PURPOSE: To describe clinical features, management, and outcomes of anterior segment-dominated persistent fetal vasculature (aPFV). DESIGN: A secondary analysis of aPFV data from a longitudinal cohort study. METHODS: The age and gender of each participant upon initial presentation, along with biological parameters, best-corrected visual acuity, and biomicroscopic assessments were collected. The aPFV eyes were categorized into 3 groups (type I: pupillary-iris-anterior lens plane, type II: retrolental-anterior hyaloid membrane plane, and type III: combined) according to the location of the vascular abnormalities and further classified into mild (a) and severe (b) according to the degree of severity. The surgical techniques used in this investigation were reported. Visual outcomes, ocular hypertension, postoperative adverse events, and additional surgical interventions were recorded at each follow-up visit. RESULTS: A total of 470 individuals (619 eyes) were enrolled (ages 36.7 ± 45.4 months, range 1-193 months), divided into 129 eyes (20.8%) Type I, 420 eyes (67.9%) Type II, and 70 eyes (11.3%) Type III. Cataracts were identified in 505 eyes (81.6%): for type I, the common forms were nuclear and membranous opacity (both 12/50 eyes, 24%)
type II: subcapsular opacity (214/399 eyes, 53.6%) and type III: complete cortex (17/56 eyes, 30.4%). Posterior capsular tear was mostly present in type IIa. Type III had the shallowest anterior chamber depth and the largest corneal astigmatism. Six hundred patients (96.9%) had surgery. After surgery, 119 eyes experienced adverse events: intravitreal hemorrhages occurred in 0.2% of eyes, visual axis obscuration occurred in 9.4% of eyes, and ocular hypertension which required eye drop medication occurred in 19.2% of eyes. In 47 patients (7.6%), a second surgery procedure was done. In 456 eyes (73.7%), visual acuity had improved at the last follow-up. The worst best-corrected visual acuity was associated with types IIb and IIIb. CONCLUSIONS: The magnitude of aPFV can be usefully characterized by a classification protocol focusing on the location and disease severity. Here, we propose a surgical and medical management algorithm for aPFV to achieve favorable outcomes with limited intraoperative and postoperative complications.