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