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Nineteen patients with aniridia and one patient with bilateral iris colobomata from eight families were examined for corneal abnormalities. Thirty-six eyes revealed changes that are consistent with a progressive corneal dystrophy. These changes begin in the corneal periphery in the early years of life and progress to the corneal center with increasing age. This dystrophy has been staged into four typical clinical patterns. Central corneal scarring, and in some cases, ulceration may occur, which may be a significant threat to vision.
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