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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.
Background And Objective:
To evaluate clinical features, course, and outcome of patients with acute exudative paraneoplastic polymorphous vitelliform maculopathy (AEPPVM).
Patients And Methods:
Retrospective case series of 5 patients.
Results:
There were 3 males and 2 females, with a median age of 74 years. The primary neoplasms were cutaneous melanoma (n = 2), choroidal melanoma (n = 1), lung adenocarcinoma (n = 1), and lung plus breast adenocarcinoma (n = 1). The mean interval between the diagnosis of the primary neoplasm and the diagnosis of AEPPVM was 42 months. The presenting symptom was blurred vision in all cases. Ophthalmoscopy disclosed multifocal localized shallow serous detachments of the post-equatorial neurosensory retina with yellow-white subretinal debris confirmed by optical coherence tomography (OCT). There was a mean of 21 individual sites of detachment per eye, each measuring a mean of approximately 0.8 millimeter in diameter. Fundus autofluorescence depicted hyperautofluorescence corresponding to the detachments. After mean follow-up of 5 months, three patients had died of metastases. Of the two survivors, one showed resolution of lesions and the other was unchanged.
Conclusion:
AEPPVM is a paraneoplastic retinopathy found in patients with metastatic melanoma or carcinoma. The most salient feature is reduced visual acuity from multifocal shallow retinal detachments less than 1-mm diameter, best depicted on OCT.
Insights
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.
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.
