|Ahead of print publication
Retinal pigment epithelium tear in an idiopathic acute onset bullous central serous chorioretinopathy
You-Ci Ou1, Cheng-Kuo Cheng2, Chang-Sue Yang3
1 Department of Ophthalmology, Shin Kong Wu Ho-Su Memorial Hospital, New Taipei, Taiwan
2 Department of Ophthalmology, Shin Kong Wu Ho-Su Memorial Hospital; Department of Ophthalmology, School of Medicine, Fu-Jen Catholic University, New Taipei, Taiwan
3 Department of Ophthalmology, Shin Kong Wu Ho-Su Memorial Hospital; Department of Ophthalmology, School of Medicine, Taipei Medical University, New Taipei, Taiwan
|Date of Submission||01-Nov-2022|
|Date of Acceptance||11-Jan-2023|
|Date of Web Publication||27-Feb-2023|
Department of Ophthalmology, Shin-Kong Wu Ho-Su Memorial Hospital, No. 95, Wen Chang Road, New Taipei
Source of Support: None, Conflict of Interest: None
Although retinal pigment epithelium (RPE) tears are common in patients with chronic conditions such as exudative age-related macular degeneration or may occur in response to anti-vascular endothelial growth factor or laser treatment, a spontaneous RPE tear can occur in patients with acute and new-onset bullous central serous chorioretinopathy (CSCR). We present a rare case of a healthy young Asian man with unilateral massive subretinal fluid (SRF). An idiopathic acute-onset bullous CSCR with an RPE tear was diagnosed through ancillary examinations. This patient exhibited good visual recovery as indicated by foveal sparing, spontaneous resolution of SRF, and the lack of a need for unnecessary surgery.
Keywords: Central serous chorioretinopathy, retinal detachment, retinal pigment epithelium tear
|How to cite this URL:|
Ou YC, Cheng CK, Yang CS. Retinal pigment epithelium tear in an idiopathic acute onset bullous central serous chorioretinopathy. Taiwan J Ophthalmol [Epub ahead of print] [cited 2023 Apr 2]. Available from: https://www.e-tjo.org/preprintarticle.asp?id=370661
| Introduction|| |
Retinal pigment epithelium (RPE) tears were first described in 1981. Vascularized retinal pigment epithelial detachment (PED) in patients with exudative age-related macular degeneration (AMD) is the most common cause of an RPE tears. Recently, more cases have been associated with anti-vascular endothelial growth factor (anti-VEGF) therapies and thermal laser treatment. An RPE tear in central serous chorioretinopathy (CSCR) is uncommon and typically associated with a large PED. Previous studies have reported an RPE tear occurring after a fibrotic scar in chronic CSCR or found in a bullous CSCR, which was initially diagnosed as a bullous retinal detachment (RD)., However, an RPE tear is rare in acute and new-onset CSCR. We present a case of an RPE tear discovered in an idiopathic acute onset bullous CSCR with high-resolution images.
| Case Report|| |
A 40-year-old male, who had glucose-6-phosphate dehydrogenase deficiency, arrived at our eye clinic with the main complaint of having blurred vision in his right eye for 2 days. He denied taking steroids and smoking and had no recent history of ocular trauma, diabetes mellitus, or hypertension. An ophthalmologic examination revealed that his best-corrected visual acuity (BCVA) was 6/60 in the right eye and 6/6 in the left eye. His intraocular pressure and anterior segment were unremarkable. A fundus examination (Canon CR-2 nonmydriatic camera, Tokyo, Japan) showed serous RD and a small depigmentation lesion superior to the fovea in the right eye [Figure 1]a. No hemorrhage or exudation was noted. Spectral-domain optical coherence tomography (SD-OCT) (RTVue Avanti, Optovue, Freemont, California, USA) revealed massive subretinal fluid (SRF) and fibrin accumulation with foveal involvement in the right eye and an RPE tear corresponding to the depigmentation lesion in fundus examination [Figure 1]b. Fundus autofluorescence examination (Heidelberg Engineering Inc., Heidelberg, Germany) showed an oval lesion with hypoautofluorescence corresponding to the area of the RPE tear [Figure 1]c. Fluorescein angiography (FA) and indocyanine green angiography examinations (Heidelberg Engineering Inc., Heidelberg, Germany) revealed hyperfluorescence of the lesion in the early phase and leakage with progression in angiogram findings [Figure 2]. The patient was given a diagnosis of an idiopathic acute-onset bullous CSCR with an RPE tear.
|Figure 1: (a) Color fundus photographs of the patient's right eye showed a SRD (white arrows) and a small depigmentation lesion superior to the fovea (white arrowheads). (b) SD-OCT of the right eye showed massive SRF and fibrin accumulation (yellow star) with foveal involvement and an RPE tear corresponding to the depigmentation lesion in the fundus examination (yellow arrows). (c) Fundus autofluorescence examination showed hypoautofluorescence of the lesion. SD-OCT: Spectral-domain optical coherence tomography, SRF: Subretinal fluid, RPE: Retinal pigment epithelium. SRD: Serous retinal detachment|
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|Figure 2: (a) Early phase and (b) late phase. FA and ICGA examinations of the right eye showed hyperfluorescence of the lesion in the early phase and leakage with progression in angiogram findings. FA: Fluorescein angiography, ICGA: Indocyanine green angiography|
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After 2 weeks treatment of oral medication (Bromelain 20000U/L-Cysteine 20 mg tablet 4 times and spironolactone 25 mg 2 times per day), the patient's BCVA gradually improved to 6/6.7 in the right eye. Fundus and SD-OCT examinations showed gradual resolution of SRF in the right eye [Figure 3]a and [Figure 3]b. We adjusted SD-OCT focus directly on the center of RPE tear lesion. The examination indicated RPE scrolling and showed that the tear was covered with glial tissue [Figure 3]c. After 1 month, his BCVA improved to 6/6 in the right eye and examinations showed complete resolution of SRF with a scar formation [Figure 4]. More aggressive CSCR treatments, such as focal laser therapy and photodynamic therapy, were not performed.
|Figure 3: (a) 1 week later, SD-OCT of the right eye showed mild resolution of the SRF, and RPE cell exhibited scrolling (white arrows) (b) 2 weeks later, SD-OCT showed greatly improved SRF and the tear being covered with glial tissue (blue arrows). (c) SD-OCT image when the focus was directed to the center of RPE tear lesion from picture b. SD-OCT: Spectral-domain optical coherence tomography, SRF: Subretinal fluid, RPE: Retinal pigment epithelium|
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|Figure 4: (a) After 1 month, color fundus photographs showed the RPE tear as a depigmentation lesion (white arrow). (b) SD-OCT of the right eye showed complete resolution of SRD with a scar formation. RPE: Retinal pigment epithelium, SD-OCT: Spectral-domain optical coherence tomography, SRD: Serous retinal detachment|
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| Discussion|| |
CSCR is often associated with a serous detachment of the neurosensory retina and sometimes with a small serous PED. The SRF is rarely large enough to form a bullous CSCR. The bullous form may be mistaken for Harada disease or a rhegmatogenous RD. A spontaneous large RPE tear is more likely to be associated with the bullous CSCR. An RPE tear in CSCR may occur due to contractile forces exerted by sub-RPE fibrin or increased hydrostatic forces inside a PED. This tear can cause fluid leakage into the subretinal space from the PED or the choriocapillaris, resulting in massive SRF. RPE tears are more common in patients with chronic CSCR or those treated with steroid therapy because it takes time to build up sufficient hydrostatic pressure within a PED or sufficient contraction force to break the RPE layer.,
We report a rare case of an RPE tear found in a patient with a bullous CSCR. Although there was fibrin accumulation in OCT image, we suggested this CSCR as acute onset type since the patient's symptom occurred within 2 days, and there was no atrophic changes in the RPE layer and FA displayed well-defined border without subtle leaks or ill-defined staining. The patient's recovery was possible because the tear spared fovea and was covered with glial tissue seen in SD-OCT, which led to the resolution of SRF. The correct diagnosis of a bullous variant of CSCR with an RPE tear is crucial because patients can avoid unnecessary surgeries and vision loss resulting from neuroretinal damage. In most cases, a layer of glial tissue heals the tear and forms a fibrous scar. This condition must be monitored closely because choroidal neovascularization may occur.
| Conclusion|| |
Although RPE tears are common in patients with chronic conditions such as exudative AMD or may occur in response to anti-VEGF or laser treatment, a spontaneous RPE tear may occur in patients with idiopathic acute-onset bullous CSCR. By performing ancillary examinations such as SD-OCT or FA, we can avoid an incorrect diagnosis of RD. The patient exhibited visual recovery as indicated by foveal sparing, resolution of SRF, and the lack of a need for unnecessary surgery. However, a scar may be formed, and patients must thus be monitored closely.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
The authors are grateful to Guan-Yi, Huang for his assistance with the article.
Financial support and sponsorship
Conflicts of interest
Dr. Cheng-Kuo Cheng, an editorial board member at Taiwan Journal of Ophthalmology, had no role in the peer review process of or decision to publish this article. The other authors declared no conflicts of interest in writing this paper.
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