Objective: To attempt to review the aniridia keratopathy pathogenesis and establish a treatment protocol according to the severity of the symptoms.
Methods: Personal experience in aniridic keratopathy management and a bibliography review.
Results: The ocular manifestations of this anomaly include defects of the cornea, glaucoma, lens subluxation, cataracts, hypoplasia of the iris, fovea and optic nerve, amblyopia and nystagmus. The keratopathy occurs in a 20% of patients with aniridia. The correct PAX6 expression is necessary for normal corneal development, limbal stem cell activity and correct corneal epithelial cell migration and adhesion.
Conclusions: The management of ocular surface diseases due to limbal stem cell deficiency in aniridia is complex but has changed in recent years, as an understanding of the limbal stem cells and their microenvironment has modified the therapeutic approach. The use of autologous serum eye drops, amniotic membrane transplantation, limbal transplantation or cultivated limbal cell transplantation have all been reported as a treatment for several ocular surface diseases.