Right-sided congenital diaphragmatic hernia in a decade of fetal surgery

Objective: To report a recent update on fetuses with right-sided congenital diaphragmatic hernia (RCDH) in the era of fetal surgery.

Design: Retrospective review of prospectively collected data.

Setting: Fetal treatment centres in Leuven and Barcelona.

Population: Consecutive cases of RCDH between 2002 and 2012.

Methods: Data on prenatal imaging, genetic testing, pregnancy and neonatal outcomes were extracted from our databases, including structural and genetic anomalies, candidate outcome predictors such as lung size, liver herniation ratio, polyhydramnios, cervical length, preterm prelabour rupture of membranes and gestational age at birth.

Main outcome measures: Survival and oxygen dependency at discharge.

Results: Ten out of 86 fetuses with RCDH had associated abnormalities. Of 76 isolated pregnancies, eight women opted for termination of pregnancy, most with severe hypoplasia and one was lost to follow up. Nineteen pregnancies were expectantly managed and delivered at a mean gestational age of 36.0 ± 3.0 weeks. Survival at discharge was 53% (10/19), one being oxygen dependent. In the fetal surgery group (n = 48), mean gestational age at delivery was 34.5 ± 3.0 weeks. In our recent experience not previously published (n = 23) survival rate was 52 and 39% were oxygen dependent at discharge. Pooling these data with earlier reported observations by our group we observed a 42% survival rate in 57 fetuses. Lung size on magnetic resonance imaging and an interval of >24 hours between reversal of tracheal occlusion and delivery were predictors of outcome.

Conclusions: Right-sided CDH seems to have a poorer outcome than that reported for fetuses with left-sided CDH with similar lung size before birth. Survival rates after expectant management with observed/expected lung-to-head ratio values ≤45 and ≤30% were 17 and 0%, respectively. In those undergoing fetal surgery (observed/expected lung-to-head ratio ≤45%) there was an apparent increase (42%).