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PURPOSE: To synthesize recent findings regarding pigment dispersion syndrome in order to arrive at a hypothesis concerning the nature of an underlying genetic predisposition. METHODS: The literature on the subject was reviewed and analyzed. RESULTS: Eyes with pigment dispersion syndrome differ from normal in that they have a larger iris, a midperipheral posterior iris concavity that increases with accommodation, a more posterior iris insertion, increased iridolenticular contact that is reversed by inhibition of blinking, possibly an inherent weakness of the iris pigment epithelium, and an increased incidence of lattice degeneration of the retina. CONCLUSION: A gene affecting some aspect of the development of the middle third of the eye early in the third trimester appears at the present time to be the most likely cause.