Fetal intervention for congenital diaphragmatic hernia: the European experience

Fetuses with CDH presenting with liver herniation and a lung area-to-head circumference ratio of less than 1.0 have a high chance for neonatal death due to pulmonary hypoplasia. Fetal tracheal occlusion (TO) prevents egress of lung liquid, which triggers lung growth. In animal experiments, we were able to develop a minimally invasive technique for Fetoscopic Endoluminal Tracheal Occlusion (FETO) with a detachable balloon. In 2001, we demonstrated feasibility of FETO by percutaneous access in fetuses with severe CDH. In a retrospective multicenter review, we obtained LHR measurements and position of the liver in 134 cases of isolated left-sided CDH between 24 and 28 weeks. Eleven patients (8%) with LHR < 1.4 opted for termination. Overall survival of liveborn babies was 47% (58/123). LHR and position of the liver correlated both to survival. Combination of both variables predicted neonatal outcome better: liver up and LHR < 1.0 predicted a survival of 9%. When LHR < 0.6, there were no survivors irrespective of liver position. We could successfully perform endotracheal placement of the balloon in 20 cases at a median gestational age of 26 weeks. The mean duration of the operation was 22 (range 5-54) minutes. In 11 (55%) of these patients, there was postoperative prelabor (ie, <37 weeks) amniorrhexis. Membranes ruptured before 32 weeks in 35%, with a decreasing trend as experience increased. Ultrasound scans after FETO demonstrated an increase in the echogenicity of the lungs within 48 hours and improvement in the LHR from a median 0.7 (range 0.4-0.9) before FETO to 1.8 (range 1.1-2.9) within 2 weeks after surgery. The median gestation at delivery was 33.2 (range 27-38) weeks, and in 14 (70%) this occurred after 32 weeks. Surgical repair of the diaphragmatic hernia could be done in 13 babies, and in all but 1 the defect was extensive and required the insertion of a patch. Survival to discharge was 50%. These 10 long-term surviving babies are now aged 7 to 26 (median 19) months without known neurologic morbidity. Eight babies died in the neonatal period due to complications of the underlying disease. Two nonsurvivors died from other causes but with appropriately developed lungs. Improved survival coincided with increasing experience, in turn related to reduced incidence of postoperative amniorrhexis, later delivery, and a change in the policy on the timing of removal of the balloon from intrapartum to the prenatal period. Survival in eligible contemporary controls was 1/12 (8%). The presence of liver herniation and a low lung-to-head ratio (LHR <1.0) is a good predictor of poor prognosis at different tertiary centers around the world. Severe CDH may be successfully treated with FETO, which is minimally invasive and may improve postnatal survival.