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Paracentral Acute Middle Maculopathy in Sickle Cell Disease

Tomas Ilginis, MBBS1,2; Pearse A. Keane, MD, FRCOphth3,4; Adnan Tufail, MD, FRCOphth3,4
[+] 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
JAMA Ophthalmol. 2015;133(5):614-616. doi:10.1001/jamaophthalmol.2014.6098.
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Extract

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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Figure 1.
Fundus Photograph at Presentation and Corresponding Optical Coherence Tomographic (OCT) Images

Patient’s fundus examination findings. Retinal atrophy is noted with an asterisk. The OCT images correspond to lines 1, 2, and 3 (top scans are at presentation; bottom, outcome).

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Figure 2.
Optical Coherence Tomographic (OCT) Collage Image

The patient’s foveal scan at outcome vs the scan of a healthy age-matched person. The red dashed line outlines the region of main difference between the scans, demonstrating atrophy of the middle retinal layers.

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