Persistent Fetal Vasculature With Elongated Ciliary Processes in Children

Purpose Outcomes and reoperation rates in infants with unilateral persistent fetal vasculature (PFV) with elongated or stretched ciliary processes have not been extensively studied and were excluded from the Infant Aphakia Treatment Study (IATS). The purpose of this study is to analyze the preoperative measurements, reoperation rates, and complications after surgery in unilateral anterior PFV with stretched processes. Design Retrospective case series. Methods Inclusion criteria consisted of unilateral cataract surgery prior to 7 months of age and anterior PFV with elongated ciliary processes. Eyes with posterior retinal involvement and less than 6 months of follow-up were excluded. All patients underwent lensectomy, posterior capsulectomy, and vitrectomy. Results Eleven eyes of 11 patients were included. Patients had a mean age at surgery of 2.4 ± 1.4 months. Average follow-up was 4.5 ± 3.7years. Globe axial lengths were 18.6 ± 1.9 mm. Ten patients (91%) were initially left aphakic. Three patients (27%) later received a secondary intraocular lens (IOL), and 1 patient underwent an IOL exchange. Six out of 10 (60%) aphakic patients developed visual axis opacification. One aphakic patient required topical therapy for glaucoma. One additional patient developed neovascular glaucoma and retinal detachment. The eye was subsequently enucleated. Three patients underwent strabismus surgery. Two patients underwent pupilloplasty. Two patients (18.2%) had a final visual acuity better than 20/200. Conclusion Eyes operated for PFV with elongated ciliary processes are unlikely to have a final visual acuity greater than 20/200 and many will need additional surgery. Postoperative visual axis opacification occurred in 60% and glaucoma developed in 18%.