A HEALTHY 28-year-old Pakistani woman developed fever, malaise, and diffuse edema, rapidly progressing to multiple organ failure with encephalopathy, anuric renal failure, hemolytic anemia, and thrombocytopenia. The patient was diagnosed with thrombotic thrombocytopenic purpura (TTP) and treated with hemodialysis, intravenous corticosteroids, and plasma exchange with cryoprecipitate-poor plasma. After 2 weeks, her mental status improved, but she complained of bilateral vision loss. Visual acuity was light perception in both eyes. Examination revealed bilateral vascular compromise with sheathing, cotton-wool spots, intraretinal hemorrhages, and optic disc pallor (Figure 1). Fluorescein angiography was significant for profound nonperfusion of retinal circulation (Figure 2). Figure 1. View LargeDownload Vascular compromise with sheathing, cotton-wool spots, intraretinal hemorrhages, and optic disc pallor. Left, right eye; right, left eye. Figure 2. View LargeDownload Fluorescein angiogram revealing nonperfusion of the retinal circulation throughout the entire course of the study. Left, Right eye, early frame. Right, Left eye, late frame. Medical evaluation yielded no apparent precipitating factor for TTP. The patient's medical status slowly improved, although there was very limited visual improvement, to the level of hand motions in both eyes. She gradually developed iris and angle neovascularization, which was treated with bilateral panretinal photocoagulation, and eventual Nd:YAG transcleral cyclophotocoagulation. Despite this treatment, the intraocular pressure remained elevated, and the patient lost all visual function. Comment The patient's ophthalmoscopic and angiographic features were compatible with combined occlusion of the central retinal artery and vein. The differential diagnosis of these findings includes collagen vascular disorders, dysproteinemia, Purtscher retinopathy, septic embolization, and leukemia. However, the findings of anemia, thrombocytopenia, fever, neurologic abnormalities, and renal failure are the 5 cardinal signs of TTP. The pathogenesis of this rare disorder involves intravascular platelet aggregation with platelet thrombus formation in the microcirculation of end organs. Thrombotic thrombocytopenic purpura may be either idiopathic or triggered by collagen vascular disease, cancer, pregnancy, human immunodeficiency virus infection, or other events. The spectrum of ophthalmologic findings in TTP is attributable to microinfarction of either retinal or choroidal circulation, and includes intraretinal hemorrhages with cotton-wool spots,1 disc hemorrhages and neovascularization with optic atrophy,2 serous retinal detachment,3-5 and Purtscherlike retinopathy.6 To our knowledge, this is the first case known to us of TTP with bilateral combined occlusion of the central retinal artery and vein, progressing to loss of light perception. This case documents the profound effect that TTP may have on the visual system. Corresponding author: William F. Mieler, MD, McPherson Retina Center, Baylor College of Medicine, 6560 Fannin, Suite 2200, Houston, TX 77030 (e-mail: firstname.lastname@example.org). References 1. Percival SPB Ocular findings in thrombotic thrombocytopenic purpura (Moschcowitz's disease). Br J Ophthalmol. 1970;5473- 78Google ScholarCrossref 2. Lewellen DRSingerman LJ Thrombotic thrombocytopenic purpura with optic disk neovascularization, vitreous hemorrhage, retinal detachment, and optic atrophy. Am J Ophthalmol. 1980;89840- 844Google Scholar 3. Lambert SRHigh KACotlier EBenz EJ Jr Serous retinal detachments in thrombotic thrombocytopenic purpura. Arch Ophthalmol. 1985;1031172- 1174Google ScholarCrossref 4. Hemeter W Presumed thrombotic thrombocytopenic purpura associated with bilateral serous retinal detachments. Am J Ophthalmol. 1988;105421- 422Google ScholarCrossref 5. Slusher MMPugh HPHackel RP Bilateral serous retinal detachment in thrombotic thrombocytopenic purpura. Arch Ophthalmol. 1990;108744- 745Google ScholarCrossref 6. Power MHRegillo CDCustis PH Thrombotic thrombocytopenic purpura associated with Purtscher retinopathy. Arch Ophthalmol. 1997;115128- 129Google ScholarCrossref
Archives of Ophthalmology – American Medical Association
Published: Sep 1, 2000
Keywords: thrombotic thrombocytopenic purpura,central retinal artery
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