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.
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.
After the extraction of congenital cataract in infancy, postoperative refraction changes differently in patients with bilateral and unilateral pseudophakia. Purpose: to compare myopia development in children with bilateral and unilateral pseudophakia. Material and methods. We examined 33 children (63 eyes) with bilateral and 21 children with unilateral pseudophakia for long-term results of congenital cataract extraction performed in their infancy, The evaluated parameters included the incidence and degree of myopia, refraction, general postsurgical astigmatism, and axial length of the eye. Refraction was measured by Nidek ARK-530A (Japan). The axial length was measured by ultrasound B-scan (Voluson E8, GE) or by optical biometry (AL-Scan, Nidek). Results. In bilateral pseudophakia, high myopia was detected if astigmatism was more than 3.25 D or oblique. In the latter case, the eyeball showed a greater growth as compared to with-the-rule astigmatism (4.67 mm and 3.26 mm, respectively; p < 0.05). With incomplete correction of astigmatism in the case of bilateral pseudophakia, myopia progressed to a high degree in nearly half of the cases (48.1 %), and a greater growth of the eye was detected compared with complete correction (4.45 mm and 3.42 mm respectively; p > 0.05). The type and degree of astigmatism did not affect the development and progression of myopia in unilateral pseudophakia. Conclusion. The effect of undercorrected astigmatic defocus on the development and progression of myopia in bilateral pseudophakia is apparently associated with a equivalent visual load on both eyes, while this load is significantly reduced in unilateral pseudophakia so that it fails to affect the development of myopia in such cases.
One of the serious challenges facing surgeons after congenital cataract extraction with IOL implantation in infancy is unplanned refractive results revealed in the long-term period, including the cases of myopia higher than 3.0 D in approximately 50 %. Purpose. The study aimed to evaluate the clinical and functional condition of the eyes in children with moderate to high myopia. Material and methods. Our study included a total of 33 patients (61 eyes) with pseudophakia, of which 16 (26 eyes) developed moderate (12 eyes) or high (14 eyes) myopia at the age of 5 to 15 years. Parameters of the anterior and posterior eye segments were evaluated using Galilei Dual Scheimpflug Analyzer and Optical Coherence Tomography RS-3000 Advance 2, and the axial length using Optical Biometer Al-Scan. Results. The central corneal thickness (CCT) was found considerably above the norm (up to 580 ± 35 pm) in most cases of pseudophakic myopia (72.2 %). Anterior chamber depth in children (4.07 ± 0.39 mm) was equal to that in adults with pseudophakia. Astigmatism and axial length in children with highmyopia significantly exceeded those in children with moderate myopia (3.5 and 1.88 D; 23.57 ± 1.31 and 25.04 ± 1.90 mm, respectively), while choroidal thickness and best corrected visual acuity (BCVA) were much lower (268 and 181 pm; 0.1 and 0.3, respectively). There were no variations in spherical equivalent between different axial length in high and moderate myopia. Conclusion. Taken together these results suggest that with the increase of astigmatism axial length elongated and high myopia developed in children with pseudophakia. Choroidal thickness declined due this process and may be a cause of poor functional outcome. Sufficient CCT opens possibilities for refractive surgery in those patients to improve visual outcomes.
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