Medicina Interna: Acesso Aberto
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Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome): A Case Report with Dental Considerations and Review of Literature

L. Kayal, S. Jayachandran and Koijam Sashikumar Singh

Ellis-van Creveld syndrome is a rare autosomal recessive disorder. It is a chondroectodermal dysplasia characterized bychondrodysplasia, polydactyly, ectodermal dysplasia and congenital heart defects. Oral manifestations include fusion of the upper lip to the gingival margin, presence of multiple frenum, abnormally shaped and microdontic teeth, malocclusion, and congenitally missing teeth. Patients with EVC syndrome require multidisciplinary approach. Because of its unique oral and dental manifestations and its association with cardiac problems, dentist plays an important role in managing dental discrepancies and diagnosing possible serious health conditions beforehand.