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%).

Correlation of observed-to-expected total fetal lung volume with intrathoracic organ herniation on magnetic resonance imaging in fetuses with isolated left-sided congenital diaphragmatic hernia.

OBJECTIVES: To assess using fetal magnetic resonance imaging (MRI) the relationship between the position of the stomach as well as the volume of herniation of organs into the thorax, and the observed-to-expected total fetal lung volume (o/e-TFLV), as a measure of pulmonary hypoplasia, in fetuses with isolated left-sided congenital diaphragmatic hernia (LCDH). METHODS: This was a single-center retrospective study using archived MR images from fetuses > 20 weeks' gestation evaluated for isolated LCDH over an 11-year period between July 2002 and September 2013. We retrieved data on the gestational age at MRI, o/e-TFLV and liver position. Images were also reviewed by a single operator to determine retrospectively the position of the stomach as well as the proportion of the total thorax volume occupied by the herniated fetal liver, stomach and other viscera. Following confirmation of reproducibility, we assessed the correlation of intrathoracic organ volumes and stomach position with o/e-TFLV. RESULTS: The study included 205 fetuses which underwent a total of 259 MR examinations. The reproducibility of organ volume measurements was excellent (intraclass correlation coefficient range, 0.928-0.997). The average time spent to obtain intrathoracic organ volumes ranged from 2.28 to 5.13 min. Of all herniated organ-to-thoracic volume ratios, the liver-to-thoracic volume ratio had the strongest correlation with o/e-TFLV (ρ = -0.429, P<0.0001). Stomach volume did not correlate, although, when categorized by the position and extent of stomach herniation, there was an inverse relationship to o/e-TFLV. No intrathoracic organ-to-thoracic volume ratio was related to gestational age. CONCLUSIONS: We observed in fetuses with isolated LCDH an inverse relationship between lung volume and the amount of liver herniated as well as the position of the stomach in the chest.

Comparison of matching by body volume or gestational age for calculation of observed to expected total lung volume in fetuses with isolated congenital diaphragmatic hernia.

OBJECTIVE: To determine the bias induced by matching fetuses according to gestational age (GA) or fetal body volume (FBV) when calculating the observed to expected total fetal lung volume (o/e TFLV) in cases of isolated congenital diaphragmatic hernia (CDH). METHODS: This was a single-center, retrospective study on archived magnetic resonance (MR) images of fetuses with isolated CDH over a 10-year period. We retrieved the TFLV, GA and o/e TFLVGA , and delineated FBV to obtain TFLVFBV in each case. We evaluated the relationship between o/e TFLVFBV and o/e TFLVGA by Bland-Altman analysis. All outliers were manually identified, and their specific clinical features were retrieved. RESULTS: Records of a total of 377 MR examinations of 225 fetuses were identified and included in the analysis. The mean ( ± SD) time spent on FBV measurement was 16.12 ± 4.95 min. On reproducibility analysis of FBV measurement (n = 10), the intraobserver intraclass correlation coefficient (ICC) was 0.998 and the interobserver ICC was 0.999. FBV was highly correlated with GA (R(2) = 0.899; P < 0.0001). There was good agreement between o/e TFLVGA and o/e TFLVFBV , with a mean difference of -1.10% and 95% limits of agreement of -8.58 to 6.39. There were no outliers in fetuses that had an o/e TFLV < 25%. Discrepancies induced by different methods were more likely in women with a body mass index ≥ 25 kg/m(2) (+16.5%), fetuses with an estimated fetal weight (EFW) ≤ 10(th) centile (+21.3%) or an EFW > 90(th) centile (+14.7%). CONCLUSIONS: Discrepancies in matching by FBV and GA when calculating o/e TFLV are more likely in fetuses with an abnormal EFW or in fetuses carried by overweight women. The clinical relevance of using FBV rather than GA for calculation of the o/e TFLV might be limited, as there was no discrepancy between the two methods in fetuses with small lungs ( < 25%), which is the group of most interest for lung volume assessment.