Fetal diaphragmatic hernia without visceral herniation
Mark D. StringerRuth B. GoldsteinRoy A. FillyLori J. HowellAugusto SolaN. Scott AdzickMichael R. Harrison
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As one kind of severe congenital malformation, congenital diaphragmatic hernia (CDH) initiates a series of pathological changes including diaphragmatic defect, pulmonary hypoplasia and pulmonary arterial hypertension. Among these, pulmonary hypoplasia is the dominant factor impacting its prognosis. Currently, postnatal surgical repair of diaphragm, ventilation and medical treatment are major therapeutic options for CDH in China. With the conceptual advances in treatment and techniques, the survival rate continues rising. However, postnatal surgical repair cannot change the pathological basis of pulmonary hypoplasia. Severe CDH children are prone to persistent pulmonary hypertension and postnatal respiratory failure so that the mortality of severe CDH stays at a high level. Therefore prenatal interventions for improving pulmonary development and achieving a higher survival have come into focus these years. At present, fetal endoscopic tracheal occlusion (FETO) is the most widely recognized intervention during fetal period. It has been applied to clinical practice in European countries, United States and some other countries. In recent decades, clinical researches and animal model experiments suggested that FETO might enable an accumulation of bronchial fluid and force lung to expand so as to improve the development of lung and boost survival rate in severe CDH cases. On the contrary, FETO increased the risk of premature rupture of membrane (PROM) and premature delivery and caused a higher mortality. Thus whether or not FETO is beneficial has remained controversial. Our research focused on research advances of FETO and evaluation of its prognosis in CDH cases.
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Fetoscopy; Congenital diaphragmatic hernia; Pulmonaryhypoplasia; Prognosis
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In previous papers, we described a new experimental model of congenital diaphragmatic hernia in rabbits, and we also reported noninvasive therapeutic strategies for prevention of the functional and structural immaturity of the lungs associated with this defect. In addition to lung hypoplasia, pulmonary hypertension, biochemical, and structural immaturity of the lungs, the hemodynamics of infants and animals with congenital diaphragmatic hernia are markedly altered. Hence, cardiac hypoplasia has been implicated as a possible cause of death in patients with congenital diaphragmatic hernia, and it is hypothesized to be a probable consequence of fetal mediastinal compression by the herniated viscera. Cardiac hypoplasia has also been reported in lamb and rat models of congenital diaphragmatic hernia. The purpose of the present experiment was to verify the occurrence of heart hypoplasia in our new model of surgically produced congenital diaphragmatic hernia in fetal rabbits.Twelve pregnant New Zealand rabbits underwent surgery on gestational day 24 or 25 (normal full gestational time - 31 to 32 days) to create left-sided diaphragmatic hernias in 1 or 2 fetuses per each doe. On gestational day 30, all does again underwent surgery, and the delivered fetuses were weighed and divided into 2 groups: control (non-surgically treated fetuses) (n = 12) and congenital diaphragmatic hernia (n = 9). The hearts were collected, weighed, and submitted for histologic and histomorphometric studies.During necropsy, it was noted that in all congenital diaphragmatic hernia fetuses, the left lobe of the liver herniated throughout the surgically created defect and occupied the left side of the thorax, with the deviation of the heart to the right side, compressing the left lung; consequently, this lung was smaller than the right one. The body weights of the animals were not altered by congenital diaphragmatic hernia, but heart weights were decreased in comparison to control fetuses. The histomorphometric analysis demonstrated that congenital diaphragmatic hernia promoted a significant decrease in the ventricular wall thickness and an increase in the interventricular septum thickness.Heart hypoplasia occurs in a rabbit experimental model of congenital diaphragmatic hernia. This model may be utilized for investigations in therapeutic strategies that aim towards the prevention or the treatment of heart hypoplasia caused by congenital diaphragmatic hernia.
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Congenital diaphragmatic hernia (CDH) is a congenital anomaly with high mortality and morbidity mainly due to pulmonary hypoplasia and hypertension. Temporary fetal tracheal occlusion to promote prenatal lung growth may improve survival. Entrapment of lung fluid stretches the airways, leading to lung growth.Fetal endoluminal tracheal occlusion (FETO) is performed by percutaneous sono-endoscopic insertion of a balloon developed for interventional radiology. Reversal of the occlusion to induce lung maturation can be performed by fetoscopy, transabdominal puncture, tracheoscopy, or by postnatal removal if all else fails.FETO and balloon removal have been shown safe in experienced hands. This paper deals with the technical aspects of balloon insertion and removal. While FETO is invasive, it has minimal maternal risks yet can cause preterm birth potentially offsetting its beneficial effects.For left-sided severe and moderate CDH, the procedure is considered investigational and is currently being evaluated in a global randomized clinical trial (https://www.totaltrial.eu/). The procedure can be clinically offered to fetuses with severe right-sided CDH.
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Congenital diaphragmatic hernia (CDH) is a complex disease associated with pulmonary hypoplasia and pulmonary hypertension. Great strides have been made in our ability to care for CDH patients, specifically in the prenatal improvement of lung volume and morphology with fetoscopic endoluminal tracheal occlusion (FETO). While the anatomic effects of FETO have been described in-depth, the changes it induces at the cellular and molecular level remain a budding area of CDH research. This review will delve into the cellular and molecular effects of FETO in the developing lung, emphasize areas in which further research may improve our understanding of CDH, and highlight opportunities to optimize the FETO procedure for improved postnatal outcomes.
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In the present study, we review the indications, technical aspects, preliminary results, risks, and clinical implications of fetal endoscopic tracheal occlusion (FETO) for severe congenital diaphragmatic hernia (CDH) performed outside the United States and its potential future directions in this country and globally. Congenital diaphragmatic hernia occurs in approximately 1 in 2500 live births and results in high neonatal morbidity and mortality, largely associated with the severity of pulmonary hypoplasia and pulmonary arterial hypertension. With the advent of prenatal imaging, CDH can be diagnosed before birth, and in utero treatment is now available in some centers. The prognosis of CDH can be evaluated by assessing the fetal lung size, the degree of liver herniation, and the fetal pulmonary vasculature in isolated forms of CDH. These parameters help classify fetuses as having mild, moderate, severe, or extremely severe isolated CDH. Severe and extremely severe diaphragmatic hernias have poor outcomes and thus are candidates for innovative therapies such as FETO. Fetal endoscopic tracheal occlusion is usually performed between 26 and 30 weeks' gestation. In utero, an endoscope is passed through the fetal mouth and down to the carina; the balloon is deployed just above the carina. After the procedure, ultrasound surveillance every 2 weeks ensures the balloon's structural integrity and measures the fetal pulmonary response. At approximately 34 weeks' gestation, the balloon is deflated and removed. Fetal endoscopic tracheal occlusion is thought to improve outcomes by decreasing mortality and allowing more rapid neonatal stabilization. Ultimately, the goal of FETO is to minimize pulmonary hypoplasia and pulmonary arterial hypertension. Following delivery, neonates still require diaphragm repair. Target Audience Obstetricians and gynecologists, family physicians Learning Objectives After completing this CME activity, physicians should be better able to evaluate the different imaging modalities available for determining prognosis in fetuses with congenital diaphragmatic hernias, identify the indications for fetal endoscopic tracheal occlusion, and compare the potential benefits and complications of fetal endoscopic tracheal occlusion.
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