Journal
SEMINARS IN FETAL & NEONATAL MEDICINE
Volume 19, Issue 6, Pages 338-348Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.siny.2014.09.006
Keywords
Congenital diaphragmatic hernia; Pulmonary hypoplasia; Fetal surgery; Tracheal occlusion; Chromosomal microarrays; Exome sequencing
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Funding
- Fonds voor Wetenschappelijk Onderzoek Vlaanderen (FWD) [1.801207]
- European Commission [2013-0040]
- Eurostec FP-6 program (EuroSTEC) [LSHC-CT-2006-037409]
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Congenital diaphragmatic hernia (CDH) may be isolated or associated with other structural anomalies, the latter with poor prognosis. The defect allows viscera to herniate through the defect into the chest, competing for space with the developing lungs. At birth, pulmonary hypoplasia leads to respiratory insufficiency and persistent pulmonary hypertension that is lethal in up to 30% of patients. When isolated, survival chances can be predicted by antenatal measurement of lung size and liver herniation. Chromosomal microarrays and exome sequencing contribute to understanding genetic factors underlying isolated CDH. Prenatal intervention aims at stimulating lung development, clinically achieved by percutaneous fetal endoscopic tracheal occlusion (FETO) under local anesthesia. The Tracheal Occlusion To Accelerate Lung growth trial (www.totaltrial.eu) is an international randomized trial investigating the role of fetal therapy for severe and moderate pulmonary hypoplasia. Despite an apparent increase in survival following FETO, the search for lesser invasive and more potent prenatal interventions must continue. (C) 2014 Elsevier Ltd. All rights reserved.
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