Mesenteric lymphatic malformation associated with acute appendicitis: a case report.

Abstract

Mesenteric lymphatic malformations are rare, benign tumors that are most commonly found in children. The presentation of these tumors is variable and may either be innocuous or life threatening. It has been suggested that mesenteric lymphatic malformations are congenital; however, there is evidence that their growth may be stimulated by local trauma.
We describe the first case of a mesenteric lymphatic malformation associated with acute appendicitis in a 13-year-old Caucasian boy. The patient is well six months after surgical excision of the tumor.
The reader should be aware that growth and/or development of mesenteric lymphatic malformations may be associated with trauma and other pro-inflammatory processes.

Full text

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Case report
Mesenteric lymphatic malformation associated with acute
appendicitis: a case report
Catherine Hunter1,2*, Meghan Connelly3, Steven Lee4, Larry Wang5
and Nam Nguyen1
Open Access
Addresses:1Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, California 90089, USA
2Department of Surgery, Harbor UCLA Medical Center, W Carson Street, Torrance, California 90502, USA
3Childrens Hospital Los Angeles, Sunset Blvd, Los Angeles, California 90027, USA
4Department of Surgery, Kaiser Permanente, 4700 Sunset Blvd, Los Angeles, California 90027, USA
5Department of Pathology, Keck School of Medicine, University of Southern California, Los Angeles, California 90089, USA
Email: CH* - chunter@chla.usc.edu; MC - mconnell@usc.edu; SL - Steven.L.Lee@kp.org; LW - LaWang@chla.usc.edu;
NN - NaNguyen@chla.usc.edu
*Corresponding author
Received: 15 May 2008Accepted: 9 February 2009Published: 17 September 2009
Journal of Medical Case Reports 2009, 3:9030doi: 10.4076/1752-1947-3-9030
This article is available from: http://jmedicalcasereports.com/jmedicalcasereports/article/view/9030
© 2009 Hunter et al.; licensee Cases Network Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Introduction: Mesenteric lymphatic malformations are rare, benign tumors that are most
commonly found in children. The presentation of these tumors is variable and may either be
innocuous or life threatening. It has been suggested that mesenteric lymphatic malformations are
congenital; however, there is evidence that their growth may be stimulated by local trauma.
Case presentation: We describe the first case of a mesenteric lymphatic malformation associated
with acute appendicitis in a 13-year-old Caucasian boy. The patient is well six months after surgical
excision of the tumor.
Conclusion: The reader should be aware that growth and/or development of mesenteric lymphatic
malformations may be associated with trauma and other pro-inflammatory processes.
Introduction
Mesenteric lymphatic malformations (MLM) are rare,
benign tumors that most commonly develop in children
[1]. The nomenclature of lymphatic malformations is at
times confusing; microcystic lymphatic malformation was
previously called lymphangioma and macrocystic was
called cystic hygroma. Lymphangiomas are commonly
located in the skin and subcutaneous tissues, although
they have been described in deeper tissues including the
neck, axilla, and retroperitoneum. The incidence of intra-
abdominal MLM is low, with less than 200 cases in the
literature[2].Oneinstitutionreported193casesofchildren
with lymphangiomas, with the following distribution:
cervical (31.4%), craniofacial (18.9%), extremity (18.9%),
trunk (9.2%), abdominal (9.2%), cervicoaxillothoracic
(4.9%), cervicomediastinal (2.2%), intrathoracic (1.6%)
and multiple (3.8%) [3]. The etiology of lymphatic
malformations is unclear. They may be congenital or may
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develop secondary to infection or trauma. We present the
first case report of a child who developed MLM associated
with acute appendicitis.
Case presentation
A 13-year-old Caucasian boy presented with acute
appendicitis, characterized by right lower quadrant pain.
He underwent a laparoscopic appendectomy, and a
pathology report confirmed the diagnosis of focal acute
appendicitis. No intra-abdominal masses were noted at
the time of surgery. However, after the operation, the
patient experienced persistent drainage of serosanginous
fluid from a trochar site. Once this drainage ceased, the
patient was discharged home ten days after admission.
During the next six months he continued to experience
intermittent abdominal pain, which led to representation
to the emergency room. Additionally, the patient experi-
enced an increase in abdominal girth, abdominal pain,
and weight loss. No constipation, diarrhea, nausea,
vomiting or jaundice was reported. A CT scan of the
abdomen and pelvis demonstrated a 23 by 12.5 cm fatty
soft tissue mass surrounded by mesenteric fat (Figure 1).
The tumor encased branches of the superior mesenteric
artery and displaced the small bowel. Multiple fluid
collections were also seen in the right lower quadrant.
A subsequent CT-guided biopsy suggested a benign fatty
tumor; the differential diagnosis included lymphangioma,
lipoma and fibrolipoma. The patient was then transferred
to our institution for definitive care.
Aphysicalexaminationofthepatientrevealedthathisvital
signs were within normal limits and that his abdomen was
distended with a large palpable mass extending from the
left flank to the right semilunar line. A large part of the
bowel appeared to be displaced in the right abdomen, and
the presence of shifting dullness suggested ascites.
The patient was taken to the operating room where
a diagnostic laparoscopy confirmed a large intra-abdom-
inal tumor arising from the mesentery and a significant
amount of free chylous fluid. The tumor adhered to the
duodenum and the superior mesenteric artery (SMA) and
vein (SMV). A frozen biopsy performed during the
operation suggested that the mass was a lymphatic
vascular malformation with a fibrous stroma and fibrous
capsule consistent with a benign tumor. The operation was
converted to a midline laparotomy for tumor resection. As
mentioned earlier, the tumor was largely entangled with
the mesentery. However, a circumferential dissection was
performed through the creation of a plane between the
tumor and mesentery. The SMA, the third and fourth
portions of the duodenum, and the proximal small bowel
were each affixed to the tumor but were successfully
dissected off. The tumor was excised along with 50 cm of
small bowel, and a primary anastamosis was performed
(Figure 2). The final pathology demonstrated a 27.5-cm
lymphatic malformation with no evidence of malignancy
(Figure 3); the ascitic fluid aspiration was deemed to be
chylous ascites. The patient recovered well and was
discharged home eight days after the operation. At a
follow-up visit six months after the operation, the patient
is doing well and tolerating full oral feeding, with
complete resolution of his abdominal complaints.
Discussion
MLM are rare with reported frequencies of 1 in 20,000 to 1
in 250,000 admissions [2]. However, the precise number
Figure 1. A computed tomography of a large intra-abdominal
mass is seen with displacement of the surrounding structures.
Figure 2. An intra-operative image of the huge circumscribed
mass is located in a small bowel serosal region with milky
secretion on the surface.
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of MLM cases is unclear because most reports do not
clearly differentiate between MLM and other mesenteric
cysts. Although MLM may occur at any age, most cases of
MLM are found in children, with an increasing number
of cases diagnosed before birth [4]. In fact, almost 60% of
cases present prior to the fifth year of life. MLM have been
described inboth men andwomen; however, some studies
have demonstrated a male predominance [5]. The
presentation of MLM is variable, and the clinical symp-
toms mayinclude pain, nauseaor vomiting[5].Mesenteric
lymphatic malformations may be misdiagnosed as benign
abdominal processes or even as malignancies [6].
Although most cases of MLM are discovered by accident,
they may be found in association with intra-abdominal
catastrophes such as intestinal volvulus [7]. Additionally,
traumatic hemorrhage in MLM has been described [8];
however, there are no reports of MLM developing as
a result of intra-abdominal trauma, surgery or infection.
The etiology of MLM is unknown, although it has been
proposed that they may be associated with developmental
anomalies of the lymphatic system, secondary to a failure
of the lymphatic system to connect with the venous
system. This theory may, in part, be supported by the
predominance of MLM in children. Other possible causes
include inflammation in the lymphatic channels, resulting
in obstruction and subsequent lymphangioma formation
[9]. It has further been suggested that injury may trigger
delayed proliferation of cells, thereby causing lymphatic
malformations to develop [10]. Although there have been
reports of injury and infection associated with lymphatic
malformations in the extremities and neck [11-13], there is
only one report of a lymphatic malformation developing
after surgery, and this is the case of a retroperitoneal cyst
which developed after cholecystectomy [14].
A highly unusual feature of this case report is that the
patient’s MLM apparently developed in connection with
his appendicitis. This raises one of two possibilities. First,
it is possible that the patient’s mass was simply not seen
during the first surgery. This is possible since persistent
drainage from the trochar sites complicated the patient’s
condition after the operation, suggesting that the MLM
may have already been present. The second possibility is
that the appendicitis or the surgery and appendectomy
triggered an exponential growth of the MLM. Since no
imaging was obtained during the initial diagnosis, there is
no way to determine conclusively whether or not the mass
was absent. However, it certainly appears that the mass
developed, or more probably, grew exponentially after the
appendectomy.
Conclusions
MLM are rare tumors that are more common among
children. They may be associated with a range of clinical
symptoms and are best treated with surgicalresection since
Figure 3. Pathologic imaging of the mesenteric lymphatic malformations. Panel A: The tumor is not encapsulated and has a
spongy appearance with multiple pinhead-sized spots in the peripheral areas. The dilated lymphatic vessels are filled with a milk-
white fluid. Panel B: (×200) The abnormally dilated endothelial-lined spaces contain lymphocytic aggregates (arrow).
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they may grow to a large size, compress vital structures and
cause intra-abdominal catastrophe. We describe the first
case of development of a MLM associated with appendi-
citis and suggest that the trauma of surgery may have
triggered the exponential growth and development of this
tumor. We recommend that surgeons and pediatric health
care providers be aware of this association and consider
it in their differential diagnoses.
Abbreviations
CT, computed tomography; MLM, mesenteric lymphatic
malformations; SMA, superior mesenteric artery; SMV,
superior mesenteric vein.
Competing interests
The authors declare that they have no competing interests.
Consent
Written informed consent was obtained from the patient’s
parents for publication of this case report and any
accompanying images. A copy of the written consent is
available for review by the Editor-in-Chief of this journal.
Authors’ contributions
CH obtained the images and wrote the manuscript. LW
prepared the images. MC, SL, and NN contributed
significantly to the writing of this manuscript.
Acknowledgements
The authors are grateful to Henri R. Ford, M.D. for his
helpful comments in the preparation of this manuscript.
References
1.Konen O, Rathaus V, Dlugy E, Freud E, Kessler A, Shapiro M,
Horev G: Childhood abdominal cystic lymphangioma. Pediatr
Radiol 2002, 32:88-94.
2.Losanoff JE, Richman BW, El-Sherif A, Rider KD, Jones JW:
Mesenteric cystic lymphangioma. J Am Coll Surg 2003, 196:
598-603.
3. Hancock BJ, St-Vil D, Luks FI, Di Lorenzo M, Blanchard H:
Complications of lymphangiomas in children. J Pediatr Surg
1992, 27: 220-224.
4.Merrot T, Chaumoitre K, Simeoni-Alias J, Alessandrini P, Guys JM,
Panuel M: Abdominal cystic lymphangiomas in children.
Clinical, diagnostic and therapeutic aspects: apropos of 21
cases. Ann Chir 1999, 53:494-499.
5.Takiff H, Calabria R, Yin L, Stabile BE: Mesenteric cysts and
intra-abdominal cystic lymphangiomas. Arch Surg 1985, 120:
1266-1269.
6.Kirzeder DJ, Kan JH: Mesenteric lymphatic malformation.
Pediatr Radiol 2007, 37:845-846.
7.Traubici J, Daneman A, Wales P, Gibbs D, Fecteau A, Kim P:
Mesenteric lymphatic malformation associated with small-
bowel volvulus - two cases and a review of the literature.
Pediatr Radiol 2002, 32:362-365.
8.Porras-Ramirez G, Hernandez-Herrera MH: Hemorrhage into
mesenteric cyst following trauma as a cause of acute
abdomen. J Pediatr Surg 1991, 26:847-848.
9. Gleason TJ, Yuh WT, Tali ET, Harris KG, Mueller DP: Traumatic
cervical cystic lymphangioma in an adult. Ann Otol Rhinol
Laryngol 1993, 102:564-566.
10. Wiggs WJ Jr, Sismanis A: Cystic hygroma in the adult: two case
reports. Otolaryngol Head Neck Surg 1994, 10:239-241.
11.Postacchini F, Sadun R: Lymphangioma of the thigh following
acute trauma. Clin Orthop Relat Res 1976, 121:169-172.
Antoniades K, Kiziridou A, Psimopoulou M: Traumatic cervical
cystic hygroma. Int J Oral Maxillofac Surg 2000, 29:47-48.
Aneeshkumar MK, Kale S, Kabbani M, David VC: Cystic lymphan-
gioma in adults: can trauma be the trigger? Eur Arch
Otorhinolaryngol 2005, 262:335-337.
Niwa H, Sumita N, Ishihara K, Hoshino T, Iwase H, Kuwabara Y:
A case of retroperitoneal chylous cyst developed after
cholecystectomy and choledochotomy. Nippon Geka Gakkai
Zasshi 1988, 89:282-285.
12.
13.
14.
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