PRADER-WILLI SYNDROME: ORAL FINDINGS

Aim: Prader-Willi syndrome is a rare multisystemic genetic disorder caused by lack of expression of certain paternal genes located on chromosome 15. The syndrome, associated to hypothalamic and pituitary disfunction, is characterized by severe neonatal hypotonia and feeding problems. Neonatal hypotonia is followed, during childhood by hyperphagia and obesity. In addition, hypothalamic alterations can cause intellectual disability, behavioral problems, a high pain threshold, respiratory sleep disorders. The aim of this work is to evaluate the main dental aspects and the therapeutic implications. Methods: an analysis of the literature was performed using databases. Results: common clinical findings: gingivitis and periodontitis, dental and skeletal malocclusions, enamel hypoplasia, tooth wear and dental erosion, bruxism, xerostomia, candidiasis and angular cheilitis, high risk of caries and increased DMFT score.The typical poor oral hygiene in PWS patients is related to qualitative-quantitative changes in saliva and incoordination in tooth brushing. Conclusion: it is necessary to motivate family and patient to maintain oral hygiene. Due to the various systemic and dental problems, a six-monthly follow-up is required since early childhood. Dental treatment purposes require a multidisciplinary approach which includes periodontal and conservative cares. In prosthetics, we suggest the use of removable dentures because of the difficulty in maintaining oral hygiene.