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Figure 1.
Fundus photographs, fluorescein angiography, and indocyanine green angiography. A, Fundus appearance at the initial visit showing yellowish placoid lesions over the posterior pole bilaterally. B, From left, hypofluorescence early and staining late on fluorescein angiography, and hypofluorescence early and late on indocyanine green angiography. C, Fundus appearance 2 months after the initial visit showing resolution of placoid lesions but with diffuse retinal pigment epithelial changes left behind.

Fundus photographs, fluorescein angiography, and indocyanine green angiography. A, Fundus appearance at the initial visit showing yellowish placoid lesions over the posterior pole bilaterally. B, From left, hypofluorescence early and staining late on fluorescein angiography, and hypofluorescence early and late on indocyanine green angiography. C, Fundus appearance 2 months after the initial visit showing resolution of placoid lesions but with diffuse retinal pigment epithelial changes left behind.

Figure 2.
Spectral-domain optical coherence tomography. A, Spectral-domain optical coherence tomographic imaging through the fovea of the right eye during the acute phase, with corresponding 3-dimensional reconstruction correlating areas of cream-colored lesions. T indicates temporal; N, nasal. B, Spectral-domain optical coherence tomographic imaging showing relatively well-preserved retinal pigment epithelium (RPE), hyperreflectance throughout the outer nuclear layer corresponding to placoid lesions, and a disrupted inner and outer segment (IS/OS) junction (arrow). Note the normal architecture of intervening areas, with a clearly identifiable IS/OS junction, Verhoeff membrane (VM), and RPE. ELM indicates external limiting membrane. C, Spectral-domain optical coherence tomographic imaging 2 months after the initial visit showing resolution of the hyperreflective lesions but with thinning of the corresponding areas in the outer nuclear layer left behind. The hyperreflective bands representing the IS/OS junction and VM were also disrupted in the intervening area (asterisk), which appeared normal during the acute phase.

Spectral-domain optical coherence tomography. A, Spectral-domain optical coherence tomographic imaging through the fovea of the right eye during the acute phase, with corresponding 3-dimensional reconstruction correlating areas of cream-colored lesions. T indicates temporal; N, nasal. B, Spectral-domain optical coherence tomographic imaging showing relatively well-preserved retinal pigment epithelium (RPE), hyperreflectance throughout the outer nuclear layer corresponding to placoid lesions, and a disrupted inner and outer segment (IS/OS) junction (arrow). Note the normal architecture of intervening areas, with a clearly identifiable IS/OS junction, Verhoeff membrane (VM), and RPE. ELM indicates external limiting membrane. C, Spectral-domain optical coherence tomographic imaging 2 months after the initial visit showing resolution of the hyperreflective lesions but with thinning of the corresponding areas in the outer nuclear layer left behind. The hyperreflective bands representing the IS/OS junction and VM were also disrupted in the intervening area (asterisk), which appeared normal during the acute phase.

1.
Souka  AAHillenkamp  JGora  FGabel  VPFramme  C Correlation between optical coherence tomography and autofluorescence in acute posterior multifocal placoid pigment epitheliopathy. Graefes Arch Clin Exp Ophthalmol 2006;244 (10) 1219- 1223
PubMed
2.
Fine  HFKim  EFlynn  TEGomes  NLChang  S Acute posterior multifocal placoid pigment epitheliopathy following varicella vaccine [published online August 26, 2008]. Br J Ophthalmol
PubMed10.1136/bjo.2008.144501
3.
Lofoco  GCiucci  FBardocci  AQuercioli  PSteigerwalt  RD  JrDe Gaetano  C Optical coherence tomography findings in a case of acute multifocal posterior placoid pigment epitheliopathy (AMPPPE). Eur J Ophthalmol 2005;15 (1) 143- 147
PubMed
4.
Lim  LLWatzke  RCLauer  AKSmith  JR Ocular coherence tomography in acute posterior multifocal placoid pigment epitheliopathy. Clin Experiment Ophthalmol 2006;34 (8) 810- 812
PubMed
5.
Scheufele  TAWitkin  AJSchocket  LS  et al.  Photoreceptor atrophy in acute posterior multifocal placoid pigment epitheliopathy demonstrated by optical coherence tomography. Retina 2005;25 (8) 1109- 1112
PubMed
6.
Fiore  TIaccheri  BAndroudi  S  et al.  Acute posterior multifocal placoid pigment epitheliopathy: outcome and visual prognosis. Retina 2009;29 (7) 994- 1001
PubMed
Research Letters
May 2010

Photoreceptor Changes in Acute and Resolved Acute Posterior Multifocal Placoid Pigment Epitheliopathy Documented by Spectral-Domain Optical Coherence Tomography

Author Affiliations

Author Affiliations: Singapore Eye Research Institute (Drs Cheung and Yeo) and Singapore National Eye Centre (Drs Cheung, Yeo, and Koh), Singapore.

Arch Ophthalmol. 2010;128(5):644-646. doi:10.1001/archophthalmol.2010.48

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is generally believed to be a self-limiting inflammatory disorder affecting the retinal pigment epithelium (RPE). Vision usually recovers within weeks. The exact cause and site of the pathologic abnormality are not confirmed. We describe spectral-domain (SD) optical coherence tomography (OCT) changes during acute and resolved phases, which support initial inflammation and swelling followed by progressive degenerative changes affecting the photoreceptor layers and outer retina.

Report of a Case

A 21-year-old man had headache and blurred vision (visual acuity was counting fingers OD, 20/30 OS) for 3 days. Anterior and vitreous chambers were quiet. Funduscopy showed multifocal yellow placoid lesions in the posterior pole bilaterally (Figure 1A). These were hypofluorescent early and stained late on fluorescein angiography, and they were hypofluorescent throughout on indocyanine green angiography (Figure 1B). Magnetic resonance imaging of the brain showed no abnormalities. Three pulses of intravenous methylprednisolone sodium succinate, 500 mg each, were given. New lesions were observed in the left eye as initial lesions began to resolve in the right eye. At 5 months, visual acuity was 20/20 OU. Funduscopy showed that the cream-colored lesions had resolved, leaving a diffuse area of pigmentary change (Figure 1C).

Spectral-domain OCT (Spectralis HRA; Heidelberg Engineering, Heidelberg, Germany) at the initial visit showed increased signal throughout the outer nuclear layer. The represented RPE was intact, but the 2 hyperreflective bands above (believed to represent the photoreceptor inner and outer segment junction and the Verhoeff membrane) were disrupted. Figure 2A shows a cut through the fovea (in the right eye) with corresponding 3-dimensional reconstruction showing the area of cream-colored lesions. Intervening areas of normal retina showed preservation of normal tomographic architecture on SD-OCT.

The SD-OCT imaging was performed during the resolved phase (3-dimensional OCT; Topcon Corp, Tokyo, Japan), which showed outer nuclear layer thinning. The lines representing the inner and outer segment junction and the Verhoeff membrane remained ill defined (Figure 2B and C).

Comment

The cause of APMPPE is believed to be either ischemic or inflammatory. The typical findings on fluorescein angiography have been attributed to masking by the overlying cream-colored patches at the RPE and outer retina level. Some investigators believe that the changes on indocyanine green angiography are also due to masking, although others have proposed choriocapillaris occlusion as the cause.13

Time-domain OCT studies have shown nonspecific hyperreflectivity in the outer retinal layers.13 Clouding of the RPE cell cytoplasm and/or an increase in cellularity due to inflammation have been proposed as the underlying cause. Anterior displacement of the neuroretina and outer reflective band have been observed and are proposed to be due to choroiditis.4 However, details of the actual layers within the outer retina are limited by the resolution of time-domain OCT (10 μm). One study using a research tool, ultra–high-resolution OCT (3-μm resolution), described outer nuclear layer backscattering during the acute phase followed by photoreceptor atrophy.5 We report here changes in the acute and resolved phases of APMPPE studied with commercially available SD-OCT (5-μm resolution), clearly showing photoreceptor layer disruption during the acute phase. While APMPPE often has a relatively benign prognosis, a recent series found incomplete visual recovery in a large proportion of patients.6 In our patient, despite visual acuity returning to 20/20, SD-OCT clearly showed photoreceptor atrophy and lack of reconstitution of the 2 hyperreflective bands representing the inner and outer segment junction and the Verhoeff membrane. While the border between affected and unaffected areas appeared well demarcated during the acute phase, pigmentary and atrophic changes seen in the resolved phases were much more diffuse on both funduscopy and SD-OCT. In particular, disruption of the hyperreflective bands representing the inner and outer segment junction and the Verhoeff membrane was seen in areas that initially appeared normal tomographically during the acute phase (Figure 2B and C). We hypothesize that in addition to the inflammatory component, secondary progressive degenerative changes of the photoreceptor and RPE may play a role in tissue destruction, resulting in the commonly recognized pigmentary changes in resolved APMPPE.

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Article Information

Correspondence: Dr Cheung, Singapore National Eye Centre, 11 Third Hospital Ave, Singapore 168751 (gemmy.cheung.c.m@snec.com.sg).

Financial Disclosure: None reported.

References
1.
Souka  AAHillenkamp  JGora  FGabel  VPFramme  C Correlation between optical coherence tomography and autofluorescence in acute posterior multifocal placoid pigment epitheliopathy. Graefes Arch Clin Exp Ophthalmol 2006;244 (10) 1219- 1223
PubMed
2.
Fine  HFKim  EFlynn  TEGomes  NLChang  S Acute posterior multifocal placoid pigment epitheliopathy following varicella vaccine [published online August 26, 2008]. Br J Ophthalmol
PubMed10.1136/bjo.2008.144501
3.
Lofoco  GCiucci  FBardocci  AQuercioli  PSteigerwalt  RD  JrDe Gaetano  C Optical coherence tomography findings in a case of acute multifocal posterior placoid pigment epitheliopathy (AMPPPE). Eur J Ophthalmol 2005;15 (1) 143- 147
PubMed
4.
Lim  LLWatzke  RCLauer  AKSmith  JR Ocular coherence tomography in acute posterior multifocal placoid pigment epitheliopathy. Clin Experiment Ophthalmol 2006;34 (8) 810- 812
PubMed
5.
Scheufele  TAWitkin  AJSchocket  LS  et al.  Photoreceptor atrophy in acute posterior multifocal placoid pigment epitheliopathy demonstrated by optical coherence tomography. Retina 2005;25 (8) 1109- 1112
PubMed
6.
Fiore  TIaccheri  BAndroudi  S  et al.  Acute posterior multifocal placoid pigment epitheliopathy: outcome and visual prognosis. Retina 2009;29 (7) 994- 1001
PubMed
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