Abstract
BackgroundCongenital 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.MethodsFetal 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.ResultsFETO 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.ConclusionFor 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.
Highlights
Congenital diaphragmatic hernia (CDH) is a congenital anomaly with high mortality and morbidity mainly due to pulmonary hypoplasia and hypertension
There is one randomized controlled trial [25, 26] that showed benefit of Fetal endoluminal tracheal occlusion (FETO); in that study all deliveries were by Ex utero intrapartum treatment (EXIT) procedure, there was a case mix of right and left CDH, and the methodology was substantially different to what is currently being practiced in Europe and the USA
FETO seems to reduce early neonatal respiratory morbidity. This potential benefit is being investigated in two parallel randomized clinical trials (RCT) “Tracheal Occlusion To Accelerate Lung growth”, in fetuses with Left-sided congenital diaphragmatic hernia (LCDH) and either severe or moderate lung hypoplasia (NCT01240057 and NCT00763737)
Summary
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. Congenital diaphragmatic hernia (CDH) is a lifethreatening condition affecting up to 3 in 10,000 live born babies [1]. The diaphragmatic defect allows abdominal organs to herniate into the thorax which prevents normal lung development. Depending on the side and size of the defect, this may be the liver, bowel, spleen, and/or stomach. The majority of defects are left sided (LCDH 85%). Thirteen percent are right sided (RCDH), and bilateral defects or other forms occur very rarely. Associated anomalies are frequent and should be ruled out by imaging and genetic testing as they independently influence survival and morbidity. Surviving patients may suffer from medium and long-term morbidity predominantly respiratory in nature, and gastro-esophageal reflux, failure to thrive, and less common orthopedic or other problems
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