The Chiari Severity index: A preoperative grading system for Chiari malformation type 1

Jacob K. Greenberg, Chester K. Yarbrough, Alireza Radmanesh, Jakub Godzik, Megan Yu, Donna B. Jeffe, Matthew D. Smyth, Tae Sung Park, Jay F. Piccirillo, David D. Limbrick

Research output: Contribution to journalArticlepeer-review

46 Scopus citations


BACKGROUND: To develop evidence-based treatment guidelines for Chiari malformation type 1 (CM-1), preoperative prognostic indices capable of stratifying patients for comparative trials are needed. OBJECTIVE: To develop a preoperative Chiari Severity Index (CSI) integrating the clinical and neuroimaging features most predictive of long-term patient-defined improvement in quality of life (QOL) after CM-1 surgery. METHODS: We recorded preoperative clinical (eg, headaches, myelopathic symptoms) and neuroimaging (eg, syrinx size, tonsillar descent) characteristics. Brief follow-up surveys were administered to assess overall patient-defined improvement in QOL. We used sequential sequestration to develop clinical and neuroimaging grading systems and conjunctive consolidation to integrate these indices to form the CSI. We evaluated statistical significance using the Cochran-Armitage test and discrimination using the C statistic. RESULTS: Our sample included 158 patients. Sequential sequestration identified headache characteristics and myelopathic symptoms as the most impactful clinical parameters, producing a clinical grading system with improvement rates ranging from 81% (grade 1) to 58% (grade 3) (P =.01). Based on sequential sequestration, the neuroimaging grading system included only the presence (55% improvement) or absence (74% improvement) of a syrinx ≥6 mm (P =.049). Integrating the clinical and neuroimaging indices, improvement rates for the CSI ranged from 83% (grade 1) to 45% (grade 3) (P =.002). The combined CSI had moderately better discrimination (c = 0.66) than the clinical (c = 0.62) or neuroimaging (c = 0.58) systems alone. CONCLUSION: Integrating clinical and neuroimaging characteristics, the CSI is a novel tool that predicts patient-defined improvement after CM-1 surgery. The CSI may aid preoperative counseling and stratify patients in comparative effectiveness trials.

Original languageEnglish
Pages (from-to)279-285
Number of pages7
Issue number3
StatePublished - Jan 1 2015


  • Chiari malformation type 1
  • Comparative effectiveness research
  • Outcome assessment (health care)
  • Pediatric neurosurgery
  • Quality of life
  • Severity of illness index

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