eMedicine Specialties > Ophthalmology > Intraocular Pressure

Glaucoma, Pigmentary: Differential Diagnoses & Workup

Author: Robert Ritch, MD, Chief of Glaucoma Service, Surgeon Director, Professor, Department of Ophthalmology, New York Eye and Ear Infirmary
Coauthor(s): Yaniv Barkana, MD, Consulting Staff, Glaucoma Unit, Department of Ophthalmology, Assaf Harofe Medical Center
Contributor Information and Disclosures

Updated: Nov 6, 2007

Differential Diagnoses

Glaucoma, Angle Recession
Glaucoma, Aphakic And Pseudophakic
Glaucoma, Drug-Induced
Glaucoma, Juvenile
Glaucoma, Plateau Iris
Glaucoma, Primary Open Angle

Other Problems to Be Considered

PDS usually can be distinguished easily 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 exists. 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.

Recently, an increase of PDS and 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.5 Phakic intraocular lenses can also result in PDS and PG. 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.

The disease process most similar to PG is exfoliation glaucoma. In this condition, a loss of pigment occurs from the IPE, iris transillumination, pigment dispersion in the anterior segment, including Krukenberg spindle, trabecular pigmentation, and 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 PG. Iris transillumination characteristically begins at the pupillary border and not the midperiphery. Unlike PDS, approximately 50% of patients with exfoliation syndrome are affected clinically in only 1 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.

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 post hyphema

Workup

Imaging Studies

  • Ultrasound biomicroscopy (UBM) has been particularly useful in evaluating the structures surrounding the posterior chamber. UBM shows the posterior iris insertion6 , iris concavity, iridozonular contact, and extensive iridolenticular contact.

More on Glaucoma, Pigmentary

Overview: Glaucoma, Pigmentary
Differential Diagnoses & Workup: Glaucoma, Pigmentary
Treatment & Medication: Glaucoma, Pigmentary
Follow-up: Glaucoma, Pigmentary
References
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References

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

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

  4. 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.

  5. 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].

  6. 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].

  7. 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].

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

  13. 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].

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

  16. 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].

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

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Further Reading

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Keywords

pigmentary glaucoma, pigment dispersion syndrome, PDS, pigment granule accumulation, progressive trabecular dysfunction, ocular hypertension, glaucomatous optic neuropathy, PG, POAG, open angle, open-angle glaucoma, myopia

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Contributor Information and Disclosures

Author

Robert Ritch, MD, Chief of Glaucoma Service, Surgeon Director, Professor, Department of Ophthalmology, New York Eye and Ear Infirmary
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.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

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.

CME Editor

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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