Pseudoexfoliation syndrome is a multifactorial disease defined by deposition of granular flakes in the anterior chamber of the eye. It is generally associated with an open angle glaucoma, rendering it one of the most common etiological factors for this disease.
Pseudoexfoliation syndrome (PEX) causes granular flake accumulation in the trabecular meshwork, blocking the physiological drainage of aqueous humor. Consequently, the obstruction creates an increase in the intraocular pressure that is typically asymptomatic and painless. For that reason, this syndrome is often diagnosed late in the course of the disease. Symptoms like a disturbance in the visual acuity or decreased field of vision may manifest in tandem with increased intraocular pressure. Advanced disease may cause injury to the optic nerve and subsequent loss of vision . Advanced PEX frequently requires surgical intervention incurring the risk of complications .
History of patients may reveal cases of PEX in the family. Non-hereditary factors include regular exposure to ultraviolet light, episodes of infections and inflammation, as well as physical injury to the eye. Likewise, hypoxic events can contribute to the progression of the disease . Additionally, inquiry about the cardiovascular system may be useful in future management as PEX is thought to be associated with coronary heart disease, aortic aneurysms, and cardiomyopathies .
As the eye is usually dilated for examination, iridodonesis is usually not apparent if there is lens subluxation. Questions: 1. What is the risk of glaucoma in pseudoexfoliation syndrome? 2. What may you see on gonioscopy? 3. [mrcophth.com]
Zonular integrity should be evaluated preoperatively at the slit lamp by looking for the presence of phacodonesis or iridodonesis. [tmj.ro]
Ultrasound biomicroscopy (UBM) can be useful in cases of iridodonesis and subluxation of the lens. Gonioscopy is an essential examination needed for PEX diagnosis. [symptoma.com]
Iridodonesis, phacodonesis, asymmetry of anterior chamber depth, and lens subluxation are manifestations of zonular weakness, but these signs may or may not be present preoperatively depending on the severity of the disease. [eyeworld.org]
- Blurred Vision
Two months later, she had blurred vision in the right eye with the best-corrected visual acuity (BCVA) of 20/40. [ncbi.nlm.nih.gov]
Case report Two years prior to presentation a 70-year-old woman had cataract surgery with placement of a posterior chamber IOL in the right eye.1 Since surgery, she had bouts of blurred vision associated with combinations of anterior segment inflammation [eyeworld.org]
Patients don’t get blurred vision from it, and that stops the pigment from rubbing off the iris and the iris from rubbing the exfoliation material off the lens. You stop clogging the meshwork.” [healio.com]
It causes severe, progressive inflammation of both the choroid and retina and symptoms include blurred vision, photopsia, and loss of color vision. [healthcare.utah.edu]
- Cotton Wool Spots
[…] narrowing of the retinal arteries, Group II: narrowing of the retinal arteries in conjunction with regions of focal narrowing and arteriovenous nicking, Group III: abnormalities seen in groups I and II, as well as retinal haemorrhages, hard exudation, and cotton-wool [doi.org]
Diagnosis of pseudoexfoliation syndrome (PEX) is based on the slit lamp examination, gonioscopy, and applanation tonometry. Changes in the anterior chamber are both unilateral and bilateral and are revealed by the slit lamp examination. Slit lamp exam findings include deposits of whitish substance in the anterior chamber and lens capsule with an arrangement of a double concentric bull's eye pattern created by iris movements on the lens surface. This type of pattern is best observed during mydriasis. Furthermore, the majority of cases in PEX present with three distinguishable areas on examination:
- Inner area consists of a centered round zone correlating to the diameter of the pupil.
- Intermediate area lies between the internal and external area and is clear due to the mobility of the iris.
- External area is characterized by radial striations that are always present in PEX .
Pseudoexfoliative flakes in addition to pigmented endothelium of the cornea may mimic inflammatory precipitates or be misinterpreted as pigment dispersion syndrome . The iris itself is known to be the target of flake accumulation on both anterior and posterior surfaces. Its margins may exhibit uneven borders caused by the friction on the lens; the margins contain the grayish deposits seen on examination. The iris in PEX can also appear rigid and resistant to contraction possibly due to fibrotic changes in the sphincter .
Gonioscopy is an essential examination needed for PEX diagnosis. The findings include modification in angle and pigmentation of the trabecular meshwork and pseudoexfoliative material deposits that are noticeable mainly in the Schwalbe line .
Applanation tonometry is used to measure the intraocular pressure, which is commonly raised in PEX .
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- Rao A, Padhy D. Pattern of Pseudoexfoliation Deposits on the Lens and Their Clinical Correlation- Clinical Study and Review of Literature. PLoS One. 2014; 9(12): e113329.