Dental treatment in a patient with Cornelia de Lange Syndrome: case report

Detalhes bibliográficos
Autor(a) principal: Gomes, Luana Ferreira
Data de Publicação: 2021
Outros Autores: Oliveira, Beatriz Reis de, Lima, Naynne Soares de, Oliveira, Victória Rocha de, Santana, Ellen Maiany Ribeiro, Vasconcellos, Sara Juliana de Abreu de, Barros, Alina Lúcia de Oliveira, Macedo, Álvaro Bezerra
Tipo de documento: Artigo
Idioma: por
Título da fonte: Research, Society and Development
Texto Completo: https://rsdjournal.org/index.php/rsd/article/view/19531
Resumo: Cornelia de Lange syndrome (CLS) is a rare genetic disease with an estimated prevalence of 1 in every 30,000 live births. The main features are distinct craniofacial disorders, abnormalities in the upper and lower limbs and intellectual deficit, varying between patients, being the main clinical diagnosis. The aim of the paper is to report a case of a patient with a dental impairment SCL. A 31-year-old male patient with a genetic diagnosis performed at the age of 3 years presented macrodontia, agenesis of upper lateral incisors, impacted teeth, crowding, protrusion of anterior teeth, caries, mandibular micrognathia, anterior open bite, posterior crossbite, palate ogival, periodontal disease and fissured tongue, therefore, he was submitted to dental treatment, under general anesthesia, due to non-collaboration for outpatient treatment. They were performed as tip extractions, restorations with composite resin in units 11 and 21, scaling of sub and supra gingival calculi, as well as topical application of fluoride. The patient remained in the hospital for a day and made a good recovery. The importance of a multidisciplinary team in monitoring patients with SCL, including dentists, should be highlighted, contributing to an improvement in the quality of life of these clients. Patients must have periodic follow-up with the dentist, including in the case reported, will be monitored every 03 months, due to the fact that it is a rare pathology, difficult to manage clinically, for the control and prevention of important dental changes.
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spelling Dental treatment in a patient with Cornelia de Lange Syndrome: case reportTratamiento dental en un paciente con Síndrome de Cornelia de Lange: relato de casoTratamento odontológico em paciente com Síndrome de Cornélia de Lange: relato de casoCraniofacial AbnormalitiesCornelia de Lange syndromePathology.Síndrome de Cornelia de LangeAnomalías CraneofacialesPatologia.Síndrome de Cornelia de LangeAnormalidades CraniofaciaisPatologia.Cornelia de Lange syndrome (CLS) is a rare genetic disease with an estimated prevalence of 1 in every 30,000 live births. The main features are distinct craniofacial disorders, abnormalities in the upper and lower limbs and intellectual deficit, varying between patients, being the main clinical diagnosis. The aim of the paper is to report a case of a patient with a dental impairment SCL. A 31-year-old male patient with a genetic diagnosis performed at the age of 3 years presented macrodontia, agenesis of upper lateral incisors, impacted teeth, crowding, protrusion of anterior teeth, caries, mandibular micrognathia, anterior open bite, posterior crossbite, palate ogival, periodontal disease and fissured tongue, therefore, he was submitted to dental treatment, under general anesthesia, due to non-collaboration for outpatient treatment. They were performed as tip extractions, restorations with composite resin in units 11 and 21, scaling of sub and supra gingival calculi, as well as topical application of fluoride. The patient remained in the hospital for a day and made a good recovery. The importance of a multidisciplinary team in monitoring patients with SCL, including dentists, should be highlighted, contributing to an improvement in the quality of life of these clients. Patients must have periodic follow-up with the dentist, including in the case reported, will be monitored every 03 months, due to the fact that it is a rare pathology, difficult to manage clinically, for the control and prevention of important dental changes.El síndrome de Cornelia de Lange (CLS) es una enfermedad genética rara con una prevalencia estimada de 1 de cada 30.000 nacidos vivos. Las principales características son los distintos trastornos craneofaciales, anomalías en miembros superiores e inferiores y déficit intelectual, que varía entre pacientes, siendo el diagnóstico principalmente clínico. El objetivo de este trabajo es reportar el caso de un paciente con SCL que presenta deterioro dentario. Paciente masculino de 31 años con diagnóstico genético realizado a la edad de 3 años que presentó macrodoncia, agenesia de incisivos laterales superiores, dientes impactados, apiñamiento, protrusión de dientes anteriores, caries, micrognatia mandibular, mordida abierta anterior, mordida cruzada posterior, paladar ojival, enfermedad periodontal y fisura de lengua, por lo que fue sometido a tratamiento odontológico, bajo anestesia general, por no colaboración para tratamiento ambulatorio. Se realizó extracción de los dientes necesarios, restauraciones con resina compuesta en las unidades 11 y 21, raspado de los cálculos sub y supragingivales, así como la aplicación tópica de flúor. El paciente permaneció en el hospital durante un día y se recuperó bien. Cabe destacar la importancia de un equipo multidisciplinar en el seguimiento de los pacientes con LCP, incluidos los odontólogos, contribuyendo a mejorar la calidad de vida de estos individuos. Los pacientes deben tener un seguimiento periódico con el dentista, incluso en el caso reportado, serán monitoreados cada 03 meses, debido a que es una patología rara, de difícil manejo clínico, para el control y prevención de alteraciones dentales importantes.A síndrome de Cornélia de Lange (SCL) é uma doença genética rara com prevalência estimada de 1 a cada 30.000 mil nascidos vivos. As principais características são desordens craniofaciais distintas, anormalidades nos membros superiores e inferiores e déficit intelectual, variando entre os portadores, sendo o diagnóstico principalmente clínico. O objetivo do trabalho é relatar um caso de um paciente com a SCL apresentando comprometimento odontológico. Paciente do gênero masculino, 31 anos com diagnóstico genético realizado aos 3 anos de idade, apresentava macrodontia, agenesia de incisivos laterais superiores, dentes impactados, apinhamento, protrusão dos dentes anteriores, cáries, micrognatia mandibular, mordida aberta anterior, mordida cruzada posterior, palato ogival, doença periodontal e língua fissurada, diante disso, foi submetido a tratamento dentário, sob anestesia geral, devido a não colaboração para tratamento em ambulatório. Foram realizadas as extrações dos dentes necessários, restaurações com resina composta nas unidades 11 e 21, raspagens dos cálculos sub e supra gengival, bem como aplicação tópica de flúor. O paciente permaneceu um dia no hospital e apresentou boa recuperação. Deve-se ressaltar a importância de uma equipe multidisciplinar no acompanhamento de pacientes com SCL, incluindo o cirurgião-dentista contribuindo para uma melhora da qualidade de vida destes indivíduos. Os pacientes devem ter acompanhamento periódico com o dentista, inclusive no caso relatado, será acompanhado a cada 03 meses, devido ao fato de ser uma patologia rara, de difícil manejo clínico, para controle e prevenção de alterações odontológicas importantes.Research, Society and Development2021-08-28info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://rsdjournal.org/index.php/rsd/article/view/1953110.33448/rsd-v10i11.19531Research, Society and Development; Vol. 10 No. 11; e190101119531Research, Society and Development; Vol. 10 Núm. 11; e190101119531Research, Society and Development; v. 10 n. 11; e1901011195312525-3409reponame:Research, Society and Developmentinstname:Universidade Federal de Itajubá (UNIFEI)instacron:UNIFEIporhttps://rsdjournal.org/index.php/rsd/article/view/19531/17423Copyright (c) 2021 Luana Ferreira Gomes; Beatriz Reis de Oliveira; Naynne Soares de Lima; Victória Rocha de Oliveira; Ellen Maiany Ribeiro Santana; Sara Juliana de Abreu de Vasconcellos; Alina Lúcia de Oliveira Barros; Álvaro Bezerra Macedohttps://creativecommons.org/licenses/by/4.0info:eu-repo/semantics/openAccessGomes, Luana Ferreira Oliveira, Beatriz Reis de Lima, Naynne Soares de Oliveira, Victória Rocha de Santana, Ellen Maiany Ribeiro Vasconcellos, Sara Juliana de Abreu de Barros, Alina Lúcia de Oliveira Macedo, Álvaro Bezerra 2021-10-23T19:01:11Zoai:ojs.pkp.sfu.ca:article/19531Revistahttps://rsdjournal.org/index.php/rsd/indexPUBhttps://rsdjournal.org/index.php/rsd/oairsd.articles@gmail.com2525-34092525-3409opendoar:2024-01-17T09:39:24.108116Research, Society and Development - Universidade Federal de Itajubá (UNIFEI)false
dc.title.none.fl_str_mv Dental treatment in a patient with Cornelia de Lange Syndrome: case report
Tratamiento dental en un paciente con Síndrome de Cornelia de Lange: relato de caso
Tratamento odontológico em paciente com Síndrome de Cornélia de Lange: relato de caso
title Dental treatment in a patient with Cornelia de Lange Syndrome: case report
spellingShingle Dental treatment in a patient with Cornelia de Lange Syndrome: case report
Gomes, Luana Ferreira
Craniofacial Abnormalities
Cornelia de Lange syndrome
Pathology.
Síndrome de Cornelia de Lange
Anomalías Craneofaciales
Patologia.
Síndrome de Cornelia de Lange
Anormalidades Craniofaciais
Patologia.
title_short Dental treatment in a patient with Cornelia de Lange Syndrome: case report
title_full Dental treatment in a patient with Cornelia de Lange Syndrome: case report
title_fullStr Dental treatment in a patient with Cornelia de Lange Syndrome: case report
title_full_unstemmed Dental treatment in a patient with Cornelia de Lange Syndrome: case report
title_sort Dental treatment in a patient with Cornelia de Lange Syndrome: case report
author Gomes, Luana Ferreira
author_facet Gomes, Luana Ferreira
Oliveira, Beatriz Reis de
Lima, Naynne Soares de
Oliveira, Victória Rocha de
Santana, Ellen Maiany Ribeiro
Vasconcellos, Sara Juliana de Abreu de
Barros, Alina Lúcia de Oliveira
Macedo, Álvaro Bezerra
author_role author
author2 Oliveira, Beatriz Reis de
Lima, Naynne Soares de
Oliveira, Victória Rocha de
Santana, Ellen Maiany Ribeiro
Vasconcellos, Sara Juliana de Abreu de
Barros, Alina Lúcia de Oliveira
Macedo, Álvaro Bezerra
author2_role author
author
author
author
author
author
author
dc.contributor.author.fl_str_mv Gomes, Luana Ferreira
Oliveira, Beatriz Reis de
Lima, Naynne Soares de
Oliveira, Victória Rocha de
Santana, Ellen Maiany Ribeiro
Vasconcellos, Sara Juliana de Abreu de
Barros, Alina Lúcia de Oliveira
Macedo, Álvaro Bezerra
dc.subject.por.fl_str_mv Craniofacial Abnormalities
Cornelia de Lange syndrome
Pathology.
Síndrome de Cornelia de Lange
Anomalías Craneofaciales
Patologia.
Síndrome de Cornelia de Lange
Anormalidades Craniofaciais
Patologia.
topic Craniofacial Abnormalities
Cornelia de Lange syndrome
Pathology.
Síndrome de Cornelia de Lange
Anomalías Craneofaciales
Patologia.
Síndrome de Cornelia de Lange
Anormalidades Craniofaciais
Patologia.
description Cornelia de Lange syndrome (CLS) is a rare genetic disease with an estimated prevalence of 1 in every 30,000 live births. The main features are distinct craniofacial disorders, abnormalities in the upper and lower limbs and intellectual deficit, varying between patients, being the main clinical diagnosis. The aim of the paper is to report a case of a patient with a dental impairment SCL. A 31-year-old male patient with a genetic diagnosis performed at the age of 3 years presented macrodontia, agenesis of upper lateral incisors, impacted teeth, crowding, protrusion of anterior teeth, caries, mandibular micrognathia, anterior open bite, posterior crossbite, palate ogival, periodontal disease and fissured tongue, therefore, he was submitted to dental treatment, under general anesthesia, due to non-collaboration for outpatient treatment. They were performed as tip extractions, restorations with composite resin in units 11 and 21, scaling of sub and supra gingival calculi, as well as topical application of fluoride. The patient remained in the hospital for a day and made a good recovery. The importance of a multidisciplinary team in monitoring patients with SCL, including dentists, should be highlighted, contributing to an improvement in the quality of life of these clients. Patients must have periodic follow-up with the dentist, including in the case reported, will be monitored every 03 months, due to the fact that it is a rare pathology, difficult to manage clinically, for the control and prevention of important dental changes.
publishDate 2021
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10.33448/rsd-v10i11.19531
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identifier_str_mv 10.33448/rsd-v10i11.19531
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dc.publisher.none.fl_str_mv Research, Society and Development
publisher.none.fl_str_mv Research, Society and Development
dc.source.none.fl_str_mv Research, Society and Development; Vol. 10 No. 11; e190101119531
Research, Society and Development; Vol. 10 Núm. 11; e190101119531
Research, Society and Development; v. 10 n. 11; e190101119531
2525-3409
reponame:Research, Society and Development
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