RESUMO
BACKGROUND: Congenital chylothorax (CC) is a rare and potentially life-threatening condition. Over 50% occurs at birth and is considered as the most common cause of neonatal thoracic fluid collection. OBJECTIVES: To analyse the main clinical and respiratory features of a contemporary group of CC infants. METHODS: Databases for CC diagnosed between 2004 and 2009 were reviewed: 10 consecutive cases were retrieved and analysed. RESULTS: Median gestational age of CC patients was 31.8 weeks. Most patients were diagnosed prenatally (7/10 pts, median GA at diagnosis 28 weeks). Severe respiratory distress at birth required respiratory support: 7/10 newborns received high-frequency oscillatory ventilation (HFOV) electively. Large effusions and/or early-onset pneumothorax did not influence the outcome, while prematurity did not impact significantly on mortality (death rate <33 weeks: 28%). The overall ICU survival rate was 70%. CONCLUSION: CC still carries a significant risk of perinatal mortality. Continuous advances in foetal/neonatal medicine and intensive care have considerably improved the prognosis in the last decades, mostly in critically ill infants. HFOV improves lung opening and volume maintenance, possibly shortening the lymph flow over time. It can play a fundamental role both to prevent hypoxic and chronic lung damage and to improve lung recruitment in neonates born with CC.