The Phenotypes of Spheno-Occipital Synchondrosis in Patients With Crouzon Syndrome.

Abstract

To characterize the phenotypes of spheno-occipital synchondrosis (SOS) in Chinese patients with Crouzon syndrome. Twelve patients with Crouzon syndrome were included in this retrospective study, and were divided into 2 groups. The first group included 5 patients (5-7-year old), whereas 7 patients were included in the second group (8-11-year old). Two age- and sex-matched control groups were constituted for comparison. All patients and controls were underwent preoperative computed tomography examinations of the craniofacial area, and the three-dimensional skull models, sagittal, and axial images were reconstructed. The density of the SOS region was also calculated. The SOS was partially closed in the first Crouzon patients group, whereas the SOS was open in control groups. The SOS was completely fused SOS in the second Crouzon patients group, whereas the SOS was open or partially closed in control groups. There also were short and hypoplastic sphenoid bone in both Crouzon groups when compared with controls. In addition, the average density of SOS in patients with Crouzon syndrome was higher than the control groups (P <0.01). The SOS begins to prematurely fuse in Chinese patients with Crouzon syndrome, and there are short and hypoplastic sphenoid bone in these patients. Although a definitive role of prematurely fused SOS in Crouzon syndrome cannot be drawn, our finds provide important clues into the mechanisms, and potentially provide a treatment target for midfacial and cranial vault hypoplasia in Crouzon patients.