Adrenocortical tumors are uncommon in the pediatric population. We report a case of a pediatric patient with Beckwith Wiedemann Syndrome (BWS) who was found to have bilateral functional adrenal adenomas: one mineralocorticoid-secreting and one androgen-secreting. The patient ultimately underwent staged surgical resection of both masses, resulting in marked reduction in hormone levels. This case highlights the pathophysiology of adrenal adenomas, association between BWS and adrenal masses, and treatment challenges of bilateral adrenal masses.

Objective To evaluate whether neighborhood-level opportunity, measured by the Child Opportunity Index (COI), is associated with short- and long-term outcomes following pediatric extracorporeal membrane oxygenation (ECMO).Design Retrospective observational cohort study.Setting Single quaternary academic children's hospital.Patients Children supported on ECMO between 2009 and 2023 (n = 472).Interventions None.Measurements and Main Results COI quintiles were derived from geocoded residential addresses at the time of ECMO cannulation. Primary outcomes were survival to discharge and one-year survival. Secondary outcomes included ECMO duration, hospital length of stay, and one-year functional morbidity (gastrostomy tube, tracheostomy, or residence in a facility). Multivariable logistic and Cox regression models adjusted for age, sex, race, ethnicity, weight, and comorbidities. Children residing in the lowest COI quintile had lower adjusted odds of survival to discharge (aOR: 0.60, 95% CI: 0.38-0.95) and one-year survival (aOR: 0.57, 95% CI 0.35-0.93). Survival differences emerged early and persisted over time (adjusted HR for mortality 1.36, 95% CI 0.99-1.87). Among cardiac ECMO patients, low COI was associated with significantly higher odds of gastrostomy tube placement and residence in a facility at one year.Conclusions Neighborhood opportunity is independently associated with survival and long-term morbidity following pediatric ECMO, particularly among cardiac patients. These findings highlight the importance of incorporating social context into prognostication, post-ECMO planning, and survivorship care in pediatric critical illness.

Background: Extracorporeal membrane oxygenation (ECMO) in pediatric patients requires monitoring by specialists to optimize outcomes. Practice variability exists among pediatric ECMO centers across the country. We present a bedside pediatric ECMO staffing model with perfusionists that combines personnel expertise and technology. Methods: At our institution, ECMO care is provided in three intensive care units across one floor. Our primary bedside ECMO provider consists of pediatric perfusionists who provide 24/7 coverage of ECMO patients via remote monitoring and hourly bedside rounding. Neonatal and pediatric ECMO patients are supported using the Cardiohelp System(TM), which uses Spectrum Medical Quantum Elite Workstation and Variable Input Patient Electronic Records (VIPER) software that remotely delivers ECMO circuit parameters and alarms digitally to a monitor in a workroom and mobile devices. ECMO education and skills are reinforced via dedicated didactic and simulation sessions by an ECMO program coordinator. We compared institutional complication rates to other centers tracked by ELSO. Results: From 2017 to 2023, 289 cannulations were performed, consisting of a total of 62,742 cumulative ECMO hours, of which 92% of that time there were simultaneous ECMO patients. This rounding model has institutional mortality and complication rates that are comparable to ELSO rates. Conclusion: We describe a bedside ECMO staffing model with perfusionists, in contrast to ECMO specialists seen at other institutions. The complication and mortality rates are comparable to ELSO rates, suggesting the safety of this model. Further exploration regarding resource utilization and costs is warranted.

Background: Pressure readings used in extracorporeal membrane oxygenation (ECMO) guide a variety of clinical interventions. However, several factors affect the accuracy of these measurements. We aimed to determine a model to mathematically predict expected pressure readings for different pediatric ECMO cannulae. Methods: We assembled an in-vitro circuit simulating those used clinically. Flow (mL/min) was adjusted and corresponding pressure drop (mm Hg) across the cannula was documented for various Medtronic Bio-medicus® and Life Support® cannulae. We utilized both water and blood product mixtures of differing hematocrit (Hct) levels – analogous to differing viscosities. Experimentally derived mathematical models were then applied to clinical data. Results: The relationship between flow and pressure drops across different cannulae and at different Hct levels was graphed and analyzed. At the same flow, larger cannula size corresponded to smaller drops in pressure and increased viscosity corresponded to larger drops in pressure. Using regression models of the experimental data, we estimated five exponential equations, which include Hct and flow, to predict expected pressure drop for different pediatric ECMO cannulae. There was high variability in data among patients with significant differences between clinically observed and expected pressures. Conclusion: Our models derived from in-vitro experimentation are the first steps towards defining the pressure-flow relationship and validating drainage pressure as a surrogate measure of intravascular volume for critically ill patients on ECMO. Further studies are still needed to refine these models and validate their clinical applicability.

Esophageal atresia/tracheoesophageal fistula (EA/TEF) causes gastrointestinal (GI) complications that can begin in infancy and extend into adulthood. The etiology of these is frequently multifactorial, and they can adversely affect feeding, growth, respiratory health, and overall quality of life. This review highlights the importance of early diagnosis, multidisciplinary surveillance, and individualized treatment strategies to effectively manage these complications and improve long-term outcomes for affected children. Recent studies have focused on the development of evidence-based surveillance protocols to optimally manage the numerous observed EA/TEF-associated digestive complications. Many of these complications stem from the combination of gastroesophageal dysmotility and mechanical obstruction inherent to EA/TEF and its surgical management, and include gastroesophageal reflux disease, feeding difficulties, dysphagia, eosinophilic esophagitis, and pulmonary consequences of aspiration. Management of GI complications in children with EA/TEF requires careful, multidisciplinary evaluation and treatment, incorporating dietary modifications, feeding therapy, pharmacotherapy, and surgical and endoscopic interventions.

Extracorporeal membrane oxygenation (ECMO) can provide an option for high-risk procedures that may result in cardiopulmonary collapse. The indications for ECMO standby are not well delineated. We describe the experiences of pediatric ECMO standby at two high-volume centers. A retrospective review of pediatric ECMO standby from 2016 to 2023 was performed (n = 394). Data regarding the locations of ECMO standby and the types of procedures were obtained. The primary outcome evaluated was requiring ECMO cannulation during standby. Of the 394 pediatric patients, only 8 (2%) required ECMO cannulation during standby. The indications for ECMO standby were cardiac (84%) and respiratory (16%) complications. Standby locations included the cardiac catheterization suite (55.6%), the operating room (OR) (20.6%), the intensive care unit (ICU) (11.9%), and the interventional radiology (IR) suite (11.9%). Standby within the cardiac catheterization suite included diagnostic only (53%) and interventional (47%), of which 0 and 4 (3.9%) patients required ECMO, respectively. Procedures in OR, IR, or ICU consisted of major surgical procedures (14%), minimally invasive minor procedures (64%), intubations (18%), and transfers/births (4%). Few ECMO standby patients require cannulation; however, it is complicated to predict patient decompensation. Further studies are warranted to delineate which patients would benefit from ECMO standby while balancing cost and resource utilization.

OBJECTIVE:Diagnosing bloodstream infections (BSIs) in patients on extracorporeal membrane oxygenation (ECMO) can be challenging due to circuit-controlled temperature, altered hemodynamics, and questionable reliability of inflammatory markers in critically ill patients. As a result, practice variability exists among ECMO centers regarding routine blood cultures for surveillance of BSI. Our study aimed to evaluate daily surveillance blood cultures in pediatric and neonatal ECMO patients. DESIGN:Retrospective review of daily surveillance blood cultures from January 2021 to July 2023. We evaluated signs, symptoms, and laboratory results in patients with positive blood cultures. SETTING:Academic children's hospital, New York, NY. PATIENTS:All pediatric and neonatal patients supported on ECMO. INTERVENTIONS:None. MEASUREMENTS AND MAIN RESULTS:We identified a cohort of 111 patients (39 neonates and 72 children) who were supported with ECMO. Daily blood cultures were obtained on 1059 (99%) of 1065 completed ECMO days. Overall, 3% (35/1059) of blood cultures were positive, affecting 6% (7/111) of patients. All seven patients had new evidence of infection on the day of their first positive blood culture; six had clinical signs of infection, including new-onset hypotension, hypothermia, or respiratory decline, and the remaining patients had newly elevated inflammatory markers. CONCLUSIONS:Daily surveillance blood cultures are low-yield in our practice with pediatric and neonatal ECMO patients. Our experience shows that changes in clinical status, including abnormal physical or laboratory findings consistent with infection, are associated with BSI, allowing for timely diagnosis. These criteria should prompt as-needed blood cultures, reducing blood draws and preventing costly and unnecessary daily surveillance cultures.

IntroductionExtracorporeal membrane oxygenation (ECMO) is utilized in critically ill neonates with severe cardiopulmonary failure. Hemolysis is a potential complication and is associated with significantly increased morbidity and mortality. The etiology of hemolysis in neonates is multifactorial, including shear forces generated by the ECMO pump, higher flow resistance from smaller tubing and smaller cannulas, the oxygenator, and other patient factors. Centrifugal pumps and oxygenators commonly have shunts with partially occluding clamps to regulate blood flow. We hypothesized that these clamps are significant contributors to hemolysis.MethodAn in vitro study was conducted with three identical ECMO circuits containing an integrated polymethylpentene (PMP) oxygenator and centrifugal pump (Cardiohelp HLS 5.0) and 1/4″ arteriovenous (AV) loop tubing. The circuits were primed with equal components, including expired ABO-compatible packed red blood cells (pRBCs), 25% albumin, 5% albumin, sodium bicarbonate, heparin, and calcium chloride. Circuit A had a completely occluded shunt. Circuit B had a partially occluded shunt, allowing 500 mL/min of shunt flow back to the oxygenator. Circuit C had a fully open shunt, generating 1000 mL/min of shunt flow back to the oxygenator. Plasma-free hemoglobin values were measured serially over 5 days.ResultsBaseline plasma-free hemoglobin levels were equal in all three circuits. Circuit C had the greatest increase in plasma-free hemoglobin daily (26.3 mg/dL/day) compared to Circuit A and Circuit B, which were 14.1 mg/dL/day and 12.9 mg/dL/day, respectively.ConclusionsOur data suggests that partially occluding clamps are not a significant contributor to hemolysis; rather, increased flow through the oxygenator significantly increased the rate of hemolysis in neonatal ECMO circuits.

Over the past decade, several multi-institutional research consortia have formed within the North American pediatric surgical community. In this article, we describe our experience with the creation and implementation of the Eastern Pediatric Surgery Network, a large and comprehensive research consortium designed to produce a wide array of high-quality clinical studies within our subspecialty. In 2020, a vision statement and rules of governance were established at thirteen academic pediatric surgery divisions in the eastern United States. The research consortium was organized based on four major pillars, namely legal ownership of aggregate data, horizontal leadership structure, mandatory participation in adopted studies, and a broad research portfolio that encompasses the full breath of the specialty. Over the past five years, the number of research projects has dramatically expanded over time and includes participation from 24 different medical centers. Despite a lack of dedicated sponsored extramural support for most projects, there have been 28 abstracts presented at national conferences and 12 manuscripts published in peer-reviewed journals. It is our hope that sharing our experience with creating this organization can help to inform others interested in establishing the academic infrastructure to engage in multi-institutional, evidence-based clinical research in other medical specialties and beyond.

Pediatric extracorporeal membrane oxygenation (ECMO) decannulation practices vary widely regarding ligation versus repair of the common carotid artery (CCA) and internal jugular vein (IJV) with potential implications for neurologic outcomes. We examined rates of ischemic cerebral vascular accident (CVA) and vessel patency in pediatric ECMO patients following decannulation. A retrospective review of pediatric ECMO patients (2009-2023) cannulated via cervical approach was conducted, excluding palliative decannulations. Clinical outcome measures were evaluated using descriptive and inferential statistics. Of 140 patients, 49 (35.0%) underwent vessel repair. These patients were more likely to have a cardiac indication for ECMO (57.1% vs . 19.8%; p < 0.001). Median time from cannulation to vessel repair was 97 hours (interquartile range [IQR]: 73-170). Rates of ischemic CVA were not different between groups (8.2% repaired vs. 8.8% ligated; p = 0.899). Surveillance imaging was performed in 49% of repaired patients, with a 95.8% CCA and 55.5% IJV patency rate. High CCA patency rates were observed following cervical vessel repair, with no difference in ischemic CVA compared with ligation. Vessel repair may be beneficial in populations at risk for future mechanical support needs, such as those with complex cardiac disease, as it preserves the option for subsequent ECMO cannulation.

Background: Institutions lack consensus on the management of patients with congenital diaphragmatic hernia (CDH) who are repaired on extracorporeal membrane oxygenation (ECMO). Our study aimed to evaluate risk factors associated with bleeding complications in patients with CDH repaired on ECMO. Methods: A single-institution retrospective review evaluated all patients with CDH who underwent on-ECMO repair between January 2005 and December 2023. A significant bleeding complication post-repair was defined as bleeding necessitating re-operation. The association between preoperative factors and bleeding complications was evaluated. Results: Forty-six patients were included. Bleeding complications developed in 11/46 (24%) patients. Birthweight (2.5 vs. 3.2 kg, p = 0.02), platelet count <100/mm(3) (64% vs. 29%, p = 0.04), elevated blood urea nitrogen (BUN; 24.5 vs. 17.5 mg/dL, p = 0.05), and older age at repair (8 vs. 5 days, p = 0.04) were associated with bleeding. In univariate analysis, patients with platelets under 100/mm(3) were more likely to develop a bleeding complication (OR = 4.4, p = 0.04). Patients who experienced a significant bleeding event experienced increased ECMO days (12 vs. 7 days, p < 0.01), ventilator days (31 vs. 18 days, p < 0.05), and lower survival to discharge (36% vs. 74%, p = 0.03). Conclusion: Among CDH patients undergoing repair on ECMO, those with lower birth weight, platelet counts under 100/mm(3), elevated BUN, and older age at repair had an increased risk of a significant bleeding complication, resulting in more ECMO and ventilator days and higher mortality. Patients undergoing on-ECMO repair should have platelet count transfused to greater than 100/mm(3). Patients at high risk for bleeding may benefit from early repair on ECMO.

AbstractPediatric pulmonary arterial hypertension (PAH) can present with a wide spectrum of disease severity. Pulmonary hypertension (PH) crises can lead to acute decompensation requiring extracorporeal membrane oxygenation (ECMO) support, including extracorporeal cardiopulmonary resuscitation (eCPR). We evaluated outcomes for pediatric PH patients requiring ECMO. A single‐institution retrospective review of pediatric PAH patients with World Symposium on PH (WSPH) groups 1 and 3 requiring ECMO cannulation from 2010 through 2022 (n = 20) was performed. Primary outcome was survival to hospital discharge. Secondary outcomes were survival to decannulation and 1‐year survival. Of 20 ECMO patients, 16 (80%) survived to decannulation and 8 (40%) survived to discharge and 1 year follow up. Of three patients who had two ECMO runs; none survived. There were five patients who had eCPR for the first run; one survived to discharge. The univariate logistic regression model showed that venovenous ECMO was associated with better survival to hospital discharge than venoarterial ECMO, (OR: 0.12, 95% CI: 0.01–0.86, p = 0.046). PH medications (administered before, during, or after ECMO) were not associated with survival to discharge. For children with decompensated PAH requiring ECMO, mortality rate is high, and management is challenging. While VA ECMO is the main configuration for decompensated PH, VV ECMO could be considered if there is adequate ventricular function, presence of a systemic to pulmonary shunt, or an intercurrent treatable illness to improve survival to discharge. A multidisciplinary approach with requisite expertise should be utilized on a case‐by‐case basis until more reliable data is available to predict outcomes.

BACKGROUND:Congenital diaphragmatic hernia (CDH) affects more than 1100 newborns in the United States each year. Severity of clinical presentation is highly variable. Standardized care improves outcomes by promoting consistency in decision-making and clarifying goals of treatment, but CDH management has not yet been standardized. METHODS:We performed a comprehensive literature review with special consideration for-cardiac dysfunction, indications for extracorporeal membrane oxygenation (ECMO), and timing of repair. In collaboration with experts across specialties, we sought to develop and implement a treatment algorithm based on current CDH literature and our own institutional experience. RESULTS:Left ventricular (LV) hypoplasia and dysfunction is increasingly recognized as an important contributor to the severity of clinical presentation and cardiac dysfunction seen with CDH. Cardiac dysfunction is associated with poor outcomes and increased mortality. CDH-associated severe hypoxic respiratory failure refractory to medical therapy is one of the most common indications for ECMO in the neonatal period. The decision to initiate ECMO and selection of configuration should be shared by members of a multidisciplinary care team. The optimal timing of repair with respect to ECMO has been evolving in the last 3 decades. CONCLUSION:Following our review, we recommend (1) timely and detailed cardiac evaluation with echocardiogram after birth, and (2) early repair on ECMO for high-risk patients and delayed repair post-ECMO for low-risk patients with anticipated short ECMO run. This treatment algorithm is a step toward standardization of CDH management practices, which we expect will improve CDH outcomes at our institution and others.

Infantile hemangiomas (IHs) are the most common tumors of infancy and, in rare instances, can present in the setting of congenital structural anomalies or as part of syndromic disorders. In this study, we present three cases of children with segmental IHs born with concurrent pulmonary anomalies: congenital pulmonary airway malformations and bronchopulmonary sequestration. To date, no known association between these entities and hemangiomas has been described.

Fibrous hamartoma of infancy is a benign tumor that typically arises within the first 2 years of life in the subcutaneous and lower dermal layers. Diagnosis can be challenging as it is a rare tumor, and the imaging appearance is not well known. To describe the imaging features in 4 cases of fibrous hamartoma of infancy focusing on ultrasound (US) and magnetic resonance (MR) findings. In this retrospective IRB-approved study, informed consent was waived. We searched patient charts for histopathology-confirmed fibrous hamartoma of infancy diagnosis between November 2013 and November 2022. We found four cases, three boys and one girl, and the mean age was 1.4 years (5 months—3 years). The lesions were located in the axilla, posterior elbow, posterior neck, and lower back. All four patients underwent ultrasound evaluation of the lesion, and two patients also underwent MRI evaluation. The imaging findings were reviewed by consensus by two pediatric radiologists. US imaging revealed subcutaneous lesions with variably defined hyperechoic regions and intervening hypoechoic bands resulting in a linear “serpentine” pattern or a “multiple semicircle” pattern. MR imaging evidenced heterogeneous soft tissue masses, localized in the subcutaneous fat, and showed hyperintense fat interspersed with hypointense septations on both T1- and T2-weighted images. Fibrous hamartoma of infancy has a suggestive appearance on US with heterogeneous, echogenic subcutaneous lesions with intervening hypoechoic portions, in parallel or circumferential arrangement that can be seen as a serpentine or semicircular pattern. On MRI, interspersed macroscopic fatty components show high signal intensity on T1- and T2-weighted images and reduced signal on fat-suppressed inversion recovery images, with irregular peripheral enhancement.

Background: Less than 10% of infants born with esophageal atresia (EA) have a long gap that precludes primary anastomosis at birth. The purpose of this study was to evaluate the diagnosis, management, and outcomes of infants with long-gap esophageal atresia (LGEA) within a regional consortium of children's hospitals. Methods: After IRB approval, a multicenter, retrospective cohort study was conducted of LGEA patients managed by 13 member institutions between 2009 and 2018. LGEA was defined as Type A or B esophageal atresia with the inability to perform a primary esophageal anastomosis at birth based on preoperative imaging or operating findings. Study outcomes, including operative repair, postoperative outcomes, and complications, were collected to detect significant associations between variables (p < 0.05). Results: There were 62 LGEA patients identified, including 50 (81%) with Type A and twelve (19%) with Type B esophageal atresia. Most (77%) were diagnosed prenatally with 98% undergoing a gastrostomy before attempted EA repair. The mean gap length at repair was 3.24 +/- 1.59 cm (n = 21). Most (95%) were managed with delayed repair (median age at repair of 96 days (IQR: 67.5-131), and 22 (35%) underwent an esophageal traction-induced lengthening process. Two (3.2%) required esophageal replacement. There was no significant difference in complications between different treatment strategies. Conclusions: In this collaborative descriptive study of 62 infants with LGEA, delayed primary repair with or without traction was the preferred approach, with outcomes that were comparable between strategies, and with high rates of esophageal preservation. We encountered variability in gap length measurement and reporting. This study highlights a critical need for a prospective, multi-institutional registry with uniform care pathways to help aid in the development of evidence-based guidelines for these challenging patients.

Infants born with esophageal atresia and tracheoesophageal fistula, a complex congenital malformation occurring in 1/2500–4000 live births, may suffer threats to their cardiac, respiratory, and digestive health in addition to anomalies that may exist in the genitourinary and musculoskeletal systems. Optimal care for these patients throughout their lives is best achieved through a coordinated, multidisciplinary approach that our health care system is not always well-equipped to provide. This review, though not exhaustive, highlights the components of care that pertain to initial surgical reconstruction and subsequent diagnosis and management of the complications that are most frequently encountered. Authors from among the many specialties involved in the care of these patients summarize the current best practice with attention to the most recent advances. Assessment and improvement of quality of life and transition to adult specialists as children grow to adulthood is also reviewed.