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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 1  |  Page : 123-126

Active concurrent central serous chorioretinopathy and pachychoroid neovasculopathy managed with combination therapy of photodynamic therapy and anti-vascular endothelial growth factor agent


Department of Vitreo Retina, Aditya Birla Sankara Nethralaya, Mukundapur, Kolkata, West Bengal, India

Date of Submission04-Apr-2020
Date of Acceptance10-Aug-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Dr. Rupak Roy
Aditya Birla Sankara Nethralaya, 147, Mukundapur, E.M. Bypass, Kolkata - 700 099, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_836_20

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  Abstract 


Pachychoroid disease spectrum includes central serous chorioretinopathy (CSR) and pachychoroid neovasculopathy (PNV). Pachychoroid phenotype is characterized by choroidal hyperpermeability, dilated choroidal vessels (pachyvessels), and focal or diffuse increase in choroidal thickness. Newer imaging modalities have aided in the diagnosis of choroidal diseases. Herein, we present a case of active concurrent CSR with PNV in a middle-aged patient with successful treatment by photodynamic therapy and intravitreal anti-vascular endothelial growth factor (VEGF) (ranibizumab) injection.

Keywords: Central serous chorioretinopathy, fluorescein angiography, indocyanine green angiography, optical coherence tomography angiography, pachychoroid, pachychoroid neovasculopathy


How to cite this article:
Garg B, Gupta I, Goel S, Senger D, Saurabh K, Roy R. Active concurrent central serous chorioretinopathy and pachychoroid neovasculopathy managed with combination therapy of photodynamic therapy and anti-vascular endothelial growth factor agent. Indian J Ophthalmol Case Rep 2021;1:123-6

How to cite this URL:
Garg B, Gupta I, Goel S, Senger D, Saurabh K, Roy R. Active concurrent central serous chorioretinopathy and pachychoroid neovasculopathy managed with combination therapy of photodynamic therapy and anti-vascular endothelial growth factor agent. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Mar 7];1:123-6. Available from: https://www.ijoreports.in/text.asp?2021/1/1/123/305541



Pachychoroid disease is a newly described disease entity. It was first described by Warrow et al. in 2013.[1] He defined pachychoroid pigment epitheliopathy (PPE) as a forme fruste of central serous chorioretinopathy (CSR). Pachychoroid phenotype is characterized by choroidal hyperpermeability, dilated choroidal vessels (pachyvessels), and focal or diffuse increase in choroidal thickness. It was reported by Dansingani et al. that eyes with pachychoroid phenotype had choroidal thicknesses more than 300 μm or they have an extrafoveal focus exceeding the choroidal thickness of the fovea by at least 50 μm.[2] Diseases included in the pachychoroid spectrum are uncomplicated pachychoroid, PPE, CSR, pachychoroid neovasculopathy (PNV), and polypoidal choroidal vasculopathy.[3]

PPE and CSR are stated to be initial manifestations of the pachychoroid spectrum, whereas PNV is an end-stage disease manifestation. The present case highlights a case of an active CSR with PNV in a pachychoroid eye and its successful management with photodynamic therapy (PDT) and anti-VEGF (ranibizumab).


  Case Report Top


A 42-year-old male patient presented with complaints of diminution of vision and distortion of images in the left eye for 1 month. He did not have any history of systemic diseases. On examination, his best-corrected visual acuity in the right eye and left eye was 20/20, N6, and 20/30, N6, respectively. The anterior segment was unremarkable in both eyes. On fundus examination, there was retinal pigment epithelium (RPE) changes over macula in both eyes [Figure 1]a and [Figure 1]b. Multicolor image highlighted the area of RPE alterations [Figure 1]c and [Figure 1]d. In the right eye, fundus fluorescein angiography (FFA) showed few hyperfluorescent window defects at the foveal region [Figure 2]a and Indocyanine green angiography (ICG) showed few dilated choroidal vessels [Figure 2]b. In the left eye, pinpoint hyperfluorescence was noted temporal to fovea and stippled hyperfluorescence at fovea [Figure 2]c with the corresponding dilated choroidal vessels on ICG [Figure 2]d. FFA of the left eye showed inkblot leak temporal to the fovea in the late phase [Figure 2]e. ICG of the left eye showed dilated choroidal vessels at the corresponding area of inkblot leak and few hypercyanescence spots along with dilated choroidal vessels at the fovea in late phase [Figure 2]f.
Figure 1: Color fundus photo of the right (a) and left eye (b) showing RPE alterations at the macula (white arrows). Multicolor image of right (c) and left eye (d) highlights the area of RPE alterations (black arrows)

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Figure 2: FFA of the right eye (a) showing window defects. ICG of the right eye (b) showing few dilated choroidal vessels. FFA of the left eye (c) in the early phase showing pinpoint hyperfluorescence temporal to fovea (black arrow) and stippled hyperfluorescence at the fovea (white arrow) with the corresponding dilated choroidal vessels on ICG (d). FFA of the left eye (e) in late phase showing inkblot leak temporal to fovea (black arrow). ICG of the left eye (f) showing dilated choroidal vessels at corresponding area of inkblot leak (white arrow) and few hypercyanescence spots along with dilated choroidal vessels at the fovea (black arrow)

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Enhanced depth imaging optical coherence tomography (EDI OCT) of the right eye showed dry fovea with dilated choroidal vessels [Figure 3]a and left eye had minimal subfoveal subretinal fluid (SRF) with a double-layer sign with SRF temporal to fovea and dilated choroidal vessels [Figure 3]b. The subfoveal choroidal thickness (SFCT) in the right and left eye was 263 μ and 363 μ, respectively. The EDI OCT of the left eye showed subretinal fluid supero-temporal to the fovea corresponding to the location of the inkblot leak on FFA [Figure 3]c and [Figure 3]d. On OCT angiography (OCTA), the left eye showed a subfoveal network of hyperreflective, tangled, filamentous vessels in the neovascular complex at the level of choriocapillaris [Figure 3]e, the corresponding B scan segmentation of OCTA is demonstrated by the purple line [Figure 3]f. A diagnosis of PNV in the case of active CSR was established. The patient was advised and underwent reduced fluence PDT to the subfoveal area of new vessels and the inkblot leak along with intravitreal anti-VEGF injection (ranibizumab) in the left eye (48 h after PDT). At 2 months posttreatment follow-up, the vision had increased to 20/20 with a significant decrease in metamorphopsia with a dry macula on OCT and reduced SFCT to 223 μ in the left eye [Figure 4]b. The right eye showed RPE alterations [Figure 4]a. There is no recurrence on follow-up after 1 year.
Figure 3: EDI OCT of the right eye (a) showing dry fovea with a subfoveal choroidal thickness of 263 μ. EDI OCT of the left eye (b) showing minimal subfoveal subretinal fluid, double-layer sign (white arrow), with subretinal fluid temporal to fovea (red arrow), and subfoveal choroidal thickness of 363 μ. Infrared reflectance image of the left eye (c) showing green line passing through the location of leak supero-temporal to the fovea with EDI OCT (d) showing corresponding subretinal fluid. OCTA of the left eye (e) demonstrating hyperreflective, tangled, filamentous new vessels at the level of the choriocapillaris (white arrow). The corresponding B scan segmentation of OCTA is demonstrated by purple line (f)

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Figure 4: EDI OCT at 2 months follow-up showing RPE alterations in the right eye (a) and complete resolution of subretinal fluid with reduced subfoveal choroidal thickness to 223 μ in left eye (b)

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  Discussion Top


The advent of newer EDI OCT has provided new insights into a variety of chorioretinal disorders.[4] Owing to improved instrumentation, a quantitative assessment of choroid has become indispensable to understand the pathogenesis of retinal diseases which are found to have major choroidal involvement. The term patchy, meaning thick, has been derived from the Greek literature. It has been used to describe a group of choroidal findings that point towards a spectrum of related retinal disorders, namely, the pachychoroid spectrum of diseases.[2],[3] PNV is a newly described entity in this spectrum. Long-standing RPE changes and possibly RPE detachment can lead to disruption of Bruch's membrane, leading to ingrowth of sub-RPE choroidal neovascular membrane (CNV), resulting in Type 1 CNV. These neovascular membranes overlie areas of the thickened choroid with dilated outer choroidal vessels and have been termed “pachychoroid neovasculopathy” and this pachychoroidopathy suggests that a pathologic choroidal process characterized by dilated outer choroidal vessels and thick choroid is the underlying common denominator.[3] The following findings point towards the diagnosis of PNV – the absence of drusen on fundus examination, presence of double-layer sign (Type 1 CNVM), focal or diffuse choroidal thickening along with SRF on EDI OCT, late phase stippled hyperfluorscence on FFA with dilated choroidal vessels on ICG and presence of a network of tangled neo vessels on OCTA. OCTA is a relatively new diagnostic modality through which we can noninvasively visualize retinal and choroidal circulation without the use of dye. Recent studies have described OCTA features of PNV. Azhar et al. has described tangled filamentous vessels on OCTA in cases of PNV.[5]

Treatment of PNV is evolving. Lee et al. showed that adjunctive PDT in eyes with Type 1 neovascularization with thickened choroid that were refractory to anti-VEGF monotherapy resulted in complete fluid absorption in most eyes, which translated to visual improvement until 1 year.[6] Hata et al. and Matsumoto et al. have described treatment outcomes of PNV with ranibizumab and aflibercept.[7],[8] The optimum treatment regimen of PNV is yet to be formulated. Since it is primarily a pachychoroid driven process, the use of PDT in its treatment seems rational. PDT induces choroidal hypoperfusion which subsequently leads to reduced choroidal thickness. Though a pachychoroid driven process, there is evidence of VEGF release in this entity as shown by Hata et al.[7] In a recent case series by Roy et al., PDT with anti-VEGF had been used as a primary treatment for PNV with promising results.[9] To address both thickened choroid and VEGF release, we decided to use a combination therapy of PDT and anti-VEGF (ranibizumab). We achieved excellent outcomes to combined therapy with complete resolution of subretinal fluid and restoration of visual acuity. Larger studies are required to formulate the optimal treatment strategy for eyes with PNV.


  Conclusion Top


The present case highlights the coexistence of both the entities in a single case and its successful management with combination therapy of PDT with anti-VEGF (ranibizumab) combination.

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 Top

1.
Warrow DJ, Hoang QV, Freund KB. Pachychoroid pigment epitheliopathy. Retina 2013;33:1659-72.  Back to cited text no. 1
    
2.
Dansingani KK, Balaratnasingam C, Naysan J, Freund KB. En face imaging of pachychoroid spectrum disorders with swept-source optical coherence tomography. Retina 2016;36:499-516.  Back to cited text no. 2
    
3.
Pang CE, Freund KB. Pachychoroid neovasculopathy. Retina 2015;35:1-9.  Back to cited text no. 3
    
4.
Spaide RF, Koizumi H, Pozzoni MC. Enhanced depth imaging spectral-domain optical coherence tomography. Am J Ophthalmol 2008;146:496-500.  Back to cited text no. 4
    
5.
Azar G, Wolff B, Mauget-Faÿsse M, Rispoli M, Savastano MC, Lumbroso B. Pachychoroid neovasculopathy: Aspect on optical coherence tomography angiography. Acta Ophthalmol 2017;95:421-7.  Back to cited text no. 5
    
6.
Lee JH, Lee WK. One-year results of adjunctive photodynamic therapy for type 1 neovascularization associated with thickened choroid. Retina 2016;36:889-95.  Back to cited text no. 6
    
7.
Hata M, Yamashiro K, Ooto S, Oishi A, Tamura H, Miyata M, et al. Intraocular vascular endothelial growth factor levels in pachychoroid neovasculopathy and neovascular age-related macular degeneration. Invest Ophthalmol Vis Sci 2017;58:292-8.  Back to cited text no. 7
    
8.
Matsumoto H, Hiroe T, Morimoto M, Mimura K, Ito A, Akiyama H. Efficacy of treat-and-extend regimen with aflibercept for pachychoroid neovasculopathyand Type 1 neovascular age-related macular degeneration. Jpn J Ophthalmol 2018;62:144-50.  Back to cited text no. 8
    
9.
Roy R, Saurabh K, Shah D, Goel S. Treatment outcomes of pachychoroid neovasculopathy with photodynamic therapy and anti-vascular endothelial growth factor. Indian J Ophthalmol 2019;67;1678-83.  Back to cited text no. 9
    


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



 

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