Treatment of colonic bleeding in Klippel-Trenaunay syndrome with combined partial colectomy and endoscopic laser

Abstract

Klippel-Trenaunay syndrome can be associated with significant lower gastrointestinal bleeding from diffuse cavernous hemangiomata of the colon. Distal colectomy is curative but the formation of a colostomy is undesirable in patients who already have a poor self-image due to the other manifestations of Klippel-Trenaunay syndrome such as multiple cutaneous hemangiomata, severe varicose veins, and prominent limb hypertrophy. Endoscopic laser ablation alone is often not feasible in these patients because of the extensive and often circumferential nature of the colonic hemangiomata. This report describes successful treatment of gastrointestinal hemorrhage due to colonic hemangiomata by a novel combination of partial colonic resection and endoscopic laser therapy. Case 1 demonstrates that endoscopic laser ablation of the diseased colonic mucosa, while not feasible with the extensive involvement of the left colon, was very effective when combined with partial colectomy at curing the bleeding while maintaining anal sphincter function. In case 2 the disease was more limited and more easily amenable to endoscopic laser ablation. Combined surgical and laser endoscopic therapy is recommended in treating the colonic hemangiomata associated with Klippel-Trenaunay syndrome.