Diffuse Retinal Arterial Occlusion, Neovascularization, and Vitreous Hemorrhage as the Presenting Sign of Sneddon Syndrome: A 7.5-YEAR Angiographic Follow-Up

Abstract

The authors describe a case of Sneddon syndrome presenting with vitreous hemorrhage and describe 7 years of follow-up with increasingly severe retinal vaso-occlusive events.S neddon syndrome (SS) is a rare disease that was first described in 1965; it is characterized by the association of cutaneous purple lacelike rash (livedo reticularis or racemosa), ischemic cerebrovascular events, and labile hypertension. 1 Visual involvement in SS has been rarely described and includes homonymous hemianopia secondary to cerebral infarction, 2 bilateral central artery occlusion, 3 and peripheral retinal neovascularization. 4 We describe a 48-year-old woman with monocular relapsing vitreous hemorrhage (VH) secondary to retinal neovascularization and diffuse arterial occlusion. To our knowledge, this is the first report of relapsing VH requiring pars plana vitrectomy as a presenting sign of SS; correlation between a 7.5-year angiographic follow-up and systemic disease progression is provided. Case ReportIn September 1998, a 48-year-old woman presented complaining of floaters and loss of vision in her left eye over the past 20 days. At presentation, visual acuity was 20/20 in the right eye and 20/400 in the left eye; slit-lamp examination of both eyes revealed clear cornea with deep and quiet anterior chamber and minimal nuclear sclerosis. Indirect ophthalmoscopy of the right eye showed mild vitreous syneresis with no cells and signs of peripheral retinal arteriolar occlusion at 4 o'clock. This analysis of the left eye showed moderate VH with some subhyaloidal heme, scattered epiretinal hemorrhages, neovascularization of the optic disk, arterial occlusion with signs of perivascular sheathing, and retinal edema superotemporally to the fovea. Fluorescein angiography (FA) revealed delayed choroidal filling inferior to the optic disk in the right eye (Fig. 1, A and B) and arterial occlusion and retinal edema superior to the fovea and some blocking due to vitreous and retinal heme in the left eye ( Fig. 2A). Early frames showed arterial filling defects, macular ischemia, and blocking due to retinal edema (Fig. 2B), while equatorial arteriovenous frames (Fig. 2, C, D, and F) disclosed vessel nonperfusion associated with staining and leakage. Late frames (Fig. 2E) showed optic nerve leakage due to neovascularization of the optic disk. Twenty days later, FA of the right eye demonstrated a filling defect of an arterial branch located 0.5 disk diameter inferior to the disk (Fig. 3, A and B). FA of the left eye had not changed substantially. Visual fields were full.The patient underwent extensive systemic evaluation and blood testing that revealed antiphospholipid antibodies present as anticardiolipin IgG and antinuclear and anti-smooth muscle antibodies well over the normal limits. Tests for rheumatoid factor and lupus anticoagulant were negative. Blood cell count was unremarkable except for thrombocytopenia (platelet count, 112,000/mL). Testing for thrombophilic conditions also was done, including screening for factors II, Leiden, and AT...