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.

Dientes Supernumerarios Suplementarios en un Paciente Adulto Parcialmente Dentado: Reporte de Caso

“…Para la pesquisa e identificación de dientes supernumerarios, además del historial clínico, el examen radiográfico es esencial (Brook, 1975;Mali et al, 2012;Thumati et al, 2014), más aún para el caso de pacientes sindrómicos (Wang et al, 2007;Lubinsky & Kantaputra), con antecedentes familiares de hiperdoncia (Skrinjaric & Barac-Furtinovic, 1991;Umweni & Osunbor, 2002;Wang et al), historial de anomalías dentarias en dentición primaria (Skrinjaric & Barac-Furtinovic;Whittington & Durward, 1996;Mukhopadhyay & Mitra, 2014) o que presenten simultáneamente alguna otra alteración dentaria. De acuerdo con Peker et al (2009), en un 8,1 % de los casos de hiperodoncia se observó también microdoncia y dilaceración en molares.…”

“…También se ha asociado la presencia de dientes supernumerarios con algunos síndromes tales como la displasia cleidocranial, síndrome de Gardner y fisura labiopalatina (Lubinsky & Kantaputra, 2016); además de la interacción de factores genéticos, epigenéticos y ambientales (Montasser & Taha, 2012;Juuri & Balic). Pueden presentarse de forma única o múltiple, unilateral o bilateral y en cualquier lugar del arco dentario maxilar o mandibular, siendo más común en dentición permanente que primaria y más frecuentes en hombres que en mujeres (Juuri & Balic).…”

Supernumerary Teeth – Literature Review

“…So hyperdontia is a disorder related to polygenic inheritance and to the hyperactivity of dental lamina. Supernumerary teeth often constitute an element of developmental syndromes and disorders, such as amelogenesis imperfecta, cleft lip and palate, craniosynostosis, cleidocranial dysplasia, Crouzon syndrome, Franceschetti syndrome, Gardner syndrome, Down syndrome, Noonan syndrome, Rubenstein-Taybi syndrome, Zimmermann-Laband syndrome, Fabry syndrome, Ellis-Van Creveld syndrome, Ehlers-Danlos syndrome III and IV type, incontinentia pigmenti syndrome, oro-facio-digital type I, tricho-rhino-phalangeal syndrome, Rothmund-Thomson syndrome, Nance-Horan syndrome, Robinow syndrome, anophthalmia syndrome and Hallerman-Streiff syndrome [9,14,19,22,24,25].…”

“…Supernumerary teeth which have mineralised at a late stage may cause diagnostic difficulties during X-ray examinations. In X-ray images, they are round translucencies delimited by a poorly mineralized bone crypt of a tooth bud [25].…”

Teeth Impaction and Structural Teeth Anomalies

“…34 Genetic transmission is autosomal dominant or X-linked, causing men to be twice as affected as women. 35 Supernumerary teeth appear as a result of the complex division of a dental bud or after excessive, but organized, development. These occur frequently in permanent dentition and develop in the incisor, premolar, and, rarely, in the molar area.…”