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Congenital diaphragmatic hernia
1Dept of Child Health, Cardiff University School of Medicine, Heath Park, Cardiff CF14 4XN, UK. kotechas@cardiff.ac.uk
Insights
Congenital diaphragmatic hernia (CDH) in infants leads to high mortality and morbidity. Expert recommendations focus on optimizing antenatal and postnatal care, including early diagnosis and tailored ventilation, to improve survival rates for this complex condition.
Area of Science:
- Pediatric Surgery
- Neonatology
- Medical Genetics
Background:
- Congenital diaphragmatic hernia (CDH) presents significant mortality (30-40%) and long-term morbidity in newborns.
- Incidence rates of CDH range from 1.7 to 5.7 per 10,000 live births, varying by study population.
- Current management strategies aim to improve outcomes, with higher survival rates observed in high-volume centers.
Framework:
- Expert task force convened to evaluate current literature and formulate recommendations for CDH management.
- Recommendations cover both antenatal and postnatal care strategies.
- Focus on evidence-based guidelines to standardize and improve patient care.
Implementation:
- Antenatal diagnosis via ultrasound and MRI is standard; antenatal interventions require further evaluation.
- Postnatal management emphasizes immediate intubation, gentle ventilation, and management of pulmonary hypertension.
- Surgical intervention is typically delayed until optimal medical stabilization is achieved.
Implications:
- Optimizing management protocols, including ventilation and pulmonary hypertension treatment, is crucial for improving CDH patient outcomes.
- Multidisciplinary follow-up is essential for managing comorbidities in CDH survivors.
- Multicenter international trials are necessary to further refine and validate optimal antenatal and postnatal management strategies for CDH.
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.
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