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Ophthalmic Images
January 10, 2019

Purtscher-Like Retinopathy in Hemolytic Uremic Syndrome

Author Affiliations
  • 1Department of Ophthalmology, Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, Maryland
JAMA Ophthalmol. 2019;137(1):e183911. doi:10.1001/jamaophthalmol.2018.3911

A 30-year-old previously healthy man presented with 1 month of rapidly declining vision in both eyes following acute gastroenteritis. His visual acuity was light perception OD and hand motions OS, respectively. Funduscopic examination revealed bilateral cotton-wool spots, Purtscher flecken, scattered intraretinal hemorrhages, and arteriolar attenuation consistent with Purtscher-like retinopathy (Figure, A). Fluorescein angiography revealed extensive macular ischemia (Figure, B). Workup revealed hemolytic anemia and thrombocytopenia concerning for thrombotic microangiopathy from likely hemolytic uremic syndrome triggered by the diarrheal illness. Clinical course was characterized by renal failure, status epilepticus, myocardial infarction, respiratory failure, and neovascular glaucoma leading to bilateral blindness. Sequential treatment with pulse dose steroids, intravenous immunoglobulin, plasmapheresis, and eculizumab led to stabilization of disease without improvement in vision or functional status. Purtscher-like retinopathy is a rare severe angiopathy secondary to complement-mediated leukoembolization associated with various systemic conditions and should prompt systemic workup.

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