Five‐year survival of infants with major congenital anomalies: a registry based study

Abstract

Aim: To determine survival of infants with major congenital anomalies (CA) and assess the effect of co-existing anomalies and gestational age.Methods: All liveborn infants with major CA born in New South Wales (NSW), Australia, 2004Australia, -2009 were identified from the NSW Register of Congenital Conditions. Deaths were identified via record linkage to death registrations and five-year survival was estimated using Kaplan-Meier methods.Results: There were 8521 liveborn infants with CA of whom 617 (7.2%) died within the first five years of life. Half of deaths occurred in the first week of life. The overall five-year survival rate was 92.8% (95%CI: 92.2-93.3) and 83.2% (95%CI: 79.0-87.4) for syndromes, 83.4% (95%CI: 80.9-85.9) for multiple, 85.1% (95%CI: 82.6-87.5) for chromosomal, 95.3% (95%CI: 94.8-95.8) for isolated and 96.2% (95%CI: 94.3-98.1) for non-Q chapter anomalies. Five-year survival for chromosomal, syndromes and subgroups was higher for isolated compared with multiple anomalies ranging from 77.5% to 98.9% and 68.6% to 89.5%, respectively. Survival was lower for preterm (79.4%; 95%CI: 77.5-81.4) than for term infants (95.8%; 95%CI: 95.3-96.3). Conclusion:Nine in ten infants with major CA survive up to five years, although there is variability in survival across CA groups. Survival of infants with major congenital anomalies has improved in recent years.Abbreviations CA, Congenital anomalies; CI, Confidence interval; EUROCAT, European surveillance of congenital anomalies; NSW, New South Wales; RoCC, Register of congenital conditions. Key notesThis study suggests that nine in ten infants with major congenital anomalies survive past five years of age.Five-year survival ranged from 83% for infants with syndromes to more than 95% for infants with isolated and non-Q chapter anomalies (e.g. congenital hypothyroidism, cystic fibrosis) Five-year survival was higher for children with isolated than those with multiple anomalies, while survival rates were lower for preterm than term infants.2008

Long-term survival of children born with congenital anomalies: A systematic review and meta-analysis of population-based studies

“…Table 1 provides the description of 55 studies included in this review. Further detail on the sources of case ascertainment and death identification and the description of the comparison group, if any, are given in S4 Table. Nine studies analysed long-term survival of all congenital anomalies combined: seven with [6,8,15,17,[40][41][42] and two without [7,43] stratification by congenital anomaly group/subtype (Table 1). Other studies (n = 46) focused on specific groups or subtypes of congenital anomalies: the central nervous system (n = 5 [44][45][46][47][48][49]), including spina bifida [44][45][46]48,49] and encephaloele [44,47]; orofacial clefts (n = 1 [16]); anomalies of the digestive system (n = 22), including oesophageal atresia [9,50,51], anorectal malformations [52], congenital diaphragmatic hernia (CDH) [18,23,51,53,54], biliary atresia [36][37][38][39][55][56][57][58][59]…”

“…Survival in live births with anencephaly analysed by four studies was extremely low and varied from 0% [15,42] to 7.3% [40] by year 1 ( Table 2). Seven studies of survival in children born with spina bifida [6,15,[40][41][42]45,48] including 7,422 live births were summarised in a meta-analysis, with pooled survival estimates of 92%, 91%, 89%, and 88% at ages 5, 10, 20, and 25 years predicted for children born in 2020 (Table 3). Survival has improved significantly over time, with an increased OR per 10-year increase in birth year 1.34 (95% CI 1.24-1.46, p < 0.001) ( Table 3 and Fig 2).…”

“…The pooled survival estimates predicted for infants born in 2020 were 93%, 93%, 92%, and 92% at ages 5, 10, 20, and 25 years, respectively (Table 3 and Fig 3). The survival estimates for children with anorectal malformations and for those with Hirschsprung disease were reported in four [15,40,41,52] and three studies [15,24,42] with survival ranging between 86% and 97% and between 93% and 98%, respectively ( Table 2).…”

Highlights in this issue

“…The survival of infants with major congenital anomalies (CA) has improved in recent years. An Australian register‐based study by Schneuer et al showed that nine in 10 infants with major CA survived past 5 years of age, although survival rates varied across CA groups. Five‐year survival was higher for children with isolated anomalies than multiple anomalies and lower for preterm than term‐born infants.…”

Asphyxia and the Occurrences of Perinatal Death in Anutapura General Hospital of Palu in 2018

“…Babies born with congenital abnormalities, generally, will be born as Low Birth Weight (LBW) Babies or small babies for their pregnancy age. Low Birth Weight Babies with congenital abnormalities that have a weight of about 20% die in the first week of life [18].…”