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Purpose: To determine whether atrophy of the retinal pigment epithelium (RPE) in eyes with neovascular age-related macular degeneration (nAMD), which meets the criteria for the suspension of anti-vascular endothelial growth factor (anti-VEGF) treatment, is associated with anti-VEGF treatments. Methods: Twelve eyes of 12 patients with nAMD who began anti-VEGF treatment and were followed for 1 year after meeting the criteria for the suspension of anti-VEGF were studied. Six eyes of six patients were placed in the continuation group, and six eyes of six patients were placed in the suspension group. The RPE atrophic area at the time of the last anti-VEGF treatment was set as the baseline size and that at 12 months after the baseline (Month 12) was taken as the final size. A comparison of the expansion rate of RPE atrophy between the two groups was made by the square-root transformed differences. Results: The expansion rate of atrophy was 0.55 (0.43, 0.72) mm/year in the continuation group and 0.33 (0.15, 0.41) mm/year in the suspension group. This difference was not significant. (p = 0.29). Conclusions: Suspension of anti-VEGF treatments in eyes with nAMD does not alter the expansion rate of RPE atrophy.

PURPOSE: To use empirical data to develop a model of cell loss in choroideremia that predicts the known exponential rate of RPE loss and central, scalloped preservation pattern seen in this disease. METHODS: A computational model of RPE loss was created in Python 3.7, which constructed an array of RPE cells clusters, binarized as either live or atrophic. Two rules were applied to this model: the background effect gave each cell a chance of dying defined by a background function, and the neighbor effect increased the chance of RPE cell death if a neighbor were dead. The known anatomic distribution of rods, RPE, choriocapillaris density, amacrine, ganglion, and cone cells were derived from the literature and applied to this model. Atrophy growth rates were measured over arbitrary time units and fit to the known exponential decay model. The main outcome measures: included topography of atrophy over time and fit of simulated residual RPE area to exponential decay. RESULTS: A background effect alone can simulate exponential decay, but does not simulate the central island preservation seen in choroideremia. An additive neighbor effect alone does not simulate exponential decay. When the neighbor effect multiplies the background effect using the rod density function, our model follows an exponential decay, similar to previous observations. Also, our model predicts a residual island of RPE that resembles the topographic distribution of residual RPE seen in choroideremia. CONCLUSIONS: The pattern of RPE loss in choroideremia can be predicted by applying simple rules. The RPE preservation pattern typically seen in choroideremia may be related to the underlying pattern of rod density. Further studies are needed to validate these findings.

Purpose This study measured and correlated degeneration of the junction between the inner and outer segments (IS/OS), the retinal pigment epithelium (RPE), and the choriocapillaris (CC) in Stargardt disease (STGD). Design Prospective cross-sectional study. Methods This study was conducted at the Casey Eye Institute. A total of 23 patients with STGD were enrolled and underwent optical coherence tomography angiography (OCTA). Scans were centered on the fovea. OCT slab projections and en face boundary maps were used to create masks to measure total IS/OS loss or RPE atrophy as well as regions of isolated IS/OS loss, isolated RPE atrophy, and matched IS/OS and RPE degeneration or intact IS/OS junction and RPE. CC vascular density (CCVD) was quantified from the CC angiogram. Outcomes included the area of loss, and the CCVD of degeneration in different areas was quantified and correlated. Results The total area of IS/OS loss was strongly correlated with the total area of RPE atrophy (r = 0.96; P Conclusions Photoreceptor and RPE degeneration exhibited a strong relationship wherein the IS/OS loss was 1.6-fold greater than that of RPE atrophy, supporting the theory that photoreceptor degeneration precedes RPE in STGD. Both the photoreceptors and the RPE degeneration contributed synergistically to CCVD attenuation, but extralesional CCVD also tended to be abnormal. The findings and techniques in this study may be of utility in developing endpoints for clinical trials.

To investigate retinal sensitivity in eyes with angioid streaks (AS). This study was a retrospective observational case series. A total of 13 eyes from 9 patients with AS underwent ophthalmologic examination, including fundus autofluorescence (FAF), spectral domain optical tomography (SD-OCT), and microperimetry. Retinal pigment epithelium (RPE) atrophy was assessed using FAF. Outer retinal atrophy, more specifically, disruption of the ellipsoid zone, was evaluated using SD-OCT images. The association between retinal sensitivity, RPE atrophy or outer retinal atrophy, and patient demographic characteristics or ophthalmologic findings were investigated. The mean area of outer retinal atrophy was 14.5 ± 12.9 mm2, significantly larger than the mean area of RPE atrophy (9.0 ± 9.0 mm2; P = 0.0028). The average retinal sensitivity in the area of RPE atrophy was 2.4 ± 5.6 dB, that in the area of outer retinal atrophy outside RPE atrophy was 14.6 ± 7.5 dB, and that in the area without any atrophy was 25.6 ± 5.1 dB. Reticular pseudodrusen (RPD) was observed in 5 eyes. Eyes with RPD showed a greater extent of both outer retinal atrophy and RPE atrophy than those without RPD and a greater decrease in mean retinal sensitivity. In eyes with AS, especially in those with RPD, large areas of outer retinal atrophy and RPE atrophy were observed, and the retinal sensitivity in the areas was correspondingly decreased.

To investigate factors contributing to the visual prognosis of choroidal neovascularization (CNV) secondary to angioid streaks (AS) in a long-term follow-up (> 5 years) study. Twenty-one patients (32 eyes) affected by CNV secondary to AS were enrolled retrospectively and divided into three groups according to the period of CNV recurrence from the final treatment: group A, no recurrence for more than 12 months; group B, no recurrence for 6–12 months; and group C, no recurrence for

Purpose To compare morphologic changes on en face images derived from wide-field swept-source optical coherence tomography (ssOCT) to hypo- and hyperautofluorescent (hypoAF, hyperAF) areas on short-wavelength autofluorescence (SW-AF), and near-infrared (NIR)-AF in recessive Stargardt disease (STGD1). Methods Wide-field ssOCT cube scans were obtained from 16 patients (16 eyes). Averaged B-scans and SW-AF images were obtained using Spectralis HRA+OCT. NIR-AF images were obtained from 6 eyes. The inner/outer segment (IS/OS), OS/RPE, and RPE/Bruch's membrane boundaries were segmented, and en face slab images generated. A subRPE slab image was used to measure the abnormal RPE area, and an IS/OS slab image, the IS/OS junction loss area. These were compared to hypo- and abnormal SW-AF areas, and hypoNIR-AF areas. A preRPE(OS) slab image was used to evaluate the spatial and intraretinal locations of flecks. Results For all eyes, RPE atrophy was visualized as a central hyperreflective area on the subRPE slab, and IS/OS junction loss as an abnormal reflective area on the IS/OS slab; the latter was significantly larger (P = 0.04). There was good agreement between the hyperreflective area on the subRPE slab image and hypoSW-AF area, and between the abnormal reflective area on the IS/OS slab and hypo-hyperSW-AF area; the hypoNIR-AF area indicated that the hyperreflective area on the subRPE slab underestimated RPE atrophy. The spatial locations of hyperreflective flecks on the en face preRPE(OS) slab image corresponded to those on the SW-AF images. Conclusions Wide-field en face OCT imaging has the potential to be a clinically useful tool for the management of STGD1.

Central serous chorioretinopathy (CSCR) is characterised by choroidal hyperpermeability which results in neurosensory detachments (NSD) along with numerous retinal pigment epithelium (RPE) alterations such as RPE atrophy. Fundus autofluorescence (FAF) demonstrates the functionality of the RPE while multicolor imaging(MCI), by means of its three incident wavelengths, provides insight into clinical changes at various levels of the retina and choroid in CSCR. This study compares various clinical findings in CSCR (NSD, subretinal deposits, RPE atrophy, pigment epithelial detachments (PED) and pachyvessels) on the above mentioned imaging modalities both qualitatively and quantitatively. MCI showed higher mean cumulative area of RPE atrophic patches (6.3 ± 6.02 vs 5.7 ± 5.7 mm2, p = 0.046), PED (1.3 ± 1.4 vs 1.1 ± 1.2 mm2, p = 0.068) and NSD (17.2 ± 11.4 vs 15.7 ± 10.7 mm2, p = 0.033). MCI demonstrated better defined lesions (NSD, PED, RPE atrophy) and more number of eyes with PED and pachyvessels in comparison to FAF.Both investigations had a 100% sensitivity in detecting NSD and 100% specificity for sub retinal deposits. This study demonstrates the ability of MCI to quantitatively and qualitatively define various clinical features in CSCR and the advantages it holds over FAF. MCI can hence be considered as a useful imaging modality in documenting and monitoring various structural changes in eyes with CSCR.

Background: Early confirmation of the effect of brachytherapy for choroidal melanoma showing that tumour coverage is valuable. The irradiated retinal pigment epithelium (RPE) commonly develops atrophy. This study compares the fundus autofluorescence (AF) changes to the development of RPE atrophy following brachytherapy. Methods: Retrospective study of 19 patients treated with 106Ru and 2 with 125I plaques with either a 3- or 6-month follow-up period. Ultra-widefield (UW) composite colour and AF images were obtained with Optomap 200Tx and interpreted as complete, partial, or no RPE changes and complete or partial hyperautofluorescence, hypoautofluorescence, or isoautofluorescence. Results: At the 3-month follow-up, 9 of 13 patients (69%) (95% confidence interval [CI], 0.389–0.896) treated with 106Ru plaques developed complete homogenous hyperautofluorescence surrounding the tumour, but only 1 of 13 (8%) (95% CI, 0.004–0.379) developed complete RPE atrophy at the same time point. Six patients in the 106Ru plaque group had their first follow-up with UW imaging at 6 months. Four of them developed homogenous hyperautofluorescence and none developed complete RPE atrophy around the tumour. The 2 patients treated with 125I did not demonstrate any clear RPE or AF changes. Conclusion: The effect of 106Ru plaque treatment on fundus UW imaging is detected as homogenous and well-demarcated hyperautofluorescence before visible RPE atrophy.

Purpose The purpose of this study was to characterize the microstructure of the nonjuxtapapillary microvasculature dropout (MvD) in healthy myopic eyes. Methods This cross-sectional study included 50 eyes (25 eyes with a nonjuxtapapillary MvD and 25 age-matched eyes without any MvD) from a cohort of 126 nonglaucomatous healthy myopic eyes having parapapillary atrophy (PPA) γ-zone. The parapapillary deep-layer microvasculature was evaluated in en-face images obtained using swept-source optical coherence tomography (OCT) angiography (OCTA). A nonjuxtapapillary MvD was defined as an area with focal absence of vascular signals in the distal portion of PPA confined to the nonjuxtapapillary area. Enhanced depth-imaging OCT scanning was performed to assess the parapapillary microstructure. Results Nonjuxtapapillary MvD was found in 25 eyes (19.8%). The parapapillary microstructure at the nonjuxtapapillary MvD in 18 eyes was characterized by the misalignment of Bruch's membrane (BM)–retinal pigment epithelium (RPE) complex, which was identified by the absence of BM-RPE complex and the presence of the inner retina and sclera. In seven eyes with a nonjuxtapapillary MvD but without such misaligned BM-RPE complex, RPE atrophy was observed at the location of the nonjuxtapapillary MvD. Eyes with a nonjuxtapapillary MvD had a longer axial length (AXL; P = 0.013) and a wider γ-zone (P < 0.001) than age-matched control eyes without any MvD. Conclusions The microstructure at the nonjuxtapapillary MvD in healthy myopic eyes was characterized in approximately 70% of eyes by temporally misaligned BM-RPE complex. Although the clinical importance of the nonjuxtapapillary MvD remains to be determined, it should be differentiated from the parapapillary choroidal MvD observed in glaucoma.

Purpose To investigate if the contiguity between native and transplanted retinal pigment epithelium (RPE) represents a protective factor against the progression of atrophy after autologous choroidal graft. In addition, the changes in fundus autofluorescence (FAF) in the contiguous and noncontiguous RPE areas were explored. Methods The first postoperative reliable FAF image was selected and divided into sectors based on the characteristics of the RPE at the edge of the graft. The sectors were categorized into three groups: contiguous RPE, noncontiguous RPE, and not classifiable. The area of RPE atrophy, inside and outside the graft, was measured for each sector at baseline, one, three, and five years of follow-up. The FAF pattern outside the graft was evaluated for every sector at baseline and during the follow-up. Results Nineteen patients met the inclusion criteria and were included in this study. Trend analysis showed that the atrophy progression outside the graft was statistically significant in areas where native and transplanted RPE were noncontiguous (P < 0.0001) yet not so in contiguous areas (P < 0.058). Inside the graft, both groups showed an increase in atrophy over time. In addition, the noncontiguous group developed more severe increased FAF patterns compared with the contiguous group. Conclusions RPE contiguity after autologous choroidal graft seems to be a protective factor against atrophy progression, whereas any area of damaged or absent RPE tends to enlarge over time. This may suggest that the transplantation of an RPE sheet is more likely to be effective than an RPE cell suspension.

Purpose: To investigate the association of optical coherence tomography (OCT)-derived drusen measures in Amish age-related macular degeneration (AMD) patients with known loci for macular degeneration. Methods: Members of the Old Order Amish community in Pennsylvania ages 50 and older were assessed for drusen area, volume and regions of retinal pigment epithelium (RPE) atrophy using a Cirrus High-Definition OCT. Measurements were obtained in the macula region within a central circle (CC) of 3 mm in diameter and a surrounding perifoveal ring (PR) of 3 to 5 mm in diameter using the Cirrus OCT RPE analysis software. Other demographic information, including age, gender and smoking status, were collected. Study subjects were further genotyped to determine their risk for the AMD-associated SNPs in the SYN3, LIPC, ARMS2, C3, CFB, CETP, CFI and CFH genes using TaqMan genotyping assays. The association of genotypes with OCT measures were assessed using linear trend p-values calculated from univariate and multivariate generalized linear models. Results: 432 eyes were included in the analysis. Multivariate analysis (adjusted by age, gender and smoking status) confirmed the known significant association between AMD and macular drusen with the number of CFH risk alleles for the drusen area (the area increased 0.12 mm2 for a risk allele increase, p &lt, 0.01), drusen volume (the volume increased 0.01 mm3 for a risk allele increase, p ≤ 0.05) and the area of RPE atrophy (the area increased 0.43 mm2 for a risk allele increase, p = 0.003). SYN3 risk allele G is significantly associated with larger area PR (the area increased 0.09 mm2 for a risk allele increase, p = 0.03) and larger drusen volume in the central circle (the volume increased 0.01 mm3 for a risk allele increase, p = 0.04). Conclusion: Among the genotyped SNPs tested, the CFH risk genotype appears to play a major role in determining the drusen phenotype in the Amish AMD population.

Purpose: To report a case of the expansion of submacular retinal pigment epithelium (RPE) atrophy after using the inverted internal limiting membrane (ILM) flap technique for a persisting, large, stage IV macular hole (MH). Case Report: A 79-year-old woman presented with a chronic large MH that remained open despite pars plana vitrectomy (PPV). The surgery was performed twice for the MH closure 14 years earlier. ILM peeling was not performed during the previous surgeries. The best-corrected visual acuity (BCVA) with the Landolt ring chart was 0.08 at her visit. The minimum MH diameter was 1,240 μm. Inverted ILM flap technique with 20% SF6 gas tamponade was performed for the MH closure. For the inverted ILM flap technique, 25-gauge PPV and ILM staining with indocyanine green were used. The ILM was peeled off for 2 disc diameters around the MH, but the ILM was not removed completely. The ILM was then inverted and covered the MH. Results: One month after surgery, the MH was closed, accompanied by glial cell proliferation spreading from the inverted ILM flap (as reported before). On the other hand, the area of the submacular RPE atrophy, which was already observed 1 week after surgery, gradually increased in size. BCVA improved to 0.3 six months after the surgery. Conclusions: The inverted ILM flap technique may be promising even for persisting large MH which were not closed in previous surgeries, but long-term observation is needed because the detailed behavior of the inverted ILM and the Müller cells after surgery is not yet known.

Central serous chorioretinopathy (CSCR) belongs to pachychoroid spectrum of diseases, and the pathogenesis is still not fully understood. It is associated with multiple systemic factors. It is characterized by the collection of fluid between the retinal pigment epithelium (RPE) and the neurosensory retina. It mostly affects young men under perceived stress. The involvement of corticosteroids is undisputed although their exact role has not been clarified; other parts of the underlying mechanism of CSCR have been mainly elucidated by imaging techniques, such as fluorescein and indocyanine green angiography. The course is usually self-limiting and in most cases resolves spontaneously within a 3–6-month period, with visual acuity usually recovering to 20/30 or better. However, chronic CSC may develop as a consequence of recurrences or persistent neurosensory detachment and can result in progressive RPE atrophy and permanent visual loss. We used search terms such as “central serous retinopathy,” “central serous chorioretinopathy,” “CSCR,” “management,” and “treatment” and also the keywords of each type of treatment on PubMed and Medline to retrieve articles for this systematic review.

Purpose of review Fundus autofluorescence is a noninvasive technique for evaluation of intrinsic autofluorescence of the tissues within the eye. In recent years, autofluorescence has become an important diagnostic tool for the assessment of various ocular diseases such as age-related macular degeneration and retinal dystrophies. In this report, we review the recent literature on autofluorescence of intraocular tumours. Recent findings The autofluorescence features of intraocular tumours range from bright hyperautofluorescence to dark hypoautofluorescence. The fundus autofluorescence generally represents the status of the overlying retinal pigment epithelium (RPE). Choroidal nevi typically have overlying hypoautofluorescence from chronic RPE atrophy as opposed to choroidal melanoma that exhibits hyperautofluorescence from overlying lipofuscin within RPE (orange pigment) and free fluorophores within fresh subretinal fluid. Choroidal metastases demonstrate overlying hyperautofluorescence that correlates to focal RPE accumulation of lipofuscin as well as subretinal fluid, particularly on the fresh advancing tumour margin. Choroidal haemangioma displays overlying hyperautofluorescence from lipofuscin within RPE and fresh subretinal fluid, but when choroidal haemangioma is chronic with resolved fluid, there is often overlying hypoautofluorescence from RPE atrophy. Congenital hypertrophy of the RPE is characterized by marked hypoautofluorescence of the RPE lesion and trace hyperautofluorescence within the lacunae. Summary Autofluorescence is a noninvasive, valuable diagnostic tool for assessment of intraocular tumours, based primarily on the effects on the overlying RPE. Some findings are strongly characteristic of certain tumours, particularly the bright hyperautofluorescence overlying small choroidal melanoma and the dark hypoautofluorescence of congenital hypertrophy of the RPE.

Mitochondrial A3243G point mutations cause variable pathologic changes in different organs. Funduscopy revealed sharply demarcated central areas of atrophy of the retinal pigment epithelium (RPE) which corresponded to the visual field defects. Fundus autofluorescence was reduced in the areas of RPE atrophy but showed granular hyperfluorescence of the adjacent RPE. Heteroplasmic mitochondrial mutations may cause variable changes in different organ systems. However, the ocular phenotype in the described pair of twins was almost identical. Fundus autofluorescence showed little progression of the RPE atrophy.

Purpose To evaluate the efficacy of subthreshold micropulse yellow laser photocoagulation(SMYLP) for subfoveal serous pigment epithelial detachment(SPED). Methods We evaluated retrospectively 6 eyes of 5 patients with symptomatic subfoveal SPED treated with SMYLP. SMYLP was performed on the entire area of SPED with Supra 577Y Laser System(Quantel Medical). The SMYLP settings were 100 μm spot diameter, 20 ms duration, 15% duty cycle, 3 × 3 pattern. If SPED was not definitely restored, we applied laser again with more increased power at 1 or 2 months interval. Results All patients were received 3 or 5 times bevacizumab or ranibizumab injections before SMYLP. The total follow-up period was 26.5 (18–36) months, mean follow-up period after laser application was 19.8 (15–32) months. Mean height of PED before treatment was 349.2 ± 53.22 μm. About 3 (2–4) sessions SMYLPs were performed. All eyes showed nearly complete resolutions of the PED for 5.6 (5–7) months, which were sustained during the follow-up. Mean BCVA before treatment was 0.11 (0–0.22) logMAR and mean BCVA at final follow-up was 0.07 (0–0.22) logMAR. After PED resolution, all patients had increased vision and metamorphopsia improvement. After PED resolution, usually there were seen in mild RPE atrophies in fundus and OCT image, and these atrophies were not aggravated. Conclusions SMYLP for subfoveal SPED induced the resolution of SPED and symptom improvement. But after SPED resolution, mild RPE atrophy was detected. We think this finding was related to subsequent epithelial atrophy after resolution of SPED or laser damage. Prospectively, the study about long-term efficacy and safety of this treatment will be needed.

BACKGROUND Subretinal transplantation of pigment epithelium may be a therapeutic option in the treatment of age-related macular degeneration. Suspensions of pigment epithelial cells as well as confluent cell layers are being considered. METHODS We developed a surgical device which permits to load living tissue, without exerting mechanical stress to the cells themselves, and to deliver it precisely onto the subretinal target site. RESULTS After having been grown to confluency on extracellular matrix, the tissue can be fixed on a cannulated spoon by creating a vacuum on its undersurface. The spoon is connected to a silicone tube attached to a syringe. The syringe is used to produce a vacuum which is delivered to the tissue through the perforated surface of the spoon. After directing the spoon into the subretinal space, the tissue can be discharged by releasing the vacuum. CONCLUSIONS In previous studies large cannulas have been used for injecting coherent cell layers underneath the retina. This technique frequently resulted in major distortion of the tissue. Furthermore, correct apico-basal orientation of the tissue often could not be achieved. We present a novel concept of a device which exerts vacuum to the entire underside of the graft allowing to hold it in position without distorting it. By releasing the vacuum, the graft can be positioned in the site of RPE atrophy. (Patent Reg. No. 29819018.4, Munchen, Germany, 1998).

Central Areolar Choroidal Dystrophy (CACD) is a rare, bilateral disorder often challenging to diagnose because of the many chorioretinal conditions with similar funduscopic findings. It is characterized by a circumscribed area of atrophy in the macular region. Fluorescein angiography reveals normal choroidal filling with obvious hyperfluorescence in the areas of RPE atrophy. Visual field defects are evidenced by a well-demarcated area of central loss. Autosomal dominant, autosomal recessive and sporadic cases have been reported. An 81-year-old white female presented to our clinic with a chief complaint of gradual vision loss over the past 3 decades. After ophthalmoscopic examination and visual field testing, a diagnosis of CACD was made. In addition to a discussion of this case, an outline of the characteristics, inheritance patterns, differential diagnoses and management of CACD is included.

The purpose of this study is to report clinical, optical coherence tomography (OCT), and fluorescein angiogram/indocyanine green angiography (FA/ICG) findings in patients with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) enzyme deficiency in two siblings. A 13-year-old girl and her 14-year-old brother presented with progressive decrease in central vision. Clinically, there were blond-looking fundi, diffuse retinal pigment epithelial (RPE) disruption/atrophy in the macula and peripheral retina with choriocapillaris atrophy in both of them. OCT showed RPE irregularity and diffuse disruption of the RPE layer. FA/ICG imaging demonstrated transmitted choroidal fluorescence secondary to diffuse RPE atrophy with no evidence of leakage. Electroretinogram and electrooculogram findings were suggestive of primary abnormality of pigment epithelium. The boy died of cardiac/respiratory illness, whereas his sister is alive at the last follow-up. Abnormal chorioretinal findings in LCHAD patients should be carefully followed. Regular follow-up is recommended to monitor the ocular and systemic status.

Objective: Neonatal screening and earlier diagnosis have improved the prognosis of long-chain 3-hydroxyacyl coenzyme A dehydrogenase (LCHAD) deficiency, which causes a need to refine the staging of the pigmentary chorioretinopathy and thus improve monitoring and comparability of patients under dietary therapy. Methods: Seven children with LCHAD deficiency caused by homozygous G1528C mutation attended sequential fundus photography for stage 2 chorioretinopathy in 1997–2006. After arranging 21 pairs of fundus photographs according to the severity of the fundus changes, the images best representing 3 different grades of pigmentary deposits (P1–P3) and retinal pigment epithelial (RPE) atrophy (A1–A3) were chosen as reference photographs. To evaluate the substaging, 29 pairs of photographs were graded according to the reference photographs. Results: In the assessment of pigmentary deposits, the 3 ophthalmologists agreed in 41% and differed by a single substage in 45% of instances (combined weighted ĸ statistic was 0.38, indicating moderate agreement). In pairwise comparisons, the weighted ĸ statistic ranged from 0.31 to 0.56 (agreement, 71–81%). In the assessment of RPE atrophy, all 3 raters agreed in 17% and 2 raters in 70% of instances (combined ĸ statistic 0.018, indicating poor agreement). Discussion: Despite variation in imaging techniques and limitations in the visual assessment of fundus photographs, the agreement obtained in grading the pigmentary deposits was comparable to that reported for photographic grading of retinopathy of prematurity. We recommend photographic documentation and substaging based on reference photographs in the follow-up of LCHAD retinopathy. The refined staging allows a more detailed assessment on the progression of the retinopathy and optimization of the therapeutic protocols in individual patients and between centres using different therapeutic protocols.

Purpose To recall that fluorescein angiography (FA) is not only the basis of our knowledge but also mandatory to improve our understanding. Methods Macular disciform lesions have been described and drawed since about 150 years. It is only in 1977 with the advent of fluorescein that the connection with choroidal new vessels (CNV) was performed. The identification of drusen as precursors of CNV was the following stage. With time a number of precursors and clinical forms of macular degeneration were described. Results Currently, the precursors (Age-Related Maculopathy) are distinguished from the neovasdcular or atrophic complications (Age-Related Macular Degeneration). The precursors presenting an early hyperfuorescence can be either hard drusen or RPE atrophy distinguished from each other on the late phase of FA.The late hyperfluorescence of soft drusen particularly when confluent requires a careful analysis of the complete FA sequence to ensure the diagnosis. The neovascular stage presents mainly as sub epithelial occult lesions of which the other types develop that display different angiographic behaviours. Specific aspects have been described gradually based on their FA features. The atrophic stage of the disease seems to behave in a stereotyped way but sub goups are presently identified. Conclusion The fluorescein features of the different component of AMD remain the reference for all other more recent imaging technics and helps to understand and differentiate the various aspects of the disease.

Atrophic form of “Dry AMD” is defined by areas of RPE atrophy, often resulting from regression of confluent soft drusen.

Non-exudative, age-related macular degeneration, which accounts for 85 percent of AMD cases, includes a spectrum of macular pathology. In its most mild form, the macula possesses few Drusen or retinal pigmentary epithelial (RPE) change with no visual disability. At the other end of this spectrum, the macula is affected by large areas of RPE atrophy, with devastating loss of central vision.

Correlate OCT-derived measures of drusen and retinal pigment epithelium (RPE) atrophy areas (RAs) with demographic features in an elderly population.Subjects aged 50 years and older underwent Cirrus OCT scanning. Drusen area and volume were obtained from the macula within a central circle (CC) of 3 mm and a surrounding perifoveal ring (PR) of 3-5 mm, using the RPE analysis software (6.0). RA measurements were generated for the 6 × 6 mm(2) retinal area. Gender, age, smoking status, and systolic blood pressure (SBP) were considered.A total of 434 eyes were included. RA was larger in women (0.63±0.16 vs 0.26±0.08 mm(2), P=0.05) and with increasing age. The PR drusen area increased with increasing age (P0.001), whereas the CC drusen area remained stable after the age of 70 years (0.25±0.06 mm(2) for ages 70-79 years and 0.25±0.07 mm(2) for ages80 years). Drusen volume in the CC was smaller after the age of 80 years (0.009±0.003 mm(3)) compared with the 70- to 79-year-old group (0.02±0.008 mm(3)). Drusen measurements were similar between smokers and nonsmokers, but the PR drusen area (0.29 mm(2), P=0.05) and volume (0.40 mm(3), P=0.005) were correlated with years smoked. RA (0.24 mm(2), P=0.10), PR drusen area (0.29 mm(2), P=0.05), and volume (0.40 mm(3), P=0.005) were found to be directly associated with SBP. There was a high correlation between the eyes of the same subject.OCT-based automated algorithms can be used to analyze and describe drusen and geographic atrophy burden in such population-based studies of elderly patients.

We report a case of Type 2A idiopathic parafoveal telangiectasis treated by Photodynamic Therapy (PDT), resulting in retinal pigment epithelial (RPE) atrophy corresponding to the size of the laser spot used. This raises a doubt regarding the safety of photodynamic therapy in CNVM secondary to type 2A parafoveal telangiectasis.

• We compared the peripapillary scleral and choroidal halos and crescents in the two eyes of 42 patients with unilateral glaucoma. In most cases, the edge of the three tissue layers (the retinal pigment epithelium [RPE], the choroid, and the sclera) that encircle the optic nerve head of the glaucomatous eye superimposed exactly on the mirror images of the edges in the fellow nonglaucomatous eye. Although the size of the peripapillary crescent or halo was the same in both eyes, it and the scleral rim were often more conspicuous in the eye with glaucomatous damage because the tissue edges were seen more easily through the reduced thickness of nerve fiber layer tissue. There were nine exceptions. In five cases, the peripapillary choroidal crescent (the area of choroid not covered by RPE) was larger in the glaucomatous eye. In four eyes, however, the crescent was larger in the nonglaucomatous eye, although the magnitude of the asymmetry was less in these four cases. Thus, in late stages of optic nerve damage, there was some RPE atrophy, but in most cases of glaucoma, the area of bared choroid was the inherent anatomic configuration of the optic nerve exit canal.