Tonsillar herniation spectrum: more than just Chiari I. Update and controversies on classification and management

Cerebellar tonsil herniation comprises a spectrum of disorders sharing a common neuroimaging finding consisting of downward displacement of the cerebellar tonsils through the foramen magnum and into the upper cervical spinal canal. This not uncommon condition may result from a large host of congenital or acquired causes, and confusion regarding its classification and pathogenesis still exists. Terminology also remains heterogeneous, including inconsistencies in the usage of the “Chiari 1” monicker. In this paper, the hypothesized mechanisms of development of tonsillar herniation are reviewed and strategies of management are discussed, with particular attention to surgical options adapted to the underlying etiology. A focus will be placed on acquired causes of tonsillar herniation.