1040-5488/15/9201-e12/0 VOL. 92, NO. 1, PP. e12Ye20 OPTOMETRY AND VISION SCIENCE Copyright * 2014 American Academy of Optometry
CLINICAL CASE
Focal Choroidal Excavation Associated with Focal Retinochoroiditis Tetsutaro Ohki*, Tsutomu Sakai*, and Hiroshi Tsuneoka*
ABSTRACT Purpose. To describe detailed spectral-domain optical coherence tomography (OCT) findings for two patients with focal choroidal excavation (FCE) associated with focal retinochoroiditis. Case Report. Three eyes from two patients with FCE associated with focal retinochoroiditis were evaluated by funduscopy, fluorescence angiography, indocyanine green angiography, and spectral-domain OCT during follow-up. Both patients with focal retinochoroiditis developed new FCE after oral steroid treatment and two eyes showed regression of the FCE during the follow-up. Both eyes from one patient transformed from the conforming to the nonconforming type and neither of the eyes were stable during the follow-up. Ultimately, all eyes exhibited the conforming-type FCE. Conclusions. Focal choroidal excavation can be seen as a tomographic phenotype after the treatment of focal retinochoroiditis. Spectral-domain OCT was useful for detecting the development of FCE after the treatment and for observing FCE regression. (Optom Vis Sci 2015;92:e12Ye20) Key Words: focal choroidal excavation, spectral-domain optical coherence tomography, focal retinochoroiditis, steroid
F
ocal choroidal excavation (FCE) is a newly recognized clinical entity that is revealed by optical coherence tomography (OCT) images.1 Recently, Obata et al.2 retrospectively reported 17 patients with FCE. The affected eyes usually have good visual acuity (VA) and lack a history of trauma, posterior uveitis, or retinal or choroidal vascular disease. Moreover, the lesions show little change over time. Thus, FCE was believed to only be a congenital malformation and to remain stable. However, most recent reports have shown that FCE is associated with choroidal neovascularization (CNV), polypoidal choroidal vasculopathy, and central serous chorioretinopathy (CSC), and is not always stable.3Y8 Spectral-domain (SD) OCT has better axial resolution than conventional time-domain OCT and is extremely useful in the evaluation of macular structures of the retina and choroid. Recent reports have revealed that SD-OCT facilitates the diagnosis of FCE.2Y9 In 2011, Margolis et al.9 expanded the spectrum of FCE and found that FCE can be classified into two types: conforming and nonconforming based on SD-OCT. They described that SDOCT revealed outer retinal layers conforming to retinal pigment epithelial alterations within the excavation, classified as the conforming type, and a separation between the outer retina and
*MD Department of Ophthalmology, Jikei University School of Medicine, Tokyo, Japan (all authors).
the retinal pigment epithelium (RPE) within the excavation, classified as the nonconforming type. They suggested the possibility of transformation from the conforming to the nonconforming type, although Obata et al.2 did not report any cases transforming from the conforming to the nonconforming type during the follow-up period. Serpiginous choroiditis is a clinically defined disorder characterized by destruction of the inner choroid and the RPE as well as secondary involvement of the retina.10 In about one-third of patients, the lesions are initially or exclusively at the macula. Such a pattern of choroiditis is recognized as macular serpiginous choroiditis. Recent SD-OCT studies of active serpiginous choroiditis have shown photoreceptor layer disruption associated with outer retinal and choriocapillaris hyperreflectivity10; however, there are no reports on the SD-OCT study of serpiginous choroiditis before and after the treatment. Herein, we describe the detailed appearance of FCE associated with focal retinochoroiditis in two patients (three eyes) using SDOCT. Both patients with focal retinochoroiditis developed new FCE after treatment with oral steroid, and two eyes showed regression of the FCE during the follow-up. Both eyes in one patient transformed from the conforming to the nonconforming type and none of the eyes were stable during the follow-up. We suggest that these cases represent the novel clinical findings of FCE and show how the findings provide further insight into this increasingly recognized inflammatory chorioretinal disorder.
Optometry and Vision Science, Vol. 92, No. 1, January 2015
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Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
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FIGURE 1. Fundus photography, FA, and ICGA of both eyes at the initial visit. Fundus photography showing some yellowish-white lesions in the right (A) and left (B) macula. Fluorescence angiography images showing weak hyperfluorescence corresponding to RPE alterations in the right (C) and left (I) macula. The hyperfluorescent area increased with time (D, E, J, K). The lesion demonstrated no early hypofluorescence or late hyperfluorescence. Indocyanine green angiography image showing hypofluorescence in all phases with late hyperfluorescence surrounding the area of hypofluorescence in the right (F to H) and left (L to N) macula. (I-) Early- to late-phase ICGA image showing no choroidal vascular hyperpermeability. Optometry and Vision Science, Vol. 92, No. 1, January 2015
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e14 Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
METHODS This observational case series evaluated patients who were diagnosed as having FCE in association with focal retinochoroiditis, based on clinical examination and diagnostic testing in a retinal practice. Each patient underwent a comprehensive ophthalmologic examination, including VA, intraocular pressure testing, slitlamp biomicroscopy, and dilated ophthalmoscopy. Visual acuity was evaluated using a Snellen chart on a scale of 0.1 to 1.5.
Patients also underwent color photography (VISCUM NM ProNM, Carl Zeiss Meditec, Dublin, CA), fluorescence angiography (FA) and indocyanine green angiography (ICGA; Heidelberg Retina Angiograph 2, Heidelberg Engineering, Heidelberg, Germany), and Cirrus SD-OCT scans (Carl Zeiss Meditec) for retinal imaging. Spectral-domain OCT scans were recorded using the HD 5-line raster scan protocol (horizontal scan of 6 mm). The OCT scans were reevaluated at a follow-up after several months. The study was
FIGURE 2. Spectral-domain OCT in both eyes at the initial (A, B) and follow-up (C to N) visits for FCE associated with focal retinochoroiditis. Horizontal SD-OCT scans showing focal excavation (arrow) and elevation (arrowhead) involving the outer retinal layers up to the ELM in the right eye (A). Disruption (arrow) and irregular elevation (arrowhead) of the RPE layer were noted in the parafovea of the left eye (B). A hyperreflective lesion with increased choroidal thickness (square bracket) was observed beneath the RPE line in both eyes (A, B). Spectral-domain OCT also revealed a disrupted ELM, ISe band, and OS/RPE, corresponding to the affected area (A, B). One week after the initiation of oral prednisolone, SD-OCT scan revealed a conforming FCE (arrow) with persistent disruption of the outer retina in the right eye (C) and a focal excavation and elevation involving the outer retinal layers up to the ELM in the left eye (D). Follow-up horizontal SD-OCT scan in the right eye showing FCE more clearly as the RPE band followed the contour of the choroidal excavation during follow-up, although a small area of outer retinal hyperreflectivity with a minimal amount of fluid collection was noted at 6 to 7 weeks (I, K). Follow-up SDOCT scan in the left eye demonstrated that a heterogeneously hyperreflective lesion was seen beneath the neurosensory retina with a small amount of fluid collection (arrow) at 4 weeks (H), after a formation of a conforming FCE was observed (F). Consecutive horizontal SD-OCT scans obtained at follow-up showed a change from the nonconforming to the conforming type with the resolution of the fluid and hyperreflective lesion (J, L, N). Ultimately, both eyes exhibited the conforming-type FCE (arrow) with subfoveal choroidal thinning (square brackets) (M, N). Optometry and Vision Science, Vol. 92, No. 1, January 2015
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Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
carried out with institutional approval and in accordance with the tenets of the Declaration of Helsinki.
RESULTS The age of the two patients was 32 and 54 years, with VAs ranging from 0.7 to 1.5. One patient had bilateral involvement. The follow-up in the two patients ranged from 3 to 4 months. The two patients with focal retinochoroiditis developed new FCE after oral steroid treatment and two eyes showed regression of the FCE during the follow-up. Both eyes from one patient transformed from the conforming to the nonconforming type and neither of the eyes were stable during the follow-up. Ultimately, all eyes exhibited the conforming-type FCE.
Case 1 A 32-year-old healthy woman with no medical or ocular history presented with visual disturbance in both eyes. Visual acuity was 0.7 with j3.50 sphere and 1.5 with j4.50 sphere in the right and left eye, respectively. There was no afferent pupillary defect. Slit-lamp examination was normal. No vitreous cells were present. There was no evidence of vitreous traction or posterior vitreous detachment. Ophthalmoscopy of both eyes revealed small, isolated, round, yellowish-white elevated lesions in the macula (Fig. 1A, B). Fluorescence angiography showed weak hyperfluorescence in the early phase and diffuse late staining and leakage of dye in both eyes (Fig. 1C to E, J, K). The lesions exhibited no early
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hypofluorescence and late hyperfluorescence. Indocyanine green angiography showed hypofluorescent areas that corresponded to the visible chorioretinal lesions from the early to the late phase (Fig. 1F to H, L to N). The hypofluorescent area observed with ICGA extended beyond the area of clinically observable lesions and that seen on FA. Spectral-domain OCT demonstrated focal excavation and elevation involving the outer retinal layers up to the external limiting membrane (ELM) in the right eye (Fig. 2A). Disruption and irregular elevation of the RPE layer were noted in the parafovea of the left eye (Fig. 2B). A hyperreflective lesion was observed beneath the RPE line in both eyes (Fig. 2A, B). Spectraldomain OCT also revealed a disrupted ELM, inner segment ellipsoid (ISe) band, and outer segment (OS)-RPE interdigitation (OS/RPE) corresponding to the affected area (Fig. 2A, B). Laboratory analysis showed a normal complete blood count, a negative QuantiFERON-TB Gold test, and a nonreactive rapid plasma reagin test. The patient had no syphilis and tuberculosis. The patient was diagnosed as having presumed macular serpiginous or ampiginous choroiditis of both eyes and treated initially with a moderate dose of prednisolone (30 mg/d) (0.6 mg/kg body weight). After 1 week of treatment, SD-OCT showed a conforming FCE with subfoveal choroidal thinning and the regression of the hyperreflective lesion in the right eye (Fig. 2C). By contrast, SD-OCT demonstrated focal excavation involving the outer retinal layers up to the ELM with a disruption of the RPE line and the hyperreflective lesion beneath the RPE line in the left eye (Fig. 2D). One week later, the VA improved to 1.2 along with almost complete recovery of the ELM (Fig. 2E). However, the patient presented with vision loss in the left eye. The VA was
FIGURE 3. Fundus photography, FA, and ICGA of both eyes at the resolution stage. Fundus photograph showing distinct yellow lesions in the right (A) and left (B) macula. Fluorescence angiography image showing focal hyperfluorescent spots nasal to the fovea in both eyes (C, E). Indocyanine green angiography image showing a hypofluorescent area with staining in the center of the lesion (D, F). No leakage was seen in midphase angiogram (D, E). Optometry and Vision Science, Vol. 92, No. 1, January 2015
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e16 Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
FIGURE 4. Fundus photography, FA, and ICGA of the left eye at the initial visit. Fundus photograph showing a yellowish-white fusiform lesion with pigmentary mottling in the left macula (A). Fluorescence angiography image showing faint circumferential hyperfluorescence with hypofluorescence in the center of the lesion (B). The hyperfluorescence increased with time (C). The lesion demonstrated no early hypofluorescence and late hyperfluorescence. Indocyanine green angiography image showing hypofluorescence in early to late phase with late hyperfluorescence surrounding the area of hypofluorescence. (D, E) Early- to late-phase ICGA image showing no choroidal vascular hyperpermeability.
0.8 along with persistent disruption of the photoreceptor layer. Spectral-domain OCT revealed a conforming FCE and regression of the hyperreflective lesion in the left eye (Fig. 2F). When the dose of oral prednisolone was decreased to 20 mg/d, photoreceptor integrity was completely disrupted with a small amount of fluid collection in the left eye (Fig. 2H) and the VA decreased to 0.5. One week after increasing the dose to 30 mg/d, incomplete reestablishment of the ELM was observed, despite an increase of the fluid (Fig. 2J) and the VA was 0.7. Spectral-domain OCT demonstrated a nonconforming FCE in the right eye (Fig. 2I, K). Two weeks later, vision in the patient’s left eye improved to 1.5 with the decrease of the fluid on SD-OCT (Fig. 2L). A minimal amount of fluid collection was noted in both eyes (Fig. 2K, L). At the end of the 12-week treatment period, the VA was 1.5, along with almost complete recovery of the ISe band and complete resorption of the fluid in both eyes (Fig. 2M, N). Three months after the treatment, fundus photography showed distinct gray lesions in the right (Fig. 3A) and left (Fig. 3B) macula. Fluorescence angiography image showed hyperfluorescent spots in both eyes (Fig. 3C, E). Indocyanine green angiography image showed late staining in the center and periphery of
the lesion (Fig. 3D, F). No leakage was seen in a midphase angiogram.
Case 2 A 54-year-old healthy man with no previous ocular history presented with a 3-week history of metamorphopsia in the left eye. Upon examination, VA was 1.0 and 0.5 with j2.75 sphere in the right and left eye, respectively. No afferent pupillary defect was present. Anterior segment examination was normal in both eyes. No vitreous cells were present in either eye. There was no evidence of vitreous traction or posterior vitreous detachment. Ophthalmoscopy of the left eye showed marked yellowish-white plaque-like lesion in the fovea (Fig. 4A). Fluorescence angiography showed early blockage with late hyperfluorescent borders that spread toward the center of the lesion (Fig. 4B), and ICGA demonstrated hypofluorescence of the lesions from the early to the late phase (Fig. 4C). The lesion did not exhibit early hypofluorescence or late hyperfluorescence. Ophthalmoscopic examination of the right eye was normal. Spectral-domain OCT of the left eye showed irregular elevation of the RPE line involving the
Optometry and Vision Science, Vol. 92, No. 1, January 2015
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Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
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FIGURE 5. Spectral-domain OCT in the left eye at the initial (A) and follow-up (B to J) visits for FCE associated with focal retinochoroiditis. Horizontal SD-OCT scans showing a focal elevation (arrowhead) involving the outer retinal layers up to the ELM at the initial visit (A). Irregular elevation of the RPE layer was noted in the fovea. A hyperreflective lesion (arrow) with increased choroidal thickness was seen beneath the RPE line (A, B). Spectral-domain OCT also revealed a disrupted ELM, ISe band, and OS/RPE corresponding to the affected area. One week after the initiation of oral prednisolone, SD-OCT scan revealed a focal excavation (arrow) with persistent disruption of outer retina (B). One week later, follow-up SD-OCT scan showing a nonconforming FCE (arrow) more clearly as the RPE band followed the contour of the choroidal excavation (C). Two weeks later, follow-up SD-OCT scan demonstrated discrete FCE with the decreased fluid and hyperreflective lesion (D). Two weeks later, SD-OCT showed almost complete reestablishment of the ELM and ISe band (E). When the dose of oral prednisolone was decreased to 12.5 mg/d, focal retinochoroiditis recurred. Spectral-domain OCT showed irregular elevation (arrowhead) of the RPE layer with hyperreflective lesion (arrow) beneath the RPE (F). One week after the treatment, SD-OCT demonstrated mild flattening of the RPE line, although the hyperreflective lesion was persistent (G). One week later, SD-OCT revealed restored ELM and ISe band with an increase of the hyperreflective lesion (H). Two weeks later, the follow-up SD-OCT demonstrated a conforming FCE (arrow) with a reduction of the hyperreflective lesion (I). Fourteen weeks after the treatment, best-corrected VA was 1.5 along with almost complete recovery of the ELM and ISe band (J).
outer retinal layers up to the ELM (Fig. 5A). A hyperreflective lesion was seen beneath the RPE line. Spectral-domain OCT also revealed a disrupted ELM, ISe band, and OS/RPE corresponding to the affected area. Laboratory analysis showed a normal complete blood count, a negative QuantiFERON-TB Gold test, and a
nonreactive rapid plasma reagin test. The patient had no syphilis and tuberculosis. The patient was diagnosed as having presumed macular serpiginous or ampiginous choroiditis of the left eye and was treated initially with a moderate dose of prednisolone (30 mg/d) (0.5 mg/kg
Optometry and Vision Science, Vol. 92, No. 1, January 2015
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e18 Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
FIGURE 6. Fundus photography, FA, and ICGA of both eyes at the resolution stage. Fundus photography showing distinct yellowish-white lesions in the left macula (A). Fluorescence angiography image showing a focal hyperfluorescent spot in the left eye (B). Indocyanine green angiography image showing a hypofluorescent area with a staining spot in the center (C).
body weight). After 1 week of treatment, SD-OCT revealed a nonconforming FCE with regression of the hyperreflective lesion beneath the RPE (Fig. 5B). The VA improved to 0.9 along with complete reestablishment of ELM on SD-OCT. When the dose of oral prednisolone was tapered to 25 mg/d, the ISe band was restored mildly, although the subretinal fluid was increased (Fig. 5C). Two weeks later, SD-OCT showed further resolution of the ISe band with reduction of the FCE and fluid (Fig. 5D). Ophthalmoscopic examination demonstrated reduction of the yellowishwhite plaque lesion. Two weeks later, SD-OCT showed almost complete reestablishment of the ELM and ISe band (Fig. 5E). The VA had improved to 1.2 in the left eye. When the dose of oral prednisolone was decreased to 12.5 mg/d, SD-OCT showed irregular elevation of the RPE layer with a hyperreflective lesion beneath the RPE in the left eye, similar to the initial findings (Fig. 5F), and the VA was 1.2. The right eye remained unaffected. The patient was diagnosed as having recurrent macular serpiginous or ampiginous choroiditis in the left eye and was treated with an increased dose of prednisolone (30 mg/d). One week later, SD-OCT demonstrated mild flattening of the RPE line, although the hyperreflective lesion remained persistent (Fig. 5G). The VA decreased to 1.0. One week later, SD-OCT revealed restoration of the ELM and ISe band, with a concomitant increase of the hyperreflective lesion (Fig. 5H). The VA
increased to 1.5. Two weeks later, follow-up SD-OCT demonstrated a conforming FCE with reduction of the hyperreflective lesion (Fig. 5I). Fourteen weeks after the treatment, the VA was 1.5 with almost complete recovery of the ELM and ISe band (Fig. 5J). Three months after the treatment, fundus photography showed distinct grayish-white lesions in the left eye (Fig. 6A). Fluorescence angiography imaging showed some hyperfluorescent spots in the left eye (Fig. 6B). Indocyanine green angiography imaging showed hypofluorescence with late staining in the center of the lesion (Fig. 6C). No leakage was seen in a midphase angiogram.
DISCUSSION Herein, we present a first case series of FCE associated with focal retinochoroiditis. Two patients with focal retinochoroiditis developed new FCE after oral steroid treatment and two eyes showed regression of the FCE during follow-up. Both eyes of one patient transformed from the conforming to the nonconforming type and none of the eyes were stable during the follow-up. Additionally, tomographic and angiographic abnormalities that were observed in the photoreceptor layer, RPE, and choroid in the acute phase of the disease regressed within a few months after oral steroid therapy. Thus, SD-OCT provided a definitive diagnosis of the FCE associated with inflammatory chorioretinal
Optometry and Vision Science, Vol. 92, No. 1, January 2015
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Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
disease, the associated outer retinal and choroidal morphological abnormalities, and the ability to document improvement. A previous report has suggested that FCE is a congenital malformation that can remain stable without any change.9 In our patients, all eyes developed new FCE and showed slow regression of FCE during the treatment and follow-up. In one eye, FCE remained stable without any change in size or shape during the resolution stage. Although many cases of FCE may be a stable congenital choroidal malformation, cases of FCE associated with chorioretinal disorders, such as CNV, polypoidal choroidal vasculopathy, CSC, and retinochoroiditis, are not stable and may change in size or shape during the treatment follow-up. Although previous studies have suggested that FCE might form because of RPE retraction attributed to focal scarring of the choroidal connective tissue,3Y8 in the current cases, we suggest that the formation of the FCE may be associated with focal contractive changes in the choroidal parenchyma after treatment of focal retinochoroiditis. Margolis et al.9 expanded the spectrum of this entity by analyzing a series of 12 patients with FCE. They9 categorized FCE into nonconforming and conforming types and suggested the possibility of transformation from the conforming to the nonconforming type, although Obata et al.2 had no cases that transformed from the conforming to the nonconforming type during their follow-up period. Recently, Ellabban et al.4 and Xu et al.6 reported that nonconforming FCE changed to the conforming type after successful treatment of CSC or CNV, respectively. In our patients, two eyes exhibited changes from the conforming to the nonconforming type and one eye changed from the nonconforming to the conforming type, although all eyes ultimately transformed to the conforming type after successful treatment. Thus, further understanding of the histopathological nature of FCE during the treatment follow-up will necessitate the accumulation of more cases. A recent study of 3D-OCT image analysis revealed that FCE may be a feature associated with choroidal thickening.4 The study showed that subfoveal choroidal thickness in eyes with FCE was greater than that observed in normal subjects, whereas foveal choroidal thickness in CSC eyes with FCE has been shown to be significantly thinner than that in CSC eyes without FCE. In our patients, SD-OCT image demonstrated that subfoveal choroidal thickness in eyes with FCE associated with inflammatory chorioretinal disorders was different between the active and resolution stages of inflammation. In the active inflammatory stage, SD-OCT revealed choroidal thickening, and in the resolution stage after steroid treatment, SD-OCT showed the FCE with marked decrease in subfoveal choroidal thickness. We suggest that a regression of inflammatory material, such as fibrin, after the treatment of acute choroidal inflammation, may result in choroidal thinning in the resolution stage. The improvement of the damaged photoreceptor integrity associated with choroidal abnormalities noted herein may be common to some clinical conditions. For example, somewhat similar improvements have been noted in cases of multifocal choroiditis,11,12 punctate inner choroidopathy,11,12 and serpiginous choroiditis.13 These cases featured hyperreflective lesions involving the photoreceptor layer on OCT, which could represent a localized inflammatory response. Given that the patients in this
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series were treated successfully with oral steroid, it may be possible that the retinal changes demonstrated on OCT represent an early transient manifestation of a localized inflammatory condition. Taken together, they may be involved in inflammatory conditions, such as multifocal choroiditis, punctate inner choroidopathy, and serpiginous choroiditis. In conclusion, the patients in this case series exhibited FCE associated with focal retinochoroiditis. Although the FCE might be related to the pathogenesis of focal retinochoroiditis, its role remains unknown. Spectral-domain OCT was extremely useful in the detection of FCE and in understanding the morphological changes of the outer retina and choroid during follow-up of inflammatory chorioretinal disorders.
ACKNOWLEDGMENTS The authors have no financial support or conflict of interest in this study. Contributions of authors: Design and conduct of study (TS); collection of data (TO); management, analysis, and interpretation of data (TO, TS, and HT); and preparation, review, and approval of the manuscript (TO, TS, and HT). Received June 30, 2014; accepted October 3, 2014.
REFERENCES 1. Jampol LM, Shankle J, Schroeder R, Tornambe P, Spaide RF, Hee MR. Diagnostic and therapeutic challenges. Retina 2006;26: 1072Y6. 2. Obata R, Takahashi H, Ueta T, Yuda K, Kure K, Yanagi Y. Tomographic and angiographic characteristics of eyes with macular focal choroidal excavation. Retina 2013;33:1201Y10. 3. Say EA, Jani PD, Appenzeller MF, Houghton OM. Focal choroidal excavation associated with polypoidal choroidal vasculopathy. Ophthalmic Surg Lasers Imaging Retina 2013;44: 409Y11. 4. Ellabban AA, Tsujikawa A, Ooto S, Yamashiro K, Oishi A, Nakata I, Miyake M, Akagi-Kurashige Y, Ueda-Arakawa N, Arichika S, Yoshitake S, Takahashi A, Yoshimura N. Focal choroidal excavation in eyes with central serous chorioretinopathy. Am J Ophthalmol 2013;156:673Y83. 5. Suzuki M, Gomi F, Hara C, Sawa M, Nishida K. Characteristics of central serous chorioretinopathy complicated by focal choroidal excavation. Retina 2014;34:1216Y22. 6. Xu H, Zeng F, Shi D, Sun X, Chen X, Bai Y. Focal choroidal excavation complicated by choroidal neovascularization. Ophthalmology 2014;121:246Y50. 7. Lee JH, Lee WK. Choroidal neovascularization associated with focal choroidal excavation. Am J Ophthalmol 2014;157:710Y8.e1. 8. Lee CS, Woo SJ, Kim YK, Hwang DJ, Kang HM, Kim H, Lee SC. Clinical and spectral-domain optical coherence tomography findings in patients with focal choroidal excavation. Ophthalmology 2014;121: 1029Y35. 9. Margolis R, Mukkamala SK, Jampol LM, Spaide RF, Ober MD, Sorenson JA, Gentile RC, Miller JA, Sherman J, Freund KB. The expanded spectrum of focal choroidal excavation. Arch Ophthalmol 2011;129:1320Y5. 10. Nazari Khanamiri H, Rao NA. Serpiginous choroiditis and infectious multifocal serpiginoid choroiditis. Surv Ophthalmol 2013;58: 203Y32.
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e20 Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al. 11. Spaide RF, Koizumi H, Freund KB. Photoreceptor outer segment abnormalities as a cause of blind spot enlargement in acute zonal occult outer retinopathy-complex diseases. Am J Ophthalmol 2008; 146:111Y20. 12. Spaide RF, Goldberg N, Freund KB. Redefining multifocal choroiditis and panuveitis and punctate inner choroidopathy through multimodal imaging. Retina 2013;33:1315Y24. 13. Punjabi OS, Rich R, Davis JL, Gregori G, Flynn HW, Jr., Lujan BJ, Rosenfeld PJ, Puliafito CA. Imaging serpiginous choroidopathy with
spectral domain optical coherence tomography. Ophthalmic Surg Lasers Imaging 2008;39:S95Y8.
Tsutomu Sakai Department of Ophthalmology Jikei University School of Medicine 3-25-8 Nishishinbashi, Minato-ku Tokyo 105-8461 Japan e-mail: [email protected]
Optometry and Vision Science, Vol. 92, No. 1, January 2015
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CLINICAL CASE
Focal Choroidal Excavation Associated with Focal Retinochoroiditis Tetsutaro Ohki*, Tsutomu Sakai*, and Hiroshi Tsuneoka*
ABSTRACT Purpose. To describe detailed spectral-domain optical coherence tomography (OCT) findings for two patients with focal choroidal excavation (FCE) associated with focal retinochoroiditis. Case Report. Three eyes from two patients with FCE associated with focal retinochoroiditis were evaluated by funduscopy, fluorescence angiography, indocyanine green angiography, and spectral-domain OCT during follow-up. Both patients with focal retinochoroiditis developed new FCE after oral steroid treatment and two eyes showed regression of the FCE during the follow-up. Both eyes from one patient transformed from the conforming to the nonconforming type and neither of the eyes were stable during the follow-up. Ultimately, all eyes exhibited the conforming-type FCE. Conclusions. Focal choroidal excavation can be seen as a tomographic phenotype after the treatment of focal retinochoroiditis. Spectral-domain OCT was useful for detecting the development of FCE after the treatment and for observing FCE regression. (Optom Vis Sci 2015;92:e12Ye20) Key Words: focal choroidal excavation, spectral-domain optical coherence tomography, focal retinochoroiditis, steroid
F
ocal choroidal excavation (FCE) is a newly recognized clinical entity that is revealed by optical coherence tomography (OCT) images.1 Recently, Obata et al.2 retrospectively reported 17 patients with FCE. The affected eyes usually have good visual acuity (VA) and lack a history of trauma, posterior uveitis, or retinal or choroidal vascular disease. Moreover, the lesions show little change over time. Thus, FCE was believed to only be a congenital malformation and to remain stable. However, most recent reports have shown that FCE is associated with choroidal neovascularization (CNV), polypoidal choroidal vasculopathy, and central serous chorioretinopathy (CSC), and is not always stable.3Y8 Spectral-domain (SD) OCT has better axial resolution than conventional time-domain OCT and is extremely useful in the evaluation of macular structures of the retina and choroid. Recent reports have revealed that SD-OCT facilitates the diagnosis of FCE.2Y9 In 2011, Margolis et al.9 expanded the spectrum of FCE and found that FCE can be classified into two types: conforming and nonconforming based on SD-OCT. They described that SDOCT revealed outer retinal layers conforming to retinal pigment epithelial alterations within the excavation, classified as the conforming type, and a separation between the outer retina and
*MD Department of Ophthalmology, Jikei University School of Medicine, Tokyo, Japan (all authors).
the retinal pigment epithelium (RPE) within the excavation, classified as the nonconforming type. They suggested the possibility of transformation from the conforming to the nonconforming type, although Obata et al.2 did not report any cases transforming from the conforming to the nonconforming type during the follow-up period. Serpiginous choroiditis is a clinically defined disorder characterized by destruction of the inner choroid and the RPE as well as secondary involvement of the retina.10 In about one-third of patients, the lesions are initially or exclusively at the macula. Such a pattern of choroiditis is recognized as macular serpiginous choroiditis. Recent SD-OCT studies of active serpiginous choroiditis have shown photoreceptor layer disruption associated with outer retinal and choriocapillaris hyperreflectivity10; however, there are no reports on the SD-OCT study of serpiginous choroiditis before and after the treatment. Herein, we describe the detailed appearance of FCE associated with focal retinochoroiditis in two patients (three eyes) using SDOCT. Both patients with focal retinochoroiditis developed new FCE after treatment with oral steroid, and two eyes showed regression of the FCE during the follow-up. Both eyes in one patient transformed from the conforming to the nonconforming type and none of the eyes were stable during the follow-up. We suggest that these cases represent the novel clinical findings of FCE and show how the findings provide further insight into this increasingly recognized inflammatory chorioretinal disorder.
Optometry and Vision Science, Vol. 92, No. 1, January 2015
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Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
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FIGURE 1. Fundus photography, FA, and ICGA of both eyes at the initial visit. Fundus photography showing some yellowish-white lesions in the right (A) and left (B) macula. Fluorescence angiography images showing weak hyperfluorescence corresponding to RPE alterations in the right (C) and left (I) macula. The hyperfluorescent area increased with time (D, E, J, K). The lesion demonstrated no early hypofluorescence or late hyperfluorescence. Indocyanine green angiography image showing hypofluorescence in all phases with late hyperfluorescence surrounding the area of hypofluorescence in the right (F to H) and left (L to N) macula. (I-) Early- to late-phase ICGA image showing no choroidal vascular hyperpermeability. Optometry and Vision Science, Vol. 92, No. 1, January 2015
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e14 Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
METHODS This observational case series evaluated patients who were diagnosed as having FCE in association with focal retinochoroiditis, based on clinical examination and diagnostic testing in a retinal practice. Each patient underwent a comprehensive ophthalmologic examination, including VA, intraocular pressure testing, slitlamp biomicroscopy, and dilated ophthalmoscopy. Visual acuity was evaluated using a Snellen chart on a scale of 0.1 to 1.5.
Patients also underwent color photography (VISCUM NM ProNM, Carl Zeiss Meditec, Dublin, CA), fluorescence angiography (FA) and indocyanine green angiography (ICGA; Heidelberg Retina Angiograph 2, Heidelberg Engineering, Heidelberg, Germany), and Cirrus SD-OCT scans (Carl Zeiss Meditec) for retinal imaging. Spectral-domain OCT scans were recorded using the HD 5-line raster scan protocol (horizontal scan of 6 mm). The OCT scans were reevaluated at a follow-up after several months. The study was
FIGURE 2. Spectral-domain OCT in both eyes at the initial (A, B) and follow-up (C to N) visits for FCE associated with focal retinochoroiditis. Horizontal SD-OCT scans showing focal excavation (arrow) and elevation (arrowhead) involving the outer retinal layers up to the ELM in the right eye (A). Disruption (arrow) and irregular elevation (arrowhead) of the RPE layer were noted in the parafovea of the left eye (B). A hyperreflective lesion with increased choroidal thickness (square bracket) was observed beneath the RPE line in both eyes (A, B). Spectral-domain OCT also revealed a disrupted ELM, ISe band, and OS/RPE, corresponding to the affected area (A, B). One week after the initiation of oral prednisolone, SD-OCT scan revealed a conforming FCE (arrow) with persistent disruption of the outer retina in the right eye (C) and a focal excavation and elevation involving the outer retinal layers up to the ELM in the left eye (D). Follow-up horizontal SD-OCT scan in the right eye showing FCE more clearly as the RPE band followed the contour of the choroidal excavation during follow-up, although a small area of outer retinal hyperreflectivity with a minimal amount of fluid collection was noted at 6 to 7 weeks (I, K). Follow-up SDOCT scan in the left eye demonstrated that a heterogeneously hyperreflective lesion was seen beneath the neurosensory retina with a small amount of fluid collection (arrow) at 4 weeks (H), after a formation of a conforming FCE was observed (F). Consecutive horizontal SD-OCT scans obtained at follow-up showed a change from the nonconforming to the conforming type with the resolution of the fluid and hyperreflective lesion (J, L, N). Ultimately, both eyes exhibited the conforming-type FCE (arrow) with subfoveal choroidal thinning (square brackets) (M, N). Optometry and Vision Science, Vol. 92, No. 1, January 2015
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Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
carried out with institutional approval and in accordance with the tenets of the Declaration of Helsinki.
RESULTS The age of the two patients was 32 and 54 years, with VAs ranging from 0.7 to 1.5. One patient had bilateral involvement. The follow-up in the two patients ranged from 3 to 4 months. The two patients with focal retinochoroiditis developed new FCE after oral steroid treatment and two eyes showed regression of the FCE during the follow-up. Both eyes from one patient transformed from the conforming to the nonconforming type and neither of the eyes were stable during the follow-up. Ultimately, all eyes exhibited the conforming-type FCE.
Case 1 A 32-year-old healthy woman with no medical or ocular history presented with visual disturbance in both eyes. Visual acuity was 0.7 with j3.50 sphere and 1.5 with j4.50 sphere in the right and left eye, respectively. There was no afferent pupillary defect. Slit-lamp examination was normal. No vitreous cells were present. There was no evidence of vitreous traction or posterior vitreous detachment. Ophthalmoscopy of both eyes revealed small, isolated, round, yellowish-white elevated lesions in the macula (Fig. 1A, B). Fluorescence angiography showed weak hyperfluorescence in the early phase and diffuse late staining and leakage of dye in both eyes (Fig. 1C to E, J, K). The lesions exhibited no early
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hypofluorescence and late hyperfluorescence. Indocyanine green angiography showed hypofluorescent areas that corresponded to the visible chorioretinal lesions from the early to the late phase (Fig. 1F to H, L to N). The hypofluorescent area observed with ICGA extended beyond the area of clinically observable lesions and that seen on FA. Spectral-domain OCT demonstrated focal excavation and elevation involving the outer retinal layers up to the external limiting membrane (ELM) in the right eye (Fig. 2A). Disruption and irregular elevation of the RPE layer were noted in the parafovea of the left eye (Fig. 2B). A hyperreflective lesion was observed beneath the RPE line in both eyes (Fig. 2A, B). Spectraldomain OCT also revealed a disrupted ELM, inner segment ellipsoid (ISe) band, and outer segment (OS)-RPE interdigitation (OS/RPE) corresponding to the affected area (Fig. 2A, B). Laboratory analysis showed a normal complete blood count, a negative QuantiFERON-TB Gold test, and a nonreactive rapid plasma reagin test. The patient had no syphilis and tuberculosis. The patient was diagnosed as having presumed macular serpiginous or ampiginous choroiditis of both eyes and treated initially with a moderate dose of prednisolone (30 mg/d) (0.6 mg/kg body weight). After 1 week of treatment, SD-OCT showed a conforming FCE with subfoveal choroidal thinning and the regression of the hyperreflective lesion in the right eye (Fig. 2C). By contrast, SD-OCT demonstrated focal excavation involving the outer retinal layers up to the ELM with a disruption of the RPE line and the hyperreflective lesion beneath the RPE line in the left eye (Fig. 2D). One week later, the VA improved to 1.2 along with almost complete recovery of the ELM (Fig. 2E). However, the patient presented with vision loss in the left eye. The VA was
FIGURE 3. Fundus photography, FA, and ICGA of both eyes at the resolution stage. Fundus photograph showing distinct yellow lesions in the right (A) and left (B) macula. Fluorescence angiography image showing focal hyperfluorescent spots nasal to the fovea in both eyes (C, E). Indocyanine green angiography image showing a hypofluorescent area with staining in the center of the lesion (D, F). No leakage was seen in midphase angiogram (D, E). Optometry and Vision Science, Vol. 92, No. 1, January 2015
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e16 Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
FIGURE 4. Fundus photography, FA, and ICGA of the left eye at the initial visit. Fundus photograph showing a yellowish-white fusiform lesion with pigmentary mottling in the left macula (A). Fluorescence angiography image showing faint circumferential hyperfluorescence with hypofluorescence in the center of the lesion (B). The hyperfluorescence increased with time (C). The lesion demonstrated no early hypofluorescence and late hyperfluorescence. Indocyanine green angiography image showing hypofluorescence in early to late phase with late hyperfluorescence surrounding the area of hypofluorescence. (D, E) Early- to late-phase ICGA image showing no choroidal vascular hyperpermeability.
0.8 along with persistent disruption of the photoreceptor layer. Spectral-domain OCT revealed a conforming FCE and regression of the hyperreflective lesion in the left eye (Fig. 2F). When the dose of oral prednisolone was decreased to 20 mg/d, photoreceptor integrity was completely disrupted with a small amount of fluid collection in the left eye (Fig. 2H) and the VA decreased to 0.5. One week after increasing the dose to 30 mg/d, incomplete reestablishment of the ELM was observed, despite an increase of the fluid (Fig. 2J) and the VA was 0.7. Spectral-domain OCT demonstrated a nonconforming FCE in the right eye (Fig. 2I, K). Two weeks later, vision in the patient’s left eye improved to 1.5 with the decrease of the fluid on SD-OCT (Fig. 2L). A minimal amount of fluid collection was noted in both eyes (Fig. 2K, L). At the end of the 12-week treatment period, the VA was 1.5, along with almost complete recovery of the ISe band and complete resorption of the fluid in both eyes (Fig. 2M, N). Three months after the treatment, fundus photography showed distinct gray lesions in the right (Fig. 3A) and left (Fig. 3B) macula. Fluorescence angiography image showed hyperfluorescent spots in both eyes (Fig. 3C, E). Indocyanine green angiography image showed late staining in the center and periphery of
the lesion (Fig. 3D, F). No leakage was seen in a midphase angiogram.
Case 2 A 54-year-old healthy man with no previous ocular history presented with a 3-week history of metamorphopsia in the left eye. Upon examination, VA was 1.0 and 0.5 with j2.75 sphere in the right and left eye, respectively. No afferent pupillary defect was present. Anterior segment examination was normal in both eyes. No vitreous cells were present in either eye. There was no evidence of vitreous traction or posterior vitreous detachment. Ophthalmoscopy of the left eye showed marked yellowish-white plaque-like lesion in the fovea (Fig. 4A). Fluorescence angiography showed early blockage with late hyperfluorescent borders that spread toward the center of the lesion (Fig. 4B), and ICGA demonstrated hypofluorescence of the lesions from the early to the late phase (Fig. 4C). The lesion did not exhibit early hypofluorescence or late hyperfluorescence. Ophthalmoscopic examination of the right eye was normal. Spectral-domain OCT of the left eye showed irregular elevation of the RPE line involving the
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Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
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FIGURE 5. Spectral-domain OCT in the left eye at the initial (A) and follow-up (B to J) visits for FCE associated with focal retinochoroiditis. Horizontal SD-OCT scans showing a focal elevation (arrowhead) involving the outer retinal layers up to the ELM at the initial visit (A). Irregular elevation of the RPE layer was noted in the fovea. A hyperreflective lesion (arrow) with increased choroidal thickness was seen beneath the RPE line (A, B). Spectral-domain OCT also revealed a disrupted ELM, ISe band, and OS/RPE corresponding to the affected area. One week after the initiation of oral prednisolone, SD-OCT scan revealed a focal excavation (arrow) with persistent disruption of outer retina (B). One week later, follow-up SD-OCT scan showing a nonconforming FCE (arrow) more clearly as the RPE band followed the contour of the choroidal excavation (C). Two weeks later, follow-up SD-OCT scan demonstrated discrete FCE with the decreased fluid and hyperreflective lesion (D). Two weeks later, SD-OCT showed almost complete reestablishment of the ELM and ISe band (E). When the dose of oral prednisolone was decreased to 12.5 mg/d, focal retinochoroiditis recurred. Spectral-domain OCT showed irregular elevation (arrowhead) of the RPE layer with hyperreflective lesion (arrow) beneath the RPE (F). One week after the treatment, SD-OCT demonstrated mild flattening of the RPE line, although the hyperreflective lesion was persistent (G). One week later, SD-OCT revealed restored ELM and ISe band with an increase of the hyperreflective lesion (H). Two weeks later, the follow-up SD-OCT demonstrated a conforming FCE (arrow) with a reduction of the hyperreflective lesion (I). Fourteen weeks after the treatment, best-corrected VA was 1.5 along with almost complete recovery of the ELM and ISe band (J).
outer retinal layers up to the ELM (Fig. 5A). A hyperreflective lesion was seen beneath the RPE line. Spectral-domain OCT also revealed a disrupted ELM, ISe band, and OS/RPE corresponding to the affected area. Laboratory analysis showed a normal complete blood count, a negative QuantiFERON-TB Gold test, and a
nonreactive rapid plasma reagin test. The patient had no syphilis and tuberculosis. The patient was diagnosed as having presumed macular serpiginous or ampiginous choroiditis of the left eye and was treated initially with a moderate dose of prednisolone (30 mg/d) (0.5 mg/kg
Optometry and Vision Science, Vol. 92, No. 1, January 2015
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e18 Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
FIGURE 6. Fundus photography, FA, and ICGA of both eyes at the resolution stage. Fundus photography showing distinct yellowish-white lesions in the left macula (A). Fluorescence angiography image showing a focal hyperfluorescent spot in the left eye (B). Indocyanine green angiography image showing a hypofluorescent area with a staining spot in the center (C).
body weight). After 1 week of treatment, SD-OCT revealed a nonconforming FCE with regression of the hyperreflective lesion beneath the RPE (Fig. 5B). The VA improved to 0.9 along with complete reestablishment of ELM on SD-OCT. When the dose of oral prednisolone was tapered to 25 mg/d, the ISe band was restored mildly, although the subretinal fluid was increased (Fig. 5C). Two weeks later, SD-OCT showed further resolution of the ISe band with reduction of the FCE and fluid (Fig. 5D). Ophthalmoscopic examination demonstrated reduction of the yellowishwhite plaque lesion. Two weeks later, SD-OCT showed almost complete reestablishment of the ELM and ISe band (Fig. 5E). The VA had improved to 1.2 in the left eye. When the dose of oral prednisolone was decreased to 12.5 mg/d, SD-OCT showed irregular elevation of the RPE layer with a hyperreflective lesion beneath the RPE in the left eye, similar to the initial findings (Fig. 5F), and the VA was 1.2. The right eye remained unaffected. The patient was diagnosed as having recurrent macular serpiginous or ampiginous choroiditis in the left eye and was treated with an increased dose of prednisolone (30 mg/d). One week later, SD-OCT demonstrated mild flattening of the RPE line, although the hyperreflective lesion remained persistent (Fig. 5G). The VA decreased to 1.0. One week later, SD-OCT revealed restoration of the ELM and ISe band, with a concomitant increase of the hyperreflective lesion (Fig. 5H). The VA
increased to 1.5. Two weeks later, follow-up SD-OCT demonstrated a conforming FCE with reduction of the hyperreflective lesion (Fig. 5I). Fourteen weeks after the treatment, the VA was 1.5 with almost complete recovery of the ELM and ISe band (Fig. 5J). Three months after the treatment, fundus photography showed distinct grayish-white lesions in the left eye (Fig. 6A). Fluorescence angiography imaging showed some hyperfluorescent spots in the left eye (Fig. 6B). Indocyanine green angiography imaging showed hypofluorescence with late staining in the center of the lesion (Fig. 6C). No leakage was seen in a midphase angiogram.
DISCUSSION Herein, we present a first case series of FCE associated with focal retinochoroiditis. Two patients with focal retinochoroiditis developed new FCE after oral steroid treatment and two eyes showed regression of the FCE during follow-up. Both eyes of one patient transformed from the conforming to the nonconforming type and none of the eyes were stable during the follow-up. Additionally, tomographic and angiographic abnormalities that were observed in the photoreceptor layer, RPE, and choroid in the acute phase of the disease regressed within a few months after oral steroid therapy. Thus, SD-OCT provided a definitive diagnosis of the FCE associated with inflammatory chorioretinal
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Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al.
disease, the associated outer retinal and choroidal morphological abnormalities, and the ability to document improvement. A previous report has suggested that FCE is a congenital malformation that can remain stable without any change.9 In our patients, all eyes developed new FCE and showed slow regression of FCE during the treatment and follow-up. In one eye, FCE remained stable without any change in size or shape during the resolution stage. Although many cases of FCE may be a stable congenital choroidal malformation, cases of FCE associated with chorioretinal disorders, such as CNV, polypoidal choroidal vasculopathy, CSC, and retinochoroiditis, are not stable and may change in size or shape during the treatment follow-up. Although previous studies have suggested that FCE might form because of RPE retraction attributed to focal scarring of the choroidal connective tissue,3Y8 in the current cases, we suggest that the formation of the FCE may be associated with focal contractive changes in the choroidal parenchyma after treatment of focal retinochoroiditis. Margolis et al.9 expanded the spectrum of this entity by analyzing a series of 12 patients with FCE. They9 categorized FCE into nonconforming and conforming types and suggested the possibility of transformation from the conforming to the nonconforming type, although Obata et al.2 had no cases that transformed from the conforming to the nonconforming type during their follow-up period. Recently, Ellabban et al.4 and Xu et al.6 reported that nonconforming FCE changed to the conforming type after successful treatment of CSC or CNV, respectively. In our patients, two eyes exhibited changes from the conforming to the nonconforming type and one eye changed from the nonconforming to the conforming type, although all eyes ultimately transformed to the conforming type after successful treatment. Thus, further understanding of the histopathological nature of FCE during the treatment follow-up will necessitate the accumulation of more cases. A recent study of 3D-OCT image analysis revealed that FCE may be a feature associated with choroidal thickening.4 The study showed that subfoveal choroidal thickness in eyes with FCE was greater than that observed in normal subjects, whereas foveal choroidal thickness in CSC eyes with FCE has been shown to be significantly thinner than that in CSC eyes without FCE. In our patients, SD-OCT image demonstrated that subfoveal choroidal thickness in eyes with FCE associated with inflammatory chorioretinal disorders was different between the active and resolution stages of inflammation. In the active inflammatory stage, SD-OCT revealed choroidal thickening, and in the resolution stage after steroid treatment, SD-OCT showed the FCE with marked decrease in subfoveal choroidal thickness. We suggest that a regression of inflammatory material, such as fibrin, after the treatment of acute choroidal inflammation, may result in choroidal thinning in the resolution stage. The improvement of the damaged photoreceptor integrity associated with choroidal abnormalities noted herein may be common to some clinical conditions. For example, somewhat similar improvements have been noted in cases of multifocal choroiditis,11,12 punctate inner choroidopathy,11,12 and serpiginous choroiditis.13 These cases featured hyperreflective lesions involving the photoreceptor layer on OCT, which could represent a localized inflammatory response. Given that the patients in this
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series were treated successfully with oral steroid, it may be possible that the retinal changes demonstrated on OCT represent an early transient manifestation of a localized inflammatory condition. Taken together, they may be involved in inflammatory conditions, such as multifocal choroiditis, punctate inner choroidopathy, and serpiginous choroiditis. In conclusion, the patients in this case series exhibited FCE associated with focal retinochoroiditis. Although the FCE might be related to the pathogenesis of focal retinochoroiditis, its role remains unknown. Spectral-domain OCT was extremely useful in the detection of FCE and in understanding the morphological changes of the outer retina and choroid during follow-up of inflammatory chorioretinal disorders.
ACKNOWLEDGMENTS The authors have no financial support or conflict of interest in this study. Contributions of authors: Design and conduct of study (TS); collection of data (TO); management, analysis, and interpretation of data (TO, TS, and HT); and preparation, review, and approval of the manuscript (TO, TS, and HT). Received June 30, 2014; accepted October 3, 2014.
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e20 Focal Choroidal Excavation with Focal RetinochoroiditisVOhki et al. 11. Spaide RF, Koizumi H, Freund KB. Photoreceptor outer segment abnormalities as a cause of blind spot enlargement in acute zonal occult outer retinopathy-complex diseases. Am J Ophthalmol 2008; 146:111Y20. 12. Spaide RF, Goldberg N, Freund KB. Redefining multifocal choroiditis and panuveitis and punctate inner choroidopathy through multimodal imaging. Retina 2013;33:1315Y24. 13. Punjabi OS, Rich R, Davis JL, Gregori G, Flynn HW, Jr., Lujan BJ, Rosenfeld PJ, Puliafito CA. Imaging serpiginous choroidopathy with
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Tsutomu Sakai Department of Ophthalmology Jikei University School of Medicine 3-25-8 Nishishinbashi, Minato-ku Tokyo 105-8461 Japan e-mail: [email protected]
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