Oral Manifestations in Angelman Syndrome: A Systematic Review of the Dental Literature

Background. Angelman syndrome (AS) is a genetic disease whose incidence varies from one in 10.000 to one in 40.000 newborns. Clinically, it is characterized by a severe growth delay, speech alterations, ataxia, craniofacial abnormalities and strange behaviors. The aim of this paper is to provide a systematic review of the literature regarding the oral manifestations of this uncommon disease. Material and methods. A literature search in PubMed/MEDLINE without date restriction until May 10th, 2016 was performed. Results. Five papers met the inclusion criteria describing a total of 14 cases with AS (the first corresponds to the year 2008 and the last in 2013). Some of the dental problems among these patients such as prognathism, diastemas, lingual protrusion or drooling are common and are included as associated criteria for the diagnosis of the syndrome. Conclusions. The anterior open bite is also a very common oral finding and could be also considered as an associated criterion.