Anatomical and functional correlation in Susac syndrome: multimodal imaging assessment

BACKGROUND: Susac’s syndrome (SuS) is an uncommon disease characterized by retinal microangiopathy that may be assessed more accurately with optical coherence tomography angiography (OCTA), a new imaging technique which provides a retinal microvasculature map. The purpose of this case report is to describe the multimodal imaging findings of SuS correlating OCTA with functional tests. CASE PRESENTATION: Retrospective review of one case with clinical and imaging evidence of SuS. Color fundus photograph, fluorescein angiography (FA), OCTA, microperimetry (MP) and visual field (VF) tests were analyzed at the time of presentation and at 1- and 6-month visit following initiation of treatment. The study patient underwent standard treatment for SuS. The patient age was 31 year-old and the baseline visual acuity was 20/60 and 20/20 in the right and left eyes, respectively. At presentation, FA showed branch retinal arterial occlusion within the macular area of the right eye and vascular leakage in the periphery of the left eye. OCTA demonstrated areas of superficial and deep retinal vascular plexuses hypoperfusion in both eyes. The OCTA segmentations in the outer retina and choriocapillaris were normal. The low VF and MP sensitivity signals precisely corresponded to the topography of decreased vascular perfusion seen on the OCTA density map in both eyes. Six months after specific SuS therapy, retinal vascular perfusion showed partial improvement in both eyes. CONCLUSION: OCTA may demonstrate superficial and deep retinal vascular non-perfusion without choriocapillary vasculature changes in SuS. This anatomical information given by OCTA corresponded to points of low sensitivity on functional tests represented by VF and MP.