Diaphragmatic herniation following total gastrectomy: review of the long-term experience of a tertiary institution.

PURPOSE: Diaphragmatic herniation (DH) is a rare but potentially fatal event after total gastrectomy (TG). Despite being life-threatening, risk factors for postoperative DH have yet to be elucidated. We conducted a retrospective analysis to identify clinical characteristics of patients developing DH after TG, along with a comprehensive review of the published literature. METHODS: Among 1361 consecutive patients undergoing TG for esophagogastric cancer between 1985 and 2013 in Toranomon Hospital, those requiring surgical intervention for postoperative DH were included. We also conducted a PubMed literature search on DH following TG. RESULTS: Five patients (four males, one female), with a median age of 68 at DH surgery, were identified. Intervals between TG and DH repair ranged from 2.9 to 189.0 (median, 78.1) months. Four patients had needed emergency surgery. Three patients had undergone open TG and two others laparoscopic TG, suggesting a significantly higher incidence of DH after laparoscopic TG (3/1302 vs. 2/59, p = 0.017). The diaphragmatic crus incision, creating the space for esophagojejunostomy, had been performed in all cases. The literature yielded seven relevant publications (16 patients). Intervals between TG and DH reduction ranged from 2 days to 36 months. All operations for DH had been carried out emergently. CONCLUSION: The risk of DH persisted after TG. DH is potentially a very late complication of TG, presenting as a surgical emergency. Laparoscopic TG was suggested to be a risk factor for postgastrectomy DH. Incising the crus might also be a predictor of DH. Measures to prevent DH, e.g., appropriate closure of the crus, would be recommended in minimally invasive TG.

An overview of medical ECMO for neonates.

Extracorporeal membrane oxygenation (ECMO), a life-saving therapy for respiratory and cardiac failure, was first used in neonates in the 1970s. The indications and criteria for ECMO have changed over the years, but it continues to be an important option for those who have failed other medical therapies. Since the Extracorporeal Life Support Organization (ELSO) Registry was established in 1989, more than 29,900 neonates have been placed on ECMO for respiratory failure, with 84% surviving their ECMO course, and 73% surviving to discharge or transfer. In this chapter, we will review the basics of ECMO, patient characteristics and criteria, patient management, ECMO complications, special uses of neonatal ECMO, and patient outcomes.

Volume and outcomes relationship in laparoscopic diaphragmatic hernia repair.

BACKGROUND: There is no published data regarding the relationship between hospital volume and outcomes in patients undergoing laparoscopic diaphragmatic hernia repair. We hypothesize that hospitals performing high case volume have improved outcomes compared to low-volume hospitals. MATERIALS AND METHODS: We reviewed the National Inpatient Sample (NIS) database between 2008 and 2012 for adults with the diagnosis of diaphragmatic hernia who underwent elective laparoscopic repair of diaphragmatic Hernia and/or Nissen fundoplication. Pediatric, emergent, and open cases were excluded. Main outcome measures included logistic regression analysis of factors predictive of in-hospital mortality and outcomes according to annual hospital case volume. RESULTS: A total of 31,228 laparoscopic diaphragmatic hernia operations were analyzed. The overall in-hospital mortality was 0.14%. Risk factors for higher in-hospital mortality included renal failure (AOR: 6.26; 95% CI: 2.48-15.78; p < 0.001), age>60 years (AOR: 5.06; 95% CI: 2.38-10.76; p < 0.001), and CHF (AOR: 3.80; 95% CI: 1.39-10.38; p = 0.009) while an incremental increase in volume of 10 cases/year (AOR: 0.89; 95% CI: 0.81-0.98; p = 0.019) and diabetes (AOR: 0.34; 95% CI: 0.12-0.93; p = 0.036) decreases mortality. There was a small but significant inverse relationship between hospital case volume and mortality with a 10% reduction in adjusted odds of in-hospital mortality for every increase in 10 cases per year. Using 10 cases per year as the volume threshold, low-volume hospitals (≤10 cases/year) had almost a twofold higher mortality compared to high-volume hospitals (0.23 vs. 0.12%, respectively, p = 0.02). CONCLUSIONS: There was a small but significant inverse relationship between the hospitals' case volume and mortality in laparoscopic diaphragmatic hernia repair.

A lethal phenotype associated with tissue plasminogen deficiency in humans.

The role of plasminogen in preventing thrombosis requires activation by tissue plasminogen activator (t-PA) encoded by PLAT. While case-control associations have been pursued for common variants in PLAT, no disease-causing mutations have been reported. We describe a consanguineous family with two children who died shortly after birth due to complications related to severe hydranencephaly and diaphragmatic hernia. A combined exome/autozygome analysis was carried out with informed consent. We identified a homozygous null mutation in PLAT that abrogated t-PA level in patient cells. This is the first reported human knockout mutation of PLAT. The apparent association with hydranencephaly, diaphragmatic hernia and postnatal lethality requires further validation.

[THE RISK FACTORS OF LETHAL COMPLICATIONS IN EARLY POSTOPERATIVE PERIOD IN PATIENTS, SUFFERING GASTROESOPHAGEAL ZONE MALIGNANCIES].

Abstract The factors, determining possibility of early postoperative morbidity occurrence in patients, suffering gastro-esophageal zone cancer, were analyzed. After radical operation performance (gastrectomy, gastric and esophageal resection) 5.7% patients died. Insufficience of the anastomosis sutures with peritonitis occurrence, an acute hepato-renal insufficience, an acute coronary syndrome, pulmonary thromboembolism, pneumonia, the brain insult, pancreonecrosis and mesenterial thrombosis constituted the main morbidities. The complications occurrence depends upon the tumoral process course severity, morphological variant of cancer, presence of concomitant diaphragmatic hernia and the blood rheological properties. Initially high indices of the blood sera present a rheological properties of blood serum may serve as a prognostic criterion of the postoperative complications occurrence in the patients.

Inhaled nitric oxide use in neonates with congenital diaphragmatic hernia.

OBJECTIVE: To describe the use of inhaled nitric oxide (INO) in newborns with congenital diaphragmatic hernia (CDH). METHODS: Pediatric Health Information System data were queried for newborns with CDH admitted at <8 days of age at tertiary care US pediatric hospitals between 2003 and 2011. INO treatment status and timing in relation to CDH repair were determined for each infant. Hospital-specific rates of INO use, extracorporeal membrane oxygenation (ECMO) use, and mortality were determined. RESULTS: Data were analyzed for 1713 neonates with CDH admitted to 33 hospitals. More than half (57%) received INO during their inpatient stay, and utilization varied dramatically between hospitals (34% to 92%). Neonates treated with INO accumulated >$81 million in pharmacy charges. The proportion of infants receiving INO as well as their duration of therapy increased significantly during the study period. The rate of ECMO utilization and mortality did not change significantly during the study period. Hospital-specific mortality rates did not correlate with INO therapy, ECMO utilization, or case volume. CONCLUSIONS: INO use in neonates with CDH is widespread, and has increased at many US tertiary pediatric hospitals without contemporaneous change in ECMO utilization or mortality. The improvement of evidence-based guidelines for the use of INO in newborns with CDH could lead to a reduction in health care costs for these patients.

A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a condition with a highly variable outcome. Some infants have a relatively mild disease process, whereas others have significant pulmonary hypoplasia and hypertension. Identifying high-risk infants postnatally may allow for targeted therapy. METHODS: Data were obtained on 2202 infants from the Congenital Diaphragmatic Hernia Study Group database from January 2007 to October 2011. Using binary baseline predictors generated from birth weight, 5-minute Apgar score, congenital heart anomalies, and chromosome anomalies, as well as echocardiographic evidence of pulmonary hypertension, a clinical prediction rule was developed on a randomly selected subset of the data by using a backward selection algorithm. An integer-based clinical prediction rule was created. The performance of the model was validated by using the remaining data in terms of calibration and discrimination. RESULTS: The final model included the following predictors: very low birth weight, absent or low 5-minute Apgar score, presence of chromosomal or major cardiac anomaly, and suprasystemic pulmonary hypertension. This model discriminated between a population at high risk of death (∼50%) intermediate risk (∼20%), or low risk (<10%). The model performed well, with a C statistic of 0.806 in the derivation set and 0.769 in the validation set and good calibration (Hosmer-Lemeshow test, P = .2). CONCLUSIONS: A simple, generalizable scoring system was developed for CDH that can be calculated rapidly at the bedside. Using this model, intermediate- and high-risk infants could be selected for transfer to high-volume centers while infants at highest risk could be considered for advanced medical therapies.

Extracorporeal life support in patients with congenital diaphragmatic hernia: how long should we treat?

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a frequently lethal birth defect and, despite advances, extracorporeal life support (ie, extracorporeal membrane oxygenation [ECMO]) is commonly required for severely affected patients. Published data suggest that CDH survival after 2 weeks on ECMO is poor. Many centers limit duration of ECMO support. STUDY DESIGN: We conducted a single-institution retrospective review of 19 years of CDH patients treated with ECMO, designed to evaluate which factors affect survival and duration of ECMO and define how long patients should be supported. RESULTS: Of two hundred and forty consecutive CDH patients without lethal associated anomalies, 96 were treated with ECMO and 72 (75%) survived. Eighty required a single run of ECMO and 65 survived (81%), 16 required a second ECMO run and 7 survived (44%). Of patients still on ECMO at 2 weeks, 56% survived, at 3 weeks 46% survived, and at 4 weeks, 43% of patients still on ECMO survived to discharge. After 5 weeks of ECMO, survival had dropped to 15%, and after 40 days of ECMO support there were no survivors. Apgar score at 1 minute, Apgar score at 5 minutes, and Congenital Diaphragmatic Hernia Study Group predicted survival all correlated with survival on ECMO, need for second ECMO, and duration of ECMO. Lung-to-head ratio also correlated with duration of ECMO. All survivors were discharged breathing spontaneously with no support other than nasal cannula oxygen if needed. CONCLUSIONS: In patients with severe CDH, improvement in pulmonary function sufficient to wean from ECMO can take 4 weeks or longer, and might require a second ECMO run. Pulmonary outcomes in these CDH patients can still be excellent, and the assignment of arbitrary ECMO treatment durations <4 weeks should be avoided.

Pneumothoraces as a fatal complication of congenital diaphragmatic hernia in the era of gentle ventilation.

INTRODUCTION: Pneumothorax remains a life-threatening complication that occurs in congenital diaphragmatic hernia (CDH), even under respiratory management with gentle ventilation. The aim of this study was to evaluate the prevalence of pneumothoraces as a fatal complication during the management of CDH based on the results of a nationwide Japanese survey conducted in the era of gentle ventilation. MATERIALS AND METHODS: A retrospective cohort study was performed as part of a nationwide Japanese survey of CDH. A total of 510 neonates with isolated CDH born between 2006 and 2010 were included in this study. The patients were divided into four groups according to operative findings related to the diaphragmatic defect size and operability, which represents the disease severity: defects less than 25%, defects more than 25% but less than 75%, defects more than 75%, and a patient group that was unable to undergo surgery. The prevalence of pneumothorax and the survival rate were compared with respect to each disease severity group. Each case was evaluated to determine whether the development of a pneumothorax was the primary cause of death. RESULTS: Of the 510 neonates with isolated CDH, 69 patients developed a pneumothorax before and/or after surgical intervention. Of the 69 patients 38 patients died, and only 26 patients were discharged from the hospital without any major morbidity that requires home treatment ("intact discharge"). The prevalence of pneumothorax increased and the survival rate and intact discharge rate decreased as the severity of the disease worsened. The number of patients whose pneumothorax was presumed to be the primary cause of death also increased as the severity of the disease worsened. The survival rate of the patients with pneumothorax was significantly lower than that of the patients without pneumothorax among the groups with 25 to 75% defects and 75% or more defects. CONCLUSIONS: Pneumothoraces was found to more likely occur in neonates with CDH associated with a large defect of the diaphragm. The survival rate and intact discharge rate decreased as the severity of the disease worsened, especially among the patients who developed pneumothorax accompanied by large diaphragmatic defects. No other risk factors related to pneumothorax occurrence were found, except for the severity of the disease itself, thus suggesting that pneumothorax was associated with a lethal outcome in neonates with CDH associated with a large defect of the diaphragm.

Standardized reporting for congenital diaphragmatic hernia--an international consensus.

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal death. A wide spectrum of disease severity and treatment strategies makes comparisons challenging. The objective of this study was to create a standardized reporting system for CDH. METHODS: Data were prospectively collected on all live born infants with CDH from 51 centers in 9 countries. Patients who underwent surgical correction had the diaphragmatic defect size graded (A-D) using a standardized system. Other data known to affect outcome were combined to create a usable staging system. The primary outcome was death or hospital discharge. RESULTS: A total of 1,975 infants were evaluated. A total of 326 infants were not repaired, and all died. Of the remaining 1,649, the defect was scored in 1,638 patients. A small defect (A) had a high survival, while a large defect was much worse. Cardiac defects significantly worsened outcome. We grouped patients into 6 categories based on defect size with an isolated A defect as stage I. A major cardiac anomaly (+) placed the patient in the next higher stage. Applying this, patient survival is 99% for stage I, 96% stage II, 78% stage III, 58% stage IV, 39% stage V, and 0% for non-repair. CONCLUSIONS: The size of the diaphragmatic defect and a severe cardiac anomaly are strongly associated with outcome. Standardizing reporting is imperative in determining optimal outcomes and effective therapies for CDH and could serve as a benchmark for prospective trials.

First 24-h SNAP-II score and highest PaCO2 predict the need for ECMO in congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: Early clinical predictors for the use of ECMO in patients with congenital diaphragmatic hernia (CDH) are lacking. We sought to evaluate the first 24-h SNAP-II score and highest PaCO2 as predictors of ECMO support and in-hospital mortality in neonates with CDH. METHODS: Retrospective review of 47 consecutive neonates with CDH admitted to our institution from January 2007 to December 2010 was performed. Covariates of ECMO use including SNAP-II score and highest PaCO2 within the first 24 h of NICU admission were evaluated. RESULTS: Of the 47 infants in this study, 24 patients were supported with ECMO. The ECMO group had a higher incidence of pulmonary hypertension, higher PaCO2, and higher 24-h SNAP-II scores. Only the SNAP-II score and not highest PaCO2 predicted mortality following multivariate adjustment. CONCLUSIONS: The first 24-h SNAP-II score and highest PaCO2 may provide some prognostic value in identifying neonates who undergo ECMO support; however neither measure was independently associated with the use of therapy. Only the SNAP-II score was associated with in-hospital mortality following multivariate adjustment. Additional study is needed to validate these results in a larger data set.

[The long-term follow-up of patients with a congenital diaphragmatic hernia: review of the literature]. / Hernie de coupole diaphragmatique: devenir à long terme.

The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. Many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity. Failure to thrive may be caused by GERD, insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but only a few studies focus on the health-related quality of life. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols.

Long-term maturation of congenital diaphragmatic hernia treatment results: toward development of a severity-specific treatment algorithm.

OBJECTIVES: To assess the impact of varying approaches to congenital diaphragmatic hernia (CDH) repair timing on survival and need for ECMO when controlled for anatomic and physiologic disease severity in a large consecutive series of patients with CDH. BACKGROUND: Our publication of 60 consecutive patients with CDH in 1999 showed that survival was significantly improved by limiting lung inflation pressures and eliminating hyperventilation. METHODS: We retrospectively reviewed 268 consecutive patients with CDH, combining 208 new patients with the 60 previously reported. Management and ventilator strategy were highly consistent throughout. Varying approaches to surgical timing were applied as the series matured. RESULTS: Patients with anatomically less severe left liver-down CDH had significantly increased need for ECMO if repaired in the first 48 hours, whereas patients with more severe left liver-up CDH survived at a higher rate when repair was performed before ECMO. Overall survival of 268 patients was 78%. Survival was 88% for those without lethal associated anomalies. Of these, 99% of left liver-down CDH survived, 91% of right CDH survived, and 76% of left liver-up CDH survived. CONCLUSIONS: This study shows that patients with anatomically less severe CDH benefit from delayed surgery whereas patients with anatomically more severe CDH may benefit from a more aggressive surgical approach. These findings show that patients respond differently across the CDH anatomic severity spectrum and lay the foundation for the development of risk-specific treatment protocols for patients with CDH.

Does website-based information add any value in counseling mothers expecting a baby with severe congenital diaphragmatic hernia?

OBJECTIVE: We aimed to measure whether website-provided information about congenital diaphragmatic hernia (CDH) and fetal therapy for severe cases provides added value compared with clinical counseling of parents. METHODS: This is a single center study in 102 couples who earlier opted for fetoscopic endoluminal tracheal occlusion (FETO) because of isolated severe CDH. They were asked to fill out an anonymized web-based survey of 12 questions. Then, they were offered access to information on the web pages of the randomized Tracheal Occlusion to Accelerate Lung Growth (TOTAL) trial. One week later, their appreciation was measured again by a second questionnaire. RESULTS: Eighty-two (80%) parents completed the first questionnaire, and 48 (47%) completed the entire survey. Several items became more clear to the parents after reading the website, such as the length of hospital stay (23.2% prior to web information, 60.4% after; P = 0.004), maternal risk, or the requirement of fetal anesthesia for FETO (43.9% resp. 79.2%; P = <0.001). CONCLUSION: Complementing prenatal counseling on CDH and FETO by standardized information via website is perceived by parents as of added value. Maternal risks and the need for fetal medication need more clarification during the verbal counseling prior to prenatal interventions.

The presence of a hernia sac in congenital diaphragmatic hernia is associated with better fetal lung growth and outcomes.

PURPOSE: The purpose of this study was to evaluate the relationship between the presence of a hernia sac and fetal lung growth and outcomes in infants with Congenital, Diaphragmatic Hernia (CDH). METHODS: The medical records of all neonates with CDH treated in our institution between 2004 and 2011 were reviewed. The presence of a hernia sac was confirmed at the time of surgical repair or at autopsy. Data were analyzed using parametric and non-parametric tests where appropriate. Multivariable regression and survival analyses were applied. RESULTS: Of 148 neonates treated for CDH, 107 (72%) had isolated CDH and 30 (20%) had a hernia sac. Infants with a hernia sac had significantly lower need for ECMO, patch repair, supplemental oxygen at 30 days of life, and shorter duration of mechanical ventilation and hospital stay. Ninety-three patients had prenatal imaging. The mean observed-to-expected total fetal lung volume in the sac group was higher throughout gestation. Although a greater percentage of sac patients had liver herniation as a dichotomous variable, the amount of herniated liver (%LH and LiTR) was significantly lower in the presence of a hernia sac. CONCLUSION: The presence of a hernia sac in Congenital Diaphragmatic Hernia is associated with less visceral herniation, greater fetal lung growth, and better post-natal outcomes.

Repair of congenital diaphragmatic hernias on Extracorporeal Membrane Oxygenation (ECMO): does early repair improve patient survival?

INTRODUCTION: The optimal timing of repair for congenital diaphragmatic hernia (CDH) patients that require ECMO is controversial. Early repair on ECMO theoretically allows for restoration of normal thoracic anatomy but entails significant bleeding risks. The purpose of this study was to examine the institutional outcomes of early CDH repair on ECMO. METHODS: The records of infants with CDH placed on ECMO from 2001 to 2011 were reviewed. Since 2009, a protocol was instituted for early repair while on ECMO. For this study, three cohorts were analyzed: early repair (<72 h), late repair (>72 h), and post-decannulation. These groups were compared for outcomes regarding morbidity and survival. RESULTS: Forty-six CDH patients received ECMO support with an overall survival of 53%. Twenty-nine patients (11 early/18 late) were repaired on ECMO, while 17 patients had repair post-decannulation. Survival was 73%, 50%, and 64% for those repaired early, late, or post-decannulation, respectively. Despite significantly worse prenatal factors, patients repaired early on ECMO had a similar survival. When comparing patients repaired on ECMO, the early group patients were decannulated 6 days earlier (p-value=0.009) and had significantly lower circuit complications (p=0.03). CONCLUSION: In conclusion, early repair on ECMO was associated with decreased ECMO duration, decreased circuit complications, and a trend towards improved survival.

Congenital diaphragmatic hernia: defect size correlates with developmental defect.

PURPOSE: The aim of our study was to elucidate if the defect size reflects the magnitude of the developmental defect in patients with CDH. METHODS: All patients recorded in the CDH Study Group registry between January 1, 2007, and December 31, 2010, and with defect classification were included in the study. They were divided according to defect size (A-D) and compared for: gestational age (GA), birth weight (BW), number of abnormal organ systems, prevalence of associated anomalies, cardiovascular malformations (CVM), chromosomal anomalies, liver in the chest, and hernia sac. RESULTS: A total of 1350 of 1778 patients had defect classification: 173 A, 557 B, 438 C, and 182 D. Mortality rate was 0.6%, 5.3%, 22.6%, and 45.6% in group A, B, C, and D, respectively, (p<0.0001; p for trend <0.0001). GA, BW, prevalence of associated anomalies, particularly CVM, number of abnormal organ systems, and prevalence of sac were significantly different between the groups, with a significant reduction of GA, BW, and prevalence of sac. There was an increase in prevalence of associated anomalies, liver in the chest, and number of abnormal systems as the defect size increased. CONCLUSION: Defect size is directly correlated with mortality rate, prevalence of other anomalies (particularly CVM), and number of abnormal systems, and inversely with GA, BW, and prevalence of hernia sac. The defect size may be a marker for the magnitude of developmental abnormality, thereby explaining its relationship with the outcome.

Pulmonary support on day 30 as a predictor of morbidity and mortality in congenital diaphragmatic hernia.

PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with significant in-hospital mortality, morbidity and length-of-stay (LOS). We hypothesized that the degree of pulmonary support on hospital day-30 may predict in-hospital mortality, LOS, and discharge oxygen needs and could be useful for risk prediction and counseling. METHODS: 862 patients in the CDH Study Group registry with a LOS ≥ 30 days were analyzed (2007-2010). Pulmonary support was defined as (1) room-air (n=320) (2) noninvasive supplementation (n=244) (3) mechanical ventilation (n=279) and (4) extracorporeal membrane oxygenation (ECMO, n=19). Cox Proportional hazards and logistic regression models were used to determine the case-mix adjusted association of oxygen requirements on day-30 with mortality and oxygen requirements at discharge. RESULTS: On multivariate analysis, use of ventilator (HR 5.1, p=.003) or ECMO (HR 19.6, p<.001) was a significant predictor of in-patient mortality. Need for non-invasive supplementation or ventilator on day-30 was associated with a respective 22-fold (p<.001) and 43-fold (p<.001) increased odds of oxygen use at discharge compared to those on room-air. CONCLUSIONS: Pulmonary support on Day-30 is a strong predictor of length of stay, oxygen requirements at discharge and in-patient mortality and may be used as a simple prognostic indicator for family counseling, discharge planning, and identification of high-risk infants.

Another dimension to survival: predicting outcomes with fetal MRI versus prenatal ultrasound in patients with congenital diaphragmatic hernia.

PURPOSE: A major determinant of survival in patients with congenital diaphragmatic hernia (CDH) is severity of pulmonary hypoplasia. This study addresses the comparative effectiveness of prenatal methods of lung assessment in predicting mortality, extracorporeal membrane oxygenation (ECMO), and ventilator dependency. METHODS: We retrospectively reviewed all patients born with isolated CDH between 2004 and 2008. Lung-to-head ratio (LHR) and observed-to-expected LHR (OELHR) were obtained from prenatal ultrasounds. Percent-predicted lung volume (PPLV) was obtained from fetal MRI (fMRI). Postnatal data included in-hospital mortality, need for ECMO, and ventilator dependency at day-of-life 30. RESULTS: Thirty-seven patients underwent 81 prenatal ultrasounds, while 26 of this sub-cohort underwent fMRI. Gestational age during imaging study was associated with LHR (p=0.02), but not OELHR (p=0.12) or PPLV (p=0.72). PPLV, min-LHR, and min-OELHR were each associated with mortality (p=0.03, p=0.02, p=0.01), ECMO (p<0.01, p<0.01, p=0.03), and ventilator dependency (p<0.01, p<0.01, p=0.02). For each outcome, PPLV was a more discriminative measure, based on Akaike's information criterion. Using longitudinal analysis techniques for patients with multiple ultrasounds, OELHR remained associated with mortality (p=0.04), ECMO (p=0.03), and ventilator dependency (p=0.02), while LHR was associated with ECMO (p=0.01) and ventilator dependency (p=0.02) but not mortality (p=0.06). CONCLUSION: When assessing fetuses with CDH, OELHR and PPLV may be most helpful for counseling regarding postnatal outcomes.