Rotholtz Nicolas, Bun Maximiliano E, Laporte Mariano, Sandra Lencinas, Carlos Peczan, Mezzadri Norberto
Hospital Alemán de Buenos Aires, Argentina.
Surg Laparosc Endosc Percutan Tech. 2009 Oct;19(5):e206-9. doi: 10.1097/SLE.0b013e3181bae76b.
Klippel-Trénaunay syndrome is a rare congenital vascular disorder characterized by varicose veins, hypertrophy of the soft tissues and bones, and hemangiomas. Although colorectal angiomatosis is uncommon, this association can lead to life-threatening complications because of acute or chronic bleeding. We report a patient with Klippel-Trénaunay syndrome who presented with transfusion-dependent anemia secondary to chronic rectal bleeding. Endoscopy and image studies revealed venous angiomata involving the rectum and distal sigmoid colon. We performed a laparoscopic ultralow anterior resection with intersphincteric dissection, obtaining good results. After a follow-up of 20 months the patient did not present hematochezia and maintained fecal continence.
克-特综合征是一种罕见的先天性血管疾病,其特征为静脉曲张、软组织和骨骼肥大以及血管瘤。虽然结直肠血管瘤病并不常见,但这种关联可因急性或慢性出血导致危及生命的并发症。我们报告一例克-特综合征患者,该患者因慢性直肠出血出现依赖输血的贫血。内镜检查和影像学研究显示直肠和乙状结肠远端有静脉血管瘤。我们进行了腹腔镜超低位前切除术并进行括约肌间分离,取得了良好效果。随访20个月后,患者未出现便血,且保持了大便失禁。