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Brief Report |

Bilateral Diffuse Uveal Melanocytic Proliferation With Multiple Iris Cysts

Anthony Joseph, MD1; Ehsan Rahimy, MD2; David Sarraf, MD1,3
[+] Author Affiliations
1Retinal Disorders and Ophthalmic Genetics Division, Jules Stein Eye Institute, University of California, Los Angeles
2Retina Service, Wills Eye Institute, Philadelphia, Pennsylvania
3Department of Surgery, Division of Ophthalmology, Greater Los Angeles Veterans Affairs Healthcare Center, Los Angeles, California
JAMA Ophthalmol. 2014;132(6):756-760. doi:10.1001/jamaophthalmol.2014.311.
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Importance  Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic syndrome with characteristic findings, including exudative retinal detachment, rapid cataract formation, and uveal melanocytic tumors. We report a case notable for bilateral iris and ciliary body cysts—a rare presentation of the disease.

Observations  A woman in her 50s presented with bilateral decreased vision. Her medical history was significant for clear cell adenocarcinoma of the endometrium. Slitlamp examination revealed a contiguous ring of pigmented translucent iris cysts at the pupillary margin of each eye, confirmed with ultrasound biomicroscopy. Ophthalmoscopic examination of the left eye showed a geographic patch of subretinal fluid temporal to the macula that was associated with orange polygonal pigment. The patient underwent periocular injection of triamcinolone acetonide, with resolution of the subretinal fluid. Recurrent fluid was treated successfully with a second injection of triamcinolone.

Conclusions and Relevance  Our case of BDUMP appears to be the first to demonstrate multiple iris and ciliary body cysts with high-quality color photography and ultrasound biomicroscopy. Involvement of the anterior uveal tract may be more common than reported in the literature because of its occult nature. Ultrasound biomicroscopy and anterior segment optical coherence tomography may be useful in patients with suspected BDUMP to identify anterior uveal tract involvement.

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Figure 1.
Iris and Ciliary Body Cysts

A, Retroillumination of the right eye showing a contiguous ring of translucent iris cysts at the pupillary margin. B, Retroillumination of the left eye showing translucent pigmented cysts at the pupillary margin. Note the pigment granules within the cysts. Also note the anterior subcapsular cataract in transillumination. C and D, Ultrasound biomicroscopy of the right eye and left eye, respectively, demonstrating diffuse ciliary body cysts posterior to the iris, narrowing of the anterior chamber angle (asterisk), and diffuse thickening of the ciliary body (dashed arrow). Note the cysts coming into contact with the anterior surface of the lens in the right eye (arrowhead).

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Figure 2.
Multimodal Imaging of the Retina

A, Color fundus montage of the left eye showing a geographic patch of subretinal fluid temporal to the macula associated with orange polygonal pigment. B, Color fundus photograph of the left eye centered on this lesion. C, Fluorescein angiography of the left eye demonstrating blocking defects corresponding to the orange polygonal lesions and hyperfluorescent window defects corresponding to foci of presumed retinal pigment epithelium (RPE) atrophy. D, 30° fundus autofluorescence (Heidelberg Retina Angiograph; Heidelberg Engineering) demonstrating intensified levels of autofluorescence corresponding to the orange polygonal lesions and complete loss of the expected RPE autofluorescence pattern corresponding to foci of presumed RPE atrophy. E, Spectral-domain optical coherence tomography (OCT) through the temporal lesion in the left eye revealing subretinal fluid and an irregular and thickened RPE. The green line in the near infrared image is registered with the adjacent OCT.

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Figure 3.
Relationship of Subretinal Fluid to Periocular Corticosteroid Injections

A, Enhanced depth imaging optical coherence tomography (EDI-OCT) of the left macula on presentation. The previously noted area of subretinal fluid and irregularly thickened retinal pigment epithelium (Figure 2E) are seen temporally. B, EDI-OCT of the left macula 2 weeks after injection of periocular triamcinolone acetonide, 40 mg. The temporal subretinal fluid has almost completely resolved. C, EDI-OCT of the left macula 5 months after the initial injection of periocular triamcinolone shows a large neurosensory detachment. D, EDI-OCT of the left macula 2 weeks after a second injection of periocular triamcinolone acetonide, 40 mg, showing a decrease in subretinal fluid. E, EDI-OCT of the left macula 1 month after the second injection of periocular triamcinolone shows further reduction in subretinal fluid, especially temporally. The thickened choroid at each follow-up remains grossly unchanged with injection (about 500 μm). The green line in the near infrared image is registered with the adjacent OCT.

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