Optical coherence tomography biomarkers in MYO7A-inherited retinal dystrophy: longitudinal study in pediatric patients.

PURPOSE: This study aims to answer a key question: is MYO7A-inherited retinal dystrophy (MYO7A-IRD) a photoreceptor-first or retinal pigment epithelium-first disease? A second aim was to determine the most useful biomarkers to monitor disease progression in pediatric patients with Usher syndrome type 1B (USH1) secondary to MYO7A mutation. METHODS: Fifty-two eyes from 26 patients with genetically-confirmed MYO7A-IRD underwent swept-source optical coherence tomography (SS-OCT). Structural abnormalities were evaluated and correlated with follow-up time and best corrected visual acuity (BCVA). All patients were evaluated at baseline and after ≥ 40 months of follow-up. RESULTS: The mean (SD) patient age was 9.92 (± 4.1) years. Mean follow-up time was 43 (± 3.2) months. At the final evaluation, the most common qualitative abnormalities in the subfoveal area were alterations in the photoreceptor outer segments (76.9% of eyes) and in the interdigitation zone (IZ) (80.8%). The presence of cystoid macular edema at baseline was independently associated with worse BCVA at the final assessment (increase in LogMAR estimate = 0.142; t(45.00) = 2.78, p = 0.009). The mean width of the ellipsoid and interdigitation zones decreased significantly (by 668 µm and 278 µm, respectively; both p < 0.001). CONCLUSION: This study shows that disruption of the photoreceptor outer segments and the IZ are the first alterations detected by SS-OCT in the early phases of MYO7A-IRD. These data highlight the potential value of measuring the width of the ellipsoid and IZ to evaluate disease progression. These findings also demonstrate the utility of monitoring for the emergence of cystic lesions as biomarkers of worse visual prognosis in patients with MYO7A-IRD.

Blocking Hemopexin With Specific Antibodies: A New Strategy for Treating Diabetic Retinopathy.

Hemopexin (HPX) is overexpressed in the retina of patients with diabetes and induces the breakdown of the blood-retinal barrier in vitro. The aim of this study was to evaluate whether HPX blockade by specific antibodies (aHPX) could avoid vascular leakage in vivo and microvascular angiogenesis in vitro and ex vivo. For this purpose, the effect of intravitreal (IVT) injections of aHPX on vascular leakage was evaluated in db/db mice and rats with streptozotocin-induced diabetes using the Evans Blue method. Retinal neurodegeneration and inflammation were also evaluated. The antiangiogenic effect of aHPX on human retinal endothelial cells (HRECs) was tested by scratch wound healing and tube formation using standardized methods, as well as by choroidal sprouting assays from retinal explants obtained in rats. We found that IVT injection of aHPX significantly reduced vascular leakage, retinal neurodegeneration, and inflammation. In addition, treatment with aHPX significantly reduced HREC migration and tube formation induced by high glucose concentration and suppressed choroidal sprouting even after vascular endothelial growth factor stimulation, with this effect being higher than obtained with bevacizumab. The antipermeability and antiangiogenic effects of IVT injection of aHPX suggest the blockade or inhibition of HPX as a new strategy for the treatment of advanced stages of diabetic retinopathy. ARTICLE HIGHLIGHTS: Hemopexin (HPX) is the best-characterized permeability factor in steroid-sensitive nephrotic syndrome. We have previously reported that HPX is overexpressed in the retina of patients with diabetes and induces the breakdown of the blood-retinal barrier in vitro. Here, we report that intravitreal injection of anti-HPX antibodies significantly reduces vascular leakage, retinal neurodegeneration, and inflammation in diabetic murine models and that the immunoneutralization of HPX exerts a significant antiangiogenic effect in vitro and in retinal explants. The blockade of HPX can be considered as a new therapy for advanced stages of diabetic retinopathy.

Multiresistant Candida Endophthalmitis Treated with Intravitreal Caspofungin: A Case Report.

BACKGROUND: Candida endophthalmitis is a severe complication of candidemia. Currently, the recommended treatment of fungal endophthalmitis is a combination of intravitreal and systemic antifungal drugs, and in some cases vitrectomy is also required. Intravitreal therapies that are commonly used are amphotericin B and voriconazole, although recently the use of intravitreal caspofungin has been described in a few case reports. However, clinical experience with intravitreal caspofungin is still limited. CASE PRESENTATION: We report a case of bilateral candida tropicalis endophthalmitis, initially managed with repeated 100 µg/0.1 ml caspofungin intravitreal injections and posteriorly treated with pars plana vitrectomy in both eyes. CONCLUSIONS: Intravitreal caspofungin could be a safe intravitreal alternative to habitual antimycotic drugs in cases with resistant candida endophthalmitis.Abbreviations: Intensive Care Unit (ICU); Best-Corrected Visual Acuity (BCVA).

Prevalence and implications of the presence of intraocular silicone oil droplets in patients treated with intravitreal injections of anti-VEGF.

BACKGROUND: To determine the percentage of patients who have silicone droplets in the vitreous after treatment with different anti-Vascular Endothelial Growth Factor (anti-VEGF) intravitreal injections (IVI) and how symptomatic they are. METHODS: One hundred fifty-two eyes of 140 patients who had at least received an IVI were recruited for this study. Data collection included the number and type of IVI (aflibercept, ranibizumab and bevacizumab) and the follow-up time. A complete ophthalmologic examination was carried out and patients were classified in four groups according to the amount of silicone droplets found in dilated fundoscopy (nonexistent, scarce, moderate and abundant). Measurement of intraocular pressure (IOP) was also carried out. An interview was conducted to report the presence and intensity of the symptomatology. RESULTS: Silicone oil droplets were reported in 109 eyes (71.7%). A positive correlation was found between the number of IVIs received and the quantity of droplets found, especially when aflibercept was used. Posterior vitreous detachment (PVD) was present in 65.8% of the patients, showing a positive correlation with the number of bubbles. Regarding the symptomatology, 60 eyes (39.5%) had floaters and the disturbance was reported to be 4 out of 10. The group with a moderate amount of silicone droplets had the highest percentage of floaters (60%). No statistical differences in the IOP were found between groups, although the group with abundant droplets had a higher mean IOP. CONCLUSION: A high prevalence of silicone droplets in vitreous of patients who undergo IVI treatment was found. It appears to have little impact on symptomatology and rise of IOP.

A Screening Tool for Self-Evaluation of Risk for Age-Related Macular Degeneration: Validation in a Spanish Population.

Purpose: The objectives of this study were the creation and validation of a screening tool for age-related macular degeneration (AMD) for routine assessment by primary care physicians, ophthalmologists, other healthcare professionals, and the general population. Methods: A simple, self-administered questionnaire (Simplified Théa AMD Risk-Assessment Scale [STARS] version 4.0) which included well-established risk factors for AMD, such as family history, smoking, and dietary factors, was administered to patients during ophthalmology visits. A fundus examination was performed to determine presence of large soft drusen, pigmentary abnormalities, or late AMD. Based on data from the questionnaire and the clinical examination, predictive models were developed to estimate probability of the Age-Related Eye Disease Study (AREDS) score (categorized as low risk/high risk). The models were evaluated by area under the receiving operating characteristic curve analysis. Results: A total of 3854 subjects completed the questionnaire and underwent a fundus examination. Early/intermediate and late AMD were detected in 15.9% and 23.8% of the patients, respectively. A predictive model was developed with training, validation, and test datasets. The model in the test set had an area under the curve of 0.745 (95% confidence interval [CI] = 0.705-0.784), a positive predictive value of 0.500 (95% CI = 0.449-0.557), and a negative predictive value of 0.810 (95% CI = 0.770-0.844). Conclusions: The STARS questionnaire version 4.0 and the model identify patients at high risk of developing late AMD. Translational Relevance: The screening instrument described could be useful to evaluate the risk of late AMD in patients >55 years without having an eye examination, which could lead to more timely referrals and encourage lifestyle changes.

Role of intraretinal and subretinal fluid on clinical and anatomical outcomes in patients with neovascular age-related macular degeneration treated with bimonthly, treat-and-extend and as-needed ranibizumab in the In-Eye study.

PURPOSE: To assess the effect of fluid status at baseline (BL) and at the end of the loading phase (LP) of three different ranibizumab regimens: treat-and-extend (T&E), fixed bimonthly (FBM) injections and pro re nata (PRN), in patients with neovascular age-related macular degeneration (nAMD). DESIGN: Post hoc analysis of the In-Eye study (phase IV clinical trial). METHODS: Patients were randomized 1:1:1 to the three study arms and were treated accordingly. The presence and type of fluid, intraretinal fluid (IRF) or subretinal fluid (SRF) and the anatomical and visual outcomes were analysed. MAIN OUTCOME MEASURES: Best-corrected visual acuity (BCVA), the mean change from baseline BCVA (BL BCVA), and the proportion of eyes gaining more than 15 letters or losing more than five letters were analysed. Morphological characteristics including the subtype of choroidal neovascular membrane and the development of atrophy and fibrosis were also evaluated. RESULTS: Patients with SRF at LP had better visual outcomes than patients with IRF. The persistence of SRF did not affect the mean change from BL BCVA among the three treatment regimens. However, in patients with IRF mean change from BL BCVA was significantly lower in the FBM group. The presence of IRF at BL and at the end of the loading phase was associated with the development of fibrosis at the end of the study; this result was contrary to that observed for patients with SRF. CONCLUSIONS: While SRF is compatible with good visual and anatomical outcomes, IRF leads to worse results in patients with nAMD; our results suggest that patients with IRF have better outcomes when individualized treatment regimens are used (PRN or T&E) in contrast with a FBM regimen.

Cell therapy with hiPSC-derived RPE cells and RPCs prevents visual function loss in a rat model of retinal degeneration.

Photoreceptor loss is the principal cause of blindness in retinal degenerative diseases (RDDs). Whereas some therapies exist for early stages of RDDs, no effective treatment is currently available for later stages, and once photoreceptors are lost, the only option to rescue vision is cell transplantation. With the use of the Royal College of Surgeons (RCS) rat model of retinal degeneration, we sought to determine whether combined transplantation of human-induced pluripotent stem cell (hiPSC)-derived retinal precursor cells (RPCs) and retinal pigment epithelial (RPE) cells was superior to RPE or RPC transplantation alone in preserving retinal from degeneration. hiPSC-derived RPCs and RPE cells expressing (GFP) were transplanted into the subretinal space of rats. In vivo monitoring showed that grafted cells survived 12 weeks in the subretinal space, and rats treated with RPE + RPC therapy exhibited better conservation of the outer nuclear layer (ONL) and visual response than RPE-treated or RPC-treated rats. Transplanted RPE cells integrated in the host RPE layer, whereas RPC mostly remained in the subretinal space, although a limited number of cells integrated in the ONL. In conclusion, the combined transplantation of hiPSC-derived RPE and RPCs is a potentially superior therapeutic approach to protect retina from degeneration in RDDs.

Transcriptomics analysis of Ccl2/Cx3cr1/Crb1rd8 deficient mice provides new insights into the pathophysiology of progressive retinal degeneration.

Chronic oxidative stress and immune dysregulation are key mechanisms involved in the pathogenesis of most retinal degenerative diseases, including age-related macular degeneration. The Ccl2-/-/Cx3cr1-/-/Crb1rd8/rd8 mouse model develops a progressive degeneration phenotype, with photoreceptor atrophy, drusen-like lesions or pigment alterations at an early age; however, the role of oxidative stress and immune function in the pathogenesis of the model is poorly understood. We performed a comprehensive characterization of the Ccl2-/-/Cx3cr1-/-/Crb1rd8/rd8 mouse to evaluate how these pathways influence pathogenesis. We generated a Ccl2-/-/Cx3cr1-/- double-knockout (DKO) mouse on a C57BL/6N background (with the rd8 mutation of the Crb1 gene), assessed its retina status and function during 9 months in both in vivo and post-mortem analysis, and performed a comprehensive transcriptomic analysis. DKOrd8 mice presented focal retinal lesions with increased infiltration of microglia and involvement of Müller cells. Lesions progressed to thinning of the photoreceptor nuclear layer, causing a loss in retinal function. Transcriptomics analysis revealed major differential expression of genes involved in oxidative stress and neuronal function, in particular genes related to the mitochondrial electron transport chain and antioxidant cellular response. Our results suggest that alterations in chemokine signaling combined with the rd8 mutation in Ccl2-/-/Cx3cr1-/-/Crb1rd8/rd8 mice involve early changes in several pathways associated with age-related macular degeneration, highlighting the relevance of these processes in the pathological retinal degeneration in the DKOrd8 model.

Differential response to intravitreal dexamethasone implant in naïve and previously treated diabetic macular edema eyes.

BACKGROUND: To identify different response patterns to intravitreal dexamethasone implants (IDI) in naïve and previously treated (PT) diabetic macular edema (DME) eyes in a real-life setting. METHODS: 342 IDI injections (203 DME eyes) were included. Number of IDI injections, percentage (%) of eyes with 1, 2, 3 and ≥ 4 injections, time to reinjections, visual acuity (VA), intraocular pressure (IOP) and central retinal thickness (CRT) were evaluated for naïve and PT DME eyes over 24 months. RESULTS: Mean number of injections was significantly lower in naïve vs PT DME eyes (1.40 ± 0.9 vs 1.82 ± 0.9, p < 0.001). The percentage of eyes receiving 1 injection was significantly higher in naïve vs PT DME eyes (76.1 vs 47.7), (p < 0.001). However, it was significantly lower for 2 (16.4 vs 29.4), or 3 injections (1.4 vs 17.6) (both p < 0.001), with no differences in eyes receiving ≥4 injections (5.9 vs 5.1 respectively, p = 0.80). Mean time to reinjection was not significantly different between both groups for the second, third and fourth injection (9.6 ± 4.0 vs 10.0 ± 5.5, p = 0.75, 13.2 ± 4.0 vs 16.0 ± 3.5, p = 0.21 and 21.7 ± 3.8 vs 19.7 ± 5.8, p = 0.55). VA scores were consistently better in naïve vs PT DME eyes at all studied timepoints, with no significant differences in CRT reduction or adverse effect rates. CONCLUSION: Naïve DME eyes received lower number of IDI injections and showed better VA levels than PT DME eyes for 24 months in a real-world setting. This data supports the IDI use in early DME stages and provide further evidence of better IDI response when used as first-line therapy.

Retinal Vasculitis Secondary to Durvalumab.

Ocular manifestations are very rarely reported as side effects to checkpoint inhibitors. We present a case of a 64-year-old Caucasian man in treatment with durvalumab for non-small-cell lung carcinoma who presented a retinal vasculitis with macular edema. After three boluses of methylprednisolone, the retinal vasculitis resolved and macular edema improved during follow-up. There was no need for durvalumab to be withdrawn.

Is optical coherence tomography angiography helpful in the differential diagnosis of choroidal nevus versus melanoma?

PURPOSE: To describe the imaging features of choroidal nevus and melanoma using optical coherence tomography angiography, and evaluate the ability of this technique to establish the differential diagnosis based on the display of the tumor's intrinsic vasculature. METHODS: Comparative analysis of optical coherence tomography angiography findings in consecutive patients diagnosed with choroidal nevus or choroidal melanoma following a complete ophthalmic evaluation, including best-corrected visual acuity and several imaging studies: color fundus photography, B-scan ultrasound, spectral-domain optical coherence tomography, and optical coherence tomography angiography. Optical coherence tomography angiography was used to investigate qualitative differences in the tumor vasculature. RESULTS: Thirty-six eyes (18 cases of choroidal nevus and 18 cases of choroidal melanoma) from 36 consecutive patients were included in the study. Only cases located posterior to equator were included to enable performance of all tests. On optical coherence tomography angiography, choroidal nevus showed well-delimited margins (78%), hyperreflective choroid capillary vasculature (83%), fewer avascular areas (17%), and neovascular membrane in one case (6%). Choroidal melanoma showed imprecise margins (72%), hyporeflective choroidal capillary vasculature (72%), multiple avascular areas (78%), and choroidal vascular changes (e.g. thick vascular networks or vascular loops; 45%). CONCLUSION: Optical coherence tomography angiography can provide useful information for assessing and differentiating between choroidal nevi and small melanomas. Significant differences between these conditions were found for the pattern of reflectivity, and presence/absence of avascular zones and vascular anomalies, which could be helpful for supporting the diagnosis.

Guidelines for the Management of Retinal Vein Occlusion by the European Society of Retina Specialists (EURETINA).

The high prevalence of cardiovascular disease particularly in the elderly population is associated with retinal vascular disease. Retinal vein occlusions represent severe disturbances of the hypoxia-sensitive neurosensory retina. Acute and excessive leakage leads to the diagnostic hallmarks of retinal hemorrhage and edema with substantial retinal thickening. Advanced diagnostic tools such as OCT angiography allow to evaluate retinal ischemia and identify the risk for late complications and will soon reach clinical routine besides fluorescein angiography. Accordingly, the duration of non-perfusion is a crucial prognostic factor requiring timely therapeutic intervention. With immediate inhibition of vascular leakage, anti-VEGF substances excel as treatment of choice. Multiple clinical trials with optimal potential for functional benefit or a lesser regenerative spectrum have evaluated aflibercept, ranibizumab, and bevacizumab. As retinal vein occlusion is a chronic disease, long-term monitoring should be individualized to combine maintenance with practicability. While steroids may be considered in patients with systemic cardiovascular risk, surgery remains advisable only for very few patients. Destructive laser treatment is an option if reliable monitoring is not feasible. Ophthalmologists are also advised to perform a basic systemic workup to recognize systemic concomitants. The current edition of the EURETINA guidelines highlights the state-of-the-art recommendations based on the literature and expert opinions in retinal vein occlusion.

Comparative study of penetrating keratoplasty and vitreoretinal surgery with Eckardt temporary keratoprosthesis in ocular trauma versus non-trauma patients.

PURPOSE: To evaluate the effectiveness of temporary keratoprosthesis combined with vitreoretinal surgery and penetrating keratoplasty in patients with or without trauma. METHODS: This retrospective study included 49 eyes in 49 non-trauma patients and 51 eyes in 48 ocular trauma patients who underwent penetrating keratoplasty and vitreoretinal surgery with Eckardt temporary keratoprosthesis between 2009 and 2016, with a follow-up of at least 12 months. Study variables included previous corneal, glaucoma, or retinal surgeries; various intraoperative surgical maneuvers; lens status; vitreoretinal and corneal pathology; functional outcomes; anatomical retinal reattachment; graft clarity; and need for glaucoma surgery or treatment. RESULTS: The mean age was 56 years in the non-trauma group and 42 years in the ocular trauma group. A total of 45% of the non-trauma cases and 24% of the ocular trauma cases had a single functional eye. Pseudophakic and aphakic keratopathy was diagnosed in 41% of the non-trauma group and corneal laceration in 65% of the ocular trauma group. In the ocular trauma group, injuries were open globe injury in 78%, closed globe injury in 12%, and intraocular foreign body in 10%. Retinal detachment with proliferative vitreoretinopathy was present in 39% of patients in the non-trauma group and in 35% of the ocular trauma group. Improvement or stability of visual acuity was higher among ocular trauma patients (86%) than in non-trauma patients (78%). The rate of clear corneal grafts was 49% in both groups. Retinal attachment was achieved in 90% and 78% of patients in the non-trauma and ocular trauma groups, respectively. The use of retinotomy had a positive influence on the final attached retina (p = 0.016). The placement of a scleral buckle significantly increased the risk of glaucoma (p = 0.004). Poor functional outcome was related to persistent retinal detachment (10% versus 16% in the non-trauma and ocular trauma groups, respectively), phthisis (25% versus 12%), hypotony (33% versus 18%), corneal graft end failure (51% in both groups), and secondary glaucoma (18% versus 24%). CONCLUSION: In patients with both vitreoretinal and corneal pathology, the use of Eckardt temporary keratoprosthesis combined with vitreoretinal surgery and penetrating keratoplasty resulted in improvement of visual acuity, particularly in the groups of ocular trauma and monocular patients. The high rate of retinal reattachment and the low rate of graft rejection was probably related to the use of new vitreoretinal techniques, including retinotomy in selected patients.

Long-term probability of intraocular pressure elevation with the intravitreal dexamethasone implant in the real-world.

PURPOSE: To evaluate the long-term cumulative probability of intraocular pressure (IOP) elevation with the intravitreal dexamethasone implant (IDI) when used to treat different indications: diabetic macular edema, uveitis, retinal vein occlusion. METHODS: 705 IDI injections (429 eyes) were assessed and Kaplan-Meier graphs were generated to assess: the probability of different levels of IOP elevation (IOP≥21, ≥25 or ≥35 mmHg), IOP change ≥10 mmHg, initiation of IOP-lowering treatment, glaucoma surgery, IOP change with repeat injections and IOP elevation in eyes with glaucoma and ocular hypertension (OHT). RESULTS: The cumulative probability of IOP ≥21, ≥25 and ≥35 mmHg was 50%-60%, 25%-30% and 6%-7% at 12-24 months, respectively. The probability of initiating IOP-lowering medication was 31%-54% at 12-24 months. Glaucoma and OHT eyes had a higher probability of mild IOP elevation (≥21 mmHg, 65.1%, 75% and 57.8%, p = 0.01), yet a similar moderate (≥25 mmHg, 22.3%, 28% and 30.2%, p = 0.91) and severe elevation of IOP (≥35 mmHg, 3.7%, 7.1% and 4%, p = 0.71) as normal eyes. Glaucoma surgery was required in only 0.9% cases (4/429). At baseline, 8.8% of the treated eyes had glaucoma, 6.7% OHT and 16.9% were already on IOP-lowering medication. CONCLUSIONS: In the long-term (24 months), IOP elevation is common, generally mild (30% IOP, ≥25 mmHg) and well-tolerated, resolving with topical treatment (54%) and rarely requiring surgery (0.9%).

PREOPERATIVE VITREORETINAL INTERFACE ABNORMALITIES ON SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY AS RISK FACTOR FOR PSEUDOPHAKIC CYSTOID MACULAR EDEMA AFTER PHACOEMULSIFICATION.

PURPOSE: We assessed the role of vitreoretinal interface status in the development of pseudophakic cystoid macular edema (PCME) after cataract surgery. METHODS: Prospective cohort study in which 112 patients (112 eyes) scheduled for cataract surgery were selected at random to undergo spectral domain optical coherence tomography (OCT) within 1 week preoperatively and at 1 and 3 months postoperatively. Spectral domain OCT macular images included no vitreoretinal contact, focal and diffuse vitreomacular adhesion, focal and diffuse vitreomacular traction, epiretinal membrane, macular hole, and macular edema. RESULTS: The incidence of PCME was 11.6% (13 eyes), all of them being diagnosed at 1 month, and 7 eyes resolved at 3 months. The only risk factor for PCME was detection of nonsurgical epiretinal membrane by spectral domain OCT before phacoemulsification, being developed in 5 of 16 eyes (χ = 0.08, odds ratio 4.53, 95% confidence interval 1.28-16.13). Other variables such as posterior vitreous detachment, subfoveal choroidal thickness, diabetes, or hypertension were not significantly associated with PCME. CONCLUSION: In this cohort, preoperative detection of epiretinal membrane by spectral domain OCT was a risk factor for PCME after cataract extraction. It is recommended to perform a spectral domain OCT before cataract surgery because the presence of an epiretinal membrane may be passed unnoticed by fundus examination.

Early and intermediate age-related macular degeneration: update and clinical review.

Age-related macular degeneration (AMD) is the leading cause of irreversible central vision loss in developed countries. With the aging of population, AMD will become globally an increasingly important and prevalent disease worldwide. It is a complex disease whose etiology is associated with both genetic and environmental risk factors. An extensive decline in the quality of life and progressive need of daily living assistance resulting from AMD among those most severely affected highlights the essential role of preventive strategies, particularly advising patients to quit smoking. In addition, maintaining a healthy diet, controlling other risk factors (such as hypertension, obesity, and atherosclerosis), and the use of nutritional supplements (antioxidants) are recommendable. Genetic testing may be especially important in patients with a family history of AMD. Recently, unifying criteria for the clinical classification of AMD, defining no apparent aging changes; normal aging changes; and early, intermediate, and late AMD stages, are of value in predicting AMD risk of progression and in establishing recommendations for the diagnosis, therapeutic approach, and follow-up of patients. The present review is focused on early and intermediate AMD and presents a description of the clinical characteristics and ophthalmological findings for these stages, together with algorithms for the diagnosis and management of patients, which are easily applicable in daily clinical practice.

Incidence of cystoid macular edema after Descemet's stripping automated endothelial keratoplasty.

AIM: To determine the incidence of cystoid macular edema (CME) after Descemet's stripping automated endothelial keratoplasty (DSAEK). METHODS: This study included all consecutive patients operated in a Spanish tertiary reference hospital over a period of four years. A total of 55 eyes from 47 patients matched the selection criteria. CME was diagnosed clinically at the slit-lamp and confirmed by optical coherence tomography. RESULTS: Six cases of CME were diagnosed postoperatively, which represented an incidence of 11%. Three patients had previously undergone DSAEK alone (7%; 3/41) and the other three, DSAEK combined with phacoemulsification (21%; 3/14). Five out of six patients with CME responded to standard therapy. CONCLUSION: CME is a possible complication after DSAEK and can be treated with standard therapy. CME appears more frequently when DSAEK is combined with phacoemulsification and posterior chamber (PC) intraocular lens (IOL) implantation. Intraoperative damage to the corneal endothelial cells might play a role in the pathogenesis of CME. As long as the causes remain unclear, we recommend administering prophylaxis when risk factors are present or when combined surgery is planned.

Guidelines for the Management of Diabetic Macular Edema by the European Society of Retina Specialists (EURETINA).

Diabetic retinal disease is envisioned to become the plague of the coming decades with a steep increase of worldwide diabetes incidence followed by a substantial rise in retinal disease. Improvements in diagnostic and therapeutic care have to cope with this dilemma in a clinically and socioeconomically efficient manner. Laser treatment has found a less destructive competitor in pharmacological treatments. As a consequence of recent rigorous clinical trials, laser photocoagulation is no longer recommended for the treatment of diabetic macular edema (DME), and anti-vascular endothelial growth factor therapy has emerged as first-line therapy. Steroids have maintained a role in the management of chronically persistent DME. The paradigm shifts in therapy are accompanied by a substantial break-through in diagnostics. The following guidance for the management of DME has been composed from the best updated knowledge of leading experts in Europe and represents another volume in the series of EURETINA recommendations for the management of retinal disease.

Anterior iris-claw intraocular lens implantation for the management of nontraumatic ectopia lentis: long-term outcomes in a paediatric cohort.

PURPOSE: To report the feasibility and long-term safety of lensectomy and iris-claw intraocular lens (IOL) implantation to treat children with severe ectopia lentis in a paediatric tertiary hospital. METHODS: Prospective cohort study of 21 eyes from 12 patients with severe ectopia lentis and visual acuity <20/63. All eyes underwent 23-gauge pars plana vitrectomy, lensectomy, iridectomy and Artisan IOL implantation in the anterior chamber with iris-claw enclavation via pars plana. Mean age at surgery was 8.0 ± 5.3 yo (range 3-17 years). A full ophthalmologic examination including best-corrected visual acuity (BCVA), biomicroscopy, intraocular pressure (IOP) measurement, fundus evaluation and central endothelial cell count (cECC) was performed pretreatment, at 3 months' postsurgery, and every 6 months thereafter. Ultrasound biomicroscopy (UBM) was performed 12 months after surgery. RESULTS: Mean follow-up was 39.3 ± 13.0 months. Best-corrected visual acuity (BCVA) (mean ± SD) improved from 0.91 ± 0.29 logMar preoperatively to 0.18 ± 0.23 logMar at final follow-up (p < 0.0001). Mean distance from the endothelium to the anterior IOL surface after surgery was 3.11 ± 0.61 mm. Postsurgically, cECC loss was 5.04% ± 9.58% with an annual cECC loss rate of 3.16% ± 4.46%. One patient developed IOL dislocation and retinal detachment after severe ocular contusion requiring vitrectomy, IOL refixation and gas tamponade. Another patient developed cystoid macular oedema, managed with intravitreal dexamethasone. CONCLUSION: This technique is both feasible and effective to manage severe ectopia lentis in children. Lifetime ophthalmic follow-up including cECC measurement, IOL position monitoring and fundus examination is mandatory in these patients.

Telemedicine for a General Screening of Retinal Disease Using Nonmydriatic Fundus Cameras in Optometry Centers: Three-Year Results.

PURPOSE: Describe the first 3 years of highly specialized retinal screening through a web platform using a retinologists' network for image reading. METHODS: All patients who came to centers in the network and consented to fundus photography were included. Images were evaluated by ophthalmologists. We describe number of patients, age, visual acuity, retinal abnormalities, medical recommendations, and factors associated with abnormal retinographies. RESULTS: Fifty thousand three hundred eighty-four patients were included; mean age 52.3 years (range 3-99). Mean visual acuity 20/25. Of the total cohort, 75% had normal retinographies, 22% had abnormalities, 1% referred acute floaters, 1% referred acute symptoms with normal retinography, and 1% could not be assessed. Ophthalmological referral was recommended in 12,634 patients: 9% urgent visit, 11% preferential (2-3 weeks), and 80% an ordinary visit. Age-related maculopathy signs were the most common abnormalities (2,456 patients, 4.8%). Epiretinal membrane was the second (764 cases, 1.5%). Diabetic retinopathy was suspected in 543 patients (1%), and nevi in 358 patients (0.7%). Patients older than 50 years had significantly more retinal abnormalities (31.5%) than younger ones (11.1%) (p < 0.0001; odds ratio [OR] 2.47; confidence interval [CI] 2.37-2.57). Patients with almost one eye with a myopic defect greater than -5 spherical equivalent had a higher risk of presenting abnormalities (p < 0.001; OR 1.04; CI 1.03-1.05). CONCLUSIONS: A high rate of asymptomatic retinal abnormalities was detected in this general screening, justifying this practice. Many patients who visit optometrists in Spain are unaware that they would benefit from ophthalmological monitoring. The ophthalmic community should lead initiatives of the type presented to preserve and guarantee quality standards.