Bilateral diffuse uveal melanocytic proliferation: Report of a novel optical coherence tomography finding and clinical response to plasmapheresis

Abstract

Purpose: To describe a novel optical coherence tomography (OCT) finding in a case of bilateral diffuse uveal melanocytic proliferation (BDUMP) and to report the clinical response to plasmapheresis. Observations: We report the case of a 54-year-old man who was being treated with adjuvant immune checkpoint inhibitors for metastatic renal cell carcinoma. He had suffered from bilateral progressive vision loss without ocular pain. At presentation in the retina clinic, visual acuity was counting fingers bilaterally. Examination revealed characteristic findings suggestive of a peculiar paraneoplastic intraocular syndrome called BDUMP. Multiple choroidal nevi-like melanocytic tumors were noted bilaterally. The diagnosis was confirmed using multimodal imaging with fluorescein angiography and fundus autofluorescence, which revealed a typical leopard pattern. Ultrasonography revealed choroidal thickening extending to the ciliary body. OCT showed multiple pockets of serous retinal detachment (SRD) and bacillary layer detachment (BALAD), a newly recognized and rarely described manifestation of the disease. The clinical response to plasmapheresis was robust with resolution of the BALAD and SRD and improvement of the vision to 20/30 in both eyes at the seven-month follow-up. Conclusions and importance: BALAD is a newly recognized manifestation of BDUMP. Early recognition of this paraneoplastic syndrome and prompt initiation of plasmapheresis has the potential to improve and stabilize vision.

Keywords

• Bilateral diffuse uveal melanocytic proliferation
• Bacillary layer detachment
• Renal cell carcinoma
• Immunotherapy