Bazvand Fatemeh, Khojasteh Hasan, Zarei Mohammad
Retina Service, Farabi Eye Hospital, Tehran University of Medical Sciences, Qazvin Square, Tehran, Iran.
Doc Ophthalmol. 2019 Dec;139(3):221-226. doi: 10.1007/s10633-019-09706-6. Epub 2019 Jul 10.
To describe a novel association of enhanced S-cone syndrome (ESCS) with macular retinal neovascularization and severe retinal vasculitis.
Clinical examination, spectral domain optical coherence tomography, fluorescein angiography, fundus autofluorescence, infrared reflectance and electroretinography were used to study a 25-year-old male with a history of night blindness from early childhood and recent accelerated visual loss in right eye.
Pigmented lesions were observed along the arcades without peripheral retinal involvement. Intraretinal cystoid spaces, retinal neovascularization of posterior pole and severe peripheral and posterior retinal vasculitis were found on clinical examination and multimodal imaging. Based on characteristic clinical and electroretinographic findings, a diagnosis of ESCS was made.
This case highlights novel associations of retinal neovascularization and vasculitis with ESCS.
描述一种增强型S锥体综合征（ESCS）与黄斑视网膜新生血管形成及严重视网膜血管炎的新型关联。
采用临床检查、光谱域光学相干断层扫描、荧光素血管造影、眼底自发荧光、红外反射和视网膜电图对一名25岁男性进行研究，该男性自幼有夜盲病史，近期右眼视力加速下降。
沿视网膜血管弓观察到色素沉着病变，不累及周边视网膜。临床检查和多模态成像发现视网膜内囊样间隙、后极部视网膜新生血管形成以及严重的周边和后部视网膜血管炎。根据特征性的临床和视网膜电图表现，诊断为ESCS。
该病例突出了视网膜新生血管形成和血管炎与ESCS的新型关联。
