The ex-utero intrapartum treatment (EXIT) strategy for congenital diaphragmatic hernia in a twin pregnancy: A case report and literature review

Congenital diaphragmatic hernia (CDH) is a complex birth defect and the etiology remains unknown. It is still challenged to estimate the prognosis and select the best prenatal therapy of CDH. The ex-utero intrapartum treatment (EXIT) is used in twin pregnancies is quite rare. We present the case of a 31-year-old woman with gravida 1, para 0 who had a dichorionic diamniotic twin by assisted reproductive technology (ART). The diaphragmatic hernia was found in one of the twins under sonographic examination during 18th week of pregnancy. The antenatal screenings for chromosomal abnormalities by Nuchal Translucency (NT), non-invasive prenatal testing (NIPT), amniocentesis were all normal. The lowest Lung area/head circumference ratio (LHR) was 1.0 and observed-to-expected (o/e) LHR was 26 % at 29 weeks. We scheduled the elective caesarean section at 37 weeks and treated through EXIT procedure. The defected twin A was treated by EXIT while the twin B was labored after. The total placental circulation time was 43 min. The perioperative blood loss of the mother was roughly 700 ml. Two babies were both survived and every follow-up of baby A had a positive outcome. Both brothers are now growing and developing like typical kids. The diaphragmatic defect can happen in single births or twins. The mortality of congenital diaphragmatic hernia is still high. Our case revealed a smooth and successful treatment. And the EXIT can be tried in twin pregnancies if one of babies was diseased and may get a positive prognosis.