Pigment dispersion syndrome presenting as endothelial dystrophy: An atypical presentation

To describe an atypical presentation of pigment dispersion syndrome (PDS) with diffuse, homogeneous pigment deposition on the corneal endothelium and its management.

A 44-year-old female was referred to a cornea clinic as a case of endothelial dystrophy. Slit-lamp examination revealed bilateral, diffuse, and homogeneous pigment deposition on entire corneal endothelium without any iris transillumination defects. Intraocular pressure (IOP) at presentation were 18 mmHg OD and 16 mmHg OS. Gonioscopy showed dense, homogeneous pigment deposition in the angles. The optic nerve head examination revealed a cup disc ratio of 0.6:1-0.7:1 in both eyes with neuroretinal rim thinning.

Peripheral Nd:YAG laser peripheral iridotomy (PI) was performed. On follow-up, a localized clear pigment free endothelial area was noted over the iridotomy sites bilaterally. IOP was well controlled within 12-14 mmHg with prostaglandin analogue at last follow-up of 24 months.

Diffuse homogeneous pigment dispersion on the endothelium may occur in atypical cases of PDS which may clear in the areas overlying the PI site and, therefore, should not be confused with endothelial disease. This case demonstrates the significance of a thorough clinical evaluation in cases with unusual presentation.