Open fetal surgery for central bronchial atresia

Abstract

Mainstem or lobar bronchial atresia is associated with massive pulmonary hyperplasia, contralateral pulmonary hypoplasia, non-immune hydrops and a fatal fetal prognosis. Open fetal surgery currently provides a potential therapeutic option for management of a fetus with this diagnosis. We present 2 cases of open fetal surgery for bronchial atresia at the level of the mainstem and lobar bronchus. The first case involved a left pneumonectomy performed at 26 weeks' gestation. After successful intraoperative resuscitation for fetal cardiac arrest, the fetus survived until 32 weeks' gestation when preterm premature rupture of membranes prompted delivery. The neonate expired on day of life (DOL)1. The second case involved a left upper lobectomy performed at 24(3)/7 weeks' gestation. The fetus was delivered at 34(3)/7 weeks' gestation and, after a prolonged NICU course including a completion pneumonectomy and tracheostomy, was discharged home at 8 months of life. This second case is the first report of survival following open fetal surgery for central bronchial atresia. This report highlights the potential benefit of fetal lung resection for central bronchial atresia while describing the many challenges to achieve the successful management of a fetus with this condition.