Electron-microscopic identification of pseudoexfoliation material in extrabulbar tissue

The structure and distribution of pseudoexfoliation material in extrabulbar tissues from five eyes with typical unilateral intraocular pseudoexfoliation syndrome and two intraocularly unaffected fellow eyes were examined by transmission electron microscopy. In all seven eyes, unevenly distributed pseudoexfoliation aggregates were found in limbal conjunctivae, extraocular rectus and oblique muscles, orbital connective-tissue septa, and the walls of the posterior ciliary arteries, vortex veins, and central retinal vessels passing through the optic nerve sheaths. Typical pseudoexfoliation fibers occurred in close association with connective-tissue components, especially elastic fibers; a moderate predisposition of pseudoexfoliation clumps to accumulate around blood vessels was observed. The findings of pseudoexfoliation material in similar extrabulbar locations in intraocularly uninvolved fellow eyes indicates that pseudoexfoliation fiber formation outside the globe precedes its intraocular manifestation. The intermingling of pseudoexfoliation fibers, microfibrils, and elastic and collagen fibers suggests that pseudoexfoliation fiber formation might result from a disordered synthesis and/or assembly of connective-tissue microfibrils.