Congenital cataract associated with persistent fetal vasculature: findings from IoLunder2

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 post-operative 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

5-year outcomes after primary intraocular lens implantation in children aged 2 years or younger with congenital or infantile cataract: findings from the IoLunder2 prospective inception cohort study

BACKGROUND: International initiatives to prevent childhood blindness have highlighted the importance of early, effective intervention for congenital and infantile cataract. In the UK, intraocular lens implantation has been widely adopted by surgeons to treat these conditions. However, evidence about the benefits and risks of this technique in different age groups is limited. The IoLunder2 study assessed outcomes following primary intraocular lens implantation in children aged 2 years and younger with congenital or infantile cataract. METHODS: The IoLunder2 study was a prospective observational cohort study done at 31 sites in the UK and Ireland. Eligible children were aged 2 years or younger who had cataract surgery concurrently with intraocular lens implantation or conventional treatment (aphakic correction with contact lenses or glasses) after cataract surgery between Jan 1, 2009, and Dec 31, 2010. Children with significant ocular comorbidity precluding lens implantation, defined by the presence of complex persistent fetal vasculature, other ocular structural anomalies, severe microcornea (horizontal corneal diameter <9·5 mm), or severe microphthalmos (axial length <16 mm), were excluded from the analysis of the key outcomes. Postoperative visual rehabilitation was assessed at 1, 3, and 5 years after surgery with a 4m logarithm of the minimum angle of resolution (logMAR) notation test. Best corrected visual outcome (acuity) overall was assessed 5 years after surgery for children with bilateral and unilateral cataract. We also used multivariable logistic and linear regression to model the association between intraocular lens implantation and outcomes of interest (vision, glaucoma, and visual axis opacity). FINDINGS: A total of 256 eligible children were recruited; two had incomplete data and were excluded. 158 of the 254 included children (102 [65%] with bilateral cataract and 56 [35%] with unilateral cataract) had no significant ocular morbidity and were analysed for the key outcomes. Primary intraocular lens implantation was done in 88 (56%) of 158 children (50 children with bilateral cataract and 38 children with unilateral cataract). 70 (44%) of 158 children had conventional treatment (52 with bilateral cataract and 18 with unilateral cataract). Overall median visual acuity at 5 years was 0·34 logMAR (IQR 0·20-0·54) for children with bilateral cataract and 0·70 logMAR (IQR 0·3-1·3) in the operated eye for children with unilateral cataract. Primary intraocular lens implantation was not associated with better visual outcome than conventional treatment in children with bilateral cataract (adjusted coefficient -0·1, 95% CI -0·5 to 0·3, p=0·48) or unilateral cataract (adjusted coefficient -0·3, -0·6 to 0·2, p=0·36), or reduced incidence of postoperative glaucoma in children with bilateral cataract (adjusted odds ratio [OR] 0·5, 0·10 to 1·80, p=0·28), but was associated with a five times higher risk of reoperation for visual axis opacity requiring general anaesthesia in children with bilateral cataract (adjusted OR 5·94, 95% CI 2·14-16·47, p=0·001) and a 20 times higher risk in children with unilateral cataract (20·15, 3·01-134·00, p=0·001). INTERPRETATION: The findings of this cohort study indicate that intraocular lens implantation does not confer better vision or protection against postoperative glaucoma, and conversely increases the risk of requiring early reoperation in children younger than 2 years with bilateral or unilateral cataract. The routine use of intraocular lens implantation in this age group cannot be recommended. FUNDING: National Institute for Health Research, Ulverscroft Foundation, and the Academy of Medical Sciences

Glaucoma following cataract surgery in the first two years of life: frequency, risk factors and outcomes from IoLunder2

Background: we investigated glaucoma related adverse events, predictors and impact at five years following surgery in the IoLunder2 cohortMethods: population based observational cohort study of children undergoing cataract surgery aged 2 years or under between January 2009 to December 2010. Glaucoma was defined using internationally accepted taxonomies based on the consequences of elevated intraocular pressure. Glaucoma related adverse events were any involving elevated intraocular pressure. Multivariable analysis was undertaken to investigate potential predictors of secondary glaucoma with adjustment for within-child correlation in bilateral cataract. Unilateral and bilateral cataract were analysed separately.Results: complete follow-up data were available for 235of 254, 93% of the inception cohort. By five years after primary cataract surgery, 20% of children with bilateral cataract and 12% with unilateral had developed secondary glaucoma. Glaucoma related complications had been diagnosed in 24% and 36% of children, respectively. Independent predictors of glaucoma were younger age at surgery (adjusted odds ratio for reduction of week in age:1.1, 95%CI 1.1–1.2, p&lt;0.001); the presence of significant ocular comorbidity (adjOR3.2, 95%CI 1.1–9.6, p=0.01); and shorter axial length (adj OR for each mm 1.7, 95% CI 10.0–1, p=0.05) for bilateral cataract. Shorter axial length was the single independent factor in unilateral disease (adjOR9.6, 95% CI 1.7–52, p=0.009)Conclusions: both younger age at surgery (the strongest marker of ocular ‘immaturity’) and smaller ocular size (a marker of both immaturity and developmental vulnerability) can be used to identify those at greatest risk of glaucoma due to early life cataract surgery