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Exfoliative syndrome and glaucoma: causes, symptoms, diagnosis, treatment
Last reviewed: 08.07.2025
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Exfoliation syndrome is a systemic disease that leads to the development of secondary open-angle glaucoma.
A specific flocculent white matter seen on examination of the anterior segment of the eye may cause obstruction of the trabecular meshwork. Flocculent white matter is also found in other tissues of the body.
[ Epidemiology of exfoliative syndrome
The prevalence of exfoliation syndrome varies from virtually none in Eskimos to 30% in Scandinavians. The incidence increases with age and time. The incidence of binocular involvement is also increased compared with monocular involvement. Patients with ES-related glaucoma may represent only a small proportion of all glaucoma patients or may represent the majority, depending on the population studied. Although patients with exfoliation syndrome have an increased risk of developing glaucoma (5 times higher, according to the Blue Mountains Eye Study), most of them do not develop glaucoma.
Pathophysiology of exfoliation syndrome
The substance that forms exfoliations has been isolated from the iris, lens, ciliary body, trabecular meshwork, corneal endothelium, and endothelial cells of the eye and orbital vessels, as well as from the skin, myocardium, lungs, liver, gallbladder, kidneys, and meninges. However, its nature has not been fully studied. The substance clogs the trabecular meshwork, which leads to the development of secondary open-angle glaucoma. Ischemia of the peripupillary part of the iris also develops, and posterior synechiae are formed. As a result, the pigment is washed out, the pupillary block and the load on the trabecular meshwork increase, which contributes to the closure of the anterior chamber angle.
Anamnesis
Although overt intraocular pressure elevation is rare, no significant anamnestic data can be identified in most patients. Familial cases are sometimes observed, characterized by a history of surgical treatment of complicated cataracts in the family. The exact mechanisms of inheritance have not been identified.
Diagnosis of exfoliative syndrome
Biomicroscopy
A hallmark of exfoliation syndrome is a flocculent white substance, most often detected during pupil dilation at its edge, in the form of concentric circles on the surface of the anterior capsule of the lens. Similar deposits are found on the iris, structures of the anterior chamber angle, endothelium, intraocular lens, and anterior limiting membrane of the vitreous body in aphakia. Transillumination often reveals peripupillary defects and atrophy of the pigment sheet. Pigment washout from the peripupillary zone is also observed. The pupil in the affected eye is usually smaller and dilates less compared to the healthy eye, which is associated with synovial effusion and iris ischemia. Pigment release associated with pupil dilation can cause a jump in intraocular pressure. Cataracts are also more common in affected eyes.
Gonioscopy
The anterior chamber angle is often narrowed in exfoliation syndrome, especially in the lower sections. Due to the risk of developing an acute attack of angle-closure glaucoma, constant monitoring of the anterior chamber angle is necessary. Gonioscopy reveals uneven pigmentation of the trabecular meshwork with large dark pigment granules. Pigment deposition anterior to the Schwalbe line leads to the characteristic waviness of the Sampaolesi line.
Posterior pole
Characteristic glaucomatous atrophy of the optic nerve is observed with prolonged rise or periodic jumps in intraocular pressure.
Treatment of exfoliative syndrome
In glaucoma associated with exfoliation syndrome, higher values of intraocular pressure and more pronounced daily fluctuations are observed. Drops that reduce intraocular pressure can be prescribed, but this method of treatment has been shown to be ineffective. Despite the postoperative increase in intraocular pressure in argon laser trabeculoplasty, the method is effective. Less laser energy can be used to affect the intensely pigmented trabecular network, which will reduce postoperative intraocular pressure surges. The results of operations aimed at increasing filtration are the same as in primary open-angle glaucoma. Special care should be taken when performing cataract extraction, taking into account the weakness of the capsule and ligamentous apparatus.