[Optic canal stenosis in Crouzon syndrome: a case report and literature review]. / Stenoz zritel'nykh kanalov pri sindrome Kruzona: klinicheskii sluchai i obzor literatury.

BACKGROUND: Incidence of Crouzon syndrome is 1 per 25.000-31.000 newborns. This syndrome is extremely rarely accompanied by optic canal stenosis. OBJECTIVE: To present a patient with Crouzon syndrome and optic canal stenosis, to discuss the management of such patients considering own and literature data. MATERIAL AND METHODS: A 6-year-old boy presented with Crouzon syndrome (verified by molecular genetic research, i.e. FGFR2 gene mutation). The patient underwent 3 surgeries for craniosynostosis and hydrocephalus. Nevertheless, visual acuity progressively decreased despite patent ventriculoperitoneal shunt. Examination revealed severe decrease in visual functions with optic disc congestion under secondary atrophy. MRI data on subarachnoid CSF accumulation over both optic nerves potentially indicated optic canal stenosis. This assumption was confirmed by 3D CT. RESULTS: The patient underwent decompression of both optic canals with subsequent improvement of visual functions. CONCLUSION: Vision decrease following Crouzon syndrome may be due to optic canal stenosis. Decompression may be effective, even in long-term course of disease, and improve visual functions.

[Electrophysiological characteristics of the retina in children with cicatrical retinopathy of prematurity]. / Élektrofiziologicheskie osobennosti setchatki v rubtsovom periode retinopatii nedonoshennykh.

Visual impairment in children with regressive retinopathy of prematurity (ROP) are diverse and widespread. PURPOSE: to describe the electrophysiological characteristics of the retina in children with I-V stages of regressive ROP. MATERIAL AND METHODS: The study included children with stages I-V aged 5 to 17 (in the disease stages of IV and V - with reattachment of the retina after surgical treatment of its detachment). Patients with concomitant diseases of the eye were excluded from the study. The main group included 133 children (163 eyes) aged 5 to 17 (mean age 7.2±0.3). Gestation age was 30.0±0.2 weeks, birth weight - 1368.0±46.6g. The control group consisted of 55 age-matched children. Standard electroretinogram (ERG) was performed in all study subjects. The amplitude-time characteristics of maximum and photopic response a- and b-waves were evaluated, and the b/a-index was calculated. RESULTS: Visual acuity of 0.6 or higher was observed in 18 eyes (54.5%) of children with stage I-II ROP and significantly less in the disease stages of III and IVa - 29.5% and 15.6% respectively (p<0.01: Φ=2,717-3,663). In patients with IVb-V stages in 94.1% of cases visual acuity was lower than 0.1. Anamnesis showed significant decrease in photopic ERG starting with active period of stage III ROP. The retinal function further progressively decreased with stages. b/a index of maximum ERG increased proportionally to the severity of ROP active period. Core b/a index was significantly reduced in all stages of ROP (p<0.05). CONCLUSION: Even the early stages of ROP have consequences for the functional status of the retina in premature infants; it is accompanied by significant disturbance of its electrogenesis.

[Potential for use of hydroxypropyl guar in tear substitute therapy]. / Vozmozhnosti primeneniya gidroksipropilguara v slezozamestitel'noi terapii.

Of the large number of polymers used in artificial tear formulations, natural polysaccharides - hydroxypropyl guar, sodium hyaluronate, chondroitin sulfate, dextran, etc., are gaining more and more popularity. Hydroxypropyl guar stands out through its ability to form a long-lasting structured matrix adhered to the damaged ocular surface and ensuring good wettability of the latter and regeneration of epithelial cells. At that, the viscosity of hydroxypropyl guar increases with increasing tear pH (which, in turn, correlates with the severity of xerosis) and further prolongs the moisturizing effect. According to experimental studies, the in vitro protective activity of hydroxypropyl guar surpasses that of sodium hyaluronate, which is widely used. Thus, corneal epithelial cell cultures were more tolerant to drying and pericardial leaflets showed lower friction coefficient, if pretreated with hydroxypropyl guar and not hyaluronic acid. Subsequent clinical studies showed that Systane Ultra was more effective in patients with dry eye syndrome than a carboxymethylcellulose and glycerol-containing drug. It has been also proved that Systane Balance provides a greater increase in thickness of the tear film lipid layer and in overall stability of the tear film as compared to SootheXP, which has a similar lipid composition. The present review also covers potential utility of other artificial tear formulations that, besides the moisturizing effect, are able to reduce the tear film osmolarity, prevent further oxidative stress, and abate the inflammatory process.