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Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study
Kim Heiwegen1, Arno Fj van Heijst2, Horst Daniels-Scharbatke3
1Department of Surgery, Division of Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Route 618, PO box 9101, 6500, Nijmegen, HB, Netherlands. Kim.Heiwegen@radboudumc.nl.
Insights
Congenital diaphragmatic eventration and hernia sacs, considered defects without a true hole, show better survival than true congenital diaphragmatic hernias. However, these conditions have higher recurrence rates and longer oxygen needs.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Neonatal Care
Background:
- Congenital diaphragmatic malformations, including congenital diaphragmatic eventration (CDE) and congenital diaphragmatic hernia (CDH), are complex conditions.
- CDE and CDH with hernia sac are often managed similarly to true CDH, despite differing pathologies.
- Outcomes for patients with CDE and CDH with hernia sac are generally considered favorable but lack detailed quantitative data.
Purpose of the Study:
- To compare the clinical outcomes of surgically treated patients with CDE or Bochdalek type CDH.
- To differentiate outcomes based on the presence or absence of a "true defect" in the diaphragm.
- To evaluate survival rates, short-term outcomes, and recurrence in these patient groups.
Main Methods:
- Retrospective analysis of 200 surgically treated patients with CDE or Bochdalek type CDH between 2000 and 2016.
- Patients were categorized into two groups: those with a "true defect" and those without (eventration or hernia sac).
- Evaluation of demographics, CDH characteristics, treatment, and clinical outcomes, including survival and 30-day parameters.
Main Results:
- Patients without a true defect (eventration or hernia sac) demonstrated significantly higher 1-year survival (97%) compared to those with a true defect (76%).
- Despite better survival, patients without a true defect required oxygen supplementation more frequently at 30 days (46% vs. 26%) and had a higher recurrence rate (8% vs. 0%).
- CDH without a true defect showed better short-term outcomes than CDH with a true defect requiring patch repair, but with increased O2 dependency and recurrence.
Conclusions:
- Congenital diaphragmatic eventration and CDH with hernia sac (patients without a true defect) exhibit clinical outcomes more similar to each other than to CDH with a true defect.
- These "no true defect" groups have superior survival rates but are associated with increased recurrence and prolonged oxygen support needs.
- The findings highlight distinct management considerations and long-term prognoses for different types of congenital diaphragmatic malformations.
Abstract:
Congenital diaphragmatic eventration (CDE) and congenital diaphragmatic hernia (CDH) with or without hernia sac are three different types of congenital diaphragmatic malformations, which this study evaluates. All surgically treated patients with CDE or Bochdalek type CDH between 2000 and 2016 were included in this retrospective analysis. Demographics, CDH-characteristics, treatment, and clinical outcome were evaluated. In total, 200 patients were included. Patients with an eventration or hernia sac had no significant differences and were compared as patients without a true defect to patients with a true defect. The 1-year survival of patients with a true defect was significantly lower than patients with no true defect (76% versus 97%, p = 0.001). CDH with no true defect had significantly better short-term outcomes than CDH with true defect requiring patch repair. However, at 30 days, they more often required oxygen supplementation (46% versus 26%, p = 0.03) and had a higher recurrence rate (8% versus 0%, p = 0.006) (three eventration and two hernia sac patients). Conclusion: Patients without a true defect seem to have a more similar clinical outcome than CDH patients with a true defect, with a better survival. However, the recurrence rate and duration of oxygen supplementation at 30 days are higher than CDH patients with a true defect.What is Known:• Congenital diaphragmatic hernia with or without hernia sac and congenital diaphragmatic eventration (incomplete muscularization) are often treated similarly.• Patients with hernia sac and eventration are thought to have a relatively good outcome, but exact numbers are not described.What is New:• Congenital diaphragmatic eventration and patients with hernia sac seem to have a more similar clinical outcome than Bochdalek type CDH with a true defect.• Patients without a true defect (eventration or hernia sac) have a high recurrence rate.
