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

Jonathan Naysan,Robert W Klein,Claudine E Pang,K Bailey Freund

doi:10.1186/s40942-016-0038-7

Abstract

BackgroundBilateral 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 presentationA 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.ConclusionsBDUMP 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.

Highlights

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
BDUMP is an important diagnosis to consider in the presence of multiple discrete melanocytic choroidal lesions, diffuse choroidal thickening, characteristic retinal pigment epithelium (RPE) changes, iris mass lesions and exudative retinal detachment
Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare, paraneoplastic syndrome characterized by bilateral painless, profound visual loss in association with an underlying systemic malignancy [1]

Summary

Conclusions

We present a case of BDUMP using multimodal imaging including the use of ultra-widefield imaging, ICG angiography and SS-OCT that helped to show the full extent of the posterior segment disease. [3, 20, 34,35,36,37] Originally described by Gass et al [4] iris involvement is infrequently seen or documented We highlight this uncommon occurrence to create awareness that BDUMP can lead to infiltration of the uveal tract including the iris and ciliary body, which may further lead to angle-closure glaucoma and cataracts. [1, 11] The rationale is to decrease the circulating autoantibodies, [1, 11, 24] shown to be in the IgG fragment termed cultured melanocyte elongation and proliferation (CMEP) factor, [38] which stimulates cutaneous and uveal melanocyte cell proliferation Success with this treatment has been limited.

Background