Ocular findings revealing a Susac syndrome: a retrospective review of 4 cases
Purpose
Our objective is to describe ocular findings and associated extraocular manifestations in Susac syndrome.
Methods
The charts of four patients (8 eyes) diagnosed with SUSAC syndrome were retrospectively reviewed.
Results
Mean age of our patients was 32 years (2 males and 2 females). The mean best corrected visual acuity was 20/32 (range, 20/63-20/20). Ocular findings at presentation were as follows: multiple branch retinal artery occlusions in 8 eyes, Gass plaques in 5 eyes, and segmental arteriolar wall hyperfluorescence on fluorescein angiography in 7 eyes. Ocular involvement was asymptomatic in 2 eyes, and subclinical in 3 eyes. Results of brain magnetic resonance imaging were abnormal in 3 patients. The audiogram showed sensorineural hearing loss in 2 patients. Treatment modalities included systemic corticosteroids (4 patients ; 100%), and cyclophosphamide (1 patient ; 25%).
Conclusion
Susac syndrome is a rare cause of occlusive retinal arteritis. Multiple branch retinal artery occlusions, Gass plaques and arteriolar wall hyperfluorescence on fluorescein angiography are highly suggestive of Susac syndrome. Association with encephalopathy and/or sensorineural hearing loss confirm the diagnosis.
Conflict of interest
No
Author 1
Last name
KHOCHTALI
Initials of first name(s)
S
Department
Department of Ophthalmology, Fattouma Bourguiba University Hospital
City
Monastir
Country
Tunisia
Author 2
Last name
Khairallah
Initials of first name(s)
M
Department
Susac syndrome is a rare cause of occlusive retinal arteritis. Multiple branch retinal artery occlus
City
Monastir
Country
Tunisia
Author 3
Last name
Smaoui
Initials of first name(s)
S
Department
Department of Ophthalmology, Fattouma Bourguiba University Hospital
City
Monastir
Country
Tunisia
Author 4
Last name
Ben Aoun
Initials of first name(s)
S
City
Monastir
Country
Tunisia
Author 5
Last name
Ben Yahia
Initials of first name(s)
S
Department
Department of Ophthalmology, Fattouma Bourguiba University Hospital
City
Monastir
Country
Tunisia
Author 6
Last name
Khairallah
Initials of first name(s)
M
Department
Department of Ophthalmology, Fattouma Bourguiba University Hospital
City
Monastir
Country
Tunisia
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