Serous Macular Detachment in Waldenstrom Macroglobulinemia: A Report of Four Cases

PURPOSE: To describe a series of 4 patients with Waldenstrom macroglobulinemia and serous macular detachment, and propose a mechanism for development of subretinal fluid based on optical coherence tomography (OCT) findings. DESIGN: Retrospective observational case series. METHODS: The records of patients with Waldenstrom macroglobulinemia and OCT documentation of serous macular detachment at Wills Eye Institute were reviewed. Data collection included clinical examination, as well as findings on fluorescein angiography (FA) and OCT. RESULTS: Four patients (8 eyes) with Waldenstrom macroglobulinemia and serous retinal detachment were identified. All eyes had varying degrees of venous stasis retinopathy and intraretinal edema overlying the macular detachment. Three patients had no FA leakage, while 1 patient had macular leakage in a petaloid pattern. Focal outer retinal defects within the detached retina were seen in 4 eyes on OCT imaging. In one eye, development of cystoid macular edema was observed before the outer retinal defect and serous macular detachment. All patients with serous macular detachment had some degree of outer retinal disruption. CONCLUSION: Discontinuity of the outer retina within the macular detachment may enable immunoglobulins along with accumulated intraretinal fluid to flow into the subretinal space, creating a serous retinal detachment. Even with systemic treatment of the underlying Waldenstrom macroglobulinemia, the visual prognosis was guarded. (Am J Ophthalmol 2013;155:448-455. (C) 2013 by Elsevier Inc. All rights reserved.)