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2/17/2020 Management of chylothorax - UpToDate

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Management of chylothorax
Author: John E Heffner, MD
Section Editor: V Courtney Broaddus, MD
Deputy Editor: Geraldine Finlay, MD

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Jan 2020. | This topic last updated: Jul 23, 2018.

INTRODUCTION

Chylothorax is the accumulation of chyle in the pleural space. Chylothorax can be due to several
etiologies, among which malignancy and surgical trauma are the commonest (table 1). Chylothorax is
associated with significant morbidity and mortality if left untreated.

The management of chylothorax will be reviewed here. The etiology, clinical presentation, and
diagnosis of chylothorax are discussed separately. (See "Etiology, clinical presentation, and diagnosis
of chylothorax".)

GENERAL PRINCIPLES

No management algorithm has been universally adopted for patients with chylothorax since multiple
clinical factors unique to each patient impact therapy including etiology (table 1), symptoms, local
expertise, and rate of chyle accumulation [1]. There are no large randomized trials comparing
therapies; therefore our approach is based upon clinical experience and data that are mostly derived
from case series of patients with postoperative chyle leak.

Low output chylothorax — Patients are considered to have low output chylothorax if the estimated
or known volume of drainage or accumulation is less than 1 L chyle per day. Many patients with
medical reasons for their chylothorax and postoperative patients with chyle leaks due to minor trauma
to the thoracic duct (eg, trauma of a small thoracic duct tributary) fall into this category. In general,
these patients benefit from a staged care plan that moves from least invasive options to more
invasive interventions. This includes:

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● Drainage for symptom control, dietary control measures, and treatment of the underlying
condition (table 1). Adjuncts (somatostatin/octreotide) are often also administered as a way to
avoid surgery in this population. As a general rule of thumb, the larger the leak, the more likely a
patient will fail such conservative therapies, increasing the need for a definitive intervention. (See
'Medical care for those not requiring immediate surgery' below.)

● Should these measures fail, then chemical or surgical pleurodesis, thoracic duct embolization or
disruption, thoracic duct ligation, or some combination thereof is considered. (See 'Patients who
fail medical therapy' below.)

High output chylothorax — High output chylothoraces (estimated or known volume >1 L chyle per
day) are most commonly seen in postsurgical patients (especially esophagectomy) and those with
liver cirrhosis [2]. In such patients, thoracic duct ligation or embolization are typically needed early
(often within the first few days after diagnosis) since conservative strategies are more likely to fail in
this population [3]. (See 'Patients who fail medical therapy' below.)

MANAGEMENT OF POSTOPERATIVE CHYLE LEAKS

Postoperative chyle leaks are the most common form of chylothorax encountered. While most
postoperative leaks are low volume (<1 L per day), some are high volume (>1 L per day) with the
former being treated conservatively and the latter typically treated aggressively with thoracic duct
repair/ligation or embolization.

Evaluation for urgent or early surgery (high output) — Patients in the postoperative setting are at
greatest risk of having high volume chyle leaks (>1 L per day) that require urgent or early surgical
repair (hours to a few days). In such cases, a chest tube is typically already in place (eg, after
thoracic or esophageal surgery) while in others a chest tube may have been placed to drain a
postoperative pleural effusion (eg, after neck or spinal surgery).

In most cases, patients with high volume leaks are treated by complete bowel rest by the
administration of parenteral nutrition and surgical repair of the thoracic duct (within hours to days).
Some patients may benefit from somatostatin/octreotide while waiting for surgery. The rationale for
this approach is that these patients are likely to have major thoracic duct injury which is unlikely to
close spontaneously rather than leaks that originate from a smaller thoracic duct tributary which are
more likely to undergo spontaneous closure. In addition, early surgical repair avoids severe metabolic
or nutritional derangements that are more likely to occur in those with large amounts of chyle loss.
(See 'Pleural drainage to control symptoms' below and 'Dietary modification' below and 'Somatostatin
and octreotide' below and 'Thoracic duct ligation' below.)

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The timing of thoracic duct repair varies. While some experts perform immediate surgery in those who
drain >1 L of chyle in the first postoperative day [4], other experts may observe for a period and
perform surgical repair in those who continue to drain >1 L for up to 5 consecutive days despite
conservative therapy. In this setting, thoracic duct repair (eg, ligation or fibrin glue repair) is highly
likely to be successful and may be combined with pleurodesis at the time of surgery [1,2,5-9].

As an alternative, thoracic duct embolization/disruption may be performed in those patients who are
poor candidates for surgery. (See 'Lymphatic embolization and disruption' below.)

Patients not in need of emergent surgery (low output) — In postoperative patients with low
volume leaks or high volume leaks that respond dramatically to conservative therapy, a management
strategy that is similar to that performed in nonsurgical cases of chylothorax is appropriate (ie, with
continued drainage of chyle, somatostatin/octreotide, and dietary modification, generally with
parenteral nutrition) (see 'Medical care for those not requiring immediate surgery' below). Surgical
repair of the thoracic duct is considered in those who fail these conservative measures, while thoracic
duct embolization may be appropriate in those not suitable for surgery. (See 'Thoracic duct ligation'
below and 'Lymphatic embolization and disruption' below.)

Postpneumonectomy chylothorax — Patients with a chylothorax in a postpneumonectomy space


in the early postoperative state are treated a little differently. Rapid postoperative filling of the
pneumonectomy space with fluid (which otherwise typically takes one to four weeks) typically raises
the suspicion for chylothorax (a chest tube is not often in place). If confirmed by thoracentesis and no
tension is present, chylothorax is managed with dietary control measures (ie, fasting and TPN)
without chest tube drainage and patients should immediately proceed directly for evaluation of
thoracic duct repair/ligation. If there is evidence of mediastinal shift to the contralateral side, patients
should have a chest tube placed emergently and also be evaluated for immediate surgical repair to
gain control of the lymphatic leak [10,11]. One case report described a patient with chylothorax and
mediastinal shift after pneumonectomy that responded with chest tube drainage and a period of
parenteral nutritional support [12], although most clinicians do not adopt this strategy.

The rationale for not placing a chest tube for drainage in those without evidence of mediastinal shift is
the depletion of nutrients, immunoglobulins, and lymphocytes as well as that the placement of a chest
tube will further reduce pressure in an already low pressure postpneumonectomy space; the latter
potentially worsens the leak by promoting flow from the thoracic duct (high pressure) to the pleural
space (low pressure), thereby prohibiting spontaneous healing. By comparison, in patients who have
a lobectomy, expansion of the remaining nonresected lung with or without a chest tube in place helps
to raise the pleural pressure and provide some "tamponade effect" on the leaking duct.

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MEDICAL CARE FOR THOSE NOT REQUIRING IMMEDIATE SURGERY

Initial therapies — Most patients with chylothorax who do not require urgent surgery should undergo
pleural drainage (for symptom control) and dietary modification while the underlying disorder is being
addressed (table 1) [13-19]. This strategy reduces the rate of accumulation in many patients and in
some cases allows the opportunity for cases due to chyle leak to heal spontaneously without surgical
intervention.

Pleural drainage to control symptoms — If a chest tube is not already in place, most patients
should undergo drainage of chyle with a chest tube or indwelling catheter (without suction) as an
initial form of therapy to relieve symptoms (typically dyspnea) due to the effusion, although in some
nonsurgical patients intermittent thoracentesis is sufficient. A chest tube or catheter left in place helps
inform the clinician regarding the volume of chyle loss per day which impacts future decisions
regarding therapy (eg, type and timing of interventional or surgical modality). However, there are
some exceptions to the rule where drainage is not typically performed:

● Asymptomatic nonsurgical patients – After an initial diagnostic thoracentesis to diagnose a


chylothorax, repeat thoracentesis or catheter drainage is not necessary in this population but
patients may benefit from dietary measures, treatment of the underlying condition, and serial
observation.

● Patients with chylous ascites. (See 'Chylous ascites' below.)

● Postpneumonectomy patients with a chylothorax in the early postoperative course who do not
have evidence of mediastinal shift. (See 'Postpneumonectomy chylothorax' above.)

In general, the decision to proceed with continued drainage or intermittent thoracentesis is dependent
upon the rate of estimated loss of chyle/rate of re-accumulation as well as nutritional status, patient
prognosis, and patient preference. While there are no guidelines to facilitate this decision, we
generally use the following principles:

● With the exception of postpneumonectomy patients (see 'Postpneumonectomy chylothorax'


above), most surgical patients who develop a postoperative chylothorax, continued drainage is
typically necessary, particularly those who have chyle loss on the higher side (eg, 500 to 1000
mL per day). For nonsurgical patients with chylothoraces that rapidly re-accumulate after a
diagnostic thoracentesis (hours to days), continuous chest tube or catheter drainage is generally
needed to relieve symptoms.

● For all patients with chylothorax, long-term, continuous chest tube drainage is associated with
immunosuppression from loss of protein, lymphocytes, and immunoglobulins [20-24]. Thus,

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continuous pleural fluid drainage is generally limited to less than 14 days to minimize the risk of
infection, malnutrition, and electrolyte losses as well as diminish tolerance for definitive surgery.
For patients with postoperative chylothorax, intervention (eg, thoracic duct ligation or
embolization) is generally indicated sooner (eg, within two to five days) if the drainage of pleural
fluid exceeds 1 to 1.5 L/day, or if the drainage of chyle results in weight loss and/or progressive
hypoproteinemia despite aggressive nutritional therapy [25,26]. (See 'Evaluation for urgent or
early surgery (high output)' above.)

● For patients who have a gradual reaccumulation of chyle (eg, outpatients with nonsurgical or
nontraumatic chylothorax who reaccumulate over days to weeks or have <50 mL chyle output
per day), intermittent therapeutic thoracentesis (eg, every two to six weeks or longer) or use of
an indwelling catheter for home drainage (eg, drainage every week) is appropriate. (See
"Ultrasound-guided thoracentesis" and "Management of malignant pleural effusions", section on
'Indwelling pleural catheter (IPC)'.)

All patients managed with continuous drainage of chylous pleural fluid require monitoring of serum
electrolytes, lymphocyte counts, albumin, total protein, and weight. For those with high volume loss,
patients may require blood tests two to three times a week until they maintain stable values while
those with lower rates of loss may have testing every few weeks. Patients who undergo continuous
drainage with restricted oral diet require careful attention to nutritional needs with provision of enteral
or parenteral nutrients designed to offset the protein and calorie losses related to drainage of the
chylothorax. Consultation with a nutritionist is advised. (See 'Dietary modification' below.)

Dietary modification — The principles of dietary management focus on reducing the flow of chyle
through the thoracic duct by either of the following:

● Oral or enteral low-fat diet – Patients suitable for an oral or enteral diet are those with low
volume chylothorax due to any etiology. Patients should be assessed by a dietician and given
clear instructions on eating a high-protein, low-fat (<10 g fat/day) diet. Since decreasing fat intake
decreases fat absorption from the gut and therefore decreases the flow of chyle, this should
reduce the accumulation of chyle in the pleural space [5,15,27-29]. Dietary exclusion of long-
chain triglycerols (LCTs) is encouraged; this avoids their conversion into monoglycerides and free
fatty acids (FFA), which are transported as chylomicrons to the intestinal lymph ducts. An oral
diet is always preferred to an enteral diet, when possible. Some clinicians provide a fat-free diet
(<5 kcal fat/serving) initially but such diets are highly unpalatable and difficult to tolerate for more
than a few days. General principles include the following:

• Initially, patients should receive a low-fat, high-protein diet with an emphasis on reducing
LCTs.

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• Oral vitamins should be provided, although decreased absorption of fat-soluble vitamins with
low-fat diets may require parenteral supplementation of these micronutrients.

• Peripheral intravenous (IV) fat emulsions (250 mL of 20 percent emulsion IV three times a
week) may be appropriate if it is felt that the patient is at high risk of essential fatty acid
deficiency.

• Enteral nutrition may be prescribed as a supplement to oral nutrition or as the sole source of
nutrition for patients who can take nothing by mouth.

• If pleural drainage decreases, medium chain triglycerols (MCTs) can be introduced orally as
a liquid or capsule or in the form of enteral feeding products, which may not be tolerated
because of taste. MCTs are absorbed directly into intestinal cells and transported directly to
the liver via the portal vein, thus bypassing the thoracic duct [13,14,27,30]. Common
adverse effects are gastrointestinal upset, steatorrhea, and elevated cholesterol blood
levels. (See "Chylous, bloody, and pancreatic ascites", section on 'Management'.)

• Fat intake is gradually increased as the patient improves and the volume of pleural drainage
decreases. Because each patient responds differently to dietary modification, no specific
regimen is available to time liberalization of fat intake. Nonetheless, we typically utilize a low-
fat diet for 7 to 10 days. If patients continue to improve with decreased pleural fluid drainage,
LCTs are added to the diet with progression to an unrestricted diet as tolerated [29]. If
patients do not improve, then total parenteral nutrition (TPN) is appropriate.

● Total parenteral nutrition – For patients with high volume chyle leaks (eg, postoperative leaks >1
L/day), the administration of TPN is generally preferred since a dramatic reduction in the flow of
chyle may promote healing and prevent acute nutritional deficiencies [31]. TPN may also be
administered to patients who are unable to be fed orally or enterally and those in whom oral and
enteral dietary measures fail. No fat restrictions for TPN apply since lipids are administered
intravenously. (See "Nutrition support in critically ill patients: Parenteral nutrition".)

Treatment of the underlying condition — A critical step in the management chylothorax is


treatment of the underlying disease (table 1) [6,32-34]. For example, patients with chylothorax due to
underlying sarcoidosis may respond to glucocorticoid therapy, those due to infection may respond to
treatment with antimicrobials, and some patients with a surgical chyle leak may require repair.

Adjunctive therapies

Somatostatin and octreotide — Somatostatin or octreotide can be used as adjunctive therapies.


They are commonly used routinely in conjunction with chest tube drainage and bowel rest with TPN in
postoperative patients with chyle leaks (<1 L per day) since this has been shown to increase the
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probability of avoiding surgical repair [35]. In medical patients with chylothorax due to nonsurgical
etiologies (eg, cancer) some experts choose to use them in conjunction with conservative therapy
while others only administer somatostatin/octreotide when conservative measures have failed or have
been partially successful, or when thoracic duct surgery or embolization is not feasible. Their use
should be limited to a trial of a few weeks. (See 'Less well established options' below.)

Somatostatin and octreotide, a somatostatin analogue that has a longer half-life in the circulation,
decrease the volume of fluid within the thoracic duct by inhibiting gastric, pancreatic, and biliary
secretions, and inhibiting absorption of chyle from the intestine [36,37]. Because of the reduction in
chyle production and flow rate, these agents have been successfully used in case reports and case
series to treat spontaneous chylothorax, congenital chylothorax, postoperative chylothorax, and
chylothorax due to malignancy and yellow nail syndrome, often in combination with a reduced fat diet
supplemented with medium chain triglycerides [35,38-46].

● One systematic review of octreotide for management of chylothorax after cardiothoracic surgery
noted benefit in patients with moderate to large volume chylothorax when used as adjunctive
therapy with TPN and bowel rest noting benefit within two to three days [35]. (See "Physiology of
somatostatin and its analogues".)

● A small case series (seven patients) and isolated case reports have described successful
management of malignant chylothorax using subcutaneous octreotide and a fat-free diet [41,43].

Reported doses of somatostatin and octreotide for treatment of chylothorax vary and the optimal dose
and route of administration are not known. In a series of adult patients, somatostatin was given by
intravenous infusion 6 mg/day for two weeks or by subcutaneous injection, 50 micrograms every eight
hours; octreotide was given in subcutaneous doses of 50 to 100 micrograms every eight hours for 2
to 14 days [36,45]. Doses in the range of 1 to 10 micrograms/kg/hour have been used in children with
mixed success [47].

In general, side effects of somatostatin and octreotide include cutaneous flushing, nausea, diarrhea,
sinus bradycardia, injection site pain, transient hypothyroidism, and elevated liver function tests.

Midodrine — Two case reports suggested that midodrine, an alpha 1-adrenergic agonist that
causes vasoconstriction of the lymph system, reduced drainage in a patient with postoperative, high-
output chylothorax and a patient with idiopathic chylothorax, both of which failed to respond to dietary
modifications and surgical intervention [48,49]. A similar case report of success with another alpha
adrenergic agonist, etilefrine, has also been described [50].

Monitoring the response — The response to conservative therapy should be monitored clinically by
measuring the volume of daily drainage for those with a chest tube or catheter in place. Periodic

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imaging, with chest radiograph or chest computed tomography, may also provide information
regarding rate of reaccumulation for those with slowly accumulating fluid who do not have a chest
tube in place.

Duration of monitoring — The duration of monitoring the response to conservative therapy


before resorting to definitive measures remains controversial and is mostly derived from the
experience with patients who have postsurgical chylothorax [31,51] since the timing for resolution is
variable. While some leaks take a few days to respond, others may take longer (two weeks or more).
The decision regarding when to intervene with definitive measures varies in surgical and non-surgical
settings:

● In the postoperative setting, early intervention within one to five days is indicated in those in
whom fluid output exceeds 1 L/day or when severe metabolic or nutrition derangements occur
[4,6,52] (see 'Evaluation for urgent or early surgery (high output)' above). In those with chyle
leaks <1 L per day, intervention is typically warranted if a chylothorax does not respond to
conservative measures by about two weeks.

● In the non-surgical patient, timing of interventions is based upon multiple factors. These include
initial rate of pleural drainage, the underlying disease, the severity of respiratory compromise due
to the chylothorax, the ability to drain the pleural space adequately, and the degree of response
to conservative measures. For example, a declining output at two weeks in response to
conservative therapies in a patient with chylothorax should encourage the clinician to persist with
such measures while no response or a plateaued response may be an indication to consider an
interventional or surgical procedure.

Efficacy — A large proportion of patients improve with conservative therapies but success rates vary
and are wide-ranging. Success depends upon factors including etiology (table 1), reversibility in
response to etiology-specific therapies, and volume of pleural fluid drainage [1,53,54]. In general,
patients who drain more than 1 L/day are unlikely to respond to conservative therapy and usually
require surgical intervention within five days or less [4,54,55]. In contrast, in patients with less than
500 mL of chest tube drainage in the first 24 hours after cessation of oral intake and initiation of total
parenteral nutrition, the chylothorax tends to resolve with conservative management [14,16]. Case
series report the following success rates:

● Conservative measures have a 50 to 80 percent success rate in postoperative chylothorax


[2,4,5,14,19,54] with higher success rates reported in patients with <500 mL/day pleural fluid
drainage, lower in those with volumes >1 L per day [4,14]. Conservative measures are also
successful in approximately 50 percent of patients with nonsurgical traumatic chylothorax [5].

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● In patients with benign disease such as infection or sarcoidosis, conservative measures are
successful in approximately 40 to 80 percent [2,5,15].

● Treatment of the underlying malignancy with first line therapies including chemotherapy and
irradiation has also been successful [6,32-34,56-58]. Among patients with lymphoma, resolution
of chylothorax with drainage, diet, and chemotherapy or radiation ranges from 25 to 70 percent,
depending on the series [56,57]. A retrospective case control study of 10 patients with malignant
chylothorax found a favorable response to outpatient use of an indwelling pleural catheter,
compared with repeated thoracenteses, a pleuroperitoneal shunt, or talc pleurodesis [59].

PATIENTS WHO FAIL MEDICAL THERAPY

For patients not responding to conservative therapy, definitive intervention is warranted [1,33].
Options include pleurodesis, thoracic duct embolization or disruption (TDE/TDD), thoracic duct
ligation (TDL), or combinations of TDL or TDE/TDD with pleurodesis.

Selecting a modality — Selection of an interventional modality is individualized and depends upon


multiple factors including the etiology of chylothorax (table 1) as well as local surgical and
interventional radiologic expertise and preference, the rate of chyle loss, the likelihood of response to
the chosen intervention, the risk of surgery, and patient preferences [14,17,19,54,60,61]. We typically
present such patients to a multidisciplinary team of pulmonologists, thoracic surgeons, and
interventional radiologists to select an appropriate intervention(s).

● For patients with postoperative chyle leaks who fail medical therapy, surgical repair of the
thoracic duct (eg, ligation/repair) with or without pleurodesis is usually the preferred choice
[1,2,5-9,14]. TDE/TDD is appropriate for patients who are poor candidates for TDL. (See
'Thoracic duct ligation' below and 'Lymphatic embolization and disruption' below and
'Pleurodesis' below.)

● For nonsurgical patients who fail medical therapy, treatment options include chemical or surgical
pleurodesis, thoracic duct embolization or disruption, ligation or repair of the thoracic duct, or
combinations of these therapies (typically pleurodesis with TDL) [61-64]. As examples:

• Patients who drain lower volumes of chyle (eg, <500 mL/day) may be suitable for
pleurodesis alone [19], while those who drain volumes >500 mL may be more suited for
TDL, TDE/TDD, or a combination of pleurodesis and TDL. (See 'Thoracic duct ligation'
below and 'Lymphatic embolization and disruption' below and 'Pleurodesis' below and
'Combination therapy' below.)

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• Patients with chyle leak from malignancy may be treated with pleurodesis alone, since
thoracic duct ligation is not generally successful in this population; reasons for this are
unknown but may relate to the likelihood of being unable to repair a diseased part of the
thoracic duct or its tributaries that are involved with malignancy. (See 'Pleurodesis' below.)

• Patients who have a patent and intact thoracic duct on lymphatic imaging and who have
chylothorax from abnormal retrograde lymphatic flow or lymphatic vessel malformations that
direct flow to the lungs may respond to TDE/TDD of retroperitoneal lymphatics rather than
TDL. For those with lymphatic masses (which often overproduce lymph), embolization of the
masses while keeping the thoracic duct patent is also appropriate. (See 'Lymphatic
embolization and disruption' below.)

It is noteworthy that occasionally when contrast-enhanced lymphangiography is performed to localize


a chyle leak or examine lymphatic anatomy, this alone may be therapeutic, perhaps due to the
sclerosing effects and temporary "oily" occlusion of lymphatics by the contrast agent, lipiodol. (See
"Etiology, clinical presentation, and diagnosis of chylothorax", section on 'Additional testing in select
patients'.)

Modality options

Pleurodesis — For patients who fail conservative therapies in whom urgent surgical repair of the
thoracic duct is not indicated (eg, those with high volume postoperative chyle leak), pleurodesis is an
option. For those in whom pleurodesis is selected, options include medical pleurodesis with talc
instillation by a chest drainage catheter or other chemicals (eg, bleomycin or tetracycline), and
surgical pleurodesis (eg, abrasion, chemical insufflation, pleurectomy) at the time of thoracoscopy or
thoracotomy [5,7,65-67]. Choosing between medial or surgical pleurodesis is dependent upon the
ability of the patient to tolerate surgery and patient preference, but in general surgical pleurodesis is
more effective than medical. The details of pleurodesis are described separately. (See "Chemical
pleurodesis" and "Talc pleurodesis".)

Because the success of pleurodesis generally depends on adequate drainage of the pleural space
prior to the introduction of chemical or talc, concomitant thoracic duct intervention such as
embolization or ligation is commonly performed [5,66]. Thus, data to support the success of
pleurodesis alone are limited:

● For patients with postoperative chyle leaks, success rates vary from 80 to 100 percent
[14,19,68]. One series of 37 patients with chylothorax after thoracotomy reported that 80 percent
of patients with persistent chylothoraces after conservative measures who had a pleural fluid
output of 300 to 500 mL/day responded to pleurodesis with OK-432 (a lyophilized preparation of
Streptococcus pyogenes) [14]. Patients with >500 mL/day of pleural fluid output responded to

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surgical interventions. In the setting of high volume postsurgical chylothorax (eg, >1 L/day), TDL
is generally considered the treatment of choice, and therefore, no data are available regarding
pleurodesis without TDL in this population. Another case series of 67 patients with chylothorax
after lung resection demonstrated success with pleurodesis as the only intervention in all 27
patients for whom 32 pleurodeses were performed [19]. (See 'Thoracic duct ligation' below.)

● In the setting of nonsurgical chylothorax, case series of patients who underwent pleurodesis
alone generally revealed a success rate between 80 and 100 percent [5,66,69,70]. For patients
with a malignant chylothorax, pleurodesis (usually chemical) is favored over surgical ligation of
the thoracic duct because malignant chylothorax does not usually benefit from ligation of the
thoracic duct [66,69,71]. However, case series have reported mixed results with pleurodesis in
patients with malignancy with some case series of patients with malignant chylothorax reporting
high success rate with pleurodesis (up to 100 percent) [69,72], and others reporting failure
[5,59,73].

Lymphatic embolization and disruption — Percutaneous catheterization and embolization or


needle disruption of the thoracic duct, cisterna chyli (figure 1 and figure 2), lymphatic masses, or
prominent retroperitoneal lymphatic ducts have been employed in patients with both traumatic and
nontraumatic chylothoraces [74-85]. Thoracic duct embolization (TDE) or thoracic duct disruption
(TDD) has the advantage of being able to identify the leak and offer a therapy in the same setting and
is less invasive when compared with thoracic duct ligation (TDL). These techniques are increasingly
used for management of chylothoraces as an alternative to TDL, chylothoraces that fail to respond to
pleurodesis alone (eg, TDL may difficult to perform in those who have had previous pleurodesis since
accessing the thoracic duct on the pleurodesed side can be challenging), and chylothoraces in
patients who are poor operative candidates [84,86]. However, expertise is limited to a few centers.
Among the multiple procedures available, TDE/TDD represent the most commonly performed.

Initial lymphangiography with pedal or ultrasound-guided intranodal lymphatic cannulation allows


visualization of large retroperitoneal lymphatics, which are then most commonly accessed by
transabdominal percutaneous needle cannulation. Some centers access the thoracic duct in a
retrograde manner via a subclavian vein approach [81,87] or by transnodal lymphangiography via
groin nodes [88] when a transabdominal approach is unsuccessful or unfeasible. Other approaches
have been reviewed in the literature [86] with one case report demonstrating success with
percutaneous thoracic duct scleroembolization guided by computed tomographic-lymphangiography
[89].

● Thoracic duct embolization (TDE) – After cannulation of the cisterna chyli, a catheter is
advanced into the thoracic duct with instillation of contrast to localize the leak. The affected
thoracic segment is then embolized with coils and surgical "glue" [74,80,81].

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● Thoracic duct disruption (TDD) – If the thoracic duct cannot be cannulated (about one third of
cases), it can be disrupted by multiple needle passes through the duct under fluoroscopic
guidance.

TDE/TDD is technically challenging but, in centers with expertise, approximately 80 percent of


patients experience a successful procedure on the first attempt [81].

Experience with TDE/TDD is limited and trials comparing it to thoracic duct ligation have not been
performed. Success rates derived from case series vary. In patients with traumatic and nontraumatic
chylothoraces who underwent TDE or TDD, clinical success rates were 72 percent for TDE and 55
percent for TDD with higher rates of clinical response for traumatic (62 percent) as compared with
nontraumatic (13 percent) chylothoraces [81]. Other series limited to patients with traumatic
chylothoraces report a complete or partial success rate with TDE/TDD of 71 to 74 percent [77,80].
Success rates for TDE alone for patients with traumatic chylothorax have been reported as high as 90
percent [80]. For patients with nontraumatic chylothoraces, success of TDE has been reported to be
highest (75 percent) when lymphatic obstruction is demonstrated by lymphangiography to be at the
level of the thoracic duct [82].

Immediate post-procedure complications are usually minor (bleeding and pain at the injection site)
and occur in less than 7 percent of patients [74,78,81,90]. Leg swelling and chronic diarrhea are the
most common delayed complications each occurring in eight percent of patients. Other complications
include lung parenchymal bleeding, embolization of lipiodol to the pulmonary circulation, and infection
at the catheter injection site. (See "Etiology, clinical presentation, and diagnosis of chylothorax",
section on 'Lymphangiography'.)

Wider experience is needed to determine other clinical factors that predict a response. As an
example, patients with nontraumatic chylothorax and lymphatic masses may have patent thoracic
ducts on lymphangiography and may not respond to thoracic duct embolization because chylothorax
results from overproduction of lymph, weeping of lymph from diseased lymph nodes into the pleural
space, or leakage from malformed or disrupted lymphatic channels within or near the region of the
lymphatic masses. However, these patients may benefit from direct injection of the lymphatic masses
during the procedure instead of embolization [91].

Thoracic duct ligation — Thoracic duct ligation (TDL) is best suited for those who have a high
volume chyle leak in a postoperative setting (eg, >1 L per day most commonly following
esophagectomy, cervical procedures, or lung resection [5,32,54,55,60,70,92]) or patients with high
volume leak due to liver cirrhosis. However, TDL can also be successful in nonsurgical patients with
low volume leak who have failed conservative therapy, although outcomes are less certain.

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TDL is performed via video-assisted thoracoscopy or open thoracotomy depending on local expertise
and is the preferred method for visibility of the thoracic duct [1,2,6,9,16,31,53,93]. However, locating
(and repairing) the leak postoperatively may be challenging due to tissue inflammation. Thus,
preoperative visualization by various lymphangiography imaging techniques can facilitate localization
of the leak. In addition, dairy cream or another fat load (eg, olive oil) alone or mixed with a lipophilic
green dye may be administered via nasogastric or nasojejunal tube 20 minutes prior to induction of
anesthesia or intraoperatively, if needed [1,2].

Access to the thoracic duct is usually via the side with the chylothorax because that side is typically
the location of the thoracic duct tear. In addition, accessing the side with the chylothorax allows
mechanical or chemical pleurodesis to be performed on that side during the operation. However,
other considerations regarding the surgical approach may influence the choice of which side to enter,
such as the exact course of the thoracic duct in that patient, the side of the initial surgery, or the ease
or difficulty of a left-sided approach in patients who have a gastric conduit following esophagectomy.

Sutures or clips are placed on both sides of an identified tear; when the exact leakage site is unclear
during surgery, the duct is usually ligated or clipped just cephalad to the aortic hiatus [2,6,31]. In
addition, a sealant or fibrin glue is sometimes applied to the area of ligation [31,94]. Extensive
dissection to identify the site of leakage is discouraged because this may create additional thoracic
duct leakage sites [32]. If a leak cannot be identified, thoracoscopic mass ligation of the duct is
sometimes performed above the esophageal hiatus between the aorta, vertebral bodies, and
pericardium. When the chyle leak is found to be subdiaphragmatic based upon thoracoscopic
examination or lymphatic imaging, a laparoscopic approach is used to ligate the cisterna chyli (figure
1) at the point of entry into the thoracic cavity [95,96].

TDL is an effective procedure. TDL for postoperative chylothorax is associated with a 90 percent
success rate [5,70]. Whether it is as successful in those with nontraumatic chylothorax is unknown
but in our experience, this population does not have the same degree of success with ligation as
those with traumatic chyle leak.

Despite the reported success rate, TDL has a morbidity and mortality of 38 and 2 percent,
respectively, when performed for postoperative chylothoraces [55]. Lymphedema is a known
complication of TDL, but usually resolves over several months as collateral lymphatic-venous
communications develop [1]. In postoperative patients, untreated chylothorax has a mortality that
ranges from 50 to 80 percent and ligation reduces mortality in the range of 10 to 16 percent
[1,5,70,97].

Combination therapy — Many clinicians perform concomitant pleurodesis in patients undergoing


TDL since both procedures can be performed in the same setting without significantly increasing the

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procedural risk. There are no absolute indications for this combination. In addition, although outcome
with this combination appears to offer the most success, data are not reliable [1,2,5-9]. Thus, the
inclusion of pleurodesis with surgical TDL is left to surgeon discretion [2,5-7,9]. Pleurodesis may also
be combined with TDE/TDD but outcomes using this approach are unknown.

Less well established options — For patients with refractory chylothorax, therapies including
adjunctive medications (see 'Adjunctive therapies' above), if not already instituted, and mechanical
shunts have been reported in multiple case series, but no randomized controlled trials exist to define
their role. (See 'Pleuroperitoneal or pleurovenous shunt' below and 'Others' below.)

Pleuroperitoneal or pleurovenous shunt — Pleurovenous or pleuroperitoneal shunting of


chylous pleural fluid is an option after failure of definitive therapies (eg, pleurodesis and/or thoracic
duct ligation, embolization, or disruption) or for those who have failed conservative management but
are poor candidates for more invasive interventions [8,28,70,98-100].

● Pleuroperitoneal shunts – Pleuroperitoneal shunts divert chyle from the pleural space to the
peritoneum; it is postulated that peritoneal chyle returns to the venous circulation via absorption
through peritoneal omentum or via the lymphatic stomata on the underside of the diaphragms
[101,102]. Two types of pleuroperitoneal shunts are available: an active version that requires
frequent activation of a manual pump (Denver pleuroperitoneal shunt) and a passive version
(LeVeen pleuroperitoneal shunt) [99]. Pleuroperitoneal shunting was successful in a patient with
a refractory high-volume chylothorax that developed after transhiatal esophagectomy [99]. On
the other hand, it was unsuccessful in a patient with yellow nail syndrome who developed
worsening abdominal distension and lower extremity edema; however, pleurovenous shunting
was successful in that patient [98]. Pleuroperitoneal shunting is contraindicated in patients with
chylothorax due to chylous ascites.

● Pleurovenous shunting – Pleurovenous shunting of chylous fluid from the pleural space to the
subclavian or jugular vein has been successful in isolated patients with
lymphangioleiomyomatosis, yellow nail syndrome, and postsurgical or post lung transplant
chylothorax [8,98,100,103].

The advantage of shunts is that they are less invasive than TDL and when compared with continued
drainage they prevent the loss of nutrients. However, complications include shunt occlusion, infection,
skin erosions, pneumoperitoneum, and shunt migration [8,99,100].

The role of transjugular intrahepatic portosystemic shunt (TIPS) in patients with chylothorax and
cirrhosis is discussed below. (See 'Chylous ascites' below.)

Others — Other isolated or anecdotal cases have reported success with the following:

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● Omental flap – One case report described the treatment of a chylous effusion with a
pleuroperitoneal window and omental flap [104]. This procedure promotes chyle resorption by
juxtaposing abdominal omentum between the pleural space and the peritoneum. It may also
prevent chyle from moving in to the pleural space.

● Decortication – Decortication is a surgical approach to producing symphysis of the lung to the


chest wall to obliterate the pleural space and prevent reaccumulation of a chylothorax [105]. We
reserve decortication for patients who have failed prior thoracic duct ligation and pleurodesis.

● Radiation – Radiation for postoperative chylothorax has been proposed based upon the success
in the management of malignant chylothorax and inguinal lymphatic fistulas [106,107].

SPECIAL POPULATIONS

Lymphangioleiomyomatosis — Lymphangioleiomyomatosis (LAM) is complicated by chylous


effusions in about 10 to 20 percent of patients. In conjunction with first line therapies, sirolimus (also
known as rapamycin) can decrease the size of or entirely resolve chylothoraces in this population, the
details of which are discussed separately. (See "Sporadic lymphangioleiomyomatosis: Treatment and
prognosis", section on 'Chylothorax and chylous ascites'.)

Chylous ascites — Patients with chylothorax due to chylous ascites do not respond to pleural
drainage or second line therapies, although drainage is rarely indicated should patients become
severely symptomatic or hypoxemic due to the effusion. Importantly, embolization, disruption, or
ligation of the thoracic duct almost invariably worsens chylous ascites. However, the underlying cause
should be treated, and dietary measures instituted. (See 'Dietary modification' above and 'Treatment
of the underlying condition' above.)

In some cases, when the cause is unknown, a lymphatic interventional expert should be consulted
since some patients may respond to local interventional therapy.

One case report in a patient with chylous ascites and cirrhosis reported successful treatment of
chylothorax with a transjugular intrahepatic portosystemic shunt (TIPS) [108].

Lung transplant — When chylothorax presents after lung transplantation, it is often associated with
LAM. Chylothorax following lung transplant is classically refractory to conservative therapy but may
respond to rapamycin in those with LAM. A spectrum of therapies has been tried, including thoracic
duct ligation, octreotide, and pleurovenous shunting. (See 'Somatostatin and octreotide' above and
'Pleuroperitoneal or pleurovenous shunt' above and "Pleural complications in lung transplantation",
section on 'Chylothorax' and "Sporadic lymphangioleiomyomatosis: Treatment and prognosis",
section on 'Lung transplantation'.)
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SUMMARY AND RECOMMENDATIONS

● Chylothorax is the accumulation of chyle in the pleural space. Chylothorax can be due to several
etiologies, among which malignancy and surgical trauma are the commonest (table 1). (See
'Introduction' above.)

● No algorithm has been universally adopted for the management of patients with chylothorax,
since multiple clinical factors unique to each patient impact therapy including etiology (table 1),
symptoms, local expertise, and rate of chyle accumulation. (See 'General principles' above and
"Etiology, clinical presentation, and diagnosis of chylothorax", section on 'Pathogenesis and
etiology'.)

● For most patients with high volume (ie, >1 L per day) postoperative chyle leaks, our approach is
the following (see 'Management of postoperative chyle leaks' above):

• We suggest early surgical repair of the thoracic duct rather than prolonged conservative
therapy with chest tube drainage and dietary modification (Grade 2C). The rationale for this
approach is that these patients are likely to have major thoracic duct injury which is unlikely
to close spontaneously, rather than leaks that originate from a smaller thoracic duct tributary
which are more likely to undergo spontaneous closure. Under special consideration are
postpneumonectomy patients should avoid pleural drainage (unless mediastinal shift to the
contralateral side is present) and who should proceed directly to surgical ligation of the
thoracic duct.

• While awaiting surgery (typically within the first one to five days), patients are treated with
continued drainage via chest tube thoracostomy and complete bowel rest by the
administration of parenteral nutrition. In addition, we suggest somatostatin/octreotide to
reduce the flow of chyle through the leak (Grade 2C).

● For most patients with low volume (ie, <1 L per day) postoperative chyle leaks or with
chylothorax for nonsurgical reasons (eg, malignancy) our approach is the following (see 'Initial
therapies' above):

• We suggest minimally invasive therapies while the underlying disorder is being addressed
rather than more invasive options (eg, pleurodesis, thoracic duct ligation [TDL]), thoracic
duct embolization/disruption [TDE/TDD]) (Grade 2C). Minimally invasive therapies involve
pleural drainage for symptom control (eg, thoracentesis, tube thoracostomy, indwelling
catheter) and dietary modification (low-fat diet or total parenteral nutrition).

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• While somatostatin/octreotide appears to benefit patients with postoperative chyle leaks,


practice varies with respect to its use nonsurgical chyle leaks. However, a trial for a few
weeks is reasonable.

● For patients receiving conservative therapy, symptoms, chyle output (or rate of reaccumulation)
and chest radiograph and/or computed tomography should be obtained within a few days of
initiating this strategy to assess the response. In the postoperative setting, more aggressive
therapy is typically warranted if a chylothorax persists for more than two weeks despite these
measures, or sooner if the fluid output exceeds 1 L/day for, at most, five consecutive days, or
when severe metabolic or nutrition derangements appear likely to occur. In the nonsurgical
setting or in those with slow accumulation of chyle, waiting for longer periods to note a response
may be more appropriate. Success rates with conservative therapy are variable ranging from 25
to 80 percent with patients who have low-output leaks more likely to respond than those with high
output leaks. (See 'Monitoring the response' above and 'Efficacy' above.)

● For patients with chylothorax who do not respond to or only partially respond to conservative
measures, options include surgical or medical pleurodesis, TDE/TDD, or TDL. Selection of an
interventional modality should be individualized and depends upon the underlying etiology (table
1), available expertise, rate of chyle loss, likelihood of response to the chosen intervention,
interventional risk, and patient preferences. A multidisciplinary team should facilitate decision
making. In general, the following principles of management are reasonable (see 'Patients who fail
medical therapy' above):

• For patients with postoperative chyle leaks who fail medical therapy, TDL is generally
preferred. TDE/TDD is appropriate for patients who are poor candidates for TDL, provided
expertise is available. For those who undergo TDL, pleurodesis may be simultaneously
performed since this combined approach may offer the best success. (See 'Selecting a
modality' above.)

• For nonsurgical patients who fail medical therapy, invasive therapy should be individualized.
For example, those who drain <500 mL/day may be suitable for pleurodesis alone, while
those who drain >500 mL may be more suited for TDL with or without pleurodesis. Patients
with chyle leak from malignancy are often treated with pleurodesis alone, since TDL is not
generally successful in this population. Patients who have a patent and intact thoracic duct
on lymphatic imaging and have chylothorax from abnormal retrograde lymphatic flow or
lymphatic vessel malformations that direct flow to the lungs may be suited to embolization of
retroperitoneal lymphatics rather than TDL. For those with lymphatic masses (which often
overproduce lymph), embolization of the masses is also appropriate. (See 'Modality options'
above.)

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• Less well established options, including pleurovenous and pleuroperitoneal shunting, may
be of value in those who are poor candidates for TDL or TDE/TDD or in those who have
chylothorax refractory to invasive therapies. (See 'Less well established options' above.)

● Populations that require special consideration include patients with lymphangioleiomyomatosis,


who should be treated with sirolimus, patients with chylous ascites who should avoid chest tube
drainage or invasive therapies such as pleurodesis, TDL, or TDE/TDD which can worsen
chylothorax, and lung transplant patients who need individualized attention. (See 'Special
populations' above.)

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GRAPHICS

Etiology of chylothorax

Number Number
Etiology of cases* [1] of cases ¶[2]
(percent) (percent)

Total nontraumatic PLUS traumatic cases 191 (100) 203 (100)

Nontraumatic

Malignant neoplasia

Lymphomatous 70 (37) 23 (11)

Nonlymphomatous (eg, primary lung, mediastinal, metastatic 17 (9) 11 (5)


extrathoracic malignancies, sarcoma, leukemia)

Total malignant neoplasia cases = 87 (46) 34 (17)

Nonmalignant

Idiopathic 26 (14) 13 (6)

Miscellaneous (benign tumors, lymphangioleiomyomatosis, 25 (13) 55 (27)


intes nal lymphangiectasis, protein-losing enteropathy, regional
ilei s, pleuri s, cirrhosis, thoracic aor c aneurysm, lupus,
tuberculosis, sarcoidosis, amyloidosis, venous thrombosis,
mitral stenosis, nephrosis, thyroid goiter, tuberous sclerosis,
filariasis, heart failure, Down syndrome, Noonan syndrome,
other diagnoses* (please refer to UpToDate text for addi onal
causes)
Total nonmalignant cases = 51 (27) 68 (33)

Total nontraumatic cases = 138 (72) 102 (50)

Traumatic

Surgical (cardiovascular, aortic, thoracoplasty, esophagectomy, 48 (25) 97 (48)


lobectomy, pneumonectomy, mediastinal mass resection, Bochdalek
herniorrhaphy, transabdominal vagotomy, central venous
catheterization, esophageal endoscopic sclerotherapy, neck surgery,
spine surgery, embolization for pulmonary arteriovenous
malformations, pacemaker insertion)

Nonsurgical (penetrating or nonpenetrating trauma to the neck, 5 (3) 4 (2)


thorax, and upper abdomen, straining, coughing, yawning, vomiting)

Total traumatic cases = 53 (28) 101 (50)

* The Valentine et al case series [1] did not provide diagnoses for 10 patients so these cases were categorized in the table as
"other diagnoses" in the Miscellaneous category.
¶ The lower incidence of chylothorax related to lymphoma in the earlier Doerr series [2], compared with the older Valentine
series [1], likely relates to the earlier diagnosis of lymphoma and prompt initiation of effective therapy, prior to development
of chylothorax.

References:
1. Valentine VG, Raffin TA. The management of chylothorax. Chest 1992; 102:586.
2. Doerr CH, Allen MS, Nichols FC 3rd, Ryu JH. Etiology of chylothorax in 203 patients. Mayo Clin Proc 2005; 80:867.

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Anatomy of the thoracic duct

Course of the thoracic duct. Although wide anatomic variation exists, in most
patients (40 to 60 percent), the left thoracic duct ascends from the cisterna chyli, which
is a sac located just anterior to the first or second lumbar vertebra and which receives
drainage from the intestinal and two lumbar lymphatic trunks. The thoracic duct passes
through the aortic hiatus of the diaphragm into the posterior mediastinum continuing
cephalad between the aorta and azygos vein until approximately the level of the fifth
thoracic vertebra where it passes behind the esophagus. Below the fifth thoracic
vertebra, the thoracic duct is commonly a dual or plexiform duct but it becomes a single
2 to 3 mm duct above that level. The thoracic duct continues cephalad adjacent the
esophagus passing posterior to the aortic arch and left subclavian artery. It then arches
over the subclavian artery descending to empty either as a single (50 percent) or
multiple lymphatic channels into the left subclavian vein near its confluence with the left

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internal jugular vein. A one-way valve at this location prevents blood from entering the
thoracic duct. The right lymphatic duct drains into the right subclavian vein.

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Anatomy of central lymphatic circulation view

Anatomic variants of the thoracic duct. In up to 40 percent of patients, two thoracic ducts
(see above) and rarely three thoracic ducts) exist through the entirety of the mediastinum
(see above). Extensive lymphatic tributaries to the thoracic duct and anastomoses course
through the posterior mediastinum adjacent to the heart and major thoracic blood vessels

a: artery; v: vein.

Modified from: Brotons ML, Bolca C, Fréchette E, Deslauriers J. Anatomy and physiology of the
thoracic lymphatic system. Thorac Surg Clin 2012; 22:139.

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Contributor Disclosures
John E Heffner, MD Nothing to disclose V Courtney Broaddus, MD Nothing to disclose Geraldine Finlay,
MD Consultant/Advisory Boards: LAM Board of directors, LAM scientific grant review committee for The LAM
Foundation.

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must conform to
UpToDate standards of evidence.

Conflict of interest policy

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