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Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy: Clinical Spectrum and Multimodal Imaging

Irene Barbazetto1, Kunal K Dansingani1, Rosa Dolz-Marco1

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This study describes clinical findings in acute exudative polymorphous vitelliform maculopathy (AEPVM), revealing a more variable course than previously understood. Early diagnosis and evaluation are crucial to differentiate AEPVM from similar conditions.

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Area of Science:

  • Ophthalmology
  • Retinal Diseases
  • Macular Disorders

Background:

  • Acute exudative polymorphous vitelliform maculopathy (AEPVM) is a rare macular disorder.
  • Previous descriptions of AEPVM suggest a relatively consistent clinical course.

Purpose of the Study:

  • To describe the clinical findings in patients diagnosed with idiopathic acute exudative polymorphous vitelliform maculopathy (AEPVM).

Main Methods:

  • A retrospective, observational, multicenter case series review was conducted.
  • Clinical charts, multimodal imaging, and electrophysiologic and genetic findings were analyzed for previously unpublished patients.
  • A comprehensive literature review was also performed.

Main Results:

  • Eighteen patients (21-74 years) presented with serous detachments and characteristic vitelliform deposits.
  • Findings evolved over weeks to years, with some patients exhibiting novel features like intraretinal cysts and choroidal neovascularization.
  • Recurrence and delayed visual recovery were observed in some cases.

Conclusions:

  • AEPVM demonstrates a more variable natural course than previously recognized.
  • Differential diagnosis is critical, as paraneoplastic retinopathy and autosomal recessive bestrophinopathy can mimic AEPVM.
  • This study expands the known clinical spectrum of AEPVM.