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Congenital Diaphragmatic Hernia (CDH): A Comparison of Regional Morbidity and Mortality Outcomes

Presented at the Neonatal Society 2014 Autumn Meeting.

Thomas B, Doherty C

Neonatal Unit, University Hospital of Wales, Cardiff, Wales

Background: Mortality in CDH remains high thus making benchmarking against both standard guidelines and outcomes from other centres important. The South West (SW) of England implemented a clinical pathway and have shown survival rates of 76% for CDH. We aimed to compare our outcomes for CDH in South Wales with the SW and to benchmark our clinical practice against both their clinical pathway and the recent European CDH guidelines (1).

Methods: All cases of CDH in Wales from 1998 to 2010 were identified through the CARIS congenital anomaly register. All live borns managed in South Wales had clinical management reviewed through retrospective case-note assessment. Outcomes from both the International CDH Study Group (2000-2009) and the SW anomaly register (1998-2010) were obtained. Clinical management of South Wales babies was benchmarked against the SW clinical pathway and the European CDH clinical guidelines (1).

Results: 176 cases of CDH of which 120 were live born were identified through CARIS. The survival rate for live borns (83) managed in South Wales was 63% (95% CI 0.53-0.73) compared to 69% (95% CI 0.68-0.70) and 76% (95% CI 0.67-0.85) in the CDH Study Group and the SW respectively. There was a difference in pulmonary morbidity with a higher rate of pneumothoraces in babies born in S Wales at 32% (95% CI0.22-0.42) compared with 13% in SW of England (95% CI 0.06-0.2). Oxygen dependency at 28 days of life in S Wales was 11% (95% CI 0.03-0.19) compared with 22% (95% CI 0.12-0.33) and 38% (95% CI 0.37-0.40) in the SW of England and CDH Study Group respectively. The main difference in the Neonatal Units’ management is the elective use of HFOV ventilation in the SW of England. 90% (95% CI 0.81-0.99) of their cases received HFOV, compared with 64% (95% CI 0.50-0.78) of cases in S Wales.

Conclusion: Survival rates over a 12 year period for CDH in South Wales are lower than the SW. The reasons may be multifactorial however optimising ventilation with permissive hypercapnia (1), may reduce pulmonary morbidity in survivors. More evidence is required to decide whether elective HFOV is associated with better outcomes.

Corresponding author: bethansara@doctors.org.uk

1. S. Kotecha et al. ERS Taskforce Report: Congenital Diaphragmatic Hernia. European Respiratory Journal; 38: 820-829

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