Ophthalmic Surgery, Lasers and Imaging Retina

Case Report 

Focal Choroidal Excavation Expansion Following Treatment of Associated Choroidal Neovascular Membrane

Kyle D. Kovacs, MD; Luis A. Gonzalez, MD, MPH; Stephanie J. Weiss, DO; Szilard Kiss, MD; Anton Orlin, MD

Abstract

The authors describe a case of a previously unreported phenomenon of focal choroidal excavation (FCE) expansion, in absence of inflammation, due to treatment of an associated choroidal neovascular membrane (CNVM). A patient with new type 2 CNVM, treated during 43 months of follow-up with aflibercept, experienced significant expansion of an FCE with conversion from nonconforming to conforming type. FCE is part of the pachychoroid spectrum and the regression of an associated CNVM during aflibercept treatment as seen in the authors' patient may elucidate the pathogenesis of some forms of focal choroidal excavation and their evolution over time.

[Ophthalmic Surg Lasers Imaging Retina. 2020;51:54–57.]

Abstract

The authors describe a case of a previously unreported phenomenon of focal choroidal excavation (FCE) expansion, in absence of inflammation, due to treatment of an associated choroidal neovascular membrane (CNVM). A patient with new type 2 CNVM, treated during 43 months of follow-up with aflibercept, experienced significant expansion of an FCE with conversion from nonconforming to conforming type. FCE is part of the pachychoroid spectrum and the regression of an associated CNVM during aflibercept treatment as seen in the authors' patient may elucidate the pathogenesis of some forms of focal choroidal excavation and their evolution over time.

[Ophthalmic Surg Lasers Imaging Retina. 2020;51:54–57.]

Introduction

Focal choroidal excavation (FCE), first described by Jampol et al. and expanded by Margolis et al., is an increasingly recognized clinical entity with a broadening spectrum of associations and variants.1,2 Since their initial description, FCEs have been described in association with choroidal neovascular membranes (CNVMs) and inflammatory choroidopathies, as well as diseases associated with the pachychoroid spectrum of findings including central serous chorioretinopathy and polypoidal choroidal vasculopathy.3–5 Although there is some speculation that FCEs can sometimes represent congenital defects, others suggest an association with choroidal processes, inflammatory or vascular, that can lead to focal choroidal vascular abnormalities or weaknesses in Bruch's membrane, allowing for outward herniation of retinal tissue.6,7 Here we present a case of a type 2 CNVM presenting in association with FCE, in absence of active associated inflammation, treated with multiple injections of intravitreal aflibercept (Eylea; Regeneron, Tarrytown, NY). During 43 months of follow-up with treatment of the CNVM, the FCE was noted to increase substantially in depth and breadth, a progression not previously reported in noninflammatory FCE.

Case Report

A 47-year-old woman presented to our Retina Service with 1 week of new onset metamorphopsia in the right eye. She had low myopia (−0.50 spherical equivalent) with no other past ocular or medical history and took no medications. Her vision was 20/80 in the right eye and 20/20 in the left eye, with intraocular pressures of 12 mm Hg in both eyes. The dilated fundus examination revealed retinal elevation with associated hemorrhage in the superonasal macula involving the fovea in the right eye, with retinal pigment epithelium (RPE) mottling temporal to fovea in the left eye. Indocyanine green angiography (ICGA) (Figure 1) and fluorescein angiography revealed the presence of a type 2 CNVM adjacent to the area of hemorrhage superonasal to the fovea in the right eye (Figure 2). Leakage was noted only in the area affected by the CNVM in the right eye, without any other signs of an acute inflammatory process including significant choroidal leakage on ICGA. Optical coherence tomography (Figure 3) demonstrated subretinal fluid (SRF) and hyperreflective material adjacent to the identified choroidal neovascular membrane superonasal to fovea, with intraretinal extension of associated hemorrhage as well as a shallow focal choroidal excavation temporal to this lesion. All images were acquired on a Heidelberg Spectralis device (Heidelberg Engineering, Heidelberg, Germany).

Right eye indocyanine green angiography at initial presentation demonstrating hyperfluorescent foci indicative of a choroidal neovascular membrane. The fovea and area of choroidal excavation is under an area of hypofluorescence due to a blocking defect from the overlying subretinal hemorrhage. There is no diffuse leakage suggestive of inflammation of the choroid.

Figure 1.

Right eye indocyanine green angiography at initial presentation demonstrating hyperfluorescent foci indicative of a choroidal neovascular membrane. The fovea and area of choroidal excavation is under an area of hypofluorescence due to a blocking defect from the overlying subretinal hemorrhage. There is no diffuse leakage suggestive of inflammation of the choroid.

Fluorescein angiography of the right eye 6:23 after dye injection. There is a blocking defect from overlying hemorrhage, with late leakage from a choroidal neovascular membrane superonasal to the fovea.

Figure 2.

Fluorescein angiography of the right eye 6:23 after dye injection. There is a blocking defect from overlying hemorrhage, with late leakage from a choroidal neovascular membrane superonasal to the fovea.

Spectral-domain optical coherence tomography of the right eye at initial presentation demonstrating small area of focal choroidal excavation (measured to be approximately 45 μm) with adjacent type 2 choroidal neovascular membrane located nasally with subretinal fluid and hyperreflective material.

Figure 3.

Spectral-domain optical coherence tomography of the right eye at initial presentation demonstrating small area of focal choroidal excavation (measured to be approximately 45 μm) with adjacent type 2 choroidal neovascular membrane located nasally with subretinal fluid and hyperreflective material.

A diagnosis of an active exudative CNVM was made and treatment was initiated with monthly aflibercept injections. The patient responded well to treatment with resolution of SRF at month 3, better exposing the presence of a conforming-type FCE. During the subsequent years of treatment, the patient was injected with aflibercept as needed when there was recurrent CNVM activity with SRF. Over time, the depth and diameter of the FCE significantly increased, confounding the assessment of recurrent CNVM activity versus the development of nonconforming FCE due to the altered choroidal architecture. At last follow-up 43 months after initial presentation, the patient had visual acuity of 20/25 in the affected eye with last aflibercept injection 11 weeks prior, with marked expansion of the FCE compared with her initial presentation (Figure 4) and some persistent SRF, but relative preservation of the outer retinal architecture. Enhanced depth imaging OCT obtained at more recent follow-up demonstrated choroidal thicknesses of 401 μm underneath an area of excavation of depth 160 μm in the right eye and 501 μm in the left eye, along with notable pachyvessels in both eyes, consistent with an underlying pachychoroid pathophysiology (Figures 5-6 ).

Spectral-domain optical coherence tomography of the right eye 43 months after initial presentation. This B-scan is registered with that from initial presentation to deliver the same anatomic segment of retina. There is significant outwards excavation of the choroid compared with initial presentation (now measuring to be at least 165 μm in depth), and still some subretinal fluid with relatively preserved outer retinal architecture overlying the excavation.

Figure 4.

Spectral-domain optical coherence tomography of the right eye 43 months after initial presentation. This B-scan is registered with that from initial presentation to deliver the same anatomic segment of retina. There is significant outwards excavation of the choroid compared with initial presentation (now measuring to be at least 165 μm in depth), and still some subretinal fluid with relatively preserved outer retinal architecture overlying the excavation.

Enhanced depth imaging spectral-domain optical coherence tomography of the right eye 43 months after initial presentation demonstrating choroidal thickness of 401 μm with overlying choroidal excavation measuring 160 μm. Pachyvessels are also present.

Figure 5.

Enhanced depth imaging spectral-domain optical coherence tomography of the right eye 43 months after initial presentation demonstrating choroidal thickness of 401 μm with overlying choroidal excavation measuring 160 μm. Pachyvessels are also present.

Enhanced depth imaging spectral-domain optical coherence tomography of the left eye 43 months after initial presentation demonstrating choroidal thickness of 501 μm. Pachyvessels are also present.

Figure 6.

Enhanced depth imaging spectral-domain optical coherence tomography of the left eye 43 months after initial presentation demonstrating choroidal thickness of 501 μm. Pachyvessels are also present.

Discussion

This patient presented with features of pachychoroid-spectrum including FCE and thickened choroid with large choroidal pachyvessels. She presented with a symptomatic type 2 CNVM, which has remained active throughout the patient's clinical course. Freund et al. previously described the entity of pachychoroid epitheliopathy, which is typically associated with type 1 CNVM lesions; however, they also described a case of type 1 CNVM associated with a FCE and pachychoroid.8 In their case, though, the FCE was not noted to change size or character demonstrably following anti-vascular endothelial growth factor (VEGF) therapy. As such, our patient's FCE expansion with anti-VEGF treatment alone represents a novel clinical course in the absence of acute inflammation.9

Matsubara et al. suggested that some forms of FCE possibly evolve due to an imbalance between intraocular and choroidal pressure, with concurrent RPE and basement membrane weakness.6 During periods of choroidal inflammation there is engorgement and swelling of the choroid, which, as it resolves, can expose the presence of or expand a known FCE. Our patient carried no history of prior episodes of inflammation or visual disturbance. On imaging there were no signs of active chorioretinal inflammation apart from those associated with the CNVM lesion itself. This case's type 2 lesion did not demonstrate a classic “pitchfork sign” associated with uveitis-associated CNVM.10 However, the underlying etiology of the FCE in our patient remains unclear as it is possible that the patient could have had a prior subclinical inflammatory episode causing the formation of an FCE versus a congenital lesion.

Regardless of the etiology of the FCE in this instance, the change in choroidal architecture with treatment of the CNVM remains atypically dramatic given the absence of acute inflammation. We hypothesize that the collapse of the CNVM, including regression of some sub-RPE component in addition to the readily visible type 2 subretinal lesion, during aflibercept treatment provided a pressure gradient that drove the outward retinal herniation. The RPE and basement membrane weakness in the FCE served as a hinge in the setting of this pressure gradient, allowing further herniation not typically seen in CNVM treatment. As such, this represents the first case of FCE expansion with anti-VEGF treatment of an associated CNVM in the absence of associated inflammatory conditions. The clinical course presents a novel progression of an FCE-associated CNVM with pachychoroid features and may glean insight into the pathogenesis and natural history of different forms of FCE.

References

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Authors

From Retina Service, Department of Ophthalmology, Weill Cornell Medical College, New York.

Supported, in part, by an unrestricted department grant from Research to Prevent Blindness.

Dr. Kiss reports the following disclosures: Alcon (consultant, honoraria), Alimera Sciences (consultant, honoraria), Adverum Biotechnologies (advisrory board, consultant, stock options), Allergan (consultant, honoraria), Biomarin Pharmaceutical (consultant, honoraria), Fortress Biotech (consultant, stockholder, honoraria, stock options), Genentech (advisory board, consultant, investigator, grants, honoraria), Novartis (consultant, honoraria), Optos pls (advisory board, consultant, grants honoraria), Regeneron Pharmaceuticals (advisory board, consultant, investigator, grants honoraria), Regenxbio (advisory board, consultant, honoraria, stock options), and Spark Therapeutics (consultant, honoraria). Dr. Orlin is on the advisory board for Zeiss and Allergan. The remaining authors report no relevant financial disclosures.

Address correspondence to Kyle D. Kovacs, MD, Weil Cornell Medicine, Department of Ophthalmology, 1305 York Avenue, 11th Floor, New York, NY 10021; email: kyle.kovacs@gmail.com.

Received: May 07, 2019
Accepted: July 29, 2019

10.3928/23258160-20191211-08

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