Pherwani Anupama, Bansal Shveta, Agrawal Shailesh, Gillow Timothy
St James' University Hospital, Leeds, UK.
Cases J. 2008 Nov 20;1(1):339. doi: 10.1186/1757-1626-1-339.
Cogan's Syndrome is typically characterised by a non syphilitic interstitial keratitis (IK), with or without conjunctivitis, iritis or subconjunctival bleeding and progressive sensorineural hearing loss within two years of ocular signs. Atypical ocular manifestations include episcleritis, scleritis, posterior scleritis, retinal artery occlusion, choroiditis, retinal vasculitis, and optic disc oedema. We report a case of Cogan's syndrome in with recurrent cystoid macular oedema was the main feature.
A patient was diagnosed with Cogan's syndrome nearly 2 years after first presentation. He had cystoid macular edema which failed to respond initially to steroid, methotrexate and azothiaprine however resolved after treatment with mycophenolate mofetil.
Cogan's syndrome is rare and presents a challenge in terms of diagnosis and treatment. Recurrent cystoid macular oedema is a unique finding in this condition and can be difficult to control. Multidisciplinary management of this multisystem disorder is vital.
科根综合征的典型特征是一种非梅毒性间质性角膜炎（IK），伴有或不伴有结膜炎、虹膜炎或结膜下出血，且在眼部症状出现后的两年内出现进行性感音神经性听力损失。非典型眼部表现包括巩膜外层炎、巩膜炎、后巩膜炎、视网膜动脉阻塞、脉络膜炎、视网膜血管炎和视盘水肿。我们报告一例以复发性黄斑囊样水肿为主要特征的科根综合征病例。
一名患者首次就诊近2年后被诊断为科根综合征。他患有黄斑囊样水肿，最初对类固醇、甲氨蝶呤和硫唑嘌呤治疗无反应，但在用霉酚酸酯治疗后消退。
科根综合征罕见，在诊断和治疗方面具有挑战性。复发性黄斑囊样水肿是这种疾病的一个独特表现，可能难以控制。对这种多系统疾病进行多学科管理至关重要。
