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.

Prematurity blunts the feeding-induced stimulation of translation initiation signaling and protein synthesis in muscle of neonatal piglets.

Postnatal growth of lean mass is commonly blunted in preterm infants and may contribute to short- and long-term morbidities. To determine whether preterm birth alters the protein anabolic response to feeding, piglets were delivered at term or preterm, and fractional protein synthesis rates (Ks) were measured at 3 days of age while fasted or after an enteral meal. Activation of signaling pathways that regulate protein synthesis and degradation were determined. Relative body weight gain was lower in preterm than in term. Gestational age at birth (GAB) did not alter fasting plasma glucose or insulin, but when fed, plasma insulin and glucose rose more slowly, and reached peak value later, in preterm than in term. Feeding increased Ks in longissimus dorsi (LD) and gastrocnemius muscles, heart, pancreas, and kidney in both GAB groups, but the response was blunted in preterm. In diaphragm, lung, jejunum, and brain, feeding increased Ks regardless of GAB. Liver Ks was greater in preterm than term and increased with feeding regardless of GAB. In all tissues, changes in 4EBP1, S6K1, and PKB phosphorylation paralleled changes in Ks. In LD, eIF4E·eIF4G complex formation, phosphorylation of TSC2, mTOR, and rpS6, and association of mammalian target of rapamycin (mTOR1) complex with RagA, RagC, and Rheb were increased by feeding and blunted by prematurity. There were no differences among groups in LD protein degradation markers. Our results demonstrate that preterm birth reduces weight gain and the protein synthetic response to feeding in muscle, pancreas, and kidney, and this is associated with blunted insulin- and/or amino acid-induced translation initiation signaling.

Fetal echocardiography (ECHO) in assessment of structural heart defects in congenital diaphragmatic hernia patients: Is early postnatal ECHO necessary for ECMO candidacy?

PURPOSE: The purpose of this study was to determine the accuracy of fetal echocardiogram (ECHO) for detecting cardiac structural anomalies that may impact Extracorporeal Membrane Oxygenation (ECMO) candidacy in infants with Congenital Diaphragmatic Hernia (CDH). METHODS: A retrospective review was performed on fetuses with CDH (January 2007-June 2017). Inclusion criteria were inborn and at least one prenatal and postnatal ECHO. ECHOs were evaluated for structural heart defects. Primary outcomes were accuracy of prenatal fetal ECHO and identify differences between prenatal and postnatal ECHO. Descriptive statistics and Chi-square analysis were performed. RESULTS: 131 inborn patients were identified. Mean gestational age of fetal ECHO was 26.6 ±â€¯5.5 weeks. The median time to postnatal ECHO was DOL 1 [0-30]. Fetal ECHO had 92% accuracy, 83% sensitivity, 93% specificity, PPV of 95%, NPV of 92%, and a 90% accuracy for visualization of at least one pulmonary vein into the left atrium on the contralateral (non-CDH) side. Thirty-five percent of patients received ECMO, and 26% had an associated cardiac anomaly. All ECMO patients had an accurate structural fetal ECHO. CONCLUSION: Fetal ECHO is sufficient for identifying major structural heart defects at large volume centers with trained pediatric cardiologists and may be used to guide clinical management, particularly in regards to ECMO candidacy. LEVEL OF EVIDENCE: Level III.

Fetal Surgery Decreases Anesthesia-Induced Neuroapoptosis in the Mid-Gestational Fetal Ovine Brain.

BACKGROUND: Studies demonstrating an association between anesthesia and brain cell death (neuroapoptosis) in young animals were performed without accompanying surgery. This study tests the hypothesis that fetal surgery decreases anesthesia-induced neuroapoptosis. MATERIALS AND METHODS: Seventy-day-pregnant ewes received 2% isoflurane for 1 h (low dose [LD]) or 4% for 3 h (high dose [HD]) with or without fetal surgery (S). Unexposed fetuses served as controls (C). Fetal brains were processed for neuroapoptosis using anti-caspase-3 antibodies. Data were analyzed using ANOVA. RESULTS: Twenty-eight fetal sheep were evaluated. Dentate gyrus neuroapoptosis was lower in the HD+S group (13.1 ± 3.76 × 105/mm3) than in the HD (19.1 ± 1.40 × 105/mm3, p = 0.012) and C groups (18.3 ± 3.55 × 105/mm3, p = 0.035). In the pyramidal layer of the hippocampus, neuroapoptosis was lower in the HD+S group (8.11 ± 4.88 × 105/mm3) than in the HD (14.8 ± 2.82 × 105/mm3, p = 0.006) and C groups (14.1 ± 4.54 × 105/mm3, p = 0.019). The LD+S group showed a trend towards a significant decrease in neuroapoptosis in the pyramidal layer (LD+S 7.51 ± 1.48 vs. LD 13.5 ± 1.87 vs. C 14.1 ± 4.54 × 105/mm3, p = 0.07) but not in the dentate gyrus. Fetal surgery did not affect neuroapoptosis in the frontal cortex or endplate. CONCLUSIONS: Fetal surgery decreases isoflurane-induced neuroapoptosis in the dentate gyrus and the pyramidal layer of mid-gestational fetal sheep. Long-term effects of these observations on memory and learning deserve further exploration.

Early vs late resection of asymptomatic congenital lung malformations.

PURPOSE: To examine postsurgical outcomes of a consecutive series of children treated with elective operations for congenital lung malformations (CLM). METHODS: A retrospective review was performed on a prospectively collected dataset of all fetuses evaluated for a CLM between July 2001 and June 2016. Prenatal findings, operative treatment and postnatal outcomes were collected. Children having elective operations were divided in two groups based on age at time of surgery. RESULTS: Of 220 fetuses, 143 had operations and follow-up at our center. Six had open fetal lobectomy, 17 had EXIT-to-resection, 16 infants had urgent resection for symptoms and 110 with asymptomatic lesions had elective resection. Of these 110, the median fetal maximum CVR was 0.8 [range 0.1-2.2], and median age at operation was 4 (1.5-60) months (58% had resection at ≤4 months). Overall complication rate, including air-leak and pleural effusion, was 15%. When comparing those having resection at ≤4 months to those >4 months, there were no significant differences in complication rates or length of stay. Operative time was shorter for patients with early resection (154 ±â€¯59 vs 181 ±â€¯89, p = 0.05). No infant having resection at ≤4 months was readmitted. Overall, children survived with good recovery. CONCLUSION: Early elective resection of congenital lung malformations prior to 4 months of age is feasible and not associated with increased operative risk. TYPE OF STUDY: Restropective study. LEVEL OF EVIDENCE: Level III.

Prematurity reduces citrulline-arginine-nitric oxide production and precedes the onset of necrotizing enterocolitis in piglets.

Necrotizing enterocolitis (NEC) is associated with low plasma arginine and vascular dysfunction. It is not clear whether low intestinal citrulline production, the precursor for arginine synthesis, occurs before and thus predisposes to NEC or if it results from tissue damage. This study was designed to test the hypothesis that whole body rates of citrulline, arginine, and nitric oxide synthesis are low in premature pigs and that they precede NEC. Piglets delivered by cesarean section at 103 days [preterm (PT)], 110 days [near-term (NT)], or 114 days [full-term (FT)] of gestation were given total parenteral nutrition and after 2 days orogastrically fed infant formula for 42 h to induce NEC. Citrulline and arginine fluxes were determined before and during the feeding protocol. Gross macroscopic and histological NEC scores and plasma fatty acid binding protein (iFABP) concentration were determined as indicators of NEC. Intestinal gene expression for enzymes of the arginine pathway were quantitated. A lower ( P < 0.05) survival rate was observed for PT (8/27) than for NT (9/9) and FT pigs (11/11). PT pigs had higher macroscopic gross ( P < 0.05) and histological NEC ( P < 0.05) scores and iFABP concentration ( P < 0.05) than pigs of more advanced gestational age. PT pigs had lower citrulline production and arginine fluxes ( P < 0.05) throughout and a reduced gene expression in genes of the citrulline-arginine pathway. In summary, intestinal enzyme expression and whole body citrulline and arginine fluxes were reduced in PT pigs compared with animals of more advance gestational age and preceded the development of NEC. NEW & NOTEWORTHY Arginine supplementation prevents necrotizing enterocolitis (NEC), the most common gastrointestinal emergency of prematurity. Citrulline (precursor for arginine) production is reduced during NEC, and this is believed to be a consequence of intestinal damage. In a swine model of NEC, we show that intestinal gene expression of the enzymes for citrulline production and whole body citrulline and arginine fluxes are reduced and precede the onset of NEC in premature pigs. Reduced citrulline production during prematurity may be a predisposition to NEC.

Surgical Outcomes of Patients with Beckwith-Wiedemann Syndrome.

PURPOSE: The purpose of this study was to evaluate treatment and surgical outcomes of patients of Beckwith-Wiedemann Syndrome (BWS) treated at a tertiary children's hospital. METHODS: A retrospective review of infants evaluated at Texas Children's Hospital for BWS from August 2000 to December 2016 was performed. Data collected included demographic information, clinical presentation, genetic evaluation, fetal imaging, operative treatment, and outcomes. RESULTS: Forty-seven children with a diagnosis of BWS were identified. Sixty-four percent (n=30) had a genetic mutation in an imprinting domain of chromosome 11p15. Thirty-two patients (68%) underwent at least one operation related to BWS with a median of 2 [range: 0-8] surgical procedures per patient. Sixteen underwent omphalocele repair, 12 had partial glossectomies-, 7 underwent surgeries related to hemihypertrophy, and 6 had resection of an embryonal tumor (two adrenal cortical adenoma, one Wilms' tumor, two hepatoblastoma). Overall, survival was 100% with feeding difficulty (47%) being the most frequent complication. CONCLUSION: A substantial number of patients with Beckwith-Wiedemann Syndrome will require surgery. However, overall outcomes are similar between those that require surgery and those that do not. LEVEL OF EVIDENCE: Level III.

Necrotizing enterocolitis in patients with congenital heart disease: A single center experience.

PURPOSE: The purpose of this study was to evaluate the characteristics of patients with congenital heart disease (CHD) who developed necrotizing enterocolitis (NEC). METHODS: A retrospective review of neonates with CHD at a tertiary care center between January 2006 and January 2016 was performed. Diagnosis of NEC was based on modified Bell's criteria. Patients were grouped by Risk Adjustment for Congenital Heart Surgery (RACHS-1) or by ductal-dependent (DD) lesions that require a patent ductus arteriosus to supply pulmonary or systemic circulation. RESULTS: Of 1811 neonates with CHD, 3.4% (n=61) developed NEC. Eighteen (30%) of these required surgical management. The rate of NEC among DD patients was 5% (n=33/653), compared to 2.4% (n=28/1158) in the non-DD group (p=0.003). RACHS-1 score>2 had a higher rate of NEC 6.2% (41/658) compared to RACHS-1≤2 cases, 1.7% (20/1153) (p=0.005). DD patients and complex patients with RACHS-1>2 were more likely to develop NEC after cardiac surgery. Hypoplastic left heart syndrome patients had a rate of 9% (n=16/185). Surgical NEC was more prevalent in the non-DD group. Mortality was similar among groups. CONCLUSION: CHD patients with ductal-dependent lesions or complex cases (RACHS-1 score>2) have higher rates of NEC than non-ductal-dependent patients or RACHS-1 score of 2 or less. Mortality is similar regardless of ductal dependence, but surgical NEC was more prevalent in non-DD patients. LEVEL OF EVIDENCE: Level IIb.

Extracorporeal Membrane Oxygenation in Premature Infants With Congenital Diaphragmatic Hernia.

Prematurity and low birth weight have been exclusion criteria for extracorporeal membrane oxygenation (ECMO); however, these criteria are not evidence based. With advances in anticoagulation, improved technology, and surgical expertise, it is difficult to deny a potential therapy based on these criteria alone. We report the outcome of three neonates who were ineligible based on traditional criteria but were offered ECMO as a life-saving measure. We highlight the interdisciplinary nature of modern decision-making. All three neonates had severe congenital diaphragmatic hernia diagnosed prenatally, had normal fetal karyotypes, were born prematurely, and weighed less than 2 kg. All three neonates underwent cervical venoarterial cannulation, stabilization on ECMO, and repair of their congenital diaphragmatic hernia early in their ECMO courses. All three infants had long courses of respiratory support attributable to lung hypoplasia, but there were no short- or long-term complications attributable to ECMO support directly. All three are alive at 2 years of age and were making progress developmentally. In conclusion, with interdisciplinary collaboration and clinical guidelines uniformly implemented, low birth weight infants may benefit from ECMO and should not be denied the therapy arbitrarily based on gestational age or size alone. Further research is essential to determine appropriate patient selection in premature infants.

A novel multimodal computational system using near-infrared spectroscopy predicts the need for ECMO initiation in neonates with congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: The purpose of this study was to develop a computational algorithm that would predict the need for ECMO in neonates with congenital diaphragmatic hernia (CDH). METHODS: CDH patients from August 2010 to 2016 were enrolled in a study to continuously measure cerebral tissue oxygen saturation (cStO2) of left and right cerebral hemispheres. NIRS devices utilized were FORE-SIGHT, CASMED and INVOS 5100, Somanetics. Using MATLAB©, a data randomization function was used to deidentify and blindly group patient's data files as follows: 12 for the computational model development phase (6 ECMO and 6 non-ECMO) and the remaining patients for the validation phase. RESULTS: Of the 56 CDH patients enrolled, 22 (39%) required ECMO. During development of the algorithm, a difference between right and left hemispheric cerebral oxygenation via NIRS (ΔHCO) was noted in CDH patients that required ECMO. Using ROC analysis, a ΔHCO cutoff >10% was predictive of needing ECMO (AUC: 0.92; sensitivity: 85%; and specificity: 100%). The algorithm predicted need for ECMO within the first 12h of life and at least 6h prior to the clinical decision for ECMO with 88% sensitivity and 100% specificity. CONCLUSION: This computational algorithm of cerebral NIRS predicts the need for ECMO in neonates with CDH. LEVEL OF EVIDENCE: II.

Feasibility and Outcomes of Fetoscopic Tracheal Occlusion for Severe Left Diaphragmatic Hernia.

OBJECTIVE: To evaluate feasibility and initial outcomes of fetoscopic tracheal occlusion for severe diaphragmatic hernia compared with a historical cohort who had not received fetal tracheal occlusion. METHODS: Outcomes in a prospective observational cohort who underwent fetoscopic tracheal occlusion for severe fetal left diaphragmatic hernia without associated anomalies were compared with our historical nontreated cohort of matched fetuses of similar severity. Fetuses were classified using the same ultrasonography and magnetic resonance imaging methodology-prospectively in the fetoscopic tracheal occlusion group and retrospectively in the historical nontreated cohort. Obstetric and postnatal outcomes were evaluated and compared. RESULTS: Between January 2004 and June 2015, 218 fetuses with diaphragmatic hernia were evaluated. Twenty (9%) fetuses had severe left diaphragmatic hernia (lung-head ratio 1.0 or less and liver herniation), of which 9 of 20 were managed without tracheal occlusion. Eleven were offered the procedure and in 10, it was successful. Mean (±standard deviation) gestational age was 27.9±1.1 weeks at attempted balloon placement, 34.1±1.1 weeks at removal, and 35.3±2.2 weeks at delivery. One patient required an ex utero intrapartum treatment procedure at delivery to remove the balloon. There were no maternal complications or fetal deaths. All neonates underwent postnatal repair with a patch. The 6-month, 1-year, and 2-year survival rates were significantly higher in our treated cohort than in our nontreated historical cohort (80% compared with 11%, risk difference 69%, 95% confidence interval [CI] 38-100%, P=.01; 70% compared with 11%, risk difference 59%, 95% CI 24-94%, P=.02; and 67% compared with 11%, risk difference 56%, 95% CI 19-93%, P=.04, respectively) with reduced need for extracorporeal membrane oxygenation (30% compared with 70%, risk difference 40%, 95% CI 10-79%, P=.05). CONCLUSION: Fetoscopic tracheal occlusion is feasible and is associated with improved postnatal outcomes in severe left diaphragmatic hernia. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov, https://clinicaltrials.gov, NCT00881660.

Space occupying lesions in the presence of congenital diaphragmatic hernia.

INTRODUCTION: Previous reports describe lung malformations and other chest lesions in association with congenital diaphragmatic hernia (CDH), yet little is known how these lesions affect outcomes. We sought to evaluate the incidence and outcomes of patients diagnosed with chest lesions in association with CDH. METHODS: The charts of all infants treated for CDH in a single tertiary center from January 2004 to January 2015 were reviewed. The outcomes of those with space occupying lesions (SOLs) in association with CDH were compared to those with isolated CDH. Statistical analysis was performed using Student's t-test and Mann-Whitney U test for continuous variables and Fisher's exact for categorical variables. RESULTS: Of the 214 infants treated, 20 had an associated SOL (4 had>1 lesion). SOLs were confirmed by pathological examination and included: bronchopulmonary sequestration (n=10; 4.7%), ectopic liver (n=9; 4.2%), foregut duplication cyst (n=2; 1%), and other lesions (n=3; 1.4%). No statistical difference was noted in the long-term outcomes of patients with SOL in comparison to those with isolated CDH. CONCLUSION: SOLs are not uncommon in neonates with CDH. Despite theoretical concerns, there is no evidence that SOLs are associated with worse outcomes, a finding which is helpful during prenatal counseling of families.

An evaluation of the role of concomitant anomalies on the outcomes of fetuses with congenital diaphragmatic hernia.

PURPOSE: The purpose of this study was to evaluate the impact of various types of associated anomalies on CDH mortality and morbidity. METHODS: All CDH patients at a tertiary care center from January 2004 to January 2014 were reviewed retrospectively. Isolated CDH was defined as CDH without any associated anomalies. Cardiac anomalies were stratified into minor and major based on the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1) scoring system. Other anatomic anomalies requiring intervention in the perinatal period were classified as major anomalies. The outcomes of interest were 6-month mortality as well as pulmonary and gastrointestinal morbidity. RESULTS: Of 189 CDH patients, 93 (49%) had isolated CDH. Others had: cardiac anomalies alone (n=47, 25%), genetic anomalies (n=28, 15%), structural anomalies alone (n=18, 10%), and both cardiac and genetic anomalies (n=20, 11%). Fifty (26.5%) patients were dead before six months of age. Mortality rate at 6months was higher in patients with genetic and major cardiac anomalies. A major cardiac anomaly was independently associated with a 102-fold increased risk of mortality at 6months (95%CI: 3.1-3402). Pulmonary morbidity was increased in patients with genetic, major cardiac, and major structural anomalies, while gastrointestinal morbidity was higher in patients with major structural anomalies alone. CONCLUSION: Major cardiac and genetic anomalies were associated with increased 6-month mortality in CDH patients. However, the association with minor cardiac anomalies and/or structural anomalies did not affect mortality and morbidity of CDH patients. The presence of minor anomalies should not adversely impact their perinatal management or consideration for in-utero therapy.

A novel multimodal computational system using near-infrared spectroscopy to monitor cerebral oxygenation during assisted ventilation in CDH patients.

BACKGROUND/PURPOSE: The aim of this study was to create a computational simulator to serve as an early alert system for cerebral hypoxemia prior to the onset of clinical symptoms. METHODS: Neonates with congenital diaphragmatic hernia (Jan 2010-Dec 2014) were recruited to collect continuous measurements of cerebral tissue oxygen saturation (cStO2) using a near-infrared spectroscopy (NIRS) device (FORE-SIGHT®, CASMED). Clinicians were blinded to NIRS data and treated infants based on pre-established clinical protocols. Charts were reviewed retrospectively to identify clinical events of hypoxemia (spontaneous, sustained decrease in preductal SpO2<85% leading to ventilator changes). We developed a computational algorithm that determined baseline values, variability and event data for each patient. RESULTS: Twenty-three of 36 patients enrolled met data criteria. The algorithm anticipated an event at least 15 minutes prior to the event in 77% of cases, with an average pre-event detection of 47 minutes (range 16-122 minutes). Post-event StO2 (SpO2<85%) was determined to be 63.7% ± 11.7. In this computational model, the sensitivity to distinguish low states of cerebral perfusion was 94% with a specificity of 96%. CONCLUSION: We have developed a computational algorithm with an early warning system that has the potential of being translated into a real-time clinical interface that may improve management of neonates.

Risk-stratification of severity for infants with CDH: Prenatal versus postnatal predictors of outcome.

PURPOSE: The purpose of this study was to compare the predication accuracy of a newly described postnatally-based clinical prediction model to fetal imaging-based predictors of mortality for infants with CDH. METHODS: We performed a retrospective review of all CDH patients treated at a comprehensive fetal care center from January 2004 to January 2014. Prenatal data reviewed included lung-to-head ratio (LHR), observed/expected-total fetal lung volume (O/E-TFLV), and percent liver herniation (%LH). Based on the postnatal prediction model, neonates were categorized as low, intermediate, and high risk of death. The primary outcome was 6-month mortality. RESULTS: Of 176 CDH patients, 58 had a major cardiac anomaly, and 28 had a genetic anomaly. Patients with O/E-TFLV <35% and %LH >20% were at increased risk for mortality (44% and 36%, respectively). There was a significant difference in mortality between low, intermediate, and high-risk groups (4% vs. 22% vs. 51%; p<0.001). On multivariate regression, the O/E-TFLV and postnatal-based mortality risk score were the two independent predictors of 6-month mortality. CONCLUSION: The CDH Study Group postnatal predictive model provides good discrimination among three risk groups in our patient cohort. The prenatal MRI-based O/E-TFLV is the strongest prenatal predictor of 6-month mortality in infants with CDH and will help guide prenatal counseling and discussions regarding fetal intervention and perinatal management.

The effect of supplemental parenteral nutrition on outcomes of necrotizing enterocolitis in premature, low birth weight neonates.

BACKGROUND: We hypothesized that supplemental parenteral nutrition (PN) decreases the need for surgery and mortality associated with necrotizing enterocolitis (NEC). METHODS: Single institution retrospective review of all premature, low birth weight infants with NEC from January 2006 to December 2013 was conducted. RESULTS: NEC was identified in 114 premature, low birth weight infants, 59 (51.8%) of which required surgical management. Surgical NEC infants were born younger (25.8 ± 4.0 vs 27.8 ± 3.3 weeks, P = .005) and weighed less at birth (829 ± 281 vs 938 ± 271 g, P = .038) than those managed medically. There was no difference in the use of PN (37.7% vs 31.4%, P = .541) between surgical and medical NEC patients. There was no statistically significant difference in mortality at discharge between patients who had PN at NEC onset and those who did not (31.4% vs 42.6%, P = .294) CONCLUSION: In this single-center study, supplemental PN at NEC onset does not appear to significantly improve outcomes as demonstrated by rates of surgical intervention and in-hospital mortality.

Revisiting outcomes of right congenital diaphragmatic hernia.

BACKGROUND: Studies 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. METHODS: We 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. RESULTS: Of 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. CONCLUSIONS: Compared 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.