Glaucoma, Pigmentary 

  • Author: Robert Ritch, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Feb 17, 2010
 

Background

Pigment dispersion syndrome (PDS) is an autosomal dominant disorder characterized by disruption of the iris pigment epithelium (IPE) and deposition of pigment granules on the structures of the anterior segment. Pigment granule accumulation in the trabecular meshwork then leads to progressive trabecular dysfunction and ocular hypertension with or without associated glaucomatous optic neuropathy. Because the age of onset is often in the third or fourth decade of life, this disorder is an important and often underdiagnosed glaucoma affecting younger people.

Pigmentary glaucoma (PG) originally was considered rare. In 1949, Sugar and Barbour described 2 young, myopic men with Krukenberg spindles, hyperpigmented trabecular meshworks and open angles, whose intraocular pressures (IOPs) increased with mydriasis and decreased with pilocarpine.[1] Investigations over the ensuing decades elucidated further features, including bilaterality, association with myopia, and a greater incidence in males.

Although primary open-angle glaucoma (POAG) usually begins after age 40 years, pigment dispersion syndrome and pigmentary glaucoma typically affect younger individuals. The diagnosis of elevated IOP at a young age should prompt the examiner to search for a cause.

Myopia is an important risk factor for the development of pigment dispersion syndrome and is present in approximately 80% of affected individuals. Patients with higher degrees of myopia and deeper anterior segments tend to develop glaucoma at an earlier age. In patients with asymmetric disease, the more affected eye is usually the eye that is more myopic.

Pigment dispersion syndrome appears to be autosomal dominant with incomplete penetration, the phenotype expression of which appears to be increased by the presence of myopia. Several pedigrees have been described with multiple affected members, and at least one genetic locus on chromosome band 7q35 has been identified.

Examples of pigmentary glaucomas are shown in the images below.

To record changes in the pigmentation of the iris,To record changes in the pigmentation of the iris, the illumination beam must be directed coaxially through the pupil so that the retinal reflection appears in areas denuded of pigment granules. This transillumination photograph shows the sectoral defects associated with pigmentary glaucoma. Goniography uses diagnostic mirrored contact lenseGoniography uses diagnostic mirrored contact lenses to overcome corneal refraction and to permit visualization of the filtration angle. The pigment liberated from the iris in pigmentary glaucoma is shown in the angle, clogging the trabecular meshwork and impeding aqueous outflow.
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Pathophysiology

The classic triad of clinical signs of pigment dispersion syndrome consists of a Krukenberg spindle, slitlike, radial, midperipheral iris transillumination defects, and pigment deposition on the trabecular meshwork. The iris tends to have a concave configuration and often inserts into the posterior ciliary body band.

Liberated pigment granules are borne by aqueous currents and deposited on the structures of the anterior segment. The vertical accumulation of these pigment granules along the corneal endothelium is known as a Krukenberg spindle. The spindle tends to be slightly decentered inferiorly and wider at its base than its apex. The spindle generally appears as a central, vertical, brown band up to 6 mm long and up to 3 mm wide. With time, it becomes smaller and lighter and often requires careful examination to identify it.

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Epidemiology

Frequency

United States

This condition is less common than open-angle glaucoma.

Mortality/Morbidity

If disease is not controlled, cupping of optic disk and reduction of visual field can occur.

Race

Pigment dispersion glaucoma almost exclusively affects whites.

Sex

A higher incidence occurs in males.

Age

Onset usually occurs before age 40 years.

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Contributor Information and Disclosures
Author

Robert Ritch, MD  Shelley and Steven Einhorn Distinguished Chair in Ophthalmology, Chief of Glaucoma Service, Surgeon Director, Professor, Department of Ophthalmology, New York Eye and Ear Infirmary, New York Medical College

Robert Ritch, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Ophthalmological Society, Chinese American Medical Society, International College of Surgeons, New York Academy of Medicine, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Coauthor(s)

Yaniv Barkana, MD  Consulting Staff, Glaucoma Unit, Department of Ophthalmology, Assaf Harofe Medical Center

Yaniv Barkana, MD is a member of the following medical societies: Israel Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Andrew I Rabinowitz, MD  Consulting Staff, Department of Ophthalmology, Barnet Dulaney Perkins Eye Center

Andrew I Rabinowitz, MD is a member of the following medical societies: Aerospace Medical Association, American Academy of Ophthalmology, and American Medical Association

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles

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.

References

  1. Sugar HS, Barbour FA. Pigmentary glaucoma: a rare clinical entity. Am J Ophthalmol. 1949;32:90.

  2. [Guideline] U.S. Preventive Services Task Force (USPSTF). Screening for glaucoma: recommendation statement. Rockville (MD): Agency for Healthcare Research and Quality (AHRQ); 2005 Mar.

  3. Campbell DG. Pigmentary dispersion and glaucoma. A new theory. Arch Ophthalmol. Sep 1979;97(9):1667-72. [Medline].

  4. Lichter PR, Shaffer RN. Diagnostic and prognostic signs in pigmentary glaucoma. Trans Am Acad Ophthalmol Otolaryngol. Sep-Oct 1970;74(5):984-98. [Medline].

  5. Chew SJ, Tello C, Wallman J, Ritch R. Blinking indents the cornea and reduces anterior chamber volume as shown by ultrasound biomicroscopy. Invest Ophthalmol Vis Sci. 1994;35 (Suppl):1573.

  6. Uy HS, Chan PS. Pigment release and secondary glaucoma after implantation of single-piece acrylic intraocular lenses in the ciliary sulcus. Am J Ophthalmol. Aug 2006;142(2):330-2. [Medline].

  7. Mora P, Sangermani C, Ghiradini S, Carta A, Ungaro N, Gandolfi SA. Ultrasound Biomicroscopy and Iris Pigment Dispersion: a Case-Control Study. Br J Ophthalmol. Oct 12 2009;[Medline].

  8. Kanadani FN, Dorairaj S, Langlieb AM, Shihadeh WA, Tello C, Liebmann JM, et al. Ultrasound biomicroscopy in asymmetric pigment dispersion syndrome and pigmentary glaucoma. Arch Ophthalmol. Nov 2006;124(11):1573-6. [Medline].

  9. Dinc UA, Kulacoglu DN, Oncel B, Yalvac IS. Quantitative assessment of anterior chamber parameters in pigmentary glaucoma using slit-lamp optical coherence tomography. Eur J Ophthalmol. Jan 13 2010;[Medline].

  10. Harasymowycz PJ, Papamatheakis DG, Latina M, De Leon M, Lesk MR, Damji KF. Selective laser trabeculoplasty (SLT) complicated by intraocular pressure elevation in eyes with heavily pigmented trabecular meshworks. Am J Ophthalmol. Jun 2005;139(6):1110-3. [Medline].

  11. Cantor L. Section 10: Glaucoma. In: Basic and Clinical Science Course. American Academy of Ophthalmology: 1996-1997.

  12. Chaudry I, Wong S. Recognizing glaucoma. In: A Guide for the Primary Care Physician. Vol 99. 1999:247-64.

  13. Gupta N, Weinreb RN. New definitions of glaucoma. Curr Opin Ophthalmol. Apr 1997;8(2):38-41. [Medline].

  14. Hitchings RA. Glaucoma: current thinking. Br J Hosp Med. Mar 20-Apr 2 1996;55(6):312-4. [Medline].

  15. Liesegang TJ. Glaucoma: changing concepts and future directions. Mayo Clin Proc. Jul 1996;71(7):689-94. [Medline].

  16. Qureshi IA. Effects of mild, moderate and severe exercise on intraocular pressure of sedentary subjects. Ann Hum Biol. Nov-Dec 1995;22(6):545-53. [Medline].

  17. Reistad CE, Shields MB, Campbell DG, Ritch R, Wang JC, Wand M. The influence of peripheral iridotomy on the intraocular pressure course in patients with pigmentary glaucoma. J Glaucoma. Aug 2005;14(4):255-9. [Medline].

  18. Shields MB. Textbook of Glaucoma. 4th ed. 1998.

  19. Siddiqui Y, Ten Hulzen RD, Cameron JD, Hodge DO, Johnson DH. What is the risk of developing pigmentary glaucoma from pigment dispersion syndrome?. Am J Ophthalmol. Jun 2003;135(6):794-9. [Medline].

  20. Van Buskirk EM. Medicolegal aspects of glaucoma care. Surv Ophthalmol. Jul-Aug 1998;43(1):83-6. [Medline].

 
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To record changes in the pigmentation of the iris, the illumination beam must be directed coaxially through the pupil so that the retinal reflection appears in areas denuded of pigment granules. This transillumination photograph shows the sectoral defects associated with pigmentary glaucoma.
Goniography uses diagnostic mirrored contact lenses to overcome corneal refraction and to permit visualization of the filtration angle. The pigment liberated from the iris in pigmentary glaucoma is shown in the angle, clogging the trabecular meshwork and impeding aqueous outflow.
 
 
 
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