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Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy - Case Series and Extended Features
Efrat Naaman1, Assaf Rosenberg2,3, Wasim Nasser4
1Department of Ophthalmology, Rabin Medical Center, Petah Tikva, Israel.
Retinal Cases & Brief Reports
|July 25, 2025
Summary
Idiopathic acute exudative polymorphous vitelliform maculopathy (AEPVM) is a rare condition. New imaging reveals outer retinal elongation in the macula, suggesting photoreceptor or RPE interaction issues.
Area of Science:
- Ophthalmology
- Retinal Diseases
- Medical Imaging
Background:
- Idiopathic acute exudative polymorphous vitelliform maculopathy (AEPVM) is a rare condition.
- Characterized by acute vision loss and distinctive retinal findings.
Purpose of the Study:
- Describe the disease course in three unpublished subjects with AEPVM.
- Identify and characterize a previously undescribed imaging feature in AEPVM.
Main Methods:
- Clinical presentation and disease progression analysis.
- Multimodal imaging, including spectral-domain optical coherence tomography (SD-OCT).
- Retinal electrophysiology (ERG) and genetic testing (PRPH2, BEST1).
Main Results:
- Observed bilateral serous macular detachments coalescing into vitelliform deposits.
- SD-OCT revealed selective outer retinal elongation in the macula.
- ERG showed reduced responses; genetic testing and malignancy screening were negative.
Conclusions:
- AEPVM presents with unique retinal imaging findings.
- Outer retinal elongation is localized to the macula, supported by ERG results.
- Findings suggest a potential disruption at the macular photoreceptor-RPE interface.
