Background: Isolated synostosis of the frontosphenoidal suture is very rare and difficult to diagnose. Little has been reported on the clinical presentation and fetal development of this suture. Objective: To understand the development of the frontosphenoidal suture and the outcome of its synostosis. Materials and methods: We studied the normal fetal development of the frontosphenoidal suture in dry human skulls and the clinical features of four patients with isolated synostosis of the frontosphenoidal suture. Results: The frontosphenoidal suture develops relatively late during the second trimester of pregnancy, which explains the mild phenotype when there is synostosis. This rare craniosynostosis results in a deformity that causes recession of the lateral part of the frontal bone and supraorbital rim, with minimal facial asymmetry. Three-dimensional CT is the best examination to confirm the diagnosis. Conclusion: Isolated frontosphenoidal synostosis should be considered in patients with unilateral flattening of the forehead at birth that does not improve within the first few months of life.

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Keywords Craniosynostosis, Frontosphenoidal suture, Skull
Persistent URL dx.doi.org/10.1007/s00247-008-0750-z, hdl.handle.net/1765/29640
Citation
Mathijssen, I.M.J., van der Meulen, J.J.N.M., van Adrichem, L.N.A., Vaandrager, J.M., van der Hulst, R., Leguin, M., & Vermeij-Keers, C.. (2008). The frontosphenoidal suture: Fetal development and phenotype of its synostosis. Pediatric Radiology: roentgenology, nuclear medicine, ultrasonics, CT, MRI, 38(4), 431–437. doi:10.1007/s00247-008-0750-z