What defines pseudoexfoliation syndrome (PXF)?

Age-related deposition of fibrillary white flaky material on ocular tissues PXF is an age-related systemic syndrome in which fibrillary white flaky material derived from the lens is gradually deposited on ocular structures.

Autoimmune destruction of the trabecular meshwork endothelium

Congenital failure of anterior chamber angle cleavage

Progressive corneal endothelial cell loss from viral infection

Approximately what proportion of pseudoexfoliation syndrome patients have open-angle glaucoma?

About 50% The article reports a higher prevalence of open-angle glaucoma, in about 50% of patients with pseudoexfoliation syndrome.

Beyond the eye, where else has pseudoexfoliation material been found?

Skin, heart, lungs, liver and kidneys Pseudoexfoliation deposits have been found in the skin, heart, lungs, liver and kidneys, and the condition is associated with myocardial infarction, cerebrovascular events and hypertension.

Only within the orbital soft tissues

Exclusively in the cerebrospinal fluid

Bone marrow and synovial membranes only

What is the Hoarfrost ring seen in pseudoexfoliation syndrome?

Fibrillar white flaky deposits on the anterior lens capsule The Hoarfrost ring describes the fibrillar white flaky deposits on the anterior lens capsule, one of the classic slit-lamp signs of PXF.

A ring of iron deposited in the corneal epithelium

A circular band of iris transillumination defects

Pigment deposited on the corneal endothelium

What is the Sampaolesi line seen on gonioscopy in pseudoexfoliation syndrome?

Linear pigment at or anterior to Schwalbe's line The Sampaolesi line is an abundance of linear pigment anterior to the trabecular meshwork at or anterior to Schwalbe's line; it is not pathognomonic and also occurs in pigment dispersion syndrome.

A break in Descemet membrane running horizontally

A wavy deposit of flaky material on the ciliary body band

A pigmented line marking the scleral spur posteriorly

What symptoms do patients with pseudoexfoliation syndrome typically report?

None, other than effects of possibly raised intraocular pressure PXF has no associated symptoms apart from those relating to a possibly significantly increased intraocular pressure, which is why it is a hidden cause of vision loss.

Coloured haloes and severe ocular pain

Progressive night blindness and field constriction

Persistent foreign body sensation and tearing

A mutation in which gene is tested for in pseudoexfoliation syndrome?

LOXL1 Laboratory testing in PXF includes a genetic test for a single mutation in LOXL1.

Which laboratory marker is found at higher levels in pseudoexfoliation patients?

Homocysteine in plasma and tear fluid Two studies found higher plasma and tear fluid homocysteine levels in pseudoexfoliation patients; elevated plasma homocysteine is also a cardiovascular risk factor.

Serum angiotensin converting enzyme

Which condition is the main differential for trabecular meshwork hyperpigmentation in pseudoexfoliation syndrome?

Pigment dispersion syndrome The article lists pigment dispersion syndrome as the differential for trabecular meshwork hyperpigmentation and glaucoma, and also for iris transillumination defects along with trauma and prior herpes infection.

Fuchs heterochromic iridocyclitis

What follow-up is recommended for a patient with pseudoexfoliation syndrome?

Routine annual eye screening examination with dilation Management is routine regular examination with annual dilated screening, glaucoma monitoring and treatment if warranted, plus special considerations around cataract surgery.

Monthly gonioscopy for the first year

Review only if visual symptoms develop

Six-monthly optical coherence tomography of the macula

Pseudoexfoliation Syndrome: A Hidden Cause of Vision Loss

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.

Physical examination

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Signs:

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)

Symptoms:

No associated symptoms aside from possibly significantly increased intraocular pressure

Laboratory test

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.

Differential diagnosis

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

Medical therapy

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.

REFERENCES

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.

Test yourself

A few questions straight from this article.

1 / 10 0 correct

What defines pseudoexfoliation syndrome (PXF)?
PXF is an age-related systemic syndrome in which fibrillary white flaky material derived from the lens is gradually deposited on ocular structures.
Approximately what proportion of pseudoexfoliation syndrome patients have open-angle glaucoma?
The article reports a higher prevalence of open-angle glaucoma, in about 50% of patients with pseudoexfoliation syndrome.
Beyond the eye, where else has pseudoexfoliation material been found?
Pseudoexfoliation deposits have been found in the skin, heart, lungs, liver and kidneys, and the condition is associated with myocardial infarction, cerebrovascular events and hypertension.
What is the Hoarfrost ring seen in pseudoexfoliation syndrome?
The Hoarfrost ring describes the fibrillar white flaky deposits on the anterior lens capsule, one of the classic slit-lamp signs of PXF.
What is the Sampaolesi line seen on gonioscopy in pseudoexfoliation syndrome?
The Sampaolesi line is an abundance of linear pigment anterior to the trabecular meshwork at or anterior to Schwalbe's line; it is not pathognomonic and also occurs in pigment dispersion syndrome.
What symptoms do patients with pseudoexfoliation syndrome typically report?
PXF has no associated symptoms apart from those relating to a possibly significantly increased intraocular pressure, which is why it is a hidden cause of vision loss.
A mutation in which gene is tested for in pseudoexfoliation syndrome?
Laboratory testing in PXF includes a genetic test for a single mutation in LOXL1.
Which laboratory marker is found at higher levels in pseudoexfoliation patients?
Two studies found higher plasma and tear fluid homocysteine levels in pseudoexfoliation patients; elevated plasma homocysteine is also a cardiovascular risk factor.
Which condition is the main differential for trabecular meshwork hyperpigmentation in pseudoexfoliation syndrome?
The article lists pigment dispersion syndrome as the differential for trabecular meshwork hyperpigmentation and glaucoma, and also for iris transillumination defects along with trauma and prior herpes infection.
What follow-up is recommended for a patient with pseudoexfoliation syndrome?
Management is routine regular examination with annual dilated screening, glaucoma monitoring and treatment if warranted, plus special considerations around cataract surgery.