title: Prenatal diagnosis, prediction of outcome and in utero therapy of isolated congenital diaphragmatic hernia.
abstract: Congenital diaphragmatic hernia (CDH) can be associated with genetic or structural anomalies with poor prognosis. In isolated cases, survival is dependent on the degree of lung hypoplasia and liver position. Cases should be referred in utero to tertiary care centers familiar with this condition both for prediction of outcome as well as timed delivery. The best validated prognostic indicator is the lung area to head circumference ratio. Ultrasound is used to measure the lung area of the index case, which is then expressed as a proportion of what is expected normally (observed/expected LHR). When O/E LHR is < 25% survival chances are < 15%. Prenatal intervention, aiming to stimulate lung growth, can be achieved by temporary fetal endoscopic tracheal occlusion (FETO). A balloon is percutaneously inserted into the trachea at 26-28 weeks, and reversal of occlusion is planned at 34 weeks. Growing experience has demonstrated the feasibility and safety of the technique with a survival rate of about 50%. The lung response to, and outcome after FETO, is dependent on pre-existing lung size as well gestational age at birth. Early data show that FETO does not increase morbidity in survivors, when compared to historical controls. Several trials are currently under design.
authors.affiliation: Fetal Medicine Unit of University Hospitals Leuven, Leuven, Belgium.
authors.forename: Elisa
authors.initials: E
authors.lastname: Doné
authors.forename: Leonardo
authors.initials: L
authors.lastname: Gucciardo
authors.forename: Tim
authors.initials: T
authors.lastname: Van Mieghem
authors.forename: Jacques
authors.initials: J
authors.lastname: Jani
authors.forename: Mieke
authors.initials: M
authors.lastname: Cannie
authors.forename: Dominique
authors.initials: D
authors.lastname: Van Schoubroeck
authors.forename: Roland
authors.initials: R
authors.lastname: Devlieger
authors.forename: Luc De
authors.initials: LD
authors.lastname: Catte
authors.forename: Philipp
authors.initials: P
authors.lastname: Klaritsch
authors.forename: Steffi
authors.initials: S
authors.lastname: Mayer
authors.forename: Veronika
authors.initials: V
authors.lastname: Beck
authors.forename: Anne
authors.initials: A
authors.lastname: Debeer
authors.forename: Eduardo
authors.initials: E
authors.lastname: Gratacos
authors.forename: Kypros
authors.initials: K
authors.lastname: Nicolaides
authors.forename: Jan
authors.initials: J
authors.lastname: Deprest
mesh_terms: D005260:Female; D005325:Fetal Organ Maturity; D005332:Fetoscopy; D006548:Hernia, Diaphragmatic / Q000000981:diagnostic imaging / Q000601:surgery*; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D008168:Lung / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D011247:Pregnancy; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal