The frontosphenoidal suture: fetal development and phenotype of its synostosis
Irene M J Mathijssen1, Jacques J N M van der Meulen, Léon N A van Adrichem
1Department of Plastic and Reconstructive Surgery, Erasmus MC, University Medical Centre, Dr. Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands. i.mathijssen@erasmusmc.nl
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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