Oral manifestations in child patient with Ellis-van Creveld syndrome: case report

Lucas Fernando Oliveira Tomáz Ferraresso; Fabio Anevan Ubiski Fagundes; Mariella Padovese; Paola Singi; Cássia Cilene Dezan Garbelini; Farli Aparecida Carrilho Boer

doi:10.47990/alop.v13i.592

Autores

Lucas Fernando Oliveira Tomáz Ferraresso Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil https://orcid.org/0000-0002-2225-9262
Fabio Anevan Ubiski Fagundes Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil
Mariella Padovese Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil
Paola Singi Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil
Cássia Cilene Dezan Garbelini Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil https://orcid.org/0000-0002-8660-0677
Farli Aparecida Carrilho Boer Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil

DOI:

https://doi.org/10.47990/alop.v13i.592

Palavras-chave:

Cardiopatias Congênitas, cárie dentária;, Odontopediatria;, Síndrome de Ellis-van Creveld

Resumo

A síndrome de Ellis-van Creveld é uma doença autossômica recessiva caracterizada por uma tétrade de baixa estatura desproporcional, displasia ectodérmica, polidactilia pós-axial e malformações cardíacas congênitas. Neste artigo, será relatado um caso de um menino brasileiro de 6 anos de idade com síndrome de Ellis-van Creveld que apresenta um número notável de características orais e dentárias clássicas e achados incomuns como taurodontismo. Ao exame clínico foi revelado hipoplasia múltipla do esmalte, sulco vestibular ausente, serrilhas alveolares na maxila anterior, dentes ausentes, dentes cônicos, canino inferior rotacionado, mordida cruzada posterior bilateral, cárie dentária e um nódulo. Radiograficamente, foi observado agenesia dentária, taurodontismo de molares decíduos e permanentes e atraso na erupção dentária. Os achados clínicos e radiográficos podem estar presentes desde o nascimento e o odontopediatra tem papel fundamental no diagnóstico precoce da síndrome de Ellis-van Creveld, bem como na prevenção de problemas bucais, reabilitação e intervenções estéticas.

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