What is Pseudoexfoliation Syndrome : Causes, Symptoms, Treatment, Prognosis, Pathophysiology, Risk Factors, Complications

Pseudoexfoliation syndrome is a condition that ails the eye by forming flaky, granular white material which gets deposited on the ocular tissues, through the lens. The affected ocular tissues include lens capsule, zonules, corneal endothelium, ciliary body, pupillary margin and iris. It is an age related condition which increases the risk of open angle glaucoma and cataract formation by fifty percent.

Pseudoexfoliation syndrome is a phenomenon that affects several organs like lungs, heart, liver, skin, kidneys, eyes and many more. The condition manifests itself and is more commonly visible in the eye.

Pseudoexfoliation syndrome is also known as PXF or PEX; it is an age related condition which creates dandruff like flakes of proteins, on the surface of the lens of the eye, pupillary margin of iris, zonules, cornea, ciliary body and anterior vitreous. It gives an exfoliated appearance to the single or both the eyes. Thus, the name comes as pseudoexfoliation syndrome.

This condition can be recognized easily as the eye has a visible “three ring” sign on the lens. The anterior part of the lens capsule forms a cloudy white granular appearance on the periphery whereas a clear zone in between. Furthermore, there is also witnessed pigment loss from the iris sphincter region.

This flaky material gets collected between the iris and cornea, which further clogs the fluid drainage within the eyes. This blockage further increases the risk of glaucoma as it elevates the fluid pressure within the eye. Such kind of glaucoma is called pseudoexfoliative glaucoma.

The most prominent symptom of pseudoexfoliation syndrome is the presence of white flake ring on the periphery of the lens and clear zone in the middle. The other symptoms are:

• Altered visual perception
• Less visual awareness.

The incidence rate of pseudoexfoliation syndrome is quite high in the US, India, Pakistan, UAE with an occurrence of over seven percent in over a 100,000 cases. The prevalence of pseudoexfoliation syndrome is also common in European nations but the incidence rate is slightly less than that of the above mentioned countries.

The outlook for pseudoexfoliation syndrome is not that good as more than half of the ailing cases are ultimately diagnosed with glaucoma. These cases that ail form pseudoexfoliation syndrome related glaucoma have further more trouble in responding to the medications which otherwise work well with regular glaucoma patients, and hence further increases he need for surgery.

There are no definitive proofs of causative factors behind the condition, but there definitely are certain speculations. These are:

• Presence of excessive free radicals that cause oxidative damage to the eye.
• Decreased levels of ascorbic acid and raised levels of malondialdehyde and prostaglandin F2a.
• The mutation in the gene associated with collagen and elastin, known as LOXL1.

Though the exact etiology behind the pseudoexfoliation syndrome is unknown but the condition is known to be associated with the abnormalities within the basal membrane in the epithelial cells. The epithelial cells of iris, ciliary body, lens capsules, zonules and pupillary margin release an amyloid like substance which is pseudoexfoliative. It further moves to the aqueous humor and then to the trabecular meshwork, by following the normal drainage flow of the eye. The substances released blocks the mesh which further raises the ocular pressure and gives rise to glaucoma.

The common risk factors that pose a threat of pseudoexfoliation syndrome are:

• Age above 50 years; the higher the age, greater is the risk.
• Origin in Scandinavian and Mediterranean countries.
• Female gender; prevalence of pseudoexfoliation syndrome is more in women in comparison to men.
• Presence of a variant of LOXL1 gene.

The complications that upsurge as a result of pseudoexfoliation syndrome are:

• Pseudoexfoliation syndrome related glaucoma
• Presence of complications related to cataract surgery like Lens dislocation, phacodenesis or vibration of eye lens, loss of lens nucleus and a need for zonular dialysis.

The frequently used diagnostic procedures followed to diagnose pseudoexfoliation syndrome are:

• Slit Lamp Examination to Diagnose Pseudoexfoliation Syndrome: It involves examination of eye using low powered microscope along with a slit lamp having high intensity light. It is done to check for abnormalities in the anterior segment of eye.
• Intraocular Pressure Check to Diagnose Pseudoexfoliation Syndrome: The test involves checking of pressure inside the eye, because the increased pressure in the eye, increases the risk of glaucoma.
• Checking the Pupil: The pupil check is done to check for abnormalities within the pupil, by checking the reaction of each eye towards light.
• Trans-illumination Check to Detect Pseudoexfoliation Syndrome: It is a painless procedure that checks for abnormalities in eye by using bright light inside a dark room.
• Dilated Eye Examination: In this diagnosis, the eye is dilated using medicated drops and then tested for abnormalities and sensitivity. The results obtained from dilated eyes are more accurate then un-dilated eyes.
• Gonioscopy Test: In gonioscopy, the anterior segment of the eye between cornea and iris is examined to check whether the drainage angle of the eye is open or closed.

Pseudoexfoliation syndrome is untreatable, but the damage due to it can be minimized to prevent the chances of glaucoma. The available treatments to minimize the damage of pseudoexfoliation syndrome are:

• Non-Surgical Measures to Treat Pseudoexfoliation Syndrome: The non-surgical measures include:
  • Eye Drops: These are the first line of treatment. The eye drops reduce the pressure in the eye. The drops contain beta blockers and prostaglandin analogues, which slow down the production of aqueous humor and increase its outflow.
• Surgical Measures for Treating Pseudoexfoliation Syndrome: Surgery is the last measure for the treatment of pseudoexfoliation syndrome. The available surgeries are:
  • Laser Surgery: It uses high energy laser beam that targets the trabecular meshwork, to improve the outflow of aqueous humor.
  • Eye Surgery: The eye surgery involves the removal of the white portion from the lens, to allow the aqueous humor discharge and lowering the pressure within the eye.

The only precautionary measure is to go for a routine ophthalmologist checkup of eye for people above the age of 50.

Pseudoexfoliation syndrome is a common old age eye condition, in which white flaky granular substances develop on the lens capsule which in most cases, gives rise to glaucoma. These substances cause blockage within the eye by not allowing aqueous humor to flow, which raise the pressure within the eye, causing glaucoma. The condition is not treatable but the available treatments like eye surgery and laser surgery helps in minimizing the damage caused by the condition.

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