Wagner Syndrome-like fundus presentation of atypical Persistent Fetal Vasculature.

Abstract

Purpose: To report the case of a woman with atypical Persistent fetal vasculature (PFV) accompanied by more typical findings of Wagner Syndrome.

Results: A 58-year-old woman complained about persistent flashes and floaters for more than 6 months. Multimodal retinal imaging showed Bergmeister papilla and a fibrous gray strand floating behind the macula in the right eye. Ultra-wide field retinography revealed a 270° paving-stone degeneration and a nasal circumferential vitreous veil in both eyes. Functional and genetic testing were negative for inherited retinal diseases.

Conclusions and importance: PFV spectrum confirms its heterogeneous presentation. We report an atypical adult PFV case in presence of retrolental and optic nerve head stalks, accompanied by extensive peripheral vitreoretinal degenerations. This case may expand the current understanding of PFV presentation in adulthood.