Double Trouble: Eales Disease in a Background of Paradoxical Embolism

Abstract

Eales disease is an idiopathic retinal vasculitis that mainly affects the periphery of the retina. The disease commonly manifests as peripheral retinal perivasculitis, peripheral retinal capillary nonperfusion, neovascularization, and recurrent vitreous hemorrhage. Here, we present the case of a 36-year-old male who was diagnosed with Eales disease after presenting with sudden onset flashes of light, reduced visual acuity, and a black spot in his left eye. Upon examination, his left eye exhibited a superior non-foveal branch retinal artery occlusion (BRAO) with a sludged blood column, an old extramacular branch retinal vein occlusion (BRVO) with hemorrhage, and vascular sheathing. Initial laboratory investigations, including antibody testing for causes of retinal ischemia and stroke workup, were negative. Later, the patient presented with a BRAO in the right eye and a cerebral infarction shortly thereafter, further complicating his clinical picture. A diagnosis of Eales disease was made based on the evolution of retinal findings showing peripheral non-perfusion, vascular sheathing, collateral formation, neovascularization with leakage, absence of additional BRAOs following repair of his patent foramen ovale, and lack of other explanatory conditions. The initiation of systemic corticosteroids resulted in the improvement and stabilization of his vision. This case highlights the challenges in diagnosing Eales disease, underscoring the importance of timely identification for the appropriate management and prevention of vision loss.

Keywords: brao; brvo; eales disease; paradoxical embolism; patent foramen ovale; retinal ischemia; retinal vasculitis.

Figure 1. Optos ultra-widefield imaging (left) showing a BRAO (blue arrow), sclerotic vessels from an old inferior temporal BRVO (red arrow), and dot blot hemorrhages (green arrow). Vascular sheathing is far in the periphery and beyond the observable field of view. FA (right) showing filling defects in the superotemporal artery (blue arrow) and an old BRVO (white arrow).

Figure 2. (Left) Nasal BRAO superonasally (blue arrow). (Right) FA showing a filling defect in the superior nasal field (blue arrow).

Figure 3. Follow-up Optos ultra-widefield imaging (left) demonstrating well-circumscribed areas of ischemia (red box) with FA (right) showing leakage of retinal vessels at the margin between the perfused and ischemic retina (yellow box).

Figure 4. Panretinal photocoagulation burn scars in both eyes at various areas of ischemia (red outlines).