Pseudoexfoliation syndrome is a systemic disease in which a matrix of fibrotic material is deposited in many organs in the body. In the eye, a basement membrane–like fibrillogranular, whitish material is deposited on the cornea, iris, lens, anterior hyaloid face, ciliary processes, zonular fibers, and trabecular meshwork. These deposits, believed to comprise elastic microfibrils, appear as grayish-white flecks that are prominent at the pupillary margin and on the midperipheral anterior lens capsule (Fig 5-24). Associated with this condition are atrophy of the iris at the pupillary margin, deposition of pigment on the anterior surface of the iris, a poorly dilating pupil, increased pigmentation of the trabecular meshwork, capsular fragility, zonular weakness, and open-angle glaucoma. Pseudoexfoliation syndrome is a unilateral or bilateral disorder that becomes more apparent with increasing age. An association between lifetime UV-light exposure and the development of pseudoexfoliation syndrome has been documented.
Figure 5-24 Pseudoexfoliation syndrome. A, Deposition of white fibrillar material in target like distribution on the anterior capsule. B, Slit-lamp photograph demonstrates atrophy of the iris margin, deposition of pigment on the anterior lens capsule, and fibrillar deposits on iris margin.
(Courtesy of James Gilman, CRA, FOPS.)
Increased oxidative stress caused by abnormalities in transforming growth factor β (TGF-β) contributes to the formation of cataracts. Patients with this syndrome may also experience weakness of the zonular fibers and spontaneous lens subluxation and phacodonesis. Poor zonular integrity may affect cataract surgery technique and intraocular lens implantation. (See Chapter 12 in this volume for a discussion of cataract surgery in special situations.) The exfoliative material will continue to be produced even after the crystalline lens is removed.
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Pasquale LR, Jiwani AZ, Zehavi-Dorin T, et al. Solar exposure and residential geographic history in relation to exfoliation syndrome in the United States and Israel. JAMA Ophthalmol. 2014;132(12):1439–1445.
Zenkel M, Lewczuk P, Jünemann A, Kruse FE, Naumann GO, Schlötzer-Schrehardt U. Proinflammatory cytokines are involved in the initiation of the abnormal matrix process in pseudoexfoliation syndrome/glaucoma. Am J Pathol. 2010;176(6):2868–2879.
Excerpted from BCSC 2020-2021 series: Section 11 - Lens and Cataract. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.