Imaging characteristics associated with surgery in Chiari malformation type I

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  • 1 Division of Pediatric Neurosurgery, Department of Neurosurgery; and
  • 2 Departments of Internal Medicine,
  • 3 Surgery, and
  • 4 Statistics, University of Alabama at Birmingham, Alabama
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OBJECTIVE

In Chiari malformation type I (CM-I), a variety of imaging findings have been purported to be important; however, results have been inconclusive, inconsistent, or not replicated in independent studies. The purpose of this study was to report imaging characteristics for a large cohort of patients with CM-I and identify the imaging findings associated with surgical decompression.

METHODS

Patients were identified using ICD-9 codes for CM-I for the period from 1996 to 2017. After review of the medical records, patients were excluded if they 1) did not have a diagnosis of CM-I, 2) were not evaluated by a neurosurgeon, or 3) did not have available preoperative MRI. Retrospective chart review was performed to collect demographic and clinical data. Imaging parameters were measured according to the Chiari I Malformation Common Data Elements.

RESULTS

A total of 731 patients were included for analysis, having a mean follow-up duration of 25.5 months. The mean age at presentation was 8.5 years. The mean tonsil position was 11.4 mm below the foramen magnum, and 62.8% of patients had a pegged tonsil shape. Two hundred patients (27.4%) underwent surgery for life-dominating tussive headache, lower cranial nerve dysfunction, syrinx, and/or brainstem dysfunction. Surgical treatment was associated with a syrinx (OR 20.4, 95% CI 12.3–33.3, p < 0.0001), CM-1.5 (OR 1.797, 95% CI 1.08–2.98, p = 0.023), lower tonsil position (OR 1.130, 95% CI 1.08–1.18, p < 0.0001), and congenital fusion of cervical vertebrae (OR 5.473, 95% CI 1.08–27.8, p = 0.040). Among patients with benign CM-I, tonsil position was statistically significantly associated with future surgery.

CONCLUSIONS

Comprehensive imaging characteristics for a large cohort of patients with CM-I are reported. Analysis showed that a lower tonsillar position, a syrinx, and CM-1.5 were associated with undergoing posterior fossa decompression. This study demonstrates the importance of considering imaging findings in the context of patient symptomatology.

ABBREVIATIONS BAI = basion-axial interval; BDI = basion-dens interval; CDE = Common Data Element; CM-I = Chiari malformation type I; CXA = clivoaxial angle; NINDS = National Institute of Neurological Disorders and Stroke; pB-C2 = posterior basion to C2; PFD = posterior fossa decompression.

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Contributor Notes

Correspondence Brandon G. Rocque: University of Alabama at Birmingham, AL. brandon.rocque@childrensal.org.

INCLUDE WHEN CITING Published online April 23, 2021; DOI: 10.3171/2020.9.PEDS20347.

Disclosures The authors report no conflict of interest concerning the material or methods used in this study or the findings specified in this paper.

  • 1

    McClugage SG, Oakes WJ. The Chiari I malformation. J Neurosurg Pediatr. 2019;24(3):217226.

  • 2

    Tubbs RS, Lyerly MJ, Loukas M, et al. The pediatric Chiari I malformation: a review. Childs Nerv Syst. 2007;23(11):12391250.

  • 3

    Schijman E. History, anatomic forms, and pathogenesis of Chiari I malformations. Childs Nerv Syst. 2004;20(5):323328.

  • 4

    Ladner TR, Dewan MC, Day MA, et al. Evaluating the relationship of the pB-C2 line to clinical outcomes in a 15-year single-center cohort of pediatric Chiari I malformation. J Neurosurg Pediatr. 2015;15(2):178188.

    • Search Google Scholar
    • Export Citation
  • 5

    Ladner TR, Dewan MC, Day MA, et al. Posterior odontoid process angulation in pediatric Chiari I malformation: an MRI morphometric external validation study. J Neurosurg Pediatr. 2015;16(2):138145.

    • Search Google Scholar
    • Export Citation
  • 6

    Tubbs RS, Wellons JC III, Blount JP, et al. Inclination of the odontoid process in the pediatric Chiari I malformation. J Neurosurg. 2003;98(1)(suppl):4349.

    • Search Google Scholar
    • Export Citation
  • 7

    Khalsa SSS, Geh N, Martin BA, et al. Morphometric and volumetric comparison of 102 children with symptomatic and asymptomatic Chiari malformation Type I. J Neurosurg Pediatr. 2018;21(1):6571.

    • Search Google Scholar
    • Export Citation
  • 8

    Liu Z, Hao Z, Hu S, et al. Predictive value of posterior cranial fossa morphology in the decompression of Chiari malformation type I: a retrospective observational study. Medicine (Baltimore). 2019;98(19):e15533.

    • Search Google Scholar
    • Export Citation
  • 9

    Yan H, Han X, Jin M, et al. Morphometric features of posterior cranial fossa are different between Chiari I malformation with and without syringomyelia. Eur Spine J. 2016;25(7):22022209.

    • Search Google Scholar
    • Export Citation
  • 10

    Atchley TJ, Alford EN, Rocque BG. Systematic review and meta-analysis of imaging characteristics in Chiari I malformation: does anything really matter?. Childs Nerv Syst. 2020;36(3):525534.

    • Search Google Scholar
    • Export Citation
  • 11

    Leon TJ, Kuhn EN, Arynchyna AA, et al. Patients with “benign” Chiari I malformations require surgical decompression at a low rate. J Neurosurg Pediatr. 2019;23(4):498506.

    • Search Google Scholar
    • Export Citation
  • 12

    Harris JH Jr, Carson GC, Wagner LK, Kerr N. Radiologic diagnosis of traumatic occipitovertebral dissociation: 2. Comparison of three methods of detecting occipitovertebral relationships on lateral radiographs of supine subjects. AJR Am J Roentgenol. 1994;162(4):887892.

    • Search Google Scholar
    • Export Citation
  • 13

    Chiari I Malformation Standards - NINDS Common Data Elements. Accessed November 19, 2020. https://www.commondataelements.ninds.nih.gov/Chiari%20I%20Malformation

    • Search Google Scholar
    • Export Citation
  • 14

    Luciano MG, Batzdorf U, Kula RW, et al. Development of common data elements for use in Chiari malformation type I clinical research: an NIH/NINDS project. Neurosurgery. 2019;85(6):854860.

    • Search Google Scholar
    • Export Citation
  • 15

    Tubbs RS, Iskandar BJ, Bartolucci AA, Oakes WJ. A critical analysis of the Chiari 1.5 malformation. J Neurosurg. 2004;101(2)(suppl):179183.

    • Search Google Scholar
    • Export Citation
  • 16

    McRae DL, Barnum AS. Occipitalization of the atlas. Am J Roentgenol Radium Ther Nucl Med. 1953;70(1):2346.

  • 17

    Curtin F, Schulz P. Multiple correlations and Bonferroni's correction. Biol Psychiatry. 1998;44(8):775777.

  • 18

    Elster AD, Chen MY. Chiari I malformations: clinical and radiologic reappraisal. Radiology. 1992;183(2):347353.

  • 19

    Greenberg JK, Yarbrough CK, Radmanesh A, et al. The Chiari Severity Index: a preoperative grading system for Chiari malformation type 1. Neurosurgery. 2015;76(3):279285.

    • Search Google Scholar
    • Export Citation
  • 20

    Saez RJ, Onofrio BM, Yanagihara T. Experience with Arnold-Chiari malformation, 1960 to 1970. J Neurosurg. 1976;45(4):416422.

  • 21

    Strahle J, Muraszko KM, Kapurch J, et al. Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging. J Neurosurg Pediatr. 2011;8(2):205213.

    • Search Google Scholar
    • Export Citation
  • 22

    Furtado SV, Thakar S, Hegde AS. Correlation of functional outcome and natural history with clinicoradiological factors in surgically managed pediatric Chiari I malformation. Neurosurgery. 2011;68(2):319328.

    • Search Google Scholar
    • Export Citation
  • 23

    Haines SJ, Berger M. Current treatment of Chiari malformations types I and II: a survey of the Pediatric Section of the American Association of Neurological Surgeons. Neurosurgery. 1991;28(3):353357.

    • Search Google Scholar
    • Export Citation
  • 24

    Haroun RI, Guarnieri M, Meadow JJ, et al. Current opinions for the treatment of syringomyelia and Chiari malformations: survey of the Pediatric Section of the American Association of Neurological Surgeons. Pediatr Neurosurg. 2000;33(6):311317.

    • Search Google Scholar
    • Export Citation
  • 25

    Rocque BG, George TM, Kestle J, Iskandar BJ. Treatment practices for Chiari malformation type I with syringomyelia: results of a survey of the American Society of Pediatric Neurosurgeons. J Neurosurg Pediatr. 2011;8(5):430437.

    • Search Google Scholar
    • Export Citation
  • 26

    Schijman E, Steinbok P. International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst. 2004;20(5):341348.

    • Search Google Scholar
    • Export Citation
  • 27

    Genitori L, Peretta P, Nurisso C, et al. Chiari type I anomalies in children and adolescents: minimally invasive management in a series of 53 cases. Childs Nerv Syst. 2000;16(10-11):707718.

    • Search Google Scholar
    • Export Citation
  • 28

    Hofkes SK, Iskandar BJ, Turski PA, et al. Differentiation between symptomatic Chiari I malformation and asymptomatic tonsilar ectopia by using cerebrospinal fluid flow imaging: initial estimate of imaging accuracy. Radiology. 2007;245(2):532540.

    • Search Google Scholar
    • Export Citation
  • 29

    Krueger KD, Haughton VM, Hetzel S. Peak CSF velocities in patients with symptomatic and asymptomatic Chiari I malformation. AJNR Am J Neuroradiol. 2010;31(10):18371841.

    • Search Google Scholar
    • Export Citation
  • 30

    Meadows J, Kraut M, Guarnieri M, et al. Asymptomatic Chiari Type I malformations identified on magnetic resonance imaging. J Neurosurg. 2000;92(6):920926.

    • Search Google Scholar
    • Export Citation
  • 31

    Novegno F, Caldarelli M, Massa A, et al. The natural history of the Chiari Type I anomaly. J Neurosurg Pediatr. 2008;2(3):179187.

  • 32

    Strahle J, Muraszko KM, Kapurch J, et al. Natural history of Chiari malformation Type I following decision for conservative treatment. J Neurosurg Pediatr. 2011;8(2):214221.

    • Search Google Scholar
    • Export Citation
  • 33

    Wu YW, Chin CT, Chan KM, et al. Pediatric Chiari I malformations: do clinical and radiologic features correlate?. Neurology. 1999;53(6):12711276.

    • Search Google Scholar
    • Export Citation
  • 34

    Dupepe EB. Inter-rater reliability of the imaging parameters defined by the Common Data Elements for Chiari I malformation. Presented at: 47th Annual Meeting of the AANS/CNS Section on Pediatric Neurological Surgery; December 9, 2018; Nashville, TN.

    • Search Google Scholar
    • Export Citation
  • 35

    Trigylidas T, Baronia B, Vassilyadi M, Ventureyra ECG. Posterior fossa dimension and volume estimates in pediatric patients with Chiari I malformations. Childs Nerv Syst. 2008;24(3):329336.

    • Search Google Scholar
    • Export Citation
  • 36

    Yarbrough CK, Greenberg JK, Smyth MD, et al. External validation of the Chicago Chiari Outcome Scale. J Neurosurg Pediatr. 2014;13(6):679684.

    • Search Google Scholar
    • Export Citation
  • 37

    Arora P, Behari S, Banerji D, et al. Factors influencing the outcome in symptomatic Chiari I malformation. Neurol India. 2004;52(4):470474.

    • Search Google Scholar
    • Export Citation
  • 38

    Ladner TR, Westrick AC, Wellons JC III, Shannon CN. Health-related quality of life in pediatric Chiari Type I malformation: the Chiari Health Index for Pediatrics. J Neurosurg Pediatr. 2016;17(1):7685.

    • Search Google Scholar
    • Export Citation

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