Stesura Seveso Archivio Italiano di Urologia e Andrologia 2019; 91, 2130 ORIGINAL PAPER Klinefelter’s syndrome and taurodontism Emilia Giambersio 1, Vincenzo Barile 2, Antonio Marcello Giambersio 1 1 “Ambulatorio Klinefelter” ASP Potenza, Italy; 2 UOC of Radiology “Poliambulatorio Madre Teresa” ASP Potenza, Italy. Objective: Taurodontism is a dental anom- aly characterized by an enlarged pulp cham- ber and apycal displacement of the pulpar floor. The preva- lence of taurodontism in normal population is controversial. It has been reported that taurodontism is frequently observed in Klinefelter’s patients. The purpose of this study was to assess the prevalence of taurodontism in a group of Italian Klinefelter’s patients and in a randomly selected male popula- tion of Italy and to compare the results with published data. Materials and methods: Digital panoramic radiographs of 16 Klinefelter’s patients and of 100 normal males were retrospec- tively studied in order to investigate the prevalence of tau- rodontism in these groups of patients. Results: Taurodont teeth were observed in 2 of the 16 Klinefelter’s patients (12.5%) and in 2 of 100 normal males (2.0%). Conclusions: Our results confirm the higher prevalence of tau- rodontism in Klinefelter’s patients compared to the normal pop- ulation (12.5% vs. 2.0%). Due to the wide discrepancy of inci- dence of taurodontism reported in literature (0.04%-48.0% in normal population; 12.5%-88.0% in Klinefelter’s patients), we conclude that it is not possible to state which is the prevalence of taurodontism in a normal population nor among Klinefelter’s patients. KEY WORDS: Klinefelter’s syndrome; Taurodontism; 47,XXY; Dental anomaly; Digital panoramic radiographs. Submitted 30 January 2019; Accepted 11 March 2019 Summary No conflict of interest declared. PATIENTS AND METHODS We retrospectively studied the digital panoramic radi- ographs of 16 Klinefelter’s patients who attended the “Ambulatorio Klinefelter” in Italy in order to investigate the prevalence of taurodontism in this group of patients. Orthopantomograms of 100 randomly selected patients of the same male Italian population were examined as a control group. Dental radiographs were analyzed for the presence of taurodontic molars using the categorization hypo-, meso-, and hyper- taurodont based on the degree of apical displacement of the pulp chamber floor. A tooth was considered as taurodont when there was an enlarged pulp chamber that was apically displaced and a lack of constriction at the cementoenamel junction (5). RESULTS Of the 16 Klinefelter’s patients, 2 were found to exhibit taurodontic teeth (1 hypo and 1 meso-taurodont) (12.5%) (Figure 1). In the group of 100 normal patients we found 2 patients with taurodontic teeth (2 meso-tau- rodont) (2.0%). DISCUSSION Clinically, a taurodont tooth appears as normal: its dis- tinguishing features cannot be recognized clinically, therefore the diagnosis is made from radiographs. Several criteria have been proposed to define a taurodont tooth, each of them having pros and cons (1, 5-8). Moreover, most Authors do not provide an objective analysis of cases presented, preferring a subjective diag- nosis (1). It has been stated that the incidence of tau- rodontism is very low in normal population (8-10), oth- ers claim that taurodontism is not a rare trait in the mod- ern man (5). Some Authors report that taurodontism is the most common dental anomaly observed (11), others even consider taurodontism a simple variation of normal teeth (12). The prevalence of taurodontism has been reported to show a wide range of discrepancy in differ- ent populations. It was 0.04% in an Italian population (2), 1.4% in children of Saudi Arabia (13), 2.25% in a German population (14), 2.5% in a Finnish population (15), 5.6% in young adult Israeli patients (6), 15.06% in a French population of 551 patients (where it was the most common dental anomaly observed) (11), 22.9% in an Iranian population (7), 46.4% in a young Chinese DOI: 10.4081/aiua.2019.2.130 INTRODUCTION Taurodontism is defined as a change in the shape of molar teeth characterized in an enlarged pulp chamber, apical displacement of pulpar floor and no constriction at the level of cementoenamel junction (1). The origin of the word comes from the Greek tauros, which means bull and odontos which refers to tooth (“bull-like” teeth). The prevalence of taurodontism in normal population has been reported to range between 0.04% (2) and 48.0% (3). An increased incidence of taurodontism has been observed in Klinefelter’s patients with a prevalence of 19.4% (4) to 88.0% (5). The purpose of this study was to assess the incidence of taurodontism in a group of Italian Klinefelter’s patients and in a randomly selected male population of Italy and to compare the results with published data of different normal population groups and of Klinefelter’s patients. 131Archivio Italiano di Urologia e Andrologia 2019; 91, 2 Klinetaurodontism population (16) and 48% in a black Senegalese popula- tion (3). The prevalence of taurodontism that we have found in our group of 100 normal patients was 2.0%. It has been claimed that the wide discrepancy observed could be due to racial variations (17), but the inconsis- tency of prevalence observed in studies exploring the same populations (0.4% of 4143 patients (9, 18) and 2.8% of 1000 patients (19) in two north Indian popula- tions and 0.26% of 6912 patients (12) and 11.2% of 1200 patients (20) in two Turkish populations) seems to indicate that the differences reported can be rather due to different criteria used to define taurodontism rather than ethnic variations. An increased prevalence of taurodontism has been reported in Klinefelter’s patients. The prevalence observed was 19.4% of 31 Klinefelter’s patients (4), 24% of 25 patients (21), 30% of 30 Finnish 47,XXY males (15), 40% of 35 Klinefelter’s patients (22), 75% of 24 patients (23), 88% of 9 Klinefelter’s patients (5). The prevalence of taurodontism that we have found in our group of 16 Klinefelter’s patients (12.5%) appears to be lower than what observed in other Klinefelter’s popu- lations. Taurodontism seems to be associated with several other syndromes such as Down’s syndrome (24), 48,XXYY syn- drome (25), Prader-Labhart-Willi syndrome (26), Wolf- Hirschhorn syndrome (27-28), Pierre Robin syndrome (29); it is also more frequent in familial groups (5, 8), in families with WNT10A defects (30) and it is a typical trait frequently found in Neanderthal teeth (17). CONCLUSIONS Our results confirm the higher prevalence of taurodon- tism in Klinefelter’s patients compared with the preva- lence observed in a normal population (12.5% vs. 2.0%). The wide discrepancy of incidence of taurodontism reported in literature (0.04%-48.0% in normal popula- tion; 12.5%-88.0% in Klinefelter’s patients) does not allow to determine which is the prevalence of taurodon- tism in a normal population nor the prevalence among Klinefelter’s patients. REFERENCES 1. Jafarzadeh H, Azarpazhooh A, Mayhall JT. Taurodontism: a review of the condition and endodontic treatment challenges. Int Endod J. 2008; 41:375-88. 2. Laganà G, Venza N, Borzabadi-Farahani A, et al. Dental anom- alies: prevalence and associations between them in a large sample of non-orthodontic subjects, a cross-sectional study. BMC Oral Health. 2017; 17:62-8. 3. Toure B, Kane AW, Sarr M, et al. Prevalence of taurodontism at the level of the molar in the black Senegalese population 15 to 19 years of age. Odontostomatol Trop. 2000; 23:36-9. 4. Komatz Y, Tomoyoshi T, Yoshida O, et al. Taurodontism and Klinefelter’s syndrome. J Med Genet. 1978; 15:452-4. 5. Jaspers MT, Witkop Jr CJ. Taurodontism, an isolated trait associ- ated with syndromes and X-chromosomal aneuploidy. Am J Hum Genet. 1980; 32:396-413. 6. Shifman A, Chanannel I. Prevalence of taurodontism found in radiographic dental examination of 1,200 young adul Israeli patients. Community Dent Oral Epidemiol. 1978; 6:200-3. 7. Jamshidi D, Tofangchiha M, Pozve NJ, et al. Prevalence of tau- rodont molars in a selected Iranian adult population. Iran Endod J. 2017; 12:282-7. 8. Panigrahi A, Panigrahi RG, Srilatha KT, et al. Non syndromic familial bilateral decidious taurodontism - a first case report. J Clin Diagn Res. 2014; 8:ZD01-2. 9. Patil S, Doni B, Kaswan S, Rahman F. Prevalence of taurodontism in the north Indian population. J Clin Exp Dent. 2013; 5:e179-82. 10. Jayashankara CM, Shivanna AK, Sridhara KS, Kumar PS. Taurodontism: a dental rarity. J Oral Maxillofac Pathol. 2013; 17:478. 11. Baron C, Houchmand-Cuny M, Enkel B, Lopez-Cazaux S. Prevalence of dental anomalies in French orthodontic patients: a retrospective study. Arch Pediatr. 2018: 25:426-30. 12. Colak H, Tan E, Byraktar Y, et al. Taurodontism in a central anatolian population. Dent Res J. 2013; 10:260-3. 13. Yassin SM. Prevalence and distribution of selected dental anom- alies among saudi children in Abha, Saudi Arabia. J Clin Exp Dent. 2016; 8:e485-90. 14. Burklein S, Breuer D, Schafer E. Prevalence of taurodont and pyramidal molars in a German population. J Endod. 2011; 37:158-62. 15. Varrela J, Alvesalo L. Taurodontism in 47,XXY males: an effect of the extra X chromosome on root development. J Dent Res. 1988; 67:501-2. 16. MacDonald-Jankowski DS. Taurodontism in a young adult Chinese population. Dentomaxillofac Radiol. 1993; 22: 140-4. 17. Benazzi S, Nguyen HN, Kullmer O, Hublin J. Exploring the bio- mechanics of taurodontism. J Anat. 2015; 226:180-8. 18. Patil S, Doni B, Kaswan S, Rahman F. Prevalence of dental anomalies in Indian population. J Clin Exp Dent. 2013; 5:e183-6. 19. Bharti R, Chandra A, Tikku AP, Arya D. Prevalence of taurodont molars in a north Indian population. Indian J Dent. 2015; 6:27-31. Figure 1. Radiograph of a taurodontic molar in a Klinefelter's patient with enlarged pulp chamber, apical displacement of the pulpar floor and lack of constriction at the level of the cementoenamel junction. Archivio Italiano di Urologia e Andrologia 2019; 91, 2 E. Giambersio, V. Barile, A.M. Giambersio 132 20. Bilge NH, Yesiltepe K, Torenek Agirman K, et al. Investigation of prevalence of dental anomalies by using digital panoramic radi- ographs. Folia Morphol. 2018; 77:323-8. 21. Hillebrand U, Mohr C, Plewa G. Taurodontism in patients with sex chromosome anomalies. Dtsch Z Mund Kiefer Gesichtschir. 1990; 14:187-9. 22. Rossiwall B. Taurodontism in Klinefelter’s syndrome. In: Bandmann HJ, Breit R, editors. Klinefelter’s syndrome. Berlin: Springer-Verlag, 1984, p. 80-4. 23. Schulman GS, Redford-Badwal D, Poole A, et al. Taurodontism and learning disabilities in patients with Klinefelter syndrome. Pediatr Dent. 2005; 27:389-94. 24. Alpoz AR, Eronat C. Taurodontism in children associated with trisomy 21 syndrome. J Clin Pediatr Dent. 1997; 22:37-9. 25. Krishnamoorthy S, Gopikrishna V. Endodontic management of a hypertaurodontic tooth associated with 48,XXYY syndrome: a review and case report. J Conserv Dent. 2015; 18:265-8. 26. Bassarelli V, Baccetti T, Bassarelli T, Franchi L. The dentomax- illofacial characteristics of the Prader-Labhart-Willi syndrome. A clinical case report. Min Stomatol 1991; 40:811-9. 27. Babich SB, Banducci C, Teplitsky P. Dental characteristics of the Wolf-Hirschhorn syndrome: a case report. Spec Care Dentist. 2004; 24:229-31. 28. Johnston NJ, Franklin DL. Dental findings of a child with Wolf- Hirschhorn syndrome. Int J Paediatr Dent. 2006; 16:139-42. 29. Mateo-Castillo JF, Pagin O, Marchi Carvalho IM, et al. Novel dental phenotype in non-syndromic Pierre Robin sequence: a retro- spective study. Arch Oral Biol. 2019; 97:170-5. 30. Yang J, Wang S, Choi M, et al. Taurodontism, variations in tooth number, and misshapened crowns in Wnt10a null mice and human kindreds. Molecular Genetics & Genomic Medicine. 2015; 3:40-58. Correspondence Emilia Giambersio, MD emilia.giambersio@gmail.com Antonio Giambersio, MF giambersio@libero.it ASP Poliambulatorio Madre Teresa - Ambulatorio Klinefelter Viale del Gallitello, 85100 Potenza (Italy) Vincenzo Barile, MD vincenzo.barile@aspbasilicata.it ASP Poliambulatorio Madre Teresa - UOC of Radiology Viale del Gallitello, 85100 Potenza (Italy)