Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2018  |  Volume : 23  |  Issue : 1  |  Page : 36--38

Ileal cystic lymphangioma presenting with acute appendicitis

Su-Ann Lui, Yoke Lin Nyo, VP Mali 
 Department of Pediatric Surgery, National University of Hospital, Singapore

Correspondence Address:
V P Mali
Department of Pediatric Surgery, National University of Hospital, 5 Lower Kent Ridge Road, 119074


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.

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;23:36-38

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 2022 Dec 1 ];23:36-38
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Full Text


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

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}{Figure 2}{Figure 3}


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]


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.

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Conflicts of interest

There are no conflicts of interest.


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