DELAYED DENTAL MATURATION IN CLEIDOCRANIAL DYSPLASIA

Cleidocranial dysplasia (CCD), a rare, inherited, generalized, skeleta l and dental dysplasia, exhibiting an autosomal dominant mode of trans mission, may be associated with delays during tooth maturation. To tes t whether permanent tooth formation is delayed in patients with CCD an d if the presence of supernumerary teeth adversely influences maturati on of the dentition, a group of CCD patients (eight females, three mal es) was compared to an equal number of control subjects matched for ag e and gender. Dental maturity was assessed using panoramic radiographs and the Dental Maturity Ratio, (DMR = mean dental age divided by the chronological age) was calculated. The mean DMR in CCD patients (0.87 +/- 0.14) was lower than in the control group (1.06 +/-: 0.14), p<0.01 . Among patients with CCD, patients with supernumerary teeth, had a lo wer DMR (0.82 +/- 0.13 vs. 0.91 +/- 0.16), but the difference did not reach statistical significance. After adjusting for the presence of su pernumerary teeth the diagnosis of CCD was still found to be associate d with lower DMR than controls, p = 0.0569. We conclude that CCD patie nts have delayed tooth development of approximately 2.1 years and that among these patients, those with supernumerary teeth were further del ayed by 1.5 years.