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- Title
Ellis-van Creveld syndrome: systemic and oral findings.
- Authors
Ribeiro Paranaíba, Lívia Maris; Moreira Fernandes, Cassandro; de Aquino, Sibele Nascimento; Nogueira dos Santos, Luis Antônio; Martelli-Júnior, Hercílio
- Abstract
Aims: To present a case of Ellis-van Creveld syndrome 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 Ellis-van Creveld syndrome, 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: Ellis-van Creveld syndrome 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.
- Subjects
ELLIS-van Creveld syndrome; ORAL manifestations of general diseases; LABIAL frenulum; DIASTEMA (Teeth); MEDICAL needs assessment
- Publication
Scientia Medica, 2012, Vol 22, Issue 2, p109
- ISSN
1806-5562
- Publication type
Case Study