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Syndromes with supernumerary teeth.

Mark Lubinsky1, Piranit Nik Kantaputra2,3,4

  • 16003 W. Washington Blvd., Wauwatosa, Wisconsin.

American Journal of Medical Genetics. Part A
|June 3, 2016
PubMed
Summary

Supernumerary teeth, extra teeth, are linked to eight genetic syndromes. While often idiopathic, these dental anomalies can signal underlying Mendelian disorders, not just coincidental findings.

Keywords:
dental anomaliesextra teethsupernumerary teeth and syndromessyndromes with extra teeth

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Area of Science:

  • Genetics
  • Dentistry
  • Medical Syndromes

Background:

  • Supernumerary teeth (ST) are common, occurring in over 6% of the general population.
  • While often idiopathic, ST can be a manifestation of Mendelian genetic syndromes.
  • Distinguishing true associations from coincidental findings requires careful analysis due to ST prevalence.

Purpose of the Study:

  • To critically evaluate the evidence linking specific Mendelian syndromes with supernumerary teeth.
  • To differentiate between strong, suggestive, and unsubstantiated associations.
  • To clarify the genetic basis of supernumerary teeth beyond idiopathic occurrences.

Main Methods:

  • Systematic review and evidence analysis of reported associations between genetic syndromes and supernumerary teeth.
  • Exclusion criteria included single-patient reports, secondary dental findings (e.g., near clefts), and natal teeth.
  • Focus on specific criteria suggesting non-coincidental association, such as multiple ST or extranumerary locations.

Main Results:

  • Eight genetic syndromes demonstrated strong evidence of association with supernumerary teeth.
  • These include cleidocranial dysplasia, familial adenomatous polyposis, and Rubinstein-Taybi syndrome, among others.
  • Suggestive evidence was found for Kreiborg-Pakistani syndrome and insulin-resistant diabetes mellitus.

Conclusions:

  • A definitive list of eight genetic syndromes with strong associations to supernumerary teeth was identified.
  • Several commonly cited syndromes lacked sufficient evidence for a clear association.
  • Understanding these associations is crucial for accurate diagnosis and genetic counseling.