Articles20-year survival of children born with congenital anomalies: a population-based study
Introduction
Congenital anomalies are a leading cause of perinatal and infant mortality, especially in developed countries.1, 2 Advances in fetal and neonatal care have improved outcomes for individuals with some congenital anomalies.3, 4, 5 However, there remains a paucity of knowledge about survival for various specific groups and subtypes.
Most studies have reported survival in individuals with either Down's syndrome6, 7, 8, 9, 10, 11, 12 or spina bifida.8, 9, 13, 14, 15, 16 For other congenital anomaly groups and subtypes, there are few, or no, data for survival, particularly beyond the first year of life. Dastgiri7 and Agha13 and their colleagues tried to address this shortage by reporting survival over several years for various congenital anomaly groups. However, many of the reported groups included a range of disorders that are likely to vary widely in their lethality. Furthermore, mortality estimates can be substantially overestimated if subtypes are not reported in isolation.17, 18
We used data from the Northern Congenital Abnormality Survey (NorCAS), a population-based register of congenital anomalies within northern England, matched with local hospital and national mortality records, to estimate survival up to 20 years of age for a range of congenital anomaly groups and subtypes.
Section snippets
Study population
The former Northern Health Region of England (UK) is a geographically distinct area with a stable population of 3 million people and approximately 35 000 deliveries per year. NorCAS gathers data for congenital anomalies occurring within this population, whether arising in late miscarriages (fetal deaths at ≥20 weeks of gestation), terminations of pregnancy for fetal anomaly after prenatal diagnosis, stillbirths (fetal deaths delivered at ≥24 weeks of gestation, or ≥28 weeks before October,
Results
13 758 cases of congenital anomaly were notified to NorCAS between 1985 and 2003, equivalent to a prevalence of 20·8 per 1000 registered births (95% CI 20·4–21·1). Table 1 shows clinical outcomes for congenital anomaly groups. Outcomes for subtypes are shown in webappendix pp 1–2. There were 124 (0·9%) late miscarriages, 2249 (16·3%) terminations of pregnancy for fetal anomaly after prenatal diagnosis, 421 (3·1%) stillbirths, and 10 964 (79·7%) livebirths. The proportion of terminations
Discussion
This study estimated survival for several congenital anomaly groups and subtypes, including many for which there are limited published data, particularly beyond 1 year of age. 20-year survival rates ranged from 66·2% in individuals born with nervous system anomalies to 97·6% in those born with orofacial clefts. Survival varied substantially by congenital anomaly subtype. The proportion of terminations of pregnancy for fetal anomaly increased throughout the study period (from 12·4% in 1985 to
References (41)
- et al.
Intrapartum treatment of acute chorioamnionitis: impact on neonatal sepsis
Am J Obstet Gynecol
(1988) - et al.
Infant mortality and congenital anomalies from 1950 to 1994: an international perspective
J Epidemiol Community Health
(2000) Five-decade international trends in the relation of perinatal mortality and congenital malformations: stillbirth and neonatal death compared
Int J Epidemiol
(1991)- et al.
Mortality associated with congenital heart defects in the United States: trends and racial disparities, 1979–1997
Circulation
(2001) - et al.
The changing survival profile of people with Down's syndrome: implications for genetic counselling
Clin Genet
(2002) - et al.
Recent advances in congenital diaphragmatic hernia
Arch Dis Child
(2005) - et al.
Life tables for Down syndrome
Hum Genet
(1989) - et al.
Survival of children born with congenital anomalies
Arch Dis Child
(2003) - et al.
First-year mortality rates for selected birth defects, Hawaii, 1986–1999
Am J Med Genet
(2003) - et al.
Patterns of first-year survival among infants with selected congenital anomalies in Texas, 1995–1997
Teratology
(2001)
Survival in infants with Down syndrome, Metropolitan Atlanta, 1979–1998
J Pediatr
Ten-year survival of Down syndrome births
Int J Epidemiol
Survival of infants born with Down's syndrome: 1980–96
Paediatr Perinat Epidemiol
Determinants of survival in children with congenital abnormalities: a long-term population-based cohort study
Birth Defects Res A Clin Mol Teratol
Survival and handicap of infants with spina bifida
Arch Dis Child
Spina bifida and hydrocephalus: a population study over a 35-year period
Am J Hum Genet
Survival of infants with spina bifida: a population study, 1979–94
Paediatr Perinat Epidemiol
First year-of-life mortality among infants with oral clefts: New York State, 1983–1990
Cleft Palate Craniofac J
The influence of congenital heart disease on survival of infants with oesophageal atresia
Arch Dis Child Fetal Neonatal Ed
A population-based study of the prenatal diagnosis of congenital malformation over 16 years
BJOG
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