[Klippel-Trénaunay syndrome with involvement of the rectum. Surgical therapy after interventional-radiologic...

Abdominal organs such as the rectum and urinary bladder are rarely involved in Klippel-Trénaunay syndrome, but may occasionally be the source of severe blood loss. Since frequently no isolated source of bleeding is identified, severe blood loss can result in a critical condition. This article describes an unusual multimodal treatment concept for a patient with Klippel-Trénaunay syndrome associated with severe recurrent rectal bleeding. We present the case of a 39-year-old patient with Klippel-Trénaunay syndrome and a history of rectal bleeding since childhood requiring multiple blood transfusions over the years. He was referred to our department in a state of continuous 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 superior rectal artery was embolized. After a 48-h interval, sphincter-preserving complete rectal excision including the sigmoid/colon was performed followed by a colon pouch anal anastomosis and protective loop ileostomy.