Nur Acar*, Ziya Kapran, Yaprak Banu Unver, Leyla Hazar (Ankara, Turkey)


Susac Syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusion, and hearing loss. It is an autoimmune endotheliopathy that primarily affects young women. Although it is a rare syndrome, it should remember in the differential diagnosis of visual impairment with accompanying neurological and auditory problems in young patients.


A 36-year old male presenting to a neurologist with a right leg paresis developed dysartria, urinary incontinence, transient diplopia and memory loss. He had a detailed neurological examination with a detailed imaging, and an ophthalmological examination with a fundus angiography, in which retinal vessel wall hyperfluorescence, and neovascularizations were observed. He had an audiogram showing deficits and was finally diagnosed as Susac Syndrome.The same patient was referred to our clinic with severe visual loss after 4 years having vitreous hemorrhage (VH) with a localized traction retinal detachment (TRD) in RE (VA: 20/200), and a combined TRD and rhegmatogenous retinal detachment (RRD) involving the macula in LE (VA: 20/320).

Effectiveness / Safety:

RE was treated with panretinal argon laser photocoagulation (PC), and remains stable. His LE underwent phacoemulsification with implantation of an intraocular lens into the capsular bag and 23-gauge bimanual pars plana vitrectomy (PPV) with membrane peeling and silicone oil tamponade. Three months later SiO removal with epiretinal membrane (ERM) peeling was performed with revitrectomy in the LE. At his last follow-up of 16months VAs was 20/100 in RE, and 20/80 in LE, with retinas attached.

Take home message:

To the best of our knowledge this case represents the first one with Susac Syndrome developing VH, TRD, and RRD in the literature. Severe ocular complications have developed due to ischemia with time. This syndrome should be kept in mind in differential diagnosis of vascular diseases especially in young patients. Timely management of ocular complications enables visual recovery.