Acute Central Retinal Artery Occlusion Associated with Livedoid Vasculopathy: A Variant of Sneddon's Syndrome
A 32-year-old Korean woman found that she had visual loss in her right eye when she woke up in the morning and was referred to our clinic for acute CRAO 16 hours after symptom occurrence. Eight years prior, she had presented with itching and tender violaceous erythematous non-elevated patches with central necrotic vesicle on the dorsum of both the lower legs and feet () The lesions were improved by colchicines 0.6 mg/day, hydroxyzine, and methylprednisolone 4 to 8 mg/day. Biopsy showed obliterative vasculopathy consistent with LV (). A segmental limb pressure test with a bidirectional Doppler test showed normal blood pressures in all four extremities. She was intermittently treated with pentoxifylline and mupirocin ointment for skin lesions for three years. She was in good medical condition except for the skin lesions and had no experience of smoking. She was not taking oral contraceptives or suffering from migraine. She had one son and no history of spontaneous abortion.\nAt presentation, her visual acuity was hand motion in the right eye and 20 / 20 in the left eye. Fundus examination of the right eye showed whitish edematous retina, a cherry-red spot, and narrowing and segmentation of retinal arteries suggesting acute CRAO () Laboratory test results, including rheumatological tests such as anti-nuclear antibody; anti-Ro and anti-La antibodies; anti-neutrophil cytoplasmic antibody; anti-DNA antibodies; rheumatoid factor; cryoglobulin; antithrombin activity; protein C and S activity; D-dimer; anti-beta2-glycoprotein; anticardiolipin antibody; lupus anticoagulant; prothrombin gene mutation; factor V Leiden gene mutation; and homocysteine, were all within normal limits except for slightly elevated C-reactive protein (0.62 mg/dL) and fibrinogen (425 mg/dL). The serology tests were positive for hepatitis B antigen while other serology tests were normal. Echocardiography and Holter monitoring showed normal findings.\nShe consented to receive intra-arterial thrombolysis after being informed of the possible risks and benefits of the intervention. Cerebral angiography showed severe focal vasospasm of the right internal carotid artery as soon as the catheter was placed (), which was subsequently relieved by continuous intra-arterial infusion of nimodipine 5 mg. Selective angiography of the origin of the right ophthalmic artery showed no definite thrombus or steno-occlusive lesion within the ophthalmic artery (). The ophthalmic artery was infused with a fibrinolytic agent (500,000 units of urokinase) until the maximal dose of fibrinolytics was reached as based on our protocol, but there was no visual improvement during the procedure. Seven hours after intra-arterial thrombolysis, retinal arterial perfusion on fundus fluorescein angiography (FFA) did not improve (arm to retina time, 29 seconds; arteriovenous transit time: about 4 minutes) (). FFA three days after intra-arterial thrombolysis showed nearly complete restoration of retinal arterial perfusion (arm to retina time, 17 seconds; arteriovenous transit time, about 30 seconds) with the exception of the macular area (no reflow phenomenon). Six weeks after thrombolysis, her visual acuity remained hand motion and fundus photography showed severe retinal atrophy in the macula and disc pallor (). Retinal perfusion was restored sparing macula on FFA () and macular photoreceptor disruption was observed on optical coherence tomography.