Naysan Jonathan, Dansingani Kunal K, Balaratnasingam Chandrakumar, Mrejen Sarah, Levasseur Steven, Merkur Andrew, Yannuzzi Lawrence A
Vitreous Retina Macula Consultants of New York, New York.
LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York.
Retin Cases Brief Rep. 2018;12(4):331-335. doi: 10.1097/ICB.0000000000000494.
To report the posterior segment and retinal vascular manifestations of calcific uremic arteriolopathy (calciphylaxis). Clinical findings are correlated with multimodal imaging results.
Observational case report.
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.
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.
报告钙化性尿毒症小动脉病(calciphylaxis)的眼后段及视网膜血管表现。将临床发现与多模态成像结果进行关联。
观察性病例报告。
一名65岁接受血液透析的白人女性因双侧视力不佳前来评估。临床检查显示为结晶性视网膜病变,并伴有既往视网膜动脉阻塞的痕迹。荧光素血管造影显示双侧视网膜动脉充盈延迟、血管鞘形成及周边无灌注。这些晶体呈高自发荧光。光谱域和增强深度成像光学相干断层扫描将晶体定位在视网膜内,且偏好于视网膜动脉血管系统。脉络膜毛细血管未受累。两年前,该患者出现坏死性皮肤病变,经活检确诊为钙化性尿毒症小动脉病。
钙化性尿毒症小动脉病是终末期肾病患者血栓性微血管病的极其罕见的病因。视网膜动脉阻塞似乎是该疾病中视力丧失的罕见但重要的原因,并且很可能是视网膜血管中晶体沉积的结果。