Recognition of Intracranial Hypertension in Children with Craniosynostosis using Handheld OCT: A Diagnostic Accuracy Study

Craniosynostosis is often associated with intracranial hypertension (IH), which can damage the brain and vision if unaddressed. This prospective, multicentre diagnostic accuracy study evaluated the diagnostic accuracy of handheld optical coherence tomography (OCT) in recognising IH in craniosynostosis. Handheld OCT was performed in children with craniosynostosis at the time of gold-standard overnight intracranial pressure (ICP) monitoring. Mixed model regression assessed for associations with ICP. Qualitative OCT signs of IH were raised cup and rim, cup obliteration, anterior displacement of Bruch’s membrane and severe optic atrophy. Primary quantitative OCT measures were disc width, cup width, rim width, and retinal nerve fibre layer (RNFL) thickness (nasal/temporal). Of 37 eligible children, handheld OCT was successful in 36 (97%). Eighteen children (50%) were female. Eleven children (31%) had syndromic craniosynostosis. Median age at examination was 76 months (range: 14–205 months; IQR: 46–105 months). When defining IH as mean ICP≥20mmHg +/- prolonged ICP spikes, qualitative OCT sensitivity was 89% (95% CI: 65–99) and specificity was 63% (95% CI: 38–84). Anterior displacement of Bruch’s membrane delivered 100% specificity for IH. Area under the ROC curve (AUC) was 0.78 (95% CI: 0.67–0.89) for disc width, 0.81 (95% CI: 0.71–0.91;) for rim width, and 0.74 (95% CI: 0.62–0.86;) for nasal RNFL thickness; all three were significantly associated with IH (P < 0.004, P < 0.001 and P = 0.014, respectively). In conclusion, handheld OCT can recognise IH in craniosynostosis, and could improve early intervention and clinical outcomes.