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β-地中海贫血伴缺铁性贫血以中央性视网膜血管阻塞为首发表现

COMBINED CENTRAL RETINAL VASCULAR OCCLUSION AS THE PRESENTING FEATURE IN β-THALASSEMIA WITH IRON DEFICIENCY ANEMIA.

机构信息

Ophthalmic Center, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, China; and.

Department of Ophthalmology, University of Colorado, Boulder, Colorado.

出版信息

Retin Cases Brief Rep. 2024 May 1;18(3):393-395. doi: 10.1097/ICB.0000000000001392.

Abstract

PURPOSE

To report a case of β-thalassemia trait with iron deficiency anemia presenting as a combined central retinal vein and artery occlusion.

METHODS

Case report. A 22-year-old woman presented with sudden-onset blurry vision in the left eye of 3-day duration.

RESULTS

Best-corrected visual acuity was 20/20 and 20/1000 in right and left eyes, respectively. Fundus examination of the left eye revealed optic disk edema, macular whitening with a cherry-red spot, markedly dilated and tortuous retinal veins, and hemorrhages both around the disk and extending into the macula and the periphery. Fundus fluorescein angiography showed delayed filling of retinal vasculature, dilated and tortuous retinal veins, and blocked fluorescence around and beyond the optic disk. Optical coherence tomography scan at presentation showed hyperreflective inner retinal layers with neurosensory detachment. Optical coherence tomography angiography showed that the vessel densities of superficial and deep capillary plexus were remarkably reduced. A diagnosis of β-thalassemia trait combined with iron deficiency anemia was made after hematologic workup. The patient was treated with a course of oral iron supplements, vasodilator (compound Xueshuantong), inhalation of a mixture of 5% carbon dioxide and 95% oxygen, and a nutritional agent (compound anisoine). Six months later, her visual acuity improved to 20/60 in the left eye with complete resolution of all clinical signs.

CONCLUSION

Combined central retinal vein and artery occlusion is a rare emergency leading to acute vision loss and can manifest in patients with β-thalassemia trait with iron deficiency anemia. Prompt diagnosis and early management is important to treat underlying systemic disorders and to prevent occurrence of a similar episode in fellow eye.

摘要

目的

报告 1 例β-地中海贫血伴缺铁性贫血表现为视网膜中央静脉和动脉阻塞的病例。

方法

病例报告。1 例 22 岁女性,左眼突发 3 天视力模糊。

结果

最佳矫正视力右眼为 20/20,左眼为 20/1000。左眼眼底检查发现视盘水肿,黄斑区变白伴樱桃红斑,视网膜静脉明显扩张迂曲,视盘周围及延伸至黄斑区和周边可见出血。眼底荧光血管造影显示视网膜血管充盈延迟,视网膜静脉扩张迂曲,视盘周围及以外区域荧光遮蔽。就诊时的光学相干断层扫描显示内视网膜层高反射,伴有神经感觉脱离。光学相干断层血管造影显示浅层和深层毛细血管丛的血管密度显著降低。经过血液学检查后,诊断为β-地中海贫血伴缺铁性贫血。患者接受了口服铁补充剂、血管扩张剂(复方血栓通)、5%二氧化碳和 95%氧气混合物吸入和营养剂(复方茴香草素)治疗。6 个月后,左眼视力提高至 20/60,所有临床体征完全消退。

结论

视网膜中央静脉和动脉阻塞是一种罕见的急症,可导致急性视力丧失,并可发生在β-地中海贫血伴缺铁性贫血的患者中。及时诊断和早期治疗对于治疗潜在的系统性疾病和预防对侧眼发生类似事件非常重要。

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