Congenital pulmonary lymphangiectasia presenting as nonimmune fetal hydrops and severe respiratory distress at birth: Not uniformly fatal

Pulmonary lymphangiectasia is a rare cause of respiratory distress in the newborn associated with a very poor outcome. We describe three premature newborns presenting at birth with nonimmune hydrops, bilateral chylothorax, and severe respiratory distress in the immediate newborn period secondary to pulmonary lymphangiectasia. We review the similarities of these cases and discuss their antenatal and neonatal course. One patient survived and is thriving at 9 months of age. With continuing advances in antenatal and neonatal care, an improved outcome may be possible in what was previously described as a uniformly fatal condition.