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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.

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The earlier presentation of disease in decade 2 suggests improvements in disease detection over time. Furthermore, there was a trend for eyes to have better final VA in this decade. This is due to a combination of factors, including earlier presentation of disease, fewer eyes being observed without treatment, and eyes, when treated, receiving a higher number of procedures.

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Alzheimer's disease (AD) and age-related macular degeneration (AMD) are important disorders of aging, but significant challenges remain in diagnosis and therapy. Amyloid-beta (Aβ), found in the brain and a defining feature of AD, has also been observed in the retina in both AD and AMD. While current diagnostic modalities for detecting Aβ in the brain are costly or invasive, Aβ in the retina can be noninvasively and conveniently imaged using modern photonic imaging systems such as optical coherence tomography (OCT). Moreover, since many of these retinal changes occur before degenerative changes can be detected in the brain, ocular amyloid biomarkers could be utilized to detect AD as well as AMD in their earliest stages when therapy may be most effective in halting disease progression. Novel technologies to quantify retinal biomarkers have the potential to facilitate early diagnosis and noninvasive monitoring of disease progression with important therapeutic implications.

Optical coherence tomography angiography (OCTA) is a novel, noninvasive imaging modality that allows depth-resolved imaging of the microvasculature in the retina and the choroid. It is a powerful research tool to study the pathobiology of retinal diseases, including inherited retinal dystrophies. In this review, we provide an overview of the evolution of OCTA technology, compare the specifications of various OCTA devices, and summarize key findings from published OCTA studies in inherited retinal dystrophies including retinitis pigmentosa, Stargardt disease, Best vitelliform macular dystrophy, and choroideremia. OCTA imaging has provided new data on characteristics of these conditions and has contributed to a deeper understanding of inherited retinal disease.