OBJECTIVE: To establish the current Chiari knowledge base and develop recommendations for future research. METHODS: Chiari malformation: state of the research and new directions was a two-day, National Institutes of Health sponsored conference. The agenda included review presentations and working groups tasked with developing specific, achievable research recommendations. Content for both the review presentations and working groups was divided into six areas: pathophysiology, symptoms and diagnosis, engineering and imaging analysis, treatment, pediatric issues, and related conditions. RESULTS: The articles in this issue represent the knowledge base that was developed at the conference, plus subsequent literature reviews. There are significant gaps in the understanding of Chiari malformation, including a clinically relevant definition of Chiari, confusing terminology, subjective diagnostic criteria, and a well-accepted standard of care. These knowledge gaps are not surprising given the relative lack of Chiari research compared to similar neurological conditions and the preponderance of case reports in the published Chiari literature. The lack of understanding, and research, regarding Chiari translates directly into negative patient experiences and outcomes. CONCLUSIONS: Implementation of the recommendations developed at the conference would not only further expand the current understanding of the condition, but would likely have a significant, positive impact on patient experiences and outcomes.
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