Klippel-Trenaunay syndrome involving the rectum: surgical treatment after interventional radiological preparation

Abdominal organs such as the rectum and urinary bladder are rarely involved in Klippel-Trenaunay syndrome, but may occasionally be the source of sever e blood loss. Since frequently no isolated source of bleeding is identified , severe blood loss can result in a critical condition. This article descri bes an unusual multimodal treatment concept for a patient with Klippel-Tren aunay syndrome associated with severe recurrent rectal bleeding. We present the case of a 39-year-old patient with Klippel-Trenaunay syndrome and a hi story of rectal bleeding since childhood requiring multiple blood transfusi ons over the years. He was referred to our department in a state of continu ous rectal bleeding. Preoperative work-up revealed a complete alteration of the rectum and the distal parts of the sigmoid/colon by hemangiomas, with diffuse bleeding from the destroyed rectal mucosa. Preoperatively the super ior rectal artery was embolized. After a 48-h interval, sphincter-preservin g complete rectal excision including the sigmoid/colon was performed follow ed by a colon pouch anal anastomosis and protective loop ileostomy.