Ophthalmic Manifestations as First Presenting Sign of Evans Syndrome

This work reports a case of Evans syndrome first presenting with ophthalmic signs.

A 27-year-old previously healthy man presented with headaches and bilateral blurred vision for 2 weeks. The visual acuity was 20/30^-1 and 20/60^-2 in the right eye and left eye, respectively. A fundus examination revealed Roth spots, diffuse multilayer retinal hemorrhages in the macula and periphery, and tortuous vessels in both eyes. Optical coherence tomography showed a disrupted foveal contour from intraretinal fluid and hemorrhage in both eyes. Fluorescein angiography demonstrated dilated and tortuous vessels with scattered blocking defects from hemorrhages.

A workup revealed warm hemolytic anemia with severe thrombocytopenia consistent with the diagnosis of Evans syndrome.

Evans syndrome is a rare blood dyscrasia that can first present as subacute vision loss and should be added to the differential of diffuse bilateral retinal hemorrhages spanning a multitude of retinal layers.