Dental and Medical Problems

Dent Med Probl
Index Copernicus (ICV 2020) – 128.41
MEiN – 70 pts
CiteScore (2021) – 2.0
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Average rejection rate (2021) – 81.35%
ISSN 1644-387X (print)
ISSN 2300-9020 (online)
Periodicity – quarterly

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Dental and Medical Problems

2017, vol. 54, nr 2, April-June, p. 201–204

doi: 10.17219/dmp/68579

Publication type: clinical case

Language: English

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Creative Commons BY-NC-ND 3.0 Open Access

Dental management in Williams-Beuren syndrome: Case report

Postępowanie stomatologiczne w zespole Williamsa-Beurena – opis przypadku

Arlete González-Sotelo1,A,B,C,D,E,F, María A. Monter-García1,A,B,C,D,E,F, Rosalía Contreras-Bulnes1,A,B,C,D,E,F

1 Dental Research and Advanced Studies Center, School of Dentistry, Autonomous University of the State of Mexico, Mexico

Abstract

Williams-Beuren syndrome is a multisystem disorder caused by microdeletion of chromosome No. 7 at the 7q11.23 band. The physical phenotype includes typical facial dysmorphism with a flat nasal bridge, short upturned nose, periorbital puffiness, long philtrum and delicate chin. Common features include supravalvular aortic stenosis, mental retardation, learning disabilities, growth delay, infantile hypercalcemia, hyperacusis, feeding difficulties, scoliosis, strabismus and oral abnormalities. The purpose of this paper was to describe the pediatric dentistry management and dental findings of a boy aged 9 years 3 months with Williams-Beuren syndrome, who had had a painful lower right first molar because of a dental abscess that was treated under local anesthesia and antibiotic prophylaxis. Consultation with the child’s cardiologist revealed pulmonary stenosis, interatrial communication, ventricular septal defect and patent ductus arteriosus. When treating a patient with Williams-Beuren syndrome, the cardiovascular disease, mental retardation and behavior of the child must be considered. Preventive measures, education, caregiver training and frequent consultations with the pediatric dentist are essential for these patients.

Key words

Williams-Beuren syndrome, dental management

Słowa kluczowe

zespół Williamsa-Beurena, postępowanie stomatologiczne

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