Before, during and after birth - how does care affect the future health prospects of infants with congenital diaphragmatic hernia? (UKOSS study)

Congenital diaphragmatic hernia (CDH) is a musculoskeletal defect of the diaphragm which occurs during fetal development affecting between 1 in 2,000 and 1 in 4,000 births. During fetal development the defect allows the abdominal contents to move into the chest and impedes cardiac and lung development. CDH can be diagnosed prenatally on ultrasound. At present we have incomplete information about the incidence, optimal pregnancy management, survival to delivery, optimal surgical management, survival to discharge and longer term outcomes. Given the rarity of CDH available data mainly come from major referral centres and may be biased in favour of better outcomes; available survival data following surgery are thus contentious. Nevertheless, available data suggest that a significant proportion of infants experience substantial ongoing problems of respiratory, neurological, growth and GI function, and life-long disability. This project is a UK-wide study of a 1-year birth cohort of all cases of CDH using three related tried and tested data collection system: the UK Obstetric Surveillance System (UKOSS) to collect antenatal and perinatal information; and BAPS-CASS to collect neonatal and surgical information, and outcomes at both discharge and age one year.

Two hundred and fifty six cases were reported up until March 2010 and data were returned for 234 cases (91%). Three cases did not meet the case definition criteria, there were 38 duplicate reports and 14 cases which were subsequently reported by clinicians as not cases. Thus, there are 179 confirmed cases in an estimated 794,300 total births. This represents an estimated incidence of 2.3 cases per 10,000 total births (95% CI 1.9 to 2.6).

NPEU Contact:
Marian Knight (
Chief Investigators:
Jenny Kurinczuk (
Last updated:
Friday, 05 February 2016 14:13