The genetic basis of a dentigerous cyst associated with a supernumerary tooth?

Oral Sessions - O12/Syndromes and Genetics 2INTRODUCTION: Supernumerary teeth are teeth additional to the normal complement and the frequent clinical indicators are noneruption or delayed eruption of teeth, crowding, displacement and dentigerous cyst (DC) formation. Approximately, 95% of DCs involve the permanent dentition and only 5% are associated with supernumerary teeth. The objectives of this study were to: (i) report a pair of siblings with supernumeraries of whom, one developed a dentigerous cyst and (ii) analyze fragile histidine triad (FHIT) and p53 gene status in DC associated with supernumerary teeth. CLINICAL MANAGEMENT: This six-year-old Chinese boy complained of swelling in the maxillary right incisor region. Radiographs revealed the presence of two supernumeraries in the premaxillary region, one of which had evidence of an enlarged follicle. Histopathology confirmed that the cystic lining was consistent with a DC. The patients elder sister also exhibited two supernumeraries in the premaxilla which was subsequently surgically removed. Blood samples were collected and the DNA extracted using the Qiagen_ mini blood kit. The DNA yield was quantified using 260 nm/280 nm ratio. After isolation, aliquots of the DNA samples were prepared for analyzing the status of the FHIT and p53 gene. CONCLUSION: Occurrence of supernumerary teeth in siblings indicates that inheritance is a major contributory factor in their development. Although DCs are uncommon in the first decade of life, regular inspections of radiographs are important. It is hypothesized that aberrations of FHIT and p53 genes could be considered as markers responsible for the development of DCs associated with supernumerary teeth.link_to_OA_fulltextThe 22nd Congress of the International Association of Paediatric Dentistry, Munich, Germany, 17-20 June, 2009. In International Journal of Paediatric Dentistry, 2009, v. 19, suppl. s1, p. 32, abstract no: O12-8