Management and Outcomes of Posterior Persistent Fetal Vasculature

Abstract

To describe the clinical features, management and outcomes of posterior persistent fetal vasculature (PFV) and suggest a management algorithm. Retrospective consecutive case series. All children diagnosed with posterior PFV and treated or followed at the Rothschild Foundation Hospital in France between June 2011 and September 2021. Retrospective analysis of the clinical characteristics of posterior PFV. We reported at diagnosis: age, gender, presenting symptoms, intraocular pressure (IOP), visual acuity (VA). Patients were divided in four group depending on severity and involvement or not of the anterior segment. We reported the vitreoretinal surgical techniques used. Anatomical results, ocular hypertension, BCVA, presence of post-operative adverse events and additional surgical interventions were recorded at each follow-up visit. A total of 96 patients were included. Median age at diagnosis was 8 months (mean 18.9±30.9 months) with a mean follow-up of 27±31.2 months. Although PFV is often an isolated disease, it was associated with a systemic disease in 8% of cases. Posterior PFV was associated with anterior involvement in 62 eyes (64%). Forty-one eyes (42.7%) were microphthalmic and were more frequently associated with severe PFV [53% vs. 25%; (p=0.01)]. Surgery was performed in 85 patients (89%). Of them, 69 (81%) were a total success, 5 (6%) were a partial success due to a persistent limited peripheral retinal detachment (RD), and 11 (13%) were a failure due to persistent total RD after surgery. Twenty-four eyes presented post-operative adverse events including ocular hypertension requiring eye drop medication (6.6%), secondary cell proliferation around the IOL (7.7%), intravitreal hemorrhages (6.6%), persistent tractional RD (9.9%). A second surgery was performed in 15 patients (16%). At last follow-up, VA could be measured in logMAR in 43 children (45%), was light perception in 21 eyes (22%), and no light perception or impossible to assess in 32 eyes (33%). In our case-series, most of patients presenting PFV with posterior involvement received complex vitreoretinal surgery. Goals of the surgery varies, and include retinal flattening, reduction of vitreoretinal traction, freeing of the visual axis and aesthetic concerns. We proposed a surgical and medical management algorithm for PFV.