ELASTOSIS OF THE LAMINA-CRIBROSA IN PSEUDOEXFOLIATION SYNDROME WITH GLAUCOMA

Background: Pseudoexfoliation syndrome is characterized by the presenc e of glycoprotein fibers in ocular and extraocular tissues, and often is associated with glaucoma. Pseudoexfoliation material may be associa ted closely with elastic microfibrillar-associated glycoprotein as wel l as elastin. Methods: Four optic nerve heads of two patients with pse udoexfoliation syndrome and glaucoma were examined using electron micr oscopy and immunogold detection of elastin. Optic nerve heads from hea lthy age-matched individuals and patients with primary open-angle glau coma were used for comparisons. Results: In all eyes with pseudoexfoli ation and glaucoma, there was marked and widespread elastosis in the c onnective tissue of the lamina cribrosa. Elastotic fibers appeared as large and irregular aggregates of electron-dense material labeled with antielastin antibody. Abundant microfibrils were interspersed in the elastotic aggregates, whereas no typical pseudoexfoliation fibers were observed. In contrast, there were less elastotic fibers in the lamina cribrosa from patients with primary open-angle glaucoma compared with pseudoexfoliation glaucoma. Other changes of extracellular matrix wer e similar to those observed in primary open-angle glaucoma: decreases in collagen fiber density, presence of basement membranes not associat ed with cell surfaces, and abundant bundles of microfibrils not labele d with elastin antibody. The elastic fibers appeared normal in other l ocations within the optic nerves of patients with pseudoexfoliation gl aucoma, including in the pial septa and blood vessels of the retrolami nar myelinated optic nerve. Conclusion The authors' findings demonstra te marked and site-specific elastosis in the lamina cribrosa of patien ts with pseudoexfoliation syndrome with glaucoma, suggesting an abnorm al regulation of elastin synthesis and/or degradation in the optic ner ve of patients with this disease. Ophthalmology 1995;102:878-886