SOLITARY ILEAL LYMPHANGIOMA, A RARE CAUSE OF OBSCURE INTESTINAL BLEEDING REQUIRING TRANSFUSION SUCCESSFULLY TREATED WITH LAPAROSCOPIC RESECTION
Small bowel lymphangioma is a rare benign vascular system tumor that mostly presents in children as head, neck or axillary region masses. Generally they are asymptomatic and don’t require treatment, unless complications arise, such as bleeding or obstruction. Small bowel lymphangiomas are very rare in adults and intestinal bleeding secondary to a solitary ileal lymphangioma presenting with severe anemia requiring transfusion are rarely reported.
54 year old male presented to ED with sudden onset of lower abdominal pain without remarkable past medical history. A small urinary tract stone with hydronephrosis was identified. Incidentally on the abdominopelvic CT an area of mural thickening of a short segment of small bowel in the left lower quadrant was noted. Mild asymptomatic anemia was found, and patient was recommended outpatient endoscopic follow-up with analgesics for the renal calculi.
The patient was lost to follow up due to insurance reasons and returned to our hospital after three months with recurrent and severe anemia requiring blood transfusion and intravenous iron treatment. He denied any episodes of melena, hematochezia, chest pain, nausea and vomiting. The patient only complained of the mild exertional SOB, but was compensating by decreasing activity at home. Clinical examination revealed marked conjunctival pallor. No masses were palpated on rectal or abdominal examination. On admission a microcytic anemia with hemoglobin of 4.3 gr/dL was noted. CT was performed showing segmental circumferencial thickening of a loop of ileum without perienteric stranding and lymphadenopathies (Fig. 1,2). Remainder of the large and small bowel including terminal ileum was identified normal. The patient received a transfusion prior to upper and lower endoscopy.
Esophagogastroduodenoscopy was normal except mild gastritis. Colonsocopy didn’t reveal active bleeding or abnormalities in the colon. No bleeding was noted in the terminal ileum. Subsequently, he underwent technetium labeled red blood cell scan and no focus of active bleeding identified (Fig. 3).
Given the CT findings and endoscopic workup, the patient underwent diagnostic laparoscopy and intraoperatively an 8 cm section of the mid-ileum was found distended distinctly from the surrounding bowel and it was resected with the feeding mesentery with a 5cm margin. Histologic examination showed multiple endothelium lined cystic spaces and associated smooth muscle fibers consistent with a lymphangiomatous lesion.
Lymphangiomas are rare lymphatic system tumors, mostly found in children younger than five years. The common sites of lymphangiomas are head, neck and axillary regions. Among these, tumors at other locations such as abdominal cavity including small bowel are extremely rare in the adult population. The exact cause of these soft tissue slow growing tumors are still unknown, but most likely due to some malformations of the lymphatic system development. The symptoms of lymphangioma are acute or chronic bleeding, rupture, infection, intussusceptions, torsion, protein-losing enteropathy.
The clinical evaluation of intestinal bleeding caused by small bowel lymphangiomas depends on the hemodynamic status of the patient. It can be diagnosed with enteroscopy, video capsule endoscopy or abdominal imaging if they are large enough. The diagnosis may be difficult with enteroscopy when they are located in distal jejenum or ileum and only few case reports causing bleeding have been described. If no source of the bleeding identified, the small bowel can be evaluated using small bowel follow-through enteroclysis, technetium-99m–tagged red blood cell scan and arteriography.
The first line of the treatment of intestinal bleeding caused by small bowel lymphangioma in a hemodynamically stable patient is bleeding control by enteroscopy. The surgery can be necessary when bleeding is not controlled by gastroenteroscopy.
When the source of bleeding cannot be identified by endoscopic, radiographic, or nuclear imaging, the patient may need exploratory laparoscopy. To avoid recurrence, laparoscopic segmental bowel resection is an optimal approach.