Congenital diaphragmatic hernia

Abstract

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.

Congenital diaphragmatic hernia (CDH) is an uncommon congenital malformation of the lung, but one with important implications for diagnosis, management and prognosis. It is associated with high mortality and although improvements in medical and surgical management have improved the outlook, survival remains at 60–70% [1–6]. Newer modalities, including antenatal screening and intervention, are available but are often introduced without formal evaluation. The current Task Force has convened experts in neonatal and paediatric respiratory medicine, paediatric pathology, fetal medicine, and paediatric surgery to evaluate the current literature and make recommendations for the management of CDH.