ArticleAmerican journal of ophthalmology2020
Management of Congenital Aniridia-Associated Keratopathy: Long-Term Outcomes from a Tertiary Referral Center.
Article in American journal of ophthalmology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 43 citations in OpenAlex.
- Congenital aniridia: European COST action ANIRIDIA-NET guidelines for diagnosis, management and care.Acta ophthalmologica · 2026Review
- Coexistence of congenital lacrimal gland agenesis and congenital aniridia: Case report.American journal of ophthalmology case reports · 2025Article
- miRNA Expression Profile in Primary Limbal Epithelial Cells of Aniridia Patients.Investigative ophthalmology & visual science · 2025Article
- The Systemic Genotype-Phenotype Characterization of PAX6-Related Eye Disease in 164 Chinese Families.Investigative ophthalmology & visual science · 2024Article
- Superficial Keratectomy Alone versus in Combination with Amniotic Membrane Transplantation in Aniridia-Associated Keratopathy and a Short-Term Clinical Outcome.Journal of clinical medicine · 2024Article
- Clinical and molecular aspects of congenital aniridia - A review of current concepts.Indian journal of ophthalmology · 2022Review
- Clinical outcomes and visual prognostic factors in congenital aniridia.BMC ophthalmology · 2022Article
- An attempt to optimize the outcome of penetrating keratoplasty in congenital aniridia-associated keratopathy (AAK).International ophthalmology · 2021Observational
- The Limbal Niche and Regenerative Strategies.Vision (Basel, Switzerland) · 2021Review
- Glaucoma Syndromes: Insights into Glaucoma Genetics and Pathogenesis from Monogenic Syndromic Disorders.Genes · 2021Review
- Longitudinal genotype-phenotype analysis in 86 patients with PAX6-related aniridia.JCI insight · 2021Observational
- Systemic diseases and the cornea.Experimental eye research · 2021Review
- Commentary: Long-Term Anatomical and Functional Survival of Boston Type 1 Keratoprosthesis in Congenital Aniridia.Frontiers in medicine · 2021Article
- Long-Term Anatomical and Functional Survival of Boston Type 1 Keratoprosthesis in Congenital Aniridia.Frontiers in medicine · 2021Article
- New horizons in aniridia management: Clinical insights and therapeutic advances.Taiwan journal of ophthalmologyReview
- Corneal epithelial development and homeostasis.Differentiation; research in biological diversityReview
- Eight-years Egyptian experience of Boston type I keratoprosthesis following failed penetrating keratoplasty or ocular surface disease.Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological SocietyArticle
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12 authors at 1 institution in 1 country.
Funding
Abstract
purposeTo report the outcomes of medical and surgical management for congenital aniridia-associated keratopathy (AAK) over a long-term follow-up period.
designRetrospective, comparative case series.
methodsMedical records of patients diagnosed with congenital aniridia were retrospectively reviewed. Age, sex, ethnicity, follow-up time, AAK stage, noncorneal abnormalities, ocular surgeries, and complications were recorded. The visual acuity equivalent (VAE), approximate Early Treatment Diabetic Retinopathy Study (appETDRS) letter score, was calculated using recorded Snellen visual acuities.
resultsA total of 92 eyes of 47 patients (31 females) with mean age of 48.0 ± 18.0 years and mean follow-up of 78.6 ± 42.2 months were included. At the initial visit, 12 eyes (13%) were classified as Stage I AAK, 33 eyes (35.9%) were Stage II, 25 eyes (27.2%) were Stage III, 17 eyes (18.5%) were Stage IV, and 5 eyes (5.4%) were Stage V. Limbal stem cell transplantation (LSCT) and Boston keratoprosthesis (KPro) were frequently performed in eyes with Stages III-V. These advanced corneal surgeries significantly improved the median (95% confidence interval [CI]) of calculated appETDRS scores from 2 (0-20) to 26 (15-41) (Snellen values, 20/20,000 to 20/300; P = 0.0004). Patients with earlier Stages (I-II) of AAK were managed medically and had stable visual acuity through their final visits (appETDRS score of 26 [20-35] to 35 [26-35]; Snellen, 20/300 to 20/200; P > 0.05). The appETDRS VAE was significantly improved from 20 (0-35) to 30 (20-55), Snellen, 20/400 to 20/250, following LSCT (P = 0.021) and from 2 (0-20) to 2 (0-41) after KPro; Snellen, 20/20,000 VAE but with improved 95% CI after follow-up (P = 0.019).
conclusionsWith proper characterization and staging of AAK, individualized medical and advanced surgical interventions preserves and improves visual acuity.
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