Fetal thoracic-amniotic shunt and postnatal repair in the management of a right congenital diaphragmatic hernia complicated by a large prenatal pleural effusion: A case report

Congenital diaphragmatic hernia (CDH) complicated by fetal pleural effusion and hydrops is rare and carries a poor prognosis, particularly when associated with severe pulmonary hypoplasia. A male fetus was diagnosed at 22 weeks’ gestation with severe right-sided CDH containing liver and bowel, complicated by large pleural effusion, ascites, and hydrops. At 25 weeks, a thoraco-amniotic shunt was placed for persistent chylous effusion, with improvement in O/E TFLV from 15–16% to 32–33%. The infant was delivered at 31+4 weeks and required surfactant, inhaled nitric oxide, and vasoactive support. He underwent patch repair of large type B defect at two weeks of life. He was extubated on postoperative day 6, weaned to room air by day of life 69, pulmonary hypertension resolved, and discharged on day of life 71. At follow-up, he is feeding fully by mouth and is progressing appropriately. Fetuses with CDH who develop large pleural effusions may benefit from prenatal thoraco-amniotic shunt placement.