This previously healthy 29YO female presented with 2 days of sudden vision loss in her left eye. Vision was 20/20 in her normal OD and light perception OS.
Color photography shows an acute central retinal artery occlusion (CRAO) with diffuse retinal opacification and a prominent foveal cherry-red spot. The retinal arterioles are markedly attenuated with segmentation of the arterial and venous blood columns (box-carring). OCT shows inner retinal thickening and opacification except in the foveal center. Cardiac imaging revealed a left atrial myxoma, and she underwent immediate surgical excision.
Learning Points:
Atrial myxoma, the most common primary cardiac tumor, is typically attached to the interatrial septum at the fossa ovalis and can shed tumor fragments, thrombi, or surface debris into the systemic circulation (Griborio-Guzman et al, Heart 2022;108:827-833). Embolism occurs in 30-40% of patients, and the cerebral and retinal arteries are the most affected vascular beds. The vast majority of CRAOs occur in a more elderly population from carotid or valvular disease. Atrial myxomas are a rare but known cause, especially in younger patients.
Half of patients have an elevated erythrocyte sedimentation rate (ESR) due to tumor-produced interleukin-6 (Acebo et al, Chest 2003;123:1379-1385). These cytokines drive the systemic inflammatory response that produces constitutional symptoms (fever, fatigue, weight loss, arthralgias, myalgias) and laboratory abnormalities (elevated ESR/CRP, anemia, leukocytosis, elevated globulins). Elevated inflammatory markers can be a diagnostic pitfall, as they frequently lead to misdiagnosis of endocarditis, rheumatic fever, vasculitis, collagen vascular disease, or even polymyalgia rheumatica.
