Central retinal artery occlusion occurring 30 years after successful revascularization surgery for moyamoya disease:...

We report a case of central retinal artery occlusion (CRAO) leading to unilateral blindness occurring in a moyamoya patient 30 years after successful pial synangiosis when she was 6 years old. Imaging studies at the time of the CRAO revealed total occlusion of the ipsilateral cervical and intracranial internal carotid artery, a vessel shown to be patent on MRI/MRA studies for decades previously. This case demonstrates that long-term follow-up of operated moyamoya patients may reveal late secondary complications, of which physicians, patients, and families need to be aware.