Prader-Willi syndrome: a case report

Abstract

The Prader-Willi syndrome (PWS) is a genetic disorder characterized by hypotonia, mental retardation, hyperphagia and compulsive eating due to hypothalamic dysfunction. The aim of this study is to present the case of a patient with PWS, reviewing the literature on its main characteristics, etiology and a number of dental considerations. A 12-year-old female referred to the Faculty of Dentistry of the University of Pernambuco presented marked obesity (grade 2), sleep apnea, muscular hypotonia, hyperphagia, compulsive eating behavior associated with a diet rich in refined carbohydrates and poor oral hygiene that had led to a large number of caries lesions and demineralization of the tooth enamel. In addition, Cl II malocclusion, anterior open bite and poor lip competence were found on examination of the teeth. Treatment was given using ansiolytic drugs only for the extraction procedures. The behavioral disorder was controlled using a cognitive model. A person suffering from PWS has neurobehavioral abnormalities of enormous interest to dental professionals. For this reason the development of strategies aimed at promoting both the general and oral health of PWS sufferers should be the goal of the dental team involved in the treatment of such patients. Descriptors: Prader-Willi syndrome; adolescent; dental care for patient with special needs.