Pseudoexfoliation syndrome

Pseudoexfoliation syndrome (other names: exfoliation syndrome, exfoliation glaucoma, eseudoexfoliation glaucoma, eseudoexfoliation of the lens) is an age-related, generalized disorder of the extra cellular matrix characterized by the production and progressive accumulation of microscopic granular amyloid-like protein fibbers in many ocular tissues. It is the most common identifiable cause of open-angle glaucoma worldwide, affecting 60 to 70 million people.

Exfoliative glaucoma is more severe than primary open-angle glaucoma and carries a worse prognosis. Patients with exfoliative material are twice as likely to convert from ocular hypertension to glaucoma. Patients with exfoliative glaucoma have a greater mean intraocular pressure, greater diurnal intraocular pressure fluctuation, more visual field loss and optic disc damage at diagnosis, poorer response to medications, and greater need for surgical intervention, more rapid progression and greater proportion of blindness.

Exfoliative glaucoma is an ocular manifestation of a systemic disease. Other ocular manifestations of the exfoliation syndrome include dry eye, cataract, lens (and intraocular lens) sub-luxation, angle closure, retinal vein occlusion and zonular dialysis, capsular rupture and vitreous loss at the time of cataract surgery. Marked rises in intraocular pressure after dilation mandate examination of the anterior chamber for pigment and measurement of intraocular pressure after pupillary dilation. Exfoliation material has been found in heart, lung, liver, kidney, gall bladder and meninges of patients with ocular exfoliation syndrome. Exfoliation syndrome has been reported to be a risk factor for coronary artery disease and has been associated with transient ischemic attacks, hypertension, angina, myocardial infarction, Alzheimer’s disease and hearing loss. Patients with exfoliation syndrome and exfoliation glaucoma have reduced cerebral blood flow velocity, ocular perfusion pressure, peripheral vascular disease, and more white matter hyperintensities (WMH) on magnetic resonance imaging than controls, demonstrating a potential link between exfoliation syndrome and ischemic brain lesions.

In exfoliation syndrome, a fibrillogranular material accumulates throughout the anterior segment, whether through excess production and/or insufficient breakdown, and is pathognomonic for the disease.