Rajabian Firuzeh, Arrigo Alessandro, Bianco Lorenzo, Antropoli Alessio, Manitto Maria Pia, Martina Elisabetta, Bandello Francesco, Chhablani Jay, Battaglia Parodi Maurizio
Department of Ophthalmology, Vita-Salute San Raffaele University, IRCCS Ospedale San Raffaele, 20132 Milan, Italy.
Department of Ophthalmology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA.
J Clin Med. 2023 Jan 31;12(3):1095. doi: 10.3390/jcm12031095.
To report optical coherence tomography angiography (OCTA) findings in patients affected by -associated retinal dystrophies.
Patients affected by a genetically confirmed -associated retinal dystrophy were prospectively enrolled in an observational study, along with age- and sex-matched healthy volunteers as control subjects. All study and control subjects received a complete ophthalmic examination and multimodal retinal imaging, including OCTA.
A total of 12 eyes from 6 patients were included in the study. The mean BCVA of patients was 0.42 ± 0.25 logMAR. Two patients showed large central atrophy, with corresponding definite hypo-autofluorescence on fundus autofluorescence (FAF). Another four patients disclosed different degrees of RPE mottling, with uneven FAF. On OCTA, the macular deep capillary plexus and choriocapillaris had a lower vessel density in eyes affected by -associated retinopathy when compared to healthy controls. On the other hand, vessel density at the peripapillary radial capillary plexus, superficial capillary plexus, and deep capillary plexus was significantly altered with respect to control eyes. Statistical analyses disclosed a negative correlation between the deep capillary plexus and both LogMAR best corrected visual acuity and central retinal thickness.
Our study reveals that -associated retinal dystrophies are characterized by vascular alterations both in the macular and peripapillary region, as assessed by OCTA.
报告与[疾病名称]相关的视网膜营养不良患者的光学相干断层扫描血管造影（OCTA）结果。
将经基因确诊为与[疾病名称]相关的视网膜营养不良患者前瞻性纳入一项观察性研究，并纳入年龄和性别匹配的健康志愿者作为对照。所有研究对象和对照均接受了全面的眼科检查和多模式视网膜成像，包括OCTA。
该研究共纳入6例患者的12只眼。患者的平均最佳矫正视力（BCVA）为0.42±0.25 logMAR。2例患者表现为大片中央萎缩，眼底自发荧光（FAF）相应区域有明确的低自发荧光。另外4例患者表现出不同程度的视网膜色素上皮（RPE）斑驳，FAF不均匀。在OCTA上，与健康对照相比，受[疾病名称]相关视网膜病变影响的眼睛中，黄斑深层毛细血管丛和脉络膜毛细血管的血管密度较低。另一方面，视乳头周围放射状毛细血管丛、浅层毛细血管丛和深层毛细血管丛的血管密度与对照眼相比有显著改变。统计分析显示，深层毛细血管丛与LogMAR最佳矫正视力和中央视网膜厚度之间呈负相关。
我们的研究表明，通过OCTA评估，与[疾病名称]相关的视网膜营养不良的特征是黄斑和视乳头周围区域均存在血管改变。
