Abstract
AbstractPurpose Susac's syndrome (SS) is a rare immune disorder. It combines encephalopathy, retinopathy and hearing loss. We report a new pediatric case, revealed by an occlusive retinal arteritis.Methods A 12‐year old girl came to emergency room due to sudden onset of photopsia in her right temporal visual field. One year before, she had developed an acute disseminated encephalomyelitis, resolved after steroids. Visual acuity was 10/10 P2. Fundus examination revealed a thin right inferior nasal artery associated with an ischemic oedema. Fluorescein angiography evidenced a right nasal artery occlusion and multiple peripheral arterial occlusive vasculitis. Steroid treatment was dispended. Brain MRI showed high signal‐intensity abnormalities in corpus callosum. The triad was completed by a bilateral hearing loss. Intravenous immunoglobulins were initiated. Occurrence of contralateral occlusive retinal arteritis two months later led to introduce cyclophosphamide. After 6 infusions, visual acuity is stable but visual field defects are still present and regression of vasculitis remains incomplete.Results 300 cases of SS have been reported with predominance in young women. Pediatric cases are extremely rare. Diagnosis is difficult, because the typical clinical triad is often incomplete. Occurrence of relapses is unpredictable. Visual prognosis depends on localisation of occluded retinal territories. Treatment is based on a combination of steroids, immunosuppressive and antiplatelet agents, which is not always able to prevent the relapse as in our young patient.Conclusion Unpredictable evolution of SS requires an early diagnosis and treatment as well as a close monitoring. It must always be considered in case of occlusive retinal arteritis even in children.
Published Version
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