Pseudoexfoliation Glaucoma 

  • Author: Mauricio E Pons, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Aug 4, 2011
 

Background

In 1917, a Finnish ophthalmologist named Lindberg first described pseudoexfoliation syndrome. This entity is characterized by flakes of granular material at the pupillary margin of the iris and throughout the inner surface of the anterior chamber. It is also associated with secondary open-angle glaucoma, known as pseudoexfoliation glaucoma, which is the most common identifiable form of secondary open-angle glaucoma worldwide. Dvorak-Thebold suggested the term pseudoexfoliation to differentiate it from true exfoliation or lamellar delamination of the lens capsule found in glassblowers. True exfoliation syndrome is due to heat or infrared-related changes in the anterior lens capsule.

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Pathophysiology

Pseudoexfoliation syndrome is a common ocular manifestation of a systemic disease, known to cause disease primarily in the eye. Exact etiology of this condition remains unknown.

Pseudoexfoliation material is associated with abnormalities of the basement membrane in epithelial cells and has a wide distribution throughout the body. Pseudoexfoliative material has been found in the walls of the vortex veins and the central retinal artery. Extraocular tissues involved include lung, skin, liver, heart, kidney, gallbladder, blood vessels, extraocular muscle, connective tissue in the orbit, and meninges. In the anterior segment of the eye, it is characterized by deposition of pseudoexfoliative amyloidlike material on the anterior lens capsule, ciliary body, zonules, pupillary margin of the iris, corneal endothelium, anterior vitreous, and trabecular meshwork.

Some investigators believe that the iris pigment epithelium, the ciliary epithelium, and the peripheral anterior lens epithelium produce this pseudoexfoliative amyloidlike material, which moves into the aqueous humor and is carried to the trabecular meshwork, following the normal flow. Obstruction of the trabecular meshwork by this fibrillar material and pigment associated with degenerative changes in the Schlemm canal and the juxtacanalicular area causes elevation of the intraocular pressure (IOP) with associated glaucoma.

Zenkel et al have studied genes differentially expressed in anterior segment tissues and have postulated that pseudoexfoliation syndrome is a stress-induced elastic microfibrillopathy.[1]

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Epidemiology

Frequency

United States

Roth and Epstein reported that pseudoexfoliation glaucoma was present in 12% of patients with glaucoma.[2] Kozart and Yanoff reported that glaucoma was present in 7% of 100 consecutive patients with pseudoexfoliation syndrome in Philadelphia.[3] In the Framingham study, prevalence of pseudoexfoliation syndrome was 1.8%. In a prospective study, Cashwell and Shields found that the prevalence of pseudoexfoliation syndrome in the southeastern United States was 1.6% of the total population and in 6% of an open-angle glaucoma subpopulation.[4] Prevalence of pseudoexfoliation syndrome in the glaucoma population of south Louisiana was found to be 2.7% in white patients and 0.4% in African American patients. Karger et al determined that the incidence of newly diagnosed pseudoexfoliation syndrome in residents of Olmsted County, Minnesota, was 25.9 cases per 100,000 population.[5]

Because of the increased mean age of populations, pseudoexfoliation syndrome may become more prevalent in the future.

International

Prevalence of pseudoexfoliation syndrome in Europe was found to be 4.7% in England, 6.3% in Norway, 4% in Germany, 1.1% in Greece, and 5.5% in France.

Bartholomew reported an 8.2% prevalence of pseudoexfoliation syndrome in the Bantu tribes of South Africa.[6] In Asia, the prevalence in a Japanese population was 3.4%. Hospital-based studies showed a prevalence of 6.45% in Pakistan and 7.4% in India. A prevalence rate of 0.4% in patients aged 60 years or older was identified in China.

Mortality/Morbidity

In a retrospective study, Shrum et al found no association between ocular pseudoexfoliation and cardiovascular or cerebrovascular mortality.[7] However, other authors have found that pseudoexfoliation is linked with Alzheimer disease, senile dementia, cerebral atrophy, chronic cerebral ischemia, stroke, transient ischemic attacks, heart disease, and hearing loss. Vessani et al found that homocysteine levels were higher among patients with pseudoexfoliation syndrome and pseudoexfoliative glaucoma compared with controls.[8] Roedl et al reported increased homocysteine concentrations in tear fluid and plasma of patients with pseudoexfoliation glaucoma.[9] Elevated plasma homocysteine levels have been described as a risk factor for cardiovascular disease.

Race

Although it occurs in virtually every area of the world, a considerable racial variation exists in the incidence of pseudoexfoliation glaucoma.

  • In Scandinavian countries, more than 50% of cases of open-angle glaucoma are caused by pseudoexfoliation syndrome.
  • Pseudoexfoliation syndrome is relatively rare among African Americans and Eskimos. It was not observed at all in the Inuit who live throughout the Canadian Arctic.
  • Prevalence is high in the Sami people who are indigenous of northern Europe.
  • Among the Bantu tribes of South Africa, exfoliation was found in 19% of patients in a glaucoma clinic.
  • There is a high prevalence of pseudoexfoliation syndrome in Arabic populations. In Saudi Arabia, Summanen and Tonjum reported a prevalence of pseudoexfoliation syndrome of 13%.[10] It has been found to be the cause of half the cases of glaucoma in Oman.[11]
  • Prevalence of pseudoexfoliation syndrome in Spanish Americans in New Mexico was estimated to be 3-6%.

Sex

Pseudoexfoliation syndrome is more common in females than in males. In a series by Kozart and Yanoff, pseudoexfoliation syndrome was 3 times more common in women than in men.[3]

Age

Pseudoexfoliation syndrome is rarely seen before age 50 years, and its incidence increases steadily with age.

  • In Norway, Aasved reported that the prevalence of pseudoexfoliation was 0.4% in individuals aged 50-59 years and 7.9% in individuals aged 80-89 years.[12] The reported mean age of pseudoexfoliation syndrome ranges from 69-75 years.
  • Jonasson et al reported a 10% annual increase for both open-angle glaucoma and pseudoexfoliation in persons aged 50 years and older in Iceland.[13]
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Contributor Information and Disclosures
Author

Mauricio E Pons, MD  Associate Physician, Charles A Garcia, MD, PA

Mauricio E Pons, MD is a member of the following medical societies: American Academy of Ophthalmology and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Coauthor(s)

Babak Eliassi-Rad, MD  Assistant Professor, Director of Glaucoma Service, Department of Ophthalmology, Boston University School of Medicine

Babak Eliassi-Rad, MD is a member of the following medical societies: American Academy of Ophthalmology and American Glaucoma Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Bradford Shingleton, MD  Assistant Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary

Bradford Shingleton, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Ophthalmology

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Martin B Wax, MD  Clinical Professor, Department of Ophthalmology, University of Texas Southwestern Medical School; Vice President, Ophthalmology Research and Development, Head, Ophthalmology Discovery Research, Alcon Labs, Inc

Martin B Wax, MD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Society for Neuroscience

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

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Pseudoexfoliative material can be seen in this eye with pseudoexfoliation glaucoma. Courtesy of S. Fabian Lerner, MD.
 
 
 
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