The prevalence of visual axis opacification in the Swedish Pediatric Cataract Register.

PURPOSE: To report on the occurrence of postoperative visual axis opacification (VAO) in children younger than 5 years of age operated for cataract in Sweden, and to analyse correlations with age at surgery and surgical method. METHODS: Data were derived from the Swedish Pediatric Cataract Register (PECARE). All children operated on between 1 January 2007 and 31 December 2020 were included. Follow-ups at 1, 2 and 5 years of age were analysed. RESULTS: Cataract surgery were performed on 770 eyes belonging to 549 children (n = 282 boys, 51.4%); 327/770 (42.5%) of the children underwent surgery before 3 months of age and 216/770 (28%) before 6 weeks of age. Data on 881 follow-up visits were registered. At the follow up-visits at 1, 2 and 5 years of age, VAO was present in 154/349 (44.1%), 41/323 (12.7%) and 25/208 (12%). The majority of the children with VAO underwent cataract surgery before age 6 months, with a predominance before age 2 months. Primary IOL was implanted in 601/770 (78%) of eyes; 40.8% had an acrylic one-piece lens, 31.8% had a bag-in-the-lens IOL, 21.9% were aphakic and 5.2% had an acrylic three-piece lens. Implantation of a bag-in-the-lens IOL was related to a significantly lower occurrence of VAO compared to other types of IOL, including aphakia (p < 0.0002). CONCLUSION: Our results are in accordance with the literature. Primary bag-in-the-lens IOL implantation before 2 years of age seems adequate and safe, with a low occurrence of VAO, and can thus be continued as routine in Sweden.

Analysis of age at detection and outcomes of dense unilateral congenital cataract surgery for children on the paediatric cataract register.

AIM: Analysis of age at time of detection and surgery of dense unilateral cataract and investigation of best-corrected visual acuity (BCVA) in a nationwide register-based cohort study, based on the routine of maternity ward eye screening. METHODS: Data were derived from the Paediatric Cataract Register (PECARE). All children (n = 54) diagnosed with dense congenital unilateral cataract between January 2007 and September 2014 who had surgery before 1 year of age, and for whom 5-year follow-up records were available, were included. RESULTS: The majority, 35/54 (65%), were detected and operated on before age 6 weeks and 30/35 (86%) were referred from maternity wards. Visual acuity (VA) ≥ 0.5 (decimal, 0.3 logMAR) was found in 7/53 (13%) of the cohort at age 5 years; further, 19 children achieved VA ≥ 0.1 (decimal, 1.0 logMAR) (36%) and 19 children VA < 0.05 (decimal, 1.30 logMAR) (36%). Ten-year follow-up records were available for 17/53 (32%) children; 1/17 (6%) achieved VA ≥ 0.5 (decimal, 0.3 logMAR), 4/17 (24%) VA ≥ 0.3-<0.5 (decimal, 0.52-0.30 logMAR), 3/17 (18%) VA ≥ 0.05-0.1 (decimal, 1.30-1.0 logMAR) and 10/17 (59%) VA < 0.05 (decimal, 1.30 logMAR). CONCLUSION: A total of 90% of the children were detected with cataract within 100 days of birth and 80% were operated on within this period. This study showed better visual acuity in those treated for dense unilateral cataracts than previously reported in an earlier Swedish cohort study.

Late-onset glaucoma following congenital cataract surgery: Occurrence, visual acuity and risk factors: A 37-year longitudinal follow-up.

PURPOSE: The aim of this study was to determine the prevalence of late-onset glaucoma after surgery for congenital cataract in a cohort with long-term follow-up and to evaluate visual development following the diagnosis of postoperative glaucoma in comparison with no glaucoma development. METHODS: All children born between 1980 and 1997 in the western counties of Sweden who had undergone congenital cataract surgery were included (patients n = 77, eyes n = 122). Cataract was considered congenital if there was no proof of clear lens at birth. Medical records were reviewed with regard to onset of glaucoma, age at surgery, surgical technique, coexisting eye anomalies and changes in visual acuity. Glaucoma was considered late onset if occurring after 1 year following surgery. RESULTS: Total glaucoma prevalence was 14.8%, including late (10.7%) and early onset (4.1%), with a mean follow-up of 23.2 ± 6.6 years. Microphthalmos was a significant risk factor for developing glaucoma (RR 7.75, p < 0.001). Bilaterally treated eyes had a mean visual acuity of 0.43 ± 0.33 (decimal value) at the last follow-up. With glaucoma, mean visual acuity was 0.19 ± 0.17 (decimal value). Treated eyes of patients with unilateral cataract surgery had a lower visual acuity. CONCLUSIONS: Life-long follow-up of all patients who have undergone surgery for congenital cataract in childhood is recommended. Annual check-ups of adults, including measurements of IOP and visual acuity, are recommended for patients with microphthalmos and/or who had surgery <3 months of age.

PECARE: Parental Feedback to Improve Congenital Cataract Care in Sweden.

PURPOSE: To analyze non-directed parental feedback to health care providers responsible for pediatric cataract care in Sweden. METHODS: A directed content analysis was used to analyze data consisting of text representing free comments provided by 40 parents. A deductive approach was employed by applying the model of balancing the child's inability and ability, which includes the categories mastering, collaborating, facilitating, and adapting. RESULTS: Parents lacked piloting and self-management support. They experienced an absence of partnership with the health care team and not being taken seriously. They also felt abandoned by health care, resulting in emotional distress. Parents highlighted the impact of their social network and the challenges involved in accepting and adapting to the changes in everyday life. CONCLUSIONS: This study emphasizes the consequences of the lack of a caring partnership with health care professionals. Because parents act as mediators of care to the child with congenital cataract, persistence on the part of parents and a family-centered approach are essential for the child's visual development. [J Pediatr Ophthalmol Strabismus. 2023;60(4):298-294.].

The Swedish National Pediatric Cataract Register (PECARE): incidence and onset of postoperative glaucoma.

PURPOSE: The aim was to report cumulative incidence and time of onset of postoperative glaucoma in a paediatric early cataract surgery cohort. METHODS: Data were retrieved from the Pediatric Cataract Register (PECARE), a prospective register of Swedish cataract operations before 8 years of age. All eyes with surgery between January 2007 and December 2014 and a registered follow-up were included. Cataracts caused by uveitis, trauma or coexisting congenital glaucoma were excluded. Glaucoma was defined as early onset if diagnosed within a year after surgery and late onset if diagnosed later. RESULTS: The study included 288 eyes in 207 children (106 girls), 81 with bilateral and 126 with unilateral cataracts, with a mean follow-up of 3.31 ± 1.77 years. Of the 288, 168 (58.3%) had surgery before 3 months of age; most of these 92.3% (155/168) were defined as dense, 208 (72.2%) were below 1 year of age. Cumulative incidence of surgically treated glaucoma among individuals was 23.7% (49/207). Median time to glaucoma onset was 0.91 years (range: 0.05-4.97 years) for eyes. Early-onset glaucoma was found in 98 % (63/64), and late onset in 2% (1/64). CONCLUSION: In this paediatric cataract cohort, a majority of eyes had surgery before 3 months of age (58.3%). Secondary glaucoma-onset peaked within the first postoperative year, with a cumulative incidence of 23.7%. Surgery performed after the first month of life, resulted in a lower glaucoma rate. Long-term follow-up will reveal whether the low rate of late-onset glaucoma with early surgery will last, and if so, the consequences.

Secondary glaucoma and visual outcome after paediatric cataract surgery with primary bag-in-the-lens intraocular lens.

PURPOSE: To determine predictors of secondary glaucoma and poor visual outcome in children undergoing cataract surgery with bag-in-the-lens (BIL)-IOL implantation. METHODS: Medical records were retrospectively analysed for children with primary implantation with BIL-IOL during 2009-2013. RESULTS: The study included 109 eyes. Median age at surgery was 2.5 years (range 2 weeks-14.1 years), 26.6% being ≤12 weeks of age (= early group). Median follow-up time was 2.8 years (7 months-5.8 years). 15 eyes (13.8%) developed glaucoma, 14 (48.3%) in the early group and 1 (1.3%) in the late group (p < 0.001). Within the early group, mean time for surgery differed significantly. Patients developing glaucoma had cataract surgery at 3.5 ± 1.1 weeks (mean ± SD) and the non-glaucoma patients at 5.7 ± 3.3 weeks (p = 0.024). In the entire cohort, 21 eyes (19.3%) had conditions associated with glaucoma; 57.1% of these developed glaucoma compared to 3.4% without these conditions (p < 0.001). Corrected distance visual acuity (CDVA) (decimal) of ≥0.5 was seen in 50 eyes (48.5%), median 0.63 in the late group and 0.15 in the early group. Glaucoma eyes in the early group (surgery at 3.5 weeks) achieved 0.56 median (range, 0.4-1.0) logMAR, (0.28 decimal) CDVA, whereas non-glaucoma eyes (surgery at 5.7 weeks) achieved 0.89 median (range 0.7-1.6) logMAR (0.13 decimal) CDVA; p = 0.016. Glaucoma development in infants between 5 weeks and 2 years of age was 6.7% (n = 2/30). CONCLUSIONS: Comorbidity strongly increases the risk of secondary glaucoma. Surgery during the first month is correlated with better CDVA outcome and glaucoma. After 5 weeks of age, glaucoma rate is low with the BIL-IOL.

Phacoemulsification and primary implantation with bag-in-the-lens intraocular lens in children with unilateral and bilateral cataract.

PURPOSE: To report outcome in a paediatric cohort with cataract extraction and implantation of bag-in-the-lens intraocular lens (BIL-IOL). METHODS: Children younger than 16 years of age subjected to phacoemulsification with primary implantation of BIL-IOL during 2009 through 2013 were analysed retrospectively. Exclusion criteria were uveitis or ≤6 months of follow-up. RESULTS: In total, 109 eyes of 84 children were included; 40 unilateral and 44 bilateral cataracts. For all eyes, median age at surgery was 2.5 years (range 2 weeks to 14.1 years) and 16 children (24 eyes) were ≤6 weeks. Coexisting systemic disease was more common in children with bilateral cataract (24 patients, 54.5%) compared to unilateral cataract (6 patients, 15.0%, p < 0.0001). Ocular comorbidity was more common in unilateral cataracts; n = 14 eyes (35.0%) compared to bilateral cataracts; n = 10 eyes (14.5%; p = 0.017). Median follow-up was 2.8 years (range 7 months to 5.8 years). During the follow-up period, 15 (13.8%) eyes developed glaucoma and five (4.6%) eyes required treatment for visual axis opacification (VAO). Corrected distance visual acuity (CDVA) for bilateral cataracts at last follow-up was 0.42 ± 0.45 (logMAR; mean±SD) with 35 (55.6%) eyes attaining a CDVA of ≥0.5 (dec). For unilateral cataracts mean CDVA was significantly poorer; 0.67 ± 0.51 (p = 0.010) with 15 (37.5%) eyes attaining a CDVA of ≥0.5. CONCLUSION: For children having cataract surgery with primary implantation of BIL-IOL, VAO is rare and visual outcome overall good. Unilateral cataracts are associated with a higher proportion of ocular comorbidity and poorer visual outcome.

The Paediatric Cataract Register (PECARE): an overview of operated childhood cataract in Sweden and Denmark.

AIM: To report basic epidemiological data concerning surgically treated childhood cataract in Sweden and Denmark. METHODS: Data were derived from the Paediatric Cataract Register (PECARE), a binational, web-based surgical register representing Sweden and Denmark. All children operated before 8 years of age between 1 January 2007 and 31 December 2013 were included. Age-specific prevalence per 100 000 population was calculated. RESULTS: A total 574 operations in 213 boys (51.7%) and 199 girls (48.3%), altogether 412 children, were registered, the vast majority (n = 395/412; 95.9%) being individuals with congenital/infantile cataract. Of these 412, a total of 294 (147 boys and 147 girls) were Swedish and 118 (66 boys and 52 girls) were Danish. The age-specific prevalence of operated cataract in Sweden was 31/100 000 and in Denmark 28/100 000. In 454 of 574 eyes (79.1%), the cataract was dense. Altogether, 266 of 574 (46.3%) were operated during the first year of life, 193 during the first 12 weeks representing 33.6% of all operations. A primary intraocular lens (IOL) implantation was done in altogether 411 of 574 eyes (71,6%). In total, 210 unilateral cataract operations (210/574; 36.6%) were performed. Persistent fetal vasculature (PFV) was present in 64 of 193 (33.1%) of those with a congenital unilateral cataract. In 84 individuals (84/395; 21.3%) with congenital or infantile cataract, a coexisting disorder was found. CONCLUSION: The age-specific binational prevalence of operated congenital/infantile cataract in Sweden and Denmark is 30/100 000. About half of the operations are performed within the first year of life, one-third within the first 3 months. In our study population, a primary IOL was implanted in the majority of cases.

The Pediatric Cataract Register (PECARE): analysis of age at detection of congenital cataract.

PURPOSE: To analyse and discuss screening for the detection of congenital cataract in two Nordic countries, Denmark and Sweden. METHODS: Until 2011, in Denmark, no guideline concerning screening for congenital cataract existed. Since 2011, Danish guidelines regarding eye examination include examination with a pencil light at age 5 weeks, whereas newborn red reflex examination using a handheld ophthalmoscope is routine protocol in Swedish maternity wards. Data regarding age of referral were derived from the Pediatric Cataract Register (PECARE). All children operated on before 1 year of age between January 2008 and December 2012 were included. Statistical comparison of the different screening strategies was made. RESULTS: The number of children undergoing surgery for congenital cataract before 1 year of age was 31 (17 bilateral cases) in Denmark and 92 (38 bilateral cases) in Sweden. The proportion was 14 per 100.000 children in Denmark and 16 in Sweden (p < 0.05). There was a statistically significant difference between Denmark and Sweden in the percentage of children referred within 42 days of birth (p < 0.0001) and within 100 days (p < 0.001). CONCLUSION: Due to the screening procedure with red reflex examination, congenital cataract in Swedish children is detected significantly earlier than in Danish children.

Outcome of surgical treatment of primary and secondary glaucoma in young children.

PURPOSE: To describe a paediatric cohort surgically treated for primary or secondary glaucoma (PG/SG), with regard to incidences, visual outcome and control of intraocular pressure (IOP). METHODS: All children (n = 29, 42 eyes in total) surgically treated for PG or SG at the age of 4 years or younger between January 2002 and December 2010 at Sahlgrenska University Hospital in Mölndal were retrospectively studied through medical records. Median follow-up time after initial surgery was 5.9 years (range 2.4-11.2 years). RESULTS: The incidence of primary congenital glaucoma was 4.3 cases per 100 000 live births in the county of Västra Götaland. For glaucoma secondary to cataract surgery, the incidence was 13% with a median postoperative duration to diagnosis of glaucoma of 3.8 months (range 1.6 months to 4.3 years). Preoperative mean IOP was 31.5 (SD 8.1) mmHg, and mean IOP at last visit was 17.1 (SD 4.4) mmHg. For the entire cohort, 30% of the glaucoma eyes required more than two IOP-lowering surgical procedures during the study period. BCVA was ≥0.3 (decimal) in 45% of glaucomatous eyes at last follow-up with no statistically significant difference between PG and SG. Analysis of functional visual outcome, that is BCVA in the better eye, showed that 83% of all patients attained a BCVA of ≥0.5. CONCLUSIONS: The incidences and outcome of surgically treated paediatric glaucoma were in accordance with previous studies. Chamber angle surgery, and if necessary, tube implantation without the use of antimetabolites, is a favourable approach leaving most sites needed for future glaucoma surgery unaffected.

Combined phacoemulsification, primary intraocular lens implantation, and pars plana vitrectomy in children with uveitis.

PURPOSE: To evaluate the outcome of combined cataract surgery with primary intraocular lens (IOL) implantation and pars plana vitrectomy (PPV) in children with uveitis. METHODS: Data regarding visual acuity (VA), inflammatory status, medical therapy, and complications was collected from the medical charts of 17 children (21 eyes) with chronic uveitis who underwent combined cataract surgery and PPV at the Eye Clinic, Sahlgrenska/Mölndal, between 2002 and 2011. RESULTS: Seventy-six percent of the children had juvenile idiopathic arthritis. Median preoperative VA was 1.70 logMAR and median VA after 12 months was 0.17 logMAR. Postoperatively, glaucoma developed in 7 eyes, cystoid macular edema in 3 eyes, and visual axis opacification requiring treatment in 5 eyes. CONCLUSIONS: Although combined phacoemulsification, primary IOL implantation, and PPV in children with uveitis resulted in favorable visual outcome and stable inflammation in a majority of children, the technique should so far be reserved for uveitic cases with vitreous pathology.

Congenital cataract screening in maternity wards is effective: evaluation of the Paediatric Cataract Register of Sweden.

AIM: To study which eye-screening protocol prevails in Swedish maternity/neonatal wards, evaluate efficacy in a prospective study and compare results with earlier Swedish retrospective results. METHODS: Surveys were sent in 2006 to maternity/neonatal and women's health departments regarding screening policy. Response frequency was 96% (122/127). Data were derived from the Paediatric Cataract Register (PECARE), Sweden. All Swedish children diagnosed with congenital cataract and operated on before 1 year of age between January 2007 and December 2009 were included. Statistical comparison with earlier retrospective results was performed. RESULTS: Eye screening is a routine protocol at a rate of 90% of Swedish maternity wards. Sixty-one children were included in the study. An increase was shown in case referrals from maternity wards compared to 10 years ago (64% vs. 50%). Detection was performed within 6 weeks of age in 75% of the cases. A significant difference between the probabilities of early referral (0.38; p < 0.001, < 6 weeks of age) and early surgery (0.36; p < 0.001) (PECARE) was found in comparison with the historical data of no maternity-ward screening. Well-baby clinics were instrumental in early detection, as well. CONCLUSION: Eye screening in maternity wards is effective. Clear Swedish directives are to be preferred.

Stability of visual outcome from 7 years in children treated surgically for bilateral dense congenital cataracts before 37 weeks of age.

PURPOSE: To study the long-term visual outcome and the age at which final visual acuity can be predicted in a population sample of children treated surgically for bilateral dense congenital cataract before 37 weeks of age. In addition, we assessed the influence of associated risk factors and compared the visual development of these aphakic children with presumably blocked visual input before early surgery to that of normal children in Sweden. METHODS: The 18 patients included were followed for at least 10 years postoperatively. The median age at last visit was 15.5 years (range 10-18). The best-corrected visual acuity (BCVA) was tested monocularly with a logarithmically scaled letter acuity test from 4 years of age to late teens. Other registered postoperative data were presence of manifest nystagmus, strabismus and complications. RESULTS: The BCVA of the better-seeing eye reached a plateau at 7 years of age. Age at surgery of 80 days or less characterized the majority of cases with a logMAR ≤ 0.3 with a threshold effect between 80 and 130 days of age. Compared to normal children in Sweden, the long-term visual outcome showed a deficit of 0.5-0.6 logMAR. CONCLUSION: In spite of optimized care and surgery before 9 months, the BCVA was subnormal in our population compared to healthy children. The long-term visual outcome can be predicted at 7 years of age. Screening with early detection followed by surgery before the end of the third month is important to decrease the risk of marked acuity loss.

Longitudinal change in aphakic refraction after early surgery for congenital cataract.

PURPOSE: To characterize the longitudinal changes of refraction in aphakic eyes after early surgery for congenital cataract and to evaluate longitudinally measured aphakic refraction (individual vs group mean) as a noninvasive indicator of postoperative disturbances in ocular development. METHODS: Records of children who had cataract surgery during their first year of life between 1980 and 1995 were obtained from a prospective, population-based study of congenital cataract. Only children with regular follow-up were included. Postoperative aphakic refraction was calculated at the corneal plane. Data were obtained up to 36 months of age. RESULTS: The study included 28 children (49 eyes) who underwent surgery at a median age of 2.8 months (range, 0-9 months). The decrease of aphakic refraction at the corneal plane followed a logarithmic trend (R(2) = 0.95). A total of 36 eyes followed this pattern, with no growth in 8 eyes and an increased growth rate in 1 eye with uncontrolled glaucoma and 4 eyes of 2 children with Down syndrome. CONCLUSIONS: Most aphakic eyes follow a predictable, logarithmic change in refraction in the first 3 years of life, Longitudinal monitoring of refraction may prove to be a useful, noninvasive screening method for early detection of disturbances in aphakic eye growth.