Dental phenotype in Crouzon syndrome: A controlled radiographic study in 22 patients.

OBJECTIVE: This retrospective radiographic controlled study investigates the dental phenotype in patients with Crouzon syndrome to determine if differences are observed as suggested by the FGFR2C342Y/+ Crouzon mouse models, and whether these models could be of interest to study the role of this mutation in tooth development. DESIGN: We assessed dental phenotype using dedicated linear measurements in 22 children with Crouzon syndrome and compared tooth morphology in both primary and permanent dentitions to an age-matched control group. Descriptive statistics were performed with "Sex" and "Age" as covariates for the permanent tooth models and "Sex" only for the primary tooth models, to take into account potential confounding factors. RESULTS: We showed that permanent but not primary tooth dimensions were globally reduced in Crouzon syndrome, without microdontia. In permanent dentition, crown height, mesiodistal and faciolingual cervical diameters were reduced by 6.3%, 5.7% and 5.5% respectively (p < 0.05). CONCLUSION: Our results underline the implication of Fibroblast Growth Factor Receptor 2 (FGFR2) in dental development of humans and contribute to support FGFR2C342Y/+ Crouzon mouse models as partial replicas of this condition, including in the oral region.