To report a case of long anterior lens zonule and pigment dispersion syndrome.

A 67-year-old female visited our clinic with complaint of visual disturbance in the left eye. She had no history of nyctalopia. Visual acuity was 0.6 in the right eye and 0.4 in the left eye. Intraocular pressure was 12 mm Hg in the right eye and 16 mm Hg in the left eye. Nuclear sclerosis was observed in the left lens. There was no pseudoexfoliative material observed. In the left eye, long anterior zonules with brown pigmented lens striae were spotted irregularly in every direction of the anterior lens. On gonioscopy, the angle was open, and dense, uniform, trabecular meshwork pigmentations were observed at the interior 120 degrees. On fundus examination, cup-to-disc ratio was 0.4 in the right eye, 0.3 in the left eye, and multiple hard exudates were observed in both retinas. Axial length was 22.03 mm in the right eye and 21.84 in the left eye. Anterior chamber depth was 2.71 mm in the right eye and 2.47 mm in the left eye. Defects in the retinal nerve fiber or visual field examination were not observed and pigment dispersion syndrome was diagnosed. The patient showed no significant change at the 9-month follow-up.

We diagnosed atypical pigment dispersion syndrome associated with long anterior zonules and pigmented lens striae. Late onset retinal degeneration should be ruled out with chromosomal analysis if patients show nyctalopia, retinal pigment epithelium atrophy, or family history.