Panuveitis with mild choroidal folds in the right eye of an 81-year-old man, 6 months after vitrectomy and intravitreal antibiotics for endophthalmitis in the left eye following a complicated cataract surgery. He noted gradual loss of vision for a week. Visual acuity was 20/50 in the right eye and light perception in the left eye. Visualization of the fundus was hazy due to 2+ keratic precipitates, anterior chamber inflammation, and mild vitritis. Note edematous and hyperemic disc and multiple light yellow lesions at the level of the retinal pigment epithelium. (Courtesy of Dr. Demetrios Vavvas)
Imaging
Fluorescein angiography (FA) is also useful in evaluating posterior segment involvement. In the acute phase of sympathetic ophthalmia, the FA typically demonstrates multiple hyperfluorescent sites of leakage at the level of the retinal pigment epithelium in the early phase followed by pooling of fluorescein dye in the detached areas (Fig. 8.2). In severe cases, these foci may consolidate with pooling of dye beneath the areas of exudative neurosensory detachment (Castiblanco and Adelman 2012). Dalen-Fuchs nodules may be hyper- or hypofluorescent depending on the integrity of the overlying RPE (Chang and Young 2011). The optic nerve may also demonstrate leakage in the later stages (Fig. 8.3). Optical coherence tomography (OCT) is helpful in demonstrating choroidal thickening in the posterior pole with or without exudative retinal detachment (Fig. 8.4).
Indocyanine green angiography (ICGA) is a useful diagnostic adjunct to confirm diagnosis and monitor response to therapy (Moshfeghi et al. 2005). Two patterns have been described. Hypofluorescence during the intermediate and late phase may correlate with chorioretinal atrophy. When there is active choroiditis, ICGA shows multiple hypocyanescent areas during the intermediate phase and not in in the late phase (Bernasconi et al. 1998). These are believed to represent focal, choroidal, inflammatory infiltrates (Fig. 8.5). B-scan ultrasonography can also be used to reveal choroidal thickening (Fig. 8.6).
Autofluorescence is a noninvasive imaging modality that can be useful in patients with sympathetic ophthalmia. Speckled areas of hypoautofluorescence resembling leopard spots can be seen in previous areas of exudative detachment. Over time, depigmentation of the RPE can occur (Fig. 8.7).
Differential
Other causes of bilateral granulomatous inflammation include Vogt–Koyanagi–Harada syndrome (VKH), sarcoidosis, phacoanaphylactic uveitis, chronic idiopathic uveitis, infectious granulomatous uveitis (bacterial and fungal) such as that occurs in tuberculosis and syphilis, and intraocular lymphoma. VKH in particular can have a similar presentation to sympathetic ophthalmia and may have Dalen-Fuchs nodules. These patients with VKH do not have a history of ocular injury and have systemic manifestations of VKH such as deafness, poliosis, and vitiligo.
Although there are no specific laboratory studies to establish the diagnosis of sympathetic ophthalmia, it is important to rule out the presence of other diseases with focused clinical studies. These include PPD skin testing, chest radiography, measurement of serum angiotensin-converting enzyme, lysozyme, RPR and FTA-Abs, and possibly lumbar puncture.
Prevention and Management
Surgical Treatment
The classic teaching has been that enucleation within 14 days after ocular injury protects the second eye from the development of sympathetic ophthalmia (Albert and Diaz-Rohena 1989). The protective role of enucleation has been largely unproven and generally discouraged unless open globe repair is deemed impossible at the time of injury (Manandhar 2011).
Controversy still exists regarding enucleation of the exciting eye once sympathetic ophthalmia has commenced. Although Lubin et al. published data that early enucleation of the exciting eye after the onset of sympathetic ophthalmia yields better vision in the sympathizing eye (Lubin et al. 1980), this has not been supported by more recent studies (Reynard et al. 1983). Enucleation should not be performed in the exciting eye with vision because not uncommonly, the exciting eye may be the one with the better vision (Moshfeghi et al. 2000; Gasch et al. 2000).
Medical Treatment
Immunosuppressive therapy is the mainstay of treatment. The initial approach, in most cases, should be with corticosteroids. Systemic corticosteroids are recommended on a daily basis, beginning with a high dose of a short-acting agent (1.0–1.5 mg/kg/day prednisone). If the disease is severe, intravenous pulse steroid therapy (1.0 g/day × 3 days) followed by oral prednisone can be considered (Hebestreit et al. 1997). Therapeutic efficacy should be evaluated at 3 months. If corticosteroid therapy is effective, then a slow taper should be initiated (Table 8.1).
Table 8.1
Treatment for sympathetic ophthalmia
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