Pigmentary Glaucoma Differential Diagnoses

Updated: Oct 23, 2018
  • Author: Jim C Wang (王崇安), MD; Chief Editor: Hampton Roy, Sr, MD  more...
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DDx

Diagnostic Considerations

Primary pigment dispersion syndrome (PDS) and pigmentary glaucoma (PG) are easily distinguished from other pigment-disseminating diseases because of their unique pattern of mid-peripheral iris transillumination defects. This characteristic pattern of pigment dispersion and pigmentary glaucoma may also be seen secondary to iris chafing by intraocular lenses implanted in the ciliary sulcus. [12] Phakic intraocular lenses can also result in PDS and pigmentary glaucoma. In these conditions, trabecular pigmentation is often less dense and is usually unevenly distributed throughout the circumference of the meshwork.  

The disease process most similar to PDS and pigmentary glaucoma is pseudoexfoliation syndrome and its associated glaucoma. 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. In pseudoexfoliation, similar pigment accumulation in the anterior chamber, including Krukenberg spindle, may be seen. However, the trabecular meshwork pigmentation in exfoliation glaucoma is not as intense as in pigmentary glaucoma. Iris transillumination characteristically begins at the pupillary border and not in the midperiphery. Unlike PDS, 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 disorders that are associated with pigment release, such as iris or ciliary body cysts and ocular melanoma, often occur unilaterally. Occasionally, pigment granules in the anterior chamber may be confused with inflammatory cells, leading to a misdiagnosis of uveitis. [13]

Differential Diagnoses