Our patient presented with silent ischemia inferotemporally in the right eye and new lesions superotemporally in the left. Note the areas of non-perfusion and leakage.
18 days later new multi-focal lesions OS are visualized dramatically on fluorescein angiography.
Fortunately, the patient has maintained excellent VA
Learning Points:
Retinocochleocerebral Vasculopathy, more commonly known as Susac Syndrome, is a rare, microangiopathic disease of debated pathophysiology characterized by the classic triad of encephalopathy, sensorineural hearing loss and branch retinal artery occlusions. The full triad may take months to evolve, frustrating the diagnosis.
Retinal arterial wall atheromatous plaques, also known as Gass plaques, are seen often in later stages. They are a result of the occlusion, not the cause.
The development of wide-field angiography has occasionally allowed for early detection which can be critical in order to minimize the often long-term, debilitating effects of the disease.
