Loss Of Pigment Epithelium Research Articles

Acute retinal pigment epitheliitis during treatment of hyperprolactinaemia

BackgroundAcute retinal pigment epitheliitis (ARPE) is a rare, idiopathic and self-limiting disease. The article aims to present ARPE in a patient using D2 dopamine receptor agonists for the treatment of hyperprolactinemia.Case presentationA 28-year-old female during hyperprolactinaemia treatment suffered from a dyschromatopsia and a central visual field defect in the left eye. She noticed a deterioration of vision and discontinued the cabergoline administration. The woman had not been diagnosed with other chronic conditions and exhibited no symptoms of infection. Upon admission, the patient was subjected to a test for COVID-19, which was negative.The ophthalmological examination revealed a decrease in visual acuity to distance in the left eye, which amounted to 18/20 on the Snellen chart. A central scotoma was noted on the Amsler chart and a loss of pigment epithelium was visible on the fundus of the left eye. Fluorescein angiography showed a discrete window defect in the left one, with no signs of leakage. Optical coherence tomography (OCT) scans of the maculae revealed a characteristic change in the photoreceptor layer and retinal pigment epithelium (RPE) in the fovea in the left eye. The electrophysiological tests revealed decreased function of cells in macular region.A magnetic resonance imaging (MRI) of the head and orbits demonstrated an asymmetric pituitary gland without chiasm compression and discrete signal enhancement from the left optic nerve.The patient underwent observation during hospitalisation. She reported improved colour vision and a decreased scotoma in the centre of her visual field.In regular outpatient follow-ups, successive improvements in visual acuity, as well as a decreased RPE damage and outer photoreceptor layer loss during an OCT test were observed.ConclusionsA case of ARPE is reported in a patient taking medications for hyperprolactinemia. The role of dopamine receptor antagonists in the photoreceptor function and causation of ARPE needs further evaluation.

Progression Dynamics of Early versus Later Stage Atrophic Lesions in Nonneovascular Age-Related Macular Degeneration Using Quantitative OCT Biomarker Segmentation

To investigate the progression of geographic atrophy (GA) secondary to non-neovascular age-related macular degeneration (AMD) in early and later stage lesions using AI-based precision tools. Retrospective analysis of an observational cohort study. Seventy-four eyes of forty-nine patients with at least one complete retinal pigment epithelial and outer retinal atrophy (cRORA) lesion secondary to AMD were included. Patients were divided between recently developed cRORA and lesions with advanced disease status. Patients were prospectively imaged by spectral domain optical coherence tomography (SD-OCT) volume scans. The study period encompassed 18 months with scheduled visits every 6 months. Growth rates of recently to cRORA converted lesions were compared with lesions in an advanced disease status using mixed effect models. The progression of retinal pigment epithelial loss (RPEL) was considered the primary endpoint. Secondary endpoints consisted of external limiting membrane disruption (ELMD) and ellipsoid zone loss (EZL). These pathognomonic imaging biomarkers were quantified using validated deep learning algorithms. Further the EZ/RPE-Loss ratio was analysed in both study cohorts. Mean [95%CI] square root progression of recently converted lesions was 79.676[-77.140 - 236.493], 68.219[-101.210 - 237.648) and 84.825[-124.816 - 294.467] mm/half year for RPEL, ELML and EZL respectively. Mean square root progression of advanced lesions was 131.737[-22.574 - 286.047], 129.964[-36.666 - 296.594] and 116.837[-90.556 - 324.301] mm/half year for RPEL, ELML and EZL respectively. RPEL (p=0.038) and ELMD (p=0.026) progression showed significant differences between the two study cohorts. Further recent converters had significantly (p<0.001) higher EZ/RPE-Loss ratios in all time points compared to patients in an advanced disease status (1.716 [1.135 - 2.296] versus 1.153 [0.576 - 1.729]). Early cRORA lesions have a slower growth rate in comparison to atrophic lesions in advanced disease stages. Differences in growth dynamics may play a crucial role in understanding the pathophysiology of non-neovascular AMD and for the interpretation of clinical trials in GA. Individual disease monitoring using AI-based quantification paves the way towards optimized GA management.

Performance of a temperature-controlled shape-memory pupil expander for cataract surgery.

To perform ex vivo and in vivo validation of a manufactured, optimized shape-memory pupil expander and compare its performance to that of existing devices. National University of Singapore and SingHealth Academy. Prospective randomized blinded assessment. Shape-memory expanders were manufactured by overmolding and were inserted into ex vivo porcine eyes and in vivo monkey eyes for validation. The shape-memory expander was compared to the Malyugin ring, OASIS iris expander, and iris hook. After insertion and removal of the devices, the eyes were fixed, and the iris images were analyzed. The shape-memory was successful in pupil expansion for both in vivo and ex vivo experiments. Subsequent ex vivo device comparison revealed iris pigment epithelial loss in 36.4% of eyes for the iris hooks, 30.8% for the iris expander, and 20.0% for the Malyugin ring. Sphincter tears were observed in 27.3% of eyes for the iris hooks and 10.0% for the Malyugin ring. No observable tissue irregularities were observed in the shape-memory expander. The shape-memory expander was optimized to minimize stress exerted onto the iris tissue. The in vivo and ex vivo experimental validation demonstrate efficacy in engineering design and further highlight the translational potential of smart materials in implant development to improve patient healthcare.

Best vitelliform macular dystrophy in a large Brazilian family

BackgroundTo describe the clinical and multimodal imaging findings of a Brazilian family with Best vitelliform macular dystrophy.MethodsA retrospective chart review of a Brazilian family was conducted and complementary fundus images (color photography, autofluorescence, fluorescein angiography and optical coherence tomography) were analyzed.ResultsSeven patients had typical macular lesions at different stages of Best vitelliform macular dystrophy. Electrooculography was performed in two of them and showed abnormal Arden ratio. The pedigree strongly suggests an autosomal dominant inheritance. Low visual acuity was mainly associated with advanced age, retinal pigment epithelium atrophy, and photoreceptors damage. However, yellow subretinal deposits were evidenced in patients with better visual acuity.ConclusionWe present the largest case series of a Brazilian family with Best vitelliform macular dystrophy. Multimodal imaging analysis is important to determine retinal abnormalities. Retinal pigment epithelium atrophy and loss of photoreceptors outer segments seem to be a late but important finding related to severe visual acuity impairment.

Irreversible laser‐induced maculopathy in a child

We would like to bring to the attention of paediatricians the increasing incidence of irreversible retinal injury in children due to commercially available handheld lasers.1, 2 Recently, a 14-year-old male was referred to our service for evaluation of a presumed inherited retinal degeneration. The patient reported bilateral visual decrease over a course of 6 months. There was no significant medical or family history. Visual acuities were recorded as 6/18 right eye and 6/36 left eye. Ophthalmoscopy demonstrated vertical retinal scars bilaterally that involved the central fovea; the region of highest visual acuity. Infrared reflectance imaging demonstrated increased reflectivity at the site of scars due to glial remodelling and also photoreceptor and retinal pigment epithelial loss (Fig. 1). Upon questioning, the mother reported purchasing a handheld laser from an online website 6 months ago. The child admitted that he would frequently activate the laser and attempt to fixate on the flash as it reflected off the bedroom mirror. Visual acuity in his right eye deteriorated to 6/36 over the ensuing 2 months without improvement. In the last 5 years, the rise in the number of cases of laser-induced retinopathy in the ophthalmic literature has paralleled the rise in the number of handheld lasers sold by online merchants. From 2011 to 2012, the Sydney international mail centre alone reported a 60% increase in laser pointers seized.3 The interaction between thermal energy, due to lasers, and retinal tissue can result in necrosis of photoreceptors and catastrophic visual loss. Retinal lesions due to laser-induced injury typically have a vertical orientation and correspond to the direction of eye movement during the involuntary Bell reflex elicited by a bright flash. The vast majority of handheld lasers that are commercially available are of a specified wavelength and power such that it cannot cause injury to ocular structures. However, a small proportion of lasers exceed the specifications reported on the label and in most instances these lasers are manufactured in countries where the industry is not regulated. A recent case of laser-induced retinopathy showed an online-acquired laser labelled with a power output of <5 mW had an actual measured output of greater than 100 mW.4 Laser-induced retinal injury is physically significant and may impair quality of life by limiting job opportunities and the ability to qualify for a driver's licence in later life. Physicians should therefore be aware of this important cause of visual injury in children that is showing an alarming trend upwards.

PREDICTING RESPONSE OF EXUDATIVE AGE-RELATED MACULAR DEGENERATION TO BEVACIZUMAB BASED ON SPECTRALIS OPTICAL COHERENCE TOMOGRAPHY.

To identify baseline optical coherence tomography factors in exudative age-related macular degeneration that predict response to bevacizumab injections. Patients underwent spectral domain optical coherence tomography at diagnosis and the width, height, area, and location of the subretinal fluid, intraretinal fluid, pigment epithelial detachment, and subretinal tissue were measured. The location and size of photoreceptor and the loss of retinal pigment epithelium were recorded as well as quantitative retinal measurements. Patients received three consecutive monthly injections of bevacizumab after which their best-corrected visual acuity was recorded. Overall 105 eyes of 105 patients aging 88 ± 8.6 years were included. In univariate correlational analyses, only subretinal fluid width demonstrated a significant positive correlation with improved best-corrected visual acuity (R = 0.230, P = 0.018). Eyes with intraretinal fluid (P = 0.020) and retinal pigment epithelial loss (P = 0.009) located in the subfoveal (as opposed to the juxtafoveal area) demonstrating worst visual outcomes. In stepwise backwards regression, the subretinal fluid width and intraretinal fluid location were the only parameters that remained significant explaining 9.23% of the variation in delta best-corrected visual acuity scores. Improvement in best-corrected visual acuity after three injections of bevacizumab can be predicted from optical coherence tomography measurements. Specifically, the authors identified subretinal fluid width and intraretinal fluid location as significant markers.

Cystoid macular oedema and epiretinal membrane formation during progression of chloroquine retinopathy after drug cessation

AimsTo evaluate progression of morphological alterations in chloroquine (CQ) or hydroxychloroquine (HCQ) retinopathy after drug cessation.MethodsEleven female patients (age range at drug cessation 46–78 years; treatment duration 5–20 years) were...

Retinal Structure and Function in Achromatopsia: Implications for Gene Therapy

To characterize retinal structure and function in achromatopsia (ACHM) in preparation for clinical trials of gene therapy. Cross-sectional study. Forty subjects with ACHM. All subjects underwent spectral domain optical coherence tomography (SD-OCT), microperimetry, and molecular genetic testing. Foveal structure on SD-OCT was graded into 5 distinct categories: (1) continuous inner segment ellipsoid (ISe), (2) ISe disruption, (3) ISe absence, (4) presence of a hyporeflective zone (HRZ), and (5) outer retinal atrophy including retinal pigment epithelial loss. Foveal and outer nuclear layer (ONL) thickness was measured and presence of hypoplasia determined. Photoreceptor appearance on SD-OCT imaging, foveal and ONL thickness, presence of foveal hypoplasia, retinal sensitivity and fixation stability, and association of these parameters with age and genotype. Forty subjects with a mean age of 24.9 years (range, 6-52 years) were included. Disease-causing variants were found in CNGA3 (n = 18), CNGB3 (n= 15), GNAT2 (n= 4), and PDE6C (n= 1). No variants were found in 2 individuals. In all, 22.5% of subjects had a continuous ISe layer at the fovea, 27.5% had ISe disruption, 20% hadan absent ISe layer, 22.5% had an HRZ, and 7.5% had outer retinal atrophy. No significant differences in age (P= 0.77), mean retinal sensitivity (P= 0.21), or fixation stability (P= 0.34) across the 5 SD-OCT categories were evident. No correlation was found between age and foveal thickness (P= 0.84) or between age and foveal ONL thickness (P= 0.12). The lack of a clear association of disruption of retinal structure or function in ACHM with age suggests that the window of opportunity for intervention by gene therapy is wider in some individuals than previously indicated. Therefore, the potential benefit for a given subject is likely to be better predicted by specific measurement of photoreceptor structure rather than simply by age. The ability to directly assess cone photoreceptor preservation with SD-OCT and/or adaptive optics imaging is likely to prove invaluable in selecting subjects for future trials and measuring the trials' impact.

Iris-fixated posterior chamber intraocular lenses: A must know for all anterior segment surgeons

Dear Editor, We read with great interest, the article reviewing the technique of retrofixated iris-claw lenses, its safety and efficacy in aphakic patients without capsular support, by Rao and Sasidharan[1] and congratulate them on the same. We have done this procedure in 32 eyes and agree with the authors, that this is a good procedure. However, we would like to highlight a few important aspects to understand this surgery better. The original lens used in this technique was the Artisan iris-claw intraocular lenses (IOL) (Ophthec BV, Groningen, The Netherlands). Like the authors, we used the lens by Excel optics, Chennai, which has three models. We prefer model no: PIC 4272, as we found it the easiest to enclave IOL power calculation is usually calculated using the SRK-T formula aiming for a 0.5-1 diopter residual myopia. There is a need to individualize the surgeon factor dependent A constant after a few surgeries Regarding the surgical technique, either superior or temporal approach can be used. The surgery can be done using an anterior chamber maintainer, pars plana infusion canula, or by liberal use of viscoelastics; since the eye is soft following vitrectomy. Though the surgical technique using only viscoelastics is easier, there is a greater incidence of postoperative IOP spikes as it is difficult to remove the entire viscoelastic agent from the posterior segment after the iris-claw lens is inserted. Once the first haptic is enclaved, great care must be taken while positioning the second haptic behind the iris as excessive manipulation can lead to iridodialysis. In case the iris is torn, for example following trauma or if there is excessive mydriasis, this procedure can be combined with a Siepser pupilloplasty Ovalization of pupil is the most common complication of this surgery and is caused by enclaving too close to the pupillary margin. Also, enclavation should not be done too close to the root to prevent interference with iris movement. Postoperative dilatation is unaffected if the technique is correct. The role of an iridectomy is debatable with some studies showing that it is not required and they did not see secondary pupillary block glaucoma.[2] We do it in all cases Postoperative inflammation is usually due to excessive intraoperative manipulation. There is also concern about pigment epithelial loss and dispersion, though studies have shown that progressive pigment dispersion and glaucoma are not common late complications;[3,4] probably due to the vaulted design of these lenses In the future, there also may be multifocal and toric retrofixated lenses, thereby offering these patients even better postoperative vision. In conclusion, implantation of the iris-claw IOL behind the iris better preserves the anatomy of the anterior segment with respect to iridocorneal angle.[2] This is a technique that every anterior segment surgeon must have in their armamentarium.

Loss of Retinal Pigment Epithelium Associated with Bilateral Diffuse Uveal Melanocytic Proliferation.

A 69-year-old woman was evaluated for poor visual acuity in both eyes despite recent cataract extractions. She had bilateral distinct melanocytic lesions in the posterior uvea and retinal pigment epithelial loss over these lesions and over normal-appearing choroid. Fluorescein angiography showed patchy areas of mottled hypofluorescence and hyperfluorescence. Optical coherence tomography demonstrated focal deposition of material over atrophic retinal pigment epithelium. Electroretinography showed decreased a- and b-wave amplitudes under scotopic and photopic conditions. The diagnosis of bilateral diffuse uveal melanocytic proliferation immediately led to the discovery of bronchogenic carcinoma and the patient died 10 days later. This case study suggests that different paraneoplastic stimuli may be operational over the retinal pigment epithelium and uveal melanocytes and that certain features of bilateral diffuse uveal melanocytic proliferation and cancer-associated retinopathy may coexist.

This issue of Acta

This issue of Acta is dominated by paediatric ophthalmology. The cover illustrations show fundus photographs and optical coherence tomography (OCT) images from a girl with 3-hydroxyacyl-CoA dehydrogenase deficiency as reported by Teär Fahnehjelm et al. in this issue. Teär Fahnehjelm discusses the role of paediatric ophthalmologists and their interaction with paediatricians. The second editorial is the yearly report by the Editor in Chief on the state of the journal. Berker & Batman, in Ankara, review several approaches to the surgical treatment of central retinal vein occlusion. Teär Fahnehjelm et al., in Stockholm, found dry eye to be common in children treated with stem-cell transplantation, particularly in patients treated with high levels of cyclosporine A. These children need intensive ophthalmological care. Lindqvist et al., in Trondheim, found that children born prematurely with very low birth weight are at risk for poor binocular function and eye movement in adolescence, whereas this does not apply to very low birth weight children who are born at term. Lavezzo et al., in São Paulo, found that newborn babies blink their eyes slower and less frequently than 4–6-year-old children. Uretmen et al., in Turkey, report that accommodative esotropia developed in 50% of children who underwent surgical treatment for presumed infantile esotropia. Teär Fahnehjelm et al., in Sweden, describe the ocular characteristics of a group of children with 3-hydroxyacyl-CoA dehydrogenase deficiency with variable degrees of chorioretinal pathology and visual impairment. Soliman et al., in Copenhagen and Egypt, propose that OCT analysis can predict the visual outcome 1 month following laser photocoagulation for diffused diabetic macular oedema. Maca et al., in Vienna, used the retinal thickness analyser to demonstrate that cystoid macular oedema is common in children with uveitis – even some with good visual acuity. Mahdizadeh et al., in Iran, found that retinal cryopexy did not benefit the anatomical reattachment rate from scleral buckling surgery. Donati et al., in Geneva, found that both necrosis and apoptosis are involved in neuronal cell death following experimental retinal vein occlusion in pigs. Krøyer et al., in Copenhagen, described the effect of acute retinal pigment epithelial loss, which destroyed scotopic but not photopic retinal function in a 68-year-old woman. Bertschinger et al., in Geneva, used the Vryghem macular function test and found this to be reliable in predicting postoperative visual acuity after cataract surgery. Cillino et al., in Italy, found Gore-Tex® implants to be useful adjuvants in glaucoma filtration surgery. Yoeruek et al., in Tübingen, used Avastin® eyedrops to reduce corneal damage and neovascularization following experimental chemical burns to the cornea. Al-Jamal & Kivelä describe an optic nerve tumour leading to progressive visual loss in a young man. I hope that this issue of Acta offers an opportunity to brush up on our knowledge in paediatric ophthalmology and provides educational opportunities in all subspecialties of ophthalmology.

Canine goniodysgenesis‐related glaucoma: a morphologic review of 100 cases looking at inflammation and pigment dispersion

To investigate the role of pigment dispersion and inflammation in the pathogenesis of goniodysgenesis-related glaucoma (GDRG). Cases of GDRG were selected when the duration of the disease was specified and there was not any confounding pathology. Cases were grouped into < or = 7-day (acute), and > 7-day (chronic) durations, based on the time required to effect end-stage retinal damage. Acute cases were further divided into < 4-day and 4-7-day groups to assess peracute changes. Slides were evaluated for four individual signs of pigment dispersion: segmental loss of posterior iris pigment epithelium, clumping of posterior iris pigment epithelium, pigmented cells in the trabecular meshwork or anterior chamber and preferential settling of pigmented cells in the ventral aspect of the iridocorneal angle. Slides were also evaluated for the presence of neutrophils and/or lymphoplasmacytic cells in the trabecular meshwork (TM). Differences between groups were analyzed statistically. Of 100 cases evaluated, 34 were < or = 7-days (acute) (14 < 4-day and 20 4-7-day) and 66 were > 7-days (chronic) in duration. Of all globes examined, 96% had at least one sign of pigment dispersion, with no significant difference between groups. Two or more signs of pigment dispersion were present in 76% of all globes. The 4-7-day group was significantly more likely than the < 4-day group to have at least two signs. The difference was not significant between < or = 7- and > 7-day groups. Neutrophils were present in the TM of 86% of < 4-day and 50% of 4-7-day cases. Cases in the < or = 7-day group were significantly more [corrected] likely than > 7-day cases to have neutrophils in the TM, with 65% and 17% [corrected] positive cases, respectively. Lymphoplasmacytic inflammation was present in 53% of all cases, with no significant difference between groups. Cases in the < or = 7-day group were significantly more likely than > 7-day cases to have both types of inflammation. Our results indicate that both acute inflammation and pigment dispersion may be key factors in the pathogenesis of GDRG. Pigment dispersion is prevalent at all time points and increases during the first 7 days. The finding of iris pigment epithelial loss supports the theory that pupillary block associated with iris-lens touching may be important in the pathogenesis of GDRG.

Endoscopy for evaluation and treatment of the ciliary body in hypotony.

To determine the clinical utility of the intraocular videoendoscope for evaluation of the ciliary body in eyes with hypotony and as a visualization tool for surgery of the ciliary body. A gradient index of refraction rod endoscope was used to evaluate the ciliary body in 14 patients. Videotapes and charts were reviewed retrospectively to correlate the appearance of the ciliary body and to analyze the clinical findings and surgical results. Videoendoscopic surgery to remove fibrous tissue from the ciliary processes was performed in nine eyes. The endoscope produced a clear detailed image of the ciliary body in a state that is undisturbed by scleral depression or other forms of mechanical distortion. In eyes without hypotony the normal ciliary processes are uniformly pigmented with a velvet-like surface at high magnification. White surface changes in the peak and crest of individual ciliary processes (white-caps) were seen in 11 of 14 patients with hypotony. At high magnification the white surface changes had a brush-like texture integral to the ciliary processes. Other findings in hypotony were fibrous covering of the ciliary body in 10 patients, loss of pigment epithelium in 5, traction elongation of the ciliary processes in 8, vascular loops in 4, pigmented sheets in 3, and areas of missing ciliary processes in 1. White caps were present in seven of the nine patients who had surgery for fibrous traction of the ciliary processes and hypotony and three patients with hypotony not due to other endoscopically visible abnormalities of the ciliary processes. Endoscopically visualized dissection of ciliary body in 9 patients resulted in normal or elevated tension immediately postoperatively in 7 (78%). At the most recent visit only three of the patients had normal tension, but for these nine patients the pressures were marginally, significantly (P = 0.08) higher compared to the preoperative pressures. The evaluation and management of hypotony is enhanced by the use of intraocular videoendoscopy. Clinical findings of whitecaps and traction elongation of the ciliary processes were associated with ocular hypotony. The endoscope facilitated surgery for dissection and removal of fibrous tissue over the ciliary processes. Further studies are needed to better understand the pathophysiology of ocular hypotony.

Limited macular translocation for the management of subfoveal retinal pigment epithelial loss after submacular surgery

PURPOSE: To report a case of subfoveal retinal pigment epithelial (retinal pigment epithelium) loss after submacular surgery managed successfully by limited macular translocation. METHODS: Case report. RESULTS: A 28-year-old woman presented with a visual acuity of 20/100 caused by subfoveal choroidal neovas-cularization secondary to ocular histoplasmosis syndrome. Submacular resection of the choroidal neovascularization was complicated by inadvertent retinal pigment epithelium loss from beneath the foveal center. She underwent limited macular translocation 5 days after the initial surgery and had successful displacement of the fovea to an area inferior to the retinal pigment epithelium defect. Her visual acuity was 20/60 4 months postoperatively. CONCLUSION: This report demonstrates the feasibility of using limited macular translocation for the management of eyes with central retinal pigment epithelium defect after submacular surgery and extends the clinical indications for limited macular translocation.

Quantitation of ischemic damage in the rat retina

In order to determine thresholds for irreversible cellular injury in the rat retina, timed acute no-flow ischemic episodes of 30–180 min duration were produced by elevation of intraocular pressure (IOP) above systolic pressure. Quantitation of irreversible degeneration and cell loss following a 2-week postischemic interval was performed by computer-assisted measurements from histologic sections. Alterations of thickness of retinal layers and linear cell density were determined for ischemia of selected durations (30, 60, 80, 90, 120 and 180 min). Different thresholds were evident for inner and outer retinal damage. Neurons of the inner nuclear layers showed extensive loss with episodes at 60 min. Decrease in the thickness of the inner plexiform layer provided the best index of this inner nuclear damage. The outer retina was more resistant, with photoreceptors showing extensive damage only after 90 min in conjunction with pigment epithelial metaplasia and degeneration. Two-hour episodes produced fullthickness degeneration with loss of pigment epithelium and sparing of the peripheral retina. Greater sensitivity of the inner retina suggested problems with restoration of the retinal circulation. Horseradish peroxidase infusions did reveal central microcirculatory defects in retinal wholemounts of some specimens with episodes longer than 60 min. Refinements of the methods resulted in outcomes sufficiently reproducible for quantitative assessment of acute ischemic injury. The rat retina provides an economical basic tissue model of acute ischemic injury affecting neurons, glia, and microvasculature. Quantitation of this injury promises great utility in testing agents with potentially protective effects on acute ischemic injury.

Vertical Folds of Central Retina and Choroid in Sudden Ocular Decompression

Sudden and limited decompression of an eye due to corneal rupture has four days later allowed for the histopathological demonstration of an interesting combination of a vertical retinal fold, cystoid macular edema, degeneration of foveal cones, a vertical choroidal fold with loss of pigment epithelium and papilledema. The rather bizarre changes in this case may be helpful for the understanding of the much more subtle histopathological findings in typical cystoid macular edema.