Format

Send to

Choose Destination
Best Pract Res Clin Obstet Gynaecol. 2019 Jan 5. pii: S1521-6934(18)30263-3. doi: 10.1016/j.bpobgyn.2018.12.010. [Epub ahead of print]

Prenatal diagnosis and management of congenital diaphragmatic hernia.

Author information

1
Academic Department of Development and Regeneration, Cluster Woman and Child, Biomedical Sciences, KU Leuven, Belgium.
2
Academic Department of Development and Regeneration, Cluster Woman and Child, Biomedical Sciences, KU Leuven, Belgium; Clinical Department of Obstetrics & Gynaecology, KU Leuven, Leuven, Belgium.
3
The Ritchie Centre, Hudson Institute of Medical Research and Department of Obstetrics and Gynaecology, Monash University, Melbourne, Victoria, Australia.
4
Obstetrics and Gynaecology Department, Centre de Référence Maladie Rare: Hernie de Coupole Diaphragmatique, Hôpital Antoine Béclère, Université Paris Sud, AP-HP, Clamart, France; European Reference Network on Rare and Inherited Congenital Anomalies "ERNICA".
5
BCNatal, Barcelona Center for Maternal-Fetal and Neonatal Medicine (Hospital Clínic and Hospital Sant Joan de Deu), Fetal I+D Fetal Medicine Research Center, Institut Clinic de Ginecologia, Obstetricia I Neonatologia, IDIBAPS, CIBER-ER, University of Barcelona, Spain.
6
Academic Department of Development and Regeneration, Cluster Woman and Child, Biomedical Sciences, KU Leuven, Belgium; Clinical Department of Obstetrics & Gynaecology, KU Leuven, Leuven, Belgium; Institute for Women's Health, University College London, London, UK; European Reference Network on Rare and Inherited Congenital Anomalies "ERNICA". Electronic address: jan.deprest@uzleuven.be.

Abstract

Congenital diaphragmatic hernia is characterized by failed closure of the diaphragm, thereby allowing abdominal viscera to herniate into the thoracic cavity and subsequently interfering with normal lung development. At birth, pulmonary hypoplasia leads to respiratory insufficiency and persistent pulmonary hypertension (PHT), that is lethal in up to 32% of patients. In isolated cases, the outcome may be predicted prenatally by medical imaging and advanced genetic testing. In those fetuses with a predicted poor outcome, fetoscopic endoluminal tracheal occlusion may be offered. This procedure is currently being evaluated in a global randomized clinical trial (www.TOTALtrial.eu). We are currently investigating alternative strategies including transplacental sildenafil administration to reduce the occurrence of persistent PHT.

KEYWORDS:

Congenital diaphragmatic hernia; Prenatal prediction; Prenatal therapy; Pulmonary hypoplasia; Sildenafil

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center