OROFACIAL FINDINGS AND DENTAL CARE MANAGEMENT IN A PATIENT WITH ROBINOW SYNDROME
Autor(a) principal: | |
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Data de Publicação: | 2019 |
Outros Autores: | , , , , , , , |
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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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 |
format |
article |
status_str |
publishedVersion |
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) instacron:CRO |
instname_str |
Conselho Regional de Odontologia do Rio de Janeiro (CRO-RJ) |
instacron_str |
CRO |
institution |
CRO |
reponame_str |
Revista Científica do CRO-RJ (Online) |
collection |
Revista Científica do CRO-RJ (Online) |
repository.name.fl_str_mv |
Revista Científica do CRO-RJ (Online) - Conselho Regional de Odontologia do Rio de Janeiro (CRO-RJ) |
repository.mail.fl_str_mv |
revista.cientifica@cro-rj.org.br || rorefa@terra.com.br |
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1797042306243624960 |