Goldenhar syndrome: clinical features with orofacial emphasis
Autor(a) principal: | |
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Data de Publicação: | 2010 |
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=S1678-77572010000600019 |
Resumo: | OBJECTIVES: Goldenhar syndrome (GS) is a relatively common developmental disorder characterized by craniofacial anomalies in association with vertebral, cardiac, renal, and central nervous system defects. This paper describes GS features with special emphasis on oral characteristics. MATERIAL AND METHODS: The clinical features of 6 patients with GS aged 3 months to 12 years are described, and a brief review of the literature about this genetic disorder is presented. RESULTS: All patients demonstrated the classical triad of GS, including mandibular hypoplasia resulting in facial asymmetry, ear and/or eye malformation, and vertebral anomalies. In addition, renal and gastrointestinal abnormalities were observed in 2 patients. Regarding the oral involvement, 2 patients presented cleft lip and palate, and 1 patient had temporomandibular joint malformation. Malocclusion was found in all patients. CONCLUSION: Our orofacial findings correlate with the reported cases in the literature, and point out that after diagnosis GS patients need to be examined for systemic abnormalities. |
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Journal of applied oral science (Online) |
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Goldenhar syndrome: clinical features with orofacial emphasisGoldenhar syndromeMandibleFacial asymmetryOBJECTIVES: Goldenhar syndrome (GS) is a relatively common developmental disorder characterized by craniofacial anomalies in association with vertebral, cardiac, renal, and central nervous system defects. This paper describes GS features with special emphasis on oral characteristics. MATERIAL AND METHODS: The clinical features of 6 patients with GS aged 3 months to 12 years are described, and a brief review of the literature about this genetic disorder is presented. RESULTS: All patients demonstrated the classical triad of GS, including mandibular hypoplasia resulting in facial asymmetry, ear and/or eye malformation, and vertebral anomalies. In addition, renal and gastrointestinal abnormalities were observed in 2 patients. Regarding the oral involvement, 2 patients presented cleft lip and palate, and 1 patient had temporomandibular joint malformation. Malocclusion was found in all patients. CONCLUSION: Our orofacial findings correlate with the reported cases in the literature, and point out that after diagnosis GS patients need to be examined for systemic abnormalities.Faculdade De Odontologia De Bauru - USP2010-12-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1678-77572010000600019Journal of Applied Oral Science v.18 n.6 2010reponame:Journal of applied oral science (Online)instname:Universidade de São Paulo (USP)instacron:USP10.1590/S1678-77572010000600019info:eu-repo/semantics/openAccessMartelli-Júnior,HercílioMiranda,Roseli Teixeira deFernandes,Cassandro MoreiraBonan,Paulo Rogério FerretiParanaíba,Lívia Máris RibeiroGraner,EdgardColetta,Ricardo D.eng2011-01-14T00:00:00Zoai:scielo:S1678-77572010000600019Revistahttp://www.scielo.br/jaosPUBhttps://old.scielo.br/oai/scielo-oai.php||jaos@usp.br1678-77651678-7757opendoar:2011-01-14T00:00Journal of applied oral science (Online) - Universidade de São Paulo (USP)false |
dc.title.none.fl_str_mv |
Goldenhar syndrome: clinical features with orofacial emphasis |
title |
Goldenhar syndrome: clinical features with orofacial emphasis |
spellingShingle |
Goldenhar syndrome: clinical features with orofacial emphasis Martelli-Júnior,Hercílio Goldenhar syndrome Mandible Facial asymmetry |
title_short |
Goldenhar syndrome: clinical features with orofacial emphasis |
title_full |
Goldenhar syndrome: clinical features with orofacial emphasis |
title_fullStr |
Goldenhar syndrome: clinical features with orofacial emphasis |
title_full_unstemmed |
Goldenhar syndrome: clinical features with orofacial emphasis |
title_sort |
Goldenhar syndrome: clinical features with orofacial emphasis |
author |
Martelli-Júnior,Hercílio |
author_facet |
Martelli-Júnior,Hercílio Miranda,Roseli Teixeira de Fernandes,Cassandro Moreira Bonan,Paulo Rogério Ferreti Paranaíba,Lívia Máris Ribeiro Graner,Edgard Coletta,Ricardo D. |
author_role |
author |
author2 |
Miranda,Roseli Teixeira de Fernandes,Cassandro Moreira Bonan,Paulo Rogério Ferreti Paranaíba,Lívia Máris Ribeiro Graner,Edgard Coletta,Ricardo D. |
author2_role |
author author author author author author |
dc.contributor.author.fl_str_mv |
Martelli-Júnior,Hercílio Miranda,Roseli Teixeira de Fernandes,Cassandro Moreira Bonan,Paulo Rogério Ferreti Paranaíba,Lívia Máris Ribeiro Graner,Edgard Coletta,Ricardo D. |
dc.subject.por.fl_str_mv |
Goldenhar syndrome Mandible Facial asymmetry |
topic |
Goldenhar syndrome Mandible Facial asymmetry |
description |
OBJECTIVES: Goldenhar syndrome (GS) is a relatively common developmental disorder characterized by craniofacial anomalies in association with vertebral, cardiac, renal, and central nervous system defects. This paper describes GS features with special emphasis on oral characteristics. MATERIAL AND METHODS: The clinical features of 6 patients with GS aged 3 months to 12 years are described, and a brief review of the literature about this genetic disorder is presented. RESULTS: All patients demonstrated the classical triad of GS, including mandibular hypoplasia resulting in facial asymmetry, ear and/or eye malformation, and vertebral anomalies. In addition, renal and gastrointestinal abnormalities were observed in 2 patients. Regarding the oral involvement, 2 patients presented cleft lip and palate, and 1 patient had temporomandibular joint malformation. Malocclusion was found in all patients. CONCLUSION: Our orofacial findings correlate with the reported cases in the literature, and point out that after diagnosis GS patients need to be examined for systemic abnormalities. |
publishDate |
2010 |
dc.date.none.fl_str_mv |
2010-12-01 |
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=S1678-77572010000600019 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1678-77572010000600019 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/S1678-77572010000600019 |
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.18 n.6 2010 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_ |
1748936436196835328 |