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European Archives of Paediatric Dentistry

, Volume 14, Issue 6, pp 411–416 | Cite as

Case report: Macrodont mandibular second premolars, a hereditary dental anomaly

  • A. Kyriazidou
  • D. Haider
  • C. Mason
  • S. Parekh
  • A. Bloch-ZupanEmail author
Case Report

Abstract

Background

Macrodontia or megadontia is a rare dental anomaly that refers to teeth that appear larger than normal. Generalised macrodontia may be associated with certain medical conditions and syndromes. Isolated macrodontia involves single teeth, might be the result of teeth fusion and is mainly seen in the incisor area.

Case reports

This paper describes two unrelated cases presenting with bilateral macrodont second lower premolars and the treatment provided. One case demonstrated the anomaly in both the patient and his father.

Conclusion

This case report suggests for the first time in the literature the genetic aetiology and heritability, as a possible autosomal dominant trait, of this rare dental anomaly.

Keywords

Macrodontia Premolars Teeth Genetics Dental anomalies 

Notes

Acknowledgments

This work was supported by a grant from API, 2009–2012, Hôpitaux Universitaires de Strasbourg, “development of the oral cavity: from gene to clinical phenotype in Human” and IFRO (Institut Français pour la Recherche Odontologique). We thank all patients and families who collaborated in this work, and all physicians, dentists involved in the care of these patients.

References

  1. Bloch-Zupan A, Goodman JR. Otodental syndrome. Orphanet J Rare Dis. 2006;1:5.PubMedCrossRefGoogle Scholar
  2. Bloch-Zupan A, Sedano H, Scully C. Dento/oro/craniofacial anomalies and genetics. London: Elsevier Inc.; 2012.Google Scholar
  3. Brook AH. A unifying aetiological explanation for anomalies of human tooth number and size. Arch Oral Biol. 1984;29(5):373–8.PubMedCrossRefGoogle Scholar
  4. Brook AH, Winter GB. Double teeth. A retrospective study of ‘geminated’ and ‘fused’ teeth in children. Br Dent J. 1970;129(3):123–30.PubMedCrossRefGoogle Scholar
  5. Canoglu E, Canoglu H, Aktas A, Cehreli ZC. Isolated bilateral macrodontia of mandibular second premolars: a case report. Eur J Dent. 2012;6(3):330–4.PubMedGoogle Scholar
  6. Canut JA, Arias S. Molarization of the lower second premolars. Angle Orthod. 1999;69(4):380–1.PubMedGoogle Scholar
  7. Chase RH. Unusual macrodontia. Gen Dent. 1994;42(3):216.PubMedGoogle Scholar
  8. Chate RA. Odontometric evaluation of mandibular premolars with tooth shape deviation: a case–control study. Angle Orthod. 2012;82(5):785–91.PubMedCrossRefGoogle Scholar
  9. Cortes-Breton Brinkmann J, Barona-Dorado C, Martinez-Rodriguez N, Martin-Ares M, Martinez-Gonzalez JM. Nonsyndromic multiple hyperdontia in a series of 13 patients: epidemiologic and clinical considerations. J Am Dent Assoc. 2012; 143(6):e16–e24.Google Scholar
  10. Dadpe MV, Kale YJ, Patil PS. Molarization of the mandibular second premolars with concurrent dentin dysplasia: a rare case report. IJCD. 2010;1(2):66–9.Google Scholar
  11. Deshingkar SA, Barpande SR, Bhavthankar JD. Congenital hemifacial hyperplasia. Contemp Clin Dent. 2011;2(3):261–4.PubMedCrossRefGoogle Scholar
  12. Dugmore CR. Bilateral macrodontia of mandibular second premolars: a case report. Int J Paediatr Dent. 2001;11(1):69–73.PubMedCrossRefGoogle Scholar
  13. Ekman-Westborg B, Julin P. Multiple anomalies in dental morphology: macrodontia, multituberculism, central cusps, and pulp invaginations. Report of a case. Oral Surg Oral Med Oral Pathol. 1974;38(2):217–22.PubMedCrossRefGoogle Scholar
  14. Fuentes R, Borie E. Bilateral macrodontia of mandibular second premolars: a case report. J Morphol Sci. 2011;28(3):212–5.Google Scholar
  15. Garib DG, Peck S. Extreme variations in the shape of mandibular premolars. Am J Orthod Dentofacial Orthop. 2006;130(3):317–23.PubMedCrossRefGoogle Scholar
  16. Gregory-Evans CY, Moosajee M, Hodges MD, et al. SNP genome scanning localizes oto-dental syndrome to chromosome 11q13 and microdeletions at this locus implicate FGF3 in dental and inner-ear disease and FADD in ocular coloboma. Hum Mol Genet. 2007;16(20):3482–93.CrossRefGoogle Scholar
  17. Groper JN. Macrodontia of a single tooth: review of literature and report of case. J Am Dent Assoc. 1987;114(1):69.PubMedGoogle Scholar
  18. Hennekam RCM, Krantz ID, Allanson JE. Gorlin’s syndromes of the head and neck (Contributors Allanson JE, Bloch-Zupan A, Cohen MM, Cormier-Daire V, Cunniff C, Devriendt K, Geraghty MT, Gorlin RJ, Graham JM, Grunewald S, Hall JG, Hennekam RCM, Krantz ID, Loeys B, MacDonald IM, Mortier G, Moss C, Muenke M, Wilson L) 5th edn. New York: Oxford University Press; 2010.Google Scholar
  19. Holmes J, Tanner MS. Premature eruption and macrodontia associated with insulin resistant diabetes and pineal hyperplasia. Report of two cases. Br Dent J. 1976;141(9):280–4.PubMedCrossRefGoogle Scholar
  20. Kamal D, Breton P, Bouletreau P. Congenital infiltrating lipomatosis of the face: report of three cases and review of the literature. J Craniomaxillofac Surg. 2010;38(8):610–4.PubMedCrossRefGoogle Scholar
  21. Lunt DA. ‘Molarization’ of the mandibular second premolars. J Dent. 1976;4(2):83–6.PubMedCrossRefGoogle Scholar
  22. Nemes JA, Alberth M. The Ekman-Westborg and Julin trait: report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006;102(5):659–62.PubMedCrossRefGoogle Scholar
  23. Peck S, Peck H, Phaneuf RA. Megadontism anomaly of the mandibular second premolars. Oral Surg Oral Med Oral Pathol. 1983;55(5):543.PubMedCrossRefGoogle Scholar
  24. Polder BJ, Van’t Hof MA, Van der Linden FP, Kuijpers-Jagtman AM. A meta-analysis of the prevalence of dental agenesis of permanent teeth. Community Dent Oral Epidemiol. 2004;32(3):217–26.PubMedCrossRefGoogle Scholar
  25. Primack JE. Individual bilateral megadontism: report of case. J Am Dent Assoc. 1967;75(3):655–7.PubMedGoogle Scholar
  26. Reichart PA, Westergaard J, Jensen KA. Macrodontia of a mandibular premolar. Oral Surg Oral Med Oral Pathol. 1977;44(4):606–9.PubMedCrossRefGoogle Scholar
  27. Rootkin-Gray VF, Sheehy EC. Macrodontia of a mandibular second premolar: a case report. ASDC J Dent Child. 2001;68(5–6):347–9, 302.Google Scholar
  28. Ruprecht A, Singer DL. Macrodontia of the mandibular left first premolar. Oral Surg Oral Med Oral Pathol. 1979;48(6):573.PubMedCrossRefGoogle Scholar
  29. Sirmaci A, Spiliopoulos M, Brancati F, et al. Mutations in ANKRD11 Cause KBG syndrome, characterized by intellectual disability, skeletal malformations, and macrodontia. Am J Hum Genet. 2011;89(2):289–94.PubMedCrossRefGoogle Scholar
  30. Thesleff I. Genetic basis of tooth development and dental defects. Acta Odontol Scand. 2000;58(5):191–4.PubMedCrossRefGoogle Scholar
  31. Tucker AS, Sharpe PT. Molecular genetics of tooth morphogenesis and patterning: the right shape in the right place. J Dent Res. 1999;78(4):826–34.PubMedCrossRefGoogle Scholar
  32. Ufomata D. Microdontia of a mandibular second premolar. Oral Surg Oral Med Oral Pathol. 1988;65(5):637–8.PubMedCrossRefGoogle Scholar

Copyright information

© European Academy of Paediatric Dentistry 2013

Authors and Affiliations

  • A. Kyriazidou
    • 1
    • 2
  • D. Haider
    • 2
  • C. Mason
    • 2
  • S. Parekh
    • 1
    • 2
  • A. Bloch-Zupan
    • 1
    • 3
    • 4
    • 5
    Email author
  1. 1.Eastman Dental InstituteUniversity College of LondonLondonUK
  2. 2.Dental and Maxillofacial DepartmentGreat Ormond Street HospitalLondonUK
  3. 3.Faculty of DentistryUniversity of StrasbourgStrasbourgFrance
  4. 4.Reference Centre for Orodental Manifestations of Rare Diseases, Pôle de Médecine et Chirurgie Bucco-DentairesHôpitaux Universitaires de Strasbourg (HUS)StrasbourgFrance
  5. 5.Institut de Génétique et de Biologie Moléculaire et Cellulaire (IGBMC), Centre Européen de Recherche en Biologie et en Médecine (CERBM), Centre National de la Recherche Scientifique (UMR 7104), Institut National de la Santé et de la Recherche Medicale (U 964), Université de StrasbourgIIIkirchFrance

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