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Pearl necklace sign not an uncommon OCT manifestation of macular edema
Hyperreflective white dots lining cystoid spaces are thought to be lipid-laden macrophages.
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A contiguous ring of small hyperreflective dots lining the inner wall of cystoid spaces in the outer plexiform layer as seen on spectral-domain optical coherence tomography is thought to be a representation of lipid-laden macrophages, according to a study.
“Patients with macular edema due to macular degeneration or various retinal vasculopathies such as diabetic retinopathy can develop hyperreflective dots that line cystoid spaces, and they may be a sign of chronic exudative macular disease,” study co-author David Sarraf, MD, said, adding that the dots have a characteristic appearance similar to a pearl necklace.
In the study published in Retina, SD-OCT was used to examine patients with chronic exudative maculopathy and chronic macular edema due to various retinal and choroidal disorders.
Methods
The multicenter study included 21 eyes of 20 patients who displayed the pearl necklace sign. SD-OCT imaging was used in conjunction with color fundus photography, near-infrared reflectance and fluorescein angiography in all patients. Mean follow-up time was 11 months.
Researchers reviewed clinical charts and recorded patient characteristics retrospectively to compare baseline characteristics with SD-OCT findings.
“I think more recent research has determined that these hyperreflective white dots that line the cystic cavities may be lipid-laden macrophages, and we are awaiting histopathological confirmation of that correlation,” Sarraf said.
Novel SD-OCT finding
Eleven patients had disciform age-related macular degeneration with an associated fibrotic submacular scar, five had chronic diabetic macular edema, two had chronic macular edema secondary to branch retinal vein occlusion, one had edema with exudation secondary to a retinal arterial macroaneurysm, and one had Coats’ disease. Visual acuity ranged from 20/30 to hand motions.
“This novel signature SD-OCT sign gives further insight into the development and progression of lipoprotein exudates in exudative maculopathy,” Sarraf and colleagues said in the study.
Seven patients were given anti-VEGF therapy after discovery of the pearl necklace sign. The other patients were not responsive to treatment or had poor visual prognosis. For some patients who received anti-VEGF treatment, the pearl necklace sign resolved but tended to recur.
“I do not think that these findings change treatment necessarily,” Sarraf said. “They are a sign of chronic exudative macular disease and may be a sign of a particularly exuberant form of macular edema where you can get severe lipid exudation and precipitation that may or may not be recalcitrant to anti-VEGF and focal laser therapy.”
Researchers noted associated hard lipoprotein exudates on funduscopic examination in 15 eyes and adjacent to the pearl necklace lesions seen on SD-OCT in eight eyes. – by Nhu Te
Reference:
Gelman SK, et al. Retina. 2014;doi:10.1097/IAE.0000000000000207.For more information:
David Sarraf, MD, can be reached at Retinal Disorders and Ophthalmic Genetics Division, Jules Stein Eye Institute, 100 Stein Plaza, Los Angeles, CA 90095; email: dsarraf@ucla.edu.Disclosure: Sarraf has received an IST grant from Regeneron Pharmaceuticals and an Advisory Board grant from Genentech.
Perspective
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Andrew P. Schachat, MD
Gelman, Freund, Shah and Sarraf report a spectral-domain optical coherence tomography finding in 20 patients with various chronic exudative maculopathies and suggest the finding relates to lipoprotein deposition or lipid-laden macrophages. I agree with the authors that the finding likely relates to chronicity. If so, grading of data sets for the presence or absence of this finding at baseline might confirm if the presence of this sign is a marker for poor treatment outcomes. Precipitates do form in cysts and can be seen on SD-OCT images, so a crisp definition of the sign will be needed to allow reliable grading. I agree with the authors that the finding is distinct from outer retinal tubulation (ORT). ORTs are not to be confused with cystoid edema, but the pearl necklace sign finding does appear to be part of a cystoid-leaking maculopathy.
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