Ellis–van Creveld syndrome with unusual oral and dental findings:A rare clinical entity

Shaik Sameeulla, Jayam Raviraj, Suresh Dirasantchu, Suman S. Venkata


Ellis–van Creveld (EVC) syndrome, a form of skeletal and chondroectodermal dysplasia, is an

autosomal recessive disorder characterized by a tetrad of disproportionate dwarfism, postaxial

polydactyly, ectodermal dysplasia, and heart defects. In the present article, we hereby present a case

of a 13‑year‑old girl of Indian ethnicity with EVC syndrome with a remarkable number of classical

oral and dental features, with unusual findings such as taurodontism and talons cusp. Such dental

findings were reported in few cases only. Despite the fact that oral manifestations play an important

role in the diagnosis of EVC, only a few detailed reports have been published in the dental literature.

Key Words: Ellis–van Creveld syndrome, polydactyly, taurodontism

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