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PHOTO ESSAY |
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Year : 2021 | Volume
: 1
| Issue : 2 | Page : 210-211 |
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A tale of two leaks – Pachychoroid spectrum
Manavi D Sindal, Bholesh Ratna
Vitreoretina Services, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Pondicherry, India
Date of Submission | 16-Jul-2020 |
Date of Acceptance | 21-Sep-2020 |
Date of Web Publication | 01-Apr-2021 |
Correspondence Address: Dr. Manavi D Sindal Vitreoretina Services, Aravind Eye Hospital, Thavalakuppam, Cuddalore Main Road, Pondicherry - 605 007 India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ijo.IJO_2323_20
Keywords: Central serous chorioretinopathy, pachychoroid spectrum, polypoidal choroidal vasculopathy
How to cite this article: Sindal MD, Ratna B. A tale of two leaks – Pachychoroid spectrum. Indian J Ophthalmol Case Rep 2021;1:210-1 |
Case Report | |  |
A 65-year-old female presented with OS disciform scar and OD hemorrhagic pigment epithelial detachment (PED) with subretinal fluid (SRF) at the inferior arcade, with no fluid at macula [Figure 1]. Best-corrected visual acuity (BCVA) at presentation was 6/9 OD and hand movements in OS. Simultaneous Fundus fluorescein angiography (FFA) and Indocyanine green angiography (ICGA) revealed polyps near inferior arcade establishing the diagnosis of polypoidal choroidal vasculopathy (PCV) [Figure 2]. She received anti-VEGF injections with focal laser to the extrafoveal polyps with good response. She was on regular follow-up with OCT monitoring. New-onset SRF with new PEDs at macula were noted 16 months later with BCVA reduction to 6/18. She received an anti-VEGF injection [Figure 3]a. At 1-month follow -up, there was increased SRF [Figure 3]b and further reduction of BCVA to 6/24. She underwent FFA and ICGA which showed a hot spot corresponding to polyp on the ICGA, a smokestack leak corresponding to central serous chorioretinopathy (CSCR) on FFA with a common PED in between [Figure 4]a and [Figure 4]b. She underwent focal laser to both lesions along with anti-VEGF, with resolution of SRF at 1 month [Figure 3]c and improvement of BCVA to 6/12. She has thereafter been on regular follow up for 45 months with monitoring on regular OCT scans. She developed recurrent subretinal and intra-retinal fluid four months later and has received 12 additional anti-VEGF injections over this time period maintaining BCVA of 6/12. There was no recurrence of CSCR during this follow-up. | Figure 1: (a) Spectral domain optical coherence tomography OD imaging passing above inferior arcade showing pigment epithelial detachment. (b) Scan through macula shows normal foveal architecture
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 | Figure 2: (a) Early phase and (b) late phase of combined fundus fluorescein and indocyanine green angiography showing a polyp like lesion near inferior arcade with a large serosanguinous pigment epithelial detachment
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 | Figure 3: (a) Spectral domain optical coherence tomography OD showing pachychoroid, pigment epithelial detachment and subretinal fluid. (b) Increased subretinal fluid following anti-VEGF injection. (c) Post-focal laser resolution of subretinal fluid with decreased height of PED is seen
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 | Figure 4: (a) Fundus fluorescein angiography (FFA) and indocyanine green angiography (ICG) OD. The FFA shows focal hyperfluorescence supero-temporal to fovea and the ICG shows a separate hot spot. The common intervening pigment epithelial detachment appears hyperfluorescent on FFA and hypofluorescent on ICG. (b) Late phase angiogram showing smoke stack appearance of the leak on FFA. Note inferiorly the scarring from previous polyps and resolved hemorrhagic pigment epithelial detachment
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Discussion | |  |
CSCR and PCV both represent components of the pachychoroid spectrum. Pachychoroid is the phenotype where there is focal or diffuse choroidal thickening. This results primarily from dilated vessels in Haller's layer, with thinning of Sattler's layer and choriocapillaries.[1] The pachychoroid leads to a hyperpermeable state, which if not compensated by the retinal pigment epithelium, results in fluid accumulation and CSCR. Chronic choroidal vascular hypertension can result in the formation of polyps.[2] Yannuzzi et al. reported the occurrence of polyps in cases previously diagnosed as CSCR.[3] Manayath et al. have reported the co-existence of CSCR like leaks with polyps.[4] This case highlights the occurrence of new polys in a secondary location after successful treatment of initial extrafoveal polyps. Combined FFA & ICG enabled us to determine the cause for the paradoxical increase in fluid,[4],[5] enabling prompt treatment. Focal laser does not address the branching vascular network component of PCV, and polyp regression can be partial. Both of these factors can contribute to recurrence or continued disease activity.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References | |  |
1. | Cheung CMG, Lee WK, Koizumi H, Dansingani K, Lai TYY, Freund KB. Pachychoroid disease. Eye (Lond) 2019;33:14-33. |
2. | Balaratnasingam C, Lee WK, Koizumi H, Dansingani K, Inoue M, Freund KB. Polypoidal choroidal vasculopathy: A distinct disease or manifestation of many? Retina 2016;36:1-8. |
3. | Yannuzzi LA, Freund KB, Goldbaum M, Scassellati-Sforzolini B, Guyer DR, Spaide RF, et al. Polypoidal choroidal vasculopathy masquerading as central serous chorioretinopathy. Ophthalmology 2000;107:767-77. |
4. | Manayath GJ, Shah VS, Saravanan VR, Narendran V. Polypoidal choroidal vasculopathy associated with central serous chorioretinopathy: Pachychoroid spectrum of diseases. Retina 2018;38:1195-204. |
5. | Ayachit G, Ayachit A, Nadgir H, Joshi S. Validating the pachychoroid disease spectrum using multimodal imaging. Indian J Ophthalmol 2018;66:1022-4.  [ PUBMED] [Full text] |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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