Vol 11, No 3 (2016)

Articles
The clinical manifestations of keratoconus in the patients presenting with Down syndrome
Bikbov M.M., Surkova V.K., Usubov E.L., Oganisyan K.K.
Abstract
Down syndrome is one the most widespread forms of genomic pathology accompanied by mental retardation and impairment of cognitive functions. One of the commonest eye diseases in the patients with Down syndrome is corneal ectasia that occurs 10 times more frequently in the children with Down syndrome than in the general population. Objective. Of the present study was the comparative analysis of the biometric parameters of the cornea and the clinical manifestations of keratoconus in the patients presenting with Down syndrome. Materials and methods. We undertook the comprehensive analysis of the biometric parameters of the cornea in 54 patients (108 eyes) with Down syndrome who made up the main study group. The control group was comprised of 62 practically healthy children (124 eyes). Results. The patients presenting with Down syndrome were found to have a higher refractive power of the cornea, a more pronounced elevation of the posterior corneal surface and its irregular patterns, a smaller corneal thickness, and lower degree of corneal hysteresis in comparison with the control children. The verified diagnosis of keratoconus was established in 11 patients of the main group. Conclusion. The present study has revealed keratoconus in 11 (20.4%) children presenting with Down syndrome which suggests a higher prevalence of this pathology in our study group in comparison with its mean prevalence reported thus far for the general population. The early subclinical forms of ceratoconus are known to more frequently occur in the children aged from 12 to 18 years, with the incidence of its later stages being especially high in the group of the patients between 19 and 38 years of age. This discrepancy is supposed to be attributable to the progression of the clinical symptoms of the eye disease with age and its late diagnostics. The deviation of corneal biometric characteristics from the respective normal values documented in 79.6% of the patients included in the present study should be regarded as reflecting the objective situation. It is concluded that the patients presenting with Down syndrome should be referred to the group at enhanced risk of development of keratoconus and undergo the regular ophthalmological examination.
Russian Pediatric Ophthalmology. 2016;11(3):118-120
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The specific clinical features of congenital aniridia in the childhood
Voskresenskaya A.A., Pozdeyeva N.A., Vasil'eva T.A., Khlebnikova O.V., Zinchenko R.A.
Abstract
Aim. The objective of the present study was to elucidate the specific features of the clinical picture of congenital aniridia in the children and adolescents and determine the frequency of complications of this pathology in the Russian Federation. Materials and methods. The study involved 37 children and adolescents at the age below 18 years (74 eyes) who were recruited from 37 unrelated families and diagnosed as having congenital aniridia at the Cheboksarsky branch of S.N. Fedorov Federal State Institute of Eye Microsurgery. All the children underwent the comprehensive ophthalmological examination based at this institution that included determination of the patients’ age and gender, diagnostics of keratopathy, cataract and glaucoma, measurements of foveal hypoplasia and hypoplasia of the optical nerve. In addition, visual acuity (VA) and the type of refraction were determined, gonioscopy and central keratopachymetry were performed on each patient. Results. The age of the patients varied from 2 months to 18 years (median: 3 years). The familial type of inheritance of congenital aniridia was documented in 16 patients whereas sporadic cases of this pathology were detected in 21 (56.7%) children. Microcornea and microphthalmus occurred in 4 and 2 eyes respectively. WAGR syndrome was diagnosed in 9.5% of the patients presenting with sporadic aniridia. Visual acuity was estimated at => 0.1 in 52% of the cases; it was => 0.3 in three patients. Abnormal refraction was documented in 88.3% of the children, marked hypermetropia was diagnosed in 15% of the examined eyes. The signs of aniridic keratopathy in the newborn infants and young children(aged below 3 years) were found in 64% of the cases. The youngest age at which the signs of aniridic keratopathy were apparent was 14 months. Cataract of different severity was documented in 77% of the eyes, glaucoma in 22.6%, foveal hypoplasia in 94%, and nystagmus in 86.5% of the eyes. The thickness of the central cornel region in the children at the age from 6 months to 2 years was 635+-47 microns compared with 606+-43 microns in the patients from 3 to 18 years of age. Conclusion. Congenital aniridia is a progressive panocular pathology affecting various structures of the eye and leading to the impairment of the visual function from the very early life.
Russian Pediatric Ophthalmology. 2016;11(3):121-129
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The new method for the treatment of relative amblyopia in the patients presenting with optical nystagmus
Gubkina G.L., Apaev A.V., Tarutta E.P.
Abstract
Aim. The objective of the present study was to develop the method for the pleoptical treatment of relative amblyopia in the children presenting with optical nystagmus with the use of sinoptophore under conditions of nystagmus blockade. The secondary objective was to evaluate the effectiveness of the proposed method. Materials and methods. The newly developed technique for the pleoptical treatment of relative amblyopia consisted of the blockade of nystagmus by conversion effort and the application of the light pulses to the central fovea of the retina under control of bifoveal fusion. Results. The proposed method was employed to treat 21 patients presenting with optical nystagmus and relative amblyopia at the age varying from 5 to 10 years. In 18 children, the changes in the parameters of nystagmus were evaluated from its amplitude and fixation area density on the retina by means of microperimetry using the NIDEK Microperimeter-MP1 apparatus. The study revealed the tendency toward the improvement of binocular visual acuity, both corrected and uncorrected (by 0.14±0.09 and 0.12±0.07 on the average, respectively), and of the absolute accommodation volume (by 2.88±1.9 diopters). The inverse relationship between the motor parameters of nystagmus and visual acuity was documented. Conclusion. The proposed new method for the pleoptical treatment of relative amblyopia consists of blockade of nystagmus by conversion effort and the delivery of the light pulses to the central fovea of the retina in both eyes under control of bifoveal fusion on sinoptofphore. The method allowed to improve both the functional (enhanced visual acuity) and motor (amplitude and fixation area density) characteristics of nystagmus.
Russian Pediatric Ophthalmology. 2016;11(3):130-132
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Early vitrectomy in the case of stage IVA retinopathy of prematurity: pro and contra
Diskalenko O.V., Konikova O.A.
Abstract
Objective. To evaluate the effectiveness and safety of early lens-sparing vitrectomy for the treatment of the children presenting with stage IVa retinopathy of prematurity during the early and late periods after the surgical intervention. Material and methods. We analyzed the results of the early surgical intervention for vitrectomy with synechiae removal in 83children (116 eyes) at the mean age of 40.0 ± 1.8 weeks with stage IVa retinopathy of prematurity with the use of modern methods including electrophysiological techniques. Results. Stabilization of stage retinopathy of prematurity with the complete reattachment of the retina and the elimination of the vascular activity in the fundus have been achieved in 97.4% of the cases (113 eyes). Thirteen patients (15 eyes or 13.3%) turned out to have glaucoma within the first 3 months after the onset of observations. The maximum corrected visual acuity after vitrectomy in the children at the age of 4.0 ± 0.6 averaged 0.34 ± 0.07, the spherical equivalent of myopic refraction was estimated at 9.8 ± 1,5 D. The electrophysiological study has demonstrated the well apparent reduction of electrogenesis in the central portion of the retina especially in the cone cells and the bipolar nuclear cell layer despite the absence of the noticeable ophthalmological changes in the posterior pole of the fundus in the children treated by lens-sparing vitrectomy as well as in the patients presenting with stage IVa retinopathy of prematurity managed given no vitreoretinal surgery. Conclusion. Urgent vitrectomy with synechiae removal durng the acute period of stage IVa of retinopathy of prematurity is an efficacious and pathogenetically substantiated method for the stabilization of this pathology provided the surgical intervention is performed upon the appearance of the very first signs of retinal detachment. However, the increased risk of glaucoma formation after minimally invasive lens-sparing vitrectomy should be taken into consideration. Moreover, the visual acuity remains impaired due to the inhibition of maturation of the retina at stage IVa of retinopathy of prematurity both after vitrectomy with synechiae removal and in the absence of such intervention.
Russian Pediatric Ophthalmology. 2016;11(3):133-137
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Analysis of the surgical treatment of posterior lenticonus associated with congenital cataract
Pershin K.B., Pashinova N.F., Cherkashina A.V., Tsygankov A.Y.
Abstract
Aim. The objective of the present study was to analyze the results of the surgical treatment of the children presenting with posterior lenticonus associated with congenital cataract. Materials and methods. The total of 8 children (8 eyes) with posterior lenticonus enrolled in this study accounted for 6.5% of all cases of congenital cataract treated at the “Excimer” Eye Center during the period from 2003 to 2015. The age of the patients varied from 0.2 to 15 (mean 8.1±3.8) years. Preoperatively, the uncorrected visual acuity was estimated to be 0.11±0.1 (0.01 - 0.3), with the best corrected visual acuity being 0.15±0.13 (range: 0.01 - 0.4). Lensectomy was performed under general anesthesia by means of the incision technology (1.8 and 2.2 mm) with the use of the Millenium, Stellaris microsurgical system (Bausch and Lomb, USA) and the Infinity system (Alcon, USA). In all the cases, different IOL models (Alcon, USA) were implanted; in four cases, the toric intraocular lenses were used. Results. In all the patients with posterior lenticonus and congenital cataract, the “dry” aspiration of the lens was performed. The distinctive features of posterior capsulorhexis included the use of two viscoelastic materials, viz. heavy Healon GV applied over the intraocular lens and ProVisc applied under the intraocular lens in the form of a “layer cake”. Thereafter, posterior capsulorhexis and vitrectomy were done. The lens was implanted into the capsular bag (n = 6) and into the ciliary sulcus (n = 2) with the anterior and posterior “optic capture”; the edges of the optical hole were placed behind the capsulorhexis hole. In the cases of the initial posterior capsular defect (n = 3), we performed vitreorhexis and vitrectomy followed by intraocular lens implantation. The best corrected visual acuity increased from 0.15 before the operation to 0.72 after surgery, with astigmatism being reduced from 2.3±0.9 to 1.6±065 diopters (overall astigmatism for the entire group) and from 1.81±0.46 to 0.56±0.56 diopters for the subgroup treated by means of toric IOL implantation. The patients remained under the observation during the follow-up period of more than 36 months. Conclusion. Lensectomy followed by intraocular lens implantation used for the treatment of the children presenting with posterior lenticonus and congenital cataract is the efficient method for the management of this combined pathology. Such features of the surgical technique as “dry” aspiration of the lens mass, posterior capsulorhexis, anterior vitrectomy, and optical capture taken together make it possible to achieve the favourable and stable functional outcome of the treatment.
Russian Pediatric Ophthalmology. 2016;11(3):138-143
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The main methods for the optical correction of progressive myopia in the children
Zaraiskaya M.M., Bodrova S.G., Pozdeyeva N.A., Pashtaev N.P., Tikhonova O.I.
Abstract
This article was designed to present the review of the published research concerning the methods for the correction of progressive myopia in the children and adolescents for the purpose of prophylaxis of its further development.
Russian Pediatric Ophthalmology. 2016;11(3):144-148
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The clinico-functional and biomechanical aspects of pathogenesis, diagnostics and treatment of congenital myopia: the review of the literature and the analysis of the native data
Markossian G.A., Tarutta E.P., Iomdina E.N., Panteleeva O.A., Ryabina M.V., Zol'nikova I.V., Kruzhkova G.V., Firsova E.N.
Abstract
This article presents the review of the literature focusing on the current state-of-the-art in genetic, clinical, functional, and biomechanical aspects of pathogenesis, diagnostics, and treatment of congenital myopia. The involvement of hereditary factors in etiology of myopia has been repeatedly confirmed in numerous publications. Suffice it to mention that at least 23 MYP loci localized in 15 different chromosomes are associated with the development of myopia and determine the quantitative characteristics of this pathology. Nevertheless, the specific genes and alleles responsible for the predisposition to myopia belong, with a high degree of probability, to different polygenic systems and are therefore extremely difficult to identify. The results of investigations into congenital myopia give evidence that it must be a special clinical form of myopia characterized by certain peculiar patterns of the retinal relief in the foveal area, specific biophysical properties of the fibrous capsule of the eye, and a particular, both ophthalmologically and morphometrically, shape of the optic nerve. A substantial variation in the optic disc size and excavation patterns associated with congenital myopia as well as the relationship between these two parameters should be taken into consideration when examining the adult highly myopic patients for the early differential diagnostics of glaucoma. The biomechanical properties of the corneoscleral capsule affect the morphometric parameters of the optic disc in congenital and acquired myopia in the children which should be taken into account when choosing the type of the surgical treatment in the event of myopia progression and controlling its advancement. The available results of investigations confirm that contact correction in combination with additional spectacle correction is the most efficient method for the treatment of the patients presenting with congenital myopia. The data obtained show that the reconstructive treatment of the sclera including buckling of the posterior pole provides a very promising approach to the prevention of progression of myopic macular dystrophy and addressing a variety of forms and stages of myopic staphylomas.
Russian Pediatric Ophthalmology. 2016;11(3):149-157
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Drainage surgery of glaucoma
Stepanov A.V., Gamzaeva U.S.
Abstract
One of the topical problems facing modern ophthalmology is the treatment of glaucoma which holds the leading position among the disabling eye diseases. Refractory glaucoma is known to be one of the most complicated forms of this pathology due to its resistance to the medical treatment. The most effective method for the management of this variety of glaucoma is drainage surgery. The present review of the literature was designed to analyze the results of the studies reported by the foreign and Russian authors with special reference to the effectiveness of antiglaucoma drainage and its role in the treatment of glaucoma with special emphasis placed on refractory glaucoma. Different approaches and methods for microdrainage implantation are considered.
Russian Pediatric Ophthalmology. 2016;11(3):158-164
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The scientific and practical conference with international participation «Retinopathy of prematurity - 2016»
Kogoleva L.V.
Abstract
Russian Pediatric Ophthalmology. 2016;11(3):165-168
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