Central Retinal Vein Occlusion as a Possible Presenting Manifestation of Sneddon Syndrome

Abstract

We report a case of central retinal vein occlusion (CRVO) and livedo reticularis in a patient in whom Sneddon syndrome was subsequently diagnosed. A 61-year-old man presented with a sudden and painless decline in visual acuity of his right eye. Visual acuities evaluated with Early Treatment Diabetic Retinopathy Study (ETDRS) measurements were 0.62 ETDRS lines (20/60 Snellen equivalent) for the right eye and 0.9 ETDRS lines (20/30 Snellen equivalent) for the left eye, there was no relative afferent pupillary defect, and intraocular pressures were 18 mm Hg in both eyes. Examination of the right fundus revealed retinal edema, optic disc swelling, tortuous and dilated retinal veins, and extensive superficial hemorrhages in all four quadrants. A diagnosis of acute CRVO was made. Pre-fluorescein angiographic red-free fundus photography (Fig. 1) showed venous stasis but good retinal capillary perfusion, disc edema, tortuosity, and dilation of all branches of the central retinal vein and a few scattered peripheral fundus hemorrhages, consistent with nonischemic CRVO. The periphery of the eye showed no extensive areas of capillary nonperfusion or neovascularization.FIG. 1: Red-free fundus photography of the right eye at presentation shows tortuosity and dilation of all branches of the central retinal vein, mild disc edema, a few scattered peripheral fundus hemorrhages, and good retinal capillary perfusion. These are features consistent with nonischemic central retinal vein occlusion.The patient had no personal or family history of eye disease, systemic hypertension, diabetes mellitus, thrombophilia, or malignancy. Systemic examination revealed a generalized livid discoloration of the skin in a net-like pattern all over the body except on the face (Fig. 2). The patient had noted the skin discoloration 13 days earlier.FIG. 2: Left hip shows livedo reticularis, a reddish netlike discoloration of the skin.Results of ultrasound studies of the heart and extracranial vessels as well as laboratory serology analyses were negative, including antinuclear antibodies (ANAs), antineutrophilic cytoplasmic antibodies (ANCAs), complement C3 and C4, antiphospholipid antibodies, and lupus anticoagulant, prothrombin time, antithrombin III, fibrinogen, protein C, protein S, and activated protein C resistance. Brain MRI showed an old cerebral infarction in the left basal ganglia. Aortic arch, neck, and brain MRA revealed no evidence of atherosclerosis or vasculitis. A skin and skeletal muscle biopsy revealed endothelial thickening in the small vessels of the subcutis without signs of vasculitis, consistent with Sneddon syndrome. The patient was treated with 400 mg intravenous pentoxifylline daily and 7,500 IU dalteparin sodium subcutaneously for 7 days, followed by 100 mg aspirin per day. On re-examination 3 months later, visual acuity had increased to 0.71 ETDRS lines (20/50 Snellen equivalent) for the right eye and remained at 0.9 ETDRS lines (20/30 Snellen equivalent) for the left eye. The right fundus revealed persisting optic disc swelling and retinal hemorrhages but a reduction in retinal edema. Repeated fluorescein angiography showed no signs of ischemia of the right eye. Sneddon syndrome, first described in 1965, is characterized by generalized livedo reticularis and stroke (1,2). The etiology of this syndrome is unknown, although there are correlations with the antiphospholipid syndrome, systemic secondary vasculitis, and coagulopathies. It may start as an inflammatory and possibly immunologically mediated disorder and lead to a proliferation of smooth cells of small blood vessels and obstruction of the vessel lumen. Although central retinal artery occlusion (3-5) and peripheral retinal capillary occlusions and neovascularization (6) have been reported previously in Sneddon syndrome, CRVO has not. It is unclear whether the CRVO in our patient was directly related to Sneddon syndrome. Tina Aggermann, MD Ludwig Boltzmann Institute for Retinology and Biomicroscopic Laser Surgery Department of Ophthalmology Rudolf Foundation Clinic Vienna, Austria [email protected] Paulina Haas, MD Ludwig Boltzmann Institute for Retinology and Biomicroscopic Laser Surgery Vienna Austria Susanne Binder, MD Ludwig Boltzmann Institute for Retinology and Biomicroscopic Laser Surgery Department of Ophthalmology Rudolf Foundation Clinic Vienna, Austria