Management and outcomes of congenital chylothorax in the neonatal intensive care unit: A case series

IMPORTANCE: Congenital chylothorax is a rare condition with pulmonary and multiorgan system effects, for which there are no standardized treatment recommendations. Collective review of known cases offers some conclusions and suggestions for treatment. OBJECTIVE: The aim of this study was to present a case series of 5 patients who were treated in the neonatal intensive care unit with chylothorax. METHODS: We describe 5 infants who were diagnosed prenatally with hydrops fetalis and postnatally had clinically significant congenital chylothorax. RESULTS: Treatment guidelines specific to congenital forms of chylothorax have not yet been developed, although there are consistent trends across our cases. Four of the 5 infants in this study have survived to date. Chylothorax was treated with chest tube placement and chylous fluid drainage, scrupulous attention to fluid balance, mechanical ventilation, and nutritional management and, in 3 cases, with octreotide infusions. Some of the infants also required treatment for immunodeficiency and altered coagulation pathways. None of the infants underwent surgical thoracic duct ligation. INTERPRETATION: Aided by the advantage of prenatal diagnosis, many cases of congenital chylothorax can be successfully treated by a combination of nutritional and medical management as well as careful attention to fluid and electrolyte balance and avoidance of infection, thereby avoiding the need for surgical ligation of the thoracic duct.