REVIEW  NEW TRENDS IN PEDIATRIC SURGERY 

Minerva Pediatrica 2018 June;70(3):270-80

DOI: 10.23736/S0026-4946.18.05186-1

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: English

Antenatal management of congenital diaphragmatic hernia today and tomorrow

Lennart van der VEEKEN ^{1, 2}, Francesca M. RUSSO ^{1, 2}, Johannes van der MERWE ^{1, 2}, David BASURTO ^{1, 2}, Dyuti SHARMA ¹, Tram NGUYEN ¹, Marie P. EASTWOOD ^{1, 3, 4}, Namesh KHOSHGOO ³, Jaan TOELEN ¹, Karel ALLEGAERT ¹, Philip DEKONINCK ⁵, Stuart B. HOOPER ⁵, Richard KEIJZER ³, Paolo DE COPPI ^{4, 6}, Jan DEPREST ^{1, 2, 6, 7} ✉

¹ Academic Department of Development and Regeneration, Cluster Woman and Child, Biomedical Sciences, KU Leuven, Leuven, Belgium; ² Clinical Department of Obstetrics and Gynecology, KU Leuven, Leuven, Belgium; ³ Division of Pediatric Surgery, Department of Surgery, University of Manitoba, Winnipeg, MB, Canada; ⁴ Institutes for Child Health, University College London, London, UK; ⁵ The Ritchie Centre, Hudson Institute of Medical Research, Monash University, Melbourne, Victoria, Australia; ⁶ Department of Obstetrics and Gynecology, Monash University, Melbourne, Victoria, Australia; ⁷ Institute for Women’s Health, University College London, London, UK

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.

KEY WORDS: Hernias, diaphragmatic, congenital - Fetal therapy - Sildenafil citrate