Secondary Logo

Journal Logo

MULTIMODAL IMAGING OF ACUTE EXUDATIVE POLYMORPHOUS VITELLIFORM MACULOPATHY WITH OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY AND ADAPTIVE OPTICS SCANNING LASER OPHTHALMOSCOPY

Skondra, Dimitra, MD, PhD*; Nesper, Peter L., BA; Fawzi, Amani A., MD

Retinal Cases and Brief Reports: July 2019 - Volume 13 - Issue 3 - p 195–198
doi: 10.1097/ICB.0000000000000591
Case Report
Free

Purpose: To report a case of acute exudative polymorphous vitelliform maculopathy including the findings of optical coherence tomography angiography and adaptive optics scanning laser ophthalmoscopy.

Methods: Findings on clinical examination, color fundus photography, spectral-domain optical coherence tomography, infrared reflectance, autofluorescence, optical coherence tomography angiography, and adaptive optics scanning laser ophthalmoscopy.

Results: A 54-year-old white man with no significant medical history and history of smoking presented with bilateral multiple serous and vitelliform detachments consistent with acute exudative polymorphous vitelliform maculopathy. Extensive infectious, inflammatory, and malignancy workup was negative. Spectral-domain optical coherence tomography showed thickened, hyperreflective ellipsoid zone, subretinal fluid, and focal as well as diffuse subretinal hyperreflective material corresponding to the vitelliform lesions. Optical coherence tomography angiography showed normal retinal and choroidal vasculature, whereas adaptive optics scanning laser ophthalmoscopy showed circular focal “target” lesions at the level of the photoreceptors in the area of foveal detachment.

Conclusion: Multimodal imaging is valuable in evaluating patients with acute exudative polymorphous vitelliform maculopathy.

Acute exudative polymorphous vitelliform maculopathy (AEPVM) is a rare entity of unknown etiology. The authors present a case of AEPVM including the findings of multimodal imaging with optical coherence tomography angiography and adaptive optics scanning laser ophthalmoscopy in addition to spectral-domain optical coherence tomography and autofluorescence.

*Retina Service, Department of Ophthalmology and Visual Science, The University of Chicago, Chicago, Illinois; and

Retina Service, Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois.

Reprint requests: Amani A. Fawzi, MD, Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, 645 N. Michigan Avenue, Suite 440, Chicago, IL 60611; e-mail: afawzimd@gmail.com

Research instrument support was provided by Boston Micromachines Corporation and Optovue, Inc.

None of the authors has conflicting interests to disclose.

Acute exudative polymorphous vitelliform maculopathy (AEPVM) is a rare entity of unknown etiology. We present a case of AEPVM including the findings of multimodal imaging with optical coherence tomography angiography (OCTA) and adaptive optics scanning laser ophthalmoscopy (AOSLO) in addition to spectral-domain optical coherence tomography (OCT) and autofluorescence.

Back to Top | Article Outline

Case Report

A 54-year-old white man with no significant medical history and history of smoking was referred to the retina clinic for decreased vision. Visual acuity was 20/25 and 20/40 in the right and left eyes, respectively. Anterior segment examination was unremarkable. Ophthalmoscopy revealed bilateral multiple serous and vitelliform detachments (Figure 1, A and B). The spectral-domain OCT (Spectralis HRA + OCT; Heidelberg Engineering, Heidelberg, Germany) showed thickened, hyperreflective ellipsoid zone, subretinal fluid, and focal as well as diffuse subretinal hyperreflective material corresponding to the vitelliform lesions (Figure 1, C and D). Fluorescein angiogram was normal, whereas fundus autofluorescence revealed hyperautofluorescence of the vitelliform detachments and stippled hyperautofluorescence corresponding with the focal hyperreflective subretinal deposits seen in OCT (Figure 1, F and G). Retinal and choroidal vasculature was evaluated with OCTA using the RTVue-XR Avanti OCTA System (Optovue Inc, Fremont, CA). Superficial capillary plexus, deep capillary plexus, outer retina, and choriocapillaris appeared normal with areas of shadowing optical artifacts in the choriocapillaris slab from overlying subretinal vitelliform material and subretinal fluid (Figure 2, A–C). En face structural OCT at the level of the photoreceptors showed signal attenuation consistent with subretinal fluid and focal areas of increased signal corresponding to hyperreflective vitelliform material seen on cross-sectional OCT (Figure 2D). The AOSLO imaging was performed using the Apaeros Retinal Imaging System (Boston Micromachines Corporation, Boston, MA), which achieves a lateral resolution of ∼3 µm. Adaptive optics scanning laser ophthalmoscopy showed a sparse population of parafoveal photoreceptors, which appear as bright dots with retained normal waveguiding properties (Figure 2E) and abnormal circular focal “target” lesions centrally in the area of foveal detachment (Figure 2F).

Fig. 1

Fig. 1

Fig. 2

Fig. 2

Based on clinical picture, age, lack of family history, and findings of spectral-domain OCT, infrared, and other imaging modalities, diagnosis was consistent with AEPVM rather than multifocal Best disease, which was considered in the differential. Acute exudative polymorphous vitelliform maculopathy can manifest as paraneoplastic syndrome with cutaneous melanoma and other malignancies and because our patient was a heavy smoker and had not had a physical examination with primary care for many years because of lack of health insurance, malignancy workup was performed. Dermatology consultation for cutaneous melanoma was negative and whole-body positron emission tomography showed a small lung nodule. Fine needle aspiration biopsy of the nodule was negative for malignant cells and pathologic examination was consistent with focal pneumonitis.

Back to Top | Article Outline

Discussion

Acute exudative polymorphous vitelliform maculopathy is a very rare disorder first described by Gass in 1988.1 This entity is characterized by bilateral multifocal, yellowish vitelliform lesions and serous detachments in the posterior pole. The etiology of this extremely rare disease remains unknown, but an inflammatory cause has been proposed.1 Vascular etiology has also been suggested based on involvement of inner choroid and choriocapillaris seen on indocyanine green angiography.2 Acute exudative polymorphous vitelliform maculopathy can also present as a paraneoplastic syndrome in patients with metastatic melanoma or carcinoma.3 In paraneoplastic AEPVM, autoantibodies against retinal pigment epithelium (RPE) proteins have been reported, suggesting an immune-mediated insult to the RPE.4

Retinal and choroidal vasculature appeared normal in OCTA, supporting RPE dysfunction rather than vascular compromise as a potential etiology. The AOSLO showed circular “target” lesions at the level of the photoreceptors in the area of foveal detachment as highlighted by the arrows in the high-magnification AOSLO images (Figure 2F). It is not clear why these lesions have “target” configuration with surrounding dark halo, but we speculate that this is because AOSLO is focused on photoreceptors overlying shallow subretinal fluid and the scan also includes the top portion of material accumulated on top of RPE as seen on OCT B-scans (Figure 2G). The content of these lesions is unknown but may represent remnants of photoreceptor outer segments that could not be phagocytosed by the RPE and focally accumulate on top of the RPE, further supporting the role of RPE dysfunction in the pathogenesis of AEPVM.

To the best of our knowledge, this is the first report of OCTA and AOSLO imaging in AEPVM. Multimodal imaging is valuable in evaluating patients with this rare entity of AEPVM.

Back to Top | Article Outline

References

1. Gass JDM, Chuang EL, Granek H. Acute exudative polymorphous vitelliform maculopathy. Trans Am Ophthalmol Soc 1988;86:354–366.
2. Vianna RNG, Muralha A, Muralha L. Indocyanine-green angiography in acute idiopathic exudative polymorphous vitelliform maculopathy. Retina 2003;23:538–541.
3. Al-Dahmash SA, Shields CL, Bianciotto CG, et al. Acute exudative paraneoplastic polymorphous vitelliform maculopathy in five cases. Ophthalmic Surg Lasers Imaging 2012;43:366–373.
4. Dalvin LA, Johnson AA, Pulido JS, et al. Non-antinestrophin anti-RPE antibodies in paraneoplastic exudative polymorphous vitelliform maculopathy. Transl Vis Sci Technol 2015;4:2.
Keywords:

acute exudative polymorphous vitelliform maculopathy; adaptive optics scanning laser ophthalmoscopy; optical coherence tomography angiography

© 2019 by Ophthalmic Communications Society, Inc.
<