Congenital pulmonary lymphangiectasia and chylothorax - a case series.

Congenital pulmonary lymphangiectasia (CPL) and chylothorax (CC) are rare lymphatic developmental disorders. We report six clinical cases of CPL and CC that were admitted to our level III neonatal intensive care unit over the last 20 years. One case of unilateral CC was successfully treated with pleuro-amniotic shunt; three cases of bilateral CC were associated to lung hypoplasia, hydrops fetalis, and generalized lymphangiectasias; one case of CPL was associated with obstructive congenital heart defect; one case of unilateral CC was successfully treated with thoracocentesis and medium-chain triglyceride diet. Mortality was high (66.6%).