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Predictive Value and Interrater Reliability of Radiographic Factors in Neurofibromatosis Patients With Dystrophic Scoliosis

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Abstract

Background

Scoliosis in patients with neurofibromatosis type I (NF1) can manifest as dystrophic or nondystrophic curves. Dystrophic scoliosis is rapidly progressive, rendering treatment challenging. Radiographic characteristics have been reported to predict dystrophic scoliosis, but their reliability and predictive value have not been well described. The purpose of this study is to assess the interobserver reliability for eight radiographic characteristics of dystrophic scoliosis and to evaluate the sensitivity and specificity of these characteristics relative to the gold standard of a definitive clinical diagnosis.

Methods

Spine radiographs of 122 NF1 patients from multiple institutions were graded by five spine surgeons as dystrophic or nondystrophic, based on eight radiographic characteristics of dystrophic modulation: rib penciling, vertebral rotation, scalloping, wedging, spindling of transverse processes, short sharp angular curve, widened interpedicular distance, and atypical location. The curves were classified by each submitting institution as dystrophic or nondystrophic based on clinical outcome. Interobserver reliability analysis was performed using Fleiss kappa.

Results

For the 122 cases, the interrater agreement among the five readers for the diagnosis of dystrophic scoliosis was good at 0.61. The agreement for individual radiographic characteristic ranged from 0.62 for wedging to 0.14 (poor) for scalloping. Surgeons underestimated the number of dystrophic curves, rating from 45% to 67% of the curve patterns as dystrophic, compared to the gold standard, which revealed 68% of the curves to be dystrophic. On multivariate analysis, rib penciling, vertebral rotation, vertebral wedging, and atypical location were significantly associated with true dystrophic status (odds ratios of 2.4, 3.0, 2.4, and 3.0, respectively).

Conclusion

Overall dystrophic diagnosis can be assessed by radiographic characteristics. Better understanding of the predictive value of specific radiographic features may assist in early diagnosis of patients with dystrophic NF and assist surgeons in identifying dystrophic curve patterns and instituting prompt, appropriate treatment.

Level of Evidence

Level III.

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Authors and Affiliations

  1. Mayo Clinic, 200 First St. SW, Rochester, MN, 55905, USA

    A. Noelle Larson MD

  2. Department of Orthopedic Surgery, University of Minnesota, 2450 Riverside Ave., Suite R200, Minneapolis, MN, 55455, USA

    Charles Gerald T. Ledonio MD, Ann M. Brearley PhD, MS, Christopher L. Moertel MD & David W. Polly Jr. MD

  3. Texas Scottish Rite Hospital, 2222 Welborn St., Dallas, TX, 75219, USA

    Daniel J. Sucato MD, MS

  4. Norton Leatherman Spine Center, 210 E. Gray St., Suite 900, Louisville, KY, 40202, USA

    Leah Y. Carreon MD

  5. Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH, 45229, USA

    Alvin H. Crawford MD

  6. Division of Medical Genetics, University of Utah, 201 Presidents Cir., Salt Lake City, UT, 84112, USA

    David A. Stevenson MD

  7. Division of Medical Genetics, Stanford University, 450 Serra Mall, Stanford, CA, 94305, USA

    David A. Stevenson MD

  8. Department of Orthopedic Surgery, Columbia University Medical Center, 630 W 168th St., New York, NY, 10032, USA

    Michael G. Vitale MD

Authors
  1. A. Noelle Larson MD
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  2. Charles Gerald T. Ledonio MD
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  3. Ann M. Brearley PhD, MS
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  4. Daniel J. Sucato MD, MS
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  5. Leah Y. Carreon MD
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  6. Alvin H. Crawford MD
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  7. David A. Stevenson MD
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  8. Michael G. Vitale MD
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  9. Christopher L. Moertel MD
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  10. David W. Polly Jr. MD
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Corresponding author

Correspondence to David W. Polly Jr. MD.

Additional information

Author disclosures: ANL (grants from Department of Defense, during the conduct of the study; grants from K2M and Orthopediatrics, outside the submitted work), CGTL (personal fees from Greatbatch, Inc., outside the submitted work), AMB (grants from Dept of Defense NF Research Program, during the conduct of the study), DJS (other from Globus, outside the submitted work), LYC (other from Spine, Spine Journal, University of Louisville, and Scoliosis Research Society; personal fees from Washington University, AO Spine, Norton Healthcare; grants from Orthopedic Research and Educational Fund, Scoliosis Research Society, Norton Healthcare James R. Petersdorf; personal fees from University of Louisville, Association for Collaborative Spine Research, Center for Spine Surgery and Research, and Region of Southern Denmark; other from NuVasive, outside the submitted work), AHC (none), DAS (grants from the National Institutes of Health; grants from Shriners Hospital, during the conduct of the study; personal fees from Alexion and GLG, outside the submitted work), MGV (grants from Pediatric Orthopaedic Society of North America, during the conduct of the study; other from Pediatric Orthopaedic Society of North America and Biomet; personal fees from Stryker, Biomet, and Medtronic; other from Wellinks, outside the submitted work), CLM (Consultant, Recombinetics, Inc., Cancer Advisory Committee, including NF1-associated neoplasia; co–primary investigator, Synodos for NF1 Children’s Tumor Foundation, “An Innovative NF1 Drug Discovery Pipeline for Preclinical Development of Novel Drugs Quickly, Safely, and Effectively”), DWP (none).

Research reported in this publication was supported by 1) Department of Defense, Congressionally Directed Medical Research Program, Neurofibromatosis Research Program, Award Number W81HWH-10-1-0469; 2) the National Center for Advancing Translational Sciences of the National Institutes of Health, Award Number UL1TR000114; 3) Shriners Research Foundation; and 4) NIH NINDS K23 NS052500. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.

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Larson, A.N., Ledonio, C.G.T., Brearley, A.M. et al. Predictive Value and Interrater Reliability of Radiographic Factors in Neurofibromatosis Patients With Dystrophic Scoliosis. Spine Deform 6, 560–567 (2018). https://doi.org/10.1016/j.jspd.2018.02.011

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  • DOI: https://doi.org/10.1016/j.jspd.2018.02.011

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