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Original Investigation | Clinical Sciences

Multimodal Imaging in Persistent Placoid Maculopathy

Mohamed G. Gendy, MD1; Amani A. Fawzi, MD1; Robert T. Wendel, MD2; Dante J. Pieramici, MD3; Joel A. Miller, MD4; Lee M. Jampol, MD1
[+] Author Affiliations
1Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois
2Retina Consultants, Sacramento, California
3California Retina Consultants, Bakersfield
4Retina Consultants of Michigan, Southfield
JAMA Ophthalmol. 2014;132(1):38-49. doi:10.1001/jamaophthalmol.2013.6310.
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Importance  Persistent placoid maculopathy (PPM) is a rare clinical entity with features that superficially resemble acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and macular serpiginous choroidopathy. It is important to differentiate PPM from APMPPE because both conditions may appear similar at presentation.

Objective  To investigate the short-term and long-term retinal changes in patients with PPM using spectral domain optical coherence tomography (SD-OCT), indocyanine green angiography (ICG-A), fluorescein angiography (FA), and fundus autofluorescence (FAF).

Design, Setting, and Participants  We performed a retrospective medical record review in 5 patients diagnosed as having PPM at tertiary retinal practices.

Main Outcomes and Measures  Findings on SD-OCT, FA, digital FAF, and ICG-A images.

Results  Patients presented within 2 weeks of subjective symptoms. Mean best-corrected visual acuity was 20/144 (range, 20/25-20/400). At presentation, all but 1 patient had bilateral macular lesions. Four eyes developed extramacular lesions during follow-up. On SD-OCT, the acute placoid lesions revealed hyperreflectivity of the outer nuclear layer; disruption of the external limiting membrane, ellipsoid layer, and interdigitation zone; and, in some patients, hyporeflective spaces at the level of absent outer segments. On follow-up, lesions revealed either partial or complete restoration of the outer retinal architecture or they progressed to atrophy. On FA, all placoid lesions were hypofluorescent in early frames and hyperfluorescent in late frames. In the acute stage, ICG-A revealed sharply delineated dense hypofluorescent lesions, which persisted on late frames in all patients. Hypofluorescent lesions faded completely or partially after resolution of the placoid lesions on SD-OCT and clinical examination. Variability was seen on the FAF patterns; most lesions were hyperautofluorescent, except in 1 patient, in whom they were hypoautofluorescent. Bilateral choroidal neovascularization developed in only 1 patient. The mean follow-up was 28 weeks (range, 2-92 weeks). On the final follow-up visit, mean best-corrected visual acuity was 20/125 (range, 20/25-20/400).

Conclusions and Relevance  On SD-OCT, acute retinal changes in PPM involve the outer nuclear layer, external limiting membrane, ellipsoid layer, and interdigitation zone. The retinal pigment epithelium and choroid are involved in severely affected patients. The variable extent of retinal pigment epithelium involvement was reflected in variable FAF findings. We discuss clinical features that differentiate this entity from other white spots, including acute placoid multifocal pigment epitheliopathy. Additional long-term imaging studies are needed to further clarify the exact location and pathogenesis of this rare disease.

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Figures

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Figure 1.
Evolution of the Placoid Lesions Into Atrophy in Patient 1

A, Fundus view of the right eye shows a large macular placoid lesion with beginning pigmentary and atrophic changes. B, Fundus view of the left eye shows a smaller placoid macular lesion. C and D, Three weeks later, macular lesions expanded with some atrophy and pigmentation.

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Figure 2.
Fundus Autofluorescence (FAF) and Indocyanine Green Angiography (ICG-A) Patterns in the Healing Phase in Patient 1

A and B, A mottled FAF pattern was seen as the macular lesion started to heal. C and D, ICG-A reveals dense hypofluorescent macular lesions with linear intervening hyperfluorescence (choroidal vessels) and hypofluorescent satellite lesions.

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Figure 3.
Severe Rapid Progressive Bilateral Outer Retinal Atrophy in Patient 1

A, A spectral domain optical coherence tomography (SD-OCT) image of the right eye shows severely thinned fovea, collapsed outer nuclear layer (ONL), and absence of the external limiting membrane (ELM) and ellipsoid layers with thickened and disrupted (RPE) (black arrowheads). B, An SD-OCT image of the left eye shows parafoveal hyperreflective bands within the ONL and disruption of the ELM and ellipsoid layers with an area of hyporeflectivity. C, Three weeks later, the right eye shows progressive thinning and outer retinal atrophy. D, Left eye scan shows thinning of the ONL with expansion of the ELM and ellipsoid layer disruption with progressive parafoveal RPE thickening (black arrowheads) and atrophy. E and F, Ten weeks later, both eyes show severely thinned fovea and extensive outer retinal atrophy. F, Nasal parafoveal RPE and ellipsoid layer of the left eye shows evidence of some recovery (white arrowheads).

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Figure 4.
Choroidal Involvement in Patient 1

A, A Cirrus SD-OCT C-scan positioned at the ONL shows circular hyperreflectance at the lesion periphery (black arrowhead) and a hyporeflective center (white arrowhead). B, A C-scan positioned at the choroid shows hyporeflectance (black arrowhead) with a central hyperreflective area (white arrowhead). C, Ten weeks later, when the ONL lesion resolved, a C-scan positioned at the choroid shows significant resolution of choroidal darkness with hyperreflective areas (black arrowheads).

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Figure 5.
Fundus Autofluorescence (FAF) Changes as Disease Progressed Into Atrophy in Patient 2

A, Fundus view of the right eye shows large macular annular placoid lesion encircling the fovea. B, An FAF image shows hyperautofluorescence with hypoautofluorescent margins. Early frames of fundus angiography show a hypofluorescent macular lesion (C), with increasing hyperfluorescence in late frames (D). Four months after treatment, a fundus view shows resolution of the placoid lesion with atrophic and pigmentary changes (E), whereas on FAF, lesions decreased in size and became hypoautofluorescent (F).

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Figure 6.
Significant Restoration of the Outer Retina and Retinal Pigment Epithelium (RPE) After Treatment in Patient 2

A, A spectral domain optical coherence tomography (SD-OCT) image of the right eye reveals a thinned outer nuclear layer (ONL) with a hyperreflective band associated with loss of the external limiting membrane (ELM) and ellipsoid layers (black arrowhead). The RPE shows disruption with overlying hyperreflective focal deposits (white arrowheads). The central foveal subfield with preserved ELM and ellipsoid layers is also seen. B, Five weeks after treatment with oral prednisone, the right eye has progressive thinning of the ONL with resolution of the hyperreflective lesions (black arrowhead). Resolution of focal RPE deposits with some restoration of the integrity of ellipsoid layer is also seen (arrowhead). C, Four months after treatment with mycophenolate mofetil, significant restoration of the ELM and ellipsoid layers and improved integrity of the RPE layer (black arrowheads) with areas of persistent ONL (white arrowhead) thinning seen.

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Figure 7.
Bilateral Development of Satellite Lesions in Patient 3

This patient was treated with acyclovir and prednisone. A, A fundus angiography (FA) image of the right eye was unremarkable. B, Early frames of FA in the left eyes show a hypofluorescent macular lesion with a few satellite hypofluorescent lesions. Four weeks later, early frames of FA of the right eye show a small hypofluoresent macular lesion (C), whereas the left eye shows enlarged hypofluorescent lesion with development of new lesions superior to the optic nerve (D). E, Three weeks later, FA shows an enlarged hypofluorescent lesion and 2 new hypofluorescent satellite lesions (arrows). F, An FA image of the left eye shows enlargement of the macular lesion with blockage from pigmentary changes and enlargement of the hypofluorescent lesions located superior to the optic nerve.

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Figure 8.
Outer Retinal and Choroidal Changes in Patient 3

A, A spectral domain optical coherence tomography (SD-OCT) image of the right eye shows normal architecture. B, An SD-OCT image of the left eye shows disruption of the external limiting membrane (ELM) and ellipsoid layers with a large hyporeflective space and aggregations of hyperreflective material. Two weeks later, a right eye SD-OCT image was normal (C), whereas the left eye shows progressive disruption of the ELM and ellipsoid layers with expansion of the outer retinal hyporeflective space nasal to the fovea (black arrowheads) (D). E, Four weeks later, an SD-OCT image of the right eye shows outer nuclear layer hyperreflectivity with disrupted ELM and ellipsoid layers and a small subretinal hyporeflective space at the level of the outer segments. F, The left eye shows restoration of the ELM (arrow) with partial resolution of the hyporeflective zone (arrowhead). D and E, Replacement of normal choroidal vascular reflectivity by homogeneous isoreflective pattern (white arrowheads) was observed in active lesions. This pattern of superficial choroidal involvement can be compared with normal pattern in these areas before involvement (C) and after healing (F).

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Figure 9.
Evolution of the Placoid Lesions Into Atrophy in Patient 3

A and B, Fundus views show resolution of the placoid lesions with atrophic and pigmentary macular changes.

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Figure 10.
Bilateral Choroidal Neovascularization (CNV) at Presentation in Patient 4

At presentation, early fluorescein angiograph frames of the right eye show an early hypofluorescent lesion (A), which exhibits late hyperfluorescence with leakage from the CNV (B). C, The left eye shows hypofluorescent macular lesion with focal areas of leakage from CNV (black arrowheads).

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Figure 11.
Spectral Domain Optical Coherence Tomography (SD-OCT) Findings at Presentation and After Treatment in Patient 4

A, At presentation, an SD-OCT image of the right eye shows type 1 choroidal neovascularization (black arrowhead) with pigment epithelium elevation, subretinal fluid (SRF), and disruption of the external limiting membrane (ELM) and ellipsoid layers. B, An SD-OCT image of the left eye shows an outer nuclear layer (ONL) hyperreflective band and focal disruption of the ellipsoid layer (white arrowhead). C, After bevacizumab injection, photodynamic therapy, and intravitreal injection of dexamethasone, decreased SRF (black arrowhead), persistent type 1 component (white arrowhead), and more pigment epithelium elevation are seen. D, The left eye shows more disruption of the ELM and ellipsoid layers and accumulation of intraretinal fluid (IRF). At the last visit after multiple treatments for 18 months, the right eye shows extensive subretinal scarring and residual intraretinal cysts (E), whereas the left eye shows resolution of the IRF with nearly complete restoration of the ELM and ellipsoid layers (F).

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Figure 12.
Fluorescein Angiography (FA), Indocyanine Green Angiography (ICG-A), and Fundus Autofluorescence (FAF) After Treatment in Patient 4

A, Three weeks after presentation, an FA image of the left eye shows hypofluorescence centrally with enlargement of the area of leakage from choroidal neovascularization. B, An ICG-A image shows dense hypofluorescent macular lesions. C, On FAF, lesions were hypoautofluorescent.

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Figure 13.
Acute Spectral Domain Optical Coherence Tomography (SD-OCT) Findings in All Cases

A, The SD-OCT images of acute placoid lesions from patient 1 (A), patient 2 (B), patient 3 (C), patient 4 (D), and patient 5 (E) show hyperreflectivity within the outer nuclear layer and disruption of the external limiting membrane and ellipsoid layers, appearing as a hyporeflective space at the level of the outer segments.

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