31 - Dental Rehabilitation of Pediatric Patient with Ellis-van Creveld Syndrome: Case Report

Ellis-van Creveld Syndrome, also known as Chondroectodermal dysplasia, is a rare autosomal recessive disorder caused by mutations in the EVC and EVC2 genes. This syndrome is characterized by polydactyly of the hands, short limbs, ectodermal dysplasia, and congenital heart defects. All three embryonic layers are affected in Ellis-van Creveld syndrome, thus there are several oral manifestations including the absence of the mucobuccal fold, a serrated alveolar ridge, multiple labial frenula, abnormalities in tooth morphology, and congenitally missing teeth. This report details a 4-year-old female who presented to the Children’s Mercy Dental Clinic in May 2019 for comprehensive dental care. The purpose of this report is to review dental treatment considerations for patients with Ellis-van Creveld Syndrome.