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Chylothorax

  • Author: Sasha D Adams, MD; Chief Editor: BS Anand, MD  more...
 
Updated: Mar 11, 2016
 

Background

Chylothorax refers to the presence of lymphatic fluid in the pleural space secondary to leakage from the thoracic duct or one of its main tributaries. In 1875, H. Quinke described the first traumatic chylothorax. In 1948, R.S. Lampson performed the first thoracic duct ligation.

Chylothorax has no predilection for age or either sex. The prevalence of chylothorax after various cardiothoracic surgeries is 0.2-1%.

See the images below.

Anteroposterior upright chest radiograph shows a m Anteroposterior upright chest radiograph shows a massive left-sided pleural effusion with contralateral mediastinal shift. Image courtesy of Allen R. Thomas, MD.
A CT scan of the chest of a 3-year old child showi A CT scan of the chest of a 3-year old child showing left side effusion and underlying parenchymal infiltrate and atelectasis. Image courtesy of Ibrahim Abdulhamid, MD.
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Etiopathophysiology

A tear or leak in the thoracic duct causes chylous fluid to collect in the pleural cavity, which can cause acute or chronic alterations in the pulmonary mechanics. In a normal adult, the thoracic duct transports up to 4 L of chyle per day, allowing a rapid and large accumulation of fluid in the chest.

Nontraumatic

Malignant etiologies account for more than 50% of chylothorax diagnoses and are separated into lymphomatous and nonlymphomatous. Lymphoma is the most common cause, representing about 60% of all cases, with non-Hodgkin lymphoma more likely than Hodgkin lymphoma to cause a chylothorax. By comparison, nonlymphomatous causes are rare.

Nonmalignant etiologies are separated into idiopathic, congenital, and miscellaneous. Clinicians must rule out all possible malignant causes before designating the chylothorax as idiopathic. Congenital chylothorax is the leading cause of pleural effusion in neonates.[1]  Miscellaneous causes include cirrhosis, tuberculosis, sarcoidosis, amyloidosis, and filariasis.

Traumatic

Trauma is the second leading cause of chylothorax (25%).

Iatrogenic injury to the thoracic duct has been reported with most thoracic procedures. In particular, cardiothoracic surgery has been associated with 69-85% of cases of chylothorax in children.[2]

Milonakis et al examined their experience in managing chylothorax following congenital heart surgery. Of the 1341 children who underwent correction of congenital heart disease, 18 (1.3%) developed postoperative chylothorax, which was managed with a therapeutic protocol that included complete drainage of chyle collection and controlled nutrition. Six children received adjunctive somatostatin. When lymph leakage persisted (range, 2.5-14.7 mL/kg/d for 8-42 days) despite conservative management, surgical intervention was implemented. Once chylothorax resolved, a 6-week diet of medium-chain triglycerides was given.

No deaths occurred. Conservative therapy was effective in 15 patients (83.3%); 3 patients with persistent drainage required thoracotomy with pleurodesis to achieve resolution, 2 of whom had not had an effective response with previously attempted chemical pleurodesis with doxycycline (range of duration leakage, 5.1-7.4 mL/kg/d for 15-47 days).

In a retrospective study involving 392 pediatric patients (mean age, 97 days) who underwent surgical treatment of congenital heart disease to determine whether the site of insertion of central venous lines was associated with the occurrence of chylothorax after cardiac surgery, Borasino et al reported that the insertion of central venous lines in the upper body has an increased association with postosurgical chylothorax.[3]  Overall, 62 of 392 patients (15.8%) developed postsurgical chylothorax; affected patients more frequently had central venous line sites in the upper body (P = .03), had higher RACHS-1 scores (risk assessment for congenital heart surgery) (P = .03), had longer bypass times (P = .02); and had longer cross-clamp times (P = .03). The investigators indicated that by avoiding the use of central venous lines in the internal jugular and subclavian veins, the incidence of chylothorax may be reduced in this setting.[3]

Nonsurgical traumatic injury is a rare cause, usually secondary to penetrating trauma.

Pseudochylothorax: Chylothorax must be distinguished from pseudochylothorax, or cholesterol pleurisy, which results from accumulation of cholesterol crystals in a chronic existing effusion. The most common cause of pseudochylothorax is chronic rheumatoid pleurisy, followed by tuberculosis and poorly treated empyema.

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Contributor Information and Disclosures
Author

Sasha D Adams, MD Assistant Professor, Department of Surgery, Division of Trauma, Critical Care and Emergency General Surgery, University of North Carolina, Chapel Hill; Surgeon, Trauma, Critical Care and Emergency General Surgery, University of North Carolina Memorial Hospital

Sasha D Adams, MD is a member of the following medical societies: American College of Surgeons, Association for Academic Surgery, Eastern Association for the Surgery of Trauma, Association of Women Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

James Cipolla, MD Attending Surgeon, Department of Traumatology and Critical Care, Program Director, Surgical Critical Care Fellowship, St Luke's University Hospital; Associate Professor of Surgery, Temple University School of Medicine; Assistant Clinical Professor of Surgery, University of Pennsylvania School of Medicine

James Cipolla, MD is a member of the following medical societies: American College of Surgeons, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

BS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Acknowledgements

Mounzer Al Samman, MD Assistant Professor, Department of Internal Medicine, Division of Gastroenterology, Texas Tech University School of Medicine

Mounzer Al Al Samman, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, and American Gastroenterological Association

Disclosure: Nothing to disclose. Rosemary Kozar, MD, PhD, Assistant Professor, Department of Surgery, Division of General Surgery, University of Texas at Houston School of Medicine

Disclosure: Nothing to disclose.

References
  1. Ergaz Z, Bar-Oz B, Yatsiv I, Arad I. Congenital chylothorax: clinical course and prognostic significance. Pediatr Pulmonol. 2009 Aug. 44(8):806-11. [Medline].

  2. Milonakis M, Chatzis AC, Giannopoulos NM, Contrafouris C, Bobos D, Kirvassilis GV, et al. Etiology and management of chylothorax following pediatric heart surgery. J Card Surg. 2009 Jul-Aug. 24(4):369-73. [Medline].

  3. Borasino S, Diaz F, Masri KE, Dabal RJ, Alten JA. Central venous lines are a risk factor for chylothorax in infants after cardiac surgery. World J Pediatr Congenit Heart Surg. 2014 Oct. 5(4):522-6. [Medline].

  4. Bender B, Murthy V, Chamberlain RS. The changing management of chylothorax in the modern era. Eur J Cardiothorac Surg. 2016 Jan. 49 (1):18-24. [Medline].

  5. Pini Prato A, Bava GL, Dalmonte P, et al. 16 years of experience with persistent chylothorax in children. Minerva Pediatr. 2015 Dec 17. [Medline].

  6. Shah D, Sinn JK. Octreotide as therapeutic option for congenital idiopathic chylothorax: a case series. Acta Paediatr. 2011 Nov 16. [Medline].

  7. Foo NH, Hwang YS, Lin CC, Tsai WH. Congenital chylothorax in a late preterm infant and successful treatment with octreotide. Pediatr Neonatol. 2011 Oct. 52(5):297-301. [Medline].

  8. Horvers M, Mooij CF, Antonius TA. Is Octreotide Treatment Useful in Patients with Congenital Chylothorax?. Neonatology. 2011 Nov 10. 101(3):225-231. [Medline].

  9. Paul S, Altorki NK, Port JL, Stiles BM, Lee PC. Surgical management of chylothorax. Thorac Cardiovasc Surg. 2009 Jun. 57(4):226-8. [Medline].

  10. Panthongviriyakul C, Bines JE. Post-operative chylothorax in children: an evidence-based management algorithm. J Paediatr Child Health. 2008 Dec. 44(12):716-21. [Medline].

  11. Callari C, Perretta S, Diana M, Dagostino J, Dallemagne B, Marescaux J. Thoracoscopic management of chylothorax after esophagectomy. Surg Endosc. 2011 Nov 15. [Medline].

  12. Nath DS, Savla J, Khemani RG, Nussbaum DP, Greene CL, Wells WJ. Thoracic duct ligation for persistent chylothorax after pediatric cardiothoracic surgery. Ann Thorac Surg. 2009 Jul. 88(1):246-51; discussion 251-2. [Medline].

  13. Guo W, Zhao YP, Jiang YG, Niu HJ, Liu XH, Ma Z, et al. Prevention of postoperative chylothorax with thoracic duct ligation during video-assisted thoracoscopic esophagectomy for cancer. Surg Endosc. 2011 Nov 2. [Medline].

  14. Clark ME, Woo RK, Johnson SM. Thoracoscopic pleural clipping for the management of congenital chylothorax. Pediatr Surg Int. 2015 Dec. 31 (12):1133-7. [Medline].

 
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Anteroposterior upright chest radiograph shows a massive left-sided pleural effusion with contralateral mediastinal shift. Image courtesy of Allen R. Thomas, MD.
A CT scan of the chest of a 3-year old child showing left side effusion and underlying parenchymal infiltrate and atelectasis. Image courtesy of Ibrahim Abdulhamid, MD.
 
 
 
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