Corneal lesions in aniridia include peripheral pannus and epithelial a
bnormalities that may advance centrally, resulting in the need for pen
etrating keratoplasty. Three patients with aniridia who underwent repe
ated corneal surgical procedures (one keratectomy and six penetrating
keratoplasties) are described herein. A clinicopathological correlatio
n was performed. In all three patients there was evidence of recurrent
pannus and epitheliopathy on histopathological examination, which cor
relates with the clinical findings. These findings suggest that the pa
thophysiology of recurrent aniridia keratopathy may be related to a pr
imary abnormality in the stem cells of the recipient corneal limbus. A
niridic pannus and epitheliopathy recur in grafts after penetrating ke
ratoplasty and threaten the transplanted cornea. The risk of recurrent
keratopathy should be considered when recommending a surgical procedu
re to patients with aniridia.