PEX Keratopathy and its Differentiation from Fuchs Dystrophy

Schlötzer-Schrehardt U (2025)


Publication Type: Authored book

Publication year: 2025

Publisher: Springer Nature

ISBN: 9783031654220

DOI: 10.1007/978-3-031-65422-0_19

Abstract

Pseudoexfoliation (PEX) has long been known to compromise all anterior segmentAnterior segment structures including the corneaCornea, particularly the corneal endotheliumCorneal endothelium. Based on clinical and histopathological evidence, a specific endotheliopathy has been described in PEX eyesEye, leading to early loss of endothelial cells and early diffuse corneal endothelial decompensation. It results from a diseased corneal endotheliumCorneal endothelium and its active involvement in the fibrotic PEX process. Clinically and histopathologically, PEX keratopathyPEX keratopathy is characterized by (1) decrease in endothelial cell densityEndothelial cell density associated with marked variations in cell size and shape; (2) pronounced pigment dispersion and phagocytosis of melanin granules by endothelial cells; (3) fibroblastic transformation of endothelial cells; (4) active production of PEX fibrils by degenerative endothelial cells, which may appear as hyperreflective deposits on clinical examination; and (5) irregular diffuse thickening of the Descemet membraneDescemet membrane with foci of incorporated PEX aggregates, but without guttaeGuttae formations. Elevated intraocular pressure may aggravate endothelial changes but appears not to be their primary cause. PEX and Fuchs endotheliopathies can be mainly differentiated by presence or lack of typical PEX fibrils or typical guttaeGuttae. PEX keratopathyPEX keratopathy plays a significant role in the clinical management of PEX patients, representing a rather common risk factor for corneal edemaCorneal edema following ocular surgery but a relatively rare indicationIndication for endothelial keratoplastyKeratoplasty.

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How to cite

APA:

Schlötzer-Schrehardt, U. (2025). PEX Keratopathy and its Differentiation from Fuchs Dystrophy. Springer Nature.

MLA:

Schlötzer-Schrehardt, Ursula. PEX Keratopathy and its Differentiation from Fuchs Dystrophy. Springer Nature, 2025.

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