OCULAR SURFACE ABNORMALITIES IN ANIRIDIA

PURPOSE: The purpose of this study was to examine ocular surface abnormalities in aniridia. METHODS: We prospectively studied the ocular surface in nine consecutive patients (18 eyes) with virtually total aniridia. Of the 18 eyes, two eyes (two patients) were excluded from the study because severe surgical damage was likely to be the main cause of their corneal abnormalities. Six volunteers whose eye examinations yielded normal results served as control subjects. Detailed slit lamp biomicroscopic examinations were performed to assess corneal findings and confirm the presence or absence of the palisades of Vogt. Goblet cell density on the peripheral corneal epithelium and inferior conjunctival epithelium was calculated via impression cytology and was statistically compared between aniridic eyes and control eyes. RESULTS: All 16 aniridic eyes had superficial corneal opacification and vascularization of either the peripheral or entire cornea. Palisades of Vogt were completely absent all around the limbal area. Goblet cell density on the peripheral cornea and inferior conjunctiva showed a statistically significant increase in the aniridic eyes (226 +/- 169 cells/mm(2) and 406 +/- 203 cells/mm(2)), as compared with the control eyes (0 cells/mm(2) and 181 +/- 89 cells/mm(2)) (P =.0005 and P =.0102). CONCLUSIONS: These findings suggest that, in the aniridic eye, conjunctival epithelium invades the cornea because of corneal epithelial stem cell deficiency.