Racial disparity of Crouzon syndrome in maxilla and mandible

Abstract

The racial disparity of facial features in craniosynostosis patients is not fully understood. The aim of this study was to explore the difference in maxillary and mandibular morphology and spatial position in Asian and Caucasian Crouzon syndrome patients. Ninety-one computed tomography scans were included (12 Asian Crouzon syndrome patients, 22 Asian controls; 16 Caucasian Crouzon syndrome patients, 41 Caucasian controls) and measured using Materialise software. The maxillary and mandibular volumes of Asian patients were both reduced by 19% (P=0.102 and P=0.187), and those of Caucasian patients were reduced by 15% (P=0.142) and 14% (P=0.211) when compared to the respective race-specific controls. Maxilla length of Asian patients was reduced by 6.36mm (14%, P=0.003), while the reduction in Caucasian patients was 4.88mm (10%, P=0.038). ANS was retracted 11.99mm (P<0.001) in Asian patients and 11.54mm (P<0.001) in Caucasian patients. The ANB angle was narrowed by 13.17° (P<0.001) in Asian patients compared to Asian controls, and by 7.02° (P<0.001) in Caucasian patients compared to Caucasian controls. The retrusive midface profiles of Asian and Caucasian Crouzon syndrome look similar; both result from the combined effect of hypoplastic size and backward displacement. However, the insufficiency was found to be more a failure of the anteroposterior maxillary length in Asian patients, and more due to posterior maxillary positioning in Caucasian patients. Therefore, prognathism in Crouzon syndrome patients is more likely caused by displacement rather than elongation of mandibular length in both races. Crouzon syndrome results in the same extent of overall volume deficiency of the maxilla and mandible in these races.