Overview
AEPVM is a very rare bilateral condition characterised by small yellowish bleb-like lesions at the level of the RPE scattered along the vascular arcades. These lesions are similar to the lesions of a vitelliform macular dystrophy and develop in a honeycomb-like pattern. Lesion patterns and progression are typically symmetrical between the two eyes.
Serous macular detachment with associated polymorphous subretinal yellowish deposits are also be associated with this condition. At this stage hyper-fluorescence is noted on fundus autofluorescence. As the vitelliform material is reabsorbed, this hyper-fluorescence decreases.
The cause of AEPVP is unclear and there are no known genetic associations.
Patients experience gradual visual recovery over months to years, but electrophysiologic abnormalities may persist. Choroidal neovascularisation is a potential complication of this condition.
Clinically, AEPVM is difficult to distinguish from paraneoplastic disease and Best disease and these differentials need to be carefully considered. Paraneoplastic disease is usually associated with a history of known cancer (particularly melanoma) while Best disease is associated with a genetic anomaly.
Differential diagnosis
References
Barbazetto, I. Dansingani, KK. Dolz-Marco, R. Giovannini, A. Piccolino, FC. Agarwal, A. Lima, LH. Vianna, RN, Yannuzzi, LA. (2017) Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy : Clinical Spectrum and Multimodal Imaging Characteristics, Ophthalmology Vol 125(1) pp 75-88.
Cruz-Villegas, V. Villate, N. Knighton, RW. Rubsamen, P. Davis, JL. (2003) Optical coherence tomographic findings in acute exudative polymorphous vitelliform maculopathy, American Journal of Ophthalmology, Volume 136, Issue 4, Pages 760-763.