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Disability and capability
Twenty-year survival of children born with congenital anomalies: a population-based study
  1. P. Tennant1,
  2. M. Pearce1,
  3. M. Bythell2,
  4. J. Rankin1,2
  1. 1
    Institute of Health and Society, Newcastle University, Newcastle upon Tyne, UK
  2. 2
    Regional Maternity Survey Office, Newcastle upon Tyne, UK

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    Objective

    To estimate the survival, up to age 20 years, for a range of congenital anomaly groups and subtypes.

    Design

    Population-based registry (Northern Congenital Abnormality Survey, NorCAS).

    Setting

    The former Northern Region of England (the area extending from North Cumbria to the Tees area and up to the Scottish border), a geographically distinct area with a stable population of three million, and approximately 35 000 deliveries per year.

    Participants

    13 738 individuals with at least one congenital anomaly (classified according to the European Surveillance of Congenital Anomalies, EUROCAT, guidelines) born between 01 January 1985 and 31 December 2003, of whom 10 951 were live born and 10 837 (99%) were traced for their survival status on 28 January 2008.

    Main Outcome Measures

    Congenital anomaly group and subtype specific survival rates at one week, four weeks, one year, five years, 10 years, 15 years and 20 years. Change in survival, and the number of terminations of pregnancy for fetal anomaly, with time.

    Results

    Survival among children with at least one congenital anomaly was 88.8% (95% CI 88.2 to 89.4) and 85.5 (95% CI 84.8 to 86.3) at one year and 20 years respectively. Twenty year survival among the most common anomaly groups was 89.5% (95% CI 88.4 to 90.6) for cardiovascular, 79.1% (95% CI 76.7 to 81.3) for chromosomal, 93.2% (95% CI 91.6 to 94.5) for urinary, 83.1% (95% CI 79.6 to 86.0) for digestive system, 97.7% (95% CI 96.1 to 98.7) for orofacial clefts, and 66.2% (95% CI 61.5 to 70.4) for nervous system anomalies. Survival varied considerably between subtypes, even within the same anomaly group. The proportion of terminations for fetal anomaly increased throughout the study period, and, together with year of birth, was an independent predictor of survival.

    Conclusions

    This study presents robust estimates of survival, uniquely to age 20 years, for a range of congenital anomaly groups and subtypes, some of which have been infrequently reported previously. This information will be valuable for families, genetic councillors and other health care professionals when a congenital anomaly is detected, and will assist in planning for the future care needs of affected individuals.

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