Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Etiology, clinical manifestations, diagnosis and evaluation of pleural effusions in the neonate

Joseph B Philips III, MD, FAAP
Section Editor
Leonard E Weisman, MD
Deputy Editor
Melanie S Kim, MD


Although pleural effusions in neonates are rare, it is important to identify the underlying cause, as it guides management decisions and helps to predict the chronicity of the course and duration of care.

The etiology, clinical manifestations, and evaluation of neonatal pleural effusions will be reviewed here. The management of neonatal pleural effusions is discussed separately. (See "Management of pleural effusions in the neonate".)


The pleural space is bounded by the visceral and parietal pleura mesothelial membranes that line the chest wall and lung surface (image 1). The volume of fluid in the pleural space results from a balance of fluid production thought to be made by the visceral pleura, and absorption by the lymphatics of the parietal pleura. A pleural effusion occurs as fluid collects in the pleural space when the production of pleural fluid is in excess of absorption. This can result from overproduction of fluid and/or reduced absorption, depending on the underlying etiology. Pleural anatomy, fluid balance, and accumulation based on data in older patients are discussed separately. Little is known about the dynamics of pleural fluid production and reabsorption in the newborn. (See "Mechanisms of pleural liquid turnover in the normal state" and "Mechanisms of pleural liquid accumulation in disease".)


Data are limited on the incidence of neonatal pleural effusions. A study of neonates admitted to six Portuguese neonatal intensive care units (NICUs) from 1997 to 2004 reported 62 cases of pleural effusions out of 112,000 deliveries resulting in an estimated incidence of 0.06 percent [1]. In an older case series from a single center, pleural effusions were noted in 33 out of 1482 infants admitted to the NICU (2.2 percent) [2].


Numerous disorders have been associated with neonatal pleural effusions. These conditions can be categorized as congenital (fetal) or acquired disorders. In the previously mentioned Portuguese case series, pleural effusions were due to congenital conditions in approximately one-third of the patients (n = 20) (32 percent) and were acquired in the remaining two-thirds (n = 42) [1].

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Mar 07, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Rocha G, Fernandes P, Rocha P, et al. Pleural effusions in the neonate. Acta Paediatr 2006; 95:791.
  2. Long WA, Lawson EE, Harned HS Jr, Kraybill EN. Pleural effusion in the first days of life: a prospective study. Am J Perinatol 1984; 1:190.
  3. van Straaten HL, Gerards LJ, Krediet TG. Chylothorax in the neonatal period. Eur J Pediatr 1993; 152:2.
  4. Caserío S, Gallego C, Martin P, et al. Congenital chylothorax: from foetal life to adolescence. Acta Paediatr 2010; 99:1571.
  5. Kallanagowdar C, Craver RD. Neonatal pleural effusion. Spontaneous chylothorax in a newborn with trisomy 21. Arch Pathol Lab Med 2006; 130:e22.
  6. Paget-Brown A, Kattwinkel J, Rodgers BM, Michalsky MP. The use of octreotide to treat congenital chylothorax. J Pediatr Surg 2006; 41:845.
  7. Schlüter G, Steckel M, Schiffmann H, et al. Prenatal DNA diagnosis of Noonan syndrome in a fetus with massive hygroma colli, pleural effusion and ascites. Prenat Diagn 2005; 25:574.
  8. Prasad R, Singh K, Singh R. Bilateral congenital chylothorax with Noonan syndrome. Indian Pediatr 2002; 39:975.
  9. George-Abraham JK, Zimmerman SL, Hinton RB, et al. Tetrasomy 15q25.2→qter identified with SNP microarray in a patient with multiple anomalies including complex cardiovascular malformation. Am J Med Genet A 2012; 158A:1971.
  10. Smets K. X-linked myotubular myopathy and chylothorax. Neuromuscul Disord 2008; 18:183.
  11. Lo IF, Brewer C, Shannon N, et al. Severe neonatal manifestations of Costello syndrome. J Med Genet 2008; 45:167.
  12. Matsumoto N, Gondo K, Kukita J, et al. A case of galactosialidosis with a homozygous Q49R point mutation. Brain Dev 2008; 30:595.
  13. Bellini C, Boccardo F, Campisi C, Bonioli E. Congenital pulmonary lymphangiectasia. Orphanet J Rare Dis 2006; 1:43.
  14. Dutheil P, Leraillez J, Guillemette J, Wallach D. Generalized lymphangiomatosis with chylothorax and skin lymphangiomas in a neonate. Pediatr Dermatol 1998; 15:296.
  15. Stevenson DA, Pysher TJ, Ward RM, Carey JC. Familial congenital non-immune hydrops, chylothorax, and pulmonary lymphangiectasia. Am J Med Genet A 2006; 140:368.
  16. Chang YL, Lien R, Wang CJ, et al. Congenital chylothorax in three siblings. Am J Obstet Gynecol 2005; 192:2065.
  17. Battin MR, Yan J, Aftimos S, Roberts A. Congenital chylothorax in siblings. BJOG 2000; 107:1516.
  18. Bialkowski A, Poets CF, Franz AR, Erhebungseinheit für seltene pädiatrische Erkrankungen in Deutschland Study Group. Congenital chylothorax: a prospective nationwide epidemiological study in Germany. Arch Dis Child Fetal Neonatal Ed 2015; 100:F169.
  19. Inoue S, Mizobuchi M, Yoshimura N, et al. Successful perinatal management of a very low birthweight infant with congenital complete atrioventricular block. Am J Perinatol 2005; 22:387.
  20. Römer S, Opgen-Rhein B, Chaoui R, et al. Bilateral agenesis of the superior vena cava associated with congenital hydrothorax. Ultrasound Obstet Gynecol 2006; 28:842.
  21. Goujon E, Cordoro KM, Barat M, et al. Congenital plaque-type glomuvenous malformations associated with fetal pleural effusion and ascites. Pediatr Dermatol 2011; 28:528.
  22. Duro EA, Moussou I. Placental chorioangioma as the cause of non-immunologic hydrops fetalis; a case report. Iran J Pediatr 2011; 21:113.
  23. Odaka A, Honda N, Baba K, et al. Pulmonary sequestration. J Pediatr Surg 2006; 41:2096.
  24. Thibeault DW, Zalles C, Wickstrom E. Familial pulmonary lymphatic hypoplasia associated with fetal pleural effusions. J Pediatr 1995; 127:979.
  25. Langlet C, Gaugler C, Castaing M, et al. An uncommon case of disseminated neonatal herpes simplex infection presenting with pneumonia and pleural effusions. Eur J Pediatr 2003; 162:532.
  26. Bennett RE Jr, Barnett DW. Perinatal herpes simplex infection presenting with pneumonia and pleural effusions. Pediatr Infect Dis J 2001; 20:228.
  27. Savarese I, De Carolis MP, Costa S, et al. Atypical manifestations of congenital parvovirus B19 infection. Eur J Pediatr 2008; 167:1463.
  28. Karagöl BS, Okumuş N, Karadağ N, Zenciroğlu A. Isolated congenital pleural effusion in two neonates. Tuberk Toraks 2012; 60:52.
  29. Beigelman A, Marks KA, Landau D. Congenital isolated pleural effusion associated with obstructive uropathy. Isr Med Assoc J 2005; 7:271.
  30. Lee CC, Fang CC, Chou HC, Tsau YK. Urinothorax associated with VURD syndrome. Pediatr Nephrol 2005; 20:543.
  31. Reitter A, Peters J, Wittekindt B, et al. Prenatal management of diaphragmatic rhabdomyosarcoma presenting with fetal hydrops. Ultrasound Obstet Gynecol 2012; 40:235.
  32. Cheng I, Chen YL, Tsai YL, et al. Is Langerhan cell histiocytosis complicated with hydrops fetalis exclusively lethal in premature neonates? Pediatr Dermatol 2011; 28:469.
  33. Wu X, Du L, Wang X. Congenital monoblastic leukemia presenting as jaundice, pleural effusion, and ascites: case report and literature review. Fetal Pediatr Pathol 2011; 30:27.
  34. Odaka A, Takahashi S, Tanimizu T, et al. Chest wall mesenchymal hamartoma associated with a massive fetal pleural effusion: a case report. J Pediatr Surg 2005; 40:e5.
  35. Ozkan H, Gülen H, Demir N, et al. Nonimmune hydrops fetalis and bilateral pulmonary hypoplasia in a newborn infant with nuchal vascular hamartoma. Turk J Pediatr 1997; 39:557.
  36. Gobbi D, Rubino M, Chiandetti L, et al. Neonatal intrapericardial teratoma: a challenge for the pediatric surgeon. J Pediatr Surg 2007; 42:E3.
  37. Law MA, McMahon WS, Hock KM, et al. Balloon Angioplasty for the Treatment of Left Innominate Vein Obstruction Related Chylothorax after Congenital Heart Surgery. Congenit Heart Dis 2015; 10:E155.
  38. Biewer ES, Zürn C, Arnold R, et al. Chylothorax after surgery on congenital heart disease in newborns and infants -risk factors and efficacy of MCT-diet. J Cardiothorac Surg 2010; 5:127.
  39. Beghetti M, La Scala G, Belli D, et al. Etiology and management of pediatric chylothorax. J Pediatr 2000; 136:653.
  40. Mills J, Safavi A, Skarsgard ED, Canadian Pediatric Surgery Network (CAPSNet). Chylothorax after congenital diaphragmatic hernia repair: a population-based study. J Pediatr Surg 2012; 47:842.
  41. Kamiyama M, Usui N, Tani G, et al. Postoperative chylothorax in congenital diaphragmatic hernia. Eur J Pediatr Surg 2010; 20:391.
  42. Pabalan MJ, Wynn RJ, Reynolds AM, et al. Pleural effusion with parenteral nutrition solution: an unusual complication of an "appropriately" placed umbilical venous catheter. Am J Perinatol 2007; 24:581.
  43. Bitar FF, Obeid M, Dabbous I, et al. Acute respiratory distress associated with external jugular vein catheterization in the newborn. Pediatr Pulmonol 2003; 36:549.
  44. Been JV, Degraeuwe PL. Pleural effusion due to intra-abdominal extravasation of parenteral nutrition. Pediatr Pulmonol 2008; 43:1033.
  45. Pignotti MS, Messeri A, Donzelli G. Thoracentesis in pericardial and pleural effusion caused by central venous catheterization: a less invasive neonatal approach. Paediatr Anaesth 2004; 14:349.
  46. Madhavi P, Jameson R, Robinson MJ. Unilateral pleural effusion complicating central venous catheterisation. Arch Dis Child Fetal Neonatal Ed 2000; 82:F248.
  47. Dhande V, Kattwinkel J, Alford B. Recurrent bilateral pleural effusions secondary to superior vena cava obstruction as a complication of central venous catheterization. Pediatrics 1983; 72:109.
  48. Hsu HF, Chou YH, Wang CR, Wu SC. Catheter-related superior vena cava syndrome complicated by chylothorax in a premature infant. Chang Gung Med J 2003; 26:782.
  49. Oppermann HC, Wille L. Hemothorax in the newborn. Pediatr Radiol 1980; 9:129.