The objective of this study was to carry out a literature review in order to elucidate general and oral physical aspects, behavioral disorders, medical considerations and discuss considerations about the dental approach in individuals with Fragile X Syndrome (FXS) as well as to provide surgeons dentists with greater care clarification towards these patients. A literature review has been carried out in the following databases: Virtual Health Library (VHL) –MEDLINE– PubMed , SciELO and in the Capes database Journals. The descriptors used were: Fragile X Syndrome, Oral Health, Systemic Condition, Behavioral Disorders. The main phenotypic characteristics found in the literature were: prominent ears, elongated face, strabismus, hyperextensible joints , macrorchidism , mitral valve prolapse, seizures. Fragile X Syndrome oral characteristics are: mandibular prognathism, atresic and deep palate, enamel hypoplasia, malocclusion, presence of biofilm due to unestablished hygiene habits, caries, calculus and gingivitis. Cognitive deficit, autism spectrum disorder, anxiety disorder and hyperactivity are behaviors found in FXS which bring difficulties for these patients to undergo a dental approach. The dental surgeon must be up-to-date and aware of the medical and behavioral conditions of FXS individuals and, thus, offer an adequate and safe dental approach. Systemic, behavioral and oral abnormalities require an initial dental treatment planning.
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