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Pseudoexfoliation syndrome is a systemic condition characterized by the deposition of a protein-like material in the anterior chamber of the eye, mainly in the anterior capsule of the crystalline lens as well as in other organs. Genetic and environmental factors play a role in the onset of the syndrome. It is more common in the elderly as well as in people of certain ethnicities. The syndrome can affect one or both eyes and is a significant risk factor for secondary open-angle glaucoma. 50% of eyes with pseudoexfoliation develop glaucoma. In contrast to primary open-angle glaucoma, pseudoexfoliative glaucoma is considerably more aggressive with very high pressure at onset, faster rate of deterioration, poor response to medication, and increased need for surgical management.
What is the cause of pseudoexfoliation?
Mutations in the elastin metabolism gene LOX1 appear to play a role in the synthesis of pseudoexfoliative material.
What are the risks factors?
– Older age
– Scandinavian and Mediterranean origin
How is the diagnosis made?
The diagnosis is clinical. Patients usually have no symptoms. Rarely, a sudden increase in intraocular pressure can cause pain and blurred vision. Clinically with the slit-lamp we see pseudoexfoliation, dandruff-like material on the pupillary rim and the anterior surface of the lens. The material can also be observed in other tissues such as the conjunctiva, the corneal endothelium, the trabeculum, etc. Iris atrophy and transillumination around the pupil are usually seen. In gonioscopy (examination of the angle of the anterior chamber) a strong deposition of pigment (melanin diffused by the atrophy of the iris) is observed. In most cases the angle of the anterior chamber is open. More rarely, in less than 20% of cases the angle may be closed mainly due to displacement of the crystalline lens due to the fragile fibers of the zonules (the ligaments that hold the crystalline lens in place). Nuclear cataract is often present. In case of increased intraocular pressure, fundoscopy reveals typical glaucomatous optic nerve damage.
What is the treatment?
Treatment is to lower intraocular pressure to stop glaucomatous damage. We usually start with medicinal treatment with eye drops. However, medication often does not achieve a sufficient reduction of intraocular pressure, so surgical treatment is necessary. In pseudoexfoliative glaucoma, laser trabeculoplasty (SLT link) can be quite effective because of significant pigment deposition in the filtering filter. But usually the pressure reduction after SLT trabeculoplasty does not last for a long time. Most patients with uncontrolled intraocular pressure require trabeculectomy (link Trabeculectomy) and, more rarely, insertion of aqueous drainage devices (link).