Department of Ophthalmology, Infant Jesus Teaching Hospital, University Clinical Center of the Medical University of Warsaw, Warszawa, Poland; and.
Department of Ophthalmology, Medical University of Warsaw, Warszawa, Poland.
Retina. 2021 Jul 1;41(7):1553-1561. doi: 10.1097/IAE.0000000000003051.
To present the clinical manifestations and results of key diagnostic investigations in patients with Susac syndrome, with special emphasis on the principal role of fluorescein angiography (FA) of the peripheral retina.
A retrospective analysis of medical records (collected 2000-2019) of 20 patients (15 women and five men), aged 20 to 51 years (mean age: 31.6 years) with complete or incomplete Susac syndrome diagnosed by an ophthalmic examination and widefield fluorescein angiography (WF-FA) by Spectralis and Optos Tx200.
Fluorescein angiography abnormalities included vascular changes in the posterior pole in 64.7% and in the peripheral retina in 82.4%. Widefield FA abnormalities in the peripheral retina alone were seen in 35.3% and in the posterior pole, but without peripheral vascular involvement, in 17.6%. Secondary leakage from veins was noted in 58.8%.
Widefield FA of the peripheral retina has a key role in cases of suspected Susac syndrome as it confirms the diagnosis and assesses disease activity. In addition to the characteristic findings, late-phase FA revealed leakage from veins which is not a typical sign. Patients experiencing migraine headaches may benefit from increasing awareness of neurologists and otologists who more promptly referred patients with suspected Susac syndrome for ophthalmologic evaluation and WF-FA of the peripheral retina.
介绍 Susac 综合征患者的临床表现和主要诊断检查结果，特别强调周边视网膜荧光素血管造影（FA）的主要作用。
对 20 名（15 名女性和 5 名男性）年龄在 20 至 51 岁（平均年龄：31.6 岁）的患者的病历进行回顾性分析（收集于 2000 年至 2019 年），这些患者通过眼科检查和 Spectralis 和 Optos Tx200 进行的广角荧光素血管造影（WF-FA）确诊为完全或不完全 Susac 综合征。
荧光素血管造影异常包括后极血管改变占 64.7%，周边视网膜血管改变占 82.4%。单纯周边视网膜广角 FA 异常占 35.3%，后极无周边血管受累占 17.6%。静脉继发性渗漏占 58.8%。
疑似 Susac 综合征患者的广角周边视网膜 FA 具有关键作用，因为它可以确认诊断并评估疾病活动度。除了特征性发现外，晚期 FA 还显示出静脉渗漏，这不是典型的迹象。患有偏头痛的患者可能会受益于提高神经科医生和耳科医生的认识，他们更及时地将疑似 Susac 综合征的患者转诊进行眼科评估和广角周边视网膜 WF-FA。
