Klippel-Trenaunay-Weber综合征发病机制及治疗方案探讨

Pathogenesis and staged treatment of Klippel-Trenaunay-Weber syndrome.

目的探讨Klippel-Trenaunay-Weber综合征(KTWS)的发病机制和分期综合治疗的可行性。方法1999年7月至2004年9月广东省人民医院和广州军区广州总医院对14例KTWS病人行患肢动脉造影,对不同的血管病变分别以平阳霉素+超液化碘油、PVA、NBCA、钢圈进行Ⅰ期血管内介入治疗,4周后再行Ⅱ期手术治疗,并观察其疗效。结果13例(13/14)病人术后症状消失,除增长、增粗的患肢未能恢复到健肢完全一致外,其余体征消失。随访12例,平均随访(26·5±9·3)个月,未见复发迹象。结论通过针对KTWS发病机制的分期综合治疗,可取得显著效果。

Objective To explore the pathogenesis of Klippel-Trenaunay-Weber syndrome（KTWS） and the feasibility of staged treatment. Methods From July 1999 to September 2004,14 cases of KTWS were performed arteriography and treated by stage I endovascular intervention with bleomycin（ A5 ） and extraliquid lipiodol,PVA,NBCA,steel coil respectively according to the different vascular lesions in Guangdong Provincial People＇ s Hospital and General Hospital of Guangdong Military Command. Four weeks later, all the cases were performed stage Ⅱ surgical procedure. The therapeutic effect were observed. Results Symptoms in 13 patients among 14 patients treated by staged treatment almost subsided ＇after surgical procedure. The other body signs except for the uncured previously lengthened and thicken limbs also disappearred. Twelve cases were followed up. The average follow-up period was （26. 5±9. 3） months. No evident recurrent signs were observed. Conclusion Staged treatment based on the pathogenesis in 14 cases of KTWS achieved significant effect.