Central retinal artery occlusion secondary to Barlow's disease

Purpose: To report a rare case of isolated, unilateral CRAO in a young patient with mitral valve prolapse secondary to Barlow's disease. Observations: A 29-year-old woman with history of premature ventricular contractions and cardiac ablation presented to the emergency room after sudden onset painless visual loss in her left eye (OS). Her vision was 20/20 in her right eye and hand motion in the left. Fundus exam demonstrated a central retinal artery occlusion (CRAO) OS. Computerized tomography of head and neck were unremarkable. She underwent cerebral angiogram and local intra-arterial thrombolysis. Her vision remained stable post-procedure, with marked APD and stable fundus examination. Her cardiac work-up revealed a left atrial mass with calcified mitral valve, and small atrial septal defect. Rheumatologic, hematologic, and auto-immune work-up were unremarkable. She underwent resection of the mass with repair of mitral valve and ASD closure. Surgical pathology was compatible with diagnosis of Barlow's disease, a cause of mitral valve prolapse. The patient underwent intravitreal injection of anti-VEGF therapy at one month follow-up, with vision stable at hand motion and without neovascularization on subsequent evaluation. Conclusions: In young patients presenting with CRAO, aggressive work-up for systemic disease or embolic source must be undertaken to avoid future sequelae.