Author:

Keywords:

Science & Technology, Life Sciences & Biomedicine, Pediatrics, Hernias, diaphragmatic, congenital, Fetal therapy, Sildenafil citrate, ENDOSCOPIC TRACHEAL OCCLUSION, SILDENAFIL CITRATE THERAPY, FLUID STEM-CELLS, PULMONARY-HYPERTENSION, LIVER HERNIATION, POSTNATAL MANAGEMENT, RABBIT MODEL, IN-VITRO, FETUSES, SURVIVAL, Female, Fetal Therapies, Fetoscopy, Hernias, Diaphragmatic, Congenital, Humans, Hypertension, Pulmonary, Phosphodiesterase 5 Inhibitors, Pregnancy, Prognosis, Sildenafil Citrate, 1114 Paediatrics and Reproductive Medicine, 3213 Paediatrics

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 development 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 and 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.