Sympathetic ophthalmia: to the twenty-first century and beyond

Abstract

Sympathetic ophthalmia is a rare bilateral granulomatous inflammation that follows accidental or surgical insult to the uvea of one eye. Onset of sympathetic ophthalmia can be insidious or acute, with recurrent periods of exacerbation. Clinical presentation shows mutton-fat keratic precipitates, choroidal infiltrations, and Dalen-Fuchs nodules. Histopathology reveals diffuse or nodular granulomatous inflammation of the uvea. Prevention and treatment strategies for sympathetic ophthalmia are currently limited to two modalities, enucleation of the injured eye and immunosuppressive therapy, aimed at controlling inflammation. The etiology and pathophysiology of the disease is still unclear but is largely thought to be autoimmune in nature. Recent insight on the molecular pathology of the disease as well as developments in imaging technology have furthered both the understanding on the autoimmune process in sympathetic ophthalmia and the targeting of prevention and treatment strategies for the future.

Figure 1

Montage fundus photograph of a sympathetic ophthalmia retina. Yellow-white subretinal spots (arrows) on clinical fundus photographs correspond to histopathological Dalen-Fuchs nodules and choroidal infiltrates.

Figure 2

Histopathology of a Dalen-Fuchs nodule. There is an accumulation of mainly macrophages beneath the retinal pigment epithelium (brown color). Hematoxylin & eosin; original magnification, ×200.

Figure 3

Visualization of mild retinal vasculitis, an uncommon finding in sympathetic ophthalmia. (A) Fluorescein angiogram of a sympathetic ophthalmia retina shows mild vascular leakage revealing retinal vasculitis (arrows). (B) Histopathology confirms retinal vasculitis with lymphocytes (arrows) alongside the vessel wall. Hematoxylin & eosin; original magnification, ×400.