|Year : 2012 | Volume
| Issue : 2 | Page : 91-95
Intra-abdominal cystic lymphangiomas in children: A case series
Parag J Karkera, Gursev R Sandlas, Ritesh R Ranjan, Krushnakumar Kesan, Abhaya R Gupta, Rahul K Gupta, Paras R Kothari
Department of Pediatric Surgery, Lokmanya Tilak Municipal General Hospital, Sion, Mumbai, India
|Date of Web Publication||3-Apr-2013|
Parag J Karkera
B/18, Rachna Apartments, V. P. Road, Andheri (West), Mumbai
Source of Support: None, Conflict of Interest: None
Background: Lymphangiomas are hamartomas of lymphatic vessels commonly arising in the head, neck, and axilla. Intra-abdominal cystic lymphangiomas are rare and are located in the retroperitoneum, the mesentery, omentum, or visceral organs. Radiological imaging in the form of ultrasound and computed tomography (CT) scan helps in preoperative diagnosis. The objective of this study is to present the management of children with intra-abdominal cystic lymphangioma in our institution.
Materials and Methods: Six cases of intra-abdominal cystic lymphangiomas comprising three males and three females, which presented over 15 months, were studied. Clinical presentation, location, mode of surgical intervention, and outcome were studied.
Results: The site of origin was retroperitoneum, omentum, and the mesentery, with two cases of each. Age of presentation ranged from 5 days to 7 years. Abdominal lump was the most common presenting feature. All the patients underwent complete surgical resection - four had open excision, one had a laparoscopy converted to open excision, and one had a complete laparoscopic excision. Postoperative recovery was uneventful in all of them, with no recurrence at 6 months follow-up.
Conclusion: Intra-abdominal cystic lymphangiomas in children are usually symptomatic. CT scan is more accurate than ultrasound to diagnose the lesion. Complete excision of the lesion with or without intestinal resection provides symptomatic relief and prevents recurrence.
Keywords: Cystic lymphangioma, intra-abdominal, mesentery, omentum, retroperitoneum
|How to cite this article:|
Karkera PJ, Sandlas GR, Ranjan RR, Kesan K, Gupta AR, Gupta RK, Kothari PR. Intra-abdominal cystic lymphangiomas in children: A case series. Arch Int Surg 2012;2:91-5
|How to cite this URL:|
Karkera PJ, Sandlas GR, Ranjan RR, Kesan K, Gupta AR, Gupta RK, Kothari PR. Intra-abdominal cystic lymphangiomas in children: A case series. Arch Int Surg [serial online] 2012 [cited 2020 Aug 9];2:91-5. Available from: http://www.archintsurg.org/text.asp?2012/2/2/91/110037
| Introduction|| |
Lymphangiomas are regarded as malformations that arise from sequestration of lymphatic tissue failing to communicate normally with the lymphatic system.  They most commonly occur in the head, neck, and axilla. Other sites include mouth, arm, mediastinum, lung, abdomen, and viscera. Intra-abdominal cystic lymphangiomas are rare and comprise less than 5% of all cystic lymphangiomas. , Intra-abdominal cystic lymphangiomas are uncommon benign malformations of congenital origin, and are often discussed in conjunction with mesenteric cysts.  They may arise from the retroperitoneum, the mesentery, omentum, and visceral organs. The common presentations of intra-abdominal cystic lymphangiomas are abdominal mass and distension.  The prognosis of lymphangiomas depends on the location and extent of the lesion and the presence of other associated abnormalities.  Differentiating cystic lymphangiomas from other cystic growths by imaging studies alone is often inconclusive, and surgery is most frequently required for definitive diagnosis and to ameliorate the symptoms.  Although an abdominal lymphangioma is considered benign, it may become locally invasive. Complete resection including the involved organ is the treatment of choice. Incomplete resection may lead to recurrence. Long-term prognosis after complete excision is excellent. Follow-up imaging is advised, with ultrasound as the modality of choice.  We present our experience with six cases with three different sites of presentation and discuss their course of management and review of literature.
| Materials and Methods|| |
We had six cases of intra-abdominal lymphangiomas in the span of 15 months, from November 2010 to January 2012 [Table 1]. There were three male and three female patients. All patients underwent radiological imaging, surgical excision, and histological confirmation of diagnosis. The modes of clinical presentation, location, surgical intervention, and outcome were studied.
|Table 1: Summary of the clinical information of six patients with intra-abdominal cystic lymphangiomas|
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| Results|| |
They presented between 5 days and 7 years of age. The male:female ratio was 1:1. Lump in abdomen, abdominal distension, and pain in abdomen were the most common presenting features. One patient (case no. 3) was incidentally detected on ultrasound of the abdomen for a routine check-up. The clinical diagnosis in two patients was considered as ascites, in one patient (case no. 1) it was attributed to large cyst size with generalized abdominal distension, and in another patient it was due to history of pulmonary tuberculosis in mother (case no. 2). The youngest patient (case no. 6) in the series was 5 days old and presented with neonatal intestinal obstruction with abdominal distension, bilious vomiting, and constipation.
All patients underwent ultrasound of the abdomen as the initial mode of investigative imaging. Ultrasonography was inconclusive in three patients, with a misdiagnosis of loculated ascites in one and a differential diagnosis of mesenteric cyst and ovarian cyst given in the other two patients. Although computed tomography (CT) scan of the abdomen could diagnose all cases as lymphangiomas, it could diagnose the location of lesions correctly only in two cases, both arising from the retroperitoneum. The location of the other four was diagnosed only after surgical exploration. On surgical exploration, it was found that two cases were arising from the omentum, two from the mesentery (one each from the ileal and jejunal mesentery), and two in the retroperitoneum. One patient (case no. 6) had volvulus of the ileal segment bearing it, resulting in intestinal obstruction.
Four cases (case nos 1, 2, 3, and 6) underwent open exploratory laparotomy to completely excise the cyst. The mesenteric cysts (case nos 2 and 6) needed resection of the adjacent bowel segment which was traversing through the cyst. Case nos 4 and 5 underwent laparoscopic excision, in which the fourth case was completely excised laparoscopically. In case no. 5, most of the dissection was done laparoscopically, but a part of the lesion was closely related to the right ureter. So, to prevent damage to the ureter, the final part of the dissection was completed with a mini-laparotomy. All patients had an uneventful postoperative recovery and are doing well on follow-up with no recurrence detected on ultrasound at 6 months follow-up. The histopathologic diagnosis in all cases was lymphangioma showing multiple cystic spaces lined by flat, low-lying epithelium with surrounding tissue of scattered lymphoid cells.[Figure 1], [Figure 2], [Figure 3]
|Figure 1: (a) CT abdomen of intra-abdominal CL – omental in origin (case no. 1), (b) intraoperative picture of omental lymphangioma (case no. 1)-a, (c) intraoperative picture of omental lymphangioma (case no. 1)-b|
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|Figure 2: (a) CT abdomen of intra-abdominal CL – arising from the mesentery (case no. 2), (b) intraoperative picture of mesenteric lymphangioma (case no. 2)-a, (c) intraoperative picture of mesenteric lymphangioma (case no. 2)-b|
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|Figure 3: (a) CT abdomen of intra-abdominal CL – retroperitoneal in origin (case no. 3), (b) postoperative specimen of retroperitoneal lymphangioma (case no. 3)|
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| Discussion|| |
Lymphangiomas are benign lesions characterized by proliferating lymphatic vessels. Various theories have been postulated on the development of lymphangiomas, focusing on a combination of inflammatory, fibrotic, and genetic components. Other factors include mechanical pressure and retention, trauma, degeneration of lymph nodes, and disorders of endothelial lymphatic vascular secretion or permeability.  The frequent development of lymphangiomas within areas of primitive lymph sacs suggests these to be malformations arising from sequestrations of lymphatic tissue that fail to communicate normally with the lymphatic system, or developmental defects between the 6 th and 9 th weeks of embryonic development resulting from abnormal budding of the lymphatic endothelium. , These abnormal lymphatic channels would dilate, frequently resulting in the formation of uni- or multi-cystic masses that might subsequently be inflamed or obstructed, leading to additional lymphangiomas. The cystic masses are filled with chylous or serous material and are lined with a layer of endothelium.  Generally, lymphangiomas are classified into three types: Capillary (or simple), cavernous, and cystic, depending on the size of the lymphatic spaces. Simple lymphangiomas consist of small, thin-walled, lymphatic channels with considerable connective tissue stroma. Cavernous types are composed of dilated lymphatic channels, whereas cystic lymphangiomas are distinguished by single or multiple cystic masses. 
Approximately 50% of lymphangiomas are present at birth, and almost 90% are diagnosed before the age of 2 years.  Only one patient (case no. 6-neonatal age) in our study was diagnosed and treated before 2 years of age. The mean age at presentation was 2.2 years.  The median age of presentation in our study was 5 years, with the mean age being 2.06 years. In most series, the males outnumber the females, but in our study the male to female ratio was 1:1. ,,
Abdominal cystic lymphangiomas occur most commonly in the small bowel mesentery, with the retroperitoneum being the second most common site. ,, We had equal incidence of occurrence in the omentum, retroperitoneum, and the mesentery, with two cases of each. The clinical presentation of an abdominal cystic lymphangioma depends on its size and location.  The most common presenting features of abdominal lymphangiomas are abdominal pain, followed by abdominal distension and abdominal lump. In this series, abdominal lump was the most common presenting feature, followed by abdominal distension and pain in abdomen. Clinical presentation may be chronic in which there may be gradual abdominal distension, and abdominal mass with or without pain. It may present in acute form in which there is acute pain, distension, fever, vomiting, and peritonitis. Features of small bowel obstruction may occur by extrinsic luminal compression, by traction on the mesentery, or by volvulus as shown in one of our patients.  Most of the retroperitoneal lymphangiomas are asymptomatic and are discovered incidentally in later life on radiological imaging for other conditions, or during surgery or autopsy.  Complications include intestinal obstruction (most common), volvulus, hemorrhage into the cyst, infection, rupture, cystic torsion, and obstruction of the urinary and biliary tract. , In accordance with literature, one of our patients with retroperitoneal lymphangioma was asymptomatic and incidentally detected on ultrasound. Only one patient presented with a complication due to the lesion, i.e., volvulus of ileum, leading to intestinal obstruction.
Ultrasound, CT scan, and MRI of the abdomen have been shown to be complimentary in the diagnosis of abdominal lymphangiomas. Ultrasonography can demonstrate the cystic nature of the lesion appearing with sharp margins, particularly the septations, as scattered internal echoes. CT scan shows well-defined, thin-walled multiseptate lesion and distinguishes from ascites by the absence of bowel loop separation or fluid in the typical sites, such as the cul-de-sac. CT and MRI can demonstrate uni-or multi-locular cysts with septae, an assessment of the relationship of lymphangiomas to neighboring organs and characteristically, fluid of water density. ,, The ability of MRI to provide images in multiple planes without loss of resolution may demonstrate additional lesions and further delineate the boundaries of the cysts.  We did ultrasound and CT scan abdomen in all cases. In our experience, unlike ultrasound, CT scan could diagnose lymphangiomas in all cases. However, in most of the cases (four out of six), it could not diagnose the location of the lesion.
The definitive treatment for abdominal cystic lymphangiomas is complete surgical excision. Recurrence is a possibility with incomplete removal of the lesion, arising from, or involving an abdominal organ. ,,, All our patients had complete resection, with resection of the adjoining involved organ in two cases (jejunal and ileal segments). A simple total excision is usually the preferred treatment to avoid super-infection, progressive growth, rupture, or bleeding, and the outcome following complete resection is generally good. Peritonitis, bleeding, abscess, and torsion are rare postoperative complications.  Cyst-enterostomy or marsupialization had been used in the past as alternate modes of surgical management, but have now become obsolete.  Laparoscopic resection for intra-abdominal lymphangiomas has been successfully attempted before and is a more recent approach to management. , We attempted laparoscopic resection in the last two cases and were successful in resecting the mass in toto in one of them.
| Conclusion|| |
Intra-abdominal cystic lymphangiomas are usually involved in young children and are usually symptomatic. CT scan of the abdomen is more accurate than ultrasound in diagnosing the lesion, but it may still be inconclusive to detect the origin of the lesion. To prevent recurrence, complete excision of the cystic lymphangioma with or without intestinal resection is mandatory.
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[Figure 1], [Figure 2], [Figure 3]