Investigation of Cranial Bone Changes Indicative of Increased Intracranial Pressure in Diverse Phenotypes of Craniosynostosis.

Abstract

Background: Despite the clinical importance of identifying increased intracranial pressure (IIP) in children with craniosynostosis (CS), its presence is often uncertain due to limited utilization of invasive measurement methods, inconclusive clinical evaluations, and its variability depending on the CS phenotype. Hence, prevalence reports are highly variable. We previously developed a computational method to identify pediatric chronic IIP of diverse etiology based on subtle cranial thickness and density anomalies quantified from computed tomography (CT) scans. In this study, we evaluate cranial signs of IIP in a large dataset of presurgical CT scans of patients with diverse phenotypes of CS and its prevalence.

Methods: We quantified local cranial thickness and density in the CT scans of 417 patients with diverse phenotypes of CS (age 0-2 y). We used a normative reference of cranial development to quantify cranial bone anomalies in each phenotypic group and compared them with 48 patients with chronic IIP unrelated to CS. We then studied the risk of IIP and its prevalence in each phenotypic group of CS.

Results: Patients with CS presented significant calvarial thickening and bone density decrease compared with normative patients (P < 0.001). Similar findings were found in patients with chronic IIP unrelated to CS (P > 0.23). Presurgical signs of IIP were more prevalent in patients with Apert syndrome (>74% patients) and nonsyndromic patients with coronal involvement (>30%) compared with other phenotypes (>18%).

Conclusions: Computational evaluation of routinely acquired presurgical CT scans can potentially support the evaluation of IIP in patients with CS.