Bilateral congenital diaphragmatic hernia: prognostic evaluation of a large international cohort.

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.