Oral findings in patients with Apert Syndrome
Autor(a) principal: | |
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Data de Publicação: | 2006 |
Outros Autores: | , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Journal of applied oral science (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1678-77572006000600014 |
Resumo: | INTRODUCTION: The Apert syndrome is a rare disorder of autosomal dominant inheritance caused by mutations in the FGFR2 gene at locus 10q26; patients with this syndrome present severe syndactyly, exophthalmia, ocular hypertelorism and hypoplastic midface with Class III malocclusion, besides systemic alterations. Most investigations available on the Apert syndrome address the genetic aspect or surgical management, with little emphasis on the oral aspects. OBJECTIVE: to investigate the oral findings, including dental anomalies, ectopic eruption of the maxillary permanent first molars and soft tissue alterations, in subjects with Apert syndrome. MATERIALS AND METHODS: clinical and radiographic examination of nine patients with Apert syndrome, aged 6 to 15 years, not previously submitted to orthodontic or orthognathic treatment. RESULTS: dental anomalies were present in all patients, with one to eight anomalies per individual. The most frequent anomalies were tooth agenesis, mainly affecting maxillary canines, and enamel opacities (44.4% for both). Ectopic eruption of maxillary first molars was found in 33.3% of patients; lateral palatal swellings were observed in 88.8% of patients. CONCLUSIONS: The occurrence of typical lateral palatal swellings agrees with the literature. The high prevalence of dental anomalies and ectopic eruption may suggest a possible etiologic relationship with the syndrome. |
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Journal of applied oral science (Online) |
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Oral findings in patients with Apert SyndromeTooth abnormalitiesMouth abnormalitiesTooth eruptionectopicCraniosynostosesAcrocephalosyndactyliaINTRODUCTION: The Apert syndrome is a rare disorder of autosomal dominant inheritance caused by mutations in the FGFR2 gene at locus 10q26; patients with this syndrome present severe syndactyly, exophthalmia, ocular hypertelorism and hypoplastic midface with Class III malocclusion, besides systemic alterations. Most investigations available on the Apert syndrome address the genetic aspect or surgical management, with little emphasis on the oral aspects. OBJECTIVE: to investigate the oral findings, including dental anomalies, ectopic eruption of the maxillary permanent first molars and soft tissue alterations, in subjects with Apert syndrome. MATERIALS AND METHODS: clinical and radiographic examination of nine patients with Apert syndrome, aged 6 to 15 years, not previously submitted to orthodontic or orthognathic treatment. RESULTS: dental anomalies were present in all patients, with one to eight anomalies per individual. The most frequent anomalies were tooth agenesis, mainly affecting maxillary canines, and enamel opacities (44.4% for both). Ectopic eruption of maxillary first molars was found in 33.3% of patients; lateral palatal swellings were observed in 88.8% of patients. CONCLUSIONS: The occurrence of typical lateral palatal swellings agrees with the literature. The high prevalence of dental anomalies and ectopic eruption may suggest a possible etiologic relationship with the syndrome.Faculdade De Odontologia De Bauru - USP2006-12-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1678-77572006000600014Journal of Applied Oral Science v.14 n.6 2006reponame:Journal of applied oral science (Online)instname:Universidade de São Paulo (USP)instacron:USP10.1590/S1678-77572006000600014info:eu-repo/semantics/openAccessDalben,Gisele da SilvaNeves,Lucimara Teixeira dasGomide,Marcia Ribeiroeng2007-02-08T00:00:00Zoai:scielo:S1678-77572006000600014Revistahttp://www.scielo.br/jaosPUBhttps://old.scielo.br/oai/scielo-oai.php||jaos@usp.br1678-77651678-7757opendoar:2007-02-08T00:00Journal of applied oral science (Online) - Universidade de São Paulo (USP)false |
dc.title.none.fl_str_mv |
Oral findings in patients with Apert Syndrome |
title |
Oral findings in patients with Apert Syndrome |
spellingShingle |
Oral findings in patients with Apert Syndrome Dalben,Gisele da Silva Tooth abnormalities Mouth abnormalities Tooth eruption ectopic Craniosynostoses Acrocephalosyndactylia |
title_short |
Oral findings in patients with Apert Syndrome |
title_full |
Oral findings in patients with Apert Syndrome |
title_fullStr |
Oral findings in patients with Apert Syndrome |
title_full_unstemmed |
Oral findings in patients with Apert Syndrome |
title_sort |
Oral findings in patients with Apert Syndrome |
author |
Dalben,Gisele da Silva |
author_facet |
Dalben,Gisele da Silva Neves,Lucimara Teixeira das Gomide,Marcia Ribeiro |
author_role |
author |
author2 |
Neves,Lucimara Teixeira das Gomide,Marcia Ribeiro |
author2_role |
author author |
dc.contributor.author.fl_str_mv |
Dalben,Gisele da Silva Neves,Lucimara Teixeira das Gomide,Marcia Ribeiro |
dc.subject.por.fl_str_mv |
Tooth abnormalities Mouth abnormalities Tooth eruption ectopic Craniosynostoses Acrocephalosyndactylia |
topic |
Tooth abnormalities Mouth abnormalities Tooth eruption ectopic Craniosynostoses Acrocephalosyndactylia |
description |
INTRODUCTION: The Apert syndrome is a rare disorder of autosomal dominant inheritance caused by mutations in the FGFR2 gene at locus 10q26; patients with this syndrome present severe syndactyly, exophthalmia, ocular hypertelorism and hypoplastic midface with Class III malocclusion, besides systemic alterations. Most investigations available on the Apert syndrome address the genetic aspect or surgical management, with little emphasis on the oral aspects. OBJECTIVE: to investigate the oral findings, including dental anomalies, ectopic eruption of the maxillary permanent first molars and soft tissue alterations, in subjects with Apert syndrome. MATERIALS AND METHODS: clinical and radiographic examination of nine patients with Apert syndrome, aged 6 to 15 years, not previously submitted to orthodontic or orthognathic treatment. RESULTS: dental anomalies were present in all patients, with one to eight anomalies per individual. The most frequent anomalies were tooth agenesis, mainly affecting maxillary canines, and enamel opacities (44.4% for both). Ectopic eruption of maxillary first molars was found in 33.3% of patients; lateral palatal swellings were observed in 88.8% of patients. CONCLUSIONS: The occurrence of typical lateral palatal swellings agrees with the literature. The high prevalence of dental anomalies and ectopic eruption may suggest a possible etiologic relationship with the syndrome. |
publishDate |
2006 |
dc.date.none.fl_str_mv |
2006-12-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1678-77572006000600014 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1678-77572006000600014 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/S1678-77572006000600014 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Faculdade De Odontologia De Bauru - USP |
publisher.none.fl_str_mv |
Faculdade De Odontologia De Bauru - USP |
dc.source.none.fl_str_mv |
Journal of Applied Oral Science v.14 n.6 2006 reponame:Journal of applied oral science (Online) instname:Universidade de São Paulo (USP) instacron:USP |
instname_str |
Universidade de São Paulo (USP) |
instacron_str |
USP |
institution |
USP |
reponame_str |
Journal of applied oral science (Online) |
collection |
Journal of applied oral science (Online) |
repository.name.fl_str_mv |
Journal of applied oral science (Online) - Universidade de São Paulo (USP) |
repository.mail.fl_str_mv |
||jaos@usp.br |
_version_ |
1748936434367070208 |