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Purpose: To identify predictive factors for RPE tear remodelling and its correlation with functional and morphological outcomes., Methods: Retrospective longitudinal study of patients with retinal pigment epithelium (RPE) tears secondary to age-related macular degeneration (AMD). Imaging was performed using spectral-domain optical coherence tomography (SD-OCT) and fundus autofluorescence (FAF). RPE layer integrity in the RPE-denuded area was examined with SD-OCT, and variation in the RPE-denuded homogeneous hypofluorescent area was examined with FAF over time for each case (eye). Patients were divided in two groups, according to the presence (Rem) or absence (No Rem) of evidence of RPE tear remodelling. Data were collected at three different time points: at baseline (at diagnosis of exudative AMD), at RPE tear diagnosis, and at the last available follow-up. Using SD-OCT, the following parameters were evaluated: type of CNV, type of PED and its dimensions, presence of subretinal (SRF) or intraretinal (IRF) fluid, central retinal thickness (CRT), presence and location of hyperreflective dots, and dimension and location of RPE tear., Results: This study included 32 eyes from 31 patients (19 female and 12 male), with RPE tears secondary to AMD. RPE remodelling after tear development was evident in 17 (53.1%) eyes after 7 [1-59] months. Anatomical recovery was associated with a younger age at RPE tear diagnosis (73 ± 7 vs. 81 ± 7 years old, p=0.01), smaller and narrower retinal pigment epithelial detachment (PED) at tear diagnosis (height 369 vs. 602 μm, p=0.02; width 2379 vs. 3378 μm, p=0.04), and the presence of SRF at tear diagnosis (94% vs. 53%, p=0.02). After adjusting for other covariates, a younger age at RPE tear diagnosis maintained significant association with RPE tear remodelling. RPE tear remodelling did not correlate with a better visual outcome at last follow-up (43 ± 22.8 vs. 34 ± 23.8 ETDRS letters, p=0.30). Final VA was directly proportional to VA at tear diagnosis (r= 0.654; p<0.001) and correlated negatively with PED width at tear diagnosis (r = -0.388; p=0.03)., Conclusion: RPE remodelling was evident in half of our sample and was associated with a younger age, smaller and narrower PED at RPE tear diagnosis, and presence of SRF also at tear diagnosis. Nevertheless, this structural recovery did not result in a better functional outcome., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH, DE part of Springer Nature.)

Purpose: To describe a case of a child with mild phenotype of Incontinentia Pigmenti (IP), with changes in Spectral-Domain Optical Coherence Tomography (SD-OCT) and Optical Coherence Tomography Angiography (OCT-A) and an electronegative dark-adapted (DA) 3.0 electroretinogram (ERG), suggestive of inner retinal dysfunction., Case Report: We described a 7-year-old female child with IP. Her best corrected acuity was 8/10 in the right eye and 6/10 in the left eye. Biomicroscopy, intraocular pressure and fundoscopy were normal. The electroretinography findings showed an electronegative DA 3.0 ERG with a normal a-wave but a b-wave that did not elevate above baseline. SD-OCT identified irregularities in the outer plexiform layer in both eyes, and OCT-A assessment revealed at the superficial capillary plexus, areas of decrease in the flow in parafoveal and perifoveal regions., Conclusion: Classically, IP affects the peripheral retina; however, vascular and structural changes in macula can occur as well. To our knowledge, we report the first electronegative electroretinogram in a patient with IP.

Purpose: To characterise a sample of patients with inflammatory choroidal neovascularization (I-CNV), including clinical profile, underlying aetiology and its course, treatments performed, associated clinical response, and visual prognosis., Methods: Retrospective analysis of patients with a diagnosis of I-CNV followed at the Ophthalmology Department of Centro Hospitalar Universitário de São João (CHUSJ). Clinical and visual outcomes were classified according to the difference in visual acuity after treatment., Results: Twenty eyes from 17 patients were analysed (11 female and 6 male patients, mean age 41.90 ± 16.457 years at CNV diagnosis). Punctate inner choroidopathy/multifocal choroiditis was the predominant inflammatory aetiology (10 patients, 58.82%). Median follow-up time was 46 months (range 10 to 188 months). Neovascularization was treated with intravitreal anti-VEGF injections (bevacizumab, aflibercept, and ranibizumab), and inflammation with anti-inflammatory/immunosuppressive therapy (oral, intravenous, and/or intravitreal corticosteroids; oral cyclosporine or methotrexate). Intravitreal anti-VEGF agents had a median number of 7.00 injections (IQR, 4.25 to 29.00). Visual acuity among 20 eyes had a mean gain of 15.10 ± 12.998 ETDRS letters after anti-VEGF treatment ( p =0.000051). According to our classification, 16 had an improved outcome (80.00%), 3 had a stable outcome (15.00%), and 1 had a worsened visual outcome (5.00%). In addition, 13 eyes (65.00%) had a final VA equal to or greater than 65 letters. Recurrence was seen in 3 eyes (15.00%). Complications included cataract (6 patients) and ocular hypertension (4 patients)., Conclusion: A combined approach with anti-VEGF agents and anti-inflammatory therapy was effective in I-CNV treatment, and an overall good visual prognosis was attainable. Intensive follow-up was fundamental in the management of both the primary inflammatory and secondary neovascular conditions., Competing Interests: The authors declare that there are no conflicts of interest regarding the publication of this study., (Copyright © 2021 Gonçalo Carrola et al.)

Purpose: To evaluate long-term changes in the foveal and parafoveal outer retina after half-dose photodynamic therapy (HD-PDT) in central serous chorioretinopathy (CSC)., Methods: Retrospective study including CSC patients submitted to HD-PDT. Best corrected visual acuity (BCVA) was evaluated. Spectral-domain optical coherence tomography automatic segmentation algorithm was used and data on retinal, inner retinal, outer retinal and outer nuclear layers (ONL) in both foveal 1 mm (C) and parafoveal 3 mm ETDRS circles for the superior, nasal, inferior and temporal sectors, were obtained at baseline and 3, 12 and 24 months post-treatment. Subfoveal choroidal thickness, photoreceptors' outer segment thickness, subretinal fluid (SRF) height and width were also measured., Results: Twenty-one eyes of 15 patients were included. At baseline, the mean ONL thickness in the foveal area was significantly thinner in affected eyes compared to their fellow unaffected ones (55,50 ± 32,75 μm vs 93,00 ± 17,0 μm; p = 0,011), and was negatively correlated to logMAR BCVA (R=-0,601, p = 0,008) ONL thickness increased by 10,94 ± 11,88 μm at 24 months in the foveal area, and all the parafoveal sectors presented a similar increase. Baseline SRF width was significantly correlated with baseline BCVA (R1 = 0,483, p = 0,036), and with ONL thickness in all sectors., Conclusion: In our study we found a significant long-term increase in foveal and parafoveal ONL thickness in CSC after HD-PDT, suggesting that this seems to be a safe treatment for the outer retina. This is the first study mapping the outer retinal changes in the macular area to 24 months follow up., (Copyright © 2021. Published by Elsevier B.V.)

Background: Retinitis punctata albescens is a form of retinitis pigmentosa characterized by white fleck-like deposits in the fundus, in most cases caused by pathogenic variants in RLBP1 gene. The purpose of this work is to report the phenotypic and genotypic data of a patient with retinitis punctata albescens carrying a deletion in the RLBP1 gene., Results: An 8-year-old Caucasian female has been complaining of nyctalopia for the last 2 years. No other ocular symptoms were present. No relevant past medical or familiar history was described. At clinical examination, the patient's best-corrected visual acuity was 20/20 in both eyes. Anterior segment evaluation and intraocular pressure were normal in both eyes. At fundoscopy, multiple punctate whitish-yellow fleck-like lesions were observed in the proximity of temporal superior and inferior vascular arcades. Scotopic electroretinogram demonstrated severely reduced rod response, without improvement or recovery of rod system function after prolonged dark adaptation. Blood DNA samples of this patient and from her parents were screened for causal variants in RLBP1 , RDH5 , and PRPH2 ., Conclusion: A probable pathogenic frameshift variant was identified in homozygosity in the RLBP1 gene with an autosomal recessive transmission as another cause of retinitis punctata albescens. This DNA variant will aid ongoing functional studies and add to our understanding of the molecular pathology about RLBP1 -associated retinopathies.

Purpose: The purpose was to compare the incidence of endophthalmitis after intravitreal injection with and without topical antibiotic prophylaxis., Methods: This is a single-center, retrospective case-control study. All patients treated with intravitreal injection of ranibizumab, bevacizumab, aflibercept, or corticosteroids for a variety of retinal vascular diseases between 1 October 2014 and 30 November 2018 were included. The total number of patients and injections were determined from a review of billing code and practice management records. Endophthalmitis cases were determined from billing records and then confirmed with chart review. A 24-month period when topical antibiotics were prescribed after intravitreal injection was compared with a 24-month period when topical antibiotics were not prescribed., Results: Between 1 October 2014 and 30 November 2018, a total of 33,515 intravitreal injections were performed and 13 cases of post-intravitreal injection endophthalmitis were identified (incidence rate of 0.0388%; 95% confidence interval, 0.0217%-0.0644%) or approximately 1 case for every 2578 intravitreal injections. Between 1 October 2014 and 31 October 2016, while topical antibiotic prophylaxis was used postoperatively, 14,828 intravitreal injections were performed and 5 cases of endophthalmitis were reported (0.0337%; 95% confidence interval, 0.0129%-0.0739%). Between 1 November 2016 and 30 November 2018, while no prophylaxis was used, 18,687 intravitreal injections were performed and 8 cases of endophthalmitis were identified (0.0428%; 95% confidence interval, 0.0202%-0.0808%). There were no statistical differences in the incidence rates between the two groups ( p  = 0.675)., Conclusion: The incidence rate of endophthalmitis in the group with topical antibiotic prophylaxis after intravitreal injection was similar to the group with no prophylaxis. Changing the current clinical practice to no antibiotic prophylaxis had no effect on the incidence of endophthalmitis.

Purpose : Evaluate the prevalence and incidence, demographic characteristics, anatomical classification, concomitant complications and treatment of uveitis, as well as its association with systemic diseases in the northern Portuguese population referred to a tertiary care centre (São João Hospital, Porto). Methods : A retrospective observational study of 545 consecutive uveitis cases was performed between 12th April 2012 and the 26th October 2017. Uveitis was classified according to Standard Uveitis Nomenclature, while aetiology and complications were diagnosed by specific clinical and laboratory testing. Results : Prevalence was calculated to be 12,4 cases per 100,000 people while incidence was 3.9 cases per 100,000 people annual incidence. The ratio of females to males was 1.32:1 and the mean age at diagnosis was 47,86 years. Anterior uveitis was the most common classification making up 47.5%, followed by non-infectious posterior uveitis 18,0%, infectious posterior uveitis 8,4%, intermediate uveitis 5,5% and panuveitis 4,2%. Extra-uveal entities made up the rest of the causes (16.4%). In terms of aetiology, the majority of uveitis was idiopathic (32,1%) followed by axial spondylarthritis (12.1%), Behçet's disease (8,8%), sarcoidosis (6,1%), tuberculosis (5,5%), herpes (5,1%), toxoplasmosis (3,1%) among other less frequent causes. Conclusion : In our referral region, 72% of uveitis cases were correctly identified surpassing other studies. There was a higher rate of intermediate uveitis and lower number of panuveitis cases when compared to a previous Portuguese study. Extensive monitoring of the development of uveitis by international multicentre prospective studies could be useful for increasing practitioner awareness and diagnostic approach.

Prcis: An Ahmed glaucoma valve (AGV) tube in the ciliary sulcus (CS) is safer for the endothelium. At 4 years of follow-up, there was a significant decrease in endothelial cell count only with anterior chamber (AC) placement., Purpose: Corneal endothelium (CE) damage is one of the most feared long-term complications that can result from glaucoma drainage devices. Nonetheless, there is a lack of studies evaluating longitudinal changes in CE cells. This study aims to compare CE changes after AGV implantation in eyes with AC or CS tube placement., Materials and Methods: This was a retrospective, nonrandomized, longitudinal study of pseudophakic eyes with open-angle glaucoma and medically uncontrolled intraocular pressure that received an AGV. Eyes with additional glaucoma surgeries performed during follow-up were excluded. The main outcome measure was endothelial cell density (ECD), which was evaluated 1 year±2 months and 4 years±2 months postoperatively. The average endothelial cell size (AS) and the distance from the tube tip to the cornea were also evaluated., Results: Twenty-six eyes from 26 patients, with a mean age of 73±10 years, were included. The tube was placed in the AC in 15 eyes and in the CS in 11 eyes. The eyes with tube placement in the AC showed a significant AS increase (P=0.007) and ECD decrease (P=0.034), whereas eyes with tube placement in the CS had no significant AS (P=0.575) or ECD (P=0.445) change. In the eyes with tube placement in the AC, there was no correlation between DTC and ECD (P=0.260) or AS (P=0.428) changes., Conclusions: Tube placement in the AC seems to lead to significant CE cell loss over time, compared with tube placement in the CS., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: This study aimed to evaluate the longitudinal changes in retinal layer thickness in patients treated with hydroxychloroquine without retinal toxicity., Methods: This is a longitudinal retrospective study of patients taking hydroxychloroquine followed in a hydroxychloroquine retinal toxicity screening program of a tertiary hospital between January 2010 and April 2019. Patients who performed 2 optical coherence tomography (OCT) scans at least 1 year apart were included. All subjects with hydroxychloroquine suspected or confirmed retinal toxicity, glaucoma, retinal pathology, or poor segmented images were excluded. Spectral-domain optical coherence tomography (Spectralis HRA-OCT, Heidelberg) was used to evaluate the macular area. Automatically segmented ETDRS retinal thickness maps were compared between the first and the last OCT evaluation available. Full retina (FR), inner retina (IRL), ganglion cells (GCL), inner nuclear (INL), and outer retina (ORL) layer thicknesses were measured in the foveolar, paracentral, and peripheral area., Results: The population included 144 eyes of 144 patients. The mean interval between OCT scans was 38.1 ± 18.4 months, and the mean cumulative dose was 406.9 ± 223.9 g. Foveolar (p = 0.040, p = 0.006, and p = 0.001, respectively) and paracentral (p = 0.006, p = 0.001, and p = 0.005, respectively) FR, IRL, and GCL decreased overtime. No differences were found in INL or ORL. A very weak correlation was found between age and foveal IRL change overtime (p = 0.037; R = 0.175), as well as between the hydroxychloroquine time of use and foveal GCL variation (p = 0.032; R = 0.179)., Conclusion: Hydroxychloroquine was found to cause progressive thinning of the inner retinal layers, specifically in the GCL of the foveolar and paracentral areas, but no changes were observed in the outer retina., (© 2020 S. Karger AG, Basel.)

Purpose: Obese patients have neurodegeneration of the optic nerve demonstrated by decreased peripapillary nerve fiber layer. Whether bariatric surgery reverses this neurodegenerative process has not been explored. We aimed to evaluate the impact of bariatric surgery in the structure of the retina and optic nerve., Methods: Multicentric observational study. Obese patients scheduled for bariatric surgery were consecutively recruited and included in the study and evaluated before and 6-12 months after the intervention. The retinal structure was evaluated as retinal thickness in the different retinal layers in the foveal, perifoveal, and parafoveal regions using optical coherence tomography. Choroidal thickness and optic nerve retinal nerve fiber layer thickness were also evaluated., Results: Eighty eyes from 40 participants were included. Globally, we found a significant thickening of the retina after bariatric surgery (foveal: 273.5 (21.5) μm vs 280.0 (28.8) μm, p < 0.001; parafoveal 332.4 ± 17.8 μm vs 336.6 ± 15.9 μm, p = 0.003; perifoveal: 293.4 ± 13.8 μm vs 295.7 ± 14.9 μm; p = 0.001), whereas no significant differences were found for the ganglion cell layer, choroid, or peripapillary nerve fiber layer thickness. The retinal thickening was confined to inner retinal layers and was independent of the diabetic status of the patients. After multivariate adjustment, HbA1c variation, preoperative C-peptide, preoperative hypertension, preoperative OSA, and preoperative LDL and TG levels seem to be clinical predictors of retinal thickening., Conclusions: We found a significant thickening of the retina after bariatric surgery that was independent of the diabetic status. The thickening was confined to inner retinal layers and may represent and improve perfusion. The peripapillary nerve fiber layer remained unchanged after the surgery.

The recent findings of increased Choroidal Thickness (CT) in Keratoconus (KC) patients raised the question of whether CT could be an indicator of progressive KC. To test this hypothesis, we evaluated and compared the choroidal profile in progressive and non-progressive KC. We ran a cross-sectional observational study in 76 patients diagnosed with KC, age 14-30, to assess KC progression. Progression was defined as when at least two of the studied variables confirmed progression (Kmax, Km, PachyMin, D-Index, Astig, K2, 3 mm PCR). Included patients performed a Spectralis Optical Coherence Tomography (OCT) with enhanced depth image (EDI) technology to evaluate choroidal profile. Choroidal measurements were taken subfoveally and at 500 µm intervals from the fovea, in 7 different locations, and compared between groups. Multivariate linear regression analyses were also performed to assess the influence of CT in KC progression. Thirty-six eyes (47.4%) were classified as KC progressors. The mean subfoveal CT observed in the total sample was 382.0 (± 97.0) μm. The comparison between groups (progressive and non-progressive KC) showed no differences in the locations evaluated (mean subfoveal CT difference between groups was 2.4 μm, p = 0.915). In the multivariate analysis CT seems not be influenced by KC progression (B = 6.72 μm, 95% CI - 40.09 to 53.53, p = 0.775). Assessment of choroidal profile does not appear to be a useful tool to differentiate progressive and non-progressive KC. Further research is needed in order to better understand the role of choroid in KC.

Background: To characterize the phenotype and genotype of a syndrome associating posterior microphthalmos (PM), retinitis pigmentosa (RP), foveoschisis, and foveal hypoplasia (FH) in a consanguineous Portuguese family., Materials and Methods: Three siblings were studied and underwent comprehensive eye examinations for best-corrected visual acuity, axial length, refractive error, B-mode ultrasound, electroretinography, retinography, fluorescein angiography (FA), kinetic visual field (VF), and optical coherence tomography (OCT). Molecular analysis was performed by Sanger sequencing of the entire coding region of the MFRP gene., Results: All members presented nyctalopia, decreased visual acuity, and constriction of the VF, as well as bilateral shortening of the posterior ocular segment and normal anterior segment dimensions. The fundoscopy and ERG results were compatible with RP. Macular OCT analysis revealed schisis of the outer retinal layer, FH, as well as retinal and choroidal folds. We identified a homozygous mutation in intron 9 of the membrane frizzled-related protein (MFRP) gene (c.1124 + 1 G > A)., Conclusions: Our study shows a family with PM and RP due to a mutation in the MFRP gene. The relationship has previously been proven, but this specific mutation has never been described. These gene mutations show wide phenotypic variability, being evident in the presence of foveoschisis, retinal and choroidal folds, and FH, other than PM and RP.

Introduction: COVID-19 is caused by the coronavirus SARS-CoV-2. Ocular manifestations have been reported including conjunctivitis and retinal changes. Therefore, it is of the utmost importance to clarify eye involvement in COVID-19 in order to help with its diagnosis and to further prevent its transmission. The purpose of this review is to describe the structure and transmission of SARS-CoV-2, reported ocular findings and protection strategies for ophthalmologists., Material and Methods: Literature search on PubMed for relevant articles using the keywords 'COVID-19', 'coronavirus', and 'SARS-CoV-2' in conjunction with 'ophthalmology' and 'eye'. Moreover, official recommendations of ophthalmological societies were reviewed., Results: Although the conjunctiva is directly exposed to extraocular pathogens, and the mucosa of the ocular surface and upper respiratory tract are connected by the nasolacrimal duct, the eye is rarely involved in human SARS-CoV-2 infection and the SARS-CoV-2 RNA positive rate by RT-PCR test in tears and conjunctival secretions from patients with COVID-19 is also extremely low., Discussion: The eye can be affected by SARS-CoV-2, which is supported by some reports of conjunctivitis and retinal changes, but its role in the spread of the disease is still unknown., Conclusion: Given the current scarce evidence, more research is needed to clarify the relationship between SARS-CoV-2 and the eye.

Introduction: Unexplained visual loss after removal of silicone oil from the eye has been described. The purpose of this study is to determine the incidence of unexplained loss of visual acuity after SO removal and to provide possible explanations for this phenomenon., Methods: This retrospective study included patients that underwent vitreoretinal surgery, at Centro Hospitalar São João, between January of 2012 and October of 2018. Inclusion criterion was vitreoretinal surgery in which the chosen endotamponade was SO, followed by removal of SO and exchange with balanced salt solution (BSS) or air. After SO removal, patients with documented loss of best corrected visual acuity (BCVA) on two or more Snellen lines were analyzed and patients in which the cause of the visual loss was identified, namely OHT (intraocular pressure > 21 mmHg), retinal re-detachment, glaucoma, retinal proliferative membrane formation, or corneal decompensation, were excluded. All patients with unexplained visual loss underwent spectral domain optical coherence tomography (SD-OCT) to exclude causes of visual reduction such as cystoid macular edema, epiretinal membrane, or ellipsoid/interdigitation zone disruption. A p value less than 0.05 was considered statistically significant., Results: A total of 46 eyes underwent SO tamponade and SO removal during the study period. In 34.8% of the cases (n = 16) there was visual acuity loss in at least two Snellen lines. Of 46 eyes, 23.9% (n = 11) showed vision loss due to known secondary causes. Unexplained loss of visual acuity after SO removal occurred in 10.9% of cases. OHT during silicone endotamponade (p = 0.046) and silicone emulsification (p = 0.001) were identified as factors associated with unexplained visual loss after SO removal., Conclusion: Unexplained loss of visual acuity after SO removal occurred in 10.9% of cases. OHT during silicone endotamponade and SO emulsification were identified as important factors in the ethology of this phenomenon.

Purpose: To compare corneal biomechanical changes after uneventful phacoemulsification cataract surgery between type 2 diabetic (DM) and nondiabetic patients., Methods: Forty-four diabetic (44 eyes) and 44 (44 eyes) age and sex-matched non-DM controls with age-related cataract were enrolled in this prospective observational study. Corneal hysteresis (CH), corneal resistance factor (CRF), and corneal-compensated intraocular pressure (IOPcc) were evaluated by using the ocular response analyzer; central corneal thickness was recorded by using the Pentacam HR. Patients were evaluated preoperatively and 1 and 6 months after surgery., Results: In the DM group, CH was observed to be significantly lower than preoperative value (9.8 ± 1.5 mm Hg) at 1 month (9.4 ± 1.2 mm Hg, P = 0.040), but not at the 6-month follow-up (9.6 ± 1.6 mm Hg, P = 0.437),whereas it did not change significantly in the non-DM group (preoperative 9.8 ± 1.3 mm Hg vs. 1 month 9.6 ± 1.1 mm Hg vs. 6 months 10.1 ± 1.1 mm Hg, P > 0.05). CRF was significantly lower than the preoperative values at 1 and 6 months in both groups (P ≤ 0.001). Postoperative CH change was significantly associated with preoperative CH (P < 0.001), preoperative IOPcc (P = 0.004), and IOPcc change (P < 0.001), whereas CRF change was only correlated with preoperative CRF (P < 0.001). There was a significant postoperative IOPcc reduction 6 months after surgery (P < 0.001) in both DM and non-DM groups; however, central corneal thickness was not found to significantly change in the postoperative period (P > 0.05)., Conclusions: This study showed that phacoemulsification causes a significant and persistent decrease in intraocular pressure and CRF in both groups, whereas CH recovered to preoperative values, although more slowly in patients with diabetes.

Introduction: Recently, vitamin D has gained importance as a diabetes risk modifier. Our aim was to assess the association between serum vitamin D levels and the prevalence of diabetic retinopathy in patients with type 1 diabetes., Material and Methods: Retrospective review of a population of patients with type 1 diabetes followed in a Portuguese tertiary center. Patients were included if they had an ophthalmological evaluation and a serum 25-hydroxyvitamin D level determination within the same year. Logistic regression analysis was used to adjust for possible confounders., Results: We included 182 patients (47% male), and 57% (n = 103) had signs of diabetic retinopathy. We found a significant association between lower circulating levels of 25-hydroxyvitamin D levels and a greater prevalence of diabetic retinopathy after adjusting for confounders (duration of diabetes, estimated glomerular filtration rate, age, sex, metabolic control, season, dyslipidemia and hypertension) (OR = 0.94; 95% CI 0.90 - 0.99, p = 0.023). Longer duration of diabetes and worse metabolic control also remained associated with diabetic retinopathy in the multivariate analysis (OR = 1.20; 95% CI 1.13 - 1.27, p < 0.001 and OR = 4.13; 95% CI 1.34 - 12.7, p = 0.013, respectively)., Conclusion: Lower levels of vitamin D were associated with an increased prevalence of diabetic retinopathy in patients with type 1 diabetes, after adjusting for possible confounders. Future controlled studies may elucidate the molecular routes for this association as well as the role of supplementation in the prevention of diabetes microvascular complications.

Aim: To evaluate inter-device agreement of anterior keratometry obtained by the IOLMaster ® 500 and Pentacam ® HR in type 2 diabetic and non-diabetic patients., Methods: Corneal measurements were sequentially performed in 60 diabetes mellitus (DM) and 48 age and sex-matched controls undergoing cataract surgery. Variables recorded included flat and steep keratometry, mean keratometry (K m ), astigmatism magnitude, axis location, J0 and J45 components. Bland-Altman plots and intraclass correlation coefficients were used for examination of agreement. Subgroup analyses were performed for astigmatism magnitude, diabetes duration, hemoglobin A1c (HbA1c) levels and diabetic retinopathy (DR) stage., Results: Agreement for K m and astigmatism magnitude were considered good and moderate, with 95% limits of agreement (LoA) of -1.09 to 1.23 diopters (D) and -0.83 to 0.86 D in DM group, respectively; and -0.59 to 0.72 D and -0.98 to 0.75 D in non-DM group, respectively. In contrast, the 95% LoA for corneal axis exceeded the clinically relevant margins in both groups. In the total sample, only 41 eyes (38%) had a smaller than 5-degree difference. Diabetes duration, HbA1c levels and DR stage were not found to significantly affect agreement. Logistic regression showed that higher corneal power ( P =0.021) and astigmatism magnitude ( P =0.011) were associated with a decreased risk of having a difference in axis location greater than 10-degrees., Conclusion: In both groups, IOLMaster and Pentacam agree well for corneal power and moderately for astigmatism. However, axis location disagreement is frequent in eyes with flatter corneas and small amounts of astigmatism., (International Journal of Ophthalmology Press.)

Diabetic macular edema (DME) is the most common cause of visual loss in patients with diabetes. Antivascular endothelial growth factors (anti-VEGF) agents are first-line therapy for DME. Nevertheless, up to 60% of patients (depending on the anti-VEGF drug used) have an inadequate response to anti-VEGF treatment. Several cytokines are increased in aqueous humor of patients with DME. Differences in response to treatment may be related to baseline cytokine levels. Intravitreal corticosteroids may be used as an alternative to anti-VEGF agents. Steroids have a different pharmacological profile and act on different pathophysiologic mechanisms. Their effect on aqueous humor cytokines is different from the effect of anti-VEGF therapy. This review highlights the major cytokines involved in DME and evaluates whether baseline cytokine levels could be predictors of response to treatment in DME. SIGNIFICANCE STATEMENT: Antivascular endothelial growth factor (anti-VEGF) agents are efficient as diabetic macular edema (DME) treatment. However, in some cases, DME fails to respond to anti-VEGF intravitreal injections. Changes in cytokine levels after treatment supported the idea that other cytokines than VEGF are implicated in DME pathogenesis and could be predictors of response to anti-VEGF treatment or corticosteroids allowing targeted and individualized therapy guided by cytokine levels., Competing Interests: Conflict of interest: None of the authors has conflict of interest with this submission., (Copyright © 2020 by The American Society for Pharmacology and Experimental Therapeutics.)

Purpose: To determine the incidence of intraoperative complications of phacoemulsification cataract surgery in a teaching hospital and to compare the intraoperative complication rate between resident trainees and staff ophthalmologists, to ascertain the overall rate of intraocular pressure (IOP) elevation on postoperative day 1 (POD1), and, again, to compare this value between resident trainees and staff surgeons., Setting: Centro Hospitalar São João, Oporto, Portugal., Design: Retrospective study., Methods: This study included eyes submitted for phacoemulsification cataract surgery from January 1, 2017, to December 31, 2017. There were no exclusion criteria. Data collected included the type of surgeon, level of resident training, case complexity, and intraoperative complications. From all noncomplicated surgeries, preoperative IOP and IOP-POD1 were collected., Results: A total of 2937 surgeries were included; 25.6% were performed by resident trainees. The complication rate was 6.3% with resident trainees and statistically significantly lower (3.3%) in staff surgeons. The complication rate of resident trainees did not differ according to their level of residency. There was a significantly increased incidence of IOP-POD1 elevation in the resident trainee group compared with the staff surgeon group. Surgeries performed by resident trainees, a preexisting history of glaucoma, and pseudoexfoliation were significant risk factors for IOP elevation., Conclusions: To our knowledge, this is the first European study comparing the incidence of intraoperative and postoperative complications in cataract surgery performed by resident trainees and staff surgeons. We report a significantly higher rate of intraoperative complications and IOP elevation on POD1 in resident trainees vs staff surgeons.

Purpose: We investigated the characteristics, prognosis, and clinical outcome of the Charles Bonnet syndrome (CBS) in patients with neovascular age-related macular degeneration (AMD)., Methods: Five hundred psychiatrically healthy patients with neovascular AMD were screened for CBS. The individuals that fulfilled the inclusion criteria were systematically interviewed using a structured questionnaire that covered the impact, prognosis, risk factors, phenomenology, symptoms, and knowledge about the syndrome. A control group of 45 patients was used for comparison. Demographic data, current medication, and ocular risk factors were collected in all patients., Results: Forty-five patients with CBS were identified. The majority of patients reported images that consisted of colored (62%) animals (44%) or faces (42%) that lasted for seconds (53%). Most patients reported a self-limited disease with a median duration of symptoms between 9 and 11.5 months, with only 7% knowing about CBS at symptom onset. The degree of visual deficit did not predict the characteristics, complexity, frequency, duration, or impact of visual hallucinations. One-third of patients reported negative outcome, which was associated with shorter duration of CBS (p = 0.023), fear-inducing images (p < 0.001), and impact on daily activities (p = 0.015)., Conclusion: The prevalence of CBS in neovascular AMD patients is high and clinically relevant. Patients with recent onset of visual hallucinations and describing fear-inducing images are at greater risk for negative outcome. Periodic screening may minimize the negative consequences of this disease., (© 2020 S. Karger AG, Basel.)

Purpose: To analyze and compare choroidal thickness between keratoconus (KC) patients and age-matched non-KC subjects., Methods: A cross-sectional, case-control study. One hundred and thirty-four keratoconic eyes and 78 control eyes, from individuals aged from 12 to 30 years old, were studied. Patients with KC followed in Corneal Department of Centro Hospitalar São João, Porto, Portugal, were identified and consecutively included between December 2017 and February 2018. A spectral-domain optical coherence tomography (OCT) using depth enhanced imaging was performed, and choroidal thickness in the center of the fovea and at 500  μ m intervals along a horizontal section was measured and compared., Results: The statistical analysis showed that keratoconic eyes present a thicker choroid in every measured location ( p < 0.05). Mean subfoveal choroidal thickness (SFCT) values obtained were 375.86 ± 89.29 and 322.91 ± 85.14 in keratoconus and control groups, respectively ( p < 0.001). In a multivariate analysis, SFCT was significantly associated with spherical equivalent ( p =0.004) and the presence of keratoconus ( p < 0.001), but not with age ( p =0.167), gender ( p =0.579), or best-corrected visual acuity ( p =0.178). In a "fixed model," keratoconus patients were found to have a 67.55  μ m (95% CI 36.61-98.49) thicker subfoveal choroid compared to controls., Conclusion: Keratoconus patients seem to have a thicker choroid than healthy individuals. The exact pathophysiological mechanism resulting in a thicker choroid in KC patients is not known, but it could possibly be associated with inflammatory choroidal mechanisms., Competing Interests: The authors declare no conflicts of interest regarding the publication of this paper., (Copyright © 2019 João Pinheiro-Costa et al.)

Purpose: To estimate the prevalence of diabetic retinopathy /(DR) in a Portuguese population with type 1 diabetes (T1DM)., Methods: Retrospective review of all patients with T1DM, whose reference center was Centro Hospitalar de São João, a tertiary center in Portugal, who were diagnosed between 1990 and 2018. DR was classified based on fundus examination in medical records as (0) no evidence of retinopathy, (1) mild non-proliferative retinopathy (NPDR); (2) moderate to severe non-proliferative retinopathy (NPDR) and (3) proliferative retinopathy (PDR). Patients were classified according to the eye with a worse retinopathy stage. Follow-up was considered as time between the diagnosis of diabetes and the last funduscopic evaluation., Results: 233 patients met the inclusion and exclusion criteria. The prevalence of any DR at less than 5, 10, 15, 20 and more than 20-years of DM was 1.8%, 10.4%, 34.8%, 54.1% and 71.2% respectively. The overall prevalence of DR was 43.3% (n = 101). At the last observation, 43 patients (18.5%) had mild NPDR, 34 patients (14.6%) had moderate to severe NPDR and 24 patients (10.3%) had PDR. The longer the disease duration, the higher the number of patients with DR., Conclusion: The prevalence of DR in our cohort was 34.8% after 15 years of systemic disease and 54.1% after 20 years, which is lower than what has been reported in the literature. Further multicentric prospective studies, are needed to clarify changes in the epidemiology of DR in type 1 diabetics., (Copyright © 2019 Elsevier B.V. All rights reserved.)

In January 2013, a 54-year-old pseudophakic woman was referred to the ocular inflammation clinic with a diagnosis of panuveitis, already on oral cyclosporine (100 mg/day) since 2010. She was a monocular patient, with the left eye eviscerated due to trauma and secondary endophthalmitis in 1995. She had experienced similar recurring episodes between 2010 and 2013. Examination revealed best-corrected visual acuity (BCVA) of 20/200. The anterior segment revealed the presence of flare and absence of cells. Intraocular pressure (IOP) was 11 mmHg. Fundus examination revealed the presence of anterior vitreous organization, exuberant cystoid macular edema (CME) and peripheral Dalen-Fuchs nodules with no activity. Systemic workup showed no changes, and a presumptive diagnosis of sympathetic ophthalmia was made. Initial therapy included topical and systemic corticosteroids. Cyclosporine was replaced with methotrexate (20 mg/week) due to nephrotoxicity. There was marked improvement in anterior inflammation and vitreous organization, but the ME remained exuberant (central ring thickness of 0.7 mm). Over the subsequent 4 years of follow-up, the patient received two intravitreous injections of a dexamethasone extended-release implant (Ozurdex ® ), with normalization of macular thickness and improvement in visual acuity (VA), but ME recurred around 6 months post-injection. During this period, the patient also underwent three sub-tenon injections of triamcinolone, with marked improvement of ME, but ME recurred between 4 and 6 months after treatment. She was thus recommended for treatment with a fluocinolone extended-release implant (ILUVIEN ® ). Post-implantation (3-year follow-up), she had controlled anterior inflammation without ME (central macular thickness of 248 µm). This case suggests that sustained control of inflammation enables better control of ME, and benefits may persist even after cessation of the direct anti-inflammatory effect of the implant. FUNDING: Alimera Sciences Ltd funded the Rapid Service Fees.

Purpose: Age at diagnosis of type 1 diabetes (DM1) has been implied as an important factor associated with the development of the microvascular complications. Our aim was to identify factors associated with prevalent diabetic retinopathy (DR) and proliferative diabetic retinopathy (PDR) in DM1 people with early and late-onset., Methods: We reviewed medical records from all DM1 people from the reference area of a tertiary center (about 340,000 persons). Univariate and multivariate logistic regression were used to assess the relationship between potential risk factors (sociodemographic, diabetes-related, co-morbidities, and laboratory parameters) and prevalent DR/PDR. We performed an analysis comparing patients diagnosed before (early-onset) and after (late-onset) 18 years of age., Results: We included 140 patients in early-onset DM1 group and 169 in late-onset DM1 group. Longer duration of diabetes and HbA1c remained associated with prevalent DR in both groups after adjusting for potential risk factors. Nephropathy was associated with prevalent DR in the late-onset group but not in the early-onset group. Peripheral neuropathy remained associated with prevalent PDR when modeled together in the multivariate model. High BMI demonstrated a significative association with PDR in early but not in the late-onset DM1 group., Conclusions: Although previous reports suggest that age at DM1 diagnosis may have a role in DR prevalence, the risk factors for DR in early and late-onset DM1 were similar for both groups. Duration of disease and lifelong metabolic control were the major predictors for DR in both groups. Nephropathy was associated with DR in patients with late-onset disease. Neuropathy was associated with PDR occurrence in both groups. BMI was associated with PDR early-onset DM1 group.

Purpose: To evaluate the efficacy of the "fogging test," performed with a +2 diopters (D) lens, in the exclusion of clinically significant hyperopia in school-aged children., Methods: We studied 54 children between 5 and 11 years of age, with 10/10 best-corrected bilateral visual acuity (VA) without significant degree of correction. VA was assessed in each eye with a "bilateral" +2 D sphere over-refraction followed by cycloplegic retinoscopy. The capacity of the test to detect hyperopia of ≥+2 D and ≥+1.5 D was evaluated by examining the respective receiver operating characteristic (ROC) curves and sensitivity and specificity values for different cutoff values of VA., Results: For the detection of hyperopia ≥+2 D, the area under the ROC curve (AUC) was 0.955 ( p ≤ 0.001). The VA cutoff with best discriminative capacity was ≥5/10, with a sensitivity of 100%, specificity of 79%, positive predictive value (PPV) of 57%, and negative predictive value (NPV) of 100%. In respect of ≥+1.5 D hyperopia, the test capacity was lower (AUC = 0.832; p ≤ 0.001). The best VA cutoff was also of ≥5/10, with a PPV of 81% and a NPV of 85%., Conclusion: The accuracy of the test was high for the evaluation of ≥+2 D hyperopia but lower for ≥+1.5 D hyperopia. For the detection of ≥+2 D hyperopia, the VA cutoff of <5/10 may permit the exclusion of clinically significant hyperopia in selected children, without the need for cycloplegia. For the same cutoff, the PPV was low, meaning that in children with ≥5/10 VA cycloplegic refraction remains obligatory., Competing Interests: The authors declare that there are no conflicts of interest.

Background: Idiopathic intracranial hypertension (IIH) is a serious condition that is frequently associated with irreversibly vision loss, having a higher incidence among obese women. Our aims were to screen subclinical IIH in obese patients scheduled to bariatric surgery using peripapillary retinal nerve fiber layer (RNFL) thickness and to evaluate if the findings demand the possible need of a preoperative evaluation in this population., Methods: This study included 111 eyes from 36 obese patients (86% female, body mass index > 35 kg/m 2 ) scheduled to bariatric surgery and 20 non-obese (body mass index < 25 kg/m 2 ) age-matched controls. We measured sectorial and mean RNFL thickness in a 3.5-mm-diameter circular scan centered on the optic nerve head, using optical coherence tomography (Heidelberg Spectralis SD-OCT) in all participants. Multivariate linear regression was used for adjustments., Results: No patient had subclinical IIH corresponding to increased RNFL thickness. However, in obese individuals, global peripapillary RNFL was thinner than in controls (104 ± 6 μm versus 99 ± 12 μm, p = 0.005). Overall, RNFL thickness was superior in the control group for all sectors. The differences reached significance for the nasal, temporal, superior temporal, and inferior temporal sectors. These differences remained even after adjusting for possible confounders (hypertension, dyslipidemia, diabetes, age, sleep apnea syndrome, and sex)., Conclusions: Routine screening asymptomatic obese patients undergoing bariatric surgery for IIH using RNFL thickness was not clinically relevant in our study. However, we found that severe obesity is associated with neurodegeneration independently of the other components of the metabolic syndrome, what may justify future investigation on the need of monitoring these patients.

Purpose: To compare intraocular pressure (IOP) and anterior segment (AS) morphometry changes after uneventful phacoemulsification between nonglaucomatous eyes with open-angles from patients with and without type 2 diabetes mellitus (DM) and determine which factors may predict greater IOP-lowering effect., Methods: Forty-five diabetic (45 eyes) and 44 (44 eyes) age- and sex-matched non-DM patients with age-related cataract were enrolled in this prospective observational study. Goldmann applanation tonometry and AS Scheimpflug tomography (Pentacam® HR) were performed preoperatively and at 1- and 6-month follow-up. Linear regression analysis was performed to evaluate the clinical variables related to postoperative IOP changes at 6 months., Results: There was a significant postoperative IOP reduction 6 months after surgery ( p < 0.001) by an average of 2.9 ± 2.9 mmHg (15.5%) and 2.4 ± 2.8 mmHg (13.0%) in the DM group and non-DM groups ( p = 0.410), respectively. All AS parameters (anterior chamber depth, volume, and angle) increased significantly postoperatively ( p < 0.001). Multivariate linear regression analysis showed that higher preoperative IOP was significantly associated with IOP reduction at 6-month follow-up ( p < 0.05)., Conclusion: Nonglaucomatous eyes with open-angles from both type 2 diabetic and nondiabetic patients experienced similar AS changes and IOP reductions following uneventful phacoemulsification, and this IOP-lowering effect was strongly correlated with preoperative IOP.

Purpose: To analyze and compare corneal structural and biomechanical properties, characterized by corneal hysteresis (CH) and resistance factor (CRF), between patients with and without type 2 diabetes mellitus (DM), and determine the main ocular variables that influence them., Methods: Sixty diabetic and 48 age- and sex-matched non-DM patients were enrolled in this cross-sectional study. The DM group was analyzed according to DM duration (

Purpose: To report the clinical (anatomic and functional) and genetic findings of Wagner Syndrome (WS) in a Portuguese family., Methods: Nine members of the family agreed to be examined. All had complete clinical eye examinations. The proband and selected patients underwent color fundus photography, spectral domain optical coherence tomography (SD-OCT), automatic static white-on-white computerized perimetry, and electrophysiology assessment (flash ERG, multifocal(mf) ERG and dark adaptometry). A pedigree was constructed based on interviews with known affected subjects. Genomic DNA samples derived from venous blood were collected from all affected family members examined., Results: Twenty-eight family members are affected. This family has the typical features of Wagner Syndrome, namely an empty vitreous cavity with veils, mild myopia and cataract. Four examined patients underwent vitreoretinal surgery due to abnormal peripheral vitreoretinal adhesions with peripheral retinal traction (n = 3). Retinal detachment was observed in 5 of the examined subjects. Four of them occurred between the ages of 5 and 15 years. Chorioretinal atrophy is also a frequent finding which results in moderate to severe visual field and advanced rod-cone dystrophy from younger ages, also confirmed by absence of scotopic function on dark adaptation. The macular dysfunction on mfERG was profound and of early onset. A heterozygous mutation in intron 7 of the VCAN gene (c.4004-1G > A) was found., Conclusions: We described a rare autosomal dominant vitreoretinopathy with near complete penetrance in a Portuguese family. Abnormal peripheral vitreoretinal adhesions, retinal detachment and chorioretinal atrophy are present in most of the examined individuals at young ages. Early onset of advanced visual field and electrophysiologic abnormalities were observed in this family. We also added relevant information to the literature by reporting our experience in surgical management of Wagner Syndrome patients with, and at risk of, retinal detachment.

Objectives: To study the microbial profile, antibiotic susceptibility pattern, risk factors, therapeutic trends, and clinical outcomes for microbial keratitis (MK) in a tertiary health care center., Methods: All cases with suspected bacterial keratitis that were followed at consultation from September 2007 to August 2015 were included. Microbial cultures were obtained and patients were managed following an internal protocol., Results: Two hundred thirty-five patients were included, with a mean age of 50.01±20.73 years. We obtained a 38.4% culture-positive rate, with higher proportion of gram-positives (70.8%). The commonest agents were Staphylococcus aureus (23.1%), Corynebacterium macginleyi (20.0%), and Pseudomonas aeruginosa (13.8%). Fluoroquinolone susceptibility was 97%, with no trend toward its decrease. A total of 95.7% had local risk factors, being trauma and contact lens wear the most common (28.9% each), with different age and pathogens distributions (P<0.001). Topical fluoroquinolones were first-line treatment in 99%, in association with aminoglycosides in 81.6%. Good initial response was registered in 95.9%, but 4.1% needed to step up treatment to fortified antibiotics, mainly if Pseudomonas (P=0.021). Good outcome was achieved in 81.8%, negatively affected by exposure and herpetic keratitis (P<0.001), central location (P=0.01), presence of Tyndall (P<0.001), corneal edema (P<0.001), and worse initial best-corrected visual acuity (P<0.001), but not Pseudomonas isolate (P=0.724)., Conclusions: Gram-positives are the most frequent pathogens and shifting trends in the isolate distribution or emergence of resistant strains were not demonstrated. The susceptibility to first-line antibiotic agents remained high. We suggest a more aggressive approach to P. aeruginosa cases or MK presenting with poor outcome variables.

Purpose: To evaluate functional and anatomical outcomes after aflibercept in patients with diabetic macular edema (DME) with poor response to bevacizumab., Methods: We retrospectively reviewed patients with DME recalcitrant to bevacizumab who were switched to aflibercept between January and December 2015. All patients had a minimal follow-up of three months before the conversion and underwent at least three injections of bevacizumab. Functional outcome consisted in best corrected visual acuity (VA). Anatomical outcomes were demonstrated through central macular thickness (CMT) measured by optical coherence tomography., Results: Forty-nine eyes of 34 subjects were reviewed. Mean VA improved from 0.55 ± 0.32 logMAR to 0.46 ± 0.33 logMAR (p = 0.038). Mean CMT decreased from 473 ± 146 μm to 349 ± 85 μm (p < 0.001). Twelve eyes (24%) demonstrated absence of macular edema after aflibercept. Previous bevacizumab exposure did not correlate with different outcomes. The variation of VA in response to aflibercept was significantly superior in the group with poorer VA before the switch (mean variation of -0.097 ± 0.21 logMAR) when compared to eyes with VA < 0.4 logMAR (mean variation of +0.019 ± 0.090 logMAR; p = 0.036). The same scenario was verified for anatomical outcomes as eyes with poor vision before the switch (≥0.4 logMAR) achieved superior reduction in CMT in response to aflibercept (mean CMT variation of -157 ± 171 μm versus -49.5 ± 39.9 μm; p < 0.01). Pre-switch CMT was a predictor of CMT reduction after switching (B = -0.945; confidence interval 95% -1.1; -0.76; p < 0.001)., Conclusions: Conversion to aflibercept for persistent DME resulted in functional and anatomical improvements and these outcomes were not influenced by previous bevacizumab exposure. Pre-switch CMT was a predictor of anatomical changes after aflibercept.

Purpose: To report a rare case of exudative maculopathy in a patient with facioscapulohumeral muscular dystrophy (FSHD), and its management., Methods: Observational case report., Results: A 62-year-old man with genetically confirmed FSHD was referred to our department complaining of decreased visual acuity in his left eye. At presentation, right eye examination was unremarkable and best-corrected visual acuity (BCVA) was 20/20. Left eye BCVA was 20/100 and it presented a dense cataract with the evidence of macular lipid exudation. Cataract surgery combined with intravitreal bevacizumab improved BCVA to 20/20. Postoperative fundus examination disclosed focal macular retinal microvascular dilations with lipid exudation inferotemporal to the fovea. Fluorescein angiography highlighted these macular telangiectatic abnormalities but no peripheral lesions were detected. Spectral domain optical coherence tomography (SD-OCT) showed mild temporal retinal thickening, sparing the fovea. A diagnosis of exudative maculopathy due to macular telangiectasia secondary to FSHD was established. One year later, his left eye vision dropped to 20/32 and macular SD-OCT showed an aggravation of the intraretinal fluid and exudation. He was then submitted to a second intravitreal injection of bevacizumab followed by one angio-guided focal laser photocoagulation session, with a significant improvement. Twelve months later, his BCVA remained 20/20 on both eyes with no recurrence of exudation., Conclusion: The present work shows that in cases of visual-threatening macular exudation, intravitreal anti-vascular endothelial growth factor injections combined with focal laser photocoagulation may be a safe and effective treatment. This article also highlights that all FSHD patients should be screened for asymptomatic retinal vascular disorders.

Purpose: We investigate the prevalence of the Charles Bonnet syndrome (CBS) in patients with neovascular age-related macular degeneration (AMD) and analyze the role of oral proton pump inhibitors (PPIs) and other potential risk factors., Methods: A total of 510 consecutive patients with neovascular-AMD followed at a single tertiary center in Portugal were screened for CBS. Using a structured questionnaire, psychiatrically healthy individuals were interviewed systematically and divided into a CBS group and a non-CBS group. Demographic data, current medication, and ocular risk factors were collected and compared between the two groups., Results: A total of 500 patients met the inclusion criteria and 471 with complete data were included in the final analysis. The prevalence of CBS was 9.0% (45/500). Using a binary logistic regression model, correlations were found between older age (P = 0.002), PPI intake (P = 0.022), poor visual acuity (P = 0.004), and development of CBS. PPIs doubled the risk of CBS from 7% (20/304) to 15% (25/167), with an odds ratio of 2.154. The increased risk for visual hallucinations caused by PPIs was independent of age (P = 0.598) and visual acuity (P = 0.739)., Conclusions: The prevalence of CBS in neovascular-AMD patients is high and mainly affects older individuals with poor visual acuity. PPIs seem to increase the risk of development of hallucinations independently of the degree of visual loss.

Purpose: Characterized by a progressive onset of gait disturbances, dementia, and urinary incontinence, idiopathic normal pressure hydrocephalus (iNPH) is considered a rare, but under-diagnosed disease. Non-invasive diagnostic markers are still insufficient to enable the diagnosis of iNPH with certainty and yet early treatment with ventriculoperitoneal (VP) shunting can reverse symptoms and stop disease progression. Vascular circulation abnormalities in iNPH may be reflected by changes in subfoveal and peripapillary choroidal thickness (PPChT). This study uses spectral domain-optical coherence tomography (SD-OCT)-based measures of retinal and choroidal thickness to test this hypothesis and to assess ophthalmological non-invasive markers for iNPH., Methods: Twelve patients who displayed neurological and neuroradiological characteristics of iNPH were subject to a full ophthalmological examination including enhanced depth imaging (EDI) SD-OCT. Of the 12 included iNPH patients, 6 had undergone VP shunting with beneficial outcome. Parameters studied with EDI SD-OCT were macular retinal thickness (MT), subfoveal choroidal thickness (SFChT), retinal nerve fiber layer thickness (RNFL), and PPChT. Results were compared with 13 healthy, age-matched controls., Results: Macular thickness and RNFL and MT values of iNPH patients did not reflect atrophy. Non-shunted iNPH patients showed significantly lowered median PPChT and SFChT values compared to healthy controls. Shunted iNPH patients displayed a significantly higher median PPChT and SFChT compared to non-shunted iNPH patients. SFChT and PPChT values in shunted patients were not significantly different to values in healthy controls., Conclusion: Although limited by small sample size, SD-OCT measures in this study reveal significant changes of choroidal thickness and support the hypothesis of choroidal susceptibility to hemodynamic alterations in iNPH. Non-shunted iNPH patients in this study show choroidal thinning in combination with normal RNFL and MT values. In addition to neurological and neuroradiological exams, this pattern may aid in the challenging diagnosis of iNPH.

Purpose: To report the success rate of children undergoing probing for congenital nasolacrimal duct obstruction (CNLDO) and the factors relating to the failure of the procedure., Methods: This retrospective case series included 88 eyes of 62 patients, aged 1 to 138 months, who underwent probing between January 2008 and December 2014 in the Pediatric Ophthalmology Unit of Centro Hospitalar São João. The procedure was performed in the operating room under general anesthesia. Surgical success was defined as successful lacrimal irrigation in-traoperatively and resolution of epiphora at the follow-up visit 1 month after surgery., Results: The overall success rate after first probing was 77.3% (68 of 88 eyes). No differences were found regarding age (P = .546), gender (P = .740), surgical experience (P = .611), or laterality (P = .328) between children who were cured and not cured. The surgical success rate decreased in children older than 4 years, although not to a statistically significant degree (P = .190). Surgical success after second probing was 85.7% (12 of 14 eyes), and the median interval between the two procedures was 3 months (range: 2 to 54 months). In 30% (7 of 20 eyes, 4 of 13 patients) of children with persistent obstruction, otorhinolaryngology evaluation evinced an adenoid hypertrophy requiring surgical correction., Conclusions: The success rate of nasolacrimal probing for CNLDO was not related to age, gender, laterality, or the surgeon's experience. Otorhinolaryngology evaluation is recommended for unresponsive patients. [J Pediatr Ophthalmol Strabismus. 2017;54(2):123-127.]., (Copyright 2017, SLACK Incorporated.)

Purpose: To evaluate choroidal morphology and thickness at the posterior pole of individuals affected by multisystemic autoimmune diseases and without known ophthalmologic manifestations., Methods: Retrospective cross-sectional study including 75 patients with autoimmune diseases (divided according to their specific disease) and 80 healthy controls. A spectral-domain optical coherence tomography using enhanced depth imaging was performed and choroidal thickness was measured in the center of fovea and at 500 μm intervals along a horizontal section., Results: Lupus patients presented a thicker subfoveal choroid than controls (408.624 vs. 356.536, P < 0.001) and in all the other measurements (P < 0.001 to P = 0.003). Rheumatoid arthritis and other autoimmune diseases had an overall thinner choroid than controls (297.867 vs. 356.536 subfoveally, P = 0.004; P = 0.005-0.019 in other measurements). Results were adjusted for the covariates age (P = 0.007), spherical equivalent (P < 0.001), and systemic steroids dose (P = 0.004). Hypertension (P = 0.102), diabetes mellitus (P = 0.672), time since the beginning of therapy with hydroxychloroquine (P = 0.104) and its cumulative dose (P = 0.307), or use of other immunosuppressives (P = 0.281) had no influence on the mean choroidal thickness. No morphologic abnormalities were found., Conclusion: The choroid may be subclinically involved in autoimmune diseases. However, the choroidal response seems to differ depending on the autoimmune disease. Infiltrative mechanisms specific for lupus may justify the thickened choroid found in these patients.

Introduction: To evaluate the safety and impact on visual acuity, retinal and choroidal morphology of simultaneous cataract surgery and intravitreal anti-vascular endothelial growth factor on patients with visually significant cataracts and previously treated exudative age-related macular degeneration., Material and Methods: Prospective study, which included 21 eyes of 20 patients with exudative age-related macular degeneration submitted to simultaneous phacoemulsification and intravitreal ranibizumab or bevacizumab. The patients were followed for 12 months after surgery using a pro re nata strategy. Visual acuity, foveal and choroidal thickness changes were evaluated 1, 6 and 12 months post-operatively., Results: There was a statistically significant increase in mean visual acuity at one (13.4 letters, p < 0.05), six (11.5 letters, p < 0.05) and twelve months (11.3 letters, p < 0.05) without significant changes in retinal or choroidal morphology. At 12 months, 86% of eyes were able to maintain visual acuity improvement. There were no significant differences between the two anti-vascular endothelial growth factor drugs and no complications developed during follow-up., Discussion: Simultaneous phacoemulsification and intravitreal anti- vascular endothelial growth factor is safe and allows improvement in visual acuity in patients with visually significant cataracts and exudative age-related macular degeneration. Visual acuity gains were maintained with a pro re nata strategy showing that in this subset of patients, phacoemulsification may be beneficial., Conclusion: Cataract surgery and simultaneous anti-vascular endothelial growth factor therapy improves visual acuity in patients with exudative age-related macular degeneration.

Purpose: To evaluate the predictive factors of long-term visual outcomes in neovascular age-related macular degeneration (nAMD) treated with antivascular endothelial growth factor (anti-VEGF) agents., Methods: Unicentric retrospective review of patients with nAMD treated with anti-VEGF agents. Visual outcomes, 12 and 60 months after diagnosis, were evaluated. In an attempt to identify predictive factors of visual outcomes, multiple variables (demographic and epidemiological characteristics, angiographic and tomographic features) were analyzed, at baseline and during follow-up., Results: One hundred and seventeen patients were included. In multivariate analysis, baseline best-corrected visual acuity was associated with all visual endpoints at 12 and 60 months. Additionally, age, gender, number of injections, and development of subretinal fibrosis during follow-up were also significant predictors of visual outcomes at 60 months., Conclusions: Several factors can be useful in clinical practice as predictors of visual outcomes in response to anti-VEGF treatment of nAMD.

Purpose: To report the long-term clinical outcomes after switching from intravitreal bevacizumab or ranibizumab to aflibercept therapy in eyes with AMD., Methods: Retrospective analysis of changes in BCVA, SD-OCT image, and frequency of injections after 1, 2, and 3 years of follow-up., Results: 164 eyes were analyzed, 101 eyes switched from bevacizumab (group 1) and 63 from ranibizumab (group 2). One year after the switch, there was an overall nonsignificant mean decrease of 2 ETDRS letters in BCVA. Three years after, there was an overall mean decrease of 7 ETDRS letters, which was statistically significant. A significant improvement in the mean CRT was found at 1, 2, and 3 years. There was a significant decrease in the mean number of injections per year (7.8 to 6.5, p < 0.005) between the first and third year., Conclusion: Aflibercept can be useful in the management of refractory neovascular AMD, with a good morphological response. However, in the long-term, BCVA stabilization was not achieved.

Purpose: To report the safety and efficacy of intravitreal recombinant tissue plasminogen activator (rtPA) with gas for managing submacular hemorrhage., Methods: Patients with submacular hemorrhage centered in or close to the fovea underwent hemorrhage displacement with intravitreal injection of rtPA (50 μg/0.05 mL) followed by gas injection (0.3 mL SF6). Anatomic and visual outcomes are described., Results: Exudative age-related macular degeneration (AMD) (n = 4) and blunt trauma (n = 2) were the etiologies of submacular hemorrhage in this sample. Intravitreal injection of rtPA decreased the extent of submacular hemorrhage in all eyes, with complete hemorrhage displacement in 2 and partial displacement in 4. Visual acuity remained stable (n = 4) or improved (n = 2) after the procedure. Improvements in anatomic and visual outcomes were less evident in exudative AMD cases, which also had longer hemorrhage duration. Recurrence occurred only in 1 eye. No evident rtPA-associated retinal toxicity was observed., Conclusions: Untreated submacular hemorrhage has poor visual prognosis. Our results suggest that rtPA injection is a minimally invasive, simple, inexpensive procedure with few related complications. Cost-benefit of this injection seems acceptable.