Wowra Bogumił Henryk, Łach-Wojnarowicz Olga, Wysocka-Kosmulska Marzena, Dobrowolski Dariusz, Wylęgała Edward
Chair and Clinical Department of Ophthalmology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia in Katowice, 65 Panewnicka Street, 40-760 Katowice, Poland.
Department of Ophthalmology, District Railway Hospital, 65 Panewnicka Street, 40-760 Katowice, Poland.
J Clin Med. 2024 Nov 4;13(21):6619. doi: 10.3390/jcm13216619.
Aniridia is a rare panocular, bilateral, and congenital disease characterized by complete or partial iris hypoplasia and foveal hypoplasia, leading to decreased visual acuity and nystagmus. AAK, also referred to as aniridic keratopathy, manifests as corneal surface damage, epithelial thinning or loss, inflammation with immune cell infiltration, vascularization, and chronic progressive opacification. Twenty-one eyes in eighteen patients with aniridia underwent the triple procedure for visual rehabilitation. Subjects with stromal scarring with mild limbal deficiency were qualified for surgery. The majority of them developed stage II (15), and a few of them had third-degree (6) aniridic keratopathy. The mean patient age was 38.4 ± 8.8. Visual acuity after one year of observation ranged from 0.4 in two eyes to 0.2 in nine eyes to below 0.1 in ten eyes. In the second year, VA remained at the same level in 13 patients (72.2%). In the third year, four patients (22.2%) experienced recurrence of AAK. A majority of the ARK cases (72.2%) had a graft providing useful vision for the patient 2 years after corneal transplantation, but the visual gain was modest at best. Longer follow-up time is required to evaluate functional graft outcomes.
无虹膜症是一种罕见的全眼球、双侧性先天性疾病,其特征为完全或部分虹膜发育不全以及黄斑发育不全,导致视力下降和眼球震颤。AAK,也被称为无虹膜性角膜病变,表现为角膜表面损伤、上皮变薄或缺失、伴有免疫细胞浸润的炎症、血管化以及慢性进行性混浊。18例无虹膜症患者的21只眼睛接受了三联手术以进行视力康复。角膜基质瘢痕伴轻度角膜缘缺损的患者符合手术条件。他们中大多数发展为II期(15例),少数为III度(6例)无虹膜性角膜病变。患者平均年龄为38.4±8.8岁。观察一年后的视力范围为:2只眼视力为0.4,9只眼视力为0.2,10只眼视力低于0.1。在第二年,13例患者(72.2%)的视力保持在同一水平。在第三年,4例患者(22.2%)出现无虹膜性角膜病变复发。大多数无虹膜性角膜病变病例(72.2%)在角膜移植术后2年有移植片为患者提供了有用视力,但视力改善充其量也很有限。需要更长的随访时间来评估移植片的功能结果。