Dandy-Walker malformation

The Dandy-Walker malformation is the most frequent cerebral malformation. It is defined by hypoplasia and upward rotation of the vermis cerebelli, a cystic enlargement of the fourth ventricle and in total an enlarged posterior fossa with cranially shifted position of the lateral sinus, tentorium and torcula herophili. This malformation was first described by Dandy and Blackfan in 1914 then supplemented again by Taggart and Walker in 1942. The current description as Dandy-Walker malformation was introduced in 1954 by Bender. In addition to these classical findings, the Dandy-Walker malformation is characterized by other abnormalities and malformations of the central nervous system (CNS) including agenesis of the corpus callosum, heterotopia, occipital meningocele, visual deficits and epilepsy. Neurogenetic and imaging examinations have led to a better understanding of this malformation. (orig.)