Correction of congenital diaphragmatic hernia in utero VII: a prospective trial

Abstract

Background: Congenital diaphragmatic hernia (CDH) remains an unsolved problem. Despite optimal postnatal care, up to 60% of CDH babies die. Experimental evidence and clinical experience have shown that in utero repair of CDH is feasible and can reverse pulmonary hypoplasia, but only in fetuses without liver herniation. For this subgroup, the safety and efficacy of repair before birth has not been compared with standard care after birth.

Methods: Four fetuses in whom CDH without liver herniation was diagnosed underwent open fetal surgery for repair of the CDH. Seven comparison fetuses were treated conventionally. Neonatal mortality was the principle outcome variable. Secondary outcome variables included death of all causes until 2 years of age, number of days of ventilatory support, length of hospital stay, requirement for extracorporeal membrane oxygenation (ECMO), and total hospital charges.

Results: There was no difference in survival between the fetal surgery group and the postnatally treated comparison group (75% v 86%). Fetal surgery patients were born more prematurely than the comparison group (32 weeks v 38 weeks' gestation). Length of ventilatory support and requirement for ECMO were equivalent in the fetal surgery group and the postnatally treated comparison group. Length of hospital stay and hospital charges did not differ between the groups.

Conclusions: Open fetal surgery is physiologically sound and technically feasible, but does not improve survival over standard postnatal treatment in the subgroup of CDH fetuses without liver herniation, primarily because overall survival in this subgroup is favorable with or without prenatal intervention. These data suggest that fetuses who have prenatally diagnosed CDH and without evidence of liver herniation should be treated postnatally.