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Ultrasound in Obstetrics & Gynecology :... Sep 2023To develop a core set of prenatal and neonatal outcomes for clinical studies evaluating perinatal interventions for congenital diaphragmatic hernia, using a validated... (Review)
Review
OBJECTIVE
To develop a core set of prenatal and neonatal outcomes for clinical studies evaluating perinatal interventions for congenital diaphragmatic hernia, using a validated consensus-building method.
METHODS
An international steering group comprising 13 leading maternal-fetal medicine specialists, neonatologists, pediatric surgeons, patient representatives, researchers and methodologists guided the development of this core outcome set. Potential outcomes were collected through a systematic review of the literature and entered into a two-round online Delphi survey. A call was made for stakeholders with experience of congenital diaphragmatic hernia to review the list and score outcomes based on their perceived relevance. Outcomes that fulfilled the consensus criteria defined a priori were discussed subsequently in online breakout meetings. Results were reviewed in a consensus meeting, during which the core outcome set was defined. Finally, the definitions, measurement methods and aspirational outcomes were defined in online and in-person definition meetings by a selection of 45 stakeholders.
RESULTS
Overall, 221 stakeholders participated in the Delphi survey and 198 completed both rounds. Fifty outcomes met the consensus criteria and were discussed and rescored by 78 stakeholders in the breakout meetings. During the consensus meeting, 93 stakeholders agreed eventually on eight outcomes, which constituted the core outcome set. Maternal and obstetric outcomes included maternal morbidity related to the intervention and gestational age at delivery. Fetal outcomes included intrauterine demise, interval between intervention and delivery and change in lung size in utero around the time of the intervention. Neonatal outcomes included neonatal mortality, pulmonary hypertension and use of extracorporeal membrane oxygenation. Definitions and measurement methods were formulated by 45 stakeholders, who also added three aspirational outcomes: duration of invasive ventilation, duration of oxygen supplementation and use of pulmonary vasodilators at discharge.
CONCLUSIONS
We developed with relevant stakeholders a core outcome set for studies evaluating perinatal interventions in congenital diaphragmatic hernia. Its implementation should facilitate the comparison and combination of trial results, enabling future research to better guide clinical practice. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Pregnancy; Female; Infant, Newborn; Child; Humans; Hernias, Diaphragmatic, Congenital; Research Design; Prenatal Care; Obstetrics; Outcome Assessment, Health Care; Delphi Technique; Treatment Outcome
PubMed: 37099763
DOI: 10.1002/uog.26235 -
European Journal of Pediatrics Feb 2021Early reports have suggested survival benefits associated with a hernia sac in congenital diaphragmatic hernia (CDH). However, these studies have included only small... (Review)
Review
Early reports have suggested survival benefits associated with a hernia sac in congenital diaphragmatic hernia (CDH). However, these studies have included only small subsets of patients. This systematic review aimed to evaluate differences in outcomes of CDH newborns with and without a hernia sac. PubMed and Embase databases were searched using relevant key terms. Papers were independently reviewed by two authors with final selection approved by the senior author. Original search retrieved 537 papers; the final review included 8 studies (n = 837 patients). There were 168 CDH patients (20%) with a hernia sac with an overall survival of 93% vs 73% for CDH newborns without a sac (p < 0.001). Twenty-three percent of patients with a CDH sac required diaphragm patch repair vs 44% patients without a sac (p < 0.001). Pulmonary hypertension was manifested in 44% of CDH babies with a hernia sac vs 64% without a sac (p < 0.001). Three studies compared ECMO requirement: 15% with a hernia sac and 34% without sac, p < 0.001.Conclusion: This study shows significant survival benefits in newborns associated with presence of a CDH sac. This may be likely related to these infants having more favourable physiology with less severe pulmonary hypertension and/or smaller anatomical defects requiring primary closure only. What is Known: • Early reports have suggested survival benefits associated with a hernia sac in CDH. • Previous studies have included only a small number of patients. What is New: • A systematic review of published studies clearly shows that CDH newborns with a hernia sac have better overall survival outcomes and less severe pulmonary hypertension. • ECMO utilization and patch repair were also less often required in newborns with a hernia sac.
Topics: Hernias, Diaphragmatic, Congenital; Humans; Infant, Newborn; Retrospective Studies
PubMed: 32808134
DOI: 10.1007/s00431-020-03779-1 -
The Journal of Pediatrics Sep 2022
Topics: Genetic Testing; Hernias, Diaphragmatic, Congenital; Humans
PubMed: 35667445
DOI: 10.1016/j.jpeds.2022.05.059 -
Prenatal Diagnosis Mar 2022To define the dimensions of patient-centeredness of prenatal care for parents who had a fetus with isolated congenital diaphragmatic hernia (CDH) by exploring their...
OBJECTIVE
To define the dimensions of patient-centeredness of prenatal care for parents who had a fetus with isolated congenital diaphragmatic hernia (CDH) by exploring their values, experiences, needs and preferences.
METHODS
In-depth interviews with parents who were faced with the prenatal diagnosis of isolated CDH.
RESULTS
Interviews (n = 18) identified seven dimensions of patient-centeredness. Parental preferences are respected by unconditional acceptance of parental choices and needs. After diagnosis access to care should occur by swift referral to a specialised centre to prevent parents receiving contradictory information. Information and education help parents to gain some sense of control and cope with the many uncertainties. A multidisciplinary team with a coordinating professional is perceived supportive (coordination and integration), and seamless referral between local and treatment centres reduces parental distress (continuity and transition). Family-centred emotional support helps parents to cope with emotions and distress. Informal support by involvement of family and friends and trusted peer-support is of added value.
CONCLUSION
The impact of the uncertainty of outcome in this condition is tremendous and needs to be considered when providing prenatal care. After initial diagnosis, one should avoid speculation about the severity of the condition and outcome, and provide swift referral to a specialist centre expert in managing CDH. Provision of realistic and comprehensive information helps parents cope. Multidisciplinary and continuous support throughout the full trajectory, but also integrated psychosocial support should become standard-of-care.
Topics: Counseling; Educational Status; Female; Hernias, Diaphragmatic, Congenital; Humans; Parents; Pregnancy; Prenatal Diagnosis
PubMed: 35184301
DOI: 10.1002/pd.6118 -
Kyobu Geka. the Japanese Journal of... Sep 2022Bochdalek hernia is the most common type of congenital diaphragmatic hernia( CDH). Patients with CDH often develop cardiopulmonary dysfunction secondary to pulmonary...
Bochdalek hernia is the most common type of congenital diaphragmatic hernia( CDH). Patients with CDH often develop cardiopulmonary dysfunction secondary to pulmonary hypoplasia and pulmonary hypertension, which necessitates emergency surgery. Advanced cardiopulmonary management is essential in such cases;therefore, patients should be treated at a well-facility that is familiar with the treatment of CDH. Respiratory management using gentle ventilation and control of pulmonary hypertension using nitric oxide are necessary for preoperative stabilization of patients. Intraoperatively, the hernia should be cautiously reduced and contents should be returned into the abdominal cavity. Surgery is performed to secure the circumference of the rim and ensure firm closure of the defect. Thoracoscopic surgery may be considered for late-onset cases with a stable general condition.
Topics: Hernias, Diaphragmatic, Congenital; Humans; Hypertension, Pulmonary; Lung; Nitric Oxide; Thoracoscopy
PubMed: 36155584
DOI: No ID Found -
The Pan African Medical Journal 2023
Topics: Adult; Humans; Hernias, Diaphragmatic, Congenital
PubMed: 37013215
DOI: 10.11604/pamj.2023.44.26.38571 -
BJOG : An International Journal of... Oct 2023To describe the outcomes of preterm born infants with congenital diaphragmatic hernia (CDH; ≤32.0 weeks of gestation) and the associations between prenatal imaging...
OBJECTIVES
To describe the outcomes of preterm born infants with congenital diaphragmatic hernia (CDH; ≤32.0 weeks of gestation) and the associations between prenatal imaging markers and survival.
DESIGN
Retrospective cohort study.
SETTING
Multicentre study in large referral centres.
POPULATION
Infants with an isolated unilateral CDH, live born at 32.0 weeks or less of gestation, between January 2009 and January 2020.
METHODS
Neonatal outcomes were evaluated for infants that were expectantly managed during pregnancy and infants that underwent fetoscopic endoluminal tracheal occlusion (FETO) therapy, separately. We evaluated the association between prenatal imaging markers and survival to discharge. Prenatal imaging markers included observed to expected lung-to-head ratio (o/e LHR), side of the defect, liver position, stomach position grade, and observed to expected total fetal lung volume (o/e TFLV).
MAIN OUTCOME MEASURE
Survival to discharge.
RESULTS
We included 53 infants born at 30 (interquartile range 29 -31 ) weeks. Survival in fetuses expectantly managed during pregnancy was 48% (13/27) in left-sided CDH and 33% (2/6) in right-sided CDH. Survival in fetuses that underwent FETO therapy was 50% (6/12) in left-sided CDH and 25% (2/8) in right-sided CDH. The o/e LHR at baseline was positively associated with survival in cases expectantly managed during pregnancy (odds ratio [OR] 1.20, 95% CI 1.07-1.42, p < 0.01), but not in cases that received FETO therapy (OR 1.01, 95% CI 0.88-1.15, p = 0.87). Stomach position grade (p = 0.03) and o/e TFLV were associated with survival (p = 0.02); liver position was not (p = 0.13).
CONCLUSIONS
In infants with CDH born at or before 32 weeks of gestation, prenatal imaging markers of disease severity were associated with postnatal survival.
Topics: Female; Humans; Infant, Newborn; Pregnancy; Hernias, Diaphragmatic, Congenital; Infant, Premature; Retrospective Studies; Ultrasonography, Prenatal; Survival Analysis; Gestational Age; Treatment Outcome; Male
PubMed: 37069727
DOI: 10.1111/1471-0528.17497 -
Scientific Reports Jun 2023Congenital diaphragmatic hernia (CDH) is a neonatal anomaly that includes pulmonary hypoplasia and hypertension. We hypothesized that microvascular endothelial cell (EC)...
Congenital diaphragmatic hernia (CDH) is a neonatal anomaly that includes pulmonary hypoplasia and hypertension. We hypothesized that microvascular endothelial cell (EC) heterogeneity is different in CDH lungs and related to lung underdevelopment and remodeling. To test this, we evaluated rat fetuses at E21.5 in a nitrofen model of CDH to compare lung transcriptomes among healthy controls (2HC), nitrofen-exposed controls (NC) and nitrofen-exposed subjects with CDH. Single-cell RNA sequencing with unbiased clustering revealed 3 distinct microvascular EC clusters: a general population (mvEC), a proliferative population and a population high in hemoglobin. Only the CDH mvEC cluster had a distinct inflammatory transcriptomic signature as compared to the 2HC and NC endothelial cells, e.g. greater activation and adhesion of inflammatory cells and production of reactive oxygen species. Furthermore, CDH mvECs had downregulated Ca4, Apln and Ednrb gene expression. Those genes are markers for ECs important to lung development, gas exchange and alveolar repair (mvCa4+). mvCa4+ ECs were reduced in CDH (2HC [22.6%], NC [13.1%] and CDH [5.3%], p < 0.0001). Overall, these findings identify transcriptionally distinct microvascular endothelial cell clusters in CDH, including the distinctly inflammatory mvEC cluster and the depleted group of mvCa4+ ECs, which together may contribute to pathogenesis.
Topics: Humans; Rats; Animals; Hernias, Diaphragmatic, Congenital; Endothelial Cells; Transcriptome; Rats, Sprague-Dawley; Lung; Disease Models, Animal
PubMed: 37330615
DOI: 10.1038/s41598-023-37050-y -
Clinics in Perinatology Mar 2024This review provides a comprehensive summary of the current understanding of pulmonary hypertension (PH) in congenital diaphragmatic hernia, outlining the underlying... (Review)
Review
This review provides a comprehensive summary of the current understanding of pulmonary hypertension (PH) in congenital diaphragmatic hernia, outlining the underlying pathophysiologic mechanisms, methods for assessing PH severity, optimal management strategies, and prognostic implications.
Topics: Humans; Hernias, Diaphragmatic, Congenital; Hypertension, Pulmonary; Lung; Prognosis; Severity of Illness Index
PubMed: 38325939
DOI: 10.1016/j.clp.2023.10.001 -
Thoracic Surgery Clinics May 2024The diaphragm is a critical musculotendinous structure that contributes to respiratory function. Disorders of the diaphragm are rare and diagnostically challenging.... (Review)
Review
The diaphragm is a critical musculotendinous structure that contributes to respiratory function. Disorders of the diaphragm are rare and diagnostically challenging. Herein, the author reviews the radiologic options for the assessment of the diaphragm.
Topics: Humans; Diaphragm; Magnetic Resonance Imaging; Tomography, X-Ray Computed; Hernia, Diaphragmatic
PubMed: 38705659
DOI: 10.1016/j.thorsurg.2024.02.002