Congenital chylothorax: associations and neonatal outcomes.

Aim: Congenital chylothorax is a rare but significant neonatal entity with major morbidity and mortality. The study aims to describe the related associations, management and outcomes of this condition in neonates.

Methods: This is a retrospective case series of all cases of congenital chylothorax admitted to a tertiary neonatal centre in the last 15 years.

Results: Ten cases of congenital chylothorax were identified. Eight infants were diagnosed antenatally and three infants had antenatal pleural drainage. Most infants were ventilated at birth and required immediate pleurocentesis. Post-natal management included drainage of fluid, ventilation, albumin replacement, octreotide and dietary modification with medium-chain triglyceride-enriched formula. Five infants had chromosomal aberrations identified, while a further two had dysmorphic features not substantiated with routine genetic testing. Noonan's syndrome was the single most common underlying genetic diagnosis. Associated anomalies and malformations were present in 80% of the cohort. There were two deaths in the series, both in infants with multiple co-morbidities.

Conclusions: Congenital chylothorax is a rare condition with overall prevalence of less than a case per year in our experience. Majority of infants had associated chromosomal anomalies and significant co-morbidities needing prolonged intensive care.