Ellis – Van Crevald syndrome- A Rare Case

Ellis – Van Crevald syndrome- A rare case

Authors

  • Tejas Mukund Kulkarni
  • Rajendra S Birangane
  • Sanjiv Onkar
  • Abhay Kulkarni
  • Kamal Yadav
  • Swati Reddy

Keywords:

Bilateral polydactaly, Chondrodysplasia, Ellis Van Crevald syndrome (EVC), Orofacial manifestations

Abstract

Ellis van crevald syndrome or chondroectodermal dysplasia is known to be a rare genetic disorder first described by Richard W.B Ellis & Simon Van crevald in 1940. This syndrome comprises tetrad of chondrodysplasia, bilateral postaxial polydactyly of the hands, ectodermal dysplasia & congenital heart defects. The orofacial manifestation includes dental malformations, hypodontia, multiple gingivolabial musculofibrous frenulae, notching of the lower alveolar process, fusion of the upper lip gingival mucosal margin. This paper includes a case report of a young female patient having similar characteristic features and orofacial manifestations suggestive of this syndrome and its management.

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Published

2015-02-28

Issue

Section

Case Report