A 53-year-old man was referred to our hospital for repetitive episodes of bloody stool with severe anemia. He had a long history of swelling of the left lower extremity and intermittent anal bleeding since the age of 7, and Klippel-Trenaunay-Weber syndrome was diagnosed at the age of 17.
On November 18, 1990, when he urderwent emergency operation for perforated duodenal ulcer at the Shichinohe Public Hospital, varices involving the rectum and sigmoid colon were also observed. Since he continued to show intestinal bleeding and colonic stenotic symptom later on, he was transferred to our hospital for radical operation for these varices. On Feb. 26, 1991, he underwent anoabdominal rectosigmoid resection with transanal coloanal anastomosis. Bowel movement frequency was initially ten to twelve times a day after closure of the diverted ileostomy. But 6 months later, his bowl movement frequency was two to five times a day, and never showed anal bleeding.
In Japan, 6 cases of Klippel-Trenaunay-Weber syndrome with rectosigmoid varices have been reported. All cases were managed conservatively, and this is the first case treated by the sphincter preserving operation. This procedure is found to effect a radical cure in these situations.
