Faculty Bibliography

PURPOSE: To report two cases of choroidal nevi associated with focal choroidal excavation (FCE) and polypoidal choroidal neovascularization (PCN). METHODS: Report of two patients with choroidal nevi showing FCE and PCN who underwent multimodal imaging including color fundus photography, fluorescein angiography, indocyanine green angiography, fundus autofluorescence, spectral domain optical coherence tomography, swept-source optical coherence tomography, and optical coherence tomography angiography. RESULTS: Two patients presented with choroidal nevi associated with FCE and PCN. In the first case, a 74-year-old woman, the nevus had sharp margins, a deep FCE, surrounding drusen, and subretinal exudation at its inferior edge due to PCN that responded well to intravitreal anti-vascular endothelial growth factor therapy. In the second case, a 64-year-old woman, the nevus had ill-defined margins, a shallow FCE, and angiographic evidence of PCN without associated exudation. CONCLUSION: There have been several reports showing an association of either choroidal nevi or FCE with PCN. To our knowledge, there have been no previous reports of FCE identified within choroidal nevi, with or without associated PCN. Since, in one of our cases, the FCE was not apparent on clinical examination, the prevalence of FCE within nevi may be underdiagnosed.

PURPOSE: We describe the long-term follow-up of a patient with multifocal Best disease with chronic bilateral serous macular detachment and unusual peripheral findings associated with a novel mutation in the BEST1 gene. METHODS: Case report. RESULTS: A 59-year-old white woman was referred for an evaluation of her macular findings in 1992. There was a family history of Best disease in the patient's mother and a male sibling. Her medical history was unremarkable. Best-corrected visual acuity was 20/20 in her right eye and 20/25 in her left eye. The anterior segment examination was normal in both eyes. Funduscopic examination showed multifocal hyperautofluorescent vitelliform deposits with areas of subretinal fibrosis in both eyes. An electrooculogram showed Arden ratios of 1.32 in the right eye and 1.97 in the left eye. Ultra-widefield color and fundus autofluorescence imaging showed degenerative retinal changes in areas throughout the entire fundus in both eyes. Optical coherence tomography, including annual eye-tracked scans from 2005 to 2016, showed persistent bilateral serous macular detachments. Despite chronic foveal detachment, visual acuity was 20/25 in her right eye and 20/40 in her left eye, 24 years after initial presentation. Genetic testing showed a novel c.238T>A (p.Phe80Ile) missense mutation in the BEST1 gene. CONCLUSION: Some patients with Best disease associated with chronic serous macular detachment can maintain good visual acuity over an extended follow-up. To our knowledge, this is the first report of Best disease associated with this mutation in the BEST1 gene.

PURPOSE: Congenital retinal macrovessels are large aberrant retinal blood vessels that cross the horizontal raphe and can traverse the central macula. Using multimodal imaging and optical coherence tomography angiography, we describe 2 cases of congenital retinal macrovessel associated with macroaneurysms. METHODS: Two patients presented for evaluation and were found to have congenital retinal macrovessels associated with macroaneurysms. Color photography, optical coherence tomography, fundus autofluorescence fluorescein angiography, and optical coherence tomography angiography were performed and used to establish the diagnosis and monitor resolution at follow-up visits. RESULTS: The first patient presented with central vision loss in the right eye and was noted to have a ruptured macroaneurysm and scattered microaneurysms along the course of a venous macrovessel. After 3 months of observation, the patient's vision improved. The second patient presented for evaluation of a cataract in her left eye and was incidentally found to have an arterial macrovessel in her right eye with an associated macroaneurysm. Both cases demonstrated an intricate capillary network in the central macula best visualized on optical coherence tomography angiography. CONCLUSION: Macroaneurysms can occur on both arterial and venous macrovessels. After rupture of these lesions, hemorrhage and exudation can resolve with observation alone. Macrovessels can also present with microaneurysms. Optical coherence tomography angiography can effectively image the complex capillary network associated with these vascular anomalies.

BACKGROUND AND OBJECTIVE/OBJECTIVE:To describe the prevalence and anatomic correlates for hyperautofluorescence related to outer retinal disruption in eyes with multifocal choroiditis (MFC). PATIENTS AND METHODS/METHODS:Retrospective review of MFC patients. RESULTS:Fifty-nine eyes from 37 patients were analyzed. Multimodal imaging was utilized to identify nine eyes (15.2%) of six patients with either transient (Group 1) or persistent (Group 2) regions of hyperautofluorescence associated with ellipsoid zone (EZ) disruption over intact retinal pigment epithelium (RPE). Group 1 included four eyes (6.8%) of three patients in which the hyperautofluorescence and EZ loss resolved within a few months (range: 28 days to 125 days) and had intact overlying outer nuclear (ONL) and outer plexiform layers (OPL) (mean follow-up: 1.3 years). Group 2 included five eyes (8.5%) of three patients with regions of permanent EZ disruption associated with absent or reduced ONL and OPL (mean follow-up: 4.6 years). CONCLUSIONS:Hyperautofluorescence correlating with EZ disruption over intact RPE is a rare occurrence in MFC. Evaluating outer retinal integrity by optical coherence tomography may help identify eyes with potential for EZ restoration, which may have implications regarding treatment strategies. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:675-683.].

Purpose/UNASSIGNED:The purpose of this retrospective study was to describe and quantify superficial hyperreflective dots within the central fovea and correlate them with age, using en face and cross-sectional B-scan optical coherence tomography (OCT). Methods/UNASSIGNED:Healthy eyes, evaluated with a spectral domain instrument (primary cohort) at the Stein Eye Institute (UCLA) and with a swept source instrument (secondary cohort) at the Vitreous Retina Macula Consultants of New York, were included in this study. En face OCT images (3 × 3 mm) segmented at the level of the superior vascular plexus were acquired, and hyperreflective dots in the foveal avascular zone were quantified by two different methodologies. The threshold reflectivity methodology quantified these dots on a cropped en face OCT image using ellipsoid zone mean reflectivity as the threshold cutoff. The OCT B-scan methodology consisted of a manual count of elevated hyperreflective signals on B-scans that colocalized with the dots by en face OCT. Primary outcome was to quantify these dots and correlate them with age. Results/UNASSIGNED:A total of 44 healthy eyes were evaluated in the primary cohort, and 16 healthy eyes were evaluated in the secondary cohort. The hyperreflective dots steadily increased in number, especially in patients older than 50 years of age, with a strongly positive statistical significant correlation, using both quantitative strategies. Conclusions/UNASSIGNED:Remarkable superficial hyperreflective dots in the central fovea of healthy subjects are novel anatomical findings that may be readily identified with both en face and cross-sectional OCT and steadily increase in number with age. We propose that these dots may represent a normal anatomical landmark, such as Müller cell end feet or inner limiting membrane basal lamina.

Photopigment bleaching occurs with saturation of photoreceptor pigment by short-wavelength fundus autofluorescence imaging. This phenomenon is seen as characteristic hyperautofluorescence with subsequent imaging acquisition. Herein, a patient with multiple sclerosis was found to exhibit increased choroidal hyperfluorescence during fluorescein angiography (FA) that corresponded with a circumscribed area of intense blue light exposure during initial scanning laser ophthalmoscopy. To the authors' knowledge, this case is the first description of photobleaching phenomenon during FA and should be recognized as nonpathologic by the clinician. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:590-592.].