THE PEARL NECKLACE SIGN A Novel Spectral Domain Optical Coherence Tomography Finding in Exudative Macular Disease

Purpose:To report a novel spectral domain optical coherence tomography finding in exudative macular disease, called the pearl necklace sign.Methods:A retrospective case series of 21 eyes (20 patients) with chronic exudative maculopathy resulting from age-related macular degeneration, diabetic macular edema, branch retinal vein occlusion, retinal arterial macroaneurysm, and Coats disease. Spectral domain optical coherence tomography images were carefully evaluated and correlated with color fundus photography, near-infrared reflectance, and fluorescein angiography.Results:A unique spectral domain optical coherence tomography macular finding of hyperreflective dots in a contiguous ring around the inner wall of cystoid spaces in the outer plexiform layer of the retina that the authors refer to as the pearl necklace sign was seen in all patients. Visual acuity ranged from 20/30 to hand motions. The cystoid spaces and the hyperreflective dots resolved in certain cases after anti-vascular endothelial growth factor therapy and/or focal macular laser, but tended to recur.Conclusion:Because the pearl necklace configuration can be found adjacent to hard exudates in the outer plexiform layer, the authors speculate that the hyperreflective material is composed of lipoproteins or lipid-laden macrophages. This novel spectral domain optical coherence tomography sign gives further insight into the development and progression of hard lipoprotein exudates in exudative maculopathy.