Sudden unexpected death in patients with Dandy-Walker malformation.
An uncommon but well-recognized occurrence in patients with Dandy-Walker malformation is sudden unexpected death. The mechanism of demise has not been established. We report three patients with Dandy-Walker malformation that experienced sudden unexpected death without uncal or tonsillar herniation, the mechanism usually proposed for demise in such situations. Our findings suggest the possibility of vascular compromise as the cause of the sudden unexpected death in these patients. Early and effective relief of the pressure in the posterior fossa may prevent the occurrence of this catastrophic complication.
D001921:Brain / Q000473:pathology*; D001933:Brain Stem / Q000473:pathology; D002531:Cerebellum / Q000473:pathology; D002552:Cerebral Ventricles / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003616:Dandy-Walker Syndrome / Q000473:pathology*; D003645:Death, Sudden / Q000473:pathology*; D005260:Female; D006801:Humans; D007427:Intracranial Pressure / Q000502:physiology; D008297:Male