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British Journal of S u q y y 1997, 84, 15-20

Review

Chylothorax
B . A . M E R R I G A N , D . C . W I N T E R and G . C . O ' S U L L I V A N
Depurtment of S i i t p y , Mercy Hospital and Universiy College Cork, Cork, Ireland
Correspondence to: Mr G. C . O'Sullivun, Department of Surgery, Mercy Hospital, Grenville Place, Cork, Ireland

Background Chylothorax is a condition that is debilitating to the point of threatening life. There is
controversy over its management, in particular the relative merits of conservative measures and the
timing of surgical intervention.
Method The literature is reviewed from the basic sciences of chyle composition and flow, to
diagnostic approaches, the complications of chyle loss and appropriate management strategies.
Results and Conclusion Prompt diagnosis is essential to institute an effective therapeutic regimen.
Surgery achieves fast, safe and effective reversal of this dire situation. Minimally invasive
thoracoscopic techniques are gaining wide recognition. Early intervention, which should be
aggressive and complete to avoid the immune and nutritional consequences of extended chyle
depletion, is recommended.
Chylothorax is a debilitating condition to the point of
threatening life. Diagnosis and subsequent management
present significant problems for the clinician, and the
adverse effects of chyle loss on nutritional status and
immune function result in devastating consequences for
the patient. This paper reviews the literature from the
basic sciences of chyle composition and flow, to diagnostic
approaches, the complications of chyle loss and
appropriate management strategies. Prompt diagnosis is
essential to institute an effective therapeutic regimen.
Early intervention is recommended, which should be
aggressive and complete to avoid extended chyle
depletion. Surgery achieves fast. safe and effective
reversal of this dire situation.

of the fifth or sixth thoracic vertebra. It then climbs


posterior to the aortic arch and, having passed anterior to
the subclavian artery, exits via the superior thoracic
aperture. In the neck, the duct forms an arch anterior to
the scalenius anterior muscle at the level of the seventh
cervical vertebra by turning laterally and downwards to
terminate at the junction of the left subclavian and
internal jugular veins (Fig. 1). The thoracic duct displays

Anatomy of the thoracic duct


The thoracic duct was first described in 1651 by Pecquet'.
Varying in length from 37 to 45 cm, it extends from the
abdomen to the root of the neck. It begins in the
abdomen at the body of the second lumbar vertebra as a
triangular dilatation called the cisterna chyli and ends at
its junction with the left subclavian vein, into which it
drains. The vessel transmits intestinal chyle to the
bloodstream and drains the lymphatics of the body, except
for the right side of the head and neck, right upper limb,
right lung, right side of the heart and the convex surface
of the liver. The cisterna chyli is situated anterior to the
second lumbar vertebra, posterior and lateral to the aorta.
From thence the thoracic duct ascends into the thorax
passing posterior to the median arcuate ligament of the
diaphragm between the azygous vein and the aorta. This
is the favoured site for elective ligation as it tends to be
constant, whereas the course in the thorax may display
considerable variability'. The ascent through the thorax is
in the posterior mediastinum, immediately anterior to the
vertebral column to the right of the aorta and posterior to
the oesophagus, with which it has a complex relationship.
It begins to the right of the distal oesophagus, but crosses
to the left in order to reach the opposite side at the level

Paper accepted 3 September 1YOh

0 1997 Blackwell Science Ltd

Fig. 1 Anatomy of the thoracic duct showing area of low ligation


between the azygous vein and the aorta

15

16 B. A . M E R R I G A N , D . C . W I N T E R and G . C . O ' S U L L I V A N
Table 1 Features of lymph fluid

considerable variability. There may be a division into two


or more branches in more than 40 per cent of
individual^'^"^. These branches may form a plexiform
arrangement in the middle of the course of the duct and
may terminate separately or as one duct5. Occasionally,
the duct divides into two branches in its upper portion
which do not rejoin but drain separately, one in the usual
fashion and the other reaching the right subclavian vein.
The histological structure comprises of three coats6.
The external coat and middle layer are formed by elastic
and muscle fibres with surrounding areolar tissue.
Flattened endothelial cells and a subendothelial plexus of
arterioles in a bed of longitudinal elastic fibres form the
innermost layer. There are numerous valves along the
lumen of the duct, numbering between eight and ten,
2-3 cm apart through its lower course but becoming more
frequent in the neck6. A large bicuspid valve near the
lymphaticovenous junction prevents downward reflux of
blood, which ebbs and flows with respiration in the
terminal portion of the duct5.

Relative density
PH
Colour

Physiology of chyle flow


The thoracic duct transports up to 4 litres of chyle per day
in a healthy adult. Quantities vary from 10 to more than
100ml per kg body-weight, depending on diet, drug
intake, intestinal function and degree of mobility*. The
hydrostatic pressure of the chylous system is between 10
and 25 cmH207owing to the effects of intraluminal forces
(the column of chyle), intramural muscle activity and
extramural pressures (e.g. local arterial pulsation).
Flow rate is dependent on the inflow from the lacteal
system (vis a tergo). Propulsion of the duct content is
effected by contraction of the muscular wall2 at intervals
of 13-14s. Cholinergic stimulation via the vagus nerve
may further increase the tone of the wall. Flow may be
increased by compression of the cisterna chyli during
periods of raised intra-abdominal pressure, for example
when coughing2. Descent of the diaphragm and liver
during inspiration has a similar effect, whereas negative
intrathoracic pressure exerts a vis a fronte. This combined
extraluminal pressure gradient enhances transmission and
unidirectional valves prevent backflow. Drainage at the
lymphaticovenous junction is encouraged by the Bernoulli
suction effect of the flowing blood.
Some 95 per cent of transmitted lymph is derived from
the gastrointestinal tract and the liver; the remainder
originates in skeletal tissue. It therefore contains a
combination of lymphatic and gut-derived substances,
including lymphocytes, immunoglobulins, enzymes and
products of digestion. Chyle contains fat from the
intestinal lacteal system, which gives a characteristic milky
appearance' and allows a helpful and simple bedside test
for thoracic duct damage. Sixty to 70 per cent of absorbed
dietary fat passes through this system at a concentration
of 5-30g/l. This is mainly as chylomicrons (short-chain
fatty acids are absorbed directly into the portal system).
Therefore triglycerides, cholesterol and fat-soluble
vitamins (A, D, E and K) are found in chyle. The
concentration of triglycerides is higher than that of
plasma, a useful diagnostic aid in the differentiation of
chylous pleural effusions from those of other origin*.
The thoracic duct plays a role in fluid homoeostasis and
is the main conducting vessel for the return of extravasated proteins to the circulation. This is particularly
important in transferring depot proteins following
haemorrhage2. The normal protein content is between 20

Sterile
Bacteriostatic
Fat (g/9
Protein (g/l)
Albumin
Globulin
Albumin :globulin ratio
Fibrinogen (mg/l)
Glucose (mmol/l)
Urea (mmol/l)
Cell count (per dl)
Lymphocytes?
Erythrocytes
Enzyme concentration (units/ml)
Pancreatic lipase
Amylase
Aspartate aminotransferase
Alanine aminotransferase
Alkaline phosphatase
Acid phosphatase
Electrolyte concentration (mMol/l)
Sodium
Potassium
Chloride
Calcium
Phosphate

1.012-1'015
7.4-7.8
Milky (colourless in
starvation)
Yes
Yes
5-30*
20-30
12-42
11-31
3: 1
160-240
2.7-1 1.1
1.4-3.0
40 000-680 000
5000-60000
0.5-2.4
50-83
22-40
5-21
2-4.8
0.3-0.8
104-108
3.8-5.0
85-130
3.4-6'0
0'8-4'2

*Triglyceride level is greater than in plasma, cholesterol


concentration is less than in plasma; cholesterol :triglyceride
ratio is less than 1; lymph fluid also contains cholesterol esters,
free fatty acids, sphingomyelins, phospholipids and neutral fats as
chylomicrons, as well as fat-soluble vitamins. $Form 95 per cent
of cellular content, 90 per cent as T cells

and 30g/l, with an albumin to globulin ratio of


approximately 3 : 1. Other proteinaceous components
include fibrinogen and the enzymes amylase, lipase and
alanine aminotransferase. Urea, glucose and electrolytes
are present at detectable levels, although the quantities do
not represent plasma approximation in all cases (Table 1 ) .
The sodium concentration is usually lower than normal
plasma values.

Effects of chyle loss


The adverse effects depend on the amount, rate and
duration of chyle loss. There may be a period of
asymptomatic loss, which varies according to the
magnitude of the leak, size of the pleural cavity and
management of the patient. For example, the
asymptomatic period is prolonged in a fasting patient,
particularly if the effusion is straw-coloured9 and diagnosis
is delayed. Eventually there is compromise of cardiorespiratory, metabolic and immune functions.
The results of chyle loss can be summarized into local,
metabolic and immunological effects. The local effects of
a chyle leak into the pleural cavity include compression of
the ipsilateral lung and, if sufficiently voluminous,
mediastinal shift which compromises the contralateral
lung and impairs cardiac function. Hypovolaemia from
continued fluid loss may be compounded by hypoproteinaemia, which results in transcapillary fluid shifts.
This plasma protein depletion further compounds the

0 1997 Blackwell Science Ltd, British Journal of Surgery 1997,84, 15-20

CHYLOTHORAX

problems of accurate fluid replacement and maintenance


of the intravascular volume. During prolonged chyle loss,
the bodys reserves of protein (in particular albumin), fat,
vitamins and electrolytes are depleted. Hyponatraemia,
acidosis and hypocalcaemia1 are the most commonly
recognized phenomena. Patients require nutritional
support because there is increased metabolic demand
from the combination of chyle loss and hypermetabolism
associated with surgery, trauma or intercurrent sepsis.
Both cell-mediated immunity, as evidenced by negative
intradermal skin testing, and humoral responses to
antigen provocation are impaired by prolonged chyle
drainage. Lymphocytes, in particular the T subclass, form
the bulk of the cellular content of chyle. This attracted
clinicians of the early organ transplant years to the
procedure of thoracic duct drainage in order to effect
immunosuppression~~-14. Prolonged
(intermittent)
drainage depletes the T cell population and impairs cellmediated immune responses, which is of benefit in the
suppression of transplant rejection states. However, the
number of B lymphocytes increases during periods of
prolonged chyle loss such that the absolute lymphocyte
count in lymph fluid remains relatively static.lS.This may
indicate a compensatory mechanism rather than a
generalized proliferative stimulus. Hypoalbuminaemia and
lymphopenia in conjunction with an altered immune
response, including lowered antibody levelsL6,increase the
risk of bacterial and viral sepsis. Despite normal
complement levels and observed granulocyte functionI8,
y-globulin and isoagglutinin levels are reduced in affected
patients16.

17

Table 2 Causes of chylothorax

Congenital
Atresia of the thoracic duct
Trauma at birth
Trauma
Iatrogenic
Surgery
Cervical
Thoracic
Abdominal
Therapeutic and diagnostic procedures
Central venous cannulation
Translumbar arteriography
Oesophageal sclerotherapy
Non-iatrogenic
Blunt
Penetrating
Intrinsic
Neoplasm
Venous thrombosis
Pulmonary lymphangiomatosis
Extrinsic
Neoplasm
Lymphoma
Metastatic disease
Lymphadenitis
Infection
Spontaneous

Aetiology of chylothorax
Chylothorax may be classified according to aetiology as
congenital, traumatic or obstructive (Table 2). Congenital
chylous effusions are extremely rare and the true
incidence is difficult to document. They are usually
idiopathic but may be associated with Downs and
Noonans syndromes, tracheo-oesophageal fistula and
polyhydramni~s~.
There may be a history of birth trauma,
but the underlying defect is congenital. There may be
complete atresia of the thoracic duct or absence of
communications between small peripheral lymphatics and
the larger central vessels as a result of hypoplasia.
Traumatic damage to the thoracic duct is largely
iatrogenic in nature. Unrecognized section or laceration
of the duct during surgical procedures on the lung,
oesophagus, aortic isthmus or mediastinal tumours has
been described. The occurrence of chylothorax has most
often been described following oesophageal resection
(Table 3)9,20-26.
Increased rates are reported for the transhiatal technique in comparison with the thoracic
approach9. Unusual and unexpected cases of iatrogenic
chylothorax exist, for example following repeated
sclerotherapy for oesophageal ~ a r i c e s ~Extrathoracic
~.
procedures, such as radical neck dissection, may also
result in thoracic duct damage. It is a rare complication of
central venous cannulation where it is associated with
subclavian vein thrombosis, is usually bilateral and
frequently
Approximately 20 per cent of cases of traumatic
chylothorax are due to non-iatrogenic causes29.
Penetrating injury to the neck, chest or abdomen may
directly lacerate the duct. Blunt trauma, for example crush
injury, or sudden forceful hyperextension of the spine may
result in ductal damage. In this circumstance the duct is
0 1997 Blackwell Science Ltd, British Journal of Surgery 1997,84, 15-20

Table 3 Reported incidence of chylothorax in oesophageal


resections
Year

No. of
resections

Incidence

Reference
SkinnerZn
Hankins et al.z
King et al. 22
Orringer et aLZ3
Woods et aLZ4
Tam et al. 25
Bolger el aL
Dougenis et aLZh

1983
1987
1987
1988
1989
1989
1991
1992

80
26
100
320
50
316
537
255

2
4
1.0
34
2
0.6
2.0
3.9

(%)

particularly vulnerable when distended after a meal. The


so-called spontaneous chylothorax is an unusual entity in
that the degree of trauma is minimal. There appears to be
a predisposition of the duct to damage in these cases, due
either to increased wall tension secondary to valvular
incompetencelo or to increased fixity owing to previous
infective, inflammatory or neoplastic insult. Sudden
increase in intraductal pressure as in coughing can initiate
a leak.
Obstruction of the duct, whether extrinsic or intrinsic,
can result in a chylous pleural effusion, although there
may be effective collateral communications2. lntraluminal
obstruction by neoplastic cells or infective organisms, and
extrinsic obstruction due to lymphadenopathy or local
tumour compression, may occur. Lymphoma accounts for
70 per cent of cases of thoracic duct leakage caused by
t~mours~~,~~.

Clinical features and diagnosis


The presentation of the chylous effusion depends on the
aetiology, which falls into two broad categories: iatrogenic
or post-traumatic, and insidious. Signs and symptoms are

18 B . A. M E R R I G A N , D . C. W I N T E R and G . C. O'SULLIVAN
classical of a pleural effusion. The diagnosis in postoperative patients, where the surgical procedure was in
the vicinity of the thoracic duct, should be considered
when there are increased losses on chest drainage or a
recurring pleural collection of fluid. The diagnosis may
not be obvious in a fasting or postoperative patient
because the fluid is often straw-coloured' or bloodstained,
and there are few signs other than low-grade pyrexia7.
Fluid sampling reveals triglyceride and lymphocyte levels
that are higher than corresponding plasma values.
Measurement of plasma protein content may reveal
hypoalbuminaemia secondary to albumin losses associated
with the lymphatic leak. The diagnosis may be confirmed
by administration of cream or other foodstuff of high fat
content by mouth or via the nasogastric tube; this is the
most reliable test in the clinical setting. It induces a
dramatic change in the colour and content of the effused
fluid owing to the transport of absorbed fat in the lacteal
system23.3"32.
Unlike in the normal patient there may not
be an increase in the level of plasma triglycerides after the
high-fat meal'".
Patients with insidious chyle leaks usually present to the
hospital with chest discomfort and dyspnoea, or as an
incidental finding on chest radiography. Confirmatory
investigations may be delayed if the possibility of chylothorax is not considered. Chest radiography, with lateral
views, defines the size of the effusion and allows
assessment Of the clarity of the contralateral lung field.
Bilateral chylous effusions do occur and are associated
with a poor prognosis2'. More complex radiological
investigations may be undertaken to confirm the
pathological diagnosis and to identify the anatomy of the
lesion. Lymphangiography provides useful information
regarding the site and size of the leak""", and enables
differentiation between thoracic duct damage and
anastomotic leaks in the postoperative group. It may also
identify complete transection or partial laceration of the
It is, however, complex and time consuming.
Computed tomography has no additional role in
iatrogenic injuv''. It can be a useful tool when chylothorax is a late presentation after cancer surgery or is
associated with trauma, or where an underlying tumour is
suspected.

Management
There is considerable controversy over the management
of this condition. Some authors advocate a conservative
a p p r ~ a c h - ' ~while
,
others favour early operative intervention2',-''. However, most agree that either approach
must be considered in the light of the specific aetiological
type of chylothorax and the patient's general condition. It
is important not to procrastinate while the condition
deteriorates to a level at which surgery would be
detrimental.
Conservative management involves drainage of the
pleural cavity, measures that reduce chyle flow and
supportive nutrition. The introduction of this protocol has
led to the lowering of mortality rates for post-traumatic
chylothorax from over 50 per cent at the turn of the
.
50 per
century to 10 per cent at p r e ~ e n t ' ~Approximately
cent of congenital and traumatic cases resolve with such
management, but it is difficult to predict when this will
O C C U ~ * . ~ . *There
~.
is some evidence to suggest that failure is
related to increased back pressure from high-pressure
venous states4". Intercostal fluid drainage evacuates chyle,

re-expands the lung and obliterates the potential pleural


space2. Dietary oral fat intake, except medium-chain
triglycerides, is withheld. After large losses or foregut
reconstruction surgery, total parenteral nutrition is the
.~'
preferred method of nutritional s ~ p p o r t ' * . ' ~ . ' ~This,
combined with inhibition of gastrointestinal secretions by
somatostatin analogues, reduces the volume of chyle and
promotes r e s ~ l u t i o n ~ ~Some
. ~ ' . success has been achieved
by substituting dietary fat with medium-chain triglycerides
that bypass the chylous s y ~ t e m ~ ~permitting
-~',
continued
oral diet. This method reduces the quantity and total
duration of chyle loss when compared with a normal
diet46.However, long-term use can result in deficiencies of
linoleic acid, an essential fatty acid present only in longchain triglyceridesl'.
The mortality rate for postoesophagectomy chylothorax
is 50 per cent or more with conservative management9
because patients treated in this way, especially children",
are prone to overwhelming bacterial and fungal sepsis".
Milsom et ~ 1 reported
. ~ ~death in five of six patients of a
group treated conservatively versus one of six having
. ~ ~
operative intervention. Similarly, Orringer et ~ 1 concluded that conservative management was not of benefit
in cases of chylothorax after oesophageal surgery. The
present authors therefore advocate conservative
in
management for a maximum of only 2 weeks2b~3'."~7X.-'y~47
the absence of any indication for surgery (e.g. unrelenting
effusion, postoesophagectomy chylothorax and complications, such as incarcerated lung) (Table 4 ) . Surgery is
indicated when pleural drainage is greater than 1 litre per
day for more than 5
Damage to the thoracic duct is rarely recognized during
operation because the duct is relatively collapsed in the
fasting state and the content blends with serous
collections. Because of this, some surgeons favour elective
ligation of the thoracic duct during radical surgical
procedures in order to prevent chylous fistula. This
significantly reduces the mortality rate from chylothorax
after oesophage~tomy~.
Dougenis et aLZ6 recommended
routine duct ligature, especially with tumours of the midoesophagus. The preferred site for elective ligation is in
the upper abdomen o r lower thorax, where there is more
constant anatomy. In the event that the duct cannot be
identified, mass ligation of the tissue between the aorta
of the
and azygous vein may be p e r f ~ r m e d ~Ligation
~.
thoracic duct may also be carried out in Pokier's triangle
(Fig. 2 ) if the leak is in the upper thorax or neck.
Emergency or non-elective surgical intervention for
established thoracic duct damage was pioneered by
L a m p ~ o nin~ ~1948. The traditional approach is open
thoracic or abdominal ligation'", with a success rate of 95
per cent and little additional m ~ r b i d i t y ~ . ~. .Th
~ ~e ,leak
~~,'~
may then be identified by the characteristic flow of milky
fluid after administering cream orally or via nasogastric
tube before or during operation. The cream is seen to
leak at a rate of one to two drops every second.
Methylene blue staining of the fat meal has also been
used for this purpose but provides little additional
Table 4 Indications for surgery
1 Chyle leak greater than 1 litre per day for more than 5 days
2 Persistent leak for more than 2 weeks despite Conservative

management

3 Nutritional or metabolic complications


4 Loculated chylothorax, fibrin clots or incarcerated lung

0 1997 Blackwell Science Ltd, British Journal ofsurgery 1997, 84, 15-20

CHYLOTHORAX

19

indwelling foreign bodies, such as intercostal drains, make


patients vulnerable to life-threatening opportunistic
infection".
Barrier nursing in isolation rooms,
meticulously sterile procedures, regular sampling for
microbiological culture and avoidance of unnecessary
antibiotics are important measures in the management
protocol.

Conclusion
The morbidity and mortality associated with chylothorax is
considerable. Early surgery should be undertaken in
postoperative cases because the response to conservative
management is poor. It is reasonable to attempt conservative management in an otherwise stable and healthy
patient. However, the application of criteria for surgical
intervention as outlined above is recommended in all
cases to avoid unnecessary delay.

Fig. 2 Arcn f o r high ligation: Pokier's triangle between the


intcrnal carotid artery. arch of aorta and vertebral column

Acknowledgements
Figs 1 and 2 were drawn by Gillian Lee Illustrations.

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Decortication to favour complete lung expansion may be
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Thoracoscopic ligation is reported to cause less
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and there may be
other advantages over open surgery. The traditional
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right thorax, but left-sided collaterals have occasionally
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Successful management of
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0 1997 Blackwell Science Ltd, British Journal of Surgery 1997, 84, 15-20

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