The role of extracorporeal life support and timing of repair in infants with congenital diaphragmatic hernia.

Extracorporeal life support (ECLS) serves as a rescue therapy for patients with congenital diaphragmatic hernia (CDH) and severe cardiopulmonary failure, and only half of these patients survive to discharge. This costly intervention has a significant complication risk and is reserved for patients with the most severe disease physiology refractory to maximal cardiopulmonary support. Some contraindications to ECLS do exist such as coagulopathy, lethal chromosomal or congenital anomaly, very preterm birth, or very low birth weight, but many of these limits are being evaluated through further research. Consensus guidelines from the past decade vary in recommendations for ECLS use in patients with CDH but this therapy appears to have a survival benefit in the most severe subset of patients. Improved outcomes have been observed for patients treated at high-volume centers. This review details the evolving literature surrounding management paradigms for timing of CDH repair for patients receiving preoperative ECLS. Most recent data support early repair following cannulation to avoid non-repair which is uniformly fatal in this population. Longer ECLS runs are associated with decreased survival, and patient physiology should guide ECLS weaning and eventual decannulation rather than limiting patients to arbitrary run lengths. Standardization of care across centers is a major focus to limit unnecessary costs and improve short-term and long-term outcomes for these complex patients.

Hepatic Metastasectomy in Pediatric Patients: An Observational Study.

BACKGROUND: The role of hepatectomy for metastatic disease in children is controversial. Rationales include potential cure, obtaining a diagnosis, and guiding chemotherapy decisions. This study examines the safety and utility of hepatic metastasectomy for children at a single institution. METHODS: After IRB approval (#22-1258), medical records were reviewed from 1995 to 2022 for children undergoing hepatic metastasectomy. En-bloc hepatectomies during primary tumor resection were excluded. RESULTS: Hepatic metastasectomy was performed in 16 patients for a variety of histologies. Median patient age was 12.2 years [IQR 6.9-22.6], and 13/16 patients were female (81 %). Number of hepatic metastases ranged from 1 to 23 and involved between 1 and 8 Couinaud segments. Anatomic resections included 4 hemihepatectomies and 1 sectionectomy. All other resections were nonanatomic. 3/6 resections for germ cell tumor (GCT) revealed only mature teratoma, driving adjuvant therapy decisions. When indicated, median time to adjuvant chemotherapy was 19 days [IQR 11-22]. No patients had Clavien-Dindo Class III or higher perioperative morbidity. Three patients (1 GCT, 1 adrenocortical carcinoma (ACC), and 1 gastric neuroendocrine tumor (GNET) experienced hepatic relapse. The patients with relapsed GCT and GNET are alive with disease at 17 and 135 months, respectively. The patient with ACC died of disease progression and liver failure. One patient with Wilms tumor experienced extrahepatic, retroperitoneal recurrence and died. With a median follow-up of 38 months, 10-year disease-specific and disease-free survival were 77 % and 61 %, respectively. CONCLUSIONS: Hepatic metastasectomy can be accomplished safely in children, may guide adjuvant therapy decisions, and is associated with long-term survival in selected patients. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Treatment Study, Case series with no comparison group.

Outcome of Extracorporeal Cardiopulmonary Resuscitation in Pediatric Patients Without Congenital Cardiac Disease: Extracorporeal Life Support Organization Registry Study.

OBJECTIVES: To describe the use of extracorporeal cardiopulmonary resuscitation (ECPR) in pediatric patients without congenital heart disease (CHD) and identify associations with in-hospital mortality, with a specific focus on initial arrest rhythm. DESIGN: Retrospective cohort study using data from pediatric patients enrolled in Extracorporeal Life Support Organization (ELSO) registry between January 1, 2017, and December 31, 2019. SETTING: International, multicenter. PATIENTS: We included ECPR patients under 18 years old, and excluded those with CHD. Subgroup analysis of patients with initial arrest rhythm. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We identified 567 patients: neonates (12%), infants (27%), children between 1 and 5 years old (25%), and children over 5 years old (36%). The patient cohort included 51% males, 43% of White race, and 89% not obese. Most suffered respiratory disease (26%), followed by acquired cardiac disease (25%) and sepsis (12%). In-hospital mortality was 59%. We found that obesity (adjusted odds ratio [aOR], 2.28; 95% CI, 1.21-4.31) and traumatic injury (aOR, 6.94; 95% CI, 1.55-30.88) were associated with greater odds of in-hospital mortality. We also identified lower odds of death associated with White race (aOR, 0.64; 95% CI, 0.45-0.91), ventricular tachycardia (VT) as an initial arrest rhythm (aOR, 0.36; 95% CI, 0.16-0.78), return of spontaneous circulation before cannulation (aOR, 0.56; 95% CI, 0.35-0.9), and acquired cardiac disease (aOR, 0.43; 95% CI, 0.29-0.64). Respiratory disease was associated with greater odds of severe neurologic complications (aOR, 1.64; 95% CI, 1.06-2.54). CONCLUSIONS: In children without CHD undergoing ECPR, we found greater odds of in-hospital mortality were associated with either obesity or trauma. The ELSO dataset also showed that other variables were associated with lesser odds of mortality, including VT as an initial arrest rhythm. Prospective studies are needed to elucidate the reasons for these survival differences.

Center Volume and Survival Relationship for Neonates With Congenital Diaphragmatic Hernia Treated With Extracorporeal Life Support.

OBJECTIVES: Literature is emerging regarding the role of center volume as an independent variable contributing to improved outcomes. A higher volume of index procedures may be associated with decreased morbidity and mortality. This association has not been examined for the subgroup of infants with congenital diaphragmatic hernia (CDH) receiving extracorporeal life support (ECLS). Our study aims to examine the risk-adjusted association between center volume and outcomes in CDH-ECLS neonates, hypothesizing that higher center volume confers a survival advantage. DESIGN: Multicenter, retrospective comparative study using the Extracorporeal Life Support Organization database. SETTING: One hundred twenty international pediatric centers. PATIENTS: Neonates with CDH managed with ECLS from 2000 to 2019. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The cohort included 4,985 neonates with a mortality rate of 50.6%. For the 120 centers studied, mean center volume was 42.4 ± 34.6 CDH ECLS cases over the 20-year study period. In an adjusted model, higher ECLS volume was associated with lower odds of mortality: odds ratio (OR) 0.995 (95% CI, 0.992-0.999; p = 0.014). For an increase in one sd in volume, that is, 1.75 cases annually, the OR for mortality was lower by 16.7%. Volume was examined as a categorical exposure variable where low-volume centers (fewer than 2 cases/yr) were associated with 54% higher odds of mortality (OR, 1.54; 95% CI, 1.03-2.29) compared with high-volume centers. On-ECLS complications (mechanical, neurologic, cardiac, hematologic metabolic, and renal) were not associated with volume. The likelihood of infectious complications was higher for low- (OR, 1.90; 95% CI, 1.06-3.40) and medium-volume (OR, 1.87; 95% CI, 1.03-3.39) compared with high-volume centers. CONCLUSIONS: In this study, a survival advantage directly proportional to center volume was observed for CDH patients managed with ECLS. There was no significant difference in most complication rates. Future studies should aim to identify factors contributing to the higher mortality and morbidity observed at low-volume centers.

Assessment and Management of Inguinal Hernias in Children.

Inguinal hernia repair is one of the most commonly performed surgical procedures in children. However, a number of controversial issues, such as optimal timing and approach to repair, continue to be debated. This publication reviews the available data pertaining to these controversial issues and identifies best practices in management of inguinal hernias in children.

Inhaled Nitric Oxide Utilization in Congenital Diaphragmatic Hernia Treated With Extracorporeal Life Support: A Propensity Score Analysis.

Although used commonly, ability of inhaled nitric oxide (iNO) to improve outcomes in infants with congenital diaphragmatic hernia (CDH) who receive extracorporeal life support (ECLS) remains controversial. We sought to determine the association between pre-ECLS use of iNO and mortality in infants with CDH from the Extracorporeal Life Support Organization (ELSO) Registry. Neonates who underwent ECLS for CDH were identified from the ELSO Registry from 2009 to 2019. Patients were categorized into those treated with iNO versus not prior to initiating ECLS. Patients were then matched 1:1 for case-mix based on pre-ECLS covariates using the propensity score (PS) for iNO treatment. The matched groups were compared for mortality. The matched cohorts were also compared for ELSO-defined systems-based complications as secondary outcomes. There were a total of 3,041 infants with an overall mortality of 52.2% and a pre-ECLS iNO use rate of 84.8%. With 1:1 matching, there were 461 infants with iNO use and 461 without iNO use. Following matching, use of iNO was not associated with a difference in mortality (odds ratio [OR] = 0.805; 95% confidence interval [CI], 0.621-1.042; p = 0.114). Results were similar in unadjusted analyses, and after controlling for covariates in the full cohort of patients and in the 1:1 matched data. Patients receiving iNO had significantly higher odds of renal complications (OR = 1.516; 95% CI, 1.141-2.014; p = 0.004), but no other significant differences were observed among secondary outcomes. ECLS use of iNO in CDH patients was not associated with a difference in mortality. Future randomized controlled studies are needed to delineate the utility of iNO in CDH patients.

Surgical Management of Congenital Diaphragmatic Hernia.

Congenital diaphragmatic hernia (CDH) is a challenging surgical disease that requires complex preoperative, perioperative, and postoperative care. Survival depends on successful reduction and repair of the defect, and numerous complex decisions must be made regarding timing and preparation for surgery. This review describes the challenges and controversies inherent to surgical CDH care and provides recommendations for management based on the most recent evidence.

Congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a rare birth defect characterized by incomplete closure of the diaphragm and herniation of fetal abdominal organs into the chest that results in pulmonary hypoplasia, postnatal pulmonary hypertension owing to vascular remodelling and cardiac dysfunction. The high mortality and morbidity rates associated with CDH are directly related to the severity of cardiopulmonary pathophysiology. Although the aetiology remains unknown, CDH has a polygenic origin in approximately one-third of cases. CDH is typically diagnosed with antenatal ultrasonography, which also aids in risk stratification, alongside fetal MRI and echocardiography. At specialized centres, prenatal management includes fetal endoscopic tracheal occlusion, which is a surgical intervention aimed at promoting lung growth in utero. Postnatal management focuses on cardiopulmonary stabilization and, in severe cases, can involve extracorporeal life support. Clinical practice guidelines continue to evolve owing to the rapidly changing landscape of therapeutic options, which include pulmonary hypertension management, ventilation strategies and surgical approaches. Survivors often have long-term, multisystem morbidities, including pulmonary dysfunction, gastroesophageal reflux, musculoskeletal deformities and neurodevelopmental impairment. Emerging research focuses on small RNA species as biomarkers of severity and regenerative medicine approaches to improve fetal lung development.

Variation across centers in standardized mortality ratios for congenital diaphragmatic hernia receiving extracorporeal life support.

BACKGROUND: We sought to elucidate the degree of variation across centers by calculating center-specific standardized mortality ratios (SMRs) for infants with congenital diaphragmatic hernia (CDH) requiring extracorporeal life support (ECLS). METHODS: The Extracorporeal Life Support Organization (ELSO) registry data (2000-2019) were used to estimate SMRs. Center-specific SMRs and their 95% confidence intervals (CIs) were used to identify centers with mortality as significantly worse (SW), significantly better (SB), or not different (ND) relative to the median standardized mortality rate. RESULTS: We identified 4,223 neonates with CDH from 109 centers. SMRs were risk-adjusted for pre-ECLS case-mix (birthweight, sex, race, 5 min Apgar, blood gases, gestational age, hernia side, prenatal diagnosis, pre-ECLS arrest, and comorbidities). Observed (unadjusted) mortality rates across centers varied substantially (range: 14.3%-90.9%; interquartile range [IQR]: 42.9%-62.1%). Thirteen centers (11.9%) had SB SMRs< 1 (SMR 0.52 to 0.84), 7 centers (6.4%) had SW SMRs>1 (SMR 1.25 to 1.43), and 89 centers (81.7%) had SMRs ND relative to the median SMR rate across all centers (i.e., SMR not different from one). Descriptive analyses demonstrated that SB centers had a lower proportion of cases with renal complications, infectious complications and discontinuation of ECLS owing to complications, as well as differences in pre-ECLS treatments and timing of CDH repair, compared to SW and ND centers. CONCLUSION: This study specifically identified ECLS centers with higher and lower survival for patients with CDH, which may serve as a benchmark for institutional quality improvement. Future studies are needed to identify those specific processes at those centers that leads to favorable outcomes with the goal of improving care globally. LEVEL OF EVIDENCE: Level III.

Prevalence of Upper Gastrointestinal Inflammation in Teens With Obesity Prior to Sleeve Gastrectomy.

INTRODUCTION: Upper gastrointestinal (UGI) pathologies are common in adolescents with obesity. This study aims to determine the prevalence of UGI inflammation on preoperative esophagogastroduodenoscopy (EGD) in adolescents undergoing sleeve gastrectomy (SG) and to assess weight loss outcomes. METHODS: This is a retrospective analysis of pathology reports from EGD biopsies performed prior to SG from September 2017 to August 2020. Percentage weight loss was measured at 3, 6, and 12 mo after surgery. Percent total body weight loss (TBWL) was compared between patients with and without UGI inflammation. RESULTS: Thirty adolescents underwent laparoscopic SG. Mean TBWL was 22% of total body weight 12 mo after surgery. Preoperative EGD identified 9 (30%) patients with esophagitis, 10 (33%) with gastritis, and 9 (30%) with duodenitis. Twenty-one patients (70%) had inflammation of at least one area, 5 (17%) were Helicobacter pylori positive, and 1 (3%) had a gastric ulcer that delayed surgery. Five (17%) patients were taking antacids prior to EGD. Patients with preoperative gastric or duodenal inflammation had significantly less TBWL 12 mo after SG compared to patients without gastric (24.6% versus 16.7%, P = 0.04) or duodenal inflammation (25.7% versus 14.1%, P = 0.02). CONCLUSIONS: There is a high prevalence of UGI inflammation in adolescents undergoing SG. Gastric and duodenal inflammation is associated with less TBWL after SG.

Center Volume and Cost-Effectiveness in the Treatment of Congenital Diaphragmatic Hernia.

BACKGROUND: The care of patients with congenital diaphragmatic hernia (CDH) is expensive, yet little is known about variability in cost-of-care for these patients. The purpose of this study was to examine the cost-effectiveness of CDH treatment, comparing high- versus low-volume centers. METHODS: This is a retrospective study of neonatal patients with CDH at U.S. hospitals using data from the Pediatric Health Information System database (2015-2018). Centers were considered high-volume if they had ≥10 patients with CDH for ≥ 2 y. Cost-effectiveness analysis was performed with cost per survivor as the outcome measure, and probabilistic sensitivity analysis was performed. RESULTS: A total of 1687 patients were included in the study. Overall mortality was 24.4%. Patients at high-volume centers had a longer mean length of stay (64 d versus 48 d for low-volume centers, P = 0.0001) and higher extracorporeal life support (ECLS) rates (32% versus 24%, P = 0.002). Risk-adjusted mortality did not differ between high- and low-volume centers (23.9% versus 25.9%, P = 0.39), except when ECLS was involved (42% versus 56%, P = 0.011). Costs were significantly higher at high-volume centers ($395,291 ± 508,351 versus $255,074 ± 308,802, P < 0.0001). Survival status, ECLS use, operative repair, length of stay, high-volume status, and gestational age were identified as independent drivers of cost. On cost-effectiveness analysis, low-volume hospitals were more cost-effective in 95% of simulations. CONCLUSIONS: High-volume centers have substantially higher costs without an associated survival benefit and are less cost-effective than low-volume centers. Standardization of care is necessary to minimize the delivery of low-value care.

Survival Benefit Associated With the Use of Extracorporeal Life Support for Neonates With Congenital Diaphragmatic Hernia.

OBJECTIVE: To measure the survival among comparable neonates with CDH supported with and without ECLS. SUMMARY OF BACKGROUND DATA: Despite widespread use in the management of newborns with CDH, ECLS has not been consistently associated with improved survival. METHODS: A retrospective cohort study was performed using ECLS-eligible CDH Study Group registry patients born between 2007 and 2019. The primary outcome was in-hospital mortality. Neonates who did and did not receive ECLS were matched based on variables affecting risk for the primary outcome. Iterative propensity score-matched, survival (Cox regression and Kaplan-Meier), and center effects analyses were performed to examine the association of ECLS use and mortality. RESULTS: Of 5855 ECLS-eligible CDH patients, 1701 (29.1%) received ECLS. "High-risk" patients were best defined as those with a lowest achievable first-day arterial partial pressure of CO2 of ≥60 mm Hg. After propensity score matching, mortality was higher with ECLS (47.8% vs 21.8%, odds ratio 3.3, 95% confidence interval 2.7-4.0, hazard ratio 2.3, P < 0.0001). For the subgroup of high-risk patients, there was lower mortality observed with ECLS (64.2% vs 84.4%, odds ratio 0.33, 95% confidence interval 0.17-0.65, hazard ratio 0.33, P = 0.001). This survival advantage was persistent using multiple matching approaches. However, this ECLS survival advantage was found to occur primarily at high CDH volume centers that offer frequent ECLS for the high-risk subgroup. CONCLUSIONS: Use of ECLS is associated with excess mortality for low- and intermediate-risk neonates with CDH. It is associated with a significant survival advantage among high-risk infants, and this advantage is strongly influenced by center CDH volume and ECLS experience.

Inborn Versus Outborn Delivery in Neonates With Congenital Diaphragmatic Hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a morbid and potentially fatal condition that challenges providers. The aim of this study is to compare outcomes in neonates with prenatally diagnosed CDH that are inborn (delivered in the institution where definitive care for CDH is provided) versus outborn. METHODS: Prenatally diagnosed CDH cases were identified from the Congenital Diaphragmatic Hernia Study Group (CDHSG) database between 2007 and 2019. Using risk adjustment based on disease severity, we compared inborn versus outborn status using baseline risk and multivariable logistic regression models. The primary endpoint was mortality and the secondary endpoint was need for extracorporeal life support (ECLS). RESULTS: Of 4195 neonates with prenatally diagnosed CDH, 3087 (73.6%) were inborn and 1108 (26.4%) were outborn. There was no significant difference in birth weight, gestational age, or presence of additional congenital anomalies. There was no difference in mortality between inborn and outborn infants (32.6% versus 33.8%, P = 0.44) or ECLS requirement (30.9% versus 31.5%, P = 0.73). Among neonates requiring ECLS, outborn status was a risk factor for mortality (OR 1.51, 95% CI 1.13-2.01, P = 0.006). After adjusting for post-surgical defect size, which is not known prenatally, outborn status was no longer a risk factor for mortality for infants requiring ECLS. CONCLUSIONS: Risk of mortality and need for ECLS for inborn CDH patients is not different to outborn infants. Future studies should be directed to establishing whether highest risk infants are at risk for worse outcomes based on center of birth.

Risk Factors for Hemolysis During Extracorporeal Life Support for Congenital Diaphragmatic Hernia.

BACKGROUND: Neonates receiving extracorporeal life support (ECLS) for congenital diaphragmatic hernia (CDH) require prolonged support compared with neonates with other forms of respiratory failure. Hemolysis is a complication that can be seen during ECLS and can lead to renal failure and potentially to worse outcomes. The purpose of this study was to identify risk factors for the development of hemolysis in CDH patients treated with ECLS. METHODS: The Extracorporeal Life Support Organization database was used to identify infants with CDH (2000-2015). The primary outcome was hemolysis (plasma-free hemoglobin >50 mg/dL). Potentially associated variables were identified in the data set. Descriptive statistics and a series of nested multivariable logistic regression models were used to identify associations between hemolysis and demographic, pre-ECLS, and on-ECLS factors. RESULTS: There were 4576 infants with a mortality of 52.5%. The overall mean rate of hemolysis was 10.5% during the study period. In earlier years (2000-2005), the hemolysis rates were 6.3% and 52.7% for roller versus centrifugal pumps, whereas in later years (2010-2015), they were 2.9% and 26.5%, respectively. The fully adjusted model demonstrated that the use of centrifugal pumps was a strong predictor of hemolysis (odds ratio: 6.67, 95% confidence interval: 5.14-8.67). In addition, other risk factors for hemolysis included low 5-min Apgar score, on-ECLS complications (renal, metabolic, and cardiovascular), and duration of ECLS. CONCLUSIONS: In our cohort of CDH patients receiving ECLS over 15 y, the use of centrifugal pumps increased over time, along with the rate of hemolysis. Patient- and treatment-level risk factors were identified contributing to the development of hemolysis.

Management of Congenital Diaphragmatic Hernia Treated With Extracorporeal Life Support: Interim Guidelines Consensus Statement From the Extracorporeal Life Support Organization.

The management of infants with congenital diaphragmatic hernia (CDH) receiving extracorporeal life support (ECLS) is complex. Significant variability in both practice and prevalence of ECLS use exists among centers, given the lack of evidence to guide management decisions. The purpose of this report is to review existing evidence and develop management recommendations for CDH patients treated with ECLS. This article was developed by the Extracorporeal Life Support Organization CDH interest group in cooperation with members of the CDH Study Group and the Children's Hospitals Neonatal Consortium.

Comparing 30-day outcomes between early versus delayed repair of anorectal malformations with perineal or rectovestibular fistulas: An analysis of the ACS NSQIP-Pediatric database.

BACKGROUND: Anorectal malformations (ARMs) have a wide spectrum of presentation ranging from mild defects with perineal fistulas to more severe defects requiring complex management. A primary repair of ARMs with perineal or rectovestibular fistulas has been shown to have good outcomes. However, the timing of the reconstruction is still debated. The aim of this study is to investigate the safety of early versus delayed repair. METHODS: This study was performed using data from the National Surgical Quality Improvement Program-Pediatric (NSQIP-P) from 2012 to 2017. Patients who underwent repair of anorectal malformation with perineal or vestibular fistula were included in the study. Patients with associated diagnosis for Hirschsprung disease, cloaca, rectal prolapse or stenosis, bladder exstrophy, and tracheoesophageal fistula were excluded. 30-day postoperative outcomes included wound and nonwound complications, readmissions, and reoperations. Outcomes were compared by early (≤7 days of age) versus delayed repair (6 weeks to 8 months). RESULTS: A total of 291 patients were included, with 66 in the early and 231 in the delayed group. Patients in the early group were more likely to be male (68.2% vs 31.8%; p < 0.01) and have cardiac risk factors (71.2% vs 49.4%, p < 0.01). The mean operative time was significantly shorter in the early group (90.1 vs 129.6 min; p < 0.01). 30-day complications were not statistically significant between the two groups (p = 0.76). After multivariate analysis, timing of repair did not affect 30-day complications (p = 0.15). CONCLUSION: Our study shows that early repair of low anorectal malformations with a perineal or vestibular fistula appears to be associated with no increase in risk of postoperative complications as compared to delayed repair. At present, the decision remains dependent on the surgeon's experience and judgment. LEVEL OF EVIDENCE: Level III. Retrospective comparative study.

Sources of regional and center-level variability in survival and cost of care for congenital diaphragmatic hernia (CDH).

PURPOSE: Enormous variability in management and cost occurs in CDH care. The purpose of this study was to identify regional mortality and cost patterns underlying this variability. METHODS: This is a retrospective study of neonatal CDH patients at U.S. hospitals using data from the Pediatric Health Information System (PHIS) database (2015-2018). Patients were risk-stratified using CDH Study Group predicted survival (CDHSG-PS), and mortality and costs were assessed by region (East, West, Mid-West, and South) and center. RESULTS: Higher mortality and extracorporeal life support (ECLS) rates were found in the Mid-West and South (p<0.0001). Higher mortality was seen with ECLS among low-volume centers in the South (p=0.007). When broken down by CHDSG-PS, higher severity patients had higher mortality in the Mid-West and South (p=0.038). Cost was significantly lower for high severity nonsurvivors than survivors ($244,005 vs $565,487, p=0.0008). The East spent more on high-severity patients with lower mortality compared to other regions, but also spent 3.5 times more on low severity nonsurvivors than survivors. Costs were higher at high-volume centers for low- and medium-severity patients, but all centers spent the same on high-severity patients. CONCLUSION: Center volume, region, and patient severity all contribute to the complex survival and cost disparities that exist in CDH care. Standardization of care may improve survival and reduce cost variability. TYPE OF STUDY: Retrospective database study. LEVEL OF EVIDENCE: Level II.

Predictors of long ECMO runs for congenital diaphragmatic hernia.

BACKGROUND: Although longer ECMO run times for patients with congenital diaphragmatic hernia (CDH) have been associated with worse outcomes, a large study has not been conducted to examine the risk factors for long ECMO runs. METHODS: The Extracorporeal Life Support Organization (ELSO) Registry from 2000 to 2015 was used to identify predictors of long ECMO runs in CDH patients. A long run was any duration of ≥14 days. Multivariable logistic regression models were used to examine the association between demographics, pre-ECMO blood gas/ventilator settings, comorbid conditions, and therapies on long ECMO runs. RESULTS: There were 4730 CDH-infants examined. The largest association with long ECMO runs was on-ECMO repair (OR: 3.72, 95% CI: 3.013-4.602, p < 0.001) and the use of THAM (OR: 1.463, 95% CI: 1.062-2.016, p = 0.02). Each drop in pH quartile was associated with an increased risk of long ECMO run: pH ≥ 7.3 (reference), pH 7.2-7.9 (OR 1.24, 95% CI: 0.98-1.57, p = 0.07), pH 7.08-7.19 (OR 1.46, 95% CI: 1.17-1.84, p = 0.001), pH ≤ 7.07 (OR 1.64, 95% CI: 1.29-2.07, p < 0.001). CONCLUSIONS: We found a correlation between both pre-ECMO demographics/timing of repair and the subsequent risk of long ECMO runs, providing insight for both providers and parents about the risk factors for longer runs. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: Level III.

The development of multiorgan dysfunction in CDH-ECMO neonates is associated with the level of pre-ECMO support.

PURPOSE: Congenital diaphragmatic hernia (CDH) is the most common indication for neonatal extracorporeal membrane oxygenation (ECMO), but mortality remains at 50%. Multiorgan failure can occur in 25% and has been linked to worse outcomes. We sought to examine the factors that would increase the risk of multiorgan dysfunction (MOD). METHODS: The Extracorporeal Life Support Organization (ELSO) database was used to identify infants with CDH (2000-2015). The primary outcome was MOD, which was defined as the presence of organ failure in ≥2 organ systems. We used a multivariable logistic regression to examine the effect of demographics, pre-ECMO respiratory status, comorbidities, and therapies on MOD. RESULTS: There were a total of 4374 CDH infants who were treated with ECMO. Overall mortality was 52.4%. The risk models demonstrated that pre-ECMO cardiac arrest (OR 1.458, CI: 1.146-1.861, p = 0.002) and hand-bagging (OR 1.461, CI: 1.094-1.963, p = 0.032) had the strongest association with MOD. In addition, other pre-ECMO indicators of disease severity (pH, HFOV, MAP, 5-min APGAR) and pre-ECMO therapies (bicarb, neuromuscular [NM] blockers) were also associated with MOD. CONCLUSIONS: The level of pre-ECMO support has a significant association with the development of MOD, and initiation of ECMO prior to arrest seems to be critical to avoid complications. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.

Prediction tools in congenital diaphragmatic hernia.

Because congenital diaphragmatic hernia (CDH) is characterized by a spectrum of severity, risk stratification is an essential component of care. In both the prenatal and postnatal periods, accurate prediction of outcomes may inform clinical decision-making, care planning, and resource allocation. This review examines the history and utility of the most well-established risk prediction tools currently available, and provides recommendations for their optimal use in the management of CDH patients.