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Best’s disease is a relatively rare form of macular dystrophy, characterized by bilateral staged course. The cases of the choroidal neovascular membrane (XNM) formation in the course of the disease development are described, however the true frequency of this complication and the management of such patients are not determined. In recent years, a new promising method of examination — OCT — angiography (OCTA) — has been actively introduced into clinical practice. Non-invasive character and contactlessness of OKTA open wide possibilities of its application in various pathologies, in particular, in pediatric ophthalmology. The purpose of this work was to assess the prospects for using OCTA in the diagnosis and monitoring of Best’s disease. Patients and methods. The data of the standard complex ophthalmological examination and OCTA of the posterior pole of the eye were analyzed in 5 patients with Best’s disease — 4 children aged 5 to 12 years and the 25-year-old woman observed for this disease from childhood. Results. In all patients, the disease was bilateral. In 1 of 9 eyes (1 out of 10 eyes was excluded from the analysis due to the presence of retinal detachment at the time of examination) the disease was at the vitelliform stage, in 2 — at the pseudohypopyon, in 5 — at the vitelliruptive stage, in 1 — at the atrophic stage. Visual acuity at the time of the examination was from 0.02 to 1.0. Clinically, the presence of CNM could be assumed in 4 eyes. According to OCTA CNM was detected in 7 cases, in 3 of them signs of membrane activity were noted. Сonclusion. OCTA provide detection that the formation of СNM in the Best’s disease occured already at the pseudohypopyon stage and in half of cases was not accompanied by the development of clinical symptoms. The study allows to assess the localization, size and activity of the membrane, which is necessary for monitoring and determining the tactics of treatment of this complication. Carrying out further research will determine the true frequency, risk factors for development and features of CNM in Best’s disease, which will allow developing optimal tactics for conducting such patients.

Best’s Disease is a macular dystrophy characterized by a lipofuscin accumulation on the retinal pigment epithelium. Five stages have been described based on fundus examination, including choroidal neovascularization. We report a case of a 59-years-old male, presented to the ophthalmologic department with visual loss in both eyes. Fundus examination revealed vitelliform lesions in both maculas. Fluorescein angiography showed a choroidal neovascularization in the left eye. The electrooculogram confirmed the diagnosis of Best's disease. Choroidal neovascularization is a rare complication of Best’s Disease in late stages. The most effective therapeutic options are photodynamic therapy with veteporfirin and antiangiogenic therapy.

Objective: To describe a case of adult-onset foveomacular vitelliform dystrophy (AOFVD). Method: This is a case report. Results: A 22-year-old female presented with painless blurring of vision and metamorphopsia 3 days prior to consultation. There were 2 similar episodes in the past that spontaneously resolved after 2 to 4 weeks. Visual acuity (VA) was 20/50 in the right eye (OD) and 20/40 in the left (OS), both best corrected to 20/25. Dilated-fundus examination revealed a discrete area of mixed hypoand hyperpigmentation 1 disc diameter over the fovea in OD and a solitary round hypopigmented lesion with a hyperpigmented border 3 to 4 disc diameters on the fovea in OS. Fluorescein angiography (FA) revealed an area of hyperfluorescence surrounded by a rim of hypoflourescence in OD and an area of blocked fluorescence with subtle hyperfluorescence superior to the lesion in OS, both of which did not increase in size and intensity toward the late phases. Optical coherence tomography (OCT) revealed neurosensory detachment in both eyes. Electrooculogram (EOG) was normal with Arden ratio of 0.91. VA returned to 20/25 in both eyes, and repeat fundus photography showed no change in the characteristics of the lesions. Conclusion: Differential diagnosis of a hypopigmented macular lesion in the young with self-limited blurring of vision should include AOFVD. FA, OCT, and EOG can help distinguish AOFVD from Best’s disease or other similar macular conditions.

Best′s vitelliform macular dystrophy is a hereditary form of progressive macular dystrophy that can be complicated by choroidal neovascularization. Authors report successful treatment of choroidal neovascularization with intravitreal bevacizumab in one such eye in an ′adult′ Indian male with visual improvement. A 23-year-old male presented with diminution of vision in the right eye for the past sixteen months. Visual acuity was 20/400 in the that eye. After three consecutive intravitreal injections of bevacizumab (1.25 mg/0.05 ml), vision improved to 20/120. Seven months following the last injection of bevacizumab, fundus appeared stable and visual acuity was maintained. No drug-related ocular or systemic side effects were encountered. To the best of our knowledge (PubMed search), this is the first report of its kind in an adult Indian patient. Intravitreal bevacizumab appears to be a promising and cost-effective modality of treatment in such eyes with potential for improvement in vision. However, a long-term follow-up is warranted.