She underwent surgical resection of the small bowel mass seen on endoscopy. Exploratory laparotomy was unable to locate the mass on a standard bowel run. Intraoperative enteroscopy was performed through an enterotomy in the midjejunum, unveiling an enlarged fold with a protruding surface approximately 10 cm distal to the ligament of Treitz. The mass was resected with adequate margins, and the small bowel was closed without complication. Anatomic examination showed a 1.3 × 1.0 cm yellow-tan, polypoidal mass-like lesion. Histologic examination confirmed dilated lymphatic channels in the submucosa consistent with lymphangiectasia (Figure 3). She resolved clinically and was discharged in a stable condition.
Lymphangiectasia is a rare, benign cystic lesion of endothelium-lined lymphatic spaces with surrounding stromal and connective tissue.3 It most commonly occurs in children in the first few years of life and present in many anatomic locations.4 The majority (>90%) occurs in the neck or axillary regions, whereas the remainder occurs in the lung, mediastinum, and abdomen.3 Gastrointestinal lymphangiomas have been reported to occur with origin in the mesentery, but lymphatic malformation with primary small bowel origin has rarely been described.4–13 To our knowledge, this is the first reported case of primary small bowel-originating lymphangioma causing intussusception.
Lymphangiectasia is thought to originate from a congenital or acquired malformation of lymphatic vessels, forming a cystic area of dilated vascular spaces.7,8 Severity can range from solitary masses (ie, lymphangioma). Our patient had focal jejunal lymphangiectasia with sparing of the surrounding mucosa. Although most occur congenitally, they can be acquired because of abdominal trauma, inflammation, radiation, or abdominal surgery.14,15 Our patient did present with pancolitis because of previously undiagnosed IBD. Although the etiology in our case is unclear, ongoing bowel inflammation may have triggered the formation of the lymphangiectasia, which resulted in this acute clinical presentation.
Signs and symptoms of this lesion vary. The most severe variant, primary intestinal lymphangiomatosis, is characterized by diffuse involvement of the small bowel lymphatics, resulting in a protein-losing enteropathy.16 Reported cases describe complications of mesenteric volvulus and perforation.7,8,17 Our patient presented with intussusception and upstream bowel distention, emesis, and gastrointestinal bleeding. Only 5% of intussusceptions occur in adults, commonly because of adhesions, metastatic or primary neoplasm, feeding tubes, or idiopathic etiology.18 It is thought to occur from a proximal bowel segment “telescoping” into a distal bowel segment using a “lead point.” Our patient's lymphangiectasia may have acted as a lead point in the distal jejunum to induce intussusception.
Lymphangiectasia may be evaluated with several imaging modalities. Ultrasound can reveal a uniloculated or multiloculated, anechoic cystic mass with possible wall contrast enhancement on CECT.19 However, cystic lesion on imaging is not diagnostic of lymphangiectasia because its appearance may mimic organ cysts, carcinomas, and abscess.19 A mass was not seen on CECT for our patient, likely for several reasons. First, the lymphangioma was small and found to be embedded in the submucosa. Second, any visible luminal mass on CECT would have been hidden in the bulk of the intussusception. Therefore, endoscopic evaluation is likely necessary if symptomatic lymphangiectasia is suspected. Surgical resection is effective in relieving symptoms with little disease recurrence.10 Underlying gastrointestinal disorders should be adequately treated to promote recovery and minimize inflammation. Our patient did not report return of any symptoms at 2 follow-up visits.
In conclusion, primary intestinal lymphangiectasia is a rare lesion that can predispose to small bowel intussusception with resulting obstruction. Intussusception in an adult should raise clinical suspicion for primary lymphangiectasia; appropriate clinical management with supportive care and surgical resection may avoid the grave consequences of peritonitis and vascular compromise.
Author contributions: All authors contributed equally to this manuscript. Y. Zafar is the article guarantor.
Financial disclosure: None to report.
Previous presentation: This case was presented at the American College of Gastroenterology Annual Scientific Meeting; October 5–10, 2018; Philadelphia, Pennsylvania.
Informed consent was obtained for this case report.
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© 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.
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