ORAL MANIFESTATIONS IN PATIENT WITH WILLIAMS-BEUREN SYNDROME: CASE REPORT

Williams-Beuren syndrome (SWB) has genetic etiology, is caused by microdeletions in the region of chromosome 7 q11-23, and presents as clinical manifestations facial dysmorphology, short stature, hypotonia, mental retardation, congenital heart disease, infantile hypercalcemia, and dental anomalies. This study describes a 10-year-old male patient, clinically diagnosed as having SWB, with congenital aortic stenosis and mental retardation, who was followed up by a cardiologist, psychologist, geneticist, speech therapist, and physiotherapist. The patient attended the dental service, evidencing the presence of a mixed dentition, dental calculus, and edematous gingiva as a consequence of poor hygiene. The treatment plan was established through radiographic examinations of the elements 16, 26, 36, and 46; complete blood count; prophylactic antibiotic prescription; medical release for extraction of teeth 85, 84, 74, 53, and 65; restorations; and prophylaxis. At the end of the procedures, the patient returned after 7 days for suture removal, with continuity only for routine examinations.