Fetal thoracoamniotic shunting as the only treatment for pulmonary sequestration with hydrops: favorable long-term outcome without postnatal surgery

Abstract

Although it is established that bronchopulmonary sequestration complicated by fetal hydrops carries a high risk of perinatal mortality, prenatal management remains controversial. Therapeutic options include immediate delivery, medical therapy based on an inotropic regimen, alcohol ablation of the vascular pedicle, open fetal surgery, or thoracoamniotic shunting of fetal pleural effusions. We report a case of pulmonary sequestration with hydrothorax and fetal hydrops which was successfully treated by a single fetal thoracoamniotic catheter drainage. Following emergency catheter placement, hydrothorax and hydrops decreased dramatically. The sequestration gradually disappeared postnatally, and long-term outcome remained normal without any postnatal therapy. Based on this observation, the natural history of pulmonary sequestration and prenatal management options are reviewed.