Elsevier

Ophthalmology

Volume 107, Issue 6, June 2000, Pages 1111-1124
Ophthalmology

Keratopathy in pseudoexfoliation syndrome as a cause of corneal endothelial decompensationHistorical image: A clinicopathologic study

Presented at the Meeting of the American Association of Ophthalmic Pathologists, New Orleans, Louisiana, November 1998.
https://doi.org/10.1016/S0161-6420(00)00087-7Get rights and content

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.

Section snippets

Material and methods

Penetrating keratoplasty for irreversible corneal endothelial decompensation with advanced corneal edema was performed in 22 consecutive patients with unilateral (7 of 22) or bilateral (15 of 22) PEX syndrome (mean age, 78.2 ± 8.7 years; age range 52–89 years; 7 men, 15 women) from 1993 until 1998 (Table 1). Seven of 22 patients had unilateral or bilateral secondary open-angle glaucoma and 1 of 22 had secondary angle-closure glaucoma.

In one 37-year-old female patient (no. 2695 in Table 1),

Biomicroscopic appearance

Preoperatively, the patients showed advanced corneal edema diffusely involving the entire cornea (Fig 2A, B). By slit-lamp biomicroscopy, the posterior corneal surface revealed a thickened appearance of Descemet’s membrane, which was diffusely covered to varying degrees with irregular excrescences differing from the typical cornea guttata. Melanin deposition on the corneal endothelium caused a diffuse, nonspecific form of retrocorneal pigmentation in all patients.

When feasible, specular

Definition of PEX keratopathy

On the basis of clinical and histopathologic evidence, we propose that eyes with PEX syndrome may in fact have a specific keratopathy leading to corneal endothelial decompensation, which results from a diseased endothelial cell layer and the active involvement of the endothelial cells in the PEX process. In view of the systemic findings14 and the active involvement of most anterior segment tissues and cell types (e.g., vascular endothelial cells, trabecular endothelial cells, epithelial cells,

Acknowledgements

The authors thank Dr. Naoko Asano-Kato (Department of Ophthalmology, Kanazawa, Japan) for valuable help with electron microscopy, and Carmen Rummelt and Elke Meyer (Department of Ophthalmology, University Erlangen-Nürnberg, Germany) for expert technical assistance.

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