Macular Microangiopathy in Sickle Cell Disease Using Optical Coherence Tomography Angiography

To characterize the optical coherence tomography angiography (OCTA) appearance of the perifoveal macular microvasculature in visually asymptomatic patients with sickle cell disease, and to compare these findings with those of fluorescein angiography (FA).

Retrospective observational case series.

Eighteen eyes of 9 consecutive patients with a median age of 41 years (range: 19-54 years) with electrophoretic confirmation of sickle cell disease were included and analyzed. A complete ophthalmologic examination was performed, including fundus examination, FA, and OCTA. Nine eyes of 5 healthy subjects were also analyzed with OCTA to serve as a control group.

OCTA demonstrated microvascular abnormalities in the perifoveal region of the macula in all eyes, whereas FA appeared normal in 9 of 18 eyes (50%). Most capillary abnormalities were located in the temporal juxtafoveal region and involved both the superficial and the deep capillary plexuses. The nonflow area (foveal avascular zone) was significantly larger in sickle cell disease patients than in the control group, both in the superficial and the deep capillary plexuses (P < .0001). The perifoveal vessel density was significantly lower in the sickle cell disease patients than in the control group in both the superficial (P = .0011) and the deep capillary plexuses (P = .0018).

OCTA provided detailed imaging of the perifoveal microvasculature in sickle cell disease. It appeared more sensitive than FA in detecting macular microangiopathy in asymptomatic patients. Microvascular abnormalities in sickle cell disease involved both the superficial and the deep capillary plexuses.