Experience in the management of Dandy-Walker syndrome in an adult patient

Abstract

Dandy-Walker Syndrome (DWS) is a congenital brain malformation involving the cerebellum and the fluid spaces around it. There is atresia of foramenofMagendie and Lushka resulting in a complete absence of the part of the brain located between the two cerebellar hemispheres (cerebellarvermis) and cystic dilatation of the fourth ventricle. It may be associated with other anomalies in the brain and other parts of the body. Symptoms such as delayed developmental milestone, bulging of skull may present early in life or may present later in life. We present a case of DWS diagnosed in adulthood with features of raised intracranial pressure (ICP) (headache, vomiting, neck stiffness, and convulsion) and poor academic performance.  Difficult airway was anticipated due to the presence of micrognathia and reduced neck mobility.The patient was managed successfully with cysto-periotoneal shunt insertion under general anaesthesiawithout postoperative ventilation.

Keywords: Dandy-Walker Syndrome, Hydrocephalus, VP-Shunt, Airway, Adulthood