title: Congenital chylothorax: associations and neonatal outcomes.
abstract: OBJECTIVE
  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.
authors.affiliation: Monash Newborn, Monash Medical Centre, Melbourne, Victoria, Australia.
authors.forename: Lilian
authors.initials: L
authors.lastname: Downie
authors.forename: Arun
authors.initials: A
authors.lastname: Sasi
authors.forename: Atul
authors.initials: A
authors.lastname: Malhotra
mesh_terms: D002916:Chylothorax / Q000151:congenital* / Q000628:therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature; D007235:Infant, Premature, Diseases / Q000628:therapy*; D008297:Male; D008485:Medical Audit; D017063:Outcome Assessment, Health Care; D012189:Retrospective Studies
keywords: chylothorax; genetic; hydrops fetalis; neonate