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Abstract Purpose To determine whether the rare NLRP3-Associated Autoinflammatory Disease (NLRP3-AID) is associated with retinal changes and to assess the ocular involvement. Methods A retrospective cohort study of 20 patients(40 eyes) diagnosed with rare NLRP3-AID at Peking Union Medical College Hospital, from April 2015 to August 2022. Patients underwent a comprehensive ophthalmological examination, including visual acuity, intraocular pressure examination, slit-lamp examination, fundus photography, optical coherence tomography(OCT), and fluorescence angiography (FA). Some patients also underwent optical coherence tomography angiography (OCTA). Results This study analyzed 40 eyes of 20 patients (11 [55.0%] male; median age, 25.0 years [range, 12–52 years]) and 13 patients (26 eyes, 65%) demonstrated ocular involvement. The most common ophthalmologic manifestation was conjunctivitis (22 eyes, 84.6%), followed by papilledema (14 eyes, 53.8%), retinopathy (10 eyes, 38.5%), optic atrophy (6 eyes, 23.1%), uveitis (4 eyes, 15.4%), reduced pupil light reflex (3 eyes, 11.5%) and cataracts (2 eyes, 7.7%). Ocular involvement was bilateral in 11 patients (55.0%). Five kinds of retinal lesions were seen in 5 patients (10 eyes, 25%) with NLRP3-AID, including peripheral retinal vascular leakage, microaneurysms, macular ischemia, macular epiretinal membrane formation and drusen. Conclusions Peripheral retinal vascular leakage, macular ischemia, microaneurysms and drusen are newly identified retinal findings in patients with NLRP3-AID, which suggests the importance of detailed retinal examination in these patients.

Purpose: This study aims to investigate the factors influencing post-treatment visual acuity (VA) in patients with central retinal vein occlusion (CRVO) with macular edema (ME). Methods: The subjects of this study were patients who visited our clinic from May 2013 to July 2019 and who could be followed up with for at least 12 months. Cases with hemi CRVO were excluded from this study. Factors considered in the evaluation of visual prognosis at the 12 months included initial best-corrected VA, central subfoveal thickness, CRVO subtype (nonischemic, ischemic, or converted from nonischemic to ischemic), time taken for the first treatment, number of anti-vascular endothelial growth factor agent injections, structural changes in the inner and outer retinal layers, and the presence of macular ischemia in a multiple regression analysis. Results: There were 41 patients with 41 eyes, 27 males and 14 females. The mean age of the patients was 70.5 ± 12.2 (mean ± standard deviation) years. The mean VA was 0.544 ± 0.576, 0.456 ± 0.568, and 0.586 ± 0.665 at the initial visit, 12 months later, and time of last observation, respectively. There were no significant differences in VAs observed between the baseline, month 12, and final visit. Multiple regression analysis revealed that the external limiting membrane score at month 12 (p = 0.030), the VA at initial visit (p < 0.001), and the presence of severe macular ischemia (p < 0.001) were the key factors associated with VA at month 12. Moreover, severe macular ischemia was identified as the only factor affecting decimal VA less than 20/200 at the last observation (p = 0.0092). Conclusions: Severe macular ischemia is strongly linked to a poor visual prognosis in patients with ME associated with CRVO. [ABSTRACT FROM AUTHOR]

Purpose: To determine whether the rare NLRP3-Associated Autoinflammatory Disease (NLRP3-AID) is associated with retinal changes and to assess the ocular involvement. Methods: A retrospective cohort study of 20 patients(40 eyes) diagnosed with rare NLRP3-AID at Peking Union Medical College Hospital, from April 2015 to August 2022. Patients underwent a comprehensive ophthalmological examination, including visual acuity, intraocular pressure examination, slit-lamp examination, fundus photography, optical coherence tomography(OCT), and fluorescence angiography (FA). Some patients also underwent optical coherence tomography angiography (OCTA). Results: This study analyzed 40 eyes of 20 patients (11 [55.0%] male; median age, 25.0 years [range, 12–52 years]) and 13 patients (26 eyes, 65%) demonstrated ocular involvement. The most common ophthalmologic manifestation was conjunctivitis (22 eyes, 84.6%), followed by papilledema (14 eyes, 53.8%), retinopathy (10 eyes, 38.5%), optic atrophy (6 eyes, 23.1%), uveitis (4 eyes, 15.4%), reduced pupil light reflex (3 eyes, 11.5%) and cataracts (2 eyes, 7.7%). Ocular involvement was bilateral in 11 patients (55.0%). Five kinds of retinal lesions were seen in 5 patients (10 eyes, 25%) with NLRP3-AID, including peripheral retinal vascular leakage, microaneurysms, macular ischemia, macular epiretinal membrane formation and drusen. Conclusions: Peripheral retinal vascular leakage, macular ischemia, microaneurysms and drusen are newly identified retinal findings in patients with NLRP3-AID, which suggests the importance of detailed retinal examination in these patients. [ABSTRACT FROM AUTHOR]

Purpose: To report the spectrum of posterior segment manifestations and visual outcomes in a large series of patients with systemic lupus erythematosus (SLE). Methods: Retrospective study at a tertiary referral eye center in south India between 2016 and 2022. Results: Charts of 109 patients diagnosed to have SLE were retrieved from our medical database. Only nine cases of SLE (8.25%) had posterior segment involvement. The male: female ratio was 1:8. The mean age was 28 years. Unilaterality was the most common presentation in eight cases (88.89%). Lupus nephritis was the most common systemic presentation in five cases (55.56%). Antiphospholipid antibodies (APLA) positivity was seen in two cases (22.22%). Ocular manifestations included microangiopathy (cotton wool spots) in one case, occlusive retinal vasculitis with cotton wool spots in four cases (five eyes), optic disc edema with combined venous and arterial occlusion (one case), central retinal vein occlusion with cotton wool spots and hemorrhages (one case), macular edema (four cases), posterior scleritis with optic disc edema and exudative retinal detachment in the posterior pole (one case), and tubercular choroidal granuloma (one case). Treatment included systemic steroids, hydroxychloroquine sulfate (HCQS), and immunosuppression in all cases, blood thinners in two cases, and laser photocoagulation in four cases. HCQS-related retinal toxicity was not seen in any of the 109 cases. Ocular manifestation was the initial presentation of SLE in one case. Visual outcome was poor in three cases. Conclusion: Presence of posterior segment findings in cases with SLE may suggest a severe systemic disease. Early detection and aggressive treatment result in better visual outcomes. Ophthalmologists could play a vital role in guiding systemic therapy.

Purpose: To report the spectrum of posterior segment manifestations and visual outcomes in a large series of patients with systemic lupus erythematosus (SLE). Methods: Retrospective study at a tertiary referral eye center in south India between 2016 and 2022. Results: Charts of 109 patients diagnosed to have SLE were retrieved from our medical database. Only nine cases of SLE (8.25%) had posterior segment involvement. The male: female ratio was 1:8. The mean age was 28 years. Unilaterality was the most common presentation in eight cases (88.89%). Lupus nephritis was the most common systemic presentation in five cases (55.56%). Antiphospholipid antibodies (APLA) positivity was seen in two cases (22.22%). Ocular manifestations included microangiopathy (cotton wool spots) in one case, occlusive retinal vasculitis with cotton wool spots in four cases (five eyes), optic disc edema with combined venous and arterial occlusion (one case), central retinal vein occlusion with cotton wool spots and hemorrhages (one case), macular edema (four cases), posterior scleritis with optic disc edema and exudative retinal detachment in the posterior pole (one case), and tubercular choroidal granuloma (one case). Treatment included systemic steroids, hydroxychloroquine sulfate (HCQS), and immunosuppression in all cases, blood thinners in two cases, and laser photocoagulation in four cases. HCQS-related retinal toxicity was not seen in any of the 109 cases. Ocular manifestation was the initial presentation of SLE in one case. Visual outcome was poor in three cases. Conclusion: Presence of posterior segment findings in cases with SLE may suggest a severe systemic disease. Early detection and aggressive treatment result in better visual outcomes. Ophthalmologists could play a vital role in guiding systemic therapy. [ABSTRACT FROM AUTHOR]

Abstract Background Diabetic retinopathy, a major complication of diabetes mellitus, is a leading cause of sigh-loss in working age adults. Progressive loss of integrity of the retinal neurovascular unit is a central element in the disease pathogenesis. Retinal ischemia and inflammatory processes drive interrelated pathologies such as blood retinal barrier disruption, fluid accumulation, gliosis, neuronal loss and/or aberrant neovascularisation. Current treatment options are somewhat limited to late-stages of the disease where there is already significant damage to the retinal architecture arising from degenerative, edematous and proliferative pathology. New preventive and interventional treatments to target early vasodegenerative and neurodegenerative stages of the disease are needed to ensure avoidance of sight-loss. Main body Historically, diabetic retinopathy has been considered a primarily microvascular disease of the retina and clinically it is classified based on the presence and severity of vascular lesions. It is now known that neurodegeneration plays a significant role during the pathogenesis. Loss of neurons has been documented at early stages in pre-clinical models as well as in individuals with diabetes and, in some, even prior to the onset of clinically overt diabetic retinopathy. Recent studies suggest that some patients have a primarily neurodegenerative phenotype. Retinal pigment epithelial cells and the choroid are also affected during the disease pathogenesis and these tissues may also need to be addressed by new regenerative treatments. Most stem cell research for diabetic retinopathy to date has focused on addressing vasculopathy. Pre-clinical and clinical studies aiming to restore damaged vasculature using vasoactive progenitors including mesenchymal stromal/stem cells, adipose stem cells, CD34+ cells, endothelial colony forming cells and induced pluripotent stem cell derived endothelial cells are discussed in this review. Stem cells that could replace dying neurons such as retinal progenitor cells, pluripotent stem cell derived photoreceptors and ganglion cells as well as Müller stem cells are also discussed. Finally, challenges of stem cell therapies relevant to diabetic retinopathy are considered. Conclusion Stem cell therapies hold great potential to replace dying cells during early and even late stages of diabetic retinopathy. However, due to the presence of different phenotypes, selecting the most suitable stem cell product for individual patients will be crucial for successful treatment.

Introduction: Dapsone has been widely used as a part of multidrug therapy for leprosy patients. Ocular side effects are rare. Ocular toxicity manifestations include retinal necrosis, optic atrophy, macular infarction, bilateral exudative retinal detachment, and choroidal detachment. We reported a rare case of dapsone-induced toxic maculopathy in a leprosy patient. Case Report: A 32-year-old male complained of blurred vision and a gray spot in central vision in the left eye (LE) for one month prior to admission. He had been treated with multidrug therapy (MDT) for leprosy for seven months. The MDT consists of dapsone, clofazimine, and rifampicin. The best-corrected visual acuity (BCVA) of the right eye (RE) and the LE were 6/6 and 6/12, respectively. A funduscopy of the LE showed decreased macular reflex. A color vision defect following the tritan axis was found in the LE. The Humphrey visual field (HVF) test of the LE revealed a central scotoma. Macular optical coherence tomography (OCT) showed intraretinal hyperreflectivity and subretinal fluid. Dapsone was then stopped in collaboration with a dermatologist. Two months after the discontinuation of Dapsone, the BCVA of the LE improved to 6/7.5, then 6/6 three months later. Color vision, macular OCT, and HVF tests revealed improvements. Multifocal ERG of both eyes (BE) also showed improvement in N1 and P1 wave amplitude in both eyes on 9-month follow-up after dapsone discontinuation. Discussion: Instead of direct drug toxicity, the mechanism of ocular side effects is thought to be ischemia caused by two distinct mechanisms. Macular ischemia is caused by acute, severe peripheral hypoxia and the physical effect of red cell fragmentation due to the hemolytic process. After discontinuation of dapsone, this case showed improvement in visual function and macular structure. Conclusion: Toxic maculopathy may be present in leprosy patients receiving dapsone treatment, although it is uncommon. Regular follow-up and evaluation of visual function and macular involvement are essential. Early detection of dapsone-induced toxic maculopathy and prompt discontinuation of dapsone may result in an improvement of visual functions.

Ayman G Elnahry,1 Alia M Noureldine,1 Ahmed A Abdel-Kader,1 Osama A Sorour,2 David J Ramsey3 1Department of Ophthalmology, Faculty of Medicine, Cairo University, Cairo, Egypt; 2Department of Ophthalmology, Faculty of Medicine, Tanta University, Tanta, Egypt; 3Division of Ophthalmology, Lahey Hospital & Medical Center, Tufts University School of Medicine, Boston, MA, USACorrespondence: Ayman G ElnahryDepartment of Ophthalmology, Faculty of Medicine, Cairo University, Cairo, 11956, Egypt, Tel +20 1224927604, Fax +20 223682030, Email ayman_elnahri@hotmail.comPurpose: To identify biomarkers that may predict an early anatomical response to the treatment of diabetic macular edema (DME) with intravitreal bevacizumab (IVB) by means of optical coherence tomography angiography (OCTA).Methods: This study is a retrospective study of treatment-naïve patients with DME who underwent 6 × 6 mm OCTA imaging of the macula at baseline and after three monthly IVB injections. Thirty-six eyes of 23 patients were included. Eyes that demonstrated evidence of an early anatomical response, consisting of a > 10% decrease in central macular thickness (CMT) (n = 18), were compared with those eyes that failed to show such an improvement (n = 18).Results: At baseline, early-response eyes had worse starting best-corrected visual acuity (BCVA, LogMAR 0.84 ± 0.41 versus LogMAR 0.51 ± 0.15, p = 0.004) and a larger CMT (490 ± 135 μm versus 356 ± 33 μm, p = 0.001), but smaller foveal avascular zones (FAZ) (0.309 ± 0.098mm versus 0.413 ± 0.095 mm, p = 0.003) compared with eyes that proved refractory to three monthly injections of IVB. The vascular density (VD) in both the foveal superficial and deep capillary plexuses was significantly greater in eyes that showed an early-treatment response compared with eyes that were non-responders (24.86 ± 6.90% versus 19.98 ± 7.13%, p = 0.045 and 32.30 ± 4.88% versus 26.95 ± 7.25%, p = 0.028, respectively). Early-treatment response to IVB was predicted by starting CMT (r2= 0.266, p = 0.001), FAZ size (r2= 0.234, p = 0.003), and VD in the superficial parafovea (r2= 0.217, p = 0.004) and deep fovea (r2= 0.157, p = 0.037).Conclusion: Projection-resolved OCTA may be useful in predicting an early anatomical response of DME to treatment with IVB.Keywords: anti-VEGF agents, bevacizumab, biomarkers, diabetic macular edema, macular ischemia, macular perfusion, optical coherence tomography angiography

Discussion The reperfusion of the occluded capillaries bed in the retina of patients with DR has been previously reported [[4]-[7]]. Keywords: Diabetic retinopathy; Macular ischemia; Reperfusion; Optic coherence tomography; Intraretinal microvascular abnormality EN Diabetic retinopathy Macular ischemia Reperfusion Optic coherence tomography Intraretinal microvascular abnormality 809 815 7 01/02/23 20220901 NES 220901 Introduction Diabetic retinopathy (DR) is the leading cause of blindness in the global working-age population [[1]]. Although there previously have been reports of spontaneous reperfusion of nonperfusion areas (NPAs) in DR patients [[4]], of which the process and morphological characteristics remain unclear due to the limitation of fundus fluorescein angiography (FFA). [Extracted from the article]

Purpose: To evaluate best-corrected visual acuity (BCVA), retina sensitivity (RS), and fixation impairment by microperimetry (MP) due to the presence and severity of disorganization of retinal inner and outer layers (DRIL/DROL) and ischemia in OCT/OCT angiography (OCTA) in diabetic retinopathy (DR)., Design: Retrospective case-control study., Subjects: Seventy-six eyes (65 patients) with DR were analyzed. Major exclusion criteria were: center-involving diabetic macular edema (DME), significant media opacity, nondiabetic macular pathology, and active proliferative DR. Patients with DRIL and DROL within central 3 mm were enrolled as cases. Patients with DR and no retina disorganization were considered as controls., Methods: A detailed grading of MP and OCT/OCTA images using Image J software, and specific Image Manipulation Program was applied to colocalize the presence of retina disorganization and RS. Best-corrected visual acuity and RS were correlated with the disorganization of retina layers' characteristics and grading (grade 1-DRIL; grade 2-DROL; grade 3-DROL plus, with involvement of the ellipsoid zone). The same procedure of colocalization was applied to the vascular layers on OCTA using MATLAB., Main Outcome Measures: Correlation between BCVA and MP parameters with disorganization of retina layers grading and OCTA parameters., Results: Best-corrected visual acuity, mean RS within 1 mm and central 3 mm (overall RS [oRS]), perfusion density, vessel density, and geometric perfusion deficit in intermediate and deep capillary plexuses were lower in cases versus controls (P < 0.001). Mean RS within 1 mm (21.4 decibels [dB] ± 2.4 vs. 13.8 dB ± 5.4, P = 0.002), oRS (22.0 dB ± 2.1 vs. 14.4 dB ± 4.6, P < 0.001), and BCVA (76.1 ± 7.4 vs. 61.2 ± 20.4 ETDRS letters; P = 0.02), had a significant decrease from grade 1 to grade 3 retina disorganization. Choriocapillaris flow voids (CC-FVs) increased from grade 1 to grade 3 (DROL plus) (P = 0.004). Overall retina sensitivity and CC-FV were identified as significant predictors of retina disorganization grade with an adjusted coefficient of determination, R 2  = 0.45. Cases had more dense scotomas (P = 0.03) than controls with a positive correlation between the worsening of fixation stability and the severity of DRIL/DROL (P = 0.04)., Conclusions: Microperimetry and BCVA documented a reduction in visual function in patients with DR and disorganization of retina layers at different grades, with greater functional impairment when outer retina layers and photoreceptors are involved. The severity of retina disorganization and the presence of ischemia could serve as a potential biomarker of functional impairment., Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2024 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

In recent years, retinal ischemia such as that which occurs in diabetic retinopathy (DR) and retinal vein occlusion (RVO) has become a hotspot of ischemic retinopathy research. High levels of vascular endothelial growth factor (VEGF) are recognized as a major cause of macular edema (ME) in DR and RVO. High concentrations of VEGF in the vitreous can lead to serious retinal ischemia and hypoxia and form retinal nonperfusion areas (NPAs). Different levels of retinal ischemia can represent disease severity and progression. Anti-VEGF therapy as the first-line treatment for ME has been found to be effective in improving vision, but there are still disputes about whether anti-VEGF therapy could improve retinal ischemia and achieve reperfusion of previously developed retinal NPAs. Here, we review and summarize studies of the effects of anti-VEGF drugs on retinal ischemia, especially NPAs.

Background: Diabetic retinopathy, a major complication of diabetes mellitus, is a leading cause of sigh-loss in working age adults. Progressive loss of integrity of the retinal neurovascular unit is a central element in the disease pathogenesis. Retinal ischemia and inflammatory processes drive interrelated pathologies such as blood retinal barrier disruption, fluid accumulation, gliosis, neuronal loss and/or aberrant neovascularisation. Current treatment options are somewhat limited to late-stages of the disease where there is already significant damage to the retinal architecture arising from degenerative, edematous and proliferative pathology. New preventive and interventional treatments to target early vasodegenerative and neurodegenerative stages of the disease are needed to ensure avoidance of sight-loss. Main body: Historically, diabetic retinopathy has been considered a primarily microvascular disease of the retina and clinically it is classified based on the presence and severity of vascular lesions. It is now known that neurodegeneration plays a significant role during the pathogenesis. Loss of neurons has been documented at early stages in pre-clinical models as well as in individuals with diabetes and, in some, even prior to the onset of clinically overt diabetic retinopathy. Recent studies suggest that some patients have a primarily neurodegenerative phenotype. Retinal pigment epithelial cells and the choroid are also affected during the disease pathogenesis and these tissues may also need to be addressed by new regenerative treatments. Most stem cell research for diabetic retinopathy to date has focused on addressing vasculopathy. Pre-clinical and clinical studies aiming to restore damaged vasculature using vasoactive progenitors including mesenchymal stromal/stem cells, adipose stem cells, CD34+ cells, endothelial colony forming cells and induced pluripotent stem cell derived endothelial cells are discussed in this review. Stem cells that could replace dying neurons such as retinal progenitor cells, pluripotent stem cell derived photoreceptors and ganglion cells as well as Müller stem cells are also discussed. Finally, challenges of stem cell therapies relevant to diabetic retinopathy are considered. Conclusion: Stem cell therapies hold great potential to replace dying cells during early and even late stages of diabetic retinopathy. However, due to the presence of different phenotypes, selecting the most suitable stem cell product for individual patients will be crucial for successful treatment. [ABSTRACT FROM AUTHOR]

Ocular manifestations of sickle cell trait have most frequently been reported in individuals with systemic medical comorbidities that predispose to erythrocyte sickling and vaso-occlusive disease. This case identifies a novel complication of sickle cell trait disorder, macular infarction, in a patient with comorbid associations. Purpose: Sickle cell trait affects 8% of African Americans. Once believed to represent a benign carrier state, it has been linked to an increased risk of several of the pathological conditions that arise in sickle cell disease in at-risk individuals with hematologic and vascular comorbidities. Macular infarction is a known complication of sickle cell disease; this article illustrates this unique presentation in a patient with sickle cell trait. Methods: Case report. Patient: A 74-year-old African American man presented with the complaint of a central scotoma of the right eye. Results: Multimodal retinal imaging identified central macular infarction with severe inner retinal atrophy. Laboratory testing confirmed the presence of sickle cell trait. Other pertinent positives included low levels of protein C and protein S, untreated obstructive sleep apnea, and elevated levels of homocysteine in the setting of alcoholic liver cirrhosis and chronic kidney disease. Conclusion: Ocular manifestations of sickle cell trait have most frequently been reported in individuals with systemic medical comorbidities that predispose to erythrocyte sickling and vaso-occlusive disease. This case identifies a novel complication of sickle cell trait disorder, macular infarction, in a patient with comorbid associations. [ABSTRACT FROM AUTHOR]

Supriya Dabir,1 Mohan Rajan,1 Liji Parasseril,1 Vaidehi Bhatt,2 Preetam Samant,3 CAB Webers,4 TTJM Berendschot4 1Department of Retina, Rajan Eye Care Pvt Ltd, Chennai, India; 2Rajiv Gandhi Medical College, Thane, India; 3Department of Retina, PD Hinduja Hospital and Medical Research Center, Mumbai, India; 4University Eye Clinic, Maastricht, the NetherlandsCorrespondence: Supriya DabirRajan Eye Care Hospital Pvt. Ltd, No. 5, Vidyodaya East 2nd Street, T. Nagar, Chennai, Tamil Nadu 600017, IndiaTel +919900974800Email supriad@gmail.comPurpose: Diabetic macular oedema (DME) is a vision-threatening complication of diabetic retinopathy. Spectral domain optical coherence tomography angiography (OCTA) is useful for assessing DME. We performed serial OCTA measurements before and after 3 monthly intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections to understand its relationship with best corrected visual acuity (BCVA), central macular thickness (CMT) and vascular indices.Methods: OCTA assessment consisting of 6 × 6 mm scans centred on fovea and en-face retinal angiograms were obtained. Scans were done at baseline and at 1 month following each of the 3 intravitreal injections of anti-VEGF (ranibizumab, Lucentis®).Results: Our prospective study included 24 eyes of 24 subjects, aged 63.0 ± 5.0 years. There was a mean gain of 0.07 LogMAR (3 letters) and reduction of CMT, vessel density (VD) and perfusion density (PD) by end of 3rd month. CMT was independently associated with improvement in BCVA. There were significant reductions in FAZ area, VD and PD over 3 months from baseline.Conclusion: At three months, intravitreal anti-VEGF therapy is associated with visual function recovery in DME, with reduction in CMT being the most reliable predictor of response. OCTA shows a reduction in VD and PD respectively suggestive of reduced macular perfusion in the short term. Simultaneous reduction in FAZ size can be due to reversal of manual displacement of the retinal capillaries resulting from resolution of intraretinal oedema.Keywords: diabetic macular oedema, superficial capillary plexus, vascular density, perfusion density, foveal avascular zone, optical coherence tomography angiography, macular ischemia

Abstract Background Varying degrees of macular ischemia generally occur in diabetic retinopathy (DR). This study aims to evaluate the effect of conbercept with 3+ pro re nata (PRN) on macular perfusion status in patients with diabetic macular edema (DME) and quantitatively assess changes in foveal avascular zone (FAZ) areas and capillary density in macular regions by applying optical coherence tomography angiography (OCTA). Methods Fifty patients were divided into ischemic (n = 31) and non-ischemic (n = 19) groups according to the presence of ischemia on OCTA at baseline. All patients received intravitreal injections of 0.5 mg of conbercept with 3+ PRN principle. The FAZ areas and macular vessel density measured using OCTA were evaluated at baseline, 3 months, and 6 months after treatment in both groups. Results At months 3 and 6, the FAZ area in the ischemic group changed from 0.510 ± 0.171 mm2 to 0.441 ± 0.158 mm2 then to 0.427 ± 0.153 mm2 (p = 0.003, p = 0.296); in the non-ischemic group, it remained stable (p = 0.269, p = 0.926). The superficial vessel density changed from 41.1 ± 4.1 to 42.5% ± 4.7% then to 42.6% ± 4.6% (p = 0.043, p = 0.812), and the deep vessel density changed from 40.7 ± 4.4 to 42.3% ± 3.6% then to 42.3% ± 4.7% (p = 0.072, p = 0.961) in the ischemic group. In the non-ischemic group, the superficial vessel density changed from 44.8 ± 3.2 to 46.0% ± 3.5% then to 45.7% ± 3.3% (p = 0.108, p = 0.666), whereas the deep vessel density changed from 43.6 ± 3.6 to 43.8% ± 3.2% then to 43.5% ± 4.5% (p = 0.882, p = 0.736). Reperfusion in macular nonperfusion areas was observed. Conclusion Anti–vascular endothelial growth factor treatment may have a positive effect on macular perfusion status. Furthermore, OCTA had advantages in quantifying and calculating blood flow index in the study of macular perfusion status.

Retinal vascular occlusion after ocular surgery is a rare but serious complication. A history of cardiovascular diseases, retrobulbar anesthesia injection, high intraocular pressure during the perioperative period, and drug toxicity have been reported as possible causative factors. We report here two cases of multiple retinal vascular occlusions after the subconjunctival injection of gentamicin at the end of uncomplicated 25-gauge vitrectomy. Case 1 was a 61-year-old man who developed a macular hole in the right eye. Phacovitrectomy with gas tamponade was performed. On postoperative day (POD) 1, dot hemorrhage was observed on the temporal side of the optic disk. On POD10, macular whitening, retinal hemorrhage, and multiple occlusion of retinal arteries and veins were observed. Case 2 was a 51-year-old woman who was diagnosed with rhegmatogenous retinal detachment in the right eye and underwent phacovitrectomy with gas tamponade. On POD3, macular whitening with cotton wool spots and retinal hemorrhage were observed with macular ischemia owing to occlusion of retinal arteries and veins. In both cases, subconjunctival injection of gentamicin given at the end of surgery was the most suspected cause of retinal vascular occlusion.

Abstract Background Diabetic macular ischemia (DMI) is an important category of diabetic retinopathy (DR) which leads to severe visual loss. Clinically, it is defined by an enlargement of the foveal avascular zone (FAZ) that can be detected by optical coherence tomography angiography (OCTA). Studies have described a relationship between renal disease and these changes in FAZ area. The aim of this study was to compare disturbances in FAZ area in diabetic patients with or without overt nephropathy. Methods Following approval of the ethics committee, we examined diabetic patients with retinopathy. Patients were divided into two groups of DR, namely, with overt nephropathy and without overt nephropathy. The FAZ area was measured using OCTA. A P value of

Purpose: To investigate the changes in the macular microvascular structure after anti-vascular endothelial growth factor (anti-VEGF) treatment in retinal vein occlusion (RVO) patients with and without macular ischemia.Methods: A total of 39 patients were divided into the macular ischemia group (n = 22) and the nonischemia group (n = 17) at baseline. All the patients received an intravitreal injection of ranibizumab with a 3+ pro re nata (PRN) regimen. The foveal avascular zone (FAZ) areas, macular vessel density (VD), and macular ischemic index (ISI) were evaluated at baseline and 3 and 6 months after treatment.Results: After treatment, some patients in the macular ischemia group achieved obvious reperfusion in macular nonperfusion areas. The VD and macular ISI improved in RVO patients, but the changes in VD and macular ISI were different in the two groups. The improvement of best corrected visual acuity (BCVA) was positively correlated with the improvement of macular perfusion status. Macular perfusion remained stable in most patients in RVO and only one patient had macular ischemia aggravation.Conclusion: The macular microvascular structures were stable in most RVO patients after anti-VEGF treatment. At the same time, some patients with macular ischemia presented reperfusion in macular nonperfusion areas, and still a few patients presented aggravated macular ischemia. Macular ISI is a good way to evaluate macular perfusion status in RVO compared to VD.

In this study, we present a case of bilateral optic neuropathy and macular ischemia in the right eye associated with neurosarcoidosis. A 26-year-old woman presented to our clinic with complaints of bilateral blurred vision. Bilateral granulomatous anterior uveitis, vitritis, optic neuropathy, and macular ischemia were detected in the right eye in slit-lamp examination. She also reported complaints of fever, weakness, sweating, arthralgia, and headache for 2 months. She was referred to the pulmonary diseases unit of our hospital due to hilar lymphadenopathy seen in her chest x-ray, and biopsies were taken for diagnostic purposes. Histological analysis of the mediastinal lymph node biopsies revealed chronic, non-caseating, granulomatous inflammation. Furthermore, the patient was referred to a neurologist due to concomitant complaint of intense headaches. She was diagnosed with neurosarcoidosis supported by findings on cranial magnetic resonance imaging and lumbar puncture. She received a 3-day course of high-dose (1 g/day) intravenous steroid treatment (methylprednisolone) followed by a tapering dose of oral prednisone. The patient began receiving oral methotrexate 15 mg/week as a steroid-sparing agent. Significant improvement in neurological and ophthalmological symptoms occurred in the first week of treatment. In this case report, we emphasized that neurosarcoidosis should be included in the differential diagnosis of patients with both bilateral optic neuropathy and macular ischemia. Furthermore, early diagnosis and timely treatment of neurosarcoidosis are important for favorable visual outcomes.

Optical coherence angiography (OCTA) is a noninvasive technique that has been introduced in recent years to detect ophthalmological pathology. The growing usage of OCTA to detect retinal abnormalities can be attributed to its advantages over the reference-standard fluorescein angiography (FA), although both of these techniques can be used in association. OCTA's advantages include its dye independency, its ability to produce depth-resolved images of retinal and choroidal vessels that yield images of different vascular layers of the retina, and the better delineation of the foveal avascular zone. OCTA's disadvantages include the lack of normalized patient data, artefactual projection issues, and its inability to detect low-flow lesions or pathologic conditions. Different OCTA platforms use unique algorithms to detect microvasculature, which are implemented in both spectral-domain (SD) and swept-source (SS) OCT machines. Microvascular changes in retinal vein occlusions (RVOs) are visible in both the superficial and deep capillary networks of the retina in OCTA. These visualizations include a decrease in foveal and parafoveal vascular densities, non-perfusion areas, capillary engorgement and telangiectasias, vascular tortuosity, microaneurysms, disruption of the foveal perivascular plexus, and formation of collateral vessels. The restricted field of view and inability to show leakage are important limitations associated with the use of OCTA in RVO cases. In this article, we present a brief overview of OCTA and a review of the changes detectable in different slabs by OCTA in RVO cases published in PubMed and Embase.

Background: Diabetic macular ischemia (DMI) is an important category of diabetic retinopathy (DR) which leads to severe visual loss. Clinically, it is defined by an enlargement of the foveal avascular zone (FAZ) that can be detected by optical coherence tomography angiography (OCTA). Studies have described a relationship between renal disease and these changes in FAZ area. The aim of this study was to compare disturbances in FAZ area in diabetic patients with or without overt nephropathy. Methods: Following approval of the ethics committee, we examined diabetic patients with retinopathy. Patients were divided into two groups of DR, namely, with overt nephropathy and without overt nephropathy. The FAZ area was measured using OCTA. A P value of < 0.05 was considered to be statistically significant. Result: A total of 46 patients (78 eyes) were enrolled in this study. All eyes with DR showed significant changes in FAZ area, but the sizes of the FAZ area were larger in both the superficial and deep layers in patients with clinical albuminuria than in those with no microalbuminuria (P = 0.007 and P = 0.002, respectively). Conclusion: These results demonstrate that OCTA provides highly detailed information on retinal microvasculature and that it is a reliable modality to assess DR progression in patients with nephropathy. They also show that renal impairment as a systemic risk factor was associated with enlarged FAZ area in DM. [ABSTRACT FROM AUTHOR]

Purpose: To report a case with ischemic macular edema (ME) due to an acute branch retinal vein occlusion (BRVO) which was treated with repeated intravitreal anti-VEGF injections. Methods: Retrospective case presentation. Results: A 66-year-old female patient was treated with repeated intravitreal anti-VEGF injections due to ischemic ME following an acute BRVO. Over a period of 2.5 years best corrected visual acuity increased from 0.06 to 0.6 (decimal notation) accompanied by a reduction in central retinal thickness from 546 to 292 µm. Overall 17 anti-VEGF injections were administered to treat repeated recurrence of ME. Macular ischemia did not worsen during this profound intravitreal anti-VEGF therapy. Conclusion: Intravitreal anti-VEGF therapy can be a beneficial treatment strategy even in ischemic ME following an acute BRVO.

Purpose: The aim of this study was to evaluate macular perfusion using OCTA automated software algorithms; vessel area density (VD) and non-flow tool to measure FAZ area in treatment-naïve diabetic eyes with moderate or severe NPDR and having macular edema, and correlate these parameters with LogMAR (logarithm of the minimum angle of resolution) visual acuity. Diabetic eyes without macular edema were included, to detect and define differences within the parameters between diabetic eyes with and without macular edema. Methods: Forty-five diabetic eyes with diabetic macular edema, forty diabetic eyes without macular edema, and forty eyes of healthy controls were examined using OCTA (RTVue-XR Avanti; Optovue, Inc, Fremont, CA). The macular vessel area density (VD) and foveal avascular zone (FAZ) area were assessed and statistically compared between the three groups and also correlated with the foveal thickness and visual acuity. Data were entered and analyzed by SPSS 19. Quantitative data were presented as mean and standard deviation, and qualitative data presented as frequency distribution; independent samples t test, Chi square test and Pearson correlation were done. Results: Mean whole image VD was 44.4 ± 3.6 in diabetic eyes with DME, 45.6 ± 4.2 in diabetics without DME, and 49 ± 3.9 in control eyes (P = 0.001). Diabetic eyes with DME had significantly lower vessels density values at the level of the deep retinal plexus (in the parafoveal, superior hemi, inferior hemi, temporal, superior, and nasal areas), when compared with diabetic eyes without DME. In diabetic eyes with DME, significant fair negative correlation was found between whole image vessels density at the level of the superficial retinal plexus and LogMAR VA (r = − 0.313, P = 0.036). Also, a significant fair positive correlation was found between FAZ area (at both the superficial and deep retinal plexus) and LogMAR visual acuity, in diabetic eyes with DME, where eyes with larger FAZ area had worse vision (P = 0.005 and P = 0.016, respectively). Diabetic eyes with DME had significantly larger FAZ area at the level of the superficial capillary plexus (mean superficial FAZ ± SD 0.55 ± 0.25) than diabetic eyes without edema (mean superficial FAZ ± SD 0.41 ± 0.12) and control subjects (mean superficial FAZ ± SD 0.35 ± 0.09). Conclusion: Using OCTA machine with AngioAnalytics parameters (vessel area density and non-flow area) helped in objective quantification of macular perfusion and accurately measuring the FAZ area in diabetic eyes with macular edema. Both parameters were significantly correlated with visual function in treatment-naïve diabetic eyes with edema. These OCTA biomarkers could be used to predict visual function in such eyes, to monitor response to treatment. [ABSTRACT FROM AUTHOR]

Bozho Todorich,1,2 Paul Hahn2 1Associated Retinal Consultants and Oakland University William Beaumont School of Medicine, Royal Oak, MI, 2Duke Eye Center, Duke University Medical Center, Durham, NC, USA Introduction: We aim to describe a mechanism of vision loss following vitrectomy surgery with retrobulbar block (RBB) associated with severe vascular stasis of the optic nerve and macula in order to improve safety of local anesthesia for ophthalmic surgery. Case presentation: We report three cases of patients who underwent pars plana vitrectomy (PPV) with retrobulbar anesthesia with no retrobulbar hemorrhage or elevated intraocular pressure (IOP). At the beginning, in each case, hypoperfusion of optic nerve and macula was noted. In the case of one patient with significant vasculopathic risk factors, the vascular stasis was severe, while in the other two cases, it was mild-to-moderate. In all cases, the perfusion of posterior pole began to improve almost immediately following the start of PPV. Because the IOP was not elevated and no retrobulbar hemorrhage was present, this suggested a compartment syndrome in the intraconal space. The patient with severe vascular stasis developed finger-counting vision but had normal postoperative angiogram findings and unrevealing cardiovascular workup. In the other two milder cases, the occurrence of ischemia was not visually significant. Conclusion: Intraoperative ischemia should be considered in all cases of unexplained vision loss after ophthalmic surgery using RBB. Attention to vasculopathic risk factors and intraoperative hemodynamic parameters, in addition to the use of parabulbar block, may avoid this complication and permanent vision loss. Keywords: macular ischemia, pars plana vitrectomy, retrobulbar block, central retinal artery occlusion

Aim The aim of this study was to correlate the visual field changes in the central macular area with the areas of capillary nonperfusion seen in fluorescein angiogram in patients with nonproliferative diabetic retinopathy (NPDR). Patients and methods This study included 40 eyes of 32 patients with NPDR attending the Ophthalmic Outpatient Clinic of El-Minia University Hospital during the period from January 2012 to July 2013. All patients were subjected to automated perimetry using a Topcon perimeter. A full-threshold strategy was applied for the central 10° field (program 10–2) fluorescein angiography using the IMAGE Net 2000 fundus camera. Results This study included 40 eyes of 32 patients between 50 and 70 years of age with a mean of 59.3 ± 7.6 years. Of them, there were 12 (37.5%) male and 20 (62.5%) female patients. All patients were noninsulin-dependent diabetic patients with a mean duration of 16.6 ± 5.4 years; 20 (62.5%) patients were hypertensive and 12 (37.5%) were normotensive. Twenty eyes had a rate of 0 dB corresponding to areas of capillary nonperfusion. Sixteen eyes showed a rate of 0 dB less than the areas of capillary nonperfusion. Four eyes showed relatively good retinal sensitivity (rate of 0 dB = 0) despite the presence of definite areas of capillary nonperfusion. The mean sensitivity in these areas ranged between 10.38 ± 1.47 and 12.91 ± 1.43 dB. Conclusion There is a significant correspondence between macular capillary nonperfusion and central field sensitivity in patients with NPDR.

In this study, we present a case of bilateral optic neuropathy and macular ischemia in the right eye associated with neurosarcoidosis. A 26-year-old woman presented to our clinic with complaints of bilateral blurred vision. Bilateral granulomatous anterior uveitis, vitritis, optic neuropathy, and macular ischemia were detected in the right eye in slit-lamp examination. She also reported complaints of fever, weakness, sweating, arthralgia, and headache for 2 months. She was referred to the pulmonary diseases unit of our hospital due to hilar lymphadenopathy seen in her chest x-ray, and biopsies were taken for diagnostic purposes. Histological analysis of the mediastinal lymph node biopsies revealed chronic, non-caseating, granulomatous inflammation. Furthermore, the patient was referred to a neurologist due to concomitant complaint of intense headaches. She was diagnosed with neurosarcoidosis supported by findings on cranial magnetic resonance imaging and lumbar puncture. She received a 3-day course of high-dose (1 g/day) intravenous steroid treatment (methylprednisolone) followed by a tapering dose of oral prednisone. The patient began receiving oral methotrexate 15 mg/week as a steroid-sparing agent. Significant improvement in neurological and ophthalmological symptoms occurred in the first week of treatment. In this case report, we emphasized that neurosarcoidosis should be included in the differential diagnosis of patients with both bilateral optic neuropathy and macular ischemia. Furthermore, early diagnosis and timely treatment of neurosarcoidosis are important for favorable visual outcomes. [ABSTRACT FROM AUTHOR]

Objectives: Is to detect the vascular morphological changes in the two intra retinal; layers superficial network layer and deep network layer by optical coherence tomography angiography (OCT-A) in cases with acute onset branch retinal vein occlusion (BRVO). Study Design: An observational cross-sectional study in new Kafer El-Sheikh Ophthalmology Hospital. Population: This study included ten patients with (BRVO) of recent onset within the last three months. Methods: Demographic data such as age, gender, sex and hypertensive history were obtained. A detailed evaluation of cases including complete anterior segment evaluation and posterior segment evaluation was done. OCT-A and fluorescein angiography (FA) were used to study the vascular morphological changes in the (BRVO) area. Results: As regarding 5 disc area of retinal ischemia FA detected ischemia in 2 cases (20%), detected no ischemia in one case (10%) and was not able to evaluate ischemia in 7 cases (70%) compared to OCT-A, that detected the ischemia in the superficial capillary plexus (SCP)in 4 cases (40%), and detected no ischemia in 6 cases (60%), but it detected the ischemia in the deep capillary plexus (DCP) in 6 cases (60%) and detected no ischemia in 4 cases (40%). Conclusion: Our study demonstrated that OCT-A was superior to FA in detection of macular ischemia in cases with acute onset branch retinal vein occlusion (BRVO), macular ischemia detected in the DCP by OCTA had stronger negative relation with V.A. than that of SCP. By OCT-A macular edema (ME) was present mainly in the DCP compared to SCP. [ABSTRACT FROM AUTHOR]

Introduction: Diabetic retinopathy (DR) is a common complication of diabetes and a leading cause of blindness in working age population. Fluorescein angiography is still the gold standard in the evaluation of retinal vascular perfusion and diagnosis of macular ischemia. However, it is a costly, time-consuming procedure and it requires intravenous injection of contrast agent, exposing patients to potential side effects. Optical coherence tomography angiography (OCTA) is a novel, non-invasive imaging technique that provides dyeless visualization of blood flow in different retinal layers. Areas covered: An extensive review of the literature was performed to detail technical principles of OCTA and to discuss the current concepts on its application in diabetic patients. Expert commentary: In patients with DR, OCTA shows early features in unprecedented detail: enlargement of the FAZ, areas of capillary non-perfusion, and some microvascular abnormalities can be seen with much better clarity than with fluorescein angiography. OCTA is also able to detect several features that are currently accepted as prognostic indicators in patients with DME. The most relevant are the presence of diabetic macular ischemia and pronounced microvascular abnormalities. It has been shown that these alterations may influence the response to anti-VEGF therapy. [ABSTRACT FROM PUBLISHER]

Snakebites are endemic in some parts of Thailand, being associated with several complications. Ocular disturbances are uncommon, except in cases of corneal or conjunctival injury, when the eye is directly exposed to the snake venom. The present study presents a case of combined ophthalmic artery occlusion and transient central retinal artery occlusion with macular ischemia after a Russell's viper bite.

Aim The aim of this study was to correlate the visual field changes in the central macular area with the areas of capillary nonperfusion seen in fluorescein angiogram in patients with nonproliferative diabetic retinopathy (NPDR). Patients and methods This study included 40 eyes of 32 patients with NPDR attending the Ophthalmic Outpatient Clinic of El-Minia University Hospital during the period from January 2012 to July 2013. All patients were subjected to automated perimetry using a Topcon perimeter. A full-threshold strategy was applied for the central 10° field (program 10-2), and fluorescein angiography: using the IMAGE Net 2000TM fundus camera. Results This study included 40 eyes of 32 patients between 50 and 70 years of age with a mean of 59.3±7.6 years. Of them, there were 12 (37.5%) male and 20 (62.5%) female patients. All patients were noninsulin-dependent diabetic patients with a mean duration of 16.6±5.4 years; 20 (62.5%) patients were hypertensive and 12 (37.5%) were normotensive. Twenty eyes had a rate of 0dB corresponding to areas of capillary nonperfusion. Sixteen eyes showed a rate of 0dB less than the areas of capillary nonperfusion. Four eyes showed relatively good retinal sensitivity (rate of 0 dB=0) despite the presence of definite areas of capillary nonperfusion. The mean sensitivity in these areas ranged between 10.38±1.47 and 12.91±1.43dB. Conclusion There is a significant correspondence between macular capillary nonperfusion and central field sensitivity in patients with NPDR. [ABSTRACT FROM AUTHOR]

In the original Phase II/III trial, however, patients with macular ischemia were excluded. In this study, we treated patients with ischemic DME. Methods: Macular ischemia was defined as a 30% increase in the area of the foveal avascular zone (FAZ) at 45 seconds on fundus fluorescein angiography. In addition, the participants had diffuse foveal-involving DME with a central subfield thickness (CST) of >300 μm on spectral-domain optical coherence tomography. Five intravitreal pegaptanib injections were given 6 weeks apart. The final study visit was 6 weeks after the fifth injection. The primary outcome was change in the size of FAZ. Secondary outcomes were change in best-corrected visual acuity (BCVA) and the change in CST. Results: Thirty participants were enrolled. Three were unable to complete the full course of treatment. Their outcomes were carried forward for the first part of this analysis. There was no statistically significant change in the mean size of the FAZ from baseline to the final visit. Subclassifying participants as those with minimal/moderate ischemia (16 participants, FAZ area <1,000 pixels) and those with more severe ischemia (14 participants, FAZ area >1,000 pixels) also showed no statistically significant change in the mean area of the FAZ. On average, BCVA increased and CST decreased from baseline to the final visit, but these changes were not statistically significant. Using per protocol analysis on those participants who completed the full course of treatment, the mean BCVA increased from 49.2 to 53.9 letters (P=0.046). Conclusion: In this study, intravitreal injection of pegaptanib did not significantly alter the size of the FAZ in participants with varying degrees of ischemic DME. There was, however, a significant improvement in mean BCVA in those who completed the treatment course. [ABSTRACT FROM AUTHOR]