Pseudoexfoliation Syndrome
Pseudoexfoliation Lens Capsule
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.
Presentation
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].
Skin
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]
Musculoskeletal
Knee Pain
- Knee pain was evaluated using a visual analogue scale (VAS). Knee OA was diagnosed according to American College of Rheumatology (ACR) criteria and graded according to the Kellgren and Lawrence (KL) grading scale on radiographic examination.[ncbi.nlm.nih.gov]
Eyes
Iridodonesis
- 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]
- 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]
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]
- 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]
Workup
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].
Serum
Homocysteine Increased
- Homocysteine metabolism genes Another factor that may be associated with PXG is homocysteine. Increased plasma homocysteine level may be a risk factor for the development of glaucoma [ 46 ].[ncbi.nlm.nih.gov]
Hypertrophy
Ventricular Hypertrophy
- Patients with overt coronary artery disease, congestive heart failure, valvular heart disease, cardiomyopathy, and left ventricular hypertrophy were excluded from the study.[doi.org]
Treatment
- 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]
- From the same database, matched control eyes with similar treatment, age, glaucoma stage, and mean IOP were identified. For each patient, data from multiple extended diurnal IOP profiles were available.[ncbi.nlm.nih.gov]
- Patients were re-examined a mean of 76 (SD 5.4) months after cataract surgery, recording IOP, glaucoma diagnosis, glaucoma treatment and LogMAR.[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]
- Make optimal use of the newest drug therapies, including Anti-VEGF treatment for wet ARMD and bevacizumab treatment for complications of diabetes.[books.google.com]
Prognosis
- 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]
- 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]
- You must recognize secondary glaucomas, the treatment and prognosis often differ from POAG. Pseudoexfoliative glaucoma (PXG) began to appear in scientific literature in the early 1900s.[optometricmanagement.com]
- The present study, however provides no information regarding the long term prognosis of these patients who upon initial diagnosis of exfoliation syndrome, had normal intra ocular -pressure.[ijo.in]
- 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]
Etiology
- The exact pathophysiology and etiology of PEX and associated glaucoma remains obscure.[ncbi.nlm.nih.gov]
- Bullous keratopathy (BK), a severe sight-threatening disorder can have a variety of etiologies such as prophylactic laser iridotomy, intraocular surgery, trauma, and other ocular disorders.[ncbi.nlm.nih.gov]
- The result of a national survey on the etiology of bullous keratopathy is presented. RESULTS: Corneal cell density was decreased in PEX eyes compared with that of normal control eyes.[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]
Epidemiology
- 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]
- METHODS: The study is based on an epidemiological survey conducted in Sør-Trøndelag county, Norway, in 1985-86. All inhabitants over 64 years of age (2109 individuals) were invited.[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]
- We performed a meta-analysis of epidemiologic studies to evaluate this relationship.[ncbi.nlm.nih.gov]
Pathophysiology
- Network analysis suggested a central role of fibronectin in the pathophysiology of the disease.[ncbi.nlm.nih.gov]
- The exact pathophysiology and etiology of PEX and associated glaucoma remains obscure.[ncbi.nlm.nih.gov]
- The levels of TGF-β 3, however, did not attain pathophysiological levels ( 100 pg/ml) in any group. CONCLUSIONS: A stage-dependent increase in the concentrations of TGF-β1 and TGF-β3, but not of TGF-β2, accords to the shrinkage of the capsular bag.[ncbi.nlm.nih.gov]
- 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]
- “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]
Prevention
- The treatment of hyperhomocysteinemia by taking low-cost vitamin B12 and folic acid preparations may prevent additional vascular problems.[ncbi.nlm.nih.gov]
- Capsular tension rings (CTR) are frequently used to prevent postoperative complications caused by zonular weakness.[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]
- Author information 1 Department of Ophthalmology, Faculty of Medicine, Fattouma Bourguiba University Hospital, University of Monastir, 5019, Monastir, Tunisia. 2 Department of Community and Preventive Medicine, Faculty of Medicine, Fattouma Bourguiba[ncbi.nlm.nih.gov]
- 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]
References
Article
- 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.