Oral findings in patients with Apert Syndrome

Detalhes bibliográficos
Autor(a) principal: Dalben,Gisele da Silva
Data de Publicação: 2006
Outros Autores: Neves,Lucimara Teixeira das, Gomide,Marcia Ribeiro
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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spelling 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)
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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
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