Dental anomalies in Richieri-Costa-Pereira syndrome

Objective. The objective of this study was to investigate the prevalence of dental anomalies in individuals with Richieri-Costa-Pereira syndrome. Study Design. A total of 13 individuals with Richieri-Costa-Pereira syndrome who were older than 8 years with at least 1 available panoramic radiograph were assessed. Dental anomalies were evaluated clinically and radiographically and classified as hyperplastic, hypoplastic, or heterotopic and as alterations of shape, number, position, and structure. Enamel alterations were classified by the DDE index. Results. All individuals exhibited anomalies, with predominance of hypoplastic disorders, mainly agenesis of mandibular incisors and second premolars and demarcated creamy-white enamel opacities primarily affecting the maxillary premolars. Conclusions. Individuals with Richieri-Costa Pereira syndrome exhibit high prevalence of tooth agenesis, especially mandibular incisors and premolars, as well as high frequency of enamel opacities. These findings are compatible with the mandibular cleft observed in all individuals and also reflect the hypoplastic characteristic of the syndrome. (Oral Surg Oral Med Oral Pathol Oral Radiol 2012;114:99-106)

Is missing maxillary lateral incisor in complete cleft lip and palate a product of genetics or local environment?

Objective: To test the null hypothesis: Subjects with isolated complete unilateral cleft lip and palate (UCLP) show no differences in overall frequency of tooth agenesis (hypodontia), comparing a subsample with cleft-side maxillary lateral incisor (MxI2) agenesis to a subsample without cleftside MxI2 agenesis. Findings could clarify the origins of cleft-side MxI2 agenesis. Materials and Methods: Tooth agenesis was identified from dental radiographs of 141 subjects with UCLP. The UCLP cohort was segregated into four categories according to the status and location of MxI2 in the region of the unilateral cleft: group M: subjects with one tooth, located on the mesial side of the alveolar cleft; group D: subjects with one tooth, located on the distal side of the alveolar cleft; group MD: subjects with two teeth present, one mesial and one distal to the cleft; and group ABS: subjects with lateral incisor absent (agenesis) in the cleft area. Results: The null hypothesis was rejected. Among UCLP subjects, there was a twofold increase (P < .0008) in overall frequency of tooth agenesis outside the cleft region in a subsample with cleftside MxI2 agenesis (ABS), compared to a subsample presenting with no agenesis of the cleft-side MxI2 (M+D+MD). Conclusions: Cleft-side MxI2 agenesis in CLP subjects appears to be largely a genetically controlled anomaly associated with cleft development, rather than a collateral environmental consequence of the adjacent cleft defect, since increased hypodontia involving multiple missing teeth observed remote from a cleft clearly has a significant genetic basis. (Angle Orthod. 2012;82:959-963.)