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Case Reports
. 2020 May 27;11(2):189-195.
doi: 10.1159/000507879. eCollection 2020 May-Aug.

Hemoglobin S/OArab: Retinal Manifestations of a Rare Hemoglobinopathy

Riley Sanders  1 , Victoria Ly  2 , Kinza Ahmad  1 , Jesse Swift  1   3 , Ahmed Sallam  1 , Sami Uwaydat  1
Affiliations
Case Reports

Hemoglobin S/OArab: Retinal Manifestations of a Rare Hemoglobinopathy

Riley Sanders et al. Case Rep Ophthalmol. .

Abstract

Hemoglobin S/OArab (Hgb S/OArab) disease is a rare hemoglobinopathy which presents similarly to sickle cell retinopathy, with only three prior reports that describe associated retinal findings. In this report, we present ophthalmic examination findings in 2 patients with Hgb S/OArab. One patient exhibited peripheral ischemia and sunburst lesions without neovascular disease, and the other patient developed proliferative retinopathy of both eyes and multiple posterior-pole branch retinal artery occlusions in one eye. To our knowledge, this is the first case of retinal arterial occlusive disease in Hgb S/OArab, and the first report of fundus autofluorescence and OCT angiography in Hgb/OArab retinopathy.

Keywords: Hemoglobin S/OArab; Hemoglobinopathy; Retinal ischemia; Sickle cell retinopathy.

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Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

Fig. 1
Fig. 1
a Red/green Optos® wide-field image demonstrates sclerotic vessels (thin arrow) and sunburst lesion (asterisk). b Optos wide-field fundus autofluorescence appearance. Sunburst (asterisk) appears as a hypoautofluorescent lesion surrounded by hyperautofluorescence.
Fig. 2
Fig. 2
Late-phase Optos® fluorescein angiography image taken of patient 1 shows peripheral ischemia (star) and arteriovenular anastomoses (bold arrows), but without any neovascular leakage.
Fig. 3
Fig. 3
Late-phase Optos® FA of patient 2 demonstrates arteriovenular anastomoses with areas of neovascular leakage.
Fig. 4
Fig. 4
a Color fundus photograph of the patient's right eye after vitrectomy to clear vitreous hemorrhage. Note the appearance of tractional membranes overlying the macula; partial segmentation had been performed, but delamination was avoided over the vascular arcade. Dark areas superiorly correspond to laser treatment. b Vertical Zeiss Cirrus® OCT cross-section of the right eye through the fovea, showing persistent tractional schisis of the macula. c Color photograph of the left eye, showing whitish areas of perifoveal edema consistent with multiple BRAO. d Horizontal OCT cross-section of the left eye through the fovea taken several weeks after the color image was taken, showing inner retinal atrophy suggestive of past retinal arterial infarct.
See this image and copyright information in PMC

References

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