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Congenital diaphragmatic hernia: an overview
1Department of Surgery, University of California, Davis, Medical Center, Sacramenlo 95817-2282, USA.
Insights
Congenital diaphragmatic hernia in infants causes lung hypoplasia and pulmonary hypertension. Modern treatment focuses on reversible issues, improving survival rates for respiratory distress.
Area of Science:
- Pediatric Surgery
- Neonatology
- Pulmonary Medicine
Background:
- Congenital diaphragmatic hernia (CDH) presents complex multisystem dysfunction in infants.
- Lung mass reduction, surfactant deficiency, and decreased compliance impair oxygenation and CO2 removal.
- Hypoplastic pulmonary arterial system exacerbates respiratory challenges.
Purpose of the Study:
- To review evolving understanding and treatment of CDH.
- To highlight shifts in therapeutic focus from operative repair to managing pathophysiological components.
- To assess the impact of new therapies on infant survival rates.
Main Methods:
- Review of current therapeutic strategies for CDH.
- Analysis of outcomes associated with extracorporeal membrane oxygenation (ECMO) and delayed repair.
- Exploration of emerging treatments like nitric oxide and fetal interventions.
Main Results:
- Treatment has shifted to address reversible pathophysiological processes.
- Extracorporeal membrane oxygenation and delayed repair have increased survival rates from 50% to 65% for infants with respiratory distress.
- Despite improvements, survival rates remain suboptimal, driving research into new therapies.
Conclusions:
- Current management strategies for CDH have improved survival.
- Pulmonary hypertension and lung hypoplasia remain critical challenges.
- Novel therapeutic approaches, including fetal intervention and lung transplantation, are under investigation to further enhance outcomes.
Abstract:
Understanding the complex multisystem dysfunction in the infant with a congenital hernia of the posterolateral diaphragm is still evolving and has changed radically during the last decade. The reduction in lung mass, in conjunction with surfactant deficiency and diminished compliance, leads to initial deficiencies in oxygenation and carbon dioxide (CO2) removal. This may then be potentiated by an extremely reactive hypoplastic pulmonary arterial system. Treatment no longer is focused on the operative repair but rather on the components of the pathophysiological process that are potentially reversible. Thus, extracorporeal membrane oxygenation and delay of repair until resolution of pulmonary artery hypertension have become mainstays of therapy and are probably responsible for increasing the survival rate in the patient who presents early with respiratory distress from 50% to 65%. Still far from acceptable, these results are giving impetus to new approaches to therapy including drugs such as nitric oxide, fetal intervention including open repair, and lung transplantation.