Outcomes for Congenital Diaphragmatic Hernia in Three Decades: A Report From a UK Surgical Centre.

Abstract

Aims: Congenital diaphragmatic hernia (CDH) is associated with lung hypoplasia, pulmonary hypertension and high mortality. Three decades experience from a UK centre is reported.

Methods: Medical records of CDH newborns between February 1990 and November 2021 and attending a multidisciplinary clinic were examined. Survival and health outcomes are recorded.

Results: Of 220 CDH newborns, left-sided defects were 177 (80%) cases. Diaphragm patch was required in 91 (41%) patients and 42 (19%) additionally required abdominal wall prosthesis. Materials deployed included Gore-Tex biological Surgisis patches all had early recurrences. Diaphragmatic patch was significantly associated with fundoplication (p 0.005). Overall survival rate was 85%-(90% survival primary defect vs. 79% patch) (p 0.035). Comparing decades, the post-2010 CDH study cohort was a sicker patient group comprising more index cases with cardiac malformations (69% vs. 28%, p < 0.001) requiring greater cardiovascular inotrope support (61% vs. 25%, p < 0.001) and ECMO (15% vs. 5%, p 0.023).

Conclusion: While a modestly excellent 85% survival rate is reported over three decades, CDH management at this UK university surgical centre witnessed a growing complexity of patients with mixed severity phenotype(s). Future challenges remain to be solved to improve survival for the most complex vulnerable patients.