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Objective. To monitor congenital glaucoma and to determine the proportion of the exposed stage of the disease revealed during the initial examination and the long-term follow up. Materials and methods. The present study included 27 children (47 eyes) at the age ranging from 8 to 17 years presenting with various forms of compensated congenital glaucoma at the initial, advanced, and terminal stages of the disease. Results. The clinical and functional characteristics of the eyes at each stage of congenital glaucoma were analyzed during the initial examination and the long-term follow-up period. It was found that the currently universally accepted classification of congenital glaucoma that subdivides the disease into stages based on the structural anatomical changes in the eyes ceases to adequately reflect the objective state of the visual system as the child grows. Conclusion. The study has demonstrated that the stage of congenital glaucoma needs to be corrected in accordance with the age and growth of the child and the possibility of its examination with the use of the psychophysical methods and the evaluation of visual acuity.

Purpose. To analyze the literature on the topic of congenital corneal opacities, their diagnosis, differential diagnosis, as well as the choice of optimal treatment tactics. Congenital corneal opacities (CCO), when effective treatment is delayed, usually lead to permanent loss of visual function. This diagnosis requires urgent measures. On the other hand, the rarity of neonatal corneal pathology and difficulties associated with evaluation of newborns yield the situation when more or less defined clinical practice patterns in such cases remain unclear for most pediatric ophthalmologists. Based on our own experience and the literature data, we describe the main issues of differential diagnosis and management of CCO in newborns. A broad range of developmental anomalies, congenital hereditary corneal dystrophies and metabolic diseases as the main causes of CCO are reviewed. In addition to this diagnostic paradigm, the prognosis in every particular pathology as well as treatment recommendations are given. Conclusion. Based on the presented review, the prognosis and therapeutic recommendations for a particular congenital pathology of the cornea are given. Key words: congenital malformations of the eye, children, corneal opacity, keratoplasty, low vision.

Objective. To evaluate the immediate and long-term biological and functional results of reconstructive penetrating keratoplasty (RPKP) in the children. Materials and methods. We undertook a comparative analysis of the outcomes of 86 cases of the surgical intervention on 74 children presenting with corneal opacities of different etiology who had been treated with the application of reconstructive penetrating keratoplasty based at the Department of Eye Pathology in Children, The Helmholtz Moscow Research Institute of Eye Diseases. All operations were made by the same surgeon during the period from 2008 to 2014. The results of reconstructive penetrating keratoplasty were compared with the outcomes of conventional penetrating keratoplasty. The biological results were evaluated in terms of the graft survival (Kaplan-Meir’s) model. The duration of the postoperative follow-up period ranged from 5 months to 8 years and averaged 20,8 ± 9,7 months in the children treated with the use of reconstructive penetrating keratoplasty and to 3,0 ± 15,4 months in the patients treated by means of conventional penetrating keratoplasty. Results. During the early postoperative period (within 1 and 6 months after surgery), the difference in the graft survival rate between the two groups was practically non-existent. After 1 month, the transparency of the transplanted cornea was fairly well preserved in the children of both groups, but persisted for 6 months only in 72% and 95% of the patients treated by reconstructive and conventional penetrating keratoplasty respectively. One year after surgery, the graft survival in the children treated with the use of reconstructive penetrating keratoplasty was documented in 54% of the cases in comparison with 78% in the patients treated by means of conventional penetrating keratoplasty. The difference between the two groups was statistically significant (p < 0,05). Two and three years after surgery, the transparency of the transplanted cornea in the children treated with the use of reconstructive penetrating keratoplasty fell down to 50% and 20% respectively. During the same periods, the transparency of the transplanted cornea in the children treated by means of conventional penetrating keratoplasty remained as high as 76% and 62% respectively. Conclusion. Although the combination of penetrating keratoplasty with other surgical modalities results in the almost three-fold reduction of the probability of engraftment of the transparent corneal transplant in the remote postoperative period in comparison with the standard implantation of the donor cornea transplant, this operation provides the only possibility for the restoration of vision in the children suffering from severe corneal pathology.