series specifically evaluating CIM and scoliosis, we found that these patients appear to present with fewer atypical curve features, with less severe scoliotic curves, fewer apex left curves, and fewer related neurological abnormalities than CIM + SM. Notably, equivalent thoracic kyphosis was observed in both groups. Future studies are needed to better understand pathogenesis of spinal deformity in CIM with and without SM.

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Acknowledgments

We would like to acknowledge the generosity of Sam and Betsy Reeves and their support of the Park-Reeves Syringomyelia Research Consortium as well as Mateo Dalla Fontana and the O’Keefe family. We would also like to thank Mike Lehmkuhl for providing assistance with project coordination.

Conflict of interest

This project was supported by the Clinical and Translational Science Award (CTSA) program of the National Center for Advancing Translational Sciences (NCATS) of the National Institutes of Health (NIH) under Award Number TL1 TR000449. Additional funding was also provided by the NIH T35 NHLBI Training Grant, under Grant Number 5 T35 HL007815. Washington University, Department of Orthopaedic Surgery—Spine Service received grant monies from Axial Biotech, DePuy Synthes Spine, an NIH grant (2010-2015), and AOSpine, SRS and Norton Healthcare, Louisville, KY (Scoli-RISK-1 study), philanthropic research funding from the Fox Family Foundation (Prospective Pediatric Spinal Deformity study), fellowship funding from AOSpine North America (funds/fellow year). Dr. Lenke shares numerous patents with Medtronic (unpaid). He is a consultant for DePuy Synthes Spine, K2 M, Medtronic (monies donated to a charitable foundation). He receives substantial royalties from Medtronic and modest royalties from Quality Medical Publishing. Dr. Lenke also receives or has received reimbursement related to meetings/courses from AOSpine, BroadWater, DePuy Synthes Spine, K2 M, Medtronic, Scoliosis Research Society, Seattle Science Foundation, Stryker Spine, The Spinal Research Foundation. Dr. Limbrick has technology licensed to Allied Minds, Inc. for an unrelated project.

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Affiliations

  1. Department of Neurosurgery, Barrow Neurological Institute, 350 W. Thomas Road, Phoenix, AZ, 85013, USA

    J. Godzik

  2. Department of Neurosurgery, Washington University School of Medicine, St. Louis, MO, USA

    A. Dardas, T. F. Holekamp, M. D. Smyth, T. S. Park, J. R. Leonard & D. D. Limbrick

  3. Department of Orthopaedic Surgery, Washington University School of Medicine, St. Louis, MO, USA

    M. P. Kelly & L. G. Lenke

  4. Department of Pediatric Neurosurgery, St. Louis Children’s Hospital, St. Louis, MO, USA

    M. D. Smyth, T. S. Park, J. R. Leonard & D. D. Limbrick

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  1. J. Godzik
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  2. A. Dardas
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  3. M. P. Kelly
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  4. T. F. Holekamp
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  5. L. G. Lenke
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  6. M. D. Smyth
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  7. T. S. Park
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  8. J. R. Leonard
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  9. D. D. Limbrick
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Correspondence to J. Godzik.

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Godzik, J., Dardas, A., Kelly, M.P. et al. Comparison of spinal deformity in children with Chiari I malformation with and without syringomyelia: matched cohort study. Eur Spine J 25, 619–626 (2016). https://doi.org/10.1007/s00586-015-4011-1

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  • DOI: https://doi.org/10.1007/s00586-015-4011-1

Keywords

  • Chiari malformation
  • Syringomyelia
  • Scoliosis
  • Kyphosis
  • Spinal deformity