Small Bowel Lymphangioma

Abstract

Purpose: Mesentric lymphangioma is a rare benign cystic tumor of lymphatics of the bowel. It is usually found in the first decade of life and has a female predominance in adults. It differs from other mesenteric and retroperitoneal cysts, as it is proliferative and invasive in nature. It is usually asymptomatic in adults and found on surgery or autopsy. It rarely presents as occult GI bleed and iron deficiency anemia. We present such a patient in whom capsule endoscopy was diagnostic. Methods: Our patient is a 36 year old female who presented with fatigue, shortness of breath, occassional diffuse abdominal pain, melena and dizziness for 6 months. She denied any nausea, vomiting, hematochezia, NSAID use or family h/o cancer. Labs revealed Hb of 3.7, Hct 12.5, MCV 60.4, Plt 461, WBC 7.2, Iron 2, Ferritin 3, TIBC 367 and positive FOBT x 3. After transfusion of 4 units PRBC, Hb improved to 9.8. Small bowel enteroscopy with biopsies revealed mild antral gastritis, H. pylori negative and normal villous architecture. Colonoscopy was completely normal. Abdominal CT and Small Bowel series showed luminal narrowing and thickening of small bowel loops in left upper quadrant. Patient was given a patency capsule which passed without any complications. Capsule endoscopy revealed a mass like lesion with yellowish – white discoloration with active bleeding in the small bowel. Laparoscopic resection of the lesion showed diffuse dilatation of the mucosal, submucosal and subserosal lymphatics consistent with small bowel lymphangioma. The post operative course was uneventful and patient was discharged. Results: Conclusion: Abdominal lymphangiomas are uncommon benign tumors. The mean age at presentation is 2.2 years with a male predominance. Etiology may be benign proliferation of ectopic lymphatics. 95% of lymphangiomas are found in the neck and axilla and very rarely found in the intestine. Clinical presentation include abdominal pain, distention, fever, and vomiting. There may be features of small bowel obstruction or volvulus. In an Australian study of 416 capsule endoscopies, 27 tumors were identified of which only 1 was a lymphangioma. Plain radiography may demonstrate small-bowel obstruction or noncalcified soft-tissue mass. CT and MRI may show multiloculated fluid-filled masses, thickening of the bowel wall and reveal size of the tumor, characteristics of the cyst wall and location. Capsule endoscopy may lead to earlier detection and treatment and an improved prognosis for patients with these neoplasms. The prognosis of lymphangiomas depends on location and extent of the lesion. Complete resection is the treatment of choice and has an excellent prognosis. The recurrence rate ranges from 0–13.6%. Malignant degeneration to a low-grade sarcoma is rare.