Dental development and tooth agenesis in children with velocardiofacial syndrome.

BACKGROUND. Variations in dental development and tooth agenesis have been reported in children with velocardiofacial syndrome (VCFS). AIM. The aim was to evaluate the dental development and missing permanent teeth in children with VCFS. DESIGN. Forty-five children (23 girls) with VCFS who had visited the cleft palate and craniofacial centre were studied retrospectively from orthopantomograms taken at the mean age of 7.9 years (range 5.8-12.9). Thirteen of the children with VCFS had palatal clefts. The deletion of 22q11 was verified by FISH techniques. The dental stages were assessed by the method of Demirjian, and the dental age was calculated according to the Finnish dental maturity reference values. A paired Student's t-test was used in the statistical analysis. RESULTS. Eight children (17%), four with palatal clefts, had tooth agenesis. Four children (9%) had agenesis of mandibular incisors. The missing teeth (n = 19) were mainly mandibular incisors (n = 6), maxillary lateral incisors (n = 2), and maxillary second premolars (n = 4). The dental age of the children with VCFS was not different from their chronological age, but there was great individual variation. CONCLUSIONS. A high prevalence of missing permanent teeth, especially mandibular incisors, was observed. The need for thorough clinical and radiological dental examination in children with VCFS is emphasized.