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Int J Retina Vitreous. 2016 May 16;2:13. eCollection 2016.

Multimodal imaging of bilateral diffuse uveal melanocytic proliferation associated with an iris mass lesion.

Author information

1
The Vitreous Retina Macula Consultants of New York, 460 Park Avenue, Fifth Floor, New York, NY 10022 USA ; LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York, NY USA ; Department of Ophthalmology, New York University School of Medicine, New York, NY USA ; Department of Ophthalmology, North Shore - Long Island Jewish Health System, New York, NY USA.
2
The Vitreous Retina Macula Consultants of New York, 460 Park Avenue, Fifth Floor, New York, NY 10022 USA ; LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York, NY USA.
3
The Vitreous Retina Macula Consultants of New York, 460 Park Avenue, Fifth Floor, New York, NY 10022 USA ; LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York, NY USA ; Department of Ophthalmology, New York University School of Medicine, New York, NY USA.

Abstract

BACKGROUND:

Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare, paraneoplastic syndrome characterized by bilateral painless visual loss and proliferation of choroidal melanocytes in association with an underlying systemic malignancy. We report a case of bilateral diffuse uveal melanocytic proliferation associated with an underlying gynecological malignancy that also features the infrequent finding of an iris mass lesion, using multimodal imaging including ultra-widefield imaging, spectral domain and swept-source optical coherence tomography.

CASE PRESENTATION:

A 59-year-old white female with a prior history of gynecological malignancy in remission presented with progressive bilateral visual loss over several weeks. The patient was noted to have a focal iris mass lesion in her right eye. Ultra-widefield color fundus photography showed a characteristic bilateral 'giraffe pattern' of pigmentary changes extending into the periphery as well as multiple discrete deeply pigmented lesions. Ultra-widefield autofluorescence was useful for visualizing the full extent of involvement. Indocyanine green angiography helped to demarcate the discrete pigmented choroidal lesions. Swept-source OCT clearly delineated the alternating zones of retinal pigment epithelium (RPE) thickening and RPE loss, as well as the prominent choroidal infiltration and thickening.

CONCLUSIONS:

BDUMP is an important diagnosis to consider in the presence of multiple discrete melanocytic choroidal lesions, diffuse choroidal thickening, characteristic RPE changes, iris mass lesions and exudative retinal detachment. Ultra-widefield imaging may demonstrate more extensive lesions than that detected on clinical examination or standard field imaging. Imaging with SS-OCT shows choroidal and RPE characteristics that correlate well with known histopathology of this entity.

KEYWORDS:

Bilateral diffuse uveal melanocytic proliferation; Iris tumor; Malignancy; Melanocytes; Swept-source optical coherence tomography; Ultra-widefield

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