Increased risk of clinically relevant neurodevelopmental disorders in survivors of congenital diaphragmatic hernia: a population-based study.

Purpose: Neurodevelopmental disorders (ND) have been recognized as the most common and potentially most disabling outcome of congenital diaphragmatic hernia (CDH). Our aim was to evaluate the incidence of ND in patients with a history of CDH referred for ND assessment on the basis of clinically relevant symptoms rather than through a screening process.

Methods: The study was a nationwide, population-based prospective nested case-control study within a cohort of newborn children who survived the newborn period in Sweden during the observational period from 1st January 1982 until 31st December 2015.

Identification of protein biomarkers associated with congenital diaphragmatic hernia in human amniotic fluid.

Congenital diaphragmatic hernia (CDH) is a severe birth defect frequently associated with pulmonary hypoplasia, pulmonary hypertension, and heart failure. Since amniotic fluid comprises proteins of both fetal and maternal origin, its analysis could provide insights on mechanisms underlying CDH and provide biomarkers for early diagnosis, severity of pulmonary changes and treatment response. The study objective was to identify proteomic changes in amniotic fluid consistently associated with CDH.

Syndromic congenital diaphragmatic hernia: Current incidence and outcome. Analysis from the congenital diaphragmatic hernia study group registry.

Background: The aim of this study was to describe the incidence of Congenital Diaphragmatic Hernia, CDH, associated with known or clinically suspected syndromes, and the postnatal outcomes from a large database for CDH.

Methods: Data from the multicenter, multinational database on infants with CDH (Congenital Diaphragmatic Hernia Study Group Registry) born from 1996 to 2020 were analyzed. Patients with known or suspected syndromes were grouped and outcome data were analyzed and compared to those without syndromic features.

Impact of Repeat Extracorporeal Life Support on Mortality and Short-term In-hospital Morbidities in Neonates With Congenital Diaphragmatic Hernia.

Objective: To evaluate the impact of repeat extracorporeal life support (ECLS) on survival and in-hospital outcomes in the congenital diaphragmatic hernia (CDH) neonates.

Background: Despite the widespread use of ECLS, investigations on multiple ECLS courses for CDH neonates are limited.

Methods: This is a retrospective cohort study of all ECLS-eligible CDH neonates enrolled in the Congenital Diaphragmatic Hernia Study Group registry between 1995 and 2019.

Hidden cardiovascular morbidity in children and young adults born with congenital diaphragmatic hernia: A population-based study.

Introduction: Congenital diaphragmatic (CDH) hernia is a rare congenital malformation with considerable mortality and morbidity in the neonatal period. The majority of the children today survive but little is known about long term cardiovascular morbidity.

Material And Methods: This was a nationwide population-based prospective case-control study within a cohort of Swedish children with CDH, born 1982-2015.

Extracorporeal Life Support Organization Guideline for Transport and Retrieval of Adult and Pediatric Patients with ECMO Support.

This guideline for the preparation for and undertaking of transport and retrieval of patients on extracorporeal membrane oxygenation (ECMO) is intended for educational use to build the knowledge of physicians and other health professionals in assessing the conditions and managing the treatment of patients undergoing ECLS / ECMO and describe what are believed to be useful and safe practice for extracorporeal life support (ECLS, ECMO) but these are not necessarily consensus recommendations. The aim of clinical guidelines are to help clinicians to make informed decisions about their patients. However, adherence to a guideline does not guarantee a successful outcome.

Premature and Extracorporeal Life Support: Is it Time? A Systematic Review.

Early preterm birth < 34 gestational weeks (GA) and birth weight (BW) <2 kg are relative contraindications for extracorporeal membrane oxygenation (ECMO). However, with improved technology, ECMO is presently managed more safely and with decreasing complications. Thus, these relative contraindications may no longer apply.

Re-ECMO for congenital diaphragmatic hernia: Is it worth the effort?

Aim: To evaluate the results in CDH patients subjected to a second course of ECMO at a single institution.

Material And Methods: Retrospective review of medical charts of patients treated for CDH and ECMO in our center since 1990 to December 2018 was performed. For patients subjected to a second course of ECMO and who survived to hospital discharge charts from follow up visits were also reviewed.

Optimizing Contrast-Enhanced Thoracoabdominal CT in Patients During Extracorporeal Membrane Oxygenation.

Rationale And Objectives: To evaluate the quality and value of contrast-enhanced (CE) chest- and abdominal computed tomography (CT) and CT angiography in neonates, children, and adults undergoing extracorporeal membrane oxygenation (ECMO) using a customized protocol for contrast delivery.

Materials And Methods: All patients admitted for CE thoracic- and/or abdominal CT while on ECMO were prospectively included in the study. A protocol for contrast delivery adapted for the type of ECMO circulation, cannulation sites, anatomy of interest, and desired contrast phase was applied.

Educational level and socioeconomic status in patients born with congenital diaphragmatic hernia: A population-based study.

Background: Neurodevelopmental dysfunction is one of the most disabling outcomes for congenital diaphragmatic hernia (CDH) survivors and may have a long lasting impact in adult life.

Aim: To evaluate to which extent being born with CDH has an impact on the educational level and socioeconomic status as a proxy for neurocognitive development.

Material And Methods: Nationwide, population-based prospective study of newborn children in Sweden from 1982 to 2015.

Congenital diaphragmatic hernia and associated omphalocele: a study from the CDHSG registry.

Background: Congenital Diaphragmatic Hernia (CDH) associated with Omphalocele is a rare condition, and only a few case reports are available in the literature. Both conditions are associated with some degree of pulmonary hypoplasia. We hypothesize that the combination of CDH with Omphalocele might be associated with poorer outcomes.

Introducing the Loop for Circuit Access during Extracorporeal Membrane Oxygenation: Feasibility and Safety.

In extracorporeal membrane oxygenation (ECMO), blood is drained from the patient, and pumped through a membrane oxygenator/lung (ML) for gas exchange and then back to the patient. For monitoring blood gases, samples may be sampled downstream from the ML. This exposes the patient for embolization risk (air/clot) when the stopcocks are flushed.

Ready for Transfer to Adult Care? A Triadic Evaluation of Transition Readiness in Adolescents With Congenital Heart Disease and Their Parents.

Transfer to adult care for adolescents with chronic conditions ought to be determined by transition readiness. The aims of this study were (a) to describe the level of readiness for transition in adolescents with congenital heart disease, (b) to compare adolescents' assessment of transition readiness with their parents' assessments, and (c) to study potential correlates of transition readiness. A total of 157 triads of adolescents aged 14 to 18 years and their parents completed the Readiness for Transition Questionnaire.

Maternal Risk Factors and Perinatal Characteristics in Congenital Diaphragmatic Hernia: A Nationwide Population-Based Study.

Background: The etiology of congenital diaphragmatic hernia (CDH) remains poorly understood. We hypothesize that environmental factors play an important role in the development of CDH.

Aim: The objective of this study was to investigate associated maternal risk factors in pregnancies with CDH.

Long-term pulmonary function and quality of life in adults after extracorporeal membrane oxygenation for respiratory failure.

Background: There is a significant long-term burden on survivors after acute respiratory distress syndrome, even 5 years after discharge. This is not well investigated in patients treated with extracorporeal membrane oxygenation. The objective of this study was to describe very-long-term (⩾3 years) disability in lung function and morphology, quality of life, mood disorders, walking capacity, and return to work status in extracorporeal membrane oxygenation survivors.

Transport on extracorporeal membrane oxygenation for congenital diaphragmatic hernia: A unique center experience.

Background: Support on Extracorporeal oxygenation membrane (ECMO) represents the last therapeutic option in the management of respiratory failure and pulmonary hypertension refractory to treatment in patients with congenital diaphragmatic hernia (CDH).

Aim: The objective of this work was to present our experience of all the cases of CDH that we have transported on ECMO.

Material And Methods: Medical records of patients, national and international, with CDH transported by our service on ECMO from 1997 to 2018 were reviewed.

Quality of life and functional outcome in Swedish children with low anorectal malformations: a follow-up study.

Purpose: The aim was to investigate the quality of life and bowel function in children with low anorectal malformations (ARM).

Additional Aim: To evaluate the Swedish version the Hirschsprung's Disease/Anorectal Malformation Quality of life Questionnaire (HAQL).

Methods: Forty-four children and their parents were invited to complete the HAQL and the Bowel Function Score (BFS).

A Single-Center Experience of 900 Interhospital Transports on Extracorporeal Membrane Oxygenation.

Background: The dawning of the extracorporeal membrane oxygenation (ECMO)-2 era, with the potential of decentralizing ECMO treatment, has stressed the need for research into the safety of ECMO transportations. The aim of this study was to (1) provide a comprehensive summary of transport arrangements and complications at a high-volume ECMO center, (2) determine predictors of severe complications occurring during transport, and (3) determine transport-related predictors of mortality.

Methods: This was a retrospective population-based observational cohort study of all interhospital ECMO transports performed by the Karolinska University Hospital between 1996 and 2017.

Parental uncertainty about transferring their adolescent with congenital heart disease to adult care.

Aims: To study parent's levels of uncertainty related to the transfer from pediatric to adult care in adolescents with congenital heart disease (CHD) and to identify potentially correlating factors.

Background: Parents acknowledge that during transition they struggle with finding ways of feeling secure in handing over the responsibility and letting go of control. Well-prepared and informed parents who feel secure are most likely better skilled to support their adolescent and to hand over the responsibility.

Impact of Low Anorectal Malformation on Parenting Stress: A Mixed-Method Study.

Unlabelled: The purpose of this study was to investigate parenting stress among parents of children with low ARM.

Study Aims: 1) Compare parenting stress among parents of children with low ARM, with parents of healthy children using questionnaires. 2) Identify subscales within the questionnaire which needed to be further explored.

Prenatally versus postnatally diagnosed congenital diaphragmatic hernia - Side, stage, and outcome.

Aim: To compare outcomes between prenatally and postnatally diagnosed CDH in a large multicenter database of prospectively collected data and evaluate factors associated with poorer outcome for prenatally diagnosed CDH.

Material And Methods: We used information from the multicenter, multinational CDH Study Group database on patients born between 2007 and 2015. We compared differences between prenatally and postnatally diagnosed CDH with respect to survival, side, size, ECMO needs, associated major cardiac malformations and liver position.

Behavioral, emotional and social functioning in children born with congenital diaphragmatic hernia.

Purpose: The aim was to investigate social competence and behavioral and emotional problems in children and adolescents born with CDH.

Methods: All children born with CDH, treated in Stockholm 1990-2009, were invited to participate. After written consent, the Child Behavior Checklist or Adult Self-Report questionnaires were sent to participants.

Health-related quality of life in children born with congenital diaphragmatic hernia.

Purpose: The aim of this study was to examine health-related quality of life (HRQoL) in children born with congenital diaphragmatic hernia (CDH).

Methods: Between 1993 and 2003, a total of 102 children born with CDH were treated at Astrid Lindgren Children's hospital in Stockholm. In 2012, long-term survivors (n = 77) were asked to participate in the present study, which resulted in a 46% (n = 35) response rate.