ULTRA-WIDE-FIELD FUNDUS IMAGING IN THE DIAGNOSIS AND FOLLOW-UP OF SUSAC SYNDROME.

PURPOSE: To present the use of ultra-wide-field (UW) fundus imaging in the diagnosis and follow-up of a patient with Susac syndrome.

METHODS: Case report of a myopic patient presenting initially with rhegmatogenous retinal detachment. A significant portion of the retina was found to be avascular bilaterally at presentation. Surgery was performed with scleral buckle. Then, UW color and autofluorescent imaging and UW fluorescein angiography were obtained.

RESULTS: Successful retinal reattachment was obtained. Enlargement of the avascular area with neovascularization was observed at eight-month follow-up. In addition, the patient presented severe neurosensory hearing loss and clinical depression postoperatively. The results of UW fluorescein angiography revealed hyperfluorescent macular spots, arteriolar wall hyperfluorescence, leakage from retinal neovascularization, and confirmed the avascularity of two thirds of the retina, whereas the results of UW autofluorescence showed absence of the normal hypofluorescent retinal vessels outside the posterior pole. Findings of UW imaging in combination with systemic involvement led to the diagnosis of Susac syndrome. Appropriate treatment stopped the disease progress, ameliorated symptoms, and some of the occluded retinal vessels were reperfused.

CONCLUSION: In conclusion, UW fundus imaging is a valuable modality in the diagnosis and follow-up of patients with Susac syndrome. Early diagnosis and treatment is critical, particularly as it can lead to reperfusion of occluded retinal vessels.