A dental approach to Proteus Syndrome: a case report

Detalhes bibliográficos
Autor(a) principal: Rocha, Vilma Inutuka Pereira
Data de Publicação: 2022
Outros Autores: Gomes, Bruna Yasmin, Cauhi, Letícia de Paula, Benicio, Rosenelle Oliveira Araújo, Haddad, Aida Sabbagh
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Research, Society and Development
Texto Completo: https://rsdjournal.org/index.php/rsd/article/view/32078
Resumo: Proteus syndrome (SP; OMIM #176920) is a rare complex disorder, characterized by the asymmetric postnatal growth of different parts of the body which tends to progress throughout life. Its prevalence is estimated at 1 per 1,000,000 live births. The clinical features are: partial gigantism of extremities, plantar hyperplasia, macrocephaly, cranial hyperostosis and asymmetry of the limbs due to the growth of long bones and soft tissues. Some of those affected may have intellectual impairment. Diagnosis is based on clinical criteria, imaging and genetic exams. The mainstay of treatment is early diagnosis of the disease and medication for symptoms presenting throughout life to improve quality of life. This case report presents a female patient, from 4½ years of age to the present, with asymmetric growth of the face and upper and lower limbs, undergoing dental follow-up at the Dental Service of a Specialist Center for Rehabilitation and Readaptation in Goiânia, Brazil. She presents delay in neuropsychomotor development, pronounced osteopenia, does not speak and is dependent in terms of activities of daily living. The eruption of deciduous teeth began at 4 months and permanent dentition at 4 years of age. During the current clinical examination of the child at 12 years and 2 months we found severe enamel hypoplasia in the teeth on the right side, mandibular prognathism, maxillary atresia, ogival palate, and anterior crossbite. The treatment plan included prevention and maintenance of oral health, monitoring of enamel hypoplasia, rhizolysis of the deciduous dentition and of eruption of permanent dentition, and restoration and extraction of teeth which were causing traumatic lesions on soft tissues. The parents' concerns were the malformations in dental enamel and traumatic lesions in the mucous membranes caused by badly positioned teeth. The interventions performed were composite resin restorations, tooth extractions, oral hygiene guidance and topical application of fluoride. Orthodontic treatment was contraindicated due to the severity of the disease. The dental approach in severe cases of PS must be individualized, with emphasis on oral hygiene and the removal of traumatic factors which damage soft tissues and cause pain. Dental surgeons must be prepared for individualized management.
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spelling A dental approach to Proteus Syndrome: a case reportAbordaje odontológico en el Síndrome de Proteus: reporte de casoAbordagem odontológica na Síndrome de Proteus: relato de casoAnormalidades congênitasAnormalidades craniofaciaisSíndrome HamartomatosaSíndrome de Proteus.Anomalías congénitasAnomalías craneofacialesSíndrome hamartomatosoSíndrome de Proteo.Congenital malformationsCraniofacial abnormalitiesHamartomatous syndromeProteus syndrome.Proteus syndrome (SP; OMIM #176920) is a rare complex disorder, characterized by the asymmetric postnatal growth of different parts of the body which tends to progress throughout life. Its prevalence is estimated at 1 per 1,000,000 live births. The clinical features are: partial gigantism of extremities, plantar hyperplasia, macrocephaly, cranial hyperostosis and asymmetry of the limbs due to the growth of long bones and soft tissues. Some of those affected may have intellectual impairment. Diagnosis is based on clinical criteria, imaging and genetic exams. The mainstay of treatment is early diagnosis of the disease and medication for symptoms presenting throughout life to improve quality of life. This case report presents a female patient, from 4½ years of age to the present, with asymmetric growth of the face and upper and lower limbs, undergoing dental follow-up at the Dental Service of a Specialist Center for Rehabilitation and Readaptation in Goiânia, Brazil. She presents delay in neuropsychomotor development, pronounced osteopenia, does not speak and is dependent in terms of activities of daily living. The eruption of deciduous teeth began at 4 months and permanent dentition at 4 years of age. During the current clinical examination of the child at 12 years and 2 months we found severe enamel hypoplasia in the teeth on the right side, mandibular prognathism, maxillary atresia, ogival palate, and anterior crossbite. The treatment plan included prevention and maintenance of oral health, monitoring of enamel hypoplasia, rhizolysis of the deciduous dentition and of eruption of permanent dentition, and restoration and extraction of teeth which were causing traumatic lesions on soft tissues. The parents' concerns were the malformations in dental enamel and traumatic lesions in the mucous membranes caused by badly positioned teeth. The interventions performed were composite resin restorations, tooth extractions, oral hygiene guidance and topical application of fluoride. Orthodontic treatment was contraindicated due to the severity of the disease. The dental approach in severe cases of PS must be individualized, with emphasis on oral hygiene and the removal of traumatic factors which damage soft tissues and cause pain. Dental surgeons must be prepared for individualized management.El síndrome de Proteus (SP; OMIM #176920) es un trastorno raro y complejo caracterizado por un crecimiento posnatal asimétrico de diferentes partes del cuerpo que tiende a progresar a lo largo de la vida. La prevalencia se estima en 1 por 1.000.000 de nacidos vivos. Las características clínicas son: gigantismo parcial de las extremidades, hiperplasia plantar, macrocefalia, hiperostosis craneal y asimetría de las extremidades por crecimiento de huesos largos y tejidos blandos. El deterioro intelectual puede estar presente. El diagnóstico se basa en criterios clínicos, pruebas de imagen y pruebas genéticas. La base del tratamiento incluye la identificación temprana de la enfermedad, medicación para los síntomas presentados durante el curso de la enfermedad, buscando calidad de vida. Presentamos el caso de una paciente de sexo femenino, de 4 años y 6 meses de edad hasta la actualidad, en tratamiento odontológico en un Centro Especializado de Rehabilitación y Readaptación, en la ciudad de Goiânia-Go, en el Centro-Oeste de Brasil con asimetría crecimiento de la cara, miembros inferiores y superiores. Presenta retraso en el desarrollo neuropsicomotor, marcada osteopenia, no habla y es dependiente para las actividades de la vida diaria. La erupción de los dientes temporales comenzó a los 4 meses de edad y la dentición permanente a los 4 años. En el examen clínico actual, con el niño de 12 años y 2 meses, se observa hipoplasia severa del esmalte en los dientes del lado derecho, prognatismo mandibular, atresia maxilar, paladar alto, mordida cruzada anterior. El plan de tratamiento consistió en prevención y mantenimiento de la salud bucal, seguimiento de la hipoplasia del esmalte, rizólisis de la dentición temporal y erupción de la dentición permanente, restauración y extracción de las piezas dentales que ocasionaron lesión traumática de los tejidos blandos. Las preocupaciones de los padres eran malformaciones en el esmalte dental y lesiones traumáticas en las mucosas causadas por dientes mal posicionados. Las intervenciones realizadas fueron restauraciones de resina compuesta, extracciones dentales, orientación de higiene bucal y aplicación tópica de flúor. El tratamiento de ortodoncia estaba contraindicado debido a la gravedad de la enfermedad. El abordaje odontológico en casos severos de PS debe ser individualizado, con énfasis en la higiene bucal y la remoción de factores traumáticos que dañan los tejidos blandos y causan dolor. Los odontólogos deben estar preparados para un manejo individualizado.A síndrome de Proteus (SP; OMIM #176920) é uma desordem rara, complexa, caracterizada por crescimento pós-natal assimétrico de diferentes partes do corpo que tendem a progredir por toda vida. A prevalência é estimada em 1 para cada 1.000.000 nascidos vivos. As características clínicas são: gigantismo parcial de extremidades, hiperplasia plantar, macrocefalia, hiperostose craniana e assimetria dos membros devido ao crescimento de ossos longos e partes moles. O comprometimento intelectual pode estar presente. O diagnóstico baseia-se em critérios clínicos, exames de imagem e teste genético. A base do tratamento inclui a identificação precoce da doença, medicamentos para sintomas apresentados com o decorrer da doença, buscando qualidade de vida. Apresentamos o caso de uma paciente do sexo feminino, desde os 4 anos e 6 meses de idade até a presente data, em acompanhamento odontológico em um Centro Especializado em Reabilitação e Readaptação, na cidade de Goiânia-Go, no Centro-Oeste do Brasil com crescimento assimétrico da face, membros inferiores e superiores. Apresenta atraso no desenvolvimento neuropsicomotor, osteopenia acentuada, não fala e é dependente para as atividades da vida diária. A erupção dos dentes decíduos iniciou-se aos 4 meses de idade e a dentição permanente aos 4 anos. Ao exame clínico atual, com a criança aos 12 anos e 2 meses, observamos hipoplasia severa de esmalte nos dentes do lado direito, prognatismo mandibular, atresia maxilar, palato ogival, mordida cruzada anterior. O plano de tratamento consistiu na prevenção e manutenção da saúde bucal, acompanhamento das hipoplasias de esmalte, da rizólise da dentição decídua e erupção da dentição permanente, restauração e exodontia de dentes que causavam lesão traumática em tecidos moles. As preocupações dos pais eram as malformações no esmalte dental e as lesões traumáticas em mucosas provocadas por dentes mal posicionados. As intervenções realizadas foram restaurações em resina composta, extrações dentárias, orientação de higiene bucal e aplicação tópica de flúor. O tratamento ortodôntico foi contraindicado devido à gravidade da doença. A abordagem odontológica nos casos graves da SP deve ser individualizada com ênfase à higiene bucal e à remoção de fatores traumáticos que lesam tecidos moles e provocam dor. Cirurgiões-dentistas devem estar preparados para o manejo individualizado.Research, Society and Development2022-07-16info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://rsdjournal.org/index.php/rsd/article/view/3207810.33448/rsd-v11i9.32078Research, Society and Development; Vol. 11 No. 9; e47811932078Research, Society and Development; Vol. 11 Núm. 9; e47811932078Research, Society and Development; v. 11 n. 9; e478119320782525-3409reponame:Research, Society and Developmentinstname:Universidade Federal de Itajubá (UNIFEI)instacron:UNIFEIenghttps://rsdjournal.org/index.php/rsd/article/view/32078/27343Copyright (c) 2022 Vilma Inutuka Pereira Rocha; Bruna Yasmin Gomes; Letícia de Paula Cauhi; Rosenelle Oliveira Araújo Benicio; Aida Sabbagh Haddadhttps://creativecommons.org/licenses/by/4.0info:eu-repo/semantics/openAccessRocha, Vilma Inutuka PereiraGomes, Bruna Yasmin Cauhi, Letícia de Paula Benicio, Rosenelle Oliveira Araújo Haddad, Aida Sabbagh 2022-07-21T12:36:16Zoai:ojs.pkp.sfu.ca:article/32078Revistahttps://rsdjournal.org/index.php/rsd/indexPUBhttps://rsdjournal.org/index.php/rsd/oairsd.articles@gmail.com2525-34092525-3409opendoar:2024-01-17T09:48:11.590341Research, Society and Development - Universidade Federal de Itajubá (UNIFEI)false
dc.title.none.fl_str_mv A dental approach to Proteus Syndrome: a case report
Abordaje odontológico en el Síndrome de Proteus: reporte de caso
Abordagem odontológica na Síndrome de Proteus: relato de caso
title A dental approach to Proteus Syndrome: a case report
spellingShingle A dental approach to Proteus Syndrome: a case report
Rocha, Vilma Inutuka Pereira
Anormalidades congênitas
Anormalidades craniofaciais
Síndrome Hamartomatosa
Síndrome de Proteus.
Anomalías congénitas
Anomalías craneofaciales
Síndrome hamartomatoso
Síndrome de Proteo.
Congenital malformations
Craniofacial abnormalities
Hamartomatous syndrome
Proteus syndrome.
title_short A dental approach to Proteus Syndrome: a case report
title_full A dental approach to Proteus Syndrome: a case report
title_fullStr A dental approach to Proteus Syndrome: a case report
title_full_unstemmed A dental approach to Proteus Syndrome: a case report
title_sort A dental approach to Proteus Syndrome: a case report
author Rocha, Vilma Inutuka Pereira
author_facet Rocha, Vilma Inutuka Pereira
Gomes, Bruna Yasmin
Cauhi, Letícia de Paula
Benicio, Rosenelle Oliveira Araújo
Haddad, Aida Sabbagh
author_role author
author2 Gomes, Bruna Yasmin
Cauhi, Letícia de Paula
Benicio, Rosenelle Oliveira Araújo
Haddad, Aida Sabbagh
author2_role author
author
author
author
dc.contributor.author.fl_str_mv Rocha, Vilma Inutuka Pereira
Gomes, Bruna Yasmin
Cauhi, Letícia de Paula
Benicio, Rosenelle Oliveira Araújo
Haddad, Aida Sabbagh
dc.subject.por.fl_str_mv Anormalidades congênitas
Anormalidades craniofaciais
Síndrome Hamartomatosa
Síndrome de Proteus.
Anomalías congénitas
Anomalías craneofaciales
Síndrome hamartomatoso
Síndrome de Proteo.
Congenital malformations
Craniofacial abnormalities
Hamartomatous syndrome
Proteus syndrome.
topic Anormalidades congênitas
Anormalidades craniofaciais
Síndrome Hamartomatosa
Síndrome de Proteus.
Anomalías congénitas
Anomalías craneofaciales
Síndrome hamartomatoso
Síndrome de Proteo.
Congenital malformations
Craniofacial abnormalities
Hamartomatous syndrome
Proteus syndrome.
description Proteus syndrome (SP; OMIM #176920) is a rare complex disorder, characterized by the asymmetric postnatal growth of different parts of the body which tends to progress throughout life. Its prevalence is estimated at 1 per 1,000,000 live births. The clinical features are: partial gigantism of extremities, plantar hyperplasia, macrocephaly, cranial hyperostosis and asymmetry of the limbs due to the growth of long bones and soft tissues. Some of those affected may have intellectual impairment. Diagnosis is based on clinical criteria, imaging and genetic exams. The mainstay of treatment is early diagnosis of the disease and medication for symptoms presenting throughout life to improve quality of life. This case report presents a female patient, from 4½ years of age to the present, with asymmetric growth of the face and upper and lower limbs, undergoing dental follow-up at the Dental Service of a Specialist Center for Rehabilitation and Readaptation in Goiânia, Brazil. She presents delay in neuropsychomotor development, pronounced osteopenia, does not speak and is dependent in terms of activities of daily living. The eruption of deciduous teeth began at 4 months and permanent dentition at 4 years of age. During the current clinical examination of the child at 12 years and 2 months we found severe enamel hypoplasia in the teeth on the right side, mandibular prognathism, maxillary atresia, ogival palate, and anterior crossbite. The treatment plan included prevention and maintenance of oral health, monitoring of enamel hypoplasia, rhizolysis of the deciduous dentition and of eruption of permanent dentition, and restoration and extraction of teeth which were causing traumatic lesions on soft tissues. The parents' concerns were the malformations in dental enamel and traumatic lesions in the mucous membranes caused by badly positioned teeth. The interventions performed were composite resin restorations, tooth extractions, oral hygiene guidance and topical application of fluoride. Orthodontic treatment was contraindicated due to the severity of the disease. The dental approach in severe cases of PS must be individualized, with emphasis on oral hygiene and the removal of traumatic factors which damage soft tissues and cause pain. Dental surgeons must be prepared for individualized management.
publishDate 2022
dc.date.none.fl_str_mv 2022-07-16
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dc.identifier.uri.fl_str_mv https://rsdjournal.org/index.php/rsd/article/view/32078
10.33448/rsd-v11i9.32078
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info:eu-repo/semantics/openAccess
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eu_rights_str_mv openAccess
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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. 11 No. 9; e47811932078
Research, Society and Development; Vol. 11 Núm. 9; e47811932078
Research, Society and Development; v. 11 n. 9; e47811932078
2525-3409
reponame:Research, Society and Development
instname:Universidade Federal de Itajubá (UNIFEI)
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reponame_str Research, Society and Development
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repository.mail.fl_str_mv rsd.articles@gmail.com
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