4.6 Article

Congenital diaphragmatic hernia

Journal

EUROPEAN RESPIRATORY JOURNAL
Volume 39, Issue 4, Pages 820-829

Publisher

EUROPEAN RESPIRATORY SOC JOURNALS LTD
DOI: 10.1183/09031936.00066511

Keywords

Antenatal ultrasound; congenital diaphragmatic hernia; congenital lung malformation

Funding

  1. NIHR Respiratory Disease Biomedical Research Unit (Royal Brompton and Harefield NHS and Imperial College London, London, UK)

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Infants with congenital diaphragmatic hernia (CDH) have significant mortality and long-term morbidity. Only 60-70% survive and usually those in high-volume centres. The current Task Force, therefore, has convened experts to evaluate the current literature and make recommendations on both the antenatal and post-natal management of CDH. The incidence of CDH varies from 1.7 to 5.7 per 10,000 live-born infants depending on the study population. Antenatal ultrasound scanning is routine and increasingly complemented by the use of magnetic resonance imaging. For isolated CDH, antenatal interventions should be considered, but the techniques need vigorous evaluation. After birth, management protocols are often used and have improved outcome in nonrandomised studies, but immediate intubation at birth and gentle ventilation are important. Pulmonary hypertension is common and its optimal management is crucial as its severity predicts the outcome. Usually, surgery is delayed to allow optimal medical stabilisation. The role of minimal invasive post-natal surgery remains to be further defined. There are differences in opinion about whether extracorporeal membrane oxygenation improves outcome. Survivors of CDH can have a high incidence of comorbidities; thus, multidisciplinary follow-up is recommended. Multicentre international trials are necessary to optimise the antenatal and post-natal management of CDH patients.

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