Abstract

Major rectal bleeding may occur in children and adults who have extensive hemangiomatous involvement of the pelvis and rectosigmoid colon, as in the Kleppel-Trenaunay syndrome (KTS). Conventional surgical techniques such as bowel resection and colostomy have often been associated with large blood loss and/or incontinence. We have used a new approach to this problem utilizing rectal mucosectomy to eliminate the bleeding rectal mucosa and to preserve anal function. Four patients born with KTS eventually developed major rectal bleeding and were successfully corrected by this surgical approach. Two were females and two males. Ages ranged from 4 to 25 years at the time of surgery. The patients developed rectal bleeding leading to chronic anemia during the first decade of life. Rectal bleeding gradually increased requiring multiple transfusions (2 to 20 units) prior to surgery. Patient 1 underwent resection of the rectosigmoid colon, with rectal mucosectomy and endorectal coloanal anastomosis. The Nd:YAG laser was used on 3 occasions on the distal remaining rectal mucosa. Patients 2, 3, and 4 underwent sigmoid resection, transanal rectal mucosectomy, and a coloanal anastomosis, with minor YAG laser therapy in one patient. Blood loss during surgery was minimal. Follow-up ranged from 1.5 to 4 years. All have excellent sphincter control with no incontinence. No strictures or sepsis occurred following surgery. Rectal bleeding was eliminated in all 4 patients. This sphincter-saving approach should be considered in patients with extensive hemangiomas of the rectosigmoid colon because of its remarkable effectiveness and safety.

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