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Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 346-348

A case of peripapillary pachychoroid syndrome treated with anti-vascular endothelial growth factor injections

Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, Chennai, Tamil Nadu, India

Date of Submission19-May-2020
Date of Acceptance07-Oct-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Parveen Sen
Department of Vitreoretina, Shri Bhagwan Mahavir Vitreoretinal Services, 18, College Road, Sankara Nethralaya, Chennai - 600 006, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1565_20

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Peripapillary pachychoroid syndrome (PPS) is a novel variant in the pachychoroid disease spectrum in which pachychoroid features are more prominent around the optic nerve head and are associated with intraretinal and/or subretinal fluid. The choroidal thickness in PPS is characterized by its atypical distribution with nasal macular choroid being thicker than the temporal counterpart. There are a few case reports of PPS in the literature that describe the morphological features of PPS but the management protocols are yet to be established. We hereby describe the clinical course of a 54-year-old gentleman who came to us with complaints of a gradual decrease in vision. On basis of multimodal imaging, a diagnosis of PPS was made. Since the patient was symptomatic, he was treated with intravitreal aflibercept which showed a favorable anatomical and functional outcome.

Keywords: Aflibercept in peripapillary pachychoroid syndrome, anti-VEGF injections, peripapillary pachychoroid syndrome, pachychoroid spectrum

How to cite this article:
Sen P, Sreenivasan J, Maitra P. A case of peripapillary pachychoroid syndrome treated with anti-vascular endothelial growth factor injections. Indian J Ophthalmol Case Rep 2021;1:346-8

How to cite this URL:
Sen P, Sreenivasan J, Maitra P. A case of peripapillary pachychoroid syndrome treated with anti-vascular endothelial growth factor injections. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 29];1:346-8. Available from: https://www.ijoreports.in/text.asp?2021/1/2/346/312333

The term “pachychoroid” was introduced by Warrow et al. in 2013 to describe a spectrum of diseases associated with a choroidal thickening.[1] Since then, the pachychoroid disease spectrum (PDS) has widened to include conditions like pachychoroid pigment epitheliopathy (PPE), central serous chorioretinopathy (CSCR), polypoidal choroidal vasculopathy (PCV), pachychoroid neovasculopathy (PNV), focal choroidal excavation (FCE), and peripapillary pachychoroid syndrome (PPS).[2],[3],[4],[5],[6],[7] Phasukkijwatana et al. first described PPS wherein the pachychoroid changes were more prominent in the temporal peripapillary area.[7] The exact clinical course and management protocols of PPS are yet to be established. Herein, we describe a case of PPS treated with anti-vascular endothelial growth factor (anti-VEGF) injections.

  Case Report Top

A 54-year-old male, presented with a complaint of defective vision in both eyes (OU) for 1-year duration. His best-corrected visual acuity (BCVA) in the right eye (OD) and left eye (OS) were 20/60 (+1D) and 20/25 (+1D), respectively. Fundus examination of OU [Figure 1]a and [Figure 1]b showed normal optic discs with cup-to-disc ratio (CDR) of 0.2 with retinal pigment epithelial (RPE) changes along the temporal peripapillary area and dull foveal reflex with few subretinal precipitates. Fundus autofluorescence (AF) showed stippled hyper-AF along the temporal peripapillary area and extending inferiorly in OD and the macular region in OS with increased hypo-AF at the fovea suggestive of RPE atrophy. Swept-source optical coherence tomography (SS-OCT- TOPCON DRI OCT Triton™) of OU showed cystic spaces at the fovea with retinal schisis extending from temporal margin of the disc to the fovea along with pachyvessels (dilated Haller's layer with compressed choriocapillaris) in the temporal peripapillary region [Figure 2]a and [Figure 2]b. Besides, SS-OCT in OS showed a flat irregular pigment epithelial detachment (FIPED) with subfoveal fluid [Figure 2]b. OCT-angiography (SS-OCTA, PLEX Elite, Carl Zeiss Meditec AG, Jena, Germany) did not detect an abnormal vascular network in either eye. Choroidal thickness (CT) was measured in three areas, namely, fovea, 1500 μ nasal (N1.5), and temporal (T1.5) to the fovea. CT at N1.5 was higher than the temporal counterparts with a ratio of N1.5/T1.5 as 1.76 in the OD and 2.36 in the OS, respectively [Figure 2]. Fluorescein angiogram (FFA) revealed RPE window defects in temporal peripapillary region OU [Figure 3]a and [Figure 3]b. No inkblot/smoke stack pattern of leakage was seen. Indocyanine green angiography (ICGA) in OU revealed leaking choroidal pachyvessels temporal to disc with no neovascular membranes/polyps [Figure 3]c and [Figure 3]d. Based on these features, a diagnosis of PPS was made.
Figure 1: Color fundus photo at the presentation of the right eye (a) and left eye (b) shows a normal optic disc with CDR of 0.2 with subretinal precipitates (black arrows). Blue autofluorescence image of right (c) eyes show showed stippled hyper-autofluorescence along the temporal peripapillary area and extending inferiorly and left eye (d) and in the macular region with increased hypo-autofluorescence at the fovea suggestive of RPE atrophy. Color fundus photo at last follow-up of the right eye (c) and left eye (d) showing reduction in precipitated (corresponding black arrows)

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Figure 2: SSOCT of the right eye (a) shows the schisis and pocket of subretinal fluid nasal to the fovea. SSOCT of the left eye (b) shows schisis nasal to the fovea, subretinal fluid, and cystoid spaces close to the fovea, flat irregular PED temporal to the fovea. Both eyes showed pachychoroid features more prominent in the peripapillary area, nasal to the fovea (asterisk). The choroidal thickness of the right eye measured at the fovea and 1500 μ (a) nasal (N1.5) and temporal (T1.5) to the fovea, and similar measurement in the left eye (b) showing a higher value nasal to the fovea than the temporal counterpart, with a ratio of N1.5/T1.5 as 1.76 in the right eye and 2.36 in the left eye, respectively.

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Figure 3: Fundus fluorescein angiography of the right eye (a) and left eye (b) show with window defects. Indocyanine green angiography of the right eye (c) and the left eye (d) show areas of choroidal hyperpermeability in the peripapillary region (red circle) but no abnormal avascular network or polyps. SSOCTA of right (e) and left eye (f) show no definite network at baseline before initiation of treatment

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Multiple areas of choroidal hyperpermeability surrounding the disc and peripapillary area with good BCVA discouraged the use of photodynamic therapy (PDT). At the first visit, the patient was advised intravitreal ranibizumab injection (IVR) only in OD. Post 1-month follow-up, a reduction in schitic spaces was noted in OD, with a slight increase in intraretinal fluid in OS. Due to some favorable anatomical response in OD [Figure 4]a and [Figure 4]b and an increase in subretinal fluid in OS in the absence of treatment [Figure 4]f, [Figure 4]g, subsequently patient received IVR in OU. Owing to incomplete response to therapy and persistent subretinal fluid at the end of four injections in each eye, a switch to intravitreal aflibercept injection (IAI) (Eylea, Regeneron) was made. The option of multi-spot PDT to the areas of choroidal hyperpermeability was also given to the patient. Given the better safety profile of IAI, a decision to treat with anti-VEGF monotherapy was taken. The patient received IAI q8 weeks with a total of five injections in OD and four in OS. SSOCT showed resolution of in schitic spaces and reduction in subretinal fluid (SRF) pocket in the temporal peripapillary area in OD, and reduction of schisis and flattening of FIPED in OS [Figure 4]. At the last follow-up, there was a reduction in precipitates [Figure 1]c and [Figure 1]d and BCVA improved to 20/30 in the OD and maintained 20/25 in the OS.
Figure 4: Response to anti-VEGF injection in the right eye (a to e) and left eye initial observation (4f to g) and later response to anti-VEGF (h to j). The right eye shows the resolution of schitic cavities and the reduction of the juxtapapillary SRF pocket. The left eye shows an initial increase in SRF on observation and later showed resolution of SRF, flattening of PED, and reduction in cystoid spaces close to the fovea in response to anti-VEGF. Also, the choroid appears more compact after treatment with IAI (e and j)

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  Discussion Top

PDS is characterized by dilated large choroidal vessels with attenuation of the overlying choriocapillaris, associated progressive RPE changes.[2],[3] Regional variation of choroidal thickness has been described, with the thickest region being subfoveally and thinnest nasally.[2],[8]

Phasukkijwatana et al. first reported PPS as a variant of PDS, in which the nasal macular choroid showed pachyvessels associated with intraretinal and/or subretinal fluid and was disproportionately thicker than those with typical PDS or control eyes, and thicker than the temporal macular choroid of the same eye.[7]

In our patient, ratios of choroidal thickness at N1.5/T1.5 (OD-1.77, OS-2.36) and N3/T3 (OD-2.08, OS-2.01OD) were higher than those described by Phasukkijwatana et al.[7] Associations of PPS described in the study were male gender, crowded disc, and hyperopia which were noted in our case too. Moreover, 70% of PPS eyes were seen to have serous PEDs or gravitational tracks.[7] Our case showed features of the pachychoroid disease spectrum with FIPED and RPE alterations predominantly in the peripapillary area. This typical choroidal thickness profile pointed towards PPS. The patient was symptomatic and Fundus autofluorescence (FAF) pictures in OU revealed the presence of areas of hypoautofluoresce (OS > OD) suggestive of RPE atrophy setting in due to the long-term presence of fluid and leakage, hence necessitating treatment.

No definitive treatment options for PPS have been established although topical dorzolamide and PDT have shown some benefit in a few cases.[7] In absence of a demonstrable Choroidal neovascular membrane (CNVM) on Swept source optical coherence tomography angiography(SSOCTA), PDT appeared to be the next rational option. However, the peripapillary distribution as well as a large area of choroidal hyperpermeability on ICGA would have necessitated extensive PDT. On the other hand, intravitreal anti-VEGF injections, which have been shown to reduce disease activity, choroidal vascular permeability, and thickness in other PDS entities like PNV and PCV, was offered as a safer treatment option.[9],[10],[11] Even in absence of CNV, choroidal hyperpermeability on ICG is hypothesized to indicate structural choriocapillaris damage producing relative ischemia.[2] Moreover, the useful role of anti-VEGF, especially Aflibercept in absence of CNV in pachychoroid diseases such as chronic CSC has been reported though its rationale is not fully understood.[12] Hence, we considered anti-VEGF monotherapy for this patient. Following unsatisfactory anatomical improvement with multiple IVRs, we switched to IAI, which has shown good outcomes as monotherapy in other PDS conditions like PCV.[1] To our knowledge, this is the first case report showing a favorable anatomical and functional response to IAI in PPS. However, long-term follow-up is necessary as recurrences of the intraretinal, and subretinal fluid may be seen as the effect of IAI wears away.

  Conclusion Top

To conclude, PPS is a part of the pachychoroid spectrum with a distinct choroidal thickness profile which has features that may overlap with other PDS entities like CSCR and PCV. In the absence of better treatment options, intravitreal anti-VEGF injections, especially IAI can be considered in PPS in selected cases given the chronicity of symptoms, bilateral involvement, the persistence of fluid, can lead to vision loss or impairement and worsening of vision as definitive management protocols which are yet to be established for this condition.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Warrow DJ, Hoang QV, Freund KB. Pachychoroid pigment epitheliopathy. Retina 2013;33:1659-72.e.  Back to cited text no. 1
Cheung CMG, Lee WK, Koizumi H, Dansingani K, Lai TYY, Freund KB. Pachychoroid disease. Eye (Lond) 2019;33:14-33.  Back to cited text no. 2
Lee WK, Baek J, Dansingani KK, Lee JH, Freund KB. Choroidal morphology in eyes with polypoidal choroidal vasculopathy and normal or subnormal subfoveal choroidal thickness. Retina 2016;36:S73-82.  Back to cited text no. 3
Pang CE, Freund KB. Pachychoroid neovasculopathy. Retina 2015;35:1-9.  Back to cited text no. 4
Margolis R, Mukkamala SK, Jampol LM, Spaide RF, Ober MD, Sorenson JA, et al. The expanded spectrum of focal choroidal excavation. Arch Ophthalmol. 2011;129:1320-5.  Back to cited text no. 5
Chung H, Byeon SH, Freund KB. Focal choroidal excavation and its association with pachychoroid spectrum disorders: A review of the literature and multimodal imaging findings. Retina 2017;37:199-221.  Back to cited text no. 6
Phasukkijwatana N, Freund KB, Dolz-Marco R, Al-Sheikh M, Keane PA, Egan CA, et al. Peripapillary pachychoroid syndrome. Retina 2018;38:1652-67.  Back to cited text no. 7
Margolis R, Spaide RF. A pilot study of enhanced depth imaging optical coherence tomography of the choroid in normal eyes. Am J Ophthalmol 2009;147:811-5.  Back to cited text no. 8
Padrón-Pérez N, Arias L, Rubio M, Lorenzo D, García-Bru P, Català-Mora J, et al. Changes in choroidal thickness after intravitreal injection of anti-vascular endothelial growth factor in pachychoroid neovasculopathy. Invest Ophthalmol Vis Sci 2018;59:1119-24.  Back to cited text no. 9
Oishi A, Kojima H, Mandai M, Honda S, Matsuoka T, Oh H, et al. Comparison of the effect of ranibizumab and verteporfin for polypoidal choroidal vasculopathy: 12-month LAPTOP study results. Am J Ophthalmol 2013;156:644-51.  Back to cited text no. 10
Wong TY, Ogura Y, Lee WK, Iida T, Chen SJ, Gemmy Cheung CM, et al; PLANET Investigators. Efficacy and safety of intravitreal aflibercept for polypoidal choroidal vasculopathy: Two-year results of the aflibercept in polypoidal choroidal vasculopathy study. Am J Ophthalmol 2019;204:80-9.  Back to cited text no. 11
Tekin K, Cakar Ozdal P, Gulpamuk B, Yasin Teke M. Intravitreal aflibercept therapy in eyes with chronic central serous chorioretihopathy: Short term results. Arch Soc Esp Oftalmol 2018;93:315-23.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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