PURPOSE: To describe corneal and ocular surface abnormalities in patients with congenital aniridia.
METHODS: We examined 50 eyes in 25 patients with congenital aniridia. Schirmer’s test I and II, tear film break-up time, vital staining, tear ferning pattern test and impression cytology, Cochet-Bonnet aesthesiometry, specular microscopy and ultrasonic pachymetry were carried out.
RESULTS: Based on Mackman’s classification, we found grade 0 in 12%, grade 1A in 52%, grade 1B in 20%, and grade 2 in 16% cases. Age, history of ocular surgery, dry eye score and aesthesiometry correlated with the degree of aniridia-related keratopathy (ARK).
Schirmer’s test I was normal in 86.8%, Schirmer’s test II in 94.4% of the eyes and TFBUT in 83.3% of the cases. Corneal staining was altered in 54.2%, and conjunctival staining was altered in 45.7%. The tear ferning pattern was abnormal in 80%. Conjunctival metaplasia was present in 76.9%, and the presence of conjunctival goblet cells on the cornea correlated weakly with the degree of ARK. Corneal endothelial cell density was normal in 13 eyes of 9 patients (2190.84 cells/mm2 ). Ultrasonic pachymetry was higher than average in all eyes examined (587 microns).
CONCLUSION: Simple corneal and ocular surface tests are useful in assessing the presence and degree of aniridia-related keratopathy. Impression cytology is a useful tool in diagnosing metaplasia and limbal stem-cell deficiency in patients with congenital aniridia. CochetBonnet aesthesiometry results and tear ferning patterns are reported for the first time in patients with congenital aniridia
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