Sympathetic ophthalmia (ORPHA:79098)
Sympathetic ophthalmia (SO) is a bilateral granulomatous anterior uveitis usually occurring within the three months following trauma or a surgical procedure involving one eye.
Epidemiology
SO is reported to affect between 1/1000 and 1/1650 individuals with a history of a penetrating eye wound.
Clinical description
Patients present with pain, photophobia, paresis of accommodation, metamorphopsia and mild to significant visual loss. The granulomatous anterior uveitis is accompanied by posterior segment findings including moderate to severe vitritis, choroiditis, papillitis, perivasculitis, and yellow-white lesions of the retinal pigment epithelium (Dalen-Fuchs nodules). The inflammation can lead to serous retinal detachment and macular edema. Extraocular symptoms include headache, meningitis or cerebrospinal fluid pleocytosis, hearing loss, poliosis and vitiligo.
Etiology
The etiology of SO is not completely understood. The inflammation is caused by a cell-mediated immune mechanism and autoimmune inflammatory response directed against ocular self-antigens released after the initial injury. The nature of these self-antigens remains controversial. Bacterial infection may potentiate the development of SO and genetic factors may also be implicated as associations with specific major histocompatibility antigens (HLA-DR4, HLA-A11 or HLA-B40) have been reported. SO may occur after ocular trauma (47 to 65 % of patients) or contusions. Wounds involving the ciliary body are associated with the highest risk. Surgical interventions may also trigger SO, with posterior segment surgery carrying a higher risk than anterior segment surgery.
Diagnostic methods
Diagnosis of SO is mainly based on patient history and clinical presentation. Imaging studies (fluorescein or indocyanine green angiography, B-scan ultrasonography and optical coherence tomography) may be useful to confirm the diagnosis.
Differential diagnosis
Laboratory analysis can be performed to eliminate infectious uveitis. When a history of trauma has been confirmed, endophthalmitis and other forms of post-traumatic uveitis (lens-induced uveitis, post-traumatic iridocyclitis) should be included in the differential diagnosis. Other diseases associated with granulomatous uveitis, including sarcoidosis and Vogt-Koyanagi-Harada syndrome (see these terms) or uveal effusion syndrome, may also be considered in the absence of a recognized history of ocular trauma.
Management and treatment
Prompt and careful closure of all wounds decreases the risk of SO. The primary treatment is high doses of steroids (for a minimum of 3 months), followed by a taper according to the inflammatory response and maintenance therapy for 6-12 months after resolution. Immunomodulators (cyclophosphamide, azathioprine or cyclosporine) may be considered when the inflammation cannot be controlled by steroids alone. Surgical treatment remains controversial and enucleation or evisceration should only be proposed in cases of blindness or pain in the inciting eye as the visual prognosis of the sympathizing eye is variable. Late enucleation has not been shown to be beneficial.
Prognosis
Without treatment, the visual prognosis is poor with SO leading to bilateral blindness. However, prompt wound closure and efficient medical treatment improve the visual prognosis, which may then be relatively good. However, relapses and complications may occur and long-term follow-up is required.

                            Last update: 
                            March 2009


                            - Expert reviewer(s): 
                            Dr Christophe ORSSAUD