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Nathaniel Sears, Sunil K Srivastava, Kimberly Baynes, Careen Y Lowder, Justis Ehlers, Robert Rennebohm; Interval changes in patients with Susac’s syndrome on wide-field fluorescein angiography. Invest. Ophthalmol. Vis. Sci. 2014;55(13):3834.
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Susac syndrome is a rare condition identified by the clinical triad of encephalopathy with varying focal neurological symptoms, recurrent branch retinal artery occlusions (BRAO) and vestibuloauditory symptoms. Wide field fluorescein angiography (WFA) allows for visualization of retinal vascular perfusion from the optic disc to the periphery. We have previously identified novel changes in the angiographic features of the retinal periphery in Susac’s syndrome. The purpose of this study is to assess whether these changes reverse with treatment.
This is a retrospective, observational, consecutive case series of patients seen at the Cleveland Clinic, Cole Eye Institute between September, 2012 and November, 2013. The diagnosis of Susac’s syndrome was confirmed by comprehensive medical and ophthalmic evaluation. WFA was administered and data from WFA was divided into macular and peripheral findings over time, which included presence of BRAO, vessel staining or leakage, neovascularization and non-perfusion.
Seven patients were identified with Susac syndrome with interval studies (14 eyes). Systemic treatment was initiated in all patients and included prednisone, cellcept and IVIG. On initial presentation macular BRAO was identified in 14% (2/14) versus peripheral BRAO in 71% (10/14) of patients. Peripheral BRAO reversed with treatment by 29% to 43%. Also on presentation, macular capillary dropout was identified in 14% (2/14) versus 100% (14/14) of peripheral retinae. With treatment there were only a few patients who had improvement of peripheral non-perfusion. Macular vessel staining and leakage was found in 0% and 14% (2/14), respectively, as compared to peripheral staining and leakage in 50% (7/14) and 64% (9/14) of patients. These changes did not improve with therapy, indicating that these changes may represent vessel injury as opposed to active inflammation. Neovascularization was rare, but did not change with therapy.
WFA is a useful adjunct to the diagnosis and management of Susac’s syndrome. WFA reveals peripheral angiographic abnormalities previously not noted. In this study, the changes noted on WFA did not improve with aggressive therapy. BRAOs did improve, but capillary non-perfusion and vessel leakage did not reverse.
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