Department of Ophthalmology, Royal Victorian Eye and Ear Hospital, 32 Gisborne St, East Melbourne, VIC, Australia.
Retin Cases Brief Rep. 2024 May 1;18(3):290-292. doi: 10.1097/ICB.0000000000001383.
To present a case of paracentral acute middle maculopathy (PAMM) associated with transient loss of vision from eosinophilic granulomatosis with polyangiitis (EGPA)-associated ischemic vasculitis.
Case report and review of the literature.
A 52-year-old man presented with recurrent episodes of amaurosis of the right eye. A diagnosis of EGPA was established with the combination of late-onset asthma, peripheral eosinophilia, patchy parenchymal consolidation, and diffuse pansinusitis. Fundus examination displayed cotton wool spots in the right eye. Optical coherence tomography (OCT) of the macula showed features of paracentral focal inner nuclear layer/outer plexiform layer hyperreflectivity consistent with PAMM. He was treated with corticosteroids and cyclophosphamide. At 2 months following the treatment, his EGPA was in remission; the patient reported no recurrence of visual symptoms. Dilated fundoscopy revealed resolution of the cotton wool spots. A repeat OCT of the macula showed resolution of PAMM.
This case supports sublethal hypoxia of the middle retinal layers as the underlying mechanism for PAMM. Ophthalmologists should be cognizant to look for PAMM on OCT in patients presenting with transient visual loss and be aware of EGPA-associated ischemic vasculitis as a rare cause of amaurosis.
报告一例与嗜酸性肉芽肿性多血管炎（EGPA）相关缺血性血管炎相关的中央旁急性中黄斑病变（PAMM）相关的一过性视力丧失病例。
病例报告和文献复习。
一名 52 岁男性出现右眼反复发作性失明。结合迟发性哮喘、外周血嗜酸性粒细胞增多、斑片状实质实变和弥漫性全组副鼻窦炎，诊断为 EGPA。右眼眼底检查显示棉絮斑。黄斑 OCT 显示符合 PAMM 的特征性中央旁局灶性内核层/外丛状层高反射性。他接受了皮质类固醇和环磷酰胺治疗。治疗后 2 个月，EGPA 缓解；患者报告无视觉症状复发。散瞳眼底检查显示棉絮斑消失。黄斑 OCT 复查显示 PAMM 消退。
该病例支持中层视网膜亚致死性缺氧是 PAMM 的潜在机制。眼科医生应该意识到在出现短暂性视力丧失的患者中，OCT 上可能出现 PAMM，并认识到 EGPA 相关缺血性血管炎是导致失明的罕见原因。
