Introduction:

Sympathetic ophthalmia is an exceedingly rare, bilateral diffuse granulomatous uveitis that develops following ocular penetrating trauma or surgery. Inflammation in the contralateral, unaffected eye develops days to years after the event, but usually occurs within three months of the injury.

Epidemiology:

Sympathetic ophthalmia is rare, affecting 0.2–0.5% of all traumatic penetrating eye injuries and 0.007% of patients after ocular surgery featuring a penetrating incision. The risk after pars plana vitrectomy is 0.01%.

Clinical Features:

Clinically, patients present with a bilateral severe, unremitting granulomatous panuveitis. There may be associated hypotony, small depigmented nodules at the level of the retinal pigment epithelium (Dalen–Fuch’s nodules), choroidal thickening, and serous retinal detachments (Fig. 17.5.1). Signs of prior injury or surgery in one eye are present.

OCT Features:

OCT findings include posterior choroidal thickening and accumulation of turbid subretinal fluid with deposition of fibrin and/or fibrinous bands in the subretinal fluid (Fig. 17.5.2). Macular edema may also be seen. In later stages of sympathetic ophthalmia, there is retinal and retinal pigment epithelium atrophy and thinning with transmission defects noted (Fig. 17.5.3).

Treatment:

Sympathetic ophthalmia can be prevented by enucleation of a blind, injured eye within two weeks after the trauma. Even after the onset of sympathetic ophthalmia, it may be of help to enucleate the eye that previously obtained injury. Once sympathetic ophthalmia is established in the contralateral eye, the mainstay of treatment consists of systemic anti-inflammatory agents such as oral corticosteroids or other immunosuppressive agents. More recently, treatments with local injections of corticosteroids in combination with or without systemic therapy, and intravitreal injections of infliximab have been reported.

Severe cases of sympathetic ophthalmia may be refractory to treatment.