Cataract Surgery in Eyes with Pseudoexfoliation (PEX) Syndrome

Pseudoexfoliation (PEX) syndrome is an age-related systemic disease with primarily ocular manifestations characterized by deposition of whitish-gray pseudoexfoliation fibrillogranular amyloid like material on the anterior lens capsule, zonules, ciliary body, pupillary margin of the iris, corneal endothelium, anterior vitreous and trabecular meshwork [1,2]. The most important and easily recognizable diagnostic sign of pseudoexfoliation is whitish-grey flaky material on the pupillary border of the iris or on the anterior surface of the lens. The lens frequently demonstrates a “three-ring sign” on the anterior lens capsule which consists of a relatively homogenous central zone and a granular cloudy peripheral zone with a clear zone in between. Whilst the characteristic signs of manifest pseudoexfoliation have often been described the early stages of exfoliation have not been well defined. Pigment loss from the iris sphincter region and its deposition on anterior chamber structures support the diagnosis. Detection of these signs requires a careful clinical examination using dilated slit-lamp biomicroscopy and additionally undilated gonioscopy but frequently undiagnosed pseudoexfoliation can lead to unexpected problems in management and during surgery. The awareness of the significance of pseudoexfoliation has increased considerably in the latest decade. Pseudoexfoliation is a risk factor not only for open-angle glaucoma, but also for angle-closure glaucoma, lens subluxation, bloodaqueous barrier impairment serious intraoperative and postoperative complication and has been correlated with an increased incidence of cataract formation. Exfoliation of fibrillogranular amyloid-like material has been found in many organs such as skin, heart, lungs, liver, kidney, gall bladder, blood vessels, extraocular muscle, connective tissue in the orbit, optic nerves and meninges suggesting that the pseudoexfoliation syndrome is not only an ocular disease but also a general disorder that involves the abnormal production of extracellular matrix material [3,4]. Recent investigations have shown the positive link between pseudoexfoliation and transient ishemic attacks, stroke, heart disease and aneurysms of the abdominal aorta [4,5]. Although the exact etiology of this condition as well as the exact structure of the material is still unknown it is presumed that the production of pseudoexfoliation *Corresponding author: S. Kaštelan, Department of Ophthalmology, Clinical Hospital Dubrava, Avenija Gojka Šuška 6, 10000 Zagreb, Croatia, Tel: +385-1-290-3396, +38599-7320-705; Fax: +385-1-290-3396; E-mail: [email protected]