Bleeding from cavernous angiomatosis of the rectum in Klippel-Trenaunay syndrome: Report of three cases and literature review

Klippel-Trenaunay syndrome (KTS) is a congenital vascular anomaly character ized by limb hypertrophy, cutaneous hemangiomas, and varicosities. GI hemor rhage is a potentially serious complication secondary to diffuse hemangioma tous involvement of the gut. We report on three patients with KTS who prese nted with transfusion-dependent anemia and life-threatening bleeding due to extensive cavernous hemangiomas involving the rectum. Two patients were tr eated by proctocolectomy and coloanal anastomosis, which preserved anal fun ction while controlling bleeding. The third patient required an abdominoper ineal resection because of extensive rectal, perianal, and perineal. angiom atosis. The literature on the evaluation and management of GI hemorrhage in KTS, particularly of colorectal origin, is reviewed. (Am J Gastroenterol 2 001;96:2783-2788. (C) 2001 by Am. Coll. of Gastroenterology).