Anomalías dentarias en un paciente con síndrome de Williams. Reporte de un caso

Abstract

Introduction: Alterations at the oral level in patients with Williams Syndrome (WS) are frequent, 85% of patients present malocclusions according to the American Academy of Pediatrics; they also show, developmental alterations such as: hypodontia, enamel hypoplasia, gemination, fusion, etc. Objective: To analyze dental anomalies for a comprehensive dental approach in patients with Williams syndrome. Presentation of the case: A 9 year old school patient with WS, consulted for evaluation at the Pediatric Dentistry Clinic of the Universidad de los Andes, Chile. After obtaining informed consent, a clinical examination was performed, showing: first stage mixed dentition, permanence of incisors, diastemas, hypomineralization and increased overbite. Radiographically, agenesis, delayed eruption and alterations in dental anatomy were observed. Treatment is subject to the response of the medical interconsultation, therefore: fluoride application, oral hygiene instruction and dietary counseling have been performed. Conclusions: Within the care should be considered that patients with SW are patients with delayed cognitive development, often unable to perform optimal dental hygiene, which added to the presence of dental anomalies, can negatively affect the elimination of bacterial plaque and the consequent increased risk of bacterial endocarditis (prevalence close to 6%). Care should consider a previous medical evaluation to define the indications to be followed prior to dental care, in combination with an interdisciplinary orthodontic team in order to provide the esthetic solutions that many of these patients are looking for.