Intracranial Venous Hypertension in Craniosynostosis: Mechanistic Underpinnings and Therapeutic Implications

Research output: Contribution to journalReview article

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 W. Britz

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)
JournalWorld neurosurgery
DOIs
StatePublished - Jan 1 2019

PMID: 30092478

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Intracranial Venous Hypertension in Craniosynostosis : Mechanistic Underpinnings and Therapeutic Implications. / Ghali, George Zaki; Zaki Ghali, Michael George; Ghali, Emil Zaki; Srinivasan, Visish M.; Wagner, Kathryn M.; Rothermel, Alexis; Taylor, Jesse; Johnson, Jeremiah; Kan, Peter; Lam, Sandi; Britz, Gavin W.

In: World neurosurgery, 01.01.2019.

Research output: Contribution to journalReview article

Harvard

Ghali, GZ, Zaki Ghali, MG, Ghali, EZ, Srinivasan, VM, Wagner, KM, Rothermel, A, Taylor, J, Johnson, J, Kan, P, Lam, S & Britz, GW 2019, 'Intracranial Venous Hypertension in Craniosynostosis: Mechanistic Underpinnings and Therapeutic Implications' World neurosurgery. https://doi.org/10.1016/j.wneu.2018.07.260

APA

Ghali, G. Z., Zaki Ghali, M. G., Ghali, E. Z., Srinivasan, V. M., Wagner, K. M., Rothermel, A., ... Britz, G. W. (2019). Intracranial Venous Hypertension in Craniosynostosis: Mechanistic Underpinnings and Therapeutic Implications. World neurosurgery. https://doi.org/10.1016/j.wneu.2018.07.260

Vancouver

Ghali GZ, Zaki Ghali MG, Ghali EZ, Srinivasan VM, Wagner KM, Rothermel A et al. Intracranial Venous Hypertension in Craniosynostosis: Mechanistic Underpinnings and Therapeutic Implications. World neurosurgery. 2019 Jan 1. https://doi.org/10.1016/j.wneu.2018.07.260

Author

Ghali, George Zaki ; Zaki Ghali, Michael George ; Ghali, Emil Zaki ; Srinivasan, Visish M. ; Wagner, Kathryn M. ; Rothermel, Alexis ; Taylor, Jesse ; Johnson, Jeremiah ; Kan, Peter ; Lam, Sandi ; Britz, Gavin W. / Intracranial Venous Hypertension in Craniosynostosis : Mechanistic Underpinnings and Therapeutic Implications. In: World neurosurgery. 2019.

BibTeX

@article{e7356dcb0b94462b94c5c17c60ec4d28,
title = "Intracranial Venous Hypertension in Craniosynostosis: Mechanistic Underpinnings and Therapeutic Implications",
abstract = "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.",
keywords = "Craniosynostosis, Hydrocephalus, ICP, Intracranial hypertension, Jugular, Transverse sinus, Venous",
author = "Ghali, {George Zaki} and {Zaki Ghali}, {Michael George} and Ghali, {Emil Zaki} and Srinivasan, {Visish M.} and Wagner, {Kathryn M.} and Alexis Rothermel and Jesse Taylor and Jeremiah Johnson and Peter Kan and Sandi Lam and Britz, {Gavin W.}",
year = "2019",
month = "1",
day = "1",
doi = "10.1016/j.wneu.2018.07.260",
language = "English (US)",
journal = "World Neurosurgery",
issn = "1878-8750",
publisher = "Elsevier",

}

RIS

TY - JOUR

T1 - Intracranial Venous Hypertension in Craniosynostosis

T2 - World Neurosurgery

AU - Ghali, George Zaki

AU - Zaki Ghali, Michael George

AU - Ghali, Emil Zaki

AU - Srinivasan, Visish M.

AU - Wagner, Kathryn M.

AU - Rothermel, Alexis

AU - Taylor, Jesse

AU - Johnson, Jeremiah

AU - Kan, Peter

AU - Lam, Sandi

AU - Britz, Gavin W.

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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.

KW - Craniosynostosis

KW - Hydrocephalus

KW - ICP

KW - Intracranial hypertension

KW - Jugular

KW - Transverse sinus

KW - Venous

UR - http://www.scopus.com/inward/record.url?scp=85065223006&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85065223006&partnerID=8YFLogxK

U2 - 10.1016/j.wneu.2018.07.260

DO - 10.1016/j.wneu.2018.07.260

M3 - Review article

JO - World Neurosurgery

JF - World Neurosurgery

SN - 1878-8750

ER -

ID: 48692693