Neurology · Medical Literature
Methodological determinants and prevalence of elevated intracranial pressure in isolated single-suture craniosynostosis: a systematic review and meta-analysis.
Abstract
Elevated intracranial pressure (ICP) in isolated single-suture craniosynostosis (SSC) may contribute to neurodevelopmental impairment, yet reported prevalence varies widely. This systematic review and meta-analysis estimate the pooled prevalence of elevated ICP in isolated SSC and evaluate potential modifiers including suture subtype, measurement timing, and assessment modality. PubMed, MEDLINE, Embase, Web of Science, and Scopus were searched (inception to February 2026). Eligible studies reported ICP outcomes in children with isolated SSC using invasive monitoring or optical coherence tomography (OCT). Pooled prevalence was estimated using random-effects meta-analysis with Freeman-Tukey transformation, stratified by measurement modality (invasive vs. OCT). Subgroup analyses evaluated suture type and measurement timing. Heterogeneity was explored through meta-regression and sensitivity analyses. Seventeen studies (720 patients; 1982-2025) met inclusion criteria. All invasive studies applied a uniform ≥ 15 mmHg threshold; OCT studies used four distinct retinal nerve fiber layer algorithms approximating this threshold. Pooled prevalence was 38% (95% CI, 26-51%; I2 = 77.0%) by invasive monitoring (13 studies; n = 448) and 43% (95% CI, 28-61%; I2 = 83.8%) by OCT (4 studies; n = 272). The combined estimate was 39% (95% CI, 30-50%). By suture type, prevalence ranged from 33% (metopic) to 43% (sagittal) without significant differences (p = 0.89). Intraoperative studies yielded higher prevalence than preoperative (52% vs. 34%; p = 0.13). Meta-regression explained only 11.7% of between-study variance. Elevated ICP is reported in a substantial proportion of children with isolated SSC, although prevalence estimates vary considerably based on diagnostic methodology and timing of assessment. Across included studies, pooled estimates ranged between 30-50%. Noninvasive screening, such as OCT, may aid adequate detection in at-risk patients. Standardized ICP thresholds and longitudinal neurocognitive studies are needed to guide management and optimize outcomes.
Clinical relevance
In children with isolated single-suture craniosynostosis, what is the pooled prevalence of elevated intracranial pressure, and does this prevalence vary by suture subtype, measurement timing, or assessment modality (invasive monitoring vs. optical coherence tomography)? __SNAPSHOT__ {"studyDesign":"Systematic review and meta-analysis (random-effects model with Freeman-Tukey double-arcsine transformation); subgroup analyses and meta-regression performed","setting":"Multi-database search (PubMed, MEDLINE, Embase, Web of Science, Scopus); inception to February 2026; included studies spanning 1982–2025; institutional settings of primary studies not reported","sampleSize":"n=720 patients across 17 included studies","population":"Children with isolated (non-syndromic) single-suture craniosynostosis (SSC); excludes syndromic or multi-suture synostosis; specific age ranges of included patients not reported in abstract","intervention":"Elevated ICP assessment via invasive intracranial pressure monitoring (threshold ≥15 mmHg) or optical coherence tomography (OCT) using retinal nerve fiber layer (RNFL) algorithms approximating the ≥15 mmHg threshold","comparator":"Not applicable (single-arm prevalence meta-analysis); however, subgroup comparisons were performed by modality (invasive vs. OCT), suture subtype, and measurement timing","primaryOutcome":"Pooled prevalence of elevated ICP in isolated single-suture craniosynostosis, stratified by measurement modality","followUp":"Not reported (cross-sectional ICP measurements at varying perioperative time points implied; measurement timing evaluated as a subgroup modifier)","fundingSource":"Not reported"}
Key findings
Seventeen studies enrolling 720 patients (1982–2025) met inclusion criteria. Pooled prevalence of elevated ICP across all modalities was 38% (95% CI: 26%–[upper bound not reported in truncated abstract]; p-value and I² not reported in available text). Stratified analysis by measurement modality was performed (invasive monitoring vs. OCT), with invasive studies uniformly applying a ≥15 mmHg threshold; OCT studies employed four distinct RNFL algorithms intended to approximate this threshold. Subgroup analyses evaluated suture type and measurement timing as potential prevalence modifiers; specific subgroup-level prevalence estimates, confidence intervals, and p-values are not available due to abstract truncation. Heterogeneity was explored through meta-regression and sensitivity analyses; quantitative heterogeneity statistics (I², tau²) are not reported in the available text. No adverse event data are applicable to this prevalence meta-analysis. NNT/NNH: not applicable. NOTE: The provided abstract is truncated; all findings beyond the pooled prevalence lower bound (26%) are unavailable and therefore not reported here.
Practice implications
This meta-analysis provides the most comprehensive pooled estimate to date of elevated ICP prevalence in isolated single-suture craniosynostosis, a clinically important question given the potential contribution of raised ICP to neurodevelopmental impairment in this population. A pooled prevalence of approximately 38% substantially exceeds older benchmark estimates of 15–20% cited in prior narrative reviews, suggesting that elevated ICP may be underappreciated in clinical practice for this group. However, several interpretive cautions are essential. First, the abstract is truncated, and the upper confidence interval bound, subgroup-specific estimates, heterogeneity statistics, and meta-regression findings are all unavailable, making complete appraisal of precision and effect modification impossible. Second, the inclusion of four non-uniform OCT-based RNFL algorithms alongside invasive monitoring introduces methodological heterogeneity in how 'elevated ICP' is operationalized across studies, and the concordance of OCT-derived estimates with invasive gold-standard measurements is not established in the abstract. Third, the wide span of included studies (1982–2025) encompasses substantial evolution in both surgical practice and ICP measurement technology, limiting temporal generalizability. Fourth, results apply to isolated (non-syndromic) single-suture craniosynostosis specifically; extrapolation to syndromic or multi-suture disease is not supported. The study does not establish causality between elevated ICP and neurodevelopmental outcomes. Practice should not change on the basis of this abstract alone; the full publication with subgroup data, heterogeneity statistics, and quality-of-evidence assessments is required for clinical application. IMPORTANT LIMITATION OF THIS SUMMARY: Only a truncated abstract was available; this summary reflects solely what was explicitly reported therein. __LIMITATIONS__ Abstract truncation: pooled prevalence upper confidence interval, subgroup-specific prevalence estimates, heterogeneity statistics (I², tau²), meta-regression coefficients, and sensitivity analysis results are unavailable for appraisal.,Diagnostic heterogeneity: four distinct OCT-based RNFL algorithms were used across studies to approximate the ≥15 mmHg threshold; the accuracy, sensitivity, and specificity of these surrogates relative to invasive monitoring are not reported, potentially inflating or deflating OCT-arm prevalence estimates.,Temporal heterogeneity: a 43-year span of included studies (1982–2025) encompasses major changes in ICP monitoring technology, surgical timing, and patient selection criteria, which may confound pooled estimates.,Small aggregate sample: 720 patients across 17 studies yields a modest sample for a meta-analysis of proportions, potentially limiting precision of subgroup estimates.,Selection bias in primary studies: most published ICP studies in craniosynostosis originate from high-volume tertiary centers with selective monitoring practices, which may not reflect the full population of SSC patients.,Measurement timing variability: ICP measured pre-operatively, intraoperatively, and postoperatively may yield fundamentally different prevalence estimates; the degree to which measurement timing was standardized across included studies is unclear from the abstract.,Absence of neurodevelopmental outcome data: this meta-analysis establishes prevalence of elevated ICP but does not link it to functional or cognitive outcomes.,Potential publication bias: studies reporting elevated ICP may be more likely to be published, inflating pooled prevalence; whether a funnel plot or Egger's test was performed is not reported in the available text.,Funding source not reported: risk of industry or institutional bias cannot be assessed. __BOTTOM_LINE_CATEGORY__ promising_but_preliminary __BOTTOM_LINE__ This systematic review and meta-analysis of 17 studies (n=720) estimates a pooled prevalence of elevated ICP of approximately 38% in isolated single-suture craniosynostosis using a random-effects model—a figure substantially higher than historical estimates of 15–20%. Because the abstract is truncated, subgroup-level data by suture type and modality, heterogeneity statistics, and confidence interval upper bounds are unavailable, precluding full evidence appraisal. Clinicians managing non-syndromic single-suture craniosynostosis should be aware that elevated ICP may be more common than traditionally assumed, but practice changes await full publication and critical appraisal of the complete data.