Lehmann T G, Düx M, von Herbay A, Lehnert T
Chirurgische Onkologie, Chirurgische Universitätsklinik, Heidelberg.
Chirurg. 2000 Feb;71(2):228-33. doi: 10.1007/s001040050039.
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.
诸如直肠和膀胱等腹部器官很少累及克-特综合征，但偶尔可能是严重失血的来源。由于常常无法确定单一的出血源，严重失血可导致危急情况。本文描述了一位患有克-特综合征并伴有严重复发性直肠出血患者的一种不同寻常的多模式治疗方案。我们报告了一例39岁的克-特综合征患者，自童年起就有直肠出血史，多年来需要多次输血。他因持续直肠出血被转诊至我科。术前检查发现直肠和乙状结肠/结肠远端被血管瘤完全改变，受损的直肠黏膜弥漫性出血。术前对直肠上动脉进行了栓塞。48小时后，进行了保留括约肌的全直肠切除术，包括乙状结肠/结肠，随后进行结肠袋肛管吻合术和保护性回肠造口术。
