Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy: Insight into Imaging Features and Outcomes

Abstract

The authors describe imagiological findings in idiopathic exudative polymorphous vitelliform maculopathy. A 41-year-old woman complained of bilateral blurry vision. Best-corrected visual acuity was 20/20 bilaterally. Bilateral small serous neurosensory detachments in the fovea were seen at fundoscopy and confirmed by spectral-domain optical coherence tomography. Fluorescein angiography was unremarkable. Indocyanine green angiography presented discrete hyperfluorescent spots on the posterior pole. Later, more bleb-like lesions with a vitelliform appearance and hyperautofluorescent on blue fundus autofluorescence were detected. One year later, a complete resolution of the fluid was observed. To conclude, multimodal evaluation of patients with idiopathic exudative polymorphous vitelliform maculopathy is essential for the correct diagnosis of this disease.

Acute Exudative Polymorphous Vitelliform Maculopathy as the Initial Presentation of Human Immunodeficiency Virus Infection

“…5 Later on, the condition was linked to paraneoplastic phenomena and viral illness. 6 Although one previous case report by Massaro et al 7 described AEPVM in a patient with HIV infection and malignant melanoma, our case describes AEPVM as the initial presentation of HIV infection in an otherwise asymptomatic 21-year-old woman.…”

Idiopathic acute exudative polymorphous vitelliform maculopathy: the importance of multimodal imaging, systemic workup and genetic testing

“…Family history is usually present in this setting. Additionally, a complete systemic workup, including search for an occult malignancy or infection, should be carried out to exclude paraneoplastic or infectious polymorphous retinopathy 13. In paraneoplastic disease, the retinal lesions are usually oval or circular elevations without the vitelliform-like material.…”

“…Regarding treatment, there is currently no consensus. Generally, the visual prognosis is favourable with a good recovery of visual function without any specific therapy 13…”

“…On the other hand, similarities are also evident between AEPVM and vitelliform macular dystrophies such as multifocal BVMD or ARB, although no genetic association has ever been found in patients with AEPVM. Interestingly, there are only ~20 idiopathic cases reported in the literature to date 1 13 14…”

Acute Exudative Polymorphous Vitelliform Maculopathy Associated With Primary Epstein–barr Virus Infection

“…Multiple cases of AEPVM have been described in the literature. [1][2][3][4][5][7][8][9] Various etiologies may be possible including infectious and paraneoplastic causes. [2][3][4][5] In our case, AEPVM occurred in an otherwise healthy 25-year-old woman with concurrent primary EBV infection at the time of onset.…”