Ocular Immunology and Inflammation ISSN: 0927-3948 (Print) 1744-5078 (Online) Journal homepage: http://www.tandfonline.com/loi/ioii20 Multimodal Imaging in Sympathetic Ophthalmia Sarakshi Mahajan MBBS, Alessandro Invernizzi MD, Rupesh Agrawal MD, Jyotirmay Biswas MD, Narsing A. Rao MD & Vishali Gupta MD To cite this article: Sarakshi Mahajan MBBS, Alessandro Invernizzi MD, Rupesh Agrawal MD, Jyotirmay Biswas MD, Narsing A. Rao MD & Vishali Gupta MD (2016): Multimodal Imaging in Sympathetic Ophthalmia, Ocular Immunology and Inflammation, DOI: 10.1080/09273948.2016.1255339 To link to this article: http://dx.doi.org/10.1080/09273948.2016.1255339 Published online: 14 Dec 2016. Submit your article to this journal Article views: 29 View related articles View Crossmark data Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ioii20 Download by: [The UC San Diego Library] Date: 01 January 2017, At: 05:42 Ocular Immunology & Inflammation, 2016; 00(00): 1–8 © Taylor & Francis Group, LLC ISSN: 0927-3948 print / 1744-5078 online DOI: 10.1080/09273948.2016.1255339 REVIEW ARTICLE Multimodal Imaging in Sympathetic Ophthalmia 1 2,3 4 Sarakshi Mahajan, MBBS , Alessandro Invernizzi, MD , Rupesh Agrawal, MD , Jyotirmay Biswas, 5 6 1 MD , Narsing A. Rao, MD , and Vishali Gupta, MD 1Advanced Eye Centre, Post Graduate Institute of Medical Education & Research, Chandigarh, India, 2Uveitis and Ocular Infectious Diseases Service – Eye Clinic, Department of Biomedical and Clinical Science, “Luigi Sacco” Liuigi Sacco Hospital, University of Milan, Milan, Italy, 3Department of Clinical Sciences and Community Health, University of Milan, Ophthalmological Unit, IRCCS-Cà Granda Foundation – Ospedale Maggiore Policlinico, Milan, Italy, 4National Healthcare Group Eye Institute, Tan Tock Seng Hospital, Singapore, 5Uveitis and Ocular Pathology Department, Sankara Nethralya, Chennai, India, and 6USC Roski Eye Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA ABSTRACT Purpose: To show the current status of multimodal imaging and its role in supporting an early diagnosis of sympathetic ophthalmia. Methods: The diagnosis is mainly clinical supported with ancillary investigations; mainly fluorescein angiography and others, including indocyanine angiography optical coherence tomography (OCT), OCT enhanced depth imaging, autofluorescence imaging, and ultrasonography. Results: Various imaging modalities such as OCT, autofluorescence imaging and angiography are critical in the diagnosis and management of sympathetic ophthalmia. The clinician must make adequate use of such ancillary investigations in the management of the patients. Conclusions: Sympathetic ophthalmia is a rare, bilateral inflammation of the uveal tract following penetrating trauma or surgery in one eye. The intraocular inflammation requires a prompt diagnosis so that the treatment can be initiated as early as possible. Keywords: Autofluorescence, fluorescein angiography, indocyanine green angiography, OCT-enhanced depth imaging, sympathetic ophthalmia Sympathetic ophthalmia (SO) is a rare, bilateral dif- posterior segment manifestations, including optic fuse granulomatous uveitis that usually occurs fol- disc edema, exudative retinal detachment, vitritis lowing penetrating ocular trauma or surgery and Dalen-Fuchs nodules, and has characteristic find- involving the uvea in one eye. The inflammation pri- ings seen during the acute phase.4 Since the inflam- marily involves the choroid to begin with presenting mation primarily involves the choroid, indocyanine – as posterior uveitis that may evolve to panuveitis.1 4 green angiography (ICG) too offers useful informa- Rarely, the inflammation may involve iris and ciliary tion and may be used during follow-up to monitor body initially, followed by panuveitis. The diagnosis response to therapy. The improved imaging modal- of SO is usually based on the history and clinical ities to visualize the choroid with the enhanced depth signs of intraocular inflammation. The ancillary imaging (EDI) OCT, is being used frequently for mea- investigations are useful adjuncts to support the diag- suring changes in the choroidal thickness from the nosis, determine the severity and extent, as well as to inflammatory process. However, the ultrasound B evaluate the response to treatment during follow-up. scan can still be used to demonstrate diffuse choroi- Fundus fluorescein angiography (FFA) has been used dal thickening in the posterior pole with or without most commonly in patients who present early with exudative retinal detachment, in case EDI OCT is Received 13 May 2016; revised 18 October 2016; accepted 27 October 2016; published online 13 December 2016 Correspondence: Vishali Gupta, MD, Professor, Advanced Eye Center, Post Graduate Institute of Medical Education and Research (PGIMER), Sector 12, Chandigarh 160012, India. E-mail: [email protected] Color versions of one or more of the figures in the article can be found online at www.tandfonline.com/ioii. 1 2 S. Mahajan et al. unavailable or media is hazy.3,4 Autofluorescence imaging changes seen from the pathologic changes of retinal pigment epithelium, mostly take place dur- ing the chronic phase. FLUORESCEIN ANGIOGRAPHY At the onset of intraocular inflammation in sympathetic ophthalmia, the FFA features of retina and choroid differ from those observed during chronic or chronic-recurrent phases of this granulomatous inflammation.Initially,the clinical presentation of SO may be restricted to the ocular fundus in the form of posterior uveitis (Figure 1)with diffuse thickening of the choroid and or multifocal nod- – fi ular changes in the choroid.1 4 The nodular changes may FIGURE 2. Wide- eld fundus image of the left eye in a patient with chronic sympathetic ophthalmia, showing a depigmented be from either Dalen-Fuchs nodules or foci of epithelioid choroid, juxta-papillary choroidal atrophy, and numerous histiocytic cell collections in the inner choroid. nummular chorioretinal scars at the periphery. Subsequently, as the inflammation progresses, the poster- ior uveitis evolves into panuveitis. However, some patients with SO may present initially with posterior leaks, which gradually increase in size and coalesce under uveitis or anterior granulomatous uveitis that may evolve focal retinal detachments (Figure 3) resulting in multilob- fl ular pooling of the dye with staining of the subretinal into panuveitis. The intraocular in ammation is known 5 to be similar in the exciting eye and in the sympathizing exudates. The focal leaks may represent disruption in eye. In the chronic phase of the inflammation, most RPE cell junctions or damage to RPE. However, there is fi patients present with panuveitis or anterior uveitis with no histopathologic con rmation of such RPE changes fi 4 extensive chorioretinal damage, including depigmenta- correlating with this angiographic nding. The choroid tion of choroid in pigmented individuals and display of perfusion may be delayed focally or as patchy areas. Usually, the optic disc shows leaking of disc vessels or multiple nummular chorioretinal scars predominantly in the periphery of the fundus (Figure 2). staining of the nerve head. Two distinct types of abnormal angiographic features The second type of the angiographic features is similar to that observed in patients with acute posterior multi- are known to occur in SO during initial presentation; 4 initial pinpoint hyperfluorescent leaks or multiple hypo- focal placoid pigment epitheliopathy (APMPPE). These fl fluorescent spots with dye pooling in the late phase. The changes are less common and consist of hypo uorescent pattern with initial pinpoint hyperfluorescent leaks is seen foci during the early phase of angiography and these foci fl more frequently and is virtually identical to that seen in become hyper uorescent subsequently. Unlike APMPPE, – – these focal lesions are slightly elevated and may reveal a the acute phase of Vogt Koyanagi Harada (VKH) dis- 3 ease. Typically, hyperfluorescent multifocal leaks of the mild mottled appearance. It is postulated that the hypo- fl dye at the level of retinal pigment epithelium (RPE) are uorescence could be from the obscuration of choroidal fl present. These leaks are commonly described as pinpoints uorescence by Dalen-Fuchs nodules or from focal FIGURE 1. A 15-year-old female with repaired full thickness anterior scleral laceration presented about 8 weeks later, with bilateral decreased vision. Fundus examination revealed bilateral hyperemic optic disc with multifocal serous detachments of retina seen in the posterior pole. Ocular Immunology & Inflammation Imaging in Sympathetic Ophthalmia 3 FIGURE 3. The patient with fundus changes depicted in Figure 1 showed multiple hyper- and hypofluorescent spots (A) during early phase of the angiography. The hyperfluorescent spots gradually increased in size and coalesced at the sites of retinal detachment (B,C). At the late phase, the angiogram shows subretinal pooling of dye in lobular pattern in right and left fundus (C,D). obliteration of the choriocapillaris by the inflammatory imaging modality to evaluate the choroid and to look cells extending from the choroid.6 Thelatehyperfluores- for the presence of choroidal infiltrates.9,10 The common cence represents staining of the lesions.7 findings on ICGA for patients with SO are multiple hypo- ThechronicphaseofSOiscomplicated by chorioret- cyanescent spots, as against hyperfluorescent spots on inal damage, nummular