Taurodontism and Van der Woude Syndrome

Abstract

Objective: To report the occurrence of taurodontism in a clinical sample of Van der Woude syndrome (VWS) and describe its association with hypodontia and cleft type. Materials and Methods: This retrospective, cross-sectional study was carried out on chart reviews and radiographs of 13 persons with VWS. Mean age was 10 years 11 months Ϯ 1 year 5 months. Panoramic radiographs were used to confirm the presence or absence of teeth and to measure crown body and root lengths of mandibular first molars. Three-dimensional cone beam computed tomography (CT) scans were available on two persons with VWS. Both volumetric and linear measurements were obtained. Results:The occurrence of taurodontism of the mandibular first molar was 35%: 27% hypodont and 8% mesodont. Of the 13 subjects with VWS, 6 (4 males and 2 females) had at least one tooth identified with taurodontism. Half of the cases were unilateral and half were bilateral, and all of the unilateral cases were on the left side. Five of the six subjects with taurodontism had missing incisors and premolars. Taurodontism was two times more frequent in those who were missing their second premolars than in those who had their second premolars. There was no correlation between cleft type and presence of taurodontism. The cone beam CT pilot study on two persons showed very abnormal morphology of both crown and roots, which was not apparent on the standard panoramic radiograph. Both the volumetric and linear measurements of the ratio of crown body to root were highly indicative of taurodontism. Further genetic studies are needed. Conclusion: There is a likely association between VWS and taurodontism.

Taurodontism - A Review on its etiology, prevalence and clinical considerations

“…Taurodontism appears most frequently as an isolated anomaly, but it has also been associated with several developmental syndromes and anomalies including amelogenesis imperfecta, Down's syndrome, ectodermal dysplasia, Klinefelter syndrome, tricho-dento-osseous syndrome, Mohr syndrome, Wolf-Hirschhorn syndrome and Lowe syndrome (10). Taurodontism has also been reported to present with other rare syndromes such as Smith-Magenis syndrome (11), Williams syndrome (12), McCune-Albright syndrome (13) and Van der Woude syndrome (14). Theories concerning the pathogenesis of taurodontic root formation are also varied: an unusual developmental pattern, a delay in the calcification of pulpal chamber, an odontoblastic deficiency, an alteration in Hertwig's epithelial root sheath (15).…”

Prevalence of Taurodont molars in a North Indian population

“…[5] Taurodontism has also been reported to present with other rare syndromes such as Smith–Magenis syndrome,[6] Williams syndrome,[7] McCune–Albright syndrome[8] and Van der Woude syndrome. [9]…”

Imaging evalution of the gingival fibromatosis and dental abnormalities syndrome

“…[12][13][14] Several studies had demonstrated the utility of the CBCT to evaluate similar situations as found in this study, such as impacted teeth, 15 dental displacements 16 and maxillary sinus membrane thickening. 17 Hashimoto et al 18 affirmed that through the CBCT it was possible to distinguish enamel, dentine, pulp cavity and alveolar bone with better clarity, whereas Noujeim et al 19 stated that the CBCT technique has better accuracy and diagnostic value than periapical films in the detection of periodontal bone defects.…”