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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. 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. 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). 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. Treatment Study. Level III. [ABSTRACT FROM AUTHOR]

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). 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. 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. 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. Treatment study. Level III. [ABSTRACT FROM AUTHOR]

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. 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). 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). 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. Treatment Study III [ABSTRACT FROM AUTHOR]

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. 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. 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 O 2 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. 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. Qualitative, Survey. IV. [ABSTRACT FROM AUTHOR]

Abstract Purpose Previous studies comparing extracorporeal membrane oxygenation (ECMO) modality for congenital diaphragmatic hernia (CDH) have not accounted for confounding by indication. We therefore hypothesized that using a propensity score (PS) approach to account for selection bias may identify outcome differences based on ECMO modality for infants with CDH. Methods We utilized ELSO Registry data (2000–2016). Patients with CDH were divided to either venoarterial (VA) or venovenous (VV) ECMO. Patients were matched by PS to control for nonrandom treatment assignment. Subgroup analyses were conducted based on timing of CDH repair relative to ECMO. Primary analysis was the "intent-to-treat" cohort based on the initial ECMO mode. Mortality was the primary outcome, and severe neurologic injury (SNI) was a secondary outcome. Results PS matching (3:1) identified 3304 infants (VA = 2470, VV = 834). In the main group, mortality was not different between VA and VV ECMO (OR = 1.01, 95% CI: 0.86–1.18) and there was no difference in SNI between VA and VV (OR = 0.80; 95% CI: 0.63–1.01). For the pre-ECMO CDH repair subgroup, 175 VA cases were matched to 70 VV. In these neonates, mortality was higher for VV compared to VA (OR = 2.10, 95% CI: 1.19–3.69), without any difference in SNI (OR = 1.48; 95% CI: 0.59–3.71). For the subgroup that did not have pre-ECMO CDH repair, 2030 VA cases were matched to 683 VV cases. In this subgroup, VV was associated with 27% lower risk of SNI relative to VA (OR = 0.73, 95% CI: 0.56–0.95) without any difference in mortality (OR = 0.94, 95% CI: 0.79–1.11). Conclusion This study revalidates that ECMO mode does not significantly affect mortality or SNI in infants with CDH. In the subset of infants who require pre-ECMO CDH repair, VA favors survival, whereas, in the subgroup of infants that did not have pre-ECMO CDH repair, VV favors lower rates of SNI. We conclude that neither mode appears consistently superior across all situations, and clinical judgment should remain a multifactorial decision. Level of evidence Level III. [ABSTRACT FROM AUTHOR]

Abstract Pyloric duplication is a rare subset of alimentary tract duplications that can present in a manner almost identical to hypertrophic pyloric stenosis. Diagnosis can typically be made with an upper gastrointestinal series and ultrasound, though axial imaging is occasionally required. Because most of these malformations do not communicate with the lumen, they can be effectively managed with partial excision and ablation of the common wall mucosa. Here we present an 11-day-old female with pyloric duplication that was successfully managed laparoscopically. [ABSTRACT FROM AUTHOR]

Choledocholithiasis is a rare diagnosis in the infant population with poorly defined optimal management. Endoscopic and operative interventions are both technically challenging. Endoscopic retrograde cholangiopancreatography (ERCP), common bile duct exploration and laparoscopic cholecystectomy are typically safe and successful when performed. However, choledocholithiasis has been reported to spontaneously resolve in up to 60% of cases in neonates and infants. We present five cases of choledocholithiasis in infants and a comprehensive review of the literature. Based on our review and experience, we make a recommendation that clinicians should proceed with a selective approach in the management of this disease based on severity of symptoms, co-morbidities, and the available surgical and endoscopic capabilities of each institution. [ABSTRACT FROM AUTHOR]

Background/purpose Restrictions for ECMO in neonates include birth weight less than 2 kg (BW < 2 kg) and/or gestational age less than 34 weeks (GA < 34 weeks). We sought to describe their relationship on mortality. Methods Neonates with a primary diagnosis code of CDH were identified in the Extracorporeal Life Support Organization (ELSO) registry, and logistic regression models were used to examine the effect of BW < 2 kg and GA < 34 weeks on mortality. Results We identified 7564 neonates with CDH. The overall mortality was 50%. There was a significantly higher risk of death with unadjusted odds ratio (OR) 2.39 (95% confidence interval [CI]: 1.53–3.74; P < 0.01) for BW < 2 kg neonates. The adjusted OR of death for BW < 2 kg neonates remained significantly high with over two-fold increase in the odds of mortality when adjusted for potential confounding variables (OR 2.11, 95% CI: 1.30–3.43; P < 0.01). However, no difference in mortality was observed in neonates with GA < 34 weeks. Conclusions While mortality among CDH neonates with a BW < 2 kg was substantially increased, GA < 34 weeks was not significantly associated with mortality. Effort should be made to identify the best candidates for ECMO in this high-risk group and develop treatment strategies to optimize their survival. Type of study Case–Control Study, Retrospective Comparative Study. Level of evidence Level III. [ABSTRACT FROM AUTHOR]