Right Congenital Diaphragmatic Hernias: Is There a Correlation between Prenatal Lung Volume and Postnatal Survival, as in Isolated Left Diaphragmatic Hernias?

Fetal Diagn Ther. 2018;43(1):12-18. doi: 10.1159/000464246. Epub 2017 Mar 21.

Abstract

Objectives: Whereas left-sided congenital diaphragmatic hernias (L-CDH) have been extensively studied and their prognostic parameters delineated, right-sided hernias (R-CDH) have not. Published results remain inconclusive. The aim of this study is to evaluate if proven prognostic indicators of postnatal survival in the fetus with L-CDH apply to the fetus with R-CDH.

Methods: Retrospective single-center study of R-CDH fetuses with available prenatal studies assessed for fetal lung volume by means of ultrasound-measured observed versus expected (O/E) lung area to head circumference (LHR) and magnetic resonance-calculated O/E total lung volume (TLV) in a 12-year time period. Percentage of herniated liver volume and postnatal use of extracorporeal membrane oxygenation (ECMO) were also evaluated.

Results: In a cohort of 24 patients, O/E LHR, O/E TLV, percentage of herniated liver, and postnatal use of ECMO are not prognostic indicators of survival in the fetus with R-CDH. Cut-off values of O/E LHR of ≤45 or O/E TLV ≤25, known to select a population of severe cases for the L-CDH fetus, do not appear to extrapolate to the R-CDH fetus, as survival in both R-CDH groups is 60%.

Conclusion: The findings in this study suggest that L- and R-CDH appear to behave differently, and that factors that make L-CDH fatal (low O/E TLV and O/E LHR, high-volume herniated liver) may not apply to the fetus with R-CDH.

Keywords: Congenital diaphragmatic hernia; Fetal endoscopic tracheal occlusion; Fetal lung volume measurement; Fetal magnetic resonance imaging; Fetal surgery; Prenatal imaging.

MeSH terms

  • Adult
  • Cephalometry
  • Extracorporeal Membrane Oxygenation
  • Female
  • Gestational Age
  • Hernias, Diaphragmatic, Congenital / diagnostic imaging*
  • Hernias, Diaphragmatic, Congenital / mortality
  • Hernias, Diaphragmatic, Congenital / therapy
  • Humans
  • Infant, Newborn
  • Liver / diagnostic imaging
  • Lung / diagnostic imaging*
  • Lung Volume Measurements / methods*
  • Magnetic Resonance Imaging*
  • Male
  • Middle Aged
  • Philadelphia
  • Predictive Value of Tests
  • Retrospective Studies
  • Risk Factors
  • Treatment Outcome
  • Ultrasonography, Prenatal*
  • Young Adult