title: Oral findings and dental treatment in a child with Williams-Beuren syndrome.
abstract: Williams-Beuren syndrome (WBS), also known as Williams syndrome, is a rare congenital disorder involving cardiovascular problems, mental retardation, distinctive facial features and tooth anomalies. It is caused by the submicroscopic deletion of 1.5 to 1.8 Mb on chromosome 7q11.23. This paper reports the dental care to a 7-year-old child with WBS syndrome. The interview also revealed visual impairment, sensorineural hearing loss, hyperacusis, photophobia and hoarse voice. The intraoral clinical examination showed anterior open bite, tongue thrusting, excessive interdental spacing, enamel hypomineralization of the incisors, hypoplasia and caries lesions. The dental treatment included: modulating sessions to control aversion to noises, the photophobia, and the dental fear and anxiety because of his reduced visual acuity; oral hygiene instructions, dietary and daily use of a 0.05% sodium fluoride mouthwash; the permanent mandibular left first molar was treated endodontically, and maxillary and mandibular first molars were restored with amalgam. Due to the patient's heart defect, a prophylactic antibiotic regimen was prescribed prior to the dental procedures. This patient has been followed up for 4 years and this case report underscores the importance of early dental evaluation and counseling for parents of WBS patients.
authors.affiliation: Department of Pediatric Clinics, Dental School of Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil.
authors.forename: Carolina Paes
authors.initials: CP
authors.lastname: Torres
authors.affiliation: Department of Pediatric Clinics, Dental School of Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil.
authors.forename: Gleice
authors.initials: G
authors.lastname: Valadares
authors.affiliation: Department of Pediatric Clinics, Dental School of Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil.
authors.forename: Mariana Izabella
authors.initials: MI
authors.lastname: Martins
authors.affiliation: Department of Pediatric Clinics, Dental School of Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil.
authors.forename: Maria Cristina
authors.initials: MC
authors.lastname: Borsatto
authors.affiliation: Department of Pediatric Clinics, Dental School of Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil.
authors.forename: Kranya Victoria
authors.initials: KV
authors.lastname: Díaz-Serrano
authors.affiliation: Department of Pediatric Clinics, Dental School of Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil.
authors.forename: Alexandra Mussolino
authors.initials: AM
authors.lastname: de Queiroz
mesh_terms: D002648:Child; D006801:Humans; D008297:Male; D014071:Tooth Abnormalities / Q000628:therapy*; D018980:Williams Syndrome / Q000235:genetics / Q000503:physiopathology*