Congenital cataract associated with persistent fetal vasculature: findings from IoLunder2

Abstract

Purpose To describe the frequency, characteristics, and treatment outcome of persistent fetal vasculature (PFV) in children undergoing surgery for congenital and infantile cataract in the first 2 years of life. Patients and methods Observational population-based cohort study with case identification through active surveillance and standardised data collection via a national clinical network, the British Isles Congenital Cataract Interest Group (BCCIG). Results The IoLunder2 cohort comprises 246 children undergoing surgery for bilateral and unilateral congenital and infantile cataract in the first 2 years of life. A total of 58/246 (24%) children had PFV (%): overall, 46/95 (46%) with unilateral cataract, and 12/141 (8%) with bilateral disease. Anterior segment vascular remnants were more common in bilateral than unilateral disease (75 vs 11%, P = 0.01). At 1 year after surgery, 20% of children with bilateral PFV and 24% with unilateral had achieved normal vision for age within the operated eye. The prevalence of postoperative glaucoma was 9% (of children with bilateral disease) and 4% (unilateral). Conclusion PFV is significantly more common than previously reported, and outcomes are comparable to that for congenital and infantile cataract overall.

Evaluation of collagen derived antiangiogenic factors and matrix metalloproteinases in anterior lens epithelial cells of pediatric eyes with persistent fetal vasculature

“…[211] In a study reported by Solebo et al , they have observed the incidence of PFV to be 24% in children with congenital cataract, out of which 46% had unilateral and 8% had bilateral cataract. [12] Age of presentation in our study was ranging from 3 months to 132 months (mean: 41.15 months), which is corresponding to the study done by Mullner-Eidenbock A et al who reported age of presentation ranging from 2 weeks to 15 years in 31 cases of unilateral cataract with PFV. [13] This wide range of age at presentation can be attributed to the fact that all the cases in our study had unilateral cataract with PFV which is likely to go unnoticed because of the normal functioning other eye.…”

Cataract morphology and risk for glaucoma after cataract surgery in infants with unilateral congenital cataract

“…The IoLunder 2 cohort study also did not find an association between cataract type and the development of glaucoma. 8 …”

Practice patterns in pediatric cataract management: Time for real world data

“…Pediatric cataract management has gained tremendous focus in the past decade. [ 1 2 3 4 5 6 7 8 9 10 11 12 13 14 ] There is a renewed sense that while cataract surgery in adults appears to be evolving at a furious pace that pediatric cataract surgery is perhaps lagging in our understanding of the basic physiological and anatomical issues that play a role in a successful outcome.…”

“…It is, in fact, the bilateral dense cataracts in children that are of greater developmental concern; evidence from a large longitudinal cohort study from the UK suggests that bilateral pseudophakia may result in better visual outcomes. [ 5 ] Why should this be ? Well most cases of unilateral cataracts are due to some form of PHPV with an eye that is usually smaller than the unaffected eye.…”

“…If we look at the numerous papers published on the subject of pediatric cataract, we begin to see that there have been numerous retrospective papers, survey data, one or two randomized control trials, some meta-analysis studies, and a Delphi consensus paper. [ 2 3 5 6 7 8 9 10 11 12 13 14 ] Do we indeed have enough data already to help answer some questions about pediatric cataract management? Maybe so but to develop RWD that leads to RWE the clinical question that we want to ask must be framed appropriately.…”