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May 2015

Paracentral Acute Middle Maculopathy in Sickle Cell Disease

Author Affiliations
  • 1Medical Retina Service, Moorfields Eye Hospital NHS Foundation Trust, London, England
  • 2Department of Ophthalmology, Glostrup Hospital, Glostrup, Denmark
  • 3National Institute for Health Research, Biomedical Research Centre at Moorfields Eye Hospital NHS Foundation Trust, London, England
  • 4Institute of Ophthalmology, University College London, London, England

Copyright 2015 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.

JAMA Ophthalmol. 2015;133(5):614-616. doi:10.1001/jamaophthalmol.2014.6098

The retina receives blood from 2 sources. The outer retina is supplied by the choroid, whereas the inner retina is supplied by inner retinal capillaries in the superficial and deep plexuses. The fovea is perfused exclusively by the choroid. On optical coherence tomographic (OCT) imaging, novel reflectivity changes in the middle layers of the retina (the inner plexiform layer, inner nuclear layer [INL], and outer plexiform layer [OPL]) have been described recently.1,2 These changes have been linked to parafoveal deep retinal capillary plexus ischemia, hence termed paracentral acute middle maculopathy. In this report, we present a case of paracentral acute middle maculopathy in a patient with sickle cell disease and hypothesize regarding the role of deep retinal capillary ischemia in this disease.

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