Adult manifestation of the Dandy-Walker syndrome. Report of two cases with review of the literature.

Two cases of Dandy-Walker syndrome that became manifest in adult life are reported. A review of the literature of Dandy-Walker syndrome revealed 13 additional cases with onset in adult life and three cases that could not be differentiated from arachnoid cysts. While shunting is the primary treatment in infantile Dandy-Walker, the usual treatment of adult Dandy-Walker has been excision of the membrane covering the enlarged fourth ventricle. The outcome of our two cases treated with membrane excision indicates that primary shunting should also be tried in adult Dandy-Walker.