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 (e.g. headaches, myelopathic symptoms) and
neuroimaging (e.g. 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=0.01). Based on sequential sequestration,
the neuroimaging grading system included only the presence (55% improvement) or
absence (74% improvement) of a syrinx ≥ 6 mm (p=0.049).
Integrating the clinical and neuroimaging indices, improvement rates for the CSI ranged
from 83% (grade 1) to 45% (grade 3) (p=0.002). 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 following CM-1 surgery. The CSI may aid
preoperative counseling and stratify patients in comparative effectiveness trials.