Department of Ophthalmology, Jones Eye Institute, Little Rock, Arkansas.
Retin Cases Brief Rep. 2023 Jan 1;17(1):44-46. doi: 10.1097/ICB.0000000000001097.
To report a case of bilateral sequential, central retinal vein occlusion (CRVO) in a man with newly diagnosed hemoglobin C trait.
A 67-year-old man presented with a one-month history of declining visual acuity. He was diagnosed with left CRVO. Bilateral temporal retinal ischemia and arteriovenous anastomoses on fluorescein angiography suggestive of sickle cell retinopathy prompted a systemic work-up. Hemoglobin electrophoresis revealed an underlying hemoglobin C trait. Six-months after his initial presentation, the patient developed symptomatic right CRVO.
The initial left CRVO was complicated by optic disc swelling and macular edema. Intravitreal antiangiogenic therapy was initiated and the macular edema resolved. The left eye subsequently developed an epiretinal membrane which was surgically removed. Macular edema in the right eye also resolved after intravitreal antiangiogenic therapy.
This is the first reported case of bilateral CRVO in a case of hemoglobin C trait. It is possible that erythrocyte inflexibility, caused by hemoglobin C-induced dehydration and crystallization, acted concomitantly with hypertension to produce occlusive microangiopathy. This case highlights the need for further investigation in patients presenting with central retinal vein occlusion, especially when bilateral, or when retinal angiography reveals bilateral pathology.
报告 1 例新诊断为血红蛋白 C 特征的男性双侧先后发生的中央视网膜静脉阻塞（CRVO）病例。
1 名 67 岁男性因视力下降 1 个月就诊。诊断为左侧 CRVO。荧光素血管造影显示双侧颞侧视网膜缺血和动静脉吻合，提示镰状细胞视网膜病变，因此进行了系统检查。血红蛋白电泳显示存在潜在的血红蛋白 C 特征。初次就诊后 6 个月，患者出现右侧 CRVO 症状。
最初的左侧 CRVO 并发视盘肿胀和黄斑水肿。给予眼内抗血管生成治疗后黄斑水肿消退。左眼随后出现视网膜内膜，通过手术切除。右眼的黄斑水肿也在眼内抗血管生成治疗后消退。
这是首例报告的血红蛋白 C 特征双侧 CRVO 病例。可能是由于血红蛋白 C 引起的脱水和结晶导致的红细胞僵硬，与高血压同时作用，导致闭塞性微血管病。该病例强调了在出现中央视网膜静脉阻塞的患者中，特别是当双侧出现或视网膜血管造影显示双侧病变时，需要进一步调查。
