DANDY-WALKER MALFORMATION

Course overview

DANDY-WALKER MALFORMATION OR SYNDROME IS A RARE CONGENITAL NEUROLOGICAL ANOMALY THAT AFFECTS THE DEVELOPMENT OF THE CEREBELLUM, THE REGION OF THE BRAIN RESPONSIBLE FOR MOTOR COORDINATION AND BALANCE. THIS POSTERIOR FOSSA ANOMALY IS CHARACTERIZED BY AGENESIS OR HYPOPLASIA OF THE VERMIS AND CYSTIC ENLARGEMENT OF THE FOURTH VENTRICLE, CAUSING UPWARD DISPLACEMENT OF THE TENTORIUM AND TORCULA. MOST PATIENTS HAVE HYDROCEPHALUS AT THE TIME OF DIAGNOSIS. DANDY-WALKER MALFORMATION IS THE MOST COMMON POSTERIOR FOSSA MALFORMATION, AND IT TYPICALLY OCCURS SPORADICALLY. THE SYNDROME CAN MANIFEST WITH A WIDE SPECTRUM OF NEUROLOGICAL AND DEVELOPMENTAL SYMPTOMS, MAKING TIMELY RECOGNITION AND MANAGEMENT CRUCIAL FOR IMPROVING PATIENT OUTCOMES. TREATMENT CONSISTS OF TREATING THE MANIFESTATIONS AND ASSOCIATED COMORBIDITIES. THIS ACTIVITY REVIEWS THE PRESENTATION OF DANDY-WALKER MALFORMATION AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN ITS MANAGEMENT.