Methodological determinants and prevalence of elevated intracranial pressure in isolated single-suture craniosynostosis: a systematic review and meta-analysis.

Abstract

Objective: 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.

Methods: 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.

Results: 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.

Conclusions: 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.