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  Table of Contents    
CASE REPORT  
Year : 2019  |  Volume : 62  |  Issue : 4  |  Page : 592-594
Sympathetic ophthalmia with incidental finding of chicken pox supported by histopathology and immunohistochemistry


1 Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
2 Larsen and Toubro Ocular Pathology Department, Sankara Nethralaya, Chennai, Tamil Nadu, India

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Date of Web Publication14-Oct-2019
 

   Abstract 


Sympathetic ophthalmia (SO) is a rare bilateral diffuse granulomatous panuveitis that occurs in few days to several years after penetrating injury. This intraocular inflammation can occur in any age group without a sex predilection. Pathology and immunohistochemistry-supported evidence is important to know the disease in a better way. We present a case of a 24-year-old female with clinical diagnosis of SO with an atypical past history of chicken pox in that eye and residual corneal opacity.

Keywords: Choriocapillaries, choroid, immunohistochemistry, sympathetic ophthalmia, uvea

How to cite this article:
Das D, Krishnakumar S, Biswas J. Sympathetic ophthalmia with incidental finding of chicken pox supported by histopathology and immunohistochemistry. Indian J Pathol Microbiol 2019;62:592-4

How to cite this URL:
Das D, Krishnakumar S, Biswas J. Sympathetic ophthalmia with incidental finding of chicken pox supported by histopathology and immunohistochemistry. Indian J Pathol Microbiol [serial online] 2019 [cited 2019 Dec 8];62:592-4. Available from: http://www.ijpmonline.org/text.asp?2019/62/4/592/269059





   Introduction Top


Sympathetic ophthalmia (SO) is a non-necrotizing diffuse granulomatous inflammation, which can cause panuveitis.[1],[2],[3],[4] It is a condition affecting the eye and known better among specialists outside ophthalmology for the dreadful outcome.[4],[5] The cause of this panuveitis is chiefly the penetrating trauma to the eye, but surgery, laser applications to the eye can induce this condition.[6],[7],[8] The injured eye is referred to as the exciting eye and the fellow eye is referred to as the sympathizing eye.[2],[3],[5] Presently, the standard care of this condition is high-dose corticosteroids followed by immunosuppressives.[3],[5],[6],[7] Immunosuppressives are required to be continued for the long term to avoid recurrences and complications.[3],[4],[5],[6] Rarely, eyes are obtained for histopathology.[5],[8],[9],[10] We present a young lady with a clinical diagnosis of SO and having some structural complications. Incidentally, she had a past history of chicken pox in the inciting eye with residual corneal opacity. The histopathology and immunohistochemistry supported the diagnosis.


   Case Report Top


A 24-year-old female came to a referral center of south India complained of diminution of vision in the left eye (OS) for 1 month. She had corneal opacity following chicken pox 12 years back. Right eye (OD) was diagnosed to have exudative retinal detachment (ERD). She was on antiglaucoma medication in OS when she came for the consultation. On examination, best-corrected visual acuity was 20/30 in the OD and no perception of light in the OS. The OD had aqueous cells 1+ and aqueous flare 1+. Optical coherence tomography (OCT) was done. OCT showed shallow foveal contour with subretinal fluid in both eyes (OU). Choroidal thickening was noted OU. Subfoveal choroidal thickness was 719 microns OS. She was suspected to have SO. The patient was put on the tablet Prednisolone 60 mg/day and gradually tapered. She had raised intraocular pressure (29 mmHg, Applanation tonometry) in the OS and the glaucoma clinic opinion was taken. She was put on Timolol maleate 0.5% eyedrops two times daily in OS. Investigations such as complete blood count with erythrocyte sedimentation rate were normal in the patient; chest X-ray, Mantoux, and serum angiotensin-converting enzyme were within normal limits. Antinuclear antibody and serology for syphilis were also negative for her. She received a course of intravenous methyl-prednisolone also. Subsequently, she was put on tablet Azathioprine 50 mg one tablet three times daily and gradually tapered. On first month visit, she had subfoveal choroidal thickness of 463 microns OS. OS was painful and blind. Therefore, she has been referred to our oculoplasty department for consideration of enucleation/evisceration. She was seen here six-week time, when her vision was 20/20; N6 in the OD. Slit lamp examination revealed a quiet eye with no vitreous cells. Fundus showed an attached retina.

Evisceration specimen showed evidence of disorganization of internal structures. There was gross choroidal thickening with infiltration of mononuclear cells including lymphocytes, few plasma, and epithelioid cells [Figure 1]a. A strip of retinal pigment epithelium (RPE)–choriocapillaris–choroid complex showed sparing of choriocapillaris by those inflammatory cells [Figure 1]b. Choroidal pigmentations were seen scattered. The central part of the tissue showed granulomatous and non-necrotizing infiltrations in the choroidal tissue. Some pigment phagocytosis was also noted along with hyperplastic changes. Isolated clustered epitheloid bodies were seen in the tissue and documented [Figure 2]. Focally, fibrosis and gliosis with few foam cells were seen. CD3 [Cell Marque, USA]: +++ was positive for the inflammatory cells in the choroid [Figure 3]a. Sparing of choriocapillaries by the immune cells was documented [Figure 3]a. CD20 [Cell Marque, USA]: ++ [Figure 3]b; CD 20 cells were less positive compared with CD3 (T-cell). The findings were consistent with posterior SO.
Figure 1: (a) ×200, H and E, diffuse lymphocytic infiltration of choroid. (b) Sparing of choriocapillaries by inflammatory cells (marked with arrows)

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Figure 2: ×400, H and E; (a) Giant cells with inflammatory reaction and (b) epitheloid cells seen along with lymphocytic cells (Marked with arrows in A and B)

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Figure 3: (a) ×100, IHC, for CD3 (T cell Marker, CELL MARQUE, USA): +++, adequate control was taken (Sparing of choriocapillaries marked with arrow). (b) IHC, for CD20 (B cell Marker, CELL MARQUE, USA): ++, slides were compared with controls

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   Discussion Top


Pathological change of SO is known to cause a diffuse uveal granulomatous inflammation made up of lymphocytic infiltration with pigment-containing epithelioid and giant cells.[1],[2],[3],[4] In the acute phase of SO, the inflammatory process does not involve the choriocapillaris due to the influences of proinflammatory and anti-inflammatory cytokines secreted by RPE.[5] This pathological change is found to be similar in the exciting and sympathizing eye.[2],[3],[4],[5],[6] In some pathological enucleated eyeball specimens, eosinophils were found to be scattered in the inner choroidal layers.[5],[6] Nodular clusters of pigment-laden epithelioid cells known as Dalen Fuchs (DF) nodules are seen lying between the RPEs and Bruch's membrane and they were mostly seen in the acute phase of the active disease process.[5],[6] Our case did not have DF nodules in histopathological examination. In the chronic phase of the disease, pigment scar is evident at the level of the RPE. Immunohistochemical study revealed T-lymphocyte predominance in the uveal tissue. The exact cause of SO is not known so far.[2],[3],[4],[5] It can predispose to penetrating trauma, surgery, etc.[1],[2],[3] In the experimental animal, SO can be induced and it was found that the condition can be due to the breakdown in tolerance to uveal melanin that initiates the inflammation.[3]

Various authors have found that the choroid to be the targeted site of SO where the predominance of T-cells was CD 8+ subset and few have also found that predominance of T-cells was CD 4 (Leu) subset, the helper and inducer.[9],[10],[11] Out of T- and B-cells' affection, T-cell predominance was seen in 20 eyes, whereas 4 eyes showed B-cell predominance.[11] The presences of B-cells were related with long-term course of the disease and phthisical change.[11] In our case, eye did not have phthisical change. From a therapeutic point of view, immunosuppressive on T-cells was much more effective for SO.[9],[10],[11],[12],[13]

Our case had an atypical history of having chicken pox in the inciting eye with corneal opacity. Till date, there is no direct evidence of infectious agent(s) causing initiation of SO, but whether delayed hypersensitive reaction as a result of microtrauma following infectious triggering factor can promote autoimmune reaction is a subject of inquiry. Varicella-zoster virus causing kerato-uveitis can indirectly trigger the SO by assaulting the immune chain affecting either T- or B-cells. The patient presented with ERD with secondary glaucoma in that eye. She was clinically diagnosed as SO and was treated with immunosuppressive. Subsequently, the eye was removed by destructive surgery following complication and subjected to histopathology and IHC, which confirmed the diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has/have given her consent for her images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity.

Acknowledgements

Ms. K Vanitha, Technician, Pathology Department, Sankara Nethralaya Dept; Mr. Apurba Deka, MSc, Technician, Ocular Pathology Laboratory, Sri Sankaradeva Nethralaya, Guwahati for IHC staining.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Kilmartin DJ, Dick AD, Forrester JV. Prospective surveillance of sympathetic ophthalmia in the UK and republic of Ireland. Br J Ophthalmol 2000;84:259-63.  Back to cited text no. 1
    
2.
Chu XK, Chan CC. Sympathetic ophthalmia: To the twenty- first century and beyond. J Ophthalmic Inflamm Infect 2013;3:49.  Back to cited text no. 2
    
3.
Rao NA, Robin J, Hartmann D, Sweeney JA, Marak GE Jr. The role of the penetrating wound in the development of sympathetic ophthalmia experimental observations. Arch Ophthalmol 1983;101:102-4.  Back to cited text no. 3
    
4.
Biswas J, Fogla R. Sympathetic ophthalmia following cyclocryotherapy-report of a case with histopathological correlation. Ophthalmic Surg Lasers 1996;27:1035-308.  Back to cited text no. 4
    
5.
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.  Back to cited text no. 5
    
6.
Gasch AT, Foster CS, Grosskreutz CL, Pasquale LR. Postoperative sympathetic ophthalmia. Int Ophthalmol Clin 2000;40:69-84.  Back to cited text no. 6
    
7.
Martin KR, Broadway DC. Cyclodiode laser therapy for painful, blind glaucomatous eyes. Br J Ophthalmol 2001;85:474-6.  Back to cited text no. 7
    
8.
Bawankar P, Das D, Tayab S, Kuri GC, Medhi J, Barman M, et al. Cyclophotocoagulation-induced sympathetic ophthalmia in a Coats' disease patient supported by histopathology and immunohistochemistry. Indian J Ophthalmol 2017;65:744-6.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
Jakobiec FA, Marboe CC, Knowles DM 2nd, 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.  Back to cited text no. 9
    
10.
Rao N. Mechanisms of inflammatory response in sympathetic ophthalmia and VKH syndrome. Eye 1997;11:213-6.  Back to cited text no. 10
    
11.
Shah DN, Piacentini MA, Burnier MN, Mclean IW, Nussenblatt RB, Chan CC. Inflammatory cellular kinetics in sympathetic ophthalmia: A study of 29 traumatized (exciting) eyes. Ocul Immunol Inflamm 1993;1:255-62.  Back to cited text no. 11
    
12.
Arevalo JF, Garcia RA, Al-Dhibi HA, Sanchez JG, Suarez-Tata L. Update on sympathetic Ophthalmia. Middle East Afr J Ophthalmol 2012;19:13-21.  Back to cited text no. 12
[PUBMED]  [Full text]  
13.
Bawankar P, Das D, Barman M, Soibam R. Sympathetic ophthalmia associated with uncomplicated retinal detachment surgery in a young male: An uncommon entity. J Clin Ophthalmol Res 2018;6:24-6.  Back to cited text no. 13
  [Full text]  

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Correspondence Address:
Dipankar Das
Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology Services, Sri Sankaradeva Nethralaya, Guwahati, Assam
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_192_19

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