Risk Factors and Outcomes of Perioperative Extracorporeal Membrane Oxygenation in Neonates and Infants Undergoing Truncus Arteriosus Repair.

Despite surgical advances, neonatal truncus arteriosus repair remains high risk and approximately 10% of patients receive perioperative extracorporeal membrane oxygenation (ECMO). We aimed to assess factors and outcomes associated with the use of perioperative ECMO in infants undergoing truncus arteriosus repair. We conducted a retrospective cohort study of patients who underwent truncus arteriosus repair between 2004 and 2019, using administrative data from the Pediatric Health Information System database.

Long-term follow-up in congenital diaphragmatic hernia.

With improvements in initial care for patients with congenital diaphragmatic hernia (CDH), the number of CDH patients with severe disease who are surviving to discharge has increased. This growing population of patients faces a unique set of long-term challenges, multisystem adverse outcomes, and post-intervention complications requiring specialized multidisciplinary follow-up. Early identification and intervention are essential to mitigate the potential morbidity associated with these challenges.

Neonatal Extracorporeal Membrane Oxygenation: Associations between Continuous Renal Replacement Therapy, Thrombocytopenia, and Outcomes.

Introduction: The incidence of thrombocytopenia in neonates receiving extracorporeal membrane oxygenation (ECMO) with and without concurrent continuous renal replacement therapy (CRRT) and associated complications have not been well described. The primary aims of the current study were to (1) characterize thrombocytopenia in neonates receiving ECMO (including those treated concurrently with CRRT) and (2) evaluate risk factors (including CRRT utilization) associated with severe thrombocytopenia. In a planned exploratory secondary aim, we explored the association of severe thrombocytopenia with outcomes in neonates receiving ECMO.

Systemic anticoagulation in ECMO.

While unfractionated heparin (UFH) remains the mainstay of anticoagulation during pediatric extracorporeal life support, direct thrombin inhibitors (DTIs) are increasingly used. In this article, we will review most recent evidence regarding utilization of both UFH and DTIs and compare their known advantages and disadvantages. We will present anticoagulation monitoring strategies during ECMO and outline the most recent Extracorporeal Life Support Organization's anticoagulation guidelines, however with the caveat that there are no true consensus recommendations for anticoagulation management in pediatric ECMO.

Complication accrual impacts pediatric extracorporeal life support mortality.

Introduction: This study tested the hypothesis that complication accrual during pediatric extracorporeal life support (ECLS) increases mortality irrespective of indication for support.

Methods: Prospectively collected Extracorporeal Life Support Organization (ELSO) registry data for all neonatal and pediatric patients cannulated for ECLS at our institution from 1/1/2015 to 12/31/2020 was stratified based on the presence or absence of complications. We excluded renal replacement therapy from complications, as this is frequently and empirically applied within our practice.

Keto-Bezoar: Adverse Event Related to Initiation of Ketogenic Diet in an Infant.

The ketogenic diet is frequently used as part of the treatment regimen for pediatric patients with refractory epilepsy. This diet is generally well tolerated, with constipation being the most described side effect. This case highlights a previously undocumented severe complication of a "keto-bezoar" formation related to the initiation of the ketogenic diet in a young infant.

Emanuel syndrome and congenital diaphragmatic hernia: A systematic review.

Background: Emanuel Syndrome (ES), a rare chromosomal disorder caused by a supernumerary chromosome 22 derivative (der(22)t(11;22)), was identified in a fetus with congenital diaphragmatic hernia (CDH) at our fetal center. We aimed to identify a precedent for clinical care and patient outcomes to guide family decision-making.

Methods: This non-funded and non-registered study queried the entire CDH Registry (CDHR) including >10,000 patients since 1995 and conducted a systematic literature review for patients with concomitant ES and CDH.

Long term follow-up in congenital diaphragmatic hernia.

Survivorship of patients with congenital diaphragmatic hernia (CDH) has created a unique cohort of children, adolescents and adults with complex medical and surgical needs. Morbidities specific to this disease benefit from multi-specialty care, and the long term follow up of these patients offers a tremendous opportunity for research and collaboration. Herein we aim to offer an overview of the challenges that modern CDH survivors face, and include a risk-stratified algorithm as a general guideline for a multi-specialty follow up program.

Long-term follow-up of congenital diaphragmatic hernia.

Increased survival of patients with congenital diaphragmatic hernia has created a unique cohort of children, adolescent, and adult survivors with complex medical and surgical needs. Disease-specific morbidities offer the opportunity for multiple disciplines to unite together to provide long-term comprehensive follow-up, as well as an opportunity for research regarding late outcomes. These children can exhibit impaired pulmonary function, altered neurodevelopmental outcomes, nutritional insufficiency, musculoskeletal changes, and specialized surgical needs that benefit from regular monitoring and intervention, particularly in patients with increased disease severity.

A risk-stratified analysis of delayed congenital diaphragmatic hernia repair: does timing of operation matter?

Background: Congenital diaphragmatic hernia (CDH) remains a significant cause of death in newborns and, despite improved outcomes with multimodality therapies, optimal timing of repair remains undefined. We sought to evaluate the influence of surgical timing on patient outcomes and hypothesized that delayed repair does not improve survival in CDH.

Methods: Prospectively collected data from 1,385 CDH Registry infants without preoperative extracorporeal membrane oxygen therapy (ECMO) were evaluated.

Microscopic minimally invasive approach to nonsyndromic craniosynostosis.

Purpose: The purpose of this retrospective study was to present the results of the authors' microscopic minimally invasive approach in the treatment of nonsyndromic craniosynostosis.

Methods: From 2001 to 2007, the authors treated a cohort of 67 infants with nonsyndromic sagittal, unicoronal, bicoronal, and metopic craniosynostosis, either with the microscopic (n = 40) or the open (n = 27) approach. In the microscopic approach, incisions were placed over the premature suture, and using a surgical microscope, the appropriate synostectomy was performed.