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.

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Objective We conducted a meta-analysis to assess the correlation of lung volume and liver position measured by magnetic resonance imaging (MRI) with survival until discharge in fetuses with isolated congenital diaphragmatic hernia (CDH).Method Systematic searches of MEDLINE and EMBASE from 1 January 1980 to 10 December 2010 were performed. Studies correlating total fetal lung volumes (TFLV, observed/expected (O/E) TFLV) and/or liver position by fetal MRI to survival in expectantly managed fetuses with CDH were included. Data on the side of the defect, position of the liver, TFLV, O/E TFLV, gestational age (GA) at MRI, GA and weight at birth were collected. Odds ratio (OR) for dichotomous data, mean differences (MD) or standardized mean differences (SMD) for continuous variables were determined using RevMan 5.0 software.Results Nineteen studies (n = 602 fetuses) were included. Survival was associated with left-sided defects (OR 2.52; p = 0.01), "liver down" (OR 0.18; p < 0.00001), a higher TFLV (MD 9.63; p < 0.00001) and O/E TFLV (SMD 0.98; p < 0.00001) as well as higher birth weight (MD 146.60; p = 0.04). GA at MRI (MD 0.70) and GA at birth (MD 0.33) were not correlated with survival.Conclusions MRI measurements of fetal lung volumes, liver position and side of the defect correlate well with neonatal survival in fetuses with isolated CDH.

The role of prenatal ultrasound and magnetic resonance imaging in the diagnosis and management of congenital diaphragmatic hernia (CDH) is reviewed. Topics include morphologic imaging and vascular assessment of the developing lung, the value of imaging parameters as prognostic predictors in CDH and the role of imaging following percutaneous fetoscopic endoluminal tracheal occlusion.

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Objective Fetuses with congenital diaphragmatic hernia (CDH)

Purpose: In fetuses with isolated congenital diaphragmatic hernia (CDH), lung development can be measured by the lung-to-head ratio (LHR) using ultrasound as well as by lung volumetry determined by fetal magnetic resonance imaging (MRI). We aimed to investigate their relationship as well as to analyze the factors that may have an impact on it. Material and Methods: In 153 consecutive fetuses with isolated CDH, both the LHR and total fetal lung volume (TFLV) were measured. The observed LHR was calculated by dividing the lung area by the head circumference. On MRI, planimetric measurements of ipsilateral, contralateral and TFLV were performed on T₂-HASTE (half-Fourier acquisition single-shot turbo spin echo) sequences in transverse as well as coronal or sagittal planes. All values were expressed as a ratio of what was observed over what is expected in a gestational age-matched normal fetus. Secondary analyses were performed for right- versus left-sided hernia and for measurements made prior to 25 weeks’ gestation. A multivariate linear regression approach was used to determine the influence of the independent variables such as observed/expected (O/E) LHR, gestational age, liver position and CDH side on the dependent variables O/E TFLV and O/E contralateral FLV, and to determine the optimal formulas for calculation of the O/E TFLV as well as contralateral FLV. Results: In total, 200 pairs of measurements were obtained between 20 and 37 weeks’ gestation (median 26+6). There was a significant association between the O/E contralateral FLV and O/E LHR (R² = 0.44; p < 0.001) as well as between the O/E TFLV and the O/E LHR (R² = 0.37; p < 0.001). After adding the independent variables that were first shown to be significant on univariate analysis, the multiple regression analysis demonstrated that gestational age (p = 0.017) and side of the defect (p < 0.001) were predictive of O/E LHR (p < 0.001) and strongly improved the estimation of O/E TFLV (R² = 0.43 instead of 0.37 when using O/E LHR only). In terms of estimating O/E contralateral FLV, only the O/E LHR was a significant (p < 0.001) independent predictor (R² = 0.44). These correlations also applied when considering only left-sided CDH cases. For measurements done prior to the third trimester, the O/E LHR (p = 0.034), gestational age (p = 0.035) as well as liver herniation (p = 0.029) were significantly correlated to the O/E TFLV (R² = 0.33). In terms of predicting the O/E contralateral FLV (R² = 0.25), only O/E LHR (p = 0.008) and gestational age (p = 0.037) were useful predictors. Conclusion: Measurement of the O/E LHR on ultrasound allows a good estimation of the O/E contralateral FLV as well as TFLV as measured by MRI. Whereas the additional parameters such as gestational age, liver position and side of the defect did not improve the estimation of the contralateral FLV, they did so for estimating the TFLV.

This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1002/pd.5719.Micro-computed tomography of isolated fetal hearts following termination of pregnancy: a feasibility study at 8 -12 week's gestation.