Surgical Treatment and Prognosis of Congenital Cataract with Persistent Fetal Vasculature

Objective: To observe the clinical characteristics and prognosis of surgical intervention in congenital cataract associated with persistent fetal vasculature (PFV). Methods: In this retrospective case series study, 28 eyes of 24 congenital cataract children with PFV were enrolled from May 2011 to June 2017 at Eye Hospital, Wenzhou Medical University (Hangzhou). Age at surgery, surgical approaches, clinical signs, and visual acuity (VA) were collected both before and after surgery. Results: We found congenital pupil anomalies in 14 eyes, umbilication of lens or zonular anomalies in 6 eyes, posterior capsule defect in 9 eyes, congenital microcornea or corneal limbal stem cell anomalies in 2 eyes, fundus lesion in 2 eyes, nystagmus in 5 eyes, strabismus in 7 eyes, and cleft palate in 1 children. Seven children over 3-years old (10 eyes) underwent lensectomy combined with anterior vitrectomy and intraocular lens (IOL) implantation. The median follow-up duration was 8 months (quartile range, 2.5-41.0 months), and all patients had achieved VAs over 0.1 at the last follow up, and 2 patients had VAs over 0.3. Seventeen children (18 eyes) under 1 year of age underwent lensectomy combined with anterior vitrectomy. The Snellen VA measured by preferential looking cards was over 0.1 in all patients, with significant improvement of pursuit and nystagmus. Six children (6 eyes) underwent both primary lensectomy combined with anterior vitrectomy (mean ± standard deviation 5.5 ± 2.6 months) and secondary IOL implantation (mean age 23.5 ± 8.1 months). One eye achieved a VA of 0.6, close to normal for children of the same age, and 5 eyes reached the standard logarithmic chart VA of more than 0.1. The follow-up duration ranged from 4 months to 6 years, and the median follow-up time was 12 months. No serious postoperative complications occurred. Conclusions: Most congenital cataracts with PFV are unilateral. The incidence of complications after microincision lensectomy combined with anterior vitrectomy is low, with significantly improved visual outcomes. Normal visual acuity can be expected in a few cases. Key words: persistent fetal vasculature; persistent hyperplastic primary vitreous; congenital cataract