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Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
CASE REPORT
Year : 2018  |  Volume : 23  |  Issue : 1  |  Page : 36-38
 

Ileal cystic lymphangioma presenting with acute appendicitis


Department of Pediatric Surgery, National University of Hospital, Singapore

Date of Web Publication27-Dec-2017

Correspondence Address:
V P Mali
Department of Pediatric Surgery, National University of Hospital, 5 Lower Kent Ridge Road, 119074
Singapore
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_44_17

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   Abstract 

Mesenteric lymphatic malformations are rare benign tumors that are most commonly found in children. The presentation of these tumors can be variable. It has been suggested that mesenteric lymphatic malformations are congenital; however, there is evidence that their size may be increased by infection. A 3-year 10-month-old boy presented with rhinorrhea and cough followed by acute abdominal pain. Ultrasonography revealed a lobulated mass in the lower abdomen. Computerized tomography scan of the abdomen diagnosed an inflamed appendix with perforation. Laparotomy revealed a multicystic mass within the mesentery closely adherent to the ileum; with omentum wrapped around. The appendix was dilated and firm. An ileal resection and an appendicectomy were performed. Histology confirmed acute transmural appendicitis with concurrent cystic lymphangioma of the ileal mesentery. Although rare, cystic lymphangioma must be considered in the differential of pediatric acute abdomen. Surgeons must be aware of this association and be reminded to inspect the rest of small bowel during appendicectomy especially if the preoperative imaging is suspicious.


Keywords: Acute appendicitis, children, cystic lymphangioma


How to cite this article:
Lui SA, Nyo YL, Mali V P. Ileal cystic lymphangioma presenting with acute appendicitis. J Indian Assoc Pediatr Surg 2018;23:36-8

How to cite this URL:
Lui SA, Nyo YL, Mali V P. Ileal cystic lymphangioma presenting with acute appendicitis. J Indian Assoc Pediatr Surg [serial online] 2018 [cited 2019 Jul 22];23:36-8. Available from: http://www.jiaps.com/text.asp?2018/23/1/36/221599





   Introduction Top


Lymphangiomas (LA) are benign congenital malformations probably caused by either sequestration of lymphatic tissue during the embryologic development or failure of communication of the lymphatics with the venous system.[1] LA constitute 5% of all benign tumors in infants and children with 95% occurring in the neck and axilla. Abdominal LA may account for 1%–9.2% of all pediatric LA. The incidence of abdominal LA in children varies from 1 to 4 per 100,000 hospital admissions. The presentations are varied with respect to age, location within the abdomen, and the symptomatology. We present an unusual case of cystic lymphangioma of the ileal mesentery incidentally found during surgery for acute abdomen due to a concomitant acute appendicitis at the age of 3 years.


   Case Report Top


A 3-year 10-month-old boy, who was previously well, presented with rhinorrhea and productive cough of 4 days duration followed by abdominal pain and fever (38.3°C) over 1 day. The pain was in both flanks, intermittent, and the child woke up from sleep. Physical examination revealed mild tenderness over the hypogastrium and the right iliac fossa. Blood investigations revealed leukocytosis (white cell count 15.58 × 109/L) with neutrophilia (12.79 × 109/L) and elevated C-reactive protein (173 mg/L). In view of the history of a respiratory tract infection, an abdominal ultrasound was performed to clarify the diagnosis. Ultrasound revealed a complex cystic mass in the mid infra-umbilical to right iliac fossa region (measuring about 48 mm × 22 mm × 45 mm in size), with some internal echoes and blood vessels traversing through the septae. The appendix was only partially visualized in the retrocecal region and seemed of normal caliber. There was free fluid in the pelvis [Figure 1]. The radiological diagnosis was either a lymphatic malformation, a duplication cyst, or an infective collection. In view of the inflammatory clinical picture, a computed tomography scan of the abdomen was performed. It revealed an inflamed appendix (1.1 cm diameter with multiple collections in the right and lower abdomen) without any other pathology [Figure 2]. At laparotomy, we encountered a multicystic mass (5 cm) within the mesentery closely related to the ileum [Figure 3]. There were purulent flakes on the surface of the mass, and the omentum had wrapped around it. Inspection of the retrocecal area revealed a dilated, firm appendix with turbid fluid. The child underwent a resection of the mass together with the closely related ileum and an appendicectomy. Histology confirmed the definitive diagnoses of acute transmural appendicitis. The mass showed multiple cystic spaces with gelatinous fluid. The walls were lined by flattened cells positive for CD31 immunostaining suggestive of a cystic LA of the mesentery of the ileum. There was a prominent acute inflammatory cellular infiltrate in the stroma adjacent to the cystic spaces. Postoperative recovery was uneventful, and the child was discharged well in 5 days. Follow-up over 6 months has been uneventful.
Figure 1: Abdominal ultrasound showing multicystic lesion (a) right iliac fossa (b) pelvis

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Figure 2: Abdominal computed tomography findings

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Figure 3: Operative findings showing a cystic mass within the ileal mesentery closely adherent to the bowel walls. Purulent exudate overlying the cystic mass

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   Discussion Top


Pediatric abdominal LA is more common in boys and usually occurs before the age of 5 years.[2] Most of the abdominal LA occur in the mesentery followed by the omentum and retroperitoneum.[3] Presentation varies from being asymptomatic to prenatal diagnosis. It may present as abdominal emergencies that mimick appendicitis.[4],[5],[6] Accurate preoperative diagnosis may be challenging.[5],[6],[7]

Although most cases of LA are discovered incidentally, they may be found in association with intra-abdominal emergencies such as intestinal volvulus.[8] In addition, traumatic hemorrhage within the LA may present as an abdominal emergency in the pediatric age group.[4]

The histological feature of acute inflammation around the cystic spaces may suggest a causative association between an abdominal infection and inflammatory presentation of the mesenteric LA. To the best of our knowledge, there was only one case report, which reported the development of a mesenteric lymphatic malformation postappendectomy, suggesting that the trauma of surgery or intra-abdominal infection may have triggered the exponential growth and development of the tumor.[9]

We believe that there is a potential for laparoscopic approach if the preoperative diagnosis was accurate. Laparoscopic excision of abdominal cystic LA in children is feasible, safe, and effective. There have been similar cases that have been treated by the laparoscopic approach.[10]


   Conclusion Top


Abdominal LA is a rare occurrence in children. Intra-abdominal infection such as appendicitis may reveal the presence of a concomitant abdominal LA due to a trigger in its growth and development. Preoperative imaging for abdominal pain may suspect these lesions and guide the operative approach.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Dowd CN. XI. Hygroma cysticum colli: Its structure and etiology. Ann Surg 1913;58:112-32.  Back to cited text no. 1
    
2.
Wake S, Abhyankar A, Hutton K. Abdominal Cystic Lymphangioma Mimicking Appendicitis. European Journal of Pediatric Surgery Reports. 2013;1(1):32-34.  Back to cited text no. 2
    
3.
Rønning G, Revhaug A, Størmer J, Eide TJ, Lindal S. Cystic lymphangioma of the small bowel mesentery. Eur J Surg 1995;161:203-5.  Back to cited text no. 3
    
4.
Porras-Ramirez G, Hernandez-Herrera MH. Hemorrhage into mesenteric cyst following trauma as a cause of acute abdomen. J Pediatr Surg 1991;26:847-8.  Back to cited text no. 4
    
5.
Eisawi A, Otter M, Asha M, Al-Temimi A. A case of a giant cystic lymphangioma mimicking acute appendicitis. Ann R Coll Surg Engl 2012;94:e24-5.  Back to cited text no. 5
    
6.
Jayasundara J, Perera E, Chandu de Silva MV, Pathirana AA. Lymphangioma of the jejunal mesentery and jejunal polyps presenting as an acute abdomen in a teenager. Ann R Coll Surg Engl 2017;99:e108-e109.  Back to cited text no. 6
    
7.
Konen O, Rathaus V, Dlugy E, Freud E, Kessler A, Shapiro M, et al. Childhood abdominal cystic lymphangioma. Pediatr Radiol 2002;32:88-94.  Back to cited text no. 7
    
8.
Traubici J, Daneman A, Wales P, Gibbs D, Fecteau A, Kim Mesenteric lymphatic malformation associated with small-bowel volvulus - two cases and a review of the literature. Pediatr Radiol. 2002;32:362–65.  Back to cited text no. 8
    
9.
Hunter C, Connelly M, Lee S, Wang L, Nguyen N. Mesenteric lymphatic malformation associated with acute appendicitis: A case report. J Med Case Rep 2009;3:9030.  Back to cited text no. 9
    
10.
Marte A, Papparella A, Prezioso M, Cavaiuolo S, Pintozzi L. Mesenteric cyst in 11-year old girl: A technical note. Case report. J Pediatr Surg Case Rep 2013;1:84-6.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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