Ocular Immunology & Inflammation, 2014; 22(6): 425–428 ! Informa Healthcare USA, Inc. ISSN: 0927-3948 print / 1744-5078 online DOI: 10.3109/09273948.2014.982060
EDITORIAL
Spectral-Domain-Optical Coherence Tomography in Uveitis Emmett T. Cunningham Jr,
1,2,3
, Mirjam E. J. van Velthoven, Manfred Zierhut, MD5
MD, PhD, MPH
MD, PhD
4
, and
1
Department of Ophthalmology, California Pacific Medical Center, San Francisco, CA, USA, 2The Department of Ophthalmology, Stanford University School of Medicine, Stanford, CA, USA, 3The Francis I. Proctor Foundation, UCSF School of Medicine, San Francisco, CA, USA, 4Uveitis and Medical Retina Service, Rotterdam Eye Hospital, Rotterdam, The Netherlands, and 5Centre for Ophthalmology, University Tuebingen, Tuebingen, Germany
Spectral-Domain-Optical Coherence Tomography (SD-OCT) has emerged in recent years as an extremely valuable, non-invasive adjunctive imaging tool in the diagnosis and management of patients with uveitis.1–3 Modern, high-resolution SD-OCT instruments create detailed cross-sectional and three-dimensional images capable of delineating abnormalities in the retina, choroid, and optic disc with a threshold resolution of 5–7 mm. Such detailed images are now used routinely to diagnose and monitor uveitic cystoid macular edema, to identify vitreomacular adhesions and traction, and to examine the integrity of the outer retinal hyper-reflective bands representing the external limiting membrane, the photoreceptor layer and the retinal pigment epithelium (RPE). Research in this area is extremely active, and focuses to a large extent on identifying and characterizing retinal and choroidal changes found in various uveitic conditions, as well as on the use of SD-OCT imaging in monitoring response to therapy and predicting long-term vision outcomes. Nine papers in this issue of Ocular Immunology & Inflammation, including five original articles4–8 and four letters,9–12 describe the use of high resolution OCT in patients with inflammatory diseases known to affect the eye. Both Ayhan Tuzu et al.4 and Kola et al.5 reported fundus findings in patients with ankylosing spondylitis (AS) using SD-OCT. Ayhan Tuzu et al.4 used a Cirrus SD-OCT to measure the thickness of the peripapillary retinal nerve fiber layer (RNFL), the central macula, and the ganglion cell-inner plexiform layers (GCIPL) in 40 patients with active AS and with no history of uveitis. Fifty age-matched healthy
subjects were used as controls. These thickness measurements made in 19 patients with relatively more advanced AS, defined as a Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) of 4, were then compared to 21 patients with a BASDAI54. The authors found no significant differences in their thickness measurements when comparing AS and control patients, but were able to detect small differences in the thickness of both the temporal RNFL and the GCIPL in AS patients with relatively more (BASDAI 4) versus less (BASDAI 54) advanced disease (nominal p = 0.04 and 0.03, respectively; uncorrected for multiple testing). Based on this analysis, the authors suggested that the degree and duration of systemic immune activation may have contributed to the RNFL and GCIPL thinning observed. It is worth noting, however, that the observed relative thinning was modest, with a mean of 8 mm or 11.1% thinning for the RNFL and 6 mm or 6.9% thinning for the GCIPL. As the authors correctly stated, further exploration of the clinical relevance of these findings is warranted. Kola et al.5 used Optovue RTVue SD-OCT to evaluate the retinal thickness and volume of the central macula, as well as the thickness of the peripapillar RNFL, ganglion cell complex, and choroid in 168 eyes of 84 patients with AS and no history of uveitis. Sixty-three sex- and age-matched healthy subjects served as controls. The patients with AS were described as having long-standing disease (mean 7.7 ± 4.5 years). The authors found no differences in retinal thickness or average RNFL measurements, but did detect a modest average increase in choroidal thickness in patients with AS of 40.3 mm
Correspondence: Dr. Emmett T. Cunningham Jr, West Coast Retina Medical Group, 1445 Bush Street, San Francisco, CA 94109, USA. Tel: 415-972-4600. Fax: 415-975-0999. E-mail: [email protected]
425
426 E. T. Cunningham et al. (14.1% increase; nominal p = 0.001, uncorrected for multiple testing). In contrast to the previous study by Ayhan Tuzu et al.,4 the authors found that retinal and RNFL thickness were not affected by AS, and observed no correlation between choroidal thickness, BASDAI score and disease duration in patients with AS. The proportions of patients with high versus low BASDAI scores were not disclosed. Others have described relative thickening of the retina in patients with AS and active uveitis,13–18 and that the thickening tends to both increase with the severity of uveitis and resolve with remission.14,17,18 Zinkernagel et al.6 used a Heidelberg Spectralis SD-OCT to evaluate structural changes in the retina in nine patients five months following treatment for severe exogenous endophthalmitis following cataract surgery. All patients received intravitreal vancomycin (1 mg/0.1 ml) and amikacin (400 micrograms/0.1 ml) followed within 12 h by pars plana vitrectomy and a second injection of vancomycin (1 mg/0.1 ml) along with dexamethasone (4 mg/0.1 ml). Culture of anterior chamber tap fluid obtained from seven subjects showed no growth in five patients, growth of coagulase negative Staphylococcus in two patients, and growth of Staphylococcus aureus in one patient. Vision at the five-month post-presentation visit ranged from 20/40 to 20/400, with a median of 20/40. A variety of specific structural abnormalities were noted by the authors, including thickening of the vitreoretinal interface, focal RNFL and inner nuclear layer defects, alterations in Henle’s layer, and areas of atrophy of the outer retinal layers. Comparison of average thickness of isolated retinal layers from the affected and fellow eyes showed no difference in the thickness of the RNFL, ganglion cell, inner nuclear, or outer nuclear layers. This was perhaps not unexpected given that both the culture results and five-month post-presentation visions suggested that these particular nine cases of exogenous post-operative endophthalmitis may have been relatively mild. Erdurmu¸s et al.7 used Heidelberg Spectralis SD-OCT to measure retinal and choroidal thickness in 30 patients with familial Mediterranean fever (FMF), an autosomal recessive autoinflammatory disorder characterized by recurrent attacks of fever and polyserositis. Twenty-eight healthy sex- and agematched children were used as controls. Only five of 30 patients (16.7%) were imaged during an acute systemic attack, and no patients were noted to have active ocular inflammation. It is perhaps not surprising, therefore, that the authors observed no abnormalities on SD-OCT in patients with FMF and no significant difference in the thickness of the retina or choroid between the two groups. Ecsedy et al.8 used a StratusOCT to describe Time Domain-OCT features in two patients with Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes (POEMS)
syndrome, a rare multisystem disorder known to occur in the setting of plasma cell dyscrasia. One patient also had multicentric Castelman’s disease, a similarly rare and heterogeneous group of lymphoproliferative disorders known to occur in approximately one in seven patients with POEMS syndrome. Both patients were in their sixth decade and were noted to have chronic bilateral optic disc edema associated with peripapillary retinal thickening and both neurosensory subfoveal macular detachment and intraretinal fluid. One patient developed unilateral peripapillary choroidal neovascularization and bilaterally enlarged blind spots on automated perimetry testing. The authors concluded that OCT imaging provides a useful adjunct to the evaluation and long-term monitoring of patients with POEMS syndrome. Symeonidis et al.9 used Cirrus SD-OCT to evaluate a 52-year-old man with idiopathic multifocal choroiditis (MFC) associated with subretinal fibrosis. Imaging through the lesions at presentation showed retinal thickening with both subretinal and intraretinal fluid and associated areas of hyper-reflectivity in the subretinal space corresponding to subretinal fibrosis seen clinically. The retinal thickening and fluid persisted and the subretinal fibrosis expanded over time despite therapy, which included both intravenous and oral prednisone, systemic cyclosporine A, and three intravitreal injections of ranubizumab. Similar OCT characteristics were reported by Jhaveri et al.,19 who described SD-OCT findings in a patient with presumed idiopathic MFC with subretinal fibrosis, and Zhao et al.,20 who analyzed the evolution of subretinal fibrosis in patients with VogtKoyanagi-Harada (VKH) disease, a recognized, albeit uncommon, complication of this condition.21,22 Hirooka et al.10 used Enhanced-Depth ImagingOCT (EDI-OCT) together with Laser Speckle FlowGraphy (LSFG) and calculated Mean Blur Rate (MBR) – a quantitative index of blood flow – in an 11year-old girl with Acute Macular Neuroretinopathy (AMN), a rare disorder characterized by the acute appearance of wedge-shaped reddish-brown lesions near the fovea. Diagnostic testing showed increased choroidal thickness and decreased choroidal blood flow in the areas of the active lesion. Additional SDOCT imaging findings through the lesion revealed disruption of the outer retinal hyper-reflective bands and an underlying area of hypo-reflectivity of the inner choroid. Whereas local choroidal blood flow increased and the integrity of the outer retinal hyperreflective bands improved over time, the choroidal thickness and reflectivity findings noted initially remained unchanged. The authors noted that such choroidal changes have not been reported in patients with AMN, but are consistent with acute choroidal inflammation seen in other setting, including both VKH disease23 and serpiginous choroiditis.24,25 Ocular Immunology & Inflammation
Spectral-Domain-Optical Coherence Tomography in Uveitis Shalchi et al.11 used SD-OCT to evaluate two women with Bilateral Diffuse Uveal Melanocytic Proliferation (BDUMP), a rare paraneoplastic disorder characterized by the occurrence of polygonal patches of RPE atrophy surrounded by aggregates of hypertrophied RPE cells – so called ‘‘giraffe pattern’’, diffuse thickening of the choroid with heavily pigmented melanocytes underlying areas of RPE atrophy, and rapidly progressive cataracts. Imaging in each patient showed localized disruption of the outer retinal hyper-reflective bands associated with a shallow serous retinal detachment. Both patients were found to have gynecological carcinomas prior to presentation – supporting the known association between BDUMP and reproductive tract malignancies in women.26 Whereas the RPE was diffusely thickened in one patient, imaging in the second patient revealed focal areas of RPE atrophy. The authors interpreted these findings as supportive of the notion that BDUMP represents a paraneoplastic condition characterized by the development of anti-RPE autoantibodies. Piri et al.12 used Heidelberg Spectralis SD-OCT imaging to evaluate a patient with in Acute Idiopathic Blind Spot Enlargement (AIBSE) syndrome and noted focal disruption of the external limiting membrane temporal to the fovea – in an area of localized attenuation on multifocal electroretinography (mfERG) testing. The authors suggested that these changes may have been related to transient outer retinal or choroidal inflammation. Together, these studies highlighted the utility of SD-OCT imaging in patients with uveitis. While the findings presented here were largely exploratory, and were as diverse as the diseases described, the promise of SD-OCT imaging is clearly great – both for research and clinical practice.
DECLARATION OF INTEREST This study was supported in part by The Pacific Vision Foundation (ETC) and The San Francisco Retina Foundation (ETC). The authors have no financial conflicts to declare.
REFERENCES 1. Regatieri CV, Alwassia A, Zhang JY, et al. Use of optical coherence tomography in the diagnosis and management of uveitis. Int Ophthalmol Clin. 2012;52:33–43. 2. Pakzad-Vaezi K, Or C, Yeh S, Forooghian F. Optical coherence tomography in the diagnosis and management of uveitis. Can J Ophthalmol. 2014;49:18–29. 3. Onal S, Tugal-Tutkun I, Neri P, P Herbort C. Optical coherence tomography imaging in uveitis. Int Ophthalmol. 2014;34:401–435. !
2014 Informa Healthcare USA, Inc.
427
4. Ayhan Tuzcu E, Ustun N, Ilhan N, et al. Peripapillary retinal nerve fiber layer and ganglion cell-inner plexiform layers thickness in ankylosing spondylitis. Ocul Immunol Inflamm. 2014;22:429–433. 5. Kola M, Kalkisim A, Karkucak M, et al. Evaluation of choroidal thickness in ankylosing spondylitis using optical coherence tomography. Ocul Immunol Inflamm. 2014;22: 434–438. 6. Zinkernagel MS, Dysli C, Wolf S, Ebneter A. Spectraldomain optical coherence tomography findings after severe exogenous endophthalmitis. Ocul Immunol Inflamm. 2014;22:439–443. 7. Erdurmu¸s M, Bekda¸s M, Demirciog˘lu F, et al. Retinal and choroidal thickness in children with familial mediterranean fever. Ocul Immunol Inflamm. 2014;22:444–448. 8. Ecsedy M, Schneider M, Nemes J, et al. optical coherence tomography features of POEMS syndrome and Castleman disease-associated papillopathy. Ocul Immunol Inflamm. 2014;22:454–460. 9. Symeonidis C, Dastiridou A, Konidaris V, et al. Subretinal fibrosis and uveitis: a spectral domain OCT Study of its evolution and the minimal therapeutic effect of the offlabel treatment with ranibizumab. Ocul Immunol Inflamm. 2014;22:449–453. 10. Hirooka K, Saito W, Noda K, Ishida S. Enhanced-depth imaging optical coherence tomography and laser speckle flowgraphy in a patient with acute macular neuroretinopathy. ocul immunol inflamm. 2014;22:485–489. 11. Shalchi Z, Shunmugam M, Mahroo OA, et al. Spectral domain optical coherence tomography findings in a case series of patients with bilateral diffuse uveal melanocytic proliferation. Ocul Immunol Inflamm. 2014;22:490–493. 12. Piri Niloofar, Kaplan H, Sigford D, Tezel T. High-defintion optical coherence tomography findings in acute idiopathic blind spot enlargement (AIBSE) syndrome. Ocul Immunol Inflamm. 2014;22:494–496. 13. Traill A, Stawell R, Hall A, Zamir E. Macular thickening in acute anterior uveitis. Ophthalmology. 2007;114:402. 14. Castellano CG, Stinnett SS, Mettu PS, et al. Retinal thickening in iridocyclitis. Am J Ophthalmol. 2009;148: 341–349. 15. Shulman S, Goldenberg D, Habot-Wilner Z, et al. Optical coherence tomography characteristics of eyes with acute anterior uveitis. Isr Med Assoc J. 2012;14:543–546. 16. Wexler A, Sand T, Elsa˚s TB. Bilateral macular thickening in mild unilateral anterior uveitis: is HLA-B27 involved? BMC Ophthalmol. 2012;12:30. 17. Balaskas K, Ballabeni P, Guex-Crosier Y. Retinal thickening in HLA-B27-associated acute anterior uveitis: evolution with time and association with severity of inflammatory activity. Invest Ophthalmol Vis Sci. 2012;53:6171–6177. 18. Moreno-Arrones JP, Gorron˜o-Echebarrı´a MB, TeusGuezala MA. Macular thickening in acute anterior uveitis with a 6-month remission period. Can J Ophthalmol. 2010; 45:91–92. 19. Jhaveri CD, Jampol LM, Van Gelder RN, Cunningham Jr ET. Diagnostic and therapeutic challenges. Retina. 2012;32: 1028–1032. 20. Zhao C, Zhang MF, Dong FT, et al. Spectral domain optical coherence tomography of Vogt-Koyanagi-Harada disease: novel findings and new insights into the pathogenesis. Chin Med Sci J. 2012;27:29–34. 21. Kuo IC, Rechdouni A, Rao NA, et al. Subretinal fibrosis in patients with Vogt-Koyanagi-Harada disease. Ophthalmology. 2000;107:1721–1728. 22. Lertsumitkul S, Whitcup SM, Nussenblatt RB, Chan CC. Subretinal fibrosis and choroidal neovascularization in Vogt-Koyanagi-Harada syndrome. Graefes Arch Clin Exp Ophthalmol. 1999;237:1039–1045.
428 E. T. Cunningham et al. 23. Hirose S, Saito W, Yoshida K, et al. Elevated choroidal blood flow velocity during systemic corticosteroid therapy in Vogt-Koyanagi-Harada disease. Acta Ophthalmol. 2008; 86:902–907. 24. Takahashi A, Saito W, Hashimoto Y, et al. Impaired circulation in the thickened choroid of a patient with serpiginous choroiditis. Ocul Immunol Inflamm. 2014;22: 409–413.
25. Cunningham Jr ET, Gupta A, Zierhut M. The creeping choroiditides – serpiginous and multifocal serpiginoid choroiditis. Ocul Immunol Inflamm. 2014;22: 345–348. 26. Rahimy E, Sarraf D. Paraneoplastic and nonparaneoplastic retinopathy and optic neuropathy: evaluation and management. Surv Ophthalmol. 2013; 58:430–458.
Ocular Immunology & Inflammation
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EDITORIAL
Spectral-Domain-Optical Coherence Tomography in Uveitis Emmett T. Cunningham Jr,
1,2,3
, Mirjam E. J. van Velthoven, Manfred Zierhut, MD5
MD, PhD, MPH
MD, PhD
4
, and
1
Department of Ophthalmology, California Pacific Medical Center, San Francisco, CA, USA, 2The Department of Ophthalmology, Stanford University School of Medicine, Stanford, CA, USA, 3The Francis I. Proctor Foundation, UCSF School of Medicine, San Francisco, CA, USA, 4Uveitis and Medical Retina Service, Rotterdam Eye Hospital, Rotterdam, The Netherlands, and 5Centre for Ophthalmology, University Tuebingen, Tuebingen, Germany
Spectral-Domain-Optical Coherence Tomography (SD-OCT) has emerged in recent years as an extremely valuable, non-invasive adjunctive imaging tool in the diagnosis and management of patients with uveitis.1–3 Modern, high-resolution SD-OCT instruments create detailed cross-sectional and three-dimensional images capable of delineating abnormalities in the retina, choroid, and optic disc with a threshold resolution of 5–7 mm. Such detailed images are now used routinely to diagnose and monitor uveitic cystoid macular edema, to identify vitreomacular adhesions and traction, and to examine the integrity of the outer retinal hyper-reflective bands representing the external limiting membrane, the photoreceptor layer and the retinal pigment epithelium (RPE). Research in this area is extremely active, and focuses to a large extent on identifying and characterizing retinal and choroidal changes found in various uveitic conditions, as well as on the use of SD-OCT imaging in monitoring response to therapy and predicting long-term vision outcomes. Nine papers in this issue of Ocular Immunology & Inflammation, including five original articles4–8 and four letters,9–12 describe the use of high resolution OCT in patients with inflammatory diseases known to affect the eye. Both Ayhan Tuzu et al.4 and Kola et al.5 reported fundus findings in patients with ankylosing spondylitis (AS) using SD-OCT. Ayhan Tuzu et al.4 used a Cirrus SD-OCT to measure the thickness of the peripapillary retinal nerve fiber layer (RNFL), the central macula, and the ganglion cell-inner plexiform layers (GCIPL) in 40 patients with active AS and with no history of uveitis. Fifty age-matched healthy
subjects were used as controls. These thickness measurements made in 19 patients with relatively more advanced AS, defined as a Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) of 4, were then compared to 21 patients with a BASDAI54. The authors found no significant differences in their thickness measurements when comparing AS and control patients, but were able to detect small differences in the thickness of both the temporal RNFL and the GCIPL in AS patients with relatively more (BASDAI 4) versus less (BASDAI 54) advanced disease (nominal p = 0.04 and 0.03, respectively; uncorrected for multiple testing). Based on this analysis, the authors suggested that the degree and duration of systemic immune activation may have contributed to the RNFL and GCIPL thinning observed. It is worth noting, however, that the observed relative thinning was modest, with a mean of 8 mm or 11.1% thinning for the RNFL and 6 mm or 6.9% thinning for the GCIPL. As the authors correctly stated, further exploration of the clinical relevance of these findings is warranted. Kola et al.5 used Optovue RTVue SD-OCT to evaluate the retinal thickness and volume of the central macula, as well as the thickness of the peripapillar RNFL, ganglion cell complex, and choroid in 168 eyes of 84 patients with AS and no history of uveitis. Sixty-three sex- and age-matched healthy subjects served as controls. The patients with AS were described as having long-standing disease (mean 7.7 ± 4.5 years). The authors found no differences in retinal thickness or average RNFL measurements, but did detect a modest average increase in choroidal thickness in patients with AS of 40.3 mm
Correspondence: Dr. Emmett T. Cunningham Jr, West Coast Retina Medical Group, 1445 Bush Street, San Francisco, CA 94109, USA. Tel: 415-972-4600. Fax: 415-975-0999. E-mail: [email protected]
425
426 E. T. Cunningham et al. (14.1% increase; nominal p = 0.001, uncorrected for multiple testing). In contrast to the previous study by Ayhan Tuzu et al.,4 the authors found that retinal and RNFL thickness were not affected by AS, and observed no correlation between choroidal thickness, BASDAI score and disease duration in patients with AS. The proportions of patients with high versus low BASDAI scores were not disclosed. Others have described relative thickening of the retina in patients with AS and active uveitis,13–18 and that the thickening tends to both increase with the severity of uveitis and resolve with remission.14,17,18 Zinkernagel et al.6 used a Heidelberg Spectralis SD-OCT to evaluate structural changes in the retina in nine patients five months following treatment for severe exogenous endophthalmitis following cataract surgery. All patients received intravitreal vancomycin (1 mg/0.1 ml) and amikacin (400 micrograms/0.1 ml) followed within 12 h by pars plana vitrectomy and a second injection of vancomycin (1 mg/0.1 ml) along with dexamethasone (4 mg/0.1 ml). Culture of anterior chamber tap fluid obtained from seven subjects showed no growth in five patients, growth of coagulase negative Staphylococcus in two patients, and growth of Staphylococcus aureus in one patient. Vision at the five-month post-presentation visit ranged from 20/40 to 20/400, with a median of 20/40. A variety of specific structural abnormalities were noted by the authors, including thickening of the vitreoretinal interface, focal RNFL and inner nuclear layer defects, alterations in Henle’s layer, and areas of atrophy of the outer retinal layers. Comparison of average thickness of isolated retinal layers from the affected and fellow eyes showed no difference in the thickness of the RNFL, ganglion cell, inner nuclear, or outer nuclear layers. This was perhaps not unexpected given that both the culture results and five-month post-presentation visions suggested that these particular nine cases of exogenous post-operative endophthalmitis may have been relatively mild. Erdurmu¸s et al.7 used Heidelberg Spectralis SD-OCT to measure retinal and choroidal thickness in 30 patients with familial Mediterranean fever (FMF), an autosomal recessive autoinflammatory disorder characterized by recurrent attacks of fever and polyserositis. Twenty-eight healthy sex- and agematched children were used as controls. Only five of 30 patients (16.7%) were imaged during an acute systemic attack, and no patients were noted to have active ocular inflammation. It is perhaps not surprising, therefore, that the authors observed no abnormalities on SD-OCT in patients with FMF and no significant difference in the thickness of the retina or choroid between the two groups. Ecsedy et al.8 used a StratusOCT to describe Time Domain-OCT features in two patients with Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes (POEMS)
syndrome, a rare multisystem disorder known to occur in the setting of plasma cell dyscrasia. One patient also had multicentric Castelman’s disease, a similarly rare and heterogeneous group of lymphoproliferative disorders known to occur in approximately one in seven patients with POEMS syndrome. Both patients were in their sixth decade and were noted to have chronic bilateral optic disc edema associated with peripapillary retinal thickening and both neurosensory subfoveal macular detachment and intraretinal fluid. One patient developed unilateral peripapillary choroidal neovascularization and bilaterally enlarged blind spots on automated perimetry testing. The authors concluded that OCT imaging provides a useful adjunct to the evaluation and long-term monitoring of patients with POEMS syndrome. Symeonidis et al.9 used Cirrus SD-OCT to evaluate a 52-year-old man with idiopathic multifocal choroiditis (MFC) associated with subretinal fibrosis. Imaging through the lesions at presentation showed retinal thickening with both subretinal and intraretinal fluid and associated areas of hyper-reflectivity in the subretinal space corresponding to subretinal fibrosis seen clinically. The retinal thickening and fluid persisted and the subretinal fibrosis expanded over time despite therapy, which included both intravenous and oral prednisone, systemic cyclosporine A, and three intravitreal injections of ranubizumab. Similar OCT characteristics were reported by Jhaveri et al.,19 who described SD-OCT findings in a patient with presumed idiopathic MFC with subretinal fibrosis, and Zhao et al.,20 who analyzed the evolution of subretinal fibrosis in patients with VogtKoyanagi-Harada (VKH) disease, a recognized, albeit uncommon, complication of this condition.21,22 Hirooka et al.10 used Enhanced-Depth ImagingOCT (EDI-OCT) together with Laser Speckle FlowGraphy (LSFG) and calculated Mean Blur Rate (MBR) – a quantitative index of blood flow – in an 11year-old girl with Acute Macular Neuroretinopathy (AMN), a rare disorder characterized by the acute appearance of wedge-shaped reddish-brown lesions near the fovea. Diagnostic testing showed increased choroidal thickness and decreased choroidal blood flow in the areas of the active lesion. Additional SDOCT imaging findings through the lesion revealed disruption of the outer retinal hyper-reflective bands and an underlying area of hypo-reflectivity of the inner choroid. Whereas local choroidal blood flow increased and the integrity of the outer retinal hyperreflective bands improved over time, the choroidal thickness and reflectivity findings noted initially remained unchanged. The authors noted that such choroidal changes have not been reported in patients with AMN, but are consistent with acute choroidal inflammation seen in other setting, including both VKH disease23 and serpiginous choroiditis.24,25 Ocular Immunology & Inflammation
Spectral-Domain-Optical Coherence Tomography in Uveitis Shalchi et al.11 used SD-OCT to evaluate two women with Bilateral Diffuse Uveal Melanocytic Proliferation (BDUMP), a rare paraneoplastic disorder characterized by the occurrence of polygonal patches of RPE atrophy surrounded by aggregates of hypertrophied RPE cells – so called ‘‘giraffe pattern’’, diffuse thickening of the choroid with heavily pigmented melanocytes underlying areas of RPE atrophy, and rapidly progressive cataracts. Imaging in each patient showed localized disruption of the outer retinal hyper-reflective bands associated with a shallow serous retinal detachment. Both patients were found to have gynecological carcinomas prior to presentation – supporting the known association between BDUMP and reproductive tract malignancies in women.26 Whereas the RPE was diffusely thickened in one patient, imaging in the second patient revealed focal areas of RPE atrophy. The authors interpreted these findings as supportive of the notion that BDUMP represents a paraneoplastic condition characterized by the development of anti-RPE autoantibodies. Piri et al.12 used Heidelberg Spectralis SD-OCT imaging to evaluate a patient with in Acute Idiopathic Blind Spot Enlargement (AIBSE) syndrome and noted focal disruption of the external limiting membrane temporal to the fovea – in an area of localized attenuation on multifocal electroretinography (mfERG) testing. The authors suggested that these changes may have been related to transient outer retinal or choroidal inflammation. Together, these studies highlighted the utility of SD-OCT imaging in patients with uveitis. While the findings presented here were largely exploratory, and were as diverse as the diseases described, the promise of SD-OCT imaging is clearly great – both for research and clinical practice.
DECLARATION OF INTEREST This study was supported in part by The Pacific Vision Foundation (ETC) and The San Francisco Retina Foundation (ETC). The authors have no financial conflicts to declare.
REFERENCES 1. Regatieri CV, Alwassia A, Zhang JY, et al. Use of optical coherence tomography in the diagnosis and management of uveitis. Int Ophthalmol Clin. 2012;52:33–43. 2. Pakzad-Vaezi K, Or C, Yeh S, Forooghian F. Optical coherence tomography in the diagnosis and management of uveitis. Can J Ophthalmol. 2014;49:18–29. 3. Onal S, Tugal-Tutkun I, Neri P, P Herbort C. Optical coherence tomography imaging in uveitis. Int Ophthalmol. 2014;34:401–435. !
2014 Informa Healthcare USA, Inc.
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4. Ayhan Tuzcu E, Ustun N, Ilhan N, et al. Peripapillary retinal nerve fiber layer and ganglion cell-inner plexiform layers thickness in ankylosing spondylitis. Ocul Immunol Inflamm. 2014;22:429–433. 5. Kola M, Kalkisim A, Karkucak M, et al. Evaluation of choroidal thickness in ankylosing spondylitis using optical coherence tomography. Ocul Immunol Inflamm. 2014;22: 434–438. 6. Zinkernagel MS, Dysli C, Wolf S, Ebneter A. Spectraldomain optical coherence tomography findings after severe exogenous endophthalmitis. Ocul Immunol Inflamm. 2014;22:439–443. 7. Erdurmu¸s M, Bekda¸s M, Demirciog˘lu F, et al. Retinal and choroidal thickness in children with familial mediterranean fever. Ocul Immunol Inflamm. 2014;22:444–448. 8. Ecsedy M, Schneider M, Nemes J, et al. optical coherence tomography features of POEMS syndrome and Castleman disease-associated papillopathy. Ocul Immunol Inflamm. 2014;22:454–460. 9. Symeonidis C, Dastiridou A, Konidaris V, et al. Subretinal fibrosis and uveitis: a spectral domain OCT Study of its evolution and the minimal therapeutic effect of the offlabel treatment with ranibizumab. Ocul Immunol Inflamm. 2014;22:449–453. 10. Hirooka K, Saito W, Noda K, Ishida S. Enhanced-depth imaging optical coherence tomography and laser speckle flowgraphy in a patient with acute macular neuroretinopathy. ocul immunol inflamm. 2014;22:485–489. 11. Shalchi Z, Shunmugam M, Mahroo OA, et al. Spectral domain optical coherence tomography findings in a case series of patients with bilateral diffuse uveal melanocytic proliferation. Ocul Immunol Inflamm. 2014;22:490–493. 12. Piri Niloofar, Kaplan H, Sigford D, Tezel T. High-defintion optical coherence tomography findings in acute idiopathic blind spot enlargement (AIBSE) syndrome. Ocul Immunol Inflamm. 2014;22:494–496. 13. Traill A, Stawell R, Hall A, Zamir E. Macular thickening in acute anterior uveitis. Ophthalmology. 2007;114:402. 14. Castellano CG, Stinnett SS, Mettu PS, et al. Retinal thickening in iridocyclitis. Am J Ophthalmol. 2009;148: 341–349. 15. Shulman S, Goldenberg D, Habot-Wilner Z, et al. Optical coherence tomography characteristics of eyes with acute anterior uveitis. Isr Med Assoc J. 2012;14:543–546. 16. Wexler A, Sand T, Elsa˚s TB. Bilateral macular thickening in mild unilateral anterior uveitis: is HLA-B27 involved? BMC Ophthalmol. 2012;12:30. 17. Balaskas K, Ballabeni P, Guex-Crosier Y. Retinal thickening in HLA-B27-associated acute anterior uveitis: evolution with time and association with severity of inflammatory activity. Invest Ophthalmol Vis Sci. 2012;53:6171–6177. 18. Moreno-Arrones JP, Gorron˜o-Echebarrı´a MB, TeusGuezala MA. Macular thickening in acute anterior uveitis with a 6-month remission period. Can J Ophthalmol. 2010; 45:91–92. 19. Jhaveri CD, Jampol LM, Van Gelder RN, Cunningham Jr ET. Diagnostic and therapeutic challenges. Retina. 2012;32: 1028–1032. 20. Zhao C, Zhang MF, Dong FT, et al. Spectral domain optical coherence tomography of Vogt-Koyanagi-Harada disease: novel findings and new insights into the pathogenesis. Chin Med Sci J. 2012;27:29–34. 21. Kuo IC, Rechdouni A, Rao NA, et al. Subretinal fibrosis in patients with Vogt-Koyanagi-Harada disease. Ophthalmology. 2000;107:1721–1728. 22. Lertsumitkul S, Whitcup SM, Nussenblatt RB, Chan CC. Subretinal fibrosis and choroidal neovascularization in Vogt-Koyanagi-Harada syndrome. Graefes Arch Clin Exp Ophthalmol. 1999;237:1039–1045.
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