Retinal arterial wall plaques in Susac syndrome.

PURPOSE

To demonstrate retinal arterial wall plaques (RAWPs) in patients with Susac syndrome, a disorder that consists of the triad of branch retinal artery occlusion, encephalopathy, and hearing loss. The clinician may misinterpret these RAWPs as emboli.

DESIGN

Observational case series.

METHODS

Four patients (one man and three women, aged 21-38 years) were examined and followed.

RESULTS

Four patients with Susac syndrome had RAWPs in association with branch retinal artery occlusions. They were present in six of eight eyes. The plaques were yellow to yellow-white and located usually away from retinal bifurcations. Retinal arterial wall plaques occur at the midarteriolar segments, whereas Hollenhorst plaques occur at vascular bifurcations. Four of six eyes had resolution of the plaques.

CONCLUSIONS

Retinal arterial wall plaques may occur with branch retinal artery occlusions in Susac syndrome. This finding should help the clinician who is struggling to make a diagnosis in a patient with an enigmatic encephalopathy and/or hearing loss. These plaques may resolve over time.