Pigment dispersion syndrome (PDS) and pigmentary glaucoma (PG) are characterized by disruption of the iris pigment epithelium (IPE) and deposition of the dispersed pigment granules throughout the anterior segment. The classic diagnostic triad consists of corneal pigmentation (Krukenberg spindle); slit-like, radial, mid-peripheral iris transillumination defects, and dense trabecular pigmentation. The iris insertion is typically posterior, and the peripheral iris tends to have a concave configuration. The basic abnormality in this hereditary disorder remained unknown.

General Features Typically, the patient is young, myopic, and male (male to female ratio approximately 2:1). The disorder appears most frequently in the third decade of life, and there is a tendency for it to decrease in severity or disappear in late life. PG is seen predominantly in the white population. Clinical features: …