Central retinal artery occlusion secondary to Barlow's disease

Abstract

PurposeTo report a rare case of isolated, unilateral CRAO in a young patient with mitral valve prolapse secondary to Barlow's disease.ObservationsA 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.ConclusionsIn young patients presenting with CRAO, aggressive work-up for systemic disease or embolic source must be undertaken to avoid future sequelae.

<p>Intra-Arterial Thrombolysis for Central Retinal Artery Occlusion</p>

“…Retinal vessels and flow normal6/9. Significant restriction with preservation of central fields.Kovach et al47, 2015VA 1/200 (previously 20/50)<12Not reportedInfusion of verapamil followed by alteplaseMMA infused – details not givenOne year later central foveal thickness reduced from 277 to 232 microns and macular volume decreased from 8.53 to 6.98 mm20/70Wilkins et al48, 2018HM10Not reportedNot reportedIAT via OA – details not givenOCT showed reduced thickness of RNFL at 1 weekHM Abbreviations: Rx, treatment; rTPA, recombinant tissue plasminogen activator; LMWH, low molecular weight heparin; MMA, middle meningeal artery; OA, ophthalmic artery; ECA, external carotid artery; FFA, fluorescein fundus angiography; VA, visual acuity; VF, visual fields; logMAR, logarithm of the minimal angle of resolution; HM, hand movements; CF, counting fingers; PL, light perception; NPL, nil perception of light.…”

“…Twelve case reports were included40–48 (Table 2). Four additional case reports are reported separately as these patients developed CRAO during or post-procedure (Table 3).…”

“…Arteritic CRAO was excluded in 6 cases41,44,45,47,48 based on reported history (arteritic symptoms excluded) and investigations (normal CRP and ESR). Embolus composition was only mentioned in 1 case41 where Hollenhurst plaques were observed and this patient achieved VA of 20/20 after IAT.…”