Reperfusion of Retinal Arteriolar Occlusion After Initiation of Immunosuppressive Therapy in Pediatric Susac Syndrome.

PURPOSE: Susac syndrome is a rare autoimmune microangiopathy that affects the precapillary arterioles of the brain, retina, and inner ear. It is typically characterized by a clinical triad of encephalopathy, branch retinal arteriolar occlusions (BRAOs), and sensorineural hearing loss. At present, reports of reperfusion of occluded retinal arterioles are scarcely reported in the literature.

METHODS

Two case reports.

RESULTS

Two pediatric patients presented with sudden visual field defects caused by multiple retinal arteriolar occlusions. Following appropriate immunosuppressive therapy, complete reperfusion of the occluded retinal arterioles was achieved.

CONCLUSION

These cases highlight the importance of the early recognition of Susac syndrome in children presenting with BRAOs, even in the absence of the complete triad. Retinal vascular occlusion in this condition may be reversible with timely and aggressive immunosuppression, underscoring the need for prompt diagnosis and consideration of biological agents for refractory pediatric diseases.