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![]() As many as three quarters of adult patients have retinal breaks. Vitreoretinal degeneration is progressive and by the second decade rhegmatogenous detachments occur in half of affected patients. Posterior vitreous detachments are common. They are often attached to areas of lattice degeneration in the retina as well as other areas. The vitreous is sometimes seen to form 'veils', especially in the retrolenticular region but they may also float throughout the posterior chamber. The vitreous often appears optically empty as it liquefies and the fibrils degenerate. High myopia and vitreous degeneration dominate the ocular manifestations of Stickler syndrome, type I. The syndromic nature of PDS is suggested by the association of lattice degeneration, retinal tears, and detachments in a significant number of individuals. The mean age at diagnosis was 42 years and myopic males were the most commonly affected. 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. Early-onset and rapidly progressive nuclear cataracts have been reported in some patients. Untreated, the characteristic optic nerve damage and visual field changes of glaucoma eventually occur. The pattern of pigment deposition on the posterior surface of the cornea is known as a Krukenberg spindle and considered diagnostic. The iris configuration is sometimes described as flat or even concave. The pigment source in humans seems to be the iris in which hypopigmentation leads to radial transillumination defects and mouse models corroborate this. It can be present asymmetrically, even unilaterally, but primarily in early stages. 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. This is a form of open angle glaucoma with early onset (usually before the age of 40 years). ![]()
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