16 - Dental Findings Associated With Dandy Walker and Orofacial Digital Syndrome

Dandy Walker Syndrome is a rare congenital brain malformation in which the cerebellum does not fully develop. The cerebellum is responsible for controlling movement and balance and is located at the back of the brain. Clinical symptoms associated with this syndrome include developmental delays, hydrocephalus, eye abnormalities and dental abnormalities including malformed or missing teeth. Orofacial Digital Syndrome is another rare genetic disorder characterized by a combination of abnormalities affecting the face, mouth, hands and feet. Clinical features of this syndrome include cleft lip, cleft palate, syndactyly, polydactyly, hypodontia and supernumerary teeth. This presentation discusses a 9-year-old female patient who was referred to Children’s Mercy Hospital due to her complex medical history. Her medical history is notable for Dandy Walker Syndrome, Orofacial Digital Syndrome Type 1, developmental delays and hydrocephalus. The patient does not have a specific chief complaint. Her mother is aware of her conditions and wishes to establish a dental home at a hospital equipped to provide necessary care. During a periodic exam, radiographic findings revealed the presence of supernumerary teeth and congenitally missing teeth. This report will detail the dental findings and treatment management for patients with Dandy Walker Syndrome and Orofacial Digital Syndrome.