Pigmentary Glaucoma Differential Diagnoses
- Author: Yaniv Barkana, MD; Chief Editor: Hampton Roy, Sr, MD more...
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. 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.
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)
- Hurler disease (mucopolysaccharidoses type IH)
- Low-tension glaucoma with pigment dispersion
- 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
Sugar HS, Barbour FA. Pigmentary glaucoma: a rare clinical entity. Am J Ophthalmol. 1949. 32:90.
Flügel-Koch CM, Tektas OY, Kaufman PL, Paulsen FP, Lütjen-Drecoll E. Morphological alterations within the peripheral fixation of the iris dilator muscle in eyes with pigmentary glaucoma. Invest Ophthalmol Vis Sci. 2014 Jun 17. 55(7):4541-51. [Medline].
Klingenstein A, Kernt M, Seidensticker F, Kampik A, Hirneiss C. Anterior-segment morphology and corneal biomechanical characteristics in pigmentary glaucoma. Clin Ophthalmol. 2014. 8:119-26. [Medline]. [Full Text].
Maidana DE, Sanz S, Lillo J, Arruga J. Serous macular detachment associated with optic nerve head cupping in pigmentary glaucoma. Can J Ophthalmol. 2014 Apr. 49(2):e56-9. [Medline].
[Guideline] U.S. Preventive Services Task Force (USPSTF). Screening for glaucoma: recommendation statement. Rockville (MD): Agency for Healthcare Research and Quality (AHRQ); 2005 Mar.
Campbell DG. Pigmentary dispersion and glaucoma. A new theory. Arch Ophthalmol. 1979 Sep. 97(9):1667-72. [Medline].
Lichter PR, Shaffer RN. Diagnostic and prognostic signs in pigmentary glaucoma. Trans Am Acad Ophthalmol Otolaryngol. 1970 Sep-Oct. 74(5):984-98. [Medline].
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.
Uy HS, Chan PS. Pigment release and secondary glaucoma after implantation of single-piece acrylic intraocular lenses in the ciliary sulcus. Am J Ophthalmol. 2006 Aug. 142(2):330-2. [Medline].
Gonzalez-Gonzalez LA, Rodríguez-García A, Foster CS. Pigment dispersion syndrome masquerading as acute anterior uveitis. Ocul Immunol Inflamm. 2011 Jun. 19(3):158-66. [Medline].
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. 2009 Oct 12. [Medline].
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. 2006 Nov. 124(11):1573-6. [Medline].
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. 2010 Jan 13. [Medline].
Aptel F, Beccat S, Fortoul V, Denis P. Biometric analysis of pigment dispersion syndrome using anterior segment optical coherence tomography. Ophthalmology. 2011 Aug. 118(8):1563-70. [Medline].
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. 2005 Jun. 139(6):1110-3. [Medline].
Cantor L. Section 10: Glaucoma. Basic and Clinical Science Course. American Academy of Ophthalmology: 1996-1997.
Chaudry I, Wong S. Recognizing glaucoma. A Guide for the Primary Care Physician. 1999. Vol 99.: 247-64.
Gupta N, Weinreb RN. New definitions of glaucoma. Curr Opin Ophthalmol. 1997 Apr. 8(2):38-41. [Medline].
Hitchings RA. Glaucoma: current thinking. Br J Hosp Med. 1996 Mar 20-Apr 2. 55(6):312-4. [Medline].
Liesegang TJ. Glaucoma: changing concepts and future directions. Mayo Clin Proc. 1996 Jul. 71(7):689-94. [Medline].
Qureshi IA. Effects of mild, moderate and severe exercise on intraocular pressure of sedentary subjects. Ann Hum Biol. 1995 Nov-Dec. 22(6):545-53. [Medline].
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. 2005 Aug. 14(4):255-9. [Medline].
Shields MB. Textbook of Glaucoma. 4th ed. 1998.
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. 2003 Jun. 135(6):794-9. [Medline].
Van Buskirk EM. Medicolegal aspects of glaucoma care. Surv Ophthalmol. 1998 Jul-Aug. 43(1):83-6. [Medline].