Ophthalmic Surgery, Lasers and Imaging Retina

Imaging: Case Report 

High-Definition OCT Findings in Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Giuseppe Querques, MD, PhD; Lea Querques, MD; Anna V. Bux, MD; Cristiana Iaculli, MD; Nicola Delle Noci, MD

Abstract

The authors report a case of acute posterior multifocal placoid pigment epitheliopathy evaluated by autofluorescence, fluorescein angiography, indocyanine green angiography, high-definition optical coherence tomography, and microperimetry in both the acute and the post-acute phase. Based on the integrated findings, the authors believe that acute posterior multifocal placoid pigment epitheliopathy is an inflammatory disease that primarily affects the choroid and that the retinal pigment epithelium is secondarily involved in the course of the disease.

From the Department of Ophthalmology (GQ, AVB, CI, NDN), Ospedali Riuniti, University of Foggia, Foggia; and the University Hospital San Raffaele (LQ), Milan, Italy.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Giuseppe Querques, MD, PhD, Policlinico Riuniti di Foggia, University of Foggia, Viale Pinto, 1, 71100, Foggia, Italy.

Accepted: September 24, 2008
Posted Online: February 21, 2013

Abstract

The authors report a case of acute posterior multifocal placoid pigment epitheliopathy evaluated by autofluorescence, fluorescein angiography, indocyanine green angiography, high-definition optical coherence tomography, and microperimetry in both the acute and the post-acute phase. Based on the integrated findings, the authors believe that acute posterior multifocal placoid pigment epitheliopathy is an inflammatory disease that primarily affects the choroid and that the retinal pigment epithelium is secondarily involved in the course of the disease.

From the Department of Ophthalmology (GQ, AVB, CI, NDN), Ospedali Riuniti, University of Foggia, Foggia; and the University Hospital San Raffaele (LQ), Milan, Italy.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Giuseppe Querques, MD, PhD, Policlinico Riuniti di Foggia, University of Foggia, Viale Pinto, 1, 71100, Foggia, Italy.

Accepted: September 24, 2008
Posted Online: February 21, 2013

Introduction

Acute posterior multifocal placoid pigment epitheliopathy is an inflammatory retinal disease that typically affects young healthy patients and usually occurs shortly after a flu-like syndrome. Symptoms include metamorphopsia, scotomas, and rapid loss of visual acuity. Given that no histopathologic studies have been reported, the presumptive location of the lesions has been based on clinical examination and it is currently unclear whether the disease is primarily a retinal pigment epithelium (RPE) abnormality or is caused by a disruption of the choroidal vasculature. 1–5

High-definition spectral-domain optical coherence tomography (HD-OCT) (OCT 4000 Cirrus; Humphrey-Zeiss, San Leandro, CA) is a high-speed OCT system (up to 27,000 axial scans per second) using spectral or Fourier domain detection, with an axial image resolution of 5 μm. Hence, in vivo visualization of intraretinal structures, and especially the RPE and the inner segment and outer segment of the photoreceptor layer, is possible.

We report a case of acute posterior multifocal placoid pigment epitheliopathy evaluated by autofluorescence, fluorescein angiography, indocyanine green angiography (ICGA), HD-OCT, and microperimetry in both the acute phase and the post-acute phase (after treatment with systemic corticosteroids).

Case Report

A 23-year-old man was referred to our department with the complaints of metamorphopsia and decreased vision for 1 week. The patient had also suffered from frontal headaches and a flu-like syndrome since the onset of his visual disturbance. He signed a comprehensive consent form according to Good Clinical Practice guidelines before proceeding with any examinations or treatments.

The visual acuity was 20/25 in both eyes. Intraocular pressure was 14 mm Hg in both eyes and anterior segments were normal, with no cells in the anterior chamber. Vitreous cells were present bilaterally. Fundus examination revealed multifocal creamy yellowish-white plaques in the posterior pole bilaterally (Figs. 1A and 1B ). Autofluorescence showed areas of hypoautofluorescence corresponding to the ophthalmoscopically seen creamy yellowish-white plaques, generally centered by hyperautofluorescent spots and surrounded by hyperautofluorescent halos (Figs. 1C and 1D ).

Figure 1. Color fundus photography, autofluorescence (AF), fluorescein angiography (FA), and indocyanine green angiography (ICGA) at the time of onset of visual symptoms. Color fundus photographs show multifocal creamy yellowish-white plaques in the posterior pole of both the right (A) and left (B) eye. AF frames show areas of hypoautofluorescence corresponding to the ophthalmoscopically seen creamy yellowish-white plaques (C and D); these areas are generally centered by hyperautofluorescent spots and surrounded by hyperautofluorescent halos in both the right (C) and left (D) eye. FA shows areas of decreased fluorescence early in the filling phase of the right (E) and left (F) eye, many more than the number of lesions seen on fundus examination and AF. Only a few of these hypofluorescent areas stain in the late phase (G and H). ICGA shows areas of early hypocyanescence in both the right (I) and left (J) eye, many more than the number of lesions seen on fundus examination, AF, and FA, and only a few of these stain in the late phase (K and L).

Fluorescein angiography showed areas of decreased fluorescence early in the filling phase (Figs. 1E and 1F ), many more than the number of lesions seen on fundus examination and autofluorescence, and only a few of these stained in the late phase (Figs. 1G and 1H ). ICGA showed areas of early hypocyanescence (Figs. 1I and 1J ), many more than the number of lesions seen on fundus examination, autofluorescence, and even fluorescein angiography, and only a few of these stained in the late phase (Figs. 1K and 1L ).

HD-OCT revealed areas of hyperreflectivity corresponding to the ophthalmoscopically seen creamy yellowish-white plaques, involving the outer retinal layers from the outer plexiform layer up to the RPE. At the level of these hyperreflective areas, HD-OCT showed a slight thickening of the neuroepithelium despite an apparent sparing of the inner retinal layers (Figs. 2A–2F ). Moreover, small RPE detachments at the level of the yellowish-white plaques were detected on HD-OCT scans (Figs. 2C–2E ). Interestingly, several vitreous hyper-reflective dots, probably corresponding to the ophthalmoscopically seen vitreous cells, were visualized by HD-OCT (Figs. 2A–2F ). Fundus-related perimetry (MP-1 Micro Perimeter; Nidek Technologies, Padova, Italy), using pattern macula 8° 0dB with threshold strategy 4–2, revealed absolute and relative scotomas within the macular area at the level of the yellowish-white plaques and a central and stable fixation (Figs. 2G and 2H ).

Figure 2. High-definition optical coherence tomography (HD-OCT) and fundus-related perimetry at the time of onset of visual symptoms. HD-OCT scans of the right (A, C, and E) and left (B, D, and F) eye show areas of hyperreflectivity (white asterisks) corresponding to the ophthalmoscopically seen creamy yellowish-white plaques, involving the outer retinal layers, from the outer plexiform layer up to the retinal pigment epithelium (RPE), with slight thickening of the neuroepithelium, despite apparent sparing of the inner retinal layers (A–F). Small RPE detachments at the level of the yellowish-white plaques can be detected by HD-OCT (C–E, arrowheads). Several vitreous hyperreflective dots are visualized on HD-OCT scans (A–F). Fundus-related perimetry of both the right (G) and left (H) eye, using pattern macula 8° 0dB with threshold strategy 4–2, show absolute and relative scotomas within the macular area, at the level of the yellowish-white plaques, and a central and stable fixation.

Based on these findings, the patient was diagnosed as having acute posterior multifocal placoid pigment epitheliopathy. We decided to administer oral prednisone 50 mg daily for 2 weeks. The patient returned with resolution of visual symptoms, frontal headaches, and other flu-like symptoms. Visual acuity had improved to 20/20 in both eyes. Intraocular pressure and anterior segment examination were unchanged. Residual trace vitreous cells were still present bilaterally. Fundus examination revealed the multifocal creamy yellowish-white plaques that turned to well-defined grayish-white lesions surrounded by pigmented halos (Figs. 3A and 3B ). On fundus autofluorescence frames, the ophthalmoscopically seen grayish-white lesions appeared hyperautofluorescent, generally surrounded by hypoautofluorescent halos (Figs. 3C and 3D ).

Figure 3. Color fundus photography, autofluorescence (AF), fluorescein angiography (FA), and indocyanine green angiography (ICGA) 2 weeks later, once the acute lesions subsided. Color fundus photographs show well-defined grayish-white lesions surrounded by pigmented halos in the posterior pole of both the right (A) and left (B) eye. AF frames show the ophthalmoscopically seen grayish-white lesions as hyperautofluorescent, surrounded by hypoautofluorescent halos in both the right (C) and left (D) eye. Late FA (E = right eye and F = left eye) and ICGA (G = right eye and H = left eye) frames show hypofluorescent lesions surrounded by hyperfluorescent halos.

On early and late fluorescein angiography (Figs. 3E and 3F ) and ICGA (Figs. 3G and 3H ) frames, the lesions appeared hypofluorescent, generally surrounded by hyperfluorescent halos. HD-OCT showed normal thickness of the neuroepithelium, total resolution of previous areas of hyperreflectivity (Figs. 4A–4D ), and an irregular RPE band increase in choroidal backscattering. Interestingly, the layer corresponding to the junction between the inner segment and outer segment of the photoreceptors appeared disrupted or absent at the level of the grayish-white lesions. Small RPE detachments and a few vitreous hyperreflective dots were still evident on HD-OCT scans (Figs. 4A–4D ). Fundus-related perimetry, using pattern macula 8° 0dB with threshold strategy 4–2, revealed reduced absolute and relative scotomas within the macular area at the level of the lesions and a central and stable fixation (Figs. 4E and 4F ).

Figure 4. High-definition optical coherence tomography (HD-OCT) and fundus-related perimetry 2 weeks later, once the acute lesions subsided. HD-OCT scans of both the right (A and C) and left (B and D) eye show normal thickness of the neuroepithelium, total resolution of previous areas of hyperreflectivity, with an irregular retinal pigment epithelium (RPE) band increase in choroidal backscattering. The layer corresponding to the junction between the inner and outer segments of the photoreceptors appear disrupted or absent (white asterisks) at the level of the grayish-white lesions. Small RPE detachments (open arrows) and a few vitreous hyperreflective dots are still evident on HD-OCT scans (A–D). Fundus-related perimetry of both the right (E) and left (F) eye, using pattern macula 8° 0dB with threshold strategy 4–2, reveal reduced (but still present) absolute and relative scotomas within the macular area, at the level of the lesions, and a central and stable fixation.

Discussion

Acute posterior multifocal placoid pigment epitheliopathy is a disease of yet unclear etiology that is usually diagnosed by ophthalmoscopy and fluorescein angiography. Recently introduced higher-definition diagnostic tools may be useful in understanding the pathophysiology of the disease. Our case had acute posterior multifocal placoid pigment epitheliopathy soon after the development of the multifocal creamy yellowish-white plaques and later, when the lesions subsided. Our patient underwent a complete ophthalmologic examination, including autofluorescence, fluorescein angiography, ICGA, HD-OCT, and microperimetry, in both the acute and the post-acute phase. In the acute phase, autofluorescence showed areas of hypoautofluorescence, suggesting absence of functional RPE cells centered by hyperautofluorescent spots, surrounded by hyperautofluorescent halos, suggesting dysfunction or metabolic change of RPE cells. Fluorescein angiography revealed numerous hypofluorescent lesions, many of which were not visible on fundus biomicroscopy. On ICGA, the lesions were even more numerous than those observed on fluorescein angiography frames.

According to Spaide et al., 6 these angiographic changes seem to suggest that the RPE is affected secondarily to the choroidal changes (choriocapillaris perfusion defects). Given that HD-OCT revealed areas of hyperreflectivity involving the outer retinal layers, just at the level of the ophthalmoscopically seen creamy yellowish-white plaques (which were far less numerous than the hypofluorescent and hypocyanescent lesions), we believe the angiographic changes would be only partially related to a masking effect of the cellular swelling of the outer retinal layers. 7 This supports the hypothesis of primary choriocapillaris perfusion defects responsible for the RPE changes.

In addition, in this patient, we described for the first time in acute posterior multifocal placoid pigment epitheliopathy the occurrence of small RPE detachments as detected by HD-OCT. Such a finding is further in favor of a primary choroidal dysfunction in the pathophysiology of acute posterior multifocal placoid pigment epitheliopathy. On the other hand, we detected by HD-OCT several vitreous hyperreflective dots, probably corresponding to the ophthalmoscopically seen vitreous cells and, in contrast to the results Souka et al. 7 reported, a slight thickening of the neuroepithelium, suggesting a massive inflammatory reaction in the pathogenesis of acute posterior multifocal placoid pigment epitheliopathy. In the post-acute phase, autofluorescence showed hyperautofluorescent lesions, surrounded by hypoautofluorescent halos, which matched areas of hypofluorescent and hypocyanescent lesions, surrounded by hyperfluorescent and hypercyanescent halos, on fluorescein angiography and ICGA, respectively.

These findings suggesting thickening or duplication of the RPE, associated with the development of atrophic lesions, were confirmed by OCT. HD-OCT scans showed an irregular RPE band associated with an increase in choroidal backscattering and disruption or complete loss of the layer corresponding to the inner segment/outer segment junction of the photoreceptors at the level of the subsided lesions. Atrophic changes after healing of acute lesions have been reported by Scheufele et al. 8 using ultrahigh-resolution OCT. We were able to confirm their findings in our patient, not only anatomically, showing the atrophic lesions by autofluorescence, fluorescein angiography, ICGA, and HD-OCT, but even functionally by revealing the persistence of absolute and relative scotomas. These data are in contrast with results reported by Souka et al., 7 who demonstrated an almost complete recovery on OCT and microperimetry once the acute lesions subsided.

Based on our integrated findings using different new-generation diagnostic tools, we speculate that acute posterior multifocal placoid pigment epitheliopathy is an inflammatory disease that primarily affects the choroid and that the RPE is secondarily involved in the course of the disease. This is a simple report and it is difficult to prove a hypothesis based on the findings in a single patient. Therefore, our observation needs to be confirmed through further studies.

References

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10.3928/15428877-20130213-01

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