Department of Ophthalmology, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Department of Ophthalmology, Duke University Medical Center, Durham, North Carolina.
JAMA Ophthalmol. 2019 Feb 1;137(2):176-183. doi: 10.1001/jamaophthalmol.2018.5654.
Coats disease is a rare pediatric vitreoretinopathy that can cause devastating visual and anatomic outcomes.
To compare optical coherence tomography (OCT) with fundus photographs, fluorescein angiography (FA), and histopathologic findings in Coats disease.
DESIGN, SETTING, AND PARTICIPANTS: This retrospective cohort study was conducted in a single tertiary institution (Duke Eye Center) and identified 28 children with Coats disease through a review of medical records from December 2002 to January 2018. Four eyes were obtained from a biorepository for histopathologic analysis.
Macular OCT, fundus photographs, and FA results were reviewed and compared for morphological changes. These were compared with retinal histopathological findings.
The mean (SD) age was 9.5 (5.5) years for the 28 children (and 29 eyes) with clinical imaging results, and 24 (86%) were boys. A comparison between imaging modalities revealed OCT features that were not visible in photographs or FA, including exudates in multiple retinal layers (23 [82.1%]), small pockets of subretinal fluid (4 [14.3%]), an outer retinal atrophy overlying fibrotic nodules (7 [25.0%]), and small preretinal hyperreflective OCT dots (25 [89.3%]). Next, a comparison with light micrographs introduced an association of OCT findings with possible pathological features, including hyperreflective linear structures on OCT that appeared consistent with cholesterol crystals, small hyperreflective dots with macrophages, outer retinal tubulations with rosettes, and analogous OCT histopathology features such as intraretinal vessels entering fibrotic nodules and retinal pigment epithelium excrescences under the subretinal fluid. An OCT analysis revealed intraretinal cystoid spaces in 19 eyes, but in 9 of 19 (47.4) this was not associated with cystoid macular leakage; rather, fluorescein leakage was observed from peripheral telangiectatic vessels. Additionally, exudates were intraretinal only (6 [21.4%]) or both intraretinal and subretinal (17 [60.7%]); none were subretinal only. In eyes with follow-up results, new fibrosis developed in 8 of 17 eyes (47.1%). Fibrosis developed in 5 of 5 eyes (100%) with baseline subretinal fluid vs 3 of 12 without (25%; 95% CI, 22%-92%) and in 7 of 9 eyes (77.8%) with subretinal exudates vs 1 of 8 (12.5%) without (95% CI, 16%-89%).
Optical coherence tomography may show the transient and permanent effects of Coats disease on the retina. These results suggest that exudates and fluid in the macular subretinal space appear later in the disease and may result in fibrosis formation. Further studies are needed to confirm if early treatment could prevent vision-threatening macular fibrosis.
Coats 病是一种罕见的儿科玻璃体视网膜病变，可导致严重的视力和解剖结果。
比较 Coats 病的光学相干断层扫描（OCT）与眼底照片、荧光素血管造影（FA）和组织病理学发现。
设计、地点和参与者：这项回顾性队列研究在一家单一的三级机构（杜克眼科中心）进行，通过对 2002 年 12 月至 2018 年 1 月的病历进行回顾，确定了 28 名 Coats 病患儿。从生物库中获得了 4 只眼睛进行组织病理学分析。
对黄斑 OCT、眼底照片和 FA 结果进行了回顾和比较，以观察形态变化。这些结果与视网膜组织病理学发现进行了比较。
28 名（29 只眼）具有临床成像结果的儿童的平均（SD）年龄为 9.5（5.5）岁，其中 24 名（86%）为男性。对成像方式的比较显示，OCT 具有照片或 FA 无法显示的特征，包括多层视网膜渗出（23 [82.1%]）、小袋状视网膜下液（4 [14.3%]）、纤维化结节上的外层视网膜萎缩（7 [25.0%]）和小的视网膜前高反射性 OCT 点（25 [89.3%]）。接下来，与光镜照片的比较引入了 OCT 发现与可能的病理特征之间的关联，包括 OCT 上呈胆固醇晶体状的高反射线性结构、具有巨噬细胞的小高反射点、带有罗塞特的外视网膜管腔以及类似的 OCT 组织病理学特征，如进入纤维化结节的视网膜内血管和视网膜色素上皮下的视网膜下液突起。OCT 分析显示 19 只眼中有 19 只存在视网膜内囊样空间，但在 19 只中的 9 只（47.4%）中，这与黄斑囊样漏无关；相反，荧光素渗漏是从周围毛细血管扩张中观察到的。此外，渗出物仅在视网膜内（6 [21.4%]）或视网膜内和视网膜下（17 [60.7%]）；没有一个仅在视网膜下。在有随访结果的眼中，17 只眼中（47.1%）出现新的纤维化。基线视网膜下液的 5 只眼（100%）出现纤维化，而无视网膜下液的 12 只眼（25%）中有 3 只（22%-92%），视网膜下渗出的 9 只眼（77.8%）中有 7 只（12.5%）无（95%CI，16%-89%）。
光学相干断层扫描（OCT）可能显示 Coats 病对视网膜的暂时和永久影响。这些结果表明，黄斑下视网膜空间的渗出物和液体在疾病后期出现，可能导致纤维化形成。需要进一步研究以确认早期治疗是否可以防止威胁视力的黄斑纤维化。
