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Neil S Lagali, Bogumil Wowra, Dariusz Dobrowolski, Tor Utheim, Edward Wylegala, Per Fagerholm; Progressive aniridia-associated keratopathy phenotype in a Polish cohort – therapeutic implications. Invest. Ophthalmol. Vis. Sci. 2017;58(8):4811. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
To investigate the central corneal phenotype in patients with aniridia-associated keratopathy (AAK) in relation to age and disease severity.
37 subjects (68 eyes) with congenital aniridia aged 7-75 years, were examined at the University of Silesia, Poland. Examinations included slit lamp biomicroscopy grading, Cochet-Bonnet esthesiometry, and in vivo confocal microscopy (IVCM) of the central and paracentral cornea.
AAK severity increased with age (P < 0.001) with logarithmic progression and onset of visual axis involvement typically between 20 – 30 years of age. 30% of subjects had an asymmetric AAK grade across eyes. Corneal epithelium in early stage AAK shifted to mixed and conjunctival phenotype in later stages. Goblet cells were more prevalent in later stages but were found in only half of late stage eyes. Subbasal nerve and mature dendritic cell presence diminished, sensitivity declined, inflammatory cell infiltration increased, and blood and lymph vessels increased with progressing AAK. Epithelial cysts were present in 30% of eyes regardless of age while Bowman’s layer abnormalities were detected in about 10% of eyes.
AAK spares the central epithelium, nerves, and immune privileged status of the cornea in an early stage, indicating a possible therapeutic window for halting progression. Phenotypic changes assessed by IVCM in progressing AAK, such as neurotrophic deficit, invasion of inflammatory cells and lymph vessels, suggest impaired wound healing and activated immune response and may in part explain the generally poor outcome following corneal surgery in subjects with aniridia.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
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