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Antenatal management of congenital diaphragmatic hernia today and tomorrow
Lennart van der Veeken1,2, Francesca M Russo1,2, Johannes van der Merwe1,2
1Academic Department of Development and Regeneration, Cluster Woman and Child, Biomedical Sciences, KU Leuven, Leuven, Belgium.
Insights
Congenital diaphragmatic hernia (CDH) is a birth defect impairing fetal lung growth. Prenatal interventions like fetoscopic endoluminal tracheal occlusion show promise for improving outcomes in severe CDH cases.
Area of Science:
- Perinatal Medicine
- Fetal Surgery
- Neonatology
Background:
- Congenital diaphragmatic hernia (CDH) is a rare birth defect with significant fetal lung development impairment.
- This condition leads to high mortality (30%) and morbidity due to underdeveloped lungs.
- Prenatal diagnosis and intervention are crucial for improving infant outcomes.
Purpose of the Study:
- To review current and emerging prenatal therapies for congenital diaphragmatic hernia.
- To highlight the potential of fetoscopic endoluminal tracheal occlusion (FETO) in managing severe CDH.
- To explore novel therapeutic strategies targeting pulmonary hypertension and lung growth.
Main Methods:
- Review of existing literature on CDH management.
- Analysis of outcomes from fetoscopic endoluminal tracheal occlusion (FETO) clinical trials.
- Discussion of preclinical and early-phase clinical investigations of new therapies.
Main Results:
- Prenatal medical imaging accurately predicts outcomes in isolated CDH cases.
- Fetoscopic endoluminal tracheal occlusion (FETO) is effective in triggering lung growth and is under global evaluation.
- Right-sided CDH cases with poor prognosis are clinically offered FETO.
Conclusions:
- Fetoscopic endoluminal tracheal occlusion (FETO) represents a significant advancement in prenatal CDH management.
- Ongoing research focuses on less invasive therapies, including prenatal sildenafil administration, to address persistent pulmonary hypertension.
- A multi-modal approach combining fetal surgery and pharmacotherapy may offer improved survival and reduced morbidity for CDH patients.
Abstract:
Congenital diaphragmatic hernia is rare birth defect, which can be easily corrected after birth. The main problem is that herniation of viscera during fetal life impairs lung development, leading to a 30% mortality and significant morbidity. In isolated cases the outcome can be accurately predicted prenatally by medical imaging. Cases with a poor prognosis can be treated before birth; clinically this is by fetoscopic endoluminal tracheal occlusion. Obstruction of the airways triggers lung growth. This procedure is currently being evaluated in a global clinical trial for left sided cases; right sided cases with poor prognosis are offered the procedure clinically. The search for more potent and less invasive therapies continues. Prenatal transplacental sildenafil administration will in due course be tried clinically, with the aim to reduce the occurrence of persistent pulmonary hypertension, either alone or in combination with fetal surgery. Other medical approaches are in an earlier translational phase.
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