Key Points
-
Describes the prevalence and possible aetiological factors of hypodontia.
-
Highlights the role of genetic factors in tooth development and agenesis.
-
Emphasises the importance of multidisciplinary approach in the management of complex cases of hypodontia.
Abstract
Hypodontia is the developmental absence of one or more teeth from the dentition and constitutes one of the most common developmental anomalies in humans with a reported prevalence of 1.6 to 9.6% in the permanent dentition. Hypodontia may occur in association with other genetic diseases, or as an isolated familial or sporadic form. This article describes the rare phenomenon of severe hypodontia in a set of triplets. The triplets presented with congenital absence of the second molars, second premolars in all quadrants and lower central incisors. An additional five teeth (upper canines, upper lateral incisors and upper left first premolar) were missing in one of the triplets. The treatment plan and the possible genetic mode of inheritance are discussed.
Similar content being viewed by others
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
References
Neville B W, Damm D D, Allen C M, Bouquot J E . Oral and maxillofacial pathology. pp 60–61. Philadelphia: WB Saunders, 1995.
Symons A L, Stritzel F, Stamatiou J . Anomalies associated with hypodontia of the permanent lateral incisors and second premolar. J Clin Pediatr Dent 1993; 17: 109–111.
Stewart R E, Barner T K, Troutman K C, Wei SH Y . Pediatric dentistry, scientific foundations and clinical practice. pp 87–109. St. Louis: CV Mosby, 1982.
Hobkirk J A, Brook A H . The management of patients with severe hypodontia. J Oral Rehabil 1980; 7: 289–298.
Hobkirk J A, King P A, Goodman J R, Jones S P . Hypodontia: 2. The management of severe hypodontia. Dent Update 1995; 22: 8–11.
Graber L W . Congenital absence of teeth: a review with emphasis on inheritance patterns. J Am Dent Assoc 1978; 96: 266–275.
Goldenberg M, Das P, Messersmith M, Stockton D W et al. Clinical, radiographic, and genetic evaluation of a novel form of autosomal-dominant oligodontia. J Dent Res 2000; 9: 1469–1475.
Jorgenson R J . Clinician's view of hypodontia. J Am Dent Assoc 1980; 101: 283–286.
Davis P J . Hypodontia and hyperdontia of permanent teeth in Hong Kong schoolchildren. Comm Dent Oral Epidemiol 1987; 15 218–220.
Silinas C F, Jorgenson R J . Dental anomalies in a black population. J Dent Res 1974; 3: 237.
Chai W L, Ngeow W C . Familial cases of missing mandibular incisor: Three case presentations. Dent Update 1999; 26: 298–302.
Lyngstadaas S P, Nordbø, Gedde-Dahl Jr T, Thrane P S . On the genesis of hypodontia and microdontia: synergism or allelism of major genes in a family with six affected members. J Med Genet 1996; 33: 137–142.
Pirinen S, Kentala A, Nieminen P et al. Recessively inherited lower incisor hypodontia. J Med Genet 2001; 38: 551–556.
Erpenstein H, Pfeiffer R A . Sex-linked dominant hereditary reduction in number of teeth. Humangenetik 1967; 4: 280–293.
Mostowska A, Kobielak A, Biedziak B, TrZeciak W H . Novel mutation in the paired box sequence of PAX9 gene in a sporadic form of oligodontia. Eur J Oral Sci 2003; 111: 272–276.
Markovic M . Hypodontia in twins. Swed Dent J 1982; Suppl. 15 153–162.
Brook A H . A unifying aetiological explanation for anomalies of human tooth number and size. Arch Oral Biol 1984; 29: 373–378.
Markovic M D . At the crossroads of oral facial genetics. Europ J Orthodont 1992; 14: 469–481.
Das P, Hai M, Elcock C et al. Novel missense mutations and a 288-bp exonic insertion in PAX9 in families with autosomal dominant hypodontia. Am J Med Genet 2003; 118A: 35–42.
Markovic M, Trisovic D . Monozygotic triplets with discordance for some traits. Europ J Orthodont 1979; 1: 189–192.
Möller P, Berg K, Ruud A F, Kvein T K . Variable expression of familial hypodontia in monozygotic triplets. Scand J Dent Res 1981; 89: 16–18.
Giuliani V, Baccetti T, Pace R, Pagavino G . The use of MTA in teeth with necrotic pulps and open apices. Dent Traumatol 2002; 18: 217–221.
Maroto M, Barberia E, Planells P, Vera V . Treatment of a non-vital immature incisor with mineral trioxide aggregate (MTA). Dent Traumatol 2003; 19: 165–169.
Hobson R S, Carter N E, Gillgrass T J et al. The interdisciplinary management of hypodontia: the relationship between an interdisciplinary team and the general dental practitioner. Br Dent J 2003; 194: 479–482.
Boruchov M J, Green L J . Hypodontia in twins and families. Am J Orthod 1971; 60: 165–174.
Grahnen H . Hypodontia in the permanent dentition. A clinical and genetical investigation. Odontol Rev 1956; 7 1–100.
Brook A H, Elcock C, Al-Sharod M H et al. Further studies of a model for the etiology of anomalies of tooth number and size in humans. Conn Tiss Res 2002; 43: 289–295.
Thesleff I . The genetic basis of normal and abnormal craniofacial development. Acta Odontol Scand 1998; 6: 321–325.
Peter H, Balling R . Teeth: where and how to make them. Trends Genet 1999; 15 59–65.
Vastardis H . The genetics of human tooth agenesis: new discoveries for understanding dental anomalies. Am J Orthod Dentofac Orthop 2000; 117 650–656.
Murray J C, Schutte B C . Cleft palate: players, pathways, and pursuits. J Clin Inves 2004; 113: 1676–1678.
Rice R, Spencer-Dene B, Connor E C et al. J Clin Inves 2004; 113: 1692–1700.
Mensah J K, Ogawa T, Kapadia H et al. Functional Analysis of a mutation in PAX9 associated with familial tooth agenesis in humans. J Biol Chem 2004; 29: 5924–5933.
Tucker A S, Sharpe P T . Molecular genetics of tooth morphogenesis and patterning: the right shape in the right place. J Dent Res 1999; 8: 826–834.
Author information
Authors and Affiliations
Corresponding author
Additional information
Refereed Paper
Rights and permissions
About this article
Cite this article
Adeboye, S., Cole, B., Hobson, R. et al. Severe hypodontia in a set of triplets. Br Dent J 201, 93–96 (2006). https://doi.org/10.1038/sj.bdj.4813818
Accepted:
Published:
Issue date:
DOI: https://doi.org/10.1038/sj.bdj.4813818


