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 .
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]
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]
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]
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 .
If a secondary angle closure associated with PEX and unstable capsular bag-CTR-IOL complex is encountered, IOL explantation and replacement by an iris-fixated IOL is a simple and effective treatment option. [ncbi.nlm.nih.gov]
An additional one third required treatment to control intraocular pressure or developed glaucoma over the next ten years. Patients with PEX have a 64% chance of requiring glaucoma treatment by 10 years. [iovs.arvojournals.org]
A better understanding of normal eye function may in turn lead to more accurate diagnosis and prognosis of ocular development, and inevitably to the emergence of novel classifications based on knowledge of the molecular pathology. [clinicaltrials.gov]
CONCLUSIONS: Our study for the first time provides evidence that PLR and NLR may be useful for predicting the prognosis of PEX patients and progression to PXG. [ncbi.nlm.nih.gov]
Prognosis… Worse than in POAG the IOP is significantly elevated and exhibits greater fluctuation. [slideshare.net]
And it is important, he added, because “the prognosis for exfoliation glaucoma is worse than for primary open-angle glaucoma.” “The gene is called LOXL1,” said Dr. Allingham. [eyeworld.org]
The exact pathophysiology and etiology of PEX and associated glaucoma remains obscure. [ncbi.nlm.nih.gov]
It is generally associated with an open angle glaucoma, rendering it one of the most common etiological factors for this disease. [symptoma.com]
Etiology Etiology is unknown. It may a generalized disorder involving abnormal production or turnover of extracellular matrix in the basement membrane. [eyewiki.aao.org]
Keywords: 352 clinical (human) or epidemiologic studies: natural history • 353 clinical (human) or epidemiologic studies: outcomes/complications • 354 clinical (human) or epidemiologic studies: prevalence/incidence 2002, The Association for Research in [iovs.arvojournals.org]
KEYWORDS: Exfoliatiation syndrome – epidemiology; Prevalence; Elderly; Glaucoma [ncbi.nlm.nih.gov]
The database of the Rochester Epidemiology Project was used to identify all patients residing in Olmsted County who were newly diagnosed with pseudoexfoliation syndrome from 1976 to 1991. [doi.org]
The treatment of hyperhomocysteinemia by taking low-cost vitamin B12 and folic acid preparations may prevent additional vascular problems. [ncbi.nlm.nih.gov]
Prevention Is there anything one can do to prevent pseudoexfoliation syndrome? Much research is ongoing to try to understand the environmental risk factors that predispose one to developing pseudoexfoliation in the first place. [brightfocus.org]
My hope is that this implantation method will prevent late dislocation, because the bag supports the lens, the lens haptics in the sulcus support the bag, the CTR expands the bag, and most importantly, the optic stents the CCC, thus preventing progressive [crstoday.com]
- Al-Saleh SA, Al-Dabbagh NM, Al-Shamrani SM, et al. Prevalence of ocular pseudoexfoliation syndrome and associated complications in Riyadh, Saudi Arabia. Saudi Med J. 2015;36(1):108–112.
- Andrikopoulos GK, Alexopoulos DK, Gartaganis SP. Pseudoexfoliation syndrome and cardiovascular diseases. World J Cardiol. 2014;6(8):847-854.
- Schlötzer-Schrehardt U. Pseudoexfoliation syndrome: the puzzle continues. J Ophthalmic Vis Res. 2012;7:187–189.
- French DD, Margo CE, Harman LE. Ocular pseudoexfoliation and cardiovascular disease: a national cross-section comparison study. N Am J Med Sci. 2012;4:468–473.
- 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.
- Ritch R, Mudumbai R, Liebmann JM. Combined exfoliation and pigment dispersion: paradigm of an overlap syndrome. Ophthalmology. 2000;107(5):1004–1008.
- Cruciani F, Lorenzatti M, Nazzarro V, Abdolrahimzadeh S. Bilateral acute angle closure glaucoma and myopia by topiramate. Clin Ter. 2009;160(3):215–216.
- 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.