Congenital pulmonary lymphangiectasis resulting in pleural effusions managed by thoracoamniotic shunting

Abstract

Congenital pulmonary lymphangiectasis (CPL) is a rare developmental disorder that has been associated with primary fetal pleural effusions. A 22 year old, Gravida-1 Caucasian woman presented at 26 gestational weeks with bilateral pleural effusions and hydrops fetalis. Fetal ultrasound revealed rapidly expanding pleural effusions and hydrops fetalis. A throacoamniotic shunt was placed in the left pleural space at 27 gestational weeks. The patient eventually developed severe preeclampsia and non-reassuring fetal heart tones necessitating immediate cesarean delivery at 32 gestational weeks. Persistent bilateral pleural effusions were noted after delivery and chest tubes were immediately placed. Despite maximal ventilatory support, continued bowel rest and an octreotide drip, both chest tubes continued to drain a significant amount of chylous fluid. The neonate eventually developed multi-organ failure and expired on day of 19. Autopsy findings revealed diffuse CPL. CPL causes markedly distended pulmonary lymphatics that result in accumulation of fluid within the fetal pleural spaces. We report a case of CPL associated fetal pleural effusions managed antenatally by throacoamniotic shunting.