Surgical outcomes of posterior persistent fetal vasculature syndrome: cases with tent-shaped and closed funnel-shaped retinal detachment

Abstract

Background/objectives To determine the role of vitreoretinal surgery (VRS) for two different forms of posterior persistent fetal vasculature syndrome (PFVS); with tent-shaped tractional retinal detachment (TRD) and closed funnel-shaped TRD. Subjects/methods Retrospective, single surgeon, consecutive case series of 52 eyes of 44 patients with posterior PFVS who underwent VRS. Cases were divided into "tent-shaped TRD" and "funnel-shaped TRD" groups based on the preoperative TRD configuration. Associated anomalies, functional and anatomical outcomes were evaluated. The cosmetic appearance was defined as poor if there was phthisis bulbi, gross buphthalmos, or corneal opacification; acceptable if there was apparent leukocoria; and excellent if none were noted at the last follow-up. Results Thirty eyes of 29 patients presented with tent-shaped TRD; 70% of which obtained counting fingers or better vision and 90% showed significant reversal of tenting achieving retinal reattachment. The cosmetic appearance was excellent in 87%. Two eyes (7%) became phthisic. Twenty-two eyes of 15 patients presented with funnel-shaped TRD and leukocoria; 45% achieved LP vision and 70% of patients with bilateral pathology had LP in at least one eye. The cosmetic appearance was acceptable to excellent in 73%. Three eyes (14%) became phthisic, one (5%) of which required enucleation. The median follow-up time was 16 (6-71) months. Conclusions VRS often provides functional vision and anatomy in posterior PFVS with tent-shaped TRD morphology. In the funnel-shaped TRD morphology, where no treatment has historically been recommended, surgery may be considered with an aim of restoring light perception and globe preservation, particularly in bilateral cases.