This is a form of open angle glaucoma with early onset (usually before the age of 40 years). Marked pigment deposition in the trabecular meshwork, on the lens, zonules, and the corneal endothelium can often be seen prior to elevation of the intraocular pressure. It can be present asymmetrically, even unilaterally, but primarily in early stages. The pigment source in humans seems to be the iris in which hypopigmentation leads to radial transillumination defects and mouse models corroborate this. The iris configuration is sometimes described as flat or even concave. The pattern of pigment deposition on the posterior surface of the cornea is known as a Krukenberg spindle and considered diagnostic. Untreated, the characteristic optic nerve damage and visual field changes of glaucoma eventually occur. Early-onset and rapidly progressive nuclear cataracts have been reported in some patients.
In one longitudinal study of 113 patients diagnosed with pigment dispersion and followed for 24 years, 23 had glaucoma initially and 9 more eventually required treatment for elevated pressure. The mean age at diagnosis was 42 years and myopic males were the most commonly affected.
The syndromic nature of PDS is suggested by the association of lattice degeneration, retinal tears, and detachments in a significant number of individuals.
