Non-paraneoplastic related retinopathy: clinical challenges and review.

Autoimmune retinopathy (AIR) is a rare inflammatory condition characterized by progressive visual loss, abnormalities in visual fields and electroretinographic exams, along with presence of circulating anti-retinal antibodies. There are two main forms of AIR: paraneoplastic AIR (pAIR) and presumed non-paraneoplastic AIR (npAIR). NpAIR is considered a diagnosis of exclusion, since it is typically made after other causes of retinopathy have been investigated and the absence of malignancy is confirmed. Work-up of a npAIR case is challenging since there are no standartizaded protocols for diagnosis and treatment. The treatment regimen may vary from case to case, and it can be best guided by a set of parameters including electrophysiological responses, visual outcomes, and presence of anti-retinal antibodies. The purpose of this review is to summarize the principal clinical features, investigation, and management of npAIR.

INTRAVITREAL ZIV-AFLIBERCEPT FOR NEOVASCULAR AGE-RELATED MACULAR DEGENERATION: 52-Week Results.

PURPOSE: To evaluate the 52-week safety and efficacy of intravitreal ziv-aflibercept in patients with neovascular age-related macular degeneration. METHODS: All patients received three monthly intravitreal injections of 0.05 mL of ziv-aflibercept (1.25 mg) followed by a pro re nata regimen. The best-corrected visual acuity and spectral domain optical coherence tomography were obtained at baseline and monthly. Full-field and multifocal electroretinograms were obtained at baseline and 4, 13, 26, and 52 weeks. For some full-field electroretinography parameters, we calculated the differences between baseline and 52 weeks and then compared those differences between treated and untreated fellow eyes. RESULTS: Fifteen patients were included and 14 completed the 52-week follow-up. The mean best-corrected visual acuity improved from 0.95 ± 0.41 (20/200) at baseline to 0.75 ± 0.51 (20/125) logarithm of the minimum angle of resolution at 52 weeks (P = 0.0066). The baseline central retinal thickness decreased from 478.21 ± 153.48 µm to 304.43 ± 98.59 µm (P = 0.0004) at 52 weeks. Full-field electroretinography parameters used to assess retinal toxicity after intravitreal injections (rod response and oscillatory potentials) remained unchanged during follow-up. The average multifocal electroretinography macular response in 5° showed increased N1-P1 amplitude and decreased P1 implicit time (P < 0.05). One patient presented with intraocular inflammation after the seventh intravitreal procedure. CONCLUSION: The results suggested that intravitreal ziv-aflibercept might be safe and effective for treating neovascular age-related macular degeneration. More patients and a longer follow-up are needed to confirm the long-term outcomes of intravitreal ziv-aflibercept.

Gene therapy in inherited retinal degenerative diseases, a review.

Hereditary diseases of the retina represent a group of diseases with several heterogeneous mutations that have the common end result of progressive photoreceptor death leading to blindness. Retinal degenerations encompass multifactorial diseases such as age-related macular degeneration, Leber congenital amaurosis, Stargardt disease, and retinitis pigmentosa. Although there is currently no cure for degenerative retinal diseases, ophthalmology has been at the forefront of the development of gene therapy, which offers hope for the treatment of these conditions. This article will explore an overview of the clinical trials of gene supplementation therapy for retinal diseases that are underway or planned for the near future.

RETINAL RACEMOSE HEMANGIOMA WITH FOCAL MACULAR INVOLVEMENT.

PURPOSE: To report a case of racemose hemangioma with macular involvement and treated with anti-vascular endothelial growth factor. METHODS: Observational case report. RESULTS: JFS, a 31-year-old woman, presented with a complaint of blurred vision in her right eye over the preceding 2 months, worsening during the previous month. An examination conducted in May 2013 showed visual acuity of 20/30 in her right eye and 20/20 in her left eye, deteriorating to 20/40 in the right eye 1 month later. Retinography of the right eye revealed a dilated tortuous retinal vessel in the lower temporal arcade, affecting the macular region. Because of progressive deterioration in the patient's visual acuity, she was treated with 3 intravitreal bevacizumab injections, with an interval of 1 month between them. At the end of the proposed treatment, the patient was followed up on an outpatient basis for 12 months, with visual acuity of 20/20 in both eyes and no signs of retinal exudation throughout the entire follow-up period. CONCLUSION: Racemose hemangioma with focal macular involvement may lead to a progressive reduction in visual acuity because of exudation. Further studies need to be conducted to confirm the efficacy of bevacizumab injections to treat vascular malformation; however, this form of management does seem promising.