Conflict of interest: None declared.
Congenital chylothorax: Associations and neonatal outcomes
Article first published online: 23 DEC 2013
© 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians)
Journal of Paediatrics and Child Health
Volume 50, Issue 3, pages 234–238, March 2014
How to Cite
Downie, L., Sasi, A. and Malhotra, A. (2014), Congenital chylothorax: Associations and neonatal outcomes. Journal of Paediatrics and Child Health, 50: 234–238. doi: 10.1111/jpc.12477
- Issue published online: 27 MAR 2014
- Article first published online: 23 DEC 2013
- Manuscript Accepted: 12 AUG 2013
- hydrops fetalis;
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.
This is a retrospective case series of all cases of congenital chylothorax admitted to a tertiary neonatal centre in the last 15 years.
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.
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.