Original articleA population-based study of congenital diaphragmatic hernia outcome in New South Wales and the Australian Capital Territory, Australia, 1992-2001
Section snippets
Methods
Data were obtained from the Neonatal Intensive Care Units Study (NICUS) data collection for a population-based study from 1992 to 2001 inclusive. The NICUS was set up as a regional audit tool to collect prospective data from the 10 neonatal intensive care units (NICUs) in NSW and the ACT [3]. Four of these units also perform major neonatal surgery [4]. Criteria for inclusion in the NICUS database are admission to a NICU and any of the following: gestation of less than 32 weeks at birth, birth
Results
There were 919,182 births in NSW and the ACT between 1992 and 2001. Table 1 indicates the pregnancy outcome data for infants with CDH. Of 242 infants identified with CDH, 8% underwent termination of pregnancy, 10% were stillborn after 20 weeks' gestation, and 82% were liveborn. The incidence of CDH was 1 in 3800 births.
Most liveborn infants with CDH (70%) were born at term with a normal birth weight and were male (Table 2). Fifty-six infants (28%) had another defect, most commonly cardiac. Most
Discussion
This population-based study from more than 900,000 births has identified a CDH incidence of 1 in 3800 births. We sourced several validated databases that collected audited, prospective, and regional information. This incidence is in keeping with previously published estimates [1], [9], [10].
We had embarked on this study primarily to identify the survival rates of infants with CDH to enable us to compare these findings with international results. Several North American and Canadian single
Conclusions
The incidence of CDH in NSW and the ACT, Australia, is 1 in 3800 births. Liveborn term infants have a 64% survival rate compared with 35% for preterm infants. A low 5-minute Apgar score, prematurity, and developing an air leak are significantly associated with mortality. Twenty-eight percent of liveborn term infants in a NICU did not survive to reach surgical repair. Unfortunately, there will be a group of infants with CDH who have overwhelming pulmonary hypoplasia, pulmonary hypertension, or
Acknowledgments
The authors thank Dr Lee Taylor, NSW Birth Defects Register, and Ms Barbara Bajuk, NICUS Coordinator.
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