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Importance: Coats’ disease is a rare pediatric vitreoretinopathy but can cause devastating visual and anatomic outcomes. Objective: To compare optical coherence tomography (OCT) to fundus photos (FP), fluorescein angiography (FA) and histopathologic findings in Coats’ disease. Design: Retrospective cohort study. Setting: Single tertiary institution Participants: Twenty-eight children with Coats’ disease were identified through a review of medical charts from December 2002 to January 2018. Four eyes were obtained from a biorepository for histopathologic analysis. Main Outcomes and Measures: Macular OCT, FP and FA were reviewed and compared for morphological changes. These were compared to retinal histopathological findings. Results: Mean age (± SD) was 9.5 ± 5.5 years for the 28 children (and 29 eyes) with clinical imaging. A comparison between imaging modalities revealed OCT features that were not visible on photos or FA: exudates in multiple retinal layers (n=23); small pockets of subretinal fluid (n=4); outer retinal atrophy overlying fibrotic nodules (n=7); and small preretinal hyperreflective OCT dots (n=25). Next, a comparison with light micrographs helped relate OCT findings to pathological features: hyperreflective linear structures on OCT appeared consistent with cholesterol crystals, small hyper-reflective dots with macrophages, outer retinal tubulations with rosettes; and analogous OCT-histopathology features: intraretinal vessels entering fibrotic nodules, and retinal pigment epithelium excrescences under subretinal fluid. OCT analysis revealed intraretinal cystoid spaces in 19 eyes but in 9/19, this did not correlate with cystoid macular leakage, rather fluorescein leakage was observed from peripheral telangiectatic vessels. Additionally, exudates were intraretinal only (n=6) or both intraretinal and subretinal (n=17); none were subretinal only. In eyes with follow-up, new fibrosis developed in 8/17 eyes. Fibrosis developed in 5/5 eyes with baseline subretinal fluid versus 3/12 without (75% difference; 95% CI, 22%−92%) and in 7/9 eyes with subretinal exudates versus 1/8 without (65% difference; 95% CI, 16%−89%). Conclusions and Relevance: OCT may show 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 disease and may result in fibrosis formation. Further studies are needed to confirm if early treatment could prevent vision-threatening macular fibrosis.

Recent advances in anterior segment imaging have transformed the way ocular surface squamous neoplasia (OSSN) is diagnosed and monitored. Ultrasound biomicroscopy (UBM) has been reported to be useful primarily in the assessment of intraocular invasion and metastasis. In vivo confocal microscopy (IVCM) shows enlarged and irregular nuclei with hyperreflective cells in OSSN lesions and this has been found to correlate with histopathology findings. Anterior segment optical coherence tomography (AS-OCT) demonstrates thickened hyperreflective epithelium with an abrupt transition between abnormal and normal epithelium in OSSN lesions and this has also been shown to mimic histopathology findings. Although there are limitations to each of these imaging modalities, they can be useful adjunctive tools in the diagnosis of OSSN and could greatly assist the clinician in the management of OSSN patients. Nevertheless, anterior segment imaging has not replaced histopathology's role as the gold standard in confirming diagnosis.