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Bilateral congenital diaphragmatic hernia: prognostic evaluation of a large international cohort
Sanne Mbi Botden1, Kim Heiwegen1, Iris Alm van Rooij2
1Department of Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, The Netherlands.
Insights
Bilateral congenital diaphragmatic hernia (CDH) has a high mortality rate. Lower Apgar scores, extracorporeal membrane oxygenation (ECMO), and patch repair are associated with poorer outcomes in these rare cases.
Area of Science:
- Pediatric Surgery
- Neonatal Medicine
- Birth Defects Research
Background:
- Congenital diaphragmatic hernia (CDH) affects 1 in 2000-3000 live births.
- Bilateral CDH is a rare variant (1%) with a significantly high mortality rate.
- This study analyzes the largest cohort of bilateral CDH patients to date.
Purpose of the Study:
- To identify factors associated with mortality in patients diagnosed with bilateral congenital diaphragmatic hernia.
- To evaluate the outcomes of bilateral CDH patients within a large registry cohort.
Main Methods:
- Retrospective analysis of patient records from the Congenital Diaphragmatic Hernia Registry (1995-2015).
- Identification of 80 patients with bilateral CDH.
- Statistical analysis to determine parameters linked to mortality.
Main Results:
- Overall mortality for bilateral CDH was 74% (59 out of 80 patients).
- Lower Apgar scores at 1 and 5 minutes were observed in non-survivors compared to survivors.
- Extracorporeal membrane oxygenation (ECMO) and patch repair were associated with increased mortality risk.
Conclusions:
- Treatment for bilateral CDH remains a significant challenge with a high fatality rate.
- Factors negatively impacting survival include low Apgar scores, use of ECMO, and patch repair.
- Further research is needed to improve outcomes for this severe condition.
Background:
Congenital diaphragmatic hernia (CDH) is a lethal birth defect, which occurs in 1:2000-3000 live births. Bilateral CDH is a rare form (1%), with a high mortality. This study presents the outcomes of the largest cohort of bilateral CDH patients.
Methods:
The records of patients with bilateral CDH from the Congenital Diaphragmatic Hernia Registry born between 1995 and 2015 were retrospectively analyzed to identify parameters associated with mortality.
Results:
Eighty patients with a bilateral CDH were identified. Overall mortality was 74% (n=59). Apgar scores at 1 and 5min were statistically lower in the non-survivors compared to the survivors (median 3.0 and 5.0, versus 6.5 and 8.0, respectively, p<0.001). All survivors were repaired (n=21), compared to 22% of the non-survivors (n=17). The type of repair was equally divided in the survivors (52% primary versus 48% patch), while non-survivors were mainly patch repaired (82% versus 12%). Nineteen were treated with extracorporeal membrane oxygenation (ECMO) (24%), only three of them survived. When calculating the risk on mortality for the patients who lived until repair, ECMO had an adjusted odds ratio for mortality of 10.8 (95% CI: 2.0-57.7) and patch repair 5.2 (95% CI: 0.8-34.9).
Conclusions:
The treatment of bilateral CDH patients remains challenging with a high mortality rate. Lower Apgar-scores, ECMO (probably as a surrogate for the severity of disease), and patch repair were negatively associated with outcome.
Level Of Evidence:
Level IV study.
