A Hebra

AR Gupta

DP Bliss Jr

GW Phillips

H Okur

H Takiff

JC Fish

JE Losanoff

K Panjwani

Kamal Nayan Rattan

Manish Pathak

N Fujita

OH Beahrs

R McEwing

RJ Kurtz

RK Raghupathy

S Chirathivat

S Engel

S Kriaa

S Singh

Sanjay Kumar

SK Ratan

Vimoj J Nair

VW Vanek

Journal of Medical Case Reports

English

PubMed

Springer - Publisher Connector

BioMed CentralJournal of Medical Case Reports
ssOpen AcceCase report
Pediatric chylolymphatic mesenteric cyst - a separate entity from 
cystic lymphangioma: a case series
Kamal Nayan Rattan1, Vimoj J Nair*2, Manish Pathak1 and Sanjay Kumar3
Address: 1Department of Pediatric Surgery, Pt. B.D.Sharma, Post Graduate Institute of Medical Sciences, Rohtak, Haryana, 124001, India, 
2Department of Radiation Oncology, Pt. B.D.Sharma, Post Graduate Institute of Medical Sciences, Rohtak, Haryana, 124001, India and 
3Department of Pathology, Pt. B.D.Sharma, Post Graduate Institute of Medical Sciences, Rohtak, Haryana, 124001, India
Email: Kamal Nayan Rattan - knrattn@gmail.com; Vimoj J Nair* - vim4u4ever@hotmail.com; Manish Pathak - mp3pathak@yahoo.co.in; 
Sanjay Kumar - drsanjayk@yahoo.co.uk
* Corresponding author    
Abstract
Introduction: Chylolymphatic mesenteric cysts are rare entities with variable presentations and
this has surgical implications in the pediatric age group.
Case presentation: We carried out a retrospective analysis of the clinical and histopathological
records of pediatric patients diagnosed and treated for chylolymphatic mesenteric cysts at our
institute from 1998 to 2008. Eight patients met the histopathological criteria of chylolymphatic
mesenteric cyst. These patients were in the age range 18 months to 10 years with a mean age of
4.5 years. Of these eight patients, four presented with an abdominal lump, and two each with
abdominal pain and acute intestinal obstruction. On clinical examination, five out of the eight
patients had a palpable abdominal mass. Laparotomy and complete excision of the cyst along with
the involved gut was performed in all patients. There were no postoperative complications or any
recurrence during the follow-up period which ranged from 4 months to 8 years.
Conclusion: Although very rare, chylolymphatic mesenteric cyst should be kept in mind as one of
the differential diagnoses of cystic masses of the abdomen including cystic lymphangioma.
Ultrasonography and computed tomography suggest the diagnosis but histopathological
examination is required for confirmation. Complete excision of the cyst yields excellent results.
Introduction
A chylolymphatic cyst is a rare variant of a mesenteric cyst
[1,2]. These cysts present within the mesentery, lined with
a thin endothelium or mesothelium and filled with chy-
lous and lymphatic fluid [3]. Although mesenteric cysts in
general have been reported in the literature fairly fre-
quently, chylolymphatic cysts in the pediatric age group
are extremely rare in the modern medical literature [2],
therefore very little information is available regarding
their presentation and complications. We present our
unique experience of eight cases during the past 10 years
in the hope that this information will reinforce the diag-
nostic and treatment strategy. This is one of the largest
reported case series of chylolymphatic cysts in the pediat-
ric age group.
Case presentation
Clinical case records at our institute from the period 1998
to 2008 were reviewed for pediatric patients with a diag-
nosis of chylolymphatic cyst of the mesentery. For the
study, an age of 16 years or younger was considered to be
in the pediatric age group. The following descriptive data
Published: 9 November 2009
Journal of Medical Case Reports 2009, 3:111 doi:10.1186/1752-1947-3-111
Received: 8 September 2008
Accepted: 9 November 2009
This article is available from: http://www.jmedicalcasereports.com/content/3/1/111
© 2009 Rattan et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 4
(page number not for citation purposes)
Journal of Medical Case Reports 2009, 3:111 http://www.jmedicalcasereports.com/content/3/1/111were analyzed: age at presentation, gender, presenting
symptoms, duration of symptoms, physical findings,
imaging results, pre-operative, peri-operative and postop-
erative management, operative findings, and complica-
tions. The histopathological characteristics of each
surgical specimen were also examined. The data obtained
were analyzed and compared with the available literature.
Results
Retrospective analysis revealed eight patients meeting the
histopathological criteria of chylolymphatic cyst. The age
of the eight patients ranged from 18 months to 10 years
with a mean age of 4.5 years. Five patients were male and
three were female. Of these eight patients, four presented
with an abdominal lump. Of the remaining four patients,
two patients presented with an abdominal pain and two
with acute intestinal obstruction presenting early, within
2 days of becoming symptomatic. Patients with an
abdominal lump and painful abdomen had delayed pres-
entation. Intermittent, colicky pain was poorly localized
and was mild to moderate in intensity. Three of the four
patients presenting with an abdominal lump and pain
also had intermittent episodes of abdominal distension,
bilious vomiting and constipation suggesting subacute
intestinal obstruction, and required hospital admission
elsewhere. On clinical examination, five out of the eight
patients had a palpable abdominal mass. In both the
patients presenting with acute intestinal obstruction,
plain abdominal radiography showed multiple air-fluid
levels. A mass defect displacing the bowel loops around it
was visible on plain abdominal radiographs in all of the
patients. On sonography, all patients showed multilocu-
lated cystic lesions in relation to the gut. Abdominal com-
puted tomography was done in only two cases and
revealed similar findings. All our patients underwent elec-
tive laparotomy after initial resuscitation. All had a multi-
loculated cyst involving the small bowel mesentery
(Figure 1). The cysts were of varying sizes with the smallest
approximately 8 mm in diameter and the largest approxi-
mately 9 cm in diameter. In two patients, acute stretching
of the bowel loop over the large cyst led to acute intestinal
obstruction. Resection of the involved gut along with the
cyst was required in all patients as the involved gut shared
the vascular supply with the cyst. Postoperative recovery
in all patients was excellent. No recurrence was noted dur-
ing the follow-up period, which ranged from 4 months to
8 years. All specimens were sent for histopathological
examination, which revealed multiloculated cysts lined
with endothelium and filled with chylous fluid and
lymph (Figure 2). Thus, all cases were confirmed to be
chylolymphatic cysts by histopathology.
Discussion
Chylous cysts are rare variants of mesenteric lesions and
constitute 7.3% to 9.5% of all abdominal cysts [1]. There
are very few cases of pediatric chylolymphatic cysts
reported in the literature. Engel et al. [1] reported four
cases of cystic lymphangioma in the pediatric age group
and only two of them were of the chylous variety. Singh et
al. [4] reported five cases of cystic lymphangioma in the
pediatric age group though only one of them was diag-
nosed to be a chylolymphatic cyst on histopathology.
Gupta et al. [2] reported a neonate who presented with
intestinal obstruction due to a chylous mesenteric cyst.
Panjwani et al. [5] reported an isolated case of a chylolym-
phatic mesenteric cyst in a 10-day-old neonate. Ratan and
coworkers [6,7] reported a mesenteric cyst causing caecal
volvulus. Our series also contained a mesenteric cyst
resulting in caecal volvulus. Here we have reported a series
of eight cases of chylolymphatic cysts in the pediatric age
group with variable presentations: abdominal lumps,
Pediatric chylolymphatic mesenteric cystFigure 1
Pediatric chylolymphatic mesenteric cyst. Resected 
specimen of pediatric chylolymphatic mesenteric cyst show-
ing multiloculated cyst along with resected gut.
Pediatric chylolymphatic mesenteric cyst, histopathologic imageFigure 2
Pediatric chylolymphatic mesenteric cyst, his-
topathologic image. Microphotograph showing cyst wall 
with endothelial lining and chylous fluid (hematoxylin and 
eosin stain ×100).Page 2 of 4
(page number not for citation purposes)
Journal of Medical Case Reports 2009, 3:111 http://www.jmedicalcasereports.com/content/3/1/111acute or subacute intestinal obstruction, and even poorly
localized abdominal pain, which resulted in the patient
receiving treatment from a private practitioner.
Beahrs et al. [8] classified mesenteric cysts into four groups
based on etiology: embryonic or developmental; trau-
matic or acquired; neoplastic; and infective or degenera-
tive. Recently, a pathologic classification system has been
proposed [9]. Types 1 (pedicled) and 2 (sessile) are lim-
ited to the mesentery and can be excised completely with
or without resection of the involved gut. Types 3 and 4
(multicentric) extend into the retroperitoneum and
require complex operations and often sclerotherapy as
well. Based on the contents of the cyst, the mesenteric cyst
can be divided into serous, chylous, hemorrhagic and chy-
lolymphatic cyst. The chylolymphatic cyst, as indicated by
its name, contains both chyle and lymph. The accumula-
tion of chyle and lymph is considered to be the result of
an imbalance between the inflow and outflow of fluid [1].
This cyst may be asymptomatic, and may cause abdomi-
nal distension or an abdominal lump or may present with
complications such as intestinal obstruction, hemorrhage,
infection, rupture of the cyst, volvulus or obstruction of
the urinary or biliary tract. In our study, four of the eight
patients presented with an abdominal lump while the
lump was clinically palpable in five patients. Painful
abdomen and acute intestinal obstruction was the presen-
tation in two patients each.
Radiological investigations form an integral part of the
management of these lesions. A plain abdominal radio-
graph may show a gasless, homogenous mass defect dis-
placing the bowel loops around it. In a child with an
obstructed intestine, multiple air-fluid levels will be seen
on an erect abdominal radiograph. Barium studies are
now only of historical interest; abdominal ultrasonogra-
phy is currently the imaging procedure of choice. This
delineates the nature of the mass, organ or site of the ori-
gin, and the extent and associated mass effects on the kid-
ney or liver, if any. In a chylolymphatic cyst, a 'fluid-fluid
level' can be seen on ultrasonography due to formation of
an upper fluid level by lighter chyle over a lower fluid level
of heavier lymph [10]. Computed tomography adds little
additional information; however, contrast-enhanced film
can show the relationship of the bowel and other vital
structures to the lesion. Some authors have described the
characteristic appearance of a chylolymphatic cyst on
computed tomography in the form of the presence of
fluid levels of differing echodensities, that is to say, an
upper fatty echodensity of chyle on top of the water
echodensity of lymph in a well-defined cystic lesion
[10,11].
Antenatal detection of cystic abdominal lesions is possi-
ble in a fetus during antenatal ultrasound scanning. The
sonologic picture may help in differentiating the lesion
from many other differential diagnoses. As this usually
does not alter the obstetrical management, a definite diag-
nosis is usually made in the postnatal period [12].
The different surgical approaches used are marsupializa-
tion, sclerotherapy, drainage, enucleation, percutaneous
aspiration, and excision of the cyst with or without resec-
tion of the involved gut [13-16]. Due to high recurrence
rates associated with marsupialization and drainage, com-
plete excision of the cyst should be attempted whenever
possible [14]. In adults, the cyst can often be enucleated
or 'shelled out' from between the leaves of the mesentery;
in children, however, bowel resection is frequently
required [14,17,18]. All of our patients underwent explor-
atory laparotomy and complete excision of the cystic
lesion with resection of the involved gut. Multiloculated
cysts filled with milky fluid were found in all of our
patients. The cysts were of varying sizes from the smallest
approximately 8 mm in diameter to the largest approxi-
mately 9 cm in diameter. The medical literature mentions
instances where laparoscopic removal of mesenteric cysts
has been tried successfully, but this might have been diffi-
cult in some of our patients, especially those with large-
sized chylolymphatic mesenteric cysts [19].
Intra-operatively, similar findings can be seen in cystic
lymphangioma, retroperitoneal cystic teratoma, caseating
tubercular lymph nodes, and hydatid cysts. Even lym-
phoma (Figure 3) and duplication cysts may also give sim-
ilar appearances. Excision biopsy is then recommended to
differentiate these cases. Histopathology is confirmatory
and differentiates chylolymphatic cysts from all these
lesions. Cystic lymphangioma has a striking resemblance
to chylolymphatic mesenteric cysts both grossly and
microscopically. Some authors consider chylolymphatic
mesenteric cysts to be a type of cystic lymphangioma, but
the medical literature also shows some authors describing
chylolymphatic cysts as a variant of mesenteric cysts
[3,9,17,20]. The absence of smooth muscle and lymphatic
spaces in the wall of the cyst differentiates mesenteric cysts
from cystic lymphangioma [3]. During the follow-up
period from 4 months to 8 years, we did not observe any
recurrences, thus complete excision of the chylolymphatic
cyst is curative.
Conclusion
Although very rare, chylolymphatic mesenteric cysts
should be kept in mind as one of the differential diag-
noses of cystic masses of the abdomen. There are now
well-established histopathological features that differenti-
ate chylolymphatic mesenteric cysts from other cystic
lesions including cystic lymphangioma. Resection of the
involved gut is frequently required in children and one
should not unduly prolong the surgery to avoid gut resec-Page 3 of 4
(page number not for citation purposes)
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tion. Complete excision of the cyst ensures excellent prog-
nosis and is curative.
Consent
Written informed consent was obtained from the parents
of the patients for publication of this case series and any
accompanying images. A copy of the written consent is
available for review by the Editor-in-Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
MP analyzed and interpreted the patient data retrospec-
tively. SK performed the histological examination of the
kidneys, and KN and VN were major contributors in writ-
ing the manuscript. All authors read and approved the
final manuscript.
References
1. Engel S, Clagett OT, Harrison EG Jr: Chylous cysts of the abdo-
men.  Surgery 1961, 50:593-599.
2. Gupta AR, Nanavati RN, Fernandez AR, Kalgutkar A, Nathani R,
Deshmukh SS: Chylous mesenteric cyst: an unusual cause of
neonatal intestinal obstruction.  Indian Pediatr 1992,
29(4):511-513.
3. Takiff H, Calabria R, Yin L, Stabile BE: Mesenteric cysts and intra-
abdominal cystic lymphangiomas.  Arch Surg 1985,
120(11):1266-1269.
4. Singh S, Baboo ML, Pathak IC: Cystic lymphangioma in children:
report of 32 cases including lesions at rare sites.  Surgery 1971,
69(6):947-951.
5. Panjwani K, Gangopadhyay AN, Sharma SP, Gupta DK, Chooramani
GS: Chylolymphatic mesenteric cyst: an unusual abdominal
mass in a new born.  Indian J Pediatr 1993, 60(5):712-714.
6. Ratan SK, Ratan KN, Kapoor S, Sehgal T: Giant chylolymphatic
cyst of the jejunal mesentery in a child: report of a case.  Surg
Today 2003, 33(2):120.
7. Kriaa S, Hafsa C, Majdoub S, Jouini R, Golli M, Nouri A, Gannouni A:
Caecal volvulus with calcified mesenteric cyst.  Arch Pediatr
2007, 14(7):924-925.
8. Beahrs OH, Judd ES Jr, Dockerty MB: Chylous cysts of the abdo-
men.  Surg Clin North Am 1950, 30(4):1081-1096.
9. Losanoff JE, Richman BW, El-Sherif A, Rider KD, Jones JW:
Mesenteric cystic lymphangioma.  J Am Coll Surg 2003,
196(4):598-603.
10. Fujita N, Noda Y, Kobayashi G, Kimura K, Watanabe H, Masu K,
Nagano M, Mochizuki F, Yusa S, Yamazaki T: Chylous cyst of the
mesentery: US and CT diagnosis.  Abdom Imaging 1995,
20(3):259-261.
11. Phillips GW, Senapati A, Young AE: Chylolymphatic mesenteric
cyst: a diagnostic appearance on computer tomography.  Br J
Radiol 1988, 61(725):413-414.
12. McEwing R, Hayward C, Furness M: Foetal cystic abdominal
masses.  Australas Radiol 2003, 47(2):101-110.
13. Hebra A, Brown MF, McGeehin KM, Ross AJ: Mesenteric, omen-
tal, and retroperitoneal cysts in children: a clinical study of
22 cases.  South Med J 1993, 86(2):173-176.
14. Kurtz RJ, Heimann TM, Holt J, Beck AR: Mesenteric and retro-
peritoneal cysts.  Ann Surg 1986, 203(1):109-112.
15. Vanek VW, Philips AK: Retroperitoneal, mesenteric, and
omental cysts.  Arch Surg 1984, 119(7):838-842.
16. Chirathivat S, Shermeta D: Recurrent retroperitoneal
mesenteric cyst. A case report and review.  Gastrointest Radiol
1979, 4(2):191-193.
17. Bliss DP Jr, Coffin CM, Bower RJ, Stockmann PT, Ternberg JL:
Mesenteric cysts in children.  Surgery 1994, 115(5):517-577.
18. Okur H, Kuchukaydin M, Ozokutan BH, Durak AC, Kazez A, Kose O:
Mesenteric, omental and retroperitoneal cysts in children.
Eur J Surg 1997, 163(9):673-677.
19. Raghupathy RK, Krishnamurthy P, Rajamani G, Babuji N, Diriviraj R,
Mohan NV, Swamy RN, Gurunathan S, Natarajan M: Intraabdomi-
nal cystic swelling in children - laparoscopic approach, our
experience.  J Indian Assoc Pediatr Surg 2003, 8:213-217.
20. Fish JC, Fair WR, Canby JP: Intestinal obstruction in the new-
born: an unusual case due to mesenteric cyst.  Arch Surg 1965,
90:317-318.
Pediatric chylolymphatic mesenteric cystFigure 3
Pediatric chylolymphatic mesenteric cyst. Resected 
specimen of multiloculated chylolymphatic mesenteric cyst 
mimicking lymphoma.Page 4 of 4
(page number not for citation purposes)


Pediatric chylolymphatic mesenteric cyst - a separate entity from cystic lymphangioma: a case series

Crossref

AE: Chylolymphatic mesenteric cyst: a diagnostic appearance on computer tomography.

AJ: Mesenteric, omental, and retroperitoneal cysts in children: a clinical study of 22 cases.

AR: Mesenteric and retroperitoneal cysts. Ann Surg

Chooramani GS: Chylolymphatic mesenteric cyst: an unusual abdominal mass in a new born.

Deshmukh SS: Chylous mesenteric cyst: an unusual cause of neonatal intestinal obstruction. Indian Pediatr

Dockerty MB: Chylous cysts of the abdomen. Surg Clin North Am

Foetal cystic abdominal masses. Australas Radiol

Gannouni A: Caecal volvulus with calcified mesenteric cyst. Arch Pediatr

Giant chylolymphatic cyst of the jejunal mesentery in a child: report of a case. Surg Today

IC: Cystic lymphangioma in children: report of 32 cases including lesions at rare sites. Surgery

Intraabdominal cystic swelling in children - laparoscopic approach, our experience. J Indian Assoc Pediatr Surg

Jr: Chylous cysts of the abdomen. Surgery

JW: Mesenteric cystic lymphangioma.

Kose O: Mesenteric, omental and retroperitoneal cysts in children.

Mesenteric cysts and intraabdominal cystic lymphangiomas. Arch Surg

Philips AK: Retroperitoneal, mesenteric, and omental cysts. Arch Surg

Shermeta D: Recurrent retroperitoneal mesenteric cyst. A case report and review. Gastrointest Radiol

T: Chylous cyst of the mesentery: US and CT diagnosis. Abdom Imaging

Ternberg JL: Mesenteric cysts in children. Surgery

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Pediatric chylolymphatic mesenteric cyst - a separate entity from cystic lymphangioma: a case series

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