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Photo Essay
September 2001

Purtscher Retinopathy as the Initial Sign of Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic Syndrome

Arch Ophthalmol. 2001;119(9):1388-1389. doi:10.1001/archopht.119.9.1388

A 52-YEAR-OLD African American woman had a 3-week history of progressive decline in bilateral vision. Ocular examination revealed bilateral diffuse retinal edema and whitening and confluent cotton-wool spots centered around the peripapillary region. Also noted were bilateral marked attenuation of the retinal arterioles, scattered retinal hemorrhages in both eyes, and a cherry-red spot in the right eye (Figure 1). Fluorescein angiography demonstrated severe capillary dropout of the macular and peripapillary retinal vasculature in both eyes in the arterial phase (Figure 2A, 2B) and perivascular leakage with severe capillary dropout in both eyes in the late arteriovenous phase (Figure 2C, 2D), accounting for a visual acuity of counting fingers at 2 feet in both eyes. The patient was initially diagnosed as having Purtscher retinopathy secondary to a questionable vasculitic/collagen vascular origin. In-hospital work-up revealed nonhemolytic anemia without thrombocytopenia, renal failure, neurological disease, or collagen vascular disease. Oral prednisone was given at the suggestion of a rheumatologic consultation to treat a presumed autoimmune origin. Follow-up ocular examination results remained unchanged. Nine days after the initial visit, the patient developed generalized seizures, acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia. Asystole followed, which was refractive to resuscitative efforts. An autopsy revealed evidence of platelet microthrombin in the eye, heart, lung, and kidney (Figure 3).

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