Ellis-van Creveld syndrome: systemic and oral findings

Authors

  • Lívia Maris Ribeiro Paranaíba Universidade Estadual de Montes Claros
  • Cassandro Moreira Fernandes Universidade José do Rosário Vellano
  • Sibele Nascimento de Aquino Universidade Estadual de Campinas
  • Luís Antônio Nogueira dos Santos Universidade Estadual de Montes Claros
  • Hercílio Martelli-Júnior Universidade Estadual de Montes Claros; Universidade José do Rosário Vellano

Keywords:

ELLIS-VAN CREVELD SYNDROME, CHONDROECTODERMAL DYSPLASIA, HYPODONTIA, ANODONTIA, CASE REPORT.

Abstract

AIMS: To present a case of Ellis-van Creveld syndrome (EvC) highlighting the systemic and oral manifestations and expand the phenotypic spectrum of the disorder. CASE DESCRIPTION: A 4-year-old female, with an initial diagnosis of EvC, was referred for dental treatment. The patient had hexadactyly of the hands, thorax disproportionate to the shortened limbs, hypopigmented and dry skin, short stature, a wide gap between the 1st and 2nd toes of the right foot and markedly dystrophic toenails. The oral manifestations were upper labial frenulum causing large vestibule and absence of diastema, labiogingival frenulum, enamel hypoplasia, conical teeth, claw-like cusp, oligodontia, microdontia and malocclusion. CONCLUSIONS: The EvC is one among several syndromes with oral manifestations that demands the participation of a multidisciplinary team to better patient assessing, treatment and monitoring. Dentists have the leading role in the control and treatment of the oral manifestations.

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Author Biographies

Lívia Maris Ribeiro Paranaíba, Universidade Estadual de Montes Claros

Clínica de Estomatologia, Departamento de Odontologia, Universidade Estadual de Montes Claros.

Cassandro Moreira Fernandes, Universidade José do Rosário Vellano

Clínica de Pediatria, Departamento de Medicina, Universidade José do Rosário Vellano.

Sibele Nascimento de Aquino, Universidade Estadual de Campinas

Departamento de Diagnóstico Oral, Faculdade de Odontologia de Piracicaba, Univerdidade Estadual de Campinas.

Luís Antônio Nogueira dos Santos, Universidade Estadual de Montes Claros

Departamento de Odontologia, Universidade José do Rosário Vellano; Clínica de Estomatologia, Departamento de Odontologia, Universidade Estadual de Montes Claros.

Hercílio Martelli-Júnior, Universidade Estadual de Montes Claros; Universidade José do Rosário Vellano

Clínica de Estomatologia, Departamento de Odontologia, Universidade Estadual de Montes Claros.

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Published

2012-07-27

How to Cite

Paranaíba, L. M. R., Fernandes, C. M., de Aquino, S. N., dos Santos, L. A. N., & Martelli-Júnior, H. (2012). Ellis-van Creveld syndrome: systemic and oral findings. Scientia Medica, 22(2), 109–112. Retrieved from https://revistaseletronicas.pucrs.br/ojs/index.php/scientiamedica/article/view/10250

Issue

Vol. 22 No. 2 (2012)

Section

Case Reports

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