Pseudoexfoliation Syndrome DISEASE entity
Pseudoexfoliation syndrome (PXF or PEX) is an age-related systemic syndrome that targets mainly ocular tissues through the gradual deposition of fibrillary white flaky material from the lens, mainly on the lens capsule, ciliary body, zonules, corneal endothelium, iris, and pupillary margin.
There is a higher prevalence of open-angle glaucoma in about 50% of these patients. Exfoliation (pseudoexfoliation) Syndrome is characterized by fibrillar deposits in the anterior segment of the eye.
The deposits have been found on and in the subconjunctival tissue, pupillary margin, ciliary epithelium, lens epithelium, lens capsule, iris pigment epithelium, trabecular meshwork, cornea, zonules, orbital soft tissues, iris stroma, and iris blood vessels.
These deposits have also been found elsewhere, including in the skin, heart, lungs, liver, kidneys, and elsewhere. The disease may be bilateral and asymmetric. It has been associated with myocardial infarction, cerebrovascular events, and systemic hypertension.
- Increased intraocular pressure
- Possible glaucomatous damage to the optic nerve
- Poor dilation with peripapillary transillumination defect
- Fibrillar white flaky deposits on the anterior lens capsule (Hoarfrost Ring)
- Fibrillar white flaky deposits at the pupillary border
- Hyperpigmentation of the trabecular meshwork (TM) with an open angle on gonioscopy
- Sampaolesi line, an abundance of linear pigment anterior to the TM at or anterior to Schwalbe’s line (not pathognomic; also seen in pigment dispersion syndrome)
No associated symptoms aside from possibly significantly increased intraocular pressure
- Genetic test for a single mutation in the LOXL1
- Check for Homocysteine levels in the tear film and plasma. Scientists believe that elevated levels of plasma homocysteine are a risk factor for cardiovascular disease, and two studies have found higher levels of plasma and tear fluids homocysteine levels in pseudoexfoliation patients.
- Lens capsule deposite: true exfoliation syndrome
- Trabecular meshwork hyperpigmentation/ glaucoma: Pigment dispersion syndrome
- Iris transillumination defects: Pigment dispersion syndrome, trauma, prior herpes infection
MANAGEMENT of Pseudoexfoliation Syndrome
- Routine regular eye exam
- Glaucoma monitoring and treatment if warranted
- Special surgical considerations for cataract surgery pre-operatively, intra-operatively and post-operatively
- Drops if the patient has glaucoma
- Possible use of antioxidants
- Lower Homocysteine if the level is high in plasma or tear film
Medical follow up
Routine annual eye screening exam with dilation.
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- Schlötzer-Schrehardt U, Naumann GO. Ocular and systemic pseudoexfoliation syndrome. Am J Ophthalmol. 2006 May;141(5):921-937.
- Jeng SM, Karger RA, Hodge DO, Burke JP, Johnson DH, Good MS. The risk of glaucoma in pseudoexfoliation syndrome. J Glaucoma. 2007 Jan;16(1):117-21.
- Plateroti P, Plateroti AM, Abdolrahimzadeh S, Scuderi G. Pseudoexfoliation Syndrome and Pseudoexfoliation Glaucoma: A Review of the Literature with Updates on Surgical Management. J Ophthalmol. 2015;2015:370371.
- Elhawy E, Kamthan G, Dong CQ, Danias J. Pseudoexfoliation syndrome, a systemic disorder with ocular manifestations. Hum Genomics. 2012 Oct 10;6(1):22.
- Zenkel M, Kruse FE, Jünemann AG, Naumann GO, Schlötzer-Schrehardt U. Clusterin deficiency in eyes with pseudoexfoliation syndrome may be implicated in the aggregation and deposition of pseudoexfoliative material. Invest Ophthalmol Vis Sci. 2006 May;47(5):1982-90.