Delayed dental maturation in cleidocranial dysplasia

Area of Science:

• Genetics
• Dentistry
• Skeletal Dysplasia

Background:

• Cleidocranial dysplasia (CCD) is a rare, inherited skeletal and dental disorder.
• CCD is characterized by autosomal dominant transmission and may affect tooth maturation.
• The relationship between CCD, supernumerary teeth, and dental development requires further investigation.

Purpose of the Study:

• To determine if permanent tooth formation is delayed in patients with Cleidocranial dysplasia (CCD).
• To assess if the presence of supernumerary teeth exacerbates dental maturation delays in CCD patients.
• To compare dental maturity between CCD patients and age/gender-matched controls.

Main Methods:

• A cohort of 8 female and 3 male CCD patients was compared to an equal number of controls.
• Dental maturity was evaluated using panoramic radiographs.
• The Dental Maturity Ratio (DMR = mean dental age / chronological age) was calculated for all subjects.

Main Results:

• CCD patients exhibited a significantly lower mean DMR (0.87 ± 0.14) compared to controls (1.06 ± 0.14), indicating delayed tooth development (p < 0.01).
• Within the CCD group, patients with supernumerary teeth showed a trend towards lower DMR (0.82 ± 0.13) than those without (0.91 ± 0.16), though not statistically significant.
• Even after accounting for supernumerary teeth, CCD diagnosis remained associated with reduced DMR (p = 0.0569).

Conclusions:

• Cleidocranial dysplasia (CCD) is associated with an approximate 2.1-year delay in tooth development.
• Supernumerary teeth in CCD patients appear to cause an additional delay of approximately 1.5 years in dental maturation.
• These findings highlight the significant impact of CCD on dental development and the potential compounding effect of supernumerary teeth.