title: The frontosphenoidal suture: fetal development and phenotype of its synostosis.
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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Plastic and Reconstructive Surgery, Erasmus MC, University Medical Centre, Dr. Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands. i.mathijssen@erasmusmc.nl
authors.forename: Irene M J
authors.initials: IM
authors.lastname: Mathijssen
authors.forename: Jacques J N M
authors.initials: JJ
authors.lastname: van der Meulen
authors.forename: Léon N A
authors.initials: LN
authors.lastname: van Adrichem
authors.forename: J Michiel
authors.initials: JM
authors.lastname: Vaandrager
authors.forename: René R W J
authors.initials: RR
authors.lastname: van der Hulst
authors.forename: Maarten H
authors.initials: MH
authors.lastname: Lequin
authors.forename: Christl
authors.initials: C
authors.lastname: Vermeij-Keers
mesh_terms: D002102:Cadaver; D003398:Craniosynostoses / Q000000981:diagnostic imaging* / Q000601:surgery; D005260:Female; D005624:Frontal Bone / Q000033:anatomy & histology / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D010641:Phenotype; D011247:Pregnancy; D011262:Pregnancy Trimester, Second; D013100:Sphenoid Bone / Q000033:anatomy & histology / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome