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Optical coherence tomography as a tool to detect early sympathetic ophthalmia in an asymptomatic patient

      Sympathetic ophthalmia (SO) is a rare and dreaded complication of accidental or surgical trauma and results in diffuse granulomatous panuveitis in both the injured (inciting) and contralateral (sympathizing) eye.
      • Cunningham Jr, E.T.
      • Kilmartin D.
      • Agarwal M.
      • Zierhut M.
      Sympathetic ophthalmia.
      Although the diagnosis of SO is made clinically and supported through histopathologic findings, multimodal imaging has been described to help support the diagnosis after disease onset.
      • Mahajan S.
      • Invernizzi A.
      • Agrawal R.
      • Biswas J.
      • Rao N.A.
      • Gupta V.
      Multimodal imaging in sympathetic ophthalmia.
      • Rathinam S.R.
      • Rao N.A.
      Sympathetic ophthalmia following postoperative bacterial endophthalmitis: a clinicopathologic study.
      • Behdad B.
      • Rahmani S.
      • Montahaei T.
      • Soheilian R.
      • Soheilian M.
      Enhanced depth imaging OCT (EDI-OCT) findings in acute phase of sympathetic ophthalmia.
      • Muakkassa N.W.
      • Witkin A.J.
      Spectral-domain optical coherence tomography of sympathetic ophthalmia with Dalen–Fuchs nodules.
      • Burkholder B.M.
      • Dunn J.P.
      Multiple serous retinal detachments seen on wide-field imaging in a patient with sympathetic ophthalmia.
      To the author's knowledge, our case is the first in which spectral-domain optical coherence tomography (OCT) was used to diagnose early SO in an otherwise asymptomatic patient.
      A 23-year-old male sustained penetrating ocular trauma to his left eye while trimming trees. Initial globe repair occurred within hours of the injury and resulted in chronically exposed uvea resulting from multiple complex lacerations. He was referred to the oculoplastics service at Maisonneuve-Rosemont Hospital in Montreal, Canada, for a painful eye with no visual potential, and uneventful enucleation of the traumatized eye was performed a total of 3 weeks after initial injury.
      Postoperatively, the patient was referred to an optometrist for fitting of polycarbonate lenses to protect the remaining right eye. One month after enucleation the patient visited an optometrist who performed routine spectral-domain OCT of the right eye. The OCT revealed retinal pigment epithelium (RPE) abnormalities that were concerning to the optometrist and the patient was referred to the uveitis service at Maisonneuve-Rosemont Hospital 2 weeks later.
      At the hospital the patient had no visual complaints, and the best corrected visual acuity (BCVA) was 6/6. The ocular examination (including slit-lamp and fundus examination) was normal (Fig. 1). Spectral-domain OCT was repeated and revealed a small serous retinal detachment with irregularity of the RPE (Fig. 2A). Fluorescein angiography (FA) showed a small pinpoint leak and optic disc leakage in the late phase (Figs. 3A and 3B). A complete uveitis work-up was carried out to rule out other causes of uveitis. The patient had never been on corticosteroids, and all laboratory and imaging tests were within normal limits. The patient was diagnosed with SO on the basis of his history and imaging findings. He was started on a tapering course of high-dose oral prednisone while mycophenolate was started. At 7 weeks’ follow-up, all OCT (Fig. 2B) and FA (Figs. 3C and 3D) abnormalities had resolved. At 6 months’ follow-up, ophthalmologic examination and imaging remained normal while solely on a long-term dose of mycophenolate 500 mg po BID.
      Fig 2
      Fig. 2(A) Optical coherence tomography (OCT) pretreatment. (B) OCT post-treatment.
      Fig 3
      Fig. 3(A, B) Fluorescein angiography (FA) pretreatment (early/late phase). (C, D) FA post-treatment (early/late phase).
      Histologic examination of the enucleated left eye demonstrated nongranulomatous inflammation (Figs. 4A–C). A strong T lymphocyte (CD3) infiltrate was noted in the choroid (Fig. 4d), as well as a limited number of B lymphocytes (CD20) (Fig. 4e) and plasma cells. There was severe chronic and acute inflammation throughout the uveal tract, extending to the choriocapillaris (Figs. 4B and 4C). A nodule-like subretinal lymphocytic aggregate was noted; however, the absence of multinucleated giant cells or monocytes within the nodule (Fig. 4f) and the lack of RPE cell involvement suggest that it did not represent a Dalen–Fuchs nodule. Pathognomonic histopathological findings for SO, such as Dalen–Fuchs nodules and multinucleated giant cells, were not observed. Moreover, the choriocapillaris is typically spared from lymphocytic infiltration in cases of SO. Nonetheless, in light of what is currently known about SO and Vogt–Koyanagi–Harada (VKH), this presentation was viewed as compatible with early SO.
      Fig 4
      Fig. 4(A) Scanning magnification. (B, C) Strong lympho-plasmacytic infiltration in the choroid, and involving the choriocapillaris. (D) T-cell-dense area using CD3. Note lack of granuloma. (E) Presence of B cells in infiltrate using CD20. Note lack of granuloma. (F) Few monocytes in infiltrate using CD68.
      Both SO and VKH disease are classically thought to demonstrate granulomatous inflammation and in fact are believed to be different phenotypic expressions of the same disease entity. A recent review of VKH
      • O'Keefe G.A.
      • Rao N.A.
      Vogt-Koyanagi-Harada disease.
      reported that the composition of Dalen–Fuchs nodules changed with stage of disease. In more acute phases, they were composed of retinal pigment epithelial cells, lymphocytes, and macrophages. With disease chronicity, they had far fewer inflammatory cells. Furthermore, it is now well known that both diseases can involve the choriocapillaris as well, particularly in severe diseases.
      Spectral-domain OCT and FA are key tools in the diagnosis of SO. The characteristic findings of these modalities in SO have been well described in the literature.
      • Mahajan S.
      • Invernizzi A.
      • Agrawal R.
      • Biswas J.
      • Rao N.A.
      • Gupta V.
      Multimodal imaging in sympathetic ophthalmia.
      • Rathinam S.R.
      • Rao N.A.
      Sympathetic ophthalmia following postoperative bacterial endophthalmitis: a clinicopathologic study.
      • Behdad B.
      • Rahmani S.
      • Montahaei T.
      • Soheilian R.
      • Soheilian M.
      Enhanced depth imaging OCT (EDI-OCT) findings in acute phase of sympathetic ophthalmia.
      • Muakkassa N.W.
      • Witkin A.J.
      Spectral-domain optical coherence tomography of sympathetic ophthalmia with Dalen–Fuchs nodules.
      • Burkholder B.M.
      • Dunn J.P.
      Multiple serous retinal detachments seen on wide-field imaging in a patient with sympathetic ophthalmia.
      However, SO usually comes to the attention of treating physicians after unequivocal disease onset, at which point vision may be permanently affected. This reality is particularly concerning in patients with functional vision in only the sympathizing eye.
      Retinal surgery is the most common cause of SO because of the frequency at which vitrectomies are performed.
      • Kilmartin D.J.
      • Dick A.D.
      • Forrester J.V.
      Prospective surveillance of sympathetic ophthalmia in the UK and Republic of Ireland.
      However, globe-penetrating trauma is likely the event most at risk to precipitate SO.
      • Gasch A.T.
      • Foster C.S.
      • Grosskreutz C.L.
      • Pasquale L.R.
      Postoperative sympathetic ophthalmia.
      Although there is a greater risk of developing SO within 1 year of globe-penetrating trauma,
      • Kilmartin D.J.
      • Dick A.D.
      • Forrester J.V.
      Prospective surveillance of sympathetic ophthalmia in the UK and Republic of Ireland.
      given the rarity of SO (minimum incidence of 0.03 cases per 100 000 people
      • Kilmartin D.J.
      • Dick A.D.
      • Forrester J.V.
      Prospective surveillance of sympathetic ophthalmia in the UK and Republic of Ireland.
      • Albert D.M.
      • Diaz-Rohena R.
      A historical review of sympathetic ophthalmia and its epidemiology.
      ), no guidelines exist for screening of the asymptomatic patient.
      Firm recommendations cannot be made on the basis of a single case. However, our report demonstrates that abnormalities on OCT can precede symptoms or even findings on ophthalmologic examination typical of SO. Therefore, in all patients who have experienced globe-penetrating trauma or in the setting of multiple or complicated surgery, we suggest complete ophthalmologic examination plus spectral-domain OCT every 2–4 months within the first year and annually thereafter.

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