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Brief Report |

Macular Vascular Abnormalities Identified by Optical Coherence Tomographic Angiography in Patients With Sickle Cell Disease

Ian C. Han, MD1; Mongkol Tadarati, MD1,2; Adrienne W. Scott, MD1
[+] Author Affiliations
1Retina Division, Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, Maryland
2Retina Division, Department of Ophthalmology, Rajavithi Hospital, College of Medicine, Rangsit University, Bangkok, Thailand
JAMA Ophthalmol. 2015;133(11):1337-1340. doi:10.1001/jamaophthalmol.2015.2824.
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Published online

Importance  Patients with sickle cell disease may develop various macular vascular abnormalities that have not been described previously and can be seen using optical coherence tomographic angiography.

Observations  Ten eyes from 5 consecutive patients (3 men and 2 women) with sickle cell disease (4 patients with hemoglobin SS disease and 1 patient with hemoglobin SC disease) were included. The mean age was 37.6 years. Five of 10 eyes (50%) had retinal thinning that was identified using spectral-domain optical coherence tomography. Each of these eyes had corresponding loss of vascular density in the superficial or deep retinal plexus (or both).

Conclusions and Relevance  Optical coherence tomographic angiography provides a novel way to view the retinal vasculature, including the superficial and deep capillary plexus, and may provide a sensitive method for identifying macular vascular abnormalities in patients with sickle cell disease.

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Figure 1.
Multimodal Imaging of the Right Eye in Case 1

A, Retinal thickness map for part B (the box shows the area imaged in parts C-E). B, Spectral-domain optical coherence tomographic (SD-OCT) findings show temporal thinning (arrowhead). C, Findings from a fluorescein angiogram show normal filling. D, Optical coherence tomographic angiography (OCT-A) findings show a superficial plexus that is relatively preserved. E, Findings from OCT-A of the deep plexus show nonperfusion corresponding to the area of retinal thinning (arrowheads, C-E).

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Figure 2.
SD-OCT and OCT-A Images of the Right Eye in Case 2

A, Optical coherence tomographic angiography (OCT-A) of the superficial plexus shows a widened foveal avascular zone (arrowheads). B, Findings from OCT-A of the deep plexus show an enlarged foveal avascular zone with adjacent areas of nonperfusion (arrowheads). C, Findings from a spectral-domain optical coherence tomographic (SD-OCT) line scan (3 mm) show a widened foveal contour (arrowheads).

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