Retinal hypoperfusion detected by wide-field optical coherence tomographic angiography in a case of retinal racemose hemangioma.

Abstract

Retinal racemose hemangioma (RRH) is a rare, non-hereditary arteriovenous malformation characterized by the appearance of dilated and tortuous retinal vessels. RRH can develop complications associated with retinal ischemia, such as vitreous hemorrhage, retinal vein occlusion and neovascular glaucoma. Here, we report a case of RRH with retinal hypoperfusion detected by wide-field swept source optical coherence tomography angiography (OCTA), which was not unambiguously illustrated by fluorescein angiography (FA). Case report. A 57-year-old woman was referred to our hospital for the evaluation of severe retinal vascular tortuosity, dilation and retinal hemorrhages in the left eye. Fundus examination revealed arteriovenous communications temporal to the fovea and multiple microaneurysms surrounded by retinal hemorrhages at the midperipheral temporal fundus. In FA, multiple hyperfluorescent lesions with leakage corresponding to microaneurysms were observed in the temporal and lower midperipheral areas; however, non-perfused areas were apparently absent. By contrast, wide-field OCTA clearly showed low-density retinal capillaries in the superotemporal quadrant in comparison with those in the inferotemporal quadrant. Wide-field OCTA detected sparse retinal capillaries, which were not well illustrated by FA, in a patient with RRH. This indicates the presence of low-grade retinal hypoperfusion caused by altered retinal hemodynamics, potentially leading to ischemia-related retinal disorders during a prolonged course, in patients with clinically quiescent RRH.