Immunogammopathy Maculopathy and Macular Schisis with En Face OCT

PURPOSE: To describe the multimodal imaging (MMI) findings, including en face optical coherence tomography (OCT), of a case of Waldenström-associated immunogammopathy maculopathy. METHODS: Medical and imaging records were retrospectively evaluated. MMI included ultra-widefield (UWF) color fundus photography (CFP), fundus autofluorescence (FAF) and fluorescein angiography (FA). Cross-sectional and en face OCT were also studied. RESULTS: A 68-year-old man with type 2 diabetes mellitus and anemia was referred for evaluation of macular edema. UWF CFP and FAF showed scattered intraretinal hemorrhages and central SRF in both eyes. UWF FA showed patchy peripheral retinal ischemia but failed to illustrate macular leakage consistent with a silent FA. Cross-sectional OCT illustrated diffuse macular schisis OD and macular detachment OS. En face OCT illustrated a remarkable stellate pattern of schisis in the macula OU and also captured multiple clusters of hyperreflective subretinal deposits, possibly due to macroglobulin accumulation. Serum plasma electrophoresis detected an abnormally elevated monoclonal immunoglobulin M (IgM) spike (4055 mg/dL), and bone marrow biopsy confirmed a systemic diagnosis of Waldenström macroglobulinemia. Anti-VEGF therapy was deferred until monoclonal reduction with systemic chemotherapy. CONCLUSION: Immunogammopathy maculopathy and retinopathy can masquerade as macular edema from retinovascular disease and should be suspected with macular detachment and severe central SRF without leakage on FA ("silent FA"). The recognition of these findings with MMI may be critical for the diagnosis and prompt treatment of this systemic disease. En face OCT captured hyperreflective deposits along the outer retinal wall which may represent monoclonal antibodies, known to draw fluid into the subretinal space.