RECURRENT BRANCH RETINAL ARTERY OCCLUSION FROM SUSAC SYNDROME: CASE REPORT AND REVIEW OF LITERATURE.

PURPOSE: We report a patient with branch retinal artery occlusion predominant Susac syndrome and review the literature on current diagnostic modalities and therapeutic approaches for this rare condition.

METHODS

Case report.

RESULTS

A 23-year-old white male presented with a 5-month history of a scotoma in his right eye vision, headaches, and intermittent paresthesias of the lower extremities. Ophthalmic examination was normal with the exception of a hypopigmented small retinal area inferonasal to the fovea in the right eye. Optical coherence tomography demonstrated inner retinal atrophy and optical coherence tomography angiography showed deep capillary layer vessel dropout in both eyes. Ultra-wide-field fluorescein angiography revealed bilateral arteriolar wall hyperfluorescence. Corpus callosal lesions were present on magnetic resonance imaging. Lumbar puncture demonstrated elevated protein. Audiogram was normal. A diagnosis of Susac syndrome was made. The patient initially received oral steroids followed by intravenous steroids and mycophenolate mofetil because of new branch retinal artery occlusions. Thirteen months after initial presentation, the vision was stable at 20/20 in both eyes.

CONCLUSION

It is a diagnostic challenge when recurrent branch retinal artery occlusion secondary to Susac syndrome presents without the classic symptoms. Optical coherence tomography angiography and subtle magnetic resonance imaging findings aided in early recognition of the diagnosis in our patient. Ultra-wide-field fluorescein angiography was an important tool in disease monitoring. Aggressive management with intravenous corticosteroids and initiation of immunosuppression resulted in long-term preservation of vision and improvement in visual field.