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].
Entire Body System
- Weakness
Shingleton, MD, presents his surgical approach to a mature cataract in the presence of pseudoexfoliation, anterior chamber asymmetry, and a possible zonular weakness. [eyetube.net]
Iris hooks also can serve to support the anterior capsulotomy if zonular weakness is significant. Care should be taken when handling the iris because injury to this tissue can occur easily. Zonular weakness. [aao.org]
Capsular tension rings (CTR) are frequently used to prevent postoperative complications caused by zonular weakness. [ncbi.nlm.nih.gov]
Poor pupillary dilation in one eye indicates a greater risk for zonular weakness on that side, as well as a shallow or deep anterior chamber or a depth that fluctuates based on patient position. [eyeworld.org]
If the zonules are weak or absent, the cataract can move beyond the zone of control of the ophthalmic surgeon, even when perfect surgical technique is used. [huntingtoneyecare.com]
- Inflammation
Laser flare photometry was used in this study to evaluate subtle amounts of postoperative inflammation. [touchophthalmology.com]
CONCLUSION: The addition of bromfenac to topical steroids after cataract surgery in eyes with PXF was associated with greater reductions in inflammation than steroids alone. [ncbi.nlm.nih.gov]
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. [symptoma.com]
Skin
- Hyperpigmentation
Diagnostic procedures Slit lamp exam Intraocular pressure check Pupil check and transillumination exam Dilated eye exam to check optic nerve Gonioscopy to check for trabecular meshwork hyperpigmentation and open angle Laboratory test Genetic test for [eyewiki.aao.org]
Gonioscopy… ▪ Trabecular hyperpigmentation-most marked inferiorly, patchy distribution ▪ Scalloped band of pigment running on to or anterior to Schwalbe line Sampaolesi line ▪ PXF deposits in the trabeculum can give rise to a ‘dandruff-like’ appearance [slideshare.net]
All pseudoexfoliative glaucoma patients had trabecular hyperpigmentation on gonioscopy. On fundus examination these patients had cup disc ratio of >0.6.Visual field testing revealed arcuate scotoma. [scopemed.org]
Musculoskeletal
- Osteoporosis
KEYWORDS: Exfoliation syndrome; osteomalacia; osteoporosis; vitamin D deficiency [ncbi.nlm.nih.gov]
Failure to diagnose and treat osteoporosis in elderly patients hospitalized with hip fracture. (Ed. 2008;1151818в25. [luxbar-starway.ru]
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]
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]
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]
- Conjunctival Disease
disease 臨床眼科医報 第101巻 第6号(2007年6月)別冊 新しいアデノウィルス結膜炎迅速診断キットの検討 instant diagnostic kit for adenoviral conjunctivitis 臨床眼科 第58巻 第8号 別刷 2004年8月15日発行 PDFファイルを御覧頂くには「Adobe Acrobat Reader」が必要です。 [ohashi-eye.jp]
- Ectopia Lentis
Capsule 221 Lens Epithelium 222 Lens Fibers 224 Suspensory Apparatus 225 Wiegers Ligament 226 Lens Capsule Epithelium Fibers and Zonules 227 Biochemical Changes 228 Surgical Pathology of the Lens 229 Lenticonus and Lentiglobus 230 Lens Dislocations Ectopia [books.google.com]
Psychiatrical
- Withdrawn
Trial Phase, Sponsor Type and End point status The report reviews top companies involved and enlists all trials (Trial title, Phase, and Status) pertaining to the company The report provides all the unaccomplished trials (Terminated, Suspended and Withdrawn [gii.co.jp]
Withdrawn Application number EP91113398A Other languages German ( de ) French ( fr ) Inventor Thomas Prof. Dr. Med. Neuhann Current Assignee (The listed assignees may be inaccurate. [google.tl]
Glaucoma may affect a person's ability to drive and may mean their licence to do so is withdrawn. See the link in 'Further reading & references' below, regarding the DVLA rules relating to ability to drive. [patient.info]
Neurologic
- Mydriasis
It becomes obvious intraoperatively after mechanical mydriasis and signals zonular instability. [atlasophthalmology.net]
METHODS: All 674 cataract patients had a comprehensive eye examination, including slitlamp biomicroscopy before and after mydriasis, IOP measurement, and fundus examination. [ncbi.nlm.nih.gov]
Intraoperative observations included anterior chamber depth, pupil size after maximal mydriasis, zonular dehiscence, capsule tear, vitreous loss and postoperatively: best corrected visual acuity, intraocular pressure (IOP), aqueous flare response and [pesquisa.bvsalud.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].
Treatment
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]
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]
Prognosis
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 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]
Etiology
The exact pathophysiology and etiology of PEX and associated glaucoma remains obscure. [ncbi.nlm.nih.gov]
Patients with glaucoma of any etiology exhibit elevated aqueous humor TGFβ, a cytokine that both regulates matrix formation and causes increases in IOP., Fuchshofer R. [oraclinical.com]
It is generally associated with an open angle glaucoma, rendering it one of the most common etiological factors for this disease. [symptoma.com]
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 [iovs.arvojournals.org]
KEYWORDS: Exfoliatiation syndrome – epidemiology; Prevalence; Elderly; Glaucoma [ncbi.nlm.nih.gov]
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]
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]
Pathophysiology
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
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]
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]
The treatment of hyperhomocysteinemia by taking low-cost vitamin B12 and folic acid preparations may prevent additional vascular problems. [ncbi.nlm.nih.gov]
References
- 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.