No abstract

No abstract

Случай развития врожденной катаракты у ребенка с синдромом грима Кабуки Национальный медицинский исследовательский центр глазных болезней им. Гельмгольца ул. Садовая-Черногрязская, 14/19, 105062, Москва, Российская Федерация РЕЗЮМЕ Синдром Кабуки (грима Кабуки)-редкое генетическое заболевание, при котором черты лица ребенка напоминают маску актеров японского театра Кабуки, сопровождающееся множественными аномалиями различных органов и систем. Случаи развития врожденной катаракты (ВК) не описаны. Представлено описание клинического случая диагностики и лечения врожденной катаракты у ребенка с синдромом грима Кабуки. Пациент и методы. Ребенок М., 15 лет с синдромом грима Кабуки. На ODврожденная зонулярная катаракта 1-й степени помутнения, на OS-врожденная зонулярная катаракта 3-й степени помутнения. Проведено обследование и факоаспирация ВК с имплантацией ИОЛ HOYA 24,0 Д на OS. Результаты. Острота зрения: OD = 0,6 с-1.0D = 0,8-0,9; OS до операции = 0,02 н/к., после операции = 0,1 н/к. (обскурационная амблиопия). Хрусталик OD-диск помутнения, через который офтальмоскопировались центральные отделы глазного дна. Хрусталик OS-атипичный диск с неравномерными помутнениями в виде темных и светлых полос, центральные отделы глазного дна не офтальмоскопировались, просматривались только периферические сосуды. Ультразвуковое исследование OU: единичные плавающие помутнения в стекловидном теле, ПЗО = 22,7 мм. Электрофизиологические исследования-снижение ряда показателей OS. Выводы. При синдроме грима Кабуки может иметь место врожденная зонулярная катаракта. Учитывая прогрессирующий характер помутнения хрусталика, необходимо динамическое обследование с широким зрачком для своевременного выявления и направления на хирургическое лечение. В раннем послеоперационном периоде имеется риск развития экссудативно-пролиферативных реакций.

115 children (159 eyes) examined 1 to 11 years after the extraction of bilateral or unilateral СС at the age of 2-11 months. Optical power of the implanted IOL had been calculated using SRKII formula for hypercorrection to result in emmetropic or weak myopic refraction by the time the eye growth finishes. The subjects underwent autorefractometry on Retinomax K-Plus 3 device and ultrasonic biometry on Humphrey 835 A/B-scan system. Results. The incidence of unplanned refraction in children aged 1 to 3 years was 61.2%, aged 3 years to 5 years 11 months-24.4%, and in children of 6-11 years-50.0%; it was associated with pronounced unplanned eye growth in 48.6% of cases with bilateral СС and in 27.3% of cases with unilateral СС. Conclusion. The main cause of unplanned refraction in pseudophakic eyes in children with СC is unpredictable increase of the length of anteriorposterior axis after surgery.

No abstract

Objective. Development of a differential approach to laser iridotomy in children with endogenous uveitis considering iris characteristics and the reaction of children’s eyes to different laser types.Patients and methods. Thirty-six laser iridotomies were conducted in 36 children aged 8–16 years with pupillary block. Nidek YAG laser was used in YAG (1064 nm) and SLT (532 nm) modes.Results. The frequency of hemorrhagic complications during surgery decreased to 19.4 % since 2009. The frequency of exudative reactions and proliferative syndrome with closure of laser coloboms in 1-6 months after surgery decreased to 33.3 % (25.7 % and 40.9 % respectively).Conclusion. We recommended the patented differential approach for effective nontraumatic laser iridotomy in children with endogenous uveitis. In a thin iris single-stage YAG laser perforation should be performed in maximum bombe and multiple perforation in cases of multi-chamber bombe. In a dense thick iris or in cases of former iridotomy closure two-stage approach should be performed. First, circular coagulation should be done in the form of a ring or spot at the site of the planned coloboma. Second, YAG-laser perforation should be done in 10–14 days. The iris vessels topography, reflexogenic zones, and the peculiarities of the iris reactions to the lasers in children with endogenous uveitis should be considered when choosing a place for a coloboma. The use of thermal coagulating lasers should be limited, especially on thin irises. Active anti-inflammatory therapy and eye monitoring are necessary after laser surgery. The differential approach is safe, effective, and can be recommended for implementation in the practice of pediatric ophthalmology.

Purpose: to analyze clinical and functional manifestations and the course of different stages of primary congenital glaucoma (CG) at different times of the disease onset, using the data of admissions to the Department of Eye Pathology in Children of the Helmholtz Research Center of Eye Diseases.Material and methods. 191 patients (320 eyes) with CG, of which 113 were children (191) eyes with primary congenital glaucoma (PCG) aged 1 month to 16 years, underwent standard ophthalmological examination (autorefractometry, visometry, biomicroscopy, ophthalmoscopy), electrophysiological examination (flash VEP, ganzfeld and flicker ERG) and echobiometry (axial eye length measurement).Results. A detailed clinical and functional characteristic of PCG, including structural and functional changes occurring at different stages. Most children with PСG showed advanced stages of the glaucomatous process: in cases where PСG was detected by 1 month of life, the advanced stages claimed 71.1 %, whilst the children diagnosed with PCG between 1 and 12 months of age, the advanced stages were found in 91.2 %. clinical manifestations were found to have variability, not always corresponding to the stages of the disease. In particular, we detected a correlation between disease progression and decreased VEP amplitude (p < 0.05), while no such correlation was found with respet to ERG parameters and VEP latency.Conclusion. Despite the fact that PCG is detected comparative early, the destructive glaucomatous eye damage leads to severe and often irreversible consequences. The timing of CG manifestation is determined by the degree of congenital abnormalities in the eye drainage system that, together with secondary structural changes of eyes (cornea, axial length, retina and ONH) entail a more severe prognosis of PCG in case of an early disease onset. New objective criteria are needed to assess the severity and prognosis of PCG, which is important for the preservation of visual functions, prevention of low vision and blindness.

Purpose:to study the morphological characteristics of the macular zone using optical coherence tomography to assess its development in congenital cataract.Patients and methods:a comprehensive ophthalmological examination was performed in 147 children (269 eyes) aged 1 to 15 years, including, along with traditional methods, optical coherence tomography on the Spectralis HRA + OCT instrument, Heidelberg Engineering. Of these, 193 eyes were with pseudophakia after the removal of congenital cataracts in children aged 2 months to 14 years, 18 eyes with aphakia after the removal of congenital cataracts in children aged 2 months to 11 years, 20 unoperated eyes with partial forms of congenital cataracts and 38 paired conditionally healthy eyes in children with unilateral congenital cataracts. Visual evaluation of linear tomograms, measurement of 5 parameters of the central zone of the fundus and calculation of the macular index were performed.Results:various features of the macular zone were revealed: changes in the macular contour, lamellar structure of the retina, thinning of the choroid, and elements of epiretinal fibrosis. Evaluation of retinal differentiation with the help of a macular index, calculated on the basis of morphometric characteristics, revealed its violations in 52.8 % of cases, which was not always revealed in the visual analysis of tomograms. The best morphometric parameters of the central zone of the fundus are noted in the eyes with pseudophakia in comparison with aphakia (p < 0.05).Conclusion:there is a variability in the state of the macula. In patients with congenital cataracts. Better indicators of the central zone of the fundus in front of the eye with pseudophakia in comparison with aphakia justify the feasibility of performing surgical treatment of congenital cataracts simultaneously with the implantation of the intraocular lens, including in infants. Thinning of the choroid in the subfoveiolar zone with congenital cataract may indicate a violation of trophism and exert additional influence on the physiological development of the macular zone.