Improving outcome in congenital diaphragmatic hernia - experience of a tertiary center without ECMO

J Neonatal Perinatal Med. 2018;11(1):37-43. doi: 10.3233/NPM-181710.

Abstract

Background: Congenital diaphragmatic hernia (CDH) has a high mortality rate, representing a therapeutic challenge. Prenatal diagnosis (PND) is essential in defining optimal perinatal strategy, particularly delivery planning. Hospital Pediátrico de Coimbra is the referral centre for all neonatal surgery, particularly CDH, for the central region of Portugal. The aim was to evaluate clinical management and outcome of newborns with CDH.

Methods: An exploratory retrospective study made up of newborns admitted to PICU with CDH was undertaken between January 1995 and December 2014. Two groups were formed based on their year of admission: group A (1995- 2004) and group B (2005- 2014) and were compared.

Results: The mean birth weight of the 69 newborns admitted was 2.762 ± 696 g; the median of the gestational age was 38 weeks. Associated malformations were observed in 28 (40.5%) and 15 (21.7%) had a right-sided diaphragm defect. The global mortality was 13.0%; in group A was significantly higher than in group B (22.2 vs 3.0%; p = 0.029). A reduction in mortality throughout the years was confirmed after adjusting for POS score (OR = 0.77; 95% CI: 0.62- 0.96, p = 0.021). PND was made in 30.6% of cases in the group A and 66.7% in the group B (p = 0.03). Tertiary perinatal hospital birth was achieved in 60% of newborns in the group A versus 84.8% in group B (p = 0.022). Maximum fraction of inspired oxygen showed a statistically significant difference between the two study groups (60% vs 40%; p = 0.009).

Conclusions: A significant decrease in mortality was observed throughout the study. The authors highlight the increase in prenatal diagnosis and an improvement in perinatal care with planning delivery as important contributors to these results.

Keywords: Congenital diaphragmatic hernia; mortality; prenatal diagnosis; therapeutic strategy.

MeSH terms

  • Birth Weight*
  • Extracorporeal Membrane Oxygenation
  • Female
  • Gestational Age
  • Hernias, Diaphragmatic, Congenital / diagnostic imaging*
  • Hernias, Diaphragmatic, Congenital / physiopathology
  • Hernias, Diaphragmatic, Congenital / therapy*
  • Herniorrhaphy
  • Humans
  • Hypnotics and Sedatives / therapeutic use
  • Immobilization
  • Infant, Newborn
  • Intubation, Intratracheal
  • Male
  • Respiration, Artificial*
  • Retrospective Studies
  • Survival Rate
  • Tertiary Care Centers
  • Treatment Outcome
  • Ultrasonography, Prenatal

Substances

  • Hypnotics and Sedatives