COLLECTIVE REVIEW
Atraumatic Chylous Ascites: Systematic Review on
Symptoms and Causes
Daniel C Steinemann, MD, Daniel Dindo, MD, Pierre-Alain Clavien, MD, FACS, Antonio Nocito, MD
Chylous ascites, or chylaskos, is a rare form of ascites char-
acterized by a milky-appearing fluid containing high levels
of triglycerides. Its incidence ranges from 1 in 20,000 to 1
in 187,000 admissions at large tertiary referral hospitals.1,2
Therapeutic interventions and trauma are well-known
causes of chylous ascites. In a cohort of 1,103 patients
undergoing abdominal surgery, the incidence of postoper-
ative chylous ascites was reported to be 1.1%. When retro-
peritoneal, esophageal, gastric, or cytoreductive surgeries
were performed, the postoperative incidence was even
higher, amounting to 7.4%.3 Apart from oncologic tho-
racic and abdominal surgery,3 chylous ascites can also occur
after abdominal aortic surgery4 and very rarely after donor
nephrectomy.5 In contrast to the direct injury of lymphatic
vessels during surgery, the hyperextension and hyperflexion
of the body during a blunt abdominal trauma entail a rup-
ture of lymphatic vessels, thus causing chylous ascites.6 An
additional cause of indirect injury leading to the develop-
ment of chylous ascites is radiotherapy. Abdominal radia-
tion is thought to induce fibrosis of the lymphatic vessels
within the small bowel and the mesentery, causing obstruc-
tion and subsequent extravasation of chylous.7 In patients
after radiation of the whole abdomen for gynecologic ma-
lignancies, the incidence was reported to be as high as 3%.8
The most common clinical presentation of chylous as-
cites is supposed to be increasing painless abdominal dis-
tension. Less frequently, postinterventional and posttrau-
matic chylous ascites can induce an acute abdomen.7
Although the clinical features and causes of chylous ascites
secondary to surgery or trauma9,10 are familiar to most cli-
nicians, little is known about the incidence, etiology, and
distribution of different clinical symptoms of atraumatic
chylous ascites. The incidental finding of a few milliliters of
chylous ascites during a routine inguinal hernia repair in a
28-year-old man finally leading to the diagnosis of non-
Disclosure Information: Nothing to disclose.
Presented at 97th Annual Meeting of the Swiss Surgical Society, Interlaken,
Switzerland, May 2010.
Received November 30, 2010; Revised January 10, 2011; Accepted January
11, 2011.
From the Department of Visceral and Transplantation Surgery, University
Hospital Zurich, Zurich, Switzerland.
Correspondence address: Antonio Nocito, MD, Department of Visceral and
Transplantation Surgery, University Hospital Zurich, Raemistrasse 100, 8091
Zurich, Switzerland.
© 2011 by the American College of Surgeons
Published by Elsevier Inc.
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Hodgkin B-cell lymphoma prompted us to perform a sys-
tematic review of the literature focusing on the clinical
presentation and underlying causes of atraumatic chylous
ascites.
METHODS
Search strategy
Two electronic databases (PubMed and Cochrane Library)
were searched for the period from January 1990 to Febru-
ary 2010. The following MeSH terms were used: chylascos,
chylaskos, chyloascites, chylous ascites, and chyloperito-
neum. The references of the retrieved articles were cross-
searched. The search was limited to studies in humans pub-
lished in English or German.
Inclusion criteria
The title and abstract of all identified articles were
screened. The publications were included for further anal-
ysis if they reported cases of chylous ascites and contained
information on the underlying etiology and the clinical
presentation. All different types of publications were
included.
Publications dealing with chylous ascites following sur-
gery, radiotherapy, and trauma were excluded. Reports fo-
cusing on the management of chylous ascites without in-
formation on the underlying pathology and clinical
presentation were also excluded. Moreover, review articles
not reporting on previously unpublished cases were
excluded.
Data extraction
Full-text articles of the studies that met all of the inclusion
criteria were retrieved. Data on the following information
were recorded: leading and minor symptoms, number of
patients, age, and underlying diagnosis of chylous ascites.
Statistical analysis
Data were entered into an Excel (version 12.0; Microsoft)
spreadsheet. Descriptive statistics and analysis of signifi-
cant differences were performed using SPSS (version 17;
SPSS Inc). Proportions between 2 groups were compared
using the Fisher exact test. The level of statistical signifi-
cance was set at 0.05.
ISSN 1072-7515/11/$36.00
899 doi:10.1016/j.jamcollsurg.2011.01.010
900 Steinemann et al Atraumatic Chylous Ascites
Publications identified in Pubmed and
Cochrane database
n=614
Publications retrieved for more
detailed evaluation
n =280 (46%)
Publications included in
systematic review
n=131 (21%)
Included patients in
systematic review
n=190
Figure 1. Flow diagram of study selection process.
RESULTS
The literature search revealed 614 potentially suitable pub-
lications (Fig. 1). A total of 334 publications (54%) were
excluded because they reported exclusively on chylous as-
cites secondary to surgery, trauma, or radiotherapy; an-
other 129 studies (21%) were excluded because they re-
ferred only to the management of chylous ascites, and 20
review articles (3%) were excluded. The remaining 131
publications (21%), including 190 patients with chylous
ascites, were analyzed. These 131 publications included
124 case reports and 7 case series. The median number of
reported patients per study was 1 (range 1 to 16). In the
study period of 20 years, a median of 6 (range 3 to 26) new
cases of atraumatic chylous ascites were published per year.
Etiology of atraumatic chylous ascites
Forty-one different etiologies of atraumatic chylous ascites
were identified. The various causes could be grouped into 7
major and 18 minor categories, as shown in Table 1. Lym-
phatic anomalies were the most frequent cause (32% [61 of
190]). They were significantly more frequent in the pedi-
atric (84% [51 of 61]) compared with the adult population
(8% [10 of 129]) (p ⬍ 0.001) (Table 2). The most frequent
anomaly was lymphangiectasia, diagnosed in 3% of adults
(5 of 190) and 26% of children and adolescents (50 of
190). The second most frequent etiology was malignant
disease, which was responsible for 17% of patients (33 of
190) with atraumatic chylous ascites. This group com-
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J Am Coll Surg
Excluded Publications n =334 (54%)
Chylaskos secondary to surgery,
trauma or radiotherapy
Excluded publications n =129 (21%)
Reports exclusively on
management of chylaskos
Excluded review articles n=20 (3%)
prised various cancers (7% [13 of 190]), sarcomas (2% [4
of 190]), neuroendocrine tumors (3% [5 of 190]), lympho-
mas (5% [10 of 190]), and leukemias (1% [1 of 190]).
Malignancy was found almost exclusively in adults (25%
versus 2% in children; p ⬍ 0.001). Finally, in adults, liver
cirrhosis (11% [20 of 190]) and infections with mycobac-
teria (10% [19 of 190]) were detected as important under-
lying causes as well.
Clinical presentation of atraumatic chylous ascites
Seven different leading symptoms for chylous ascites were
detected. By far the most common symptom was massive
abdominal distension (81% [154 of 190]). This was fol-
lowed by pain, described as peritonism in 11% (20 of 190)
or nonspecific in 3% (6 of 190) of the cases. The distribu-
tion of the leading symptoms is shown in Table 3.
Incidental diagnosis of chylous ascites was identified in
3% of all patients (6 of 190). In this group, chylous ascites
was detected in the course of a groin hernia repair in 3
patients. In these patients, further investigations revealed
yellow nail syndrome11 and intestinal malrotation12 as un-
derlying causes; the cause remained unclear in 1 patient.13
Furthermore, incidental chylous ascites was detected dur-
ing a caesarean section in 1 patient with a history of a recent
episode of acute pancreatitis14 and in 1 patient with fibro-
sing mesenteritis.15 Finally, a lymphoma was detected as the
cause for chylous ascites in a patient who underwent sur-
gery for a symptomatic enterocele.16
Vol. 212, No. 5, May 2011 Steinemann et al Atraumatic Chylous Ascites 901

Table 1. Etiology of Atraumatic Chylous Ascites

n (%) Reference(s)
Lymphatic anomalies 61 (32)
Lymphangiectasia detected in adults 5 (3) 28, 39, 43, 80, 127
Lymphangiectasia detected in children 50 (26) 10, 30, 40, 45, 52, 68, 75, 76, 83, 91, 115, 121
Klippel-Trenaunay-Weber syndrome 1 (1) 42
Yellow nail syndrome 4 (2) 16, 86, 87, 101
Lymphangioma 1 (1) 1
Malignancy 33 (17)
Solid organ cancer
Stomach cancer 2 (1) 32, 98
Esophageal cancer 1 (1) 47
Pancreatic cancer 1 (1) 88
Islet cell cancer 1 (1) 71
Small bowel cancer 1 (1) 114
Lung cancer 1 (1) 106
Prostate cancer 2 (1) 74, 129
Testicular cancer 1 (1) 4
Endometrial cancer 1 (1) 102
Cancer of unknown primary 2 (1) 96, 111
Sarcoma
Histiocytic sarcoma 1 (1) 9
Kaposi sarcoma 3 (2) 65, 107, 120
Neuroendocrine tumors
Carcinoid 5 (3) 15, 51, 67, 70, 131
Lymphoma
Non-Hodgkin lymphoma 9 (5) 8, 11, 21, 22, 55, 59, 61, 95, 130
Hodgkin lymphoma 1 (1) 34
Chronic lymphatic leukemia 1 (1) 128
Liver cirrhosis 20 (11) 3, 46, 50, 57, 58, 63, 66, 84, 93, 104, 108, 112
Mycobacterium infections 19 (10)
Mycobacterium avium in AIDS 14 (7) 2, 5, 79, 100, 118
Tuberculosis 5 (3) 38, 73, 89, 99, 109
Lymphangioleiomyomatosis 10 (5) 18, 19, 25, 29, 49, 53, 64, 81, 110, 113
Pancreatitis 8 (4) 1, 31, 37, 44, 56, 77, 116, 119
Uncommon causes 39 (21)
Cardiac
Constrictive pericarditis 6 (3) 54, 60, 62, 72, 85, 125
Left ventricular dysfunction 1 (1) 48
Fibrosing mesenteritis 5 (3) 23, 24, 26, 27, 82
Nephrotic syndrome 5 (3) 6, 35, 41, 78, 124
Collagenosis/vasculitis
Behçet disease 1 (1) 103
Systemic lupus erythematodes 3 (2) 36, 69
Sarcoidosis 2 (1) 117, 123
Gastrointestinal disorders
Intestinal malrotation 2 (1) 13, 14
Volvulus of small intestine 1 (1) 17
Ménétrier disease 1 (1) 94
Congenital syndromes
Familial visceral myopathy 1 (1) 97
Costello syndrome 4 (2) 20
Others
Castleman disease 1 (1) 105
Hyperthyroidism 1 (1) 33
Ascariasis 1 (1) 90
Unclear causes 4 (2) 12, 92, 122, 126
References for Table 1 are listed in Appendix 1, online only.

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902 Steinemann et al Atraumatic Chylous Ascites J Am Coll Surg

Table 2. Distribution of Causes of Atraumatic Chylous Ascites in Adults and Children

All patients (N ⴝ 190), n (%) Adults (n ⴝ 129), n (%) Children (n ⴝ 61), n (%) p Value
Lymphatic anomalies 61 (32) 10 (8) 51 (84) ⬍0.001*
Malignancy 33 (17) 32 (25) 1 (2) ⬍0.001*
Solid organ cancer 13 (7) 13 (10) 0 (0) 0.01*
Sarcoma 4 (2) 3 (2) 1 (2) 1.00
Carcinoid 5 (3) 5 (4) 0 (0) 0.178
Lymphoma 10 (5) 10 (8) 0 (0) 0.032*
Leukemia 1 (1) 1 (1) 0 (0) 1.00
Liver cirrhosis 20 (11) 20 (16) 0 (0) 0.001*
Mycobacterium infection 19 (15) 19 (15) 0 (0) 0.001*
Lymphangioleiomyomatosis 10 (5) 10 (8) 0 (0) 0.032*
Pancreatitis 8 (4) 8 (6) 0 (0) 0.056
Uncommon causes 39 (21) 30 (23) 9 (15)
Cardiac 7 (4) 7 (5) 0 (0) 0.099
Fibrosing mesenteritis 5 (3) 5 (4) 0 (0) 0.178
Nephrotic syndrome 5 (3) 5 (4) 0 (0) 0.178
Collagenosis/vasculitis 6 (3) 6 (5) 0 (0) 0.179
Gastrointestinal disorders 4 (2) 2 (2) 2 (3) 0.595
Congenital syndromes 5 (3) 1 (1) 4 (7) 0.037*
Others 7 (4) 4 (3) 3 (5) 0.679
*Significant values.

According to the presence or absence of abdominal dis-
tension, patients were divided into 2 subgroups (Table 4).
Abdominal distension was present in 81% (154 of 190)
and absent in 19% (36 of 190). Lymphatic anomalies were
more than 4 times more frequent in the group with abdom-
inal distension (38% [58 of 154] versus 8% [3 of 36]; p ⫽
0.001). The most common cause of chylous ascites in the
group without distension was infection with mycobacteria,
mostly as an opportunistic infection in HIV-infected pa-
tients (31% [11 of 36]). Mycobacterial infection was found
significantly more frequently in patients lacking abdominal
distension (p ⬍ 0.001). Interestingly, in patients without
abdominal distension, a relatively high incidence of history
of acute or chronic pancreatitis (17% [6 of 36]; p ⫽ 0.001)
was found. In contrast, liver cirrhosis was only seen when
abdominal distension was present (13% [20 of 154]).
Malignancies were slightly more prevalent in the group
Table 3. Leading Symptoms of Atraumatic Chylous Ascites
(N ⫽ 190)
Symptom
Abdominal distension
Peritonism
Unspecific abdominal pain
Incidental
Diarrhea
Dysphagia
Progressive peripheral edema
with abdominal distension (19% [29 of 154] versus 11%
[4 of 36]). However, this difference did not reach statistical
significance (p ⫽ 0.336).
Interestingly, in patients presenting with peritonism as
the leading symptom (n ⫽ 20), a Mycobacterium avium
infection could be identified in 55% of the patients (11 of
20). The other causes were pancreatitis (10% [2 of 20]),
lymphatic anomaly (10% [2 of 20]), solid organ cancer
(10% [2 of 20]), and gastrointestinal malrotation (5% [1 of
20]). In 2 patients (10% [2 of 20]), the underlying disease
remained unclear.
Pediatric population
Eighteen of 131 publications (14%) reported on 61 pe-
diatric patients as defined by an age at diagnosis of 16 years
or younger. In this specific subgroup, almost all patients
(95% [58 of 61]) presented with abdominal distension.
The cause for chylous ascites was lymphatic anomaly in
84% (51 of 61). The other rare causes are shown in Table 2.
n (%) DISCUSSION
153 (81) This is the first study to systematically review the symp-
20 (11) toms and causes of atraumatic chylous ascites. To this end,
6 (3) we conducted a systematic search of the literature and re-
6 (3) viewed more than 600 publications. We found lymphatic
2 (1) anomalies, malignancy, liver cirrhosis, and mycobacterial
1 (1) infections to be the most frequent causes of atraumatic
1 (1) chylous ascites. Thus, the finding of atraumatic chylous

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Vol. 212, No. 5, May 2011 Steinemann et al Atraumatic Chylous Ascites 903

Table 4. Distribution of Causes of Atraumatic Chylous Ascites in Correlation With Leading Symptoms
Patients with Patients without
All patients abdominal distension abdominal distension
(N ⴝ 190), n (%) (n ⴝ 154), n (%) (n ⴝ 36), n (%) p Value
Lymphatic anomalies 62 (33) 58 (38) 3 (8) 0.001*
Malignancy 33 (17) 29 (19) 4 (11) 0.336
Solid organ cancer 13 (7) 10 (6) 3 (8) 0.715
Sarcoma 4 (2) 4 (3) 0 (0) 1.00
Carcinoid 5 (3) 5 (3) 0 (0) 0.585
Lymphoma 10 (5) 9 (6) 1 (3) 0.691
Leukemia 1 (1) 1 (1) 0 (0) 1.00
Liver cirrhosis 20 (11) 20 (13) 0 (0) 0.016*
Mycobacterium infection 19 (10) 8 (5) 1 (31) ⬍0.001*
Lymphangioleiomyomatosis 10 (5) 7 (5) 3 (8) 0.404
Pancreatitis 8 (4) 2 (1) 6 (17) 0.001*
Uncommon causes 38 (19) 30 (19) 9 (25)
Cardiac 7 (4) 7 (5) 0 (0) 0.350
Fibrosing mesenteritis 5 (3) 4 (3) 1 (3) 1.00
Nephrotic syndrome 5 (3) 4 (3) 1 (3) 1.00
Collagenosis/vasculitis 6 (3) 6 (4) 0 (0) 0.597
Gastrointestinal disorders 4 (2) 2 (1) 2 (6) 0.163
Congenital syndromes 5 (3) 5 (3) 0 (0) 0.585
Others 6 (3) 2 (1) 5 (14) 0.003*
*Significant values.

ascites should always prompt the surgeon to rule out these
serious underlying causes.
The causes of chylous ascites can be roughly categorized
into traumatic and atraumatic. In daily clinical practice,
surgeons are most often faced with chylous ascites second-
ary to a surgical procedure or abdominal trauma.3,6,17 Less
frequently, they come into contact with patients suffering
from chylous ascites not related to surgery or trauma. This
is corroborated by the fact that only 21% of the studies
retrieved by our literature search reported on chylous as-
cites of atraumatic origin (Fig. 1). Because the assessment
of the cause is fundamental for the treatment of atraumatic
chylous ascites, we tried to elucidate its different etiologies.
Interestingly, we found lymphatic anomalies and malig-
nancy to be the most frequent causes. Although lymphatic
anomalies are the predominant cause in the pediatric pop-
ulation, malignancies, liver cirrhosis, and mycobacterial in-
fections are the leading causes in adults. Intestinal lymp-
hangiectasia, characterized by the presence of dilated
lymph vessels in the submucosa of the small bowel, can be
of primary or secondary origin. Primary intestinal lymp-
hangiectasia, responsible for the majority of cases of chy-
lous ascites in the pediatric population (84%), usually
manifests in early childhood.18-20 In contrast, lymphatic
anomalies account only for 9% of atraumatic chylous as-
cites in adults. In this population, acquired impairments of
chyle flow leading to congestion of the lymphatic vessels, as
in yellow nail syndrome,21 are more prevalent. Further-
more, various diseases can lead to disruption of lymphatic
vessels and thus chylous ascites owing to congestion, as seen
in constrictive pericarditis and severe right heart failure, or
owing to obstruction, as seen in fibrosing mesenteritis, in-
fectious lymph node bulks in tuberculosis, intestinal mal-
rotation, sarcoidosis, and Castleman disease.18
According to the current literature, malignancies are be-
lieved to account for up to 85% of cases of atraumatic
chylous ascites in adults.7 However, in our systematic re-
view, malignancies were only responsible for 25% of the
cases of atraumatic chylous ascites in adults. Among the
group of malignancies, lymphoma was the predominant
tumor. This is in line with a large case series, in which
lymphoma accounted for at least one-third of the cases.7
Because lymphomas are responsible for only approxi-
mately 4% of the malignancies in the world,22 patients
with atraumatic chylous ascites owing to lymphoma are
overrepresented.
Apart from lymphoma, we found a quite frequent asso-
ciation of chylous ascites with carcinoid tumors (15% of all
malignant causes) and Kaposi sarcoma (9%). This stands in
contrast to a study of 875 patients with carcinoid tumors,
which reported a rather low incidence of chylous ascites in
those patients (1%).23
Although ascites is a common feature of liver cirrhosis,
chylous ascites is found in only 0.5% to 1% of patients

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904 Steinemann et al Atraumatic Chylous Ascites
with cirrhosis.24,25 Nevertheless, we found liver cirrhosis to
be responsible for 11% of atraumatic chylous ascites. This
discrepancy might be related to the fact that not all patients
with ascites are treated by paracentesis, and therefore chy-
lous ascites remains undetected. To date, all available stud-
ies on this subject are of retrospective design and do not
include data on routine paracentesis with subsequent anal-
ysis of triglycerides. The pathophysiologic mechanisms of
chylous ascites in liver cirrhosis are thought to be an in-
creased intra-abdominal pressure combined with degener-
ative changes in the splanchnic lymph vessels, thus leading
to an intra-abdominal leakage of lymph fluid.24,25
Surprisingly, our review revealed that infections with
mycobacterial species, as M. avium infection and tubercu-
losis, tend to induce chylous ascites, thus contributing to
10% of all cases.26 Except for mycobacteria, no other bac-
terial or viral infection caused chylous ascites. Interestingly,
we found that in the absence of abdominal distension and
when peritonism was present, mycobacterial infections
were the predominant cause of atraumatic chylous ascites.
Our analysis showed that patients with atraumatic chy-
loperitoneum present most often with either abdominal
distension (81%) or unspecific pain, including peritonism
(14%). However, in 3% of the patients, chylous ascites was
found incidentally during the course of investigations on
complaints not related to chylous ascites (eg, dysphagia in
esophageal cancer). Although a review of 140 cases pointed
to a similar distribution of leading symptoms,2 this study
dating back to 1967 is severely limited because of its non-
systematic nature and the inclusion of 50 cases of unknown
etiology and 19 postoperative or posttraumatic cases.
Little is known about the pathogenesis of pain in chylous
ascites. One hypothesis suggests that pain results from the
distension of the mesenteric serosa and retroperitoneum.27
However, although this hypothesis may be valid when ab-
dominal distension is present, it cannot be applied to pa-
tients without distension. Another hypothesis is that pain is
evoked by direct contact of chylous with the peritoneal
serosa.28 However, this does not explain why only acute
onset of chylous ascites causes pain. Probably the clinical
manifestation of chylous ascites is not only dependent on
the velocity of its development (acute versus chronic) but
also on its volume.
Because atraumatic chylous ascites can be a sequela of
numerous different diseases, the question on how to best
identify the underlying disorder remains. Based on our
detailed analysis, we suggest beginning with a thorough
analysis of the ascites, including the determination of tri-
glyceride levels (confirmation of chylous ascites), bacteri-
ology (above all, testing for mycobacteria), and cytology. In
parallel, blood samples should be drawn to check for signs
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J Am Coll Surg
of liver damage and to perform hepatitis serology. In cases
for which these tests are inconclusive, a CT scan of the
thorax and abdomen should be carried out. Subsequently,
therapy is directed against the identified underlying
disease.
In conclusion, although atraumatic chylous ascites is a
rare clinical finding, surgeons should be aware of the seri-
ous underlying causes and therefore always rule out malig-
nancy and mycobacterial infection even in asymptomatic,
supposedly healthy patients.
Author Contributions
Study conception and design: Steinemann, Dindo, Nocito
Acquisition of data: Steinemann
Analysis and interpretation of data: Steinemann, Dindo, Cla-
vien, Nocito
Drafting of manuscript: Steinemann, Dindo, Clavien, Nocito
Critical revision: Dindo, Clavien
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