Jonathan Naysan1,2,3,4, Kunal K Dansingani1,2, Chandrakumar Balaratnasingam1,2, Sarah Mrejen1,2, Steven Levasseur5, Andrew Merkur5, Lawrence A Yannuzzi1,2,3,4. 1. Vitreous Retina Macula Consultants of New York, New York. 2. LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York. 3. Department of Ophthalmology, New York University School of Medicine, New York, New York. 4. Department of Ophthalmology, North-Shore Long Island Jewish Health System, Manhasset, New York. 5. Department of Ophthalmology and Visual Sciences, University of British Columbia, Vancouver, British Columbia, Canada.
Abstract
PURPOSE: To report the posterior segment and retinal vascular manifestations of calcific uremic arteriolopathy (calciphylaxis). Clinical findings are correlated with multimodal imaging results. METHODS: Observational case report. RESULTS: A 65-year-old white woman on hemodialysis was referred for assessment of poor vision bilaterally. Clinical examination demonstrated a crystalline retinopathy with stigma of previous retinal arterial occlusion. Fluorescein angiography revealed delayed retinal arterial filling bilaterally, sheathing of vessels, and peripheral nonperfusion. The crystals were hyperautofluorescent. Spectral domain and enhanced depth imaging optical coherence tomography localized the crystals within the retina with a predilection for the retinal arterial vasculature. The choriocapillaris was not involved. Two years prior, the patient developed necrotic skin lesions which were biopsied and confirmed the diagnosis of calciphylaxis. CONCLUSION: Calcific uremic arteriolopathy is an extremely rare cause of thrombogenic microangiopathy in end-stage renal disease patients. Retinal arterial occlusion appears to be a rare but significant cause of visual loss in this disease and is likely to be consequent to crystalline deposition in the retinal vasculature.
PURPOSE: To report the posterior segment and retinal vascular manifestations of calcific uremic arteriolopathy (calciphylaxis). Clinical findings are correlated with multimodal imaging results. METHODS: Observational case report. RESULTS: A 65-year-old white woman on hemodialysis was referred for assessment of poor vision bilaterally. Clinical examination demonstrated a crystalline retinopathy with stigma of previous retinal arterial occlusion. Fluorescein angiography revealed delayed retinal arterial filling bilaterally, sheathing of vessels, and peripheral nonperfusion. The crystals were hyperautofluorescent. Spectral domain and enhanced depth imaging optical coherence tomography localized the crystals within the retina with a predilection for the retinal arterial vasculature. The choriocapillaris was not involved. Two years prior, the patient developed necrotic skin lesions which were biopsied and confirmed the diagnosis of calciphylaxis. CONCLUSION:Calcific uremic arteriolopathy is an extremely rare cause of thrombogenic microangiopathy in end-stage renal diseasepatients. Retinal arterial occlusion appears to be a rare but significant cause of visual loss in this disease and is likely to be consequent to crystalline deposition in the retinal vasculature.