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The frontosphenoidal suture: fetal development and phenotype of its synostosis

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Abstract

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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Acknowledgement

We would like to thank Dries van Dam, conservator of the Museum of Anatomy of the Leiden University Medical Centre, for allowing us to use the fetal skeletons.

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Authors and Affiliations

  1. Department of Plastic and Reconstructive Surgery, Erasmus MC, University Medical Centre, Dr. Molewaterplein 60, 3015 GJ, Rotterdam, The Netherlands

    Irene M. J. Mathijssen, Jacques J. N. M. van der Meulen, Léon N. A. van Adrichem, J. Michiel Vaandrager & Christl Vermeij-Keers

  2. Department of Plastic and Reconstructive Surgery, University Hospital Maastricht, Maastricht, The Netherlands

    René R. W. J. van der Hulst

  3. Department of Radiology, Erasmus MC, University Medical Centre, Rotterdam, The Netherlands

    Maarten H. Lequin

Authors
  1. Irene M. J. Mathijssen
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  2. Jacques J. N. M. van der Meulen
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  3. Léon N. A. van Adrichem
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  4. J. Michiel Vaandrager
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  5. René R. W. J. van der Hulst
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  6. Maarten H. Lequin
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  7. Christl Vermeij-Keers
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Corresponding author

Correspondence to Irene M. J. Mathijssen.

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Mathijssen, I.M.J., van der Meulen, J.J.N.M., van Adrichem, L.N.A. et al. The frontosphenoidal suture: fetal development and phenotype of its synostosis. Pediatr Radiol 38, 431–437 (2008). https://doi.org/10.1007/s00247-008-0750-z

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  • DOI: https://doi.org/10.1007/s00247-008-0750-z

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