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Ulla Eden, Per Fagerholm, Neil S. Lagali; Pathologic Epithelium And Anterior Corneal Nerves In Congenital Aniridic Keratopathy. Invest. Ophthalmol. Vis. Sci. 2011;52(14):292.
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To document pathologic corneal findings in Swedish families with congenital aniridia.
Detailed ophthalmic examinations were conducted in 18 aniridia patients and in 7 unaffected relatives. Digital slit lamp photography, anterior segment optical coherence tomography (ASOCT) and in-vivo confocal microscopy (IVCM) examinations were performed bilaterally to document corneal morphology.
Affected family members presented with different stages of aniridic keratopathy, with a corneal appearance varying from transparent to opaque and highly vascularised. Increased corneal thickness in affected members, particularly those with severe keratopathy, was noted by ASOCT. By IVCM, opaque corneas were characterized by active vessels and dense inflammatory cell infiltration. In corneas with milder keratopathy, pathologic epithelial findings included epithelial pleomorphism, and an absence of morphology corresponding to normal limbal crypts. In the central basal epithelium in particular, several unusual features were observed, including prominent focal opacities, a close association of subbasal nerves with epithelial cells, a high dendritic cell density, a high subbasal nerve density with highly branched nerves, and a prominent subbasal whorl region. Interestingly, abnormal morphology of nerve fiber bundles in the basal epithelium was observed in 3 of 7 unaffected family members.
Altered epithelial morphology, vigorous innervation of the anterior cornea, a high dendritic cell density, and basal epithelial opacities were the most prominent corneal findings in family members with milder forms of aniridic keratopathy. Further findings confirmed the known increase in corneal thickness and limbal stem cell deficiency in aniridia.
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