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Published on: February 5, 2021
Evidence-based management of infants with congenital diaphragmatic hernia
Fernando R Moya1, Kevin P Lally
1Department of Pediatrics, Coastal Area Health Education Center, Wilmington, NC 28402-9025, USA. fernando.moya@coastalahec.org
Insights
Congenital diaphragmatic hernia (CDH) outcomes improve with expert centers, delayed surgery, and careful ventilation. More research is needed to optimize treatment for this serious infant condition.
Area of Science:
- Pediatric Surgery
- Neonatology
- Critical Care Medicine
Background:
- Congenital diaphragmatic hernia (CDH) presents a high and variable mortality rate.
- Current therapeutic advancements lack robust evidence from controlled studies.
- Limited randomized trials (9 trials, ~250 infants) have been published in the last 10-15 years.
Purpose of the Study:
- To review current evidence on congenital diaphragmatic hernia (CDH) management.
- To identify factors associated with improved outcomes in CDH patients.
- To highlight the need for further research and collaborative networks.
Main Methods:
- Literature review of randomized trials and therapeutic modalities for CDH.
- Analysis of factors influencing survival and outcomes in infants with CDH.
- Assessment of the current evidence base for CDH treatments.
Main Results:
- Infants with CDH benefit from delivery at experienced centers.
- Delayed surgical repair until hemodynamic and respiratory stability is achieved is recommended.
- Nonaggressive mechanical ventilation and permissive hypercapnia appear beneficial.
Conclusions:
- Experienced centers, delayed surgical repair, and judicious ventilation improve CDH outcomes.
- Specialized therapies like HFOV, iNO, and ECMO may benefit select infants.
- Establishing networks for large-scale randomized trials is crucial for advancing CDH care.
Abstract:
The mortality rate associated with congenital diaphragmatic hernia (CDH) varies widely between centers and remains relatively high despite widespread use of new therapeutic modalities. Many of these have been implemented without properly controlled studies. Over the past 10 to 15 years, only 9 randomized trials enrolling a total of approximately 250 infants with CDH have been published. The limited evidence available suggests that better outcomes are observed by delivering infants with CDH at experienced centers, by delaying surgical repair until hemodynamic and respiratory stability is achieved, and by the judicious utilization of nonaggressive mechanical ventilation and permissive hypercapnea. Other therapeutic modalities, such as high frequency oscillatory ventilation, inhaled nitric oxide, and ECMO, may provide additional advantages for selected infants. There is a dire need to establish networks of centers that manage enough infants with CDH, to conduct appropriately sized randomized trials that can answer some of the critical questions about the management and long-term outcome of these infants.
