Dandy Walker Syndrome – Always A Challenge

Dandy Walker Syndrome

Abstract

Dandy-Walker syndrome is classically described as a neuropathological triad consisting of hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and hydrocephalus. Clinical manifestations of the syndrome usually appear in the first year of life, but can occur during the neonatal period. Obstructive hydrocephalus with dilatation of the third and lateral ventricles occurs frequently. The syndrome was first described in 1914 by Dandy and Blackfan who postulated the pathogenetic theory of atresia of the fourth ventricles foramina of Luschka and Magendie; but today most authors consider the cerebellar hypoplasia to be a malformation independent of this atresia. DWS is also associated with abnormalities in the skeletal, cardiac, and genitourinary systems. In this report, we aimed to present a case of a patient with DWS who was scheduled for VP shunt surgery and was managed successfully without any perioperative complications.