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 [1]. Advanced PEX frequently requires surgical intervention incurring the risk of complications [2].

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 [3]. 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 [4].

• Sparse Hair

  Diagnosis was based on clinical features, including developmental delay, sparse hair, and craniofacial features along with de novo mutation in SMARCA2. Eye findings included bilateral glaucoma, cataracts, and degenerative vitreoretinopathy. [medworm.com]

• Splinter Hemorrhage

  Diagnosis of glaucoma was made if: intra ocular pressure (IOP) ≥ 22 mm Hg in either eye; vertical cup disc ratio (VCDR) ≥ 0.7 or a cup disc ratio (CDR) asymmetry ≥ 0.2; and focal thinning, notching, or a splinter hemorrhage. [bmcophthalmol.biomedcentral.com]

• Iridodonesis

  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]

  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]

  Zonular integrity should be evaluated preoperatively at the slit lamp by looking for the presence of phacodonesis or iridodonesis. [tmj.ro]

  RESULTS: Preoperative slit lamp examination showed phacodonesis in 17.91% (47), iridodonesis in 2.98% (8), pigment dispersion in 6.72% (18), lens subluxation in 4.85% (13) on the total. [healthdocbox.com]

• Retinal Pigmentation

  Choroidal thickness was measured manually from the outer border of the retinal pigment epithelium to the inner scleral border at the subfovea, 3 mm temporal to the fovea, and 3 mm nasal to the fovea using EDI-OCT. [ncbi.nlm.nih.gov]

  ARMD was defined as the presence on examination of any of the following: (1) Soft or dry drusen within the posterior pole (2) Choroidal neovascular membrane or disciform macular scar (3) Atrophy of the retinal pigment epithelial membrane. [bjo.bmj.com]

  Prostaglandins can cause breakdown of the blood-retinal barrier, including vasodilation, increased capillary permeability from compromise of tight endothelial junctions in the retinal capillaries, and decreased removal of fluid by the retinal pigment [emedicine.medscape.com]

  Expression of CLU in the human eye has been previously reported in the cornea, ciliary body, lens, retina, retinal pigment epithelium, and aqueous and vitreous humor [ 30 - 33 ]. [molvis.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]

  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]

  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]

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 [5].

Pseudoexfoliative flakes in addition to pigmented endothelium of the cornea may mimic inflammatory precipitates or be misinterpreted as pigment dispersion syndrome [6]. 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 [5].

Ultrasound biomicroscopy (UBM) can be useful in cases of iridodonesis and subluxation of the lens [7].

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 [5].

Applanation tonometry is used to measure the intraocular pressure, which is commonly raised in PEX [8].

• Ventricular Hypertrophy

  Patients with overt coronary artery disease, congestive heart failure, valvular heart disease, cardiomyopathy, and left ventricular hypertrophy were excluded from the study. [nature.com]

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]

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]

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]

Prognosis - worse prognosis: because the IOP is high relative to POAG. poor response to medication : leads to faster optic nerve damage increased risk of developing cataracts. Postoperative complications of cataract extraction. [slideshare.net]

Prognosis Pseudoexfoliation glaucoma has a worse prognosis than primary open-angle glaucoma because the IOP is high relative to POAG, and pseudoexfoliation glaucoma’s poor response to medication leads to faster optic nerve damage, and to visual field [aao.org]

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]

Prognosis Overall, 50% of patients with PXF are ultimately diagnosed with glaucoma. Therefore annual assessment is required. [eyewiki.aao.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 [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]

In this recent article published in the journal Ophthalmic Epidemiology, scientists in Spain conducted a study "To estimate the prevalence of Pseudoexfoliation syndrome (PXF) and to investigate its relationship with systemic and ocular diseases and lifestyle [web.archive.org]

Network analysis suggested a central role of fibronectin in the pathophysiology of the disease. [ncbi.nlm.nih.gov]

“Elucidation of the pathophysiology and genetics of pseudoexfoliation, and application of directed treatment could eventually lead to the eradication of this disease.” Editors’ note : Drs. [eyeworld.org]

Pathophysiology Unclear, but there is a gentic link to the gene LOXL1. In March 2008 of the American Glaucoma Society in Washington, D.C. Dr. [eyewiki.aao.org]

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]

The treatment of hyperhomocysteinemia by taking low-cost vitamin B12 and folic acid preparations may prevent additional vascular problems. [ncbi.nlm.nih.gov]

Pseudoexfoliation syndrome cannot be prevented or cured but can be managed effectively in cooperation with your doctor. There is some evidence to show that sun exposure plays a role and sunglasses should be worn when outdoors. [eyecarenj.com]

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8. 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.