Fluorescein and indocyanine green angiographies in Susac syndrome

Abstract

Purpose: Susac syndrome is a rare microangiopathy of the brain, retina, and cochlea that mainly affects young women. We studied the management of this disease using retinal fluorescein and indocyanine green angiographies.

Methods: Retrospective, observational case series of seven patients with Susac syndrome identified in ophthalmology and internal medicine departments. We reviewed medical, ophthalmologic, and angiographic records at study enrollment and during long-term treatment. Mean follow-up was 37 months. Best-corrected visual acuity, intraocular inflammation score, ophthalmoscopy data, automated perimetry score, and fluorescein and indocyanine green angiographic features were analyzed with the results of cerebral magnetic resonance imaging and spinal fluid analysis and ENT signs.

Results: Retinal fluorescein angiography showed focal nonperfused retinal arterioles with hyperfluorescent walls in all cases. Indocyanine green angiography showed normal choroidal circulation. Retinal vasculitis was uncontrolled in a patient treated with steroids. Improvement of retinal arteriole perfusion occurred during immunosuppressive treatment with cyclophosphamide.

Conclusion: Absence of intraocular inflammation and focal, labile nonperfused retinal arterioles with integrity of choroidal circulation are monomorphic ophthalmologic features that could immediately suggest the diagnosis of Susac syndrome. Early ophthalmologic examination aided by retinal fluorescein angiography could be useful for managing cases of unexplained neurologic symptoms in women.