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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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Medknow Publications & Media Pvt Ltd
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4810919/ https://www.ncbi.nlm.nih.gov/pubmed/27076836 |
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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 |
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