Keratopathy in pseudoexfoliation syndrome as a cause of corneal endothelial decompensation: a clinicopathologic study

Abstract

Purpose: To provide clinical and histopathologic evidence of a distinct keratopathy as a potential cause of corneal edema in patients with pseudoexfoliation syndrome.

Design: Retrospective observational case series.

Participants: Twenty-two patients with clinically diagnosed pseudoexfoliation syndrome undergoing penetrating keratoplasty for irreversible corneal endothelial decompensation.

Methods: The clinical and histopathologic findings of the corneal buttons are described compared with classic Fuchs' endothelial dystrophy.

Results: Clinically, the patients showed diffuse corneal edema, a pleomorphic and numerically reduced corneal endothelium, and retrocorneal flakes of pseudoexfoliation material in three cases. Histopathologically, all corneal buttons showed an abnormal diffuse, irregular thickening of Descemet's membrane and focal accumulations of locally produced pseudoexfoliation material onto or within Descemet's membrane in seven cases. The absence of typical guttata, a higher degree of fibroblastic transformation and melanin phagocytosis of endothelial cells, and a more pronounced endothelial cell loss distinguished the pseudoexfoliation specimens from specimens with classical Fuchs' dystrophy even in the absence of the pathognomonic pseudoexfoliation material.

Conclusions: In patients with pseudoexfoliation syndrome, a distinct type of corneal endotheliopathy may occur, which can lead to an early corneal endothelial decompensation and which might have been previously misdiagnosed as an "atypical nonguttata Fuchs' endothelial dystrophy." This pseudoexfoliation keratopathy may potentiate the known complications in pseudoexfoliation eyes.