Small infarctions of cochlear, retinal and encephalic tissue (SICRET syndrome)

To report 3 new cases suggestive of SICRET syndrome.

Case reports. Three patients underwent clinical, laboratory and neuroradiological examination for recurrent retinal branch artery occlusions, encephalopathy and/or hearing loss.

In all 3 patients the laboratory tests were unremarkable for infectious or systemic disease. All 3 cases showed retinal artery occlusions on funduscopy and fluorescein angiography. Visual acuity impairment and visual field defects were related to the retinal artery occlusion site. Variations in the presenting sign were noted in the 3 patients. The first patient (a 32 year old man) showed a documented association of small infarctions of the brain and the retina. The retinal occlusions were bilateral and 8 recurrences were observed. In the second case (a 26 year old woman) the brain was initially involved. Fluorescein angiography showed unilateral retinal artery occlusion. Cochlear manifestations were not reported. The third case (a 35 year old woman) showed initially bilateral recurrent branch artery occlusion and hearing loss without clinical signs of encephalopathy. Three recurrences were documented.

SICRET syndrome is a microangiopathy that causes infarcts in the cochlea, retina, and brain of young patients, usually women. The aetiology is unknown. Clinically some infarcts may remain asymptomatic. Bilateral sensorineural hearing loss on low frequency on audiology, recurrent bilateral retinal branch artery occlusions on fluorescein angiography and small multiple areas of signal hyperintensity in white and gray matter on Brain MRI T2-weighted images are necessary for the diagnosis. Our 3 patients did not fulfill these criteria, but their disease was suggestive of SICRET syndrome. SICRET syndrome should be included in the differential diagnosis of recurrent branch artery occlusions. The clinical course is self-limited and treatment options are not codified.