|Year : 2014 | Volume
| Issue : 3 | Page : 164-166
Rare location of mesenteric cystic lymphangioma in an infant
Minakshi Sham1, Jyoti Kudrimoti2
1 Department of Paediatric Surgery, B. J. Medical College, Pune, Maharashtra, India
2 Department of Pathology, B. J. Medical College, Pune, Maharashtra, India
|Date of Web Publication||8-Sep-2014|
Dr. Minakshi Sham
G/101, Sudarshan Apartments, Behind Spencer's Daily, Karve Nagar, Pune - 411 052, Maharashtra
Source of Support: None, Conflict of Interest: None
A 2-month-old female infant presented with subacute intestinal obstruction of 1½ month duration. Investigations revealed a cystic intra-abdominal lesion. On exploration, a large multicystic mass was noted in the ileal mesentery with induration and inflammatory interbowel adhesions. It had resulted in bowel volvulus. It was completely excised along with the involved bowel. Histopathologic examination diagnosed the tumor to be a cystic lymphangioma. It is the youngest patient with mesenteric cystic lymphangioma ever reported in the literature.
Keywords: Ileum, infant, intestinal resection-anastomosis, mesenteric cystic lymphangioma, multiloculated tumor
|How to cite this article:|
Sham M, Kudrimoti J. Rare location of mesenteric cystic lymphangioma in an infant. J Clin Neonatol 2014;3:164-6
| Introduction|| |
Cystic lymphangioma of the mesentery is a rare pathology, often not described in literature and occasionally confused with mesenteric cysts. , Moreover, its' etiopathogenesis is still uncertain. Surgery is the only modality of treatment for both acute and subacute forms.  It is strongly recommended even in asymptomatic cases because of the potential to grow, invade vital structures and develop life-threatening complications from associated volvulus. 
| Case Report|| |
A 2-month-old female child had history of abdominal fullness and constipation of 1½ month duration. She presented in the emergency department with vomiting, decreased urine output and increased abdominal distension over past 3-4 days. The child was febrile and dehydrated. She weighed 5 kg. A lump of 8 × 7 × 5 cm size was palpable in the right iliac fossa. It was firm, mobile in all directions and dull to percussion. There was no history of detecting the lump antenatally. She was otherwise a healthy baby, delivered at 38 weeks of gestation with birth weight of 3 kg. There was no evidence of other congenital anomalies.
Erect X-ray abdomen showed few gas filled rounded opacities in cohesion, on the left side of the abdomen with paucity of gas in the rest of the abdomen and pelvis [Figure 1]. Ultrasonography of the abdomen detected a heterogeneous, predominantly cystic mass lesion on the right side of the abdomen, causing displacement of bowel loops. Computerised tomography scan showed a complex, septate lesion extending from the sub-hepatic region to the right iliac fossa with enhancing soft tissue components and few tiny eccentric calcific foci within it. Presence of mesenteric lymph nodes was also noted. The liver, spleen, kidneys, pancreas, and ovaries appeared normal. Her hematological investigations were normal. In view of the examination findings and radiologic evaluation, working diagnosis was that of a mesenteric cyst.
On abdominal exploration, a large multiloculated cystic mass was found within the mesentery of ileum with the overlying bowel segment completely engulfed by it [Figure 2]. It had resulted in bowel volvulus. However, there was no gangrene. Several interbowel adhesions needed to be released before actually reaching the bowel containing the cyst. The surrounding bowel loops were also inflammed and edematous. The cysts were filled with chylous fluid.
|Figure 2: Operative photograph showing large multiloculated cystic mass in ileal mesentery with overlying bowel|
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In view of presence of a large multiloculated cyst and its infiltrative nature, en mass excision of the cyst, portion of mesentery containing the cyst and approximately 12-15 cm of adjoining bowel segment was done. Bowel continuity was established by single layer, interrupted, extramucosal sutures using 4-0 Polyglactin. Postoperative course was uneventful. Breastfeeds were started from postoperative day 6 and were well-tolerated.
The excised tumor weighed 300 g. On cut section, it showed multiple lymph-filled cysts encroaching onto the adjacent bowel [Figure 3]a. Histopathology showed multiple cystic spaces lined by flattened endothelial cells and containing lymph within them. The cysts were separated by fibrous septa infiltrated by lymphocytes, consistent with lymphangioma [Figure 3]b. Bowel margins were tumor free.
|Figure 3: (a) Cut open specimen showing multiloculated lymph filled cysts and narrow bowel at center (b) Histopathology slide showing evidence of cystic lymphangioma (H and E, ×40)|
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Presently, at a follow-up of 2 years, the child is totally asymptomatic, thriving well and has attained age-appropriate milestones. There is no radiographic evidence of tumor recurrence. She is on regular follow-up to assess tumor recurrence in the long run.
| Discussion|| |
Cystic lymphangiomas are considered to be congenital malformations stemming from sequestration of the lymphatic tissue. They characteristically infiltrate surrounding structures by local extension and can produce new lesions by extension or new growth.  These lesions are seen in the tongue, cheek, chest, buttocks, extremities, and peritoneal cavity in decreasing order of frequency.  Gluteal region, pelvis, retroperitoneum, mesentery, inguinal region and inguinoscrotal region are considered to be rare locations of cystic lymphangioma. 
Intra-abdominal cystic lymphangiomas have an estimated incidence of <1/1,00,000 hospital admissions.  They present with chronic abdominal distension or acute intestinal obstruction with or without peritonitis.  They occur in children under 5 years of age and are more common among boys. , Though abdominal lymphangiomas are usually clubbed together with mesenteric cysts, they differ from the later by age of presentation, location, histology, and the potential for recurrence. Hence, they should be considered a separate clinical entity.  Although the exact etiology of mesenteric lymphangioma is unknown, their occurrence in neonates and infants supports the theory that they are primary congenital malformations.  The best and only radical treatment is total removal of the lesions with microscopically clear borders. 
In our case, the preoperative diagnosis was that of a mesenteric cyst. However, a large multiloculated mesenteric tumor was noted, on opening the abdomen. The overlying portion of the bowel was completely surrounded and almost engulfed by the cyst. In view of the giantic size, presence of induration and surrounding inflammation, resection of the multiloculated cyst, portion of mesentery and adjacent intestine was done.
de Perrot et al., have reported three children with mesenteric cystic lymphangiomas (MCLs). In their series of six patients (three children and three adults), abdominal lymphangiomas presented more acutely in children and usually involved the mesentery; whereas in adults the history was longer and the tumor was found in the retroperitoneum.  Similar is the presentation in our case, since the child presented at 2 months with subacute intestinal obstruction. Weeda et al. have reported two cases of MCLs in combination with malrotation and intermittent volvulus. Both MCLs were located near the duodenojejunal junction.  Contrary to this, in our case the tumor was located in the terminal ileum near the IC junction. There was presence of volvulus, albeit without gangrene.
As in our case, MCL is often diagnosed on histopathological examination. Since complete surgical resection with histopathologically clear margins is important to prevent tumor recurrence, one should look for tale-tale signs which lead to preoperative suspicion of MCL. Firm to hard mass on palpation, distinct radiological picture suggestive of a multiloculated mass, intra-operative evidence of tumor invasion are few preoperative indicators of MCL. Taking heed of them will lead to suspicion of MCL, so that complete radical excision can be achieved and tumor recurrence and future catastrophic complications can be prevented.
| References|| |
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[Figure 1], [Figure 2], [Figure 3]