title: Syndromes with supernumerary teeth.
abstract: While most supernumerary teeth are idiopathic, they can be associated with a number of Mendelian syndromes. However, this can also be a coincidental finding, since supernumerary teeth occur in 6% or more of the normal population. To better define this relationship, we analyzed the evidence for specific associations. We excluded conditions with a single affected patient reported, supernumerary teeth adjacent to clefts or other forms of alveolar disruption (as secondary rather than primary findings), and natal teeth, which can involve premature eruption of a normal tooth. Since, the cause of supernumerary teeth shows considerable heterogeneity, certain findings are less likely to be coincidental, such as five or more supernumerary teeth in a single patient, or locations outside of the premaxilla. We found only eight genetic syndromes with strong evidence for an association: cleidocranial dysplasia; familial adenomatous polyposis; trichorhinophalangeal syndrome, type I; Rubinstein-Taybi syndrome; Nance-Horan syndrome; Opitz BBB/G syndrome; oculofaciocardiodental syndrome; and autosomal dominant Robinow syndrome. There is also suggestive evidence of an association with two uncommon disorders, Kreiborg-Pakistani syndrome (craniosynostosis and dental anomalies), and insulin-resistant diabetes mellitus with acanthosisnigricans. An association of a Mendelian disorder with a low frequency manifestation of supernumerary teeth is difficult to exclude without large numbers, but several commonly cited syndromes lacked evidence for clear association, including Hallermann-Streiff syndrome, Fabry disease, Ehlers-Danlos syndrome, Apert and Crouzon syndromes, Zimmermann-Laband syndrome, and Ellis-van Creveld syndrome. © 2016 Wiley Periodicals, Inc.
authors.affiliation: 6003 W. Washington Blvd., Wauwatosa, Wisconsin.
authors.forename: Mark
authors.initials: M
authors.lastname: Lubinsky
authors.affiliation: Center of Excellence in Medical Genetics Research, Chiang Mai University, Chiang Mai, Thailand. dentaland17@gmail.com.|Division of Pediatric Dentistry, Faculty of Dentistry, Department of Orthodontics and Pediatric Dentistry, Chiang Mai University, Chiang Mai, Thailand. dentaland17@gmail.com.|Dentaland Clinic, Chiang Mai, Thailand. dentaland17@gmail.com.
authors.forename: Piranit Nik
authors.initials: PN
authors.lastname: Kantaputra
mesh_terms: D000818:Animals; D003937:Diagnosis, Differential; D056726:Genetic Association Studies; D006801:Humans; D013577:Syndrome; D014096:Tooth, Supernumerary / Q000175:diagnosis* / Q000209:etiology*
keywords: dental anomalies; extra teeth; supernumerary teeth and syndromes; syndromes with extra teeth