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Review Article

A Contemporary Review of Chylothorax


Arunabh Talwar1 and Hans J. Lee1

Department of Medicine, Division of Pulmonary and Critical Care Medicine, North Shore University Hospital1,
Manhasset, New York and Department of Medicine, NYU School of Medicine1, New York, USA

ABSTRACT

Objectives. This review will focus on anatomical and aetiologic factors as well as the conservative and operative therapy
of chylothorax.
Data Source. A Pubmed search for studies pertaining to the aetiology and/or treatment published in the English language
from 1960 to 2007.
Study Selection. Studies presenting case reports, series, observational and/or retrospective studies, and those with unique
issues pertaining to chylothorax were reviewed independantly by both authors. Studies that were selected by both authors
contain most clinically relevant data.
Results. Chylothorax is caused by injury or obstruction of the thoracic duct or its main tributaries leading to chyle
accumulation in the pleural space. It most commonly occurs from trauma or malignancy, but other causes have been
described. Although chylous effusions are rare, they have serious clinical consequences including cachexia and
immunodeficnecy. There are no evidence-based guidelines to assist in the management of this disease.
Conclusions. A prompt diagnosis is needed to start treatment of the underlying cause. Treatment can be divided into
conservative and surgical interventions. There are no evidence-based guidelines to assist in the management of this disease.
Initial conservative therapy includes intercostal decompression of the pleural effusion along with nutritional support in the
form of total parenteral nutrition, and reduction of chylous formation with somatostatin. Surgical interventions include tho-
racic duct ligation, pleuroperitoneal shunt and percutaneous embolisation. [Indian J Chest Dis Allied Sci 2008; 50: 343-351]

Key words: Chyle, Thorax, Pleural effusion, Injury, Lymph, Treatment

INTRODUCTION cisterna chyli and then through the thoracic duct to


eventually empty into the venous system. The thoracic
duct begins at the cisterna chyli near the T-12 vertebra
Chyle in the pleural space was first described by and ascends through the aortic hiatus of the
Bartolet in 1633 and since its initial description, diaphragm on the anterior surface of the vertebral
numerous causes have been well described. 1 The body between the aorta and the azygos vein into the
pleural fluid in chylothorax consists of chylomicrons posterior mediastinum. At the level of the fifth tho-
and very low-density lipoproteins. It results from an racic vertebra, it then crosses to the left of the vertebral
anatomical disruption of the thoracic duct and/or a column and ascends behind the aortic arch to the left
major lymphatic contributary. It was not until 1948 that of the subclavian artery adjacent to the mediastinal
Lampson 2 reported the first successful treatment of pleura. At the level of the transverse process of the
chylothorax by supradiaphragmatic ligation of the seventh cervical vertebra, the duct turns laterally and
thoracic duct. Although cylothorax accounts for a small runs anterior to the vertebral and thyrocervical
proportion of clinical pleural effusions, prompt arteries and the sympathetic trunk. Passing behind
recognition is needed to avoid malnutrition, immuno- the carotid sheath, it descends anterior to the origin of
deficency, and fibrothorax. Management requires the the left subclavian artery and terminates near the
physician to individualise the work-up, partly because junction of internal jugular and subclavian veins. A
of diversity in the pathogenesis and anatomy. bicuspid valve at the lymphovenous junction pre-
vents the reflux of blood into the duct. The duct itself
has numerous valves throughout its length. However,
ANATOMY in up to 50% of individuals, the route of thoracic duct
is anomalous and unpredictable, thus making it
Chyle passes from the intestinal lymphatics to more susceptible to damage during surgical
[Received: August 14, 2007; accepted: November 15, 2007]
Correspondence and reprint requests: Dr Arunabh Talwar, Department of Medicine, Division of Pulmonary and Critical
Care Medicine, North Shore University Hospital, Manhasset, NY 11030, USA; Phone: 516-465-5400; Fax: 516-465-5454; E-mail:
arunabh@nshs.edu
344 Chylothorax A. Talwar and H.J. Lee

procedures.3 This duct carries lymph from the entire thoracic duct, that causes an increase in intraductal
body, except from the right side of the head, neck, pressure. This increased pressure promotes the
chest, both lungs, and the right upper extremity that formation of dilated collateral channels that eventu-
empties into the right lymphatic duct. There are ally drain into the pleural space. The most common
extensive anastomotic vessels present between cause of chylothorax is neoplasm, which is
various lymphatics, and numerous lymphaticoven- responsible for more than 50% of cases. The most
ous anastomoses between the thoracic duct and the common malignancy leading to this condition is lym-
azygos, intercostals, and lumbar veins.4 The richness phoma,8 which leads to chylothorax by compressing
of this collateral circulation allows the safe ligation of or invading the thoracic duct or obliterating the
the thoracic duct at any level. lymphatics after radiation therapy.9 Obstruction of
the thoracic duct by lymphoma or bronchogenic
carcinoma tends to cause a right-sided chylothorax
PHYSIOLOGY when the lower portion of the duct is involved,
whereas a left-sided chylothorax results when the
The word “chyle” comes from the Latin word upper portion of the thoracic duct is involved.
meaning “juice” and is applied to the lymph of Chylothorax in the setting of malignancy below the
intestinal origin. Ingested fats are transported by diaphragm invariably indicates metastasis. 10
chyle into the venous blood via the thoracic duct. In The other leading cause of chylothorax are surgery
the fasting state, chyle is usually clear, owing to a low and trauma. Although any penetrating injury that
fat content. Both the protein content and the volume disrupts the thoracic duct would produce chylotho-
are also diminished. After ingestion of a fatty meal, rax, even severe coughing and straining have
chyle assumes its characteristic “milky” appearance. reportedly caused this condition. 11 Surgical
It is estimated that up to 60% of ingested fat passes procedures on the heart, mediastinum, operations in
into the lymphatics. the neck, such as radical neck dissections, and
Between 1,500 to 2,500 milliliters of chyle is placement of catheters in the superior vena cava for
normally emptied into the venous system daily. The hemodynamic monitoring may result in chylothorax.
protein content of chyle is more than 3 gm/dL, and Chylothorax is the most common cause of pleural
the electrolyte composition of chyle is similar to that effusion in neonates, and it usually appears
of serum.5 Significant losses of chyle may result in spontaneously. 1,12-16 Systemic conditions, such as
severe nutritional depletion, especially Behcet’s amyloidosis, sarcoidosis,19 heart failure, and
hypoproteinemia. The chyle formed in the cisterna nephritic syndrome, 20 comprise another group of
chyli is an odourless, white, opaque liquid. Analysis miscellaneous conditions associated with
of chyle reveals approximately 90% lymphocytes and chylothorax.
a white cell count of 2,000 to 10,000 cells per cubic Jaffe-Campanacci syndrome, which includes
millimeter, which generally renders this fluid disseminated non-ossifying fibromata has also been
bacteriostatic. It is believed that most of the reported to be associated with chylothorax. 21
lymphocytes in chyle are T-lymphocytes. 6 Chemical Gorham’s syndrome is a rare disease associated with
analysis reveals protein and fat globules that stain progressive osteolysis along with intraosseous
with Sudan III dye. Chyle is also alkaline with a angiomatosis of lymphatics and blood vessels. Up to
specific gravity of greater than 1.012 and will settle on 17% of patients develop chylothorax as a
standing with a fat-rich portion on the top and a complication in this disease. 22 Pulmonary
cellular sediment on the bottom. Chyle is noted to be lymphangioleomyomatosis is characterised by prolif-
high in neutral fats and fatty acids, but low in eration of immature smooth muscle throughout the
cholesterol. Flow through the thoracic duct can vary peribronchial, perivascular, and perilymphatic
widely from as low as 14 mL/h in the fasting state to region. It occurs primarily in women of reproductive
over 100 mL/h postprandial. Thoracic duct flow is age and the perilymphatic proliferation of smooth
related to response of the duct wall smooth muscle to muscle results in lymphatic obstruction and
splanchnic and vagal stimulation. Serotonin, chylothorax in up to 50% of the patients. 23 This
norepinephrine, histamine, dopamine, and condition may at times be present as a part of the
acetylcholine all increase thoracic duct contraction syndrome of pulmonary tuberous sclerosis.
and chyle flow. 7 In addition to starvation, intestinal A chylothorax may develop acutely without an
rest and administration of opiates will decrease the underlying cause and is referred to as spontaneous
volume of chyle. chylothorax.24,25 Idiopathic chylothorax is uncommon
and comprises cases where no underlying cause can
be found. A sudden increase in duct pressure from
PATHOGENESIS coughing, especially after a heavy meal when the
duct is engorged, may be a contributory factor. 26, 27
The pathogenesis of chylothorax can be divided into Despite detailed work-up the cause of chylothorax
two major categories—surgical and medical causes may not be established in some instances.
(Table). Chylothorax from medical causes results from Most of the cases of chylothorax are unilateral, but
either extrinsic compression or infiltration of the bilateral chylothorax has also been reported, 25, 26, 28-32
2008; Vol. 50 The Indian Journal of Chest Diseases & Allied Sciences 345

for example, Kaposi’s sarcoma 33 has been reported to doubt, the fluid should be analysed for chylomicrons.
result in bilateral chylothorax. Chylothorax and Another useful test is the ingestion of lipophilic
chyloperitoneum have also been reported in patients dye or radio-labeled triglyceride ( 131 I-triolein).
with nephritic syndrome, 19 malignancies such as Presence of the dye colour in the fluid within one
Wilms’ tumour 34, gall-bladder carcinoma,35 uterine hour or detection of high radioactivity in the pleural
cancer,36 stomach cancer,10 lymphoma,37 retroperitoneal fluid after 48 hours confirms the presence of
surgery, 38 hypothyroidism, 39 sarcoidosis 18, yellow chylothorax. 5 Lymphangiography may be helpful in
nail syndrome, 40 idiopathic, 41 primary lymphatic defining the site of chyle leak or obstruction or
dysplasia, 15 lymphangiomyomatosis, 42 and penetrating trauma in spontaneous chylothorax and
43
pancreatitis. in lymphangiomatous malformations. 47 Lym-
phangiography has also demonstrated a therapeutic
role in assisting occlusion of the post-operatively
CLINICAL FEATURES AND DIAGNOSIS damaged lymphatic vessel. This may occur from an
inflammatory granulomatous reaction by the lipiodol
The usual presenting symptom is dyspnoea due to dye during extravasation. 48
accumulation of pleural fluid. Chest pain and fever
are uncommon because chyle is not irritating to the
pleural surface. Traumatic chylothorax usually MANAGEMENT
develops within two to ten days post injury.5 In non-
traumatic chylothorax, the onset of symptoms is more Initial management is determining the aetiology.
insidious. Spontaneous chylothorax may rarely Surgical causes are more obvious. As lymphoma is
present as a sudden neck mass. 24,31 The severity of the most common cause of chylothorax in the non-
symptoms is related to the rate of accumulation of surgical setting, a computed tomography (CT) of the
chyle and the size of the pleural effusion. chest and abdomen should be performed to evaluate
The more serious sequelae of chylothorax are mediastinal and para-aortic lymph nodes. Primary
malnutrition, weakness, dehydration, metabolic treatment must be directed at the underlying cause. In
acidosis, and compromised immunologic status due most instances surgical therapy should be pursued
to the loss of chyle, which is rich in proteins, fats, only after failure of conservative therapy. However,
electrolytes, bicarbonate, lymphocytes, and fat- the timing of surgical management does not have
soluble vitamins. Prolonged chylothorax may be consensus.
associated with reversible T-cell deficiency.44 Hypoal-
buminemia and lymphopenia secondary to Conservative Therapy [Figure 1]
prolonged loss of chyle increase the risk of systemic
bacterial and viral infections. There is a good The initial approach to management of chylothorax
correlation between rate of chyle loss, operative involves chest tube drainage of the pleural space.49-51
intervention, and survlval.45 Continuous suction drainage helps to relieve the
Chyle is often suspected only after a thoracentesis. pressure of chyle on the lungs, re-expands the
Chyle is distinctively white, odourless, and milky in partially collapsed lungs, obliterates the pleural
appearance. The diagnosis of chylothorax is estab- space, and permits an accurate measurement of chyle
lished by measuring triglyceride levels in the pleural production. Lung re-expansion is sometimes hin-
fluid. If triglycerides are greater than 110 mg/dL, the dered by formation of a fibrinous membrane around
diagnosis is probably chylothorax; conversely, if the the lung, which may necessitate surgical
level is less than 50 mg/dL, chylothorax is unlikely. intervention. 52 The rate of chyle leakage can be
When levels are between 50 to 110 mg/dL, actually measured and recorded, if the chest tube is in
lipoprotein analysis should be performed. place. Chest tube drainage of less than 500 mL during
Chylomicrons in the fluid establish the diagnosis of the first 24 hours aftar complete oral intake cessation
chylothorax.46 Milky or creamy pleural fluid can also and total parenteral nutrition may predict successful
be associated with a condition called conservative treatment.53
pseudochylothorax. 5 In this condition, usually Because up to three liters of chyle may drain daily,
associated with chronic diseases, such as large amounts of fluid, electrolytes, fat, protein, and
tuberculosis, turbidity is the result of high levels of lymphocytes may be lost leading to severe nutritional
cholesterol or lecithin-globulin complexes and not depletion and an immunodeficiency state. Careful
chylomicrons. The effusion is usually of long- monitoring of chyle output and replacement of daily
standing duration and may cause thickened and even losses are essential, as is monitoring of the patient’s
calcified pleura. The presence of cholesterol crystals weight, serum albumin, total protein, absolute
in the fluid is diagnostic of pseudochylothorax, and lymphocyte count, and electrolyte levels. A non-fat,
chylomicrons are never present on lipoprotein high-protein, high-calorie diet will produce some
analysis. Both chylous and pseudochylous fluid reduction of chyle flow. Administration of medium-
remain opaque after centrifugation, but the turbidity chain triglycerides (MCTs) as a source of fat is invalu-
of chylous fluid clears out on addition of two able.54 The MCTs are absorbed directly into the portal
millilitres of ethyl ether. Thus, whenever there is any system rather than the intestinal and thoracic
346 Chylothorax A. Talwar and H.J. Lee

Chylothorax


Conservative Management


Treat Underlying Cause


Chest Tube Drainage
Dietary Changes (MCT, TPN, NPO) ±
Somatostatin

Chylothorax Resolves within



Two Weeks

Remove Chest Tube


Monitor for Recurrence

Effusion > 1L/day


Nutritional Deterioration


Surgical Management

Figure 1. Conservative treatment and management of chylothorax.

lymphatics. With the use of MCTs, not only is the anaphylactic reactions have been reported after
nutrition satisfactorily maintained, but thoracic duct intravenous transfusion of chyle.61 Somatostatin is an
flow is minimised to promote healing of the leak. It inhibitor of gastric, pancreatic, and intestinal secre-
has been suggested that in the selection of MCTs, tions, thereby helping to keep the gastrointestinal tract
trioctanoin (C8:O) may be the preferable MCT empty, which in turn decreases the chyle
substrate for these patients.55 The MCT diets have met production. 62
with variable success in the treatment of chylothorax. Octreotide is an analog of somatostatin and has
This is because: (i) any oral enteral feeding increases been used in conjunction with other modalities (i.e.,
lymph flow, 56 and (ii) intestinal triglycerides are TPN, effusion drainage) in conservative management
derived from both endogenous and exogenous of various aetiologies of chylothorax.63-65 Because of
sources.57 If drainage remains unchanged, parenteral octreotide’s longer half-life, it offers a subcutaneous as
alimentation should be started. Others have well as intravenous route of administration. The
recommended stopping oral feeding and initiating support for octreotide is limited to case reports and
total parenteral nutrition (TPN) at the time of diag- small series, making the therapeutic effectiveness of
nosis. 5 Comparison of enteral versus parenteral octreotide difficult to differentiate from spontaneous
nutrition in the setting of chylothorax shows that the improvement or other concurrent treatments. Due to
thoracic duct closure occurs faster with TPN.58 the lack of existing evidence, we cannot recommend
If there is imminent nutritional deterioration, octreotide as a first-line agent at this time.
surgical intervention is indicated. If a patient is on Treatment of the underlying cause may be helpful,
mechanical ventilation, adding a positive end- especially in patients where chylothorax is secondary
expiratory pressure would lead to an increase in to lymphoma, where radiotherapy to the medi-
intrathoracic pressure, help approximate the two astinum is given to resolve the tumour mass.
intrapleural surface and potentially decrease chyle Mediastinal tumours associated with chylothorax,
leakage. 59 Thomson and Simms 60 have reported such as lymphangiomas, also respond to
successful reinfusion of chyle drained from the chest radiotherapy. 66
tube using blood filters and a volumetric pump, but The duration of the conservative management is
2008; Vol. 50 The Indian Journal of Chest Diseases & Allied Sciences 347

not firmly established and must be related to conservative management, 45,52 but there are no large
pathogenesis, underlying comorbid illness, and controlled studies to confirm this recommendation. It
institutional experience. Operative intervention in is appreciated that patients draining more than 1,000
chylothorax should be considered when: (i) average millilitres per day may benefit from earlier (5-7 days)
daily loss has exceeded 1,500 milliliters per year of surgical intervention 74 because of a higher mortality
age in children for a five-day period;52 (ii) chyle flow with increased output.
has not diminished over 14 days; (iii) nutritional Failure of conservative treatment in cases of
complications appear imminent; or (iv) accumulation surgical chylothorax requires a surgical intervention
of chyle is continuous despite chest tube drainage. for definitive management. Lampson 2 first
Chylothorax associated with sarcoidosis will demonstrated that chylothorax could be controlled by
respond very well if the patient is treated with ligation of the thoracic duct. Pre-operative
steroids. Patients with Gorham’s syndrome and administration of lipophilic dye (e.g., Evans blue or
chylothorax should be treated with a cream) helps to locate the site of lymphatic leakage
pleuroperitoneal shunt or thoracic duct ligation. 22 during the procedure. The thoracic duct is then
Pulmonary lymphangiomyomatosis can be treated identified, isolated, and ligated just above the aortic
with hormonal manipulation targeting high-affinity, hiatus between T-8 and T-12.72 After ligation, there is
low-capacity progestin-binding sites. 67, 68 In a meta- usually some obstruction to lymph flow distal to the
analysis of 30 cases of lymphangioleiomyomatosis ligated site until new collateral channels are formed
treated with various hormonal manipulations, it was (2 to 3 weeks).73 An abdominal approach to ligate the
concluded that the administration of progesterone, thoracic duct is an alternative in patients where
oophorectomy, or both results in improvement or thoracic approach is not feasible. 74 Others have
stabilisation of the disease. 69 Lung transplantation
described a posterior extra-pleural approach to
may be the ultimate answer to this problem.70
ligation of the thoracic duct. 75 Most recently, video-
Surgical Therapy [Figure 2] assisted thoracic surgery has provided an effective
and potentially less invasive approach to
Two weeks is often used as the limit for resolution by chylothorax.

Conservative Management
Failure

Treat Underlying Causes


Surgical Management

Lymphatic Imaging Study


▼ ▼

Thoracic Duct Ligation Percutaneous Embolisation


Chylothorax Resolves Chylothorax Resolves


Consider Percutaneous Consider Thoracic Duct


Embolisation or Ligation or

Pleuroperitoneal Shunt or Pleuroperitoneal Shunt or


Pleurodesis Pleurodesis

Figure 2. Surgical treatment and management of chylothorax


348 Chylothorax A. Talwar and H.J. Lee

Successful ligation of the thoracic duct by the thoracic duct. Talc pleurodesis is most often used
thoracoscopy using fibrin glue or endoscopic clips at in chylothorax due to malignant disease that is
the site of leak has been attempted,76-78 and has the resistant to conservative treatment. Other approaches
advantages of less post-operative pain and a shorter include intrapleural infusion of tetracycline, 81 fibrin
hospital stay. Graham and colleagues 79 have glue, 82 and OK-432, 83 a Substrain of streptococcus
described successful use of video-assisted thoracic pyogenes. Intra-pleural bleomycin has been found to
surgery in 10 patients of chylothorax and recommend be more effective in patients with chylothorax
early operative intervention with this procedure. secondary to lymphoma.84
Early thoracoscopic repair has two major advantages: The surgical approach to nontraumatic chylous
(i) the risk of malnutrition is minimised; and (ii) post- effusions is more variable. Milsom and co-workers85
thoracotomy pain and discomfort are avoided. In recommend pleuroperitoneal shunting after failure of
situations where it is impossible to identify the duct conservative therapy and before thoracotomy.
surgically, especially in malignancy and radiation Murphy and associates1 have recommended placing
fibrosis, pleurodesis or pleurectomy may be used. 80 the shunt if the drainage persists beyond five days. A
These procedures have not only been used alone but pleuro-peritoneal shunt consists of pleural and
also in combination with thoracotomy and ligation of peritoneal catheters connected to a manual pumping
Table. Causes of chylothorax
Surgical Medical Causes
Post-operative Neoplasms
Thoracoscopic procedures Lymphoma (8)
Thymectomy (32) Gastric carcinoma (10)
Coronary artery bypass (95) Chronic lymphocytic leukemia (118)
Congenital heart disease (96) Bronchogenic carcinoma (107)
Bochdalek herniography (97) Gall-bladder carcinoma (35)
Esopageal resection (98) Multiple myeloma (108)
Sclerotherapy for varices (99) Wilms tumour (34)
Celiac plexus block (100) Prostate carcinoma (109, 110)
Radical neck dissection (101) Uterine cancer (36)
High translumbar aortography (102) Pulmonary Kaposi’s (111)
SVC or subclavian catherisation (29, 103) Mediastinal lymphagiomas (66)
Amputation of upper arm (104) Infections
Gastric resection (105)
Pulmonary resection (106) Filariasis (1)
Bilharziasis (1)
Non-surgical Trauma
Tuberculosis (1,112)
Penetrating gunshot or stab wound (5) Miscellaneous
Blast or crush injuries (5)
Weight lifting or straining (5) Behcet’s syndrome (17)
Severe bout of coughing or vomiting (5) Amyloidosis (18)
Vigorous stretching while yawning (11) Sarcoidosis (19)
Cirohosis (1)
Heart failure (1)
Tuberous sclerosis (1)
Nephrotic syndrome (20)
Gorham’s syndrome (22)
Post-irradiation (9)
Pregnancy (113)
Retrosternal goiter (114)
Hypothyroidism (39)
Yellow nail syndrome (40)
Lymphangiomyomatosis (42)
Intestinal lymphatic dysplasia (115)
Primary lymphatic dysplasia (15)
Congenital pulmonary lymphangiectasia (16)
Jaff-Campanacci syndrome (21)
Sclerosing mediastinitis (116)
Hypobetalipoproteinemia (117)
Pancreatitis (43)
Spontaneous (26)*
Down’s syndrome (12)*
Noonan’s syndrome (13)*
Turner’s syndrome (1)*
Maternal polyhydraminos (1)*

Number in parantheses indicate study reference number; *Associated with neonatal chylothorax
2008; Vol. 50 The Indian Journal of Chest Diseases & Allied Sciences 349

chamber capable of handling viscous fluid such as 6. Machleder HI, Paulus H. Clinical and immunological
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