Clinical Manifestations and Complications of Pseudo-Exfoliation Syndrome. A Degenerative Fibrillopathy
Schlötzer-Schrehardt U., Naumann G. O. H.
Department of Ophthalmology, University Erlangen-Nuremberg, Erlangen
Pseudoexfoliation (PEX) syndrome represents a generalized degenerative fibrillopathy of unknown etiology, which leads to progressive accumulations of an abnormal fibrillar matrix product with subsequent degenerative changes in intra- and extraocular tissues. Recent data support the pathogenetic concept of a stress-induced elastosis associated with the increased production of elastic microfibrils.
Active involvement of the trabecular meshwork in this pathologic matrix process may lead to secondary open-angle glaucoma development in about half of the patients. This PEX-associated glaucoma accounts for approximately 25% of all glaucomas worldwide. The primary cause of chronic pressure elevation appears to be the blockage of outflow channels by locally produced PEX material accumulations in the juxtacanalicular tissue of the trabecular meshwork with subsequent degenerative changes of Schlemms canal area. Apart from glaucoma development, PEX syndrome represents an important risk factor for additional spontaneous or intra- and postoperative ocular complications, such as zonular weakness with a tendency to phakodonesis and lens subluxation, pronounced melanin dispersion and peripupillary atrophy, poor mydriasis, formation of posterior synechiae, vasculopathy of iris vessels with blood-aqueous barrier defects and anterior chamber hypoxia as well as a corneal endotheliopathy leadi
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