scholarly journals International recommendations on neonatal management with congenital diaphragmatic hernia: literature review

2021 ◽  
pp. 51-60
Author(s):  
А.G. Babintseva ◽  
◽  
Yu.D. Godovanets ◽  
Yu.Yu. Khodzinska ◽  
N.O. Popeliuk ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Large Scale ◽  
Level Of Evidence ◽  
Vasodilator Therapy ◽  
Membrane Oxygenation ◽  
Pulmonary Vasodilator ◽  
Extracorporal Membrane Oxygenation ◽  
Ultrasound Diagnostics

Congenital diaphragmatic hernia (CDH) is a defect of diaphragm development which is characterized by herniation of the abdominal content into the chest resulting in pulmonary hypoplasia and pulmonary hypertension of various degrees. Mortality and sickness rates among survived children with this pathology remain high all over the world. Prenatal diagnostics of high quality will help to find neonates with severe CDH and promote mobilization of human and material resources for optimal management at the tertiary level of giving medical aid. Attention of specialists in prenatal ultrasound diagnostics should be drawn to the necessity of measuring observed/expected lung(to(head ratio and location of the liver in fetus. Optimal treatment of neonates during preoperative period is focused on mild ventilation, avoidance of oxygen toxicity, baro/volutrauma of a hypoplastic lung and cardiac support. Administration of pulmonary vasodilator therapy including inhaled nitric oxide and extracorporal membrane oxygenation is pathogenetically substantiated for patients with pulmonary hypertension, and surgical correction should be delayed till stabilization of patient's condition. Echocardiography is a critically important diagnostic method. It enables to determine severity of pulmonary hypertension and myocardial functional disorders, to find targeted systemic methods of treatment that improve hemodynamic function or decrease pulmonary vascular resistance. Considering an insufficient level of evidence of the existing international recommendations, large(scale multi(center randomized studies on investigation of the best methods to prevent, diagnose and treat neonates with CDH are essential. No conflict of interest was declared by the authors. Key words: congenital diaphragmatic hernia; neonate; lung-to-head ratio; pulmonary hypertension; extracorporal membrane oxygenation.

CONTEMPORARY NEONATAL INTENSIVE CARE MANAGEMENT IN CONGENITAL DIAPHRAGMATIC HERNIA: DOES THIS OBVIATE THE NEED FOR FETAL THERAPY?

2009 ◽  
Vol 20 (4) ◽  
pp. 319-339 ◽  
Author(s):  
DESMOND BOHN
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Case Reports ◽  
Rescue Therapy ◽  
Intervention Strategy ◽  
Case Series ◽  
High Volume ◽  
Vasodilator Therapy ◽  
Operative Stabilization ◽  
Pulmonary Vasodilator

The management of congenital diaphragmatic hernia (CDH) in the newborn infant has changed radically since the first successful outcomes were reported 60 years ago. Then it seemed a surgical problem with a surgical solution – do an operation, remove the intestines and solid viscera from the thoracic cavity, repair the defect and allow the lung to expand. CDH in that era was regarded as the quintessential neonatal surgical emergency. The expectation was that urgent surgery would result in improvement in lung function and oxygenation. That approach persisted up to the 1980s when it was realized that the problem was far more complex and involved both an abnormal pulmonary vascular bed as well as pulmonary hypoplasia. The use of systemically delivered pulmonary vasodilator therapy, principally tolazoline, became a focus of interest in the 1980s with small case reports and case series suggesting improved survival. In the 1990s, based on studies that showed worsening thoracic compliance and gas exchange following surgical repair, deferred surgery and pre-operative stabilization became the standard of care. At the same time extracorporeal membrane oxygenation (ECMO) was increasingly used either as part of pre-operative stabilization or as a rescue therapy after repair. Other centres chose to use high frequency oscillatory ventilation (HFOV). Despite all these innovations the survival in live born infants with CDH did not improve to more than 50% in large series published from high volume centres. However, in the past 10 years there has been an appreciable improvement in survival to the extent that many centres are now reporting survival rates of greater than 80%. Probably the biggest impact on this improvement has been the recognition of the role that ventilation induced lung injury plays in mortality and the need for ECMO rescue. This has ushered in an era of a lung protective or “gentle ventilation” strategy which has been widely adopted as a standard approach. While there have been these radical changes in postnatal management attempts have been made to improve outcome with prenatal interventions, starting with prenatal repair, which was abandoned because of preterm labour. More recently there has been increasing experience in the use of balloon occlusion of the trachea as a prenatal intervention strategy with patients being selected based on prenatal predictors of poor outcome. This approach can only be justified if those predictors can be validated and the outcomes (death or serious long term morbidity) can be shown to be better than those currently achievable, namely 80% survival in high volume CDH centres rather than the 50–60% survival frequently quoted in historical papers.

scholarly journals Lung eicosanoids in perinatal rats with congenital diaphragmatic hernia

1997 ◽  
Vol 6 (1) ◽  
pp. 39-45 ◽  
Author(s):  
H. Ijsselstijn ◽  
F. J. Zijlstra ◽  
J. P. M. Van Dijk ◽  
J. C. De Jongste ◽  
D. Tibboel
Keyword(s):  
Pulmonary Hypertension ◽  
Caesarean Section ◽  
Bronchoalveolar Lavage ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Bronchoalveolar Lavage Fluid ◽  
Lavage Fluid ◽  
Prostaglandin E ◽  
Compensation Mechanism ◽  
Pulmonary Vasodilator

Abnormal levels of pulmonary eicosanoids have been reported in infants with persistent pulmonary hypertension (PPH) and congenital diaphragmatic hernia (CDH). We hypothesized that a dysbalance of vasoconstrictive and vasodilatory eicosanoids is involved in PPH in CDH patients. The levels of several eicosanoids in lung homogenates and in bronchoalveolar lavage fluid of controls and rats with CDH were measured after caesarean section or spontaneous birth. In controls the concentration of the stable metabolite of prostacyclin (6-keto-PGF1α), thromboxane A2(TxB2), prostaglandin E2(PGE2), and leukotriene B4(LTB4) decreased after spontaneous birth. CDH pups showed respiratory insufficiency directly after birth. Their lungs had higher levels of 6- keto-PGF1α, reflecting the pulmonary vasodilator prostacyclin (PGI2), than those of controls. We conclude that in CDH abnormal lung eicosanoid levels are present perinatally. The elevated levels of 6-keto-PGF1αin CDH may reflect a compensation mechanism for increased vascular resistance.

Combination of PGE1 and Pulmonary Vasodilator Therapy in Managing a Challenging Case of Severe PAH Secondary to CDH

2020 ◽  
Vol 11 (4) ◽  
pp. 525-527
Author(s):  
Othman A. Aljohani ◽  
Rohit Rao ◽  
Duncan Mackie ◽  
Shylah Haldeman ◽  
Tara Karamlou
Keyword(s):  
Right Ventricular ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Right Ventricular Dysfunction ◽  
Vasodilator Therapy ◽  
Venoarterial Extracorporeal Membrane Oxygenation ◽  
Pulmonary Vasodilator ◽  
Pulmonary Parenchyma ◽  
Pulmonary Arterial ◽  
Severe Pulmonary Arterial Hypertension

Congenital diaphragmatic hernia (CDH) is a rare disease, which affects 1 in 2,500 newborns. Congenital diaphragmatic hernia can interfere with the normal development of the pulmonary parenchyma and vascular bed, and in severe cases, it can lead to the development of severe pulmonary arterial hypertension (PAH) and right ventricular failure. We present a neonate with CDH who developed severe PAH and right ventricular dysfunction and was managed with a unique strategy combining venoarterial extracorporeal membrane oxygenation, prostaglandin E1, and a variety of PAH therapies.

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