Abstract
Congenital diaphragmatic hernia is rare birth defect, which can be easily corrected after birth. The main problem is that herniation of viscera during fetal life impairs lung development, leading to a 30% mortality and significant morbidity. In isolated cases the outcome can be accurately predicted prenatally by medical imaging. Cases with a poor prognosis can be treated before birth; clinically this is by fetoscopic endoluminal tracheal occlusion. Obstruction of the airways triggers lung growth. This procedure is currently being evaluated in a global clinical trial for left sided cases; right sided cases with poor prognosis are offered the procedure clinically. The search for more potent and less invasive therapies continues. Prenatal transplacental sildenafil administration will in due course be tried clinically, with the aim to reduce the occurrence of persistent pulmonary hypertension, either alone or in combination with fetal surgery. Other medical approaches are in an earlier translational phase.
Original language | English |
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Pages (from-to) | 270-280 |
Number of pages | 11 |
Journal | Minerva Pediatrica |
Volume | 70 |
Issue number | 3 |
DOIs | |
Publication status | Published - Jun 2018 |
Externally published | Yes |
Bibliographical note
Funding sources:J.D.P. was a Clinical Researcher of the Flanders Research Foundation (FWO Vlaanderen; 1.8.01207) and
is partly funded by GOSH Children’s Charity. RK is Thorlakson Chair in Surgical Research and the
recipient of a Career Development Award from the Canadian Child Health Clinician Scientist Program
and a New Investigator Salary Award from the Canadian Institutes of Health Research/Manitoba Lung
Association/Children’s Hospital Research Institute of Manitoba. P.D.C. is an NIHR Professor and
supported by the Catapult Cell Therapy, the GOSH Children’s Charity, and GOSH NIHR Biomedical
Research Centre’s funding scheme. Our research into CDH is funded by the KU Leuven (IOF/C3),
GOSHCC, Sparks, CDHUK, Canadian Institutes of Health Research, Fetal Health Foundation (Brianna
Marie) and by an Innovative Engineering for Health award by the Wellcome Trust (WT101957), the
Engineering and Physical Sciences Research Council (NS/A000027/1). L.v.V., F.R., T.N., P.E. and D.B. are
supported by the Erasmus+ Program of the European Commission (2013-0040). This publication
reflects the views only of the author, and the Commission cannot be held responsible for any use which
may be made of the information contained therein. * PDK is currently working at the Erasmus Medical
Centre, Rotterdam, the Netherlands.