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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Dental management in Williams-Beuren syndrome: Case report
Postępowanie stomatologiczne w zespole Williamsa-Beurena – opis przypadku
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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