Total Fetal Lung Volume Research Articles

Prenatal assessment of congenital diaphragmatic hernia at north american fetal therapy network centers: A continued plea for standardization.

Prenatal work-up for congenital diaphragmatic hernia (CDH) is important for risk stratification, standardization, counseling, and optimal therapeutic choice. To determine current practice patterns regarding prenatal CDH work-up, including prenatal ultrasound and magnetic resonance imaging (MRI) use, and to identify areas for standardization of such evaluation between fetal centers. A survey regarding prenatal CDH work-up was sent to each member center of the North American Fetal Therapy Network (NAFTNet) (n = 36). All responded. Sonographic measurement of lung-to-head ratio (LHR) was determined by all, 89% (32/36) of which routinely calculate observed-to-expected LHR. The method for measuring LHR varied: 58% (21/36) used a "trace" method, 25% (9/36) used "longest axis," and 17% (6/36) used an "antero-posterior" method. Fetal MRI was routinely used in 78% (28/36) of centers, but there was significant variability in fetal lung volume measurement. Whereas all generated a total fetal lung volume, the planes, methodology and references values varied significantly. All evaluated liver position, 71% (20/28) evaluated stomach position and 54% (15/28) quantified the degree of liver herniation. More consistency in workup was seen between centers offering fetal intervention. Prenatal CDH work-up and management differs considerably among North American fetal diagnostic centers, highlighting a need for its standardization.

Prenatal prediction of postnatal survival in fetuses with congenital diaphragmatic hernia using MRI: lung volume measurement, signal intensity ratio, and effect of experience

Objective To evaluate various signal intensity ratios in isolated congenital diaphragmatic hernia (CDH) and to compare their potential in predicting survival with that of the observed-to-expected (O/E) ratio of total fetal lung volume (TFLV) using magnetic resonance imaging (MRI) measurements. Our second objective was to evaluate the impact of operator’s experience in comparing the prediction of postnatal survival by O/E-TFLV. Methods In 75 conservatively managed CDH fetuses and in 50 who underwent fetoscopic endoluminal tracheal occlusion (FETO), the fetal lung-to-amniotic fluid, lung-to-liver, lung-to-muscle, lung-to-spinal fluid signal intensity ratios, respectively LAFSIR, LLSIR, LMSIR, and LSFSIR, were measured, as was O/E-TFLV. Receiver operating characteristic (ROC) curves were constructed and used to compare the various signal intensity ratios with O/E-TFLV in the prediction of postnatal survival. In 72 MRI lung volumes assessed by the referring radiologists in Paris and Lille and secondarily by our expert radiologist in Brussels (M.M.C.) using the same MRI examinations, ROC curves were constructed and used to compare the value of O/E-TFLV determined by the two centers in the prediction of postnatal survival. Results In the total cohort of CDH fetuses, O/E-TFLV and LLSIR were predictive of postnatal survival whereas in the conservatively managed group O/E-TFLV, LLSIR, and LMSIR predicted postnatal survival. O/E-TFLV predicted postnatal survival far better than the signal intensity ratios: area under the ROC curve for prediction by O/E-TFLV in the total cohort was 0.866 (p < .001; standard error = 0.031). The area under the ROC curve for prediction of postnatal survival using O/E-TFLV by MRI evaluated at the referral centers was 0.640 (p = 102; standard error = 0.085), and with O/E-TFLV reevaluated by M.M.C., it was 0.872 (p < .001; standard error = 0.061). Pairwise comparison showed a significant difference between the areas under the ROC curves (difference = 0.187, p = .012; standard error = 0.075). Conclusion In fetuses with CDH with/without FETO, LLSIR was significantly correlated with the prediction of postnatal survival. However, measurement of O/E-TFLV was far better in predicting postnatal outcome. Operator experience in measurement of lung volumes using MRI seem to play a role in the predictive value of the technique.

Standard Normal Fetal Lung Volume by MRI Measurement

Background: Pulmonary hypoplasia is a significant decrease in lung volume by any cause in comparison to the normal volume for gestational age. Objectives: To evaluate the application of prenatal magnetic resonance imaging in order to predict pulmonary hypoplasia, measurement of fetal lung volume (FLV) was aimed to establish a reference data in normal fetuses. Patients and Methods: Three hundred forty two fetuses with abnormal ultrasound findings underwent fast spin-echo T2-weighted MR imaging at a single tertiary care hospital (Shahid Faghihi Hospital, Shiraz, Iran). Data of 241 fetuses at 18 - 36 weeks’ gestation without thoracic malformations were obtained for a FLV normative curve. To acquire a best-fit formula for the mean total fetal lung volume based on each gestational week with 95% confidence interval (CI), we used the regression model applied by Rypens et al. This resulted in a formula calculating the expected fetal lung volume based on gestational age. A one-sample t-test was also carried out to compare the mean total fetal lung volume at each gestational week with the expected mean total fetal lung volume predicted by the formula by Rypens et al., Meyers et al., and Osada et al. Results: Normal FLV increased with gestational age as the spread of values. The expected fetal lung volume was derived from the following formula: Fetal lung volume (mL) = 0.002 × (gestational age [GA]2.913), in which GA is gestational weeks. FLV in our population had a more consistent correlation with Japanese fetuses than European or American fetuses. Conclusion: In fetuses with normal lungs, FLV distribution against gestational age is simply measured prenatally with MR imaging. A single universal formula might not be suitable for fetuses worldwide. In our region, acquired data were more consistent with Asian normal values.

Fetal MRI assessment of mediastinal shift angle in isolated left congenital diaphragmatic hernia: A new postnatal survival predictive tool?

To quantify the mediastinal shift angle (MSA) in fetuses with isolated left congenital diaphragmatic hernia (CDH) by magnetic resonance imaging and evaluate survival. Fetuses from singleton pregnancies with isolated left CDH were matched for gestational age with controls without thoracic malformations. For all fetuses the MSA was determined by two operators and inter-operator variability and differences between cases and controls were investigated. For all cases total fetal lung volume (TFLV) was calculated and the correlation between MSA and TFLV was assessed, and its predictive value towards survival was determined. Thirty-four fetuses were included as cases and 42 as controls. The mean gestational age for assessment of CDH fetuses was 32 weeks (range 27-38). Twenty-four fetuses survived until discharge and 10 did not. There was an excellent inter-operator reliability for measuring the MSA and a significant difference between MSA in cases and controls. There was an inverse correlation between MSA values and survival, a correlation between TFLV and survival and an inverse correlation between MSA and TFLV. The area under the ROC curve for MSA in predicting survival was 0.931 (95% CI 0.851-1.000). The MSA measured late in gestation correlates with postnatal survival in patients with isolated left CDH.

Calculating Observed-to-Expected Total Fetal Lung Volume in CDH Fetuses in Twin Gestation: Is There a Better Way?

Background: Congenital diaphragmatic hernia (CDH) is a potentially lethal birth defect, and identifying prenatal predictors of outcome is important. Observed-to-expected total fetal lung volume (o/e TFLV) has been shown to be a predictor of severity and useful in risk stratification but is variable due to different TFLV formulas. Objectives: To calculate o/e TFLV for CDH patients part of a twin gestation using the unaffected sibling as an internal control and comparing these values to those calculated using published formulas for TFLV. Methods: Seven twin gestations with one fetus affected by CDH were identified between 2006 and 2017. The lung volume for each twin was calculated using magnetic resonance imaging (MRI), and o/e TFLV was calculated using the unaffected twin’s TFLV. This percentage was then compared to the o/e TFLV calculated using published formulas. Results: Lung volumes in the unaffected twins were within normal ranges at the lower end of the spectrum. No single TFLV formula was found to correlate perfectly. Intraclass correlation coefficient estimate was most consistent for o/e TFLV calculated with the Meyers formula and supported by Bland-Altman plots. Conclusions: O/e TFLV measured in CDH/non-CDH twin gestations using the unaffected sibling demonstrated agreement with o/e TFLV calculated using the Meyers formula. We urge the fetal community to standardize the method, use, and interpretation of fetal MRI in the prenatal evaluation of CDH.

Timing of Prenatal Magnetic Resonance Imaging in the Assessment of Congenital Diaphragmatic Hernia

Introduction: Fetal magnetic resonance imaging (MRI) has been used to stratify severity of congenital diaphragmatic hernia (CDH) after ultrasound diagnosis. The purpose of this study was to determine if timing of MRI influenced prediction of severity of outcome in CDH. Methods: A single institution retrospective review of all CDH referred to our institution from February 2004 to May 2017 was performed. Patients were included if they underwent at least 2 fetal MRIs prior to delivery. Prenatal MRI indices including observed-to-expected total fetal lung volume (o/e TFLV) were evaluated. Indices were categorized by trimester, either 2nd (20–27 weeks gestation) or 3rd trimester (>28 weeks gestation) and further analyzed for outcome predictability. Primary outcomes were survival, extracorporeal membrane oxygenation (ECMO), and pulmonary hypertension (PAH). Student t test and logistic and linear regression were used for data analyses. Results: Of 256 fetuses evaluated for CDH, 197 were further characterized by MRI with 57 having both an MRI in the 2nd and 3rd trimesters. There was an average of 9.95 weeks (±4.3) between the 1st and 2nd MRI. Second trimester o/e TFLV was the only independent predictor of survival by logistic regression (OR 0.890, p < 0.01). Third trimester MRI derived lung volumes were associated with, and independent predictors of, severity of PAH and need for ECMO. Interval TFLV growth was a strong predictor of PAH postnatally (OR 0.361, p < 0.01). Overall cohort survival was 79%. Conclusion: Accuracy of MRI lung volumes to predict outcomes is dependent on the ­gestational age at the time of exam. While MRI lung volumes at either the 2nd or 3rd trimester are predictive of morbidity, 2nd trimester lung volumes strongly correlated with mortality.

A multifactorial severity score for left congenital diaphragmatic hernia in a high-risk population using fetal magnetic resonance imaging.

Adverse outcomes for infants born with left congenital diaphragmatic hernia (CDH) have been correlated with fetal imaging findings. We sought to corroborate these correlations in a high-risk cohort and describe a predictive mortality algorithm combining multiple imaging biomarkers for use in prenatal counseling. We reviewed fetal MRI examinations at our institution from 2004 to 2016 demonstrating left-side CDH. MRI findings, hospital course and outcomes were recorded and analyzed using bivariate and multivariable analysis. We generated a receiver operating curve (ROC) to determine a cut-off relation for mortality. Finally, we created a predictive mortality calculator. Of 41 fetuses included in this high-risk cohort, 41% survived. Per bivariate analysis, observed-to-expected total fetal lung volume (P=0.007), intrathoracic position of the stomach (P=0.049), and extracorporeal membrane oxygenation (ECMO) requirement (P<0.001) were significantly associated with infant mortality. Youden J statistic optimized the ROC for mortality at 24% observed-to-expected total fetal lung volume (sensitivity 64%, specificity 82%, area under the curve 0.72). On multivariable analysis, observed-to-expected total fetal lung volume ± 24% was predictive of mortality (adjusted odds ratio, 95% confidence interval: 0.09 [0.02, 0.55]; P=0.008). We derived a novel mortality prediction calculator from this analysis. In this high-risk cohort, decreased observed-to-expected total fetal lung volume and stomach herniation were significantly associated with mortality. The novel predictive mortality calculator utilizes information from fetal MR imaging and provides prognostic information for health care providers. Creation of similar predictive tools by other institutions, using their distinct populations, might prove useful in family counseling, especially where there are discordant imaging findings.

Prenatal prediction of survival in congenital diaphragmatic hernia: An audit of postnatal outcomes

PurposeEffective antenatal counseling in congenital diaphragmatic hernia (CDH) relies on proper measurement of prognostic indices. This quality initiative audited the accuracy of prenatal imaging with postnatal outcomes at two tertiary pediatric referral centers. MethodsPrenatal lung–head ratio (LHR) and total fetal lung volume (TFLV) for CDH patients treated between 2006 and 2017 were retrieved. Study inclusion required at least one LHR or TFLV measurement between 24 and 32 weeks gestational age. Postnatal outcomes [mortality, extracorporeal life support (ECLS) need, patch repair, persistent pulmonary hypertension, oxygen requirement at 28 days] were abstracted from the Canadian Pediatric Surgery Network (CAPSNet) database and local chart review. Univariate and descriptive analyses were conducted. ResultsEighty-two of 121 eligible CDH patients (68%) were included. Overall mortality, ECLS rates, and patch repair were 33%, 12.5%, and 45%, respectively. Lower LHR values correlated with increased rates of each outcome and persisted despite multiple measurements. Values obtained were higher than those in published schemata. LHR values >45% were most associated with survival, avoidance of ECLS, and primary repair. TFLV values only correlated with mortality and patch repair. ConclusionsThis audit confirms that LHR and TFLV values predict CDH outcomes. However, absolute values obtained require careful interpretation and internal review. Level of evidenceIV.

Antenatal management and outcomes of pregnancies with congenital diaphragmatic hernia

The objective of this study is to evaluate the obstetric outcomes of pregnancies with congenital diaphragmatic hernia (CDH). Fifty one pregnancies prenatally diagnosed with CDH at our institution between January 1, 2002 and August 31, 2018 were retrospectively evaluated. The pregnancies were divided into two groups according to neonatal survival. Demographic features, clinical characteristics and prognostic factors were compared between the neonatal survival (n = 16) and non-survival (n = 28) groups. Cut-off values of fetal lung area to head circumference ratio (LHR), observed/expected LHR (o/e LHR) and observed/expected total fetal lung volume (o/e TFLV) for neonatal survival were calculated. Thirty six (70.6%) and fifteen (29.4%) fetuses had left and right sided CDH respectively. Seven patients chose termination of their pregnancies (13.7%). Statistically significant differences were found between survival and non-survival groups in terms of parity, median gestational week at diagnosis, polyhydroamniosis rate, CDH type, stomach position, liver position, median LHR, o/e LHR, o/e TFLV, median 5th minute Apgar score and neonatal operation rate values (p values were 0.03,<0.001, 0.02, 0.006,<0.001, 0.006,<0.001,<0.001,<0.001, 0.04 and <0.001 respectively). According to ROC curve analysis, 1.05 (82% sensitivity, 74% specificity) for LHR, 22.5 (78.6% sensitivity, 73.9% specifity) for o/e LHR and 23.5 (85.7% sensitivity,74.2% specificity) for o/e TFLV were determined to be cut-offs for neonatal survival, respectively, with highest sensitivity and specificity. Earlier gestational week at diagnosis, right sided CDH, presence of liver herniation, supradiaphragmatic stomach position, lower LHR, o/e LHR and o/e TFLV were associated with decreased rates of neonatal survival.

Fetal brain morphometry on prenatal magnetic resonance imaging in congenital diaphragmatic hernia

Many infants with congenital diaphragmatic hernia (CDH) show brain abnormality on postnatal brain MRI related to severity of CDH, degree of lung hypoplasia, intrathoracic liver, right diaphragmatic hernia and large diaphragmatic defect. It is not known whether these factors affect brain growth in utero in CDH. To assess prenatal brain morphometry and abnormalities on fetal MR in congenital diaphragmatic hernia. We retrospectively reviewed 109 fetal MRIs in 63 fetuses with CDH from 2009 to 2014 (27 died before discharge, 36 survived to discharge). We compared brain injury and gestational-age-corrected z-scores of brain measurements between survivors and non-survivors. We assessed correlations between brain abnormalities and CDH severity. Enlarged extraaxial space was the most common abnormality, frequently seen on fetal MRI at >28weeks of gestation, similar in survivors versus non-survivors. Anteroposterior cerebellar vermis dimension at >28weeks of gestation was smaller in non-survivors compared to survivors (P=.02) and positively correlated with observed/expected total fetal lung volume (P=.01). Transverse cerebellar diameter at >28weeks of gestation was also positively correlated with observed/expected total fetal lung volume (P=.04). We did not identify maturational delay, abnormal parenchymal signal or intracranial hemorrhage on fetal MRI. Enlarged extraaxial spaces in the third trimester was the most common abnormality on fetal MRI in congenital diaphragmatic hernia. Cerebellar dimensions on fetal MRI are associated with CDH severity. There was no major brain parenchymal injury on fetal MRI, even in the third trimester, in CDH survivors and non-survivors.

Early Postnatal Ventricular Dysfunction Is Associated with Disease Severity in Patients with Congenital Diaphragmatic Hernia

To assess patterns of postnatal ventricular function and their relationship to prenatal and postnatal markers of disease severity in infants with congenital diaphragmatic hernia (CDH). In this observational case-control study of cardiac function in infants with CDH in the first 5 days of life, systolic and diastolic function in the right ventricle (RV) and left ventricle (LV) were assessed using speckle tracking echocardiography-derived global strain and tissue Doppler imaging. Correlation between cardiac function and prenatal observed:expected total fetal lung volume (TFLV), oxygenation index (OI), duration of intubation, and hospital length of stay were assessed. All measures of systolic and diastolic function were significantly reduced in the CDH group (n = 25) compared with controls (n = 20) at <48 hours, and were improved by 72-120 hours. LV global systolic longitudinal strain (GLS) correlated with prenatal TFLV (R2 = 0.32; P = .03), OI (R2 = 0.35; P < .001), duration of intubation (R2 = 0.24; P = .04), and length of stay (R2 = 0.4; P = .006). Mean (SD) LV GLS at <48 hours was significantly lower in infants with CDH who did not survive and/or required ECMO compared with those who did not: -11.5 (5.3)% vs -16.9 (5.3)% (P = .02). RV and LV function are impaired in the transitional period in infants with CDH. Early LV systolic function correlates with prenatal and postnatal markers of clinical disease severity and may be an important determinant of disease severity and therapeutic target in CDH. These findings support regular assessment of cardiac function in CDH and investigational trials of targeted cardiovascular therapies.

Fetal Lung Volumes by MRI: Normal Weekly Values From 18 Through 38 Weeks' Gestation.

The purpose of this study is to establish normal total fetal lung volume reference ranges from 18 to 38 weeks' gestation at 1-week intervals. A retrospective analysis of 665 patients who underwent fetal MRI at two tertiary fetal centers from 2001 to 2013 was performed. Total fetal lung volume was measured in at least two planes, either manually or using a 3D workstation. The mean, median, SD, minimum, maximum, and lower and upper quartiles for total fetal lung volume were determined per gestational week. A t test was used to compare our values to previously reported values. A new formula to calculate total fetal lung volume derived from our data was created using a regression model. Comparisons between total fetal lung volume obtained by different imaging planes and manual versus semiautomatic calculation were also performed. The mean normal total fetal lung volume showed a weekly increase from 18 through 35 weeks' gestation. Means were compared with the expected total fetal lung volume generated by the Rypens formula, showing statistically significant lower mean total fetal lung volume from week 19 to week 22 (p < 0.05). Comparison between our data-derived total fetal lung volume formula and the Rypens formula showed very similar values at every gestational age. No difference in total fetal lung volume was seen when comparing imaging planes or manual versus semiautomatic methods. Measured mean total fetal lung volume values at 19-22 weeks are significantly lower than those predicted by the Rypens formula. Therefore, we propose preferential use of our values for prenatal counseling and delivery planning.

Neurodevelopmental outcomes in CDH survivors: A single institution's experience

PurposeSurvivors of congenital diaphragmatic hernia (CDH) face high morbidity. We studied the neurodevelopmental outcomes of CDH survivors at a single institution. MethodsCDH survivors born July 2006–March 2016 at a free-standing children's hospital were reviewed. Neurodevelopment was assessed using the Peabody Developmental Motor Scales (PDMS-2) broken into gross, fine, and total motor quotients. Data collected included prenatal variables (liver herniation, defect laterality, observed:expected total fetal lung volume (o:eTFLV) on MRI), birth demographics (sex, race, estimated gestational age (EGA), birth weight (BtWt), 5 min APGAR, associated anomalies), and therapies/hospital course (HFOV/HFJV, ECMO, timing of repair, pulmonary hypertension (PHTN) severity, length of stay, ventilator days). Variables were analyzed using mixed linear modeling. ResultsSixty-eight children were included. Most patients had left-sided CDH (55/68, 81%) without liver herniation (42/68, 62%). ECMO utilization was 25/68 (37%). The mean [95% confidence interval] gross motor quotient for the entire cohort was 87 [84–91], fine motor quotient was 92 [88–96], and total motor quotient was 88 [84–93], representing below average, average, and below average functioning, respectively. o:eTFLV predicted fine motor quotient among prenatal variables. Associated anomalies and ECMO use predicted all quotients in the final model. ConclusionsAssociated anomalies and ECMO use predict neurodevelopmental delay in CDH survivors. Type of StudyRetrospective observational study; Prognostic. Level of EvidenceII.

Predictive value of oxygenation index for outcomes in left-sided congenital diaphragmatic hernia

Background & objectivesCongenital Diaphragmatic Hernia (CDH) is associated with significant morbidity and mortality. This study compares the efficacy of the highest oxygenation index in the first 48 h (HiOI) versus current prenatal indices to predict survival and morbidity. MethodsMedical records of 50 prenatally diagnosed, isolated, left-sided CDH patients treated from January 2011 to April 2016 were reviewed. Data abstracted included HiOI, lung to head ratio (LHR), observed to expected total fetal lung volume (O/E TFLV), percent liver herniation (%LH), 6 month survival, respiratory support at discharge, ventilator days and length of stay. Data were analyzed using parametric and nonparametric tests and regression analyses as appropriate. ResultsHiOI was associated with significantly increased LOS (p<0.001), respiratory support at discharge (p<0.001), greater ventilator days (p=0.001) and higher odds of death (p=0.004) with risk of death increasing by 5% for every one-unit increase in OI. HiOI was statistically a better predictor of LOS than O/E TFLV (p=0.007) and %LH (p=0.02). ConclusionsIn isolated, left-sided CDH patients, HiOI is associated with higher mortality, greater length of stay, more ventilator days and increased respiratory support at discharge. HiOI is a better predictor of length of stay than O/E TFLV and %LH. Type of studyRetrospective Study Level of evidenceII

In vivo evidence by magnetic resonance volumetry of a gestational age dependent response to tracheal occlusion for congenital diaphragmatic hernia

We aimed to assess in vivo changes in lung and liver volumes in fetuses with isolated congenital diaphragmatic hernia, either expectantly managed or treated in utero. This is a secondary analysis of prospectively collected data at two fetal therapy centers. We used archived magnetic resonance images of fetuses taken ≥7 days apart, creating paired observations in 20 expectantly managed cases, 41 with a second magnetic resonance prior to balloon reversal and 64 after balloon removal. We measured observed to expected total fetal lung volume (O/E TFLV) and liver-to-thoracic volume ratio. We calculated changes in volume as compared with the initial measurement and its rate as a function of gestational age (GA) at occlusion. The liver-to-thoracic volume ratio did not change in either group. In expectantly managed fetuses, O/E TFLV did not increase with gestation. In fetuses undergoing tracheal occlusion, the measured increase in volume was 2.6 times larger with balloon in place as compared with that after its removal. GA at tracheal occlusion was an independent predictor of the O/E TFLV. The net rate seems to initially increase and plateau at a maximum of 1.5% per week by 35 to 45 days after occlusion. Tracheal occlusion induces a net increase in volume, its magnitude essentially dependent on the GA at occlusion.

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.

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). 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. 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. 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.

Prenatal prediction of pulmonary arterial hypertension in congenital diaphragmatic hernia

To evaluate the role of prenatal prognostic markers obtained routinely by ultrasound examination and magnetic resonance imaging (MRI) in the prediction of development of postnatal pulmonary arterial hypertension (PAH) in isolated congenital diaphragmatic hernia (CDH). One hundred and ten cases of isolated CDH were referred to our fetal medicine unit between January 2004 and April 2013. Mortality and morbidity rates were reviewed for those presenting with postnatal PAH. The following prenatal markers were evaluated as potential predictive factors of PAH: liver position, side of the CDH defect, lung area to head circumference ratio (LHR) and observed/expected LHR (o/e-LHR), which were measured by ultrasound, and observed/expected total fetal lung volume (o/e-TFLV), which was measured by MRI. Univariable logistic regression was used to assess associations. PAH was significantly associated with perinatal mortality and morbidity (P < 0.001). The occurrence of PAH decreased significantly with an increasing LHR, o/e-LHR and o/e-TFLV and was significantly increased for cases with an intrathoracic liver, but not for those with right-sided defects. Univariable regression revealed that o/e-TFLV (odds ratio (OR), 0.9 (95% CI, 0.86-0.95); P < 0.05 for percentage unit change in o/e), LHR (OR, 0.19 (95% CI, 0.09-0.40); P < 0.05 for unit change), o/e-LHR (OR, 0.95 (95% CI, 0.93-0.98); P < 0.05 for percentage unit change in o/e) and liver position (OR, 2.82 (95% CI, 1.13-7.00); P < 0.05 for intrathoracic liver) were significant predictors of subsequent PAH. No differences were found after adjusting for gestational age at delivery. The areas under the receiver-operating characteristics curve were 0.80 and 0.75 for o/e-TFLV and o/e-LHR, respectively. In cases of CDH, PAH is associated with high rates of mortality and morbidity. Routinely obtained prenatal markers, usually used for the assessment of pulmonary hypoplasia, are also relevant for the postnatal prediction of PAH.

Revisiting outcomes of right congenital diaphragmatic hernia

BackgroundStudies comparing outcomes of right- and left-sided congenital diaphragmatic hernia (R-CDH and L-CDH) have yielded conflicting results. We hypothesized that R-CDH is associated with higher short-term pulmonary morbidity than L-CDH. MethodsWe reviewed all CDH patients at a tertiary children's hospital over 10 y. In prenatally diagnosed CDH, the observed-to-expected total fetal lung volume and percentage liver herniation (%LH) were calculated using fetal magnetic resonance imaging-based measurements. Outcomes were compared in patients with isolated CDH. Patients were subsequently matched by %LH to compare outcomes. ResultsOf 189 CDH patients, 37 (20.1 %) were R-CDH and 147 (79.9%) were L-CDH. Those with R-CDH were prenatally diagnosed at a significantly lower rate (40.5% versus 73.5%; P < 0.001) and later gestational age (26.5 ± 7.7 versus 22.6 ± 5.65 wk; P = 0.062). There was no difference in observed-to-expected total fetal lung volume between those with R-CDH and L-CDH (30.2 ± 11.1% versus 33.1 ± 14.2%; P = 0.471). Fetuses with R-CDH had a higher %LH than those with L-CDH (37.5 ± 14.1% versus 18.6 ± 12.2%; P < 0.001). Patients with isolated R-CDH had a higher need for extracorporeal membrane oxygenation than L-CDH (48% versus 27%; P = 0.055). There was no difference in duration of tracheal intubation, hospital stay, need for supplemental oxygen at 30-d of life or 6-mo mortality between groups. There was no difference in mortality and pulmonary morbidity when patients were matched by %LH. ConclusionsCompared to those with L-CDH, fetuses with R-CDH are less likely to be diagnosed prenatally and have a higher need for extracorporeal membrane oxygenation. The sidedness of the hernia defect was not associated with differences in short-term pulmonary morbidity in this large, contemporary single-institution experience of neonates with CDH.

Giant omphaloceles: surgical management and perinatal outcomes

BackgroundThe purpose of this study was to describe the current management and outcomes of infants with omphalocele. MethodsThe medical records of all patients treated for omphalocele at a large children's hospital from January, 2003–February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies. Prenatal data collected included fetal magnetic resonance imaging-based observed-to-expected total fetal lung volumes. Giant omphalocele (GO) was defined as >50% of liver in the omphalocele sac. ResultsOf 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated anomalies. No live-born baby with an isolated defect (n = 19) died, whereas mortality was 41% and 17% for those with major and minor anomalies, respectively (P = 0.006). Infants with major anomalies had significantly longer median length of intubation (36 versus 0 versus 0 d; P = 0.04) and hospital stay (157 versus 28.5 versus 18 d; P < 0.001) compared with those with minor or no anomalies. Of 40 infants with GO, the majority (85%) were managed surgically by delayed closure with a median age at repair of 10 mo (range, 3.4–23.6 mo). Six-month survival was 80%. None of the delayed repair patients required a later operative revision, whereas 2 of 5 with early repair did. ConclusionsThe presence of associated anomalies is the strongest predictor of morbidity and mortality in fetuses or neonates with omphalocele. In patients with GO, delayed closure is associated with good outcomes, but larger, prospective studies comparing delayed to early closure are needed to determine the optimal timing of repair.

Are all pulmonary hypoplasias the same? A comparison of pulmonary outcomes in neonates with congenital diaphragmatic hernia, omphalocele and congenital lung malformation

IntroductionPatients with congenital diaphragmatic hernias (CDH), omphaloceles, and congenital lung malformations (CLM) may have pulmonary hypoplasia and experience respiratory insufficiency. We hypothesize that given equivalent lung volumes, the degree of respiratory insufficiency will be comparable regardless of the etiology. MethodsRecords of all fetuses with CDH, omphalocele, and CLM between January 2000 and June 2013 were reviewed. MRI-based observed-to-expected total fetal lung volumes (O/E-TFLV) were calculated. An analysis of outcomes in patients with O/E-TFLV between 40% and 60%, the most inclusive range, was performed. Results285 patients were evaluated (161, CDH; 24, omphalocele; 100, CLM). Fetuses with CDH had the smallest mean O/E-TFLV. CDH patients were intubated for longer and had a higher incidence of pulmonary hypertension. Fifty-six patients with the three diagnoses had an O/E-TFLV of 40%–60%. The need for ECMO, supplemental oxygen at 30days of life, and 6-month mortality were similar among groups. CDH patients had a significantly longer duration of intubation and higher incidence of pulmonary hypertension than the other two diagnoses. ConclusionGiven equivalent lung volumes (40%–60% of expected), CDH patients require more pulmonary support initially than omphalocele and CLM patients. In addition to lung volumes, disease-specific factors, such as pulmonary hypertension in CDH, also contribute to pulmonary morbidity and overall outcome.