Sympathetic ophthalmia, its pathogenesis and prophylaxis.
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Sympathetic ophthalmia was given its complete clinical description by William Mackenzic in 1840, and fully described in terms of its histopathology by Ernst Fuchs in 1905. A review of epidemiologic data from the 19th and 20th centuries reveals that acceptable statistical studies have yet to be carried out, but suggests that the disease has always been of extremely low incidence. Further, there does not appear to be a markedly decreased incidence in the "modern era." The etiology of the disease remains elusive, although the pathogenesis appears to be due to delayed hypersensitivity (cell-mediated immunity), possibly directed at a surface membrane antigen that is shared by photoreceptors, retinal pigment epithelium and choroidal melanocytes. The only effective therapy remains preventive enucleation of the injured eye, despite modern usage of immunosuppressive agents. Studies have suggested that early enucleation of a blind exciting eye can improve the prognosis for the sympathizing eye.
Eight cases of sympathetic ophthalmia diagnosed by histopathologic examinations of the enucleated exciting eyes were collected and the fundus fluorescein angiographic (FFA) findings of the sympathized eyes in acute stages were analyzed. The manifestations of the FFA were divided into three types. We suggest that if one eye has a history of trauma and the other eye loses visual acuity rapidly accompanied with uveitis signs after certain incubation period, the fundus fluorescein angiography should be examined to achieve a clinically objective diagnostic basis so that the patients are treated in time.
A case of sympathetic ophthalmia is presented in which a single lesion located posteriorly at the level of the retinal pigment epithelium caused a serous macular detachment. We believe that this lesion represented an atypical Dalen-Fuchs nodule. Most reports of the fluorescein angiographic appearance of Dalen-Fuchs nodules describe multiple sites of choroidal leakage during the early phases, which may coalesce during the late phases of the angiogram. The lesion we detected was hypofluorescent in early phases of the fluorescein angiogram and showed late staining. The early hypofluorescence seen in the lesion may be explained by an intact retinal pigment epithelium over the presumed Dalen-Fuchs nodule, blockage of choroidal fluorescence by the nodule, and areas of granulomatous inflammation in the inner choroid and choriocapillaris. The staining in the late phases of the fluorescein angiogram may be explained by accumulation of fluorescein into the presumed Dalen-Fuchs nodule.
Twenty patients with histopathologically confirmed sympathetic ophthalmia (Group 1) and eight patients with "presumed sympathetic ophthalmia," based on a clinical history of bilateral uveitis occurring within four months of a perforating ocular injury (Group 2), were typed for HLA-A, HLA-B, and HLA-C antigenic determinants by a micro-lymphocytotoxicity technique. HLA antigenic determinants found in Groups 1 and 2 were compared to a control group of 107 patients with sequelae of perforating ocular injuries from accidents, intraocular surgery, or corneal ulcers (Group 3). HLA-A11 antigen showed an increased frequency of 30% in Group 1 (relative risk = 11.0; P less than .002) and an increased frequency of 32% in Groups 1 + 2 (relative risk = 10.5; P less than .0005) compared to Group 3 (frequency = 4%). This finding was validated against a second control group of 453 healthy subjects without ocular disease or trauma (Group 4). This association suggests that a genetic factor may play an important role in the pathogenesis of sympathetic ophthalmia.
Sympathetic ophthalmia following cyclocryotherapy is extremely rare. The authors report a case of sympathetic ophthalmia after cyclocryotherapy for neovascular glaucoma due to central retinal vein occlusion. Histopathologic examination of the exciting eye showed features of significant sympathetic ophthalmia along with migration of uveal pigments to the limbal conjunctiva with lymphocytic infiltration around it, thereby indicating probable etiopathogenesis.
CASE REPORT: Sympathetic ophthalmia typically presents as bilateral granulomatous panuveitis that occurs after penetrating ocular injury. We describe a case of bilateral exudative retinal detachments in a patient with Turner syndrome and sympathetic ophthalmia. COMMENTS: To our knowledge, this is the first report of sympathetic ophthalmia in Turner syndrome.
The long held notion that sympathetic ophthalmia represents an autoimmune reaction to uveal pigment is no longer tenable. Pigmentation influences the histopathologic picture of sympathetic ophthalmia but no evidence supports the role of uveal pigment as inciting antigen. Several recent studies have confirmed our initial report of the participation of cellular hypersensitivity to ocular tissues in the pathogenesis of this disease. Both clinical and experimental studies implicate retinal tissue as being more immunogenic than uveal antigens. The recent histopathologic observation that eosinophils concentrate near the choriocapillaris suggests that outer retina and retinal pigment epithelium should not be overlooked as a potential source of the stimulating antigen in sympathetic ophthalmia.
Sympathetic uveitis developed in a 64-year-old woman 11 weeks after posttraumatic vitrectomy accompanied by vitreous hemorrhage. After removal of the exciting eye three weeks later, the sympathetic inflammation in the second eye subsided. Histologic examination revealed typical signs of an early stage of sympathetic ophthalmia characterized by a focally pronounced infiltration of lymphoid and T cells exclusively in the choroid. These T cells consisted of Leu 2a+ suppressor-cytotoxic T cells and a few Leu 3a+ inducer T cells situated near HLA-DR+ cells of the uvea. The latter contained electron-lucent cytoplasm without pigment granules and may represent the accessory cells for the initiation of the T-cell reaction. The findings suggest that sympathetic ophthalmia represents a T-cell-mediated cytotoxic reaction (similar to allograft rejection and graft-v-host disease) toward antigens on or near the pigment epithelium or the uveal melanocytes.
Sympathetic ophthalmia remains a dreadful complication of ocular traumas, accidentally but also surgically occurred; it may induced by certain ocular components, which are strongly antigenic, such as the uveal material or the recently discovered retinal material, represented by the interphotoreceptor binding protein and the S-antigen. The occurrence of the disease seems to be favoured by the transferring of the antigenic material through the conjunctival lymphatic mesh opened by the trauma. The paper presents a female patient with sympathetic ophthalmia occurred after cataract surgery.
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