title: The Chiari Severity Index: a preoperative grading system for Chiari malformation type 1.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Departments of *Neurological Surgery, ‡Otolaryngology, and §Medicine, ¶Mallincrodt Institute of Radiology, Washington University School of Medicine in St. Louis, St. Louis, Missouri.
authors.forename: Jacob K
authors.initials: JK
authors.lastname: Greenberg
authors.forename: Chester K
authors.initials: CK
authors.lastname: Yarbrough
authors.forename: Alireza
authors.initials: A
authors.lastname: Radmanesh
authors.forename: Jakub
authors.initials: J
authors.lastname: Godzik
authors.forename: Megan
authors.initials: M
authors.lastname: Yu
authors.forename: Donna B
authors.initials: DB
authors.lastname: Jeffe
authors.forename: Matthew D
authors.initials: MD
authors.lastname: Smyth
authors.forename: Tae Sung
authors.initials: TS
authors.lastname: Park
authors.forename: Jay F
authors.initials: JF
authors.lastname: Piccirillo
authors.forename: David D
authors.initials: DD
authors.lastname: Limbrick
mesh_terms: D000328:Adult; D000368:Aged; D001139:Arnold-Chiari Malformation / Q000145:classification* / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D059906:Neuroimaging; D019635:Neurosurgical Procedures; D011300:Preoperative Care; D011379:Prognosis; D011788:Quality of Life; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D016896:Treatment Outcome