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Updated: Jul 22, 2025

A Detailed Protocol for Physiological Parameters Acquisition and Analysis in Neurosurgical Critical Patients
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What We Know About Intracranial Hypertension in Children With Syndromic Craniosynostosis

Priya N Doerga1, Robbin de Goederen1, Marie-Lise C van Veelen2

  • 1Sophia Children's Hospital, Dutch Craniofacial Center, Department of Plastic and Reconstructive Surgery and Hand Surgery, Erasmus MC, University Medical Center.

The Journal of Craniofacial Surgery
|July 24, 2023
PubMed

Insights

Intracranial hypertension in syndromic craniosynostosis is multifactorial. Cognitive and behavioral issues in these patients are more likely linked to genetic factors than intracranial hypertension.

Area of Science:

  • Neuroscience
  • Genetics
  • Pediatric Surgery

Background:

  • Syndromic craniosynostosis (sCS) involves premature fusion of skull sutures, potentially leading to intracranial hypertension (ICH).
  • Understanding the mechanisms of ICH and its impact on neurodevelopmental outcomes in sCS is crucial.

Purpose of the Study:

  • To conduct a scoping review on the mechanisms of ICH in sCS.
  • To explore whether cognitive and behavioral outcomes in sCS are primarily due to genetic factors or ICH.

Main Methods:

  • A comprehensive literature search was performed across multiple databases (EMBASE, MEDLINE, Web of Science, Cochrane, Google Scholar).
  • Selected articles underwent full-text review and data extraction.
  • Analysis focused on ICH determination criteria and the Monro-Kellie doctrine components (cerebral blood volume, CSF, intracranial volume).

Main Results:

  • Ninety full-text articles were analyzed from 1893 search results.
  • Invasive intracranial pressure measurement is the gold standard for ICH diagnosis; ophthalmologic methods are the most researched noninvasive alternatives.
  • The Monro-Kellie doctrine helps explain ICH development in sCS.

Conclusions:

  • ICH in sCS arises from factors including skull growth deflection, sleep apnea, venous hypertension, and CSF flow/absorption issues.
  • Cognitive and behavioral deficits in sCS are more strongly associated with genetic origins.
  • Cortical thinning and visual impairments may result from ICH.
Abstract

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