Introduction
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.
Pathophysiology
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.
Frequency
International
The prevalence after various cardiothoracic surgeries is 0.2-1%.
Mortality/Morbidity
Mortality and morbidity rates are approximately 10% in major clinical medical centers.
Sex
Chylothorax has no predilection for either sex.
Age
Chylothorax has no predilection for age.
Clinical
History
- Usually, the patient remains asymptomatic until a large amount of chyle accumulates in the pleural space.
- The average latent period between the insult and the onset of symptoms is 7-10 days. Symptoms include the following:
- Dyspnea
- Tachypnea
- Classic symptoms of pleural effusion
- Rarely, patients may experience a rapid accumulation of fluid in the pleural space, causing a tension chylothorax. This is of particular concern following a pneumonectomy. These patients experience a rapid hemodynamic and respiratory compromise, similar to classic tension pneumothorax.
Physical
- Findings on examination are nonspecific and include the following:
- Decreased breath sounds
- Shifting dullness
- If the patient has an existing chest tube, excess drainage of 400-600 cc per 8-hour period is concerning for a chylous leak, particularly in postsurgical patients.
Causes
- 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.
- 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.
- 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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Overview: Chylothorax |
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References
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Paes ML, Powell H. Chylothorax: an update. Br J Hosp Med. May 4-17 1994;51(9):482-90. [Medline].
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Rice TW, Milstone AP. Chylothorax as a result of chronic lymphocytic leukemia: case report and review of the literature. South Med J. Mar 2004;97(3):291-4. [Medline].
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Further Reading
Keywords
thoracic duct leak, pleural chyle, pleural effusion, lymphoma, thoracic duct ligation, pleuroperitoneal shunt, pleurodesis, pleurectomy, chyle, pleural space, postesophagectomy chylothorax, thoracentesis, lymphatic fluid, thoracic duct injury, loculated chylothorax
Overview: Chylothorax