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Dragged-fovea diplopia syndrome (DFDS) is a type of binocular double vision caused by a displacement of the fovea in one or both eyes due to retinal disorders including epiretinal membranes or other maculopathies. DFDS induces diplopia through a mismatch between peripheral motor fusion and central (foveal) fusion. It can be diagnosed by utilizing the Lights on - Lights off test. While there is no cure, there are treatments for DFDS including monocular occlusion or blurring (tape, lenses, IOL), Bangerter filter, and Fresnel prisms. While this syndrome has been identified in the literature by multiple names including central-peripheral Rivalry (CPR)-type diplopia, macular diplopia, and foveal displacement syndrome, this article works to summarize the current known characteristics, diagnostic tests, and treatment for this syndrome.

Background: Whereas crystals deposit in the retina, the cornea and limbus in Bietty corneo-retinal dystrophy (BCD) is now well established and documented, only two published cases report their findings in the lens and no cases deep in the lens cortex., Material and Methods: Four consecutive adult patients from three different unrelated families presenting lens crystals associated with advanced genetically confirmed BCD were enrolled with advanced disease and long follow up (>12 years). Demographics, visual acuity, slit lamp biomicroscopy, lens and posterior pole photography, optical coherence tomography (OCT), autofluorescence, and screening for CYP4V2 type of mutation were performed. The setting was Jules Gonin Eye Hospital, Switzerland, between 1.1 2013 and 1.11. 2019., Results: All patients were European women. The ages ranged from 40 to 81 years. Best Snellen visual acuity ranged from light perception to 1.0. All patients presented with limbus and retinal crystals deposit that disappeared over time and the development of severe chorioretinal atrophy. With long-term follow up, multiple crystal-like deposits appeared in the anterior, posterior lens capsule and cortex. All patients, but one, had homozygous or compound heterozygous mutations in CYP4V2 gene., Conclusions: To the best of our knowledge, there are no published cases of crystal deposits in the cortex of the lens of patients diagnosed with BCD associated with CYP4V2 gene mutation. This could be a feature of advanced BCD, and their presence in the lens cortex questions the hypothesis of floating deposits from posterior pole although their exact etiology remains to be determined.

Purpose : To analyze the abnormalities of foveal microvasculature in patients with systemic lupus erythematosus (SLE). Methods : A cross-sectional study of 41 eyes of patients diagnosed with SLE and 40 eyes of healthy controls. Sections measuring 6 mm×6 mm centered on the fovea were obtained using optical coherence tomography angiography (OCTA). Results : The area and perimeter of foveal avascular zone (FAZ) in SLE patients were greater than those in controls (both p < .05). The parafoveal vessel densities in the deep capillary plexus (DCP) of SLE patients were significantly decreased than those in controls ( p < .001). There was a negative correlation between SLE disease activity index and parafoveal vessel density in DCP ( p < .05). Conclusion : Enlargement of the FAZ and decline of the parafoveal vessel density of DCP were revealed by OCTA in patients with SLE. Moreover, a negative correlation between parafoveal vessel density in DCP and disease activity index was detected in SLE patients.

Purpose : To analyze quantitative OCT-angiography (OCTA) measurements of Vogt-Koyanagi-Harada (VKH) disease at different disease stages. Methods : OCTA scans of 110 VKH patients in acute uveitic, convalescent and chronic recurrent stage were reviewed; posterior uveitis recurrent cases were excluded. Superficial and deep capillary plexus (SCP and DCP) vascular densities (VD) and subfoveal choroidal thickness (SFCT) were compared. Results : DCP VD was significantly lower and SFCT higher in VKH than normal controls (NC) in all 3 stages (all p < .001). Anterior uveitis recurrent patients had lower SCP and DCP VDs and thicker SFCT than convalescent patients ( p = .001, p < .001, and p = .003, respectively). DCP VD was significantly correlated with visual acuity ( p < .05). Patients with history of anterior recurrence had lower retinal VDs in the convalescent stage than those without ( p < .001). Conclusion : Retinal microcirculation is impaired in VKH. DCP VD might be a sensitive indicator of inflammatory status and appeared to be well associated with visual outcome.

Purpose : To investigate the prevalence and clinical characteristics of myelinated retinal nerve fibre (MRNF) in a large teleophthalmology system. Methods : All records between January 2015 and December 2015 from Daheng Prust teleophthalmology system were reviewed by 2 ophthalmologists independently. MRNF was classified into continuous group and discontinuous group according to the relationship between MRNF patches and optic disc. The number, total area and location of MRNF patches were analysed. Concomitant ocular diseases were documented. Results : Out of 51469 subjects, MRNF was detected in 304 eyes of 263 subjects with a prevalence rate of 0.51 ± 7.1% per subject and 0.30 ± 5.4% per eye. Among 304 eyes with MRNF, 239 (78.6%) eyes were in continuous group and 65 (21.4%) eyes were in discontinuous group. Single MRNF patch was found in 249 (81.9%) eyes and multiple MRNF patches were found in 55 (18.1%) eyes. MRNF of small size was found in 150 (49.3%) eyes. The ratios of multiple MRNF patches and small-sized MRNF in the continuous group were significantly higher than those in the discontinuous group ( P = .014 and P < .001). In continuous group, the MRNF patches were located most frequently in the superior region (68.6%) of the optic disc; In discontinuous group, the MRNF patches were located most frequently in the inferotemporal region (38.5%) of the retina. Epiretinal membrane (12 eyes, 3.9%) was the most common concomitant ocular disease. Conclusion : MRNF is uncommon in China. MRNF usually presents unilaterally and as a single small whitish patch that is connected with optic disc.

Purpose : To report a case of ocular dirofilariasis causing extensive chorio-retinal damage. Method : Surgical removal of the worm and identification of the same by microscopic examinationand polymerase chain reaction. Results : The worm was identified as Dirofilaria repens . Conclusion : Ocular dirofilariasis is an arthropod borne parasitic infestation affecting the subconjunctival tissue and anterior chamber. Vitreous involvement in Dirofilaria is very rare. Dirofilaria repens can cause extensive chorio-retinal damage resembling diffuse unilateralsubacute neuroretinitis.

Purpose : To evaluate longitudinal changes in retinal layer thickness and clinical outcome in patients with MEWDS. Methods : In 20 patients with MEWDS, SD-OCT images and BCVA were assessed at baseline, and at months 1, 3, and 12. SD-OCTs were segmented and measurements were performed within the fovea and a MEWDS lesion. Baseline and follow-up values in the affected eye were compared to measurements performed at the corresponding location in the fellow eye. Results : ONL thickness was 4.7% thicker in MEWDS-eyes compared with the baseline, with a significant decrease of 9% at 3 months. Within the lesion, INL thickness was 7.9% increased at baseline and decreased significantly over the follow-up of 12 months. BCVA was decreased at baseline (0.2 ± 0.18logMAR) and at the 3 months but after 12 months had increased to 0.01 ± 0.04 logMAR. Conclusion : MEWDS shows the involvement of different retinal layers and characteristic changes over the disease course.

Purpose: The mitochondrial DNA A3243G (m.3243A>G) variant causes a wide spectrum of phenotypes, with pigmentary retinopathy as the most common ocular finding. We undertook this meta-analysis to investigate the clinical course of visual acuity (VA) in patients with m.3243A>G variant and provide key clinical correlations with systemic manifestations., Methods: A PubMed literature search was performed and studies were selected after satisfying pre-set inclusion criteria. Demographic and clinical data, including retinal findings and systemic manifestations were recorded. Cross-sectional and linear regression analyses were used to investigate the relationship between VA and age, as well as between the age at diagnosis of retinopathy and the mean ages at diagnosis of sensorineural hearing loss or diabetes. The age and prevalence of systemic manifestations among patients with and without retinopathy were studied using t- tests and Mann-Whitney U-tests (performed on binarized data). Likelihood ratios were computed., Results: The mean VA (average of both eyes) of 90 patients (72.2% female; 65/90) were collected from 18 studies published between 1990 and 2018. The baseline mean age was 45.2 years (range 17 to 92). The mean logMAR VA was 0.10 (- 0.12 to 1.39). There was a statistically significant linear correlation between the logMAR VA and age ( p =  .008). The VA of patients less than or equal to 50 years of age was significantly better than that of patients older than 50 years (0.06 vs.0.18 logMAR, p = .002). 67 patients (74.4%) showed a characteristic pigmentary retinopathy with a mean age at diagnosis of 47.9 years (17 to 92) and VA of 0.14 logMAR (- 0.12 to 1.24). Age at diagnosis of retinopathy was linearly correlated with age at diagnosis of hearing loss or diabetes ( p <  .001). Patients with retinopathy were more likely to have hearing loss (83.6% vs. 56.5%, p =  .03) or diabetes (56.7% vs. 17.4%, p =  .001) than those without retinopathy. Those with both hearing loss and diabetes had an earlier onset of retinopathy than those without (46.4 vs. 60.4 years, p = .01). Patients without both hearing loss and diabetes were 5.3-fold less likely to develop a retinopathy., Conclusions: Patients with m.3243A>G variant pigmentary retinopathy maintain highly functional VA until around the fifth decade of life, after which significant visual decline ensues. Patients without hearing loss and diabetes have a lower likelihood of exhibiting a retinopathy, which tends to appear about one decade after hearing loss and diabetes are diagnosed.

Purpose: This study aimed to evaluate the retinal blood vessel parameters and the foveal avascular zone (FAZ) area using optical coherence tomography angiography (OCTA) in patients with acromegaly in comparison with healthy controls., Methods: A total of 45 patients with acromegaly and 45 healthy controls were included in this study. In all patients, the vessel density (VD) of the deep and superficial macular vascular networks and the foveal avascular zone (FAZ) were measured using OCTA. The correlation of insulin-like growth factor 1 (IGF-1) level and disease duration with deep macular VD and FAZ values was analyzed. All parameters were registered. Results were assessed and compared between the two groups., Results: Deep macular plexus VD values were lower in patients with acromegaly compared to the control group. No significant differences in VD values in the superficial segment, except for that in the inferior parafovea ( P = .01) were found between the two groups. Furthermore, patients with acromegaly showed enlargement in the superficial ( P = .30) and deep FAZ areas ( P < .001). IGF-1 level and disease duration showed a significant negative correlation with the deep whole image ( R = -0.216, P = .041, R = -0.339, P = .001, respectively), deep parafovea ( R = -0.271, P = .01; R = -0.372, P < .001, respectively), deep parafovea superior hemi ( R = -0.342, P = .001; R = -0.350, P = .001, respectively), deep parafovea temporal ( R = -0.224, P = .034; R = -0.234, P = .026, respectively), deep parafovea nasal ( R = -0.320, P = .002; R = -0.361, P < .001, respectively), and deep parafovea superior VD values ( R = -0.293, P = .005; R = -0.307, P = .003, respectively) and a significant positive correlation with the deep FAZ area values ( R = 0.244, P = .02; R = 0.329, P = .002, respectively)., Conclusion: VD values in the deep macular capillary plexus were lower in patients with acromegaly, and the superficial and deep FAZ area enlarged. Patients with acromegaly may have an increased risk of developing ocular vascular complications. OCTA can be used to evaluate retinal blood VD in patients with acromegaly.

Purpose : To assess the characteristics of parafoveal microvascular abnormalities in Behcet's uveitis (BDU) using projection-resolved optical coherence tomographic angiography (PR-OCTA). Methods : A retrospective study of BDU patients who underwent PR-OCTA examination between April 1, 2017 and October 31, 2018. Results : Sixty consecutive BDU patients (102 eyes) were included. Sixty-two (124 eyes) healthy subjects served as normal controls (NCs). As compared with NCs, the vessel densities (VDs) of superficial and deep retinal capillary plexuses were decreased, and the fovea avascular zone area, perimeter and acircularity index were increased in BDU eyes (all p < .001). Macular edema ( p < .001) and long uveitis course ( p = .033) were identified as risk factors for capillary disruptions. Consistent VD reductions were observed in the quiescent fellow eyes of BDU patients during unilateral uveitis relapse. Conclusions : BDU patients had significant OCTA changes. Macular circulatory disturbances may exist in the quiescent fellow eyes during unilateral BDU attack.

Purpose : To investigate retinal vascular changes in pregnant women using optical coherence tomography angiography (OCTA). Materials and Methods : This prospective, cross-sectional study included 97 pregnant women and age-matched 34 healthy control subjects. The OCTA parameters of one eye (left) of pregnancy group and control group were compared. Besides, the OCTA parameters of three trimesters were compared between themselves and the control group. The foveal avascular zone (FAZ) area and the vessel density percentages of the superficial capillary plexus (SCP), deep capillary plexus (DCP), and radial peripapillary capillary (RPC) of all the groups were evaluated by OCTA. Results : In the pregnancy group, the vessel density of DCP was higher in all regions than the control group except the foveal region, but this difference could not reach statistical significance. On the other hand, the vessel density of SCP in the pregnancy group did not show a manifest change in all regions except the foveal region as in DCP. Although the FAZ area was wider in pregnancy group, this difference was not statistically significant (p:0.661). There was no statistically significant difference observed in vessel density of SCP, DCP, and RPC between the different trimesters and the control group, and none of these data correlated with the gestational week. Conclusion : This study showed retinal vascular changes secondary to pregnancy, especially in the vessel density of DCP. These results can provide information that should be taken into account in the evaluation and investigation of pregnancy-specific retinal vascular pathologies.

Ocular lesions in patients with human immunodeficiency virus (HIV) are commonly due to underlying opportunistic infections. With highly active antiretroviral therapy (HAART), infectious lesions have reduced and noninfectious ocular manifestations including drug-related side effects have been noted. While retinal toxicity has been noted with few other HAART drugs, there are not many on the same with Efavirenz usage. We report a series of five patients with possible efavirenz-related retinal toxicity, visual function abnormalities, and its management. Efavirenz was replaced with alternate anti-retroviral drug. Reversal of ocular side effects were noted subjectively in the form of symptom amelioration of the patients. Objectively, it could be documented with electroretinogram changes and other visual function tests reverting back to normal after change in HAART regime. Early identification of this uncommon side effect in select patients can prevent irreversible vision loss due to efavirenz-associated retinal toxicity.

We report a 10-year-old boy with chronic myelogenous leukemia (CML)-related retinopathy of the eyes. Foveal photoreceptors loss was noted in the right eye, but it was restored with a continued ellipsoid zone after systemic 6-week imatinib mesylate and hydroxyurea treatment. Spectral-domain optical coherence tomography images of the foveal photoreceptors change in the right eye were taken. His best-corrected visual acuity of the right eye recovered from 20/100 to 20/20. Prompt treatment of the underlying CML could result in improvement or resolution of the ocular findings, and even foveal photoreceptors loss might be reversible with good visual acuity recovery.

Purpose: Lysergic acid diethylamide (LSD) is a powerful hallucinogen with high potential for abuse. There is far less known about its effects on the retina, especially the underlying mechanisms. This study was to investigate the acute toxicity of LSD on the retina of C57 mice and its mechanisms of action. Methods: C57 mice were treated with LSD at progressively increasing doses (0.2-1.2 mg/kg) intraperitoneally two times daily for 5 days, mice treated with saline served as negative control. Electroretinography (ERG) was used to test the function of the retina. Toluidine blue staining was used to detect the morphology of the retina. Enzyme-linked immunosorbent assay (ELISA) was used to measure the apoptosis-related factors. Real-time PCR and western blot techniques were used to measure expression changes of genes and proteins, respectively. Results: LSD treatment caused retinal damage, as shown by a decrease in ERG response and the loss of photoreceptor cells. LSD treatment also increased apoptosis through up-regulating the expression of p-JAK1/p-STAT1. Conclusions: Our study indicated that intraperitoneal administration of LSD-induced retinal damage of C57 mice, at least partially through regulating the JAK/STAT pathway.

Purpose : To evaluate macular and radial peripapillary capillary vessel densities (RPC VD) across normal, pre-perimetric (PPG), and early perimetric primary open-angle glaucoma (EG) using optical coherence tomography angiography (OCTA). Materials and Methods : Forty-four PPG eyes, 42 EG eyes, and 41 normal eyes were consecutively enrolled. All subjects underwent visual field, OCT, and OCTA examinations. Macular vessel densities were measured in the superficial layer. RPC VD was measured within a 750-μm-wide elliptical annulus extending outward from disc boundary. Diagnostic ability was evaluated with area under the receiver operating curve. Results : Parafoveal vessel densities (ParaVDs) in the PPG group were comparable to the normal group ( p > .05), except for temporal quadrant ( p = .044), while perifoveal vessel densities (PeriVD) were lower in the PPG group ( p < .05). ParaVD and PeriVD were lower in the EG group compared with the normal group ( p < .001). Superior-temporal and inferior-temporal retinal nerve fiber layers (RNFLs) were significantly decreased in the EG group compared with the PPG group ( p < .05), while only inferior-temporal RPC VD was lower in the EG group ( p < .05). RPC VD was comparable to RNFL and ganglion cell complex (GCC) in the diagnosis of PPG ( p > .05). Macular whole image vessel density and PeriVD were comparable to RPC VD, RNFL, and GCC in diagnosing EG ( p > .05). Conclusions : In early primary open-angle glaucoma, significant microvascular damage was present in both macular and peripapillary areas. The damage of macular microvasculature was more prominent in the peripheral area. Vessel density parameters showed promising diagnostic ability in early glaucoma. Abbreviations : POAG = primary open-angle glaucoma; RGC = retinal ganglion cell; OCTA = optical coherence tomography angiography; RNFL = retinal nerve fiber layer; PPG = pre-perimetric primary open-angle glaucoma; EG = early perimetric primary open-angle glaucoma; IOP = intraocular pressure; PSD = pattern standard deviation; MD = mean deviation; GCC = ganglion cell complex; SBP = systolic blood pressure; DBP = diastolic blood pressure; MAP = mean arterial pressure; MOPP = mean ocular perfusion pressure; wiVD = whole image vessel density; ParaVD = parafoveal vessel density; PeriVD = perifoveal vessel density; RPC VD = radial peripapillary capillary vessel density; AUC = area under the receiver operating curve.

Pregnancy is a normal state of physiological stress that induces significant changes in the human body, some of which can potentially affect the visual system. The pregnant state may exacerbate pre-existing ocular disorders (i.e. diabetic retinopathy or central serous retinopathy). Moreover, other systemic conditions related to pregnancy can induce ophthalmic disorders affecting the retina and the choroid, some of which are of acute nature. Increasing awareness and improving collaboration between ophthalmologists, obstetricians and internists can play a pivotal role in the management of complex conditions during pregnancy. Special attention should be given while prescribing medications or deciding about other diagnostic or therapeutic techniques. The purpose of this review is to summarise the physiologic and pathologic effects of pregnancy in the eyes, highlighting the most acute clinical entities that may be threatening for the vision or even the life of the mother and her baby.

Purpose : The goal of this work was to determine whether the retinal tissue perfusion (RTP) is impaired in patients with multiple sclerosis (MS). Methods : Seventy-four patients [66 relapsing-remitting MS (RRMS) and 8 clinically isolated syndrome (CIS)] and 74 age- and gender-matched healthy controls were recruited. RTP was calculated as the retinal blood flow (measured using retinal function imager) supplying the macular area divided by the corresponding tissue volume of the inner retina from the inner limiting membrane to the outer plexiform layer, as measured by ultrahigh-resolution optical coherence tomography. Results : The RTP in the MS group was 2.37 ± 0.59 nl/s/mm 3 (mean ± standard deviation), which was significantly lower than the control group (4.06 ± 0.89 nl/s/mm 3 , P < .001), reflecting a decrease of 42%. The blood flow volume was 2.50 ± 0.50 nl/s in MS, which was 45% lower than in the control group (4.56 ± 0.91 nl/s, P < .001). In addition, the tissue volume of the inner retina was significantly lower than in the control group (P < .05). The RTP in patients with MS was significantly correlated with the retinal blood flow volume (r = 0.84, P < .001) and retinal tissue volume (r = -0.56, P < .001). However, the retinal blood flow in patients with MS was not related to the tissue volume (r = -0.06, P = .59). Conclusions : Impaired retinal tissue perfusion occurred in patients with MS, which could be developed as a possible biomarker in monitoring disease progression in MS.

Purpose : To investigate the visual outcomes and occurrences of perioperative complications after three-dimensional (3D) heads-up vitrectomy compared with traditional microscopic (TM) surgery for vitreoretinal diseases. Methods : A retrospective case series comparison of 3D and TM vitrectomy performed on 124 and 202 eyes with a minimum follow-up of 6 months, respectively, by the same surgeon. Changes in visual acuity, primary anatomical success, operation time, and perioperative complications were evaluated. Results : There were no significant differences in age, gender, axial length, ocular and surgical history, surgical indication, and visual acuity between the two groups at baseline. Both groups showed a significant improvement in visual acuity at the final visit (both P < .05) and experienced comparable anatomical recoveries regarding epiretinal membranes, vitreous hemorrhage, macular holes, retinal detachment, and pathological myopic foveoschisis. The mean overall operation time was not significantly longer in the 3D group (31.0 ± 17.6 min) than the TM (31.0 ± 15.9 min) group ( P = .994). The incidence rates of postoperative complications during the follow-up were similar between the 3D (30.6%) and TM groups (30.2%) ( P = .932). Conclusions : The 3D and TM vitrectomy surgeries yielded comparable visual and anatomical outcomes for treatment of vitreoretinal diseases without a significant difference in the rate of complications. The 3D heads-up vitrectomy may be considered the treatment of choice for patients with various vitreoretinal diseases.

Introduction : Chronic inflammatory diseases, including retinal diseases that are a major cause of vision loss, are associated with activation of the nucleotide-binding domain and leucine-rich repeat containing (NLR) protein-3 (NLRP3) inflammasome pathway. In chronic disease, the inflammasome becomes self-perpetuating, indicating a common pathway in such diseases irrespective of underlying etiology, and implying a shared solution is feasible. Connexin43 hemichannels correlate directly with NLRP3 inflammasome complex assembly (shown here in models of retinal disease). Connexin43 hemichannel-mediated ATP release is proposed to be the principal activator signal for inflammasome complex assembly in primary signal-sensitized cells. Connexin hemichannel block on its own is sufficient to inhibit the inflammasome pathway. Areas covered : We introduce chronic retinal disease, discuss available preclinical models and examine findings from these models regarding the targeting of connexin43 hemichannels and its effects on the inflammasome. Expert opinion : In over 25 animal disease models, connexin hemichannel regulation has shown therapeutic benefit, and one oral connexin hemichannel blocker, tonabersat (Xiflam), is Phase II ready with safety evidence in over 1000 patients. Regulating the connexin hemichannel provides a means to move quickly into clinical trials designed to ameliorate the progression of devastating chronic diseases of the eye, but also elsewhere in the body.

Aim: Lutein is a potent antioxidant that is found in ocular tissue. It protects retina against oxidative stress. We aimed to increase lutein efficiency by encapsulating it into liposome and testing its neuroprotective effect against cisplatin-induced retinal injury in rabbits. Materials and methods: Twenty-four male, New Zealand, rabbits weighing 1.5-2 kg were divided into four groups, as follows: group I as a control, group II received cisplatin, group III received free lutein + cisplatin and group IV received liposomal lutein + cisplatin. All treatments were administrated twice per week for 14 days. Electroretinogram (ERG) was recorded for all rabbits just before decapitation. Then, the retinae were subjected to histopathological evaluations and comet assay. Results: Results indicated significant decrease ( p  ˂ 0.01) in ERG waves, significant increase ( p  ˂ 0.01) in all parameters of comet assay (% tailed cells, tail length, DNA% in tail and tail moment), severe fragmentation in photoreceptors layer and changes in inner retina after the administration of cisplatin. There were some sort of improvement in ERG, comet assay and the histological results after the administration of lutein with cisplatin, whereas these tests yielded values comparable to control in the liposomal lutein group. Conclusions: Liposomal lutein administration could prevent the detrimental effects of cisplatin on the retina, while avoiding the use any artificial chemicals. The latter might be preferable but with possible highly toxic effects. Results were promising and worse further future investigations.

Purpose : To investigate the clinical characteristics of perifoveal exudative vascular anomalous complex (PEVAC) in Korean patients. Methods : This retrospective study included 8 eyes (8 patients) diagnosed with PEVAC. Demographic characteristics of the patients, fundus photography, optical coherence tomography (OCT), fluorescein angiography, and indocyanine-green angiography (ICGA) findings were analyzed. Available follow-up data were also reviewed. Results : The mean ± standard deviation age was 61.0 ± 11.0 years and mean logarithm of minimal angle of resolution (logMAR) best-corrected visual acuity was 0.15 ± 0.14. PEVAC was noted in 2 eyes with coincident age-related macular degeneration (AMD) and in the fellow eye of 1 eye with unilateral type 3 neovascularization. On fundus photography, PEVAC presented as a perifoveal isolated aneurysm associated with retinal exudation and hemorrhage. Well-defined hyperfluorescent lesions with leakage were noted on fluorescein angiography, but no leakage was observed on ICGA. Anti-vascular endothelial growth factor (VEGF) therapy was performed for 2 patients. However, there was no notable improvement after treatment. Conclusions : The clinical characteristics of PEVAC in Korean patients were similar to those reported in patients of other ethnicities. Further studies are needed to better understand the nature of this newly described clinical entity.

Purpose: To review the clinical applications and diagnostic value of the retinal function imager (RFI), briefly compare RFI to other optical imaging devices, and to describe recent developments., Methods: The search words "Retinal Functional Imager," "optical imaging," "retina angiography," "avascular zone," "foveal avascular zone," and other closely related terms were used in PubMed to review current literature involving the RFI., Results: The functions of the RFI were utilized in over 44 microvascular studies, which reported that the microvasculature may alter in velocity, morphology, and oximetry when affected by a number of ocular, neurological, or systemic diseases. Recently developed automatic algorithms for noninvasive angiography of large retinal regions, segmenting vessels, measuring blood flow, blood velocity, vessel diameter, and oximetry may enhance the clinical applications of the RFI., Conclusion: The RFI has been used to characterize the retinal microvasculature under various conditions of all prevalent retinal diseases in addition to some central nerve system (CNS) and systemic diseases. Applying the RFI in research and clinical settings should help earlier diagnosis, support disease prevention, and improve treatment management.

Microperimetry (MP) is a technology that allows the study of retinal sensitivity at different foveal and parafoveal areas as well as eye fixation. It is a technique of functional evaluation, providing a direct correlation between anatomical and functional outcomes. There are a great variety of studies which evaluate the repeatability or reliability of measurements obtained with this technology and also describe and explore different clinical applications. MP has been shown to be useful in the characterization of sensory and motor conditions, such as amblyopia or nystagmus. Concerning ocular pathology, several studies have confirmed the usefulness of MP for evaluating and analyzing different retinal pathological conditions, such as age-related macular degeneration or glaucoma, and for analyzing the effect of different medical or surgical treatments for these conditions. MP has also been shown to be useful for visual training or rehabilitation in some specific cases.

Introduction: We report a case of retinal and posterior ocular findings in a 33-year-old man diagnosed with Hunter syndrome (Mucopolysaccharidosis type II) in a multimodal imaging way., Case Presentation: Our patient was complaining of blurred night vision for the past 3 years. He had not received any systemic treatment for Hunter syndrome. Vision acuity was 20/20 in both eyes and corneas were clear. Fundus examination revealed bilateral crowded and hyperemic optic nerve heads (elevated in the ocular ultrasound) and areas of subretinal hypopigmentation. There was hyperautofluorescence at the central fovea and perifovea, and a diffuse bilateral choroidal fluorescence in angiography. Macular SD-OCT showed a thinning of the external retina at the perifovea in both eyes. Visual field testing showed a bilateral ring scotoma. The full field ERG was subnormal, with a negative response in the scotopic phase. Visual Evoked Potencial test and cranial MRI were normal., Conclusion: Our multimodal analysis reported here attempted to contribute to the knowledge of the natural history of GAG deposition in the eye, focusing on the retina and retinal pigment epithelium. Defining this natural history is essential for a proper comparison with Hunter patients receiving systemic treatment, thus determining if it can or cannot improve retinal function in humans with this disorder.

Adult-onset Still's disease (AOSD) is a rare multisystemic immune-mediated disease of unknown etiology with quotidian spiking fever, evanescent rash, arthralgia, and multiple organ involvement. The few AOSD cases that have been reported developed Purtscher's-like retinopathy associated with thrombotic microangiopathy (TMA). Here, we report Purtscher's-like retinopathy without TMA in a patient with AOSD. A 29-year-old-man who presented for evaluation of blurred vision was diagnosed with AOSD based on Yamaguchi criteria. He had Purtscher's-like retinopathy in his right eye. Lesions improved after steroid treatment. Although almost all reported AOSD cases with Purtscher's-like retinopathy are associated with TMA, in this case such a complication was not encountered.

Purpose: The purpose of this study was to evaluate retinal and choroidal thickness in patients with Parkinson's disease (PD) using spectral domain-optical coherence tomography (SD-OCT)., Material and Methods: Participants in our study were 31 PD patients and 25 age-matched healthy controls. All participants underwent best-corrected visual acuity (BCVA) measurement, slit-lamp biomicroscopy, dilated fundoscopy, and SD-OCT. Macular and retinal nerve fiber layer (RNFL) thickness and choroidal thickness in all quadrants were recorded., Results: There was a statistically significant reduction in average, superior, and temporal RNFL thickness, average ganglion cell complex, and choroidal thickness in the subfoveal area and in all quadrants between patients with PD and controls., Conclusions: The role of the choroidal vasculature in the pathophysiology of PD should be further scrutinized.

Purpose: Imaging technology has advanced by leaps and bounds in the recent past and has resulted in a much greater understanding of ocular diseases. The aim of this review article is to summarize optical coherence tomography (OCT) findings of various systemic conditions., Method: A systematic literature search of the Medline/PubMed database was performed. English articles up to April 2015 were included. Terms used for search included: Alzheimer's Disease; Multiple Sclerosis; Parkinson's Disease; Behçet's Disease; Schizophrenia; Migraine; Obstructive Sleep Apnea Syndrome; Neurofibromatosis; Sickle Cell Disease; Renal diseases; Lupus Retinopathy; Valsalva Retinopathy; Whiplash Retinopathy; Shaken-Baby Syndrome; Choroidal metastases; Intracranial Hypertension; Drug toxicity; Deferoxamine; Sildenafil; Tamoxifen; Hydroxychloroquine; Chloroquine; Ethambutol; Lead; Sickle Cell Disease; and Thalassemia along with OCT., Results: Studies have shown that inner retinal thinning could be the earliest sign of neurological diseases and may help to differentiate individuals with abnormalities. Outer retinal damage was noted in cancer-related retinopathy and secondary to drug toxicity as a diagnostic sign. This review article summarizes the OCT findings and their importance in early diagnosis, treatment, and follow-up in a varying spectrum of systemic diseases including neurological diseases, hematological diseases, cancer-related retinopathies, and systemic drug toxicity., Conclusion: OCT findings are useful to predict the probability of a disease, to diagnose it early, to differentiate between healthy and unhealthy tissue, and to assess the effect of therapeutic interventions in many systemic diseases.

Purpose: To investigate best corrected visual acuity (BCVA), and choroidal and retinal thickness values between high myopes without myopic maculopathy and emmetropes., Materials and Methods: Case control study where 53 myopes with axial length (AL) above 26 mm without myopic maculopathy and 53 age-matched emmetropes with AL between 21.50 and 24.50 were included as controls. Complete ophthalmological examination and biometry were performed. Choroidal and individual retinal layer thickness maps using spectral domain optical coherence tomography were obtained in the macular and peripapillary area with enhanced depth imaging. Peripapillary retinal nerve fiber layer (pRNFL) thickness was obtained using the circular 12°diameter scan., Results: Mean age was 31.9 ± 9.9 and 32.5 ± 9.3 years in the myopes and controls, respectively (p > 0.05). Mean BCVA was 55.32 ± 2.50 versus 57.04 ± 2.27 ETDRS letters, in the myopes and controls, respectively (p = 0.0004). AL was the principal predictive factor for macular and peripapillary CT in myopes and macular CT in controls. BCVA was not influenced by choroidal thickness (CT). BCVA positively correlated with global pRNFL, following correction for age and AL, in both groups (r = 0.38, p = 0.008 and r = 0.38, p = 0.007 in the myopic and control groups, respectively). Statistical analysis following correction for the potential confounding factors of age, gender, AL, gender, AL, macular CT, and peripapillary CT, showed no significant differences in macular and peripapillary thicknesses between the two groups., Conclusions: AL is the principal predictive factor for macular and peripapillary CT in high myopes without maculopathy, and CT is not an independent predictor of visual acuity. Global pRNFL thickness is the only independent predictive factor of BCVA.

Purpose: Multimodal imaging has not been documented for CABP4-related retinopathy. In this study, we describe optical coherence tomography (OCT) and fundus autofluorescence findings for five genetically confirmed cases., Methods: Retrospective case series., Results: Four patients with the previously described homozygous Saudi CABP4 founder mutation c.81&#95;82insA (p.Pro28ThrfsX44) and one patient with the homozygous mutation c.1A>G (p.Met1?) in CABP4 were examined. The ages ranged between 9 and 16 years at last follow-up, and the duration of follow-up ranged from 2 to 12 years. Foveal thickness was reduced ranging between 175 and 212 micrometers. Wide field imaging including fundus autofluorescence was unremarkable. All patients presented with a negative electroretinogram, with a variable amount of cone and rod dysfunction. Over follow-up, there was no electroretinographic indication of any progressive retinal dysfunction., Conclusions: Foveal thinning is a feature of CABP4 retinopathy. Normal autofluorescence is consistent with inner retinal dysfunction and suggests the condition could be amenable to gene therapy. Retinal dysfunction was stable throughout follow-up.

Purpose: To detect the safety of intravitreal injection of anti-connective tissue growth factor (CTGF) (IVAC) in rat eyes in order to apply this neutralizing antibody for experimental animal studies., Methods: Forty-five Lister Hooded male pigmented rats were divided into five groups that received IVAC (2 μl) corresponding to the doses of 10 (B), 20 (C), 50 (D), and 100 μg/ml (E), equal to 1.25, 2.5, 6.25, and 12.5 µg/ml of antibody concentration in rat vitreous, respectively. The sham group (A) received 2 μl of normal saline. Full field electroretinography (ERG) was performed at baseline and on days 7 and 28 after IVAC. The animals were euthanized and the corresponding eyes were subjected to routine histopathology, immunohistochemistry for glial fibrillary acidic protein (GFAP), and terminal transferase dUTP nick end-labeling (TUNEL) assay., Results: Scotopic rod b-wave amplitude and maximal combined b-wave amplitude were 111.89 ± 71.2 and 178.57 ± 55.58 μV, respectively, at baseline which significantly reduced to 79.31 ± 52.59 and 128.73 ± 41.61 μV, respectively, after 28 days in group E (p < 0.05). There was no significant reduction of amplitudes in other groups with lower doses of anti-CTGF antibody. Retinal ganglion cells were significantly decreased in group E as compared to other groups. GFAP immune reactivity was not significant in any of the groups. TUNEL test showed inner retinal neural cell apoptosis only in group E., Conclusions: ERG, histopathologic, and apoptotic assays revealed no toxic effects of 10-50 μg/ml of IVAC in rat eyes. Using 100 μg/ml IVAC led to a significant toxic effect in terms of functional, histopathologic, and TUNEL findings.

Purpose: To investigate prognostic factors for retinal sensitivity assessed by microperimetry in patients with Vogt-Koyanagi-Harada (VKH) disease., Methods: In total, 34 patients with initial-onset acute disease and 19 patients with chronic recurrent disease were retrospectively evaluated., Results: The mean follow-up period was 40.4 ± 40.5 months. Sensitivity was significantly worse in eyes with more severe anterior segment inflammation at presentation, as indicated by the presence of mutton-fat keratic precipitates, anterior chamber reaction ≥2+, and posterior synechiae. Chronic recurrent presentation, development of complications, and 'sunset glow fundus' were significantly associated with worse sensitivity. Using logistic regression analysis, better sensitivity was significantly associated with initial-onset acute presentation (odds ratio, OR = 6.9; 95% confidence interval, CI = 1.53-9.66)., Conclusions: Chronic recurrent presentation and development of complications and 'sunset glow fundus' are associated with a worse sensitivity outcome.

Purpose: To evaluate the agreement of intraocular pressure (IOP) measurements using dynamic contour tonometry (DCT) and Goldmann applanation tonometry (GAT) in eyes after vitrectomy with silicone oil endotamponade and controls., Methods: In this prospective comparative study, IOP was measured with GAT and DCT in 30 eyes with oil endotamponade 1-3 days after vitrectomy and 40 untreated controls. In addition, ocular pulse amplitude (OPA), corneal pachymetry (CCT), and axial length (AL) were measured., Results: GAT values in the oil group were significantly higher compared to control eyes (mean GAT oil 13.6 ± 5.1 mmHg; mean GAT control 10.8 ± 2.1 mmHg; p = 0.003). There was no significant difference in DCT measurements (mean DCT oil 12.0 ± 4.1 mmHg; mean DCT control 11.9 ± 2.9 mmHg; p = 0.9). This led to a significant difference of GAT-DCT between the oil and control group (mean difference of GAT-DCT oil 1.6 ± 4.7 mmHg; mean difference of GAT-DCT control -1.1 ± 2.6 mmHg; p = 0.004). The difference between GAT and DCT was negatively correlated with the mean IOP measured by both methods (r = -0.36, p = 0.02) and positively correlated with CCT only in the control group (r = 0.36, p = 0.02), as well as to AL only in the oil group (r = 0.46, p = 0.01). The OPA did not differ significantly between groups., Conclusion: GAT and DCT showed a good agreement in control eyes. The difference of GAT and DCT is significantly changed in eyes after vitrectomy with silicone oil endotamponade. Our findings suggest that GAT overestimates IOP in this situation.

Purpose: Retinal ischemia is a common cause of visual impairment and blindness. Sildenafil, a PDE5 inhibitor which inhibits cGMP degradation and in turn prolongs the effect of nitric oxide, has been shown to be protective in a number of ischemia/reperfusion (I/R) injuries, as well as in neuronal damage. We hypothesized that treatment with sildenafil might be neuroprotective in a model of acute retinal I/R injury., Methods: Anterior chamber cannulation was performed to induce unilateral I/R injury in 38 Lewis rats. Animals received intraperitoneal injections of sildenafil (0.5 and 1 mg/kg once a day, for a period of 7 and 18 days, respectively), or saline. Electroretinography recordings, retinal ganglion cell (RGC) counts following retrograde labeling with fluorogold, histopathology, and immunohistochemistry (IHC) using antibodies against PDE5, NOS2, caspase-3, caspase-9, and Bcl-2 were conducted., Results: No significant differences in electroretinography, RGC counts, or retinal morphometry were observed between experimental eyes of sildenafil- and saline-treated animals. A tendency toward less necrosis in histopathology, and a slight trend toward lower PDE5, NOS2, and caspase-9 and higher Bcl-2 IHC scores were evident in experimental retinas of sildenafil-treated animals., Conclusions: Electroretinography, RGC counts, and retinal morphometry failed to show any neuroprotective effect of sildenafil in acute retinal I/R injury in rats. A slight positive effect of sildenafil was qualitatively indicated by histopathology and IHC.

Background: Mulvihill-Smith syndrome is a rare progeroid syndrome of sporadic nature. Previously reported ophthalmological findings include astigmatism, myopia, endothelial dystrophy, keratoconus, cataract, band keratopathy, meibomian gland dysfunction, dry eye disease, amblyopia, and allergic conjunctivitis., Materials and Methods: The proband, a 25-year-old male subject diagnosed with Mulvihill-Smith syndrome in childhood developed retinal changes with onset of adulthood. The retinal changes were monitored for progression with fundus photography, electrodiagnostic tests, and spectral domain optical coherence tomography., Results: The fundus examination revealed grossly normal looking retina with dull foveal reflex. The optical coherence tomography scan of the retina revealed diffuse thickening, schisis, and folding of retinal layers in both eyes. The structural changes in retina were progressive with wrinkling of inner retinal layers and loss of foveal contour as observed over 3 years. The electrodiagnostic tests revealed normal photoreceptor-retinal pigment epithelial interface., Conclusions: This is the first report of retinal features in Mulvihill-Smith syndrome. These ocular changes coincided with other systemic changes with the onset of adulthood. These changes may indicate the natural history of retinal features in this progeria syndrome with short life span. The detailed analysis and progression of structural changes in retina is possible with optical coherence tomography.

Purpose: Astaxanthin exhibits various pharmacological activities, including anti-oxidative, anti-tumor, and anti-inflammatory effects, and is thought to exert a neuroprotective effect via these mechanisms. The purpose of this study was to investigate the protective effects of astaxanthin on neuronal cell death using a retinal ischemia/reperfusion model., Methods: In vivo, retinal ischemia was induced by 5 h unilateral ligation of the pterygopalatine artery (PPA) and the external carotid artery (ECA) in ddY mice. Astaxanthin (100 mg/kg) was administered orally 1 h before induction of ischemia, immediately after reperfusion, at 6 or 12 h after reperfusion, and twice daily for the following 4 days. Histological analysis and an electroretinogram (ERG) were performed 5 days after ischemia/reperfusion. In vitro, cell death was induced in the RGC-5 (retinal precursor cells) by oxygen-glucose deprivation (OGD), and the rates of cell death and production of intracellular reactive oxygen species (ROS) were measured using nuclear staining and a ROS reactive reagent, CM-H 2 DCFDA., Results: Histological studies revealed that astaxanthin significantly reduced retinal ischemic damage and ERG reduction. In in vitro studies, astaxanthin inhibited cell death and ROS production in a concentration-dependent manner., Conclusions: Collectively, these results indicate that astaxanthin inhibits ischemia-induced retinal cell death via its antioxidant effect. Hence, astaxanthin might be effective in treating retinal ischemic pathologies.

Purpose: To assess the effect of a 50 Hz power line digital filter on the response to a 2-global-flash multifocal electroretinogram (mfERG) in primary open angle glaucoma (POAG) compared to control., Materials and Methods: A 2-global-flash mfERG (VERIS™) was recorded (23 control, 34 POAG). Eight recordings were noise contaminated: 4 control, 4 POAG. Response averages from the central 10° and 7 surrounding groups were analyzed with and without a 50 Hz digital filter for the following mfERG response epochs: direct component (15-45 ms), 1st (45-75 ms) and 2nd (75-105 ms) induced components., Results: A digital 50 Hz filter had little effect on uncontaminated with noise waveforms but, in noisy recordings, changed the waveform dramatically to resemble uncontaminated waveforms. In controls' 50 Hz-filtered uncontaminated with noise mfERGs differed significantly from unfiltered responses in induced components. Uncontaminated with noise recordings from glaucoma patients did not differ with or without the notch filter (p > 0.1 for all three epochs of mfERG). The mfERG response in the central 10°in glaucoma patients differed significantly from controls, whether the notch filter was used or not (p < 0.001)., Conclusions: A 50 Hz notch filter allows grossly contaminated waveforms to be analyzed in a meaningful manner. With a 50 Hz filter, glaucoma patients still differed significantly from normal.

Background: The purpose of this study was to assess retinal vascular characteristics of patients with Laron syndrome (LS) as a genetic model of IGF-I deficiency before and after rhIGF1/IGFBP3 treatment and to compare them with healthy controls., Methods: A total of 28 subjects (11 LS, and 17 controls) were enrolled. Patients with LS received combined rhIGF1/rhIGFBP3 1-2 mg/kg/d in a single dose and digital fundus imaging was performed. The number of branching points and tortuosity of retinal vessels were studied. Pre- and post-treatment findings were compared with each other and with controls., Results: The number of branching points was significantly lower in patients with LS in comparison to controls (12.73 ± 3.41, and 17.47 ± 5.82 respectively, p = 0.012). This difference persisted after treatment (12.09 ± 2.66 post-treatment LS versus controls, p = 0.017). Tortuosity indices of nasal arteries (NA) were significantly less in LS than that of controls (upper NA 1.07 ± 0.04 and 1.12 ± 0.06 respectively p = 0.022; lower NA 1.07 ± 0.03 and 1.13 ± 0.07 respectively, p = 0.004). This difference also persisted following treatment (p < 0.05). Remaining vessels did not differ in tortuosity index. There was no significant difference of tortuosity index and number of branching points before and after treatment in patients with LS., Conclusion: Retinal vascular development may be adversely affected in the setting of severe IGF-I deficiency confirming a major role for GH/IGF-I axis during retinal vascular development in humans antenatally. Resolution of IGF-I deficiency following birth using rhIGF1, however, may not reverse these changes, suggesting that IGF-I may be necessary but insufficient by itself for postnatal angiogenesis.

Purpose: To report on clinical manifestations of Purtscher-like retinopathy (PLR) associated with systemic lupus erythematosus (SLE) and visual outcomes., Methods: We performed a retrospective cohort study of 11 patients (21 affected eyes) with PLR in SLE., Results: All patients were treated with systemic corticosteroids ± immunosuppressive agents. Ocular therapy included intravitreal injections with bevacizumab in 18/21 eyes and posterior sub-Tenon injections with triamcinolone acetonide 13/21 eyes. Panretinal photocoagulation (PRP) was performed in 19/21 eyes and pars plana vitrectomy was required in 5/21 eyes. Visual improvement was found at follow-up of 3 and 6 months (p = 0.05). Poor visual outcome was associated with presence of neovascularizations at onset (p = 0.009), development of vitreous hemorrhage during PRP (p = 0.015), and active status of SLE after onset of PLR (p = 0.029)., Conclusions: PLR might manifest as a devastating complication of SLE. We recommend treating any systemic activity of SLE and starting an early ocular treatment.

Background: "Poppers" is a slang term for a group of alkyl nitrites that are used as recreational drugs. Their inhalative intoxication leads to muscle relaxation, analgesia, and euphoria. Maculopathy is a rare but serious side-effect., Patients/methods: Clinical, imaging, and electrophysiological findings of seven patients with maculopathy after consumption of poppers were presented., Results: All seven patients were male with a median age of 35 years (range 28-45 years), the median duration of periodical poppers use until the onset of symptoms was 9.8 years (one day to 25 years). Five of seven patients were HIV-positive, one patient was negative, and the HIV-status of one patient was unknown. Median average of visual acuity at presentation was 20/30 in each eye. In all patients, optical coherence tomography (OCT) showed pathognomonic alterations of the outer foveal retina. One patient showed an almost complete restitution of the maculopathy six months after cessation of drug use and following the oral intake of Lutein. Imaging alterations returned to normal and visual acuity recovered from 20/50 and 20/30 (right and left eye, respectively) to 20/20 on both eyes. Follow up of two other cases showed no relevant functional decline or improvement., Discussion: Toxic maculopathy due to the consumption of poppers is an important differential diagnosis in acute visual loss without clinico-morphological correlate. Optical coherence tomography is the only reliable diagnostic tool in these cases. Complete recovery of visual function and macular morphology is rare, even after cessation of drug abuse. Oral lutein therapy may have a beneficial effect.

Purpose: To investigate whether retinal impairment was affected by age of rats in acute glaucoma model., Methods: Young adult and aged rats were randomly divided into normal control, 45 mmHg, 60 mmHg and 90 mmHg groups. Intraocular pressures (IOP) of rats were acutely elevated to 45 mmHg, 60 mmHg and 90 mmHg, respectively. Neuron loss in ganglion cell layer (GCL) and activation of retinal macrolgia and microglia 3 days after high IOP treatment were detected by immunofluorescence and further quantitatively analyzed., Results: Compared with normal control, significant loss of neurons at GCL of young adult retina wasn't detected until IOP treatment of 90 mmHg. In contrast, obvious loss of neurons at GCL of aged retina was detected at IOP of 45 mmHg (p = 0.002 for central; p = 0.001 for peripheral). The loss level of neurons of aged retina was significantly higher than that of young adult retina at different IOP treatments. Compared with the young adult retina, high IOP induced more significant increase at area percentage of microglia and microglia number in inner part of aged retina. Activation of microglia and macroglia was either in parallel to or earlier than neuron loss of GCL of aged and young adult retina., Conclusion: Our data suggest there exists an age-related susceptibility of rat retina to the increased IOP. Therefore, the effect of ages should be considered at glaucoma study of rat models.

Purpose: To describe three siblings with childhood cone-rod dystrophy and macular cystic degeneration in a family with apparently variable phenotypes of CRB1-related recessive retinal dystrophy., Methods: Ophthalmologic examination (including electroretinography (ERG), ocular coherence tomography (OCT), and intravenous fluorescein angiography when possible) and homozygosity analysis guided candidate gene testing., Results: When the proband was evaluated at 7 years old for progressive visual loss, fundus exam was unremarkable (including no macular thickening clinically or by OCT) but ERG revealed cone-rod dysfunction with an electronegative waveform. Four years later repeat examination was significant for bilateral macular cystic degeneration and immediate family members were evaluated. Both the older sister (15 years old) and the younger brother (7 years old) had cone-rod dystrophy with macular cystic degeneration. Both the father (45 years old) and mother (35 years old) had had early adult-onset nyctalopia with later eventual loss of central vision; examination revealed dystrophic retinas with mostly peripheral clumped and/or nummular pigment and macular atrophy. ERG for both the older sister and younger brother confirmed cone-rod dysfunction (without an electronegative waveform) and was non-recordable for both the parents. Homozygosity analysis guided candidate gene analysis and confirmatory Sanger sequencing for the family uncovered a homozygous CRB1 mutation (c.80G > T [p.Cys27Phe]) in affected family members., Conclusions: The phenotypic spectrum of recessive CRB1 mutation includes childhood cone-rod dystrophy with macular cystic degeneration and the associated ERG can be electronegative.

Purpose: To describe 3 representative cases of autoimmune retinopathy (AIR)., Methods: Clinical records of patients with a diagnosis of AIR were analyzed for demographic data, clinical findings, ancillary and laboratory tests, and treatment employed., Results: Three female patients diagnosed with AIR had bilateral reduction of electroretinogram amplitudes and elevation of visual field threshold within the central 30 degrees of the visual field that was disproportionately more severe than the clinical findings of retinal degeneration. The diagnoses were cancer-associated retinopathy, non-neoplastic AIR, and hereditary retinal dystrophy with secondary inflammation. Optic nerve involvement was also present in all cases. The patient with non-neoplastic AIR was successfully treated with systemic corticosteroids and immunomodulatory agents., Conclusion: High index of suspicion is essential for an early diagnosis of AIR. Visual function and electrophysiological tests should be included in the initial workup of patients who present with suggestive clinical signs and symptoms of AIR.

Purpose: To evaluate changes in the retinal vasculature in eyes with myopic retinopathy., Methods: Population-based, cross-sectional study. Emmetropic and myopic participants from the Blue Mountains Eye Study baseline survey were included in this study. Myopia was defined as a refractive error of less than -1.00 diopter. Myopic retinopathy was defined if either staphyloma, lacquer crack, Fuchs' spot or chorioretinal atrophy were present in myopic eyes. Retinal vascular caliber was measured from fundus photographs using standardized methods. The association of retinal vascular caliber with myopic retinopathy was assessed using generalized estimating equation models., Results: A total of 2598 eyes of 1409 subjects were selected from 3654 baseline participants, with 2076 emmetropic eyes (normal controls), 486 myopic eyes without myopic retinopathy (myopic controls) and 36 myopic eyes with myopic retinopathy (cases). After adjusting for age, gender, height, body mass index and blood pressure, eyes with myopic retinopathy had significantly narrower mean arteriolar (166.6μm) and venular caliber (213.3μm), compared to normal (188.1μm and 226.9μm, respectively) or myopic control eyes (190.4μm and 227.0μm, respectively) (all P < 0.001). These retinal vessel caliber differences between myopic retinopathy and the two control groups remained significant after additional adjustment for refraction (all P < 0.001)., Conclusions: Our findings suggest that myopic retinopathy is associated with attenuation of retinal vessels.

Purpose: To investigate the effect of anti-high mobility group box 1 (HMGB1) monoclonal antibody (mAb) against ischemia-reperfusion injury in the rat retina., Materials and Methods: Retinal ischemia was induced by increasing and then maintaining intraocular pressure at 130 mmHg for 45 min. An intraperitoneal injection of anti-HMGB1 mAb was administered 30 min before ischemia. Retinal damage was evaluated at 7 days after the ischemia. Immunohistochemistry and image analysis were used to measure changes in the levels of reactive oxygen species (ROS) and the localization of anti-HMGB1 mAb. Dark-adapted full-field electroretinography (ERG) was also performed., Results: Pretreatment with anti-HMGB1 mAb significantly enhanced the ischemic injury of the retina. HMGB1 expression increased at 6-12 h after ischemia in the retina. After the ischemia, production of ROS was detected in retinal cells. However, pretreatment with anti-HMGB1 mAb increased the production of ROS. On the seventh postoperative day, the amplitudes of both the ERG a- and b-waves were significantly higher in the vehicle group than in the groups pretreated with anti-HMGB1 mAb., Conclusions: The current in vivo model of retinal injury demonstrated that anti-HMGB1 mAb plays a large deleterious role in ischemia-reperfusion injury. In order to develop neuroprotective therapeutic strategies for acute retinal ischemic disorders, further studies on anti-HMGB1 mAb function are needed.

Purpose: To report measures of inner retinal integrity following improvement in visual acuity and visual fields in a patient with hereditary motor and sensory neuropathy type VI (HMSN VI)., Case Report: The patient is a Caucasian male with HMSN VI (type 2A Charcot-Marie-Tooth disease and associated optic atrophy) and a c.1090C→T (p.R364W) mutation in the mitofusin 2 (MFN2) gene. The patient's best-corrected visual acuity improved from 20/200 (OD) and 20/400 (OS) at the initial visit to 20/25 in each eye when tested 7 years later. The visual field defects in both eyes that were present at the initial visit were absent at the follow-up visit. The structural integrity of the inner retina was assessed by an evaluation of retinal nerve fiber layer thickness (RNFLT) using optical coherence tomography (OCT), and the functional integrity was assessed by the amplitude of the photopic negative response (PhNR) of the electroretinogram (ERG). At the follow-up visit, the patient's RNFLT was less than the 5th percentile for control subjects in the superior and inferior quadrants OD and in one sector of the temporal quadrant OS, but was within normal limits elsewhere. The PhNR amplitude of each eye was below the lower limit of the normal range., Conclusion: The abnormally low PhNR amplitudes and abnormally thin RNFL in certain quadrants of the retina following improvement of visual acuity and visual fields to near-normal values illustrates the potential usefulness of assessing the structure and function of the inner retina in HMSN VI patients.

Purpose: To report the rare observation of CNGA3 mutation as a cause of oligocone trichromacy (OT) and present phenotypic characteristics., Methods: A 20 year old male patient underwent ophthalmological evaluation including detailed color vision assessment using Ishihara pseudoisochromatic plates, American Optical Hardy Rand Rittler plates (HRR) and Mollon-Reffin Minimalist test (MRM). Optical coherence tomography (OCT), fundus autofluorescence (FAF), visual field assessment and electrophysiological testing was also performed. The patient's DNA was sequenced for mutations in the coding sequence of CNGA3 and CNGB3 genes., Results: Best corrected visual acuity (BCVA) was 20/50 and 20/30 in the right and left eyes respectively. His color vision was normal to Ishihara, HRR and MRM tests. Fundus appearance, FAF, OCT and Goldmann visual fields (GVF) were all normal. Humphrey visual field analysis (HVF) demonstrated reduced sensitivity and paracentral scotomas (5-20°). The full-field electroretinogram (ERG) showed normal rod responses and severely reduced cone responses. The multifocal electroretinogram (mfERG) was non-recordable above noise. Compound heterozygous mutations in exon 8 of the CNGA3 coding sequence were identified; c.1070 A > G (Tyr357Cys; novel) and c.1694 C > T (Thr565Met). Allele-specific polymerase chain reaction confirmed that the mutations were located on separate alleles. No mutations were identified in CNGB3., Conclusion: This is the second reported case of CNGA3 associated OT. Mutations in CNGA3 have previously been associated with incomplete and complete achromatopsia. This report confirms that OT forms the mildest end of the spectrum of CNGA3 related diseases.

Purpose: To report results and complications of 23-gauge transconjunctival sutureless pars plana vitrectomy for a variety of vitreoretinal diseases., Methods: A prospective consecutive case series study was performed in 66 eyes of 66 patients. Indications for surgery were epiretinal membrane (n = 20), rhegmatogenous retinal detachment (n = 19: 14 pseudophakic, 5 phakic), macular hole (n = 16), vitreous hemorrhage (n = 5), cyclodialysis (n = 1), intraocular lens luxation (n = 1), asteroid hyalosis (n = 1), congenital retinoschisis (n = 1), and endophtalmitis (n = 2). Main outcome measures included visual acuity, intraocular pressure, and intra- and post-operative complications., Results: Mean patient age at time of operation was 68 ± 12 years. Overall, visual acuity improved from 1.03 ± 1.00 logMAR preoperatively to 0.32 ± 0.33 logMAR postoperatively (p < 0.01) after a mean follow-up time of 9.3 ± 4.7 months. Mean preoperative intraocular pressure was 13.9 ± 3.5 mmHg, and mean postoperative intraocular pressure was 17.9 ± 9.6 mmHg on day 1 (p < 0.01) and 14.7 ± 2.8 mmHg (p = 0.05) at final visit. Concerning complications, 2 cases of hypotony and 7 of hypertony occurred on day 1, a macular hole reopened some weeks later, and a retinal detachment recurred in one case., Conclusion: 23-gauge transconjunctival sutureless vitrectomy is an effective and safe technique for a variety of vitreoretinal diseases.

Purpose: Retinal ischemia-induced neuronal death plays a crucial role in certain severe visual impairment diseases. The aims of this study were to investigate the effects of low dose cobalt protoporphyrin IX (CoPP), an inducer of heme oxygenase-1 (HO-1), on the retina of rats against ischemia-reperfusion (IR) injury., Methods: Retinal IR was achieved in rats by raising intraocular pressure for 60 min. CoPP (1 mg/ kg) was injected intraperitoneally 24 hr before IR. Retinal injury was assessed by the number of retinal ganglion cells (RGCs) seven days after reperfusion. TUNEL assay was used to detect the appearance of apoptotic cells 24 hr after reperfusion. The expressions of the HO-1 and Bax proteins were evaluated by Western blot., Results: Both HO-1 expression, examined by Western blot, and enzyme activity were increased strongly after CoPP administration. Rats treated with CoPP before IR had more RGCs (p = 0.034) and less apoptotic cells (p = 0.04) together with downregulated Bax protein levels (p = 0.03) compared to ischemic rats without CoPP. The protective effects of CoPP were HO-1 dependent because the upregulation of HO-1 and the RGC protection were both abolished by the HO-1 inhibitor tin protoporphyrin (SnPP)., Conclusions: In this study, we demonstrated that induction of HO-1 expression by low dose CoPP ameliorated retinal damage from IR injury. The favorable effect appears to be related with modulations of the apoptotic pathway.

Purpose: To describe the clinical phenotype and electroretinographic changes in two siblings with primary hereditary lateral sclerosis., Materials and Methods: Case series of two male siblings from a consanguineous family. A clinical evaluation and full field electroretinography according to International Society for Clinical Electrophysiology of Vision (ISCEV) standards was performed., Results: Both siblings had reduced central vision without nystagmus and a normal fundus examination. The electroretinogram showed cone dysfunction with markedly reduced amplitude 30-Hz flicker, and broad, delayed photopic single flash of normal amplitude. The rod driven electroretinography (ERG) b-waves were within the normal range., Conclusions: Cone dysfunction has not been reported previously in patients with primary lateral sclerosis. Although it is possible this is a chance association it is more likely that the retinal dysfunction is caused by the same genetic mutation causing the neurological disease. Investigation of other cases will be helpful in delineating the phenotype. The association may also help prioritize candidate gene analysis in the search for the underlying genetic mutation causing primary lateral sclerosis.

Purpose: To describe a case of persistent placoid maculopathy (PPM) associated with juxtafoveal choroidal neovascularization (CNV) treated with intravitreal bevacizumab injection (IVBI)., Design: Interventional case report., Methods: A 60-year-old patient referred for sudden vision loss was diagnosed with PPM complicated by a disciform scar in the right eye and juxtafoveal CNV in the left eye, undergoing 2 IVBIs., Results: Best-corrected visual acuity passed from 20/32 to 20/20 after 2 IVBIs, with complete resolution of intraretinal and subretinal fluid and disappearance of fluorescein leakage., Conclusions: IVBI is a promising treatment option for juxtafoveal CNV secondary to PPM.