|Year : 2014 | Volume
| Issue : 4 | Page : 179-183
A novel view of punctate inner choroidopathy: Characterizing the serial changes by high resolution spectrum-domain optical coherence tomography
Yu-Bai Chou1, Yu-Chien Chung1, Shih-Jen Chen2, Fenq-Lih Lee2, Chang-Sue Yang3
1 Department of Ophthalmology, Taipei Veterans General Hospital, Taipei, Taiwan
2 Department of Ophthalmology, Taipei Veterans General Hospital; Department of Ophthalmology, National Yang-Ming University School of Medicine, Taipei, Taiwan
3 Department of Ophthalmology, Taipei Veterans General Hospital; Department of Ophthalmology, National Yang-Ming University School of Medicine; Department of Ophthalmology, Taipei Medical University, Taipei, Taiwan
|Date of Web Publication||1-Oct-2014|
Department of Ophthalmology, Taipei Veterans General Hospital, 201, Section 2, Shih-Pai Road, Taipei 112
Source of Support: None, Conflict of Interest: None
We report a case of punctate inner choroidopathy (PIC) treated with oral prednisolone and intravitreous bevacizumab injection (IVB). The case was studied and followed for 5 months by serial spectrum-domain optical coherence tomography (SD-OCT). At the early active phase of PIC, SD-OCT showed dome-shaped retinal pigment epithelium (RPE) elevation with underlying intact Bruch’s membrane (BM), overlying photoreceptor inner/outer segment junction (PRJ) disappearance, and homogenous hyperreflective sub-RPE signals. After oral prednisolone and IVB, SD-OCT showed resolution of RPE changes with overlying intact PRJ. Further, the localized outer retinal layers were found to gradually move outward. Our case of PIC was followed by SD-OCT and was successfully treated with oral prednisolone and IVB. SD-OCT may play an important role in early identification of PIC and evaluation of disease progression. According to the serial changes in SD-OCT, we hypothesize that the origin of PIC is most likely to be localized at the RPE level. Early detection and intervention may prevent the progression of PIC to choroidal neo-vascularization and may lead to good prognosis.
Keywords: bevacizumab, optical coherence tomography, punctate inner choroidopathy
|How to cite this article:|
Chou YB, Chung YC, Chen SJ, Lee FL, Yang CS. A novel view of punctate inner choroidopathy: Characterizing the serial changes by high resolution spectrum-domain optical coherence tomography. Taiwan J Ophthalmol 2014;4:179-83
|How to cite this URL:|
Chou YB, Chung YC, Chen SJ, Lee FL, Yang CS. A novel view of punctate inner choroidopathy: Characterizing the serial changes by high resolution spectrum-domain optical coherence tomography. Taiwan J Ophthalmol [serial online] 2014 [cited 2020 May 25];4:179-83. Available from: http://www.e-tjo.org/text.asp?2014/4/4/179/204136
| 1. Introduction|| |
Punctate inner choroidopathy (PIC) is a rare idiopathic ocular inflammatory disease. It was first reported by Watzke et al in 1984 to describe the multifocal, yellow-white, well-circumscribed choroidal lesions over the posterior pole in 10 myopic female pa-tients. These lesions may evolve variably into hyperpigmented scars, with atrophic changes at the level of the inner choroid and retinal pigment epithelium (RPE), or subretinal choroidal neo-vascularization (CNV). There is no sign of inflammation elsewhere in the eyes. Image studies have revealed fluorescent dye leakage of the active lesions on fluorescein angiography (FA),,, and involvement of choriocapillaries on indocyanine green angiography (ICG).,, However, to date, the exact localization of the origin of PIC lesions remains unclear.
With advances in the technique of optical coherence tomography (OCT), it is increasingly being used in studies for a detailed morphological view of the retina, even of the choroid. In 2012, Channa et al reported characteristic findings by SD-OCT in a retrospective, observational case series with PIC lesions. They had collected 27 lesions from seven patients (8 eyes), and classified them into four categories according to the disease activity and temporal changes. The lesions with CNV were excluded. They concluded that early active lesions were characterized by RPE elevation and photoreceptor inner/outer segment junction (PRJ) disruption. However, there was no detailed description in treatment and serial follow-up by SD-OCT.
In this report, we aimed to utilize the high resolution SD-OCT with its enhanced depth imaging (EDI) mode to follow up a case with active PIC lesions treated with oral prednisolone and intra-vitreous bevacizumab (Avastin, Genetech, San Francisco, CA, USA) injection (IVB), and to hypothesize the original localization in this rare disease.
| 2. Case report|| |
A 32-year-old Asian female with myopia initially complained of white patches of the central scotoma in her left eye from January 8, 2013 and came to our clinic on January 11, 2013 (Day 1). She denied any systemic disease, ocular disease, or family history. She mentioned travel history to Thailand on January 4, 2013, and occasional contact history with cats; she denied any contact history with soil, bats, or birds. The serum laboratory data was normal for complete blood count and biochemistry. Enzyme immunoassays of herpes simplex virus and herpes zoster virus were also arranged, to rule out virus infection; the reports were all negative.
On ophthalmic examination, the best corrected visual acuity showed 6/6 in both eyes. The spherical equivalent refraction was approximately -7 diopters in both eyes. There were no inflammatory signs in the anterior chamber or vitreous humor. Fundo-scopy revealed multiple yellow-white lesions over the posterior pole and some old punch-out lesions near the arcades [Figure 1]A. No peripheral lesion was present. FA of these yellow-white lesions revealed hyperfluorescence in the mid-phase with gradual staining and mild leakage in the late phase [Figure 1]B. ICG showed the following: multiple hypofluorescent spots in the early, middle, and late phases; the small hyperfluorescent crescent area at the margin of one hypofluorescent spot [Figure 1]C, which was temporal-superior to the fovea, may represent choroidal vasculitis or oncoming neovascularization.
|Figure 1: (A-1 and A-2) At the first visit, the color photograph demonstrated multiple yellow-white lesions over the posterior pole in the left eye, and the three active punctate inner choroidopathy (PIC) lesions were at the fovea, causing the central scotoma (white arrowheads). (B-1 and B-2) FA revealed hyperfluorescent PIC lesions with gradual staining and mild leakage in the late phase. (C-1 and C-2) indocyanine green angiography (ICG) showed hypofluorescent PIC lesions; only one bigger active lesion temporal-superior to the fovea showed a hyperfluorescent crescent area at the margin (yellow arrow), which may represent choroidal vasculitis or oncoming neovascularization.|
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Cirrus SD-OCT (Cirrus HD-OCT, Carl Zeiss Meditec, Dublin, CA, USA) in high definition raster with EDI mode (software: version 6.0) was performed at the first visit and revealed [Figure 2]A: (1) dome-shaped RPE elevation with underlying intact Bruch’s membrane (BM); (2) overlying disappearance of the photoreceptor junction (PRJ); (3) localized homogenous hyperreflective signals below the RPE.
|Figure 2: We followed and focused on the central three active punctate inner choroidopathy (PIC) lesions by spectrum-domain optical coherence tomography (SD-OCT). (A) Day 1. SDOCT showed dome-shaped retinal pigment epithelium (RPE) elevation with intact Bruch's membrane, disappearance of photoreceptor inner/outer segment junction (PRJ), and homogeneous sub-RPE hyperreflective signal. (B) Day 10. SD-OCT showed resolution of RPE change with visible overlying PRJ. The sub-RPE hyperreflective signals were still noted. (C) Day 28. Above the hyperreflective signals, the localized outer plexiform layers (OPL) were gradually moving outward, and the corresponding RPE and PRJ were more depressed. The hyperreflective signals were smaller and fainter. (D) Day 48. The RPE and PRJ were smoother, and the hyperreflective signals gradually became fainter.|
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On Day 1, we started to administer oral prednisolone 20 mg twice daily, then tapered the dose every 4–5 days, and stopped it after 1 month. The IVB 1.25 mg/0.05 mL and subtenon injection of triamcinolone 40 mg were given on Day 15 to prevent further choroidal neovascularization formation and decrease the inflammatory reaction.
On Day 10 [Figure 2]B, the SD-OCT showed regression of RPE elevation, with overlying visible intact PRJ in a concave shape. The homogenous sub-RPE hyperreflective signals were still noted. The active lesions seemed to be smaller, with a clearer margin than the initial color fundus pictures.
On Day 30 [Figure 2]C, the outer retinal layers overlying the active lesions seemed to be moving outward at the level of outer nuclear layer (ONL) and outer plexiform layer (OPL); the remaining sub-RPE hyperreflective signals were still noted, but had shrunk in diameter.
However, one active lesion temporal to the fovea seemed to have PRJ disruption [Figure 3], consistent with the hypofluorescent spot which had the hyperfluorescent crescent margin on the ICG angiography [Figure 1]C.
|Figure 3: (A) Day 1. The bigger active punctate inner choroidopathy (PIC) lesions temporal-superior to the fovea revealed the same dome-shaped retinal pigment epithelium (RPE) elevation with intact Bruch's membrane (yellow arrow). (B) Day 30. More prominent outer plexiform layers (OPL) outward movement, sub-RPE hyperreflective signal, and photoreceptor inner/outer segment junction (PRJ) discontinuity were noted, causing visual localized metamorphopsia.|
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The SD-OCT findings were unchanged after 1 month, and the symptoms were also improved [Figure 4]. The administration of the different kinds of medication ceased after 2 months, and no symptomatic recurrence or change in SD-OCT was noted for the next 3 months of follow-up. No ocular complications, such as cataract formation or ocular hypertension, were noted during follow-up. The final visual acuity was 6/6 in both eyes.
|Figure 4: Visual field showed central scotoma in the left eye on Day 7. After oral prednisolone and intravitreous bevacizumab injection, the symptoms improved and the central scotoma disappeared on Day 32 and Day 60.|
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| 3. Discussion|| |
PIC was first described by Watzke et al as small, multifocal, yellow-white lesions originating from the choroid. However, the definite pathophysiological origin of PIC has been debated for a long time. In this study, our purpose was to utilize high resolution SD-OCT to follow the structural changes in active PIC lesions before and after treatment in a disease course of 5 months.
The initial findings of the three new central foci of PIC in high resolution SD-OCT revealed dome-shaped RPE elevation, with the overlying disappearance of the PRJ, intact BM, and underlying homogeneous hyperreflective signals below the lesions. No prominent choroidal lesions were noted. After administration of oral prednisolone, we observed the regression of RPE elevation and the overlying visible intact PRJ in a concave shape, and similar sub-RPE homogenous hyperreflective signals. After bevacizumab injection, SD-OCT showed outward movement of the OPL and ONL in the same three lesions. Our initial findings are compatible with the clinical observation made by Watzke et al and Watzke and Claussen; serous RPE detachments occurred either at the first appearance of a new lesion or during recurrence. Therefore, we hypothesize that the original localization of PIC may be related to the outer retina, and more specifically, related to the RPE. The inflammation in the RPE may cause RPE elevation as the initial change before CNV formation.
Channa et al reported similar RPE and outer retinal changes in the clinically active PIC lesions. Unfortunately, there were limitations of image quality at the sub-RPE and choroid levels with the use of conventional Spectralis OCT. However, Cirrus OCT with EDI mode was capable of providing better image quality in the deep tissue. For this reason, in this report, we are able to provide a better morphological view of active PIC lesions with the higher resolution SD-OCT.
In our study, the other, bigger active lesion, temporal-superior to the fovea, seemed to have PRJ disruption [Figure 3], which is consistent with the hypofluorescent spot that had the hyper-fluorescent crescent margin in the ICG angiography. Our inference is that the serious inflammation has led to choroidal neo-vascularization and outer retinal changes, including the disruption in the PRJ and more prominent outward movement of the OPL. As the disease progresses, it may lead to further disruption of the RPE and choroid.
In previous studies we reviewed,,,, bevacizumab was mainly given when a CNV membrane developed in PIC disease. Pachydaki et al made a clinicopathologic and ultrastructural report of CNV membranes excised from the subretinal space. The study revealed that well-formed neovascular units consistently exhibiting peri-cytes may have been unresponsive to bevacizumab. Therefore, the early intervention of bevacizumab before CNV membrane formation is likely to reduce the risk of CNV progression and prevent the recurrence.
In conclusion, this report provides a morphological view of active PIC lesions by high resolution SD-OCT. According to the serial changes in SD-OCT, we hypothesize that the origin of PIC may be localized at the RPE level. Furthermore, early detection and intervention may prevent CNV formation and lead to a good prognosis.
Conflicts of Interest: The authors declare that they have no conflicts of interest.
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