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Observation
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
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.

Report of a Case

A young black woman with sickle cell disease (HbSS variant) presented with a 2-day history of visual loss in the left eye. No dehydration or other precipitating factors were noted. Visual acuity was 20/20 OD and 20/800 OS. Results of the examination of her asymptomatic right eye were unremarkable with the exception of peripheral retinal black sunburst lesions. In her left eye, ischemic macular whitening was noted (Figure 1). No cherry-red spot, hemorrhage, or peripheral neovascularization was present. Findings of fluorescein angiography of the left eye showed normal cilioretinal, retinal arteriolar, and retinal venous filling; however, the retinal capillary bed was abnormal across the macula, with increased intercapillary spacing and small areas of hypofluorescence nasal to the fovea (eFigure in the Supplement). On OCT of the left eye, hyperreflective plaques at the level of the INL/OPL were present, corresponding to the ophthalmoscopically opaque areas. Optical coherence tomography of the right eye showed temporal macular thinning.

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

Seven months later, visual acuity in the left eye remained poor (3/200). The left macula showed a distorted reflex, and OCT revealed atrophic changes—predominantly of the INL/OPL—in the areas corresponding to previous swelling (Figure 2). Results of fluorescein angiography did not demonstrate any leakage, nonperfusion, or new vessel formation.

Figure 2.  Optical Coherence Tomographic (OCT) Collage Image
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.

Discussion

The loss of vision in this case was considered to be caused by an ischemic event. However, no evidence of persistent central retinal or cilioretinal artery occlusion was seen on fluorescein angiographic findings 2 days after the acute event. No pathognomonic cherry-red spot or signs of choroidal ischemia were detected. Despite these findings, the patient’s vision did not improve, and atrophy of the INL/OPL subsequently developed, indicating that the ischemic event was of sufficient duration to cause permanent retinal cell damage. In addition, the distribution of retinal swelling did not anatomically correspond to the area supplied by cilioretinal arteries and was too selectively localized in the middle retina to be caused by central retinal artery occlusion. No signs consistent with partial central retinal artery occlusion were seen (eg, peripapillary cotton-wool spots, venous cyanosis, or periarterial sparing).3 The absence of multiple cotton-wool spots in our case may be explained by preserved superficial capillary plexus perfusion with no interruption to axoplasmic transport in the retinal nerve fiber layer. Thus, we hypothesize that ischemia occurred selectively within the deep retinal capillary plexus. In animal models, the OPL has been proved to be a watershed zone between retinal and choroidal circulation while being characterized by very high oxygen consumption.4 A transient arterial vasospasm or hypoperfusion of another cause, severe enough to cause infarction of the middle retina directly or secondarily by inducing acidosis and intravascular sickling of erythrocytes, might have resulted in occlusion of the deep capillary circulation and the signs of paracentral acute middle maculopathy on OCT imaging.

Silent and acute macular thinning and splaying in sickle cell disease have been well described previously; however, they have not been attributed to selective occlusion of the deep capillary plexus.5,6 Optical coherence tomographic imaging may provide new insights into the mechanisms of visual loss in sickle cell disease, particularly in the acute phases.

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Article Information

Corresponding Author: Tomas Ilginis, MBBS, Department of Ophthalmology, Glostrup Hospital, Nordre Ringvej 57, 2600 Glostrup, Denmark.

Published Online: February 19, 2015. doi:10.1001/jamaophthalmol.2014.6098.

Conflict of Interest Disclosures: Dr Ilginis has served on the advisory board of Bayer. Dr Keane has received travel grants from the Allergan European Retina Panel. Dr Tufail has served on the advisory boards of Allergan, Bayer, GSK, Heidelberg Engineering, Novartis, Pfizer, and Thrombogenics. No other disclosures were reported.

Funding/Support: Drs Keane and Tufail have received a proportion of their funding from the Department of Health’s National Institute for Health Research, Biomedical Research Centre at Moorfields Eye Hospital NHS Foundation Trust and the Institute of Ophthalmology, University College London.

Role of the Funder/Sponsor: The funding sources had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Disclaimer: The views expressed in the publication are those of the authors and do not necessarily represent those of the Department of Health.

References
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Hoang  QV, Chau  FY, Shahidi  M, Lim  JI.  Central macular splaying and outer retinal thinning in asymptomatic sickle cell patients by spectral-domain optical coherence tomography.  Am J Ophthalmol. 2011;151(6):990-994.e1. doi:10.1016/j.ajo.2010.12.010.PubMedGoogle ScholarCrossref
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