Cargando…

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

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-ye...

Descripción completa

Detalles Bibliográficos
Autores principales: Shaik, Sameeulla, Raviraj, Jayam, Dirasantchu, Suresh, Venkata, Suman S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4810919/
https://www.ncbi.nlm.nih.gov/pubmed/27076836
_version_ 1782423859596623872
author Shaik, Sameeulla
Raviraj, Jayam
Dirasantchu, Suresh
Venkata, Suman S.
author_facet Shaik, Sameeulla
Raviraj, Jayam
Dirasantchu, Suresh
Venkata, Suman S.
author_sort Shaik, Sameeulla
collection PubMed
description 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.
format Online
Article
Text
id pubmed-4810919
institution National Center for Biotechnology Information
language English
publishDate 2016
publisher Medknow Publications & Media Pvt Ltd
record_format MEDLINE/PubMed
spelling pubmed-48109192016-04-13 Ellis–van Creveld syndrome with unusual oral and dental findings: A rare clinical entity Shaik, Sameeulla Raviraj, Jayam Dirasantchu, Suresh Venkata, Suman S. Dent Res J (Isfahan) Case Report 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. Medknow Publications & Media Pvt Ltd 2016 /pmc/articles/PMC4810919/ /pubmed/27076836 Text en Copyright: © Dental Research Journal http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution NonCommercial ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Case Report
Shaik, Sameeulla
Raviraj, Jayam
Dirasantchu, Suresh
Venkata, Suman S.
Ellis–van Creveld syndrome with unusual oral and dental findings: A rare clinical entity
title Ellis–van Creveld syndrome with unusual oral and dental findings: A rare clinical entity
title_full Ellis–van Creveld syndrome with unusual oral and dental findings: A rare clinical entity
title_fullStr Ellis–van Creveld syndrome with unusual oral and dental findings: A rare clinical entity
title_full_unstemmed Ellis–van Creveld syndrome with unusual oral and dental findings: A rare clinical entity
title_short Ellis–van Creveld syndrome with unusual oral and dental findings: A rare clinical entity
title_sort ellis–van creveld syndrome with unusual oral and dental findings: a rare clinical entity
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4810919/
https://www.ncbi.nlm.nih.gov/pubmed/27076836
work_keys_str_mv AT shaiksameeulla ellisvancreveldsyndromewithunusualoralanddentalfindingsarareclinicalentity
AT ravirajjayam ellisvancreveldsyndromewithunusualoralanddentalfindingsarareclinicalentity
AT dirasantchusuresh ellisvancreveldsyndromewithunusualoralanddentalfindingsarareclinicalentity
AT venkatasumans ellisvancreveldsyndromewithunusualoralanddentalfindingsarareclinicalentity