Search

Objectives: To compare 5-year survival rate and morbidity in children with spina bifida, transposition of great arteries (TGA), congenital diaphragmatic hernia (CDH) or gastroschisis diagnosed prenatally with those diagnosed postnatally. Methods: Population-based registers' data were linked to hospital and mortality databases. Results: Children whose anomaly was diagnosed prenatally (n = 1088) had a lower mean gestational age than those diagnosed postnatally (n = 1698) ranging from 8 days for CDH to 4 days for TGA. Children with CDH had the highest infant mortality rate with a significant difference (p < 0.001) between those prenatally (359/1,000 births) and postnatally (116/1,000) diagnosed. For all four anomalies, the median length of hospital stay was significantly greater in children with a prenatal diagnosis than those postnatally diagnosed. Children with prenatally diagnosed spina bifida (79% vs 60%; p = 0.002) were more likely to have surgery in the first week of life, with an indication that this also occurred in children with CDH (79% vs 69%; p = 0.06). Conclusions: Our findings do not show improved outcomes for prenatally diagnosed infants. For conditions where prenatal diagnoses were associated with greater mortality and morbidity, the findings might be attributed to increased detection of more severe anomalies. The increased mortality and morbidity in those diagnosed prenatally may be related to the lower mean gestational age (GA) at birth, leading to insufficient surfactant for respiratory effort. This is especially important for these four groups of children as they have to undergo anaesthesia and surgery shortly after birth. Appropriate prenatal counselling about the time and mode of delivery is needed. Significance: What is already known on this subject?: Prenatal detection of congenital anomalies has become more common with improvements in technology and increased accuracy of detection. Previous findings on the effect of timing of diagnoses on newborn outcomes are limited and conflicting. What this study adds?: Our findings do not show better outcomes for prenatally diagnosed infants with spina bifida, transposition of great arteries, congenital diaphragmatic hernia or gastroschisis. More severe anomalies may be more likely to be prenatally detected. Appropriate prenatal counselling about the time and mode of delivery is needed. [ABSTRACT FROM AUTHOR]

Linking routinely collected healthcare administrative data is a valuable method for conducting research on morbidity outcomes, but linkage quality and accuracy needs to be assessed for bias as the data were not collected for research. The aim of this study was to describe the rates of linking data on children with and without congenital anomalies to regional or national hospital discharge databases and to evaluate the quality of the matched data. Eleven population-based EUROCAT registries participated in a EUROlinkCAT study linking data on children with a congenital anomaly and children without congenital anomalies (reference children) born between 1995 and 2014 to administrative databases including hospital discharge records. Odds ratios (OR), adjusted by region, were estimated to assess the association of maternal and child characteristics on the likelihood of being matched. Data on 102,654 children with congenital anomalies were extracted from 11 EUROCAT registries and 2,199,379 reference children from birth registers in seven regions. Overall, 97% of children with congenital anomalies and 95% of reference children were successfully matched to administrative databases. Information on maternal age, multiple birth status, sex, gestational age and birthweight were >95% complete in the linked datasets for most regions. Compared with children born at term, those born at ≤27 weeks and 28–31 weeks were less likely to be matched (adjusted OR 0.23, 95% CI 0.21–0.25 and adjusted OR 0.75, 95% CI 0.70–0.81 respectively). For children born 32–36 weeks, those with congenital anomalies were less likely to be matched (adjusted OR 0.78, 95% CI 0.71–0.85) while reference children were more likely to be matched (adjusted OR 1.28, 95% CI 1.24–1.32). Children born to teenage mothers and mothers ≥35 years were less likely to be matched compared with mothers aged 20–34 years (adjusted ORs 0.92, 95% CI 0.88–0.96; and 0.87, 95% CI 0.86–0.89 respectively). The accuracy of linkage and the quality of the matched data suggest that these data are suitable for researching morbidity outcomes in most regions/countries. However, children born preterm and those born to mothers aged <20 and ≥35 years are less likely to be matched. While linkage to administrative databases enables identification of a reference group and long-term outcomes to be investigated, efforts are needed to improve linkages to population groups that are less likely to be linked. [ABSTRACT FROM AUTHOR]

Aim: Children with congenital anomalies often require surgery but data on the burden of surgery for these children are limited. Methods: A population‐based record‐linkage study in Finland, Wales and regions of Denmark, England, Italy and Spain. A total of 91 504 children with congenital anomalies born in 1995–2014 were followed to their tenth birthday or the end of 2015. Electronic linkage to hospital databases provided data on inpatient surgical procedures and meta‐analyses of surgical procedures were performed by age groups. Results: The percentage of children having surgery in the first year was 38% with some differences across regions and 14% also underwent surgery at age 1–4 years. Regional differences in age at the time of their first surgical procedure were observed for children with cleft palate, hydronephrosis, hypospadias, clubfoot and craniosynostosis. The children had a median of 2.0 (95% CI 1.98, 2.02) surgical procedures before age 5 years with children with oesophageal atresia having the highest median number of procedures (4.5; 95% CI 3.3, 5.8). Conclusion: A third of children with congenital anomalies required surgery during infancy and often more than one procedure was needed before age 5 years. There was no European consensus on the preferred age for surgery for some anomalies. [ABSTRACT FROM AUTHOR]

Are children with major congenital anomalies more likely to develop diabetes requiring insulin therapy, as indicated by prescriptions for insulin, than children without congenital anomalies? The aim of this study is to evaluate prescription rates of insulin/insulin analogues in children aged 0–9 years with and without major congenital anomalies. A EUROlinkCAT data linkage cohort study, involving six population-based congenital anomaly registries in five countries. Data on children with major congenital anomalies (60,662) and children without congenital anomalies (1,722,912), the reference group, were linked to prescription records. Birth cohort and gestational age were examined. The mean follow-up for all children was 6.2 years. In children with congenital anomalies aged 0–3 years, 0.04 per 100 child-years (95% CIs 0.01–0.07) had > 1 prescription for insulin/insulin analogues compared with 0.03 (95% CIs 0.01–0.06) in reference children, increasing ten-fold by age 8–9 years. The risk of > 1 prescription for insulin/insulin analogues aged 0–9 years in children with non-chromosomal anomalies (RR 0.92, 95% CI 0.84–1.00) was similar to that of reference children. However, children with chromosomal anomalies (RR 2.37, 95% CI 1.91–2.96), and specifically children with Down syndrome (RR 3.44, 95% CIs 2.70–4.37), Down syndrome with congenital heart defects (RR 3.86, 95% CIs 2.88–5.16) and Down syndrome without congenital heart defects (RR 2.78, 95% CIs 1.82–4.27), had a significantly increased risk of > 1 prescription for insulin/insulin analogues aged 0–9 years compared to reference children. Female children had a reduced risk of > 1 prescription aged 0–9 years compared with male children (RR 0.76, 95% CI 0.64–0.90 for children with congenital anomalies and RR 0.90, 95% CI 0.87–0.93 for reference children). Children without congenital anomalies born preterm (< 37 weeks) were more likely to have > 1 insulin/insulin analogue prescription compared to term births (RR 1.28, 95% CIs 1.20–1.36). Conclusion: This is the first population-based study using a standardised methodology across multiple countries. Males, children without congenital anomalies born preterm and those with chromosomal anomalies had an increased risk of being prescribed insulin/insulin analogues. These results will help clinicians to identify which congenital anomalies are associated with an increased risk of developing diabetes requiring insulin therapy and allow them to reassure families of children who have non-chromosomal anomalies that their risk is similar to that of the general population. What is Known: • Children and young adults with Down syndrome have an increased risk of diabetes requiring insulin therapy. • Children born prematurely have an increased risk of developing diabetes requiring insulin therapy. What is New: • Children with non-chromosomal anomalies do not have an increased risk of developing diabetes requiring insulin therapy compared to children without congenital anomalies. • Female children, with or without major congenital anomalies, are less likely to develop diabetes requiring insulin therapy before the age of 10 compared to male children. [ABSTRACT FROM AUTHOR]

Background: Congenital anomalies are a leading cause of childhood morbidity, but little is known about the long-term outcomes. Objective: To quantify the burden of disease in childhood for children with congenital anomalies by assessing the risk of hospitalisation, the number of days spent in hospital and proportion of children with extended stays (≥10 days). Methods: European population-based record-linkage study in 11 regions in eight countries including children with congenital anomalies (EUROCAT children) and without congenital anomalies (reference children) living in the same regions. The children were born between 1995 and 2014 and were followed to their tenth birthday or 31/12/2015. European meta-analyses of the outcome measures were performed by two age groups, <1 year and 1–4 years. Results: 99,416 EUROCAT children and 2,021,772 reference children were linked to hospital databases. Among EUROCAT children, 85% (95%-CI: 79–90%) were hospitalised in the first year and 56% (95%-CI: 51–61%) at ages 1–4 years, compared to 31% (95%-CI: 26–37%) and 25% (95%-CI: 19–31%) of the reference children. Median length of stay was 2–3 times longer for EUROCAT children in both age groups. The percentages of children with extended stays (≥10 days) in the first year were 24% (95%-CI: 20–29%) for EUROCAT children and 1% (95%-CI: 1–2%) for reference children. The median length of stay varied greatly between congenital anomaly subgroups, with children with gastrointestinal anomalies and congenital heart defects having the longest stays. Conclusions: Children with congenital anomalies were more frequently hospitalised and median length of stay was longer. The outlook improves after the first year. Parents of children with congenital anomalies should be informed about the increased hospitalisations required for their child's care and the impact on family life and siblings, and they should be adequately supported. [ABSTRACT FROM AUTHOR]