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

Abstract

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 AE 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 AE 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%).Keywords Congenital diaphragmatic hernia, fetal therapy, magnetic resonance imaging, tracheal occlusion, ultrasound.Linked article This article is commented on by J Balayla, p. 947 in this issue. To view this mini commentary visit http:// dx.doi.org/10.1111/1471-0528.13077.Please cite this paper as: DeKoninck P, Gomez O, Sandaite I, Richter J, Nawapun K, Eerdekens A, Ramirez JC, Claus F, Gratacos E, Deprest J. Right-sided congenital diaphragmatic hernia in a decade of fetal surgery. BJOG 2015;122:940-946.

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

“…Another marker of pulmonary hypoplasia, the US-calculated O/E LHR, also did not seem to predict outcome in the fetus with R-CDH. The European group, which recently reported an overall survival of 53% in the group managed expectantly, described a significant increase in mortality if O/E LHR was ≤ 45 (survival: 17%) and O/E LHR was ≤ 30 (survival: 0%) [15] . Our group did not show this trend for O/E LHR ≤ 45 (survival 60%); the single case with LHR ≤ 30 did not survive, although no conclusions can be reached from one case.…”

“…These R-CDH survival rates can be compared to 70% survival of R-CDH cases reported from the Texas Children's Hospital group ( n = 37) [16] , 59% of the University of California, San Francisco group ( n = 34) [17] , and 53% from the European consortium ( n = 19) [15] .…”

“…Our group did not show this trend for O/E LHR ≤ 45 (survival 60%); the single case with LHR ≤ 30 did not survive, although no conclusions can be reached from one case. This discrepancy in O/E LHR values for survival between the US and European groups is important, as an LHR value of <45 has been proposed as a marker of severe pulmonary hypoplasia and cut-off value for offering antenatal intervention by means of fetal endoscopic tracheal occlusion [15,18] . The difference between these outcomes may be based on the relatively low number of cases reported in each center (19 in the European group, 21 with available O/E LHR values at our center) or differences in postnatal clinical protocols and care of the infant between the centers.…”

“…Another marker of severe pulmonary hypoplasia is the US-calculated O/E LHR. An O/E LHR of ≤ 45% has been shown to indicate severe pulmonary hypoplasia [15] . In the present study, 5 patients had an O/E LHR ≤ 45 (range 30-44, mean 40), of whom 3 survived (60% survival).…”

Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline

“…16 The observed-to-expected total fetal lung volume has performed well for survival prediction in CDH, with areas under the curve ranging from 0.79 to 0.89.…”