Monika J Turczyńska1, Przemysław Krajewski1, Joanna E Brydak-Godowska1,2. 1. Department of Ophthalmology, Infant Jesus Teaching Hospital, University Clinical Center of the Medical University of Warsaw, Warszawa, Poland; and. 2. Department of Ophthalmology, Medical University of Warsaw, Warszawa, Poland.
Abstract
PURPOSE: 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. METHODS: 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. RESULTS: 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%. CONCLUSION: 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.
PURPOSE: 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. METHODS: 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. RESULTS: 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%. CONCLUSION: 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.