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Long-term maturation of congenital diaphragmatic hernia treatment results: toward development of a severity-specific
David W Kays1, Saleem Islam, Shawn D Larson
1From the Department of Surgery, University of Florida, Gainesville, FL; and UF Health Shands Children's Hospital, Gainesville, FL.
Insights
Timing of congenital diaphragmatic hernia (CDH) repair impacts survival. Less severe CDH cases benefit from delayed surgery, while severe cases may need earlier intervention for better outcomes.
Area of Science:
- Pediatric surgery
- Neonatal care
- Congenital abnormalities
Background:
- Previous research in 1999 highlighted improved survival in congenital diaphragmatic hernia (CDH) patients through lung protective ventilation strategies.
- A consistent management and ventilator strategy was maintained throughout the study period.
Purpose of the Study:
- To evaluate how different congenital diaphragmatic hernia (CDH) repair timings affect patient survival and the need for extracorporeal membrane oxygenation (ECMO).
- To analyze these outcomes while accounting for anatomic and physiological disease severity in a large cohort.
Main Methods:
- Retrospective review of 268 consecutive congenital diaphragmatic hernia (CDH) patients, including 208 new cases and 60 previously reported.
- Analysis of surgical timing variations applied as the patient series progressed.
Main Results:
- Patients with less severe left liver-down CDH showed increased ECMO need when repaired within 48 hours.
- More severe left liver-up CDH patients had higher survival rates when repaired before ECMO.
- Overall survival was 78%, with 88% survival for those without lethal anomalies. Specific survival rates were 99% for left liver-down, 91% for right CDH, and 76% for left liver-up CDH.
Conclusions:
- Congenital diaphragmatic hernia (CDH) repair timing should be individualized based on anatomic severity.
- Less severe CDH cases may benefit from delayed surgical intervention.
- More severe CDH cases might require a more aggressive surgical approach for improved survival.
Objectives:
To assess the impact of varying approaches to congenital diaphragmatic hernia (CDH) repair timing on survival and need for ECMO when controlled for anatomic and physiologic disease severity in a large consecutive series of patients with CDH.
Background:
Our publication of 60 consecutive patients with CDH in 1999 showed that survival was significantly improved by limiting lung inflation pressures and eliminating hyperventilation.
Methods:
We retrospectively reviewed 268 consecutive patients with CDH, combining 208 new patients with the 60 previously reported. Management and ventilator strategy were highly consistent throughout. Varying approaches to surgical timing were applied as the series matured.
Results:
Patients with anatomically less severe left liver-down CDH had significantly increased need for ECMO if repaired in the first 48 hours, whereas patients with more severe left liver-up CDH survived at a higher rate when repair was performed before ECMO. Overall survival of 268 patients was 78%. Survival was 88% for those without lethal associated anomalies. Of these, 99% of left liver-down CDH survived, 91% of right CDH survived, and 76% of left liver-up CDH survived.
Conclusions:
This study shows that patients with anatomically less severe CDH benefit from delayed surgery whereas patients with anatomically more severe CDH may benefit from a more aggressive surgical approach. These findings show that patients respond differently across the CDH anatomic severity spectrum and lay the foundation for the development of risk-specific treatment protocols for patients with CDH.
