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THE AMERICAN JOURNAL OF GASTROENTEROLOGY

2002 by Am. Coll. of Gastroenterology


Published by Elsevier Science Inc.

Vol. 97, No. 8, 2002


ISSN 0002-9270/02/$22.00
PII S0002-9270(02)04268-5

CLINICAL REVIEWS

Chylous Ascites
Andres Cardenas, M.D., and Sanjiv Chopra, M.D.
Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Harvard Medical
School, Boston, Massachusetts

INTRODUCTION
Chylous ascites is the accumulation of a milk-like peritoneal
fluid rich in triglycerides, due to the presence of thoracic or
intestinal lymph in the abdominal cavity. It develops when
there is disruption of the lymphatic system due to traumatic
injury or obstruction (from benign or malignant causes). In
Western countries abdominal malignancy and cirrhosis account for over two thirds of all cases. In contrast, infectious
etiologies such as tuberculosis are responsible for the majority of cases in developing countries. Other causes of
chylous ascites include congenital, inflammatory, postoperative, traumatic, and miscellaneous disorders. Congenital
abnormalities of the lymphatic system and trauma should be
considered as etiologic factors in children. Diagnosis of
chylous ascites can be readily made. The presence of a
milky and creamy ascitic fluid with a triglyceride content
above 200 mg/dL makes the diagnosis of chylous ascites. In
a cirrhotic patient, unless there is a strong suspicion of
malignancy, unnecessary, expensive and invasive diagnostic
modalities to rule out a malignant process should be
avoided. The management of patients with chylous ascites is
based on identifying and treating the underlying cause.
Nutritional support with a low-fat diet and medium-chain
triglyceride supplementation is of paramount importance.
Therapeutic paracentesis and diuretics are recommended in
patients with underlying cirrhosis.

DEFINITION
Chylous ascites is defined as the extravasation of milky or
creamy appearing peritoneal fluid rich in triglycerides,
caused by the presence of thoracic or intestinal lymph in the
abdominal cavity (1).
In 1627, Asellius first described the lymphatic system in
a dog after observing vessels in the mesentery containing a
white milky fluid. Morton, in 1691, performed a paracentesis in an 18-month boy with disseminated tuberculosis and
noted the presence of chylous ascites (2). Trauma was
recognized as the most common cause in the 17th century.
However, subsequently, other disease processes such as
malignancies, chronic liver disease, and a wide range of
inflammatory causes have come to be recognized as major
causes of chylous ascites (35).
Chylous ascites is an uncommon finding with a reported

incidence of approximately one in 20,000 admissions at a large


university-based hospital over a 20-yr period (4). Nonetheless,
it is believed that the incidence has increased because of more
aggressive cardiothoracic and abdominal surgery and because
of the longer survival of patients with cancer.

ANATOMY AND COMPOSITION OF LYMPH


The lymphatic system is an accessory route by which fluids
and protein can flow from the interstitial spaces to the
vascular system. Almost all tissues of the body have lymphatic channels composed of one-way valves that drain the
excess fluid from the interstitial spaces of tissues. Lymph
from the lower part of the body drains into the thoracic duct.
This duct arises from the cisterna chyli, which lies between
the aorta and the inferior vena cava anterior to the bodies of
the first and second lumbar vertebrae. The thoracic duct
passes through the aortic hiatus of the diaphragm to enter the
posterior mediastinum. It continues between the aorta and
azygous vein until it reaches the fifth thoracic vertebrae. At
this point, it crosses to the left to enter the superior mediastinum behind the aortic arch and empties into the venous
system at the juncture of the internal jugular vein and
subclavian vein (5, 6).
Lymph is composed of fluid from the interstitial space
that contains protein, bacteria, and lipids in the form of
chylomicrons. Approximately half of the total body lymph
originates in the gut and liver. In the gut, long chain triglycerides are converted into monoglycerides and free fatty
acids and absorbed as chylomicrons. This explains the high
content of triglycerides and the milky and cloudy appearance of lymph (6).

PATHOPHYSIOLOGY
The underlying mechanisms for the formation of chylous
ascites are related to disruption of the lymphatic system,
which may occur because of traumatic injury or obstruction.
The cause can be benign or malignant. Three basic mechanisms have been proposed (2):
1. Primary lymph node fibrosis caused by malignancy obstructing the flow of lymph from the gut to the cysterna
chyli, resulting in leakage from the dilated subserosal
lymphatics into the peritoneal cavity. The chronic effects
of increased pressure of the lymphatic system can also

AJG August, 2002

result in collagen deposition of the basement membrane


of lymphatics with further impairment of the absorptive
capacity of the gut mucosa. Consequently, a proteinlosing enteropathy with chronic diarrhea (steatorrhea),
malabsorption, and malnutrion may ensue.
2. Exudation of lymph through the walls of retroperitoneal
megalymphatics, which leak fluid through a fistula into
the peritoneal cavity (i.e., congenital lymphangiectasia).
3. Dilated retroperitoneal lymphatic vessels (megalymphatics) caused by acquired thoracic duct obstruction from
trauma, resulting in direct leakage of chyle through a
lymphoperitoneal fistula.
Finally, cardiovascular disease (as observed in constrictive
pericarditis, right-sided heart failure, and dilated cardiomyopathy) may precipitate chylous ascites (7, 8). Increased
caval and hepatic venous pressures cause a large increase in
the production of hepatic lymph. Cirrhosis also causes an
increased formation of hepatic lymph. Indeed, decompression of the portal vein in patients with portal hypertension
has been shown to relieve lymphatic hypertension (9, 10).

ETIOLOGY
There are multiple causes of chylous ascites. The most
common causes in Western countries are abdominal malignancy and cirrhosis, which account for over two thirds of all
cases. In contrast, infectious etiologies, such as tuberculosis
and filariasis, account for the majority of cases of chylous
ascites in Eastern and developing countries. Other causes of
chylous ascites include congenital, inflammatory, postoperative, traumatic, and miscellaneous disorders (Table 1).
Malignancy
Malignancy is a common cause of chylous ascites in adults,
and lymphoma accounts for at least one half to one third of
the cases according to a large series followed for 20 yr (4).
Obstruction and invasion into the lymphatic channels cause
chylous ascites by disruption of the normal flow of lymph.
Other neoplastic causes include breast, pancreatic, colon,
renal, testicular, ovarian, and prostate cancer as well as
Kaposis sarcoma, carcinoid tumors, and lymphangiomyomatosis (4, 5). In patients with chylous ascites and secretory
diarrhea, carcinoid tumors should be excluded.
Cirrhosis
Cirrhosis is another cause of chylous ascites. It may be
present in 0.51% of patients with cirrhosis and ascites
(9 12). In a study analyzing 901 samples of ascitic fluid,
there were 11 cases of chylous ascites; of them, 10 cases
were found to be secondary to cirrhosis, and there was only
a single case of malignant ascites (11). Chylous ascites may
be a presenting symptom or can occur later in the disease as
a consequence of hepatocellular carcinoma, after shunt surgery or thoracic duct injury from sclerotherapy (12, 13). For
a cirrhotic patient with chylous ascites, unless clinically
indicated, an aggressive approach to exclude malignancy is

Chylous Ascites

1897

Table 1. Causes of Chylous Ascites


Neoplastic (common in adult population)
Lymphoma
Other cancers (see text)
Lymphangiomyomatosis
Carcinoid tumors
Kaposis sarcoma
Cirrhosis (common in adult population)
Infectious
Tuberculosis
Filariasis (Wuchereria bancrofti)
Mycobacterium avium intracellulare
Congenital (more common in pediatric population)
Primary lymphatic hypoplasia
Yellow Nail Syndrome
Klippel-Trenaunay Syndrome
Primary lymphatic hyperplasia
Bilateral hyperplasia
Intestinal lymphangiectasia
Inflammatory
Radiation
Pancreatitis
Constrictive pericarditis
Retroperitoneal fibrosis
Sarcoidosis
Celiac sprue
Whipples disease
Retractile mesenteritis
Postoperative
Abdominal aneurysm repair
Retroperitoneal node dissection
Catheter placement for peritoneal dialysis
Inferior vena cava resection
Traumatic
Blunt abdominal trauma
Battered Child syndrome
Other causes
Right heart failure
Dilated cardiomyopathy
Nephrotic syndrome

not warranted. The underlying pathophysiology is caused by


the rupture of serosal lymphatic channels, which are dilated
because of excessive lymph flow (up to 20 L/day).
Infectious
Peritoneal tuberculosis and filariasis are common infectious
causes of chylous ascites. Peritoneal tuberculosis occurs
worldwide, in particular in areas of low socioeconomic
status, malnutrition, and poor access to medical care (14).
Lymphatic filariasis is a disease caused by the parasite
Wuchereria bancrofti, which leads to chylous ascites because the parasite causes a severe inflammatory reaction in
the lymphatic vessels resulting in lymphedema and chylous
ascites (15). In patients with AIDS, infection with Mycobacterium avium intracellulare has been reported to cause
chylous ascites (16).
Congenital
Congenital lymphatic abnormalities are more commonly
noted in children with chylous ascites. Primary lymphatic
hypoplasia is a condition seen in childhood and character-

1898

Cardenas and Chopra

ized by lymphedema, chylothorax, and/or chylous ascites


(17). The Klippel-Trenaunay syndrome is an autosomaldominant disorder in which venous and lymphatic hypoplastic malformations cause hypertrophy of all the tissues of
an involved limb and often times, chylous ascites (18). The
Yellow-Nail syndrome is a disorder of unclear etiology seen
in childhood. Patients have hypoplastic lymphatics leading
to chylous effusions. The characteristic triad consists of
lymphedema, pleural effusion, and/or chylous ascites and a
yellow discoloration together with dystrophy of the nails (19).
Primary lymphatic hyperplasia has also been recognized
as cause of chylous ascites (20). There are two forms:
bilateral hyperplasia in which the lymphatics are not severely dilated and contain valves, and lymphangiectasia
(megalymphatics) in which lymphatics are very dilated and
lack valves.
Inflammatory
Chylous ascites can develop after radiation therapy to the
abdomen, which can cause fibrosis and obstruction of the
lymphatic vessels in the small bowel and mesentery (21).
Pancreatitis (acute or chronic) by causing compression of adjacent lymphatic channels can result in chylous ascites and
pleural effusions (22). Constrictive pericarditis, which can increase the hepatic venous pressure, can cause chylous ascites
(8). Although rare, chylous ascites resulting from constrictive
pericarditis is a reversible and treatable cause of ascites. Other
rare inflammatory causes include idiopathic retroperitoneal fibrosis (Ormonds disease), sarcoidosis, retractile mesenteritis,
celiac sprue, and Whipples disease (2327).
Postoperative and Traumatic
Chylous ascites can occur early (around 1 wk) after abdominal surgery because of disruption of the lymphatic vessels,
or late (several weeks to months) because of adhesions or
extrinsic compression of lymphatic vessels. The surgical
procedures that can result in chylous ascites include aortic
and abdominal aneurysm repair, retroperitoneal lymph node
dissection, inferior vena cava resection, catheter implantation for peritoneal dialysis, distal splenorenal shunts, and
liver transplantation (28 32).
Blunt abdominal trauma resulting in intestinal and/or
mesenteric injury is also a cause of chylous ascites (33). In
children, the Battered Child syndrome, which accounts for
approximately 10% of cases of chylous ascites in the pediatric population, should be excluded (34).
Other Causes
Right heart failure and dilated cardiomyopathy can also cause
impaired drainage of lymph, with lymphatic hypertension producing stasis, dilation, and chylous ascites (7). The nephrotic
syndrome has also been reported to cause chylous ascites and
chylothorax (35, 36). The underlying pathogenesis is not understood. In one report of 140 patients with nephrotic syndrome, 90 had ascites and of those, 35 underwent paracentesis.
Sixteen patients (52%) had chylous ascites defined by the
presence of milky white ascitic fluid. However, it should be

AJG Vol. 97, No. 8, 2002

noted that the triglyceride levels were not measured, and some
patients may have had just opalescent effusions (37).

EVALUATION OF PATIENTS WITH CHYLOUS ASCITES


Clinical Findings
As in any patient who presents with ascites, a careful history
and physical examination should be performed. Careful questioning regarding weight loss or gain, symptoms of malignancy, family history, recent abdominal surgery, travel, abdominal trauma, and underlying liver or kidney disease should
be done. Constitutional symptoms such as anorexia, weakness,
and malaise are very common, but are nonspecific.
Chylous ascites frequently presents as progressive and painless abdominal distention, which occurs over the course of
weeks to months depending on the underlying cause. Nonetheless, patients who have undergone abdominal or thoracic
surgery may present with an acute onset of chylous ascites.
Patients may complain of weight gain and of shortness of
breath and dyspnea resulting from increased abdominal pressure. Other features include nonspecific abdominal pain,
weight loss, diarrhea and steatorrhea, malnutrition, edema,
nausea, enlarged lymph nodes, early satiety, fevers, and night
sweats (2, 4, 5). Most often, the diagnosis of chylous ascites is
not considered before performing a paracentesis.
Findings that may be present on physical examination
include ascites, pleural effusions, lower extremity edema,
lymphadenopathy, cachexia, temporal wasting, abdominal
masses, and hernias. In cirrhotic patients, stigmata of
chronic disease such as jaundice, palmar erythema, spider
angiomata of the chest, and encephalopathy may be present.
Paracentesis and Laboratory Data
Abdominal paracentesis is the most important diagnostic
tool in evaluating and managing patients with ascites. Typically, chyle has a cloudy and turbid appearance, in contrast
to the straw-colored and transparent appearance of ascites
caused by cirrhosis and portal hypertension. Ascitic fluid
should be sent for cell count, culture, Grams stain, total
protein concentration, albumin, glucose, LDH, amylase, triglyceride level, and cytology (38, 39). Tuberculosis smear,
culture, and adenosine deaminase should be performed in
selected cases when tuberculosis is suspected. The utility of
adenosine deaminase measurement in populations with a
high prevalence of cirrhosis such as the United States,
however, may be limited (40). The total protein content in
chylous ascites varies depending on the underlying cause
and ranges between 2.5 to 7.0 g/dL (41) (Table 2).
The triglyceride levels in ascitic fluid are very important
in defining chylous ascites. Triglyceride values are typically
above 200 mg/dL, although some authors use a cutoff value
of 110 mg/dL (1, 4, 41, 42). In some patients with cirrhosis,
the appearance of ascites may be opalescent but not chylous,
and in most cases, it is caused by low levels of triglycerides,
although cellular damage from infections or malignancy
may contribute to its color (43).

AJG August, 2002

Table 2. Characteristics of Ascitic Fluid in Chylous Ascites


Color
Triglyceride level
Cell count

Milky and cloudy


Above 200 mg/dL
Above 500 (predominance of
lymphocytes)
Total protein
Between 2.57.0 g/dL
SAAG
Below 1.1 g/dL*
Cholesterol
Low (ascites/serum ratio 1)
Lactate dehydrogenase Between 110200 IU/L
Culture
Positive in selected cases of tuberculosis
Adenosine deaminase Elevated in cases of tuberculosis
Cytology
Positive in malignancy
Amylase
Elevated in cases of pancreatitis
Glucose
Under 100 mg/dL
IU international units; SAAG serum-ascites albumin gradient.
* Is elevated above 1.1 g/dL in chylous ascites secondary to cirrhosis.

Standard blood tests including a complete blood count,


electrolytes, liver tests, total protein, albumin, LDH, triglycerides, cholesterol, amylase, and lipase should be performed (38, 39). Also, the serum to ascites albumin gradient
should be calculated by subtracting the ascitic fluid value of
albumin from the serum value to determine if the ascites is
related to portal hypertension or other causes (11, 38) (Table 2).
Radiology
CT of the abdomen is useful in identifying pathological intraabdominal lymph nodes and masses. In the setting of postoperative or traumatic causes of chylous ascites, it also helps in
determining the extent and localization of fluid, in particular, if
there is a suspicion of thoracic duct injury. Other radiological
studies such as lymphangiography and lymphoscintigraphy
help in detecting abnormal retroperitoneal nodes, leakage from
dilated lymphatics, fistulization, and patency of the thoracic
duct. Lymphangiography is the gold standard in defining cases
of obstruction, but has several complications related to the
volume and type of contrast used. These tests may be useful in
selecting patients for surgery (44).

MANAGEMENT
There are limited numbers of studies addressing the best treatment regimens. Most chylous effusions respond to an initial
approach with a high-protein and low-fat diet with mediumchain triglycerides. Dietary restriction of long-chain triglycerides avoids the conversion of these into monoglycerides and
free fatty acids, which are transported as chylomicrons to the
intestinal lymph ducts. In contrast, medium-chain triglycerides
are absorbed directly into intestinal cells and transported as free
fatty acids and glycerol directly to the liver via the portal vein.
The use of a low-fat diet with medium-chain triglyceride supplementation therefore reduces the production and flow of
chyle (45, 46). Patients with cirrhotic chylous ascites should be
managed with a low-sodium diet and diuretics such as spironolactone (39).
Patients who do not respond to the above measures should
be fasted to reduce lymph flow and started on total parenteral alimentation. Somatostatin and octreotide have been

Chylous Ascites

1899

successfully used to treat chylous effusions in patients with


the Yellow Nail syndrome and lymphatic leakage caused by
abdominal and thoracic surgery. It has been speculated that
somatostatin improves chylous ascites by inhibition of
lymph fluid excretion through specific receptors found in the
normal intestinal wall of lymphatic vessels (47, 48).
Treatment of the underlying cause of chylous ascites is of
pivotal importance in managing patients with chylous ascites. In most cases, correction of the underlying pathology
will result in resolution of symptoms and of the ascites. This
is particularly true of patients who have an infectious, inflammatory, or hemodynamic cause.
Surgery may benefit patients with postoperative, neoplastic, and congenital causes (5). Before surgery, a lymphangiogram or lymphoscintigraphy is helpful in identifying the site of leakage or the presence of a fistula.
In patients with a large amount of ascites, a total paracentesis to relieve abdominal discomfort and dyspnea
should be performed and repeated as needed. Replacement
of intravascular volume with albumin to prevent postparacentesis circulatory dysfunction is not necessary unless the
patient has underlying cirrhosis. Repeated large-volume paracentesis is a reasonable option for patients who have end-stage
disease not amenable to medical or surgical treatment.
In patients who are poor surgical candidates and refractory to nonoperative treatment, peritoneovenous shunting
may be an option. Unfortunately, these shunts carry a high
rate of complications including sepsis, disseminated intravascular coagulopathy, electrolyte imbalance, small bowel
obstruction, and risk for air embolism. In addition, the high
viscosity of the chyle results in a high rate of shunt occlusion in the majority of cases (49, 50).
In summary, chylous ascites is a relatively uncommon
disorder. Malignancy and cirrhosis are the leading causes of
this condition in adults. Congenital abnormalities of the
lymphatic system and trauma should be considered as etiological factors in children. Diagnosis of chylous ascites can
be readily made with simple tests. In a cirrhotic patient,
unless there is a strong suspicion of malignancy, unnecessary, expensive, and invasive diagnostic modalities to rule
out a malignant process should be avoided. Finally, treating
the underlying cause is of paramount importance in the
management of these patients.
Reprint requests and correspondence: Sanjiv Chopra, M.D., Liver
Center, Division of Gastroenterology, 110 Francis Street, Suite 8E,
Beth Israel Deaconess Medical Center, Boston, MA 02215.
Received Nov. 20, 2001; accepted Mar. 4, 2002.

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