(P 29) ABDOMINAL LYMPHANGIOMA CAUSING EXCESSIVE GASTROINTESTINAL BLEEDING FOR MORE THAN TWO DECADES

Objective: Intraabdominal lymphangioma is a very rare benign tumor of the lymphatic system. These tumors are characterized by dilated lymphatic spaces, and predominantly occur on the mesentery, the greater omentum, mesocolon and retroperitoneum. Small bowel or duodenal lymphangiomas are very seldom, and may cause severe gastrointestinal bleeding. The preferred treatment for small bowel lymphangioma is surgical resection. We report on a unique case of a duodenal/small bowel lymphangioma which caused excessive gastrointestinal bleeding for more than two decades.

Methods: We obtained data from the patient’s health journal and the Danish Registry for Pathology. The patient provided written, signed informed consent.

Results: A 64-year-old male presented with severe gastrointestinal bleeding at 12 years of age. Surgical resection of a lymphangioma was performed, and the patient did not have bleeding for 28 years. Gastrointestinal bleeding reoccurred 24 years ago, and a CT-scan showed a tumor at the 3rd-4th part of the duodenum and the proximal jejunum. Endoscopic biopsy was performed and the diagnosis of lymphangioma was established. The tumor caused severe gastrointestinal bleeding and medical treatment was unsuccessful. The patient was presented for surgery at a tertiary hospital, but was told several times that resection of the tumor was impossible, and that he probably would die during surgery. During the first ten years, the patient received transfusions with up to 18 units of packed red blood cells weekly. Ten years after diagnosis, treatment with tranexamic acid was initiated, and transfusion was reduced to 7 packed red blood cells weekly. Despite massive transfusions the patient was doing fine. After 24 years, the total number of transfusions was exceeding 5000 pRBC. Twenty-four years after diagnosis, the patient was presented at our surgical department. A CT-scan was performed which showed the lymphangioma and a tumor at the left kidney. A kidney biopsy revealed papillary renal cell carcinoma. Surgery was performed with resection of a 16 x 25 cm large lymphangioma including the 3rd-4th part of the duodenum and the proximal jejunum. A side-to-side anastomosis between the 2nd part of the duodenum and the proximal jejunum was performed. Furthermore, a left hemi-nephrectomy was performed. The postoperative course was uncomplicated, and the patient was discharged at the 7th postoperative day. At follow-up, he was fine and without transfusions four months after surgery.

Conclusion: Duodenal/Small bowel lymphangiomas may cause excessive bleeding, and surgical resection can be a successful treatment option.