Resumen de Oral findings in Williams-Beuren syndrome

Shirlene-Barbosa-Pimentel Ferreira, Melissa-Machado Viana, Naiara-Gonçalves-Fonseca Maia, Letícia Lima Leão, Renato Assis Machado, Ricardo Della Coletta, Marcos José Burle Aguiar, Hercílio Martelli Júnior

• Williams-Beuren syndrome (WBS; OMIM #194050) is a developmental disorder characterized by congenital heart disease, intellectual disability, dysmorphic facial features and ophthalmologic abnormalities. Oral abnormalities are also described in clinical manifestations of the disease. This paper describes orofacial features in patients with WBS.

  Seventeen patients with a confirmed molecular diagnosis of WBS were examined for oral abnormalities through clinical oral evaluations and panoramic radiography.

  Malocclusion, specifically with dental midline deviation, and high-arched palate were the most common findings.

  The present results contribute to knowledge on the orofacial manifestations of WBS. Since such patients with WBS may develop severe oral abnormalities, early detection and treatment can help improve their quality of life.