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Author Spotlight: Ex Vivo OCT-Based Multimodal Imaging of Human Donor Eyes for Research into Age-Related Macular Degeneration
Published on: May 26, 2023
ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY AND PLACOID VARIANT DISEASES MASQUERADING AS AGE-RELATED
John Yu Cheng1, Ahmad Santina1, J Ben Margines1
1Jules Stein Eye Institute, University of California Los Angeles, Los Angeles.
Purpose:
To report eight cases of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) or persistent placoid maculopathy (PPM) initially masquerading as age-related macular degeneration in elderly individuals.
Methods:
APMPPE or PPM eyes in patients above age 55 years with macular retinal pigment epithelium disruption including drusenoid lesions on macular examination and/or with multimodal imaging were included. At least one method of multimodal imaging including fluorescein angiography (FA), indocyanine green angiography, optical coherence tomography (OCT), and OCT angiography (OCTA) was performed in all eyes for diagnosis and to monitor for macular neovascularization.
Results:
Eight elderly male patients presented with vision loss and were all initially diagnosed with non-neovascular or neovascular age-related macular degeneration. With the aid of multimodal retinal imaging, a final diagnosis of either APMPPE or PPM was rendered. With FA and indocyanine green angiography, choroidal hypoperfusion was detected in all but one eye. With OCT, the angular sign of Henle fiber layer hyperreflectivity was identified in >50% of eyes. With OCTA, inner choroidal flow deficits were detected in all eyes. Macular neovascularization requiring anti-vascular endothelial growth factor injection therapy complicated three of eight cases.
Conclusion:
Both APMPPE and PPM may develop in elderly individuals and may masquerade as age-related macular degeneration on presentation. Multimodal imaging including FA, indocyanine green angiography, and OCTA are important diagnostic modalities to assess for inner choroidal hypoperfusion to arrive at an accurate diagnosis and to detect macular neovascularization, which frequently complicates APMPPE and PPM. In these patients, serial anti-vascular endothelial growth factor intravitreal injections are essential in treating macular neovascularization and in preventing significant vision loss.
Insights
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and persistent placoid maculopathy (PPM) can mimic age-related macular degeneration in older adults. Multimodal imaging is crucial for accurate diagnosis and detecting complications like macular neovascularization.
Area of Science:
- Ophthalmology
- Retinal Diseases
- Medical Imaging
Background:
- Age-related macular degeneration (AMD) is a common cause of vision loss in the elderly.
- Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and persistent placoid maculopathy (PPM) are inflammatory conditions that can affect the macula.
- These conditions can present with symptoms similar to AMD, leading to diagnostic challenges.
Purpose of the Study:
- To describe eight cases of APMPPE or PPM in elderly patients that initially resembled AMD.
- To highlight the role of multimodal imaging in differentiating these conditions from AMD.
- To report on the management and outcomes of these patients.
Main Methods:
- Retrospective review of eight elderly male patients diagnosed with APMPPE or PPM.
- Initial diagnosis considered was non-neovascular or neovascular AMD.
- Multimodal imaging including fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography (OCT), and OCT angiography (OCTA) was utilized.
Main Results:
- All eight patients were initially diagnosed with AMD.
- Multimodal imaging revealed APMPPE or PPM as the final diagnosis.
- FA and ICGA showed choroidal hypoperfusion in most cases.
- OCT identified the angular sign in over 50% of eyes.
- OCTA detected inner choroidal flow deficits in all eyes.
- Macular neovascularization occurred in three cases, requiring anti-VEGF therapy.
Conclusions:
- APMPPE and PPM can present atypically in elderly individuals, mimicking AMD.
- Multimodal imaging is essential for accurate diagnosis by identifying choroidal hypoperfusion and other characteristic signs.
- Early detection and management of macular neovascularization with anti-VEGF therapy are critical for preserving vision.
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