BJOG

. 2023 Apr 17.

 doi: 10.1111/1471-0528.17497. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/37069727/

Survival in very preterm infants with congenital diaphragmatic hernia and association with prenatal imaging markers: A retrospective cohort study

Emily J J Horn-Oudshoorn ¹, Francesca M Russo ^2 3, Jan A Deprest ^2 3, Florian Kipfmueller ⁴, Annegret Geipel ⁵, Thomas Schaible ⁶, Neysan Rafat ⁶, Anne-Gael Cordier ^7 8, Alexandra Benachi ^7 8, Nimrah Abbasi ⁹, Priscilla P L Chiu ^10 11, Willem P de Boode ¹², Esther Sikkel ¹³, Nina C J Peters ¹⁴, Bettina E Hansen ^15 16 17, Irwin K M Reiss ¹, Philip L J DeKoninck ¹⁴

Affiliations expand

• PMID: 37069727
• DOI: 10.1111/1471-0528.17497

Abstract

Objectives: To describe the outcomes of preterm born infants with congenital diaphragmatic hernia (CDH; ≤32.0 weeks of gestation) and the associations between prenatal imaging markers and survival.

Design: Retrospective cohort study.

Setting: Multicentre study in large referral centres.

Population: Infants with an isolated unilateral CDH, live born at 32.0 weeks or less of gestation, between January 2009 and January 2020.

Methods: Neonatal outcomes were evaluated for infants that were expectantly managed during pregnancy and infants that underwent fetoscopic endoluminal tracheal occlusion (FETO) therapy, separately. We evaluated the association between prenatal imaging markers and survival to discharge. Prenatal imaging markers included observed to expected lung-to-head ratio (o/e LHR), side of the defect, liver position, stomach position grade, and observed to expected total fetal lung volume (o/e TFLV).

Main outcome measure: Survival to discharge.

Results: We included 53 infants born at 30⁺⁴ (interquartile range 29⁺¹ -31⁺² ) weeks. Survival in fetuses expectantly managed during pregnancy was 48% (13/27) in left-sided CDH and 33% (2/6) in right-sided CDH. Survival in fetuses that underwent FETO therapy was 50% (6/12) in left-sided CDH and 25% (2/8) in right-sided CDH. The o/e LHR at baseline was positively associated with survival in cases expectantly managed during pregnancy (odds ratio [OR] 1.20, 95% CI 1.07-1.42, p < 0.01), but not in cases that received FETO therapy (OR 1.01, 95% CI 0.88-1.15, p = 0.87). Stomach position grade (p = 0.03) and o/e TFLV were associated with survival (p = 0.02); liver position was not (p = 0.13).

Conclusions: In infants with CDH born at or before 32 weeks of gestation, prenatal imaging markers of disease severity were associated with postnatal survival.

Keywords: congenital diaphragmatic hernia; counselling; mortality; prediction; prematurity; prenatal imaging; pulmonary hypoplasia.

© 2023 The Authors. BJOG: An International Journal of Obstetrics and Gynaecology published by John Wiley & Sons Ltd.