Impacts of X-linked Retinitis Pigmentosa and Patient Pathways in European Countries: Results from the Cross-sectional EXPLORE XLRP-1 Physician Survey.

INTRODUCTION: X-linked retinitis pigmentosa (XLRP) is a rare, incurable, vision-threatening, genetic disease. In this study, we aimed to reveal the real-world burden of this disease from the viewpoint of retina specialists and geneticists involved directly in XLRP care and to identify unique insights that may not otherwise be available through typical clinical studies or health economic research. METHODS: In this exploratory, cross-sectional study (EXPLORE XLRP-1), retina specialists (n = 20) and geneticists (n = 5) in France, Germany, Italy, Spain, and the UK provided anonymized insights on their experiences managing patients with XLRP (n = 80) via an online survey and 60-min telephone interview. RESULTS: Survey respondents reported that patient independence decreased over time, where 37% of patients were considered "completely autonomous" at diagnosis versus 23% at the last consultation. At their last visit, 45% of patients were active in the workforce; 67% (12/18) of "completely autonomous" patients had active working status compared with 13% (1/8) of "completely dependent" patients. The average time from onset of symptoms to diagnosis was 4 years and varied among countries. In 78% of patients, XLRP was confirmed by genetic testing, the rate of which varied among countries (range, 50-94%), taking up to 6 months to receive results. Specialists identified unmet needs in XLRP management including more standardized assessments of quality of life (QoL) as well as easier and earlier access to specialists, genetic testing, patient support programs, and effective treatment options. CONCLUSIONS: The diagnosis, genetic testing, and management pathways among patients with XLRP can vary considerably. There is a need for more standardized diagnosis and management pathways, and QoL assessments, due to the major impact that XLRP has on patients' lives.

Quality of life, functioning and participation of children and adolescents with visual impairment: A scoping review.

BACKGROUND: Vision has a key role in children's neuromotor, cognitive and social development. Children with visual impairment attain developmental milestones at later stages and are at higher risk of developing psychological disorders and social withdrawn. AIMS: We performed a scoping review to summarize the mostly used instruments assessing the impact of visual impairment on quality of life, functioning and participation of children and adolescents. In addition, the main findings of the included studies are discussed. METHODS AND PROCEDURES: We searched for papers assessing quality of life, functioning and participation of children and adolescents with visual impairment from 0 to 18 years old conducted between 2000 and 2023. OUTCOMES AND RESULTS: In total, 69 studies met the inclusion criteria and were included in the review. Child self-report, caregivers-proxy and self-report questionnaires as well as interviews were used. The results showed that quality of life, functioning and participation are significantly reduced in children and adolescents with visual impairment, and that the impact depends on different factors (e.g., severity of the impairment, age). CONCLUSIONS AND IMPLICATIONS: Considering the significant impact of visual impairment on quality of life, functioning and participation on this population, it is fundamental to develop integrated and multi-dimensional assessment programs that evaluate the impact of visual impairment on those dimensions considering different contexts of life (e.g., family, school, leisure time). WHAT THIS PAPER ADDS?: The present review aims to give an overview of what is known about the impact of visual impairment on quality of life, functioning and participation of children and adolescents. We assumed a biopsychosocial perspective which, in line with the definition of health by the International Classification of Functioning, Disability and Health (WHO, 2001), considered how body functions and structures, functioning, participation and environmental factors dynamically interact to define the health, or the disease, status of a person at a certain moment of life. We reported the most used instruments for the assessment of quality of life, participation, and functioning, with a specific interest on Patient-Reported Outcome Measures and self-report measures. By reporting the different instruments used, we gave a broad overview about the available tools that can be used in clinical as well as in research field to assess quality of life, functioning and participation in this population. Additionally, the review of the existing literature allowed us to demonstrate that those dimensions are negatively impacted by visual impairment and thus they should be considered in the assessment programs. Specifically, there is the need to provide more integrated assessment programs that investigate the impact of visual impairment on children and adolescents' social and emotional wellbeing, everyday functioning and social relationship, considering their subjective experience together with the one of caregivers, teachers, health care professionals, and other relevant adults involved in their life. Additionally, it is essential to plan and implement multidimensional assessment programs that consider how all areas of life are differently impacted by visual impairment.

Long-term functional and structural outcomes after large chorioretinectomy for ruptured globe following blunt trauma.

BACKGROUND: The purpose of this study was to present a modified surgical technique involving pars plana vitrectomy with large chorioretinectomy for eyes with rupture of the globe due to severe ocular blunt trauma. METHODS: This retrospective study included consecutive patients with rupture of the globe due to blunt trauma who were treated at the King Khaled Eye Specialist Hospital (Riyadh, Saudi Arabia). All patients underwent 25-gauge pars plana vitrectomy with large chorioretinectomies involving all the tissue around the posterior scleral wounds. Outcome measures included best-corrected visual acuity (BCVA), anatomical success and globe survival, rates of complications. RESULTS: 15 eyes of 15 patients were included. Mean BCVA was 2.88 ± 0.13 logMAR at presentation, and significantly improved to 0.83 ± 0.28 logMAR (P < 0.001), with 10 patients (67%) achieving a final BCVA ≥ 20/200. Anatomical success and globe survival were achieved in 11 (73%) and 15 (100%) of eyes, respectively. Postoperative complications included retinal detachment in 6 eyes (40%), epiretinal membrane in 6 (40%), hypotony in 4 (26%), PVR in 2 (13%). CONCLUSIONS: Pars plana vitrectomy with large chorioretinectomy is an effective treatment for globe rupture following severe blunt trauma, yielding good visual outcomes and anatomical success rates.

Denuded Descemet's membrane supports human embryonic stem cell-derived retinal pigment epithelial cell culture.

Recent clinical studies suggest that retinal pigment epithelial (RPE) cell replacement therapy may preserve vision in retinal degenerative diseases. Scaffold-based methods are being tested in ongoing clinical trials for delivering pluripotent-derived RPE cells to the back of the eye. The aim of this study was to investigate human embryonic stem cell-derived retinal pigment epithelial (hESC-RPE) cells survival and behaviour on a decellularized Descemet's Membrane (DM), which may be of clinical relevance in retinal transplantation. DMs were isolated from human donor corneas and treated with thermolysin. The DM surface topology and the efficiency of the denudation method were evaluated by atomic force microscope, scanning electron microscopy and histology. hESC-RPE cells were seeded onto the endothelial-side surface of decellularized DM in order to determine the potential of the membrane to support hESC-RPE cell culture, alongside maintaining their viability. Integrity of the hESC-RPE monolayer was assessed by measuring transepithelial resistance. RPE-specific gene expression and growth factors secretion were assessed to confirm maturation and functionality of the cells over the new substrate. Thermolysin treatment did not affect the integrity of the tissue, thus ensuring a reliable method to standardize the preparation of decellularized DM. 24 hours post-seeding, hESC-RPE cell attachment and initial proliferation rate over the denuded DM were higher than hESC-RPE cells cultured on tissue culture inserts. On the new matrix, hESC-RPE cells succeeded in forming an intact monolayer with mature tight junctions. The resulting cell culture showed characteristic RPE cell morphology and proper protein localization. Gene expression analysis and VEGF secretion demonstrate DM provides supportive scaffolding and inductive properties to enhance hESC-RPE cells maturation. Decellularized DM was shown to be capable of sustaining hESC-RPE cells culture, thus confirming to be potentially a suitable candidate for retinal cell therapy.

Risk of retinal vein occlusion following COVID-19 vaccination: a self-controlled case series.

BACKGROUND: To evaluate the association between COVID-19 vaccination and retinal vein occlusion (RVO). METHODS: This multicentre self-controlled case series included patients with RVO seen in five tertiary referral centres in Italy. All adults who received at least one dose of the BNT162b2, ChAdOx1 nCoV-19, mRNA-1273 or Ad26.COV2.S vaccine and had a first diagnosis of RVO between January 01, 2021, and December 31, 2021 were included. Incidence rate ratios (IRRs) of RVO were estimated using Poisson regression, comparing rates of events in a 28-day period following each dose of vaccination and in the unexposed control periods. RESULTS: 210 patients were included in the study. No increased risk of RVO was observed after the first dose (1-14 days IRR: 0.87, 95% CI: 0.41-1.85; 15-28 days IRR: 1.01, 95% CI: 0.50-2.04; 1-28 days IRR: 0.94, 95% CI: 0.55-1.58) and second dose of vaccination (1-14 days IRR: 1.21, 95% CI: 0.62-2.37; 15-28 days IRR: 1.08, 95% CI: 0.53-2.20; 1-28 days IRR: 1.16, 95% CI: 0.70-1.90). No association between RVO and vaccination was found in subgroup analyses by type of vaccine, gender and age. CONCLUSIONS: This self-controlled case series found no evidence of an association between RVO and COVID-19 vaccination.

Leber Mitochondrial Optic Neuropathy in Pediatric Females With Focus on Very Early Onset Cases.

The aim of this study was to describe the phenotype of Leber hereditary optic neuropathy occurring in pediatric females. This disease generally affects young adult males, but it can occur also in females, and research data in this population is lacking. The very early onset can challenge the diagnosis and delay treatment. We searched PubMed through February 2021 and identified 226 pediatric females with genetically confirmed Leber hereditary optic neuropathy and added a new case of a 3-year-old female. The male-female ratio was 1.8:1; the mean onset age in females was 11 years with the onset at 3 years of age occurring in 3 females only. Acute onset with mild visual impairment was the most common presentation, associated with optic disc edema in 16%. Differential diagnoses are pseudotumor cerebri, optic nerve drusen and optic neuritis. The outcome is poor with partial recovery in 50%, despite some receiving Idebenone therapy.

RPE65-Associated Retinopathies in the Italian Population: A Longitudinal Natural History Study.

Purpose: To investigate the course of inherited retinal degenerations (IRD) due to mutations in the RPE65 gene. Methods: This longitudinal multicentric retrospective chart-review study was designed to collect best corrected visual acuity (BCVA), Goldman visual field, optical coherence tomography (OCT), and electroretinography (ERG) measurements. The data, including imaging, were collected using an electronic clinical research form and were reviewed at a single center to improve consistency. Results: From an overall cohort of 60 Italian patients with RPE65-associated IRD, 43 patients (mean age, 27.8 ± 19.7 years) were included and showed a mean BCVA of 2.0 ± 1.0 logMAR. Time-to-event analysis revealed a median age of 33.8 years and 41.4 years to reach low vision and blindness based on BCVA, respectively. ERG (available for 34 patients) showed undetectable responses in most patients (26; 76.5%). OCT (available for 31 patients) revealed epiretinal membranes in five patients (16.1%). Central foveal thickness significantly decreased with age at a mean annual rate of -0.6%/y (P = 0.044). We identified 43 different variants in the RPE65 gene in the entire cohort. Nine variants were novel. Finally, to assess genotype-phenotype correlations, patients were stratified according to the number of RPE65 loss-of-function (LoF) alleles. Patients without LoF variants showed significantly (P < 0.05) better BCVA compared to patients with one or two LoF alleles. Conclusions: We described the natural course of RPE65-associated IRD in an Italian cohort showing for the first time a specific genotype-phenotype association. Our findings can contribute to a better management of RPE65-associated IRD patients.

Diabetic macular edema: Safe and effective treatment with intravitreal triamcinolone acetonide (Taioftal).

PURPOSE: To suggest the safety and efficacy of preservative-free triamcinolone acetonide intravitreal injectable suspension (Taioftal) for the treatment of diabetic macular edema. METHODS: A prospective clinical study involved 49 patients (49 eyes), that were treated with Taioftal and followed-up for six months. Complete ophthalmic examination, including spectral domain optical coherence tomography, was performed at baseline, and at month 1, 3, 6 after the intravitreal injection. Accurate collection and analysis of best-corrected visual acuity (BCVA), central foveal thickness (CFT), intraocular pressure (IOP), and adverse events (AEs) were carried out in order to evaluate visual function and macular morphology before and after treatment. RESULTS: Median BCVA value chosen as comparing statistics was significantly improved at every follow-up time points (gain of 6 letters at month 1, 12 at month 3 -improvement up to 24% at month 3 with stabilization until month 6) compared to baseline, as certified by Kruskal-Wallis rank sum test (P<0.05). Median CFT significantly waned at each follow-up times (decrease of about 65 µm at month 1, 155 at month 3 -reduction up to 28% at month 3 keeping good outcome until month 6) compared to baseline (P<0.05). IOP elevation, with no severe increases, was the most common among spotted AEs (median of 23 mmHg at month 1, 20 at month 3). CONCLUSION: Intravitreal injection of preservative-free triamcinolone (Taioftal) is an effective, safe and inexpensive drug used to improve visual acuity and reduce central foveal thickness in eyes affected by diabetic macular edema during an average time of 6 months. Temporary, never severe, elevation of IOP is totally manageable with topical medications. No serious vision-threatening complications are related to the use of intravitreal triamcinolone injections.

Fluorescein Angiography Findings in Eyes With Lamellar Macular Hole and Epiretinal Membrane Foveoschisis.

Purpose: The purpose of this paper was to study fluorescein angiography (FA) findings in eyes with lamellar macular hole (LMH), and epiretinal membrane (ERM) foveoschisis. Methods: In this prospective, observational case series, 46 eyes of patients affected by either LMH or ERM foveoschisis were examined using optical coherence tomography (OCT) and FA. All patients underwent a comprehensive ophthalmological examination and a general workup to exclude uveitis. Main outcome measures were: presence of FA abnormalities, measurements of the areas of vascular leakage, and intensity of pixels in the vitreous. Results: Twenty-four (52.2%) eyes with LMH and 22 (47.8%) with ERM foveoschisis were studied. Overall, FA abnormalities were found in 20 (83.3%) eyes with LMH and 18 (81.8%) with ERM foveoschisis. The median areas of posterior pole and peripheral leakage were 7.52 vs. 1.07 mm2 (P = 0.03) and 21.8 vs. 3.74 mm2 (P = 0.02) in the LMH and ERM foveoschisis group, respectively. Disk hyperfluorescence was found in 8 and 4 eyes and perivascular leak in 10 and 4 eyes with LMH and ERM foveoschisis, respectively. OCT-derived measurements of vitreous intensity did not differ between the two groups, and the investigational workup for uveitis was negative in all patients. Conclusions: Discrete areas of central and peripheral leakage are commonly found in eyes with LMH and ERM foveoschisis, whereas perivascular leak and hyperfluorescence of the disc are less frequently observed. These findings suggest that breakdown of the retinal blood barrier, involving the posterior pole and the periphery, is frequently associated with these two vitreoretinal disorders.

A novel mutation of BEST1 gene in Best disease.

PURPOSE: To describe a new genetic variation of BEST1 gene in Best vitelliform macular dystrophy. METHODS: A patient with bilateral multiple retinal yellowish lesions at the posterior pole underwent fluorescein angiography, fundus autofluorescence, optical coherence tomography, electrooculogram and blood sample for genetic testing. RESULTS: A diagnosis of a Best vitelliform macular dystrophy was made. Heterozygous mutation c.76G > A (p.Gly26Ser) in exon 2 of the BEST1 gene was found. CONCLUSION: These findings contribute to expand the mutation spectrum of BEST1 gene.

Effects of the first month of lockdown for COVID-19 in Italy: A preliminary analysis on the eyecare system from six centers.

PURPOSE: To compare the number of eye surgical procedures performed in Italy during the first month of lockdown with those performed in the same period in 2019. METHODS: Review of the surgical procedures performed from 10 March to 9 April 2019 and from 10 March to 9 April 2020 (the first month of lockdown because of the COVID-19 outbreak) at six academic institutional centers of Italy. A distinction was made between urgent procedures: any trauma repairment, trabeculectomy/drainage implant for glaucoma, any operation for rhegmatogenous retinal detachment (RRD) repair, pars plana vitrectomy (PPV) for vitreous hemorrhage (VH), macular hole, or retained lens fragments; elective procedures: corneal transplant, phacoemulsification for cataract extraction, silicone oil removal, and PPV for epiretinal membrane; and intravitreal injections (either anti-vascular endothelial growth factor [VEGF] or dexamethasone) to treat exudative maculopathies. The main outcome measure was the rate of reduction in urgent and elective surgeries performed. RESULTS: Overall, 3624 and 844 surgical procedures were performed from 10 March to 9 April 2019 and from 10 March to 10 April 2020, respectively (-76.7% in 2020 compared to 2019, p < 0.0001). Urgent and elective surgical procedures and intravitreal injections of anti-VEGF drugs or dexamethasone reduced significantly in 2020 in comparison to 2019 (p < 0.0001 for urgent and elective surgeries and 0.01 for intravitreal injections). CONCLUSION: A significant reduction in the rate of urgent and elective surgeries and intravitreal injections was recorded during the first month of lockdown compared to the same period in 2019. With this analysis, the authors hope to provide some preliminary insights about the consequences of lockdown for the eyecare system in Italy.

Application of Artificial Intelligence in Targeting Retinal Diseases.

Retinal diseases affect an increasing number of patients worldwide because of the aging population. Request for diagnostic imaging in ophthalmology is ramping up, while the number of specialists keeps shrinking. Cutting-edge technology embedding artificial intelligence (AI) algorithms are thus advocated to help ophthalmologists perform their clinical tasks as well as to provide a source for the advancement of novel biomarkers. In particular, optical coherence tomography (OCT) evaluation of the retina can be augmented by algorithms based on machine learning and deep learning to early detect, qualitatively localize and quantitatively measure epi/intra/subretinal abnormalities or pathological features of macular or neural diseases. In this paper, we discuss the use of AI to facilitate efficacy and accuracy of retinal imaging in those diseases increasingly treated by intravitreal vascular endothelial growth factor (VEGF) inhibitors (i.e. anti-VEGF drugs), also including integration and interpretation features in the process. We review recent advances by AI in diabetic retinopathy, age-related macular degeneration, and retinopathy of prematurity that envision a potentially key role of highly automated systems in screening, early diagnosis, grading and individualized therapy. We discuss benefits and critical aspects of automating the evaluation of disease activity, recurrences, the timing of retreatment and therapeutically potential novel targets in ophthalmology. The impact of massive employment of AI to optimize clinical assistance and encourage tailored therapies for distinct patterns of retinal diseases is also discussed.

Curcumin-Based Treatment for Macular Edema from Uncommon Etiologies: Efficacy and Safety Assessment.

The aim of this study was to investigate the efficacy and safety of curcumin formulation with a polyvinylpyrrolidone-hydrophilic carrier (CHC*; Diabec®-AlfaIntes, Italy) for the treatment of macular edema (ME) from uncommon etiologies. We conducted retrospective interventional case series, reviewing the medical records of patients referred to the Eye Center, Humanitas Hospital, Bergamo due to persistent ME related to uncommon causes and treated by oral administration of CHC. The main outcomes assessed were best-corrected visual acuity (BCVA), central macular thickness (CMT), and the presence of intraretinal and/or subretinal fluid (SRF). Only patients with a minimum follow-up (f/u) of 6 months were included. The occurrence of any adverse effect was registered. Thirty-one eyes of 30 patients were included, with a mean f/u of 8.32 ± 1.77 months. Of them, 9 patients (10 eyes) were affected by postoperative ME and 21 by chronic central serous chorioretinopathy. Median BCVA significantly improved after treatment, changing from 0.3 [0.16-0.5] to 0.1 [0-0.3] logarithm of the minimum angle of resolution (P < .001). Also CMT was significantly improved, as it decreased from 400 [364-438] µm before treatment to 280 [242-307] µm at the last f/u visit (P < .001). The complete absorption of intraretinal/SRF was detected in 23 of 31 eyes (74%) at the final f/u. No adverse effects were registered. In conclusion, treatment with CHC was effective and safe for eyes affected by ME of various uncommon etiologies, resulting in significant improvement of both functional and anatomical outcomes, with the complete resolution of the edema in the majority of cases (74%).

Dexamethasone Implant Produces Better Outcomes than Oral Acetazolamide in Patients with Cystoid Macular Edema Secondary to Retinitis Pigmentosa.

Purpose: To compare the clinical outcome of intravitreal dexamethasone implant versus oral acetazolamide in patients with cystoid macular edema (CME) secondary to retinitis pigmentosa (RP). Design: Multicenter, prospective, propensity-score-matched, comparative study. Methods: Eyes with RP and CME were treated either with intravitreal dexamethasone implant or with oral acetazolamide (500 mg/day). Patients were evaluated monthly and followed up for 12 months. Primary outcome measures were changes in central retinal thickness and best corrected visual acuity (BCVA). Adverse events were recorded. Results: Propensity score matching resulted in 2 groups of 30 eyes each. All patients completed 12 months of follow-up. Mean central retinal thickness decreased from 535 µm at baseline to 208 µm at month 12 in the dexamethasone implant group and from 519 to 339 µm in the oral acetazolamide group (P < 0.001, Student's t-test). Mean BCVA average change from baseline during the study (area-under-the-curve approach) was -0.084 logarithm of the minimum angle of resolution (logMAR) (+4.2 letters) in the dexamethasone implant group and -0.032 (+1.6 letters) in the oral acetazolamide group (P < 0.05, Mann-Whitney U test). Patients in the dexamethasone implant group required on average 1.7 treatments during 1 year of therapy. Conclusions: In this study, intravitreal dexamethasone implant produced better anatomic and functional improvements over oral acetazolamide in patients affected by CME secondary to RP. Larger, randomized clinical trials with longer follow-up are warranted to confirm these data.

Peripheral Intrastromal Hydration Facilitates Safe, Deep Trephination in Corneas of Irregular Thickness.

PURPOSE: To evaluate the feasibility of regularizing the peripheral corneal thickness before deep trephination in highly irregular corneas undergoing deep anterior lamellar keratoplasty (DALK) by means of intrastromal hydration with saline. METHODS: This is an interventional case series including all eyes with irregular peripheral corneal thickness undergoing DALK for any indication between January 1, 2016, and January 1, 2017, at a single tertiary center in Forli, Italy. Before trephination, 1 mL of normal saline was injected intrastromally into each clock hour of peripheral thinning (determined using preoperative pachymetry) using a 30-gauge needle. A deep trephination of 400 to 450 µm was then performed, and DALK was completed as per our previously described technique. Primary outcome measures were perforation during trephination and intraoperative complications, with secondary outcomes of best corrected visual acuity and refraction. RESULTS: Peripheral intrastromal hydration was performed in 61 eyes of 61 patients. Intrastromal hydration ensured a safe trephination without perforation into the anterior chamber (AC) in 59 of 61 eyes. In the 2 cases in which perforation occurred, the perforation site was sutured with a full-thickness suture and the surgery was completed successfully. No cases required conversion to penetrating keratoplasty. Intrastromal injection of 1 mL of normal saline resulted in an increase in corneal thickness of 31%. After surgery, double AC was observed in 3 cases (4.9%), with all cases being managed successfully by air injection into the AC. CONCLUSIONS: Zonal peripheral intrastromal hydration is a feasible technique to enable safe, deep trephination even in corneas of highly irregular thickness.

Central Serous Chorioretinopathy: Pathogenesis and Management.

Central serous chorioretinopathy (CSC) is a common retina disease and has a relative high recurrence rate, etiology, and pathogenesis of which remains largely ambiguous. The effects on the retina are usually self-limited, although some people are left with permanent vision loss due to progressive and irreversible photoreceptor damage or retinal pigment epithelium atrophy. There have been a number of interventions used in CSC, including, but not limited to, laser treatment, photodynamic therapy (PDT), intravitreal injection of anti-vascular endothelial growth factor agents, and subthreshold lasers. It is not clear whether there is a clinically important benefit to treating acute CSC, which often resolves spontaneously as part of its natural history. Of the interventions studied to date, PDT and micropulse laser treatment appear the most promising. .

Systemic thromboembolic adverse events in patients treated with intravitreal anti-VEGF drugs for neovascular age-related macular degeneration: an update.

Introduction: Intravitreal anti-VEGF is the most effective therapy for wet AMD, although systemic effects on the endothelium cannot be excluded. Areas covered: The purpose of this review was to evaluate risk of thromboembolic events associated with intravitreal anti-VEGF. Expert opinion: Current data are insufficient to confirm the safety of these compounds, due to the paucity of specific studies. Thus, pharmacovigilance for all anti-VEGF should be improved to verify the true role of anti-VEGF in the occurrence of systemic adverse events.

One-Year Safety and Performance Assessment of the Argus II Retinal Prosthesis: A Postapproval Study.

IMPORTANCE: The Argus II Retinal Prosthesis System is indicated for patients with vision loss due to severe to profound outer retinal degeneration, a group with few treatment options. OBJECTIVES: To collect postapproval safety and visual function data for the Argus II. DESIGN, SETTING, AND PARTICIPANTS: Multicenter, postapproval clinical trial conducted at 9 sites in Germany and Italy. Data were collected from December 2, 2011, to September 30, 2017, and patients were followed-up for 12 months or longer. Patients were 25 years or older with severe to profound outer retinal degeneration, some residual light perception or the ability of the retina to respond to electrical stimulation, and a history of useful form vision and were already planning to undergo Argus II implantation. MAIN OUTCOMES AND MEASURES: The primary end point of this study was the nature and rate of adverse events. Secondary end points included 3 visual function tests: square localization (SL), direction of motion, and grating visual acuity (GVA). RESULTS: Forty-seven patients were followed for 12 months or longer after implant. Mean (SD) age was 56 (12) years, 37 (79%) had retinitis pigmentosa, and 27 (57%) were male. Through the first 12 months postimplantation, 23 patients (49%) experienced 51 nonserious adverse events and 12 (26%) experienced 13 serious adverse events (SAEs), 9 of which were judged to be related to the Argus II, and 4 of which were judged to be related to the procedure. The most common SAE was conjunctival erosion, reported in 4 patients. No significance testing was done for group analysis for the SL or direction-of-motion tests. When averaged across the group, patients' accuracy on the SL test, but not on the direction-of-motion test, appeared better when the Argus II was on than when it was switched off. For GVA, more patients at each point in time achieved the 2.9 GVA cutoff in the implanted eye when the Argus II was on compared with it switched off. CONCLUSIONS AND RELEVANCE: Safety and visual function outcomes in this clinical practice setting cohort of patients with Argus II implants were consistent with previously reported results. Longer follow-up of these patients and data from additional patients are required to better outline the risks and benefits of this approach to addressing blindness secondary to severe-to-profound outer retinal degeneration. TRIAL REGISTRATION: ClinicalTrials.gov identifier: NCT01490827.