Resumen

FERRARESSO, Lucas Fernando Oliveira Tomáz et al. Oral manifestations in child patient with Ellis-van Creveld syndrome: case report. Rev Odontoped Latinoam [online]. 2023, vol.13, e-223592.  Epub 27-Mar-2024. ISSN 2174-0798.  https://doi.org/10.47990/alop.v13i1.592.

Ellis-van Creveld syndrome is an autosomal recessive disorder characterized by a tetrad of disproportionate dwarfism, ectodermal dysplasia, postaxial polydactyly, and congenital heart malformations. In this article, we hereby present a case of a 6-year-old Brazilian boy with Ellis-van Creveld syndrome who presented with a remarkable number of classical oral and dental features and uncommon findings such as taurodontism. Clinical examination revealed multiple enamel hypoplasia, absent vestibular sulcus, alveolar serrations in the maxilla anterior region, missing teeth, conical teeth, lower canine rotation, bilateral posterior crossbite, dental caries, and a nodule. Radiographically were observed teeth agenesis, taurodontism of deciduous and permanent molars, and delayed tooth eruption. Clinical and radiographic findings may be present from birth and the pediatric dentist has a fundamental role in the early diagnosis of Ellis-van Creveld syndrome, as well as oral problems prevention, rehabilitation, and aesthetic interventions.

Palabras clave : congenital heart disease; dental caries; pediatric dentistry; Ellis-van Creveld syndrome.

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