Ellis–Van Creveld Syndrome With Unusal Expression Of Multiple Supernumerary Teeth : A Case Report

Ellis–van creveld syndrome is a rare autosomal recessive syndrome of the heart, skeleton and most of the ectodermal derivatives. It is caused due to genetic defects in the chromosome 4p16; it belongs to short rib-polydactyly group (SRP). It is characterized by short ribs, short limb, polydactyly, ectodermal dysplasia and congenital heart defects. The oral manifestation is one of the main characteristic diagnostic features and it includes partial anodontia, microdontia, fusion of upper lip to the gingival margin, multiple frenum, anterior open bite etc. We the dentist play a major role in diagnosing such patients, managing their dental problem and help them lead a normal life. We report a case of EVC syndrome expressing all the findings with multiple supernumerary teeth.