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Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy - Case Series and Extended Features.

Efrat Naaman¹, Assaf Rosenberg^2,3, 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.

Keywords:

AEPVM Idiopathic acute exudative polymorphous vitelliform maculopathy outer retina elongation

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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Describe the disease course in three unpublished subjects with AEPVM.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.