A review and update on the ophthalmic implications of Susac syndrome

Abstract

Susac syndrome is a rare condition presumed to be immune-mediated occlusion of small arterial vasculature principally of the brain, inner ear, and retina. Clinically the syndrome manifests as a pathognomonic triad of encephalopathy, hearing loss, and branch retinal artery occlusion. Early recognition and diagnosis is important as delayed treatment may be profound and result in deafness, blindness, dementia and other neurological deficits. The plethora of imaging technology, including magnetic resonance imaging, retinal fluorescein angiography, optical coherence tomography (OCT) and OCT-angiography allows deeper and more discrete anatomical-physiological correlation of underlying pathology, early diagnosis, and imaging biomarkers for early detection of relapse during follow up. We highlight the current clinical classification of Susac syndrome, available investigations, treatment and care pathways.

Multimodal Imaging in Susac Syndrome: A Case Report and Literature Review

“…OCTA may be an effective alternative to the standard FA as it consents to the microvascular analysis of both superficial and deep capillary plexi and to monitoring of the vascular density changes without dye injection [24]. However, additional prospective studies are necessary to establish the role of OCTA, widefield color fundus photography, and AF to monitor disease activity and to determine the efficacy of new therapeutic approaches [25].…”

Susac syndrome as a multidisciplinary diagnostic challenge

“…In FA, BRAO is observed, which in the acute form of the disease may occur with pseudoemboli known as Gass plaques, present in the central segments of the retinal arterioles, accompanied by characteristic vascular staining and fluorescein leakage at sites remote from the actual BRAO. The changes are usually bilateral, may appear early or at a later stage of the disease, and in many cases enable the final diagnosis to be made [12].…”

Scoping review of cochlear implantation in Susac's syndrome

“…Branch retinal artery occlusion is seen on fluorescein angiography, and Gass plaques, which are yellow refractile lesions that simulate emboli along retinal arteries, may be seen on fundoscopy. 8 , 12 …”