Acute exudative paraneoplastic polymorphous vitelliform maculopathy in five cases

Ophthalmic Surg Lasers Imaging. 2012 Sep-Oct;43(5):366-73. doi: 10.3928/15428877-20120712-01. Epub 2012 Jul 19.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acute Disease
  • Adenocarcinoma / pathology
  • Aged
  • Breast Neoplasms / pathology
  • Choroid Neoplasms / pathology
  • Electroretinography
  • Exudates and Transudates
  • Female
  • Fluorescein Angiography
  • Humans
  • Lung Neoplasms / pathology
  • Male
  • Melanoma / pathology
  • Middle Aged
  • Paraneoplastic Syndromes, Ocular / diagnosis*
  • Paraneoplastic Syndromes, Ocular / physiopathology
  • Retina / physiopathology
  • Retinal Detachment / diagnosis
  • Retrospective Studies
  • Skin Neoplasms / pathology
  • Tomography, Optical Coherence
  • Vision Disorders / diagnosis
  • Visual Acuity
  • Vitelliform Macular Dystrophy / diagnosis*
  • Vitelliform Macular Dystrophy / physiopathology