Congenital chylothorax in newborn infants: Experience from 5 neonatal units.

Abstract

Aims: Congenital chylothorax is a rare disorder occurring in 1 in 10,000 births. This study reports the aetiology, clinical course, management and outcome of newborn infants with chylothorax who were managed in 5 NICUs. Method(s): Diagnostic criteria for chylothorax were: clear and pale yellow / chylous pleural fluid, high protein content (>=2g/dL), presence of numerous leukocytes predominantly (>=70%) lymphocytes, sterile on culture and exclusion of other causes. The data were collected from the case records of the infants. Result(s): Out of 21 infants, 13 (62%) had spontaneous chylothorax and 8 (32%) had associated malformations (Down Syndrome 2, Noonan Syndrome 2, Lymphangioma 2, multiple malformation 2). Majority (15) were diagnosed antenatally at 15-36 weeks including 14 (67%) who had non-immune hydrops fetalis. In 8 (38%) fetuses antenatal pleural taps were performed, including one who had thoracoamniotic shunt. Ten infants needed thoracocentesis in the delivery room. The pleural effusions were bilateral in 14 (67%), left-sided 5 and right-sided in 4. The infants were managed with assisted ventilation (nasal-prong CPAP 2, mechanical ventilation 15, HFOV 4), chest drainage for 7-47 days, parenteral nutrition for 7-52 days and replacement of pleural losses which ranged from 20-600ml per day. 3 refractory cases were treated with Octreotide infusions commencing from day 12-24. Mortality was higher in those associated with malformations than those without, (4 out of 8, 50% vs 1 out of 16, 8%) p=0.023. The chylous effusions resolved within a month in 14 (88%) infants and within 35 to 47 days in 2 (12%), and there were no recurrences in all of them. Conclusion(s): Congenital chylothorax has a favourable prognosis and outcome in the absence of associated major malformations. The role of early treatment with Octreotide in modifying the protracted course of this disorder needs to be studied by a randomised trial.