以下肢病变为主的Klippel-Trenaunay综合征的诊治新进展

摘要

Klippel-Trenaunay syndrome( KTS) is a congenital peripheral vascular disease. Simple and shallow lower limb venous anomaly deformity is more commonly seen. Wine stain pole, ectopic varicose veins associated with deep vein normal or lacking,and limb hypertrophy triad is the most common in clinic. The etiology is unclear,the pathogenesis is related with unclosed congenital ischial venous system. The diagnosis of KTS relies on DSA or CT. Implementation of reasonable surgical treatment after diagnosis can greatly improve patients' quality of life.%Klippel-Trenaunay综合征(KTS)是一种先天性周围血管疾病.一般以下肢单纯浅静脉异常畸形较为常见,临床上以葡萄酒色斑痣、异位浅静脉曲张伴深静脉正常或阙如以及肢体肥大三联征最为常见.病因尚不明确,发病机制与先天性坐骨静脉系未闭合有关.目前,KTS的诊断主要依靠数字减影血管造影或者CT,明确诊断后行合理的手术治疗可极大地改善患者的生活质量.