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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 3  |  Page : 411-412
Lymphangiectasia of small intestine presenting as intussusception

Department of Pathology, Government Medical College, Jammu, Jammu and Kashmir, India

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Intussusception is defined as telescoping of a segment of gastrointestinal tract into an adjacent one. In small children, it is the commonest cause of intestinal obstruction. More than 90% of childhood intussusceptions are idiopathic. We report a rare case of localized small intestinal lymphangiectasia, presenting as intussusception in a 6-month-old male child. The child presented with features of acute intestinal obstruction for which he was later operated. The gross examination of excised ileocecal mass revealed intussusception. Histopathologic examination revealed lymphangiectasia of small intestine, which acted as a lead point for ileocecal intussusception. Postoperative period was uneventful.

Keywords: Intussusception, lymphangiectasia

How to cite this article:
Katoch P, Bhardwaj S. Lymphangiectasia of small intestine presenting as intussusception. Indian J Pathol Microbiol 2008;51:411-2

How to cite this URL:
Katoch P, Bhardwaj S. Lymphangiectasia of small intestine presenting as intussusception. Indian J Pathol Microbiol [serial online] 2008 [cited 2020 Jul 8];51:411-2. Available from: http://www.ijpmonline.org/text.asp?2008/51/3/411/42543

   Introduction Top

Intestinal lymphangiectasia was originally described in 1961 by Waldmann et al . [1] Lymphangiectasia is characterized by dilated and proliferating lymphatic channels. Although regarded as a malformation, the precise nature of the lesion, whether neoplastic or hamartomatous, is uncertain. [2]

Most common sites of lymphangiectasia are head, neck and axilla. [2] Involvement of intra-abdominal organs is quite rare, constituting 1.6% of all cases. [3],[4] The ectatic lymphatics may be located in the mucosa, submucosa, or subserosa, leading to loss of proteins and lymphocytes into gut or peritoneal cavity. [5]

A rare case of small intestinal lymphangiectasia, which presented as intussusception in a 6-month-old child, is being reported here.

   Case history Top

A 6-month-old male child was presented in the pediatric surgery department of Government Medical College, Jammu, with abdominal pain and vomiting for 1 week. Physical examination revealed acute abdomen with a palpable mass in right upper quadrant. Preoperative diagnosis of intestinal obstruction was made.

Emergency laparotomy was performed for intestinal obstruction. During the operation, ileocecal mass was removed (ileocecal resection) along with the appendix and a lymph node. Postoperative period was uneventful.

Pathological findings

Gross examination revealed ileocecal mass with attached appendix measuring 7 3 1 cm. The cut section showed intraluminal cystic growth, 3 cm in diameter, obstructing the lumen and dragging with it the ileum into cecum. Cyst contained mucoid fluid. The cyst wall was whitish smooth and 0.3 cm in thickness. Appendix was grossly normal. Lymph node measured 0.6 cm in size.

Microscopic examination revealed numerous dilated and anastomosing lymphatic channels in the mucosa lined by flat and endothelial cells, containing mature lymphocytes. In addition, the lamina propria contained engorged blood vessels and infiltration by chronic inflammatory cells [Figure 1].

Appendix was within normal histological limits. The lymph node showed non-specific reactive changes.

Pathologic diagnosis of small intestinal lymphangiectasia was made.

   Discussion Top

Acute intussusception, presenting as intestinal obstruction, is most common in children; 70-95% are idiopathic and peak age is between 3 and 9 months. [6],[7] Children presenting with intussusception and associated with a lead point such a Meckel's diverticulum, polyp, duplication, Henoch-Shonlein purpura, or appendix are usually older than idiopathic cases. [6]

However, it is not unusual for other small bowel lesions to act as lead point leading to intussusception. Lymphoblastic lymphoma of ileum and small bowel hemangiomas have been reported to present as intussusceptions.[8],[9] Extramedullary hematopoitic tumors in a β-thalasemia/HbE boy have also been reported producing intussusception of intestine. [10] However, 70-95% childhood intussusceptions are idiopathic. [6] In contrast, intussusception in adults is rare and in 70-80% of these cases, there is a demonstrable underlying cause. [6],[7]

The pathogenesis of lymphangiectasia is uncertain but generally attributed to the presence of congenital hypoplastic visceral lymphatic channels, leading to obstruction of lymphatic flow and dilatation of intestinal lymphatics. [5]

In our patient, lymphangiectasia of small intestine acted as a lead point to intussusception and subsequent obstruction. However, very few cases of colonic intussusception due to lymphangioma/lymphangiectasia have been reported in the literature to date. [11],[12]

So, it should be taken into consideration that lymphangiectasia is one of the organic lesions that can cause intussusception.

   References Top

1.Waldmann TA, Steinfeld JL, Dutcher JD, Gordon RS. Role of gastrointestinal system in idiopathic hyproteinemia. Gastroenterology 1961;41:197-207.  Back to cited text no. 1    
2.Ashley DJ. Tumours of vasoformative tissue. In : Evan's Histopathological appearance of tumours, 4th ed. London: Churchill Livingstone; 1990. p. 77-108.  Back to cited text no. 2    
3.Henzel JH, Pories WJ, Bruget DE, Smith JL. Intra-abdominal lymphangiomata. Arch Surg 1966;93:304-8.  Back to cited text no. 3    
4.Chaturvedi KU, Singh N, Sakhuja P. Intra-abdominal lymphangioma- Clinicopathological profile of rare lesions. Indian J Pathol Microbiol 1997;40:563-6.  Back to cited text no. 4    
5.Abramowsky C, Hupertz V, Kilbridge P, Czinn. Small intestinal lymphangiectasia in children: A study of upper GI endoscopic biopsies. Paediatr Pathol 1989;9:289-91.  Back to cited text no. 5    
6.Winslet MC. Intestinal obstruction: In Russel RC, Williams NS, Christopher JK, editors. Bailey and Love's Short practice of surgery; 24 th ed. London: Edward Arnold (Publishers) Ltd; 2004. p. 1186-202.  Back to cited text no. 6    
7.Coran AG. Intussusception in adults. Am J Surg 1969;117:735.  Back to cited text no. 7    
8.Shah P, Ramakantan R. Ileocaceco-colic intussusception produced by lymphoblastic lymphoma. Indian J Gastroenterol 1989;8:302-3.  Back to cited text no. 8    
9.Morgan DR, Mylankal K, el Barghouti N, Dixon MF. Small bowel haemangioma with local lymph node involvement presenting as intussusception. J Clin Pathol 2000;53:552-3.  Back to cited text no. 9    
10.Wongwaisayawan S, Pornkul R, Teerarakul S, Pakakasama S, Treepongkaruna S, Nithiyanant P. Extra-medullary haematopoietic tumour producing intussusception of intestine in a β-Thalasemia-HbE Boy. J Med Assoc Thia 2000;83:S17-22.  Back to cited text no. 10    
11.Wan YL, Lee TY, Hung CF, Ng KK. Ultrasound and CT findings of a caecal lymphangioma presenting as intussusception. Eur J Radiol 1998;27:77-9.  Back to cited text no. 11    
12.Matsuba Y, Mizuiri H, Murata T, Niimi K. Adult intussusception due to lymphangioma of the colon. J Gastroenterol 2003;38:181-5.  Back to cited text no. 12    

Correspondence Address:
Pervez Katoch
E-15, Temple View, Medical Enclave, Government Medical College, Jammu, Jammu and Kashmir - 180 001
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.42543

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  [Figure 1]

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