Pigment dispersion syndrome and its implications for glaucoma

Abstract Pigment dispersion syndrome (PDS) represents a clinical spectrum of a relatively common and usually underdiagnosed phenomenon produced by spontaneous pigment dispersion from the iris into the anterior segment. PDS is often bilateral, has no gender predisposition, and presents at a young age, particularly in myopes. Although most patients experiencing an episode of pigment dispersion are asymptomatic, extreme photophobia, ocular pain, redness, and blurred vision may occur. Other characteristic signs are iridolenticular contact, concave iris configuration, 360° peripheral iris transillumination, and pigment deposition on the anterior chamber angle or the corneal endothelium (Krukenberg spindle). Early PDS diagnosis is crucial to detect patients with pigment-related ocular hypertension (POHT) that can eventually lead to pigmentary glaucoma (PG). The latter represents a sight-threatening condition in which mechanical, environmental, and genetic factors contribute to optic nerve damage. In this review, we update the pathogenic mechanisms involved in the clinical spectrum of the disease. We describe its clinical presentation, ophthalmologic manifestations, and complications, including the factors influencing the development of POHT and PG. Because PDS has variable clinical presentations that lead to misdiagnoses, we emphasize the differential diagnosis and the actual therapeutic strategies according to disease status.