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An Ultrastructural Study

Thomas Hammer, MD; Ursula Schlötzer-Schrehardt, MD; Gottfried O. H. Naumann, MD

Arch Ophthalmol. 2001;119:1023-1031.

Background  Clinically, most patients with pseudoexfoliation (PEX) syndrome reveal only unilateral ocular involvement. However, the generalized nature of the disorder suggests that PEX syndrome is clinically asymmetric rather than strictly unilateral.

Objective  To perform an ultrastructural study of the contralateral eyes in patients with unilateral PEX syndrome.

Methods  Five pairs of donor eyes with slitlamp microscopic, macroscopic, and light microscopic evidence of unilateral PEX syndrome and 6 normal control eyes were investigated by transmission electron microscopy and light and electron microscopic immunohistochemistry using antibodies against the human natural killer (HNK-1) epitope and against latent transforming growth factor ₁–binding protein, both markers for the identification of PEX deposits.

Results  Ultrastructural alterations were observed in anterior segment tissues of all apparently not involved fellow eyes. These included (1) deposits of typical PEX fibrils on the iris and ciliary epithelia and in the dilator muscle of the iris; (2) increased accumulation of extracellular matrix, including microfibrils and reduplicated basement membrane material in the periphery of iris vessels, in the dilator muscle and in the juxtacanalicular tissue of the trabecular meshwork; and (3) degenerative changes of the iris pigment epithelium and dilator muscle cells. Latent transforming growth factor ₁–binding protein– and HNK-1–positive deposits indicating PEX material accumulations were detected in the periphery of iris vessels and in the dilator muscle in all affected and contralateral eyes, but not in the control eyes.

Conclusions  These subclinical alterations of contralateral eyes in clinically so-called unilateral PEX syndrome support the concept that PEX syndrome is a generalized basically bilateral disorder with a clinically marked asymmetric manifestation. The iris changes may account for the clinical signs characteristic of early stages, such as melanin dispersion, peripupillary atrophy, trabecular meshwork pigmentation, and insufficient asymmetric mydriasis. The findings should be considered in the clinical management of the patients.

Clinical Relevance  In view of the fact that PEX syndrome is the most common identifiable cause of open-angle glaucoma worldwide and as it is an important risk factor for a wide spectrum of ocular complications, particularly during cataract surgery, the potential involvement of both eyes in the PEX process is of clinical significance.

From the Departments of Ophthalmology, University of Erlangen-Nürnberg, Erlangen (Drs Hammer, Schlötzer-Schrehardt, and Naumann), and University of Halle-Wittenberg, Halle/Saale (Dr Hammer), Germany.

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