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Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 279-280

Paracentral acute middle maculopathy in a patient with sickle cell trait

Moorfields Eye Hospital NHS Foundation Trust, London, UK

Date of Submission06-Jun-2021
Date of Acceptance19-Aug-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Peng Yong Sim
Moorfields Eye Hospital, 162 City Road, London, EC1V 2PD
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1577_21

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Keywords: Paracentral acute middle maculopathy, retinal ischemia, sickle cell trait

How to cite this article:
Sim PY, Yang E. Paracentral acute middle maculopathy in a patient with sickle cell trait. Indian J Ophthalmol Case Rep 2022;2:279-80

How to cite this URL:
Sim PY, Yang E. Paracentral acute middle maculopathy in a patient with sickle cell trait. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 14];2:279-80. Available from: https://www.ijoreports.in/text.asp?2022/2/1/279/334890

A 56-year-old African woman with sickle cell trait (HbSA) presented with a 1-week history of visual loss in her left eye. Best-corrected visual acuity was 20/40 OD and 20/400 OS. Examination of the asymptomatic right eye was unremarkable. Left fundoscopy revealed a whitish, near-oval lesion in the parafoveal region [Figure 1]a with no cherry-red spot, hemorrhage, or peripheral neovascularization. Optical coherence tomography (OCT) demonstrated hyperreflectivity of the inner retina consistent with paracentral acute middle maculopathy (PAMM; [Figure 1]b), and fundus fluorescein angiography showed an enlarged foveal avascular zone [Figure 2]. Reduced parafoveal flow with attenuation of deep capillary plexus was seen on en face OCT angiography [Figure 3]. Comprehensive infectious, inflammatory, autoimmune, and thrombophilia screen were negative. Six-month follow-up with conservative management and observation revealed partial anatomical resolution but persistence of visual deficit.
Figure 1: Left fundus photo (a) demonstrating the parafoveal area of retinal whitening. Optical coherence tomography (b), corresponding to the green line in (a), highlighting inner retinal thickening and hyperreflectivity in the area of retinal whitening, consistent with paracentral acute middle maculopathy

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Figure 2: Left fundus fluorescein angiogram demonstrating enlarged foveal avascular zone with capillary dropout suggestive of an ischemic maculopathy

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Figure 3: En face optical coherence tomography angiography (OCTA) of the deep capillary plexus (a – right; b – left) angiography demonstrating reduced parafoveal flow and attenuation in the left macula. Corresponding OCT B-scan with flow overlay (c – right; d – left) highlighting the position of the line scan for the OCTA images

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  Discussion Top

PAMM has been associated with SC disease and retinopathy previously[1],[2]; however, it has not been reported in SC trait. Although there appears to be no increased risk of retinopathy in healthy individuals with SC trait,[3] there have been documented cases of retinopathy in such patients.[4] Ischemic retinopathy in such situations can be caused by conditions of hypoxic stress or concomitant systemic diseases. Thus, we hypothesize that an ischemic event, possibly precipitated by a previously undetected stressor, might have selectively affected the parafoveal deep retinal capillary plexus causing PAMM in our patient. Nonetheless, it remains unclear whether SC trait is causative or simply coincidental in our case given the high prevalence of SC trait in the general population.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Ilginis T, Keane PA, Tufail A. Paracentral acute middle maculopathy in sickle cell disease. JAMA Ophthalmol 2015;133:614-6.  Back to cited text no. 1
Ong SS, Ahmed I, Scott AW. Association of acute macular neuroretinopathy or paracentral acute middle maculopathy with sickle cell disease. Ophthalmol Retina 2021:S2468-6530(21) 00013-0. doi: 10.1016/j.oret. 2021.01.003.  Back to cited text no. 2
Nia J, Lam W-C, Kleinman DM, Kirby M, Liu ES, Eng KT. Retinopathy in sickle cell trait: Does it exist? Can J Ophthalmol 2003;38:46-51.  Back to cited text no. 3
Nagpal KC, Asdourian GK, Patrianakos D, Goldberg MF, Rabb MF, Goldbaum M, et al. Proliferative retinopathy in sickle cell trait: Report of seven cases. Arch Intern Med 1977;137:325-8.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3]


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