Current and Emerging Treatment for Congenital Diaphragmatic Hernia

2001 ◽  
Vol 20 (2) ◽  
pp. 5-15 ◽  
Author(s):  
Jeanne Braby
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Treatment Modalities ◽  
Exogenous Surfactant ◽  
High Frequency Ventilation ◽  
Liquid Ventilation ◽  
The Past ◽  
Antenatal Steroids ◽  
Frequency Ventilation ◽  
Tracheal Ligation

Management of congenital diaphragmatic hernia has changed dramatically over the past couple of decades. Until the early 1980s, it was felt that the abdominal contents should be returned to the abdomen as soon as possible to allow the lungs to expand. It is now known that it is not the defect that causes respiratory distress, but the infant’s hypoplastic lungs and accompanying pulmonary hypertension. Advances in treatment and technology have contributed to changes in management. Ultrasonography now allows for early prenatal detection. Prenatal treatment modalities include in utero tracheal ligation and maternal antenatal steroids. Postnatal modalities have expanded to include permissive hypercapnia, high-frequency ventilation, inhaled nitric oxide, pharmacologic support, exogenous surfactant, and extracorporeal membrane oxygenation. Liquid ventilation and lobar lung transplantation have also been tried. In spite of these advances, the overall survival rate remains about 63 percent.

Congenital Diaphragmatic Hernia: Two Case Studies with Atypical Presentations

2006 ◽  
Vol 25 (4) ◽  
pp. 239-249 ◽  
Author(s):  
Barbara Puckett
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
High Frequency ◽  
Pulmonary Hypoplasia ◽  
High Frequency Ventilation ◽  
Ultrasound Screening ◽  
Severe Respiratory Distress ◽  
The Family ◽  
Frequency Ventilation ◽  
Atypical Presentations

Congenital diaphragmatic hernia (CDH) affects 1 in every 2,000 to 4,000 live births. Many infants with this condition are diagnosed antenatally through routine ultrasound screening. Nearly 90 percent present at delivery with severe respiratory distress requiring intubation. Many of these infants develop persistent pulmonary hypertension of the newborn due to hypoplasia of the affected lung. The survival of infants with CDH is limited by the degree of pulmonary hypoplasia and requires sophisticated medical technology such as high-frequency ventilation and inhaled nitric oxide. Some infants also require treatment with extracoporeal membrane oxygenation. This article gives details of two cases of CDH in which the presentation was atypical. The more subtle presentation is discussed, as well as the embryology and pathophysiology of CDH and the possibility of associated anomalies. Clinical management and impact on the family are outlined.

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.

scholarly journals Prenatal tracheal ligation or intra-amniotic administration of surfactant or dexamethasone prevents some structural changes in the pulmonary arteries of surgically created diaphragmatic hernia in rabbits

2002 ◽  
Vol 57 (1) ◽  
pp. 1-8 ◽  
Author(s):  
Consuelo J. Rodrigues ◽  
Uenis Tannuri ◽  
Ana Cristina A. Tannuri ◽  
João Maksoud-Filho ◽  
Aldo J. Rodrigues Junior
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Structural Changes ◽  
Elastic Fiber ◽  
Pulmonary Arteries ◽  
External Diameter ◽  
Cell Nuclei ◽  
Fiber Density ◽  
Positive Effects ◽  
Tracheal Ligation

PURPOSE: Characterization of the structural changes occurring in the pulmonary arteries resulting from surgically produced congenital diaphragmatic hernia in rabbits, with particular emphasis on the preventive effects of prenatal tracheal ligation or administration of intra-amniotic dexamethasone or surfactant. METHODS: Twenty rabbit fetuses underwent surgical creation of a left-sided congenital diaphragmatic hernia on the 24th or 25th gestational day. They were divided according to the following procedures: congenital diaphragmatic hernia (n = 5), congenital diaphragmatic hernia plus tracheal ligation (n = 5), congenital diaphragmatic hernia plus intra-amniotic administration of dexamethasone 0.4 mg (n = 5) or surfactant (Curosurf 40 mg, n = 5). On gestational day 30, all the fetuses were delivered by caesarean section and killed. A control group consisted of five nonoperated fetuses. Histomorphometric analysis of medial thickness, cell nuclei density, and elastic fiber density of pulmonary arterial walls was performed. RESULTS: Arteries with an external diameter > 100 mum have a decreased medial thickness, lower cell nuclei density, and greater elastic fiber density when compared with arteries with external diameter <= 100 mum. Congenital diaphragmatic hernia promoted a significant decrease in medial thickness and an increase in cell nuclei density in artery walls with external diameter > 100 mum. Prenatal treatments with tracheal ligation or intra-amniotic administration of dexamethasone or surfactant prevented these changes. In arteries with external diameter <= 100 mum, congenital diaphragmatic hernia promoted a significant increase in medial thickness and in cell nuclei density and a decrease in elastic fiber density. The prenatal treatments with tracheal ligation or intra-amniotic administration of dexamethasone or surfactant prevented these changes, although no effect was observed in elastic fiber density in the congenital diaphragmatic hernia plus dexamethasone group. CONCLUSIONS: Congenital diaphragmatic hernia promoted different structural changes for large or small arteries. The prenatal intra-amniotic administration of dexamethasone or surfactant had positive effects on the lung structural changes promoted by congenital diaphragmatic hernia, and these effects were comparable to the changes induced by tracheal ligation.

Tracheal ligation does not correct the surfactant deficiency associated with congenital diaphragmatic hernia

1996 ◽  
Vol 31 (4) ◽  
pp. 546-550 ◽  
Author(s):  
Stuart J. O'Toole ◽  
Anjmun Sharma ◽  
Hratch L. Karamanoukian ◽  
Bruce Holm ◽  
Richard G. Azizkhan ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Surfactant Deficiency ◽  
Tracheal Ligation
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