Taurodontism and Van der Woude syndrome. Is there an association?

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 +/- 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.