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.

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.

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.

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.

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.

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).

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Toward Standardized Management of Congenital Diaphragmatic Hernia: An Analysis of Practice Guidelines.

Background: Standardized care may improve outcomes in many diseases including congenital diaphragmatic hernia (CDH). Our study assesses the variability of CDH clinical practice guidelines (CPG) among North American centers.

Methods: North American member institutions of the CDH Study Group and the Pediatric Surgical Research Collaborative were solicited to submit their CDH CPG.

The management of pilonidal disease: A systematic review.

Objective: The goal of this systematic review by the American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee was to derive recommendations from the medical literature regarding the management of pilonidal disease.

Methods: The PubMed, Cochrane, Embase, Web of Science, and Scopus databases from 1965 through June 2017 were queried for any papers addressing operative or non-operative management of pilonidal disease. The Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines were followed.

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.