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.
Ilginis T, Keane PA, Tufail A. Paracentral Acute Middle Maculopathy in Sickle Cell Disease. JAMA Ophthalmol. 2015;133(5):614-616. doi:10.1001/jamaophthalmol.2014.6098