OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME

Detalhes bibliográficos
Autor(a) principal: Sena, Marina Fernandes
Data de Publicação: 2019
Outros Autores: Mira, Paôla Caroline da Silva, Santos, Thais Tedeschi dos, Torres, Carolina Paes, Paula-Silva, Francisco Wanderley Garcia, Romano, Fábio Lourenço, Silveira, Sandra Maria Garcia da, Brito, Karina Mayra de Queiroz, Queiroz, Alexandra Mussolino de
Tipo de documento: Artigo
Idioma: por
Título da fonte: Revista Científica do CRO-RJ (Online)
Texto Completo: https://cro-rj.org.br/revcientifica/index.php/revista/article/view/114
Resumo: Introduction: Robinow syndrome is a rare genetic disorder of skeletal development. It is characterized by short stature, facial dysmorphisms and orodental anomalies, underdeveloped genitalia, mesomelic brachymelia. Case Report: A 6 year old girl with Robinow Syndrome was referred in the Center for Formation of Human Resources Specialized in Dental Care to Special Needs Patients (Ribeirão Preto, São Paulo, Brazil) for evaluation. Medical history investigation and clinical examination were observed in short stature, facial dimorphism and hypoplastic genitalia. Orofacial findings showed retrognathism, a mid-line hemangiomaapproximately 1 cm wide with upturned borders, bifid tongue, ankyloglossia, general gingival hypertrophy, deep bite and mild tooth crowding. Cardiac abnormality and neuromotor developmental delay consisted of systemic manifestations present which demanded individualized dental care. The dental treatment consisted of preventive and restoratives procedures to adequate the oral health condition of the patient and orthodontic treatmentwas planned. The patient has been in follow-up for six years. Conclusion: Orofacial anomalies and other alterations found in the present case contributed to complement the orofacialfindings described in the literature and to assist in diagnosis of the syndrome. In this case, amultiprofessional team and integral treatment were essential to rescue oral health and improvelife quality of the patient.
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spelling OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROMEOROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROMERobinow syndromedisabled childrendental careIntroduction: Robinow syndrome is a rare genetic disorder of skeletal development. It is characterized by short stature, facial dysmorphisms and orodental anomalies, underdeveloped genitalia, mesomelic brachymelia. Case Report: A 6 year old girl with Robinow Syndrome was referred in the Center for Formation of Human Resources Specialized in Dental Care to Special Needs Patients (Ribeirão Preto, São Paulo, Brazil) for evaluation. Medical history investigation and clinical examination were observed in short stature, facial dimorphism and hypoplastic genitalia. Orofacial findings showed retrognathism, a mid-line hemangiomaapproximately 1 cm wide with upturned borders, bifid tongue, ankyloglossia, general gingival hypertrophy, deep bite and mild tooth crowding. Cardiac abnormality and neuromotor developmental delay consisted of systemic manifestations present which demanded individualized dental care. The dental treatment consisted of preventive and restoratives procedures to adequate the oral health condition of the patient and orthodontic treatmentwas planned. The patient has been in follow-up for six years. Conclusion: Orofacial anomalies and other alterations found in the present case contributed to complement the orofacialfindings described in the literature and to assist in diagnosis of the syndrome. In this case, amultiprofessional team and integral treatment were essential to rescue oral health and improvelife quality of the patient.Introdução: A síndrome de Robinow é uma doença genética rara caracterizada por baixa estatura, dismorfismos faciais e anomalias orodentais, genitália subdesenvolvida e falhas no desenvolvimento esquelético. Relato de Caso: Uma paciente de 6 anos com Síndrome de Robinow foi encaminhada ao Centro de Formação de Recursos Humanos Especializado no Atendimento Odontológico de Pacientes com Necessidades Especiais (Ribeirão Preto, São Paulo, Brasil). Na investigação da história médica e ao exame clínico foram observados baixa estatura, dismorfismo facial e genitália hipoplásica. Os achados orofaciais mostraram retrognatismo, hemangioma de linha média de aproximadamente 1 cm de largura com bordas voltadas para cima, língua bífida, anquiloglossia, hipertrofia gengival geral, mordida profunda e apinhamento dentário leve. A anormalidade cardíaca e o atraso no desenvolvimento neuromotor demandaram cuidados individualizados. O tratamento odontológico consistiu em procedimentos preventivos e restauradores para adequar a condição de saúde bucal da paciente. Além disso, foi realizada avaliação ortodôntica e planejamento do tratamento. A paciente está em acompanhamento há seis anos. Conclusão: Anomalias orofaciais e outras alterações encontradas no presente caso contribuíram para complementar os achados orofaciais na literatura e auxiliar no diagnóstico da síndrome. Neste caso, um plano de tratamento integral e equipe multiprofissional foram essenciais para resgatar a saúde bucal e propiciar melhor qualidade de vida à paciente. Rio de Janeiro Dental Journal2019-10-12info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://cro-rj.org.br/revcientifica/index.php/revista/article/view/11410.29327/24816.4.2-11Revista Científica do CRO-RJ (Rio de Janeiro Dental Journal); Vol. 4 No. 2: May-August 2019; 60-66Revista Científica do CRO-RJ (Rio de Janeiro Dental Journal); v. 4 n. 2: May-August 2019; 60-662595-47331518-524910.29327/24816.4.2reponame:Revista Científica do CRO-RJ (Online)instname:Conselho Regional de Odontologia do Rio de Janeiro (CRO-RJ)instacron:CROporhttps://cro-rj.org.br/revcientifica/index.php/revista/article/view/114/75Copyright (c) 2019 Revista Científica do CRO-RJ (Rio de Janeiro Dental Journal)info:eu-repo/semantics/openAccessSena, Marina FernandesMira, Paôla Caroline da SilvaSantos, Thais Tedeschi dosTorres, Carolina PaesPaula-Silva, Francisco Wanderley GarciaRomano, Fábio LourençoSilveira, Sandra Maria Garcia daBrito, Karina Mayra de QueirozQueiroz, Alexandra Mussolino de2019-12-09T19:03:17Zoai:ojs3.cro-rj.org.br:article/114Revistahttps://cro-rj.org.br/revcientifica/index.php/revistahttps://cro-rj.org.br/revcientifica/index.php/revista/oairevista.cientifica@cro-rj.org.br || rorefa@terra.com.br2595-47331518-5249opendoar:2019-12-09T19:03:17Revista Científica do CRO-RJ (Online) - Conselho Regional de Odontologia do Rio de Janeiro (CRO-RJ)false
dc.title.none.fl_str_mv OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME
OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME
title OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME
spellingShingle OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME
Sena, Marina Fernandes
Robinow syndrome
disabled children
dental care
title_short OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME
title_full OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME
title_fullStr OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME
title_full_unstemmed OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME
title_sort OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME
author Sena, Marina Fernandes
author_facet Sena, Marina Fernandes
Mira, Paôla Caroline da Silva
Santos, Thais Tedeschi dos
Torres, Carolina Paes
Paula-Silva, Francisco Wanderley Garcia
Romano, Fábio Lourenço
Silveira, Sandra Maria Garcia da
Brito, Karina Mayra de Queiroz
Queiroz, Alexandra Mussolino de
author_role author
author2 Mira, Paôla Caroline da Silva
Santos, Thais Tedeschi dos
Torres, Carolina Paes
Paula-Silva, Francisco Wanderley Garcia
Romano, Fábio Lourenço
Silveira, Sandra Maria Garcia da
Brito, Karina Mayra de Queiroz
Queiroz, Alexandra Mussolino de
author2_role author
author
author
author
author
author
author
author
dc.contributor.author.fl_str_mv Sena, Marina Fernandes
Mira, Paôla Caroline da Silva
Santos, Thais Tedeschi dos
Torres, Carolina Paes
Paula-Silva, Francisco Wanderley Garcia
Romano, Fábio Lourenço
Silveira, Sandra Maria Garcia da
Brito, Karina Mayra de Queiroz
Queiroz, Alexandra Mussolino de
dc.subject.por.fl_str_mv Robinow syndrome
disabled children
dental care
topic Robinow syndrome
disabled children
dental care
description Introduction: Robinow syndrome is a rare genetic disorder of skeletal development. It is characterized by short stature, facial dysmorphisms and orodental anomalies, underdeveloped genitalia, mesomelic brachymelia. Case Report: A 6 year old girl with Robinow Syndrome was referred in the Center for Formation of Human Resources Specialized in Dental Care to Special Needs Patients (Ribeirão Preto, São Paulo, Brazil) for evaluation. Medical history investigation and clinical examination were observed in short stature, facial dimorphism and hypoplastic genitalia. Orofacial findings showed retrognathism, a mid-line hemangiomaapproximately 1 cm wide with upturned borders, bifid tongue, ankyloglossia, general gingival hypertrophy, deep bite and mild tooth crowding. Cardiac abnormality and neuromotor developmental delay consisted of systemic manifestations present which demanded individualized dental care. The dental treatment consisted of preventive and restoratives procedures to adequate the oral health condition of the patient and orthodontic treatmentwas planned. The patient has been in follow-up for six years. Conclusion: Orofacial anomalies and other alterations found in the present case contributed to complement the orofacialfindings described in the literature and to assist in diagnosis of the syndrome. In this case, amultiprofessional team and integral treatment were essential to rescue oral health and improvelife quality of the patient.
publishDate 2019
dc.date.none.fl_str_mv 2019-10-12
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
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dc.identifier.uri.fl_str_mv https://cro-rj.org.br/revcientifica/index.php/revista/article/view/114
10.29327/24816.4.2-11
url https://cro-rj.org.br/revcientifica/index.php/revista/article/view/114
identifier_str_mv 10.29327/24816.4.2-11
dc.language.iso.fl_str_mv por
language por
dc.relation.none.fl_str_mv https://cro-rj.org.br/revcientifica/index.php/revista/article/view/114/75
dc.rights.driver.fl_str_mv Copyright (c) 2019 Revista Científica do CRO-RJ (Rio de Janeiro Dental Journal)
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Copyright (c) 2019 Revista Científica do CRO-RJ (Rio de Janeiro Dental Journal)
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Rio de Janeiro Dental Journal
publisher.none.fl_str_mv Rio de Janeiro Dental Journal
dc.source.none.fl_str_mv Revista Científica do CRO-RJ (Rio de Janeiro Dental Journal); Vol. 4 No. 2: May-August 2019; 60-66
Revista Científica do CRO-RJ (Rio de Janeiro Dental Journal); v. 4 n. 2: May-August 2019; 60-66
2595-4733
1518-5249
10.29327/24816.4.2
reponame:Revista Científica do CRO-RJ (Online)
instname:Conselho Regional de Odontologia do Rio de Janeiro (CRO-RJ)
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instname_str Conselho Regional de Odontologia do Rio de Janeiro (CRO-RJ)
instacron_str CRO
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reponame_str Revista Científica do CRO-RJ (Online)
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repository.name.fl_str_mv Revista Científica do CRO-RJ (Online) - Conselho Regional de Odontologia do Rio de Janeiro (CRO-RJ)
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