Contributing Factors for Angle's Class III Phenotype in Crouzon Syndrome.

Crouzon syndrome (CS) is a rare genetic condition characterised by craniofacial malformations due to mutations in the FGFR2 gene. This study aimed to evaluate the contributing factors for Angle's Class III malocclusion in patients with CS through cephalometric analysis. Six patients with confirmed CS diagnosis were included in the study. Cephalometric measurements were performed using CT scans and compared to established norms. Pathogenic variants in the FGFR2 gene were identified in all patients. Phenotypic and cephalometric characteristics were assessed in each patient. Results showed consistent craniofacial abnormalities, including midface retrusion, mandibular prognathism, and malocclusion. Cephalometric analysis revealed specific patterns indicating growth deficiency at the posterior base of the skull, maxillary retroposition, and an anterior accommodation of the mandibular condyles in the glenoid fossa. These findings provide valuable insights into the underlying factors contributing to Angle's Class III malocclusion in patients with Crouzon syndrome.

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Journal
PubMed
Published
2026-03-04
DOI
https://doi.org/10.23804/ejpd.2025.2207
Primary Topic
Craniofacial Disorders and Treatments
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article
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article

Contributing Factors for Angle's Class III Phenotype in Crouzon Syndrome.

Juan Clinton Llerena, Fernanda R. Riba, Tatiana Protzenko, Sayonara Gonzalez et al.
PubMed
Craniofacial Disorders and Treatments
article

Contributing Factors for Angle's Class III Phenotype in Crouzon Syndrome.

Juan Clinton Llerena, Fernanda R. Riba, Tatiana Protzenko, Sayonara Gonzalez, C A Viana de Araújo
article en

Abstract

Crouzon syndrome (CS) is a rare genetic condition characterised by craniofacial malformations due to mutations in the FGFR2 gene. This study aimed to evaluate the contributing factors for Angle's Class III malocclusion in patients with CS through cephalometric analysis. Six patients with confirmed CS diagnosis were included in the study. Cephalometric measurements were performed using CT scans and compared to established norms. Pathogenic variants in the FGFR2 gene were identified in all patients. Phenotypic and cephalometric characteristics were assessed in each patient. Results showed consistent craniofacial abnormalities, including midface retrusion, mandibular prognathism, and malocclusion. Cephalometric analysis revealed specific patterns indicating growth deficiency at the posterior base of the skull, maxillary retroposition, and an anterior accommodation of the mandibular condyles in the glenoid fossa. These findings provide valuable insights into the underlying factors contributing to Angle's Class III malocclusion in patients with Crouzon syndrome.

PubMedVol. 27(1)
Fundação Oswaldo Cruz (BR)
Openalex Percentile: Top 100%
Craniofacial Disorders and Treatments
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Contributing Factors for Angle's Class III Phenotype in Crouzon Syndrome. — Juan Clinton Llerena, Fernanda R. Riba, et al. · PubMed (2026) | TGRS Research Map | TGRS