Temporal and geographical variations in survival of children born with congenital anomalies in Europe: A multi‐registry cohort study

Abstract

Background: Congenital anomalies are a major cause of perinatal, neonatal and infant mortality. Objectives: The aim was to investigate temporal changes and geographical variation in survival of children with major congenital anomalies (CA) in different European areas. Methods: In this population-based linkage cohort study, 17 CA registries members of EUROCAT, the European network for the surveillance of CAs, successfully linked data on 115,219 live births with CAs to mortality records. Registries estimated Kaplan-Meier survival at 28 days and 5 years of age and fitted Cox's proportional hazards models comparing mortality at 1 year and 1-9 years of age for children born during 2005-2014 with those born during 1995-2004. The hazard ratios (HR) from each registry were combined centrally using a random-effects model. The 5-year survival conditional on having survived to 28 days of age was calculated. Results: The overall risk of death by 1 year of age for children born with any major CA in 2005-2014 decreased compared to 1995-2004 (HR 0.68, 95% confidence interval [CI] 0.53, 0.89). Survival at 5 years of age ranged between registries from 97.6%to 87.0%. The lowest survival was observed for the registry of OMNI-Net (Ukraine)

European study showed that children with congenital anomalies often underwent multiple surgical procedures at different ages across Europe

“…The percentage of the children that had surgery was generally higher in Wales and the three English regions and lower in Finland. A possible explanation may be that the Finnish EUROCAT registry includes more children with less severe anomalies than the English EUROCAT registries 15 . Another possibility is that we may not have had the complete list of surgery codes for Finland as the surgical procedures were identified using three‐digit codes rather than more specific codes 15 .…”

“…A possible explanation may be that the Finnish EUROCAT registry includes more children with less severe anomalies than the English EUROCAT registries 15 . Another possibility is that we may not have had the complete list of surgery codes for Finland as the surgical procedures were identified using three‐digit codes rather than more specific codes 15 . It is well‐known that there are differences in the prevalence of congenital anomalies requiring surgery across Europe with higher rates of gastroschisis and neural tube defects in England and Wales 16,17 .…”

Ten-year survival of children with trisomy 13 or trisomy 18: a multi-registry European cohort study

“…6 Survival of children with major CAs beyond one year has substantially improved during the last few decades due to advances in neonatal care and surgical interventions. 7,8 As shown in our recent multi-centre European study, 10year survival exceeded 90% for most major structural anomalies and the commonest chromosomal anomaly, Down syndrome (trisomy 21). 9 Trisomy 13 (T13) (Patau syndrome) and trisomy 18 (T18) (Edwards syndrome) are the most common autosomal trisomies after Down syndrome and are characterised by multiple structural anomalies and intellectual disability in survivors.…”

“…They are also a growing contributor to mortality of children under 5 years of age5 and of older children 6. Survival of children with major CAs beyond 1 year has substantially improved during the last few decades due to advances in neonatal care and surgical interventions 7 8. As shown in our recent multi-centre European study, 10-year survival exceeded 90% for most major structural anomalies and the most common chromosomal anomaly, Down syndrome (trisomy 21) 9.…”

Survival, hospitalisation and surgery in children born with Pierre Robin sequence: a European population-based cohort study

“…For the investigation of survival rates, data on mortality were obtained through linkage with vital statistics and mortality databases. A detailed description of the methods has been published elsewhere 14–16…”