title: Venous hypertension in syndromic and complex craniosynostosis: the abnormal anatomy of the jugular foramen and collaterals.
abstract: Why craniosynostosis patients develop elevated intracranial pressure (ICP) is still a mystery. Our aim was to investigate jugular foramen size and its relation to venous hypertension and elevated ICP. Secondly, we evaluated whether occipital collateral veins develop as a compensatory mechanism for elevated ICP. We conducted a prospective study in 41 children with craniosynostosis who underwent a 3D-CT-angiography. We evaluated the anatomical course of the jugular vein, the diameter of the jugular foramen and the relation to the presence of papilledema. Additionally, we studied the anatomical variations of the cerebral venous drainage system. The diameter of the jugular foramen was significantly smaller in our patients. Abnormal venous collaterals were most often observed in patients with Apert, Crouzon-Pfeiffer and Saethre-Chotzen syndrome, even in children under two years of age. There was no significant difference in the number of collateral veins in patients with or without papilledema. Collaterals appear to reflect an inborn abnormality of the venous system, rather than a compensating mechanism for elevated ICP. This study confirms the presence of jugular foraminal narrowing in craniosynostosis patients and an abnormal venous system, which may predispose to elevated ICP.
  
  
  METHODS
  Diagnostic II.
authors.affiliation: Department of Plastic and Reconstructive Surgery and Hand Surgery, Craniofacial Center The Netherlands, Erasmus Medical Center Sophia Children's Hospital, Rotterdam, The Netherlands. Electronic address: j.florisson@erasmusmc.nl.
authors.forename: Joyce M G
authors.initials: JM
authors.lastname: Florisson
authors.affiliation: Department of Radiology, Craniofacial Center The Netherlands, Erasmus Medical Center Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Georgios
authors.initials: G
authors.lastname: Barmpalios
authors.affiliation: Department of Radiology, Craniofacial Center The Netherlands, Erasmus Medical Center Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Maarten
authors.initials: M
authors.lastname: Lequin
authors.affiliation: Department of Neurosurgery, Craniofacial Center The Netherlands, Erasmus Medical Center Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Marie-Lise C
authors.initials: ML
authors.lastname: van Veelen
authors.affiliation: Department of Pediatrics, Craniofacial Center The Netherlands, Erasmus Medical Center Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Natalja
authors.initials: N
authors.lastname: Bannink
authors.affiliation: Department of Neurosurgery, Great Ormond Street Hospital for Children, London, England, UK.
authors.forename: Richard D
authors.initials: RD
authors.lastname: Hayward
authors.affiliation: Department of Plastic and Reconstructive Surgery and Hand Surgery, Craniofacial Center The Netherlands, Erasmus Medical Center Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Irene M J
authors.initials: IM
authors.lastname: Mathijssen
mesh_terms: D000168:Acrocephalosyndactylia / Q000150:complications / Q000503:physiopathology; D000293:Adolescent; D002533:Cerebral Angiography / Q000379:methods; D002550:Cerebral Veins / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D003097:Collateral Circulation / Q000502:physiology; D003392:Cranial Sinuses / Q000000981:diagnostic imaging; D003398:Craniosynostoses / Q000150:complications* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted / Q000379:methods; D021621:Imaging, Three-Dimensional / Q000379:methods; D007223:Infant; D019586:Intracranial Hypertension / Q000209:etiology*; D007601:Jugular Veins / Q000000981:diagnostic imaging*; D008297:Male; D009777:Occipital Bone / Q000000981:diagnostic imaging*; D010211:Papilledema / Q000209:etiology; D011446:Prospective Studies; D013701:Temporal Bone / Q000000981:diagnostic imaging*; D014057:Tomography, X-Ray Computed / Q000379:methods
keywords: Craniosynostosis; Intracranial pressure; Jugular foramen