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.
Mutations in a novel gene, VMD2, encoding a protein of unknown properties cause juvenile-onset vitelliform macular dystrophy (Best's disease)
