The Role of Bone Centers in the Pathogenesis of Craniosynostosis: An Embryologic Approach Using CT Measurements in Isolated Craniosynostosis and Apert and Crouzon Syndromes

Abstract

This paper describes the role of the displacement of bone centers, i.e., the tubers, in the pathogenesis of craniosynostosis. This displacement was studied in 54 patients with isolated or syndromic craniosynostosis in the form of CT scans as well as in two dry neonate skulls with Apert syndrome. For comparison, 49 fetal and 8 normal infant dry skulls were studied. Our investigation was restricted to the coronal and metopic sutures. The results showed a significantly more occipital localization of the frontal bone center and a more frontal localization of the parietal bone center at the side of a synostotic coronal suture in the isolated form as well as in Apert syndrome. In contrast, this was not the case in Crouzon syndrome, thus showing that these two syndromes have a different pathogenesis. For trigonocephaly, a more anteromedial localization of the frontal bone centers was found.

Foramen Magnum Size and Involvement of Its Intraoccipital Synchondroses in Crouzon Syndrome

“…Because posterior intraoccipital synchondroses in Crouzon syndrome already started to fuse before the second year of life, it seems that synchondroses are constructed prenatally, but that they show premature fusion in accordance with cranial vault sutures in Crouzon syndrome patients. 34 This means that fusion might start before birth. Because posterior intraoccipital synchondroses seem to be affected earlier than anterior intraoccipital synchondroses in Crouzon syndrome, it is likely that the shape of the foramen magnum is altered into a relatively small posterior part compared with the anterior part (Fig.…”

Quantitative Analysis of Developmental Process of Cranial Suture in Korean Infants

“…In addition, most reports describing physiologic suture closure observe only the time of completion of suture closure (completely fused sutures, suture closure grade 3). Quantitative degree of closure by age has not been yet fully described, especially for sagittal, coronal and lambdoidal sutures, which usually complete their ossification process at adulthood14). Furthermore, there have been no reports to date that studied development of the cranium in Korean infants.…”

Prenatal Diagnosis of Cloverleaf Skull: Watch the Hands!

“…In the fetus, sutures are optimally viewed as echo-free structures between the calvarian bones from 14 to 16 weeks' gestation [12] . In case of craniosynostosis, instead of normal suture formation, direct fusion of dislocated bone centers take place at this period [13] .…”