So far, however, no information is available on the sidedness of the cleft or on hypodontia in syndromic clefting associated with developmental heart defects. Local developmental factors that have an effect on hypodontia in the cleft area could include lack of outgrowth of the median nasal and/or maxillary process during embryological development.23 In addition, surgical procedures in the cleft region performed during tooth formation could be an etiological factor for absence of a tooth there. The most crucial surgical procedures that

might influence tooth formation are early periosteoplasty,24 primary bone grafting, and neonatal hard palate closure.25 and 26 Two different surgical procedures are performed in the cleft region in patients with CUCLP

in the Cleft Palate Craniofacial Unit in Nijmegen according to Selleck LGK 974 the treatment protocol followed,27 i.e. soft palate repair (modified von Langenbeck procedure) at the age of 12 months, and hard palate repair together with bone grafting of the alveolar cleft at 9 year of age.27 Owing to the timing of the previously mentioned surgical procedures, it is however, highly unlikely those patients treated according to this protocol to experience tooth agenesis because of iatrogenic factors. Therefore, cleft-side maxillary lateral incisor agenesis in patients with CUCLP probably is much more a genetically controlled anomaly associated with cleft development, rather than a collateral environmental consequence of the adjacent cleft defect.28 This sustains the hypothesis that Omipalisib order hypodontia is a phenotype of the cleft spectrum.29 A recently published study,28 much among CUCLP subjects, found that there was a twofold increase in overall frequency of tooth agenesis outside the cleft region in patients with maxillary lateral incisor agenesis at the cleft-side, compared with patients with no maxillary

lateral incisor agenesis at the cleft-side.28 Their sample was of Brazilian origin and a mixed racial background. Our findings, in Caucasians, are not in accordance with this study. There was an equal distribution of patients with tooth agenesis outside the cleft quadrant only and patients with agenesis of the maxillary lateral incisor in the cleft quadrant in combination with any of the 3 other quadrants outside the cleft. In any case, though, in almost 50% of the patterns observed in our group, agenesis was observed only outside the cleft quadrant of the maxilla or in the mandible. Ten out of the 13 agenesis patterns included missing teeth outside the cleft quadrant. The most common missing teeth in CUCLP, in the present study, and in a large group of CBCLP are the lateral incisors in the cleft quadrant and the maxillary and mandibular second premolars.30 The reported agenesis outside the cleft area in CUCLP is about 27–28%,9 and 31 whereas a higher prevalence (of 36.4%10 or even 48.8%)4 has been reported in the existing literature. In this CUCLP group, the prevalence of tooth agenesis outside the cleft was only 20.