Keratopathy in pseudoexfoliation syndrome as a cause of corneal endothelial decompensationHistorical image: A clinicopathologic study☆
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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Supported by Deutsche Forschungsgemeinschaft (SFB-539).