Wan Teng Lee, Paul D. Losty
University of Liverpool. Alder Hey Children’s Hospital Liverpool. Ramathibodi Hospital and Mahidol University.
United Kingdom and Thailand
Acta Paediatrica
Acta Paediatr 2025;
DOI: 10.1111/apa.70295
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.
Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes