Oral findings in Williams-Beuren syndrome

• Shirlene-Barbosa-Pimentel Ferreira ^[2] ; Melissa-Machado Viana ^[1] ; Naiara-Gonçalves-Fonseca Maia ^[2] ; Letícia-Lima Leão ^[1] ; Renato-Assis Machado ^[3] ; Ricardo-Della Coletta ^[3] ; Marcos-José-Burle de Aguiar ^[1] ; Hercílio Martelli-Júnior ^[4]
  1. [1] Universidade Federal de Minas Gerais

     Universidade Federal de Minas Gerais

     Brasil

     
  2. [2] Health Science Program, State University of Montes Claros, Unimontes, Minas Gerais State, Brazil
  3. [3] Department of Oral Diagnosis, Piracicaba Dental School - FOP/Unicamp, Piracicaba, São Paulo, Brazil
  4. [4] Health Science Program, State University of Montes Claros, Unimontes, Minas Gerais State, Brazil; Stomatology Clinic, Dental School, State University of Montes Claros, Unimontes, Montes Claros, Minas Gerais State, Brazil

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• Localización: Medicina oral, patología oral y cirugía bucal. Ed. inglesa, ISSN-e 1698-6946, Vol. 23, Nº. 1 (January ), 2018
• Idioma: inglés
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• Resumen

  • 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.