Elsevier

Ophthalmology

Volume 105, Issue 6, 1 June 1998, Pages 951-968
Ophthalmology

Pseudoexfoliation syndrome for the comprehensive ophthalmologist: Intraocular and systemic manifestationsHistorical image

Presented as a “Special Guest Lecture” at the Centennial Meeting of the American Academy of Ophthalmology, Chicago, Illinois, October 27, 1996.
https://doi.org/10.1016/S0161-6420(98)96020-1Get rights and content

Abstract

Background

Renewed interest in pseudoexfoliation syndrome (PEX) may be attributed to an increased awareness of many clinical risks not only for open-angle glaucoma and its recent recognition as a generalized disorder. This review summarizes the range of intraocular and extraocular manifestations. Involvement of all tissues of the anterior segment of the eye results in a spectrum of intraocular complications that have management implications for all practicing ophthalmologists.

Design

The study design was a review.

Methods

Clinical diagnosis depends on biomicroscopy, biocytology, and laser-tyndallometry. Laboratory research methods range from light and electron microscopy to immunohistochemical and molecular biologic approaches.

Observations

Clinical-histopathologic correlations focus on the involvement of lens (PEX-phacopathy), zonular apparatus (zonulopathy), ciliary body (cyclopathy), iris (iridopathy), trabecular meshwork (trabeculopathy), and cornea (corneal endotheliopathy) leading to the following complications: (1) open-angle glaucoma as well as angle-closure glaucoma due to pupillary and ciliary block; (2) phacodonesis, lens dislocation, and increased incidence of vitreous loss in extracapsular cataract surgery caused by alterations of the zonular apparatus and its insertion into the ciliary body and lens; (3) blood-aqueous barrier breakdown (pseudouveitis), anterior chamber hypoxia, iris stromal hemorrhage, pigment epithelial melanin dispersion, poor or asymmetric pupillary dilatation, and formation of posterior synechiae due to involvement of all cell populations of the iris; and (4) early diffuse corneal endothelial decompensation explained by a damaged and numerically reduced endothelium.

Conclusions

In view of the multitude of clinical complications, PEX is of relevance to comprehensive ophthalmologists, including specialists in glaucoma, cataract, cornea, neuro-ophthalmology, and retina. Special attention to the risks associated with PEX is advised before, during, and after surgery.

Section snippets

Background

The history of PEX syndrome starts with the Finnish ophthalmologist Lindberg,3 who referred to observations of Fuchs, Meller, and Axenfeld, but it was Lindberg who defined the changes on the anterior lens capsule in precise drawings. Vogt1, 2 emphasized the association with glaucoma capsulare; Sampaolesi4 elaborated on melanin dispersion and changes of the blood-aqueous barrier; Tarkkanen5 focused on the involvement of the ciliary processes; Ringvold6 showed PEX material in the conjunctival

Intraocular involvement

As ophthalmic clinicians, we are mainly interested in the practical consequences of PEX syndrome. The clinical relevance of our observations is better understood if we look beyond the surfaces of biomicroscopy and perform basic, thorough, clinicohistopathologic correlations. Our didactic terminology is supposed to emphasize the participation of the various tissues in one underlying process (Table 3).

Intraocular complications

Many ophthalmologists routinely still associate PEX syndrome with open-angle glaucoma. It therefore needs to be emphasized that PEX is not only important in the various types of glaucoma (including angle-closure glaucoma) but also for the cataract surgeons in extracapsular surgery (e.g., lens luxation, zonular ruptures, posterior synechiae, incomplete mydriasis, pseudouveitis), for neuro-ophthalmologists (reduced and asymmetric pupillary action), for cornea specialists (corneal

Types of glaucoma

Glaucoma usually occurs in the presence of an open angle, but angle-closure glaucoma via pupillary block may be overlooked frequently.35, 36 Angle closure via ciliary block, without intraocular surgery, does occur but seems to be a relatively rare phenomenon.37, 38 The pathogenetic mechanisms of secondary open-angle glaucoma with PEX syndrome may involve several factors (Table 6). The PEX-associated glaucoma is well differentiated from primary open-angle glaucoma not only clinically but also

Cataract and lens

Lens involvement has several features:

  • 1.

    Cataract. Increased lens opacification is suggested to be associated with PEX by most studies. The predominant type of cataract in PEX syndrome has been reported to be nuclear sclerosis.39, 40

  • 2.

    Phakodonesis and spontaneous lens luxation. Weakening of the zonular support predisposes to phakodonesis41 and spontaneous lens dislocation.42, 43

  • 3.

    Extracapsular cataract extraction: zonular dialyses and vitreous loss. The switch from intracapsular to extracapsular

Corneal endotheliopathy

Corneal endotheliopathy results from a reduced density of the corneal endothelium and morphologic alterations, including the direct involvement of the endothelial cells in PEX syndrome, which increases the risk of early corneal endothelial decompensation, even with only moderate rises in intraocular pressure (e.g., after mydriasis [Table 6]). It differs from Fuchs’ corneal dystrophy in several features (Table 8).49 Puzzling and yet unexplained is the occurrence of PEX material in patients

Pseudoexfoliation-iridopathy

Pseudoexfoliation-iridopathy leads to blood-aqueous barrier leakage and symptoms that include pseudouveitis, melanin dispersion, poor pupillary motility, and reduced oxygen levels in the aqueous (Table 6). These changes as well as adhesive PEX accumulations on the surfaces of iris and lens induce posterior synechiae before and after surgery.

Retina and pseudoexfoliation syndrome

Both the increased rate of vitreous loss and the more frequent need of Nd:YAG capsulotomy for secondary cataract could be expected to enhance the risk of retinal detachment. The PEX syndrome might, therefore, be regarded as a risk factor for the development of retinal detachment (Table 6). Furthermore, central retinal vein occlusion appears to be more common in patients with PEX syndrome.51, 52

Principal complications

Chronic complications of PEX syndrome Table 6, Table 7 are known as ocular hypertension and secondary open-angle glaucoma since Lindberg3 and Vogt.1, 2 Other chronic alterations like pseudouveitis, melanin dispersion, poor pupillary motility, and posterior synechiae may deserve more attention than they generally get. In addition, acute complications may be part of the disease, such as spontaneous lens subluxation and luxation, vitreous loss during extracapsular surgery, and corneal endothelial

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