Faculty Bibliography

AIM/OBJECTIVE:To report the discordance in central serous chorioretinopathy (CSCR) classification among practising retina specialists. METHODS:The study conducted was a multicentre survey. Multimodal retinal images along with relevant clinical details of 100 cases diagnosed as CSCR (from six centres) were circulated among six retina specialists across the globe. The image sets included colour fundus photographs, fundus autofluorescence images, optical coherence tomography b-scans, fluorescein and indocyanine green angiography of the study and fellow eyes. The graders were asked to classify the disease of study eye, according to their own criteria. The graders were masked to the responses of other graders. The final analysis of the pooled response data was done based on the diagnosis of study eye only. The main outcome measure was degree of agreement between six independent observers using Fleiss Kappa statistics. RESULTS:Grading for 100 eyes of 100 patients (men, 93%) was included in the analysis. 20 patients had a history of steroid use. The graders provided 36 different terms to classify the disease, with poor agreement among graders (Fleiss Kappa=0.134). The consistency in diagnosing acute CSCR was statistically higher than for either chronic (p=0.012) or recurrent CSCR (p<0.0001). When collapsing descriptors into six main terms, agreement remained poor (Fleiss Kappa=0.218). CONCLUSION/CONCLUSIONS:The high discordance among experienced retina specialists in describing CSCR clinical subtypes is highlighted. The current work demonstrates the limitations of current empirical CSCR classification methods and the need for a more objective and refined system to bring uniformity in diagnosis and prognostication of the disease.

Pachychoroid is a relatively novel concept describing a phenotype characterized by attenuation of the choriocapillaris overlying dilated choroidal veins, and associated with progressive retinal pigment epithelium dysfunction and neovascularization. The emphasis in defining pachychoroid-related disorders has shifted away from simply an abnormally thick choroid (pachychoroid) toward a detailed morphological definition of a pathologic state (pachychoroid disease) with functional implications, which will be discussed in this review. Several clinical manifestations have been described to reside within the pachychoroid disease spectrum, including central serous chorioretinopathy, pachychoroid pigment epitheliopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy/aneurysmal type 1 neovascularization, focal choroidal excavation, peripapillary pachychoroid syndrome. These conditions all exhibit the characteristic choroidal alterations and are believed to represent different manifestations of a common pathogenic process. This review is based on both the current literature and the clinical experience of our individual authors, with an emphasis on the clinical and imaging features, management considerations, as well as current understanding of pathogenesis of these disorders within the context of the recent findings related to pachychoroid disease.

BACKGROUND:To investigate the long-term visual and optical coherence tomography (OCT) anatomical outcomes of type 3 neovascularisation (NV) and to identify any baseline predictors of poor outcomes. METHODS:In this retrospective study, patients diagnosed with treatment naïve type 3 NV were identified and categorised into two groups: good or poor vision based on final vision at 1 year. Baseline demographic features and visual acuity (VA) and baseline and 1-year spectral domain OCT (SD-OCT) anatomical findings were studied and correlated with good versus poor visual outcomes. RESULTS:Ten of 25 eyes were classified as having a poor visual outcome (20/50 or worse) at 1 year. Increased age (P=0.049), male gender (p=0.041) and worse baseline VA (ρs=0.61, p=0.001) were associated with poor vision at 1 year. Greater foveal atrophy was noted at 1 year in the poor visual outcome group (p=0.030). Subretinal hyper-reflective material and choroidal thinning were additional features noted more commonly in this group. CONCLUSION/CONCLUSIONS:Increased age, male gender and lower baseline vision may be important baseline predictors of poor visual outcomes in eyes with type 3 NV. The development of central outer retinal atrophy and fibrosis, as identified with SD-OCT, may limit long-term vision in eyes with type 3 NV.

PURPOSE: To describe the multimodal imaging findings of transient subretinal deposits occurring in multiple evanescent white dot syndrome (MEWDS). METHODS: The multimodal imaging characteristics of transient subretinal deposits occurring in MEWDS were investigated with ultra-widefield color and fundus autofluorescence, cross-sectional and en-face optical coherence tomography (OCT), en face OCT-angiography, and quantitative autofluorescence. RESULTS: A 28-year-old woman presented with photopsia and temporal visual field loss in her right eye. Her best-corrected visual acuity was 20/20 in her right eye and 20/25 in her left eye. Funduscopic examination showed characteristic peripapillary hyperautofluorescent white dots of MEWDS corresponding to ellipsoid zone disruption on OCT. These lesions became confluent throughout the posterior fundus over the next 4 weeks. As the patient's symptoms were resolving, a second type of transient hyperautofluorescent lesion was noted which corresponded to hyperreflective subretinal deposits on cross-sectional and en face structural OCT. These subretinal deposits were most evident at 10-week follow-up and had nearly resolved at 14-week follow-up. Quantitative autofluorescence showed that, unlike the acute MEWDS lesions, the hyperautoflurescence of the subretinal deposits persisted after photobleaching. At multiple time points over 14 weeks of follow-up, OCT angiography showed no evidence of retinal or choroidal flow abnormalities. CONCLUSION: Transient subretinal deposits may develop during MEWDS in areas of previous diffuse outer retinal disruption. As these deposits remain hyperautoflurescent on quantitative autofluorescence after photobleaching, they may represent accumulations of debris originating from damaged photoreceptor outer segments.

PURPOSE: To report a case of "posterior scleral melanocytosis," a pigmented lesion of the posterior sclera that clinically resembles a flat choroidal nevus. METHODS: Case report of a patient with posterior scleral melanocytosis. Multimodal imaging, including swept source optical coherence tomography, was used to demonstrate the scleral location of the pigmented lesion and to distinguish its features from a typical choroidal nevus present in the same eye. RESULTS: An 86-year-old woman was seen for regular follow-up for neovascular age-related macular degeneration in her right eye and 2 pigmented lesions in her left eye, both presumed to be choroidal nevi. Anterior segment examination showed no evidence of ocular or dermal melanocytosis. Optical coherence tomography of the pigmented lesion in the left eye showed two distinct patterns. One lesion showed hyperreflectivity within the choroidal tissue associated with posterior shadowing, whereas the second lesion showed normal choroidal reflectivity with hyperreflectivity confined to the inner sclera associated with marked posterior shadowing. CONCLUSION: To the authors' knowledge, this is the first report of posterior scleral melanocytosis, a pigmented fundus lesion confined to the inner sclera. The need for high-penetrance optical coherence tomography to differentiate these lesions from a typical choroidal nevus may explain why this entity has not been previously described. The true nature of this entity will ultimately require histopathologic study.

PURPOSE: To describe a patient with a presumed retinal pigment epithelium (RPE) tumor originating from unilateral RPE dysgenesis. METHODS: Case report. RESULTS: A 30-year-old woman with an unremarkable medical and ocular history was referred for an evaluation of progressive central metamorphopsia in her left eye. Visual acuity was 20/20 in her right eye and 20/25 in her left eye. Funduscopic examination of the left eye revealed an elevated mass within an area of unilateral RPE dysgenesis showing hyperpigmentation and hypopigmentation with scalloped margins. Fundus autofluorescence of the lesion showed a marginal pattern of hyperautofluorescence and hypoautofluorescence that was the inverse of the fluorescein angiography pattern. A well-circumscribed subretinal mass appeared to originate from the unilateral RPE dysgenesis lesion with surrounding subretinal fluid extending beneath the fovea. Ultrasonography showed medium-to-high reflectivity of the mass with no evidence of choroidal involvement. Optical coherence tomography showed a subretinal hyporeflective mass consistent with a tumor of RPE origin. The tumor appeared to invade the overlying retina where fluorescein angiography showed hyperfluorescent leakage and OCT angiography showed retinal vascular deformation. Indocyanine green angiography showed no evidence of choroidal neovascularization. Findings in the right fundus were normal. Over a 5-month follow-up, intravitreal anti-vascular endothelial growth factor therapy induced a resolution of subretinal exudation and modest reduction in tumor thickness. CONCLUSION: To the authors' knowledge, this is the first report of a presumed RPE tumor described as originating from unilateral RPE dysgenesis. Multimodal imaging was crucial for establishing the diagnosis and showing that the patient's visual symptoms were a product of the exudation produced by the tumor's invasion of the retina.

PURPOSE: To demonstrate longitudinal multimodal imaging findings in a case of neovascular age-related macular degeneration presenting with multiple retinal pigment epithelium (RPE) tears showing progressive RPE restoration. METHODS: Observational clinical case report. RESULTS: A 79-year-old woman diagnosed with neovascular age-related macular degeneration developed 3 consecutive RPE tears in her right eye during the course of treatment with intravitreal anti-vascular endothelial growth factor therapy. The RPE tears initially appeared hypoautofluorescent on fundus autofluorescence. Spectral domain optical coherence tomography showed contractile folds of the RPE with adjacent subretinal fluid and overlying ellipsoid zone disruption. Over an 8-year follow-up period, the RPE defects progressively resolved with a return of patchy fundus autofluorescence. Eye-tracked spectral domain optical coherence tomography showed gradual restoration of the RPE band defects over an enlarging Type 1 neovascular lesion. CONCLUSION: Some RPE tears may show observable remodeling and restoration over time. These changes may be followed longitudinally with multimodal imaging, including eye-tracked spectral domain optical coherence tomography and fundus autofluorescence.

PURPOSE/OBJECTIVE:To describe the preclinical and very early stages of type 3 neovascularization using multimodal retinal imaging to expand our understanding of the pathogenesis of this disorder and potentially to prevent late treatment. DESIGN/METHODS:Retrospective cohort study. PARTICIPANTS/METHODS:Patients diagnosed with treatment-naïve type 3 neovascularization in the setting of age-related macular degeneration were identified at 4 retina referral centers. Inclusion criteria were: patients older than 55 years with at least 1 OCT and OCT angiography (OCTA) examination before the onset of clinically active type 3 neovascularization (i.e., preclinical stage). METHODS:Patients underwent a complete ophthalmologic examination including at least OCT and OCTA at the baseline and preclinical stage examinations, and dye angiographies when available. Demographics and clinical findings were analyzed. MAIN OUTCOME MEASURES/METHODS:Description of multimodal imaging features of nascent type 3 neovascularization. RESULTS:Fifteen eyes (15 patients; mean age, 83 ± 9 years) were included. At the baseline, mean BCVA was 0.32 ± 0.17 logarithm of the minimum angle of resolution and central macular thickness was 313 ± 50 μm. Preclinical (i.e., prebaseline) structural OCT illustrated the presence of intraretinal hyperreflective foci (HRF) at the site of type 3 neovascularization development in all patients. These foci were characterized by hyperfluorescence on dye angiography and by detectable flow on OCTA, identified with either the avascular slab (20%) or with both the deep retinal capillary plexus (DCP) and avascular slabs (80%). Typically, HRF with detectable flow on OCTA were characterized by the absence of intraretinal exudation (or very mild microcystic changes) until the lesion progressed from the DCP into the retinal pigment epithelium (RPE) and sub-RPE space. Of note, in 1 patient we observed the complete resolution of HRF despite the presence of OCTA flow and dye angiography hyperfluorescence detected at the preclinical stage examination. CONCLUSIONS:Hyperreflective foci on structural OCT may represent early intraretinal neovascularization originating from the DCP, namely nascent type 3 neovascularization; these lesions can progress to active type 3 neovascularization or more rarely may regress without functional impairment. An advanced multimodal imaging approach is useful in detecting nascent type 3 lesions, which should be followed up carefully and treated as soon as possible if flow progresses to the RPE and sub-RPE space to prevent progression to late stages.