Advancing Non-Invasive Ophthalmic Imaging in Sturge–Weber Syndrome: Clinical Guidelines Towards Early Choroidal Hemangioma Detection

<b>Background/Objectives</b>: Sturge-Weber syndrome (SWS) is a rare neuro-oculocutaneous disorder characterized by leptomeningeal angioma, naevus flammeus, and ocular manifestations, including diffuse choroidal hemangioma (DCH). This study compares the diagnostic performance of near-infrared reflectance (NIR) imaging and enhanced depth imaging spectral-domain optical coherence tomography (EDI-SDOCT) with fundus photography in detecting DCH. <b>Methods</b>: Seventeen patients with SWS underwent comprehensive ophthalmologic evaluation, including fundus photography, NIR, and EDI-SDOCT imaging. Sensitivity, specificity, and accuracy of fundus photography, NIR, and EDI-SDOCT were calculated. <b>Results</b>: Sixteen patients had evaluable data. DCH was identified by fundus photography in five (31%), NIR in three (18.75%), and EDI-SDOCT in fourteen patients (87.50%). EDI-SDOCT alone demonstrated 100% sensitivity and 100% accuracy, outperforming both NIR (21.4% sensitivity; 31.6% accuracy) and fundus photography (35.7% sensitivity; 43.8% accuracy). When positive findings on NIR and/or SDOCT were combined, sensitivity and accuracy reached 100%. EDI-SDOCT provided detailed morphologic visualization of the choroid, allowing for early diagnosis of DCH even in pediatric cases with limited patient cooperation. <b>Conclusions</b>: EDI-SDOCT significantly improves the detection of DCH in SWS compared with fundus photography and NIR. Given its superior sensitivity and accuracy, incorporating EDI-SDOCT into routine clinical assessment may enable earlier diagnosis and reduce retinal complications in SWS.