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Heliyon
Heliyon
Heliyon
2405-8440
Elsevier

S2405-8440(24)12277-3
10.1016/j.heliyon.2024.e36246
e36246
Case Report
Neurosensory retina detachment combined with retinal pigment epithelium separation from the choroid in the course of dry macular degeneration – A case report
Gawęcki Maciej a
Grzybowski Andrzej ae.grzybowski@gmail.com
bcd⁎
a Dobry Wzrok Ophthalmological Clinic, Zabi Kruk 10, 80-402, Gdańsk, Poland
b Department of Ophthalmology, University of Warmia and Mazury, Oczapowskiego 2, 10-719, Olsztyn, Poland
c Institute for Research in Ophthalmology, 60-554, Poznan, Poland
d Institute for Research in Ophthalmology, Foundation for Ophthalmology Development, Mickiewicza 24, 61-836, Poznan, Poland
⁎ Corresponding author. Department of Ophthalmology, University of Warmia and Mazury, Oczapowskiego 2, 10-719 Olsztyn, Poland. ae.grzybowski@gmail.com
20 8 2024
30 8 2024
20 8 2024
10 16 e3624624 5 2024
9 8 2024
13 8 2024
© 2024 The Authors. Published by Elsevier Ltd.
2024

https://creativecommons.org/licenses/by-nc/4.0/ This is an open access article under the CC BY-NC license (http://creativecommons.org/licenses/by-nc/4.0/).
Introduction

of advanced diagnostic methods shed the light on the variable course of age-related macular degeneration (AMD). Despite establishing AMD classifications used in clinical practice, there are still forms of AMD that do not fit into these systems. The case report presents a rare evolution of non-neovascular form of AMD presenting at baseline as large soft drusen. Within the 5 years of observation one eye with such form of AMD transformed to retinal pigment epithelial detachment and subsequently simultaneous separation of the neurosensory retina and the choroid from the RPE. As a result, on the spectral domain optical coherence tomography scan, the case presented with lone line of the RPE neighbored by subretinal fluid from the inner side and choroidal excavation from the outside. Macular neovascularization was excluded at each timepoint of the follow-up. During 2.5 years of observation post the onset of RPE separation, the case remained stable with maintained visual acuity at 0.25 Snellen and lack of progression to wet form of AMD. Further observation is needed to fully assess the eye's potential for visual preservation in the long term.

Highlights

Q6: Please check the address for the corresponding author that has been added here, and correct if necessary.• Separation of the RPE from the neurosensory retina and the choroid is a rare form of non-neovascular AMD.

• This clinical entity has rather stationary course in a few years' observation time.

• SD-OCT is essential for disease recognition and follow-up.

Keywords

Age related macular degeneration
Focal choroidal excavation
Retinal pigment epithelium detachment
Macular neovascularization
Case report
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pmc1 Introduction

Age related macular degeneration (AMD) is a disease with variety of phenotypes. Despite different classifications of that entity, used in clinical practice, there are forms of AMD that can hardly be placed in any of the subgroups. Development of modern diagnostic technologies, especially spectral domain optical coherence tomography (SD-OCT) and OCT angiography (OCTA) added more to the morphological picture of that disorder and enabled to find new scenarios of the disease course. We present a case of evolution of dry form of AMD from typical drusen to neurosensory retina (NSR) detachment combined with choroidal excavation.

2 Case presentation

Female patient aged 85 without systemic comorbidities remained under care of Dobry Wzrok Ophthalmological Clinic for the last 5 years. At presentation in 2019 she was diagnosed with intermediate stage of dry form of AMD. Both eyes presented with large and intermediate confluent drusen with minor pigmentary abnormalities (Fig. 1 A – D).). Spectral domain optical coherence tomography scans of both eyes are shown at Fig. 2.Fig. 1 A–D. Color fundus photographs and fundus autofluorescence at baseline. Both hard and soft drusen as well as pigment abnormalities are detected at the fundus of both eyes. FAF reveals focal areas of RPE loss.

Fig. 1

Fig. 2 SD-OCT scans of both eyes at baseline. The scans show areas of increased reflectance under the RPE corresponding to large and intermediate soft drusen. There is no sign of exudation in neurosensory retina.

Fig. 2

Best corrected visual acuity (BCVA) in both eyes was 0.5 on the Snellen chart. The patient was advised for intake of supplements according to AREDS 2 formula, self-control with Amsler grid and SD-OCT examinations twice a year. For the next two years the AMD remained a dry type with large and intermediate drusen and stable BCVA. At the point of two years post presentation the right eye (RE) developed serous pigment epithelial detachment (PED) (Fig. 3.). Best corrected visual acuity in RE dropped to 0.25, while left eye (LE) maintained visual acuity at 0.5.Fig. 3 Pigment epithelial detachment in the right eye. The space under RPE remains hyporeflective what indicates the resorption of drusenoid material.

Fig. 3

Fluorescein angiography (FA) did not reveal any leakage related changes (cysts or fluid) in neurosensory retina (NSR) (Fig. 4 A and B). The only abnormality was hyperreflective dots above the RPE related to pigment. The presence of macular neovascularization (MNV) was not observed on angio-OCT (OCTA) and indocyanine green angiography (ICGA) (Fig. 5, Fig. 6 A – D). The patient was scheduled for the next visit in three months. At that point SD-OCT examination showed separation of the NSR from the RPE with subretinal fluid and simultaneous separation of the RPE from the choroid, that appeared concave in shape. (Fig. 7).Fig. 4 A and B. Macular neovascularization is excluded in fluorescein angiography (FA), OCT-angiography (OCTA) and indocyanine green angiography (ICGA). No leakage is noted on early and late phase of the FA, but staining related to the presence of drusen and pooling corresponding to subretinal fluid. There are no pathological vessels detected at the level of choriocapillaris on OCTA. ICGA (intermediate and late phases) presents without the presence of the hyperfluorescent “plaque” typical for recognition of MNV.

Fig. 4

Fig. 5 A–B. Macular neovascularization is excluded in fluorescein angiography (FA), OCT-angiography (OCTA) and indocyanine green angiography (ICGA). No leakage is noted on early and late phase of the FA, but staining related to the presence of drusen and pooling corresponding to subretinal fluid. There are no pathological vessels detected at the level of choriocapillaris on OCTA. ICGA (intermediate and late phases) presents without the presence of the hyperfluorescent “plaque” typical for recognition of MNV.

Fig. 5

Fig. 6 A–B. Macular neovascularization is excluded in fluorescein angiography (FA), OCT-angiography (OCTA) and indocyanine green angiography (ICGA). No leakage is noted on early and late phase of the FA, but staining related to the presence of drusen and pooling corresponding to subretinal fluid. There are no pathological vessels detected at the level of choriocapillaris on OCTA. ICGA (intermediate and late phases) presents without the presence of the hyperfluorescent “plaque” typical for recognition of MNV.

Fig. 6

Fig. 7 Simultaneous separation of neurosensory retina and the choroid from the RPE at SD-OCT scan. Elongation of photoreceptors is noted as well as clarity of the subretinal and sub-RPE fluid.

Fig. 7

Despite lack of any definite signs of MNV on fluorescein angiography, OCTA and ICGA, however with strong suspicion of presence of MNV, the series of three intravitreal injections of aflibercept with monthly intervals were performed, however without any morphological or functional effect. Visual acuity in the RE remained at the same level of 0.25 Snellen. In the view of that data, the anti-VEGF treatment was abandoned and patient was closely observed with control visits every 3 months. Since then and up to 2024 no progression of AMD towards the wet type was noted in any of the patient's eyes. Retinal morphology of the RE remains stable with NSR detachment, choroidal depression and RPE standing out as a lone line in the central macular area. Best corrected visual acuity remains at the same level of 0.25 Snellen. The follow-up of the RE on the SD-OCT is presented at Fig. 8.Fig. 8 The progression of SD-OCT changes of the right eyes during analyzed 5 years. Stability of the retinal morphology is noted since separation of the RPE.

Fig. 8

The latest color fundus photograph and fundus autofluorescence (FAF) are presented at Fig. 9 (A – D). Besides drusen, there are pigmentary changes and plaques of depigmentation noted in both eyes. Fundus autofluorescence reveals small areas of geographic atrophy of the RPE outside the foveal center. Significant progression towards confluent drusen is noted in the right eye. Focal areas of RPE loss are larger compared to baseline photographs.Fig. 9 Current color fundus photograph and fundus autofluorescence of patient's right and left eyes. Significant progression towards confluent drusen is noted in the right eye. Perifoveal loss of the RPE is noted in both eyes, noticably larger in comparison with baseline examination.

Fig. 9

Patient is followed in the clinic every 3–4 months with regularly performed SD-OCT, OCTA, FAF and color fundus photographs.

3 Discussion

Basic classification of AMD used in clinical practice indicates early and advanced forms (drusen, pigmentary changes, MNV or GA) and determines the stage of dry AMD according to drusen size and presence of pigmentary abnormalities [1].

However, some specific forms of AMD may not be classifiable and we believe that our report represents such case. One of the important issues in our patient is the assessment of the potential presence of MNV. All the diagnostic tests excluded the presence of MNV. Also, the presentation of the fundus of the eyes did not indicate such complication – there were no intra- or subretinal hemorrhages present. For a short moment the AMD took the form of serous PED, apparently after resorption of drusenoid material. Such scenario is not atypical and frequently precedes the progression to advanced forms of AMD: geographic atrophy of the RPE or MNV [2,3]. Pigment epithelial detachment may become vascularized from the choroid and disease may progress to fully developed MNV or, alternatively, absorption of drusenoid material may be followed by RPE loss. In our case, PED was followed by simultaneous separation of both: NSR and the choroid. Morphologically such AMD type looks awkward on SD-OCT, however it is plausible, that large separation of the choroid together with its concave shape (focal choroidal excavation – FCE) prevents the progression of neo-vessels under the NSR. This concept remains in consent with reports of other authors, who report presence of MNV in choroidal excavation rather rarely [4,5]. On the other hand, RPE becomes devoid of nutrition from the choroid, what in the long term might cause its deterioration and subsequent visual loss. In our case, however, during the period of 2.5 years BCVA remained stable at the same level. Further morphological destruction of retinal architecture was not noted either.

Interesting aspect of our case is the etiology of the occurrence of the space between the RPE and the choroid. Such entity resembles non-conforming focal choroidal excavation (FCE) located in the spectrum of pachychoroid disorders or accompanying AMD [6,7]. Choroidal excavation accompanying AMD is usually bowl-shaped as described by Shinojima et al. what is represented by our case as well [8]. Typically, however in choroidal excavation the RPE stays attached to the choroid within the choroidal cavity with just NSR detached. In our case, prominent RPE detachment from the choroid is noted. To our knowledge, it is the first AMD case described with such atypical alteration of both: retinal and choroidal morphology with relatively intact RPE layer. Further observation is needed to fully describe the course of that entity with special attention put into prevention of neovascular complications.

4 Conclusion

Simultaneous separation of the RPE from the neurosensory retina and the choroid sustains a rare form of non-neovascular AMD. A few years’ follow-up proves its stationary character, however precise disease characteristics requires larger sample and longer observation time.

PATIENT’S PERSPECTIVE: The patient appreciated the close follow-up that she received during five years of observation. Despite lack of the effective treatment the patient is happy with maintaining stable visual acuity.

Ethics statement

All procedures performed in this study were in accordance with the ethical standards of the institutional research committee (approval number: 4/2024, dated 02.04.2024) and with compliance to the principles of 1964 Helsinki declaration.

Written consent statement

The patient provided a written consent for the procedures performed during treatment of her case. She also consents for publication of the images and details from the course of the disease.

Data availability statement

All additional data will be made available from the corresponding author upon request.

CRediT authorship contribution statement

Maciej Gawęcki: Writing – review & editing, Writing – original draft, Visualization, Validation, Supervision, Software, Resources, Project administration, Methodology, Investigation, Funding acquisition, Formal analysis, Data curation, Conceptualization. Andrzej Grzybowski: Writing – review & editing, Supervision, Methodology.

Declaration of competing interest

The authors declare the following financial interests/personal relationships which may be considered as potential competing interests:Andrzej Grzybowski is noted as an AE/ABM/SE of Heliyon Cell Press Journal. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
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