Immunogammopathies and Acquired Vitelliform Detachments: A Report of Four Cases – Accepted Manuscript

Abstract: Purpose: To describe the nature and evolution of acquired macular detachments in patients with immunogammopathies and to propose a mechanism for their development.Design: Retrospective observational case series.Methods: Three patients with multiple myeloma and one with light chain deposition disease were diagnosed with vitelliform macular detachments based on clinical exam, fundus autofluorescence, fluorescein angiography, and optical coherence tomography. These patients were followed over time and their clinical exams and imaging studies were compared and contrasted.Results: Three patients (5 eyes) with multiple myeloma and 1 patient (2 eyes) with light chain deposition disease presented with acquired macular yellowish subretinal deposits on funduscopic exam, that corresponded to hyperautofluorescent lesions on fundus autofluorescence imaging and subretinal hyperreflective material on spectral-domain optical coherence tomography. Two patients (4 eyes) had diffuse serous retinal detachments involving not only the macular region but also the mid-periphery of the retina. These acquired macular vitelliform detachments were not associated with signs of hyperviscosity retinopathy in 5 eyes and resolved after successful treatment of the multiple myeloma in 4 eyes.Conclusion: Patients with an immunogammopathy such as multiple myeloma or light chain deposition disease may develop serous elevations of the macula that we classify as acquired vitelliform detachments using multimodal imaging. Appropriate work up including serum protein electrophoresis and hematology consultation should be considered in the management of patients with acquired vitelliform detachments of uncertain etiology.