Ghasemi Falavarjani Khalil, Scott Adrienne W, Wang Kang, Han Ian C, Chen Xuejing, Klufas Michael, Hubschman Jean-Pierre, Schwartz Steven D, Sadda Srinivas R, Sarraf David, Tsui Irena
*Department of Ophthalmology, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California; †Eye Research Center, Rassoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran; ‡Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, Maryland; and §Greater Los Angeles VA Healthcare Center, Los Angeles, California.
Retina. 2016 Dec;36 Suppl 1:S111-S117. doi: 10.1097/IAE.0000000000001230.
To correlate macular findings on spectral domain optical coherence tomography (SDOCT) and optical coherence tomography angiography (OCTA) with quantitative ischemic index calculations on ultra-wide-field fluorescein angiography (UWFFA) in patients with sickle cell retinopathy.
In this retrospective case series, SDOCT, OCTA, and UWFFA images of patients with sickle cell retinopathy were evaluated. Eyes were staged based on the Goldberg classification of proliferative sickle cell retinopathy. Focal areas of macular thinning were assessed on SDOCT, macular vessel density was derived from OCTA, and peripheral ischemic index was calculated from UWFFA.
Eighteen eyes of 10 patients were included. Mean age was 36.8 ± 16.8 years, and 6 patients (11 eyes) were SS, 3 patients (5 eyes) were SC, and 1 patient (2 eyes) was Sβ thalassemia in hemoglobin electrophoresis. Abnormal macular findings included inner retinal atrophy in 11 eyes (61%) on SDOCT, vascular remodeling and nonperfusion in the superficial and deep retinal capillary plexus in 12 eyes (67%) on OCTA, and macular microvascular abnormalities in 9 eyes (50%) on UWFFA. Sickle cell retinopathy Stage I was identified in 4 eyes (22.2%), Stage II in 8 eyes (44.4%), and Stage III in 6 eyes (33.3%). Mean ischemic index was 14.1 ± 9.1%. Ischemic index was significantly correlated with hemoglobinopathy subtype (23.7 ± 9.8%, 9.3 ± 5.4%, and 16.3 ± 3.2%, for SC, SS, and Sβ thalassemia disease, respectively), stage of sickle cell retinopathy (22.5 ± 9.2%, 12.5 ± 4.9%, and 4.5 ± 0.73% for Stages III, II, and I, respectively), and presence of retinal thinning on SDOCT (17.4 ± 9.7% vs. 8.8 ± 5.1%, respectively).
Multimodal imaging can provide a more complete description of the microvascular and structural alterations associated with sickle retinopathy. The correlation between the severity of peripheral nonperfusion and stage and subtype of retinopathy suggests that UWF imaging may be a useful tool in the evaluation of these patients.
在镰状细胞视网膜病变患者中，将光谱域光学相干断层扫描（SDOCT）和光学相干断层扫描血管造影（OCTA）上的黄斑表现与超广角荧光素血管造影（UWFFA）上的定量缺血指数计算结果进行关联。
在这个回顾性病例系列中，对镰状细胞视网膜病变患者的SDOCT、OCTA和UWFFA图像进行了评估。根据增殖性镰状细胞视网膜病变的戈德堡分类对眼睛进行分期。在SDOCT上评估黄斑变薄的局灶区域，从OCTA得出黄斑血管密度，并从UWFFA计算周边缺血指数。
纳入了10例患者的18只眼。平均年龄为36.8±16.8岁，血红蛋白电泳显示6例患者（11只眼）为SS型，3例患者（5只眼）为SC型，1例患者（2只眼）为Sβ地中海贫血型。黄斑异常表现包括：SDOCT上11只眼（61%）出现视网膜内层萎缩；OCTA上12只眼（67%）的浅层和深层视网膜毛细血管丛出现血管重塑和无灌注；UWFFA上9只眼（50%）出现黄斑微血管异常。4只眼（22.2%）为镰状细胞视网膜病变I期，8只眼（44.4%）为II期，6只眼（33.3%）为III期。平均缺血指数为14.1±9.1%。缺血指数与血红蛋白病亚型（SC、SS和Sβ地中海贫血病分别为23.7±9.8%、9.3±5.4%和16.3±3.2%）、镰状细胞视网膜病变分期（III期、II期和I期分别为22.5±9.2%、12.5±4.9%和4.5±0.73%）以及SDOCT上视网膜变薄的存在情况（分别为17.4±9.7%和8.8±5.1%）显著相关。
多模态成像可以更全面地描述与镰状视网膜病变相关的微血管和结构改变。周边无灌注的严重程度与视网膜病变的分期和亚型之间的相关性表明，超广角成像可能是评估这些患者的有用工具。
