Abstract
Aniridia is a panocular disorder that involves many structures of the eye. Aniridic keratopathy is caused by a primary dysfunction of the limbal stem cells, probably by a limbal microenvironment alteration caused by the PAX6 gene mutation. Keratopathy, together with cataract and glaucoma, are the main causes of progressive visual loss in patients with aniridia, and it represents the main source of non visual symptoms in these patients. It is very important to classify the keratopathy in order to plan the therapeutic management. Similar to others patients with limbal deficiency, the treatment should be focused on repopulating the sclerocorneal limbus of limbal stem cells and/or on restoring the microenvironment surrounding them in order to ensure the expansion and survival of the epithelial cells. The therapeutic management will depend on the degree of ocular surface involvement: In patients with sub-clinical or slight limbal deficiency, the treatment with preservative-free lubricants could be sufficient. In patients with moderate keratopathy, the treatment with autologuos serum or amniotic membrane transplantation may be a useful (although temporary) measure to enhance the survival and expansion of limbal stem cells. Finally, patients with severe keratopathy need a source of limbal stem cell.
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García, J.S.L., Lozano, I.G. (2015). Aniridic Keratopathy: Conservative Approaches. In: Parekh, M., Poli, B., Ferrari, S., Teofili, C., Ponzin, D. (eds) Aniridia. Springer, Cham. https://doi.org/10.1007/978-3-319-19779-1_7
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DOI: https://doi.org/10.1007/978-3-319-19779-1_7
Publisher Name: Springer, Cham
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