Ciancimino Chiara, Di Pippo Mariachiara, Rullo Daria, Ruggeri Francesco, Grassi Flaminia, Scuderi Gianluca, Abdolrahimzadeh Solmaz
Ophthalmology Unit, Neurosciences, Mental Health, and Sense Organs (@NESMOS) Department, Faculty of Medicine and Psychology, University of Rome Sapienza, St. Andrea Hospital, 00189 Rome, Italy.
Vision (Basel). 2023 Oct 6;7(4):64. doi: 10.3390/vision7040064.
Sturge-Weber syndrome (SWS) is characterized by facial port-wine stains, leptomeningeal hemangiomas, and prominent ocular manifestations such as glaucoma and diffuse choroidal hemangiomas (DCHs). Imaging modalities are critical for diagnosing and longitudinally monitoring DCHs in SWS. Fundus photography is fundamental in assessing both eyes simultaneously, fluorescein angiography and indocyanine green angiography effectively map the retinal and choroidal circulation, and ultrasonography offers essential structural insights into the choroid and retina. NIR imaging reveals subtle retinal pigment changes, often overlooked in standard fundus examination. Enhanced depth imaging spectral domain optical coherence tomography (EDI-SDOCT) and swept-source OCT (SSOCT) improve the visualization of the choroidal-scleral boundary, essential for DCH characterization. The potential of OCT angiography (OCTA) is under exploration, particularly its role in predicting signs of disease progression or worsening, as well as potential new biomarkers such as the choroidal vascularity index (CVI). The present review aims to provide an update on multimodal imaging of DCHs in SWS.
斯特奇-韦伯综合征（SWS）的特征为面部葡萄酒色斑、软脑膜血管瘤以及明显的眼部表现，如青光眼和弥漫性脉络膜血管瘤（DCH）。成像方式对于诊断和纵向监测SWS中的DCH至关重要。眼底照相术对于同时评估双眼至关重要，荧光素血管造影和吲哚菁绿血管造影可有效描绘视网膜和脉络膜循环，超声检查可提供有关脉络膜和视网膜的重要结构信息。近红外成像可揭示标准眼底检查中经常被忽视的细微视网膜色素变化。增强深度成像光谱域光学相干断层扫描（EDI-SDOCT）和扫频源光学相干断层扫描（SSOCT）可改善脉络膜-巩膜边界的可视化，这对于DCH的特征描述至关重要。光学相干断层扫描血管造影（OCTA）的潜力正在探索中，特别是其在预测疾病进展或恶化迹象以及潜在新生物标志物（如脉络膜血管指数（CVI））方面的作用。本综述旨在提供SWS中DCH多模态成像的最新进展。
