Long-term follow-up of children with congenital chylothorax.

Congenital chylothorax, defined as the accumulation of lymphatic fluid in the pleural cavity, is a rare neonatal disorder. Nevertheless, congenital chylothorax represents the most common cause of pleural effusion in fetuses and newborns [1]. Due to the rarity of this disorder only a few case series are reported in the literature and current treatment recommendations are based on individual case reports or case series only [2–5]. The diagnosis of chylothorax is considered when pleural fluid triglyceride levels are .1.1 mmol?L and total cell counts are .1,000 cells per mL with .80% lymphocytes. Without oral fat intake chylomicrons, which are often an indication for the diagnosis of chylothorax, are absent in the pleural fluid and distinction between chylous and non-chylous effusion might be difficult [6]. Long-term follow-up of children with congenital chylothorax, however, was documented in only four studies [7–10] and none included pulmonary function testing. We describe the clinical course and long-term outcome of infants with congenital chylothorax including neurodevelopmental follow-up and pulmonary function testing.