Search

Background Our aim was to evaluate the prognostic value of magnetic resonance imaging (MRI)-based ratio of fetal lung volume (FLV) to fetal body volume (FBV) as a marker for development of chronic lung disease (CLD) in fetuses with congenital diaphragmatic hernia (CDH). Patients and Methods FLV and FBV were measured and the individual FLV/FBV ratio was calculated in 132 fetuses. Diagnosis of CLD was established following prespecified criteria and graded into mild/moderate/severe if present. Logistic regression analysis was used to calculate the probability of postnatal development of CLD in dependence of the FLV/FBV ratio. Receiver operating characteristic curves were analysed by calculating the area under the curve to evaluate the prognostic accuracy of this marker. Results 61 of 132 fetuses developed CLD (46.21%). The FLV/FBV ratio was significantly lower in fetuses with CLD (p=0.0008; AUC 0.743). Development of CLD was significantly associated with thoracic herniation of liver parenchyma (p<0.0001), requirement of extracorporal membrane oxygenation (ECMO) (p<0.0001) and gestational age at delivery (p=0.0052). Conclusion The MRI-based ratio of FLV to FBV is a highly valuable prenatal parameter for development of CLD. The ratio is helpful for early therapeutic decisions by estimating the probability to develop CLD. Perinatally, gestational age at delivery and ECMO requirement are useful additional parameters to further improve prediction of CLD., (© Georg Thieme Verlag KG Stuttgart · New York.)

Objective: The purpose of this study is to compare the observed-to-expected MRI fetal lung volume and the observed-to-expected ultrasound lung-to-head ratio intraindividually for prediction of survival, the need for extracorporeal membrane oxygenation (ECMO) therapy, and the development of chronic lung disease (CLD) at different times of gestation (< 28, 28-32, and > 32 weeks) in fetuses with congenital diaphragmatic hernia (CDH), and to analyze the intraindividual correlation of observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio., Materials and Methods: Two hundred one fetuses were included in our study. Observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio were calculated for 270 MRI and ultrasound examinations performed within 72 hours of each other. The prognostic accuracy of observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio was assessed by performing ROC analysis. Correlation was determined using linear regression analysis., Results: The results revealed statistically significant differences between the observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio for the prediction of survival, the need for ECMO therapy, and the development of CLD in fetuses with left-sided CDH (p ≤ 0.0444), with the highest prognostic accuracy for survival (AUC = 0.863). Observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio correlated statistically significantly (p < 0.0001) for left-sided CDH with correlation coefficients (r) of 0.71, 0.71, and 0.56 for early, intermediate, and late times of gestation, respectively. The correlation was not statistically significant at any time for right-sided CDH (p ≥ 0.3947; r ≤ 0.26)., Conclusion: Observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio are valuable prognostic parameters for prenatal prediction of survival, the need for ECMO therapy, and the development of CLD in fetuses with left-sided CDH at all times of gestation. Observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio correlate statistically significantly for left-sided CDH, and the correlation is best before 32 weeks' gestation, but they do not correlate statistically significantly for right-sided CDH.

Purpose: The accuracy of the sonographic weight estimation (WE) of fetuses with congenital diaphragmatic hernia (CDH) is significantly lower than that of fetuses without any malformations. The objective of this study was to develop and evaluate the first specific sonographic weight formula for fetuses with CDH., Materials and Methods: In a retrospective, multicenter, cohort study, a statistical estimation technique known as "multivariable fractional polynomial regression" was applied to a group of 146 fetuses with CDH. Each fetus underwent an ultrasound examination with complete biometric parameters within 7 days of delivery. A new formula was derived using the obtained data and was then compared with other commonly used equations. The accuracy of the different formulas was compared using means of signed percentage errors (SPE), medians of absolute percentage errors (MAPE), and fractions of estimates within prespecified error bounds., Results: The new derived formula is: EFW = 10^(4.6729 107 371 + 0.2365 011 768 * HC + 0.2228 897 682 * FL^2 - 0.0129 895 773 * FL^3 - 1.0470 039 072 * (FL * HC)^0.5 + 0.0004 314 661 * (AC * HC) - [in case of liver herniation] 0.0062 112 122), where EFW is the estimated fetal weight, HC is the head circumference, AC is the abdominal circumference, and FL is the femur length. The new formula proved to be superior to other established equations, showing both the lowest median absolute percentage error (MAE: 6.97) and mean signed percentage error (SPE: 0.40), and the best distribution of absolute percentage errors within prespecified error bounds., Conclusion: This new formula significantly improves weight estimation in fetuses with CDH., (© Georg Thieme Verlag KG Stuttgart · New York.)

Objective: The objective of our study was to evaluate the prognostic accuracy of the MRI-based ratio of fetal lung volume (FLV) to fetal body volume (FBV) for predicting survival and the need for extracorporeal membrane oxygenation (ECMO) therapy in fetuses with congenital diaphragmatic hernia (CDH)., Materials and Methods: MRI was performed in 96 fetuses (34 females and 62 males; mean gestational age, 33 ± 5 [SD] weeks; range, 23-39 weeks) with CDH and 44 healthy control subjects. FLV and FBV were measured for each fetus by one of three investigators, and we calculated the FLV/FBV ratio. Additionally, a subgroup analysis of growth-restricted fetuses was performed. Logistic regression analysis was used to model the dependence of survival and ECMO requirement on the FLV/FBV ratio. Prognostic accuracy was evaluated by applying the area under the curve (AUC)., Results: Seventy-eight of the 96 (81%) fetuses survived and 35 (36%) neonates needed ECMO therapy. The FLV/FBV ratio was significantly higher in fetuses who survived (p < 0.0001; AUC = 0.829). Neonates who received ECMO therapy showed a significantly lower prenatal FLV/FBV ratio (p = 0.0001; AUC = 0.811) than neonates who did not need ECMO therapy. A subgroup analysis (n = 13) showed a trend toward a higher FLV/FBV ratio of survivors in comparison with nonsurvivors (p = 0.065; AUC = 0.875)., Conclusion: In our study, the MRI-based FLV/FBV ratio was able to predict neonatal survival and ECMO requirement in children with CDH with high accuracy. Unlike other prognostic parameters, FLV/FBV ratio is independent of a reference to a control group and may enhance prognostic accuracy particularly in growth-restricted neonatal patients.

Objectives: The purpose of this study was to compare different methods for measuring the fetal lung area-to-head circumference ratio and to investigate their prediction of postpartum survival and the need for neonatal extracorporeal membrane oxygenation (ECMO) therapy in fetuses with isolated congenital diaphragmatic hernias., Methods: This prospective study included 118 fetuses of at least 20 weeks' gestation with isolated left-sided congenital diaphragmatic hernias. The lung-to-head ratio was measured with 3 different methods (longest diameter, anteroposterior diameter, and tracing). To eliminate the influence of gestational age, the observed-to-expected lung-to-head ratio was calculated. Receiver operating characteristic (ROC) curves were calculated for the statistical prediction of survival and need for ECMO therapy by the observed-to-expected lung-to-head ratio measured with the different methods., Results: For survival and ECMO necessity 118 and 102 cases (16 neonates were not eligible for ECMO) were assessed, respectively. For prediction of postpartum survival and ECMO necessity, the areas under the ROC curves and 95% confidence intervals showed very similar results for the 3 methods for prediction of survival (tracing, 0.8445 [0.7553-0.9336]; longest diameter, 0.8248 [0.7360-0.9136]; and anteroposterior diameter, 0.8002 [0.7075-0.8928]) and for ECMO necessity (tracing, 0.7344 [0.6297-0.8391]; longest diameter, 0.7128 [0.6027-0.8228]; and anteroposterior diameter, 0.7212 [0.6142-0.8281]). Comparisons between the areas under the ROC curves showed that the tracing method was superior to the anteroposterior diameter method in predicting postpartum survival (P = .0300)., Conclusions: Lung-to-head ratio and observed-to-expected lung-to-head ratio measurements were shown to accurately predict postnatal survival and the need for ECMO therapy in fetuses with left-sided congenital diaphragmatic hernias. Tracing the limits of the lungs seems to be the favorable method for calculating the fetal lung area.

Objective: To investigate individual changes in fetal lung volume (FLV) in fetuses with isolated congenital diaphragmatic hernia (CDH) and to calculate weekly growth rates of the FLV using serial MR examinations during pregnancy., Methods: MR-FLV was measured in 89 fetuses with CDH. All fetuses received two MRIs. A mean weekly growth rate of the FLV was determined for each fetus and compared with the growth rate of healthy fetuses., Results: Mean observed-to-expected MR-FLV (o/e MR-FLV) measured at the first MRI was 33.3 ± 12.2% and 29.5 ± 10.9% at the second MRI. In 61% of all fetuses (54/89) the o/e MR-FLV decreased during pregnancy, 26% (23/89) showed an increase in the o/e MR-FLV and 13 % (12/89) had stable values. First and last o/e MR-FLV values were significantly associated with mortality and neonatal extracorporeal membrane oxygenation (ECMO) requirement with a higher prognostic accuracy of MR-FLV measurements near delivery. Patients with CDH had lower weekly lung growth rates than healthy fetuses. There was a significant difference in the mean weekly growth rate between survivors and non-survivors and patients with and without ECMO requirement., Conclusion: Individual development of FLV in patients with CDH during pregnancy is extremely variable. Follow-up MR-FLV measurements are advisable before deciding upon pre- and postnatal therapeutic options., Key Points: • Lung development in congenital diaphragmatic hernia (CDH) during pregnancy is extremely variable. • MRI demonstrates that lung growth rate is reduced in fetuses with CDH. • The final observed-to-expected fetal lung volume provides the best prognostic information. • Follow-up measurements are advisable before deciding upon therapeutic options.

Purpose: To determine the accuracy of sonographic weight estimation (WE) for fetuses with congenital diaphragmatic hernia (CDH), and to assess whether certain sonographic models perform better than others in cases of CDH., Material and Methods: In a retrospective, multicenter cohort study, the accuracy of WE in fetuses with CDH (n = 172) was evaluated using eight sonographic models and was compared with a control group of fetuses without malformations (n = 172). Each fetus underwent ultrasound examination with complete biometric parameters within 7 days of delivery. The accuracy of the different formulas was compared using means of percentage errors (MPE), medians of absolute percentage errors (MAPE), and proportions of estimates within 10 % of actual birth weight., Results: Fetuses with CDH had a significantly lower abdominal circumference (AC) in comparison with the control group (293.6 vs. 312.0 mm, p < 0.001). All of the formulas tested in fetuses with CDH, except for the Siemer equation (the only model that does not incorporate any abdominal measurements), showed significantly lower (more negative) MPEs, larger MAPEs, and smaller proportions of estimates within 10 % of actual birth weight in comparison with the control group. , Conclusion: The accuracy of sonographic WE in fetuses with CDH is significantly poorer than in fetuses without malformations, principally because of a larger systematic error due to artificially small AC measurements. The development of new, specific models derived from fetuses with CDH could improve the accuracy of WE for infants with this condition., (© Georg Thieme Verlag KG Stuttgart · New York.)

Objective: The purpose of the study was to investigate the ability to predict survival, need for extracorporeal membrane oxygenation (ECMO), and incidence of chronic lung disease in patients with congenital diaphragmatic hernia in the context of a classification into three different times of gestation (< 28, 28-32, and > 32 weeks) by assessing the ratio between observed and expected MRI fetal lung volume., Materials and Methods: The data analysis included 226 fetuses with congenital diaphragmatic hernia. MRI was performed at different times of gestation with a T2-weighted HASTE sequence. Receiver operating characteristic curve analysis was performed to investigate the prognostic value of assessment of the ratio between observed and expected MRI fetal lung volumes at different stages of fetal growth., Results: For all reviewed times of gestation, the ratio between observed and expected MRI fetal lung volumes had almost equivalent statistically significant differences for neonatal survival (p ≤ 0.0029), need for ECMO therapy (p ≤ 0.0195), and development of chronic lung disease (p ≤ 0.0064). Results with high prognostic accuracy for early and for medium and late times in gestation also were obtained. Receiver operating characteristic analysis showed the highest area under the curve (≥ 0.819) for neonatal survival., Conclusion: In patients with congenital diaphragmatic hernia, the relation between observed and expected MRI fetal lung volume is a valuable prognostic parameter for predicting neonatal mortality, morbidity represented by the development of chronic lung disease, and the need for ECMO therapy in early gestation (< 28 weeks) as well as later gestation with no statistically significant differences.

Objectives: To compare different rotation angles for assessment of fetal lung volume by 3-dimensional (3D) sonography with magnetic resonance imaging (MRI) regarding prediction of mortality and the need for neonatal extracorporeal membrane oxygenation (ECMO) therapy in fetuses with congenital diaphragmatic hernias., Methods: One hundred patients with fetal congenital diaphragmatic hernias between 22 and 39 weeks' gestation were examined by 3D sonography and MRI. Sonographic contralateral fetal lung volumes were assessed by the rotational technique (virtual organ computer-aided analysis) at 3 different rotation angles: 6°, 15°, and 30°. The MRI fetal lung volumes were calculated based on multiplanar T2-weighted MRI. To eliminate the influence of gestational age, the observed to expected contralateral fetal lung volume on sonography and the observed to expected fetal lung volume on MRI were calculated. Receiver operating characteristic (ROC) curves were calculated for the statistical prediction of survival and need for ECMO therapy by the observed to expected contralateral fetal lung volume (sonography) and observed to expected fetal lung volume (MRI)., Results: One hundred cases were assessed for survival and 89 for ECMO necessity (11 neonates were not eligible for ECMO). For prediction of postpartum survival and ECMO necessity, the areas under the ROC curves (AUCs) showed very similar results for MRI and 3D sonography: observed to expected fetal lung volume by MRI, 0.819 (95% confidence interval, 0.730-0.909) and 0.835 (0.748-0.922), respectively; 6° sonography, 0.765 (0.647-0.883) and 0.820 (0.734-0.905); 15° sonography, 0.784 (0.672-0.896) and 0.811 (0.719-0.903); and 30° sonography, 0.732 (0.609-0.855) and 0.772 (0.671-0.872). Comparisons between the AUCs revealed no statistical differences., Conclusions: We have shown the good prognostic value of 3D sonography in fetuses with congenital diaphragmatic hernias compared with MRI, particularly when using small rotation angles. Therefore, it can be an appropriate diagnostic tool when counseling patients for congenital diaphragmatic hernias.

Aim: To evaluate the influence of gender in neonates with congenital diaphragmatic hernia (CDH) on survival and to assess the necessity of extracorporeal membrane oxygenation (ECMO) therapy., Patients and Methods: All parturients with newborns suffering from CDH were included. A total of 425 infants with CDH were analyzed. The primary outcome parameters evaluated were the necessity of ECMO and the survival. Secondary outcome parameters were the mode of delivery, the arterial umbilical cord pH value, the Apgar score, and the postpartum day of death. Cases with incomplete data were excluded., Results: An analysis of the gender distribution of neonates in our study revealed that more males (n=248) than females (n=177) suffered from CDH. This represented a male versus female gender ratio of 58.4% to 41.6%, a significantly different ratio from that for neonates without CDH (p=0.003). Comparing both groups, statistical analysis showed no significant differences in mode of delivery, arterial umbilical cord blood pH, Apgar score, or CDH-relevant parameters of postpartum survival, including the need for ECMO therapy., Conclusion: CDH occurred significantly more frequently in male newborns. However, there were no significant differences in postnatal survival nor in the necessity of ECMO therapy.

Cytokines play an important role in immune regulation and fetal lung development. The systemic inflammatory response in newborns with congenital diaphragmatic hernia (CDH) has not been characterized so far. We compared various concentrations of cytokines in serum from newborns with CDH and in healthy term neonates. We analyzed cytokine patterns of CDH newborns under extracorporeal membrane oxygenation (ECMO) and mechanical ventilation (MV).38 newborns with CDH were included: ECMO group (n=13) and non-ECMO group (n=25). Healthy term neonates served as controls (n=13). Serum samples were obtained prospectively after birth and during therapy.Concentrations of IFN-α, IL-3,-6,-7,-8,-10, MIP-1α,-1β and TNF-α in serum of newborns with CDH were higher than in umbilical cord blood of term neonates. Infants with severe CDH requiring ECMO therapy had higher postnatal IL-8,-10, and MIP-1α levels than newborns with milder disease in the non-ECMO treated group. IL-10 progressively decreased during the first 3 days following birth under ECMO. In contrast, the chemokine MIP-1α remained elevated during ECMO therapy compared to mechanically ventilated CDH newborns.The pattern of cytokines in the serum of newborns with CDH showed significant elevations compared to term neonates. Our findings indicate that CDH is associated with systemic inflammatory response immediately after birth. ECMO and MV show a similar increase of IL-1α and IP-10 in CDH newborns assuming a persistent pulmonary inflammatory reaction irrespective of the conducted treatment., (© Georg Thieme Verlag KG Stuttgart · New York.)

Objectives: The purpose of this study was to evaluate the influence of different rotation angles in assessment of the contralateral lung volume by 3-dimensional (3D) sonography in comparison to magnetic resonance imaging (MRI) in fetuses with congenital diaphragmatic hernias., Methods: A total of 126 measurements by 3D sonography and MRI were conducted in 81 patients between 18 and 39 weeks' gestation. The 3D sonographic volumes of the contralateral fetal lung were calculated by the rotational technique (virtual organ computer-aided analysis) with rotation angles of 6°, 15°, and 30°. Transverse multiplanar T2-weighted MRI was performed for the MRI measurements. To compare the accuracy of 3D sonographic volumetry using different rotation angles, MRI assessment was taken as the reference method, and percentage errors and limits of agreement were calculated for each angle., Results: Three-dimensional sonographic volume measurements showed a high correlation with MRI (6° angle, R(2) = 0.86; 15° angle, R(2) = 0.78; 30° angle, R(2) = 0.68). The mean percentage error showed no systematic error. With regard to random error, the 6° step had significantly lower values than the larger angles 30° step (R = 0.472) and the narrowest limits of agreement., Conclusions: Especially when using a small rotation angle, assessment of the contralateral fetal lung volume by 3D sonography in congenital diaphragmatic hernias is a reliable alternative to MRI.

Congenital diaphragmatic hernia (CDH) is a severe neonatal anomaly. The aim of this study was to evaluate the frequency and types of malformations associated with CDH. The outcome was compared with that in newborns with CDH alone. The study included 362 fetuses and newborns at a single national center for CDH. Associated malformations and chromosomal aberrations were noted prenatally and postnatally. The neonatal outcome was assessed relative to the use of extracorporeal membrane oxygenation (ECMO) and the mortality rate. At least one associated malformation was diagnosed in 143 cases (39.5%). Altogether, 272 associated malformations were found. Only 50 (18.4%) anomalies were diagnosed antenatally. In 62 (17.1%) cases, 102 major malformations were found along with CDH, with a prenatal detection rate of 35.3%. The associated malformations were very heterogeneous, but cardiovascular malformations were the most common. Newborns with major anomalies, chromosomal aberrations, or syndromes additional to CDH had a significantly lower survival rate than newborns with an isolated CDH. Associated malformations did not affect the rate of ECMO treatment. Associated malformations in CDH are frequent and heterogeneous, and diligent and experienced antenatal and postnatal care is important., (©Thieme Medical Publishers.)