Dental Maturation in Children With the Syndrome of Crouzon and Apert

Purpose: Developing teeth are used to assess maturity and estimate age in a number of disciplines. The purpose of this investigation was to study the dental maturation in children with Crouzon or Apert syndrome compared with nonsyndromic controls. Patients and Methods: Records of 40 children with Crouzon syndrome (18 boys and 22 girls, aged 4.0 to 17.9 years) and 28 children with Apert syndrome (10 boys and 18 girls, aged 3.9 to 15.1 years) were referred to the Department of Orthodontics, Cleft Palate Team and Craniofacial Team, Erasmus MC-Sophia. Data from syndromic children were compared with data from 451 nonsyndromic children (225 boys and 226 girls, aged 2.9 to 16.9 years). From panoramic radiographs, dental maturation was determined for patients with Crouzon and Apert syndromes and compared with data collected from control children. Logistic functions were constructed for dental maturation over time for syndromes and gender. Results: Statistically significant gender differences in dental maturation scores were found for girls with Crouzon (P < .05) and Apert syndrome (P < .05). Patients with Apert syndrome demonstrated a significantly delayed dental maturation (P < .05), while patients with Crouzon syndrome showed a nonsignificant delay. Conclusions: Dental maturation in patients with Apert syndrome was more delayed than in patients with Crouzon syndrome. The delay of tooth formation in patients with Crouzon or Apert syndrome suggests a possible common genetic association

The association between WNT10A variants and dental development in patients with isolated oligodontia

In this study we aimed to determine the effect of WNT10A variants on dental development in patients with oligodontia. Forty-three (25 boys and 18 girls) individuals were eligible for this study. Stage of development for each present tooth was assessed using the Demirjian method. In case no corresponding tooth was present, regression equations were applied for dental age to be calculated. The ratio between length of root and length of crown was ascertained for each present tooth in all quadrants. All patients were physically examined by a clinical geneticist and DNA analysis of the WNT10A gene was performed. Linear regression models were applied to analyze the association between WNT10A variants and dental age. The same analysis was applied to study the association between WNT10A variants and root elongation for each present tooth. One ordinal regression model was applied to analyze the association between WNT10A variants and development of present maxillary and mandibular teeth. Thirty-six (84%) patients were detected with WNT10A variants of which six patients displayed evident ectodermal features. Dental age was 1.50 (95% confidence interval (CI): -2.59, -0.42) to 1.96 (95% CI: -3.76, -0.17) years lower in patients with WNT10A variants compared with patients without variants. The development of maxillary canine, maxillary second molar and mandibular second molar was statistically significantly delayed in patients with WNT10A variants compared with patients without variants. The impact of WNT10A variants on dental development increases with presence of the nonsense c.(321C>A p.(C107*)) variant and the number of missing teeth