Katsaros D, Grundfest-Broniatowski S
Department of Surgery, Fairview General Hospital, Cleveland, Ohio, USA.
Am Surg. 1998 Apr;64(4):302-4.
Klippel-Trenaunay-Weber syndrome (KTWS) is a rare, congenital disorder characterized by vascular nevus formation, deep venous thrombosis, varicosities, and hypertrophy of affected tissues. A patient with known thrombosis of his splanchnic circulation and visceral KTWS presented with life-threatening hemorrhage from rectosigmoid varices. Portosystemic shunting was not feasible. Endoscopic sclerosis, variceal ligation, and proctocolectomy were not possible due to the size and number of the varices. Previous treatment with epsilon-aminocaproic acid had been unsuccessful and complicated by thrombophlebitis. Conservative treatment with blood transfusions, cryoprecipitate, fresh frozen plasma, vitamin K, propanolol, and somatostatin analog failed to stop the bleeding. The patient was given the antifibrinolytic agent, tranexamic acid, with cessation of his hemorrhage. Serial thromboelastograms confirmed improved reaction time, coagulation time, clot formation rate, and maximum amplitude. We conclude that tranexamic acid may be a useful adjunct in the medical treatment of high-risk patients with KTWS and other vascular nevi complicated by coagulopathy.
克-特-韦综合征（KTWS）是一种罕见的先天性疾病，其特征为血管痣形成、深部静脉血栓形成、静脉曲张以及受累组织肥大。一名患有内脏KTWS且已知存在内脏循环血栓形成的患者，出现了来自直肠乙状结肠静脉曲张的危及生命的出血。门体分流术不可行。由于静脉曲张的大小和数量，内镜下硬化治疗、曲张静脉结扎术和直肠结肠切除术均无法实施。先前使用ε-氨基己酸治疗未成功，且并发了血栓性静脉炎。输血、冷沉淀、新鲜冰冻血浆、维生素K、普萘洛尔和生长抑素类似物的保守治疗未能止血。给予患者抗纤维蛋白溶解剂氨甲环酸后，出血停止。系列血栓弹力图证实反应时间、凝血时间、血块形成率和最大振幅均有所改善。我们得出结论，氨甲环酸可能是治疗伴有凝血病的高危KTWS患者和其他血管痣的有用辅助药物。
