External validation of current prediction systems of improvement after decompression surgery in Chiari malformation type I patients: can we do better?

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  • 1 Department of Neurosurgery, Johns Hopkins University School of Medicine;
  • 2 Krieger School of Arts and Sciences, Johns Hopkins University, Baltimore, Maryland; and
  • 3 Pratt School of Engineering, Duke University, Durham, North Carolina
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OBJECTIVE

The Chiari Severity Index (CSI) and points-based algorithm of Thakar et al. are two prognostic tools that have been developed to predict the likelihood of improvement after suboccipital decompression in adult patients with Chiari malformation type I (CM-I). This study aimed to externally validate and critically evaluate these algorithms in the interest of guiding the development of improved prediction systems.

METHODS

A consecutive cohort of CM-I patients undergoing suboccipital decompression between September 2006 and September 2018 were included. The CSI and Thakar point score were computed for all patients, and associations with improvement were analyzed. The ability of both prediction systems to predict improvement as measured by different Chicago Chiari Outcome Scale (CCOS) cutoffs was assessed using receiver operating curve analysis. Post hoc correlations between the algorithms and different CCOS subcomponents were also assessed.

RESULTS

The surgical cohort was composed of 149 adult CM-I patients, of whom 39 (26%) had a syrinx. Most patients experienced improvement after surgery (80% CCOS ≥ 13; 96% CCOS ≥ 11). The proportion of patients improving decreased with increasing CSI, but the results were not statistically significant (p = 0.246). No statistically significant difference in the mean Thakar point score was identified between improved and nonimproved patients using both CCOS cutoffs (p = 0.246 for a cutoff of 13 and p = 0.480 for a cutoff of 11). The CSI had a poor ability in identifying improved patients at a CCOS cutoff of 13 (area under the curve [AUC] 0.582) and 11 (AUC 0.646). The Thakar point score similarly had poor discrimination at a cutoff of 13 (AUC 0.467) and 11 (AUC 0.646). Neither algorithm had significant correlation with any of the CCOS subcomponents except for CSI and nonpain symptom improvement (coefficient = −0.273, p = 0.001).

CONCLUSIONS

Previously published algorithms failed to provide prediction value with regard to clinically meaningful improvement following suboccipital decompression in adult CM-I patients. Future models and practical scoring systems are still required to improve the decision-making process.

ABBREVIATIONS AUC = area under the curve; CCOS = Chicago Chiari Outcome Scale; CM-I = Chiari malformation type I; CSI = Chiari Severity Index; ROC = receiver operating characteristic.

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Contributor Notes

Correspondence Judy Huang: Johns Hopkins Hospital, Baltimore, MD. jhuang24@jhmi.edu.

INCLUDE WHEN CITING Published online May 1, 2020; DOI: 10.3171/2020.2.JNS20181.

Disclosures Dr. Huang: stock ownership interest in Longeviti Neuro Solutions.

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