Congenital Chylothorax of the Newborn: A Systematic Analysis of Published Cases between 1990 and 2018

Respiration. 2022;101(1):84-96. doi: 10.1159/000518217. Epub 2021 Sep 1.


Background: Congenital chylothorax (CCT) of the newborn is a rare entity but the most common cause of pleural effusion in this age-group. We aimed to find the optimal treatment strategy.

Material and methods: A PubMed search was performed according to the PRISMA criteria. All cases were analyzed according to prenatal, perinatal, and postnatal treatment modalities and follow-ups.

Results: We identified 753 cases from 157 studies published between 1990 and 2018. The all-cause mortality rate was 28%. Prematurity was present in 71%, male gender dominated 57%, mean gestational age was 34 weeks, and birth weight was 2,654 g. Seventy-nine percent of newborns had bilateral CCT, the most common associated congenital anomalies with CCT were pulmonary lymphangiectasia and pulmonary hypoplasia, and the most common chromosomal aberrations were Down, Noonan, and Turner syndromes, respectively. Mechanical ventilation was reported in 381 cases for mean 17 (range 1-120) days; pleural punctuations and drainages were performed in 32% and 64%, respectively. Forty-four percent received total parenteral nutrition (TPN) for mean 21 days, 46% medium-chain triglyceride (MCT) diet for mean 37 days, 20% octreotide, and 3% somatostatin; chemical pleurodesis was performed in 116 cases, and surgery was reported in 48 cases with a success rate of 69%. In 462 cases (68%), complete restitution was reported; in 34 of 44 cases (77%), intrauterine intervention was carried out.

Conclusion: Respiratory support, pleural drainages, TPN, and MCT diet as octreotide remain to be the cornerstones of CCT management. Pleurodesis with OK-432 done prenatally and povidone-iodine postnatally might be discussed for use in life-threatening CCT.

Keywords: Associated anomalies; Congenital chylothorax; Mortality; OK-432; Octreotide; Pleurodesis; Povidone-iodine.

Publication types

  • Systematic Review

MeSH terms

  • Chylothorax* / congenital
  • Chylothorax* / therapy
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Octreotide / therapeutic use
  • Pleural Effusion* / etiology
  • Pleurodesis / adverse effects


  • Octreotide

Supplementary concepts

  • Chylothorax, congenital