P2D123: Central Retinal Artery Occlusion in a 17-year-old Female

Introduction: Central retinal artery occlusion (CRAO) manifests as sudden, painless monocular vision loss. In the pediatric and young adult population, retinal artery occlusion is typically associated with a hypercoagulable or embolic state. In adults, atheromatous disease typically causes an occlusion. Less common etiologies include Antiphospholipid Syndrome, Systemic Lupus Erythematosus and infection. Idiopathic cases of Branch Retinal Artery Occlusion (BRAO) have been documented. This case report describes a 17-year-old female with CRAO of the right eye.

Case Description: A 17-year-old previously healthy female presented to the emergency room with sudden painless right-sided vision loss. About 7 hours prior to presentation, she developed blurry vision followed by complete vision loss in her right eye.
On physical exam, she had complete visual field impairment in the right eye without tenderness on palpation or to extraocular movements. Her right fundus exam showed extensive Berlin’s edema with a cherry red macula. No embolus was seen. She also had livedo racemosa in bilateral lower legs. The rest of her exam was unremarkable.
Broad laboratory workup was notable for normocytic anemia, mild leukopenia, d-dimer of 2,292, erythrocyte sedimentation rate of 89 and mildly elevated partial thromboplastin time. MRI brain and MRI orbits W WO showed nonspecific peripheral enhancement of the intra-orbital segment of the right optic nerve sheath. This was felt to be secondary to ischemia or an underlying inflammatory process. Ophthalmology, neurology and neurosurgery were consulted. Systemic tissue plasminogen activator (TPA) was not recommended. Intra-arterial TPA was unavailable at our center. Ophthalmology intervened with intraocular pressure lowering agents via ocular massage, intraocular pressure drops, and anterior chamber paracentesis. She was started on low dose aspirin. Rheumatology and hematology were consulted; additional laboratory workup was notable for positive cardiolipin Immunoglobulin (Ig) M (25), positive antistreptolysin O, elevated IgG 1, IgG 2 and IgG 3, and normal IgG 4. An echocardiogram with bubble study was unremarkable. The patient was discharged with close follow up. She was later given a three day steroid pulse with Solumedrol followed by a taper. This did not result in gain of vision.

Discussion: The management of CRAO is challenging given the time sensitivity and varying approaches to treatment. In this case, treatment options were limited due to delayed presentation. Varma et al (2013) showed that treatment for CRAO should be given within 6 hours of insult. Rudkin et al. demonstrated similar outcomes between the treatment regimen including anterior chamber paracentesis, ocular massage and intraocular hypotensive agents versus observation (2010). Additional therapies, including intra-arterial thrombolytic therapy, surgical revascularization, systemic steroids and systemic thrombolytic therapy are being studied.

Conclusion: Time to diagnose and treat CRAO is crucial. Rapid determination of the etiology will define treatment. The underlying etiology workup is in this patient is ongoing.