Sympathetic ophthalmitis after limbal corneal tear repair--a case study.
Sympathetic ophthalmitis is a rare bilateral cause of blindness. It is non-necrotising granulomatous disease. Uveitis occur after penetrating trauma, ocular surgery, i.e. pars plana vitrectomy, cataract surgery, retinal reattachment surgery, penetrating keratoplasty, trabeculectomy and even after enucleation.  Disease occurs within days or months (15-18%) 80% occurring within 3 months and 90% within one year.  Fuchs provide first histopathological detail in 1905 of inflammatory nodular aggregates found in choroid known as Dalen-Fuchs nodules. 
The disease has no predilection towards particular age, race or gender. Injured eye known as exciting eye and other eye as sympathising eye having similar pathology and clinical sign and symptoms suggestive of autoimmune response due to sensitisation of ocular tissue following penetrating injury.  Mainly cell mediated immune response directly against ocular self-antigen found on photoreceptor cell on RPE or choroidal melanocyte. Patient with sympathetic ophthalmitis express HLA-DR4. This phenotype also found in patient with VKH disease. 
An 8-year-old female child came to OPD with complaint of diminution of vision, pain and photophobia both eyes since 5 days. Patient has history of ocular trauma left eye by bamboo stick 2 months back while playing. There was limbal tear at 1 to 3 o'clock position with uveal tissue incarceration in LE. Meticulous limbal tear repair with excision of iris tissue was done under general anaesthesia 3 1/2 months back. Postoperatively fundus was normal with vision of CF 3 metres in LE and 6/6 in RE.
She came after 15 days for first follow-up, eye was quiet. Vision in LE was 3 metres and 6/6 in RE. Next follow-up, she did not come. Now she came after 3 1/2 months with pain and diminution of vision in both eyes.
Patient's vision was CF close to face in RE and hand movement in LE. Slit lamp examination of BE shows mixed conjunctival congestion, KPs, cells 4+ with exudation in anterior chamber, vitreous cell+, in RE pupil irregular due to posterior synechia. LE pupil was dilated, pigments present over lens, limbal suture present at 1-3 O'clock (Fig. 1, 2). RE media hazy due to vitritis, disc oedema and exudative retinal detachment present. LE fundus details not clear due to severe vitritis. B scan shows bilateral exudative retinal detachment. (Fig.3,4) FFA of RE show multiple hypo- and hyperfluorescence spots at the RPE level in the early venous phase, which then continues to late leakage. The optic nerve head staining seen in the late stage in both eyes (Fig. 5, 6). OCT right eye neurosensory detachment present. LE-details not clear due to hazy media (Fig. 7).
All routine investigations of blood and specific for uveitis was found normal. ANA level was normal, HIV was nonreactive. Paediatric and hearing assessment done, which was normal and non-significant. Treatment started with systemic I/V methylprednisolone 1 gm/kg body wt. for five days followed by oral prednisolone 50 mg OD. Locally prednisolone 1% drop 6/day, atropine 1% drop twice a day given. Sub-tenon triamcinolone given to exciting eye and suture was removed. Patient responded well from steroid therapy. AC reaction decreased and exudates resolved. Vision improved CF 5 meters in RE and counting finger 2.5 meters in LE. On follow-up vision was 6/60 in RE, CF 3 metre in LE after 1 week. Both eyes anterior chamber was quite. After 15 days, vision was 6/60 in RE and CF 3 meters in LE. OCT shows very less amount of subretinal fluid in both eyes. After 1 month, BCVA in RE 6/24 and in LE 6/36. OCT shows almost complete absorption of subretinal fluid (Fig. 8, 9).
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Sympathetic ophthalmitis affecting entire uveal tissue except choriocapillaries and retinal vessel. Presented as severe pan uveitis, granulomatous KP (Mutton fat), severe anterior chamber reaction, iris thick and posterior synechiae formation. Exudative retinal detachment, vasculitis, gliosis, choroid get thickened due to white lesion found in choroid known as Dalen-Fuchs nodule. It is similar like classical granuloma composed of inflammatory cell, lymphocyte, epithelioid cell and multinucleated giant cell found between RPE and Bruch's membrane.  Optic disc swelling, macular oedema, subretinal neovascularisation are other fundus finding.
Differential diagnosis of sympathetic ophthalmitis is VKH having same HLA typing, but more systemic association and malignancy.  Differential diagnosis also involve disease presented as panuveitis like tuberculosis, sarcoidosis, syphilis; these are accompanied by constitutional sign and symptoms by appropriate tests like PPD and X-ray chest for TB, sarcoidosis ruled out by serum ACE, lysozyme and chest X-ray. Syphilis rule out by RPR and FTA-ABS. Lymphoma, bilateral phacoanaphylactic reaction, multifocal choroiditis can have similar picture but neither infection nor iridocyclitis involve fellow eye.
Visual prognosis depends on the involvement of retina and early start of treatment. It responds well to steroid and systemic anti-inflammatory therapy, steroid given for longterm basis. Corticosteroid treatment is associated with weight gain, systemic side effect such as diabetes mellitus, adrenal insufficiency, hypertension and osteoporosis must be considered carefully. In refractory to steroid therapy, systemic immunosuppressive drugs (i.e. cyclosporine and azathioprine) can be combined with steroid, which may improve prognosis.  Research into treatment that focus on mechanism in the pathogenesis of sympathetic ophthalmitis has produced a number of potential immune mediators. Disruption of leucocyte recruitment by targeting gelatinase B (Mature metalloproteinase-9) CCL2 and CXCL12 may hold promise for future treatment.  Targeting of TNF-a in sympathetic ophthalmitis may also be promising, treatment with anti-TNF agent has been used for uveitis may benefit for sympathetic ophthalmitis. 
The case of sympathetic ophthalmitis is very rare nowadays due to improved surgical intervention early. Studies are very less due to less occurrence of disease. Recently treatment modalities should be changed from systemic therapy toward local therapy. Benefit of anti-inflammatory and immunomodulatory therapy has convincing trends and significant variability in outcome and adverse effect associated with long-term systemic therapy. It needs further studies of immunopathogenesis and targeted therapy for sympathetic ophthalmitis.
[1.] Brackup AB, Carter KD, Nerad JA. Long term follow up of severely injured eyes following globe rupture. Ophthalmic Plast Reconstr Surg 1991;7(3):1994-7.
[2.] Goto H, Rao NA. Sympathetic ophthalmia and Vogt-Koyanagi-Harada syndrome. Int Ophthalmol Clin 1990;30(4):279-285.
[3.] Albert DM, Diaz-Rohena R. A historical review of sympathetic ophthalmia and its epidemiology. Surv Ophthalmol 1989;34(1):1-14.
[4.] Kilmartin DJ, Dick AD, Forrester JV. Sympathetic ophthalmia risk following vitrectomy. Should we council patients? Br J Ophthalmol 2000;84:448-9.
[5.] Davis JL, Mittal KK, Freidlin V, et al. HLA associations and ancestry in Vogt-Koyanagi-Harada disease and sympathetic ophthalmia. Ophthalmology 1990;97(9): 1137-1142.
[6.] Jakobiec FA, Marboe CC, Knowles DM. Human sympathetic ophthalmia: an analysis of the inflammatory infiltrate by hybridoma-monoclonal antibodies, immunochemistry, and correlative electron microscopy. Ophthalmology 1983; 90(1):76-95.
[7.] Al-Halafi A, Dhibi HA, Hamade IH, et al. The association of systemic disorders with Vogt-Koyanagi-Harada and sympathetic ophthalmia. Graefes Arch Clin Exp Ophthalmol 2011;249(8):1229-33.
[8.] Vote BJ, Hall A, Cairns J, et al. Changing trends in sympathetic ophthalmia. Clin Experiment Ophthalmol 2004;32(5):542-545.
[9.] Abu El-Asrar AM, Struyf S, van den Broeck C, et al. Expression of chemokines and gelatinase B in sympathetic ophthalmia. Eye 2007;21(5):649-657.
[10.] Vazquez-Cobian LB, Flynn T, Lehman TJ. Adalimumab therapy for childhood uveitis. J Pediatrics 2006;149 (4):572-575.
Swati Kujur (1), Mangi Lal Garg (2), Rajesh Sahu (3)
(1) Associate Professor, Department of Ophthalmology, Pt JNM Medical College, Raipur, Chhattisgarh.
(2) Professor & HOD, Department of Ophthalmology, Pt JNM Medical College, Raipur, Chhattisgarh.
(3) Assistant Professor, Department of Ophthalmology, Pt JNM Medical College, Raipur, Chhattisgarh.
Financial or Other, Competing Interest: None.
Submission 22-06-2016, Peer Review 18-07-2016, Acceptance 23-07-2016, Published 01-08-2016.
Dr. Rajesh Sahu, C/o R. Mishra, In Fron t of Old Toy Factory, Sai Nagar, Jaii Road, Raipur, Chhattisgarh-492001.
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|Title Annotation:||Case Report|
|Author:||Kujur, Swati; Garg, Mangi Lal; Sahu, Rajesh|
|Publication:||Journal of Evolution of Medical and Dental Sciences|
|Article Type:||Clinical report|
|Date:||Aug 1, 2016|
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