Changes in peri-ocular anatomy and physiology in pseudoexfoliation syndrome (Review)

Abstract

Pseudoexfoliation syndrome (PEX) is characterized by the deposition of proteinaceous material in the anterior ocular segment (resulting in ophthalmic pathologies such as glaucoma and increased risk of complications in cataract surgery), but also by several systemic manifestations. The involvement of peri-ocular tissues in PEX, including the eyelid skin, lacrimal gland, conjunctiva, orbital fat and vessels, as well as the optic nerve, has been reported by several previous studies. The peri-ocular effects of PEX include the development of eyelid laxity, conjunctival chalasis, tear film abnormalities, pronounced orbital fat atrophy in response to the administration of prostaglandin analogues in pseudoexfoliative glaucoma, deficient orbital vascular supply and biomechanical changes in both the eyeball and the optic nerve. These effects may have important clinical implications, including increased difficulty in cataract surgery, ocular surface disease and eyelid margin malpositions.

Keywords: eyelid; lacrimal; orbit; pseudoexfoliation; skin.

Figure 1

Characteristic appearance of pseudoexfoliation syndrome in the anterior ocular segment in a 75-year-old male patient. (A) Whitish flakes of pseudoexfoliative material visible in the pupillary plane. (B) Deposition of the material in the form of a membrane on the anterior lens capsule (blue arrow).

Figure 2

Inadequate intraoperative mydriasis in cataract surgery of an eye with PEX in a 87-year-old female patient, necessitating the use of iris hooks to enhance pupil dilatation. Despite this measure, a posterior capsular rupture occurred in this case (blue arrow), a frequently occurring complication during cataract surgery in eyes with PEX. Magnification, x18. PEX, pseudoexfoliation syndrome.

Figure 3

Horizontal and vertical eyelid laxity in the presence of pseudoexfoliation syndrome. (A) Lateral traction test and (B) pinch test from a 68-year-old male patient. Eyelid laxity in the presence of pseudoexfoliation syndrome predisposes to the patient to eyelid margin malpositions, such as (C) inward rotation (entropion) from a 83-year-old female patient or (D) outward rotation (ectropion) from an 71-year-old male patient, both shown with blue arrows.

Figure 4

Periocular fat atrophy. (A) Fat atrophy with deepening of the superior palpebral sulcus (blue arrows) in a 69-year-old male patient with pseudoexfoliation syndrome glaucoma under treatment with bimatoprost (blue arrows). (B) Associated enophthalmos (retro-grade displacement of the globe), more pronounced in the left eye of the same patient (blue arrow), visible when examining from an inferior point of view.

Figure 5

Schematic representation of periocular in pseudoexfoliation syndrome. (A) Orbital changes include orbital fat atrophy and enophthalmos (red arrow 1) and biomechanical changes in optic nerve and orbital vasculature (red arrow 2). (B) Eyelid, lacrimal and conjunctival changes include conjunctival chalasis and lacrimal deficiency (red arrows 3) and horizontal and vertical eyelid laxity (red arrows 4).