Diagnostic accuracy of prenatal ultrasound and MRI in predicting survival in severe isolated congenital diaphragmatic Hernia: A systematic review and meta-analysis.

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.

Antenatal predictors of outcome in prenatally diagnosed congenital diaphragmatic hernia (CDH)

Objective Antenatal recognition of severe cases of congenital diaphragmatic hernia (CDH) by ultrasound (US) and magnetic resonance imaging (MRI) may aid decisions regarding the indication of fetal endoscopic tracheal occlusion.Methods An integrative review was performed. Searches in MEDLINE and EMBASE used terms related to CDH, diagnosis, MRI, and US. The inclusion criteria were reviews and guidelines approaching US and MRI markers of severity of CDH published in English in the past 10 years.Results The search retrieved 712 studies, out of which 17 publications were included. The US parameters were stomach and liver positions, lung-to-head ratio (LHR), observed/expected LHR (o/e LHR), and quantitative lung index. The MRI parameters were total fetal lung volume (TFLV), observed/expected TFLV, relative fetal or percent predicted lung volumes, liver intrathoracic ratio, and modified McGoon index. None of the parameters was reported to be superior to the others.Conclusion The most mentioned parameters were o/e LHR, LHR, liver position, o/e TFLV, and TFLV.

Fetal endoscopic tracheal occlusion (FETO) is associated with increased perinatal survival and reduced need for extracorporeal membrane oxygenation (ECMO) in fetuses with severe congenital diaphragmatic hernia (CDH). This study evaluates the impact of FETO on the resolution of pulmonary hypertension (PH) in fetuses with isolated CDH. We reviewed retrospectively the medical records of all fetuses evaluated for CDH between January 2004 and July 2017 at a single institution. Fetuses with additional major structural or chromosomal abnormalities were excluded. CDH cases were classified retrospectively into mild, moderate and severe groups based on prenatal magnetic resonance imaging indices (observed-to-expected total fetal lung volume and percentage of intrathoracic liver herniation). Presence of PH was determined based on postnatal echocardiograms. Logistic regression analyses were performed to evaluate the relationship between FETO and resolution of PH by 1 year of age while controlling for side of the CDH, use of ECMO, gestational age at diagnosis, gestational age at delivery, fetal gender, sildenafil use at discharge and CDH severity. Resolution of PH by 1 year of age was compared between a cohort of fetuses with severe CDH that underwent FETO and a cohort that did not have the procedure (non-FETO). A subanalysis was performed restricting the analysis to isolated left CDH. Parametric and non-parametric tests were used for comparisons. Of 257 CDH cases evaluated, 72% (n = 184) had no major structural or chromosomal anomalies of which 58% (n = 107) met the study inclusion criteria. The FETO cohort consisted of 19 CDH cases and the non-FETO cohort (n = 88) consisted of 31 (35%) mild, 32 (36%) moderate and 25 (28%) severe CDH cases. All infants with severe CDH, regardless of whether they underwent FETO, had evidence of neonatal PH. FETO (OR, 3.57; 95% CI, 1.05-12.10; P = 0.041) and ECMO (OR, 5.01; 95% CI, 2.10-11.96; P < 0.001) were independent predictors of resolution of PH by 1 year of age. A higher proportion of infants with severe CDH that underwent FETO had resolution of PH by 1 year after birth compared with infants with severe CDH in the non-FETO cohort (69% (11/16) vs 28% (7/25); P = 0.017). Similar results were observed when the analysis was restricted to cases with left-sided CDH (PH resolution in 69% (11/16) vs 28% (5/18); P = 0.032). In infants with severe CDH, FETO and ECMO are independently associated with increased resolution of PH by 1 year of age. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Cost-effective fetal lung volumetry for assessment of congenital diaphragmatic hernia

Congenital diaphragmatic hernia: lung-to-head ratio and lung volume for prediction of outcome

To prospectively examine the relationship between contralateral lung area by two-dimensional ultrasound and contralateral and total fetal lung volume (FLV) by MRI in the assessment of fetuses with isolated CDH. Sixty-six fetuses with isolated CDH were included. Contralateral fetal lung area was measured by 2D ultrasound (longest axis method). Ipsilateral, contralateral and total FLV were measured using multiplanar axial T2-WI MRI. Regression analysis was used to determine the significance of the association between contralateral lung area and total FLV and subsequently the predicted total FLV was calculated using a regression equation. Univariate regression analysis was used to investigate the effect on the proportionate difference between the predicted and the observed total FLV of gestational age (GA), proportionate volume of ipsilateral vs. total FLV, side of CDH, intrathoracic herniation of the liver and intra-tracheal presence of the balloon. Sixty-six fetuses underwent a total of 191 paired 2D ultrasound and MRI examinations at a median GA of 30 (range, 18–38) weeks. It was possible to visualize and measure the contralateral lung area by 2D ultrasound as well as both the ipsilateral and contralateral lung volumes by MRI in all instances. There was a significant association between contralateral lung area and total FLV (r = 0.84; P < 0.001). Univariate regression analysis showed that the proportionate difference between the predicted and the observed total FLV was significantly associated with the proportionate volume of ipsilateral vs. total FLV but not with GA, side of CDH, intrathoracic herniation of the liver or intra-tracheal presence of the balloon. In CDH, contralateral lung area measurement by 2D ultrasound correlates well with total FLV by MRI irrespective of GA, liver herniation or side of herniation. Inconsistencies between both measurements are attributable to the contribution of the ipsilateral lung to the total FLV.

Comparison of prenatal lung volume measurements associated with extracorporeal membrane oxygenation (ECMO) utilization and mortality in congenital diaphragmatic hernia.

To investigate the predictive value of mediastinal shift angle (MSA) in congenital diaphragmatic hernia (CDH). A retrospective analysis was performed on 87 fetuses with prenatally diagnosed left-sided CDH (LCDH) and 88 controls. MSA was measured on magnetic resonance imaging (MRI). Lung area to head circumference ratio (LHR), ratio of the observed/expected LHR (O/E LHR), total fetal lung volume (TFLV), and observed/expected total fetal lung volume (O/E TFLV) were also measured. Correlation of MSA with pulmonary hypertension (PH), extracorporeal membrane oxygenation (ECMO) use, duration of hospitalization and survival in neonates with CDH was analyzed. Performance of MSA in prediction of postnatal outcomes was compared with LHR, O/E LHR, TFLV, and O/E TFLV. There were significant differences in MSA values not only between the CDH group and the control group but also in CDH patients with different survival outcomes. MSA was inversely correlated with O/E LHR, O/E TFLV, and TFLV. MSA, LHR, O/E LHR, TFLV, and O/E TFLV could all be used to predict survival of CDH patients. In addition, the receiver operating characteristic (ROC) curve showed that the test performance of MSA was similar to that of TFLV, O/E TFLV, and O/E LHR, but superior to that of LHR. MSA was also correlated with PH, need for ECMO support, and duration of hospitalization. MRI measurement of MSA can provide various prognostic information for prenatally diagnosed LCDH, in addition to postnatal survival. The test performance of MSA is similar to TFLV, O/E TFLV, and O/E LHR. • Mediastinal shift angle (MSA) can be measured quickly and reproducibly on MRI images. • MSA could provide more prognostic information other than postnatal survival for LCDH with good test performance. • MSA should be incorporated into prenatal risk stratification for LCDH to improve planning of postnatal management.

To prospectively examine the relationship between contralateral lung area measured by two-dimensional (2D) ultrasound examination and contralateral and total fetal lung volume (FLV) estimated by magnetic resonance imaging (MRI) in the assessment of fetuses with congenital diaphragmatic hernia (CDH). Sixty-six fetuses with isolated CDH were entered in this prospective study. Contralateral fetal lung area was measured by 2D ultrasonography using the longest axis method. Ipsilateral, contralateral and total FLV were measured using multiplanar axial T2-weighted MRI. Regression analysis was used to determine the significance of associations between contralateral lung area and contralateral and total FLV, and the predicted total FLV was subsequently calculated using the regression equation. Univariate regression analysis was used to investigate the effect on the proportionate difference between the predicted and the observed total FLV of gestational age, proportionate volume of ipsilateral vs. total FLV, side of CDH, intrathoracic herniation of the liver and intratracheal presence of a balloon. The 66 fetuses underwent a total of 191 paired 2D ultrasound and MRI examinations at a median gestational age of 30 (range, 18-38) weeks. It was possible to visualize and measure the contralateral lung area by 2D ultrasound, as well as both the ipsilateral and contralateral lung volumes by MRI, in all instances. There was a significant association between contralateral lung area and contralateral lung volume (r = 0.86; P < 0.001) and with total FLV (r = 0.84; P < 0.001). Univariate regression analysis showed that the proportionate difference between the predicted and the observed total FLV was significantly associated with the proportionate volume of ipsilateral vs. total FLV but not with gestational age, side of CDH, intrathoracic herniation of the liver or intratracheal presence of the balloon. In CDH, contralateral lung area measurement by 2D ultrasound correlates well with the total FLV estimated by MRI, irrespective of gestational age, liver herniation or side of herniation. Inconsistencies between the two measurements are attributable to the contribution of the ipsilateral lung to the total lung volume.

To assess and compare the value of antenatally determined observed-to-expected (O/E) lung-area-to-head-circumference ratio (LHR) on ultrasound examination vs O/E total fetal lung volume (TFLV) on magnetic resonance imaging (MRI) examination to predict postnatal survival of fetuses with isolated, expectantly managed left-sided congenital diaphragmatic hernia (CDH). This was a multicenter retrospective study including all consecutive fetuses with isolated CDH that were managed expectantly in Mannheim, Germany, and in five other European centers, that underwent at least one ultrasound examination for measurement of O/E-LHR and one MRI scan for measurement of O/E-TFLV during pregnancy. All MRI data were centralized, and lung volumes were measured by two experienced operators blinded to the pre- and postnatal data. Multiple logistic regression analyses were performed to examine the effect on survival at hospital discharge of various perinatal variables, including the center of management. In left-sided CDH with intrathoracic herniation of the liver, receiver-operating-characteristics (ROC) curves were constructed separately for cases from Mannheim and the other five European centers and were used to compare O/E-TFLV and O/E-LHR in the prediction of postnatal survival. From Mannheim, 309 patients were included with a median gestational age (GA) at ultrasound examination of 29.6 (range, 19.7-39.1) weeks and median GA at MRI examination of 31.1 (range, 18.0-39.9) weeks. From the other five European centers, 116 patients were included with a median GA at ultrasound examination of 26.7 (range, 20.6-37.6) weeks and median GA at MRI examination of 27.7 (range, 21.3-37.9) weeks. Regression analysis demonstrated that the survival rates at discharge were lower in left-sided CDH (odds ratio (OR), 0.349 (95% CI, 0.133-0.918), P = 0.033) and those with intrathoracic liver (OR, 0.297 (95% CI, 0.141-0.628), P = 0.001), and higher with increasing O/E-TFLV (OR, 1.123 (95% CI, 1.079-1.170), P < 0.001), advanced GA at birth (OR, 1.294 (95% CI, 1.055-1.588), P = 0.013) and when birth occurred in Mannheim (OR, 7.560 (95% CI, 3.368-16.967), P < 0.001). Given the difference in survival rate between Mannheim and the five other European centers, ROC curve comparisons between the two imaging modalities were presented separately. For cases of left-sided CDH with intrathoracic herniation of the liver, pairwise comparison showed no significant difference between the area under the ROC curves for the prediction of postnatal survival between O/E-TFLV and O/E-LHR in Mannheim (mean difference = 0.025, P = 0.610, standard error = 0.050), whereas there was a significant difference in the other European centers studied (mean difference = 0.056, P = 0.033, standard error = 0.056). In fetuses with left-sided CDH and intrathoracic herniation of the liver, the predictive value for postnatal survival of O/E-TFLV on MRI examination and O/E-LHR on ultrasound examination was similar in one center (Mannheim), but O/E-TFLV had better predictive value compared to O/E-LHR in the five other European centers. Hence, in these five European centers, MRI should be included in the diagnostic process for left-sided CDH. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

To investigate the prognostic value of increased nuchal translucency (NT) thickness in the first trimester in fetuses with isolated congenital diaphragmatic hernia (CDH). We conducted a retrospective study of all consecutives cases of CDH referred to our unit for prenatal care between January 2004 and June 2010. NT was measured at between 11 and 13 + 6 weeks during first trimester routine scan therefore blinded to the subsequent diagnosis and outcome. Cases with termination of pregnancy or associated genetic, chromosomal or anatomical anomalies were discarded. We investigated the prognostic value of enlarged NT as well as the correlation between NT (MoM) and liver location, side of CDH, lung-to-head ratio (LHR), LHR observed/expected (O/E), lung volume estimated by MRI. Perinatal outcome was defined as survival at discharge from the NICU. 83 cases were reviewed. Enlarged NT, defined as > 95th percentile occurred in 9/83 (11%) cases. Neonatal death occurred in 7/9 (78%) cases with increased nuchal translucency, compared with 35/74 (47%) cases with normal translucency. No significant correlations were found between the value of NT (MoM) and the LHR, LHR O/E, lung volume estimated by MRI. The median NT was 1 MoM and 0.9 MoM for right-sided and left-sided CDH respectively (P = 0.17). Median NT was 1 MoM and 0.86 MoM for liver-up and liver-down cases respectively (P = 0.05) Nuchal translucency may be a prognostic factor for CDH. Furthermore, the prognostic value of enlarged first trimester NT may be unrelated to other markers of lung compression except liver location.

To review the effect of fetoscopic endoluminal tracheal occlusion (FETO) at 26–29 w on fetuses with severe congenital diaphragmatic hernia (CDH) and define a strategy for future clinical trials. Eligibility for FETO was single fetus with severe CDH (lung-to-head ratio (LHR) < 1 and intrathoracic liver) without associated problems. FETO was at 26–29 w gestational age (GA). Outcome measure was postnatal pulmonary outcome i.e. survival withoutor death due to pulmonary hypoplasia and/or hypertension. Regression analysis was used to investigate the effect on survival of LHR prior to FETOgestational age at FETO and at deliverypreterm amniorrhexis or prenatal removal of the balloon. 43 left and 10 right-sided CDH cases had a median LHR = 0.7 prior to FETO (range: 0.5–0.9). All but 1 operation were successful at 1st attempt with a median operation time of 10 min (range: 3–60). There were no maternal complications. Amniorrhexis occurred in 26% at < 32 wksin the last half it was 16%. Median GA at delivery was 35 wks (range 27–41). Airways were restored either perinatally (n = 18) or by planned retrieval at 34 wks (n = 35). Early neonatal survival resp. at discharge were 60% (32/53) and 51% (27/53). LHR prior to FETO is predicting survivalincreasing from 13% for LHR < 0.6 (n = 8) to 52% for LHR of 0.6⩽LHR < 0.8 (n = 25) and 65% for 0.8⩽LHR < 1 (n = 20). Left sided lesions did better than right sided. A learning curve is suggested by improving PPROM rates and GA at delivery (37 wks in the latter two thirds of a single surgeon experience with FETO (22/33). Survival after FETO is dependent on preoperative LHR and we suggest to stratify likewise. When LHR < 0.6 survival rate is only 13%therefore pulmonary response to very early FETO (< 24 w) should be studied. Survival in fetuses with CDH and 0.6⩽LHR < 1.0 is 58%. In the more common left sided group we suggest randomisation against optimal postnatal care.

Objective To explore the prenatal diagnosis and factors influencing prognosis of congenital diaphragmatic hernia (CDH).Methods Fifty seven cases of CDH prenatally diagnosed by ultrasound in the Department of Obstetrics,Xinhua Hospital from January 1,2006 to March 31,2013 were retrospectively reviewed.Prognosis and risk factors were analyzed by ultrasound characteristics and newborn status.Statistical analysis was performed using t test,x2 test and Logistic regression.Results Forty-five (79％)of all cases were left-side CDH and the rest 12 (21％)were right-side.Fourteen fetuses were aborted.Thirty three were delivered and treated surgically,twenty-three infants survived after surgery and ten died,with an overall survival rate of 40％ (23/57).The other ten cases were delivered,but died of no treated surgically.The birth weight of the surviving neonates was heavier than that of those who died [(3 173 ±348) vs (2 846±568)g,t=2.238,P=0.033].The gcstational age at diagnosis of the aborted fetuses was younger than that of the surviving neonates [(24.0 ± 4.1) vs (30.0± 6.0) weeks,t=3.181,P=0.003].Twenty-one (91％,21/23) of the surviving neonates had a lung-to-head ratio (LHR) ≥ 1.4.A linear trend test showed a negative correlation between LHR and adverse outcome (x2=9.223,P=0.002) with a correlation coefficient-0.730 (P=0.000).LHR＜1.4 (OR=35.867,95％CI:1.861-691.341,P=0.018) and polyhydramnios (OR=9.285,95％CI:1.102-78.212,P=0.040) were risk factors for neonatal death.Right-side CDH (OR=0.158,95％CI:0.009-2.824,P=0.210),liver herniation into the thorax (OR=1.730,95％CI:0.144 20.707,P=0.665) and birth weight (OR=l.002,95％CI:0.999-1.005,P=0.135) were not risk factors.Conclusions The survival rate of CDH is still low.Gestational age on diagnosis,presence of polyhydramnios and LHR＜ 1.4 predict an adverse outcome. Key words: Hernia, diaphragmatic; Ultrasonography, prenatal; Prognosis

To investigate the potential value of antenatally determined total fetal lung volume (TFLV) by magnetic resonance imaging (MRI) in the prediction of the postnatal survival in congenital diaphragmatic hernia (CDH). We examined fetuses with isolated CDH, in which MRI was used at 22-38 weeks of gestation to measure TFLV and assess intrathoracic herniation of abdominal viscera, that were liveborn after 30 weeks of gestation and had postnatal follow-up until death or discharge from hospital. Regression analysis was used to investigate the effect on survival of gestational age at diagnosis, observed to expected (o/e) TFLV, intrathoracic herniation of the liver, side of CDH, gestational age at MRI, institution, year and gestational age at delivery. In 76 fetuses measurements of o/e TFLV and the lung area to head circumference ratio (LHR) were performed within 2 weeks of each other; in these cases o/e TFLV and o/e LHR were compared for their prediction of postnatal survival. In the 148 cases that fulfilled the entry criteria, multiple regression analysis demonstrated that significant predictors of survival were the presence or absence of intrathoracic herniation of the liver and o/e TFLV. The area under the receiver-operating characteristics curves for prediction of postnatal survival from o/e TFLV was 0.786 (standard error, 0.059; P < 0.001) and that from o/e LHR was 0.743 (standard error, 0.069; P = 0.001). In the assessment of fetuses with CDH, MRI-based o/e TFLV is useful in the prediction of postnatal survival.