Management of Chylothorax : A Literature Review

Aris Furqon, M. Arza Putra2, Pribadi W. Busro2

1
Resident of Cardiac, Thoracic and Vascular Surgery Division, Department of Surgery
Cipto Mangunkusumo Hospital—Faculty of Medicine, Universitas Indonesia
2
Cardiac, Thoracic and Vascular Surgery Division, Department of Surgery
Cipto Mangunkusumo Hospital—Faculty of Medicine, Universitas Indonesia

Chylothorax is usually caused by disruption or obstruction of the thoracic duct or it’s tributaries that
lead to leakage of chyle (lymphatic fluid of intestinal origin) into the pleural cavity. The leakage of
chyle from abnormal lymphoid masses and lymphoid malformations and the flow of chylousascites
into the pleural space may also cause chylothorax. The liquid typically has a high triglyceride content
and often a cloudy or milky white appearance. In contrast, cholesterol pleural effusion, which may
also have a milky appearance, has a high concentration of cholesterol and lecithin globulin
complexes that accumulate due to pleural inflammation or infection. 1

Anatomy of the thoracic duct

Figure 01. Anatomy of thoracic dut in orientation to aorta.

Cited from: Fortin D, Inculet RI, Malthaner RA. The Thoracic duct
and chylothorax. In: Pearson’s thoracic and esophageal surgery.
Patterson GA, Cooper JD, Deslauriers J. Et al. Philadelphia:
Elsevier; 2008

The thoracic duct is the left main collecting vessel of the lymphatic system and is much larger than
the right terminal lymphatic gland (see Figure 01). The duct originate from the cisterna chyli in the
abdomen, but may be absent in 1 out of 50 people. The Cisterna chyli is a spherical structure that is
3 to 4 cm long and 2 to 3 cm in diameter. It is located along the spine at the level of L2, but it can be
found anywhere between T10 and L3 on the right side of the aorta. From the cisterna chyli, the
thoracic canal ascends along the spine to enter the thorax through the aortic hiatus at T10 to T12,
just to the right of the aorta. It rises extrapleurally along the right anterior surface of the vertebral
bodies, behind the esophagus, between the aorta and the azygos vein and anterior to the right
intercostal arteries.2
At the level of T5 to T7, the duct crosses the aorta to the left posterior aspect of the mediastinum
and ascends on the left side of the esophagus under the pleural reflection and behind the left
clavicle artery. In this region the duct is susceptible to damage due to surgery with the aortic arch,
the left clavicle or the esophagus. 4 cm above the clavicle the duct rotates laterally behind the
carotid sheath and the jugular vein in front of the lower thyroid and the vertebral artery, the
subclavian artery and the phrenic nerve. At the medial margin of the scalenus anterior muscle, it
turns cranially and enters the venous system at the left subclavian internal jugular vein, although it is
evacuated into the left innominate, the left internal jugular, the left spine, or even the right can
internal jugular vein. Over its course, the canal contains a variable number of valves with a
continuous bicuspid valve at the lympho-venous junction, protecting it from the reflux of the blood.

Variability is common, with 40% to 60% of individuals having abnormal collaterals communicating
with the azygos, intercostal and lumbar veins. It has also been found that 25% to 33% of individuals
have multiple ducts at the level of the membrane which has an impact if an operation is
considered.There are some variation of thoracic duct drainage into jugulosubclavian confluence. The
scematic anatomy to the variance is described at figure 02. 2

Figur 02. Variation of jugulosubclavian confluence.

Cited from: Lyon S, Mott N, Koukounaras J.
Shoobridge J, Hudson PV. Role of interventional
radiology in the management of chylothorax: a
review of current management of high output
chylothorax. Cardiovasc intervent Radiol (2013) 36:
599-607.

The right duct is small (2 cm long) and is rarely able to be visualized. It drains lymph from the right
side of the head, neck, and chest wall through the jugular trunk and from the right lung, heart and
lower half of the left lung through the bronchomediastinal trunk. The lymph from the hepatic dome,
the right diaphragm, and the right upper thoracic drainage penetrate through the right internal
mammary trunk into the right atrium.

The main function of thoracic duct is the transport of digestive fat to the venous system. Fatty acids
with small molecule of less than 10 carbon atoms are absorbed directly into the portal vein system,
while larger lipids are incorporated as micelles into the intestinal lymphatic vessels. The transport
time of the absorbed fat from the mouth to venous blood is less than 1 hour after ingestion and peak
absorption is 6 hours after ingestion. The volume of lymphatic flow is estimated to be 1.38 ml/kg of
body weight per hour. Volume of up to 2500 ml chyle in 24 hours were collected from the
cannulated human thoracic duct. Lymphatic flow in the thoracic canal ranges from 0.38 ml/min at
rest to 3.9 ml/min after a meal or during abdominal massage. It has been found that 95% of the
volume comes from the liver and intestinal lymph channels, although the amount of the extremities
is negligible. The lymphatic flow in amphibians, reptiles and some birds is driven by the lymph nodes,
while mammals have more complex mechanisms. The forward flow of chyle from the abdomen in
humans is influenced by four factors

1. Vis a tergo from latins which means force excerted from back. It means that lymph flow is
proportional to force from the back which push forward the lymphatic fluid. In thoracic duct
it means the chyle will increase if the pressure in thoracic duct increases due to it’s
production from guts. That’s why chyle production will increase from faty meal.
2. Negative intrathoracic pressure and positive intraabdominal pressure create gradient for
lymph to flow into systemic cyrculation.
3. Muscular contraction of the duct and valves are important for lymph movement. The
contraction occur every 10 to 15 second. Acethyl coline from vagal nerve will contract the
duct and epinephrine will dilate it. Normal range of thoracic duct pressure are 10 to 25
cmH2O and can rise to 50 cm H2O if obstruction occur.
4. The bernoulli effect from blood which flow passing the lymphatic venous junction will create
vacum wich will suck the lymph from thoracic duct.

The lymphatic vessels perform the vital functions of collecting and transporting tissue fluid,
extravasated plasma proteins, absorbed lipids, and other large molecules from the interstitial space
into the intravascular space. Most of the body's lymphocytes circulate through the duct.

Diagnose

Based on it’s own definition, the gold standard for diagnosing chylothorax is detection of
chylomicron on pleural fuid which acumulate inside pleural space. The characteristic and
composition of pleural fluid is sumarized at figure 03.

A pleural fluid triglyceride concentration greater than 110 mg / dl (1.24 mmol / l) strongly supports
the diagnosis of chylothorax, but triglyceride concentrations may be less than 110 mg / dl in 15% of
patients and less than 50 mg / dl in 3 Percent. A pleural fluid triglyceride concentration of less than
50 mg / dL provides strong support that the patient does not have chylothorax. If the triglyceride
level is less than 110 mg / dL (less than 1.24 mmol / L) but the diagnosis is clinically suspected, the
presence of chylomicrons in the pleural fluid by lipoprotein electrophoresis may confirm the
presence of chylothorax.1,2,3
 Figure 03. characteristic and composition of pleural fluid of chylothorax. Cited from: Fortin D,
Inculet RI, Malthaner RA. The Thoracic duct and chylothorax. In: Pearson’s thoracic and esophageal
surgery. Patterson GA, Cooper JD, Deslauriers J. Et al. Philadelphia: Elsevier; 2008

Etiology

Etiology for chylothorax generaly divided into two categories traumatic and non-traumatic. One
single center case series report has described their experience for 21 years. Their report consist of
203 patient. About 50 percent are traumatic and 50 percent nontraumatic. From nontraumatic
group 6 patients are diagnosed as idiophatic chylothorax. This result differ from another older report
which describe 72% of their chylothorax patient are non traumatic. It seems like the etiology of
chylothorax varies from one center to another based on their population. 3

Malignancy is the most common cause of chylothorax on non traumatic group. Lymphoma, chronic
lymphocytic leukimia, and metastati cancer have been noted to be associated to chylothorax. 3

Idiopatic chylothorax range from 5 to 10% of chylothorax. 3

Rare cause of chylothorax are castleman’s disease, kaposiform lymphangiomatosis, generalized

lympphatic anomaly, sarcoidosis, tuberculosis, lymphangioleimiomatosis (LAM), Noonan Syndrome,
etc.3

Traumatic chylothorax happen mainly from surgical intervention such as esophagectomy, pulmonary
resection with lymph node dissection, congenital heart disease (including heart transplant). Those
surgery posses the greatest risk for chylothorax but actuallu all thoracic surgery have been reported
could cause chylothorax.3

Management

The first step in the treatment of chylothorax is prevention. A possible thoracic duct injury must be
detected or anticipated intraoperatively. The ductal ligation at the aortic hiatus can be easily
performed at the time of oesophageal or thoracic aortic dissection. If extensive lymphadenectomy or
posterior mediastinal lymph node dissection is performed, routine thoracic ligation must be
considered. Another way is to give milk via nasogastric tube too increase visualization of thoraci
duct.2

If a chylothorax is detected postoperatively or is simply not associated with a surgical procedure, the
diagnosis must be timely and treated promptly. Untreated chylothorax can lead to impaired
breathing, dehydration, malnutrition and immunodeficiency. Impaired breathing happen since chyle
leak cause ventalation reduction and cause ventilation fo perfusion ratio. Dehidration and
malnutriotion happen since chyle is basically transporter for fat and other macromolecule. If it
drained outside body constantly it of course cause malnutrition and dehydration. Chyle fluid also
contains many lymphocyte. Almost 90% of the are T lymphocyte which react differently to different
antigen.1

A variety of modalities have been described for the treatment and are divided into two general
categories: conservative (non-surgical) and surgical. In most cases, a combination of modalities is
used to treat five major principles of therapy: support for respiratory function, re-expansion of the
lung and obliteration of the pleural space, prevention of dehydration and malnutrition, reduction of
chyle production and, in certain cases, treatment of the underlying cause. 1,2

Conservatife treatment

Most cases are initially treated conservatively. Overall, up to 50% of patients have an answer.
Conservative treatments are often used as an aid to surgical treatment.

Drainage, re-expansion of the lungs and obliteration of the pleural space is the basic treatment of
any pleural effusion. The drainage can be achieved by thoracentesis, thoracostomy or
pleuroperitoneal shunt. Thoracocentesis often needs to be repeated, rarely achieves complete
pleural evacuation, and can result in loculated effusions, hematothorax, pneumonia, or empyema.
 Figure 01. Anatomy of thoracic dut in orientation to aorta. Cited from: Fortin D, Inculet RI,
Malthaner RA. The Thoracic duct and chylothorax. In: Pearson’s thoracic and esophageal surgery.
Patterson GA, Cooper JD, Deslauriers J. Et al. Philadelphia: Elsevier; 2008

The pleuroperitoneal shunt is generally regarded as a conservative treatment in patients who would
not tolerate a more invasive surgical procedure. A pleuroperitoneal shunt with the Denver dual-
valve shunt has been reported in high-risk patients. Success rates of 75% to 90% have been reported
in pediatric chylothorax. The shunt is relatively easy to place and deflates the pleural space. It can
also reduce the nutrient, fluid and cell losses observed with external drainage. Patient compliance is
required to periodically compress the pump chamber. Fibrin shunt occlusion, which requires shunt
replacement, occurs in 10% of cases. Chylosis ascites does not appear to be a significant problem,
but its presence remains a contraindication to shunt placement. 2

Pleurodesis is a medical procedure to “adhese” or adhere visceral pleura to parietal pleura. Chemical
pleurodesis via the thoracostomy tube with talc, nitrogen mustard, or bleomycin have all been
described to help with obliteration of the pleural cavity. Although pleurodesis is often unsuccessful,
it is usually added to other conservative modalities in high risk patients where surgery is not an
option. For the surgical candidate, chemical pleurodesis may also be used after the ligature of the
thoracic duct.2
Treatment to support adequate respiratory function can not be stressed enough. Many patients with
chylothorax already have a precarious respiratory status, which is induced by their underlying
pathology (eg, lymphoma, chest trauma) or by their postoperative condition (eg, sternotomy,
thoracotomy, high abdominal incision). Another compromise with accumulation of chylic fluid in the
pleural space with secondary atelectasis can trigger acute respiratory failure. Re-expansion of the
lungs by drainage of the pleural space and aggressive chest physiotherapy are the main modalities
should be done. If these treatment fail, the support must be provided by mechanical ventilation,
which, despite the associated complications, can reduce the chylous leakage by increase intra-
thoracal pressure.2

Key aspects of chylothorax management include the prevention of dehydration, the maintenance of
adequate nutrition, and the correction of fluid and electrolyte imbalances. See figure 03, the content
of chyle fluid is prety much nutrient from digestion system so, if it get leaked to external drainage
system such as tube thoracostomy the risk for developing mal-nutrition is imminent. Fluid leakage
through the thoracostomy tube must be properly monitored and volume replacement aggressively
initiated to prevent hypovolemia.2

To avoid mal nutrition, the intravenous injection of chylus was used in the 1930s and then
abandoned in the 1940s since it cause anaphylactic reactions. Chyle was successfully reinfused using
a 40 μm blood filter and a volumetric pump. A small test dose was recommended to avoid
anaphylaxis. Although attractive, this treatment has not gained in popularity, most likely because of
its technical complexity and potential risk of infection by manipulation. 2

Maintaining nutrition is usually done with the help of MCT diet or total parenteral nutrition (TPN).
Low-enteral formulas supplemented with MCT have been recommended but rarely work. Long-chain
fatty acids (> 10 carbon atoms) undergo a second esterification and enter the lymph as
chylomicrons.3 However, medium-chain fatty acids pass directly into the albumin-bound portal vein.
Despite this preferential uptake of MCT directly into the portal circulation, intestinal triglycerides are
recovered from both endogenous and exogenous sources. Eighty percent of chylotriglycerides have
been found to be non-MCT despite the MCT diet. Although the MCT diet safely maintains nutrition,
chylus production is not completely abolished. Any oral intake actually increases the production of
chylus. Therefore, TPN and NPO are the most effective methods for reducing the production of
chyles and supporting nutrition. 1,2

Figure 05. Fat Absorption from gut lumen to lymph vessel. Cited from: Costanzo LS.
Physiology. 4th ed. Philadelphia: Saunders Elsevier; 2010.
The most effective way to reduce chyles is to completely avoid oral ingestion. More recently,
additional conservative measures have been used in an attempt to reduce chylus release. The use of
somatostatin, octreotide, etilefrine, mechanical ventilation with positive and end-expiratory
pressure (PEEP) and percutaneous embolization of the chest tube has been described. 2

Etilefrin, a sympathomimetic drug used to treat postural hypotension, was described in a case report
in addition to conservative therapy. The postulated mechanism for the reduction of the flow of
chylus is the contraction of the smooth muscle of the Thoracic duct. 2

The use of percutaneous transabdominal catheterization of the cisterna chyli to embolize the
thoracic duct was first described in 1998 and subsequently reported by others.Under local
anesthesia and sedation, unipedal or bipedal lymphography is performed with iodinated oil to
opacify the cisterna chyli. With the use of a percutaneous approach through the anterior abdominal
wall, the cisterna chyli or other large (2-3 mm) retroperitoneal lymphatics are cannulated under
fluoroscopic guidance and then embolized. A success rate of 70% was achieved in 42 patients with
unresolving chylothorax, with no complications related to the technique. 4

For this procedure identification of anatomic of lacteal vesel anatomy is important. It could be done
by injecting specific dye subcutaneously at foot. The lymph vesel should be visible after 10 to 30
minutes. The do the cut down of lymph vessel and direct canulation of lymphatic vessel using a
dedicated lymphatic needle (30G). After cannulation lymphatic system could be opacified for
fluoroscopy by injeting lipidol. Non-oil based caontrast should be avoided since it tends to remain in
a column and dilute at some point. It also has sclerosant and occlusive effect. It can succesfully treat
high output chylothorax in 50-75% patient. 4

If cysterna chyli is present in 40% of patient it could be accesed for anterograde intervention with
embolic agent to occlude the chisterna chyli via anterior approach. If it not present direct acces to
thoracic duct shoudl be done via transabdominal approach like cisternal chyli acces or retrograde
approach.4
 Figure 06. Lymphangiogram and fluoroscop guided
antegrade acces to the cisterna chyli via
percutaneous approach. Cited from: Lyon S, Mott N,
Koukounaras J. Shoobridge J, Hudson PV. Role of
interventional radiology in the management of
chylothorax: a review of current management of
high output chylothorax. Cardiovasc intervent Radiol
(2013) 36: 599-607.

In chylothorax not related to trauma or surgery(iatrognic), the cause must be determined and The
underlying disease should be effectively treated. For lymphomas or other malignancies, radiation,
chemotherapy, or both often forms an integral part of the treatment. Combination with other
modalities (e.g., TPN, NPO, surgical ligation, pleurodesis) is often needed to achieve control.

Surgical management

The first issue for surgical management is timming of the surgery. Since then, conservative
management should be done before more infusive treatment. The long lasting the conservative
treatment done before invasive treatment used. 1,2

There is no consensus about duration of conservative therapy should last. Generally it should be wait
for fourteen days at mosst because between 25% and 50% of leaks close spontaneously during this
interval, and the remainder require surgical intervention. A shorter course of nonsurgical
management is recommended, especially in neonates and in debilitated patients who are severely
compromised by the lymphocyte, antibody, and protein loss resulting from an active thoracic duct
fistula.1,2

Dugue et al, reviewed their experience with postesophagectomy chylothorax and identify factors
which could predict success or failure of conservative therapy. A chylous output of less than 10
mL/kg on the fifth day after the onset of leakage reliably predict success of nonoperative treatment
(sensitivity, 86%); a higher output was seen in all patients who ultimately required reoperation
(specificity, 100%). Other author suggested early operative therapy, after 2 days if the volume of
drainage is more than 2 L/24 hr at that time, because of high rate of failure with conservative
management in those population.1,2

Figure 07. supradiaphragmatic mass ligation

of thoracic duct. Cited from: Fortin D, Inculet RI,
Malthaner RA. The Thoracic duct and chylothorax.
In: Pearson’s thoracic and esophageal surgery.
Patterson GA, Cooper JD, Deslauriers J. Et al.
Philadelphia: Elsevier; 2008

Surgical intervention basicallya have two important principle which is reduce the chylous leak and
providing good lung expansion and obliteration of pleural space. Open approach is generally
prefered than VATS since thoracic duct is hardly visible. Direct ligation of the thoracic duct,
supradiaphragmatic mass ligation of the thoracic duct, infradiaphragmatic mass ligation,
anastomosis of the duct to the azygos vein, and fibrin glue application have all been performed in an
attempt to stop chylous leaks. Lung decortication, pleurectomy, chemical pleurodesis, and
pleuroperitoneal shunting have been described to improve lung expansion, obliterate the pleural
space, and hopefully control the leak. These techniques are typically used in addition to direct
control of the duct, or on their own if the site of leakage is not found. Almost all of them can be
performed with the use of a video-assisted thoracoscopy (VATS) approach. 1,2

Localization of thoracic duct leak could be done by lymphangiogram or direct visualization at

operation.but general opinion agree it’s not necesary to do lymphangiogram preoepratively and
reserve it for surgical failure. Tough a center stated that their standard operating procedure is to do
lymphangiogram as treatment before using surgical treatment. 1,2

To increase visibility of thoracic duct and it’s leaking site. Administration of faty meal can be done.
Faty meal could be olive oil, milk, or cream. Dye such as sudan black could be add to fatty meal to
increase visibility even more. The administration of meal should be done 2 – 3 hours before surgery
via nasogastric tube.1,2

If lung is traped by fibrin deposit it need decortication to re-expand it. Some author recommend
using pleurectomy for pleurodesis methods but some other argue that it could damage intercostal
lymph vessel. Use of chemical pleurodesis is justified for that reason. 1,2

Minimal invasive technique has grown rapidly.Now, VATS could be used to administer fibrin glue,
duct cliping, chemical pleurodesis admisnistration. 2
Succes of surgical intervention vary between 60 to 100% depend on the cause of the chylothorax.
Even if duct cannot be identified at time of surgery mass ligation could achieve 80% succes rate. 2

Conclusion

Chylothorax is a rare condition in which lymphatic fluid accumulate in pleural space caused by leak
or obstruction at thoracic duct. It generally divided into two main categories traumatic and non-
traumatic.

Treatment should be done by conservative methods first. Chylothorax patient should be monitored
carefully for dehidration, electrolite imbalance, and malnutrition.

Surgical treatment should be done if conservative treatment fail.

Reference

1. Heffner JE, Broaddus VC, Finlay G. Management of Chylothorax. UpToDate.

https://www.uptodate.com/contents/management-of-chylothorax (accesed at: 7 februari
2018 9:17)
2. Fortin D, Inculet RI, Malthaner RA. The Thoracic duct and chylothorax. In: Pearson’s thoracic
and esophageal surgery. Patterson GA, Cooper JD, Deslauriers J. Et al. Philadelphia: Elsevier;
2008
3. Heffner JE, Broaddus VC, Finlay G. Etiology, clinical presentation, and diagnosis of
chylothorax. UpToDate. https://www.uptodate.com/contents/etiology-clinical-
presentation-and-diagnosis (accesed at: 7 februari 2018 9:38)
4. Costanzo LS. Physiology. 4th ed. Philadelphia: Saunders Elsevier; 2010.
5. Lyon S, Mott N, Koukounaras J. Shoobridge J, Hudson PV. Role of interventional radiology in
the management of chylothorax: a review of current management of high output
chylothorax. Cardiovasc intervent Radiol (2013) 36: 599-607.