Abstract

Introduction: Detecting elevated intracranial pressure (ICP) in craniosynostosis patients may enable timely intervention to prevent neurocognitive impairment, but is challenging with conventional methods. This study employed optical Coherence tomography (OCT) to non-invasively quantify retinal laminae, and sought to determine patterns of elevated ICP by craniosynostosis type. Methods: Elevated ICP (≥15 mmHg) was prospectively assessed using peri-operative bilateral OCT. ICP was considered elevated if retinal nerve fiber layer (RNFL) max thickness >208microns, or max retinal inner projection > 159 microns in either eye, thresholds established by a previous study correlating OCT findings with directly measured ICP with high sensitivity and specificity. Univariate and multivariate analysis of OCT findings and patient and surgical characteristics was performed. Results: 129 subjects aged 0.1–17.1 years were enrolled at time of initial surgical cranial vault expansion, 100 (78%) of which had single-suture craniosynostosis. Patients with single-suture synostosis were less likely nto have OCT-findings suggestive of elevated ICP (39, 39%) than those with multi-suture involvement (18, 62%; p=0.031). Patients with syndromic diagnoses were similarly likely to have elevated ICP (17, 59%; p=0.033.) Among patients with single-suture synostosis, those with metopic suture involvement trended toward less likelihood of elevated ICP (6, 23%) compared to those with unicoronal (4, 36%), sagittal (13, 36%), or lambdoid (3, 60%) suture involvement (p=0.062.) In a multivariate logistic regression, multi-suture craniosynostosis was significantly associated with OCT-detected elevated ICP (OR = 2.789, 95% CI = [1.245, 5.991], p=0.013). There was substantial inter-eye reliability (k=0.616, 95% CI = [0.462, 0.770], p<0.001). Conclusion: Elevated ICP should be suspected in patients with syndromic or multi-suture craniosynostosis. OCT shows promise as a non-invasive diagnostic modality.

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