Congenital Diaphragmatic Hernias

Protrusion of abdominal structures into the THORAX as a result of embryologic defects in the DIAPHRAGM often present in the neonatal period. It can be isolated, syndromic, non-syndromic or be a part of chromosome abnormality. Associated pulmonary hypoplasia and PULMONARY HYPERTENSION can further complicate stabilization and surgical intervention.

Also Known As:

Hernias, Diaphragmatic, Congenital; Agenesis of Hemidiaphragm; Bochdalek Hernias; Congenital Diaphragmatic Defect; Morgagni Hernias; Morgagni's Hernias; Unilateral Agenesis of Diaphragm; Congenital Diaphragmatic Defects; Defect, Congenital Diaphragmatic; Defects, Congenital Diaphragmatic; Diaphragm Unilateral Ageneses; Diaphragm Unilateral Agenesis; Diaphragmatic Defect, Congenital; Diaphragmatic Defects, Congenital; Diaphragmatic Hernia, Congenital; Diaphragmatic Hernias, Congenital; Hemidiaphragm Ageneses; Hemidiaphragm Agenesis; Hernia, Bochdalek; Hernia, Congenital Diaphragmatic; Hernia, Morgagni; Hernia, Morgagni's; Hernias, Bochdalek; Hernias, Congenital Diaphragmatic; Hernias, Morgagni; Hernias, Morgagni's; Morgagnis Hernia; Morgagnis Hernias; Bochdalek Hernia; Congenital Diaphragmatic Hernia; Morgagni Hernia; Morgagni's Hernia

Networked: 1524 relevant articles (53 outcomes, 163 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pulmonary Hypertension
2.	Respiratory Insufficiency (Respiratory Failure)
3.	Newborn Respiratory Distress Syndrome
4.	Meconium Aspiration Syndrome
5.	Congenital Heart Defects (Congenital Heart Defect)

Experts

1.	Puri, Prem: 85 articles (02/2021 - 05/2002)
2.	Tibboel, Dick: 43 articles (01/2022 - 03/2003)
3.	Friedmacher, Florian: 40 articles (02/2021 - 02/2012)
4.	Takahashi, Toshiaki: 28 articles (02/2021 - 08/2014)
5.	Schaible, Thomas: 26 articles (01/2022 - 11/2005)
6.	Deprest, Jan: 22 articles (09/2022 - 04/2006)
7.	Doi, Takashi: 22 articles (10/2017 - 10/2009)
8.	Zimmer, Julia: 21 articles (02/2021 - 02/2016)
9.	Harting, Matthew T: 19 articles (11/2022 - 12/2016)
10.	Congenital Diaphragmatic Hernia Study Group: 19 articles (09/2022 - 06/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Congenital Diaphragmatic Hernias:

1.	Nitric Oxide (Nitrogen Monoxide)FDA Link 09/01/2023 - "Correction To: Early nitric oxide is not associated with improved outcomes in congenital diaphragmatic hernia." 06/01/2023 - "Early nitric oxide is not associated with improved outcomes in congenital diaphragmatic hernia." 12/01/2014 - "Initial oxygenation response to inhaled nitric oxide predicts improved outcome in congenital diaphragmatic hernia." 03/01/2000 - "The cost-effectiveness analysis is based on outcome and utilization data from two multicentered randomized clinical trials conducted by the Canadian Inhaled Nitric Oxide Study group, one for patients with congenital diaphragmatic hernia (CDH) and one for patients without CDH. " 06/01/2023 - "Inhaled nitric oxide (iNO) is widely used for the management of infants with congenital diaphragmatic hernia (CDH); however, evidence of benefit is limited. "
2.	nitrofenIBA 09/01/2014 - "The aim of this study was to evaluate the efficacy of mesenchymal stem cells (MSCs) in a nitrofen-induced congenital diaphragmatic hernia (CDH) rat model. " 01/01/2019 - "The mouse nitrofen/bisdiamine model is a potential model allowing the study of congenital diaphragmatic hernia lung development from early stages using a wide array of methods. " 01/01/2019 - "To study pulmonary hypoplasia (PH) associated with congenital diaphragmatic hernia (CDH), investigators have been employing a fetal rat model based on nitrofen administration to dams. " 08/01/2017 - "Fetal Cardiac Impairment in Nitrofen-Induced Congenital Diaphragmatic Hernia: Postmortem Microcomputed Tomography Imaging Study." 02/01/2017 - "The aim of this study was to investigate the pulmonary vascular expression of NFATc2 and KV1.5 in rats with nitrofen-induced congenital diaphragmatic hernia (CDH). "
3.	Surface-Active Agents (Surfactants)IBA 01/01/2004 - "Animal models of congenital diaphragmatic hernia (CDH) have hypoplastic lungs with evidence of decreased lamellar bodies in their type II pneumocytes and resultant surfactant deficiency, and respond to surfactant replacement with improved gas exchange and lung mechanics. " 02/01/1994 - "The aim of this study was to assess the impact of surfactant deficiency on the pathophysiology of congenital diaphragmatic hernia (CDH). " 02/01/2009 - "We hypothesize that surfactant deficiency is involved in CDH and depressed activity of exocytosis and endocytosis in AEC II is responsible for the surfactant deficiency in the lung of newborn with congenital diaphragmatic hernia." 02/01/2009 - "Depressed exocytosis and endocytosis of type II alveolar epithelial cells are responsible for the surfactant deficiency in the lung of newborn with congenital diaphragmatic hernia." 07/31/2007 - "Surfactant levels in congenital diaphragmatic hernia."
4.	Sildenafil Citrate (Viagra)FDA Link 01/01/2023 - "PBPK-based dose finding for sildenafil in pregnant women for antenatal treatment of congenital diaphragmatic hernia." 01/01/2022 - "To evaluate the effect of combining antenatal sildenafil with fetal tracheal occlusion (TO) in fetal rabbits with surgically induced congenital diaphragmatic hernia (CDH). " 01/01/2022 - "Complementary Effect of Maternal Sildenafil and Fetal Tracheal Occlusion Improves Lung Development in the Rabbit Model of Congenital Diaphragmatic Hernia." 01/01/2021 - "Use of sildenafil in late postoperative period of congenital diaphragmatic hernia." 01/01/2021 - "Here, we present a young infant with congenital diaphragmatic hernia and PH who received an unintentional 10-fold overdose of oral sildenafil for 6 consecutive days. "
5.	tetrandrineIBA 05/01/2011 - "[Protective effects of tetrandrine against congenital diaphragmatic hernia in rats]." 05/01/2011 - "To study the protective effect of the prenatal use of tetrandrine (TET) against congenital diaphragmatic hernia (CDH) in rats and possible mechanisms. " 05/01/2009 - "Prenatal tetrandrine treatment can reverse the abnormal conditions in the lung of newborn with congenital diaphragmatic hernia." 08/28/2007 - "To assess the efficacy of prenatal administration of tetrandrine (TET) on pulmonary hypoplasia in the nitrofen-induced congenital diaphragmatic hernia (CDH) fetal rat model. " 03/01/2016 - "To investigate the effects of Tetrandrine (TET) prenatal intervention on the differentiation of alveolar epithelial cells type I (AEC I) in rat model of Nitrofen-induced congenital diaphragmatic hernia (CDH). "
6.	FluorocarbonsIBA 03/01/2003 - "A prospective, randomized pilot trial of perfluorocarbon-induced lung growth in newborns with congenital diaphragmatic hernia." 08/01/2001 - "Continuous intrapulmonary distension with perfluorocarbon accelerates lung growth in infants with congenital diaphragmatic hernia: initial experience." 03/01/2003 - "Initial laboratory and clinical data suggest that partial liquid ventilation (PLV) can enhance pulmonary function and that lung growth can be induced via distension of the newborn lung using perfluorocarbon in patients with congenital diaphragmatic hernia (CDH). " 05/01/1996 - "The authors evaluated the safety and efficacy of liquid ventilation with perfluorocarbon in four newborns with congenital diaphragmatic hernia and severe respiratory failure, who were on extracorporeal life support (ECLS). " 11/01/1995 - "To determine the efficacy of perfluorocarbon-associated gas exchange and the effects of inhaled nitric oxide during perfluorocarbon-associated gas exchange in the congenital diaphragmatic hernia lamb model. "
7.	perflubron (L 1913)FDA Link 07/01/2015 - "Safety and efficacy of perflubron-induced lung growth in neonates with congenital diaphragmatic hernia: Results of a prospective randomized trial." 07/01/2015 - "We examined the safety and efficacy of perflubron-induced lung growth (PILG) in neonates with congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO). " 05/01/1996 - "The authors conclude that perflubron can be safely administered into the lungs of newborn patients with congenital diaphragmatic hernia and severe respiratory failure, and it may be associated with improvement in gas exchange and pulmonary compliance." 06/01/2016 - "Effect of Perflubron-induced lung growth on pulmonary vascular remodeling in congenital diaphragmatic hernia." 07/01/1996 - "To determine the serial radiographic appearance of the lungs of neonates who underwent partial liquid ventilation with perflubron because of congenital diaphragmatic hernia (CDH) or primary pulmonary hypertension. "
8.	Tolazoline (Priscoline)FDA Link 12/01/1980 - "Although tolazoline resulted in a significant improvement in the paO2 in 4 infants with congenital diaphragmatic hernia, it did not appear to improve mortality in these infants." 05/01/1981 - "Tolazoline in the treatment of congenital diaphragmatic hernias." 09/01/1985 - "A neonate, managed with tolazoline for pulmonary hypertension after repair of a congenital diaphragmatic hernia, developed a duodenal perforation. " 12/01/1980 - "There were 47 seriously-ill neonates with medical causes of respiratory distress and 10 infants with severe respiratory distress secondary to a congenital diaphragmatic hernia treated with tolazoline according to a strict protocol designed to manage persistent fetal circulation (PFC). " 12/01/1980 - "Tolazoline therapy for persistent pulmonary hypertension after congenital diaphragmatic hernia repair."
9.	GlucocorticoidsIBA 05/01/2002 - "In experimentally produced congenital diaphragmatic hernia (CDH), antenatal glucocorticoids have been shown to improve morphologic and biochemical lung immaturity and normalize the thickened pulmonary vascular wall. " 02/01/1997 - "Prenatal glucocorticoids reverse pulmonary immaturity in rodents with pharmacologically induced congenital diaphragmatic hernia (CDH). " 02/01/1997 - "Prenatal glucocorticoids improve pulmonary morphometrics in fetal sheep with congenital diaphragmatic hernia." 05/01/1994 - "Antenatal glucocorticoid corrects pulmonary immaturity in experimentally induced congenital diaphragmatic hernia in rats." 01/01/2000 - "Antenatal glucocorticoid therapy (AGT) has been demonstrated to improve pulmonary immaturity and abnormal structure of pulmonary arteries in animal models of congenital diaphragmatic hernia (CDH). "
10.	Oxygen (Dioxygen)IBA 01/01/2021 - "This trial involving fetuses with moderate congenital diaphragmatic hernia on the left side did not show a significant benefit of FETO performed at 30 to 32 weeks of gestation over expectant care with respect to survival to discharge or the need for oxygen supplementation at 6 months. " 01/01/2018 - "To evaluate whether infants with congenital diaphragmatic hernia (CDH) can be safely resuscitated with a reduced starting fraction of inspired oxygen (FiO2) of 0.5. " 10/01/2014 - "Assessment of pulmonary vascular reactivity to oxygen using fractional moving blood volume in fetuses with normal lung development and pulmonary hypoplasia in congenital diaphragmatic hernia." 07/01/1998 - "The authors hypothesized that in utero tracheal occlusion would reverse the high impedance to pulmonary blood flow associated with congenital diaphragmatic hernia (CDH) and normalize the fetal physiological response to oxygen at term. " 11/01/1992 - "One boy with congenital diaphragmatic hernia who was intubated for 19 days after birth aged 4 months on the day of operation and weighed 5.3 kg. None of the children was oxygen-dependent on the day of surgery. "

Therapies and Procedures

1.	Extracorporeal Membrane Oxygenation 03/01/1995 - "This report suggests that stabilization of the intrauterine to extrauterine transitional circulation combined with a respiratory care strategy that avoids pulmonary overdistension, takes advantage of inherent biological cardiorespiratory mechanics, and very delayed surgery for congenital diaphragmatic hernia results in improved survival and decreases the need for extracorporeal membrane oxygenation (ECMO). " 02/01/1991 - "The purpose of this study was to evaluate the efficacy of extracorporeal membrane oxygenation (ECMO) for patients with congenital diaphragmatic hernia (CDH). " 04/01/2016 - "Delayed repair of congenital diaphragmatic hernia (CDH) for days or longer has become standard, allowing improved stabilization for many, but potentially complicating treatment in severely affected infants who require extracorporeal membrane oxygenation (ECMO) and arrive unrepaired. " 04/01/2016 - "Improved Survival in Left Liver-Up Congenital Diaphragmatic Hernia by Early Repair Before Extracorporeal Membrane Oxygenation: Optimization of Patient Selection by Multivariate Risk Modeling." 08/01/1997 - "Improved results in patients who have congenital diaphragmatic hernia using preoperative stabilization, extracorporeal membrane oxygenation, and delayed surgery."
2.	Therapeutics 08/01/2014 - "Congenital diaphragmatic hernia (CDH) remains a significant cause of death in newborns and, despite improved outcomes with multimodality therapies, optimal timing of repair remains undefined. " 04/01/2008 - "However, for the neonates with HRF caused by congenital diaphragmatic hernia, iNO therapy did not result in a significant reduction in the oxygenation index and death rate. " 12/01/1990 - "Our review demonstrates that ECMO improved survival significantly in infants with congenital diaphragmatic hernia who had a "poor prognosis" by the criteria of Bohn et al. We recommend consideration of ECMO for all infants with congenital diaphragmatic hernia for whom maximal medical therapy has failed." 01/01/2022 - "Previous studies have shown that ex utero intrapartum therapy (EXIT) is safe and feasible for newborns with congenital diaphragmatic hernia (CDH). " 09/01/2015 - "The aim of this study is to characterize medical and surgical therapies and short-term outcomes in infants with congenital diaphragmatic hernia (CDH). "
3.	Artificial Respiration (Mechanical Ventilation) 05/01/2016 - "Conventional Mechanical Ventilation Versus High-frequency Oscillatory Ventilation for Congenital Diaphragmatic Hernia: A Randomized Clinical Trial (The VICI-trial)." 11/02/2011 - "The VICI-trial: high frequency oscillation versus conventional mechanical ventilation in newborns with congenital diaphragmatic hernia: an international multicentre randomized controlled trial." 01/01/2006 - "The PURPOSE of the present study was to establish the treatment results of the applied approach for preoperative stabilization through mechanical ventilation and delayed surgical intervention as opposed to emergency surgical treatment in children with congenital diaphragmatic hernia. " 01/01/2001 - "The purpose of this prospective consecutive patient study was to test if perioperative respiratory system compliance of patients undergoing surgical repair of congenital diaphragmatic hernia (CDH) can predict the need for prolonged postoperative mechanical ventilation. " 03/01/1993 - "Previous studies from our institution have shown that neonates with congenital diaphragmatic hernia (CDH), whose best postductal PaO2 (BPDPO2) was less than 100 mm Hg while on maximal conventional mechanical ventilation (CMV), had a mortality exceeding 90%. "
4.	Ligation 01/01/1979 - "It is suggested that ligation of the patient ductus arteriosus and administration of pulmonary vasodilatators are both effective in improving oxygenation, in patients who may die an anoxic death after repair of a severe congenital diaphragmatic hernia." 07/01/1998 - "We have learned previously that in utero tracheal ligation reverses the structural and physiological effects of surgically created congenital diaphragmatic hernia. " 02/01/1998 - "We have previously demonstrated that experimental fetal tracheal ligation reverses the structural and physiological effects of pulmonary hypoplasia associated with congenital diaphragmatic hernia. " 03/01/1997 - "Currently there are two in utero procedures that have been proposed for the treatment of Congenital diaphragmatic hernia (CDH); reduction of the herniated viscera with repair of the diaphragmatic defect (CDH repair) and stimulation of lung growth by ligation of the fetal trachea (CDH + TL). " 03/01/1997 - "Tracheal ligation: the dark side of in utero congenital diaphragmatic hernia treatment."
5.	Minimally Invasive Surgical Procedures 04/01/2016 - "To compare the safety and efficacy of minimally invasive surgery (MIS) with traditional open surgical approach for congenital diaphragmatic hernia (CDH). " 08/01/2013 - "The aim of the study was to determine clinical indications for performing minimally invasive surgery (MIS) with acceptable results by reviewing our experience in congenital diaphragmatic hernia (CDH) repair and comparing outcomes of MIS with open surgery. " 04/01/2012 - "The aim of this study was to evaluate outcome of patients with congenital diaphragmatic hernia (CDH) undergoing open versus minimally invasive surgery. " 02/01/2023 - "A Systematic Review Comparing the Surgical Outcomes of Open Versus Minimally Invasive Surgery for Congenital Diaphragmatic Hernia Repair." 12/01/2022 - "Background: Minimally invasive surgery (MIS) is increasingly used for repair of congenital diaphragmatic hernia (CDH). "