|Year : 2023 | Volume
| Issue : 1 | Page : 170-174
Healing pattern of subfoveal retinal pigment epithelium rip and aperture in central serous chorioretinopathy
George Joseph Manayath, Amishi Khanna, Ratnesh Ranjan, Narendran Venkatapathy
Department of Vitreo-Retina, Aravind Eye Hospital and Post-Graduate Institute of Ophthalmology, Coimbatore, Tamil Nadu, India
|Date of Submission||11-Nov-2021|
|Date of Decision||25-Oct-2022|
|Date of Acceptance||02-Dec-2022|
|Date of Web Publication||21-Feb-2023|
Department of Vitreo-Retina, Aravind Eye Hospital and Post-Graduate Institute of Ophthalmology, Coimbatore - 641 014, Tamil Nadu
Source of Support: None, Conflict of Interest: None
| Abstract|| |
This report describes a rare case of spontaneous Grade-4 retinal pigment epithelium (RPE) rip of serous pigment epithelial detachment (PED) in central serous chorioretinopathy (CSC) and RPE aperture in the fellow eye, with favorable long-term outcomes. A 38-year-old man presented with defective vision (20/30) in the left eye (LE) due to bullous CSC associated with a large extramacular RPE rip located temporally and inferior exudative retinal detachment. Optical coherence tomography (OCT) confirmed a subfoveal serous PED with RPE aperture, subretinal fluid (SRF) and fibrinous exudation, and a large extramacular RPE rip temporally. The right eye (RE) had an asymptomatic large serous PED. The LE was treated with low-fluence photodynamic therapy, which resulted in the closure of RPE aperture and complete resolution of PED and SRF. Six-month later, the patient presented with sudden defective vision (20/120) in the RE secondary to a large fovea-involving (Grade-4) RPE rip with SRF as confirmed on OCT. Fluorescein angiography showed two extrafoveal active point leaks, which were treated with focal photocoagulation. He was also started on oral eplerenone. On subsequent serial follow-ups over 1 year, OCT showed SRF resolution and patchy reorganization of the subfoveal RPE-photoreceptor complex with good visual outcome (20/30).
Keywords: Central serous chorioretinopathy, pigment epithelial detachment, retinal pigment epithelial aperture, retinal pigment epithelial rip
|How to cite this article:|
Manayath GJ, Khanna A, Ranjan R, Venkatapathy N. Healing pattern of subfoveal retinal pigment epithelium rip and aperture in central serous chorioretinopathy. Oman J Ophthalmol 2023;16:170-4
|How to cite this URL:|
Manayath GJ, Khanna A, Ranjan R, Venkatapathy N. Healing pattern of subfoveal retinal pigment epithelium rip and aperture in central serous chorioretinopathy. Oman J Ophthalmol [serial online] 2023 [cited 2023 Mar 26];16:170-4. Available from: https://www.ojoonline.org/text.asp?2023/16/1/170/370050
| Introduction|| |
The incidence of retinal pigment epithelium (RPE) tears in central serous chorioretinopathy (CSC)-associated serous pigment epithelial detachments (PEDs) is rare compared to those complicating vascularized PEDs secondary to neovascular age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy.,, The RPE rip is a classical form of large RPE tear, associated with 10%–17% of AMD-related vascularized PED, developing either spontaneously or following anti-vascular endothelial growth factor (VEGF) therapy. The RPE tears are graded based on the size of the tears and the involvement of the fovea: Grade 1 tears as <200 μm, Grade 2 tears between 200 μm and 1-disc diameter, Grade 3 tears as >1-disc diameter, and Grade 4 tears as Grade 3 tears involving the center of the fovea. The RPE aperture is another form of RPE defect, usually of smaller size, which has been reported to complicate avascular PED in AMD. The pathogenesis and features of RPE rip and aperture in CSC differ from those seen in association with AMD.,
We report a patient who developed Grade-4 RPE rip spontaneously in a CSC-associated large serous PED followed by its favorable healing response in one eye, and RPE aperture with bullous fibrinous CSC in a large serous PED in the fellow eye and its outcome following photodynamic therapy (PDT).
| Case Report|| |
A 38-year-old man presented with complaints of diminution of vision in his left eye (LE) for 2 months. He was on treatment for panic disorder for the past 1 year. His best-corrected visual acuity (BCVA) was 20/20 in the right eye (RE) and 20/30 in the LE. Anterior segment examinations of both eyes were within normal limits. Fundus examination of the LE showed a large crescent-shaped RPE rip located three disc-diameter temporal to fovea with inferior bullous exudative retinal detachment. Optical coherence tomography (OCT) of the LE showed a subfoveal serous PED with an RPE aperture on the nasal aspect, subretinal fluid (SRF) with fibrinous exudation, and a large extramacular RPE rip temporally [Figure 1]a and [Figure 1]b. Fluorescein angiography (FA) of the LE showed window defect corresponding to RPE rip as well as the RPE aperture, and an early hyperfluorescence with late leakage along the temporal edge of the RPE aperture [Figure 2]. The RE fundus showed a large serous PED, with FA and OCT confirming the same without any SRF and leakage [Figure 3]a, [Figure 3]b, and [Figure 4]a. A diagnosis of serous PED in the RE and bullous fibrinous CSC with subfoveal serous PED with aperture in the LE was made. The patient underwent low-fluence PDT in the LE with a setting of 25 J/cm2 energy, 300mW/cm2 irradiance, and 3600 μm spot size and 83 s. At the 1-month post-PDT visit, BCVA of the LE remained the same (20/30) with OCT showing healing RPE aperture, reduction in PED height, and resolving SRF, fibrin, and exudative retinal detachment [Figure 1]c and [Figure 1]d. At 6-month follow-up, the LE had improved to a BCVA of 20/20, completely resolved PED, healed RPE aperture, and normal foveal contour [Figure 1]e, [Figure 1]f, and [Figure 2]e.
|Figure 1: Left eye – OCT images: at presentation, subfoveal PED with serofibrinous subretinal exudation and the edge of the RPE rip on the temporal side (a), and RPE aperture (encircled area) within the serous PED superonasal to the fovea (b), At 1-month post-PDT, flattening PED (c), and healing RPE aperture with reduced subretinal exudation (d), at 6-month post-PDT, completely flattened PED and healed RPE aperture (e and f, respectively). OCT: Optical coherence tomography, PED: Pigment epithelial detachment, RPE: Retinal pigment epithelium, PDT: Photodynamic therapy|
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|Figure 2: Left eye: FA image at the first presentation showing window defect corresponding to large extramacular RPE rip and RPE aperture (encircled area) in the early phase (a), and leakage through RPE aperture with hyperfluorescence corresponding to RPE rip in the late phase (b). ICGA images showing hypocyanescence corresponding to the foveal PED and hypercyanescence through the rip in the early phase (c), and pooling of dye around RPE aperture and choroidal hyperpermeability in the late phase (d). Color fundus image at 6 months showing RPE atrophic changes at the macula and the scarred RPE rip (e). FA: Fluorescein angiography, PED: Pigment epithelial detachment, RPE: Retinal pigment epithelium, ICGA: Indocyanine green angiogram|
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|Figure 3: Right eye: Early phase FA and ICGA showing hypofluorescence corresponding to the asymptomatic bullous PED at initial presentation (a and b), Color fundus image, at 6 months, showing macula involving large crescent-shaped RPE rip (c), Mid-phase FA image at 6 months showing large window defect corresponding to the Grade-4 RPE rip with two focal leaks temporal to the rip (encircled areas) (d). FA: Fluorescein angiography, PED: Pigment epithelial detachment, RPE: Retinal pigment epithelium, ICGA: Indocyanine green angiogram|
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|Figure 4: Right eye – OCT images: bullous serous PED at the first visit (a); PED with spontaneous RPE rip (edges marked with white arrows) at 6 months (b); at 6-week posttreatment, flattened out PED with patchy islands of reappearing RPE and photoreceptor layer (white arrows) with hypertransmission defects seen in between the islands through foveal and juxtafoveal sections (c and d); at 1-year posttreatment, subfoveal reorganization of the RPE-photoreceptor complex and better continuity of the ELM and ellipsoid zones (white arrows) (e and f). PED: Pigment epithelial detachment, RPE: Retinal pigment epithelium, OCT: Optical coherence tomography, ELM: External limiting membrane|
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At 6 months of the first presentation, the patient developed a sudden diminution of vision in the RE with a BCVA of 20/120. Fundus examination of the RE showed a large crescent-shaped hypopigmented area, at the nasal edge of serous PED, suggestive of Grade-4 RPE rip involving the fovea with SRF and the same was confirmed on OCT [Figure 3]c and [Figure 4]b. A repeat FA of the RE revealed a crescent-shaped window defect across the fovea corresponding to the RPE rip and two extrafoveal active point leaks temporally [Figure 3]d. The patient underwent focal photocoagulation to the point leaks and was started on oral eplerenone (25 mg once daily (OD) for 1 week followed by 50 mg OD for 6 weeks). Follow-up OCT at 6 weeks showed resolved SRF and a healing pattern of the subfoveal RPE rip with patchy islands of the RPE and photoreceptor (PR) layer reappearing in the subfoveal region with hypertransmission defects seen in between the islands [Figure 4]c and [Figure 4]d.
On the past follow-up at 1 year, BCVA was improved to 20/30 and 20/20 in the RE and LE, respectively. OCT of the RE showed normal foveal contour with healing of the subfoveal RPE rip by reorganization of RPE-PR complex and islands of regenerating subfoveal PR layer coalesced giving better continuity of the external limiting membrane and ellipsoid zones with reduced hypertransmission defects in between [Figure 4]e and [Figure 4]f.
| Discussion|| |
Typical RPE rips, usually developing along the edge of the PED, appear as well-demarcated crescentic areas of the bare choroid with retracted and rolled torn edge of the RPE orienting itself parallel to the margin of the tear., Spontaneous rip develops in AMD following a sudden increase in the vascular permeability of the neovascular membrane resulting in sudden enlargement of PED leading to the rippling of the RPE, whereas sudden contraction of underlying vascular membrane following anti-VEGF therapy or PDT is implicated in the development of rip following treatment.,, Unlike AMD, the incidence of RPE tears in CSC is considerably lower owing to the absence of the contraction forces from the neovascular membrane and is usually atypical. Rips in serous PED usually result from the sudden increase in sub-RPE hydrostatic pressure and contraction of fibrinous exudation.
The foveal RPE rip in our case was atypical with only RPE retraction and without rolling off the edge. This could be explained due to the absence of underlying vascular membrane and consequently the absence of contractile force accounting for the same. Although RPE rips in CSC are reported more in younger patients after steroid exposure, rare cases of idiopathic CSC complicated with giant RPE rips have also been reported., In these eyes, rips may be associated with inferior exudative detachment and subretinal fibrin with no accompanying hemorrhage. Our case developed bilateral RPE rips, a large subfoveal rip (Grade-4) in the RE, and large extramacular rip (Grade-3) in the LE, in the setting of large serous PED with bullous CSC.
A unique finding, in this case, was the presence of coexisting foveal serous PED with RPE aperture along with an extramacular RPE rip in the LE, which is previously unreported. Originally, Querques et al. described RPE aperture in association with avascular PED, either serous or drusenoid, in AMD patients. The RPE apertures appear as round discontinuities in the RPE layer either at the apex or the base of the PED without any rippling or retraction of the RPE. It was described as focal atrophic loss of the RPE layer secondary to progressive atrophy of sub-RPE drusenoid deposits. Iovino et al. have recently described RPE aperture as a new finding in 17 eyes of 15 patients in the setting of chronic CSC. None of these patients were reported to have RPE tears other than aperture. They concluded apertures should be distinguished by RPE tears for the different pathogenesis and evolution in time.
The RPE rip healing in neovascular AMD is proposed to occur by several mechanisms including regeneration of the RPE cells, atrophy of the choriocapillaris, or fibrous scarring. Extramacular rips usually heal by fibrous scarring, whereas macula involving rips may heal either by fibrosis or RPE-PR regeneration depending on the underlying conditions. In regenerative healing, RPE cells repopulate centripetally from the edge of the tear to the center of the debrided area, except for the edge of the rolled RPE., However, the RPE migration and proliferation may not be in the right plane in AMD-related large RPE rips (Grades 3 and 4) leading to scarring and poor outcome. Therefore, Grade-4 rips (large rips involving fovea) are usually associated with worse visual prognosis. Sarraf et al. noted subfoveal fibrosis in 67% of the eyes with AMD-associated Grade-4 rips treated with anti-VEGF therapy. In our case, although Grade-3 extramacular rip in the LE healed by scarring, Grade-4 rip in the RE healed by regeneration and reorganization of RPE-photoreceptors suggesting that the healing mechanism may be different in macular and peripheral RPE rips. The long-term healing pattern of subfoveal Grade-4 RPE rips in CSC-associated nonvascularized PED has not been reported previously using spectral-domain OCT imaging documentation.
Although the exact mechanism leading to regenerative healing of Grade-4 rip in our case is unclear, the CSC-associated foveal RPE rips may have a better prognosis compared to the AMD-associated rips due to various factors., The absence of the RPE retraction or rolling, facilitates faster healing process as the entire edge of the tear participates in the RPE proliferation and migration. The healthy Bruch's membrane is a prerequisite for the RPE resurfacing. Unlike neovascular AMD, younger age and intact Bruch's membrane which acts like a scaffold for the RPE migration and may play an important role in spontaneous regenerative healing of CSC-associated rips. When the repair mechanisms of the RPE tears are considered, prompt treatment of the underlying disease is important. Mukai et al. proposed that early resolution of SRF after the RPE tear in neovascular AMD results in direct attachment of the outer retina to Bruch's membrane, whereas persistent SRF leads to subsequent repair with thickened proliferative fibrous tissue and poor outcome. In our case also, the prompt treatment with eplerenone and focal laser might have helped in the rapid resolution of SRF and contributed to the regenerative healing of the RPE rip with a good visual outcome.
The clinical course of the RPE rips or tears tends to be stable and not to enlarge after their onset, once the tractional forces cease to act. Conversely, the RPE apertures have been reported to have a progressive homogeneous increase in size over time, leading to the flattening of PED. This entity appears resistant to various treatment modalities including anti-VEGF agents and PDT., In the series of CSC-associated RPE apertures by Iovino et al., two patients underwent PDT, which led to the resolution of SRF; however, the RPE defect of aperture persisted. The RPE aperture in our patient showed good response to PDT leading to the closure of the RPE defect and subsequent complete regression of the subfoveal PED. The healing of the RPE aperture following PDT, as seen in our case, has not been previously reported.
| Conclusion|| |
We report a rare case of spontaneous Grade-4 RPE rip of foveal serous PED and fellow eye RPE aperture, in the setting of idiopathic bullous CSC, with the favorable regenerative healing pattern of both. Promptly treated Grade-4 RPE tears in CSC may have a better prognosis, due to various favorable pathophysiological mechanisms, compared to fovea-involving rips in neovascular AMD.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Gupta A, Chhabra M, Mehta P, Ghosh B. Tears of the retinal pigment epithelium – A review. Delhi J Ophthalmol 2017;27:243-9.
Sarraf D, Reddy S, Chiang A, Yu F, Jain A. A new grading system for retinal pigment epithelial tears. Retina 2010;30:1039-45.
Iovino C, Chhablani J, Parameswarappa DC, Pellegrini M, Giannaccare G, Peiretti E. Retinal pigment epithelium apertures as a late complication of longstanding serous pigment epithelium detachments in chronic central serous chorioretinopathy. Eye (Lond) 2019;33:1871-6.
Querques G, Capuano V, Costanzo E, Corvi F, Querques L, Introini U, et al.
Retinal pigment epithelium aperture: A previously unreported finding in the evolution of avascular pigment epithelium detachment. Retina 2016;36 Suppl 1:S65-72.
Ersoz MG, Karacorlu M, Arf S, Sayman Muslubas I, Hocaoglu M. Retinal pigment epithelium tears: Classification, pathogenesis, predictors, and management. Surv Ophthalmol 2017;62:493-505.
Clemens CR, Eter N. Retinal pigment epithelium tears: Risk factors, mechanism and therapeutic monitoring. Ophthalmologica 2016;235:1-9.
Parchand S, Gupta V, Gupta A, Dogra MR. Bilateral giant retinal pigment epithelial rip in idiopathic central serous chorioretinopathy. Retina 2011;31:1977-8.
Fok AC, Lai TY, Wong VW, Wong AL, Lam DS. Spontaneous resolution of retinal pigment epithelial tears and pigment epithelial detachment following blunt trauma. Eye (Lond) 2007;21:891-3.
Mukai R, Sato T, Kishi S. Repair mechanism of retinal pigment epithelial tears in age-related macular degeneration. Retina 2015;35:473-80.
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