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Acute exudative paraneoplastic polymorphous vitelliform maculopathy in five cases.

Saad A Al-Dahmash1, Carol L Shields, Carlos G Bianciotto

  • 1Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.

Ophthalmic Surgery, Lasers & Imaging : the Official Journal of the International Society for Imaging in the Eye
|July 25, 2012
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Acute exudative paraneoplastic polymorphous vitelliform maculopathy (AEPPVM) is a rare paraneoplastic retinopathy. This condition presents with vision loss due to multifocal retinal detachments in patients with metastatic melanoma or carcinoma.

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Area of Science:

  • Ophthalmology
  • Oncology
  • Retinal Diseases

Background:

  • Investigating acute exudative paraneoplastic polymorphous vitelliform maculopathy (AEPPVM).
  • Evaluating clinical features, disease course, and outcomes in affected patients.

Observation:

  • Retrospective case series of 5 patients (median age 74 years).
  • Neoplasms included melanoma and adenocarcinoma; mean interval to AEPPVM diagnosis was 42 months.
  • Presenting symptom was blurred vision; ophthalmoscopy revealed multifocal shallow neurosensory retinal detachments (<1mm diameter) with subretinal debris.

Findings:

  • Optical coherence tomography (OCT) confirmed retinal detachments and subretinal debris.
  • Fundus autofluorescence showed corresponding hyperautofluorescence.
  • Mean follow-up of 5 months revealed mortality in 3 patients due to metastases; 1 survivor had lesion resolution, another was stable.

Implications:

  • AEPPVM is a paraneoplastic retinopathy associated with metastatic melanoma or carcinoma.
  • Key features include reduced visual acuity from multifocal shallow retinal detachments.
  • OCT is crucial for diagnosing AEPPVM, highlighting its link to advanced cancers.