Case Reports in Pediatrics

Case Reports in Pediatrics / 2019 / Article

Case Series | Open Access

Volume 2019 |Article ID 3903598 |

Marie K. White, Ravindra Bhat, Anne Greenough, "Neonatal Chylothoraces: A 10-Year Experience in a Tertiary Neonatal Referral Centre", Case Reports in Pediatrics, vol. 2019, Article ID 3903598, 4 pages, 2019.

Neonatal Chylothoraces: A 10-Year Experience in a Tertiary Neonatal Referral Centre

Academic Editor: Atul Malhotra
Received07 Dec 2018
Accepted27 Feb 2019
Published13 Mar 2019


Background. Neonatal chylothorax is a rare condition, but has a high mortality. Study Objectives. To analyse the outcomes of a series of neonates with chylothorax and review the literature to determine best practice. Design. A case series review and a literature review using electronic databases including the key words neonates and chylothorax. Results. Six cases of neonatal chylothorax were identified during a ten-year period, two had congenital chylothoraces and four iatrogenic chylothoraces after thoracic surgery or chest instrumentation. The neonates were ventilated for a median of 30 (range 13–125) days with a median maximum daily pleural fluid output of 218 (range 86–310) ml/kg/day. All the neonates were given medium-chain triglyceride (MCT) feeds which stabilised pleural fluid output in four and reduced it in another. Octreotide was used in three neonates, but the dosage used had no significant effect on pleural output. Two neonates required surgical intervention. The literature review demonstrated MCT feeds can reduce or stabilise pleural fluid output, but highlighted variable use of octreotide and inconsistent dosing regimens and outcomes. No consensus regarding indications for surgical intervention was identified. Summary and Conclusion. Neonatal chylothorax is uncommon, but affected neonates require high healthcare utilisation.

1. Introduction

Neonatal chylothorax is a rare condition with an incidence of 1 in 5775 [1] to 24000 [2] and has a high mortality rate of up to 64% [2]. Neonatal chylothorax can be congenital or acquired, the latter most commonly occurs after damage to the thoracic duct during surgery [3]. We have reviewed our experience in a tertiary neonatal surgical unit and set this in the context of a literature review to establish if it was possible to determine best practice.

2. Case Series

2.1. Method

A retrospective study was undertaken to determine the outcomes of all neonates with neonatal chylothoraces admitted to a tertiary medical and surgical neonatal intensive care unit in London, UK, from July 2008 to July 2018. Neonates were identified through the hospital clinical coding and an electronic database (BadgerNet). The diagnosis of a neonatal chylothorax was made if the pleural fluid had a cell count of >1000/ml with a lymphocyte predominance and if the triglyceride level was greater than 1.1 mmol/L in a neonate who was being fed [4]. Initial treatment for our cohort included stabilisation, ventilatory support, and replacement of pleural fluid losses. Specific treatment included medium-chain triglyceride (MCT) feeds, and then octreotide if the pleural fluid output continued, followed by surgical intervention if those interventions failed.

A literature review was undertaken using electronic databases including the key words neonates and chylothorax.

2.2. Results

There were 54,488 births in the study period, and six neonates were diagnosed with chylothorax, giving a local overall incidence of 1 in 9081 births. The six neonates had a median gestation of 37 + 6 (range 33 + 5 to 39 + 6) weeks and a median birth weight of 2935 (range 2252–4078) grams (Table 1). All neonates had a “lymphocytic” effusion, and three had a raised pleural fluid triglyceride of 2.2 (range 1.7–3.2) mmol/L. Two neonates had genetic variants of unknown significance and one, with subtle dysmorphic features, had a benign copy variant of paternal inheritance.

NeonateBirth weight (g)Gestation (weeks)AntenatalMode of deliveryResuscitationDiagnosesMCT feedsOctreotide and doseVentilation (days)Outcome

1308039NormalVDThick meconium intubated at 10 minutesAcquired NC, MAS, multiple pneumothoracesYesNo38Alive
2225233Hydrops, bilateral effusions, antenatal shuntsVDPleural shunts clamped & intubated at birthCongenital NCYesYes (2–8 mcg/kg/hr)31Alive
3407838Left pleural effusion & mediastinal shiftCSIntubated at birthCongenital NCYesNo28Alive
4279037Left CDHVDIntubated at birthAcquired NC, CDHYesYes (1–4 mcg/kg/hr)125Died
5269038PolyhydramniosVDNo resuscitationAcquired NC, OA, TOFYesYes (1–2 mcg/kg/hr)13Alive
6335037PolyhydramniosVDNo resuscitationAcquired NC, OA, TOFYesNo19Alive

CDH: congenital diaphragmatic hernia; CS: caesarian section; MAS: meconium aspiration syndrome; MCT: medium-chain triglycerides; NC: neonatal chylothorax; OA: oesophageal atresia; TOF tracheoesophageal fistula; VD: vaginal delivery; neonate had a reduction in pleural output following introduction of MCT feeds; octreotide was given prior to surgical intervention.

Four neonates required intubation in the delivery suite, and all required an extended period of mechanical ventilation with a median duration of 30 (range 13–125) days (Table 1). Two neonates required high-frequency oscillation ventilation and inhaled nitric oxide. Five neonates were successfully extubated and self-ventilating in air by a median of 37 (range 30–78) days. The neonates required a median of five (range 2–6) chest drains for a median duration of 36 (range 28–99) days. The median maximum total daily pleural fluid output was 218 (range 86–310) ml/kg/day. The majority of pleural drain losses were replaced by human albumin solution (HAS) or occasionally by 0.9% saline or fresh frozen plasma (FFP).

All the neonates required total parental nutrition and were commenced on MCT feeds after diagnosis. Five achieved full MCT feeds at a median of 37 (range 11–44) days of age. One neonate had resolution of the chylothorax following introduction of MCT feeds. In four neonates, MCT feeds were associated with stabilisation of the pleural fluid output. Two neonates were graded back to either a standard neonatal formula or maternal expressed breast milk, and three were discharged on MCT feeds. One neonate had increased pleural fluid output after the introduction of MCT feeds which were subsequently stopped and the neonate remained on total parental nutrition until reorientation of care.

Three neonates were treated with a continuous octreotide intravenous infusion for a median of seven (range 6–16) days with a starting dose of 1-2 micrograms/kg/hr to a maximum of 2–8 micrograms/kg/hr. One neonate developed transient hypothyroidism and, therefore, remained on low-dose octreotide at 2 micrograms/kg/hr, which was stopped after thoracic duct ligation. Octreotide was stopped in the other two neonates as there was no reduction in pleural fluid output. Two neonates with acquired chylothorax required operative intervention. One had a successful thoracic duct ligation and one had bilateral pleurodesis, but this failed to improve the neonate’s outcome. Five neonates survived, and the total length of stay until discharge or death was a median of 68 (range 44–127) days.

3. Discussion

Neonatal congenital chylothorax is a rare condition as evidenced by an incidence of 1 in 27,244 in our cohort, which is in keeping with another study (1 in 24,000 [2]). Our mortality (17%) is in accordance with other studies [1, 2, 12]. Up to 50% of neonates with neonatal chylothorax can be associated with genetic syndromes [1, 14]. In our cohort, although two neonates had genetic variants, none were found to have a defined syndrome.

In our cohort, only one neonate had a reduction in pleural fluid output following the introduction of MCT feeds, but in others the output was stabilised. MCT feeds have been shown to be effective in reducing lymphatic flow in 25% of neonates with surgically related neonatal chylothorax [15]. Indeed, our literature review (Table 2) demonstrated that the majority of neonates who have a neonatal chylothorax receive MCT feeds.

Author and yearNumber of neonatesVentilation (days)Number given MCT feedsNumber treated with octreotide and doseOctreotide duration (days)Octreotide efficacyPleurodesis or thoracic duct ligation

Altuncu et al. (2007) [5]3N/Rn = 3n = 1 (1–10 mcg/kg/hr)28No responsen = 1
Matsukuma et al. (2009) [6]2N/Rn = 2n = 2 (0.5–10 mcg/kg/hr)16–20No responsen = 2
Bellini et al. (2012) [7]3030n = 28n = 6 (1–10 mcg/kg/hr)8–38Decreased chylous production, but not quantifiedn = 1
Horvers et al. (2012) [8]716n = 7n = 7 (2–12 mcg/kg/hr)Median 22 (11–46)Possible response in two neonatesn = 0
Shah and Sinn (2012) [9]630n = 6n = 6 (0.5–10 mcg/kg/hr)Median 20 (4–41)Resolution in five neonatesn = 0
Downie et al. (2013) [1]108n = 7n = 3 (3.5–10 mcg/kg/hr)N/RResponse in two neonates: not quantifiedn = 0
Landis et al. (2013) [10]1111N/Rn = 6 (1–13 mcg/kg/hr)N/RNo responsen = 0
Bialkowski et al. (2015) [2]2823n = 25n = 7 (3–10 mcg/kg/hr)Median 21 (10–45)No responsen = 0
Hua et al. (2016) [11]41N/Rn = 3 (N/A)14No responsen = 4
Yin et al. (2017) [12]1413n = 12n = 3 (1–6 mcg/kg/hr)Median 6Average drain output significantly lower: three days after treatment. Median 62 ml versus 133 ml ()n = 0
Shillitoe et al. (2018) [13]21N/Rn = 2n = 1 (maximum dose 10 mcg/kg/hr)N/ROn the maximum dose there was a 50% reduction in five daysn = 0
Zaki et al. (2018) [14]9N/Rn = 9n = 9 (1–10 mcg/kg/hr)5–66Resolution in three neonates: not quantifiedn = 0

N/R: not reported.

A systematic review in which the efficacy of octreotide, a somatostatin analogue, was assessed did not recommend its use [4]. Our literature review demonstrated since then multiple case series in which octreotide was used have been published with variable dosing regimens, length of treatment, and outcomes and that octreotide was not used in all neonates (Table 2) [1, 2, 714]. A recent systematic review [3] found octreotide to be effective in 47% of neonates with neonatal chylothorax. Many of the papers included, however, did not describe what constituted a clinical improvement or the timescale over which any changes occurred. In our cohort, there was no significant improvement in the pleural fluid output in the three neonates treated with octreotide. None of these neonates reached the dose of 10 micrograms/kg/hr which some studies have used. Multiple side effects have been reported in up to 14% neonates treated with octreotide including hyperglycaemia, necrotising enterocolitis, transient mild cholestasis, transient hypothyroidism, pulmonary hypertension, and severe hypotension [3]. In our cohort, one neonate developed transient hypothyroidism.

The literature review (Table 2) demonstrated only a minority of neonates required either pleurodesis or thoracic duct ligation. This is consistent with the findings of our cohort. A review of a case series of neonatal “surgical” chylothoraces mainly following congenital diaphragmatic hernia repair also demonstrated conservative management was successful in the majority [15]. It would be important to develop evidence-based guidelines as to when such interventions should be used.

4. Conclusions

Our results highlight neonatal chylothorax is an uncommon condition, but is associated with high healthcare utilisation. Neonates require stabilisation with ventilation, pleural drainage, and fluid replacement. MCT feeds can reduce and stabilise pleural fluid output. It is important to optimise the dose of octreotide and monitor for side effects. Multicentre trials are required to identify the optimum evidenced-based management for neonatal chylothorax. Although neonatal chylothorax is an uncommon condition, we believe that multicentre trials would be feasible as shown by the randomised trial of respiratory support in neonates with congenital diaphragmatic hernia [16].


The views expressed are those of the authors and not necessarily those of the NHS, the NIHR, or the Department of Health.

Conflicts of Interest

The authors declare that there are no conflicts of interest regarding the publication of this paper.


This research was supported by the National Institute for Health Research (NIHR) Biomedical Research Centre at Guy’s and St Thomas’ NHS Foundation Trust and King’s College London.


  1. L. Downie, A. Sasi, and A. Malhotra, “Congenital chylothorax: associations and neonatal outcomes,” Journal of Paediatrics and Child Health, vol. 50, no. 3, pp. 234–238, 2014. View at: Publisher Site | Google Scholar
  2. A. Bialkowski, C. F. Poets, and A. R. Franz, “Congenital chylothorax: a prospective nationwide epidemiological study in Germany,” Archives of Disease in Childhood Fetal Neonatal Edition, vol. 100, no. 2, pp. F169–F172, 2015. View at: Publisher Site | Google Scholar
  3. C. Bellini, R. Cabano, L. C. De Angelis et al., “Octreotide for congenital and acquired chylothorax in newborns: a systematic review,” Journal of Paediatrics and Child Health, vol. 54, no. 8, pp. 840–847, 2018. View at: Publisher Site | Google Scholar
  4. A. Das and P. S. Shah, “Octreotide of the treatment of chylothorax in neonates,” Cochrane Database of Systematic Reviews, vol. 9, no. 8, Article ID CD006388, 2010. View at: Publisher Site | Google Scholar
  5. E. Altuncu, İ. Akman, G. Kıyan et al., “Report of three cases: congenital chylothorax and treatment modalities,” Turkish Journal of Pediatrics, vol. 49, no. 4, pp. 418–421, 2007. View at: Google Scholar
  6. E. Matsukuma, Y. Aoki, M. Sakai et al., “Treatment with OK-432 for persistent congenital chylothorax in newborn infants resistant to octreotide,” Journal of Pediatric Surgery, vol. 44, no. 3, pp. E37–E39, 2009. View at: Publisher Site | Google Scholar
  7. C. Bellini, Z. Ergaz, M. Radicioni et al., “Congenital fetal and neonatal visceral chylous effusions: neonatal chylothorax and chylous ascites revisited: a multicenter retrospective study,” Lymphology, vol. 45, no. 3, pp. 91–102, 2012. View at: Google Scholar
  8. M. Horvers, C. F. Mooij, and T. A. J. Antonius, “Is octreotide treatment useful in patients with congenital chylothorax,” Neonatology, vol. 101, no. 3, pp. 225–231, 2012. View at: Publisher Site | Google Scholar
  9. D. Shah and J. K. Sinn, “Octreotide as therapeutic option for congenital idiopathic chylothorax: a case series,” Acta Paediatrica, vol. 101, no. 4, pp. e151–e155, 2012. View at: Publisher Site | Google Scholar
  10. M. W. Landis, D. Butler, F. Y. Lim et al., “Octreotide for chylous effusions in congenital diaphragmatic hernia,” Journal of Pediatric Surgery, vol. 48, no. 11, pp. 2226–2229, 2013. View at: Publisher Site | Google Scholar
  11. Q.-W. Hua, Z.-Y. Lin, X.-T. Hu, and Q.-F. Zhao, “Treatment of persistent congenital chylothorax with intrapleural injection of sapylin in infants,” Pakistan Journal of Medical Science, vol. 32, no. 5, pp. 1305–1308, 2016. View at: Publisher Site | Google Scholar
  12. R. Yin, R. Zhang, J. Wang et al., “Effects of somatostatin/octreotide treatment in neonates with congenital chylothorax,” Medicine, vol. 96, no. 29, Article ID e7594, 2017. View at: Publisher Site | Google Scholar
  13. B. M. J. Shillitoe, J. Berrington, and N. Athiraman, “Congenital pleural effusions: 15 years single-centre experience from North-East England,” Journal of Maternal-Fetal & Neonatal Medicine, vol. 31, no. 15, pp. 2086–2089, 2018. View at: Publisher Site | Google Scholar
  14. S. A. Zaki, M. H. Krishnamurthy, and A. Malhotra, “Octreotide use in neonates: a case series,” Drugs in R&D, vol. 18, no. 3, pp. 191–198, 2018. View at: Publisher Site | Google Scholar
  15. K. M. Costa and A. K. Saxena, “Surgical chylothorax in neonates: management and outcomes,” World Journal of Pediatrics, vol. 14, no. 2, pp. 110–115, 2018. View at: Publisher Site | Google Scholar
  16. K. G. Snoek, I. Capolupo, J. van Rosmalen et al., “Conventional mechanical ventilation versus high-frequency oscillatory ventilation for congenital diaphragmatic hernia,” Annals of Surgery, vol. 263, no. 5, pp. 867–874, 2016. View at: Publisher Site | Google Scholar

Copyright © 2019 Marie K. White et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

More related articles

 PDF Download Citation Citation
 Download other formatsMore
 Order printed copiesOrder

Related articles

Article of the Year Award: Outstanding research contributions of 2020, as selected by our Chief Editors. Read the winning articles.