Abstract / Summary
Abstract Background Current neonatal stabilization guidelines recommend immediate endotracheal intubation for congenital diaphragmatic hernia (CDH), although selected neonates with favorable prenatal features may tolerate an initial spontaneous breathing approach (SBA). We systematically reviewed the evidence on non-routine intubation in prenatally predicted mild CDH. Methods PubMed/MEDLINE, Embase, Scopus, LILACS, and SciELO were searched from inception. The search was performed on November 27, 2024, and updated through August 8, 2026. Two reviewers independently screened studies. Risk of bias was assessed according to study design, and certainty of evidence was evaluated using GRADE. Owing to clinical and methodological heterogeneity, findings were synthesized narratively. Results Three observational studies comprising 30 neonates initially managed with SBA were included. Fourteen neonates (46.7%) avoided unplanned pre-operative intubation, whereas 16 (53.3%) required intubation because of respiratory deterioration. Across the included studies, 16 of 30 neonates (53.3%) required unplanned pre-operative intubation. While no deaths were reported in the included cohorts, subgroup-specific survival to discharge could not be independently confirmed in one study due to incomplete denominator reporting. Overall, adverse events and ECMO use were uncommon, but reporting limitations preclude definitive conclusions regarding safety or survival. Conclusions Available evidence regarding spontaneous breathing in prenatally predicted mild CDH is of very low certainty and insufficient to establish safety or effectiveness relative to routine intubation. Given the substantial heterogeneity and small sample sizes, routine intubation remains the standard of care. Further prospective, standardized research is required before any clinical recommendation can be made.