TY - JOUR
T1 - Prenatal diagnosis and management of congenital diaphragmatic hernia
AU - Basurto, David
AU - Russo, Francesca Maria
AU - Van der Veeken, Lennart
AU - Van der Merwe, Johannes
AU - Hooper, Stuart
AU - Benachi, Alexandra
AU - De Bie, Felix
AU - Gomez, Olga
AU - Deprest, Jan
PY - 2019/7
Y1 - 2019/7
N2 - 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.
AB - 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.
KW - Congenital diaphragmatic hernia
KW - Prenatal prediction
KW - Prenatal therapy
KW - Pulmonary hypoplasia
KW - Sildenafil
UR - http://www.scopus.com/inward/record.url?scp=85061363452&partnerID=8YFLogxK
U2 - 10.1016/j.bpobgyn.2018.12.010
DO - 10.1016/j.bpobgyn.2018.12.010
M3 - Review Article
AN - SCOPUS:85061363452
SN - 1521-6934
VL - 58
SP - 93
EP - 106
JO - Best Practice and Research: Clinical Obstetrics and Gynaecology
JF - Best Practice and Research: Clinical Obstetrics and Gynaecology
ER -