Purtscher-Like Retinopathy Associated with Atypical Hemolytic Uremic Syndrome

Abstract

A 25-year-old woman presented with acute bilateral blurred vision and history of headache, dizziness, and syncope for three days. Her visual acuity was 20/60 in both eyes. Fundoscopy revealed multiple bilateral peripapillary yellow-white patches like cotton wool spots, intraretinal hemorrhages and macular edema. The patient was diagnosed with Purtscher-like retinopathy based on the retinal findings and lack of trauma history. She was urgently admitted to the nephrology clinic due to thrombotic microangiopathy findings (hemoglobinemia, thrombocytopenia, and acute renal failure). After excluding thrombotic microangiopathy, the patient was diagnosed with atypical hemolytic uremic syndrome (aHUS) with the clinical and laboratory findings. Eculizumab treatment was added to hemodialysis and plasmapheresis therapy. Three months after starting treatment, retinal lesions regressed and visual acuity increased to 20/20 in both eyes. To the best of our knowledge, this is the first reported case of Purtscher-like retinopathy associated with aHUS.

Keywords: Atypical hemolytic uremic syndrome; Purtscher retinopathy; Purtscher-like retinopathy; eculizumab; thrombotic microangiopathy.

Figure 1. Initial fundus photography, fluorescein angiography and optical coherence tomography findings: (a) Bilateral multiple peripapillary yellow-white patches, flame-shaped intraretinal hemorrhages, and macular edema (b) Bilateral peripapillary hyperfluorescent spots (c) Serous macular detachment at optical coherence tomography in both eyes

Figure 2. Fundus photography and optical coherence tomography findings in the third month of follow-up: (a) Total resolution of the retinal findings (b) Complete regression of subretinal fluid demonstrated by optical coherence tomography