Treatment of Congenital Diaphragmatic Hernia
Congenital diaphragmatic hernia (CDH) can be associated with genetic or structural anomalies that render the prognosis overall poor. In isolated cases, survival is dependent on the degree of lung hypoplasia and potentially also liver position. Isolated CDH remains today, fatal in around 30% of prenatally diagnosed cases. Cases should be referred in utero to tertiary care centers familiar with this condition for comprehensive assessment, prediction of individual outcome as well as timed delivery. Individual prognosis can be estimated based on lung size measurement as well as documentation of the degree of liver herniation into the thorax. The use of imaging to document pulmonary vascularization is promising, but is at present still under investigation. Based on the predicted survival rate, parents can be counseled about their options. In those with a poor prognosis, termination of pregnancy can be considered. Antenatal intervention, aiming to stimulate lung growth, has become an alternative for these cases. Lung growth is triggered by temporary fetal endoscopic tracheal occlusion (FETO). Growing experience has demonstrated the feasibility and safety of the technique, and survival has increased from around 15% to over 50% in this group. In Europe a trial has now started, documenting the place of FETO in moderate cases (predicted survival rate 50%), and soon also in severe cases. It is hypothesized that prenatal intervention might decrease oxygen dependency in survivors (moderate), or increase survival (severe).
KeywordsCongenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia Pulmonary Hypoplasia High Frequency Oscillatory Ventilation Lung Growth
The European Commission supports our work in its sixth Framework (EuroSTEC; LSHC-CT-2006-037409). The Flemish Community of Belgium supports the clinical trial (IWT/070715). J.D. is the recipient of a “Fundamental Clinical Researcher” grant of the Fonds Wetenschappelijk Onderzoek-Vlaanderen (1.8.012.07.N.02). The collaborating centers from the antenatal CDH registry group and our colleagues from the FETO-task force or referring doctors are acknowledged for their contribution to the clinical program.
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