Congenital anomalies and predisposition to severe COVID-19 among pediatric patients in the United States.

BACKGROUND AND OBJECTIVE: Congenital heart defects are known to be associated with increased odds of severe COVID-19. Congenital anomalies affecting other body systems may also be associated with poor outcomes. This study is an exhaustive assessment of congenital anomalies and odds of severe COVID-19 in pediatric patients. METHODS: Data were retrieved from the COVID-19 dataset of Cerner® Real-World Data for encounters from March 2020 to February 2022. Prior to matching, the data consisted of 664,523 patients less than 18 years old and 927,805 corresponding encounters with COVID-19 from 117 health systems across the United States. One-to-one propensity score matching was performed, and a cumulative link mixed-effects model with random intercepts for health system and patients was built to assess corresponding associations. RESULTS: All congenital anomalies were associated with worse COVID-19 outcomes, with the strongest association observed for cardiovascular anomalies (odds ratio [OR], 3.84; 95% CI, 3.63-4.06) and the weakest association observed for anomalies affecting the eye/ear/face/neck (OR, 1.16; 95% CI, 1.03-1.31). CONCLUSIONS AND RELEVANCE: Congenital anomalies are associated with greater odds of experiencing severe symptoms of COVID-19. In addition to congenital heart defects, all other birth defects may increase the odds for more severe COVID-19. IMPACT: All congenital anomalies are associated with increased odds of severe COVID-19. This study is the largest and among the first to investigate birth defects across all body systems. The multicenter large data and analysis demonstrate the increased odds of severe COVID19 in pediatric patients with congenital anomalies affecting any body system. These data demonstrate that all children with birth defects are at increased odds of more severe COVID-19, not only those with heart defects. This should be taken into consideration when optimizing prevention and intervention resources within a hospital.

Racial Disparity among Youth Decedents of Legal Intervention Trauma.

BACKGROUND: Legal intervention trauma (LIT) is defined as injury due to any encounter with law enforcement. This study investigates associations between demographics, violent status, and law enforcement tactics among youth decedents of LIT. STUDY DESIGN: Decedents of LIT age 26 years or younger were identified using the CDC's National Violent Death Reporting System from 2003 to 2018. Decedents were classified as "violent" if they possessed a weapon, were committing a violent crime, or if law enforcement reported justified use of force. All others were classified as "nonviolent." Law enforcement tactics were stratified into "lethal" (firearm with standard ammunition) or "less lethal" (any other) force. Differences in the racial distribution across these classifications were assessed using chi-square tests of proportions. RESULTS: We identified 1,281 youth decedents of LIT; of which, 92.5% met violent criteria. Black youths were less likely than White youths to possess a weapon (71.6% vs 77.4%, p = 0.02) and were not more likely to be committing a violent crime (63.6% vs 60.4%, p = 0.27). They were, however, more likely than White youths to experience force reported as justified by law enforcement (89.9% vs 82.4%, p = 0.002) and to experience exclusively lethal force not preceded by less-lethal tactics (94.0% vs 88.7%, p = 0.001). Among the subset of 85 cases where law enforcement reported justified use of force despite the decedent not possessing a weapon or committing a violent crime, the precipitating event was more often a traffic stop for Black youths than for White youths (28.5% vs 6.66%, p = 0.02). CONCLUSIONS: These findings indicate a racial disparity among youth decedents of LIT.

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.

A Novel VACTERL Assessment Tool to Facilitate Counseling for Expectant Families.

INTRODUCTION: VACTERL is defined as 3 or more of the following congenital defects: vertebral, anorectal, cardiac, tracheoesophageal (TE), renal, and limb. The purpose of this study was to create an easy-to-use assessment tool to help providers counsel expecting families regarding the likelihood of additional anomalies and postnatal outcomes. METHODS: Employing the Kids' Inpatient Database from 2003-2016, neonates (<29 days old) with VACTERL were identified using ICD-9-CM and ICD-10-CM codes. For each unique combination of VACTERL, multivariable logistic regression was used to estimate inpatient mortality, and Poisson regression was used to estimate length-of-stay during the initial hospitalization. RESULTS: The assessment tool used in this study is available at https://choc-trauma.shinyapps.io/VACTERL. 1,886 of 11,813,782 (0.016%) neonates presented with VACTERL. 32% weighed <1,750 g, and 239 (12.7%) died prior to discharge. Associated with mortality were limb anomaly (1.8 [1.01-3.22], p < 0.05), prematurity (1.99 [1.14-3.47], p < 0.02), and weight <1,750 g (2.19 [1.25-3.82], p < 0.01). Median length-of-stay was 14 days (IQR: 7-32). Associated with increased length-of-stay were cardiac defect (1.47 [1.37-1.56], p < 0.001), vertebral anomaly (1.1 [1.05-1.14], p < 0.001), TE fistula (1.73 [1.66-1.81], p < 0.001), anorectal malformation (1.12 [1.07-1.16], p < 0.001), and weight <1,750 g (1.65 [1.57-1.73], p < 0.001). CONCLUSION: This novel assessment tool may help providers counsel families confronting a VACTERL diagnosis.

A pilot study of multi-modal pain management for same-day discharge after minimally invasive repair of pectus excavatum (Nuss procedure) in children.

BACKGROUND: Despite advancements in minimally invasive repair of pectus excavatum (MIRPE), Nuss procedure, postoperative pain control remains challenging. This report covers a multimodal regimen using bilateral single-shot paravertebral block (PVB) and bilateral thoracoscopic intercostal nerve (T3-T7) cryoablation, leading to significant reduction in length of stay (LOS) and high rate of same-day discharge. METHODS: This is a comparative study of pain management protocols for patients undergoing the Nuss procedure at a single center from 2016 through 2020. All patients underwent the the same surgical technique for the treatment of pectus excavatum at a single center. Patients received bilateral PVB with continuous infusion (Group 1, n = 12), bilateral PVB with infusion and right-side cryoablation (Group 2, n = 9), or bilateral single-shot PVB and bilateral cryoablation (Group 3, n = 17). The primary outcome was LOS with focus on same-day discharge, and the secondary outcome was decreased opioid usage. RESULTS: Eleven of 17 patients in Group 3 (65%) (bilateral single-shot PVB and bilateral cryoablation) were discharged the same day as surgery. The remaining Group 3 patients were discharged the following day with no complications or interventions. Compared to Group 1 (no cryoablation), Group 3 had shorter LOS (median 4.4 days vs. 0.7 days, respectively, p < 0.001) and significantly decreased median opioid use on the day of surgery (0.92 mg/kg vs. 0.47 mg/kg, p = 0.006). CONCLUSION: Findings demonstrate the feasibility of multimodal pain management for same-day discharge after the Nuss procedure. Future multisite studies are needed to investigate the superiority of this approach to established methods. LEVEL OF EVIDENCE: III.

Single-center comparison of outcomes between laparoscopic appendectomy and transumbilical laparoscopic assisted appendectomy.

PURPOSE: Appendectomy is the most common pediatric emergency surgery performed to date. This study compared outcomes between laparoscopic appendectomy (LA) and transumbilical laparoscopic assisted appendectomy (TULAA) for 1154 uncomplicated patients across 5 years at a single institution. Primary outcomes include length of stay (LOS), post-operative complications, pain score, and operating room (OR) time. METHODS: Demographic and clinical data was collected for 1154 eligible patients treated for uncomplicated appendicitis between August 2014-October 2019, with 830 patients in the LA group, and 324 in the TULAA group. Mixed effects modeling procedure using logistic and linear regression examined the effect of surgery type on the four primary outcomes after adjustment for potential clustering effect of surgeon and confounding factors. RESULTS: Of 1154 patients, 62.7% were male, and mean (SD) age was 10.9 (3.6) years. Median [IQR] LOS was 28.0 h [22.0, 36.0], mean (SD) OR time was 29.0 (10.0) minutes, and median [IQR] pain at maximum level was 5.5 (2.7). The complication rate overall was <5.0% and did not differ between TULAA and LA groups (p > 0.05). OR time was reduced by an average of 5.2 min in the TULAA group (p < 0.001), pain did not differ between groups overall (p > 0.05), and patients were more likely to be discharged within 24 h in patients who underwent TULAA (OR = 5.3 [1.6, 17.4], p = 0.007). CONCLUSION: Retrospective analysis of 1154 pediatric appendectomies, found no difference in complications between single- and three-incision laparoscopic procedures (TULAA vs. LA). Findings suggest TULAA is a safe procedure for acute appendicitis in pediatrics. LEVEL OF EVIDENCE: IV.

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.

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.

The role of race in pediatric legal intervention as a cause of injury.

BACKGROUND: Previous studies have assessed the prevalence and nature of traumatic injuries due to legal intervention in adults. The purpose of this study is to characterize and understand legal intervention trauma in children. METHODS: The National Trauma Data Bank (NTDB) was queried from 2007 to 2015. Patients (0-18 years old) who sustained injuries due to legal intervention were compared to those injured from other causes in the general NTDB population. Descriptive statistics were used to characterize the study population. Multivariate logistic regression models were utilized to predict firearm trauma and mortality. RESULTS: 622 patients sustained injuries involving legal intervention. Compared to general NTDB pediatric population, those who sustained legal intervention injuries were more likely to be older (age 16.5 vs. 10.6, p < 0.01), male (91.96% vs. 34.95%, p < 0.01), test positive for illegal drugs (64.64% vs. 38.35%, p < 0.01) or alcohol (26.36% vs. 17.25%, p < 0.01), and be African-American (44.37% vs. 17.00%, p < 0.01), Latino (22.82% vs. 15.10%, p < 0.01), or Native American (0.96% vs.. 0.94%, p < 0.01). Logistic regression models identified an 11% increased odds (95% CI 1.02-1.21, p = 0.02) of death among African-Americans when compared to other racial groups receiving legal intervention trauma. African-American status was associated with a 12% increased odds (95% CI 1.02-1.22, p = 0.01) of firearm trauma when compared to other racial groups receiving legal intervention trauma. CONCLUSION: Legal intervention-related pediatric trauma disproportionately affects the African-American population. This is particularly pronounced in cases of firearm related injuries. LEVEL OF EVIDENCE: III.

Development and validation of machine learning models for the prediction of blunt cerebrovascular injury in children.

BACKGROUND: Blunt cerebrovascular injury (BCVI) is a rare finding in trauma patients. The previously validated BCVI (Denver and Memphis) prediction model in adult patients was shown to be inadequate as a screening option in injured children. We sought to improve the detection of BCVI by developing a prediction model specific to the pediatric population. METHODS: The National Trauma Databank (NTDB) was queried from 2007 to 2015. Test and training datasets of the total number of patients (885,100) with complete ICD data were used to build a random forest model predicting BCVI. All ICD features not used to define BCVI (2268) were included within the random forest model, a machine learning method. A random forest model of 1000 decision trees trying 7 variables at each node was applied to training data (50% of the dataset, 442,600 patients) and validated with test data in the remaining 50% of the dataset. In addition, Denver and Memphis model variables were re-validated and compared to our new model. RESULTS: A total of 885,100 pediatric patients were identified in the NTDB to have experienced blunt pediatric trauma, with 1,998 (0.2%) having a diagnosis of BCVI. Skull fractures (OR 1.004, 95% CI 1.003-1.004), extremity fractures (OR 1.001, 95% 1.0006-1.002), and vertebral injuries (OR 1.004, 95% CI 1.003-1.004) were associated with increased risk for BCVI. The BCVI prediction model identified 94.4% of BCVI patients and 76.1% of non-BCVI patients within the NTDB. This study identified ICD9/ICD10 codes with strong association to BCVI. The Denver and Memphis criteria were re-applied to NTDB data to compare validity and only correctly identified 13.4% of total BCVI patients and 99.1% of non BCVI patients. CONCLUSION: The prediction model developed in this study is able to better identify pediatric patients who should be screened with further imaging to identify BCVI. LEVEL OF EVIDENCE: Retrospective diagnostic study-level III evidence.

Analysis of Unintentional Falls in Pediatric Population and Predictors of Morbidity.

INTRODUCTION: Unintentional falls are a leading cause of pediatric traumatic injury. This study evaluates clinical outcomes of fall-related injuries in children under the age of 10. METHODS: The National Trauma Database was queried for children who experienced an unintentional fall. Patients were stratified by age in two groups: 1-5 and 6-10 years old. The primary outcome was post discharge extension of care, defined as transfer to skilled nursing facility or rehabilitation center after discharge from the hospital. Descriptive statistics and a multivariable logistic regression analysis were used to compare the two groups. RESULTS: From 2009 to 2016, a total of 8,277 pediatric patients experienced an unintentional fall, with 93.6% of patients being discharged home. Falls were more common in younger children, with greater odds of post discharge extension of care. Predictors of increased associated risk of extended medical care included intracranial hemorrhage (OR 1.05, 95% CI 1.03-1.06) and thoracic injuries (OR 1.03, 95% CI 1.00-1.1.05) (P< 0.05). Mortality in pediatric patients suffering unintentional falls was a rare event occurring in 0.7% of cases in children 1-5 years old and 0.4% of children 6-10 years old. CONCLUSION: The majority of children experiencing an unintentional fall are discharged home, with mortality being very rare. However, younger age is prone to more severe and serious injury patterns. Intracranial hemorrhage and thoracic injury were a predictor of need for extended medical care.

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.

A Nested Mixed Effects Multicenter Model Examining the Risk Factors for Pediatric Trauma Return Visits Within 72 Hours.

BACKGROUND: Return visits within 72 h are an important metric in evaluating the performance of emergency rooms. This has not been well studied in the pediatric trauma population. We sought to determine novel risk factors for return visits to the emergency department (ED) after trauma that may assist in identifying patients most at risk of revisit. METHODS: We used the Cerner Health Facts Database to retrieve data from 34 EDs across the United States that care for pediatric trauma patients aged <15 y. The data consist of 610,845 patients and 816,571 ED encounters. We retrieved variables encompassing demographics, payor, current and past health care resource utilization, trauma diagnoses, other diagnoses/comorbidities, medications, and surgical procedures. We built a nested mixed effects logistic regression model to provide statistical inference on the return visits. RESULTS: Traumas resulting from burns and corrosion, injuries to the shoulder and arms, injuries to the hip and legs, and trauma to the head and neck are all associated with increased odds of returning to the ED. Patients suffering from poisoning relating to drugs and other biological substances and patients with trauma to multiple body regions have reduced odds of returning to the ED. Longer ED length of stay and prior health care utilization (ED or inpatient) are associated with increased odds of a return visit. The sex of the patient and payor had a statistically significant effect on the risk of a return visit to the ED within 72 h of discharge. CONCLUSIONS: Certain traumas expose patients to an increased risk for return visits to the ED and, as a result, provide opportunity for improved quality of care. Targeted interventions that include education, observation holds, or a decision to hospitalize instead of discharge home may help improve patient outcomes and decrease the rate of ED returns. LEVEL OF EVIDENCE: III (Prognostic and Epidemiology).

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.

The role of ECMO in the management of congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is the most common indication for extra-corporeal membrane oxygenation (ECMO) for neonatal respiratory failure. CDH management is evolving with advanced prenatal diagnostic imaging modalities. The risk profiles of infants receiving ECMO for CDH are shifting towards higher risk. Many clinicians are developing and following clinical practice guidelines to standardize and optimize the care of CDH neonates. Despite these efforts, there are significant differences in the practice patterns among ECMO centers as to how and when they choose to initiate ECMO for CDH, when they believe repair is safe, as well as many other nuances that are based on center experience or style. The purpose of this report is to summarize our current understanding of the new and recent developments regarding management of infants with CDH managed with ECMO.

Potential survival benefit with repair of congenital diaphragmatic hernia (CDH) after extracorporeal membrane oxygenation (ECMO) in select patients: Study by ELSO CDH Interest Group.

PURPOSE: Studying the timing of repair in CDH is prone to confounding factors, including variability in disease severity and management. We hypothesized that delaying repair until post-ECMO would confer a survival benefit. METHODS: Neonates who underwent CDH repair were identified within the ELSO Registry. Patients were then divided into on-ECMO versus post-ECMO repair. Patients were 1:1 matched for severity based on pre-ECMO covariates using the propensity score (PS) for the timing of repair. Outcomes examined included mortality and severe neurologic injury (SNI). RESULTS: After matching, 2,224 infants were included. On-ECMO repair was associated with greater than 3-fold higher odds of mortality (OR 3.41, 95% CI: 2.84-4.09, p<0.01). The odds of SNI was also higher for on-ECMO repair (OR 1.49, 95% CI: 1.13-1.96, p<0.01). A sensitivity analysis was performed by including the length of ECMO as an additional matching variable. On-ECMO repair was still associated with higher odds of mortality (OR 2.38, 95% CI: 1.96-2.89, p<0.01). Results for SNI were similar but were no longer statistically significant (OR 1.33, 95% CI: 0.99-1.79, p=0.06). CONCLUSIONS: Of the infants who can be liberated from ECMO and undergo CDH repair, there is a potential survival benefit for delaying CDH repair until after decannulation. TYPE OF STUDY: Treatment Study LEVEL OF EVIDENCE: III.

Management preferences in ECMO mode for congenital diaphragmatic hernia.

PURPOSE: The purpose of this study was to identify management preferences that may exist in the care of infants with CDH receiving ECMO with emphasis on VV-ECMO. METHODS: A survey was created to measure treatment preferences regarding ECMO use in CDH. The survey was distributed to all APSA and ELSO/Euro-ELSO members via e-mail. Survey results were summarized using descriptive statistics. RESULTS: The survey had 230 respondents. The survey participants were surgeons (75%), neonatologists/intensivists (23%), and "other" (2%). The mean annual center volume was 11.6(±9.6) CDH cases, and the average number treated with ECMO was 4.5 (±6.4) cases/yr. The most agreed upon criteria for ECMO initiation were preductal O2 saturation <80% refractory to ventilator manipulation and medical therapy (89%), oxygenation index >40 (80%), severe air-leak (79%), and mixed acidosis (75%). Over 60% of respondents agreed the VV-ECMO would be optimum for average risk neonates. However, this preference diminished as the pre-ECMO level of cardiac support increased. When asked about why each respondent would choose VA-ECMO over VV-ECMO, the responses varied significantly between surgeons and non-surgeons. CONCLUSION: While there seem to be areas of consensus among practitioners, such as criteria for initiation of ECMO, this survey revealed substantial variation in individual practice patterns regarding the use of ECMO for CDH. TYPE OF STUDY: Qualitative, Survey. LEVEL OF EVIDENCE: IV.

Trends in Mortality and Risk Characteristics of Congenital Diaphragmatic Hernia Treated With Extracorporeal Membrane Oxygenation.

Although the mortality of infants with congenital diaphragmatic hernia (CDH) has been improving since the late 1990s, this observation has not been paralleled among the CDH cohort receiving extracorporeal membrane oxygenation (ECMO). We sought to elucidate why the mortality rate in the CDH-ECMO population has remained at approximately 50% despite consistent progress in the field by examining the baseline risk profile/characteristics of neonates with CDH before ECMO (pre-ECMO). Neonates with a diagnosis of CDH were identified in the Extracorporeal Life Support Organization (ELSO) Registry from 1992 to 2015. Individual pre-ECMO risk score (RS) for mortality was categorized to pre-ECMO risk-stratified cohorts. Temporal trends based on individual-level mortality by risk cohorts were assessed by logistic regression. We identified 6,696 neonates with CDH. The mortality rates during this time period were approximately 50%. The average baseline pre-ECMO RS increased during this period: mean increase of 0.35 (95% confidence interval [CI]: 0.324-0.380). In the low-risk cohort, the likelihood of mortality increased over time: each 5 year change was associated with a 7.3% increased likelihood of mortality (odds ratio [OR]: 1.0726; 95% CI: 1.0060-1.1437). For the moderate-risk cohort, the likelihood of mortality decreased by 7.05% (OR: 0.9295; 95% CI: 0.8822-0.9793). There was no change in the odds of mortality for the high-risk cohort (OR: 0.9650; 95% CI: 0.8915-1.0446). Although the overall mortality rate remained approximately constant over time, the individual likelihood of death has declined over time in the moderate-risk cohort, increased in the low-risk cohort, and remained unchanged in the high-risk cohort.

Effect of pump type on outcomes in neonates with congenital diaphragmatic hernia requiring ECMO.

PURPOSE: With the exception of neonatal respiratory failure, most centers are now using centrifugal over roller-type pumps for the delivery of extracorporeal membrane oxygenation (ECMO). Evidence supporting the use of centrifugal pumps specifically in infants with congenital diaphragmatic hernia (CDH) remains lacking. We hypothesized that the use of centrifugal pumps in infants with CDH would not affect mortality or rates of severe neurologic injury (SNI). METHODS: Infants with CDH were identified within the ELSO registry (2000-2016). Patients were then divided into those undergoing ECMO with rollertype pumps or centrifugal pumps. Patients were matched based on propensity score (PS) for the ECMO pump type based on pre-ECMO covariates. This was done for all infants and separately for each ECMO mode, venovenous (VV) and venoarterial (VA) ECMO. RESULTS: We identified 4,367 infants who were treated with either roller or centrifugal pumps from 2000-2016. There was no difference in mortality or SNI between the two pump types in any of the groups (all infants, VA-ECMO infants, VV-ECMO infants). However, there was at least a six-fold increase in the odds of hemolysis for centrifugal pumps in all groups: all infants (odds ratio [OR] 6.99, p<0.001), VA-ECMO infants (OR 8.11, p<0.001 and VV-ECMO infants (OR 9.66, p<0.001). CONCLUSION: For neonates with CDH requiring ECMO, there is no survival advantage or difference in severe neurologic injury between those receiving roller or centrifugal pump ECMO. However, there is a significant increase in red blood cell hemolysis associated with centrifugal ECMO support.