Prenatal surgery for congenital diaphragmatic hernia

Abstract

Congenital diaphragmatic hernia (CDH) has a mortality rate of up to 77% despite optimal pre- and postnatal care. Fetuses with liver herniation, a low lung-to-head ratio, and an early diagnosis before 24 weeks have a particularly poor prognosis. In utero open repair of these fetuses does not improve patient survival. The PLUG (Plug the Lung Until it Grows) technique was reported to be able to reverse pulmonary hypoplasia in CDH. A foam plug or a titanium clip is used and the trachea can be unplugged using Ex Utero Intrapartum Tracheoplasty (EXIT) at birth. Since hysterotomy causes premature labour, a video-fetoscopic intrauterine technique of tracheal occlusion called Fetendo-PLUG was developed. Compared to those who receive standard postnatal care or fetal tracheal occlusion via open hysterotomy, patients who undergo Fetendo-PLUG are reported to have a higher survival rate of 75% and fewer fetal and maternal complications. A recent refinement is to use a detachable balloon for intratracheal occlusion through a single 5 mm port under real-time ultrasound guidance. Without the need for neck dissection, injury to the recurrent laryngeal nerves and trachea and vocal cord paresis can be minimized. The result of this form of treatment for CDH is promising, but further refinement of fetal instrumentation and development of effective tocolytic drugs are still required.