scholarly journals Knowledge Gaps in the Fetal to Neonatal Transition of Infants With a Congenital Diaphragmatic Hernia

2021 ◽  
Vol 9 ◽  
Author(s):  
Philip L. J. DeKoninck ◽  
Emily J. J. Horn-Oudshoorn ◽  
Ronny Knol ◽  
Kelly J. Crossley ◽  
Irwin K. M. Reiss
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Transition Period ◽  
Adverse Outcomes ◽  
Lung Hypoplasia ◽  
Future Research ◽  
Knowledge Gaps ◽  
Physiological Adaptations ◽  
Interesting Approach ◽  
Neonatal Transition

Clinical research for infants born with a congenital diaphragmatic hernia (CDH) has until recently mainly focused on advances in prenatal and postnatal treatment. However, during the early perinatal transition period there are major physiological adaptations. For most infants these changes will happen uneventfully, but for CDH infants this marks the beginning of serious respiratory complications. In recent years, there is emerging evidence that the clinical management during the perinatal stabilization period in the delivery room may influence postnatal outcomes. Herein, we discuss major knowledge gaps and novel concepts that aim to optimize fetal to neonatal transition for infants with CDH. One such novel and interesting approach is performing resuscitation with an intact umbilical cord, the efficacy of this procedure is currently being investigated in several clinical trials. Furthermore, close evaluation of neonatal physiological parameters in the first 24 h of life might provide early clues concerning the severity of lung hypoplasia and the risk of adverse outcomes. We will provide an overview of trending concepts and discuss potential areas for future research.

scholarly journals Extracellular Vesicles and Their miRNA Content in Amniotic and Tracheal Fluids of Fetuses with Severe Congenital Diaphragmatic Hernia Undergoing Fetal Intervention

Cells ◽  
2021 ◽  
Vol 10 (6) ◽  
pp. 1493
Author(s):  
Isabella Fabietti ◽  
Tiago Nardi ◽  
Chiara Favero ◽  
Laura Dioni ◽  
Laura Cantone ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Extracellular Vesicles ◽  
Diaphragmatic Hernia ◽  
Target Genes ◽  
In Utero ◽  
Lung Hypoplasia ◽  
Future Research ◽  
Tracheal Occlusion ◽  
Postnatal Mortality ◽  
Prenatal Intervention

Infants with congenital diaphragmatic hernia (CDH) are at high risk of postnatal mortality due to lung hypoplasia and arterial pulmonary hypertension. In severe cases, prenatal intervention by fetal endoscopic tracheal occlusion (FETO) can improve survival by accelerating lung growth. However, postnatal mortality remains in the range of about 50% despite fetal treatment, and there is currently no clear explanation for this different clinical response to FETO. We evaluated the concentration of extracellular vesicles (EVs) and associated microRNA expression in amniotic and tracheal fluids of fetuses with CDH undergoing FETO, and we examined the association between molecular findings and postnatal survival. We observed a higher count of EVs in the amniotic fluid of non-survivors and in the tracheal fluid sampled in utero at the time of reversal of tracheal occlusion, suggesting a pro-inflammatory lung reactivity that is already established in utero and that could be associated with a worse postnatal clinical course. In addition, we observed differential regulation of four EV-enclosed miRNAs (miR-379-5p, miR-889-3p; miR-223-3p; miR-503-5p) in relation to postnatal survival, with target genes possibly involved in altered lung development. Future research should investigate molecular therapeutic agents targeting differentially regulated miRNAs to normalize their expression and potentially improve clinical outcomes.

Possibility of application of prenatal ultrasound indexes in congenital diaphragmatic hernia in fetus to determine postnatal outcomes

2021 ◽  
Author(s):  
N.V. Mashinets
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Pleural Cavity ◽  
Prenatal Ultrasound ◽  
Lung Hypoplasia ◽  
Prenatal Period ◽  
Compression Index ◽  
Disease Group ◽  
Group I ◽  
Group Ii

Objectives. To assess the effectiveness of the use of prenatal ultrasound indexes in congenital diaphragmatic hernia of the fetus to determine the postnatal prognosis. Materials. The analysis of 95 observations of left-sided congenital diaphragmatic hernia of the fetus was carried out. In the prenatal period, the composition of organs displaced into the pleural cavity was determined, the heart compression index (HCI), O/E LHR according to Jani and DeKoninck, and QLI were calculated. Results. Survival rate of newborns was 57.9%, mortality rate was 42.1%. The newborns were divided into two groups depending on the outcome of the disease. Group I — surviving newborns (n = 55), group II — deceased patients (n = 40). In the analyzed groups, there were no statistical differences in the timing of delivery, birth weight of newborns, the severity of asphyxia after birth and the type of hernia. In group I, the intestinal loops and stomach were significantly more often identified in the pleural cavity in isolation, less often the liver. HCI corresponded to 1.3, Jani O/E LHR 45.7%, DeKoninck O/E LHR 38.7%, QLI 0.7. In group II, concomitant malformations, polyhydramnios and displacement of the liver into the pleural cavity were significantly more frequent. HCI was 1.5, Jani O/E LHR 38.6%, DeKoninck O/E LHR 32.0%, QLI 0.6. Conclusions. In predicting the outcome of the disease for a newborn, the most effective is a comprehensive assessment of the location of the liver, the heart compression index and the index of lung hypoplasia (O/E LHR according to Jani). The diagnostic accuracy of the method is 80%, the sensitivity is 74.4%, and the specificity is 83.3%.

scholarly journals Prenatal Diagnosis and Management of a Rare Central Tendon Defect Type of Congenital Diaphragmatic Hernia with a Massive Pericardial Effusion

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Inas Babic ◽  
Haifa Al-Jobair ◽  
Osama Al Towaijri ◽  
Huda Al-Shammary ◽  
Merna Atiyah ◽  
...  
Keyword(s):  
Pericardial Effusion ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Neonatal Morbidity ◽  
Lung Hypoplasia ◽  
Defect Type ◽  
Tendon Defect ◽  
Prenatal Diagnostic ◽  
Large Pericardial Effusion ◽  
Prenatal Intervention

The central tendon defect type of congenital diaphragmatic hernia (CDH) is extremely rare and usually associated with a significant pericardial effusion. Prenatal diagnostic ultrasound features of this quite rare entity remain often overlooked or misdiagnosed. There is a dearth of literature about the role of prenatal intervention, often through an elective pericardiocentesis, for the prevention of lung hypoplasia and to decrease the overall neonatal morbidity and mortality. To the best of our knowledge, till date, there is only one case that was subjected to a prenatal intervention. Here, we present a second case of a central tendon defect type of CDH with a large pericardial effusion that was subjected to a prenatal transthoracic pericardiocentesis. Although smooth intubation and ventilation were performed immediately after birth, the infant suffered for several months from respiratory instability. Laparoscopic central tendon hernia repair was performed, and neonate was discharged home at seven months of age. Although prenatal pericardiocentesis may facilitate smoother postnatal intubation and ventilation, its broader effect on respiratory function is uncertain and still remains elusive.

scholarly journals Cellular Origin(s) of Congenital Diaphragmatic Hernia

2021 ◽  
Vol 9 ◽  
Author(s):  
Gabriëla G. Edel ◽  
Gerben Schaaf ◽  
Rene M. H. Wijnen ◽  
Dick Tibboel ◽  
Gabrielle Kardon ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Birth Defect ◽  
Congenital Disorder ◽  
Diaphragmatic Defect ◽  
Lung Hypoplasia ◽  
Cellular Origin ◽  
Recent Developments ◽  
Mechanical Factors

Congenital diaphragmatic hernia (CDH) is a structural birth defect characterized by a diaphragmatic defect, lung hypoplasia and structural vascular defects. In spite of recent developments, the pathogenesis of CDH is still poorly understood. CDH is a complex congenital disorder with multifactorial etiology consisting of genetic, cellular and mechanical factors. This review explores the cellular origin of CDH pathogenesis in the diaphragm and lungs and describes recent developments in basic and translational CDH research.

Tetralogy of Fallot with congenital diaphragmatic hernia and left lung hypoplasia: assessment of the adequacy of the peripheral pulmonary vascular growth after hernia repair

2011 ◽  
Vol 22 (2) ◽  
pp. 235-238 ◽  
Author(s):  
Hong Ju Shin ◽  
Won Kyoung Jhang ◽  
Tae Jin Yun
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Tetralogy Of Fallot ◽  
Diaphragmatic Hernia ◽  
Left Lung ◽  
Lung Hypoplasia ◽  
Vascular Growth ◽  
Threatening Condition ◽  
Central Pulmonary Artery ◽  
Life Threatening ◽  
Vascular Beds

AbstractCongenital diaphragmatic hernia is a life-threatening condition frequently associated with various congenital cardiac diseases. In congenital diaphragmatic hernia associated with tetralogy of Fallot, central pulmonary artery size of the affected side may not reflect the capacitance of peripheral pulmonary vascular beds. We report a case of congenital diaphragmatic hernia associated with tetralogy of Fallot, which was repaired after assessing the adequacy of the pulmonary vascular beds by intra-operative pulmonary blood flow study.

Lipocalin 2 as a new biomarker for fetal lung hypoplasia in congenital diaphragmatic hernia

2016 ◽  
Vol 462 ◽  
pp. 71-76 ◽  
Author(s):  
Hiroyuki Tsuda ◽  
Tomomi Kotani ◽  
Tomoko Nakano ◽  
Kenji Imai ◽  
Shima Hirako ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Fetal Lung ◽  
Lung Hypoplasia ◽  
Lipocalin 2

scholarly journals Perinatal stabilisation of infants born with congenital diaphragmatic hernia: a review of current concepts

2020 ◽  
Vol 105 (4) ◽  
pp. 449-454
Author(s):  
Emily J J Horn-Oudshoorn ◽  
Ronny Knol ◽  
Arjan B Te Pas ◽  
Stuart B Hooper ◽  
Suzan C M Cochius-den Otter ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Management Strategies ◽  
Fetal Surgery ◽  
Adverse Outcomes ◽  
Delivery Room ◽  
Treatment Modalities ◽  
Mortality And Morbidity ◽  
Early Predictors ◽  
Stabilisation Period

Congenital diaphragmatic hernia (CDH) is associated with high mortality rates and significant pulmonary morbidity, mainly due to disrupted lung development related to herniation of abdominal organs into the chest. Pulmonary hypertension is a major contributor to both mortality and morbidity, however, treatment modalities are limited. Novel prenatal and postnatal interventions, such as fetal surgery and medical treatments, are currently under investigation. Until now, the perinatal stabilisation period immediately after birth has been relatively overlooked, although optimising support in these early stages may be vital in improving outcomes. Moreover, physiological parameters obtained from the perinatal stabilisation period could serve as early predictors of adverse outcomes, thereby facilitating both prevention and early treatment of these conditions. In this review, we focus on the perinatal stabilisation period by discussing the current delivery room guidelines in infants born with CDH, the physiological changes occurring during the fetal-to-neonatal transition in CDH, novel delivery room strategies and early predictors of adverse outcomes. The combination of improvements in the perinatal stabilisation period and early prediction of adverse outcomes may mitigate the need for specific postnatal management strategies.

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