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David Shiple, Peter Netland, Brenton Finklea; Survey of Cataracts and Corneal Disease in Eyes with Aniridia. Invest. Ophthalmol. Vis. Sci. 2012;53(14):73.
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Aniridia is a panocular disorder with problems including glaucoma, foveal and optic nerve hypoplasia, visual disturbance, and aniridia associated keratopathy. This study aims to describe corneal problems and their clinical management in a group of patients with aniridia.
This is a retrospective review of survey data collected in the Aniridia Foundation Internationa registry. Subjects were queried about corneal pannus or scarring, age of onset, corneal surgical intervention, cataract, and dry eyes. The cumulative proportion of subjects with corneal disease and cataract versus time was determined.
The number of subjects was 103. Of these subjects, 43.7% had corneal pannus or scarring, 45% did not, and 21.4% were unsure. Of those with scarring, the average age of diagnosis was 20 years old with a standard deviation of 12.2 years. Of the 103 individuals, 15.5% and 12.9% had keratolimbal allograft (KLAL) and penetrating keratoplasty (PK), respectively. Of those who received both PK and KLAL, 1.9% had PK prior to KLAL, and 6.8% reported PK after KLAL. Of the total subjects, 63.3% reported Cataracts with an average age of 9.4 years old at time of diagnosis. Dry eyes were reported in 56 of 102 (55%) subjects.
Corneal disease is common in aniridia, with an average onset in young adulthood. Subjects with aniridia also had high prevalence of cataract and dry eyes.
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