Abstract

Introduction: Better diagnosis of elevated intracranial pressure (ICP) in patients with craniosynostosis may facilitate more timely intervention to improve neurocognitive outcomes. Spectral-domain optical coherence tomography (OCT) of the retinal laminae can non-invasively diagnose elevated ICP. We previously reported thresholds of maximal retinal nerve fiber layer (RNFL) thickness, retinal thickness, and anterior retinal projection measured with OCT that appeared to correlate with subacutely elevated ICP. This study sought to prospectively validate this OCT methodology in a cohort of patients with craniosynostosis. Methods: Infants and children with craniosynostosis were prospectively enrolled, and demographic, clinical, and radiographic characteristics collected. Retinal parameters were prospectively assessed in both eyes using spectral-domain OCT under general anesthesia. Subsequently, ICP was measured directly using a subdural catheter during cranial vault surgery. OCT retinal analysis was performed in a masked fashion, then compared to directly-measured ICP data. Results: Seventeen Nineteen subjects (aged 0.4-6.75.6 years) with craniosynostosis were enrolled; among these 3 (16%) were syndromic with bicoronal fusion, 8 (42%) had sagittal, 4 (21%) unicoronal, and 4 (21%) metopic suture fusion. The sample (mean age 1.9 years) included many patients with late diagnosis; 2 (67%) syndromic and 8 (50%) nonsyndromic patients had elevated ICP (>= 15mmHg.) OCT parameters (either maximal RNFL thickness or anterior retinal projection exceeding 2 standard deviations, in either eye) predicted elevated ICP in 10 patients, 7 of which were diagnosed with elevated ICP directly. This yielded OCT parameter sensitivity of 70%, and specificity of 80% when compared to the gold standard of direct ICP measurement. Sensitivity analysis of a modified protocol assessing bilateral anterior retinal projection was associated with increased specificity of 100%, but reduced sensitivity of 60%. Conclusions: Prospective assessment of a optical coherence tomography protocol to non-invasively detect elevated ICP suggests superior sensitivity and similar specificity to conventional methods in a small cohort of patients with craniosynostosis.

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