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基因型为ss的血红蛋白病患者双侧同时发生黄斑梗死并自发视力恢复

Bilateral simultaneous macular infarction with spontaneous visual recovery in genotype ss hemoglobinopathy patient.

作者信息

Okonkwo O N, Hassan A O, Gyasi M E, Oderinlo O

机构信息

Eye Foundation Center for Prevention of Blindness, Eye Foundation Retina Institute, Lagos, Nigeria.

Department of Ophthalmology, St. Thomas Eye Hospital, Accra, Ghana.

出版信息

Niger J Clin Pract. 2017 Dec;20(12):1651-1655. doi: 10.4103/njcp.njcp_95_17.

Abstract

To report the rare and dramatic event of bilateral macular infarction in a sickle cell hemoglobinopathy (SS genotype) patient, resulting in bilateral severe reduction in visual acuity. Without any intervention, the patient's vision gradually improved over the follow-up period. Central visual field defects however persisted. A 21-year-old male Nigerian, presented with a 1-week history of bilateral sudden painless loss of vision. His symptom was associated with fever, feeling of heaviness in the chest and head, and a dizzy spell. Visual acuity was reduced to 20/200 in both eyes and near acuity was; right eye: N24, left eye: N36. Funduscopy showed a pale, milky white, thickened retinal patch superotemporal to the fovea in both eyes. Fluorescein Angiograph: revealed features consistent with occlusion of the parafoveal terminal arterioles in both eyes. Although he did not receive any ocular treatment, and exchange blood transfusion was not done, he regained near-normal visual acuity in both eyes over a 17-month follow-up period, central visual field defects persisted in both eyes. Visual recovery in this patient demonstrates that macular function could improve over time following macular ischemia, without any treatment. Patients and caring physicians should be aware of this possibility.

摘要

报告一例镰状细胞血红蛋白病(SS基因型)患者发生双侧黄斑梗死这一罕见且严重的事件,导致双眼视力严重下降。未经任何干预,患者视力在随访期间逐渐改善。然而,中心视野缺损持续存在。一名21岁的尼日利亚男性,出现双侧突然无痛性视力丧失1周病史。其症状伴有发热、胸部和头部沉重感以及一阵头晕。双眼视力降至20/200,近视力为:右眼:N24,左眼:N36。眼底检查显示双眼黄斑颞上侧有一个苍白、乳白色、增厚的视网膜斑。荧光素血管造影显示双眼黄斑旁终末小动脉阻塞的特征。尽管他未接受任何眼部治疗,也未进行换血输血,但在17个月的随访期间,他双眼视力恢复至接近正常,双眼中心视野缺损持续存在。该患者的视力恢复表明,黄斑缺血后,黄斑功能可随时间改善,无需任何治疗。患者及护理医生应意识到这种可能性。

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