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.

Purpose: to present a clinical case of a child with hypertensive retinopathy developed against the background of undiagnosed pheochromacytoma and primarily regarded as neuroretinitis. The description of the case shows that a comprehensive examination of the child together with a pediatrician and endocrinologist allowed to identify the etiology of the pathological process and choose the right treatment strategy. Discussion. It is important to note that the alertness of the pediatric ophthalmologist in terms of the development of hypertensive retinal angiopathy is reduced. Arterial hypertension in children is much less common than in adults, and cases of hypertensive retinal lesions in children are rare. This can lead to a number of errors in the diagnosis and treatment of eye pathology, as well as underestimation of the severity of the General somatic condition of the child, in this connection it is important to emphasize the importance of close interdisciplinary cooperation in the examination and treatment of children of ophthalmic profile.

Studying immunological aspects of pathogenesis of retinopathy of prematurity (ROP) helps develop new approaches to prevention, rational diagnosis and increase of the effectiveness of treatment of the disease. Purpose: to analyze the level of growth factors in blood serum of premature infants belonging to the group of ROP risk at the pre-clinical stage and find out how this level is related to further development and course of the disease. Material and methods. 85 prematurely born infants of the ROP risk group were examined. The average gestational age at birth was 27.7 ± 2.2 weeks, the average birth weight - 1086.1 ± 266.1 g. The examination included dynamic ophthalmoscopy and determination of the content of VEGF-A in blood serum (using a flow cytometer BD FACS Canto II) and TGF- β1, IGF-I, IGF-II (by enzyme-linked immunosorbent assay (ELISA) using a c-test system Bender MedSystems, Austria). Results. During the first ophthalmoscopic examination, i.e. before the clinical manifestation of the disease, blood serum of children who later developed ROP that subsequently required laser coagulation of avascular retinal areas, was found to have relatively higher concentrations of VEGF-A (above 1300 pg/ml) and IGF-II (above 140 pg/ml) and lower IGF-I concentrations (below 24 pg/ml) and TGF- β1 (below 8000 pg/ml) as compared with the groups of "safe" children. A dynamic study of VEGF-A content and, simultaneously, TGF- β1 content showed uniform changes in the concentration of these growth factors at all observation times. Conclusion. It was shown for the first time that high IGF-II and low TGF- β1 concentrations revealed before the clinical manifestation of the disease can be viewed as prognostically unfavorable as far as subsequent development of severe ROP is concerned. Uniform changes of TGF- β1 and VEGF-A level in blood serum during ROP development may indicate to the synergistic participation of these growth factors in the pathological vasoproliferation. Further studies are required to determine the details of how this mechanism works // Russian Ophthalmological Journal, 2017; 1: 20-5. doi: 10.21516/2072-0076-2017-10-1-20-25

Retinopathy of prematurity is the main cause of visual impairment and blindness in infants with low birth weight and preterm gestational age, in spite of the achievements in neonatology and wide applying of diagnostic and treatment guidelines. The pathogenetic role of VEGF is determined in course of normal angiogenesis and in retinopathy of prematurity. Scientists continue the search of another significant vasoprolifirative factors and methods how to inhibit them. This review is devoted to monoamines’ role in angiogenesis. The search for the relevant literature was carried out using the Medline database.

Purpose: to assess the level of catecholamines norepinephrine and dopamine in rat retina with experimental retinopathy of prematurity (EROP) at a time corresponding to the peak of neovascularization. Material and methods. The study was performed on 41 infant Wistar rats (82 eyes). The rats were divided into 2 groups: the experimental group (with EROP, n = 21) and the control group (n = 20). In order to reproduce the EROP, the newborn rats were placed for 14 days in the incubator together with their mother. Every 12 hours, the oxygen concentration in the incubator ranged from 60 to 15 %. The control group consisted of pups who were held in conditions with a normal oxygen content (21 %) from the moment of birth. The pups were withdrawn from the experiment on the 10th, 14th, 23rd and 28th day, whereupon they were subjected to binocular enucleation followed by histological examination, in addition, the content of noradrenaline and one of the metabolites of dopamine (L-DOPA) was determined in retinal samples of the eyeballs obtained on the 23rd day by highly effective liquid chromatography technique with electrochemical detection.Results. Histological examination showed that in our EROP model the neovascularization peak occurs on the 23rd day of the experiment. At this time rat pups with EROP showed a significantly lower retinal L-DOPA level as compared to the control values (13.99 ng/g and 30.5 ng/g, respectively), and the norepinephrine level significantly exceeded such values (63.7 ng/g and 7.69 ng/g, respectively).Conclusion. A relative deficiency of dopamine and a relative excess of norepinephrine of the rat pups with EROP is noted at the time of the highest vascular activity of the retina. The obtained data confirm anti-angiogenic properties of dopamine and pro-angiogenic properties of noradrenaline in the second phase of EROP development.

In addition to the classic Coats’ disease characterized by retinal vascular telangiectasias and aneurysmal dilatations surrounded by yellowish intra- and subretinal exudates and developing in somatically healthy children, Coats’-like retinal changes can occur in a number of systemic diseases and complicate the course of other eye pathologies. One of the diseases that occur with Coats’-like exudative vasculopathy is facioscapulohumeral muscular dystrophy, or Landouzy - Dejerine disease. Systemic manifestations of this pathology are the myodystrophic process, which usually affects muscles of the face and arms and distal muscles of the legs, as well as sensorineural hearing loss, respiratory failure, cardiac arrhythmia, in the infantile form - mental retardation and episyndrome. The paper describes two clinical cases involving children with facioscapulohumeral muscular dystrophy, presents the results of treatment, and discusses the importance of a systematic approach in examining patients with Coats’-like retinal changes. For citation: Denisova E.V., Katargina L.A., Kogoleva L.V., Belova M.V., Osipova N.A., Fedoseeva E.V. Eye pathologies in facioscapulohumeral muscular dystrophy (case report and literary analysis). Russian ophthalmological journal. 2018; 11 (3): 50-4 (In Russian). doi: 10.21516/2072-0076-2018-11-3-50-54

Studying immunological aspects of pathogenesis of retinopathy of prematurity (ROP) helps develop new approaches to prevention, rational diagnosis and increase of the effectiveness of treatment of the disease. Purpose: to analyze the level of growth factors in blood serum of premature infants belonging to the group of ROP risk at the pre-clinical stage and find out how this level is related to further development and course of the disease. Material and methods. 85 prematurely born infants of the ROP risk group were examined. The average gestational age at birth was 27.7 ± 2.2 weeks, the average birth weight - 1086.1 ± 266.1 g. The examination included dynamic ophthalmoscopy and determination of the content of VEGF-A in blood serum (using a flow cytometer BD FACS Canto II) and TGF- β1, IGF-I, IGF-II (by enzyme-linked immunosorbent assay (ELISA) using a c-test system Bender MedSystems, Austria). Results. During the first ophthalmoscopic examination, i.e. before the clinical manifestation of the disease, blood serum of children who later developed ROP that subsequently required laser coagulation of avascular retinal areas, was found to have relatively higher concentrations of VEGF-A (above 1300 pg/ml) and IGF-II (above 140 pg/ml) and lower IGF-I concentrations (below 24 pg/ml) and TGF- β1 (below 8000 pg/ml) as compared with the groups of safe children. A dynamic study of VEGF-A content and, simultaneously, TGF- β1 content showed uniform changes in the concentration of these growth factors at all observation times. Conclusion. It was shown for the first time that high IGF-II and low TGF- β1 concentrations revealed before the clinical manifestation of the disease can be viewed as prognostically unfavorable as far as subsequent development of severe ROP is concerned. Uniform changes of TGF- β1 and VEGF-A level in blood serum during ROP development may indicate to the synergistic participation of these growth factors in the pathological vasoproliferation. Further studies are required to determine the details of how this mechanism works // Russian Ophthalmological Journal, 2017; 1: 20-5. doi: 10.21516/2072-0076-2017-10-1-20-25