scholarly journals Respiratory Support of Infants With Congenital Diaphragmatic Hernia

2021 ◽  
Vol 9 ◽  
Author(s):  
Emma Williams ◽  
Anne Greenough
Keyword(s):  
Clinical Trials ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Lung Damage ◽  
Respiratory Support ◽  
Abnormal Development ◽  
Pulmonary Vasculature ◽  
Lung Protective Ventilation ◽  
Lung Hypoplasia ◽  
Oxygen Control

Optimisation of respiratory support of infants with congenital diaphragmatic hernia (CDH) is critical. Infants with CDH often have severe lung hypoplasia and abnormal development of their pulmonary vasculature, leading to ventilation perfusion mismatch. It is vital that lung protective ventilation strategies are employed during both initial stabilisation and post-surgical repair to avoid ventilator induced lung damage and oxygen toxicity to prevent further impairment to an already diminished gas-exchanging environment. There is a lack of robust evidence for the routine use of surfactant therapy during initial resuscitation of infants with CDH and thus administration cannot be recommended outside clinical trials. Additionally, inhaled nitric oxide has been shown to have no benefit in reducing the mortality rates of infants with CDH. Other therapeutic agents which beneficially act on pulmonary hypertension are currently being assessed in infants with CDH in randomised multicentre trials. The role of novel ventilatory modalities such as closed loop automated oxygen control, liquid ventilation and heliox therapy may offer promise for infants with CDH, but the benefits need to be determined in appropriately designed clinical trials.

Anesthesia for Congenital Diaphragmatic Hernia Repair in Infants

2021 ◽  
pp. 221-228
Author(s):  
Chirag Shah ◽  
Anna Clebone ◽  
Caitlin Aveyard
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Abnormal Development ◽  
Pulmonary Vasculature ◽  
Lung Hypoplasia ◽  
Developmental Defect ◽  
Thoracic Cavity ◽  
Bochdalek Hernia ◽  
Congenital Condition ◽  
Diaphragmatic Hernia Repair

A congenital diaphragmatic hernia is a congenital condition in which a developmental defect in the diaphragm allows abdominal contents to migrate into the thoracic cavity. It occurs in about 1 in 2,000 newborns. This leads to lung underdevelopment and lung hypoplasia in utero. A defect in the diaphragm is usually on the left side, most commonly in the posterolateral region, also known as a Bochdalek hernia. A defect in the anterior region is known as a Morgagni hernia. Lung hypoplasia and the abnormal development of pulmonary vasculature resulting in pulmonary hypertension contribute to the morbidity and mortality associated with a congenital diaphragmatic hernia.

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

CONGENITAL DIAPHRAGMATIC HERNIA AND HYPOPLASTIC LEFT HEART

PEDIATRICS ◽  
1967 ◽  
Vol 40 (3) ◽  
pp. 329-333
Author(s):  
Frederick N. Firestone ◽  
Robert W. Popper ◽  
Hooshang Taybi ◽  
Richard Leonards
Keyword(s):  
Clinical Trials ◽  
Atrial Septal Defect ◽  
Congenital Diaphragmatic Hernia ◽  
Causal Relationship ◽  
Diaphragmatic Hernia ◽  
Septal Defect ◽  
Left Heart ◽  
Lung Growth ◽  
Hypoplastic Left Heart ◽  
Subsequent Change

An infant with the rare combination of congenital diaphragmatic hernia and hypoplastic left heart has been reported. Although herniated viscera in the chest impair bronchial division and cause hypoplastic lungs, this patient demonstrated lung growth and histologic maturation after successful correction of the diaphragmatic hernia. No subsequent change of the hypoplastic left heart toward normal occurred. There is no apparent causal relationship between these two defects. Creation of an atrial septal defect has been useful in the treatment of hypoplastic left heart. Further clinical trials are encouraged.

Abnormalities of the Fetal Chest

2019 ◽  
Author(s):  
Karen M. Davidson
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Airway Obstruction ◽  
Diaphragmatic Hernia ◽  
Pulmonary Hypoplasia ◽  
Abnormal Development ◽  
Neurenteric Cyst ◽  
Congenital Pulmonary Airway Malformation ◽  
Bronchopulmonary Sequestration ◽  
Lung Agenesis ◽  
Pulmonary Airway

The normal and abnormal development of the organs lying within the fetal thorax is discussed.  The abnormalities reviewed include more common findings of pulmonary hypoplasia, congenital diaphragmatic hernia, congenital pulmonary airway malformation, bronchopulmonary sequestration, as well as the rarer conditions of congenital fetal hydrothorax, congenital high airway obstruction syndrome, bronchogenic cysts, neurenteric cysts, and lung agenesis.  With each abnormality, the clinical implications for the fetus, best methods for prenatal diagnosis, as well as possible additional anomalies, syndromes, and aneuploidies are described.  In utero and postnatal treatments are also reviewed.   This review contains 10 figures, and 37 references. Key Words: Pulmonary hypoplasia, lung-head ratio, congenital diaphragmatic hernia, congenital pulmonary airway malformation, congenital fetal hydrothorax, bronchopulmonary sequestration, congenital high airway obstruction syndrome, bronchogenic cyst, neurenteric cyst, lung agenesis

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