68 research outputs found
Posterior subhyaloid precipitates in cytomegalovirus retinitis
This study aims to report a novel finding of posterior subhyaloid precipitates (PSPs) in two patients with cytomegalovirus (CMV) retinitis.
A small case series was conducted.
Clinical findings, treatment, and follow-up of two patients with CMV and PSPs are presented.
Inflammatory precipitates may collect in the posterior subhyaloid space in acute CMV retinitis and resolve with treatment
Pharmacotherapy of retinal disease with visual cycle modulators
Introduction: Pharmacotherapy with visual cycle modulators (VCMs) is under investigation for retinitis pigmentosa (RP), Leber congenital amaurosis (LCA), Stargardt macular dystrophy (SMD) and nonexudative age-related macular degeneration (AMD), all blinding diseases that lack effective treatment options.
Areas covered: The authors review investigational VCMs, including oral retinoids, 9-cis-retinyl-acetate (zuretinol) and 9-cis-β-carotene, which restore 11-cis-retinal levels in RP and LCA caused by LRAT and RPE65 gene mutations, and may improve visual acuity and visual fields. Therapies for SMD aiming to decrease accumulation of toxic Vitamin A dimers and lipofuscin in the retina and retinal pigment epithelium (RPE) include C20-D3-vitamin A (ALK-001), isotretinoin, VM200, emixustat, and A1120. Mouse models of SMD show promising data for these treatments, though proof of efficacy in humans is currently lacking. Fenretinide and emixustat are investigational VCMs for dry AMD, though neither has been shown to reduce geographic atrophy or improve vision in human trials. A1120 prevents retinol transport into the RPE and may spare the side effects typically seen in VCMs (nyctalopia and chromatopsia) per mouse studies.
Expert opinion: Oral VCMs may be feasible treatment options for degenerative retinal diseases based on pre-clinical and some early clinical studies. Further trials are warranted to assess their efficacy and safety in humans
Stargardt macular dystrophy and evolving therapies
Introduction: Stargardt macular dystrophy (STGD1) is a hereditary retinal degeneration that lacks effective treatment options. Gene therapy, stem cell therapy, and pharmacotherapy with visual cycle modulators (VCMs) and complement inhibitors are discussed as potential treatments.
Areas covered: Investigational therapies for STGD1 aim to reduce toxic bisretinoids and lipofuscin in the retina and retinal pigment epithelium (RPE). These agents include C20-D3-vitamin A (ALK-001), isotretinoin, VM200, emixustat, and A1120. Avacincaptad pegol is a C5 complement inhibitor that may reduce inflammation-related RPE damage. Animal models of STGD1 show promising data for these treatments, though proof of efficacy in humans is lacking. Fenretinide and emixustat are VCMs for dry AMD and STGD1 that failed to halt geographic atrophy progression or improve vision in trials for AMD. A1120 prevents retinol transport into RPE and may spare side effects typically seen with VCMs (nyctalopia and chromatopsia). Stem cell transplantation suggests potential biologic plausibility in a phase I/II trial. Gene therapy aims to augment the mutated ABCA4 gene, though results of a phase I/II trial are pending.
Expert opinion: Stem cell transplantation, ABCA4 gene therapy, VCMs, and complement inhibitors offer biologically plausible treatment mechanisms for treatment of STGD1. Further trials are warranted to assess efficacy and safety in humans
Worldwide Argus II implantation: recommendations to optimize patient outcomes
Abstract
Background
A position paper based on the collective experiences of Argus II Retinal Prosthesis System investigators to review strategies to optimize outcomes in patients with retinitis pigmentosa undergoing retinal prosthesis implantation.
Methods
Retinal surgeons, device programmers, and rehabilitation specialists from Europe, Canada, Middle East, and the United States were convened to the first international Argus II Investigator Meeting held in Ann Arbor, MI in March 2015. The recommendations from the collective experiences were collected. Factors associated with successful outcomes were determined.
Results
Factors leading to successful outcomes begin with appropriate patient selection, expectation counseling, and preoperative retinal assessment. Challenges to surgical implantation include presence of staphyloma and inadequate Tenon’s capsule or conjunctiva. Modified surgical technique may reduce risks of complications such as hypotony and conjunctival erosion. Rehabilitation efforts and correlation with validated outcome measures following implantation are critical.
Conclusions
Bringing together Argus II investigators allowed the identification of strategies to optimize patient outcomes. Establishing an on-line collaborative network will foster coordinated research efforts to advance outcome assessment and rehabilitation strategies.http://deepblue.lib.umich.edu/bitstream/2027.42/134581/1/12886_2016_Article_225.pd
Intravitreal Ranibizumab for Macular Edema Secondary to Juxtafoveal Retinal Telangiectasia Type 1A
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Surgical Observations from the First 120 Cases of Subretinal Gene Therapy for Inherited Retinal Degenerations
To report surgical observations formulated during the first 120 cases of subretinal gene therapy in patients with inherited retinal degenerations (IRDs).
A two-surgeon team compiled surgical observations and formulated surgical pearls based on the consecutive cases of subretinal viral vector injection in patients enrolled in clinical trials focusing on choroideremia, achromatopsia, and RPGR-associated retinitis pigmentosa, as well as patients with RPE65-associated Leber congenital amaurosis receiving FDA-approved voretigene neparvovec-rzyl therapy.
120 subretinal surgeries were performed by a two-surgeon team. Key anatomical features pertinent to surgical management were noted and are described in this manuscript. Surgical decision making for successful subretinal administration of viral vectors and management of potential surgical challenges were formulated.
Lessons learned during subretinal gene therapy cases may be helpful to other surgeons entering clinical trials or performing post-approval gene therapy administration. Surgical pearls outlined in this manuscript may also be helpful for other targeted subretinal therapies such as cellular transplantation or retinal prosthesis implantation
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Successes and Challenges of Retinal Implants for Profound Visual Loss From Outer Retinal Degeneration
Self-Inflicted Laser-Induced Retinopathy
This photo essay details a patient with self-inflicted laser-induced retinal injury progressing to full-thickness macular holes in both eyes. A 40-year-old patient presented after a self-inflicted injury by a handheld class 3 blue laser (450 nm) he purchased on the internet. The patient reported shining the laser through a window, which reflected the beam back into his eyes. Visual acuity was measured at 20/400 in both eyes. The initial fundus photographs revealed vitreous and preretinal hemorrhages in the right eye, and multiple yellow-white fresh laser burns in the macula of the left eye. Optical coherence tomography (OCT) showed preretinal hemorrhage in the right eye and retinal disruption with preretinal hyper-reflective lesion in the left eye. After one month, his vision deteriorated to finger counting in each eye. He developed a full-thickness macular hole and hyperfluorescent curvilinear streaks in the superior maculae in both eyes. OCT images showed retinal pigment epithelium clumping and outer retinal atrophy in curvilinear streak areas in both eyes, which point to self-inflicted injury. This case illustrates laser-pointer-induced retinopathy and reinforces the necessity of public education on the dangers of utilizing handheld lasers without eye protection
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Which Dry Eye? The Case for Precise Diagnostic Terminology in Ophthalmology
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