Genetic background of supernumerary teeth

Abstract

Supernumerary teeth (ST) are odontostomatologic anomaly characterized by as the existence excessive number of teeth in relation to the normal dental formula. This condition is commonly seen with several congenital genetic disorders such as Gardner's syndrome, cleidocranial dysostosis and cleft lip and palate. Less common syndromes that are associated with ST are; Fabry Disease, Ellis-van Creveld syndrome, Nance-Horan syndrome, Rubinstein-Taybi Syndrome and Trico–Rhino–Phalangeal syndrome. ST can be an important component of a distinctive disorder and an important clue for early diagnosis. Certainly early detecting the abnormalities gives us to make correct management of the patient and also it is important for making well-informed decisions about long-term medical care and treatment. In this review, the genetic syndromes that are related with ST were discussed.

A novel NHS mutation causes Nance-Horan Syndrome in a Chinese family

“…Nance-Horan Syndrome (NHS) (OMIM: 302350) was first described in detail by two independent studies in 1974 as a rare X-linked developmental disorder characterized by congenital cataracts, with occasional dental anomalies, brachymetacarpia and mental retardation [1–4]. It is inherited as an X-linked recessive trait, whereby heterozygous females manifest a similar but milder disease phenotype than affected males [5].…”

Non‐syndromic cleft lip with or without palate‐susceptible SNPs is associated with hyperdontia

“…Supernumerary teeth (ST) or hyperdontia is one of the most common oral hereditary diseases, characterized with the presence of more than 20 deciduous, or 32 permanent teeth in individuals (Anthonappa, King, & Rabie, ). It occurs from 1.5% to 3.5% in permanent dentition and 0.3% to 0.8% in primary dentition (Garvey, Barry, & Blake, ; Mahabob, Anbuselvan, Kumar, Raja, & Kothari, ) and always follows with other complications such as impacted teeth, malocclusion, and crowded dentition (Subasioglu et al, ), contributing much burden to oral health.…”

Radiographic Features of Supernumerary Teeth in the Southern Turkish Individuals

“…2,[4][5][6][7] Some studies suggested that the main etiological factor is genetic predisposition related to a disorder associated with a dominant autosomal gene. 2,8 According to other literature, supernumerary teeth are an autosomal recessive feature with incomplete penetrance, or they may be related with the X chromosome. 8 Supernumerary teeth may be related to varied syndromes like Ehlers-Danlos syndrome, Gardner's syndrome, Apert Syndrome, cleidocranial dysplasia and Down's syndrome.…”

“…2,8 According to other literature, supernumerary teeth are an autosomal recessive feature with incomplete penetrance, or they may be related with the X chromosome. 8 Supernumerary teeth may be related to varied syndromes like Ehlers-Danlos syndrome, Gardner's syndrome, Apert Syndrome, cleidocranial dysplasia and Down's syndrome. Supernumerary teeth associated with these syndromes often appear in multiple forms 9 , but they may also occur in patients without such syndromes.…”

“…5 Nevertheless, they can responsible root resorption, delayed or abnormal root development, displacement or rotation of the adjacent teeth, eruption failure, crowding, malocclusion, dilacerations, fistulas and cystic formation. 8 They can be classified as conical, tuberculate, supplemental, and odontomas according to their morphology. 3 Most frequently, supernumerary teeth are conical and are generally presented as mesiodens.…”