The Triple Procedure in Patients with Congenital Aniridia

<b>Background:</b> 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. <b>Methods:</b> 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. <b>Results:</b> 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. <b>Conclusions:</b> 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.

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Publication Details

Journal
Journal of Clinical Medicine
Published
2024-11-04
DOI
https://doi.org/10.3390/jcm13216619
Primary Topic
Corneal Surgery and Treatments
Type
article
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article

The Triple Procedure in Patients with Congenital Aniridia

Edward Wylęgała, Olga Łach-Wojnarowicz, Bogumił Wowra, Marzena Wysocka-Kosmulska et al.
Journal of Clinical Medicine
Corneal Surgery and Treatments
article

The Triple Procedure in Patients with Congenital Aniridia

Edward Wylęgała, Olga Łach-Wojnarowicz, Bogumił Wowra, Marzena Wysocka-Kosmulska, Dariusz Dobrowolski
article en

Abstract

<b>Background:</b> 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. <b>Methods:</b> 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. <b>Results:</b> 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. <b>Conclusions:</b> 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.

Journal of Clinical MedicineVol. 13(21)
Instytut Kolejnictwa (PL), Medical University of Silesia (PL), University of Silesia in Katowice (PL)
Good health and well-being
Openalex Percentile: Top 67%
Corneal Surgery and Treatments
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