Hemorrhagic unilateral retinopathy: a report of multimodal imaging in three cases.

PURPOSE: To report three cases of hemorrhagic unilateral retinopathy, diagnosed by multimodal imaging. METHODS: Case report of 3 patients, 2 women and one man, aged 51, 74, and 52, respectively. RESULTS: Symptoms were acute floaters, blurred vision, or central scotoma, unilateral in all cases. The best-corrected visual acuity was 20/20 in the affected eye in 2 patients with a paracentral scotoma, and 20/160 in the third patient. Funduscopic examination revealed multiple unilateral posterior hemorrhages located in the Henle fiber layer in the macula and beneath the internal limiting membrane around the optic disc on spectral-domain optical coherence tomography (SD-OCT). Fluorescein angiography and indocyanine green angiography (ICGA) did not show any vascular abnormalities. SD-OCT angiography (SD-OCT-A) did not show any capillary drop-out or choroidal abnormalities. In all patients, the visual symptoms completely disappeared within a few weeks, with spontaneous regression of the hemorrhages. CONCLUSION: Hemorrhagic unilateral retinopathy is a rarely reported and poorly understood disorder. ICGA and SD-OCT-A did not allow better understanding the condition. No etiology has been associated with this entity so far. The spontaneous resolution of the present cases confirmed the favorable visual prognosis of the condition.

RIDGE-SHAPED PERIPAPILLA.

PURPOSE: To report a case of peripapillary subretinal fluid associated with a ridge-shaped morphology surrounding the optic disk, which we termed ridge-shaped peripapilla. METHODS: Case report. RESULTS: A 6-year-old girl with mild-to-moderate myopia was referred for an abnormal fundus appearance of the left eye. Fundus examination of the left eye showed a vertical whitish elevation just temporal to the disk with pigment clumping. Spectral domain optical coherence tomography of the left eye showed an elevation of the fundus at the temporal edge of the disk with thinning of the choroid overlying the thickened scleral protrusion and a serous subretinal fluid. Fluorescein angiography of the left eye showed a hyperfluorescent area without leakage at the temporal edge of the disk, indicative of retinal pigment epithelium atrophy. There was no sign of choroidal neovascularization. Based on the fluorescein angiography and optical coherence tomography findings, the protrusion of the sclera seemed to result in overlying choroidal thinning with choroidal blood flow disturbances, and consequent retinal pigment epithelium atrophy, leading to the subretinal fluid. CONCLUSION: This case highlights an unusual presentation of ridge-shaped peripapilla, characterized by inward convexity of the peripapillary area with a ridge-shaped morphology and localized thickening of the peripapillary sclera, in eyes with myopia.

Fovea Plana and Fundus Hypopigmentation in Prader-Willi Syndrome.

PURPOSE: To report a case of fovea plana with fundus hypopigmentation in a patient with Prader-Willi syndrome (PWS). CASE REPORT: During a routine examination, fovea plana and fundus hypopigmentation were observed in both eyes in a 34-year-old male patient with PWS, and documented with fundus photography, spectral-domain optical coherence tomography (SD-OCT) and OCT-angiography. CONCLUSION: Fovea plana and fundus hypopigmentation may be associated with PWS. Indeed, both PWS and oculocutaneous albinism (OCA) may be explained by the deletion of the same genomic region on chromosome 15. The present case of a PWS patient with fundus hypopigmentation supports the genetic and clinical overlap between PWS and OCA.

Tilted disc in eyes with fovea plana.

PURPOSE: To report the association of tilted disc (TD) with fovea plana. METHODS: Monocentric retrospective study of consecutive eyes diagnosed with fovea plana, assessed by spectral-domain optical coherence tomography. Analysis of the medical charts and imaging findings of patients to collect demographics, the visual acuity, and the clinical context. The presence of associated conditions was checked by two independent readers in order to classify fovea plana as isolated or part of other conditions. RESULTS: Twenty-one patients, 9 men and 12 women, aged 12 to 91 years, were included. Fovea plana was isolated and asymptomatic in 10 (47.6%) patients. In 6 (28.5%) patients, fovea plana was associated with ocular albinism and/or nystagmus. In 6 (28.5%) patients, fovea plana was associated with an obliquity of the optic disc typical of TD, isolated (5 cases), or associated with nystagmus (1 case). CONCLUSION: An association between TD and fovea plana had been reported only once in the literature and had been considered likely coincidental. However, this association could be more common than initially reported and suggests a common pathological process in eye development during embryogenesis.

LONG-TERM PROGNOSIS OF CHOROIDAL NEOVASCULARIZATION COMPLICATING ANGIOID STREAKS.

PURPOSE: To report the very long-term visual prognosis of choroidal neovascularization complicating angioid streaks in the antivascular endothelial growth factor era. METHODS: Retrospective monocentric study aimed at analyzing patients' demographics, choroidal neovascularization features, angioid streak-associated conditions, and previous and current therapies for choroidal neovascularization. The main outcome measures were the quantitative measurement of central retinal pigment epithelial atrophy enlargement by comparing the ratio of pixels involved on automated infrared images acquired by spectral-domain optical coherence tomography and the changes in best-corrected visual acuity. The secondary outcome measures were the number of intravitreal injections and the changes in central choroidal thickness and central retinal thickness. Subgroup analyzes were performed to compare macular atrophy extent between eyes of patients with or without proven pseudoxanthoma elasticum ("PXE" or "no PXE") and between eyes previously treated or not with photodynamic therapy ("PDT" or "no PDT"). RESULTS: Thirty-three eyes of 23 patients were included. The mean best-corrected visual acuity decreased significantly from 66 ± 19 Early Treatment Diabetic Retinopathy Study letters at the time of the first antivascular endothelial growth factor injection to 52 ± 23 Early Treatment Diabetic Retinopathy Study letters at the end of the follow-up (mean follow-up duration: 109 ± 42 months, range: 47-175 months). The ratio of central retinal pigment epithelial atrophy enlargement was 201%, 110%, 240%, and 111% in the PXE, no PXE, PDT, and no PDT groups, respectively. CONCLUSION: Despite the use of antivascular endothelial growth factor agents, the very long-term prognosis appeared relatively poor, especially in patients with PXE. This study also suggests that PDT should be used with caution in the management of choroidal neovascularization in eyes with angioid streaks.

Long-Term Resolution of Perifoveal Exudative Vascular Anomalous Complex after Intravitreal Injections of Anti-Vascular Endothelial Growth Factor.

Perifoveal exudative vascular anomalous complex (PEVAC) is a perifoveal aneurysmal vascular lesion found in healthy subjects. A 68-year-old woman was diagnosed with a typical unilateral and unifocal PEVAC lesion after extensive multimodal imaging and was treated with three-monthly intravitreal injections of ranibizumab. An immediate and complete resolution of the intraretinal fluid was observed. Visual acuity returned to 20/20 without any recurrence of the exudative signs along the 5 years of follow-up. Therefore, an initial anti-VEGF treatment with three-monthly intravitreal injections may be considered as a first-line treatment in PEVAC lesions and may result in long-term preservation of visual acuity.

Atypical case of perifoveal exudative vascular anomalous complex associated with pachychoroid pigment epitheliopathy.

Purpose: To report an unusual association of a perifoveal exudative vascular anomalous complex (PEVAC) and a bilateral pachychoroid pigment epitheliopathy (PPE), which responded positively to anti-vascular endothelial growth factor (VEGF) intravitreal injections (IVI). Observations: A 44 year-old man with no significant medical or ocular history, complained of unilateral blurred vision in his right eye (RE) over several months. On examination, best corrected visual acuity (BCVA) was 75 letters in the RE and 85 in the left eye (LE). Fundus examination in the RE showed a large perifoveal aneurysmal lesion with a macular thickening, small hemorrhages and linear hard exudates accumulation, associated with multifocal retinal pigment epithelium (RPE) changes in the posterior pole of both eyes. Optical coherence tomography of the RE showed the PEVAC as a large round retinal capillary aneurysm with surrounding intraretinal fluid, associated with serous and drusenoid RPE elevations in both eyes, consistent with PPE. Subfoveal choroidal thickness was more than 500 µm in both eyes, with several dilated choroidal veins. Fluorescein angiography showed, in the RE, the hyperfluorescent aneurysmal lesion with late leakage, associated with scattered hyperfluorescent areas in the posterior pole of both eyes. Indocyanine green angiography showed, in the RE, the same hyperfluorescent lesion but without leakage, associated with areas of choroidal hyperpermeability in both eyes. After 2 anti-VEGF IVI in the RE, good functional and anatomical improvement was observed. After 10 months of follow-up, there was no evidence of new exudation. BCVA remained stable and RPE abnormalities remained unchanged. Conclusion and importance: We describe an atypical case of PEVAC associated with PPE, which responded positively to anti-VEGF therapy. To our knowledge, this is the first report of a patient presenting PEVAC and diseases of the pachychoroid spectrum. Further studies, assessing the choroid in PEVAC, are required to investigate the hypothetical relationship between these 2 entities and the efficiency of anti-VEGF therapy.

Optical coherence tomography angiography quantification of choriocapillaris blood-flow after half-fluence photodynamic therapy for chronic central serous chorioretinopathy.

PURPOSE: To quantitatively analyze characteristics of choriocapillaris flow using spectral domain optical coherence tomography angiography (SD-OCTA) in eyes with chronic central serous chorioretinopathy (CSC) before and after treatment by photodynamic therapy (PDT). METHODS: Retrospective interventional study. Macular 3X3 SD-OCT scans were analyzed in eyes diagnosed with chronic CSC before and after treatment with half-fluence PDT. The choriocapillaris en face slabs were extracted from the SD-OCTA device after manual segmentation. En face choriocapillaris flow images were compensated with en face choriocapillaris structure images. Phansalkar local thresholding method was then used with a radius of 4 and 8 pixels. Percentage of flow deficits (FD%), number, size, and total area of FDs were computed for comparison, before and after treatment by half-fluence PDT. RESULTS: Mean choriocapillaris FD% before PDT was of 58.36 + / - 11.88 and of 60.82 + / - 11.08 after PDT using radius 4 pixels with no significant difference (p = 0.140). Mean choriocapillaris FD% was of 58.63 + / - 11.08 before PDT and of 60.87 + / - 10.36 after PDT using radius 8 pixels with no significant difference (p = 0.200). Similarly, no significative difference was found in number, size, and total area of FDs, before and after half-fluence PDT, using radius 4 and 8 pixels in patients with chronic CSC. CONCLUSION: Using Phansalkar local thresholding method, quantitative analysis of choriocapillaris with SD-OCTA found no significant change in choriocapillaris flow deficits before and after successful half-fluence PDT in patients with chronic CSC. Therefore, it seems that half-fluence PDT may not alter choriocapillaris perfusion, at least on a relative short-term basis.

CHORIORETINAL FOLDS IN PATIENTS WITH CENTRAL SEROUS CHORIORETINOPATHY.

BACKGROUND AND PURPOSE: To the best of our knowledge, there is no study of patients with central serous chorioretinopathy associated with chorioretinal folds, since a short mention in Gass' stereoscopic atlas. We report here six cases with this association. METHODS: Six patients with both conditions were examined in our institution and underwent fluorescein angiography and optical coherence tomography. RESULTS: Patients were 3 men and 3 women, aged 44 years to 82 years. All patients were hyperopic and two received corticosteroids. Fluorescein angiography showed pigmentary changes, diffuse leakage areas typical of chronic central serous chorioretinopathy, and chorioretinal folds mainly located in the upper temporal part of the fundus. Enhanced depth imaging optical coherence tomography was performed in 5 cases and revealed a thick choroid in all cases (mean subfoveal choroidal thickness: 381 µm, range: 280-510 µm). CONCLUSION: Although possibly coincidental, the presence of chorioretinal folds in hyperopic central serous chorioretinopathy eyes could be due to the excessive thickness of the choroid in eyes with hyperopia related to short axial length.

Swept-Source OCTA Imaging of a Presumed Solitary Circumscribed Retinal Astrocytic Proliferation.

Presumed solitary circumscribed retinal astrocytic proliferation (pSCRAP) is a rare, benign retinal tumor, typically presenting as a yellow-white, well-defined lesion. It was first qualified as astrocytic, but thanks to the development of optical coherence tomography (OCT), the lesion was identified as a deep glial lesion, as the nerve fiber layer appeared to be spared. Herein, the authors report the case of a 90-year-old man with inferopapillary pSCRAP who benefited from swept-source OCT angiography images. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:232-235.].

Progression from Type 2 Macular Neovascularization to Fibrovascular Pigment Epithelial Detachment.

The aim of this study was to report unusual progression of type 2 macular neovascularization (MNV) associated with age-related macular degeneration (AMD), high myopia or angioid streaks. Retrospective multicentric observational case series data were used. Eyes that progressed from type 2 MNV secondary to AMD, high myopia or angioid streaks to fibrovascular pigment epithelial detachment (PED) were included. A total of 29 treatment-naive eyes from 29 patients with type 2 MNV secondary to AMD (n = 14), high myopia (n = 10) or angioid streaks (n = 5) that progressed to a fibrovascular PED on Spectral Domain-Optical Coherence Tomography were used. This progression occurred within 3 months after anti-VEGF therapy initiation. Logarithm of minimum angle of resolution (LogMAR) visual acuity improved significantly after anti-VEGF therapy, from 0.55 (SD ± 0.30) (20/63-20/80) at baseline to 0.30 (20/40) at 3 months, and 0.33 (20/40) at the final follow-up (mean follow up: 3.68 years). Mean number of intravitreal injections per year for patients with a total follow-up ≥ 12 months (n = 24) was 4.3 ± 2.1 per year. Progression from type 2 MNV to a fibrovascular PED may occur in patients suffering from AMD, high myopia or angioid streaks. This progression appears early after initiation of anti-VEGF therapy and is associated with a favorable visual and anatomical outcome, at least on a short follow up basis.

INTRARETINAL HYPERREFLECTIVE LINES.

PURPOSE: To report intraretinal hyperreflective lines related to various macular conditions. METHODS: All cases were imaged with color photographs, autofluorescence images, and spectral-domain optical coherence tomography, some with fluorescein and/or indocyanine green angiography. Demographic data, imaging, course and outcome were retrospectively analyzed. RESULTS: Forty-nine eyes of 43 patients (16 men and 27 women) were included. Hyperreflective vertical lines (38 eyes) or curvilinear lines along the Henle fiber layer (11 eyes) were present in association with various macular conditions: adult vitelliform dystrophy or pattern dystrophy (24 eyes) frequently associated with an epiretinal membrane (six eyes) and/or thick choroid (nine eyes), age-related maculopathy or macular degeneration (nine eyes), partial resorption of subretinal or intraretinal hemorrhages (five eyes), idiopathic macular microhole (two eyes), vitreomacular traction (three eyes), multiple evanescent white dot syndrome (three eyes), fundus flavimaculatus (two eyes), and pachychoroid pigment epitheliopathy (one eye). The lines fully vanished in cases of hemorrhages, multiple evanescent white dot syndrome or resolution of vitreomacular traction, but usually persisted with gradual thinning in the other conditions. CONCLUSION: The present series showed that intraretinal hyperreflective lines could occur in various inflammatory, degenerative, or tractional conditions. They could reflect a previously unrecognized reaction to various photoreceptor, Müller cell, and/or retinal pigment epithelium damage.

Outer Foveal Microdefects.

PURPOSE: To report a spectral-domain (SD) OCT clinical sign, outer foveal microdefect (OFMD), corresponding to a focal disruption of the foveal photoreceptors in association with various macular conditions. DESIGN: Retrospective cohort study. PARTICIPANTS: Forty-five patients with OFMD. METHODS: All patients were imaged with color photography and SD OCT, and some were imaged with autofluorescence imaging, fluorescein angiography, indocyanine green angiography, or a combination thereof. Patient demographics, OFMD imaging, courses, and outcomes were analyzed retrospectively. MAIN OUTCOME MEASURES: Demographics, conditions associated with OFMD, and diameter of OFMD. RESULTS: Fifty-one eyes of 45 patients (15 men and 30 women; age range, 10-88 years) were included. Symptoms included central scotoma, metamorphopsia, and mild to moderate visual loss. Outer foveal microdefects were observed in association with various macular conditions: presumed posterior vitreous detachment, vitreomacular interface changes, or both (24 eyes); sequelae of macular edema (3 eyes); blunt trauma (2 eyes); retinal phototoxicity resulting from laser or solar maculopathy (5 eyes); and macular telangiectasia type 2 (2 eyes). An improvement with a reduction in OFMD diameter was documented in 9 of 14 eyes that could be followed up. CONCLUSIONS: We suggest renaming the condition outer foveal microdefect instead of macular microhole, which is usually associated with a vitreomacular disorder. The pattern indeed also was observed in traumatic or degenerative conditions. We assumed that various injuries led to changes in foveal morphologic features and that a full recovery of the foveal architecture could be altered, especially in eyes with findings suggestive of the pachychoroid spectrum of disorders.

Impact of intravitreal aflibercept dosing regimens in treatment-naïve patients with neovascular age-related macular degeneration in routine clinical practice in France: results from the RAINBOW study.

OBJECTIVE: To evaluate 12-month outcomes in treatment-naïve patients with neovascular (wet) age-related macular degeneration (AMD) stratified by intravitreal aflibercept (IVT-AFL) regimen. METHODS AND ANALYSIS: Patients included in the 12-month interim analysis of Real life of intravitreal Aflibercept In FraNce: oBservatiOnal Study in Wet AMD (RAINBOW), a 4-year, ongoing observational study conducted in France, were stratified by IVT-AFL dosing regimen. Safety (n=593) and effectiveness (n=428) data were analysed. Regimens included a regular cohort (three initial monthly IVT-AFL injections and ≥6 injections) and irregular cohorts (<6 injections) with and without three initial monthly injections. The main outcome measure was mean gain in best-corrected visual acuity (BCVA) at 12 months. RESULTS: Mean number of IVT-AFL injections was 6.0 (all patients, n=513), 7.2 (regular cohort, n=102), 6.1 (irregular cohort with three initial monthly injections, n=266) and 5.2 (irregular cohort without three initial monthly injections, n=60). Overall mean gain in BCVA at 12 months was 5 letters; +7.1 letters (regular cohort) and +5.6 letters (irregular cohort with three initial monthly injections), both p<0.001 versus baseline, and -1.1 letters (irregular cohort without three initial monthly injections), p=0.669. Improvements in BCVA were also significantly greater in the regular cohort (p<0.001) and irregular cohort with three initial monthly injections (p=0.003) compared with the irregular cohort without three initial monthly injections. Ocular and non-ocular adverse events were reported in 14.7% and 17.4% of all patients, respectively. CONCLUSION: Treatment-naïve patients with neovascular AMD receiving three initial monthly injections followed by regular or irregular injections over 12 months experienced better visual acuity outcomes than those receiving irregular treatment without three initial monthly injections. TRIAL REGISTRATION NUMBER: NCT02279537.