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克-特综合征中的直肠出血:腹腔镜超低位前切除术联合括约肌间分离术治疗

Rectal bleeding in Klippel-Trénaunay syndrome: treatment with laparoscopic ultralow anterior resection with intersphincteric dissection.

作者信息

Rotholtz Nicolas, Bun Maximiliano E, Laporte Mariano, Sandra Lencinas, Carlos Peczan, Mezzadri Norberto

机构信息

Hospital Alemán de Buenos Aires, Argentina.

出版信息

Surg Laparosc Endosc Percutan Tech. 2009 Oct;19(5):e206-9. doi: 10.1097/SLE.0b013e3181bae76b.

Abstract

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.

摘要

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。

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