Purpose

To report clinical and imaging features in 4monocular patients presenting with a rare variant of histologically proven sympathetic ophthalmia(SO).

Methods

Retrospective description of clinical and Multimodal findings of 4patients with atypical SO. Enucleation of inciting non seeing eye established histopathological diagnosis. Extensive laboratory work up ruled out other possible differentials. All received treatment with systemic corticosteroids and immunosuppressive therapy/biologicals with a minimum 3month follow up available for all

Results

The mean age of patients was 39±13.1years. Fellow eye of 3had history of trauma while 1 underwent multiple surgeries.Presenting visual acuity in sympathizing eye ranged from HMCF to finger counting. All eyes had anterior chamber inflammation and diffuse vitritis. Fundus showed peripapillary fibrosis and multiple peripheral patches of choroiditis. SS OCT showed subretinal uniformly hyperreflective material in area of fibrosis, subretinal fluid at macula, outer retinal layer disruption and variable choroidal thickness. FFA showed hyperfluorescent choroiditis lesions in early and late frames and staining of peripapillary fibrosis with one patient showing retinal vascular occlusion. ICGA showed hypo spots which persisted in late frames. Histopathology of enucleated eyes showed granulomatous inflammation in outer retina and collagen in areas of fibrosis. All eyes remained stable with a final visual acuity ranging from 2/60 to 6/36

Conclusions

This report highlights a rare presentation of subretinal fibrosis with multifocal chorioretinitis lesions in SO. Diagnostic difficulties,rapid progression,and poor response to steroids and immunosuppressant pose challenge in these cases. Early institution of targeted biologics might prevent these eyes from going blind