Research: Prematurity and Congenial Diaphragmatic Hernia: Revisiting Outcomes in a Contemporary Cohort

J Pediatr

. 2025 Mar 19:114545.

 doi: 10.1016/j.jpeds.2025.114545. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/40118245/

Prematurity and Congenial Diaphragmatic Hernia: Revisiting Outcomes in a Contemporary Cohort

Petru I Alexe 1Ana Sanchez Torres 2Ashley H Ebanks 3Enrico Danzer 4Krisa van Meurs 5Matthew T Harting 3Carmen Mesas Burgos 6Congenital Diaphragmatic Hernia Study Group

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Abstract

Objective: To describe outcomes in a contemporary cohort of infants born at gestational age (GA) <34 weeks with congenital diaphragmatic hernia (CDH).

Study design: Data from the multicenter, multinational CDH Study Group (CDHSG) registry on patients born between 1995 to 2022 were analyzed. Variables including prenatal characteristics and therapy, postnatal management, and outcomes to discharge were evaluated. A t-test was used for continuous variables, and for categorical variables, Fisher exact test.

Results: 13471 CDH patients were entered into the registry during the study period. 810 (6%) were born at < 34 weeks´ GA. The survival rate for the preterm cohort was 45%, and 43.5% were not repaired. Survival after surgical repair was 79%, mean age at surgery was 11 days of life (DOL), and 65% required patch repair. Extracorporeal life support (ECLS) was used in only 7% of the cases, and 35% survived. 48.9% had an associated anomaly. Oxygen at 30 DOL was required by 76.7%. Very preterm birth (<32 weeks´ GA) occurred in 361 patients (2.8%), with a 39.3% survival rate and a high rate of non-repairs (50.1%). The incidence of birth at <34 weeks´ GA for those undergoing fetal tracheal occlusion (FETO) was 30%.

Conclusion: Survival rates for infants born preterm with CDH remain low and the implementation of FETO is likely to increase the rate of prematurity in CDH. The high rate of non-repair is a significant contributor to lower survival and more aggressive use of ECLS and surgical repair may improve survival.

Keywords: CDH; CDH Study Group; CDHSG; ECLS; Extracorporeal Membrane Oxygenation (ECMO); Extracorporeal life support; congenital diaphragmatic hernia; early term; fetal tracheal occlusion (FETO); late term; outcome; prematurity; preterm; very preterm.

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