A Novel Quantitative Assessment Method of Disease Activity in Susac's Syndrome Based on Ultra-Wide Field Imaging.

PURPOSE: Susac's syndrome (ScS) is a rare, potentially life-threatening auto-immune disease. Ophthalmic imaging can depict characteristic branch retinal arteriolar occlusions (BRAO) and arterial wall hyperfluorescences that form one of the three diagnostic pillars of this condition. We aim to demonstrate that ophthalmological ultrawide-field (UWF) imaging allows for a qualitative and quantitative assessment in ScS, with application in diagnostics, monitoring of treatment response, and titration of therapy. METHODS: In seven ScS patients (♀:♂ = 2:5), with a median age of 36 years, range 18 to 57 years, serial ultrawide-field fluoresceine angiography (UWF-FA) studies were performed, with adjunctive wide-field optical coherence tomography angiography analyses (WF-OCTA) in five patients. Mean follow-up was 12.5 months, range 1 to 46 months. RESULTS: In all seven patients, BRAO and arterial wall hyperfluorescences were present in UWF-FA and calculated as a quantitative score of disease activity in fluorescein angiography (DA-FA) during follow-up visits. Treatment response was accessible in follow-up imaging as partial reperfusion of retinal vessels, resolution of arterial wall hyperfluorescences and consequently, in reduction of DA-FA score. While qualitative analysis of WF-OCTA provided further information about retinal micro-perfusion, quantitative analysis did not demonstrate a vectored treatment response as it was accessible in FA. CONCLUSION: DA-FA score, as a comprehensive disease activity parameter in ScS has potential to facilitate optimal communication between subspecialties and thereby treatment success.

Assessment of the microvasculature in poppers maculopathy.

PURPOSE: To investigate a possible microvascular component of poppers maculopathy (PMP) using optical coherence tomography angiography (OCTA). METHODS: Twelve patients suffering from poppers maculopathy were included. Health records, optical coherence tomography (OCT), and OCTA data was gathered and compared to a healthy control group (HC). PMP lesion type was determined by manifestation in OCT. OCTA-based evaluation of retinal vascular plexus and choriocapillaris (CC) was executed. Vessel density (VD) and vessel length density (VLD) in superficial and deep capillary plexus (SCP, DCP), as well as flow deficits (FD), within the foveal avascular zone (FAZ) in CC were assessed. RESULTS: Median age of PMP patients was 40 (min 24; max 64) years, all male. Eleven patients presented with ellipsoid zone-type lesions; one patient showed a vitelliform-type lesion. No qualitative microvascular changes between PMP patients and HC were identified. Quantitative values for VD and VLD of SCP and DCP did not differ in between the two groups. The analysis of FDs in CC showed no deviation from PMP patients to HC. CONCLUSIONS: No vascular anomalies in qualitative and quantitative analysis in OCTA were detected in PMP patients. The constitution of the CC within FAZ of PMP patients does not differ from HC when assessed as FD.