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Pigmentary Glaucoma Differential Diagnoses

  • Author: Yaniv Barkana, MD; Chief Editor: Hampton Roy, Sr, MD  more...
Updated: Oct 13, 2014

Diagnostic Considerations

Pigment dispersion syndrome (PDS) can usually be easily distinguished from most other abnormalities in which dissemination of pigment is part of the disease process because no other condition that results in the characteristic iris transillumination defects is known. Other disorders associated with signs of pigment dispersion in the disruption of melanoma cells (eg, melanomalytic dispersion), cysts of the iris and ciliary body, postoperative conditions (eg, intraocular lens–iris chafing), and exfoliation syndrome often occur unilaterally.

An increase of pigment dispersion syndrome and pigmentary glaucoma (PG) secondary to iris chafing by intraocular lenses that were implanted in the ciliary sulcus, leading to the removal of the lens and/or trabeculectomy in some cases, has been reported.[10] Phakic intraocular lenses can also result in pigment dispersion syndrome and pigmentary glaucoma. In these conditions, trabecular pigmentation is often less dense and is usually unevenly distributed throughout the circumference of the meshwork. Occasionally, pigment granules in the anterior chamber may be confused with inflammatory cells, leading to a misdiagnosis of uveitis.[11]

The disease process most similar to pigmentary glaucoma is exfoliation glaucoma. In this condition, a loss of pigment occurs from the iris pigment epithelium (IPE), iris transillumination, pigment dispersion in the anterior segment, including Krukenberg spindle, trabecular pigmentation, and intraocular pressure (IOP) elevation. The clinical history combined with a careful slit lamp biomicroscopic examination easily separates the 2 diseases.

The age of onset for exfoliation glaucoma is usually older than 60 years, and onset is rare in persons younger than 40 years. No sexual or racial predilection exists for exfoliation syndrome, although reports seem to indicate a higher prevalence of the disease in individuals of Scandinavian ancestry.

Meshwork pigmentation in exfoliation glaucoma is not as intense as in pigmentary glaucoma. Iris transillumination characteristically begins at the pupillary border and not the midperiphery. Unlike pigment dispersion syndrome, approximately 50% of patients with exfoliation syndrome are affected clinically in only one eye. Finally, the presence of white flakes of exfoliation material at the pupillary border and on the anterior lens surface is diagnostic of exfoliation syndrome.

Other factors to consider include the following:

  • Pigmentation of trabecular network
    • Elderly individuals (inferior nasal or faint band circumstantial)
    • Pseudoexfoliation of lens with or without glaucoma (unilateral or bilateral)
    • Pigmentary glaucoma
    • Krukenberg spindle without glaucoma
    • Malignant melanoma (1 eye)
    • Cyst of pigment layer or iris (unilateral)
    • Previous intraocular operation, inflammation, or hyphema (scattered, mostly in lower angle)
    • Nevus (dense, isolated patch)
    • Open-angle glaucoma (patchy band, whole circumference)
    • Following glaucoma irradiation for malignancy of nasal sinus
  • Pigment liberation into anterior chamber with dilation of pupil
    • Diabetes mellitus (Willis disease)
    • Exercise
    • Hurler disease (mucopolysaccharidoses type IH)
    • Low-tension glaucoma with pigment dispersion
  • Retrocorneal pigmentation
    • Endothelial phagocytosis of free melanin pigment as Krukenberg spindle
    • Iris melanocytes, iris pigment epithelial cells, or pigment-containing macrophages in the posterior corneal surface can follow operative or accidental ocular trauma
    • Status posthyphema

Differential Diagnoses

Contributor Information and Disclosures

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: Israeli Medical Association

Disclosure: Nothing to disclose.


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, New York Academy of Sciences

Disclosure: Received none from Sensimed for board membership; Received none from iSonic Medical for board membership; Received consulting fee from Aeon Astron for consulting; Received honoraria from Pfizer for speaking and teaching; Received honoraria from Allergan for speaking and teaching; Received honoraria from Ministry of Health of Kuwait for speaking and teaching; Received honoraria from Aeon Astron for speaking and teaching; Received royalty from Ocular Instruments for other.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department 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, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

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

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

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, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Andrew I Rabinowitz, MD Director of Glaucoma Service, Barnet Dulaney Perkins Eye Center

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

Disclosure: Nothing to disclose.

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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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