In 2 cases of infants with the Chiari II malformation, a prepontine cyst presented in association with the hindbrain abnormalities characteristic of the Chiari II syndrome. Both infants presented with bulbar dysfunction and had poor outcome despite decompression of the cerebellar tonsillar herniation and drainage of the prepontine cysts. No such association between prepontine lesions and Chiari II has been described previously.

Keywords:
Arnold-Chiari malformation, Posterior fossa, Neuroepithelial cyst, Histiocytosis, Brainstem
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2000
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