Electronic retinal prosthesis for severe loss of vision in geographic atrophy in age-related macular degeneration: First-in-human use.

BACKGROUND: To date there are yet no available approved therapies for Geographic Atrophy (GA) secondary to age-related macular degeneration (AMD). METHODS: Single site, non-randomized safety and efficacy study presenting the preliminary results in a cohort of five late stage AMD (GA) patients successfully implanted with the Argus II Retinal Prosthesis System (Second Sight Medical Products Inc., Sylmar, CA, USA). Extensive fundus imaging including retinal photographs from which the GA area was measured. A combination of custom and traditional tests designed for very low vision subjects assessed visual function in study subjects. A Functional Low-Vision Observer Rated Assessment was carried out to evaluate the impact of the system on the subject's daily life. In addition, a study to evaluate structural characteristics of the visual cortex of the brain was performed in one subject using magnetic resonance imaging. RESULTS: Seven device-related adverse events were reported, four of which were classed as serious adverse events. Retinal detachment was reported in three patients and was successfully treated within 12 months of onset. Testing showed an improvement in visual function in three of five patients with the system turned on. Magnetic resonance imaging assessed in one patient after implantation indicates a selective increase in cortical myelin and thickness in visual brain regions 1 year post implantation. CONCLUSIONS: Epiretinal prostheses can successfully be implanted in those affected by GA secondary to late-stage AMD and can elicit visual percepts by electrical stimulation of residual neuroretinal elements and improve basic visual function in those affected.

Effects of the Argus II Retinal Prosthesis System on the Quality of Life of Patients With Ultra-Low Vision Due to Retinitis Pigmentosa: Protocol for a Single-Arm, Mixed Methods Study.

BACKGROUND: Retinitis pigmentosa is an incurable, degenerative retinal condition causing progressive sight loss, significantly affecting patients' quality of life. The Argus II Retinal Prosthesis is a surgically implanted medical device that delivers electrical stimulation to the retina. It is intended to produce a form of artificial vision for blind people with severe-to-profound retinitis pigmentosa by stimulating the remaining viable retinal cells to induce visual perception. This study has been initiated by National Health Service England's Commissioning through Evaluation program and funded through the National Institute of Health Research of the United Kingdom. OBJECTIVE: The aim of this study was to assess the effect of the Argus II device on patient's daily activities and quality of life. METHODS: This protocol is a prospective, single-arm, open-label, mixed methods study on 10 consecutive participants receiving the Argus II device. The patient representatives played an integral role in the design of this study. Eligibility criteria include ultra-low vision in both eyes as a result of end-stage retinitis pigmentosa and a willingness and capacity to complete the postimplantation rehabilitation program. Participants will be interviewed by independent researchers at baseline and 12 months later by using a semistructured, in-depth approach, alongside validated questionnaires (Impact of Vision Impairment-Very Low Vision, 5-level EuroQoL-5 dimensions scale, EuroQoL-visual analog scale, and Hospital Anxiety and Depression Scale) and a bespoke device-related questionnaire, which includes questions about users' experiences with the procedure, the device, and rehabilitation. The effect of the device on patients' functional vision and activities of daily living will be assessed by vision rehabilitation specialists using a set of tests measured on an ordinal scale (eg, ability to locate objects and avoid obstacles). Clinical outcomes include full-field stimulus light threshold, square localization, direction of motion, grating visual acuity, Landolt-C, procedural success, and adverse events. Qualitative and quantitative outcomes will be linked in a single database to enable individual participant measures to be considered in toto, comparing baseline to the final review. RESULTS: This study was approved by the local ethics committee on April 24, 2019 (London-Camberwell St. Giles Research Ethics Committee, reference 19/LO/0429). It has also been approved by the Health Research Authority and Health and Care Research Wales. At the time of protocol writing, Argus II was available for use in the United Kingdom; however, the manufacturer recently withdrew the Argus II device from sale in the United Kingdom. Therefore, the study is not going ahead at this time. CONCLUSIONS: The mixed methods approach provides a rich and in-depth assessment of the effect of the device on participants' quality of life. Despite the work not going ahead, the publication of this publicly funded protocol is important for researchers planning similar work. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): PRR1-10.2196/17436.

Macular fibrosis in Coats' disease demonstrated by Optical Coherence Tomography Angiography - new data toward pathophysiology.

PURPOSE: To analyze if Segmented Swept-Source Optical Coherence Tomography Angiography (SS OCT-A) can provide additional information on morphology and pathophysiology of macular fibrosis in Coats' patients. METHODS: A consecutive case series of three male patients (5, 7 and 15 years old), with Coats' disease-related macular fibrosis (stage 2b-2 patients, 3b-1 patient). SS OCT-A 3×3 mm macular scans of affected eyes were performed. RESULTS: In all three cases the inner portion of macular fibrosis displayed a dense network of vessels, continuing into deeper layers. This structure was similar to that observed in retinal angiomatous proliferations (RAP). There was associated loss of the foveal avascular zone. In one case we observed evolution of the lesion. CONCLUSION: SS-OCT imaging of macular fibrosis in Coats' disease reveals a distinct intralesional vascular structure with elements resembling RAP, probably developing as a secondary process.

Initial results from a first-in-human gene therapy trial on X-linked retinitis pigmentosa caused by mutations in RPGR.

Retinal gene therapy has shown great promise in treating retinitis pigmentosa (RP), a primary photoreceptor degeneration that leads to severe sight loss in young people. In the present study, we report the first-in-human phase 1/2, dose-escalation clinical trial for X-linked RP caused by mutations in the RP GTPase regulator (RPGR) gene in 18 patients over up to 6 months of follow-up (https://clinicaltrials.gov/: NCT03116113). The primary outcome of the study was safety, and secondary outcomes included visual acuity, microperimetry and central retinal thickness. Apart from steroid-responsive subretinal inflammation in patients at the higher doses, there were no notable safety concerns after subretinal delivery of an adeno-associated viral vector encoding codon-optimized human RPGR (AAV8-coRPGR), meeting the pre-specified primary endpoint. Visual field improvements beginning at 1 month and maintained to the last point of follow-up were observed in six patients.

ADVERSE EVENTS OF THE ARGUS II RETINAL PROSTHESIS: Incidence, Causes, and Best Practices for Managing and Preventing Conjunctival Erosion.

PURPOSE: To analyze and provide an overview of the incidence, management, and prevention of conjunctival erosion in Argus II clinical trial subjects and postapproval patients. METHODS: This retrospective analysis followed the results of 274 patients treated with the Argus II Retinal Prosthesis System between June 2007 and November 2017, including 30 subjects from the US and European clinical trials, and 244 patients in the postapproval phase. Results were gathered for incidence of a serious adverse event, incidence of conjunctival erosion, occurrence sites, rates of erosion, and erosion timing. RESULTS: Overall, 60% of subjects in the clinical trial subjects versus 83% of patients in the postapproval phase did not experience device- or surgery-related serious adverse events. In the postapproval phase, conjunctival erosion had an incidence rate of 6.2% over 5 years and 11 months. In 55% of conjunctival erosion cases, erosion occurred in the inferotemporal quadrant, 25% in the superotemporal quadrant, and 20% in both. Sixty percent of the erosion events occurred in the first 15 months after implantation, and 85% within the first 2.5 years. CONCLUSION: Reducing occurrence of conjunctival erosion in patients with the Argus II Retinal Prosthesis requires identification and minimization of risk factors before and during implantation. Implementing inverted sutures at the implant tabs, use of graft material at these locations as well as Mersilene rather than nylon sutures, and accurate Tenon's and conjunctiva closure are recommended for consideration in all patients.

FIRST-IN-HUMAN CLINICAL STUDY TO INVESTIGATE THE EFFECTIVENESS AND SAFETY OF PARS PLANA VITRECTOMY SURGERY USING A NEW HYPERSONIC TECHNOLOGY.

PURPOSE: Investigate the effective performance and safety of a new hypersonic vitrector technology. METHODS: Postapproval, prospective, single-arm, noncomparative, open-label study at one clinical site in India. INDICATIONS: macular hole (9/20), vitreous hemorrhage (7/20), vitreomacular traction (3/20), and vitreomacular traction with pseudomacular hole (1/20). Safety endpoints included intraoperative and postoperative adverse events. Effective performance endpoints were surgeon-rated effectiveness, range of surgical time, and device settings. Other performance measures were preoperative and postoperative best-corrected visual acuity, slit-lamp and indirect ophthalmoscopy, applanation tonometry, color fundus photography, fundus fluorescein angiography, and spectral domain optical coherence tomography. RESULTS: Core vitreous removal (20/20 subjects), peripheral vitreous removal (18/20), and posterior vitreous detachment induction (13/15) surgeries were successfully completed. Total surgical time was 22.5 minutes to 106 minutes. Serious adverse events through 3 months were 2 device-associated retinal tears and detachment (one intraoperative) and one unrelated postoperative enlargement of macular hole with subretinal fluid. CONCLUSION: This first-in-human study suggests that this new hypersonic vitrector technology is a promising alternative to commercially available guillotine vitrectors. The hypersonic vitrector was effective in core vitreous removal in all cases. Larger-scale studies are required to expand on our initial findings for induction of a posterior vitreous detachment or peripheral vitrectomy.

Classification and Guidelines for Widefield Imaging: Recommendations from the International Widefield Imaging Study Group.

PURPOSE: To summarize the results of a consensus meeting aimed at defining terminology for widefield imaging across all retinal imaging methods and to provide recommendations for the nomenclature used to describe related images. DESIGN: An international panel with expertise in retinal imaging was assembled to define consensus terminology for widefield imaging and associated terminology. PARTICIPANTS: A panel of retina specialists with expertise in retinal imaging. METHODS: Before the consensus meeting, a set of 7 images acquired with a range of imaging methods and representing both healthy and diseased eyes was circulated to the expert panel for independent assignment of nomenclature for each example. The outputs were assembled and used as the starting point for discussions occurring at a subsequent roundtable meeting. The anatomic location, field of view, and perspective provided by each image example was reviewed. A process of open discussion and negotiation was undertaken until unanimous terminology for widefield imaging was achieved. MAIN OUTCOME MEASURES: Definitions of widefield imaging applicable to multiple imaging methods. RESULTS: Across a range of different imaging methods, the expert panel identified a lack of uniform terminology being used in recent literature to describe widefield images. The panel recommended the term widefield be limited to images depicting retinal anatomic features beyond the posterior pole, but posterior to the vortex vein ampulla, in all 4 quadrants. The term ultra widefield was recommended to describe images showing retinal anatomic features anterior to the vortex vein ampullae in all 4 quadrants. The definitions were recommended over other device-specific terminology. CONCLUSIONS: A consistent nomenclature for widefield imaging based on normal anatomic landmarks that is applicable to multiple retinal imaging methods has been proposed by the International Widefield Imaging Study Group. The panel recommends this standardized nomenclature for use in future publications.

SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY ASSESSMENT OF FELLOW EYES IN COATS DISEASE.

PURPOSE: To assess foveal and parafoveal vasculature at superficial capillary plexus (SCP), deep capillary plexus, and choriocapillaris using optical coherence tomography angiography in the fellow eyes of patients with Coats disease. METHODS: Observational and prospective case series. Thirteen patients with unilateral Coats and 14 healthy age- and sex-matched controls were consecutively recruited at Manchester Royal Eye Hospital and the Department of Ophthalmology of San Raffaele Hospital. Both groups underwent complete ophthalmologic examination, including optical coherence tomography angiography (Topcon Corp) 3 mm × 3 mm scans. Images were imported into ImageJ software and binarized; foveal avascular zone area was manually outlined and vessel density analyzed in inner (foveal) and outer (parafoveal) areas of SCP, deep capillary plexus, and choriocapillaris. RESULTS: Fellow eyes disclosed a significant increase in the foveal vessel density of SCP (P = 0.04); in particular, superior and temporal quadrants showed more marked alterations (P = 0.02 and 0.04, respectively). Analysis of foveal avascular zone area revealed a significant enlargement in the SCP (P = 0.04). No correlation was found between fellow eyes and the stage of affected eyes. CONCLUSION: Fellow eyes of Coats patients carry quantitative foveal vascular alterations at SCP. These may represent markers of altered inner blood-retinal barrier, due to a bilateral defect in midcapillary angiogenesis.

CAPILLARY NETWORK ALTERATIONS IN X-LINKED RETINOSCHISIS IMAGED ON OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To assess foveal and parafoveal vasculature at the superficial capillary plexus, deep capillary plexus, and choriocapillaris of patients with X-linked retinoschisis by means of optical coherence tomography angiography. METHODS: Six patients with X-linked retinoschisis (12 eyes) and seven healthy controls (14 eyes) were recruited and underwent complete ophthalmologic examination, including best-corrected visual acuity, dilated fundoscopy, and 3 × 3-mm optical coherence tomography angiography macular scans (DRI OCT Triton; Topcon Corp). After segmentation and quality review, optical coherence tomography angiography slabs were imported into ImageJ 1.50 (NIH; Bethesda) and digitally binarized. Quantification of vessel density was performed after foveal avascular zone area measurement and exclusion. Patients were additionally divided into "responders" and "nonresponders" to dorzolamide therapy. RESULTS: Foveal avascular zone area resulted markedly enlarged at the deep capillary plexus (P < 0.001), particularly in nonresponders. Moreover, patients disclosed a significant deep capillary plexus rarefaction, when compared with controls (P: 0.04); however, a subanalysis revealed that this damage was limited to the fovea (P: 0.006). Finally, the enlargement of foveal avascular zone area positively correlated with a decline in best-corrected visual acuity (P: 0.01). CONCLUSION: Prominent foveal vascular impairment is detectable in the deep capillary plexus of patients with X-linked retinoschisis. Our results correlate with functional outcomes, suggesting a possible vascular role in X-linked retinoschisis clinical manifestations.

Improvements in vision-related quality of life in blind patients implanted with the Argus II Epiretinal Prosthesis.

BACKGROUND: The purpose of this analysis is to report the change in quality of life (QoL) after treatment with the Argus II Epiretinal Prosthesis in patients with end-stage retinitis pigmentosa. METHODS: The Vision and Quality of Life Index (VisQoL) was used to assess changes in QoL dimensions and overall utility score in a prospective 30-patient single-arm clinical study. VisQoL is a multi-attribute instrument consisting of six dimensions (injury, life, roles, assistance, activity and friendship) that may be affected by visual impairment. Within each dimension, patients were divided into two groups based on how much their QoL was affected by their blindness at baseline (moderate/severe or minimal). Outcomes were compared within each dimension sub-group between baseline and the combined follow-up periods using the Friedman test. In addition, data from the six dimensions were combined into a single utility score, with baseline data compared to the combined follow-up periods. RESULTS: Overall, 80 per cent of the patients reported difficulty in one or more dimensions pre-implant. Composite VisQoL utility scores at follow-up showed no statistically significant change from baseline; however, in three of the six VisQoL dimensions (injury, life and roles), patients with baseline deficits showed significant and lasting improvement after implantation with Argus II. In two of the three remaining dimensions (assistance and activity), data trended toward an improvement. In the final VisQoL dimension (friendship), none of the patients reported baseline deficits, suggesting that patients had largely adjusted to this attribute. CONCLUSION: Patients whose vision negatively affected them with respect to three VisQoL dimensions (that is, getting injured, coping with the demands of their life and fulfilling their life roles) reported significant improvement in QoL after implantation of the Argus II retinal prosthesis. Furthermore, the benefit did not deteriorate at any point during the 36-month follow-up, suggesting a long-term, durable improvement.

Performance of real-world functional vision tasks by blind subjects improves after implantation with the Argus® II retinal prosthesis system.

BACKGROUND: The main objective of this study was to test Argus II subjects on three real-world functional vision tasks. DESIGN: The study was designed to be randomized and prospective. Testing was conducted in a hospital/research laboratory setting at the various participating centres. PARTICIPANTS: Twenty eight Argus II subjects, all profoundly blind, participated in this study. METHODS: Subjects were tested on the three real-world functional vision tasks: Sock Sorting, Sidewalk Tracking and Walking Direction Discrimination task MAIN OUTCOME MEASURES: For the Sock Sorting task, percentage correct was computed based on how accurately subjects sorted the piles on a cloth-covered table and on a bare table. In the Sidewalk Tracking task, an 'out of bounds' count was recorded, signifying how often the subject veered away from the test course. During the Walking Direction Discrimination task, subjects were tested on the number of times they correctly identified the direction of testers walking across their field of view. RESULTS: The mean percentage correct OFF versus ON for the Sock Sorting task was found to be significantly different for both testing conditions (t-test, P < 0.01). On the Sidewalk Tracking task, subjects performed significantly better with the system ON than they did with the system OFF (t-test, P < 0.05). Eighteen (18) of 27 subjects (67%) performed above chance with the system ON, and 6 (22%) did so with system OFF on the Walking Direction Discrimination task. CONCLUSIONS: Argus II subjects performed better on all three tasks with their systems ON than they did with their systems OFF.

Five-Year Safety and Performance Results from the Argus II Retinal Prosthesis System Clinical Trial.

PURPOSE: The Argus II Retinal Prosthesis System (Second Sight Medical Products, Inc, Sylmar, CA) was developed to restore some vision to patients blind as a result of retinitis pigmentosa (RP) or outer retinal degeneration. A clinical trial was initiated in 2006 to study the long-term safety and efficacy of the Argus II System in patients with bare or no light perception resulting from end-stage RP. DESIGN: Prospective, multicenter, single-arm clinical trial. Within-patient controls included the nonimplanted fellow eye and patients' native residual vision compared with their vision with the Argus II. PARTICIPANTS: Thirty participants in 10 centers in the United States and Europe. METHODS: The worse-seeing eye of blind patients was implanted with the Argus II. Patients wore glasses mounted with a small camera and a video processor that converted images into stimulation patterns sent to the electrode array on the retina. MAIN OUTCOME MEASURES: The primary outcome measures were safety (the number, seriousness, and relatedness of adverse events) and visual function, as measured by 3 computer-based, objective tests. Secondary measures included functional vision performance on objectively scored real-world tasks. RESULTS: Twenty-four of 30 patients remained implanted with functioning Argus II Systems at 5 years after implantation. Only 1 additional serious adverse event was experienced after the 3-year time point. Patients performed significantly better with the Argus II on than off on all visual function tests and functional vision tasks. CONCLUSIONS: The 5-year results of the Argus II trial support the long-term safety profile and benefit of the Argus II System for patients blind as a result of RP. The Argus II is the first and only retinal implant to have market approval in the European Economic Area, the United States, and Canada.

Swept-Source Optical Coherence Tomography Angio™ (Topcon Corp, Japan): Technology Review.

Optical coherence tomography (OCT) angiography (OCTA) is a novel, noninvasive, three-dimensional imaging technique that allows for the visualization of intravascular flow in the microvasculature. Swept-source OCT technology utilizes longer-wavelength infrared light than conventional spectral-domain OCT. This enables improved penetration into tissue and imaging through optical opacities and is invisible to the subject. Topcon has recently developed an innovative OCTA algorithm, OCTARA (OCTA Ratio Analysis), which benefits from being paired with swept-source OCT. OCTARA aims to provide improved detection sensitivity of low blood flow and reduced motion artifacts without compromising axial resolution. In this chapter, we describe the implementation of OCTARA with swept-source OCT technology, the technical specifications of acquisition (e.g. the number of scans, area of examination field, etc.) along with the algorithm's function and principles for analysis of B-scan data to achieve angiographic visualization. Examples of OCTA scans performed using the OCTARA algorithm and a comparison of these scans with images obtained using other technologies are also presented.

Swept-Source Optical Coherence Tomography Angiography of Paediatric Macular Diseases.

PURPOSE: To describe the optical coherence tomography (OCT) angiography (OCTA) features of paediatric macular pathologies. METHODS: Retrospective serial case reports of patients who underwent routine clinical examination and OCTA of the posterior pole using both a DRI OCT Atlantis prototype and Triton Swept-Source OCT. When considered necessary, imaging was performed using Optos wide-field imaging or another non-invasive imaging system. The findings were compared with the current literature. RESULTS: Three cases with X-linked retinoschisis, 2 with epiretinal membrane, 1 with Best disease and 2 with Coats disease are fully illustrated. CONCLUSION: OCTA is an effective, non-invasive imaging technique that can offer additional information regarding the morphologies and vascular characteristics of macular lesions in paediatric ophthalmology. Because of the rarity and characteristics of many paediatric macular pathologies, further multi-centric research is required with regard to the utilisation and features of OCTA imaging.

New Findings in Diabetic Maculopathy and Proliferative Disease by Swept-Source Optical Coherence Tomography Angiography.

PURPOSE: To describe the optical coherence tomography (OCT) angiography (OCTA) features of diabetic retinopathy. METHODS: Retrospective serial case reports were examined of patients who underwent routine clinical examination and OCTA with both DRI OCT Atlantis prototype and Triton Swept-Source OCT of the posterior pole and mid-periphery. When considered necessary, fluorescein fundus angiography (FFA) with OPTOS California wide-field imaging was performed. The findings were compared with the current literature. RESULTS: Forty-three consecutive patients (86 eyes) were evaluated. Fourteen of these patients (28 eyes) underwent an additional FFA examination due to advanced retinopathy signs, such as diabetic macular edema, ischemia or neovascularization (NV). OCTA was able to detect the microvascular lesions observed on color fundus images in the whole sample. Thirty-six of the 86 eyes showed foveal avascular zone enlargement on OCTA. Microvascular lesions, diabetic macular edema, and NV of the optic disc observed on FFA were also detected on OCTA in all cases (28/28 eyes). Features of NV elsewhere were detected on FFA in 16/28 eyes. Ten of the 16 eyes had signs of NV within the 100 central degrees, and OCTA was able to detect these signs in 9 of the eyes. CONCLUSION: OCTA is an effective noninvasive imaging technique that can provide additional information regarding the localization and morphology of vascular lesions in all cases of NV of the optic disc and in more than half of cases of NV elsewhere, suggesting that it is a noninferior technique for the study of posterior pole alterations compared with FFA, which remains the gold standard and is fundamental for the study of the retinal periphery.

An analysis of observer-rated functional vision in patients implanted with the Argus II Retinal Prosthesis System at three years.

OBJECTIVE: The purpose of this analysis was to compare observer-rated tasks in patients implanted with the Argus II Retinal Prosthesis System, when the device is ON versus OFF. METHODS: The Functional Low-Vision Observer Rated Assessment (FLORA) instrument was administered to 26 blind patients implanted with the Argus II Retinal Prosthesis System at a mean follow-up of 36 months. FLORA is a multi-component instrument that consists in part of observer-rated assessment of 35 tasks completed with the device ON versus OFF. The ease with which a patient completes a task is scored using a four-point scale, ranging from easy (score of 1) to impossible (score of 4). The tasks are evaluated individually and organised into four discrete domains, including 'Visual orientation', 'Visual mobility', 'Daily life and 'Interaction with others'. RESULTS: Twenty-six patients completed each of the 35 tasks. Overall, 24 out of 35 tasks (69 per cent) were statistically significantly easier to achieve with the device ON versus OFF. In each of the four domains, patients' performances were significantly better (p < 0.05) with the device ON versus OFF, ranging from 19 to 38 per cent improvement. CONCLUSION: Patients with an Argus II Retinal Prosthesis implanted for 18 to 44 months (mean 36 months), demonstrated significantly improved completion of vision-related tasks with the device ON versus OFF.

TRANSCLERAL DRAINAGE OF SUBRETINAL FLUID, ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR, AND WIDE-FIELD IMAGING-GUIDED LASER IN COATS EXUDATIVE RETINAL DETACHMENT.

PURPOSE: To evaluate the safety and efficacy of combined transscleral drainage of subretinal fluid (SRF) with intravitreal bevacizumab and laser photocoagulation in the management of advanced Coats disease (Stage 3) with exudative retinal detachment. DESIGN: Retrospective interventional case series. METHODS: Retrospective case review of eight eyes in eight children with advanced Coats disease manifested as total or subtotal retinal detachment. All eyes initially underwent surgical drainage of exudative SRF followed by intravitreal injection of bevacizumab and laser photocoagulation. Patients were subsequently followed up for up to 60 months. RESULTS: In all eyes, after SRF drainage and administration of one to two intravitreal injections, SRF was completely eliminated. Patients required up to four sessions of laser photocoagulation. Retinal detachment consequently reduced with all patients showing total retinal reattachment and resolution of the subretinal exudates. At the last follow-up, no patient showed recurrent SRF and no ocular complications related to bevacizumab nor evidence of further disease progression were noted. CONCLUSION: The authors present a new therapeutic approach that allows for the first time successful treatment of advanced cases of exudative retinal detachment in Coats disease without the need for vitrectomy. Transscleral drainage of SRF accompanied by anti-vascular endothelial growth factor injection and laser photocoagulation appears to be successful in halting progression of advanced Coats disease with exudative detachment and a less invasive approach when compared with conventional management.

Long-Term Results from an Epiretinal Prosthesis to Restore Sight to the Blind.

PURPOSE: Retinitis pigmentosa (RP) is a group of inherited retinal degenerations leading to blindness due to photoreceptor loss. Retinitis pigmentosa is a rare disease, affecting only approximately 100 000 people in the United States. There is no cure and no approved medical therapy to slow or reverse RP. The purpose of this clinical trial was to evaluate the safety, reliability, and benefit of the Argus II Retinal Prosthesis System (Second Sight Medical Products, Inc, Sylmar, CA) in restoring some visual function to subjects completely blind from RP. We report clinical trial results at 1 and 3 years after implantation. DESIGN: The study is a multicenter, single-arm, prospective clinical trial. PARTICIPANTS: There were 30 subjects in 10 centers in the United States and Europe. Subjects served as their own controls, that is, implanted eye versus fellow eye, and system on versus system off (native residual vision). METHODS: The Argus II System was implanted on and in a single eye (typically the worse-seeing eye) of blind subjects. Subjects wore glasses mounted with a small camera and a video processor that converted images into stimulation patterns sent to the electrode array on the retina. MAIN OUTCOME MEASURES: The primary outcome measures were safety (the number, seriousness, and relatedness of adverse events) and visual function, as measured by 3 computer-based, objective tests. RESULTS: A total of 29 of 30 subjects had functioning Argus II Systems implants 3 years after implantation. Eleven subjects experienced a total of 23 serious device- or surgery-related adverse events. All were treated with standard ophthalmic care. As a group, subjects performed significantly better with the system on than off on all visual function tests and functional vision assessments. CONCLUSIONS: The 3-year results of the Argus II trial support the long-term safety profile and benefit of the Argus II System for patients blind from RP. Earlier results from this trial were used to gain approval of the Argus II by the Food and Drug Administration and a CE mark in Europe. The Argus II System is the first and only retinal implant to have both approvals.

Swept-source optical coherence tomography of retinal cavernous hemangioma: a new imaging modality.

The authors report a new, non-invasive diagnostic method in the diagnosis of retinal cavernous hemangioma (RCH). A 6-year-old girl was referred for a non-clearing retinal hemorrhage of 6 months' duration. Fourier-domain optical coherence tomography (FD-OCT) showed an intraretinal lesion with cystic-like internal appearance. Optical shadowing was present, preventing establishment of any subretinal component to the lesion. Swept-source OCT (SS-OCT) showed an intraretinal lesion consisting of a group of clearly defined grape-like caverns with overlying preretinal tissue. Wide-field fundus fluorescein angiography (WF-FFA) confirmed the diagnosis of RCH. SS-OCT was superior to FD-OCT in showing the internal anatomy of the RCH and allowing for the measurement of its structures, confirming the intraretinal location of the lesion and the presence of an associated preretinal tissue. SS-OCT may assist in cases in which hemorrhage prevents an accurate diagnosis by ophthalmoscopy or angiography, thus becoming an alternative imaging method to confirm the diagnosis of RCH while avoiding the risks of fluorescein angiography in children.