Ocular Immunology and Inflammation
ISSN: 0927-3948 (Print) 1744-5078 (Online) Journal homepage: http://www.tandfonline.com/loi/ioii20
Optical Coherence Tomography Imaging of Presumed Sarcoid Retinal and Optic Nerve Nodules Naomi R. Goldberg MD, PhD, Douglas A. Jabs MD, MBA & Jacqueline Busingye MD To cite this article: Naomi R. Goldberg MD, PhD, Douglas A. Jabs MD, MBA & Jacqueline Busingye MD (2014): Optical Coherence Tomography Imaging of Presumed Sarcoid Retinal and Optic Nerve Nodules, Ocular Immunology and Inflammation, DOI: 10.3109/09273948.2014.971972 To link to this article: http://dx.doi.org/10.3109/09273948.2014.971972
Published online: 30 Oct 2014.
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Date: 13 November 2015, At: 14:33
Ocular Immunology & Inflammation, Early Online, 1–4, 2014 ! Informa Healthcare USA, Inc. ISSN: 0927-3948 print / 1744-5078 online DOI: 10.3109/09273948.2014.971972
Optical Coherence Tomography Imaging of Presumed Sarcoid Retinal and Optic Nerve Nodules Naomi R. Goldberg, MD, PhD1, Douglas A. Jabs, Jacqueline Busingye, MD1
MD, MBA
1,2,3,4
, and
Departments of Ophthalmology, 2Departments of Medicine, 3The Institute for Translational Epidemiology, The Icahn School of Medicine at Mount Sinai, New York, NY, and 4The Center for Clinical Trials, The Department of Epidemiology, The Johns Hopkins University Bloomberg School of Public Health, Baltimore, MD
Downloaded by [University of Florida] at 14:33 13 November 2015
1
ABSTRACT Purpose: To characterize nodular lesions of the retina and optic nerve with spectral-domain optical coherence tomography (SD-OCT) in patients with sarcoidosis. Methods: This is a retrospective series of 6 eyes from 5 patients with an established diagnosis of sarcoidosis, with clinically detected nodules of the optic nerve or retina. All lesions were imaged with fundus photography and SD-OCT on presentation, and followed with serial imaging after treatment with corticosteroids and/or immunomodulatory therapy. Results: Spectral OCT through the lesions revealed nodular hyperreflective processes obscuring the retinal layers or optic cup, with local structural changes, including subretinal and intraretinal fluid. After treatment with corticosteroids and/or immunosuppression in 4 followed patients, all lesions regressed with improvement in associated structural changes, but did not entirely disappear. Conclusions: Spectral OCT can be useful in identifying lesion morphology and location, and in tracking the response to treatment in eyes with posterior-segment nodules, presumably secondary to sarcoidosis. Keywords: Granuloma, nodule, OCT, optic nerve, retina, sarcoidosis
MATERIALS AND METHODS
Sarcoidosis is characterized histologically by the presence of noncaseating granulomas involving multiple organs, including the lungs, skin, and eyes. Though the most common ocular manifestation is anterior uveitis, posterior findings including panuveitis, retinal vasculitis, and more rarely, choroidal and retinal granulomas, can be seen.1–3 With the advent of spectral-domain optical coherence tomography (SD-OCT, Heidelberg Engineering, Germany), high-resolution imaging of the retina has enabled improved understanding of posteriorsegment pathology. Spectral OCT and enhanceddepth imaging have been formerly utilized to characterize presumed sarcoid choroidal granulomas and preretinal nodular lesions,4–6 but here we utilize SD-OCT for the first time to study the extrachoroidal targets of inflammatory nodules, within the retina and optic nerve, in patients with sarcoidosis.
We conducted a retrospective review of all patients diagnosed with sarcoidosis and clinically detected nodules of the optic nerve or retina. All patients except one had an established diagnosis of sarcoidosis upon presentation, and the remaining patient (case 5) acquired a diagnosis following systemic workup guided by the ophthalmic findings. Diagnoses of sarcoidosis were based on tissue biopsy (cases 3–5) or imaging, including gallium scanning or chest radiography showing hilar adenopathy (cases 1, 2). All included patients reported a chronic uveitis history ranging from months to years. Upon presentation, all patients underwent a complete serologic workup to exclude alternative infectious etiologies of nodular lesions, including syphilis and tuberculosis. All patients had best-corrected Snellen visual acuity determined and all were examined with slit-lamp
Received 13 June 2014; revised 21 September 2014; accepted 29 September 2014; published online 29 October 2014 Correspondence: Naomi R. Goldberg, MD, PhD, Department of Ophthalmology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029, USA. E-mail: [email protected]
1
2
N. R. Goldberg et al.
biomicroscopy, as well as fundus photography and SD-OCT of identified lesions, during the active phases of their diseases. Patients were treated for their uveitis with corticosteroids and/or immunosuppression for variable amounts of time, and repeat eye-tracked OCT images over identified lesions were again obtained when the uveitis quieted. Follow-up analysis was available for 4 of 5 patients. The study had approval by the Mount Sinai School of Medicine institutional review board and the Program for the Protection of Human Subjects.
Downloaded by [University of Florida] at 14:33 13 November 2015
RESULTS On presentation, SD-OCT through retinal lesions revealed nodular hyperreflective intraretinal processes protruding into the vitreous, with disorganized retinal architecture, reduced choroidal transmission, and associated structural change, such as intraretinal or subretinal fluid. The retinal pigment epithelium (RPE) appeared elevated at the base of these lesions, but the underlying choroidal contour appeared intact. The disc lesions were seen as hyperreflective masses obscuring the central cup, with adjacent peripapillary retinal edema and disturbed retinal anatomy. After treatment in all followed eyes, the lesions consolidated and diminished in height, the exudative changes decreased or resolved, and the anatomy of the neighboring tissues improved. No lesion completely disappeared following treatment.
Report of Cases Case 1 A 62-year-old female on 30 mg alternate daily use of prednisone for neurosarcoidosis was referred for uveitis management with a complaint of ongoing right eye distortion. On examination, visual acuity was 20/25 and there were 2 discrete elevated intraretinal nodules along the inferior arcade, with subretinal fluid and trace vitritis. She was treated with 3 g daily mycophenolate, and the prednisone was transiently increased to 60 mg daily and tapered to 5 mg daily with improvement of the acuity to 20/20, regression of the lesions by 1 year with a more confluent appearance, and resolution of the fluid, as detected by SD-OCT (Figures 1A, 2A-B).
Case 2 A 25-year-old male with longstanding sarcoid uveitis reported progressively decreasing vision in both eyes. Visual acuity was 20/80 in the right eye and 20/200 in the left eye, and there were posterior synechiae and cataract obscuring visualization of the fundus. Spectral OCT highlighted bilateral peripapillary intraretinal nodules disturbing the retinal architecture with RPE elevation at the base of the lesions, and subretinal fluid in the right eye (not shown). Following 60 mg prednisone and taper to 10 mg daily, the acuity improved to 20/40 in the right eye,
FIGURE 1. Various sites and morphologies of inflammatory nodules, by spectral-domain optical coherence tomography (SD-OCT), in patients with sarcoidosis. Color photographs and SD-OCT scans over sections of presumed sarcoid nodules (black lines). (A, B) Retinal lesions in cases 1 (A) and 3 (B), shown by SD-OCT as hyperreflective nodular intraretinal processes obscuring the retinal layers, with posterior shadowing. The pigment epithelium is slightly elevated at the base of the lesion (B, arrow) and the underlying choroid is flat. (C, D) Optic nerve nodules in cases 4 (C) and 5 (D), seen as hyperreflective masses obscuring the optic cup; adjacent choroidal granulomatous infiltration is also seen (C, arrow). Ocular Immunology & Inflammation
Downloaded by [University of Florida] at 14:33 13 November 2015
OCT of Sarcoid Retinal and Optic Nerve Nodules
3
FIGURE 2. Morphologic changes of nodular lesions following treatment, with eye-tracked spectral-domain optical coherence tomography, and corresponding fundus photographs (insets). (A) Intraretinal nodules in case 1 with subretinal fluid extending subfoveally, on presentation. (B) Following treatment with prednisone and mycophenolate, the fluid resolved (4 weeks), and lesions regressed with residual scarring (1 year). (C) Optic nerve nodule in case 5 on presentation, with adjacent retinal edema and secondary attenuation of the photoreceptor ellipsoid band (arrow). Following (D) 2 and (E) 12 months of prednisone, the lesion height decreased with residual gliosis and normalization of the adjacent peripapillary outer-retinal anatomy (arrow).
the lesions consolidated, and subretinal fluid resolved within 4 months. Case 3 A 30-year-old patient with a 6-month uveitis history presented with 20/20 visual acuity in the left eye, mild vitritis, and a yellow peripapillary intraretinal nodule (Figure 1B). Prednisone was initiated at 60 mg and tapered to 7.5 mg daily, with reduction of the lesion height within 9 months, as confirmed by SD-OCT. Case 4 A 41-year-old female on intermittent corticosteroids for chronic sarcoid uveitis had counting fingers acuity in the left eye. The eye was aphakic and there was an ill-defined yellowish lesion surrounding the nerve. Spectral OCT confirmed the presence of an infiltrative lesion within the optic nerve and adjacent choroid, with thickening of the neighboring peripapillary retina and attenuation of the photoreceptor ellipsoid zone (Figure 1C). !
2014 Informa Healthcare USA, Inc.
Case 5 A 38-year-old male with a 3-month uveitis history in the left eye, presented with 20/50 acuity, and examination revealing vitritis, a whitened optic nerve lesion, and cotton-wool spots. Spectral OCT showed an elevated hyperreflective central lesion of the optic cup with adjacent retinal edema. He received 1 g daily intravenous methylprednisolone for 3 days, followed by prednisone, tapered to 5 mg daily. The acuity improved to 20/20 within 6 weeks and the disc nodule gradually regressed, with normalization of the neighboring retinal anatomy, as seen with SD-OCT (Figures 1D, 2C–E).
DISCUSSION Sarcoid granulomas of the optic nerve and retina have been described histopathologically and presumptively illustrated clinically,1–3 and in this series, SD-OCT was used for in vivo characterization of the various sites, morphologies, and treatment responses
Downloaded by [University of Florida] at 14:33 13 November 2015
4
N. R. Goldberg et al.
of inflammatory nodules, the likely clinical correlates of granulomas, in patients with sarcoidosis. The OCT features of the retinal nodules here (cases 1–3) reflect formerly described histologic features of granulomas, including retinal thickening and disorganized retinal architecture by foci of accumulated perivascular epithelioid cells (possibly implicating the retinal vessels as a conduit for inflammation), as well as occasional subpigment epithelial granulomas in continuity with intraretinal granulomas with choroidal sparing.1 In addition, non-necrotizing granuloma emerging from the surface of the optic disc into the vitreous has been described,1 and likely correlates to the SD-OCT findings of hyperreflective masses obscuring the optic cup, as in cases 4 and 5. Though enhanced-depth imaging OCT was not utilized to study the choroid in these cases, the choroidal contour could be assessed with standard SD-OCT, and appeared to be normal in all eyes except for case 4, in which the peripapillary choroid appeared infiltrated with a dome-shaped configuration. Clearly, presumed sarcoid retinal and disc granulomas may exist either in isolation or in combination with choroidal granulomas, and need not necessarily extend from choroidal infiltration. Isolated Toxocara granulomas of the optic nerve and retina have also been documented by SD- or high-penetration OCT, and have shown similar findings of a superficial mass filling the optic cup7 or an elevated intraretinal lesion protruding from the inner retina into the vitreous, receding with treatment.8 Clinical examination of nodular masses, with attention to lesion position in reference to neighboring vessels, is usually sufficient in distinguishing choroidal from retinal involvement, and fluorescein angiography can facilitate delineation of those tissues involved. Yet, SD-OCT offers a noninvasive and rapid method of confirming the targeted tissues and associated structural changes, thereby facilitating the staging of disease severity, and tracking of treatment response. Moreover, SD-OCT is helpful in highlighting sites of pathology in eyes with clinically indistinct lesions or obscured visibility, as shown in cases 2 and 4. The ability to ascertain the level of tissue involvement of these lesions and recognize their appearance by SD-OCT, may in some cases, help distinguish these inflammatory lesions from similar appearing oncologic lesions whose imaging characteristics are known,9 particularly in eyes without additional sign of inflammation or in patients with a negative evaluation for sarcoidosis. In a former sarcoid choroidal granuloma series, only a small minority of lesions, treated acutely, completely disappeared.10 Here, all lesions, present chronically, regressed, but none entirely resolved despite aggressive treatment, supporting the possibility that lesion chronicity limits treatment response;10 alternatively, retinal and disc lesions may be less
responsive than their choroidal counterparts, echoing former reports of poorer outcomes in eyes with presumed sarcoid optic nerve granulomas.2,10 The ability, with SD-OCT, to accurately follow the evolution of granuloma-like lesions in various locations clearly facilitates diagnosis and treatment planning in sarcoidosis patients. A larger comparative OCT series of presumed sarcoid granulomas of the choroid, disc, and retina, with standardized treatment and followup, would enhance our understanding of posteriorsegment pathogenesis in sarcoidosis.
ACKNOWLEDGMENTS We’d like to acknowledge Dr. Scott Brodie, M.D., Dr. Scott Forman, M.D., and Dr. Lori Tindel, M.D. for referring three patients in this series and for providing prior imaging studies and serologic workup.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. We’d like to acknowledge Scott Brodie, MD, Scott Forman, MD, and Lori Tindel, MD for referring three patients in this series and for providing prior imaging studies and serologic workup.
REFERENCES 1. Gass JD, Olson CL. Sarcoidosis with optic nerve and retinal involvement. Arch Ophthalmol. 1976;94:945–950. 2. Laties AM, Scheie HG. Sarcoid granuloma of the optic disk: evolution of multiple small tumors. Trans Am Ophthalmol Soc. 1970;68:219–233. 3. Brownstein S, Jannotta FS. Sarcoid granulomas of the optic nerve and retina: report of a case. Can J Ophthalmol. 1974;9: 372–378. 4. Rostaqui O, Querques G, Haymann P, et al. Visualization of sarcoid choroidal granuloma by enhanced depth imaging optical coherence tomography. Ocul Immunol Inflamm. 2014;22:239–241. 5. Modi YS, Epstein A, Bhaleeya S, et al. Multimodal imaging of sarcoid choroidal granulomas. J Ophthalmic Inflamm Infect. 2013;3:58. 6. Wong M, Janowicz M, Tessler HH, et al. High-resolution optical coherence tomography of presumed sarcoid retinal granulomas. Retina. 2009;29:1545–1546. 7. Chelnis J, Selvadurai AD, Fasiuddin A. Enhanced depth imaging and fundus autofluorescence of Toxocara optic nerve granuloma. Ocul Immunol Inflamm. 2013;21:82–83. 8. Hashida N, Nakai K, Nishida K. Diagnostic evaluation of ocular toxocariasis using high-penetration optical coherence tomography. Case Rep Ophthalmol. 2014;5:16–21. 9. Say EA, Shah SU, Ferenczy S, et al. Optical coherence tomography of retinal and choroidal tumors. J Ophthalmol. 2012;2012:385058. 10. Desai UR, Tawansy KA, Joondeph BC, et al. Choroidal granulomas in systemic sarcoidosis. Retina. 2001;21:40–47. Ocular Immunology & Inflammation
ISSN: 0927-3948 (Print) 1744-5078 (Online) Journal homepage: http://www.tandfonline.com/loi/ioii20
Optical Coherence Tomography Imaging of Presumed Sarcoid Retinal and Optic Nerve Nodules Naomi R. Goldberg MD, PhD, Douglas A. Jabs MD, MBA & Jacqueline Busingye MD To cite this article: Naomi R. Goldberg MD, PhD, Douglas A. Jabs MD, MBA & Jacqueline Busingye MD (2014): Optical Coherence Tomography Imaging of Presumed Sarcoid Retinal and Optic Nerve Nodules, Ocular Immunology and Inflammation, DOI: 10.3109/09273948.2014.971972 To link to this article: http://dx.doi.org/10.3109/09273948.2014.971972
Published online: 30 Oct 2014.
Submit your article to this journal
Article views: 55
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: [University of Florida]
Date: 13 November 2015, At: 14:33
Ocular Immunology & Inflammation, Early Online, 1–4, 2014 ! Informa Healthcare USA, Inc. ISSN: 0927-3948 print / 1744-5078 online DOI: 10.3109/09273948.2014.971972
Optical Coherence Tomography Imaging of Presumed Sarcoid Retinal and Optic Nerve Nodules Naomi R. Goldberg, MD, PhD1, Douglas A. Jabs, Jacqueline Busingye, MD1
MD, MBA
1,2,3,4
, and
Departments of Ophthalmology, 2Departments of Medicine, 3The Institute for Translational Epidemiology, The Icahn School of Medicine at Mount Sinai, New York, NY, and 4The Center for Clinical Trials, The Department of Epidemiology, The Johns Hopkins University Bloomberg School of Public Health, Baltimore, MD
Downloaded by [University of Florida] at 14:33 13 November 2015
1
ABSTRACT Purpose: To characterize nodular lesions of the retina and optic nerve with spectral-domain optical coherence tomography (SD-OCT) in patients with sarcoidosis. Methods: This is a retrospective series of 6 eyes from 5 patients with an established diagnosis of sarcoidosis, with clinically detected nodules of the optic nerve or retina. All lesions were imaged with fundus photography and SD-OCT on presentation, and followed with serial imaging after treatment with corticosteroids and/or immunomodulatory therapy. Results: Spectral OCT through the lesions revealed nodular hyperreflective processes obscuring the retinal layers or optic cup, with local structural changes, including subretinal and intraretinal fluid. After treatment with corticosteroids and/or immunosuppression in 4 followed patients, all lesions regressed with improvement in associated structural changes, but did not entirely disappear. Conclusions: Spectral OCT can be useful in identifying lesion morphology and location, and in tracking the response to treatment in eyes with posterior-segment nodules, presumably secondary to sarcoidosis. Keywords: Granuloma, nodule, OCT, optic nerve, retina, sarcoidosis
MATERIALS AND METHODS
Sarcoidosis is characterized histologically by the presence of noncaseating granulomas involving multiple organs, including the lungs, skin, and eyes. Though the most common ocular manifestation is anterior uveitis, posterior findings including panuveitis, retinal vasculitis, and more rarely, choroidal and retinal granulomas, can be seen.1–3 With the advent of spectral-domain optical coherence tomography (SD-OCT, Heidelberg Engineering, Germany), high-resolution imaging of the retina has enabled improved understanding of posteriorsegment pathology. Spectral OCT and enhanceddepth imaging have been formerly utilized to characterize presumed sarcoid choroidal granulomas and preretinal nodular lesions,4–6 but here we utilize SD-OCT for the first time to study the extrachoroidal targets of inflammatory nodules, within the retina and optic nerve, in patients with sarcoidosis.
We conducted a retrospective review of all patients diagnosed with sarcoidosis and clinically detected nodules of the optic nerve or retina. All patients except one had an established diagnosis of sarcoidosis upon presentation, and the remaining patient (case 5) acquired a diagnosis following systemic workup guided by the ophthalmic findings. Diagnoses of sarcoidosis were based on tissue biopsy (cases 3–5) or imaging, including gallium scanning or chest radiography showing hilar adenopathy (cases 1, 2). All included patients reported a chronic uveitis history ranging from months to years. Upon presentation, all patients underwent a complete serologic workup to exclude alternative infectious etiologies of nodular lesions, including syphilis and tuberculosis. All patients had best-corrected Snellen visual acuity determined and all were examined with slit-lamp
Received 13 June 2014; revised 21 September 2014; accepted 29 September 2014; published online 29 October 2014 Correspondence: Naomi R. Goldberg, MD, PhD, Department of Ophthalmology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029, USA. E-mail: [email protected]
1
2
N. R. Goldberg et al.
biomicroscopy, as well as fundus photography and SD-OCT of identified lesions, during the active phases of their diseases. Patients were treated for their uveitis with corticosteroids and/or immunosuppression for variable amounts of time, and repeat eye-tracked OCT images over identified lesions were again obtained when the uveitis quieted. Follow-up analysis was available for 4 of 5 patients. The study had approval by the Mount Sinai School of Medicine institutional review board and the Program for the Protection of Human Subjects.
Downloaded by [University of Florida] at 14:33 13 November 2015
RESULTS On presentation, SD-OCT through retinal lesions revealed nodular hyperreflective intraretinal processes protruding into the vitreous, with disorganized retinal architecture, reduced choroidal transmission, and associated structural change, such as intraretinal or subretinal fluid. The retinal pigment epithelium (RPE) appeared elevated at the base of these lesions, but the underlying choroidal contour appeared intact. The disc lesions were seen as hyperreflective masses obscuring the central cup, with adjacent peripapillary retinal edema and disturbed retinal anatomy. After treatment in all followed eyes, the lesions consolidated and diminished in height, the exudative changes decreased or resolved, and the anatomy of the neighboring tissues improved. No lesion completely disappeared following treatment.
Report of Cases Case 1 A 62-year-old female on 30 mg alternate daily use of prednisone for neurosarcoidosis was referred for uveitis management with a complaint of ongoing right eye distortion. On examination, visual acuity was 20/25 and there were 2 discrete elevated intraretinal nodules along the inferior arcade, with subretinal fluid and trace vitritis. She was treated with 3 g daily mycophenolate, and the prednisone was transiently increased to 60 mg daily and tapered to 5 mg daily with improvement of the acuity to 20/20, regression of the lesions by 1 year with a more confluent appearance, and resolution of the fluid, as detected by SD-OCT (Figures 1A, 2A-B).
Case 2 A 25-year-old male with longstanding sarcoid uveitis reported progressively decreasing vision in both eyes. Visual acuity was 20/80 in the right eye and 20/200 in the left eye, and there were posterior synechiae and cataract obscuring visualization of the fundus. Spectral OCT highlighted bilateral peripapillary intraretinal nodules disturbing the retinal architecture with RPE elevation at the base of the lesions, and subretinal fluid in the right eye (not shown). Following 60 mg prednisone and taper to 10 mg daily, the acuity improved to 20/40 in the right eye,
FIGURE 1. Various sites and morphologies of inflammatory nodules, by spectral-domain optical coherence tomography (SD-OCT), in patients with sarcoidosis. Color photographs and SD-OCT scans over sections of presumed sarcoid nodules (black lines). (A, B) Retinal lesions in cases 1 (A) and 3 (B), shown by SD-OCT as hyperreflective nodular intraretinal processes obscuring the retinal layers, with posterior shadowing. The pigment epithelium is slightly elevated at the base of the lesion (B, arrow) and the underlying choroid is flat. (C, D) Optic nerve nodules in cases 4 (C) and 5 (D), seen as hyperreflective masses obscuring the optic cup; adjacent choroidal granulomatous infiltration is also seen (C, arrow). Ocular Immunology & Inflammation
Downloaded by [University of Florida] at 14:33 13 November 2015
OCT of Sarcoid Retinal and Optic Nerve Nodules
3
FIGURE 2. Morphologic changes of nodular lesions following treatment, with eye-tracked spectral-domain optical coherence tomography, and corresponding fundus photographs (insets). (A) Intraretinal nodules in case 1 with subretinal fluid extending subfoveally, on presentation. (B) Following treatment with prednisone and mycophenolate, the fluid resolved (4 weeks), and lesions regressed with residual scarring (1 year). (C) Optic nerve nodule in case 5 on presentation, with adjacent retinal edema and secondary attenuation of the photoreceptor ellipsoid band (arrow). Following (D) 2 and (E) 12 months of prednisone, the lesion height decreased with residual gliosis and normalization of the adjacent peripapillary outer-retinal anatomy (arrow).
the lesions consolidated, and subretinal fluid resolved within 4 months. Case 3 A 30-year-old patient with a 6-month uveitis history presented with 20/20 visual acuity in the left eye, mild vitritis, and a yellow peripapillary intraretinal nodule (Figure 1B). Prednisone was initiated at 60 mg and tapered to 7.5 mg daily, with reduction of the lesion height within 9 months, as confirmed by SD-OCT. Case 4 A 41-year-old female on intermittent corticosteroids for chronic sarcoid uveitis had counting fingers acuity in the left eye. The eye was aphakic and there was an ill-defined yellowish lesion surrounding the nerve. Spectral OCT confirmed the presence of an infiltrative lesion within the optic nerve and adjacent choroid, with thickening of the neighboring peripapillary retina and attenuation of the photoreceptor ellipsoid zone (Figure 1C). !
2014 Informa Healthcare USA, Inc.
Case 5 A 38-year-old male with a 3-month uveitis history in the left eye, presented with 20/50 acuity, and examination revealing vitritis, a whitened optic nerve lesion, and cotton-wool spots. Spectral OCT showed an elevated hyperreflective central lesion of the optic cup with adjacent retinal edema. He received 1 g daily intravenous methylprednisolone for 3 days, followed by prednisone, tapered to 5 mg daily. The acuity improved to 20/20 within 6 weeks and the disc nodule gradually regressed, with normalization of the neighboring retinal anatomy, as seen with SD-OCT (Figures 1D, 2C–E).
DISCUSSION Sarcoid granulomas of the optic nerve and retina have been described histopathologically and presumptively illustrated clinically,1–3 and in this series, SD-OCT was used for in vivo characterization of the various sites, morphologies, and treatment responses
Downloaded by [University of Florida] at 14:33 13 November 2015
4
N. R. Goldberg et al.
of inflammatory nodules, the likely clinical correlates of granulomas, in patients with sarcoidosis. The OCT features of the retinal nodules here (cases 1–3) reflect formerly described histologic features of granulomas, including retinal thickening and disorganized retinal architecture by foci of accumulated perivascular epithelioid cells (possibly implicating the retinal vessels as a conduit for inflammation), as well as occasional subpigment epithelial granulomas in continuity with intraretinal granulomas with choroidal sparing.1 In addition, non-necrotizing granuloma emerging from the surface of the optic disc into the vitreous has been described,1 and likely correlates to the SD-OCT findings of hyperreflective masses obscuring the optic cup, as in cases 4 and 5. Though enhanced-depth imaging OCT was not utilized to study the choroid in these cases, the choroidal contour could be assessed with standard SD-OCT, and appeared to be normal in all eyes except for case 4, in which the peripapillary choroid appeared infiltrated with a dome-shaped configuration. Clearly, presumed sarcoid retinal and disc granulomas may exist either in isolation or in combination with choroidal granulomas, and need not necessarily extend from choroidal infiltration. Isolated Toxocara granulomas of the optic nerve and retina have also been documented by SD- or high-penetration OCT, and have shown similar findings of a superficial mass filling the optic cup7 or an elevated intraretinal lesion protruding from the inner retina into the vitreous, receding with treatment.8 Clinical examination of nodular masses, with attention to lesion position in reference to neighboring vessels, is usually sufficient in distinguishing choroidal from retinal involvement, and fluorescein angiography can facilitate delineation of those tissues involved. Yet, SD-OCT offers a noninvasive and rapid method of confirming the targeted tissues and associated structural changes, thereby facilitating the staging of disease severity, and tracking of treatment response. Moreover, SD-OCT is helpful in highlighting sites of pathology in eyes with clinically indistinct lesions or obscured visibility, as shown in cases 2 and 4. The ability to ascertain the level of tissue involvement of these lesions and recognize their appearance by SD-OCT, may in some cases, help distinguish these inflammatory lesions from similar appearing oncologic lesions whose imaging characteristics are known,9 particularly in eyes without additional sign of inflammation or in patients with a negative evaluation for sarcoidosis. In a former sarcoid choroidal granuloma series, only a small minority of lesions, treated acutely, completely disappeared.10 Here, all lesions, present chronically, regressed, but none entirely resolved despite aggressive treatment, supporting the possibility that lesion chronicity limits treatment response;10 alternatively, retinal and disc lesions may be less
responsive than their choroidal counterparts, echoing former reports of poorer outcomes in eyes with presumed sarcoid optic nerve granulomas.2,10 The ability, with SD-OCT, to accurately follow the evolution of granuloma-like lesions in various locations clearly facilitates diagnosis and treatment planning in sarcoidosis patients. A larger comparative OCT series of presumed sarcoid granulomas of the choroid, disc, and retina, with standardized treatment and followup, would enhance our understanding of posteriorsegment pathogenesis in sarcoidosis.
ACKNOWLEDGMENTS We’d like to acknowledge Dr. Scott Brodie, M.D., Dr. Scott Forman, M.D., and Dr. Lori Tindel, M.D. for referring three patients in this series and for providing prior imaging studies and serologic workup.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. We’d like to acknowledge Scott Brodie, MD, Scott Forman, MD, and Lori Tindel, MD for referring three patients in this series and for providing prior imaging studies and serologic workup.
REFERENCES 1. Gass JD, Olson CL. Sarcoidosis with optic nerve and retinal involvement. Arch Ophthalmol. 1976;94:945–950. 2. Laties AM, Scheie HG. Sarcoid granuloma of the optic disk: evolution of multiple small tumors. Trans Am Ophthalmol Soc. 1970;68:219–233. 3. Brownstein S, Jannotta FS. Sarcoid granulomas of the optic nerve and retina: report of a case. Can J Ophthalmol. 1974;9: 372–378. 4. Rostaqui O, Querques G, Haymann P, et al. Visualization of sarcoid choroidal granuloma by enhanced depth imaging optical coherence tomography. Ocul Immunol Inflamm. 2014;22:239–241. 5. Modi YS, Epstein A, Bhaleeya S, et al. Multimodal imaging of sarcoid choroidal granulomas. J Ophthalmic Inflamm Infect. 2013;3:58. 6. Wong M, Janowicz M, Tessler HH, et al. High-resolution optical coherence tomography of presumed sarcoid retinal granulomas. Retina. 2009;29:1545–1546. 7. Chelnis J, Selvadurai AD, Fasiuddin A. Enhanced depth imaging and fundus autofluorescence of Toxocara optic nerve granuloma. Ocul Immunol Inflamm. 2013;21:82–83. 8. Hashida N, Nakai K, Nishida K. Diagnostic evaluation of ocular toxocariasis using high-penetration optical coherence tomography. Case Rep Ophthalmol. 2014;5:16–21. 9. Say EA, Shah SU, Ferenczy S, et al. Optical coherence tomography of retinal and choroidal tumors. J Ophthalmol. 2012;2012:385058. 10. Desai UR, Tawansy KA, Joondeph BC, et al. Choroidal granulomas in systemic sarcoidosis. Retina. 2001;21:40–47. Ocular Immunology & Inflammation
