Intracranial Venous Hypertension in Craniosynostosis: Mechanistic Underpinnings and Therapeutic Implications

George Zaki Ghali, Michael George Zaki Ghali, Emil Zaki Ghali, Visish M. Srinivasan, Kathryn M. Wagner, Alexis Rothermel, Jesse Taylor, Jeremiah Johnson, Peter Kan, Sandi Lam, Gavin Britz

Research output: Contribution to journalReview articlepeer-review

7 Scopus citations


Patients with complex, multisutural, and syndromic craniosynostosis (CSO) frequently exhibit intracranial hypertension. The intracranial hypertension cannot be entirely attributed to the craniocephalic disproportion with calvarial restriction because cranial vault expansion has not consistently alleviated elevated intracranial pressure. Evidence has most strongly supported a multifactorial interaction, including venous hypertension along with other pathogenic processes. Patients with CSO exhibit marked venous anomalies, including stenosis of the jugular–sigmoid complex, transverse sinuses, and extensive transosseous venous collaterals. These abnormal intracranial–extracranial occipital venous collaterals might represent anomalous development, with persistence and subsequent enlargement of channels normally present in the fetus, either as a primary defect or as nonregression in response to failure of the development of the jugular–sigmoid complexes. It has been suggested by some investigators that venous hypertension in patients with CSO could be treated directly via jugular foraminoplasty, venous stenting, or jugular venous bypass, although these options are not in common clinical practice. Obstructive sleep apnea, occurring as a consequence of midface hypoplasia, can also contribute to intracranial hypertension in patients with syndromic CSO. Thus, correction of facial deformities, as well as posterior fossa decompression, could also play important roles in the treatment of intracranial hypertension. Determining the precise mechanistic underpinnings underlying intracranial hypertension in any given patient with CSO requires individualized evaluation and management.

Original languageEnglish (US)
Pages (from-to)549-558
Number of pages10
JournalWorld Neurosurgery
StatePublished - Jul 2019
Externally publishedYes


  • Craniosynostosis
  • Hydrocephalus
  • ICP
  • Intracranial hypertension
  • Jugular
  • Transverse sinus
  • Venous

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology


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