title: The pearl necklace sign: a novel spectral domain optical coherence tomography finding in exudative macular disease.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: *Retinal Disorders and Ophthalmic Genetics Division, Jules Stein Eye Institute, University of California Los Angeles, Los Angeles, California; †Greater Los Angeles VA Healthcare Center, Los Angeles, California; and ‡Vitreous Retina Macula Consultants of New York, New York, New York.
authors.forename: Susan K
authors.initials: SK
authors.lastname: Gelman
authors.forename: K Bailey
authors.initials: KB
authors.lastname: Freund
authors.forename: Vinnie P
authors.initials: VP
authors.lastname: Shah
authors.forename: David
authors.initials: D
authors.lastname: Sarraf
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D000068258:Bevacizumab; D002908:Chronic Disease; D005122:Exudates and Transudates; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D058449:Intravitreal Injections; D008268:Macular Degeneration / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D008269:Macular Edema / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D012160:Retina / Q000473:pathology*; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence / Q000379:methods*; D042461:Vascular Endothelial Growth Factor A / Q000037:antagonists & inhibitors; D014792:Visual Acuity / Q000502:physiology
chemical_list: D020533:Angiogenesis Inhibitors; D061067:Antibodies, Monoclonal, Humanized; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D000068258:Bevacizumab