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Year : 2016  |  Volume : 9  |  Issue : 3  |  Page : 374-376  

Lymphangioma causing duodenal obstruction in adult, rare presentation

Department of Radiology, KEM Hospital, Pune, Maharashtra, India

Date of Web Publication17-May-2016

Correspondence Address:
Prashant W Khade
Department of Radiology, KEM Hospital, Rastapeth, Pune - 411 011, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-2870.182511

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A submucosal lymphangioma is a rare pathology in the alimentary tract. It is a benign entity of the lymphatic system. A duodenal lymphangioma is extremely rare and has an unknown etiology. Clinical and laboratory findings are nonspecific, and they are incidentally found by endoscopy and their treatment is surgical excision. Small lesions are often asymptomatic. Recently, the authors experienced a 62-year-old man, who had a duodenal lymphangioma, showing gastric outlet obstruction symptoms. A multidetector computed tomography abdomen study revealed a single submucosal cystic lesion in second part of duodenum. The lesion was successfully excised after Whipple's procedure. Histopathology confirmed the diagnosis of lymphangioma.

Keywords: Duodenum, lymphangioma, multidetector computed tomography, submucosal

How to cite this article:
Khade PW, Kalekar T, Shetty D, Tambe S. Lymphangioma causing duodenal obstruction in adult, rare presentation. Med J DY Patil Univ 2016;9:374-6

How to cite this URL:
Khade PW, Kalekar T, Shetty D, Tambe S. Lymphangioma causing duodenal obstruction in adult, rare presentation. Med J DY Patil Univ [serial online] 2016 [cited 2023 May 31];9:374-6. Available from:

  Introduction Top

Submucosal lymphangioma of the duodenum is a very rare benign tumor. Only a few cases have previously been mentioned in the literature. Even lymphangioma with intestinal obstruction is extremely rare. This tumor is detected by multidetector computed tomography (CT) as a submucosal cystic lesion and diagnosis confirmed by histopathology followed by surgery. We herein report a case of submucosal lymphangioma of the duodenum, diagnosed on radiological imaging, removed by duodenectomy and confirmed on histopathology.

  Case Report Top

A 62-year-old man presented with complaints of nonbilious vomiting after taking food since 5 days, with increasing episodes of vomiting since 1-day. Patient was anemic with hemoglobin value of 10.0 mg%. Patient had history of pain in abdomen and intermittent vomiting. There is no history of hematemesis. On admission day patient had present with three episodes of vomiting. Lab investigations reported abnormal serum electrolytes (serum Na + -133 mEq/L, serum K + -3.4 mEq/L, serum Cl-98 mEq/L and HCO 3 -33 mEq/L). Upper gastrointestinal (GI) endoscopy study suggestive of polyp in second part of duodenum. Patient was referred to our department for CT scan. CT abdomen was performed by using negative oral contrast and intravenous nonionic contrast. It revealed well-defined cystic lesion (CT value 12-15 HU) at junction of first and second part of duodenum along the inner margin of wall with bulging of mucosal layer causing near total luminal compromise. Dilated stomach and first part of duodenum filled with negative contrast media was noted. Alimentary tract distal to lesion was appeared to be collapsed. On nonionic contrast imaging, there was marginal enhancement [Figure 1] and [Figure 2]. Hence, diagnosis of submucosal lymphangioma was made and duplication cyst was given as other possibility. Surgical removal was done by duodenectomy with Whipple's procedure. Histopathology report confirmed the diagnosis of lymphangioma [Figure 3] and [Figure 4].
Figure 1: Computed tomography abdomen axial and sagittal sections reveals a well-defi ned submucosal cystic lesion (arrow) in duodenum compromising the bowel lumen with resultant signifi cant dilatation of proximal duodenum and stomach

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Figure 2: Computed tomography abdomen axial and sagittal sections reveals a well-defi ned submucosal cystic lesion (arrow) in duodenum compromising the bowel lumen with resultant signifi cant dilatation of proximal duodenum and stomach

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Figure 3: Pathological specimen under low power light microscopy reveals multiple dilated lymphatic channels (asterisk) filled proteinous fluid in mucosa and submucosa of bowel wall

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Figure 4: Pathological specimen under low power light microscopy reveals multiple dilated lymphatic channels (asterisk) filled proteinous fluid in mucosa and submucosa of bowel wall

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  Discussion Top

Lymphangioma is commonly seen in head and neck region and rare in alimentary tract and it is even extremely rare in duodenum. Lymphangiomas are lymphatic malformation arising from sequestered lymphatic tissue that fail to communicate with normal lymphatic system. Subsequently, cellular proliferation and fluid accumulation results in cyst formation. They occur in many anatomic locations with pediatric or adult clinical presentation. Most (95%) occur in the neck and axillary regions; the remaining 5% are located in the mesentery, retroperitoneum, abdominal viscera, lung, and mediastinum. [1] Morphologically, lymphangiomas are classified into three groups: Lymphangioma simplex, cystic lymphangioma and cavernous lymphangioma. [2] According to the classification of Watson and McCarthy, three different types of lymphangiomas may be found in the GI tract: Simple, cavernous, and cellular. These lesions have the potential to grow to considerable size. [3]

Most of the intestinal lymphangiomas are asymptomatic and found accidentally on endoscopy or in imaging. Symptomatic lymphangioma are very rare. These lesions present as upper abdominal pain and or malena. Rarely shows intestinal obstruction with pain, distension and repeated episodes of vomiting. Chronic nonspecific abdominal signs include chronic abdominal pain and progressive abdominal distension. [3] Radiologically, it appears a well-defined cystic lesion with smooth margin, arising from the intestinal wall causing partial or complete luminal compromise. On endoscopy, it appears a yellow, polypoidal mass lesion in the bowel lumen. [2] Lymphangiomas are thin-walled cystic masses with a smooth gray, pink, tan, or yellow external surface. The cysts may contain chylous, serous, hemorrhagic, or mixed fluid. Histologically, dilated lymphatic spaces are lined with attenuated endothelial cells and resembling the cells that line normal lymphatics. The lymphatic spaces are usually filled with proteinaceous eosinophilic fluid. [1]

Three entities must be differentiated from lymphangioma, first is duplication cyst and other are lymphatic cyst and lymphangiectasia. The jejunum is the most common site of duplication cyst followed by the duodenum. [4],[5],[6] Duplication cyst is common at the medial border of 1 st and 2 nd part of the duodenum. [4],[5],[6] It is congenital abnormality present in childhood and extremely rare with a presentation in older age. [4] Duplication cyst can be cystic or tubular. They also classified as communicating and noncommunicating. [4] On trans-abdominal or endoscopic real-time ultrasound (US) cyst showing "gut signature" and peristalsis are characteristic findings of duplication cyst. [4],[7] A lymphatic cyst is never larger than 1 cm and not causing obstructive symptoms. [8] These are associated with aging process and on endoscopy appear as a small yellowish nodule in proximal small bowel protruding from mucosal or serosal surface. [8] Lymphangiectasia are primary or secondary. Small intestine is common site; although stomach and colon may also be involved. These are presents with steatorrhea, chyluria and chylous ascites. Histologically showing numerous dilated lymphatic channels in mucosa and submucosa.

Radiological discussion

Lymphangiomas appear as cystic lesions with thin-walls and with or without septations on imaging studies. On US, it is an anechoic cyst but may contain echogenic debris as a result of infection or hemorrhage. [3] Lymphangiomas are of fluid attenuation on CT having CT values range 5-20. It is along the mucosal layer of duodenal wall with bulging of the mucosal layer in lumen. Most of the lesions rarely show bowel obstruction. Few lesions show complete compromise of the bowel lumen. These lesions in proximal duodenum show symptoms and signs of gastric outlet obstruction. On CT abdomen postcontrast images, a cystic nonenhancing lesion with or without peripheral enhancement is seen bulging into the lumen. [3] In case of large lymphangioma local vascular congestion may be seen due stretching of wall. Outer duodenal margin appear regular with normal enhancement. No surrounding fat planes are maintained. No exophytic component is seen. Calcifications may be seen in the wall. On magnetic resonance imaging, the cyst would show low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. Peripheral marginal enhancement is seen on postgadolinium sequences. Wall or septa can enhance. [3]

  Conclusion Top

Lymphangioma is a very rare entity in the duodenum. Bowel obstruction due to lymphangioma even has rare occurrence. Imaging is very helpful for describing the location, appearance, signs of obstruction and narrowing window for possible differentials. However, to confirm the diagnosis pathological investigations are required. Management on early incidental finding can avoid the occurrence of the acute presentation.


We thank our Institute Academic Section, KEM Hospital for valuable support. We thank our colleagues from Department of Pathology, who provided insight and expertise that greatly assisted the article. We thank our Head of Department Dr. Dayanand Shetty for valuable guidance. We thank all our contributor authors for their hard work for this article.

Financial support and sponsorship

Pathology Department, KEM Hospital, Pune and online publications.

Conflicts of interest

There are no conflicts of interest.

  References Top

Levy AD, Cantisani V, Miettinen M. Abdominal lymphangiomas: Imaging features with pathologic correlation. AJR Am J Roentgenol 2004;182:1485-91.  Back to cited text no. 1
Ikura Y, Hashimoto T, Takamine Y, Tani T, Konishi Y, Uchida H, et al. Lymphangioma of the duodenum: Report of a case. Surg Today 1994;24:160-3.  Back to cited text no. 2
Mehrotra AK, Sobin LH. Gastrointestinal lymphangioma. 2009;8:9-10. Available from:  Back to cited text no. 3
Al-Harake A, Bassal A, Ramadan M, Chour M. Duodenal duplication cyst in a 52-year-old man: A challenging diagnosis and management. Int J Surg 2013;4:296-8.  Back to cited text no. 4
Merrot T, Anastasescu R, Pankevych T, Tercier S, Garcia S, Alessandrini P, et al. Duodenal duplications. Clinical characteristics, embryological hypotheses, histological findings, treatment. Eur J Pediatr Surg 2006;16:18-23.  Back to cited text no. 5
Macpherson RI. Gastrointestinal tract duplications: Clinical, pathologic, etiologic, and radiologic considerations. Radiographics 1993;13:1063-80.  Back to cited text no. 6
Spottswood SE. Peristalsis in duplication cyst: A new diagnostic sonographic finding. Pediatr Radiol 1994;24:344-5.  Back to cited text no. 7
Davis M, Fenoglio-Preiser C, Haque AK. Cavernous lymphangioma of the duodenum: Case report and review of the literature. Gastrointest Radiol 1987;12:10-2.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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