Management of Congenital Chylothorax in Intensive Neonatal Care Unit in Sfax, Tunisia: A Case Series and Review of the Literature

Document Type : Case Report

Authors

Department of neonatology, Hedi Chaker Hospital, University of Sfax, Sfax, Tunisia

Abstract

Background: Congenital Chylothorax (CC) is a rare condition, which is defined as an accumulation of the chyle in the pleural cavity;
moreover, it is associated with significant morbidities, including respiratory distress, malnutrition, immunodeficiency, and infections. 
Case Report: The diagnosis of chylothorax was made upon count cell analysis of the pleural fluid with ≥80% lymphocytes
detected before birth or within 28 days after birth. In this study, we presented five cases of CC infants. They were discharged
from our tertiary center at Hedi Chaker Hospital, Sfax, Tunisia, from January 2010 to December 2018. There were three males
and two females. Prenatal diagnosis was made in four cases. There were four full-term newborns and one near-term of 36
weeks. Pleural effusion was on the right side in three cases, on the left side in one case, and bilateral in one case. Four cases
required mechanical ventilation. Somatostatin was indicated in one case. The treatment was successful in four cases. One case
presented a dysmorphic syndrome and died of pneumothorax.
Conclusion: The treatment of CC is based on conservative management. Somatostatin or its analog octreotide is considered an
adjunctive treatment of CC. However, the refractory cases are treated with chemical pleurodesis or surgical treatment. We
propose an algorithm for the treatment of CC.
 

Keywords


  1. Al-Tawil K, Ahmed G, Al-Hathal M, Al-Jarallah Y, Campbell N. Congenital chylothorax. Am J Perinatol. 2000;17(3):121–126.
  2. Attar MA, Donn SM. Congenital chylothorax. Semin Fetal Neonatal Med. 2017;22(4):234-239.
  3. Toptan HH, Ozalkaya E, Karadag N, Topcuoglu S, Dincer E, Karatekin G. Neonatal Lymphatic Flow Disorder. Indian J Pediatr. 2023:1-6.
  4. Chen Y, Zhang D, Li Y, Hu X, Mi R, Kang L, Liu S, et al. Clinical characteristics and management of congenital chylothorax refractory to conservative treatment: a retrospective study of 11 cases from a single center. Minerva Med.
  5. Handal-Orefice R, Midura D, Wu JK, Parravicini E, Miller RS, Shawber CJ. Propranolol therapy for congenital chylothorax. Pediatrics. 2023;151(2): e2022058555.
  6. Resch B, Sever Yildiz G, Reiterer F. Congenital Chylothorax of the Newborn: A Systematic Analysis of Published Cases between 1990 and 2018. Respiration. 2022;101(1):84-96.
  7. Büttiker V, Fanconi S, Birger R. Chylothorax in children: guidelines for diagnosis and management. Chest 1999; 116(3):682–7.
  8. Wang B, Feng Y, Guo Y, Kan Q, Zou Y, Wu Y, Zheng M, et al. Clinical features and outcomes of congenital chylothorax: a single tertiary medical center experience in China. J Card Surg. 2022;17(1):276.
  9. Bhagiratha S, Bandiya P, Shivanna N, Benakappa N. Congenital Chylothorax: Conservative Management Counts. J Indian Assoc Pediatr Surg. 2022; 27(5):652-54.
  10. Höck M, Höller A, Hammerl M, Wechselberger K, Krösslhuber J, Kiechl-Kohlendorfer U, et al. Dietary treatment of congenital chylothorax with skimmed breast milk. Ital J Pediatr. 2021;47(1):175.
  11. Goto M, Kawamata K, Kitano M, Watanabe K, Chiba Y. Treatment of chylothorax in a premature infant using somatostatin. J Perinatol. 2003;23(7):563-4.
  12. Bellini C, Cabano R, De Angelis LC, Bellini T, Calevo MG, Gandullia P, et al. Octreotide for congenital and acquired chylothorax in newborns: A systematic review. J Paediatr Child Health. 2018;54(8):840-47.
  13. Zaki SA, Krishnamurthy MB, Malhotra A. Octreotide Use in Neonates: A Case Series. Drugs R D. 2018;18(3):191-8.
  14. Kankananarachchi I, Priyankara KKS, Lakman KKK, Withanaarachchi K, Gunathilaka PKG. Two Cases of Congenital Chylethorax: A Successful Story of Medical Management. Case Rep Pediatr. 2021;
    2021:1-4.
  15. Maayan‐Metzger A, Sack J, Mazkereth R, Vardi A, Kuint J. Somatostatin treatment of congenital chylothorax may induce transient hypothyroidism in newborns. Acta Paediatr. 2005;94(6):785-9.
  16. Krishnamurthy MB, Malhotra A. Congenital chylothorax: current perspectives and trends. Research and Reports in Neonatology. 2017;7:53-63.
  17. Costa KM, Saxena AK. Surgical chylothorax in neonates: management and outcomes. World J Pediatr. 2018;14:110–5.
  18. Healy H, Gipson K, Hay S, Bates S, Kinane TB. Management and outcomes of congenital chylothorax in the neonatal intensive care unit: A case series. PediatrInvestig. 2017;1(1):21-5.
  19. Malleske DT, Yoder BA. Congenital chylothorax treated with oral sildenafil: a case report and review of the literature. J Perinatol. 2015;35(5):384-6.
  20. Das A, Shah PS. Octreotide for the treatment of chylothorax in neonates. Cochrane Database Syst Rev. 2010;9:CD006388.
  21. Krishnamurthy MB, Malhotra A. Congenital chylothorax: current perspectives and trends. Research and Reports in Neonatology. 2017 ;
    7:53-63.
  22. Brissaud O, Desfrere L, Mohsen R, Fayon M, Demarquez JL. Congenital idiopathic chylothorax in neonates: chemical pleurodesis with povidone-iodine (Betadine). Arch Dis Child Fetal Neonatal Ed. 2003;88(6): F531–3.
  23. Mitanchez D, Walter‐Nicolet E, Salomon R, Bavoux F, Hubert P. Congenital chylothorax: what is the best strategy? Arch Dis Child FetalNeonatal Ed. 2006;91(2):F153-4.
  24. Murki S, Faheemuddin M, Gaddam P. Congenital Chylothorax-Successful management with chemical pleurodesis. Indian J Pediatr. 2010;77(3):332-4.
  25. Le Nué R, Molinaro F, Gomes-Ferreira C, Scheib-Brolly C, Escande B, Kühn P, et al. Surgical management of congenital chylothorax in children. Eur J Pediatr Surg 2010;20(5):307-11
  26. Hmami F, Oulmaati A, Bouchikhi C, Banani A, Bouharrou A. Congenital chylothorax: rapid and complete response to polyvidone iodine. Arch Pediatr 2014;21(9):1002-05.
  27. Scottoni F, Fusaro F, Conforti A, Morini F, Bagolan P. Pleurodesis with povidone–iodine for refractory chylothorax in newborns: Personal experience and literature review. J Pediatr Surg 2015;50(10):1722-5.
  28. Resch B, Freidl T, Reiterer F. Povidone-iodine pleurodesis for congenital chylothorax of the newborn. Arch Dis Child Fetal Neonatal Ed. 2016;101(1):87-8.
  29. Maoulainine F, Slitine NE, Erguig L. Congenital Chylothorax: Rapid and Effective Response to Polyvidone Iodine. Open J Pediatr. 2016;6(1):10-3.
  30. Kasdallah N, Kbaier H, Salem B, Blibech S, Bouziri A, Douagi M. Povidone Iodine Pleurodesis for Refractory CongenitalChylothorax: A Review of Literature. Tunis Med. 2016;94(12):834.
  31. Borcyk K, Kamil A, Hagerty K, Deer M, Tomich P, Berry AL. Successful management of extremely high-output refractory congenitalchylothorax with chemical pleurodesis using 4% povidone-iodine and propranolol: a case report. Clin Case Rep. 2018;6(4):702-08.
  32. Matsukuma E, Aoki Y, Sakai M, Kawamoto N, Watanabe H, Iwagaki S, et al. Treatment with OK-432 for persistent congenital chylothorax in newborn infants resistant to octreotide. J Pediatr Surg. 2009;44(3):e37–9.
  33. Kim JE, Lee C, Park KI, Park MS, Namgung R, Park IK. Successful pleurodesis with OK-432 in preterm infants with persistent pleural effusion. Korean J Pediatr. 2012;55(5):177–180.
  34. Hua QW, Lin ZY, Hu XT, Zhao QF. Treatment of persistent congenital chylothorax with intrapleural injection of sapylin in infants. Pak J Med Sci. 2016;32(5):1305-08.
  35. Sze SW, Ng PC, Lam HS. Life-Threatening Hemolytic Anemia after Intrapleural Instillation of OK-432 for Treatment of Congenital Chylothorax. Neonatology 2016;110(4):303–06
  36. Soto-Martinez M, Massie J. Chylothorax: diagnosis and management in children. Paediatr Respir Rev. 2009;10(4):199–07.

Yin R, Zhang R, Wang J, Yuan L, Hu L, Jiang S, et al. Effect of somatostatin/octreotide treatment in neonates with congenital chylothorax. Medicine. 2017;96(29):e7594.