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Purpose: To present the longitudinal, multimodal imaging of Bilateral Diffuse Uveal Melanocytic Proliferation secondary to gallbladder carcinoma over a 17 month period, demonstrating the natural history, the evolution with treatment and salient features to support timely diagnosis of this condition with life-threatening associations. Observations: A systemically well 73 year old woman presented with a 2 month history of progressive visual loss in the right eye. We report the initial findings on clinical examination and with retinal imaging including fluorescein and indocyanine angiography, optical coherence tomography and autoflourescence. An initial diagnosis of atypical central serous chorioretinopathy with secondary choroidal neovascularisation led to treatment with intravitreal aflibercept before the correct diagnosis of BDUMP was made 2 months later, aided by evolution of signs on imaging and examination. Subsequent investigation led to detection of gallbladder adenocarcinoma. The patient underwent systemic chemotherapy and eventual phacoemulsification and insertion of intraocular lens to both eyes. The patient died 21 months after presentation of visual symptoms, with latest imaging at 17 months. Conclusion: We report the evolution of BDUMP utilising multi-modal imaging pre-treatment and during treatment with chemotherapy, and highlight peripheral progression of disease despite consolidation at the macula.

Purpose: To present the longitudinal, multimodal imaging of Bilateral Diffuse Uveal Melanocytic Proliferation secondary to gallbladder carcinoma over a 17 month period, demonstrating the natural history, the evolution with treatment and salient features to support timely diagnosis of this condition with life-threatening associations., Observations: A systemically well 73 year old woman presented with a 2 month history of progressive visual loss in the right eye. We report the initial findings on clinical examination and with retinal imaging including fluorescein and indocyanine angiography, optical coherence tomography and autoflourescence. An initial diagnosis of atypical central serous chorioretinopathy with secondary choroidal neovascularisation led to treatment with intravitreal aflibercept before the correct diagnosis of BDUMP was made 2 months later, aided by evolution of signs on imaging and examination. Subsequent investigation led to detection of gallbladder adenocarcinoma. The patient underwent systemic chemotherapy and eventual phacoemulsification and insertion of intraocular lens to both eyes. The patient died 21 months after presentation of visual symptoms, with latest imaging at 17 months., Conclusion: We report the evolution of BDUMP utilising multi-modal imaging pre-treatment and during treatment with chemotherapy, and highlight peripheral progression of disease despite consolidation at the macula., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors.)

Ocular-involving paraneoplastic syndromes present a wide variety of clinical symptoms. Understanding the background pathophysiological and immunopathological factors can help make a more refined differential diagnosis consistent with the signs and symptoms presented by patients. There are two main pathophysiology arms: (1) autoimmune pathomechanism, which is presented with cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), cancer-associated cone dysfunction (CACD), paraneoplastic vitelliform maculopathy (PVM), and paraneoplastic optic neuritis (PON), and (2) ectopic peptides, which is often caused by tumor-expressed growth factors (T-exGF) and presented with bilateral diffuse uveal melanocytic proliferation (BDUMP). Meticulous systematic analysis of patient symptoms is a critical diagnostic step, complemented by multimodal imaging, which includes fundus photography, optical coherent tomography, fundus autofluorescence, fundus fluorescein angiography, electrophysiological examination, and sometimes fundus indocyjanin green angiography if prescribed by the clinician. Assessment of the presence of circulating antibodies is required for diagnosis. Antiretinal autoantibodies are highly associated with visual paraneoplastic syndromes and may guide diagnosis by classifying clinical manifestations in addition to monitoring treatment. [ABSTRACT FROM AUTHOR]