Peripheral polypoidal choroidal vasculopathy in Sorsby fundus dystrophy: Report of 2 cases.

Posterboard#: A0459

Abstract Number: 4996 - A0459

AuthorBlock: Laure Van Bol1, Irina Balikova2, Florence Rasquin1 1Ophthalmology, Erasme Hospital , Brussels, Belgium; 2Ophthalmology, Hôpital Brugmann, , Belgium;

DisclosureBlock: Laure Van Bol, None; Irina Balikova, None; Florence Rasquin, None;

Purpose To report for the first time a new phenotype of Sorsby fundus dystophy (SFD), characterized by the presence of multiple peripheral polypoidal choroidal vasculopathy (PCV).Methods Retrospective review of medical records and multimodal imaging of 2 patients with clinical features consistent with SFD except the presence of peripheral PCV. Both individuals underwent genetic testing which confirmed a mutation of the TIMP3 gene.Results Both patients had a history of early visual loss in their fifties, a positive family history of early onset visual decline and presented with extensive drusen-like deposits, chorioretinal atrophy and recurrent choroidal neovascularization. They equally showed bilateral multiple polypoidal vascular abnormalities in the mid-peripheral . Genetic testing revealed the same heterozygous nucleotide substitution of C113G, causing a Ser38Cys change in Exon 1 of the N-terminal domain of the TIMP3 gene.Conclusions Multiple peripheral PCV has never been described among patients carrying a TIMP3 mutation. This study therefore expands the spectrum of phenotypic features of SFD and highlights the important role of ICG-A in the initial assessment and follow-up of patients with SFD.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details.

Natural History of Recessive in Untreated Eyes: An Analysis of Study- and Individual-level Data

Posterboard#: A0477

Abstract Number: 5014 - A0477

AuthorBlock: Liangbo Linus Shen1, Mengyuan Sun3, Holly Grossetta Nardini2, Lucian V. Del Priore1 1Department of and Visual Science, Yale School of Medicine, New Haven, Connecticut, United States; 2Harvey Cushing/John Hay Whitney Medical Library, Yale University, New Haven, Connecticut, United States; 3Department of Molecular Biophysics and Biochemistry, Yale University, New Haven, Connecticut, United States;

DisclosureBlock: Liangbo Linus Shen, None; Mengyuan Sun, None; Holly Grossetta Nardini, None; Lucian V. Del Priore, None;

Purpose Controversy exists regarding the natural history of atrophic lesion secondary to recessive Stargardt disease (STGD) with the reported growth rates of lesion area varying widely across clinical trials. We performed a study- and individual-level analysis to investigate how the lesions grow over time in untreated eyes.Methods We searched in 5 databases for studies that monitored atrophic lesion progression in untreated eyes with STGD over ≥ 6 months. We extracted both study- and individual-level data from the included studies, and analyzed both levels of data using 3 models: (1) the area linear model (ALM), in which the lesion area enlarges linearly with time, (2) the radius linear model (RLM), in which the lesion radius expands linearly with time, and (3) the area exponential model (AEM), in which the lesion area changes exponentially with time. A horizontal translation factor was added to shift each dataset to correct for differences in subjects’ entry time into the studies. The model that best fit data was determined by the predicted age of lesion onset and dependence of growth rate on baseline lesion size.Results Of 1630 articles screened, we identified 9 studies that met our inclusion criteria. Among them, we extracted study-level data from all 9 studies (990 eyes) and individual-level data from 5 studies (228 eyes). Cumulative study- and individual-level datasets fit along a straight line in the RLM after horizontal translation (r2 = 0.98 and 0.93, respectively; Fig. 1 and 2). The age of onset of atrophy predicted by the RLM (21.0 ± 4.0 years) is similar to the reported age of onset (20.7 ± 3.4 years); in contrast, the predictions by the ALM and AEM deviate by more than 5 years (Fig. 1C). Based on the individual-level data, the growth rate of effective lesion radius was found independent of the baseline lesion size (r = 0.06); in comparison, the growth rate of area and natural log of area is significantly dependent on the baseline lesion size (r = 0.47 and -0.33, respectively).Conclusions The progression of atrophic lesion secondary to STGD best fits the RLM for both study- and individual- level data (growth rate = 0.091 mm/yr). This may assist the design of clinical trials on STGD.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details.

Effect of 0.1% Bromfenac for Preventing after Surgery in Patients with Diabetes

Posterboard#: A0466

Abstract Number: 5003 - A0466

AuthorBlock: Younghoon Lee1,2, Seok Hyeon Song1,2, Byung Yi Ko1,2 1Ophthalmology, Konyang univerisity College of medicine, Daejeon, Korea (the Republic of); 2Ophthalmology, Konyang univeristy hospital, Daejeon, Korea (the Republic of);

DisclosureBlock: Younghoon Lee, None; Seok Hyeon Song, None; Byung Yi Ko, None;

Purpose To investigate the effect of 0.1% bromfenac sodium hydrate ophthalmic solution for prevention of macular edema after cataract surgery in patients with diabetes.Methods We performed a retrospective analysis of 71 patients with diabetes who underwent cataract surgery and followed up more than 6 months. 38 patients (51 eyes) instilled 0.1% bromfenac solution(bromfenac group) and 33 patients (39 eyes) did not(control group).Results There were no significant defference between the group. Best corrected visual acuity (BCVA) was significantly improved at all visit time in both groups than before surgery, and there was no significant difference between the two groups. At 1 month postoperatively, the central macular thickness (CMT) of the control group (314.15 ± 76.11μm) was significantly thicker than the bromfenac group (264 ± 31μm)(p <0.001), but no significant difference was found at 4 and 6 months. The mean change of CMT was significantly less in bromfenac group at 1 (-1.44 ± 11.72μm vs 47.19 ± 70.24μm, p <0.001) and 4 months (10.44 ± 22.48μm vs 31.69 ± 48.04μm, p=0.016), but there was no statistically significant difference between the two groups at 6 months after surgery (3.7 ± 24.13μm for the bromfenac group vs 16.65 ± 38.59μm for the control group, p=0.053).Conclusions 0.1% Bromfenac sodium hydrate ophthalmic solution showed good efficacy for preventing cystoid macular edema after cataract surgery in patients with diabetes.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Case Series of Bilateral Idiopathic Multiple Pigment Epithelial Detachments

Posterboard#: A0458

Abstract Number: 4995 - A0458

AuthorBlock: Boris Rosin1, Eyal Banin1 1Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel;

DisclosureBlock: Boris Rosin, None; Eyal Banin, None;

Purpose Pigment Epithelial Detachments (PEDs), elevations of the Retinal Pigment Epithelium (RPE) above the Bruch's membrane, are a common finding in retinal disease. They appear in Age-Related (AMD) and its variants (e.g., Polypoidal Choroidal Vasculopathy – PCV), several disorders of the RPE and other conditions. Here we describe the findings in three patients (2 males, 1 female) with presumed Idiopathic Multifocal Pigment Epithelium Detachments (IMPED), a condition first identified by Gass and colleagues1 and thus far described in only five patients in the literature.Methods A retrospective case series of three patients with the presumed rare diagnosis of IMPED.Results The three patients, in their forties, presented to the Hadassah Retinal Degenerations clinic between the years 2015-2018. All exhibited multiple bilateral PEDs on clinical examination and on wide-field fundus imaging, in the absence of drusen. We confirmed the classification of the lesions as PEDs by OCT. Notably, the patients demonstrated preserved visual acuity and an otherwise normal ophthalmic examination. Electrophysiological testing demonstrated normal retinal and RPE function by full-field electroretinography and electrooculography, respectively. Angiographic studies excluded the diagnoses of NVAMD and PCV. The patients remained stable during the course of follow-up, which was 7, 3.5 and 0.9 years for the study group (including revision of previous imaging studies), during which time we did not note any dynamic changes, either clinically or by ancillary testing.Conclusions While in themselves PEDs usually do not necessitate treatment, dynamic changes of PEDs have been associated with possibility of disease progression in AMD and were shown to herald a significant bleeding episode in PCV patients. Here we demonstrate that IMPED, despite being exceedingly rare, appears to represent a distinct clinical entity, seen in relatively young patients. The long-term implications and prognosis of these lesions are not known and warrant further follow-up.

1 Gass. et al. Bilateral idiopathic multifocal retinal pigment epithelium detachments in otherwise healthy middle-aged adults: a clinicopathologic study. Retina 2005; 25:304-310Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Here we describe a rare disorder of the Retinal Pigment Epithelium (RPE), a supportive monolayer of cells located immediately under and adjacent to the retina. The disorder is characterized by multifocal RPE lesions in which the RPE detaches from the retina, known as Pigment Epithelium Detachments (PEDs). The disorder was therefore termed Idiopathic Multiple Pigment Epithelial Detachments (IMPED). The patients remained stable during the course of the follow-up. Long-term follow up will allow to determine whether these lesions put the patients at risk for other, significantly more common disorders involving the RPE, such as Ager-Related Macular Degeneration. To date, only five cases of IMPED have been described and no long-term follow-up exists.

Topography Shows Limited Ellipsoid Zone Recovery from Mild Hydroxychloroquine Toxicity

Posterboard#: A0461

Abstract Number: 4998 - A0461

AuthorBlock: Brandon Pham1, Luis De Sisternes2, Mary K. Durbin2, Michael F. Marmor1 1Byers Eye Institute at Stanford, Palo Alto, California, United States; 2Carl Zeiss Meditec, Dublin, California, United States;

DisclosureBlock: Brandon Pham, None; Luis De Sisternes, Carl Zeiss Meditec Code E (Employment) , Mary K. Durbin, Carl Zeiss Meditec Code E (Employment) , Michael F. Marmor, None;

Purpose While severe hydroxychloroquine (HCQ) progresses relentlessly, milder damage stabilizes clinically, raising the possibility of limited anatomic recovery after drug cessation. Scattered anecdotal reports have indicated some improvement, but the consistency and extent has been unclear.Methods Our recent report on long-term progression after HCQ cessation included 5 patients with early- moderate toxicity without RPE damage that remained stable with respect to foveal thickness, ellipsoid zone (EZ) line integrity, and visual acuity for up to 9 years after HCQ cessation. To assess more critically the possibility of cellular recovery, we have used an advanced automated segmentation paradigm to define the EZ boundary (pixel by pixel) from SD-OCT volume scans and produce topographic maps of EZ integrity and loss. These images and measurements of EZ area were compared to sequential visual fields (when available), fundus autofluorescence (FAF), and SD-OCT cross-sections obtained at the time of HCQ cessation, and 2 and 7 years later.Results Topographic maps obtained from 4 of the 5 patients (one was excluded due to poor OCT resolution from ) illustrate the anatomic extent of EZ line loss at our 3 time points. One patient had minimal EZ line abnormalities, one showed a 30% decrease in the area of EZ loss over 7 years, one showed small but focal improvements, and one remained stable during the observation period. Sequential visual fields in 2 patients showed no clear changes beyond field variability. FAF hyperfluorescence rarely followed EZ changes. Matching SD-OCT cross-sections to corresponding topographic maps showed that improvement occurred irregularly in areas where the EZ line was thinned or fragmented. However, areas of complete EZ line loss or markedly thinned ONL showed no significant improvement. Other areas of damage deepened locally over time.Conclusions The potential for limited anatomic recovery related to the initial extent of EZ line damage at the time of drug cessation. Regions of complete EZ line absence showed essentially no improvement, while other regions of mild EZ line disruption, where the ONL was mostly intact, could be repaired (though functional improvement was likely rather minimal). Efforts to detect subtle signs of retinopathy earlier might allow for more functional recovery but may carry risk of inappropriately stopping a valuable drug for ambiguous findings.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. The drug hydroxychloroquine (HCQ) is commonly used to treat autoimmune disorders, such as systemic lupus erythematosus (SLE) and rheumatoid arthritis. While generally considered quite safe, it can damage the retina when taken for long periods of time or at excessive dose. If this damage is already at a severe stage when first discovered, it can progressively worsen over the course of many years. On the other hand, toxicity that is discovered early from proper screening, before it has progressed in severity, has been shown to stabilize. The question remains, however, as to whether there can be any actual recovery. In this study, we used a new analytic method to make topographic maps of the area of retinal damage. We followed patients with early HCQ toxicity for up to 9 years after stopping the drug, and found that some recovery can indeed occur. However, the extent is quite limited, and areas where cellular structures are lost do not seem to recover. Quantitative Fundus Autofluorescence in PROM1-Macular Dystrophy

Posterboard#: A0488

Abstract Number: 5025 - A0488

AuthorBlock: Maarjaliis Paavo1,2, Winston Lee1, Jose R. Carvalho-Jr1,3, Stephen Tsang1,4, Rando Allikmets1,4, Janet Sparrow1,4 1Columbia University Medical Center, Ophthalmology , New York, New York, United States; 2Helsinki University Eye Hospital, , Finland; 3Department of Ophthalmology, Federal University of Pernambuco, , Brazil; 4Department of Pathology and Cell Biology, Columbia University Medical Center, New York, United States;

DisclosureBlock: Maarjaliis Paavo, None; Winston Lee, None; Jose R. Carvalho-Jr, None; Stephen Tsang, None; Rando Allikmets, None; Janet Sparrow, None;

Purpose Mutations in PROMININ 1 (PROM1), a transmembrane glycoprotein, alter photoreceptor outer segment disk morphogenesis and can cause Stargardt-like macular dystrophy (STGD4; MIM603786) with fundus features similar to ABCA4-disease (recessive Stargardt disease, STGD1). As short wavelength fundus autofluorescence (SW-AF) is elevated in STGD1 we sought to quantify the levels of autofluorescence in STGD4 to seek a better understanding of the disease process.Methods Ten eyes of 7 patients (33-66 years, mean age = 49,4 years) were prospectively studied by clinical exam and multimodal imaging. Autofluorescence (AF) images were acquired with the Spectralis SLO- OCT (Heidelberg Eng.) modified by insertion of an internal AF reference to account for variable laser power and detector sensitivity for quantitative autofluorescence (qAF). Further clinical testing also included near-infrared autofluorescence (NIR-AF), spectral domain-optical coherence tomography (SD- OCT) and full-field electroretinography (ffERG). The genetic cause of the disease was confirmed by direct sequencing of the PROM1 gene (23 exons) in all patients.Results All patients presented with isolated lesions of macular atrophy, however one family with individuals affected across two generations exhibited granular fleck-like deposits across the posterior pole. Areas of granular deposition corresponded to intermittent loss of the EZ band while discrete regions of hypoautofluorescence corresponded to complete loss of outer retinal layers on SD-OCT. Four patients presented with foveal sparing. Autofluorescence levels within the macula (qAF8) the macula of all patients were found to be within the 95% confidence intervals of healthy age-matched individuals.Conclusions Although PROM1-macular dystrophy (STGD4) can exhibit phenotypic overlap with STGD1, significantly increased autofluorescence levels are not detected. As such, lipofuscin accumulation may not be a significant event in the pathophysiology of PROM1-disease. The qAF approach could serve as a method of early differential diagnosis when considering different types of macular dystrophy. qAF will help to identify appropriate disease targets as therapeutics become available to treat retinal disease.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Predictive factors for metamorphopsia after reduced- fluence photodynamic therapy in central serous chorioretinopathy patients with good visual acuity at baseline.

Posterboard#: A0475

Abstract Number: 5012 - A0475

AuthorBlock: Mayuka Hayashida1, Akiko Miki1, Shunichiro Nakai1, Wataru Matsumiya1, Hisanori Imai1, Sentaro Kusuhara1, Makoto Nakamura1 1Kobe University, Kobe, Japan;

DisclosureBlock: Mayuka Hayashida, None; Akiko Miki, None; Shunichiro Nakai, None; Wataru Matsumiya, None; Hisanori Imai, None; Sentaro Kusuhara, Kobe University Code P (Patent) , Makoto Nakamura, None;

Purpose To investigate predictive factors for metamorphopsia after reduced-fluence photodynamic therapy (RFPDT) in central serous chorioretinopathy (CSC) patients with good visual acuity at baseline.Methods 26 eyes of 26 consecutive patients with resolved CSC after RFPDT and best-corrected visual acuity (BCVA) better than 0.6 (74 letters in ETDRS chart) were retrospectively reviewed. We measured metamorphopsia using M-CHARTS at 6 months after RFPDT. A greater one of the horizontal and vertical M-CHARTS scores was applied for the extent of metamorphopsia. Patients were divided into 2 groups depending on the presence or absence of metamorphopsia defined as M-CHARTS score equal to or greater than 0.5°; M (+) group (n=14, M-CHARTS score≧0.5°), or M (-) group (n=12, M-CHARTS score<0.5°). We investigated the differences in age, sex, time of duration, baseline BCVA, and findings of optical coherence tomography (OCT) between groups.Results Duration of symptom in M (+) group was significantly longer compared with that in M (-) group. (P=0.027). Outer nuclear layer (ONL) thickness before RFPDT was significantly thinner in M (+) group than M (-) group (P=0.020). In the multivariate analysis, thinner ONL thickness before RFPDT (P=0.014) was related to large metamorphopsia in CSC patients with good BCVA at baseline.Conclusions ONL thickness may be useful as predictive factors for metamorphopsia after RFPDT in CSC patients with good BCVA at baseline.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Predictive Value of Pachyvessels in Patients with Central Serous Chorioretinopathy (CSCR) Treated by Photodynamic Therapy (PDT)

Posterboard#: A0474

Abstract Number: 5011 - A0474

AuthorBlock: Johanna Wiedemann1, Vasilena Sitnilska1, Lebriz Altay1 1Department of Ophthalmology, University of Cologne, Cologne, Germany;

DisclosureBlock: Johanna Wiedemann, None; Vasilena Sitnilska, None; Lebriz Altay, Quantel Medical Code R (Recipient)

Purpose To establish an automatic quantification method of pachyvessel area in patients with central serous chorioretinopathy (CSCR) and to evaluate predictive value of pachyvessel area for successful treatment by half-dose photodynamic therapy (PDT).Methods All patients who received a PDT in the Eye Department of the University of Cologne between 01.08.2015 - 01.09.2018 were retrospectively analyzed (n=146). Only therapy-naïve CSCR patients with good quality of indocyanin green angiography (ICGA) at baseline, and with a follow-up within 1-3 months after first half-dose PDT were included. Presence of pachyvessels (defined as dilated choroidal vessel) was qualitatively evaluated on ICGA frames within 6 mm grid (manually centered on the fovea). Thickest choroidal vessel diameter above the lesion was measured manually using Heidelberg Engineering Heyex Software (Version 1.10.2.0). Automatic quantitative analysis of the choroidal vessel area based on ICGA pictures (from injection up to 1:30 minutes) was done with Fiji Image J Processing Package (Version 2.0.0, trainable weka segmentation plugin) after manual training. Therapeutic success was defined as at least 50% reduction of subretinal fluid height after one PDT.Results Thirthy-nine eyes of 36 patients (male n = 28 (71.8%), female n= 11 (28.2%), mean age of all 53.28.4 years) of whom three where affected in both eyes fitted all above defined criteria. All patients showed visible dilated choroidal vessels (pachyvessels) within 6 mm grid. Eighteen patients (46.2 %) showed therapy response after half-dose PDT. Pachyvessel diameter above the lesions ranged from 132 μm to 317 μm, whereas pachyvessel area ranged from 15.83 mm2 to 27.67 mm2. Pachyvessel diameter and pachyvessel area were positively correlated (p=0.005, r=-0.438). Smaller pachyvessel area was positively associated with a successful outcome after half-dose PDT (mean pachyvessel area in responders 20.93 ± 2.71 mm2vs. in non-responders 23.24 ± 4.02 mm2, p = 0.046).Conclusions Measurement of pachyvessel area and thickness via ICGA may be an useful tool to predict therapy response to half-dose PDT in CSCR patients.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Short wavelength automated perimetry and standard achromatic perimetry in central serous chorioretinopathy

Posterboard#: A0501

Abstract Number: 5038 - A0501

AuthorBlock: Han Peng Zhou1, Tatsuya Inoue1, Ryo Asaoka1, Takumi Hara1, So Makino1, Hiroshi Murata1, Ryo Obata1 1Ophthalmology, The University of Tokyo Hospital, Tokyo, Japan;

DisclosureBlock: Han Peng Zhou, None; Tatsuya Inoue, None; Ryo Asaoka, None; Takumi Hara, None; So Makino, None; Hiroshi Murata, None; Ryo Obata, None;

Purpose Short wavelength automated perimetry (SWAP) is known for detecting reduction of retinal sensitivity in advance of standard achromatic perimetry in patients. However, its application in other retinal diseases is still limited. In the current study, we investigated the difference in retinal sensitivity between standard white-on-white automated perimetry and blue-on-yellow SWAP in central serous chorioretinopathy (CSC) patients with macular serous (SRD).Methods Differences in retinal sensitivity (RS) between standard white-on-white automated perimetry (W/W) and blue-on-yellow SWAP (B/Y) was investigated in 24 eyes of 24 CSC patients with macular SRD. RS thresholds were measured within 10 degrees of the fovea. The mean overall RS of W/W (RSw) and B/Y (RSb) as well as the mean RS within (RSwin, RSbin) and outside (RSwout, RSbout) of the SRD region were calculated. Central retinal thickness (CRT), central choroidal thickness (CCT), SRD area (SRDa), and SRD height at the fovea (SRDh) were measured using spectral domain OCT. The correlation between RS (RSw, RSb) and OCT parameters (CRT, CCT, SRDa, SRDh) was assessed.Results The mean age of the participants was 55.6 ± 11.6 years and the mean baseline logMAR BCVA was 0.056 ± 0.157. Among the 24 patients who completed the study, RSb was significantly lower than RSw (p < 0.001). Furthermore, the difference between the RS inside and outside of the SRD area (RSout – RSin) was significantly greater in B/Y compared to W/W (p < 0.001). Significant negative correlations were observed between logMAR BCVA and RSw (p < 0.001) and RSb (p < 0.001). SRDa was significantly related to both RSw and RSb (p < 0.001, respectively). In a series of multivariate analyses, logMAR BCVA was significantly related to CCT and SRDa (p<0.001, respectively). RSw was significantly related to age, SRDa, and SRDh (p<0.01, respectively). RSb was significantly related to age and SRDa (p < 0.001, respectively).Conclusions Our study demonstrated that blue-on-yellow SWAP is able to detect the difference in retinal sensitivity between inside and outside of the SRD region in advance of that by conventional achromatic perimetry in CSC patients. These results may suggest the usefulness of SWAP for early detection of the changes in retinal function in CSC.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Reading with macular telangiectasia type 2 – a case of binocular confusion.

Posterboard#: A0460

Abstract Number: 4997 - A0460

AuthorBlock: Tjebo Heeren1,3, Simone Tzaridis2, Catherine A. Egan1, Frank G. Holz2, Peter Charbel Issa4,5, Simona Degli Esposti1, Philipp Herrmann2, Siegfried Karl Wagner3, Marcus Fruttiger3, Gary S. Rubin3 1Moorfields Eye Hospital NHS Foundation Trust, London, ENGLAND, United Kingdom; 2Department of Ophthalmology, University Bonn, Bonn, Germany; 3Institute of Ophthalmology, University College London, London, United Kingdom; 4Oxford Eye Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom; 5Nuffield Laboratory of Ophthalmology, Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom;

DisclosureBlock: Tjebo Heeren, None; Simone Tzaridis, None; Catherine A. Egan, None; Frank G. Holz, None; Peter Charbel Issa, None; Simona Degli Esposti, None; Philipp Herrmann, None; Siegfried Karl Wagner, None; Marcus Fruttiger, None; Gary S. Rubin, None;

Purpose To assess binocular gain in patients with macular telangiectasia type 2 (MacTel) and its correlation to the characteristic paracentral .Methods Sixty eight patients with macular telangiectasia type 2 were consecutively recruited for a cross- sectional analysis. Best corrected visual acuity (BCVA), reading acuity and reading speed were tested monocularly and binocularly with ETDRS letter charts and Radner reading charts. Macular retinal sensitivity was examined with fundus-controlled perimetry (microperimetry). Scotomas were quantified by depth, size and proximity to the fovea.Results Mean reading speed was higher in the right than in the left eyes (155 vs. 137 words per minute [wpm], p-value < 0.0001). Binocular reading acuity and speed were lower than reading acuity and speed in the functionally better eye (142 vs. 159 wpm and 0.43 vs. 0.28 logRAD, p-value < 0.0001). The difference between the right eye and the left eye was correlated to the height of binocular inhibition of reading speed (R2= 0.61), but not of reading acuity or of BCVA (R2<0.03). Scotoma depth, size and proximity to the fovea were strongly correlated to binocular reading speed (adjusted R2 = 0.65), but there was only a weak correlation to binocular reading acuity or BCVA (adjusted R2 = 0.39). The effect of characteristics on binocular reading speed was significant for scotomas of the left eye, but not for scotomas of the right eyes.Conclusions The results indicate a significant impact of the worse eye on reading ability. Patients with binocular inhibition of reading performance might benefit from occlusion of one eye for reading. The worse performance of left eyes may be explained by the typical localisation of the scotoma nasally to fixation, resulting in projection of the scotoma into reading direction.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Combination therapy with oral mineralocorticoid antagonist and ophthalmic glucocorticoid for non-resolving central serous chorioretinopathy.

Posterboard#: A0502

Abstract Number: 5039 - A0502

AuthorBlock: Der-Chong Tsai1,2, De-Kuang Hwang3, Shih Jen Chen3 1Ophthalmology, National Yang-Ming University Hospital, Yilan, Taiwan; 2School of Medicine, National Yang-Ming University, Taipei, Taiwan; 3Ophthalmology, Taipei Veterans General Hospital, Taipei, Taiwan;

DisclosureBlock: Der-Chong Tsai, None; De-Kuang Hwang, None; Shih Jen Chen, None;

Purpose Emerging evidence has revealed the mineralocorticoid receptor (MR) antagonist as an effective treatment of central serous chorioretinopathy (CSC) with non-resolving subretinal fluid (SRF). Besides, the MR pathway may partially explain the paradoxical pro-edematous effect of glucocorticoids in CSC. After MR blockage, it is hypothesized that glucocorticoids may further reduce, rather than aggravate, the persistent SRF in CSC. This study aims to investigate the short-term effectiveness and safety of combination therapy with spironolactone and ophthalmic glucocorticoids among the patients with non-resolving CSC.Methods This retrospective case series included fifteen eyes of fifteen consecutive patients (12 [80%] male) with non-resolving CSC after oral MR antagonist treatment. Once enrolled, patients were treated with the combination of oral spironolactone (25 mg twice a day) and ophthalmic glucocorticoids (betamethasone eye drop four times a day and dexamethasone gel once a day). Best-corrected visual acuity (BCVA), intraocular pressure (IOP) and spectral-domain optical coherence tomography examinations were performed at each visit. The primary outcome measure was the change of SRF height at fovea during the 3-month interval between the initiation of combination therapy (baseline) and the last visit. The secondary outcome measures were the changes of central foveal thickness (CFT), subfoveal choroidal thickness (SFCT) and BCVA during this 3-month period.Results The mean (± standard deviation [SD]) foveal SRF height at baseline, 1-month and 3-month visits were 205.2 (± 101.3) μm, 125.1 (± 83.2) μm, and 45.6 (± 65.9) μm, respectively. During the 3-month follow up, there were significant reduction of SRF height (p= 0.001), CFT (p= 0.002) and SFCT (p=0.017). Six (40%) patients experienced complete resolution of SRF. The mean BCVA improved from 0.51 (± 0.36) to 0.37 (± 0.37) logMAR (p= 0.008). No patient had more than 10 % increase in CFT and any decrease in BCVA from baseline. One patient developed a significant rise in IOP (>30 mmHg).Conclusions With favorable short-term visual and anatomical outcomes in this study, combination therapy of MR antagonist and ophthalmic glucocorticoids appears to be a safe option for non-resolving CSC. The long-term outcome of this therapy warrants further investigation.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Glucocorticoid usage has been considered a risk factor for central serous chorioretinopathy (CSC). The pro-edematous effect of glucocorticoid in CSC may result from its cross-reactivity at the mineralocorticoid receptor. Our results showed that after the mineralocorticoid receptors are blocked, ophthalmic glucocorticoids may further reduce, rather than aggravate, the subretinal fluid in eyes with CSC. The short-term safety and visual and anatomical outcomes of combination therapy with the oral mineralocorticoid receptor antagonist and ophthalmic glucocorticoids were favorable for non- resolving CSC. Real world anti-vascular endothelial growth factor therapy in myopic choroidal neovascularisation: long term outcomes

Posterboard#: A0470

Abstract Number: 5007 - A0470

AuthorBlock: Oonagh Crothers1, Devangna Bhatia1, Muhammad Raza Cheema1, Joanna DaCosta1, James S. Talks1 1Newcastle Eye Centre, Royal Victoria Infirmary, Newcastle upon Tyne, United Kingdom;

DisclosureBlock: Oonagh Crothers, None; Devangna Bhatia, None; Muhammad Raza Cheema, None; Joanna DaCosta, None; James S. Talks, Allergan Code F (Financial Support) , Bayer Code F (Financial Support) , Novartis Code F (Financial Support) , Bayer Code R (Recipient)

Purpose Evaluation of the long-term efficacy of intravitreal anti-vascular endothelial growth factor (anti-VEGF) therapy in the primary treatment of choroidal neovascularisation (CNV) secondary to pathological myopia.Methods We performed a retrospective analysis of electronic medical records of treatment naive patients with myopic CNV who received treatment with either intravitreal ranibizumab or aflibercept (n = 37 eyes) over a 10.5 year period from September 2007 to May 2018. Patients with prior treatment for CNV including photodynamic therapy, laser photocoagulation, or other causes of CNV such as AMD, trauma, or angioid streaks were excluded. Patient age, gender, degree of myopia, status, best corrected visual acuity (BCVA) and central retinal thickness (CRT) before and after treatment, and number of injections administered was recorded.Results The data set included 37 eyes of 35 patients (25 females, 12 males). The mean age was 60 years (range 26 - 89 years). 25/35 (71%) patients were aged > 50 years, 12/35 (34%) were < 50 years. 28/37 (76%) eyes were phakic and 9/37 (24%) eyes were pseudophakic. 2 patients received bilateral treatment for myopic CNV. Mean spherical equivalent was -5.81 dioptres. 26/37 (70%) eyes commenced primary treatment with ranibizumab and 11/37 (30%) eyes commenced treatment with aflibercept. Mean number of injections was 4.47 over a mean follow up period of 13.43 months. Mean CRT decreased by 92.9μm, mean BCVA improvement was 3.91 letters. Univariate linear regression with BCVA as the outcome showed younger age (p =0.02) and fewer injections (p =0.01) were associated with final BCVA. Baseline VA (p =0.13), and spherical equivalent (p =0.23) were not associated with final BCVA. Multiple regression showed age and fewer injections (p = 0.005) were associated with final BCVA. Conclusions Myopic CNV requires less frequent anti-VEGF intravitreal therapy over a shorter follow up period than both neovascular age related macular degeneration and diabetic macular oedema. Increased frequency of administration of intravitreal anti-VEGF treatment did not improve vision. Younger age is associated with a better final visual outcome. This may be due to the effects of macular atrophy in the older age group.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. DEFERRED FOCAL LASER PHOTOCOAGULATION IN CENTRAL SEROUS CHORIORETINOPATHY: A RETROSPECTIVE CASE SERIES

Posterboard#: A0503

Abstract Number: 5040 - A0503

AuthorBlock: Kajal Sangal1, Steven D. Ness1, Nicole Siegel1, Manju L. Subramanian1 1Ophthalmology, Boston University School of Medicine, Boston, Massachusetts, United States;

DisclosureBlock: Kajal Sangal, None; Steven D. Ness, None; Nicole Siegel, None; Manju L. Subramanian, None;

Purpose Limited data exists to evaluate the outcomes of deferred focal laser photocoagulation in the treatment of central serous chorioretinopathy (CSCR). We performed a retrospective case series to examine visual and anatomic outcomes of laser intervention after initial observation.Methods This retrospective review analyzed data from 2005-2018 and enrolled 32 eyes of 30 patients (22 males, 8 females) diagnosed with CSCR with a mean age of 48.35 ± 9.74 years. Patients with no history of treatment underwent focal laser photocoagulation after a period of observation of at least 2 months. Exclusion criteria included lack of follow up, an observation period of less than 2 months, and any treatment for CSCR prior to undergoing focal laser. Outcomes measured included visual acuity (VA), central macular thickness (CMT) in microns, as measured by optical coherence tomography (OCT), recurrence of fluid, and complications. Data were analyzed using paired, two-tailed t- tests.Results Mean pre-laser VA was 0.38 ± 0.31 logMAR (Snellen equivalent 20/48). Average CMT was 375.75 ± 122.17. The median period of observation was 4.50 months. The average best-corrected post-laser visual acuity (BCVA), noted at a median of 4.00 months after laser, was 0.19 ± 0.25 logMAR (Snellen equivalent 20/31) with an average CMT of 243.25 ± 62.52. Final follow up occurred at a median of 9.50 months after laser with a final vision of 0.37 ± 0.65 logMAR (Snellen equivalent 20/47) and a mean CMT of 239.74 ± 58.47. Pre-laser VA to BCVA, pre-laser CMT to CMT at BCVA, and pre-laser CMT to CMT at final follow up significantly improved (p=<0.001). Pre-laser VA to final VA did not significantly improve (p=0.895). Three eyes developed complications of choroidal neovascular membrane (CNVM), and if these 3 were removed from the analysis, then final VA significantly improved (p=0.003). Of 32 eyes, 23 (71.9%) had complete resolution without recurrence, 3 (9.4%) developed recurrent subretinal fluid after resolution, and 6 (18.8%) had persistent subretinal fluid following laser.Conclusions Our results show that deferred focal laser photocoagulation significantly improves VA and CMT, and final visual outcome can be limited by complications such as CNVM. This study demonstrates that focal laser photocoagulation is a viable and less invasive treatment alternative to photodynamic therapy for CSCR that does not improve after initial observation.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Central serous choroioretinopathy (CSCR) is a disorder that can cause poor vision from the development of fluid pockets in the back of the eye under the retina. Many times this improves on its own, but sometimes it does not. There are different treatment options available, one of which is hot laser treatment known as laser photocoagulation. Our study looked at the effect of laser treatment in a series of patients who did not respond to at least two months of observation. It showed that 72% of patients improved with this treatment, and the remaining patients did not respond or did respond but it came back. This study shows that using laser treatment for CSCR may be a good treatment option for patients who do not respond to observation. Best Disease is a Phenocopy of North Carolina Macular Dystrophy (NCMD / MCDR1)

Posterboard#: A0504

Abstract Number: 5041 - A0504

AuthorBlock: Kent W. Small1,2, Benjamin Bakall4, Edwin M. Stone5, Robert Wiggins3, Nitin Udar1,2, Steven Agemy6, Fadi Shaya1,2 1Molecular Insight Research Foundation, Glendale and Los Angeles, California, United States; 2Macula and Retina Institute, Glendale and Los Angeles, California, United States; 3Asheville Eye Associates, North Carolina, United States; 4University of Arizona College of Medicine, Phoenix, Arizona, United States; 5University of Iowa, Iowa City, Iowa, United States; 6SUNY Downstate Medical Center University, Brooklyn, New York, United States;

DisclosureBlock: Kent W. Small, None; Benjamin Bakall, None; Edwin M. Stone, None; Robert Wiggins, None; Nitin Udar, None; Steven Agemy, None; Fadi Shaya, None;

Purpose North Carolina Macular Dystrophy (NCMD / MCDR1) and Best disease are both autosomal dominant macular dystrophies. While both have phenotypic variability, NCMD has considerably more. NCMD has three grades of severity which are present from birth. NCMD grade 1 is drusen, grade 2 is confluent drusen and / or small fibrosis and CNVM, grade 3 is a developmental defect (coloboma like) in the central macula. Isolated individuals outside the context of other affected family members can be difficult to accurately diagnose and differentiate the two disease intities. We will perform a case series evaluation of the similarities and differences between Best disease and NCMD grade 2.Methods Clinical findings including fundus photography and OCTs were evaluated in 5 individuals with NCMD grade 2 and two with Best disease. EOG was performed in one NCMD subject. Sanger DNA sequencing was performed to confirm diagnoses. IRB approval was obtained.Results Five of NCMD grade 2 individuals have clinical findings indistinguishable from Best's macular dystrophy. One NCMD subject had an abnormal EOG with a normal ERG, which has previously been considered a unique feature of NCMD. OCT in Best disease can demonstrate a lucency deep to the elevated vitelliform lesion which is not seen in NCMD. One NCMD grade 2 subject had had elevated submacular fluid giving a pseudo-vitelliform appareance on OCT.Conclusions Best disease is another phenocopy of NCMD. There is considerable clincial overlap between NCMD and Best macualr dystrophy. Like Best disease, NCMD can also have a normal EOG with a normal ERG. Examining other family members can help differentiate the two. DNA sequencing can provide a more definitivve molecular diagnosis.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Best disease and North Carolina Macular Dystrophy can have significant phenotypic overlap, which can make it difficult for clinicians to distinguish between the two. Herein are provided the similarities and differences clincally, electrophysiologically and molecularly. Medical Treatment of Macular Holes

Posterboard#: A0505

Abstract Number: 5042 - A0505

AuthorBlock: Jared Todd Sokol1, Sidney Schechet2, Jerome Vincent Giovinazzo3, Shaun Ittiara4, Asim Farooq2, Veeral Shath5,6, Rahul Komati2, Ronald Gentile3, Dimitra Skondra MD,PhD2 1Pritzker School of Medicine, University of Chicago, Chicago, Illinois, United States; 2Ophthalmology and Visual Science, University of Chicago, Chicago, Illinois, United States; 3Ophthalmology, New York Eye and Ear of Mount Sinai, New York, New York, United States; 4Retinal Vitreal Consultants, Chicago, Illinois, United States; 5University Retina, Oak Forest, Illinois, United States; 6llinois Eye and Ear Infirmary, University of Illinois at Chicago, Chicago, Illinois, United States;

DisclosureBlock: Jared Todd Sokol, None; Sidney Schechet, None; Jerome Vincent Giovinazzo, None; Shaun Ittiara, None; Asim Farooq, None; Veeral Shath, Genentech Code C (Consultant) , Alimera Code C (Consultant) , NotalVision Code C (Consultant) , Rahul Komati, None; Ronald Gentile, Alcon Laboratories, Inc. Code C (Consultant), Acucela Code F (Financial Support), Genentech Code F (Financial Support), Notal Vision Inc. Code F (Financial Support), Quark Pharmaceuticals Code F (Financial Support), Regeneron Pharmaceuticals, Inc. Code F (Financial Support), Dimitra Skondra MD,PhD, None;

Purpose Macular holes (MHs) are a common retinal disorder which usually requires surgical treatment with pars plana vitrectomy. Here we present a case series of MHs that had successful closure with topical medical treatment.Methods We conducted a retrospective review of the medical records, including optical coherence tomography (OCT) studies, of 11 patients with full-thickness macular holes that responded favorably to medical treatment. Treatment included steroid, non-steroidal anti-inflammatory (NSAID), and carbonic anhydrase inhibitor (CAI) drops.Results 8 men and 3 women with an average age of 67.5 years were included in the study. 7 out of 11 eyes had prior vitrectomy (4 for retinal detachment and 3 for previous MH) and non-vitrectomized eyes had vitreous separation from the macula. MHs were diagnosed clinically and confirmed by OCT. The initial OCT study in all cases revealed a full-thickness MH (mean size +/- SD = 184um +/- 80um) with surrounding cystoid hydration. After starting topical medical therapy (steroid + NSAID + CAI), follow- up OCT studies revealed decreases in cystic changes within 2 to 4 weeks and MH closure with visual acuity improvement in 4-8 weeks. Average best-corrected visual acuity improved from 20/82 at initial presentation to 20/40 after MH closure.Conclusions Small full-thickness MHs, especially in previously vitrectomized eyes or those with macular vitreous separation, may benefit from topical medical therapy with steroid, NSAID, and CAI drops. Prospective controlled studies are warranted to further elucidate the use of medical therapy in the treatment of MHs and to better understand if medical treatment could be a conservative alternate to surgery.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Choroidoscleral Interface Irregularity Index: A novel optical coherence tomographybased parameter in patients with

Posterboard#: A0506

Abstract Number: 5043 - A0506

AuthorBlock: Mirinae Kim1, Young-Hoon Park1 1Seoul St.Mary's Hospital, Seoul, Korea (the Republic of);

DisclosureBlock: Mirinae Kim, None; Young-Hoon Park, None;

Purpose This study aimed to assess the regularity of the choroidoscleral interface (CSI) using a novel parameter, CSI-irregularity index, before and after epiretinal membrane (ERM) surgery.Methods This retrospective cohort study included 36 patients with idiopathic ERM who underwent uncomplicated pars plana vitrectomy and ERM removal. All subjects underwent ocular examinations at baseline and at 1, 2, 4, and 6 months after surgery.Results The bowl-shaped (regular) contour of the CSI was found in 14 patients (38.9%); mean CSI-irregularity index was 14.8 ± 11.0 in this group. The inflective (irregular) contour of the CSI was found in 22 patients (61.1%) and mean CSI-irregularity index was 34.0 ± 20.6. The CSI irregularity index decreased gradually after ERM surgery, and was correlated with postoperative best-corrected visual acuity.Conclusions The CSI-irregularity index could serve as a surrogate marker to quantitatively represent the CSI morphology. We observed the gradual decrease of the CSI-irregularity index after ERM surgery in quantitative manner. This study revealed correlations between the CSI-irregularity index and visual outcomes after ERM surgery. Our results suggest that the CSI-irregularity index might be an intuitive anatomic indicator of the CSI and might be useful as a possible prognostic marker for patients undergoing ERM surgery.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Association of Macular Disease with Long-term Use of Pentosan Polysulfate Sodium: Findings from a Large U.S. National Insurance Database

Posterboard#: A0507

Abstract Number: 5044 - A0507

AuthorBlock: Alexa L. Li1, Nieraj Jain1, Yinxi Yu2, Brian L. VanderBeek2 1Ophthalmology, Emory Eye Center, Atlanta, Georgia, United States; 2Ophthalmology, Scheie Eye Institute University of Pennsylvania, Philadelphia, Pennsylvania, United States;

DisclosureBlock: Alexa L. Li, None; Nieraj Jain, None; Yinxi Yu, None; Brian L. VanderBeek, None;

Purpose We recently described a unique pigmentary in the setting of chronic exposure to pentosan polysulfate sodium (PPS), a treatment for interstitial cystitis. In all affected patients, these pigmentary changes were previously ascribed to a diagnosis of dry macular degeneration or pattern dystrophy. Herein, we performed a retrospective review of medical claims data from a national U.S. insurer to determine if long-term PPS usage is linked to diagnoses of varying macular disorders in a large U.S. population.Methods A retrospective, matched cohort study using data from a medical claims database was performed. The exposure cohort consisted of all patients prescribed PPS from 2002-2011. The index date was the date when the patient first filled a prescription for PPS, and patients were required to have at least 2 years prior to and 5 years after the index date for inclusion. Controls were matched 5:1 for age, gender, race, insurance plan eligibility start/end date and were assigned the same index date their exposed match. Exclusion occurred for being <18 years old and any dry macular degeneration (dAMD) or drusen diagnosis prior to the index date. The primary outcome was defined as a new International Classification of Disease (ICD-9/10) diagnosis code for a hereditary or secondary pigmentary retinopathy with a secondary outcome also including a new diagnosis of dAMD or drusen. Multivariate logistic regression analyses were performed controlling for demographic and systemic health variables.Results 1187 PPS users and 5939 controls met the eligibility criteria. At the 5-year follow-up, 5(0.4%) and 71(6.0%) PPS patients compared to 16(0.3%) and 266(4.5%) control patients progressed to the primary and secondary outcomes respectively. Multivariate analysis showed no significant association for PPS and the primary outcome (OR=1.49, 95% CI:0.54-4.11, P=0.45); however, exposure to PPS significantly increased the odds of having a secondary outcome (OR=1.39, 95% CI:1.04–1.85, P=0.03).Conclusions PPS exposure was associated with a new diagnosis of macular disease at the 5-year follow-up in a large national cohort. These results corroborate findings of the initial case series that suggested an association between PPS exposure and a novel maculopathy, although the association was noted at a lower exposure duration than in the index cases.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Relationship between the retinal displacement and the thickness change after vitrectomy in MH eyes

Posterboard#: A0508

Abstract Number: 5045 - A0508

AuthorBlock: Kensuke Goto1, Takeshi Iwase1, Tomohiko Akahori1, Hiroko Terasaki1 1Nagoya University , Nagoya, Japan;

DisclosureBlock: Kensuke Goto, None; Takeshi Iwase, None; Tomohiko Akahori, None; Hiroko Terasaki, Carl-Zeiss Code F (Financial Support) , Santen Code F (Financial Support) , Pfizer Code F (Financial Support) , Wakamoto Code F (Financial Support) , Otsuka Code F (Financial Support) , Kowa Code F (Financial Support) , Senju Code F (Financial Support) , Alcon Japan Code F (Financial Support) , Novartis Code F (Financial Support) , HOYA Code F (Financial Support) , Allergan Japan Code F (Financial Support) , Nidek Code F (Financial Support) , Santen Code R (Recipient) , Alcon Japan Code R (Recipient) , Novartis Code R (Recipient) , Bayer Code R (Recipient) , Carl Zeiss Code R (Recipient)

Purpose The macular displacement after the vitrectomy with inner limiting membrane (ILM) peeling for idiopathic macular hole (MH) has been reported. We investigated whether the retinal thickness change is accompanied with the macular displacement.Methods Twenty eyes of 20 patients who received the vitrectomy with ILM peeling for MH at the Nagoya University Hospital were included. OCTA (Angioplex) was used to obtain 3mm × 3mm en face images before and 2, 8 weeks after the surgery. We measured the distance between the center of the and an easily identifiable bifurcation of the retinal vessels in the 4 quadrants (temporal, superior, nasal, and inferior). Spectral-domain OCT (Spectralis) was used to measure the retinal thickness at the point 1500μm away from the fovea at the same period. In measuring the thickness, four retinal layer thicknesses were measured: the nerve fiver layer to the inner nuclear layer (inner retina), the outer plexiform layer to the retinal pigment epithelium (RPE) (outer retina), the external limiting membrane (ELM) to the ellipsoid zone (EZ) (ELM-EZ), the EZ to the RPE (EZ-RPE). The relationship between the macular displacement and the retinal layer thickness change was analyzed.Results The retina was significantly displaced nasally in all of the quadrants after Week 2 (p<0.001), whereas there were no significant differences in the displacement among postoperative periods. The temporal retinal thickness significantly decreased 2 weeks after the surgery (p=0.005). The inner retinal thickness in the temporal quadrant significantly decreased 2 and 8 weeks after the surgery (p=0.001, p=0.002, respectively). The outer retinal thickness in the nasal and inferior quadrants significantly increased 8 weeks after the surgery (p=0.008, p=0.004, respectively). The ELM-EZ thickness showed no significant changes, while the EZ-RPE thickness showed significantly thicker at Week 8 compared with before the surgery in all of the quadrants (all, p<0.05). The macular displacement was not correlated with the changes of retinal thickness in any layers.Conclusions The retinal layer thickness was significantly changed and the macula was significantly displaced after the surgery. However, the layer thickness changes were not correlated with the macular displacement, indicating that the postoperative retinal layer thickness changes would not be influenced by the retinal displacement.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. A Case Series of Spontaneous Opening and Closing Macular Holes Associated with Lamellar Hole Epiretinal Proliferation

Posterboard#: A0509

Abstract Number: 5046 - A0509

AuthorBlock: Cecile Truong1, Royce Chen1 1Ophthalmology, Columbia University Medical Center, New york, New York, United States;

DisclosureBlock: Cecile Truong, None; Royce Chen, Zeiss Code C (Consultant)

Purpose Lamellar hole epiretinal proliferation (LHEP) originates from vitreous material and occurs in 8% of patients with full-thickness macular holes (FTMH). Previously, several patients with LHEP were reported to have spontaneous opening and closing FTMH after cataract extraction or vitrectomy. Therefore, we hypothesize that the presence of LHEP is associated with idiopathic opening and closing of FTMH.Methods We retrospectively analyzed spectral domain optical coherence tomography (SD-OCT) scans of 73 consecutive patients diagnosed with macular holes, lamellar holes, and pseudoholes to find cases of spontaneous development and resolution of FTMH without surgical correction. We then analyzed those 8 patients for common factors, such as presence of traditional epiretinal membrane (ERM) and LHEP.Results Out of 73 patients, 81 eyes were classified as having macular, lamellar, or pseudoholes. 8 of those 81 eyes (10%) had FTMH that spontaneously opened and closed. In those 8 eyes, 8 had LHEP (100%), 8 had traditional ERM (100%), 7 had complete PVD (87.5%), 5 had a history of cataract surgery (62.5%), 4 had glaucoma (50%), 4 had myopia (50%), 2 had (25%), and 2 had diabetes (25%). Four patients with spontaneously opening and closing FTMH (50%) eventually had vitrectomies, internal limiting membrane peels, and gas tamponades performed while the other 4 (50%) continued to be observed and maintained stable visual acuity. All 4 patients who underwent vitrectomy had successful closure with one operation and remained closed after the initial surgery.Conclusions Spontaneous opening and closing macular holes appear to be associated with the combination of both traditional ERM and LHEP. ERM exert tangential traction on the fovea, likely causing spontaneous macular holes in these eyes in the absence of vitreomacular traction. LHEP is predominantly composed of fibroblasts and hyalocytes, cells with less contractile properties than those found in standard ERM. LHEP may help to induce spontaneous closure of some macular holes. Depending on the severity and recurrence of ERM/LHEP-associated macular holes, some patients can continue to be observed while others require an operation.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details.

Eplerenone and Spironolactone Have Similar Efficacy in the Treatment of Chronic Central Serous Chorioretinopathy

Posterboard#: A0471

Abstract Number: 5008 - A0471

AuthorBlock: David Massop1, Sepehr Bahadorani1, Sejal Lahoti2, Michael Singer3,1 1Ophthalmology, University of Texas Health Science Center San Antonio, San Antonio, Texas, United States; 2Long School of Medicine, University of Texas Health Science Center San Antonio, San Antonio, Texas, United States; 3Medical Center Ophthalmology Associates, San Antonio, Texas, United States;

DisclosureBlock: David Massop, None; Sepehr Bahadorani, None; Sejal Lahoti, None; Michael Singer, None;

Purpose Central serous chorioretinopathy (CSCR) is a disease of multifactorial etiology that is often observed until resolution. The purpose of this study is to compare the efficacy of mineral receptor antagonists eplerenone and spironolactone in the treatment of chronic CSCR. We hypothesize that both medications have a similar efficacy.Methods An IRB-approved retrospective review was conducted on patients that were diagnosed with chronic CSCR, which is defined as persistence of subretinal fluid for more than 3 months. Patients were divided into eplerenone (25mg daily) and spironolactone (50mg twice per day) treatment groups. The primary outcomes included change in visual acuity and central macular thickness (CMT) before and after therapy. Statistical significance was determined using analysis of variance (ANOVA). Secondary outcomes included any reported adverse events while taking these oral mineral receptor antagonists.Results Clinical charts from 15 eyes with chronic CSCR were reviewed, of which 7 eyes were treated with eplerenone and 8 eyes were treated with spironolactone. Optical coherence tomography (OCT) was obtained upon the initiation of the respective medication and again at the 6-12 week follow up period. There was no statistically significant difference between the initial CMT of both treatment groups. At the follow up visit, CMT reductions in the eplerenone and spironolactone groups were 66.9 µm and 99.8 µm, respectively (P=0.56). Likewise, there was no statistically significant difference (P=0.6) in ETDRS letter gain between eplerenone and spironolactone, at 1.9 letters and 3.5 letters respectively. No systemic adverse events were noted in the 6-12 week treatment period. The average 30 day supply cost1 of eplerenone 25 mg daily is $124.97, while spironolactone 50 mg twice per day is $57.40.

1. GoodRx.comConclusions Eplerenone and spironolactone have a similar efficacy in the treatment of chronic CSCR. The cost of spironolactone is approximaetly half the cost of eplerenone. In a small sample size, we have no reportable side effects or safety concerns.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Etiology, treatment and visual outcomes of patients with choroidal neovascularization under the age of 50

Posterboard#: A0510

Abstract Number: 5047 - A0510

AuthorBlock: David Xu1,2, Anthony Obeid1,2, Hannah J. Levin1,2, Douglas Matsunaga1, Kalla A. Gervasio1, Thomas L. Jenkins1,2, Ravi R. Pandit1,2, Allen C. Ho1,2 1Retina Service, Wills Eye Hospital, Philadelphia, Pennsylvania, United States; 2Retina, Mid Atlantic Retina, Philadelphia, Pennsylvania, United States;

DisclosureBlock: David Xu, None; Anthony Obeid, None; Hannah J. Levin, None; Douglas Matsunaga, None; Kalla A. Gervasio, None; Thomas L. Jenkins, None; Ravi R. Pandit, None; Allen C. Ho, Regeneron Code C (Consultant), Genentech Code C (Consultant)

Purpose Choroidal neovascularization (CNV) complicates a range of degenerative, dystrophic, inflammatory and traumatic disorders. This report characterizes patients under the age of 50 who developed CNV and analyze the etiology, treatment and visual outcomes.Methods This retrospective study enrolled patients from Wills Eye Hospital and Mid Atlantic Retina. Patients were included if they were under 50 years old, clinically diagnosed with CNV and had at least three months of follow up. Patients were excluded if they had retinal neovascularization or had received prior treatment for CNV. The underlying etiology causing CNV was reported. Mean age, gender, frequency of bilateral CNV, number of anti-vascular endothelial growth factor (VEGF) injections and visual acuities (VA) at baseline and last follow up were recorded. Logarithm of the minimum angle of resolution (logMAR) VA was compared using paired t test.Results 168 eyes of 154 patients were included. Median age was 36 (range 6-50) years and 103 (61%) were female. 14 (9%) patients had bilateral CNV. 23 cases were in pediatric patients. The most prevalent etiologies were inflammatory/uveitic causes (80 eyes, 47%), idiopathic (35 eyes, 21%), high myopia (33 eyes, 20%), and central serous chorioretinopathy (15 eyes, 9%). The single most prevalent disease was multifocal choroiditis affecting 53 eyes (32%). Traumatic choroidal rupture accounted for 11 cases (7%). Mean follow up was 22 ± 16 months. 131 eyes (78%) required anti-VEGF treatment with a mean of 6.0 ± 5.6 injections with while 35 (21%) were observed. The location of CNV was macular in 146 cases (87%), peripapillary in 14(8%), and peripheral in 5 (3%). CNV was subfoveal in 85 eyes (51%). Those with subfoveal CNV had worse vision at baseline (+0.31 logMAR, p < 0.001) and last follow up (+0.28 logMAR, p < 0.001) compared to those without. The majority (80%, 135 eyes) of eyes exhibited stable or improved VA over the follow up duration. Overall, mean VA improved by 0.13 +/- 0.07 logMAR (p < 0.001) compared to their initial presentation.Conclusions In contrast to adults over age 50 where neovascular age-related macular degeneration predominates as the cause of CNV, younger patients develop CNV for a heterogeneous range of etiologies including inflammatory disorders, idiopathic, or secondary to high myopia. Layman Abstract (optional): Provide a 50-200 word description of your work that non- scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Abnormal blood vessel growth, or choroidal neovascularization (CNV), can occur due to a variety of retinal disorders. We analyzed patients who had CNV under the age of 50 and found that they were most often caused by inflammatory/uveitic conditions, idiopathic (no identifiable cause), or were due to high myopia. These findings can help make other ophthalmologists aware of the causes of CNV so that patients can be appropriately treated or referred for care. Familial discordance in disease phenotype in siblings with Stargardt disease

Posterboard#: A0478

Abstract Number: 5015 - A0478

AuthorBlock: Dyon Valkenburg1,2, Esmee Runhart1,2, Bart Liefers2,3, stanley lambertus1,2, Clara I. Sanchez3,2, Frans P. Cremers4,2, nathalie m. bax1,2, Carel C B Hoyng1,2 1Radboud university medical center, Department of Ophthalmology, Nijmegen, Gelderland, Netherlands; 2Radboud University Medical Center, Donders Institute for Brain, Cognition and Behaviour, Cognitive Neuroscience Department, Nijmegen, Gelderland, Netherlands; 3Radboud university medical center, Department of Radiology, Nijmegen, Gelderland, Netherlands; 4Radboud university medical center, Department of Human Genetics, Nijmegen, Gelderland, Netherlands;

DisclosureBlock: Dyon Valkenburg, None; Esmee Runhart, None; Bart Liefers, None; stanley lambertus, None; Clara I. Sanchez, None; Frans P. Cremers, None; nathalie m. bax, None; Carel C B Hoyng, None;

Purpose To investigate intersibling discordance of the Stargardt disease (STGD1) phenotype.Methods We performed a retrospective cohort study among siblings with genetically confirmed STGD1 and at least one available fundus autofluorescence (FAF) image of both eyes. We compared age of onset within families using the youngest patient as the reference and a predetermined threshold value of 10 years for significant differences. Disease duration was matched to investigate differences in best- corrected visual acuity, and we determined and compared the survival time for reaching severe (SVI); (<20/200 Snellen or > 1.3 Logarithm of the Minimal Angle of Resolution (LogMAR)). Central retinal atrophy surface area was quantified and compared by two independent graders using the semi-automated EyeNED software. Additionally, both graders performed qualitative assessment of FAF patterns to identify phenotypic differences and commonalities. Main outcome measures included differences in age of onset, best-corrected visual acuity (BCVA), time to develop legal blindness, FAF atrophy surface area and autofluorescence patterns.Results Significant differences in age of onset were present in 5/17 families, ranging from 13 to 39 years. BCVA was matched in 12/17 families and the median difference was 0.41 (0 – 1.10) LogMAR for the right and 0.41 (0 – 1.08) LogMAR for the left eye, and we found extreme differences in five families ranging from 0.58 to 1.1 LogMAR. The median age at which patients developed SVI was 14 years. We observed significant differences in time to develop SVI in three out of 12 families with matched survival times, ranging from 14 to 29 years. Median central retinal atrophy surface area was 11.38 mm2 in the right (range 1.98 – 44.78 mm2) and 10.59 mm2 in the left (range 1.61 – 40.59 mm2) eyes and was highly comparable between siblings, with the exception of family one. Qualitative FAF phenotypes were comparable in all sibling pairs.Conclusions Phenotypic discordance between siblings with STGD1 disease carrying the same ABCA4 variants is a prevalent phenomenon. Functional outcomes can differ substantially despite highly comparable FAF phenotypes, which complicates sibling-based prognosis. While environmental factor are likely to modify the disease course, the relatively young median age at which patients develop SVI indicates an important role for genetic factors as disease modifiers.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Characteristics of Outer Choroidal Vessels in Eyes with Chronic Central Serous Chorioretinopathy Assessed by En Face Optical Coherence Tomography

Posterboard#: A0511

Abstract Number: 5048 - A0511

AuthorBlock: Jun Takeuchi1, Keiko Kataoka1, Yuyako Nakano1, Ai Fujita1, Yasuki Ito1, Hiroko Terasaki1 1Department of Ophthalmology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan;

DisclosureBlock: Jun Takeuchi, None; Keiko Kataoka, Novartis Pharma K.K., Japan Code R (Recipient), Santen Pharmaceutical Co., Ltd, Japan Code R (Recipient), Bayer Health Care, Japan Code R (Recipient), Yuyako Nakano, None; Ai Fujita, None; Yasuki Ito, Alcon Novartis Pharma, Japan Code R (Recipient), Bayer Health Care, Japan Code R (Recipient), Canon Life Care Solutions Inc., Japan Code R (Recipient), Carl Zeiss Meditec Co., Ltd., Japan Code R (Recipient), Kowa Pharmaceutical Company LTD., Japan Code R (Recipient), Pfizer Japan Inc., Japan Code R (Recipient), Santen Pharmaceutical Co., Ltd, Japan Code R (Recipient), Hiroko Terasaki, Otsuka Pharmaceutical Co., Ltd., Japan Code F (Financial Support), NIDEK Co., Ltd., Japan Code F (Financial Support), Kowa Pharmaceutical Company Ltd., Japan Code F (Financial Support), Santen Pharmaceutical Co., Ltd, Japan Code F (Financial Support), Senju Pharmaceutical Co., Ltd., Japan Code F (Financial Support), Alcon Pharma, Japan Code F (Financial Support), Bayer Health Care, Japan Code F (Financial Support), Pfizer Japan Inc., Japan Code F (Financial Support), ROHTO Pharmaceutical Co., Ltd., Japan Code F (Financial Support), WAKAMOTO Co., Ltd., Japan Code F (Financial Support), Carl Zeiss Meditec Co., Ltd., Japan Code R (Recipient), Nitten Pharmaceutical Co., Ltd., Japan Code R (Recipient), Tomey Corporation Ltd., Japan Code F (Financial Support), AMO Japan K.K., Japan Code F (Financial Support), Eisai Co., Ltd., Japan Code F (Financial Support), Mitsubishi Tanabe Pharma Corporation Ltd., Japan Code R (Recipient), AbbVie GK, Japan Code R (Recipient), DAIICHI SANKYO Co., Ltd., Japan Code C (Consultant), CHUO SANGIO Co., Ltd., Japan Code R (Recipient), Sanofi K.K., Japan Code R (Recipient), HOYA Corporation Ltd., Japan Code F (Financial Support)

Purpose Central serous chorioretinopathy (CSC) is characterized by choroidal thickening mainly due to pathologic dilatation of outer choroidal vessels. Recently, it was reported that most vortex veins in the eyes of acute CSC show vertically asymmetric, and more than three-fourths of those eyes had dominant vortex veins extended beyond macula region. This study aimed to evaluate the outer choroidal vessels in eyes with chronic CSC.Methods This retrospective study collected 48 eyes of 48 patients with treatment naïve chronic CSC. 12 × 12 scan area of Swept-source optical coherence tomography (PLEX Elite 9000, AngioPlex, Zeiss) were performed in all patients to obtain en face images of the posterior pole in addition to complete ophthalmic examination including indocyanine green angiography. We manually chose the slab containing Haller vessel and Sattler vessel to construct en face images. Three independent investigators examined all the en face images and classified them into 2 groups: the symmetry group with vertical symmetric outer choroidal vessels and the asymmetry group with vertical asymmetric outer choroidal vessels. The asymmetry group was further classified into 3 grades according to how far the dominant vortex vein extends: the macular region (grade 1), the posterior pole (grade 2), or beyond the vascular arcades (grade 3). The excluding criteria was the defective en face image due to severe cataract or excess motion artifacts.Results The mean age was 58.31 ± 13.20 years (range 36-83 years), and 38 subjects (79.2%) were male. The mean axial length was 23.80 ± 1.15 mm (range 21.90-26.83 mm, unknown in 3 subjects), and the mean symptom duration to diagnosis was 38.02 ± 51.02 months (range 4-234 months, unknown in 4 subjects). Of total 48 chronic CSC eyes,14 eyes (29.2%) were classified into the symmetry group, and 34 eyes (70.8%) were classified into the asymmetry group. In the asymmetry group, 23 eyes were classified as grade 1, 5 eyes as grade 2, and 6 eyes as grade 3.Conclusions Almost 30% of chronic CSC has vertical symmetric outer choroidal vessels. Even in the asymmetry group, only 32.4% eyes had dominant vortex veins extending beyond macula region (grade 2 + grade 3). It suggests that as CSC becomes chronic, the blood flow of dilated dominant vortex veins may be compensated by the contralateral vortex veins.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Blue-light reflectance imaging in macular telangiectasia type 2 (MacTel) using two different SLO systems.

Posterboard#: A0512

Abstract Number: 5049 - A0512

AuthorBlock: Ferenc B. Sallo1,2, Vincent Rocco3, Irene Leung3,7, Adam M. Dubis3,1, Ute EK Wolf- Schnurrbusch8, Catherine A. Egan3, Tunde Peto6,7, Traci E. Clemons10, Daniel Pauleikhoff4, Emily Y. Chew9, Alan C. Bird5 1Visual Neuroscience, UCL Institute of Ophthalmology, London, ENGLAND, United Kingdom; 2Hopital Ophtalmique Jules-Gonin, Lausanne, Vaud, Switzerland; 3Research & Development, Moorfields Eye Hospital, London, United Kingdom; 4Ophthalmology, St Franziskus Spital, Munster, Nordrhein- Westphalen, Germany; 5Inherited , Moorfields Eye Hospital, London, United Kingdom; 6Ophthalmology, Queens University Belfast, Belfast, United Kingdom; 7NIHR Biomedical Research Center for Ophthalmology, at Moorfields Eye Hospital NHS Foundation Trust and UCL Institute of Ophthalmology, London, , United Kingdom; 8Ophthalmology, Inselspital, University of Bern, Bern, Switzerland; 9National Eye Institute, Bethesda, Maryland, United States; 10Emmes Corporation, Rockville, Maryland, United States;

DisclosureBlock: Ferenc B. Sallo, None; Vincent Rocco, None; Irene Leung, None; Adam M. Dubis, None; Ute EK Wolf-Schnurrbusch, None; Catherine A. Egan, Novartis Pharmaceuticals Code C (Consultant), Heidelberg Engineering Code C (Consultant), Tunde Peto, None; Traci E. Clemons, None; Daniel Pauleikhoff, None; Emily Y. Chew, None; Alan C. Bird, None;

Purpose MacTel is a disease of the central retina, leading over time to photoreceptor degeneration and potentially to bilateral loss of central vision. One of the earliest signs of MacTel is a pattern of abnormal parafoveal reflectance, thought to originate in the inner retina due to increased scattering caused by structural reorganisation in these layers. Reflective scanning laser ophthalmoscopic (SLO) imaging using short wavelength light (blue light reflectance, BLR imaging) provides a superior tool for recording this phenomenon. Our aim was to investigate how different SLO systems compare in BLR imaging in MacTel.Methods Eyes of participants with a diagnosis of MacTel were selected from the cohort of the MacTel Study on the basis of the availability of confocal blue-light reflectance (BLR) images collected using the two SLO systems of interest. Images were acquired using a Heidelberg Retina Angiograph II and a Heidelberg Spectralis SLO system (Heidelberg Engineering GmbH, Heidelberg, Germany) using the 'red free' setting (utilizing a 488nm blue laser for illumination of the retina). Images were aligned using I2K Retina (v1.3.8, DualAlign™ LLC) and analyzed in Adobe Photoshop v CS6. The lateral extent of hyper-reflectivity was delineated and measured (expressed in pixels) and their relative areas and locations were compared. Agreement between measurements was assessed by calculation of Spearman's coefficient of rank correlation using MedCalc (v12.7.0.0, MedCalc Software BVBA, Ostend, Belgium).Results Full image sets were available for 21 eyes of 11 patients (5 females, 6 males), ranging in age between 46-71 years, (mean=58.2 years, SD=7.3 years). MacTel disease severity ranged from stage 1-5 on the Gass & Blodi scale. Agreement of area measurements of BLR hyper-reflectivity was good, Spearman's coefficient of rank correlation (ρ) = 0.868 (95%CI 0.664 - 0.951, p=<0.0001, n=17) In two eyes no hyper-reflective area was apparent and in another two eyes the hyper-reflective area could not be delineated accurately. A specific pattern was discernible: Spectralis images often contained central round artifacts whereas HRA2 images demonstrated an hourglass-shaped reduction in luminance.Conclusions While the correlation of BLR lesion extent is good, studies aiming for a quantitative analysis of BLR intensity must take into account the technical differences between HRA2 and Spectralis SLO devices.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Micropulsed laser treatment of Central Serous Retinopathy

Posterboard#: A0469

Abstract Number: 5006 - A0469

AuthorBlock: Davide Allegrini1, Giovanni Montesano2, Gabriella Ricciardelli1, Chiara Coretti1, Mariantonia Ferrara1, Mario R. Romano1 1Ophthalmology, Humanitas University, Bergamo, Italy; 2Ophthalmology, University of Milan, Milano, Italy;

DisclosureBlock: Davide Allegrini, None; Giovanni Montesano, None; Gabriella Ricciardelli, None; Chiara Coretti, None; Mariantonia Ferrara, None; Mario R. Romano, None;

Purpose To evaluate the response to micropulsed laser treatment and its effect on visual outcome in patients with Central Serous Retinopathy (CRS)Methods We retrospectively analysed data from 15 patients with CRS who underwent micropulsed laser treatment for subertinal fluid (SRF). As per clinical practice, patients were reviewed at 1 and 3 months after the first treatment and then every 3 months, undergoing retreatment in case of relapses. All subjects had a follow up at 9 months (12 months for 5 subjects). Central Macular Thickness (CMT) were used as a surrogate measure for changes in SRF. Response to treatment was defined as the absence of SRF. First responses and complete responses (no fluid at the end of the follow up) were analysed performing a survival analysis. Since the response could have occurred in-between visits, interval censoring was used. Therefore, survival curves are composed of interval estimates of equal likelihood. Correlation of response rate (RR) with baseline conditions was explored via a proportional hazard model. CMT trend was analysed using a linear mixed model (for repeated measurements). Correlations between baseline and outcome CMT and Visual Acuity (VA) were analysed trough simple linear models.Results Most of the subjects (75%) had their first response within 1 month (RR = 0.38 [0.04, 0.71], Median [95% CIs]). Eight patients were retreated for relapses. Complete responses occurred at a steadier slower rate over time (RR = 0.18 [0.02, 0.36], Figure 1A). Baseline conditions (VA and CMT) had no significant effect on the response and were similar between retreated and non-retreated eyes. A significant reduction of CMT and increase in VA from baseline (p < 0.05) was achieved at all time points (Figure 2A), with a mean increase in VA at 9 months of 0.3 ±0.24 (decimals). Such an increase was positively correlated (p = 0.038) with baseline CMT (Figure 2B).Conclusions Micropulsed laser is a viable option to treat CRS, but retreatments may be necessary. Randomized controlled trials will be needed to assess long-term effects, safety and efficacy over other treatments. Interestingly, baseline CMT is positively correlated with the final visual outcome and may be a useful prognostic parameter. The physiopathological interpretation is, however, unclear.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details.

A Novel Autosomal Dominant Retinal Dystrophy: Phenotypic description and Preliminary Genetic Investigations in a Pedigree.

Posterboard#: A0513

Abstract Number: 5050 - A0513

AuthorBlock: Ajoy Vincent1, Matteo DiScipio2, Erika Tavares3, Nicole Roslin2, Elise Heon1 1Ophthalmology and Vision Sciences, Hospital for Sick Children, Toronto, Ontario, Canada; 2Hospital for Sick children, Ontario, Canada; 3Hospital for Sick Children, Ontario, Canada;

DisclosureBlock: Ajoy Vincent, None; Matteo DiScipio, None; Erika Tavares, None; Nicole Roslin, None; Elise Heon, None;

Purpose To describe the clinical phenotype in a peculiar retinal dystrophy predominantly affecting the posterior pole in a pedigree.Methods Fourteen members (3 – 70 years of age; including five affected) of a large European pedigree were recruited. All members underwent detailed ophthalmological examination including best corrected visual acuity, color vision, contrast sensitivity and fundus examination. Spectral-domain optical coherence tomography (SD-OCT), fundus auto-fluorescence testing (FAF) and full-field electroretinography (ERG) were performed in three affected individuals. Panel based genetic testing for known retinal dystrophy genes (n = 244) was performed using a CLIA certified lab in proband’s mom. DNA was extracted from blood or saliva in all family members. Linkage analysis was performed using data generated from single nucleotide polymorphism (SNP) BeadChip using Omni5Exome kit (Illumina).Results The best corrected visual acuity ranged between 20/20 and 20/300 in all patients. Mild red-green color vision deficit was noted in the oldest affected individual (67 years of age). Fundus examination showed confluent hypo-pigmented deep retinal changes, and retinal atrophy within the posterior pole; macular atrophy was noted in older affected individuals (64 and 67 years, respectively). The FAF testing showed a speckled pattern (hyper and hypo) of auto-fluorescence within the posterior pole in all affected eyes that were tested (n = 3); large atrophic patches were noted in older affected individuals. Macular SD-OCT scans showed numerous, small, hyper-reflective depositions at the level of the pigment epithelium in the proband (24 years); the older affected individuals demonstrated marked disruption of photoreceptor inner and outer segments within the macula. The ERG was normal to all tested stimuli in the proband; the photopic ERG responses were borderline in the older affected individuals. Panel based genetic testing results returned negative. Linkage analysis showed a maximum LOD score of 1.79 under a dominant model of inheritance assuming 99% penetrance and 0.001 disease allele frequency; 11 linked regions spanning 3.3% of the genome was identified.Conclusions The clinical phenotype described in this study appears novel and likely represents a slowly progressive retinal dystrophy affecting the posterior pole. The underlying genetic basis remains to be elucidatedLayman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Characterizing Retinal-Choroidal Anastomosis in Macular Telangiectasia Type 2

Posterboard#: A0462

Abstract Number: 4999 - A0462

AuthorBlock: Mark P. Breazzano1,2, Lawrence A. Yannuzzi1,2, Richard F. Spaide1,2 1Vitreous Retina Macula Consultants of New York, New York, New York, United States; 2Columbia University College of Physicians and Surgeons, New York, New York, United States;

DisclosureBlock: Mark P. Breazzano, None; Lawrence A. Yannuzzi, None; Richard F. Spaide, Topcon Medical Systems Code C (Consultant), Topcon Medical Systems Code P (Patent), Dutch Ophthalmic Research Center Code P (Patent)

Purpose To characterize structural and angiographic findings in macular telangiectasis type 2 (MacTel2) and examine associations with visual acuity (VA).Methods MacTel2 patients with examination, fundus photography including autofluorescence, spectral-domain optical coherence tomography (SD-OCT), and projection-resolved OCT angiography were retrospectively evaluated.Results There were 43 eyes of 22 patients with mean age 63.9 (±10.3) years. Six had diabetes. Twenty-one (48.8%) eyes had retinal-choroidal anastomoses (RCAs) without any evidence of subretinal or choroidal neovascularization. When found, an average 55 (±33.7) individual RCAs were clustered primarily in temporal perifoveal in each of these eyes. Right-angle veins were seen in all 21 eyes with RCAs. Hyperpigmentation was present in 18 eyes, all with RCAs (p<0.001). Conical hyperreflective material spanning Bruch’s past external limiting membrane ≥200um basal diameter was found in 21 eyes, and labeled outer retinal hyperreflective lesion. RCAs colocalized with outer retinal hyperreflective lesion (p<0.001), ≥2/3 outer nuclear layer absence with posterior vascular descent (grade 2 retinal subsidence, p<0.001), and ellipsoid zone defect ≥500um (p=0.002) as shown in Figure. Diabetes (p=0.015), RCA quantity (p=0.04), and ellipsoid zone defect extent (p=0.01) each correlated independently with decreased VA.Conclusions The outer retinal hyperreflective lesion is a novel SD-OCT finding consistent with altered architecture in MacTel2, offering an intriguing insight regarding pathophysiologic changes. Diabetes, ellipsoid zone defect extent, and RCAs predict poorer vision with MacTel2. Additional cases may be needed to tease out individual contributions from these highly inter-correlated findings for decreased vision in MacTel2.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Fluorescence Lifetime Imaging Ophthalmoscopy (FLIO) in Clinically Unaffected Family Members of MacTel Patients

Posterboard#: A0489

Abstract Number: 5026 - A0489

AuthorBlock: Alexandra Vitale1, Lydia Sauer1, Karl Andersen1, Paul S. Bernstein1 1Moran Eye Center, Salt Lake City, Utah, United States;

DisclosureBlock: Alexandra Vitale, None; Lydia Sauer, Heidelberg Engineering Code R (Recipient), Karl Andersen, None; Paul S. Bernstein, Heidelberg Engineering Code F (Financial Support), Heidelberg Engineering Code R (Recipient)

Purpose Macular telangiectasia type 2 (MacTel) is an inherited retinal disease following an autosomal dominant pattern with reduced penetrance. As genetic causes of MacTel remain unknown, ophthalmic imaging helps determine the diagnosis. FLIO shows distinct and early changes in MacTel. This study investigates FLIO-changes in unaffected family members with the aim to diagnose the disease at earlier stages.Methods 68 patients with MacTel (61±13 yrs), 41 clinically healthy children of these MacTel patients (MacTel-C, 34±8 yrs) and 30 other family members (siblings, parents, cousins) were investigated at the Moran Eye Center with a prototype Heidelberg FLIO. A healthy control group for the MacTel-C, all of whom exhibited only normal FLIO patterns, was also included. Fundus autofluorescence (FAF) was excited with a 473 nm laser, and fluorescence lifetimes were recorded in short (498-560 nm) and long (560-720 nm) spectral channels. All subjects also underwent OCT, macular pigment, autofluorescence intensity and infrared reflectance images.Results All patients with MacTel show the specific MacTel-related FLIO pattern. Of the 41 investigated MacTel- C with clinically normal eye exams and conventional imaging, 16 subjects (39%) show a FLIO pattern consistent with early stages of MacTel. FAF lifetimes were significantly (p<0.001) prolonged across all regions of the fundus in FLIO-positive compared to FLIO-negative MacTel-C. This was most significant superior to the fovea (FLIO-positive 246 ps vs. FLIO- negative 207 ps).Conclusions FLIO detects characteristic changes in the FLIO patterns in MacTel with high contrast. 39% of clinically unaffected children of MacTel show FLIO-patterns indicative of early stage MacTel. 19% of siblings typically manifest disease – in contrast, we have found such changes at twice that rate. FLIO is able to show abnormal patterns in some individuals who may carry a MacTel gene but show no signs of disease (e.g. some parents of MacTel patients). This indicates the very high sensitivity of the FLIO modality to detect MacTel. FLIO could help to diagnose MacTel even before conventional imaging modalities show changes and before patients experience visual disturbances.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Fluorescence Lifetime Imaging Ophthalmology (FLIO) is a novel imaging modality that offers new perspectives and understanding of retinal diseases. FLIO produces promising diagnostic outcomes by detecting disease-related changes at a high contrast and sensitivity. This may potentially help clinicians diagnose and treat diseases early on. By utilizing FLIO in clinical research, diseases that were once considered synonymous and difficult to delineate have become easier to differentiate based on their respective FLIO signals. FLIO continues to revolutionize the way clinical scientists approach complex diseases like Macular Telangiectasia Type 2 (MacTel) and offers a unique approach to understanding metabolic changes in the eye throughout disease progression. Examinations in Asian Patients with Hydroxychloroquine Retinopathy

Posterboard#: A0490

Abstract Number: 5027 - A0490

AuthorBlock: Seong Joon Ahn1, Byung Ro Lee1 1Ophthalmology, Hanyang University Hospital, Seoul, Korea (the Democratic People's Republic of);

DisclosureBlock: Seong Joon Ahn, None; Byung Ro Lee, None;

Purpose To report findings on visual field (VF) examinations using different protocols in Asian patients with hydroxychloroquine (HCQ) retinopathy and to compare the diagnostic capabilities among the VF protocolsMethods Nine-hundred six patients with rheumatologic conditions were screened for HCQ retinopathy, using optical coherence tomography, fundus autofluorescence, and automated VF examinations. By excluding eyes with glaucoma or combined macular diseases, a total of 1651 eyes in 860 patients taking HCQ with reliable VF results were analyzed for this study. Results from Humphrey VF obtained using 10-2 and 30-2 protocols and those using full-field 120 (FF-120) protocol in selected patients for screening tests were reviewed. Visual field abnormality and patterns were evaluated for each protocol of VF tests (Figure 1). In the patients with reliable 30-2 and 10-2 results, sensitivities and specificities based on the abnormal VF findings were compared between the protocols. Visual field progression was evaluated by comparing VF results between visits.Results Sensitivity and specificity of 10-2 protocol were 75% and 76.5%, respectively, whereas 30-2 showed 92.5% sensitivity and 76.0% specificity. Most common pattern in Asian patients with HCQ retinopathy was constricted VF and paracentral ring scotoma on 10-2 and 30-2 tests, respectively. Atypical VF patterns included bitemporal and nasal step. FF-120 showed 89.3% sensitivity and 63.5% specificity; however, 2 patients with early retinopathy were diagnosed with HCQ reitnopathy by VF abnormality identified only on FF-120. Comparison between 10-2 and 30-2 resulted in significant difference in sensitivity (P=0.010). Particularly, there was significant difference in the sensitivities between the two protocols in eyes with pericentral pattern (P=0.003) and those with early retinopathy (P=0.011). Eyes with severe retinopathy showed progression after drug cessation and 10-2 protocol was useful for evaluation of visual field progression as structural progression correlated with field constriction in the eyes.Conclusions VF 30-2 can be performed for the primary VF test for HCQ retinopathy screening in Asian patients. Additional protocols such as 10-2 and FF120 can be performed according to the location of photoreceptor defects. Visual field progression is important for evaluation of retinopathy progression, particularly in eyes with severe retinopathy.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Hydroxychloroquine (HCQ) is widely used for treatment of several rheumatologic and dermatologic diseases. It is well-known that long-term use of HCQ may lead to retinal toxicity, called HCQ retinopathy, characterized by progressive, irreversible photoreceptor and/or retinal pigment epithelium damage. Interestingly, pattern of HCQ retinopathy varies according to ethnicity and variability of the pattern significantly affect detection of retinopathy. Due to different pattern of retinopathy in Asian patients, these patients require careful selection of visual field (VF) examination, one of the two primary tests for HCQ retinopathy screening. We reported different pattern of VF defects in Asian patients and compared sensitivities among several VF protocols. We proposed 30-2 protocol as the primary screening VF test for Asian patients and suggested VF protocols for eyes suspicious of HCQ retinopathy personalized to the pattern of retinopathy. Genetic factors associated with the treatment response to reduced-fluence photodynamic therapy for chronic central serous chorioretinopathy

Posterboard#: A0491

Abstract Number: 5028 - A0491

AuthorBlock: Akiko Miki1, Mayuka Hayashida1, Shunichiro Nakai1, Wataru Matsumiya1, Hisanori Imai1, Sentaro Kusuhara1, Shigeru Honda2, Makoto Nakamura1 1ophthalmology, Kobe University Graduate School of Medicine, Kobe, HYOGO, Japan; 2ophthalmology, Osaka city university, , Japan;

DisclosureBlock: Akiko Miki, None; Mayuka Hayashida, None; Shunichiro Nakai, None; Wataru Matsumiya, None; Hisanori Imai, None; Sentaro Kusuhara, kobe university Code P (Patent), Shigeru Honda, None; Makoto Nakamura, None;

Purpose To investigate genetic factors associated with the treatment response to reduced-fluence photodynamic therapy (RFPDT) for chronic central serous chorioretinopathy (cCSC).Methods This was a retrospective study of 85 eyes from 85 patients with cCSC who underwent RFPDT and were followed up for more than 12 months. Patients were divided into good response group (54 patients in group A) and poor response group (31 patients in group B) based on either persistence or recurrence of subretinal fluid detected by spectral domain optical coherence tomography after first RFPDT. Genotyping of four single nucleotide polymorphisms (SNPs), including age-related maculopathy susceptibility (ARMS2) A69S (rs10490924), complement factor H (CFH) I62V (rs800292), CFH Y402H (rs1061170) and CFH (rs1329428) was conducted using TaqMan technology.Results There were no significant differences in baseline characteristics including sex, age and best corrected logMAR visual acuity between groups. However, there was a significant difference in T-allele frequency in ARMS2 A69S (rs10490924) between groups (24.5% in group A, 40.3% in group B, p=0.032).Conclusions ARMS2 variants might be associated with the treatment response of RFPDT in cCSC patients.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Photodynamic therapy for central serous chorioretinopathy with choroidal neovascularization on optical coherence tomography angiography combined with intravitreal anti- vascular endothelial growth factor therapy or alone

Posterboard#: A0467

Abstract Number: 5004 - A0467

AuthorBlock: Nao Sonoda1, Takashi Araki1, Yuki Komuku1, Hisashi Iwami1,2, Fumi Gomi1 1Hyogo College of Medicine, Nishinomiya, Hyogo prefecture, Japan; 2Iwami Eye Clinic, , Japan;

DisclosureBlock: Nao Sonoda, None; Takashi Araki, None; Yuki Komuku, None; Hisashi Iwami, None; Fumi Gomi, BAYER Code R (Recipient), Alcon pharma Code R (Recipient)

Purpose To investigate the efficacy of photodynamic therapy (PDT) combined with intravitreal anti-vascular endothelial growth factor (VEGF) therapy or alone for central serous chorioretinopathy (CSC) with choroidal neovascularization (CNV) by optical coherence tomography angiography (OCTA).Methods A total of 27 eyes of 27 patients (18 men / 9 women, mean age 63.3 ± 10.3 years) with CSC and CNV, treated and followed for 3 months or more between June 2016 and May 2018 at Hyogo College of Medicine were examined. Patients with CSC were identified by fluorescein and indocyanine green angiography, and network structure in the outer retina and choriocapillaris was determined by OCTA. Sixteen (16) patients underwent PDT alone (the PDT group) and 11 underwent PDT combined with intravitreal anti-VEGF therapy within 3 days beforehand (the combination group). We analyzed best corrected visual acuity (BCVA), changes in subretinal fluid, central retinal thickness, subfoveal choroidal thickness, and CNV area before PDT and 1, 3, and 6 months after treatment for each single- treatment group and the combination group.Results For the PDT group, logMAR BCVA did not change significantly after treatment. In the combination group, logMAR BCVA improved significantly at 3 and 6 months from baseline (p = 0.043 and p = 0.012). Subretinal fluid was resolved in 62.5% of the cases at 1 month after treatment, 75.0% at 3 months, and 58.3% at 6 months for the PDT group. For the combination group, the rate was 72.7% at 1 month, 72.7% at 3 months, and 66.7% at 6 months. Mean central retinal thickness was significantly reduced at 1, 3 and 6 months for the PDT group and for the combination group. Subfoveal choroidal thickness was significantly reduced at 3 and 6 months for the PDT group (p = 0.0002 and p = 0.021), and at 1, 3 and 6 months for the combination group (p = 0.001, p = 0.001,and p = 0.008). The total CNV area did not significantly change in the PDT group. In the combination group, it was significantly decreased at 1 and 3 months (p = 0.023 and p = 0.037).Conclusions PDT combined with anti-VEGF therapy or alone for CSC with CNV significantly improved central retinal thickness and subretinal fluid amounts, but anti-VEGF therapy is necessary to decrease the CNV area.Layman Abstract (optional): Provide a 50-200 word description of your work that non- scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. A changeable flap: clinical observation of macular reconstruction after implementation of autologous neurosensory retinal free flap technique(ANRFFT).

Posterboard#: A0492

Abstract Number: 5029 - A0492

AuthorBlock: Zhaohui Li1, Yanzi Li1, Jianhua Wu1 1vitreous & retina diseases of Wuhan Ai'er Eye Hospital, Ai'er ophthalmic college, Central-South University, Changsha, Hunan, China;

DisclosureBlock: Zhaohui Li, None; Yanzi Li, None; Jianhua Wu, None;

Purpose To observethe anatomical and functional reconstruction of the macular, especially for positional and morphological changes of the flap，after implementation of ANRFFT for recurrent macular hole retinal detachment (MHRD) in patients with highly myopic eyes.Methods 10 eyes with recurrent MHRD in patients with highly myopia were retrospectively reviewed. All eyes had undergone PPV with ANRFFT. The main outcome measures contain the macular hole closure and retinal reattachment rate, the thickness and sensitivity threshold of the flap, the blood flow signal and position change of the flap, the sensitivity threshold and fixation ability of macula area, best corrected visual acuity (BCVA).Results At the last follow-up, “Closed MH” were in 7 eyes (70%). The retina reattached in 8 eyes (88.9%) after the silicone oil extraction surgery. “No shift” of the flap appeared in 7 eyes (70%). The mean flap thickness at 1 month was 220.86±31.57μm, and it decreased significantly to 175.71±23.56μm at 6 months after surgery(P=0.005, paired t-test ). The mean macular sensitivity threshold at 6 months (11.04±3.02 dB) was significantly higher than 1 month (6.28±2.23dB) (P=0.000, paired t-test ). There was no blood flow signal either superficial or deep retina layer of the flap. The post-BCVA (mean,1.32±0.31 log MAR ) had a significant improvement compared with pre- BCVA(mean,2.06±0.33log MAR) (P＜0.05 , paired t-test).Conclusions ANRFFT appeared safe and effective in facilitating macular hole closure and retinal reattachment, thus achieved the visual improvements in highly myopic eyes with recurrent MHRD, In the course of macular reconstruction, the flap shifts slightly and thins gradually.The study also suggested the flap adhere and fuse to the neurosensory retinal layer besides coupling of pigment epithelium which maybe is the mechanism of MH closure and retinal reattachment.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details.

Characterizing two novel mouse models of Ctrp5/C1qtnf5 to understand disease mechanism in late-onset retinal degeneration.

Posterboard#: A0493

Abstract Number: 5030 - A0493

AuthorBlock: Shyamanga Borooah1, Marina voronchikhina1, Anil Kumar Chekuri1, Venkata Ramana Murthy Chavali2, John Suk1, Akhila Alapati1, Dirk-Uwe G. Bartsch1, Naheed W. Khan4, Monica M. Jablonski3, Radha Ayyagari1 1Ophthalmology, University of California San Diego, San diego, California, United States; 2University of Pennsylvania, Pennsylvania, United States; 3University of Tennessee, Tennessee, United States; 4University of Michigan, Michigan, United States;

DisclosureBlock: Shyamanga Borooah, None; Marina voronchikhina, None; Anil Kumar Chekuri, None; Venkata Ramana Murthy Chavali, None; John Suk, None; Akhila Alapati, None; Dirk-Uwe G. Bartsch, None; Naheed W. Khan, None; Monica M. Jablonski, None; Radha Ayyagari, None;

Purpose Late Onset Retinal Degeneration (L-ORD) is an autosomal dominant macular dystrophy which results from mutations in Ctrp5/C1qtnf5. A mouse model (Ctrp5+/-) for the most common S163R mutation has previously been described. It is still unclear whether L-ORD results from a gain or loss of function. We seek to resolve this by studying two novel mouse models with homozygous knock-in (S163R) (Ctrp5+/+) and homozygous Ctrp5 knock-out (Ctrp5-/-) genotypes.Methods The Ctrp5+/+ mice were bred from the Ctrp5+/- mice whilst the Ctrp5-/- mice were created using Cre mediated homologous recombination. Their genotypes were confirmed by sequencing. Both models were rd1 and rd8 mutation free. Autofluorescence scanning laser ophthalmoscopy, spectral domain optical coherence tomography and electroretinography (ERG) were used for phenotyping at 20 months (mo) of age (n=6). Retinal phenotypes were studied using light microscopic and ultrastructural analysis at 4.5, 6, 11 and 18.5 mo (n=3) in Ctrp5-/- mice and at 13, 16 and 20 mo in Ctrp5+/+ mice (n=3). We performed histological analysis for cone opsin counts and CTRP5 and studied deposits using lipid and cholesterol markers Oil red O, Plin2 and Filipin staining using 18-20 mo eyecups (n=3 for each). The findings were compared with age-matched wild-type (WT) and Ctrp5+/- mice eyecups.Results Autofluorescent fundus spots were significantly greater in Ctrp5-/- and Ctrp5+/+ mice than in WT (P<0.0001). ERG also demonstrated a significant reduction in photopic and scotopic response in both new models compared with WT. Light microscopy showed increased RPE stress with vacuolization and thinning as early as 6 mo in Ctrp5-/- mice and 13 mo in Ctrp5+/+ mice. Ultrastructural analysis revealed the presence of sub-RPE deposits in both new models but histology failed to show any significant differences in lipid or cholesterol markers when compared with WT and Ctrp5+/- mice. Additionally, a sub-RPE accumulation of CTRP5 was noted only in Ctrp5+/- mice.Conclusions In theses studies we found that Ctrp5+/+ mice shared retinal pathology with Ctrp5-/- mice. Additionally, the electrophysiological and ultrastructural findings in Ctrp5-/- mice are similar to those previously reported in Ctrp5+/- mice. This suggests that pathology in L-ORD mouse model (Ctrp5+/-) results from a loss of CTRP5 function.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Late-onset retinal degeneration (L-ORD) is an inherited macular degeneration caused by a mutation in a gene known as CTRP5. The disease shares many features with age-related macular degeneration (AMD) and so is a good model for AMD. A better understanding of the causes of L-ORD may also therefore provide a clue to the causes of AMD. In this study we look to see if disease is caused by the new mutation itself or the loss of normal gene function which is secondary to the mutation. This will help us understand what causes L-ORD and how to design strategies to treat L-ORD. Dark adaptometry and dark-adapted two-color microperimetry in patients with Pseudoxanthoma elasticum

Posterboard#: A0494

Abstract Number: 5031 - A0494

AuthorBlock: Kristina Hess1, Martin Gliem2,1, Maximilian Pfau1, Philipp L. Mueller1, Johannes Birtel1, Frank G. Holz1, Peter Charbel Issa2, Philipp Herrmann1 1Department of Ophthalmology, University of Bonn, Bonn, Germany; 2University of Oxford, Nuffield Laboratory of Ophthalmology, Oxford, United Kingdom;

DisclosureBlock: Kristina Hess, Carl Zeiss MediTec Code F (Financial Support), CenterVue Code F (Financial Support), Heidelberg Engineering Code F (Financial Support), Optos Code F (Financial Support), Martin Gliem, Carl Zeiss MediTec Code F (Financial Support), CenterVue Code F (Financial Support), Heidelberg Engineering Code F (Financial Support), Optos Code F (Financial Support), Heidelberg Engineering Code R (Recipient), Bayer Code R (Recipient), Maximilian Pfau, Carl Zeiss MediTec Code F (Financial Support), Heidelberg Engineering Code F (Financial Support), CenterVue Code F (Financial Support), Optos Code F (Financial Support), Novartis Code R (Recipient), Philipp L. Mueller, Carl Zeiss MediTec Code F (Financial Support), CenterVue Code F (Financial Support), Heidelberg Engineering Code F (Financial Support), Optos Code F (Financial Support), Johannes Birtel, Optos Code F (Financial Support), Heidelberg Engineering Code F (Financial Support), CenterVue Code F (Financial Support), Carl Zeiss MediTec Code F (Financial Support), Frank G. Holz, Acucela Code C (Consultant), Allergan Code R (Recipient), Bayer Code C (Consultant), Bioeq Code C (Consultant), Boehringer-Ingelheim Code C (Consultant), Carl Zeiss MediTec Code R (Recipient), CenterVue Code F (Financial Support), Genentech/Roche Code F (Financial Support), Heidelberg Engineering Code F (Financial Support), NightstarX Code F (Financial Support), Novartis Code F (Financial Support), Optos Code F (Financial Support), Thea Code C (Consultant), Acucela Code F (Financial Support), Allergan Code F (Financial Support), Bayer Code F (Financial Support), Bayer Code R (Recipient), Bioeq Code F (Financial Support), Carl Zeiss MediTec Code F (Financial Support), Graybug Vision Code C (Consultant), Lin BioScience Code C (Consultant), Pixium vision Code C (Consultant), Oxurion Code C (Consultant), Stealth biosciences Code C (Consultant), Genentech/Roche Code R (Recipient), Peter Charbel Issa, Heidelberg Engineering Code F (Financial Support), Philipp Herrmann, Carl Zeiss MediTec Code F (Financial Support), CenterVue Code F (Financial Support), Heidelberg Engineering Code F (Financial Support), Optos Code F (Financial Support)

Purpose To investigate the impact of pathologically altered Bruch’s membrane on dark adaption and low- luminance conditions in patients with Pseudoxanthoma elasticum (PXE).Methods Dark adaptation was measured after bleaching (10 minutes, 1600 apostilb) with a 11° stimulus in 35 PXE patients and 35 age-matched controls (Goldmann-Weekers Adaptometer, Haag-Streit, Switzerland). Low luminance deficit and contrast sensitivity were assessed in the same cohorts. In addition, mesopic and dark-adapted two-color (cyan [505 nm] and red [627 nm]) fundus-controlled microperimetry (10-2 test grid, Goldmann III stimuli; S-MAIA, CenterVue; italy) was performed in 20 eyes of 20 PXE patients and compared to age-adjusted normative data in a point-wise manner. The results were analyzed using linear regression and t-tests.Results PXE-eyes (age 49.1 ±11.7 years) exhibited an overall prolonged dark adaptation with high interindividual differences compared to controls (52.2 ±12.2 years). The time to adaptation of 4 log Units was significantly longer in PXE-Patients compared with controls (20.1±8.6 vs. 7.7 ±1.3 minutes, p<0.001). Higher low luminance deficit (mean 6.9 ± SD 4.5 ETDRS letters vs. 14.2 ± 7.5 EDTRS letters) and lower contrast sensitivity ((1.31 ±0.36 vs. 1.96 ±0.16 [logUnits]) compared with controls were measured (p<0.001 respectively). On microperimetry, PXE patients exhibited significant dysfunction compared to age-adjusted normative data with average sensitivity losses of -4.79±3.54 dB for mesopic, -3.81± 5.95 dB for dark-adapted cyan and -5.33 ± 4.32 dB for dark-adapted red testing (p<0.001 respectively). Point-wise analysis revealed that that mesopic sensitivity loss was mostly observable for test-points exhibiting dark-adapted cyan sensitivity losses of -14 dB or greater.Conclusions The findings indicate that patients with PXE exhibit prolonged dark adaptation kinetics and generally reduced visual function in low ambient light conditions. Impaired permeability of Bruch’s membrane for retinoid cycle intermediates may contribute to these impairmants, while the exact cause currently remains to be determined. Since Bruch’s membrane aging is a pathogenetic factor in age-related macular degeneration (AMD), these findings could add to the understanding of the pathophysiology in AMD.Layman Abstract (optional): Provide a 50-200 word description of your work that non- scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Patients with Pseudoxanthoma elasticum (PXE) exhibit calcifications of elastic fibres and therefore develop cardiovascular disorders, skin alterations and ophthalmologic impairments. Elastic fibres are highly concentrated in the Bruch’s membrane (BrM) of the retina, likely leading to a reduced diffusion of metabolites from the blood stream across Bruch’s membrane towards the photoreceptors in PXE. This study demonstrates the strategic role of the BrM and the significance of functional deficits when impermeability occurs. Likely the impaired BrM leads to a local deficit of metabolites at the site of the photoreceptors, leading to prolonged dark adaptation and low luminance function deficits. In age- related macular degeneration, the leading cause of blindness in developed countries, BrM-alterations are one of the contributing factors in the multifactor pathophysiology. Understanding the impact of BrM changes could lead to better understanding of the importance of the respective factors. Hydroxychloroquine therapy: Effects on retinal function and structure

Posterboard#: A0495

Abstract Number: 5032 - A0495

AuthorBlock: Luz Amaro-Quireza1, Stephen Tsang1, Donald C. Hood1, Vivienne C. Greenstein1 1Ophthalmology, Columbia University, New York, New York, United States;

DisclosureBlock: Luz Amaro-Quireza, None; Stephen Tsang, None; Donald C. Hood, Topcon Inc Code F (Financial Support), Topcon Inc Code C (Consultant), Topcon Inc Code R (Recipient), Heidelberg Eng Code F (Financial Support), Heidelberg Eng Code C (Consultant), Heidelberg Eng Code R (Recipient), Novartis Code F (Financial Support), Novartis Code R (Recipient), Novartis Code C (Consultant), Vivienne C. Greenstein, None;

Purpose To evaluate the effects of hydroxychloroquine (HCQ) therapy on the retina by comparing the results of functional and structural measures.Methods 39 patients (10-79 years of age) on HCQ had a complete ophthalmologic examination and the following recommended screening tests for HCQ retinopathy: 10-2 automated visual fields (VFs: Humphrey CZM); spectral domain optical coherence tomography volume and high resolution horizontal line scans (OCT: Spectralis Heidelberg Eng); short-wavelength fundus autofluorescence (SW-AF) and mfERGs (Diagnosys LLC). (1) For the mfERG, a scaled103 hexagon array was used and response densities of the first order kernel were analyzed by grouping the 103 responses into six concentric rings and calculating ring ratios using the R5 ring response as the “ reference ring “ and dividing by all other ring response amplitudes (R1-R6). (2) The ratios of the R5 to each of the other rings were compared to 95% CIs from control eyes.Results Of the 39 patients, 23 had been treated with HCQ for ≥ 5 years with a cumulative dose > 1000g. 14 of the patients had functional damage as seen by either mfERG and/or VF abnormalities. In particular, 11 of the patients had abnormal mfERG ring ratios and 10 had abnormal VFs. A comparison of the mfERG and VF results showed that of the 11 patients with abnormal ring ratios, 7 had partial or complete ring scotomas. These were more pronounced in the superior field. Fewer eyes showed structural damage. In particular, outer retinal abnormalities on OCT were observed in 8 patients and were associated with abnormal VFs. The outer retinal abnormalities ranged from disruption of the inner/outer segment junction and cone outer segment interdigitation zone in the parafoveal region, to thinning of the outer nuclear layer to atrophy of the retinal pigment epithelium in the central macular area. They were more prevalent in the inferior retinal scans. Only 5 of the 8 patients with outer retinal abnormalities showed signs of abnormal SW-AF in the central macula.Conclusions With HCQ therapy, functional deficits were more frequent than structural or morphological deficits. Also the inferior retina, in addition to the pericentral region, appeared to be more vulnerable to the toxic effects of HCQ. 1. Marmor MF et al. Ophthalmology 2016. 2. Adam MK et al. BJO 2012.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. North Carolina Macular Dystrophy (NCMD): 50 year follow up of the original family

Posterboard#: A0496

Abstract Number: 5033 - A0496

AuthorBlock: Fadi Shaya1,2, Robert Wiggins3, Jessica Avetisjan1,2, Kent W. Small1,2 1Macule and Retina Institute, Glendale, California, United States; 2Molecular Insight Research Foundation, Los Angeles and Glendale, California, United States; 3Asheville Eye Associates, Asheville, North Carolina, United States;

DisclosureBlock: Fadi Shaya, None; Robert Wiggins, None; Jessica Avetisjan, None; Kent W. Small, None;

Purpose The clinical phenotype of NCMD is highly variable and remains poorly appreciated and understood. One of the features of NCMD is the lack of progression in an individual despite its original name by Lefler, Wadsworth and Sidbury who named it “dominant progressive foveal dystrophy” as reported in 1971. We reexamined 25 of the original NCMD family 30-50 years after first being examined by one of us (Kent Small).Methods Dr. Kent Small (KS) examined twenty-five individuals of the original NCMD pedigree during a recent field in an office setting. Standard Snellen visual acuity, slit lamp and fundus examinations were performed by KS. Fundus photography was performed with an OPTOS California camera (Marlborough, MA) and SD-OCT was performed with Zeiss Cirrus 5000 (Oberkochen, Germany). These findings were compared with the original data collected by KS in 1988. SD-OCT was not available during the original ascertainment of the family in 1988. Blood was collected for DNA.Results The 25 subjects examined were part of the original NCMD family, which we previously found the point mutation CHR6: 99593030 (Hg38) in a non-coding region of a DNASE1 hypersensitivity binding site on chromosome 6. Eight were affected children of those originally examined 30 years ago by KS and were not yet born in 1988 at the time of the original ascertainment. The remaining 17 subjects (34 eyes) had been examined 30 years previously. Dr. Lefler had examined six of these subjects 50 years previously. Of these, 4 eyes showed worsening of vision with fundus photos showing evidence of fibrosis from choroidal neovascularization some with surrounding atrophy suggesting previously resolved subretinal fluid. One showed improvement in visual acuity for unexplainable reasons.Conclusions Most NCMD patients have stable vision and fundus findings throughout their lives even up to 50 years follow-up. The ones who lost vision did so with grade 2 disease, which developed choroidal neovascularization that spread or leaked into the nasal maculae. With anti-VEGFs now available, some of these patients may benefit from these treatments.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. This abstract demonstrates the non-progressive nature of North Carolina Macular Dystrophy (NCMD). It also provides evidence into how patients with grade 2 NCMD can experience vision decline as a result of choriodal neovascularization. Quantification of residual ellipsoid zone in patients with cone-rod dystrophy

Posterboard#: A0497

Abstract Number: 5034 - A0497

AuthorBlock: Takumi Hara1, Tatsuya Inoue1, Marie Kitano1, Han Peng Zhou1, So Makino1, Ryo Obata1 1The University of Tokyo Hospital, Tokyo, Japan;

DisclosureBlock: Takumi Hara, None; Tatsuya Inoue, None; Marie Kitano, None; Han Peng Zhou, None; So Makino, None; Ryo Obata, None;

Purpose Cone-rod dystrophy (CRD) is a retinal inherited disorder that results in central vision loss and decreased visual acuity. To evaluate the status of cone photoreceptors in CRD, visual acuity(VA) measurement, visual field tests, electroretinogram and optical coherence tomography (OCT) are usually performed. Recently, we identified the quantification method of residual ellipsoid zone (rEZ) in resolved central serous chorioretinopathy. The aim of our current study is to quantify the index of rEZ and to investigate the correlation between OCT parameters and visual functions in patients with CRD.Methods We examined 20 eyes of 10 patients (7 males and 3 females). All the eyes were diagnosed as CRD and tested with the Humphrey Visual Field Analyzer (HFA 10-2 program; Carl Zeiss Meditec, USA). We obtained spectral domain OCT (SD-OCT) images using the Spectralis OCT (Heidelberg Engineering, Heidelberg, Germany). For each eye, the area of decreased EZ intensity was identified (EZa). OCT images were then converted into binary images using the Niblack method. With the binarized images, the percentage of the residual EZ for each eye (3x3-mm area at the macula) was analyzed (rEZ). The rEZ index for each eye was measured by two investigators and intraclass correlation coefficient (ICC) was calculated to clarify interrator reproducibility. Central retinal thickness (CRT), and central choroidal thickness (CCT) were also measured. The correlation between OCT parameters and visual functions was investigated.Results The mean age of the patients was 51.2±18.1 years and the mean logMAR VA was 0.51±0.52. The mean MD value was -17.8±12.7 dB. The mean CRT and CCT were 131.8±59.7μm and 245.3±72.9μm, respectively. The mean EZa and rEZ were 22.4±24.0μm2 and 42.4±30.7%, respectively. The ICC of each rEZ index was 0.924 (95% confidence interval: 0.82

Posterboard#: A0498

Abstract Number: 5035 - A0498

AuthorBlock: Kumiko Hirayama1, Manabu Yamamoto1, Takeya Kohno1, Dirk Theisen-Kunde2, Ralf Brinkmann2,3, Yoko Miura2,3, Shigeru Honda1 1Ophthal & Visual Sciences, Osaka City Univ Grad Sch of Med, Osaka-shi, OSAKA, Japan; 2Medical Laser Center Luebeck, , Germany; 3Institute of Biomedical Optics, University of Luebeck,, , Germany;

DisclosureBlock: Kumiko Hirayama, None; Manabu Yamamoto, None; Takeya Kohno, None; Dirk Theisen-Kunde, None; Ralf Brinkmann, None; Yoko Miura, None; Shigeru Honda, None;

Purpose Tilted disc syndrome (TDS) may be associated with a macular serous retinal detachment (SRD). However, ideal therapy for this complication is still unestablished yet to date. The purpose of this study is to investigate the effect of selective retina therapy (SRT) for macular SRD associated with TDS.Methods This retrospective study included 10 eyes of 9 patients (9 females), who were treated with SRT for SRD associated with TDS at the department of Ophthalmology of Osaka City University Hospital, and observed at least 12 months after treatment. The mean age was 56 years old (range 44-66). SRT laser (527 nm, 1.7 µs, 100 Hz; Medical Laser Center Lübeck, Germany) was used for treatment, and was irradiated at and around the leakage point assessed with fluorescein angiography. The changes of best corrected visual acuity (BCVA) and central macular thickness (CMT) in optical coherence tomography (OCT) were examined. The BCVA was measured by Landolt C chart and converted to logarithm of the minimum angle of resolution (logMAR) for calculation.Results The mean follow-up period was 24.4 months (range 12-48 months). The mean BCVA and CMT changed from 0.03 ± 0.11 and 328 ± 86 µm preoperatively to 0.08 ± 0.17 and 293 ± 99 µm at final follow-up, respectively, with no significant difference (BCVA: p=0.26, CMT: p=0.24). The SRD disappeared in 5 eyes (50%) and the average period between initial treatment and resolution of SRD was 11 months (range 2-18 months). The average number of SRT irradiation until resolution of SRD was 2.6 times (range 1-5 times). In the other 5 eyes, SRD was decreased after every SRT but didn't disappear by multiple treatments.Conclusions SRT may promote absorption of subretinal fluid and maintenance of BCVA for TDS. The resolution of SRD was achieved in half of the cases.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Evaluation of patients with Stargardt disease by Microperimetry and Fundus Autofluorescence: identification of a new biomarker in defining the natural history of disease

Posterboard#: A0499

Abstract Number: 5036 - A0499

AuthorBlock: Valentina Di lorio1, Ada Orrico1, Raffaella Brunetti-Pierri1, Mariaelena Filippelli1, Paolo Melillo1, Anna Nesti1, Settimio Rossi1, Alberto Auricchio2,3, Francesca Simonelli1, Francesco Testa1 1Eye Clinic, Multidisciplinary Department of Medical, Surgical and Dental Sciences, University of Campania Luigi Vanvitelli, Naples, Italy; 2Telethon Institute of Genetics and Medicine,, Pozzuoli, Italy; 3Department of Advanced Biomedicine, University of Naples Federico II, Naples, Italy;

DisclosureBlock: Valentina Di lorio, None; Ada Orrico, None; Raffaella Brunetti-Pierri, None; Mariaelena Filippelli, None; Paolo Melillo, Sanofi Code R (Recipient), Anna Nesti, None; Settimio Rossi, None; Alberto Auricchio, None; Francesca Simonelli, Sanofi Code R (Recipient), Francesco Testa, Sanofi Code R (Recipient)

Purpose We evaluated the progression of Stargardt disease due to ABCA4 mutations (STGD1) using Microperimetry (MP1) and Fundus Autofluorescence (FAF) imaging in order to investigate their capability to detect macular degeneration over a short-term follow-up (1 year).Methods We analyzed data from 57 patients with a clinical and molecular diagnosis of STGD1 (mean age: 33.5±1.8 years; mean disease duration: 13.4±1.3 years), undergoing a baseline and a follow-up visit, including best corrected visual acuity (BCVA), fundus examination, electroretinogram (ERG), FAF and MP1. Fundus and ERG were classified as shown in Fig. 1. To estimate disease progression, we evaluated definitely decreased autofluorescence (DDAF) from FAF images and absolute scotoma area from MP1. The study, approved by the Local Ethics Committee, adhered to the Declaration of Helsinki and each patient gave written informed consent.Results BCVA was 0.67±0.06 LogMAR. Fundus lesions were consistent with Fishman I phenotype in 22 patients (38.6%), with Fishman II phenotype in 17 patients (29.8%), and with Fishman III phenotype in 18 patients (31.6%). ERG responses were: Lois I in 29 patients (50.9%), Lois II in 24 patients (42.1%), and Lois III in 4 patients (7.0%). Absolute scotoma area was 5.54 ± 1.19 mm2, whereas DDAF was 2.78 ± 0.44 mm2. Actually, the absolute scotoma area was significantly greater than the DDAF (p = 0.009). Moreover, we observed a significant (p-value<0,001) correlation of the absolute scotoma area and of DDAF with age and duration of the disease, but not with the age of disease onset. Patients with Fishman III had significantly higher values of DDAF compared to the Fishman I phenotype and patients with Lois III presented areas of absolute scotoma significantly larger than the Lois group I. Finally, the longitudinal analysis showed that absolute scotoma area increased more quickly compared to DDAF (absolute scotoma: 1.41 mm2 / year vs DDAF: 0.20 mm2/year).Conclusions The observation of a wider absolute scotoma area compared to DDAF at baseline and its faster progression suggest that a significant loss in photoreceptor function occurs before the death of retinal pigmented epithelium cells. This study shows that MP1 could be a sensible examination to evaluate retinal degeneration in STGD1 and consequently the effect of experimental treatments.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Clinical and Genetic Characteristic of Pachydrusen in Eyes with Central Serous Chorioretinopathy and Normal Subjects

Posterboard#: A0468

Abstract Number: 5005 - A0468

AuthorBlock: Ayako Takahashi1, Yoshikatsu Hosoda1, Masahiro Miyake1, Akio Oishi1, Sotaro Ooto1, Akitaka Tsujikawa1 1Department of Ophthalmology, Kyoto University Graduate School of Medicine, Kyoto, Japan;

DisclosureBlock: Ayako Takahashi, Bayer Code R (Recipient), Santen Pharmaceutical Co. Code R (Recipient), Yoshikatsu Hosoda, Wakamoto Pharmaceutical Co. Code F (Financial Support), Masahiro Miyake, Wakamoto Pharmaceutical Co. Code R (Recipient), Novartis Pharma K.K. Code R (Recipient), Japan Alcon Code R (Recipient), HOYA Code R (Recipient), Kowa Pharmaceutical Co. Code R (Recipient), Akio Oishi, Novartis Pharma K.K. Code F (Financial Support), Alcon Code F (Financial Support), Novartis Pharma K.K. Code R (Recipient), Alcon Code R (Recipient), Bayer Code R (Recipient), Santen Pharmaceutical Co. Code R (Recipient), HOYA Code R (Recipient), Tokai Optical Co. Code F (Financial Support), Sotaro Ooto, Alcon Code R (Recipient), Bayer Code R (Recipient), Santen Pharmaceutical Co. Code R (Recipient), Senju Pharmaceutical Co. Code R (Recipient), Pfizer Code R (Recipient), Nidek Code R (Recipient), Akitaka Tsujikawa, Novartis Pharma K.K. Code F (Financial Support), Bayer Code F (Financial Support), Santen Pharmaceutical Co. Code F (Financial Support), Canon Code F (Financial Support), Alcon Code R (Recipient), Pfizer Code R (Recipient), Nidek Code R (Recipient), Senju Pharmaceutical Co. Code R (Recipient), Kowa Pharmaceutical Co. Code R (Recipient), HOYA Code R (Recipient), Chugai Pharmaceutical Co. Code R (Recipient), Wakamoto Pharmaceutical Co. Code R (Recipient), Otsuka Pharmaceutical Co. Code R (Recipient), Daiichi Sankyo Company Code R (Recipient), Johnson & Johnson Code R (Recipient), AMO Japan Code R (Recipient), Tomey Corporation Code F (Financial Support), JFC Sales Plan Code F (Financial Support), Alcon Code F (Financial Support), Bayer Code F (Financial Support), HOYA Code F (Financial Support)

Purpose To survey the prevalence and, clinical and genetic characteristic of pachydrusen in eyes with central serous chorioretinopathy (CSC) and normal subjectsMethods Six hundred fourteen eyes of 307 patients with CSC without any history of treatment in either eye, and 1472 eyes of 736 normal Japanese cohort were recruited. Pachydrusen were detected using color fundus photography and subfoveal choroidal thickness was measured using optical coherence tomography images. The genotype distributions with respect to 3 single nucleotide polymorphisms (SNPs) associated with age-related macular degeneration or CSC were evaluated: ARMS2 A69S, CFH I62V and CFH Y402H.Results The prevalence of pachydrusen was significantly higher among CSC patients than that among normal controls (39.7% vs 15.8%, P<0.001). CSC patients with pachydrusen were older than those without pachydrusen (62.2 vs 48.7 years, P<0.001). In the control group, also individuals with pachydrusen were older than those without pachydrusen (70.3 vs 51.8 years, P<0.001). Among both participants with CSC and normal volunteers, the choroidal thickness was greater in those with pachydrusen than in those without pachydrusen (364μm vs 376μm P=0.013, 293μm vs 305μm, P=0.019, respectively; P value was adjusted for age, sex and ). Among CSC patients there was no significant difference in genotype distributions between those with and without pachydrusen. In the normal Japanese cohort, the frequency of the T allele of ARMS2 A69S were higher among individuals with pachydrusen than among those without pachydrusen (42.9% vs 33.9%; P=0.008, OR:1.68, adjusted for age, sex and choroidal thickness), but the A allele frequency in CFH I62V was not significantly different between in those with pachydrusen and without pachydrusen (46.6% vs 39.9%, OR: 1.27, P=0.216, adjusted with age, sex and choroidal thickness).Conclusions Pachydrusen was observed more frequently in CSC patients compared with normal subjects. In normal subjects, pachydrusen was associated with a thicker , and we suggest that pachydrusen should be considered as a significant sign of pachychoroid spectrum. As CFH I62V was not found to be associated with pachydrusen, it may be associated with choroidal thickness rather than with pachydrusen occurrence. We speculate that ARMS2 A69S may be associated with the occurrence of pachydrusen in normal subjects.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. , Metamorphopsia, and Optical Coherence Tomography Parameters in Patients with Idiopathic Epiretinal Membrane

Posterboard#: A0500

Abstract Number: 5037 - A0500

AuthorBlock: DONG EIK LEE1, Un Chul Park1, Hyeong Gon Yu1 1Ophthalmology, Seoul National University, Seoul, Korea (the Republic of);

DisclosureBlock: DONG EIK LEE, None; Un Chul Park, None; Hyeong Gon Yu, None;

Purpose The cause of aniseikonia and metamorphopsia in patients with epiretianl membrane (ERM) remains unknown. We performed a retrospective, observational study to investigate the relationship between the severity of aniseikonia and metamorphopsia and the parameters measured with spectral-domain optical coherence tomography (SD-OCT) in patients with ERM.Methods This study included 110 eyes of 110 patients with idiopathic ERM. We examined the best-corrected visual acuity (BCVA) and the severity of aniseikonia and metamorphopsia using the New Aniseikonia Test and M-CHARTS. Central foveal thickness (CFT), macular volume (MV), and retinal nerve fiber layer (RNFL) symmetry were automatically measured with SD-OCT software.Results RNFL symmetry was significantly correlated with CFT, MV, horizontal and vertical metamorphopsia and aniseikonia (Spearman correlation coefficient: all P < 0.001). CFT was significantly correlated with BCVA, and horizontal and vertical metamorphopsia (Spearman correlation coefficient: P = 0.011, P < 0.001, and P < 0.001, respectively). Multiple regression analysis revealed that the severity of metamorphopsia and aniseikonia significantly related to the RNFL symmetry (all P < 0.001), whereas other parameters were not relevant.Conclusions The severity of metamorphopsia and aniseikonia are associated with RNFL symmetry, but not with CFT in patients with ERM.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Epiretinal membrane (ERM) is a relatively common disease requiring surgery. However, there is no easy and time-saving way to assess the degree of symptoms of ERM. So, our study tried to find a simple and objective parameter which is associated with the symptoms of ERM.

Visual Outcome Following Intravitreal Anti-VEGF Injection In Eyes With Choroidal Neovascular Membrane Secondary to Chronic Central Serous Chorioretinopathy

Posterboard#: A0472

Abstract Number: 5009 - A0472

AuthorBlock: Filipa Rodrigues1, Hagar Khalid2,3, Farid Afshar1, Katrin Fasler4, Giuseppe Casalino2, Magdy Moussa3, Konstantinos Balaskas2, Pearse Andrew Keane2 1Medical Retina Department, Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom; 2NIHR Biomedical Research Centre at Moorfields Eye Hospital NHS Foundation Trust and UCL Institute of Ophthalmology, London, United Kingdom; 3Ophthalmology Department, Faculty of Medicine, Tanta University, Tanta, Egypt; 4Department of Ophthalmology, University Hospital Zurich, Zurich, Switzerland;

DisclosureBlock: Filipa Rodrigues, Novartis Code R (Recipient), Bayer Code R (Recipient), Allergan Code R (Recipient), Hagar Khalid, None; Farid Afshar, None; Katrin Fasler, Alfred Vogt Stipendium Code F (Financial Support), Schweizerischer Fonds zur Verhütung und Bekämpfung der Blindheit Code F (Financial Support), Google Deepmind Code C (Consultant), Novartis Code C (Consultant), Giuseppe Casalino, None; Magdy Moussa, None; Konstantinos Balaskas, Novartis Code R (Recipient), Bayer Code R (Recipient), Allergan Code R (Recipient), Alimera Code R (Recipient), TopCon Code R (Recipient), Heidelberg Code R (Recipient), Novartis Code F (Financial Support), Bayer Code F (Financial Support), Pearse Andrew Keane, Heidelberg Engineering Code R (Recipient), Topcon Code R (Recipient), Carl Zeiss Meditec Code R (Recipient), Haag-Streit Code R (Recipient), Allergan Code R (Recipient), Novartis Code R (Recipient), Bayer Code R (Recipient), Novartis Code S (Non- remunerative), Bayer Code S (Non-remunerative), DeepMind Code C (Consultant), Optos Code C (Consultant)

Purpose To evaluate the long term efficacy of the Intravitreal Anti-VEGF injection on the visual outcome in the treatment of Choroidal Neovascular Membrane (CNV) secondary to Chronic Central Serous Chorioretinopathy (CSCR) and its correlation with the morphological characteristics in Optical Coherence Tomography (OCT).Methods This study included patients recruited retrospectively from Moorfields Eye Hospital Electronic Medical Record system who had received intravitreal Anti-VEGF injection as a monotherapy in treating CNV secondary to CSCR. Patients were treated with either bevacizumab, ranibizumab, or aflibercept, depending on the standard of care. Best corrected visual acuity (ETDRS), Number of injections, and OCT morphological changes were recorded.Results Seventy-eight eyes from 72 patients (63.9 % males) with CNV secondary to chronic CSCR were included in the study with a mean baseline age (66.68 ± 12.86) years. The mean injection number was (12.33 ± 11.65) in a mean follow up period (37.43 ±23.67) months. Of the included patients, 57.69% received aflibercept, 34.62% received bevacizumab, while ranibizumab was injected in 24.36%. Switching between anti-VEGF was recorded in 19.23% of patients. The mean baseline visual acuity was 57.31±17.80 letters, whereas post injection treatment mean visual acuity was 61.92±18.56 letters. There was a significant difference between the pre and post injection (p =0.021). No significant correlation detected between number of injection and visual outcome (p =0.54). Improvement in visual acuity by five letters or more (mean 18.24±12.88 letters) was recorded in 48.72%, while in 24.36% the visual acuity was stable. In 26.92 % visual acuity deteriorated by 5 letters or more (mean -16.11±11.61). of these, EZ disruption was detected in 94.73%, disciform scar was detected in 42.10 %. Intraretinal cystic spaces was detected in 52.63% compared to only 18.42% in whom visual improvement was recorded. No significant correlation between visual outcome and both SRF or SRHM.Conclusions Intravitreal Anti-VEGF injection is effective in treating CNV secondary to CSCR. Visual outcome is influenced by the morphological changes in the structural OCT, in which EZ disruption, disciform scar and intraretinal fluid have poor prognostic index.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Effect and risk of internal limiting membrane peeling for idiopathic epiretinal membrane

Posterboard#: A0463

Abstract Number: 5000 - A0463

AuthorBlock: Kono Ryota1, Yasser Helmy Mohamed1, Yuki Maekawa1, Ai Yoneda1, Hirohumi Kinoshita1, Yoshihisa Yamada1, Eiko Tsuiki1, Azusa Fujikawa1, Takeshi Kumagami1, Takashi Kitaoka1 1Department of Ophthalmology and Visual Sciences, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan;

DisclosureBlock: Kono Ryota, None; Yasser Helmy Mohamed, None; Yuki Maekawa, None; Ai Yoneda, None; Hirohumi Kinoshita, None; Yoshihisa Yamada, None; Eiko Tsuiki, None; Azusa Fujikawa, None; Takeshi Kumagami, None; Takashi Kitaoka, None;

Purpose The purpose of this study was to investigated the functional and anatomical results after vitrectomy for idiopathic epiretinal membrane (ERM) with and without internal limiting membrane (ILM) peeling.Methods In this prospective and comparative study, patients with idiopathic ERM were randomized into ERM removal with or without intentional ILM peeling. Best-corrected visual acuity (BCVA), retinal sensitivity (RS) measured using microperimetry, and central retinal thickness (CRT) measured using optical coherence tomography were evaluated preoperatively and postoperatively at 1, 3, and 6 months.Results After the vitrectomy, ERM was removed with intentional ILM peeling (“with peeling” group). On the other hand, cases of ERM removal followed without intentional ILM peeling was divided into two groups: ILM was stayed in situ (“without peeling” group) and ILM was removed unintentionally with ERM removal (“with” group). Eventually, there were 45 eyes in the “with peeling” group and 9 eyes “without peeling” group. Result of “with peeling” group, BCVA improved (logMAR 0.27±0.18→0.08±0.14, p<0.0001) and CRT decreased (450±72μm→388±41μm, p<0.0001) at 6 months compared to before the vitrectomy. There was no significant difference in RS (15.9±1.9dB→15.6±2.0dB, p=0.86). Result of “without peeling” group, there was no significant difference in BCVA (logMAR 0.40±0.29→0.20±0.24, p=0.21), CRT (453±48μm→400±56μm, p=0.11), RS (13.5±2.2dB→14.6±3.0dB, p=0.69) at 6 months compared to before the vitrectomy. Comparison between both groups, there was no significant difference in BCVA, RS, and CRT at 6 months. ERM postoperative recurrence was observed in one case (2.2%) in “with peeling” group and two cases (22.2%) in “without peeling” group.Conclusions After vitrectomy for idiopathic ERM, ILM peeling improve visual acuity, retinal thickness, and reduce the risk of ERM recurrence, but not improve retinal sensitivity.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Fundus autofluorescence in macular telangiectasia type 2

Posterboard#: A0464

Abstract Number: 5001 - A0464

AuthorBlock: Peter Charbel Issa1, Laurenz Pauleikhoff1, Tjebo Heeren2, Konstantinos Balaskas2, Catherine A. Egan2 1Department of Ophthalmology, Oxford Eye Hospital, University of Oxford, Oxford, ENGLAND, United Kingdom; 2Moorfields Eye Hospital, London, United Kingdom;

DisclosureBlock: Peter Charbel Issa, Heidelberg Engineering Code F (Financial Support), Laurenz Pauleikhoff, None; Tjebo Heeren, None; Konstantinos Balaskas, None; Catherine A. Egan, None;

Purpose Macular telangiectasia type 2 (MacTel type 2) is a binocular disease with characteristic vascular and degenerative changes of the macula, and a disease epicentre temporal to the foveal centre. Multiple imaging modalities aiding the diagnosis of MacTel type 2 have been studied. The role of fundus autofluorescence (AF) imaging has not yet been investigated in detail. The aim of this study was to analyze AF characteristics in a large cohort of patients with MacTel type 2Methods Fundus AF images of 757 eyes (395 patients) from the Moorfields Reading Centre database of the MacTel Natural History Observation and Registry Study were analyzed. Graded features included 1) presence of macular AF changes, 2) increased, mixed or decreased AF signal of the nasal and temporal half graded individually, 3) temporal-nasal asymmetry of AF changes, and 4) visibility of vascular changes such as blunted vessels or ectatic capillaries. In a subset of 200 eyes, fundus AF was analysed in combination with other imaging modalities in order to identify and describe alterations associated with macular AF changes.Results The vast majority of eyes (742/757; 98%) showed macular AF changes. The most common change was an increase in AF (86% nasally, 61% temporally). In 95% of all cases (717/757), macular AF changes were more pronounced temporal compared to nasal to the foveal centre. Image quality allowed assessment of the vascular changes including macular capillaries in 697 eyes and revealed vascular changes in 79% (548). The most common cause of increased AF were macular pigment- related phenomena: the characteristic loss of macular pigment results in a loss of the normal foveal AF-masking and thus an apparently increased AF signal. Similar loss-of masking effects were found in areas of photoreceptor atrophy (loss of masking due to lack of photopigment), or lack of retinal tissue in areas of hyporeflective spaces. Decreased macular AF was frequently caused by pigment plaques or neovascular membranes, and was only rarely due to an isolated atrophy of the retinal pigment epithelium. Eyes that did not show any changes on AF images did not show any obvious changes on optical coherence tomography either.Conclusions The study demonstrated that almost all registry study patient with MacTel type 2 showed characteristic findings on fundus AF imaging, underlining the importance of AF imaging as a diagnostic criterion and for determining disease state in family members.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Long-term Outcomes of Half-dose Photodynamic Therapy in Chronic Central Serous Chorioretinopathy

Posterboard#: A0465

Abstract Number: 5002 - A0465

AuthorBlock: Zhihang Cheng1, Salma Babiker1, Pauline Lenfestey1, Nick Beare1, Ian A. Pearce1 1St Paul's Eye Unit, Royal Liverpool University Hospital, Liverpool, United Kingdom;

DisclosureBlock: Zhihang Cheng, None; Salma Babiker, None; Pauline Lenfestey, None; Nick Beare, None; Ian A. Pearce, None;

Purpose Half-dose verteporfin photodynamic therapy (half-dose PDT) is an established treatment for chronic central serous chorioretinopathy (CSCR), with well known short-term visual benefits. However there is limited data in the long-term benefits of half-dose PDT. This retrospective interventional case series at a tertiary ophthalmology department looks into the 2 year outcomes of patient who had half dose photodynamic therapy (PDT), and factors that may affect outcomes post treatment.Methods Patients with chronic CSCR treated with half-dose PDT between 9/2014 and 11/2016 were followed-up for 2 years. Outcome measures included resolution of sub-retinal fluid (SRF), change in best-corrected visual acuities (BCVAs), change in central foveal thickness (CFT) and recurrence rate of CSCR at 2 years after treatment.

Diagnosis was made with spectral domain optical coherence tomography (SdOCT), fluorecein (FFA) and indocyanine green angiography (ICG) at time of treatment. All patients had BCVA in ETDRS letters. All CFT was measured with SdOCT.Results Total of 20 eyes in 16 patients were followed up for an average of 26.9 months(22-35 months). At 2 year follow-up after PDT, 13 eyes (65%) achieved complete resolution of SRF, 18 eyes (90%) had an partial improvement of central foveal thickness (CFT) on SdOCT. At 2 year follow-up after PDT, 6 eyes (30%) had improved BCVA of more than 5 ETDRS letters and 10 eyes (50%) had stable BCVA of change of 5 letters or less. 4 eyes (20%) had a reduction of BCVA of more than 5 ETDRS letters. At 2 year follow-up after PDT, 5 eyes (25%) had a recurrence of SRF. Within the 2 year follow-up period, 7 eyes (35%) developed secondary choroidal neovascularisation (CNV) which required intra-vitreal Anti-VEGF treatment. Of the patients who developed secondary CNV, 5 of 7 eyes (71.4%) had persistent SRF whereas in patient without secondary CNV, 2 of 13 eyes (15.4%) has persistent SRF (p=0.0223). However, the average gain of BCVA was similar in eye with secondary CNV (1.14 letters) and without (1.08 letters)(p=0.994).Conclusions Half-dose PDT provides long-term improvements to SRF, CFT and stable vision. A significant proportion of patients has developed secondary CNV within 2 years of treatment, and this is associated with persistent SRF, however this does not significant alter their long-term visual outcomes.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Multimodal Imaging in Best Vitelliform Macular Dystrophy

Posterboard#: A0476

Abstract Number: 5013 - A0476

AuthorBlock: Jose R. Carvalho-Jr1,2, Winston Lee2, Maarjaliis Paavo2, Karen Sophia Park2,3, Lijuan Chen2, Stephen Tsang3,2, Janet R. Sparrow2 1Hospital das Clinicas de Pernambuco-Empresa Brasileira de Servicos Hospitalares (EBSERH), Department of Ophthalmology, Federal University of Pernambuco, Recife, Pernambuco, Brazil; 2Department of Ophthalmology, Columbia University, New York, New York, United States; 3Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory, Columbia University, New York, New York, United States;

DisclosureBlock: Jose R. Carvalho-Jr, None; Winston Lee, None; Maarjaliis Paavo, None; Karen Sophia Park, None; Lijuan Chen, None; Stephen Tsang, None; Janet R. Sparrow, None;

Purpose To assess RPE integrity within and outside the vitelliform lesion by analysis of short wavelength (SW- AF) and near-infrared fundus autofluorescence (NIR-AF) quantitative analysis and spectral domain optical coherence tomography (SD-OCT).Methods Fourteen patients heterozygous for BEST1 were recruited. Each patient had a complete ophthalmologic evaluation and the images were acquired. Best Vitelliform Macular Dystrophy (BVMD) patients were staged according to OCT findings. Quantitative autofluorescence (qAF) imaging was performed using SW-AF images generated by Spectralis HRA+OCT (Heidelberg Engineering, Germany) equipped with an internal fluorescent reference. Mean gray levels were determined in eight concentric segments at an eccentricity of approximately 7 to 9° from the fovea and the outer contour of the high AF signal was used as the outer limits of the lesion. An HRA2 (Heidelberg Engineering, Germany) (787 nm; sensitivity of 96) was used to capture NIR-AF images that were saved in the non- normalized mode. ImageJ (Microsoft Java 1.1.4) was used to quantify the NIR-AF image. SD-OCT images were also acquired with the Spectralis HRA+OCT.Results We analyzed 28 eyes from 14 patients with BVMD. One patient was excluded from the quantitative analysis because lesion size and position precluded measurements outside the lesion. SW-AF images within the lesion varied with the stage of disease from normal in pre-clinical to a mottled pattern in vitelliruptive stage. NIR-AF imaging revealed an area of reduced fluorescence in pre-vitelliform stage while in vitelliruptive stage puncta of elevated signal were present. In both SW and NIR-AF images the atrophic stage had reduced signal. Importantly, non-lesion qAF was within the 95% confidence intervals for healthy eyes, independent of the stage of the disease. Similarly, the NIR-AF intensity measurements outside lesion were comparable to the control eyes. SD-OCT scans revealed a fluid- filled detachment between the interdigitation zone (IZ) and the hyperreflectivity band attributable to RPE/Bruch’s membrane.Conclusions Mutations in BEST1 are not associated with increased levels of SW-AF outside the vitelliform lesion. The high levels of SW-AF within the vitelliform lesion likely reflect the inability of RPE to phagocytose shed outer segment membrane due to the fluid-filled separation between RPE and photoreceptor cells together with progression photoreceptor cell impairment.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Analysis of ABCA4 mutation spectrum in Stargardt/ABCA4 disease

Posterboard#: A0479

Abstract Number: 5016 - A0479

AuthorBlock: Jana Zernant1, Winston Lee1, Fred Collison2, Gerald A. Fishman2, Stephen Tsang1,3, Rando Allikmets1,3 1Ophthalmology, Columbia University, New York, New York, United States; 2The Pangere Center for Hereditary Retinal Diseases, The Chicago Lighthouse, Chicago, Illinois, United States; 3Pathology and Cell Biology, Columbia University, New York, New York, United States;

DisclosureBlock: Jana Zernant, None; Winston Lee, None; Fred Collison, None; Gerald A. Fishman, None; Stephen Tsang, None; Rando Allikmets, None;

Purpose Mutations in the ABCA4 gene are the most frequent cause of inherited macular disease. To date, ~1000 disease-associated ABCA4 variants have been identified, resulting in vast genetic and phenotypic heterogeneity of ABCA4 disease. Therefore, establishing the disease prevalence and genotype/phenotype correlations is complicated. This analysis was conducted to assess the different mutation groups and the phenotypic outcome of their combinations in a large, well-characterized ABCA4 disease cohort.Methods Patients (645) and family members were recruited over 18 years. Sequencing of the ABCA4 exons and non-coding sequences was performed on the Illumina TruSeq platform. Pathogenicity of variants was assessed according to the ACMG guidelines, combining information from allele frequency data in patients vs the general population, predictive algorithms (e.g., CADD), and segregation analysis in families. The analysis included only patients with two (both) ABCA4 disease-associated alleles. Mutation severity was determined through genotype frequencies in specific disease subgroups determined by age of onset and disease progression – early-onset/severe, classical, late- onset/mild.Results 340 different ABCA4 mutations were identified in 645 Stargardt patients. 186 (54.7%) of those were missense variants, while the other 45% included 40 (11.8%) stop mutations, 38 (11.2%) small indels, 19 (5.6%) deep intronic variants, and 57 (16.7%) splice site variants. The most frequent ABCA4 disease-associated alleles in this cohort of (mostly) European origin included: p.G1961E (23% of patients, MAF=0.12), p.N1868I (11.6%, MAF=0.06), p.[L541P;A1038V] (8.8%, MAF=0.046), p.P1380L (8.4%, MAF=0.046), p.[G863A;N1868I] (7.13%, MAF=0.037), and c.5461-10T>C (7%, MAF=0.035). The most represented allele combinations were p.[G1961E];[L541P;A1038V] (2.2% of patients), p.[G1961E];[P1380L] (1.7%), and p.[L541P;A1038V];[N1868I] (1.6%). p.G1961E and p.N1868I variants identified the two hypomorphic, late-onset groups, while patients in the early onset, severe group harbored exclusively deleterious alleles. p.P1380L, p.C54Y and some other severe variants were enriched in patients of specific ethnic origin.Conclusions While ABCA4 disease is clinically and genetically very heterogeneous, (almost) definitive correlations can be made with the most frequent ABCA4 alleles and resulting phenotypes.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Stargardt disease: Rapid quantification of color vision using the Rabin Cone Contrast Test

Posterboard#: A0480

Abstract Number: 5017 - A0480

AuthorBlock: Santiago Soberón1, Vanessa Tirado1, Guillermo Salcedo- Villanueva1, Luis A. Hernández1, Elsa C. Hernández1, Jazmin Baca Moreno1, Luis Daniel Garcia Arzate1, Enrique Garcia1, Yurico Lopez1, Ana Gonzalez-H.Leon1, Ana Suárez Licona1, Roberto Gonzalez-Salinas1, Virgilio Morales- Canton1, Hugo Quiroz-Mercado1 1Asociaci�n para evitar la ceguera en M�xico (APEC), Mexico City, Mexico;

DisclosureBlock: Santiago Soberón, None; Vanessa Tirado, None; Guillermo Salcedo- Villanueva, None; Luis A. Hernández, None; Elsa C. Hernández, None; Jazmin Baca Moreno, None; Luis Daniel Garcia Arzate, None; Enrique Garcia, None; Yurico Lopez, None; Ana Gonzalez-H.Leon, None; Ana Suárez Licona, None; Roberto Gonzalez-Salinas, None; Virgilio Morales-Canton, None; Hugo Quiroz- Mercado, Allegro Ophtalmics Code C (Consultant)

Purpose We intend to quantify acquired color vision deficiency in Stargardt disease patients by using the Rabin cone contrast test (RCCT).Methods Transversal, observational and descriptive study of 6 patients with Stargardt disease (12 eyes) and 6 healthy subjects (12 eyes) were evaluated using the Rabin cone contrast test [RCCT] (Innova Systems, Burr Ridge, IL.) Diagnosis of Stargardt disease was based on ophthalmic history, complete ophthalmic evaluation and complemented with genetic testing. A minimum best corrected visual acuity of 20/300 and 20/20 respectively were part of the inclusion criteria for evaluation. We compare their acuity test and their color perseption with LogMAR values and values from L, M and S CCT results respectively. Mann-Whitney U test was used to extablished a p value to settle the significance between case and control values.Results Six patients with Stargardt disease with a mean age of 20.33±6.18 (range 12-26) years old and 6 healthy subjects with a mean age of 21.83±1.16 (range 21-24) years old were evaluated. RCCT detected that all Stargardt disease patients eyes conserved blue cones with a mean S CCTs of 62.9 (15-90), three eyes conserved green cones with a mean M CCTs of 25 (20-30) and four eyes conserved red cones with a mean L CCTs of 21.25 (10-35). The healthy subjects eyes RCCTs reported a mean S CCTs of 100, mean M CCTs of 99.5 (95-100) and a L CCTs of 95.4 (90-100). A significant p value was shown acording to Mann-Whitney test for visual acuity and RCCT results showing significant diference between both groups. Mann- Whitney test which showed visual acuity difference between cases and controls had a p value of 0.023 and RCCT difference with a p value of .0.40. Both comparisons had a significant p value.Conclusions Acquired color deficiency measured by RCCT and their visual acuity in Stargardt disease patients were significantly different from those measured on healthy subjects. The RCCT provides a rapid test (6 minutes) and findings from this study may potentially contribute to the rapid diagnosis of this disease, in comparison to other tests found in literature. Further research is needed to determine if the use of RCCT can be used as a follow up test of the progression of these patients.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Characteristics of hydroxychloroquine retinopathy in African American patients

Posterboard#: A0481

Abstract Number: 5018 - A0481

AuthorBlock: Robert Carroll1, Drew Scoles1, Brian L. VanderBeek1 1Scheie Eye Institute, University of Pennsylvania, Philadelphia, Pennsylvania, United States;

DisclosureBlock: Robert Carroll, None; Drew Scoles, None; Brian L. VanderBeek, None;

Purpose Hydroxychloroquine is an anti-malarial medication with immunosuppressive properties commonly used to treat many autoimmune diseases. An infrequent, yet serious, side effect is a toxic retinopathy leading to irreversible blindness. Classical cases demonstrate a parafoveal “bull’s-eye” pattern; however Asian patients are more likely to have a unique “pericentral” or “mixed” pattern that manifests further from the foveal center. It is less clear which pattern African Americans exhibit. We aim to determine the characteristics of hydroxychloroquine retinopathy in this population.Methods A retrospective chart review of all African American patients prescribed hydroxychloroquine and having an ophthalmology visit at an academic institution from January 2010 through November 2018 was carried out. Patients with a retinal screening exam, defined as being performed by an ophthalmologist with at least a macula spectral domain ocular coherence tomography or 10-2 automated visual field, were included. Demographic characteristics, dosage and length of hydroxychloroquine therapy, and renal function were recorded for all patients. For patients with toxicity, ophthalmic exam findings, diagnostic imaging modalities, and pattern and severity of retinopathy were also recorded. All patients with toxicity were required to have been diagnosed by a retina specialist.Results Of 436 patients reviewed, 200(45.9%) had a screening examination. 190(95%) were women and 5(2.5%, 9 eyes total) were found to have evidence of hydroxychloroquine retinopathy. Toxicity could not be determined in 1 eye of 1 patient with end stage viral . The dosage and length of therapy for 1 patient was unclear; for all others the average daily dose at diagnosis was 350 mg and the average cumulative dose was 2,273.75 gm over 17.5 years. All but 1 eye had a best-corrected visual acuity of 20/60 or better. 5 eyes(55.6%) showed evidence of pericentral or mixed retinopathy, and 7 eyes(77.8%) demonstrated moderate or severe toxicity.Conclusions Results suggest an increased prevalence of pericentral or mixed hydroxychloroquine retinopathy in African Americans. The majority with retinopathy exhibited at least a moderate level of toxicity, suggesting that current screening measures may not adequately identify all patterns of retinopathy. Future evaluation of additional African American patients with hydroxychloroquine toxicity may help to support these findings.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Low Signal Intensity Structure in The Choroid Detected in Optical Coherence Tomography Angiography

Posterboard#: A0482

Abstract Number: 5019 - A0482

AuthorBlock: keiichiro tanaka1, Tetsuju Sekiryu1 1ophthalmology, Fukushima Medical University, Fukushima-shi, Fukushima, Japan;

DisclosureBlock: keiichiro tanaka, None; Tetsuju Sekiryu, NOVARTIS PHARMA Code F (Financial Support)

Purpose We noticed low signal intensity tube-like structure (LTLS) along the choroidal vessel in both optical coherence tomography angiography (OCTA) and structural optical coherence tomography. The characteristics of LTLS was investigated in this study.Methods Retrospective observational study. 116 eyes of 77 patients (, central serous chorioretinopathy (CSC), age related macular degeneration, branch retinal vein occlusion) were examined. The images in size 12x12 mm was taken by using swept source OCT-A (PLEX™ Elite9000; Carl Zeiss Meditec). The chracteristics of LTLS in multimodal imaging, OCT-S, fundus autofluorescence (FAF), fluorescein angiography (FA) and indocyanine green angiography (IA), were analyzed.Results Fifteen (12.9%) of 116 eyes showed LTLS . There was no statistically significant difference between the eyes with and without LTLS in age, sex, logMAR, intraocular pressure, spherical equivalent and axial length. Nine (60%) of 15 eyes with CSC and Two (66%) of 3 fellow eyes with CSC showed LTLS. The incidence of LTLS in CSC was statistically significantly higher than that in the other diseases. LTLS showed iso-fluocescence to the surroundings in FA. IA showed hypofluorescence on LTLS in the early phase, and iso-fluorescence in the late phase. Intensity of FAF did not change on LTLS.Conclusions LTLS in OCTA may be inter choroidal space along the choroidal vessel isolated from the vessel because LTLS did not show remarkable changes in angiography. Since LTLS appeared frequently in CSC, LTLS might be interstitial fluid in the choroid.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Vitreopapillary adhesion in eyes treated with pneumatic vitreolysis - a morphology-based study of the vitreoretinal interface

Posterboard#: A0483

Abstract Number: 5020 - A0483

AuthorBlock: Julian Elias Klaas1, Nikolaus Feucht1, Chris Lohmann1, Mathias Maier1 1Augenklinik, Klinikum Rechts der Isar, Munich, Germany;

DisclosureBlock: Julian Elias Klaas, None; Nikolaus Feucht, None; Chris Lohmann, None; Mathias Maier, None;

Purpose The presence of vitreopapillary adhesion (VPA) in the context of anomalous posterior vitreous detachment (PVD) has been frequently described in association with the formation and possible progression of vitreomacular traction syndrome (VMTS). The purpose of this SD-OCT-based morphological study was to evaluate the vitreoretinal interface (VRI) in patients with vitreomacular traction (VMT) for the existence of VPA before and after treatment with pneumatic vitreolysis.Methods In this retrospective, observational case study we examined the morphology of the VRI in 10 eyes of 10 consecutive patients (5 male, 5 female) with focal VMT (≤1500 µm) who were treated with pneumatic vitreolysis (perfluoropropane C3F8, 0,3 ml). Patients underwent complete ophthalmic evaluation, including SD-OCT examination immediately before and at recommended follow-up visits after the procedure. We carefully reviewed the morphology of the VRI for the presence of VPA in SD- OCT-scans (30°-longitudinal, radial and volume scans) at all visits during the first 8 weeks after pneumatic vitreolysis. We further evaluated SD-OCT data for significant classification parameters, including size of VMT as well as structural changes of retinal layers.Results All eyes showed VMT with concomitant persisting VPA immediately before the procedure. With regards to post-surgical morphology of the VRI, 9 of 10 eyes (95%) showed VMT release in between the studied post-procedural interval of 8 weeks. Mean-time for VMT release was 18.57 days (SD=17,19). 3 of these eyes (33,33 %) revealed persistent VPA in the presence of VMT release (incomplete PVD). One eye showed persistent VMT with concomitant VPA in all follow-up-exams (5%). 6 of 10 patients (60%) showed both release of VMT as well as VPA release, morphologically correlating with a complete PVD in SD-OCT. In one patient a full thickness macular hole developed with consequential necessity of pars-plana-vitreoretinal surgery. We found no VPA release in presence of persisting VMT.Conclusions Pneumatic vitreolysis is considered to be a highly promising treatment option for VMTS by effectively inducing PVD in eligible cases. Our SD-OCT-based review showed comparable results of VMT release to previous studies. We found that VPA release as a result of pneumatic vitreolysis was in all cases preceded by release of VMT.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Pneumatic vitreolysis - the intravitreal injection of gas - and its capability of inducing posterior vitreous detachment will be of great relevance to patients, clinicians and socioeconomics alike. Spontaneous closure of idiopathic macular holes, a case series

Posterboard#: A0484

Abstract Number: 5021 - A0484

AuthorBlock: Michael Nee1, Delia Sang1 1VitreoRetinal Consultants, Hingham, Massachusetts, United States;

DisclosureBlock: Michael Nee, None; Delia Sang, None;

Purpose To report the incidence of spontaneous macular hole closure in a retrospective series of idiopathic full thickness macular holes, and to evaluate the mechanism of closure and association of size of macular hole and use of topical cortisone/NSAIDS.Methods Charts of sequential patients diagnosed with macular hole were reviewed The diagnosis of full thickness spontaneous macular hole was confirmed by chart review and OCT B. Patients were excluded if they were followed for less than six months, or for history of concurrent retinal disease.Results A total of 101 idiopathic macular holes were identified in 91 patients from 11/15/15--11/14/18. Thirteen macular holes were excluded with branch or central retinal vein occlusion, diabetic retinopathy, uveitis, or wet ARMD. Six patients were not surgical candidates or refused. Vitrectomy was performed on 76 eyes. Of the remaining 6 patients, the macular holes all closed without surgery; the width of the macular hole ranged 60-273 microns. Of the 6 macular holes, 2 closed spontaneously without topical medications, and 4 closed with cortisone (prednisolone acetate or difluprednate) and NSAIDs (bromfenac, ketorolac or nepafenac). A 'bridging' closure was noted on OCT B in 5/ 6 patients, with release of vitreomacular traction in one eye.

Of the 4 holes treated with cortisone / NSAIDSs (avge base diameter 196um), closure occurred at 13 to 84 days, with average duration of 50 days. Three out of four closed with 'bridging.' The outer retinal cyst resolved by 72 days (55-72 days). Of these patients, 2 closed despite epiretinal membrane, and 1 with cystoid macular edema. Of the 2 that closed spontaneously without topical medications (diameter 107um), one showed bridging at 21 days.

Visual outcome in non-surgical eyes was excellent, ranging from 20/20 to 20/60. The initial vision at time of diagnosis ranged from 20/20-2 to 20/60-2.Conclusions Less than 6% of macular holes closed without surgery. Of note, ERM and CME did not prevent closure. Eyes treated with cortisone / NSAIDS did not close more rapidly than those closed without topical medications. Width of macular hole did not appear related to the likelihood of non-surgical macular hole closure.

Larger studies are needed to further investigate treatments, mechanisms and biomarkers in in non- surgical closure of idiopathic macular holes.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Eplerenone for the treatment of central serous chorioretinopathy: 3 year clinical experience

Posterboard#: A0473

Abstract Number: 5010 - A0473

AuthorBlock: Daniel Petkovsek1, Felipe Conti1, Daniel G. Cherfan1, Justis P. Ehlers1, Peter K. Kaiser1, Andrew Schachat1, Sumit Sharma1, Sunil K. Srivastava1, Aleksandra Rachitskaya1, Amy Babiuch1, Rishi P. Singh1 1Cole Eye Institute, Cleveland Clinic Foundation, Cleveland Heights, Ohio, United States;

DisclosureBlock: Daniel Petkovsek, None; Felipe Conti, None; Daniel G. Cherfan, None; Justis P. Ehlers, Bioptigen Code P (Patent), Bioptigen Code C (Consultant), Thrombogenics Code C (Consultant), Genentech Code C (Consultant), Leica Code C (Consultant), Zeiss Code C (Consultant), Alcon Code C (Consultant), Thrombogenics Code F (Financial Support), Genentech Code F (Financial Support), Alcon Code F (Financial Support), Regeneron Code F (Financial Support), Aerpio Code F (Financial Support), Boerhinger-Ingelheim Code F (Financial Support), Leica Code P (Patent), Peter K. Kaiser, Zeiss Code C (Consultant), Topcon Code C (Consultant), Alcon Code C (Consultant), Novartis Code C (Consultant), Bausch & Lomb Code C (Consultant), Andrew Schachat, Elsevier Code R (Recipient), American Academy of Ophthalmology Code R (Recipient), Easton Capital Code R (Recipient), Sumit Sharma, Allergan Code C (Consultant), Sunil K. Srivastava, Bausch & Lomb Code C (Consultant), Leica Code C (Consultant), Zeiss Code C (Consultant), Bausch & Lomb Code F (Financial Support), Allergan Code F (Financial Support), Bioptigen Code P (Patent), Aleksandra Rachitskaya, Allergan Code C (Consultant), Amy Babiuch, None; Rishi P. Singh, Novartis/Alcon Code C (Consultant), Regeneron Code C (Consultant), Genentech/Roche Code C (Consultant), Zeiss Code C (Consultant), Regeneron Code F (Financial Support), Genentech Code F (Financial Support), Apellis Code F (Financial Support)

Purpose Central serous chorioretinopathy (CSCR) is a condition that presents with sub-retinal fluid and can be sight-threatening in its chronic form. As understanding of the pathophysiology behind CSCR continues to evolve, the use of oral therapies, including the anti-mineralocorticoid oral agent eplerenone, has been increasingly reported. Though the efficacy of this agent has been established, prior studies have been limited by small cohort size and short follow up duration. The aim of this report is to present data on the anatomical and clinical response to oral eplerenone over a three year period in eyes of patients with CSCR.Methods An IRB approved retrospective chart analysis from 2012-2018 was performed. Eyes with a diagnosis of chronic CSCR, treated with 25 or 50mg/day oral eplerenone were included. Baseline best-corrected visual acuity and anatomical measurements measured by spectral domain optical coherence tomography (SD-OCT) were collected at eplerenone initiation. Follow up data was collected at the closest date to 12, 24, and 36 months follow up, with minimum follow up of 6 months. Eyes with a pre-existing retinal disorder that was likely to limit visual acuity or confound OCT measurements were excluded. The primary outcome was reduction in central subfield thickness at the final follow up visit. Secondary outcomes included mean change in visual acuity (logMAR equivalent of Snellen best corrected visual acuity), macular cube volume, macular cube average thickness, and maximum sub- retinal fluid diameter and height.Results Follow up data was obtained for 100 eyes of 83 patients at 1 year (mean 11.18 ± 4.00 months), 49 eyes at 2 year (24.01 ± 3.33 months), and 33 eyes at three year (mean 35.5 ± 7.89 months) follow up visits. For these samples, the rate of complete sub-retinal fluid resolution was 31%, 28%, and 33%, respectively. At final follow up, logMAR visual acuity change from baseline was +0.10 ± 0.24 (p=0.130). Average change from baseline at final follow up for CST was -97 ± 140.6 µm (p < 0.001), cube volume was – 1.07 ± 1.71 mm3 (p < 0.001), macular thickness – 28. 5 ± 47.5 µm (p < 0.001), maximum sub-retinal fluid height was -95.6 ± 160.5 µm (p < 0.001), maximum sub retinal fluid diameter was -1169.0 ± 1638.7 µm (p = 0.008).Conclusions Anatomic improvement occurs primarily within the first year of eplerenone treatment for chronic central serous chorioretinopathy.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Fibulin-3 mutation in mice dysregulates RPE cells and causes exosome markers to accumulation in drusen

Posterboard#: A0485

Abstract Number: 5022 - A0485

AuthorBlock: Jasmine Geathers1, Weiwei Wang1, Alistair Barber1, Jeffrey M. Sundstrom1 1Pennsylvania State College of Medicine, Hershey, Pennsylvania, United States;

DisclosureBlock: Jasmine Geathers, None; Weiwei Wang, None; Alistair Barber, None; Jeffrey M. Sundstrom, None;

Purpose Malattia Leventinese (ML), an autosomal dominant inherited degenerative eye disease, is caused by a point mutation (R345W) in the fibulin-3 (Fib3) gene (efemp1) that results in deposition of sub-retinal pigment epithelium (RPE) drusen. Proteomic studies have revealed that these deposits contain extracellular matrix proteins, including Fib3, and exosome markers such as CD63. Recent studies in our lab have shown that Fib3 is bound to the surface of exosomes, suggesting that it may mediate exosome function. The purpose of the present study was to characterize changes in RPE morphology and drusen accumulation in mice carrying the Fib3-R345W mutation.Methods Frozen radial cross-sections (10 µm thick) of eyes from wild-type (Fib3+/+), heterozygous (Fib3+/ki) and homozygous (Fib3ki/ki) mice were labelled for Fib3, ApoE, ALIX, and CD63 using immunofluorescence and imaged by confocal microscopy (Leica SP8). Adjacent sections were stained with Periodic Acid Schiff (PAS) and Hematoxylin and Eosin (H&E) and imaged using bright-field microscopy. Electron microscopy was also used to detect subcellular changes in the RPE layer.Results The RPE of Fib3+/+ mice in PAS and H&E sections were arranged in a uniform monolayer. In contrast, the RPE of Fib3+/ki and Fib3ki/ki mice were pleomorphic and arranged in multiple layers. The RPE layer of Fib3+/ki and Fib3ki/ki was thicker with more abundant nuclei, suggesting cell proliferation. Discrete ApoE and Fib3 immunofluorescence was identified in sub-RPE patches in all three groups of mice. These lesions appeared to be more abundant in the Fib3+/ki and Fib3ki/ki mice after 6 months of age compared to the Fib3+/+ mice. CD63 immunofluorescence also localized to similar sub-RPE patches in the Fib3+/ki and Fib3ki/ki mice, but not the Fib3+/+ mice, after 18 months of age. ALIX immunofluorescence localized to similar sub-RPE patches in the Fib3ki/ki only.Conclusions The results suggest that the Fib3-R345W mutation induces RPE hyperplasia prior to the development of overt sub-RPE drusen formation, and that Fib3-positive aggregates begin to appear after 6 months of age, followed by a similar pattern of CD63 immunoreactivity after 18 months. ApoE and ALIX immunoreactivity also appear after 18 months, suggesting a relationship between exosome marker proteins and Fib3.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Internal Limiting Membrane Peeling with Air Tamponade for Idiopathic Full Thickness Macular Hole : A Case-matched Study

Posterboard#: A0486

Abstract Number: 5023 - A0486

AuthorBlock: Feng Gu1, PENG SUN1, Han Zhang1, Jun Li1, Yi Zhang1, Zheli Liu1 1Ophthalmology, First Hospitol of China Medial University., Shenyang, China;

DisclosureBlock: Feng Gu, None; PENG SUN, None; Han Zhang, None; Jun Li, None; Yi Zhang, None; Zheli Liu, None;

Purpose Idiopathic full-thickness macular hole (FTMH) is treated by vitrectomy with internal limitingmembrane (ILM) peeling with long-lasting inert gas tamponade routinely. We proposed this retrospective, case- matched study to investigate whether air tamponade is equivalent to gas tamponade.Methods

This study included 82 patients with idiopathic FTMH experienced vitrecto my/ILM peeling and air tamponade. We matched another 82 patients who were treated with vitrectomy/ILM peeling and C3F8 tamponade by age, gender, BCVA, size of macular hole, and range of ILM peeling. Preoperative and postoperative best corrected visual acuity(BCVA), intraocular pressure, spectral-domain optical coherence tomography (SD-OCT) were recorded for statistical analysis. Results

All patients were followed up 10.4 months(6-12 months). For air tamponade group, the postoperative BCVA at1 week, 1, 3, 6 months was 0.8±0.12，0.53±0.11, 0.32±0.02, 0.30±0.08 in LogMAR, 71 patients achieved macular hole closure (86.69%)by one operation, and additional 4 patients had successful macular hole closure by 1.6(1-3) times air-fluid exchange in office. In gas tamponade group, the postoperative BCVA at 1week, 1, 3, 6 months was 2.0±0.19，0.51±0.17, 0.33±0.16, 0.30±0.17 , 75 patients achievedmacular hole closure (91.46%) by one operation. There was no significant difference between the two groups either on postoperative BCVA after 1 month(p=0.453) or macular closure rate(p=0.629). The postoperative BCVA of air group was better than gas group at 1 week follow-up.Conclusions

Vitrectomy with ILM peeling is an effective treatment for idiopathic FTMH. Air tamponade can achieve faster vision recovery and similar final outcome both on visual acuity and macular hole closure rate compared with gas tamponade.

Layman Abstract (optional): Provide a 50-200 word description of your work that non- scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details. Prevalence and characteristics of hyporeflective intraretinal spaces secondary to idiopathic epiretinal membranes using spectral domain optical coherence tomography

Posterboard#: A0487

Abstract Number: 5024 - A0487

AuthorBlock: Christelle GRONDIN1, Aude COUTURIER1, Jennifer Marie Louise1, Valérie Mané1, Alain Gaudric1, Ramin Tadayoni1 1Ophthalmology, Lariboisière Hospital, PARIS, France;

DisclosureBlock: Christelle GRONDIN, None; Aude COUTURIER, None; Jennifer Marie Louise, None; Valérie Mané, None; Alain Gaudric, None; Ramin Tadayoni, None;

Purpose To determine the prevalence, characteristics of hyporeflective intraretinal spaces (HIRS) secondary to idiopathic epiretinal membranes (ERMs) using spectral domain optical coherence tomography (SD OCT)Methods Design: Retrospective, observational case series. Materials : The files of 257 consecutive eyes operated with the diagnosis of ERMs between January 2017 and December 2017 were reviewed . Secondary ERMs (n =101) and other retinal disorders, i.e. diabetic retinopathy, vein occlusion, glaucoma, ( n= 43) were excluded. One hundred and thirty three eyes with idiopathic ERMs were included and divided in two groups: HIRS + group and HIRS - group (eyes without preoperative HIRS). Main Outcome Measures: The preoperative prevalence of HIRS in patients with idiopathic ERMs. The SD OCT features included central foveal thickness (CFT), the defect of the cone outer segment tips (COST) and ellipsoid lines, the presence of subfoveal deposit.Results The prevalence of HIRS in idiopathic ERMs was of 8.91% (14/157 eyes) . The HIRS appeared as homogeneous clusters of the internal nuclear layer (7 eyes), heterogeneous clusters (5 eyes) and retinal splitting spaces (2 eyes). The comparison of HIRS+ group with HIRS-group showed no significant difference in the proportion of pseudophakic eyes (28.6% vs. 28.3%; p>0.05), in the preoperative CFT (485.00 ± 60.59 µm vs 447.20 ± 76.56 µm, p= 0.04338), in the disruption of the ellipsoid layer (35.7% vs 38.4%, p> 0.05) and the COST line (35.7% vs 59.6%, p> 0.05), in the subfoveal deposits (42.9% versus 41.4%, p > 0.05), and in the preoperative BCVA (0.62 ± 0,31 logMAR vs 0.41 ± 0.19 logMAR, p= 0.62414). The number of eyes with a partial posterior vitreous detachment (PVD) was significantly higher in the HIRS + group (57.2 % versus 9.1% p= 0.0001) ; consequently the presence of complete PVD was higher in the HIRS – Group (83.8 % versus 35.7%, p= 0.0003).No difference about the absence of PVD was found between the both groups ( 7.1%, p> 0.05).Conclusions The prevalence of HIRS in idiopathic ERMs was 8.91% prior to surgery. The HIRS presented a higher rate of partial PVD which suggests their origin may be tractional. The predilection for the internal nuclear layer (INL) and the preserved outer retinal layers may suggest also a role of Muller cells dysfunction in HIRS occurrence.Layman Abstract (optional): Provide a 50-200 word description of your work that non-scientists can understand. Describe the big picture and the implications of your findings, not the study itself and the associated details.