Right congenital diaphragmatic hernias: Is there a correlation between prenatal lung volume and postnatal survival, as in isolated left diaphragmatic hernias?

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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.

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Victoria, T., Danzer, E., Oliver, E. R., Edgar, J. C., Iyoob, S., Partridge, E. A., … Adzick, N. S. (2018). Right congenital diaphragmatic hernias: Is there a correlation between prenatal lung volume and postnatal survival, as in isolated left diaphragmatic hernias? Fetal Diagnosis and Therapy, 43(1), 12–18. https://doi.org/10.1159/000464246

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