Twelve-year follow up of a case of autosomal recessive bestrophinopathy with transient resolution of retinal edema in one eye

Purpose To report 12-year follow-up of a patient with ARB. Case report A 25-year-old man presented with blurred vision in his both eyes (OU). Best-corrected visual acuity (BCVA) was 20/63 Snellen equivalent in the right eye (OD) and 20/32 Snellen equivalent in the left eye. The intraocular pressures and anterior segment examination were unrevealing in OU. Posterior segment examination revealed multiple yellowish flecks and dots in the posterior pole in OU. Optical coherence tomography (OCT) showed subretinal fluid (SRF), intraretinal hyporeflective spaces, elongated and shaggy photoreceptors and outer retinal defects. Fundus autofluorescence demonstrated mottling hyperautofluorescence and hypoautofluorescence in the posterior pole in OU. Fluorescein angiography illustrated hyperfluorescence in the posterior pole and surrounding the arcades in OU. Multifocal electroretinography objectified mild to markedly abnormal responses in all ring areas in OU. Molecular genetic testing confirmed two heterozygous sequence variations in the BEST1 gene. At 4 years of follow-up, OCT revealed a complete resolution of SRF and a partial resolution of intraretinal hyporeflective spaces in the OD with corresponding improvement in the BCVA to 20/23 Snellen equivalent in the OD, even though outer retinal defects persisted. Our patient denied recent changes in his alimentary habits and medical history at that time. Posteriorly, SRF and intraretinal hyporeflective spaces reappeared in the OD. Conclusion To the best of our knowledge, this is the first case report of ARB with a transient resolution of retinal edema in one eye without medical treatment and dietary therapy.

Topographic patterns of retinal edema in eyes with branch retinal vein occlusion and their association with macular edema recurrence

In this study, we evaluated the topographic pattern of retinal edema in eyes with macular edema (ME) secondary to branch retinal vein occlusion (BRVO) using a widefield retinal thickness map of optical coherence tomography and its association with ME recurrence. In 87 eyes with ME secondary to BRVO who were treated with anti-vascular endothelial growth factor (VEGF) injections and followed up for ≥ 1 years, 12 × 9 mm macular volume scans of swept-source optical coherence tomography (DRI-OCT Triton; Topcon Inc, Japan) were performed and retinal thickness maps were automatically generated at baseline and follow-up visits. Topographic patterns of retinal edema on the maps at baseline and 1 month after the first anti-vascular endothelial growth factor (VEGF) treatment were classified as extramacular (outside the ETDRS grid), macular (within the grid), and combined pattern and correlated with ME recurrences. Seventy-five of 87 (86.2%) eyes with BRVO ME showed combined edema at baseline. There were 4 topographic patterns of edema at 1 month following anti-VEGF injection as follows: no residual edema, extramacular only, macular only, and combined edema. In contrast to the baseline pattern, the pattern of retinal edema 1 month following anti-VEGF therapy showed significant association with 6-month recurrence, number of ME recurrences during a 1-year period, and time to first recurrence. (all P < 0.05) An automatically generated widefield retinal thickness map could be used to effectively visualize the topographic patterns of retinal edema in eyes with BRVO. The map can be used as a valuable tool for detection of retinal edema on widefield retinal areas and prediction of ME recurrence in eyes with BRVO.

Glucagon-Like Peptide-1 Receptor Agonist, Liraglutide, Attenuated Diabetic Retinal Edema in Spontaneously Diabetic Torii Fatty Rats

Background: This study aims to investigate the effect of the glucagon-like peptide-1 (GLP-1) receptor agonist (GLP-1RA) liraglutide on retinal edema as compared with insulin and hydralazine using an animal model of type 2 diabetes with obesity, hypertension, and hyperlipidemia.Methods: Male spontaneously diabetic Torii (SDT) fatty rats at 8 weeks of age were randomly assigned to three groups: the liraglutide group (SDT-lira, n = 6) received a subcutaneous injection of liraglutide from the age of 8 to 16 weeks, the SDT-ins-hyd group (n = 6) was provided both insulin against hyperglycemia and hydralazine against hypertension to match levels of both blood glucose and blood pressure to those of the liraglutide group, and the control group of SDT fatty rats (SDT-vehicle, n = 7) and a nondiabetic control group of Sprague–Dawley rats (SD, n = 7) were injected with vehicle only. Both eyeballs of all groups were collected at the age of 16 weeks.Results: Retinal thickness, which was found in the SDT-vehicle group, was significantly prevented to similar levels in both the SDT-lira and SDT-ins-hyd groups. Immunohistological analysis revealed that GLP-1 receptor was not expressed in the retina of all rats. The ocular protein expression of monocyte chemoattractant protein-1, which causes a proinflammatory situation, was significantly upregulated in all SDT fatty rats as compared to SD rats, but the expression levels were similar between all SDT fatty rats. With regard to neovascularization in the eyes, there were no significant differences in protein expressions of vascular endothelial growth factor, CD31, or endothelial nitric oxide synthase in all rats.Conclusions: The present study indicates that liraglutide prevents retinal thickening, dependent on blood glucose and blood pressure levels in SDT fatty rats without ocular neovascularization. However, the effects did not improve the ocular proinflammatory state.

Retinal morphometric parameters of epiretinal membrane in the early period of surgical treatment

The analysis of the morphometric of the retina in patients with epiretinalmembrane was carried out using spectral coherent optical tomography and determination of retinal photosensitivity in the early postoperative period. A decrease in retinal edema was revealed due to a decrease in the thickness of the inner layers of the neuroepithelium in patients with vitreoretinal pathology after surgical treatment. Keywords: epiretinal membrane, morphometric parameters of the retina, vitrectomy, membranopilling.

Multimodal Imaging in Idiopathic Neuroretinitis with Localized Choroidal Insufficiency: A Case Report

Neuroretinitis is a rare clinical entity, characterized by optic nerve edema and star-shape hard exudate around fovea. The clinical features include acute unilateral visual loss, dyschromatopsia, relative afferent pupillary defect and visual field abnormalities. Increased vascular permeability of the optic disc is the main pathophysiology. As it is a not fully known clinical entity, diagnosis is challenging. In this case, we use multimodal imaging to reveal pathophysiology and anatomical change of early mild neuroretinitis. Case presentation: A 28-year-old healthy woman presented to the clinic with mild blurred vision in her left eye. After complete ophthalmic examination, outer retinal thickening of the temporal peripapillary area and optic disc edema were observed. Two days after diagnosis, the retinal edema and visual symptoms were aggravated. A hard exudate, maybe a part of macular star, was observed. Multimodal imaging including optical coherence tomography (OCT), swept-source OCT angiography (SS-OCTA), fluorescein angiography, and indocyanine green angiography visualized choroidal thinning and insufficient circulation beneath the outer retinal edema. Following steroid pulse therapy, the retinal edema and blurred vision were completely resolved. Conclusions: Multimodal imaging suggested that unilateral optic disc edema and early macular star help the diagnosis of neuroretinitis. In SS-OCTA, we found focal choroidal insufficiency. The focal insufficient choroidal circulation might be a contribution factor for idiopathic neuroretinitis. Multimodal imaging including SS-OCTA may be a valuable tool for detecting and monitoring disease progression.

Optical coherence tomography in patients with somatotropin-producing pituitary adenoma

Optical coherence tomography (OCT) is widely used in ophthalmological practice. The review presents the results of OCT in patients with somatotropin-producing pituitary adenoma, or somatotropinoma, which is a hormone-active tumor of the adenohypophysis, characterized by excessive production of somatotropic hormone (STH). It stimulates the secretion of type I insulin-like growth factor (IGF-I). The mechanisms of STH action (pro-angiogenic action, stimulation endothelial cell proliferation and migration, development of endothelial dysfunction and retinal edema) requires much attention to the results of examination of patients with somatotropinomas using modern diagnostic methods, such as OCT.

Large-cube 30° × 25° optical coherence tomography in diabetic macular edema

Background To evaluate the contribution of large-cube 30° × 25° optical coherence tomography (OCT) in the characterization of diabetic macular edema (DME) by assessing its extent and the presence of additional retinal edemas and to evaluate the factors that influenced their occurrence. Methods This retrospective study enrolled patients with diabetes who presented with retinal edema detected by horizontal large-cube 30° × 25° (8.7 × 7.3 mm) OCT. Two individualized areas were selected from the thickness map: the area within the 6-mm Early Treatment of Diabetic Retinopathy Study (ETDRS) grid, and that outside the ETDRS grid. Retinal edemas located within the ETDRS grid were designated as “main DME” and those located outside the ETDRS grid were designated as “peripheral retinal edemas.” For each area, OCT features were assessed while the extent of the main DME and the presence of peripheral retinal oedema were analysed in the area outside the ETDRS grid. Finally, part of included eyes was followed by the same protocol, of which a part benefited from intravitreal injections. Results Peripheral events were detected outside the ETDRS area in 279 eyes (74.4%) of the 375 eyes of the 218 patients included in this study: an extension of the main DME outside ETDRS grid in 177 eyes (47.2%) and/or the presence of peripheral retinal edemas in 207 eyes (55.2%). The analysis of associations between main DME and peripheral retinal edemas patterns did not find an association for retinal cyst localization (P = 0.42) while a week association was found fort cyst size (Cramer’s V = 0.188, p = 0.028). Nevertheless, a moderate association was found for the presence of microaneurysms (Cramer’s V = 0.247, p < 0.001) and strong association for hard exudates (Cramer’s V = 0.386, p < 0.001), The binary logistic regression analysis retained the following influencing factors of the occurrence of peripheral events: advanced DR stage (Odds ratio OR = 2.19, p = 0.03), diffuse DME (OR = 7.76, p < 0.001) and its location in outer fields (OR = 7.09, p = 0.006). Likewise, the extension of the main DME outside the ETDRS area in was influenced by the same factors in addition to CMT (OR = 0.98, p = 0.004) while the presence of peripheral retinal edema was influenced by the same factors except the outer location of the Main DME. Finally, from the 94 eyes treated by intravitreal injections, extension of the main DME outside the ETDRS grid was detected in 54 eyes (56.44%) at baseline visit and still remained detectable in 37 eyes (39.36%) after treatment initiation. Conclusions Large-cube 30° × 25° OCT allowed for more precise assessment of DME extension and better detection of retinal thickening mainly in the advanced stages of diabetic retinopathy with significant DME whether at the baseline visit or during follow-up. The combination of this protocol with a wider ETDRS grid would enhance DME detection and topography.

The Efficacy of Melanin Precursor QIAPI 1© against Age-Related Macular Degeneration (AMD): Case Report

Background: AMD is becoming one of the leading causes of blindness in older adults. Prevalence rate of wet form of AMD has been increasing due to lack of the selective therapeutic modalities. Current therapeutic interventions such as drugs targeting VEGF, and VEGF receptors, laser coagulants have unsuccessful clinical outcomes in AMD patients. Hence, the cost-effective anti-oxidant therapeutic interventions like molecular hydrogen to protect retinal pigment epithelium (RPE) by mitigating oxidative stress may deliver effective clinical outcome in AMD patients. Methods: Female patients with late-stage of AMD of aged above 70-years were chosen for this case report. The patients were administered QIAPI1©, a melanin precursor via sublingual route and the photographs were obtained for left and right eye to depict the efficacy of QIAPI1© against wet form of AMD. Results: The administration of QIAPI1© extensively mitigated yellow colored drusen accumulations in retina, retinal edema, exudates, and hemorrhages in right eye but the effect was minimal in case of left eye but the overall drusen accumulation was lesser than the first consultation. Conclusion: Current case report has concluded the intrinsic effect of melanin in producing the molecular hydrogen and chemical energy across the retinal tissues by dissociating water molecules and dissipates the drusen accumulations, retinal edema, and hemorrhages in AMD patients. Our preliminary study reported the usage of QIAPI1© as a prospective therapeutic modality to mitigate the oxidative stress-mediated pathophysiology of wet form of AMD.