灾难性抗脂综合征患者的冠状腺缺血症:一个案例系列
Melvin Gerardy1, Alain Gaudric2, Pierre Duraffour1
1Department of Ophthalmology, University of Paris Cité, OphtalmoPôle, Cochin Hospital, Assistance Publique-Hôpitaux de Paris, France.
Retinal cases & brief reports
|June 30, 2025
概括
患有灾难性抗脂综合征 (CAPS) 的孕妇急性胆管缺血症呈现出特定的视网膜发现. 早期识别和及时治疗导致完全康复,强调了眼科评估在疑似CAPS病例的重要性.
科学领域:
- 眼科医生 眼科 眼科
- 类风湿病学 类风湿病学
- 产科 产科 产科 产科 产科
背景情况:
- 灾难性抗脂综合征 (CAPS) 是一种罕见的,危及生命的疾病,其特征是广泛的血栓事件.
- 抗脂综合征的眼部表现可能是多样化的,但急性胆道缺血不常见.
研究的目的:
- 在被诊断患有CAPS的孕妇患者中描述三例急性胆道缺血症.
- 要突出与CAPS相关的胆管缺血症相关的特征性视网膜成像发现.
主要方法:
- 对CAPS患者的医疗和成像记录的回顾性审查.
- 视网膜成像包括 fundus 摄影,光谱域光学连贯性断层扫描 (SD-OCT),光素血管造影 (FA) 和绿色血管造影 (ICGA).
主要成果:
- 三名患有CAPS的孕妇呈现后眼部卷入,影响了六只眼睛.
- SD-OCT显示了多个亚皮脱落.
- FA和ICGA揭示了三角形非透区域 (阿马里克三角胆道梗塞) 和子内泄漏.
结论:
- 抗脂综合征中的急性胆道缺血需要怀疑CAPS,这是一个危急的情况.
- 在所有报告的病例中,迅速转诊专家和重症监护管理 (皮质类固醇,抗凝剂,血交换/IVIg) 导致完全康复.
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