Correction of congenital diaphragmatic hernia in utero IX: Fetuses with poor prognosis (Liver herniation and low lung-to-head ratio) can be saved by fetoscopic temporary tracheal occlusion

Harrison, Michael R.; Mychaliska, George B.; Albanese, Craig T.; Jennings, Russell W.; Farrell, Jody A.; Hawgood, Sam; Sandberg, Per; Levine, Anne H; Lobo, Errol; Filly, Roy A.

doi:10.1016/s0022-3468(98)90524-3

Cited by 285 publications

(182 citation statements)

References 13 publications

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“…Fetal surgery may salvage the most severely affected fetuses. 8 Many sonographic parameters have been proposed to predict the prognosis of CDH. Polyhydramnios in the third semester, 10 early diagnosis (less than 25 weeks' gestation), 4 presence of an intrathoracic stomach, 11 small lung-thorax transverse area ratio, 12 underdevelopment of the left heart, 13 and visceral herniation 14 have all been evaluated, but none of these has been widely accepted or applied clinically.…”

Section: Discussionmentioning

confidence: 99%

“…Those with an LHR of less than 1.4 have a 38% survival and may benefit from prenatal intervention. 8 It is unknown, however, whether the LHR is predictive of survival if the liver is not herniated into the hemithorax ("liver down"). Although difficult to prove, it has been believed that a low LHR indicates a large volume hernia with subsequent severe mediastinal shift.…”

mentioning

confidence: 99%

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Congenital diaphragmatic hernia without herniation of the liver: does the lung-to-head ratio predict survival?

Sbragia

Paek

Filly

et al. 2000

Journal of Ultrasound in Medicine

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The purpose of the present study was to determine the ability of lung‐to‐head ratio to predict survival and need for extracorporeal membrane oxygenation support in fetuses with left congenital diaphragmatic hernia without herniation of the liver into the chest. The perinatal records of 20 fetuses with isolated left congenital diaphragmatic hernia without herniation of the left lobe of the liver into the chest were reviewed. Fetuses were stratified into two groups depending on lung‐to‐head ratio: those with a ratio of less than 1.4 (historically a poor prognosis group) and those with a ratio of greater than 1.4. The outcome of both groups was compared with chi‐square analysis. Eight of 11 fetuses with a lung‐to‐head ratio greater than 1.4 survived, whereas 8 of 9 fetuses with a ratio of less than 1.4 survived. No differences were noted in the need for extracorporeal membrane oxygenation support or survival between the two groups. Fetuses with a prenatally diagnosed left congenital diaphragmatic hernia without herniation of liver into the chest have a favorable prognosis even in the presence of a low lung‐to‐head ratio.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Congenital diaphragmatic hernia without herniation of the liver: does the lung-to-head ratio predict survival?

Sbragia

Paek

Filly

et al. 2000

Journal of Ultrasound in Medicine

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“…This idea of blocking the airway emerged from an experiment of nature in patients with congenital high airway obstruction syndrome resulting in big polyalveolar lungs [50]. Previous research has shown that tracheal occlusion can indeed cause an increase in lung growth [51,52] and removing the balloon before birth has shown to be necessary for a better maturation of the lung by decreasing the apoptosis of the alveolar type 2 cells, which produce surfactant, an essential compound for lung function [53]. So far, FETO has been shown to improve survival rate in high-risk CDH patients, but at the cost of increased morbidity and premature delivery [54][55][56].…”

Section: Treatment Of Pulmonary Vascular Defects In Cdhmentioning

confidence: 99%

Pulmonary vascular development in congenital diaphragmatic hernia

et al. 2018

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Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly characterised by a diaphragmatic defect, persistent pulmonary hypertension (PH) and lung hypoplasia. The relative contribution of these three elements can vary considerably in individual patients. Most affected children suffer primarily from the associated PH, for which the therapeutic modalities are limited and frequently not evidence based. The vascular defects associated with PH, which is characterised by increased muscularisation of arterioles and capillaries, start to develop early in gestation. Pulmonary vascular development is integrated with the development of the airway epithelium. Although our knowledge is still incomplete, the processes involved in the growth and expansion of the vasculature are beginning to be unravelled. It is clear that early disturbances of this process lead to major pulmonary growth abnormalities, resulting in serious clinical challenges and in many cases death in the newborn. Here we provide an overview of the current molecular pathways involved in pulmonary vascular development. Moreover, we describe the abnormalities associated with CDH and the potential therapeutic approaches for this severe abnormality. Normal pulmonary vascular developmentHuman lung development can be divided into different stages based on histology, starting with the embryonic stage at 4 weeks of gestation, followed by the pseudoglandular stage in which branching of the lung buds continues. During the canalicular stage, starting at ∼16 weeks of gestation, the terminal bronchioli are formed. From 24 weeks of gestation until term, the saccular stage, airspaces widen and alveoli are formed. During the alveolar stage, which persists into the postnatal period up to 3 years of age, maturation of the airways occurs [1]. Concomitant with the expansion of the airways is the adaptation of the microvasculature to optimise the exchange of oxygen and carbon dioxide between the blood and the airways. We and others have shown that very early in lung development pulmonary vessels are present and connected to the systemic circulation. The vasculature develops in close relation with the airways and is a rate-limiting factor in branching morphogenesis [2][3][4]. This implies that the pulmonary vasculature plays an important role in lung development. The formation of new blood vessels primarily occurs through

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“…Postnatal treatment of infants with pulmonary hypoplasia, including extracorporeal membrane oxygenation, inhaled nitric oxide therapy, and ventilator strategies that minimize ventilator-induced lung injury, may often be of minimal benefit to infants with severe congenital DH due to underlying gross deficits in pulmonary tissue. Recently, there has been great interest in prenatal correction of lung hypoplasia in human fetuses predicted to have poor postnatal outcome by in utero tracheal occlusion (TO) (12,16). It is well established from animal studies that occluding the fetal trachea prevents the normal egress of liquid from the lung, leading to increased levels of lung tissue stretch and accelerated pulmonary growth.…”

mentioning

confidence: 99%

Temporary tracheal occlusion in fetal sheep with lung hypoplasia does not improve postnatal lung function

Davey

Hedrick

Bouchard

et al. 2003

Journal of Applied Physiology

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Correction of congenital diaphragmatic hernia in utero IX: Fetuses with poor prognosis (Liver herniation and low lung-to-head ratio) can be saved by fetoscopic temporary tracheal occlusion

Cited by 285 publications

References 13 publications

Congenital diaphragmatic hernia without herniation of the liver: does the lung-to-head ratio predict survival?

Congenital diaphragmatic hernia without herniation of the liver: does the lung-to-head ratio predict survival?

Pulmonary vascular development in congenital diaphragmatic hernia

Temporary tracheal occlusion in fetal sheep with lung hypoplasia does not improve postnatal lung function

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