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| CASE REPORT |
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| Year : 2015 | Volume
: 3
| Issue : 2 | Page : 63-66 |
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Recurrent sympathetic ophthalmitis
Mona Deshmukh, Chandan G Tiple
Department of Ophthalmology, Government Medical College (GMC) Nagpur, Maharashtra, India
| Date of Web Publication | 17-Dec-2015 |
Correspondence Address:
Chandan G Tiple
Ashok Nagar, Near Ashok Buddha Vihar, Visapur, Tah-Ballarpur, Chandrapur - 442 701, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/1858-6538.172103
Sympathetic ophthalmitis (SO) is a rare, bilateral granulomatous uveitis occurring after perforating eye injury or ocular surgical procedure to one eye. The pathophysiology of this entity is not clearly understood, but an autoimmune hypersensitivity reaction against exposed ocular antigens in the injured eye is believed to be responsible for this disease. In this article, we present a patient with clinical diagnosis of SO with it recurrence. Keywords: Autoimmune hypersensitivity, granulomatous uveitis, ocular antigens, perforating eye injury, recurrent sympathetic ophthalmitis, steroid, sympathetic ophthalmitis
How to cite this article:
Deshmukh M, Tiple CG. Recurrent sympathetic ophthalmitis. Albasar Int J Ophthalmol 2015;3:63-6 |
| Introduction | |  |
Sympathetic ophthalmitis (SO) is defined as a bilateral granulomatous uveitis of unknown etiology, occurring after penetrating trauma or intraocular surgery. It is believed to represent a form of autosensitization of ocular tissue following a perforating injury to one eye. Its exact incidence is not known but is thought to be 0.19% after penetrating injuries and 0.007% after intraocular surgery.[1]
SO is an uncommon disease due to improved surgical techniques employed in the repair of ocular injuries and early enucleation of the blind eye. The risk of developing SO in severely traumatized eyes with no visual potential that are not enucleated is exceedingly low.[2]
Numerous surgical procedures such as 23-guage vitrectomy,[3] cataract extraction,[4] retinal detachment surgery,[5] penetrating keratoplasty,[6] and trabeculectomy [7] have been complicated by SO.
We report a case of recurrent SO seen in our Outpatient Department (OPD) at Government Medical College and Hospital, Nagpur.
| Case Report | | |
A 25-year-old male patient, labor by occupation, residence of seoni, Madhya Pradesh, came to OPD with a complaint of gradual progressive loss of vision in the left eye since 15 days. There was a history of sutured full thickness extensive scleral perforation in the right eye 3 months back at the Tertiary Care Center. Considering the risk of SO, it was decided for enucleation of the injured eye, but the patient denied it. On examination, his best-corrected visual acuity (BCVA) was no perception of light in the right eye and finger counting 2 m with a projection of rays present in all four quadrants (FC 2 m PR + all 4 quadrants). The patient's right was physical with sutured sclera perforation [Figure 1]. His left eye on biomicroscopic examination showed multiple mutton-fat keratic precipitates (KPs) with 2–3 plus aqueous cells and flare in the anterior chamber [Figure 2]. The pupil was fixed and dilated with few posterior synechiae (PS) formations. The intraocular pressure (IOP) measured 14 mmHg. The fundus examination revealed optic disc edema. The patient went under laboratory investigations of complete blood count, erythrocyte sedimentation rate, rapid plasma regain, venereal disease research laboratory, fluorescent treponemal antibody absorption test, toxoplasmosis immunoglobulin G and M, angiotensin converting enzyme, and antinuclear antibody test. The patient also had X-ray chest, B-scan, fundus fluorescein angiography (FFA), and optical coherence tomography. All laboratory tests including X-ray chest were within normal limits. A clinical diagnosis of SO was confirmed, and the patient was commenced intravenous methylprednisolone 1 g/day for 3 days followed by tapering tablet prednisone 1 mg/kg body weight along with topical prednisolone 1% drops several times a day and atropine 1% thrice a day. The patient responded to treatment with a decrease in aqueous reaction and increase in visual acuity [Figure 3]. The optic disc margin, though still appeared blurred but edema had subsided significantly [Figure 4]. Patient during the first follow-up visit responded to treatment with less aqueous reaction and improved BCVA to 6/12. However, patient came with recurrence [8] of symptoms after 2 months with severe aqueous reaction with worsening of visual acuity from previously maintained stable therapy.
| Discussion | | |
The etiology of SO has not been completely understood, but the underlying pathophysiology is believed to be an autoimmune reaction against the exposed ocular antigens from the inciting eye.[9] The location of such antigens remains controversial and may be found in the uveal tissue, retina, or choroidal melanocytes. The immunologic studies have shown CD4 helper and inducer T cells during the early phase of inflammation compared to infiltration by CD8 suppressor and cytotoxic T cells in the later stage. There are B lymphocytes also found in some patients.[10]
Lymphocytes from patients with SO were demonstrated to respond to several uveoretinal antigens. Although no circulating antiretinal S-antigen antibodies were found, the serum from patients with SO showed antiretinal antibodies directed against the outer segment of photoreceptors and the Muller cells, when placed over the normal human retinal tissue.[11]
It has also been hypothesized that a purulent infection within the eye would destroy the uveal tissue in such a way that SO would not develop. However, some cases have been reported in eyes with endophthalmitis or fungal keratitis, indicating that the infection may not offer any prevention against development of SO.[12]
The SO can occur between 2 weeks and 3 months after an ocular injury, although it can develop as early as 5 days and as late as 66 years, 90% of cases become manifest in the 1st year after the injury.[13] Classically, the inflammation is granulomatous with multiple mutton-fat KPs adhered to corneal endothelium. The iris can be thick and sticky with PS formation. The IOP can be normal or fluctuating upward or downward due to the inflammatory involvement of ciliary body and trabecular meshwork. The vitreous is usually infiltrated with moderate to severe cellular reaction. The fundus can show swollen optic disc and multiple yellow-white lesions in the periphery, corresponding to the presence of Dalen-Fuchs nodules, which may not be seen in almost 50% of the cases. Serous retinal detachment or macular edema may be present with subretinal neovascularization.
On FFA, the optic nerve head shows hyperemia and dye leakage more pronounced in the late frames. There are multiple hyperfluorescent areas of choroidal leakage corresponding to the presence of Dalen-Fuchs nodules. The less common appearance on FFA is that of early hyperfluorescent lesions with staining in the late phase. This type of picture is thought to be related to whether the Dalen-Fuchs nodules have an intact or disrupted overlying retinal pigment epithelium (RPE).[14]
Extraocular findings such as pleocytosis of cerebrospinal fluid, hearing loss, alopecia, poliosis, and vitiligo have been reported with SO, although these findings are more common in Vogt-Koyanagi-Harada (VKH) disease.
The sequelae of the ocular inflammation include secondary glaucoma, cataract, optic atrophy, retinal detachment with subretinal fibrosis, and choroidal atrophy.
SO is characterized by a diffuse granulomatous, nonnecrotizing inflammation involving entire uveal tract. The choroid is thickened with lymphocytic infiltration along with the presence of eosinophils and plasma cells. Typically, the choriocapillaris is spared. The Dalen-Fuchs nodules representing migrated and transformed RPE cells are typical but not pathognomonic and may be present in other disease such as VKH syndrome. These nodules are a collection of epithelioid histocytes and lymphocytes, present between RPE and Bruch's membrane.[15],[16]
It is important to rule out the other causes of granulomatous uveitis before a diagnosis of SO can be entertained. Although diagnosis of SO is clinical, histopathology can be confirmatory. Autoimmune disease such as VKH, sarcoidosis, and multifocal choroiditis can have a similar presentation. Infections such as tuberculosis and syphilis should always be excluded.
Although corticosteroids have not been shown to be effective in the prevention of SO, they do constitute the mainstay of its therapy. Large doses of steroids should be given early in the course of the disease and continued for at least 6 months. For the 1st week, 1.5–2.0 mg/kg of body weight of oral prednisolone is given daily and then gradually tapered over several months, following clinical response of the uveitis. In a number of patients, medical problems or systemic complications may prevent the long-term use of high doses of steroids. In these patients, supplemental treatment with immunosuppressive agents such as azathioprine, cyclosporine, mycophenolate mofetil, and methotrexate has been shown to suppress inflammation effectively allow reduction of steroid therapy to nontoxic levels (<10 mg/day). The alkylating agents cyclophosphamide and chlorambucil are reserved for more severe and refractory cases.[13]
| Conclusion | | |
SO is a rare but a significant complication of penetrating ocular injury. In addition to systemic and intravitreal steroid therapy, immunosuppressive drugs also play a significant role in the medical management of this disease. The patient's medical treatment needs to be carefully monitored to reduce any side effects and improve visual prognosis.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
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| 2. | Brackup AB, Carter KD, Nerad JA, Folk JC, Pulido JS. Long-term follow-up of severely injured eyes following globe rupture. Ophthal Plast Reconstr Surg 1991;7:194-7. |
| 3. | Cha DM, Woo SJ, Ahn J, Park KH. A case of sympathetic ophthalmia presenting with extraocular symptoms and conjunctival pigmentation after repeated 23-gauge vitrectomy. Ocul Immunol Inflamm 2010;18:265-7. |
| 4. | Kinyoun JL, Bensinger RE, Chuang EL. Thirty-year history of sympathetic ophthalmia. Ophthalmology 1983;90:59-65. |
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| 11. | Chan CC, Palestine AG, Nussenblatt RB, Roberge FG, Benezra D. Anti-retinal auto-antibodies in Vogt-Koyanagi-Harada syndrome, Behcet's disease, and sympathetic ophthalmia. Ophthalmology 1985;92:1025-8. |
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| 14. | Sharp DC, Bell RA, Patterson E, Pinkerton RM. Sympathetic ophthalmia. Histopathologic and fluorescein angiographic correlation. Arch Ophthalmol 1984;102:232-5. |
| 15. | Jakobiec FA, Marboe CC, Knowles DM 2 nd, Iwamoto T, Harrison W, Chang S, et al. Human sympathetic ophthalmia. An analysis of the inflammatory infiltrate by hybridoma-monoclonal antibodies, immunochemistry, and correlative electron microscopy. Ophthalmology 1983;90:76-95. |
| 16. | Chan CC, Benezra D, Rodrigues MM, Palestine AG, Hsu SM, Murphree AL, et al. Immunohistochemistry and electron microscopy of choroidal infiltrates and Dalen-Fuchs nodules in sympathetic ophthalmia. Ophthalmology 1985;92:580-90. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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