Central Corneal Thickness in Patients With Congenital Aniridia

Purpose. To compare the mean central corneal thickness (CCT) in patients with congenital aniridia to that of a group of age-matched control subjects. The findings of specular and confocal microscopy in a patient with aniridia are discussed. Methods. The mean values of five consecutive pachymetry measurements of patients with aniridia and control subjects were used for analysis. Statistical analysis was performed with a Mann–Whitney rank sum test. Specular microscopy was performed on one patient with aniridia using a Konan Specular Microscope Noncon ROBO CA (Hyogo, Japan). Confocal microscopy through focusing was performed with the Tandem Scanning Confocal Microscope (Reston, VA). Results. Mean CCT measured 691.8 75.4 m for patients with aniridia (16 eyes of 10 patients) and 548.2 21.2 m for control subjects (P0.001). Specular microscopy in a patient with aniridia showed normal endothelial cell counts and structure. Confocal microscopy through focusing of this patient showed normal-appearing keratocytes and a thick corneal stroma. Conclusions. Patients with congenital aniridia have significantly thicker corneas than do age-matched control subjects. This difference can have important implications for the treatment of those patients who develop secondary glaucoma. The increased CCT in patients with aniridia is not a result of endothelial dysfunction but appears to be the result of the production of a thickened but otherwise healthy cornea by the mutated PAX6 gene.