Intestinal Lymphangioma Presenting With Small Bowel Bleeding

ABSTRACT

Obscure gastrointestinal bleeding poses a diagnostic challenge for gastroenterologists, though advancements such as video capsule endoscopy and enteroscopy have led to increased detection of small bowel lymphangiomas. Lymphangiomas are benign tumors with dilated lymphatic channels that rarely affect the jejunum or ileum. We present a rare case of jejunal lymphangioma causing obscure gastrointestinal bleeding diagnosed by video capsule endoscopy, confirmed by enteroscopy, and successfully treated with surgical resection.

INTRODUCTION

Lymphangiomas are benign tumors caused by dilation of lymphatic channels, commonly occurring in the head, neck, axillary regions, and parenchymal organs such as the spleen, liver, and bone. However, they are rarely found in the gastrointestinal tract (GI) and are particularly uncommon in the small intestine. Lymphangiomas account for 6% of small bowel tumors in children and 1.4%–2.4% in adults.¹

Small bowel lymphangiomas are often asymptomatic when small; larger lesions can cause symptoms including abdominal pain, GI bleeding, anemia, intestinal obstruction, intussusception, or protein-losing enteropathy.

We present a rare case of jejunal lymphangioma causing significant recurrent anemia diagnosed through VCE, confirmed by enteroscopy, and successfully treated with surgical resection.

CASE REPORT

A 46-year-old woman presented with recurrent iron deficiency anemia with a hemoglobin level of 46 and 51 g/L (normal 117–160) 3 months apart. She required blood transfusions on both occasions and was subsequently placed on intravenous iron transfusions with a partial response. She was referred to the gastroenterology clinic for evaluation. Her symptoms included fatigue, palpitations, exertional dyspnea, and dizziness, but no overt GI bleeding.

Workup with esophagogastroscopy and colonoscopy failed to reveal the source of iron deficiency anemia. Subsequently, video capsule enteroscopy (VCE) revealed a large frond-like/villous mass with active bleeding in the distal duodenum to the proximal jejunum (Figure 1). Double balloon enteroscopy (DBE) identified a large, ulcerated distal jejunal mass, which was biopsied and tattooed for surgical reference (Figure 2). Pathology from biopsies revealed dilated lymphatics in the lamina propria of the jejunal mucosa, representing secondary lymphangiectasia from lymphatic obstruction due to the mass lesion. However, the mass lesion itself was not included in the biopsy. No granuloma or mass lesion was identified on examination of deeper levels.

Figure 1.

Images from the video capsule endoscopy showing a large frond-like/villous mass with bleeding (arrows) was found at 0 hour 31 minutes and areas corresponding to the distal duodenum/proximal jejunum.

Figure 2.

Images from double balloon enteroscopy showing a large ulcerated mass showing typical endoscopic findings of white spots on its surface.

The patient underwent laparoscopic resection of the mass, with intraoperative findings confirming a palpable tumor at 115 cm from the ligament of Treitz. Resection of the mass with 10 cm margins and a jejunojejunal side-to-side anastomosis was performed (Figure 3).

Figure 3.

Images from laparoscopy showing tattooed area with distal palpable mass.

Histologic examination confirmed the diagnosis of lymphangioma characterized by dilated and anastomosing thin-walled proteinaceous material-filled lymphatic channels lined by a single layer of flattened endothelial cells extending into the lamina propria and abutting the underlying muscularis. Margins were tumor free, and the maximum tumor size was 3.5 cm (Figure 4).

Figure 4.

Images from pathology slide of the lesion labeled as intestinal lymphangioma showing dilated and anastomosing thin-walled lymphatic vessels filled with proteinaceous material.

The patient had an uncomplicated postoperative course and was discharged on the third postoperative day. At 3 months, a repeat abdominal computed tomography scan showed no recurrent lesion, her hemoglobin level had improved from 95 to 101 g/L, and she remained symptom free with no abdominal pain or overt GI bleeding.

DISCUSSION

Lymphangiomas are rare benign tumors of the lymphatic system characterized by the presence of dilated lymphatic channels. Intestinal lymphangioma primarily affects the small intestine, with jejunal or ileal tumors being extremely rare, accounting for less than 1% of all lymphangiomas.²

They have been mainly described in case reports and case reviews, including 34 reported cases of lymphangiomas in Japan from 1967 to 1991. A review of the English-language literature from 1960 to 2009 identified only 40 cases of small bowel lymphangiomas, with equal sex distribution and occurrence among all age groups.³

Lymphangiomas are often discovered incidentally but can present with abdominal pain, acute or chronic GI bleeding, intestinal obstruction, volvulus, intussusception, or protein-losing enteropathy.^4–7 According to previous reports, jejunal tumors often present with melena and abdominal pain, while ileal tumors may manifest with intussusception or obstruction.²

The mechanism by which lymphangiomas cause GI bleeding remains unclear. It is hypothesized that obstruction of lymphatic flow within these lesions leads to increased pressure on the lymphatic-venous connections, resulting in retrograde blood flow into the lymphatic channels, ultimately causing bleeding.⁸

Advancements in diagnostic tools, such as VCE and DBE, have led to improved detection of small bowel lymphangiomas.

Typical endoscopic appearance includes a soft submucosal mass with white spots on the surface that, when grasped, may exude milky liquid.⁹ VCE can help locate small intestinal lymphangiomas and detect their associated complications including bleeding as in our case.

Several case reports have explored the efficacy of DBE in the diagnosis and treatment of bleeding intestinal lymphangiomas with therapies, such as endoscopic polypectomy, hemoclipping, argon plasma coagulation, and endoscopic submucosal dissection (ESD).¹⁰

Endoscopic ultrasonography is also helpful in the diagnosis of small intestinal lymphangioma showing characteristic appearance of anechoic-hypoechoic cystic tumor originating from the third layer.⁹

Generally, lymphangiomas smaller than 2 cm with no local invasion can be treated by endoscopic resection if accessible endoscopically. In a previous case report, complete en bloc resections contributed to excellent outcomes and prognoses for patients.¹¹ Many studies have illustrated that endoscopic resection is suitable for smaller lymphangiomas, while laparoscopic surgery is the recommended management for larger tumors involving the muscularis propria or when the lesion is suspected malignant. In a systemic retrospective analysis evaluating 24 cases of upper GI, lymphangioma was resected by endoscopic mucosal resection (EMR) (91.67% n = 22) or ESD (8.33% n = 2), with successful resection. In addition to the adverse events that occurred in 3 patients who finally recovered, there were no other cases of recurrence during the median follow-up period of 43 months.¹² The associated adverse events included postoperative acute pancreatitis and postoperative bleeding. Endoscopic therapy is characterized by less trauma, lower technical complexity and complications, less pain, and faster recovery compared with surgical resection.¹³ This was also demonstrated in another case series where one proximal jejunal and one distal duodenal sessile lesion were completely resected by en bloc EMR with no complications reported.¹⁴ Lesions that are small and thought to be symptomatic and are in a favorable position should be considered for EMR, while lesions that are >2 cm and are submucosal should be removed by advanced techniques such as ESD. EMR with hot snare resection or band-EMR should be decided based on lesion size, location, and expertise. GI lymphangiomas can present morphologically in multiple forms, such as flat, protruding, or polypoid cystic lesions that can be difficult to resect with conventional polypectomy techniques. Deroofing was explored and successfully performed in those cases with cyst drainage.¹⁵

Surgical treatment is required in about 20% of cases to control related hemorrhage, after failure of endoscopic therapy, resection of larger lesions, or to treat mechanical complications, including obstruction, intussusception, or volvulus. Lymphangiomas have reported recurrence rates of 12% and 53% when completely or partially resected, respectively. In addition, surgery can be associated with complications such as lymphatic leaks, fistula formation, and chronic wounds.¹⁶

Lymphangiomas are classified as benign tumors, and resection should be reserved for symptomatic or large lesions.^17,18 Interestingly, in the abovementioned case series, many lesions were resected for unrelated complaints, such as patients with esophageal lymphangiomas presenting with bloating or duodenal lymphangiomas in patients presenting with regurgitations.¹¹ It is essential that endoscopic or surgical resections are done judicially to avoid unnecessary health costs and complications.

This case highlights the rarity and diagnostic complexity of jejunal lymphangiomas presenting with obscure gastrointestinal bleeding. Despite advancements in diagnostic modalities such as VCE and DBE, a definitive diagnosis may still necessitate surgical intervention and histopathological examination, as demonstrated in this case. The patient's successful management through complete surgical resection underscores the importance of a multidisciplinary approach involving gastroenterologists, radiologists, and surgeons in cases of persistent or life-threatening symptoms.

Although lymphangiomas are benign, their potential complications, including significant GI bleeding and obstruction, necessitate prompt recognition and appropriate treatment if indicated. Surgical resection remains the gold standard for large or symptomatic lesions. This case adds to the limited literature on jejunal lymphangiomas and highlights the role of modern endoscopic techniques in their detection and management, ultimately improving patient outcomes.

DISCLOSURES

Author contributions: F. Mahmoud: writing and editing; P. Anglade, J. Ghaith, and J. Raza: editing. F. Mahmoud is the article guarantor.

Financial disclosure: None to report.

Informed consent was obtained for this case report.

ABBREVIATIONS:

DBE

double balloon enteroscopy

EMR

endoscopic mucosal resection

ESD

endoscopic submucosal dissection

GI

gastrointestinal

VCE

video capsule enteroscopy