The pathogenesis of Chiari I malformation and syringomyelia

Objective: The pathogeneses of Chiari malformation type I and syringomyelia are incompletely understood. In this article, the authors attempt to review the current theories on the pathogeneses of Chiari I malformation and syringomyelia. Methods: A literature review for articles pertaining to Chiari I malformation or syringomyelia before August 2010 was conducted; in addition, the author's own experience in treating Chiari I malformation and syringomyelia is included. Results: Chiari I malformation has been defined radiographically as cerebellar tonsillar herniation or ectopia 5 mm or greater below the foramen magnum. By this narrow definition, Chiari I malformation (i.e. cerebellar tonsillar herniation or ectopia 5 mm or greater below the foramen magnum) likely encompasses a heterogeneous grouping of disorders caused by different mechanisms. Molecular and genetic studies have been helpful in furthering our understanding of Chiari I malformation. Conclusion: A review of the pathogeneses of Chiari I malformation and syringomyelia is reported.