Faculty Bibliography

PURPOSE: To evaluate and characterize multiple evanescent white dot syndrome abnormalities with modern multimodal imaging modalities. METHODS: This retrospective cohort study evaluated fundus photography, fluorescein angiography, indocyanine green angiography, optical coherence tomography, enhanced depth imaging optical coherence tomography, short-wavelength autofluorescence, and near-infrared autofluorescence. RESULTS: Thirty-four multiple evanescent white dot syndrome patients with mean age of 28.7 years were studied (range, 14-49 years). Twenty-six patients were women, and eight were men. Initial mean visual acuity was 0.41 logMAR. Final mean visual acuity was 0.03 logMAR. Fluorescein angiography shows a variable number of mid retinal early fluorescent dots distributed in a wreathlike pattern, which correlate to fundus photography, fundus autofluorescence, and indocyanine green angiography. Indocyanine green angiography imaging shows the dots and also hypofluorescent, deeper, and larger spots, which are occasionally confluent, demonstrating a large plaque of deep retinal hypofluorescence. Optical coherence tomography imaging shows multifocal debris centered at and around the ellipsoid layer, corresponding to the location of spots seen with photography, indocyanine green angiography, and fluorescein angiography. Protrusions of the hyperreflectant material from the ellipsoid layer toward the outer nuclear layer correspond to the location of dots seen with photography, indocyanine green angiography, and fluorescein angiography. CONCLUSION: Multimodal imaging analysis of the retina in patients with multiple evanescent white dot syndrome shows additional features that may help in the diagnosis of the disease and in further understanding its etiology. Multiple evanescent white dot syndrome is predominantly a disease of the outer retina, centered at the ellipsoid zone, but also involving the interdigitation zone and the outer nuclear layer.

Importance: The Diabetic Retinopathy Clinical Research Network (DRCR Network), sponsored by the National Eye Institute, reported the results of a comparative effectiveness randomized clinical trial (RCT) evaluating the 3 anti-vascular endothelial growth factor (anti-VEGF) agents aflibercept (2.0 mg), bevacizumab (1.25 mg), and ranibizumab (0.3 mg) for treatment of diabetic macular edema (DME) involving the center of the retina and associated with visual acuity loss. The many important findings of the RCT prompted the American Society of Retina Specialists to convene a group of experts to provide their perspective regarding clinically relevant findings of the study. Objectives: To describe specific outcomes of the RCT judged worthy of highlighting, to discuss how these and other clinically relevant results should be considered by specialists treating DME, and to identify unanswered questions that merit consideration before treatment. Evidence Review: The DRCR Network-authored publication on primary outcomes of the comparative effectiveness RCT at 89 sites in the United States. The study period of the RCT was August 22, 2012, to August 28, 2013. Findings: On average, all 3 anti-VEGF agents led to improved visual acuity in eyes with DME involving the center of the retina and with visual acuity impairment, including mean (SD) improvements by +13.3 (11.1) letters with aflibercept vs +9.7 (10.1) letters with bevacizumab (P < .001) and +11.2 (9.4) letters with ranibizumab (P = .03). Worse visual acuity when initiating therapy was associated with greater visual acuity benefit of aflibercept (+18.9 [11.5]) over bevacizumab (+11.8 [12.0]) or ranibizumab (14.2 [10.6]) 1 year later (P < .001 for interaction with visual acuity as a continuous variable, and P = .002 for interaction with visual acuity as a categorical variable). It is unknown whether different visual acuity outcomes associated with the use of the 3 anti-VEGF agents would be noted with other treatment regimens or with adequately repackaged bevacizumab, as well as in patients with criteria that excluded them from the RCT, such as persistent DME despite recent anti-VEGF treatment. Conclusions and Relevance: On average, all 3 anti-VEGF agents led to improved visual acuity in eyes with DME involving the center of the retina and visual acuity impairment. Worse visual acuity when initiating therapy was associated with greater visual acuity benefit of aflibercept over bevacizumab or ranibizumab 1 year later. Care needs to be taken when attempting to extrapolate outcomes of this RCT to differing treatment regimens. With access to adequately repackaged bevacizumab, many specialists might initiate therapy with bevacizumab when visual acuity is good (ie, 20/32 to 20/40 as measured in the DRCR Network), recognizing that the cost-effectiveness of bevacizumab outweighs that of aflibercept or ranibizumab.

BACKGROUND AND OBJECTIVE: To evaluate choroidal involvement in acute posterior multifocal placoid pigment epitheliopathy (APMPPE). PATIENTS AND METHODS: Retrospective study in five eyes of three patients evaluated through multimodal imaging, including enhanced-depth imaging optical coherence tomography (OCT), ultra-wide field color photography, fundus autofluorescence, and fluorescein angiography (FA). Choroidal thickness and structure were evaluated on OCT. RESULTS: During the acute phase, choroidal OCT showed choroidal thickening and a lucency at the level of the inner choroid. Subclinical lesions detected in the retinal periphery using wide-field retinal imaging were isoautofluorescent and corresponded to choriocapillaris filling-defects on FA. At final follow-up, all patients showed resolution of choroidal thickening and the inner choroidal lucency, as well as the disappearance of subclinical lesions. CONCLUSION: These results suggest a transient ischemic choroiditis in APMPPE that may lead to secondary permanent retinal pigment epithelium damage in the posterior pole but not in the retinal periphery. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:20-26.].

PURPOSE: To describe a patient who showed progression of age-related macular degeneration along the course of an underlying large choroidal vessel. METHODS: Retrospective case report. RESULTS: An 84-year-old woman was diagnosed with age-related macular degeneration and followed for more than 6 years. Retinal pigment epithelial changes were more advanced in her right eye which had a thinner choroid than the fellow left eye. Multimodal imaging with swept-source optical coherence tomography showed that the progression of outer retinal and retinal pigment epithelial changes appeared to follow the course of a large choroidal vessel. CONCLUSION: The authors hypothesize that large choroidal vessels can compress the overlying choriocapillaris, possibly leading to outer retinal ischemia, which subsequently could focally accelerate the course of age-related macular degeneration.

PURPOSE: To report a posterior segment phenotypic manifestation of Coxsackie virus infection that has not been previously appreciated. METHODS: The clinical course and multimodal imaging findings, including spectral domain optical coherence tomography, fluorescein angiography, near infrared reflectance, and fundus autofluorescence of two patients with Coxsackie virus infections were documented. RESULTS: A neurosensory macular detachment was present in both patients on baseline examination. Fluorescein angiography demonstrated pooling within this lesion and spectral domain optical coherence tomography identified thickening of the retinal pigment epithelial band with variable degrees of attenuation of the ellipsoid zone and interdigitation zone. Fundus autofluorescence and near infrared reflectance imaging revealed multiple satellite lesions adjacent to the neurosensory detachment. These lesions were not seen on fluorescein angiography or color photography. Satellite lesions were hyporeflective on near infrared reflectance imaging and hyperautofluorescent on fundus autofluorescence imaging. The satellite lesions correlated with sites of ellipsoid disruption on spectral domain optical coherence tomography. Both patients were observed and their condition improved over the course of time. There was total resolution of satellite lesions, reconstitution of the ellipsoid zone and interdigitation zone, and return of retinal pigment epithelial thickness to the normal range. A bull's eye pattern of macular retinal pigment epithelial disturbance persisted on color and near infrared reflectance images, despite good visual acuity. CONCLUSION: Posterior segment Coxsackie virus infection may concurrently express the clinical characteristics of acute idiopathic maculopathy and multifocal retinitis. The visual prognosis in this variant is usually favorable. The multimodal imaging features that characterize this entity should be recognized to avoid confusion with other diseases that have a similar presentation.

PURPOSE: To report a case of focal choroidal excavation and choroidal neovascularization in the setting of pachychoroid disease. METHODS: Multimodal imaging, including fundus photography, fundus autofluorescence, enhanced depth imaging optical coherence tomography, indocyanine green angiography, and en face structural and angiographic optical coherence tomography. RESULTS: The authors describe a 39-year-old female with moderately high myopia presenting with focal choroidal excavation and associated choroidal neovascularization. Multimodal imaging demonstrated pachychoroid features with dilated choroidal vessels surrounding the lesion. Optical coherence tomography angiography showed Type 2 neovascularization. CONCLUSION: Some cases of focal choroidal excavation and associated neovascularization may be related to structural abnormalities of the associated choroidal vasculature.

BACKGROUND: Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare, paraneoplastic syndrome characterized by bilateral painless visual loss and proliferation of choroidal melanocytes in association with an underlying systemic malignancy. We report a case of bilateral diffuse uveal melanocytic proliferation associated with an underlying gynecological malignancy that also features the infrequent finding of an iris mass lesion, using multimodal imaging including ultra-widefield imaging, spectral domain and swept-source optical coherence tomography. CASE PRESENTATION: A 59-year-old white female with a prior history of gynecological malignancy in remission presented with progressive bilateral visual loss over several weeks. The patient was noted to have a focal iris mass lesion in her right eye. Ultra-widefield color fundus photography showed a characteristic bilateral 'giraffe pattern' of pigmentary changes extending into the periphery as well as multiple discrete deeply pigmented lesions. Ultra-widefield autofluorescence was useful for visualizing the full extent of involvement. Indocyanine green angiography helped to demarcate the discrete pigmented choroidal lesions. Swept-source OCT clearly delineated the alternating zones of retinal pigment epithelium (RPE) thickening and RPE loss, as well as the prominent choroidal infiltration and thickening. CONCLUSIONS: BDUMP is an important diagnosis to consider in the presence of multiple discrete melanocytic choroidal lesions, diffuse choroidal thickening, characteristic RPE changes, iris mass lesions and exudative retinal detachment. Ultra-widefield imaging may demonstrate more extensive lesions than that detected on clinical examination or standard field imaging. Imaging with SS-OCT shows choroidal and RPE characteristics that correlate well with known histopathology of this entity.