Congenital Chylothorax: Common and Uncommon Findings in an Infant with Chylous Effusions

Congenital chylothorax is defined as an abnormal accumulation of lymphatic fluid in the pleural space. It is a rare condition in the neonate that causes significant respiratory, nutritional, and immunologic problems resulting in a high mortality rate. Presented here is a case of congenital bilateral chylothorax in a preterm infant. Fetal ultrasound at 33 weeks' gestation showed polyhydramnios, bilateral pleural effusions, ascites, and subcutaneous edema. Fetal pleuracentesis was done on the right side with 118 mL of fluid aspirated. This article addresses the management of this difficult disease process, complications, and implications for research of controversial therapies.